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Binienda A, Salaga M, Patel M, Włodarczyk J, Fichna J, Venkatesan T. Serotonin Receptors Polymorphisms Are Associated With Cyclic Vomiting Syndrome. Neurogastroenterol Motil 2025; 37:e15012. [PMID: 39888244 DOI: 10.1111/nmo.15012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/29/2024] [Revised: 01/07/2025] [Accepted: 01/14/2025] [Indexed: 02/01/2025]
Abstract
BACKGROUND Cyclic vomiting syndrome (CVS) is a disorder characterized by sudden, recurrent episodes of severe nausea and vomiting. The pathophysiology of CVS is not known but genetic factors that regulate emetic neurocircuitry have been proposed. The aim of this study was to investigate whether different variations in genes encoding serotonin receptors (HTRs) are associated with susceptibility to CVS and/or CVS symptoms. METHODS This case-control study included 70 patients with CVS:16 male and 54 female, and 2504 healthy controls from the 1000 Genomes Project database. Single-nucleotide polymorphisms (SNPs) in genes encoding serotonin receptors (HTR1B, HTR1D, HTR3B and HTR3C) and correlations between SNPs and the symptoms of CVS were determined. KEY RESULTS Our study discovered that patients with GG, AA and GG genotypes of HTR1B/D rs6296, rs6298 and rs6300, respectively, as well as the CC genotype of HTR3B rs176744 are associated with an increased risk (p < 0.001), whereas allele C in rs3788987 (HTR3B, p < 0.01) and allele A in rs6766410 (HTR3C, p < 0.05) were associated with a decreased risk of CVS. In addition, statistical analysis indicated that CVS patients with GA or AA genotypes of HTR1D rs676643 gene have a seven-fold increase in risk of depression compared to patients with GG genotype (p < 0.01). CONCLUSIONS AND INFERENCES Our study revealed for the first time that variations in 5-HTR genes may contribute to CVS susceptibility and CVS-related symptoms.
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Affiliation(s)
- Agata Binienda
- Department of Biochemistry, Faculty of Medicine, Medical University of Lodz, Lodz, Poland
| | - Maciej Salaga
- Department of Biochemistry, Faculty of Medicine, Medical University of Lodz, Lodz, Poland
| | - Milan Patel
- Division of Gastroenterology and Hepatology, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
| | - Jakub Włodarczyk
- Department of Biochemistry, Faculty of Medicine, Medical University of Lodz, Lodz, Poland
| | - Jakub Fichna
- Department of Biochemistry, Faculty of Medicine, Medical University of Lodz, Lodz, Poland
| | - Thangam Venkatesan
- Division of Gastroenterology, Hepatology and Nutrition, The Ohio State University, Columbus, Ohio, USA
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Malagelada C, Keller J, Sifrim D, Serra J, Tack J, Mulak A, Stengel A, Aguilar A, Drewes AM, Josefsson A, Bonaz B, Dumitrascu D, Keszthelyi D, Barba E, Carbone F, Zerbib F, Marchegiani G, Hauser G, Gourcerol G, Tornblom H, Hammer H, Aziz I, Matic JR, Mendive J, Nikaki K, Wauters L, Alcalá‐González LG, Waluga M, Jinga M, Corsetti M, Rommel N, Shidrawi R, De Giorgio R, Kadirkamanathan S, Surdea‐Blaga T. European Guideline on Chronic Nausea and Vomiting-A UEG and ESNM Consensus for Clinical Management. United European Gastroenterol J 2025; 13:427-471. [PMID: 39754724 PMCID: PMC11999049 DOI: 10.1002/ueg2.12711] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/16/2024] [Revised: 09/03/2024] [Accepted: 09/04/2024] [Indexed: 01/06/2025] Open
Abstract
INTRODUCTION Chronic nausea and vomiting are symptoms of a wide range of gastrointestinal and non-gastrointestinal conditions. Diagnosis can be challenging and requires a systematic and well-structured approach. If the initial investigation for structural, toxic and metabolic disorders is negative, digestive motility and gut-brain interaction disorders should be assessed. United European Gastroenterology (UEG) and the European Society for Neurogastroenterology and Motility (ESNM) identified the need for an updated, evidence-based clinical guideline for the management of chronic nausea and vomiting. METHODS A multidisciplinary team of experts in the field, including European specialists and national societies, participated in the development of the guideline. Relevant questions were addressed through a literature review and statements were developed and voted on according to a Delphi process. RESULTS Ninety-eight statements were identified and voted following the Delphi process. Overall agreement was high, although the grade of scientific evidence was low in many areas. Disagreement was more evident for some pharmacological treatment options. A diagnostic algorithm was developed, focussing on the differentiating features between gastrointestinal motility and gut-brain interaction disorders with predominant nausea and vomiting. CONCLUSION These guidelines provide an evidence-based framework for the evaluation and treatment of patients with chronic nausea and vomiting.
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Hasler WL, Li BUK, Levinthal DJ, Venkatesan T. Cyclic vomiting syndrome: Future clinical and research priorities for: Special supplement/proceedings of 3rd international symposium. Neurogastroenterol Motil 2025; 37:e14825. [PMID: 38775195 DOI: 10.1111/nmo.14825] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/28/2024] [Revised: 04/29/2024] [Accepted: 05/13/2024] [Indexed: 09/21/2024]
Abstract
BACKGROUND An increasing number of studies have explored the clinical features, epidemiology, pathophysiology, and management of cyclic vomiting syndrome (CVS). CVS is common in adults and children and negatively impacts patients, families, and the healthcare system. A related condition, cannabinoid hyperemesis syndrome (CHS), has been a focus of interest in the lay press and published literature. PURPOSE Clinical presentations of CVS have been defined by small series and expert opinion, but recent prospective studies are refining our understanding of the spectrum of emetic episodes and the breadth of comorbid conditions. Large cross-sectional population analyses are clarifying CVS prevalence and factors related to age, ethnicity, and geographic region. CVS pathophysiology is multifactorial with contributions from migraines, dysautonomia, endogenous cannabinoids, mitochondrial dysfunction, genetic abnormalities, and rapid gastric emptying. CVS treatment relies on antiemetics and antimigraine therapies to abort acute episodes coupled with prophylactic regimens employing neuromodulators and antiepileptics. CHS represents a challenge partly because of difficulties in achieving sustained cannabis abstinence. Benefits of other therapies in CHS remain poorly defined. Several areas warrant further scrutiny including better identification of CVS triggers and characterization of different CVS subsets including those with frequent severe episodes, refined description of epidemiology to allow targeting of populations predisposed to CVS development, rigorous definition of pathogenic factors to provide a foundation for exploratory studies of novel therapies, and conduct of controlled trials by multicenter collaborations to confirm benefits of existing and new therapies in development. Progress in these areas will be facilitated by generous governmental and industry support.
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Affiliation(s)
| | - B U K Li
- Medical College of Wisconsin, Milwaukee, Wisconsin, USA
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Levinthal DJ, Killian B, Issenman RM. Acute care of cyclic vomiting syndrome and cannabinoid hyperemesis syndrome in the home and emergency department. Neurogastroenterol Motil 2025; 37:e14901. [PMID: 39155452 PMCID: PMC11911290 DOI: 10.1111/nmo.14901] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/15/2024] [Revised: 08/05/2024] [Accepted: 08/07/2024] [Indexed: 08/20/2024]
Abstract
BACKGROUND Cyclic vomiting syndrome (CVS) and cannabinoid hyperemesis syndrome (CHS) are both characterized by episodic, acute transitions from asymptomatic states to highly symptomatic states of nausea, repetitive vomiting, and often severe abdominal pain. Patients with CVS and CHS face significant challenges to abort or mitigate episodes at home and often require emergency department (ED)-based care. PURPOSE This paper reviews the current treatment approach to abort acute CVS and CHS episodes at home and in ED settings. Multiple pharmacologic and nonpharmacologic interventions have been demonstrated to potentially abort CVS or CHS episodes. Systemic pharmacologic agents often used as abortive therapy include triptans, antiemetics, anxiolytics, NK-1 receptor antagonists, antipsychotics, sedatives in general, and various analgesic / anti-inflammatory medications. Nonsystemic, nonpharmacologic approaches include reducing external stimuli (quiet room, dim lights, etc.), and hot water bathing or the application of topical capsaicin cream. More research is needed to develop evidence-based, individualized abortive treatment plans, as well as to determine whether the abortive treatment for CVS requires a fundamentally different approach than for CHS. When home-based approaches fail, all patients with CVS or CHS should receive nonjudgmental, informed, and compassionate care in the ED to abort their episode. Patients with more severe forms of CVS/CHS who require more frequent ED utilization should develop care plans with their ED to assure predictable and effective treatment.
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Affiliation(s)
| | - Blynda Killian
- Cyclic Vomiting Syndrome AssociationMilwaukeeWisconsinUSA
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5
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Li BUK. Landscape of Cyclic Vomiting Syndrome: From Bedside to Bench, Past to Present. Neurogastroenterol Motil 2025; 37:e14990. [PMID: 39789960 PMCID: PMC11911070 DOI: 10.1111/nmo.14990] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/24/2024] [Revised: 11/25/2024] [Accepted: 12/12/2024] [Indexed: 01/12/2025]
Abstract
Investigations into mechanisms of cyclic(al) vomiting syndrome (CVS) began at the bedside more than a century ago. The modern era started with the formation of the Cyclic Vomiting Syndrome Association in 1993 that helped initiate robust efforts in education, advocacy, family physician conferences, scientific symposia, dedicated clinical programs, therapeutic guidelines, and research. Even today, bedside clues continue to emerge with the recent description of cannabinoid hyperemesis syndrome (CHS) and subsequent evidence of a perturbed endocannabinoid system. The clinical picture of CVS has evolved from that of a straightforward emetic disorder related to migraine requiring short-term antiemetics or prophylactic anti-migraine therapy, to a complicated, heterogenous one with multiple comorbid associations (anxiety, dysautonomia) and endophenotypes (migraine, Sato, CHS). This expanded view has important therapeutic implications which necessitate managing the comorbidities which can in turn impact the disease itself and proffered promising evidence that behavioral management (meditation) and vagal neuromodulation appear efficacious with few untoward effects, perhaps by reestablishing autonomic (parasympathetic) balance. The pathophysiologic picture now appears to be inscribed on an autonomic polyvagal design but multiple additional pathways interact, some confirmed (NK1, CB1, HPA axis, PPM1D gene, biological calendar, estrogen), and others, possible (TRPV-1, CGRP, GDF-15, mitochondrial dysfunction, impaired cation transport). CVS and its cousin CHS continue to challenge clinicians and perplex investigators and in the current era require not only a critical mass of specific pathway expertise but also syncretic biopsychosocial thinking to integrate these disparate threads. We may have reached such a tipping point at this Symposium.
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Affiliation(s)
- B U K Li
- Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, USA
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Bujarska M, Bora G, Li BUK, Banda J, Thomas D, Simpson P, Karrento K. Diagnostic characteristics of pediatric cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2025; 80:417-425. [PMID: 39737559 DOI: 10.1002/jpn3.12448] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/03/2024] [Revised: 08/29/2024] [Accepted: 10/11/2024] [Indexed: 01/01/2025]
Abstract
OBJECTIVES Cyclic vomiting syndrome (CVS) remains a diagnostic challenge due to its nonspecific presentation despite consensus-based diagnostic criteria. There is a need for improved, evidence-based diagnostic criteria. We hypothesized that symptoms differ quantitatively between children with CVS versus other vomiting conditions and that current diagnostic criteria are not sufficiently sensitive for diagnosing CVS. METHODS Observational, prospective study of children ages 3-18 years with unexplained vomiting episodes evaluated in the outpatient gastroenterology clinic, emergency department, and inpatient units at Children's Wisconsin. Parents completed symptom surveys at 0, 3, and 6 months. Diagnostic workup and treatment response were monitored by chart review. A final diagnosis (CVS vs. non-CVS) was assigned for group comparisons and receiver operating characteristics (ROC) analysis of diagnostic cutoffs. RESULTS Of 108 subjects enrolled, 46 CVS and 54 non-CVS patients were analyzed. The groups reported overall different episode frequencies with more CVS versus non-CVS (81% vs. 55%) having ≥4 episodes/preceding 12 months (p = 0.013). CVS patients also had longer vomiting episodes (p = 0.03). To distinguish CVS from non-CVS, ROC analyses demonstrated the highest sensitivity for a frequency of 4-10 episodes/12 months (p = 0.002) and a duration threshold of >2 h (p < 0.001). CVS patients reported specific episode characteristics: photophobia (p = 0.003), diaphoresis (p = 0.002), multiple emeses/hour (p = 0.001), stereotypical episodes (p = 0.008), and continued retching after gastric emptying (p = 0.008). Less than half of CVS patients met Rome IV and North American Society for Pediatric Gastroenterology, Hepatology & Nutrition diagnostic criteria. CONCLUSIONS Children with CVS display a distinctive vomiting pattern and clinical features compared to other vomiting conditions. Our findings will help improve current diagnostic criteria.
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Affiliation(s)
- Malgorzata Bujarska
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
| | - Geetanjali Bora
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Children's National Hospital, Washington, DC, USA
| | - B U K Li
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
| | - Julie Banda
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
| | - Danny Thomas
- Division of Emergency Medicine, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
| | - Pippa Simpson
- Division of Quantitative Health Sciences, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
| | - Katja Karrento
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
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Pizzol A, Adams KA, de Vries BBA, Curry CJ, Calvo PL. Cyclic Vomiting Syndrome in Patients Affected by Jansen-de Vries Syndrome: Results From an International Survey. Am J Med Genet A 2025; 197:e63918. [PMID: 39494522 DOI: 10.1002/ajmg.a.63918] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/30/2024] [Revised: 10/02/2024] [Accepted: 10/14/2024] [Indexed: 11/05/2024]
Abstract
Jansen-de Vries syndrome (JdVS) is an autosomal dominant neurodevelopmental disorder with intellectual disability and gastrointestinal (GI) abnormalities, including recurrent vomiting. This study aimed to understand the frequency and severity of GI symptoms in JdVS patients and to investigate the potential association with cyclic vomiting syndrome (CVS), which has not been previously reported. An international online survey assessed the prevalence and features of CVS and GI disorders in JdVS patients using Rome IV Criteria. The anonymous survey was conducted via Google Forms in April 2021. A total of 21 patients/guardians responded to the survey. The average age at JdVS diagnosis was 8.22 years (range: 1-42). Of the respondents, 6 (28.5%) had a CVS diagnosis, 5 (23.8%) had migraine, and 2 (9.5%) had abdominal migraine. Additionally, 8 (38%) had gastroesophageal reflux disease (GERD) and 8 (38%) had functional constipation. An analysis targeted questions showed that 7 (33%) met the Rome IV Criteria for CVS but were undiagnosed, leading to a CVS prevalence of 61% in this cohort. This study highlights a high prevalence of CVS in JdVS patients and underscores the need for increased awareness and accurate diagnosis to address misdiagnosis.
