Hemostatic abnormalities in Noonan syndrome

Andrea Artoni, Angelo Selicorni, Serena M. Passamonti, Anna Lecchi, Paolo Bucciarelli, Marta Cerutti, Paola Cianci, Francesca Gianniello, Ida Martinelli

Research output: Contribution to journalArticlepeer-review


BACKGROUND: A bleeding diathesis is a common feature of Noonan syndrome, and various coagulation abnormalities have been reported. Platelet function has never been carefully investigated. METHODS: The degree of bleeding diathesis in a cohort of patients with Noonan syndrome was evaluated by a validated bleeding score and investigated with coagulation and platelet function tests. If ratios of prothrombin time and/or activated partial thromboplastin time were prolonged, the activity of clotting factors was measured. Individuals with no history of bleeding formed the control group. RESULTS: The study population included 39 patients and 28 controls. Bleeding score was ≥2 (ie, suggestive of a moderate bleeding diathesis) in 15 patients (38.5%) and ≥4 (ie, suggestive of a severe bleeding diathesis) in 7 (17.9%). Abnormal coagulation and/or platelet function tests were found in 14 patients with bleeding score ≥2 (93.3%) but also in 21 (87.5%) of those with bleeding score 90% of them had platelet function and/or coagulation abnormalities. Results of these tests should be taken into account in the management of bleeding or invasive procedures in these patients.

Original languageEnglish
Issue number5
Publication statusPublished - 2014


  • Bleeding score
  • Coagulation
  • Noonan syndrome
  • Platelets

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Arts and Humanities (miscellaneous)
  • Medicine(all)


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