mehrnoush kosaryan

OsteoPenia and osteoPorosis are known to be one of the main comPlications of β-thalassemia major (β-TM). The aim of this study was to determine the factors associated with decreased bone density of these Patients.

Lumbar and femoral neck bone mineral density (BMD) in β-TM Patients characterized via Dual-energy X-ray absorPtiometry (DXA) to PinPoint the association of iron chelators, diabetes mellitus, serum ferritin above 3000 ng/mL, hemoglobin below 8 gr/dl, using hydroxyurea, liver siderosis and moderate to severe hePatic and cardiac siderosis with BMD. The information was collected from the thalassemia registry in 2019 and statistical analysis was Performed by STATA-13 software.

Out of 1959 ParticiPants with β-thalassemia major registered in the registry, 139 ones (7.09%) had bone mineral density (53 males and 86 females). The Prevalence of abnormal bone density in the lumbar and femoral neck was (52.48 to 47.66) 57.5 and (40.18 to 57.52) 48.85 Percent, resPectively. After adjusting for the effects of Possible interventions, the odds ratio of abnormal bone density in the Patients consuming hydroxyurea and deferasirox were 0.24 (0.03 to 1.73) and 0.25 (0.03 to 1.69), resPectively. The highest odds were 8.21 (0.18 to 360.22), and 6.72 (0.60 to 74.83) for moderate to severe cardiac and hePatic dePosition, resPectively.

The Prevalence of osteoPenia and osteoPorosis is high among thalassemia major Patients. ConsumPtion of hydroxyurea and deferasirox is the most imPortant Protective factor and moderate to severe cardiac and hePatic siderosis are the most imPortant risk factors for abnormal bone density.

One of the ways to improve the quality of life of patients with thalassemia major is to empower patients through education to deal with the effects and complications of illness and control disease and life further. This study aimed to determine the impact of E-learning intervention on self-care behaviors in patients with thalassemia major.

The present study is a quasi-experimental study of clinical trial type. The effect of e-learning on the lifestyle of eligible adolescents with thalassemia major of both sexes referred to Razi, and Bu Ali Sina Hospitals was investigated. Samples were divided into two groups control (N=25) and intervention (N=25). The control group received routine training from the relevant centers or health care staff, and the intervention group received e-learning and regular exercise. Data were collected through valid and reliable questionnaires, including two main parts. The first part contains demographic information. The second part of the questionnaire was a standard health-promoting behaviors questionnaire that assesses the lifestyle of adolescents in three dimensions nutrition, physical activity, and stress management. Finally, data were analyzed using SPSS version 22, and P-value less than 0/05 was considered significant.

Obtained results demonstrated a significant difference in the quality of nutrition in adolescents with thalassemia before e-learning intervention between intervention and control groups (P< 0.05). Moreover, after the intervention, there was a significant difference in the stress management of the intervention and control groups, and the scores of the intervention group increased significantly (P< 0.05). The findings also indicated that physical health was also considerably increased in adolescents with thalassemia after intervention by e-learning compared to the control group (P< 0.05).

It can be concluded that e-learning has a significant effect on patients' quality of life with thalassemia in three dimensions: nutrition, physical and mental health. The results of this study can serve as a guideline for designing appropriate educational programs for thalassemia patients.

Electronic registry system of beta-thalassemia patients was run by Thalassemia Research Center (TRC) in 2017. The aim of the current study was presentation of therapeutic status in these patients at Mazandaran Province, Iran. Therapeutic status variables including: Name of cities and hospitals, age and sex of patients, dependent and non-transfusion-dependent, starting age of the blood transfusion and iron-chelating agents, blood group and Rh, washed blood transfusion, abnormal antibody, transfusion reactions, mean hemoglobin during the last 3 months, type of iron chelators, iron chelators dosage, serum ferritin, and the use of hydroxyurea. Overall, 1831 patients were registered [891 male (48.7%)]. Mean age of patients was 30±9.7 yr. Average of hemoglobin levels for female and male were 9.1±5.1 and 9.4±6.3 gr/dl, respectively. Seventy-six percent of transfusion-dependent patients (1385) have received iso-group PRBC (packed red blood cells), after crossmatch. The most common blood group among patient was type O-positive (35.7%). Monotherapy with desferrioxamine was most type of used iron-chelating agent in these patients (47.2%). Mean of ferritin was 3300±7800 (ng/ml). Twenty-eight percent of patients (484) have received hydroxyurea; proportion of male and female was approximately equal. T2 weighted magnetic resonance imaging (MRIT2*) was measured in 62.2% of patients. Moderate and severe hepatosiderosis was 10.1% and 2.9%, respectively. Patients with moderate and severe cardiac siderosis were 11% and 5%, respectively. Registry findings are valuable for treatment management and ensuring patients medications. It will also provide accessibility to various levels of patients’ information for health care managers and experts to help them make appropriate decisions.

