Insight into prevalence, etiology, and modalities of pediatric chronic dialysis: a comprehensive nationwide analysis.

BACKGROUND: This study aimed to determine the prevalence and etiology of kidney failure (KF) among children below 15 years of age receiving chronic dialysis in Saudi Arabia and describe their dialysis modalities. METHODS: This cross-sectional descriptive study was conducted on 8 August 2022, encompassing all 23 pediatric dialysis centers in Saudi Arabia. Data gathered comprised patient demographics, causes of KF, and the dialysis methods employed. Collected data underwent analysis to determine prevalence of children undergoing chronic dialysis, discern underlying causes of KF, and evaluate distribution of patients across different dialysis modalities. RESULTS: The prevalence of children on chronic dialysis is 77.6 per million children living in Saudi Arabia, equating to 419 children. The predominant underlying cause of KF was congenital anomalies of the kidneys and urinary tract (CAKUT), representing a substantial 41% of cases. Following this, others or unknown etiologies accounted for a noteworthy 25% of cases, with focal segmental glomerulosclerosis (FSGS) comprising 13%, glomerulonephritis at 11%, and congenital nephrotic syndrome contributing 10% to etiological distribution. Regarding dialysis modalities employed, 67% of patients were on peritoneal dialysis (PD), while the remaining 33% were on hemodialysis (HD). CONCLUSIONS: This first nationwide study of pediatric chronic dialysis in Saudi Arabia sheds light on the prevalence of children undergoing chronic dialysis and underlying causes of their KF, thereby contributing to our understanding of clinical management considerations. This research serves as a stepping stone for the development of national registries.

A Multicenter Study Evaluating the Discontinuation of Eculizumab Therapy in Children with Atypical Hemolytic Uremic Syndrome.

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening thrombotic microangiopathy (TMA), which has been treated successfully with eculizumab. The optimal duration of eculizumab in treating patients with aHUS remains poorly defined. METHODS: We conducted a multicenter retrospective study in the Arabian Gulf region for children of less than 18 years of age who were diagnosed with aHUS and who discontinued eculizumab between June 2013 and June 2021 to assess the rate and risk factors of aHUS recurrence. RESULTS: We analyzed 28 patients with a clinical diagnosis of aHUS who had discontinued eculizumab. The most common reason for the discontinuation of eculizumab was renal and hematological remission (71.4%), followed by negative genetic testing (28.6%). During a median follow-up period of 24 months after discontinuation, 8 patients (28.5%) experienced HUS relapse. The risk factors of recurrence were positive genetic mutations (p = 0.020). On the other hand, there was no significant relationship between the relapse and age of presentation, the need for acute dialysis, the duration of eculizumab therapy before discontinuation, or the timing of eculizumab after the presentation. Regarding the renal outcomes after discontinuation, 23 patients were in remission with normal renal function, while 4 patients had chronic kidney disease (CKD) (three of them had pre-existing chronic kidney disease (CKD) before discontinuation, and one case developed a new CKD after discontinuation) and one patient underwent transplantation. CONCLUSIONS: The discontinuation of eculizumab in patients with aHUS is not without risk; it can result in HUS recurrence. Eculizumab discontinuation can be performed with close monitoring of the patients. It is essential to assess risk the factors for relapse before eculizumab discontinuation, in particular in children with a positive complement variant and any degree of residual CKD, as HUS relapse may lead to additional loss of kidney function. Resuming eculizumab promptly after relapse is effective in most patients.

AGREEing on clinical practice guidelines for idiopathic steroid-sensitive nephrotic syndrome in children.

