Acta Medica Iranica
Volume:54 Issue: 10, Oct 2016

LPS-Responsive Beige-like Anchor (LRBA) deficiency is a disease which has recently been described in a group of patients with common variable immunodeficiency (CVID) in association with autoimmunity and/or inflammatory bowel disease (IBD)-like phenotype. We here describe a 10-year-old boy who experienced recurrent infections, mainly in the respiratory system, associated with thrombocytopenia and anemia. Immunological workup showed low numbers of B cells and low IgG, but normal IgM levels. In spite of therapeutic doses of antibiotics, antivirals, and antifungal agents, in addition to immunoglobulin replacement therapy, he developed disseminated involvement of both lungs with peripheral nodules; transbronchial lung biopsy revealed possible bronchiolitis obliterans organizing pneumonia (BOOP). Combined homozygosity mapping and exome sequencing identified a homozygous LRBA mutation in this patient (p.Asp248Glufs*2). Such clinical and immunological findings have not been described to date and illustrate the broad and variable clinical phenotype of human LRBA deficiency.

Renal ischemia-reperfusion (IR) contributes to the development of acute renal failure (ARF). Oxygen free radicals are considered to be principal components involved in the pathophysiological tissue alterations observed during renal IR. The purpose of this study was to investigate the combination effect of melatonin (MEL) and erythropoietin (EPO), which are a potent antioxidant and anti-apoptotic agents, in IR-induced renal injury in rats. Wistar Albino rats were unilaterally nephrectomized and subjected to 45 min of renal pedicle occlusion followed by 24 h reperfusion. MEL (10 mg/kg, i.p) and EPO (5000 U/kg, i.p) were administered prior to ischemia. After 24 h reperfusion, following decapitation, blood samples were collected for the determination of superoxide dismutase (SOD), glutathione peroxidase (GPx), and malondialdehyde (MDA) levels. Also, renal samples were taken for histological evaluation and apoptosis assay. Ischemia-reperfusion increased SOD, GPx, MDA levels, and TUNEL positive cells. Histopathological findings of the IR group confirmed that there was renal impairment in the tubular epithelium. Treatment with EPO and MEL decreased SOD, GPx, and MDA levels, histopathological changes, and TUNEL positive cells. These results indicated that the combination of MEL and EPO could not exert more nephroprotective and anti-apoptotic effects than MEL treatment in renal ischemia-reperfusion injury.

Cirrhosis is the consequence of chronic liver disease. Deleterious effects of oxidative stress on hepatocytes may be reflected in the erythrocyte membrane. Naltrexone (NTX) has been shown to attenuate hepatocellular injury in fibrotic animal models. The aim of this study was to investigate the progressive effect of CCl4 on the liver and whether the improvement of liver cirrhosis can be monitored through alterations in the erythrocyte membrane. In this study, 84 male Wistar rats were divided into 4 groups and received reagents (i.p.) as follows: 1- CCl₄, 2- NTX CCl₄, 3- Mineral Oil (M), and 4- NTX M. After 2, 6 and 8 weeks, the blood and liver tissue samples were collected. Plasma enzyme activities, the content of erythrocyte GSH and some membrane compositions, including protein carbonyl, protein sulfhydryl, and malondialdehyde were assessed. After 6 and 8 weeks, plasma enzyme activities and the content of protein carbonyl were higher in CCl4 group significantly, as compared to other groups (P

Resection is the most common treatment choice for sigmoid volvulus, a common complication in our region. A new minimally invasive technique for sigmoid resection with local anesthesia was done in this study. This method is invented to avoid general on regional anesthesia in high-risk patients. Nineteen patients were evaluated and then 14 were enrolled in this study. Sigmoidectomy with a left lower quadrant incision was performed and demographic data, the length of hospital stay, complications and procedure time were recorded. The mean age of participants was 65.68, and the male to female ratio was 1:2.7. The mean duration of the operation was 91.42 min. Complications include one case each of wound hematoma and wound infection. The intraoperative pain score was 1.2/10 and postoperative pain score was 2.35/10. The mean hospital staying was 8.3 days. By meticulous patient selection, sigmoidectomy under local anesthesia for sigmoid volvulus could be a surgeons’ armamentarium in special situations.

