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Clinical characteristics and therapeutic outcomes of paroxysmal nocturnal hemoglobinuria patients in Turkey: a multicenter experience
No full textErkurt, Mehmet Ali/0000-0002-3285-417XPubMed: 33988739WOS:000650535000001The aim of this study is to collect paroxysmal nocturnal hemoglobinuria (PNH) patient data from hematology centers all over Turkey in order to identify clinical features and management of PNH patients. Patients with PNH were evaluated by a retrospective review of medical records from 19 different institutions around Turkey. Patient demographics, medical history, laboratory findings, and PNH-specific information, including symptoms at the diagnosis, complications, erythrocyte, and granulocyte clone size, treatment, and causes of death were recorded. Sixty patients (28 males, 32 females) were identified. The median age was 33 (range; 17-77) years. Forty-six patients were diagnosed as classic PNH and 14 as secondary PNH. Fatigue and abdominal pain were the most frequent presenting symptoms. After eculizumab became available in Turkey, most of the patients (n = 31/46, 67.4%) were switched to eculizumab. Three patients with classic PNH underwent stem cell transplantation. The median survival time was 42 (range; 7-183 months) months. This study is the first and most comprehensive review of PNH cases in Turkey. It provided us useful information to find out the differences between our patients and literature, which may help us understand the disease
Gallbladder Uptake Mimicking Liver Metastasis on Lu-177-DOTATATE Posttherapy Scan Gallbladder Uptake on Lu-177-DOTATATE Scan
No full textPubMed: 33512839WOS:000618216000004We represent the case of a 61-year-old man with atypical carcinoid tumor of the lung. On posttherapy Lu-177-DOTATATE whole-body scan, focal intense uptake in the inferomedial side of the liver was detected. Pretherapy Ga-68-DOTATATE PET/CT showed no sign of liver metastasis, and posttherapy diagnostic dynamic liver MRI is used to exclude metastatic liver disease. Focal intense uptake was attributed to physiological gallbladder uptake
Oxidative stress and inflammatory response of ghrelin on myocardial and aortic tissues in insulin-resistant rats
No full textcoskun, zeynep mine/0000-0003-4791-6537PubMed: 33772291WOS:000637939900010Objectives This study was designed to clarify the effects of ghrelin on myocardial and aortic tissues in insulin-resistant rats. Methods Sprague-Dawley rats were divided into the following groups: control (Group 1), insulin resistance (IR, Group 2), ghrelin (Group 3) and IR+Ghrelin (Group 4) groups. Levels of HOMA-IR, fibronectin, hydroxyproline, collagen-1, collagen-3, matrix metalloproteinase-3, and matrix metalloproteinase-9, and tissue inhibitor of metalloproteinase-1, and oxidative stress parameters as protein carbonyl (PCO), lipid hydroperoxides (LHPs), malondialdehyde, total thiol were determined in myocardial tissue. Expressions of IL-6, NF-kappa B and TNF-alpha mRNAs were detected by RT-qPCR. Aorta tissue was stained Masson trichrome. Key findings The HOMA-IR level decreased in the IR+Ghrelin group compared with the IR group (P B and TNF-alpha in the IR+Ghrelin group compared with the IR group (P < 0.001). There was no difference among the groups in the histology of aortic tissue. Conclusions Ghrelin, a regulator of appetite and energy homeostasis, may be effective in regulating oxidative stress and the inflammatory response when impaired by IR. Therefore, ghrelin may reduce the risks of myocardial dysfunction in IR
Functional Connectivity Analysis in Heterozygous Glucocerebrosidase Mutation Carriers
