Physiotherapeutic performance in tuberous sclerosis associated with lymphangioleiomyomatosis
Autor(a) principal: | |
---|---|
Data de Publicação: | 2022 |
Outros Autores: | , , , , , , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Research, Society and Development |
Texto Completo: | https://rsdjournal.org/index.php/rsd/article/view/26272 |
Resumo: | Tuberous sclerosis is a rare disease, of genetic and degenerative character, where there are anomalies in the TSC1 or TSC2 genes, of chromosomes 9 and 16. Secondary to the pathophysiological process of this disease, pulmonary lymphangioleiomyomatosis (LAM) can occur, a low-grade neoplasm characterized by the proliferation of smooth muscle cells in the lung parenchyma, which can cause several clinical manifestations, such as: spontaneous and recurrent pneumothorax, dyspnea, cough and reduced functional capacity of the individual. Thus, patients have a clinical evolution towards deterioration of lung capacity and respiratory homeostasis disorder.Even with the advancement of medicine, there is still no cure for these diseases. In this study, we report a case of an adult female patient, diagnosed with tuberous sclerosis and pulmonary lymphangioleiomyomatosis, treated at the university hospital in western paraná (HUOP). hospitalization in a patient with tuberous sclerosis and lymphangioleiomyomatosis and thus correlate with the possible benefits of intervention and physical therapy treatment. Patient evolved to discharge improved, active and without the need for oxygen therapy. Therefore, it is concluded that physical therapy is an important ally in helping to treat tuberous sclerosis and lymphangioleiomyomatosis. |
id |
UNIFEI_fd9bb664380a209069072bf515f3c67f |
---|---|
oai_identifier_str |
oai:ojs.pkp.sfu.ca:article/26272 |
network_acronym_str |
UNIFEI |
network_name_str |
Research, Society and Development |
repository_id_str |
|
spelling |
Physiotherapeutic performance in tuberous sclerosis associated with lymphangioleiomyomatosisActuación fisioterapéutica en la esclerosis tuberosa asociada a linfangioleiomiomatosis Atuação fisioterapêutica na esclerose tuberosa associada a linfangioleiomiomatose Tuberous SclerosisLymphangioleiomyomatosisPhysiotherapy hospital serviceRehabilitation.Esclerosis tuberosaLinfangioleiomiomatosisServicio hospitalario de fisioterapiaRehabilitación.Esclerose tuberosaLinfangioleiomiomatoseServiço hospitalar de fisioterapiaReabilitação.Tuberous sclerosis is a rare disease, of genetic and degenerative character, where there are anomalies in the TSC1 or TSC2 genes, of chromosomes 9 and 16. Secondary to the pathophysiological process of this disease, pulmonary lymphangioleiomyomatosis (LAM) can occur, a low-grade neoplasm characterized by the proliferation of smooth muscle cells in the lung parenchyma, which can cause several clinical manifestations, such as: spontaneous and recurrent pneumothorax, dyspnea, cough and reduced functional capacity of the individual. Thus, patients have a clinical evolution towards deterioration of lung capacity and respiratory homeostasis disorder.Even with the advancement of medicine, there is still no cure for these diseases. In this study, we report a case of an adult female patient, diagnosed with tuberous sclerosis and pulmonary lymphangioleiomyomatosis, treated at the university hospital in western paraná (HUOP). hospitalization in a patient with tuberous sclerosis and lymphangioleiomyomatosis and thus correlate with the possible benefits of intervention and physical therapy treatment. Patient evolved to discharge improved, active and without the need for oxygen therapy. Therefore, it is concluded that physical therapy is an important ally in helping to treat tuberous sclerosis and lymphangioleiomyomatosis.La esclerosis tuberosa es una enfermedad rara, de carácter genético y degenerativo, donde existen anomalías en los genes TSC1 o TSC2, de los cromosomas 9 y 16. Secundaria al proceso fisiopatológico de esta enfermedad, puede ocurrir linfangioleiomiomatosis pulmonar (LAM), una baja neoplasia de grado caracterizada por la proliferación de células de músculo liso en el parénquima pulmonar, que puede ocasionar diversas manifestaciones clínicas, tales como: neumotórax espontáneo y recurrente, disnea, tos y disminución de la capacidad funcional del indivíduo. Así, los pacientes tienen una evolución clínica hacia el deterioro de la capacidad pulmonar y el trastorno de la homeostasis respiratoria. Incluso