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1.
Foods ; 13(7)2024 Apr 02.
Article En | MEDLINE | ID: mdl-38611396

The agribusiness sector is constantly seeking solutions to enhance food security, sustainability, and resilience. Recent estimates indicate that one-third of the total food production remains unused due to waste or limited shelf life, resulting in negative environmental and ethical consequences. Consequently, exploring technological solutions to extend the shelf life of food products could be a crucial option to address this issue. However, the success of these technological solutions is closely linked to the perception of the end-consumers, particularly in the short term. Based on these considerations, this paper presents a systematic literature review of the main technological innovations in the fresh meat industry and of consumers' perceptions of such innovations. Regarding innovative technologies, this review focused on active and smart packaging. Amidst various technological innovations, including the utilization of fundamental matrices and natural additives, a noticeable gap exists in consumer perception studies. This study represents the first comprehensive compilation of research on consumers' perceptions and acceptance of innovations designed to extend the shelf life of fresh meat. Moreover, it sheds light on the existing barriers that hinder the complete embrace of these innovations.

2.
Heliyon ; 10(8): e28661, 2024 Apr 30.
Article En | MEDLINE | ID: mdl-38644900

The purpose of this paper is to thoroughly assess the value of colours in consumers' preferences for sweet peppers, and the association with more sustainable methods of production in the consumers' minds. Furthermore, this study provides novel insights into the influence of colours on the willingness to pay (WTP) for vegetables. It explores the interplay between colours, food attributes, and socio-demographic characteristics among consumers, marking the first attempt to examine this relationship comprehensively. A discrete choice experiment, based on consumers' preferences for sweet pepper attributes (price, colour, and production method), was implemented and a Seemingly Unrelated Regression model was then applied to evaluate the willingness to pay for different colours. The study has revealed that different colours provide consumers with different utilities and WTP in the choices of potential healthier foods. In addition, gender, age, education and number of family components play a role in affecting consumers' WTP of food linked to colour. Finally, it was found that consumers' knowledge for certain pepper colours with different antioxidant contents is interrelated. This study introduces several novelties, in particular a positive correlation between interest in antioxidants and colours was found, suggesting that interest in healthy food properties might move consumers towards a specific sensory choice.

3.
Front Nutr ; 11: 1338925, 2024.
Article En | MEDLINE | ID: mdl-38379543

Introduction: Women play a crucial role in food shopping and preparation, and their food choices have significant implications for their health and that of their families. This study aims to provide a perspective on women's eating lifestyle, which has undergone significant changes. Methods: A factor analysis was conducted to assess the degree of involvement in food choices and the types of food items consumed among a sample of 399 Italian women. Results: Through cluster analysis, four segments were identified: hedonic food consumers, sustainable- and balanced-diet consumers, food experimenters, and no food fondness consumers. The results reveal a correlation between the degree of food involvement and the type of food consumed. Discussion: Furthermore, the food lifestyle of the sample is partially dependent on age. Individuals aged 25-28 years show more hedonic food consumption behavior, while the older age group (44-64 years) falls into the sustainable and balanced diet consumer cluster (the largest cluster) and the cluster of those who do not express definable food choices (no food fondness).

4.
J Neurol Neurosurg Psychiatry ; 95(5): 434-441, 2024 Apr 12.
Article En | MEDLINE | ID: mdl-37918904

BACKGROUND: Shoe inserts, orthopaedic shoes, ankle-foot orthoses (AFOs) are important devices in Charcot-Marie-Tooth disease (CMT) management, but data about use, benefits and tolerance are scanty. METHODS: We administered to Italian CMT Registry patients an online ad hoc questionnaire investigating use, complications and perceived benefit/tolerability/emotional distress of shoe inserts, orthopaedic shoes, AFOs and other orthoses/aids. Patients were also asked to fill in the Quebec User Evaluation of Satisfaction with assistive Technology questionnaire, rating satisfaction with currently used AFO and related services. RESULTS: We analysed answers from 266 CMT patients. Seventy per cent of subjects were prescribed lower limb orthoses, but 19% did not used them. Overall, 39% of subjects wore shoe inserts, 18% orthopaedic shoes and 23% AFOs. Frequency of abandonment was high: 24% for shoe inserts, 28% for orthopaedic shoes and 31% for AFOs. Complications were reported by 59% of patients and were more frequently related to AFOs (69%). AFO users experienced greater emotional distress and reduced tolerability as compared with shoe inserts (p<0.001) and orthopaedic shoes (p=0.003 and p=0.045, respectively). Disease severity, degree of foot weakness, customisation and timing for customisation were determinant factors in AFOs' tolerability. Quality of professional and follow-up services were perceived issues. CONCLUSIONS: The majority of CMT patients is prescribed shoe inserts, orthopaedic shoes and/or AFOs. Although perceived benefits and tolerability are rather good, there is a high rate of complications, potentially inappropriate prescriptions and considerable emotional distress, which reduce the use of AFOs. A rational, patient-oriented and multidisciplinary approach to orthoses prescription must be encouraged.


