scholarly journals Nutritional Management and Biochemical Outcomes during the Immediate Phase after Liver Transplant for Methylmalonic Acidemia

Nutrients ◽  
2020 ◽  
Vol 12 (10) ◽  
pp. 2976
Author(s):  
Casey Siegel ◽  
Ronen Arnon ◽  
Sander Florman ◽  
John Bucuvalas ◽  
Kimihiko Oishi

Methylmalonic acidemia (MMA) is caused by a deficiency of methyl-malonyl-CoA mutase. It is a multisystemic condition with poor clinical outcomes characterized by frequent metabolic decompensation with acidosis, hyperammonemia and encephalopathy. Restriction of intact protein and supplementation with amino acid-based formula play an important role in its management. Recently, liver transplant (LT) became a treatment option for MMA patients. However, there has been no current consensus on the post-operative nutrition management for MMA patients undergoing transplant, particularly during the initial phase of recovery period with catabolic stressors. We performed a retrospective analysis of clinical and nutritional management as well as biochemical profiles before and after LT in five patients with MMA. Through this study, we observed significant improvement of MMA-associated metabolites after LT. MMA patients were able to tolerate increased intact protein intake post-operatively. At least 1–1.5 g/kg/day of total protein during the acute phase after transplant may be tolerated without worsening of the metabolite levels. This information provides a guide in how to nutritionally manage MMA after LT.

1985 ◽  
Vol 69 (5) ◽  
pp. 505-510 ◽  
Author(s):  
E. Hultman ◽  
S. Del Canale ◽  
H. Sjöholm

1. Five volunteers were studied before and after oral administration of NH4Cl (0.3 g/kg body wt.) given in order to create a moderate acidosis. 2. The quadriceps femoris muscles were stimulated electrically for 75 s and muscle biopsies for determination of pH and metabolite content were taken before, at the end of contraction and after 10 min in the recovery period. 3. Muscle pH at rest (mean 7.04) was not significantly decreased after acidification despite an extracellular pH decrease of 0.15 unit. 4. After contraction muscle pH was significantly lower after NH4Cl. Mean values before and after acidification were 6.70 and 6.54 respectively. 5. The buffer capacity calculated as the total capacity of the muscle to buffer H+ produced during the isometric contraction before and after NH4Cl ingestion was reduced from 68.6 sl to 54.5 sl. 6. The force produced by contracting muscle was significantly lower at the end of the contraction period after NH4Cl ingestion, 44.6% of initial compared with 55.4% without NH4Cl.


2011 ◽  
Vol 22 (5) ◽  
pp. 236-241 ◽  
Author(s):  
Michelle Clayton

2018 ◽  
Vol 23 (5) ◽  
pp. 298-308 ◽  
Author(s):  
Dominique Vibert ◽  
John H.J. Allum ◽  
Martin Kompis ◽  
Simona Wiedmer ◽  
Christof Stieger ◽  
...  

The aim of this study was to investigate changes in balance control for stance and gait tasks in patients 2 years before and after vestibular neurectomy (VN) performed to alleviate intractable Meniere’s disease. Amplitudes of trunk sway in roll and pitch directions were measured for stance and gait tasks in 19 patients using gyroscopes mounted at the lower-back. Measurements before VN and 2 years later were compared to those of healthy age-matched controls (HC). We also examined if changes in trunk sway amplitudes were correlated with patients’ subjective assessment of disability using the AAO-HNS scale. For patients with low AAO-HNS scores 0–2 (n = 14), trunk roll and pitch sway velocities, standing eyes closed on foam, increased 2 years post VN compared to HC values (p < 0.01). Trunk sway amplitudes remained at levels of HC for simple gait tasks, but task durations were longer and therefore gait slower. For complex gait tasks (stairs), balance control remained impaired at 2 years. In patients with AAO-HNS high scores level 6 (n = 5), balance control remained abnormal, compared to HC, 2 years postoperatively for all stance, several simple and all complex gait tasks. Trunk sway in the pitch and roll directions for stance tasks was correlated with clinical (AAO-HNS) scores (p ≤ 0.05). These results indicate that VN leads to chronic balance problems for stance and complex gait tasks. The problems are greater for patients with high compared to low AAO-HNS scores, thereby explaining the different symptoms reported by these patients. The lack of balance recovery in VN patients to levels of HCs after 2 years contrasts with the 3 months average recovery period for acute vestibular neuritis patients and is indicative of the effects of neurectomy on central compensation processes.


