scholarly journals Hypotonia with lactic acidemia and hyperammonemia

2020 ◽  
Author(s):  
Keyword(s):  
Lactic Acidemia

scholarly journals Discordance between lactic acidemia and hemodynamics in patients with advanced heart failure

2021 ◽  
Author(s):  
Nikhil Narang ◽  
Mark Dela Cruz ◽  
Teruhiko Imamura ◽  
Ben Chung ◽  
Ann B. Nguyen ◽  
...  
Keyword(s):  
Heart Failure ◽  
Advanced Heart Failure ◽  
Lactic Acidemia

scholarly journals E4F1 controls a transcriptional program essential for pyruvate dehydrogenase activity

2016 ◽  
Vol 113 (39) ◽  
pp. 10998-11003 ◽  
Author(s):  
Matthieu Lacroix ◽  
Geneviève Rodier ◽  
Olivier Kirsh ◽  
Thibault Houles ◽  
Hélène Delpech ◽  
...  
Keyword(s):  
Pyruvate Dehydrogenase ◽  
Energy Demand ◽  
Clinical Symptoms ◽  
Tricarboxylic Acid Cycle ◽  
Striated Muscles ◽  
Pyruvate Oxidation ◽  
Mitochondrial Pyruvate Carrier ◽  
Lactic Acidemia ◽  
Transcription Factor 1 ◽  
Dihydrolipoyl Dehydrogenase

The mitochondrial pyruvate dehydrogenase (PDH) complex (PDC) acts as a central metabolic node that mediates pyruvate oxidation and fuels the tricarboxylic acid cycle to meet energy demand. Here, we reveal another level of regulation of the pyruvate oxidation pathway in mammals implicating the E4 transcription factor 1 (E4F1). E4F1 controls a set of four genes [dihydrolipoamide acetlytransferase (Dlat), dihydrolipoyl dehydrogenase (Dld), mitochondrial pyruvate carrier 1 (Mpc1), and solute carrier family 25 member 19 (Slc25a19)] involved in pyruvate oxidation and reported to be individually mutated in human metabolic syndromes. E4F1 dysfunction results in 80% decrease of PDH activity and alterations of pyruvate metabolism. Genetic inactivation of murine E4f1 in striated muscles results in viable animals that show low muscle PDH activity, severe endurance defects, and chronic lactic acidemia, recapitulating some clinical symptoms described in PDC-deficient patients. These phenotypes were attenuated by pharmacological stimulation of PDH or by a ketogenic diet, two treatments used for PDH deficiencies. Taken together, these data identify E4F1 as a master regulator of the PDC.

LACTIC ACIDEMIA, HYPERALANEMIA AND CARNITINE ABNORMALITIES IN DOGS WITH LIPID STORAGE MYOPATHY

1995 ◽  
Vol 54 (3) ◽  
pp. 457 ◽  
Author(s):  
G D Shelton ◽  
R H Hass ◽  
B Holmes ◽  
L Mitchell ◽  
R Broock ◽  
...  
Keyword(s):  
Lipid Storage ◽  
Lipid Storage Myopathy ◽  
Lactic Acidemia

Abstract 19: Progressive Metabolic Derangement During Prolonged Resuscitation for Refractory VT/VF Cardiac Arrest and the Relationship to Neurologically Intact Survival With Extracorporeal Cardiopulmonary Resuscitation

Circulation ◽  
2018 ◽  
Vol 138 (Suppl_2) ◽  
Author(s):  
Jason A Bartos ◽  
Claire Carlson ◽  
Ganesh Raveendran ◽  
Ranjit John ◽  
Tom P Aufderheide ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Extracorporeal Cardiopulmonary Resuscitation ◽  
Metabolic Derangement ◽  
Catheterization Laboratory ◽  
Cardiac Catheterization Laboratory ◽  
Substantial Decline ◽  
Lactic Acidemia ◽  
First Time ◽  
Neurologically Intact ◽  
Over Time

