Clinical Features of Sickle Cell Disease in Eastern Saudi Arab Children
| Autor: | M. I. El Mouzan, M.T. Al Torki, B H al Awamy |
|---|---|
| Rok vydání: | 1990 |
| Předmět: |
Male
Pediatrics medicine.medical_specialty Anemia Saudi Arabia Anemia Sickle Cell Asymptomatic Dactylitis Fetal hemoglobin medicine Humans Mass Screening Prospective Studies Prospective cohort study Fetal Hemoglobin Mass screening business.industry Infant Newborn Hematology Fetal Blood medicine.disease Sickle cell anemia Hemoglobinopathy Oncology Pediatrics Perinatology and Child Health Female medicine.symptom business Follow-Up Studies |
| Zdroj: | Journal of Pediatric Hematology/Oncology. 12:51-55 |
| ISSN: | 1077-4114 |
| DOI: | 10.1097/00043426-199021000-00009 |
| Popis: | The clinical features of sickle cell disease (SCD) in Saudi Arab children of eastern origin are presented. One hundred and seventy-three children were diagnosed at birth and followed prospectively from 3 months to up to 4 years of age. There were 87 boys and 86 girls. Genotype distribution included 146 sickle cell anemia, 24 sickle beta +-thalassemia, two sickle beta 0-thalassemia, and one sickle hemoglobin C disease. Of our patients, 7% presented in the first 12 months of age and 27% remained asymptomatic at 4 years. Painful crises of bones and joints were the most common initial symptoms, followed by dactylitis, abdominal crises and acute splenic sequestration (ASS), occurring in 60%, 31.6%, 6.7%, and 1.7% of the patients, respectively. None of the patients presented with severe bacterial infections. During this study, 175 sickle cell crises were documented, but only 16 (9.1%) required hospital admissions. There were no deaths in this series. High hemoglobin F levels correlated with delayed clinical presentation and reduced number of crises. We conclude that SCD in children of eastern origin is clinically milder than earlier descriptions from the Eastern Province of Saudi Arabia. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|