title: Clinical phenotypes and the biological parameters of Congolese patients suffering from sickle cell anemia: A first report from Central Africa.
abstract: BACKGROUND
  The influence of phenotype on the clinical course and laboratory features of sickle cell anemia (SCA) is rarely described in sub-Saharan Africa.
  
  
  METHODS
  A cross-sectional study was conducted in Kinshasa. A clinical phenotype score was built up. The following definitions were applied: asymptomatic clinical phenotype (ACP; score≤5), moderate clinical phenotype (MCP; score between 6 and 15), and severe clinical phenotype (SCP; score≥16). ANOVA test were used to compare differences among categorical variables.
  
  
  RESULTS
  We have studied 140 patients. The mean body mass index (BMI) value of three groups was lower (<25 kg/m2) than the limit defining overweight. BMI of the subjects with ACP was significantly higher than those of other phenotypes (P<.05). Sickle cell patients with ACP have a high mean steady-state hemoglobin concentration compared to those with MCP and SCP (P<.001). A significant elevated baseline leukocyte count is associated with SCP (P<.001). Fetal Hemoglobin (HbF) was significantly higher in ACP. Significant elevation of alpha 1 and alpha 2 globulins in SCP were observed.
  
  
  CONCLUSIONS
  In our study, fetal hemoglobin has an influence on the clinical severity and the biological parameters of SCA. The study provides data concerning the sickle cell anemia clinical and biological variability in our midst.
authors.affiliation: Department des Sciences de Bases, Laboratory of Biochemistry and Molecular Biology, Faculty of Medicine, University of Kinshasa, Kinshasa, Democratic Republic of Congo.
authors.forename: Tite M
authors.initials: TM
authors.lastname: Mikobi
authors.affiliation: Unit of Human Genetics, Department of Paediatrics, Faculty of Medicine, University Hospital of Kinshasa, University of Kinshasa, Kinshasa, Democratic Republic of Congo.
authors.forename: Prosper
authors.initials: P
authors.lastname: Lukusa Tshilobo
authors.affiliation: Division of Hemato-oncology and Nephrology, Department of Paediatrics, University Hospital of Kinshasa, Faculty of Medicine, University of Kinshasa, Kinshasa, Democratic Republic of Congo.
authors.forename: Michel N
authors.initials: MN
authors.lastname: Aloni
authors.affiliation: Division of Biostatistics and Epidemiology, School of Public Health, University of Kinshasa, Kinshasa, Democratic Republic of Congo.
authors.forename: Pierre Z
authors.initials: PZ
authors.lastname: Akilimali
authors.affiliation: Department des Sciences de Bases, Laboratory of Biochemistry and Molecular Biology, Faculty of Medicine, University of Kinshasa, Kinshasa, Democratic Republic of Congo.
authors.forename: Georges
authors.initials: G
authors.lastname: Mvumbi-Lelo
authors.affiliation: Division of Hemato-Immuno-rhumatology, Department of Internal Medicine, Faculty of Medicine, University Hospital of Kinshasa, University of Kinshasa, Kinshasa, Democratic Republic of Congo.
authors.forename: Jean Marie
authors.initials: JM
authors.lastname: Mbuyi-Muamba
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000145:classification / Q000453:epidemiology / Q000503:physiopathology; D015992:Body Mass Index; D002648:Child; D003430:Cross-Sectional Studies; D015023:Democratic Republic of the Congo / Q000453:epidemiology; D005260:Female; D005319:Fetal Hemoglobin / Q000032:analysis; D006801:Humans; D008297:Male; D010641:Phenotype; D055815:Young Adult
chemical_list: D005319:Fetal Hemoglobin
keywords: Africa; Democratic Republic of Congo; Kinshasa; body mass index; clinical phenotype; fetal hemoglobin; globulins; hemoglobin; leukocytes; sickle cell anemia