ammonium hydroxide has been researched along with Maple Syrup Urine Disease in 18 studies
azane : Saturated acyclic nitrogen hydrides having the general formula NnHn+2.
Maple Syrup Urine Disease: An autosomal recessive inherited disorder with multiple forms of phenotypic expression, caused by a defect in the oxidative decarboxylation of branched-chain amino acids (AMINO ACIDS, BRANCHED-CHAIN). These metabolites accumulate in body fluids and render a maple syrup odor. The disease is divided into classic, intermediate, intermittent, and thiamine responsive subtypes. The classic form presents in the first week of life with ketoacidosis, hypoglycemia, emesis, neonatal seizures, and hypertonia. The intermediate and intermittent forms present in childhood or later with acute episodes of ataxia and vomiting. (From Adams et al., Principles of Neurology, 6th ed, p936)
| Excerpt | Relevance | Reference |
|---|---|---|
| "The biochemical and growth responses to dietary branched-chain amino acid (BCAA) intake were studied in two children; one with a disorder of branched-chain amino acid metabolism, maple syrup urine disease (MSUD) (McKusick 24860), and another with methylmalonic aciduria (MMA) (McKusick 25100)." | 7.68 | Evaluation of branched-chain amino acid intake in children with maple syrup urine disease and methylmalonic aciduria. ( Carter, RJ; Parsons, HG; Snyder, FF; Unrath, M, 1990) |
| "Maple syrup urine disease (MSUD) is a defect in the catabolism of the branched-chain amino acids; leucine, isoleucine, and valine." | 3.75 | The first use of N-carbamylglutamate in a patient with decompensated maple syrup urine disease. ( Coker, M; Duran, M; Habif, S; Kalkan Ucar, S; Karapinar, B; Kitis, O; Saz, EU; Ucar, H, 2009) |
| "The biochemical and growth responses to dietary branched-chain amino acid (BCAA) intake were studied in two children; one with a disorder of branched-chain amino acid metabolism, maple syrup urine disease (MSUD) (McKusick 24860), and another with methylmalonic aciduria (MMA) (McKusick 25100)." | 3.68 | Evaluation of branched-chain amino acid intake in children with maple syrup urine disease and methylmalonic aciduria. ( Carter, RJ; Parsons, HG; Snyder, FF; Unrath, M, 1990) |
| "Neonatal seizures, as distinguished from nonconvulsive abnormal movements, are a significant problem in neonatal intensive care units." | 2.37 | Neonatal seizures. ( Bergman, I; Crumrine, P; Painter, MJ, 1986) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 11 (61.11) | 18.7374 |
| 1990's | 5 (27.78) | 18.2507 |
| 2000's | 1 (5.56) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 1 (5.56) | 2.80 |
| Authors | Studies |
|---|---|
| Eminoğlu, FT | 1 |
| Öncül, Ü | 1 |
| Kahveci, F | 1 |
| Okulu, E | 1 |
| Kraja, E | 1 |
| Köse, E | 1 |
| Kendirli, T | 1 |
| Kalkan Ucar, S | 1 |
| Coker, M | 1 |
| Habif, S | 1 |
| Saz, EU | 1 |
| Karapinar, B | 1 |
| Ucar, H | 1 |
| Kitis, O | 1 |
| Duran, M | 1 |
| Efron, ML | 1 |
| Bremer, HJ | 1 |
| Falk, MC | 1 |
| Knight, JF | 1 |
| Roy, LP | 1 |
| Wilcken, B | 1 |
| Schell, DN | 1 |
| O'Connell, AJ | 1 |
| Gillis, J | 1 |
| Jan, D | 1 |
| Poggi, F | 1 |
| Laurent, J | 1 |
| Rabier, D | 1 |
| Jouvet, P | 1 |
| Lacaille, F | 1 |
| Beringer, A | 1 |
| Hubert, P | 1 |
| Revillon, Y | 1 |
| Saudubray, JM | 1 |
| Nyhan, WL | 1 |
| Ring, E | 1 |
| Zobel, G | 1 |
| Parsons, HG | 1 |
| Carter, RJ | 1 |
| Unrath, M | 1 |
| Snyder, FF | 1 |
| Rutledge, SL | 1 |
| Havens, PL | 1 |
| Haymond, MW | 1 |
| McLean, RH | 1 |
| Kan, JS | 1 |
| Brusilow, SW | 1 |
| Gortner, L | 1 |
| Leupold, D | 1 |
| Pohlandt, F | 1 |
| Bartmann, P | 1 |
| Painter, MJ | 1 |
| Bergman, I | 1 |
| Crumrine, P | 1 |
| Kolodny, EH | 1 |
| Yatziv, S | 1 |
| Ampola, MG | 1 |
| Frézal, J | 1 |
| Menne, F | 1 |
| D'iachkova, AIa | 1 |
| Lebedev, BV | 1 |
| Martin, JJ | 1 |
| Schlote, W | 1 |
4 reviews available for ammonium hydroxide and Maple Syrup Urine Disease
| Article | Year |
|---|---|
Neonatal seizures.
