Pediatric Nephrology Research Articles

The Potential of Vitamin-D-Binding Protein as a Urinary Biomarker to Distinguish Steroid-Resistant from Steroid-Sensitive Idiopathic Nephrotic Syndrome in Iraqi Children

Objective: To determine the ability of uVDBP to discern SRNS from steroid-sensitive nephrotic syndrome (SSNS) in Iraqi children. Materials and Methods: This cross-sectional study enrolled children with SRNS (n=31) and SSNS (n=32) from the pediatric nephrology clinic of Babylon Hospital for Maternity and Pediatrics over three months. Patients' characteristics in terms of demographics, clinical data, and urinary investigations were collected. Quantitative analysis of uVDBP levels was undertaken via a commercially available ELISA kit. Results: The median uVDBP values were significantly higher (p-value<0.001) in the SRNS group (median=10.26, IQR=5.91 μg/mL) than in the SSNS group (median=0.953, IQR=4.12 μg/mL). A negative correlation was noted between uVDBP levels and estimated glomerular filtration rate (eGFR) (Spearman's rho coefficient= − 0.494, p=0.001). Nevertheless, the rise in uVDBP concentrations was still considerable in children with SRNS whose eGFR measurements were above 60 mL/min/1.73 m2. The study revealed a good discriminatory power for uVDBP as a predicting parameter to distinguish SRNS from SSNS (AUC= 0.909, p<0.0001. The optimal uVDBP cut-off value of 5.781 μg/mL was associated with a sensitivity of 0.839 and specificity of 0.844 to differentiate SRNS from SSNS. Conclusion: Considering its significant discriminatory strength, uVDBP can be considered as a potential marker to noninvasively distinguish children with SRNS from those with SSNS.

MP70-12 THE WIDE URETER AND THE SMALL BLADDER, THE CUFF-NIPPLE REIMPLANTATION RATHER THAN URINARY DIVERSION

You have accessJournal of UrologyCME1 Apr 2023MP70-12 THE WIDE URETER AND THE SMALL BLADDER, THE CUFF-NIPPLE REIMPLANTATION RATHER THAN URINARY DIVERSION Moneer Hanna and Wael Abosena Moneer HannaMoneer Hanna More articles by this author and Wael AbosenaWael Abosena More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003338.12AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Reimplantation of a dilated Ureter into an infantile small capacity bladder represents a surgical challenge. The surgical options for reflux megaureters who developed breakthrough urinary infections while on prophylaxis are temporary diversion (vesicostomy for bilateral and ureterostomy for unilateral megaureters). In cases of obstructed megaureter the options are ureterostomy and refluxing reimplantation. METHODS: Between 1995 and 2021, 46 infants aged 3 and 12 months were referred for surgical management following recurrent pyelonephritis while on prophylaxis (30 infants) and for worsening hydronephrosis (16 infants). There were 32 boys and 14 girls. The diagnosis was bilateral reflux megaureters in 18 and unilateral in 8 children. The other twenty children had bilateral obstructed megaureter in 9 and unilateral in 11 children. The radionuclide renal scan was performed in 43 children, and it showed reflux nephropathy in 35 kidneys with VUR and reduced function of more than 20% in 9/11 obstructed kidneys. A total of 73 megaureters were operated on. The ureteral tortuosity was straightened, and the caliber was reduced when indicated. Each ureter was reimplanted in a short (1 cm) submucosal tunnel, a cuff-nipple ureteral orifice was created in 62/73 ureters. Simple cuff-nipple without submucosal tunnelling in 11 ureters. RESULTS: The postoperative course was uneventful in all children and follow up US & VCUG were done at four months. Thereafter the follow-up continued by the pediatric nephrology service. The post operative US showed improved hydronephrosis in 59/73 kidneys and stable in 14 units. The VCUG showed no evidence of VUR in 58/62 (93%) of the group who had short tunnel and cuff-nipple reimplantation, in 4 ureters the VUR was downgraded to grade II. 10/11 (91%) of the ureters with the cuff-nipple without tunneling showed no evidence of VUR, and one ureter the VUR was grade II. 7 children had significant reflux nephropathy outgrew their renal function and underwent renal transplantation CONCLUSIONS: The time -tested Paquin dogma of tunnel length/width ratio of 5/1 was challenged by Lyons et.al, the latter contention was that the shape of the ureteral orifice (UO) is more important than the length of the tunnel for reflux prevention, (the cuff-nipple creates a volcano like UO). Recently Villaneuva et.al performed a parametric simulation study of ureteral collapse (LS-DYNA finite-element software). They confirmed Lyons observation. These observations would explain the high success rate which was achieved by the cuff-nipple technique. Source of Funding: none © 2023 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 209Issue Supplement 4April 2023Page: e1008 Advertisement Copyright & Permissions© 2023 by American Urological Association Education and Research, Inc.MetricsAuthor Information Moneer Hanna More articles by this author Wael Abosena More articles by this author Expand All Advertisement PDF downloadLoading ...

