Pediatric Nephrology Research Articles

Body mass index in pediatric kidney transplant selection criteria.

Obesity is associated with increased complications, rejection, and graft loss after kidney transplantation in adult and pediatric recipients. Elevated body mass index (BMI) is a common contraindication to transplant at adult kidney transplant programs; however, there is no data on such limitations for pediatric patients. Between October and December 2022, we conducted a survey of Pediatric Nephrology Research Consortium centers assessing the use of BMI in pediatric kidney transplant evaluation. Centers reporting utilization of BMI cutoffs were invited to submit patient-level data on children declined for active transplant listing due to BMI. Thirty-nine centers responded to the survey (42% response rate); 51% include BMI in their written listing criteria, with a median BMI "cutoff" of 39kg/m2 (range 30-50kg/m2). Between January 1, 2016, and December 31, 2021, 30 children at 15 transplant centers were declined for listing status due to BMI. Patient-level data was provided for 19 children (63%) who were denied active listing status; median BMI was 42kg/m2 (range 35.8-49.4kg/m2) and 84% were on dialysis. One year after evaluation, seven patients (37%) had proceeded to active wait list status. Eight (42%) remained in inactive status and four (21%) were unlisted; ten of these 12 patients (83%) were on dialysis. The use of BMI in pediatric kidney transplant evaluation and listing varies among centers, but BMI limits access to transplant for some children. More information is needed on the outcomes of obese pediatric kidney candidates who are and are not transplanted, to guide development of national and international consensus.

The Effect of Sleep Habits on Quality of Life in Pediatric Patients With Chronic Kidney Disease.

Sleep disturbance has been studied in adult patients with early and end-stage chronic kidney disease (CKD). However, there are limited publications on the pediatric patient population. This paper evaluated the association between sleep disturbances and quality of life (QoL) inpediatricpatients with CKD. The study included 22 patients and 22 healthy controls from the pediatric nephrology outpatient clinic.All participants completed the Turkish Generic Health-Related Quality of Life Questionnaire for Children and Adolescents (HRQoLQ) and the Child Sleep Habits Questionnaire(CSHQ). Patients diagnosed with CKD were compared in terms of HRQoLQ and CSHQ scores within themselves as kidney replacement therapy (KRT) recipients and non-recipients and with the control group. The mean HRQoLQ total score of the patients was 89.0 ± 12.4 and the mean CSHQ total score was 46.7 ± 5.6; there was no correlation between the total scores (p=0.599). CSHQ total and subgroup scores were similar in patients with and without KRT. The CSHQ total and subgroup median scores were not different in the patient and control groups. According to the HRQoL scale, the total QoL score and the physical and emotional well-being subscale scores were lower in patients receiving KRT than in those not receiving KRT. Sleep problems and HRQoL should not be underestimated in the pediatric CKD population, especially in patients receiving KRT. Large-scale studies with long-term outcomes are needed to understand better and improve QoL.

Controversy between biopsy and risk in children with proteinuria: is there a paradigm war?

BackgroundProteinuria is a prevalent symptom of pediatric nephrology, while kidney biopsy remains the gold standard for kidney tissue analysis, and it is currently controversial. We report the rare case that the mutation in the AMN gene was considered to cause chronically isolated proteinuria and also suggest that renal biopsy should be chosen with caution in children with chronic isolated non-nephrotic levels of proteinuria and that genetic testing may be feasible for the early precise diagnosis.Case presentationA 35-month-old boy presented with excessive urine foaming for more than half a month; his proteinuria was considered non-nephrotic range and urine protein electrophoresis was suggestive of mixed proteinuria; other than that, the investigations are non-specific. Given the child’s chronic isolated proteinuria and good renal function, we chose to refine the genetic test rather than a renal biopsy; a compound heterozygous variant was found in the AMN gene of this child which was caused by a point mutation in the father, and a partial chromosomal deletion in the mother.ConclusionsCubilin(encoded by CUBN), amnionless(encoded by AMN), and megalin form a multiligand receptor complex; CUBN or AMN gene variants have been implicated as a hereditary cause of megaloblastic anemia, proteinuria, and neurological impairment. In the past few decades, chronic isolated proteinuria caused by CUBN gene variants is benign, non-progressive, and has normal renal function. However, the child is the first reported case of isolated proteinuria of AMN gene mutation, indicating that the earlier diagnostic genetic sequencing in an otherwise well, not nephrotic proteinuria child may be a convenient, cost-effective, and harmless option, challenging the traditional paradigm.

