Submitted by Automa??o e Estat?stica (sst@bczm.ufrn.br) on 2017-01-03T20:58:46Z No. of bitstreams: 1 MorganaDeAraujoEvangelista_DISSERT.pdf: 2607941 bytes, checksum: 7cff1386828e64e1f63b7ce32b375bb0 (MD5) / Approved for entry into archive by Arlan Eloi Leite Silva (eloihistoriador@yahoo.com.br) on 2017-01-09T14:10:37Z (GMT) No. of bitstreams: 1 MorganaDeAraujoEvangelista_DISSERT.pdf: 2607941 bytes, checksum: 7cff1386828e64e1f63b7ce32b375bb0 (MD5) / Made available in DSpace on 2017-01-09T14:10:37Z (GMT). No. of bitstreams: 1 MorganaDeAraujoEvangelista_DISSERT.pdf: 2607941 bytes, checksum: 7cff1386828e64e1f63b7ce32b375bb0 (MD5) Previous issue date: 2015-06-17 / INTRODU??O: A Distrofia Miotonica ? caracterizada por atrofia, fraqueza presen?a de miotonia nos m?sculos esquel?ticos. A presen?a de miotonia nos musculos respirat?rios ? duvidosa assim como as t?cnicas para avalia-l?s. OBJETIVOS: Avaliar a sensibilidade /especificidade da taxa m?xima de relaxamento dos m?sculos inspirat?rios (MRR), a eletromiografia de superf?cie (EMGs) dos m?sculos esternocleidomast?ideo (SCM), escaleno (ESC), paraesternal (2EIC), reto abdominal (RA) e a fun??o pulmonar/muscular respiratoria em pacientes com DM1 e sujeitos saudaveis. M?TODOS: Foram convidados a participar do estudo, 71 indiv?duos, 44 pacientes com DM1 e 27 controles. Foram inclu?dos no estudo 28 sujeitos, (18 DM1 e 10 controles). Ap?s exlcus?es a amostra final foi de 25 sujeitos, 16 pacientes com DM1 e 9 sujeitos saud?veis. Todos foram avaliados em rela??o MRR dos m?sculos inspirat?rios, (dP/dt)/Psniff*100(%10ms), a EMGs dos m?sculos respirat?rios, ? fun??o muscular e pulmonar. RESULTADOS: A MRR foi menor nos pacientes com DM1 vs. controle (p=0,003) e foi considerada sens?vel e espec?fica para identificar a doen?a na DM1 e descart?-la no grupo controle, ?rea de ROC 0,87 (95%IC, 0,729 a 1,01, p=0,003). Foi observado valores reduzidos de PIm?x (p=0,0029), PEm?x (p=0,0007) e SNIP (p=0,0030), CVF%pred. (p=0,0014) e VEF1%pred. (p=0,0003) e maior atividade da EMGs em repouso nos m?sculos SCM (p=0,004), ESC (p=0,009) e RA (p=0,045) e no m?sculo SCM (p=0,001) durante o sniff teste. CONCLUS?ES: A MRR ? sensivel e especifica para identificar atraso no relaxamento dos musculos respirat?rios e a fun??o muscular respirat?ria encontra-se alterada nos pacientes com DM1. / INTRODUCTION: Myotonic dystrophy (DM) is a neuromuscular disease characterized by unstable expansion of CTG triplet on chromosome 19. It has two forms: type 1 (DM1 or Steinert's disease); and type 2 (DM2), being the former the most frequent and showing systemic manifestations, such as myotonia (muscle relaxation difficulty), cataracts, arrhythmias, muscle weakness and atrophy, among others, respiratory failure being one of the main factors predictive of mortality and a leading cause of death. OBJECTIVES: To evaluate the sensitivity / specificity of the maximum relaxation rate of the inspiratory muscles (MRR), the surface electromyography (sEMG) of the sternocleidomastoid (SCM), scalene (SCA), parasternal (2ICS), rectus abdominis (RA) and lung function/respiratory muscle strength in patients with DM1 and healthy subjects. METHODS: We invited 74 subjects to participate in the study, 44 patients with DM1 and 30 controls. The study included 31 subjects (18 DM1 and 13 controls). After exclusions, the final sample was of 27 subjects, 16 patients with DM1 and 11 healthy subjects. All of them were assessed for MRR of inspiratory muscles, for sEMG of the respiratory muscles, muscle and lung function. RESULTS: The MRR was lower in patients with DM1 vs. control (p = 0.001) and was considered sensitive and specific to identify the disease in DM1 and discard it in the control group, ROC area of 0.87 (95% CI, 0.74 to 1.01, p=0.001). DM1 group showed lower PImax (p=0.0006), PEmax (p=0.0002), SNIP (p=0.0014), CVF%pred (p=0.0018) and FEV1%pred. (p=0.0004) and higher sEMG activity of the SCM (p=0.0022) and ESC muscles (p=0.004) at rest; of 2EIC (p=0.003) during PEmax and of SCM (p=0.02) and ESC muscles (p= 0.03) during the sniff test. CONCLUSIONS: The MRR is sensitive and specific to identify delayed relaxation of the respiratory muscles and respiratory muscle function is altered in patients with DM1.

