Question

What long-term disease is a complete or partial deficiency in UDPG-T that causes a conjugation failure leading to hepatic jaundice?

a) Wilson's disease
b) Dubin-Johnson syndrome
c) Crigler-Najjar syndrome
d) Hemochromatosis

Answer 1

Crigler-Najjar syndrome is a long-term disease that results in hepatic jaundice due to a deficiency in UDPG-T and conjugation failure. It is characterized by the absence of UDP glucuronyl transferase activity in the liver cells.

Step-by-step explanation:

The long-term disease that is a complete or partial deficiency in UDPG-T, causing a conjugation failure leading to hepatic jaundice, is Crigler-Najjar syndrome. This syndrome is due to the absence of UDP glucuronyl transferase activity in the liver cells. There are two types of Crigler-Najjar syndrome, with type I having a serum bilirubin level exceeding 20 mg/dl and type II where the liver can form bilirubin monoglucuronide but cannot further glucuronate it due to the absence of UDP glucuronyl transferase-II.