ABSTRACT
Objective: Juvenile myasthenia gravis (JMG) is a rare disease with possible severe forms. Thymectomy is supported by many authors particularly in generalised forms with positive AcetylCholine Receptor Antibody (AChR- Ab). The aim of our study was to discuss the indication and the outcome of thymectomy in five children with JMG. Methods: We performed a retrospective study of the medical files of patients with JMG, who were hospitalised in our pediatric surgery department for thymectomy. For all the children we performed Acetylcholine Receptor Autoantibody (ACHRAB) test-system producer, a chest x-ray, a chest Computed Tomography Scan (CT-scan) and a Magnetic Resonance Imaging (MRI). Thymectomy was indicated on the presence of anomalies on CT-Scan or MRI and realised via either thoracotomy or video-assisted thoracoscopy. The study was conducted with the approval of the ethics committee at our institution. Results: Based on our findings, thymectomy in JMG with imaging anomalies seems to be associated with clinical improvement. In our patients complete remission after surgery was seen in one case, a clinical improvement with a decrease of the medical therapeutic dosage was noted in three other cases. One patient could not be evaluated because of non adherence to medical treatment. Surgery by thoracotomy or video assisted thoracoscopy (VAT) was also well tolerated by all the patients. Conclusion: The benefits of thymectomy in children with JMG are still controversial. However, many researchers have reported a sustained improvement of symptoms in the majority of patients after thymectomy. Our findings seem to encourage thymectomy in cases of JMG and particularly when imaging anomalies are found. VAT thymectomy was well tolerated and it is considered by many researchers to be the standard method for thymectomy in children.
ABSTRACT
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
ABSTRACT
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
ABSTRACT
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