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2.  Long Term Follow up Study on a Large Group of Patients with Congenital Factor XIII Deficiency Treated Prophylactically with Fibrogammin P® 
Factor XIII deficiency (FXIIID) is an extremely rare hemorrhagic disorder with a prevalence of 1/3-5 million. Management of disease is performed by fresh frozen plasma (FFP), Cryoprecipitate (CP) or FXIII concentrate (Fibrogammin P®). Our objective was to assess safety and effectiveness of Fibrogammin P® in patients with FXIIID. For this purpose we designed this long-term follow up study on a large group of patients with FXIIID.
This prospective study was conducted on 213 patients with FXIIID since 2009 to 2013. Administrated dose for Fibrogammin P® according to clinical situations of patients ranged from 10 to 26 IU/kg every 4 – 6 weeks. All patients in 6-month intervals were checked for human immunodeficiency virus (HIV), hepatitis A, B and C viruses (HAV, HBV, HCV).
Twelve percent of participants had at least one ICH episode until 2008 but after administration of Fibrogammin P® did not have any major bleeding or episode of ICH, except in one patient. We also had 7 females with recurrent miscarriage that were managed successfully with a dose of 10 to 26 IU/kg every 4 – 6 weeks. This dose also was quite successful in management of major and minor surgery. None of the participants showed allergic reaction during treatment. A total of 7155450 IU of Fibrogammin P® were infused but nobody was positive for HIV, HAV, HBV, and HCV. We found that Fibrogammin P® is a safe and effective therapeutic choice in management of FXIIID.
PMCID: PMC5018293  PMID: 27642336
Factor XIII deficiency; Fibrogammin P®; Safety; Effectiveness
4.  The Effect of Educational-Spiritual Intervention on The Burnout of The Parents of School Age Children with Cancer: A Randomized Controlled Clinical Trial 
Background:
Parents of children with cancer experience high levels of stress and discomfort. Religious beliefs are important sources of comfort and support for many cancer patients and their families. The present study aimed to assess the effect of educational-spiritual intervention on burnout of the parents of the children with cancer.
Methods:
In this randomized clinical trial, 135 parents of children with cancer were randomly assigned into intervention and control groups. Data were collected through SMBQ (Shirom and Melamed Burnout Questionnaire) from both groups, before, immediately after and one month after the intervention. Educational-spiritual programs were held for six weeks, one session every week. The data were analyzed by SPSS using independent t-test, and repeated measure ANOVA.
Results:
The results showed that the mean burnout score before the intervention in the intervention group was 4.28±0.61 and in the control group it was 4.23±0.50; most of the parents reported moderate to high burnout. But, there was a significant difference between the intervention and control groups immediately after and one month after the intervention (t=10.16, P<0.0001). The mean burnout score in the intervention group was less than the control group. Results also showed that there was a significant difference between the two groups in terms of parental burnout in three times of measurements (F=58.62, P<0.0001).
Conclusion:
This study indicated that educational-spiritual intervention was effective on reduction of the burnout of the parents of the children with cancer. Due to high burnout of most of the parents, offering such a program could be beneficial for them. More studies in this regard are recommended.
Trial Registration Number: IRCT2014061818144N1
PMCID: PMC4709819  PMID: 26793734
Burnout; Cancer; Parents; Spirituality
5.  The ICET-A Survey on Current Criteria Used by Clinicians for the Assessment of Central Adrenal Insufficiency in Thalassemia: Analysis of Results and Recommendations 
Background
In March 2015, the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescent Medicine (ICET-A) implemented a two-step survey on central adrenal insufficiency (CAI) assessment in TM patients and after analysis of the collected data, recommendations for the assessment of hypothalamic-pituitary- adrenal (HPA) axis in clinical practice were defined.
Methods
To ascertain the current practice for assessment of CAI in thalassemia, the Coordinator of ICET-A sent two questionnaires by email: i) The first to evaluate the current interpretation of basal serum cortisol level (first step) and ii) The second to assess the current usage of ACTH test and the variability in practice” (second step). Based on the surveys the core ICET-A group prepared the recommendations for the assessment of suspected CAI in thalassemia (third step).
Results
A total of 19 thalassemologists/endocrinologists have participated in the first survey and 35 specialists participated in the second step questionnaire. The study demonstrated a considerable variability in almost all aspects of relevant current criteria used for the diagnosis of CAI. An ROC analysis using peak value > 20 μg/dl (> 550 nmol/L), after ACTH stimulation test, was performed with the aim of identifying the optimal basal serum cortisol cut-off. The optimal threshold that maximizes sensitivity plus specificity for morning basal cortisol against peak post-ACTH value >20 μg/dl (>550 nmol/L) was 10 μg/dl (275 nmol/L). Furthermore, the values associated with the highest negative predictive value (NPV) and highest, positive predictive value (PPV) were 4.20 (115 nmol/L) and 18.45 μg/dl (510 nmol/L), respectively.
