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1.  Quality of life in patients with thalassemia major 
With modern medical management, thalassemia major is now extending into adulthood and it is expected to have a negative impact on the quality of life (QOL) of the patients. The aim of this study was to evaluate quality of life in patients with thalassemia major.
Materials and Methods
This is an analytic case control study. Two hundred and fifty patients and 51 participants as controls were assessed using WHOQOL- BREF (Farsi version) questionnaire. All questions were answered based on the self-evaluated status in the past 2 weeks before enrollment and were rated on a five-point Likert scale. Therefore, the raw item score ranged from 1 to 5 and scaled in a positive direction and 6 dimensions including overall QOL, overall health, physical, psychological, social, and environmental relationship were assessed.
Results showed that the QOL in all 6 dimensions was lower in patients compared to the controls (P< 0.05).Also age, higher education level, lower ferritin level and using oral iron chelator were associated with better QOL scores. On the other hand, cardiac disease, hepatitis C and history of psychiatric disorders were associated with impaired QOL scores.
These findings were important for future refinement of national thalassemia program. So, we recommended regular screening for psychiatric disorders and facilitated access to oral iron chelators. Regular monitoring and treatment of complications especially cardiac disease and hepatitis along with strict quality control of blood products were also mandatory. Also, higher education of the patients may improve quality of life.
PMCID: PMC4083201  PMID: 25002926
Quality of life; Thalassemia Major; WHOQOL- BREF
2.  Evaluation of new cases of HCV infection in thalassaemia patients for source of infection 
Screening tests on blood bags is important step for blood safety. In Iran, screening for HCV started from 1996. We decided to determine the new cases of hepatitis C in our thalassemic patients, after screening of blood bags was initiated and trace backing from recipients to find their donors.
Materials and Methods:
The study was done on patients with complete files for HCVAb test results. Only cases that had a positive HCVAb result following a negative result were considered as new cases. For trace backing, we recorded the blood transfusions’ date and the blood bags’ number from last negative test results (HCVAb) to the first positive test result. These data were sent to the transfusion center. The suspected donors were contacted and asked to be tested again in the transfusion center.
A total of 395 patients were studied; 229 (58%) males and 166 (42%) females. Mean age was 27.5 years. We had 109 HCV (27.5%) positive cases of whom 21 were infected after 1996. We traced the last five cases contaminated during 2003 and 2004. These five patients had 13, 10, 13, 12, and 6 donors, respectively (totally 54 donors were found). We proved the healthy state in 68.5% (37 of 54) of our donors population. Of them, 81% were repeated donors and 17 of 54 donors (31.5%) could not be traced (because of change in addresses). We did not have any HCV new cases after 2004.
We could not prove HCV transmission from donors as the source of infection. Although parenteral transmission is always on top of the list in HCV infection, the possibility of hospital and/or nursing personnel transmission and/or patient-to-patient transmission such as use of common instruments like subcutaneous Desferal® infusion pumps; which the patients used for iron chelation therapy, should also be kept in mind.
PMCID: PMC3159241  PMID: 21897590
Hepatitis C; thalassemia; transfusion-transmitted infections
3.  T2* magnetic resonance imaging of the liver in thalassemic patients in Iran 
AIM: To investigate the accuracy of T2*-weighted magnetic resonance imaging (MRI T2*) in the evaluation of iron overload in beta-thalassemia major patients.
METHODS: In this cross-sectional study, 210 patients with beta-thalassemia major having regular blood transfusions were consecutively enrolled. Serum ferritin levels were measured, and all patients underwent MRI T2* of the liver. Liver biopsy was performed in 53 patients at an interval of no longer than 3 mo after the MRIT2* in each patient. The amount of iron was assessed in both MRI T2* and liver biopsy specimens of each patient.
RESULTS: Patients’ ages ranged from 8 to 54 years with a mean of 24.59 ± 8.5 years. Mean serum ferritin level was 1906 ± 1644 ng/mL. Liver biopsy showed a moderate negative correlation with liver MRI T2* (r = -0.573, P = 0.000) and a low positive correlation with ferritin level (r = 0.350, P = 0.001). Serum ferritin levels showed a moderate negative correlation with liver MRI T2* values (r = -0.586, P = 0.000).
