Muhammad Rafique, Shumaila Zia, Muhammad Rafique, Muhammad Abid Khan, Youssef Ali Mohamad Alqahtani, Mohammed A Huneif.
Demographic and Clinical Characteristics of Children with Sickle Cell Disease.
Pak Paed J Jan ;39(2):85-92.

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. Three-quarter 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. Saudi Arabia, children, sickle cell disease, demographics, manifestations, complications

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