[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

[Lung abscess revealing a congenital cystic adenomatoid lung malformation]

Congenital cystic adenomatoid lung malformations [CCAM] are, todays, more frequently diagnosed during the pregnancy thanks to progresses in ultrasonographic explorations. On occasion, CCAM may remain asymptomatic and be discovered after being complicated by infection. This was the case of our patient. Amani _ was delivered by spontaneous vaginal delivery at term after a well-controlled pregnancy. She had good staturo-ponderal and psychomotor developments. When she was eight-year-old, she was treated for right pneumonia. Three years later, she was admitted in a general pediatrie unit because of right lower lobe abscess. Thoracic computed tomography, indicated since the infection was recurrent in the same lobe and after the constatation of a persistent cystic feature on chest radiograph, revealed multiple cystic lesions compatible with CCAM. The anatomopathological study of the surgically removed lobe concludes for a cystic adenomatoid lung malformation type I. Cystic adenomatoid lung malformations are frequently revealed by recurrent infections in old children. Thoracic computed tomography is very interesting for the diagnosis when pneumonia relapses in the same site

[Wilson's disease in children - about 21 cases]

Wilson's disease is a familial affection, described the first time in 1912, which has an autosomic recessive transmission. It is characterized by an association of hepatic cirrhosis and neurologic manifestations caused by tissular accumulation of copper. To show clinical, genetic, diagnostic, therapeutic particularities and outcomes of this disease, we carried out a retrospective study concerning three pediatric departments in the Center of Tunisia [Kairouan, Farhat Hached and Sahloul hospitals]. We have collected 21 cases during a period of 17 years [from January 1983 to december 2000]. The finding's age of the disease ranges from 5 to 13 years [mean age 8 years and 9 months]. The sex ratio is 1,1. Consanguinity is found in 90% of cases. Finding circumstances are dominated by edematous and ascitic syndrome [28%] and by hepatomegaly [57.14%] a jaundice [19,5%], a pallor [9.52] and neurologic disorders [19% of cases]. A hepatic cytolysis was found in 38% and a decrease of prothrombin rate in 61% of cases. The ceruloplasmin concentration is lower than 200 mg/1 in 80% and a decrease in the blood rate of copper in 75% of cases. The urinary rate of copper was measured only in 9 patients; it was increased in 8 cases. The therapy consisted in D penicillamin in the first line. Outcome was marked by 3 cases of death. 18 patients are followed up and they are treated by D penicilamin

[Canavan disease or n-acetyl aspartic aciduria [one case]]

Canavan's disease [CD] or N-Acetylaspartic aciduria, is an autosomal recessive leukodystrophy characterized by spongy degeneration of brain. The disease is an inborn error of metabolism caused by aspartoacylase deficiency resulting from accumulation of N- acetylaspartic acid in the brain. The authors report a case in a ten-month old boy who presented with developmental delay and megalencephaly noticeable afterfour months of age. Magnetic resonance imaging of the brain show diffuse white matter degeneration. The diagnosis of CD was confirmed by nuclear magnetic resonance spectroscopy and gas chromatography-mass spectrometry

[Glucose transporteur type 1 deficiency: a mild form]

Glucose transporter type 1 deficiency is a rare but curable disease whose real frequency is probably under estimated. This defect is responsible df reduced glucose brain supply across the blood-brain barrier. We report a mild form of GLUT1 deficiency suspected in al 9 months old boy when low cerebrospinal fluid rate glucose had persisted several months after recovery from presumed viral meningitis. GLUT 1 deficiency should be evoked in any child with delayed milestones development or seizures of unclear cause. The sole effective treatment is based on ketogenic diet

[Listeria monocytogenes meningitis in immunocompetent children. [a case report]]

Introduction: Listeria monocytogenes is, increasingly, recognised as a cause of life-threatening disease mainly in immunocompromised persons, but it is an uncommon cause of bacterial meningitis beyond the newborn period in healthy subjects

