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Sunar Yayla EN, Sarı S, Gürcan Kaya N, Eğrİtaş Gürkan Ö, Sözen H, Özen İO, Dalgıç A, Dalgıç B. Portal Hypertension in Children: A Tertiary Center Experience in Turkey. Pediatr Gastroenterol Hepatol Nutr 2023; 26:301-311. [PMID: 38025487 PMCID: PMC10651363 DOI: 10.5223/pghn.2023.26.6.301] [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: 05/19/2023] [Revised: 08/09/2023] [Accepted: 09/16/2023] [Indexed: 12/01/2023] Open
Abstract
Purpose Portal hypertension (PH) and its complications have a significant impact on morbidity and mortality. This study aimed to evaluate the etiology; clinical, laboratory, and endoscopic findings; treatment approaches; long-term outcomes; and prognosis of pediatric PH. Methods This retrospective study included 222 pediatric patients diagnosed with PH between 1998 and 2016, and data encompassing clinical, laboratory, and radiological features; treatments; and complications were analyzed. Results The most common causes of PH were portal vein thrombosis (20.3%), progressive familial intrahepatic cholestasis (18.9%), and biliary atresia (12.2%). Among the enrolled patients, 131 (59.0%) were included in the cirrhotic group and 91 (41.0%) in the non-cirrhotic group. Hepatomegaly and increased transaminase levels were more frequent in the cirrhotic group than in the non-cirrhotic group. Additionally, portal gastropathy, esophageal varices, and variceal bleeding were more frequent in the non-cirrhotic group, whereas ascites, hepatopulmonary syndrome and hepatic encephalopathy were more common in the cirrhotic group. The incidence of hepatomegaly was higher in the presinusoidal group than in the prehepatic group (p<0.001). Hyperbilirubinemia was more frequent in the prehepatic group (p=0.046). The frequency of esophageal varices was similar between the prehepatic and presinusoidal groups; however, variceal bleeding was more frequent in the prehepatic group (p=0.002). Conclusion Extrahepatic portal vein obstruction, genetic-metabolic diseases, and biliary atresia were the most prevalent causes of PH in our country. In patients with PH, hepatomegaly, increased transaminase levels, and synthesis dysfunction were suggestive of cirrhotic PH. Notably, PH in patients without cirrhosis might be more severe than that in those with cirrhosis.
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Affiliation(s)
| | - Sinan Sarı
- Department of Pediatrics, Division of Pediatric Gastroenterology, Faculty of Medicine, Gazi University, Ankara, Turkey
| | - Neslihan Gürcan Kaya
- Department of Pediatrics, Division of Pediatric Gastroenterology, Faculty of Medicine, Gazi University, Ankara, Turkey
| | - Ödül Eğrİtaş Gürkan
- Department of Pediatrics, Division of Pediatric Gastroenterology, Faculty of Medicine, Gazi University, Ankara, Turkey
| | - Hakan Sözen
- Division of Transplantation Surgery, Faculty of Medicine, Gazi University, Ankara, Turkey
| | - İbrahim Onur Özen
- Department of Pediatric Surgery, Faculty of Medicine, Gazi University, Ankara, Turkey
| | - Aydın Dalgıç
- Division of General Surgery, Faculty of Medicine, Gazi University, Ankara, Turkey
| | - Buket Dalgıç
- Department of Pediatrics, Division of Pediatric Gastroenterology, Faculty of Medicine, Gazi University, Ankara, Turkey
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Marrache MK, Bou Daher H, Rockey DC. The relationship between portal hypertension and portal hypertensive gastropathy. Scand J Gastroenterol 2022; 57:340-344. [PMID: 34904513 PMCID: PMC9717700 DOI: 10.1080/00365521.2021.2012591] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
