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Research paper thumbnail of The effect of combined therapy with deferoxamine and deferiprone on serum ferritin level of beta-thalassemic patients

Hematology, 2012

To determine the effect of combined therapy with deferoxamine and deferiprone on serum ferritin l... more To determine the effect of combined therapy with deferoxamine and deferiprone on serum ferritin level of beta-thalassemic patients. This controlled clinical trial was conducted on 26 major beta-thalassemic patients. Twelve patients in case group received deferiprone 75 mg/kg/day three times a day (TDS) orally with deferoxamine 30-50 mg/kg subcutaneously every other day. Fourteen patients in control group received deferoxamine 30-50 mg/kg/day subcutaneously for 6-12 hours/day and 5-6 days per week. Serum ferritin level was measured at the beginning and at the end of the third and the sixth months of study. Side effects of combined therapy were assessed. Data were analyzed by SPSS software v: 16.0 and t-Student and compared t-tests. The mean of serum ferritin level in case group significantly decreased from 7539.8 ± 3434.9 µg/l at the beginning of study to 4848.7 ± 2706.2 µg/l (P < 0.001) and to 4338.3 ± 2308.8 µg/l (P < 0.001) at the end of the third and the sixth months of study, respectively. The mean of serum ferritin level in control group insignificantly increased from 5668 ± 3613.8 to 6210.8 ± 3940.9 µg/l and to 5742 ± 3205.9 µg/l at the end of the third and the sixth months of study, respectively. The common side effects of combined therapy were nausea (8.3%) and arthropathy (8.3%). A mild transient neutropenia and liver enzymes elevation happened in four patients of case group. Combined therapy with deferoxamine and deferiprone significantly decreases serum ferritin level. It has little side effect and is suggested for major beta-thalassemic patients as a suitable therapy.

Research paper thumbnail of The effect of combined therapy with deferoxamine and deferiprone on serum ferritin level of beta-thalassemic patients

Hematology, 2012

To determine the effect of combined therapy with deferoxamine and deferiprone on serum ferritin l... more To determine the effect of combined therapy with deferoxamine and deferiprone on serum ferritin level of beta-thalassemic patients. This controlled clinical trial was conducted on 26 major beta-thalassemic patients. Twelve patients in case group received deferiprone 75 mg/kg/day three times a day (TDS) orally with deferoxamine 30-50 mg/kg subcutaneously every other day. Fourteen patients in control group received deferoxamine 30-50 mg/kg/day subcutaneously for 6-12 hours/day and 5-6 days per week. Serum ferritin level was measured at the beginning and at the end of the third and the sixth months of study. Side effects of combined therapy were assessed. Data were analyzed by SPSS software v: 16.0 and t-Student and compared t-tests. The mean of serum ferritin level in case group significantly decreased from 7539.8 ± 3434.9 µg/l at the beginning of study to 4848.7 ± 2706.2 µg/l (P < 0.001) and to 4338.3 ± 2308.8 µg/l (P < 0.001) at the end of the third and the sixth months of study, respectively. The mean of serum ferritin level in control group insignificantly increased from 5668 ± 3613.8 to 6210.8 ± 3940.9 µg/l and to 5742 ± 3205.9 µg/l at the end of the third and the sixth months of study, respectively. The common side effects of combined therapy were nausea (8.3%) and arthropathy (8.3%). A mild transient neutropenia and liver enzymes elevation happened in four patients of case group. Combined therapy with deferoxamine and deferiprone significantly decreases serum ferritin level. It has little side effect and is suggested for major beta-thalassemic patients as a suitable therapy.

Research paper thumbnail of Serum Ferritin in Thalassemic (Major) Patients with Subcutaneous Desferal Versus Subcutaneous and Intravenous Desferal Therapy

Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ... more Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ﻪﻘﺒﻃ تﺎﻘﻴﻘﺤﺗ ﺰﻛﺮﻣ E.mail: s_besharat_gp@yahoo.com ﻪﺴﻳﺎﻘﻣ روژﺎﻣ ﻲﻤﺳﻻﺎﺗ ﻪﺑ ﻼﺘﺒﻣ نارﺎﻤﻴﺑ رد مﺮﺳ ﻦﻴﺘﻳﺮﻓ ناﺰﻴﻣ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد ماﻮﺗ هﺪﻨﻨﻛ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد هﺪﻨﻨﻛ نﻮﺧ ﻖﻳرﺰﺗ مﺎﮕﻨﻫ رد يﺪﻳرو لاﺮﻔﺳد ...

