Abstract
Severe hypertriglyceridemia has been observed in infants with β-thalassemia major, an association termed hypertriglyceridemia-thalassemia syndrome. The pathophysiological basis for this association has remained unclear. We describe 6-month-old American girl with red cell pyruvate kinase (PK) deficiency, failure to thrive, and marked hypertliglyceridemia (=1500 mg/dL). The hyperlipidemia resolved with hypertransfusion therapy. At age 18 months she underwent a splenectomy and has remained transfusion-independent with normal serum triglyceride levels. We suggest that severe hemolysis and chronic wasting are probably responsible for the hypertriglyceridemia seen in infants with thalassemia or PK deficiency.
Original language | English |
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Pages (from-to) | 303-305 |
Number of pages | 3 |
Journal | Indian pediatrics |
Volume | 44 |
Issue number | 4 |
State | Published - Apr 2007 |
Keywords
- Hemolytic anemia
- Hyperlipidemia
- Thalassemia