Tehran University Medical Journal

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Background: Although endometrial cancer is primarily a disease of the postmenopausal female, 25% of patients are premenopausal, with 3-5% in women 40 years old or younger. The younger group of women with endometrial carcinoma are frequently nulligravid with a history of infertility, and a strong desire to preserve fertility. This may pose a therapeutic dilemma for both patients and treating physician.
Case report: We reported 3 young patients with atypical complex hyperplasia or early stage endometrial cancer that treated with conservative hormonal therapy.
Conclusion: Medical treatment of young patients with endometrial carcinoma and complex atypical hyperplasia who wish to preserve fertility is a reasonable and appealing option. A comprehensive evaluation prior to counseling the patient should include A complete history and physical examination. A formal D&C with review of history with an experienced gyn-onc pathologist. Evaluation of the pelvic and abdomen preferably with contrast-enhanced MRI or transvaginal ultrasound. In patients found to have a clinical stage I grade I tumor and who want to preserve fertility , thorough counseling include risks and benefits, and explanation that the data is partial and incomplete due to the lack of appropriate controlled studies is mandatory. In patients considered for medical treatment, a high dose progestin regimen should be started with endometrial sampling every 3 months until complete regression of the tumor is documented. Although most responses are long standing, there is a small risk of progression during or after cessation of progestin therapy.

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Background: Kernicterus, also known as bilirubin encephalopathy, is a neurologic syndrome resulting from the deposition of unconjugated bilirubin in the basal ganglia and brainstem nuclei. Indirect bilirubin is toxic for brain. Neurologic dysfunction (BIND) that include acute phase (hyperbilirubin encephalopathy) and chronic phase (Kernicterus) resulting from hyperbilirubinemia and disruption of blood brain barrier. In this study, the association between bilirubin encephalopathy and risk factors was evaluated.
Methods: In this retrospective study, 312 icteric neonates were admitted in the neonatal ward of Children's Hospital, Medical Center, Tehran, and 305 of these cases were evaluated. Patient histories were taken and physical examinations were performed. For each patient, the age, sex, birth weight, time of discharge from the hospital and risk factors were recorded, and a questionnaire was completed.
Results: In this study, of the 305 icteric neonates evaluated, 25 cases had kernicterus. Risk factors included acidosis, prematurity, hemolysis, hypoglycemia, sepsis, respiratory distress, low birth weight, ABO incompatibility and G6PD deficiency. The mean level of bilirubin in cases of kernicterus was 32 mg/dl and in the others was 20 mg/dl (p=0.001). Kernicterus was most common among high risk neonates (p<0.001). Birth weight less than 2,500 gm was also an important factor (p=0.04).
Conclusion: High-risk neonates need prompt treatment for hyperbilirubinemia compared to low risk neonates.

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Background: The aim of this study was to evaluate the association between atypical glandular cell (AGC) on Pap smear and significant pathologic finding to tailor management protocols.
Methods: Between 2002 and 2005, Among 26893 Pap smears 122 women with AGC Pap smears (prevalence=0.45%) were referred to our colposcopy clinic. Forty one women underwent colposcopy directed biopsy, endocervical curettage, endometrial sampling and cervical conization to determine the cytologic and histologic correlations of AGC on pap smears.
Results: A total of 122 women with AGC Pap smear were found. Only 41 women accepted to participate in the study and followed the workup procedures. The mean age of the patients was 46.92 ±11.48 years (range, 23-80 years). Of these patients 13 patients (31.7%) were post menopause and 28 patients (68.2%) were in reproductive age. We found 13 (31.7%) significant pathologic findings including 4 (9.7%) high grade squamous intraepithelial lesion (HG-SIL), 3(7.3%) low grade squamous intraepithelial lesion (LG-SIL), 2(4.8%) Endometrial hyperplasia, 1(2.4%) Endometrial adenocarci-noma, 1(2.4%) adenocarcinoma of cervix, 1(2.4%) squamous cell carcinoma of cervix and 1(2.4%) papillary serous tumor of ovary. There was not any significant difference in the prevalence of significant pathologic findings and subtype of squamous or adenomatous lesions between pre and postmenopausal group.
Conclusion: AGC on Pap smear was associated with a clinically significant diagnosis in approximately one third of our cases. The women with a diagnosis of AGC on cervicovaginal smear are needed to be evaluated at least with colposcopy, endocervical and endometrial curettage. Clinicians should be careful about the significance of AGC in pap smears.

