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Cardiovascular diseases in congenital prekallikrein deficiency: comparison with other chance-associated morbidities

Girolami, Antonioa; Ferrari, Silviaa; Cosi, Elisabettaa; Girolami, Brunob

Blood Coagulation & Fibrinolysis: July 2018 - Volume 29 - Issue 5 - p 423–428
doi: 10.1097/MBC.0000000000000735

To compare the prevalence of cardiovascular diseases with other chance-associated morbidities in patients with congenital prekallikrein deficiency. Patients with prekallikrein deficiency were gathered from two time unlimited PubMed searches and from personal files. Inclusion criteria were prekallikrein level less than 15% of normal; correction of aPTT on long incubation times; prolonged aPTT corrected by normal plasma or serum; normal prothrombin time and normal FXII and FXI. Acquired forms were excluded. Out of 106 patients, we have found that 45 patients had at least one chance-associated defect or morbidity at the time of the diagnosis of the clotting defect. Twenty-nine of these 45 patients had cardiovascular disorders. Other comorbidities were found in a much smaller proportion. Congenital prekallikrein deficiency is frequently associated with cardiovascular conditions, namely hypertension, coronary disease, ischemic stroke, venous thrombosis. The significance of these findings is critically discussed as association between two diseases does not necessarily indicate the existence of a causative relation between the two. However, the findings presented here clearly indicate the possibility that such a relation might indeed exist.

aDepartment of Medicine, University of Padua Medical School

bDivision of Medicine, Padua City Hospital, Padua, Italy

Correspondence to Antonio Girolami, MD, Department of Medicine, Via Ospedale, 105, Padua 35128, Italy Tel: +39 498213026; fax: +39 49657391; e-mail:

Received 23 October, 2017

Revised 6 March, 2018

Accepted 26 March, 2018

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