Background and Nephrolithiasis in the pediatric population includes 7% of all kidney stone disorders in all age groups. Potassium citrate is one of medical agents used in treatment of these disorders. This study investigated the role of potassium citrate in treatment of patients with kidney stones without any metabolic or infectious risk factors.
A clinical trial was conducted in all patients less than 16 years of age in whom ultrasonography confirmed kidney or urinary tract stones A total of 149 patients with nephrolithiasis was assessed of whom 80 included in current study. Improvements in number of stones was seen in 29 (76%) patients in group I and 17 (57%) in group II (P=0.403). Improvements in maximum size of stones were found in 30 (79%) cases in group I and 22 (73%) patients in group II (P=0.791). Total burden of stones showed improvements in 82% of patients in group I and 73% of those in group II (P=0.887). There were no significant differences between the two groups in any of these items.
Potassium citrate can reduce the size and number of stones in most children with renal stones less than 7mm who have no underlying risk factor. But current study found non-pharmacological supportive treatments to yield similar results.

Background and The electronic registry of patients with beta thalassemia major was developed in Thalassemia Research Center affiliated with Mazandaran University of Medical Sciences in order to provide an accurate database for researchers and health planners. This study reports the condition of patients registered in Mazandaran province till summer 2016.
In this descriptive study, the following information was recorded in the registry system: epidemiologic data, clinical examination data, complications, routine laboratory test results, medications, new cases, and death. Descriptive statistics were used to summarize the data.
Until the summer 2016, 1053 patients including 500 (47.5%) males and 553 (52.5%) females were registered in 14 hospitals. These were half of the number of patients receiving care by the aforementioned University. Among the patients, 920 (87.4%) were transfusion dependent. Single patients included 54.2% (n= 571). The level of formal education was beyond high school in 29.8% (n= 314). Three hundred and fifty (33.2%) patients were employed. Splenectomy was done in 606 (58%) patients. Diabetes mellitus, hypoparathyroidism, and hypothyroidism were diagnosed in at least 12% (n= 120), 14.3% (n= 145), and 11% (n= 112), respectively. Cardiomyopathy was observed in 113 (11.2%) cases. The most common iron chelators medication was desferrioxamine, which was used alone (50.4%) or in combination with deferiprone (24.3%).
Current research showed that in spite of incompleteness of records at the electronic registry of patients with beta thalassemia major, a comprehensive electronic system could enhance the level of care provided for involved patients.

The hematologic response to hydroxyurea (HU) is varied among β-thalassemia (BT) patients. The BCL11A and SOX6 genes are involved in response to HU. This study aimed to investigate the in-vitro responsiveness of HU among BT major patients homozygote for IVSII-1G>A mutation and XmnI single nucleotide polymorphism (SNP) in order to find whether the in-vitro Hb concentration is a predictor of clinical (HU) responsiveness.
In this case-control study, twenty BT patients homozygote for IVSII-1G>A mutation and XmnI SNP from Thalassemia Research Center, Sari, Iran in 2015 were selected and categorized into two groups of 10 Responder (R) and 10 Non-Responder (NR) according to their clinical HU response. Ten healthy individuals as a control group were also selected. Hematopoietic erythroid progenitors were expanded from peripheral blood. Hb concentration was measured using photometry method. The flow cytometry and real-time PCR methods were applied for the analysis of cell surface markers (CD71 and CD235a) and gene expression (BCL11A and SOX6), respectively.
R and NR groups produced higher amount of Basic Hb than C group in cell culture medium at day 14 (P Since different factors including wide networks of intracellular factors and individual differences between patients can affect response to HU in patients, the increasing Hemoglobin on culture medium alone cannot predict clinical responsiveness to that drug.

