holelithiasis in neonates is usually pigmentary and composed of calcium bilirubinate.1,2 In children, cholelithisasis and inspissated bile or sludge are associated with prematurity, cystic fibrosis, hemolytic diseases, hemorrhage, congenital heart disease, starvation, total parenteral nutrition, infection, dehydration, ileal resection, abdominal surgery, congenital anomalies of the biliary trac...

AIM To describe the clinical phenotype in infants with ARC syndrome, the association of arthrogryposis, renal tubular acidosis, and cholestasis. METHODS The medical records for six patients with ARC syndrome were reviewed, presenting over 10 years to three paediatric referral centres. RESULTS All patients had the typical pattern of arthrogryposis. Renal Fanconi syndrome was present in all b...

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...

Aim—To describe the clinical phenotype in infants with ARC syndrome, the association of arthrogryposis, renal tubular acidosis, and cholestasis. Methods—The medical records for six patients with ARC syndrome were reviewed, presenting over 10 years to three paediatric referral centres. Results—All patients had the typical pattern of arthrogryposis. Renal Fanconi syndrome was present in all but o...

Elevated serum level of bilirubin is a common manifestation which is occurred in several diseases. Hyperbilirubinemia can manifest either conjugated or unconjugated. Conjugated or direct hyperbilirubinemia usually are caused by hepatocellular diseases or cholestatic liver diseases. Merely conjugated hyperbilirubinemia is the main manifestation of two congenital syndromes, including Dubin-Johnso...

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...

CONTEXT Biliary obstruction secondary to intrabiliary mucin is a relatively rare presentation of malignant intraductal pancreatic mucinous tumor. We report a case of unresectable intraductal pancreatic mucinous tumor associated with obstructive jaundice secondary to intrabiliary mucin. This case and a review of the literature, highlight the difficulty in obtaining sustained palliation from jaun...