JOURNAL OF ISFAHAN MEDICAL SCHOOL (I.U.M.S)
Year:2006 | Volume:24 | Issue:82 (SUPPLEMENT)|Papers Count:20

Sturge-Weber syndrome is a phakomatosis with two pathognomic features including cutaneous vascular nevus of the face and the leptomeningeal angiomatosis affecting the ipsilateral occipital lobe. The genetic basis of this syndrome is not known, the reported cases are sporadic and there is no report of any woman having given birth to more than one child with the Sturge-Weber syndrome. In this paper we report two children from one family affected by the Sturge-Weber syndrome, which to the best of our knowledge, is the first report of its kind.

Introduction: This study was conducted to compare the effects of inferior oblique muscle recession and inferior oblique muscle myectomy in treatment of inferior oblique overaction.Methods: This single-blind clinical trial was performed on 76 patients (133 eyes) who had undergone inferior oblique recession (58 eyes) and myectomy (75 eyes). The effects of the two procedures on hyperdeviation in adduction, V pattern and the complications were analyzed six months after operation.Results: Mean reduction of hyperdeviation in adduction in recession procedure and myectomy was 26.3±6.60 prism diopter and 24.8±5.9 prism diopter, respectively (P<0.001). The two procedures were equally effective in improving V pattern. The two procedures were highly effective in improving mild to moderate degrees of hyperelevation (2+ to 3+) but their effect on severe degrees of hyperdeviation was less than on mild to moderate types.Undercorrection was seen in 8% of patients undergoing myectomy and 1.2% of those undergoing recession (P=0.049).Conclusions: The procedures were highly effective in mild to moderate degrees of inferior oblique muscle overaction and improving the V pattern. The rate of complication in the recession procedure was lower than in myectomy.

Background: Peripheral nerve involvement in systemic lupus erythematosus (SLE) is most frequently subclinical and asymptomatic. The involvement of peripheral nervous system as the first manifestation in SLE is rare and is dominated by distal symmetrical polyneuropathy.Case Presentation: A 37-year-old woman presented with left wrist drop due to radial nerve palsy. After seven months, the patient developed right shoulder pain and weakness (axillary nerve neuropathy). Until that time, no signs or symptoms of systemic lupus erythematosus (SLE) were detected. One month later, the patient presented with mild edema and proteinuria and SLE was diagnosed in kidney biopsy.Conclusions: This case report indicated that SLE can present with mononeuritis multiplex without any other signs or symptoms.

Introduction: Human papilloma virus (HPV) is associated 0Hh a variety of benign and malignant lesions. The role of HPV infection in bladder transitional cell carcinoma (TCC) of bladder was investigated in this study.Methods: This descriptive study was performed on 96 cases of bladder TCC (28; cases of Grade I, 51 cases of grade II, 17 cases of grade III). All specimens were stained by the immunohistochemistry staining method.Results: Of 96 TCC cases, 17 (17.8%) were HPV positive, most of which (51.3%) were grade n. Hence despite the low frequency of HPV in our specimens, it may have a role in TCC and more extensive studies are recommended.Conclusions: HPV infection in TCC is probably a significant but low occurrence. More extensive studies with other analytical techniques are warranted.

Introduction: Previous studies on exercise have showed that exercise improves learning and memory. This study was performed to determine the effects of acute and chronic treadmill running on spatial learning and memory.Methods: Spatial learning; and memory were measured in 8 days. The rats were divided into four groups as fallows: control group (C) (no exercise), group A (30 days of exercise before and 8 days of exercise during the Morris water maze test), group B (30 days of exercise before the Morris water maze test), and group D (8 days of exercise during the Morris water maze test). The time to reach the platform (latency) and the length of swim path were used to evaluate spatial learning and memory.Results: Our results showed that treadmill running produces a significant enhancement in spatial learning and memory with a decreased path length and latency to reach the platform. These findings suggest that bath spatial learning and memory improve dramatically by exposing the rats to chronic and acute modes of exercising at the same time and this improvement is more notable than when only one mode of exercise is used.Conclusions: Improvement in spatial learning and memory may be mediated by activation of specific molecular pathways.

