lactic acid has been researched along with Glycogen Storage Disease Type I 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.
Glycogen Storage Disease Type I: An autosomal recessive disease in which gene expression of glucose-6-phosphatase is absent, resulting in hypoglycemia due to lack of glucose production. Accumulation of glycogen in liver and kidney leads to organomegaly, particularly massive hepatomegaly. Increased concentrations of lactic acid and hyperlipidemia appear in the plasma. Clinical gout often appears in early childhood.
| 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 Glycogen Storage Disease Type I
| 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 Glycogen Storage Disease Type I
| 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 Glycogen Storage Disease Type I
| 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 |