Epidemiology of pemphigus in Turkey: One-year prospective study of 220 cases

Pemphigus is a group of rare and life-threatening autoimmune blistering diseases of the skin and mucous membranes. Although they occur worldwide, their incidence shows wide geographical variation, and prospective data on the epidemiology of pemphigus are very limited. Objective of this work is to evaluate the incidence and epidemiological and clinical features of patients with pemphigus in Turkey. All patients newly diagnosed with pemphigus between June 2013 and June 2014 were prospectively enrolled in 33 dermatology departments in 20 different provinces from all seven regions of Turkey. Disease parameters including demography and clinical findings were recorded. A total of 220 patients were diagnosed with pemphigus during the 1-year period, with an annual incidence of 4.7 per million people in Turkey. Patients were predominantly women, with a male to female ratio of 1:1.41. The mean age at onset was 48.9 years. Pemphigus vulgaris (PV) was the commonest clinical subtype (n=192; 87.3%), followed by pemphigus foliaceus (n=21; 9.6%). The most common clinical subtype of PV was the mucocutaneous type (n=83; 43.2%). The mean Pemphigus Disease Area Index was 28.14±22.21 (mean ± Standard Deviation).  The incidence rate of pemphigus in Turkey is similar to the countries of South-East Europe, higher than those reported for the Central and Northern European countries and lower than the countries around the Mediterranean Sea and Iran. Pemphigus is more frequent in middle-aged people and is more common in women. The most frequent subtype was PV, with a 9-fold higher incidence than pemphigus foliaceus.   </p

Volume CXIV, Number 4, November 7, 1996

Objective: Turner syndrome (TS) is a chromosomal disorder caused by complete or partial X chromosome monosomy that manifests various clinical features depending on the karyotype and on the genetic background of affected girls. This study aimed to systematically investigate the key clinical features of TS in relationship to karyotype in a large pediatric Turkish patient population.Methods: Our retrospective study included 842 karyotype-proven TS patients aged 0-18 years who were evaluated in 35 different centers in Turkey in the years 2013-2014.Results: The most common karyotype was 45,X (50.7%), followed by 45,X/46,XX (10.8%), 46,X,i(Xq) (10.1%) and 45,X/46,X,i(Xq) (9.5%). Mean age at diagnosis was 10.2±4.4 years. The most common presenting complaints were short stature and delayed puberty. Among patients diagnosed before age one year, the ratio of karyotype 45,X was significantly higher than that of other karyotype groups. Cardiac defects (bicuspid aortic valve, coarctation of the aorta and aortic stenosis) were the most common congenital anomalies, occurring in 25% of the TS cases. This was followed by urinary system anomalies (horseshoe kidney, double collector duct system and renal rotation) detected in 16.3%. Hashimoto's thyroiditis was found in 11.1% of patients, gastrointestinal abnormalities in 8.9%, ear nose and throat problems in 22.6%, dermatologic problems in 21.8% and osteoporosis in 15.3%. Learning difficulties and/or psychosocial problems were encountered in 39.1%. Insulin resistance and impaired fasting glucose were detected in 3.4% and 2.2%, respectively. Dyslipidemia prevalence was 11.4%.Conclusion: This comprehensive study systematically evaluated the largest group of karyotype-proven TS girls to date. The karyotype distribution, congenital anomaly and comorbidity profile closely parallel that from other countries and support the need for close medical surveillance of these complex patients throughout their lifespa

Degradation of Reactive Dyes Using Advanced Oxidation Method

WOS: 000358509300010In this study, the photo-Fenton process was used to identify degradation conditions of Reactive Blue 19 (RB19) and Reactive Red 21 (RR21). The effects of pH, initial H2O2 and FeSO4 concentrations, time, and UV light intensity in determining the degradation rate were studied. The optimal conditions for the degradation of 0.156 mmol L-1 RB19 and 0.036 mmol L-1 RR21 in water were found to be: pH 4, 0.1 mL 30% H2O2 for RB19 and RR21, 0.2 mL 0.5% FeSO4 for RB19 and 0.1 mL 0.5% FeSO4 for RR21, 20 mL volume, and a temperature of 20 degrees C. Optimal conditions were applied to synthetic dye wastewater using small amounts of H2O2 in 1 L for degradation. The degree of degradation efficiency of synthetic wastewater by the photo-Fenton process was found to be > 95% within 10 min.Council of Scientific Research Projects of Marmara University [FEN-BGS-290506-0116]This work was supported by Council of Scientific Research Projects of Marmara University, with FEN-BGS-290506-0116 project number

The 4A syndrome association with osteoporosis

WOS: 000079471800030PubMed ID: 104265924A syndrome is characterised by adrenocortical insufficiency, achalasia, alacrima, autonomic and other neurological abnormalities. We report an 18-year-old boy with 4 A syndrome and having all classical features of the disease including sensorimotor neuropathy. In addition, the patient had low aldosterone levels and signs of osteoporosis, which apparently developed without glucocorticoid replacement therapy. Although it is speculated that the lack of local growth factors, nutritional deficiency secondary to achalasia or receptor abnormalities regarding bone metabolism contribute to osteoporosis, its etiopathogenesis still needs to be clarified

