The research presented in this thesis provides several novel insights regarding the _-thalassemia intermedia phenotype. Earlier studies observed that patients with _-thalassemia intermedia... Show moreThe research presented in this thesis provides several novel insights regarding the _-thalassemia intermedia phenotype. Earlier studies observed that patients with _-thalassemia intermedia experience a clinical complications profile that is different from that in patients with _-thalassemia major; which was primarily attributed to their transfusion-independence. In this work, a variety of clinical morbidities were explored and their associations with the underlying disease pathophysiology and risk factors were examined. The morbidities evaluated throughout the studies involved several organs and organ systems including the vasculature (venous thrombosis, pulmonary artery hypertension, cerebrovascular disease, and leg ulcers), heart, liver, kidney, endocrine glands (diabetes mellitus, hypothyroidism, and hypogonadism), bone (osteoporosis), and the hematopoietic system (extramedullary hematopoietic tumors). Findi ngs confirm that _-thalassemia intermedia should no longer be regarded as a mild form of thalassemia as patients experience serious manifestations involving almost every organ system. Show less
The general aim of this thesis was to assess the effectiveness of a standardized psychosocial education program. The first studies of this thesis were aimed to assess the effectiveness of the... Show moreThe general aim of this thesis was to assess the effectiveness of a standardized psychosocial education program. The first studies of this thesis were aimed to assess the effectiveness of the Patient Education Program for Parkinson__s disease (PEPP), as the original program was directed at Parkinson__s disease. Thereafter, it was aimed to assess the program__s feasibility in other diseases. A second step in the thesis was an evaluation of the feasibility of the program adapted to a new disease-specific form for Huntington__s disease (PEP-HD). The third step was to evaluate a generic form of the program in a heterogeneous group of patients with chronic diseases and co-morbid psychiatric problems (PEP-CD). Show less
Treatment of oral and oropharyngeal cancer may cause dysphagia. Purpose is to examine whether cine magnetic resonance imaging (MRI) yields additional information compared to standard examination in... Show moreTreatment of oral and oropharyngeal cancer may cause dysphagia. Purpose is to examine whether cine magnetic resonance imaging (MRI) yields additional information compared to standard examination in the evaluation of posttreatment dysphagia and mobility of oral and oropharyngeal structures. Thirty-four cine MRIs were made in 23 patients with advanced oral and oropharyngeal cancer, consisting of an MR image every 800 ms during swallowing which is compared to videofluoroscopy and quality of life questionnaires. A scoring system was applied to assess mobility on cine MR and videofluoroscopy leading to a score ranging from 9 to 17. Cine MRI of the swallowing in a midsagittal plane visualized the tumor (if located in the same plane), important anatomic structures and surgical reconstructions. Posttreatment mobility on cine MRI and videofluoroscopy was significantly diminished compared to pretreatment, mean pretreatment cine MRI score was 10.8 and posttreatment 12.4 ( = 0.017). Impaired mobility on cine MRI was significantly correlated to more swallowing problems (Spearman's correlation coefficient 0.73, = 0.04), on videofluoroscopy not. Cine MRI is a promising new technique as an adjunct to standard examinations for evaluation of swallowing in patients with oral and oropharyngeal cancer. Cine MRI directly visualizes the dynamics of swallowing and allows evaluation of pre- and posttreatment differences. Abnormal findings are significantly correlated with subjective swallowing complaints of patients. Show less
Nowadays, pituitary adenomas can be appropriately treated, but patients continue to report impaired quality of life (QoL) despite long-term remission or cure. In patients with Cushing__s disease,... Show moreNowadays, pituitary adenomas can be appropriately treated, but patients continue to report impaired quality of life (QoL) despite long-term remission or cure. In patients with Cushing__s disease, Cushing__s syndrome or acromegaly, doctors should be aware of subtle cognitive impairments and the increased prevalence of psychopathology and maladaptive personality traits after long-term remission. In addition, these patients use ineffective coping strategies and have negative illness perceptions that are not always correct. Patients are labeled __cured__, but still experience chronic and persisting impairments due to their pituitary disease. Doctors should consider informing patients better about the long-term consequences and act as a guide in this __acceptance process__ of the patient. In addition, a multi-disciplinary team of endocrinologists, psychologists, and community health workers might also help patients in accepting and dealing with the consequences of their pituitary disease. Show less
