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Non cirrhotic portal hypertension in children

Stephanie Barbara Schatz, Christoph Jüngst, Verena Keitel-Anselmo, Ralf Kubitz, Christina Becker, Patrick Gerner, Eva-Doreen Pfister, Imeke Goldschmidt, Norman Junge, Daniel Wenning, Stephan Gehring, Stefan Arens, Dirk Bretschneider, Dirk Grothues, Guido Engelmann, Frank Lammert, Ulrich Baumann
Genetic variants in the adenosine triphosphate-binding cassette subfamily B member 4 ( ABCB4 ) gene, which encodes hepatocanalicular phosphatidylcholine floppase, can lead to different phenotypes, such as progressive familial intrahepatic cholestasis (PFIC) type 3, low phospholipid-associated cholelithiasis, and intrahepatic cholestasis of pregnancy. The aim of this multicenter project was to collect information on onset and progression of this entity in different age groups and to assess the relevance of this disease for the differential diagnosis of chronic liver disease...
May 2018: Hepatology Communications
Rajeev Khanna, Shiv Kumar Sarin
BACKGROUND: Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are non-cirrhotic vascular causes of portal hypertension (PHT). Variceal bleed and splenomegaly are the commonest presentations. AIM: The present review is intended to provide the existing literature on etiopathogenesis, clinical profile, diagnosis, natural history and management of IPH and EHPVO. RESULTS: IPH and EHPVO are both characterized by normal hepatic venous pressure gradient, moderate to massive splenomegaly with preserved liver synthetic functions...
February 2018: Hepatology International
N Patel, A Grieve, J Hiddema, J Botha, J Loveland
BACKGROUND: Portal hypertension is a common and potentially devastating condition in children. Notwithstanding advances in the nonsurgical management of portal hypertension, surgery remains an important treatment modality in select patients. We report here on our experience in the past 12 years. OBJECTIVES: To describe the profile of, indication for, and complications of shunt surgery in children with portal hypertension. METHODS: Twelve children underwent shunt surgery between 2005 and 2017...
November 6, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
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
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
Bart G P Koot, Marielle Alders, Joanne Verheij, Ulrich Beuers, Jan M Cobben
Non-cirrhotic portal hypertension is characterized by histopathological abnormalities in the liver, mostly affecting small intrahepatic portal veins that cause portal hypertension in the absence of cirrhosis. It can be secondary to coagulation disorders or toxic agents. However, most cases are idiopathic non-cirrhotic portal hypertension (INCPH) and familial cases are rare. We report a family in which a father and three of his four children conceived with three different mothers are affected by INCPH. Whole exome and Sanger sequencing showed the father to have a de novo single nucleotide substitution c...
April 2016: Journal of Hepatology
Vibhor V Borkar, Ujjal Poddar, Aditya Kapoor, Srivatsa Ns, Anshu Srivastava, Surender K Yachha
BACKGROUND & AIMS: Hepatopulmonary syndrome in children has not been extensively studied particularly in different subsets of portal hypertension. We prospectively studied hepatopulmonary syndrome in children with cirrhosis and extrahepatic portal venous obstruction. Their comparison might shed light on the pathogenesis of hepatopulmonary syndrome. METHODS: Between 1 to 18 years of age, 135 children with portal hypertension (cirrhosis 35, extrahepatic portal venous obstruction 100) were studied...
June 2015: Liver International: Official Journal of the International Association for the Study of the Liver
Rabea Alhosh, Yuri Genyk, Sophoclis Alexopoulos, Daniel Thomas, Shengmei Zhou, George Yanni, Nanda Kerkar
HPS is a significant complication of portal hypertension in children with chronic liver disease and is an established indication for LT. It is characterized clinically by the triad of pulmonary vascular dilatation causing hypoxemia in the setting of advanced liver disease. NRH, a cause of non-cirrhotic portal hypertension, is characterized by diffuse benign transformation of the hepatic parenchyma into small regenerative nodules with minimal or no fibrosis. Development of NRH and HPS in pediatric LT recipients has not been reported, although occasional cases have been reported in adult LT recipients...
