L -2-Hydroxyglutaric aciduria (L -2-OHGA) is a rare autosomal recessive inherited encephalopathy. This inborn error, characterized by psychomotor retardation, progressive ataxia and typical magnetic resonance imaging findings, presents in early infancy. To make a definitive diagnosis, an anomalous accumulation of L -2-hydroxyglutaric acid must be detected in body fluids. Here, we present a 17-year-old boy with L: -2-OHGA who developed an anaplastic ependymoma during the course of this disease. We also present a literature review including seven other patients who developed malignant brain tumors during the course of L -2-OHGA. This correlation may indicate a possible increased risk of brain tumors among patients with L -2-hydroxyglutaric aciduria.
Necrotizing fasciitis is a rapidly progressive infection and is a necrosis of the fascia and surrounding tissues. Despite recent advances in its management, outcomes have not improved and mortality rate is still high. Between September 2007 and August 2009, we prospectively studied twenty-four histopathologically proven necrotizing fasciitis patients to assess the prognostic factors that indicate the outcome. Mortality rate was 20.8%. Twelve patients (50%) improved, while seven patients (29.2%) were complicated by limb loss. Mortality rates related to upper and lower limb involvement were similar (20% vs. 22.2%). The rates of gangrene and amputation in patients with diabetes mellitus were significantly higher than other comorbidities. Patients with gram-positive infections had significantly lower rates of amputation (15.4% vs. 54.5%, P = 0.04). Mean band cell count and serum potassium level were significantly higher in the nonsurvivors same as leukocyte count in the patients with gangrene, while serum sodium level was significantly lower in nonsurvivors. We conclude that hyponatremia, hyperkalemia, and increased band cells in the peripheral blood of patients may be useful parameters in distinguishing life-threatening necrotizing fasciitis; hence, we recommended lower threshold to amputation during surgery for this group of patients.
Background: Aeroallergens and food allergens are found to be relevant in atopic dermatitis. The atopy patch test (APT) can help to detect food allergies in children with atopic dermatitis. This study evaluates if the APT is a valuable tool in the diagnostic workup of children with food allergy-related atopic dermatitis. Methods: 42 children between 6 months and 12 years of age were selected at the Mofid Children Hospital. Atopic dermatitis was diagnosed, and the severity of the disease was determined. At the test visit, the patients underwent a skin prick test (SPT), APT, and serum IgE level measurement for cow's milk, egg yolk, egg white, wheat, and soy. Results: We found a sensitivity of 91.7%, a specificity of 72.7%, a positive predictive value (PPV) of 88%, a negative predictive value (NPV) of 80%, and an accuracy of 85.7% for APT performed for cow's milk. APT performed for egg yolk had a sensitivity and a NPV of 100%, while the same parameters obtained with egg white were 84.2 and 75%, respectively. The sensitivity, specificity, and NPV of the APT for wheat were 100, 75, and 100%, respectively. The sensitivity, PPV, and NPV of the APT for soy were 87.5, 70, and 87.5%, respectively. Conclusions: Our data demonstrate that the APT is a reliable diagnostic tool to evaluate suspected food allergy-related skin symptoms in childhood and infancy.
Background: Hypertension is one of the most common disorders and is a major public health problem that causes 20% to 50% of world mortality. On the other hand, obesity is also a risk factor for type 2 diabetes, cancer, cardiovascular disease, osteoarthritis and has a major share in health costs. Therefore, the relationship between body mass index and blood pressure has been considered. By this study, in general, about these indicators in the age group of under 30 years, useful information can be found. Methods: In order to collect information, a questionnaire was prepared and a random sampling was completed for a number of students from Shahrekord University of Medical Sciences. The data were analyzed using spss version 22. Results: By analyzing the results, it was found that systolic and diastolic blood pressures of both hands had a direct and significant relationship with age, so that by increasing the age, there is an increasing in hypertension (p≤0.001). Systolic blood pressure also showed a significant relationship with gender, and it was found that in men it is more than women. Finally, in examining the relationship between BMI and blood pressure, the results show that there is a positive and significant relationship between these two parameters, so that the increase in BMI also increases the systolic and diastolic blood pressure (p = 0.0001). Conclusion: Blood pressure in students has a direct and significant correlation with age, sex, and body mass index.
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