Responding to the workforce crisis: consensus recommendations from the Second Workforce Summit of the American Society of Pediatric Nephrology

ImportancePediatric patients with complex medical problems benefit from pediatric sub-specialty care; however, a significant proportion of children live greater than 80 mi. away from pediatric sub-specialty care.ObjectiveTo identify current knowledge gaps and outline concrete next steps to make progress on issues that have persistently challenged the pediatric nephrology workforce.Evidence reviewWorkforce Summit 2.0 employed the round table format and methodology for consensus building using adapted Delphi principles. Content domains were identified via input from the ASPN Workforce Committee, the ASPN’s 2023 Strategic Plan survey, the ASPN’s Pediatric Nephrology Division Directors survey, and ongoing feedback from ASPN members. Working groups met prior to the Summit to conduct an organized literature review and establish key questions to be addressed. The Summit was held in-person in November 2023. During the Summit, work groups presented their preliminary findings, and the at-large group developed the key action statements and future directions.FindingsA holistic appraisal of the effort required to cover inpatient and outpatient sub-specialty care will help define faculty effort and time distribution. Most pediatric nephrologists practice in academic settings, so work beyond clinical care including education, research, advocacy, and administrative/service tasks may form a substantial amount of a faculty member’s time and effort. An academic relative value unit (RVU) may assist in creating a more inclusive assessment of their contributions to their academic practice. Pediatric sub-specialties, such as nephrology, contribute to the clinical mission and care of their institutions beyond their direct billable RVUs. Advocacy throughout the field of pediatrics is necessary in order for reimbursement of pediatric sub-specialist care to accurately reflect the time and effort required to address complex care needs. Flexible, individualized training pathways may improve recruitment into sub-specialty fields such as nephrology.Conclusions and relevanceThe workforce crisis facing the pediatric nephrology field is echoed throughout many pediatric sub-specialties. Efforts to improve recruitment, retention, and reimbursement are necessary to improve the care delivered to pediatric patients.Graphical A higher resolution version of the Graphical abstract is available as Supplementary information.

The Effect of Renastart Formula Supplementation in Serum Electrolytes in Children with Acute Kidney Injury

