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Portal hypertension children

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https://www.readbyqxmd.com/read/27906797/non-cirrhotic-portal-fibrosis-in-pediatric-population
#1
Vikrant Sood, Bikrant Bihari Lal, Rajeev Khanna, Dinesh Rawat, Chhagan Bihari Sharma, Seema Alam
BACKGROUND: Non-cirrhotic portal fibrosis (NCPF) has been classically described as a disease of young to middle age with limited literature regarding its occurrence, onset or clinical presentation in children. We hereby present a series of 19 patients diagnosed and managed as NCPF in pediatric age group. METHODS: A retrospective review of all the patients presenting to the pediatric hepatology department (age < 18 years) and diagnosed as NCPF was done and data was evaluated...
November 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27896406/evaluation-of-rex-shunt-on-cavernous-transformation-of-the-portal-vein-in-children
#2
Ruo-Yi Wang, Jun-Feng Wang, Xiao-Gang Sun, Qian Liu, Jia-Long Xu, Qi-Gang Lv, Wei-Xiu Chen, Jin-Liang Li
BACKGROUND: Children with cavernous transformation of the portal vein (CTPV) develop severe complications from prehepatic portal hypertension, such as recurrent variceal bleeding and thrombocytopenia. In this study, we reported the results of 30 children with symptomatic CTPV that were treated by a Rex shunt. The effectiveness of this surgical approach was evaluated. METHODS: A retrospective review was performed of 30 children aged between 3 and 18 years with CTPV, who underwent a Rex shunt between 2008 and 2015...
November 28, 2016: World Journal of Surgery
https://www.readbyqxmd.com/read/27895519/portal-hypertension-an-uncommon-clinical-manifestation-of-takayasu-arteritis-in-a-9-year-old-child
#3
Cristina N Herrera, Javier E Tomala-Haz
Takayasu arteritis (TA) is the third most common childhood vasculitis and its clinical manifestations depend on the arteries involved. We report a case of a 9-year-old boy with multiple aneurysms in carotid and iliac arteries, subclavian and coronary arteries, and abdominal aorta. At the age of 7 years, he presented with recurrent fever and hepatosplenomegaly. An angio-computed tomography scan showed aneurysms in the left subclavian artery, abdominal aorta, and both proximal iliac arteries. He was diagnosed with TA and was treated with corticosteroids, aspirin, and enalapril...
2016: Open Access Rheumatology: Research and Reviews
https://www.readbyqxmd.com/read/27801752/survey-on-clinical-practice-of-primary-prophylaxis-in-portal-hypertension-in-children
#4
Odile Jeanniard-Malet, Mathieu Duché, Alexandre Fabre
Primary prophylaxis in portal hypertension in children is controversial, as there are few studies documenting its efficacy on the risk of bleeding.28 centres out of the 38 we contacted returned a completed questionnaire about their clinical practices. More than 75% of the centres use endoscopy to screen patients diagnosed with portal cavernoma, biliary atresia, cystic fibrosis and other fibrotic chronic liver diseases with suspected portal hypertension.In cases of grade 2 varices with red marks and grade 3 varices more than 90% of centres perform sclerotherapy or endoscopic variceal ligation (EVL)...
October 31, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27782957/correlation-of-transient-elastography-with-severity-of-cystic-fibrosis-related-liver-disease
#5
Amal Aqul, Maureen M Jonas, Sarah Harney, Roshan Raza, Gregory S Sawicki, Paul D Mitchell, Rima Fawaz
OBJECTIVE: To evaluate whether liver stiffness measurement (LSM), determined by transient elastography (TE), correlates with presence and severity of liver disease (LD) in children and young adults with cystic fibrosis (CF). METHODS: Subjects underwent LSM at routine CF visits. Presence and severity of CFLD was determined by clinical parameters. Subjects were classified as no CFLD, CFLD without portal hypertension (PHTN), and CFLD with PHTN. LSM was compared to AST/platelet ratio index (APRI) as a correlate to severity of CFLD...
