Pediatric Nephrology最新文献

Assessment of the true impact of therapeutic interventions is a challenge in the absence of universal, standardized definitions for clinical trial endpoints in children with kidney diseases. Steroid-resistant nephrotic syndrome (SRNS) is a difficult kidney disease to treat, with unremitting disease progressing to kidney failure. Currently, available therapies result in suboptimal cure rates. Clinical trials with innovative, targeted treatments will likely be conducted for this disease in the foreseeable future. An international consortium of the IPNA Best Practices and Standards Committee and the Pediatric Nephrology Expert Group of the conect4children (c4c) network developed through consensus, standardized, internationally acceptable definitions for trial outcomes for SRNS. The endpoint definitions were formulated for use with urine protein to creatinine ratios and estimated glomerular filtration rates. Definitions of complete remission, partial remission, non-remission of disease, reduction in proteinuria, kidney disease progression, kidney failure, and composite kidney outcome were refined using an iterative process until a consensus was achieved.

Background: While genetic testing is now more accessible in pediatric nephrology, little is known about the views of pediatric nephrologists regarding genetic testing in clinical settings.

Methods: An online 41-item survey was developed and distributed via professional listservs to self-identified U.S. licensed pediatric nephrologists from January 22 to May 4, 2021.

Results: Pediatric nephrologists had a high referral rate to genetic counseling and agreed on the significant impact of genetic testing on diagnosis, treatment, prognosis, counseling, and kidney transplant planning. Challenges for the utilization of genetic testing among pediatric nephrologists include the need to (1) learn how to counsel patients on the risks and benefits of genetic testing, (2) choose appropriate testing, (3) interpret genetic results, and (4) return those results to patients and families.

Conclusion: There exists an opportunity to expand genetic testing education for pediatric nephrologists to assist incorporation of genetic testing into clinical practice.

Despite its widespread use in patients undergoing organ transplantation or managing nephrotic syndrome, there is a lack of literature on the acute toxicity of cyclosporine A (CsA). This report presents a case of acute CsA toxicity resulting from an accidental overdose in a 2-year-old boy with steroid dependent nephrotic syndrome. Remarkably, despite a tenfold overdose and elevated trough levels, the patient remained asymptomatic, and the CsA trough level normalized within 48 h with rapid intervention, including discontinuation of CsA, intravenous hydration, and cytochrome P450 induction. This case emphasizes the critical importance of prompt and appropriate management to prevent serious consequences in such scenarios.

Background: Survival after pediatric liver transplantation has increased dramatically over the years, revealing extra-hepatic complications including impaired kidney function. We conducted a large single-center retrospective study to evaluate kidney outcomes after pediatric liver transplantation.

Methods: From electronic charts of 121 children who underwent liver transplantation during 2007-2020, we collected pre- and post-transplant data. We investigated the presence of post-transplant permanent kidney injury, including proteinuria, hypertension, and decreased estimated glomerular filtration rate (eGFR). We excluded children who died, underwent liver-kidney transplantation, or had less than 1 year of follow-up.

Results: During a median follow-up of 5.1 (interquartile range 2.9-7.3) years, eGFR decreased, mostly in the first year post-transplant. In addition, 41% of the children presented with acute kidney injury. At their last follow-up, 35% showed permanent kidney injury (hypertension 13%, proteinuria 36%, and eGFR < 90 mL/min per 1.73 m² 7%). Kidney ultrasounds were abnormal for 44% of the children at the last visit, compared to 11% before transplant (p < 0.001). In multivariate analysis, abnormal kidney ultrasound before transplant (odds ratio = 4.53, 95% CI 1.1-18.7) and liver disease with potential risk of primary kidney involvement (odds ratio = 4.77, 95% CI 1.58-14.4) were predictors for hypertension or decreased eGFR at the last follow-up.

Conclusions: The high prevalence of kidney injury after pediatric liver transplantation and the pretransplant predictors for kidney injury highlight the importance of a thorough kidney pretransplant evaluation and follow-up.

Background: Over the last 20 years, pediatric hypertension (pHTN) prevalence in Western society has risen from 3.5 to 9% due to childhood overweight, obesity, and secondary kidney and cardiological conditions. Few studies have assessed commonly used antihypertensive medication lisinopril's (ACE-inhibitor) long-term efficacy and the long-term value of renin-angiotensin-aldosterone system (RAAS) biomarkers.

Methods: This is a retrospective cohort study at Ghent University Hospital, Belgium, with 106 young patients (1-18 years) treated with lisinopril due to hypertension (HTN) and chronic kidney disease (CKD) assessed for treatment outcomes against clinical benchmarks over 10 years.

Results: Lisinopril was mainly initiated for secondary hypertension or nephroprotection (89%) due to kidney causes. A starting dose across groups was lower than 0.07 mg/kg for 48% (n = 50). HTN patients without CKD achieved systolic blood pressure below the 95th percentile within 2 years, but efficacy declined after 2.5 years. CKD patients maintained a steady response, reaching systolic targets by 40 months and showing improved diastolic control over 70 months. Proteinuria reduction had a median urine protein creatinine ratio (UPCR) to 0.57 g/g at 6 months, with a reappearance of UPCR 2 g/g creatinine after 40 months. Aldosterone breakthrough occurred from 6 months onward in all groups. Over 70 months, aldosterone and aldosterone-renin-ratio (ARR) progression significantly differ between children with and without normal kidney function.

Conclusions: Treatment efficacy for systolic blood pressure in hypertensive patients with abnormal kidney function diminishes after 2.5 years and for proteinuria in children after 3 years, highlighting the need for dosage recalibration according to guidelines and/or the need for alternative treatments.

Background: Hyponatremia can occur in the acute phase of any illness through various mechanisms. However, the frequency and severity of hyponatremia are not well known across a broad range of illnesses including medical and surgical diseases and trauma.

Methods: The present, retrospective chart review was conducted at Tokyo Metropolitan Children's Medical Center from 2018 to 2019. Included were healthy children aged < 16 years with an acute illness, who were urgently admitted, and had their serum sodium level measured on arrival.

Results: In total, 2717 patients were urgently admitted and had their serum sodium level measured. Of these, 1890 were included. Hyponatremia was found in 260 patients (13.8%). The most common hyponatremic disease was type 1 diabetes mellitus (69%) followed by acute infectious encephalopathy (60%), pyogenic arthritis (60%), and Kawasaki disease (51%). Kawasaki disease, seizure, urinary tract infection, acute appendicitis, lower respiratory tract infection, and acute gastroenteritis were associated with a significantly lower serum sodium value than cases of fracture comprising a control group. Conversely, acute bronchial asthma exacerbation (3%), anaphylaxis (0%), intussusception (0%), acute scrotal disease (0%), head injury (1%), and fracture (0%) were very infrequently associated with hyponatremia.

Conclusions: The present study determined the frequency and severity of hyponatremia in various, acute, pediatric illnesses, including medical and surgical diseases and trauma. Despite reports of respiratory distress and pain inducing vasopressin secretion, hyponatremia was rarely observed on arrival in patients with acute bronchial asthma exacerbation, anaphylaxis, intussusception, acute scrotal diseases, head injury, or fracture.