Autonomic thermoregulatory dysfunction in neurofibromatosis type 1

• Autor(a) principal: Luciana Gonçalves Madeira
• Data de Publicação: 2016
• Outros Autores: Renata Lane de Freitas Passos, Juliana Ferreira de Souza, Nilton Alves de Rezende, Luiz Oswaldo Carneiro Rodrigues
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Repositório Institucional da UFMG
• Texto Completo: http://hdl.handle.net/1843/62658
• topic: Neurofibromatosis 1; Primary Dysautonomias; Sweating; primary dysautonomias; Body Temperature Regulation; Neurofibromatosis 1; Sweating; Primary Dysautonomias; Body Temperature Regulation

Objective: Neurofibromatosis type 1 (NF1) causes neural and cutaneous disorders and reduced exercise capacity. Exercise/heat exposure increasing internal temperature must be compensated by eccrine sweat function and warmed skin vasodilation. We suspected NF1 could adversely affect eccrine sweat function and/or vascular thermoregulatory responses (VTR). Methods: The eccrine sweat function and VTR of 25 NF1 volunteers (14 males, 11 females; 16–57 years old) were compared with 23 non-NF1 controls matched by sex, age, height and weight (CG). Sweating was induced by 1) pilocarpine 1% iontophoresis (PILO); and 2) by passive heating (HEAT) via the lower third of the legs being immersed in 42°C water for one hour. Previously established eccrine sweat function and VTR protocols were used. Results: The NF1 group showed: a) lower sweat rate than the CG group during PILO; b) a smaller diastolic pressure decrease; and c) higher tympanic temperatures than controls during HEAT (p < 0.05). Conclusion: Reduced sweating and vascular thermoregulatory responses suggest autonomic dysfunction in NF1 individuals