Best practice guidelines for idiopathic nephrotic syndrome: recommendations versus reality.

BACKGROUND: The optimal therapeutic regimen for managing childhood idiopathic nephrotic syndrome (INS) is still under debate. We have evaluated the choice of steroid regimen and of symptomatic treatment adopted by pediatricians and pediatric nephrologists in a large number of centers as the first step towards establishing a shared protocol METHODS: This was a multicenter, retrospective study. A total of 231 children (132 admitted to pediatric units) aged 6 months to <15 years who presented with onset of nephrotic syndrome to 54 pediatric units and six pediatric nephrology units in Italy between 2007 and 2009 were eligible for entry into the study. RESULTS: Median steroid dosing was 55 (range 27-75) mg/m(2)/day. The overall median cumulative dose regimen for the first episode was 3,440 (1,904-6,035) mg/m(2), and the median duration of the therapeutic regimen was 21 (9-48) weeks. The total duration and cumulative steroid dose were significantly higher in patients treated by pediatricians than in those treated by pediatric nephrologists (p = 0.001 and p = 0.008). Among the patient cohort, 55, 64 and 22 % received albumin infusions, diuretics and acetyl salicylic acid treatment, respectively, but the laboratory and clinical data did not differ between children treated or not treated with symptomatic drugs. Albumin and diuretic use did not vary between patients in pediatric units and those in pediatric nephrology units. CONCLUSIONS: This study shows major differences in steroid and symptomatic treatment of nephrotic syndrome by pediatricians and pediatric nephrologists. As these differences can influence the efficacy of the treatments and the appearance of side-effects, shared guidelines and their implementation through widespread educational activities are necessary.

Anderson-Fabry disease: a protean clinical behavior and a chance diagnosis.

Anderson-Fabry disease is a rare inborn X-linked glycosphingolipid storage disorder in which the deficient activity of the enzyme alfa-galactosidase A (alfa-gal A) leads to the progressive tissular accumulation of lipidic molecules which, in turn, cause a protean pattern of multi-organ disfunction. Enzyme replacement therapy has recently become available and has proved to be effective in controlling the disorder. We present and discuss the case of a family with this disease, with special attention to the variability of clinical features and the difficulty of a correct diagnosis.

Early origin of adult renal disease.

Observational studies in humans and experimental studies in animals have clearly shown that renal failure may start early in life. 'Fetal programming' is regulated by adaptations occurring in uterus including maternal nutrition, placental blood supply, and epigenetic changes. Low birth weight predisposes to hypertension and renal insufficiency. Congenital abnormalities of the kidney and urinary tract, adverse postnatal events, wrong nutritional habits may produce renal damage that will become clinically relevant in adulthood. Prevention should start early in children at risk of renal disease.

Glycaemic variability and inflammation in subjects with metabolic syndrome.

Subjects who develop diabetes have an increased cardiovascular risk even before the appearance of diabetes. The aim of this study was to investigate the glycaemic variability measured by continuous glucose monitoring (CGM CV%) in nondiabetic subjects with metabolic syndrome (MS) and to explore if glycaemic variability was associated with circulating levels of interleukin-6 (IL-6), a proinflammatory cytokine, or with an anti-inflammatory factor like adiponectin. Three groups of obese subjects with (MS+: 6m, 8f; BMI 33.1+/-1.4 mean+/-SEM) or without metabolic syndrome (MS-: 2m, 4f; BMI 29.2+/-2.2) and with MS associated with type 2 diabetes (MS/T2D: 3m, 5f; BMI 32.9+/-1.4) were investigated. The glycaemic variability was measured in all subjects in terms of CV% of the glycaemic values obtained every 3 min during the course of a 48 h CGM performed using a subcutaneous glucose sensor. The average CGM CV% increased from MS- group (21.1%) to the MS+ group (23.9%) and to the MS+/T2D group (27.4%) but it was not correlated to the CGM mean glycaemia (r=0.20; P=ns). In some instances, CGM CV% was found higher in MS+ subjects than in some MS+ T2D ones. Stepwise multiple correlation analysis showed that IL-6 predicted CGM CV% (R(2)=0.35, beta=0.13; P<0.05) independently from BMI, waist circumference, adiponectin and insulin concentrations. In conclusion, the CGM CV% may contribute to better describe the individual metabolic state and to understand the pathogenesis of endothelial dysfunction in non diabetic subjects with MS.

The efficacy of antiviral therapy after renal transplant in a HCV-positive recipient from a HCV-positive donor / La eficacia de la terapia antiviral después del trasplante renal en un receptor con HCV positivo de un donante con HCV positivo

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