E. Michel, Ö. Kilavuz, R. Jäger, R. Nasir
Biliary atresia due to delayed maturation of the gut hormones system? – Introducing a new treatment modality
Background: Congenital biliary atresia is suspected to
originate from prenatal biliary duct inflammation of
unknown etiology. Objective: Based on clinical grounds,
we aimed to establish a hypothesis on the primary cause
of inflammation, and to suggest a causal treatment
modality. Case report: History. A 28 years old Turkish
woman had lost her first child aged two years from congenital
biliary atresia (parents second degree cousins).
After a miscarriage, in her otherwise uneventful third
pregnancy sonography at 34 wks revealed echogenic
material in the fetal gallbladder. Nine days later the gallbladder
was completely filled with sludge. Chemical
inflammation was suspected, and birth was induced at
36+3 weeks in order to allow for surgical flushing of the
bile duct. Neonatal clinical chemistry was insuspicious.
There was no spontaneous resolution of the sludge within
the first 24 hours of life. A trial of medical treatment
with intermittent iv secretin (0.03 CU/kg/h) and iv coeruletid
(60 ng/kg/h) was started. Within 24 hours, sludge
had resolved. Conclusions: We hypothesize that dysmaturation
may lead to insufficient induction/production/activity of intrinsic gut hormones resulting in prenatally
impaired bile flow, or even inspissated bile. Familial
occurrence suggests a genetic defect. Exogenous hormone
therapy might be an appropriate treatment
modality.
Journal of Perinatal Medicine, Walter de Gruyter
Print ISSN: 1619-3997
Volume: 32, 05/2004
Pages: 288 - 292
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