Physiotherapeutic performance in tuberous sclerosis associated with lymphangioleiomyomatosis
DOI:
https://doi.org/10.33448/rsd-v11i2.26272Keywords:
Tuberous Sclerosis; Lymphangioleiomyomatosis; Physiotherapy hospital service; Rehabilitation.Abstract
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.
References
Alshoabi, S. A., Hamid, A. M., Alhazmi, F. H., Qurashi, A. A., Abdulaal, O. M., Aloufi, K. M., & Daqqaq, T. S. (2022). Diagnostic features of tuberous sclerosis complex: case report and literature review. Quantitative Imaging in Medicine and Surgery, 12(1), 846.
Associação dos portadores de Linfangionleiomiomatose no Brasil - ALAMBRA. (2017). Recuperado de https://www.alambrabrasil.org/
Bissler, J. J., Kingswood, J. C., Radzikowska, E., Zonnenberg, B. A., Frost, M., Belousova, E., & Budde, K. (2013). Everolimus for angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): a multicentre, randomised, double-blind, placebo-controlled trial. The Lancet, 381(9869), 817–824.
Carvalho, M. R. M., Silva, N. A. S., Oliveira, G. M. M., & Klein, C. H. (2011). Complicações e tempo de internação na revascularização miocárdica em hospitais públicos no Rio de Janeiro. Revista Brasileira de Terapia Intensiva, 23(3), 312–320.
Carneiro, A. S., Mafort, T. T., & Lopes, A. J. (2021). A 34-Year-Old Woman from Brazil with Pulmonary Lymphangioleiomyomatosis Diagnosed by Raised Serum Vascular Endothelial Growth Factor-D (VEGF-D) Levels and Lung Cysts on Computed Tomography Imaging Presenting with COVID-19 Pneumonia. The American Journal of Case Reports, 22, e932660-1.
Conselho Regional de Enfermagem de São Paulo. (2011). Documentos básicos de enfermagem: enfermeiros, técnicos, auxiliares. Boas práticas Dreno de tórax. Recuperado de https://portal.coren-sp.gov.br/sites/default/files/dreno-de-torax.pdf
Cunha., C. S., Soares, B., & Nascimento, R. R. (2009). Técnicas reexpansivas no derrame pleural - uma revisão de literatura. Cadernos UniFOA, 4(9), 63–67.
Santos, E. C., & Lunardi, A. C. (2015). Efficacy of the addition of positive airway pressure to conventional chest physiotherapy in resolution of pleural effusion after drainage: protocol for a randomised controlled trial. Journal of Physiotherapy, 61(2), 93.
Duarte, M. P., Rosa, L. L. D., Pinheiro, E. M., Alves, I. K., Santos, A. A., Andrade, Á. D. B., & Diniz, W. A. (2020). Influência da fisioterapia na reabilitação de pacientes submetidos à drenagem torácica em um hospital de urgência e emergência da Amazônia legal. Revista Eletrônica Acervo Saúde, (45), e2959-e2959.
Evangelista, A. R., Morais, R. A., Moura, F. M., Carvalho, K. M. M. B. (2019). Intervenções fisioterapêuticas no tratamento e estabilização de pacientes com pneumotórax. Encontro de extensão, docência e iniciação científica (eedic), 5(1).
Freitas, C. S. G., Baldi, B. G., Araújo, M. S., Heiden, G. I., Kairalla, R. A., & Carvalho, C. R. R. (2015). Use of sirolimus in the treatment of lymphangioleiomyomatosis: favorable responses in patients with different extrapulmonary manifestations. Jornal Brasileiro de Pneumologia, 41(3), 275–280.
Grigorescu C., Bosânceanu M., Boişteanu D., Aldea A., Chiseliţă I., Cozma L.G. (2007). Limfangioleiomiomatoza cauză rară de pneumotorax spontan bilateral [Lymphangioleiomyomatosis (LAM)--an uncommon cause of bilateral spontaneous pneumothorax]. Revista Medico-chirurgicala a Societatii de Medici si Naturalist Din Iasi, 111(1), 125-128.
Medeiros, P. J., & Carvalho, C. R. R. (2004). Linfangioleiomiomatose pulmonar. Jornal Brasileiro de Pneumologia, 30(1), 66–77.
Nomori, H., Horio, H., Suemasu, K. (2001). Early removal of chest drainage tubes and oxygen support after a lobectomy for lung cancer facilitates earlier recovery of the 6-minute walking distance. Surgery Today, 31, 395-399.
Alvez, A. N. (2012). A importância da atuação do fisioterapeuta no ambiente hospitalar. Ensaios e Ciência: Ciências Biológicas, Agrárias e Da Saúde, 16(6), 173–184.
Pasini, R. (2004). Complicação relacionada ao uso da respiração com pressão positiva intermitente. Fisioterapia Brasil, 5(3), 242–245.
Lima, V. P., Bonfim, D., Risso, T. T., Paisani, D. M., Fiore, J. F. J., Chiavegato, L. D., & Faresin, S. M. (2008). Influência do dreno pleural sobre a dor, capacidade vital e teste de caminhada de seis minutos em pacientes submetidos à ressecção pulmonar. In Jornal Brasileiro de Pneumologia, 34(12), 1003-1007.
Reis, J. S. F., Netto, M. H. M., Loyola, J. G. N., Araújo, J. C., Antoniuk, S., & Torres, L. F. B. (1998). Eclerose tuberosa: relato de caso com estudo histopatológico e ultraestrutural. Arquivos de Neuro-Psiquiatria, 56(3 B), 671–676.
Riojas RA., Bahr BA., Thomas DB, Perciballi J., Noyes L. (2012) A case report of lymphangioleiomyomatosis presenting as spontaneous pneumothorax. Mil Med,177(4):477-80.
Cutolo, L. R. A., Bonassa, R. T., Stahelin, A. H. S., & Macedo, L. S. (2018). linfangioleiomiomatose pulmonar em paciente pediátrico: relato de caso. Arquivos Catarinenses de Medicina, 47(1), 216–223.
Silva, C. C. M., & Santos, I. M. (2019). A importância da fisioterapia no setor de urgência e emergência: uma revisão de literatura. Brazilian Journal of Development, 5(10), 18335-18343.
Vieira, S., & Hossne, W. S. (2021). Metodologia científica para a área da saúde (3a ed.). Guanabara Koogan.
Yen, K. T., Putzke, J. D., Staats, B. A., & Burguer, C. D. (2005). The prevalence of acute response to bronchodilator in pulmonary lymphangioleiomyomatosis. Respirology, 10(5), 643–648.
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Copyright (c) 2022 Caio Fernandes de Almeida; Juliana Hering Genske; Fernanda da Silva Tori; Juliana Almeida Burgarelli; Alessandro Schaefer; Janaina Abramovecht; Jessica Magnante; Daiane Maria Pastorio
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