BACKGROUND: Steroid-resistant nephrotic syndrome (SRNS) is a leading cause of stage 5 chronic kidney disease (CKD 5) in children and carries a substantial risk of post-transplant disease recurrence. Data from the Arabian...BACKGROUND: Steroid-resistant nephrotic syndrome (SRNS) is a leading cause of stage 5 chronic kidney disease (CKD 5) in children and carries a substantial risk of post-transplant disease recurrence. Data from the Arabian Peninsula are sparse. METHODS: This retrospective cohort study included all paediatric patients (aged < 18 years) who underwent a first kidney transplant at King Fahad Specialist Hospital-Dammam between January 2008 and December 2023 with a confirmed diagnosis of SRNS, focal segmental glomerulosclerosis (FSGS), congenital nephrotic syndrome, or a pathogenic monogenic variant known to cause SRNS. Patients were classified as having genetic or non-genetic SRNS according to next-generation sequencing results. Primary outcomes were post-transplant recurrence rate and graft survival. Secondary outcomes included estimated glomerular filtration rate (eGFR) values and treatment response. RESULTS: Among 208 paediatric kidney transplant recipients, 60 (28.8%) had CKD 5 attributable to SRNS/FSGS. Genetic testing (performed in 59/60 patients, 98.3%) identified pathogenic variants in 44 patients (74.6%); NPHS2 was most prevalent (28.8%). Post-transplant recurrence occurred in 6 patients (10%): 5 of 15 in the non-genetic group (33%) and 1 of 44 in the genetic group (2.3%) (p < 0.001). All six patients with recurrence received plasmapheresis; five also received rituximab. Complete remission was achieved in 5 patients (83%) and partial remission in 1 (17%). The 5-year graft survival rate was 98% overall-100% in the genetic group versus 93% in the non-genetic group (log-rank p = 0.21). The eGFR at 5 years was significantly higher in the genetic group (73.5 ± 17.6 vs. 60.6 ± 22.9 mL/min/1.73 m, p = 0.049). CONCLUSIONS: SRNS/FSGS accounts for approximately 29% of paediatric CKD 5 in this cohort and is characterised by a high prevalence of monogenic variants reflecting regional consanguinity. Genetic testing reliably stratifies recurrence risk: non-genetic SRNS carries a substantially higher recurrence rate, yet combined plasmapheresis and rituximab achieves remission in most affected patients. Graft outcomes are comparable to those seen in other CKD 5 aetiologies.
BACKGROUND: Although there are numerous studies on the parents of children undergoing peritoneal dialysis (PD) treatment, there are only a limited number of studies examining what children undergoing PD treatment experie...BACKGROUND: Although there are numerous studies on the parents of children undergoing peritoneal dialysis (PD) treatment, there are only a limited number of studies examining what children undergoing PD treatment experience until adulthood and their experiences with PD treatment during childhood. The aim of this study is to examine the changes that occur in the lives of adults who received PD treatment during childhood after diagnosis and their experiences related to the treatment process. METHODS: This retrospective and qualitative study was conducted with 18 adult participants living in the province of Izmir in Turkey who had received at least 6 months of PD treatment during childhood. Participants were selected using snowball sampling. A semi-structured interview form was used as the data collection tool. Study data were collected through face-to-face interviews using the individual in-depth interview technique. Data obtained from interviews were analyzed using Braun and Clarke's thematic analysis approach. RESULTS: The study identified four main themes: psychological effects, daily life effects, social effects, and academic effects. Psychological effects include the sub-themes of fear, shock, sadness, hopelessness, and shame. Daily life effects include the sub-themes of changes in eating habits, changes in body image, difficulty taking medication, repeated hospitalizations, stress coping, and adaptation. Social effects include social isolation, peritoneal dialysis room, social support, and personal life. Academic effects include the sub-themes of absenteeism, taking a break from education, academic failure, and being forced to change career choice. CONCLUSION: The findings from this research will provide guidance for a deeper understanding of the needs of children undergoing PD today and for initiatives planned in this context.
