Pediatric Nephrology最新文献

Background: Some inborn errors of metabolism (IEMs) resulting in aberrations to blood leucine and ammonia levels are commonly treated with kidney replacement therapy (KRT). Children with IEMs require prompt treatment, as delayed treatment results in increased neurological and developmental morbidity.

Objectives: Our systematic review in neonates and pediatrics evaluates survival rates and reductions in ammonia and leucine levels across different KRT modalities (continuous KRT (CKRT), hemodialysis (HD), peritoneal dialysis (PD)).

Data sources: A literature search was conducted through PubMed, Web of Science, and Embase databases for articles including survival rate and toxic metabolite clearance data in pediatric patients with IEM undergoing KRT.

Study eligibility criteria: Cross-sectional, prospective, and retrospective studies with survival rates reported in patients with IEM with an intervention of CKRT, PD, or HD were included. Studies with patients receiving unclear or multiple KRT modalities were excluded.

Study appraisal and synthesis methods: Analysis variables included efficacy outcomes [% reduction in ammonia (RIA) from pre- to post-dialysis and time to 50% RIA] and mortality. The Newcastle Ottawa Risk of Bias quality assessment was used to assess bias. All statistical analyses were performed with MedCalc Statistical Software version 19.2.6.

Results: A total of 37 studies (n = 642) were included. The pooled proportion (95% CI) of mortality on CKRT was 24.84% (20.93-29.08), PD was 34.42% (26.24-43.33), and HD 34.14% (24.19-45.23). A lower trend of pooled (95% CI) time to 50% RIA was observed with CKRT [6.5 (5.1-7.8)] vs. PD [14.4 (13.3-15.5)]. A higher mortality was observed with greater plasma ammonia level before CKRT (31.94% for ≥ 1000 µmol/L vs. 15.04% for < 1000 µmol/L).

Conclusions and implications of key findings: Despite the limitations in sample size, trends emerged suggesting that CKRT may be associated with lower mortality rates compared to HD or PD, with potential benefits including prevention of rebound hyperammonemia and improved hemodynamic control. While HD showed a trend towards faster achievement of 50% RIA, all modalities demonstrated comparable efficacy in reducing ammonia and leucine levels.

Prospero registration: CRD42023418842.

Biliary, Renal, Neurological, and Skeletal syndrome (BRENS syndrome) is a very rare ciliopathy caused by variants in the TTC26 (OMIM 617453) gene. There are only a few case reports of BRENS syndrome in the literature. We report here a Chinese case of BRENS syndrome who presented with kidney, neurological, skeletal, and other features. It is the first description of BRENS syndrome without biliary involvement. Gene testing revealed three novel compound heterozygous variants in the TTC26 gene, c.1069 + 5G > A in one allele from the mother and c.511A > G (p.Ile171Val) and c.1099T > C (p.Ser367Pro) in another allele from the father. We suggest that patients with BRENS syndrome may exhibit variable phenotypes.

Background: Children with chronic kidney disease (CKD) face extensive healthcare needs, leading to substantial financial strain on both families and healthcare systems due to costly kidney replacement therapies and associated comorbidities. Limited research on inpatient healthcare utilization is available for the individual stages of pediatric CKD.

Methods: This retrospective cohort study included inpatient encounters for pediatric patients (≤ 18 years) using the Pediatric Health Information System Database (PHIS) between January 2016 and December 2022, with an ICD-10 code for any CKD stage (1-5). Hospitalization cost, length of stay (LOS), morbidity, and mortality data were collected.

Results: We identified 23,980 pediatric CKD cases [stage 1: 5,059, stage 2: 6,763, stage 3: 7,012, stage 4: 3,102, and stage 5: 2,044] across 49 different children's hospitals in the United States. Mortality rates were observed to increase with increasing CKD severity, but no clear trend was observed for surgical, medical, and infection rates by CKD stage. The LOS for patients with CKD stage 4 and CKD stage 5 was 56% and 71% longer relative to the LOS of a typical hospitalization for a patient without CKD, and the billed charges for these hospitalizations were 92% and 147% higher than those of a typical hospitalization.

Conclusions: LOS and hospitalization costs were significantly higher for patients with CKD than for patients without CKD. Furthermore, pediatric patients with stage 5 CKD had significantly higher in-patient costs and all-cause hospitalization LOS compared to those with CKD stages 1-4.

Posterior urethral valves (PUV) are the most common cause of congenital urethral obstruction and are unique in the challenges they pose in management. Endoscopic ablation/incision of the valves is usually offered as the primary treatment of choice. Following this, a range of different clinical patterns are observed, each with varying bladder dysfunction and continence issues and associated with different grades of chronic kidney disease. This review outlines a systematic approach that could help pediatric nephrologists, pediatricians, and pediatric urologists, as well as nursing and allied health specialists, assess these children and develop well-informed management plans. The need for surveillance for bladder dysfunction (incidence approximately 55%), identification of red flags for progression to kidney failure (incidence approximately 20%), and multidisciplinary approach to care are presented, with a focus on reducing long-term morbidity in patients and difficulties for the families. Where possible, an alternative in resource-constrained situations is suggested. We also briefly outline the role of pharmacotherapy, assisted bladder emptying/drainage, and other interventions that have a role in the medium- to long-term management of these patients.

Xanthogranulomatous pyelonephritis is a rare condition in paediatric patients, mostly described in middle-aged female patients. We present a 7-year-old female with juvenile idiopathic arthritis, who was found to have a kidney mass with a concurrent Escherichia coli urinary tract infection. Surgical excision was done out of concern for possible malignancy. Histology confirmed the diagnosis of xanthogranulomatous pyelonephritis and persistent E. coli infection.

Background: Pneumococcal vaccines have been available worldwide since the early 2000s; consequently, few reports exist of poststreptococcal acute glomerulonephritis (PSAGN) or complications of pneumococcal infection. We describe a patient with PSAGN and bacteremia with Streptococcus pneumoniae serotype 22F (not covered by the 13-valent pneumococcal vaccine (PCV 13)).

Case diagnosis/treatment: A 5-year-old boy received the PCV13 vaccine and was admitted to our hospital with a fever and gross hematuria. A throat swab was positive for a streptococcal antigen, and his serum anti-streptolysin O and creatinine levels were increased. Low serum C3 levels suggested PSAGN, with an infiltrating shadow on chest X-ray. His blood culture isolated S. pneumoniae serotype 22F, and he was administered intravenous ceftriaxone for 10 days. His kidney function, pneumonia, and bacteremia improved.

Conclusions: Children with PSAGN should be evaluated for pneumococcal bacteremia due to strains not covered by the vaccine.