No full textSezgin, Mine/0000-0001-6525-0658; KURT, ELIF/0000-0003-1956-575X; Uyguner, Zehra Oya/0000-0002-2035-4338PubMed: 33492243WOS:000640553700016Background: There is evidence that alterations in functional connectivity (FC) of the striatocortical circuits may appear before the onset of clinical symptoms of Parkinson's disease (PD). Objective: The aim of this study was to investigate FC of the striatocortical circuitry in asymptomatic carriers of heterozygous glucocerebrosidase (GBA) mutations, which pose a significant risk for developing PD. Methods: Twenty-one parents of confirmed Gaucher disease patients who were carrying heterozygous GBA mutations and 18 healthy individuals matched for age and gender were included. GBA mutation analysis was performed in all participants. Clinical evaluation included neurological examination, Mini Mental State Examination, and UPDRS Part III. Structural and functional MRI data of 18 asymptomatic GBA mutation carriers (asGBAmc) and 17 healthy controls (HC) were available. FC was analyzed with seed-based approach. Results: Eleven asymptomatic mutation carriers had heterozygous p.L483P mutation, 6 subjects heterozygous p.N409S mutation and 1 subject heterozygous p.R392G mutation in GBA gene. Mini-Mental State Examination mean score was 28.77 (+/- 1.16) and 29.64 (+/- 0.70) in asGBAmc and HC groups, respectively (p = 0.012). Significant increased connectivity between left posterior putamen and the left postcentral gyrus was found in the asGBAmc group compared to HC, whereas left caudate showed hyper-connectivity with the right parietal operculum and right planum temporale (Familywise alpha<0.05, adjusted for cluster size). Conclusion: Our results suggest that alterations in striatocortical FC can be detected in asymptomatic heterozygous GBA mutation carriers who are at risk of developing PD. These findings may provide insight into network changes during the asymptomatic phase of PD.Scientific Research Projects Coordination Unit of Istanbul UniversityIstanbul University [25144, 42362]This work was supported by Scientific Research Projects Coordination Unit of Istanbul University (Project numbers 25144 and 42362)
Prevalence of lower urinary tract symptoms in nurses and civil servants working at a hospital: A cross-sectional study
No full text2-s2.0-85104985089Background: Lower urinary tract symptoms (LUTS) are common in women and can interrupt daily living activities of the individuals. The study aimed at determining the LUTS prevalence and the influencing factors in nurses and civil servants working at a hospital. Methods: This cross-sectional and descriptive study was conducted with 158 female nurses and 105 female civil servants. The data were obtained with a data collection form and the Bristol Female Lower Urinary Tract Symptoms-Short Form. Results: This paper exposes that the prevalence of at least one LUTS was 94.2% in nurses and 97.1% in civil servants. The most common LUTS symptoms of nurses and civil servants were urgency (60.1%nurses, 81.9%civil servants) and urge incontinence (59.5%nurses, 81.9%civil servants). Nurses (60.8%) expressed significantly higher rates of having inadequate time going to the toilet due to their work conditions compared to the civil servants (41.9%) (p<0.05). BFLUTS-SF scores in terms of age, BMI, parity, having cesarean and vaginal delivery and urinary incontinence in their previous pregnancies were compared between two groups, statistically significant differences were found (p<0.05). Conclusion: Workplace conditions of the health workers should be reorganizing to have healthy urinary habits for preventing them from the development of LUTS. © 2021 Kok G et al
Hodgkin Lenfomanın Monoklonal Antikorlardan Nivolumab ile Tedavisi Ardından Gelişen Otoimmün Ensefalit
No full textSon yıllarda, monoklonal antikorların çeşitli onkolojik hastalıklardakullanılmaya başlanmasıyla birlikte, nörolojik yan etkiler daha sıkgörülmeye başlamıştır. Monoklonal antikorlardan biri olan nivolumab,programlanmış hücre ölüm proteini 1’i bloke eder ve metastatikmelanoma, skuamöz hücreli akciğer kanseri, renal hücre karsinomasıve Hodgkin lenfoma tedavisinde onaylanmış bir insan IgG4 antikorudur.Burada, Hodgkin lenfoma tanısı ile nivolumab tedavisi uygulanmasısonrası ensefalopati tablosu gelişen 40 yaşında bir olguyu sunmaktayız.Literatürde, monoklonal antikorlardan ipilimumab ve nivolumabınkombinasyon tedavisi sonrası otoimmün ensefalit vakaları dahaönce bildirilmiştir. Bildiğimiz kadarıyla bu olgu, Hodgkin lenfomaiçin kullanılan nivolumab monoterapi tedavisi sonrası bildirilen ilkensefalopati vakasıdır