CONCLUSION: Our study suggests that MRI T2* is a non-invasive, safe and reliable method for detecting iron load in patients with iron overload.
PMCID: PMC3027020  PMID: 21274383
T2*-weighted magnetic resonance imaging; Liver; Iron overload; Major thalassemia; Ferritin
4.  Efficacy and safety of Iranian made Deferasirox (Osveral®) in Iranian major thalassemic patients with transfusional iron overload: A one year prospective multicentric open-label non-comparative study 
Purpose of the study
to determine the efficacy, adverse effects and safety of a new Iranian generic product of deferasirox (Osveral®) in Iranian transfusion dependent major thalassemic (TD-MT) patients.
In 9 main thalassemia treatment centers, all of TD-MT patients (aged ≥2 yrs) with serum ferritin (SF) levels≥1000 ng/ml, or >100 ml/kg of RBC transfusion,who could not tolerate parental iron chelating were recruited regardless of their previous iron chelation therapy. Periodical clinical and laboratory evaluations were conducted for adverse effects (AEs). Primary efficacy end point was Mean of Relative Change of Serum Ferritin (MRC-SF) from the baseline level during one year. Analysis of variance (ANOVA), t test, chi-square or Fisher exact test were used for statistic analysis appropriately (P values <0.05 were considered as statistical significant).
In 407 cases the male/female ratio was 0.98. Mean age was 11.5±7.4 (2–58) years. The mean of initiating dose of Osveral® and mean usage dose during the study was 23.5±4.9 mg/kg and 24.9±4.9 mg/kg respectively. MRC-SF was −11.44% ±38.92 and it showed significant decline in SF (P value<0.001) one hundred and forty eight patients out of 407 patients experienced at least one. AE, the most common of them were transient increase in serum creatinin (97;24.1%) and>5 time increase in transaminases (24;5.89%).The causes of discontinuation of treatment were non-satisfactory treatment ( 24; 5.8%), poor or non-compliance of patients (21;5.1%), and adverse effects (13; 3.1%)
A detailed comparison with similar studies on deferasirox (Exjade®) shows a promising efficacy and safety for its Iranian generic product (Osveral ®).
PMCID: PMC3232111  PMID: 22615664
Thalassemia; Iron Chelation,; Osveral ®; Deferasirox; Safety; Efficacy.
5.  Associates of poor physical and mental health-related quality of life in beta thalassemia-major/intermedia 
Using two logistic regression models, we determined the associates of poor physical and mental health related quality of life (HRQoL) among beta thalassemia patients.
In this cross-sectional study which was conducted during 2006 and 2007 in outpatient adult thalassemia clinic, Blood Transfusion Organization, Tehran, Iran, Short Form 36 (SF-36) was used for measuring HRQoL in 179 patients with beta thalassemia (major/intermedia). We determined scores higher than third quartiles of obtained PCS and MCS scores as the cutoff points of good HRQoL. Poor HRQoL was defined scores lower than first quartiles of obtained PCS and MCS scores. Two distinct logistic regression models were used to derive associated variables including demographic, clinical, and psychological factors.
The regression models suggested that poor physical HRQoL was positively associated with somatic comorbidities (OR = 1.472, CI = 1.021-2.197, p = 0.048) and depression score (OR = 8.568, CI = 2.325-31.573, p = 0.001). The variables that were associated with poor mental HRQoL were anxiety score (OR = 9.409, CI = 1.022-89.194, p = 0.049) and depression score (OR = 20.813, CI = 4.320-100.266, p < 0.001).
Depression is associated with both poor physical and mental HRQoL among patients with major/intermedia beta thalassemia, however somatic comorbidities and anxiety are associated with poor physical and mental HRQoL, respectively.
PMCID: PMC3129078  PMID: 21772908
Thalassemia; Health Related Quality of Life; Anxiety; Depression; Somatic Comorbidities

Results 1-5 (5)