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1.  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
2.  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
Prothrombin Complex Concentrates; Liver Diseases; General Surgery; International Normalized Ratio
3.  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
Inherited; Pregnancy loss; Thrombophilia
4.  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
5.  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
6.  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
7.  Transcranial Doppler Ultrasonography in Beta-thalassemia Major Patients Without and With Thrombocytosis 
Background
Beta-thalassemia is a type of anemia in which the patients may require splenectomy and this can lead to thrombocytosis with increased risk of stroke. Transcranial Doppler ultrasound is a method for determining cerebral vessel stenosis.
Objectives
The aim of this study was to investigate whether the risk of a future stroke secondary to cerebral artery stenosis can be predicted with the use of transcranial Doppler ultrasound in beta-thalassemia major patients.
Patients and Methods
This study included 54 beta-thalassemia major patients divided into 2 groups; group A consisted of 28 patients who have thrombocytosis secondary to a previous splenectomy and group B comprised of 26 patients who did not have a splenectomy with normal platelet count, as well as a control group of 30 healthy individuals.
Results
Transcranial Doppler ultrasound of the cerebral vessels were performed in all participants, and the results for each group were compared with the controls. In addition, patients were evaluated for evidence of high flow velocity in the cerebral vessels that met the clinically significant criteria of ≥ 50% stenosis. Transcranial Doppler ultrasound velocity criteria for > 50% stenosis, indicating a risk of stroke, were not documented in any patients but increase in cerebral blood velocities in many arteries in group A and in some arteries in group B were revealed.
Conclusion
Following splenectomy, thrombocytosis can predispose the patients to an increase in cerebral blood velocities more than respected with anemia. But by transcranial doppler ultrasonography no evidence of significant stenosis were found in intracerebral arteries to conclude that the beta-thalassemia major patients were more prone to the development of stroke secondary to this abnormality.
doi:10.5812/ircmj.4606
PMCID: PMC3745753  PMID: 23984004
Beta-thalassemia; Thrombocytosis; Ultrasonography, Doppler, Transcranial
8.  Survival Rate of Childhood Leukemia in Shiraz, Southern Iran 
Iranian Journal of Pediatrics  2013;23(1):53-58.
Objective
Leukemia is the most prevalent type of cancer in children. The aim of this study was to estimate the 5-year survival rates of Acute Lymphoblastic Leukemia (ALL) and Acute Myeloblastic Leukemia (AML) as well as factors influencing them.
Methods
This is a nonrandomized retrospective study conducted on 280 patients with ALL and AML. They were all below 15 years old children admitted to Shahid Faghihi hospital, Shiraz, Iran from 2004 to 2008.The survival rates were estimated by applying the Kaplan-Meier method. In addition, the log rank test was used to estimate the statistical significance of differences in the survival probability. Cox regression model was applied to conduct multivariate analysis for adjusting confounding variable. All analyses were performed in SPSS statistical software (version 16). P-values less than 0.05 were considered as statistically significant.
Findings
The mean (± standard deviation) of the observation period was 28.2±16.1 months. In this period, 60 (24.7%) patients (47 ALL and 15 AML) passed away. The cumulative rate of survival in this study was 53.3±0.1 percent. This probability was 56.6±0.1% and 44.2±0.1% for ALL and AML patients, respectively, which indicates no statistically significant difference between them (P=0.8). According to Cox model, there was a significant relationship among the variables of platelet count and relapse with the survival rate.
Conclusion
Platelet count was identified as a positive prognostic factor of the survival rate in ALL patients. However, on the base of our results and other studies, incidence of relapse and the number of relapses are significant factors of survival rates of leukemia.
PMCID: PMC3574992  PMID: 23550191
Lymphoblastic Leukemia; Myeloblastic Leukemia; Survival Rate; Relapse
9.  Growth and endocrine disorders in thalassemia: The international network on endocrine complications in thalassemia (I-CET) position statement and guidelines 
The current management of thalassemia includes regular transfusion programs and chelation therapy. It is important that physicians be aware that endocrine abnormalities frequently develop mainly in those patients with significant iron overload due to poor compliance to treatment, particularly after the age of 10 years. Since the quality of life of thalassemia patients is a fundamental aim, it is vital to monitor carefully their growth and pubertal development in order to detect abnormalities and to initiate appropriate and early treatment. Abnormalities should be identified and treatment initiated in consultation with a pediatric or an adult endocrinologist and managed accordingly. Appropriate management shall put in consideration many factors such as age, severity of iron overload, presence of chronic liver disease, thrombophilia status, and the presence of psychological problems. All these issues must be discussed by the physician in charge of the patient's care, the endocrinologist and the patient himself. Because any progress in research in the field of early diagnosis and management of growth disorders and endocrine complications in thalassemia should be passed on to and applied adequately to all those suffering from the disease, on the 8 May 2009 in Ferrara, the International Network on Endocrine Complications in Thalassemia (I-CET) was founded in order to transmit the latest information on these disorders to the treating physicians. The I-CET position statement outlined in this document applies to patients with transfusion-dependent thalassemia major to help physicians to anticipate, diagnose, and manage these complications properly.
