English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Global: Zendy Plus annual

Presents the access of premium content as premium feature

Premium Content

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the summarisation as premium feature

Summarisation

Insights

Presents the pdf analysis as premium feature

PDF Analysis

Presents the zaia usage as premium feature

ZAIA

Global: Zendy Tools annual

Global: Zendy Free Plan

Global: Zendy Tools monthly

Global: Zendy Plus monthly

We report dramatic improvement in clinical and laboratory parameters after intensive plasma exchange therapy in a 15-yr-old girl with acquired generalized lipoatrophy and refractory hypertriglyceridemia. One hundred and twenty-five procedures were performed over 720 d. Two or three plasma volumes were exchanged per procedure, using peripheral venous access and albumin as replacement solution. Regression of painful cutaneous xanthomata and reduction in massive hepatomegaly were noted within the first two procedures. Triglyceride levels started at 109 mmol/liter (9670 mg/dl) and decreased acutely by 60-85%/procedure. Lipid removal averaged 83 g/procedure and was highly correlated with preexchange lipid levels. Lipid levels rebounded to baseline values within 7 d after exchange and appeared to rebound more rapidly after larger exchanges. Maximum benefit was achieved with weekly 1.5- to 2.0-volume exchanges. No significant decrease in apolipoprotein CII levels was detected after plasma exchange regardless of the volume of exchange; however, other plasma factors regulating triglyceride synthesis or clearance may have been removed during the procedures. Plasma exchange was well tolerated, without clinical, immunological, or hormonal deterioration. These data indicate that intensive plasma exchange therapy over a protracted time may produce sustained benefit in patients with severe, symptomatic hypertriglyceridemia refractory to standard medical therapy.

Intensive, Long-Term Plasma Exchange Therapy for Severe Hypertriglyceridemia in Acquired Generalized Lipoatrophy