Document Detail

Pregnancy outcome in familial homozygous hypercholesterolemic females treated with long-term plasma exchange.
MedLine Citation:
PMID:  9688236     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: The use of plasma-exchange therapy has increased the longevity of patients with homozygous familial hypercholesterolemia (HFH), and pregnancy in affected women is expected to become more common. We describe the clinical course, feasibility and risks of repeated pregnancies in patients with HFH treated by long-term plasma exchange. METHODS: We followed the clinical course of five pregnancies in two HFH patients, with attention to the effect of repeated plasma exchange on maternal and fetal status; specifically, lipid profile, hemodynamics, and uteroplacental circulation on Doppler flow study. RESULTS: Increasing the frequency of plasma-exchange therapy prevented the extreme serum cholesterol elevation that occurs in pregnant HFH patients and was associated with a significant improvement in uteroplacental circulation. In four pregnancies the clinical course was uneventful, ending in normal deliveries of full-term infants with heterozygous familial hypercholesterolemia. The third pregnancy of one of the patients had to be terminated owing to the development of hypotension and syncope during plasma exchange because of severe aortic stenosis. CONCLUSIONS: Repeated pregnancies in HFH patients treated by long-term plasma exchange are feasible but may be associated with untoward effects, especially hemodynamic compromise. The frequency of plasma exchange therapy should be increased to prevent marked hypercholesterolemia and its possible deleterious effect on maternal and fetal status. Cardiac evaluation with close hemodynamic monitoring are needed during pregnancy to detect complications of the cardiac valvular lesion and the coronary atherosclerosis that are associated with HFH.
Y Beigel; J Bar; M Cohen; M Hod
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Acta obstetricia et gynecologica Scandinavica     Volume:  77     ISSN:  0001-6349     ISO Abbreviation:  Acta Obstet Gynecol Scand     Publication Date:  1998 Jul 
Date Detail:
Created Date:  1998-08-18     Completed Date:  1998-08-18     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0370343     Medline TA:  Acta Obstet Gynecol Scand     Country:  DENMARK    
Other Details:
Languages:  eng     Pagination:  603-8     Citation Subset:  IM    
Department of Medicine A, Rabin Medical Center, Beilinson Campus, Petah Tiqva, Israel.
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MeSH Terms
Cholesterol / blood
Feasibility Studies
Hypercholesterolemia / blood,  genetics,  therapy*,  ultrasonography
Plasma Exchange*
Pregnancy Complications, Hematologic / blood,  therapy*,  ultrasonography
Pregnancy Outcome
Ultrasonography, Doppler
Umbilical Cord / blood supply,  ultrasonography
Reg. No./Substance:

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