DISEASES Nervous System Diseases [C10] Nervous System Diseases Central Nervous System Diseases [C10.228] Central Nervous System Diseases Brain Diseases [C10.228.140] Brain Diseases Brain Diseases, Metabolic [C10.228.140.163] Brain Diseases, Metabolic Brain Diseases, Metabolic, Inborn [C10.228.140.163.100] Brain Diseases, Metabolic, Inborn Adrenoleukodystrophy [C10.228.140.163.100.084] Adrenoleukodystrophy Cerebral Amyloid Angiopathy, Familial [C10.228.140.163.100.168] Cerebral Amyloid Angiopathy, Familial Galactosemias [C10.228.140.163.100.320] Galactosemias Hartnup Disease [C10.228.140.163.100.355] Hartnup Disease Hepatolenticular Degeneration [C10.228.140.163.100.360] Hepatolenticular Degeneration Hereditary Central Nervous System Demyelinating Diseases [C10.228.140.163.100.362] Hereditary Central Nervous System Demyelinating Diseases Homocystinuria [C10.228.140.163.100.365] Homocystinuria Hyperglycinemia, Nonketotic [C10.228.140.163.100.375] Hyperglycinemia, Nonketotic Hyperlysinemias [C10.228.140.163.100.380] Hyperlysinemias Leigh Disease [C10.228.140.163.100.412] Leigh Disease Lesch-Nyhan Syndrome [C10.228.140.163.100.425] Lesch-Nyhan Syndrome Lysosomal Storage Diseases, Nervous System [C10.228.140.163.100.435] Lysosomal Storage Diseases, Nervous System Maple Syrup Urine Disease [C10.228.140.163.100.520] Maple Syrup Urine Disease MELAS Syndrome [C10.228.140.163.100.535] MELAS Syndrome Menkes Kinky Hair Syndrome [C10.228.140.163.100.540] Menkes Kinky Hair Syndrome MERRF Syndrome [C10.228.140.163.100.545] MERRF Syndrome Mevalonate Kinase Deficiency [C10.228.140.163.100.593] Mevalonate Kinase Deficiency Oculocerebrorenal Syndrome [C10.228.140.163.100.640] Oculocerebrorenal Syndrome Phenylketonurias [C10.228.140.163.100.687] Phenylketonurias Pyruvate Carboxylase Deficiency Disease [C10.228.140.163.100.725] Pyruvate Carboxylase Deficiency Disease Pyruvate Dehydrogenase Complex Deficiency Disease [C10.228.140.163.100.750] Pyruvate Dehydrogenase Complex Deficiency Disease Refsum Disease [C10.228.140.163.100.813] Refsum Disease Refsum Disease, Infantile [C10.228.140.163.100.844] Refsum Disease, Infantile Tyrosinemias [C10.228.140.163.100.875] Tyrosinemias Urea Cycle Disorders, Inborn [C10.228.140.163.100.937] Urea Cycle Disorders, Inborn Zellweger Syndrome [C10.228.140.163.100.968] Zellweger Syndrome DISEASES Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16] Congenital, Hereditary, and Neonatal Diseases and Abnormalities Genetic Diseases, Inborn [C16.320] Genetic Diseases, Inborn Metabolism, Inborn Errors [C16.320.565] Metabolism, Inborn Errors Amino Acid Metabolism, Inborn Errors [C16.320.565.100] Amino Acid Metabolism, Inborn Errors Albinism [C16.320.565.100.102] Albinism Alkaptonuria [C16.320.565.100.187] Alkaptonuria Hyperglycinemia, Nonketotic [C16.320.565.100.477] Hyperglycinemia, Nonketotic Hyperhomocysteinemia [C16.320.565.100.480] Hyperhomocysteinemia Hyperlysinemias [C16.320.565.100.544] Hyperlysinemias Maple Syrup Urine Disease [C16.320.565.100.608] Maple Syrup Urine Disease Multiple Acyl Coenzyme A Dehydrogenase Deficiency [C16.320.565.100.614] Multiple Acyl Coenzyme A Dehydrogenase Deficiency Multiple Carboxylase Deficiency [C16.320.565.100.620] Multiple Carboxylase Deficiency Phenylketonurias [C16.320.565.100.766] Phenylketonurias Prolidase Deficiency [C16.320.565.100.794] Prolidase Deficiency Propionic Acidemia [C16.320.565.100.823] Propionic Acidemia Tyrosinemias [C16.320.565.100.880] Tyrosinemias Urea Cycle Disorders, Inborn [C16.320.565.100.940] Urea Cycle Disorders, Inborn DISEASES Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16] Congenital, Hereditary, and Neonatal Diseases and Abnormalities Genetic Diseases, Inborn [C16.320] Genetic Diseases, Inborn