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Anesth Analg 2000;91:837-855
© 2000 International Anesthesia Research Society

PEDIATRIC ANESTHESIA

Specific Genetic Diseases at Risk for Sedation/Anesthesia Complications

Merlin G. Butler, MD, PhD, FACMG, Blaine G. Hayes, BS, Melanie M. Hathaway, BA, BS, and Michael L. Begleiter, MS, CGC

Section of Medical Genetics and Molecular Medicine, Children’s Mercy Hospitals and Clinics, Kansas City, Missouri

Address correspondence and reprint requests to Merlin G. Butler, MD, PhD, Section of Medical Genetics and Molecular Medicine, Children’s Mercy Hospitals and Clinics, 2401 Gillham Rd., Kansas City, MO 64108. Address e-mail to mgbutler{at}cmh.edu

We reviewed of a number of genetic diseases known or at risk for sedation or anesthesia complications. Some of these conditions are relatively common (e.g., Down’s syndrome) whereas others are rare or present with multiple congenital anomalies that have an impact on health care delivery. We listed complications, recommended presedation evaluations, and included checklist items to assist the health care provider administering sedation and anesthesia. A better recognition and awareness of risk factors associated with specific genetic diseases should lessen the likelihood of complications during these procedures.

Implications: This article provides a brief description of potential problematic genetic disorders and associated complications that may manifest during sedation or anesthesia. Recommendations for presedation evaluation and checklist items are given that may impact on the delivery of care for these patients.




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Lippincott, Williams & Wilkins Anesthesia & Analgesia® is published for the International Anesthesia Research Society® by Lippincott Williams & Wilkins with the assistance of Stanford University Libraries' HighWire Press®. Copyright 2006 by the International Anesthesia Research Society. Online ISSN: 1526-7598   Print ISSN: 0003-2999 HighWire Press
Copyright © 2000 by the International Anesthesia Research Society.