From the Guidelines
Congenital muscular dystrophy (CMD) has a limited direct relationship with the fibrinolysis process, but certain subtypes and secondary effects may influence local fibrinolytic activity. Fibrinolysis, the process that dissolves blood clots, is not typically considered a primary mechanism in CMD pathophysiology. However, certain CMD subtypes, particularly those with basement membrane defects like merosin-deficient CMD (MDC1A), may have secondary effects on vascular integrity that could influence local fibrinolytic activity. The extracellular matrix proteins affected in some forms of CMD, such as laminin-α2, interact with cellular components that may indirectly affect clotting and fibrinolysis pathways.
Some studies have investigated the coagulation and fibrinolysis processes in relation to cardiac diseases, but their direct relevance to CMD is limited 1. For instance, a study on the prevention and treatment of thrombosis in pediatric and congenital heart disease found increased fibrinolysis in cyanotic CHD, as indicated by increased levels of d-dimers 1. However, this finding is not directly applicable to CMD.
Another study on the management of cardiac involvement associated with neuromuscular diseases, including congenital myopathies, highlights the complexity of muscle disorders and their varying effects on cardiac function 1. While this study provides valuable insights into the cardiac aspects of neuromuscular diseases, it does not specifically address the relationship between CMD and fibrinolysis.
Key points to consider in the relationship between CMD and fibrinolysis include:
- Secondary effects of certain CMD subtypes on vascular integrity and local fibrinolytic activity
- Potential alterations in coagulation parameters, including fibrinolytic activity, due to chronic inflammation in muscular dystrophies
- Increased thrombosis risk in severe CMD cases due to immobility, necessitating consideration of fibrinolytic status
- The importance of awareness among clinicians regarding potential coagulation abnormalities when treating CMD patients, especially during surgical interventions or periods of reduced mobility.
In clinical practice, it is essential to consider the potential for coagulation abnormalities, including alterations in fibrinolytic activity, when managing patients with CMD, particularly during periods of reduced mobility or surgical interventions. However, the current evidence does not support a direct and significant relationship between CMD and the fibrinolysis process, and further research is needed to fully understand this potential connection 1.
From the Research
Relationship between Congenital Muscular Dystrophy (CMD) and Fibrinolysis Process
- The relationship between Congenital Muscular Dystrophy (CMD) and the fibrinolysis process is not directly addressed in most of the studies provided 2, 3, 4, 5.
- However, one study 6 investigated coagulation and fibrinolysis disorders in muscular dystrophy, including Fukuyama congenital muscular dystrophy (FCMD), a subtype of CMD.
- The study found that fibrin and fibrinogen degradation products (FDP) levels were elevated in patients with FCMD, suggesting enhanced coagulation and fibrinolysis associated with muscle degeneration 6.
- The elevated FDP levels in FCMD patients significantly correlated with serum levels of the MM isozyme of creatine kinase (CK-MM), indicating a relationship between muscle damage and fibrinolysis 6.
- While this study provides some insight into the relationship between CMD and fibrinolysis, more research is needed to fully understand this connection.