Research paper thumbnail of Serum Ferritin in Thalassemic (Major) Patients with Subcutaneous Desferal Versus Subcutaneous and Intravenous Desferal Therapy

Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ... more Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ﻪﻘﺒﻃ تﺎﻘﻴﻘﺤﺗ ﺰﻛﺮﻣ E.mail: s_besharat_gp@yahoo.com ﻪﺴﻳﺎﻘﻣ روژﺎﻣ ﻲﻤﺳﻻﺎﺗ ﻪﺑ ﻼﺘﺒﻣ نارﺎﻤﻴﺑ رد مﺮﺳ ﻦﻴﺘﻳﺮﻓ ناﺰﻴﻣ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد ماﻮﺗ هﺪﻨﻨﻛ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد هﺪﻨﻨﻛ نﻮﺧ ﻖﻳرﺰﺗ مﺎﮕﻨﻫ رد يﺪﻳرو لاﺮﻔﺳد ...

Research paper thumbnail of Management of the Sponataneous Bleeding Episodes in Factor VII Deficiency. A Prospective Evaluation of the STER

Blood

3368 Introduction Patients with an inherited factor VII (FVII) deficiency may display a wide rang... more 3368 Introduction Patients with an inherited factor VII (FVII) deficiency may display a wide range of clinical phenotypes, from an asymptomatic condition to serious hemorrhagic episodes such as fatal central nervous system (CNS) or gastrointestinal (GI) bleeds (Mariani G et al. Thromb Haemost 2005; 93: 481–7). Symptomatic patients can be divided into two major categories: those with mild-to-moderate bleeding tendency and individuals with a severe bleeding tendency which may be more severe than that in hemophilia. The former group mainly experience mucosal bleeding, a clinical picture that mimics that of a platelet disorder and often does not call for treatment. In contrast, for the most severely affected patients, characterized by life- or limb-threatening hemorrhages, more aggressive Replacement Therapy (RT) as long or short-term prophylxis may be required. Aim Within the frame of the Seven Treatment Evaluation Registry we have prospectively collected large numbers of treatments fo...

Research paper thumbnail of Different treatment strategies for Haemophilia A with Low Inhibitor

... It is also one of the only studies (with the exception of Dundar S et al5) comparing the cost... more ... It is also one of the only studies (with the exception of Dundar S et al5) comparing the cost and effective-ness of rFVIIa vs. ... Authors gratefully acknowledge Patricia Tan Wai Ling and Yan Cai, Juliette Anne Plun and Dr. Elif Coskuncay Yener from NovoNordisk for their help in ...

Research paper thumbnail of TyG index and insulin resistance in beta-thalassemia

International Journal of Diabetes in Developing Countries, 2015

Insulin resistance (IR) underlies some glucose metabolism abnormalities in thalassemia major. Rec... more Insulin resistance (IR) underlies some glucose metabolism abnormalities in thalassemia major. Recently, triglyceride glucose index (TyG) has been proposed for evaluating insulin resistance as a simple, low cost, and accessible tool. In this study, the TyG index were studied for IR monitoring in beta-thalassemia major (βTM) patients. The participants were 90 βTM patients on chronic regular transfusion therapy. The TyG index was computed based on fasting plasma glucose (FPG) and triglyceride (TG). The time gap between the first and the second TyG index survey (TyG.1 and TyG.2) was 2 years. The agreement between TyG and HOMA-IR were studied with the extension of limit of agreement (LOA). We included 90 patients 53.3 % men (n=48). Among them, 14.4 % (14.6 % male, 14.3 % female) had impaired fasting glucose level (e.g., 100-125 mg/dl) at first test. It rose to 37.8 % (27.1 % male, 50 % female) during 2 years. Based on TyG.1, the 34.4 % of patients was detected as IR cases. After 2 years, the percent of IR based on TyG.2 was 82.2 %. The mean differences between TyG.1 and TyG.2 and their differences from the considered cutoff values were significant (P<0.001). The prediction limits between TyG and HOMA-IR had good agreement. These data may suggest the use of TyG index for detection/monitoring of IR in βTM patients.