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Background: Ultrasound (US) has been used in neuroprotection after cerebral ischemia however, its use is controversial. Application of US in combination with fibrinolytic agents may improve fibrinolytic effects. In this study the effects of US, alone or in combination with tissue plasminogen activator (tPA), on brain ischemic injury were examined and we studied whether US is protective in the brain injured by ischemia under normothermic conditions.
Methods: We performed two studies. In the first study, rectal and brain temperatures were compared. In the second study, we studied whether US alone or in combination with tPA is neuroprotective in thromboembolic stroke. To induce focal cerebral ischemia, a clot was formed in a catheter. Once the clot had formed, the catheter was advanced 17 mm in the internal carotid artery until its tip was 1-2 mm away from the origin of the middle cerebral artery (MCA). The preformed clot in the catheter was then injected, and the catheter was removed. The wound was then closed and the infarction volume, edema and neurological deficits were measured after MCA occlusion.
Results: The temperature in the brain was approximately 0.50 ºC lower than the rectal temperature. In the control, US+low tPA, low tPA, US+high tPA and, high tPA groups, the infarct volume (%) was 34.56±4.16, 17.09±6.72, 21.25±7.8, 13.5±10.72 and 20.61±6.17 (mean ±SD) at 48 h after MCA occlusion, respectively. The results indicate that US alone reduces the infarct volume by 30% compared to that of the control group (P<0.05). US improved neurological deficits and reduced brain edema significantly (p<0.05).
Conclusions: This study indicate that US appears to have a protective effect, alone and in combination with tPA, in an embolic model of stroke.

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Background: The aim of this study was to compare the outcome of treatment for ovarian cancer patients who have been treated by gynecologist oncologists and patients who have been treated by general gynecologists or general surgeons.

Methods: We enrolled in this cohort retrospective study all patients diagnosed with primary ovarian cancer in Vali-e-Asr Hospital, Tehran, Iran, between April 1999 and January 2005. A total 157 consecutive patients with ovarian cancer were available for analysis. Of these, 60 patients were treated by gynecologist oncologists and 95 by general gynecologists, and two patients were treated by general surgeons.

Results: The number of patients who underwent optimal cytoreductive surgery (residual tumor <1 cm) was higher in the gynecologist oncologist group, than in the general gynecologist group (P<0.001). Repeated surgeries were required for a majority of patients in the general gynecologist group, while only a few patients in the gynecologist oncologist group needed a second operation (P<0.0001). The interval between the initial surgery and the beginning of chemotherapy was significantly longer in the gynecologist oncologist group compared to that of the general oncologist group (P=0.001). Overall survival and disease-free survival was considerably greater in the gynecologist oncologist group. Optimal cytoreductive surgery and stage of disease are prognostic factors in patients with ovarian cancer. We can therefore conclude that patients with ovarian cancer who are treated by gynecologist oncologists have a better outcome.

Conclusions: We suggest that patients requiring cytoreductive surgery for ovarian cancer be referred to a gynecologist oncologist rather than having a less specialized physician care for such cases.

