Low dialysate sodium in children and young adults on maintenance hemodialysis: a prospective, randomized, crossover study.

BACKGROUND: The optimal dialysate sodium concentration (dNa) in children on hemodialysis (HD) is unknown. The aim of this study was to compare the effect on interdialytic weight gain (IDWG) and blood pressure (BP) of a low (135 mmol/l) and standard dNa (138 mmol/l) in children and young adults on maintenance HD. METHODS: This prospective single-blind randomized crossover study consisted of a randomized sequence of two phases: "standard dNa" of 138 mmol/L and "low dNa" of 135 mmol/L. Each phase lasted 4 weeks. Inclusion criteria were age < 25 years, hypertension, pre-HD serum Na (sNa) ≥ 130 mmol/L, and occurrence of symptoms in less than 25% of sessions. Primary outcomes were pre-HD systolic and diastolic BP and IDWG. RESULTS: Fifteen patients were recruited, mean age 17.8 ± 4.4 years. Pre-HD SBP and DBP were not different between the two treatments. Mean IDWG was significantly lower with low dNa than with standard dNa: 2.12 ± 1.39% vs. 2.77 ± 1.53%, respectively (p = 0.008). The first-hour refill index (a volume index based on blood-volume monitoring) was significantly lower with dNa 135 mmol/L (p = 0.018). The mean Na gradient (dNa-sNa) was - 2.53 ± 2.4 mmol/L with dNa 135 mmol/L and 0.17 ± 2.8 mmol/L with dNa 138 mmol/L (p = 0.0001). The incidence of symptomatic sessions was similar (1.0% vs. 1.0%). CONCLUSIONS: In a selected population of hypertensive pediatric and young adult HD patients, a dNa of 135 mmol/L was associated with a significant reduction of IDWG compared with a dNa of 138 mmol/L. Furthermore, long-term studies are needed to investigate the effect of lowering dNa on BP. A higher resolution version of the Graphical abstract is available as Supplementary information.

Family burden of children suffering from epidermolysis bullosa.

BACKGROUND: Living with a rare disease has profound effects on the patient's life and that of their entire family, with practical and psychosocial consequences. This is particularly true when the patient is a child. The principal aim of this study was to measure the family burden in Epidermolysis Bullosa (EB). The secondary endpoint was to evaluate the possible correlation between family burden and the severity of EB. METHODS: A sample of 50 families with one or two children affected by EB were recruited between January 2016 and February 2017 to answer a 20-item questionnaire - the EB Burden of Disease (EB-BoD) - developed and validated to assess the family burden of children with EB. RESULTS: The presence of a child with EB may have profound negative implications on several different areas of daily life. In particular, the results demonstrate important differences between the different subtypes of epidermolysis bullosa regarding most of the categories considered by the questionnaire. For three categories out of four (family life, child's life, economic and social impact), a higher score is observed for children with the more debilitating forms of EB: recessive dystrophic EB (RDEB) and junctional EB (JEB). CONCLUSIONS: It is important to work with patients and their families to identify and strengthen adaptive and coping behaviors. That is possible only through the synergistic working of a multidisciplinary team made up of experienced doctors, psychologists, and social workers while in contact with patient Associations.