BACKGROUND: The long-term management of chronic kidney disease (CKD) in adolescents places a heavy burden on their families; it also adversely affects adolescents' quality of life (QoL). Family management adjustment and...BACKGROUND: The long-term management of chronic kidney disease (CKD) in adolescents places a heavy burden on their families; it also adversely affects adolescents' quality of life (QoL). Family management adjustment and healthcare transition (HCT) readiness have been identified as important protective factors for quality of life among adolescents. This study aimed to explore the association between family management adjustment and QoL in adolescents with CKD and verify the mediating effect of HCT readiness in this relationship. METHODS: A total of 354 adolescents with CKD aged 10-18 years (mean age 12.62; 52.8% male) and their parents were recruited. Participants completed the Family Management Measure, STARx Questionnaire, and PedsQL™ 4.0. Bivariate correlations, multiple linear regressions, and mediation analysis were the statistical analyses performed. RESULTS: The results revealed a significant indirect effect of family management adjustment on QoL via HCT readiness (β = 0.209, 95% CI [0.133-0.296]). The direct effect of family management adjustment on QoL remained significant after accounting for the mediator (β = 0.265, p < 0.001, 95% CI [0.140-0.390]), indicating a partial mediation effect of HCT readiness. CONCLUSIONS: This study shows that better family management adjustment is associated with better QoL in adolescents with CKD both directly and indirectly through its association with HCT readiness. These findings highlight the importance of supporting family management adjustment and fostering HCT readiness in clinical practice to optimize QoL in this vulnerable population.
BACKGROUND: Pediatric membranous nephropathy (MN) is a rare glomerular disease with limited data available. This single-center study aims to describe risk stratification-based treatment and its outcomes. METHODS: This re...BACKGROUND: Pediatric membranous nephropathy (MN) is a rare glomerular disease with limited data available. This single-center study aims to describe risk stratification-based treatment and its outcomes. METHODS: This retrospective observational study included children younger than 18 years of age with primary MN diagnosed from July, 2021 to December, 2024. KDIGO risk stratification criteria were modified with author consensus for pediatric thresholds, and children were stratified into low, moderate, high and very high risk. Immunosuppressive drugs included calcineurin inhibitors (CNIs), cyclophosphamide (CYC) and rituximab (RTX). Outcomes included complete remission, partial remission, no response, relapse, and decline in kidney function at 12 months and last follow-up, and were summarized across baseline risk categories and initial treatment groups. Time to first complete or partial remission was assessed using Kaplan-Meier analysis and compared using the log-rank test. RESULTS: Thirty-three children were included with a mean age of 11.15 ± 3.16 years, of whom 24 (73%) were males. Baseline risk stratification showed 24 (73%) were in the high-risk category. Initially, CNIs were administered in 17 (51.5%), while CYC and RTX in 5 (15%) and 3 (9%) patients, respectively. Among high-risk patients, non-response was observed in 8 (34%) at 12 months and 4 (17%) at last follow-up. Anti-PLA2R positivity was associated with better outcomes, with higher rates of complete or partial remission (p = 0.03). At last follow-up, 4 (12.1%) children demonstrated decline in kidney function. CONCLUSION: We describe treatment allocation and short-term outcomes in pediatric MN using a modified risk stratification-based approach. Higher risk categories appeared to have less favorable responses, while anti-PLA2R positivity was associated with better outcomes.
