We report a case of secondary sclerosing cholangitis that manifested itself during pregnancy. A tentative diagnosis of intrahepatic cholestasis of pregnancy was considered, but after her third delivery, a liver biopsy and imaging, as well as review of past records, confirmed the diagnosis of secondary sclerosing cholangitis. Maternal and fetal outcomes of primary sclerosis cholangitis have been...

А im: to highlight the importance of broad differential diagnosis and possibility conversion benign recurrent intrahepatic cholestasis type 2 into more aggressive clinical phenotype. Key points. A 19-year-old female patient was admitted Clinic with skin pruritus, jaundice, dark urine, clay-colored stool, general fatigue. Past medical history significant for aforementioned symptoms since 3 years...

Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal recessive inherited disorder characterized by multiple recurrent episodes of severe cholestatic jaundice without obstruction of extrahepatic bile duct. We present the case of a 7-year-old boy with BRIC confirmed by mutation analysis in the ATP8B1 gene and typical clinical manifestation. Despite inheritance of BRIC, we detected...

Cholestasis is a common sequela of liver transplantation. Although the majority of cases remain subclinical, severe cholestasis may be associated with irreversible liver damage, requiring retransplantation. Therefore, it is essential that clinicians be able to identify and treat the syndromes associated with cholestasis. In this review, we consider causes of intrahepatic cholestasis. These may ...

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....

To determine whether a defect in uptake of organic anions is present in patients with benign recurrent cholestasis, the plasma disappearance of simultaneously injected indocyanine green and cholyglycine-1-14C was measured in eight patients with asymptomatic benign recurrent cholestasis, four with the familial type, as well as 22 healthy control subjects. Evans blue was also simultaneously injec...