Doença de Erdheim Chester com envolvimento isolado do SNC

Erdheim Chester disease with isolated CNS involvement

Authors

  • Alexandre Abdelaziz Rodrigues
  • Aline Luiza Ribeiro
  • Arthur Carvalho Faria
  • Caroline Machado Mendes dos Santos
  • Marcos Alexandre Lourenço Almeida
  • Maria Carolina Costa Teixeira
  • Mateus Tavares de Souza
  • Rafaela Santa Cruz Cad
  • Luana Santos Menezes Rabuske
  • Ana Paula de Santana
  • Jaslana Cristina Braga Bragagnolo
  • Ana Clara Honorato Chaves
  • Ana Júlia Marçal Pereira Dias
  • Isabella de Freitas Faleiro
  • Isabella Freire Pedrini
  • Mariana Gabriela Ferreira Mota
  • Fernanda Beatriz Galvani
  • Isadora Vieira de Sousa
  • Jenniffer Faria Ribeiro
  • Julia Drumond Baptista
  • Juliana Barros Vianna de Oliveira
  • Jéssica Silva Nicácio

DOI:

https://doi.org/10.34119/bjhrv5n5-103

Keywords:

Doença de Erdheim-Chester, células não-Langerhans, manifestações, tratamentos

Abstract

Introdução: A Doença de Erdheim-Chester é  uma forma de  histiocitose  de  células  não-Langerhans  que  ocorre  mais  frequentemente  após  os  40  anos  de  idade,  com leve  predomínio  no  sexo  masculino. Apresentação do caso: Paciente, sexo masculino, branco, X anos de idade, admitido no Hospital das Clínicas de Goiânia, com sintomas de cefaléia crônica, de caráter progressivo de inicio há 3 meses, associados a sintomas neurológicos de ataxia e diplopia. Referiu febre baixa diária, não aferida, com perda de 10kg no período descrito, não associado a dieta ou mudança de hábitos de vida. Nega comorbidades ou uso de medicamentos.  Ao exame físico, apresenta quadro de Nistagmo e Romberg positivo e alteração da úvula para direita. Paciente não apresentava outros sinais dignos de nota. Discussão: Foram selecionados para a revisão, os artigos de relato de caso ou séries de caso relacionados a manifestações clínicas da doença de Erdheim-Chester. Os principais tipos manifestações clínicas relatadas na literatura são: neurológicas, cardiovasculares, oftálmicas, endócrinas, urinárias, hematológicas, ósseas, cutâneas, respiratórias, gastrointestinais, envolvimento de múltiplos sistemas e outras. Entre esses tipos de manifestações, as mais frequentes foram neurológicas, cardiovasculares e oftálmicas, correspondendo a 48% das manifestações relatadas. Conclusão: É fundamental ter uma equipe multidisciplinar acompanhando o paciente,  para que sejam evitadas complicações e se possa proporcionar qualidade de vida ao paciente.

 

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Published

2022-09-16

How to Cite

RODRIGUES, A. A.; RIBEIRO, A. L.; FARIA , A. C.; DOS SANTOS, C. M. M.; ALMEIDA, M. A. L.; TEIXEIRA , M. C. C.; DE SOUZA, M. T.; CAD , R. S. C.; RABUSKE, L. S. M.; DE SANTANA, A. P.; BRAGAGNOLO, J. C. B.; CHAVES, A. C. H.; DIAS, A. J. M. P.; FALEIRO, I. de F.; PEDRINI, I. F.; MOTA, M. G. F.; GALVANI , F. B.; DE SOUSA , I. V.; RIBEIRO, J. F.; BAPTISTA, J. D.; DE OLIVEIRA, J. B. V.; NICÁCIO, J. S. Doença de Erdheim Chester com envolvimento isolado do SNC: Erdheim Chester disease with isolated CNS involvement. Brazilian Journal of Health Review, [S. l.], v. 5, n. 5, p. 18916–18927, 2022. DOI: 10.34119/bjhrv5n5-103. Disponível em: https://ojs.brazilianjournals.com.br/ojs/index.php/BJHR/article/view/52161. Acesso em: 28 mar. 2024.

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Section

Original Papers