lactic acid and Luft Disease

lactic acid has been researched along with Luft Disease in 56 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.

Research

Studies (56)

Authors

Clinical Trials (3)

Trial Overview

Trial Outcomes

Change of Peak Workload (in Watts/kg) During Exercise Testing

Cycle ergometry using a stationary recumbent bike was used to conduct maximal exercise testing. Peak work is defined as the workload at which patients reach maximal volition (defined as an inability to continue to exercise due to exhaustion). Change of peak workload during exercise testing was measured at baseline, Week 4, and Week 12. Change from baseline at Week 12 reported. (NCT02255422)
Timeframe: 12 weeks

Change in 6-minute Walk Test (6MWT) Distance

Patients were instructed to walk as far as they could along a marked path for 6 minutes. Distance walked was measured. If patients used a cane or walking assist device at Screening, the same walking assist device was to be used for all 6MWT assessments. (NCT02255422)
Timeframe: 6MWT was assessed at Week 4, Week 8, and Week 12 and compared to baseline

Change From Baseline in 6 Minute Walk Test

"The investigator selected the two most preeminent symptoms for each participant during the screening visit from the following: Myopathy, dystonia, ataxia, retarded motor development, reduced activities of daily living, and vision. The 2 symptoms selected for each participant were then assessed at each subsequent study visit.~Myopathy was assessed using the 6 minute walk test, which measures the distance walked in a 6 minute walk test." (NCT02023866)
Timeframe: Baseline and Weeks 4, 8, 12, 16, 20, 24

Change From Baseline in Barry-Albright Dystonia Scale Total Score

"The investigator selected the two most preeminent symptoms for each participant during the screening visit from the following: myopathy, dystonia, ataxia, retarded motor development, reduced activities of daily living, and vision. The 2 symptoms selected for each participant were then assessed at each subsequent study visit.~Dystonia symptoms were assessed using the Barry-Albright Dystonia Scale for Dystonia. Participants were assessed for dystonia in each of the following regions: eyes, mouth, neck, trunk, and each upper and lower extremity (8 body regions) on a scale from 0 (absent) to 4 (severe symptoms). The individual scores were summed to calculate the total score which ranges from 0 (dystonia absent) to 32 (severe dystonia)." (NCT02023866)
Timeframe: Baseline and Weeks 4, 8, 12, 16, 20, 24

Change From Baseline in Friedreich Ataxia Rating Scale

"The investigator selected the two most preeminent symptoms for each participant during the screening visit from the following: myopathy, dystonia, ataxia, retarded motor development, reduced activities of daily living, and vision. The 2 symptoms selected for each participant were then assessed at each subsequent study visit.~Ataxia was assessed using the Friedreich Ataxia Rating Scale (FARS). FARS comprises a functional ataxia staging score of overall mobility (score 0 to 6), an assessment of the activities of daily living (ADL) (score 0 to 36) and a neurological assessment (score from 0 to 117) which is composed of bulbar (score 0-11), upper limb (score 0- 36) and lower limb (score 0-16), peripheral nerve (score 0-26) and upright stability/gait (score 0-28). The scores were summed to calculate the total score which ranges from 0 to 159. A higher score indicates a greater level of disability." (NCT02023866)
Timeframe: Baseline and Weeks 4, 8, 12, 16, 20, 24

Change From Baseline in Glutathione

(NCT02023866)
Timeframe: Baseline and Weeks 4, 8, 12, 16, 20, 24

Change From Baseline in Glutathione Disulfide

(NCT02023866)
Timeframe: Baseline and Weeks 4, 8, 12, 16, 20, 24

Change From Baseline in Gross Motor Function

"The investigator selected the two most preeminent symptoms for each participant during the screening visit from the following: myopathy, dystonia, ataxia, retarded motor development, reduced activities of daily living, and vision. The 2 symptoms selected for each participant were then assessed at each subsequent study visit.~Retarded motor development was assessed using the Gross Motor Function Measure (GMFM)-88 which consists of 88 items scored on a scale of 0 to 3:~0: Does not initiate the task;~Initiates the task (completes < 10%);~Partially completes the task (10 to 99%);~Completes the task (100%).~The 88 items are grouped into five dimensions: 1) lying and rolling, 2) sitting, 3) crawling and kneeling, 4) standing, and 5) walking, running and jumping. Scores are expressed as a percentage of the maximum score for that dimension. The total score is the average of the 5 the percentage scores where higher scores indicate better performance." (NCT02023866)
Timeframe: Baseline and Weeks 4, 8, 12, 16, 20, 24

Change From Baseline in Jamar Dynamometer Hand Strength

"The investigator selected the two most preeminent symptoms for each participant during the screening visit from the following: myopathy, dystonia, ataxia, retarded motor development, reduced activities of daily living, and vision. The 2 symptoms selected for each participant were then assessed at each subsequent study visit.~Myopathy was assessed using standard grip strength evaluation, which measures hand strength in both hands using a Jamar dynamometer." (NCT02023866)
Timeframe: Baseline and Weeks 4, 8, 12, 16, 20, 24

Change From Baseline in Lactic Acid

(NCT02023866)
Timeframe: Baseline and Weeks 4, 8, 12, 16, 20, 24

Change From Baseline in Modified Lansky Play Performance Scale

"The investigator selected the 2 most preeminent symptoms for each participant during the screening visit from the following: myopathy, dystonia, ataxia, retarded motor development, reduced activities of daily living, and vision. The 2 symptoms were assessed at each subsequent study visit.~Reduced activities of daily living was assessed using the modified Lansky Play Performance Scale, completed by parents based on their child's activity in the past week, where 100=fully active; 90=minor restrictions in strenuous physical activity; 80=active, gets tired more quickly; 70=greater restriction of play, less time spent in play activity; 60=up and around, active play minimal; quieter activities; 50=lying around much of the day; no active playing, all quiet play and activities; 40=mainly in bed; quiet activities; 30=bedbound; needs assistance even for quiet play; 20=sleeps often; play limited to very passive activities; 10=doesn't play or get out of bed; 5=unresponsive 0=dead" (NCT02023866)
Timeframe: Baseline and Weeks 4, 8, 12, 16, 20, 24

Change From Baseline in Newcastle Paediatric Mitochondrial Disease Scale (NPMDS) Sections I-IV

"The NPMDS evaluates the progression of mitochondrial disease in pediatric patients in 4 domains:~I - Current Function (vision, hearing, communication, feeding, and mobility) with scores ranging from 0 to 21; II -System Specific Involvement (seizures, encephalopathy, bleeding diathesis or coagulation defects, gastrointestinal, endocrine, respiratory, cardiovascular, renal, liver, and blood) with scores ranging from 0 to 30.~III - Current Clinical Assessment (growth and development over past 6 months, vision, strabismus and eye movement, myopathy, ataxia, pyramidal, extrapyramidal, and neuropathy) with scores ranging from 0 to 28; and IV - Quality of Life with scores ranging from 0 to 25. For sections I-III, higher scores reflect more severe disease. For Section IV, a higher score reflects a lower quality of life." (NCT02023866)
Timeframe: Baseline through Week 24

Reviews

3 reviews available for lactic acid and Luft Disease

Trials

10 trials available for lactic acid and Luft Disease

Other Studies

43 other studies available for lactic acid and Luft Disease