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Pilania RK, Tremoulet AH, Prinja S, Dahdah N, Singh S. Kawasaki disease: the most common cause of acquired heart disease among children globally. Cardiol Young 2025:1-3. [PMID: 39968873 DOI: 10.1017/s1047951125000459] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/20/2025]
Abstract
Kawasaki disease is a childhood vasculitic disorder that has a special predilection for coronary arteries. Kawasaki disease has been reported from all regions of the world, with an increasing incidence in several countries. Kawasaki disease is now the most common cause of acquired heart disease in children all over the world. However, it is concerning that the estimated vast majority of Kawasaki disease cases in low- and middle-income countries are not getting diagnosed and treated. The World Health Organization acknowledges cardiovascular disease in their priority of actions. The World Health Organization is invited to acknowledge the reality of Kawasaki disease in its list of cardiovascular diseases and take steps to facilitate the diagnosis and treatment of Kawasaki disease, especially in low- and middle-income countries. It is a disease of public health importance and needs urgent prioritisation by the World Health Organization.
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Affiliation(s)
- Rakesh Kumar Pilania
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Adriana H Tremoulet
- Kawasaki disease Research Center, Department of Pediatrics, University of California San Diego, La Jolla, CA, USA
- Rady Children's Hospital-San Diego, San Diego, CA, USA
| | - Shankar Prinja
- Department of Community Medicine and School of Public Health, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Nagib Dahdah
- Department of Pediatric Cardiology, CHU Sainte-Justine, University of Montreal, Montreal, QC, Canada
| | - Surjit Singh
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
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Thangaraj A, Pilania RK, Hamada H, Singh S. The Legacy of Dr. Tomisaku Kawasaki-A Tribute to the Legendary Japanese Pediatrician on His 100th Birth Anniversary: February 07, 2025. Int J Rheum Dis 2025; 28:e70074. [PMID: 39812107 DOI: 10.1111/1756-185x.70074] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/03/2025] [Accepted: 01/06/2025] [Indexed: 01/16/2025]
Affiliation(s)
- Abarna Thangaraj
- Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Rakesh Kumar Pilania
- Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Hiromichi Hamada
- Department of Pediatrics, Graduate School of Medicine, Chiba University, Chiba, Japan
| | - Surjit Singh
- Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
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Singhal M, Pilania RK, Thangaraj A, Chaudhary H, Gummadi A, Soundararajan R, Loganathan S, Sharma A, Gupta A, Bhattad S, Jindal AK, Vignesh P, Suri D, Sandhu MS, Singh S. The value of CT coronary angiography for a comprehensive assessment of left circumflex artery in Kawasaki disease: 9 years of experience from a tertiary center. THE LANCET REGIONAL HEALTH. SOUTHEAST ASIA 2024; 29:100471. [PMID: 39258250 PMCID: PMC11386306 DOI: 10.1016/j.lansea.2024.100471] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/17/2024] [Revised: 08/02/2024] [Accepted: 08/07/2024] [Indexed: 09/12/2024]
Abstract
Background Transthoracic echocardiography (TTE) has traditionally been the primary method for coronary imaging in children with Kawasaki disease (KD). We aimed to evaluate coronary artery lesions (CALs) of the left circumflex artery (LCx) in KD on computed tomography coronary angiography (CTCA). Methods Over a 9-year period (November 2013-December 2022), 225 children with KD underwent radiation-optimized CTCA on a 128-slice dual-source platform. TTE was performed on the same day, or a day prior or after CTCA. Findings On CTCA, LCx CALs were seen in 41/225 (18.2%) patients. However, TTE detected CALs in only one third of these patients [15/41 (36.6%)]. CTCA showed 47 LCx CALs in 41 patients-aneurysms in 39 patients (40 fusiform, 2 saccular; 7 giant aneurysms), stenoses in 3, and thrombosis in 2. Thromboses and stenoses were both missed on TTE. Proximal LCx aneurysms were seen in 39 patients-of these, 12 had distal extension. Six patients had distal LCx aneurysms without proximal involvement and 2 non-contiguous multiple aneurysms. Four (9.75%) patients had isolated LCx involvement. Based on CTCA findings, treatment protocols had to be modified in 3/41 (7.3%) patients. Interpretation This study highlights anatomical findings of LCx involvement in KD. Isolated LCx CALs were noted in 4/41 (9.75%) patients. TTE alone proved inadequate for LCx assessment in children with KD. With abnormalities detected in 18.2% of cases, including those missed by TTE, CTCA emerges as an essential imaging modality. The findings have implications for treatment planning and follow-up strategies in children with KD. Funding None.
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Affiliation(s)
- Manphool Singhal
- Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Rakesh Kumar Pilania
- Paediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Abarna Thangaraj
- Paediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Himanshi Chaudhary
- Paediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Anjani Gummadi
- Paediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Raghuraman Soundararajan
- Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Sathish Loganathan
- Paediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Arun Sharma
- Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Aman Gupta
- Paediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Sagar Bhattad
- Paediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Ankur Kumar Jindal
- Paediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Pandiarajan Vignesh
- Paediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Deepti Suri
- Paediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Manavjit Singh Sandhu
- Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Surjit Singh
- Paediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
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Pilania RK, Basu S, Dixit J, Kumrah R, Jindal AK, Thangaraj A, Nimesh R, Kaur T, Vignesh P, Suri D, Rawat A, Naganur SH, Singhal M, Prinja S, Singh S. Incidence of Kawasaki disease among children in Chandigarh, India during 2015-2019: a trend analysis. THE LANCET REGIONAL HEALTH. SOUTHEAST ASIA 2024; 29:100474. [PMID: 39310717 PMCID: PMC11416213 DOI: 10.1016/j.lansea.2024.100474] [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: 03/12/2024] [Revised: 08/04/2024] [Accepted: 08/21/2024] [Indexed: 09/25/2024]
Abstract
Background Only limited information exists regarding the epidemiology of Kawasaki disease (KD) in low-income and middle-income countries. The present study provides the incidence of KD during 2015-2019 in Chandigarh, north India. Our centre follows the largest KD cohort in India. Methods Children with KD at Chandigarh diagnosed during January 2015-December 2019 were enrolled in the study. Annual incidence rates were determined using decadal growth rates of the National Census 2011. We computed the incidence of KD in children aged <5, and <15 years. We also undertook linear trend analysis using our incidence data from 1994 to 2019. Findings During 2015-2019, 83 patients (66 males, 17 females) were diagnosed with KD in Chandigarh. Incidence rates during these 5 years were 5.64, 9.25, 9.11, 9.87, and 9.72/100,000 in children aged <5 years, and 2.65, 4.44, 3.86, 5.07, 4.74/100,000 in children aged <15 years. The median age at diagnosis was 48 months (range: 12 days to 15 years). Compared to previous data (2009-2014), there was a 53.1% increase in annual incidence of KD in children aged <5 years, and a 53.7% increase in children aged <15 years. Coronary artery abnormalities during acute phase were noted in 16.9%, and in 7.2% of patients at 6 weeks of follow-up. The trend analysis indicated a monthly rise of 0.002 cases per 100,000 children aged <5 years, and 0.0165 cases per 100,000 children aged <15 years. Interpretation The incidence of KD has continued to show an upward trend in Chandigarh over the period 2015-2019. This may indicate a true rise in the occurrence of KD or may reflect better disease ascertainment as a result of greater awareness about KD amongst healthcare professionals. Funding None.
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Affiliation(s)
- Rakesh Kumar Pilania
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
| | - Suprit Basu
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
| | - Jyoti Dixit
- Department of Community Medicine and School of Public Health, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
| | - Rajni Kumrah
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
| | - Ankur Kumar Jindal
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
| | - Abarna Thangaraj
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
| | - Ruby Nimesh
- Department of Community Medicine and School of Public Health, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
| | - Taranpreet Kaur
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
| | - Pandiarajan Vignesh
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
| | - Deepti Suri
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
| | - Amit Rawat
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
| | - Sanjeev H. Naganur
- Department of Cardiology, Advanced Cardiac Centre, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
| | - Manphool Singhal
- Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
| | - Shankar Prinja
- Department of Community Medicine and School of Public Health, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
| | - Surjit Singh
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
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Kumrah R, Goyal T, Rawat A, Singh S. Markers of Endothelial Dysfunction in Kawasaki Disease: An Update. Clin Rev Allergy Immunol 2024; 66:99-111. [PMID: 38462555 DOI: 10.1007/s12016-024-08985-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/27/2024] [Indexed: 03/12/2024]
Abstract
Kawasaki disease (KD) is a medium vessel vasculitis that has a special predilection for coronary arteries. Cardiovascular complications include the development of coronary artery abnormalities (CAAs) and myocarditis. Endothelial dysfunction (ED) is now recognized to be a key component in the pathogenesis of KD and is believed to contribute to the development of CAAs. ED has been evaluated by several clinical parameters. However, there is paucity of literature on laboratory markers for ED in KD. The evaluation of ED can be aided by the identification of biomarkers such as oxidative stress markers, circulating cells and their progenitors, angiogenesis factors, cytokines, chemokines, cell-adhesion molecules, and adipokines. If validated in multicentric studies, these biomarkers may be useful for monitoring the disease course of KD. They may also provide a useful predictive marker for the development of premature atherosclerosis that is often a concern during long-term follow-up of KD. This review provides insights into the current understanding of the significance of ED in KD.
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Affiliation(s)
- Rajni Kumrah
- Allergy Immunology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Taru Goyal
- Allergy Immunology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Amit Rawat
- Allergy Immunology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
| | - Surjit Singh
- Allergy Immunology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India
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Reddy Konda VV, Jindal AK, Nadig PL, Banday AZ, Vinay K, Singh S. Microvascular changes on nailfold capillaroscopy in acute stage of Kawasaki disease: a new diagnostic paradigm for an enigmatic condition. Rheumatology (Oxford) 2024; 63:392-398. [PMID: 37202349 DOI: 10.1093/rheumatology/kead213] [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: 12/22/2022] [Revised: 04/01/2023] [Accepted: 04/30/2023] [Indexed: 05/20/2023] Open
Abstract
OBJECTIVES Kawasaki disease (KD) is a medium vessel vasculitis with a predilection to involve coronary arteries. However, there is a paucity of literature on microvascular changes in patients with KD. METHODS Children diagnosed with KD based on American Heart Association guidelines 2017 were enrolled prospectively. Demographic details and echocardiographic changes in coronaries were recorded. Nailfold capillaries were assessed using Optilia Video capillaroscopy and data were analysed using Optilia Optiflix Capillaroscopy software at acute (prior to IVIG administration) and subacute/convalescent phase. RESULTS We enrolled 32 children with KD (17 boys) with a median age of 3 years. Nailfold capillaroscopy (NFC) was performed in 32 patients in the acute phase (compared with 32 controls) and in 17 during the subacute/convalescent phase at a median follow-up of 15 (15-90) days after IVIG treatment. The following findings were seen in NFC in the acute phase of KD: reduced capillary density (n = 12, 38.6%), dilated capillaries (n = 3, 9.3%), ramifications (n = 3, 9.3%) and capillary haemorrhages (n = 2, 6.2%). Capillary density was reduced significantly in the acute phase of KD (38.6%) as compared with the subacute/convalescent phase (25.4%) (P-value <0.001) and controls (0%) (P-value = 0.03). We observed no correlation between coronary artery involvement and mean capillary density (P = 0.870). CONCLUSION Results show that patients with KD have significant nailfold capillary changes in the acute phase. These findings may provide a new diagnostic paradigm for KD and a window to predict coronary artery abnormalities.