BACKGROUND: Nephrotic syndrome is the most common kidney disease in children worldwide. Our aim was to critically appraise the quality of recent Clinical Practice Guidelines (CPGs) for idiopathic steroid-sensitive nephrotic syndrome (SSNS) in children in addition to summarize and compare their recommendations. METHODS: Systematic review of CPGs. We identified clinical questions and eligibility criteria and searched and screened for CPGs using bibliographic and CPG databases. Each included CPG was assessed by four independent appraisers using the Appraisal of Guidelines for REsearch & Evaluation II (AGREE-II) instrument. We summarized the recommendations in a comparison practical table. RESULTS: Our search retrieved 282 citations, of which three CPGs were eligible and appraised: Kidney Disease: Improving Global Outcomes (KDIGO) 2012, Japan Society for Pediatric Nephrology (JSPN) 2014, and American Academy of Pediatrics (AAP) 2009. Among these, the overall assessment of two evidence-based CPGs scored > 70% (KDIGO and JSPN), which was consistent with their higher scores in the six domains of the AGREE II Instrument. In domain 3 (rigor of development), KDIGO, JSPN, and AAP scored 84%, 74%, and 41%, respectively. In domain 5 (applicability), they scored 22%, 16%, and 19%, respectively, and in domain 6 (editorial independence), they scored 94%, 65%, and 88%, respectively. CONCLUSIONS: The methodological quality of the KDIGO CPG was superior, followed by JSPN and AAP CPGs with the relevant recommendations for use in practice. SYSTEMATIC REVIEW REGISTRATION: The protocol was registered in the Center for Open Science (OSF) DOI: 10.17605/OSF.IO/6QTMD and in the International prospective register of systematic reviews PROSPERO 2020 CRD42020197511 .

Aminophylline for improving acute kidney injury in pediatric patients: A systematic review and meta-analysis.

OBJECTIVES: Acute kidney injury (AKI) is a major cause of morbidity and mortality. Whether aminophylline administration can prevent or treat AKI among pediatric patients are not clear. This meta-analysis aimed to assess the efficacy and effectiveness of aminophylline for pediatric AKI. METHODS: We carried out a systematic search of six databases: PubMed, EMBASE/Excerpta Medica, Scopus, Cochrane library, and Google Scholar from January 1995 up till May 2019. Summary measures of risk ratios and standard mean difference were calculated using the random effects model. RESULTS: We identified seven papers containing data on aminophylline use in children with AKI. Meta-analysis of single-arm studies indicated no statistically significant difference in mean rate of serum creatinine clearance (-0.39 [-0.80-1.58], P = 0.52), mean urine output (1.99 [-1.43-5.42]; P = 0.25), or mean blood urea nitrogen levels (0.83 [-1.86-3.03], P = 0.54) before and after aminophylline administration. However, among double-arm studies, aminophylline administration in the intervention arm significantly reduced the serum creatinine level as compared to control arm (mean diff = -34 [-55.18--12.83]; P = 0.002). Mean urine output (-112.68 [-27.43-48.9], P = 0.17), incidence of AKI (RR = 1.05 [0.80-1.37], P = 0.72), and mortality rates (RR = 0.79 [0.42-1.47], P = 0.45) were found to be statistically insignificant. CONCLUSIONS: Aminophylline administration in children with AKI reduces serum creatinine level without significant adverse effects or effect on the incidence of AKI, urine output, or mortality. Further, large-scale well-planned randomized controlled trials are needed to evaluate its use and its potential long-term effects.

Barriers Facing Primary Health Care Physicians When Dealing with Emergency Cases in Jeddah, Saudi Arabia.

OBJECTIVES: To estimate the prevalence of emergency cases reporting to Primary Health Care centers (PHC), Jeddah, Saudi Arabia and to explore the barriers facing PHC physicians when dealing with such emergency cases. METHODS: A cross-sectional analytic study, where all physicians working in the PHC of the Ministry of Health (MOH) in Jeddah; were invited to participate (n=247). The study period was from July 2013 till December 2013. Data were collected through two sources. 1- A self-administered questionnaire used to determine the physicians' perceived competence when dealing with emergency cases. 2- A structured observation sheet used to evaluate availability of equipment, drugs, ambulances and other supporting facilities required to deal with emergency cases. RESULTS: The response rate was 83.4%. The physicians' age ranged between 25 and 60 years with a mean ±SD of 34.4±7.5 years. Majority of them (83.5%) did not attend ATLS courses at all whereas 60.7% never attended ACLS courses. The majority (97.1%) had however attended BLS courses. Physicians in the age group 36-45 years, non-Saudi, those who had SBFM, those who reported experience in working in emergency departments and physicians who reported more working years in PHCCs (>5 years) had a significant higher score of perceived level of competence in performing emergency skill scale than others (P<0.05). The prevalence of emergency cases attending PHC in Jeddah (2013) was 5.2%. CONCLUSION: Emergency services at PHC in Jeddah are functioning reasonably well, but require fine tuning of services and an upgrade in their quality.