A 36-year-old man presents with recurrent acute pancreatitis, eruptive xanthomas, and lipemia retinalis. Vital signs show BP 138/88, HR 92, RR 18, temp 37°C, SpO₂ 98% on room air. Serum triglycerides are 8,500 mg/dL with normal LDL cholesterol. Genetic testing excludes lipoprotein lipase and apoC-II mutations. Hepatic lipase activity is severely reduced. He denies alcohol use. Which lipoprotein abnormality characterizes this condition?
- A)Chylomicron remnant accumulation with normal VLDL
- B)Profound HDL deficiency with apoA-I accumulation
- C)Isolated LDL elevation with normal triglycerides
- D)Severe chylomicronemia with cholesterol-rich particles
- E)Accumulation of VLDL remnants (IDL) due to impaired triglyceride hydrolysisGABARITO
Explicação
Hepatic lipase catalyzes triglyceride hydrolysis in VLDL and IDL, and phospholipid hydrolysis in HDL. Hepatic lipase deficiency causes accumulation of triglyceride-rich VLDL remnants (IDL), extremely elevated triglycerides (Type III hyperlipoproteinemia), and ... Ver explicação completa e trilha adaptativa →