Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The relationship between mortality and disability, was studied in SMON patients, who had participated in the questionnaire survey in 1980, by following them for 10 years through municipal resident registration information offices. Eighty deaths were observed among 409 subjects during the follow-up period. The effect of each disability on the mortality of SMON was estimated by applying the Cox proportional hazard model. The presence of severe gait disturbance and low levels of activities of daily living (ADL) were associated with significantly higher risk of death after adjustment for age and sex. The relative risk of visual disturbance was not significant after adjusting for age, sex and other kinds of disability. Those who complained of continuous dysesthesia at the time of the initial survey showed better prognosis than those without such dysesthesia. Among subjects who died during the follow-up period, those who had reported low levels of ADL at the time of the initial survey had a greater proportion of deaths attributable to heart failure and pneumonia than those who had reported high levels of ADL.
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PMID:[Mortality of SMON patients in relation to their disabilities]. 899 99

We report an autopsy case of a pedigree of familial amyloidotic polyneuropathy (FAP) with a mutation of isoleucine-50 transthyretin (ATTR Ser50Ile). A 47-year-old man started developing severe diarrhea and weight loss at age 41 years, followed by urinary incontinence, autonomic-nervous-system abnormalities and serious heart failure; the diagnosis of FAP (ATTR Ser50Ile) was made on the basis of genetic, histochemical and immunohistochemical analysis. Six years after the initial symptoms, he died of septic shock. Autopsy revealed suppurative peritonitis, perforation of the sigmoid colon and marked systemic amyloid deposition. The total amount of amyloid deposited in the heart was greatly increased and was much lower in the thyroid gland and kidneys compared with amyloid deposits in ordinary FAP (ATTR Val30Met). Amyloid deposition in peripheral vessel walls was prominent, particularly in lymphatics and veins. His elder sister, 54 years old, started to develop orthostatic hypotension at age 49 years, followed by dysesthesia, diarrhea and severe congestive heart failure. Endomyocardial biopsy revealed severe TTR-amyloid deposition; ultrastructural examination demonstrated that amyloid fibrils were deposited disproportionately and extended radially around microvessels.
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PMID:Familial amyloidotic polyneuropathy (ATTR Ser50Ile): the first autopsy case report. 1083 37