Introduction: Epilepsy is a chronic disorder with a prevalence of 40 million worldwide. Seizure is one of the most common causes of admission to neurology emergency services. Seizures are influenced by physiologic variations in sex hormone secretion during the menstrual cycle. Both estrogen and progesterone exert significant effects on seizure threshold. The aim of this study was to measure estrogen, progesterone, LH and FSH levels during the first hour after seizure.Methods: This is a descriptive cross-sectional study. Based on statistical recommendations, we selected 49 epileptic patients with grand mal seizure referred to the Emergency Rooms bf Alzahara and Noor Hospitals, affiliated with Isfahan University of Medical Sciences, with a history of recent seizure. These patients were selected using the simple randomized method. Patients of both sexes were selected and all women were in the mid-follicular phase of the menstrual cycle. Pregnant or menopausal women or those with any history of hormonal abnormality were excluded. Blood samples were obtained during the first hour after seizure and FSH, LH, estrogen and progesterone were assayed.Data were analyzed with EPI statistical software.Results: Thirty-six patients were men and thirteen were women. Twenty three of the men (69.9%) and seven of the women had increased levels of estrogen. Progesterone level had decreased in two of the men and increased in four of the women. Twenty-five of the men (69-4%) and seven of the women (53.8%) had decreased FSH levels. Twenty six of the men (72.2%) and eleven of the women (84.6%) had normal LH levels after seizure.Conclusions: Increased serum levels of prolactin and ACTH differentiate seizure from pseudoseizure. This study found that mean serum levels of estrogen increase and FSH levels decrease following seizure, while progesterone and LH levels do not change significantly.

Introduction: Prior to immunoprophylaxis with RhIG, 7-8 percent of Rh-negative women with. Rh-positive, ABO-compatible infants develop anti-Rh (D) within six months of delivery, while only one percent of Rh-negative mothers delivering ABO-incompatible babies are immunized. A significant number of women in Iran become sensitized because immunoprophylaxis is not widely practiced, resulting in hemolytic diseases of the newborn.Methods: In this paper, the small-volume (500-600 cc) plasma exchange method was investigated in treatment of 32 women with Rhesus disease. Over a period of 4-16 years, these sensitized women had 157 unsuccessful pregnancies.Small-volume plasma exchange was carried out weekly from the end of their first trimester throughout their pregnancy to reduce anti-D titer.Results: 31 out of 32 mothers delivered babies successfully.Conclusions: Small-volume plasma exchange is an easy and safe method, both for the mother and the fetus and can producebri11iant results in treatment of Rh disease. This method is inexpensive and practical, even in small blood transfusion centers.

Introduction: Breast-feeding is a major cause of prolonged non-hemolytic jaundice and may disguise other important causes such hypothyroidism. The aim of this study was to evaluate the frequency of congenital hypothyroidism (CH) in neonates with prolonged jaundice in an area without a hypothyroid screening program but high breastfeeding rate.Methods: Jaundiced, otherwise clinically well infants more than 2 weeks of age with complaint of visible yellow color of skin or eye, brought or referred to the Newborn Nursery of Imam Hospital, Urumieh Inin, for evaluation' of prolonged jaundice were included in this study between April 2005 and March 2006. Work-up of prolonged indirect hyperbilirubinemia including direct Coombs test, blood types of infant and mother, complete blood count, blood smear, glucose-6-phosphate dehydrogenase level, reticulocyte, bilirubin level, T4, TSH and urine analysis and culture were performed.Results: All cases were breastfed. Forty-three male infants (43%) and 57 female infants (57%) were studied. Among 10.0.neonates enrolled in the study, 6 (6%) suffered from CH; 2 of these were male and the rest female.Conclusions: The frequency of CH is high in prolonged jaundice in the area where the study was conducted. Based on the results, we recommend that CH be aggressively investigated in breast-fed infants in regions without a hypothyroid screening program, but high breastfeeding rate.

Introduction: Healing of skin defects following trauma, surgery, and chronic diseases remains a significant clinical problem. Recently, the addition of rhGH (recombinant human growth hormone) has permitted the modulation of healing. Although increasing numbers of papers are reporting the positive influence of GH on wound healing, some studies have rejected it. In fact, the side effects of systemic GH administration are not fully understood. This study attempts to examine the effect of topical and low-dose rhGH administration on skin wound healing.Methods: Thirty male Wister rats weighing 250-400 g were chosen. They were anaesthetized and a full-thickness wound was created on their right flank.Then they were randomized into cases (treated with topical rhGH applied to wound surface) and controls (given saline in isovolumic amount). Each rat was physically examined 7, 10, 14, 17 and 21 days after the operation and wound beds were qualitatively ranked. Differences between the control and case groups were analyzed. P values less than 5% were considered significant.Results: There were no allergic reactions around the wound in the case group. We found the healing rate of the wounds to be significantly different between the two groups on all days (P values were 0.005, 0.008, 0.014, 0.025, and 0.046 on days 7, 10, 14, 17, and 21, respectively).Conclusions: Skin healing was different in the two groups in this animal trial. We considered wound bed condition as the criterion for comparing the two groups. On days of examination, the differences between the groups were significant. The results showed the beneficial effect of GH on healing. We used very-low-dose topical rhGH which improved the healing process. The effects of long-term rhGH administration on human scarring remain undefined and further studies are required.