Surprisingly, 20 specialists in thalassemia working in blood bank, thalassemia centres (day hospital), internal medicine, hematology and onco-hematology had poor knowledge and experience in testing for CAI and stopped filling the questionnaire after the second question. In contrast, 9 endocrinologists (8 pediatricians) and 6 hematologists working in collaboration with endocrinologists completed the questionnaire.
Conclusions
While waiting for more extensive adequately powered and targeted studies, physicians should adopt an acceptable policy for accurate assessment of HPA in TM patients. Regular surveillance, early diagnosis, treatment and follow-up in a multi-disciplinary specialized setting are also recommended. The ICET-A recommendations are reported in order to facilitate for interested physicians the approach to a successful assessment of adrenal function in thalassemia.
doi:10.4084/MJHID.2016.034
PMCID: PMC4928531  PMID: 27413526
6.  Relationship Between Serum Hepcidin and Ferritin Levels in Patients With Thalassemia Major and Intermedia in Southern Iran 
Background:
Hepcidin is a key regulator of iron absorption in humans. It is mainly affected by hypoxia and iron stores.
Objectives:
The current study aimed to determine the correlation between serum hepcidin and ferritin levels in patients with Thalassemia Major (TM) and Thalassemia Intermedia (TI).
Patients and Methods:
The current cross-sectional study investigated 88 randomly selected patients with Thalassemia, 48 TM and 40 TI, registered at the Thalassemia Clinic of Shiraz University of Medical Sciences, a referral center for Thalassemia in Southern Iran in 2013. All patients with TI were receiving Hydroxyurea (HU) 10 - 15 mg/kg/day for at least 10 years. The serum hepcidin, ferritin levels, hemoglobin (Hb) and nucleated Red Blood Cell (RBC) of the two groups were measured.
Results:
No statistically significant correlation was observed between serum hepcidin and ferritin levels in any of the two groups of patients with TM (rs = 0.02, P = 0.892) or TI (rs = 0.055, P = 0.734). The median Interquartile Range (IQR) for serum hepcidin and ferritin levels were significantly higher in TM compared to TI group, (hepcidin: 87.6 (43.9) vs. 51.8 (23.4), P < 0.001; ferritin: 2208 (3761) vs. 465 (632), P < 0.001).
Conclusions:
There was insignificant correlation between serum hepcidin and ferritin levels in the two groups of patients with TM and TI. It seems that regulation of hepcidin in patients with Thalassemia is more affected by erythropoeitic activity than iron stores. Also, hepcidin levels were significantly higher in patients with TM than TI, possibly due to higher erythropoeitic activity in TI. In TI, it seems that low dose HU increases Hb levels and leads to transfusion-independence, but it is not high enough to suppress bone marrow activity and ineffective erythropoiesis. Consequently, serum hepcidin level decreases.
doi:10.5812/ircmj.17(5)2015.28343
PMCID: PMC4583770  PMID: 26421179
Hepcidin; Hydroxyurea; Ferritin; Thalassemia Intermedia; Thalassemia Major
7.  Infantile fibrosarcoma of ethmoid sinus, misdiagnosed as an adenoid in a 5-year-old child 
Infantile fibrosarcoma of head and neck is rare and the presence of this tumor in ethmoid sinus is even more uncommon. To the best of our knowledge, <5 cases have been reported in the last 20 years in the English literature, so far, only one of which has been infantile type in a 15 months old girl. In this case report, we will explain our experience with a rare case of infantile fibrosarcoma originating from ethmoid sinus in a 5-year-old boy who presented with dyspnea and epistaxis. After biopsy, it was diagnosed as fibrosarcoma of sinus origin.
