Long-Lasting Follow-Up with Low-Dose Steroid in an 18-Year-Old Male with Rosai-Dorfman Disease.

Rosai-Dorfman disease (RDD) is a rare and benign pathology of sinus histiocytosis of unknown etiology. Lymphadenopathy is the predominant clinical manifestation, but diverse organs can also be affected. Histological features involve S-100+ histiocytes with characteristic nuclear features within the enlarged sinusoids of the lymph nodes. The clinical course is unpredictable, but is often benign with spontaneous resolution of disease in most patients. We report a patient with bilateral massive enlargement of cervical, axillary, and inguinal lymph nodes, moderately enlarged spleen, and a weight loss of 15 kg. Excisional biopsy from the cervical lymph node showed that the dilated sinusoids were infiltrated by lymphocytes, plasma cells, and large histiocytes with CD 68 and S-100 protein positive. Due to the slow progression of the disease, oral prednisolone with a body weight of 1 mg/kg was started in March 2016. The steroid dosage has been adjusted many times during the clinical follow-up. After 33 months, steroid treatment resulted in partial shrinkage of lymph nodes, the spleen returned to its normal size, and the patient gained weight. After 38 months of follow-up, no systemic symptoms, sign, or extranodal involvement were detected, and the patient continued with low-dose steroid treatment.

Evaluation of Iron Overload in the Heart and Liver Tissue by Magnetic Resonance Imaging and its Relation to Serum Ferritin and Hepcidin Concentrations in Patients with Thalassemia Syndromes.

Iron overload is one of the major prognostic factor in thallassemia patients. We aimed to evaluate iron accumulation in the heart and liver by MRI in thalassemia major, thalassemia intermedia, and S-ß thalassemia patients and to examine its association with ferritin and hepcidin levels. Serum ferritin and hepcidin levels were recorded. Iron overload (IOL) in the heart and liver parenchyma was determined based on the standardized T2* and R2 values measured by MRI. The results were evaluated considering the tissue iron overload, serum ferritin and hepcidin levels. Comparing the 109 patients with the 30 healthy controls revealed the mean age: 24.4 ± 11 versus 31.2 ± 5 years, median levels of serum ferritin: 1693 versus 40 ng/mL, and hepcidin: 1.94 versus 0.355 ng/mL; p < 0.001, respectively. Comparison of age, serum ferritin and hepcidin levels and MRI findings of the patients with or without IOL revealed that, ferritin and T2* were significantly different in the patients with IOL in cardiac tissue (p = 0.004 and p < 0.001), and, age, ferritin and R2 were significantly different in the patients with IOL in liver tissue (p = 0.036, p < 0.001 and p < 0.001). The MRI-based T2* and R2 values were moderately and inversely correlated with serum ferritin (r = -0.37; p < 0.001 and r = -0.46; p < 0.001). No correlations were found between the MRI-based T2*, R2 values and serum hepcidin. A moderate and positive correlation existed between serum ferritin and hepcidin (r = 0.45; p < 0.001). We considered that, enhanced intestinal iron absorption characterized by decreased serum hepcidin levels in the intervals between successive transfusions were resulted in iron accumulation in our patients.

Prevalence of the metabolic syndrome in rheumatoid arthritis.

OBJECTIVE: Patients with rheumatoid arthritis (RA) experience a markedly increased prevalence of cardiovascular disease (CVD), but the causal factors have yet to be completely elucidated. Metabolic syndrome (MetS) is a cluster of risk factors of CVD and identifies additional cardiovascular risk beyond the sum of its individual components. In this study, we investigated the prevalence of MetS and its possible relationship with disease-related factors in patients with RA. MATERIAL AND METHODS: Fifty-two patients with RA and 30 age- and sex-matched healthy controls were studied. Adult Treatment Panel III of the National Cholesterol Education Program (NCEP-ATP III) and modified World Health Organization (WHO) criteria were used to define MetS. RA disease activity is assessed by the disease activity score of 28 joints (DAS28), and the functional status of patients was evaluated by Health Assessment Questionnaire (HAQ). RESULTS: Although there was no difference between groups regarding the frequency of MetS according to NCEP-ATP III criteria (17.3% and 6.5% in RA and control groups, respectively (p=0.158)) if modified WHO criteria were used, the prevalence of MetS was significantly higher in patients with RA (28.8%) than in controls (9.7%) (p=0.04). Central obesity and hypertension were found to be more frequent in patients with RA by both NCEP-ATP III and WHO criteria. RA patients with MetS had higher systolic and diastolic blood pressure, BMI and frequency of smoking than patients without MetS. Disease-related factors were similar in RA patients with or without MetS. CONCLUSION: The evaluation of patients with RA for MetS, which is a multidimensional risk factor of CVD, may be beneficial.