Increased Oxidative DNA Damage in Lean Normoglycemic Offspring of Type 2 Diabetic Patients

karadeniz, muammer/0000-0002-3345-5437WOS: 000295612100004PubMed: 21472659Objective: Several studies have shown increased oxidative stress in patients with pre-diabetes and newly diagnosed Type 2 diabetes mellitus (T2DM). It has been proposed that oxidative stress initiates insulin resistance in genetically predisposed individuals. The aim of this study was to evaluate the markers of oxidative stress in the off spring of patients with T2DM. Material and Methods: We examined 60 lean normoglycemic off spring of Type 2 diabetics, and 52 age, sex and body mass index matched subjects without family history of T2DM as controls. Anthropometric, biochemical and carotid intima media thickness (IMT) measurements and oral glucose tolerance test (OGTT) were performed. Erythrocyte superoxide dismutase and glutathione peroxidase activities, serum nitric oxide, plasma total sulfhydryl (tSH) groups, plasma total antioxidant status, plasma malondialdehyde and serum 8-hydroxydeoxy-guanosine (8-OHdG) levels were compared between 2 groups. Results: 2 groups were similar for the measurements of anthropometric, blood pressure, lipids, fasting glucose, HOMA-IR and carotid IMT. Glucose levels during OGTT were significantly higher in the off spring of Type 2 diabetics than controls (p = 0.035). The off spring of Type 2 diabetics showed a significant increase in serum 8-OHdG level (p = 0.005) and plasma tSH groups (p = 0.032) when compared to the controls. Significant differences were not obtained in other oxidative stress marker levels between 2 groups. Conclusion: Main finding of our study was the presence of increased oxidative DNA damage in lean normoglycemic off spring of Type 2 diabetic patients. There is a need for further clinical studies in order to explain whether oxidative stress is present in genetically predisposed subjects and induces the insulin resistance

Increased Oxidative DNA Damage in Lean Normoglycemic Offspring of Type 2 Diabetic Patients

WOS: 000295612100004PubMed ID: 21472659Objective: Several studies have shown increased oxidative stress in patients with pre-diabetes and newly diagnosed Type 2 diabetes mellitus (T2DM). It has been proposed that oxidative stress initiates insulin resistance in genetically predisposed individuals. The aim of this study was to evaluate the markers of oxidative stress in the off spring of patients with T2DM. Material and Methods: We examined 60 lean normoglycemic off spring of Type 2 diabetics, and 52 age, sex and body mass index matched subjects without family history of T2DM as controls. Anthropometric, biochemical and carotid intima media thickness (IMT) measurements and oral glucose tolerance test (OGTT) were performed. Erythrocyte superoxide dismutase and glutathione peroxidase activities, serum nitric oxide, plasma total sulfhydryl (tSH) groups, plasma total antioxidant status, plasma malondialdehyde and serum 8-hydroxydeoxy-guanosine (8-OHdG) levels were compared between 2 groups. Results: 2 groups were similar for the measurements of anthropometric, blood pressure, lipids, fasting glucose, HOMA-IR and carotid IMT. Glucose levels during OGTT were significantly higher in the off spring of Type 2 diabetics than controls (p = 0.035). The off spring of Type 2 diabetics showed a significant increase in serum 8-OHdG level (p = 0.005) and plasma tSH groups (p = 0.032) when compared to the controls. Significant differences were not obtained in other oxidative stress marker levels between 2 groups. Conclusion: Main finding of our study was the presence of increased oxidative DNA damage in lean normoglycemic off spring of Type 2 diabetic patients. There is a need for further clinical studies in order to explain whether oxidative stress is present in genetically predisposed subjects and induces the insulin resistance

Radiologic features of COPD exacerbations: quantitative analysis of thorax computerised tomography

AbstractBackground-aim:&nbsp;Acute exacerbation of chronic obstructive pulmonary disease (AECOPD) has a negative impact on the prognosis of the disease. Radiological findings might be a helpful assessment tool to predict future exacerbations. Here we identified quantitative computerized tomography (CT) findings in relation to AECOPD.Methods:&nbsp;Thorax CT features of COPD patients who applied to our clinic during the last 5 years were retrospectively evaluated and analyzed based on voxel attenuation. Subjects were divided into two groups according to the presence of acute severe exacerbation during the last 12 months after the date of the CT scan. Thus, groups are named GOLD A-B and GOLD E&nbsp;according to&nbsp;GOLD 2023. Quantitative voxel-based attenuation analysis was done by Myrian® Expert software (Intrasense SA-1231, Montpellier, France). Emphysema was defined as attenuation values&lt;-950 Hounsfield Unit (HU).Results:&nbsp;Of 106, 24 subjects had at least one severe AECOPD in the following year of CT. Emphysema percentage was significantly higher in GOLD E in the GOLD A-B group (3% vs 10%,&nbsp;p=0.001) (Table 1). However, both groups displayed similar values in lung volume and mean attenuation.Conclusion:&nbsp;Higher emphysema percentage might indicate increased AECOPD risk. Quantitative CT analysis could be a helpful assessment tool to evaluate exacerbation risk.</p