Abstract Background patients of renal disease suffer from electrolyte disturbances including hyperkalemia, hyperphosphatemia, hyponatremia, hypocalcemia and hypermagnesemia. Unfortunately, patients with kidney disease require close monitoring for serum electrolytes to maintain normal levels; with traditional nutritional interventions they may need receiving potassium and phosphorus binders Aim: This study will concentrate on the effect and tolerability of Renastart formula supplementation on correction and maintaining serum levels of electrolytes in AKI or AKI on top of CKD pediatric patients. Methods Double arm clinical convenient trial, conducted in Pediatric Nephrology Unit,Ain Shams University, Cairo, Egypt, where 24 participants who had AKI or AKI on top of CKD with hyperphosphatemia and / or hyperkalemia were recruited, the mean of age of participants was 4.1±3.15 years ranged from 0.5 to 10 years. Subjects were divided into two groups, 12 patients per group (group A received a calcium carbonate (phosphorus binder) (9 patients) with dosage 45-65 mg/kg/day orally alone or withsodium polystyrene sulfonate (potassium binder) (11 patients) with dosage 0.5-1mg/kg/day oral and group B received Renastart formula supplemented with breast milk, standard infant formula or diet), the dosage ratio was adjusted to be 1:3 (Renastart was 1/4 daily caloric intake) if serum potassium level ≥5.5 - <6 meq/L and / or serum phosphorus > 7mg/dl. But, if potassium level was ≥ 6 meq/L, the ratio was 1:1 (Renastart was ½ daily caloric intake). Readjusting dosage ratio was according to serum electrolyte levels response for treatment. Follow up of serum levels of potassium was every day until its serum level returned to normal then every month for three months and serum phosphorus was every month for three consecutive months. Results All the included subjects in both groups had high serum potassium (> 5.5 meq/L) and / or high serum phosphorus (>7 mg/dl) upon enrollment, mean serum potassium and serum phosphorus were (5.7±0.41 meq/L) (7.2±1.6 mg/dl) respectively. Regarding the decrease of serum potassium, Renastart formula supplementation in group B and potassium binders in group A resulted in equal decrease in serum potassium levels; Potassium after 3 months of management in group B was (4.22±0.28 mEq/L) (p value between 3rd month- baseline month in group B p < 0.01) and potassium binders in group A (4.48±0.27 mEq/L) (p value between 3rd month – baseline month in group A p < 0.01). Renastart formula in group B ((potassium after 1 month of management = 4.7±0.32 meq/L) (p value between 1st month – baseline month in group B p < 0.01)) had more rapid effect than potassium binders in group A during 1st month of the study ((potassium after 1 month of management =5.1±0.41 meq/L) (p value between 1st month – baseline month in group A p > 0.05). Regarding decrease in serum phosphorus, Renastart formula supplementation in group B resulted in more decrease in serum phosphorus levels though both showed significant drop in phosphorus level (( serum phosphorus after 3 months of management = 4.60±0.29 mg/dl (p value between 3rd month – baseline month in group B p = 0.002)) in comparison to that achieved by phosphorus binders in group A ((5.21±0.29mg/dl) (p value between 3rd month – baseline month in group A p = 0.01)), moreover Renastart supplementation in group B was more rapid than phosphorus binders in group A in decrease serum phosphorus (p value between 1st month and baseline in group B p = 0.002) (p value between 1st month and baseline in group A p = 0.17) Conclusion We can conclude that Renastart formula supplementation was more rapid than potassium binders in decreasing serum potassium levels in AKI pediatric patients, moreover more effective and rapid than phosphorus binders in decreasing serum phosphorus.

Assessment of Caregiver Burden, Depression and Quality of Life Levels Among Mothers of Children with Chronic Kidney Disease

Background The causes of mental health problems among mothers of children with chronic kidney diseases (CKD) associated with childcare are still not fully understood. This study, thus, has aim to assess the intensity of caregiver burden, depression and quality of life levels in mothers of children with CKD compared to a control group. It also aims to identify determinants associated with mental health outcomes. Methods: Mothers of children diagnosed with CKD who were followed up in Pediatric Nephrology outpatient clinics were included as the case group. Mothers with similar demographic characteristics were taken as the control group. During the study, 72 children with CKD, 78 control children and their mothers were enrolled. The study data was obtained using the Sociodemographic Data Form (SDF) developed by the researchers, the Zarit Burden Interview (ZBI), the Beck Depression Inventory (BDI), and The World Health Organization Quality of Life (WHOQOL-Bref). Results: The groups did differ significantly in terms of maternal psychopathology (p = .024). The most common maternal psychopathology among mothers was Major Depressive Disorder. Mothers of children with CKD had significantly higher level of caregiver burden and depression levels. Quality of life levels among mothers of children with CKD were significantly decreased compared to controls. Caregiver burden was found to predict quality of life in mothers of children with CKD (β = −0.539; p < .050). Conclusion: Caregiving burden, depression, and poor quality of life are common among mothers of children with CKD. Addressing caregiver burden may be beneficial for quality life of mothers.

