[Chronic granulomatous disease: clinical and outcome profiles in 20 cases]

Chronic granulomatous disease [CGD] is an uncommon primary immuno deficiency, resulting in bacterial infections produced by catalase- positive microorganism and fungal diseases. The present study describes the clinical characteristics, the infections detected in a group of pediatric patients diagnosed with CGD. A review is made of the clinical records of 20 pediatric patients diagnosed with CGD between first January 1999 and 31th December 2008. The mean age at diagnosis was 10.6 years. The diagnosis was confirmed using the nitroblue tetrazolium test [NBT]. All patients received antibiotic prophylaxis with cotrimoxazole and itraconzole. The median age at the time of diagnosis was 23.8 months. There were 14 boys and 6 girls. In the course of these disease processes there were 97 infections: abscesses [n = 32], lymphadenitis [n=9], pneumoniae [n=8], invasive aspergillus pneumonitis [n=4], osteomyelitis [n=5], gastroenteritis [n=6], sepsis [n=2] and others [n=31].The principal microorganisms isolated were: Staphylococcus sp. [n=32], Aspergillus sp. [n=5]. Two patients had died due to Salmonella infections. CGD isn't rare in Tunisia. The most common clinical manifestations were abscesses and pneumonia

[Splenectomy among thalassemia major patients: a study of 44 cases]

The present study was designed to explore the principle indications of splenectomy in transfusion dependant thalassemie patients and to evaluate incidence, risk factors and degree of severity of splenectomy among these patients. In a retrospective analysis, we identified patients with thalassemia major who underwent splenectomy within an 9 years period. Transfusion needs were calculated by the volume of annual blood units devided by the patients' weight at the middle of the same period. Occurrence of hypersplenism is defined by transfusion's requirement over 250ml/kg/year with or without periphericcytopenia. Statistical analysis was carried out using software [SPSS version 11.5]. 44 thalassemie patients took part in the study [mean age, 13 years; range: 42 months-18years]. The mean age of splenectomy was 6 years and 9months [2years-1 Syears and Gmonths]. The mean indication of splenectomy was the increase of transfusion requirement. Pneumococcal, haemophilus influenzae and meningococcal A+C vaccinations were administered respectively to 97.7 per cent, 88.6 per cent and 90.9 per cent of patients. We assisted clinically to a reduction of transfusion requirement and biologically to the increase of haemoglobin's mean level. Ferritinemia and iron overload were notably decreased. Of the 44 patients who underwent splenectomy 10 developed complications [22.7 per cent]. Splenectomy is also beneficial in treating thalassemia major. It reduces patients' transfusion requirement and iron overload and increases the mean level of haemoglobin. Overwhelming post splenectomy infection remains a serious complication in these patients