Research paper thumbnail of Effect of early iron supplementation on iron deficiency anemia in preterm infants

Background & Objective: Preterm infants have less iron storage compared with the term one. Due to... more Background & Objective: Preterm infants have less iron storage compared with the term one. Due to rapid growth they need more iron during infancy. This study was designed to evaluate the effect of early iron supplementation on hematologic indices and incidence of iron deficiency anemia in preterm infants. Materials & Methods: This experimental study was done on 20-days-old preterm breast-feed infants referred to the Gorgan-North of Iran primary health care services for vaccination, without any underline disease or growth retardation during 2005. They were divided to two groups (n=15 in each) and a questionnaire was completed for each case. Iron drop (2mg/kg) was given in the interventional group. Iron deficiency anemia was assesed in all sample T-student test and chi-square were used to analyze the independent variables and comparing the hematologic indices, after entering in SPSS-13 software and testing the normal distribution with komologrof-smearnoff test. Relative risk index (RR...

Research paper thumbnail of Gaucher disease: A 10 year old girl with anemia and huge spelenomegaly (a case report)

Pakistan journal of biological sciences : PJBS, 2008

Gaucher's disease is a rare lipid storage disorder, affecting one in 40,000-200,000 people an... more Gaucher's disease is a rare lipid storage disorder, affecting one in 40,000-200,000 people and results from a genetic deficiency of the enzyme glucocerebrosidase (glucosylceramidase). We report a 10-year old Iranian girl with chief complaint of anemia from 8 years ago, managed for iron deficiency anemia. The patient had hepatomegaly associated with huge splenomegaly which was confirmed by sonography. No skeletal disorder was found. Bone marrow aspiration revealed typical Gaucher cells. Low level of beta-glucocerebrosidase enzyme activity confirmed the Gaucher disease. The patient is now under treatment with CEREZIME, a recombinant DNA modified form of glucocerebrosidase with good condition.

Research paper thumbnail of Serum Ferritin in Thalassemic (Major) Patients with Subcutaneous Desferal Versus Subcutaneous and Intravenous Desferal Therapy

Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ... more Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ﻪﻘﺒﻃ تﺎﻘﻴﻘﺤﺗ ﺰﻛﺮﻣ E.mail: s_besharat_gp@yahoo.com ﻪﺴﻳﺎﻘﻣ روژﺎﻣ ﻲﻤﺳﻻﺎﺗ ﻪﺑ ﻼﺘﺒﻣ نارﺎﻤﻴﺑ رد مﺮﺳ ﻦﻴﺘﻳﺮﻓ ناﺰﻴﻣ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد ماﻮﺗ هﺪﻨﻨﻛ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد هﺪﻨﻨﻛ نﻮﺧ ﻖﻳرﺰﺗ مﺎﮕﻨﻫ رد يﺪﻳرو لاﺮﻔﺳد ...

Research paper thumbnail of Serum Ferritin in Thalassemic (Major) Patients with Subcutaneous Desferal Versus Subcutaneous and Intravenous Desferal Therapy

Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ... more Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ﻪﻘﺒﻃ تﺎﻘﻴﻘﺤﺗ ﺰﻛﺮﻣ E.mail: s_besharat_gp@yahoo.com ﻪﺴﻳﺎﻘﻣ روژﺎﻣ ﻲﻤﺳﻻﺎﺗ ﻪﺑ ﻼﺘﺒﻣ نارﺎﻤﻴﺑ رد مﺮﺳ ﻦﻴﺘﻳﺮﻓ ناﺰﻴﻣ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد ماﻮﺗ هﺪﻨﻨﻛ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد هﺪﻨﻨﻛ نﻮﺧ ﻖﻳرﺰﺗ مﺎﮕﻨﻫ رد يﺪﻳرو لاﺮﻔﺳد ...

Research paper thumbnail of Serum Ferritin in Thalassemic (Major) Patients with Subcutaneous Desferal Versus Subcutaneous and Intravenous Desferal Therapy

Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ... more Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ﻪﻘﺒﻃ تﺎﻘﻴﻘﺤﺗ ﺰﻛﺮﻣ E.mail: s_besharat_gp@yahoo.com ﻪﺴﻳﺎﻘﻣ روژﺎﻣ ﻲﻤﺳﻻﺎﺗ ﻪﺑ ﻼﺘﺒﻣ نارﺎﻤﻴﺑ رد مﺮﺳ ﻦﻴﺘﻳﺮﻓ ناﺰﻴﻣ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد ماﻮﺗ هﺪﻨﻨﻛ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد هﺪﻨﻨﻛ نﻮﺧ ﻖﻳرﺰﺗ مﺎﮕﻨﻫ رد يﺪﻳرو لاﺮﻔﺳد ...