August 2014: Pediatric Transplantation
Abhik Sinha, Tryambak Samanta, Sarmila Mallik, Dipak Pal, Sutapa Ganguly
Non-cirrhotic portal fibrosis (NCPF) is reported to be a very infrequent cause of portal hypertension (PHT) in paediatric population, but a number of cases of NCPF were found in the paediatric ward of NRS Medical College and Hospital, Kolkata which prompted us to do a study with objectives to find out the proportion of NCPF cases among the study population (the total number of PHT cases admitted in the study period) and their sociodemographic profile with special reference to residence in arsenic affected areas...
December 2011: Journal of the Indian Medical Association
Chiara Grimaldi, Jean de Ville de Goyet, Valerio Nobili
The main causes of intrahepatic portal hypertension in children are cirrhosis and congenital hepatic fibrosis. Non cirrhotic portal hypertension in children is mostly due to extrahepatic portal vein obstruction. In half of cases, no underlying disorder is found. The meso-Rex bypass is the preferred treatment, when it is possible. The closest to the portal vein the obstruction, the highest the risk of esophagogastric varices.
June 2012: Clinics and Research in Hepatology and Gastroenterology
Sinan Sari, Deniz Oguz, Tugba Sucak, Buket Dalgic, Tamer Atasever
BACKGROUND: Hepatopulmonary syndrome (HPS) is defined as an arterial oxygenation defect induced by intrapulmonary vascular dilatation (IPVD) associated with hepatic disease. The prevalence and clinical characteristics of HPS in portal hypertensive children is not well characterized. AIMS: The aim of this study was to investigate the prevalence and clinical characteristics of HPS in 40 portal hypertensive children. METHODS: We studied 40 children (11 girls and 29 boys; mean age, 111 months ± 52 months; range, 24-216 months) with portal hypertension (24 cirrhotic, 16 non-cirrhotic) for the presence of HPS using blood gas analysis, contrast-enhanced echocardiography (CEE), and Tc99m-macroaggregated albumin scintigraphy...
January 2012: Digestive Diseases and Sciences
R B Pinto, P E Fröehlich, E H Pitrez, J A Bragatti, J Becker, A F H Cornely, A C R Schneider, T R da Silveira
BACKGROUND: Few studies have evaluated abnormalities on brain magnetic resonance imaging (MRI) in children and adolescents with chronic liver disease. AIMS: The aim of this study was to investigate the presence of T1 hyperintensity in the basal ganglia of pediatric patients with portal hypertension and its association with blood manganese levels. METHODS: A case control study of 22 patients with portal hypertension (14 Child-Pugh A cirrhosis, 8 non-cirrhotic portal hypertension) and 15 controls was conducted from 2006 to 2007...
February 2010: Neuropediatrics
E F Georgescu, Ligia Stănescu, Daniela Dumitrescu, Reanina Ionescu, Iuliana Georgescu
UNLABELLED: Sturge-Weber syndrome is a rare disorder consisting of a port-wine nevus in the distribution of the ophthalmic branch of the trigeminal nerve and central nervous system malformations. Facial cutaneous vascular malformation, seizures, and glaucoma are among the most common symptoms and signs. The syndrome results from malformation of the cerebral vasculature located within the pia mater, most commonly over the occipital region. These malformations led to venous hypertension and subsequent hypoperfusion on the underlying cortex, causing chronic cerebral ischemia, atrophy, calcification and neurological deterioration...
2007: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
Ajay Vora, Chris D Mitchell, Lynne Lennard, T O B Eden, Sally E Kinsey, John Lilleyman, Sue M Richards
BACKGROUND: 6-mercaptopurine has been a standard component of long-term continuing treatment for childhood lymphoblastic leukaemia, whereas 6-thioguanine has been mainly used for intensification courses. Since preliminary data have shown that 6-thioguanine is more effective than 6-mercaptopurine, we compared the efficacy and toxicity of the two drugs for childhood lymphoblastic leukaemia. METHODS: Consecutive children with lymphoblastic leukaemia diagnosed in the UK and Ireland between April, 1997, and June, 2002, were randomly assigned either 6-thioguanine (750 patients) or 6-mercaptopurine (748 patients) during interim maintenance and continuing therapy...
