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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/day. The overall median cumulative dose regimen for the first episode was 3,440 (1,904-6,035) mg/m, 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. [ABSTRACT FROM AUTHOR]

Background: The management of steroid-sensitive nephrotic syndrome (SSNS) requires treatment with high-dose glucocorticoids (GCs), but GC usage causes the most frequent form of drug-induced osteoporosis. The aim of our study was to evaluate the impact of GCs on bone mineralization in patients with SSNS using two diagnostic tools, dual-energy X-ray densitometry (DXA) and quantitative ultrasound (QUS), and to compare the diagnostic efficacy of these two imaging tools. Methods: A total of 30 children with SSNS (age 5.20 ± 2.20 years) were evaluated at the start (T0) and after 1 (T1), 2.44 ± 0.75 (T2, 18 patients) and 5.96 ± 2.33 years (T4, 12 patients) of GC treatment. Patients who stopped at T2 were also evaluated at the 1-year timepoint after ceasing GC treatment (T3). Results: Of the patients assessed at T2, 11 had bone mineralization at the lower limit of normal versus those at T0 and T1, with bone mineralization rescue at the 1-year timepoint after GC discontinuation. At T4, 6/12 patients had densitometric parameters at the lower limit of normal values, and 3/12 patients showed reduced bone mineralization. The parameters derived from measurements of DXA and QUS were significantly related to each timepoint. Conclusions: Patients with SSNS receiving GC therapy undergo bone status alteration related to the dosage and duration of the therapy. In terms of diagnostic efficacy, DXA and QUS were comparable, indicating that QUS is a reliable tool to evaluate bone health in children with SSNS. [ABSTRACT FROM AUTHOR]

Epidermolysis bullosa (EB) consists of a group of dominant or recessive autosomal diseases characterised by skin and mucosa fragility. The lesions leave erosions and scars that, in turn, can cause stenosis of tracheal, oesophageal, and genitourinary tract mucosae. The significantly increased survival of EB patients has determined the onset of complications never observed before, including genitourinary disorders such as hydroureteronephrosis, recurrent urinary tract infections, renal amyloidosis, IgA nephropathy and post-infectious glomerulonephritis. A 6-year-old boy diagnosed with recessive dystrophic EB Hallopeau–Siemens type (RDEB-HS) was referred to our clinic because of microhaematuria that evolved into intra-infectious macrohaematuria. Renal biopsy revealed an increase in both extracellular matrix and mesangial cells, with a focal segmental glomerulosclerosis with severe chronic tubulointerstitial damage. Immunofluorescence showed IgA mesangium deposits. Five years later, he was started on haemodialysis, because of worsening renal function. This is a rare case of a child with EB who was successfully treated with haemodialysis. The pertinent literature has been reviewed. [ABSTRACT FROM AUTHOR]

The aim of this study was to investigate the physiopathology of isolated or coexisting ureteropelvic junction obstruction (UPJ-O) and high-grade vesicoureteral reflux (VUR), including the clinical characteristics and management.The association between UPJ-O and VUR was reported more frequently in boys with high-grade VUR; however, the physiopathology of concomitant UPJ-O and VUR is still unknown. Primary pyeloplasty, followed by ureteral reimplantation, if needed, has been widely accepted, although VUR should be treated first (most often by endoscopic treatment) in the presence of a functional obstruction.We reviewed the charts of 78 children with isolated or coexisting high-grade VUR/UPJ-O. Among the children, 14 had isolated UPJ-O, 16 had high-grade VUR/UPJ-O, and 48 had high-grade VUR. Children with other urological or extrarenal conditions were excluded.Patients with isolated UPJ-O showed significantly different clinical characteristics compared with the other two groups of patients with high-grade VUR. Among the patients of group 2, 3/13 (23%) showed progression from functional to obstructive UPJ-O after endoscopic treatment. All of them underwent secondary pyeloplasty, which was complicated at follow-up by VUR recurrence needing further endoscopic injection.We suggest that UPJ-O in high-grade VUR patients is just a complication of severe VUR that produces structural changes in predisposed children. The treatment of children with associated high-grade VUR/UPJ-O may be complicated by the progression of urinary flow obstruction or VUR recurrence after pyeloplasty. Endoscopic treatment of high-grade VUR is associated with a high rate of VUR recurrence in children requiring subsequent pyeloplasty. [ABSTRACT FROM AUTHOR]

Levels of von Willebrand factor antigen (vWf: Ag) and factor XIII activity (F XIII) were studied in relation to the severity of clinical symptoms (scored from 0 to 3) and to immunological parameters [IgA, C3, C4, and circulating immune complexes (CIC) in 16 children (7 males, 9 females, aged 3-11 years) with Henoch-Schonlein purpura (HSP) at presentation. vWf: Ag was increased in 7 patients, F XIII activity was decreased in 6. In all children we found high levels of IgA, while C3 and C4 levels were normal; CIC were elevated in 11. vWf: Ag correlated with clinical score and with IgA and CIC, probably as a result of immune-mediated endothelial cell damage. The haemostatic alterations observed in HSP are important for understanding the pathophysiology of the disease. [ABSTRACT FROM AUTHOR]