doi:10.4103/2230-8210.107808
PMCID: PMC3659911  PMID: 23776848
Endocrine complications; growth; guidelines; iron overload; thalassemia; treatment
11.  A Case Report of Congenital Insensitivity to Pain and Anhidrosis (CIPA) 
Congenital insensitivity to pain and anhidrosis (CIPA) or hereditary sensory autonomic neuropathies type IV (HSAN type IV) is an extremely rare autosomal recessive disorder initially described by Swanson in 1963.
We report a 2.5-year-old boy with clinical features of CIPA as the first case in Iran. The symptoms included recurrent episodes of hyperthermia and unexplained fever that began in early infancy, anhidrosis (inability to sweat), profound loss of pain sensitivity, neurodevelopmental delay, unconscious self-mutilation of fingers, lips and tongue, corneal lacerations, palmar hyperkeratosis, non-painful fracture and joint deformities in the right ankle. Tearing, deep tendon reflexes and motor and sensory nerve action potentials were normal.
Prenatal screening is the sole accessible option to prevent the birth of an affected child as no cure is available. Early recognition of CIPA patients and its orthopedic complications, prevention of accidental injuries, regular visual and eye follow-up and specific dental management could be useful in the reduction of frequency and severity of complications.
PMCID: PMC3943025
HSAN type IV; Congenital insensitivity to pain; Anhidrosis
12.  Intracranial Blood Flow Velocity in Patients with β-Thalassemia Intermedia Using Transcranial Doppler Sonography: A Case-Control Study 
Anemia  2011;2012:798296.
Introduction. Patients with β-thalassemia intermedia have a higher incidence of thromboembolic events compared to the general population. Previous studies have shown that patients with sickle cell disease, who are also prone to ischemic events, have higher intracranial arterial blood flow velocities measured by transcranial Doppler sonography (TCD). The aim of this study is to evaluate intracranial arterial flow velocities in patients with β-thalassemia intermedia and compare the results with those found in healthy subjects. Methods. Sixty-four patients with β-thalassemia intermedia and 30 healthy subjects underwent transcranial Doppler sonography. Results. Significantly higher flow velocities were found in intracranial arteries of patients compared to controls (P = 0.001). Previously splenectomized patients with thrombocytosis showed higher flow velocities than nonsplenectomized patients without thrombosis. Conclusion. The increased flow velocities in patients with β-thalassemia intermedia may point to a higher risk of ischemic events. Preventive measures such as blood transfusion or antiplatelet treatment may be beneficial in these patients.
doi:10.1155/2012/798296
PMCID: PMC3254003  PMID: 22263107
13.  Initial presentation of childhood leukaemia with facial palsy: three case reports 
BMJ Case Reports  2009;2009:bcr10.2008.1046.
Acute lymphoblastic leukaemia and acute myeloblastic leukaemia are the most common malignancies diagnosed in children. Facial palsy is an acute peripheral palsy involving the facial nerve and is an unusual presentation of childhood acute leukaemia. We present three cases (a 9-year-old boy, a 14-year-old boy and a 10-year-old boy) of acute leukaemia with initial presentation of facial palsy. It is important for physicians to recognise the neurological manifestations of childhood leukaemia and extensive work-up should be carried out to exclude secondary causes of facial palsy.
doi:10.1136/bcr.10.2008.1046
PMCID: PMC3030303  PMID: 22162740
14.  Major Causes of Hospital Admission in Beta Thalassemia Major Patients in Southern Iran 
Iranian Journal of Pediatrics  2011;21(4):509-513.
Objective
Beta thalassemia major is a prevalent hereditary disease in Mediterranean region especially Iran. Early blood transfusion is necessary for most of the patients and frequent transfusion can cause various medical problems for the patients. The aim of this study was to find major causes of hospital admission in beta thalassemia major patients to reach the accurate preventive and therapeutic plans for these patients.
