مهرنوش کوثریان

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

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

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

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

Iron deficiency anemia lead to a decrease in intelegence question (IQ) and a decrease in learning and mental and physical abilities. This study was done to determine the status of iron intake in infants aged 6 to 24 months under the cover of comprehensive health centers in Miandorood city, Mazandaran province, Iran during 2019.

This descriptive-analytical study was carried out on 400 infants aged 6-24 months who were enrolled in comprehensive health centers in Miandorood city, Mazandaran province, Iran during 2019. The status of iron drop consumption and mothers' awareness in this field were evaluated.

The mean age of the infants was 13.8±4.8 months. The range of the mother’s age was 28.8±5.6 years. Of these, 274 (68.5%) of infants used iron drops regularly, 106 case (26.5%) often, and 20 case (5.0%) never. The mother's knowledge score was significantly correlated with the type of iron intake, height percentile, and maternal education level (P<0.05). Evaluation of the possible causes of irregular iron intake shows that the most frequent cases were forgetting (28.8%), 9.5% for nausea and vomiting, and ‎‎9.0% for teeth color changing.‎

Mothers' awareness of iron drops consumption was good. The most important barriers to regular iron intake in the present study were forgetfulness, vomiting, and tooth discoloration.

Prenatal diagnose (PND) in carrier β-Thalassemia couples is very important. PND of Thalassemia, is a part of national prevention program. In all over the world, conventional prenatal diagnosis of Thalassemia is CVS or AC. These methods are invasive, we want to experience the new NIPD for β-Thalassemia with this hypothesis that 50% of invasive PND removed.Non invasive prenatal diagnose of Thalassemia is possible via detection of paternally mutation in maternal plasma. The history of fetal DNA (fDNA) in maternal circulation dates back to the 1948 by Mandel and Metais. The discovery of fDNA in maternal plasma and serum has opened up new opportunities in non invasive prenatal diagnosis (NIPD). This method has been shown to be potentially useful for prenatal diagnosis of certain neurological disorders, fetal sex determination, sex – linked disease, RhD genotyping, and aneuploidies. Fetal DNA originated from trophoblast cells in the placenta, and it detectable very soon during pregnancy, it only comprised about 3 to 6% (although up to 10% has been reported). Fetal DNA fragments are small in size (< 313 bp). It present during pregnancy until birth. They are rapidly cleared within 2 hours after delivery.Taq- Man is one of the first methods introduced for Real-time sequence detection. A Taq-Man reaction involves 2 specific primers and probes. Each probe is complimentary to one of the alleles of a mutation and each of them is labeled with a different fluorophore. This method exploits the 5chr('39') endonucleolytic activity of Taq DNA polymerase to slice an oligonucleotide probe through PCR amplification thus generating a detectable signal.The aim of this study was non invasive prenatal diagnosis of β-Thalassemia and determination the sensitivity and specificity of this technique for detection paternally inherited IVS II-I (GA) mutation in fetus.

