Document Detail

Low density lipoprotein apheresis in pediatric patients with homozygous familial hypercholesterolemia.
MedLine Citation:
PMID:  19379151     Owner:  NLM     Status:  MEDLINE    
The aim of the present study is to clarify the low density lipoprotein apheresis procedure for pediatric patients with homozygous familial hypercholesterolemia (FH) in terms of efficacy, adverse effects and difficulties. The follow-up was carried out using an open, prospective uncontrolled clinical design. Data were collected from 10 patients (with an average age of 8.4 +/- 4.7 years) with FH treated with double filtration plasmapheresis. The total time span of follow-up covered five years (30.2 +/- 17.8 months [range 9-60 months]) and more than 600 sessions (62.1 +/- 35.5 sessions per patient [range 18-120 sessions]) were evaluated. The mean low density lipoprotein cholesterol (LDL-C) pre-treatment value was 375.5 +/- 127.5 mg/dL, and the post-treatment value was 147.5 +/- 73.9 mg/dL. This corresponded to a 62.8 +/- 10.3% (43-73%) acute reduction of LDL-C, while the mean high density lipoprotein cholesterol losses amounted to 41%. The chronic reduction in LDL-C ranged from 18 to 52%, with a mean level of 36.4 +/- 11.7%. The most frequently occurring technical problems were related to blood lines: puncture difficulties (4.5%), insufficient blood flow (3.5%), and obturation of the blood lines (2.4%). The main clinical adverse effects were hypotension (0.2%), chills/feeling cold (0.1%), and nausea and vomiting (0.2%). We observed that the low pediatric patient tolerance is the main problem in compliance with treatment. In conclusion, LDL apheresis, started under the age of eight years, combined with lipid-lowering drugs, provides a safe and effective lowering of the mean LDL-C levels in pediatric homozygous FH; and there are more problems with compliance for pediatric LDL apheresis than in the adult population.
Mahmut Coker; Sema Kalkan Ucar; Damla Goksen Simsek; Sukran Darcan; Mustafa Bak; Sule Can
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy     Volume:  13     ISSN:  1744-9987     ISO Abbreviation:  Ther Apher Dial     Publication Date:  2009 Apr 
Date Detail:
Created Date:  2009-04-21     Completed Date:  2009-07-16     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101181252     Medline TA:  Ther Apher Dial     Country:  Australia    
Other Details:
Languages:  eng     Pagination:  121-8     Citation Subset:  IM    
Department of Pediatric Endocrinology and Metabolism, Medical Faculty, Ege University, Izmir, Turkey.
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MeSH Terms
Child, Preschool
Chills / etiology
Cholesterol, HDL / blood
Cholesterol, LDL / blood*
Follow-Up Studies
Hyperlipoproteinemia Type II / therapy*
Hypotension / etiology
Nausea / etiology
Patient Compliance
Plasmapheresis / adverse effects,  methods*
Prospective Studies
Treatment Outcome
Vomiting / etiology
Reg. No./Substance:
0/Cholesterol, HDL; 0/Cholesterol, LDL

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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