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1.
Pediatric Surgery International - Since Hirschsprung’s disease (HSCR) already proved to benefit from robotic surgery, we aimed at describing a wider series of patients with this rare disease...  相似文献   
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Journal of Neurology - To investigate the prognostic role and the major determinants of serum phosphorylated neurofilament heavy -chain (pNfH) concentration across a large cohort of motor neuron...  相似文献   
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Background

Scientific guidelines consider atrial fibrillation (AF) complicating degenerative mitral regurgitation (DMR) a debated indication for surgery.

Objectives

This study analyzed the prognostic/therapeutic implications of AF at DMR diagnosis and long-term.

Methods

Patients were enrolled in the MIDA (Mitral Regurgitation International Database) registry, which reported the consecutive, multicenter, international experience with DMR due to flail leaflets echocardiographically diagnosed.

Results

Among 2,425 patients (age 67 ± 13 years; 71% male, 67% asymptomatic, ejection fraction 64 ± 10%), 1,646 presented at diagnosis with sinus rhythm (SR), 317 with paroxysmal AD, and 462 with persistent AF. Underlying clinical/instrumental characteristics progressively worsened from SR to paroxysmal to persistent AF. During follow-up, paroxysmal and persistent AF were associated with excess mortality (10-year survival in SR and in paroxysmal and persistent AF was 74 ± 1%, 59 ± 3%, and 46 ± 2%, respectively; p < 0.0001), that persisted 20 years post-diagnosis and independently of all baseline characteristics (p values <0.0001). Surgery (n = 1,889, repair 88%) was associated with better survival versus medical management, regardless of all baseline characteristics and rhythm (adjusted hazard ratio: 0.26; 95% confidence interval: 0.23 to 0.30; p < 0.0001) but post-surgical outcome remained affected by AF (10-year post-surgical survival in SR and in paroxysmal and persistent AF was 82 ± 1%, 70 ± 4%, and 57 ± 3%, respectively; p < 0.0001).

Conclusions

AF is a frequent occurrence at DMR diagnosis. Although AF is associated with older age and more severe presentation of DMR, it is independently associated with excess mortality long-term after diagnosis. Surgery is followed by improved survival in each cardiac rhythm subset, but persistence of excess risk is observed for each type of AF. Our study indicates that detection of AF, even paroxysmal, should trigger prompt consideration for surgery.  相似文献   
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Although breast implant‐associated anaplastic large cell lymphoma (BIA‐ALCL) is a rare disease, its incidence has been increasing. The aim of this study was to assess the risk of BIA‐ALCL in women with breast implants. A systematic search was carried out in Pubmed, Scopus, ScienceDirect, LIVIVO, Cochrane Library, Google Scholar, and OpenGrey databases. The risk assessment of bias was based on the Newcastle‐Ottawa Scale. The rarity of BIA‐ALCL was a major limitation. Although we have found evidence of an increased risk of BIA‐ALCL, further studies are needed to understand why some large samples did not present any case of the disease.  相似文献   
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The present study was conducted to evaluate the validity of the Food Frequency Questionnaire (FFQ) used in the RPS Birth Cohort Consortium (Ribeirão Preto, Pelotas, and São Luís) to assess dietary intake of adolescents from São Luís, Maranhão. The research was developed with 152 adolescents aged 18 and 19 years. For the validation of the FFQ, the average of three 24-hour recalls (24HRs) was used as the reference method. The mean and standard deviation of energy and nutrient intake extracted from the surveys were estimated. The paired Student''s t-test was used to verify the differences between the instruments. Pearson correlation coefficient, intraclass correlation coefficient (ICC), weighted Kappa, and the Bland-Altman plot were calculated in order to measure the agreement. The study adopted a level of significance <5%. Compared with the three 24HRs, the FFQ overestimated the consumption of most nutrients. Energy-adjusted and de-attenuated concordance Pearson correlation coefficients ranged from 0.06 to 0.43, and correlations were significant for iron, calcium, riboflavin, sodium, saturated fat, niacin, and vitamin C. The energy-adjusted and de-attenuated ICCs ranged from 0.01 to 0.31, and the weighted Kappa ranged from 0.01 to 0.46. The analyses of agreement were significant for vitamin C, fiber, calcium, riboflavin, niacin, sodium, lipids, and iron. In conclusion, the FFQ presented acceptable relative validity for lipids, saturated fatty acids, fiber, calcium, iron, riboflavin, niacin, vitamin C, and sodium. This instrument will be useful in studies about food consumption of adolescents in São Luís, Maranhão.  相似文献   
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Anaplastic thyroid carcinoma (ATC) is an aggressive thyroid malignancy with high mortality rate. This malignancy arises in thyroid follicular cells either denovo or with an associated differentiated thyroid carcinoma component. Clinically, it usually presents as a rapidly enlarging mass, pain and locally compressive symptoms. Histopathologic variability and heterogeneity often pose diagnostic challenges, especially in scant and paucicellular specimens. This article describes the clinical, histopathologic and molecular features of ATC and also addresses the associated diagnostic limitations and challenges.  相似文献   
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