Subjects were selected following ethical approval of study protocol from the ethical committee of Mazandaran University of Medical Science, Iran. The sample size of this study calculated according the related studies . Fifty Iranian pregnant women enrolled in this study, they were at risk of having fetuses with β-Thalassemia major. They attended in prenatal diagnosis of Thalassemia center (at 11-14 weeks of pregnancy, before performance of CVS), between May 2014 and Feb 2015, in Sari, Iran. All women assigned informed consent form. We focused exclusively on couples in which the father was carrier for IVS II-I GàA mutation and the mother had been genotyped to carry another β-globin gene mutation, (Of course, the mutation in each subject had been determined previously by conventional PCR and ARMS method, and followed by agarose gel electrophoresis). Also, 10 milliliters of maternal blood from each pregnant woman were collected into EDTA-containing tube. Maternal plasma was separated from cells by high –speed centrifugation (1900 g or 3000 rpm), and it stored at – 80 0 c until analysis. We were masked as to the identity the samples with numerical coding system, so, these were examined in a blinded manner. We designed two specific primers for IVSII-1(GàA) mutation (Accession No.141900) (www.hgvs.org), on the HBB DNA sequences (NCBI Reference Sequence: NG_000007.3 3 (Homo sapiens beta globin region (HBB)), the oligonucleotid primers of this study were; forward primer: 5chr('39')TCACCTGGACAACCTCAAG3chr('39'), and reverse primer: 5chr('39')CCCATTCTAAACTGTACCC3chr('39'). The primers pair was optimized to particularly amplify a 200-bp segment of exon 2 of the human β-globin gene. Of course we consider the locus of maternal mutation in β-globin gene that doesnchr('39')t interfere to our PCR product. Also, for design the Taq-Man probes, we refer in Taq- Man design site (primer digital.com/fast PCR/m15.html FAST PCR manual). The characterics of probes were; GC content 45-65%, no dimer with primer, high Tm 60-65 oc, probe length 18-30 bp, and Tm of primer was 8-10 oc higher than Tm primer. Also, the kind of quencher and reporter were matched with kind of quencher and reporter in the kit. Probe for wild allele was; FAM-AGAACTTCAGGGTGAGTCTATGGG-BHQ1, and the Probe for mutant allele was; VIC-AGAACTTCAGGATGAGTCTATGGG-BHQ1. Circulatory DNA was extracted from 200 µl of peripheral blood plasma with QIAamp DNA mini and blood kit according to the manufacturerchr('39')s instructions. DNA was eluted in a final volume of 200 µl AE buffer and stored at -20 0 c until used for additional analyzing. Also, the genomic DNA concentration was measured by NanoDrope system and electrophoresis. For detection of paternally inherited mutation Taq-Man Real time PCR was used and the result of Taq- man method compared with result of chorionic vilus sampling method (CVS) as a gold standard.

The clinical performance of Taq-Man test was assayed by comparing with routine test. Included in this study were 50 pregnant mothers. In Taq-Man method 38 samples cited in scatter analysis graph as a negative control, because the concentration of DNA was very low and this method didn’t have enough sensitivity and specificity for detection, so, the final analysis for assessment of this method was done on 12 samples. We reported 5 cases true positive, 4 cases true negative, 2 cases false negative and 1 cases false positive. Also the sensitivity of Taq-Man was 71.43% with CI95% (60%-83.43%), and specificity 66.67% with CI 95% (54.67%-78.67%). LR+ and LR- were 2.15 and 0.5 respectively. Also NPV and PPN of Taq-Man test were 66.67%, 83.4% respectively.

Non invasive prenatal diagnose can be detect paternally inherited mutation of thalassemia.

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

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

Selection of iron chelator medication for thalassemic patients depends on clinical and laboratory characteristics such as age, serum ferritin and severity of different organ damage. Patient choice is also important because the compliance to treatment is essential. Policy makers should decide to import brands versus to facilitate making medicationsin the country. Insurance companies also should decide for coverage the costs. In this paper we report the current situation of iron chelator usage for patients with beta thalassemia major in our center.
We reviewed data the medical records of patients in November 2015.
There were 463 patients on iron chelator medications. Three hundred and thirty patients (71.2%) had regular blood transfusions and reminders were not on regular blood transfusion. Two hundred and fifty one (54%), and 212(46%) were using one or two iron chelators, respectively. Two hundred and two (44%) patients were using a combination of a parenteral and one oral medication. Sixty seven (14%) patients were using only parenteral iron chelator. The most common used medication was Osveral (made in Iran by Osveh Pharmaceutical) in about one third of patients. All non-transfusion dependent patients were using Osveral. The parenteral medication (Desferrioxamine) was distributed in two brands. One imported brand as Desferal (Novartis) and the other as Desfonak (made in Iran by Ronak Daru).Deferiprone(made in Avesina, Iran) was used in combination with desferrioxamine in 141 patients and as the only treatment in 11 patients.
Availability of different types of medications makes the opportunity to select the best protocol for patients.

Background and Exjade® is developed by Novartis pharmaceutical company and contains the active substance deferasirox, an orally active iron chelator for treatment of chronic iron overload following blood transfusions such as beta thalassemia. The aim of this study was to compare the physicochemical characteristics of branded generic product of deferasirox, Osveral®, produced by Osveh pharmaceutical company (in Iran) with the original brand.
Three doses (125, 250 and 500 mg) of deferasirox reference tablets (Exjade®) were compared with the same doses of Osveral®. Three batches of each product were randomly selected and physicochemical evaluations including appearance, disintegration time, hardness, assay, in vitro drug release and content uniformity were determined. Also, the similarity factor was calculated based on ICH (International Conference Harmonization) and Food and Drug Administration (FDA) guidelines. The drug entrapped in the tablets was determined by HPLC. The dissolution rates were measured in solution media (phosphate buffered and sodium Lauryl sulfate at 37 ºC) within 30 min by USP apparatus II (Paddle) at 50 RPM speed after 5, 10, 20 and 30 min intervals.
The results indicated that physicochemical properties of Osveral® were similar to those of Exjade®. Disintegration time for both products was less than 1 min and drug assay was between 90 – 110%. In addition, the similarity factor was more than 50% for all doses.
Osveral® is a reliable branded generic formulation of deferasirox for treatment of chronic iron overload states.