A rare case of systemic lupus erythematosus with chylous ascites and chylothorax.

During the course of the disease a patient with systemic lupus erythematosus (SLE) may develop inflammation of one or more serous membranes, resulting in pleural, peritoneal, or pericardial effusion. Chylous ascites and chylothorax have rarely been described in patients with SLE. Therefore, in parallel with the analysis of blood samples, detailed analysis of the effusions should be carried out. Supportive measures are often needed to relieve the symptoms of chylothorax or chylous ascites together with the treatment of the primary disease. The available literature had reported just 4 cases of chylous ascites and/or chylothorax in association with SLE, and this patient presented here is one of the rare cases apart from the reported ones.

The capillaroscopic findings in idiopathic pernio: is it a microvascular disease?

OBJECTIVES: Pernio is a disorder that affects the unprotected skin regions of individuals who are exposed to nonfreezing, damp cold. We aimed to examine nailfold capillaries by video capillaroscopy and evaluate the vascular involvement in patients with idiopathic pernio. METHODS: Fifty-three patients with idiopathic pernio (male/female ratio 35:18, mean age 25 ± 9 years) and 38 age- and sex-matched healthy volunteers (male/female ratio 30:8, mean age 24 ± 4 years) were included in the study. Forty-seven of the 53 patients and all the healthy volunteers were evaluated by nailfold video capillaroscopy. RESULTS: In the patient group, the mean capillary diameter and the mean apical capillary diameter were 56 ± 15 and 24 ± 7 µm, respectively. In the control group, the mean capillary diameter and the mean apical capillary diameter were 37 ± 8 and 15 ± 4 µm, respectively (both p < 0.001). Both of these differences were independent of the disease activity, smoking, and the number of pernio episodes. There were no architectural derangements, avascular areas, or hemorrhages. CONCLUSIONS: In the present study, increased nailfold capillary diameter and increased apical capillary diameter were found in patients with pernio regardless of the disease activity. These findings suggest organic damage of the microcirculation.

Evaluation of transient hyperglycemia in non-diabetic patients with febrile neutropenia.

BACKGROUND: We aimed to examine the effect of transient hyperglycemia in non-diabetic patients with febrile neutropenia. METHODS: A total of 86 patients with febrile neutropenia were evaluated between June 2006 and December 2009. After measuring random blood glucose level at admission, cases with stress hyperglycemia were included in the study. Stress hyperglycemia was defined as documented random blood glucose level of 140 mg/dl and above without known diabetes mellitus, impaired glucose tolerance and impaired fasting glucose. A Multinational Association for Supportive Care in Cancer (MASCC) scoring system was used for the prediction of low and high risk patients according to medical complications at the onset of the febrile episode. RESULTS: There were more patients with stress hyperglycemia than the patients with normoglycemia in the high risk group (p = 0.001). The growth of gram negative bacteria and fungi was higher in patients with stress hyperglycemia than with normoglycemia (p = 0.001). The patients receiving antifungal therapy had a higher rate of stress hyperglycemia than the patients without receiving antifungal therapy (p = 0.009). The patients with stress hyperglycemia had higher mortality rates than the patients with normoglycemia (p = 0.007). According to the MASCC risk-index, stress hyperglycemia increased 3.35 fold in the high risk patients compared to the low risk patients (p = 0.046) and 4.14 fold in the patients treated with antibacterial and antifungal agents compared to the patients treated with only antibacterial agents (p = 0.038). CONCLUSION: Patients with stress hyperglycemia had more adverse clinical outcomes than patients with normoglycemia. We think further studies are needed to evaluate the relationship between stress hyperglycemia and febrile neutropenia.

Monoballism associated with newly onset ketotic hyperglycemia.

