RÉSUMÉ
AIM: In heterozygous Familial Hypercholesterolemia (FH) woman atherosclerotic cardiovascular disease occurs 20-years earlier respect woman without FH while homozygous FH women may suffer from atherosclerotic cardiovascular disease even in childhood. Lipoprotein apheresis, a therapeutic "last chance saloon", is a well-tolerated procedure that markedly lowers LDL-cholesterol and Lp(a) levels in patients who do not achieve acceptable levels with maximal lifestyle and drug therapy. METHODS AND RESULTS: The experience of LA treatment in 3 female homozygous FH patients was described. Moreover, an explore analysis on pre and post-LA hormonal levels was performed in 8 HeFH women showing a significant improvement in the atherogenic lipid profile (total cholesterol -56%, LDL cholesterol -71%, triglycerides -72%, Apo B lipoprotein -69%, Lp(a) -59%;) and a reduction of FSH and LH values (FSH - 28%, LH -31%). CONCLUSIONS: Women with FH experience specific barriers to care, including underrepresentation in research, significant underestimation of risk, and discontinuation of therapy during pregnancy. Therefore, in this study, we investigated the possible effects of LA treatment on plasma FSH and LH levels.
Sujet(s)
Aphérèse , Observance par le patient , Qualité de vie , Humains , Mâle , Femelle , Adulte d'âge moyen , Lipoprotéines/sang , Adulte , Sujet âgéSujet(s)
Aphérèse , Maladies cardiovasculaires , Lipoprotéine (a) , Inhibiteurs de PCSK9 , Humains , Lipoprotéine (a)/sang , Aphérèse/effets indésirables , Maladies cardiovasculaires/prévention et contrôle , Maladies cardiovasculaires/épidémiologie , Maladies cardiovasculaires/sang , Incidence , Mâle , Femelle , Adulte d'âge moyen , Marqueurs biologiques/sang , Résultat thérapeutique , Inhibiteurs de la sérine protéinase/usage thérapeutique , Inhibiteurs de la sérine protéinase/effets indésirables , Sujet âgé , Hyperlipoprotéinémies/sang , Hyperlipoprotéinémies/thérapie , Hyperlipoprotéinémies/complications , Hyperlipoprotéinémies/épidémiologie , Hyperlipoprotéinémies/traitement médicamenteux , Hyperlipoprotéinémies/diagnostic , Proprotéine convertase 9/métabolismeRÉSUMÉ
PURPOSE: In Familial Hypercholesterolemia (FH), female atherosclerotic cardiovascular disease occurs 20 years earlier than in women without FH. The aim of this study is to describe the differences in lipoprotein apheresis (LA), a last therapeutic option, in terms of efficacy, safety and clinical outcomes between the two sexes. MATERIALS AND METHODS: Sex related differences were analysed in 31 subjects in on LA treatment with FH and not achieving LDL-cholesterol and/or Lp(a) target values on maximum lipid-lowering therapies. Moreover, sex related differences in time to major cardiovascular event (MACE) was investigated in 68 subjects, with at least one year of follow-up. RESULTS: Among the 31 patients currently undergoing LA treatment who did not achieve LDL-cholesterol and/or Lp(a) target values, no differences in comorbidity were recorded despite a worse pre-LA treatment lipid profile (LDL-C 77 ± 60 mg/dl in males vs. 128 ± 105 mg/dl in females; p 0.025) and a longer mean inter-apheresis interval (17 ± 4 days in males vs. 19 ± 5 days in females; p 0.012) reported in females compared to males. Additionally, in comparison with men, it was found that the time between the first cardiovascular event and the beginning of LA, as well as the age at the beginning of LA, were significantly higher in females than in males (p 0.027 and 0.007, respectively). CONCLUSIONS: Sex differences in FH subjects not only affect the diagnosis and treatment but also influence varied responses to the treatment itself.
Sujet(s)
Aphérèse , Hyperlipoprotéinémie de type II , Lipoprotéine (a) , Humains , Mâle , Femelle , Aphérèse/méthodes , Adulte d'âge moyen , Hyperlipoprotéinémie de type II/thérapie , Hyperlipoprotéinémie de type II/sang , Résultat thérapeutique , Adulte , Lipoprotéine (a)/sang , Facteurs sexuels , Cholestérol LDL/sang , Sujet âgé , Maladies cardiovasculaires/épidémiologie , Caractères sexuels , Lipoprotéines/sangRÉSUMÉ
The lower, the better is the recommended approach in the management of high LDL cholesterol. Unfortunately, this does not always achieve as in the case of a 69-year-old woman referred to our Institute for her lipid profile (LDL cholesterol 412mg/dl), bilateral xanthelasma and cutaneous xanthomas. With a maximized and personalized lipid-lowering therapies (rosuvastatin, ezetimibe, PCSK9i and lipoprotein apheresis), after only six months, the patient showed an impressive regression in her cutaneous xanthomas. (AU)
«Cuanto más bajo, mejor» es el enfoque recomendado en el tratamiento del colesterol LDL alto. Lamentablemente esto no siempre se logra como en el caso de una mujer de 69 años remitida a nuestro Instituto por su perfil lipídico (colesterol LDL 412 mg/dL), xantelasma bilateral y xantomas cutáneos. Con terapias hipolipemiantes maximizadas y personalizadas (rosuvastatina, ezetimiba, iPCSK9 y aféresis de lipoproteínas), después de solo seis meses, la paciente mostró una regresión impresionante en sus xantomas cutáneos. (AU)