doi:10.4103/0973-029X.164567
PMCID: PMC4611951  PMID: 26604519
Ethmoid sinus; epistaxis; infantile fibrosarcoma
8.  The Effect of Yoga on the Quality of Life in the Children and Adolescents with Haemophilia 
The problems caused by haemophilia lead to impairments of the quality of life in patients with haemophilia. This study aimed to investigate the effects of yoga on quality of life in the children and adolescents with haemophilia in Shiraz, Iran. This semi-experimental study with pre- and post-test design was performed on 27 boys between 8 and 16 years old who suffered from haemophilia. The patients were divided into two groups. The number of bleedings, absences from school, and referrals to the clinic was evaluated. The demographic data were collected through interviews and using the patients’ records in the haemophilia center. Besides, the quality of life was assessed through the Haemo-QoL questionnaire. Then, the yoga intervention was performed for 14 weeks and the data were collected in three stages. The collected data were entered into the SPSS statistical software, version 18 and were analyzed using non-parametric Friedman test. After the intervention, significant differences were observed in the mean scores of quality of life dimensions and the number of bleedings, school absences, and referrals to the haemophilia clinic (P<0.001). Thus, yoga may improve the haemophilia children’s and adolescents’ perception of quality of life without the risk of injury. This intervention also seemed to be effective in reducing the number of bleedings, referrals to the haemophilia clinic, and absences from school.
PMCID: PMC4441353  PMID: 26005695
Quality of life; Haemophilia; Children; Adolescents; Yoga
9.  Serum Ferritin Levels Correlation With Heart and Liver MRI and LIC in Patients With Transfusion-Dependent Thalassemia 
Background:
Iron-loaded cardiac complication is the essential cause of mortality in patients with thalassemia. Early detection and treatment of cardiac over-load can reduce mortality.
Objectives:
The current study aimed to evaluate the relationship between serum ferritin levels and T2* magnetic resonance imaging (MRI) of heart and liver and liver iron concentration (LIC) to diagnose iron over load in countries with limited access.
Patients and Methods:
In the current cross-sectional study, 85 Iranian patients with thalassemia with the mean age of 22.7 ± 7 years were randomly selected. All patients were on regular blood transfusion. Echocardiography of heart and liver T2* MRI, determination of serum ferritin levels, and LIC were performed in all subjects at the same time. The correlation of serum ferritin levels with T2*MRI of heart and liver, and LIC was assessed. P value < 0.05 was considered statistically significant.
Results:
Abnormal myocardial iron load (T2* MRI < 20 ms) was detected in 58% of the patients and among whom, 36% had severe myocardial iron load (T2* MRI < 10 ms). Median and interquartile range of serum ferritin levels were 1434 and 2702 respectively in patients with thalassemia. Serum ferritin levels showed a statistically significant positive correlation with LIC (rs = 0.718, P < 0.001) and significant negative correlation with T2* Heart (rs = -0.329, P = 0.002), and T2* Liver (rs = -0.698, P < 0.001). However, Ejection fraction was not significantly correlated with serum ferritin levels in the patients (P = 0.399).
Conclusions:
Serum ferritin levels can be used to diagnose iron over-load in patients with thalassemiaas an alternative method in areas where T2* MRI is not available.
doi:10.5812/ircmj.17(4)2015.24959
PMCID: PMC4443302  PMID: 26023341
Beta-thalassemia; Ferritin; Magnetic Resonance Imaging; Liver; Heart
10.  Comparative Study of Radiographic and Laboratory Findings Between Beta Thalassemia Major and Beta Thalassemia Intermedia Patients With and Without Treatment by Hydroxyurea 
Background:
In patients with thalassemia, chronic anemia causes bone marrow expansion and consequently skeletal manifestation in spine, skull, face and rib bones.
Objectives:
We aimed to compare chest radiographic findings and facial bone deformity in patients with thalassemia major (TM) and intermedia.
Patients and Methods:
In this cross sectional study, 86 consecutive thalassemia patients referring to the Thalassemia clinic in Shiraz, Southern Iran were evaluated during 2012. Patients were divided into three groups including TM and thalassemia intermedia (TI) with and without taking hydroxyurea (HU). Findings ofchest radiography (trabeculation, rib widening and paraspinal masses) as well as facial bone deformity were evaluated by an expert radiologist. Besides, laboratory findings were measured regarding hemoglobin, ferritin, NRBC and platelet count.
Results:
All radiologic findings were significantly higher in patients with TI compared to TM (P< 0.05). In patients with TI, only trabeculation was observed with a higher frequency in patients with HU compared to those without HU (68% vs. 27.3%, P= 0.008). In the regression model, from all variables evaluated, only NRBC showed a significant correlation with trabeculation (Exp B = 1.014, CI: 1.004-1.025, P = 0.008) and age showed a significant correlation with paravertebral mass (Exp B = 1.147, CI: 1.03-1.27, P = 0.013).