Methods
Four hundred twenty six patients were admitted to the Nemazee Hospital (the main University referral Hospital Center affiliated to Shiraz University of Medical Sciences in Fars Province, southern Iran) during 3 years period (January 2007 to January 2010). A questionnaire was filled containing age, gender, hemoglobin level, frequency of blood transfusions, deferoxamine injection, cause of hospital admission and hospital course.
Findings
The mean age of patients was 11.28 years. The mean serum ferritin level was 1820±749 µg/lit. Two hundred fifty five (59.75%) patients were male and 171 (40.25%) patients were female. The top five most prevalent causes of hospital admission were splenectomy (21.8%), infections (19.9%), congestive heart failure (19.0%), diabetes mellitus (13.4%), and Liver biopsy (11.5%). (P=0.0002)
Conclusion
Results of this study revealed that infections and complications due to iron overload are major causes of hospital admission in beta thalassemia major patients.
PMCID: PMC3446139  PMID: 23056840
Beta Thalassemia major; Hospitalization; Complication; Iran
15.  Cost-utility analysis of immune tolerance induction therapy versus on-demand treatment with recombinant factor VII for hemophilia A with high titer inhibitors in Iran 
Background
In developing countries, the treatment of hemophilia patients with inhibitors is presently the most challenging and serious issue in hemophilia management, direct costs of clotting factor concentrates accounting for >98% of the highest economic burden absorbed for the health care of patients in this setting. In the setting of chronic diseases, cost-utility analysis, which takes into account the beneficial effects of a given treatment/health care intervention in terms of health-related quality of life, is likely to be the most appropriate approach.
Objective
The aim of this study was to assess the incremental cost-effectiveness ratios of immune tolerance induction (ITI) therapy with plasma-derived factor VIII concentrates versus on-demand treatment with recombinant-activated FVIIa (rFVIIa) in hemophilia A with high titer inhibitors from an Iranian Ministry of Health perspective.
Methods
This study was based on the study of Knight et al, which evaluated the cost- effectiveness ratios of different treatments for hemophilia A with high-responding inhibitors. To adapt Knight et al’s results to the Iranian context, a few clinical parameters were varied, and cost data were replaced with the corresponding Iranian estimates of resource use. The time horizon of the analysis was 10 years. One-way sensitivity analyses were performed, varying the cost of the clotting factor, the drug dose, and the administration frequency, to test the robustness of the analysis.
Results
Comparison of the incremental cost-effectiveness ratios between the three ITI protocols and the on-demand regimen with rFVIIa shows that all three ITI protocols dominate the on-demand regimen with rFVIIa. Between the ITI protocols the low-dose ITI protocol dominates both the Bonn ITI protocol and the Malmö ITI protocol and would be the preferred ITI protocol. All of the three ITI protocols dominate the on-demand strategy, as they have both a lower average lifetime cost and higher quality-adjusted life-years (QALYs) gained. The cost per QALY gained for the Bonn ITI protocol compared with the Malmö ITI protocol was $249,391.84. The cost per QALY gained for the Bonn ITI protocol compared with the low-dose ITI protocol was $842,307.69.
Conclusion
The results of data derived from our study suggest that the low-dose ITI protocol may be a less expensive and/or more cost-effective option compared with on-demand first-line treatment with rFVIIa.
doi:10.2147/CEOR.S25909
PMCID: PMC3234155  PMID: 22163168
cost-utility analysis; immune tolerance induction; on-demand; rFVIIa
16.  Non-Hodgkin Lymphoma in a Child with Schimke Immuno-Osseous Dysplasia 
Schimke immuno-osseous dysplasia is a rare autosomal recessive multisystem disorder characterized by steroid-resistant nephrotic syndrome, immunodeficiency, and spondyloepiphyseal dysplasia. Mutations in SWI/SNF2 related, matrix associated, actin dependent regulator of chromatin, subfamily a-like 1 (SMARCAL1) gene are responsible for the disease.
The present report describes, for the first time, a Schimke immuno-osseous dysplasia child with SMARCAL1 missense mutation (R561H) and manifestations of intussusception secondary to Epstein-Barr virus-negative non-Hodgkin lymphoma, who expired due to septicemia following chemotherapy. The report emphasizes the necessity of more limited immunosuppressive protocols in Schimke immuno-osseous dysplasia patients with lymphoproliferative disorders.