The comparison of quantitative CT features of COPD and pre-COPD: results from a real-life study

AbstractBackground:&nbsp;Although fixed airway obstruction on spirometry is crucial for COPD diagnosis, it might be insufficient to identify subjects with pre-COPD. Quantitative computerized tomography (CT) measurements might be helpful to distinguish the subjects with pre-COPD. Thus, we aimed to compare quantitative CT findings of subjects with pre-COPD and COPD.Methods:&nbsp;Subjects who were admitted to our clinic with chronic respiratory symptoms and were evaluated for COPD as a pre-diagnosis were included in the study. Subjects were categorized as COPD and pre-COPD groups according to the FEV1/FVC ratio. Thorax CT voxel-based attenuation analysis was performed by Myrian® Expert software(Intrasense SA-1231, Montpellier, France). Attenuation values lower than -950 Hounsfield Units (HU) were defined as emphysema.Results:&nbsp;Of 163, 106 COPD and 57 pre-COPD cases were included. COPD group had significantly higher lung volume (5683 ml vs 4921 ml,&nbsp;p=0.001) and lower mean attenuation value (-830.0 HU vs -808.2 HU,&nbsp;p&lt;0.001) than the pre-COPD group. Moreover, the COPD group had a higher emphysema percentage than the pre-COPD group (3.87% vs 0.28%,&nbsp;p&lt;0.001).Conclusion:&nbsp;Quantitative CT of pre-COPD demonstrated radiologic findings that might help to corroborate COPD diagnosis, which was also associated with the severity of the airflow obstruction and be an alternative method for COPD diagnosis when it is difficult to reach spirometry." data-icon-position="" data-hide-link-title="0" style="-webkit-font-smoothing: antialiased; margin: 0px; padding: 8px; border: none; outline-style: initial; outline-width: 0px; vertical-align: baseline; font-style: inherit; font-variant: inherit; font-stretch: inherit; font-size: 0.857rem; line-height: inherit; font-family: inherit; font-optical-sizing: inherit; font-kerning: inherit; font-feature-settings: inherit; font-variation-settings: inherit; color: rgb(0, 85, 148); display: block; box-shadow: rgba(0, 0, 0, 0.15) 0px 2px 10px 0px; background: rgb(255, 255, 255);">" data-icon-position="" data-hide-link-title="0" style="-webkit-font-smoothing: antialiased; margin: 0px; padding: 8px; border: none; outline-style: initial; outline-width: 0px; vertical-align: baseline; font-style: inherit; font-variant: inherit; font-stretch: inherit; font-size: 0.857rem; line-height: inherit; font-family: inherit; font-optical-sizing: inherit; font-kerning: inherit; font-feature-settings: inherit; font-variation-settings: inherit; color: rgb(0, 85, 148); display: block; box-shadow: rgba(0, 0, 0, 0.15) 0px 2px 10px 0px; background: rgb(255, 255, 255);">" data-icon-position="" data-hide-link-title="0" style="-webkit-font-smoothing: antialiased; margin: 0px; padding: 8px; border: none; outline-style: initial; outline-width: 0px; vertical-align: baseline; font-style: inherit; font-variant: inherit; font-stretch: inherit; font-size: 0.857rem; line-height: inherit; font-family: inherit; font-optical-sizing: inherit; font-kerning: inherit; font-feature-settings: inherit; font-variation-settings: inherit; color: rgb(0, 85, 148); display: block; box-shadow: rgba(0, 0, 0, 0.15) 0px 2px 10px 0px; background: rgb(255, 255, 255);"></div

A subset of patients with acquired partial lipodystrophy developing severe metabolic abnormalities

###EgeUn###Purpose/Aim of the study: Acquired partial lipodystrophy (APL) is a rare disease characterized by selective loss of adipose tissue. In this study, we aimed to present a subset of patients with APL, who developed severe metabolic abnormalities, from our national lipodystrophy registry. Materials and Methods: Severe metabolic abnormalities were defined as: poorly controlled diabetes (HbA1c above 7% despite treatment with insulin more than 1 unit/kg/day combined with oral antidiabetics), severe hypertriglyceridemia (triglycerides above 500 mg/dL despite treatment with lipid-lowering drugs), episodes of acute pancreatitis, or severe hepatic involvement (biopsy-proven non-alcoholic steatohepatitis (NASH)). Results: Among 140 patients with all forms of lipodystrophy (28 with APL), we identified 6 APL patients with severe metabolic abnormalities. The geometric mean for age was 37 years (range: 27-50 years; 4 females and 2 males). Five patients had poorly controlled diabetes despite treatment with high-dose insulin combined with oral antidiabetics. Severe hypertriglyceridemia developed in five patients, of those three experienced episodes of acute pancreatitis. Although all six patients had hepatic steatosis at various levels on imaging studies, NASH was proven in two patients on liver biopsy. Our data suggested that APL patients with severe metabolic abnormalities had a more advanced fat loss and longer disease duration. Conclusions: We suggest that these patients represent a potential subgroup of APL who may benefit from metreleptin or investigational therapies as standard treatment strategies fail to achieve a good metabolic control