Introduction: Sickle cell disease (SCD) is a genetic disorder that can be diagnosed by early onset screening tests. In embryos and newborns with sickle cell syndrome, the anatomic development and brain circulation is less than the normal people, and brain circulation plays an important role in brain development. Objectives: The purpose of this study was to evaluate the level of IQ in children with SCD. Patients and Patients and Methods: The study was a descriptive-epidemiologic. The population of the present study was all children aged 7-14 years old with SCD in Ahvaz. The sample of this study was 50 children with SCD. They were selected from among clients referring to the hepatitis clinic of Shafa hospital in Ahvaz. About 50 healthy children were selected from the first or second-degree family members of the patients with SCD as the control group. The data was collected using Raven’s Progressive Matrices (RPM) and demographic information questionnaire. Results: The mean and standard deviation of IQ scores of the patients with SCD was 94.52 ± 14.41, and the mean and standard deviation of IQ scores of healthy subjects was 105.86 ± 11.38. The results showed a significant difference between the two groups in terms of IQ score (P<0.05). Moreover, the results showed that IQ level in patients with SCD was significant regarding their place of residence (P<0.05), however IQ level was not significant in patients with SCD regarding gender and race (P>0.05). Conclusion: The results showed that IQ in children with SCD is lower than that of the healthy subjects. Thus, the present study showed the importance of SCD on children’s IQ.
Autoimmune neutropenia is a type of immune-mediated neutropenia, caused by antibody-induced neutrophil destruction. Here, we report two cases (a 3-year-old boy and a 9-year-old girl) with suspected autoimmune neutropenia. The presence of neutrophil antibodies in the sera of these two patients was investigated; using standard neutrophil antibody screening tests such as granulocyte immunofluorescence test (GIFT), granulocyte agglutination test (GAT), and lymphocyte immunofluorescence test (LIFT). A positive reactivity with two-panel cells was found in GIFT. No reactivities with panel cells were observed in GAT and LIFT. To the best of our knowledge, this is the first report for detecting the neutrophil reactive antibodies; using genotyped neutrophils in patients with autoimmune neutropenia in Iran. The final diagnosis of our patients was primary autoimmune neutropenia for the boy and autoimmune neutropenia associated with familial Mediterranean fever for the girl.
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