[Primary lung abscess: unusual situation in child a case report and review of literature]

Lung abscess is a localized area of non tuberculosis suppurative necrosis of the parenchyma of lung, resulting in formation of cavity containing purulent materiel. It is uncommun in child. A 3 years boy was admitted with prolonged fever and dyspnea. Chest X ray showed a thick walled hydric cavitation opacity containing an air-fluid level. Chest ultrasound examination showed collection of 6,8 cm of diameter in the right pulmonary field with an air-fluid level. Hemoculture showed Staphylococcus aureus. Patient presented septic shock and surgical drainage was indicated. Histological examination confirmed diagnosis of lung abscess. Any inderlying condition was noted and diagnosis of primary abscess was made. Patient demonstrated complete recovery. He's asymptoamtic with normal chest X ray and pulmonary function after 3 years of evolution. Lung abscess represent a rare cause of prolonged fever in child. An underlying condition must be excluded to eliminate secondary abscess. Long term follow up of pulmonary function is capital

[Barakat syndrome a report of two cases]

Barakatsyndrome or HDR syndrome [Hypoparathyroidism, sensorineural deafness and renal disease] is an inherited condition. It's a very rare disease. Patients may present with tetany or convulsions due to hypocalcemia at any age. Deafness is usually bilateral and may range from mild to profound impairment. Renal disease has several manifestations. We report the cases of a girl [Ferdaous] and a boy [Taha] which are respectively of 16 years old and 6 years old. The girl only was born of intermarriage. Her brother and sister were treated for hypoparathyroidism. Both of our patients had sensorineural deafness and a nephropathy: a nephrotic syndrome in Ferdaous's case and a proteinury in Taha's case. The girl was treated for hypoparathyroidism since she was 12 while the boy was admitted in emergency for tonico-clonic seizures, reported to a profound hypocalcaemia. Hypoparathyroidism was confirmed by a very low parathormone rate. The endocrinal and cerebral radiological investigations were normal. By this case report, the authors remember the Barakat syndrome: its clinical and biological features, and its different possible progressions. The mode of inheritance is believed to be autosomal dominant. Mutations in the GATA3 gene, mapped to chromosome 10p [gene map locus 10p15, 10p15.1-p14], have been identified in several families with Barakat syndrome. Possibilities of antenatal diagnostic are offered to theses families

[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

Diabetic acido-ketosis revealing thiamine responsive megaloblastic anemia

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

[Inaugural diabetic acido-ketosis: rare endocrine complication in thalassemia child]

Diabetes is a sparse endocrine complication of f3 thalassemia major. We report the case of a 14year-old boy with beta thalassemia major, who was polytransfused without regular iron-chelation therapy. He was admitted to hospital with acidosic dyspnoea and severe deshydratation without signs of cardiac failure. Urine examination showed glucosuria and acetonuria. On laboratory exams, glycemia was 39 mmol/l with severe metabolic acidosis. The diagnosis was a diabetic acido-ketosis requiring insulin therapy. Diabetes was hardly controlled; serum ferritin level was 4500 mg/l. Serum ferritin level is the main risk factor for diabetes in patients with beta thalassemia. Thus, an adequate iron-chelation therapy can prevent this complication

[Severe acute liver failure. About 32 cases]

The current study was conducted to determinate clinical, biological and outcome characteristics of children hospitalized for severe acute liver failure [SALF] and to identify prognosis indicators in our patients. Patients hospitalized with SALF, between January 1995 and June 2005, were reviewed retrospectively. Thirty two patients, aged from 2 months to 13 years and 7 months were studied, 72 percent for them developed encephalopathy. The main causes of SALF were viral hepatitis in 31 percent of cases, toxic and drug induced liver disease in 28 percent of cases, inherited metabolic disorders in 31 percent of cases, auto-immune hepatitis in one cases and Budd Chiari syndrome in one case. The etiology of SALF remained undetermined in 16 percent of normal, serum bilirubin level above 300 micro mol/l and the grade of encephalopathy. In the absence of liver transplantation, a total of 11 patients died from sepsis or multiple organ failure. The spontaneous prognosis of severe acute liver failure is poor with an overall mortality. Preventive treatment of viral and toxic hepatitis is one of the most effective approaches

Cow's milk allergy: a study of 15 cases

Cows milk allergy [CMA] is a frequent disorder in infancy. The aim of this report is to describe the wide range of symptoms and underline particular aspects. We studied retrospectively 15 cases of CMA collected beetwen 1998 and 2003 in Medecine infantile A service. 15 infants had CMA. They were 10 boys and 5 girls, aged 15 days to 6 months. Sixpatients had a positive familial history of atopy. Patients were given cow milk by the mean age of 24 days [birth, 3 months]. Symptoms were: urticaria [n=3], rectal bleeding [n=5] associated to diarrhoea and to haematemesis [n=3], and chronic diarrhoea [n=7]. Ten patients have various symptoms atopic dermatitis [n=4], gastroesophageal reflux [n=3], two of them have esophagitis, gastritis [n=3], and recurrent wheeze [n=3]. 14 infants received extensively hydrolysed formula, and one patient requires amino acid based formula. Six patients required specific nutrition: continuious enteral nutrition [n=3], and parenteral nutrition [n=3]. All patients tolerated cow's milk protein by the mean delay of 17 months [11 to 32 months]

[Intrathoracic extramedullary haematopoiesis: a study of 1 case]

Intrathoracic extramedullary haematopoiesis [IEH] is a rare entity accompanying some chronic haematologic diseases. The authors report the case of a 16 year old beta thalassemia girl with IEH, presenting in the form of asymptomatic posterior mediastinial mass. Computed tomography scanning and magnetic resonance imaging of the chest showed multiples polylobar paravertebral masses which have the characteristics of IEM. The radiological diagnosis is essential to avoid biopsy and surgery which can be very harmful because of the extreme vascularity of the haematopoietic tissue