Thiamine-responsive megaloblastic anemia (TRMA) is a rare autosomal recessive disorder including megaloblastic anemia, thrombocytopaenia, diabetes mellitus and progressive sensorineural deafness. We report cases of two infants, aged respectively four and five months, hospitalized for diabetic-acido-ketosis requiring insulin therapy. Laboratory tests revealed megaloblasic anemia, thrombocytopenia and normal thiamine-levels. Neurosensorial investigations showed bilateral deafness and ophthalmic involvements. Treatment with oral thiamine normalized hematological parameters and ameliorated diabetes.

Objectives. - This report describes different clinical pictures of cystic pulmonary malformation (CPM) and problems in diagnosis.Patients and methods. - Cases of CPM between 01 January 1994 and 31 December 2004 diagnosed in our institution were reviewed.Results. - Thirty-three cases of CPM were diagnosed in 30 children. They consisted of 17 boys and 13 girls ranging from 20 days to 16 years of age at the time of the diagnosis. The CPM included: 17 cases of congenital lobar emphysema (CLE), seven bronchogenic cysts (BC), five cystic adenomatoid malformations (CAM) and four pulmonary sequestrations (PS). Three patients presented two associated lung malformations. The mean ages at the time of diagnosis varied from 2 to 88 months. The symptoms consisted of respiratory distress (n = 14, 46.6%); recurrent attacks of respiratory embarrassment (n = 6, 20%); pulmonary infection (n = 8, 26.6%) associated with haemoptysis in two cases; haemothorax (n = 1) and a chance discovery (n = 1). Radiological investigations led to the diagnosis in all cases of CLE and CAM although it contributed less to the diagnosis of BC and PS. Twenty-nine patients required chirurgical treatment involving lobectomy (n = 22), pneumonectomy (n = 2) and cystectomy (n = 8). The histopathological examinations confirmed the diagnosis in all cases and rectified the preoperative diagnosis in four cases. Except for one patient with CLE, who died a few days after a lobectomy due to acute nosocomial pneumonia, the postoperative period was uneventful in 26 children with a mean of follow-up of 24 months (4 months to 7 years). Three patients developed transient and episodic attacks of dyspnoea.Conclusion. - CPM may be responsible for many clinical. and radiological pictures that present difficulties in their diagnosis. Polymorphism is related to the type of malformation, its topography and the evolutive complications. (C) 2009 Published by Elsevier Masson SAS.

Plastic bronchitis (PB) is a rare disease, characterized by the formation of obstructive branching airways tracheobronchial casts. Commonly, PB often complicates the course of cardiac or respiratory disorders. The occurrence of PB before manifestation of the underlying respiratory disease is unusual. We report on the case of a boy, aged three years and eight months, free from underlying pulmonary disease, who presented with extensive atelectasis of the left lung during an acute respiratory tract infection. Bronchoscopy revealed the obstruction of the left tracheobronchial tree with large purulent casts. After bronchoscopic removal of the casts, the boy became asymptomatic. Initial aetiological investigations were negative. Two years later, the patient developed an asthma. (C) 2008 Elsevier Masson SAS. Tous droits reserves.

The aim of this report was to screen for celiac disease (CD) in patients with idiopathic pulmonary hemosiderosis (IPH).Patients with IPH treated at the Children's Hospital of Tunis between 1976 and 2006 were reviewed and investigated for CD, using serological and histological tests.A total of 10 children (two boys and eight girls) had IPH. The mean age at diagnosis was 3.1 years. Three had digestive symptoms and positive CD serology, which was confirmed by histological data. Clinical and radiological findings improved markedly in all CD patients with corticosteroid treatment combined with a gluten-free diet. Symptoms of IPH and CD both returned in one patient who stopped the gluten-free diet.Three of our 10 patients with IPH also had CD. These data illustrate the close etiopathogenic link between IPH and CD, and strongly suggest that CD be looked for in IPH patients, especially in those with symptoms suggestive of CD.

ont necessite une assistance nutritionnelle a type de nutrition enterale a debit constant (n=3), et une alimentation parenterale exclusive (n=3). La reintroduction du lait de vache a ete realisee au bout d'un delai moyen d'eviction de 17 mois (11 a 32 mois).

Spinal vascular malformations are exceptional in children. We report a case in 1 year-old patient due to a spinal vascular malformation which was revealed by paraplegia after lumbary punction. The diagnosis was made by MRI. Spontaneous outcome was characterised by the persistence of paraplegia.

Spinal vascular malformations are exceptional in children. We report a case in 1 year-old patient due to a spinal vascular malformation which was revealed by paraplegia after lumbary punction. The diagnosis was made by MRI. Spontaneous outcome was characterised by the persistence of paraplegia. (C) 2004 Elsevier SAS. Tous droits reserves.

