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biliary atresia with splenic malformation syndrome - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

biliary atresia with splenic malformation syndrome

MONDO:0016577

Also known as:BASM syndrome

Overview

Biliary atresia with splenic malformation syndrome (BASM) designates the association of biliary atresia and splenic abnormalities (mainly polysplenia and less frequently asplenia, double spleen). Cardiac defect, situs inversus and a preduodenal portal vein can also be present. It represents the embryonal or syndromic form of biliary atresia. It affects newborns or infants and is characterized by jaundice, pale stools, dark urine, failure to thrive, hepatomegaly, coagulopathy, anemia and often palpable spleen.

ResearchLimited published research (7 articles)

Prognosis

Estimated prevalence: Unknown (Unknown prevalence).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for biliary atresia with splenic malformation syndrome.

7 publications have been identified in PubMed for biliary atresia with splenic malformation syndrome. Research spans Case Report / Case Series (57%), Review / Meta-Analysis (14%), and Clinical Trial Publication (14%).

Recent Publications

Gebreziher YT (2026). [PMID: 41815465](https://pubmed.ncbi.nlm.nih.gov/41815465/). *International journal of surgery case reports*. [Clinical Trial Publication]
Hong B (2025). [PMID: 40384652](https://pubmed.ncbi.nlm.nih.gov/40384652/). *Quantitative imaging in medicine and surgery*. [Case Report / Case Series]
Davenport M (2025). [PMID: 39857926](https://pubmed.ncbi.nlm.nih.gov/39857926/). *Children (Basel, Switzerland)*. [Case Report / Case Series]
Davenport M (2025). [PMID: 40519534](https://pubmed.ncbi.nlm.nih.gov/40519534/). *World journal of pediatric surgery*. [Review / Meta-Analysis]
Schwarz D (2025). [PMID: 40907883](https://pubmed.ncbi.nlm.nih.gov/40907883/). *Journal of pediatric surgery*. [Epidemiology / Natural History]

Data assembled from 3 of 12 sources · Last updated Sep 20, 2026, 6:03 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:244283

GARD

Genetic and Rare Diseases Info Center

GARD:20652

Questions for Your Care Team

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biliary atresia with splenic malformation syndrome

Overview

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team