The cut-off values for Visual Analogue Scale (VAS) and for the Assessment Tool for Asthma (ATA) according the Control for Asthma and Allergic Rhinitis Test (CARAT)
No full text[No Abstract Available
Evaluation of Thiol/Disulfide Homeostasis and Other OxidativeStress Markers in Patients Undergoing Hemodialysis
No full textObjective: To investigate the potential role of thiol/disulphide homeostasis as a novel biomarker of oxidative stress in pa tients with diabetes and undergoing hemodialysis (HD) and its correlation with other oxidative stress markers. Materials and Methods: This study included 82 patients with end-stage renal disease undergoing HD for four hours, threetimes weekly for more than 24 months in the dialysis center. Of the 82 patients, 47 were non-diabetic and 35 were diabet ic. Blood samples were collected from the patients before and after the HD sessions. The thiol/disulfide pair tests wereperformed and total antioxidant status (TAS), total oxidant status (TOS), oxidative stress index (OSI), ischemia-modifiedalbumin (IMA) levels, albumin levels, ceruloplasmin, catalase activity (CAT), and myeloperoxidase (MPO) activity were de termined in the serum.Results: The TAS values in all the patients, both diabetic and non-diabetic, decreased significantly after HD (p<0.001,p=0.003, and p<0.001; respectively). The TOS, albumin, native thiol (p=0.001, p=0.007, p=0.001, respectively), OSI, CAT,ceruloplasmin, IMA, MPO, and total thiol (p<0.001, p<0.001, p<0.001, p<0.001, p<0.001, and p<0.001, respectively) valuesincreased significantly in all the patients after the HD session. The TOS, OSI, CAT, IMA, albumin, MPO, native thiol, and per centages of native/total thiol, ceruloplasmin, and total thiol values (p=0.002, p=0.002, p=0.002, p=0.001, p=0.008, p=0.001,p=0.001, p=0.003, p=0.023, and p<0.001; respectively) increased significantly in patients with diabetes after the HD session.Conclusion: In this study, we demonstrated the relationship between oxidative stress markers, which play a significant rolein the pathogenesis of diabetes, and thiol/disulfide balance undergoing HD patients
Nöromiyelitis Optika’da Kognitif Bozukluk
No full textAmaç: Nöromiyelitis optika (NMO) hastalarında kognisyonudeğerlendirmek üzere yapılan az sayıda çalışma mevcuttur. Bu nedenleNMO tanısı olan Türk hastalarda kognitif fonksiyon bozukluğunun(KFB) sıklığı ve bununla ilişkili olabilecek etkenlerin değerlendirilmesiamaçlanmıştır.Yöntem: NMO tanısıyla izlenmekte olan 22 olgu retrospektif olarakdeğerlendirilmiştir. Hastalara Kısa Tekrarlanabilir Test Bataryası (BRB-N),Beck Depresyon Envanteri (BDE) ve Addenbrooke Kognitif Değerlendirme(ACE-R) testleri uygulanmış; KFB olan ve olmayan gruplar yaş, cinsiyet,eğitim düzeyi, NMO Ig G sonucu, EDSS skoru, kraniyal MRG’de lezyonolup olmamasına göre karşılaştırılmıştır. Hastaların demografik, klinikve radyolojik verilerinin depresyonla ilişkisi değerlendirilmiştir. ACE-Rtestinin skorları incelenerek NMO hastalarında ACE-R testinin KFBsaptamada duyarlılığı ve özgüllüğü değerlendirilmiştir.Bulgular: Olguların ortalama yaşı 42,8±10,9 yıldır. Hastaların %45,5’inde(n=10) KFB; %50’sinde ise (n=11) depresyon saptanmıştır. KFB olanolgularda istatistiksel olarak anlamlı bir şekilde yaşın daha yüksek olduğu,eğitim düzeyinin daha düşük olduğu, EDSS ve BDE skorlarının dahayüksek olduğu saptanmıştır (p<0,05). Hastalarda en sık etkilenen kognitifalanın; bellek kusuru, dikkat ve yürütücü işlevlerde bozulma olduğuizlenmiştir. ACE-R testinin NMO hastalarında duyarlılığı ve özgüllüğüdeğerlendirildiğinde ACE-R testinin tanı değerinin istatistiksel olarakiyi seviyesinde olduğu; 82,5 kesme değerinde, %88 duyarlılık ve %75özgüllük ile KFB saptayabileceği bulunmuştur.Sonuç: Çalışmamızda BRB-N ve BDE testleri ile kognitif bozukluk vedepresyon NMO hastalarının yaklaşık yarısında saptanmıştır. ACE-Rtestinin de klinik pratikte NMO’da KFB tanısında kullanılabileceğikanısına varılmıştır. NMO’da KFB ve depresyon sıklıkla eşlik eden tablolarolması nedeni ile hastaların bu yönden mutlaka değerlendirilmesi vetedavilerinin düzenlenmesi, hastaların yaşam kalitesinin yükseltilmesiaçısından büyük önem arz etmektedir