Research paper thumbnail of With Congenital Factor VII Deficiency A Comparison Between Recombinant Activated Factor VII (Aryoseven) and Novoseven in Patients

In order to establish the efficacy and biosimilar nature of AryoSeven to NovoSeven in the treatme... more In order to establish the efficacy and biosimilar nature of AryoSeven to NovoSeven in the treatment of congenital factor VII (FVII) deficiency, patients received either agent at 30 mg/kg, intravenously per week for 4 weeks, in a randomized fashion. The primary aim was to compare FVII:coagulation activity (FVII:C), 20 minutes after recombinant activated FVII (rFVIIa) injection, in the 2 groups. A secondary measure was self-reported bleeding. The median interquartile baseline range of the plasma level of activated FVII (FVIIa) activity in the 2 groups was 1.6 (1.1-14.0) IU/dL and 5.0 (1.1-25.5) IU/dL. All patients achieved levels of FVIIa (FVII:C) >30 IU/dL, 20 minutes after the injection of rFVIIa. Bleeding was similar between the 2 groups, with a comparable decrease in severity and frequency compared to the last month prior to treatment. AryoSeven is similar to NovoSeven in increasing postinjection FVIIa activity as well as in clinical safety and efficacy.

Research paper thumbnail of National Approach to Premarital Diagnosis of Trait Thalassemia and Silent Carriers

Background: Major β-thalassemia occurs when impaired genes received by a neonate from the parents... more Background: Major β-thalassemia occurs when impaired genes received by a neonate from the parents. In most of the parents, there are no apparent clinical manifestation and they just show some impairments in hematological indices. This study was designed to determine prevalence of minor and silent carries parents that have children suffering from major β-thalassemia, compare it with national protocol about prevention of thalassemia. Methods: A blood sample was taken from parents of all major thalassemic patients covered by Taleghani Hospital in Gorgan (n = 195), CBC and He-moglobin electrophoresis were done. Data were analyzed using SPSS software. Results: Amongst 196 parents one case have normal level of MCV, MCH, RBC, HbA, HbF and mentzer = 22.05 (0.51%) that diagnosed as alpha triplication/N by real time PCR, RFLP informative. The means of hematological indices were based on the national protocol. Conclusion: Present results showed that there are a few cases of thalassemia disorders with normal MCV, MCH, RBC, Mentzer index and Hb electrophoresis which could be missed in routine and premarital screening tests, resulted in a thalassemia child that it is possible in every screening test. Generally, indices were according to range of the national guideline.

Research paper thumbnail of National Approach to Premarital Diagnosis of Trait Thalassemia and Silent Carriers

International Journal of Clinical Medicine, 2013

Major β-thalassemia occurs when impaired genes received by a neonate from the parents. In most of... more Major β-thalassemia occurs when impaired genes received by a neonate from the parents. In most of the parents, there are no apparent clinical manifestation and they just show some impairments in hematological indices. This study was designed to determine prevalence of minor and silent carries parents that have children suffering from major β-thalassemia, compare it with national protocol about prevention of thalassemia. Methods: A blood sample was taken from parents of all major thalassemic patients covered by Taleghani Hospital in Gorgan (n = 195), CBC and Hemoglobin electrophoresis were done. Data were analyzed using SPSS software. Results: Amongst 196 parents one case have normal level of MCV, MCH, RBC, HbA, HbF and mentzer = 22.05 (0.51%) that diagnosed as alpha triplication/N by real time PCR, RFLP informative. The means of hematological indices were based on the national protocol. Conclusion: Present results showed that there are a few cases of thalassemia disorders with normal MCV, MCH, RBC, Mentzer index and Hb electrophoresis which could be missed in routine and pre-marital screening tests, resulted in a thalassemia child that it is possible in every screening test. Generally, indices were according to range of the national guideline.

