Respiration in myotonic dystrophy: characteristics and therapeutic interventions

Myotonic Dystrophy (DM1) is a high-burden progressive neuromuscular disorder in which all organs can be involved in a various way. Progressive respiratory muscle weakness is the main reason for life-threatening complications like pneumonia and respiratory failure, which are the number causes of premature death. In this thesis we analysed body composition, lung volumes, effects of home mechanical ventilation and inspiratory muscle training as a new treatment option in DM1. We found in a study of 105 DM1 patients that body composition was abnormal in nearly all patients. Overweight is present in more than half of DM1 patients, which is combined with either decreased muscle mass or normal muscle mass. Lung volumes are mainly reduced due to respiratory muscle weakness, whereas this thesis showed that the presence of overweight further decreases lung volumes. In case of chronic respiratory failure patients can start home mechanical ventilation (HMV), which restore the disturbed carbon dioxide levels. We found that DM1 patients uses their treatment adequately and survival improves when HMV is for used at least 5h/night. A new treatment option could be inspiratory muscle training, which impressive improved respiratory muscle strength (+109%) and respiratory duration capacity (+129%) after 12 weeks of training in a pilot study of 10 patients. In one additional case report we found that carbon dioxide levels reduced after training. Future studies should explore whether IMT can delay the onset of respiratory failure and could become a complementary treatment with HMV in DM1

Laboratorium onderzoek van omhullingsmaterialen voor drainbuizen

Het doel van het onderzoek was: - Het ontwikkelen van een relatie tussen karakteristieke eigenschappen van omhullingsmaterialen voor drainbuizen (zoals poriëngrootteverdeling en -dikte) enerzijds en de korrelgrootteverdeling van het omgevend bodemmateriaal anderzijds; - Het ontwerpen van eenvoudige standaardmethoden ter bepaling van die relevante karakteristieke eigenschappen, welke resultaten opleveren voor de beoordeling van toepassingsmogelijkheden in de praktijk; - Het formuleren van kwaliteitseisen voor synthetische omhullingsmaterialen. De voor dit onderzoek verrichte werkzaamheden zijn: - Bepaling van de poriëngrootteverdeling van de omhullingsmaterialen krammat, polypropyleen en kokos;- Diktemetingen van omhullingsmaterialen; - Doorstromingsproeven