Progressive familial intrahepatic cholestasis (PFIC) is characterized by early onset cholestasis, progressive liver cirrhosis, pruritus, poor growth and inexorable progression to liver cirrhosis in early childhood. The serum level of gamma-glutamyl transferase is low or normal, which is discordant with severe cholestasis. Five Malaysian patients with PFIC, who all had typical features of PFIC w...

Cholestasis has limited therapeutic options and is associated with high morbidity and mortality. The A(1) adenosine receptor (A(1)AR) was postulated to participate in the pathogenesis of hepatic fibrosis induced by experimental extrahepatic cholestasis; however, the contribution of A(1)AR to intrahepatic cholestatic liver injury remains unknown. Here, we found that mice lacking A(1)AR were resi...

Intestinal origin alkaline phosphatase activity (ALP) in plasma was measured by a sensitive immunocapture assay in 104 jaundiced patients--84 with intrahepatic and 20 with post-hepatic jaundice. Increased enzyme activities were observed in those with intrahepatic disease and subnormal values in those with post-hepatic disease. At a discriminant level intestinal origin ALP showed a diagnostic se...

Background: Intrahepatic cholangiocarcinoma (IHCCA) is an aggressive tumor for which surgical resection is a mainstay of treatment. However, recurrence after resection is common associated with a poor prognosis. Studies regarding recurrence of mass-forming IHCCA are rare; therefore, we investigated the pattern with our dataset. Methods: We retrospectively reviewed the medical and pathological r...

The histopathology of the liver is fundamental for the differential diagnosis between intra- and extrahepatic causes of neonatal cholestasis. However, histopathological findings may overlap and there is disagreement among authors concerning those which could discriminate between intra- and extrahepatic cholestasis. Forty-six liver biopsies (35 wedge biopsies and 11 percutaneous biopsies) and on...

Itching is a common complaint in pregnancy, occurring in up to 14 to 23% of women. Pruritus may be so severe that it affects sleep and quality of life. It has a prevalence of 0.7 to 5% in different populations.1 Prevalence is influenced by genetic and environmental factors and varies between populations worldwide. In Chile, 2.4% of all pregnancies are affected with a 5% prevalence in women of A...

BACKGROUND Partial External Biliary Diversion (PEBD) is a surgical intervention to treat children with Progressive Familial Intrahepatic Cholestasis (PFIC) and Alagille syndrome (AGS). PEBD can reduce disease progression, and examining the alterations in biliary lipid composition may be a prognostic factor for outcome. METHODS Biliary lipid composition and the clinical course of AGS and PFIC ...

The article is devoted to the most common liver disease which associated with pregnancy – obstetric cholestasis. frequency of this pathology among all disorders in pregnant women almost 27%, second only viral hepatitis. On average, it diagnosed 2–4 cases per 1000 pregnancies. A characteristic feature cholestasis tendency recurrent course future pregnancies, observed 60–70% next This disorder us...

Cholestasis may result from a failure in bile secretion in hepatocytes or ductular cells, or from a blockade to the free bile flow. Human cholestasis may be induced by many drugs, being antibiotics the more common. Other types of cholestasis seen in humans are a group of familial cholestatic disorders, obstructive cholestasis, primary biliary cirrhosis, extrahepatic biliary atresia, primary scl...