Page last updated: 2024-10-20

uric acid and Deficiency, Glucosephosphatase

uric acid has been researched along with Deficiency, Glucosephosphatase in 46 studies

Uric Acid: An oxidation product, via XANTHINE OXIDASE, of oxypurines such as XANTHINE and HYPOXANTHINE. It is the final oxidation product of purine catabolism in humans and primates, whereas in most other mammals URATE OXIDASE further oxidizes it to ALLANTOIN.
uric acid : An oxopurine that is the final oxidation product of purine metabolism.
6-hydroxy-1H-purine-2,8(7H,9H)-dione : A tautomer of uric acid having oxo groups at C-2 and C-8 and a hydroxy group at C-6.
7,9-dihydro-1H-purine-2,6,8(3H)-trione : An oxopurine in which the purine ring is substituted by oxo groups at positions 2, 6, and 8.

Research Excerpts

ExcerptRelevanceReference
"Studies were performed to determine whether hypoglycemia or the glucagon response to hypoglycemia increases uric acid production in glycogen storage disease type I (glucose-6-phosphatase deficiency)."7.67Hyperuricemia in glycogen storage disease type I. Contributions by hypoglycemia and hyperglucagonemia to increased urate production. ( Cohen, JL; Faller, J; Fox, IH; Vinik, A, 1985)
"Hyperuricemia (serum urate level >7 mg/dL) is caused by accelerated generation of uric acid and/or impaired excretion in the kidney."4.84[Definition and classification of hyperuricemia]. ( Yamamoto, T, 2008)
"Carbohydrate requirements (milligrams per kilogram per minute), triglyceride and uric acid levels, liver ultrasonography, and creatinine clearance were investigated before, during, and after pregnancy."3.74Pregnancies in glycogen storage disease type Ia. ( Martens, DH; Merkel, M; Rake, JP; Sauer, PJ; Schwarz, M; Smit, GP; Ullrich, K; Weinstein, DA, 2008)
"Studies were performed to determine whether hypoglycemia or the glucagon response to hypoglycemia increases uric acid production in glycogen storage disease type I (glucose-6-phosphatase deficiency)."3.67Hyperuricemia in glycogen storage disease type I. Contributions by hypoglycemia and hyperglucagonemia to increased urate production. ( Cohen, JL; Faller, J; Fox, IH; Vinik, A, 1985)
"Hepatomegaly was present in 39 out of 40, while 11 out of 27 reported patients had marked hepatomegaly (> 10 cm below the costal margin in the midclavicular line)."1.29The long-term outcome of patients with glycogen storage disease type Ia. ( Smit, GP, 1993)
"In addition, a single patient with von Gierke's disease showed a marked increase in the plasma activity of this enzyme, relative to that apparent in normal subjects."1.29Determination of human plasma xanthine oxidase activity by high-performance liquid chromatography. ( Higashino, K; Hiroishi, K; Moriwaki, Y; Nasako, Y; Takahashi, S; Tsutsumi, Z; Yamakita, J; Yamamoto, T, 1996)
"Seven patients with glycogen disease type I have been treated with nocturnal intragastric feeding combined with frequent daytime feeding."1.27Glycogen storage disease type I. Results of treatment with frequent daytime feeding, combined with nocturnal intragastric feeding and with administration of an alpha-glucosidase inhibitor. ( Gröbe, H; Ullrich, K, 1983)
"Glycogen storage disease type IB was detected by comparing glucose-6-phosphatase (EC 3."1.27Glycogen storage disease type I: laboratory data and diagnosis. ( Allam, CK; Bitar, JG; Wakid, NW, 1987)

Research

Studies (46)

