The risk of family history of ischemic heart disease independent of other well described risk factors has remained difficult to quantitate. Significant coronary artery disease was determined by coronary arteriography to be present in 223 patients and absent in 57 control subjects. Age, sex, blood pressure, serum cholesterol, cigarette smoking and the presence of diabetes and left ventricular hypertrophy on the electrocardiogram were tabulated for each patient and the data used to assign a risk score based on the American Heart Association multivariate model. Subjects were stratified and matched according to risk score to estimate risk of family history independent of familial aggregation of these seven other risk factors. Angina, myocardial infarction, cardiac death and any ischemic heart disease were ascertained in 1,319 first degree relatives. Odds ratios for overall, stratified and matched comparisons of these end points in relatives of patients and control subjects ranged between 2.0 and 3.9 (p less than 0.01 for all comparisons), indicating a higher frequency of all ischemic heart disease end points in relatives of patients with documented coronary artery disease. Life table comparison of patients at lowest risk with those at higher risk showed significantly greater cumulative frequency and earlier age of onset of all ischemic heart disease end points in relatives of low risk patients. These observations indicate that some of the risk associated with family history is independent of familial aggregation of other known risk factors and suggest that the independent effects of family history may be most important in individuals who otherwise are at low risk.
SUMMARY All 995 persons with Down's syndrome who died in the United States during 1976 and whose death certificates listed Down's syndrome as the underlying or a contributing cause of death were identified. This allowed the underlying causes of death of 793 affected persons to be analysed and compared to deaths in the whole US population for that year. Mortality ratios provided evidence that the excess risk of leukemia mortality continues into adulthood and that deaths from other hematopoietic malignancies also occur excessively among Down's syndrome adults. Congenital anomalies of all kinds in infancy and congenital defects of the heart in infancy and later were also excessive. Respiratory tract infections and pneumonia showed persistently high ratios. Diabetes was raised only at ages 24 to 34 years. Ischemic heart disease, non‐hematopoietic cancers, accidents, suicides and violence were under‐represented among the causes of death. Methodological limitations of proportional mortality analysis are discussed. RÉSUMÉ Tous les 995 sujets porteurs de mongolisme, morts aux Etats‐Unis en 1976 et dont le certificat de décès mentionnait le mongolisme comme cause de décès sous‐jacente ou contributive ont été identifiés. Cela a permis d'analyser les causes de décès chez 793 sujets et une comparaison avec les causes de décès de la population générale aux Etats‐Unis pour la même année. Les taux de mortalité démontrent que le risque élevé de mortalité par leucémie persiste à l'âge adulte et que la mort par d'autre hémopathies malignes survien également avec un taux excessif chez les mongoliens adultes. Les anomalies congénitales de toutes sortes durant la première enfance, les affections cardiaques congénitales de l'enfance ou de survenue plus tardive étaient également d'un taux excessif. Les infections du tractus respiratoire et la pneumonie présentaient également des taux constamment élevés. Le diabète ne s'élevait que pour les âges de 24–34 ans. L'ischémie myocardique, les cancers non hémopathiques, les accidents, les suicides et les faits de violence étaient sous‐représentés parmi les causes de décès. La limitation méthodologique d'une analyse proportionnelle des causes de mortalité est discutée. ZUSAMMENFASSUNG Es wurde alle 995 Personen mit Down Syndrom erfaßt, die 1976 in den Vereinigten Staaten gestorben sind und auf deren Totenschein als primüre oder sekundäre Todesursache Down Syndrom angegeben war. Anhand der Daten konnten die primären Todesursachen von 793 Personen analysiert werden und mit den Todesfällen in der Gesamtpopulation der Vereinigten Staaten in diesem Jahr verglichen werden. Die Mortalitätszahlen weisen darauf hin, daß das sehr hohe Risiko der Leukämiesterblichkeit bis ins Erwachsenenalter fortbesteht und daß auch andere hämatopoetische Erkrankungen sehr häufig bei erwachsenen Patienten mit Down Syndrome zum Tode führen. Excessiv hoch waren die kongenitalen Mißbildungen verschiedener Art im Kindesalter und angeborene Herzfehler im Kindesalter und später. Der Anteil der respiratorischen Infekte und Pneu...
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