Background: Multiple studies have shown declining likelihood of neurologically intact survival with prolonged resuscitation with standard CPR. With standard CPR, survival after VT/VF arrest declines from 35-45% at initiation of CPR to 10-20% survival at 30 min. Objective: The aim of this study was to examine the effects of resuscitation duration on neurologically intact survival in the Minnesota Resuscitation Consortium ECPR protocol. Further, the progressive metabolic derangement of prolonged resuscitation was observed for the first time in this population. Methods: Between December 1, 2015 and May 1, 2018, 115 consecutive adult patients with refractory out-of-hospital VT/VF cardiac arrest requiring ongoing CPR were transported to the cardiac catheterization laboratory where ECLS was initiated and coronary angiography and PCI were performed as needed. Patients achieving an organized cardiac rhythm were admitted for further treatment. Results: Overall, 41% of patients receiving full resuscitative efforts were discharged neurologically intact. Neurologically intact survival declined with increasing duration of CPR with 100% survival in patients placed on ECLS within 30 min. Survival declined to 50% within 50 min and 20% within 70 min. Lactic acid and paCO2 increased over time peaking at 15 mmol/L and 72 mmHg, respectively. pH declined accordingly reaching 6.92 at its nadir. paO2 was stable over time but variable between patients. Conclusions: Likelihood of neurologically intact survival declined with increasing duration of CPR for patients going on to receive ECLS. The metabolic profile worsened during prolonged CPR with increasing lactic acidemia and hypercapnia but survival could be achieved with the hemodynamic support provided by ECLS. Together these findings support the need for rapid transport of patients to teams prepared to place ECLS. Substantial decline in survival begins at 30 min of CPR.

Successful treatment of infantile-onset ACAD9-related cardiomyopathy with a combination of sodium pyruvate, beta-blocker, and coenzyme Q10

2019 ◽  
Vol 32 (10) ◽  
pp. 1181-1185 ◽  
Author(s):  
Takumi Kadoya ◽  
Azumi Sakakibara ◽  
Kana Kitayama ◽  
Yuki Yamada ◽  
Shinji Higuchi ◽  
...  
Keyword(s):  
Beta Blocker ◽  
Coenzyme Q ◽  
Chain Complex ◽  
Left Ventricular ◽  
Additional Treatment ◽  
Left Ventricular Ejection ◽  
Sodium Pyruvate ◽  
Ventricular Ejection Fraction ◽  
Treatment Measures ◽  
Lactic Acidemia

Abstract Mitochondrial acyl-CoA dehydrogenase 9 (ACAD9) deficiency is one of the common causes of respiratory chain complex I deficiency, which is characterized by cardiomyopathy, lactic acidemia, and muscle weakness. Infantile cardiomyopathy is the most common phenotype and is usually lethal by the age of 5 years. Riboflavin treatment is known to be effective in ~65% of the patients; however, the remaining are unresponsive to riboflavin and are in need of additional treatment measures. In this report, we describe a patient with ACAD9 deficiency who developed progressive cardiomyopathy at 8 months of age. As the patient’s left ventricular ejection fraction (LVEF) kept decreasing to 45.4% at 1 year 8 months, sodium pyruvate treatment was introduced together with a beta-blocker and coenzyme Q10. This resulted in a steady improvement, with full and sustained normalization of cardiac function without riboflavin. The therapy, therefore, might be a useful addition for the treatment of ACAD9 deficiency.

scholarly journals D-lactic acidemia and aciduria in pediatric and adult patients with short bowel syndrome

1995 ◽  
Vol 41 (1) ◽  
pp. 107-110 ◽  
Author(s):  
G Bongaerts ◽  
J Tolboom ◽  
T Naber ◽  
J Bakkeren ◽  
R Severijnen ◽  
...  
Keyword(s):  
Lactate Production ◽  
High Serum ◽  
Dietary Factors ◽  
Serum Concentrations ◽  
Short Bowel ◽  
Gram Positive Bacteria ◽  
Adults And Children ◽  
Lactic Acidemia ◽  
Lactate Excretion ◽  
Main Factor