Charcot-Marie-Tooth Disease , Humans , Charcot-Marie-Tooth Disease/therapy , Orthotic Devices , Lower Extremity , Shoes , Patient Acuity
6.
J Neurol ; 270(11): 5561-5568, 2023 Nov.
Article En | MEDLINE | ID: mdl-37540277

BACKGROUND: Sleep abnormalities have been reported in Charcot-Marie-Tooth disease (CMT), but data are scanty. We investigated their presence and correlation in a large CMT patients' series. METHODS: Epworth Sleepiness Scale (ESS) and Pittsburgh Sleep Quality Index (PSQI) were administered to CMT patients of the Italian registry and controls. ESS score > 10 indicated abnormal daytime somnolence, PSQI score > 5 bad sleep quality. We analyzed correlation with disease severity and characteristics, Hospital Anxiety and Depression Scale (HADS), Modified Fatigue Impact Scale (MFIS), Body Mass Index, drug use. RESULTS: ESS and PSQI questionnaires were filled by 257 and 253 CMT patients, respectively, and 58 controls. Median PSQI score was higher in CMT patients than controls (6 vs 4, p = 0.006), with no difference for ESS score. Abnormal somnolence and poor sleep quality occurred in 23% and 56% of patients; such patients had more frequently anxiety/depression, abnormal fatigue, and positive sensory symptoms than those with normal ESS/PSQI. Moreover, patients with PSQI score > 5 had more severe disease (median CMT Examination Score, CMTES, 8 vs 6, p = 0.006) and more frequent use of anxiolytic/antidepressant drugs (29% vs 7%, p < 0.001). CONCLUSIONS: Bad sleep quality and daytime sleepiness are frequent in CMT and correlated with anxiety, depression and fatigue, confirming that different components affect sleep. Sleep disorders, such as sleep apnea and restless leg syndrome, not specifically investigated here, are other factors known to impact on sleep quality and somnolence. CMT patients' management must include sleep behavior assessment and evaluation of its correlated factors, including general distress and fatigue.


Charcot-Marie-Tooth Disease , Disorders of Excessive Somnolence , Sleep Wake Disorders , Humans , Sleep Quality , Sleepiness , Charcot-Marie-Tooth Disease/complications , Disorders of Excessive Somnolence/etiology , Sleep , Fatigue/etiology , Surveys and Questionnaires , Sleep Wake Disorders/epidemiology , Sleep Wake Disorders/etiology
7.
Eur J Neurol ; 30(8): 2461-2470, 2023 08.
Article En | MEDLINE | ID: mdl-37170966

BACKGROUND AND PURPOSE: Data are reported from the Italian CMT Registry. METHODS: The Italian CMT Registry is a dual registry where the patient registers and chooses a reference center where the attending clinician collects a minimal dataset of information and administers the Charcot-Marie-Tooth (CMT) Examination/Neuropathy Score. Entered data are encrypted. RESULTS: Overall, 1012 patients had registered (535 females) and 711 had received a genetic diagnosis. Demyelinating CMT (65.3%) was more common than axonal CMT2 (24.6%) and intermediate CMT (9.0%). The PMP22 duplication was the most frequent mutation (45.2%), followed by variants in GJB1 and MPZ (both ~10%) and MFN2 (3.3%) genes. A relatively high mutation rate in some "rare" genes (HSPB1 1.6%, NEFL 1.5%, SH3TC2 1.5%) and the presence of multiple mutation clusters across Italy was observed. CMT4A was the most disabling type, followed by CMT4C and CMT1E. Disease progression rate differed depending on the CMT subtype. Foot deformities and walking difficulties were the main features. Shoe inserts and orthotic aids were used by almost one-half of all patients. Scoliosis was present in 20% of patients, especially in CMT4C. Recessive forms had more frequently walking delay, walking support need and wheelchair use. Hip dysplasia occurred in early-onset CMT. CONCLUSIONS: The Italian CMT Registry has proven to be a powerful data source to collect information about epidemiology and genetic distribution, clinical features and disease progression of CMT in Italy and is a useful tool for recruiting patients in forthcoming clinical trials.


