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Hermansky-Pudlak syndrome is a rare disease characterized by bleeding diathesis, oculocutaneous albinism and lysosomal ceroid lipofuscin pigment deposits. Pulmonary fibrosis may also accompany with the disease. A 48-year-old male patient with a diagnosis of Hermansky-Pudlak syndrome admitted with dyspnea. A thorax computed tomography revealed bilateral diffuse interlobular septal thickness which was more prominent in the basal segments of lower lobes. Although pirfenidone therapy was planned, clinical deteroriation developed and patient died because of respiratory failure. In conclusion; this report describes a patient with pulmonary fibrosis caused by lung involvement of Hermansky-Pudlak syndrome which is an extremely rare and mortal disease.

Endobronchial metastases (EBM) from extrapulmonary malignant tumors are rare. The most common extrathoracic malignancies associated with EBM are breast, renal and colorectal carcinomas. In this study, we aimed to evaluate the clinical, radiographic and bronchoscopic aspects of patients with EBM who were diagnosed between 1992 and 2002. Data about patients' clinical conditions, symptoms, radiographic and endoscopic findings, and histopathological examination results were investigated. EBM was defined as bronchoscopically visible lesions histopathologically identical to the primary tumor in patients with extrapulmonary malignancies. We found 15 cases with EBM. Primary tumors included breast (3), colorectal (3), and renal (2) carcinomas; Malignant Melanoma (2); synovial sarcoma (1), ampulla of Vater adenocarcinoma (1), pheochromocytoma (1), hypernephroma (1), and Hodgkin's Disease (1). The most common symptoms were dyspnea (80%), cough (66.6%) and hemoptysis (33.3%). Multiple (40%) or single (13.3%) pulmonary nodules, mediastinal or hilar lymphadenopathy (40%), and effusion (40%) were the most common radiographic findings. The mean interval from initial diagnosis to diagnosis of EBM was 32.8 months (range, 0-96 months) and median survival time was 18 months (range, 4-84). As a conclusion, various extrapulmonary tumors can metastasize to the bronchus. Symptoms and radiographic findings are similar with those in primary lung cancer. Therefore, EBM should be discriminated from primary lung cancer histopathologically. Although mean survival time is usually short, long-term survivors were reported. Consequently, treatment must be planned according to the histology of the primary tumor, evidence of metastasis to other sites and medical status of the patient.

Introduction: Vascular endothelial growth factor (VEGF) and Angiopoietin-2 (Ang-2) are major angiogenic mediators in neovascularization process. In current literature both biomarkers are discussed separately and only for non-small cell lung cancer (NSCLC). So in this study we aimed to examine them together for both cell types NSCLC and small cell lung cancer (SCLC). Patients and Methods: 100 patients with lung cancer were enrolled to this single center study. 87 of patients were diagnosed with NSCLC including 28 adenocarcinomas and 59 squamous cell cancers and 13 were SCLC. Results were compared with 30 healthy volunteers. Pre-treatment serum VEGF and Ang-2 levels were measured by using ELISA method. Results: While serum Ang-2 levels were higher in patients than healthy controls (23395 pg/mL vs. 4025 pg/mL, p< 0.001), VEGF levels didn't differ (2308 pg/mL vs. 2433 pg/mL, p> 0.05). There was no difference between cases with SCLC and NSCLC in terms of Ang-2. But serum VEGF values were significantly lower in SCLC than NSCLC and control groups. None of these mediators were correlated with cell type, tumor size, TNM staging, performance status and operability. VEGF levels were higher in patients with chronic obstructive pulmonary disease (COPD), but it was not significant. Three cut of values were determined according to sensitivity and specificity by using youden index. They were 8515.73 pg/mL (sensitivity 78%, specificity 76%), 7097 pg/mL (sensitivity 80%, specificity 70%) and 11063.48 pg/mL (sensitivity 76%, specificity 70%). Patients with SCLC had shorter survival time above cut-off values (p> 0.05). VEGF and Ang-2 showed a weak positive correlation (p= 0.1 and r= 0.638). Conclusion: In conclusion, serum VEGF wasn't useful to predict lung cancer, prognosis or cell type. Albeit Ang-2 was higher in patients with lung cancer without any effect on survival. Due to the heterogeneity of the studies done with serum measurement Ang-2 on tumor tissue should be more meaningful.

Epilepsy is a chronic neurological disorder in which the normal pattern of neuronal activity in the brain becomes disturbed. Identification of the brain region that is abnormally active during an epileptic seizure is vital for epilepsy surgery. One way of achieving so is to collect electroencephalography ( EEG) signals from epileptic people and then to identify the active region as a seizure occurs. In this work, we present a Bayesian change point model that detects when seizures occur. We applied our method to a data set that contains 48 EEG and electrocardiography (EKG) record pairs collected from epileptic people and observed that the model is able to detect all seizures. Turk Telekom, Arcelik A S, Aselsan, ARGENIT, HAVELSAN, NETAS, Adresgezgini, IEEE Turkey Sect, AVCR Informat Technologies, Cisco, i2i Syst, Integrated Syst & Syst Design, ENOVAS, FiGES Engn, MS Spektral, Istanbul Teknik Univ