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Affiliation(s)
- Vishnu Vardhan Reddy Konda
- Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Ankur Kumar Jindal
- Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Pallavi L Nadig
- Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Aaqib Zaffar Banday
- Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Keshavamurthy Vinay
- Department of Dermatology, Venerology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Surjit Singh
- Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India
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Patra PK, Jindal AK, Rikhi R, Kaur A, Srivastava P, Suri D, Rawat A, Pilania R, Singh S. CD40 gene polymorphism and its expression in children with Kawasaki disease from North India: a preliminary case-control study and meta-analysis. Front Pediatr 2023; 11:1252024. [PMID: 37808562 PMCID: PMC10551130 DOI: 10.3389/fped.2023.1252024] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/03/2023] [Accepted: 08/31/2023] [Indexed: 10/10/2023] Open
Abstract
Introduction CD40 gene single-nucleotide polymorphisms (SNPs) have been associated with susceptibility and development of coronary artery abnormalities (CAAs) in children with Kawasaki disease (KD) in Japanese, Chinese, and Taiwanese populations. However, data on SNPs of the CD40 gene in patients with KD from the Indian subcontinent are not available. We studied the CD40 gene polymorphisms and its expression in children with KD from North India. Methods SNPs of the CD40 gene (rs4810485, rs1535045) were studied using Sanger sequencing. CD40 expression was studied by flow cytometry. Meta-analysis was carried out to assess the role of both SNPs of the CD40 gene in KD. GRADEpro GDT software (v.3.2) was used to assess the "certainty of evidence." Results Forty-one patients with KD and 41 age-, sex-matched febrile controls were enrolled. However, none of the alleles and genotypes of the CD40 gene were found to be associated with KD. CD40 expression was higher in KD and in KD with CAAs compared to controls, but it failed to reach statistical significance. In a meta-analysis, the T allele of rs153045 was found to be significantly associated with KD (OR = 1.28; 95% confidence interval (: 1.09-1.50; p = 0.002). The GRADE of evidence for this outcome, however, is of " very low certainty." Conclusion The present study found no association between SNPs (rs4810485 and rs153045) and susceptibility to KD. This could be a reflection of a modest sample size. CD40 expression was higher in KD and in KD with CAAs. In the meta-analysis, the T allele of rs153045 was significantly associated with KD. Our study confirms a significant genetic heterogeneity in KD among different ethnicities.
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Affiliation(s)
- Pratap Kumar Patra
- Department of Pediatrics, All India Institute of Medical Sciences, Patna, India
| | - Ankur Kumar Jindal
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Rashmi Rikhi
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Anit Kaur
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Priyanka Srivastava
- Genetics and Metabolic Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Deepti Suri
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Amit Rawat
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Rakesh Pilania
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Surjit Singh
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
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Banday AZ, Babbar A, Patra PK, Jindal AK, Suri D, Pandiarajan V, Rawat A, Gupta A, Singh S. Occurrence of Kawasaki disease and neoplasms in temporal proximity-single-center experience and systematic review of literature. J Trop Pediatr 2023; 69:fmad022. [PMID: 37608506 DOI: 10.1093/tropej/fmad022] [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] [Indexed: 08/24/2023]
Abstract
Various factors (e.g., infections) have been postulated to trigger Kawasaki disease (KD) in genetically predisposed individuals. Whether neoplasms can trigger KD is largely unknown due to paucity of data. Herein, we provide a detailed account of KD occurring in temporal proximity (within 6 months) to neoplasms ('neoplasm-KD'). Patients with 'neoplasm-KD' diagnosed/treated at our center from January 1994 to May 2021 were included. Additionally, we performed a systematic literature review (as per PRISMA 2020 guidelines) utilizing PubMed, Web of Science and Scopus databases to retrieve details of all patients with 'neoplasm-KD' reported till June 2021. Patients with multisystem inflammatory syndrome in children were excluded. As all reports pertained to case description(s), risk of bias assessment was not performed. The details of patients with 'neoplasm-KD' were analyzed using SPSS software. Primary and secondary outcomes were occurrence of coronary artery abnormalities (CAAs) and clinical characteristics of 'neoplasm-KD', respectively. A total of 25 patients (data from 18 reports) were included in the 'neoplasm-KD' dataset. The most frequently diagnosed neoplasm was acute lymphoblastic leukemia followed by neuroblastoma and acute myeloblastic leukemia. Overall, CAAs were noted in 48% of patients. Interval between diagnoses of KD and neoplasm was shorter in patients with CAAs as compared to patients with normal coronary arteries (p-value = 0.03). Besides providing a comprehensive description of 'neoplasm-KD', this study raises a possibility that neoplasms might trigger KD. Also, 'neoplasm-KD' may be associated with a higher risk of development of CAAs. However, the small size of 'neoplasm-KD' dataset precludes definitive conclusions regarding this association. Funding: nil. Registration: PROSPERO (CRD42021270458).
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Affiliation(s)
- Aaqib Zaffar Banday
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India
| | - Alisha Babbar
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India
| | - Pratap Kumar Patra
- Allergy Immunology Unit, Department of Pediatrics, All India Institute of Medical Sciences (AIIMS), Patna 801105, India
| | - Ankur Kumar Jindal
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India
| | - Deepti Suri
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India
| | - Vignesh Pandiarajan
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India
| | - Amit Rawat
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India
| | - Anju Gupta
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India
| | - Surjit Singh
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India
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Zhang Y, Wang X, Cai J, Yang Y, Liu Y, Liao Y, Zhou Y, He B, Wen W, Zhuang Q, Lin Y. Status and influencing factors of medication literacy among Chinese caregivers of discharged children with Kawasaki disease. Front Public Health 2022; 10:960913. [PMID: 36324474 PMCID: PMC9618952 DOI: 10.3389/fpubh.2022.960913] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2022] [Accepted: 10/03/2022] [Indexed: 01/24/2023] Open
Abstract
Background The information on medication literacy among Chinese caregivers of discharged children with Kawasaki disease (KD) is unknown. We aimed to investigate the status of medication literacy among caregivers of discharged children with KD and evaluate the influencing factors of medication literacy. Methods From March 2020 to February 2021, 106 caregivers with a KD child were recruited for the present study. We collected the sociodemographic characteristics of the KD caregivers using structured interviews. The medication literacy of the KD caregivers was assessed by the Chinese version of Medication Literacy Assessment. KD patients' demographic and clinical data were obtained from the medical records. The multiple logistic regression was performed to identify factors associated with medication literacy. Results (1) The average medication literacy score was 4.91 ± 1.51. (2) Most of the Chinese KD caregivers had insufficient medication literacy (≤ 5 scores), and only 39.2% of the caregivers had adequate medication literacy (>5 scores). (3) The multiple logistic regression shows that education level, monthly income, and duration of hospitalization are the independent influencing factors on the medication literacy of KD caregivers. Conclusion There is preliminary evidence that medication literacy among KD caregivers is low and needs improvement. A higher level of education, higher income, and longer duration of hospitalization were influencing factors of adequate medication literacy.
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Affiliation(s)
- Yingzi Zhang
- Department of Pediatric Cardiology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
| | - Xiuqiong Wang
- Department of Pediatric Cardiology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
| | - Jianghui Cai
- Department of Pharmacy, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
| | - Yanfeng Yang
- Department of Pediatric Cardiology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China,*Correspondence: Yanfeng Yang
| | - Yiling Liu
- Department of Pediatric Cardiology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
| | - Yeling Liao
- Department of Pediatric Cardiology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
| | - Yanhong Zhou
- Department of Pediatric Cardiology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
| | - Baoqin He
- Department of Pediatric Cardiology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
| | - Wen Wen
- Department of Pediatric Cardiology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
| | - Qian Zhuang
- Department of Pediatric Cardiology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
| | - Yonghong Lin
- Department of Obstetrics and Gynecology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
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Association of ITPKC gene polymorphisms rs28493229 and rs2290692 in North Indian children with Kawasaki disease. Pediatr Res 2022; 92:1090-1098. [PMID: 34952936 DOI: 10.1038/s41390-021-01830-x] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/30/2021] [Revised: 08/18/2021] [Accepted: 10/18/2021] [Indexed: 12/17/2022]
Abstract
BACKGROUND Single-nucleotide polymorphisms (SNPs) of several genes are linked to the etiopathogenesis of Kawasaki disease (KD). Association of SNPs of inositol 1,4,5-triphosphate-3-kinase C (ITPKC) gene with susceptibility to KD and coronary artery lesions (CALs) has been observed in children of certain ethnicities, but not from others. The present study was planned to explore this genetic association in the North Indian cohort. METHODS Fifty children with KD and 50 age- and sex-matched controls were studied for two SNPs (rs28493229 and rs2290692) of the ITPKC gene using polymerase chain reaction and restriction fragment length polymorphism. Findings were confirmed by Sanger sequencing. A meta-analysis was also carried out for GG and CC genotypes of the SNPs. RESULTS There was significant association between KD susceptibility and CG + GG genotype of rs2290692 (p = 0.015, odds ratio = 4.1, 95% confidence interval = 1.38-13.83). None of the single alleles or genotypes of the SNPs of ITPKC were, however, significantly associated with KD susceptibility. A meta-analysis also did not show any significant association of these SNPs to KD susceptibility. CONCLUSIONS Our findings suggest that ITPKC gene SNPs (rs28493229 and rs2290692) did not have a significant association with susceptibility to KD in children from North India. Larger multicentric studies incorporating different ethnicities are required to understand the genetic basis of KD. IMPACT While SNP rs28493229 of the ITPKC gene is not found to be associated with susceptibility to KD, the combined genotype of SNP rs2290692 is shown to be associated. Impact of ITPKC gene SNP on KD is different across different races and ethnicities. We could find an association of the combined genotype of rs2290692 with it in the Indian population. This study highlights that phenotype and genotypic association of KD varies with ethnicities. Larger multicentric studies are required to reach a conclusion regarding the genetic association of KD.