Retrorectal tumors are rare lesions in adults, which remains a difficult diagnostic and management problem. The purpose of this study was to evaluate the results of surgical management of retrorectal tumors in our institution. In a retrospective study, a consecutive series of patients who underwent surgical excision of a retrorectal tumor were identified from a database. Medical records, radiology, pathology reports and surgical approach were checked retrospectively. The data was analyzed using SPSS statistical software (version 18). From 50 patients, 24 were male, and 26 were female with the mean age of 41.7 years. The origin of mass was congenital in 46% (23 cases) and neurogenic in 14% (7 patients), bone origin in 12% (6 cases) and miscellaneous in 24% (12 cases). In total, 56.7% (21 cases) were malignant. Surgical approaches included laparotomy in 11 cases, the sacral approach in 17 cases, the anterior-posterior approach in 14 cases and one case through abdomino-sacral approach. The mean follow-up was 56.7 (10-277) month. Ten patients died due to extensive metastases with a mean survival of 46.6 (1-158) months. Primary urethrorectal tumors are very rare. Successful treatment of these tumors requires careful clinical evaluation and expertise in pelvic surgery.

Gestational diabetes increases the risks for mother and fetus during pregnancy. As a principle of caring for diabetes, recommending patients to lose weight through more physical activities and a restrictive diet has a long history. The present study was carried out aiming at determining and comparing dietary style and physical activity in women with gestational diabetes and healthy pregnant women. This descriptive-comparative study was carried out simultaneously on 200 pregnant women (with gestational diabetes and healthy). Tools for data collection were questionnaires of dietary style and physical activity during pregnancy. After obtaining research informed consent from samples, sampling was done by multi-stage convenience random sampling. Data was analyzed using SPSS 21. The results of the study revealed that the demographic and obstetric variables were identical. The mean scores for the dietary style of case and control groups were 65.05 and 74.12, P

Cardiovascular adverse effects are interesting aspects of occupational noise exposure. One possible mechanism of these effects is an alternation in hypothalamic-pituitary-adrenal axis. Our aim was to measure salivary cortisol response to relatively high-intensity noise exposure in a controlled randomized trial study. We exposed 50 male volunteers to 90 dBA noise for 20 minutes and compared their level of salivary cortisol with 50 non-exposed controls. Salivary samples obtained before and after exposure. Before intervention means (SD) salivary cortisol level were 3.24 (0.47)ng/ml and 3.25 (0.41)ng/ml for exposed and non-exposed groups respectively. Mean salivary cortisol level increased to 4.17 ng/mlafter intervention in exposure group. This increment was statistically significant (P=0.00). Mean salivary cortisol level of the non-exposed group had statistically non-significant decrement after this period (0.2 ng/ml). The difference between salivary cortisol level of non-exposed and exposed groups after the intervention was statistically significant. Noise exposure may affect the hypothalamic-pituitary-adrenal axis activity, and this may be one of the mechanisms of noise exposure cardiovascular effects.

The widespread use of internet has caused new psychological, social, and educational problems for the students. The aim of this study was to examine the quality of life in medical students who suffer from internet addiction. This cross-sectional survey was carried out in Tehran University of Medical Sciences, and a total of 174 fourth-to seventh-year undergraduate medical students were enrolled. The quality of life was assessed by WHOQOL-BREF questionnaire which covers four domains of physical health, psychological, social relationships, and the environment. For assessing internet addiction, we used Internet Addiction Test (IAT) of Young. The students with IAT score higher than 50 were considered as addicted. For evaluating academic performance, the students were requested to report their grade point average (GPA). The mean IA score (±SD) was 34.13±12.76. Twenty-eight students (16.90%) had IAT score above 50. The mean quality of life score in internet addicted group was 54.97±11.38 versus 61.65±11.21 in normal group (P=0.005). Furthermore, there was a negative correlation between IA score and physical domain (r=-0.18, P=0.02); psychological domain (r=-0.35, P=0.000); and social relation domain (r=-0.26, P=0.001). Mean GPA was significantly lower in the addicted group. It seems that quality of life is lower in the internet addicted medical students; moreover, such students academically perform poorer in comparison with non-addicts. Since internet addiction is increasing at a rapid pace which may provoke considerable academic, psychological and social implications; as a result, it may require screening programs to the immediate finding of such problem to give consultations to prevent unwanted complications.

