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Sjögren-Larsson syndrome (SLS) is a rare neurocutaneous disease showing an autosomal recessive transmission due to a lack of fatty acid aldehyde dehydrogenase. Spastic diplegia or triplegia, mental retardation and congenital lamellar ichthyosis are the major findings of the disease. The syndrome may be accompanied by various eye and teeth features, skeletal system anomaly, speaking defects, hypertelorism and epilepsy. A 9-month male patient has been hospitalized for convulsion and flaking on body. The patient history showed that flaking skin thickening and peeling was started at the birth, and he suffered a right-side focused seizure when he was three month-old and he was treated with phenobarbital and carbamazepine upon the epilepsy diagnosis. Wide ichthyosis, hypertelorism and bilateral simian line were observed in the physical examination. Bilateral punctuate lesions in cornea, pigment epithelial atrophy in the right eye and esotropia in the left eye have been determined during the eye examination. An epiteliform anomaly has been observed in the left hemisphere by electroencephalography. In brain magnetic resonance imaging (MRI), an increase in cerebral-cerebellar brain parenchyma and T1-T2 relaxation time and in the signal in corpus callosum (delayed myelination) have been determined. With the observation of the white matter in centrum semi oval using brain MRI spectroscopy, signs of a sphingolipid peak at 1.3 ppm have been observed. An SLS diagnosis has been proposed upon clinical and laboratory observations. We want to emphasize on the fact that in epilepsy cases with ichthyosis, SLS should be considered. [ABSTRACT FROM AUTHOR]

Although influenza is an acute and uncomplicated disease, that limits itself in the healthy children, it may lead to death by rarely forming the sickness. The most common complication of influenza is pneumonia and it is a rare complication which is developed together with myocarditis by influenza A and B viruses. A 32 months-old male patient was admitted for rapidly developed respiratory distress and tachycardia after fever, cough, vomiting, malaise and runny nose. His general status was medium, he had conscious and had hepatomegaly, tachycardia, dyspnea, tachypnea, intercostal-subcostal retractions and bilateral rhonchus. Cardiac enzyme levels and other laboratory parameters were found normal. Myocarditis and ejection fraction was determined as 42% in echocardiography. However, hospitalization hours between 24 and 48, the patient, whose significant respiratory compromise developed, was intubated and fastened to a mechanical ventilator. H1N1 is produce in nasopharyngeal swab culture at the sixth day of follow-up. Because we think H1N1 virus was responsible from current myocarditis, oseltamivir treatment was initiated. In the fourth day of the treatment the patient's fever returned to normal, in the ninth day a dramatic recovery was observed. In tracking echocardiography, a significant improvement was observed in the ejection fraction and myocarditis picture compared with admission time. This case was presented in order to remind that in a patients, who present with influenza findings but have respiratory distress and tachycardia in addition to lower respiratory tract infection, myocarditis should also be considered in the differential diagnosis and to remind that promising results could be obtained with the early diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Objective: The World Health Organization labelled the COVID-19 outbreak a pandemic on March 11, 2020, the first day COVID-19 cases were diagnosed in Turkey. Since then, people's main concerns have been whether their lives or the lives of their relatives would be at risk if they became infected. The objective of this study was to determine the risk factors that increase mortality in COVID-19 patients treated in ICUs and to facilitate the appropriate precautions. Methods: In this study, patients who were diagnosed with COVID-19 [positive on a polymerase chain reaction (PCR) test] and monitored in the Pandemic Hospital at Diyarbakır Dicle University between 15 March 15 and 15 May, 2020, were evaluated for risk factors for mortality. Results: A total of 50 ICU patients were included in the study. Of them, 52% were males, and the mean age was 63.8±17.6 years. A comparison of deceased and surviving patients showed that being female, older than 62, and a smoker and having diabetes mellitus, hypertension, and/or coronary artery disease significantly increased mortality. An investigation of the findings from the patients' lung tomography examinations revealed that relatively prominent pulmonary involvement and localization did not affect mortality. Conclusions: Because patients who are advanced in age, female and smokers and have comorbidities are in the high-risk group, measurements related to treating the COVID-19 disease should be improved. Laboratory parameters are useful for the evaluation of mortality and morbidity, while mechanical ventilation increased mortality. [ABSTRACT FROM AUTHOR]

Objective: According to 2017 records 17.000 people are infected with HIV (human immunodeficiency virus) in Turkey. Drug compliance is one of the most important points in the treatment of the disease. Patient training is very important in HIV / AIDS treatment. This study aimed to evaluate drug compliance in a patient group undergoing training intervention. Methods: The study was conducted as a retrospective analysis of an intervention. Totally, 107 HIV / AIDS patients followed up at the Infectious Diseases and Clinical Microbiology Department of Dicle University Medical Faculty between May-July 2017 were included in the study. Patients who were evaluated as Group 1 received personalized detailed training, the other 25 patients (Group 2) took standard briefing. Patients' drug compliances evaluated by tablet counting method at third and sixth months of treatment. Results: The mean age of 107 patients trained was 28.6 years. Eighty-six (80.4%) of the patients were male. The educational status of the patients, 8,4% (n = 9) were literate, 67,3% (n = 72) studied primary and secondary education, no statistically significant difference was found between the educational status of the patients and drug compliance. (p = 0,159 and p = 0,274, respectively). Regardless of the number of tablets per day, drug compliance was found to be statistically significantly higher in Group 1. (p <0,001) Discussion and Conclusion: In patients with HIV, providing disease-specific training significantly increases drug compliance regardless of the number of tablets and education level. Using of regimens containing fewer tablets increases drug compliance. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to evaluate the relationship between thyroid functions and metabolic and anthropometric parameters in obese children and adolescents. Methods: The study included 147 patients diagnosed with exogenous obesity in the pediatric endocrinology outpatient clinic and 46 healthy subjects with similar age and sex. Obese patients were divided into two groups as Group 1 (non-fatty liver) and Group 2 (with fatty liver). Fasting glucose, insulin, total cholesterol, triglyceride, aspartate aminotransferase, alanine aminotransferase, thyroid stimulating hormone (TSH), free triiodothyronine (fT3) and free thyroxine (fT4) samples were performed from all cases in patient groups and control group (Group 3). Insulin resistance was assessed by HOMA-IR (Homeostatic Model Assessment of Insulin Resistance). Results: In Group 2, waist circumference (WC), body mass index-standard deviation scores (SDS), hip circumference (HC), WC/HC ratio and triglyceride, fasting insulin and HOMA-IR values were significantly higher than Group 1 and Group 3 while high density lipoprotein cholesterol level was lower. In Group 1 and Group 2, fT4 level was significantly lower than Group 3, whereas fT3 /fT4 ratio was higher. The mean TSH level was higher in Group 2 than Group 3. fT3 / fT4 ratio; WC, WC / HC ratio was positively correlated with fasting insulin and HOMA-IR. Conclusion: We found that fT4 level was low and fT3 / fT4 ratio was higher in obese children. Considering the high fT3 / fT4 ratio in obese children, this ratio should be stimulating for the clinician regarding the complications associated with visceral adipose tissue as it is positively correlated with WC and insulin resistance. [ABSTRACT FROM AUTHOR]

Although fibroma is the second most common benign cardiac tumor following rabdomyoma, its incidence is quite low. Fibromas causing ventricular outflow tract obstruction, arrhythmia, and/or severe valvular dysfunction necessitate surgical resection. An infant with asymptomatic clinical course, despite having a giant right ventricular fibroma was reported herein. [ABSTRACT FROM AUTHOR]