Outcome of biliary atresia among Saudi children: A tertiary care center experience
Background/Aims: Data from Western countries indicate that biliary atresia (BA) is the leading cause of end-stage liver disease in children and the most common indication for liver transplantation (LT) in the pediatric population. There is no data on the epidemiology and outcome of BA in Saudi Arabi...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2019-01-01
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| Series: | The Saudi Journal of Gastroenterology |
| Subjects: | |
| Online Access: | http://www.saudijgastro.com/article.asp?issn=1319-3767;year=2019;volume=25;issue=3;spage=176;epage=180;aulast=Holdar |
| _version_ | 1828394746803912704 |
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| author | Sinan Holdar Badr Alsaleem Ali Asery Abdulrahman Al-Hussaini |
| author_facet | Sinan Holdar Badr Alsaleem Ali Asery Abdulrahman Al-Hussaini |
| author_sort | Sinan Holdar |
| collection | DOAJ |
| description | Background/Aims: Data from Western countries indicate that biliary atresia (BA) is the leading cause of end-stage liver disease in children and the most common indication for liver transplantation (LT) in the pediatric population. There is no data on the epidemiology and outcome of BA in Saudi Arabia. The main objective of our study was to understand the clinical and epidemiological characteristics and outcome of BA in the Saudi population.
Patients and Methods: We retrospectively reviewed the database of infantile cholestasis cases that presented to our center from 2008 to 2015 and identified BA cases. Data on clinical, biochemical, imaging, and histopathological characteristics were collected by chart review. The two primary study outcomes were (1) successful Kasai portoenterostomy (KPE) defined as resolution of jaundice (total serum bilirubin <20 μmol/L) and (2) survival with native liver.
Results: Over the study period, we evaluated 450 cases of infantile cholestasis. In all, 21 cases (11 males) were diagnosed with BA (4.7%). BA cases were first seen by pediatric gastroenterologists at a median age of 65 days. KPE was performed in 12 cases at a median age of 73 days. Successful KPE was achieved in four cases (33%). Five of the remaining eight cases had LT and three died before LT. Nine of the 21 BA cases were denied KPE and had primary LT at median 8 months of age. The native liver 4-year survival rate was 14.3% and the overall survival rate was 81%.
Conclusion: BA is an uncommon cause of infantile cholestasis in Saudi Arabia. Our study provides a snapshot of the epidemiology of BA in Saudi Arabia that is characterized by late referral to pediatric gastroenterologists and poor outcome without LT. |
| first_indexed | 2024-12-10T07:59:45Z |
| format | Article |
| id | doaj.art-992972ab01a443148163c2b95f406ce4 |
| institution | Directory Open Access Journal |
| issn | 1319-3767 1998-4049 |
| language | English |
| last_indexed | 2024-12-10T07:59:45Z |
| publishDate | 2019-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | The Saudi Journal of Gastroenterology |
| spelling | doaj.art-992972ab01a443148163c2b95f406ce42022-12-22T01:56:48ZengWolters Kluwer Medknow PublicationsThe Saudi Journal of Gastroenterology1319-37671998-40492019-01-0125317618010.4103/sjg.SJG_306_18Outcome of biliary atresia among Saudi children: A tertiary care center experienceSinan HoldarBadr AlsaleemAli AseryAbdulrahman Al-HussainiBackground/Aims: Data from Western countries indicate that biliary atresia (BA) is the leading cause of end-stage liver disease in children and the most common indication for liver transplantation (LT) in the pediatric population. There is no data on the epidemiology and outcome of BA in Saudi Arabia. The main objective of our study was to understand the clinical and epidemiological characteristics and outcome of BA in the Saudi population. Patients and Methods: We retrospectively reviewed the database of infantile cholestasis cases that presented to our center from 2008 to 2015 and identified BA cases. Data on clinical, biochemical, imaging, and histopathological characteristics were collected by chart review. The two primary study outcomes were (1) successful Kasai portoenterostomy (KPE) defined as resolution of jaundice (total serum bilirubin <20 μmol/L) and (2) survival with native liver. Results: Over the study period, we evaluated 450 cases of infantile cholestasis. In all, 21 cases (11 males) were diagnosed with BA (4.7%). BA cases were first seen by pediatric gastroenterologists at a median age of 65 days. KPE was performed in 12 cases at a median age of 73 days. Successful KPE was achieved in four cases (33%). Five of the remaining eight cases had LT and three died before LT. Nine of the 21 BA cases were denied KPE and had primary LT at median 8 months of age. The native liver 4-year survival rate was 14.3% and the overall survival rate was 81%. Conclusion: BA is an uncommon cause of infantile cholestasis in Saudi Arabia. Our study provides a snapshot of the epidemiology of BA in Saudi Arabia that is characterized by late referral to pediatric gastroenterologists and poor outcome without LT.http://www.saudijgastro.com/article.asp?issn=1319-3767;year=2019;volume=25;issue=3;spage=176;epage=180;aulast=HoldarBiliary atresiacholestasisKasailate referralliver transplantoutcomeportoenterostomy |
| spellingShingle | Sinan Holdar Badr Alsaleem Ali Asery Abdulrahman Al-Hussaini Outcome of biliary atresia among Saudi children: A tertiary care center experience The Saudi Journal of Gastroenterology Biliary atresia cholestasis Kasai late referral liver transplant outcome portoenterostomy |
| title | Outcome of biliary atresia among Saudi children: A tertiary care center experience |
| title_full | Outcome of biliary atresia among Saudi children: A tertiary care center experience |
| title_fullStr | Outcome of biliary atresia among Saudi children: A tertiary care center experience |
| title_full_unstemmed | Outcome of biliary atresia among Saudi children: A tertiary care center experience |
| title_short | Outcome of biliary atresia among Saudi children: A tertiary care center experience |
| title_sort | outcome of biliary atresia among saudi children a tertiary care center experience |
| topic | Biliary atresia cholestasis Kasai late referral liver transplant outcome portoenterostomy |
| url | http://www.saudijgastro.com/article.asp?issn=1319-3767;year=2019;volume=25;issue=3;spage=176;epage=180;aulast=Holdar |
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