Updates in Biliary Atresia: Aetiology, Diagnosis and Surgery
Biliary atresia (BA) is an obliterative disease of the bile ducts affecting between 1 in 10,000–20,000 infants with a predominance in Asian countries. It is clinically heterogeneous with a number of distinct variants (e.g., isolated, Biliary Atresia Splenic Malformation syndrome, Cat-eye syndrome, c...
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2025-01-01
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| author | Mark Davenport |
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| description | Biliary atresia (BA) is an obliterative disease of the bile ducts affecting between 1 in 10,000–20,000 infants with a predominance in Asian countries. It is clinically heterogeneous with a number of distinct variants (e.g., isolated, Biliary Atresia Splenic Malformation syndrome, Cat-eye syndrome, cystic BA, and CMV-associated BA). Facts about its aetiology are hard to encounter but might include genetic, developmental, exposure to an environmental toxin, or perinatal virus infection. However, the cholestatic injury triggers an intrahepatic fibrotic process beginning at birth and culminating in cirrhosis some months later. Affected infants present with a triad of conjugated jaundice, pale stools, and dark urine and may have hepatosplenomegaly upon examination, with later ascites coincident with the onset of progressive liver disease. Rapid, efficient, and expeditious diagnosis is essential with the initial treatment being surgical, typically with an attempt to restore the bile flow (Kasai portoenterostomy (KPE)) or primary liver transplantation (<5%) if considered futile. Failure to restore bile drainage or the onset of complications such as recurrent cholangitis, treatment-resistant varices, ascites, hepatopulmonary syndrome, and occasionally malignant change are usually managed by secondary liver transplantation. This issue summarises recent advances in the disease and points a way to future improvements in its treatment. |
| format | Article |
| id | doaj-art-df3f2a3f01094e20ac046958746e752f |
| institution | Kabale University |
| issn | 2227-9067 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Children |
| spelling | doaj-art-df3f2a3f01094e20ac046958746e752f2025-01-24T13:27:17ZengMDPI AGChildren2227-90672025-01-011219510.3390/children12010095Updates in Biliary Atresia: Aetiology, Diagnosis and SurgeryMark Davenport0Department of Paediatric Surgery, Kings College Hospital, Denmark Hill, London SE5 9RS, UKBiliary atresia (BA) is an obliterative disease of the bile ducts affecting between 1 in 10,000–20,000 infants with a predominance in Asian countries. It is clinically heterogeneous with a number of distinct variants (e.g., isolated, Biliary Atresia Splenic Malformation syndrome, Cat-eye syndrome, cystic BA, and CMV-associated BA). Facts about its aetiology are hard to encounter but might include genetic, developmental, exposure to an environmental toxin, or perinatal virus infection. However, the cholestatic injury triggers an intrahepatic fibrotic process beginning at birth and culminating in cirrhosis some months later. Affected infants present with a triad of conjugated jaundice, pale stools, and dark urine and may have hepatosplenomegaly upon examination, with later ascites coincident with the onset of progressive liver disease. Rapid, efficient, and expeditious diagnosis is essential with the initial treatment being surgical, typically with an attempt to restore the bile flow (Kasai portoenterostomy (KPE)) or primary liver transplantation (<5%) if considered futile. Failure to restore bile drainage or the onset of complications such as recurrent cholangitis, treatment-resistant varices, ascites, hepatopulmonary syndrome, and occasionally malignant change are usually managed by secondary liver transplantation. This issue summarises recent advances in the disease and points a way to future improvements in its treatment.https://www.mdpi.com/2227-9067/12/1/95biliary atresiaKasai portoenterostomyBASMadjuvant therapyliver transplantation |
| spellingShingle | Mark Davenport Updates in Biliary Atresia: Aetiology, Diagnosis and Surgery Children biliary atresia Kasai portoenterostomy BASM adjuvant therapy liver transplantation |
| title | Updates in Biliary Atresia: Aetiology, Diagnosis and Surgery |
| title_full | Updates in Biliary Atresia: Aetiology, Diagnosis and Surgery |
| title_fullStr | Updates in Biliary Atresia: Aetiology, Diagnosis and Surgery |
| title_full_unstemmed | Updates in Biliary Atresia: Aetiology, Diagnosis and Surgery |
| title_short | Updates in Biliary Atresia: Aetiology, Diagnosis and Surgery |
| title_sort | updates in biliary atresia aetiology diagnosis and surgery |
| topic | biliary atresia Kasai portoenterostomy BASM adjuvant therapy liver transplantation |
| url | https://www.mdpi.com/2227-9067/12/1/95 |
| work_keys_str_mv | AT markdavenport updatesinbiliaryatresiaaetiologydiagnosisandsurgery |