Coagulation Profile Dynamics in Pediatric Patients with Cushing Syndrome: A Prospective, Observational Comparative Study

Leah Birdwell, Maya Lodish*, Amit Tirosh, Prashant Chittiboina, Meg Keil, Charlampos Lyssikatos, Elena Belyavskaya, Richard A. Feelders, Constantine A. Stratakis

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

Objective To evaluate the association between Cushing syndrome and hypercoagulability in children. Study design A prospective, observational study was performed of 54 patients with Cushing syndrome, 15.1 ± 3.9 years, treated at the National Institutes of Health Clinical Center. Coagulation profiles were taken before and 6-12 months after surgery and compared with18 normocortisolemic children, 13.7 ± 3.6 years. Results At baseline, patients with Cushing syndrome had greater levels of the procoagulant factor VIII (FVIII) vs controls (145 IU/dL ± 84 vs 99 ± 47, P = .04); 6-12 months after surgery, FVIII levels decreased to 111 ± 47, P = .05. Patients with Cushing syndrome had greater levels of the antifibrinolytic α2-antiplasmin, 96 ± 17% vs 82 ± 26%, P = .015. After surgery, antifibrinolytic α2-antiplasmin levels decreased to 82 ± 24%, P < .001. Anticoagulants were greater in patients with Cushing syndrome vs controls at baseline, including protein C (138 ± 41% vs 84 ± 25%, P < .001), protein S (94 ± 19% vs 74 ± 19%, P = .001), and antithrombin III (96 ± 18% vs 77 ± 13%, P < .0001). The 24-hour urinary free cortisol levels correlated positively with FVIII levels, r = 0.43, P = .004. Conclusion Children with Cushing syndrome had elevated procoagulants, antifibrinolytics, and anticoagulants at baseline compared with controls; normalization of coagulation measures was seen after surgical cure. Despite the increase in anticoagulants, hypercortisolemia is associated with a hypercoagulable state in children, as is the case in adults. This finding has potential implications for prevention of venous thromboembolism in children with Cushing syndrome. Trial registration ClinicalTrials.gov:

Original languageEnglish
Pages (from-to)227-231
Number of pages5
JournalJournal of Pediatrics
Volume177
DOIs
StatePublished - 2016
Externally publishedYes

Funding

FundersFunder number
National Institute of Neurological Disorders and StrokeZIANS003150
Eunice Kennedy Shriver National Institute of Child Health and Human Development

    Keywords

    • blood clot
    • hypercoagulable state
    • thromboembolic event

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