Analysis of Various Laparoscopic Pediatric Urology Surgeries: Five-Year Experience of a Single Institution.

Background In this study, we aim to report our single-center experience with laparoscopic pediatric urological surgeries. We aim to determine the feasibility of various urological and urogenital laparoscopic procedures and the tricks that increase surgical success. Methodology Data from 98 patients who underwent laparoscopic urological and/or urogenital procedures for diagnostic and therapeutic purposes in our clinic between June 2018 and February 2023 were retrospectively analyzed. All surgeries were performed by the same surgical team. Laparoscopic procedures included orchidopexy, gonadectomy, vaginoplasty, hysterectomy, pyeloplasty, nephrectomy/partial nephrectomy, ureteroneocystostomy, bladder diverticulum excision, renal cyst excision, proximal ureter stone removal, oophorectomy, ovarian detorsion, oophoropexy, and lymph node excision for diagnostic purposes. The surgical planning of the patients was based on the decisions of the pediatric nephrology, pediatric endocrinology, and pediatric oncology departments and the multidisciplinary council. Demographic characteristics of the patients, surgical indications, and intraoperative data, as well as postoperative pathological diagnoses and complications, were recorded. All patients underwent a transperitoneal approach. The duration of the operation was obtained from anesthesia records and defined as the time from the beginning of the surgical incision to the closure of the skin incision. Results Of the patients, 54 were males and 44 were females. The median age was 7.8 years. No complications other than grade 1 according to the Clavien-Dindo classification were observed in our patients. As different types of surgeries were analyzed, the mean operative duration was estimated. Conclusions The laparoscopic method should be performed by surgeons experienced in advanced surgeries in pediatric urology. It is critical to consider the difference in the size of pediatric patients in preparation for laparoscopic surgery to minimize technical and ergonomic problems. We believe that each surgery has its specific tricks and that these should be a part of laparoscopy training. Moreover, developing and sharing this information would be very useful for pediatric urologists.

Secondary pseudohypoaldosteronism: a 15-year experience and a literature review.

Secondary pseudohypoaldosteronism (S-PHA) is a rare condition resulting from renal tubular resistance to aldosterone in children with urinary tract infection (UTI) and/or nephrourological malformations. It is characterized by nonspecific symptoms but with the potential for life-threatening complications. We aim to evaluate the clinical manifestations, diagnostic approach, and therapeutic interventions in children with S-PHA, along with a review of recent publications. A retrospective observational descriptive study was conducted on S-PHA cases diagnosed over the last 15years at a tertiary pediatric nephrology unit. The literature for the last 10years was reviewed. Twelve patients (10 males, 6days to 6months) were identified. Weight loss was the main reason for consultation (50%). Ninety-two percent of patients had an underlying nephrourological pathology and 62% concomitant confirmed UTI. Seven out of 12 children were admitted to the PICU. A subsequent extrapontine myelinolysis was observed in one patient as neurological sequelae. Twenty-one articles related to S-PHA have been identified on PubMed and Embase. S-PHA should be considered in infants under 6months of age with UTI and/or CAKUT. Obstructive anomalies and vesicoureteral reflux can be found, affecting both unilateral and bilateral systems. Early medical and surgical interventions are crucial and require close monitoring to avoid iatrogenic complications.

Prevalence of secondary arterial hypertension in patients with acute renal failure in a secondary-level pediatric hospital in Northwestern Mexico.