Charcot-Marie-Tooth Disease , Female , Humans , Charcot-Marie-Tooth Disease/epidemiology , Charcot-Marie-Tooth Disease/genetics , Charcot-Marie-Tooth Disease/diagnosis , Mutation , Disease Progression , Italy/epidemiology
8.
Foods ; 12(10)2023 May 19.
Article En | MEDLINE | ID: mdl-37238877

Rural development is complex in marginal and disadvantaged areas, such as mountains, which impose high labour costs and restrict farmers in their choices of crop and livestock. To recognise this problem, the European Union regulates the use of the optional quality term "Mountain product" on the label. Consumers may recognise this label and be more willing to pay for it, resulting in higher revenues for producers using it. This study estimates the willingness to pay (WTP) for a mountain quality label. This WTP is then compared to that of functional and nutrition claims. For this purpose, we used a ranking conjoint experiment, using goat's milk yoghurt-a typical mountain product-as a case study. Using a rank-ordered logit, we show that mountain quality labels generate a significant WTP, higher than that of functional claims. WTP differs by the demographic profile of the consumer. The study provided useful insights about the combination of the mountain quality label with different attributes. However, future studies are needed to adequately understand the potential of mountain certification as a supporting tool for farmers in marginal areas and for rural development.

9.
Eur J Neurol ; 30(3): 710-718, 2023 03.
Article En | MEDLINE | ID: mdl-36458502

BACKGROUND AND PURPOSE: Fatigue, a disabling symptom in many neuromuscular disorders, has been reported also in Charcot-Marie-Tooth disease (CMT). The presence of fatigue and its correlations in CMT was investigated. METHODS: The Modified Fatigue Impact Scale (MFIS) was administered to CMT patients from the Italian Registry and a control group. An MFIS score >38 indicated abnormal fatigue. The correlation with disease severity and clinical characteristics, the Hospital Anxiety and Depression Scale and Epworth Sleepiness Scale scores, and drug use was analysed. RESULTS: Data were collected from 251 CMT patients (136 women) and 57 controls. MFIS total (mean ± standard deviation 32 ± 18.3, median 33), physical (18.9 ± 9.7, 20) and psychosocial (2.9 ± 2.4, 3) scores in CMT patients were significantly higher than controls. Abnormal fatigue occurred in 36% of the patients who, compared to patients with normal scores, had more severe disease (median CMT Examination Score 9 vs. 7), more frequent use of foot orthotics (22% vs. 11%), need of support for walking (21% vs. 8%), hand disability (70% vs. 52%) and positive sensory symptoms (56% vs. 36%). Patients with abnormal fatigue had significantly increased frequency of anxiety/depression/general distress (Hospital Anxiety and Depression Scale), somnolence (Epworth Sleepiness Scale), obesity (body mass index ≥ 30) and use of anxiolytic/antidepressant or anti-inflammatory/analgesic drugs. CONCLUSIONS: Fatigue is a relevant symptom in CMT as 36% of our series had scores indicating abnormal fatigue. It correlated with disease severity but also with anxiety, depression, sleepiness and obesity, indicating different components in the generation of fatigue. CMT patients' management must include treatment of fatigue and of its different generators, including general distress, sleepiness and obesity.


Charcot-Marie-Tooth Disease , Humans , Female , Charcot-Marie-Tooth Disease/complications , Charcot-Marie-Tooth Disease/epidemiology , Sleepiness , Walking , Fatigue/epidemiology , Fatigue/etiology , Upper Extremity
10.
J Neurol ; 270(1): 394-401, 2023 Jan.
Article En | MEDLINE | ID: mdl-36114297

