Predictors of biliary atresia outcome: Saudi National Study (2000 - 2018).

Background: Outcomes in biliary atresia (BA) have been well-documented in large national cohorts from Europe, North America, and East Asia. Understanding the challenges that preclude success of the Kasai portoenterostomy (KPE) is the key to improve the overall outcomes of BA and implementing intervention strategies. Here, we analyzed the data from the Saudi national BA study (204 BA cases diagnosed between 2000 and 2018) to identify the prognostic factors of BA outcomes. Methods: One hundred and forty-three cases underwent KPE. Several prognostic factors (center case load, congenital anomalies, serum gamma-glutamyl transferase, use of steroids, ascending cholangitis post-operatively, and degree of portal fibrosis at time of KPE) were investigated and correlated with the primary outcomes of interest: 1) success of KPE (clearance of jaundice and total serum bilirubin <20 mmol/l after KPE), 2) survival with native liver (SNL), and 3) overall survival. Results: Use of steroids after KPE was associated with clearance of jaundice, 68% vs. 36.8% in the BA cases that did not receive steroids (P = 0.013; odds ratio 2.5) and a significantly better SNL rate at 2 - and 10-year of 62.22% and 57.77% vs. 39.47% and 31.57%, respectively (P = 0.01). A better 10-year SNL was observed in centers with caseload <1/year (group 1) as compared to centers that performed ≥1/year (group 2) [45.34% vs. 26.66%, respectively; P = 0.047]. On comparison of the 2 groups, cases in group 1 had KPE at significantly earlier age (median 59.5 vs. 75 days, P = 0.006) and received steroids after KPE more frequently than group 2 (69% vs. 31%, P < 0.001). None of the remaining prognostic variables were identified as being significantly related to BA outcome. Conclusion: Steroids use post-KPE predicted clearance of jaundice and better short- and long-term SNL. There is a need to establish a national BA registry in Saudi Arabia aiming to standardize the pre- and post-operative clinical practices and facilitate clinical and basic research to evaluate factors that influence BA outcome.

The Epidemiology and Outcome of Biliary Atresia: Saudi Arabian National Study (2000-2018).

Background: The epidemiology and outcomes of biliary atresia (BA) have been well-documented in national cohorts from two main ethnicities, namely, the Asian Orientals and Caucasians, with incidence ranging from 1 in 5,000 to 1 in 9,000 live births in East Asia and 1 in 15,000 to 19,000 live births in Europe and North America. Objective: We report the first nationwide BA study outside North America, Europe, and East Asia to describe the epidemiology and outcomes of BA in Saudi Arabia. Methods: A national database of BA cases diagnosed between 2000 and 2018 was analyzed. We assessed clearance of jaundice (bilirubin <20 µmol/L) in all cases that underwent Kasai portoenterostomy (KPE). We then estimated survival using the Kaplan-Meier method with endpoints of liver transplantation (LT), death, or survival with native liver (SNL). Results: BA was diagnosed in 204 infants (106 females; 10% pre-term). The incidence of BA was 1 in 44,365, or 2.254 in 100,000 live births (range, 0.5-4 in 100,000). Polysplenia was diagnosed in 22 cases (11%). The median age at referral was 65 days. A total of 146 children (71.5%) underwent KPE at a median age of 70 days. Clearance of jaundice was achieved in 66 of the 146 (45%) infants. The 10-year SNL after KPE was 25.5%, and the overall 10-year estimated survival was 72.5%. The Kaplan-Meier survival curves for patients undergoing KPE at the age of <60, 61-90, and >90 days showed a SNL rate at 51.6, 33, and 12.5%, respectively, at 5 years (P < 0.001). The 2-, 5-, and 10-year post-LT survival rates were 92.5, 90.6, and 90%, respectively. Undergoing an initial KPE did not impact negatively on the overall LT survival rate when compared to BA cases that underwent primary LT (P = 0.88). Conclusion: The incidence rate of BA in Saudi Arabia is lower than the incidence reported elsewhere. Late referral of BA cases remains a problem in Saudi Arabia; as a result, the SNL rate was lower than reported by other national registries. Hence, national policies devoted to timely referral and earlier age at KPE are needed.

Serum pancreatic enzymes and imaging in paediatric acute pancreatitis: Does lipase diagnostic superiority justify eliminating amylase testing?