con el avance de la medicina, todavía no existe cura para estas enfermedades. En este estudio se reporta el caso de una paciente adulta, diagnosticada de esclerosis tuberosa y linfangioleiomiomatosis pulmonar, atendida en el hospital universitario de paraná occidental (HUOP). Hospitalización en un paciente con esclerosis tuberosa y linfangioleiomiomatosis y así poder correlacionar con los posibles beneficios de la intervención y el tratamiento de fisioterapia. El paciente evolucionó hasta el alta mejorada, activa y sin necesidad de oxigenoterapia. Se concluye que la fisioterapia es un aliado importante para ayudar a tratar la esclerosis tuberosa y la linfangioleiomiomatosis.A esclerose tuberosa é uma doença rara, de caráter genético e degenerativo, onde ocorre anomalias no genes TSC1 ou TSC2, dos cromossomos 9 e 16, secundariamente ao processo fisiopatológico dessa doença pode ocorrer a linfangioleiomiomatose pulmonar (LAM), uma neoplasia de baixo grau caracterizada pela proliferação de células musculares lisas no parênquima pulmonar, podendo ocasionar diversas manifestações clínicas, tais como: pneumotórax espontâneo e recorrente, dispneia, tosse e redução da capacidade funcional do indivíduo. Desta forma os pacientes possuem uma evolução clínica para deterioração das capacidades pulmonares e distúrbio da homeostase respiratória. Mesmo com o avanço da medicina ainda não existe uma cura para essas doenças. Neste estudo, relata-se um caso de uma paciente adulta, do sexo feminino, com diagnóstico de esclerose tuberosa e linfangioleiomiomatose pulmonar, tratada no Hospital Universitário do Oeste do Paraná (HUOP), portanto, este relato descreve abordagens fisioterapêuticas aplicadas em diferentes momentos da hospitalização em uma paciente portadora de esclerose tuberosa e linfangioleiomiomatose e assim podendo correlacionar com os possíveis benefícios da intervenção e tratamento fisioterapêutico. Paciente evoluiu para alta melhorada, ativa e sem necessidade de oxigenoterapia. Conclui-se que a fisioterapia é uma importante aliada no auxílio do tratamento da esclerose tuberosa e linfangioleiomiomatose.Research, Society and Development2022-02-05info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://rsdjournal.org/index.php/rsd/article/view/2627210.33448/rsd-v11i2.26272Research, Society and Development; Vol. 11 No. 2; e56511226272Research, Society and Development; Vol. 11 Núm. 2; e56511226272Research, Society and Development; v. 11 n. 2; e565112262722525-3409reponame:Research, Society and Developmentinstname:Universidade Federal de Itajubá (UNIFEI)instacron:UNIFEIporhttps://rsdjournal.org/index.php/rsd/article/view/26272/22870Copyright (c) 2022 Caio Fernandes de Almeida; Juliana Hering Genske; Fernanda da Silva Tori; Juliana Almeida Burgarelli; Alessandro Schaefer; Janaina Abramovecht; Jessica Magnante; Daiane Maria Pastoriohttps://creativecommons.org/licenses/by/4.0info:eu-repo/semantics/openAccessAlmeida, Caio Fernandes de Genske, Juliana Hering Tori, Fernanda da Silva Burgarelli, Juliana Almeida Schaefer, Alessandro Abramovecht, JanainaMagnante, Jessica Pastorio, Daiane Maria 2022-02-07T01:42:50Zoai:ojs.pkp.sfu.ca:article/26272Revistahttps://rsdjournal.org/index.php/rsd/indexPUBhttps://rsdjournal.org/index.php/rsd/oairsd.articles@gmail.com2525-34092525-3409opendoar:2024-01-17T09:44:21.334379Research, Society and Development - Universidade Federal de Itajubá (UNIFEI)false |
dc.title.none.fl_str_mv |
Physiotherapeutic performance in tuberous sclerosis associated with lymphangioleiomyomatosis Actuación fisioterapéutica en la esclerosis tuberosa asociada a linfangioleiomiomatosis Atuação fisioterapêutica na esclerose tuberosa associada a linfangioleiomiomatose |
title |
Physiotherapeutic performance in tuberous sclerosis associated with lymphangioleiomyomatosis |
spellingShingle |
Physiotherapeutic performance in tuberous sclerosis associated with lymphangioleiomyomatosis Almeida, Caio Fernandes de Tuberous Sclerosis Lymphangioleiomyomatosis Physiotherapy hospital service Rehabilitation. Esclerosis tuberosa Linfangioleiomiomatosis Servicio hospitalario de fisioterapia Rehabilitación. Esclerose tuberosa Linfangioleiomiomatose Serviço hospitalar de fisioterapia Reabilitação. |
title_short |
Physiotherapeutic performance in tuberous sclerosis associated with lymphangioleiomyomatosis |
title_full |
Physiotherapeutic performance in tuberous sclerosis associated with lymphangioleiomyomatosis |
