[Cardiovascular disorders in Kawasaki disease: results of a Tunisian collaborative study]

Kawasaki disease [KD] is the first cause of cardiopathy acquired by children in developed countries. The aim of this study is to find out, in a precise way, the epidemiological, clinical and evolutionary aspects of the cardiovascular disorders in the Kawasaki disease and to determine their potential risk factors. It is a multicenter retrospective study conducted over the laps of 10 years [1007-2006] and which was a subject of interest for five hospital-university pediatrics services, during which the total of 29 observations of KD including 16 boys and 13 girls whose ages ranged between 6 months and 12 years [average age = 4 years] were analysed. The cardiovascular disorder was noticed in 11 cases [37, 93 per cent]. It consists in a myocarditis in 4 cases, a pericarditis in 1 cas and an endocarditis in 1 other case. The coronary artery problem [6 cases] from 54, 54 per cent of the cardiovascular disorders and 20, 68 per cent of the total number of patients. The diagnosis of the coronary disorders was made between 7 and 90 days [average 24 days]. Asai score was not correlated at the risk of appearance of coronary aneurysms. 5 patients out of 6 had received veinoglobulins and 3 of them before the 10[th] day of the disease evolution. The evolution was favourable in 4 cases who developed anevrysms under 8 mm. The 2 patients with multiple and huge anevrysms were complicated with a thrombosis and myocardial ischemia in 1 case and with stenosis of the left interventricular in the other. The cardiovascular disorder in the KD is dominated by the anevrysmal coronary disorder. This conditions the short term prognosis and causes the later complications

[Cerebral pachygyria and congenital cytomegalovirus infection: about one observation]

We report a case of a new born admitted for convulsions. The cranial magnetic resonance imaging showed pachygyria. Caryotype was normal, a congenital cytomegalovirus infection was diagnosed. The children decease at the age of two months

[Chronic hepatitis B: a study of 15 cases]

The aim of this study is to describe clinical, biological, histological aspects and outcome of children with chronic hepatitis B [CHB], and to analysis treatment modalities. a retrospective study of 15 children with CHB was conducted between January 1992 and December 2003. All patients had HBs Ag> 6 months, fourteen had Hbe Ag. Initial histological examination was performed in eight cases. intrafamilial horizontal transmission was noted in five cases. Fifty three percent of patients presented symptoms: asthenia [57 percent], anorexia [1 case], loss of weight [1 case], digestive disturbance [3 cases], abdominal pain [5 cases]. Cytolysis was noted in eight patients [53, 3 per cent], they had positive Ag Hbe. Liver emzymes were increased in eight cases [53.3%], they had positive Hbe Ag, mean rate of AST was 95.6 UI/I [extremes: 56 -251], mean rate of ALT was 118,8 Ul/l [extremes: 50 - 204]. Four patients had chronic active hepatitis and four had chronic persistent hepatitis. Three patients received I'interferon a; seroclearance was noted in two patients without negativation of HBs Ag. Twelve patients didn't receive any antiviral therapy, liver emzymes were decreased in three patients, seroclearance of HBe Ag was noted in five cases and HBs Ag elimination was noted in only one patient. Chronic hepatitis B is often asymptomatic and transmitted horizontaly. Antiviral therapy must be indicated in children with viral replication and active chronic hepatitis. Large vaccination at birth reduces vertical transmission and prevalence particularly in endemic areas

[Neurological diseases in the "triple a" syndrome]

Introduction: The 3A syndrome or Allgrove syndrome is an autosomic recessive disease characterized by the association of oesophagus achalasia, alacrymie and suprarenal insufficiency. Neurological disorders can appear at any time of the course's disease and would be due to a neuropathy affecting the central nervous system as well as the peripheral nervous system

Patient and methods: From1991 to 2005, we noticed 22 cases of Allgrove syndrome. The follow-up of these patients enabled us to notify the neurological abnormalities appear later in the evolution

Results: neurological symptoms appear at any time of the course's disease. All cases were backward pupil. 8 patients had a nasal voice due to palate paralysis. 7 patients had xerostomia wich is included in the dry syndrome due to the degeneration of salivary glands. One patient had a neurological motor disorder and 3 patients had a sensitive disorders with loss of cold feeling and vasomotor disorders in the hands and feet. These symptoms appear after 4 years on average after diagnosis. An orthostatics hypotension was noticed among three patients as a dysautonomic sign

Conclusion: All of these data approve that neurological disorders appear at a variable time of the course's disease. This polyneuropathy affects the oesophagus and always leads to the recurrence of the digestives signs