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Updated: Jun 6, 2026

Esophageal Heat Transfer for Patient Temperature Control and Targeted Temperature Management
Published on: November 21, 2017
Poikilothermia in a 38-year-old Fabry patient
Marieke Biegstraaten1, Ivo N van Schaik, Carla E M Hollak
1Department of Neurology, Academic Medical Centre, PO Box 22660, 1100 DD, Amsterdam, The Netherlands. m.biegstraaten@amc.uva.nl
Abstract:
A Fabry patient with poikilothermia is described. Laboratory investigations, neuro-imaging and autonomic function tests did not disclose a cause. Assessment of intra-epidermal nerve fibre density and quantitative sensory testing revealed small fibre neuropathy with a highly impaired cold sensation. We speculate that the poikilothermia is either caused by a vascular lesion in the hypothalamus not visible on MRI or by small fibre neuropathy leading to disturbed body temperature perception and therefore impaired thermoregulation.
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