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Background: This study aimed to assess the quality of life in vitiligo sufferers and its relationship with a number of variables such as age, gender, educational level, place of residence, marital status, disease duration, disease extension, visibility of lesions and skin phototype (SPT).
Methods: In this study we evaluate the quality of life in patients with vitiligo attending Razi Hospital in July and August 2005. Permission from Professor Finlay was obtained to use the DLQI (Dermatology Life Quality Index) questionnaire to evaluate the quality of life. One hundred patients with at least one vitiligo patch (age range= 14–57) answered the question-naire. Other survey questions about mentioned variables were also answered. Scoring was done according to Finlay`s guidelines. The higher the score, the greater the impairment of quality of life.
Results: The mean DLQI score in our study was 8.16 (sd=5.423) with a minimum of 0 and a maximum of 28. There were statistically significant relationships between DLQI scores and marital status, skin phototype and disease extension independently, but not between DLQI scores and other variables. The mean DLQI score was significantly higher in married compared to single patients. In fact this difference was significant in women. Married women had a statistically higher score than single women while single and married men had no significant difference. Patients with Skin Phototype IV showed a higher DLQI score than other SPTs, which was statistically notable (p=0.000). The patients with more disease extension had higher score that was statistically significant (p=0.000).
Conclusions: This study shows that vitiligo has a major impact on the quality of life and indicates specific groups that are most affected by the disease. Hence, dermatologists should pay attention to the psychologic effects of this cosmetic disease and try to decrease its extension and disfiguring effects by various treatment modalities.

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Background: Psychosocial stressors and the quality of the support system are important factors in attempted suicide. This research has studied these stressors and the condition of the support system in suicide attempt cases in Karaj, Iran. Methods: This is a part of the Iranian section of the widest multisite study on suicide prevention (SUPRE-MISS) proposed and directed by the WHO in eight countries, including Iran. Here we present data obtained from 632 suicide attempters presenting in emergency centers over a period of ten months.
Results: According to the time lapse from the stressor to the suicide attempt, proximal stressors are considered to be precipitating while distal stressors are considered to be perpetuating factors. Although, family conflicts were found to increase the risk of suicide one year after the conflict, conflicts with family (25%) and spouse (35%) were the most frequent stressors one month before the attempt, acting as proximal factors. Conflict with spouse was more prominent for people who had been married less than seven years. Conflict with family was the most important stressors for 15-25 year-old attempters. Though it is not clear which areas of conflict are more crucial in a suicide attempt, especially considering gender differences, educational and financial problems were more prominent one year before the suicide attempt. The support system was more crucial for female attempters: Although they received more practical support than males, females complained more of deficiencies in support.
Conclusions: The roles of different psychological stressors in attempted suicide vary according to the time lapse from the stressor until the suicide. Family and marital conflicts can be precipitating and perpetuating factors, while educational and financial problems appear to be perpetuating factors. Family is considered to be an important support system for a great number of attempted suicide cases, especially since it offers practical support. Support systems are crucial in preventive programs for suicide, especially among women.

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Background: Bone marrow transplantation (BMT) is the treatment of choice for many patients with malignant and nonmalignant diseases. Long-term complications such as osteoporosis should be considered, because it is directly associated with the morbidity and mortality. The purpose of this study is to assess the bone mineral density after allogenic or autologous bone marrow transplantation in patients with leukemia or lymphoma.
Methods: We prospectively investigated 63 patients undergoing BMT for acute and chronic leukemia and lymphoma. At the end of the study, a total of 28 patients were assessed. Bone mineral density (BMD) was measured prior BMT, and 6 and 12 months after BMT. Osteocalcin, bone alkaline phosphatase and C-terminal telopeptides of type 1 collagen (ICTP) were assessed. Serum concentration of calcium, phosphorous, vitamin D, PTH and sex hormones (FSH, LH, testosterone and estradiol) were also measured.
Results: There was a significant decrease in the bone mineral density of the femoral neck six months after BMT (p<0.001), 1.01±0.13g/cm² prior to BMT and 0.96±0.13 g/cm² at six months, but no considerable changes were seen in lumbar vertebrae. Bone loss between the 6th and 12th months was not observed. The levels of ICTP and phosphorus increased significantly by the 12th month (p=0.04). The level of calcium was higher at the 6th month (p=0.002) but the level of vitamin D and PTH decreased by the end of the study (p=0.04 and p=0.01, respectively) and the average of osteocalcin did not increase significantly. In women, the level of estradiol decreased by the 6th month (p=0.01), but the testosterone changes were not significant.
Conclusion: The risk of bone loss in both allogeneic and autologous BMT is higher in the femoral neck than the lumbar vertebrae, occurring mainly in the first six months after BMT. Preventive and clinical procedures should be considered.