October 25, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27762481/jejunal-varices-diagnosed-by-capsule-endoscopy-in-patients-with-post-liver-transplant-portal-hypertension
#6
Lee M Bass, Stanley Kim, Riccardo Superina, Saeed Mohammad
Portal hypertension secondary to portal vein obstruction following liver transplant occurs in 5%-10% of children. Jejunal varices are uncommon in this group. We present a case series of children with significant GI blood loss, negative upper endoscopy, and jejunal varices detected by CE. Case series of patients who had CE for chronic GI blood loss following liver transplantation. Three patients who had their initial transplants at a median age of 7 months were identified at our institution presenting at a median age of 8 years (range 7-16 years) with a median Hgb of 2...
October 20, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27757413/endoscopic-management-of-bleeding-gastric-varices-with-n-butyl-2-cyanoacrylate-glue-injection-in-children-with-non-cirrhotic-portal-hypertension
#7
Ujjal Poddar, Vibhor Borkar, Surender Kumar Yachha, Anshu Srivastava
Background and study aims: In view of the paucity of literature, we carried out this audit to evaluate the safety and efficacy of N- butyl, 2-cynoacrylate glue injection therapy in secondary prophylaxis of gastric varices in children. Patients and methods: Consecutive children (≤ 18 years) with non-cirrhotic portal hypertension who presented with bleeding from gastric varices and who had undergone cyanoacrylate glue injection therapy were included. They were evaluated for safety, efficacy and complications...
October 2016: Endoscopy International Open
https://www.readbyqxmd.com/read/27757140/bipolar-and-related-disorders-induced-by-sodium-4-phenylbutyrate-in-a-male-adolescent-with-bile-salt-export-pump-deficiency-disease
#8
Giovanni Vitale, Giulia Simonetti, Martina Pirillo, Gianfranco Taruschio, Pietro Andreone
Bile Salt Export Pump (BSEP) Deficiency disease, including Progressive Familial Intrahepatic Cholestasis type 2 (PFIC2), is a rare disease, usually leading within the first ten years to portal hypertension, liver failure, hepatocellular carcinoma. Often liver transplantation is needed. Sodium 4-phenylbutyrate (4-PB) seems to be a potential therapeutic compound for PFIC2. Psychiatric side effects in the adolescent population are little known and little studied since the drug used to treat children and infants...
September 2016: Psychiatry Investigation
https://www.readbyqxmd.com/read/27749613/king-s-variceal-prediction-score-a-novel-non-invasive-marker-of-portal-hypertension-in-paediatric-chronic-liver-disease
#9
Peter Witters, Dominic Hughes, Palaniswamy Karthikeyan, Somashekara Ramakrishna, Mark Davenport, Anil Dhawan, Tassos Grammatikopoulos
BACKGROUND AND AIMS: Variceal haemorrhage can be a life-threatening complication of chronic liver disease in children. There is limited evidence about the optimal prophylactic management and selection criteria of children who will benefit from upper gastrointestinal endoscopy. METHODS: Children presenting in our centre with suspected portal hypertension or gastrointestinal bleeding and undergoing their first oesophagogastroduodenoscopy between 2005-2012 were included...
October 4, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27706456/evaluation-of-primary-prophylaxis-with-propranolol-and-elastic-band-ligation-in-variceal-bleeding-in-cirrhotic-children-and-adolescents
#10
Júlio Rocha Pimenta, Alexandre Rodrigues Ferreira, Paulo Fernando Souto Bittencourt, Camilo Brandão de Resende, Eleonora Druve Tavares Fagundes, Isabela Maria Lopes da Silva
Background: The efficacy of nonselective β-blocker and endoscopic procedures, such as endoscopic variceal ligation, as primary prophylaxis of variceal hemorrhage in cirrhotic adults was demonstrated by numerous controlled trials, but in pediatric population, few are the number of studies. Objective: The objective of this study is to evaluate the primary prophylaxis with β-blocker in cirrhotic children and adolescents with portal hypertension. Methods: This is a cohort study encompassing 26 cirrhotic patients...
