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ammonium hydroxide and BCKD Deficiency

ammonium hydroxide has been researched along with BCKD Deficiency in 18 studies

azane : Saturated acyclic nitrogen hydrides having the general formula NnHn+2.

Research Excerpts

ExcerptRelevanceReference
"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)."7.68Evaluation 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."3.75The 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)."3.68Evaluation 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."2.37Neonatal seizures. ( Bergman, I; Crumrine, P; Painter, MJ, 1986)

Research

Studies (18)

TimeframeStudies, this research(%)All Research%
pre-199011 (61.11)18.7374
1990's5 (27.78)18.2507
2000's1 (5.56)29.6817
2010's0 (0.00)24.3611
2020's1 (5.56)2.80

Authors

AuthorsStudies
Eminoğlu, FT1
Öncül, Ü1
Kahveci, F1
Okulu, E1
Kraja, E1
Köse, E1
Kendirli, T1
Kalkan Ucar, S1
Coker, M1
Habif, S1
Saz, EU1
Karapinar, B1
Ucar, H1
Kitis, O1
Duran, M1
Efron, ML1
Bremer, HJ1
Falk, MC1
Knight, JF1
Roy, LP1
Wilcken, B1
Schell, DN1
O'Connell, AJ1
Gillis, J1
Jan, D1
Poggi, F1
Laurent, J1
Rabier, D1
Jouvet, P1
Lacaille, F1
Beringer, A1
Hubert, P1
Revillon, Y1
Saudubray, JM1
Nyhan, WL1
Ring, E1
Zobel, G1
Parsons, HG1
Carter, RJ1
Unrath, M1
Snyder, FF1
Rutledge, SL1
Havens, PL1
Haymond, MW1
McLean, RH1
Kan, JS1
Brusilow, SW1
Gortner, L1
Leupold, D1
Pohlandt, F1
Bartmann, P1
Painter, MJ1
Bergman, I1
Crumrine, P1
Kolodny, EH1
Yatziv, S1
Ampola, MG1
Frézal, J1
Menne, F1
D'iachkova, AIa1
Lebedev, BV1
Martin, JJ1
Schlote, W1

Reviews

4 reviews available for ammonium hydroxide and BCKD Deficiency

ArticleYear
Neonatal seizures.
    Pediatric clinics of North America, 1986, Volume: 33, Issue:1

    Topics: Adrenoleukodystrophy; Amino Acid Metabolism, Inborn Errors; Ammonia; Anesthetics, Local; Anticonvuls

1986
Phenylketonuria and other disorders of amino acid metabolism.
    Pediatric clinics of North America, 1973, Volume: 20, Issue:2

    Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Child; Child, Preschool; Counseling; Cystathionine; C

1973
[Feeble mindedness caused by genetic disorders of amino acid metabolism].
    Hippokrates, 1968, May-31, Volume: 39, Issue:10

    Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Citrulline; Glycine; Hartnup Disease; Histi

1968
[Mental retardation and hereditary enzymopathy (review)].
    Zhurnal nevropatologii i psikhiatrii imeni S.S. Korsakova (Moscow, Russia : 1952), 1971, Volume: 71, Issue:10

    Topics: Ammonia; Carboxy-Lyases; Child; Child, Preschool; Eye Diseases; Galactosemias; Growth Disorders; Har

1971

Other Studies

14 other studies available for ammonium hydroxide and BCKD Deficiency

ArticleYear
Characteristics of continuous venovenous hemodiafiltration in the acute treatment of inherited metabolic disorders.
    Pediatric nephrology (Berlin, Germany), 2022, Volume: 37, Issue:6

    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.
    Metabolic brain disease, 2009, Volume: 24, Issue:3

    Topics: Acidosis; Ammonia; Brain; Child, Preschool; Consanguinity; Female; Glutamates; Humans; Infant, Newbo

2009
Diet therapy for inborn errors of amino acid metabolism.
    Journal of the American Dietetic Association, 1967, Volume: 51, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Child, Preschool; Coenzymes; Cystinuria; Diet Therapy

1967
[Metabolic emergencies in the newborn infant].
    Monatsschrift Kinderheilkunde : Organ der Deutschen Gesellschaft fur Kinderheilkunde, 1983, Volume: 131, Issue:6

    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.
    Pediatric nephrology (Berlin, Germany), 1994, Volume: 8, Issue:3

    Topics: Acute Disease; Amino Acid Metabolism, Inborn Errors; Ammonia; Blood Transfusion; Carbamoyl-Phosphate

1994
Liver transplantation: new indications in metabolic disorders?
    Transplantation proceedings, 1994, Volume: 26, Issue:1

    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.
    Current problems in pediatrics, 1977

    Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Chromatography, Gas; Diagnosis, Differen

1977
[Hemofiltration in acute neonatal metabolic crisis].
    Wiener klinische Wochenschrift, 1992, Volume: 104, Issue:21

    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.
    Journal of inherited metabolic disease, 1990, Volume: 13, Issue:2

    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.
    The Journal of pediatrics, 1990, Volume: 116, Issue:1

    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.
    Acta paediatrica Scandinavica, 1989, Volume: 78, Issue:5

    Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Creatinine; Female; Humans; Infant, Newbo

1989
Laboratory approaches for inherited neurometabolic diseases.
    Developmental medicine and child neurology, 1985, Volume: 27, Issue:2

    Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Galactosemias; Homocystinuria; Humans; Lactates; Lact

1985
Medical physiopathology, enzymology and diagnosis.
    Monographs in human genetics, 1972, Volume: 6

    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.
    Journal of the neurological sciences, 1972, Volume: 15, Issue:1

    Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Brain; Central Nervous System; Chi

1972