Page last updated: 2024-10-16

ammonium hydroxide and Hyperinsulinism

ammonium hydroxide has been researched along with Hyperinsulinism in 25 studies

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

Hyperinsulinism: A syndrome with excessively high INSULIN levels in the BLOOD. It may cause HYPOGLYCEMIA. Etiology of hyperinsulinism varies, including hypersecretion of a beta cell tumor (INSULINOMA); autoantibodies against insulin (INSULIN ANTIBODIES); defective insulin receptor (INSULIN RESISTANCE); or overuse of exogenous insulin or HYPOGLYCEMIC AGENTS.

Research Excerpts

ExcerptRelevanceReference
" Biochemical evaluation extended to the first-degree relatives is consistent with a familial form of hypoglycemia due to a leucine-sensitive hyperinsulinism."7.69Biochemical evaluation of a patient with a familial form of leucine-sensitive hypoglycemia and concomitant hyperammonemia. ( Donati, MA; Filippi, L; Novembre, E; Zammarchi, E, 1996)
" We hypothesized that this syndrome of hyperinsulinism and hyperammonemia was caused by excessive activity of glutamate dehydrogenase, which oxidizes glutamate to alpha-ketoglutarate and which is a potential regulator of insulin secretion in pancreatic beta cells and of ureagenesis in the liver."3.70Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene. ( Burlina, AB; Greenberg, CR; Hopwood, NJ; Hsu, BY; Lieu, YK; Perlman, K; Poncz, M; Rich, BH; Stanley, CA; Zammarchi, E, 1998)
" Biochemical evaluation extended to the first-degree relatives is consistent with a familial form of hypoglycemia due to a leucine-sensitive hyperinsulinism."3.69Biochemical evaluation of a patient with a familial form of leucine-sensitive hypoglycemia and concomitant hyperammonemia. ( Donati, MA; Filippi, L; Novembre, E; Zammarchi, E, 1996)
"All patients had convulsions or loss of consciousness resulting from hypoglycemia at less than 1 year of age."1.31Novel missense mutations in the glutamate dehydrogenase gene in the congenital hyperinsulinism-hyperammonemia syndrome. ( Hayashi, Y; Kato, H; Miki, Y; Ohura, T; Taki, T; Yanagisawa, M, 2000)
"The hyperinsulinism was successfully treated with diazoxide."1.31Functional hyperactivity of hepatic glutamate dehydrogenase as a cause of the hyperinsulinism/hyperammonemia syndrome: effect of treatment. ( de Klerk, JB; Duran, M; Huijmans, JG; Rovers, MJ; Scholte, HR, 2000)
"A boy suffered from hypoglycemic coma with relative hyperinsulinemia on day 1 after birth, and received subtotal pancreatectomy."1.31A Japanese case of congenital hyperinsulinism with hyperammonemia due to a mutation in glutamate dehydrogenase (GLUD1) gene. ( Akanuma, Y; Kadowaki, H; Kadowaki, T; Kimura, S; Koda, N; Ogawa, Y; Yasuda, K, 2001)

Research

Studies (25)

TimeframeStudies, this research(%)All Research%
pre-19901 (4.00)18.7374
1990's12 (48.00)18.2507
2000's8 (32.00)29.6817
2010's3 (12.00)24.3611
2020's1 (4.00)2.80

Authors

AuthorsStudies
Brandt, A1
Agarwal, N1
Giri, D1
Yung, Z1
Didi, M1
Senniappan, S1
Walker, V1
Brannelly, NT1
Hamilton-Shield, JP1
Killard, AJ1
Balasubramaniam, S1
Kapoor, R1
Yeow, JH1
Lim, PG1
Flanagan, S1
Ellard, S1
Hussain, K1
Kofoed, KF1
Hove, JD1
Freiberg, J1
Høst, U1
Holm, S1
Kelbaek, H1
Stanley, CA5
Ihara, K1
Miyako, K1
Ishimura, M1
Kuromaru, R1
Wang, HY1
Yasuda, K2
Hara, T1
Ang, BC1
Halliday, D1
Powell-Tuck, J1
Zammarchi, E2
Filippi, L1
Novembre, E1
Donati, MA1
Weinzimer, SA1
Berry, GT1
Yudkoff, M1
Tuchman, M1
Thornton, PS2
Wolfsdorf, JI1
Lieu, YK1
Hsu, BY2
Burlina, AB1
Greenberg, CR1
Hopwood, NJ1
Perlman, K1
Rich, BH1
Poncz, M2
Sann, L1
al-Shamsan, L1
al-Essa, M1
al-Mutair, A1
al-Ashwal, A1
Sakati, N1
Ozand, PT1
Kamoun, P1
Rabier, D1
Saudubray, JM1
Parini, R1
Colombo, F1
Lombardi, AM1
Menni, F1
Beccaria, L1
Kitaura, J1
Miki, Y2
Kato, H2
Sakakihara, Y1
Yanagisawa, M2
Peterson, PE1
Smith, TJ1
Yorifuji, T1
Muroi, J1
Uematsu, A1
Hiramatsu, H1
Momoi, T1
Taki, T1
Ohura, T1
Hayashi, Y1
Fang, J1
Kutyna, K1
Ming, JE1
Glaser, B1
Huijmans, JG1
Duran, M1
de Klerk, JB1
Rovers, MJ1
Scholte, HR1
Koda, N1
Kadowaki, H1
Ogawa, Y1
Kimura, S1
Kadowaki, T1
Akanuma, Y1
Kelly, A1
Ng, D1
Ferry, RJ1
Grimberg, A1
Koo-McCoy, S1
Tews, JK1
Carter, SH1
Stone, WE1

