Universität zu Köln

Taylan, Christina ORCID: 0000-0001-9585-054X and Weber, Lutz T. (2023). An update on lipid apheresis for familial hypercholesterolemia. Pediatr. Nephrol., 38 (2). S. 371 - 383. NEW YORK: SPRINGER. ISSN 1432-198X

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Abstract

Familial hypercholesterolemia (FH) is an inherited metabolic defect leading to increased total cholesterol and low-density cholesterol (LDL) from birth onwards. Homozygous FH, presenting with clear clinical features, has a prevalence of similar to 1 per million. Prevalence of heterozygous FH is 1/500 European population. Atherosclerotic burden depends on the degree and duration of high LDL exposure. In severe cases, early detection is critical, and aggressive lipid-lowering therapies should begin in early childhood to reduce coronary heart disease risk. Pediatric therapeutic concepts correspond to adults and are orientated at LDL plasma concentration. Mean LDL plasma target value during treatment is < 135 mg/dL. Medication in childhood consists of ezetemibe, statins, resins, and PCSK-9 inhibitors, with consideration for age restrictions. Only a minority achieve the treatment target with drug therapy alone. Therapeutic apheresis for the treatment of hypercholesterolemia selectively removes lipoproteins from blood (lipid apheresis (LA)). LA has a long tradition in adult medicine and is also safely used in children by a variety of methods, if customized to special pediatric needs. LA reduces cholesterol levels independently of residual LDL-receptor function and not only achieves reduction or disappearance of xanthomas but also inhibits progression of or mitigates aortic valve stenosis and supravalvular aortic stenosis as well as coronary artery and other atherosclerotic lesions. Cardiovascular prognosis of patients with otherwise untreatable FH depends largely on timely use of LA. Taking into account LA as a lifelong treatment, starting early in childhood, it is important to accommodate therapy modalities, such as treatment frequency and point of time, into the life of the individual.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Taylan, Christina UNSPECIFIED orcid.org/0000-0001-9585-054X UNSPECIFIED Weber, Lutz T. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-662774
DOI:	10.1007/s00467-022-05541-1
Journal or Publication Title:	Pediatr. Nephrol.
Volume:	38
Number:	2
Page Range:	S. 371 - 383
Date:	2023
Publisher:	SPRINGER
Place of Publication:	NEW YORK
ISSN:	1432-198X
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language CARDIOVASCULAR-DISEASE; LIPOPROTEIN APHERESIS; PEDIATRIC-PATIENTS; GENETIC-ANALYSIS; PLASMA-EXCHANGE; CHILDREN; PREVALENCE; PREVENTION; MANAGEMENT; CLINICIAN Multiple languages Pediatrics; Urology & Nephrology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/66277