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The Evaluation of Hematologic Screening and Perioperative Management in Patients with Noonan Syndrome: A Retrospective Chart Review.

Authors
  • Briggs, Benjamin1
  • Savla, Dipal2
  • Ramchandar, Nanda3
  • Dimmock, David4
  • Le, Dzung5
  • Thornburg, Courtney D6
  • 1 Department of Pediatrics, University of California San Diego, La Jolla, CA; Division of Hematology and Oncology, University of California San Diego, La Jolla, CA; Rady Children's Institute of Genomic Medicine, San Diego, CA. Electronic address: [email protected]
  • 2 Department of Pediatrics, University of California San Diego, La Jolla, CA.
  • 3 Department of Pediatrics, University of California San Diego, La Jolla, CA; Rady Children's Institute of Genomic Medicine, San Diego, CA; Division of Infectious Disease, University of California San Diego, La Jolla, CA.
  • 4 Rady Children's Institute of Genomic Medicine, San Diego, CA.
  • 5 Department of Pathology, University of California San Diego, La Jolla, CA.
  • 6 Department of Pediatrics, University of California San Diego, La Jolla, CA; Division of Hematology and Oncology, University of California San Diego, La Jolla, CA; Hemophilia and Thrombosis Treatment Center, Rady Children's Hospital San Diego, San Diego, CA.
Type
Published Article
Journal
The Journal of pediatrics
Publication Date
May 01, 2020
Volume
220
Identifiers
DOI: 10.1016/j.jpeds.2020.01.048
PMID: 32111381
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

To assess the potential impact of using screening recommendations for bleeding disorders in patients with Noonan syndrome on perioperative bleeding complications. We performed a retrospective, single-site cohort study; patients were identified by query of the electronic medical record. All patients with a clinical diagnosis of Noonan syndrome over a 10-year period were included. Data on surgeries, hematologic evaluation, bleeding symptoms, and bleeding complications were extracted. Surgeries were graded as major or minor. We identified 101 patients with Noonan syndrome, 70 of whom required surgery for a total of 164 procedures. Nine patients (9/70; 12.8%) had bleeding complications, occurring in those without comprehensive testing or perioperative intervention and undergoing major or dental surgery. Based on these findings, the risk of a bleeding complication for patients with Noonan syndrome who did not have comprehensive testing or perioperative intervention was 6.2% (95% CI 2.3%-10.1%), indicating the number needed to treat or screen would be 16 to prevent 1 bleeding complication (95% CI 9.9-43.9). The majority of patients had either no or incomplete evaluation (59 of 101; 58.4%). With proper evaluation and management, the bleeding risk in patients with Noonan syndrome can be minimized. Efforts are needed to address the knowledge and implementation gap in this evaluation. Copyright © 2020 Elsevier Inc. All rights reserved.

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