1993 ◽  
Vol 75 (1) ◽  
pp. 173-180 ◽  
Author(s):  
M. J. McKenna ◽  
T. A. Schmidt ◽  
M. Hargreaves ◽  
L. Cameron ◽  
S. L. Skinner ◽  
...  

This study investigated the effects of sprint training on muscle Na(+)-K(+)-adenosinetriphosphatase (ATPase) concentration, plasma [K+] regulation, muscle performance, and fatigue during severe intermittent exercise. Six untrained male subjects underwent intensive cycle-sprint training for 7 wk. Muscle biopsies were taken at rest from the vastus lateralis muscle before and after 7 wk of training and were assayed for Na(+)-K(+)-ATPase concentration using vanadate-facilitated [3H]ouabain binding to intact samples. Before and after the training period, subjects performed four maximal 30-s exercise bouts (EB) on a cycle ergometer, each separated by a 4-min recovery. Arterialized venous blood samples were drawn immediately before and after each sprint bout and were analyzed for plasma [K+]. The work output was significantly elevated (11%) across all four EBs after training. The muscle [3H]ouabain binding site concentration was significantly increased (16%) from 333 +/- 19 to 387 +/- 15 (SE) pmol/g wet wt after training but was unchanged in muscle obtained from three control subjects. Plasma [K+] rose by 1–2 mmol/l with each EB and declined rapidly by the end of each recovery period. The increases in plasma [K+] resulting from each EB were significantly lower (19%) after training. The ratios of rise in plasma [K+] relative to work output during each EB were also significantly lower (27%) after training. The increased muscle [3H]ouabain binding site concentration and the reduced ratio of rise in [K+] relative to work output with exercise are both consistent with improved plasma and skeletal muscle K+ regulation after sprint training.


2020 ◽  
Vol 22 (100) ◽  
pp. 71-77
Author(s):  
N. M. Khomyn ◽  
A. R. Mysak ◽  
S. V. Tsisinska ◽  
V. V. Pritsak ◽  
N. V. Nazaruk ◽  
...  

Periodontal disease is known to be the most common and serious health problem in dogs today. Despite the rather large arsenal of medicinal substances, the problem of treatment and prevention of periodontal disease remains relevant. Based on this, the purpose of the work was to study the features of chronic catarrhal gingivitis and to develop an effective treatment regimen for dentistically ill dogs. For research, two groups of animals with chronic catarrhal gingivitis were formed in 5 dogs in each (control and experimental), selected on the principle of analogues in terms of age, character and localization of the inflammatory process. Animals of the control and experimental groups were performed tartar removal, irrigation of the oral cavity with water and drying of the mucosa with a sterile gauze swab. Dogs of the control group on the mucous membrane was applied 1 ml of septogel 2 times a day, and the experimental – argumentistin 2 times a day. Before and after the procedures on the mucous membrane was applied a 3 % solution of hydrogen peroxide. It was determined the prevalence of dental disease in dogs, the influence of microflora on the condition of the oral cavity of dogs with chronic catarrhal gingivitis, the clinical condition and the main indices and samples were studied, reflecting the intensity of the inflammatory process in the gums of sick dogs and was developed a method of treatment. The results of researches have shown that the use of argumentistin in the complex treatment of dogs with chronic catarrhal gingivitis helps to reduce the recovery period by 5 days.


2009 ◽  
Vol 296 (4) ◽  
pp. E787-E795 ◽  
Author(s):  
Christian Frøsig ◽  
Carsten Roepstorff ◽  
Nina Brandt ◽  
Stine J. Maarbjerg ◽  
Jesper B. Birk ◽  
...  