TimeframeStudies, this research(%)All Research%
pre-199029 (63.04)18.7374
1990's9 (19.57)18.2507
2000's6 (13.04)29.6817
2010's2 (4.35)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Peng, WT1
Pan, CJ1
Lee, EJ1
Westphal, H1
Chou, JY1
Das, AM1
Lücke, T1
Meyer, U1
Hartmann, H1
Illsinger, S1
Adenwalla, HN1
O'Connor, CR1
Yamasaki, T1
Mineo, I1
Di Rocco, M1
Calevo, MG1
Taro', M1
Melis, D1
Allegri, AE1
Parenti, G1
Martens, DH1
Rake, JP1
Schwarz, M1
Ullrich, K3
Weinstein, DA1
Merkel, M1
Sauer, PJ1
Smit, GP2
Yamamoto, T3
Nuki, G1
Gröbe, H1
Pritchard, MH1
Wolfsdorf, JI3
Crigler, JF3
Moriwaki, Y2
Takahashi, S1
Tsutsumi, Z1
Yamakita, J1
Nasako, Y1
Hiroishi, K1
Higashino, K2
Wittenstein, B1
Klein, M1
Finckh, B1
Kohlschütter, A1
Spahr, RC1
Stormont, D1
Davies, C1
Emmerson, BT2
Watts, RW1
Roe, TF1
Kogut, MD1
Benke, PJ1
Gold, S1
Greene, HL2
Wilson, FA1
Hefferan, P1
Terry, AB1
Moran, JR1
Slonim, AE2
Claus, TH1
Burr, IM2
Ekert, H1
Waters, KD1
Marinello, E1
Pompucci, G1
Riario-Sforza, G1
Leonard, JV1
Francis, DE1
Dunger, DB1
O'Neill, JA1
Ravenscroft, PJ1
Bartolozzi, G1
Bernini, G1
Marianelli, L1
Nassi, P1
Caimi, G1
Lo Presti, R1
Chen, YT2
Feinstein, KA1
Coleman, RA2
Effmann, EL1
Scheinman, JI1
Park, HK1
Roe, CR1
Plotkin, RA1
Laffel, LM1
Rudlin, CR1
Wakid, NW1
Bitar, JG1
Allam, CK1
Cohen, JL1
Vinik, A1
Faller, J1
Fox, IH2
Pennes, DR1
Martel, W1
Dosman, J1
Crawhall, JC1
Klassen, GA1
Mamer, OA1
Neumann, P1
Boyle, JA1
Seegmiller, JE3
Howell, RR2
Fine, RN1
Strauss, J1
Donnell, GN1
Alepa, FP1
Klinenberg, JR1
Zuppinger, K1
Papenberg, J1
Schürch, P1
von Wartburg, JP1
Colombo, JP1
Rossi, E1
Benedetti, A1
Bucciante, G1
Cattano, G1
Nardini, A1
Neubaur, J2
Willms, B2
Söling, HD1
Creutzfeldt, W1
Matschke, I1
Wolf, H1

Clinical Trials (1)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Anaplerotic Therapy Using Triheptanoin for Patients With Glycogen Storage Disease Type I[NCT03665636]Early Phase 14 participants (Actual)Interventional2020-10-16Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Reviews

8 reviews available for uric acid and Deficiency, Glucosephosphatase

ArticleYear
[Secondary hyperuricemia in glycogen storage disease types I, III, V and VII].
    Nihon rinsho. Japanese journal of clinical medicine, 2003, Volume: 61 Suppl 1

    Topics: Glycogen Storage Disease Type I; Glycogen Storage Disease Type III; Glycogen Storage Disease Type V;

2003
[Definition and classification of hyperuricemia].
    Nihon rinsho. Japanese journal of clinical medicine, 2008, Volume: 66, Issue:4

    Topics: Alcohol Drinking; Diet, Reducing; Fructose; Glucose-6-Phosphatase; Glycogen Storage Disease Type I;

2008
Human purine metabolism: some recent advances and relationships with immunodeficiency.
    Annals of the rheumatic diseases, 1983, Volume: 42 Suppl 1

    Topics: Adenine Phosphoribosyltransferase; Adenosine Deaminase; Glycogen Storage Disease Type I; Gout; Human

1983
Gout and pseudogout: crystal-induced arthropathies.
    Clinics in endocrinology and metabolism, 1981, Volume: 10, Issue:1

    Topics: Calcium Pyrophosphate; Chondrocalcinosis; Crystallization; Diuretics; Ethanol; Glycogen Storage Dise

1981
Metabolic causes of renal stone formation.
    Postgraduate medical journal, 1977, Volume: 53 Suppl 2

    Topics: Adenine Phosphoribosyltransferase; Cystinuria; Glycogen Storage Disease Type I; Humans; Hypoxanthine