October 14, 2006: Lancet
Surender Kumar Yachha, Vikrant Khanna
Ascites is a common clinical problem in children with liver disease. The peripheral arterial vasodilation hypothesis is mostly accepted as the pathophysiological basis of ascites. The most important complication is spontaneous ascitic fluid infection in the form of spontaneous bacterial peritonitis (SBP) and its variants. Aerobic gram-negative bacteria, primarily Escherichia coli, are the most common isolates. Diagnostic paracentesis is done in patients with ascites when diagnosed first time and at the beginning of each admission to hospital...
September 2006: Indian Journal of Pediatrics
Jing-Tong Wang, Hui-Ying Zhao, Yu-Lan Liu
BACKGROUND: Most physicians in China may neglect portal vein thrombosis (PVT) in clinical practice. In fact, portal vein thrombosis is an important cause of non-cirrhotic portal hypertension. As the diversity of its clinical manifestations, misdiagnosis is common if we donot bear PVT in mind during differential diagnosis. Therefore, we systematically reviewed PVT in terms of etiology, pathophysiology, pathology, clinical manifestations, and management. DATA SOURCES: An English language literature search (from 1980 to 2004) was performed using Medline and Medscape, and articles closely related to PVT were selected...
November 2005: Hepatobiliary & Pancreatic Diseases International: HBPD INT
Sema Aydogdu, Ipek Akil, Talha Akil, Caner Kabasakal, Refik Killi, Sevgi Mir, Rasit Yagci
BACKGROUND: The aim of the present study was to investigate renal vascular resistive changes in children with different stages of liver cirrhosis without obvious renal failure. METHODS: Twenty-nine children (14 girls, 15 boys, mean age 11.6 years) with cirrhosis and 20 healthy children (mean age 10.3 years) were investigated for renal vascular resistance with Doppler ultrasonography, urinary sodium, N-acetyl-beta-D glucosaminidase (NAG) and microalbuminuria excretion...
February 2004: Pediatrics International: Official Journal of the Japan Pediatric Society
S K Yachha, Kamal Chetri, Richa Lal
Portal hypertension (PHT) is common in children and a majority of cases in India are constituted by extrahepatic portal venous obstruction or cirrhosis of liver. Morbidity and mortality in this condition is related to variceal bleeding, most commonly from esophageal varices. Acute variceal bleeding is best controlled by endoscopic therapy. Somatostatin and octreotide are useful in acute variceal bleeding as a supplementary therapy. Acute variceal bleeding uncontrolled by medical therapy merits preferably a shunt surgery or devascularization depending upon etiology of PHT and expertise of the surgeon...
September 2002: Indian Journal of Pediatrics
D Sharma, S Agrawal, A Saxena, V K Raina
In developing countries surgery is indicated in patients with portal hypertension for a variety of reasons. This study prospectively evaluates a modified technique of devascularization for secondary prophylaxis of variceal bleeding in patients with portal hypertension of different aetiologies. Transabdominal extensive oesophagogastric devascularization combined with transmural ligation of oesophageal and gastric varices was performed in 16 paediatric patients (nine with extrahepatic portal venous obstruction, and seven with non-cirrhotic portal fibrosis) in an elective setting...
April 2001: Tropical Doctor
G M Trenschel, A Schubert, V Dries, G Benz-Bohm
BACKGROUND: Nodular regenerative hyperplasia (NRH) of the liver is a multi-acinar regenerative nodular lesion in a non-cirrhotic liver. It is a rare entity, especially in children, and remains of unknown aetiology. OBJECTIVE: NRH is often seen in association with other diseases or drug intake. In half of patients it is complicated by portal hypertension. Radiologically, its nodular appearance may look like neoplasia. RESULTS: We report a case of NRH with enormous hepatomegaly and multiple huge nodules...
January 2000: Pediatric Radiology
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