Abstract D-Lactate produced by abundant intestinal lactobacilli during acidotic episodes in short bowel (SB) patients is commonly regarded as a main factor in the pathogenesis of SB syndrome-associated (D-lactic) acidosis. Since we had observed that gram-positive bacteria, mainly lactobacilli, were abundant even in the absence of acidosis, we studied serum concentrations and urinary excretions of D- and L-lactate in young and adult SB patients, especially during nonacidotic periods. Serum L-lactate and urinary L-lactate excretion were similar in adults and children. Serum D-lactate and urinary D-lactate excretion were higher in SB children than in SB adults. Food consumption affects D-lactate production and alters D-lactic acidemia and aciduria. We conclude that D-lactate is frequently present in serum of SB patients even in the absence of acidosis. High serum concentrations and urinary excretions may reflect dietary factors in these patients.

Pneumatosis Intestinalis in Patients Receiving Tube Feeds

2017 ◽  
Vol 83 (8) ◽  
pp. 825-831
Author(s):  
Alexander C. Cavalea ◽  
Robert E. Heidel ◽  
Brian J. Daley ◽  
Christy M. Lawson ◽  
Darrell A. Benton ◽  
...  
Keyword(s):  
Statistical Significance ◽  
Tube Feeding ◽  
Pneumatosis Intestinalis ◽  
Bowel Function ◽  
Feeding Tube ◽  
Outcome Data ◽  
Free Fluid ◽  
Feeding Intolerance ◽  
Lactic Acidemia ◽  
Bowel Dilatation

Pneumatosis intestinalis (PI) identified on computed tomography (CT) suggests an underlying pathology including bowel ischemia. Patients receiving tube feeds can develop PI, potentially requiring surgical intervention. We identify clinical factors in PI to predict those that may be safe to observe versus those that need immediate intervention. We retrospectively reviewed patients from a single institution from 2008 to 2016 with CT findings of PI and an enteric feeding tube. Patients who had not received tube feeds within one week of the CT were excluded. We analyzed clinical, operative, and outcome data to differentiate benign from pathologic outcomes. P values < 0.05 were set as significant. Forty patients were identified. We classified 24 as benign (no intervention) and 16 as pathologic (requiring intervention). A pathologic outcome was demonstrated for free fluid on CT [odds ratio (OR) = 5.00, confidence interval (CI) 1.23-20.30, P = 0.03)], blood urea nitrogen (BUN) elevation (OR = 8.27, CI 1.53-44.62, P = 0.01), creatinine (Cr) elevation (OR = 5.00, CI 1.27-19.62, P = 0.02), BUN/Cr ratio >30 (OR = 8.57, CI 1.79-40.98, P = 0.006), and vomiting/ feeding intolerance (OR = 9.38, CI 1.64-53.62, P = 0.01). Bowel function within 24 hours of the CT, bowel dilatation (small ≥ 3 cm; large ≥6 cm), and lactic acidemia were not significant. Peritonitis was only seen in pathologic states, but this did not reach statistical significance (P = 0.06). This represents the largest single-center retrospective analysis of tube feeding-induced PI to date. The presence of free fluid on CT, BUN and Cr elevation, BUN/Cr >30, vomiting/feeding intolerance and peritonitis were predictive of a pathologic etiology of PI.

scholarly journals Abnormal Pyruvate and α-Ketoglutarate Dehydrogenase Complexes in a Patient with Lactic Acidemia

1979 ◽  
Vol 13 (8) ◽  
pp. 928-931 ◽  
Author(s):  
Yasuhiro Kuroda ◽  
Jeffrey J Kline ◽  
Lawrence Sweetman ◽  
William L Nyhan ◽  
Ted D Groshong
Keyword(s):  
Lactic Acidemia ◽  
Ketoglutarate Dehydrogenase
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