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Saccharomyces cerevisiae, known as baker's yeast, is also used as a probiotic agent to treat gastroenteritis by modulating the endogenous flora and immune system. However, since there have been increasing reports of fungemia due to S.cerevisiae and its subspecies S.boulardii, it is recommended that probiotics should be cautiously used in immunosuppressed patients, people with underlying diseases and low-birth weight babies. To emphasize this phenomenon, in this report, a case of S.cerevisiae fungemia developed in a patient given probiotic treatment for antibiotic-associated diarrhea, was presented. An 88-year-old female patient was admitted to our hospital with left hip pain, hypotension, and confusion. Her medical history included hypertension, chronic renal failure, left knee replacement surgery, and recurrent urinary tract infections due to neurogenic bladder. She was transferred to the intensive care unit with the diagnosis of urosepsis. After obtaining blood and urine samples for culture, empirical meropenem (2 x 500 mg) and linezolid (1 x 600 mg) treatment were administered. A central venous catheter (CVC) was inserted and after one day of inotropic support, her hemodynamic parameters were stabilized. The urine culture obtained on admission yielded extended-spectrum beta-lactamase-producing Klebsiella pneumoniae and Escherichia coli. Urine culture was repeated after three days and no bacteria were isolated. On the 4th day of admission she developed diarrhea. Toxin A/B tests for Clostridium difficile were negative. To relieve diarrhea, S.boulardii (Reflor 250 mg capsules, Sanofi Aventis, Turkey) was administered twice a day, without opening capsules. Two days later, her C-reactive protein (CRP) level increased from 23.2 mg/L to 100 mg/L without fever. Her blood culture taken from the CVC yielded S.cerevisiae. Linezolid and meropenem therapies were stopped on the 13th and 14th days, respectively, while prophylactic fluconazole therapy was replaced with caspofungin 1 x 50 mg on the fifth day. After seven days of therapy CRP and serum creatinine levels decreased to 9.1 mg/L and 1.2 mg/dl, respectively; and she was discharged from the hospital with improvement. The probiotic capsules were used unopen, thus, it was proposed that S.cerevisiae fungemia originated from translocation from the intestinal mucosa. Since it was not possible to investigate the molecular genetics of the strain isolated from the blood culture and the strain present in the probiotic, a definite conclusion about the origin of the strain could not be reached. It was thought that old age and underlying disease of the patient were the related predisposing factors for S.cerevisiae fungemia. This case emphasized that clinicians should be cautious in case of probiotic application even though in encapsulated form, even in immunocompetent patients with a history of long-term hospital stay and use of broad-spectrum antimicrobials since there may be a risk of S.cerevisiae fungemia development.

International audience; AbstractDrug hypersensitivity reactions (DHRs) affect an unknown proportion of the general population, and are an important public health problem due to their potential to cause life-threatening anaphylaxis and rare severe cutaneous allergic reactions. DHR evaluations are frequently needed in both ambulatory and hospital settings and have a complex diagnosis that requires a detailed clinical history and other tests that may include in vitro tests and in vivo procedures such as skin tests and drug provocation tests. Although over the years both European and U.S. experts have published statements on general procedures for evaluating DHRs, a substantial discordance in their daily management exists. In this review, we highlight both the differences and the similarities between the European and U.S. perspectives. While a general consensus exists on the importance of skin tests for evaluating DHRs, concordance between Americans and Europeans exists solely regarding their use in immediate reactions and the fact that a confirmation of a presumptive diagnosis by drug provocation tests is often the only reliable way to establish a diagnosis. Finally, great heterogeneity exists in the application of in vitro tests, which require further study to be well validated.

Primary hyperoxaluria (PH) is a rare autosomal recessive disease caused by the functional defect of alanine-glyoxylate aminotransferase (AGT) enzyme in the liver and it is characterized by the deposition of diffuse calcium oxalate crystals. A 38-year-old male patient presented with history of recurrent nephrolithiasis and has received chronic hemodialysis treatment for 2 years. Cadaveric renal transplantation was applied to the case. The patient was reoperated on postoperative day 13 because of the collection surrounding the urethra. During this operation, kidney biopsy was made due to late decrease in creatinine levels. Deposition of diffuse oxalate crystal was detected in allograft kidney biopsy, whereas in the 0-hour biopsy there were no oxalate crystals. Oxalate level was found to be high in a 24-hour urine specimen (118 mg/L, normal level: 7-44 mg/L). The patient was identified with primary hyperoxaluria and followed up in terms of systemic oxalate deposition as well as allograft kidney. In the kidney biopsy taken after 18 months, we detected that oxalate crystals almost entirely disappeared. In our case, bilateral preretinal, intraretinal, and intravascular diffuse oxalate crystals were detected, and argon laser photocoagulation treatments were needed for choroidal and retinal neovascularization. Repeated ophthalmic examinations showed the regressive nature of oxalate depositions. In the 18th month, fundus examination and fluorescein angiography revealed that oxalate crystals were significantly regressed. To increase the quality of life and slow down the systemic effects of oxalosis, kidney-only transplantation is beneficial.

