We experienced the general anesthesia for tracheotomy in 2-year-old male child diagnosed as Nonketotic Hyperglycinemia (NKH). To date the anesthesia in NKH have never been reported.
NKH is conjectured that it is an autosomal recessive disorder of glycine metabolism characterized by intractable seizures, lethargy, severe psychomotor retardation, and early death. Biochemically, there is marked elevation of glycine levels in plasma, urine, and CSF.
The anesthesia was inducted with 50% N
2O and 0.5% isoflurane, and maintained with 50% N
2O and 0.5-1.0% isoflurane. Before the anesthesia, we anticipated some complications which would develop during and after operation (increase of airway secretion, respiratory depression, exacerbation of seizure etc). But there was no trouble during and after operation.
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