Clinical Trials (1)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Efficacy of Vitamin E in Hyperinsulinism/Hyperammonemia Syndrome[NCT04984798]Phase 20 participants (Actual)Interventional2022-11-30Withdrawn (stopped due to No study activity took place. The IND was withdrawn with the FDA by the Sponsor Investigator because of insurmountable hurdles in moving proposed research forward)
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Reviews

3 reviews available for ammonium hydroxide and Hyperinsulinism

ArticleYear
Ammonia metabolism and hyperammonemic disorders.
    Advances in clinical chemistry, 2014, Volume: 67

    Topics: Ammonia; Animals; Arginine; Biological Transport; Brain; Cell Membrane; Humans; Hyperammonemia; Hype

2014
The Measurement of Ammonia in Human Breath and its Potential in Clinical Diagnostics.
    Critical reviews in analytical chemistry, 2016, Volume: 46, Issue:6

    Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Breath Tests; Humans; Hyperinsulinism; Hypoglycemia;

2016
Hyperinsulinism/hyperammonemia syndrome: insights into the regulatory role of glutamate dehydrogenase in ammonia metabolism.
    Molecular genetics and metabolism, 2004, Volume: 81 Suppl 1

    Topics: Adolescent; Ammonia; Animals; Female; Glutamate Dehydrogenase; Humans; Hyperammonemia; Hyperinsulini

2004

Trials

2 trials available for ammonium hydroxide and Hyperinsulinism

ArticleYear
Variability of insulin-stimulated myocardial glucose uptake in healthy elderly subjects.
    European journal of nuclear medicine and molecular imaging, 2002, Volume: 29, Issue:12

    Topics: Ammonia; Blood Glucose; Dipyridamole; Fluorodeoxyglucose F18; Glucose; Glucose Clamp Technique; Hear

2002
Whole-body protein turnover in response to hyperinsulinemia in humans postabsorptively with [15N]glycine as tracer.
    The American journal of clinical nutrition, 1995, Volume: 61, Issue:5

    Topics: Adult; Ammonia; Blood Glucose; Carbon Isotopes; Cross-Over Studies; Female; Glucose Clamp Technique;

1995

Other Studies

20 other studies available for ammonium hydroxide and Hyperinsulinism

ArticleYear
Hyperinsulinism hyperammonaemia (HI/HA) syndrome due to GLUD1 mutation: phenotypic variations ranging from late presentation to spontaneous resolution.
    Journal of pediatric endocrinology & metabolism : JPEM, 2020, May-26, Volume: 33, Issue:5

    Topics: Ammonia; Blood Glucose; Child; Child, Preschool; Diazoxide; Female; Glutamate Dehydrogenase; Humans;

2020
Biochemical evaluation of an infant with hypoglycemia resulting from a novel de novo mutation of the GLUD1 gene and hyperinsulinism-hyperammonemia syndrome.
    Journal of pediatric endocrinology & metabolism : JPEM, 2011, Volume: 24, Issue:7-8

    Topics: Ammonia; Blood Glucose; Delayed Diagnosis; Female; Glutamate Dehydrogenase; Humans; Hyperinsulinism;

2011
A case of hyperinsulinism/hyperammonaemia syndrome with reduced carbamoyl-phosphate synthetase-1 activity in liver: a pitfall in enzymatic diagnosis for hyperammonaemia.
    Journal of inherited metabolic disease, 2005, Volume: 28, Issue:5

    Topics: Adolescent; Ammonia; Carbamoyl-Phosphate Synthase (Ammonia); Diagnosis, Differential; DNA, Complemen

2005
Biochemical evaluation of a patient with a familial form of leucine-sensitive hypoglycemia and concomitant hyperammonemia.
    Metabolism: clinical and experimental, 1996, Volume: 45, Issue:8

    Topics: Adult; Ammonia; Drug Resistance; Female; Glutamates; Humans; Hyperinsulinism; Hypoglycemia; Infant;