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11
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Praharaj DL, Rawat A, Gupta A, Arora K, Pilania RK, Bhattad S, Singh S. Adipocytokine profile in children with Kawasaki disease at a mean follow-up period of 5.5 years: A study from North India. World J Clin Pediatr 2022; 11:360-368. [PMID: 36052116 PMCID: PMC9331403 DOI: 10.5409/wjcp.v11.i4.360] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/31/2021] [Revised: 08/21/2021] [Accepted: 05/12/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Kawasaki disease (KD) is an acute self-limited vasculitis with a predilection for coronary arteries. Children with KD may have altered lipid metabolism and abnormal lipid profiles that may last for prolonged periods. However, there is a paucity of literature on the role of adipocytokines in KD. AIM To estimate the levels of adipocytokines (adiponectin, leptin and resistin) during the convalescent phase of KD. METHODS Twenty children, who had KD at least three years earlier, were enrolled in this study. In addition, 20 healthy controls were also enrolled. Clinical and laboratory profiles of patients were obtained from hospital records. Serum adiponectin, leptin and resistin levels were estimated by enzyme-linked immunosorbent assay. RESULTS Mean age of the patients in the study group was 10.15 ± 3 years and the male: female ratio was 1.5:1. Median serum resistin levels in patients with KD (27.77 ng/mL; [IQR: 18.66, 48.90]) were decreased compared to controls (21.20 ng/mL; [IQR: 14.80, 27.00]) (P = 0.04). Median serum leptin levels in cases and controls were 1.83 ng/mL; (IQR: 1.13, 3.80), and 1.10 ng/mL; (IQR: 0.41, 2.88), respectively (P = 0.09). Median serum adiponectin levels were similar in both cases (12.20 µg/mL; [IQR: 9.76, 17.97]) and controls (13.95 µg/mL; [IQR: 11.17, 22.58]); (P = 0.18). There was no significant difference in all 3 adipocytokines between children with (4/20) and without coronary artery abnormalities (16/20). CONCLUSION Serum resistin levels were significantly elevated in patients with KD during the convalescent phase compared to controls. Serum leptin levels appeared to be higher in patients with KD, although the difference was not statistically significant. Adiponectin levels were similar in both cases and controls. Raised resistin and leptin levels may partially explain lipid perturbations observed during the convalescent phase of KD.
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Affiliation(s)
- Dibya Lochan Praharaj
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Amit Rawat
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Anju Gupta
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Kanika Arora
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Rakesh Kumar Pilania
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Sagar Bhattad
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Surjit Singh
- Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
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12
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Abstract
The many forms of vasculitis are characterized by inflammation of blood vessels, leading to potentially long-term sequelae including vision loss, aneurysm formation and kidney failure. Accurate estimation of the incidence and prevalence has been hampered by the absence of reliable diagnostic criteria and the rarity of these conditions; however, much progress has been made over the past two decades, although data are still lacking from many parts of the world including the Indian subcontinent, China, Africa and South America. Giant cell arteritis occurs in those aged 50 years and over and seems to mainly affect persons of northern European ancestry, whereas Takayasu arteritis occurs mainly in those aged under 40 years. By contrast, Kawasaki disease mainly occurs in children aged under 5 years and is most common in children of Asian ancestry, and IgA vasculitis occurs in children and adolescents. Although much less common than giant cell arteritis, the different forms of antineutrophil cytoplasmic antibody-associated vasculitis are being increasingly recognized in most populations and occur more frequently with increasing age. Behçet syndrome occurs most commonly along the ancient silk road between Europe and China. Much work needs to be done to better understand the influence of ethnicity, geographical location, environment and social factors on the development of vasculitis.
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Affiliation(s)
- Richard A Watts
- Norwich Medical School, University of East Anglia, Norwich, UK.
| | - Gulen Hatemi
- Department of Internal Medicine, Division of Rheumatology and Behçet's Disease Research Centre, Istanbul University-Cerrahpaşa, Istanbul, Turkey
| | - Jane C Burns
- Department of Pediatrics, University of California San Diego, La Jolla, CA, USA
| | - Aladdin J Mohammad
- Department of Clinical Sciences, Division of Rheumatology, Lund University and Department of Rheumatology, Skåne University Hospital, Lund, Sweden
- Department of Medicine, University of Cambridge, Cambridge, UK
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13
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Selamet Tierney ES, Runeckles K, Tremoulet AH, Dahdah N, Portman MA, Mackie AS, Harahsheh AS, Lang SM, Choueiter NF, Li JS, Manlhiot C, Low T, Mathew M, Friedman KG, Raghuveer G, Norozi K, Szmuszkovicz JR, McCrindle BW. Variation in Pharmacologic Management of Patients with Kawasaki Disease with Coronary Artery Aneurysms. J Pediatr 2022; 240:164-170.e1. [PMID: 34474088 DOI: 10.1016/j.jpeds.2021.08.072] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/16/2021] [Revised: 07/23/2021] [Accepted: 08/24/2021] [Indexed: 11/17/2022]
Abstract
OBJECTIVE To evaluate practice variation in pharmacologic management in the International Kawasaki Disease Registry (IKDR). STUDY DESIGN Practice variation in intravenous immunoglobulin (IVIG) therapy, anti-inflammatory agents, statins, beta-blockers, antiplatelet therapy, and anticoagulation was described. RESULTS We included 1627 patients from 30 IKDR centers with maximum coronary artery aneurysm (CAA) z scores 2.5-4.99 in 848, 5.0-9.99 in 349, and ≥10.0 (large/giant) in 430 patients. All centers reported IVIG and acetylsalicylic acid (ASA) as primary therapy and use of additional IVIG or steroids as needed. In 23 out of 30 centers, (77%) infliximab was also used; 11 of these 23 centers reported using it in <10% of their patients, and 3 centers used it in >20% of patients. Nonsteroidal anti-inflammatory agents were used in >10% of patients in only nine centers. Beta-blocker (8.8%, all patients) and abciximab (3.6%, all patients) were mainly prescribed in patients with large/giant CAAs. Statins (2.7%, all patients) were mostly used in one center and only in patients with large/giant CAAs. ASA was the primary antiplatelet modality for 99% of patients, used in all centers. Clopidogrel (18%, all patients) was used in 24 centers, 11 of which used it in >50% of their patients with large/giant CAAs. CONCLUSIONS In the IKDR, IVIG and ASA therapy as primary therapy is universal with common use of a second dose of IVIG for persistent fever. There is practice variation among centers for adjunctive therapies and anticoagulation strategies, likely reflecting ongoing knowledge gaps. Randomized controlled trials nested in a high-quality collaborative registry may be an efficient strategy to reduce practice variation.
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Affiliation(s)
- Elif Seda Selamet Tierney
- Division of Pediatric Cardiology, Department of Pediatrics, Stanford University, School of Medicine, Palo Alto, CA.
| | - Kyle Runeckles
- Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
| | - Adriana H Tremoulet
- Department of Pediatrics, University of California San Diego, Rady Children's Hospital-San Diego, San Diego, CA
| | - Nagib Dahdah
- Division of Pediatric Cardiology, Centre Hospitalier Universitaire Ste-Justine, University of Montreal, Montreal, Quebec, Canada
| | | | | | - Ashraf S Harahsheh
- Pediatrics-Cardiology, Children's National Hospital/George Washington University School of Medicine, Washington, DC
| | - Sean M Lang
- Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
| | | | | | - Cedric Manlhiot
- Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
| | - Tisiana Low
- Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
| | - Mathew Mathew
- Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
| | | | | | - Kambiz Norozi
- Department of Pediatrics, Western University, London, Canada
| | | | - Brian W McCrindle
- Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
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Behera JR, Rup AR, Dash AK, Jain MK, Sahu SK, Polei R. Kawasaki Disease: A 3 Years' Experience from a Single Center. Indian J Pediatr 2021; 88:950-951. [PMID: 34215945 DOI: 10.1007/s12098-021-03849-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/15/2021] [Accepted: 06/07/2021] [Indexed: 10/21/2022]
Affiliation(s)
- Jyoti Ranjan Behera
- Department of Pediatrics, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, 751024, India
| | - Amit Ranjan Rup
- Department of Pediatrics, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, 751024, India
| | - Arun Kumar Dash
- Department of Pediatrics, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, 751024, India
| | - Mukesh Kumar Jain
- Department of Pediatrics, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, 751024, India
| | - Sanjay Kumar Sahu
- Department of Pediatrics, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, 751024, India.
| | - Rasananda Polei
- Department of Pediatrics, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, 751024, India
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Banday AZ, Mondal S, Barman P, Sil A, Kumrah R, Vignesh P, Singh S. What Lies Ahead for Young Hearts in the 21 st Century - Is It Double Trouble of Acute Rheumatic Fever and Kawasaki Disease in Developing Countries? Front Cardiovasc Med 2021; 8:694393. [PMID: 34250047 PMCID: PMC8263915 DOI: 10.3389/fcvm.2021.694393] [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: 04/13/2021] [Accepted: 06/01/2021] [Indexed: 12/19/2022] Open
Abstract
Rheumatic heart disease (RHD), the principal long-term sequel of acute rheumatic fever (ARF), has been a major contributor to cardiac-related mortality in general population, especially in developing countries. With improvement in health and sanitation facilities across the globe, there has been almost a 50% reduction in mortality rate due to RHD over the last 25 years. However, recent estimates suggest that RHD still results in more than 300,000 deaths annually. In India alone, more than 100,000 deaths occur due to RHD every year (Watkins DA et al., N Engl J Med, 2017). Children and adolescents (aged below 15 years) constitute at least one-fourth of the total population in India. Besides, ARF is, for the most part, a pediatric disorder. The pediatric population, therefore, requires special consideration in developing countries to reduce the burden of RHD. In the developed world, Kawasaki disease (KD) has emerged as the most important cause of acquired heart disease in children. Mirroring global trends over the past two decades, India also has witnessed a surge in the number of cases of KD. Similarly, many regions across the globe classified as "high-risk" for ARF have witnessed an increasing trend in the incidence of KD. This translates to a double challenge faced by pediatric health care providers in improving cardiac outcomes of children affected with ARF or KD. We highlight this predicament by reviewing the incidence trends of ARF and KD over the last 50 years in ARF "high-risk" regions.
