Appendix D: Metabolism of Branched-Chain Amino Acids
Appendix E: Glycogen Metabolism
Appendix F: The Porphyrias
Thoroughly updated for its Second Edition, this reference is the only single-source guide to the anesthetic management of children and adults with genetic, metabolic, and dysmorphic syndromes. In a format designed for quick, easy look-up, this edition provides an encyclopedic review of well over 500 distinct syndromes, with up-to-date information, complete current bibliographies, and over 140 clinical photographs.
Syndromes and synonyms are listed alphabetically and synonyms are cross-referenced. Each syndrome is presented in an easy-to-follow format: name, synonym(s), common and uncommon manifestations in each organ system, and anesthetic considerations. If a syndrome has no anesthetic implications, this is also indicated.
Reviewer: Anne M. Lynn, MD (University of Washington)
Description: The authors of this book have assembles an encyclopedic listing of syndromes, an outline of their findings, and highlights of their anesthetic implications.
Purpose: They have collated the information needed to answer the question, "What do I need to know about someone with Syndrome X to anesthetize them safely?"
Audience: The book is designed for all anesthesiologists, both those in general practice who care for children and pediatric anesthesiologists. Some trainees in anesthesia may find the anesthetic implications not detailed enough, because the authors "assume the anesthesiologist is competent" and, knowing the syndrome features, "will arrive at a safe anesthetic plan."
Features: The syndromes are listed alphabetically, and their features in seven areas (HEENT, chest, cardiovascular, neuromuscular, orthopedic, GI/GU, other), the anesthetic considerations, and pertinent bibliography from 1975-present are also covered. The black-and-white photographs are good; more would be better. There is no appendix to cross-reference syndromes with similar features (such as difficult airway anatomy). The introduction is very helpful in orienting the reader to the setup of the book.
Assessment: The authors thoroughly compile syndromes, most with implications for anesthetic care and some that do not impact anesthesia (a good addition). I believe every anesthesia practice would be well served to have a copy in a library accessible to daily care. Information also found in Katz & Steward¢¥s Anesthesia and Uncommon Pediatric Diseases (W. B. Saunders 1993), and Smith¢¥s Recognizable Patterns of Human Malformation (W. B. Saunders 1997) is expanded and reformatted here.