BACKGROUND: In acute pancreatitis (AP), serum amylase, lipase and imaging help establish a diagnosis with recognised lipase superiority. Recent literature has debated serum amylase testing and proposed its elimination, but little is known about the diagnostic role of simultaneously measured serum amylase levels in patients with non-diagnostic lipase. This study examined the contribution of pancreatic enzymes and imaging and the role of simultaneously measured serum amylase in children with non-diagnostic serum lipase. METHODS: Retrospective medical records review of children aged <18 years with a verified discharge diagnosis of first-attack AP between January 01, 1994, and December 31, 2016. RESULTS: First-attack AP was confirmed in 127 children (median age, 12.5 years). The sensitivity was 90.4%, 54.3%, 42.2% and 36.4% for lipase, amylase, contrast-enhanced computed tomography and ultrasonography (US), respectively. Combination US and lipase identified 96.6% of AP cases. Simultaneous amylase and lipase measurements in 125 children showed that either was ≥3× the upper limit of normal (ULN) in 95.2%, while both were <3× the ULN in 4.8% of cases. Nondiagnostic lipase was seen in 12 (9.6%) children, and diagnosis was based on amylase level ≥3× the ULN in six children and imaging in the other six. CONCLUSIONS: Serum amylase, serum lipase and imaging should continue for the conclusive diagnosis of AP in children. Simultaneous serum amylase measurement helped diagnose AP with non-diagnostic lipase.

Frequency of bacteremia in patients with sickle cell disease: a longitudinal study.

Bacterial infections in sickle cell disease (SCD) are associated with major risks of morbidity and mortality. Here, we describe the occurrence of bacteremia in SCD patients from 2000 to 2017. This is an observational study which included children and adults with SCD and fever and had confirmed positive blood cultures. During the study period, there were 1095 SCD patients with 17,053 blood cultures. Of all the patients, 699 (63.8%) were children and 396 (36.2%) adults with 576 (52.6%) males and 519 (47.4%) females. The mean age ± SD was 17.8 (± 14.7), and a median age (IQR) of 13.6 (6.8-23.5) years. The mean (SD) follow up was 7.4 (5.4) years and the total number of patient-years was 8069.1 years. Out of the 1095 patients, 91 (8.3%) had bacteremia with 35 (38.5%) children and 65 (61.5%) adults (p = .079). The rate of bacteremia in all patients, children, and adults were 1.5 (95% CI: 1.3-1.8), 0.6 (95% CI: 0.4-0.8), and 2.4 (95% CI: 1.8-3.1) per 100 patient-years, respectively. The risk of Gram-positive bacteremia was 0.5 (96% CI: 0.36-0.69) in all patients, 0.1 (95% CI: 0.06-0.20) in children, and 1.4 (95% CI: 1.0-2.0) in adults per 100 patient-years. The risk of Gram-negative bacteremia was 1.0 (95% CI: 0.81-1.3) in all patients, 0.6 (95% CI: 0.4-0.8) in children, and 2 (95% CI: 1.5-2.7) in adults per 100 patient-years. The risk of Gram-negative bacteremia was higher than Gram-positive bacteremia in children (p < .001) but not in adults (p = .113) and adults had higher risk in general than children. In this study of SCD cohort, 8.3% had bacteremia with predominant Gram-negative infections. Bacteremia was more frequently encountered in the adult age group. Further studies are needed to verify the findings and explore possible reasons predisposing SCD patients to bacteremia.

Regional variation of pediatric inflammatory bowel disease in Saudi Arabia: Results from a multicenter study.

BACKGROUND: Incidence and severity variations of inflammatory bowel disease (IBD) have been reported from Western populations between continents and regions of the same countries. However, no data were available from other countries. AIM: To investigate the regional differences in the IBD profiles of pediatric patients from the Kingdom of Saudi Arabia. METHODS: Data from a national multicenter IBD study were used. The incidence, time trend, and clinical presentation of Crohn's disease (CD) and ulcerative colitis (UC) in the Central region (CR), Western region (WR), and Eastern region (ER) were analyzed and compared. Statistical analysis included Poisson regression analysis for incidence variation and Chi-square test for demographic and clinical parameters. A P < 0.05 was considered significant. RESULTS: The prevalence of positive family history was lower in children with CD from the ER than the CR or the WR. Consanguinity rate was higher in children with CD and UC from the CR and the ER, respectively. The incidences and time trends of CD and UC were not significantly different between regions. In the ER, a significantly higher percentage of children with CD presented with abdominal pain (P < 0.001), blood in stools (P = 0.048), stricturing or penetrating disease (P = 0.029), higher erythrocyte sedimentation rate (P < 0.001), higher C-reactive protein (P < 0.001), higher anemia (P = 0.017), and lower albumin level (P = 0.014). For children with UC from the ER, a significantly higher percentage presented with anemia (P = 0.006) and a lower percentage with pancolitis (P < 0.001). CONCLUSION: The most important finding is the identification of significantly more severe presentation of CD in the ER of the Kingdom of Saudi Arabia. Prospective studies are needed to explain such variations.