Over the past decade, few reports suggested that the drug thalidomide (HbF inducer) may be of value in a subset of transfusion-dependent and non-transfusion dependent thalassemia patients. A cohort of 37 patients with symptomatic b-thalassemia syndrome [14 transfusions dependent thalassemia (TDT), and 23 Non-transfusion dependent Thalassemia (NTDT)], who were unable to pursue conventional therapy with transfusion and chelation, were recruited over 3 years in a center in Iraqi Kurdistan. After taking informed consent, patients were put on low dose Thalidomide (2-10 mg/kg), with regular follow up after that for a minimum of 8 months for a response. Patients with TDT were considered responders if their yearly transfusion requirement dropped by 25% or more, while NTDT responders were those who had a hemoglobin raise of 1 g m/dL or more. The median age of enrolled patients was 10 years (range 3-43) and included 21 males and 16 females. After a mean of 1.7 months (SD 0.76), responses were documented in 28 patients (75.7%). Among NTDT patients, a significant increase in hemoglobin from a mean of 7.83 (SD 1.07) to 9.96 g/dL (SD 1.11 g m/dL) was documented. While among TDT patients, there was a significant drop in yearly transfusions from 27 (SD 17.7) to 7.79 (SD 7.5) blood unit per year. The response in both categories was sustained after a median follow up of 15 months (8-36 m). Only minimal side effects were documented throughout in the form of constipation and only one patient developed extramedullary hemopoietic abdominal masses. A significant response to thalidomide was documented in the majority of TDT and NTDT patients, a response which was obtained after a mean of 1.7 months, and the response was sustained with limited side effects. The results support a possible role for this medication in a subset of thalassemia patients.
Background: Smoking is a well-known related factor for many health problems in a human being through different ways of exposure. Objectives: Thie aim of the study was to examine the effects of different types of cigarette smoking on hemoglobin level, high sensitive C-Reactive Protein (hsCRP), Malondialdehyde (MDA), and IgE levels in healthy adult subjects. Methods: One hundred seventy-one healthy adult females and males were included in this study. They divided into four groups: cigarette, shisha, passive smokers, and non-smokers groups. Serum samples from all groups analyzed for hemoglobin, hsCRP, IgE, and malondialdehyde level. Results: The mean MDA, IgE, and hemoglobin levels significantly increased in both smokers (cigarette and Shisha groups) and passive smokers than in non-smokers group (p<0.05). The hsCRP levels were significantly increased (p<0.05) in cigarette and Shisha smokers compared to non-smokers. At the same time, there was a non-significant relationship between passive smoker in comparison to non-smokers (p>0.05). Conclusion: This study concluded that smoking, including cigarette and shisha, even passive smoking harmed health through increasing Malondialdehyde, serum IgE and hs-CRP levels in the body.
Mendelian susceptibility to mycobacterial diseases (MSMD) is a rare condition of primary immunodeficiency disorder. Interleukin-12 receptor β1 (IL12RB1) deficiency, is the most common genetic etiology of MSMD, which is characterized by the selective predisposition to clinical disease caused by weakly-virulent mycobacteria, such as Bacillus Calmette-Guérin (BCG) vaccines, and environmental non-tuberculous mycobacteria (NTM). To the best of our knowledge, this is the first case of IL12RB1 deficiency to be reported from Iraq. Our case is an 8-year-old Syrian girl, for first-cousin parents, with a refugee-status in the North of Iraq. She had a history of disseminated BCG infection 2 months after receiving BCG vaccine, in addition to repeated episodes of mild or severe illnesses, such as maculopapular skin rash, lymphadenopathy, gastroenteritis, meningitis, and clinically diagnosed tuberculosis (TB) based on local TB-prevalence setting. Because of limited medical facilities in the war-torn countries; in Syria and Iraq, no diagnosis could be reached. We used Flinders Technology Associates (FTA) cards to transfer her bone marrow aspirate to Japan. A homozygous IL12RB1 mutation was detected by whole exome sequencing in Japan, using genomic-DNA extracted from dried bone marrow sample spots on FTA filter paper. In conclusion, diagnosis of MSMD due to IL12RB1 deficiency was possible by transferring the FTA sample of the patient for genetic evaluation in Japan. Our report recalls the need of pediatricians in countries with TB-prevalence and high parental consanguinity, to consider IL12RB1 deficiency in the differential diagnosis of a child with clinical evidence of TB, especially with the history of disseminated BCG disease.
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