This study evaluated whether improved insulin-stimulated glucose uptake in recovery from acute exercise coincides with reduced malonyl-CoA (MCoA) content in human muscle. Furthermore, we investigated whether a high-fat diet [65 energy-% (Fat)] would alter the content of MCoA and insulin action compared with a high-carbohydrate diet [65 energy-% (CHO)]. After 4 days of isocaloric diet on two occasions (Fat/CHO), 12 male subjects performed 1 h of one-legged knee extensor exercise (∼80% peak workload). Four hours after exercise, insulin-stimulated glucose uptake was determined in both legs during a euglycemic-hyperinsulinemic clamp. Muscle biopsies were obtained in both legs before and after the clamp. Four hours after exercise, insulin-stimulated glucose uptake was improved (∼70%, P < 0.001) independent of diet composition and despite normal insulin-stimulated regulation of insulin receptor substrate-1-associated phosphatidylinositol 3-kinase, Akt, GSK-3, and glycogen synthase. Interestingly, exercise resulted in a sustained reduction (∼20%, P < 0.05) in MCoA content 4 h after exercise that correlated ( r = 0.65, P < 0.001) with improved insulin-stimulated glucose uptake. Four days of Fat diet resulted in an increased content of intramyocellular triacylglycerol ( P < 0.01) but did not influence muscle MCoA content or whole body insulin-stimulated glucose uptake. However, at the muscular level proximal insulin signaling and insulin-stimulated glucose uptake appeared to be compromised, although to a minor extent, by the Fat diet. Collectively, this study indicates that reduced muscle MCoA content in recovery from exercise may be part of the adaptive response leading to improved insulin action on glucose uptake after exercise in human muscle.


2018 ◽  
Vol 34 (5) ◽  
pp. 355-363
Author(s):  
Filipe S. Cardoso ◽  
Constantine J. Karvellas

Respiratory complications before and after liver transplant are common, diverse, and potentially have a negative impact on patient outcomes. In this review, we discuss the most frequent respiratory conditions that patients may develop in the perioperative period. Their prevention and/or treatment may help to maximize the benefit these patients may derive from liver transplant. This review examines diagnostic and therapeutic approaches to these complications for hepatologists, surgeons, and critical care physicians.


2013 ◽  
pp. 133-137 ◽  
Author(s):  
R. Parini ◽  
F. Furlan ◽  
A. Brambilla ◽  
D. Codazzi ◽  
S. Vedovati ◽  
...  

2019 ◽  
Vol 18 (10) ◽  
pp. 2121-2137 ◽  
Author(s):  
Joshua W. Jeffs ◽  
Nilojan Jehanathan ◽  
Stephanie M. F. Thibert ◽  
Shadi Ferdosi ◽  
Linda Pham ◽  
...  

Exposure of blood plasma/serum (P/S) to thawed conditions (> −30 °C) can produce biomolecular changes that skew measurements of biomarkers within archived patient samples, potentially rendering them unfit for molecular analysis. Because freeze-thaw histories are often poorly documented, objective methods for assessing molecular fitness before analysis are needed. We report a 10-μl, dilute-and-shoot, intact-protein mass spectrometric assay of albumin proteoforms called “ΔS-Cys-Albumin” that quantifies cumulative exposure of archived P/S samples to thawed conditions. The relative abundance of S-cysteinylated (oxidized) albumin in P/S increases inexorably but to a maximum value under 100% when samples are exposed to temperatures > −30 °C. The difference in the relative abundance of S-cysteinylated albumin (S-Cys-Alb) before and after an intentional incubation period that drives this proteoform to its maximum level is denoted as ΔS-Cys-Albumin. ΔS-Cys-Albumin in fully expired samples is zero. The range (mean ± 95% CI) observed for ΔS-Cys-Albumin in fresh cardiac patient P/S (n = 97) was, for plasma 12–29% (20.9 ± 0.75%) and for serum 10–24% (15.5 ± 0.64%). The multireaction rate law that governs S-Cys-Alb formation in P/S was determined and shown to predict the rate of formation of S-Cys-Alb in plasma and serum samples—a step that enables back-calculation of the time at which unknown P/S specimens have been exposed to room temperature. A blind challenge demonstrated that ΔS-Cys-Albumin can detect exposure of groups (n = 6 each) of P/S samples to 23 °C for 2 h, 4 °C for 16 h, or −20 °C for 24 h—and exposure of individual specimens for modestly increased times. An unplanned case study of nominally pristine serum samples collected under NIH-sponsorship demonstrated that empirical evidence is required to ensure accurate knowledge of archived P/S biospecimen storage history.


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