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. There is anhidrosis due to lack of innervation of normal sweat glands resulting in recurrent episodes of hyperpyrexia. These patients usually present in late infancy. Here we present a case of HSAN Type IV who presented with fever from day 2 of life and so the possibility of HSAN should be kept in mind while dealing a case of fever without focus in a neonate.
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