Conclusions:
In patients with TM, bone widening, trabeculation, paraspinal masses and facial bone deformity were lower than patients with TI, whichcan be related to effectiveness of therapy with blood transfusion irrespective of its adverse effects in TM patients.
doi:10.5812/ircmj.23607
PMCID: PMC4376990  PMID: 25838937
Beta-Thalassemia; Hydroxyurea; Mass Chest X-Ray
11.  Effect of Coping Strategies Training On Its Use by Thalassemia Major Adolescents: A Randomized Controlled Clinical Trial 
Background: Thalassemia is a chronic disease with serious clinical and psychological challenges. The incidence of thalassemia in a family member may cause a psychological crisis in all family members and in this case coping strategies are required. This clinical trial study aimed to determine the impact of training coping strategies on their use by major thalassemic adolescents referred to Dastgheib Hospital in Shiraz.
Methods:  In this randomized clinical trial, 87 adolescents with major thalassemia who were randomly assigned to two groups of experiment and control were enrolled. Then the experiment group attended six sessions of coping strategies training program in isolation, each lasting for one and half hour. In order to examine the coping strategies, Jalowice’s coping strategy questionnaire was used in three periods including pre-intervention period and one month and two months after the intervention. The collected data were analyzed using independent t-test and Chi-square.
Results: Mean scores of problem-focused coping strategies in the experiment group increased in one month and two months after the intervention from 45±12.7 to 54.8±7.3 and 55.7±7.2, respectively. Also, the difference in mean scores of problem-focused coping strategies was significant in the two groups (P<0.001).  Furthermore, the difference in mean scores of emotion-focused coping strategies was significantly different between the two groups in two months after the intervention (P=0.007).
Conclusion: Based on the obtained results, teaching coping strategies has improved the use of problem-focused coping strategies and also effective coping with stress and disease problems in patients with thalassemia major. Therefore, it is recommended that authorities should consider this as a part of treatment program.
Trial Registration Number: IRCT2013112215484N1
PMCID: PMC4280559  PMID: 25553336
Coping; Education; Thalassemia major
12.  The Frequency of Adrenal Insufficiency in Adolescents and Young Adults with Thalassemia Major versus Thalassemia Intermedia in Iran 
Background
Endocrine dysfunction is not uncommon complication in patients with transfusion-dependent thalassemia and is thought to occur as a consequence of excessive iron overload. The primary objective of this study is to determine the frequency of adrenal insufficiency in patients with thalassemia major and thalassemia intermediate.
Methods
This cross-sectional study was done at the Shiraz University of Medical Sciences, Shiraz, Southern Iran, in 2013. One hundred and ninety patients were divided into two groups; thalassemia major(TM) and thalassemia intermediate (TI) groups. We measured 8 AM serum cortisol, ACTH and ferritin concentrations in all patients.
Results
The mean age of the TM and TI group were 22.5±5.7 and 23.8±6 years, respectively. 90 patients (47.4%) were splenectomized, 34 (36.2%) with TM and 56 (58.2%) with TI (p :<0.001). The median and interquartile range of serum ferritin levels were 2184±3700 ng/ml and 437±443ng/ml in TM and TI respectively (p< 0.001). Three patients with TM (1.6%) had low basal cortisol and ACTH levels. However, their cortisol response to ACTH stimulation was normal.
Conclusions
Low basal concentrations of cortisol and ACTH occurred in 1.6% of our adolescents young adult patients with TM suggesting a central defect in cortisol secretion at the basal state. However, cortisol response to standard – dose ACTH was normal in all patients with TM and TI.
doi:10.4084/MJHID.2015.005
PMCID: PMC4283918  PMID: 25574364
13.  Serum cancer antigen 15.3 concentrations in patients with betathalassemia minor compared to those with cancer and healthy individuals 
Background: High serum level of cancer antigen 15.3 (CA15.3) has been reported in some malignant and nonmalignant conditions including thalassemia major which could have been resulted from ineffective erythropoiesis. We aimed to evaluate the serum level of CA15.3 in carriers of beta-thalassemia by comparing them with cancer patients and healthy individuals.
Methods: This cross-sectional study was done from February to December 2011 in Southern Iran. Participants consisted of 32 subjects with beta-thalassemia minor, 49 with cancer and 25 healthy individuals. The serum levels of CA15.3 were measured and compared in different groups.
Results: The serum levels of CA 15.3 in all participants were in the normal range (<35 U/mL). Also it did not significantly differ among various groups of the participants (p=0.723). Age was not significantly correlated with the serum level of CA 15.3 (r= 0.039, p=0.702). The most frequent cancer in the group of patients with malignancies was hematologic malignancies (96%) with the highest frequency for acute lymphoblastic leukemia (37 patients). Frequency of thalassemia minor in patients with cancer was 11 (22.4%).