Report: We present the case of a 10-year-old previously healthy child who was admitted at a general pediatric unit for high fever, impaired consciousness and intra cranial hypertension syndrome. Cerebrospinal fluid examination revealed 980 cells/mm3 in mixed formula [neutrophils = 50 per cent, lymphocytes = 50 per cent], decreased glycorrachia [1.5 mmol/l], increased albuminorrachia [2g/l] and normal chlorurachia. Gram's stain was negative. initial parenteral antibiotics combining cefotaxime and vancomycin didn't lead to clinical improvement. In fact, after 48 hours, the temperature was usually high and the patient remained obnubilated and, also, developed bilateral convergent strabismus. The second cerebrospinal fluid sample showed lymphocytic meningitis [125 cells/mm3, neutrophils = 5 per cent, lymphocytes = 95 per cent], decreased glycorrachia [0.5 mmol/l], increased albuminorrachia [1.2 g/l], normal chlorurachia and negative soluble antigens. Gram's stain showed Gram positive bacilli. Subsequent culture yielded Listeria monocytogenes. The patient was so treated with parenteral ampicillin for fifteen days and showed uneventful recovery. No immunocompromised conditions were identified in this patient

Discussion: In its most severe form, listeriosis is an invasive disease that affects not only immunocompromised but also immunocompetent persons in whom the neuromeningeal form is the most frequent

Conclusion: L. monocytogenes should be considered among the aetiologies of bacterial meningitis in children who do not respond initially to conventional antimicrobial treatment or who deteriorate rapidly even if they are immunocompetent and non-neonates

[Kaposi-juliusberg's syndrome complicating atopic dermatitis]

Kaposi-Juliusberg dermatitis is a severe infection of the skin caused by herpes simplex virus. It occurs, most often, in the presence of another skin disease such as eczema. We report the case of a 12 month old boy who was previously examined for atopic dermatitis treated by topic steroids. He was admitted in a general pediatric unit for a diffuse vesicular and ulcero-necrotic eruption which was extremely severe and hemorrhagic in the face. Buccal mucosis was also ill. The lesions were accompanied by high fever and wirse general state. Kaposi's dermatitis was highly suspected and the boy was so treated with parenteral Zovirax and topic product for three weeks. An uneventful recovery within one month

Group A streptococci in children with acute pharyngitis in Sousse, Tunisia

A 1-year prospective study in 2 paediatric outpatient clinics in Sousse, Tunisia, aimed to determine the presence of group A streptococci in acute pharyngitis cases and carriers, and the distribution of the serotypes and biotypes. Group A streptococci were found in 9.0% of throat swabs from 155 controls and 17.7% from 474 patients [P < 0.05]. Of 43 strains isolated from patients and submitted for typing, 15 different types were identified, the most common being M75 [14 strains; 32.5%], M9 [6 strains; 14.0%], M76 [5 strains; 11.6%] and M12 [4 strains; 9.3%]. Three strains were non-typeable [7.0%]. Biotyping of the strains showed 3 predominant biotypes: biotype 3 [n = 14], biotype 2 [n = 11], and biotype 1 [n = 7]

Study of cardiovascular disease risk factors among rural schoolchildren in Sousse, Tunisia

We undertook an epidemiological survey based on a representative sample of 793 rural schoolchildren in Sousse, Tunisia to assess the prevalence of certain cardiovascular disease risk factors. The prevalence of hypertension [11.2%], hypercholesterolaemia [2.9%], hypertriglyceridaemia [1.0%], high levels of low-density lipoprotein cholesterol [0.6%] and obesity [4.0%] showed no statistically significant difference based on sex. However, smoking [4%] showed a significant gender difference [boys: 7.3%; girls 1.2%]. The relatively low cardiovascular disease risk factor profile of Tunisian children needs to be encouraged through to adulthood. Thus a school programme of heart health promotion should be established