Non cirrhotic portal hypertension in children

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...

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...

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...

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...

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.

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...

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...

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...

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...

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...

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...

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...

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...

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...

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...

BACKGROUND AND OBJECTIVES: Non-cirrhotic portal fibrosis (NCPF) is an infrequent cause of portal hypertension in children. We report 11 children with NCPF, from among 338 with portal hypertension, seen over 6.5 years. METHODS: The diagnosis was based on patent splenoportal axis on ultrasonography and/or splenoportal venography and liver biopsy showing no evidence of cirrhosis or other diagnosis, in children with portal hypertension. Those with variceal bleed were managed with endoscopic sclerotherapy and/or shunt surgery...

Portal Hypertension (PH) is the commonest cause of upper gastrointestinal bleeding in children. Most Indian studies have highlighted extrahepatic portal venous obstruction (EHPVO) as the major cause of PH in children. As there is paucity of data from the eastern part of the country we decided to study the major causes of PH in children in this region and to ascertain the efficacy of sclerotherapy for its management. Fifty children aged 14 months to 10 years with PH were studied from April 1990 to April 1995...

Thirty-one children with variceal bleeding due to portal hypertension (extrahepatic obstruction 19, non-cirrhotic portal fibrosis five, and cirrhosis of liver seven patients) were treated with endoscopic sclerotherapy with absolute alcohol. Acute variceal bleeding was successfully controlled in 10 patients by emergency sclerotherapy. A 3 weekly schedule of sclerotherapy could achieve obliteration of varices in all the patients. The mean (+/- SD) number of sclerotherapy courses and the time required for variceal eradication was 4...

Signs of portal hypertension, history of upper gastro-intestinal tract bleeding episodes and outcome of the latter were recorded in 76 cirrhotic children evaluated for liver transplantation. Fifty-three (70%) had varices and 22 (29%) had experienced upper gastro-intestinal tract bleeding. Of these 22, 19 bled from varices and 3 from ulcers. Non bleeding ulcers were also found in five patients bleeding from varices. Iterative sclerotherapy controlled acute variceal bleeding in all but one patient in whom emergency transplantation was performed...