BACKGROUND AND AIM Portal hypertensive gastropathy (PHG) most commonly occurs in the setting of increased portal pressure in patients with cirrhosis. Here, we aimed to understand the correlation between hepatic venous pressure gradient (HVPG) and the presence and severity of PHG in patients with cirrhosis. METHODS We examined patients with cirrhosis who underwent HVPG measurement at the Medical University of South Carolina between 2014 and 2020. Extensive demographic, clinical, laboratory, procedural (including precise grading of PHG severity using standard definitions), and outcome data were abstracted at the time of HVPG measurement. RESULTS Three hundred and ten patients with HVPG measurements and cirrhosis were identified. Seventy-three patients having endoscopy within 6 months of HVPG measurement were included (mean age 54 ± 11, 44% female). The most common causes of cirrhosis were alcohol (41%) and non-alcoholic steatohepatitis (32%). The average HVPG was 15 mmHg (±6) and 62 patients had clinically significant portal hypertension (CSPH) (HVPG ≥ 10 mmHg). Of the 73 patients with HVPG measured, 45 (62%) had PHG, including 40 (89%) of whom had CSPH. Out of the 45 patients with PHG, 41 and four had mild or severe PHG, respectively. MELD scores were similar in patients with and without PHG [15 ± 9 (SD) and 17 ± 9, respectively; p = .37]. HVPG was higher in patients with PHG (17 ± 7 mmHg) than those without PHG (13 ± 4 mmHg) (p = .01) but did not differ between mild and severe PHG. CONCLUSION A weak correlation exists between HVPG level and the presence of PHG.
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Affiliation(s)
- Mohamad Kareem Marrache
- Digestive Disease Research Center, Medical University of South Carolina, Charleston, SC, USA
| | - Halim Bou Daher
- Digestive Disease Research Center, Medical University of South Carolina, Charleston, SC, USA
| | - Don C. Rockey
- Digestive Disease Research Center, Medical University of South Carolina, Charleston, SC, USA
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Misra V, Agrawal R, Misra SP. Portal hypertensive vasculopathy - An entity worth remembering with non-neoplastic gastrointestinal lesions. INDIAN J PATHOL MICR 2021; 64:S32-S42. [PMID: 34135136 DOI: 10.4103/ijpm.ijpm_201_21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Portal hypertensive vasculopathy (PHV) represents an increase in the pressure in the portal circulation. This increased pressure leads to changes in the mucosa that can be appreciated endoscopically as well as histopathologically. Lesions can be observed in the entire gastrointestinal tract (GIT) including stomach, duodenum, jejunum and colon. The histological changes are appreciated mainly in the mucosal and submucosal blood vessels. A knowledge of these lesions as well as the changes helps in separating them from other close differentials with specific treatment to be instituted. The pathogenesis of the hemodynamic changes is not well-understood. The underlying factor is increased portal pressure. Studies indicate, that besides increased portal pressure other factors in combination led to the observed changes. Portal hypertensive gastropathy (PHG) is seen in the gastric body and fundus, while varices are noted in the cardia and fundus. Changes may be seen in the small intestine and throughout the colon with anorectal varices. Histopathological changes include dilated, congested and ectatic capillaries and edema in the lamina propria besides a large spectrum of other histopathological changes. Mucosal capillaries with thick irregular wall show absence of red blood cells in the lumen. Thickening of the vessel wall serves as a better marker than the vascular diameter when portal hypertension (PHT) is considered. Long standing cases may show fibrosis in lamina propria. At times, these changes may lead to occult gastrointestinal bleeding. Important differentials need to be ruled out in all the cases as the treatment and the outcome of all differs. Endoscopically or histopathologically if the mucosal changes are observed they should not be overlooked and a detailed work-up must be carried out.
