Pediatric Nephrology最新文献

Background: IgA nephropathy (IgAN) is a common glomerular disease in children that may progress to chronic kidney disease (CKD) and kidney failure. Identifying reliable prognostic markers is important for guiding clinical management. This study investigated independent predictors of poor prognosis in pediatric IgAN and their impact on eGFR slope, as well as the dynamic patterns of complete kidney remission and relapse, predictive factors associated with remission, and the influence of remission on poor prognosis.

Methods: A retrospective cohort study of 224 children (aged 3-18 years) with biopsy-proven IgAN and ≥ 3 years of follow-up was enrolled. Poor prognosis was defined as persistent eGFR < 90 mL/min/1.73 m² for ≥ 3 months. Multivariate logistic regression, receiver operating characteristic (ROC) curve analysis, and linear mixed-effects model were used to identify predictive factors and evaluate eGFR slope. Complete kidney remission was defined as the absence of hematuria and proteinuria with eGFR ≥ 90 mL/min/1.73 m² for ≥ 1 year. Cumulative incidence function and Fine-Gray competing risk regression model were applied to analyze remission dynamic patterns.

Results: Over a median follow-up of 5.41 years, 12.05% reached the poor prognosis. Independent risk factors included male sex, older age at biopsy, Oxford classification E1 and S1 lesions, absence of gross hematuria, and no remission of proteinuria during follow-up, while higher birth weight was protective. eGFR slope was influenced by age at biopsy, gross hematuria and proteinuria remission status during follow-up. Complete kidney remission occurred in 70.98%, with a recurrence rate of 60.38%. Predictors of complete kidney remission included antecedent infection, kidney IgM deposition of 2 + , elevated serum IgM, increased pathological casts count, and prednisone treatment, while prolonged disease duration before biopsy and higher tubular casts count were risk factors. Group comparisons and logistic regression analysis showed no significant associations between kidney remission and poor.

Conclusions: Pediatric IgAN shows slow disease progression over 5 years. Despite high complete kidney remission rates, recurrence remains common. These findings support extended follow-up and improved predictive models.

Background: Diabetic kidney disease (DKD) is the primary cause of kidney failure in type 1 diabetes (T1D). Early identification of subclinical DKD is based on albuminuria and glomerular filtration rate (GFR). However, no GFR equations have been validated in youth with T1D. This study evaluates the performance of GFR equations in youth with T1D.

Methods: We compared a direct measure of GFR (mGFR) via plasma clearance of exogenous inulin, with estimated GFR (eGFR). eGFR was calculated with the following equations: Full-Age Spectrum (FAS-height and FAS-age); revised Lund-Malmö (LM-Rev); European Kidney Function Consortium (EKFC); Chronic Kidney Disease Epidemiology Collaboration, 2009 (CKD-EPI 2009); CKD-EPI 2021 (revised CKD-EPI 2009 without race adjustment); CKD-EPI40 (revised CKD-EPI 2021 with age-adjusted creatinine); Chronic Kidney Disease in Children (CKiD); CKiD Under 25 (sex-dependent); CKiD Under 25 (age- and sex-dependent); and Improving Renal Complications in Adolescents with Type 2 Diabetes through Research (iCARE). Bland-Altman analysis estimated performance (bias and accuracy (P10 and P30)).

Results: In total, 141 children and adolescents at a mean age of 13.5 ± 3.3 years (range 6.2-18.5) and diabetes duration 5.7 ± 1.6 years were included. Their mean serum creatinine was 45.6 ± 12.1 μmol/L (0.52 ± 0.14 mg/dL) and mGFR 142.3 ± 25.0 mL/min/1.73 m². For the whole population, FAS-height (bias = 8.37 mL/min/1.73 m²; P30 = 85.1%) and CKiD (bias = 11.9 mL/min/1.73 m²; P30 = 81.6%) performed best. These equations also outperformed others in subgroups (females, age groups < 11 years and 11-15.9 years). In contrast, CKiD in males (bias = 5.23 mL/min/1.73 m²; P30 = 81.0%) and CKD-EPI 2009 in the 16-18.5 years subgroup (bias = -0.04 mL/min/1.73 m²; P30 = 84.6%) were top performers.

Conclusions: Subgroups of children and adolescents with T1D may benefit from different eGFR equations for accurate subclinical DKD evaluation.

Proton pump inhibitor (PPI)-induced acute tubulointerstitial nephritis (ATIN) is rarely recognized in children. A 16-year-old boy presented with a 2-month history of vomiting, abdominal pain, and weight loss and had received PPIs repeatedly. He had elevated creatinine, metabolic acidosis, sterile pyuria, glucosuria, and markedly increased urine β2-microglobulin. Ultrasound showed enlarged echogenic kidneys. Despite hydration, kidney function did not improve. Kidney biopsy revealed T lymphocyte-predominant tubulointerstitial inflammation with eosinophils, consistent with hypersensitivity-mediated ATIN due to PPI use. He recovered fully with corticosteroids and cessation of PPI use. Concurrent Helicobacter pylori gastritis was treated with a PPI-free eradication regimen. This case highlights the importance of considering PPI-associated ATIN in unexplained pediatric AKI.