Movement disorders as the initial symptoms of diabetes mellitus are rare. Here, we describe one of these rare manifestations of primary diabetes: a case of newly diagnosed diabetes mellitus in an old-age female patient with transient monoballismus during an episode of ketotic hyperglycemia.

C-reactive protein in unstable angina pectoris and its relation to coronary angiographic severity and diffusion scores of coronary lesions.

OBJECTIVE: We aimed to assess the relationship between C-reactive protein (CRP) and the severity and diffusion of coronary artery lesions in patients with unstable angina pectoris (UAP) and the independent association of CRP with this clinical situation. METHODS: This cross-sectional, observational study included 50 patients. Classification by Braunwald was used for UAP. The severity and diffusion of angiographic coronary disease were graded according to Reardon's modified scoring system. Plasma CRP levels were quantified by immunoturbidimetry. Nonparametric tests were used for comparison of CRP and other risk factors, and logistic regression analysis for evaluation of independent association between CRP and unstable angina pectoris. RESULTS: The severity score was 46±18 points in class IIB1 UAP, 36±20 points in class IIB2 and 53±18 points in class IIIB2 (p=0.017, class IIIB2 vs IIB2). Respectively, CRP levels were 6.6 mg/L, 3.8 mg/L and 4.8 mg/L (p=0.371, class IIB1 vs IIB2 vs IIIB2). Lesions with diffusion score 4 revealed higher CRP values than lesions with diffusion score 1 (11.1 mg/L vs 3.1 mg/L, p=0.048). Adjusting age, sex and smoking, assessment of partial correlation analysis showed a positive, moderately powerful and significant association between CRP levels and the severity and diffusion scores of the coronary lesions (r=0.30; p=0.034 and r=0.31; p=0.030, respectively) in the whole study group. Multiple logistic regression analysis showed no appreciable independent association between CRP and UAP (OR: 1.63, 95%CI: 0.90-5.63, p=0.093). CONCLUSION: Although, CRP was correlated with the severity and diffusion of angiographic coronary disease in patients with UAP, there was no independent association between CRP and clinical severity of UAP.

Management of health-care waste in Izmir, Turkey.

The aim of this study was to evaluate health-care waste in the 18 districts of metropolitan municipality of the third biggest city in Turkey. This cross-sectional study was carried out with 825 health institutions established in the 18 districts of Izmir metropolitan municipality, in 2007. The total amount of health-care waste collected was 4841 tons and 621 kilograms per patient's bed in 2007. Most of the medical wastes were collected from Konak, Karsiyaka and Bornova districts and were 2308, 272 and 1020 tons, respectively. Regarding to overpopulation, the number of health institutions in these districts are more than the number of health institutions in the other administrative districts. There was a statistically significant, positive correlation between the amount of health-care waste collected and population of the 18 districts (r = 0.79, p < 0.001), and number of beds/patients (r = 0.83, p < 0.001). To provide a safe health-care waste management metropolitan municipality must provide hazardous waste separation in health institutions, establish sterilization units for infectious waste, and provide the last storage of medical waste in completely different, safe and special areas apart from the municipal waste storage areas.

Adrenocortical oncocytic neoplasm presenting with Cushing's syndrome: a case report.

INTRODUCTION: Oncocytic neoplasms occur in several organs and are most commonly found in the thyroid, kidneys and salivary glands. Oncocytic neoplasms of the adrenal cortex are extremely rare and are usually non-functioning. CASE PRESENTATION: We report the case of an adrenocortical oncocytic neoplasm with uncertain malignant potential in a 31-year-old man with Cushing's syndrome. The patient had been operated on following diagnosis of a 7 cm adrenal mass. Following surgery, the Cushing's syndrome resolved. The patient is still alive with no metastases one year after the surgery. CONCLUSION: Adrenocortical oncocytic neoplasms must be considered in the differential diagnosis of both functioning and non-functioning adrenal masses.

Coagulation disorders secondary to acute liver failure in Amanita phalloides poisoning: a case report.

Amanita mushroom poisoning is a serious occurrence. Physicians need to recognize the syndrome promptly and institute effective treatment as soon as possible in order to avoid the often fatal complications. We report a case of a young man with mushroom poisoning of the genus Amanita with a clinical spectrum that ranged from hepatic failure and coagulation disorders to an outcome with a partial recovery. The aim of this report was to emphasize the clinical outcomes and treatment regimens in this type of mushroom poisoning seen with increasing frequency in our region.

