Familial hypercholesterolemia is associated with premature atherosclerosis. Since endothelial dysfunction is an early event in atherogenesis, we used a noninvasive method to assess endothelial function in the systemic arteries of 30 children aged 7-17 yr (median 11 ) with familial hypercholesterolemia (2 homozygotes, 28 heterozygotes, total cholesterol 240-696 mg/ dl) and 30 healthy age-and sex-matched controls. Using high resolution ultrasound, the diameter of the superficial femoral artery was measured at rest, in response to reactive hyperemia (with increased flow causing endothelium-dependent dilation), and after sublingual glyceryltrinitrate (causing endothelium-independent vasodilation).Flow-mediated dilation was present in the controls (7.5±0.7%) but was impaired or absent in the hypercholesterolemic children (1.2±0.4%, P < 0.0001). Total cholesterol was inversely correlated with flow-mediated dilation (r = -0.61, P < 0.0001). In the hypercholesterolemic children, flow-mediated dilation was inversely related to the lipoprotein(a) level (r = -0.61, P = 0.027) but not to other lipid fractions. Glyceryltrinitrate-induced dilation was present in all subjects but was lower in the hypercholesterolemia group (10.0±0.6% vs 12.4±0.8%, P = 0.023).Thus, impaired endothelium-dependent dilation is present in children with familial hypercholesterolemia as young as 7 yr of age and the degree of impairment is related to the lipoprotein(a) level. (J. Clin. Invest. 1994. 93:50-55.)
. Treatment of hypernatraemic dehydration in infancy. Thirty-eight infants with severe hyperosmolar dehydration and hypernatraemia were treated, using three regimens of intravenous fluids: A. i normal saline, given fast; B. i normal saline given slowly; C. normal saline. 28 of the infants were studied in a treatment trial, and it is concluded that 0 * 18% saline in 4 *3 % dextrose, with the early addition of potassium given at a rate of 100 ml/kg estimated rehydrated weight per 24 hours gives satisfactory rehydration within 48 hours, with little risk of convulsions.The best means of rehydrating hypernatraemic infants remains controversial. Though there is general agreement that reduction of hyperosmolality should be gradual, some achieve this with slow rates of fluid of low sodium content, while others use faster rates of high sodium content. It was therefore decided to study the use of different intravenous fluid regimens in severely affected infants. As the use of normal saline or full strength Darrow's solution has been incriminated in the production of hypernatraemia in infants originally suffering from normonatraemic dehydration (Skinner and Moll, 1956;Weil and Wallace, 1956; Stickler, 1967;Ahmed and Agusto-Odutola, 1970) this study was confined to a comparison of 0-18% saline in dextrose with 0 *45 /% saline in dextrose.Patients and method Infants suffering from hypernatraemic dehydration (plasma sodium > 150 mEq/l) and a measured plasma osmolality >350 mOsm/kg were studied. With the exception of one infant with Down's syndrome, infants known to have previous cerebral or renal abnormalities, which might have influenced the course of treatment, were excluded. During 18 months 37 infants with 38 episodes of hypematraemia of this severity were studied in 2 children's hospitals. All were suffering from primary water depletion, and subsequent urine excretion showed that all had some degree of sodium and potassium depletion. The primary cause of illness was gastroenteritis in 27, respiratory infection in 8, septicaemia in 2, and unknown in one. The one infant seen during this time with evidence of sodium
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