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Affiliation(s)
- Vatsala Misra
- Department of Pathology and Gastroenterology, MLN Medical College, Prayagraj, Uttar Pradesh, India
| | - Ranjan Agrawal
- Department of Pathology, Rohilkhand Medical College and Hospital, Bareilly, Uttar Pradesh, India
| | - Sri Praksh Misra
- Department of Pathology and Gastroenterology, MLN Medical College, Prayagraj, Uttar Pradesh, India
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Cisarò F, Pizzol A, Calvo PL. Helicobacter pylori infection in children with portal hypertensive gastropathy: a clear indication for clinical practice. Minerva Pediatr 2018; 72:72. [PMID: 30299030 DOI: 10.23736/s0026-4946.18.05395-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
Affiliation(s)
- Fabio Cisarò
- Unit of Pediatric Gastroenterology, Department of Pediatrics, Città della Salute e della Scienza, University of Turin, Turin, Italy -
| | - Antonio Pizzol
- Unit of Pediatric Gastroenterology, Department of Pediatrics, Città della Salute e della Scienza, University of Turin, Turin, Italy
| | - Pier L Calvo
- Unit of Pediatric Gastroenterology, Department of Pediatrics, Città della Salute e della Scienza, University of Turin, Turin, Italy
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Massoni F, Ricci L, Pelosi M, Ricci S. The Portal Hypertensive Gastropathy: A Case and Review of Literature. J Clin Diagn Res 2016; 10:HD01-2. [PMID: 27504310 DOI: 10.7860/jcdr/2016/14489.7942] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/15/2015] [Accepted: 04/15/2016] [Indexed: 11/24/2022]
Abstract
Upper gastrointestinal bleeding is a cause of high risk for morbidity and mortality. It has been debated in alcoholic cirrhosis, if alcohol exerts an exclusive and causal role upon gastropathy or whether it is linked to cirrhotic portal hypertension. The authors describe an autopsy report regarding mortality caused by gastric bleeding in a 53-year-old patient who suffered from cirrhosis. Literature has evidence of direct, marked damage of alcohol upon the gastric mucosa and there is noteworthy statistical data implying the revaluation of the pathogenesis of the bleeding.
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Affiliation(s)
- Francesco Massoni
- Post-Doctoral Fellow, Department of Anatomy, Histology, Legal Medicine and Orthopedics - "Sapienza" University of Rome , Italy
| | - Lidia Ricci
- Research and Teaching Assistant, Department of Anatomy, Histology, Legal Medicine and Orthopedics - "Sapienza" University of Rome , Italy
| | - Marcello Pelosi
- Lecturer, Department of Anatomy, Histology, Legal Medicine and Orthopedics - "Sapienza" University of Rome , Italy
| | - Serafino Ricci
- Professor, Department of Anatomy, Histology, Legal Medicine and Orthopedics - "Sapienza" University of Rome , Italy
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Gjeorgjievski M, Cappell MS. Portal hypertensive gastropathy: A systematic review of the pathophysiology, clinical presentation, natural history and therapy. World J Hepatol 2016; 8:231-262. [PMID: 26855694 PMCID: PMC4733466 DOI: 10.4254/wjh.v8.i4.231] [Citation(s) in RCA: 49] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/02/2015] [Revised: 11/30/2015] [Accepted: 01/16/2016] [Indexed: 02/06/2023] Open
Abstract
AIM To describe the pathophysiology, clinical presentation, natural history, and therapy of portal hypertensive gastropathy (PHG) based on a systematic literature review. METHODS Computerized search of the literature was performed via PubMed using the following medical subject headings or keywords: "portal" and "gastropathy"; or "portal" and "hypertensive"; or "congestive" and "gastropathy"; or "congestive" and "gastroenteropathy". The following criteria were applied for study inclusion: Publication in peer-reviewed journals, and publication since 1980. Articles were independently evaluated by each author and selected for inclusion by consensus after discussion based on the following criteria: Well-designed, prospective trials; recent studies; large study populations; and study emphasis on PHG. RESULTS PHG is diagnosed by characteristic endoscopic findings of small polygonal areas of variable erythema surrounded by a pale, reticular border in a mosaic pattern in the gastric fundus/body in a patient with cirrhotic or non-cirrhotic portal hypertension. Histologic