BACKGROUND: BK polyomavirus (BKPyV)-associated nephropathy in kidney transplant recipients is an important cause of allograft dysfunction and premature allograft failure. Treatment options for BKPyV-DNAemia following kid...BACKGROUND: BK polyomavirus (BKPyV)-associated nephropathy in kidney transplant recipients is an important cause of allograft dysfunction and premature allograft failure. Treatment options for BKPyV-DNAemia following kidney transplantation remain limited. Adjunctive pathogen adsorption devices such as the Seraph® 100 Microbind® Affinity Blood Filter (Seraph® 100) have previously been used for clearance of viruses, bacteria, and toxins in critical illness under FDA Emergency Use Authorization. CASE OUTLINE: An 18-year-old recipient of a deceased donor kidney transplant developed severe BKPyV-DNAemia and biopsy-proven BKPyV-nephropathy refractory to reduced immunosuppression, antiviral therapy, and adjunctive intravenous immunoglobulin (IVIG). Given progressive allograft dysfunction, consideration was given to adjunctive extracorporeal therapies targeting BKPyV-DNAemia removal. COMPLICATIONS: Despite multiple interventions including reduction of immunosuppression, cidofovir, leflunomide, and IVIG, BKPyV-DNAemia continued to worsen with levels reaching approximately 2 million copies/mL by 7 months post-transplant. The patient developed de novo donor-specific antibodies and biopsy-proven antibody-mediated rejection concurrent with BKPyV-nephropathy and rising serum creatinine. Within 8 weeks of Seraph® treatment, BKPyV-DNA levels resolved to < 5000 copies/mL. During the subsequent 12-month follow-up period, her graft function stabilized with serum creatinine of 1.2-1.3 mg/dL without proteinuria and stable class II DSA. LIST OF RELEVANT GUIDELINES: The Second International Consensus Guidelines on the Management of BK Polyomavirus in Kidney Transplantation (TTS 2024) [1] BK polyomavirus in solid organ transplantation - Guidelines from the American Society of Transplantation Infectious Diseases Community of Practice (AST-IDCOP 2019) [2].
Phosphate homeostasis is essential for skeletal integrity, cellular metabolism, and endocrine regulation. In humans, circulating phosphate is typically maintained between 2.5 and 4.5 mg/dL by coordinated processes that b...Phosphate homeostasis is essential for skeletal integrity, cellular metabolism, and endocrine regulation. In humans, circulating phosphate is typically maintained between 2.5 and 4.5 mg/dL by coordinated processes that balance gastrointestinal uptake from the diet, storage in cells and bone matrix, and renal reclamation that regulates excretion. Dietary phosphate enters primarily through the small intestine via a low‑affinity paracellular diffusion and a high‑affinity, sodium‑coupled transcellular route mediated by transporters such as NPT2b, PIT1, and PIT2. After absorption, phosphate is incorporated into hydroxyapatite within the skeleton and continuously shuttled between extra‑ and intracellular compartments mediated by transporters such as PIT1, PIT2, and XPR1 as metabolic demands change. At the kidney, proximal tubular transporters-especially NPT2a and NPT2c-set the tone for systemic phosphate balance. Endocrine regulators, including parathyroid hormone (PTH), fibroblast growth factor 23 (FGF23), and 1,25‑dihydroxyvitamin D (calcitriol), adjust intestinal uptake, skeletal turnover, and renal handling to avert hyper‑ or hypophosphatemia. Beyond these hormones, phosphate appears to signal directly to cells, engaging pathways such as ERK1/2 and thereby modulating secretion of PTH and FGF23. Perturbations in these circuits manifest as rickets, osteomalacia, and vascular calcification. Understanding the interplay between transport systems, hormonal control, and cellular phosphate sensing is crucial for preventing and treating phosphate‑related disease.
