Background and Aim: Cardiac complications due to Iron overload are the most common cause of death in B-thalassemicpatients. Although regular blood transfusions in THALASSEMIA MAJOR(TM) patients have improved the quality oflife of the patients but the most important complication of such transfusions is iron overload in cardiac tissues. In spite of iron overload in untransfused THALASSEMIA intermedia (TI) patients, the intestinal absorption of iron increases in these patients because of ineffective erythropoesis. The aim of this study was to evaluate cardiac status in THALASSEMIA MAJOR and intermedia patients and the investigation of the possible effect of iron overload in the heart of B-thalassemicpatients.Material and Methods: 46 patients entered into this study. 26 patients had THALASSEMIA MAJOR with regular blood and also chelator transfusions and 20 patients with THALASSEMIA intermedia who had not received regular transfusions. The age' of the patients in the 2 groups were similar. The results of clinical evaluation and echocardiographies of the patients of the 2 groups were compared with each other. Collected data were analyzed by means of X2 and man whitney U tests.Results: Heart failure occurred in two patients with TM (9.52%) and one patient with TI (4.76%). Considerable pulmonary hypertension (systolic tricuspid gradien~ >35mmHg) was only present in 3 patients with TI (14.28%). But systolic dysfunction of left ventricle (ejection ftaction<55% or shortening ftaction<35%) occurred in 5 patients with TM (23.8%). In the patients without apparent heart disease, cardiac dimensions, LV mass, LV shortening and ejection ftactions, cardiac output and valvular involvement were significantly more in patients with TI. But the maximum speed of systolic flow out of mitral valve in primary phase was higher significantly in TM patients than TI patients.Conclusion: Regular lifelong transfusion and chelation therapy in TM patients prevents premature heart disease and pulmonary hypertension, but LV dysfunction can occur and lead to heart failure. In contrast in TI patients left ventricular function is normal but pulmonary hypertension occurs which may lead to heart failure. Left ventricular performance is better preserved when chelation treatment is adjusted to maintain the serum ferritin concentration at <1000 nglml.

Objective: In THALASSEMIA MAJOR, extramedulary hematopoiesis results in bony deformities such as sever malocclusion in the head and neck, delayed pneumatization of paranasal sinuses and so on. Also, there are many systemic and iatrogenic problems that may affect the head and neck region. The purpose of this study was to determine otorhinolaryngologic manifestations as clinical diseases in THALASSEMIA MAJOR patients. Material & Methods: In a cross sectional study 190 THALASSEMIA MAJOR patients were evaluated (by history and physical examination) for snoring, epistaxis, nasal obstruction, sinusitis, temporomandibular joint (TMJ) pain and TMJ dislocation, tinnitus and hearing loss. Radiological studies of the skull and paranasal sinuses and audiological tests were performed. The data was analyzed in different age groups with chi2 test. Findings: Relative frequency of some otorhinolaryngologic manifestations in this population was high. The differences between some clinical diseases as TMJ pain, and epistaxis in different age groups were statistically significant. Conclusion: THALASSEMIA MAJOR increases some clinical diseases in the Otolarygology field. With early diagnosis and early treatment many of them may be prevented.

Quality and life span of THALASSEMIA patients dependent on blood transfusion has been changed during recent years. But early death due to cardiovascular problems is still important. The most important reasons for mortality and, morbidity are cardiac complications.This research, randomized on 78 patients with Beta-THALASSEMIA, cases were selected from patients who were referred to hematology clinic of Dr. Sheikh Hospital in Mashhad University as out patients. There were significant differences between blood transfusion times and left ventricular feeling period (LVFP) [P=0.026] and its ratio to left ventricular emptying period (P=0.050), between serum ferittin and PR interval on EKG (P=0.000). In other evaluations there was no significant deference. LV end-diastolic, end-systolic, interventricular septum and posterior wall diameters was upper than 75 percentiles, which could be because of their chronic anemia. None of the patients had overt heart failure.

