Physiotherapeutic performance in tuberous sclerosis associated with lymphangioleiomyomatosis

Detalhes bibliográficos
Autor(a) principal: Almeida, Caio Fernandes de
Data de Publicação: 2022
Outros Autores: Genske, Juliana Hering, Tori, Fernanda da Silva, Burgarelli, Juliana Almeida, Schaefer, Alessandro, Abramovecht, Janaina, Magnante, Jessica, Pastorio, Daiane Maria
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