The synthesis of bilirubin glucuronide in animal and human liver
between bilirubin levels and C3 ..
This disorder is clinicallyand biochemically similar to benign intrahepatic cholestasis. Itoccurs in the third trimester of pregnancy when the estrogen level is the highestand disappears postpartum. The affected subjects appear to belong to familieswith benign intrahepatic cholestasis trait. Gonadal steroid appear to ply a determiningrole in the cause of this syndrome. Histology of the liver showscentrolobular cholestasis similar to benign intrahepatic cholestasis. It is mostfrequent in Scandinavia (1/100), Bolivia and Chile (1/10).The disorder is safefor the mother but not for the fetus who will suffer premature births andstillbirths due to placental infarcts. The mothers have higher incidence ofgallstones. Sometimes the disorder manifests itself only with presence ofpruritus without jaundice. (Pruritus gravidarum). The patients are notseverely ill as in fatty liver of pregnancy, hepatitis, obstructive jaundice.
Porphyrin and Heme Synthesis and Bilirubin Metabolism
Many drugs producecholestasis. The first cases reported were due to chlopromazine and syntheticsteroids now out of market (Nilavar). Synthetic oral contraceptives are high inthe list. They appeared to act on sensitivity base and affect only sensitiveindividuals. Many appear to impair the secretory function of the hepatocytes. Andthe list is increasing with the advent of new drugs. The liver in these casesmay show
Heme to Bilirubin in liver to gall bladder ..
It is due to avery transient insufficiency of glucuronyl transferase. During the first fewdays of life there is an overproduction of bilirubin and an underdevelopedmechanism of the liver to dispose of bilirubin.
Defects in Protein Synthesis-Jong | Liver | Cirrhosis
Is due to a severedeficiency of glucuronyl tranferase. Deep jaundice develops tat birth, Highserom unconjugated hyprbilirubinemia, >20 mg/dl., not responding tophenobarbital. Absent formation of diglucuronides. Death usually in the firstyear or two with kernicterus. Phototherapy, plasmaferesis and albumin exchangeare beneficial. Liver transplantation may be life-saving. The liver ishistologically normal. A similar condition exists in Gann rat. Fortunately this syndromeis rare. Only 100 or more cases have been described. It is apparently ahereditary autosomal recessive trait.