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To distinguish hereditary peripheral neuropathies (HPN) from other forms of neuropathies a longitudinal approach of the family tree is recommended. HPN and Charcot-Marie-Tooth disease (CMT) are the two most common neurological disorders. A boy with CMT, neuropathy, ataxia and mental retardation, and his sister with CMT 1A and spinal muscular atrophy were diagnosed by clinical and genetic analysis. A middle-aged patient presented with pain, sensory ataxia, and fixed kyphosis was found to have polyneuropathy, Charcot-Marie-Tooth disease type 1B (CMT1B). A young man with neuropathy and electrophysiological abnormalities was diagnosed with autosomal recessive inherited CMT2. All patients had normal cAMP responsive-element binding protein (CREB) protein expression. Mutations in 12 genes had been detected among 6 patients with hereditary neuropathy. With further efforts the genetics of the other 3 patients were proved negative. HPN may go undiagnosed unless a family tree is drawn.Evaluation of the COPD assessment test among healthcare workers in an intensive care unit.
The COPD Assessment Test (CAT) has been shown to be a reliable and valid tool to measure the impact of COPD on patients’ daily living, regardless of COPD status. The aim of this study was to evaluate the performance of CAT for detecting undiagnosed COPD. We performed a cross-sectional study among adult healthcare workers in a large tertiary university-affiliated medical centre in Taiwan. We developed a visual analogue scale to assess respiratory symptoms, and administered the CAT, an eight-item questionnaire designed to measure the impact of COPD on daily living. One hundred and one participants completed the questionnaire and underwent pulmonary function testing. Twenty participants were diagnosed with COPD, two had chronic bronchitis, and 79 had a smoking history. The total CAT score was significantly lower in the undiagnosed group than in the control group. The mean CAT scores of the control group were 13.7 ± 6.9 and the mean CAT score of the undiagnosed
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