Introduction: Cardiac disease due to iron overload remains the main cause of death in beta-thalassemia major (TM). Successful iron chelation is therefore essential for the optimal management of thalassemia. Deferoxamine (DFO) is still the first-line chelating agent in these patients, but compliance is a major hindrance to achieving optimal therapeutic results. Some studies have suggested that deferiprone (L1), an orally active chelating drug, provides superior cardioprotection compared to deferoxamine. The minimum time for this effect is over 6 months. We compared L1 with DFO, in beta-thalassemia major patients for cardioprotection during a one-yare period.Methods: A prospective clinical study was performed on 48 patients assigned to two equal groups. Group 1 patients only received deferiprone and group 2 patients received deferoxamine. We compared serum ferritin and left ventricular ejection fraction (LVEE) of the two groups after one year.Results: Left ventricular ejection fraction and serum ferritin were not significantly different between the two groups at the end of study (mean LVEE±SD: 62.5±5.5 in group 1, 61.2±5.8 in group 2, P=0.43; mean serum ferritin±SD: 2354±1200 ng/cc in group 1, 2284±1244 ng/cc in group2, P=0.92). Transient gastrointestinal symptoms were the most frequent adverse effects in deferipronetreated patients and there were no episodes of agranulocytosis.Conclusions: Monotherapy with L1 during a one-year period offered no superior cardioproteetion compared to DFO. Future longitudinal studies with longer follow-ups are warranted.

Introduction: While the progressed phase of urinary tuberculosis is known entity and has been characterized by the findings of intravenous pyelogram and sterile pyuria, symptoms in the earlier stages of the disease have not been fully described. The clinical presentation, methods of diagnosis and outcome of 15 cases of isolated and early detected urinary tract tuberculosis and a review of literature are presented here.Methods: A prospective analysis of 89 patients with persistent urinary tract symptoms referred to' nephrology and infectious diseases clinics of Isfahan University of Medical Sciences revealed 15 cases of isolated urinary tract tuberculosis.Results: Fifteen specimens (from 5 males, 10 females) were positive for Mycobacterium tuberculosis (13.3%) and all of the cases were immune competent and seronegative for HIV and had no other evidence of disease based on vigorous workup.Conclusions: We discuss isolated urinary tract tuberculosis and symptoms related to early stages of the disease that have not been previously addressed., We conclude that any patient with prolonged urinary symptoms and microscopic hematuria - even with normal radiologic findings deserves to be evaluated for tuberculosis.

Blunt traumatic aortic transection (TAT) is an uncommon injury with high morbidity and mortality. There has been controversy about the diagnostic methods, approach to the injury, repair time, and surgical techniques. Active augmentation of distal perfusion pressure during cross clamp is still considered as the best surgical treatment. Today, helical CT scan has replaced angiography as the best diagnostic method. There is an incidence of life-threatening arrhythmia such as ventricular fibrillation (VF) during TAT repair, especially in critically unstable patients. One of the catastrophic events, especially occurring in hemodynamically unstable patients (due to multiple trauma and/or myocardial contusion) or severely anemic patients (due to hemorrhage from different body sites) or hypoxic patients (due to myocardial and/or lung contusion) are dangerous cardiac arrhythmias, especially VF due to the manipulation of TAT site or unexpected hemorrhage from the TAT site. Meanwhile, if aorta is clamped on both sides of the TAT site despite the patient being on full cardiopulmonary bypass (CPB) through femoral cannulation, VF carries the risk of low perfusion in the ascending aorta and the arch of aorta and subsequent brain hypoxia and damage. Prophylactic cannulation of the right subclavian artery at the beginning of the surgery, in addition to femoral cannulation before left thoracotomy, can be life-saving for the patients. Presented in this report is a 21-year-old man with multiple trauma and severe head injury due to a motorcycle accident who developed TAT. First, the patient underwent cannulation of femoral artery and vein, as well as cannulation of the right subclavian artery. After opening the left pleural cavity with posterolateral thoracotomy incision, full CPB was started through femoral cannulation. The patient suddenly developed VF during manipulation of the TAT site. The right subclavian artery cannula was immediately opened resulting in complete perfusion of ascending aorta and arch of aorta preventing brain hypoxia.Conclusions: Prophylactic right subclavian artery cannulation in TAT repair, especially in hemodynamically unstable or hypoxic patients, or those with severe anemia can create a safe margin for the surgeon to prevent brain damage during this high-risk-operation.

