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nonketotic hyperglycinemia


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Dear all.....

We have seen three babies with nonketotic hyperglycinemia over a working period of 27 years here in our NICU ,GAZA, Palestine...all were dead ..in about month of age...if any has such cases i will be grateful for feedback..thanks

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  • 2 weeks later...

Dear Thabet Masri, I have not come across cases with non-ketonic hyperglycinemia and cannot be of any help with this question.

But, I took a look in my old Nelson Textbook of pediatrics, that says that this metabolic disorder "appears to be" autosomal recessively inherited. No effective treatment exists, according to Nelson, but the textbook tells that attempts have been done to counteract the effect of glycine on neuronal cells, such as treatment with strychnine or diazepam and that "beneficial effects" have been seen in mild forms of this condition.

Does anyone else have experience from this condition?

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