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1.
Modern approach to the management of genitourinary syndrome in women with gynecological malignancies
Nina Kovačević, Ines Cilenšek, Sebastjan Merlo, Barbara Šegedin, 2023, review article

Abstract: The term genitourinary syndrome of menopause was first used in 2014 by the North American Menopause Society and the International Society for the Study of Women's Sexual Health to describe conditions previously known as atrophic vaginitis, urogenital atrophy, or vulvovaginal atrophy. It is a complex, chronic, progressive condition characterized by a wide range of signs and symptoms affecting sexual function and the tissues of the urinary and genital tracts. The main cause of genitourinary syndrome of menopause is estrogen deficiency caused by ovarian removal or dysfunction. The most bothersome symptoms are vaginal dryness, decreased vaginal lubrication, and pain during penetration and intercourse. They all have a negative impact on the quality of life. Conclusions: The main goal of treatment is to relieve the symptoms. Treatment modalities are pharmacological or non-pharmacological. The first-line treatment for mild to moderate symptoms is the use of personal lubricants and moisturizers, but the gold standard is estrogen replacement therapy. Hormone therapy may not be an option for women with hormone-dependent cancer.
Keywords: genitourinary syndrome, gynecological malignancies, therapy
Published in DiRROS: 25.07.2024; Views: 119; Downloads: 34
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2.
Advances in the management of craniopharyngioma in children and adults
Mojca Jensterle Sever, Sončka Jazbinšek, Roman Bošnjak, Mara Popović, Lorna Zadravec-Zaletel, Tina Vipotnik-Vesnaver, Barbara Faganel Kotnik, Primož Kotnik, 2019, review article

Abstract: Childhood and adult-onset craniopharyngioma is a rare embryogenic tumor of the sellar, suprasellar, and parasellar region. Survival rates are high; however, tumor location and treatment sequalae including endocrine deficits, visual impairment, metabolic complications, cognitive and psychosocial deficits can significantly impair patient%s quality of life. There is considerable controversy regarding the optimal management of craniopharyngiomas. Subtotal resection of the tumor followed by targeted irradiation to avoid further hypothalamic damage is currently indicated. Novel insights in the tumor%s molecular pathology present the possibility for targeted therapy possibly decreasing the rate and severity of treatment-associated morbidity. Conclusions. Craniopharyngioma should be seen as a chronic disease. To achieve optimal outcomes a multidisciplinary team of specialized neurosurgeons, neuro-radiologists, neuro-oncologists, pathologists and endocrinologists should be involved in the diagnosis, planning of the surgery, irradiation and long-term follow-up.
Keywords: craniopharyngioma, hypopituitarism, metabolic syndrome
Published in DiRROS: 09.07.2024; Views: 157; Downloads: 77
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3.
Mesenteric ischemia after capecitabine treatment in rectal cancer and resultant short bowel syndrome is not an absolute contraindication for radical oncological treatment
Ana Perpar, Erik Brecelj, Nada Rotovnik-Kozjek, Franc Anderluh, Irena Oblak, Marija Skoblar Vidmar, Vaneja Velenik, 2015, other scientific articles

Abstract: Thrombotic events, arterial or venous in origin, still remain a source of substantial morbidity and mortality in cancer patients. The propensity for their development in oncology patients is partially a consequence of the disease itself and partially a result of our attempts to treat it. One of the rarest and deadliest thromboembolic complications is arterial mesenteric ischemia. The high mortality rate is caused by its rarity and by its non-specific clinical presentation, both of which make early diagnosis and treatment difficult. Hence, most diagnoses and treatments occur late in the course of the disease. The issue survivors of arterial mesenteric ischemia may face is short bowel syndrome, which has become a chronic condition after the introduction of parenteral nutrition at home. We present a 73-year-old rectal cancer patient who developed acute arterial mesenteric thrombosis at the beginning of the pre-operative radiochemotherapy. Almost the entire length of his small intestine, except for the proximal 50 cm of it, and the ascending colon had to be resected. After multiorgan failure his condition improved, and he was able to successfully complete radical treatment (preoperative radiotherapy and surgery) for the rectal carcinoma, despite developing short bowel syndrome (SBS) and being dependent upon home-based parenteral nutrition to fully cover his nutritional needs. Mesenteric ischemia and resultant short bowel syndrome are not absolute contraindications for radical oncological treatment since such patients can still achieve long-term remission.
Keywords: acute mesenteric ischemia, capecitabine, multiorgan failure, rectal cancer, short bowel syndrome
Published in DiRROS: 23.04.2024; Views: 278; Downloads: 82
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4.
Effect of graded exercise therapy and medical care on chronic fatigue syndrome
Tamara Zličić, Darinka Korovljev, Tijana Šćepanović, Ivana Milovanović, 2023, review article