Made available in DSpace on 2014-12-17T15:16:06Z (GMT). No. of bitstreams: 1 ThaiseLA.pdf: 779453 bytes, checksum: fe87c118f2c753bc8ffd4a32cb6a1146 (MD5) Previous issue date: 2009-07-22 / Background: The myotonic dystrophy (MD) is a multisystem neuromuscular disease that can affect the respiratory muscles and heart function, and cause impairment in quality of life. Objectives: Investigate the changes in respiratory muscle strength, health-related quality of life (HRQoL) and autonomic modulation heart rate (HR) in patients with MD. Methods: Twenty-three patients performed assessment of pulmonary function, sniff nasal inspiratory pressure (SNIP), the maximal inspiratory (MIP) and expiratory (MEP) pressure, and of HRQoL (SF-36 questionnaire). Of these patients, 17 underwent assessment of heart rate variability (HRV) at rest, in the supine and seated positions. Results: The values of respiratory muscle strength were 64, 70 and 80% of predicted for MEP, MIP, and SNIP, respectively. Significant differences were found in the SF-36 domains of physical functioning (58.7 ? 31,4 vs. 84.5 ? 23, p<0.01) and physical problems (43.4 ? 35.2 vs. 81.2 ? 34, p<0.001) when patients were compared with the reference values. Single linear regression analysis demonstrated that MIP explains 29% of the variance in physical functioning, 18% of physical problems and 20% of vitality. The HRV showed that from supine position to seated, HF decreased (0.43 x 0.30), and LF (0.57 x 0.70) and the LF/HF ratio (1.28 x 2.22) increased (p< 0.05). Compared to healthy persons, LF was lower in both male patients (2.68 x 2.99) and women (2.31 x 2.79) (p< 0.05). LF / HF ratio and LF were higher in men (5.52 x 1.5 and 0.8 x 0.6, p <0.05) and AF in women (0.43 x 0.21) (p< 0.05). There was positive correlation between the time of diagnosis and LF / HF ratio (r = 0.7, p <0.01). Conclusions: The expiratory muscle strength was reduced. The HRQoL was more impaired on the physical aspects and partly influenced by changes in inspiratory muscle strength. The HRV showed that may be sympathetic dysfunction in autonomic modulation of HR, although with normal adjustment of autonomic modulation during the change of posture. The parasympathetic modulation is higher in female patients and sympathetic tends to increase in patients with longer diagnosis / Introdu??o: A distrofia miot?nica (DM) ? uma doen?a neuromuscular multissist?mica que pode afetar a musculatura respirat?ria e a fun??o card?aca, e ocasionar preju?zos na qualidade de vida. Objetivos: Investigar as altera??es na for?a muscular respirat?ria, qualidade de vida relacionada ? sa?de (QVRS), e modula??o auton?mica da freq??ncia card?aca (FC) em pacientes com DM. M?todos: Foram avaliados 23 pacientes quanto ? fun??o pulmonar, press?o inspirat?ria nasal sniff (SNIP), press?es respirat?rias m?ximas (PIm?x e PEm?x), e QVRS (question?rio SF-36). Destes, 17 realizaram avalia??o da variabilidade da frequ?ncia card?aca (VFC) em repouso, nas posturas supina e sentada. Resultados: Os valores da for?a muscular respirat?ria foram de 64, 70 e 80%predito para PEm?x, PIm?x, e SNIP, respectivamente. Foi encontrada diminui??o significativa nos dom?nios do SF-36 capacidade funcional (58.7 ? 31,4 vs. 84.5 ? 23, p<0.01) e disfun??o f?sica (43.4 ? 35.2 vs. 81.2 ? 34, p<0.001) comparado a valores de refer?ncia. A an?lise de regress?o linear mostrou que a PIm?x explica 29% da vari?ncia na capacidade funcional, 18% na disfun??o f?sica e 20% na vitalidade. A VFC mostrou que, da postura supina para a sentada, o espectro AF diminuiu (0.43 x 0.30) e o espectro BF (0.57 x 0.70) e a raz?o BF/AF (1.28 x 2.22) aumentaram, com p<0.05. Comparado a valores de refer?ncia, BF foi inferior (p<0.05) tanto nos pacientes homens (2.68 x 2.99), como nas mulheres (2.31 x 2.79). A raz?o BF/AF e o espectro BF foram maiores nos homens (5.52 x 1.5 e 0.8 x 0.6), e o espectro AF, nas mulheres (0.43 x 0.21), com p<0.05. Houve correla??o significativa positiva entre tempo de diagn?stico e raz?o BF/AF (r= 0.7, p< 0.01). Conclus?es: Indiv?duos com DM t?m for?a muscular expirat?ria diminu?da. A QVRS mostrou-se mais prejudicada em rela??o a aspectos f?sicos e parcialmente influenciada por varia??es na for?a muscular inspirat?ria. Pode haver disfun??o simp?tica na modula??o auton?mica da FC, com ajuste normal da postura supina para a sentada. A modula??o parassimp?tica ? superior em pacientes mulheres e a modula??o simp?tica tende a aumentar nos pacientes com maior tempo de diagn?stico