BACKGROUND: Severe carbamazepine intoxication in children may cause coma, seizures, respiratory failure, and hemodynamic instability. When gastrointestinal decontamination and supportive care are insufficient, extracorpo...BACKGROUND: Severe carbamazepine intoxication in children may cause coma, seizures, respiratory failure, and hemodynamic instability. When gastrointestinal decontamination and supportive care are insufficient, extracorporeal blood purification may be considered to enhance drug elimination. Hemoadsorption has recently emerged as a potential option, but pediatric evidence remains very limited. OBJECTIVES: To report the first pediatric case successfully managed with hemoadsorption and to systematically review extracorporeal removal strategies in pediatric carbamazepine intoxication. DATA SOURCES: CINAHL, Cochrane Library, Embase, MEDLINE via PubMed, Scopus, Web of Science, ClinicalTrials.gov, and reference lists of eligible articles were searched through April 6, 2025. STUDY ELIGIBILITY CRITERIA: Articles reporting pediatric patients (≤ 18 years) with acute carbamazepine intoxication who were treated with extracorporeal blood purification techniques were included. Eligible studies were randomized controlled trials, observational studies, case series, and case reports. Reports without extractable individual patient data were excluded. PARTICIPANTS AND INTERVENTIONS: Twenty studies provided individual data for 27 children (16 months-18 years) treated with charcoal hemoperfusion, hemodialysis, continuous kidney replacement therapy, therapeutic plasma exchange, or sequential/combined approaches. We additionally report one index case of acute carbamazepine overdose managed with isolated hemoadsorption using a CytoSorb cartridge. STUDY APPRAISAL AND SYNTHESIS METHODS: Records were screened, full texts were reviewed for eligibility, and individual patient data were extracted. Methodological quality was assessed using Joanna Briggs Institute critical appraisal checklists. Because of substantial heterogeneity, findings were summarized descriptively only, without meta-analysis, formal comparison, or ranking of extracorporeal modalities. RESULTS: Case report A 7-year-old girl was admitted to our hospital after ingestion of carbamazepine at a dose of 182 mg/kg, with an initial serum carbamazepine concentration of 144 µmol/L. She was unresponsive and hypotensive, with respiratory acidosis requiring endotracheal intubation. Isolated hemoadsorption using a CytoSorb cartridge on a multiFiltrate monitor was initiated 6.5 h after ingestion, and serum carbamazepine concentration decreased to the therapeutic range (17-51 µmol/L) within 18 h post-ingestion. The patient was extubated on day 2 and discharged on day 5 without neurological sequelae. SYSTEMATIC REVIEW: Twenty studies met the inclusion criteria and provided extractable individual data for 27 patients (16 months-18 years). Ingested doses ranged from 47 to 1077 mg/kg, with peak serum carbamazepine concentrations between 85 and 584 µmol/L. Manifestations included coma (85%), respiratory failure (56%), seizures (52%), and hypotension (26%). Management required mechanical ventilation (48%) and inotropic-vasopressor support (22%). Hemodialysis (56%), continuous veno-venous hemodiafiltration (26%), charcoal hemoperfusion (22%), therapeutic plasma exchange (19%), continuous veno-venous hemodialysis (7%), and continuous veno-venous hemofiltration (4%) were used. Documented time to normalization ranged from 4.5 to 84 h. Serum carbamazepine concentrations decreased across the included reports, and no deaths were reported. Pediatric intensive care unit and hospital lengths of stay ranged from 2 to 9 and from 2 to 26 days, respectively. LIMITATIONS: The evidence consisted of heterogeneous case reports and small case series, precluding causal inference or formal comparisons across extracorporeal modalities. CONCLUSIONS AND IMPLICATIONS OF KEY FINDINGS: Extracorporeal techniques may play an important role in severe pediatric carbamazepine intoxication when supportive measures are insufficient. Hemoadsorption may be feasible in carefully selected children. However, current pediatric evidence remains too limited to determine whether it offers any advantage over other extracorporeal modalities. Prospective registries, pharmacokinetic studies, and comparative evaluations are warranted to define indications, timing, and modality selection. SYSTEMATIC REVIEW REGISTRATION NUMBER: PROSPERO CRD420251236374.
