Wolfram syndrome: A case report

Wolfram syndrome (WFS) is a rare disease inherited as an autosomal dominant trait. Type I diabetes mellitus and optic atrophy are the main symptoms of the disease. It is also known as DIDMOAD syndrome due to the association of diabetes insipidus, diabetes mellitus, optic atrophy, and deafness. WFS may be associated with other disorders such as kidney failure, gonadal atrophy, and mental and behavioral disorders. This report is about a 14-year-old teenager who had suffered from vision loss and cataracts when he was 4 years old. At the age of 7 he has been diagnosed with type I diabetes mellitus due to polyuria and polydipsia. At the age of 12 he developed diabetes insipidus, neural hearing loss, urinary incontinence and bilateral hydronephrosis, neurogenic bladder, and increased blood pressure. Physicians should think of this disease and recommend genetic counselling before marriage.

Comparison of seismic performance of steel frames equipped with diagonal and chevron viscous damper under near and far field earthquakes

A complete comparison of seismic behavior of the application of Diagonal and Chevron Viscous Damper in steel structures is presented in this paper. OpenSees software is used for the numerical modeling of structures. Fifteen numbers of earthquake records are applied to the structures and full nonlinear time history analysis is done. Force-displacement of chevron braces with and without a damper are compared and the results of IDA curves are also presented for different percentiles

Comparing APACHE II, APACHE IV, SAPS II, and SOFA Predictive Power in Poisoned Patients Admitted to the Intensive Care Unit

Background: This study aimed to assess the performance of the Acute Physiology and Chronic Health Evaluation (APACHE) II, APACHE IV, Simplified Acute Physiologic Score (SAPS) II, and Sequential Organ Failure Assessment (SOFA) scores in predicting mortality rate in poisoning patients admitted to an intensive care unit (ICU).Methods: This cross-sectional study was performed on all admitted patients in the poisoning ICU of Imam Reza Hospital, Mashhad City, Iran. All patients were evaluated for three consecutive days since admission time and then every two days until discharge from ICU or death. The scoring systems mentioned above were calculated and analyzed by MedCalc statistical software version 18.9.1 and SPSS version 16.Results: Overall, 150 patients were studied, out of whom 67% (101) were male. Their mean±SD age was 41.6±18.9 years. In their whole hospitalization period, APACHE II (79.5%), SAPS II (78.7%), APACHE IV (78.4%), and SOFA (72.9%) were the most precise measures. On the first day of admission APACHE II (77.4%), on the second day, APACHE II (83.1%), on the third day, APACHE II (90.7%), and on the fifth day, SOFA (81.6%) were the most precise measures.Conclusion: All four systems have acceptable discriminatory power for poisoned patients. However, it seems that APACHE II can be used for mortality prediction, especially in the early days of admission.

Wolfram syndrome: A case report

Wolfram syndrome (WFS) is a rare disease inherited as an autosomal dominant trait. Type I diabetes mellitus and optic atrophy are the main symptoms of the disease. It is also known as DIDMOAD syndrome due to the association of diabetes insipidus, diabetes mellitus, optic atrophy, and deafness. WFS may be associated with other disorders such as kidney failure, gonadal atrophy, and mental and behavioral disorders. This report is about a 14-year-old teenager who had suffered from vision loss and cataracts when he was 4 years old. At the age of 7 he has been diagnosed with type I diabetes mellitus due to polyuria and polydipsia. At the age of 12 he developed diabetes insipidus, neural hearing loss, urinary incontinence and bilateral hydronephrosis, neurogenic bladder, and increased blood pressure. Physicians should think of this disease and recommend genetic counselling before marriage

Larvicidal activity of essential oil and methanol extract of Nepeta menthoides against malaria vector Anopheles stephensi

AbstractObjectiveTo investigate the larvicidal activity of essential oil and methanol extract of the Nepeta menthoides (N. menthoides) against main malaria vector, Anopheles stephensi (An. stephensi).MethodsThe essential oil of plant was obtained by Clevenger type apparatus and the methanol extract was supplied with Percolation method. Larvicidal activity was tested by WHO method. Twenty five fourth-instar larvae of An. stephensi were used in the larvicidal assay and four replicates were tested for each concentration. Five different concentrations of the oil and extract were tested for calculation of LC50 and LC90 values.ResultsThe LC50 and LC90 values were determined by probit analysis. LC50 was 69.5 and 234.3 ppm and LC90 was 175.5 and 419.9 ppm for the extract and essential oil respectively.ConclusionsAccording to the results of this study methanolic extract of plant exhibited more larvicidal activity than essential oil. This could be useful for investigation of new natural larvicidal compounds

Prognosis of patients with severe burns based on plasma base excess and serum lactate level and comparison with ABSI