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Affiliation(s)
| | | | | | | | | | - Pandiarajan Vignesh
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
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16
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Datta AK, Debnath P, Chaudhuri J, Mukherjee A. Adult-onset Kawasaki disease: A great masquerader. BMJ Case Rep 2021; 14:14/3/e239746. [PMID: 33727292 PMCID: PMC7970249 DOI: 10.1136/bcr-2020-239746] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022] Open
Abstract
Kawasaki disease is a necrotising small-to-medium vessel vasculitis affecting children between age groups of 6 months and 5 years. Following the first description in Japanese infants, it has been recognised as the single most common cause of non-infectious vasculitis in children worldwide. Presentation in adult age groups, although described, is rare. Herein, we report a case about a 19-year-old female Indian patient diagnosed with Kawasaki disease and managed with antiplatelets and intravenous immunoglobulin, without further sequalae. We aim to highlight the importance of recognising this entity in adult age groups in day-to-day clinical practice.
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Affiliation(s)
- Amlan Kusum Datta
- Department of Neurology, Bangur Institute of Neurology, Institute of Postgraduate Medical Education and Research, Kolkata, India
| | - Partha Debnath
- Internal Medicine, RG Kar Medical College and Hospital, Kolkata, India
| | - Jasodhara Chaudhuri
- Department of Neurology, Bangur Institute of Neurology, Institute of Postgraduate Medical Education and Research, Kolkata, India
| | - Adreesh Mukherjee
- Department of Neurology, Bangur Institute of Neurology, Institute of Postgraduate Medical Education and Research, Kolkata, India
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17
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Vignesh P, Rawat A, Shandilya JK, Singh Sachdeva MU, Ahluwalia J, Singh S. Monocyte platelet aggregates in children with Kawasaki disease- a preliminary study from a tertiary care centre in North-West India. Pediatr Rheumatol Online J 2021; 19:25. [PMID: 33712020 PMCID: PMC7953550 DOI: 10.1186/s12969-021-00515-3] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/30/2020] [Accepted: 03/04/2021] [Indexed: 11/26/2022] Open
Abstract
BACKGROUND Platelet activation is an integral part of pathogenesis of Kawasaki disease (KD). However, there is paucity of literature on flow-cytometry based assessment of platelet activation in KD. We aimed to analyse monocyte-platelet aggregates (MPAs), one of the sensitive markers for platelet activation, by flow cytometry in children with KD. FINDINGS In this single-centre prospective study, we have enrolled 14 children with KD and results were compared with age-matched febrile (n = 15) and healthy (n = 13) controls. After gating monocytes in side-scatter plot, MPAs were identified based on CD14 and CD41 expression. Two (2) ml of blood samples for children with KD were collected at 3 phases of illness- acute stage before start of intravenous immunoglobulin or aspirin, 24 h after completion of IVIg infusion, and 3 months after acute episode of KD. Children with KD had a significantly higher MPA% values [Median (IQR)- 41.3% (26.6, 52.7)] when compared with febrile [Median (IQR)- 5.98% (2.98-9.72)] and normal [Median (IQR)- 4.48% (2.57-5.59)] controls, p<0.01. On follow-up, the MPA% showed a gradual decline in children with KD, but even at 3 months, the value [Median (IQR)- 7.55% (4.15-14.6)] was higher compared to healthy controls [Median (IQR)- 4.48% (2.57-5.59)]. CONCLUSIONS Our results suggest that MPA% was significantly elevated in acute stages in children with KD and activated platelets may continue to persist even after systemic inflammation has subsided. Future studies are warranted whether objective evidence of platelet activation may guide the use of immunomodulatory and anti-platelet therapy in KD.
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Affiliation(s)
- Pandiarajan Vignesh
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, 160012, Chandigarh, India.
| | - Amit Rawat
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, 160012, Chandigarh, India
| | - Jitendra Kumar Shandilya
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, 160012, Chandigarh, India
| | - Man Updesh Singh Sachdeva
- Department of Hematology, Postgraduate Institute of Medical Education and Research, 160012, Chandigarh, India
| | - Jasmina Ahluwalia
- Department of Hematology, Postgraduate Institute of Medical Education and Research, 160012, Chandigarh, India
| | - Surjit Singh
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, 160012, Chandigarh, India.
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18
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Bhattad S, Gupta S, Israni N, Mohanty S. Profile of Kawasaki disease at a tertiary care center in India. Ann Pediatr Cardiol 2021; 14:187-191. [PMID: 34103858 PMCID: PMC8174629 DOI: 10.4103/apc.apc_57_20] [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: 04/03/2020] [Revised: 05/21/2020] [Accepted: 12/06/2020] [Indexed: 11/04/2022] Open
Abstract
Background Kawasaki disease (KD) is the most common cause of acquired heart disease in developed countries. KD is increasingly being reported from India; however, studies involving the large number of patients are few. Methods All children presenting to the center from January 2017 to December 2019, diagnosed to have KD, were retrospectively included in the study. Clinical and laboratory profiles, including echocardiograms, were reviewed. Factors contributing to intravenous immunoglobulin (IVIg) refractoriness and the development of coronary artery abnormalities (CAA) were assessed. Results A total of 39 children with KD presented to the center during the study. While 32 received initial treatment at our center, seven were referred after the initial IVIg infusion. The age range was 2 months to 11 years (mean 42.15 ± 38.51 months). More than two-thirds of the cohort was male (n = 27/39). Mucosal involvement was the commonest clinical abnormality for the group, followed by rash. Hemoglobin was significantly lower in the group with coronary artery involvement (P = 0.001). CAA (61.5%), incomplete KD, and atypical features were much more common in infants compared to the rest. Refractoriness to treatment was significantly more common in infants (P = 0.029). Conclusions A significant proportion of infants with KD had cardiac involvement. Infants were more likely to have IVIg-resistant disease.
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Affiliation(s)
- Sagar Bhattad
- Department of Pediatrics, Division of Pediatric Rheumatology and Immunology, Aster CMI Hospital, Bengaluru, Karnataka, India
| | - Sandip Gupta
- Department of Pediatrics, Division of Pediatric Intensive Care, Aster CMI Hospital, Bengaluru, Karnataka, India
| | - Neha Israni
- Department of Pediatrics, Aster CMI Hospital, Bengaluru, Karnataka, India
| | - Sweta Mohanty
- Department of Cardiac Sciences, Division of Pediatric Cardiology, Aster CMI Hospital, Bengaluru, Karnataka, India
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19
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Sahu S, Behera J, Rup A, Dash A, Jain M, Swain N, Polei R. Clinical manifestations and outcomes of Kawasaki Disease: A retrospective hospital-based data from Eastern India. INDIAN JOURNAL OF RHEUMATOLOGY 2021. [DOI: 10.4103/injr.injr_150_21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
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20
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Shenoy B, Singh S, Ahmed MZ, Pal P, Balan S, Viswanathan V, Bhattad S, Rao AP, Chaudhuri M, Shastri DD, Soans ST. Indian Academy of Pediatrics Position Paper on Kawasaki Disease. Indian Pediatr 2020; 57:1040-1048. [PMID: 32471961 DOI: 10.1007/s13312-020-2033-1] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
OBJECTIVE To formulate practice guidelines on diagnosis and management of Kawasaki disease (KD) for Indian children. JUSTIFICATION KD is a systemic vasculitis that predominantly affects infants and children less than 5 years of age. Coronary artery abnormalities (CAA) develop in around 15-25% of untreated children with KD. Coronary artery involvement can lead to long-term cardiovascular implications such as development of premature coronary artery disease. Diagnosis of KD is essentially clinical based on recognition of a constellation of characteristic symptoms and signs. Timely diagnosis and initiation of intravenous immunoglobulin (IVIG) therapy is known to produce five-fold reduction in the incidence of CAA. As there is no confirmatory laboratory test for KD, the diagnosis may be missed if one is not familiar with the nuances of clinical diagnosis. PROCESS A committee was formed under the auspices of Indian Academy of Pediatrics in early 2018 for preparing guidelines on KD in Indian children. A meeting of the consultative committee was held in Mumbai, and a draft protocol was devised. All members scrutinized the recent publications on the subject and an attempt was made to arrive at a broad consensus. Published guidelines on the subject were also reviewed. RECOMMENDATIONS The diagnosis is clinical and is aided by laboratory and 2D echocardiography. First line of therapy is IVIG, and should be started expeditiously once the diagnosis is made.
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Affiliation(s)
- Bhaskar Shenoy
- Department of Pediatrics, Manipal Hospitals, Bangalore, Karnataka, India. Correspondence to: Dr Bhaskar Shenoy, Head, Department of Pediatrics, Manipal Hospitals, Bangalore, Karnataka, India.
| | - Surjit Singh
- Advanced Pediatric Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - M Zulfikar Ahmed
- Department of Cardiology, Pushpagiri Medical College, Tiruvalla, Kerala, India
| | - Priyankar Pal
- Department of Pediatric Rheumatology Institute of Child Health, Kolkata, West Bengal, India
| | - Suma Balan
- Department of Rheumatology, Amrita Institute of Medical Sciences, Kochi, Kerala, India
| | | | | | - Anand P Rao
- Manipal Hospitals, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India
| | - Maitri Chaudhuri
- Department of Cardiology, Manipal Hospital, Bangalore, Karnataka, India
| | | | - Santosh T Soans
- AJ Institute of Medical Sciences, Mangalore, Karnataka, India
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21
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Abstract
Kawasaki disease (KD) is a medium vessel vasculitis that affects young children. Despite extensive research over the last 50 years, the etiology of KD remains an enigma. Seasonal change in wind patterns was shown to have correlation with the epidemics of KD in Japan. Occurrence of disease in epidemiological clusters, seasonal variation, and a very low risk of recurrence suggest that KD is triggered by an infectious agent. The identification of oligoclonal IgA response in the affected tissues suggests an antigen-driven inflammation. The recent identification of a viral antigen in the cytoplasm of bronchial ciliated epithelium also favors infection as the main trigger for KD. Pointers that suggest a genetic basis of KD include a high disease prevalence in North-East Asian populations, a high risk among siblings, and familial occurrence of cases. Dysregulated innate and adaptive immune responses are evident in the acute stages of KD. In addition to the coronary wall inflammation, endothelial dysfunction and impaired vascular remodeling contribute to the development of coronary artery abnormalities (CAAs) and thrombosis. Genetic aberrations in certain intracellular signaling pathways involving immune effector functions are found to be associated with increased susceptibility to KD and development of coronary artery abnormalities (CAAs). Several susceptible genes have been identified through genome-wide association studies (GWAS) and linkage studies (GWLS). The genes that are studied in KD can be classified under 4 major groups-enhanced T cell activation (ITPKC, ORAI1, STIM1), dysregulated B cell signaling (CD40, BLK, FCGR2A), decreased apoptosis (CASP3), and altered transforming growth factor beta signaling (TGFB2, TGFBR2, MMP, SMAD). The review aims to highlight the role of several genetic risk factors that are linked with the increased susceptibility to KD.