BACKGROUND: There is little information about neuropsychiatric comorbidities in Charcot-Marie-Tooth disease (CMT). We assessed frequency of anxiety, depression, and general distress in CMT. METHODS: We administered online the Hospital Anxiety-Depression Scale (HADS) to CMT patients of the Italian registry and controls. HADS-A and HADS-D scores ≥ 11 defined the presence of anxiety/depression and HADS total score (HADS-T) ≥ 22 of general distress. We analysed correlation with disease severity and clinical characteristics, use of anxiolytics/antidepressants and analgesic/anti-inflammatory drugs. RESULTS: We collected data from 252 CMT patients (137 females) and 56 controls. CMT patient scores for anxiety (mean ± standard deviation, 6.7 ± 4.8), depression (4.5 ± 4.0), and general distress (11.5 ± 8.1) did not differ from controls and the Italian population. However, compared to controls, the percentages of subjects with depression (10% vs 2%) and general distress (14% vs 4%) were significantly higher in CMT patients. We found no association between HADS scores and disease duration or CMT type. Patients with general distress showed more severe disease and higher rate of positive sensory symptoms. Depressed patients also had more severe disease. Nineteen percent of CMT patients took antidepressants/anxiolytics (12% daily) and 70% analgesic/anti-inflammatory drugs. Patients with anxiety, depression, and distress reported higher consumption of anxiolytics/antidepressants. About 50% of patients with depression and/or general distress did not receive any specific pharmacological treatment. CONCLUSIONS: An appreciable proportion of CMT patients shows general distress and depression. Both correlated with disease severity and consumption of antidepressants/anxiolytics, suggesting that the disease itself is contributing to general distress and depression.


Anti-Anxiety Agents , Charcot-Marie-Tooth Disease , Female , Humans , Charcot-Marie-Tooth Disease/complications , Charcot-Marie-Tooth Disease/epidemiology , Depression/epidemiology , Depression/diagnosis , Anti-Anxiety Agents/therapeutic use , Anxiety/epidemiology , Registries , Italy/epidemiology , Antidepressive Agents/therapeutic use
11.
J Clin Med ; 11(23)2022 Nov 28.
Article En | MEDLINE | ID: mdl-36498606

The present study aims to investigate the benefits induced by physical activity/practiced sport in Charcot-Marie-Tooth 1A (CMT1A). Patients were divided into sport and no-sport groups according to their sports performance habit. Thirty-one patients were enrolled, of which 14 practiced sports and 17 did not. Clinical assessments were administered to evaluate disability, self-esteem, depression, quality of life, and pain. Statistical analysis revealed significant differences in terms of gender in the no-sport group compared to the sport group (p = 0.04). Regarding the quality of life, physical function (p = 0.001), general health (p = 0.03), social function (p = 0.04), and mental health (p = 0.006) showed better patterns in the sport group than no-sport group. Moreover, neuropathic pain was reduced in the sport group according to the Neuropathic Pain Symptom Inventory (p = 0.001) and ID-PAIN (p = 0.03). The other administered questionnaires showed no significant differences. Our study confirms that CMT1A patients, who practice sports, with a similar severity of disability, may have a better physical quality of life while suffering less neuropathic pain than their peers who do not practice sports. Results recommend the prescription of sport in CMT1A patients.

12.
Neurol Sci ; 43(12): 6639-6655, 2022 Dec.
Article En | MEDLINE | ID: mdl-36138296

AIMS: The objective of the present study was to evaluate sleep features and sleep-modifying factors in patients with chronic migraine (CM) during the first Italian COVID-19 lockdown. MATERIAL AND METHODS: The study was based on an e-mail survey addressed to CM patients of our headache center. The survey investigated demographic, life-style, sleep, psychological, and migraine features during the first COVID-19 lockdown period and the month before. The outcomes were sleep quality (measured using PSQI) and variation in sleep quality, duration, and latency. RESULTS: Ninety-two patients were included. The mean PSQI was 11.96. Sleep quality was improved in 14.1%, stable in 47.8%, and worsened in 38.0%. Sleep latency was reduced in 5.4%, stable in 46.7%, and increased in 47.8%. Sleep duration was reduced in 29.3%, stable in 34.8%, and increased in 35.9%. Significant associations were found with age, work/study, remote working, job loss, meal quality change, smoking variation, COVID-19 province prevalence, home-inhabitant relationship, ratio of house size/number of people, stress, state anxiety, anxiety/depression variation, future concern variation, computer hours, internet hours, and television hours. CONCLUSION: The study described sleep features of chronic migraineurs during COVID-19 lockdown, pinpointing the main factors involved in sleep quality and sleep changes. Our findings revealed that migraineurs' sleep was closely linked with life-style and psychological features. Several modifiable factors came to light and they should be considered in order to develop an optimal management of CM. An appropriate and more aware treatment of sleep problems could be a way to improve migraineurs' life.