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Background: Septic arthritis in neonates can be difficult to diagnose and may be associated with severe morbidity and even mortality. A majority of apparent septic arthritis cases may have a negative culture, thereby creating a dilemma regarding treatment. The present study investigates demographic, laboratory and clinical variables of septic arthritis in neonates hospitalized over a period of 17 years.
Methods: The medical files of all neonates hospitalized in Imam Khomeini and Vali-e-Asr Hospitals in Tehran, Iran, from 1989 to 2005 were assessed and the files of 59 neonates with a diagnosis of septic arthritis were further evaluated. Variables such as age, sex, gestational age, birth weight, history of hospitalization, body temperature, involved joints, blood and joint fluid cultures, ESR, CRP, and WBC count were evaluated in this group of hospitalized patients. Results: Of the 59 neonates, 39 cases were male and 20 cases were female. At the time of hospitalization, 45.8% of the neonates were 20-28 days old and 52.2% were 10-20 days old, and 67.7% and 35.6% of cases had been preterm and had low birth weight, respectively. Histories of hospital admission were found for 30.5% of patients. The two most common joints involved were hip (30.5%) and knee (23.72%). The mean body temperature was 36.6oC. ESR was elevated in all patients (mean 85 mm/h) and CRP was positive in all cases. The WBC count was >15,000/mm3 in 39% of the neonates and was in the range of 10,000-15,000/mm3 for 47.4%. Blood and joint fluid cultures were positive in 51% and 91.5% of cases, respectively. The organisms isolated from joint fluid culture were Klebsiella (54%), S. aureus (37%) and E. coli (9%). Conclusion: Due to the rarity of septic arthritis in neonates and paucity of signs and symptoms, all neonates with a high index of suspicion should be examined carefully with septic arthritis in mind.

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Background: Vaginal vault prolapse is a significant longer-term complication in patients undergoing hysterectomy. An important cause is generally acknowledged to be weakness in the uterosacral and cardinal ligaments. Vaginal delivery is known to be a risk factor, and obesity and menopause are predisposing factors. Urogynecologists are constantly looking for simple, safe and effective ways to cure vaginal apex prolapse. Infracoccygeal sacropexy, also known as posterior intravaginal slingplasty, is a useful and less-invasive procedure, with low morbidity. The aim of this study is to confirm the efficacy, safety and long-term outcome of infracoccygeal sacropexy for the treatment of vault prolapse.
Methods: In a prospective observational study, we performed infracoccygeal sacropexy to treat 26 patients who had at least grade 3 symptomatic vault prolapse secondary to transvaginal hysterectomy. Data collection included anatomic outcome, and intra- and post-operative complications.
Results: The mean patient age was 67 years. The mean operation time was 30 minutes and mean blood loss was 130 ml. No intraoperative rectal perforation was seen, and all patients were discharged within 24 hours of surgery. The symptomatic cure of prolapse rate, urgency, nocturia and pelvic pain was 96.2%, 88.8%, 92.6% and 77% orderly. Only one tape rejection occurred in one of the 26 patients.
Conclusion: Our initial experience with infracoccygeal sacropexy shows an efficacy similar to other more established surgical techniques for the cure of vault prolapse, but with less surgical morbidity. Therefore, the procedure is found to be efficient and safe, with shorter operation times, reduced postoperative complication and shorter hospital convalescence.

