Non-benign sickle cell anaemia in western Saudi Arabia. 1985

J K Acquaye, and A Omer, and K Ganeshaguru, and S A Sejeny, and A V Hoffbrand

Seventy-one Saudi and Yemeni Arabs with sickle cell anaemia from western Saudi Arabia aged between 1 1/2 and 42 years were studied. The mean steady state haemoglobin concentration of 8.1 g/dl was lower than that of 10.7 g/dl reported previously for sickle cell anaemia in eastern Saudi Arabia. The patients were divided into an SSLF group with fetal haemoglobin (HbF) of 10.0% or below (44 patients) and an SSHF group having HbF above 10.0% (27 patients). No significant differences were found in the haemoglobin concentrations, haematological indices and incidences of bone changes of the two groups. SSLF patients were significantly more prone to infections (P less than 0.01), however. Also, there was an overall high incidence of hepatomegaly (69.0%) and splenomegaly (54.9%) and hepatomegaly was significantly more common in the SSLF group (P less than 0.02). Many of the patients, even with HbF levels over 10.0%, did not follow a benign course and suffered from severe anaemia, infections of the respiratory and urinary tracts, bone pains and infarcts, or bossing of the skull. Rarer complications included hepatic crisis, chest syndrome, retinal haemorrhage, epistaxis and hemiplegia. It is therefore apparent that Saudi Arabian sickle cell anaemia, even in patients with raised haemoglobin F levels, may be as clinically severe as in African patients.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D011247 Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH. Gestation,Pregnancies
D011250 Pregnancy Complications, Hematologic The co-occurrence of pregnancy and a blood disease (HEMATOLOGIC DISEASES) which involves BLOOD CELLS or COAGULATION FACTORS. The hematologic disease may precede or follow FERTILIZATION and it may or may not have a deleterious effect on the pregnant woman or FETUS. Complications, Hematologic Pregnancy,Hematologic Pregnancy Complications,Pregnancy Complications, Hematological,Pregnancy, Hematologic Complications,Complication, Hematologic Pregnancy,Complication, Hematological Pregnancy,Complications, Hematological Pregnancy,Hematologic Pregnancy Complication,Hematological Pregnancy Complication,Hematological Pregnancy Complications,Pregnancies, Hematologic Complications,Pregnancy Complication, Hematologic,Pregnancy Complication, Hematological
D001847 Bone Diseases Diseases of BONES. Bone Disease,Disease, Bone,Diseases, Bone
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005260 Female Females
D005319 Fetal Hemoglobin The major component of hemoglobin in the fetus. This HEMOGLOBIN has two alpha and two gamma polypeptide subunits in comparison to normal adult hemoglobin, which has two alpha and two beta polypeptide subunits. Fetal hemoglobin concentrations can be elevated (usually above 0.5%) in children and adults affected by LEUKEMIA and several types of ANEMIA. Hemoglobin F,Hemoglobin, Fetal
D006760 Hospitalization The confinement of a patient in a hospital. Hospitalizations

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