No sweat boys! Think x-linked hypohidrotic ectodermal dysplasia

Heather Massey, Danielli Mello, Liam Colley, James R. House, Mike Tipton

Research output: Contribution to conferenceAbstractpeer-review

Abstract

X-Linked Hypohidrotic Ectodermal Dysplasia (XLHED) is a rare genetic disorder diagnosed from sparse hair; few and often pointed teeth; and diminished or absent sweat function. Little is known about their thermoregulation. Vasomotion may also be affected (Brengleman, 1981), limiting opportunities for heat loss and increasing mortality rates (Clarke, 1987). This study assessed heat loss in children with XLHED whilst intermittently treadmill walking and running in 30°C, 60% RH air, and during subsequent recovery. Six boys with XLHED (median(range) age 12 (8 to 13) years gave their assent (parental consent) and volunteered for this ethically approved study. Insulated auditory canal temperature (Tac) and index finger pad and forearm laser Doppler skin blood flow were measured. Whole body sweating was calculated by naked weighing before and after heat exposure. Two 20-min bouts of exercise were performed with 20-min passive recovery in the first rest period and hand immersion (12-15 °C) or whole body spraying and fanning (volunteer’s preference) in the second recovery period. Median (range) Tac change was 2.58 °C.hr-1(1.68 to 3.48 °C.hr-1) for the first exercise session and 2.22 °C.hr-1(1.83 to 2.55 °C.hr-1 )for the second exercise session. One volunteer was stopped at a Tac of 38.5 °C (the stopping criterion for this study) during the second exercise session. The mean cooling rate during passive recovery was -0.84 °C.hr-1 (+0.51 to -1.44 °C.hr-1) and during the cooling intervention was -2.31 °C.hr-1(-1.47 to -4.08 °C.hr-1). Whole body sweat rate was 0.17 L.hr-1(0.05 to 0.24 L.hr-1) and skin blood flow was (Finger 375 Laser Doppler units [LDU] [67 to 646 LDU], Forearm 83 LDU [14 to 562 LDU]). Children with XLHED have a rapid increase in Tac and low sweat response. Skin blood flow was elevated by increased deep body temperature, in addition vasodilatation was present and maintained during cooling interventions. Despite diminished sweating, people with XLHED can lose excess heat using active cooling interventions. It remains to be seen how the changes in Tac compare to healthy weight and height matched controls.
Original languageEnglish
Pages159
Number of pages1
Publication statusPublished - 1 Nov 2017
Event17th International Conference on Environmental Ergonomics - Kobe, Japan
Duration: 12 Nov 201718 Nov 2017

Conference

Conference17th International Conference on Environmental Ergonomics
Abbreviated titleICEE 2017
CountryJapan
CityKobe
Period12/11/1718/11/17

Keywords

  • ectodermal dysplasia
  • cooling
  • heat strain
  • children

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