The prevalence of metabolic syndrome among young adults in Izmir, Turkey.

OBJECTIVE: The aim of this study was to determine the prevalence of metabolic syndrome and its components among the young adult ages in Izmir, Turkey. METHODS: A population-based cross-sectional study was held including 885 subjects aged between 20 to 39 years from 45 primary health care centres in Konak, which is the biggest urban area in Izmir. A stratified sampling method was used to select the participants of 318 males and 567 females. In the study, metabolic syndrome was classified according to NCEP ATP III criterion on the basis of metabolic risk factors evaluated between December 2001 and April 2002. RESULTS: The crude prevalence of metabolic syndrome (having three or more of the metabolic risk factors) was 3.6% among 20 and 29 years old men and 19.6% among 30 and 39 years old men and the increase by age was significant (p<0.001). The results were similar in women and the crude prevalence of metabolic syndrome increased significantly from 7.5 % in 20 and 29 years old women to 24 % in 30 and 39 years old women (p<0.001). With regard to the highest prevalences of the first three metabolic risk factors, hypertriglyceridemia, low HDL-cholesterol and high blood pressure, the age-adjusted prevalence of the metabolic syndrome was significantly higher in women than in men and all subjects (15% vs 10.2% vs 13.6%, p<0.01) respectively. CONCLUSION: This study revealed that the prevalence of metabolic syndrome was extremely high between young adults in urban areas and the most prevalent components of the metabolic syndrome were found to be high triglycerides, low HDL cholesterol, high blood pressure, abdominal obesity and high fasting glucose, respectively.

The association of plasma homocysteine, coronary risk factors and serum nitrite in patients with coronary artery disease, vascular syndrome x and healthy subjects.

OBJECTIVE: We evaluated the association of plasma total homocysteine (tHcy), cardiac risk factors and total nitrite in coronary artery disease (CAD) patients, cardiac syndrome X patients and in healthy subjects. METHODS: Forty two CAD, 22 cardiac syndrome X patients and 30 healthy subjects, aged 30 to 75 years were included into the study. Blood samples of tHcy, serum total nitrite and cardiac risk factors were studied appropriately. The results were compared between the groups. The independent contributions of tHcy and total nitrite to CAD and cardiac syndrome X and their interactions with cardiac risk factors were evaluated. RESULTS: After adjusting for age, median values of tHcy and total nitrite were evaluated for their skewness. Coronary artery disease patients had higher median plasma tHcy levels than cardiac syndrome X patients (p<0.001) and healthy subjects (p<0.001) and lower serum total nitrite levels than patients in the two other groups (p<0.05), respectively. Using a univariate linear regression analysis tHcy had a moderately significant positive correlation with age (beta=0.34, p=0.002) and a weakly significant inverse correlation with female gender (beta=-0.24, p=0.032). Using a partial correlation analysis by controlling for age, gender and clinical situations tHcy had a positive but moderately significant correlation with LDL cholesterol (r=0.23, p=0.01) and triglycerides (r=0.27, p=0.016). Total nitrite had a positive but weakly significant correlation with HDL cholesterol (r=0.23, p=0.04) and fibrinogen (r=0.24, p=0.03) and an inverse but moderately significant correlation with LDL cholesterol (r=-0.37,p=0.001). Using a multivariate stepwise regression analysis total nitrite was inversely and significantly associated with tHcy (beta=-0.45) in the control group. The contribution of HDL cholesterol to the association was beta=-0.45, p=0.044, R2=36.2%, HDL cholesterol with fibrinogen--beta=-0.45, p=0.05, R2=36.6% and HDL cholesterol with LDL cholesterol--beta=-0.45, p=0.05, R2=36.3%. In a forward stepwise logistic regression analysis the age adjusted odds ratio (OR) for coronary artery disease per standard deviation change in log-transformed tHcy concentration was - 0.82, p=0.013 and in total nitrite concentration was - 1.08, p=0.02. Using the same model neither tHcy nor total nitrite was associated with cardiac syndrome X (p=0.221 and p=0.112), respectively. CONCLUSION: The low nitrite levels can be a marker of endothelial dysfunction in the presence of hyperhomocysteinemia and other cardiac risk factors. Our results might support endothelial dysfunction in CAD but not in cardiac syndrome X patients.