COVID-19 , Migraine Disorders , Humans , Communicable Disease Control , Migraine Disorders/drug therapy , Sleep/physiology , Anxiety/epidemiology
13.
Handb Clin Neurol ; 189: 259-270, 2022.
Article En | MEDLINE | ID: mdl-36031308

In amyotrophic lateral sclerosis (ALS), Guillain-Barré syndrome (GBS), and neuromuscular junction disorders, three mechanisms may lead, singly or together, to respiratory emergencies and increase the disease burden and mortality: (i) reduced strength of diaphragm and accessory muscles; (ii) oropharyngeal dysfunction with possible aspiration of saliva/bronchial secretions/drink/food; and (iii) inefficient cough due to weakness of abdominal muscles. Breathing deficits may occur at onset or more often along the chronic course of the disease. Symptoms and signs are dyspnea on minor exertion, orthopnea, nocturnal awakenings, excessive daytime sleepiness, fatigue, morning headache, poor concentration, and difficulty in clearing bronchial secretions. The "20/30/40 rule" has been proposed to early identify GBS patients at risk for respiratory failure. The mechanical in-exsufflator is a device that assists ALS patients in clearing bronchial secretions. Noninvasive ventilation is a safe and helpful support, especially in ALS, but has some contraindications. Myasthenic crisis is a clinical challenge and is associated with substantial morbidity including prolonged mechanical ventilation and 5%-12% mortality. Emergency room physicians and consultant pulmonologists and neurologists must know such respiratory risks, be able to recognize early signs, and treat properly.


Amyotrophic Lateral Sclerosis , Guillain-Barre Syndrome , Myasthenia Gravis , Neuromuscular Diseases , Neuromuscular Junction Diseases , Respiratory Insufficiency , Humans , Motor Neurons , Peripheral Nerves
14.
Amyloid ; 29(3): 197-204, 2022 Sep.
Article En | MEDLINE | ID: mdl-35465808

BACKGROUND: Cardiac amyloidosis (CA) is cardiomyopathy with a hypertrophic phenotype characterised by diffuse deposition of anomalous fibrillar proteins in the extracellular matrix. OBJECTIVES: To evaluate the prevalence and diagnostic value of extra left ventricle echocardiographic findings in patients with left ventricular (LV) hypertrophic phenotype and amyloid deposition. METHODS: A group of 146 patients with LV thickness ≥15 mm were enrolled: 70 patients who received a definite diagnosis of sarcomeric hypertrophic cardiomyopathy (HCM group) and 76 patients with transthyretin cardiac amyloidosis (CA group). Echocardiographic analysis of crista terminalis (CriT), atrio-ventricular plane (AVP), mitro-aortic lamina (MAL), anterior ascending aortic wall, interatrial septum (IAS), Eustachian valve (EusV) and coumadin ridge (CouR) was performed in all patients, and these structures were compared among the two groups. RESULTS: CA group showed significantly higher dimensions of CriT, IAS, CouR, AVP, MAL and IAS compared to the HCM group. The logistic analysis showed that LV EF, LV septal thickness, CriT presence, CriT area, MAL and IAS were all predictors of CA in univariate analyses. The stepwise multivariate analysis showed independent predictors of CA: CriT area, MAL and LVEF. According to areas under the receiver operating characteristic curves the best cut-off values to determine CA were identified (IAS > 9 mm, MAL > 7 mm, CriT > 9 mm2). Among these 3 independent predictors, IAS > 9 mm had the best specificity (96%) and positive predictive value (93%) in identifying CA. CONCLUSIONS: evidence of extra left ventricle sites of amyloid deposition is a frequent finding in CA. In the context of hypertrophic phenocopies, an increased thickness of IAS, and/or CT and/or MAL should suggest a diagnosis of transthyretin CA.