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Editorial introduction

Risk determination for upper urinary tract damage in children with neuropathic bladder.

Neuropathic bladder dysfunction (NBD) or neurogenic lower urinary tract dysfunction occurs as a result of a lesion at any level of central nervous system. The most common aetiology of NBD in children is abnormal development of spinal column. These defects cause neurogenic detrusor overactivity which contributes to detrusor-sphincter dysfunction and results in lower urinary tract symptoms such as incontinence. One of the more progressive and insidious, at the same time preventable result of neuropathic bladder is upper urinary tract deterioration. It is vital to aim reduction in bladder pressures and the minimisation of urine stasis in order to prevent or at least attenuate renal disease. Despite world-wide strategies for prevention of neural tube defects currently, we will still be involved in the care of spina bifida patients born every year with a neuropathic bladder and at risk of long-term renal damage. This study was planned for evaluation of results and detection of possible risk factors for upper urinary tract deterioration during routine visits of neuropathic bladder population. The electronic medical records of the patients who were followed up for at least 1 year with the diagnosis of neuropathic bladder in Pediatric Urology and Nephrology units of Adana City Training and Research Hospital were reviewed retrospectively. A total of 117 patients, whose blood, urine, imaging and urodynamic study required for the evaluation of nephrological and urological status were completed and included in the study. Patients under the age of one were excluded from the study. Demographic characteristics, medical history, laboratory and imaging results were recorded. All statistical analyses were analysed by SPSS version 21 software package and analysed by descriptive statistical. Of the 117 patients who participated in the study, 73 (62.4%) were female and 44 (37.6%) were male. Mean age of the patients was 6.7 ± 4.9 years. The leading cause of neuropathic bladder was neuro-spinal dysraphism with 103 (88.1%) patients. Urinary tract ultrasound imaging revealed hydronephrosis in 44 patients (35.9%), parenchymal thinning in 20 patients (17.1%), increased parenchymal echo in 20 patients (17.1%) and trabeculation or increase in wall thickness of the bladder in 51 patients (43.6%). Voiding cystogram revealed the presence of vesicoureteral reflux in a total of 37 patients (31.6%), with 28 patients being unilateral and 9 patients bilateral. More than half of the patients had abnormal bladder findings (52.1%). In the Tc 99 m DMSA scan of the patients, 24 patients (20.5%) had unilateral renal scars and 15 (12.8%) had bilateral. Loss of renal function was detected in 27 (23.1%) of the patients. Urodynamic study revealed decreased bladder capacity in 65 patients (55.6%) and increased detrusor leakage pressure was found in 60 patients (51.3%). The mean leak point pressure of the patients was 36 ± 26 cm H2 O, and the mean leakage volume was determined as 157 ± 118 mL. Findings obtained from imaging and urodynamic studies during routine investigation of neuropathic bladder patients can be a guide for the upper urinary tract. According to our results, age, bladder changes in ultrasound and voiding cystogram, high leak point pressure obtained during urodynamic studies are thought to have strong correlation with upper urinary tract damage. The prevalence of progressive chronic kidney disease in children and adults with spina bifida is remarkable and completely preventable. The strategies for prevention of renal disease in this patient population should be planned by coordinated work of urologist with nephrologist and requires family cooperation.