The worldwide prevalence of arterial hypertension in pediatric patients is 3.5%, and it has repercussions at renal, cardiovascular, neurological, and lifestyle levels. This study aimed to estimate the prevalence of arterial hypertension, mortality, and follow-up in patients with acute renal failure in the nephrology outpatient clinic at a second-level hospital in Northwestern Mexico. We conducted a descriptive, retrospective, and observational study. Men and women aged 1-18 years diagnosed with acute kidney injury were analyzed from January 1, 2012, to December 31, 2021. The medical and electronic records of the candidate patients were analyzed, and nutritional data, laboratory analysis, most frequent etiology, and follow-up in the pediatric nephrology clinic were collected. Those with exacerbated chronic kidney disease and previous diagnosis of high blood pressure were excluded. One hundred and seventy-four patients were evaluated, and only 40 were eligible for the study (22.98%), predominantly males with a mean age of 9.9 years. The degree of arterial hypertension was 50% for grade I and 50% for grade II (p = 0.007); the mortality rate was 32%. One hundred percent of hypertension cases were controlled at 6 months after discharge (p = 0.000080). Our results were similar to those reported in other studies. Follow-up and early detection of arterial hypertension in children need to be strengthened.

Sleep Duration and Blood Pressure in Youth Referred for Elevated Blood Pressure Evaluation.

Sleep promotion is not specifically recommended as a target for hypertension management. We examined associations of sleep duration and timing with blood pressure parameters in patients referred to pediatric nephrology clinic for elevated blood pressure evaluation. This is a retrospective study of initial ambulatory blood pressure monitoring data and self-report sleep data collected from patients referred to nephrology clinic for the evaluation of elevated blood pressure. Linear and logistic regression modeling determined associations between sleep exposures (duration and timing) and continuous and dichotomous blood pressure outcomes, respectively, adjusted for age, sex, body mass index, and weekday versus weekend status. The study sample included 539 patients with mean age 14.6 years and 56% meeting hypertension criteria. Sleep duration averaged 9.1 hours per night. Average timing of sleep onset and offset were 11:06 pm and 8:18 am, respectively. Longer sleep duration was associated with better daytime blood pressure parameters (eg, every extra hour of sleep duration was associated with a reduced odds of wake hypertension [odds ratio, 0.88; 95% CI, 0.79-0.99]). Later sleep onset was associated with worse daytime blood pressure parameters (eg, each additional hour of later sleep onset was associated with higher wake systolic blood pressure index [mean wake blood pressure/95th percentile]) (β = 0.07; 95% CI, 0.02-0.13). Associations were consistent across sex, age, body mass index, and weekday status. Longer sleep duration and earlier sleep onset were associated with lower blood pressure. This suggests that sleep optimization may be an important target for intervention in hypertension management.

Pediatric Kidney Biopsy: Clinical Perspectives Based on Survey of Pediatric Nephrologists and Interventional Radiologists

Pediatric Kidney Biopsy: Clinical Perspectives Based on Survey of Pediatric Nephrologists and Interventional Radiologists

Management of Urinary Tract Infections and Vesicoureteric Reflux: Key Updates from Revised Indian Society of Pediatric Nephrology Guidelines 2023.

Non-specific symptoms and difficulty in collecting urine specimens make diagnosis of urinary tract infection (UTI) challenging in young children. However, timely diagnosis and initiation of therapy are essential to prevent complications. Children with recurrent UTIs require detailed evaluation and follow-up for optimal management. We report key updates from revised evidence-based practice guidelines of the Indian Society of Pediatric Nephrology for urinary tract infections and primary vesicoureteric reflux.

Creatinine-based formulas are not ideal to estimate glomerular filtration rate in selected pediatric patients: data from a tertiary pediatric nephrology center.