Abstract: Purpose: Chronic Fatigue Syndrome (CFS) / Myalgic Encephalomyelitis (ME) is a complex condition with debilitating symptoms that significantly impact individuals, particularly those in the working population. This study aims to investigate the effec-tiveness of Graded Exercise Therapy (GET) and Cognitive Behavioral Therapy (CBT) along with additional methods such as Graded Exercise Self-help (GES), Adaptive Pac-ing Therapy (APT), and Specialist Medical Care (SMC), in managing Chronic Fatigue Syndrome (CFS) / Myalgic Encephalomyelitis (ME) among the working population. Methods: A systematic analysis of five randomized controlled trials conducted be-tween 2013 and 2023, encompassing GET, CBT, APT, SMC, and GES was performed using PubMed.Results: The selected studies consistently demonstrate that GET positively impacts physical functioning and reduces fatigue levels in working individuals with CFS. Ad-ditionally, CBT proves valuable, emphasizing the importance of addressing the mental aspects of CFS in occupational contexts.Conclusion: This review underscores the need for further research, advocating for direct assessment methods like biomarkers to enhance our understanding of CFS and improve treatment outcomes. These insights are crucial for healthcare practitioners, researchers, and policymakers navigating the complexities of CFS within the work-place. Emphasizing the integration of psychological interventions with physical therapies is essential for a comprehensive approach to managing CFS among the working population.
Keywords: Chronic Fatigue Syndrome / Myalgic Encephalomyelitis, CFS/ME, graded exercise therapy, cognitive behavioral therapy, adaptive pacing therapy, specialist medical care, working population
Published in DiRROS: 17.04.2024; Views: 283; Downloads: 182
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5.
Vismodegib in locally advanced basal cell carcinoma in Slovenia
Tanja Mesti, Maša Sever, Janja Ocvirk, 2022, original scientific article

Abstract: Background: Vismodegib is a first-in-class inhibitor of the hedgehog pathway for treatment of locally advanced basal cell carcinoma (laBCC) and metastatic BCC. Objectives: The purpose of this study is to report outcomes of patients with laBCC, with basal cell carcinoma nevus syndrome (Gorlin Goltz syndrome [G-G Syn]) treated with vismodegib in routine clinical practice in Slovenia in 8.3-year period. Methods: In this retrospective cohort study, we analyzed baseline characteristics, outcomes, and treatment-related adverse events from locally advanced BCC. The patients were divided into two cohorts: 39 laBCC or multiple BCC patients and 7 patients with G-G Syn who were treated with vismodegib from November 2012 till January 2021. Results: During 100-month period, 46 patients were diagnosed with laBCC (26), multiple BCC (13), and G-G Syn (7), all inappropriate for surgery or radiotherapy. Baseline characteristics: median age was 72.8 years in laBCC + multiple BCC cohort and 47.4 years in G-G Syn cohort. The objective response rate was 80% in laBCC + multiple BCC and 86% in G-G Syn cohort. Disease control rate (DCR) was 95% in laBCC + multiple BCC and 100% in G-G Syn cohort. Median duration of treatment was 9.9 months (range: 1.5-43.1) in laBCC and multiple BCC cohort and 19.5 months (range: 3.6-94.1) in G-G Syn cohort. Majority of treatment-emergent adverse events (TEAEs) in laBCC or multiple BCC cohort were grade 1 or 2 (96%), only 4% of AEs were grade 3. Majority of TEAEs in G-G Syn cohort were also grade 1 or 2 (87%), 13% of AEs were grade 3. No grade 4 or 5 vismodegib-related AEs were reported. Conclusion: Vismodegib has shown meaningful efficacy with DCR from 95% to 100% in patients with laBCC, multiple BCC, and G-G Syn in Slovenia. TEAEs were successfully alleviated with multidisciplinary approach and early supportive care.
Keywords: basal cell carcinoma, early supportive care, Gorlin Goltz syndrome, multidisciplinary approach
Published in DiRROS: 07.09.2022; Views: 589; Downloads: 297
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6.
Dietary acid load but not Mediterranean diet adherence score is associated with metabolic and cardiovascular health state : ǂa ǂpopulation observational study from Northern Italy
Juana Maria Sanz, Domenico Sergi, Simona Colombari, Eleonora Capatti, Roberta Situlin, Gianni Biolo, Filippo Giorgio Di Girolamo, Stefano Lazzer, Boštjan Šimunič, Rado Pišot, Angelina Passaro, 2022, original scientific article