Assessment of Hypertension in Children with Autosomal Dominant Polycystic Kidney Disease; Single-Center Experience

Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common hereditary renal cystic diseases. Althoughits clinical manifestations usually occur in adulthood, hypertension (HT) is known to develop in most patients before the decline inrenal function and it is associated with faster progression to end stage kidney disease (ESKD). We investigated ambulatory bloodpressure monitoring (ABPM) results of 23 patients with ADPKD, followed up in the Pediatric Nephrology Clinic of Ondokuz MayısUniversity Medical Faculty Hospital. Patients’ demographic characteristics, laboratory and ultrasonography (US) results, officeblood pressure, and ABPM measurements were evaluated. The parameters of gender, age, increased kidney size, proteinuria,glomerular filtration rate (GFR) was compared in hypertensive and non-hypertensive group. Twenty three patients (13 girls, tenboys) with a mean age of 11.94±4.01 (min-max: 4.6-18) years and a female/male ratio of 1.3/1 were examined. Ultrasound revealedincreased kidney sizes in 12 patients (52.2%) and multiple cysts in the bilateral kidneys in 20 patients (87%). Mild to moderateproteinuria was detected in 7 patients (30.4%). The HT ratio of patients was 52.2% and 39.1% when assessed with office bloodpressure (BP) measurement and ABPM respectively. A non-dipper pattern was established in 14 patients (60.9%). Gender, age,increased kidney size, proteinuria, GFR did not differ significantly between ADPKD patients with and without ambulatory HT. Thisstudy shows that nearly half of children with ADPKD have HT by ABPM. BP should be regularly screened by ABPM in all pediatricADPKD patients.

Clinical and Biochemical Profile of Children with Chronic Kidney Disease in a Tertiary Care Hospital of Bangladesh

Background: Chronic kidney disease (CKD) is an insidious and irreversible condition that eventually progresses to end stage renal failure and important cause of morbidity and mortality in children worldwide. However, the care of these patients represents a challenge for health care providers, which must pay attention not only to renal disease, but also to the various extra renal manifestations that affect growth and development. Evaluation of clinical and biochemical profile of children with chronic kidney disease can help to meet up this challenge. Methods: This cross-sectional observational cohort study evaluated clinical, anthropometric and biochemical variables of children with chronic kidney disease aged 2 to 16 years in the department of Pediatric Nephrology, Dhaka Shishu (Children) Hospital, Bangladesh, during July’ 2018 to December’ 2018. A total of thirty-six children with chronic kidney disease with creatinine clearance <60ml/min/1.73 m2 and on supportive treatment and haemodialysis were included. In control group, equal number of age and sex matched healthy children without any preexisting chronic diseases were included. Both study group and control group were assessed for sociodemographic data, nutritional status, clinical and biochemical parameters. Results: The mean age was 9.09±3.01 years in case group and 7.85±3.69 years in control group. In case group, 22 patients (61.1%) were male and 14 (38.9%) were female and the ratio was 1.5:1. In this study we found that a statistically significant decrease in Z-score for (Weight, Height and Body Mass Index) in the CKD patients’ group than control. In terms of blood pressure, 66.7% of patients in the cases group were hypertensive (P = 0.001). There was no significant difference of hypertension among CKD stages in this study. CKD Patients were found anemic with significant decrease in hemoglobin level. Anemia found in 35 patients (97.2%) in the case group than control (P=0.001). CKD stage 5 children had significantly lower hemoglobin level. Serum phosphate and potassium were significantly higher while serum calcium was significantly lower and parathyroid level was higher in case group than control. Conclusion: The present study stated that CKD patients had significantly lower BMI and hemoglobin but hypertensive in comparison to case group. Serum potassium, phosphate and parathyroid higher but serum calcium level was lower in case group. J Bangladesh Coll Phys Surg 2023; 41: 120-125

Renal vein thrombosis in neonates: a case series of diagnosis, treatment and childhood kidney function follow-up.

