[Hereditary sensory and autonomic neuropathy type IV: A report on two cases.]
Authors: Achouri E, Gribaa M, Bouguila J, Haddad S, Souayeh N, Saad A, Essoussi AS Hereditary sensory and autonomic neuropathy type IV (HSAN IV) is a very rare autosomal recessive disorder characterized by recurrent episodes of unexplained fever, extensive anhidrosis, total insensitivity to pain, hypotonia, and mental retardation. The absence of urticarial reaction to intradermal injection of histamine is a sign of great diagnostic value, but this is common to all types of HSAN. The most frequent complications of this disease are corneal scarring, multiple fractures, joint deformities, osteomyelitis, and disabling self-mutilations. Malignant hyperthermia and sepsis are major causes of mortality. We relate the first observations of two Tunisian children with genetically confirmed HSAN I...

CHLORPROMAZINE HYDROCHLORIDEtablet, Sugar Coated [Sandoz Inc]
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