Emotional Implications of Primary Familial Hyperlipoproteinemia in Childhood and Adolescence

¹ Departments of Psychiatry and Paediatrics, Cardiology Division, The Hospital for Sick Children, Toronto

To determine whether 43 patients and their 23 unaffected siblings were at increased risk for psychologic upset, 28 families of children being treated for primary familial hyperlipoproteinemia were studied. Nine fathers but no mothers had died of the disease. The families were interviewed for psychiatric assessment, and the parent(s) scored a questionnaire concerning eight behavioral factors. Male patients had significantly higher scores for impulsive hyperactive behavior than their male siblings. Fatherless female patients had the highest scores for perfectionism and the lowest scores for conduct problems and impulsive hyperactive behavior. Fatherless children scored lower for impulsive hyperactive behavior than children with fathers; fatherless male children were more anxious than fatherless female children.

There were more significant differences in scores for children whose father was still alive vs those whose father had died than in scores for affected children vs unaffected siblings. Thus, the emotional impact of the premature death of an affected parent appears greater than that of having the disease. Therefore, one should be alert for changes in behavior in children with primary familial hyperlipoproteinemia when a parent dies of the disease and as the patients approach the age at which the parent died.