Evaluating glomerular filtration rate (GFR) remains challenging in pediatrics; new formulas were developed to increase performance of GFR estimation (eGFR). We aimed to evaluate the recently published formulas as applied to another pediatric population. A retrospective study was conducted in a cohort of 307 patients with a "kidney risk" (mean age 12.1 ± 4.5 years, sex ratio 1/1) assessed in a tertiary pediatric nephrology center and a mean measured GFR (mGFR) using plasma iohexol clearance of 85.5 ± 25.3 mL/min/1.73 m2; creatinine levels were measured by IDMS-standardized enzymatic method and cystatin C by immunonephelometry. The following eGFRs were calculated: Schwartz2009, Schwartz-Lyon, CKiDU25creat, and EKFC for eGFR using creatinine (eGFR-creat), CKiDU25cys and FAScys for eGFR using cystatin (eGFR-cys) as well as combined SchwartzCreat-Cys, average (CKiDU25creat-CKiDU25cys), and average (EKFC-FAScys) for eGFR using both biomarkers. The performance of the different formulas was evaluated compared to mGFR by absolute bias measurement and accuracy (p10%, p30%). Results are expressed as mean ± SD. Creatinine-based formulas and especially the new CKiDU25 and EKFC overestimate GFR, even in children with normal kidney function. However, the bias is constant with these two formulas whatever the age group or gender, contrary to the previously published formulas. In contrast, cystatin C-based equations and combined formulas showed good performance in all age groups and all medical conditions with an acceptable bias and p30%. In our pediatric population, the performance of all creatinine-based formulas is inadequate with significant GFR overestimation, mainly in subjects with mGFR > 75 mL/min/1.73 m2. Conversely, cystatin C-based or combined formulas have acceptable performance in patients followed in a tertiary pediatric nephrology unit.

АТИПИЧНЫЙ ГЕМОЛИТИКО-УРЕМИЧЕСКИЙ СИНДРОМ У ДЕТЕЙ В РЕСПУБЛИКЕ БЕЛАРУСЬ: ПЕРВЫЙ ОПЫТ КОМПЛЕМЕНТБЛОКИРУЮЩЕЙ ТЕРАПИИ

Atypical hemolytic uremic syndrome (aHUS) is one of the most complex and severe forms of thrombotic microangiopathy with an unfavorable outcome in its natural course. The purpose of this research was to study the epidemiology, demographics, course and outcomes of aHUS in comparison with patients with typical hemolytic uremic syndrome (tHUS) as well as to conduct a personal analysis of all cases of aHUS and evaluate the response to therapy, including comple-ment-blocking with Eculizumab. Materials and methods used: the study was longitudinal, obser-vational and included all 349 cases of HUS in children in Belarus in 2015-2023. A comparative analysis of demographic and clinical data, laboratory results and outcomes was performed in pa-tients with HUS hospitalized at the Center for Pediatric Nephrology and Renal Replacement Therapy of the Republic of Belarus (Minsk, Belarus). Results: of 349 HUS cases, the proportion of aHUS was 3,7% (13). Children with aHUS were older (4,6 (1,6; 6,7) v. 2,7 (1,7; 4,8) y/o with tHUS) due to the portion of patients older than 5 y/o (46%). In aHUS, severe central nervous system disorders were more often observed (62% v. 14%, p=0,002), the development of critical conditions requiring transfer to mechanical ventilation (39% v. 9%, p=0,006), prolonged anuria with the need for dialysis therapy in 100% of cases and severe adverse outcomes (death or end-stage renal disease) during natural course (66,0% v. 2,7%, p&lt;0,001). The most common trigger for aHUS was acute intestinal infections in 54% of cases. A genetic study was performed in 9 (69%) children with aHUS and in 8 (89%) of them the CFHR3/CFHR1 deletions were detected: in 4 cases (3 hetero- and a single homozygous) and heterozygous pathogenic and probably patho-genic gene mutations, CFHR5 in 2 cases, CFH in a single case and combined CD46 and CFI in a single case as well. Antibodies to complement factor H were detected in 5 patients, 3 of which had a CFHR3/CFHR1 deletion. Therapy with a biosimilar of the original Eculizumab showed its effectiveness in aHUS: in 3 (100%) children it was possible to prevent the return of the disease in the kidney graft, in single patient who did not receive prophylaxis it was able to block the recur-rence of aHUS in the transplanted kidney while preserving its function, in 3 (100%) patients with late administration of treatment the urine output was restored after prolonged anuria (24, 33 and 59 days); in a single child with the antibody variant of aHUS, with early administration of the drug, renal function was preserved. No complications of complement blocking therapy were not-ed. Conclusions: the introduction into clinical practice of a biosimilar of the original Eculizumab in children in Belarus has significantly improved the outcomes and prognosis of the disease.

