1,721,004 research outputs found
The heart in rare endocrine diseases: cardiometabolic studies in Cushing’s syndrome and acromegaly
Introduction: Cushing’s syndrome (CS) and acromegaly are two rare endocrine diseases associated to increased morbidity and mortality, mainly for cardiovascular events. Cardiac magnetic resonance (CMR) is the established non-invasive gold standard method for measuring (left ventricle) LV volume, cardiac function and LV mass (LVM) due to its higher accuracy and reproducibility and lower variability in comparison with echocardiography.
Aim: The aim of the current study was to evaluate the metabolic profile and to perform a cardiological study through CMR in patients with CS and acromegaly.
Materials and methods: This was a prospective multicentric case-control study. Consecutive patients with CS and acromegaly, both cured and with active disease, entered the study. The control group included patients with non-functioning adrenal incidentaloma matched with patients for sex, age and BMI. Metabolic and clinical parameters and CMR parameters have been compared between patients and controls.
Results: Sixteen patients with CS, 20 patients with acromegaly and 18 controls entered the study. Fasting glucose levels were significantly lower in CS patients than controls (p=0.003), whereas they were significantly higher in acromegaly patients than controls (p=0.033). No significant differences were found neither in lipid levels nor in systolic and diastolic blood pressure levels between patients and controls. No significant differences in the prevalence of
cardiometabolic complications were found between patients and controls. LV end-systolic volume (LV-ESV) and LV-ESV indexed with respect to the body surface (LV-ESVi) were significantly higher in CS patients than controls (p=0.041; p=0.030). Right ventricle enddiastolic volume (RV-EDV), RV-EDVi, RV-ESV and RV-ESVi were significantly higher in CS patients than controls (p=0.025; p=0.033; p=0.004; p=0.008). LV-EDV, LV-EDVi, LV-ESV and LV-ESV were significantly and markedly higher in acromegaly patients than controls (p=0.001; p=0.003; p=0.001; p=0.001). LVM was significantly higher in acromegaly patients 3 than controls (p=0.002). RV-EDV, RV-EDVi, RV-ESV and RV-ESVi were significantly and markedly higher in acromegaly patients than controls (p=0.000; p=0.001; p=0.000; p=0.000). Consequently, acromegaly patients had a significantly lower RV-EF than controls (p=0.002). Moreover, a significant correlation was found between IGF-1 levels and cardiac parameters at CMR in acromegaly patients.
Conclusions: CS and acromegaly have been demonstrated to have biventricular cardiac structural and functional impairment at CMR, which seem to have a multifactorial pathogenesis: the presence of disease-related cardiovascular risk factors and a direct effect of hormone excess. The results of the current studies suggest that CMR may have a place in the cardiac work-up of selected patients with rare endocrine diseases, such as CS and acromegaly
Quality of Life in Patients with Cushing's Disease: A Modern Approach
Cushing’s disease (CD) is known to be associated with an increased mortality especially for
cardiovascular diseases (1,2). Even years after cure, patients with a history of CD maintain an
increased cardiovascular risk (3). The most important and frequent systemic complication in CD is
represented by the metabolic syndrome, namely the combination of visceral obesity, systemic
arterial hypertension, glucose intolerance and dyslipidemia, which together with thrombosis
diathesis, is responsible for the increased cardiovascular risk (2,4). Glucocorticoids excess exerts
important effects also on the skeletal system, determining osteoporosis, and on gonadal function,
inducing polycystic ovary syndrome and menstrual disturbances in women or sexual dysfunction in
men, with consequent damage of fertility. Overall, these complications induce significantly
impaired health-related quality of life (HRQoL) which persists even after resolution of cortisol
excess (2). Patients mainly complain of fatigue or weakness, changes in physical appearance,
emotional instability, cognitive problems, depression and also sleeping difficulties; the majority of
patients report interference with family life and relations with their partner and half of them with
school or work performance (5). Despite successful treatment of CD, long-term residual effect on
the HRQoL has been observed, which includes poorer physical and social functioning, role
limitations due to physical and emotional problems, more pain, and less general wellbeing (5). The
mechanisms through which CD determines HRQoL impairment are probably multifactorial
involving physical and psychological features. Severe fatigability and changes in body composition
and image, anxiety, irritability, mood swings, depression, decreased memory, less self-confidence,
and difficulties in sleeping are common and the main concerns for these patients (5)
Role of FGF System in Neuroendocrine Neoplasms. Potential Therapeutic Applications
Neuroendocrine neoplasms (NENs) are a heterogeneous group of tumors originating from neuroendocrine cells dispersed in different organs. Receptor tyrosine kinases are a subclass of tyrosine kinases with a relevant role in several cellular processes including proliferation, differentiation, motility and metabolism. Dysregulation of these receptors is involved in neoplastic development and progression for several tumors, including NENs. In this review, we provide an overview concerning the role of the fibroblast growth factor (FGF)/fibroblast growth factor receptor (FGFR) system in the development and progression of NENs, the occurrence of fibrotic complications and the onset of drug-resistance. Although no specific FGFR kinase inhibitors have been evaluated in NENs, several clinical trials on multitarget tyrosine kinase inhibitors, acting also on FGF system, showed promising anti-tumor activity with an acceptable and manageable safety profile in patients with advanced NENs. Future studies will need to confirm these issues, particularly with the development of new tyrosine kinase inhibitors highly selective for FGFR
Disturbios endocrinos e metabolicos variados
Si descrive il caso clinico di un giovane paziente con sospetta Sindrome di Cushing da secrezione ectopica di ACT
The Treatment of Cushing's Disease.
