Case report: The efficacy and safety of lomitapide in a homozygous familial hypercholesterolemic child.

We report for the first time the efficiency and safety of a 49-month compassionate use of the microsomal transfer protein inhibitor lomitapide in a child with homozygous familial hypercholesterolemia. On average, 20 mg of lomitapide caused a 37% reduction in low-density lipoprotein cholesterol levels on top of ezetimibe and atorvastatin. The drug was well tolerated with no changes in liver enzymes and occurrence of steatosis on hepatic ultrasound. The patient presented adequate growth and sexual maturation. Nonetheless, there was progression in either subclinical atherosclerotic carotid or aortic valve diseases. Further studies are necessary to test the impact and safety of lomitapide in children with homozygous familial hypercholesterolemia.

Clearance of a 3H-labeled chylomicron-like emulsion following the acute phase of myocardial infarction.

BACKGROUND: Plasma lipids may be altered during acute myocardial infarction and may not reflect patient baseline lipid profile. The metabolism of chylomicrons, the lipoproteins that carry the dietary lipids in the bloodstream has not yet been studied in acute myocardial infarction patients. METHODS: In this study, a lipidic emulsion that mimics the intravascular behavior of chylomicrons labeled with cholesteryl oleate ((3)H-CO) was injected intravenously in 17 normolipidemic patients on the seventh and on the 45th day post-non complicated acute myocardial infarction after a 12-h fast. The plasma decay curve of the emulsion label was determined from blood samples collected during 60 min. Data were also compared with a group of 10 patients with chronic coronary artery disease. RESULTS: In the acute myocardial infarction group, the plasma fractional catabolic rates of the emulsion (3)H-CO, expressed as median and confidence intervals, did not change from the seventh to the 45th day after the acute event [0.0773 (0.061, 0.1025) min(-1) vs. 0.0672 (0.00507, 0.1009) min(-1) P=0.61] and was similar to that determined in chronic coronary artery disease patients. High-density lipoprotein cholesterol and apolipoprotein AI were lower on the seventh day when compared to the 45th day post acute myocardial infarction (P=0.01 and P=0.004, respectively). No changes were found in LDL and total cholesterol as well as in plasma triglycerides in myocardial infarction group. CONCLUSIONS: No changes were found in chylomicron metabolism is in the acute phase of myocardial infarction.

Valor da ressonância nuclear magnética na detecção da perviabilidade da artéria coronária após trombólise / Role of the Magnetic Resonance Imaging to Detect Artery Patency After Thrombolysis

PURPOSE--To determine the value of magnetic resonance imaging (MRI) in the noninvasive detection of infarct related coronary artery patency after thrombolysis. METHODS--We studied 26 patients with acute myocardial infarction submitted to thrombolysis underwent MRI studies before and after 0.1mmol/kg gadolinium-DTPA injection within the first 48 h of MI. Signal intensity was assessed by circumferential profile analysis techniques. RESULTS--The average ratio of signal intensity of infarcted tissue over normal myocardium (I/N) was significantly higher in patients with patent arteries (1.3 +/- 0.13 vs 1.12 +/- 0.07, p < 0.02). Compared to coronariography MRI, sensitivity of 81 and specificity of 100 for the diagnosis of coronary patency. CONCLUSION--Gadolinium infusion increased infarcted and normal myocardium differentiation. The study of gadolinium kinetics at MRI is a promising technique for noninvasive diagnosis of coronary patency.