Cholestatic Jaundice Information

Jaundice caused by thickened bile or bile plugs in the small biliary passages of the liver. Cholestatic jaundice of pregnancy is a condition characterized by pruritis, icterus, or both during pregnancy. A total of 9 pregnancies complicated by cholestatic jaundice are presented. Three of these cases are presented in detail because they reflect complications that can occur with this condition. The literature on cholestatic jaundice is reviewed. This condition has been regarded in the past as benign to the mother and fetus, but these cases and other recent investigations suggest high perinatal mortality and morbidity rates.

Cholestatic Jaundice Information

The saga of neonatal cholestasis syndrome (NCS) continues ever since Dr John Cooke in 1769 referred to mortality occurring in infants due to jaundice. The presenting clinical features of NCS are jaundice, dark urine, with or without passage of pale stools. The major stumbling block in the management of these babies is the varied etiology of this condition. This includes biliary atresia, choledochal cyst, giant cell hepatitis, intrauterine infections, ductal paucity, and metabolic defects. The final outcome of many of these causes is dependent on early diagnosis and timely management, while the presenting clinical features are usually alike.

Cholestatic jaundice during infancy

More than 80% of cases with extrahepatic biliary atresia (EHBA), one of the major causes of NCS, who undergo Kasai portoenterostomy before 60 days of age become jaundice-free, as compared to 20%-35% operated on later.1 Of the infants with successful biliary drainage, a 15-year survival of 87% has been shown.2 Subgroups of NCS other than EHBA also need early and
targeted management. Timely treatment of metabolic causes like galactosemia and tyrosinemia, choledochal cyst, and infections, and early recognition of disorders like ductal paucity and progressive familial intrahepatic cholestasis will decrease the morbidity due to late presentation. The consequences of infantile cholestasis are profound, resulting in malabsorption, failure to thrive, and deficiencies of fat-soluble vitamins. NCS babies are at
special risk for life-threatening bleeding due to vitamin K deficiency. These babies need more calories to maintain
growth, as also supplementation of vitamins A, D, E and K at diagnosis and thereafter.

In this issue of the Journal, Bazlul Karim and Kamal from Bangladesh,3 in their study of 62 infants with
cholestatic jaundice, highlighted the etiology, delay in presentation of cases with EHBA, importance of acholic stools, and investigative modalities to diagnose EHBA. In their study an identifiable cause for neonatal hepatitis was seen in 35.5% of cases, EHBA in 25.8%, idiopathic neonatal hepatitis in 24.2%, choledochal cyst in 6.5%, and miscellaneous disorders in 8%. In a report we published earlier based on pooled data from eight medical centers in India,4 among 1008 analyzed cases of NCS, hepatocellular causes were seen in 53% (neonatal hepatitis 47%, metabolic 4%, and 2% other causes), obstructive etiology in 38% (biliary atresia 34%, choledochal cyst 4%), ductal paucity in 3%, and 6% were idiopathic. Among those with neonatal hepatitis (n=468), diopathic giant cell hepatitis constituted 64%, TORCH infections 22% (including cytomegalovirus in 58% of these, toxoplasmosis in 23%, hepatitis B in 10%, rubella in 4.5%, syphilis in 4%, and herpes in 1%), sepsis 8%, and other causes like malaria, urinary tract infection 6%. Among the metabolic group (n=43) 35% were due to galactosemia, 33% tyrosinemia, 4% had storage disorders, and 2% had hemochromatosis. Ductal paucity (n=29) was due to nonsyndromic variety in 83%.