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Pediatric Portal Hypertension

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https://www.readbyqxmd.com/read/28260452/unusual-indications-for-a-liver-transplant-a-single-center-experience
#1
Aydincan Akdur, Mahir Kirnap, Ebru H Ayvazoglu Soy, Figen Ozcay, Gokhan Moray, Gulnaz Arslan, Mehmet Haberal
OBJECTIVES: This study sought to evaluate the efficacy of liver transplant for unusual liver diseases. MATERIALS AND METHODS: The results of 476 patients who underwent liver transplant from 1988 to January 2015 were retrospectively analyzed. Two hundred forty-five of them were adult patients and 231 of them were pediatric. Thirty-one patients had unusual liver disease. RESULTS: Of the 31 patients with unusual liver disease, 9 (29%) were adult and 22 (71%) were pediatric patients...
February 2017: Experimental and Clinical Transplantation
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...
February 23, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28115278/the-role-of-thromboelastography-in-pediatric-patients-with-sinusoidal-obstructive-syndrome-receiving-defibrotide
#3
Joanna L Gendreau, Christine Knoll, Roberta H Adams, Leon L Su
Sinusoidal obstructive syndrome (SOS) is a potentially fatal form of hepatic injury after hematopoietic stem cell transplantation. Patients can develop liver dysfunction, portal hypertension, ascites, coagulopathies, and multisystem organ failure. The mortality rate of severe SOS has been reported as high as 98% by day 100 after transplantation. Defibrotide, which is now approved for the treatment of SOS, has significantly decreased mortality. Defibrotide is a polynucleotide with profibrinolytic, anti-ischemic, and anti-inflammatory activity...
January 20, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28003725/neonatal-cholestasis-as-initial-presentation-of-portosystemic-shunt-a-case-report
#4
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
#5
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/27895519/portal-hypertension-an-uncommon-clinical-manifestation-of-takayasu-arteritis-in-a-9-year-old-child
#6
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/27706456/evaluation-of-primary-prophylaxis-with-propranolol-and-elastic-band-ligation-in-variceal-bleeding-in-cirrhotic-children-and-adolescents
#7
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/27661550/-management-of-pediatric-patients-with-esophageal-varices
#8
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/27504083/very-early-presentation-of-extrahepatic-portal-vein-obstruction-causing-portal-hypertension-in-an-infant-uncertainties-in-the-management-and-therapeutic-limitations
#9
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
#10
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/27229494/body-mr-angiography-in-children-how-we-do-it
#11
REVIEW
Rajesh Krishnamurthy, LaDonna Malone, Karen Lyons, Pamela Ketwaroo, Nicholas Dodd, Daniel Ashton
Vascular pathology is ubiquitous in children. Common indications for angiographic imaging in the body include congenital anomalies, portal hypertension, assessing resectability of neoplasms, renovascular hypertension, vascular malformations, vasculitis, systemic vein thrombosis, and trauma. MR angiography, with or without the use of intravenous contrast agents, is therefore a mainstay in the repertoire of MR imaging in children. Pediatric contrast-enhanced MR angiography has benefited from several innovations in recent years, including improved hardware options like high-field-strength scanners and integrated high-density coil arrays, new sequences that combine parallel imaging, innovative k-space sampling and Dixon fat suppression with time-resolved imaging, new contrast agents with longer blood-pool residence time, and advanced post-processing solutions like image fusion...
May 2016: Pediatric Radiology
https://www.readbyqxmd.com/read/27083897/usefulness-of-a-recanalized-umbilical-vein-for-vascular-reconstruction-in-pediatric-hepatic-surgery
#12
Masato Shinkai, Kyoko Mochizuki, Norihiko Kitagawa, Hiroshi Take, Hidehito Usui, Kaori Nakamura Yamoto, Shogo Fujita, Youkatsu Ohhama
PURPOSE: Pediatric surgeons currently engage in various abdominal vascular surgeries, which sometimes require vascular conduits or grafts. Herein, we report our experience with patients undergoing vascular reconstruction using a recanalized umbilical vein (rUV) and their long-term outcome. METHOD: Five patients with extrahepatic portal vein obstruction (EHPVO) underwent mesenterico-/porto-left portal vein (PV) bypass surgery using a short rUV conduit with an interposition vein graft...
June 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/26992455/autoimmune-hepatitis-in-a-child-presenting-with-hepatopulmonary-syndrome-hps
#13
Zoe Grabinski, Mirza Beg, Prateek Wali
HPS has been described in 9-20% of children with end-stage liver disease. We present a case of a previously, asymptomatic nine-yr-old incidentally found to have low oxygen saturation. Physical exam was remarkable for digital clubbing, splenomegaly and orthodeoxia. Laboratory evaluation revealed a low platelet count, hyperammonemia, and prolonged coagulation studies. Sonography showed evidence of splenomegaly and portal venous hypertension. High resolution CT thorax and CTA were normal. HPS was confirmed by agitated saline contrast enhanced echocardiography and Tc-99m MAA scan with evidence of intrapulmonary vascular dilatations...
May 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/26963461/4d-contrast-enhanced-mr-angiography-with-the-keyhole-technique-in-children-technique-and-clinical-applications
#14
Rajesh Krishnamurthy, Sara M Bahouth, Raja Muthupillai
Unlike in adults, contrast agent-enhanced magnetic resonance (MR) angiography in the pediatric population raises unique challenges such as faster heart rates, more rapid arteriovenous transit, smaller structures, smaller volumes of contrast agent used, and more complex disease processes. A need exists for a rapid contrast-enhanced MR angiographic technique that can separate the arterial and venous phases of contrast enhancement in sedated pediatric patients breathing freely during the course of an examination...
