Origin of the Cystic Fibrosis Sweat Test

According to the Cystic Fibrosis Foundation, in the 1950s, few children with cystic fibrosis lived long enough to attend elementary school. Prior to the 1950s, however, there was no definitive test to determine whether a child was actually suffering from this fatal lung disease. This changed with the development of what is now considered to be the gold standard in cystic fibrosis diagnostic tests, the sweat test, in that decade.

As stated in a story on CFMedicine.com, Cystic Fibrosis in the Fifties, a New York City heat wave in 1948 led to the development of the sweat test. During that heat wave, according to CF pioneer Dr. Dorothy Andersen, 10 patients were admitted to her hospital with heat prostration, with two others admitted in following years. Of those 12 individuals, no less than seven were known to have cystic fibrosis, as stated by Dr. Andersen in a 1951 paper on this topic.

At that time, it was not understood why CF patients were susceptible to heat prostration and salt depletion that was the basis for the above circumstance noted by Dr. Andersen. However, another cystic fibrosis pioneer, Dr. Paul di Sant’Agnese, utilized findings related to heat tolerance and the adaptation to sweating among troops sent to North Africa as a basis for studying the sweat function in CF patients. Dr. di Sant’Agnese composed this research “as a shot in the dark to see if sweating function was impaired in CF patients that would lead to a smaller than normal volume of sweat, or whether there was something wrong with the sweat electrolyte concentration.” Accordingly, in 1952, Dr. di Sant’Agnese performed testing on four teenagers, two of whom had cystic fibrosis, to review this topic. It was discovered that although the amount of sweat between the CF and non-CF participants was similar, the sweat electrolyte concentration among the two groups was significantly different.

A paper published by Dr. di Sant’Agnese in 1953 released the results of a subsequent sweat study he designed that was performed on 102 subjects, 43 of whom had cystic fibrosis, which confirmed the difference in sweat electrolyte concentration among those with and without CF. It was found that in the perspiration collected, sweat chloride in the cystic fibrosis patients was 106 meq/l (milliequivalents/liter) versus 32 meq/l in the other individuals, and the sodium content in the sweat was 133 meq/l in CF patients against 59 meq/l for the others.

Regardless of this finding being arguably the most important advance in the understanding of cystic fibrosis at the time, it was not recognized as such when first introduced. Dr. di Sant’Agnese recalled that the importance of his findings received a very cool reception when introduced at a 1953 meeting of the American Pediatric Association. In fact, when the findings were presented to a distinguished sweat physiologist of the time, the individual uttered the word “impossible” before leaving the room. Further, even the above-mentioned Dr. Andersen was skeptical of the presented results at first. However, a colleague of Dr. di Sant’Agnese found this information to be much more worthwhile, and was subsequently able to garner support and acceptance of the ground-breaking sweat study in a 1954 presentation.

Source:

Cystic Fibrosis in the Fifties , CFMedicine.com


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