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Affiliation(s)
- Rajni Kumrah
- Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India
| | - Pandiarajan Vignesh
- Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
| | - Amit Rawat
- Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India
| | - Surjit Singh
- Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India
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22
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Jindal AK, Pilania RK, Guleria S, Vignesh P, Suri D, Gupta A, Singhal M, Rawat A, Singh S. Kawasaki Disease in Children Older Than 10 Years: A Clinical Experience From Northwest India. Front Pediatr 2020; 8:24. [PMID: 32117831 PMCID: PMC7034337 DOI: 10.3389/fped.2020.00024] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/07/2019] [Accepted: 01/17/2020] [Indexed: 12/25/2022] Open
Abstract
Background: Kawasaki disease (KD) is predominantly seen in young children (<5 years). Diagnosis of KD is often delayed in older children and adolescents, leading to a higher risk of coronary artery abnormalities (CAAs). There is a paucity of literature on KD in older children. Methods: Data were collated from a review of records of patients diagnosed with KD who were aged ≥10 years at the time of diagnosis, during the period from January 1994 to June 2019. Results: Eight hundred and sixty five patients were diagnosed with KD during this period. Of these, 46 (5.3%; 26 boys and 20 girls) were aged 10 years or older at the time of diagnosis. The median age at diagnosis was 11 years (range of 10-30 years). The median interval between the of fever and the diagnosis of KD was 12 days (range of 4-30 days). Eight patients (17.4%) presented with hypotensive shock. Coronary artery abnormalities (CAAs) were seen in six patients (13.04%), and three patients had myocarditis. Patients with CAAs were found to have significantly higher median platelet counts and higher median C-reactive protein levels. First-line treatment included intravenous immunoglobulin. Adjunctive therapy was given in five patients (infliximab in four patients and steroids in one patient). The median time between the onset of fever and the administration of IVIg was 13.5 days (range of 6-2). The total duration of follow up is 2,014.5 patient-months. Conclusion: Diagnosis of KD in children older than 10 years is usually delayed, and these patients are thus at a higher risk of CAAs.
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Affiliation(s)
- Ankur Kumar Jindal
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Rakesh Kumar Pilania
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Sandesh Guleria
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Pandiarajan Vignesh
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Deepti Suri
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Anju Gupta
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Manphool Singhal
- Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Amit Rawat
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Surjit Singh
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
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Elakabawi K, Lin J, Jiao F, Guo N, Yuan Z. Kawasaki Disease: Global Burden and Genetic Background. Cardiol Res 2020; 11:9-14. [PMID: 32095191 PMCID: PMC7011927 DOI: 10.14740/cr993] [Citation(s) in RCA: 41] [Impact Index Per Article: 8.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/20/2019] [Accepted: 12/05/2019] [Indexed: 12/14/2022] Open
Abstract
Kawasaki disease (KD) is a childhood vasculitides associated with serious coronary artery lesions. It is the most common cause of pediatric acquired heart disease in developed countries, and is increasingly reported from many rapidly industrializing developing countries. The incidence varies widely among different nations and is highest in North-East Asian countries, where almost 1 in 100 children in Japan having the disease by age of 5, where the lowest incidence reported in sub-Saharan Africa. The etiology of KD is still uncertain; interaction between a genetic predisposition and several environmental and immunological factors has been hypothesized. Several susceptibility genes were identified to be associated with the development of KD and increased risk of coronary artery lesions. Gene-gene associations and alteration of deoxyribonucleic acid (DNA) methylation are also found to play key roles in the pathogenesis and prognosis of KD. This article will focus on the global epidemiological patterns of KD, and the currently known genetic predisposition.
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Affiliation(s)
- Karim Elakabawi
- Cardiovascular Department, First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi 710061, China.,Cardiovascular Department, Benha University, Benha 13518, Egypt.,These two authors contributed equally
| | - Jing Lin
- Department of Child and Adolescent Health Science Center, School of Public Health, Xi'an Jiaotong University, Xi'an, Shaanxi 710061, China.,These two authors contributed equally
| | - Fuyong Jiao
- Children's Hospital, Shaanxi Provincial People's Hospital, Xi'an, Shaanxi 710061, China
| | - Ning Guo
- Cardiovascular Department, First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi 710061, China
| | - Zuyi Yuan
- Cardiovascular Department, First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi 710061, China
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24
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Affiliation(s)
- Rolando Cimaz
- Department of Clinical Sciences and Community Health, University of Milano, Milano, Italy
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25
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Sakina S, Owais SS, Khan EA, Sheikh AM. Kawasaki disease: Clinico-laboratory spectrum and outcome in a cohort of children treated at a tertiary care hospital in Islamabad, Pakistan. Pak J Med Sci 2019; 36:260-264. [PMID: 32063971 PMCID: PMC6994906 DOI: 10.12669/pjms.36.2.910] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022] Open
Abstract
Objective: To describe the demographics; clinical, laboratory, echocardiographic findings; treatment and outcome in a cohort of children with Kawasaki disease in a tertiary care hospital. Methods: This is a descriptive, observational, retrospective cohort study conducted at Shifa International Hospital, Islamabad, from January 2013-June 2019. Children who met the criteria for Kawasaki disease according to the American Heart Association and American Academy of Pediatrics guidelines were included. Results: A total of 25 children who met the criteria of Kawasaki disease were included. Their mean age was 43 months (4-150 months). Majority (76%) were males. Eighteen (72%) had complete Kawasaki disease and 7 (28%) had incomplete Kawasaki disease. Fever (> five days) was present in 20 (80%) patients. Eight patients (32%) had echocardiographic changes, out of which two patients (25%) had complete Kawasaki disease and six patients (75%) had incomplete Kawasaki disease. Intravenous immunoglobulin was given to all patients. Fifteen children (60%) received intravenous immunoglobulin within 10 days of fever. None required a second dose. All patients received high dose aspirin at diagnosis which was reduced to antiplatelet dose after resolution of fever for ≥48hrs. Eighteen patients (72%) came for regular follow up. Follow up at 6 months showed complete resolution of echocardiographic changes in six patients (75%), 1 (12.5%) was lost to follow up and one (12.5%) child had persistent coronary artery dilatation. Conclusion: Complete KD was present in 72% of our cohort of children. Coronary artery abnormalities were present in one third of these children, at younger age and more common in those with incomplete KD but recovered in most.
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Affiliation(s)
- Seema Sakina
- Seema Sakina, MBBS. Post Graduate Resident, Department of Pediatrics, Shifa International Hospital and Shifa Tameer-e-Millat University, H-8/4, Islamabad, Pakistan
| | - Syeda Sobya Owais
- Syeda Sobya Owais, MD. Associate Consultant Pediatrician, Emergency Department, Shifa International Hospital and Shifa Tameer-e-Millat University, H-8/4, Islamabad, Pakistan
| | - Ejaz Ahmed Khan
- Ejaz Ahmed Khan, MD. Chief of Pediatrics, Shifa International Hospital and Shifa Tameer-e-Millat University, H-8/4, Islamabad, Pakistan
| | - Abdul Malik Sheikh
- Abdul Malik Sheikh, FCPS. Associate Consultant Pediatric Cardiologist. Shifa International Hospital and Shifa Tameer-e-Millat University, H-8/4, Islamabad, Pakistan
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Gopalan K, Singh S, Vignesh P, Gupta A, Rohit M, Attri SV. Carotid Intima-Media Thickness and Lipid Profile in Children With Kawasaki Disease: A Single-Center Follow-up Study After a Mean Duration of 6.9 Years. J Clin Rheumatol 2018; 24:385-389. [PMID: 29538084 DOI: 10.1097/rhu.0000000000000754] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
BACKGROUND Kawasaki disease (KD) has a predilection to involve coronary arteries, leading to several long-term cardiovascular sequelae. Apart from coronary artery abnormalities, children with KD are also prone to develop premature atherosclerosis, endothelial dysfunction, and lipid abnormalities. Some of these complications may occur even in children who have received appropriate treatment with intravenous immunoglobulin in the acute phase. METHODS In 2009, we had studied carotid intima-media thickness (cIMT) and lipid profile in 27 children with KD at least 1 year after the acute episode. In the present study, we have followed up the same cohort of 27 children at least 5 years after the acute episode of KD. We measured the cIMT, a surrogate marker for premature atherosclerosis, and fasting lipid profile in the cohort and compared the results with values obtained in our previous study. RESULTS There was significantly higher mean cIMT in children with KD as compared with control subjects. However, there was no significant difference in cIMT among children in the cohort at 1 and 5 years of follow-up. Abnormal lipid profile was seen in 7 of 27 children in the present study, 5 of whom also had had lipid abnormality at 1-year follow-up. This suggests that lipid abnormalities in KD may be long lasting. CONCLUSIONS Children with KD need careful long-term follow-up even when they do not have overt and persistent coronary artery abnormalities. It is possible that consequences of KD in childhood may impact health status of young adults several years later.
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Affiliation(s)
- Kavitha Gopalan
- From the Allergy Immunology Unit and the Division of Pediatric Biochemistry, Advanced Pediatrics Centre, and
| | - Surjit Singh
- From the Allergy Immunology Unit and the Division of Pediatric Biochemistry, Advanced Pediatrics Centre, and
| | - Pandiarajan Vignesh
- From the Allergy Immunology Unit and the Division of Pediatric Biochemistry, Advanced Pediatrics Centre, and
| | - Anju Gupta
- From the Allergy Immunology Unit and the Division of Pediatric Biochemistry, Advanced Pediatrics Centre, and
| | - Manojkumar Rohit
- Department of Cardiology, Postgraduate Institute of Medical Education & Research, Chandigarh, India
| | - Savita Verma Attri
- From the Allergy Immunology Unit and the Division of Pediatric Biochemistry, Advanced Pediatrics Centre, and
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Marchesi A, Tarissi de Jacobis I, Rigante D, Rimini A, Malorni W, Corsello G, Bossi G, Buonuomo S, Cardinale F, Cortis E, De Benedetti F, De Zorzi A, Duse M, Del Principe D, Dellepiane RM, D'Isanto L, El Hachem M, Esposito S, Falcini F, Giordano U, Maggio MC, Mannarino S, Marseglia G, Martino S, Marucci G, Massaro R, Pescosolido C, Pietraforte D, Pietrogrande MC, Salice P, Secinaro A, Straface E, Villani A. Kawasaki disease: guidelines of the Italian Society of Pediatrics, part I - definition, epidemiology, etiopathogenesis, clinical expression and management of the acute phase. Ital J Pediatr 2018; 44:102. [PMID: 30157897 PMCID: PMC6116535 DOI: 10.1186/s13052-018-0536-3] [Citation(s) in RCA: 67] [Impact Index Per Article: 9.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/23/2017] [Accepted: 05/03/2018] [Indexed: 12/18/2022] Open
Abstract
The primary purpose of these practical guidelines related to Kawasaki disease (KD) is to contribute to prompt diagnosis and appropriate treatment on the basis of different specialists' contributions in the field. A set of 40 recommendations is provided, divided in two parts: the first describes the definition of KD, its epidemiology, etiopathogenetic hints, presentation, clinical course and general management, including treatment of the acute phase, through specific 23 recommendations.Their application is aimed at improving the rate of treatment with intravenous immunoglobulin and the overall potential development of coronary artery abnormalities in KD. Guidelines, however, should not be considered a norm that limits treatment options of pediatricians and practitioners, as treatment modalities other than those recommended may be required as a result of peculiar medical circumstances, patient's condition, and disease severity or complications.
