Studies in Medical Sciences
Year:2007 | Volume:18 | Issue:2|Papers Count:17

Introduction: Wilson disease (WD) is one of the rare disorders affecting most of the organs. The WD gene located on chromosome 13, encodes for a copper-transporting P-type ATPase (ATP7B).Diagnosis of WD is based on low copper serum levels, high urinary copper elimination, and genetic analysis of families. The aim of this project was to study affected families referred to Genetic Center by application of microsatellites linked to WD gene in order to diagnose pre-symptomatic and carrier members.Material and Methods: Families referred to genetic center by specialists had at least one affected member. The parents of these families were genetically analyzed by applying microsatellites tightly linked to WD gene. Informative markers were selected and utilized to study all members of the families including patients and siblings to trace the mutated gene.Results: Twenty four families out of 28 families referred by specialists were selected for the study. Nine microsatellites from WD gene region including: D13S133, D13S228, D13S296, D13S297, D13S298, D13S301, D13S314, D13S315, and D13S316 were selected for haplotype study. 19 families were informative for the study. Two of the markers, D13S314 and D13S301 were highly informative in these families respectively. These markers were utilized to study 12 families and the remaining families (7 families) were studied by applying a combination of one of these markers and other markers.Conclusion: Two of the microsatellites, D13S314 and D13S301 have the most common application in diagnosis of carrier and pre-symptomatic members of affected families referred from East and West Azarbaijan. Therefore, it is suggested to apply these markers respectively to study further referred families from the mentioned provinces at the first step.

Background & aim: Succinylcholine, a depolarizing muscle relaxant, is used to facilitate end tracheal intubation. One of its adverse effects, transient fasciculation after its administration, is in close connection with its other side effects. The present study aimed to investigate the effects of its slow administration on the incidence and severity of fasciculation.Materials & method: Fifty patients, aged 18-50 years old, ASA class I-II who had been admitted for elective surgery under general anesthesia, were randomly divided in to two equal groups of control(C) and study(S). Under equal conditions, in control group succinylcholine was administrated rapidly (the usual way) and in study group in 60 seconds. Incidence and severity of fasciculation, postoperative myalgia and intubating condition in each group were evaluated.Results: In the study group, the incidence and severity of fasciculation due to succinylcholine administration, compared with control group was less (17 vs 25, P=0.026) (7 vs 20, p=0.035).Intubating condition and postoperative myalgia in two groups were comparable. Discussion: It seems that slow administration of succinylcholine is a simple and feasible way of decreasing severity of fasciculation with no adverse effects.

Background: Kidney transplant is a successful treatment for patients with end stage renal Disease. Survival of graft necessitates immunosuppressive therapy. Multiple drugs utilized for prevention of rejection and the human knowledge was interested in finding the most effective drugs that would maintain graft and patient survival with the lowest possible complications. In this study we compare the effects of AZA and MMF on one year graft survival. Rate of rejection in first year after transplantation predict the one year and consequently long-term survival.Materials & Methods: This study was conducted as a historical cohort on 191 patients that were transplanted from living unrelated donor in urmia Emam Khomeini hospital from 1381- 1383. The patients were followed up for one year in two different groups (AZA & MMF). Rejection and graft function were considered as events in this study. Life table, Kaplan meire and cox proportional hazard were used. P-value was set at 0.05.Results: Eighty one patients were in MMF and 90 in AZA group. Acute rejection in the first year was higher in AZA than MMF %43.7 vs %32.8. One year graft survival on MMF was higher than AZA %90.9 Vs %87.7 but not statistically significant P= 0.32. Survival in patients with delayed graft function (DGF) were significantly better in MMF %89.6 Vs %75 & P= 0.005. In AZA, rate of acute rejection in first year was predictive for graft survival. (CI = 1.93 - 11.26, Psig = 0.001) HR = 4.6 Conclusion: Long term graft survival related to one year survival. Graft function at the end of the first year was not significantly different between two groups; although after DGF, kidney function in MMF was better than AZA. Acute rejection in MMF was lower than AZA. Comparing long term survival can better differentiate these drugs' effectiveness.

Backgrounds & Aims: In the past, use of drain in thyroid surgeries was generally advised because of the fear of post operative hematoma and airway obstruction. This method is not approved by some surgeons. However, to our knowledge no study has shown the benefits of drainage. For this reason, we designed a study to evaluate the benefits or disadvantages of this technique.Materials & Methods: In a randomized prospective analytic study 60 patients undergoing thyroidectomy were studied for one year (from 1/1/1384 to 1/1/1385) in two groups: A (with drain) and B (without drain). The patients were assessed and physically examined, at the first, third and thirty days after operation. Patients with substernal thyroid, coaglopathy and need for modified radical neck dissection were excluded from this study.Results: One patient from each both groups was needed to be reoperated due to hematoma and airway obstruction, on the first postoperative day. Statistical analysis showed no differences between two groups according to hematoma, bleeding and seroma. The length of hospital staying time was significantly shorter in the undrain group. (Mean 2.13±0.34 versus 3.03±1.09 day, p = 0.0001). Most of patients in no drain group were ready for discharge within 24-48 hours after surgery.Conclusion: This study demonstrates that the uses of drain doesn’t prevent massive hematoma, airway obstruction, and needs meticulous dissection and transoperative hemostasis. We concluded that, use of drainage has no benefit after uncomplicated primary thyroid surgery and should be used selectively.

