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Assessment of nutritional status of patients with cystic fibrosis of the pancreas (CFP) showed that poor growth was associated with low concentrations of albumin, urea nitrogen, and cholesterol in serum and with elevated white blood cell (WBC) counts. Patients with CFP maintained weight approximately 1 standard deviation below the mean until age 8 years, after which there was a progressive decline in growth rate compared to normal. A complete dietary supplement consisting of a beef serum hydrolysate, a glucose polymer, and medium-chain triglycerides was given to 15 patients for ayear. Patients who received the diet showed significant gains in weight, significant increase in clinical score, significant increase in serum albumin level, and a significant drop in the WBC count compared to control patients who did not receive the supplement.Pancreatic insufficiency, leading to serious impairment of diges¬ tive functions, can be demonstrated in approximately 85% of patients with cystic fibrosis of the pancreas (CFP). In the majority of patients, progressive malnutrition becomes a feature of the disease in spite of sup¬ plementary enzyme therapy and con¬ sumption of more than the recom¬ mended allowance for calories and protein.1 Shohl et al, in 1943, reported studies of nitrogen and fat metabolism in in¬ fants and children with CFP.2 A striking feature of the disease was the excess nitrogen found in the bulky feces when regular diets con¬ taining whole protein and fat were consumed. When a fat-free diet con¬ sisting of casein hydrolysate and glu¬ cose was administered, nitrogen re¬ tention was in the range found in normal infants, and the amount of fe¬ cal excretion and the nitrogen con¬ tent were reduced to within normal limits. They concluded that even mod¬ erate amounts of protein were not di¬ gested and absorbed, but that gener¬ ous amounts of casein hydrolysate could be utilized. Administration of pancreatin did not lead to any im¬ provement in fat metabolism. Their objective was to develop a rational therapy for the disease by using a diet that was within the digestive ca¬ pacity of the patient.In 1946 West and others3 compared the effects of absorption of whole pro¬ tein with that of a protein hydroly¬ sate in normal children; in sick, mal¬ nourished children; and in children with CFP. They found increases in the blood amino nitrogen level of 1 to 4 mg/100 ml above fasting levels within lk to V-k hours after oral feed¬ ing of whole protein. Children with CFP showed either no elevation or a drop in blood amino nitrogen level and only a slow rise from fasting lev¬ els within three to five hours. Follow¬ ing ingestion of casein hydrolysate, children with CFP and control chil¬ dren showed similar increases in the blood amino nitrogen level. They also concluded that substitution of hydrolyzed protein for intact protein should have therapeutic value for patients with CFP.Substitution of medium-chain tri¬ glycérides (MCT) for long-chain fats was shown to be beneficial to patients with CFP in reducing the bulk and frequ...

The reaction of skin with dihydroxyacetone to produce a brown "artificial tan" appears to proceed through combination with free amino groups in skin proteins, and particularly by combination of dihydroxyacetone with the free guanido group in arginine.