lactic acid has been researched along with Deficiency, Glucosephosphatase in 33 studies
Lactic Acid: A normal intermediate in the fermentation (oxidation, metabolism) of sugar. The concentrated form is used internally to prevent gastrointestinal fermentation. (From Stedman, 26th ed)
2-hydroxypropanoic acid : A 2-hydroxy monocarboxylic acid that is propanoic acid in which one of the alpha-hydrogens is replaced by a hydroxy group.
| Excerpt | Relevance | Reference |
|---|---|---|
| "Therapy in glycogen storage disease type Ia (GSD Ia), an inherited disorder of carbohydrate metabolism, relies on nutritional support that postpones but fails to prevent long-term complications of GSD Ia." | 7.71 | Delivery of glucose-6-phosphatase in a canine model for glycogen storage disease, type Ia, with adeno-associated virus (AAV) vectors. ( Beaty, RM; Benjamin, DK; Bird, A; Boney, A; Brown, T; Chen, YT; Jackson, M; Juopperi, T; Kishnani, P; Koeberl, DD; Peterson, D, 2002) |
| "Therapy in glycogen storage disease type Ia (GSD Ia), an inherited disorder of carbohydrate metabolism, relies on nutritional support that postpones but fails to prevent long-term complications of GSD Ia." | 3.71 | Delivery of glucose-6-phosphatase in a canine model for glycogen storage disease, type Ia, with adeno-associated virus (AAV) vectors. ( Beaty, RM; Benjamin, DK; Bird, A; Boney, A; Brown, T; Chen, YT; Jackson, M; Juopperi, T; Kishnani, P; Koeberl, DD; Peterson, D, 2002) |
| "Patients with deficient activity of hepatic glucose-6-phosphatase (glycogen storage disease type I [GSD-I]) have fasting-induced hypoglycemia, lactic acidemia, hyperuricemia, hyperlipidemia, and a markedly increased capacity for ethanol elimination." | 3.67 | Rapid ethanol elimination in patients with type I glycogen storage disease is an adaptive change resulting from recurrent hypoglycemia. ( Greene, HL; Hall, SD; Hoyumpa, AM; Parker, PH, 1986) |
| "The plasma glucose, insulin, glucagon, lactate and amino acid response patterns to glucose and protein meals were examined in 11 patients with type III glycogen storage disease (GSD-III)." | 3.66 | Amino acid disturbances in type III glycogenosis: differences from type I glycogenosis. ( Bashan, N; Coleman, RA; Moses, S; Mushlin, P; Shipp, E; Slonim, AE, 1983) |
| "Glycogen storage disease type Ia is a genetic disorder that is associated with persistent fasting hypoglycemia and the inability to produce endogenous glucose." | 1.46 | Diabetes mellitus in a patient with glycogen storage disease type Ia: a case report. ( Cohn, A; Ohri, A, 2017) |
| "This report presents lens opacities not previously described in patients with type I or III GSD." | 1.42 | Lens opacities in glycogenoses type I and III. ( Allegrini, D; Autelitano, A; De Cillà, S; Fogagnolo, P; Mazza, M; Montanari, C; Paci, S; Piozzi, E; Riva, E; Rossetti, L, 2015) |
| "Glycogen storage disease type I is associated with menorrhagia." | 1.39 | Menorrhagia in patients with type I glycogen storage disease. ( Austin, SL; El-Gharbawy, AH; James, A; Kasturi, VG; Kishnani, PS, 2013) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 10 (30.30) | 18.7374 |
| 1990's | 4 (12.12) | 18.2507 |
| 2000's | 10 (30.30) | 29.6817 |