Neonatal renal vein thrombosis (NRVT) is a rare condition with little data available. We retrospectively analyzed newborns diagnosed with NRVT admitted to3 pediatric nephrology units in Paris from 2005 to 2020. Twenty-seven patients were analyzed (male = 59%). The median age at diagnosis was2.5days (1 - 4.5). Diagnosis was suspected based on at least one of the three cardinal signs of renal vein thrombosis in 93%: flank mass (67%), hematuria(67%) and thrombocytopenia (70%). In all patients, diagnosis was confirmed by ultrasound. All patients had at least one known perinatal risk factor. A prothrombotic risk factor was found in 13 patients (48%). NRVT was unilateral in 70%, involving the left renal vein in 58%. Among 25 treated patients, 19 (76%) received low molecular weight heparin (LMWH) as initial therapy, 2 (8%) received unfractionated heparin and4 (16%) received fibrinolysis. Median duration of treatment was 8weeks (4 - 12). Bleeding occurred significantly more often with fibrinolysis than with LMWH/supportive therapy (3 of 4: 75% vs 0 of 4: 0%, p = 0.05). Clot resolution in patients treated with fibrinolysis did not differ significantly from those treated with LMWH/supportive therapy. After a median follow-up of 5.7years (3years-9.9years), pathological kidney features were observed in 73% of the patients (19 of 26), kidney atrophy in 18 (69%), hypertension in 2 (8%), chronic kidney disease(CKD) in 1 (4%) and proteinuria in2(8%). NRVT remains a challenging condition, which still requires further study because of its associated morbidity. A higher resolution version of the Graphical abstract is available as Supplementary information.

Nutritional management of children with acute kidney injury-clinical practice recommendations from the Pediatric Renal Nutrition Taskforce.

The nutritional management of children with acute kidney injury (AKI) is complex. The dynamic nature of AKI necessitates frequent nutritional assessments and adjustments in management. Dietitians providing medical nutrition therapies to this patient population must consider the interaction of medical treatments and AKI status to effectively support both the nutrition status of patients with AKI as well as limit adverse metabolic derangements associated with inappropriately prescribed nutrition support. The Pediatric Renal Nutrition Taskforce (PRNT), an international team of pediatric renal dietitians and pediatric nephrologists, has developed clinical practice recommendations (CPR) for the nutritional management of children with AKI. We address the need for intensive collaboration between dietitians and physicians so that nutritional management is optimized in line with AKI medical treatments. We focus on key challenges faced by dietitians regarding nutrition assessment. Furthermore, we address how nutrition support should be provided to children with AKI while taking into account the effect of various medical treatment modalities of AKI on nutritional needs. Given the poor quality of evidence available, a Delphi survey was conducted to seek consensus from international experts. Statements with a low grade or those that are opinion-based must be carefully considered and adapted to individual patient needs, based on the clinical judgment of the treating physician and dietitian. Research recommendations are provided. CPRs will be regularly audited and updated by the PRNT.

Vesicoureteral Reflux and Innate Immune System: Physiology, Physiopathology, and Clinical Aspects.

Vesicoureteral reflux represents one of the most concerning topics in pediatric nephrology due to its frequency, clinical expression with the potential to evolve into chronic kidney disease, and last but not least, its socio-economic implications. The presence of vesicoureteral reflux, the occurrence of urinary tract infections, and the development of reflux nephropathy, hypertension, chronic kidney disease, and finally, end-stage renal disease represent a progressive spectrum of a single physiopathological condition. For the proper management of these patients with the best clinical outcomes, and in an attempt to prevent the spread of uropathogens' resistance to antibacterial therapy, we must better understand the physiopathology of urinary tract infections in patients with vesicoureteral reflux, and at the same time, we should acknowledge the implication and response of the innate immune system in this progressive pathological condition. The present paper focuses on theoretical aspects regarding the physiopathology of vesicoureteral reflux and the interconditionality between urinary tract infections and the innate immune system. In addition, we detailed aspects regarding cytokines, interleukins, antimicrobial peptides, and proteins involved in the innate immune response as well as their implications in the physiopathology of reflux nephropathy. New directions of study should focus on using these innate immune system effectors as diagnostic and therapeutic tools in renal pathology.

