Pathophysiology of Physical Exercise in Kidney Patients: Unveiling New Players- The Role of Myokines.

Background Chronic kidney disease (CKD) is a progressive systemic condition characterized by numerous complications. Among these, alterations in skeletal muscle physiology, such as sarcopenia, are particularly significant, as they are associated with poor outcomes and reduced quality of life. Summary Various interventions, including pharmacological approaches and lifestyle modifications have been investigated to slow CKD progression and prevent or treat its complications. Physical exercise, in particular, has emerged as a promising intervention with multiple beneficial effects. These include improvements in physical functioning, increased muscle mass, modulation of metabolic abnormalities, and reduced cardiovascular risk. However, the pathophysiology of physical exercise in patients with kidney disease is complex and remains only partially understood. A crucial advancement in understanding this phenomenon has been the identification of myokines-molecules expressed and released by skeletal muscle in response to physical activity. These myokines can exert both paracrine and systemic effects, influencing not only skeletal muscle physiology but also other processes such as energy metabolism and lipid regulation. Key Messages The interplay among skeletal muscle, physical activity, and myokines may act as a pivotal regulator in various physiological processes, including aging, as well as in pathological conditions like cachexia and sarcopenia, frequently observed in CKD patients at different stages, including patients on dialysis. Despite the potential importance of this relationship, only a limited number of studies have explored the relationship between exercise and myokine, and the effect of this interaction on experimental models or individuals with kidney disease. In the following sections, we review and discuss this topic.

How Do Physical Activity and Exercise Affect Fabry Disease? Exploring a New Opportunity.

BACKGROUND: Fabry disease (FD) is a multisystem, monogenic, X-linked storage disorder caused by mutations in the GLA gene, resulting in reduced alfa-galactosidase A enzyme activity. This effect leads to the accumulation of glycosphingolipids, particularly globotriaosylceramide, in various tissues, including the heart, kidney, vasculature, smooth muscle, and peripheral nervous system. Hemizygous males are usually more severely affected than females, in whom random inactivation of an X chromosome may lead to variable phenotype. SUMMARY: Among the manifestations of FD, exercise intolerance is commonly diagnosed but often underestimated, even though it significantly limits quality of life, especially in young patients. This review primarily discusses the various pathophysiological mechanisms involved in exercise intolerance in FD patients, such as altered muscle composition, compromised cardiopulmonary framework, and peripheral neuropathy. Secondarily, it explores the potential effect of available therapy, including enzyme replacement therapy and chaperone therapy (migalastat), in reducing exercise intolerance while considering the potential impact of physical activity and exercise training as adjunctive treatments. CONCLUSION: Exercise intolerance has a major impact on the well-being of people with FD. Exercise training can play an important role in addition to drug therapy.

Evidence of Treatment Benefits in Patients with Mucopolysaccharidosis Type I-Hurler in Long-term Follow-up Using a New Magnetic Resonance Imaging Scoring System.

We developed a brain and spine magnetic resonance scoring system based on a magnetic resonance assessment of 9 patients with mucopolysaccharidosis type I-Hurler who underwent hematopoietic stem-cell transplantation. The score is reliable and correlates with long-term clinical and cognitive outcome in patients with mucopolysaccharidosis type I-Hurler.

Physical activity and exercise in chronic kidney disease: consensus statements from the Physical Exercise Working Group of the Italian Society of Nephrology.

The Italian Society of Nephrology has tasked its Working Group on Physical Exercise with developing a consensus statement document on physical activity and exercise in patients with chronic kidney disease (CKD). This paper consists of 16 points that were discussed, and approved using the mini-Delphi method by 15 members of the working group. Each statement is based on a comprehensive review of the literature, clinical experience, and expert opinions. Overall, the statements affirm that regular physical activity and exercise training offer numerous benefits to CKD patients, including improved physical function, enhanced cardiometabolic and neuromuscular function, cognitive benefits, and an overall improvement in quality of life. Furthermore, exercise may provide nephroprotection and reduce mortality. These advantages are observed across all CKD stages, whether on conservative therapy or kidney replacement therapy (hemodialysis or peritoneal dialysis), and in kidney transplant recipients. Moreover, when physical activity and exercise training are implemented with appropriate precautions, they are safe in CKD patients. Gradual physical activity and customized exercise programs should be tailored to the patient's exercise tolerance, potentially enhancing compliance. Clinicians are encouraged to use a series of questionnaires and tests to assess the patient's level of physical activity and performance. However, exercise and physical activity are poorly implemented in clinical practice due to many barriers related to patients and healthcare staff. Overcoming these barriers requires the proactive role of the nephrologists, who should actively incorporate exercise training and promote physical activity within routine care plans. Adopting a multidisciplinary team approach, which includes nephrologists, nurses, exercise professionals, and dietitians, is crucial for providing comprehensive rehabilitation for CKD patients. Integrating new technologies and remote check ups could further enhance the effectiveness of these interventions.