Alpha Thalassemia is one of the most prevalent hemaglobinophaties worldwide. Alpha thalasseima patients may represent wide spectrum of symptoms ranging from asymptomatic to severe life threatening anemia. This study was done to assess the carrier frequency of alpha globin gene mutations among newborns in north of Iran. In this descriptive study, 412 cord blood samples of neonate from Amir Mazandari hospitali were randomly selected during 2012. Genomic DNA was extracted using phenol-chloroform method. Multiplex Gap- PCR and PCR-RFLP methods were applied in order to detect three common gene deletions, one triplication and one point mutation. Total allelic frequency of investigated mutations was 0.0825. The -α3.7 deletion with allelic frequency of 0.0485 was the most prevalent mutation among 412 neonates. Allelic frequencies of -α4.2, αααanti3.7 triplication and α-5nt mutations were 0.0206, 0.0109 and 0.0024 respectively and -Med double gene deletion was not detected. Most mutated cases had single gene deletion that is asymptomatic while -Med double gene deletion was not detected among the neonates. Therefore, there is low probability of a child birth with Hb H disorder in the region.

Background and Patients with Beta thalassemia major need consistent blood transfusion from early years of life. Deferasirox is used as an oral chelating agent (once daily) to excrete excess iron. This study aimed to compare the efficacy of deferasirox twice daily and the usual once daily dosing. This before after clinical trial was performed in 2013-2014 in patients who were at least 2 years of age and received only deferasirox as the chelating agent. All patients had received deferasirox for at least six month once daily. The last ferritin before entering the study and the mean deferasirox daily dose during the previous six months were considered as baseline ferritin and deferasirox dose, respectively. Laboratory tests were performed including CBC-diff, serum ferritin, Creatinine (Cr), Aspartate Aminotransferase (AST) and Alanine Aminotransferase (ALT) to evaluate the efficacy and safety of deferasirox. A total of 21 transfusion-dependent patients (mean age: 21±6 years old) were included of whom 67% were male. The mean ferritin level decreased significantly from 1814± 922 ng/ml to 1472± 907 ng/ml (P= 0.02). There were no any significant changes in AST, ALT and Cr levels compared to baseline values. Twice daily dosing of deferasirox was associated with more decrease in ferritin level compared to baseline single daily dose values without any hepatic or renal adverse effects.

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

The national screening program for G6PD enzyme deficiency is not able to detect all affected neonates. This study was done to compare the fluorescent spot test (FST), decolorization test, and quantitative enzyme assay (QEA) for detecting G6PD enzyme deficiency in neonates. In this descriptive study, cord blood samples of 365 neonates were collected. Decolorization test, QEA and DNA test was done for each sample. All of the neonates were tested by FST as a part of national screening program on heel-prick blood sample collected on day 3–5 after birth. QEA was considered as the gold standard. According to QEA test results, neonates with <20% and 20–60% of mean normal enzyme activity were considered as total deficient and partial deficient, respectively. Fluorescent spot test detected 13 male neonates with G6PD enzyme deficiency while decolorization test identified 18 male and 1 female neonates. Using QEA, 19 of male and 28 of female neonates with G6PD enzyme deficiency (26 cases with partial and 2 cases with total deficiency) were diagnosed. DNA analysis detected 34 female case as heterozygote and 14 male neonates as hemizygote for the disease. Fluorescent spot test do not have required sensitivity for screening of neonates with G6PD enzyme deficiency. QEA test is recommended to replace the fluorescent spot test in national screening program.

