Nelson Textbook of Pediatrics 22nd Ed — Sections 105.12 & 105.7. Urea cycle defects (OTC, CPS1, ASS, ASL, ARG1): hyperammonemia, respiratory alkalosis, treatment with scavengers and hemodialysis. Glycine disorders: Nonketotic hyperglycinemia (NKH), CSF/plasma glycine ratio >0.08, seizures, hypotonia.
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