Etiology and outcomes of acute kidney disease in children: a cohort study

There is paucity of information regarding the etiology and outcomes of Acute Kidney Disease (AKD) in children. The objectives of this cohort study were to evaluate the etiology and outcomes of AKD; and analyze predictors of kidney survival (defined as free of CKD 2, 3a, 3b, 4 or 5). Patients aged 1month to 18years who developed AKD over a 4-year-period (January 2018-December 2021) were enrolled. Survivors were followed-up at the pediatric nephrology clinic, and screened for residual kidney injury. Among 5710 children who developed AKI, 200 who developed AKD were enrolled. The median (IQR) eGFR was 17.03 (10.98, 28) mL/min/1.73 m2. Acute glomerulonephritis, acute tubular necrosis (ATN), hemolytic uremic syndrome (HUS), sepsis-associated AKD, and snake envenomation comprised of 69 (34.5%), 39 (19.5%), 24 (12%), 23 (11.5%) and 15 (7.5%) of the patients respectively. Overall, 88 (44%) children required kidney replacement therapy (KRT). There were 37 (18.5%) deaths within the AKD period. At a follow-up of 90days, 32 (16%) progressed to chronic kidney disease stage-G2 or greater. At a median (IQR) follow-up of 24 (6, 36.5) months (n = 154), 27 (17.5%) had subnormal eGFR, and 20 (12.9%) had persistent proteinuria and/or hypertension. Requirement of KRT predicted kidney survival (free of CKD 2, 3a, 3b, 4 or 5) in AKD (HR 6.7, 95% CI 1.2, 46.4) (p 0.04). Acute glomerulonephritis, ATN, HUS, sepsis-associated AKD and snake envenomation were common causes of AKD. Mortality in AKD was 18.5%, and 16% progressed to CKD-G2 or greater at 90-day follow-up.

Illness-related parental stress and quality of life in children with kidney diseases.

This cross-sectional study investigated quality of life (QoL) and illness-related parental stress in children with kidney diseases by (1) comparing mean levels of these two variables between several kidney disease categories; (2) exploring correlations between QoL and parental stress; and (3) describing which disease category reports lowest QoL and highest parental stress. We included 295 patients with a kidney disease (0-18years) and their parents, followed at 6 reference centers for pediatric nephrology. Children's QoL was assessed by the PedsQL™ 4.0 Generic Core Scales, and illness-related stress by the Pediatric Inventory for Parents. All patients were divided into 5 kidney disease categories according to the multidisciplinary care program criteria prescribed by the Belgian authorities: (1) structural kidney diseases, (2) tubulopathies and metabolic diseases, (3) nephrotic syndrome, (4) acquired diseases with proteinuria and hypertension, and (5) kidney transplantation. Child self-reports showed no differences in QoL between kidney disease categories, in contrast to parent proxy reports. Parents of transplant patients reported lower QoL in their child and more parental stress compared with the 4 non-transplant categories. QoL and parental stress were negatively correlated. Lowest QoL and highest parental stress scores were mainly found in transplant patients. This study showed lower QoL and higher parental stress in pediatric transplant patients compared with non-transplants, based on parent reports. Higher parental stress is associated with worse QoL in the child. These results highlight the importance of multidisciplinary care for children with kidney diseases, with special attention to transplant patients and their parents. A higher resolution version of the Graphical abstract is available as Supplementary information.

Serum levels and significance of soluble B-cell maturation antigen in childhood-onset systemic lupus erythematosus with renal involvement.