Amyloidosis , Cardiomyopathy, Hypertrophic , Amyloidosis/diagnostic imaging , Amyloidosis/epidemiology , Cardiomyopathy, Hypertrophic/diagnostic imaging , Cardiomyopathy, Hypertrophic/epidemiology , Cardiomyopathy, Hypertrophic/genetics , Echocardiography , Heart Ventricles/diagnostic imaging , Humans , Prealbumin/genetics , Prevalence
15.
Nutrients ; 14(8)2022 Apr 15.
Article En | MEDLINE | ID: mdl-35458217

This study aims to examine Italian consumer preferences for extra virgin olive oil (EVOO) enriched with vitamins and to analyze the key drivers that affect consumer choices for this product. Specifically, we assessed consumers' intention to purchase the enriched product compared to the conventional one. The methodology adopted inferential and multivariate statistical techniques: (1) exploratory factor analysis (EFA), (2) ordinary least squares regression (OLS) and (3) non-hierarchical clustering. This study appears to be the first research project related to exploring consumers' interest in an extra virgin olive oil enhanced with vitamins, thereby providing preliminary indications. The main results represent a significant starting point for the development of new marketing strategies for the food industry.


Consumer Behavior , Intention , Attitude , Olive Oil , Vitamins
16.
Int J Food Sci Nutr ; 73(6): 709-725, 2022 Sep.
Article En | MEDLINE | ID: mdl-35264074

Food-related diseases are a topic of concern for the population and institutions; therefore, regulations have been envisaged to improve consumer knowledge about food properties and to guide them towards healthy food consumption. Europe has addressed the use of claims on labels with two important regulations in 2006 and 2012. The use of claims allows for the differentiation of food products, particularly functional foods, i.e., those that can provide health benefits as part of a standard diet. The study aims to describe the interest of researchers on the topic of functional foods and health claims by observing the trend of publications and highlighting the role of the country on the topic through a network analysis. Another objective is to evaluate the role of lifestyle, psychological and socio-demographic predictors in the choice of functional foods bearing label claims or not, using a systematic literature review. To this aims, a bibliometric and a network analysis were assessed. The results reveal a positive trend in publications and a growing interest in the topic, thus showing that socio-demographic characteristics and lifestyle variables partially influence consumer behaviour. In particular, it emerges that educated people and women are more interested in functional foods, showing a higher willingness to pay for them. In addition, physical activity and beliefs regarding the nutritional quality of functional foods are positively correlated with WTP. However, several contradictory results were observed for some variables in the collected studies. For these reasons and considering the high number of functional foods and claims yet to be evaluated, further investigations are recommended.


Food Preferences , Functional Food , Bibliometrics , Consumer Behavior , Demography , Female , Food Labeling , Humans , Life Style
18.
Eur Radiol ; 32(5): 3035-3044, 2022 May.
Article En | MEDLINE | ID: mdl-35031838

OBJECTIVES: Although expert consensus recommendations suggest 2-3 h as the time interval between bone-seeking radiotracers injection and acquisition, it has been reported that images obtained early after [99mTc]Tc-HMDP administration are sufficient to diagnose cardiac amyloidosis. We evaluated the diagnostic performance of [99mTc]Tc-DPD early phase whole body scan with respect to late phase imaging. METHODS: We qualitatively and semiquantitatively reviewed [99mTc]Tc-DPD imaging of 53 patients referred for suspect cardiac amyloidosis. Findings of early and late phase images were compared with SPECT results (considered the standard-of-reference) determining sensitivity and specificity for visual analysis of each phase imaging and for each semiquantitative index. RESULTS: SPECT imaging was negative for cardiac accumulation in 25 patients and positive in 28. Visual analysis of early phase whole body scan had an extremely significant capability to predict SPECT results; nevertheless, complete agreement was not reached. Visual analysis of late phase imaging showed slightly better results. Semiquantitative analysis of early phase images, namely heart to mediastinum ratio, performed better than semiquantitative analysis of late phase images. CONCLUSION: Visual analysis of [99mTc]Tc-DPD early phase whole body scan is promising in diagnosing cardiac amyloidosis; further studies are needed to confirm our results in different clinical scenarios. KEY POINTS: • Visual analysis of early phase planar imaging using [99mTc]Tc-DPD is accurate to diagnose cardiac amyloidosis and may be satisfactory at least in frail patients with high cardiac burden of amyloid fibrils.


