| Biliary atresia due to delayed maturation of the gut hormones' system?--Introducing a new treatment modality. | |
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MedLine Citation:
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PMID: 15188807 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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 i.v. secretin (0.03 CU/kg/h) and i.v. 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. |
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Authors:
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Erik Michel; Omer Kilavuz; Ralph Jäger; Rohangiz Nasir |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Journal of perinatal medicine Volume: 32 ISSN: 0300-5577 ISO Abbreviation: J Perinat Med Publication Date: 2004 |
Date Detail:
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Created Date: 2004-06-10 Completed Date: 2004-08-26 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 0361031 Medline TA: J Perinat Med Country: Germany |
Other Details:
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Languages: eng Pagination: 288-92 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, Neukölln General Hospital, Berlin, Germany. Erik.Michel@t-online.de |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Biliary Atresia / diagnosis*, embryology, genetics, therapy, ultrasonography Diagnosis, Differential Female Genetic Predisposition to Disease Humans Infant, Newborn Male Pregnancy Ultrasonography, Prenatal* |
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