Pediatric victims of unexplained stroke and their families: familial lipid and lipoprotein abnormalities
Glueck, C.J.; Daniels, S.R.; Bates, S.; Benton, C.; Tracy, T.; Third, J.L.
Pediatrics 69(3): 308-316
1982
ISSN/ISBN: 0031-4005 PMID: 6977760 Document Number: 184104
The finding of low levels of high-density lipoprotein cholesterol (C-HDL) and/or high levels of triglyceride in several children with unexplained ischemic cerebrovascular accidents led to a systemic review of lipids and lipoproteins in 11 pediatric victims of unexplained stroke and their families. Each of the children, 1-17 yr of age, had an acute, nonhemorrhagic cerebrovascular accident documented by symptoms, signs, history, physical examination and laboratory radiographic studies; no known causes or strong predisposing factors were elicited. Of the 11 pediatric stroke probands, only 1 had normal lipid and lipoprotein levels; 5 had low C-HDL levels alone, 2 had elevated levels of plasma triglycerides with low C-HDL levels, 2 had high levels of triglycerides with normal C-HDL levels, and 1 had high levels of low-density lipoprotein cholesterol (C-LDL) alone. A consistent trend toward familial clustering of low C-HDL, elevated triglyceride and/or C-LDL levels was observed. Nine of the 11 kindreds had 2 generation parent:child stroke proband, or parent:child clustering of elevated triglyceride and/or depressed C-HDL levels, while 1 parent:child stroke proband pair had elevated levels of C-LDL. In 5 of 9 kindreds for whom siblings of the pediatric stroke probands were available for testing, there were low C-HDL and/or high triglyceride levels in otherwise healthy and entirely asymptomatic siblings. In 9 of the 11 kindreds premature coronary heart disease and/or ischemic cerebrovascular disease was observed in the probands' adult relatives. Familial lipoprotein abnormalities, particularly those involving low levels of C-HDL and/or high levels of triglycerides, may mediate occlusive cerebrovascular arteriosclerosis, and thus may predispose children to ischemic cerebrovascular strokes. The apparent association of lipoproteins and ischemic strokes in children and their families merits further exploration with a particular focus on familial aggregation of low C-HDL levels, already known to be a significant risk factor for ischemic cerebrovascular and cardiovascular disease in adults.