A series of three case reports in patients with phenylketonuria performing regular exercise: first steps in dietary adjustment

2019 ◽  
Vol 32 (6) ◽  
pp. 635-641 ◽  
Author(s):  
Júlio César Rocha ◽  
Esther van Dam ◽  
Kirsten Ahring ◽  
Manuela Ferreira Almeida ◽  
Amaya Bélanger-Quintana ◽  
...  
Keyword(s):  
Acute Exercise ◽  
Case Reports ◽  
Recovery Phase ◽  
Hydration Status ◽  
Case Presentation ◽  
Post Exercise ◽  
Protein Substitute ◽  
Low Protein ◽  
Clinical Scenarios ◽  
Post Exercise Recovery

Abstract Background Phenylketonuria (PKU), a rare, inherited metabolic condition, is treated with a strict low-phenylalanine (Phe) diet, supplemented with Phe-free protein substitute. The optimal nutritional management of a sporting individual with PKU has not been described. Therefore, guidelines for the general athlete have to be adapted. Case presentation Three clinical scenarios of sporting patients with PKU are given, illustrating dietary adaptations to usual management and challenges to attain optimal sporting performance. Therefore, the main objectives of sports nutrition in PKU are to (1) maintain a high carbohydrate diet; (2) carefully monitor hydration status; and (3) give attention to the timing of protein substitute intake in the immediate post-exercise recovery phase. Optimal energy intake should be given prior to, during and post exercise training sessions or competition. Fortunately, a usual low-Phe diet is rich in carbohydrate, but attention is required on the types of special low-protein foods chosen. Acute exercise does not seem to influence blood Phe concentrations, but further evidence is needed. Summary Well-treated PKU patients should be able to participate in sports activities, but this is associated with increased nutritional requirements and dietary adjustments. Conclusions It should be the goal of all sporting patients with PKU to maintain good metabolic Phe control and attain maximal athletic performance.

scholarly journals Reposición del Glucógeno Muscular en la Recuperación del Deportista

2019 ◽  
Vol 8 (1) ◽  
pp. 57-66 ◽  
Author(s):  
F. Mata-Ordoñez ◽  
M. Grimaldi-Puyana ◽  
A. J. Sánchez-Oliver
Keyword(s):  
Muscle Glycogen ◽  
Athletic Performance ◽  
Recovery Phase ◽  
Post Exercise ◽  
Nutritional Interventions ◽  
Carbohydrate Consumption ◽  
Cellular Processes ◽  
Intense Training ◽  
Post Exercise Recovery ◽  
Glycogen Stores

Las estrategias nutricionales durante la fase de recuperación del deportista son fundamentales. Uno de los principales objetivos de la recuperación es la reposición del glucógeno muscular. Este aspecto se hace más importante cuando los deportistas se enfrentan a entrenamientos intensos o eventos competitivos con cortos periodos de recuperación. Además, la manipulación deliberada de su disponibilidad puede mejorar las adaptaciones moleculares al entrenamiento. La presente revisión tiene por objetivo informar sobre los aspectos fisiológicos básicos de esta situación, así como conocer el momento del consumo, la cantidad, el tipo y la interacción de diferentes nutrientes con los hidratos de carbono, para poder maximizar o jugar con la reposición del mismo en función de las necesidades y/o las estrategias planteadas. El glucógeno ya no debe ser visto como un simple almacén de energía sino como una molécula que puede desencadenar numerosos procesos celulares importantes para el deportista. Nutritional interventions play a fundamental role during the post-exercise recovery phase. One of the main goals of recovery is restoring muscle glycogen stores. This becomes more important when athletes are subjected to intense training or competition with short recovery periods be-tween bouts. Furthermore, manipulating muscle glycogen availability can improve molecular adaptations to training. The objective of this review is thus to present the basic physiological aspects of this phenomenon, and to discuss carbohydrate consumption, timing, type, and amount, as well as its interaction with different nutrients, in order to maximize or play with the restoration of muscle glycogen depending on the needs and/or the strategies proposed. Glyco-gen should no longer be seen as a simple form of energy storage, but as a molecule that can trigger numerous cellular processes important for athletic performance.

scholarly journals Aberrant autonomic pattern during the post-exercise recovery phase in long QT syndrome patients

2021 ◽  
pp. 102897
Author(s):  
Anna Lundström ◽  
Urban Wiklund ◽  
Lucy Law ◽  
Steen Jensen ◽  
Marcus Karlsson ◽  
...  
Keyword(s):  
Long Qt Syndrome ◽  
Recovery Phase ◽  
Exercise Recovery ◽  
Long Qt ◽  
Post Exercise ◽  
Qt Syndrome ◽  
Post Exercise Recovery

scholarly journals Exhaustive Exercise and Post-exercise Protein Plus Carbohydrate Supplementation Affect Plasma and Urine Concentrations of Sulfur Amino Acids, the Ratio of Methionine to Homocysteine and Glutathione in Elite Male Cyclists

