Purpose The impact of the original International Children's Continence Society terminology document on lower urinary tract function resulted in the global establishment of uniformity and clarity in ...the characterization of lower urinary tract function and dysfunction in children across multiple health care disciplines. The present document serves as a stand-alone terminology update reflecting refinement and current advancement of knowledge on pediatric lower urinary tract function. Materials and Methods A variety of worldwide experts from multiple disciplines in the ICCS leadership who care for children with lower urinary tract dysfunction were assembled as part of the standardization committee. A critical review of the previous ICCS terminology document and the current literature was performed. In addition, contributions and feedback from the multidisciplinary ICCS membership were solicited. Results Following a review of the literature during the last 7 years the ICCS experts assembled a new terminology document reflecting the current understanding of bladder function and lower urinary tract dysfunction in children using resources from the literature review, expert opinion and ICCS member feedback. Conclusions The present ICCS terminology document provides a current and consensus update to the evolving terminology and understanding of lower urinary tract function in children. For the complete document visit http://jurology.com/.
Dietary management in pediatric chronic kidney disease (CKD) is an area fraught with uncertainties and wide variations in practice. Even in tertiary pediatric nephrology centers, expert dietetic ...input is often lacking. The Pediatric Renal Nutrition Taskforce (PRNT), an international team of pediatric renal dietitians and pediatric nephrologists, was established to develop clinical practice recommendations (CPRs) to address these challenges and to serve as a resource for nutritional care. We present CPRs for energy and protein requirements for children with CKD stages 2–5 and those on dialysis (CKD2–5D). We address energy requirements in the context of poor growth, obesity, and different levels of physical activity, together with the additional protein needs to compensate for dialysate losses. We describe how to achieve the dietary prescription for energy and protein using breastmilk, formulas, food, and dietary supplements, which can be incorporated into everyday practice. Statements with a low grade of evidence, or based on opinion, must be considered and adapted for the individual patient by the treating physician and dietitian according to their clinical judgment. Research recommendations have been suggested. The CPRs will be regularly audited and updated by the PRNT.
Atypical hemolytic uremic syndrome (aHUS) is a rare, genetic, life-threatening disease. The Global aHUS Registry collects real-world data on the natural history of the disease. Here we characterize ...end-stage renal disease (ESRD)-free survival, the rate of thrombotic microangiopathy, organ involvement and the genetic background of 851 patients in the registry, prior to eculizumab treatment. A sex-specific difference was apparent according to age at initial disease onset as the ratio of males to females was 1.3:1 for childhood presentation and 1:2 for adult presentation. Complement Factor I and Membrane Cofactor Protein mutations were more common in patients with initial presentation as adults and children, respectively. Initial presentation in childhood significantly predicted ESRD risk (adjusted hazard ratio 0.55 95% confidence interval 0.41–0.73, whereas sex, race, family history of aHUS, and time from initial presentation to diagnosis, did not. Patients with a Complement Factor H mutation had reduced ESRD-free survival, whereas Membrane Cofactor Protein mutation was associated with longer ESRD-free survival. Additionally extrarenal organ manifestations occur in 19%–38% of patients within six months of initial disease presentation (dependent on organ). Thus, our real-world results provide novel insights regarding phenotypic variables and genotypes on the clinical manifestation and progression of aHUS.
Background
Eculizumab is approved for atypical hemolytic uremic syndrome (aHUS). Guidelines discuss the importance of prompt treatment. We report a post hoc analysis investigating the effect of ...baseline factors, including patient characteristics and time from the latest aHUS manifestation to eculizumab initiation, on change from baseline in estimated glomerular filtration rate (eGFR) and other outcomes.
Methods
Data were pooled from four phase 2, open-label, single-arm, prospective clinical studies of eculizumab for patients with aHUS. Multivariate regressions identified predictors of eGFR change from baseline. The proportion of patients achieving sustained eGFR increase (defined: ≥15 ml/min/1.73 m
2
for ≥28 days) and platelet count normalization were evaluated 1 year post-treatment. Baseline characteristics and eGFR outcomes were summarized by time to treatment from last aHUS manifestation ≤7 days (
n
= 21) versus >7 days (
n
= 76).
Results
Baseline eGFR were similar between groups. Multivariate regression analysis demonstrated time from aHUS manifestation to eculizumab treatment, age, baseline lactate dehydrogenase (LDH) and baseline hemoglobin were independently predictive of eGFR change from baseline. Mean eGFR change from baseline at 1 year was significantly higher in patients treated in ≤7 days than >7 days (57 vs. 23 ml/min/1.73 m
2
, p = 0.0098). After 1 year, 17/21 and 36/76 patients in the ≤7 and >7 day groups, respectively, achieved a sustained increase in eGFR. Mean time to platelet count normalization was similar between groups.
