Outcome of biliary atresia among Saudi children: A tertiary care center experience

Holdar, Sinan; Alsaleem, Badr; Asery, Ali; Al–Hussaini, Abdulrahman

doi:10.4103/sjg.sjg_306_18

Cited by 10 publications

(10 citation statements)

References 15 publications

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“…This late timing of the LB is a consequence of late referral to our center at a median age of 65 days [13]. As a result, the outcome of BA cases in our center was poor with a success rate of KPE at 33% and the shortterm survival rate without LT at 14.3% [13], as compared to data from Western countries and Japan which showed a success rate of KPE at 60 to 80%, and 4-year native liver survival of 40 to 60% [15][16][17][18]. Many of the liver diseases in infants manifest cholestasis with significant overlap clinically and biochemically which makes early diagnosis of BA a clinical challenge.…”

Section: Discussionmentioning

confidence: 99%

“…The commonest cause of IC worldwide is BA, ranging between 20 to 40% [14]. In contrast, BA is an uncommon cause of IC in Saudi Arabia (5%) [13]. Our study cohort represents a LB-driven differential diagnosis of IC in our community as it is composed of a highly selective group of infants who underwent LB due to high suspicion of BA or uncertainty of diagnosis following unrevealing investigations.…”

Section: Discussionmentioning

confidence: 99%

“…Previous studies reported that LB had an accuracy ranging between 60 and 95% in diagnosing BA [8][9][10][11][12]. BA, a sporadic condition, is an uncommon cause of IC in Saudi Arabia (4.7%) [13], as compared to 20-40% of IC cases in many countries around the world [14]. Data from other countries may be relevant to Arabs, however data cannot be directly extrapolated.…”

Section: Introductionmentioning

confidence: 99%

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Role of percutaneous liver biopsy in infantile cholestasis: cohort from Arabs

Ahmed¹,

Fagih

Bashir

et al. 2021

BMC Gastroenterol

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Background Investigators from different parts of the world are calling for a re-evaluation of the role of liver biopsy (LB) in the evaluation of infantile cholestasis (IC), especially in the light of emerging non-invasive diagnostic technologies. Therefore, this retrospective single-center study was conducted to determine the impact of LB on the diagnosis and management of IC in a cohort from Arabs. Methods From 2007 until 2019, 533 cases of IC were referred for evaluation. All infants who underwent LB were included in the study. We categorized the yield of LB into: (1) defined specific diagnosis; (2) excluded an important diagnosis. A single pathologist reviewed and made the histology report. Results 122 LB specimens met the inclusion criteria. The main indication for LB was a high suspicion of biliary atresia (BA) [high gamma-glutamyl transferase (GGT) cholestasis and pale stool] in 46 cases (37.8%). Liver biopsy had sensitivity of 86.4%, specificity (66.7%), PPV (70.4%), NPV (84.2%) in diagnosing BA. LB had a direct impact on clinical management in 52 cases (42.6%): (1) The true diagnosis was suggested by LB in 36 cases; (2) LB excluded BA and avoided intraoperative cholangiogram in 16 cases with high suspicion of BA. Among the 76 cases with low suspicion of BA, LB suggested the true diagnosis or helped to initiate specific management in 8 cases only (10.5%). In contrast, molecular testing confirmed the diagnosis in 48 (63%). Conclusion LB continues to be an important tool in the workup of cases with a high suspicion of BA. The low yield of LB in cases with low suspicion of BA calls for a re-evaluation of its role in these cases in whom early incorporation of cholestasis sequencing gene panels can have a better diagnostic yield.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Role of percutaneous liver biopsy in infantile cholestasis: cohort from Arabs

Ahmed¹,

Fagih

Bashir

et al. 2021

BMC Gastroenterol

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“…При анализе данных литературы, посвященной детской онкологии, не встречаются источники, упоминающие ППН, а также методы оценки функционального резерва будущего остатка печени у детей [16]. Сцинтиграфию печени применяют в педиатрии со второй половины прошлого столетия по ограниченным показаниям, как правило, связанным с врожденными или приобретенными болезнями желчевыводящих путей [33][34][35][36], тогда как при планировании объема и метода оперативного этапа лечения новообразований печени у детей основным исследованием, направленным на предупреждение ППН, остается КТ-волюметрия, а пороговое значение FLR-V экстраполировано из взрослой практики и составляет 25% и более объема здоровой паренхимы печени [13,15,19,20,37].…”