2020 ◽  
Vol 11 ◽  
Author(s):  
Thomas Olsen ◽  
Ove Sollie ◽  
Eha Nurk ◽  
Cheryl Turner ◽  
Fredrik Jernerén ◽  
...  
Keyword(s):  
Acute Exercise ◽  
Time Trial ◽  
Recovery Phase ◽  
Fold Change ◽  
Exhaustive Exercise ◽  
Early Recovery ◽  
Post Exercise ◽  
Carbohydrate Supplementation ◽  
Methylation Capacity ◽  
Urine Concentrations

Plasma and tissue sulfur amino acid (SAA) availability are crucial for intracellular methylation reactions and cellular antioxidant defense, which are important processes during exercise and in recovery. In this randomized, controlled crossover trial among eight elite male cyclists, we explored the effect of exhaustive exercise and post-exercise supplementation with carbohydrates and protein (CHO+PROT) vs. carbohydrates (CHO) on plasma and urine SAAs, a potential new marker of methylation capacity (methionine/total homocysteine ratio [Met/tHcy]) and related metabolites. The purpose of the study was to further explore the role of SAAs in exercise and recovery. Athletes cycled to exhaustion and consumed supplements immediately after and in 30 min intervals for 120 min post-exercise. After ~18 h recovery, performance was tested in a time trial in which the CHO+PROT group cycled 8.5% faster compared to the CHO group (41:53 ± 1:51 vs. 45:26 ± 1:32 min, p < 0.05). Plasma methionine decreased by ~23% during exhaustive exercise. Two h post-exercise, further decline in methionine had occured by ~55% in the CHO group vs. ~33% in the CHO+PROT group (pgroup × time < 0.001). The Met/tHcy ratio decreased by ~33% during exhaustive exercise, and by ~54% in the CHO group vs. ~27% in the CHO+PROT group (pgroup × time < 0.001) post-exercise. Plasma cystathionine increased by ~72% in the CHO group and ~282% in the CHO+PROT group post-exercise (pgroup × time < 0.001). Plasma total cysteine, taurine and total glutathione increased by 12% (p = 0.03), 85% (p < 0.001) and 17% (p = 0.02), respectively during exhaustive exercise. Using publicly available transcriptomic data, we report upregulated transcript levels of skeletal muscle SLC7A5 (log2 fold-change: 0.45, FDR:1.8e−07) and MAT2A (log2 fold-change: 0.38, FDR: 3.4e−0.7) after acute exercise. Our results show that exercise acutely lowers plasma methionine and the Met/tHcy ratio. This response was attenuated in the CHO+PROT compared to the CHO group in the early recovery phase potentially affecting methylation capacity and contributing to improved recovery.

scholarly journals A case report of an aorto-pulmonary-venacaval fistula after penetrating cardiac injury

2020 ◽  
Author(s):  
Johannes Gerhardus Koen ◽  
Riegardt Wagenaar ◽  
Jacques T Janson
Keyword(s):  
Case Report ◽  
Case Reports ◽  
Cardiac Injury ◽  
Operative Management ◽  
Case Presentation ◽  
Clinical Scenarios ◽  
Operative Planning ◽  
Institutional Experience ◽  
Penetrating Cardiac Injury

Abstract Background Penetrating cardiac injury (PCI) is an accepted burden in high violent crime areas. Traumatic intracardiac fistulae are however not that commonly detected on initial presentation, with most of these injuries being detected post-operatively or at routine follow-up. The literature is devoid of general principles around the pre-operative planning and intra-operative management in these cases, and thus warrant documented case reports by clinical units experienced in the management of these challenging clinical scenarios. Case summary We describe a case report of a 29-year-old male patient presenting to our Cardiothoracic Unit with an aorto-pulmonary-venacaval fistula after a traumatic PCI. We describe the clinical presentation, diagnostic challenges, and institutional experience in the operative management of this case. Discussion The patient was treated successfully with repair via sternotomy and femoral cardiopulmonary bypass with no neurological, pulmonary, or cardiac sequelae at early follow-up. The importance of selective pre-operative imaging in PCI, appropriate pre-operative surgical planning, and surgical experience in the management of these injuries is highlighted in this case presentation.