March 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/26904975/long-term-clinical-outcome-of-budd-chiari-syndrome-in-children-after-radiological-intervention
#15
Varun K Sharma, Prajakta R Ranade, Shaji Marar, Fazal Nabi, Aabha Nagral
OBJECTIVES: Budd-Chiari syndrome (BCS) is an uncommon cause of chronic liver disease in children. The literature on the management of pediatric BCS is scarce. Our aim was to determine the long-term outcome of patients undergoing a radiological intervention for the treatment of BCS. METHODS: Thirty-two children diagnosed with BCS between 2004 and 2014 were included. Data on the course of disease, medical management, response, and complications related to radiological interventions and outcome were collected...
May 2016: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/26640930/liver-transplant-outcomes-in-a-newly-started-program
#16
COMPARATIVE STUDY
Tokan Sultanaliev, Assan Zhexembayev, Adilbek Mukazhanov, Bakhyt Zharkymbekov, Gani Kuttymuratov, Zhanat Spatayev, Bauyrzhan Ibrayev, Kakharman Yesmembetov, Aiymkul Ashimkhanova, Mels Asykbayev
OBJECTIVES: Kazakhstan is experiencing a high demand for liver transplants. More than 1000 patients have end-stage liver disease in the country, and liver transplant is the only viable option for their treatment. MATERIALS AND METHODS: Liver transplant patients, treated from February 2013 to December 2014, were included in this retrospective analysis. RESULTS: From February 2013 to December 2014, seven patients received a liver transplant in our center (1 pediatric patient was excluded)...
November 2015: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/26586487/fontan-associated-liver-disease-implications-for-heart-transplantation
#17
REVIEW
Steven C Greenway, David S Crossland, Mark Hudson, Steven R Martin, Robert P Myers, Tim Prieur, Asif Hasan, Richard Kirk
Chronic liver diseases are associated with multiple complications, including cirrhosis, portal hypertension, ascites, synthetic dysfunction and hepatocellular carcinoma, and these processes are increasingly recognized in post-Fontan patients. Fontan-associated liver disease (FALD) can be defined as abnormalities in liver structure and function that result from the Fontan circulation and are not related to another disease process. FALD arises due to chronic congestion of the liver created by the elevated venous pressure and low cardiac output of the Fontan circulation, which may be superimposed on previous liver injury...
January 2016: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/26361673/acute-liver-failure-in-an-adult-a-rare-complication-of-alagille-syndrome-case-report-and-brief-review
#18
REVIEW
F Frongillo, G Bianco, N Silvestrini, M C Lirosi, A M Sanchez, E Nure, R Gaspari, A W Avolio, G Sganga, S Agnes
Alagille syndrome (AS) is an autosomal-dominant, multisystem disorder affecting the liver, heart, eyes, skeleton, and face. The manifestations are predominantly pediatric. Diagnosis is based on findings of a paucity of bile ducts on liver biopsy combined with ≥3 of 5 major clinical criteria. Orthotopic liver transplantation (OLT) is the only option for treating patients who developed liver failure, portal hypertension, severe itching, and xanthomatosis. It is difficult to establish clear criteria for OLT; indications are controversial because of the wide variety of clinical symptoms and the multisystem involvement...
September 2015: Transplantation Proceedings
https://www.readbyqxmd.com/read/26358549/primary-prophylaxis-of-variceal-bleeding-in-children-and-the-role-of-mesorex-bypass-summary-of-the-baveno-vi-pediatric-satellite-symposium
#19
REVIEW
Benjamin L Shneider, Jean de Ville de Goyet, Daniel H Leung, Anshu Srivastava, Simon C Ling, Mathieu Duché, Patrick McKiernan, Riccardo Superina, Robert H Squires, Jaime Bosch, Roberto Groszmann, Shiv K Sarin, Roberto de Franchis, George V Mazariegos
UNLABELLED: Approaches to the management of portal hypertension and variceal hemorrhage in pediatrics remain controversial, in large part because they are not well informed by rigorous clinical studies. Fundamental biological and clinical differences preclude automatic application of approaches used for adults to children. On April 11-12, 2015, experts in the field convened at the first Baveno Pediatric Satellite Meeting to discuss and explore current available evidence regarding indications for MesoRex bypass (MRB) in extrahepatic portal vein obstruction and the role of primary prophylaxis of variceal hemorrhage in children...
April 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/26223427/aspartate-aminotransferase-to-platelet-ratio-and-fibrosis-4-as-biomarkers-in-biopsy-validated-pediatric-cystic-fibrosis-liver-disease
#20
Daniel H Leung, Mahjabeen Khan, Charles G Minard, Danielle Guffey, Louise E Ramm, Andrew D Clouston, Gregory Miller, Peter J Lewindon, Ross W Shepherd, Grant A Ramm
UNLABELLED: Up to 10% of cystic fibrosis (CF) children develop cirrhosis by the first decade. We evaluated the utility of two simple biomarkers, aspartate aminotransferase to platelet ratio index (APRI) and FIB-4, in predicting degree of fibrosis in pediatric CF liver disease (CFLD) validated by liver biopsy. In this retrospective, cross-sectional study, 67 children with CFLD had dual-pass liver biopsies and 104 age- and sex-matched CF children without liver disease (CFnoLD) had serum to calculate APRI and FIB-4 collected at enrollment...
November 2015: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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