Autonomic thermoregulatory dysfunction in neurofibromatosis type 1

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...

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Published inArquivos de neuro-psiquiatria Vol. 74; no. 10; pp. 796 - 802
Main Authors Madeira, Luciana G, Passos, Renata Lf, Souza, Juliana F de, Rezende, Nilton A, Rodrigues, Luiz O C
Format Journal Article
LanguageEnglish
Published Brazil Academia Brasileira de Neurologia - ABNEURO 01.10.2016
Academia Brasileira de Neurologia (ABNEURO)
Subjects
Adolescent
Adult
Age Factors
Analysis of Variance
body temperature regulation
Body Temperature Regulation - physiology
Case-Control Studies
Female
Humans
Male
Middle Aged
neurofibromatosis 1
Neurofibromatosis 1 - physiopathology
NEUROSCIENCES
primary dysautonomias
Primary Dysautonomias - physiopathology
PSYCHIATRY
Reference Values
Sex Factors
Skin - physiopathology
Sweat - physiology
sweating
Sweating - physiology
Time Factors
Young Adult
sudorese
disautonomia primária
regulação da temperatura corporal
primary dysautonomias
neurofibromatosis 1
sweating
body temperature regulation
neurofibromatose 1
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Abstract 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). 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. 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). Reduced sweating and vascular thermoregulatory responses suggest autonomic dysfunction in NF1 individuals.
AbstractList OBJECTIVENeurofibromatosis 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). METHODSThe 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. RESULTSThe 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). CONCLUSIONReduced sweating and vascular thermoregulatory responses suggest autonomic dysfunction in NF1 individuals.
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[degrees]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.Original Abstract: Objetivo Neurofibromatose do tipo 1 (NF1) causa problemas neurais e cutaneos e diminuicao da capacidade fisica. O aumento da temperatura interna durante exercicio e exposicao ao calor precisa ser compensada pela funcao sudoripara ecrina (FSE) e aquecimento cutaneo por vasodilatacao (RVT). Suspeitou-se clinicamente que a NF1 poderia prejudicar a FSE e a RVT. Metodos A FSE e RVT de 25 voluntarios com NF1 (14 homens, 11 mulheres; 16-57 anos) e de 23 sem-NF1, emparelhados por sexo, idade, estatura e peso corporal, foram medidas com protocolos validados anteriormente. A sudorese foi induzida por iontoforese com pilocarpina (PILO) e aquecimento passivo por imersao das pernas em agua a 42[degrees]C durante uma hora (HEAT). Resultados O grupo NF1 apresentou menor taxa de sudorese na situacao PILO, menor reducao da pressao diastolica e maior temperatura timpanica na situacao HEAT (p < 0.05). Conclusao As respostas sudoripara e vascular reduzidas sugerem disfuncao autonomica nas pessoas com NF1.
ABSTRACT 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. RESUMO Objetivo Neurofibromatose do tipo 1 (NF1) causa problemas neurais e cutâneos e diminuição da capacidade física. O aumento da temperatura interna durante exercício e exposição ao calor precisa ser compensada pela função sudorípara écrina (FSE) e aquecimento cutâneo por vasodilatação (RVT). Suspeitou-se clinicamente que a NF1 poderia prejudicar a FSE e a RVT. Métodos A FSE e RVT de 25 voluntários com NF1 (14 homens, 11 mulheres; 16–57 anos) e de 23 sem-NF1, emparelhados por sexo, idade, estatura e peso corporal, foram medidas com protocolos validados anteriormente. A sudorese foi induzida por iontoforese com pilocarpina (PILO) e aquecimento passivo por imersão das pernas em água a 42°C durante uma hora (HEAT). Resultados O grupo NF1 apresentou menor taxa de sudorese na situação PILO, menor redução da pressão diastólica e maior temperatura timpânica na situação HEAT (p < 0.05). Conclusão As respostas sudorípara e vascular reduzidas sugerem disfunção autonômica nas pessoas com NF1.
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). 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. 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). Reduced sweating and vascular thermoregulatory responses suggest autonomic dysfunction in NF1 individuals.
ABSTRACT 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.
Author Madeira, Luciana G
Rezende, Nilton A
Rodrigues, Luiz O C
Passos, Renata Lf
Souza, Juliana F de
AuthorAffiliation Universidade Federal de Minas Gerais
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  givenname: Renata Lf
  surname: Passos
  fullname: Passos, Renata Lf
  organization: Universidade Federal de Minas Gerais, Escola de Educação Física, Fisioterapia e Terapia Ocupacional, Programa de Pós Graduação em Ciências do Esporte, Belo Horizonte MG, Brasil
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  givenname: Juliana F de
  surname: Souza
  fullname: Souza, Juliana F de
  organization: Universidade Federal de Minas Gerais, Hospital das Clínicas, Centro de Referência em Neurofibromatoses, Belo Horizonte MG, Brasil
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  givenname: Luiz O C
  surname: Rodrigues
  fullname: Rodrigues, Luiz O C
  organization: Universidade Federal de Minas Gerais, Hospital das Clínicas, Centro de Referência em Neurofibromatoses, Belo Horizonte MG, Brasil
BackLink https://www.ncbi.nlm.nih.gov/pubmed/27759804$$D View this record in MEDLINE/PubMed
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DocumentTitleAlternate Disfunção autonômica termorregulatória na neurofibromatose do tipo 1
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Issue 10
Keywords sudorese
disautonomia primária
regulação da temperatura corporal
primary dysautonomias
neurofibromatosis 1
sweating
body temperature regulation
neurofibromatose 1
Language English
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Snippet Neurofibromatosis type 1 (NF1) causes neural and cutaneous disorders and reduced exercise capacity. Exercise/heat exposure increasing internal temperature must...
ABSTRACT Objective Neurofibromatosis type 1 (NF1) causes neural and cutaneous disorders and reduced exercise capacity. Exercise/heat exposure increasing...
OBJECTIVENeurofibromatosis type 1 (NF1) causes neural and cutaneous disorders and reduced exercise capacity. Exercise/heat exposure increasing internal...
Objective Neurofibromatosis type 1 (NF1) causes neural and cutaneous disorders and reduced exercise capacity. Exercise/heat exposure increasing internal...
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SubjectTerms Adolescent
Adult
Age Factors
Analysis of Variance
body temperature regulation
Body Temperature Regulation - physiology
Case-Control Studies
Female
Humans
Male
Middle Aged
neurofibromatosis 1
Neurofibromatosis 1 - physiopathology
NEUROSCIENCES
primary dysautonomias
Primary Dysautonomias - physiopathology
PSYCHIATRY
Reference Values
Sex Factors
Skin - physiopathology
Sweat - physiology
sweating
Sweating - physiology
Time Factors
Young Adult
Title Autonomic thermoregulatory dysfunction in neurofibromatosis type 1
URI https://www.ncbi.nlm.nih.gov/pubmed/27759804
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