Conclusion: No correlation was found between CA 15.3 serum level with beta-thalssemia minor or with childhood malignancies. Compared to general population, a high proportion of beta-thalssemia minor was observed in patients with cancer in our study. Future prospective studies are needed to evaluate the relationship between cancer and beta-thalassemia minor accurately.
PMCID: PMC4301236  PMID: 25664292
Beta-thalassemia minor; Cancer; Cancer antigen 15.3
14.  Frequency of Cholelithiasis in Patients With Beta-Thalassemia Intermedia With and Without Hydroxyurea 
Background:
Recent studies regarding the effect of hydroxyurea (HU) in thalassemia have revealed favorable effects on the reduction of ineffective erythropoiesis.
Objectives:
The aim of the current study was to evaluate whether or not HU can have an effect on the gallstone formation rate in patients with beta-thalassemia intermedia (BTI).
Patients and Methods:
In this case control cross-sectional study, from a total of 250 transfusion-independent BTI patients, 51 patients who were taking HU, participated in the study. Patients were registered in the Thalassemia clinic of Shiraz University of Medical Sciences, Shiraz, which is a referral center located in southern Iran, during 2011-2012. Mean dose of HU consumption in the case group was 10 ± 2.5 mg/kg/day (range of 8-15 mg/kg/day), with a mean duration of consumption of 7.5 ± 3.8 years (range 1-14 years). In addition, 41 age- and sex-matched BTI patients who did not use HU were randomly selected as a control group. All patients underwent abdominal ultrasound by a radiologist for evaluation of gallstones.
Results:
Mean age of the participants was 21.4 ± 6.5 years (10-40 years). There was no statistically significant difference regarding the frequency of cholelithiasis between the two groups of patients (P = 0.822). Our study showed significantly lower hemoglobin levels and a higher percentage of nucleated red blood cells in the HU group compared with the control group (P = 0.001 and P = 0.005, respectively).
Conclusions:
It seems that taking HU for long periods can reduce hemolysis and bone marrow suppression, and that decreases the rate of cholelithiasis. We believe that if these patients had not been treated with HU, we would probably have observed a significantly higher frequency of cholelithiasis, due to more hemolysis compared with patients not taking HU. Further studies with larger sample sizes are suggested.
doi:10.5812/ircmj.18712
PMCID: PMC4166105  PMID: 25237586
Beta Thalassemia Intermedia; Cholelithiasis; Hydroxyurea
15.  Hydroxyurea Treatment in Transfusion-Dependent β-Thalassemia Patients 
Background:
β-Thalassemia is an inherited hemoglobin disorder caused by defective synthesis of ß-globin chains. Hemoglobin (Hb) F induction is a possible therapeutic approach which can partially compensate for α and non-α globin chains imbalance.
Objectives:
We aimed to investigate the efficacy and safety of Hydroxyurea (HU) in diminishing transfusion requirements of patients with β-thalassemia major in Southern Iran.
Patients and Methods:
In this single-arm clinical trial, all transfusion-dependent β-thalassemia patients older than two years old (n = 97) who had inclusion criteria of the study and had been registered for at least six months in Dastgheib thalassemia outpatient clinic (a referral center affiliated to Shiraz University of Medical Sciences) were evaluated from October 2010 to December 2011. The patients were treated with HU with a mean dose of 10.5 mg/kg for a mean duration of 8 months (range 3-14 months). Transfusion needs and Hb levels were compared before and after HU treatment.
Results:
The mean volume of blood transfusion decreased significantly following HU treatment (0.71 mL/kg/day vs. 0.43 mL/kg/day, P < 0.001). Two-thirds of the patients showed good and partial response. No serious adverse reaction was observed except persistent neutropenia in two patients.
Conclusions:
Hydroxyurea can be safely used in some transfusion-dependent β-thalassemia patients to decrease their transfusion needs.
doi:10.5812/ircmj.18028
PMCID: PMC4102988  PMID: 25068055
Hydroxyurea; Blood Transfusion; β-Thalassemia
16.  The effect of metoprolol succinate on the cardiac function of patients with thalassaemia cardiomyopathy: a double-blind randomised study 
Heart Asia  2014;6(1):54-58.
Background
Heart failure is the most common cause of mortality in β-thalassaemia major. However, the management of this disease, apart from chelation therapy, is largely empirical. Therefore, we decided to evaluate the effect of metoprolol succinate on patients with thalassaemia cardiomyopathy (TCM).
