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Query: UMLS:C0024591 (
malignant hyperthermia
)
2,353
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Smith-Lemli-Opitz syndrome
(
SLOS
) is an autosomal recessive syndrome, characterized by severe growth failure and congenital anomalies (for example dysgenesis, mental retardation, renal and cardiac defects, and various malformation).
SLOS
results from error of a cholesterol enzyme and generalized cholesterol deficiency. This report describes our experience of a patient with
SLOS
and thrombocytopenia who underwent anesthesia twice for surgical procedures in a year. The patient received drip platelet transfusion for thrombocytopenia before operations. Anesthesia was induced with inhalation of oxygen, nitrous oxide and sevoflurane, and maintained with oxygen, propofol, fentanyl and low concentrations of sevoflurane. Airway was maintained with laryngeal mask airway. Complications were not seen in this case. One of the problems in anesthetic management of
SLOS
is difficult intubation because of the typical dysmorphic facial features such as micrognathia, cleft palate and abnormal tongue. We thought that laryngeal mask airway was useful and safe for
SLOS
patients. Two cases of
malignant hyperthermia
were reported in anesthetic management of
SLOS
by using halothane or suxamethonium. In this case, the anesthetic maintenance was mostly with propofol and fentanyl.
Malignant hyperthermia
did not occur but sevoflurane was used at low concentrations.
SLOS
presents various problems with anesthetic management and we have to administer general anesthesia carefully.
...
PMID:[Anesthetic management of a patient with Smith-Lemli-Opitz syndrome complicated with thrombocytopenia]. 1466 77
