Factors Effecting Hospitalization Frequency of Sickle Cell Anemia Patients

Purpose: In this study, we aimed to find factors effecting hospitalization frequency of our sickle cell anemia (SCA) patients with painful crisis. Material and Methods: Eighty four SCA patients in steady-state condition participated to the study: Seventy with homozygous, and 14 with sickle cell-beta thalassemia disease. In this study we also reported demographic and clinical data of our patients from February 2010 to October 2012. Results and Conclusion: We found that patients using hydroxyurea and penicillin prophylaxis stayed in hospital more times than the others. No relationship between high hospitalization and other variables have been found. However genetic differences may be present among these patients which warrant further studies

Seroprevalence of Hepatitis B, Hepatitis C and HIV in Patients with Hemoglobinopathy Patients

Purpose: Thalassemia and sickle cell anemia patients have frequent transfusions. Hepatitis B (HBV), hepatitis C (HCV) and human immunodeficiency virus (HIV) are tramsmitted infections with blood. The objective of this study is to determine frequency of these infections in our hemoglobinopathy patients. Material and Methods: We investigated 410 hemoglobinopathy patients. Viral serologies were detected with second-generation enzyme-linked immunosorbent assay method. In 410 patients (116 thalassemia major, 16 thalassemia intermedia, 12 hemoglobin H, 222 sickle cell anemia, 43 sickle-beta thalassemia and 1 Hb SE), there were 258 males and 152 females. Results: The rate of HBV is 1,2%, HCV is 3,2% and HIV is 0%. Our results shows that transfusion transmitted viral infection prevelance is not high compared to the literature. Conclusion: Using sensitive screening tests with periodically and right donor selection are very important for preventing these infections in hemoglobinopathy patients who are under high risk. [Cukurova Med J 2014; 39(4.000): 784-787

Hemoglobin SE disease in Hatay, in the southern part of Turkey

WOS: 000366114600002Double heterozygosity for hemoglobin (Hb) E and S, known as HbSE disease, is a rare, clinically benign condition involving mild hemolysis. Only 25 cases have been reported to date. The current literature generally associates HbSE with a benign clinical course, although vaso-occlusive complications have been reported. Although only single case reports were previously available, we have observed 20 cases of HbSE and wish to report them. We examined the records of patients presenting to our hemoglobinopathy center in 2001-2013. High performance liquid chromatography (HPLC) was used for hematological assessment of blood samples with ethylenediaminetetraacetic acid. Eight patients were male and 12 female. Mean hemoglobin electrophoresis values were Hb Al: 6.3%, Hb E 34.5%, HbS: 59.5% and Hb F: 1.9%. Three patients (15%) were symptomatic with vaso-occlusive crisis and one had cerebral stroke. These were siblings. The patient with cerebral stroke was using hydroxyurea. The incidence of HbSE disease is rising due to population admixtures and racial intermarriages. Increased numbers of cases of HbSE have been detected after premarital hemoglobinopathy screening in the Antakya and Cukurova regions of Turkey. The aim of this study was to report large numbers of patients with HbSE diagnosed through the routine HPLC method. The secondary aim was to emphasize that severe vaso-occlusive crisis such as infarction symptoms could be seen in HbSE.Hemoglobinopathy Center, Hatay Antakya Government Hospital, Turkeythis study was supported bythe Hemoglobinopathy Center, Hatay Antakya Government Hospital, Turke

What chances do children have against COVID-19? Is the answer hidden within the thymus?

A new type of coronavirus named as SARS-CoV-2 pandemic has begun to threaten human health. As with other types of coronaviruses, SARS-CoV-2 affects children less frequently, and it has been observed that the disease is mild. In the pathogenesis of a standard viral infection, the pathogen's contact with the mucosa is initially followed by an innate immunity response. T cells are the primary decisive element in adaptive immunity capability. For this reason, the adaptive immune response mediated by the thymus is a process that regulates the immune response responsible for preventing invasive damage from a virus. Regulatory T cells (T-reg) are active during the early periods of life and have precise roles in immunomodulation. The thymus is highly active in the intrauterine and neonatal period; it begins to shrink after birth and continues its activity until adolescence. The loss of T-reg function by age results in difficulty with the control of the immune response, increased inflammation as shown in coronavirus disease (COVID-19) as an inflammatory storm. Also, the thymus is typically able to replace the T cells destroyed by apoptosis caused by the virus. Thymus and T cells are the key factors of pathogenesis of SARS-CoV-2 in children

Hemophagocytic lymphohistiocytosis associated with oxcarbazepine

Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening multisystem disorder. Reports of the disorder as a side effect of drugs are extremely rare. We report the case of a 3-year-old boy with a history of epileptic seizures in which oxcarbazepine was added to treatment for the last 35 days and dose had been increased. For 10 days he had a fever, hepatosplenomegaly, rash, edema and other systemic symptoms. He was diagnosed with HLH after bone marrow examination. Oxcarbazepine treatment was terminated after the intravenous immunoglobulin treatment. The next day, clinical and laboratory results had improved. This is the first HLH report of an association with oxcarbazepine. Bone marrow aspiration may be indicated to confirm the diagnosis when facing a patient with systemic symptoms after newly added antiepileptic drug treatment