Introduction: Although new treatments have prolonged survival and improved quality of life in the patients with MAJOR THALASSEMIA, endocrine complications especially hypogonadotropic hypogonadism still occur. The objective of this study is to determine prevalence and risk factors of hypogonadism in 47 patients with MAJOR THALASSEMIA.Material and Methods: 47 patients with THALASSEMIA MAJOR aged 14 years and older were studied. Those who had any other problems were omitted from this study. Serum level of LH, FSH, Testosterone, Estradiol were measured and correlation with parameters including duration of transfusion, duration and dose of Desferal treatment, Ferreting level and pertinent clinical findings was evaluated and compared in groups of patients with and without hypogonadism. Results: Short stature and underweight was seen in 68/1% of patients. Mean hight was 151 ± 10.2 and mean of weight was 43.5 ± 8.4 . Hypogonadotropic hypogonadism was seen in 63/8 %. The prevalence of hypogonadism was more in males than females (p= 0.025). No significant correlation was found between duration of transfusion, duration and dose of Desferal treatment and Ferretin level with hypogonadism.Conclusion: Endocrine complications of MAJOR THALASSEMIA is frequent and factors other than iron overload including zinc deficiency or genetic differences may influence susceptibility to hypogonadism.

Background THALASSEMIA is the most common hereditary disorder worldwide. The patient's’ survival is dependent on lifetime blood transfusion which leads to iron overload and its toxicity on various organs including endocrine glands. The study aimed to investigate endocrine disorders in patients with BetaTHALASSEMIA MAJOR in the Southern Khorasan province, Iran. Materials and Methods In this descriptive cross sectional study of the 42 patients with beta-THALASSEMIA MAJOR blood samples were taken in the fasting to check laboratory tests such as fasting blood sugar, calcium, phosphorous, thyroid stimulating hormone (TSH), thyroxine (T4), luteinizing hormone (LH), follicle stimulating hormone (FSH), parathyroid hormone (PTH), Ferritin. Data analyzed using SPSS version 16. 0 software. Results Results showed that 85. 7% of patients had endocrine disorders. The most common endocrine abnormalities were hypogonadism (71. 4%), hypoparathyroidism (21. 4%), diabetes (14. 3%), and hypothyroidism (7. 2%), respectively. Conclusion Our results concluded that endocrine evaluation must be carried out regularly in patients with betaTHALASSEMIA MAJOR. Patients who have abnormal endocrine laboratory results should be reevaluated for compliance with chelation therapy and the transfusion program.

Introduction: Beta-THALASSEMIA MAJOR is a hemolytic anemia. If these patients are not treated, bone marrow hyperplasia will happen. Hematopoiesis in the jaw results in mandibular and/or maxillary enlargement. To our knowledge, there are few studies about frequency of malocclusion in these patients. Therefore, this study was designed to investigate the frequency of malocclusion in beta-THALASSEMIA patients and compare it to healthy control group.Materials and Methods: This research was a cross sectional study. Seventy beta-THALASSEMIA patients and 70 healthy controls (age and sex matched) were examined for malocclusion using Angle classification, WHO malocclusion classification, determination of overjet and overbite. If crossbite, spacing, openbite, crowding and mouth breathing were observed in clinical examination they were recorded. Statistical analysis was done using Chi-square and Variants analysis.Results: Beta-THALASSEMIA patients showed class II malocclusion (angle classification) and grade 2 (WHO classification) more than healthy control group. There were significant differences in angle & WHO malocclusion classification between two groups (P<0.05). Increased overjet and mouth breathing were seen in patients more than healthy control group. There were significant differences in overjet and mouth breathing between two groups (P<0.05). There was a positive correlation between WHO malocclusion classification and serum ferritin levels, but not between angle malocclusion classification, overjet and serum ferritin levels.Conclusion: Beta-THALASSEMIA MAJOR is associated with increased malocclusion and these anomalies will occur if the patients are not treated early. Patients with malocclusion should be visited and followed up by a dentist for dental problems. Fortunately, as a result of new treatments these patients live longer than before. Therefore, it is important to pay attention to their functional and esthetical problems.