[Juvenile autoimmune nyasthenia gravis analysis of 8 patients]

Juvenile myasthenia gravis is a rare disease in childhood. It can be dangerous by the possibility of respiratory failure which is the dread-ful complication in myasthenia gravis. We studied 8 childs with myasthenia gravis [there were a familial form with 2 sisters], followed at the Pediatrics departments of Sahloul and Farhat Hached University Hospitals, during 23 years [since 1982 to 2004]. All patients, were with the generalized form, moderate or severe disease. The median age at diagnosis moment were 8 years and a half. The anticholinesterase test was positive in all cases, EMG with repetitive nerve stimulations showed a decremental response in 4 patients. Seropositivity to acetylcholine receptor antibodies was found in 5 patients. Treatment of juvenile myasthenia gravis was based on anticholinesterase drugs. Steroids and immunosuppressive medications were associated in the severe form. Thymectomy was indicated in the thymic abnormalities form. The follow-up of our patients found a complete remission in 4 cases, an improvement in 3 cases and a death in one case by myasthenic crisis

[Congenital tracheal stenosis about a case report]

The congenital tracheal stenosis is a very rare malformation in the child and it is potentially lethal. The diagnosis is difficult and we note the absence of therapeutic standardisation. We report a new observation of a 4 months old child, presenting since the birth a wheezing with accesses of polypnea and cyanosis, with progressive aggravation. He was hospitalized for acute respiratory distress. The physical examination found an eutrophic infant, with a fever at 38°C, polypnea, and a stridor with sibilants at the pulmonary sounding. The initial diagnosis was a severe bronchiolitis and the child had a symptomatic treatment with respiratory kinesitherapy. The evolution was marked by the persistence of an intense dyspnea with expiratory stridor. A laryngotracheoscopy found a larynx of normal aspect but presence of a tight tracheal stenosis located at 1cm of the hull and extended on 1 cm heigh. A helicoid thoracocervical CT scan confirmed the tracheal stenosis and eliminated an extrinsic compression tracheal. The initial therapeutic attitude consisted on several attempts of installation of a tube T of Montgomery but without success. The child was maintained in artificial ventilation by tracheotomy for 3 months. A surgical treatment [resection anastomosis of the tracheal stenosis] was made in front of the absence of other therapeutic alternatives. A control by CT scan [3 mois post operative] didn't show any abnormalities. The congenital tracheal stenosis requires an early diagnosis and a multidisciplinary treatment

[Clinical and evolutive features of invasive pneumococcal infections in child abot 10 cases]

Currently, pneumococcal infections are an important public health problem in Tunisia and through the world. The aim of this study is to analyze clinical and evolutive profile of penicillin-resistant pneumococci in children in our region. We studied retrospectively 10 cases enrolled in the paediattics department of Sousse during an 11 years period [1993-2003]. There were eight boys and two girls [sex-ratio 4] aged from 5 to 42 months [mean age: 13 months]. Four children were carriers of purulent meningitis, three a pneumonia, two an osteo-articular infection and one an isolated septicemia. The pneumococcus was isolated in pathological products and the oxacillin disk screening test detected all penicillin-resistant isolated germs The survey of penicillin sensitivity was completed by determination of minimal inhibitory concentration [MIC] by E. Test [R] every time that the diameter of inhibition was lower to 26 mm. After antipneum000ceal antibiotics treatment adapted to infections sites, six patients presented severe complications and two among them died, Frequency gravity and resistance of pneumococcal infections give a renewal interest for antipneumococcal vaccination in patients with risk

[Clinical and evolutive features of invasive pneumococcal infections in child. About 10 cases]

Currently, pneumococcal infections are an important public health problem in Tunisia and through the world. In order to analyze the epidemiological, clinical and evolutive profile of penicillin-resistant pneumococci in children, we studied retrospectively 10 cases enrolled in the pediatrics department of Sousse during 11 years period [1993-2003]. There were eight boys and two girls [sex - ratio = 4] aged 5 to 42 months [mean age: 13 months]. Four children were carriers purulent meningitis, three a pneumonia, two an osteo-articular infection and one an isolated septicemia. The pneumococcus was isolated in pathological products and the oxacillin disk screening test detected all penicillin-resistant isolated germs. The survey of penicillin sensitivity was completed by determination of minimal inhibitory concentration [MIC] by E.Test[trademark] every time that the diameter of inhibition was lower to 26 mm. After antipneumococcal antibiotics treatment adapted to infections sites, six patients presented severe complications and two among them were died. Frequency, gravity and resistance of pneumococcal infections give a renewal interest for antipneumococcal vaccination in patients with risk

Precocious puberty and hypothalamic hamartoma

Central precocious puberty is exceptionally due to hypothalamic hamartoma in girls under one year of age. A small girl hospitalized for exploration of precocious puberty started since the age of 7 months. Exploration of the hypothalamic-pituitary axis confirmed the central origin of this puberty. Magnetic resonance imaging [MRI] showed a small pediculate hypothalamic hamartoma [HH]. Evolution under triptorelin [recoil of 6 years] was favourable with notably the secondary sexual characters disappearance, the slowing of the speed of growth and the bony maturation. Central precocious puberty must make search HH even in girl. Its pathogenesis again controversial, seem to be in this age probably multifactorial. Our observation illustrates the efficiency of long-acting GnRH agonists during the central precocious puberty due to HH even in infantile form