Etiology and outcome of neonatal cholestasis: an experience in a tertiary center of Bangladesh

• Published in: Egyptian Liver Journal 2022-01, Vol.12 (1), p.1-11
• Main Authors: Mahmud, Salahuddin, Gulshan, Jahida, Parvez, Mashud, Tasneem, Farhana, Ahmed, Syed Shafi
• Format: Article
• Language: English
• Subjects: Bangladesh; Biliary atresia; Bilirubin; Causes and theories of causation; Child; Cholestasis; Development and progression; Diseases; Galactosemia; Health aspects; Hypothyroidism; Idiopathic neonatal hepatitis; India; Infants (Newborn); Jaundice, Obstructive; Liver; Mortality; Neonatal cholestasis; Outcome; Phosphatases; Thyroid hormones; Urinary tract infections
• ISSN: 2090-6218; 2090-6226
• DOI: 10.1186/s43066-021-00168-7

Background 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. Results Out 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. Conclusion BA 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.