Metabolism, Inborn Errors [C16.320.565] Metabolism, Inborn Errors Brain Diseases, Metabolic, Inborn [C16.320.565.189] Brain Diseases, Metabolic, Inborn Adrenoleukodystrophy [C16.320.565.189.084] Adrenoleukodystrophy Cerebral Amyloid Angiopathy, Familial [C16.320.565.189.168] Cerebral Amyloid Angiopathy, Familial Galactosemias [C16.320.565.189.320] Galactosemias Hartnup Disease [C16.320.565.189.355] Hartnup Disease Hepatolenticular Degeneration [C16.320.565.189.360] Hepatolenticular Degeneration Hereditary Central Nervous System Demyelinating Diseases [C16.320.565.189.362] Hereditary Central Nervous System Demyelinating Diseases Homocystinuria [C16.320.565.189.365] Homocystinuria Hyperglycinemia, Nonketotic [C16.320.565.189.375] Hyperglycinemia, Nonketotic Hyperlysinemias [C16.320.565.189.380] Hyperlysinemias Leigh Disease [C16.320.565.189.412] Leigh Disease Lesch-Nyhan Syndrome [C16.320.565.189.425] Lesch-Nyhan Syndrome Lysosomal Storage Diseases, Nervous System [C16.320.565.189.435] Lysosomal Storage Diseases, Nervous System Maple Syrup Urine Disease [C16.320.565.189.520] Maple Syrup Urine Disease MELAS Syndrome [C16.320.565.189.535] MELAS Syndrome Menkes Kinky Hair Syndrome [C16.320.565.189.540] Menkes Kinky Hair Syndrome MERRF Syndrome [C16.320.565.189.545] MERRF Syndrome Mevalonate Kinase Deficiency [C16.320.565.189.593] Mevalonate Kinase Deficiency Oculocerebrorenal Syndrome [C16.320.565.189.640] Oculocerebrorenal Syndrome Phenylketonurias [C16.320.565.189.687] Phenylketonurias Pyruvate Carboxylase Deficiency Disease [C16.320.565.189.725] Pyruvate Carboxylase Deficiency Disease Pyruvate Dehydrogenase Complex Deficiency Disease [C16.320.565.189.750] Pyruvate Dehydrogenase Complex Deficiency Disease Refsum Disease [C16.320.565.189.813] Refsum Disease Refsum Disease, Infantile [C16.320.565.189.844] Refsum Disease, Infantile Tyrosinemias [C16.320.565.189.875] Tyrosinemias Urea Cycle Disorders, Inborn [C16.320.565.189.937] Urea Cycle Disorders, Inborn Zellweger Syndrome [C16.320.565.189.968] Zellweger Syndrome DISEASES Nutritional and Metabolic Diseases [C18] Nutritional and Metabolic Diseases Metabolic Diseases [C18.452] Metabolic Diseases Brain Diseases, Metabolic [C18.452.132] Brain Diseases, Metabolic Brain Diseases, Metabolic, Inborn [C18.452.132.100] Brain Diseases, Metabolic, Inborn Adrenoleukodystrophy [C18.452.132.100.084] Adrenoleukodystrophy Cerebral Amyloid Angiopathy, Familial [C18.452.132.100.168] Cerebral Amyloid Angiopathy, Familial Galactosemias [C18.452.132.100.320] Galactosemias Hartnup Disease [C18.452.132.100.355] Hartnup Disease Hepatolenticular Degeneration [C18.452.132.100.360] Hepatolenticular Degeneration Hereditary Central Nervous System Demyelinating Diseases [C18.452.132.100.362] Hereditary Central Nervous System Demyelinating Diseases Homocystinuria [C18.452.132.100.365] Homocystinuria Hyperglycinemia, Nonketotic [C18.452.132.100.375] Hyperglycinemia, Nonketotic Hyperlysinemias [C18.452.132.100.380] Hyperlysinemias Leigh Disease [C18.452.132.100.412] Leigh Disease Lesch-Nyhan Syndrome [C18.452.132.100.425] Lesch-Nyhan Syndrome Lysosomal Storage Diseases, Nervous System [C18.452.132.100.435] Lysosomal Storage Diseases, Nervous System Maple Syrup Urine Disease [C18.452.132.100.520] Maple Syrup Urine Disease MELAS Syndrome [C18.452.132.100.535] MELAS Syndrome Menkes Kinky Hair Syndrome [C18.452.132.100.540] Menkes Kinky Hair Syndrome MERRF Syndrome [C18.452.132.100.545] MERRF Syndrome Mevalonate Kinase Deficiency [C18.452.132.100.593] Mevalonate Kinase Deficiency Oculocerebrorenal Syndrome [C18.452.132.100.640] Oculocerebrorenal Syndrome Phenylketonurias [C18.452.132.100.687] Phenylketonurias Pyruvate Carboxylase Deficiency Disease [C18.452.132.100.725] Pyruvate