Pediatric Nephrology最新文献

Background: Idiopathic nephrotic syndrome (NS) is the most common glomerular disease in children. While corticosteroids remain the first-line treatment for inducing remission, their prolonged or frequent use can suppress the hypothalamic-pituitary-adrenal (HPA) axis, potentially leading to adrenal insufficiency (AI). However, the true burden of this complication remains poorly understood and inconsistently reported. This study aimed to systematically evaluate the prevalence, diagnostic methods, risk factors, and clinical implications of steroid-induced AI in children with NS.

Methods: A systematic search of Medline was conducted up to October 3, 2025, and the references of relevant publications were also hand-searched for eligible studies. We looked for studies that assessed adrenal function or AI in pediatric patients with NS treated with corticosteroids. Only English-language studies were included; case reports, abstracts, and reviews were excluded. In total, 13 studies involving 516 pediatric patients met the inclusion criteria. The review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA 2020) guidelines and was registered in the PROSPERO database (CRD420251082353, June 27, 2025). Due to methodological heterogeneity, a narrative synthesis was performed.

Results: The reported prevalence of AI ranged from 5.9 to 92.9%, largely influenced by the diagnostic test used and the timing of assessment. Seven different testing methods were identified, with the 2-h ACTH stimulation test demonstrating the highest diagnostic yield. Definitions of AI and cortisol cutoff values varied considerably across studies. AI was more commonly observed in children with frequently relapsing or steroid-dependent NS, as well as in those with prolonged or repeated exposure to corticosteroids. Associations with age were inconsistent. AI was also linked to an increased risk of relapse, particularly during infections. Children with suppressed adrenal function may be at risk of adrenal crisis if not administered stress-dose corticosteroids during periods of physiological stress.

Conclusion: Included studies were mostly small, single-center, and methodologically heterogeneous. There was a lack of consensus on diagnostic criteria and limited long-term follow-up data. Steroid-induced AI is a common and potentially under-recognized complication in children with idiopathic NS, especially in high-risk groups. Due to inconsistent diagnostic practices, the actual prevalence of AI is unclear. There is a critical need for further research, standardized testing protocols, and clinical guidelines. Clinicians may consider screening for AI in children with high cumulative steroid exposure and consider cortisol replacement accordingly.

Background: Antenatal hydronephrosis (HN) is the most common congenital urinary tract abnormality, but management is controversial. Current guidelines recommend continuous antibiotic prophylaxis (CAP), voiding cystourethrogram (VCUG), and diuretic renal scintigraphy (DRS) for high-grade HN, yet these measures may expose many asymptomatic infants to unnecessary antibiotics, radiation, and anesthesia. We evaluated whether a selective approach-reserving CAP for hydroureteronephrosis (HUN), limiting VCUG to HUN or confirmed urinary tract infection (UTI), and using DRS only when ultrasound trends were equivocal-could reduce interventions without increasing adverse outcomes.

Methods: We conducted a retrospective cohort study of 1,008 consecutive infants (< 24 months) with isolated HN or HUN (2015 and 2025). Patients were grouped by treatment era: 2015-2018 (pre-protocol), 2019-2021 (implementation), and 2022-2025 (post-clinic). Outcomes included use and duration of CAP, VCUG, and DRS, UTI incidence, and timing of surgery.

Results: VCUG (52% → 28%) and DRS (58% → 30%) use declined significantly over time. Median CAP duration decreased from 7.7 to 2.9 months (p < 0.001). Surveillance UTI rates remained stable (2-4%, p = 0.74). In isolated HN, CAP conferred no measurable benefit (number needed to treat [NNT] 100), whereas in HUN, CAP reduced infections (6% vs. 21%; NNT 27).

Conclusions: Selective management of antenatal HN-avoiding routine CAP, VCUG, and DRS in most infants-safely reduces unnecessary interventions without increasing UTI risk or delaying surgery. CAP should be reserved for higher-risk groups, such as infants with HUN, particularly uncircumcised males. These findings support updating international guidelines toward risk-based, individualized care.

