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Outcomes of Biliary Atresia in a Single Center in Jeddah, Saudi Arabia

Background Biliary atresia (BA) is a rare but severe cause of obliterative cholangiopathy in neonates. Its incidence differs worldwide varying from 5/100,000 to 32/100,000 live births. The highest incidence is seen in Asia and the Pacific region. Diagnosing this disease is difficult in its early sta...

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Autores principales: Alaifan, Meshari A, Simbawa, Sara H, Fayoumi, Tala A, Bokhari, Hotoun F, Al-Ghamdi, Buthainah
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9468508/
https://www.ncbi.nlm.nih.gov/pubmed/36120286
http://dx.doi.org/10.7759/cureus.27871
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author Alaifan, Meshari A
Simbawa, Sara H
Fayoumi, Tala A
Bokhari, Hotoun F
Al-Ghamdi, Buthainah
author_facet Alaifan, Meshari A
Simbawa, Sara H
Fayoumi, Tala A
Bokhari, Hotoun F
Al-Ghamdi, Buthainah
author_sort Alaifan, Meshari A
collection PubMed
description Background Biliary atresia (BA) is a rare but severe cause of obliterative cholangiopathy in neonates. Its incidence differs worldwide varying from 5/100,000 to 32/100,000 live births. The highest incidence is seen in Asia and the Pacific region. Diagnosing this disease is difficult in its early stages; thus, screening is necessary to avoid serious complications that can be minimized with early intervention during the first few months of life. Currently, although there are no medical treatments for BA, once the diagnosis is confirmed, the Kasai procedure may be a treatment option. The earlier the Kasai surgery is performed, the higher the success rate. Liver transplantation may be needed if the operation fails. This study aimed to determine the incidence of BA and the factors influencing the outcomes of the Kasai procedure at King Abdulaziz University Hospital, Jeddah, Saudi Arabia. Methodology This retrospective cohort study was conducted in the Pediatric Department at King Abdulaziz University Hospital, Jeddah from January 2019 to July 2019 and included consecutive patients with BA from 2010 to 2018. Results In total, 14 patients (57.1% female) were included in the study. The median age at the time of presentation was 90 (19-720) days, and the median age at the time of implementing the Kasai procedure was 90 (60-150) days. Eight patients underwent the Kasai procedure, and only one patient had a liver transplant. Conclusions Antenatal screening for BA tended to ensure early diagnosis and better outcomes. Delay in diagnosis and intervention is associated with increased morbidity and mortality.
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spelling pubmed-94685082022-09-15 Outcomes of Biliary Atresia in a Single Center in Jeddah, Saudi Arabia Alaifan, Meshari A Simbawa, Sara H Fayoumi, Tala A Bokhari, Hotoun F Al-Ghamdi, Buthainah Cureus Pediatrics Background Biliary atresia (BA) is a rare but severe cause of obliterative cholangiopathy in neonates. Its incidence differs worldwide varying from 5/100,000 to 32/100,000 live births. The highest incidence is seen in Asia and the Pacific region. Diagnosing this disease is difficult in its early stages; thus, screening is necessary to avoid serious complications that can be minimized with early intervention during the first few months of life. Currently, although there are no medical treatments for BA, once the diagnosis is confirmed, the Kasai procedure may be a treatment option. The earlier the Kasai surgery is performed, the higher the success rate. Liver transplantation may be needed if the operation fails. This study aimed to determine the incidence of BA and the factors influencing the outcomes of the Kasai procedure at King Abdulaziz University Hospital, Jeddah, Saudi Arabia. Methodology This retrospective cohort study was conducted in the Pediatric Department at King Abdulaziz University Hospital, Jeddah from January 2019 to July 2019 and included consecutive patients with BA from 2010 to 2018. Results In total, 14 patients (57.1% female) were included in the study. The median age at the time of presentation was 90 (19-720) days, and the median age at the time of implementing the Kasai procedure was 90 (60-150) days. Eight patients underwent the Kasai procedure, and only one patient had a liver transplant. Conclusions Antenatal screening for BA tended to ensure early diagnosis and better outcomes. Delay in diagnosis and intervention is associated with increased morbidity and mortality. Cureus 2022-08-11 /pmc/articles/PMC9468508/ /pubmed/36120286 http://dx.doi.org/10.7759/cureus.27871 Text en Copyright © 2022, Alaifan et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Pediatrics
Alaifan, Meshari A
Simbawa, Sara H
Fayoumi, Tala A
Bokhari, Hotoun F
Al-Ghamdi, Buthainah
Outcomes of Biliary Atresia in a Single Center in Jeddah, Saudi Arabia
title Outcomes of Biliary Atresia in a Single Center in Jeddah, Saudi Arabia
title_full Outcomes of Biliary Atresia in a Single Center in Jeddah, Saudi Arabia
title_fullStr Outcomes of Biliary Atresia in a Single Center in Jeddah, Saudi Arabia
title_full_unstemmed Outcomes of Biliary Atresia in a Single Center in Jeddah, Saudi Arabia
title_short Outcomes of Biliary Atresia in a Single Center in Jeddah, Saudi Arabia
title_sort outcomes of biliary atresia in a single center in jeddah, saudi arabia
topic Pediatrics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9468508/
https://www.ncbi.nlm.nih.gov/pubmed/36120286
http://dx.doi.org/10.7759/cureus.27871
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