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BackgroundWe investigated the risk of kidney injury among adolescents with and without a congenital single functioning kidney (SFK).MethodsThis retrospective study is based on a medical evaluation database of 17-year-old Israeli conscripts, born during 1989–1999. Those with congenital SFK diagnosis, verified by a pediatric nephrologist’s review of the original military medical committee classifications, were compared to the rest of the cohort. Kidney injury (KI) was defined as proteinuria, high blood pressure (BP), or estimated glomerular filtration rate (eGFR) < 90 ml/min/1.73 m2 prior to army recruitment. Risk factors for KI were examined using logistic regression.ResultsOf 979,630 screened candidates, 353 were diagnosed with SFK. The yearly incidence of SFK gradually increased in the first years of the study, reaching a plateau in 1995 (5.5 ± 1.2/10,000 births/year). The male to female ratio was 2.7:1. Concomitant genital malformations were documented in 5.5% of those with SFK. KI was more prevalent in the SFK than the control group (42.2% vs. 23.5%, p < 0.001). All three components of KI were more common in the SFK than the control group: high BP (31.7% vs. 23.1%, p < 0.001), proteinuria (18.2% vs. 0.4%, p < 0.001), and eGFR <90 ml/min/1.73m2 (12.0% vs 0.1%, p < 0.001). Multivariate analysis of the SFK group revealed associations of higher mean BMI, male sex, and smaller ultrasonographic kidney length with KI.ConclusionsThis large population-based study documents a significant risk for KI among adolescents with SFK. Obesity represents a major modifiable risk factor for KI, implicating the need for closer follow-up in this group during childhood.

Relapses of steroid-sensitive nephrotic syndrome are traditionally treated with prednisone 2 mg/kg/day or 60 mg/m2/day. Retrospective data support the use of lower doses. We designed a prospective randomized pilot study to investigate the efficacy of different doses in achieving remission of steroid sensitive nephrotic syndrome relapse. The cohort included 30 children with relapsed steroid sensitive nephrotic syndrome, mean age 6.3 ± 3 years and mean disease duration 2.2 ± 1.8 years. The children were randomized to receive 2, 1.5, or 1 mg/kg/day prednisone. The corresponding times to response, defined as the first of 3 consecutive days without proteinuria, were 7.2 ± 1.4, 10.2 ± 5.1, and 9 ± 3.3 days; the difference between the 1.5 and 2 mg/kg/day groups was statistically significant. One patient each in the 1 mg/kg/day and the 1.5 mg/kg/day groups failed to respond and were switched to 2 mg/kg/day, leading to a response after 3 and 10 days, respectively. Mean cumulative prednisone doses in the 3 groups were 45.5 ± 3.4, 42.7 ± 25.9, and 24.9 ± 7.4 mg/kg, respectively (P < 0.05).Conclusion: In the present study, treatment of childhood steroid sensitive nephrotic syndrome relapse with prednisone 1–1.5 mg/kg/day led to a significantly lower cumulative dose than the standard dose. Treatment with a lower dose may be equally safe and effective to the standard dose.What is Known:• Relapses of steroid-sensitive nephrotic syndrome are traditionally treated with standard-dose steroids.• Treatment with corticosteroids may have significant adverse effects mainly with long-term use.What is New:• Treatment of steroid sensitive nephrotic syndrome relapse with 1–1.5 mg/kg/day prednisone may lead to a significantly lower cumulative dose.• Treatment with a lower steroid dose may be as effective as the standard dose in achieving remission of steroid sensitive nephrotic syndrome relapse.

Protective immunity against COVID-19 is orchestrated by an intricate network of innate and adaptive anti-viral immune responses. Several vaccines have been rapidly developed to combat the destructive effects of COVID-19, which initiate an immunological cascade that results in the generation of neutralizing antibodies and effector T cells towards the SARS-CoV-2 spike protein. Developing optimal vaccine-induced anti-SARS- CoV-2 protective immunity depends on a fully competent immune response. Some evidence was gathered on the effects of vaccination outcomes in immunocompromised adult individuals. Nonetheless, protective immunity elicited by the Pfizer Biontech BNT162b2 vaccine in immunocompromised adolescents received less attention and was mainly focused on the antibody response and their neutralization potential. The overall immune response, including T-cell activities, was largely understudied. In this study, we characterized the immune response of vaccinated immunocompromised adolescents. We found that immunocompromised adolescents, which may fail to elicit a humoral response and develop antibodies, may still develop cellular T-cell immunity towards SARS-CoV-2 infections. Furthermore, most immunocompromised adolescents due to genetic disorders or drugs (Kidney and liver transplantation) still develop either humoral, cellular or both arms of immunity towards SARS-CoV-2 infections. We also demonstrate that most patients could mount a cellular or humoral response even after six months post 2 nd vaccination. The findings that adolescents immunocompromised patients respond to some extent to vaccination are promising. Finally, they question the necessity for additional vaccination boosting regimens for this population who are not at high risk for severe disease, without further testing of their post-vaccination immune status.