Materials and methods
In this clinical trial, 45 patients with TCM were randomised to receive either metoprolol (n=26) or placebo (n=19). Echocardiography and a 6 min walk test were performed at baseline and repeated after 6 months and the values compared.
Results
In the metoprolol group, left ventricular ejection fraction (LVEF) rose from 38.65% to 42.84% (p<0.001), while it decreased in the placebo group from 37.89% to 35.84% (p=0.01); the difference between the two groups was significant (p<0.001). Left ventricular (LV) mass in the metoprolol group decreased from 154.31 to 144.26 g (p=0.02), while in the placebo group it increased from 174.32 to 200.15 g (p=0.68); the difference between the two groups was significant (p<0.001). End systolic volume (ESV) decreased in the metoprolol group from 42.19 to 36.73 cm3 (p<0.001) but increased from 47.37 to 57.42 cm3 in the placebo group (p=0.144); the difference between the groups was significant (p<0.001). No differences in exercise capacity or pulmonary capillary wedge pressure were seen between the two groups (p=0.268 and p=0.535, respectively).
Conclusions
Metoprolol succinate as a β-blocker may have the potential to significantly improve systolic function in patients with TCM and reverse LV remodelling to the same extent as in other types of cardiomyopathy.
Trial registration number
NCT01863173.
doi:10.1136/heartasia-2013-010460
PMCID: PMC4832708  PMID: 27326168
17.  Evaluation of Red Cell Membrane Cytoskeletal Disorders Using a Flow Cytometric Method in South Iran 
Turkish Journal of Hematology  2014;31(1):25-31.
Objective: The diagnosis of hereditary red blood cell (RBC) membrane disorders, and in particular hereditary spherocytosis (HS) and Southeast Asian ovalocytosis (SAO), is based on clinical history, RBC morphology, and other conventional tests such as osmotic fragility. However, there are some milder cases of these disorders that are difficult to diagnose. The application of eosin-5’-maleimide (EMA) was evaluated for screening of RBC membrane defects along with some other anemias. We used EMA dye, which binds mostly to band 3 protein and to a lesser extent some other membrane proteins, for screening of some membrane defects such as HS.
Materials and Methods: Fresh RBCs from hematologically normal controls and patients with HS, SAO, hereditary elliptocytosis, hereditary spherocytosis with pincered cells, severe iron deficiency, thalassemia minor, and autoimmune hemolytic anemia were stained with EMA dye and analyzed for mean fluorescent intensity (MFI) using a flow cytometer.
Results: RBCs from patients with HS and iron deficiency showed a significant reduction in MFI compared to those from normal controls (p<0.0001 and p<0.001, respectively), while macrocytic RBCs showed a significant increase in MFI (p<0.01). A significant correlation was shown between mean corpuscular volume and MFI, with the exceptions of HS and thalassemia minor.
Conclusion: Our results showed that the flow cytometric method could be a reliable diagnostic method for screening and confirmation, with higher sensitivity and specificity (95% and 93%, respectively) than conventional routine tests for HS patients prior to further specific membrane protein molecular tests.
doi:10.4274/Tjh.2012.0146
PMCID: PMC3996639  PMID: 24764726
RBC; Membrane disorders; Band 3; Flow cytometry
18.  Insulin-like Growth Factor-1 (IGF-1): Demographic, Clinical and Laboratory Data in 120 Consecutive Adult Patients with Thalassaemia Major 
Introduction
IGF-1 deficiency in TM patients in children and adolescents has been attributed to chronic anemia and hypoxia, chronic liver disease, iron overload and other associated endocrinopathies, e.g. growth hormone deficiency (GHD). Few data are available in the literature regarding adult TM patients and growth disorders. The aim of this study was to measure IGF-1 values and other clinical data in a large number of adult patients with TM to evaluate the possible relationships between them.
Patients and Methods
A cohort of 120 adult patients with TM was studied for plasma levels of IGF-1. Plasma total IGF-1 was determined by chemiluminescent immunometric assay (CLIA) method. In eleven patients (3 females) the GH response during glucagon stimulation test (GST) was also evaluated.