Background & Aims: It has been shown that peroxidation of lipids and lipoproteins are important stages in the development and progress of arteriosclerosis. The aim of the present study was evaluation of the difference between serum lipid profiles and malondialdhyde (MDA) concentration on patients with different grading of retina arteriosclerosis.Materials & Methods: The study group included 99 patients with retina arteriosclerosis proven by fluoresce in angiography and 65 healthy volunteers as control group. Classification of retina arteriosclerosis was done according to Scheie classification, that degree of insidure is from I, II, III and IV. Lipid profile concentration was measured by enzymatic methods and MDA was measured by thiobarbituric acid method.Results: Patients had, in sum an increased cholesterol, triglyceride, LDL-C and decreased HDL-C concentration when compared with the control group (p<0.0001 in all the cases). A significant difference in MDA concentrations between patients with different retina arteriosclerosis was found. A significant correlation between retina arteriosclerosis degree and lipid profile was noticed.Conclusion: The results suggest the association of altered serum levels of lipid, lipoproteins, and increased concentrations of lipid peroxides with pathogenesis of retina arteriosclerosis. However, the effect of MDA levels in increasing retina arteriosclerosis, especially degree I with other degrees, underlined with these findings.

Objective: Increments of oxidative stress has been considered as one of potential cause for the accelerated atherosclerosis in hemodialysed patients. Vitamin C represents one of the most prominent antioxidants exerting beneficial effects by inhibition of lipid peroxidation and by reducing endothelial dysfunction. The aim of this project was to study the effects of vitamin C supplementation on serum lipid profile and oxidative marker, malondialdehyde (MDA), in hemodialysed patients admitted to Shahid Hashemi Nejad Hospital.Materials & Methods: forty-two hemodialysed patients randomly received 250 mg vitamin C supplementation in case group (21 patients) or placebo pills in control group (21 patients) every other day for the period of 12 weeks ten ml blood samples were drawn after fasting overnight, at baseline and after 12 weeks of supplementation. Serum TG, total cholesterol, LDL-C, HDL-C, MDA and vitamin C were measured.Results: At the end of the study, serum vitamin C concentration showed a significant increase in the case group compared to baseline (p=0.033) and control group as well (p=0.001). Vitamin C supplementation decreased serum MDA concentration (p=0.057) so that the changes of MDA between two groups indicated a significant difference (p=0.024). After 12 weeks of supplementation a significant reduction was observed in serum total cholesterol (p=0.005), LDL-C (0.012) and the changes of LDL-C/ LDL-C ratio (p=0.018) in case group were compared to the control. HDL-C Vitamin C supplementation did not lead to any significant changes in other variables such as serum TG and HDL-C concentrations.Conclusion: Supplementation with 250 mg vitamin C taken every other day, increased associated serum concentration level and decreased serum MDA level of subjects on hemodialysis. It also inhibits the rise of serum TG, total cholesterol, and LDL-C levels in hemodialysis patients after 12 weeks of supplementation.

Background & Aims: IUFD (Intra uterine fetal death) is defined as the lack of fetal heart beat during last twenty weeks of pregnancy and it is a threat to pregnancy outcomes. Additionally it has great expenses to family and health system with emotional effects on pregnant women and may carry some poor prognosis on the next pregnancies. Prevalence rate of IUFD after twenty two weeks of pregnancy was reported 3/3-20 per 1000 birth in other countries. There are few studies done on IUFD in the world and also Iran. The aim of this study was to survey its frequency at Kosar hospital to achieve the exact statistics and investigate the possible related causes of IUFD as a descriptive method.Materials & Methods: This cross-sectional descriptive study was carried out on patients who were hospitalized in Kosar hospital and their pregnancy was terminated in more than twenty weeks of gestational age due to IUFD. (4, 5).Results: The results showed that IUFD frequency after 20 weeks of gestation in studying population was 33.4% per 1000 birth and the mean age of IUFD incidence was 30.87 + 5.7 weeks. The Frequency of³ 22w IUFD was 23.9% and 25.2% of total CIUFD's had occurred in 34-36 weeks of pregnancy.48.4% of cases had one of the highly known related causes of IUFD and 51.6% had none. Among the factors which were strongly related to IUFD, preeclampsia with 24.5% frequency had the highest rate. Discussion: Comparison of IUFD frequency in studied population with other studies indicated that its prevalence rate was high in Urmia, Iran. Probably, it is due to referral origins of our hospital and also the lack of ideal of prenatal cares especially in high risk women. The occurrence of 25.2% of IUFD at 34-36 weeks of pregnancy indicates the last problem. It is suggested to improve the quality of prenatal care in this group and necessary actions must be performed.