Creating a safe of health care system requires the establishment of High Reliability Organizations (HROs), which reduces errors, and increases the level of safety in hospitals. This model focuses on improving reliability through higher process design, building a culture of accreditation, and leveraging human factors. The present study intends to determine the readiness of hospitals for the establishment of HROs model in Tehran University of Medical Sciences from the viewpoint of managers of these hospitals. This is a descriptive-analytical study carried out in 2013-2014. The research population consists of 105 senior and middle managers of 15 hospitals of Tehran University of Medical Sciences. The data collection tool was a 55-question researcher-made questionnaire, included six elements of HROs to assess the level of readiness for establishing HROS model from managers’ point of view. The validity of the questionnaire was calculated through the content validity method using 10 experts in the area of hospitals’ accreditation, and its reliability was calculated through test-retest method with a correlation coefficient of 0.90. The response rate was 90 percent. The Likert scale was used for the questions, and data analysis was conducted through SPSS version 21 Descriptive statistics was presented via tables and normal distributions of data and means. Analytical methods, including t-test, Mann-Whitney, Spearman, and Kruskal-Wallis, were used for presenting inferential statistics. The study showed that from the viewpoint of senior and middle managers of the hospitals considered in this study, these hospitals are indeed ready for acceptance and establishment of HROs model. A significant relationship was showed between HROs model and its elements with demographic details of managers like their age, work experience, management experience, and level of management. Although the studied hospitals, as viewed by their managers, are capable of attaining the goals of HROs, it seems there are a lot of challenges in this way. Therefore, it is suggested that a detailed audit is conducted among hospitals’ current status regarding different characteristics of HROs, and workshops are held for medical and non-medical employees and managers of hospitals as an influencing factor; and a re-assessment process afterward, can help moving the hospitals from their current position towards an HROs culture.

Team-based learning is designed to provide students with both conceptual and procedural knowledge, aiming to enhance active learning and critical thinking. In the present study, team-based learning and lecture methods in teaching the “hospital organization and management” course among hospital management students were compared. This quasi-experimental study was conducted on 25 undergraduate students of management. Teaching sessions were divided into two parts. The first part was taught with interactive lectures and the second part with team-based learning method. The student's knowledge was measured before, immediately and two months (late post-test) after teaching. Finally, the mean scores of the final exam and student's satisfaction towards the methods of teaching were measured. There was an improvement in test scores of the students after the TBL sessions when compared to the test scores after lecture sessions (P

Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. There is not currently any specific serological markers for AOSD , and diagnosis still relying on the exclusion of other likely diagnoses. Yamaguchi’s criteria are used as a diagnostic criterion which contains negative serologic markers for other collagen vascular diseases including systemic lupus erythematosus and rheumatoid arthritis. Here we report a 28-year-old woman with arthralgia, fever, rash, leukocytosis, lymphadenopathy, sore throat, abnormal liver function and negative rheumatoid factor and ANA but seropositive for anti-CCP, anti-dsDNA, and C-ANCA. It seems that despite AOSD is considered as a seronegativedisorder; it should be remembered in patients with compatible findings who are seropositive.

Anatomical variations of duodenum such as atresia, stenosis, and variations in shape have been described by various authors, but the existence of a gross anomaly in shape and position of midgut segment of the duodenum is rare. Few reported cases of duodenal anomalies date back to early twentieth century. In the wake of advancement of imaging techniques and minimal access surgeries, authors report a case of a rare duodenal anomaly. The reported case assumes significance because of thepossibility of misinterpretation of radiological images which has been discussed while citing such references. The present case reports an anomaly of the position of the midgut segment of the duodenum in an adult female cadaver. It was observed that the lower half of second part of duodenum was coiled like a serpent on the upper pole of right kidney. This part coursed initially upwards running parallel and to the right of the upper half of the second part of the duodenum. The third part coursed downwards and to the left, posterior to head and neck of pancreas, in its course sandwiching the commencement of portal vein.