findings include capillary and venule dilatation, congestion, and tortuosity, without vascular fibrin thrombi or inflammatory cells in gastric submucosa. PHG is differentiated from gastric antral vascular ectasia by a different endoscopic appearance. The etiology of PHG is inadequately understood. Portal hypertension is necessary but insufficient to develop PHG because many patients have portal hypertension without PHG. PHG increases in frequency with more severe portal hypertension, advanced liver disease, longer liver disease duration, presence of esophageal varices, and endoscopic variceal obliteration. PHG pathogenesis is related to a hyperdynamic circulation, induced by portal hypertension, characterized by increased intrahepatic resistance to flow, increased splanchnic flow, increased total gastric flow, and most likely decreased gastric mucosal flow. Gastric mucosa in PHG shows increased susceptibility to gastrotoxic chemicals and poor wound healing. Nitrous oxide, free radicals, tumor necrosis factor-alpha, and glucagon may contribute to PHG development. Acute and chronic gastrointestinal bleeding are the only clinical complications. Bleeding is typically mild-to-moderate. Endoscopic therapy is rarely useful because the bleeding is typically diffuse. Acute bleeding is primarily treated with octreotide, often with concomitant proton pump inhibitor therapy, or secondarily treated with vasopressin or terlipressin. Nonselective β-adrenergic receptor antagonists, particularly propranolol, are used to prevent bleeding after an acute episode or for chronic bleeding. Iron deficiency anemia from chronic bleeding may require iron replacement therapy. Transjugular-intrahepatic-portosystemic-shunt and liver transplantation are highly successful ultimate therapies because they reduce the underlying portal hypertension. CONCLUSION PHG is important to recognize in patients with cirrhotic or non-cirrhotic portal hypertension because it can cause acute or chronic GI bleeding that often requires pharmacologic therapy.
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Affiliation(s)
- Mihajlo Gjeorgjievski
- Mihajlo Gjeorgjievski, Mitchell S Cappell, Division of Gastroenterology and Hepatology, William Beaumont Hospital, Royal Oak, MI 48073, United States
| | - Mitchell S Cappell
- Mihajlo Gjeorgjievski, Mitchell S Cappell, Division of Gastroenterology and Hepatology, William Beaumont Hospital, Royal Oak, MI 48073, United States
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Oh SH, Kim SJ, Rhee KW, Kim KM. Endoscopic cyanoacrylate injection for the treatment of gastric varices in children. World J Gastroenterol 2015; 21:2719-2724. [PMID: 25759541 PMCID: PMC4351223 DOI: 10.3748/wjg.v21.i9.2719] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/23/2014] [Revised: 10/28/2014] [Accepted: 12/08/2014] [Indexed: 02/06/2023] Open
Abstract
AIM: To evaluate the efficacy and safety of N-butyl-2-cyanoacrylate in treating acute bleeding of gastric varices in children.
METHODS: The retrospective study included 21 children with 47 episodes of active gastric variceal bleeding who were treated by endoscopic injection of N-butyl-2-cyanoacrylate at Asan Medical Center Children’s Hospital between August 2004 and December 2011. To reduce the risk of embolism, each injection consisted of 0.1-0.5 mL of 0.5 mL N-butyl-2-cyanoacrylate diluted with 0.5 or 0.8 mL Lipiodol. The primary outcome was incidence of hemostasis after variceal obliteration and the secondary outcome was complication of the procedure.
RESULTS: The 21 patients experienced 47 episodes of active gastric variceal bleeding, including rebleeding, for which they received a total of 52 cyanoacrylate injections. Following 42 bleeding episodes, hemostasis was achieved after one injection and following five bleeding episodes it was achieved after two injections. The mean volume of each single aliquot of cyanoacrylate injected was 0.3 ± 0.1 mL (range: 0.1-0.5 mL). Injection achieved hemostasis in 45 of 47 (95.7%) episodes of acute gastric variceal bleeding. Eleven patients (52.4%) developed rebleeding events, with the mean duration of hemostasis being 11.1 ± 11.6 mo (range: 1.0-39.2 mo). No treatment-related complications such as distal embolism were noted with the exception of abdominal pain in one patient (4.8%). Among four mortalities, one patient died of variceal rebleeding.