Research paper thumbnail of The effect of combined therapy with deferoxamine and deferiprone on serum ferritin level of beta-thalassemic patients

Hematology, 2012

To determine the effect of combined therapy with deferoxamine and deferiprone on serum ferritin l... more To determine the effect of combined therapy with deferoxamine and deferiprone on serum ferritin level of beta-thalassemic patients. This controlled clinical trial was conducted on 26 major beta-thalassemic patients. Twelve patients in case group received deferiprone 75 mg/kg/day three times a day (TDS) orally with deferoxamine 30-50 mg/kg subcutaneously every other day. Fourteen patients in control group received deferoxamine 30-50 mg/kg/day subcutaneously for 6-12 hours/day and 5-6 days per week. Serum ferritin level was measured at the beginning and at the end of the third and the sixth months of study. Side effects of combined therapy were assessed. Data were analyzed by SPSS software v: 16.0 and t-Student and compared t-tests. The mean of serum ferritin level in case group significantly decreased from 7539.8 ± 3434.9 µg/l at the beginning of study to 4848.7 ± 2706.2 µg/l (P &amp;amp;lt; 0.001) and to 4338.3 ± 2308.8 µg/l (P &amp;amp;lt; 0.001) at the end of the third and the sixth months of study, respectively. The mean of serum ferritin level in control group insignificantly increased from 5668 ± 3613.8 to 6210.8 ± 3940.9 µg/l and to 5742 ± 3205.9 µg/l at the end of the third and the sixth months of study, respectively. The common side effects of combined therapy were nausea (8.3%) and arthropathy (8.3%). A mild transient neutropenia and liver enzymes elevation happened in four patients of case group. Combined therapy with deferoxamine and deferiprone significantly decreases serum ferritin level. It has little side effect and is suggested for major beta-thalassemic patients as a suitable therapy.

Research paper thumbnail of The effect of combined therapy with deferoxamine and deferiprone on serum ferritin level of beta-thalassemic patients

Hematology, 2012

To determine the effect of combined therapy with deferoxamine and deferiprone on serum ferritin l... more To determine the effect of combined therapy with deferoxamine and deferiprone on serum ferritin level of beta-thalassemic patients. This controlled clinical trial was conducted on 26 major beta-thalassemic patients. Twelve patients in case group received deferiprone 75 mg/kg/day three times a day (TDS) orally with deferoxamine 30-50 mg/kg subcutaneously every other day. Fourteen patients in control group received deferoxamine 30-50 mg/kg/day subcutaneously for 6-12 hours/day and 5-6 days per week. Serum ferritin level was measured at the beginning and at the end of the third and the sixth months of study. Side effects of combined therapy were assessed. Data were analyzed by SPSS software v: 16.0 and t-Student and compared t-tests. The mean of serum ferritin level in case group significantly decreased from 7539.8 ± 3434.9 µg/l at the beginning of study to 4848.7 ± 2706.2 µg/l (P &amp;amp;lt; 0.001) and to 4338.3 ± 2308.8 µg/l (P &amp;amp;lt; 0.001) at the end of the third and the sixth months of study, respectively. The mean of serum ferritin level in control group insignificantly increased from 5668 ± 3613.8 to 6210.8 ± 3940.9 µg/l and to 5742 ± 3205.9 µg/l at the end of the third and the sixth months of study, respectively. The common side effects of combined therapy were nausea (8.3%) and arthropathy (8.3%). A mild transient neutropenia and liver enzymes elevation happened in four patients of case group. Combined therapy with deferoxamine and deferiprone significantly decreases serum ferritin level. It has little side effect and is suggested for major beta-thalassemic patients as a suitable therapy.

Research paper thumbnail of Serum Ferritin in Thalassemic (Major) Patients with Subcutaneous Desferal Versus Subcutaneous and Intravenous Desferal Therapy

Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ... more Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ﻪﻘﺒﻃ تﺎﻘﻴﻘﺤﺗ ﺰﻛﺮﻣ E.mail: s_besharat_gp@yahoo.com ﻪﺴﻳﺎﻘﻣ روژﺎﻣ ﻲﻤﺳﻻﺎﺗ ﻪﺑ ﻼﺘﺒﻣ نارﺎﻤﻴﺑ رد مﺮﺳ ﻦﻴﺘﻳﺮﻓ ناﺰﻴﻣ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد ماﻮﺗ هﺪﻨﻨﻛ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد هﺪﻨﻨﻛ نﻮﺧ ﻖﻳرﺰﺗ مﺎﮕﻨﻫ رد يﺪﻳرو لاﺮﻔﺳد ...

