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Article Type

Article

Subject Area

Pediatric

Abstract

Background Cholestatic liver disease constitutes a large percentage of chronic liver diseases during infancy. Cholestasis is defined as interference with bile formation or flow owing to pathology anywhere between the hepatocyte and the ampulla of Vater. Aim To evaluate the different modalities used for diagnosing cholestasis in infants in Damanhour Medical National Institute (DMNI) to find out the sensitivity and the predictive values of each modality in defining the cause of cholestatic jaundice, whether surgical or medical. Patients and methods This study was a retrospective one which included 153 infants who were referred with cholestasis in first year of life to the Pediatric Hepatology Clinic of the Children's Department (DMNI) from June 2013 to December 2018. The medical records of the 153 infants with confirmed cholestasis were reviewed regarding the history and clinical examination, laboratory data, ultrasound, and liver biopsy. The cases were classified into two groups according to the etiology. Group I comprised (Extrahepatic Biliary Atresia) EHBA, and there were 40 patients in this group. Group II comprised non-EHBA (the medical causes of cholestasis), and there were 113 patients in this group. Results Nearly half (52%) of the patients were females. There were no significant differences regarding sex between EHAB and non-EHBA patients. An enlarged liver was also a common finding, being present in more than 66% of infants, irrespective of the underlying cause. Splenomegaly was more commonly noted in group II (27.43%) versus group I (12.5%), but the differences were not statistically significant. Persistently day stools were observed by 95% of mothers of babies of group I compared with 15% in group II, with a highly significant statistical difference. No significant differences were found between group I and group II regarding most tests of the biochemical profile, except Gamma-glutamyl transferase (GGT) and alkaline phosphatase (AL Ph), as they were significantly higher in group I compared with group II. However, aspartate transaminase (AST), alanine transaminase (ALT), and partial thromboplastin time were significantly higher in group II compared with group I. Conclusion and recommendations It is recommended the use of the developed model, GGT, and AL Ph as first line of investigations, and follow-up for excluding EHBA. Urgent referral of patients to perform a liver biopsy should be done based on the calculated probability of the developed model more than 0.128, GGT more than 500, and Al Ph more than 600. Campaigns to increase awareness among parents and primary care doctors are recommended for early drug and management.

Keywords

Infancy, medical cause of cholestasis, noninvasive methods, surgical

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