BackgroundDuring kidney transplantation, the transplanted kidney undergoes ischemia reperfusion injury, with adenosine being a major mediator. This study aimed to assess whether aminophylline, an adenosine receptor antagonist, improves early graft function and reduces incidence of delayed graft function (DGF) and slow graft function (SGF).MethodsSingle center, double-blinded, placebo-controlled randomized clinical trial. Pediatric patients admitted for renal transplantation from donation after brain death donors were randomized into a treatment arm receiving aminophylline and a placebo arm receiving normal saline infusions. Primary outcome was estimated glomerular filtration rate (eGFR) at 5 days post-transplant. Secondary outcomes were rates of DGF/SGF and urinary neutrophil gelatinase–associated lipocalin (NGAL) levels.ResultsTwenty-three patients were randomized to aminophylline and 27 to placebo. There was no difference in day 5 eGFR, rate of DGF/SGF, or urine NGAL/Creatinine level between aminophylline vs. placebo arm (eGFR 67.39 ± 38.9 ml/min/1.73m2 vs. 80.48 ± 52.1 ml/min/1.73m2p = 0.32; DGF/SGF 5/23 (21.7%) vs. 3/27 (11.1%) p = 0.31; urine NGAL/creatinine 300.5 ng/mg IQR 105.5–1464.5 ng/mg vs. 425.4 ng/mg IQR 140.3–1126.2 ng/mg, p = 0.95; respectively). At 12 months, there was 100% patient survival and 98% graft survival. eGFR at 12 months was similar between the two arms.ConclusionsThere was no benefit in peri-transplant aminophylline administration. Our results are limited by small sample size, since sample calculations were based on primary outcome of day 5 eGFR and low rate of DGF/SGF, which may have precluded us from demonstrating efficacy. Further clinical studies are necessary to determine any benefit of aminophylline in kidney transplant recipients, particularly from high-risk donors.

Background Recombinant human erythropoietin, such as epoetin alfa and darbepoetin alfa, is an important therapy for anemia due to chronic renal failure. Allergy to recombinant human erythropoietin and the need for desensitization are rare. Case presentation We report here a novel epoetin alfa outpatient desensitization protocol in a girl who developed delayed cutaneous hypersensitivity to subcutaneous epoetin alfa and intravenous darbepoetin alfa. An initial attempt at traditional epoetin alfa desensitization failed, so we created a slower 17-day outpatient desensitization that succeeded and allowed treatment continuation. Conclusions This case highlights the notion that delayed-type hypersensitivity to recombinant human erythropoietin can occur as evident by reproducible reactions after repeated exposures and slow outpatient desensitization can be considered when a trial of more rapid induction of tolerance is unsuccessful.

BackgroundEculizumab has caused a revolution in the treatment and prognosis of atypical hemolytic uremic syndrome. Early initiation of treatment is recommended to increase chances of renal recovery.Case-diagnosis/treatmentWe describe a boy with atypical hemolytic uremic syndrome who started eculizumab therapy after being on dialysis for 4.5 months, with complete anuria. With treatment, he was weaned off dialysis.ConclusionWe review the evidence in the literature and discuss the possible mechanism by which eculizumab induces renal recovery even in patients already on prolonged dialysis. This case report highlights the importance of a treatment trial with eculizumab, even in patients already on prolonged dialysis.

