Cholestasis results from impairment in the excretion of bile, which may be due to mechanical obstruction of bile flow or impairment of excretion of bile components into the bile canaliculus. When present, cholestasis warrants prompt diagnosis and treatment. The differential diagnosis of cholestasis beyond the neonatal period is broad and includes congenital and acquired etiologies. It is impera...

One hundred and twenty-four infants admitted to hospitals in Norway between 1955 and 1974 during the first 3 months of life with cholestatic jaundice were studied retrospectively. Sixty-four infants had had extrahepatic atresia of the biliary tree and 60 had had intrahepatic cholestasis. This gives an incidence of about 1:9000 live births for cholestasis. In 4 of the 64 infants with extra-hepat...

OBJECTIVE To review animal and human data available regarding the etiology, maternal and fetal impact, and treatment of intrahepatic cholestasis of pregnancy (ICP). METHODS Pertinent studies on human and animal models of ICP were selected through a MEDLINE database search, focusing on etiology and clinical impact of the disease. Analytic and descriptive studies were included, and the data wer...

Liver specimens from three cases of the so-called intrahepatic cholestasis were submitted to graphic reconstructions of the cholangiolar system by means of serial histological sections and were examined for obstructive changes of the system. The results were evaluated in comparison with those obtained with a normal liver. Although routine histological examinations failed to demonstrate any sign...

Class III multidrug resistance (MDR) P-glycoproteins (P-gp), mdr2 in mice and MDR3 in man, mediate the translocation of phosphatidylcholine across the canalicular membrane of the hepatocyte. Mice with a disrupted mdr2 gene completely lack biliary phospholipid excretion and develop progressive liver disease, characterized histologically by portal inflammation, proliferation of the bile duct epit...

A male infant presented with intrahepatic cholestasis due to idiopathic eosinophilia damaging the biliary epithelium. No other etiological agent or cause could be identified. He responded well to prednisone therapy.

Progressive familial intrahepatic cholestasis (PFIC) includes a group of infrequent genetic diseases with autosomal recessive heredity, characterized by intrahepatic cholestasis, usually in childhood and in adolescence. It is caused by defective bile salt secretion and other bile components. The progression leading to liver failure and cirrhosis usually appears in the first few decades of life....

Intrahepatic cholestasis of pregnancy (ICP) represents a rare but severe pathology with serious consequences on the outcome of pregnancy. We present the cases of two sisters that came to our clinic with ICP in successive pregnancies. The fetus from a pregnancy with cholestasis can be affected by preterm birth, respiratory distress syndrome, intrauterine death in the third trimester and a possib...

Intractable pruritus secondary to bile salts retention in Progressive Familial Intrahepatic Cholestasis (PFIC) can be relieved surgically by diverting bile drainage from ileum to reduce bile salts reabsorption into entero-hepatic circulation. We are reporting on the successful biliary diversion in a child with PFIC, with the use of the appendix as a conduit to drain bile from gallbladder to the...

Serum liver function tests were estimated in 57 patients admitted to an Intensive Therapy Unit (ITU) with a diagnosis of septic shock. Following an initial biochemical disturbance, persisting hyperbilirubinaemia was associated with a poor prognosis. Post-mortem liver histology in 22 patients showed varying degrees of non-specific reactive change, venous congestion, ischaemic necrosis, fatty cha...