| 2010's | 9 (27.27) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Cohn, A | 1 |
| Ohri, A | 1 |
| Kasapkara, ÇS | 1 |
| Cinasal Demir, G | 1 |
| Hasanoğlu, A | 1 |
| Tümer, L | 1 |
| Austin, SL | 2 |
| El-Gharbawy, AH | 1 |
| Kasturi, VG | 1 |
| James, A | 1 |
| Kishnani, PS | 2 |
| Jun, HS | 2 |
| Weinstein, DA | 2 |
| Lee, YM | 2 |
| Mansfield, BC | 2 |
| Chou, JY | 3 |
| Allegrini, D | 1 |
| Autelitano, A | 1 |
| Fogagnolo, P | 1 |
| De Cillà, S | 1 |
| Piozzi, E | 1 |
| Mazza, M | 1 |
| Paci, S | 1 |
| Montanari, C | 1 |
| Riva, E | 1 |
| Rossetti, L | 1 |
| Peng, WT | 1 |
| Pan, CJ | 1 |
| Lee, EJ | 1 |
| Westphal, H | 1 |
| Reddy, SK | 1 |
| Spencer-Manzon, M | 1 |
| Koeberl, DD | 2 |
| Clary, BM | 1 |
| Desai, DM | 1 |
| Smith, AD | 1 |
| Matsumoto, H | 1 |
| Kajiwara, S | 1 |
| Ogura, Y | 1 |
| Asano, T | 1 |
| Horikawa, R | 1 |
| Nonoyama, S | 1 |
| Cheung, YY | 1 |
| McDermott, DH | 1 |
| Murphy, PM | 1 |
| De Ravin, SS | 1 |
| Nagasaka, H | 2 |
| Yorifuji, T | 2 |
| Bandsma, RH | 1 |
| Takatani, T | 2 |
| Asano, H | 1 |
| Mochizuki, H | 1 |
| Takuwa, M | 1 |
| Tsukahara, H | 1 |
| Inui, A | 1 |
| Tsunoda, T | 1 |
| Komatsu, H | 1 |
| Hiejima, E | 1 |
| Fujisawa, T | 1 |
| Hirano, K | 2 |
| Miida, T | 2 |
| Ohtake, A | 2 |
| Taguchi, T | 1 |
| Miwa, I | 1 |
| Shah, KK | 1 |
| O'Dell, SD | 1 |
| Däublin, G | 1 |
| Schwahn, B | 1 |
| Wendel, U | 1 |
| Law, LK | 1 |
| Tang, NL | 1 |
| Hui, J | 1 |
| Lam, CW | 1 |
| Fok, TF | 1 |
| Mundy, HR | 1 |
| Georgiadou, P | 1 |
| Davies, LC | 1 |
| Cousins, A | 1 |
| Leonard, JV | 3 |
| Lee, PJ | 2 |
| Saunders, AC | 1 |
| Feldman, HA | 1 |
| Correia, CE | 1 |
| Murayama, K | 1 |
| Kobayashi, K | 1 |
| Kanazawa, M | 1 |
| Ogawa, A | 1 |
| Takayanagi, M | 1 |
| Di Rocco, M | 1 |
| Calevo, MG | 1 |
| Taro', M | 1 |
| Melis, D | 1 |
| Allegri, AE | 1 |
| Parenti, G | 1 |
| Slonim, AE | 1 |
| Coleman, RA | 1 |
| Moses, S | 1 |
| Bashan, N | 2 |
| Shipp, E | 1 |
| Mushlin, P | 1 |
| Smit, GP | 4 |
| Berger, R | 5 |
| Potasnick, R | 1 |
| Moses, SW | 2 |
| Fernandes, J | 5 |
| Powell, RC | 1 |
| Wentworth, SM | 1 |
| Brandt, IK | 1 |
| Chatterton, C | 1 |
| Wolfsdorf, JI | 3 |
| Crigler, JF | 2 |
| Hagen, T | 1 |
| Korson, MS | 1 |
| Beaty, RM | 1 |
| Jackson, M | 1 |
| Peterson, D | 1 |
| Bird, A | 1 |
| Brown, T | 1 |
| Benjamin, DK | 1 |
| Juopperi, T | 1 |
| Kishnani, P | 1 |
| Boney, A | 1 |
| Chen, YT | 1 |
| Ehrlich, S | 1 |
| Landy, HS | 1 |
| Collins, JE | 1 |
| Bartlett, K | 1 |
| Aynsley-Green, A | 1 |
| Williamson, DH | 1 |
| Hagai, Y | 1 |
| Potashnik, R | 1 |
| Ververs, MT | 1 |
| Belderok, B | 1 |
| Van Rijn, M | 1 |
| Greene, HL | 1 |
| Parker, PH | 1 |
| Hoyumpa, AM | 1 |
| Hall, SD | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Anaplerotic Therapy Using Triheptanoin for Patients With Glycogen Storage Disease Type I[NCT03665636] | Early Phase 1 | 4 participants (Actual) | Interventional | 2020-10-16 | Completed | ||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
1 review available for lactic acid and Deficiency, Glucosephosphatase
| Article | Year |
|---|---|
Effect of dietary interventions in the maintenance of normoglycaemia in glycogen storage disease type 1a: a systematic review and meta-analysis.