BackgroundBlood pressure (BP) monitoring following pediatric kidney transplantation is essential for optimizing graft perfusion. Differences between invasive BP and noninvasive BP (NIBP) measurements are sometimes considerable. We aimed to assess agreement between invasive BP and NIBP in pediatric patients after kidney transplantation and compare with measurements obtained by systolic Doppler with manual sphygmomanometer as a reference technique.MethodsA prospective, observational cohort study, of children aged 18 years or younger, admitted immediately following kidney transplantation to the pediatric intensive care unit of a tertiary, university-affiliated medical center, between May 2019 and June 2021.ResultsEighty-two paired simultaneous measurements of invasive BP, NIBP, and Doppler BP in 18 patients were compared. Patients were significantly hypertensive, with mean systolic NIBP above the 95th percentile (96 ± 6%). Systolic invasive BP measurements were significantly higher than NIBP (149 ± 20 vs. 136 ± 15 mmHg, p < 0.001). Substantial differences (≥ 20 mmHg) were found in 23% (95% CI 15–34%). Similar disagreement was found between systolic invasive and Doppler BP (150 ± 23 and 137 ± 17 mmHg, respectively, p < 0.001). In contrast, systolic NIBP was in good agreement with Doppler BP (135 ± 17 and 138 ± 18, respectively, p = 0.27). A moderate to strong correlation was found between higher systolic invasive BP and the difference to systolic Doppler BP (Spearman's ρ = 0.63, p < 0.001).ConclusionsIn children immediately following kidney transplantation, clinically significant disagreement was found between invasive and noninvasive BP measurements. Invasive BP values were significantly higher than those obtained by Doppler. Better agreement was found between NIBP and Doppler. These issues should be considered when interpreting BP measurements in this sensitive patient population.A higher resolution version of the Graphical abstract is available as Supplementary information

Background Incremental hemodialysis follows the concept of adjusting dialysis dose according to residual kidney function. Data on incremental hemodialysis in pediatric patients is lacking. Methods We conducted a retrospective analysis of children initiating hemodialysis between January 2015 and July 2020 in a single tertiary center, comparing the characteristics and outcomes of those who commenced with incremental hemodialysis vs with conventional thrice-weekly regimen. Results Data on forty patients, 15 (37.5%) on incremental hemodialysis and 25 (63%) on thrice-weekly hemodialysis were analyzed. No differences in age, estimated glomerular filtration rate and metabolic parameters were noted between groups at baseline, but there were more males (73 vs 40%, p  = 0.04), more patients with congenital anomalies of kidney and urinary tract (60 vs 20%, p  = 0.01), higher urine output (2.5 ± 1 vs 1 ± 0.8 ml/kg/h, p  < 0.001), lower use of antihypertensive medications (20 vs 72%, p  = 0.002) and lower prevalence of left ventricular hypertrophy (6.7 vs 32%, p  = 0.003) in the incremental hemodialysis group vs thrice-weekly hemodialysis. During follow up, 5 (33%) incremental hemodialysis patients were transplanted, 1 (7%) remained on incremental hemodialysis at 24 months, and 9 (60%) transitioned to thrice-weekly hemodialysis at a median (IQR) time of 8.7 (4.2, 11.8) months. At last follow up, fewer patients who initiated incremental hemodialysis had left ventricular hypertrophy (0 vs 32%, p  = 0.016) and urine output < 100 ml/24 h (20 vs 60%, p  = 0.02) compared to thrice-weekly hemodialysis, with no significant differences in metabolic or growth parameters. Conclusion Incremental hemodialysis is a viable option for initiating dialysis in selected pediatric patients, that may help improve patients’ quality of life and reduce dialysis burden without compromising clinical outcome. Graphical abstract

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 m 2 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. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information

To compare the application of three standardized definitions of acute kidney injury (AKI), using corrected serum creatinine values, in children immediately after liver transplantation. Retrospective search of a tertiary pediatric hospital database yielded 77 patients (age < 18 years) who underwent liver transplantation in 2007–2017. Serum creatinine levels during the 24 h before and after surgery were corrected to daily fluid balance, and the prevalence of AKI was calculated using the Pediatric RIFLE (pRIFLE), AKI Network (AKIN), and Kidney Disease Improving Global Outcomes (KDIGO) criteria. AKI occurred in 44 children (57%) according to the pRIFLE criteria (stage I, 34%; stage II, 10%, stage III, 13%) and 33 children (43%) according to the AKIN and KDIGO criteria (stage I, 20%; stage II, 10%; stage III, 13%). There was a good correlation (kappa = 0.78) among the three criteria. AKI was associated with longer duration of mechanical ventilation (5.5 ± 6.2 vs 3.6 ± 4.0 days, p < .05) and longer ICU stay (15.2 ± 8.8 vs 12.1 ± 7.5 days, p < .05). Serum creatinine normalized in all patients (mean, 0.43 ± 0.17 mg/dl) by one year. There is a good correlation among the three criteria defining AKI in pediatric liver transplant recipients. AKI is highly prevalent in this patient group and confers a worse ICU course. •AKI is common among pediatric LTx patients and ranges from 43% to 57%, depends on the classification criteria used.•There is a good correlation between KDIGO, pRIFLE and AKIN criteria when defining AKI among pediatric LTx patients.•Children with AKI had more days on mechanical ventilation and longer length of ICU stay compared to children without AKI.•post LTx children had, adjusted for age, normal creatinine values, within the year post transplantation.