Improved prognosis in CF patients with normal fat absorption.

CF patients with normal fat absorption, as a group, have lower mean sweat chloride levels, maintain better pulmonary function and weight for their age, and appear to survive longer than CF patients with steatorrhea. The prognostic advantage for CF males in general is not seen in the pulmonary function data for patients with normal fat absorption, but may be reflected in the smaller number of females in this group. Males in both groups are clearly better at maintaining good weight than are females. Whether this means that nutritional intervention can improve pulmonary course or that other factors (genetic, endocrinological, environmental) dictate nutritional and pulmonary state, as well as sex differences, remains to be shown. Non-steatorrheic patients are far less likely than steatorrheic patients to have Pseudomonas infecting their lungs. This is a significant prognostic advantage since the progressive lung disease and eventual mortality of most CF patients can be charted by their acquisition of P. aeruginosa and the increasing frequency of exacerbation and attempts to eradicate this organism. CF has been called a lethal genetic disease because affected homozygotes did not generally survive to procreate. However, increasing numbers of young women with CF are surviving to an age where pregnancy and child-rearing are options for them. The majority of patients who reach this stage with sufficiently good health to embark on a pregnancy are patients with normal fat absorption. They also appear to be more likely to remain well throughout pregnancy and as young mothers. There is no doubt that CF patients with normal fat absorption have a better prognosis than those with typical CF malabsorption.(ABSTRACT TRUNCATED AT 250 WORDS)