[Primitive acute polyradiculo-nevritis in child: the Kairouan pediatric department experience. about 22 cases]

Guillain-Barre Syndrome [GBS] is an acute polyradidulonevritis which is primitive inflammatory and demyelinisant. It represents the most frequent cause of acute peripheric paralysis of the child. To study the epidemiologic, clinic, electromyographic, outcome and therapeutic features of this disease. 22 cases of GBS were reported in the pediatric department of Ibn El Jazzar's hospital of Kairouan from January 1990 to September 2009. The GBS represents a hospital frequency of 0.45%o. The mean age of the patients was 6.88 years with a sex ratio of 1.2. The prodromic infectious manifestations were observed in 54.5% of cases. The clinic symptomatology was the muscular deficiency observed in all cases with absence of deep reflex an albumincytologic dissociation was observed in 12 cases. The electromyographic manifestations were: an axonal disorder in four cases, axonomyelinic in eight cases and myelinic in seven cases. A specific therapy by intravenous polyvalent immunoglobulin was prescribed for 14 patients. The evolution was favourable in 10 cases with total recovery three cases of drop foot gait were observed, seven patients were lost to follow up and two patients are dead. GB syndrome is the most frequent cause of child acute primitive distal paralysis, since acute polio has been eradicated. Acute Respiratory disorder is the most severe complication this syndrome can lead to in 5% of cases. The course of the disease is often mild and severe scars are only encountered in 5 to 10% of cases. Indeed, the use intravenous immunoglobulin has utterly changed prognosis

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

[Clinical feature and course of Kawasaki disease in central Tunisia: a study about 14 cases collected over a period of three years[2000-2002]]

To analyze the clinical features and course of Kawasaki disease in central Tunisia. We studied retrospectively 14 cases of children with Kawasaki disease collected in Tunisian center during three years [2000-2002]. The study is about II boys and 3 girls [sex - ratio: 3.6/I] aged from 6 months to 8 years [mean age: 4 years]. Twelve patients had at least 5 diagnostic criteria of the illness, the two others had an incomplete form. We noted cardiac complications in seven patients treated belatedly, beyond 10 days of progression, because of atypical clinical presentations. All patients had all a middle caliber coronary aneurysm that was complicated by a thrombus in three cases, associated with pericarditis and minimal mitral insufficiency in a case and with a cardiac rhythm disturbance [block of branch] in another cease. Besides the cardiac complications, several other visceral manifestation could be noted: joint symptoms in five cases, GI tract symptoms in three cases, neuro-meningeal in two cases and urinary tract symptoms in two other cases. Specific treatment [aspirin with anti-inflammatory dose and intravenous immune globulin [IVIG]] has been instituted in all patients. The course was favorable for 12 patients with fast regression of clinical manifestation and progressive normalisation of biologic values. Two patients did not respond to the initial IVIG treatment, and had to recense received an additional course of IGIV but without clinical nor biological improvement. These two patients were treated with corticosteroids. Cardiac lesions disappeared completely in all patients even for those with thrombosis and in patients with IVIG-resistant Kawasaki disease. Only one patient had kept neurologic sequellae: aphasia, bevavioral problem's and partial epilepsy. Kawasaki disease is not rare in our region. Incomplete or atypical presentations are frequent and are a source of diagnostic delay. Coronary aneurysm due to the delay of treatment often regresses even in patients with IVIG-resistant Kawasaki disease