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Affiliation(s)
- A Pizzol
- Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital, Azienda Ospedaliera-Universitaria Città Della Salute e Della Scienza, Turin, Italy
| | - K A Adams
- Cyclic Vomiting Syndrome Association, Milwaukee, Wisconsin, USA
| | - B B A de Vries
- Department of Human Genetics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands
| | - C J Curry
- Genetic Medicine, Department of Pediatrics, University of California, San Francisco, California, USA
| | - P L Calvo
- Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital, Azienda Ospedaliera-Universitaria Città Della Salute e Della Scienza, Turin, Italy
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Gandhi JJ, Khlevner J. A step closer to bridging the knowledge gap in pediatric cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2025; 80:395-396. [PMID: 39718114 DOI: 10.1002/jpn3.12447] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/27/2024] [Accepted: 12/01/2024] [Indexed: 12/25/2024]
Affiliation(s)
- Jermie J Gandhi
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Columbia University Irving Medical Center/NewYork Presbyterian Morgan Stanley Children's Hospital, New York, New York, USA
| | - Julie Khlevner
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Columbia University Irving Medical Center/NewYork Presbyterian Morgan Stanley Children's Hospital, New York, New York, USA
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Karrento K, Wu M, Rodriguez D, Coyne KS, Tahir MJ, Richmond CA, Chen YJ, Williams J, Venkatesan T. Understanding the adult and adolescent patient experience with cyclic vomiting syndrome: a concept elicitation study. BMC Gastroenterol 2025; 25:85. [PMID: 39962369 PMCID: PMC11834555 DOI: 10.1186/s12876-025-03595-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/24/2024] [Accepted: 01/06/2025] [Indexed: 02/20/2025] Open
Abstract
BACKGROUND Cyclic vomiting syndrome (CVS) is a phasic disorder of gut-brain interaction characterized by episodes of severe nausea and vomiting. In-depth qualitative research on phase-specific CVS symptoms and impacts is lacking. The study objectives were to explore the experience of patients with CVS in the United States and to identify CVS symptoms and impacts on adults, adolescents, and caregivers. METHODS Qualitative, cross-sectional, semi-structured concept elicitation interviews were conducted with adults and adolescents with CVS and with adolescents' caregivers. Adolescents either participated alone or in a dyad format with their caregiver. Interview data were analyzed using an open coding approach. RESULTS Concept elicitation interviews were conducted with 13 adults (mean age 45.3 years [standard deviation (SD) 13.1]) and 15 adolescents (mean age 14.6 years [SD 1.8]). The most frequently reported prodrome phase symptoms were nausea (n = 12, 92.3%), anxiety (n = 10, 76.9%), and abdominal pain (n = 9, 69.2%) in adults, and nausea (n = 15, 100%), abdominal pain (n = 11, 73.3%), and headache (n = 11, 73.3%) in adolescents. All adults reported nausea, tiredness, and dry heaves in the emetic phase, and 12 (92.3%) reported vomiting and retching. The remaining patient said they no longer vomited due to abortive medications. All adolescents reported nausea and vomiting in the emetic phase; other common emetic phase symptoms were abdominal pain (n = 14, 93.3%), dehydration (n = 13, 86.7%), and tiredness (n = 13, 86.7%). The leading most bothersome impact reported by adults was anxiety associated with impending vomiting (n = 5, 38.5%). Among adolescents, the leading most bothersome impact was on school (n = 7/13 asked, 53.8%), and among their caregivers, it was seeing their child suffer (n = 6/11 asked, 54.5%). CONCLUSIONS Patients with CVS experience considerable gastrointestinal and extra-intestinal symptoms. CVS impacts the activities of daily life of patients and their caregivers, with patients reporting negative effects of CVS on their emotional status and their ability to maintain a normal school or work routine.
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Affiliation(s)
| | - Melody Wu
- Takeda Development Center Americas, Inc., 500 Kendall Street, Cambridge, MA, 02142, USA.
| | | | | | - Muna J Tahir
- Takeda Development Center Americas, Inc., 500 Kendall Street, Cambridge, MA, 02142, USA
| | - Camilla A Richmond
- Takeda Development Center Americas, Inc., 500 Kendall Street, Cambridge, MA, 02142, USA
| | - Yaozhu J Chen
- Takeda Development Center Americas, Inc., 500 Kendall Street, Cambridge, MA, 02142, USA
| | - James Williams
- Takeda Development Center Americas, Inc., 500 Kendall Street, Cambridge, MA, 02142, USA
| | - Thangam Venkatesan
- Medical College of Wisconsin, Milwaukee, WI, USA
- The Ohio State University College of Medicine, Columbus, OH, USA
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Kósa M, Horváth E, Kalmár T, Maróti Z, Földesi I, Bereczki C. A Patient Diagnosed with Mosaic Trisomy 18 Presenting New Symptoms: Diaphragmatic Relaxation and Cyclic Vomiting Syndrome. Updated Review of Mosaic Trisomy 18 Cases. J Pediatr Genet 2024; 13:320-325. [PMID: 39502855 PMCID: PMC11534437 DOI: 10.1055/s-0042-1757621] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/07/2021] [Accepted: 08/31/2022] [Indexed: 12/03/2022]
Abstract
Although data on T18 are widespread, there is a lack of knowledge on mosaic trisomy 18 (mT18). A current review of mT18 symptomatology, long-term follow-up, and potential health risks is lacking for health care professionals. Our paper addresses these, emphasizing the importance of regular tumor screening as a key message for mT18 patient follow-up. We also present the case of a female patient with mT18 who presented with diaphragmatic relaxation and cyclic vomiting syndrome (CVS), which had previously not been reported in this genetic condition. On further investigating the etiology of CVS, we revealed a novel mitochondrial mutation in the MT-ND6 gene in heteroplasmic form. Based on the literature, we hypothesize that the mitochondrial mutation together with mT18 could result in CVS.
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Affiliation(s)
- Magdolna Kósa
- Department of Pediatrics and Pediatric Health Center, Albert Szent-Györgyi Health Centre, University of Szeged, Szeged, Hungary
| | - Emese Horváth
- Department of Medical Genetics, Albert Szent-Györgyi Health Centre, University of Szeged, Szeged, Hungary
| | - Tibor Kalmár
- Department of Pediatrics and Pediatric Health Center, Albert Szent-Györgyi Health Centre, University of Szeged, Szeged, Hungary
| | - Zoltán Maróti
- Department of Pediatrics and Pediatric Health Center, Albert Szent-Györgyi Health Centre, University of Szeged, Szeged, Hungary
| | - Imre Földesi
- Department of Laboratory Medicine, Albert Szent-Györgyi Health Centre, University of Szeged, Szeged, Hungary
| | - Csaba Bereczki
- Department of Pediatrics and Pediatric Health Center, Albert Szent-Györgyi Health Centre, University of Szeged, Szeged, Hungary
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McFee RB, Abdelsayed GG. Nausea and vomiting - General considerations. Dis Mon 2024; 70:101831. [PMID: 39627101 DOI: 10.1016/j.disamonth.2024.101831] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2024]
Affiliation(s)
- Robin B McFee
- Director, Ellis Medical Toxicology Consulting, USA; DCOM - Lincoln Memorial University, USA.
| | - George G Abdelsayed
- Associate Professor of Medicine, Rutgers-Robert Wood Johnson Medical School, USA; Director Ambulatory Hepatology, Chief Quality Officer - Division of Gastroenterology, USA
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Kolacz J. Autonomic assessment at the intersection of psychosocial and gastrointestinal health. Neurogastroenterol Motil 2024; 36:e14887. [PMID: 39118212 DOI: 10.1111/nmo.14887] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/05/2024] [Revised: 07/09/2024] [Accepted: 07/24/2024] [Indexed: 08/10/2024]
Abstract
BACKGROUND Wearable technology is increasingly used in clinical practice and research to monitor functional gastrointestinal symptoms and mental health. AIMS This article explores the potential of wearable sensors to enhance the understanding of the autonomic nervous system (ANS), particularly its role in linking psychological and gastrointestinal function. The ANS, facilitates brain-gut communication and is responsive to psychosocial conditions. It is implicated in disorders related to psychological stress and gut-brain interaction. Wearable technology enables tracking of the ANS in daily life, offering complementary and alternative methods from traditional lab-based measures. This review places focus on autonomic metrics such as respiratory sinus arrhythmia, vagal efficiency, and electrodermal activity as well as self-reports of autonomic symptoms. DISCUSSION Potential applications include use of wearable sensors for tracking autonomic activity in disorder of gut-brain interaction such as cyclic vomiting syndrome, in which ANS dysregulation may be triggered by psychosocial factors. Considerations for data interpretation and contextualization are addressed, acknowledging challenges such as situational confounders of ANS activity and accuracy of wearable devices.
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Affiliation(s)
- Jacek Kolacz
- Department of Psychiatry and Behavioral Health, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA
- Traumatic Stress Research Consortium (TSRC) at the Kinsey Institute, Indiana University, Bloomington, Indiana, USA
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Varisova AK, Svirava AM, Dudnikova EV, Badyan AS, Besedina EA, Chernova MS. Modern concepts about cyclic vomiting syndrome in children. ROSSIYSKIY VESTNIK PERINATOLOGII I PEDIATRII (RUSSIAN BULLETIN OF PERINATOLOGY AND PEDIATRICS) 2024; 69:117-126. [DOI: 10.21508/1027-4065-2024-69-2-117-126] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/05/2025]
Abstract
Despite the relatively low prevalence in the world and in Russia, in particular, of such a pathology as cyclic vomiting syndrome, the relevance of the problem is due to the lack of research and sufficient information about the etiology, pathogenesis, and most importantly about methods of treatment and prevention of the disease. This syndrome is typical for children aged 3 to 7 years and is manifested by repeated stereotypical episodes of vomiting, alternating with periods of complete well-being. Cyclic vomiting syndrome worsens the child’s quality of life and seriously affects their further development and socialization. The article provides an overview of scientific research on cyclic vomiting syndrome in children.
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Aydemir Y, Aydemir O, Dinleyici M, Saglik AC, Cam D, Kaya TB, Canpolat FE. Screening for functional gastrointestinal disorders in preterm infants up to 12 months of corrected age: a prospective cohort study. Eur J Pediatr 2024; 183:2091-2099. [PMID: 38347262 PMCID: PMC11035472 DOI: 10.1007/s00431-024-05451-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/14/2023] [Revised: 01/08/2024] [Accepted: 01/23/2024] [Indexed: 04/23/2024]
Abstract
Functional gastrointestinal disorders (FGIDs) are characterized by a variety of symptoms that are frequently age-dependent, chronic, or recurrent and are not explained by structural or biochemical abnormalities. There are studies in the literature reporting different results regarding the relationship between prematurity and FGIDs. The main objective of this study was to compare the frequency of FGIDs between preterm and term infants. The secondary objective was to evaluate whether there was any association between neonatal characteristics and development of FGIDs. A multicenter prospective cohort study that included preterm infants born before 37 weeks of gestation and healthy term infants was carried out. At 1, 2, 4, 6, 9, and 12 months of age, infants were assessed for the presence of FGIDs using the Rome IV criteria. In preterm infants, an additional follow-up visit was made at 12 months corrected age. 134 preterm and 104 term infants were enrolled in the study. Infantile colic, rumination syndrome, functional constipation, and infant dyschezia were more common in preterm infants. Incidence of other FGIDs (infant regurgitation, functional diarrhea and cyclic vomiting syndrome) were similar among preterm and term infants. Preterm infants who are exclusively breastfeed in the first 6 months of life have a lower incidence of infantile colic (18.8% vs 52.1%, p = 0.025). In terms of chronological age, FGIDs symptoms started later in preterm infants; this difference was statistically significant for infantile colic and regurgitation (median age 2 months vs 1 month, p < 0.001). Conclusions: Preterm infants have a higher prevalence of FGIDs compared with term controls. Therefore, especially if they have gastrointestinal complaints, they should be screened for FGIDs. Possibly due to maturational differences, the time of occurrence of FGIDs may differ in preterm infants. Infantile colic incidence decreases with exclusive breastfeeding. What is Known: • The functional gastrointestinal disorders are a very common in infancy. • Data on preterm infants with FGIDs are currently very limited. What is New: • Preterm infants have a higher incidence of infantile colic, rumination syndrome, functional constipation and infant dyschezia when compared to term infants. • Preterm infants who are exclusively breastfed during the first 6 months of life experience a lower incidence of infantile colic.
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Affiliation(s)
- Yusuf Aydemir
- Faculty of Medicine Department of Pediatrics, Division of Gastroenterology and Hepatology, Eskisehir Osmangazi University, Meselik, 26040, Eskisehir, Turkey.
| | - Ozge Aydemir
- Faculty of Medicine Department of Pediatrics, Division of Neonatology, Eskisehir Osmangazi University, Eskisehir, Turkey
| | - Meltem Dinleyici
- Faculty of Medicine Department of Pediatrics, Division of Social Pediatrics, Eskisehir Osmangazi University, Eskisehir, Turkey
| | - Adviye Cakil Saglik
- Faculty of Medicine Department of Pediatrics, Division of Neonatology, Eskisehir Osmangazi University, Eskisehir, Turkey
| | - Demet Cam
- Neonatal Intensive Care Unit, Dr. Zekai Tahir Burak Womens Health Research and Education Hospital, Ankara, Turkey
| | - Tugba Barsan Kaya
- Faculty of Medicine Department of Pediatrics, Division of Neonatology, Eskisehir Osmangazi University, Eskisehir, Turkey
| | - Fuat Emre Canpolat
- Department of Pediatrics, Division of Neonatology, University of Health Science Ankara Bilkent City Hospital, Ankara, Turkey
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15
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Falsaperla R, Scalia B, Collotta AD, Giacchi V, Cimino C, Ruggieri M. Treatment Options for Cyclic Vomiting Syndrome: A Real-World, Single-Center Experience with Systematic Literature Review and Meta-Analysis. J Clin Pharmacol 2024; 64:227-239. [PMID: 37867371 DOI: 10.1002/jcph.2374] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/27/2023] [Accepted: 10/10/2023] [Indexed: 10/24/2023]
Abstract
The optimal therapeutic management of cyclic vomiting syndrome (CVS) remains elusive. The objective of this study was to document our clinical experience in the Pediatric Department of San Marco Hospital and to survey the literature on pediatric CVS treatment, aiming to update the guidance on the most effective treatment strategies for this not-so-uncommon condition. Data from 70 patients with CVS, admitted to our Pediatric Department between September 2011 and December 2021, were aggregated and included in the study. A systematic review of the literature was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The quality of the included studies was assessed using the Quality Assessment of Diagnostic Accuracy Studies 2 (QUADAS-2) tool and the A Measurement Tool to Assess Systematic Reviews 2 (AMSTAR-2) method. Treatment responses, as observed both in the literature and in our own experience, are variable. In our cohort, topiramate demonstrated superiority over other pharmacological treatments, exhibiting an efficacy of 85% in the patients treated. A universally accepted treatment protocol for pediatric CVS has yet to be established. The efficacy of first-line treatments is generally suboptimal, suggesting that topiramate might serve as a safe and effective primary therapeutic option for pediatric CVS.
