Cystic fibrosis in Southern Africa. Including the preparation of a register of carriers and potential carriers.

Little has been published on cystic fibrosis (CF) in Whites in southern 
Africa, and no figures as to incidence exist. A register of 
CF patients, their parents (obligatory carriers), siblings, uncles, aunts and first 
cousins (potential carriers) has been compiled for southern Africa. The degree 
of co-operation shown by colleagues and by families whose addresses have 
been provided by them, and possible reasons for non-co-operation are discussed. 
From the numbers and birth dates of patients a rough estimate 
of the incidence in the Republic of South Africa, South West 
Africa and Rhodesia has been made. In all three regions, but 
especially in South Africa, incidence is likely to have been underestimated. 
Details available from the register include the number of CF patients 
alive and dead, those who presented with meconium ileus, the number 
of affected patients per family, consanguinity among the parents or grandparents, 
the frequency with which identical surnames were encountered, and the sibship 
sizes of all those on the register. Towns and districts with 
a population rich in the CF gene are mentioned. The number 
of potential carriers has been determined, so that they can be 
screened when a practicable detection test is devised. The register has 
answered a number of questions about CF in southern Africa. It 
has focused attention on the disease in the region and played 
a major catalytic role in the formation of the Southern African 
Cystic Fibrosis Association. 