Abstract: Diet plays a pivotal role in shaping the trajectory of chronic diseases. In this regard, the Mediterranean diet has been widely shown to exert beneficial effects on cardiometabolic health. On the contrary, the Western diet, which has also been reported to be an acidogenic dietary pattern, elicits detrimental effects on both metabolic and cardiovascular (CV) health. However, the role of dietary acid load (DAL) as a predictor of cardiometabolic prognosis remains to be elucidated. Thus, this study aims to compare Mediterranean diet adherence (MDA) and DAL focusing on their relationship with metabolic and CV prognosis. A total of 448 individuals aged 55–80 years were grouped depending on their MDA, assessed using food frequency questionnaires, or DAL, evaluated using potential renal load acid (PRAL) and net-endogenous acid production (NEAP). Study participants underwent anthropometric and biochemical measurements. The metabolic syndrome (MetS) prevalence was evaluated according to the National Cholesterol Education Program-Adult Treatment Panel III. Finally, the CV risk was evaluated using three independent algorithms: atherosclerotic cardiovascular disease (ASCVD), European Systematic COronary Risk Evaluation (SCORE), and Cuore risk scores. Mediterranean diet adherence was negatively associated with PRAL and NEAP. Individuals in the higher MDA tertile group had higher HDL cholesterol as well as lower homeostasis model assessment index (HOMA-IR) and fat mass relative to the lowest MDA tertile. However, in the high-MDA tertile group, there was neither a significantly lower MetS prevalence nor CV risk. Instead, both the MetS prevalence and CV risk were higher in individuals in the higher acid PRAL quartile relative to the lower alkaline PRAL quartile. Dietary acid load, especially assessed using PRAL but not MDA, was associated with indices of metabolic and CV prognosis. Thus, DAL assessed by 24-h dietary recalls may represent a better predictor of cardiometabolic health if compared to MDA evaluated using food frequency questionnaires.
Keywords: Mediterranean diet, dietary acid load, alkaline diet, acidic diet, metabolic syndrome, cradiovascular risk score
Published in DiRROS: 19.05.2022; Views: 921; Downloads: 536
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Hereditary systemic autoinflammatory diseases and Schnitzler's syndrome
Mark Kačar, Shelly Pathak, Sinisa Savic, 2019, review article

Abstract: The systemic autoinflammatory diseases are disorders of the innate immune system distinguished by severe inflammation resulting from dysregulation of the innate immune system. Hereditary fever syndromes, such as FMF, TNF receptor-associated periodic syndrome, cryopyrin-associated periodic syndromes and mevalonate kinase deficiency, were the first group of systemic autoinflammatory diseases for which a genetic basis was established, between 1999 and 2001. Currently according to the latest report of the international union of immunological societies, 37 separate monogenic disorders were classified as autoinflammatory. In addition to the abovementioned monogenic conditions, we describe Schnitzler's syndrome, a well-defined, acquired autoinflammatory condition without a clear genetic basis. For the purposes of this review, we discuss several conditions defined by the latest consensus process as systemic autoinflammatory diseases. We focus on those disorders where recent studies have contributed to further phenotypic characterization or had an impact on clinical management.
Keywords: pyrin, Schnitzler syndrome, haploinsufficiency, autoinflammatory diseases, pyrin-associated autoinflammatory diseases, NLRP3-related autoinflammatory diseases, undifferentiated systemic autoinflammatory disease, relopathies
Published in DiRROS: 08.04.2021; Views: 1429; Downloads: 1077
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10.
Evidence of B cell clonality and investigation into properties of the IgM in patients with Schnitzler syndrome
Shelly Pathak, Dorota Rowczenio, Samuel Lara-Reyna, Mark Kačar, Roger Owen, Gina Doody, Karoline Krause, Helen J Lachmann, Rainer Doffinger, Darren Newton, Sinisa Savic, 2020, original scientific article

Abstract: The Schnitzler Syndrome (SchS) is an acquired, autoinflammatory condition successfully treated with IL-1 inhibition. The two main defining features of this late-onset condition are neutrophilic urticarial dermatoses (NUD) and the presence of an IgM monoclonal component. While the former aspect has been extensively studied in this disease setting, the enigmatic paraproteinaemia and its potential consequential effects within SchS, has not previously been thoroughly addressed. Previous studies analyzing clonal B cell repertoires have largely focused on autoimmune disorders such as Systemic Lupus Erythematous (SLE) and hematological malignancies such as Chronic Lymphocytic Leukaemia (CLL), where B-cell clonality is central to disease pathology. The present study uses next-generation sequencing to provide detailed insight into aspects of B cell VDJ recombination and properties of the resulting immunoglobulin chains. An overview of IgH regional dynamics in 10 SchS patients, with a particular focus on CDR3 sequences and VDJ gene usage is reported, highlighting the presence of specific B cell expansions. Protein microarray detected a substantial proportion of autoreactive IgM to nuclear target proteins, though a single universal target was not identified. Together, these genetic and functional findings impart new understanding into this rare disorder.
Keywords: Schnitzler syndrome, B-lymhocytes, paraproteinemias, pararoteins, immunoglobulin M, autoinflammatory diseases, IgM
Published in DiRROS: 08.04.2021; Views: 1416; Downloads: 921
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