Al Riyami MS, Al Wahaibi K, Ahmed H
… +9 more, Ourdoucen M, Al Azani M, Al Maskari A, Alfar A, Al Ghaithi B, Al Saidi S, Al Alawi I, Al Kalbani N, Al-Omairi A
BACKGROUND: Autosomal recessive polycystic kidney disease (ARPKD) is a rare inherited disorder characterized by kidney and hepatic involvement, often presenting in early life. Data from Arab populations are limited, part...BACKGROUND: Autosomal recessive polycystic kidney disease (ARPKD) is a rare inherited disorder characterized by kidney and hepatic involvement, often presenting in early life. Data from Arab populations are limited, particularly in Oman, where consanguinity is common and may influence genotype-phenotype correlations. METHODS: This retrospective study included Omani children (0-13 years) diagnosed with ARPKD between January 2011 and December 2024 at two national tertiary centers. Clinical features, genetic variants, kidney and hepatic outcomes, and mortality were analyzed. Kidney and hepatic severity were compared by genotype, and kidney and patient survival were assessed using Kaplan-Meier analysis. RESULTS: A total of 114 patients were included; 58.9% presented before 6 months, and 64% had parental consanguinity. Hypertension occurred in 55.3%, 60% developed chronic kidney disease (CKD), and 12.3% progressed to kidney failure (KF). Hepatic complications were frequent, including hepatomegaly (65.8%) and portal hypertension (20.2%). Genetic testing (n = 81) revealed a limited PKHD1 variant spectrum predominantly c.107C > T, c.406A > G (novel), and c.4870C > T with no truncating mutations identified. 107C > T homozygosity was associated with more severe kidney and hepatic disease, while c.4870C > T correlated with milder kidney involvement. Overall mortality was 10.5% and was associated with early presentation (p < 0.05). Kaplan-Meier analysis showed high survival, with median survival not reached; estimated 5- and 10-year survival rates were 96.2% and 82.6%, respectively. CONCLUSIONS: ARPKD in Omani children typically presents early and is strongly associated with high rates of consanguinity and recurrent PKHD1 founder variants, with heterogeneous kidney and hepatic outcome. Early diagnosis, genetic counseling, and multidisciplinary care is essential in this setting.
BACKGROUND: Obesity and hypertension are major risk factors for cardiovascular target organ damage in childhood, including increased left ventricular mass index (LVMI) and left ventricular hypertrophy (LVH). Blood pressu...BACKGROUND: Obesity and hypertension are major risk factors for cardiovascular target organ damage in childhood, including increased left ventricular mass index (LVMI) and left ventricular hypertrophy (LVH). Blood pressure variability (BPV) has been suggested as a potential contributor to cardiovascular damage; however, its role in pediatric obesity remains unclear. This study was aimed at evaluating short-term BPV and pulse wave analysis (PWA) parameters in children and adolescents with obesity and their associations with LVMI and LVH. METHODS: This retrospective cross-sectional study included 42 children and adolescents with obesity evaluated between October 2023 and August 2025, and 42 age- and sex-matched healthy children and adolescents (8-18 years). All participants underwent 24-h ambulatory blood pressure monitoring with simultaneous PWA and echocardiographic assessment of LVMI. Short-term BPV was assessed using standard deviation (SD), coefficient of variation (CV), and average real variability (ARV). LVH was defined as LVMI above the 95th percentile for age and height. Correlation analyses and multivariable logistic regression analysis were performed. RESULTS: Participants with obesity had higher ambulatory blood pressure levels, BPV indices, pulse wave velocity and central blood pressure values than healthy controls (p < 0.05). LVH prevalence was higher in the obese group (40% vs. 9%; OR = 6.29, 95% CI: 1.90-20.9). Short-term BPV parameters were not independently associated with LVH and showed no significant correlations with LVMI or the LVMI ratio. CONCLUSIONS: Despite increased BPV and arterial stiffness in children and adolescents with obesity, short-term BPV was not independently associated with LVMI or LVH. Further prospective studies are warranted to clarify the clinical relevance of BPV in pediatric obesity.
Complement dysregulation may occur secondary to systemic immune activation, but its relationship with inflammatory bowel disease (IBD) remains poorly defined. We report a 10-year-old boy presenting with severe anemia and...Complement dysregulation may occur secondary to systemic immune activation, but its relationship with inflammatory bowel disease (IBD) remains poorly defined. We report a 10-year-old boy presenting with severe anemia and newly diagnosed IBD who developed acute kidney injury with microscopic hematuria and proteinuria. Complement studies showed markedly reduced C3 and CH50 with normal C4. Kidney biopsy demonstrated diffuse proliferative glomerulonephritis with dominant C3 deposition and subendothelial immune complexes, consistent with C3 glomerulopathy. Additional complement testing revealed factor H depletion, high titers of anti-factor H autoantibodies, and elevated soluble C5b-9, while C3 nephritic factor and genetic testing of complement genes were negative. Multiple systemic autoantibodies were simultaneously present. Treatment with glucocorticoids resulted in rapid clinical improvement with normalization of kidney function, complement parameters, and disappearance of anti-factor H antibodies during follow-up. This case illustrates how transient systemic immune activation associated with intestinal inflammation may induce acquired complement dysregulation and trigger C3 glomerulopathy.