Research paper thumbnail of Serum Ferritin in Thalassemic (Major) Patients with Subcutaneous Desferal Versus Subcutaneous and Intravenous Desferal Therapy

Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ... more Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ﻪﻘﺒﻃ تﺎﻘﻴﻘﺤﺗ ﺰﻛﺮﻣ E.mail: s_besharat_gp@yahoo.com ﻪﺴﻳﺎﻘﻣ روژﺎﻣ ﻲﻤﺳﻻﺎﺗ ﻪﺑ ﻼﺘﺒﻣ نارﺎﻤﻴﺑ رد مﺮﺳ ﻦﻴﺘﻳﺮﻓ ناﺰﻴﻣ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد ماﻮﺗ هﺪﻨﻨﻛ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد هﺪﻨﻨﻛ نﻮﺧ ﻖﻳرﺰﺗ مﺎﮕﻨﻫ رد يﺪﻳرو لاﺮﻔﺳد ...

Research paper thumbnail of Management of the Sponataneous Bleeding Episodes in Factor VII Deficiency. A Prospective Evaluation of the STER

Blood

3368 Introduction Patients with an inherited factor VII (FVII) deficiency may display a wide rang... more 3368 Introduction Patients with an inherited factor VII (FVII) deficiency may display a wide range of clinical phenotypes, from an asymptomatic condition to serious hemorrhagic episodes such as fatal central nervous system (CNS) or gastrointestinal (GI) bleeds (Mariani G et al. Thromb Haemost 2005; 93: 481–7). Symptomatic patients can be divided into two major categories: those with mild-to-moderate bleeding tendency and individuals with a severe bleeding tendency which may be more severe than that in hemophilia. The former group mainly experience mucosal bleeding, a clinical picture that mimics that of a platelet disorder and often does not call for treatment. In contrast, for the most severely affected patients, characterized by life- or limb-threatening hemorrhages, more aggressive Replacement Therapy (RT) as long or short-term prophylxis may be required. Aim Within the frame of the Seven Treatment Evaluation Registry we have prospectively collected large numbers of treatments fo...

Research paper thumbnail of Different treatment strategies for Haemophilia A with Low Inhibitor

... It is also one of the only studies (with the exception of Dundar S et al5) comparing the cost... more ... It is also one of the only studies (with the exception of Dundar S et al5) comparing the cost and effective-ness of rFVIIa vs. ... Authors gratefully acknowledge Patricia Tan Wai Ling and Yan Cai, Juliette Anne Plun and Dr. Elif Coskuncay Yener from NovoNordisk for their help in ...

Research paper thumbnail of TyG index and insulin resistance in beta-thalassemia

International Journal of Diabetes in Developing Countries, 2015

Insulin resistance (IR) underlies some glucose metabolism abnormalities in thalassemia major. Rec... more Insulin resistance (IR) underlies some glucose metabolism abnormalities in thalassemia major. Recently, triglyceride glucose index (TyG) has been proposed for evaluating insulin resistance as a simple, low cost, and accessible tool. In this study, the TyG index were studied for IR monitoring in beta-thalassemia major (βTM) patients. The participants were 90 βTM patients on chronic regular transfusion therapy. The TyG index was computed based on fasting plasma glucose (FPG) and triglyceride (TG). The time gap between the first and the second TyG index survey (TyG.1 and TyG.2) was 2 years. The agreement between TyG and HOMA-IR were studied with the extension of limit of agreement (LOA). We included 90 patients 53.3 % men (n=48). Among them, 14.4 % (14.6 % male, 14.3 % female) had impaired fasting glucose level (e.g., 100-125 mg/dl) at first test. It rose to 37.8 % (27.1 % male, 50 % female) during 2 years. Based on TyG.1, the 34.4 % of patients was detected as IR cases. After 2 years, the percent of IR based on TyG.2 was 82.2 %. The mean differences between TyG.1 and TyG.2 and their differences from the considered cutoff values were significant (P<0.001). The prediction limits between TyG and HOMA-IR had good agreement. These data may suggest the use of TyG index for detection/monitoring of IR in βTM patients.