Amyloidosis , Cardiomyopathies , Amyloid , Amyloidosis/diagnostic imaging , Cardiomyopathies/diagnostic imaging , Humans , Prealbumin , Radionuclide Imaging , Radiopharmaceuticals/pharmacology , Whole Body Imaging
19.
J Endocrinol Invest ; 45(3): 517-525, 2022 Mar.
Article En | MEDLINE | ID: mdl-34524678

PURPOSE: An increased fracture risk is commonly reported in Duchenne muscular dystrophy (DMD). Our aim was to investigate bone mineral density (BMD) and bone turnover, including sclerostin, and their association with markers of cardiac and respiratory performance in a cohort of DMD subjects. METHODS: In this single center, cross sectional observational study, lumbar spine (LS) BMD Z-scores, C-terminal telopeptide of procollagen type I (CTX) and osteocalcin (BGP), as bone resorption and formation markers, respectively, and sclerostin were assessed. Left ventricular ejection fraction (LVEF) and forced vital capacity (FVC) were evaluated. Clinical prevalent fractures were also recorded. RESULTS: Thirty-one patients [median age = 14 (12-21.5) years] were studied. Ambulant subjects had higher LS BMD Z-scores compared with non-ambulant ones and subjects with prevalent clinical fractures [n = 9 (29%)] showed lower LS BMD Z-scores compared with subjects without fractures. LS BMD Z-scores were positively correlated with FVC (r = 0.50; p = 0.01), but not with glucocorticoid use, and FVC was positively associated with BGP (r = 0.55; p = 0.02). In non-ambulant subjects, LS BMD Z-scores were associated with BMI (r = 0.54; p = 0.02) and sclerostin was associated with age (r = 0.44; p = 0.05). Age, BMI, FVC and sclerostin were independently associated with LS BMD Z-score in a stepwise multiple regression analysis. Older age, lower BMI, FVC and sclerostin were associated with lower LS BMD Z-scores. CONCLUSION: In a cohort of DMD patients, our data confirm low LS BMD Z-scores, mainly in non-ambulant subjects and irrespective of the glucocorticoid use, and suggest that FVC and sclerostin are independently associated with LS BMD Z-scores.


Adaptor Proteins, Signal Transducing/metabolism , Collagen Type I/metabolism , Fractures, Bone , Glucocorticoids/therapeutic use , Muscular Dystrophy, Duchenne , Peptides/metabolism , Ventricular Dysfunction, Left , Adolescent , Biomarkers/metabolism , Bone Density , Bone Remodeling , Fractures, Bone/epidemiology , Fractures, Bone/etiology , Fractures, Bone/prevention & control , Humans , Italy/epidemiology , Lumbar Vertebrae/diagnostic imaging , Lumbar Vertebrae/pathology , Mobility Limitation , Muscular Dystrophy, Duchenne/diagnosis , Muscular Dystrophy, Duchenne/drug therapy , Muscular Dystrophy, Duchenne/metabolism , Muscular Dystrophy, Duchenne/physiopathology , Stroke Volume , Ventricular Dysfunction, Left/diagnosis , Ventricular Dysfunction, Left/etiology , Vital Capacity
20.
Neurol Sci ; 42(11): 4403-4418, 2021 Nov.
Article En | MEDLINE | ID: mdl-34365547

AIMS: The objective of this study was to evaluate the impact of the first Italian COVID-19 lockdown on patients with chronic migraine (CM). MATERIAL AND METHODS: The study was based on an e-mail survey addressed to CM patients of our headache center. The survey evaluated demographic, life style, sleep, psychological, and migraine features during the COVID-19 lockdown period and the month before. The outcomes were migraine impact on daily life and variation in attack frequency, attack duration, migraine pain intensity, migraine symptomatic drugs use per week, and efficacy. RESULTS: Ninety-two patients completed the survey. During the lockdown period, attack frequency was stable in 40,2%, increased in 33,7%, and reduced in 26,1% of patients; attack duration was stable in 55,4%, increased in 23,9%, and reduced in 20,7%. Migraine pain was stable or reduced in 65,2% and increased in 34,8%; number of symptomatic drugs per week was stable in 50%, reduced in 29,3%, and increased in 20,7%; migraine drug efficacy was stable in 73,9%, reduced in 17,4%, and increased in 8,7%. Patients had a HIT-6 score of 64,63 ± 8,81. Significant associations were found with remote working, smoke, education, discontinuation of the therapy performed within headache center, migraine familiarity, sleep, anxiety, perceived stress, concern about future, and COVID-19. CONCLUSION: During the lockdown, approximately half of the patients had a clinical stability, a quarter an improvement, and another quarter a worsening. We identified different migraine-influencing elements; in particular, the remote working could represent an easy way to ameliorate migraineurs' life.


COVID-19 , Migraine Disorders , Communicable Disease Control , Humans , Migraine Disorders/epidemiology , SARS-CoV-2 , Sleep , Teleworking
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