Topics: Adrenoleukodystrophy; Amino Acid Metabolism, Inborn Errors; Ammonia; Anesthetics, Local; Anticonvuls | 1986 |
Phenylketonuria and other disorders of amino acid metabolism.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Child; Child, Preschool; Counseling; Cystathionine; C | 1973 |
[Feeble mindedness caused by genetic disorders of amino acid metabolism].
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Citrulline; Glycine; Hartnup Disease; Histi | 1968 |
[Mental retardation and hereditary enzymopathy (review)].
Topics: Ammonia; Carboxy-Lyases; Child; Child, Preschool; Eye Diseases; Galactosemias; Growth Disorders; Har | 1971 |
14 other studies available for ammonium hydroxide and Maple Syrup Urine Disease
| Article | Year |
|---|---|
Characteristics of continuous venovenous hemodiafiltration in the acute treatment of inherited metabolic disorders.
Topics: Aged; Ammonia; Child; Continuous Renal Replacement Therapy; Hemodiafiltration; Humans; Hypotension; | 2022 |
The first use of N-carbamylglutamate in a patient with decompensated maple syrup urine disease.
Topics: Acidosis; Ammonia; Brain; Child, Preschool; Consanguinity; Female; Glutamates; Humans; Infant, Newbo | 2009 |
Diet therapy for inborn errors of amino acid metabolism.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Child, Preschool; Coenzymes; Cystinuria; Diet Therapy | 1967 |
[Metabolic emergencies in the newborn infant].
Topics: Ammonia; Critical Care; Emergencies; Humans; Infant, Newborn; Infant, Newborn, Diseases; Maple Syrup | 1983 |
Continuous venovenous haemofiltration in the acute treatment of inborn errors of metabolism.
Topics: Acute Disease; Amino Acid Metabolism, Inborn Errors; Ammonia; Blood Transfusion; Carbamoyl-Phosphate | 1994 |
Liver transplantation: new indications in metabolic disorders?
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Child; Female; Humans; Liver Transplantation; Male; M | 1994 |
An approach to the diagnosis of overwhelming metabolic disease in early infancy.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Chromatography, Gas; Diagnosis, Differen | 1977 |
[Hemofiltration in acute neonatal metabolic crisis].
Topics: Amino Acids, Branched-Chain; Ammonia; Emergencies; Female; Hemofiltration; Humans; Infant, Newborn; | 1992 |
Evaluation of branched-chain amino acid intake in children with maple syrup urine disease and methylmalonic aciduria.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids, Branched-Chain; Ammonia; Body Weight; Child, Pres | 1990 |
Neonatal hemodialysis: effective therapy for the encephalopathy of inborn errors of metabolism.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids, Branched-Chain; Ammonia; Argininosuccinate Syntha | 1990 |
Peritoneal dialysis in the treatment of metabolic crises caused by inherited disorders of organic and amino acid metabolism.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Creatinine; Female; Humans; Infant, Newbo | 1989 |
Laboratory approaches for inherited neurometabolic diseases.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Galactosemias; Homocystinuria; Humans; Lactates; Lact | 1985 |
Medical physiopathology, enzymology and diagnosis.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Homocystinuria; Humans; Intellectual Disability; Mapl | 1972 |
Central nervous system lesions in disorders of amino-acid metabolism. A neuropathological study.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Brain; Central Nervous System; Chi | 1972 |