Background: Magnetic resonance urography (MRU) examinations allow the assessment of kidney functions through recently developed image post-processing techniques, in addition to providing detailed visualization of urinary system anatomy. MRU and MAG3 scintigraphy were compared in terms of functional evaluation.

Methods: Dynamic contrast-enhanced MRU images of 76 children who had previously undergone MAG3 scintigraphy examinations were evaluated. Morphological parameters, volumetric split renal functions (SRF), and renal transit times (RTT) were calculated from the dynamic MRU phases using the CHOP-fMRU software. Results were compared with the half-time (T1/2) and SRF values obtained from MAG3 scintigraphy. Student's t-test and Mann-Whitney U test were used to compare quantitative variables, and Pearson correlation analysis was performed. ROC analysis was performed to reveal the prediction of obstruction by MRU-based quantitative parameters.

Results: The ages of included patients ranged from 1 to 216 months (median: 59.50 months, IQR = 4.1-179.5 months). Statistically significant positive correlations (r > 0.9, p = 0.001) were found between SRFs of the right and left kidneys, measured by MRU and scintigraphy. When the cut-off value was set to 11.5 min for RTT, the sensitivity, specificity, positive and negative predictive values, and accuracy of RTT were found to be 94%, 86%, 90%, 88%, and 88%, respectively.

Conclusion: MRU could be an invaluable tool in the assessment of renal functions. Its diagnostic accuracy in detecting the level and extent of obstruction is comparable to that of MAG3 scintigraphy. Despite the lack of extensive comparative data, the potential benefits of MRU justify the need for further studies.

Despite an increasing number of therapeutic options for pediatric patients with steroid-resistant nephrotic syndrome (SRNS), treatment resistance and risk of progression to kidney failure are still high. Children with monogenic forms of SRNS and resistance to non-steroidal immunosuppressants are at the highest risk. Advances in the understanding of SRNS pathogenesis have enabled the development of novel therapies that target genetic, immunologic, and metabolic mechanisms of disease development and progression. In this review, we summarize select contemporary cohort study findings, novel therapeutic agents and their mechanistic targets, and eight recent and ongoing clinical trials for pediatric patients with SRNS.

Background: Primary hyperoxaluria type 2 is a rare genetic disorder of oxalate due to a defect in the glyoxalate reductase/hydroxypyruvate reductase enzyme. This study aimed to describe the characteristics and outcomes in a pediatric population from a single center in Pakistan.

Methods: This study was conducted at the Sindh Institute of Urology and Transplantation (SIUT), Karachi, from January 2010 to December 2022, involving children under 18 years with nephrocalcinosis. Data collected included demographics, clinical features, laboratory findings, imaging results, family history of kidney stones, and consanguinity. Genetic testing, including next-generation sequencing and Sanger sequencing, was performed, and patients were followed for 24 months to monitor the progression of chronic kidney disease (CKD) stages.

Results: Fifty-two children were diagnosed with primary hyperoxaluria type 2 (PH2) confirmed by genetic testing. The majority were male (56%), between 5 and 10 years of age (46%), and from the Sindh province (62%). Seventeen distinct GRHPR gene mutations were identified, predominantly missense variants. The most frequent mutation was Gly165Asp (observed in 12 patients), followed by Leu6Phe and Trp138Arg (8 and 7 patients, respectively). Six of the identified mutations were novel. At presentation, 30% of children were in CKD stage 5, and this proportion increased to 42% after 24 months of follow-up. Male sex and higher baseline serum creatinine were significant predictors of progression to CKD stage 5.

Conclusions: This is the first reported PH2 cohort from Pakistan, highlights a significant disease burden with diverse GRHPR mutations, with most patients presenting in advanced CKD stage 5 at diagnosis.