RESUMEN
Idiopathic steroid-resistant nephrotic syndrome [ISRNS] is rare and represents a significant therapeutic dilemma for paediatricians and paediatric nephrologists.To analyze characteristics of the ISRNS in the child. Retrospective study of 20 cases of ISRNS enrolled in paediatric department of nephrology in Sahloul hospital [Tunisia] between June 1993 and December 2007 [14 years period]. There were eight girls and 12 boys [mean age: 5.8 +/- 3.7 years] originating from the center or the south of Tunisia. Eight of them had a minimal-change disease [MCD], 11 a focal and segmental glomerulosclerosis [FSGS] and one a mesangioproliferative glomerulonephritis [MePGN]. In this group, no family form could be identified. All patients were treated by cyclosporine associated with low dose of steroid. We noted a complete remission [CR] in nine cases, partial remission [PR] in three cases and no response to cyclosporine in eight cases. Among patients with CR, six presented MCD and three a FSGS. In this group, we observed relapse of nephrotic syndrome in six cases. End stage renal disease [ESRD] was noted in 10 patients of which five not responded to cyclosporine, two initially having presented a RC and three having since the beginning a PR. Among them, two only could be grafted; one relapses on transplant was observed with a single patient initially presenting a secondarily transformed MePGN in FSGS. Our study confirms the clinical, histological and evolutive heterogeneity of idiopathic steroid-resistant nephrotic syndrome. Although there is any therapeutic consensus in this domain, cyclosporine remains indicated in first intention in sporadic forms of ISRNS. On the other hand, renal transplantation constitutes the only therapeutic alternate in genetic forms that constantly evolve at ESRD
RESUMEN
In spite of its rarity, the haemolytic and uremic syndrome [HUS] constitutes the first aetiology of acute renal insufficiency [ARI] in child. The aim of this work is to analyze clinical and evolutive aspects of the HUS in child. We studied retrospectively 17 cases of HUS in child enrolled in the paediatrics' department of Sahloul Hospital during eight years period [1996 to 2003]. It is about four boys and 13 girls [sex - ratio= 0.3] aged three months to nine years [mean age: 32 months]. Typical HUS was observed in eight child and atypical HUS in the nine others which three presenting a familial form and one associated with steroid resistant nephrotic syndrome. Diagnosis of HUS was established on the classic triad of the disease [anaemia, thrombopenia and ARI] and/on by the histology. Extra-renal manifestations [neurological or digestive involvement] were observed in 11 patients. A blood transfusion was indicated in 13 patients presenting severe anaemia. Peritoneal dialysis was indicated for nine patients while three others required haemodialysis because renal insufficiency had evolved quickly to the end stage. Thirteen cases of HUS [eight typical and five atypical] have received plasma therapy during two to five days. The short-term evolution was favourable with recuperation of normal renal function in seven cases [five with typical SHU and two with atypical SHU]. Three children developed terminal renal insufficiency and were currently in haemodialysis. Five patients [four cases of atypical HUS and one case of typical HUS] died of the continuations of the ARI and/or nosocomial infection. The HUS remains a serious illness because of the risk of complications that can occur to short and long-term. Currently, the specific treatment is only recommended in patients presenting an atypical form of HUS
Asunto(s)
Humanos , Masculino , Femenino , Niño , Estudios Retrospectivos , Anemia , Trombocitopenia , Lesión Renal Aguda , Diálisis Peritoneal , Diálisis RenalRESUMEN
Renal involvement is one of the most severe and frequent manifestations of the systemic lupus erythematosus [SLE]. In this study, we analyzed clinical and evolutive particularities of 14 paediatric cases of lupus nephritis [LN]. It's a retrospective study in 14 children with lupus nephritis followed-up in the paediatrics department of Sousse and Mahdia between 1983 and 2004. There were 12 girls and two boys [sex - ratio = 0.16] aged four to 14 years [mean age =10 years]. At the first presentation, we noted proteinuria in all patients with nephrotic syndrome in six cases, hypertension with variable severity in five cases, hematuria in six cases and a variable severity of renal insufficiency in six cases. Histological examination of kidney performed in 10 patients with severe nephropathy, revealed class IV glomerulonephritis in four cases, class V in two cases and class III in four cases. Thirteen patients were treated by corticosteroids associated with immunosuppressive agent in six cases. One patient had not received any treatment. Five patients were dead of the continuations of SLE complications or immunosuppressive therapy. For the other patients, one is in clinical and biological remission since six years, four are lost of view, one is in end stage renal failure, two presented relapsing evolution and one presents refractory form of LN. Lupus nephritis is severe in our patients with predominance of class III and IV. New therapeutic strategies permitted to improve the renal survival but at the cost of an important iatrogenic morbidity