Edema, Familial Idiopathic, Prepubertal

Dunnigan and Pelosi (1993) reported an experience with 18 children from 13 unrelated kindreds with idiopathic edema commencing between the neonatal period and age 12 years. A triad of swelling, affective disturbance and functional autonomic symptoms was observed. Sixteen of the children (89%), including all 15 girls, had a family history of idiopathic edema, and 12 children (67%) had a family history of diabetes mellitus. Adherence to a restricted carbohydrate diet and the avoidance of refined carbohydrate appeared to be beneficial. Dunnigan and Pelosi (1993) presented the pedigree of a kindred in which the grandmother, all 4 daughters, and all 5 grandchildren (1 male, 4 females) showed idiopathic edema. Some of the children had severe vomiting, often projectile, beginning at an early age, frequent micturition with poor bladder control; frequent 'accidents' were noted. Irritability, lability of mood, lassitude and fatigue accompanied swelling in some of the children. Intermittent swelling of the face and abdomen was noted as early as the first year of life. Children with later onset often had swelling beginning after a period of weight gain.