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Background: Tonsillectomy is the second most common pediatric surgery. Despite improvements in anesthetic and surgical technique, post-tonsillectomy pain continues to be a significant clinical concern for the patient, family, and physician. Young patients undergoing tonsillectomy experience postoperative pain and vomiting resulting in delays in oral feeding and in discharge from the hospital. Reduction of these side effects will lead to the improved quality of postoperative care. This study was performed to compare the efficacies of local Bupivacaine and intravenous Dexamethasone with that of a placebo on post-tonsillectomy pain and vomiting.
Methods: This clinical trial included 120 ASA I children, aged 3-15 years, undergoing tonsillectomy. The patients were randomly categorized into three groups: 1- local infiltration of 2 ml normal saline into the tonsillar pillar as a placebo 2- IV Dexamethasone (0.5 mg/kg, with a maximum of 16 mg) 3- local infiltration of 2 ml 0.5% Bupivacaine into the tonsillar pillar. After the operation, patients were observed regarding vomiting and pain at 0.5, 4, 24, 120 hours postextubation.
Results: Of 120 patients, 70 were male and 50 were female. The mean age of patients was 8.4 years. Three patients were missed in follow up. The questionnaire was completed for 117 patients. The mean duration of operation was longest in the placebo group (55 minutes) and shortest in Dexamethasone group (50 minutes). We noticed significant reduction in postoperative pain only in the Bupivacaine group and at the fourth postoperative hour. In the Dexamethasone group, during the first 24 hours, we could not statistically analyze the effect on vomiting. Since Bupivacaine and Dexamethasone reduce postoperative pain and vomiting, respectively, and are safe, cost-effective and available, we recommend using these drugs for tonsillectomy patients.
Conclusion: Considering the greater efficacy of Dexamethasone in the reduction of vomiting and that of Bupivacaine in pain reduction in other studies, we recommend further study to assess the use of these two drugs together.

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Background: Multiple sclerosis is the most common demyelinating disease of central nervous system. We prepared this study to find its epidemiologic pattern in the Iranian society.
Methods: This case-series study involved 70 patients diagnosed with multiple sclerosis according to the McDonald criteria and admitted to the Iranian Center of Neurological Research at Imam Khomeini Hospital from 2002 to 2004. Informed consent was obtained prior to patients completing a questionnaire, which included age, gender, age of onset of clinical symptoms, home location, familial history, education level, smoking habits and the season during which the disease occurred, was exacerbated or relapsed. SPSS 11 statistical software was used to analyze the data.
Results: The mean age of the patients was 32.6 years. Approximately one-third of our patients were male, 92.9% resided in urban areas, 14.3% had an academic education, and 14.3% were cigarette smokers. The average age of onset of disease was 27.55±10.42 years, and 8.6% had positive a familial history for multiple sclerosis. The symptoms most commonly started in the spring (31.4%).
Conclusion: The alternation of temperature and sunlight may be one reason for the high incidence of multiple sclerosis in spring and autumn. It seems that multiple sclerosis epidemiologic patterns in Iran are the same as those of other countries. Thus, applying the common diagnostic and treatment methods in used in other countries may raise our patients' quality of life.

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Background: Dental amalgam, a mixture of approximately 50% mercury with silver, tin, zinc and copper in varying ratios, is a major source of mercury pollution in the general population not occupationally exposed. The toxicity of mercury is enhanced because it is so readily absorbed, with around 90-100% of mercury vapor being absorbed through the oral mucosa. The aim of the current study is to examine the mercury levels in hair and nails in subjects with amalgam fillings.
Methods: For a sample of forty university students reporting infrequent fish consump-tion, with their only known exposure to mercury from amalgam fillings, mercury levels were measured in hair and nail samples using the LECO AMA 254 Advanced Mercury Analyzer (USA), according to the ASTM standard No. D-6722 test method.
Results: Mercury concentration in hair ranged from 0.09 to 3.11 mg/kg, and in nail from zero to 1.35 mg/kg. We found that subjects with five or more amalgam fillings had significantly higher levels in their hair than subjects with zero to 5 amalgam fillings (CI 95% P=0.003). However, the number of amalgam fillings had no effect on the mercury concentration in nails in these two groups (P=0.26). There was no significant difference between the levels of mercury of males and that of females tested (P=0.26 for nail and P=0.15 for hair).
Conclusion: The mercury amount in hair was 1.5 times as much as that of the nail samples, may be due to the differences in the chemical compounds, particularly those with sulfur, or the deposition of those compounds that would be affected by blood circulation during formation of hair and nails. Although the amounts of mercury found in this study were below the WHO maximum acceptable level of 6 mg/kg for mercury in human hair, the levels were sufficient to warrant the use of other dental materials such as composites in order to decrease the overall rate of exposure to mercury.

