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Objective To evaluate the sensitivity of anthropometry and bioelectrical impedance analysis (BIA) in detecting alterations in body composition of children treated with peritoneal dialysis (PD), and to determine the prevalence of malnutrition in this population, in short- and long-term PD duration, using anthropometric and BIA-derived indices. Patients Eighteen children treated with automated PD (11 males, 7 females; mean age 8.7 ± 4.7 years). Design Eighteen patients were studied using anthropometry and BIA at the start (t0) and after 6 months (t1) of PD, 15 of these patients at 12 months (t2), and 8 at 24 months (t3) of PD. Midarm muscle circumference (MAMC), arm muscle area (AMA), and arm fat area (AFA) were calculated from anthropometric measures according to Frisancho (FrisanchoAR. New norms of upper limb fat and muscle areas for assessment of nutritional status. Am J Clin Nutr 1981; 34:2540–5.). The bioelectrical measures of resistance (R) and reactance (Xc) were obtained directly from the impedance signal; phase angle (PA) and distance (D) were calculated using mathematical formulas. Nutritional status was assessed by anthropometric measurements and BIA-derived indices, expressed as standard deviation scores (SDS), and by a score system based on BIA and anthropometric parameters. The percentage of children with values of anthropometric and BIA-derived indices below the 3rd percentile or between the 3rd and 25th percentiles, and the percentage of children with scores of 7 – 12 and 4 – 6 were calculated in order to detect patients with severe or moderate derangement of nutritional status. Results The mean SDS values of Xc, PA, and D significantly improved ( p = 0.05, p = 0.001, p = 0.02) during the first 6 months of PD and remained almost stable during the following months. The SDS values of the anthropometric indices were less compromised than those of the BIA-derived indices, particularly at the start of dialysis. By 6 months, the percentages of children with values of BIA and anthropometric indices below the 3rd percentile had decreased. The percentages of patients with moderate and severe derangement of BIA and anthropometric indices remained substantially unchanged after 12 months. However, at 24 months, the percentage of patients with moderate derangement of BIA indices increased. All these findings were confirmed by the nutritional score system. Conclusion BIA is more sensitive than anthropometry in detecting alterations in body composition of children on PD. The prevalence of malnutrition, high at the commencement of PD, decreases during the first year of treatment but not over the long term.

Gitelman disease was diagnosed in two unrelated children with hypokalemic metabolic alkalosis and growth failure (a boy and a girl aged 7 mo and 9.5 y, respectively, at clinical presentation) on the basis of mutations detected in the gene encoding the thiazide-sensitive NaCl cotransporter of the distal convoluted tubule. GH deficiency was demonstrated by specific diagnostic tests in both children. Hypertonic saline infusion tests showed a partial vasopressin deficiency in the girl and delayed secretion of this hormone in the boy. Magnetic resonance imaging revealed an empty sella in both cases. Up to now, hypomagnesemia and hypocalciuria have been considered obligatory criteria for the diagnosis of Gitelman disease; however, our two patients had hypomagnesemia and hypocalciuria in less than half the determinations. GH replacement treatment was associated with a good clinical response in both children. It appears that these cases represent a new phenotype, not previously described in Gitelman disease, and that the entity may be considered a new complex hereditary renal tubular-pituitary syndrome.

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To evaluate the efficacy of renal transplantation in small pediatric patients, we have reviewed 41 allografts performed in 39 children (28 M/11 F) less than 6 years of age between 1987 and 1998 in the North Italy Transplant Program. Of these patients, 39 had a cadaver donor and 2 a living-related donor, with ages ranging from 20 days to 35 years. The mean follow-up was 56 months. Graft survival was 74.5% and 70.5% at 1 and 5 years, respectively. The causes of graft lost were acute rejection (4), graft vascular thrombosis (4), and hemolytic uremic syndrome recurrence (1). Only 1 patient has died due to chickenpox. Double and triple immunosuppressive therapies were used in 63% and 37% of patients, respectively, on the basis of different center protocols, without differences in graft survival. Steroids were successfully administered on alternate days in 37% of patients, 6-12 months after transplantation. Thrombosis was reported in 2 of 6 kidneys from donors less than 1 year of age and in 2 of 35 donors older than 1 year (P<0.05). Thirty rejections occurred in 23 patients: 7 episodes were steroid resistant and were treated with ATG/OKT3. Thirty-four infections were reported in 16 of 41 patients; of these 17 were viral, 14 bacterial, and 3 due to Mycoplasma. Four surgical complications were reported: 1 graft artery stenosis, 1 ureteral stenosis, 1 urinary leak, and 1 lymphocele. Mean height standard deviation score improved from -2.0±1.3 pre transplantation to -1.8±1.4, -1.5±1.3, and -1.5±1.5 at 1, 2, and 5 years post transplantation. Linear growth was significantly better in infants treated with alternate-day steroids. Hypertension was a frequent complication, since 19 of the 30 patients with a 5-year follow-up were still being treated with antihypertensive drugs. In conclusion, graft survival in patients less than 6 years old is satisfactory and similar to that obtained in children aged from 6 to 18 years (70.5% vs. 78.9% at 5 years, P=NS). Consequently, since there are many difficulties in managing infants on maintenance dialysis, an early transplant should be considered. Donors older than 24 months carry a low risk of vascular thrombosis and may be successfully grafted in infants.