Management of pregnancy in Crigler Najjar syndrome type 2.
Crigler Najjar syndrome is associated with indirect hyperbilirubinemia due to a deficiency of enzyme Uridine Di Phospho Glucoronosyl Transferase (UDPGT). Presented here is a case of a female in the first trimester of pregnancy, who was diagnosed to have type 2 Crigler Najjar syndrome. We also discuss the management of this rare disease especially in pregnancy. Unconjugated bilirubin can cross the placental barrier causing neurological damage in the newborn. Patient was carefully monitored during pregnancy and treatment with phenobarbitone in low doses was adjusted such that the serum bilirubin levels were below 10 mg/dL. Crigler Najjar syndrome being rare needs to be diagnosed early in pregnancy to avoid adverse fetal outcomes. Phenobarbitone being an inducer of enzyme UDPGT is used as the first line of treatment and is not teratogenic in the low doses used. Treatment protocol followed was on the basis of previous reported cases and successful perinatal outcome was achieved.
Alisha Nitin Chaubal, Ruchir Patel, Dhaval Choksi, Kaivan Shah, Meghraj Ingle, Prabha Sawant, Department of Gastroenterology, LTMG Hospital, Mumbai 400022, India.
Alisha Nitin
AN
Chaubal
Alisha Nitin Chaubal, Ruchir Patel, Dhaval Choksi, Kaivan Shah, Meghraj Ingle, Prabha Sawant, Department of Gastroenterology, LTMG Hospital, Mumbai 400022, India.
Ruchir
R
Patel
Alisha Nitin Chaubal, Ruchir Patel, Dhaval Choksi, Kaivan Shah, Meghraj Ingle, Prabha Sawant, Department of Gastroenterology, LTMG Hospital, Mumbai 400022, India.
Dhaval
D
Choksi
Alisha Nitin Chaubal, Ruchir Patel, Dhaval Choksi, Kaivan Shah, Meghraj Ingle, Prabha Sawant, Department of Gastroenterology, LTMG Hospital, Mumbai 400022, India.
Kaivan
K
Shah
Alisha Nitin Chaubal, Ruchir Patel, Dhaval Choksi, Kaivan Shah, Meghraj Ingle, Prabha Sawant, Department of Gastroenterology, LTMG Hospital, Mumbai 400022, India.
Meghraj
M
Ingle
Alisha Nitin Chaubal, Ruchir Patel, Dhaval Choksi, Kaivan Shah, Meghraj Ingle, Prabha Sawant, Department of Gastroenterology, LTMG Hospital, Mumbai 400022, India.
Prabha
P
Sawant
Crigler Najjar type 2; Folic acid; Kernicterus; Phenobarbitone; Pregnancy