Demographic and clinical characteristics of children with sickle cell disease

Objectives: to analyze the socio-demographics and clinical presentation of saudi children with sickle cell disease (scd). study site: this study was conducted at assir central hospital abha,kingdom of saudi arabia. study type: this was a hospital based cross sectional prospective study. material and methods: this cross-sectional,prospective study was conducted from january 2012-december 2013 at assir central hospital,abha,south-western saudi arabia. data of patients was collected by interview of parents/guardian and also from records of patients. results: study included 175 children less than 15 years of age. fifty one percent parents had consanguinity and 97% were carrier to scd. threequarter of fathers were government servants while 91% mothers were house wives. four percent fathers and 30% mothers were illiterate. one third fathers while only 7% mothers had university education. per capita income was rs.58,000 ($574)/month. mean number of children/house were 4.5. patients had mean age 5.7 years and diagnostic age one year with 91% homozygous disease. all were vaccinated and using folic acid. seventy one percent children were regularly using prophylactic penicillin and 18% hydroxyurea. common clinical manifestations were anemia 97%,vaso-occlusive crisis 54%,hepatomegaly 45%,malnutrition and splenomegaly 40%,hemolytic crisis 29%,gall stones 13%,acute chest syndrome 10%,and osteomyelitis 8%. mean hb was found 7.1g/dl,hbs 72%,hbf 17.5%,leukocyte 20.9x109/l and reticulocyte count 10.1%. patients had previous admissions three/year and blood transfusions two/year. conclusion: socioeconomic status had significant association with severity of disease. fathers' education was higher than mothers. infections,vaso-occlusive crisis,acute chest syndrome and aplastic crises were common manifestations while frequent complications observed were cholilithiasis,osteomyelitis,and stroke. key words: saudi arabia,children,sickle cell disease,demographics,manifestations,complications. © 2015,pakistan pediatric journal. all rights reserved.