Carboxylase Deficiency Disease Pyruvate Dehydrogenase Complex Deficiency Disease [C18.452.132.100.750] Pyruvate Dehydrogenase Complex Deficiency Disease Refsum Disease [C18.452.132.100.813] Refsum Disease Refsum Disease, Infantile [C18.452.132.100.844] Refsum Disease, Infantile Tyrosinemias [C18.452.132.100.875] Tyrosinemias Urea Cycle Disorders, Inborn [C18.452.132.100.937] Urea Cycle Disorders, Inborn Zellweger Syndrome [C18.452.132.100.968] Zellweger Syndrome DISEASES Nutritional and Metabolic Diseases [C18] Nutritional and Metabolic Diseases Metabolic Diseases [C18.452] Metabolic Diseases Metabolism, Inborn Errors [C18.452.648] Metabolism, Inborn Errors Amino Acid Metabolism, Inborn Errors [C18.452.648.100] Amino Acid Metabolism, Inborn Errors Albinism [C18.452.648.100.102] Albinism Alkaptonuria [C18.452.648.100.187] Alkaptonuria Hyperglycinemia, Nonketotic [C18.452.648.100.477] Hyperglycinemia, Nonketotic Hyperhomocysteinemia [C18.452.648.100.480] Hyperhomocysteinemia Hyperlysinemias [C18.452.648.100.544] Hyperlysinemias Maple Syrup Urine Disease [C18.452.648.100.608] Maple Syrup Urine Disease Multiple Acyl Coenzyme A Dehydrogenase Deficiency [C18.452.648.100.614] Multiple Acyl Coenzyme A Dehydrogenase Deficiency Multiple Carboxylase Deficiency [C18.452.648.100.620] Multiple Carboxylase Deficiency Phenylketonurias [C18.452.648.100.766] Phenylketonurias Propionic Acidemia [C18.452.648.100.823] Propionic Acidemia Tyrosinemias [C18.452.648.100.880] Tyrosinemias Urea Cycle Disorders, Inborn [C18.452.648.100.940] Urea Cycle Disorders, Inborn DISEASES Nutritional and Metabolic Diseases [C18] Nutritional and Metabolic Diseases Metabolic Diseases [C18.452] Metabolic Diseases Metabolism, Inborn Errors [C18.452.648] Metabolism, Inborn Errors Brain Diseases, Metabolic, Inborn [C18.452.648.189] Brain Diseases, Metabolic, Inborn Adrenoleukodystrophy [C18.452.648.189.084] Adrenoleukodystrophy Cerebral Amyloid Angiopathy, Familial [C18.452.648.189.168] Cerebral Amyloid Angiopathy, Familial Galactosemias [C18.452.648.189.320] Galactosemias Hartnup Disease [C18.452.648.189.355] Hartnup Disease Hepatolenticular Degeneration [C18.452.648.189.360] Hepatolenticular Degeneration Hereditary Central Nervous System Demyelinating Diseases [C18.452.648.189.362] Hereditary Central Nervous System Demyelinating Diseases Homocystinuria [C18.452.648.189.365] Homocystinuria Hyperglycinemia, Nonketotic [C18.452.648.189.375] Hyperglycinemia, Nonketotic Hyperlysinemias [C18.452.648.189.380] Hyperlysinemias Leigh Disease [C18.452.648.189.412] Leigh Disease Lesch-Nyhan Syndrome [C18.452.648.189.425] Lesch-Nyhan Syndrome Lysosomal Storage Diseases, Nervous System [C18.452.648.189.435] Lysosomal Storage Diseases, Nervous System Maple Syrup Urine Disease [C18.452.648.189.520] Maple Syrup Urine Disease MELAS Syndrome [C18.452.648.189.535] MELAS Syndrome Menkes Kinky Hair Syndrome [C18.452.648.189.540] Menkes Kinky Hair Syndrome MERRF Syndrome [C18.452.648.189.545] MERRF Syndrome Mevalonate Kinase Deficiency [C18.452.648.189.593] Mevalonate Kinase Deficiency Oculocerebrorenal Syndrome [C18.452.648.189.640] Oculocerebrorenal Syndrome Phenylketonurias [C18.452.648.189.687] Phenylketonurias Pyruvate Carboxylase Deficiency Disease [C18.452.648.189.725] Pyruvate Carboxylase Deficiency Disease Pyruvate Dehydrogenase Complex Deficiency Disease [C18.452.648.189.750] Pyruvate Dehydrogenase Complex Deficiency Disease Refsum Disease [C18.452.648.189.813] Refsum Disease Refsum Disease, Infantile [C18.452.648.189.844] Refsum Disease, Infantile Tyrosinemias [C18.452.648.189.875] Tyrosinemias Urea Cycle Disorders, Inborn [C18.452.648.189.937] Urea Cycle Disorders, Inborn Zellweger Syndrome [C18.452.648.189.968] Zellweger Syndrome