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WOS: 000306989000006, PubMed ID: 21889254, Background: There are no country-based data focused on aspirin (ASA)-exacerbated respiratory disease (AERD) in Turkey. Objective: To assess the prevalence of AERD in adult patients with asthma. Methods: A structured questionnaire was administered via face-to-face interview by a specialist in pulmonology/allergy at seven centres across Turkey. Results: A total of 1344 asthma patients (F/M: 1081/263: 80.5%/19.5%, mean age: 45.7 +/- 14.2 years) were enrolled. Atopy rate was 47%. Prevalence of allergic rhinitis, chronic rhinosinusitis/rhinitis, and nasal polyposis (NP) were 49%, 69% and 20%, respectively. Of 270 patients with NP, 171 (63.3%) reported previous nasal polypectomy and 40 (25%) had a history of more than three nasal polypectomies. Aspirin hypersensitivity was diagnosed in 180 (13.6%) asthmatic patients, with a reliable history in 145 (80.5%), and oral ASA provocation test in 35 (19.5%) patients. Clinical presentations of ASA hypersensitivity were respiratory in 76% (n = 137), respiratory/cutaneous in 15% (n = 27), and systemic in 9% (n = 16) of the patients. Multivariate analysis indicated that a family history of ASA hypersensitivity (p: 0.001, OR: 3.746, 95% CI: 1.769-7.929), history of chronic rhinosinusitis/rhinitis (p: 0.025, OR: 1.713, 95% CI: 1.069-2.746) and presence of NP (p < 0.001, OR: 7.036, 95% CI: 4.831-10.247) were independent predictors for AERD. Conclusion: This cross-sectional survey showed that AERD is highly prevalent among adult asthmatics and its prevalence seems to be affected by family history of ASA hypersensitivity, history of rhinosinusitis and presence of NP. (C) 2011 SEICAP. Published by Elsevier Espana, S.L. All rights reserved.

Background: Augmentation index (AIx) and pulse wave velocity (PWV) are early markers of atherosclerotic vascular changes and also have been shown to be predictive of cardiovascular disease and total mortality. The aim of our study was to evaluate the relationship between PWV and AIx-HR75, which is the corrected form of AIx according to a heart rate of 75 beats/min, echocardiographic parameters and biochemical parameters in chronic hemodialysis (HD) patients. Subjects and methods: AIx-HR75 and PWV were measured in 556 HD patients by applanation tonometry using the SphygmoCor device. Results: The mean PWV and AIx-HR75 values of the study group were 10.2 +/- 2.4 and 28.4 +/- 10.2 m/s. A positive correlation was found between PWV and AIx-HR75 (r = 0.214, p = 0.000). AIx-HR75 correlated with age (r = 0.093, p = 0.028), body surface area (BSA) (r = -0.194, p = 0.000), mean arterial pressure (MAP) (r = 0.335, p = 0.000), pulse pressure (PP) (r = 0.212, p = 0.000), cardiothoracic index (r = 0.155, p = 0.016), and presence of left ventricular hypertrophy (r = 0.152, p = 0.001). PWV correlated with MAP (r = 0.208, p = 0.000), PP (r = 0.098, r = 0.021), left ventricular mass (r = 0.105, p = 0.023), and predialysis sodium level (r = -0.105, p = 0.023). In the multivariate analyses, PWV was associated with MAP (t = 3.78, p = 0.000), presence of diabetes (t = 3.20, p = 0.001), and predialysis sodium level (t = -2.06, p = 0.040), and AIx-HR75 was associated with age (t = 2.48, p = 0.014), female sex (t = 3.98, p = 0.000), BSA (t = -2.15, p = 0.033), and MAP (t = 7.02, p = 0.000). Conclusion: There is a strong association between MAP and arterial stiffness parameters in HD patients. We feel that efficient control of blood pressure could lead to reduced arterial stiffness in HD patients.

WOS: 000266684100017, PubMed ID: 19181507, Epidemiological characteristics of sarcoidosis differ according to geographical distribution. The aim of our study was to disclose epidemiological characteristics in our country. The data was collected from investigators, who sent information on newly-diagnosed patients via internet. In 2 years 198 female and 95 mate patients were enrolled to the study (f/m:2.08). Mean age of patients was 44 +/- 13 years (17-90). Mean age of mate patients was 38 12 while mean age of female patients was 48 13 (p < 0.001). 73.4% of patients were nonsmokers (85.4% of females; 48.4% of mates; (p < 0.001)). About 50% of our 293 patients were housewives. Familial sarcoidosis was found in 3 patients' first degree relatives. Estimated annual incidence of sarcoidosis for Turkey was calculated as 4 per 100,000 person. According to our study, 2/3 of sarcoidosis patients were women; mean age of patients was 45 and the disease began 10 years later in female patients. 80% of patients were nonsmokers; negative relation between sarcoidosis and smoking was evident especially in women. Familial sarcoidosis frequency was lower compared to other studies in the literature. There was no occupational exposure history in our patients. Our incidence rate, is similar with the results of other European studies. (C) 2008 Elsevier Ltd. All rights reserved.