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Hereditary sensory and autonomic neuropathy types IV and V in Japan.
Pediatr Int 2015; 57(1):30-6PI

Abstract

Hereditary sensory and autonomic neuropathy (HSAN) is a group of genetic disorders involving varying sensory and autonomic dysfunction. HSAN types IV and V are characterized by congenital generalized loss of pain and thermal sensation. HSAN type IV is additionally accompanied by decreased sweating and intellectual disability. From 2010 to 2013, we (members of the Japanese Research Group on Congenital Insensitivity to Pain) carried out research on HSAN types IV and V. Research by this group included epidemiological data, examination of clinical findings, solutions of disease etiology, investigation of complications and development of their management. Complications were categorized into musculoskeletal complications, oral/dental complications, dermal complications, ocular complications, complications resulting from impaired thermal control, anesthetic considerations, other complications possibly related to autonomic dysfunction, and abnormal mental development and behavior. Treatment and care for patients with HSAN types IV and V require a wide range of knowledge and experience, and a multidisciplinary team approach. Therefore, we produced the "Guideline of Total Management and Care for Congenital Insensitivity to Pain (Ver.1)" in 2012, to provide information for medical specialists based on our knowledge and experience. This guideline includes medical issues, as well as descriptions of social participation and welfare. This review outlines the situation of HSAN types IV and V in Japan, and the recommendations of treatment and care for patients, mostly based on research conducted by the Japanese Research Group.

Authors+Show Affiliations

Department of Rehabilitation Medicine, University of Tokyo, Tokyo, Japan.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

25422087

Citation

Haga, Nobuhiko, et al. "Hereditary Sensory and Autonomic Neuropathy Types IV and V in Japan." Pediatrics International : Official Journal of the Japan Pediatric Society, vol. 57, no. 1, 2015, pp. 30-6.
Haga N, Kubota M, Miwa Z, et al. Hereditary sensory and autonomic neuropathy types IV and V in Japan. Pediatr Int. 2015;57(1):30-6.
Haga, N., Kubota, M., & Miwa, Z. (2015). Hereditary sensory and autonomic neuropathy types IV and V in Japan. Pediatrics International : Official Journal of the Japan Pediatric Society, 57(1), pp. 30-6. doi:10.1111/ped.12538.
Haga N, et al. Hereditary Sensory and Autonomic Neuropathy Types IV and V in Japan. Pediatr Int. 2015;57(1):30-6. PubMed PMID: 25422087.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Hereditary sensory and autonomic neuropathy types IV and V in Japan. AU - Haga,Nobuhiko, AU - Kubota,Masaya, AU - Miwa,Zenzo, AU - ,, PY - 2014/09/03/received PY - 2014/10/26/revised PY - 2014/10/29/accepted PY - 2014/11/26/entrez PY - 2014/11/26/pubmed PY - 2016/12/15/medline KW - Charcot's joint KW - anhidrosis KW - complications KW - congenital insensitivity to pain KW - hereditary sensory and autonomic neuropathies SP - 30 EP - 6 JF - Pediatrics international : official journal of the Japan Pediatric Society JO - Pediatr Int VL - 57 IS - 1 N2 - Hereditary sensory and autonomic neuropathy (HSAN) is a group of genetic disorders involving varying sensory and autonomic dysfunction. HSAN types IV and V are characterized by congenital generalized loss of pain and thermal sensation. HSAN type IV is additionally accompanied by decreased sweating and intellectual disability. From 2010 to 2013, we (members of the Japanese Research Group on Congenital Insensitivity to Pain) carried out research on HSAN types IV and V. Research by this group included epidemiological data, examination of clinical findings, solutions of disease etiology, investigation of complications and development of their management. Complications were categorized into musculoskeletal complications, oral/dental complications, dermal complications, ocular complications, complications resulting from impaired thermal control, anesthetic considerations, other complications possibly related to autonomic dysfunction, and abnormal mental development and behavior. Treatment and care for patients with HSAN types IV and V require a wide range of knowledge and experience, and a multidisciplinary team approach. Therefore, we produced the "Guideline of Total Management and Care for Congenital Insensitivity to Pain (Ver.1)" in 2012, to provide information for medical specialists based on our knowledge and experience. This guideline includes medical issues, as well as descriptions of social participation and welfare. This review outlines the situation of HSAN types IV and V in Japan, and the recommendations of treatment and care for patients, mostly based on research conducted by the Japanese Research Group. SN - 1442-200X UR - https://www.unboundmedicine.com/medline/citation/25422087/Hereditary_sensory_and_autonomic_neuropathy_types_IV_and_V_in_Japan_ L2 - https://doi.org/10.1111/ped.12538 DB - PRIME DP - Unbound Medicine ER -