er disease, male infertility, and an elevation of sweat chloride levels (4:33). Most patients die before the end of their third decade. Cystic fibrosis occurs in all ethnic groups and in all geographic locations (11:133). It tends to occur most frequently, however, in Caucasians. It is most prevalent in Northern and Central Europe, Canada, and the United States. Moreover, high incidence rates have been observed in Australia and among the white population of South Africa (4:56).
The disease affects about 1 in every 2,500 Caucasian newborns (2:7). Thus, cystic fibrosis is a relatively common genetic disorder (11:133). In addition, it follows an autosomal recessive inheritance pattern. According to its frequency then, about 1 in 25 Caucasians is a carrier. This high carrier rate as well as the disease's lethality has made CF the subject of much research.
The first historical references to cystic fibrosis can be found in European folklore. For example, one quote from a book titled German Children's Songs and Games read as follows: "the child will die soon, whose forehead tastes salty when kissed" (12:718). These children were described as "bewitched," and some cultures recommended that they not be baptized.
While such early references most certainly pertained to victims of CF, the disease itself was not accurately described until the first half of the 20th century (11:133). In 1936, Fanconi wrote about a "coeliac syndrome with pancreatic changes." He noted that these changes were different from regular coeliac disease. Then, two years later, Anderson (1938) published a paper describing meconium ileus and its treatment. It was not until 1950 though that Farber called this disease mucoviscidosis. Even today the term persists as part of the logo of the International Cystic Fibrosis (Mucoviscidosis) Association (ICF(M)A) (12:718).
Strides in the delineation of CF occurred in the summer of 1951. That year a h...