Hemoglobin D-Punjab is one of the variant of hemoglobin caused by a mutation on position 121 of beta globin gene which is frequent in India, Pakistan and Iran. Heterozygote form of this variant is mainly asymptomatic while in combination with hemoglobin S, severe form of anemia occure. This study was carried out to determine the beta globin gene haplotypes associated with hemoglobin D-Punjab in Northern Iran. This descriptive study was carried out on families of 18 individuals whom were carriers of hemoglobin D-Punjab in Sari in Northern Iran. Genomic DNA was extracted from peripheral blood samples using Phenol-chloroform standard protocol. In order to identify different haplotypes associated with hemoglobin D-Punjab, PCR-RFLP method and family linkage analysis were used. In 17 subjects hemoglobin D-Punjab was linked to [+ - - - - + +] haplotype and in one case association with [- + + - + + +] haplotype was observed. The hemoglobin D-Punjab alleles have mainly unicentric origin and [- + + - + + +] rare haplotype may have different genetic origin or is created as a result of gene recombination.

Background and Screening for congenital hypothyroidism in newborns is carried out with TSH measurement of blood samples from newborn heel in Mazandaran province since 2006. This paper reports the status of these children until the end of 2012 The study was performed in 2013-2014 using current information. Babies with TSH levels greater than 5 mU / L were treated with levothyroxine for three years. If the drug was stopped abruptly or gradually in three years and normal TSH achieved the disease was regarded transient, otherwise the patients were considered for lifelong follow-up treatments. For data analysis SPSS V.16 was applied. During this period, 269088 infants were screened. Among the cases 548 (54.4% male) were diagnosed with congenital hypothyroidism. The results showed that infants who were treated at each time interval were 43% of permanent and 57% transient hypothyroidism. Age at onset therapy in 95% of cases was before 60 days. The mean time to start treatment was 20.8 ± 10 days. No significant relationship was found between the sex and permanent or transient hypothyroidism. The screening program for congenital hypothyroidism is being performed successfully in Mazandaran province. In some cases, the dosage of drugs and infants’ follow-up intervals varied even between physicians. Also, levothyroxine is either not prescribed according to the infant’s weight or is recorded incorrectly. Follow-up procedures and the decision to discontinue treatment were not similar at 3 years of age of the subjects. Physicians should be informed for the strict implementation of the guidelines of the Ministry of Health. To obtain more accurate information cases of death or immigration should not be removed from the statistics.

Affecting more than 400 million people worldwide, glucose-6-phosphate dehydrogenase (G6PD) enzyme deficiency is the most common enzymopathy in the world. In Northern provinces of Iran high rates of incidence of the disease have been reported (8.7% to16.4% of the whole population) and most of these patients carry one of the three common G6PD gene mutations: Mediterranean, Chatham or Cosenza. The aim of this study was to investigate prevalence of each of these mutations among neonates in Mazandaran – a Northern Province of Iran. Four hundred and twelve blood samples were collected and using standard protocols DNA was extracted. In order to detect the above mutations PCR-RFLP method was applied. Fifty three of neonates had G6PD gene mutation (12.9%). About 17% of female and 9% of male newborns were carriers for one of the three common G6PD gene mutations. The Mediterranean type had the highest gene frequency (0.0607) among the three examined mutations. Our study shows around 17% of Mazandarani female population is carrier for one the three common mutations, and since the likelihood of having an affected child in a carrier woman is 1 in every 4 child births, we recommended all women to be screened for the presence of three common G6PD gene mutations prior to pregnancy in order to estimate the possibility of having an affected child and preparation for management of the disease.

Repeated blood transfusion is the major treatment for patients with major thalassemia. However due to antigen encounters, it may initiate body reactions, including alloantibodies against red blood cell antigens. This study was done to determine the Prevalence of alloimmunization in major beta thalassemia patients in northern Iran. This descriptive - analytic study was carried out on 218 thalassemic patients (100 males and 118 females) with average age of 22.5±7 years in northern Iran during 2010. Each sample was tested for the presence of Alloantibodies including C, Cw, Lea, E, Lua, Leb, K, Jkb, N, P1, D, Jka, M, S, Xga, e, Fya, s, c, Fyb, k, Kpa, Jsb, Lub and Coa. Eighty eight cases (40.4%; 95% CI: 33.9-46.9) were positive for the presence of alloantibodies. Alloantibodies against C, Cw, Lea red blood cell surface antigens were the most prevalent (40%). No significant correlation was found between emergence of alloantibody with the age of initial, frequency and duration of blood transfusion. Alloimmunization is a common observation in thalassemic patients and should be prevented by transfusing compatible blood.

