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The Director of Pathology of London's Hospital for Sick Children, in collaboration with a pediatrician and a geneticist, thoroughly reviews a series of one hundred sixteen cases seen from 1943 through 1950.
Initially, pertinent literature up through 1951 is surveyed. Two points are particularly stressed: the evolution of the concept of the pathogenesis of the disease to the present "mucosis" hypothesis and the evidence for meconium ileus being its earliest clinical form.
The study reveals that although the first major symptom occurs in the neonatal period in 80% of cases and by one year of age in all but one case in the series, 50% of cases are not diagnosed until after one year of age. The clinical picture is reviewed, and the laboratory diagnosis is discussed. The latter principally concerns duodenal fluid trypsin levels. That the electrolyte concentration of sweat is not mentioned is to be expected because the
Fibrocystic Disease of the Pancreas.. AMA Am J Dis Child. 1956;91(1):86-87. doi:10.1001/archpedi.1956.02060020088018