Læknablaðið - 15.09.1980, Blaðsíða 22

208
LÆKNABLADID
hjarta eru eðlileg. Er talið vafalítið, að hún hafi
sjúkdóminn á byrjunarstigi. Sonurinn (III, 3)
hefur ekki sjúkdóminn.
í þessari ætt hefur sjúkdómurinn erfst
samkvæmt ríkjandi erfðaeiginleika (Mendeli-
an dominant inheritance) og er pað í samræmi
við niðurstöður úr svipuðum og viðameiri
rannsóknum annars staðar (2, 5).
PAKKIR
Við pökkum dr. Árna Kristinssyni leiðbein-
ingar og aðstoð við læknisskoðanir á sjúkling-
um, dr. Ólafi Jenssyni ábendingar um uppsetn-
ingu efnis og Erfðafræðinefnd Háskólans
könnun á ættartengslum.
SUMMARY
In lceland most autopsies are performed in the
Department of Pathology at the University of
lceland. The autopsy rate is high and relatively
unselected and therefore well suited for population
studies of chronic diseases. In a series of 3598 male
and 2182 female autopsies, which represent 37.5 per
cent of males and 28.7 per cent of females who died
in the 12 year period 1966-1977, 11 patients were
found with hypertrophic cardiomyopathy. The inci-
dence for males was 0.22 per cent and for females
0.14 per cent. By using the autopsy rate and the
incidence found in the series, one can estimate that
an average of 2.5 patients per year had died with
hypertrophic cardiomyopathy, which corresponds
to 0.17 per cent of the deaths in the population.
Nine of the 11 patients died under the age of 50
and 8 were males. Hypertrophic cardiomyopathy
thus accounts for a considerable proportion of
cardiac deaths among young males.
The records of 5 of the 11 patients contained
information about the occurence of hypertrophic
cardiomyopathy among one or more close relatives,
which gives an incidence of probable genetic
transmission in 46 per cent of the group.
HEIMILDIR
1. Braunwald, E. The natural history of idiopathic
hypertrophic subaortic stenosis, í Ciba Founda-
tion Study Group, No 37, Hypertrophic obstruc-
tive cardiomyopathy (ed. Wolsteinholm, G. E.
W. and O'Connor, M.), 30-41. (Churchill). Lon-
don 1971.
2. Brent, L. B., Aburano, A., Fisher, D. L., Moran, T,
J., Myers J. D. and Taylor, W. J. Familial
muscular subaortic stenosis. An unrecognized
form of »idiopathic heart disease«, with clinical
and autopsy observations. Circulations 21: 167,
1960.
3. Brock, R. Functional obstruction of the left
. ventricle (acquired aortic subvalvar stenosis),
Guy’s Hosp. Rep. 106: 221, 1957.
4. Brock, R. Functional obstruction of the left
ventricle (acquired aortic suvvalvar stenosis).
Guy’s Hosp. Rep. 108: T26, 1959.
5. Clark, C. E. Henry, W. L. and Epstein, S. E.
Familial prevalence and genetic transmission of
idiopathic hypertrophic subaortic stenosis. N.
Engl. J. Med. 289: 709, 1973.
6. Cohen, J. Effat, H., Goodwin, J. F., Oakley, C .M.
and Steiner, R. E. Hypertrophic obstructive
cardiomyopathy. Br. Heart. J. 26: 16, 1964.
7. Emanuel, R., Withers, R. and O’Brien, K. Domin-
ant and recessive modes of inheritance in
idiopathic cardiomyopathy. Lancet 2: 1065,
1971.
8. Emanuel, R. The familial incidence of idiopathic
cardiomyopathy, í Ciba Foundation Study Gro-
up, No 37, Hypertrophic obstructive cardiomyo-
pathy (ed. Wolsteinholm, G. E. W. and O’Con-
nor, M.) 50-54. (Churchill). London 1971.
9. Frank, S. and Braunwald, E. Idiopathic hyper-
trophic subaortic stenosis. Clinical analysis of
126 patients with emphasis on the natural
history. Circulation 37: 759, 1968.
10. Goodwin, J. F. Congestive and hypertrophic
cardiomyopathies. A decade of study. Lancet 1:
731, 1970.
11. Hallgrímsson, J. Chronic rheumatic valvular
heart disease. An autopsy study. Acta path.
microbiol. scand. Sect. A, 83: 633, 1975.
12. Hallgrímsson, J. and Tulinius, H. Chronic non-
rheumatic aortic valvular disease. A population
study based on autopsies. J. Chron. Dis. 32: 355,
1979.
13. Hardarson, T., De La Calzada, C. S., Curiel, R.
and Goodwin, J. F. Prognosis and mortality of
hypertrophic obstructive cardiomyopathy. Lan-
cet 2: 1462, 1973.
14. Roberts, W. C. Cardiomyopathy and myocardi-
tis: morphological features. Adv. Cardiol. 22:
184, 1978.
15. Teare, D. Asymmetrical hypertrophy of the
heart in young adults. Br. Heart J. 20: 1, 1958.
16. Whiting, R. B., Powell, W. J„ Dinsmore, R. E. and
Sanders, C. A. Idiopathic hypertrophic subaortic
stenosis in the elderly. N. Engl. J. Med. 285: 196,
1971.
17. Þjóðleifsson, B. Dauðsföll af völdum kransæða-
sjúkdóma á íslandi 1951-1976. Læknablaðið 64:
55, 1978.