Alpha Thalassemia is one of the most prevalent disorders worldwide with a [T1] high carrier rate in Mazandaran province (north of Iran). Carriers of --MED double gene deletion are at risk of having a child with hemoglobin haemoglobin[T2] H (HbH) disease, if they marry a silent carrier. Co-inheritance of αααAnti3.7 triplication that cannot be detected using hematological indices and β-globin gene mutations, in heterozygote states, leads to intermediate form of thalassemia. Using precise molecular analysis, the mutations that do not change the hematological parameters can be identified. The diagnosis of these mutations is important in screening programs. Multiplex Gap-PCR and reverse hybridization assay analysis were applied for the detection of mutations on α and β-globin genes in a patient with abnormal hematological[T3] indices from Sari at 2016. A rare co-inheritance of --MED double gene deletion and αααAnti3.7 triplication was identified. The presented case can be at risk of having a child with HbH disease and thalassemia intermedia. So, the presented case shows the [T4] importance of precise molecular analysis in premarital screening in order to prevent having a child with thalassemia.

Background and Despite extensive research on family planning and education, reproductive behavior of high-risk couples for thalassemia remains a major concern among medical professionals. This study aimed to evaluate the knowledge and attitude of carrier couples for transfusion-dependent thalassemia major (TDTM).
This case-control study was conducted 327 carrier couples for thalassemia minor. Contact information of the couples was obtained from three healthcare centers randomly selected from the eastern, central and western parts of Mazandaran province, Iran. To match the socioeconomic status, two control families (right and left neighbors) were interviewed per each carrier couple. Epidemiological characteristics and knowledge of the carrier couples were assessed via 10 and 20 questions, respectively, and scores above 15 were interpreted as adequate knowledge. Moreover, attitude of the subjects was evaluated via 16 items scored based on a four-point Likert scale. Data analysis was performed in SPSS V.16 using Mann-Whitney U test and T-test for the comparison of study groups, and P value of less than 0.05 was considered significant.
In total, 327 high-risk couples for minor thalassemia and 660 control families were enrolled in this study. Among the subjects, 32.6% and 22.3% had adequate knowledge about thalassemia. In addition, attitude of the carrier couples was positive in 58% and 41% of the cases. Also, 30.5% and 31.2% of case and control subjects had favorable socioeconomic status, respectively.
According to the results of this study, knowledge of carrier couples was adequate with regard to TDTM, and they had a positive attitude toward receiving prenatal diagnosis for this syndrome. However, reproductive practice of high-risk couples was relatively poor. Therefore, it is recommended that reproductive behavior of high-risk couples for TDTM be monitored regularly in order to improve their practice.

Chronic hepatitis C infection used to be one of the most important burdens on patients with transfusion-dependent beta thalassemia major (TDTM). Chronic active hepatitis reduces quality of life, and liver cirrhosis and cancer shorten life expectancy in many cases. We compared the characteristics of our patients at the Thalassemia Research Center (TRC) regarding hepatitis C infection at two time points.Patients and A review was conducted in a cohort of 390 TDTM patients with a history of at least one blood transfusion in 2014. Type of treatment protocol for hepatitis C virus (HCV) and the number of courses were defined. Descriptive statistics were performed using SPSS software (V16). Screening for HCV started in 1995 at the TRC. Seventy-seven (15%) patients were antibody-positive in 1995. Tests for virus detection were not available at the time. Patients have been examined using serum AST, ALT, bilirubin, PT, PTT, and liver biopsy, and 45 were treated using alpha interferon alone. A second liver biopsy was performed at the end of treatment for 21 patients, and a blinded pathologist compared the histology according to the Knodell score. According to normalization of liver enzymes, the treatment was successful (McNemar test, P < 0.02). Based on the Knodell score, 54%, 31%, and 11% had complete, partial, and no response, respectively. A quantitative test for viremia became available thereafter. Thirteen patients who were resistant to alpha interferon have been treated using “Pegasys”™ ± ribavirin. Ten patients responded; however, three have been resistant and are still viremic. Twenty-seven patients received no treatment. Twenty-two (81.4%) had negative PCR tests. Five viremic patients refused treatment. A second screening test for HCV antibody was introduced in 2001, and, since then, annual screening for HCV antibody has been performed for all patients. No new case has been found since 2001. During the follow-up period, two deaths have been recorded in the cohort; none was a direct consequence of liver disease. Both patients had negative PCR tests for viremia. In 2014, there were 72 patients (52% men) with positive antibody tests, with a mean age of 30.5 ± 5.7 years. They mean age at the first blood transfusion was 2.8 ± 2.5 years. At the time of publishing, 15 patients (3.8%; 95% confidence interval 2 - 5.6) had viremia. Five patients had documented liver cirrhosis. The prevalence of hepatitis C virus has decreased dramatically owing to primary prevention (donor blood screening and discarding infected blood) and antiviral treatment of affected patients. Better clinical management with iron chelating agents and supportive therapy for cirrhotic patients is also in place.

