Abstract
BackgroundNeonatal cholestasis (NC) is a major cause of morbidity and mortality in young infants. This study examines the etiology of NC and its outcome during 2 years of follow-up at a tertiary referral center in Bangladesh.ResultsOut of 80 cholestatic infants, 60% had intrahepatic cholestasis with a mean age of onset of 12.4±2.8 days and a mean age of admission of 82.4±29.0 days. The remaining 40% were extrahepatic with a mean age of onset of 6.7±2.3 days and a mean age of admission of 94.6±50.4 days. Biliary atresia (BA), idiopathic neonatal hepatitis (INH), and TORCH (Toxoplasma, rubella, cytomegalovirus, and herpes simplex) infection except rubella were the most common causes. After receiving treatment, 46.2% of the cases improved, 23.8% deteriorated with morbidity, and 30% died. The majority of the children with INH, TORCH, choledochal cyst, hypothyroidism, galactosemia, and urinary tract infection (UTI) with sepsis were improved. Significant mortality was found in BA (56.6%), intrahepatic bile duct paucity (PIBD) (100%), and progressive familial intrahepatic cholestasis (PFIC) (100%) whereas the rest of BA (43.4%) live with persistent morbidity. Significant clinical improvement was observed in 37 (46.2%) cases of cholestasis evidenced by decreasing jaundice, change of color of urine from dark to normal color, change of stool color from pale to yellow, and gradual decrease in liver size from hepatomegaly state. In addition, decreasing median total bilirubin, direct bilirubin, alanine transaminase, gamma-glutamyl transferase, and alkaline phosphatase showed biochemical improvement at 2 years follow-up. The age of admission, etiology, and presence of ascites are the predictors of outcomes.ConclusionBA was the most common cause of extrahepatic while INH and TORCH infection were the most common cause of intrahepatic cholestasis. Majority of children with intrahepatic cholestasis improved but deteriorated with BA and genetic causes. Prompt referral and early diagnosis as well as the etiology of NC were the main determinants of the favorable outcome.
Highlights
Neonatal cholestasis (NC) is a major cause of morbidity and mortality in young infants
Study design A retrospective cohort study was done in the Department of Pediatric Gastroenterology, Hepatology & Nutrition, Dhaka Shishu (Children) Hospital, Dhaka, Bangladesh, among 80 infants admitted to the hospital from July 2014 to June 2016 with cholestatic jaundice and were followed up monthly for a minimum of 2 years
The mean age of onset of intra and extrahepatic cases were significantly different (p value
Summary
Neonatal cholestasis (NC) is a major cause of morbidity and mortality in young infants. This study examines the etiology of NC and its outcome during 2 years of follow-up at a tertiary referral center in Bangladesh. Neonatal cholestasis (NC) is a reduced bile formation or flow with retention of biliary substances inside the liver, which can be presented as conjugated hyperbilirubinemia. Mahmud et al Egyptian Liver Journal (2022) 12:1 parenteral nutrition, and chromosomal anomalies as well as familial syndromes [10]. A low or normal GGT suggests the presence of progressive familial intrahepatic cholestasis (PFIC type or PFIC type 2) [4, 7, 12,13,14,15]. Liver biopsy (LB) is considered as one of the most reliable investigations for the diagnosis of BA, with an accuracy of 88.2–96.9% [20, 21]. The gold standard for the diagnosis of BA is a per-operative cholangiogram which has a diagnostic accuracy of 100% [1, 12, 20, 21]
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