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Cystic fibrosis: Cell culture classes in a Danish population
Author(s) -
Danes B. Shan,
Beck B.,
Flensborg E. Winge
Publication year - 1978
Publication title -
clinical genetics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.543
H-Index - 102
eISSN - 1399-0004
pISSN - 0009-9163
DOI - 10.1111/j.1399-0004.1978.tb01188.x
Subject(s) - danish , cystic fibrosis , population , medicine , genetics , biology , pathology , environmental health , philosophy , linguistics
The cystic fibrosis (CF) culture phenotype of dermal fibroblasts (metachromasia, metabolic cooperation) of a group of 131 Danish CF patients and their families were studied to determine the distribution of the two CF culture classes and their prognostic significance. Of these, 62.6 % (82) were Class I, 31.3 % (41) Class II and 6.1 % (8) were proposed to be genetic compounds. The occurrence of Class II was twice that found in a group of patients from New York (13 %) and Minnesota (18 %). The prognosis for Class II CF patients was considered to be poorer as: (1) The initial diagnosis was made earlier in Class II than in Class I or the compounds (63 % of Class II were diagnosed in the first year of life, as compared to 35 % in Class I and 13 % of the compounds). (2) Only 5 % of the Class II patients survived over the age of 15 years, both being deceased at the end of the study in 1976, whereas 24 % of Class I and 63 % of the compounds were over 15 years at the end of the study. This research added further evidence for genetic heterogeneity within the clinical syndrome, cystic fibrosis.