1996
A syndrome of congenital hyperinsulinism and hyperammonemia.
    The Journal of pediatrics, 1997, Volume: 130, Issue:4

    Topics: Ammonia; Child; Child, Preschool; Humans; Hyperinsulinism; Infant; Syndrome

1997
Hyperinsulinemic hypoglycemia of infancy.
    The Journal of pediatrics, 1998, Volume: 132, Issue:1

    Topics: Ammonia; Humans; Hyperinsulinism; Hypoglycemia; Infant

1998
Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene.
    The New England journal of medicine, 1998, May-07, Volume: 338, Issue:19

    Topics: Ammonia; Child; Child, Preschool; DNA Mutational Analysis; Female; Glutamate Dehydrogenase; Humans;

1998
Mechanism of hyperammonemia.
    The Journal of pediatrics, 1998, Volume: 133, Issue:1

    Topics: Ammonia; Humans; Hyperinsulinism; Hypoglycemia; Infant; Infant, Premature

1998
Hyperinsulinism and hyperammonaemia.
    Journal of inherited metabolic disease, 1998, Volume: 21, Issue:6

    Topics: Adolescent; Ammonia; Humans; Hyperinsulinism; Male

1998
Muscular origin of hyperammonaemia.
    European journal of pediatrics, 1998, Volume: 157, Issue:10

    Topics: Ammonia; Child, Preschool; Glutamate Dehydrogenase; Humans; Hyperinsulinism; Male; Muscle, Skeletal

1998
Hyperinsulinism plus hyperammonemia.
    The Journal of pediatrics, 1998, Volume: 133, Issue:6

    Topics: Ammonia; Child, Preschool; Female; Humans; Hyperinsulinism; Infant

1998
Hyperinsulinaemic hypoglycaemia associated with persistent hyperammonaemia.
    European journal of pediatrics, 1999, Volume: 158, Issue:5

    Topics: Ammonia; Female; Glutamate Dehydrogenase; Humans; Hyperinsulinism; Hypoglycemia; Infant; Infant, New

1999
The structure of bovine glutamate dehydrogenase provides insights into the mechanism of allostery.
    Structure (London, England : 1993), 1999, Jul-15, Volume: 7, Issue:7

    Topics: Adenosine Diphosphate; Allosteric Regulation; Amino Acid Sequence; Amino Acid Substitution; Ammonia;

1999
Hyperinsulinism-hyperammonemia syndrome caused by mutant glutamate dehydrogenase accompanied by novel enzyme kinetics.
    Human genetics, 1999, Volume: 104, Issue:6

    Topics: Adenosine Diphosphate; Ammonia; Base Sequence; Dose-Response Relationship, Drug; Genetic Markers; Ge

1999
Novel missense mutations in the glutamate dehydrogenase gene in the congenital hyperinsulinism-hyperammonemia syndrome.
    The Journal of pediatrics, 2000, Volume: 136, Issue:1

    Topics: Adenine; Ammonia; Codon; Cytosine; Female; Glutamate Dehydrogenase; Guanine; Heterozygote; Humans; H

2000
Molecular basis and characterization of the hyperinsulinism/hyperammonemia syndrome: predominance of mutations in exons 11 and 12 of the glutamate dehydrogenase gene. HI/HA Contributing Investigators.
    Diabetes, 2000, Volume: 49, Issue:4

    Topics: Adenosine Diphosphate; Allosteric Site; Amino Acid Sequence; Ammonia; Enzyme Activation; Exons; Fema

2000
Functional hyperactivity of hepatic glutamate dehydrogenase as a cause of the hyperinsulinism/hyperammonemia syndrome: effect of treatment.
    Pediatrics, 2000, Volume: 106, Issue:3

    Topics: Ammonia; Female; Glutamate Dehydrogenase; Humans; Hyperinsulinism; Hypoglycemia; Infant, Newborn; Li

2000
A Japanese case of congenital hyperinsulinism with hyperammonemia due to a mutation in glutamate dehydrogenase (GLUD1) gene.
    Internal medicine (Tokyo, Japan), 2001, Volume: 40, Issue:1

    Topics: Allosteric Regulation; Amino Acid Substitution; Ammonia; Apnea; Coma; Genetic Heterogeneity; Glutama

2001
Acute insulin responses to leucine in children with the hyperinsulinism/hyperammonemia syndrome.
    The Journal of clinical endocrinology and metabolism, 2001, Volume: 86, Issue:8

    Topics: Adolescent; Adult; Amino Acid Substitution; Ammonia; Blood Glucose; Child; Child, Preschool; Diazoxi

2001
Chemical changes in the brain during insulin hypoglycaemia and recovery.
    Journal of neurochemistry, 1965, Volume: 12, Issue:8

    Topics: Acetylcholine; Alanine; Amino Acids; Aminobutyrates; Ammonia; Animals; Aspartic Acid; Brain; Brain C

1965