The aim of this study was to investigate serum levels of soluble B-cell maturation antigen (sBCMA) in childhood-onset systemic lupus erythematous (cSLE) patients with renal involvement, and to elucidate their association with clinical characteristics. 116 cases of cSLE patients with renal involvement (84 females and 32 males; median age 11.6 (10.1, 12.9) years) hospitalized in Department of Pediatric Nephrology and Rheumatology, the First Affiliated Hospital, Sun Yat-sen University and 31 healthy controls (HCs) were enrolled. Serum concentrations of sBCMA were determined using enzyme-linked immunosorbent assay (ELISA). Clinical and laboratory information of cSLE patients were retrospectively analyzed. Serum sBCMA levels were significantly increased in primary cSLE when compared with treated cSLE patients and HCs, whereas there was no significant difference between treated cSLE patients and HCs. Patients with high disease activity displayed higher serum sBCMA levels compared with those with no or mild to moderate disease activity. Positive correlation was observed between serum sBCMA levels and systemic lupus erythematosus disease activity index-2K (SLEDAI-2K), antinuclear antibody titers, anti-double-stranded DNA titers, erythrocyte sedimentation rate, and immunoglobulin G levels, while sBCMA levels were negatively correlated with blood white blood cell count, hemoglobin, platelet count, complement C3 and C4 levels. Serum sBCMA levels decreased as disease ameliorated after treatments among 11 cases with follow-up examinations. In cSLE patients with renal involvement, serum sBCMA levels correlated significantly with disease activity, immunological, and hematological parameters, but not with renal parameters. Our results suggest the potential and significance of serum sBCMA as a biomarker in cSLE patients.

Donor specific antibody surveillance among pediatric kidney transplant programs: A report from the improving renal outcome collaborative.

Kidney transplantation (KT) is the preferred treatment for children with end-stage kidney disease. Recent advances in immunosuppression and advances in donor specific antibody (DSA) testing have resulted in prolonged allograft survival; however, standardized approaches for surveillance DSA monitoring and management of de novo (dn) DSA are widely variable among pediatric KT programs. Pediatric transplant nephrologists in the multi-center Improving Renal Outcomes Collaborative (IROC) participated in a voluntary, web-based survey between 2019 and 2020. Centers provided information pertaining to frequency and timing of routine DSA surveillance and theoretical management of dnDSA development in the setting of stable graft function. 29/30 IROC centers responded to the survey. Among the participating centers, screening for DSA occurs, on average, every 3 months for the first 12months post-transplant. Antibody mean fluorescent intensity and trend most frequently directed changes in patient management. Increased creatinine above baseline was reported by all centers as an indication for DSA assessment outside of routine surveillance testing. 24/29 centers would continue to monitor DSA and/or intensify immunosuppression after detection of antibodies in the setting of stable graft function. In addition to enhanced monitoring, 10/29 centers reported performing an allograft biopsy upon detection of dnDSA, even in the setting of stable graft function. This descriptive report is the largest reported survey of pediatric transplant nephrologist practice patterns on this topic and provides a reference for monitoring dnDSA in the pediatric kidney transplant population.

A survey on nutritional management of critically ill children on continuous renal replacement therapy

We conducted a survey of pediatric nephrologists to examine the knowledge and current practices of and identify challenges in the nutritional management of critically ill children during continuous renal replacement therapy (CRRT). Although it is known that there is a significant effect on nutrition during CRRT, there seems to be a lack of knowledge as well as variability in the practices of nutritional management in these patients, as indicated by our survey results. The heterogeneity of our survey results highlights the need to establish clinical practice guidelines and develop consensus around optimal nutritional management in pediatric patients requiring CRRT. The results as well as the known effects of CRRT on metabolism should be considered during the development of guidelines in critically ill children on CRRT. Our survey findings also highlight the need for further research in the assessment of nutrition, determination of energy needs and caloric dosing, specific nutrient needs, and management.

A multicenter retrospective study of calcineurin inhibitors in nephrotic syndrome secondary to podocyte gene variants

While 44-83% of children with steroid-resistant nephrotic syndrome (SRNS) without a proven genetic cause respond to treatment with a calcineurin inhibitor (CNI), current guidelines recommend against the use of immunosuppression in monogenic SRNS. This is despite existing evidence suggesting that remission with CNI treatment is possible and can improve prognosis in some cases of monogenic SRNS. Herein, our retrospective study assessed response frequency, predictors of response and kidney function outcomes among children with monogenic SRNS treated with a CNI for at least three months. Data from 203 cases (age 0-18 years) were collected from 37 pediatric nephrology centers. Variant pathogenicity was reviewed by a geneticist, and 122 patients with a pathogenic and 19 with a possible pathogenic genotype were included in the analysis. After six months of treatment and at last visit, 27.6% and 22.5% of all patients respectively, demonstrated partial or full response. Achievement of at least partial response at six months of treatment conferred a significant reduction in kidney failure risk at last follow-up compared to no response (hazard ratio [95% confidence interval] 0.25, [0.10-0.62]). Moreover, risk of kidney failure was significantly lower when only those with a follow-up longer than two years were considered (hazard ratio 0.35, [0.14-0.91]). Higher serum albumin level at CNI initiation was the only factor related to increased likelihood of significant remission at six months (odds ratio [95% confidence interval] 1.16, [1.08-1.24]). Thus, our findings justify a treatment trial with a CNI also in children with monogenic SRNS.