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Background: Some pregnant women are exposed to occupational noise, a risk factor for the development of the auditory system. The auditory system is one of the areas in embryonic development in which noise might induce aberrant development. Noise can change the gene expression pattern of an embryo and thereby modify the physiology of the auditory system. Therefore, noise can change the molecular structure of the developing ear. One of the critical molecules involved in development of auditory system is glycoconjugate. The aim of this study was to investigate the molecular changes of the developing spiral ganglion after exposure to industrial levels of noise.

Methods: A total of 42 pregnant mice were divided into control and experimental groups. Each group was further divided into three subgroups. The three experimental subgroups were exposed to daily noise with an intensity of 100 db for 2.5 hours until sacrifice (for the first group to be sacrificed) or day seven of postnatal life (for the other two groups). The mice offspring were sacrificed at the first, seventh and 14^th days of postnatal life. The inner ears were prepared histologically. The specimens were stained with the lectins wheat germ antigen (WGA), peanut agglutinin (PNA), Dolichos biflorus agglutinin (DBA) and BSAI-B4.

Results: The results indicated that, although there were no histological changes at the light-microscopic level in the ear development, statistical analysis showed that there was a significant decrease in the uptake of the BSA1-B4 lectin by neurons of spiral ganglion in 14th day of postnatal life in the experimental group compared to  that of the control group (p<0.05).

Conclusions: After noise exposure, in spite of normal neuronal structure, these cells were modified at the molecular level, especially in glycoconjugate expression, influencing the normal physiology of neurons and causing auditory disorders.

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Background: Frozen shoulder is a debilitating disease characterized by pain and progressive loss of motion in shoulder secondary to fibrotic inflammation of the joint capsule. The incidence is 2% in the general population, mostly affecting women 40 to 70 years of age. Therapeutic measures include physiotherapy, shoulder manipulation, corticosteroid injection and arthroscopic release. The purpose of this study was to compare the results of manipulation under anesthesia with or without concomitant intra-articular injection of corticosteroid.

Methods: Twenty-six patients (21 females, 5 males) with the clinical diagnosis of idiopathic frozen shoulder were evaluated in a randomized clinical trial. The average age of the patients was 55.7 years. Twelve cases had diabetes mellitus, which is considered a strong predisposing factor and has a poor prognosis. Thirteen patients received intra-articular corticosteroid injection just before manipulation. The outcome was assessed by evaluating functional score (Constant scoring system) at the six-month follow-up.

Results: The average score before manipulation was 28.7 out of 100, which significantly improved to 84.8 at six months after the procedure (p<0.05). The results were slightly better in the group who received corticosteroid injection, but the difference was not statistically significant (p=0.1). The outcome in the diabetic group was less favorable than in the non-diabetic patients (p<0.05).

Conclusions: Manipulation under anesthesia is a very effective method of treatment for idiopathic frozen shoulder. Concomitant intra-articular injection of corticosteroid does not seem to improve significantly the final outcome. Diabetes mellitus can negatively affect the results of this treatment method.

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Background: Burnout is one of the most important factors in reduced productivity in organizations and involves physical and mental signs, especially in the human service professions. The role of nurses in the healthcare system is vital and motivation to ensure health security is extremely important. We carried out this research to examine the relationship between burnout and mental health in the nursing staff of educational hospitals of Tehran University of Medical Sciences.
Methods: A descriptive cross-sectional survey was conducted among 200 nurses selected via probable multistage sampling. We used three instruments in this study: 1) demographic questionnaire 2) General Health Questionnaire-28 (GHQ-28) and 3) Maslach Burnout Inventory (MBI).
Results: On the whole, using the MBI subscale, we found low levels of emotional exhaustion and depersonalization and high levels of reduced sense of personal accomplishment, both in frequency and intensity. The prevalence of symptomatic samples in the GHQ-28 was 43%, and two variables, burnout and poor mental health, were related (p<0.001). Burnout was to be related to gender, age and years of work. The correlation between poor mental health and years of work as well as hours of work in a week were significant.
Conclusions: Our results suggest that there is a strong correlation between poor mental health and burnout. Furthermore, the prevalence of symptomatic samples detected in our study using the GHQ-28 was much higher than that reported in studies of the general population. The high prevalence of symptomatic samples and high prevalence of burnout in the dimension of self accomplishment, especially in younger nurses, combined with the strong correlation between poor mental health and burnout all show that care should be taken to improve the stressful conditions that nurses face.

