Biliary atresia-splenic malformation (BASM) syndrome: a case report

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dc.contributor.authorAlhashmi, Hadeil
dc.contributor.authorChawshly, Esra
dc.contributor.authorÇelebi, Süleyman
dc.date.accessioned2024-07-12T10:43:14Z
dc.date.available2024-07-12T10:43:14Z
dc.date.issued2024en_US
dc.departmentFakülteler, Tıp Fakültesi, Cerrahi Tıp Bilimleri Bölümüen_US
dc.descriptionArticle number: 109937
dc.description.abstractIntroduction and importance: Biliary atresia (BA) is characterized by the presence of persistent cholestatic jaundice during the neonatal period. This group of patients is at higher risk for liver disease and/or portal hypertension compared with other chronic liver diseases. Case presentation: We present a newborn patient who had biliary stools early postnatally, but her gallbladder was not seen on radiological examination and was referred to our clinic. On examination, polysplenia was seen, and the inferior vena cava was not seen. The patient's stool color changed to a creamy stool without bile three weeks postnatally. The neonate was diagnosed with biliary atresia and splenic malformation (BASM) syndrome. The patient underwent a Kasai portoenterostomy in the 3rd week of age and was preoperatively diagnosed with malrotation and treated with a Ladd procedure at the same time. The patient had a postoperative stool with bile and decreased bilirubinemia with a normal defecation period. Clinical discussion: BASM syndrome, clinically has different causes and a worse prognosis than isolated BA. The syndrome can lead to end-stage cirrhosis and liver failure if left untreated. Although timely Kasai surgery is the standard treatment for BA, the age of the patient at the time of the surgery is different depending on BA groups. Conclusion: The most important prognostic factors for BASM syndrome are early diagnosis and treatment. Despite this, the patients need careful follow-up. Postoperative adjuvant steroid therapy may have a good impact on the outcomes.en_US
dc.identifier.citationAlhashmi, H., Chawshly, E., Çelebi, S. (2024). Biliary atresia-splenic malformation (BASM) syndrome: a case report. International Journal of Surgery Case Reports, 121. 10.1016/j.ijscr.2024.109937en_US
dc.identifier.issn2210-2612
dc.identifier.scopus2-s2.0-85196511352
dc.identifier.scopusqualityQ3
dc.identifier.urihttps://hdl.handle.net/20.500.12939/4740
dc.identifier.volume121en_US
dc.identifier.wosWOS:001259692700001
dc.identifier.wosqualityN/A
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.institutionauthorAlhashmi, Hadeil
dc.institutionauthorChawshly, Esra
dc.institutionauthorÇelebi, Süleyman
dc.language.isoen
dc.publisherElsevier Ltden_US
dc.relation.ispartofInternational Journal of Surgery Case Reports
dc.relation.isversionof10.1016/j.ijscr.2024.109937en_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - İdari Personel ve Öğrencien_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectBASMen_US
dc.subjectBiliary atresiaen_US
dc.subjectCase reporten_US
dc.subjectKasai portoenterostomyen_US
dc.subjectNeonatal cholestatic jaundiceen_US
dc.titleBiliary atresia-splenic malformation (BASM) syndrome: a case report
dc.typeArticle

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