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Portal hypertension children

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https://www.readbyqxmd.com/read/28390159/the-natural-history-of-primary-sclerosing-cholangitis-in-781-children-a-multicenter-international-collaboration
#1
Mark R Deneau, Wael El-Matary, Pamela L Valentino, Reham Abdou, Khaled Alqoaer, Mansi Amin, Achiya Z Amir, Marcus Auth, Fateh Bazerbachi, Annemarie Broderick, Albert Chan, Jillian Cotter, Sylvia Doan, Mounif El-Youssef, Federica Ferrari, Katryn N Furuya, Madeleine Gottrand, Frederic Gottrand, Nitika Gupta, Matjaz Homan, M K Jensen, Binita M Kamath, Kyung Mo Kim, Kaija-Leena Kolho, Anastasia Konidari, Bart Koot, Raffaele Iorio, Oren Ledder, Cara Mack, Mercedes Martinez, Tamir Miloh, Parvathi Mohan, Niamh O'Cathain, Alexandra Papadopoulou, Amanda Ricciuto, Lawrence Saubermann, Pushpa Sathya, Eyal Shteyer, Vratislav Smolka, Atushi Tanaka, Raghu Varier, Veena Venkat, Bernadette Vitola, Miriam B Vos, Marek Woynarowski, Jason Yap
BACKGROUND: There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long-term outcomes of pediatric PSC. METHODS: We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death...
April 8, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28237379/readmission-after-gastrointestinal-bleeding-in-children-a-retrospective-cohort-study
#2
Thomas M Attard, Mikaela Miller, Chaitanya Pant, Mike Thomson
INTRODUCTION: To compare the demographic, clinical, and therapeutic characteristics in a cohort of patients discharged following acute gastrointestinal bleeding, representing to the emergency department (ED) and readmitted within 30 days of discharge with the characteristics of non-readmitted patients. STUDY DESIGN: Hospitalization data was obtained from the Pediatric Hospital Information System including 49 tertiary children's hospitals in the US. Children 1-21 years of age diagnosed with acute gastrointestinal bleeding, admitted between January 2007 and September 2015 were included...
May 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28107286/liver-stiffness-measured-by-shear-wave-elastography-for-evaluating-intra-hepatic-portal-hypertension-in-children
#3
Hee Mang Yoon, So Yeon Kim, Kyung Mo Kim, Seak Hee Oh, Gi-Young Ko, Yangsoon Park, Jin Seong Lee, Ah Young Jung, Young Ah Cho
OBJECTIVES: To correlate liver stiffness (LS) and hepatic venous-pressure gradient (HVPG) and to evaluate the diagnostic performance of shear-wave elastography (SWE) for predicting clinically significant portal hypertension in children with suspected liver diseases, in consideration of the reliability criteria. METHODS: We identified 33 SWEs from 32 children who underwent HVPG measurement within two weeks between June 2012 and October 2015. The correlation between LS and HVPG was assessed...
January 20, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28099341/laparoscopic-distal-splenoadrenal-shunt-for-the-treatment-of-portal-hypertension-in-children-with-congenital-hepatic-fibrosis-a-case-report
#4
Jin-Shan Zhang, Wei Cheng, Long Li
BACKGROUND: The distal splenorenal shunt is an effective procedure for the treatment of portal hypertension in children. However, there has been no report about laparoscopic distal splenorenal shunt in the treatment of portal hypertension in children. METHODS: From December 2015 to August 2016, 4 children with upper gastrointestinal bleeding underwent laparoscopic distal splenoadrenal shunt. Portal hypertension and splenomegaly were demonstrated on the preoperative computed tomography (CT) and sonography...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28079234/evaluation-of-endoscopic-secondary-prophylaxis-in-children-and-adolescents-with-esophageal-varices
#5
Júlio Rocha Pimenta, Alexandre Rodrigues Ferreira, Eleonora Druve Tavares Fagundes, Paulo Fernando Souto Bittencourt, Alice Mendes Moura, Simone Diniz Carvalho
BACKGROUND: - Bleeding of esophageal varices is the main cause of morbidity and mortality in children and adults with portal hypertension and there are few studies involving secondary prophylaxis in children and adolescents. OBJECTIVE: - To evaluate the efficacy of endoscopic secondary prophylaxis in prevention of upper gastrointestinal bleeding in children and adolescents with esophageal varices. METHODS: - This is a prospective analysis of 85 patients less than 18 years of age with or without cirrhosis, with portal hypertension...
