The bactericidal activity of neutrophils depends primarily on free oxygen radicals released by the activation of NADPH oxidase when neutrophils are stimulated by microorganisms. Severe glucose-6-phosphate dehydrogenase (G6PD) deficiency is associated with decreased NADPH production. Increased susceptibility to recurrent bacterial infections in children with severe neutrophil G6PD deficiency as a consequence of decreased NADPH production has been reported earlier. In this study, the in vitro activity of neutrophils from normal and G6PD-deficient individuals was assessed by measuring the [14C]CO2 released via the hexose monophosphate shunt from radiolabeled [1-14C]-glucose and the nitroblue tetrazolium (NBT) dye reduction test. Our results show that the G6PD activity of neutrophils from 48 individuals, identified as severely erythrocyte (RBC) G6PD deficient ( < 2 U/1012 RBC) was 23% of the enzyme activity of neutrophils from 53 individuals with normal RBC G6PD levels (98.8 U/1012 RBC). However, the results of functional assays of neutrophils as measured by hexose monophosphate shunt and the NBT test were comparable in G6PD-deficient and normal individuals, suggesting that a reduced activity of G6PD to as low as 23% of normal does not affect neutrophil function.
Through a survey of more than 20 patients with a specific subgroup of autosomal recessive congenital cutis laxa (ARCL), namely ATP6V0A2-related cutis laxa, we noted that the clinical findings on three patients included pretibial pseudo-ecchymotic skin lesions very similar to those found in classical Ehlers-Danlos syndrome. The finding is apparently age-related, occurring during the second decade in two of the three patients.
Since its description in Japan in 1967, Kawasaki disease has been reported from several parts of the world but has been reported only once in an Arab child and never from the African continent. We report a typical case of Kawasaki disease in an Arab child in Africa who later developed coronary and several peripheral aneurysms.
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