Background and Ischemic Heart Disease (IHD) is one of the most important complications of diabetes mellitus (DM). This study was designed to investigate the prevalence of IHD and related factors in patients with type II DM in north of Iran. A cross sectional study was conducted in 1021 patients with diabetes type II attending endocrine clinics affiliated with Mazandaran University of Medical Sciences, Iran, 2010 to 2012. Age, gender, history of myocardial infarction, hypertension, hyperlipidemia, smoking and other recognized complications of DM were recorded. All patients were referred to cardiologist and the exercise test was used and if needed the thallium perfusion scan and coronary angiography were done for patients without history of myocardial infarction,coronary artery bypass surgery or stent placement. There were 1021 patients and 907 were female (88.8%). Mean age and duration of diabetes were 54.4 (95% CI: 53.8- 55.1) and 8.8 (95% CI: 8.4 - 9.2) years, respectively. Almost 87% (95% CI: 85-89) and 53% (95% CI: 50-56) had dyslipidemia and hypertension, respectively. Among the patients, 274 patients (37% males and 25% females, 26.8%; 95% CI: 24.1-29.5) were suffering from IHD (P =0.006). According to regression logistic analysis duration of diabetes, older age, hypertension, and low left ventricular ejection fraction were independent predictor factors for IHD. Approximately one-third of diabetic patients are suffering from IHD. This disorder can be asymptomatic in individuals with diabetes, therefore, it may not be diagnosed in almost half of patients. Most important associated factors are older age, duration of diabetes, hypertension and low left ventricular ejection fraction.

Extramedullary hematopoiesis in the kidney and adrenal are rarely reported in medical literature and are usually found as incidentaloma. It usually occurs in patients with hematologic disorder such as thalassemia. The patient was a 23-year-old Iranian man with beta thalassemia who was admitted with a suprarenal mass. Adrenal mass was detected by ultrasonography and computed tomography. Results of biochemical evaluations were insignificant. The patient underwent right adrenalectomy. Gross and microscopic histopathologic examination demonstrated extramedullary hematopoiesis without any adrenal tissue. To the best of our knowledge, we document the first reported case of adrenal extramedullary hematopoiesis in Iran, which seems to be rare and remarkable.

To review the current situation regarding prenatal diagnosis (PND) in Islamic countries. A descriptive study (narrative review) has been done based on the available data in formal international and national published documents in 2013. The sources were papers, websites and electronic books. Time limitation of searches has started 20 years ago. The main languages were English and Persian. Frothy seven nations were officially referred as Islamic since more than 50% of the citizens are Muslims. The holy Qur''an and Islamic traditions (Shari''aht) are the core of the civil laws, however, the legal grounds for prenatal diagnosis differ in Islamic countries. The main ground is the endangerment of a mother''s life, however, severe suffering of parents (Osr va Haraj) is also considered in the Islamic Republic of Iran. Some other important issues such as pregnancies as a result of rape should be discussed more in some Islamic countries. Many “hard to treat diseases” such as chromosomal disorders, major hemoglobinopathies, inborn error of metabolism, Duchene muscular dystrophy, spinal muscular dystrophy are being diagnosed early in embryonic period that medical abortion is advisable. Prenatal diagnosis is an acceptable practice in both religious and secular governments in the so-called Islamic countries.

