Neonatal Cholestasis in Two Siblings: a Variant of Dubin-Johnson Syndrome?

Overview

Journal J Paediatr Child Health

Specialty Pediatrics

Date 1995 Dec 1

PMID 8924312

Citations 4

Authors

A Kimura

K YUGE

K I Kosai

M Kage

T Fujisawa

T Inoue

Y Yamashita

E Nakashima

H Kato

Affiliations

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Abstract

Objective: Two Japanese brothers with neonatal cholestasis associated with pigment granules in the hepatocytes and hepatosteatosis were evaluated for the possible role of hepatosteatosis in the Dubin-Johnson syndrome.

Methodology And Results: The morphology of pigment accumulation and the laboratory data in these cases were examined. The elevation of urinary coproporphrin isomer I to more than 90% and the presence cholestasis resembled that in the Dubin-Johnson syndrome, but the hypertriglyceridaemia ( > 1.13 mmol/L as triolein) and the hepatosteatosis differed. Both infants were thought to have familial hypertriglyceridaemia. However, this diagnosis was difficult to confirm in the absence of data on the normal values of apolipoprotein and lipoprotein isomer for infants.

Conclusions: A neonatal variant of the Dubin-Johnson syndrome may account for the unusual findings in these infants.

Citing Articles

Clinical, Biochemical, and Molecular Characterization of Neonatal-Onset Dubin-Johnson Syndrome in a Large Case Series From the Arabs.

Al-Hussaini A, Alsaleem B, AlHomaidani H, Asery A, Alruwaithi M, Alameer M Front Pediatr. 2021; 9:741835.

PMID: 34858902 PMC: 8631451. DOI: 10.3389/fped.2021.741835.

[Clinical features and ABCC2 genotypic analysis of an infant with Dubin-Johnson syndrome].

Meng L, Qiu J, Lin W, Song Y Zhongguo Dang Dai Er Ke Za Zhi. 2019; 21(1):64-70.

PMID: 30675866 PMC: 7390169.

Heparin-binding epidermal growth factor-like growth factor and hepatocyte growth factor inhibit cholestatic liver injury in mice through different mechanisms.

Sakamoto K, Khai N, Wang Y, Irie R, Takamatsu H, Matsufuji H Int J Mol Med. 2016; 38(6):1673-1682.

PMID: 27779646 PMC: 5117744. DOI: 10.3892/ijmm.2016.2784.

Genetic and biochemical study of dual hereditary jaundice: Dubin-Johnson and Gilbert's syndromes. Haplotyping and founder effect of deletion in ABCC2.

Slachtova L, Seda O, Behunova J, Mistrik M, Martasek P Eur J Hum Genet. 2015; 24(5):704-9.

PMID: 26350512 PMC: 4930088. DOI: 10.1038/ejhg.2015.181.