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Affiliation(s)
- Raffaele Falsaperla
- Neonatal Intensive Care Unit, Azienda Ospedaliero Universitaria Policlinico "G. Rodolico - San Marco", San Marco Hospital, Catania, Italy
- Unit of Pediatrics and Pediatric Emergency, Azienda Ospedaliero Universitaria Policlinico "G. Rodolico - San Marco", San Marco Hospital, Catania, Italy
- Department of Educational Science, Chair of Pediatrics, A.O. Policlinico, "G. Rodolico - San Marco", Catania, Italy
| | - Bruna Scalia
- Neonatal Intensive Care Unit, Azienda Ospedaliero Universitaria Policlinico "G. Rodolico - San Marco", San Marco Hospital, Catania, Italy
| | - Ausilia Desiree Collotta
- Unit of Pediatrics and Pediatric Emergency, Azienda Ospedaliero Universitaria Policlinico "G. Rodolico - San Marco", San Marco Hospital, Catania, Italy
| | - Valentina Giacchi
- Neonatal Intensive Care Unit, Azienda Ospedaliero Universitaria Policlinico "G. Rodolico - San Marco", San Marco Hospital, Catania, Italy
| | - Carla Cimino
- Neonatal Intensive Care Unit, Azienda Ospedaliero Universitaria Policlinico "G. Rodolico - San Marco", San Marco Hospital, Catania, Italy
| | - Martino Ruggieri
- Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry, University of Catania, Azienda Ospedaliero Universitaria Policlinico"G. Rodolico - San Marco", Catania, Italy
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16
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Niehaus AD, Cooper H, Lee CU. Mitochondrial HMG-CoA Synthase Deficiency: A Cyclic Vomiting Mimic Without Reliable Biochemical Markers. J Investig Med High Impact Case Rep 2024; 12:23247096241267154. [PMID: 39143735 PMCID: PMC11325301 DOI: 10.1177/23247096241267154] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/09/2024] [Revised: 06/14/2024] [Accepted: 06/22/2024] [Indexed: 08/16/2024] Open
Abstract
Here, we report an individual, eventually diagnosed with HMG-CoA synthase deficiency, who presented with a cyclic vomiting phenotype. HMG-CoA synthase deficiency is a rare disorder affecting ketone body synthesis in which affected individuals typically present at a young age with hypoketotic hypoglycemia, lethargy, encephalopathy, and hepatomegaly, usually triggered by catabolism (e.g., infection or prolonged fasting). This individual presented with recurrent episodes of vomiting and lethargy, often associated with hypoglycemia or hyperglycemia, at 3 years of age. Metabolic labs revealed nonspecific abnormalities in her urine organic acids (showing mild elevation of dicarboxylic acids with relatively low excretion of ketones) and a normal acylcarnitine profile. Given her clinical presentation, as well as a normal upper gastrointestinal series, esophagogastroduodenoscopy with biopsies, and abdominal ultrasound, she was diagnosed with cyclic vomiting syndrome at 3 years of age. Molecular testing completed at 7 years of age revealed a previously reported pathogenic sequence variant (c.1016+1G>A) and a novel likely pathogenic deletion (1.57 kB deletion, including exon 1) within HMGCS2 consistent with HMG-CoA synthase deficiency. This individual's presentation, mimicking cyclic vomiting syndrome, widens the clinical spectrum of HMG-CoA synthase deficiency. In addition, this case highlights the importance of molecular genetic testing in such presentations, as this rare disorder lacks specific metabolic markers.
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17
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Togha M, Babaei M, Jameie M. Good response to rectal diazepam in refractory cases of cyclic vomiting: A case-series and review of the literature. Clin Case Rep 2023; 11:e8109. [PMID: 38028097 PMCID: PMC10661318 DOI: 10.1002/ccr3.8109] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/29/2023] [Revised: 07/19/2023] [Accepted: 08/28/2023] [Indexed: 12/01/2023] Open
Abstract
Key Clinical Message Although increasing in number, cases of CVS are being frequently misdiagnosed and many are refractory to the available treatments. This paper draws attention to a timely consideration of this disorder upon suspicion and proposes rectal diazepam and cinnarizine as highly effective treatments in refractory cases of CVS. Abstract Cyclic vomiting syndrome (CVS) is a set of recurrent episodic attacks of nausea and vomiting. This is a migraine-related disorder that mostly affects children. Several medications have been recommended for abortive and prophylactic treatment. Unfortunately, in some cases, the treatment is not completely effective and affects the quality of life of the sufferer. In this paper, we report on two cases of children experiencing refractory CVS attacks who were not responsive to recommended medications for acute phase and prophylaxis. This account highlights the efficacy of rectal diazepam for the acute phase of CVS and cinnarizine, an anti-migraine and anti-histamine agent, for prophylaxis of further attacks.
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Affiliation(s)
- Mansoureh Togha
- Neurology department, Sina Hospital, School of MedicineTehran University of Medical SciencesTehranIran
- Headache department, Iranian Center of Neurological Research, Neuroscience InstituteTehran University of Medical SciencesTehranIran
| | - Mahsa Babaei
- Headache department, Iranian Center of Neurological Research, Neuroscience InstituteTehran University of Medical SciencesTehranIran
| | - Melika Jameie
- Headache department, Iranian Center of Neurological Research, Neuroscience InstituteTehran University of Medical SciencesTehranIran
- Iranian Center of Neurological Research, Neuroscience InstituteTehran University of Medical SciencesTehranIran
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18
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Caffarelli C, Santamaria F, Piro E, Basilicata S, D'Antonio L, Tchana B, Bernasconi S, Corsello G. Advances for pediatricians in 2022: allergy, anesthesiology, cardiology, dermatology, endocrinology, gastroenterology, genetics, global health, infectious diseases, metabolism, neonatology, neurology, oncology, pulmonology. Ital J Pediatr 2023; 49:115. [PMID: 37679850 PMCID: PMC10485969 DOI: 10.1186/s13052-023-01522-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/18/2023] [Accepted: 08/30/2023] [Indexed: 09/09/2023] Open
Abstract
The last year saw intensive efforts to advance knowledge in pediatric medicine. This review highlights important publications that have been issued in the Italian Journal of Pediatrics in 2022. We have chosen papers in the fields of allergy, anesthesiology, cardiology, dermatology, endocrinology, gastroenterology, genetics, global health, infectious diseases, metabolism, neonatology, neurology, oncology, pulmonology. Novel valuable developments in epidemiology, pathophysiology, prevention, diagnosis and treatment that can rapidly change the approach to diseases in childhood have been included and discussed.
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Affiliation(s)
- Carlo Caffarelli
- Clinica Pediatrica, Department of Medicine and Surgery, Azienda Ospedaliera- Universitaria, University of Parma, Parma, Italy.
| | - Francesca Santamaria
- Department of Translational Medical Sciences, Federico II University, Naples, Italy
| | - Ettore Piro
- Department of Sciences for Health Promotion and Mother and Child Care "G. D'Alessandro", University of Palermo, Palermo, Italy
| | - Simona Basilicata
- Department of Translational Medical Sciences, Federico II University, Naples, Italy
| | - Lorenzo D'Antonio
- Department of Translational Medical Sciences, Federico II University, Naples, Italy
| | - Bertrand Tchana
- Cardiologia Pediatrica, Azienda-Ospedaliero Universitaria, Parma, Italy
| | | | - Giovanni Corsello
- Department of Sciences for Health Promotion and Mother and Child Care "G. D'Alessandro", University of Palermo, Palermo, Italy
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19
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Thavamani A, Velayuthan S, Patel D, Al-Hammadi N, Sferra TJ, Sankararaman S. Association of Anxiety and Gastrointestinal Comorbidities in Repeat Hospital Admissions in Pediatric Cyclic Vomiting Syndrome. Am J Gastroenterol 2023; 118:1439-1445. [PMID: 37052354 DOI: 10.14309/ajg.0000000000002292] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/15/2022] [Accepted: 03/28/2023] [Indexed: 04/14/2023]
Abstract
INTRODUCTION Cyclic vomiting syndrome (CVS) is a functional gastrointestinal disorder with recurrent episodes of intense nausea and vomiting and thus may require frequent hospitalizations. There is paucity of data exploring the association of psychiatric and gastrointestinal comorbidities in repeat hospitalizations among pediatric patients with CVS. METHODS We analyzed the Pediatric Health Information System database and included all patients up to 18 years of age with a diagnosis of CVS between 2016 and 2020. We excluded patients with chronic conditions, which mimic CVS. The primary outcome variable was 90-day admission rate, which was defined as a visit to emergency department or admission to observation/inpatient unit with a primary diagnosis of CVS within 90 days after an index CVS hospitalization. RESULTS We evaluated a total of 2,604 hospitalizations represented by 1,370 unique individuals. The overall 90-day admission rate was 28.5%, which steadily decreased from 35.7% in 2016 to 23% in 2019 ( P < 0.001). Patients in the repeat hospitalization cohort were slightly older and more often men. Patients with repeat admissions had an increased proportion of anxiety and other gastrointestinal disorders. Multivariable logistic regression showed that anxiety, gastroesophageal reflux disease, functional dyspepsia, and abdominal migraine were associated with increased odds of repeat admissions. DISCUSSION Ninety-day admission rates in pediatric CVS are decreasing overall, although still contributing to significant healthcare expenditure. Anxiety and gastrointestinal comorbidities were associated with increased risk of repeat admissions. Further prospective studies are needed to better understand the complex interactions of these comorbidities and their management affecting the natural course of CVS.
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Affiliation(s)
- Aravind Thavamani
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, UH Rainbow Babies and Children's Hospital, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
| | - Sujithra Velayuthan
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, UH Rainbow Babies and Children's Hospital, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
| | - Dhiren Patel
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, SSM Health Cardinal Glennon Children's Medical Center, Saint Louis University School of Medicine, St. Louis, Missouri, USA
| | - Noor Al-Hammadi
- The Advanced HEAlth Data (AHEAD) Research Institute, Saint Louis University School of Medicine, St. Louis, Missouri, USA
| | - Thomas J Sferra
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, UH Rainbow Babies and Children's Hospital, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
| | - Senthilkumar Sankararaman
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, UH Rainbow Babies and Children's Hospital, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
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20
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Karrento K, Zhang L, Conley W, Qazi Z, Venkatesan T, Simpson P, Li BU. Percutaneous electrical nerve field stimulation improves comorbidities in children with cyclic vomiting syndrome. FRONTIERS IN PAIN RESEARCH 2023; 4:1203541. [PMID: 37389229 PMCID: PMC10300638 DOI: 10.3389/fpain.2023.1203541] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/10/2023] [Accepted: 05/23/2023] [Indexed: 07/01/2023] Open
Abstract
Introduction Children with cyclic vomiting syndrome (CVS) frequently suffer from disabling abdominal pain and comorbidities that impair quality of life. A noninvasive, auricular percutaneous electrical nerve field stimulation (PENFS) device is shown to be effective for abdominal pain in children with disorders of gut-brain interaction. We aimed to determine the effects of PENFS on pain, common comorbidities, and quality of life in pediatric CVS. Methods Children aged 8-18 years with drug-refractory CVS were enrolled in a prospective, open-label study receiving 6 consecutive weeks of PENFS. Subjects completed the following surveys at baseline, during/after therapy (week 6), and at extended follow-up approximately 4-6 months later: Abdominal Pain Index (API), State-Trait Anxiety Inventory for Children (STAI-C), Pittsburgh Sleep Quality Index (PSQI), and Patient Reported Outcome Measurement Information System (PROMIS) Pediatric Profile-37. Results Thirty subjects were included. Median (interquartile range, IQR) age was 10.5 (8.5-15.5) years; 60% were female. Median API scores decreased from baseline to week 6 (p = 0.003) and to extended follow-up (p < 0.0001). State anxiety scores decreased from baseline to week 6 (p < 0.0001) and to extended follow-up (p < 0.0001). There were short-term improvements in sleep at 6 weeks (p = 0.031) but not at extended follow-up (p = 0.22). Quality of life measures of physical function, anxiety, fatigue, and pain interference improved short-term, while there were long-term benefits for anxiety. No serious side effects were reported. Conclusions This is the first study to demonstrate the efficacy of auricular neurostimulation using PENFS for pain and several disabling comorbidities in pediatric CVS. PENFS improves anxiety, sleep, and several aspects of quality of life with long-term benefits for anxiety.Clinical trial registration: ClinicalTrials.gov, identifier NCT03434652.