Abstract Congenital hypothyroidism (CH) is a preventable mental retardation cause that can be diagnosed in early days of infancy and it can be treated on time.CH screening can decrease social-economic burden of the disease. The aim of this study was to determine the prevalence of permanent CH and increase transient TSH in newborns in Mazandaran. This was a review from official statistical records of Mazandaran University of Medical Sciences to control the non-transitional diseases. Screening was performed using heel prick blood collecting in the 3rdto fifth days after birth in 139,111 neonates who were born from June 2006 to June 2010 in Mazandaran. The blood sample was taken on a special filter sheet and TSH >5 mu/L was recalled. TSH>10 and T4<6.5 was diagnosed CH by venous blood sampling and treatment was done.However, in TSH ≥20 treatment began immediately. The data was analyzed using SPSS 16. 139,111 newborns were screened. 71,301(51.2%) were male and 67,810 were (48.3%) female. Also, 8,438(6%) were recalled. Prevalence of permanent CH was 0.45 in 1000 newborns (1/2272) and transient TSH was 0.62 in 1000 newborns (1/1612). Prevalence of CH in Mazandaran is more than most countries. Thus, CH screening is necessary to be continued andsearch for CH high prevalence causes in Mazandaran is needed.

Beta thalassemia is one of the most common hereditary disorders worldwide. In Iran، it is frequently reported from northern and southern provinces. In order to prevent child birth affected by this complication، prenatal screening and diagnosis is carried out nationwide. However in some instances، this program is unable to identify rare mutations leading to thalassemia. A married couple، who took part in prenatal screening and diagnosis، gave birth to a child who is affected by thalassemia major. After several molecular examination، a rare mutation [+22 5UTR (G>A)] in compound heterozygote state with a common mutation [codon 8 (-AA)] was found. This case study suggests that more advanced molecular evaluations must be integrated in prenatal screening programs to identify rare mutations and antenatal diagnosis of thalassemia cases.

Diabetes mellitus is one of complications that thalassemia major patients face with. Hence، blood glucose monitoring is of vital importance to these patients. Because of high level of fetal hemoglobin in these patients، the measurement of hemoglobin A1c is not reliable and should be displaced by fructosamine test. The current descriptive study was carried out on 33 beta-thalassemia major patients afflicted with diabetes mellitus (21 female and 12 male cases). Blood glucose level، fructosamine، hemoglobin A1c، serum ferritin and fetal hemoglobin were measured. Blood glucose levels are 204±103 mg/dL and 221±101 mg/dL (p=0. 63); fetal hemoglobin levels are 9%±7% and 13%±9% (p=0. 22); serum ferritin levels are 1744±1534 ng/mL and 3253±1773 ng/mL (p=0. 96) in female and male patients، respectively. The level of fructosamine (42±124 mmol/L) and glycosylated hemoglobin (8. 9%±1. 8%) are correlated significantly (r=0. 69، p<0. 01). Both Hemoglobin A1c (r=0. 75، p<0. 01) and fructosamine (r=0. 54، p<0/01) show a significant correlation with blood glucose level. In diabetic thalassemia major patients with frequent blood transfusion، the level of fructosamine and glycosylated hemoglobin are related significantly، therefore; they can be used alternatively.

Providing suitable facilities for the handicapped that have moving disability and have to use wheelchairs, is mandatory by a law published in 2004. The responsible person of all community service buildings are obliged to provide minimum standards for convenient access and use for the handicapped. In this study the community service places of Sari, were evaluated. This has been a cross-sectional study done in 2010. Buildings were randomly selected when there were many samples, however, all were assessed if were unique or less than 4. A check list was developed according to governmental standards. Content validity was obtained by consultation with experts and consumers. Reliability was tested by the "test-re-test" method (using Pearson correlation Coefficient, r=0.77). Two hundred and twelve buildings in 5 categories (health care, community services, educational, residential and leisure activities) were assessed by 11-20 items regarding the function and services to provide. The included items were: presence of ramp, sliding door, lift, handicap sign, reserved parking place, suitable bathroom, toilet, special mattress, height adjustable bed. Scales for evaluation were as; existed as standard, existed but not as standard, and not existed. The administrative asked if there was routine inspection visits by responsible bodies. Data entered the SPSS (16) software for analyzing. eighty two community service buildings (bank, post office, registry office), 75 health centers (hospital/clinic/physiotherapy/radiology/pharmacy), 31 educational buildings, 16 entertaining/leisure places (cinema, park, restaurant, gymnasium) and 8 residential buildings (hotels, sanatorium), were evaluated. Routine periodic and formal administrative inspections were hardly performed for evaluated facilities. In most centers there have not been any standard slopes. In almost all above-mentioned centers, there were no signs, parking space, and even restrooms dedicated to handicapped. Only 8% of facilities for community services, 12.5% of residential buildings and 3% of health centers had standard slope. There were no suitable beds, toilet and bathrooms in hotels. Despite the existence of law in the country and international standards, almost all basic community buildings are unsuitable for the disabled people, yet they are not under routine surveillance. Planning for correct performance of laws is necessary and essential