The Basis for the Increase in Factor VIII Procoagulant Activity During Exercise

1983 ◽  
Vol 49 (01) ◽  
pp. 053-057 ◽  
Author(s):  
Robert G Kopitsky ◽  
Mary Ellen P Switzer ◽  
R Sanders Williams ◽  
Patrick A McKee
Keyword(s):  
Factor Viii ◽  
Acute Exercise ◽  
Post Exercise ◽  
Fviii Activity ◽  
Plasma Factor ◽  
The Subject ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Healthy Males ◽  
Related Increase

SummaryWe studied the effect of acute exercise on the ability of thrombin to activate plasma factor VIII (FVIII) activity in 20 healthy males. The subject showed an average exercise-related increase in FVIII activity of 54.5±8.2% over pre-exercise FVIII activity (p<0.001). When exposed to the same concentration of thrombin, post-exercise FVIII activity showed greater enhancement than pre-exercise FVIII activity: 157.1±12.8% increase in activity versus 117.3±9.9%, respectively (p<0.01). The degree of the potentiated thrombin effect in post-exercise samples relative to pre-exercise samples was linearly correlated with the degree of the exercise-related increase in FVIII activity. Taken together with our previous observations that the extent of thrombin enhancement of FVIII activity varies inversely with the mole ratio of FVIII/von Willebrand factor subunits to thrombin, these findings imply that release of FVIII does not occur during exercise, and that the exercise-related increase in FVIII activity results primarily, if not completely, from activation of already circulating but inactive FVIII.

scholarly journals Spontaneous haemorrhage of an adrenal angiomyolipoma: case report

2019 ◽  
Vol 25 (1) ◽  
Author(s):  
Danielle Whiting ◽  
Ian Rudd ◽  
Amit Goel ◽  
Seshadri Sriprasad ◽  
Sanjeev Madaan
Keyword(s):  
Case Reports ◽  
Case Presentation ◽  
Open Adrenalectomy ◽  
Large Size ◽  
History Of ◽  
Loin Pain ◽  
Benign Tumours ◽  
Spontaneous Haemorrhage ◽  
Mesenchymal Tumours

Abstract Background Angiomyolipomas are rare mesenchymal tumours arising from the perivascular epithelioid cells consisting of variable amounts of adipose, thick-walled blood vessels and smooth muscle cells. These benign tumours commonly occur in the kidney with only a few case reports of adrenal angiomyolipomas which have the potential to reach a large size and haemorrhage. Case presentation A 45-year-old lady presented with a 3-week history of right loin pain, nausea and vomiting. A CT scan revealed a right adrenal angiomyolipoma measuring 6.3 × 6.8 cm with associated haemorrhage. The lesion was successfully treated with right open adrenalectomy, and histology confirmed the diagnosis of adrenal angiomyolipoma. The patient remained well with no evidence of recurrence at the 36-month follow-up. Conclusion Adrenal angiomyolipomas are rare benign tumours that have the ability to reach a large size and potential to bleed. Here, we report the second case of spontaneous haemorrhage in an adrenal angiomyolipoma, which was successfully treated with open adrenalectomy.

scholarly journals Acute motor-sensory axonal polyneuropathy variant of Guillain–Barre syndrome complicating the recovery phase of coronavirus disease 2019 infection: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Ahmed Maseh Haidary ◽  
Sarah Noor ◽  
Esmatullah Hamed ◽  
Tawab Baryali ◽  
Soma Rahmani ◽  
...  
Keyword(s):  
Recovery Phase ◽  
Sudden Onset ◽  
Therapeutic Interventions ◽  
Guillain Barre Syndrome ◽  
The Novel ◽  
Case Presentation ◽  
Progressive Muscle Weakness ◽  
Guillain Barré Syndrome ◽  
Novel Coronavirus ◽  
Guillain Barré

Abstract Introduction The novel coronavirus, since its first identification in China, in December 2019, has shown remarkable heterogeneity in its clinical behavior. It has affected humans on every continent. Clinically, it has affected every organ system. The outcome has also been variable, with most of the older patients showing grave outcomes as compared with the younger individuals. Here we present a rare and severe variant of Guillain–Barre syndrome that complicated the disease in recovery phase. Case presentation A 60-year-old Afghan man, who had been recovering from symptoms related to novel coronavirus associated disease, presented with sudden onset of progressive muscle weakness and oxygen desaturation. Electrophysiological workup confirmed the diagnosis of Guillain–Barre syndrome, and early institution of intravenous immunoglobulin resulted in complete resolution. Conclusion Guillain–Barre syndrome has recently been reported in many patients diagnosed with novel coronavirus associated disease. While clinical suspicion is mandatory to guide towards an effective diagnostic workup, early diagnosis of this complication and timely institution of therapeutic interventions are indispensable and lifesaving.

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