RESUMO
Mucopolysaccharidosis [MPS] is a group of genetic disorders that presents challenges during anesthetic care and in particular difficulty with airway management. Patients should be managed by experienced anesthesiologists at centers that are familiar with these types of conditions. Rarely encountered disease states have been identified as important topics in the continuing education of clinical anesthesiologists. This review will define MPS, describe the pathophysiology of MPS, describe how patients with this rare lysosomal storage disorders have dysfunction of tissues, cite the incidence of MPS, list the clinical manifestations and specific problems associated with the administration of anesthesia to patients with MPS, present treatment options for patients with MPS, define appropriate preoperative evaluation and perioperative management of these patients, including, to anticipate potential postoperative airway problems
RESUMO
A diagnosis of rheumatoid arthritis carries with it a lifelong progressive disease; however twenty percent of patients enjoy periods of partial to total remission. After remission, the disease will frequently plague previously unaffected joints. Life expectancy is reduced by an average of three to seven years. Complications of RA include vasculitis and amyloidosis affecting any vessel, including the aorta. Additionally, complications of therapy such as chronic NSAID use leading to GI bleeding and infections associated with long term steroid use, can add to the difficulties of the disease. The recent discovery and use of anticytokines and DMARDs has lead to greatly reduced symptomology associated with RA and greater patient comfort. Side effects of drugs should be well understood including the risk of bleeding from NSAIDs. Management and surgical intervention of problems that arise from this disease vary dramatically. The anesthesiologist must be aware of airway pathologies, pain management techniques, and available pharmacology parameters