1977
[Screening for enzyme abnormality causing hyperuricemia].
    Nihon rinsho. Japanese journal of clinical medicine, 1991, Volume: 49, Issue:5

    Topics: Glycogen Storage Disease Type I; Humans; Hypoxanthine Phosphoribosyltransferase; Lesch-Nyhan Syndrom

1991
[Physiopathological aspects of secondary hyperuricemia].
    Recenti progressi in medicina, 1991, Volume: 82, Issue:11

    Topics: Acute Disease; Coronary Disease; Drug-Related Side Effects and Adverse Reactions; Fructose; Glycogen

1991
Selected aspects of the aetiology, pathogenesis and treatment of disorders of purine metabolism.
    Modern trends in rheumatology, 1971, Volume: 2

    Topics: Allopurinol; Colchicine; Glycogen Storage Disease Type I; Gout; Humans; Lesch-Nyhan Syndrome; Metabo

1971

Trials

2 trials available for uric acid and Deficiency, Glucosephosphatase

ArticleYear
Biochemical evidence for the requirement of continuous glucose therapy in young adults with type 1 glycogen storage disease.
    Journal of inherited metabolic disease, 1994, Volume: 17, Issue:2

    Topics: Adolescent; Adult; Blood Glucose; Female; Glucose; Glycogen Storage Disease Type I; Humans; Hypoglyc

1994
Continuous glucose for treatment of patients with type 1 glycogen-storage disease: comparison of the effects of dextrose and uncooked cornstarch on biochemical variables.
    The American journal of clinical nutrition, 1990, Volume: 52, Issue:6

    Topics: Adolescent; Blood Glucose; Child; Child, Preschool; Cholesterol; Female; Glucose; Glycogen Storage D

1990

Other Studies

36 other studies available for uric acid and Deficiency, Glucosephosphatase

ArticleYear
Generation of mice with a conditional allele for G6pc.
    Genesis (New York, N.Y. : 2000), 2009, Volume: 47, Issue:9

    Topics: Alleles; Animals; Blood Glucose; Cholesterol; Disease Models, Animal; DNA Primers; Gene Components;

2009
Glycogen storage disease type 1: impact of medium-chain triglycerides on metabolic control and growth.
    Annals of nutrition & metabolism, 2010, Volume: 56, Issue:3

    Topics: Adult; Body Height; Body Weight; Child, Preschool; Diet; Dietary Carbohydrates; Dietary Fats; Energy

2010
Catastrophic axial gout causing paraplegia in a patient with glycogen storage disease.
    Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases, 2011, Volume: 17, Issue:7

    Topics: Adult; Catastrophic Illness; Drug Therapy, Combination; Febuxostat; Glycogen Storage Disease Type I;

2011
Hepatocellular adenoma and metabolic balance in patients with type Ia glycogen storage disease.
    Molecular genetics and metabolism, 2008, Volume: 93, Issue:4

    Topics: Adenoma, Liver Cell; Adolescent; Adult; Body Mass Index; Case-Control Studies; Child; Child, Prescho

2008
Pregnancies in glycogen storage disease type Ia.
    American journal of obstetrics and gynecology, 2008, Volume: 198, Issue:6

    Topics: Adult; Dietary Carbohydrates; Female; Glycogen Storage Disease Type I; Humans; Infant, Newborn; Preg

2008
Glycogen storage disease type I. Results of treatment with frequent daytime feeding, combined with nocturnal intragastric feeding and with administration of an alpha-glucosidase inhibitor.
    European journal of pediatrics, 1983, Volume: 140, Issue:2

    Topics: Acarbose; Alanine; Aspartate Aminotransferases; Blood Glucose; Child; Enteral Nutrition; Female; Gly

1983
The long-term outcome of patients with glycogen storage disease type Ia.
    European journal of pediatrics, 1993, Volume: 152 Suppl 1

    Topics: Adenoma; Adolescent; Adult; Aged; Apolipoproteins; Blood Glucose; Child; Cholesterol; Female; Glycog

1993
Determination of human plasma xanthine oxidase activity by high-performance liquid chromatography.
    Journal of chromatography. B, Biomedical applications, 1996, Jun-07, Volume: 681, Issue:2

    Topics: Chromatography, High Pressure Liquid; Ethanol; Glycogen Storage Disease Type I; Gout; Heparin; Hepat

