You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information.
What is Which of the following pigments accumulates in liver in Dubin-Johnson syndrome? and explain in detail?
Ans. b (Epinephrine metabolite) (Ref. Robbins Pathologic basis of disease 7th/ 887-888; Table 18-4)Dubin-Johnson syndrome# Hereditary defect in hepatocellular excretion of bilirubin glucuronides across the canalicular membrane due to absence of the canalicular protein, the multidrug resistance protein 2 (MRP2; located on chromosome 10q24), that isresponsible for transport of bilirubin glucuronides and related organic anions into bile.# The liver is darkly pigmented owing to coarse pigmented granules within the cytoplasm of hepatocytes.# Electron microscopy reveals that the pigment is located in lysosomes, and it appears to be composed of polymers of epinephrine metabolites., not bilirubin pigment.# The liver is otherwise normal.# Apart from chronic /recurrent jaundice (fluctuating intensity), patients are asymptomatic and have normal life span.OTHER HEREDITARY HYPERBILIRUBINEMIASCrigler-Najjar syndrome type I# The enzyme UGT1A1 is completely absent.6# The liver is incapable of synthesizing a functional enzyme, and the colorless bile contains only trace amounts of unconjugated bilirubin.# The liver is morphologically normal by light and electron microscopy.# However, serum unconjugated bilirubin reaches very high levels, producing severe jaundice and icterus.# Without liver transplantation, this condition is invariably fatal.Crigler-Najjar syndrome type II# It is a less severe, nonfatal disorder in which UGT1A1 enzyme activity is greatly reduced, and the enzyme is capable of forming only monoglucuronidated bilirubin.# Almost all patients develop normally and the only major consequence is extraordinarily yellow skin from high levels of circulating unconjugated bilirubin; but there is a risk of neurologic damage from kemicterus.# Phenobarbital improves bilirubin glucuronidation by inducing hypertrophy of hepatic endoplasmic reticulum.Gilbert syndrome# It is a relatively common, benign, somewhat heterogeneous inherited condition presenting with mild, fluctuating hyperbilirubinemia.# Main cause is reduction in hepatic bilirubin glucuronidating activity (UGT1A1) to about 30% of normal.# Gilbert syndrome has no clinical consequence except for the anxiety.Rotor syndrome# It is a rare form of asymptomatic conjugated hyperbilirubinemia with multiple defects in hepatocellular uptake and excretion of bilirubin pigments.# The liver is not pigmented.# As with Dubin-Johnson syndrome, patients with Rotor syndrome exhibit jaundice but otherwise live normal lives.