title: Hereditary sensory autonomic neuropathy Type IV.
abstract: Hereditary sensory autonomic neuropathy Type IV is an autosomal recessive disorder due to lack of maturation of small myelinated and unmyelinated fibers of peripheral nerves, which convey sensation of pain and temperature, therefore, resulting in self mutilation. There is anhidrosis due to lack of innervation of normal sweat glands resulting in recurrent episodes of hyperpyrexia. The clinical presentation of two children with this rare disease is described.
authors.affiliation: University College of Medical Sciences, New Delhi, India. tdua@sify.com
authors.forename: Tarun
authors.initials: T
authors.lastname: Dua
authors.forename: Jyoti
authors.initials: J
authors.lastname: Sharma
authors.forename: Tanu
authors.initials: T
authors.lastname: Singhal
authors.forename: Ved Bhushan
authors.initials: VB
authors.lastname: Arya
mesh_terms: D002648:Child; D005260:Female; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000175:diagnosis*; D006358:Hot Temperature / Q000009:adverse effects; D006801:Humans; D007223:Infant; D008297:Male; D019957:Motor Skills Disorders / Q000235:genetics; D010147:Pain Measurement; D012652:Self Mutilation / Q000235:genetics