1996
Plasma antioxidants in pediatric patients with glycogen storage disease, diabetes mellitus, and hypercholesterolemia.
    Free radical biology & medicine, 2002, Jul-01, Volume: 33, Issue:1

    Topics: Adolescent; Adult; Antioxidants; Ascorbic Acid; Child; Child, Preschool; Cholesterol; Chromatography

2002
Von Gierke's disease: a review.
    Southern medical journal, 1976, Volume: 69, Issue:8

    Topics: Allopurinol; Bicarbonates; Blood Glucose; Diazoxide; Gluconeogenesis; Glucose-6-Phosphatase; Glycoge

1976
Urate production in heterozygotes for glucose-6-phosphatase deficiency.
    Clinica chimica acta; international journal of clinical chemistry, 1976, Sep-06, Volume: 71, Issue:2

    Topics: Adult; Blood Platelets; Child, Preschool; Erythrocytes; Female; Glucose-6-Phosphatase; Glucosephosph

1976
The pathogenesis of hyperuricemia in glycogen storage disease, type I.
    Pediatric research, 1977, Volume: 11, Issue:5

    Topics: Adolescent; Adult; Child; Child, Preschool; Female; Fructose; Glucagon; Glycogen Storage Disease Typ

1977
Uric acid metabolism in therapy of glycogen storage disease type I.
    Pediatric research, 1978, Volume: 12, Issue:3

    Topics: Child; Dietary Carbohydrates; Glycine; Glycogen Storage Disease Type I; Humans; Intubation, Gastroin

1978
ATP depletion, a possible role in the pathogenesis of hyperuricemia in glycogen storage disease type I.
    The Journal of clinical investigation, 1978, Volume: 62, Issue:2

    Topics: Adenosine Triphosphate; Adolescent; Adult; Animals; Child; Child, Preschool; Glucagon; Glycogen Stor

1978
Hematologic emergencies in children.
    Paediatrician, 1978, Volume: 7, Issue:4-5

    Topics: Acute Disease; Acute Kidney Injury; Adolescent; Anemia, Aplastic; Anemia, Hemolytic, Autoimmune; Asp

1978
[Interrelationship between purine, carbohydrate, lipid and aminoacid metabolism, in hyperuricaemia and gout (author's transl)].
    Quaderni Sclavo di diagnostica clinica e di laboratorio, 1978, Volume: 14, Issue:1

    Topics: Alcoholic Intoxication; Alcoholism; Amino Acids; Carbohydrate Metabolism; Diabetes Mellitus; Fructos

1978
The dietary management of hepatic glycogen storage disease.
    The Proceedings of the Nutrition Society, 1979, Volume: 38, Issue:3

    Topics: Blood Glucose; Cholesterol; Glucose; Glycogen Storage Disease; Glycogen Storage Disease Type I; Huma

1979
Continuous nocturnal intragastric feeding for management of type 1 glycogen-storage disease.
    The New England journal of medicine, 1976, Feb-19, Volume: 294, Issue:8

    Topics: Adolescent; Aspartate Aminotransferases; Blood Glucose; Child; Cholesterol; Clofibrate; Eating; Ente

1976
Abnormal renal urate homeostasis in systemic disorders.
    Nephron, 1975, Volume: 14, Issue:1

    Topics: Alcoholism; Diabetes Mellitus; Female; Fructose Intolerance; Glomerular Filtration Rate; Glycogen St

1975
[Type I glycogenosis. Description of 2 cases with hyperuricemia].
    Minerva pediatrica, 1975, Jun-23, Volume: 27, Issue:22

    Topics: Biopsy; Child; Galactose; Glucagon; Glucose Tolerance Test; Glycogen Storage Disease Type I; Gout; H

1975
Variability of renal length in type I glycogen storage disease.
    Journal of inherited metabolic disease, 1990, Volume: 13, Issue:3

    Topics: Adolescent; Child; Child, Preschool; Cholesterol; Female; Glycogen Storage Disease Type I; Humans; K

1990
Amelioration of proximal renal tubular dysfunction in type I glycogen storage disease with dietary therapy.
    The New England journal of medicine, 1990, Aug-30, Volume: 323, Issue:9

