[Protein profile in 45 Congolese children with sickle cell anaemia]. / Etude du profil protéique de 45 enfants drépanocytaires homozygotes congolais.

ABSTRACT

Sickle cell anemia (SCA) is a genetic disorder characterized by severe hemolytic anemia, frequent vaso-occlusive events and infections. In tropical environment, people are continuously in contact with infection agents. The present study was undertaken to measure 10 protein parameters in order to test humoral immunity, nutrition status and the relation between inflammation and hemolysis in sickle cell anemia patients in 45 Congolese sickle cell children (15 females and 30 males, median age 7 yrs) and a control group of 43 well healthy congolese group (18 females, 25 males; median age 18 yrs). Mean values for immunoglobulins (IgG, IgM, IgA), nutrition proteins (albumin, transthyretin and transferrin) and inflammatory and hemolysis markers (C3, CRP, A1GP alpha1-Glycoprotein acid and haptoglobin) were compared between two groups. Hyperstimulation of humoral immunity was observed in the SCA group. Most significative difference was found with IgA (p < 0,001). Intravascular hemolysis was illustrated by a significant decrease of the haptoglobin/A1GP ratio, and was constantly present in SCA patients. We also described a significative decrease (p < 0,001) of haptoglobin/A1GP ratio between SCA patients with inflammatory syndrom when compared to those without inflammation. All data confirm that haemolysis is quite linked to inflammation in SCA. In addition, nutrition parameters were significantly decreased in SCA group vs healthy congolese group.