Hypercholesterolemia in children with cystinosis.
Ten children with cystinosis had their diet, serum creatinine, and total cholesterol (averaged in 6-month blocks and normalized by calculating a z score (TCz)] monitored for a mean of 5 years. The TCz scores were elevated in boys (P < 0.01) but not girls (P = 0.67). The boys had elevated TCz scores for 58% of their 29 pre-transplantation years of follow-up, rising as early as 1 year of age at a mean rate of 0.67 standard deviations per year. Cystinotic boys had higher TCz scores than children with other tubular disorders (P < 0.025). All 4 cystinotic boys who developed renal insufficiency had a rise in TCz scores at a rate greater than control children with comparable renal impairment. Two cystinotic boys received a renal transplant; subsequent TCz scores were markedly lower, but remained elevated. The nutritional status of cystinotic children deteriorates after 3 years of age: they have decreased fat stores, elevated TCz scores and consume high fat diets insufficient in total calories. In summary, cystinotic boys have marked, progressive hypercholesterolemia which improves after renal transplantation.
Murphy
J L
JL
Department of Pediatrics, University of California, Davis, Sacramento 95817.
Papathakis
P C
PC
D002648:Child; D002675:Child, Preschool; D002784:Cholesterol / Q000097:blood; D003404:Creatinine / Q000097:blood; D003554:Cystinosis / Q000097:blood*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006937:Hypercholesterolemia / Q000097:blood*; D007223:Infant; D008297:Male; D009752:Nutritional Status; D051437:Renal Insufficiency / Q000097:blood
D002784:Cholesterol; D003404:Creatinine