Pediatric Nephrology最新文献

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.

Background: A 2017 international survey of neonatologist and pediatric nephrologist (PN) perceptions and practice showed that neonatal acute kidney injury (AKI) was underappreciated, and diagnosis and management varied significantly across centers. An updated survey was developed to determine whether perceptions and practices among international pediatric subspecialists have changed with increased awareness of AKI.

Methods: A 50-question electronic survey of AKI detection, monitoring, and follow-up was distributed to neonatologists, PNs, pediatric intensivists, and advanced practice providers (APPs) via 11 professional organizations (mid-March to mid-May 2024). Responses were compared by subspecialty and economic classification of respondents' countries.

Results: Respondents totaled 676 (44.4% neonatologists, 38.3% PNs, 12.0% APPs, < 1.0% pediatric intensivists) representing 68 countries (36.7% high-income, 32.3% upper-middle income, 23.5% lower-middle income, 5.9% low-income, and 1.5% with unclassified economy). The modified, neonatal KDIGO criteria were most frequently used overall (46.1%) to diagnose AKI; however, only 30.7% of neonatologists and 14.8% of APPs utilized KDIGO. Most respondents reported no AKI identification (75.8%) nor monitoring protocol (75%). However, we identified higher rates of reported creatinine surveillance around aminoglycoside (72.9%) and indomethacin use (61.9%) than previously reported (34-36% and 24-62%, respectively) and improved creatinine access via a routine electrolyte panel (> 80% vs. 25-40% in the original survey). PN's availability increased from 75% to > 80%.

Conclusions: Neonatal AKI perceptions and practices continue to vary across subspecialities, though improvements in access to serum creatinine monitoring and nephrology consultation are noted. Standardized approaches for AKI detection, management, and follow-up remain lacking indicating opportunities to improve care through provider education and guideline development.

An underlying medical condition as a cause for elevated blood pressure is suspected when hypertension occurs in younger children or those with markedly high readings (hypertensive urgency or emergency). We highlight a unique presentation and underlying medical condition, polycythemia vera (PV), as the cause of secondary hypertension in an asymptomatic 5-year-old child presenting for a routine physical examination and found to have hypertensive urgency. Laboratory evaluations revealed leukocytosis, polycythemia, thrombocytosis, and low erythropoietin level. JAK2-V617F mutational analysis confirmed the diagnosis of PV. She was managed with antihypertensive medications, therapeutic phlebotomy, and cytoreductive therapy with notable improvement in hypertension over time. She continues regular follow-up with nephrology and hematology/oncology. She has not had any further serious complications or evolution of her PV. This case demonstrates the importance of a broad differential diagnosis in the setting of very early onset hypertension, including myeloproliferative disorders.