G6PD DEFICIENCY IN A DIABETIC PATIENT WITH A TURNER SYNDROME

Abstract

Introduction. With 7 millions inhabitants, 3% population growlh, Benin is considered as the most affected by hereditary hemoglobinic abnormalities amongst ail Benin Gulf countries. Hemoglobin (Hb) Hb S prevalence is 24% versus 9% for Hb C. The rate or sickle cells disease (SCO) in the population is 4%. MonlJgement of the disease. SCO management began in the seventies in free out-patients consultations, Antalgics, antipyretics, non steroid anti·inflammatory drugs, antibiotics, blood transfusion, folie acid supplementalion and hydratation are usual treatments, SCD management got improvement since. the National University Hospital (CNHU) created lhe Haematology Service in 1988. The SCO patients represent 65% of the work load and they consume 35% of blood collected· by the Cotonou blood transfusion centre. Out of 519 patients hospitalized in two years, 63.6% were l-lb SS and 14.8% Hb Sc. Hb SS subjects were mainly.hospilalized for anaemic syndrome (40.6%), hyperalgie allacks (34.5%) and infections (20.9%). Sustained follow·up increased patients'Iife qualily. This results in a need of addressing their training, occupation and marriage issues, In 1991, patients were charged for the medical consultation; then paying profession al services progressively reduces (60%) the HaematoJogy Service altendance rate from 1991 to date. Reseorch. Traditional medicine, assessed through phytotherapy, seems to be only symplornatic. Other research topics concem psychosocial aspects, blood transmitted diseases, chroniccomplications, feasibility of physical aClivities as integration and self esteem factors. Documents are available to support fair information on SCD. SCO financial bur<.Jen has becn evaluate<.J. ASCO Centre for children and pregnant women (.1990) and a National Programme are availablc. Associative (lctivities. Associative activities are implemenlcd 1O rai se solidarity among patients, their relatives and the population. Ifs the main goal of Benin Slckle Cells Association (Association Béninoise de Lutte contre la