Cushing's disease (CD), or pituitary-dependent Cushing's syndrome, is a severe endocrine disease caused by a corticotroph pituitary tumor and associated with increased morbidity and mortality. The first-line treatment for CD is pituitary surgery, which is followed by disease remission in around 78% and relapse in around 13% of patients during the 10-year period after surgery, so that nearly one third of patients experience in the long-term a failure of surgery and require an additional second-line treatment. Patients with persistent or recurrent CD require additional treatments, including pituitary radiotherapy, adrenal surgery, and/or medical therapy. Pituitary radiotherapy is effective in controlling cortisol excess in a large percentage of patients, but it is associated with a considerable risk of hypopituitarism. Adrenal surgery is followed by a rapid and definitive control of cortisol excess in nearly all patients, but it induces adrenal insufficiency. Medical therapy has recently acquired a more important role compared to the past, due to the recent employment of novel compounds able to control cortisol secretion or action. Currently, medical therapy is used as a presurgical treatment, particularly for severe disease; or as postsurgical treatment, in cases of failure or incomplete surgical tumor resection; or as bridging therapy before, during, and after radiotherapy while waiting for disease control; or, in selected cases, as primary therapy, mainly when surgery is not an option. The adrenal-directed drug ketoconazole is the most commonly used drug, mainly because of its rapid action, whereas the glucocorticoid receptor antagonist, mifepristone, is highly effective in controlling clinical comorbidities, mainly glucose intolerance, thus being a useful treatment for CD when it is associated with diabetes mellitus. Pituitary-directed drugs have the advantage of acting at the site responsible for CD, the pituitary tumor. Among this group of drugs, the dopamine agonist cabergoline and the somatostatin analog pasireotide result in disease remission in a consistent subgroup of patients with CD. Recently, pasireotide has been approved for the treatment of CD when surgery has failed or when surgery is not an option, and mifepristone has been approved for the treatment of Cushing's syndrome when associated with impairment of glucose metabolism in case of the lack of a surgical indication. Recent experience suggests that the combination of different drugs may be able to control cortisol excess in a great majority of patients with CD
Is diabetes in Cushing's syndrome only a consequence of hypercortisolism?
OBJECTIVE:
Diabetes mellitus (DM) is one of the most frequent complications of Cushing's syndrome (CS). The aim of this study was to define the changes in insulin sensitivity and/or secretion in relation to glucose tolerance categories in newly diagnosed CS patients.
DESIGN:
Cross-sectional study on 140 patients with CS.
METHODS:
A total of 113 women (80 with pituitary disease and 33 with adrenal disease, aged 41.7±15.7 years) and 27 men (19 with pituitary disease and eight with adrenal disease, aged 38.1±20.01 years) at diagnosis were divided according to glucose tolerance into normal glucose tolerance (CS/NGT), impaired fasting glucose and/or impaired glucose tolerance (CS/prediabetes), and diabetes (CS/DM) groups.
RESULTS:
Seventy-one patients had CS/NGT (49.3%), 26 (18.5%) had CS/prediabetes and 43 (30.8%) had CS/DM. Significant increasing trends in the prevalence of family history of diabetes (P<0.001), metabolic syndrome (P<0.001), age (P<0.001) and waist circumference (P=0.043) and decreasing trends in HOMA-β (P<0.001) and oral disposition index (DIo) (P<0.002) were observed among the groups. No significant trends in fasting insulin levels, area under the curve for insulin (AUCINS), Matsuda index of insulin sensitivity (ISI-Matsuda) and visceral adiposity index were detected.
CONCLUSIONS:
Impairment of glucose tolerance is characterized by the inability of β-cells to adequately compensate for insulin resistance through increased insulin secretion. Age, genetic predisposition and lifestyle, in combination with the duration and degree of hypercortisolism, strongly contribute to the impairment of glucose tolerance in patients with a natural history of CS. A careful phenotypic evaluation of glucose tolerance defects in patients with CS proves useful for the identification of those at a high risk of metabolic complications
Going Beyond Counting First Authors in Author Co-citation Analysis
The present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Edible Flowers Used in Some Countries of the Mediterranean Basin: An Ethnobotanical Overview
Edible flowers are becoming an essential component of people’s nutrition in the Mediterranean basin. In the last decades, many researchers also have focused their attention on the nutritional composition of the edible flowers, as well as their antioxidant and antimicrobial properties, including studies on their safety issues. Despite the growing interest in the use of flowers in human nutrition, the ethnobotanical literature is lacking coverage of some important issues, particularly those which concern the use of flowers in the folk tradition. Only recently, a review regarding the contribution of 32 edible flowers to the Mediterranean diet was published. The aim of the present review is to document the plant lore regarding the wild and cultivated edible flowers consumed in the Mediterranean basin. Based on the 112 studies reviewed, we documented 251 taxa as being used in the Mediterranean basin as edible flowers. The plant species belong to 45 families and 141 genera. Asteraceae (54 taxa) is the most frequently cited family. Sambucus nigra L. is the most cited species. This study can be the basis for future research on the supposed bioactivity and toxicity of wild and cultivated flowers
Variations on the Author
“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
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