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Affiliation(s)
- Katja Karrento
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, United States
| | - Liyun Zhang
- Division of Quantitative Health Sciences, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, United States
| | - William Conley
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, United States
| | - Zeeshan Qazi
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, United States
| | - Thangam Venkatesan
- Division of Gastroenterology, The Ohio State University, Columbus, OH, United States
| | - Pippa Simpson
- Division of Quantitative Health Sciences, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, United States
| | - B U.K. Li
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, United States
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21
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Gosalvez-Tejada A, Li BUK, Simpson P, Zhang L, Kovacic K. Natural History of Pediatric Cyclic Vomiting Syndrome: Progression to Dysautonomia. J Pediatr Gastroenterol Nutr 2023; 76:737-742. [PMID: 36800281 DOI: 10.1097/mpg.0000000000003738] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/18/2023]
Abstract
BACKGROUND The clinical features of pediatric cyclic vomiting syndrome (CVS) often evolve over time. Many patients develop a constellation of chronic symptoms that suggest autonomic nervous system (ANS) dysfunction during adolescence. We aimed to determine the proportion of children with CVS who develop chronic rather than episodic symptoms consistent with ANS dysfunction. METHODS Retrospective chart review of children ages 0-18 years followed in an outpatient tertiary care CVS center. Patients completed standardized questionnaires at intake and follow-up visits, documenting clinical symptom pattern. Continuous variables are summarized as median [interquartile range (IQR)]. A Mann-Whitney test was used for group comparisons. RESULTS One hundred subjects were included. A total of 40% developed symptoms of ANS dysfunction (ANS+); 20% were confirmed by comprehensive ANS testing, 11% by orthostatic vital sign abnormalities, and 9% by clinical symptoms. The median (IQR) age at onset of chronic symptoms was 14 (10.02, 15) years. The presence of another disorder of gut-brain interaction ( P = 0.018) and a greater number of comorbidities ( P = 0.031) were more common in the ANS+ group. ANS+ subjects missed more school days ( P = 0.047) and were seen less frequently in the emergency department ( P = 0.023). CONCLUSIONS Many children with CVS (40%) develop symptoms consistent with clinical dysautonomia in adolescence. These patients experience more comorbid conditions and a greater impact on school attendance, possibly representing a worsened quality of life as their disease course transitions to daily symptoms. When symptoms of CVS change over time, therapeutic interventions may need to be adjusted and targeted accordingly.
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Affiliation(s)
- Andrea Gosalvez-Tejada
- From the Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI
| | - B U K Li
- From the Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI
| | - Pippa Simpson
- the Division of Quantitative Health Sciences, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI
| | - Liyun Zhang
- the Division of Quantitative Health Sciences, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI
| | - Katja Kovacic
- From the Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI
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22
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Bar O, Ebenau L, Weiner K, Mintz M, Boles RG. Whole exome/genome sequencing in cyclic vomiting syndrome reveals multiple candidate genes, suggesting a model of elevated intracellular cations and mitochondrial dysfunction. Front Neurol 2023; 14:1151835. [PMID: 37234784 PMCID: PMC10208274 DOI: 10.3389/fneur.2023.1151835] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/26/2023] [Accepted: 04/03/2023] [Indexed: 05/28/2023] Open
Abstract
OBJECTIVE To utilize whole exome or genome sequencing and the scientific literature for identifying candidate genes for cyclic vomiting syndrome (CVS), an idiopathic migraine variant with paroxysmal nausea and vomiting. METHODS A retrospective chart review of 80 unrelated participants, ascertained by a quaternary care CVS specialist, was conducted. Genes associated with paroxysmal symptoms were identified querying the literature for genes associated with dominant cases of intermittent vomiting or both discomfort and disability; among which the raw genetic sequence was reviewed. "Qualifying" variants were defined as coding, rare, and conserved. Additionally, "Key Qualifying" variants were Pathogenic/Likely Pathogenic, or "Clinical" based upon the presence of a corresponding diagnosis. Candidate association to CVS was based on a point system. RESULTS Thirty-five paroxysmal genes were identified per the literature review. Among these, 12 genes were scored as "Highly likely" (SCN4A, CACNA1A, CACNA1S, RYR2, TRAP1, MEFV) or "Likely" (SCN9A, TNFRSF1A, POLG, SCN10A, POGZ, TRPA1) CVS related. Nine additional genes (OTC, ATP1A3, ATP1A2, GFAP, SLC2A1, TUBB3, PPM1D, CHAMP1, HMBS) had sufficient evidence in the literature but not from our study participants. Candidate status for mitochondrial DNA was confirmed by the literature and our study data. Among the above-listed 22 CVS candidate genes, a Key Qualifying variant was identified in 31/80 (34%), and any Qualifying variant was present in 61/80 (76%) of participants. These findings were highly statistically significant (p < 0.0001, p = 0.004, respectively) compared to an alternative hypothesis/control group regarding brain neurotransmitter receptor genes. Additional, post-analyses, less-intensive review of all genes (exome) outside our paroxysmal genes identified 13 additional genes as "Possibly" CVS related. CONCLUSION All 22 CVS candidate genes are associated with either cation transport or energy metabolism (14 directly, 8 indirectly). Our findings suggest a cellular model in which aberrant ion gradients lead to mitochondrial dysfunction, or vice versa, in a pathogenic vicious cycle of cellular hyperexcitability. Among the non-paroxysmal genes identified, 5 are known causes of peripheral neuropathy. Our model is consistent with multiple current hypotheses of CVS.
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Affiliation(s)
- Omri Bar
- NeurAbilities Healthcare, Voorhees, NJ, United States
| | - Laurie Ebenau
- NeurAbilities Healthcare, Voorhees, NJ, United States
| | - Kellee Weiner
- NeurAbilities Healthcare, Voorhees, NJ, United States
| | - Mark Mintz
- NeurAbilities Healthcare, Voorhees, NJ, United States
| | - Richard G. Boles
- NeurAbilities Healthcare, Voorhees, NJ, United States
- NeuroNeeds, Old Lyme, CT, United States
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23
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Ogata M, Shimizu D, Ichikawa S, Araki S, Esumi G, Yamamoto J, Kusuhara K. Early diagnosis of neonatal-onset cyclic vomiting syndrome. NAGOYA JOURNAL OF MEDICAL SCIENCE 2023; 85:357-361. [PMID: 37346830 PMCID: PMC10281848 DOI: 10.18999/nagjms.85.2.357] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 01/31/2022] [Accepted: 05/09/2022] [Indexed: 06/23/2023]
Abstract
Cyclic vomiting syndrome (CVS) is characterized by recurrent episodes of severe vomiting with a completely asymptomatic interictal interval. Relatively few patients develop CVS in the neonatal period, and an early diagnosis is difficult. We experienced an infant who was diagnosed with neonatal-onset CVS in early infancy. An 8-day-old girl was admitted to our neonatal intensive care unit because of frequent vomiting beginning 12 h after birth and weight loss reaching 84.2% of her birth weight. Despite extensive examinations, no abnormalities to explain the vomiting were found. She continued to vomit, and a cyclical pattern with a vomiting phase lasting for three days followed by a non-vomiting phase lasting for about one to two weeks became obvious. Based on her clinical course, the family history of migraine and the effectiveness of Phenobarbital, she was diagnosed with CVS at three months old. Although CVS is a diagnosis of exclusion, a family history of migraine can aid its early diagnosis. If the illness is suspected in the neonatal period, diagnostic treatment with Phenobarbital may be considered. The case suggests the need to include CVS in the differential diagnosis of neonates with unexplained repetitive vomiting.
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Affiliation(s)
- Manami Ogata
- Department of Pediatrics, School of Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan
| | - Daisuke Shimizu
- Department of Pediatrics, School of Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan
| | - Shun Ichikawa
- Department of Pediatrics, School of Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan
| | - Shunsuke Araki
- Department of Pediatrics, School of Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan
| | - Genshiro Esumi
- Department of Pediatric Surgery, School of Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan
| | - Junko Yamamoto
- Department of Pediatrics, Japan Community Healthcare Organization Kyushu Hospital, Kitakyushu, Japan
| | - Koichi Kusuhara
- Department of Pediatrics, School of Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan
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Lathrop JR, Rosen SN, Heitkemper MM, Buchanan DT. Cyclic Vomiting Syndrome and Cannabis Hyperemesis Syndrome: The State of the Science. Gastroenterol Nurs 2023; 46:208-224. [PMID: 37074964 DOI: 10.1097/sga.0000000000000730] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/08/2022] [Accepted: 10/10/2022] [Indexed: 04/20/2023] Open
Abstract
This article provides a narrative review of the state of the science for both cyclic vomiting syndrome and cannabis hyperemesis syndrome along with a discussion of the relationship between these 2 conditions. The scope of this review includes the historical context of these conditions as well as the prevalence, diagnostic criteria, pathogenesis, and treatment strategies for both conditions. A synopsis of the endocannabinoid system provides a basis for the hypothesis that a lack of cannabidiol in modern high-potency Δ 9 -tetrahydrocannabinol cannabis may be contributory to cannabis hyperemesis syndrome and possibly other cannabis use disorders. In concluding assessment, though the publications addressing both adult cyclic vomiting syndrome and cannabis hyperemesis syndrome are steadily increasing overall, the state of the science supporting the treatments, prognosis, etiology, and confounding factors (including cannabis use) is of moderate quality. Much of the literature portrays these conditions separately and as such sometimes fails to account for the confounding of adult cyclic vomiting syndrome with cannabis hyperemesis syndrome. The diagnostic and therapeutic approaches are, at present, based generally on case series publications and expert opinion, with a very limited number of randomized controlled trials and a complete absence of Level 1 evidence within the cyclic vomiting literature overall as well as for cannabis hyperemesis syndrome specifically.
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Affiliation(s)
- James R Lathrop
- James R. Lathrop, DNP, FNP, ARNP, is a PhD student under the Department of Biobehavioral Nursing & Health Informatics, School of Nursing, University of Washington, Seattle
- Sheldon N. Rosen, MD, is Clinical Associate Professor, Division of Gastroenterology, School of Medicine, University of Washington, Seattle
- Margaret M. Heitkemper, PhD, RN, FAAN, is Professor and Elizabeth Sterling Soule Endowed Chair in Nursing, Department of Biobehavioral Nursing & Health Informatics, School of Nursing, University of Washington, Seattle
- Diana Taibi Buchanan, PhD, RN, is Associate Professor and Mary S. Tschudin Endowed Professor of Nursing Education, Department of Biobehavioral Nursing & Health Informatics, School of Nursing, University of Washington, Seattle
| | - Sheldon N Rosen
- James R. Lathrop, DNP, FNP, ARNP, is a PhD student under the Department of Biobehavioral Nursing & Health Informatics, School of Nursing, University of Washington, Seattle
- Sheldon N. Rosen, MD, is Clinical Associate Professor, Division of Gastroenterology, School of Medicine, University of Washington, Seattle
- Margaret M. Heitkemper, PhD, RN, FAAN, is Professor and Elizabeth Sterling Soule Endowed Chair in Nursing, Department of Biobehavioral Nursing & Health Informatics, School of Nursing, University of Washington, Seattle
- Diana Taibi Buchanan, PhD, RN, is Associate Professor and Mary S. Tschudin Endowed Professor of Nursing Education, Department of Biobehavioral Nursing & Health Informatics, School of Nursing, University of Washington, Seattle
| | - Margaret M Heitkemper
- James R. Lathrop, DNP, FNP, ARNP, is a PhD student under the Department of Biobehavioral Nursing & Health Informatics, School of Nursing, University of Washington, Seattle
- Sheldon N. Rosen, MD, is Clinical Associate Professor, Division of Gastroenterology, School of Medicine, University of Washington, Seattle
- Margaret M. Heitkemper, PhD, RN, FAAN, is Professor and Elizabeth Sterling Soule Endowed Chair in Nursing, Department of Biobehavioral Nursing & Health Informatics, School of Nursing, University of Washington, Seattle
- Diana Taibi Buchanan, PhD, RN, is Associate Professor and Mary S. Tschudin Endowed Professor of Nursing Education, Department of Biobehavioral Nursing & Health Informatics, School of Nursing, University of Washington, Seattle
| | - Diana Taibi Buchanan
- James R. Lathrop, DNP, FNP, ARNP, is a PhD student under the Department of Biobehavioral Nursing & Health Informatics, School of Nursing, University of Washington, Seattle
- Sheldon N. Rosen, MD, is Clinical Associate Professor, Division of Gastroenterology, School of Medicine, University of Washington, Seattle
- Margaret M. Heitkemper, PhD, RN, FAAN, is Professor and Elizabeth Sterling Soule Endowed Chair in Nursing, Department of Biobehavioral Nursing & Health Informatics, School of Nursing, University of Washington, Seattle
- Diana Taibi Buchanan, PhD, RN, is Associate Professor and Mary S. Tschudin Endowed Professor of Nursing Education, Department of Biobehavioral Nursing & Health Informatics, School of Nursing, University of Washington, Seattle
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Redon S, Donnet A. Cyclic vomiting syndrome, a common language? A 20-year bibliometric study. Acta Neurol Belg 2023:10.1007/s13760-023-02223-z. [PMID: 36988834 DOI: 10.1007/s13760-023-02223-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/06/2022] [Accepted: 02/21/2023] [Indexed: 03/30/2023]
Abstract
BACKGROUND Cyclic vomiting syndrome (CVS) is recognized as an episodic syndrome associated with migraine in the last version of the International Classification of Headache Disorders (ICHD-3). It manifests as stereotypical episodes of intense nausea and vomiting, occurring preferentially in childhood. Over the last 2 decades, the knowledge of this disorder has increased. The diagnostic criteria have been modified, through the evolution of several successive classifications. Actually, two classifications are prominent in the literature: the ICHD-3 and the Rome IV classification. The predictable periodicity of episodes is only recognized in the ICHD-3. OBJECTIVE We aimed to analyze the evolution of CVS literature in the last 2 decades, with a focus on CVS criteria used in these papers. METHODS We conducted a bibliometric study. We searched in the Web of Science database all papers in English literature with the term CVS in the abstract or title, in the category "article" or "review", published from 2001 to 2020. We searched within the paper which classification was used or mentioned. RESULTS In total, 213 papers were analyzed. 116 papers exclusively concerned childhood and adolescence CVS, or were written by pediatric practitioners. For most of the papers, the corresponding author was specialized in the field of gastroenterology. The Rome III classification was the main classification used or mentioned. The ICHD-3 and its beta version were mostly used or mentioned by the authors affiliated to the neurologic field. CONCLUSION This study shows the growth in the number of publications on CVS. It highlights the lack of reference to the ICHD, in particular by practitioners in the field of gastroduodenal disorders. This should encourage the achievement of a common classification with the different scientific societies.
