[Pediatric Crohn's disease in Tunisia]

Crohn's disease occur mainly in adults. However, pediatric onset forms are not rare and have many characteristics.To study clinical, diagnostic, therapeutic and evolutive characteristics of crohn's disease in tunisian children. Retrospective multicenter study conducted in 10 pediatric departments on a period of 10 years [2000-2008]. 43 children were included. The sex-ratio was 1.68. The mean age at the onset of the symptoms was 11 +/- 2.3 years [5-16 years]. The age of onset was inferior to 10 years in 25% of the children. The delay before management was superior to 1 year in 25% of cases. The initial symptoms were dominated by diarrhea [95%]. Perineal manifestations were present at diagnosis in 30% of children and extra-digestive manifestations in 53%. Ileocolonic localization was the most frequent [46%]. The initial disease flare was moderate in 83% of cases. The treatment was medical in 77% of cases, nutritional and medical in 18.5%. Maintenance therapy was instituted in 86% of cases and consisted essentiallay in azathioprin [62%]. The mean follow-up was 3 years and 4 months. 60% of the children had at least one acute flare. During evolution, 7% of children had anoperineal surgery and 11% an intestinal resection. Crohn's disease seems rare in Tunisia. The time of diagnosis is often delayed. The management is based on immunosuppressive therapy and nutritional support

[Idiopathic steroid-resistant nephrotic syndrome in child: Study of 20 cases]

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

[Graves's disease in children and adolescent: study of seven cases]

In spite of its rarity in the paediatric age. Graves' disease constitutes the principal aetiology of hyperthyroidism in child. Our goal is to analyze the clinical and evolutive particularities of Graves's disease in children. We studied retrospectively seven cases of Graves' disease in children enrolled in the pediatrics department of Sousse during ten years period [1993-2002]. There were six girls and one boy [sex - ratio = 0.16] aged 4.5 to 16 years [mean age: nine years and one month].The diagnosis has been established clinically on the presence of classic symptoms of the illness associated to the biological and radiological findings. As part of research of possible associations with this illness, we observed solely in a case, in addition of Graves's disease, the coexistence of Down syndrome and coeliac disease, rarely described., -Among the HLA antigens predisposing the Graves's disease, we only found HLA B8 antigen in a patient. The evolution under antithyroid drug treatment [ATD] has been marked by fast disappearance of functional signs in all patients. However, biological and clinical euthyroiidism was more difficult to achieve. The treatment has been stopped in only one patient after 40 months period. Graves' disease is usually easy to recognize but difficult to treat. Radical treatments [thyroidectomy or radioactive iodine therapy] are indicated in second intention after having tempted ATD beforehand

[Uremic and haemolytic syndrome in children: Study of 17 cases]

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

[Lupus nephritis in childhood: clinical and evolutive study of 14 cases]

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

Primary hyperoxaluria in children in central Tunisia

The goal of this work is to analyze clinical and therapeutics particularities of primary hyperoxaluria in children in Tunisian centre. We studied retrospectively 15 cases of primary hyperoxaluria enrolled during 9 years period [1994-2002]. It is about 2 boys and 13 girls [sex - ratio = 4.5] aged 2 month to 13 years [mean age: 4 years]. Six patients presented the infantile form and nine the juvenile form of HP. At the moment of diagnosis, renal function was normal in one patient, moderately altered in another and severely altered in the other patients. All patients had nephrocalcinosis and 6 among them radio-opaque renal calculi associated. Diagnosis of HP was established in 11 cases by hyperoxaluria and/or important hyperoxalemia or on the data of the renal biopsy and biochemical analysis of renal calculi in 4 cases. The so-called [maghrebin] mutation [Ile244Thr] sought-after in 9 children, has cannot be identified that in 2 among them. Eight patients died of the continuations of their illness. The seven other patients again in life present a terminal renal insufficiency treated by haemodialysis. No patient could benefit from organ transplantation. Primary hyperoxaluria is a very heterogeneous disease on the plan clinic that genetic. In Tunisia where it constitutes a frequent cause of end stage renal failure, prenatal diagnosis of this disease is of a big interest