BACKGROUND Intrahepatic cholestasis of pregnancy (ICP) affects approximately 0.7% of pregnancies in the UK and is associated with prematurity, fetal distress, and intrauterine death. Homozygous mutations in the ATP8B1 gene cause cholestasis with a normal serum gamma-glutamyl transpeptidase (gamma-GT), and have been reported in two forms of cholestasis: progressive familial intrahepatic cholesta...

Nutritional status of 380 hospitalised children aged from 1 month to 5 years with liver disease was evaluated in a single paediatric centre. The total prevalence of stunting (height-for-age Z (HAZ) < -2), underweight (weight-for-age Z (WAZ) < -2) and wasting (weight-for-height Z < -2) was 9·8, 9·0 and 7·9 %, respectively. The overall nutritional risk (-2 ≤ Z < -1) of stunting, underweight and w...

BACKGROUND & AIMS To date, no studies concerning the presence of small intestinal bacterial overgrowth in patients with progressive familial intrahepatic cholestasis were published. Based upon characteristic of progressive familial intrahepatic cholestasis one can expect the coexistence of small intestinal bacterial overgrowth. The aim of the study was to assess the incidence of small intestina...

OBJECTIVE To verify blood levels of vitamin D in patients with chronic cholestasis, and relate them to nutritional status, length of time since the onset of cholestasis and use of vitamin supplement. METHODS Controlled cross-sectional study with chronic cholestasis as study factor and blood levels of vitamin D as outcome. The study included patients aged between 4 months and 18 years, who wer...

Hepatocyte injury during cholestasis depends in part on the release of proinflammatory mediators that cause neutrophils to accumulate in the liver and become activated to damage hepatocytes. The mechanism by which cholestasis stimulates production of proinflammatory mediators in the liver is not completely understood. The studies presented here tested the hypothesis that the transcription facto...

background: neonatal cholestasis is an important infant’s diseases of liver and biliary, they are wide range of situation including; idiopathic neonatal hepatitis, extrahepatic biliary tract atresia, choledochal cysts and infectious diseases. lack of early diagnosis of the disease can be followed by irreparably complications. the aim of this study was determination the histopathological causes ...

Neonatal cholestasis is a potentially life-threatening condition requiring prompt diagnosis. Mutations in several different genes can cause progressive familial intrahepatic cholestasis, but known genes cannot account for all familial cases. Here we report four individuals from two unrelated families with neonatal cholestasis and mutations in NR1H4, which encodes the farnesoid X receptor (FXR),...

Progressive familial intrahepatic cholestasis type 3 (PFIC3) is an autosomal recessive disorder of cholestasis of hepatocellular origin, typically seen in infancy or childhood caused by a defect in the ABCB4 located on chromosome 7. Here we report on an older patient, aged 15, who presented with biochemical testing that led to an initial consideration of a diagnosis of Wilson disease (WD) resul...

A middle-aged woman suffering from jaundice and pruritus that had begun a month previously was presented to a physician.At the first assessment, laboratory findings had revealed a cholestatic pattern and serologic tests for hepatitis B virus (HBV), hepatitis C virus (HCV) and hepatitis A virus (HAV) were negative. Normal findings of abdominal computed tomography (CT) scan and endoscopic retrogr...

Intrahepatic cholestasis of pregnancy is the most common pregnancy-specific liver disease that typically presents in the third trimester. The clinical features are maternal pruritus in the absence of a rash and deranged liver function tests, including raised serum bile acids. Intrahepatic cholestasis of pregnancy is associated with an increased risk of adverse perinatal outcomes, including spon...