الفهرس | Only 14 pages are availabe for public view |
Abstract Inherited metabolic diseases, while individually rare conditions, contribute significantly to pediatric morbidity and mortality. Anesthetists may encounter patients with inherited metabolic diseases presenting for both emergency and elective surgery. The management of these patients is often challenging due to the multisystemic manifestations of many IMDs. Catastrophic metabolic decompensation may occur in the perioperative period and a multidisciplinary approach is essential to ensure safe management of these patients. Anesthesia of hepatic porphyric patients can trigger potentially serious complications even though documented accidents are rare. Although most anesthetic agents have been used in cases of latent Porphyria (except highly contraindicated barbiturates), caution is necessary. If an acute attack is suspected, only agents known to be safe in this context should be used. This rarely occurring disorder must not be underestimated. Any suspicion must lead to diagnosis assessment and possibly to research into the family history. Inherited defects in glycogen metabolism prevent the mobilization of glucose from glycogen and typically cause the accumulation of glycogen within tissues such as the liver or muscles. The management of the GSD involves maintaining an adequate blood glucose level and provision of alternate energy sources. Management of anesthesia in patients with disorders of amino acid metabolism is directed toward maintenance of intravascular fluid volume and acid-base homeostasis. Use of anesthetics that could evoke seizures may be questionable in view of the likely presence of seizure disorders in these patients. |