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Affiliation(s)
- Alessandra Marchesi
- Bambino Gesù Children's Hospital, Rome, Italy, Piazza S. Onofrio n. 4, 00165, Rome, Italy.
| | | | - Donato Rigante
- Fondazione Policlinico Universitario A. Gemelli, Università Cattolica Sacro Cuore, Rome, Italy
| | | | | | | | | | - Sabrina Buonuomo
- Bambino Gesù Children's Hospital, Rome, Italy, Piazza S. Onofrio n. 4, 00165, Rome, Italy
| | | | | | - Fabrizio De Benedetti
- Bambino Gesù Children's Hospital, Rome, Italy, Piazza S. Onofrio n. 4, 00165, Rome, Italy
| | - Andrea De Zorzi
- Bambino Gesù Children's Hospital, Rome, Italy, Piazza S. Onofrio n. 4, 00165, Rome, Italy
| | - Marzia Duse
- , Università degli Studi Sapienza, Rome, Italy
| | | | | | | | - Maya El Hachem
- Bambino Gesù Children's Hospital, Rome, Italy, Piazza S. Onofrio n. 4, 00165, Rome, Italy
| | | | | | - Ugo Giordano
- Bambino Gesù Children's Hospital, Rome, Italy, Piazza S. Onofrio n. 4, 00165, Rome, Italy
| | | | | | | | | | - Giulia Marucci
- Bambino Gesù Children's Hospital, Rome, Italy, Piazza S. Onofrio n. 4, 00165, Rome, Italy
| | | | | | | | | | | | - Aurelio Secinaro
- Bambino Gesù Children's Hospital, Rome, Italy, Piazza S. Onofrio n. 4, 00165, Rome, Italy
| | | | - Alberto Villani
- Bambino Gesù Children's Hospital, Rome, Italy, Piazza S. Onofrio n. 4, 00165, Rome, Italy
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Affiliation(s)
| | - Surjit Singh
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
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Abstract
It has been 50 years since the legendary Japanese pediatrician, Dr Tomisaku Kawasaki, published his classic paper in 1967. Little was he to know at that time that this condition would not only be known after his name but would also become the commonest cause of acquired heart disease in children in most of the developed world. The etiology of this condition continues to remain an enigma, and the diagnosis is still based on a set of criteria that are entirely clinical. All pediatricians must be familiar with the various clinical presentations of this disease because delays in diagnosis and treatment can have disastrous consequences.
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Affiliation(s)
- Surjit Singh
- Allergy Immunology unit, Department of Pediatrics, Advanced Pediatrics Centre, PGIMER, Chandigarh, India. Correspondence to: Dr Surjit Singh, Head, Department of Pediatrics and Chief, Allergy Immunology Unit, Advanced Pediatrics Centre, PGIMER, Chandigarh 160 012, India.
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31
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Vaidya PC, Narayanan K, Suri D, Rohit MK, Gupta A, Singh S, Singh M. Pulmonary presentation of Kawasaki disease: an unusual occurrence. Int J Rheum Dis 2017; 20:2227-2229. [PMID: 26662672 DOI: 10.1111/1756-185x.12815] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
Affiliation(s)
- Pankaj C Vaidya
- Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Karthik Narayanan
- Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Deepti Suri
- Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Manoj Kumar Rohit
- Department of Cardiology, Advanced Cardiology Center, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Anju Gupta
- Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Surjit Singh
- Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Meenu Singh
- Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, India
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Jiao F, Jindal AK, Pandiarajan V, Khubchandani R, Kamath N, Sabui T, Mondal R, Pal P, Singh S. The emergence of Kawasaki disease in India and China. Glob Cardiol Sci Pract 2017; 2017:e201721. [PMID: 29564342 PMCID: PMC5856971 DOI: 10.21542/gcsp.2017.21] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2017] [Accepted: 09/19/2017] [Indexed: 12/18/2022] Open
Abstract
Kawasaki disease (KD) is recognized as a leading cause of acquired heart disease in children in developed countries. Although global in distribution, Japan records the highest incidence of KD in the world. Epidemiological reports from the two most populous countries in the world, namely China and India, indicate that KD is now being increasingly recognized. Whether this increased reporting is due to increased ascertainment, or is due to a true increase in incidence, remains a matter of conjecture. The diagnosis and management of KD in developing countries is a challenging proposition. In this review we highlight some of the difficulties faced by physicians in managing children with KD in resource-constrained settings.
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Affiliation(s)
- Fuyong Jiao
- Children’s Hospital, Shaanxi Provincial People’s Hospital of Xian, Jiaotong Univeristy, China
| | - Ankur Kumar Jindal
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, PGIMER, Chandigarh, India
| | - Vignesh Pandiarajan
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, PGIMER, Chandigarh, India
| | | | - Nutan Kamath
- Department of Pediatrics, Kasturba Medical College, Mangalore, India
| | - Tapas Sabui
- Department of Pediatrics, RG Kar Medical College, Kolkata, India
| | - Rakesh Mondal
- Institute of Post Graduate Medical Education and Research, Kolkata, India
| | | | - Surjit Singh
- Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, PGIMER, Chandigarh, India
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Kil HR, Yu JW, Lee SC, Rhim JW, Lee KY. Changes in clinical and laboratory features of Kawasaki disease noted over time in Daejeon, Korea. Pediatr Rheumatol Online J 2017; 15:60. [PMID: 28784161 PMCID: PMC5545846 DOI: 10.1186/s12969-017-0192-y] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/24/2017] [Accepted: 08/01/2017] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Kawasaki disease (KD) becomes one of the common diseases in Korea. Changes in clinical features and laboratory findings of KD were evaluated over a period of 10 years. METHODS We reviewed the medical records of KD patients and compared the clinical and laboratory features of two KD patient groups: those admitted from 2000 to 2004 (group A, 284 cases) and those admitted from 2010 to 2014 (group B, 331 cases). RESULTS There were a total of 615 KD patients (mean age: 29.7 months; male-to-female ratio = 1.6:1), including 228 incomplete KD patients. Incomplete KD patients had milder values in some laboratory indices. The preadmission and total fever durations were longer in group A than in group B. The proportion of incomplete KD was higher in group B, but incidence of coronary artery lesions (CALs) was lower. For laboratory indices, the C-reactive protein and follow-up platelet values were lower, and the hemoglobin and albumin values were higher in group B. The same clinical and laboratory findings were confirmed in the KD subgroups; those with the same fever duration of 5 or 6 days and same ages, those with complete KD, and those with incomplete KD in the two different time periods. CONCLUSIONS Our findings suggest that clinical features of KD tend to be milder over time and manifest in a higher incidence of incomplete KD, lower incidence of CALs, and less severe laboratory findings in recent KD patients in Korea compared with their historic counterparts.
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Affiliation(s)
- Hong-Ryang Kil
- 0000 0001 0722 6377grid.254230.2Department of Pediatrics, Chungnam National University Hospital, School of Medicine, Chungnam National University, Daejeon, Korea
| | - Jae-Won Yu
- 0000 0001 0722 6377grid.254230.2Department of Pediatrics, Chungnam National University Hospital, School of Medicine, Chungnam National University, Daejeon, Korea
| | - Sung-Churl Lee
- 0000 0004 0470 4224grid.411947.eDepartments of Pediatrics, Daejeon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, 64 Daeheung-ro, Jung-gu, Daejeon, 34943 Republic of Korea
| | - Jung-Woo Rhim
- 0000 0004 0470 4224grid.411947.eDepartments of Pediatrics, Daejeon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, 64 Daeheung-ro, Jung-gu, Daejeon, 34943 Republic of Korea
| | - Kyung-Yil Lee
- Departments of Pediatrics, Daejeon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, 64 Daeheung-ro, Jung-gu, Daejeon, 34943, Republic of Korea.
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McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M, Baker AL, Jackson MA, Takahashi M, Shah PB, Kobayashi T, Wu MH, Saji TT, Pahl E. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation 2017; 135:e927-e999. [PMID: 28356445 DOI: 10.1161/cir.0000000000000484] [Citation(s) in RCA: 2378] [Impact Index Per Article: 297.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
BACKGROUND Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acquired heart disease in children in developed countries. METHODS AND RESULTS To revise the previous American Heart Association guidelines, a multidisciplinary writing group of experts was convened to review and appraise available evidence and practice-based opinion, as well as to provide updated recommendations for diagnosis, treatment of the acute illness, and long-term management. Although the cause remains unknown, discussion sections highlight new insights into the epidemiology, genetics, pathogenesis, pathology, natural history, and long-term outcomes. Prompt diagnosis is essential, and an updated algorithm defines supplemental information to be used to assist the diagnosis when classic clinical criteria are incomplete. Although intravenous immune globulin is the mainstay of initial treatment, the role for additional primary therapy in selected patients is discussed. Approximately 10% to 20% of patients do not respond to initial intravenous immune globulin, and recommendations for additional therapies are provided. Careful initial management of evolving coronary artery abnormalities is essential, necessitating an increased frequency of assessments and escalation of thromboprophylaxis. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up. CONCLUSIONS These recommendations provide updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, but clinical decision making should be individualized to specific patient circumstances.
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36
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Abstract
Kawasaki disease (KD) is a systemic vasculitis that mostly affects children below the age of 5. The vasculitis involves arteries of medium size, especially the coronaries. Various etiologies have been proposed including association with micro-organisms, bacterial superantigens, and genetic factors, however, the exact cause remains unknown. There is no specific laboratory test for KD. Diagnosis is clinical and depends upon the presence of fever for ≥5 days and 4 or more of five principal features, viz. polymorphous exanthem, extremity changes, mucosal changes involving the lips and oral cavity, bilateral bulbar conjunctival injection, and unilateral cervical lymphadenopathy. The term "incomplete KD" refers to the presence of fever and less than four principal clinical features. Recognition of this group of patients is important because it is usually seen in infants and risk of coronary abnormalities is increased probably because of delays in diagnosis. However, what appears to be "incomplete" at a given point of time may not actually be so because some of the features may have already subsided and others may evolve over time. Hence, a detailed dermatological examination is warranted in all cases, especially in incomplete KD, to ensure timely diagnosis. Although KD is a self-limiting disease in most patients, coronary artery abnormalities (CAAs) including coronary dilatations and aneurysms may develop in up to 25% of untreated patients. CAAs are the most common cause of morbidity and mortality in patients with KD. Treatment is aimed at reducing inflammation and consists of intravenous immunoglobulin (IVIG) along with aspirin. Despite treatment, some patients may still develop CAAs, and hence, long-term follow up is of utmost importance.
