Nutritional rehabilitation of malnourished patients with cystic fibrosis.

We examined the effect of nutritional rehabilitation in cystic fibrosis patients with severe disease. Thirteen malnourished patients (seven males, six females, age 7-27 yr) were studied over 7-16 mo. Oral supplementation was attempted initially in 12 patients (mean duration 6.7 mo); only 2 patients gained weight, 2 withdrew, and 1 died. The remaining 7 patients failed to achieve adequate weight gain and were commenced on nasogastric supplementation with a semisynthetic formula. An additional patient was entered without a prior period of oral supplementation because of the severity of malnutrition. Weight gain was achieved in 7 of 8 patients with nasogastric supplementation (mean duration 6.4 mo). Weight gain was associated with an increase in lean body mass, total body fat, and height velocity. While pulmonary function and biochemical parameters were unchanged, patient well-being improved and episodes of pneumonia decreased.