Assessment of nutritional status in children with kidney diseases-clinical practice recommendations from the Pediatric Renal Nutrition Taskforce

In children with kidney diseases, an assessment of the child’s growth and nutritional status is important to guide the dietary prescription. No single metric can comprehensively describe the nutrition status; therefore, a series of indices and tools are required for evaluation. The Pediatric Renal Nutrition Taskforce (PRNT) is an international team of pediatric renal dietitians and pediatric nephrologists who develop clinical practice recommendations (CPRs) for the nutritional management of children with kidney diseases. Herein, we present CPRs for nutritional assessment, including measurement of anthropometric and biochemical parameters and evaluation of dietary intake. The statements have been graded using the American Academy of Pediatrics grading matrix. 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. Audit and research recommendations are provided. The CPRs will be periodically audited and updated by the PRNT

Hemodiafiltration maintains a sustained improvement in blood pressure compared to conventional hemodialysis in children-the HDF, heart and height (3H) study

BACKGROUND: Hypertension is prevalent in children on dialysis and associated with cardiovascular disease. We studied the blood pressure (BP) trends and the evolution of BP over 1 year in children on conventional hemodialysis (HD) vs. hemodiafiltration (HDF). METHODS: This is a post hoc analysis of the "3H - HDF-Hearts-Height" dataset, a multicenter, parallel-arm observational study. Seventy-eight children on HD and 55 on HDF who had three 24-h ambulatory BP monitoring (ABPM) measures over 1 year were included. Mean arterial pressure (MAP) was calculated and hypertension defined as 24-h MAP standard deviation score (SDS) ≥95th percentile. RESULTS: Poor agreement between pre-dialysis systolic BP-SDS and 24-h MAP was found (mean difference - 0.6; 95% limits of agreement -4.9-3.8). At baseline, 82% on HD and 44% on HDF were hypertensive, with uncontrolled hypertension in 88% vs. 25% respectively; p < 0.001. At 12 months, children on HDF had consistently lower MAP-SDS compared to those on HD (p < 0.001). Over 1-year follow-up, the HD group had mean MAP-SDS increase of +0.98 (95%CI 0.77-1.20; p < 0.0001), whereas the HDF group had a non-significant increase of +0.15 (95%CI -0.10-0.40; p = 0.23). Significant predictors of MAP-SDS were dialysis modality (β = +0.83 [95%CI +0.51 - +1.15] HD vs. HDF, p < 0.0001) and higher inter-dialytic-weight-gain (IDWG)% (β = 0.13 [95%CI 0.06-0.19]; p = 0.0003). CONCLUSIONS: Children on HD had a significant and sustained increase in BP over 1 year compared to a stable BP in those on HDF, despite an equivalent dialysis dose. Higher IDWG% was associated with higher 24-h MAP-SDS in both groups

Hemodiafiltration Is Associated With Reduced Inflammation and Increased Bone Formation Compared With Conventional Hemodialysis in Children: The HDF, Hearts and Heights (3H) Study

BACKGROUND: Patients on dialysis have a high burden of bone-related comorbidities, including fractures. We report a post hoc analysis of the prospective cohort study HDF, Hearts and Heights (3H) to determine the prevalence and risk factors for chronic kidney disease-related bone disease in children on hemodiafiltration (HDF) and conventional hemodialysis (HD). METHODS: The baseline cross-sectional analysis included 144 children, of which 103 (61 HD, 42 HDF) completed 12-month follow-up. Circulating biomarkers of bone formation and resorption, inflammatory markers, fibroblast growth factor-23, and klotho were measured. RESULTS: Inflammatory markers interleukin-6, tumor necrosis factor-α, and high-sensitivity C-reactive protein were lower in HDF than in HD cohorts at baseline and at 12 months (P < .001). Concentrations of bone formation (bone-specific alkaline phosphatase) and resorption (tartrate-resistant acid phosphatase 5b) markers were comparable between cohorts at baseline, but after 12-months the bone-specific alkaline phosphatase/tartrate-resistant acid phosphatase 5b ratio increased in HDF (P = .004) and was unchanged in HD (P = .44). On adjusted analysis, the bone-specific alkaline phosphatase/tartrate-resistant acid phosphatase 5b ratio was 2.66-fold lower (95% confidence interval, −3.91 to −1.41; P < .0001) in HD compared with HDF. Fibroblast growth factor-23 was comparable between groups at baseline (P = .52) but increased in HD (P < .0001) and remained unchanged in HDF (P = .34) at 12 months. Klotho levels were similar between groups and unchanged during follow-up. The fibroblast growth factor-23/klotho ratio was 3.86-fold higher (95% confidence interval, 2.15–6.93; P < .0001) after 12 months of HD compared with HDF. CONCLUSION: Children on HDF have an attenuated inflammatory profile, increased bone formation, and lower fibroblast growth factor-23/klotho ratios compared with those on HD. Long-term studies are required to determine the effects of an improved bone biomarker profile on fracture risk and cardiovascular health