Conclusions
Younger age, higher baseline LDH and lower baseline hemoglobin were associated with greater eGFR improvements. Early eculizumab initiation led to improved renal recovery, demonstrating the importance of rapid diagnosis and treatment of patients with aHUS.
Purpose We provide updated, clinically useful recommendations for treating children with monosymptomatic nocturnal enuresis. Materials and Methods Evidence was gathered from the literature and ...experience was gathered from the authors with priority given to evidence when present. The draft document was circulated among all members of the International Children's Continence Society as well as other relevant expert associations before completion. Results Available evidence suggests that children with monosymptomatic nocturnal enuresis could primarily be treated by a primary care physician or an adequately educated nurse. The mainstays of primary evaluation are a proper history and a voiding chart. The mainstays of primary therapy are bladder advice, the enuresis alarm and/or desmopressin. Therapy resistant cases should be handled by a specialist doctor. Among the recommended second line therapies are anticholinergics and in select cases imipramine. Conclusions Enuresis in a child older than 5 years is not a trivial condition, and needs proper evaluation and treatment. This requires time but usually does not demand costly or invasive procedures.
Background
The first uninterrupted sleep period (FUSP, time up to the first episode of enuresis/nocturia after falling asleep) is a frequently investigated parameter in adults with nocturia, as it ...correlates with quality of life. However, it has not been included in pediatric enuresis studies.
Aim
Investigate FUSP, circadian renal water and sodium handling, as well as sleep quality before and after desmopressin therapy in enuresis.
Materials and Methods
We conducted a post hoc analysis of a prospective study in 30 treatment‐naïve children with enuresis who underwent a video‐polysomnography and a 24‐h urine concentration profile before and after 6 months of desmopressin therapy. We analyzed FUSP, periodic limb movements in sleep (PLMS), and arousal indexes and their correlations with the urinary parameters.
Results
Sixteen children with a mean age of 10.9 ± 3.1 years had full registrations and were included in this subanalysis. After therapy, FUSP was significantly longer (p < 0.001), and the PLMS index was lower (p = 0.023). Significant differences in the circadian rhythm of diuresis (night/day diuresis, p = 0.041), nocturnal urinary osmolality (p = 0.009), and creatinine (p = 0.001) were found, demonstrating the increase of urinary concentration overnight by desmopressin, as well as a significant antidiuretic effect (diuresis p = 0.013 and diuresis rate (p = 0.008). There was no correlation between the difference of FUSP, PLMS index, and urinary parameters. Nevertheless, despite this study being underpowered, there are indications of a correlation between nocturnal diuresis and diuresis rate.
Results
Our results support the need for further research regarding FUSP in children with enuresis, in accordance with nocturia studies in adults, as this parameter could be valuable in the follow‐up and evaluation of therapeutic strategies for enuresis.
Background
COVID-19 was declared a global health emergency. Since children are less than 1% of reported cases, there is limited information to develop evidence-based practice recommendations. The ...objective of this study was to rapidly gather expert knowledge and experience to guide the care of children with chronic kidney disease during the COVID-19 pandemic.
Methods
A four-round multi-center Delphi exercise was conducted among 13 centers in 11 European countries of the European Pediatric Dialysis Working Group (EPDWG) between March, 16th and 20th 2020. Results were analyzed using a mixed methods qualitative approach and descriptive statistics.
Results
Thirteen COVID-19 specific topics of particular need for guidance were identified. Main themes encompassed testing strategies and results (
n
= 4), changes in use of current therapeutics (
n
= 3), preventive measurements of transmission and management of COVID-19 (
n
= 3), and changes in standard clinical care (
n
= 3). Patterns of center-specific responses varied according to regulations and to availability of guidelines.
Conclusions
As limited quantitative evidence is available in real time during the rapid spread of the COVID-19 pandemic, qualitative expert knowledge and experience represent the best evidence available. This Delphi exercise demonstrates that use of mixed methodologies embedded in an established network of experts allowed prompt analysis of pediatric nephrologists’ response to COVID-19 during this fast-emerging public health crisis. Such rapid sharing of knowledge and local practices is essential to timely and optimal guidance for medical management of specific patient groups in multi-country health care systems such as those of Europe and the US.
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