Abstract Hirsutism is a common symptom presenting to primary care endocrinologists, gynecologists, and dermatologists. Management is usually a long and troublesome process. This study was designed to evaluate the effect of fennel topical gel on mild to moderate idiopathic hirsutism. The randomized, double blind, placebo controlled clinical trial was carried out from 2009 to 2011, in Sari, Iran. Forty four women with mild to moderate idiopathic hirsutism were randomly divided to case and control groups, each group included 22 cases. The case group received fennel gel 3% and the control group received placebo. The effect of fennel gel 3% was defined as reduction of thickness of facial hair in micrometer by microscope in comparison with placebo. Measurements were performed at zero time and 24 weeks after treatment. This study was registered in the Iranian Registry of Clinical Trial (www.irct.ir) with registration number ID: IRCT138901213672N1. The mean age of patients was 26.9±6.7 and 25.6±4.3 years in case and control groups, respectively. Hair thickness was similar between the two groups before intervention. The hair thickness reduced from 97.9±31.5 to 75.6±26.7 micron in patients receiving fennel gel after 28 weeks (P<0.001). Four patients complained of itching (3 in case group) and 4 patients complained of irritation and itching (3 in case group). However, this difference was not statistically significant. The study indicated that fennel gel 3% is effective in decreasing hair thickness in women with idiopathic mild to moderate hirsutism

While thalassemia major (TM) used to be a prevalent genetic disease in the past, however, Phenylketonuria (PKU) is quite rare in spite of consanquiness marriage rate of about 40% in the region. Preventive efforts for TM started >20 years ago but neonatal screening for PKU started since 2007. This is the first report of co-existence of thalassemia and PKU in Middle East and in consideration of the prevalence of each genes, this chance association is a very unusual event. We report a case of having PKU and TM.

Background and Ôsteopenia and osteoporosis are well known and common complication of beta thalassemia major (TM).Ïn order to find the prevalence and related factors and also effect of ongoing treatment this study was done. Âll medical records of patients older than 16 years old were reviewed. Non transfusion dependent patients called as thalassemia intermediate. Demography of patients was extracted as well as, the result of the BMD by Nor land machine as Z-Score of femoral neck and lumbar vertebrate were reported comparing normal matched population. Z-Scores down to -1, -1 to -2.5, and less than -2.5 were considered as normal, osteopenic and osteoporotic respectively. Spss soft ware was used and Ôdds ratio and its 95% ÇÏ was calculated. BMD of TM and Ïntermediate patients was done by Mann-Whitney Ü test. Before and after BMD were compared by Wilcoxon Rank test. P<0.05 was considered statistically significant. 125 patients had at least one BMD measurement. Regarding the eligible patients approximately for one in every 4-5 patients there was a BMD. 73(58%) of patients were TM and reminder were not transfusion dependent. Mean age was 24 ± 6 and regarding gender, F/M ratio was 3/1. Âccording to Z-Scores, 17%, 61% and 17% were normal, osteopenic and osteoporotic respectively. Âge and severity of pathology were correlated and, and 7% reduction of BMD/ year was calculated (P<0.000). BMD of lumbar vertebra was worse in men (P<0.001). Â negative significant correlation was detected between BMD and the mean of hematocrite in the ast 5 years (P<0.005).Çorrelation of the age at starting transfusion and severity of BMD was not significant. Nineteen patients had 2 BMD measurements, 3 ± 1 years apart. Patients who had osteoporosis in the first measurements were using oral medicine (Âlendronate). No significant improvement was noticed and in fact femoral BMD was worse in men (P<0.001).Çonclusion: BMD measurements was less than anticipated. Ôsteopenia and even osteoporosis are common. Treatment duo to non compliance or inadequacy was not effective and more aggressive treatment is needed.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common disease of the hexose monophosphate pathway existing in more than 400 million people worldwide. The aim of this study was to identify neonates with G6PD deficiency following national program for screening and education of affected newborns’ parents started since June 2007 in Mazandaran, a northern Province of Iran. Blood sampling was performed via a heel prick prepared for screening of congenital hypothyroidism, Phenylketonuria, and G6PD deficiency. Fluorescent spot test kit set by Kimia Pajohan Company (Iran) was used. A confirmatory test with a venous blood sample was done within 4th month of age according to the protocol. The second enzyme activity test was a quantitative photometric method using a commercially available kit (Randox, U.K.) with sensitivity of 154 IU and normal range of 6.97- 20.5 U/gHb. Data were analyzed using SPSS software version 16 and descriptive methods. During 36 months, 115622 newborns (51.4 % male) were studied. G6PD enzyme deficiency was found in 6.1% of the newborns (CI95%= 5.92-6.28%). As expected, male/female ratio of affected newborns was 6.19:1. Of confirmed affected infants in second enzyme activity test, 86.1% were male and 13.9% were female. The reported rate of G6PD enzyme deficiency was less than expected (12-13%). We strongly recommend continuing screening and education of parents and also mass media education against consuming “Fava bean” and “Naphtalen”.