Section: обсуждение результатов исследованияunclassified

Extensive liver resection in a child with an extremely low future liver remnant volume

Ахаладзе

Rabaev

Меркулов

et al. 2021

Voprosy gematologii/onkologii i immunopatologii v pediatrii

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The incidence of posthepatectomy liver failure in adult patients and a large number of complications of two-stage liver resections require a search for criteria that allow highly accurate assessment of the risk of liver failure. For this purpose, the study of the future liver remnant volume and function have been widely introduced among adult patients, and the future liver remnant function measurement reflects the greater sensitivity. The absence of references to posthepatectomy liver failure, as well as the experience of determining the functional reserve of the future liver remnant in children, let us to suggest the possibility of a wider using one-stage liver resections when the future liver remnant volume is below the generally accepted threshold (25% of the healthy liver parenchyma volume) in the case of the functional reserve sufficient value. This clinical case describes the successful extended right hemihepatectomy and segmentectomy 1 in a 3-year patient with a future liver remnant volume of 16.5% without clinical signs of postresection hepatic failure, which confirms the thesis of the need to assess the functional liver reserve in pediatric oncology to reduce the frequency of two-stage resections and liver transplants. The patient’s parents gave consent to the use of their child’s data, including photographs, for research purposes and in publications.

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“…11 Successful Kasai portoenterostomy is normalization of bilirubin within first 6 months. 12 Without KPE and liver transplant 50%-80% will die within 1 year of age. KPE allows 14%-44% of BA patients to escape liver transplant until adulthood.…”

mentioning

confidence: 99%

Biliary atresia—An experience from the first pediatric liver transplant center in Pakistan

Khan

Ali

Dar

et al. 2022

Pediatric Transplantation

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Background: Biliary atresia (BA) is the most common cause of neonatal cholestatic syndrome. The true incidence of BA in Pakistan is largely unknown.Aim: This study aimed to report the clinical features, age at diagnosis and outcomes of biliary atresia from the first pediatric liver transplant center in Pakistan. Methods:The study was done in Shifa International hospital from 2013 to 2020. All babies who had biliary atresia confirmed by laboratory investigation were included.Demographic data, age of presentation, clinical presentation, supporting investigations like liver function tests, ultrasound abdomen, HIDA scan and liver biopsy were noted. Outcome related to Kasai portoenterostomy, liver transplant, complications and immunosuppressant agents were noted.Result: A total of 42 children were included, 23 (54.7%) males and 19 (45.2%) were females. Jaundice was seen in all patients (100%) followed by acholic stools (81%). Associated malformations were noted in 6 (14.2%) patients. Liver function tests confirmed obstructive cholestasis (p 0.04). Kasai was done in 19 (45%) patients only, living donor liver transplant was performed in 6 (14%) patients. Age range of transplant patients was from 3 months to 1 year. Indication for liver transplant was failed Kasai in 1(16.7%) patient and chronic liver disease in 5 (83.3%) patients. LDLT survivors were 10 months to 1 year of age at the time of transplant, mean age was 10.6 months.Maximum survival noted so far is 7 years. Acute complications seen post-transplant were sepsis (three patients), surgical site infections (two patients), biliary leaks and acute cellular rejection in one patient each. Chronic graft rejection, portal vein stricture needing stenting was done in one patient.Discussion: All patients underwent LDLT from related donors wih no donor related mortality. All are deceased patients were yonger and had advanced disease. BA remains third most commo indication of transplant in our center. Conclusion:Liver transplant is the only lifesaving procedure after failed Kasai or as primary liver transplant due to advance liver disease. The advent of liver transplantation services offers survival and improving outlook of the disease.

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Outcome of biliary atresia among Saudi children: A tertiary care center experience

Cited by 10 publications

References 15 publications

Role of percutaneous liver biopsy in infantile cholestasis: cohort from Arabs

Role of percutaneous liver biopsy in infantile cholestasis: cohort from Arabs

Extensive liver resection in a child with an extremely low future liver remnant volume

Biliary atresia—An experience from the first pediatric liver transplant center in Pakistan

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