Autosomal dominant hypocalcaemia type 1 is rare and clinically challenging. Altered calcium handling may lead to progressive nephrocalcinosis and chronic kidney disease. We present the first known report of a child with ADH1 caused by the genetic variant c.2528C > A; p.Ala843Glu, who successfully underwent kidney transplantation without simultaneous parathyroid gland transplant aged 11yrs. We outline our reasoning for this and our management strategy for maintaining calcium homeostasis post-transplant over a 4-year period.

Background: Skin involvement in haemolytic uremic syndrome (HUS) raises suspicion for atypical HUS; here, we report a child with gangrene and confirmed STEC-HUS.

Case-diagnosis/treatment: A 3-year-old boy with aggressive HUS presented with leukocytes 33,100/mm³, haemoglobin 7.8 g/dL, platelets 93,000/mm³, LDH 6020 IU/L, creatinine 2.4 mg/dL, sodium 124 mEq/L, albumin 1.9 g/dL, C-reactive protein 94 mg/L. He required peritoneal dialysis, mechanical ventilation for seizures, and milrinone for cardiac dysfunction. On day seven, ischemic lesions on two fingertips developed, prompting plasma infusions. Skin biopsy confirmed thrombotic microangiopathy (TMA). Further investigations confirmed STEC infection (anti-LPS IgM positive) and excluded aHUS and other TMA causes, allowing plasma therapy discontinuation and avoidance of eculizumab. After 21 days of dialysis and 13 days of mechanical ventilation, the patient was discharged. Two months later, fingertip auto-amputation occurred.

Conclusions: This case highlights the importance of differentiating STEC-HUS from aHUS when skin involvement is present, given the major therapeutic and prognostic implications.

Background: The effect of continuous kidney replacement therapy (CKRT) with regional citrate anticoagulation (RCA) on bone mineral disease of acute kidney injury-disease (AKI-D) has not been well studied. We designed a case-control study to evaluate osteopenia and mineral balance markers in prolonged CKRT with RCA.

Methods: Cases were patients with AKI-D on CKRT with RCA; controls were patients immobilized ≥ 28 days, matched with propensity scoring. Data collected at day 0, 14, and 28. Two blinded radiologists independently evaluated for osteopenia/fractures.

Results: Osteopenia in cases was higher at day 14 (20/53 cases vs. 10/49 controls, p 0.05), and day 28 (21/53 cases vs. 11/49 controls, p 0.06). Younger age, CKRT, gastrointestinal/liver comorbidity increased the odds of osteopenia in cases and controls. Citrate rate adjusted for blood flow had higher odds of day 28 osteopenia. New fractures were higher in cases (13/53) than controls (3/49) (p 0.01). Younger age and osteopenia at baseline, day 14, and 28 had higher odds of fractures in cases. There was moderate agreement among radiologists for osteopenia (Kappa 0.62).

Conclusions: This is an important comparative study in children with AKI-D on prolonged CKRT and bone complications. Increased fractures and osteopenia were noted in children undergoing prolonged CKRT compared to immobilization alone. Increased risk of fractures was associated with the presence/persistence of osteopenia and younger age. Further research is needed to elucidate underlying mechanisms and optimize management strategies for osteopenia and fractures in patients receiving prolonged CKRT.

Background: Kidney scarring (KS) secondary to urinary tract infections is a common cause of secondary hypertension in children. We investigated the association between serum cystatin C and hypertension in children with KS using 24-h ABPM.

Methods: One hundred eleven children (aged 6-18 years) with DMSA-confirmed KS were included. All patients underwent 24-h ABPM, and serum cystatin C, creatinine, creatinine-based eGFR, and cystatin C-based eGFR were calculated. Hypertension was defined according to current AAP and ESH recommendations, using age and gender/height-specific threshold values in ABPM. The relationships between hypertension, biochemical parameters, and scar severity were assessed using multivariate analysis. No participant was taking antihypertensive medication at the time of ABPM.

Results: Hypertension was detected in 36.9% (n = 41) of patients. Serum cystatin C levels were significantly higher in the hypertensive group (1.06 mg/L vs. 0.94 mg/L, p = 0.004). When assessed for kidney function, both creatinine-based eGFR (p = 0.044) and cystatin C-based eGFR (67 [42-183] vs. 74.9 [30.9-183] mL/min/1.73 m², p = 0.004) were significantly lower in the hypertensive group than in the normotensive group. Mean systolic nocturnal dip was < 10% (non-dipper) in both groups. In multivariate logistic regression, only high-grade scarring (Grades 3-4) remained an independent risk factor, increasing the risk of hypertension by 3.44-fold (95% CI: 1.45-8.16, p = 0.005).

Conclusions: High-grade scarring is a significant independent risk factor for hypertension. Although cystatin C reflects the severity of kidney damage, its association with hypertension depends on the scar burden. Since circadian rhythm disturbances (non-dipping) are common, even in children with normal office blood pressure, ABPM may be necessary for monitoring those with KS.