LORENZO'S OIL WITH DIET THERAPY DOES NOT AFFECT THE GROWTH OF ASYMPTOMATIC ALD BOYS

Objectives: X-linked adrenoleukodystrophy (XALD) is associated with the accumulation of saturated very long chain fatty acids (VLCFA) which is believed to contribute to the disease pathogenesis. The Lorenzo's oil (LO) with diet therapy normalizes the level of VLCFA in plasma. Aim of this study was to investigate the effects of this regimen on the physical growth of asymptomatic young boys.

Methods: 19 asymptomatic X-ALD boys (median age: 3.7 years, range: 1.6–6) were recruited between 2003–05 to investigate the efficacy of LO in conjunction with a low fat diet. LO, dietary fat and walnut oil provided 20%, 10% and 5% of total calorie need respectively. Diet ensured adequate calories and nutrients needed for normal growth. Z-scores of weight, height, arm muscle area (AMA) and arm fat area (AFA) were assessed as growth and body composition parameters using the 2000 CDC growth charts and Frisancho's anthropometric standards. Subjects complying with the diet and LO therapy for at least one year were included.

Results: 18 boys gained weight and height appropriate to their age. One boy gained weight and height but his respective z-scores decreased one standard deviation (4 vs. 3). Mean Z-scores of weight and height remained stable during the two years of therapy. Body composition remained unchanged for 15 boys (n=16) after one year of follow-up.

Conclusion: Our data indicate that the fat restrictive diet and LO which can prevent neurological damage in asymptomatic boys does not affect the normal physical growth.