    Topics: Adolescent; Adult; beta 2-Microglobulin; Blood Glucose; Cholesterol; Dietary Carbohydrates; Female;

1990
Physical growth and development of children with type 1 glycogen-storage disease: comparison of the effects of long-term use of dextrose and uncooked cornstarch.
    The American journal of clinical nutrition, 1990, Volume: 52, Issue:6

    Topics: Adolescent; Blood Glucose; Body Height; Body Weight; Child; Child, Preschool; Cholesterol; Female; G

1990
Glycogen storage disease type I: laboratory data and diagnosis.
    Clinical chemistry, 1987, Volume: 33, Issue:11

    Topics: Adult; Alanine Transaminase; Aspartate Aminotransferases; Child, Preschool; Cholesterol; Female; Glu

1987
Hyperuricemia in glycogen storage disease type I. Contributions by hypoglycemia and hyperglucagonemia to increased urate production.
    The Journal of clinical investigation, 1985, Volume: 75, Issue:1

    Topics: Adenosine Triphosphate; Adolescent; Adult; Female; Glucagon; Glucose; Glycogen Storage Disease Type

1985
Hyperuricemia and gout.
    Seminars in roentgenology, 1986, Volume: 21, Issue:4

    Topics: Adolescent; Age Factors; Arthritis; Arthrography; Bone Diseases; Child; Diagnosis, Differential; Fem

1986
Adenosine triphosphate degradation in specific disease.
    The Journal of laboratory and clinical medicine, 1985, Volume: 106, Issue:2

    Topics: Adenine; Adenosine Triphosphate; Biotransformation; Carnitine O-Palmitoyltransferase; Ethanol; Fruct

1985
Urinary excretion of C6-C10 dicarboxylic acids in glycogen storage disease types I and 3.
    Clinica chimica acta; international journal of clinical chemistry, 1974, Feb-28, Volume: 51, Issue:1

    Topics: Adolescent; Blood Glucose; Body Height; Body Weight; Carbon Radioisotopes; Child; Cholesterol; Chrom

1974
The interrelationship of glycogen storage disease and gout.
    Arthritis and rheumatism, 1965, Volume: 8, Issue:5

    Topics: Adult; Child; Glycogen Storage Disease Type I; Gout; Humans; Kidney Function Tests; Lactates; Uric A

1965
Hyperuricemia in glycogen-storage disease type 1.
    American journal of diseases of children (1960), 1966, Volume: 112, Issue:6

    Topics: Adolescent; Adult; Child; Child, Preschool; Glucose; Glycogen Storage Disease; Glycogen Storage Dise

1966
Relationships between glycogen storage disease and tophaceous gout.
    The American journal of medicine, 1967, Volume: 42, Issue:1

    Topics: Adult; Female; Glucose Tolerance Test; Glycogen Storage Disease Type I; Gout; Humans; Inulin; Lactat

1967
[Increased alcohol oxydation in glycogenosis type I].
    Schweizerische medizinische Wochenschrift, 1967, Aug-26, Volume: 97, Issue:34

    Topics: Adolescent; Adult; Blood Glucose; Child; Ethanol; Fatty Acids, Nonesterified; Glycerides; Glycogen S

1967
[Gout in the evolution of Von Gierke's disease].
    Minerva medica, 1969, May-12, Volume: 60, Issue:38

    Topics: Adolescent; Adult; Allopurinol; Child; Female; Glycogen Storage Disease; Glycogen Storage Disease Ty

1969
[Gout as a complication of adult glycogen storage disease].
    Archiv fur klinische Medizin, 1969, Volume: 216, Issue:2

    Topics: Adolescent; Adult; Blood Glucose; Female; Glycogen Storage Disease Type I; Gout; Humans; Lactates; M

1969
Metabolic aberrations in gout.
    Clinical orthopaedics and related research, 1970, Volume: 71

    Topics: Female; Glycogen Storage Disease Type I; Gout; Humans; Kidney; Kidney Calculi; Kidney Diseases; Male

1970
[Metabolism studies in children and young adults with glycogenoses].
    Monatsschrift fur Kinderheilkunde, 1969, Volume: 117, Issue:4

    Topics: Adolescent; Adult; Child; Fatty Acids; Glucagon; Glucosidases; Glucosyltransferases; Glycogen Storag

1969