Erythrocyte adhesion marker [c36] in pediatric sickle cell anemia: relation to clinical Syndrome, HbF and hydroxyurea therapy

To investigate the possible role of the erythrocyte adhesion marker [CD36] in modulating adhesion of sickle erythrocytes to vascular endothelium and its relation to HbF% and hydroxyurea [HU] therapy, 28 pediatric sickle cell anemia [SCA] patients, divided into 3 groups, were studied. Group I included 11 patients in a steady state; group II comprised 10 patients in a steady state receiving HU and group III involved 7 patients in acute painful crises. Ten healthy age matched children served as controls. CD36+ erythrocytes were assessed using flow cytometry and expressed as % of positive cells. HU-treated patients [Group II] improved clinically and hematologically even before the peak level of HbF were reached. The mean Hb, Ht and RBCs were higher in-group II VS groups I and III [P < 0.05] and the WBCs and platelet counts of group II were comparable to controls. HbF%, was significantly higher in group II VS groups I and III [P < 0.001]. The mean [336% was significantly elevated in every patient group compared to controls [P< 0.001]. In-group II, it was 1.35 +/- 0.3% which was lower than group I and higher than group III [2.75 +/- 1.4, 0.9 +/- 0.1 7, P < 0.01]. 036% was positively correlated to reticulocyte % [r = 0.894, P = 0.000] being two faces of the same coin in vaso-occlusion. Also there was an inverse significant relationship between CD36% and HbF% [r -0.569, P < 0.007]. In addition a strong positive correlation was found between CD36% and duration of HU therapy [r = 0.767, P < 0.01]. This could be a form of drug tolerance over-time

Conclusion: more therapeutic modalities are needed to tackle more effectively the prime pathogenetic mechanism of vaso-occlusion as CD36 in SCA children

Plasma chitotriosidase activity in patients with. beta-thalassemia

Variable increase in plasma chitotriosidase levels have been reported in Italian patients with beta thalassemia major and intermedia. We measured plasma chitotriosidase levels in Egyptian patients with beta thalassemia to ascertain its use as a universal marker of the disease and /or response to therapy. Chitotriosidase levels in 30 children [1.5-15 years] with beta thalassemia major and 10 children [6-15 years] with beta thalassemia intermedia were compared with other measures of the disease, such as ferritin, hemoglobin F, pre-transfusion hemoglobin, and liver function tests. Plasma chitotriosidase levels were normal in all cases with thalassemia intermedia [median 22.5, range 8.8-69 nmol/ml/h]. Eight patients with thalassemia major had elevated levels [<69.4 nmol/ml/h]. A significant correlation was found between plasma chitotriosidase and hemoglobin F, ferritin levels, and with duration of transfusion. Normal chitotriosidase levels in thalassemia intermedia and elevated levels only with thalassemia major and correlation with duration of blood transfusion may reflects status of iron overload in macrophages. The highly significant correlation with hemoglobin F might reflect the role of thalassemia genotype in chitotriosidase production. Thus there may be a role for monitoring chitotriosidase in patients with beta thalassemia. Our results confirm results of the Italian cohort; however in the latter chitotriosidase levels were much higher

Study of complement activation product in active rheumatic fever

Serum complement C3 and C4 levels immunoglobulin levels have been done for 160 children with active rheumatic fever. The C3 serum level was found to be significantly decreased in the group of rheumatic chorea. The immunoglobulins IgM and IgA were significantly increased in all the patients studied. All C3, C4 and the immunoglobulins [G, A, M] serum levels were assayed by the automated immunochemical precipitation techniques using the ICS

Complement as a mediator of inflammation in post-streptococcal glomerulonephritis

Serum complement C3 and C4 and the immunoglobulins IgG, IgA and IgM were studied in 40 children with post-streptococcal glomerulonephritis. The serum levels of C3 and IgG levels showed significant decrease when compared to a control group of 20 healthy children, while the serum IgM showed significant increase. The decreased C3 and IgG level could be attributed to increased utilization in antigen/antibody complexes. These serum C3, C4 and the 3 classes of immunoglobulins were assayed by automated immunochemical precipitation using the ICS

value of acidified glycerol lysis time test in the diagnosis of unexplained cases on neonatal hyperbilirubinemia

Erythrocyte acidified glycerol lysis time test AGLT50 is used for screening a number of erythrocyte abnormalities. An abnormally low value occurs frequently in hereditary spherocytosis. This test was performed on 50 neonates with unconjugated hyperbilirubinemia after exclusion of ABO and Rh incompatibilities. AU had in addition varying degrees of anemia and reticulocytosis which are indicative of haemolysis. The AGLT50 was abnormally low in 10 percent [5 cases] indicating the diagnosis of spherocytosis and spherocytes were present in the peripheral blood film in 60 percent [3 cases] of those. The results were equivocal in 14 percent [7 cases] which requires further investigations to reach the diagnosis. Our results indicate that spherocytosis is the cause of neonatal jaundice in 10 percent of the cases. AGLT50 is recommended to be done in cases of unexplained neonatal jaundice as it is a quick, simple, inexpensive test that requires minute amounts of blood and is highly sensitive and specific for all cases of spherocytosis