Results
Fifty percent of patients (33 males and 27 females) had IGF-1 levels <- 2 SDs below normative values for healthy subjects matched for age and sex. In these patients endocrine complications and elevations of aminotransferases (ALT) were more common compared to TM patients with IGF1 > -2SDs. In multivariate regression analyses, height, weight, BMI, serum ferritin, ALT, HCV serology and left ventricular ejection fraction (LVEF) were not significantly related to IGF-1, but a significant correlation was found in females between HCV-RNA positivity and IGF-1, ALT and serum ferritin. AGHD was diagnosed in 6 (4 males) out of 11 patients (54.5%) who had glucagon stimulation tests and in 5 out of 8 (62.5%) with IGF-1 <-2SD. The mean age of patients with GHD was 39.3 years (range: 25–49 years, median: 39 years) versus 35.8 years (range: 27–45 years, median: 37.5 years) in non-GHD patients. A positive correlation between GH peak after GST and IGF-1 level was found (r: 0.6409; p: < 0.05).
Conclusions
In 50% of TM patients the IGF-1 levels were 2SDs below average values for healthy individuals. IGF-1 deficiency was more common in TM patients with associated endocrine complications, and a significant correlation was found in HCV-RNA positive females among IGF-1, ALT, and serum ferritin. Further data in a larger group of patients are needed to confirm whether IGF-1 level <-2 SDs may be a potential criterion for additional studies in TM patients. This datum could avoid performing GH stimulation tests in the majority of them.
doi:10.4084/MJHID.2014.074
PMCID: PMC4235482  PMID: 25408860
19.  Hypothyroidism in β-Thalassemia Intermedia Patients with and without Hydroxyurea 
Hydroxyurea (HU) has been successfully used in patients with β-thalassemia intermedia (β-TI). We aimed to evaluate the effect of the long-term use of HU on thyroid function in patients with β-TI. Seventy-five patients with β-TI aged≥11 years and taking HU were randomly selected during 2010 in southern Iran. Thirty-one patients with β-TI without HU were considered as a control group. Serum levels of thyroid stimulating hormone (TSH) and T4 were measured. The mean age of the participants was 22.7±5.1 years (age range=12-41 years). Serum ferritin level had no significant correlation with HU consumption (P>0.05). Overall, we detected 10 (9.4%) patients with hypothyroidism. We found that the use of HU at a dose of 8-15 mg/kg/day has no significant association with thyroid function in β-TI patients. However, due to the small sample size in our study, documentation of this finding needs further studies with higher numbers of patients.
PMCID: PMC3895896  PMID: 24453395
Thalassemia intermedia; Hydroxyurea; Thyroid function
20.  Management of Bleeding in Post-liver Disease, Surgery and Biopsy in Patients With High Uncorrected International Normalized Ratio With Prothrombin Complex Concentrate: An Iranian Experience 
Background:
To evaluate the efficacy of prothrombin complex concentrate (PCC) in the management of bleeding in patients with liver disease and patients undergoing surgery or biopsy who had a high uncorrected international normalized ratio (INR).
Objectives:
In this study, we examined an Iranian sample and investigated the efficacy of PCC to manage bleeding in patients with liver disease and also patients with high uncorrected INR who were scheduled for surgery or biopsy.
Materials and Methods:
A total of 25 patients including 16 patients with post-liver disease bleeding (group 1) and 9 patients with high uncorrected INR who were scheduled for surgery or biopsy (group 2) were enrolled. All patients were treated with 25 IU/kg PCC, and efficacy was defined as any reduction in or cessation of bleeding episodes and correction of INR before surgery or biopsy. The patients were also evaluated for any adverse effects.
Results:
INR decreased significantly in both groups of patients, with no bleeding episodes during or after the study in group 1 and during or after surgery/biopsy in group 2. All patients tolerated the therapy well without any significant adverse effects.
Conclusions:
The efficacy of PCC therapy was satisfactory in this study. PCC therapy in patients with liver disease and patients undergoing surgery or biopsy seems to be effective and safe, and may be a good treatment strategy for these patients, if fresh frozen plasma or vitamin K are not effective.
doi:10.5812/ircmj.12260
PMCID: PMC3955503  PMID: 24693388
Prothrombin Complex Concentrates; Liver Diseases; General Surgery; International Normalized Ratio
21.  Inherited Thrombophilia and Recurrent Pregnancy Loss 
Background:
Recurrent pregnancy loss (RPL) is a common health problem. The polymorphisms G20210A of prothrombin gene (FII G 20210A), and G 1691A of factor V gene (Factor V Leiden, FVL) are the most extensively studied thrombophilic mutations in association to recurrent miscarriage.
Objectives:
To determine the frequency of FII G20210A and FVL polymorphisms as well as protein C and protein S deficiency in a series of patients with RPL compared with control group.
