Low-density lipoprotein apheresis in children with familial hypercholesterolemia: follow-up to 21 years |
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Authors: | Palcoux Jean-Bernard Atassi-Dumont Marielle Lefevre Patrice Hequet Olivier Schlienger Jean-Louis Brignon Pierre Roussel Bernard |
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Affiliation: | 1. Department of Pediatrics, H?tel‐Dieu, Clermont‐Ferrand,;2. Clinical Center of Hemobiotherapy, Pitié Salpêtrière Hospital, Paris,;3. French Blood Service, La Conception, Marseille,;4. French Blood Service, Edouard Herriot Hospital, Lyon,;5. Department of Internal Medicine, Hautepierre Hospital, Strasbourg,;6. Department of Nephrology, Colmar General Hospital, Colmar, and;7. Department of Pediatrics, American Memorial Hospital, Reims, France |
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Abstract: | Twenty-seven patients (14 girls, 13 boys) affected by familial hypercholesterolemia who had begun low-density lipoprotein (LDL) apheresis treatment before the age of 15 were studied. The median age at diagnosis was 4 years and the blood LDL cholesterol level was 704 +/- 163 mg/dL. Screening was performed for homozygous or double heterozygous mutations of the LDL cholesterol receptor gene and mutations were found in 24 of the patients. The mean age at the beginning of treatment was 8.5 years and the mean length of follow up was 12.6 years. The two main procedures used were direct adsorption of lipoproteins and dextran sulfate cellulose adsorption. Nine patients experienced anaphylactic reactions due to bradykinin and six had to have their treatment changed. The LDL cholesterol level before the session was lowered by 45 +/- 11% of the value at diagnosis. The LDL cholesterol reduction in a session was 72 +/- 10%. Tendinous xanthomas disappeared or diminished dramatically in 62% of the children. In 22 patients no cardiovascular event occurred during LDL apheresis treatment. Three had angina pectoris; two others had surgical management of aortic stenosis, but no clinical manifestations. Seven children had normal cardiovascular pictures while on treatment. Eleven had abnormalities of the aortic root or coronary arteries, which in six cases had appeared before treatment; the other five children did not undergo prior cardiac evaluation. In five children the abnormalities appeared during treatment. Based on these data, LDL-apheresis can be recommended for the treatment of homozygous familial hypercholesterolemia, even in young children, with good efficiency on biological parameters, cutaneous lesions and cardiovascular events. |
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Keywords: | Childhood Dextran sulfate cellulose adsorption Direct adsorption of lipids Familial hypercholesterolemia Low‐density lipid apheresis |
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