[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

[Digestive manifestations of hyperoxaluria]

Primary hyperoxaluria is a rare hereditary disease caracterised by deposits of calcium oxalate in different tissues, essentially the kidney. The digestive localization was exceptional. We reported two childs who are aged respectively 13 and 16 years; they are admitted for end stage renal failure caused by primitive hyperoxaluria. The diagnosis was suspected because there were consanguinity, nephrolithiasis in the two cases and nephrocalcinosis in one. It is confirmed by increase oxalic and oxaluric levels. The hemodialysis was started. After some years, the two patients developed crystal deposition in her digestive wall

[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

[Renal osteodystrophy in children: about 26 cases]

The authors reported 26 cases of osteodystrophy in witch 10 girls and 16 boys aged between 3 and 22 years. 9 patients are asymptomatic, 5 have bone pain and 6 with lower member distortion. The hypocalcaemia is recovered at 7 patient, the hyperphosphoremia at 17 and the increase of the alkalin phosphatases at 20. The PTH is constantly increased. The x-ray standard shows geodes in 8 cases, a reduction of the transparency in 9. All patient received substitutive treatment. The evolution is marked by a stabilization or improvement of lesions in 20 cases and an aggravation at 6

[Atypical haemolytic uremic syndrome associated with steroid-resistant idiopathic nephrotic syndrome]

The association of atypical hemolytic uremic syndrome [HUS] with idiopathic nephrotic syndrome has been exceptionally described and its physiopathology is not yet understood

Observation: We report the observation of a four year and two month old boy descended of consanguineous parents which have since the age of three years a steroid-resistant idiopathic nephrotic syndrome with minimal glomerular lesions treated favorably by cyclosporine A during 12 months but whose ulterior evolution was marked by relapse of the disease and after a few months by the installation of an atypical hemolytic uremic syndrome and afterwards a terminal renal insufficiency

Conclusion: Idiopathic nephrotic syndrome may constitute the terrain of an atypical HUS. Its physiopathology, complex and not yet clarified, does probably incriminate an infectious factor and/or a genetic predisposition that remains to confirm

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

[Post infectious extracapillary glomerulonephritis: about 8 paediatric cases]

We reported 8 cases of post infectious extracapillary glomerulonephritis enrolled in the pediatrics department of Sousse during 10 years, diagnosed histologically. It is about 4 girls and 4 boys aged 3 at 13 years. Clinically, all patients had oedema and haematuria. Hypertension was present in 6 cases; 7 children were anuric. All patients have renal failure, associate at nephrotic syndrome in 6 cases. The ASLO titres are elevated in 7 patients. Renal biopsy showed diffuse crescents in 6 cases and circumferential cresents in 3. 7 patients well treated by corticoids and immunosupresseurs. The outcome is good in 4 cases

[Purulent pleural effusion in children. About 47 cases]

The authors report retrospectively 47 cases of purulent pleural effusion, collaged in paediatrics department CHU Sahloul Sousse in 11 years [1994-2004]. It's about 30 boys and 17 girls. The symptomatology was dominated by fever, cough and dyspnea. The bacteria is identified in 18 cases: staphylococcus in 11 cases, streptococcus pneumonia in 3 cases and haemophilus in 3. The treatment was based in double antibiotherapy associated with kinetherapy in all cases; the drainage was required in 15 cases. The outcome is good in 38 cases; 7 cases of sequelle were noted. Two children were dead

[Out come of steroid resistant and steroid dependant nephrotic syndrome traeted by cyclosporine]

We study retrospectively 9 cases. We colliged 5 boys and 4 girls aged between 2 years 3 months and 13 years how have steroid resistant nephrotic syndrome in 6 cases and steroid dependant nephrotic syndrome in 3 cases.The renal biopsy show minimum lesion in 2 cases, segmental glomerular sclerosis in 6 cases, and proliferative glomerulonephritis in one. All this patients received cyclosporine.The out come is good in 5 cases: 4 enfants have complete remission, one have tow relapse steroide sensible. Two enfant were ciclodependant and need prolangation of the treatement and two were resistant

[Familial hyperchylomicronemia associated with diabetes about one case]

In this study, we report one case of a 10 years old girl presenting hyperchylomicronemia colliged in department of pediatrics CHU Sahloul Sousse. The principal clinical symptoms were abdominal pains and splenomegaly. Eruptive xanthomata was not found. Biologically, we noted a high level of triglyceride [40.2 mmol/l] and cholesterol [10.6 mmol/l]. Lipoprotein electrophoresis showed an hyperchylomicronemia. The post heparin lipolytic activity is decreased. Molecular basis showed a new mutation in intron 6: substitution of cytosin by adenin. Five years later she is presenting diabetis. The outcome was good