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Clinical and laboratory characterization of adult sickle cell anemia patients in Kinshasa.
Lumbala, Paul Kabuyi; Mbayabo, Gloire; Ngole, Mamy Nzita; Lumaka, Aimé; Race, Valerie; Matthijs, Gert; Van Geet, Chris; Lukusa, Prosper Tshilobo; Devriendt, Koenraad; Mikobi, Tite Minga.
Afiliação
  • Lumbala PK; Department of Pediatrics, University of Kinshasa, Kinshasa, DRC.
  • Mbayabo G; Department of Pediatrics, University of Kinshasa, Kinshasa, DRC.
  • Ngole MN; Department of Clinical Biology, University of Kinshasa, Kinshasa, DRC.
  • Lumaka A; Faculty of Medicine, Center of Human Genetics, University of Kinshasa, Kinshasa, DRC.
  • Race V; Center for Human Genetics, KU Leuven and University Hospitals Leuven, Leuven, Belgium.
  • Matthijs G; Center for Human Genetics, KU Leuven and University Hospitals Leuven, Leuven, Belgium.
  • Van Geet C; Department of Cardiovascular Sciences, Center for Molecular and Vascular Biology, KU Leuven, Leuven, Belgium.
  • Lukusa PT; Faculty of Medicine, Center of Human Genetics, University of Kinshasa, Kinshasa, DRC.
  • Devriendt K; Center for Human Genetics, KU Leuven and University Hospitals Leuven, Leuven, Belgium.
  • Mikobi TM; Faculty of Medicine, Center of Human Genetics, University of Kinshasa, Kinshasa, DRC.
PLoS One ; 17(12): e0278478, 2022.
Article em En | MEDLINE | ID: mdl-36525434
BACKGROUND: Sickle cell anemia (SCA) is a monogenic hemoglobinopathy associated with severe acute and chronic complications, with the highest incidence worldwide in Sub-Saharan Africa. The wide variability in clinical manifestations suggest that a uniform response to hydroxurea may not be attained. In view of a potential treatment with hydroxyurea (HU), we assessed the variability of clinical and hematological manifestations in a cohort of adults with SCA in Kinshasa, capital of the DR Congo in Central Africa. METHODS: A cross-sectional study was conducted in a hospital dedicated to SCA management in Kinshasa. Clinical history of patients was recorded, a complete physical examination performed. The diagnosis was confirmed by means of DNA analysis. A full blood count and hemolysis markers were measured. The severity of the disease was evaluated by means of a previously reported score. RESULTS: The study group consisted of 166 genetically confirmed SCA patients. The SCA severity was mild in 28.9%, moderate in 64.5% and severe in 6.6%. The disease severity score increased with patient's age (p ≤ 0.001). The severity was higher in males compared to females (p = 0.012). In males, the severity score was correlated with the presence of priapism (p = 0.045), a manifestation not previously incorporated in the severity score. The severity score was inversely correlated with the fetal hemoglobin (HbF) rate (p = 0.005). Malnutrition (BMI <18.5 kg/m2) was present in 47% of patients and was related to the male sex, hip disease (aOR 3.11; p = 0.019) and severe phenotype (aOR 3.53; p = 0.012). Leg ulcers were more frequent in males than in females (p = 0.001; OR 24.3) and were correlated with the number of days of hospitalization (p = 0.029). Hip disease was related to the increasing age (p = 0.008). CONCLUSION: In this selected, hospital-based populations of adults with SCA, severe disease was rare, which may be due to survival bias. However, two thirds had moderate severity of the disease, mostly with a low HbF, and they may benefit from HU treatment. In the Central-African setting the separation between vaso-occlusive and hyperhemolytic sub-phenotypes was not applicable.
Assuntos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Anemia Falciforme Tipo de estudo: Observational_studies / Prevalence_studies / Risk_factors_studies Limite: Female / Humans / Male País/Região como assunto: Africa Idioma: En Revista: PLoS One Assunto da revista: CIENCIA / MEDICINA Ano de publicação: 2022 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Anemia Falciforme Tipo de estudo: Observational_studies / Prevalence_studies / Risk_factors_studies Limite: Female / Humans / Male País/Região como assunto: Africa Idioma: En Revista: PLoS One Assunto da revista: CIENCIA / MEDICINA Ano de publicação: 2022 Tipo de documento: Article