Neonatal Care Academy

aEEG in Hypophosphatasia

Findings of amplitude-integrated electroencephalogram recordings and serum vitamin B6 metabolites in perinatal lethal hypophosphatasia during enzyme replacement therapy.

An Interesting article from Japan on the utilisation of aEEG in Hypophosphatasia. This case showed that aEEG monitoring was useful for early treatment intervention and safe pyridoxine withdrawal. Together with enzyme replacement therapy by asfotase alfa, pyridoxine hydrochloride was administered, then the seizures were rapidly controlled – monitoring with aEEG was a very helpful adjunct to confirm control of seizure activity and safely withdraw pyridoxine.

Moreover, it also showed that perinatal HPP patients can present pyridoxine-responsive seizure even 24 h after birth. Enzyme replacement therapy changed the vitamin B6 metabolism and made it possible to discontinue pyridoxine in one month.

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August 7, 2019
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