Askarian F, Karimizadeh Z, Kalantari A
… +10 more, Mousakhan Bakhtiari M, Karimzadeh A, Jahanpanah M, Eshghi S, Sandaramu N, Jafari L, Hamidieh AA, Salmanifard Ardestani MT, Behfar M, Pasha F
BACKGROUND: Acute kidney injury (AKI) is an understudied complication in pediatric Fanconi anemia (FA) patients undergoing hematopoietic stem cell transplantation (HSCT). This study evaluates AKI incidence, risk factors,...BACKGROUND: Acute kidney injury (AKI) is an understudied complication in pediatric Fanconi anemia (FA) patients undergoing hematopoietic stem cell transplantation (HSCT). This study evaluates AKI incidence, risk factors, renal trajectories, and survival impact in pediatric FA patients receiving uniform non-TBI conditioning. METHODS: This retrospective cohort study enrolled 37 pediatric patients (< 18 years) with confirmed FA who underwent allogeneic HSCT between April 2017 and June 2023 at Children's Medical Center, Tehran. AKI was defined and staged using KDIGO serum creatinine criteria, and renal function was tracked via estimated glomerular filtration rate (eGFR; bedside Schwartz equation). RESULTS: AKI developed in 13 of 37 patients (35.1%), mostly stage 1 (53.8%), with 92.3% of cases occurring within the first three months post-transplantation. Older age at HSCT (p = 0.023) and higher admission eGFR (p = 0.010) were associated with increased AKI risk. While binary CAKUT presence did not significantly correlate with AKI, subgroup analysis revealed single kidney or severe renal malrotation carried the highest risk. Longitudinal eGFR modelling showed a marked decline within one month post-HSCT and partial recovery by six months. Calcineurin inhibitor levels, conditioning intensity, GvHD grade, and CMV infection did not significantly correlate with AKI. CONCLUSIONS: AKI is common, mostly mild, and predominantly occurs early after transplantation, highlighting the need for risk-stratified renal monitoring, tailored immunosuppressive management, and long-term nephrological follow-up in FA patients surviving HSCT.
BACKGROUND: We aimed to determine the incidence, predictors, and outcomes of acute kidney injury (AKI) in children hospitalized with acute febrile tropical illnesses (AFTI). METHODS: This prospective observational study...BACKGROUND: We aimed to determine the incidence, predictors, and outcomes of acute kidney injury (AKI) in children hospitalized with acute febrile tropical illnesses (AFTI). METHODS: This prospective observational study was conducted at a tertiary-care centre in north India over 1.5 years. Consecutive children (2 months-18 years) admitted with AFTI were enrolled. AKI was defined using KDIGO criteria based on serum creatinine and urine output. The primary outcome was the incidence of AKI in children with AFTI. Secondary outcomes included predictors for AKI, need for organ support, and mortality. Least absolute shrinkage and selection operator (LASSO) regression followed by multivariable logistic regression with bootstrap internal validation was used to identify independent predictors of AKI. RESULTS: Amongst 585 enrolled children with AFTI, AKI occurred in 68 (11.6%), of whom 30 (44.2%) had severe AKI (KDIGO stage 2/3). With respect to etiology, AKI was significantly more frequent in dengue with warning signs, malaria, leptospirosis and brucellosis. Children with AKI had significantly higher requirements for intensive care interventions, including intubation, inotropes, kidney replacement therapy, and longer duration of hospitalization. Mortality was markedly higher in the AKI group (19.1% vs. 0.19%, p < 0.001). On multivariable logistic regression, age (months), inotropic support and use of ≥ 2 nephrotoxic medications in 24 h period were independently associated with development of AKI. CONCLUSIONS: AKI affects one in ten children hospitalized with AFTI and is strongly associated with disease severity, organ support, and mortality. Age, inotropic support and use of ≥ 2 nephrotoxic medications were independent predictors of AKI in children with AFTI.