[ relationship of febrile convulsion with serum magnesium level in children between 6 months and 5 years of age]

Seizure is the most common neurological disorder among children that not only is known as a diagnosis but also as a clinical manifestation of several underlying diseases. Its pathogenesis is not yet known, but the genetic background and alterations in neurotransmitters and the rare elements such as magnesium may be involved. This study was conducted to evaluate the relationship between magnesium levels in children and seizure. This study was a case - control study and included 78 children aged between 6 months and 5 years with febrile convulsion and 74 children hospitalized for reasons other than febrile seizure in Besat Hospital from 2011 to 2012. Demographic characteristics, history and duration of disease were obtained from their parents and if the patients met the inclusion criteria, blood samples were taken for serum preparation. After preparation of serum using Hitachi 902 automated analyzer and Pars Azmoon kits, magnesium levels were measured in milligrams per liter by spectrophotometeric method. Normal range of magnesium level was considered 15-23 mg/ liter. Using SPSS version 18, descriptive statistic tests and t-test, the data were used to compare magnesium levels in both sexes and groups. We used chi-square and OR calculation to calculate the odds ratio for seizures. The highest frequency belonged to seizures with duration of 5 minutes [24%] and tonic-clonic seizures [74/4%]. Mean levels of magnesium in the boys and girls were 22/87 +/- 3/18 and 24/77 +/- 5/89 respectively, which showed a statistically significant difference [p = 0.018]. The mean magnesium level was 24/09 +/- 3/87 in children with seizure and 23/40 +/- 5/50 in healthy children which had no differences [p = 0.37]. The odds ratio for the risk of seizure in children with magnesium deficiency was 1.38.s. In general we can say that magnesium levels in children with seizure and healthy children do not differ and deficiency of this element alone cannot cause seizure, although we found higher than normal levels of magnesium in most children in our study

[Evaluation of hearing loss and otolaryngeal disorders in beta thalassemic patients treated with desferrioxamine]

Thalassemia is one of the most prevalent heamoglobinopathies in the world in particular in Iran. Major thalassemia patients need blood transfusion and desferrioxamin injections throughout their life. Regarding improved life quality of thalassemic patients, new clinical problems, such as hearing loss, need more attention. This study was done to determine the frequency of hearing loss and otolaryngeal disorders together with their related factors in major beta thalassemic patients. This was a descriptive analytic study and 84 beta thalassemia patients were examined and evaluated for hearing loss by an otolaryngologist. Standard pure tone audiometry was performed for the patients. Serum ferritin level was measured. Considering blood transfusion the patients were divided into two groups: those with suitable transfusions and those with unsuitable transfusions. Also in regard to desferrioxamin injections again the patients were divided into two groups: those with regular injections and those with irregular injections. Among 84 beta thalassemic patients [40 M, 44 F] with mean age of 12.8 +/- 5.7 years, 10 [11.9%] had sensorineural hearing loss, 8 [9.5%] had conductive hearing loss, while 8 [9.5%] showed mixed hearing loss. There was no relationship between hearing loss and age, sex, ferritin level, but hearing loss had a significant relationship with doses and duration of desferrioxamine therapy [P<0.01]. Also hearing loss had no significant relationship with regular and irregular blood transfusions and desferrioxamine injections [P<0.01]. The results of this study implicated that high dose desferrioxamine was the main factor in the pathogenesis of ototoxicity in thalassemic patients. For management of these patients it is necessary to use proper doses of desferoxamine. Also blood transfusions should be proportional to body iron burden and hemoglobin. In addition, regular periodic otolaryngologic and audiometric follow up examinations are required for early diagnosis of hearing disorders in prevention of permanent hearing loss

[Assessment of thyroid dysfunction in patients with beta- thalassemia major]

The thalassemia is believed to be the most prevalent of all human genetic diseases and caused by mutations of the synthesis of hemoglobin.Regular blood transfusions are necessary in major thalassemia patients. The combination of transfusion and chelation therapy has dramatically extended the life expectancy of thalassemic patients but an important complication is iron overload in different organs of the body. Despite improved hematologic care in recent years, in these patients primary hypothyroidism and other endocrine disorders due to iron overload are still common complication and affect the patient's quality of life. The aim of this study was to identify the prevalence of thyroid dysfunction and to determine its correlation with ferritin plasma level, amount of blood transfused and liver function in thalassemic patients. In this cross sectional study, fourty patients with beta thalassemia [20 males and 20 females; mean age, 12.7 +/- 5.8 yrs] were evaluated. Serum ferritin levels, SGOT, SGPT were evaluated by ELISA method and TSH were evaluated byIRMA. Hypothyroid index was defined according to the criteria of Kronberg et al. Normal thyroid hormone values were found in 34 patients [85%] and 6 [15%] had subclinical hypothyroidism. Mean ferritin levels in hypothyroid and normal patients were2220 +/- 1056 mg/l and 2028 +/- 1548 mg/l respectively, [p = 0.2]. Thyroid dysfunction could not be correlated with amount of blood transfused, liver function or ferritin plasma level. The high rate of thyroid dysfunction may be the result of poor disease control and manegement in early life when irreversible tissue damage occurs due to iron overload and chronic hypoxia, and indicates the importance of regular follow-up of beta thalassemia patients for early detection and manegement of associated complications

[Study of prevalence of hepatitis C and its relationship with impaired glucose tolerance and diabetes mellitus in beta-thalassemic patients]

Impaired glucose tolerance [IGT] and diabetes mellitus [DM] are well known complications in multitransfused beta thalassemia patients. Iron overload and chronic liver disease, viral infection and/or genetic factors may play important roles in the development of glucose intolerance. The aim of the present study is to determine the prevalence of hepatitis C and its relation with diabetes and impaired glucose tolerance. The study included 195 multitransfused beta thalassemic patients, 97 females and 98 males with a mean age of 14.9 +/- 6.07 years [range 5-36 years]. Diagnosis of DM and IGT was based on the criteria of ADA and WHO. Hepatitis markers were detected by use of ELISA test. Results were analyzed by means of Chi-square test. The results of ELISA test revealed forty [20.51%] patients were seropositive for HCV. After removal of confounding factors, serum ferritin level [p = 0.039] and hepatitis C infection [p = 0.006] were identified as independent risk factors, having significant relationship with abnormal glucose tolerance. With increasing age the number of blood transfusions increases and subsequently the possibility of iron overload and infection with hepatitis C virus increases. In our study the prevalence of diabetes in adult thalassemic patients suffering from HCV infection increased. It is probable that hemosiderosis makes the effect of HCV infection on glucose metabolism more evident clinically. Aggressive iron chelating therapy as well as prevention and treatment of hepatitis C infection are the most important measures in glucose homeostasis in transfusion dependent beta thalassemic patient

Cardiovascular complications of thalassemia major and thalassemia intermedia

Cardiac complications due to Iron overload are the most common cause of death in beta-thalassemic patients. Although regular blood transfusions in thalassemia major [TM] patients have improved the quality of life 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 beta-thalassemic patients. 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 Chi square and man whitney U tests. Heart failure occurred in two patients with TM [9.52%] and one patient with TI [4.76%]. Considerable pulmonary hypertension [systolic tricuspid gradient >35mmHg] was only present in 3 patients with TI [14.28%]. But systolic dysfunction of left ventricle [ejection fraction<55% or shortening fraction<35%] occurred in 5 patients with TM [23.8%]. In the patients without apparent heart disease, cardiac dimensions, LV mass, LV shortening and ejection fractions, 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. 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 nanogram/ml