BACKGROUND Abdominal tuberculosis is a rare location of this infection with a lot of diagnostic difficulties. The aim of this study was to review our experience of pediatric abdominal tuberculosis with that of the literature data. PATIENTS AND METHODS A retrospective study was conducted over a 7-year period; 10 cases of abdominal tuberculosis in children were reviewed (6 girls and 4 boys, mean age: 11 years, extremes 4-14). Eight patients enrolled in this study satisfied the following criteria: a positive culture for mycobacterium tuberculosis on samples of ascites fluid, sputum, urine, abscess puncture and/or caseating granulomas on histologic examination of biopsies obtained by endoscopy, percutaneous aspiration or needle biopsy or exploratory laparotomy. Two other patients (patients No 1 & 5) had a favorable response within 10 weeks of antituberculous therapy. RESULTS We observed 8 cases of peritoneal tuberculosis and 2 cases of intestinal tuberculosis. Extra-abdominal tuberculosis was found in 4 of those patients. Two patients had a contact with a tuberculosis positive individual. Nine children had BCG immunization. The main clinical features were abdominal distension (6 cases) and abdominal pain (6 cases). Abdominal ultrasonography visualized mesenteric lymphadenopathies (6 cases), an abdominal mass (5 cases), free (1 case) and localized ascites (1 case). Barium enema and small bowell series showed small bowel stenosis (1 case) and ileal ulcerations (1 case). Exploratory laparotomy, performed in 3 patients, showed whitish granulations and peritoneal abscesses with caseous necrosis on histology. Quadruple therapy with tuberculostatic agents was prescribed in all patients, associating isoniazid, rifampicin, pyrazinamide combined at the start of the treatment with streptomycin (7 cases) and ethambutol (3 cases). Short term outcome was favorable with no deaths. The long term outcome was complicated by persistent ascites (1 case) and the development of portal hypertension (1 case). CONCLUSION Abdominal tuberculosis remains a rare localization with a lot of diagnostic difficulties.

L'evolution clinique des meningites virales est le plus souvent benigne, la possibilite de survenue de cas graves ainsi que le potentiel epidemique rendent compte de l'importance majeure de ces affections en sante publique. Dans cette etude, une premiere partie retrospective, menee sur une periode de 3 ans dans trois services hospitaliers, a montre une incidence des meningites lymphocytaires chez l'enfant 2,4 fois plus importante que celle des meningites purulentes. La deuxieme partie prospective, a ete realisee sur 12 mois : 94 cas de meningites a formule lymphocytaire ont ete investigues par culture cellulaire a la recherche d'enterovirus ; une PCR specifique des enterovirus a ete realisee en parallele. Ce travail est une premiere approche des meningites virales en Tunisie. Nos resultats illustrent l'interet des techniques moleculaires et la necessite d'ameliorer la prise en charge diagnostique des malades atteints de ce type de pathologie. Cependant, la culture cellulaire garde une place particuliere car elle nous permet l'isolement et l'identification du serotype viral.

Despite the favourable clinical outcome in most cases, viral meningitis can cause a serious public health problem especially when several cases occur during outbreaks. The first part of this work is a retrospective study conducted in three hospitals in Tunisia and covering a period of three years. It showed an incidence of viral meningitis 2.4. The second part of the study is a prospective one, it included 94 cases of aseptic meningitis notified during a period of 12 months. Virus isolation in cell culture was performed on CSF and stool samples, using cell lines sensitive to enteroviruses. A PCR to detect enteroviruses was also used in parallel. This study represents a first approach to viral meningitis in Tunisia. It highlights the importance of a regular surveillance of the disease and the contribution of molecular methods to a more sensitive diagnostic. However, cell culture remained necessary for viral isolation and serotyping.

La toxoplasmose congénitale est une embryofœtopathie pouvant être responsable de complications neurologiques graves.

The authors report the first tunisian pediatric series of Pneumocystis carinii pneumonia in six immunodepressed HIV negative infants. All infants were admitted in intensive care unit for hypoxemic pneumonia. In all the cases diagnosis was confirmed by positive microscopy for Pneumocystis carinii in broncho-alveolar lavage fluids after Gomori-Grocott and May-Grunwald-Giemsa staining. Congenital immunodeficiency highly probable in one case, was confirmed in five cases: HLA class H deficiency (4 cases), T cell activation immunodeficiency (1 case). Despite specific treatment, 4 patients died for refractory hypoxemia. Early diagnosis and prophylactic treatment are necessary in these patients.

Les auteurs rapportent la premiere serie pediatrique tunisienne de pneumopathie a Pneumocystis carinii survenant chez des nourrissons immuno-deprimes non seropositif pour le Virus de l'Immunodeficience Humaine (VIH) Il s'agit de nourrissons ayant tous sejourne en reanimation pour pneumopathie hypoxemiante evoluant vers un syndrome de detresse respiratoire aigue. Le diagnostic est porte dans tous les cas par la mise en evidence de Pneumocystis carinii dans le liquide de lavage broncho-alveolaire apres coloration au Gomori-Grocott et au May-Grunwald-Giemsa. Le bilan immunitaire realise chez cinq de nos patients a montre qu'il s'agit d'un deficit immunitaire primitif par defaut d'expression des antigenes HLA de classe II dans 4 cas et d'une alteration de la fonction des lymphocytes T dans un cas. Malgre un traitement specifique, l'evolution est marquee par le deces de 4 nourrissons. Ainsi un diagnostic precoce et une prophylaxie chez les malades a risque s'avere necessaire.

Utilisation of new diagnosis means and particularly non invasive oues in visceral leishmaniasis can be very valuable for the biologist, the clinician as well as the patient. In this, detection of leishmania in peripheral blood, well know for VIH patients, has been applied to 37 immunocompetent tunisan children suffering from kala azar that has been shown through direct examination of bone marrow. Observed results show that culture on NNN peripheral blood medium was positive in 25 cases (67.57%). On the other side, detection of leishmania through concomitant culture of blood and marrow bone for 24 children with visceral leishmaniasis match the results in 75% of the cases. Detection of leishmania by mean of blood culture for immunocompetent children is a diagnosis mean of visceral leishmaniasis and has also an epidemiologic utility by isoenzymatic characterization of isolated leishmania strains.