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Affiliation(s)
- Aman Gupta
- Allergy Immunology Unit, Department of Pediatrics, PGIMER, Chandigarh, India
| | - Surjit Singh
- Allergy Immunology Unit, Department of Pediatrics, PGIMER, Chandigarh, India
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37
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Jiao F, Yan X, Wu X. The Xi'an Experience of Kawasaki Disease - Lessons Learnt over 5 years. Indian J Pediatr 2016; 83:1195-6. [PMID: 27094776 DOI: 10.1007/s12098-016-2100-8] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/17/2016] [Accepted: 03/21/2016] [Indexed: 12/19/2022]
Abstract
Kawasaki disease (KD), also well known as mucocutaneous lymph node syndrome, is an acute febrile disease in children with systemic vasculitis as its main pathological change, and usually occurs in children under the age of 5. KD is now the commonest cause of acquired heart disease in children in Japan, Europe and North America. Although KD is now being increasingly diagnosed in mainland China, there is paucity of data on this condition. To provide clinical basis for the treatment of KD, the authors report their experience on 170 children with KD at Shaanxi People's Hospital, Xi'an, China in this research. This also is one of the largest single centre experiences from China so far.
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Affiliation(s)
- Fuyong Jiao
- Children's Hospital, Shaanxi Provincial People's Hospital, Xi'an Jiaotong University, Xi'an, 710061, People's Republic of China.
| | - Xiaohua Yan
- Children's Hospital, Shaanxi Provincial People's Hospital, Xi'an Jiaotong University, Xi'an, 710061, People's Republic of China
| | - Xiaofang Wu
- Xi'an Jiaotong University, Xi'an, 710061, People's Republic of China
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Singh S, Bhattad S. Kawasaki disease incidence at Chandigarh, North India, during 2009-2014. Rheumatol Int 2016; 36:1391-1397. [PMID: 27491781 DOI: 10.1007/s00296-016-3543-y] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/15/2016] [Accepted: 07/26/2016] [Indexed: 12/17/2022]
Abstract
The aim of this study was to estimate incidence of Kawasaki disease (KD) in Chandigarh, North India, during 2009-2014. Diagnosis of KD was based on American Heart Association guidelines. Records of all children diagnosed with KD below 15 years at a large tertiary care referral centre from North India for paediatric immunology were analysed from January 2009 to December 2014. Children residing in Chandigarh were identified. Incidence rates were calculated based on population of Chandigarh in National Census 2011. Methodology was similar to our previously reported study from 1994 to 2008. Incidence of KD in children below 5 was also computed. A total of 258 children were diagnosed to have KD. Of these, 54 (43 boys, 11 girls) resided in Chandigarh. Coronary artery abnormalities on echocardiography were noted in 6. Incidence rate varied between 1.11 (in 2012) and 4.71/100,000 children below 15 (in 2009). In children below 5, incidence rate varied between 1.0 (in 2012) and 9.1/100,000 (in 2009). Peak incidence of KD was in third year of life. There was clustering of cases in February, April, June and October with a nadir in July. While the overall number of KD cases has increased, the 2009-2014 Chandigarh incidence is comparable to our previous figures. Our study is based on hospitalized children with KD and may be missing patients diagnosed elsewhere but that number is likely to be small. Further, patients in whom the diagnosis has never been made would also be missed. Median age at diagnosis has reduced as compared to our previous study. This is probably a reflection of increased awareness about KD amongst paediatricians and physicians in the region as a result of which the proportion of infants and young children diagnosed to have KD has shown a significant increase. Seasonal pattern of occurrence of KD is consistent with our previous observation.
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Affiliation(s)
- Surjit Singh
- Allergy Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
| | - Sagar Bhattad
- Allergy Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India
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Singh S, Agarwal S, Bhattad S, Gupta A, Suri D, Rawat A, Singhal M, Rohit M. Kawasaki disease in infants below 6 months: a clinical conundrum? Int J Rheum Dis 2016; 19:924-928. [PMID: 26990891 DOI: 10.1111/1756-185x.12854] [Citation(s) in RCA: 39] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
AIM Kawasaki disease (KD) is a medium vessel vasculitis of childhood. In infancy KD is often characterized by incomplete and atypical forms. There is paucity of literature on KD in children below 6 months and there are no data from any developing country. This study defines the profile of children with KD below 6 months at our centre. METHODS During January 1994 to March 2015, 460 children were diagnosed with KD and 17 (3.6%) were below 6 months. Diagnosis was based on American Heart Association (AHA) criteria. All children were treated with intravenous immunoglobulin and aspirin; three also received infliximab. RESULTS Mucosal changes were present in 11 patients (64%); extremity changes in 11 (64%); rash in nine (53%); conjunctival injection in eight (47%); and cervical lymphadenopathy in three (17%). Irritability was noted in 15 patients (88%); four (23%) had respiratory symptoms; and two (11%) had bacille Calmette-Guérin scar reactivation. Fifteen (88%) had incomplete KD. Twelve patients were diagnosed beyond day 10 of illness. Thrombocytopenia was seen in four. Coronary artery abnormalities were present in six (35%) patients. Two children died from disease-related complications - one of these had giant coronary artery aneurysms. CONCLUSION Our data show that incomplete forms of KD are commonly seen in children below 6 months of age, thereby resulting in delayed diagnoses. Pediatricians need to have a high index of suspicion of KD when dealing with a young infant with unexplained fever beyond 5 days. The AHA criteria appear to be inadequate for diagnosing KD in infants below 6 months.
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Affiliation(s)
- Surjit Singh
- Allergy Immunology Unit, Advanced Pediatrics Centre, Chandigarh, India. ,
| | - Sikha Agarwal
- Allergy Immunology Unit, Advanced Pediatrics Centre, Chandigarh, India
| | - Sagar Bhattad
- Allergy Immunology Unit, Advanced Pediatrics Centre, Chandigarh, India
| | - Anju Gupta
- Allergy Immunology Unit, Advanced Pediatrics Centre, Chandigarh, India
| | - Deepti Suri
- Allergy Immunology Unit, Advanced Pediatrics Centre, Chandigarh, India
| | - Amit Rawat
- Allergy Immunology Unit, Advanced Pediatrics Centre, Chandigarh, India
| | - Manphool Singhal
- Department of Radiodiagnosis and Imaging, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Manojkumar Rohit
- Department ofCardiology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
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Mathew JL, Jain A. Can breastfeeding in early life protect infants and children from Kawasaki disease? Indian Pediatr 2016; 53:723-5. [DOI: 10.1007/s13312-016-0917-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
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Mauro A, Fabi M, Da Frè M, Guastaroba P, Corinaldesi E, Calabri GB, Giani T, Simonini G, Rusconi F, Cimaz R. Kawasaki disease: an epidemiological study in central Italy. Pediatr Rheumatol Online J 2016; 14:22. [PMID: 27068134 PMCID: PMC4828822 DOI: 10.1186/s12969-016-0084-6] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/25/2016] [Accepted: 04/04/2016] [Indexed: 11/16/2022] Open
Abstract
BACKGROUND Kawasaki disease (KD) is a systemic vasculitis with an acute and self-limited course. The incidence of KD differs widely among ethnic groups and is higher in the Asian population. In Italy, no recent data are available. Our purpose is to define the epidemiology of Kawasaki disease in the years 2008-2013 in children aged < 14 years in the Italian regions of Tuscany and Emilia Romagna through administrative data. METHODS We studied the epidemiology of KD in the years 2008-2013 in children 0-14 years old resident in Tuscany and in Emilia Romagna regions using hospital ICD-9 discharge codes with a thorough data cleaning for duplicates. RESULTS The distribution of the KD patients across ages was similar for the two regions with a peak in the second year of life. When considering data of the two regions together, the rate of incidence was 17.6 for 100,000 children under 5 years. For both Regions the incidence rose slightly during the study period and had a seasonal distribution, with higher incidence in spring and winter. CONCLUSION This is the first Italian study performed through the use of administrative data. Figures are in line but slightly higher than those published in other European countries.
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Affiliation(s)
- Angela Mauro
- Department of Pediatrics, Second University of Naples, via Luigi De Crecchio, 80138, Naples, Italy.
| | - Marianna Fabi
| | - Monica Da Frè
| | - Paolo Guastaroba
| | - Elena Corinaldesi
| | - Giovanni Battista Calabri
| | - Teresa Giani
| | - Gabriele Simonini
| | - Franca Rusconi
| | - Rolando Cimaz
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Abstract
X-linked agammaglobulinemia (XLA) is one of the commonest primary immune deficiencies encountered in pediatric clinical practice. In adults, common variable immunodeficiency (CVID) is the most common primary immunodeficiency disease (PID). It is an X-linked disorder characterized by increased susceptibility to encapsulated bacteria, severe hypergammaglobulinemia and absent circulating B cells in the peripheral blood. Replacement immunoglobulin therapy is the main cornerstone of treatment. Aggressive management of intercurrent infections and prophylactic antimicrobials are needed. This review attempts to highlight varied clinical manifestations and management of XLA, especially in the context of developing country.
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Affiliation(s)
- Deepti Suri
- Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
| | - Amit Rawat
- Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India
| | - Surjit Singh
- Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India
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Reddy M, Singh S, Rawat A, Sharma A, Suri D, Rohit MK. Pro-brain natriuretic peptide (ProBNP) levels in North Indian children with Kawasaki disease. Rheumatol Int 2016; 36:551-559. [PMID: 26849890 DOI: 10.1007/s00296-016-3430-6] [Citation(s) in RCA: 29] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/16/2015] [Accepted: 01/26/2016] [Indexed: 12/20/2022]
Abstract
The diagnosis of Kawasaki disease (KD), a common pediatric vasculitis, is based solely on clinical criteria. There is a need for a robust laboratory marker that can help differentiate KD from other acute, febrile, childhood illnesses and also to predict cardiac involvement. We conducted a cross-sectional study of 25 consecutive patients admitted with diagnosis of KD from January 2013 to April 2014 and compared them with age- and sex-matched febrile controls. We studied the serum pro-brain natriuretic peptide (ProBNP) [ProBNP and N-terminal pro-B-type natriuretic peptide (NT-ProBNP) levels], a marker of myocardial dysfunction, in children with KD in acute and convalescent phases of disease. These levels were also estimated in febrile controls for comparison. The ProBNP (ProBNP and NT-ProBNP) levels were much higher in the acute phase of the KD patients compared to levels in the convalescent phase of KD (p = 0.000014). Similarly, the levels in the acute phase were higher when compared to the age- and sex-matched febrile controls (p = 0.000126). The receiver operating curve (ROC) analysis for the ProBNP levels in the acute phase of KD yielded an area under the curve of 0.954 ± 0.034 (p < 0.000, 95 % CI 0.886-1.0). Based on ROC analysis, a cutoff of 1025 pg/mL for ProBNP levels in the acute phase of KD had 88 % sensitivity and 96 % specificity for the diagnosis of KD. A lower cut-off of 514 pg/mL yielded a 100 % sensitivity and 80 % specificity for the diagnosis of KD. The ProBNP levels were higher in those with coronary artery abnormalities (CAA) compared to those without CAA in both acute (p = 0.013) and convalescent (p = 0.045) phases. ProBNP levels may be used as a surrogate marker for the differentiation of KD from other febrile, infectious illnesses and may also predict the involvement of coronary arteries.