Patients and Methods:
The study group included 90 randomly selected patients with three or more consecutive miscarriages with the same partner in <20 weeks gestation in 2012. The control population consisted of 44 age-matched women with at least one live born children and no history of pregnancy loss. Functional activity of protein C and S, activated protein C resistance, FVL assay by polymerase chain reaction and prothrombin gene mutation were assessed. The polymorphism frequencies were recorded for each group and comparisons were made.
Results:
The mean functional activity of protein C and protein S were not significantly different between case and control groups (P >0.05). Frequency of protein C deficiency was also not significantly different between the case and control groups (P=0.906), but frequency of protein S deficiency was significantly higher in patients than controls (P=0.03). Genotype pattern of the patients and healthy individuals were not significantly different with regard to either FVL or Prothrombin G20210A (P > 0.05).
Conclusions:
We determined a significant higher frequency of protein S deficiency in patients with RPL compared with controls. But the frequency of protein C deficiency and the frequency of two common thrombophilic mutations (Factor V Leiden and Prothrombin G20210A), were not significantly different between patients with recurrent miscarriage and healthy women.
doi:10.5812/ircmj.13708
PMCID: PMC3955508  PMID: 24693393
Inherited; Pregnancy loss; Thrombophilia
22.  Unexpected postmortem diagnosis of acanthamoeba meningoencephalitis in an immunocompetent child 
BMJ Case Reports  2011;2011:bcr0320113954.
Meningoencephalitis caused by Acanthamoeba spp. is a rare opportunistic infection, difficult to diagnose and treat, which causes death in almost all cases. Here, the authors report a 5-year-old Iranian immunocompetent girl who died of fulminant acanthamoeba meningoencephalitis. To the authors’ knowledge, this is the first case of acanthamoeba meningoencephalitis in Iran.
doi:10.1136/bcr.03.2011.3954
PMCID: PMC3189635  PMID: 22679147
23.  Diffuse Hepatic Calcifications in a Transfusion-Dependent Patient with Beta-Thalassemia: A Case Report 
Hepatic calcification is usually associated with infectious, vascular, or neoplastic processes in the liver. We report the first case of beta-thalassemia major with isolated diffuse hepatic calcification in a 23 year old woman, who had been transfusion-dependent since the age of 6 months. She was referred to our center with a chief complaint of abdominal pain. Computed tomography scan of the abdomen revealed diffuse hepatic calcification in the right, left, and caudate lobes of the liver. Her medical history disclosed hypoparathyroidism as well as chronic hepatitis C virus infection, which was successfully treated but led to early micronodular cirrhosis on liver biopsy. Other studies done to search for the cause of hepatic calcification failed to reveal any abnormalities. We suspect that hypoparathyroidism caused liver calcification, and should be, therefore, considered in the differential diagnosis of hepatic calcification if other causative factors have been ruled out.
PMCID: PMC3808953  PMID: 24174700
Beta-Thalassemia major; Hepatic; Calcification; Hypoparathyroidism
24.  DDAVP Might Reduce the Risk of Preeclampsia in Pregnant Women with VWF Deficiency 
Turkish Journal of Hematology  2013;30(2):214-215.
doi:10.4274/Tjh.2012.0044
PMCID: PMC3878470  PMID: 24385790
DDAVP; preeclampsia; Pregnancy; deficiency; Platelet
25.  Exposure to Second-hand Smoke During Pregnancy and Preterm Delivery 
Background
Prematurity is an issue related to increasing the neonatal morbidity and mortality and smoking pregnant women cause the risk of low birth weight and prematurity increase, compared to non-smoking ones.
Objectives
This study investigates second-hand smoke (SHS) exposure’s effects over pregnant women on gestational age and birth weight.
Materials and Methods
In this descriptive-analytic study, 205 women referred to both public and private hospitals in the third trimester were questioned about second-hand smoke (SHS) exposure during pregnancy. In addition to birth weight and gestational age, other variables including mother’s education and job and sex of the newborns were also assessed.
Results
Of all 205 women, 43 (20.97%) women exposed to SHS during pregnancy and 162 (79.02%) women did not. In SHS exposure group, 11 infant (25.6%) and in non- SHS exposure group, 17 infant (10.5%), were born prematurely (< 37 weeks) (P = 0.01). Also birth weight of newborn in non-SHS exposure group was 118 gram more than other group but the differences were not significant (P = 0.09).
Conclusions
Our findings showed that the secondhand smoke (SHS) exposure of pregnant women may be significantly associated with early preterm delivery.
doi:10.5812/ijhrba.7630
PMCID: PMC4070123  PMID: 24971254
Smoke; Pregnancy; Preterm Delivery; low birth weight

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