Cystic fibrosis in Denmark 1945 to 1985. An analysis of incidence, mortality and influence of centralized treatment on survival

TY - JOUR

T1 - Cystic fibrosis in Denmark 1945 to 1985. An analysis of incidence, mortality and influence of centralized treatment on survival

AU - Nielsen, O H

AU - Thomsen, B L

AU - Green, A

AU - Andersen, P K

AU - Hauge, M

AU - Schiøtz, P O

PY - 1988/11

Y1 - 1988/11

N2 - The entire population of cystic fibrosis (CF) patients in Denmark diagnosed in the period January 1, 1945, to June 30, 1985, comprised 514 persons of whom 62 were diagnosed at autopsy. Of the remaining 452 patients, 372 were probands and 80 were secondary cases. The effect of a centralized treatment on the prognosis was evaluated using a Cox's regression model. Furthermore an incidence analysis was performed using probands including autopsies. Centralized treatment had a significantly beneficial effect on the prognosis (p = 0.02), the death intensity (hazard rate) being decreased by a factor of 0.60. It was calculated that at present 1 out of 4,760 newborn Danes may be diagnosed as suffering from CF before the age of 15 years, and 3% of the Danish population are heterozygotes for CF.

AB - The entire population of cystic fibrosis (CF) patients in Denmark diagnosed in the period January 1, 1945, to June 30, 1985, comprised 514 persons of whom 62 were diagnosed at autopsy. Of the remaining 452 patients, 372 were probands and 80 were secondary cases. The effect of a centralized treatment on the prognosis was evaluated using a Cox's regression model. Furthermore an incidence analysis was performed using probands including autopsies. Centralized treatment had a significantly beneficial effect on the prognosis (p = 0.02), the death intensity (hazard rate) being decreased by a factor of 0.60. It was calculated that at present 1 out of 4,760 newborn Danes may be diagnosed as suffering from CF before the age of 15 years, and 3% of the Danish population are heterozygotes for CF.

KW - Adolescent

KW - Adult

KW - Anti-Bacterial Agents/therapeutic use

KW - Child

KW - Child, Preschool

KW - Cohort Studies

KW - Cystic Fibrosis/drug therapy

KW - Denmark

KW - Female

KW - Forced Expiratory Volume

KW - Humans

KW - Infant

KW - Male

KW - Prognosis

M3 - Journal article

C2 - 3264656

SN - 0001-656X

VL - 77

SP - 836

EP - 841

JO - Acta Paediatrica Scandinavica

JF - Acta Paediatrica Scandinavica

IS - 6

ER -