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Affiliation(s)
- Sylvain Redon
- Department of Evaluation and Treatment of Pain, FHU INOVPAIN, CHU Timone, AP-HM, Marseille, France.
| | - Anne Donnet
- Department of Evaluation and Treatment of Pain, FHU INOVPAIN, CHU Timone, AP-HM, Marseille, France
- INSERM U-1107, CHU de Clermont-Ferrand, Clermont-Ferrand, France
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Cardiac Vagal Regulation Is Impeded in Children With Cyclic Vomiting Syndrome. Am J Gastroenterol 2023:00000434-990000000-00668. [PMID: 36716443 DOI: 10.14309/ajg.0000000000002207] [Citation(s) in RCA: 8] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/23/2022] [Accepted: 01/19/2023] [Indexed: 02/01/2023]
Abstract
INTRODUCTION The pathophysiology underlying cyclic vomiting syndrome (CVS) remains undefined. Scant data and distinct clinical features point to altered autonomic nervous system function. Autonomic signaling can be noninvasively assessed through cardiac indices of parasympathetic vagal regulation, which is reduced in children with disorders of gut-brain interaction. We aimed to examine dynamic cardiac vagal regulation in children with CVS compared with that in healthy controls (HC). METHODS A total of 31 children with CVS evaluated in a tertiary care CVS center and 66 HC (ages 8-18 years) underwent cardiac autonomic function assessment. Electrocardiogram recordings were conducted during 3-minute sit/stand/sit posture challenges. The electrocardiogram-derived variables heart period, respiratory sinus arrhythmia (RSA), and vagal efficiency (VE) were analyzed using linear regression and mixed-effects modeling. RESULTS After exclusion of medication confounders, 23 patients with CVS were included in analyses. Both groups were comparable in age, gender, and body mass index. Compared with HC, children with CVS had shorter heart period (standardized mean difference range: 1.15-1.22, all P values < 0.05) and lower RSA (SMD range: 0.66-0.88, all P values < 0.05). Patients with CVS had significantly lower VE during the entire course of posture shifts, compared with HC ( B = -19.87, SE = 6.95, t = -2.86, P = 0.005, SMD = 0.76). DISCUSSION Children with CVS have suboptimal parasympathetic autonomic regulation compared with HC, indexed by reduced RSA and VE, even during their interepisodic well phase. Abnormal vagal modulation may underlie CVS pathophysiology, comorbidities, and triggers. Assessing VE during posture stressors could inform therapeutic interventions.
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Haghighat M, Gholami Shahrebabak M, Dehghani SM, Ataollahi M, Amin Farzaneh N, Hamzeloo Hoseinabadi S, Javaherizadeh H. Relapse Rate of Clinical Symptoms After Stopping Treatment in Children with Cyclic Vomiting Syndrome. Middle East J Dig Dis 2023; 15:32-36. [PMID: 37547164 PMCID: PMC10404072 DOI: 10.34172/mejdd.2023.317] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/10/2022] [Accepted: 11/15/2022] [Indexed: 08/08/2023] Open
Abstract
Background: Cyclic vomiting syndrome (CVS) is a chronic functional gastrointestinal disorder. It is characterized by recurrent episodes of vomiting typically separated by periods of symptom-free or baseline health. The present study aimed at evaluating the effectiveness of propranolol and the relapse rate of clinical symptoms after stopping treatment in children suffering from CVS. Methods: Records of 504 patients below the age of 18 years with CVS who were treated with propranolol from March 2008 to March 2018 were reviewed. The duration of follow-up was 10 years. Results: The average age of CVS affliction was 4.3 years and the average age at the diagnosis was 5.8 years. All subjects were treated with propranolol (for an average of 10 months). 92% of treated subjects were cured, causing a dramatic decrease in the rate of vomiting (P < 0.001). Only an average of 10.5% of the studied subjects (53 people) showed a relapse of symptoms after stopping the treatment. The results of a 10-year follow-up period of the patients showed that 24 had abdominal migraine and 6 had migraine headaches, all of whom lacked the symptoms of disease relapse (prognostic evaluation). Conclusion: The findings of this investigation show that the duration of treating CVS with propranolol could be shortened to 10 months with a low percent of symptoms relapse and this shortening may be effective in preventing the undesirable side effects of the drug. The presence of abdominal migraine and migraine headaches in patients after treatment accomplishment and the lack of disease relapse can be prognostic measures for this disease, which require intensive attention.
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Affiliation(s)
- Mahmoud Haghighat
- Gastroenterohepatology Research Center, Nemazee Teaching Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Maryam Gholami Shahrebabak
- Gastroenterohepatology Research Center, Nemazee Teaching Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Seyed Mohsen Dehghani
- Gastroenterohepatology Research Center, Nemazee Teaching Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Maryam Ataollahi
- Gastroenterohepatology Research Center, Nemazee Teaching Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Nazanin Amin Farzaneh
- Gastroenterohepatology Research Center, Nemazee Teaching Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Samaneh Hamzeloo Hoseinabadi
- Gastroenterohepatology Research Center, Nemazee Teaching Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Hazhir Javaherizadeh
- Clinical Sciences Research Institute, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran
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Prevalence and incidence of cyclic vomiting syndrome in Japan: A study using Japanese claims data. PLoS One 2022; 17:e0279502. [PMID: 36548340 PMCID: PMC9778604 DOI: 10.1371/journal.pone.0279502] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/06/2022] [Accepted: 12/08/2022] [Indexed: 12/24/2022] Open
Abstract
Cyclic vomiting syndrome (CVS) is a gastrointestinal disorder that is characterized by recurrent episodes of vomiting. Previous studies have provided reliable data on the prevalence of CVS among children in Japan; however, neither prevalence data nor incidence of CVS is available for adults. Hence, we obtained detailed prevalence and incidence data for CVS and estimated the total number of CVS cases in Japan. This retrospective cross-sectional study was conducted using the JMDC (JMDC, Inc.; formerly known as Japan Medical Data Center Co., Ltd.,) database, which is a de-identified Japanese claims database. Individuals enrolled between January 2017 and December 2017 were included in this study. Longitudinal data for eligible populations were used to identify patients who displayed CVS symptoms throughout the follow-up period. Due to the lack of a specific diagnosis code for CVS in 2017, the Rome IV criteria were applied to identify CVS cases in the pediatric and adult populations. The prevalence was standardized to the 2017 Japanese census and extrapolated to estimate the number of CVS cases. A total of 2,093 patients with CVS were identified from 3,506,144 individuals. The overall age-and-sex-standardized prevalence was 0.32 per 1,000 population (95% confidence interval [CI]: 0.30-0.34), projected to approximately 49,000 patients with CVS across Japan. The pediatric age-and-sex-standardized prevalence was 2.10 per 1,000 population (95% CI: 2.01-2.19), and the adult prevalence was 0.05 per 1,000 population (95% CI: 0.04-0.06). Marked sex differences were observed before and after 12 years of age. Thus our study provides the first large-population-based estimates of CVS prevalence and incidence in Japan, and currently, the only estimates for adult CVS in Japan.
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Frazier R, Venkatesan T. Current understanding of the etiology of cyclic vomiting syndrome and therapeutic strategies in its management. Expert Rev Clin Pharmacol 2022; 15:1305-1316. [PMID: 36259482 DOI: 10.1080/17512433.2022.2138341] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022]
Abstract
INTRODUCTION Cyclic vomiting syndrome is a chronic debilitating disorder of the gut-brain interaction and is characterized by recurrent episodes of nausea and vomiting.Recent studies indicate that it is common and affects 2% of the US population. Unfortunately, there is significant heterogeneity in the management of these patients in the medical community. This review article aims to bridge this gap and will review the epidemiology and etiology with a focus on management of CVS. AREAS COVERED This article reviews the epidemiology, and pathophysiology of CVS and impact on patients. It also discusses management based on recent guidelines based on which is intended for the busy clinician. A literature search was done using PubMed and key words "cyclic vomiting", "management", "etiology", and pathophysiology were used to identify articles of importance. EXPERT OPINION CVS is a complex, poorly understood disorder of gut-brain interaction (DGBI) and has a significant negative impact on patients, families and the healthcare system. Recent guidelines recommend a multidisciplinary approach to management using prophylactic therapy in moderate-severe CVS and abortive medication for acute flares. However more research is needed to better understand the pathophysiology and develop targeted therapies for CVS.
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Affiliation(s)
- Rosita Frazier
- Gastroenterology and Hepatology, Mayo Clinic, Scottsdale, Arizona
| | - Thangam Venkatesan
- Division of Gastroenterology, Hepatology and Nutrition, The Ohio State University, Columbus, OH
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Beals L, Sarjinsky S, Faltyn M, Issenman RM, Kam AJ. Cyclic Vomiting Syndrome in the Emergency Department: A 10-Year Review of Clinical Presentation and Management. Pediatr Emerg Care 2022; 38:e1578-e1583. [PMID: 35560301 DOI: 10.1097/pec.0000000000002694] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
OBJECTIVES The aims of the study were (1) to determine how frequently patients with cyclic vomiting syndrome (CVS) present to the pediatric emergency department (ED) with CVS-related symptoms, (2) to identify variables in clinical presentation that occur frequently in patients with multiple ED visits, and (3) to compare ED management of CVS with recommended guidelines. METHODS This study is a retrospective chart review of all ED visits for CVS between April 1, 2008, and April 1, 2018, at a single center. Patients were identified from a master list of patients diagnosed with CVS in a pediatric gastroenterology clinic at the same center between June 1, 2004, and June 19, 2018. RESULTS Of the 181 CVS clinic patients identified, 65 had visited to the ED (35.9%). Two hundred twenty-eight visits met inclusion criteria. A total of 42.5% of these visits were made by a small number of high-intensity patients (n = 6) who had an average of 16.1 visits each. These patients represented less than 10% of the total patient group. Patients with frequent visits had longer visits (536.52 vs 380.55 minutes), more frequent hospital admissions (57.73% vs 29.01%), and more visits to the ED before a formal diagnosis of CVS was made (5.83 vs 1.22), but few other distinguishing characteristics. Only 27% of eligible visits were managed with an available order set, and management varied from recommended guidelines. CONCLUSIONS An ambiguous presentation makes the identification and consistent management of CVS in the ED difficult. Physicians should consider CVS for patients who present multiple times with unremitting vomiting to ensure appropriate referral for diagnosis and prophylactic treatment. Future studies are warranted to evaluate anticipatory processing and treatment of the "high-intensity patients" who account for much of the clinical morbidity and resource utilization.
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Affiliation(s)
- Lauren Beals
- From the McMaster Division of Emergency Medicine, FRCPC Residency Program
| | | | - Mateusz Faltyn
- McMaster University Arts & Science Undergraduate Program
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Isoldi S, Di Nardo G, Mallardo S, Parisi P, Raucci U, Tambucci R, Quitadamo P, Salvatore S, Felici E, Cisarò F, Pensabene L, Banzato C, Strisciuglio C, Romano C, Fusco P, Rigotti F, Sansotta N, Caimmi S, Savasta S, Zuin G, Di Stefano M, Provera S, Campanozzi A, Rossi P, Gatti S, Corpino M, Alvisi P, Martelossi S, Suppiej A, Gandullia P, Verrotti A, Terrin G, Pacenza C, Fornaroli F, Comito D, D'Arrigo S, Striano P, Raviglione F, Carotenuto M, Orsini A, Belcastro V, Di Corcia G, Raieli V, Ferilli MAN, Ruscitto C, Spadoni E, Grosso S, D'Alonzo R, Papa A, Pavone P, Meli M, Velardita M, Mainetti M, Vanacore N, Borrelli O. Cyclic vomiting syndrome in children: a nationwide survey of current practice on behalf of the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP) and Italian Society of Pediatric Neurology (SINP). Ital J Pediatr 2022; 48:156. [PMID: 36042474 PMCID: PMC9429644 DOI: 10.1186/s13052-022-01346-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/16/2022] [Accepted: 08/10/2022] [Indexed: 11/23/2022] Open
Abstract
BACKGROUND Cyclic Vomiting Syndrome (CVS) is a rare functional gastrointestinal disorder, which has a considerable burden on quality of life of both children and their family. Aim of the study was to evaluate the diagnostic modalities and therapeutic approach to CVS among Italian tertiary care centers and the differences according to subspecialties, as well as to explore whether potential predictive factors associated with either a poor outcome or a response to a specific treatment. METHODS Cross-sectional multicenter web-based survey involving members of the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP) and Italian Society of Pediatric Neurology (SINP). RESULTS A total of 67 responses were received and analyzed. Most of the respondent units cared for less than 20 patients. More than half of the patients were referred after 3 to 5 episodes, and a quarter after 5 attacks. We report different diagnostic approaches among Italian clinicians, which was particularly evident when comparing gastroenterologists and neurologists. Moreover, our survey demonstrated a predilection of certain drugs during emetic phase according to specific clinic, which reflects the cultural background of physicians. CONCLUSION In conclusion, our survey highlights poor consensus amongst clinicians in our country in the diagnosis and the management of children with CVS, raising the need for a national consensus guideline in order to standardize the practice.
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Affiliation(s)
- Sara Isoldi
- Maternal and Child Health Department, Santa Maria Goretti Hospital, Sapienza-University of Rome, Latina, Italy
| | - Giovanni Di Nardo
- Department of Neuroscience, Mental Health and Sense Organs (NESMOS), Faculty of Medicine & Psychology, Sant'Andrea Hospital, Sapienza University of Rome, 00189, Rome, Lazio, Italy
| | - Saverio Mallardo
- Maternal and Child Health Department, Santa Maria Goretti Hospital, Sapienza-University of Rome, Latina, Italy
| | - Pasquale Parisi
- Department of Neuroscience, Mental Health and Sense Organs (NESMOS), Faculty of Medicine & Psychology, Sant'Andrea Hospital, Sapienza University of Rome, 00189, Rome, Lazio, Italy.