title_fullStr |
Physiotherapeutic performance in tuberous sclerosis associated with lymphangioleiomyomatosis |
title_full_unstemmed |
Physiotherapeutic performance in tuberous sclerosis associated with lymphangioleiomyomatosis |
title_sort |
Physiotherapeutic performance in tuberous sclerosis associated with lymphangioleiomyomatosis |
author |
Almeida, Caio Fernandes de |
author_facet |
Almeida, Caio Fernandes de Genske, Juliana Hering Tori, Fernanda da Silva Burgarelli, Juliana Almeida Schaefer, Alessandro Abramovecht, Janaina Magnante, Jessica Pastorio, Daiane Maria |
author_role |
author |
author2 |
Genske, Juliana Hering Tori, Fernanda da Silva Burgarelli, Juliana Almeida Schaefer, Alessandro Abramovecht, Janaina Magnante, Jessica Pastorio, Daiane Maria |
author2_role |
author author author author author author author |
dc.contributor.author.fl_str_mv |
Almeida, Caio Fernandes de Genske, Juliana Hering Tori, Fernanda da Silva Burgarelli, Juliana Almeida Schaefer, Alessandro Abramovecht, Janaina Magnante, Jessica Pastorio, Daiane Maria |
dc.subject.por.fl_str_mv |
Tuberous Sclerosis Lymphangioleiomyomatosis Physiotherapy hospital service Rehabilitation. Esclerosis tuberosa Linfangioleiomiomatosis Servicio hospitalario de fisioterapia Rehabilitación. Esclerose tuberosa Linfangioleiomiomatose Serviço hospitalar de fisioterapia Reabilitação. |
topic |
Tuberous Sclerosis Lymphangioleiomyomatosis Physiotherapy hospital service Rehabilitation. Esclerosis tuberosa Linfangioleiomiomatosis Servicio hospitalario de fisioterapia Rehabilitación. Esclerose tuberosa Linfangioleiomiomatose Serviço hospitalar de fisioterapia Reabilitação. |
description |
Tuberous sclerosis is a rare disease, of genetic and degenerative character, where there are anomalies in the TSC1 or TSC2 genes, of chromosomes 9 and 16. Secondary to the pathophysiological process of this disease, pulmonary lymphangioleiomyomatosis (LAM) can occur, a low-grade neoplasm characterized by the proliferation of smooth muscle cells in the lung parenchyma, which can cause several clinical manifestations, such as: spontaneous and recurrent pneumothorax, dyspnea, cough and reduced functional capacity of the individual. Thus, patients have a clinical evolution towards deterioration of lung capacity and respiratory homeostasis disorder.Even with the advancement of medicine, there is still no cure for these diseases. In this study, we report a case of an adult female patient, diagnosed with tuberous sclerosis and pulmonary lymphangioleiomyomatosis, treated at the university hospital in western paraná (HUOP). hospitalization in a patient with tuberous sclerosis and lymphangioleiomyomatosis and thus correlate with the possible benefits of intervention and physical therapy treatment. Patient evolved to discharge improved, active and without the need for oxygen therapy. Therefore, it is concluded that physical therapy is an important ally in helping to treat tuberous sclerosis and lymphangioleiomyomatosis. |
publishDate |
2022 |
dc.date.none.fl_str_mv |
2022-02-05 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://rsdjournal.org/index.php/rsd/article/view/26272 10.33448/rsd-v11i2.26272 |
url |
https://rsdjournal.org/index.php/rsd/article/view/26272 |
identifier_str_mv |
10.33448/rsd-v11i2.26272 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
https://rsdjournal.org/index.php/rsd/article/view/26272/22870 |
dc.rights.driver.fl_str_mv |
https://creativecommons.org/licenses/by/4.0 info:eu-repo/semantics/openAccess |
rights_invalid_str_mv |
https://creativecommons.org/licenses/by/4.0 |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Research, Society and Development |
publisher.none.fl_str_mv |
Research, Society and Development |
dc.source.none.fl_str_mv |
Research, Society and Development; Vol. 11 No. 2; e56511226272 Research, Society and Development; Vol. 11 Núm. 2; e56511226272 Research, Society and Development; v. 11 n. 2; e56511226272 2525-3409 reponame:Research, Society and Development instname:Universidade Federal de Itajubá (UNIFEI) instacron:UNIFEI |
instname_str |
Universidade Federal de Itajubá (UNIFEI) |
instacron_str |
UNIFEI |
institution |
UNIFEI |
reponame_str |
Research, Society and Development |
collection |
Research, Society and Development |
repository.name.fl_str_mv |
Research, Society and Development - Universidade Federal de Itajubá (UNIFEI) |
repository.mail.fl_str_mv |
rsd.articles@gmail.com |
_version_ |
1797052762737868800 |