[Predictive factors of severe henoch-schonlein nephritis in children: About 34 cases]

Henoch Schonlein Purpura is the most frequent vasculitis in children. Renal involvement is variable. Renal manifestations vary from isolated microscopic hematuria to the association on nephrotic syndrome to nephritic syndrome. To determine the predictors of severe Henoch - Schönlein nephritis. Retrospective study over 15 years [1996-2010] of 34 chidren, with henoch-schonlein nephritis. Renal involvement was determined in 68.7%. Mean age was 7.23 years [3-14 years]. Renal manifestations were variable. Moderate renal manifestations were noted in 15 cases. Microscopic hematuria was observed in 23.5% of cases and moderate proteinuria with or without hematuria is noted in 20.5% of cases. Severe nephritis was noted in 18 cases: nephrotic syndrome in 29.5% and nephrotic syndrome associated to nephritic syndrome in 23.5%. Hypertension without urinary anomalies was observed in one case. In univariate analysis, factor predictive of severe nephritis were: male sex, macroscopic hematuria, biologic inflammatory syndrome and leukocytosis. In multivariate analysis, only the leukocytosis was predictor of severity. In our study, only leukocytosis was predictor of severity in henoch-schönlein nephritis

Primary hyperoxaluria type 1 in Tunisian children

Primary hyperoxaliuria type 1 is an autosomal-recessive disorder characterized by increasing urinary excretion of calcium oxalate, recurrent urolithiasis, nephrocalcinosis, and accumulation of insoluble oxalate throughout the body. This inborn error of metabolism appears to be a common cause of end stage renal disease in Tunisia. To review the clinical, biological and radiological futures of primary hyperoxaluria type 1 and to correlate these aspects with the development of end-stage renal disease. we retrospectively reviewed 44 children with Primary hyperoxaliuria type I who were treated in our department during a period of 15 years between 1995 and 2009. The diagnosis was established by quantitative urinary oxalate excretion. In patient with renal impairment, the diagnosis was made by infrared spectroscopy of stone or by renal biopsy. Male to female ratio was 1.2. The median age at diagnosis was 5.75 years. About 43% of those were diagnosed before the age of 5 years. Initial symptoms were dominated by uraemia. Four patients were asymptomatic and diagnosed by sibling screening of known patients. Nephrocalcinosis was present in all patients. It is cortical in 34%, medullary in 32% and global in 34%. At diagnosis, twelve children were in end-stage renal disease [27%]. Pyridoxine response, which is defined by a reduction in urine oxalate excretion of 60% or more, was found in 27%. In the majority of patients, the clinical expression of Primary hyperoxaliuria type 1 is characterized by nephrocalcinosis, urolithiasis and renal failure. Pyridoxine sensitivity is associated with better outcome

Histopathological spectrum of childhood idiopathic steroid-resistant nephrotic syndrome in Tunisia

In children, renal biopsy is routinely required in the management of idiopathic steroid-resistant nephrotic syndrome particularly prior to starting nephrotoxic immunosuppressive agents. To investigate the correlations between the results of initial renal biopsy in Tunisian children with idiopathic steroid-resistant nephrotic syndrome and the subsequent response to cyclosporine-prednisolone combination. We conducted a retrospective study of children with idiopathic steroid-resistant nephrotic syndrome over the period 2002- 2009. Data on clinico-biological features, histological diagnosis and response to cyclosporine-prednisolone were collected. Thirty patients were enrolled, of whom 16 had focal segmental glomerulosclerosis, eight had minimal change disease and six had diffuse mesangial proliferation. Complete Remission was achieved in 15 patients [50%]. Nine patients [30%] went into partial remission. Only six patients presented no response [20%]. No statistically significant relationship between the different pathological types and the response to CsA-prednisone was found. In our study, two important facts were noted: 1] the predominant histopathological subtype was the focal segmental glomerulosclerosis; 2] a high remission rate was achieved in our patients using a combined cyclosporine-prednisolone treatment regimen. This response is not dependent on the histological type

[Uropathies and nephropathies in the Bardet-Biedl in child]

The Bardet-Biedl syndrome is an autosomal recessive disease, characterized by obesity, retinal degeneration, hypegenitalism in men, polydactylism and on often moderate mental retardation. With these cardinal features, others clinical findings [secondary features] including diabetes, congenital heart defects, hypertension or syndactyly can be seen. Renal involvement is almost constant, but varies from a moderate impairment of the tubular functions to chronic renal failure caused by malformative uropathy or glomerulopathy. Report a new cases. We report 6 patients with Bardet-Biedel syndrome who had renal involvement. Three patients had cystic dysplasia, one patient an increased fractional sodium excretion, one other a vesico-ureteral reflux and the last patient developed end-stage renal failure following acute post streptococcal glomerulonephritis. We insist on precocious diagnosis and multidisciplinary treatment of these renal lesions, to avoid or, at least, to slow down the evolution to the terminal renal failure, essential prognosis factor. Renal involvement, is considered as a major criteria predicting high morbidity and mortality during Bardet-Diedl disease

[Urinary tract infections in children following renal transplantation: a single-center experience]

Urinary tract infection [UTI] is the most common complication after kidney transplantation and represents a potential life-threatening risk for the immuocompromised child. The aim of this report is to determinate incidence, risk factors, microbilogic features and evaluate the impact of this complication on graft outcome and patient mortality. We performed a retrospective cohort study reviewing the medical records of 17 children from 38 who received a renal transplant in our center between January 1992 and June 2008 and who present an urinary tract infection. All patients received Lich-Gregoire implantation and insertion of double-J stunt. Antibioprophylaxis was not systematic. After a mean period of 6 years, 9 children [5 boys+4 girl] developed early UTI [during the first month after transplantation] and 5 [3boys + 2 girl] had late UTI. Three patients [2boys+1girl] with an indeterminate nephropathy developed early and late UTI. Causal agents are: E.Coli, Klebsiella Pneumoniae and Candida albicans. The further voiding cystourethrography showed a vesico-ureteral reflux on graft in 5 cases. Among the 17 patients, 4 lost their graft and are actually on haemodialysis. The urinary tract infection represents the major complication after renal transplantation. Diagnosis ant treatment must be made early to avoid the loss of the graft