nonketotic hyperglycinemia

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[nonkētot′ik]

a usually fatal autosomal-recessive aminoacidopathy with accumulation of glycine in body fluids, particularly the blood, urine, and cerebrospinal fluid. It has neonatal onset and is characterized by lethargy, metabolic acidosis with ketosis, absence of cerebral development, seizures, myoclonic jerks, and frequently coma and respiratory failure. It is caused by a defect in one or more of the enzymes involved in the cleavage of glycine. Also called methylmalonicacidemia, propionicacidemia.

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No references found			Glycine has not been shown to be so consistently elevated at 24 hours of age as to make this useful in screening for nonketotic hyperglycinemia, or NKH. Potential and pitfalls of NBS, and the reference lab's role by Bartholomew, Dennis William / Medical Laboratory Observer com NONKETOTIC HYPERGLYCINEMIA See: Acidemia, Organic NONNE-MILROY-MEIGE SYNDROME See: Lymphatic Ma/formations; NOONAN SYNDROME See: Cardio-Facio-Cutaneous Syndrome; Growth Disorders; Heart Disorders; Malignant Hyperthermia; Williams Syndrome (TNSSG) Noonan Syndrome Support Group, Inc. National Resources for Specific Disabilities by The Exceptional Parent			nonhemolytic jaundice nonhuman primate nonhypertension syndrome noni noni juice nonidentical nonigravida nonimmune fetal hydrops nonimmune hemolytic anemia nonimmune serum nonimmunity nonimpact printer noninfective valvular mass Noninflammatory noninflammatory arthritis noninflammatory diarrhea noninflammatory joint disease noninnovative drug noninvasive noninvasive ventilation nonionic nonionizing radiation nonipara nonisolated proteinuria nonkeratinizing squamous cell tumor nonketotic hyperglycinemia nonlamellar bone nonleaded nonlinear nonlipid histiocytosis nonliving disease agents nonlocal nonlocal-naturalistic mechanisms nonmaleficence nonmalficence nonmedullated fiber nonmelanoma skin cancer nonmendelian disorder nonmetals nonmetastatic nonmilk nonmodifiable risk factor nonmotile sperm nonmyelinated nerve fiber nonmyelinic spongiform encephalomyelopathies Nonne-Apelt test Nonne-Milroy-Meige syndrome nonneurolytic block nonneuronal nonneurotic awareness nonneutral			nonis Nonischemic Cardiomyopathy Nonischemic Zone Flow nonisolated proteinuria nonissue Noniterative Integral Equation Method Nonius Nonius, Petrus Nonjob-Routed nonjoinder nonjoinders nonjudgmental nonjudicial foreclosure Nonjudicial punishment Nonjurant nonjuring nonjuring Nonjuring schism Nonjuring schism nonjuror nonjuror nonjuror Nonjurorism Nonjurors Nonjurors nonkeratinized mucosa nonkeratinized mucosa nonkeratinized mucosa nonkeratinizing squamous cell tumor Nonketotic Hyperglycemic Hyperosmolar Coma nonketotic hyperglycinemia nonkosher NONL nonlamellar bone Nonlanguage Qualification Test nonleaded nonleaded Nonleafy Normal Stature Nonleafy Reduced Stature nonlegal nonlegal Nonlegal Investment Nonlegal Investments nonlethal nonlethal Nonlethal weapons nonleukemic myelosis nonlexical nonlexically nonliability nonliable Nonlimitation Nonline of Sight nonlinear nonlinear nonlinear Nonlinear acoustics Nonlinear algebra Nonlinear algebra nonlinear amplifier Nonlinear Amplifying Loop Mirror

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