Introduction: Zinc deficiency is one of the most prevalent micronutrient deficiencies in developing countries, including Iran. The prevalence of this deficiency is more important among vulnerable groups such as preschool children, due to the role of zinc in physical and mental growth. This study was carried out to determine the prevalence of zinc deficiency in preschool children in order to assist policymakers.Methods: A cross-sectional study was carried out on 4374 randomly-selected healthy preschool children of Iranian families nationwide in 2001. Serum zinc concentration was measured in 5 ml whole blood using atomic absorption technology. The cutoff point for zinc deficiency was set at 70 mg/dl serum zinc level.Results: The prevalence of zinc deficiency in Iranian preschool-age children was estimated to be around 31% (29.2-32.8%). The highest prevalence was seen in the region covering Sistan and Baluchistan Province, South Khorasan and Southeast Kerman (43.1-48.9%). The prevalence of zinc deficiency was significantly higher in rural areas compared to urban areas (24.9-28.8% vs. 35.8-41.6% respectively, P<0.05). No significant difference was seen between boys and girls (27.2-34.1% vs. 28.8-38.8%, respectively).Conclusions: Zinc deficiency is a major health problem among Iranian preschool children, hence adopting an appropriate strategy for dealing with this problem in all areas of the country is urgent.

Introduction: Prenatal stress may have detrimental effects on hippocampus, leading to memory disorders. However, there is thus far little information on, the, morphometrical alterations of hippocampus in prenatally stressed models. We investigated the effect of prenatal stress on memory function and volumes of the layers of hippocampus in adult rat offspring.Methods: This experimental study was performed in Yazd University of Medical Sciences and Aarhus University of Denmark from 2002 to 2004. Adult virgin female Wistar rats weighing 220 g were housed with a sexually experienced male Wistar rat. Pregnant rats were randomly assigned to prenatal stress (PS) or control (Cont) groups. Stressed animals were individually restrained in plexiglass tubes for 1 hour, on days 15-21 of gestation. Control pregnant rats were left undisturbed in their home cages. Two male pups were taken from each litter. They were housed and tested at 60 days of age (PS, n=9; Cont, n=8). Memory function was assessed according to passive avoidance test.Animals were then anesthetized and transcardially perfused with fixative solution. The brains were removed and divided into two hemispheres. The Right hemispheres were selected and 100mm thick sections were cut with vibratome and were collected along the entire extent of the hippocampus. The Cavalieri principle was used for volume estimation.Results: A significant reduction in both memory and volume in all layers of the dentate gyrus and CA3 hippocampal field was found in rats exposed to stress in the prenatal period.Conclusions: Our results provide a neuroanatomical basis that may be relevant to the reported disturbances in behavior and learning in prenatally stressed offspring.

Introduction: Arterial blood pressure and heart rate increase following laryngoscopy and tracheal intubation. This has been referred to as the pressor response which may be harmful in the presence of myocardial or cerebral pathology and necessitate the use of prophylactic measures to control it.Preoxygenation as a main component of rapid-sequence induction of general anesthesia counteracts the development of hypoxia and can induce a marked inhibition of the pressor response before endotracheal intubation. This study investigates the effect of preoxygenation and also compares it with that of lidocaine on the pressor response to tracheal intubation.Methods: Seventy-two adult ASA (American Society Anesthesiologists) I patients scheduled for elective operation under general anesthesia and endotracheal intubation were included in this study. The patients were instructed randomly to breath from a fitted facemask of either air or 100% O2 for a period of 5 minutes. Four minutes after facemask application, lidocaine or normal saline as placebo was administered and general anesthesia was induced after one minute.After intubation, inspiratory gas in air groups was switched to 100% O2. Blood pressure and heart rate were recorded at different times before and after intubation. Statistical analyses were done by using post-hoc, Kruskal-Wallis H, Friedman and chi square tests.Results: Blood pressure and heart rate increased significantly in air groups at 1 and 2 minutes after endotracheal intubation. The heart rate increased significantly in all groups except in the oxygen + lidocaine group at 1 and 2 minutes after intubation.Conclusions: The present study introduces a new use of preoxygenation, namely inhibition of the pressor response to laryngoscopy and endotracheal intubation which is comparable to the effect of lidocaine administration. In addition, combination of preoxygenation and lidocaine administration seems to be an ideal means of suppressing the components of the pressor response.