Læknablaðið - 01.09.1964, Síða 99

LÆKNABLAÐIÐ
163
veldara en ella, og berum við
því þakkarhug til höfundar
þessa mikla framlags.3
Þakkarorð.
Við þökkum eftirtöldum
mönnum fyrir fyrirgreiðslu og
aðstoð:
Ólafi Sigurðssyni yfirlækni,
Óskari Þórðarsyni yfirlækni,
Sigurði Samúelssyni prófessor,
Sigmundi Magnússyni vfir-
lækni, Davíð Davíðssyni próf-
essor, Þóroddi Jónassjmi héraðs-
lækni, Magnúsi L. Stefánssyrii
lækni og Eggerli Ó. Jóhannssyni
yfirlækni. Enn fremur þökkum
við Kjartani Guðjónssyni list-
málara, Barböru Stanzeit-Guð-
jónsson og Indriða Indriðasvni
rithöfundi. Síðast, en ekki sízt,
þökkum við öllu því fólki, sem
við höfmn rannsakað, en það
hefur undantekningarlaust sýnf
góðan skilning og samvinnulip-
urð.
Ó. J. þakkar Vísindasjóði styrk
á árinu 1964.
Jensson, Ó. and Ólafsson, Ó.:
Elliptocytosis hereditaria
in Iceland.
SUMMARY.
Of 35 casas of hereditary ellipto-
cytosis diagnosed in Iceland 1958
—1964 clinical data and hæmato-
logical observations on over 20 is
reported in this paper. These are
the first cases of this hereditary
condition found in Iceland.
The main features of hereditary
elliptocytosis are reviewed. Pedi-
gree-data and illustrative clinical
histories of propasiti are present-
ed, supplemented by hæmatologi-
cal observations in some of the
cases. Majority of the 170 kindred
so far scanned for the elliptocytic
trait, including some spouses, have
been investigated only by study-
ing stained blood-film, wet pre-
paration occasionally .studied also.
Two cases have been splenec-
tomized with good results. Four
cases have had gallbladder opera-
tions.
The hereditary pattern is charac-
teristic, with non-significant devia-
tion from the expected ratio.
The penetrance seems to be com-
plete and expressivity fairly con-
stant, judged by the shape and
magnitude of the elliptocytas of
the affected. But variability of ex-
pressivity judged by varying grad-
es of hæmolysis within sibships is
found.
A probable common progenitor
(II-l pedigree) is found for the
great bulk of the elliptocytic. But
as all affected are from the same
area it is considered probable that
the same abnormal alleie is opera-
tive in all affected cases found
up to now.
In .spite of known high frequen-
cy of this allele and inbreeding
in the area in question no exam-
ples of homozygosis have been en-
countered yet.
Data on linkage and allele fre-
quency in the district is being col-
lected.
A book (geneological table) on
the kindred has been published
(see ref. 3). This has been very
helpful.
HEIMILD ASKRÁ:
1. Dacie, J. V. (1960): The Hæmo-
lytic Anæmias: Congenital and
Acquired. Part I: The Conge-
nital Anæmias, 2nd ed.; Church-
ill, London.