Hyperglycinemia, Nonketotic - Preferred

Concept UI	M0328332
Scope note	An autosomal recessive metabolic disorder caused by deficiencies in the mitochondrial GLYCINE cleavage system.
Preferred term	Hyperglycinemia, Nonketotic
Entry term(s)	Encephalopathies, Glycine Encephalopathy, Glycine Glycine Encephalopathies Glycine Encephalopathy Hyperglycinemia, Non-ketotic Hyperglycinemias, Non-ketotic Hyperglycinemias, Nonketotic Non ketotic Hyperglycinemia Non-ketotic Hyperglycinemia Non-ketotic Hyperglycinemias Nonketotic Hyperglycinemia Nonketotic Hyperglycinemias

Hyperglycinemia, Nonketotic, Type III - Narrower

Concept UI	M0335287
Preferred term	Hyperglycinemia, Nonketotic, Type III
Entry term(s)	Nonketotic Hyperglycinemia, Type III Type III Nonketotic Hyperglycinemia

Hyperglycinemia, Nonketotic, Type I - Narrower

Concept UI	M0335285
Preferred term	Hyperglycinemia, Nonketotic, Type I
Entry term(s)	Nonketotic Hyperglycinemia, Type I Type I Nonketotic Hyperglycinemia

Hyperglycinemia, Nonketotic, Type II - Narrower

Concept UI	M0335286
Preferred term	Hyperglycinemia, Nonketotic, Type II
Entry term(s)	Nonketotic Hyperglycinemia, Type II Type II Nonketotic Hyperglycinemia