IPNA clinical practice recommendations on care of pediatric patients with pre-existing kidney disease during seasonal outbreak of COVID-19.

The coronavirus disease 2019 (COVID-19) pandemic, instigated by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has profoundly impacted healthcare infrastructures around the globe. While children are usually asymptomatic or have mild symptoms, children with pre-existing kidney conditions require specialized attention. This pivotal report, championed by the International Pediatric Nephrology Association (IPNA), delivers precise and actionable recommendations tailored for pediatric patients with kidney ailments in this pandemic landscape.

Sepsis-Associated Acute Kidney Injury in Critically Ill Children: Incidence and Outcomes.

Acute kidney injury (AKI) is a major concern in pediatric critical care, often occurring in conjunction with sepsis. This study aimed to identify the incidence, outcomes, and risk factors for AKI in the context of pediatric sepsis. This was a bicentric retrospective cohort study conducted at two university hospitals in Saudi Arabia.

Adapting Clinical Practice Guidelines for Chronic Kidney Disease: Blood Pressure Management and Kidney Replacement Therapy in Adults and Children in the Saudi Arabian Context Using the Grading of Recommendations Assessment, Development, and Evaluation-ADOLOPMENT Methodology.

This practice guideline was developed by the chronic kidney disease (CKD) Task Force, which was composed of clinical and methodological experts. The Saudi Arabian Ministry of Health and its health holding company commissioned this guideline project to support the realization of Vision 2030's health-care transformation pillar. The synthesis of these guidelines was guided by the Grading of Recommendations Assessment, Development, and Evaluation (GRADE)- ADOLOPMENT methodology.

Point-of-Care Ultrasound (POCUS) Training Curriculum for Pediatric Nephrology: PCRRT-ICONIC Group Recommendations.

Key Points: Despite a high need and interest in point-of-care ultrasound (POCUS) in trainees and faculty of Pediatric Nephrology, the majority are not receiving POCUS training. There is a need to establish a structured pediatric nephrology POCUS program for clinicians and fellows. This study suggests a blueprint of POCUS curriculum which can serve as a foundation for POCUS education in pediatric nephrology centers worldwide.

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.

Clinical and molecular characterization of a large primary hyperoxaluria cohort from Saudi Arabia: a retrospective study.

Background: Primary hyperoxalurias (PHs) constitute rare disorders resulting in abnormal glyoxalate metabolism. PH-associated phenotypes range from progressive nephrocalcinosis and/or recurrent urolithiasis to early kidney failure.

Methods: A retrospective study was conducted for patients with confirmed PH diagnoses from three tertiary centers in Saudi Arabia.

Hypermanganesemia with Dystonia Type 2: A Potentially Treatable Neurodegenerative Disorder: A Case Series in a Tertiary University Hospital.

Importance: Hypermanganesemia with dystonia type 2 is a rare autosomal recessive neurodegenerative disorder characterized by the loss of previously acquired milestones, dystonia, parkinsonian features, a high serum manganese level, and characteristic neuroimaging findings such as bilateral and symmetrically increased T1 and decreased T2/fluid-attenuated inversion recovery signal intensity in the basal ganglia. This condition is secondary to a mutation in the gene.

Objective: To present a series of three cases of hypermanganesemia with dystonia type 2, which was genetically confirmed secondary to a mutation in the gene, and to describe the treatment and clinical course in these cases.

Cinacalcet for Severe Secondary Hyperparathyroidism in Children with End-stage Kidney Disease.

Advanced chronic kidney disease with mineral and bone disorder have a significant obstacles to control serum bone profile [serum intact parathyroid hormone (iPTH), calcium and phosphorus] which subsequently have major effect on optimal bone strength, final adult height, and cardiovascular health. A retrospective, observational study, including a total of 36 children with end-stage kidney disease (ESKD). Fourteen children who were prescribed cinacalcet had been compared with the remaining 22 children who were managed with standard care.

The outcome of rituximab in treating steroid dependent nephrotic syndrome.: Histopathology and immunosuppressive drugs as predicting factors.

Objectives: To present our experience of treating steroid-dependent nephrotic syndrome (SDNS) in children with repeated doses of rituximab (RTX) with a relatively long follow-up, and to discuss the role of the histopathology type and previous immune-suppressor (IS) drugs on the outcome of these patients.

Methods: The patients included in this prospective study were children with SDNS who were in remission on a high-dose steroid or with additional IS drugs. All patients underwent renal biopsy before RTX treatment.

Comparison of Renal Stones and Nephrocalcinosis in Children: Findings From Two Tertiary Centers in Saudi Arabia.

Renal stones (nephrolithiasis and urolithiasis) and nephrocalcinosis are uncommon in children; however, their incidences in pediatric populations have been increasing. This multicenter retrospective study compared the clinical presentation, etiology, and outcomes of childhood nephrolithiasis or urolithiasis with those of nephrocalcinosis. The study included 144 children: 93 with renal stones and 51 with nephrocalcinosis.

