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. 2021 Jun;7(2):205-214.
doi: 10.5114/ceh.2021.107066. Epub 2021 Jun 22.

Variant etiologies of neonatal cholestasis and their outcome: a Middle East single-center experience

Mohamed Abdel-Salam El-Guindi  1 , Magdy Anwar Saber  1 , Samar Ahmed Shoeir  1 , Ayat Roushdy Abdallah  2 , Ahmad Mohamed Sira  1
Affiliations

Variant etiologies of neonatal cholestasis and their outcome: a Middle East single-center experience

Mohamed Abdel-Salam El-Guindi et al. Clin Exp Hepatol. 2021 Jun.

Abstract

Aim of the study: Neonatal cholestasis (NC) constitutes a large proportion of pediatric liver disorders. Nevertheless, awareness of the variant etiologies and how to manage them appropriately are lacking. So, out of a few specialized centers, many cases pass without appropriate management. This study aimed to present our tertiary level center's experience in NC that could increase the pediatrician's awareness of handling this problematic and common medical morbidity efficiently.

Material and methods: It is a retrospective study in which we analyzed the NC cases admitted to the inpatient department within three years. For all recruited patients, the available data were retrieved and recorded.

Results: A total of 412 patients were reviewed with 20 different etiologies diagnosed. The most common cause was biliary atresia (n = 151, 37%), followed by progressive familial intrahepatic cholestasis (n = 51, 12%), neonatal sepsis (n = 39, 9%), and cytomegalovirus (n = 33, 8%). Of the 412 patients, 394 (81%) had follow-up ranging from 1 to 36 months. A total of 173 patients improved with supportive and/or specific therapy, while 108 patients died at a median age of 6 months. The commonest cause of death was liver failure (40.7%), followed by pneumonia (28.7%), sudden death (13%), septicemia (6.5%), and hepatorenal syndrome (5.5%).

Conclusions: NC constitutes more than one-third of the inpatient admissions of all pediatric liver disorders and has a high rate of mortality. Awareness of the variety of etiologies and a rapid stepwise approach to diagnosis could have an impact on the outcome of this devastating disease.

Keywords: Kasai portoenterostomy; biliary atresia; idiopathic neonatal hepatitis; progressive familial intrahepatic cholestasis.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Fig. 1
Fig. 1
Included and excluded cases within the study with different variants of the reported neonatal cholestasis etiologies
Fig. 2
Fig. 2
Algorithm for a diagnostic approach to neonatal cholestasis
See this image and copyright information in PMC

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