Topics: Blood Glucose; Cholesterol; Dietary Carbohydrates; Glucose; Glycogen Storage Disease Type I; Humans; | 2013 |
1 trial available for lactic acid and Deficiency, Glucosephosphatase
| Article | Year |
|---|---|
Cornstarch regimens for nocturnal treatment of young adults with type I glycogen storage disease.
Topics: Adolescent; Adult; Blood Glucose; Fatty Acids; Female; Glycogen Storage Disease Type I; Humans; Insu | 1997 |
31 other studies available for lactic acid and Deficiency, Glucosephosphatase
| Article | Year |
|---|---|
Diabetes mellitus in a patient with glycogen storage disease type Ia: a case report.
Topics: Acarbose; Adult; Blood Glucose; Canagliflozin; Diabetes Mellitus, Type 2; Female; Glycated Hemoglobi | 2017 |
Continuous glucose monitoring in children with glycogen storage disease type I.
Topics: Adolescent; Alanine Transaminase; Aspartate Aminotransferases; Blood Glucose; Child; Child, Preschoo | 2014 |
Menorrhagia in patients with type I glycogen storage disease.
Topics: Adenoma; Adult; Anemia; Blood Glucose; Female; Glycogen Storage Disease Type I; Hemoglobins; Hormone | 2013 |
Molecular mechanisms of neutrophil dysfunction in glycogen storage disease type Ib.
Topics: Adenosine Triphosphate; Adolescent; Adult; Antiporters; Child; Child, Preschool; Enzyme Activation; | 2014 |
Lens opacities in glycogenoses type I and III.
Topics: Adolescent; Adult; Cataract; Creatine Kinase; Female; Glycogen Storage Disease Type I; Glycogen Stor | 2015 |
Generation of mice with a conditional allele for G6pc.
Topics: Alleles; Animals; Blood Glucose; Cholesterol; Disease Models, Animal; DNA Primers; Gene Components; | 2009 |
Liver transplantation for glycogen storage disease type Ia.
Topics: Adult; Blood Glucose; Carcinoma, Hepatocellular; Cholesterol; Follow-Up Studies; Glycogen Storage Di | 2009 |
A case of glycogen storage disease type Ib presenting with prolonged neonatal hypoglycaemia and minimal metabolic abnormalities.
Topics: Antiporters; Diagnosis, Differential; Glucose; Glycogen Storage Disease Type I; Humans; Hypoglycemia | 2010 |
Lack of glucose recycling between endoplasmic reticulum and cytoplasm underlies cellular dysfunction in glucose-6-phosphatase-beta-deficient neutrophils in a congenital neutropenia syndrome.
Topics: Adenosine Triphosphate; Adolescent; Animals; Annexin A5; Apoptosis; Caspase 3; Child; Cytoplasm; End | 2010 |
Sustained high plasma mannose less sensitive to fluctuating blood glucose in glycogen storage disease type Ia children.
Topics: Adolescent; Blood Glucose; Child; Fasting; Female; Glucose; Glucose-6-Phosphate; Glycogen Storage Di | 2013 |
Type I glycogen storage disease: favourable outcome on a strict management regimen avoiding increased lactate production during childhood and adolescence.
Topics: Adolescent; Adult; Blood Glucose; Body Mass Index; Child; Child, Preschool; Female; Glycogen Storage | 2002 |
3-methyglutaconic aciduria in a Chinese patient with glycogen storage disease Ib.
Topics: Adolescent; Blood Glucose; Cholesterol; Diet; DNA; Gluconeogenesis; Glycogen Storage Disease Type I; | 2003 |
Exercise capacity and biochemical profile during exercise in patients with glycogen storage disease type I.
Topics: Adolescent; Adult; Blood Glucose; Exercise; Exercise Test; Fatty Acids, Nonesterified; Female; Glyco | 2005 |
Clinical evaluation of a portable lactate meter in type I glycogen storage disease.
