Epidemiology of Hereditary Sensory and Autonomic Neuropathy Type IV and v in Japan.

Epidemiology of hereditary sensory and autonomic neuropathy type IV and V in Japan.

Am J Med Genet A. 2013 Mar 12;
Haga N, Kubota M, Miwa Z

Hereditary sensory and autonomic neuropathy (HASN) refers to a group of rare congenital disorders characterized by loss of pain sensation and other sensory or autonomic abnormalities. Among them, a relatively large proportion of patients with HSAN type IV, which is accompanied by anhidrosis and intellectual disability, are reported from Israel and Japan. HSAN type V, with normal sweating and mental development, is rarely reported in Japan. In 2009, we founded a research group for congenital insensitivity to pain and performed the first epidemiological survey of HSAN types IV and V in Japan. Questionnaires were sent to a total of 3,488 certified training institutions of five nationwide medical societies comprising pediatricians, neurologists, orthopedic surgeons, and dentists. Answers were obtained from 1,610 institutions, and 192 HSAN patients (152 with type IV and 28 with type V) were reported from 105 institutions. After excluding duplicated patients, we identified a total of 62 current, 36 past, and five deceased patients for HSAN-IV, and a total of 14 current, 13 past, and 0 deceased patients for HSAN-V. Using these figures, we estimated that the number of Japanese patients with HSAN types IV and V as 130-210 and 30-60 patients, respectively. We identified no gender differences, and patients with a family history of the disorder were limited to affected siblings in both conditions. Most patients with HSAN-IV were 5-40 years of age, whereas half of the patients with HSAN-V were 40 years or older. © 2013 Wiley Periodicals, Inc. HubMed – rehab


Response to letter to editor.

Arthritis Care Res (Hoboken). 2013 Mar 12;
Grossman J, Hahn BH, Maureen M, Joan T M, Yazdany J, Ramsey-Goldman R, Daniel W, Fitzgerald J

HubMed – rehab


An experimental assessment of the influence of exercise versus social implementation intentions on physical activity during and following pulmonary rehabilitation.

J Behav Med. 2013 Mar 15;
Rodgers WM, Selzler AM, Haennel RG, Holm S, Wong EY, Stickland MK

Techniques to increase physical activity among pulmonary rehabilitation patients outside of the rehabilitation context are warranted. Implementation intentions are a strategy used to initiate goal-directed behaviour, and have been found to be useful in other populations. This study compared the long-term effects of exercise and social implementation intentions interventions on objectively measured physical activity in 40 pulmonary rehabilitation patients randomly assigned to condition. Repeated measures ANOVAs found that those in the exercise implementation intentions group took more steps (p = .007) at the end of pulmonary rehabilitation than those in the social implementation intentions group. Improvements attained by the exercise group during the intervention were not maintained 6-months following rehabilitation. Implementation intentions targeting physical activity appear to have positive short term effects on physical activity, although the long term effects are less consistent. This may be due in part to methods used to assess physical activity behaviour. HubMed – rehab



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