January 2017: Arquivos de Gastroenterologia
https://www.readbyqxmd.com/read/28045719/hepatic-histology-and-morphometric-measurements-in-idiopathic-extrahepatic-portal-vein-thrombosis-in-children-correlated-to-clinical-outcome-of-meso-rex-bypass
#6
Niramol Tantemsapya, Riccardo Superina, Deli Wang, Grace Kronauer, Peter F Whitington, Hector Melin-Aldana
OBJECTIVE: The aim of this study was to correlate clinical, histologic, and morphometric features of the liver in children with extrahepatic portal vein thrombosis (EHPVT), with surgical outcome after Meso-Rex bypass (MRB). BACKGROUND: Idiopathic EHPVT, a significant cause of portal hypertension, is surgically corrected by MRB. Correlation of histologic and morphometric features of the liver with outcome has not been reported in children. METHODS: We retrospectively reviewed clinical and intraoperative data of 45 children with idiopathic EHPVT...
December 30, 2016: Annals of Surgery
https://www.readbyqxmd.com/read/28003725/neonatal-cholestasis-as-initial-presentation-of-portosystemic-shunt-a-case-report
#7
Güzide Doğan, Fatih Düzgün, Serdar Tarhan, Yeliz Çağan Appak, Erhun Kasırga
Congenital intrahepatic portosystemic shunts are rare in children. Portosystemic venous malformations are characterized by extreme clinical variability. We report a full-term 33-day-old male infant presenting with neonatal jaundice. On physical examination, he had generalized icterus and the liver was palpable 3.5 cm below the right costal margin. He had no other symptoms. Laboratory tests showed AST 632 U/L, ALT 198 U/L, total bilirubin 12.1 mg/dL, conjugated bilirubin 10.2 mg/dL, alkaline phosphatase 753 U/L, GGT 47 U/L and glucose 67 U/L...
December 2016: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/27906797/non-cirrhotic-portal-fibrosis-in-pediatric-population
#8
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
#9
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...
April 2017: 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
#10
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
#11
Odile Jeanniard-Malet, Mathieu Duché, Alexandre Fabre
Primary prophylaxis in portal hypertension in children is controversial, because there are few studies documenting its efficacy on the risk of bleeding. Twenty-eight 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 >90% of centres perform sclerotherapy or endoscopic variceal ligation...
April 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27782957/correlation-of-transient-elastography-with-severity-of-cystic-fibrosis-related-liver-disease
#12
Amal Aqul, Maureen M Jonas, Sarah Harney, Roshan Raza, Gregory S Sawicki, Paul D Mitchell, Rima Fawaz
OBJECTIVES: The aim of the study was to evaluate whether liver stiffness measurement (LSM), determined by transient elastography, correlates with presence and severity of liver disease in children and young adults with cystic fibrosis (CF). METHODS: Subjects underwent LSM at routine CF visits. Presence and severity of cystic fibrosis liver disease (CFLD) was determined by clinical parameters. Subjects were classified as no CFLD, CFLD without portal hypertension (PHTN), and CFLD with PHTN...
April 2017: 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
#13
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...
February 2017: 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
#14
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
#15
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-noninvasive-marker-of-portal-hypertension-in-pediatric-chronic-liver-disease
#16
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 and 2012 were included...
April 2017: 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
#17
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
#18
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...
December 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27663417/portal-hypertension-in-children-high-risk-varices-primary-prophylaxis-and-consequences-of-bleeding
#19
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...
February 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/27661550/-management-of-pediatric-patients-with-esophageal-varices
#20
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
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