Topics: Adolescent; Adult; Biochemistry; Chemistry, Clinical; Child; Child, Preschool; Colorimetry; Evaluati | 2005 |
Improvements of hypertriglyceridemia and hyperlacticemia in Japanese children with glycogen storage disease type Ia by medium-chain triglyceride milk.
Topics: Adolescent; Animals; Biomarkers; Child, Preschool; Cholesterol; Dietary Carbohydrates; Dietary Suppl | 2007 |
Hepatocellular adenoma and metabolic balance in patients with type Ia glycogen storage disease.
Topics: Adenoma, Liver Cell; Adolescent; Adult; Body Mass Index; Case-Control Studies; Child; Child, Prescho | 2008 |
Amino acid disturbances in type III glycogenosis: differences from type I glycogenosis.
Topics: Adolescent; Adult; Amino Acids; Blood Glucose; Child; Child, Preschool; Diagnosis, Differential; Fem | 1983 |
The dietary treatment of children with type I glycogen storage disease with slow release carbohydrate.
Topics: Adolescent; Blood Glucose; Child; Child, Preschool; Dietary Carbohydrates; Female; Glucose Tolerance | 1984 |
The lactate concentration of the urine, a parameter for the adequacy of dietary treatment of patients with glucose-6-phosphatase deficiency.
Topics: Glycogen Storage Disease Type I; Humans; Lactates; Lactic Acid | 1984 |
Lactate as a cerebral metabolic fuel for glucose-6-phosphatase deficient children.
Topics: Brain; Child; Child, Preschool; Glucose; Glycogen Storage Disease Type I; Humans; Hypoglycemia; Lact | 1984 |
Endogenous glucose production in Type I glycogen storage disease.
Topics: Adult; Blood Glucose; Ethanol; Fasting; Female; Fructose; Glucagon; Glycogen Storage Disease Type I; | 1981 |
Urinary lactate excretion in type 1 glycogenosis--a marker of metabolic control or renal tubular dysfunction?
Topics: Child; Child, Preschool; Female; Glycogen Storage Disease Type I; Humans; Kidney Tubules; Lactates; | 1996 |
Urinary lactate excretion to monitor the efficacy of treatment of type I glycogen storage disease.
Topics: Adolescent; Adult; Child; Child, Preschool; Glycogen Storage Disease Type I; Humans; Lactic Acid; Pa | 2000 |
Delivery of glucose-6-phosphatase in a canine model for glycogen storage disease, type Ia, with adeno-associated virus (AAV) vectors.
Topics: Animals; Blood Glucose; Cholesterol; Dependovirus; Dogs; Genetic Therapy; Genetic Vectors; Glucose-6 | 2002 |
Optimal daytime feeding regimen to prevent postprandial hypoglycemia in type 1 glycogen storage disease.
Topics: Adolescent; Adult; Blood Glucose; Child; Cholesterol; Eating; Female; Glycogen Storage Disease Type | 1992 |
Glucose production rates in type 1 glycogen storage disease.
Topics: Adolescent; Adult; Alanine; Blood Glucose; Female; Glucagon; Glucose; Glycogen Storage Disease Type | 1990 |
Brain substrates and the effects of nutrition.
Topics: Animal Nutritional Physiological Phenomena; Animals; Brain; Glucose-6-Phosphatase; Glycogen Storage | 1987 |
Impaired carbohydrate metabolism of polymorphonuclear leukocytes in glycogen storage disease Ib.
Topics: Adenosine Triphosphate; Adolescent; Carbohydrate Metabolism; Child, Preschool; Deoxyglucose; Glucose | 1988 |
Complex carbohydrates in the dietary management of patients with glycogenosis caused by glucose-6-phosphatase deficiency.
Topics: Adolescent; Blood Glucose; Child; Dietary Carbohydrates; Glycogen Storage Disease; Glycogen Storage | 1988 |
Rapid ethanol elimination in patients with type I glycogen storage disease is an adaptive change resulting from recurrent hypoglycemia.
Topics: Adaptation, Physiological; Adolescent; Adult; Blood Glucose; Child; Child, Preschool; Ethanol; Femal | 1986 |
Urinary excretion of lactate, 2-oxoglutarate, citrate, and glycerol in patients with glycogenosis type I.
Topics: Adolescent; Body Height; Child; Child, Preschool; Citrates; Citric Acid; Creatinine; Glycerol; Glyco | 1987 |