October 2016: Arquivos de Gastroenterologia
https://www.readbyqxmd.com/read/27697318/surgical-strategy-according-to-the-anatomical-types-of-congenital-portosystemic-shunts-in-children
#11
Toshiharu Matsuura, Yoshiaki Takahashi, Yusuke Yanagi, Koichiro Yoshimaru, Kenichiro Yamamura, Eiji Morihana, Hazumu Nagata, Kiyoshi Uike, Hidetoshi Takada, Tomoaki Taguchi
BACKGROUND: Congenital portosystemic shunts (CPSS) with intrahepatic portal vein (IHPV) hypoplasia or absence cause encephalopathy or pulmonary hypertension (PH). Acute shunt closure may result in postoperative portal hypertension. The aim of this study was to propose a surgical strategy according to the anatomical types of CPSS and IHPV. METHODS: Twenty-three CPSS patients were diagnosed from1990 to 2015. All patients were evaluated by computed tomography, angiography, and PV pressure monitoring under a shunt occlusion test...
September 21, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27663417/portal-hypertension-in-children-high-risk-varices-primary-prophylaxis-and-consequences-of-bleeding
#12
Mathieu Duché, Béatrice Ducot, Oanez Ackermann, Florent Guérin, Emmanuel Jacquemin, Olivier Bernard
BACKGROUND & AIMS: Primary prophylaxis of bleeding is debated for children with portal hypertension because of the limited number of studies on its safety and efficacy, the lack of a known endoscopic pattern carrying a high-risk of bleeding for all causes, and the assumption that the mortality of a first bleed is low. We report our experience with these issues. METHODS: From 1989 to 2014, we managed 1300 children with portal hypertension. Endoscopic features were recorded; high-risk varices were defined as: grade 3 esophageal varices, grade 2 varices with red wale markings, or gastric varices...
September 20, 2016: Journal of Hepatology
https://www.readbyqxmd.com/read/27661550/-management-of-pediatric-patients-with-esophageal-varices
#13
Sofía Verdaguer D, Juan Cristóbal Gana A
BACKGROUND: There is a paucity of good quality research about the diagnosis of esophageal varices and the prophylaxis and treatment of variceal bleeding in pediatric patients with portal hypertension There is little consensus and practically no evidence-based approach about the management of these patients. AIM: To describe the behavior and preferences of pediatric gastroenterologists in Chile in the management of portal hypertension in children. MATERIAL AND METHODS: An online survey was sent to Chilean pediatric gastroenterologists, with questions evaluating the physicians’ approaches to screening of esophageal varices in children with portal hypertension, and their preferred methods of prophylaxis and initial management of variceal bleeding...
July 2016: Revista Médica de Chile
https://www.readbyqxmd.com/read/27650268/biliary-atresia-indications-and-timing-of-liver-transplantation-and-optimization-of-pre-transplant-care
#14
Shikha S Sundaram, Cara L Mack, Amy G Feldman, Ronald J Sokol
Biliary Atresia is a progressive, fibro-obliterative disorder of the intra and extrahepatic bile ducts in infancy. The majority of affected children will eventually develop end-stage liver disease and require liver transplantation. Indications for liver transplant in biliary atresia include failed Kasai portoenterostomy, significant and recalcitrant malnutrition, recurrent cholangitis, and the progressive manifestations of portal hypertension. Extra-hepatic complications of this disease, such as hepatopulmonary syndrome and portopulmonary hypertension, are also indications for liver transplantation...
September 21, 2016: Liver Transplantation
https://www.readbyqxmd.com/read/27602703/congenital-vascular-malformations-of-the-liver-an-association-with-trisomy-21
#15
Oliver C Burdall, Tassos Grammatikopoulos, Maria Sellars, Nedim Hadzic, Mark Davenport
A link between congenital vascular malformation (CVM) of the liver and trisomy 21 has been suggested. We reviewed all children with trisomy 21 referred for investigation to a specialist pediatric hepatobiliary unit (1985-2015). Forty-five children with trisomy 21 were identified; 7 (15%) had a defined CVMs (4 girls). All such infants were also diagnosed with a range of cardiac defects. CVMs were divided according to the nature of the vascular connection. Group (i) (n = 3): Abnormal venovenous anomaly. This included portocaval shunt and patent ductus venosus (n = 2)...
