Maxwell P Opoku, Negmeldin Alsheikh, Daniel Miezah, Haseena Shah, Hala Elhoweris, Ashraf Moustafa
Competence of teachers towards managing trauma among children with disabilities in Ghana
African journal of disability
homozygous familial hypercholesterolaemia hofh rare genetic disease characterised extremely high plasma ldl cholesterol birth causing atherosclerotic cardiovascular disease young age lipoprotein apheresis combination lipidlowering drugs effectively reduce ldl cholesterol longterm health outcomes treatment unknown aimed investigate longterm cardiovascular outcomes associated lipoprotein apheresis initiated childhood adolescence cohort study data drawn hofh international clinical collaboration hicc international registry children homozygous hypercholesterolemia lipoprotein apheresis chain overall cohort included patients diagnosed hofh aged 018 years alive followup jan 1 2010 nov 8 2021 whose high plasma ldl cholesterol concentrations made eligible lipoprotein apheresis compare cardiovascular outcomes patients initiated lipoprotein apheresis childhood lipoprotein apheresis group patients received lipidlowering drugs pharmacotherapyonly group matched sex untreated plasma ldl cholesterol concentrations primary outcome composite cardiovascular death myocardial infarction ischaemic stroke percutaneous coronary intervention coronary artery bypass grafting aortic valve replacement peripheral artery disease carotid endarterectomy angina pectoris supraaortic aortic stenosis collectively referred atherosclerotic cardiovascular disease survival analyses performed matched cohort cox regression analyses used compare diseasefree survival cohorts calculate hazard ratio hr 95 ci adjusted sex age diagnosis untreated plasma ldl cholesterol concentration number lipidlowering therapies lipoprotein apheresis overall cohort included 404 patients median age diagnosis 60 years iqr 3095 median untreated plasma ldl cholesterol 178 mmoll 147208 matched cohorts included 250 patients 125 patients per group median untreated ldl cholesterol 172 mmoll 148197 mean reduction plasma ldl cholesterol concentrations baseline final followup greater lipoprotein apheresis group 55 95 ci 60 51 vs 31 36 25 p00001 patients lipoprotein apheresis group longer atherosclerotic cardiovascular diseasefree survival adjusted hr 052 95 ci 032085 longer cardiovascular deathfree survival 00301 0002104295 cardiovascular death common pharmacotherapyonly group lipoprotein apheresis group ten 8 vs one 1 p0010 whereas median age coronary artery bypass grafting lower lipoprotein apheresis group pharmacotherapyonly group 150 years iqr 120240 vs 305 years 190338 p0037 among patients hofh lipoprotein apheresis initiated childhood adolescence associated reduced longterm risk atherosclerotic cardiovascular disease death clear benefits early initiation highfrequency treatment reducing plasma cholesterol found consensus recommendations needed guide widespread timely use lipoprotein apheresis children hofh research required optimise treatment ensure benefits early aggressive treatment delivery balanced effects quality life amsterdam university medical centers location academic medical center perelman school medicine university pennsylvania european atherosclerosis society us national heart lung blood institute national institutes health