| | - Umberto Raucci
- Pediatric Emergency Department, Bambino Gesù Children's Hospital, Institute for Research, Hospitalization and Health Care (IRCCS), Rome, Italy
| | - Renato Tambucci
- Digestive Endoscopy and Surgery Unit, Bambino Gesù Children's Hospital, Institute for Research, Hospitalization and Health Care (IRCCS), Rome, Italy
| | - Paolo Quitadamo
- Department of Pediatrics, A.O.R.N. Santobono-Pausilipon, Naples, Italy
| | - Silvia Salvatore
- Pediatric Department, Ospedale "F. Del Ponte", University of Insubria, Varese, Italy
| | - Enrico Felici
- Unit of Pediatrics, The Children Hospital, Azienda Ospedaliera SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy
| | - Fabio Cisarò
- Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital, Azienda Ospedaliera-Universitaria, Città della Salute e della Scienza di Torino, Turin, Italy
| | - Licia Pensabene
- Pediatric Unit, Department of Medical and Surgical Sciences, University "Magna Graecia" of Catanzaro, Catanzaro, Italy
| | - Claudia Banzato
- Department of Surgical Sciences, Dentistry, Gynecology and Pediatrics, Pediatric Division, University of Verona, Verona, Italy
| | - Caterina Strisciuglio
- Department of Woman, Child, General and Specialistic Surgery, University of Campania "Luigi Vanvitelli", Naples, Italy
| | - Claudio Romano
- Pediatric Gastroenterology Unit, Department of Human Pathology in Adulthood and Childhood "G. Barresi", University of Messina, Messina, Italy
| | - Patrizia Fusco
- Pediatric Unit, Montis Regalis Hospital, Mondovì, Cuneo, Italy
| | | | - Naire Sansotta
- Paediatric Hepatology, Gastroenterology and Transplantation, Hospital Papa Giovanni XXIII, Bergamo, Italy
| | - Silvia Caimmi
- Pediatric Clinic, Foundation IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
| | - Salvatore Savasta
- Pediatric Clinic, Foundation IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
| | - Giovanna Zuin
- Pediatric Department, University of Milano Bicocca, FMBBM, San Gerardo Hospital, Monza, Italy
| | - Marina Di Stefano
- Department of Pediatrics, IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Silvia Provera
- Department of Pediatrics, IRCCS Sacro Cuore Don Calabria Hospital, Negrar, Verona, Italy
| | - Angelo Campanozzi
- Department of Medical and Surgical Sciences, Pediatric Unit, University of Foggia, Foggia, Italy
| | - Paolo Rossi
- Department of Pediatrics, Pediatric Gastroenterology and Liver Unit, Sapienza University of Rome, Rome, Italy
| | - Simona Gatti
- Department of Pediatrics, Università Politecnica delle Marche, Ancona, Italy
| | - Mara Corpino
- Gastroenterologia Pediatrica, Clinica Pediatrica e Malattie Rare, Ospedale Pediatrico Microcitemico A. Cao, ARNAS Brotzu, Cagliari, Italy
| | - Patrizia Alvisi
- Department of Paediatrics, Ospedale Maggiore, Azienda USL, Bologna, Italy
| | | | - Agnese Suppiej
- Pediatric Section, Department of Medical Sciences, University of Ferrara, Ferrara, Italy
| | - Paolo Gandullia
- UOC Gastroenterologia, IRCCS Istituto Giannina Gaslini, Genoa, Italy
| | | | - Gianluca Terrin
- Department of Maternal and Child Health, Policlinico Umberto I, Sapienza University of Rome, Rome, Italy
| | - Caterina Pacenza
- Department of Pediatrics, San Giovanni di Dio Hospital, Crotone, Italy
| | - Fabiola Fornaroli
- Gastroenterology and Endoscopy Unit, Department of Medicine and Surgery, University of Parma, Parma, Italy
| | | | - Stefano D'Arrigo
- Developmental Neurology Division, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
| | - Pasquale Striano
- Pediatric Neurology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy
| | | | - Marco Carotenuto
- Clinic of Child and Adolescent Neuropsychiatry, Department of Mental Health and Physical and Preventive Medicine, Università degli Studi della Campania "Luigi Vanvitelli", Caserta, Italy
| | - Alessandro Orsini
- Paediatric Neurology Section, Paediatric Department, Pisa University Hospital, University of Pisa, Pisa, Italy
| | | | | | - Vincenzo Raieli
- Child Neuropsychiatry Unit - ISMEP- ARNAS CIVICO, Palermo, Italy
| | - Michela Ada Noris Ferilli
- Department of Neuroscience, Paediatric Headache Center, Bambino Gesù Children Hospital, IRCCS, Rome, Italy
| | - Claudia Ruscitto
- Child Neurology and Psychiatry Unit, Systems Medicine Department, Tor Vergata University Hospital of Rome, Rome, Italy
| | - Elisabetta Spadoni
- Pediatric Department, Azienda Ospedaliera Nord-Ovest of Tuscany, San Luca Hospital of Lucca, Lucca, Italy
| | - Salvatore Grosso
- Molecular Medicine and Development, University of Siena, Siena, Italy
| | - Renato D'Alonzo
- Pediatric and Neonatological Unit, Maternal and Child Department, Nuovo Ospedale San Giovanni Battista, Foligno, Italy
| | - Amanda Papa
- Infantile Neuropsychiatry Departement Maggiore della Carità University Hospital, Novara, Italy
| | - Piero Pavone
- Section of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy
| | - Mariaclaudia Meli
- Azienda Policlinico, "Rodolico-San Marco Hospital", University of Catania, Catania, Italy
| | - Mario Velardita
- Department of Pediatrics, Hospital "Salvatore e Saverio Gravina", Caltagirone, Italy
| | - Martina Mainetti
- Department of Pediatrics, Santa Maria delle Croci Hospital, Ravenna, Italy
| | - Nicola Vanacore
- National Centre for Epidemiology, Surveillance and Health Promotion, National Institute of Health, Rome, Italy
| | - Osvaldo Borrelli
- Division of Neurogastroenterology and Motility, Department of Pediatric Gastroenterology, University College London (UCL) Institute of Child Health and Great Ormond Street Hospital, London, UK
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Chen YJ, Song X, Winer I, Smith P, Bhandari S, Almansa C, Richmond C, Venkatesan T, Levinthal DJ. Health Care Resource Use and Associated Costs of Cyclic Vomiting Syndrome in the United States. GASTRO HEP ADVANCES 2022; 1:963-973. [PMID: 39131242 PMCID: PMC11307738 DOI: 10.1016/j.gastha.2022.06.013] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 03/25/2022] [Accepted: 06/29/2022] [Indexed: 08/13/2024]
Abstract
BACKGROUND AND AIMS This study aimed to estimate the extent of US health care resource use (HRU) and direct cost burden of cyclic vomiting syndrome (CVS). Methods We selected patients in the MarketScan Commercial and Medicare Supplemental databases with ≥1 inpatient (IP) or ≥2 outpatient (OP) claims for CVS between October 1, 2015 and June 30, 2019, and continuous insurance enrollment for ≥12 months before (baseline) and ≥3 months after first CVS diagnosis (index). Using propensity scores based on baseline characteristics, each patient with CVS was matched to ∼3 non-CVS controls. We annualized HRU and costs to accommodate varying follow-up periods. Multivariable regressions further balanced CVS and non-CVS groups, and differences in HRU and costs between the matched cohorts were compared to quantify the direct cost burden of CVS. Results Patients with CVS incurred significantly higher average annualized HRU, with the largest differences in emergency room (1.9 vs 0.4) visits and hospital IP (0.9 vs 0.1) stays (P < .001). Patients with CVS had significantly higher annual total health care costs ($57,140 vs $14,912), with IP spending as the primary driver ($28,522 vs $3250) of the cost difference (all P < .001). After multivariable regression adjustments, total health care costs remained 4.1 times higher for patients with CVS relative to non-CVS controls, with IP costs 12.3 times higher, emergency room costs 5.8 times higher, OP visit costs 2.9 times higher, and OP pharmacy costs 1.5 times higher (all P < .001). Conclusion Newly diagnosed patients with CVS have greater health care utilization and higher costs than matched non-CVS counterparts, suggesting substantial economic burden of CVS on the US health care system.
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Affiliation(s)
- Yaozhu J. Chen
- Takeda Development Center Americas, Inc, Cambridge, Massachusetts
| | - Xue Song
- IBM Watson Health, Cambridge, Massachusetts
| | | | | | - Sanjay Bhandari
- Division of Gastroenterology and Hepatology, Department of Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin
| | | | - Camilla Richmond
- Takeda Development Center Americas, Inc, Cambridge, Massachusetts
| | - Thangam Venkatesan
- Division of Gastroenterology and Hepatology, Department of Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin
| | - David J. Levinthal
- Division of Gastroenterology, Hepatology and Nutrition, Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania
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Veenin K, Wattanasirichaigoon D, Suktitipat B, Noojarern S, Lertrit P, Tim-Aroon T, Kaewsutthi S, Treepongkaruna S. Association of Mitochondrial DNA Polymorphisms With Pediatric-Onset Cyclic Vomiting Syndrome. Front Pediatr 2022; 10:876436. [PMID: 35685918 PMCID: PMC9170898 DOI: 10.3389/fped.2022.876436] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/15/2022] [Accepted: 04/22/2022] [Indexed: 11/25/2022] Open
Abstract
BACKGROUND Cyclic vomiting syndrome (CVS) is a functional gastrointestinal disorder characterized by recurrent stereotypic episodes of vomiting. The pathophysiology of CVS remains obscure. Previous studies have supported the hypotheses of mitochondrial dysfunction. However, data on association studies between mitochondrial DNA (mtDNA) polymorphisms and pediatric-onset CVS are limited and inconsistent. The aims of this study were to describe clinical characteristics, evaluate association of mtDNA polymorphisms 16519T and 3010A with pediatric-onset CVS and identify new mtDNA candidate variants. METHODS This study involved Thai patients diagnosed with CVS according to the Rome III or IV criteria before the age of 15 years. Patients' demographic data, clinical characteristics, previous investigations and treatment outcomes were obtained. Blood samples were collected for next-generation (whole exome) sequencing, followed by analysis of chromosome M (mitochondrial. Variants were filtered according to clinical significance using ClinVar and MITOMAP. mtDNA polymorphisms in 148 normal Thai individuals were used as controls. RESULTS Forty-eight children were enrolled in the clinical study, and 30 participated in the genetic analysis. The median age at onset and median age at diagnosis was 3.0 (1.5-5.6) and 6.3 (3.0-8.6) years, respectively. Maternal history of migraine was positive in 16.7%. About 45.7% (21 of 46) of the patients achieved complete clinical remission, with the mean symptom duration of 5.9 ± 3.3 years. The prevalence of mtDNA variants 16519T and 3010A among the patient group and Thai general population (control) were as follows: 40.0% (12/30) vs. 27.7% (P = 0.18) and 6.7% (2/30) vs. 0.7% (P = 0.07), respectively. Five known pathogenic variants were identified in 6 patients, including mtDNA 8528C in one patient who also had infantile hypertrophic cardiomyopathy. Six likely pathogenic variants were found but without statistical significance. We identified 11 variants with significant prevalence in the patient group. Though, these variants were classified as variants of unknown significance (VUS), several of them were located in mt functional regions and therefore they deserve further investigations as new candidates for association with pediatric CVS. CONCLUSION There were no associations of mtDNA polymorphisms 16519T and 3010A with CVS in our pediatric cohort. Five pathogenic variants and 11 VUS were found associated with pediatric-onset CVS.
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Affiliation(s)
- Kirana Veenin
- Division of Gastroenterology, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
| | - Duangrurdee Wattanasirichaigoon
- Division of Medical Genetics, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
| | - Bhoom Suktitipat
- Department of Biochemistry, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand
| | - Saisuda Noojarern
- Division of Medical Genetics, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
| | - Patcharee Lertrit
- Department of Biochemistry, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand
| | - Thipwimol Tim-Aroon
- Division of Medical Genetics, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
| | - Supannee Kaewsutthi
- Department of Biochemistry, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand
| | - Suporn Treepongkaruna
- Division of Gastroenterology, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
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Kumagai N, Kondoh T, Matsumoto Y, Ikezumi Y. Transient Type 3 Renal Tubular Acidosis during Cyclic Vomiting Syndrome. TOHOKU J EXP MED 2022; 257:73-76. [PMID: 35354692 DOI: 10.1620/tjem.2022.j015] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
Affiliation(s)
- Naonori Kumagai
- Department of Pediatrics, Fujita Health University School of Medicine
| | - Tomomi Kondoh
- Department of Pediatrics, Fujita Health University School of Medicine
| | - Yuji Matsumoto
- Department of Pediatrics, Fujita Health University School of Medicine
| | - Yohei Ikezumi
- Department of Pediatrics, Fujita Health University School of Medicine
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Mauritz MD, Hasan C, Schreiber L, Wegener-Panzer A, Barth S, Zernikow B. Differential Diagnosis of Cyclic Vomiting and Periodic Headaches in a Child with Ventriculoperitoneal Shunt: Case Report of Chronic Shunt Overdrainage. CHILDREN (BASEL, SWITZERLAND) 2022; 9:432. [PMID: 35327804 PMCID: PMC8946983 DOI: 10.3390/children9030432] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 02/22/2022] [Revised: 03/10/2022] [Accepted: 03/16/2022] [Indexed: 11/17/2022]
Abstract
Fourteen months after the implantation of a ventriculoperitoneal shunt catheter, a six-year-old boy developed recurrent, severe headaches and vomiting every three weeks. The attacks were of such severity that hospitalizations for analgesic and antiemetic therapies and intravenous rehydration and electrolyte substitution were repeatedly required. The patient was asymptomatic between the attacks. After an extensive diagnostic workup-including repeated magnetic resonance imaging (MRI) and neurosurgical examinations-common differential diagnoses, including shunt overdrainage, were ruled out. The patient was transferred to a specialized pediatric pain clinic with suspected cyclic vomiting syndrome (CVS). Despite intensive and in part experimental prophylactic and abortive pharmacological treatment, there was no improvement in his symptoms. Consecutive MRI studies reinvestigating the initially excluded shunt overdrainage indicated an overdrainage syndrome. Subsequently, the symptoms disappeared after disconnecting the shunt catheter. This case report shows that even if a patient meets CVS case definitions, other differential diagnoses must be carefully reconsidered to avoid fixation error.
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Affiliation(s)
- Maximilian David Mauritz
- Paediatric Palliative Care Centre, Children’s and Adolescents’ Hospital, Witten/Herdecke University, 45711 Datteln, Germany; (C.H.); (B.Z.)
| | - Carola Hasan
- Paediatric Palliative Care Centre, Children’s and Adolescents’ Hospital, Witten/Herdecke University, 45711 Datteln, Germany; (C.H.); (B.Z.)
- Department of Children’s Pain Therapy and Paediatric Palliative Care, Faculty of Health, School of Medicine, Witten/Herdecke University, 58448 Witten, Germany
| | - Lutz Schreiber
- Department of Pediatric Neurosurgery, Klinikum Vest, Academic Teaching Hospital, Ruhr University Bochum, 45657 Recklinghausen, Germany;
| | - Andreas Wegener-Panzer
- Department of Radiology, Children’s and Adolescents’ Hospital, Witten/Herdecke University, 45711 Datteln, Germany;
| | - Sylvia Barth
- Department of Pediatrics, Ostalb Klinikum Aalen, 73430 Aalen, Germany;
| | - Boris Zernikow
- Paediatric Palliative Care Centre, Children’s and Adolescents’ Hospital, Witten/Herdecke University, 45711 Datteln, Germany; (C.H.); (B.Z.)