Background and Aim: This study was aimed to evaluate the prognosis of patients with severe burns by measuring the plasma base excess and serum lactate levels and comparing it with the ABSI scoring system as an important indicator in the resuscitation of burn patients.Methods: This prospective cohort study was performed on all patients hospitalized in the burn ward of the hospital. The demographic data and ABSI scoring of all patients were recorded. Patients' clinical information and routine tests, gasometry (for measuring plasma base excess) and serum lactate levels were measured at the time of admission, 12 hours, and 24 hours after admission.Results: A total of 311 burning patients were evaluated in this study. Mann-Whitney test showed a significant difference in the mean plasma BE1 (at the time of admission) and BE2 (24 hours after admission) between the patients who were discharged and the patients who died (P <0.001). The results indicated a difference in the process of lactate changes between two groups of patients in terms of intensity of ABSI(P <0.001). The multivariate logistic regression with the entry of BE and lactate at the time of admission and ABSI as predictive variables indicated a significant BE and ABSI.Conclusion: The results of this study confirmed that the plasma base excess can be used as a valuable tool in the monitoring of burn patients' resuscitation along with clinical criteria. On the other hand, the ABSI scoring system is still a valuable tool to predict the deaths of burn patients

Five years of a single burn center experience with toxic epidermal necrolysis: retrospective study of causative drugs and the clinical outcome

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, but potentially life-threatening reactions to medications. Both conditions have significant morbidity and mortality. This study aimed to document the epidemiological features, aetiologies, treatment and clinical outcomes of such patients.Method: In this retrospective cross-sectional study the records of all patients with TEN treated for5 years in central Hospital, Mashhad, Iran were reviewed. Results: Thirty-four patients were studied with a mean age of 26.5 years. Mean age in the mortality and survivors groups was 33.6 and 25.3 years, respectively. Drugs accounted for all 34 cases were including Anti-convulsants (52.9%) other the most common implicated drug followed by antibiotics (26.5%), allopurinol (5.9%) and multiple drugs (anticonvulsants plus antibiotics) (14.7%). Antibiotics had the shortest interval between ingestion time and onset of symptoms. The mean ICU length of stay was 12.7 days, with a range of 1 to 30 days. The mean of SCORTEN was 2.3; it was 3.3 and 2.1 in the mortality and survivors group, respectively (P=0.001).All 34 TEN cases were given intravenous immunoglobulins (IVIG). Six patients with TEN died (17.6%). The highest mortality was found in the allopurinol group with 50%, whereas anticonvulsants and antibiotics had a mortality rate of 16.6% and 15.3%, respectively.Conclusion: Anti-convulsants especially Lamotrigine were the most frequently implicated drug, followed by antibiotics and allopurinol. IVIG was shown beneficial effects in TEN syndrome

Nutritional requirements and actual dietary intake of adult burn patients

Background: Nutritional support of the burn patient is essential to optimize, host immune defenses and to promote prompt wound healing. Furthermore, the increased needs in calorie requirement of burned patients, the composition of proteins, carbohydrates and fats in their diet is important. The purpose of this study was to evaluate energy, macronutrient and micronutrient intake and comparing with Reference Daily Intake (RDI) in adult burn patients. Methods: Sixty thermally injured patients who were hospitalized in a burn care Imam Reza hospital in Mashhad, Iran, were included in this cross-sectional study. Information about actual intake was collected by ‘nutrient intake analysis (NIA) through direct observation. Individual nutritional intakes were assessed with the use of nutritionist 4 software and Data was analyzed by SPSS version 18. Results: The mean of energy, carbohydrate and protein intake was significantly lower than the mean total energy requirement and carbohydrate and protein RDA (

Dysfunctional lens syndrome

Purpose: To report the pre- and post-operative findings of a case with dysfunctional lens syndrome. Methods: An adult patient was evaluated using iTrace aberrometer, Tomey topographer and slitlamp biomicroscopy to confirm dysfunctional lens syndrome. Results: A 45-year-old male patient presented with the chief complaint of poor visual quality; uncorrected visual acuity 20/40 in the right eye, best spectacle corrected visual acuity 20/25 in the right eye with refraction Plano/−1.50 × 80 (SE = −0.75D). Pre- and post-operative root-mean-square (RMS) of total higherorder aberrations in the entire eye, the internal optics and the cornea were 0.350 & 0.257, 0.311 & 0.236 and 0.214 & 0.191 micron (μ), respectively. Also, the magnitude of preoperative total, internal and corneal coma was 0.254 μ × 222°, 0.274μ×242° and 0.097μ × 131° and postoperative values were 0.170 μ × 162°, 0.131 μ × 177°, 0.054 μ × 125°, respectively. Conclusion: These results show that sometime the HOAs of the internal optics, mainly crystalline lens, are not compensated by the cornea and this may cause visual discomfort in the absence of any significant cataract, this situation is known as dysfunctional lens syndrome (DLS)