Peritonitis in children on peritoneal dialysis: 12 years of tertiary center experience.

Background And Objective: Peritoneal dialysis (PD) associated peritonitis is the most common cause of morbidity, mortality, and treatment failure in patients undergoing PD. We aimed to identify the incidence, pathogens, antibiotic susceptibility, and the outcome of peritoneal dialysis (PD)-associated peritonitis in children.

Methods: Data from medical records of children who underwent PD between 2007 and 2018 in King Fahad Medical City were retrospectively collected.

Clinical improvement of encapsulating peritoneal sclerosis after challenging course and 6 months of total parenteral nutrition in child with nephronophthisis: a case report.

Background: Encapsulating peritoneal sclerosis is a rare but potentially lethal complication of long-term peritoneal dialysis that is associated with significant morbidity and mortality. The occurrence of encapsulating peritoneal sclerosis varies worldwide, but is increased in patients maintained on peritoneal dialysis for 5-8 years. The etiology of encapsulating peritoneal sclerosis remains unidentified, and a high index of clinical suspicion is required for diagnosis.

A discarded synonymous variant in NPHP3 explains nephronophthisis and congenital hepatic fibrosis in several families.

Half of patients with a ciliopathy syndrome remain unsolved after initial analysis of whole exome sequencing (WES) data, highlighting the need for improved variant filtering and annotation. By candidate gene curation of WES data, combined with homozygosity mapping, we detected a homozygous predicted synonymous allele in NPHP3 in two children with hepatorenal fibrocystic disease from a consanguineous family. Analyses on patient-derived RNA shows activation of a cryptic mid-exon splice donor leading to frameshift.

Improved Renal Recovery with Eculizumab Therapy among Children with High Prevalence of Mutation-Associated Atypical Hemolytic Uremic Syndrome: A Retrospective Cohort Study.

Introduction: Genetic defects that determine uncontrolled activation of the alternative complement pathway have been well documented, which account for approximately 40-60% of atypical hemolytic uremic syndrome (aHUS) cases worldwide. In Saudi Arabia, nearly half of the marriages are consanguineous, resulting in a high prevalence of such genetic diseases. Recent studies have demonstrated the effectiveness of eculizumab against aHUS.

Fungal peritonitis in children on peritoneal dialysis at a tertiary care Centre.

Background: Fungal peritonitis (FP) is an infrequent but serious complication in children undergoing peritoneal dialysis (PD). This study aimed to explore the risk factors, clinical manifestations, causative organisms, fungal susceptibility findings, and outcomes of FP in children from Saudi Arabia.

Methods: In this case-control study, the medical records and laboratory results of paediatric patients aged 0-14 years who underwent PD were reviewed for FP episodes.

Spectrum of pediatricbiopsy-proven renal diseases: A single center experience.

Glomerular diseases are considered to be a significant cause of chronic kidney disease. Kidney biopsy continues to be an essential diagnostic tool. We review the renal biopsies which were done on children below the age of 14 years in the past 10 years (from January 2008 to September 2018) in a single tertiary pediatric hospital in Saudi Arabia to determine the patterns of renal disease among Saudi children as well-correlating clinical presentation with histopathological diagnosis.

Collagenofibrotic (Collagen Type III) glomerulopathy in association with diabetic nephropathy.

Collagenofìbrotic (collagen type III) glomerulopathy (CG) is a rare nonimmune-mediated glomerular disease. It is characterized by massive deposition of organized collagen type III fibers, which is localized in the mesangial and subendothelial glomerular areas and associated with increased serum levels of procollagen type III peptide. Association with systemic diseases and malignancies is extremely rare.

Rituximab for refractory cases of childhood nephrotic syndrome.

Rituximab has been used over the last decade as a rescue therapy for refractory cases of nephrotic syndrome (NS). Here we report the use of rituximab in four children with idiopathic steroid-resistant nephrotic syndrome (SRNS) with various histological backgrounds (two cases with focal segmental glomerulosclerosis, one case with IgM nephropathy, and one case with minimal change disease), who failed to respond to other immunsuppressions. Their median age (range) was 10 (8-11) years.

Risk factors for catheter-related complications in pediatric peritoneal dialysis.

Catheter-related complications, including infection, dialysate leak, and malfunction, are the principal causes of peritoneal dialysis (PD) failure. To determine risk factors predisposing to these complications, we conducted a retrospective study of 90 children on chronic PD who received 127 catheters from January 1990 to December 2000. There was a significant risk for dialysate leak when PD catheters were used early (14 days).