- Department of Children’s Pain Therapy and Paediatric Palliative Care, Faculty of Health, School of Medicine, Witten/Herdecke University, 58448 Witten, Germany
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Reeves PT, Kovacic K, Rogers PL, Sanghavi R, Levinthal DJ, Echelmeyer S, Li BUK. Development and Assessment of a Low Literacy, Pictographic Cyclic Vomiting Syndrome Action Plan. J Pediatr 2022; 242:174-183.e1. [PMID: 34740589 DOI: 10.1016/j.jpeds.2021.10.047] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/13/2021] [Revised: 10/04/2021] [Accepted: 10/26/2021] [Indexed: 12/17/2022]
Abstract
OBJECTIVE To develop and assess an evidence-based, individualized Cyclic Vomiting Syndrome Action Plan (CVSAP) to optimize both preventative and acute care. STUDY DESIGN This implementation science project synthesized a combination of clinical practice guidelines, published literature, and clinical experience by a team of CVS clinicians to develop the CVSAP. The tool was developed to include validated pictograms and an automatic, embedded, weight-based dosing calculator to output acute management recommendations. The final version of the CVSAP was tested by patients/caregivers, readability calculators, medical librarians, and clinicians using validated metrics. RESULTS All pictograms met the criteria for inclusion in the CVSAP. A composite readability score of 5.32 was consistent with a fifth-grade level. Patients/caregivers (n = 70) judged the CVSAP to be of high quality with consumer information rating form rating of 84.2%. Six medical librarians rated the CVSAP to have 93% understandability and 100% actionability, and 33 clinicians completing the SAM generated a suitability rating of 87.5%. CONCLUSIONS The CVSAP visually highlights individualized care plan components to facilitate optimized preventative and acute CVS care. Further investigation will determine if CVSAP increases caregiver confidence and compliance in home management and improves quality of life and clinical outcomes for patients with CVS.
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Affiliation(s)
- Patrick T Reeves
- Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Walter Reed National Military Medical Center, Bethesda, MD; Department of Pediatrics, Uniformed Services University of the Health Sciences, Bethesda, MD.
| | - Katja Kovacic
- Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Medical College of Wisconsin, Milwaukee, WI
| | - Philip L Rogers
- Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Walter Reed National Military Medical Center, Bethesda, MD; Department of Pediatrics, Uniformed Services University of the Health Sciences, Bethesda, MD
| | - Rinarani Sanghavi
- Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, U T Southwestern Medical Center, Dallas, TX
| | - David J Levinthal
- Department of Medicine, Division of Gastroenterology, Hepatology and Nutrition, University of Pittsburgh Medical Center, Pittsburgh, PA
| | - Sofia Echelmeyer
- Department of Pediatrics, Uniformed Services University of the Health Sciences, Bethesda, MD
| | - B U K Li
- Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Medical College of Wisconsin, Milwaukee, WI
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Dipasquale V, Falsaperla R, Bongiovanni A, Ruggieri M, Romano C. Clinical features and long-term outcomes in pediatric cyclic vomiting syndrome: A 9-year experience at three tertiary academic centers. Neurogastroenterol Motil 2022; 34:e14224. [PMID: 34431167 DOI: 10.1111/nmo.14224] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/02/2021] [Revised: 06/08/2021] [Accepted: 07/07/2021] [Indexed: 02/08/2023]
Abstract
BACKGROUND Pediatric cyclic vomiting syndrome (CVS) is a little-known clinical condition, frequently diagnosed with delay. This study aims to describe the clinical presentation and management and to define possible predictive factors of the disease outcome. METHODS In this retrospective study, all children who were diagnosed with CVS during the period 2010-2019 in three tertiary academic centers were included. The association between clinical variables and outcomes was investigated. KEY RESULTS Fifty-seven children were included (male/female ratio 1.3:1; mean age at diagnosis 8.2 years). At the time of diagnosis, 63% of children had at least one episode every month. One or more prodromes were reported by 75% of patients. Family history of migraine was reported for 47% of children. Nearly, all of the children were started on prophylactic treatment. The median follow-up period was 29 months ± 15. Overall, 56% of children had resolution of vomiting. Twenty-six percent of children developed migraine. There were no differences in gender, age at onset, duration of follow-up, severity, medication, family history, or trigger factors between children who underwent resolution of vomiting and those who did not, or between children who suffered from migraine or not at follow-up. Four types of disease outcome were recognized, that is, resolution of vomiting with or without associated symptoms; persistence of vomiting with or without associated symptoms. CONCLUSIONS AND INFERENCES Cyclic vomiting syndrome clinical presentation has inter-individual variability. Most children recover at follow-up, but often develop further somatic symptoms. In this study, no clinical variable clearly predicted the evolution of the syndrome toward one or the other outcome.
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Affiliation(s)
- Valeria Dipasquale
- Unit of Pediatric Gastroenterology and Cystic Fibrosis Unit, Department of Human Pathology in Adulthood and Childhood ''G. Barresi'', University of Messina, Messina, Italy
| | - Raffaele Falsaperla
- Neonatal Intensive Care Unit [NICU], Pediatrics and Pediatric Emergency Unit, AOU "Policlinico", PO "San Marco", University of Catania, Catania, Italy
| | - Annarita Bongiovanni
- Neonatal Intensive Care Unit [NICU], Pediatrics and Pediatric Emergency Unit, AOU "Policlinico", PO "San Marco", University of Catania, Catania, Italy
| | - Martino Ruggieri
- Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry, University of Catania, Catania, Italy
| | - Claudio Romano
- Unit of Pediatric Gastroenterology and Cystic Fibrosis Unit, Department of Human Pathology in Adulthood and Childhood ''G. Barresi'', University of Messina, Messina, Italy
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Tillman EM, Harvath EM. Cyclic Vomiting Syndrome in Pediatric Patients: A Review of Therapeutics. J Pediatr Pharmacol Ther 2022; 27:12-18. [PMID: 35002554 DOI: 10.5863/1551-6776-27.1.12] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/10/2021] [Accepted: 07/15/2021] [Indexed: 11/11/2022]
Abstract
Cyclic vomiting syndrome (CVS) is a functional gastrointestinal disorder that can present quite a challenge to clinicians caring for children with this complex disease. Different therapeutic interventions are recommended for prophylaxis and acute abortive therapy for a CVS attack. The aim of this review is to summarize therapeutic treatment recommendations from the 2008 North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHN) Consensus Statement on the Diagnosis and Management of Cyclic Vomiting Syndrome and discuss studies contemporary to this expert recommendation. After an extensive search of medical databases, 8 studies that evaluated therapeutic treatments for CVS were identified. Amitriptyline and cyproheptadine remain the standard of care for prophylaxis. Nutritional supplements such as carnitine and coenzyme Q10 have shown efficacy in decreasing episodes and severity in small studies with high tolerability among patients. The combination of ondansetron and sumatriptan are recommended for abortion of an acute vomiting episode, but other agents such as aprepitant and sedative agents can be considered when vomiting is refractory to initial treatments.
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Affiliation(s)
- Emma M Tillman
- Department of Medicine (EMT), Indiana University School of Medicine, Indianapolis, IN
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Frattale I, Ruscitto C, Papetti L, Ursitti F, Sforza G, Moavero R, Ferilli MAN, Tarantino S, Balestri M, Vigevano F, Mazzone L, Valeriani M. Migraine and Its Equivalents: What Do They Share? A Narrative Review on Common Pathophysiological Patterns. Life (Basel) 2021; 11:1392. [PMID: 34947923 PMCID: PMC8705894 DOI: 10.3390/life11121392] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/21/2021] [Revised: 11/30/2021] [Accepted: 12/10/2021] [Indexed: 01/02/2023] Open
Abstract
Migraine is the first in order of frequency of the neurological disorders, affecting both adult and paediatric populations. It is also the first cause of primary headaches in children. Migraine equivalents are periodic disorders that can be associated with migraine or considered as prognostic features of a future migraine manifestation. Despite the mechanisms underlying migraine and its equivalents are not entirely clear, several elements support the hypothesis of common pathophysiological patterns shared by these conditions. The aim of this review is thus to analyze the literature in order to highlight which currently known mechanisms may be common between migraine and its equivalents.
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Affiliation(s)
- Ilaria Frattale
- Child Neurology and Psychiatry Unit, Systems Medicine Department, Tor Vergata University, Hospital of Rome, 00165 Rome, Italy; (I.F.); (C.R.); (R.M.); (L.M.)
| | - Claudia Ruscitto
- Child Neurology and Psychiatry Unit, Systems Medicine Department, Tor Vergata University, Hospital of Rome, 00165 Rome, Italy; (I.F.); (C.R.); (R.M.); (L.M.)
| | - Laura Papetti
- Neurology Unit, Department of Neuroscience, Bambino Gesù Children Hospital, IRCCS, 00165 Rome, Italy; (L.P.); (F.U.); (G.S.); (M.A.N.F.); (S.T.); (M.B.); (F.V.)
| | - Fabiana Ursitti
- Neurology Unit, Department of Neuroscience, Bambino Gesù Children Hospital, IRCCS, 00165 Rome, Italy; (L.P.); (F.U.); (G.S.); (M.A.N.F.); (S.T.); (M.B.); (F.V.)
| | - Giorgia Sforza
- Neurology Unit, Department of Neuroscience, Bambino Gesù Children Hospital, IRCCS, 00165 Rome, Italy; (L.P.); (F.U.); (G.S.); (M.A.N.F.); (S.T.); (M.B.); (F.V.)
| | - Romina Moavero
- Child Neurology and Psychiatry Unit, Systems Medicine Department, Tor Vergata University, Hospital of Rome, 00165 Rome, Italy; (I.F.); (C.R.); (R.M.); (L.M.)
- Neurology Unit, Department of Neuroscience, Bambino Gesù Children Hospital, IRCCS, 00165 Rome, Italy; (L.P.); (F.U.); (G.S.); (M.A.N.F.); (S.T.); (M.B.); (F.V.)
| | - Michela Ada Noris Ferilli
- Neurology Unit, Department of Neuroscience, Bambino Gesù Children Hospital, IRCCS, 00165 Rome, Italy; (L.P.); (F.U.); (G.S.); (M.A.N.F.); (S.T.); (M.B.); (F.V.)
| | - Samuela Tarantino
- Neurology Unit, Department of Neuroscience, Bambino Gesù Children Hospital, IRCCS, 00165 Rome, Italy; (L.P.); (F.U.); (G.S.); (M.A.N.F.); (S.T.); (M.B.); (F.V.)
| | - Martina Balestri
- Neurology Unit, Department of Neuroscience, Bambino Gesù Children Hospital, IRCCS, 00165 Rome, Italy; (L.P.); (F.U.); (G.S.); (M.A.N.F.); (S.T.); (M.B.); (F.V.)
| | - Federico Vigevano
- Neurology Unit, Department of Neuroscience, Bambino Gesù Children Hospital, IRCCS, 00165 Rome, Italy; (L.P.); (F.U.); (G.S.); (M.A.N.F.); (S.T.); (M.B.); (F.V.)
| | - Luigi Mazzone
- Child Neurology and Psychiatry Unit, Systems Medicine Department, Tor Vergata University, Hospital of Rome, 00165 Rome, Italy; (I.F.); (C.R.); (R.M.); (L.M.)
| | - Massimiliano Valeriani
- Neurology Unit, Department of Neuroscience, Bambino Gesù Children Hospital, IRCCS, 00165 Rome, Italy; (L.P.); (F.U.); (G.S.); (M.A.N.F.); (S.T.); (M.B.); (F.V.)
- Center for Sensory-Motor Interaction, Aalborg University, 9220 Aalborg Øst, Denmark
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Goldman RD. Cyclic vomiting syndrome in children. CANADIAN FAMILY PHYSICIAN MEDECIN DE FAMILLE CANADIEN 2021; 67:837-838. [PMID: 34772711 PMCID: PMC8589136 DOI: 10.46747/cfp.6711837] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
Abstract
QUESTION Several children in my office have recurrent vomiting events and they usually end up in the emergency department for intravenous rehydration. One of them has been suffering from those attacks approximately once per month for the past 2 years, leading to a reduction in her quality of life. What is known about cyclic vomiting syndrome and can it be prevented? ANSWER Cyclic vomiting syndrome includes severe episodic vomiting lasting for hours or days, separated by symptom-free intervals. This gut-brain interaction is poorly understood and is difficult to diagnose. Children suffer from relentless vomiting, lethargy, abdominal pain, anorexia, and nausea. Half of the children require intravenous rehydration. Once diagnosis is made, supportive measures to reduce suffering are recommended and include administering fluids, encouraging sleep, promoting quiet environments, and administering antiemetics or sedatives. In adults, tricyclic antidepressants such as amitriptyline, as well as topiramate as second-line therapy, have been proposed for prophylactic treatment. However, pediatric data are very limited and evidence does not support any recommended course of prophylactic therapy for children with the condition.
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Electrocardiogram Before Tricyclic Antidepressant Use: Minimal Impact in Pediatric Functional Gastrointestinal Disorders. J Pediatr Gastroenterol Nutr 2021; 73:523-528. [PMID: 34546995 DOI: 10.1097/mpg.0000000000003226] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
OBJECTIVES The aim of this study was to determine the effect of electrocardiogram (ECG) findings on the initiation of tricyclic antidepressants (TCAs) for functional gastrointestinal disorders (FGIDs) and to evaluate cardiac outcomes related to low dose TCA use. METHODS We performed a retrospective chart review of all pediatric outpatients at a tertiary pediatric hospital with an ECG ordered by a pediatric gastroenterologist when considering initiation of a TCA between January 2011 and February 2018. We collected demographics, previous cardiovascular testing results, TCA dosing, and pertinent outcomes, including cardiology referrals, emergency department, and hospital admissions, and death during the study period. All ECGs were reviewed for corrected QT (QTc) interval, heart rate, and other abnormalities. RESULTS Of 233 patients with screening ECGs, most (84.1%) were prescribed a TCA. Functional abdominal pain or dyspepsia account for 82.0% of diagnoses. Initial TCA dosing of amitriptyline varied widely, 10-50 mg/day, and the dose was not associated with QTc intervals. TCAs were not started in only 1.7% (4/233) due to ECG results. A significant ECG abnormality prompting cardiology referral was found in eight (3.4%) with a prolonged QTc interval in one (0.4%). In 10.7% (25/233) of patients, screening ECG was obtained despite available ECG in the chart. No deaths and no emergency department or hospital visits for arrhythmia or drug overdose occurred. CONCLUSION Screening ECGs infrequently influence TCA initiation and may lead to increased resource utilization. The overall frequency of cardiology referral due to ECG results is low. Serious adverse cardiac events are unlikely with low dose TCA administration.