Research paper thumbnail of Effect of early iron supplementation on iron deficiency anemia in preterm infants

Background & Objective: Preterm infants have less iron storage compared with the term one. Due to... more Background & Objective: Preterm infants have less iron storage compared with the term one. Due to rapid growth they need more iron during infancy. This study was designed to evaluate the effect of early iron supplementation on hematologic indices and incidence of iron deficiency anemia in preterm infants. Materials & Methods: This experimental study was done on 20-days-old preterm breast-feed infants referred to the Gorgan-North of Iran primary health care services for vaccination, without any underline disease or growth retardation during 2005. They were divided to two groups (n=15 in each) and a questionnaire was completed for each case. Iron drop (2mg/kg) was given in the interventional group. Iron deficiency anemia was assesed in all sample T-student test and chi-square were used to analyze the independent variables and comparing the hematologic indices, after entering in SPSS-13 software and testing the normal distribution with komologrof-smearnoff test. Relative risk index (RR...

Research paper thumbnail of Gaucher disease: A 10 year old girl with anemia and huge spelenomegaly (a case report)

Pakistan journal of biological sciences : PJBS, 2008

Gaucher's disease is a rare lipid storage disorder, affecting one in 40,000-200,000 people an... more Gaucher's disease is a rare lipid storage disorder, affecting one in 40,000-200,000 people and results from a genetic deficiency of the enzyme glucocerebrosidase (glucosylceramidase). We report a 10-year old Iranian girl with chief complaint of anemia from 8 years ago, managed for iron deficiency anemia. The patient had hepatomegaly associated with huge splenomegaly which was confirmed by sonography. No skeletal disorder was found. Bone marrow aspiration revealed typical Gaucher cells. Low level of beta-glucocerebrosidase enzyme activity confirmed the Gaucher disease. The patient is now under treatment with CEREZIME, a recombinant DNA modified form of glucocerebrosidase with good condition.

Research paper thumbnail of Serum Ferritin in Thalassemic (Major) Patients with Subcutaneous Desferal Versus Subcutaneous and Intravenous Desferal Therapy

Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ... more Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ﻪﻘﺒﻃ تﺎﻘﻴﻘﺤﺗ ﺰﻛﺮﻣ E.mail: s_besharat_gp@yahoo.com ﻪﺴﻳﺎﻘﻣ روژﺎﻣ ﻲﻤﺳﻻﺎﺗ ﻪﺑ ﻼﺘﺒﻣ نارﺎﻤﻴﺑ رد مﺮﺳ ﻦﻴﺘﻳﺮﻓ ناﺰﻴﻣ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد ماﻮﺗ هﺪﻨﻨﻛ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد هﺪﻨﻨﻛ نﻮﺧ ﻖﻳرﺰﺗ مﺎﮕﻨﻫ رد يﺪﻳرو لاﺮﻔﺳد ...

Research paper thumbnail of Serum Ferritin in Thalassemic (Major) Patients with Subcutaneous Desferal Versus Subcutaneous and Intravenous Desferal Therapy

Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ... more Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ﻪﻘﺒﻃ تﺎﻘﻴﻘﺤﺗ ﺰﻛﺮﻣ E.mail: s_besharat_gp@yahoo.com ﻪﺴﻳﺎﻘﻣ روژﺎﻣ ﻲﻤﺳﻻﺎﺗ ﻪﺑ ﻼﺘﺒﻣ نارﺎﻤﻴﺑ رد مﺮﺳ ﻦﻴﺘﻳﺮﻓ ناﺰﻴﻣ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد ماﻮﺗ هﺪﻨﻨﻛ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد هﺪﻨﻨﻛ نﻮﺧ ﻖﻳرﺰﺗ مﺎﮕﻨﻫ رد يﺪﻳرو لاﺮﻔﺳد ...

Research paper thumbnail of Serum Ferritin in Thalassemic (Major) Patients with Subcutaneous Desferal Versus Subcutaneous and Intravenous Desferal Therapy

Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ... more Page 1. * ﻟﺎﻘﻣ لﻮﺌﺴﻣ ؛ﻪ سردآ : ،نﺎﮔﺮﮔ ،رذآ ﻢﺠﻨﭘ راﻮﻠﺑ ،مرﺎﻬﭼ رذآ ﻪﭼﻮﻛ ،يﻮﺒﻧ ﺪﻴﻬﺷ ﻚﻴﻨﻴﻠﻛ ﻲﻠﭘ ،مود ﻪﻘﺒﻃ تﺎﻘﻴﻘﺤﺗ ﺰﻛﺮﻣ E.mail: s_besharat_gp@yahoo.com ﻪﺴﻳﺎﻘﻣ روژﺎﻣ ﻲﻤﺳﻻﺎﺗ ﻪﺑ ﻼﺘﺒﻣ نارﺎﻤﻴﺑ رد مﺮﺳ ﻦﻴﺘﻳﺮﻓ ناﺰﻴﻣ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد ماﻮﺗ هﺪﻨﻨﻛ ﺖﻓﺎﻳرد و يﺪﻠﺟﺮﻳز لاﺮﻔﺳد هﺪﻨﻨﻛ نﻮﺧ ﻖﻳرﺰﺗ مﺎﮕﻨﻫ رد يﺪﻳرو لاﺮﻔﺳد ...