PMCID: PMC3556764  PMID: 23359635
Schimke immunoosseous dysplasia; lymphoproliferative; intussusception
17.  Efficacy of Carvedilol in Patients with Dilated Cardiomyopathy due to Beta-thalassemia major; a Double-blind Randomized Controlled Trial 
Iranian Journal of Pediatrics  2010;20(3):277-283.
Objective
Dilated cardiomyopathy is the end result of chronic iron overload in patients with beta thalassemia major. The objective of the present study was to evaluate the safety and efficacy of Carvedilol in patients with beta thalassemia major and dilated cardiomyopathy.
Methods
During a six-month period, fourteen patients with beta-thalassemia major and heart failure without diabetes mellitus referred to pediatric cardiology clinic enrolled in this double blind, randomly assigned study. All patients were on anti failure therapy with Digoxin, Captopril and Furosemide. Carvedilol was started at a dosage of 3.12 mg bid and for patients who had a systolic blood pressure >100 mmHg, heart rate >60/min and no signs of low cardiac output the dosage was increased every two weeks to a maximum of 25 mg bid. Clinical signs and symptoms, systolic and diastolic echocardiographic indexes and Tissue Doppler Imaging (TDI) data were collected from each patient.
Findings
Eight patients received Carvedilol (Group 1) and six received placebo (Group 2). The mean age of patients in Group1 and 2 were 16±0.7 years and 17±3 years respectively. Only one patent in Group 1 tolerated increasing Carvedilol dosage to more than 6.25 mg bid. Changes in New York Heart Association (NYHA) classification, Ejection fraction, End diastolic dimension changes, TDI systolic(S), early (Ea) and late (Aa) diastolic waves were not statistically significant in these two Groups (P>0.05). Pulse Doppler E/A wave ratio of mitral valve in Group1 and Group 2 changed from 1.1±0.37 m/s to 1.8±0.40 m/s and from 1.34±0.30 m/s to 2.6±0.23m/s respectively (P=0.04).
Conclusion
Patients with thalassemia and dilated cardiomyopathy have poor tolerance to increasing Carvedilol dosage and develop decreased systolic blood pressure during advancement of the drug dosage. Carvedilol can be effective in prevention of progression of diastolic dysfunction in these patients.
PMCID: PMC3446036  PMID: 23056717
Beta thalassemia major; Heart failure; Carvedilol; Dilated cardiomyopathy
18.  Frequency of Sensory Neural Hearing Loss in Major Beta-Thalassemias in Southern Iran 
Iranian Journal of Pediatrics  2010;20(3):308-312.
Objective
The thalassemias are among the most common genetic disorders worldwide, occurring more frequently in the Mediterranean region. The aim of this study was to determined frequency of sensory-neural hearing loss in major ß- thalassemias transfusion dependent patients in south of Iran.
Methods
A cross sectional study on 308 cases of major beta-thalassemia patients referring to Thalassemia Center of Shiraz University of Medical Sciences between 2006–2007 years. The diagnosis of ß- thalassemia major was based on clinical history, complete blood count and hemoglobine electrophoresis. Clinical data such as serum ferritin level, deferoxamine (DFO) dose, mean daily doses of DFO (mg/kg) and audiometric variables was recorded.
Findings
Out of 308 cases, 283 (96.5%) had normal hearing and 10 (3.5%) sensorineural hearing loss. There was no statically significant difference between two groups regarding mean age, weight, age at the first blood transfusion, age at the first DFO infusion.
Conclusion
We found the lowest incidence of sensorineural hearing loss in a large population of patients suffered from major thalassemia who received DFO. We show that DFO is not ototoxic at a low dose. When considering all related literature, as a whole there has been much critical misrepresentation about DFO ototoxicity.
PMCID: PMC3446050  PMID: 23056722
Thalassemia; Sensorineural Hearing Loss; Blood Transfusion; Deferoxamine; Ferritin
19.  Serum lipid profiles in patients with beta-thalassemia major and intermedia in southern Iran 
BACKGROUND:
Beta-thalassemia is considered to be the most frequent hereditary blood disorder worldwide. Lipid abnormalities have been detected in different types of beta-thalassemia. The aim of this study is to assess the lipid profiles in beta-thalassemia major (BTM) and beta-thalassemia intermedia (BTI) patients in southern Iran.