CONCLUSION: Endoscopic variceal obliteration using a small volume of aliquots with repeated cyanoacrylate injection was an effective and safe option for the treatment of gastric varices in children.
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Osman D, Djibré M, Da Silva D, Goulenok C. Management by the intensivist of gastrointestinal bleeding in adults and children. Ann Intensive Care 2012; 2:46. [PMID: 23140348 PMCID: PMC3526517 DOI: 10.1186/2110-5820-2-46] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/11/2012] [Accepted: 10/05/2012] [Indexed: 12/12/2022] Open
Abstract
Intensivists are regularly confronted with the question of gastrointestinal bleeding. To date, the latest international recommendations regarding prevention and treatment for gastrointestinal bleeding lack a specific approach to the critically ill patients. We present recommendations for management by the intensivist of gastrointestinal bleeding in adults and children, developed with the GRADE system by an experts group of the French-Language Society of Intensive Care (Société de Réanimation de Langue Française (SRLF), with the participation of the French Language Group of Paediatric Intensive Care and Emergencies (GFRUP), the French Society of Emergency Medicine (SFMU), the French Society of Gastroenterology (SNFGE), and the French Society of Digestive Endoscopy (SFED). The recommendations cover five fields of application: management of gastrointestinal bleeding before endoscopic diagnosis, treatment of upper gastrointestinal bleeding unrelated to portal hypertension, treatment of upper gastrointestinal bleeding related to portal hypertension, management of presumed lower gastrointestinal bleeding, and prevention of upper gastrointestinal bleeding in intensive care.
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Affiliation(s)
- David Osman
- AP-HP, Hôpitaux universitaires Paris-Sud, Hôpital de Bicêtre, Service de réanimation médicale, Le Kremlin-Bicêtre, F-94270, France.
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Prise en charge par le réanimateur des hémorragies digestives de l’adulte et de l’enfant. MEDECINE INTENSIVE REANIMATION 2012. [DOI: 10.1007/s13546-012-0489-2] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
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Portal gastropathy and duodenopathy in children with extrahepatic and intrahepatic portal hypertension: endoscopic diagnosis and histologic scoring. J Pediatr Gastroenterol Nutr 2011; 52:612-6. [PMID: 21464749 DOI: 10.1097/mpg.0b013e3182125e7c] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/25/2022]
Abstract
OBJECTIVES The aim of the study was to determine the frequency of portal gastropathy (PG) and duodenopathy (PD) in children, to document the correlation of various clinical and laboratory parameters associated with portal hypertensive gastroduodenal lesions, to compare the endoscopic portal hypertensive lesions with different histologic findings, and to evaluate the use of a possible histologic scoring system. METHODS All children undergoing endoscopic investigation for portal hypertension (PH) between January 2006 and November 2007 were analysed retrospectively. Clinical and demographical data and endoscopic and histologic findings were recorded. Histologic findings suggestive of PG and PD (capillary dilation, increased numbers of capillaries, histologic bleeding, and edema) were scored. RESULTS Of 51 consecutive children (29 boys, mean age 10.1 ± 3.6 years [range 2.5-15.8 years]), 28 were cirrhotic. PG was diagnosed in 58.8% endoscopically. Children with cirrhotic PH had the highest rate of PG (64.3%), whereas those with extrahepatic or intrahepatic noncirrhotic PH were alike (50% and 54.5%, respectively). Baveno PG scores were higher in children with cirrhosis with higher Child-Pugh scores. Capillary dilation was the only histologic finding showing significant association with the endoscopic diagnosis. Only 9% had PD on endoscopy. None of the histologic findings correlated with endoscopic diagnosis of PD. CONCLUSIONS PG and PD are seen in children with extrahepatic and intrahepatic PH at rates similar to those reported in adult studies. Baveno PG scores increased in parallel with Child-Pugh class in children with cirrhosis. Capillary dilation was the only histologic finding showing significant association with the endoscopic diagnosis of PG in this study.
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