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Dosani K, Koletic C, Alhosh R. Cannabinoid Hyperemesis Syndrome in Pediatrics: An Emerging Problem. Pediatr Rev 2021; 42:500-506. [PMID: 34470869 DOI: 10.1542/pir.2019-0097] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
Abstract
Cannabis use has been evolving in both recreational drug use and medicinal uses, in part due to the recent introduction of various strains and components. With increasing use, we have seen emergence of a hyperemesis syndrome called cannabinoid hyperemesis syndrome characterized as intractable vomiting, nausea, and abdominal pain in the context of prolonged cannabinoid use. Although the antiemetic properties of cannabis have been known for years, the paradoxical effect of hyperemesis has yet to be elucidated. Herein we discuss the current research, epidemiology, and diagnosis and treatment of cannabinoid hyperemesis syndrome. Without timely diagnosis and treatment, patients may experience significant physical and emotional distress, as well as place unnecessary financial burden on the medical system.
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Affiliation(s)
- Kaushal Dosani
- University of Nevada Las Vegas School of Medicine, Las Vegas, NV
| | - Carolina Koletic
- University of Nevada Las Vegas School of Medicine, Las Vegas, NV
| | - Rabea Alhosh
- University of Nevada Las Vegas School of Medicine, Las Vegas, NV
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Talukder NT, Clorfeine AH, Black MK, Moody SB. Atypical idiopathic intracranial hypertension presenting as cyclic vomiting syndrome: a case report. J Med Case Rep 2021; 15:440. [PMID: 34461990 PMCID: PMC8405252 DOI: 10.1186/s13256-021-03068-x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2021] [Accepted: 08/14/2021] [Indexed: 11/21/2022] Open
Abstract
Background Idiopathic intracranial hypertension is a disorder of increased intracranial pressure in the absence of cerebrospinal outflow obstruction, mass lesion, or other underlying cause. It is a rare phenomenon in prepubertal children and is most typically found in women of childbearing age. The classic presentation consists of headaches, nausea, vomiting, and visual changes; however, children present more atypically. We report a case of idiopathic intracranial hypertension in an otherwise healthy, 4-year-old child with atypical symptoms resembling those of cyclic vomiting syndrome. Case presentation A 4-year-old Caucasian, otherwise healthy, male child presented to our emergency department with episodic intermittent early-morning vomiting occurring once every 1–3 weeks without interepisodic symptoms, starting 10 months prior. With outpatient metabolic, autoimmune, endocrine, allergy, and gastroenterology work-up all unremarkable, he was initially diagnosed with cyclic vomiting syndrome. Discovery of mild optic nerve sheath distension on magnetic resonance imaging of the brain 10 months after symptom onset led to inpatient admission and a lumbar puncture notable for an opening pressure of 47 mmHg, with normal cell count and protein levels. He had no changes in visual acuity or optic disc edema on dilated fundoscopic examination. The patient was started on acetazolamide, with resolution of episodic emesis at his last follow-up visit 12 weeks after discharge. Conclusions Idiopathic intracranial hypertension presents atypically in prepubescent children, with about one-fourth presenting asymptomatically, and only 13–52% presenting with “classic” symptoms. With a prevalence of only 0.6–0.7 per 100,000, much remains unknown regarding the underlying pathophysiology in this demographic. Cyclic vomiting syndrome, however, has a much higher prevalence in this age group, with a prevalence of 0.4–1.9 per 100. It is thought to be an idiopathic, periodic disorder of childhood, often linked to neurological conditions such as abdominal migraines, epilepsy, mitochondrial disorders, and structural lesions such as chiari malformation and posterior fossa tumors. While cyclic vomiting syndrome is thought to have a benign course, untreated idiopathic intracranial hypertension can have long-term detrimental effects, such as visual loss or even blindness. We present a case of idiopathic intracranial hypertension presenting with symptoms resembling cyclic vomiting syndrome in a 4-year-old child, diagnosed 10 months after initial onset of symptoms. We aim to demonstrate the need for a high level of clinical suspicion and the need for further investigation into underlying pathophysiology in this vulnerable population.
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Affiliation(s)
- Nafee T Talukder
- Department of Neurology, Children's Memorial Hermann Hospital, 6410 Fannin St., Ste 1014, Houston, TX, 77030, USA. .,University of Texas Health Science Center, Houston, TX, USA.
| | - Amanda H Clorfeine
- Department of Neurology, Children's Memorial Hermann Hospital, 6410 Fannin St., Ste 1014, Houston, TX, 77030, USA.,University of Texas Health Science Center, Houston, TX, USA
| | - Moira K Black
- Department of Neurology, Children's Memorial Hermann Hospital, 6410 Fannin St., Ste 1014, Houston, TX, 77030, USA.,University of Texas Health Science Center, Houston, TX, USA
| | - Shade B Moody
- Department of Neurology, Children's Memorial Hermann Hospital, 6410 Fannin St., Ste 1014, Houston, TX, 77030, USA.,University of Texas Health Science Center, Houston, TX, USA
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Abstract
Migraine is a complex genetic brain disorder with an intricate pathogenesis and polymorphous clinical presentations, particularly in children. In this Perspective, we describe the different phenotypes of migraine in children, including conditions that have been referred to in the International Classification of Headache Disorders as "syndromes that may be related to migraine''. Evidence is presented for the integration of abdominal migraine, cyclical vomiting syndrome, benign paroxysmal vertigo, benign paroxysmal torticollis and infantile colic into the unified diagnosis of 'childhood migraine syndrome' on the basis of clinical and epidemiological characteristics, and shared inheritance. In our opinion, such integration will guide clinicians from specialities other than neurology to consider migraine in the assessment of children with these disorders, as well as stimulate research into the genetics, pathophysiology and clinical features of all disorders within the syndrome. A diagnosis of childhood migraine syndrome would also enable patients to benefit from inclusion in clinical trials of old and new migraine treatments, thus potentially increasing the number of treatment options available.
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Abdulkader ZM, Bali N, Vaz K, Yacob D, Di Lorenzo C, Lu PL. Predictors of Hospital Admission for Pediatric Cyclic Vomiting Syndrome. J Pediatr 2021; 232:154-158. [PMID: 33259858 DOI: 10.1016/j.jpeds.2020.11.055] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/28/2020] [Revised: 11/09/2020] [Accepted: 11/24/2020] [Indexed: 12/26/2022]
Abstract
OBJECTIVES To identify predictors of hospitalization in pediatric patients presenting to an emergency department (ED) for a cyclic vomiting syndrome (CVS) attack. STUDY DESIGN We retrospectively reviewed patients with CVS seen at our institution between 2015 and 2018 and included those who met the Rome IV criteria for CVS. We identified all CVS-related ED visits and subsequently performed a case-control analysis, utilizing multivariate logistic regression, to identify clinical and demographic factors that may predict hospitalization. RESULTS In total, 219 patients with CVS (using International Statistical Classification of Diseases and Related Health Problems, 10th Revision) were identified, of which 65% met the inclusion criteria (median age 11 years). We identified 152 CVS-related ED visits, of which 62% resulted in hospitalization. Factors found to predict hospitalization using multivariate analyses included male sex (P = .04), younger age (P = .027), delayed presentation (>24 hours) to the ED (P < .001), and longer wait time prior treatment with antiemetics (P = .029). CONCLUSION One-quarter of all patients with CVS had presented to the ED and nearly two-thirds of these ED visits resulted in hospitalization. A delayed presentation to the ED following the onset of symptoms was the strongest independent predictor of hospital admission, alongside male sex, younger age, and longer ED wait times before treatment with antiemetics. These findings suggest that early intervention may be key to successfully mitigating the risk of hospitalization for a CVS attack.
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Affiliation(s)
- Zeyad M Abdulkader
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Nationwide Children's Hospital, Columbus, OH.
| | - Neetu Bali
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Nationwide Children's Hospital, Columbus, OH
| | - Karla Vaz
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Nationwide Children's Hospital, Columbus, OH
| | - Desalegn Yacob
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Nationwide Children's Hospital, Columbus, OH
| | - Carlo Di Lorenzo
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Nationwide Children's Hospital, Columbus, OH
| | - Peter L Lu
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Nationwide Children's Hospital, Columbus, OH
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Li BUK. Treating Cyclic Vomiting Syndrome in the Emergency Department: Sooner Appears Better. J Pediatr 2021; 232:12-14. [PMID: 33373673 DOI: 10.1016/j.jpeds.2020.12.066] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/22/2020] [Accepted: 12/23/2020] [Indexed: 02/07/2023]
Affiliation(s)
- B U K Li
- Department of Pediatrics, Medical College of Wisconsin, Middleton, Wisconsin.
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47
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Nivatsi M, Aslanidou I, Mantadakis E. Highly effective use of aprepitant in an adolescent girl with severe cyclic vomiting syndrome. BMJ Case Rep 2021; 14:e241132. [PMID: 33664041 PMCID: PMC7934711 DOI: 10.1136/bcr-2020-241132] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/21/2021] [Indexed: 01/16/2023] Open
Abstract
Cyclic vomiting syndrome (CVS) is a debilitating functional gastrointestinal disorder. Diagnosis is based on the Rome III criteria. There are no evidence-based guidelines for the management of paediatric CVS, although ondansetron and antimigraine medications are frequently tried. We describe a 13-year-old adolescent girl with severe CVS and numerous hospital admissions for dehydration because of cyclic vomiting. She had failed oral ondansetron therapy. Oral aprepitant (125 mg in the first, and 85 mg on the second and third days), a neurokinin 1 receptor antagonist that has been approved for preventing chemotherapy-induced vomiting or postoperative emesis, was tried in our patient at home during the first prodromal signs of an upset stomach. She had a dramatic response to it, with no further episodes of vomiting since its start. There is an urgent need for randomised clinical studies to assess the efficacy of available treatment options, including aprepitant in patients with severe CVS.
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Affiliation(s)
- Maria Nivatsi
- Department of Paediatrics, University General Hospital of Alexandroupolis, Alexandroupolis, Thrace, Greece
| | - Ilona Aslanidou
- Department of Paediatrics, University General Hospital of Alexandroupolis, Alexandroupolis, Thrace, Greece
| | - Elpis Mantadakis
- Department of Paediatrics, Democritus University of Thrace Faculty of Medicine, Alexandroupolis, Thrace, Greece
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48
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Kovacic K, Li BUK. Cyclic vomiting syndrome: A narrative review and guide to management. Headache 2021; 61:231-243. [PMID: 33619730 DOI: 10.1111/head.14073] [Citation(s) in RCA: 16] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/18/2020] [Revised: 01/03/2021] [Accepted: 01/06/2021] [Indexed: 12/18/2022]
Abstract
OBJECTIVES/BACKGROUND Cyclic vomiting syndrome (CVS) is a disabling disorder of gut-brain interaction manifested by stereotypical and severe episodes of nausea and vomiting. Prevalence data indicate that CVS affects 1-2% of children and there has been a recent dramatic rise in diagnosed adults. METHODS This narrative review summarizes relevant literature pertaining to pediatric and adult CVS and provides a guide to management based on extensive clinical experience. RESULTS More timely diagnosis is facilitated by an expert consensus diagnostic approach and limited testing. Some diagnostic tests of exclusion remain essential. These include an upper gastrointestinal (GI) contrast study to exclude intestinal malrotation and basic laboratory screening. An abdominal ultrasound is recommended to exclude renal hydronephrosis in children and biliary disease in adults. Exclusion of metabolic/genetic conditions is warranted in those with specific warning signs, presentation in infants/toddler age, and in those with refractory disease. In the absence of chronic GI symptoms, referral to a GI specialist for upper endoscopy is generally not necessary in children but recommended in adults. A large subset termed migraine-equivalent CVS display strong clinical and genetic features of migraine. A unifying pathophysiologic core concept involves neuronal hyperexcitability and aberrant central modulation of autonomic signals. This is coupled with multiple susceptibility factors including mitochondrial dysfunction/cellular energy deficits, a hyper-responsive hypothalamic-pituitary-adrenal axis and many comorbidities that increase vulnerability to triggering events. CVS episodes are frequently triggered by stressors and intercurrent illnesses. Lifestyle and non-pharmacological interventions thus play a pivotal role in successful management. Pharmacological therapies are categorized into abortive, supportive/rescue, and prophylactic treatments. The majority respond particularly well to migraine-focused treatment strategies. CONCLUSION Despite improved characterization and understanding, CVS remains classified as a functional disorder of brain-gut interaction that is often disjointly managed by generalists and subspecialists. Early recognition, evaluation, and management will facilitate care and improve outcomes. Further research into its natural history with common progression to migraine headaches, neuroendocrine mechanisms, and the pathophysiologic relation to migraine diathesis is much needed.
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Affiliation(s)
- Katja Kovacic
- Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Medical College of Wisconsin, Milwaukee, WI, USA
| | - B U K Li
- Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Medical College of Wisconsin, Milwaukee, WI, USA
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Abstract
BACKGROUND In order to distinguish seizure mimics from seizures in children it is important to clarify the event duration, frequency, semiology, and any precipitating factors. METHODOLOGY This review provides a succinct and up-to-date overview aimed at general and subspecialty non-neurologist pediatric and adolescent health care providers to help guide a diagnostic approach when a child presents with paroxysmal events of unclear etiology. RESULTS In many cases, seizure mimics are consistently triggered by an event, location, or emotion, and often resolve with distraction or tactile stimulation. Suspicion should be raised for seizures when events occur out of deep sleep, there is a loss of consciousness with the event, movements are not suppressible, and there is a period of fatigue afterword (minutes to hours). Further, a past medical history of developmental delay (e.g. autism), developmental regression, or neurologic injury increases patients' risk for seizures. CONCLUSIONS Common seizure mimics are highlighted in the age group in which they are most prevalent.
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Affiliation(s)
- Rebecca S Stainman
- The Department of Neurology, NYU Langone Health, New York, NY, United States.
| | - Eric H Kossoff
- The Department of Neurology and The Department of Pediatrics, Johns Hopkins Medicine, 601N. Caroline St., Baltimore, MD 21287
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50
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Hikita T, Hoshino H, Mimaki M. Ramosetron as a Treatment for Cyclic Vomiting Syndrome: A Small-Scale Patient Trial. Glob Pediatr Health 2020; 7:2333794X20969281. [PMID: 33225023 PMCID: PMC7656858 DOI: 10.1177/2333794x20969281] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/08/2020] [Revised: 08/21/2020] [Accepted: 09/30/2020] [Indexed: 11/16/2022] Open
Affiliation(s)
- Toshiyuki Hikita
- Teikyo University School of Medicine, Itabashi-ku, Tokyo, Japan.,Hikita Pediatric Clinic, Gunma, Japan
| | - Hideki Hoshino
- Teikyo University School of Medicine, Itabashi-ku, Tokyo, Japan
| | - Masakazu Mimaki
- Teikyo University School of Medicine, Itabashi-ku, Tokyo, Japan
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