METHODS:
The study group consisted of 55 BTM patients and 50 BTI patients. The control group included 130 sex-and age-matched healthy participants. Serum lipids profiles (total cholesterol, triglycerides, LDL-cholesterol, and HDL-cholesterol) as well as hemoglobin (Hb) and ferritin, were compared between the three groups. P value < 0.05 was considered statistically significant.
RESULTS:
There were no significant differences between BTM and BTI patients regarding age or sex. Mean triglyceride concentration was not significantly different between patients and controls. Total cholesterol and LDL-cholesterol were significantly lower in patients with BTM and BTI in comparison with controls (p < 0.001). HDL-cholesterol was significantly lower in patients with BTI than in controls (p < 0.03).
CONCLUSIONS:
In patients with BTM and BTI, total cholesterol and LDL-cholesterol were lower than in control participants. The mechanisms that may account for these findings are increased erythropoiesis and cholesterol consumption in BTI, and iron overload and oxidative stress in BTM.
PMCID: PMC3082801  PMID: 21526074
Beta-Thalassemia Major; Beta-Thalassemia Intermedia; Total Cholesterol; LDL-Cholesterol; HDL-Cholesterol; Triglycerides
20.  Haemophilia in the developing countries: the Iranian experience 
Introduction
Management of haemophilia and inherited bleeding disorders is a major challenge especially in developing countries, because of a shortage or absence of products, the cost and the infrastructural health problems. Development of local expertise which results in an improved outlook and reduction in mortality and morbidity in these countries can be helpful for advocators in other developing countries. However, very little information on demography and organizational models for haemophilia care in developing countries are available in the literature. Our aim is a comprehensive report of haemophilia status and its management in Iran.
Material and methods
The Management Center of Transplantation and Special Diseases (MCTSD) of the Ministry of Health of Iran decided to carry out a complete review and compilation of all of the published or available data about patients with haemophilia (PWH) in Iran: their health status, their management planning, organizations, treatment products, facilities and care problems during 2007.
Results
6496 patients with congenital bleeding disorders were registered. Most of them had haemophilia A and B and von Willebrand disease (vWD). However, rare bleeding disorders are seen more than expected. Inhibitor development is 14–28%. There are different data about virological status of PWH. Factor products and facilities are fairly available with more than 1.5 units per capita of inhabitant factor consumption.
Conclusions
A national formulary based on facilities of the country should be considered and followed by collaboration among the Ministry Of Health, universities and non-governmental organizations.
doi:10.5114/aoms.2010.13512
PMCID: PMC3278948  PMID: 22371725
Iran; haemophilia care; developing world
21.  Is red cell from an otherwise healthy G6PD-deficient donor efficient for transfusion to fauvism patients? 
Aim
Glucose-6-Phosphate dehydrogenase (G6PD) deficiency is the most common X-linked defect of enzyme pathways in human. The aim of this study was to inspect the efficacy of G6PD deficient red cell transfusion to G6PD patients, who were admitted for hemolysis after Broad bean ingestion, and to find out whether prolong management program and retransfusion are needed as severe hemolysis would take place.
Methods
This was a cross-sectional study, which was performed on patients admitted to hospital due to hemolytic anemia after broad bean ingestion in Jahrom and Shiraz medical school hospitals on February and March 2007. 2ml samples of transfusion blood bags, were sent to the laboratory in order to investigate the G6PD enzyme sufficiency. Hemoglobin level was checked six hours after blood transfusion. We compared the amount of hemoglobin level elevation after transfusion in patients received G6PD sufficient and G6PD deficient red cell using independent sample t-test.
Result
In 114 transfused bags, 14 bags (12.3%) were G6PD deficient. We divided patients into two groups. Group A received G6PD sufficient red cell and group B were transfused with G6PD deficient red cell. Hemoglobin level was significantly raised after transfusion of red cell in each group. The amount of hemoglobin elevation was more in group A in comparison to group B.