Prevalence, Associated Factors, and Outcomes of Severe Acute Kidney Injury in Pediatric Acute Liver Failure: Single-Center Retrospective Study, 2003-2017.

Our aim was to determine the prevalence and explanatory factors associated with outcomes in children with acute liver failure (ALF) admitted to the PICU, who also develop severe acute kidney injury (AKI). Retrospective cohort, 2003 to 2017. Sixteen-bed PICU in a university-affiliated tertiary care hospital. Admissions to the PICU with ALF underwent data review of the first week and at least 90-day follow-up. Patients with stages 2-3 AKI using the British Association of pediatric Nephrology definitions, or receiving continuous renal replacement therapy (CRRT) for renal indications, were defined as severe AKI. We excluded ALF cases on CRRT for hepatic-only indications. None. Baseline characteristics, proportion with severe AKI, illness severity and interventions, and outcomes (i.e., transplant, survival with native liver, overall survival, duration of PICU stay, and mechanical ventilation). Ninety-four children with ALF admitted to the PICU were included. Over the first week, 29 had severe AKI, and another eight received CRRT for renal/mixed reno-hepatic indications; hence, the total severe AKI cohort was 37 of 94 (39.4%). In a multivariable logistic regression model, peak aspartate aminotransferase (AST) and requirement for inotropes on arrival were associated with severe AKI. Severe AKI was associated with longer PICU stay and duration of ventilation, and lower spontaneous survival with native liver. In another model, severe AKI was associated with greater odds of mortality (odds ratio 7.34 [95% CI, 1.90-28.28], p = 0.004). After 90 days, 3 of 17 survivors of severe AKI had serum creatinine greater than the upper limit of normal for age. Many children with ALF in the PICU develop severe AKI. Severe AKI is associated with the timecourse of PICU admission and outcome, including survival with native liver. Future work should look at ALF goal directed renoprotective strategies at the time of presentation.

Probiotic use in pediatric kidney transplant recipients: What are current practices, and are they evidence-based? A pediatric nephrology research consortium study.

Probiotics are living microorganisms that may confer health benefits to their host if administered in sufficient quantities. However, data on the use of probiotics in transplant recipients are scarce. This multi-center survey of pediatric nephrologists aimed to examine variations in practice regarding the use of probiotics in pediatric kidney transplant recipients. The survey was conducted via a 10-item questionnaire (developed in Survey Monkey) administered to pediatric nephrologists participating in the Pediatric Nephrology Research Consortium meeting in April 2023. Sixty-four pediatric nephrologists completed the survey. Twenty-seven (42.2%) respondents reported having prescribed probiotics to pediatric kidney transplant recipients. The primary reason for probiotic use was the treatment of antibiotic-associated diarrhea (n = 20), with other reasons including recurrent Clostridium difficile infection (n = 15), general gut health promotion (n = 12), recurrent urinary tract infections (n = 8), and parental request (n = 1). Of those who prescribed probiotics, 48.1% held them during periods of neutropenia and 14.8% during central venous line use. Of the 64 respondents, 20 reported the lack of safety data as a concern for using probiotics in kidney transplant recipients. Pediatric nephrologists are increasingly prescribing probiotics to pediatric kidney transplant recipients; nevertheless, substantial practice variations exist. The paucity of safety data is a significant deterrent to probiotic use in this population.