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Background: Mutations of ELA2, the gene encoding neutrophil elastase (NE) are known to be associated with cyclic neutropenia (CN) and severe congenital neutropenia (SCN). However, high variability of these mutations has been reported. This study was designed to describe the analysis of the ELA2 gene, clinical manifestations and demographic characteristics in patients with CN and SCN.
Methods: A series of 21 patients with CN or SCN were selected, based on SCINR criteria, from the immunology ward of the Pediatric Medicine Center, Tehran, Iran, from March 2004 to August 2005. The ELA2 gene, isolated from blood samples, was analyzed using RT-PCR and automated capillary sequencing. Informed consent was obtained under the tenets of the Helsinki Declaration and the Ethical Committee of the Tehran University of Medical Sciences.
Results: Kostmann's syndrome and CN was diagnosed in three and 18 patients respectively. Of all the patients, one or two mutations were found in 18 cases (85.7%), including all three patients with SCN and 15 of the patients with CN. Exons two and four had the most mutations (eight and seven cases, respectively). Seven patients had double mutations in two distinct exons. Overall, 16 different mutations were found. At the time of presentation, the mean age of patients was 13.4 ±17.6 months, ranging from one month to seven years. Overall, 61.9% of patients had consanguineous parents. The mean absolute neutrophil count was 830.5 ±419.4 (150-2000)/mm3. On average, each patient had been admitted to the hospital 2.2 ±1.6 times. The neutrophil counts of the SCN patients were significantly higher than those of the CN patients. However, there was no significant difference in the neutrophil counts between patients with mutations and those without mutations. All patients with SCN had two or more infectious complications, although the prevalence of infectious or non-infectious complications did not correlate with ELA2 mutations or the neutropenic disorders.
Conclusion: Mutations in ELA2 appear to play an important role in the phatogenetic mechanisms of CN and SCN. Patients with CN had significantly higher neutrophil counts than SCN patients with CN. Although it possible for the gene encoding neutrophil elastase to have more than one mutation in distinct exons, we found no association between the mutations in ELA2 and their complications in CN and SCN patients.

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Background: Heat-shock proteins are part of a strictly controlled biological system that allows organisms to respond to environmental stresses. Different proinflammatory cytokines are present in the synovial tissue of rheumatoid arthritis patients. Such tissues respond to stress and induce heat-shock proteins. In addition, synovial cells are exposed to mechanical stress caused by joint motion. The effects of mechanical stress on the metabolism of the synovial cells may be substantial, even pathogenic. Heat-shock proteins are often implicated in the pathogenesis of rheumatoid arthritis. Here, we compare the levels of heat-shock protein 70 from the synovial fluid of rheumatoid arthritis and osteoarthritis patients.

Methods: Synovial fluid samples from 34 rheumatoid arthritis patients and 34 osteoar-thritis patients were analyzed for heat-shock protein 70 by an ELISA method. Statistical analysis was performed using independent T-test and one-way ANOVA. Differences were considered statistically significant at p< 0.05.