Conclusion
Based on our study results, proper hemoglobin level would be achieved using non G6PD deficient red cell for transfusion to G6PD deficient patients.
doi:10.1007/s12288-009-0005-7
PMCID: PMC3453486  PMID: 23100967
Glucose-6-Phosphate dehydrogenase enzyme; G6PD deficiency; Hemolysis; Blood transfusion
22.  Cancer-Associated Thrombosis 
Thrombosis is a common complication in patients with cancer and it is estimated that about 20% of patients with cancer experience venous thromboembolism (VTE). This complication is associated with high rate of morbidity and mortality and is sometimes the first manifestation of an occult cancer. The risk profiles and markers involved in cancerassociated thrombosis share similarities with inflammation-induced atherosclerosis and thrombosis. The type of cancer, chemotherapy, surgery, central venous catheters, pre-chemotherapy platelet and leukocyte count are associated with high risk of VTE in cancer patients. Landmark studies demonstrated that effective prophylaxis and treatment of VTE reduced morbidity and increased survival. Low-molecular-weight heparin (LMWH) is preferred as an effective and safe means for prophylaxis and treatment of VTE. It has largely replaced unfractionated heparin and vitamin K antagonists. The advantages of LMWH include increased survival and quality of life, decreased rate of VTE, low incidence of thrombocytopenia. New guidelines for prophylaxis and treatment are now available and prophylaxis is recommended in hospitalized cancer patients and patients undergoing major surgery. Treatment with LMWH should be considered as the first line of therapy for established VTE and to prevent recurrent thrombosis in patients with cancer.
doi:10.2174/1874192401004020078
PMCID: PMC2847209  PMID: 20360976
Cancer; Thrombosis; Low-molecular-weight heparin.
23.  Does Helicobacter pylori play a role in the pathogenesis of childhood chronic idiopathic thrombocytopenic purpura? 
Pediatric Reports  2009;1(1):e2.
Idiopathic thrombocytopenic purpura (ITP) is an acute self-limited bleeding disorder that can progress to chronic form in 10–15% of the cases. Helicobacter pylori (H. pylori) infection is a possible cause of chronic ITP. We studied 30 children with resistant chronic ITP for H. pylori infection based on the detection of H. pylori fecal antigen. This retrospective study was based on data obtained from medical records of 30 children aged between five and 17 years (median age at ITP diagnosis was ten years). A specially-designed data sheet was used to record information on age, sex, duration of disease, family history of bleeding disorders, previous treatments and median platelet count. In patients with H. pylori infection, antimicrobial treatment consisted of amoxicillin, metronidazol and omeprazol. Response was assessed every month for one year and defined as complete (platelet count >150×109/L) or partial (platelet count between 50 and 150×109/L). We detected H. pylori infection in 5 patients. In 4 of them increased platelet count was seen during one year of follow-up and in one patient the platelet count was acceptable during six months. Although the pathological mechanism of H. pylori-induced thrombocytopenia was unclear in our patient sample, the assessment of H. pylori infection and use of eradication therapy should be attempted in chronic and resistant ITP patients.
doi:10.4081/pr.2009.e2
PMCID: PMC3096032  PMID: 21589818
idiopathic thrombocytopenic purpura; Helicobacter pylori; platelet count.
24.  Termination of pregnancy due to Thalassemia major, Hemophilia, and Down's Syndrome: the views of Iranian physicians 
BMC Medical Ethics  2008;9:19.
Background
Genetic disorders due to kindred marriages are common medical conditions in Iran; however, the legal aspects of abortion remain controversial. This study was undertaken to determine physicians' opinions regarding the termination of pregnancy for three genetic diseases: thalassemia major, hemophilia, and Down's syndrome.
Methods
A questionnaire was administered to selected physicians by stratified random sampling to determine the following: age, gender, knowledge about prenatal diagnosis of diseases in high risk pregnancies, agreement with abortion, recommended gestational age for abortion, and, if opposed to abortion, the reason.
Results
Of 323 physicians, who participated in the study, 91.3(295), 40.6(131), and 78.6%(254) were in agreement and 8.7(28), 59.4(192), and 21.4%(69) were opposed to abortion for thalassemia major, hemophilia, and Down's syndrome, respectively. Among 289 physicians opposed to abortion in respect of each of all three conditions, the following reasons were cited: religion, 18; emotional, 10; quality of care, 23; hope to find a new treatment option in the future, 103; miscellaneous reasons, 6; and a combination of these reasons, 129. Among 680 physicians in agreement with abortion in relation to all of the diseases, 4.6%(31) were agreed with abortion in less than 12 weeks gestation, 79.2%(538) in less than 16 weeks gestation, 5.6%(38) in less than 20 weeks gestation, 2.2%(15) in less than 24 weeks gestation, and 8.4%(58) were agreed with beyond the 24 weeks of gestational age.
Conclusion
The majority of physicians were in agreement with abortion for thalassemia major and Down's syndrome because of the overall prognosis, but opposed to abortion for hemophilia.
doi:10.1186/1472-6939-9-19
PMCID: PMC2628659  PMID: 19105809

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