Long-term renal outcome in children with OCRL mutations: retrospective analysis of a large international cohort

BACKGROUND: Lowe syndrome (LS) and Dent-2 disease (DD2) are disorders associated with mutations in the OCRL gene and characterized by progressive chronic kidney disease (CKD). Here, we aimed to investigate the long-term renal outcome and identify potential determinants of CKD and its progression in children with these tubulopathies. METHODS: Retrospective analyses were conducted of clinical and genetic data in a cohort of 106 boys (LS: 88 and DD2: 18). For genotype-phenotype analysis, we grouped mutations according to their type and localization. To investigate progression of CKD we used survival analysis by Kaplan-Meier method using stage 3 CKD as the end-point. RESULTS: Median estimated glomerular filtration rate (eGFR) was lower in the LS group compared with DD2 (58.8 versus 87.4 mL/min/1.73 m(2), P < 0.01). CKD stage II-V was found in 82% of patients, of these 58% and 28% had moderate-to-severe CKD in LS and DD2, respectively. Three patients (3%), all with LS, developed stage 5 of CKD. Survival analysis showed that LS was also associated with a faster CKD progression than DD2 (P < 0.01). On multivariate analysis, eGFR was dependent only on age (b = -0.46, P < 0.001). Localization, but not type of mutations, tended to correlate with eGFR. There was also no significant association between presence of nephrocalcinosis, hypercalciuria, proteinuria and number of adverse clinical events and CKD. CONCLUSIONS: CKD is commonly found in children with OCRL mutations. CKD progression was strongly related to the underlying diagnosis but did not associate with clinical parameters, such as nephrocalcinosis or proteinuria

Management of children with congenital nephrotic syndrome: challenging treatment paradigms

Background: Management of children with congenital nephrotic syndrome (CNS) is challenging. Bilateral nephrectomies followed by dialysis and transplantation are practiced in most centres, but conservative treatment may also be effective. / Methods: We conducted a 6-year review across members of the European Society for Paediatric Nephrology Dialysis Working Group to compare management strategies and their outcomes in children with CNS. / Results: Eighty children (50% male) across 17 tertiary nephrology units in Europe were included (mutations in NPHS1, n = 55; NPHS2, n = 1; WT1, n = 9; others, n = 15). Excluding patients with mutations in WT1, antiproteinuric treatment was given in 42 (59%) with an increase in S-albumin in 70% by median 6 (interquartile range: 3–8) g/L (P < 0.001). Following unilateral nephrectomy, S-albumin increased by 4 (1–8) g/L (P = 0.03) with a reduction in albumin infusion dose by 5 (2–9) g/kg/week (P = 0.02). Median age at bilateral nephrectomies (n = 29) was 9 (7–16) months. Outcomes were compared between two groups of NPHS1 patients: those who underwent bilateral nephrectomies (n = 25) versus those on conservative management (n = 17). The number of septic or thrombotic episodes and growth were comparable between the groups. The response to antiproteinuric treatment, as well as renal and patient survival, was independent of NPHS1 mutation type. At final follow-up (median age 34 months) 20 (80%) children in the nephrectomy group were transplanted and 1 died. In the conservative group, 9 (53%) remained without dialysis, 4 (24%; P < 0.001) were transplanted and 2 died. / Conclusion: An individualized, stepwise approach with prolonged conservative management may be a reasonable alternative to early bilateral nephrectomies and dialysis in children with CNS and NPHS1 mutations. Further prospective studies are needed to define indications for unilateral nephrectomy

Pilot initiatives of adult hearing screening in Italy

A series of pilot initiatives of adult hearing screening programs were organized in eight large and small-size cities in Italy in the past two years. The screening initiatives were held in public places, supermarkets, drugstores, and in some universities of the third age, and involved an overall population of 2,278 screened subjects with age ranging from 13 to 93 years. Three different screening tests were used to assess hearing ability in the participants, i.e.: screening pure tone audiometry (PTA), an automated speech-in-noise screening test (the SUN-test), and a screening questionnaire of self-perceived hearing handicap (the HHIE-S). This paper describes the organization and management of these screening initiatives and reviews the main results obtained in the screened population, using the three different screening tests. Results obtained in these pilot initiatives showed that screening adults for hearing problems might be feasible, on a local level, in non clinical settings and can be performed quite easily with the support of local coordinators and partners, such as associations or local authorities. It is recognized that further initiatives and studies will have to be performed to better define the key aspects related to the organization and management of adult hearing screening programs, either at a local, regional, or national level