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

Background and Âlthough congenital hypothyroidism is a preventable cause of mental disabilities, less than 10% of newborns are diagnosed based on clinical symptoms in the first month. Ând if the diagnosis is not made based on screening programs, it would be delayed and irreparable cerebral and auditory complications will result. The purpose of this study was to determine the prevalence of congenital hypothyroidism in the Mazandaran province. The study is a descriptive (screening) survey. From infants born from January 2007 to January 2008 in Mazandaran province heel blood samples on filter paper were obtained. TSH values were measured by ËLÏSÂ method and TSH> 5 mlu/L was considered as the recall criterion. Diagnosis of neonatal hypothyroidism was made based on TSH>10 and T4<6.5. Treatment was performed based on the results of second tests. Ëxcept in cases where the initial TSH was greater or equal to 20 -where the treatment was started from the beginning- if the second test results were reported normal, treatment was discontinued and the case was considered as a transient hypothyroidism. Ôut of 45218 infants studied, 49% were female and 51% were male. 4.6% of newborns (2109) were recalled, among whom 1885 had 5< TSH< 10 mlu/L and 185 had 10< TSH< 20 mlu/L and 39 had TSH ³ 20.73 infants were diagnosed with hypothyroidism. Thus, the prevalence of neonatal hypothyroidism was 1.6/1000 in live births in Mazandaran province. Âlso prevalence of transient neonatal hypothyroidism was 1 in every 2500 live births.Çonclusion: Given the high prevalence of hypothyroidism in the province, more screening program is essential.

Background and Frequent transfusions put the patients with thalassemia at risk of iron overload complications which in turn can lead to failure in the function of organs such as heart and endocrine glands like liver and pancreas. Hence، in this study، the researchers are aiming at investigating the clinical and laboratory changes of pancreas in thalassemic patients. This is a descriptive-analytic study in which 64 thalassemia major patients were enrolled and divided in two groups of diabetic and non diabetic. Ïn addition to some demographic and laboratory characteristics، serum amylase، trypsin، lipase، and fecal elastase were measured in both groups. Data were analyzed using t-test، Mann-Whitney، and Fisher’s exact tests. Significant difference was observed in the mean of the last three serum ferritin level between the two groups (P<0. 05). Findings showed that diabetic and non-diabetic patients didn’t have significant differences in the mean level of serum insulin and amylase، and fecal elastase، while significant differences were observed in FBS and 2h postprandial serum glucose (P<0. 05). The analysis of the laboratory tests showed significant differences in serum lipase and fecal elastase in the two groups (P<0. 05). Çonclusion: Diabetes mellitus can lead to some adverse effects on pancreatic functions in the thalassemic children. The incidence of diabetes in thalassemia is more due to tissue resistance against insulin rather than pancreatic beta cells destruction and a decline in the serum insulin level.

Congenital hypothyroidism is a neonatal disorder that late diagnosis and treatment of it can be resulted to mental retardation and irreversible brain damage. Neonatal goiter is a rare condition that can be associated with hypothyroidism or hyperthyroidism. The aim of this report is early diagnosis and treatment. CASE: In this article we report two female twins that had goiter and prenatal diagnosis by ultrasonography had been documented for them. At third days of life patients with diagnosis of congenital hypothyroidism treated with Levothyroxin. Now they are 10 months old and developmentally normal. Size of goiter has been reduced and thyroid function tests are acceptable. Results of this report revealed that in conditions that prenatal management of fetal goiter is not available; brain damage in patients can be prevented by early etiologic diagnosis of neonatal goiter and early treatment of dyshormonogenesis.