Multicore myopathy in a patient with anhidrotic ectodermal dysplasia
| Autor: | Colin P. Gordon, Sharon Litz |
|---|---|
| Rok vydání: | 1992 |
| Předmět: |
Male
Ectodermal dysplasia medicine.medical_specialty Respiratory Distress Syndrome Adolescent business.industry Cardiomyopathy Malignant hyperthermia General Medicine Scoliosis medicine.disease Surgery Anesthesiology and Pain Medicine Pulmonary aspiration Muscular Diseases Ectodermal Dysplasia Medicine Humans medicine.symptom business Complication Myopathy Anesthesia Inhalation Respiratory Insufficiency Central core disease |
| Zdroj: | Canadian journal of anaesthesia = Journal canadien d'anesthesie. 39(9) |
| ISSN: | 0832-610X |
| Popis: | We report a patient with multicore myopathy, a rare myopathy not previously reported in the anaesthetic literature. It is characterised by a myopathy of proximal muscles which tends to follow a benign course but may be associated with a severe form of cardiomyopathy. The myopathy is related to central core disease so these patients should be considered to have a potential for developing malignant hyperthermia. Complicating this case was an associated anhidrotic type of ectodermal dysplasia resulting in the absence of sweating, febrile episodes, recurrent pulmonary infections, conical and missing teeth, scaly skin and fine, sparse hair. The patient had a scoliosis repair which was uneventful but died three weeks later following a major pulmonary aspiration while on the ward. The cause of the aspiration is thought to have been unsuspected laryngeal incompetence associated with ectodermal dysplasia, the myopathy involving his bulbar muscles and analgesic medication. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full text from SpringerLink