Childhood nephrolithiasis and nephrocalcinosis caused by metabolic diseases and renal tubulopathy: A retrospective study from 2 tertiary centers.

Objectives: To study childhood nephrolithiasis and nephrocalcinosis caused by metabolic disorders, distal renal tubular acidosis (dRTA), and familial hypomagnesemia, hypercalciuria, and nephrocalcinosis (FHHNC).

Methods: We retrospectively evaluated 86 children presented over 10 years (2011-2021), with nephrolithiasis (89%) and nephrocalcinosis (11%) caused by metabolic disorders (62%), FHHNC (21%), and dRTA (17%).

Results: The mean age at discovery was 72.

Factors That Influence Mortality in Critically Ill Patients with SARS-CoV-2 Infection: A Multicenter Study in the Kingdom of Saudi Arabia.

Background: SARS-CoV-2 infection has a high mortality rate and continues to be a global threat, which warrants the identification of all mortality risk factors in critically ill patients.

Methods: This is a retrospective multicenter cohort study conducted in five hospitals in the Kingdom of Saudi Arabia (KSA). We enrolled patients with confirmed SARS-COV-2 infection admitted to any of the intensive care units from the five hospitals between March 2020 and July 2020, corresponding to the peak of recorded COVID-19 cases in the KSA.

Effect of lumbar puncture educational video on parental knowledge and self-reported intended practice.

Background: Lumbar puncture (LP) remains an essential diagnostic procedure for neurological and infectious diseases. However, it remains a source of anxiety to patients and families. This research aimed to assess the impact of a newly developed educational simplified video about LP, in the parents' native language, that is tailored to their social background and beliefs and to assess whether it can facilitate their consent for the procedure.

Acute kidney injury in children with COVID-19: a retrospective study.

Background: Acute kidney injury (AKI) is a complication of coronavirus disease 2019 (COVID-19). The reported incidence of AKI, however, varies among studies. We aimed to evaluate the incidence of AKI and its association with mortality and morbidity in children infected with severe acute respiratory distress syndrome coronavirus 2 (SARS-CoV-2) who required hospital admission.

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.

Coronavirus disease in children: A multicentre study from the Kingdom of Saudi Arabia.

Background: The COVID-19 global pandemic caused by severe acute respiratory syndrome coronavirus 2 infection, warranted attention for whether it has unique manifestations in children. Children tend to develop less severe disease with a small percentage present with clinical manifestations of paediatric multisystem inflammatory syndrome and have poor prognosis. We studied the characteristics of COVID-19 in children requiring hospitalisation in the Kingdom of Saudi Arabia and assessed the clinical presentation and the risk factors for mortality, morbidity, and paediatric intensive care (PICU) admission.

Diversity of Phenotype and Genetic Etiology of 23 Cystinuria Saudi Patients: A Retrospective Study.

Cystinuria is an inborn error of metabolism that manifests with renal stones due to defective renal epithelial cell transport of cystine which resulted from pathogenic variants in the and/or genes. Among nephrolithiasis diseases, cystinuria is potentially treatable, and further stone formation may be preventable. We report 23 patients who were identified biochemically and genetically to have cystinuria showing the diversity of the phenotype of cystinuria and expanding the genotype by identifying a broad spectrum of mutations.

Long-stay patients in pediatric intensive care units. Five-years, 2-points, cross-sectional study.

Objectives: To explore the changing patterns of long-stay patients (LSP) to improve the utilization of pediatric intensive care units (PICUs) resources.

Methods: This is a 2-points cross-sectional study (5 years apart; 2014-2019) conducted among PICUs and SCICUs in Riyadh, Saudi Arabia. Children who have stayed in PICU for more than 21 days were included.

Cinacalcet use in pediatric chronic kidney disease. A survey study.

Objectives: To evaluate the practice and attitude of pediatrics nephrologists about cinacalcet use in children.

Methods: An electronic structured questionnaire was answered by pediatric nephrologists practicing in the Kingdom of Saudi Arabia (KSA) and Gulf Council countries (GCC).

Results: A total of 42  pediatric nephrologists responded, of them, 42% used cinacalcet for young children ≤5 years of age and 79% used for children.

Correction to: Short-term outcome associated with disease severity and electrolyte abnormalities among critically ill children with acute kidney injury.

Following publication of the original article [1].

Hypokalemic periodic paralysis due to CACNA1S gene mutation.

Hypokalemic periodic paralysis (HypoPP) is a relatively rare but treatable disorder caused by mutations in the CACNA1S gene. HypoPP patients may experience paralytic episodes associated with hypokalemia and, infrequently, may develop late-onset proximal myopathy. The paralytic attacks are characterized by reversible flaccid paralysis and, in most cases, spare the respiratory muscles and heart.