Presenting symptoms and time to diagnosis for Pediatric Central Nervous System Tumors in Qatar: a report from Pediatric Neuro-Oncology Service in Qatar.

INTRODUCTION: There are no previous published reports on primary pediatric tumors of the central nervous system (CNS) in Qatar. We undertook this retrospective cohort study to review the diagnosis of CNS tumors in children in Qatar to analyze the presentation characteristics including symptoms, referral pathways, and time to diagnosis. METHODS: All children registered with Pediatric Neuro-Oncology service (PNOS) were included in the study. Data from the time of diagnosis (October 2007 to February 2020) were reviewed retrospectively. Presenting symptoms were recorded and pre-diagnosis symptom interval (PSI) was calculated from the onset of the first symptom to the date of diagnostic imaging. RESULTS: Of the 61 children registered with PNOS during the study period, 51 were included in the final analysis. Ten children were excluded because they were either diagnosed outside Qatar (n = 7) or were asymptomatic at the time of diagnosis (n = 3). The median age was 45 (range 1-171) months. Common tumor types included low-grade glioma (LGG) (47.1%) and medulloblastoma/primitive neuro-ectodermal tumors (PNET) (23.5%). Nine children had an underlying neurocutaneous syndrome. Thirty-eight patients (74.5%) had at least one previous contact with healthcare (HC) professional, but 27 (52%) were still diagnosed through the emergency department (ED). Presenting symptoms included headache, vomiting (36.2%), oculo-visual symptoms (20.6%), motor weakness (18.9%), seizures, ataxia (17.2% each), irritability, cranial nerve palsies (12% each), and endocrine symptoms (10.3%). Median PSI was 28 days (range 1-845 days) for all CNS tumors. Longest PSI was seen with germ cell tumors (median 146 days), supratentorial location (39 days), and age above 3 years (30 days). Tumor characteristics of biological behavior (high-grade tumor) and location (infratentorial) were significantly associated with shorter PSI, as were presenting symptoms of ataxia, head tilt, and altered consciousness. CONCLUSIONS: Although overall diagnostic times were acceptable, some tumor types were diagnosed after a significant delay. The awareness campaign, such as the "HeadSmart" campaign in the United Kingdom (UK), can improve diagnostic times in Qatar. Further research is required to better understand the reasons for the delay.

Magnetic Resonance Cholangiopancreatography Severity Predicts Disease Outcomes in Pediatric Primary Sclerosing Cholangitis: A Reliability and Validity Study.

Magnetic resonance cholangiopancreatography (MRCP) has not been assessed as a surrogate biomarker in pediatrics. We aimed to determine the inter-rater reliability, prognostic utility, and construct validity of the modified Majoie endoscopic retrograde cholangiopancreatography classification applied to MRCP in a pediatric primary sclerosing cholangitis (PSC) cohort. This single-center, retrospective, cohort study included children with PSC undergoing diagnostic MRCP between 2008 and 2016. Six variations of the Majoie classification were examined: 1) intrahepatic duct (IHD) score, 2) extrahepatic duct (EHD) score (representing the worst intrahepatic and extrahepatic regions, respectively), 3) sum IHD-EHD score, 4) average IHD score, 5) average EHD score, and 6) sum average IHD-EHD score. Inter-rater reliability was assessed using weighted kappas and intraclass correlation coefficients (ICCs). Ability to predict time to PSC-related complications (ascites, esophageal varices, variceal bleed, liver transplant [LT], or cholangiocarcinoma) (primary outcome) and LT (secondary outcome) was assessed with Harrell's concordance statistic (c-statistic) and univariate/multivariable survival analysis. Construct validity was further assessed with Spearman correlations. Forty-five children were included (67% boys; median, 13.6 years). The inter-rater reliability of MRCP scores was substantial to excellent (kappas/ICCs, 0.78-0.82). The sum IHD-EHD score had the best predictive ability for time to PSC complication and LT (c-statistic, 0.80 and SE, 0.06; and c-statistic, 0.97 and SE, 0.01, respectively). Higher MRCP scores were independently associated with a higher rate of PSC-related complications, even after adjusting for the PSC Mayo risk score (hazard ratio, 1.74; 95% confidence interval, 1.14-2.). MRCP sum scores correlated significantly with METAVIR fibrosis stage, total bilirubin, and platelets (r = 0.42, r = 0.33, r = -0.31, respectively; P < 0.05). Conclusion: An MRCP score incorporating the worst affected intrahepatic and extrahepatic regions is reliable and predicts meaningful outcomes in pediatric PSC. Next steps include prospective validation and responsiveness assessment.

Factors determining renal impairment in unilateral ureteral colic secondary to calcular disease: a prospective study.

OBJECTIVE: To evaluate all possible risk factors that can cause impairment of overall renal function in patients with unilateral ureteral calculus and a normal contralateral kidney. METHODS: This is a prospective study of 90 patients who presented to our institute complaining of renal colic secondary to unilateral ureteral calculus. All patients were evaluated with a thorough history, physical examination, and laboratory and radiological investigations including renal function testing, urine analysis, non-contrast computed topography, and radionucleotide scan. Patients were divided into two groups according to their calculated creatinine clearance using the Modification of Diet in Renal Disease (MDRD) formula. Group I (favorable group) had a creatinine clearance >60 ml/min, while group II (unfavorable group) had a creatinine clearance <60 ml/min. RESULTS: The patients' mean age ± SD was 38.8 ± 11.4 years. Group I included 54 patients (60 %), while group II included 36 patients (40 %). On univariate analysis, factors that were associated with overall renal function impairment were patients' age, urea-to-creatinine ratio (UCR), use of nonsteroidal anti-inflammatory drugs, stone location, and presence of obstruction. However, using binary logistic regression analysis, only patients' age, UCR, and presence of obstruction sustained statistical significance in association with renal function impairment. CONCLUSION: The study of factors that help explain the presence of renal impairment in patients with unilateral ureteral calculus is important in the clinical setting. Patients' age, urea-to-creatinine ratio, and degree of obstruction seem to be significantly associated with overall renal function impairment.