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Affiliation(s)
- Mounika Reddy
- Division of Allergy-Immunology, Advanced Pediatrics Centre, Postgraduate Institute of Medical, Education and Research (PGIMER), Chandigarh, India
| | - Surjit Singh
- Division of Allergy-Immunology, Advanced Pediatrics Centre, Postgraduate Institute of Medical, Education and Research (PGIMER), Chandigarh, India.
| | - Amit Rawat
- Division of Allergy-Immunology, Advanced Pediatrics Centre, Postgraduate Institute of Medical, Education and Research (PGIMER), Chandigarh, India
| | - Avinash Sharma
- Division of Allergy-Immunology, Advanced Pediatrics Centre, Postgraduate Institute of Medical, Education and Research (PGIMER), Chandigarh, India
| | - Deepti Suri
- Division of Allergy-Immunology, Advanced Pediatrics Centre, Postgraduate Institute of Medical, Education and Research (PGIMER), Chandigarh, India
| | - Manoj Kumar Rohit
- Department of Cardiology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
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Prakash J, Singh S, Gupta A, Bharti B, Bhalla AK. Sociodemographic profile of children with Kawasaki disease in North India. Clin Rheumatol 2016; 35:709-713. [PMID: 25413734 DOI: 10.1007/s10067-014-2825-6] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/24/2014] [Revised: 11/08/2014] [Accepted: 11/09/2014] [Indexed: 12/19/2022]
Abstract
Kawasaki disease (KD) is now the commonest cause of acquired heart disease in children in developed countries. KD occurs all over the world, including developing countries. The present study steps out to explore our hypothesis, driven by clinical observation over the last 18 years, whether children with KD in North India are of a higher socioeconomic status than children with other rheumatologic diseases. One hundred consecutive children with KD, registered in Pediatric Rheumatology Clinic before January 2011, were enrolled as cases. Children with other rheumatologic diseases were taken as controls. Assessment of socioeconomic status was done by administering the Aggarwal scale. Data were collected through interview. Statistical analysis was done using SPSS package version 16. On univariate analysis, male sex, higher educational status of parents, urban residence, immunization status being complete, and higher scores on Aggarwal scale were found to be significantly associated with KD. On multivariate analysis, only male sex and urban residence were found to be significantly associated with KD (p < 0.001). Families of children with KD tend to have a better sociodemographic profile when compared with other pediatric rheumatologic disorders in North India. These results, however, need to be replicated in a multicentric study for any firm conclusions to be drawn.
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Affiliation(s)
- Jeya Prakash
- Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India
| | - Surjit Singh
- Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
| | - Anju Gupta
- Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India
| | - Bhavneet Bharti
- Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India
| | - A K Bhalla
- Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India
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Abstract
Over the last 20 years, Kawasaki disease is being increasingly recognized in India and it may soon replace acute rheumatic fever to become the commonest cause of acquired heart disease amongst children. However, the vast majority of children with Kawasaki disease in India are still not being diagnosed. Diagnosis of Kawasaki disease is based on a constellation of clinical findings which have a typical temporal sequence. All pediatricians must we familiar with the nuances involved in arriving at a diagnosis of Kawasaki disease. With early diagnosis and prompt treatment, the risk of coronary artery abnormalities can be significantly reduced.
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Affiliation(s)
- Surjit Singh
- Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India; and the *Japan Kawasaki Disease Research Centre, Chiyoda-Ku, Tokyo, Japan Correspondence to: Dr Surjit Singh, Professor of Pediatrics and Incharge Allergy Immunology Unit, Advanced Pediatrics Centre, PGIMER, Chandigarh 160 012, India.
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Singh S, Sharma A, Jiao F. Kawasaki Disease: Issues in Diagnosis and Treatment--A Developing Country Perspective. Indian J Pediatr 2016; 83:140-145. [PMID: 26400032 DOI: 10.1007/s12098-015-1890-4] [Citation(s) in RCA: 27] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/28/2015] [Accepted: 08/17/2015] [Indexed: 12/21/2022]
Abstract
Kawasaki disease (KD) is a common vasculitis in children and is the commonest cause of pediatric acquired heart disease in children in Japan and countries in North America and the European Union. It is now being increasingly reported from several developing countries, including China and India. Diagnosis of KD is based on a set of clinical criteria, none of which is individually pathognomonic for this condition. Further, these clinical features appear sequentially over a few days and all findings may not be present at a given point of time. Like many other vasculitides, there is no confirmatory laboratory test for KD. Treatment of KD involves use of intravenous immunoglobulin (IVIg) and aspirin. IVIg is an expensive product and poses several difficulties for patients in developing countries who may find it difficult to access therapy even if a diagnosis of KD has been made in time. In this review, the authors discuss some of these challenges that pediatricians have to face while managing KD in resource constrained settings.
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Affiliation(s)
- Surjit Singh
- Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India.
| | - Avinash Sharma
- Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India
| | - Fuyong Jiao
- Department of Pediatrics, Shaanxi Provincial People's Hospital of Xian, Jiaotong University, Shanghai, China
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Singh S, Sharma D, Bhattad S, Phillip S. Recent Advances in Kawasaki Disease - Proceedings of the 3rd Kawasaki Disease Summit, Chandigarh, 2014. Indian J Pediatr 2016; 83:47-52. [PMID: 26318177 DOI: 10.1007/s12098-015-1858-4] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/02/2015] [Accepted: 07/22/2015] [Indexed: 12/19/2022]
Abstract
Kawasaki disease (KD) is the most common cause of acquired heart disease in children in Japan, North America and Europe. It is now being increasingly recognized from the developing countries as well. If not diagnosed and treated in time, KD can result in coronary artery abnormalities in approximately 15-25% cases. The long-term consequences of these abnormalities may manifest in adults as myocardial ischemia and congestive heart failure. Intravenous immunoglobulin (IVIg) remains the drug of choice for treatment of KD, but several new agents like infliximab, cyclosporine, glucocorticoids and statins are now being increasingly used in these patients. While echocardiography has been the preferred imaging modality hitherto, CT coronary angiography has emerged as an exciting new supplementary option and provides an entirely new dimension to this disease. The incidence of KD has shown a progressive increase in several countries and it is likely that this disease would impact public health programmes in the near future even in the developing countries.
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Affiliation(s)
- Surjit Singh
- Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India.
| | - Dhrubajyoti Sharma
- Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India
| | - Sagar Bhattad
- Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India
| | - Saji Phillip
- Kawasaki Disease Foundation of India, St. Gregorios Cardiovascular Centre, Parumala, Kerala, India
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Sharma D, Singh S. Kawasaki disease - A common childhood vasculitis. INDIAN JOURNAL OF RHEUMATOLOGY 2015; 10:S78-S83. [PMID: 32288385 PMCID: PMC7135837 DOI: 10.1016/j.injr.2015.07.010] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2015] [Accepted: 07/01/2015] [Indexed: 12/16/2022] Open
Abstract
Kawasaki disease (KD) is an acute self-limiting vasculitis of children predominantly affecting the medium sized arteries. The disease was first described by Dr. Tomisaku Kawasaki in 1967 from Japan. KD has now been reported from more than 60 countries and is the commonest cause of acquired heart disease in children in the developed countries. Japan reports the highest incidence of KD at 265/100,000 children below 5 years, followed by Korea and Taiwan. In North America and Europe, the incidence of KD is much lower (9-25/100,000 children below 5) and appears to have plateaued down over the last few decades. The reasons for these differences in epidemiology are not clearly understood. KD has been increasingly reported from India over the last 20 years. At Chandigarh, an incidence of 4.54/100,000 children below 15 years was reported in 2011. However, this was likely to be an underestimate. The etiology of KD remains unknown. Although a genetic basis of KD seems plausible, an intercurrent infectious process seems to act as a trigger for the inflammatory cascade. Like many other vasculitides, the diagnosis of KD is essentially clinical and is based on a set of criteria first elaborated by Dr. Kawasaki himself. However, several children (especially infants) with KD can have incomplete and atypical presentations. This can result in diagnostic and therapeutic delays. Approximately 15-25% children with KD can develop coronary artery abnormalities (CAAs) if left untreated. Two dimensional echocardiography remains the gold standard in detecting CAAs in patients with KD. Dual source CT coronary angiography is a recent advance in accurate detection of CAAs with minimal radiation risk. Intravenous immunoglobulin (2 g/kg) remains the drug of choice and is administered as an infusion. Other therapeutic agents that have been used include infliximab, cyclosporine, glucocorticoids, and statins. KD has been associated with several long-term sequelae.
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Affiliation(s)
| | - Surjit Singh
- Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India
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Singh S, Vignesh P, Burgner D. The epidemiology of Kawasaki disease: a global update. Arch Dis Child 2015; 100:1084-1088. [PMID: 26111818 DOI: 10.1136/archdischild-2014-307536] [Citation(s) in RCA: 255] [Impact Index Per Article: 25.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/10/2015] [Accepted: 06/02/2015] [Indexed: 12/19/2022]
Abstract
Kawasaki disease (KD) is a childhood vasculitis and the most frequent cause of paediatric acquired heart disease in North America, Europe and Japan. It is increasingly recognised in rapidly industrialising countries such as China and India where it may replace rheumatic heart disease as the most common cause of acquired heart disease in children. We review the current global epidemiology of KD and discuss some public health implications.
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Affiliation(s)
- Surjit Singh
- Paediatric Allergy Immunology Unit, Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Pandiarajan Vignesh
- Paediatric Allergy Immunology Unit, Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - David Burgner
- Murdoch Children's Research Institute, Parkville, Victoria, Australia Department of Paediatrics, Melbourne University, Parkville, Victoria, Australia Department of Paediatrics, Paediatric Infectious Diseases Department, Monash University, Monash Children's Hospital, Clayton, Victoria, Australia
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