Apps for hearing science and care

Purpose: Our research aims at the identification and assessment of applications (referred to as apps) in the hearing health care domain. This research forum article presents an overview of the current availability, affordability, and variety of hearing-related apps. Method: The available apps were reviewed by searching on the leading platforms (iOS, Android, Windows Phone stores) using the keywords hearing, audiology, audio, auditory, speech, language, tinnitus, hearing loss, hearing aid, hearing system, cochlear implant, implantable device, auditory training, hearing rehabilitation, and assistive technology/tool/device. On the basis of the offered services, apps were classified into 4 application domains: (a) screening and assessment, (b) intervention and rehabilitation, (c) education and information, and (d) assistive tools. Conclusions: A large variety of apps are available in the hearing health care domain. These cover a wide range of services for people with hearing or communication problems as well as for hearing professionals, families, or informal caregivers. This evolution can potentially bring along considerable advantages and improved outcomes in the field of hearing health care. Nevertheless, potential risks and threats (e.g., safety, quality, effectiveness, privacy, and regulation) should not be overlooked. Significant research— particularly in terms of assessment and guidance—is still needed for the informed, aware, and safe adoption of hearing-related apps by patients and professionals

SUN-test (Speech Understanding in Noise): a method for hearing disability screening

The SUN-test (Speech Understanding in Noise) is a speech-innoise test to screen adults and older adults for hearing disability. The SUN-test consists in a short list of intervocalic consonants (VCV, vowel-consonant-vowel) in noise that are presented in a three-alternatives forced choice (3AFC) paradigm by means of a touch-screen interface. Based on the number of stimuli correctly identified, the tested subject gets one of three possible test outcomes: no listening difficulties, a hearing check would be advisable, or a hearing check is recommended. This paper reviews the main results obtained with the SUNtest in the Italian language in a population of nearly 1,300 adults and older adults with varying degrees of audiometric thresholds and audiometric configurations, tested both in low and in high ambient noise settings. Results obtained in the tested population revealed that the outcomes of the SUN-test were in line with the outcomes of pure-tone testing, and that the test performance was similar both in low and in high ambient noise (up to 65 dB A). Results obtained with the SUNtest were not biased by the age of the subject because the performance of younger and older subjects in the test was similar. The mean duration of the SUN-test was nearly 40 s/ear, and was lower than 1 minute per ear even in subjects older than 80 years so that both ears could be tested, on average, in 2 minutes. The SUN-test was considered easy or slightly difficult by nearly 90% of subjects; test duration was judged short or fair by nearly 95% of subjects, and the overall evaluation of the test was pleasant, or neutral, in more than 90% of subjects. Overall, results of this study indicated that the SUN-test might be feasible for application in adult hearing screening. The test is fast, easy, self convincing, and reflects differences in hearing sensitivity between the tested subjects. The outcomes of the SUN-test were not influenced by the noise level in the test room (up to 65 dB A) indicating that the test, as such, might be feasible to screen adults and older adults both in clinical and in non clinical settings, such as convenient care clinics, hearing aid providers, or pharmacies, where the ambient noise is, typically, not controlled

The ALFA4hearing model (at-a-glance labeling for features of apps for hearing health care) to characterize mobile apps for hearing health care

Purpose: The aim of the study was to analyze, by using the ALFA4Hearing model (At-a-Glance Labeling for Features of Apps for Hearing Health Care), a sample of apps over a wide range of services in the hearing health care (HHC) domain in order to take a first picture of the current scenario of apps for HHC. Method: We tested 120 apps, and we characterized them by using the ALFA4Hearing model, which includes 29 features in 5 components (Promoters, Services, Implementation, Users, and Descriptive Information). We analyzed (a) the distribution of the 29 features in the sample, (b) the relationship between the Implementation features and the Services provided by the apps, and (c) the distribution of the 29 features in apps for professional use. Results: The analysis of our sample of apps by means of the ALFA4Hearing model highlighted interesting trends and emerging challenges. Also, results suggested many potential opportunities for research and clinical practice, such as greater involvement of stakeholders, improved evidence base, higher technical quality, and usability. Conclusions: The ALFA4Hearing model is able to represent, at a glance, a large amount of information about apps for HHC, highlighting trends and challenges. It might be useful to HHC professionals as a basis for app characterization and informed decision making