Focal segmental glomerulosclerosis in children

Background: Focal segmental glomerulosclerosis [FSGS] represents 20% of nephrotic syndrome in children. The clinical course and prognosis is heterogeneous in children. The aim of this study was to analyze treatment and outcome of children with FSGS

Methods: This retrospective study was conducted in the Department of Pediatrics in Charles Nicolle Hospital during a 15-year period [1996-2010]

Results: There were 30 children, 16 boys and 14 girls. The mean age was 7 +/- 4 years. Nephrotic syndrome was observed in 26 patients, hematuria was noticed in 2 patients and renal insufficiency was detected in 2 patients at presentation. FSGS, not otherwise specified, was the predominant variant. All patients with nephrotic syndrome were treated with steroids. Only three patients responded to it. Twenty one patients were treated with cisclosporin A and this resulted in a 57% complete remission and a 24% partial response. Cyclophosphamide was administered to 6 patients and engendered a 50% complete remission. Six patients were treated with mycophenolate mophetil and showed no response in all cases. Renal insufficiency has been developed in 12 children

Conclusion: Results from this study showed that the majority of children with FSGS achieve a high sustained remission rate with ciclosporine A

[IL1/IL1 Ra, DTLA-4 and Apol/Fas genes polymorphisms and susceptibility to IgA nephropathy in Tunisian patients]

The IgA nephropathy [IgA-N] is considered the most common form of primary glomerulonephritis and its pathogenic mechanisms are very complex. The study of several genes which encode for immunoregulator molecules in inflammatory and immunological responses during the disease, allowed to describe some number of polymorphisms would be involved in the molecular expression, the road marking, the synthesis and/or the binding to the receptors. So an abnormality of the molecular function associated with its polymorphism would be suggested in the genetic predisposition to the disease. To determine interleukin 1 [IL1], interleukin1 receptor antagonist [IL1 Ra], CTLA-4 and Apol/Fas genespolymorphisms frequencies in IgA-N in order to estimate the impact of these polymorphisms in the disease susceptibility. The polymorphism of a single nucleotide [SNP] at [-889] IL1 alpha of 21 IgA-N patients and 100 healthy blood donors, as controls, was studied by PCRSSP. The SNPs of the IL1 beta [+3954], CTLA-4 [+49] and l'Apol/Fas were analyzed by PCR RFLP and finally the polymorphism of the IL1 Ra gene was determined by a PCR VNTR [variable number tandem repeat]. Investigation of IL1 alpha/beta and Apol/ Fas polymorphisms showed no differences in genotypes and allelesfrequencies between IgA-N patients and controls. However, genotype AA of CTLA-4 exon 1 [+49] was significantly higher in patients [47.62%] than in controls [9,1%] p<0.001. Nevertheless, the clinical histological and biological characteristics of IgA-N were similar in AA CTLA-4 genotype patients compared to AG or GG genotype patients. We fund also, a significant increased frequency of 1/1 IL1 Ra genotype in IgA-N patients [95,24%] compared to controls [54%] [p<0,001] [p<0,001]. We conclude that the susceptibility to IgA-N seems to be associated with the presence of CTLA-4 AA and iL1 Ra 1/1 genotypes in Tunisian population. However, the lack of association between IL1 alpha/ beta and Apol/fas genes polymorphisms should be further investigated by large population based studies

[Renal involvement in the Behcet's disease]

Renal involvement in the Behcet's disease is rare. The clinical features vary from urinary sediment's abnormalities to ESRD. We propose to study the clinical, biological and histological data, the therapeutic management and the prognosis of patients. We report a retrospective study including 8 patients representing 1.23% of cases. The average age of the patient was of 37 +/- 12. 35 years with a clear male prevalence. Urinary signs were discovered fortuitously by the strips in the majority of the cases after an average of 18 months. It's about proteinuria and hematuria. Renal insufficiency and hypertension were rare. Pathological study highlighted 3 cases of amyloidosis, 2 cases of IgA nephropathy, 1 case of minimal change disease, 1 case of endo and extracapillary glomerulonephritis and 1 case of interstitial nephropathy. Patients having GN were treated by corticoids and immunosuppressive agents and those having an interstitial nephropathy were treated symptomatically with good evolution in the majority of the cases. Only one patient is dead, he had amyloidosis. Prognosis depended on the precocity of the diagnosis, the histological type and the treatment. The renal involvement during Behcet's disease is rare. Amyloidosis and Ig A nephropathy are the most frequent. Treatment is still controversial

[Peritoneal dialysis in elderly]

Peritoneal dialysis is used more and more as treatment of substitution of the end stage renal disease at the old subjects of more than 65 years and its effectiveness and its good tolerance were shown. To study the epidemiological and clinical profile of these patients, the indication and the advantages of PD, the various complications, the elements of prognostic and the survival of the patients and the technique. We made a retrospective study including a series of 13 old patients of more than 65 years and treaties by PD during the period extending from the 1983/2/11 to the 2005/12/31. They are 10 men and 3 women on average of 70 +/- 3, 1 year and representing 3, 62%of the totality of the patients. The diabetic and vascular nephropathies represent the first cause of ESRD. The PD was used of first intention at 53,84%of the patients whose majority suffers from a malnutrition due to advanced age, anorexia, psychological disorders, bad dental state and uraemia. The cardiovascular complications are frequent in this age bracket explaining heavy morbidity. The pulmonary and urinary infectious complications are also frequent. The more frequent peritonitis compared to the literature, are comparable between the 2 age brackets