Variant Creutzfeldt–Jakob Disease in a Patient with Heterozygosity at PRNP Codon 129

Abstract

The authors reply: Paton raises the thorny is-sue of attribution of primacy. We were aware of multiple observational studies indicating favor-able outcomes with the use of antihypertensive agents in patients with malignant hypertension. 1 In reexamining the literature, we did indeed find an important trial involving patients with severe hy-pertension (diastolic blood pressure, >130 mm Hg) who were randomly assigned to antihypertensive therapy (with reserpine, chlorothiazide, or hydro-chlorothiazide–guanethidine) or matching pla-cebos. 2 We will resist calling this the first trial and instead use the term " early. " Shamy and Fedyk appropriately remind us that there are special situations in responsible clinical research in which informed consent may be waived. Their point was recently illustrated in the Informed Fresh versus Old Red Cell Manage-ment (INFORM) trial. 3 In this trial, each site's ethics committee waived the need for patient con-sent " because all the patients received treatment that was consistent with the current standard of care. " 3 We agree with their additional points in-dicating that obtaining consent is only one of the many responsibilities of investigators in conduct-ing quality clinical investigation. 1. Smirk FH. The prognosis of untreated and of treated hyper-tension and advantages of early treatment. Am Heart J 1972; 83: 825-40. 2. Wolff FW, Lindeman RD. Effects of treatment in hyperten-sion: results of a controlled study. J Chronic Dis 1966; 19: 227-40. 3. Heddle NM, Cook RJ, Arnold DM, et al. Effect of short-term vs. long-term blood storage on mortality after transfusion. N Engl J Med 2016; 375: 1937-45.