Results: The mean value of synovial fluid heat-shock protein 70 levels in rheumatoid arthritis patients was 156.30 ±128.51 and that of osteoarthritis patients was 14.98 ±11.58. The differences were statistically significant at p<0.0001. For seven rheumatoid arthritis patients suffering from mechanical knee pain, synovial fluid analysis revealed non-inflammatory effusion. The mean value of synovial fluid heat-shock protein 70 level in inflammatory synovial fluid of rheumatoid arthritis patients was significantly higher at 191±121.73 and that of non-inflammatory synovial fluid from rheumatoid arthritis patients was 21.93 ±10.06 (p< 0.05).

Conclusion: The level of heat shock protein 70 is higher in inflammatory arthritis than in non-inflammatory arthritis. Considering that patients with rheumatoid arthritis are known to have a hypertrophic synovial-lining layer, and that heat-shock protein 70 is known to protect cells against a variety of toxic conditions as well as apoptotic death, further research is needed to determine if heat-shock protein 70 induction is a sign of significant changes in the cellular and tissue metabolism or is actively participating in the pathogenesis of rheumatoid arthritis.

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Background: Definite diagnosis and treatment of Cushing's syndrome is still a dilemma. The aim of this study was to evaluate the accuracy of diagnostic tests and follow-up of patients with Cushing's syndrome.
Methods: Two hundred and fifty three consecutive cases with Cushing's syndrome during 1370-78 were studied. The screening tests were performed in all patients. High dose dexamethasone suppression test (HDDST) and ACTH measurement were carried out. MRI/CT Scan were performed and compared with laboratory data and pathologic specimens as a gold standard test.
Results: The age range was 32±11 yrs. The most frequent symptoms were weakness hypertension, typical striae, and depression .The frequency of hypertension in ACTH-dependent case were 77% vs. 36% in adrenal tumors (P< 0.001). HDDST was positive in 99% of micro and 71% of macroadenomas. Adrenal tumors showed 3.6% suppression but none in ectopic cases. HDDST had a sensitivity of 98%, specificity of 97% and accuracy equals to 98%. The frequency of different etiologies was as following: Cushing's disease in 64.8%, adrenal tumors in 32.8% and ectopic ACTH in 2.4% of patients. Trans-sphenoidal surgery (TSS) was performed in 120 patients .The patients were followed for 53±25 months whose remission periods were 46.7±23.8 months (range 4-114 months). Survival analysis showed 93% remission rate in 12mo, 82% in 2yr and only 33% after 5yr.This recurrence didn't have any platue level.
Conclusion: In our study, hypertension was more prevalent in ACTH-dependent Cushing's syndrome. HDDST had acceptable sensitivity, specificity and accuracy. Lifelong follow up of pituitary adenomas is inevitable in the case of progressive and gradual nature of recurrence in these tumors.

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Background: In rhinoplasty, periorbital edema and ecchymosis is due to soft tissue trauma and small vessel injury with subsequent exudation and bleeding. The main purpose of this study is to determine the effect of dexamethasone in reducing periorbital edema and ecchymosis and intraoperative bleeding in rhinoplasty patients.

Methods: This double-blind study included 90 patients who underwent rhinoplasty from October 2004 to March 2005. In group A, 8 mg of intravenous dexamethasone was administered only preoperatively. In group B, 8 mg of dexamethasone was administered preoperatively and continued every 8 hours postoperatively. Group C, the control group, received no dexamethasone.

Results: The degree of upper lid edema in groups A and B was significantly less than that of group C. During the first and second day the severity of upper lid edema in group B was less than that of group A, but the difference was not significant. The degree of lower lid edema during the first and second days in groups A and B was significantly less than that of group C, although it was identical in all groups during the fifth and seventh days. The degree of upper lid ecchymosis during the first and fifth days in group C was significantly more than that of groups A and B, but it was similar on the seventh day in all groups. The degree of lower lid ecchymosis on the first day in groups A and B was significantly less than that of group C however, it was similar in all groups during the second, fifth and seventh days. The volume of intraoperative bleeding in the three groups was similar. The mean period of recovery (12 days) was comparable in all groups.

Conclusions: Dexamethasone administration leads to the reduction of upper lid edema, ecchymosis and lower lid edema during the first and second postoperative days, and reduction of lower lid ecchymosis on the first postoperative day.