Background & Aims: The harmful effects of electromagnetic field (EMF) on biological system have been reported previously. Electromagnetic field as an environmental factor is produced by routinely used electrical appliances such as TV and computers. The aim of the present study was to investigate the effects of EMF on semen producing organs.Materials & Methods: For EMF production, a 50 Hz alternative current is used and a 3 mT EMF was produced. Male Wistar rats were exposed to 3 mT EMF for 2 months, 5 h/day. After the experimental period, rats in both control and experimental groups were killed, and specimens from ductus epididymis, prostate and seminal vesicle prepared for electron microscopic studies.Results: Microscopic studies showed the following changes in experimental groups, in comparison to controls; in epididiymis, the height of epithelial cells and also the height of sterocilia were increased (P<0.05), but the number of secretary cells decreased (P<0.05); in prostate, the number of intracellular secretary granules seemed to increase and nuclei of the cells became irregular in shape; in seminal vesicles, the density of secretary granules significantly changed.Conclusions: The changes produced by EMF are all indications of changes in secretory function of cells that are involved in semen production. These changes in turn can result in qualitative changes in semen.

Tumors originated from nose and paranasal sinuses consist 0.2%-0.8% of total malignancies of the body. This case report introduces a l4-year-old female who during examining for evaluation of epitasis following trauma, was found to have extensive abnormalities in the sinuses and a space occupying mass in the left maxillary and ethmoidal sinuses. Giant cell reparative granuloma is classified as a benign tumor, and is usually found in young patients and is usually associated with trauma. This lesion is an uncontrollable osteogeneticreaction.Treatment of this disease consists of preseruative surgicalr esoction of the tumor without unnecessary procedures.

Behcet's disease is a systematic disorder with unknown etiology that usually is presented with recurrent oral, vaginal, and eyes aphthous. The disease may involve the heart, lungs, gastrointestinal tract and nervous system This case is a 50 year old woman with epilepsy and hemiplegic presentation. After being evaluated with EEG and brain MRI the patient was operated with the possibility of brain tumor. Through operation no evidence of tumor was reported and the biopsy revealed reactive processes. The clinical judgment focused on vacuities. She treated with corticosteroids and phenytoin and after two-year follow up the lesion size became smaller than the previous. Eight years later neurological a symptoms recurred with oral, genital aphthous. After the concise clinical and preclinical evaluations and ruling out other differential diagnosis, a Neuro-behcet’s  disease is considered with a cerebral tumor-like lesion.Neuro-behcet’s be presented with signs of cerebral tumor-like lesions that is very rare.

Wilm's tumor is the most common renal malignancy in children. Denys-Drash syndrome (DDS) and Fraisier syndrome (PS) were identified 30 years ago as both rare syndromes leading to end-stage renal failures. DDS includes a specific nephropathy characterized by diffuse mesangial sclerosis, associated with male pseudohermaphroditism, and/ or Wilm's tumor. FS is defined by the association of focal and segmental G.N, male pseudohermaphroditism and gonadoblastoma.Our subject, a 10- month-old girl, had abdominal mass (Wilm's tumor) and nephrotic syndrome. Left nephrectomy was perfumed. Histology of non- tumoral renal tissues showed minimal changed lesions. Nephrotic syndrome was steroid-resistant but after few months her symptoms and hypertension was improved spontaneously. Now after 4 years she is good and has no problem.

The concept of oocyte donation with in vitro fertilization (IVF) and embryo transfer to recipient endometria followed the successful development of IVF technology after 1980. At about the same time attempts were made for donor embryo transfers. With regard to development of IVF technologies nowadays, the embryos resulted from IVF or ICSI (intracytoplasmic sperm injection) procedures are cry preserved and then transferred to the uterus of the same persons or donated to recipients. A 49 year-old woman with 10 year infertility duration was referred to Kosar IVF center. She had experienced premature ovarian failure from 10 years ago with no ovarian function and regular menses. After preparation of her uterus with exogenous hormone replacement therapy (HRT), a fresh 16 cellular grade A donated embryo was transferred to the uterus on the 19th day of HRT. After 15 days of fortunate transferring, the pregnancy test (serum bHCG) was positive. The ongoing pregnancy continued until 38th weeks of gestation without any complications, and then because of decreased AF index the pregnancy terminated by C-section. A healthy 3200 gr new born was discharged from the hospital on the 3th day of C-section by her mother.Embryo donation is an important step for treatment of infertility in couples with an absolute factor of infertility especially in menopause and older women. Although pregnancy in advanced ages is associated with complications such as preec1ampsia, diabetes and hypertension but with a meticulously prenatal care and proper hormonal therapy we expect a normal pregnancy and a healthy baby.