December 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27504083/very-early-presentation-of-extrahepatic-portal-vein-obstruction-causing-portal-hypertension-in-an-infant-uncertainties-in-the-management-and-therapeutic-limitations
#16
Parisá Khodayar-Pardo, Andrés Peña Aldea, Ana Ramírez González, Adela Meseguer Carrascosa, Cristina Calabuig Bayo
Extrahepatic portal vein obstruction, although rare in children, is a significant cause of portal hypertension (PHT) leading to life-threatening gastrointestinal bleeding in the pediatric age group. PHT may also lead to other complications such as hyperesplenism, cholangyopathy, ascites, and even hepatopulmonary syndrome and portopulmonary hypertension that may require organ transplantation. Herein we report the case of an asymptomatic 11-month-old infant wherein a hepatomegaly and cavernous transformation of the portal vein was detected by liver ultrasound...
May 2016: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/27498582/long-term-follow-up-of-children-and-adolescents-with-primary-sclerosing-cholangitis-and-autoimmune-sclerosing-cholangitis
#17
Vratislav Smolka, Eva Karaskova, Oksana Tkachyk, Kvetoslava Aiglova, Jiri Ehrmann, Kamila Michalkova, Michal Konecny, Jana Volejnikova
BACKGROUND: Sclerosing cholangitis (SC) is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate long-term results in children with SC according to the types of SC. METHODS: We retrospectively followed up 25 children with SC over a period of 4-17 years (median 12). The diagnosis of SC was based on biochemical, histological and cholangiographic findings. Patients fulfilling diagnostic criteria for probable or definite autoimmune hepatitis at the time of diagnosis were defined as having autoimmune sclerosing cholangitis (ASC); other patients were included in a group of primary sclerosing cholangitis (PSC)...
August 2016: Hepatobiliary & Pancreatic Diseases International: HBPD INT
https://www.readbyqxmd.com/read/27496799/factors-associated-with-bleeding-secondary-to-rupture-of-esophageal-varices-in-children-and-adolescents-with-cirrhosis
#18
Júlio Rocha Pimenta, Alexandre Rodrigues Ferreira, Eleonora Druve Tavares Fagundes, Thaís Costa Nascentes Queiroz, Regiane Aparecida Nascimento Baptista, Eduardo Guimarães de Araújo Moreira, Camilo Brandão de Resende, Paulo Fernando Souto Bittencourt, Simone Diniz Carvalho, José Andrade Franco Neto, Francisco José Penna
OBJECTIVE: Bleeding of esophageal varices is the main cause of morbidity and mortality in children with portal hypertension. It is important to understand the factors related with a bleeding episode to evaluate more effective primary prophylaxis. This study aims to describe the endoscopic and laboratory findings associated with upper gastrointestinal bleeding (UGIB) secondary to esophageal varices. METHOD: A cross-sectional study with 103 children and adolescents with cirrhosis, divided into a group that had experienced an episode of upper UGIB (35 patients) and a group without a history of UGIB (68 patients), was carried out...
August 4, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27289254/medical-complications-following-splenectomy
#19
R Buzelé, L Barbier, A Sauvanet, B Fantin
Splenectomy is attended by medical complications, principally infectious and thromboembolic; the frequency of complications varies with the conditions that led to splenectomy (hematologic splenectomy, trauma, presence of portal hypertension). Most infectious complications are caused by encapsulated bacteria (Meningococcus, Pneumococcus, Hemophilus). These occur mainly in children and somewhat less commonly in adults within the first two years following splenectomy. Post-splenectomy infections are potentially severe with overwhelming post-splenectomy infection (OPSI) and this justifies preventive measures (prophylactic antibiotics, appropriate immunizations, patient education) and demands prompt antibiotic management with third-generation cephalosporins for any post-splenectomy fever...
August 2016: Journal of Visceral Surgery
https://www.readbyqxmd.com/read/27278239/ascites-in-children
#20
Ashish Bavdekar, Nitin Thakur
Ascites is an accumulation of serous fluid within the peritoneal cavity. It is the most common complication of liver cirrhosis. In children, hepatic, renal and cardiac disorders are the most common causes. Portal hypertension and sodium and fluid retention are key factors in the pathophysiology of ascites. Peripheral arterial vasodilatation hypothesis is the most accepted mechanism for inappropriate sodium retention and formation of ascites. Diagnostic paracentesis is indicated in children with newly diagnosed ascites and in children with suspected complications of ascites...
June 9, 2016: Indian Journal of Pediatrics
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