The extent of kidney involvement in paediatric tuberous sclerosis complex

BackgroundTuberous sclerosis (TSC)–associated kidney disease is a leading cause of mortality in adults with TSC. This study aimed to understand TSC features in children, particularly kidney involvement, to inform clinical care for this specific group.MethodsThis retrospective cohort study included all paediatric (< 19 years) TSC cases at a large tertiary paediatric nephrology centre. Relevant data were collected from patients’ records, statistical analyses were performed to identify associations between variables, survival probabilities were estimated with Kaplan‒Meier curves, and log-rank tests were conducted to assess survival differences among genetic mutations.ResultsA total of 182 children with TSC were included. Among the 145 children with available kidney imaging data, 78.6% (114/145) exhibited kidney lesions. Angiomyolipomas (AMLs) were significantly more prevalent in the TSC2 mutation group (p = 0.018). Children with TSC2 mutations generally had poorer lesion-free survival than those with TSC1 mutations, but this difference was only significant for AMLs (p = 0.030). The change in size of largest AMLs increased with age and doubled in children above 9 years; a similar pattern was observed when stratified by genetic mutation. In contrast, kidney cysts exhibited two peaks: one in children under 5 years (2.31 mm/year) and the second in children between 15–19 years (2.82 mm/year). Chronic kidney disease was observed in 12.3% (10/81) of children, and high-risk AMLs above 3 cm were observed in 9% (13/145).ConclusionsWhile TSC kidney disease emerges later in the disease course than neurological features, our findings emphasise the importance of kidney surveillance during childhood, including routine kidney imaging, kidney function, and blood pressure monitoring.Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information

Clippings: Pediatric Nephrology

Clippings: Pediatric Nephrology

The Management of Dietary Fiber Intake in Children With Chronic Kidney Disease – Clinical Practice Recommendations From the Pediatric Renal Nutrition Taskforce

The benefits of dietary fiber are widely accepted. Nevertheless, a substantial proportion of children fail to meet the recommended intake of dietary fiber. Achieving adequate fiber intake is especially challenging in children with chronic kidney disease (CKD). An international team of pediatric renal dietitians and pediatric nephrologists from The Pediatric Renal Nutrition Taskforce (PRNT) has developed clinical practice recommendations (CPRs) for the dietary intake of fiber in children and adolescents with CKD. In this CPR paper, we propose a definition of fiber, provide advice on the requirements and assessment of fiber intake, and offer practical guidance on optimizing dietary fiber intake in children with CKD. In addition, given the paucity of available evidence and to achieve consensus from international experts, a Delphi survey was performed in which all the clinical practice recommendations were reviewed.

Improving Transition of Care for Pediatric Patients With Chronic Kidney Disease: A Pilot Project.

Introduction Transition is the process of preparing an adolescent or young adult for the adult model of care. Poor transitions have been linked to increased medical utilization and poorer kidney outcomes. There are limited studies evaluating predictors of transition readiness or interventions in pediatric patients with chronic kidney disease (CKD). Methods We enrolled 42 non-dialysis, non-transplant patients with CKD stage 2 or higher and 14 years and older receiving care in our pediatric nephrology clinic. Data collected included demographics, clinical information, and transition readiness as measured by the Transition Readiness Assessment Questionnaire (TRAQ). Patients were provided with a structured, remote curriculum with resources that addressed areas of need. Patients were followed every three to six months. Repeat TRAQ questionnaires were administered six months after enrollment. Results Our study found that younger age and male gender were risk factors for poor transition. Age was consistently a positive predictor of higher TRAQ scores in the medication, appointment, and total score domains (p < 0.05). Male gender was a risk factor for lower TRAQ scores in the appointment and communication domains (p < 0.05). Additionally, our curriculum was effective at improving scores across all TRAQ domains, with an average increase of about 25% in six months. There was no difference in patients who had a three-month follow-up as compared to a six-month follow-up (p > 0.05). Conclusion Our study finds that younger age and male gender are risk factors for poor transition. Additionally, a structured, remote curriculum is effective at improving transition readiness.