Acute Kidney Injury Among Children Likely Associated with Diethylene Glycol-Contaminated Medications - The Gambia, June-September 2022.

On July 26, 2022, a pediatric nephrologist alerted The Gambia's Ministry of Health (MoH) to a cluster of cases of acute kidney injury (AKI) among young children at the country's sole teaching hospital, and on August 23, 2022, MoH requested assistance from CDC. CDC epidemiologists arrived in The Gambia, a West African country, on September 16 to assist MoH in characterizing the illness, describing the epidemiology, and identifying potential causal factors and their sources. Investigators reviewed medical records and interviewed caregivers to characterize patients' symptoms and identify exposures. The preliminary investigation suggested that various contaminated syrup-based children's medications contributed to the AKI outbreak. During the investigation, MoH recalled implicated medications from a single international manufacturer. Continued efforts to strengthen pharmaceutical quality control and event-based public health surveillance are needed to help prevent future medication-related outbreaks.

Hemolytic-uremic syndrome: 24 years' experience of a pediatric nephrology unit.

A better understanding of hemolytic-uremic syndrome (HUS) pathophysiology significantly changed its treatment and prognosis. The aim of this study is to characterize the clinical features, severity, management, and outcomes of HUS patients. Retrospective study of HUS patients admitted to a Pediatric Nephrology Unit between 1996 and 2020. Demographic and clinical data regarding etiology, severity, treatment strategies, and patient outcome were collected. Twenty-nine patients with HUS were admitted to our unit, but four were excluded. Median age at diagnosis was two years (2 months - 17 years). Clinical manifestations included diarrhea, vomiting, oliguria, hypertension, and fever. During the acute phase, 14 patients (56%) required renal replacement therapy. Infectious etiology was identified in seven patients (five Escherichia coli and two Streptococcus pneumoniae). Since 2015, 2/7 patients were diagnosed with complement pathway dysregulation HUS and there were no cases of infectious etiology detected. Six of these patients received eculizumab. The global median follow-up was 6.5 years [3 months-19.8 years]. One patient died, seven had chronic kidney disease, four of whom underwent kidney transplantation, one relapsed, and seven had no sequelae. These results reflect the lack of infectious outbreaks in Portugal and the improvement on etiological identification since genetic testing was introduced. The majority of patients developed sequels and mortality was similar to that of other countries. HUS patients should be managed in centers with intensive care and pediatric nephrology with capacity for diagnosis, etiological investigation, and adequate treatment. Long-term follow-up is essential.

Fetal and Perinatal Nephrology: Small but Mighty.

Fetal and Perinatal Nephrology: Small but Mighty.

Histoire de la néphrologie pédiatrique en France

Histoire de la néphrologie pédiatrique en France

Chronic kidney disease and growth failure: Efficacy of growth hormone treatment

Growth failure is a frequent complication observed in children with chronic kidney disease (CKD) and correlated to increased morbidity and mortality. To achieve a normal growth in children with CKD remains challenging for pediatric nephrologists. Growth failure in the setting of pediatric CKD is multifactorial and related to an impaired sensitivity to growth hormone and to a deficiency of IGF1 (insulin-like growth factor1). Growth failure management has improved during the last two decades and consists of correcting any nutritional and metabolic abnormalities, of an improvement of dialysis for children on end-stage renal disease, and of an administration of a supraphysiologic dose of recombinant growth hormone to overcome GH insensitivity. This article summarizes the causes, outcomes and assessment tools of growth in children with CKD as well as the management of recombinant growth hormone.