Research paper thumbnail of With Congenital Factor VII Deficiency A Comparison Between Recombinant Activated Factor VII (Aryoseven) and Novoseven in Patients

In order to establish the efficacy and biosimilar nature of AryoSeven to NovoSeven in the treatme... more In order to establish the efficacy and biosimilar nature of AryoSeven to NovoSeven in the treatment of congenital factor VII (FVII) deficiency, patients received either agent at 30 mg/kg, intravenously per week for 4 weeks, in a randomized fashion. The primary aim was to compare FVII:coagulation activity (FVII:C), 20 minutes after recombinant activated FVII (rFVIIa) injection, in the 2 groups. A secondary measure was self-reported bleeding. The median interquartile baseline range of the plasma level of activated FVII (FVIIa) activity in the 2 groups was 1.6 (1.1-14.0) IU/dL and 5.0 (1.1-25.5) IU/dL. All patients achieved levels of FVIIa (FVII:C) >30 IU/dL, 20 minutes after the injection of rFVIIa. Bleeding was similar between the 2 groups, with a comparable decrease in severity and frequency compared to the last month prior to treatment. AryoSeven is similar to NovoSeven in increasing postinjection FVIIa activity as well as in clinical safety and efficacy.

Research paper thumbnail of National Approach to Premarital Diagnosis of Trait Thalassemia and Silent Carriers

Background: Major β-thalassemia occurs when impaired genes received by a neonate from the parents... more Background: Major β-thalassemia occurs when impaired genes received by a neonate from the parents. In most of the parents, there are no apparent clinical manifestation and they just show some impairments in hematological indices. This study was designed to determine prevalence of minor and silent carries parents that have children suffering from major β-thalassemia, compare it with national protocol about prevention of thalassemia. Methods: A blood sample was taken from parents of all major thalassemic patients covered by Taleghani Hospital in Gorgan (n = 195), CBC and He-moglobin electrophoresis were done. Data were analyzed using SPSS software. Results: Amongst 196 parents one case have normal level of MCV, MCH, RBC, HbA, HbF and mentzer = 22.05 (0.51%) that diagnosed as alpha triplication/N by real time PCR, RFLP informative. The means of hematological indices were based on the national protocol. Conclusion: Present results showed that there are a few cases of thalassemia disorders with normal MCV, MCH, RBC, Mentzer index and Hb electrophoresis which could be missed in routine and premarital screening tests, resulted in a thalassemia child that it is possible in every screening test. Generally, indices were according to range of the national guideline.

Research paper thumbnail of National Approach to Premarital Diagnosis of Trait Thalassemia and Silent Carriers

International Journal of Clinical Medicine, 2013

Major β-thalassemia occurs when impaired genes received by a neonate from the parents. In most of... more Major β-thalassemia occurs when impaired genes received by a neonate from the parents. In most of the parents, there are no apparent clinical manifestation and they just show some impairments in hematological indices. This study was designed to determine prevalence of minor and silent carries parents that have children suffering from major β-thalassemia, compare it with national protocol about prevention of thalassemia. Methods: A blood sample was taken from parents of all major thalassemic patients covered by Taleghani Hospital in Gorgan (n = 195), CBC and Hemoglobin electrophoresis were done. Data were analyzed using SPSS software. Results: Amongst 196 parents one case have normal level of MCV, MCH, RBC, HbA, HbF and mentzer = 22.05 (0.51%) that diagnosed as alpha triplication/N by real time PCR, RFLP informative. The means of hematological indices were based on the national protocol. Conclusion: Present results showed that there are a few cases of thalassemia disorders with normal MCV, MCH, RBC, Mentzer index and Hb electrophoresis which could be missed in routine and pre-marital screening tests, resulted in a thalassemia child that it is possible in every screening test. Generally, indices were according to range of the national guideline.