Tek semptomu baş ağrısı olan serebral venöz sinüs trombozu: VENOST çalışmasından elde edilen verilerin alt grup analizi
No full textObjectives: Headache is the most common complaint in cerebral venous sinus thrombosis (CVST) and it may sometimes be the only symptom in these patients. This retrospective and prospective study was an investigation of any differences in terms of clinical risk factors, radiological findings, or prognosis in patients with CVST who presented with isolated headache (IH) and cases with other concomitant findings (non-isolated headache [NIH]). Methods: A total of 1144 patients from a multicenter study of cerebral venous sinus thrombosis (VENOST study) were enrolled in this research. The demographic, biochemical, clinical, and radiological aspects of 287 IH cases and 857 NIH cases were compared. Results: There were twice as many women as men in the study group. In the IH group, when gender distribution was evaluated by age group, no statistically significant difference was found. The onset of headache was frequently subacute and chronic in the IH group, but an acute onset was more common in the NIH group. Other neurological findings were observed in 29% of the IH group during follow-up. A previous history of deep, cerebral, or other venous thromboembolism was less common in the IH group than in the NIH group. Transverse sinus involvement was greater in the IH group, whereas sagittal sinus involvement was greater in the NIH group. The presence of a plasminogen activator inhibitor (PAI) mutation was significantly greater in the IH group. Conclusion: IH and CVST should be kept in mind if a patient has subacute or chronic headache. PAI, which has an important role in thrombolytic events, may be a risk factor in CVST. Detailed hematological investigations should be considered. Additional studies are needed.Amaç: Baş ağrısı serebral venöz sinüs trombozunda (SVST) en sık görülen şikayettir ve bazen CVST ile başvuran hastalarda görülen tek semptom olabilir. Bu retrospektif ve prospektif çalışmada, izole başağrısı (IB) ile başvuran SVST olgularında ve baş ağrısı ile ilişkili diğer bulguları olan (izole olmayan başağrısı-İOBA) olgularda klinik risk faktörleri, radyoloji ve prognoz açısından herhangi bir farklılık olup olmadığını araştırdık. Gereç ve Yöntem: Serebral venöz sinüs trombozu (VENOST) çok merkezli çalışmasından 1144 hasta çalışmamıza alındı. Tüm demografik, biyokimyasal, klinik ve radyolojik yönler 287 IBA vakası ve 857 İOBA vakası için karşılaştırılmıştır. Bulgular: Toplam grup içinde kadın oranı iki kat daha yüksekti. İBA grubunda cinsiyet dağılımını yaş gruplarına göre değerlendirildiğinde istatistiksel olarak anlamlı bir fark bulunmadı. İBA grubunda, baş ağrısının başlangıcı sıklıkla subakut ve kronikti, ancak akut başlangıç, İOBA grubunda daha yaygındı. İBA grubunda% 29’luk takip sırasında diğer nörolojik bulgular eklenmiştir. Daha önce serebral, derin ve diğer venöz tromboembolizm öyküsü İBA grubunda İOBA grubuna göre daha azdı. Transvers sinüs tutulumu İBA grubunda daha yüksek iken sagital sinüs tutulumu İOBA grubunda daha fazlaydı. Plazminojen aktivatör inhibitörü (PAI) mutasyonu IBA grubunda anlamlı olarak daha yüksekti. Sonuç: Hastaların subakut veya kronik baş ağrısı varsa SVST tanısı için IBA akılda tutulmalıdır. Trombolitik olaylar için önemli bir role sahip olan PAI, SVST’da bir risk faktörü olabilir, bu nedenle ayrıntılı hematolojik araştırmalar düşünülmelidir. Daha ileri çalışmalara ihtiyaç vardı