Chronic Granulomatous Disease (CGD) is an uncommon inherited primary immunodeficiency, that can lead to life-threatening infections due to defect in phagocytes to kill some microbes. The incidence is variable among different countries, and the clinical course range from mild to severe disease. This study was carried out to increase the awareness of the disease among medical media which is very deficit especially in the absence of diagnostic investigations in most centers in Libya.To the best of our knowledge, this survey can be considered as the largest Libyan study which demonstrated the clinical characteristics of 54 CGD patients. Male cases were 34 out of the surveyed patients. The parents were consanguineous in 30 CGD patients (55.6%).About 16 patients were from Benghazi city and 38 were from outside Benghazi.The most common manifestation in CGD patients was infection in the lymph nodes (87%), followed by skin infection (85%), infection in gastrointestinal tract (77%), lung infection (72%), BCG disease (44%), urinary tract infection and septicemia (33%), bone and joint infections (19%), inflammatory complications and brain infection (11%) and infection in the liver (9%). 33.3% of patients received antibiotic prophylaxis, 40.7% received combined antibiotic plus antifungal prophylaxis, while 26% underwent hematopoietic stem cell transplantation (HSCT).
This study was designed to examine the effect of different dose of Pentostam administration in male albino mice as a model, todetermine their effect on some biochemical and heamatological parameters. 28 adult male of BALB/c wild mice aged 8- 12 weeks andweighing 25-39 gm were used and i.p. injected with 10mg/kg, 20mg/kg, and 40mg/kg pentostam in addition to a control group. After28 therapeutic days, blood sample were collected from each animal. The result of Biochemical Index of the serum obtained showedthat there were increase significant differences between different concentration doses on AST, ALT, ALP, Urea, Creatinine and BUNas compare to control group at (p >0.01). Conversely in case of Albumin and Total protein were the result showed decrease significantdifference between different concentration doses on this parameter as compare to control group of the same at (p> 0.01). while nosignificant differences between the different concentrations group of doses on Total bilirubin, where the F value was (1.00). The resultof Hematological Index of blood sample showed that there were decrease significant difference at (P ≤ 0.01-0.05) in hemoglobin, HCT,WBCs, LYM and MON concentration in the different concentrations dose of treatment as compared to control group. Conversely, forMCHC, NEU and EOS there were increase significant difference at (P ≤ 0.05-0.01) as compared to the control group. While nosignificant changes (P ≤ 0.0-01.05) in the parameters of RBCs, MCV, MCH, BAS count and Platelet were found when compared withcontrol animals. Biochemical and hematological evaluation pattern still an important role to evaluate drug toxicity. However, theresults of this study concluded that, different doses of Pentostam can cause hepatotoxicity and nephrotoxicity. Therefore, it will beimportant of change in anti-leishmanial regimen to reduce their side effect
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