Is phenylketonuria causes bronchospasm during general anesthesia?

Abstract

Phephenylketonuria (PKU) is caused by an accumulation of phenylalanine in blood and tissue due to phenylalaninehydroxylase enzyme deficiency, which means that phenylalanine, an essential amino acid, cannot be converted to tyrosine. The accumulation of phenylalanine in nonbrain tissues and the decreased production of tyrosine can cause various clinical symptoms. Catecholamines are synthesized via a series of reactions initiated by tyrosine 3,4-dihydroxyphenylalanine hydroxylation. And in the absence of tyrosine, the synthesis of epinephrine can be reduced. There appear to be no studies in the present literature on non-neurological symptoms associated with decreased catecholamine synthesis in patients with PKU. In the present case, we described a severe bronchospasm in a child with PKU during general anesthesia. Further research is needed to confirm whether the bronchospasm that occurred in this case was due to a lack of catecholamine induced by PKU. A link between a deficiency of catecholamines, which are required for neuronal and hormonal control, and pulmonary findings in PKU can be established with clinical and experimental studies. © 2016 OMU.