LONG CHAIN NON-ESTERIFIED FATTY ACID PATTERN IN PLASMA OF CYSTIC FIBROSIS PATIENTS AND THEIR PARENTS

Abstract

Summary: The absolute and the percentage plasma NEFA pattern of healthy children, Cystic Fibrosis (C.F.) patients and their parents have been determined (table I, III, V) and compared with those of age and sex matched control subjects (table II, IV, V).

There is a striking difference in the percentage plasma NEFA pattern of C.F. patients with pancreatic insufficiency: palmitic, palmitoleic and oleic acid are significantly increased while linoleic, linolenic and stearic acid are decreased (table IV). Four C.F. children without pancreatic involvement have the same abnormal NEFA pattern (fig. 3).

The overlapping areas of the distributions of both palmitoleic and linoleic acid (as shown in fig. 2α and β) are small for healthy and C.F. subjects. About 90% of the C.F. subjects examined show an abnormal palmitoleic and/or linoleic acid concentration. Tested individually, recognition of this typical C.F. pattern involves a possible error of 15% (fig. 3).

A correlation exists between the abnormality of the plasma NEFA pattern in C.F. patients and the severity of pulmonary disease.

The percentage plasma NEFA pattern of C.F. parents is modified by an increase of linoleic acid and a decrease of saturated fatty acids (table V, fig. 1α,β). This is possibly due to an higher dietary intake of polyunsaturated fatty acids.

Speculation: It has not been convincingly demonstrated yet that the abnormal plasma NEFA pattern in Cystic Fibrosis is entirely due to malabsorption of dietary essential fatty acids. Rather, a primary defect in fatty acid metabolism also remains possible.

Log in or create a free account to read this content

Gain free access to this article, as well as selected content from this journal and more on nature.com

Access through your institution

or

Sign in or create an account

Continue with Google

Continue with ORCiD