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Abstract Background Chronic thromboembolic pulmonary hypertension (CTEPH) is associated with proximal pulmonary artery obstruction and vascular remodeling. We hypothesized that pulmonary arterial smooth muscle (PASMC) and endothelial cells (PAEC) may actively contribute to remodeling of the proximal pulmonary vascular wall in CTEPH. Our present objective was to characterize PASMC and PAEC from large arteries of CTEPH patients and investigate their potential involvement in vascular remodeling. Methods Primary cultures of proximal PAEC and PASMC from patients with CTEPH, with non-thromboembolic pulmonary hypertension (PH) and lung donors have been established. PAEC and PASMC have been characterized by immunofluorescence using specific markers. Expression of smooth muscle specific markers within the pulmonary vascular wall has been studied by immunofluorescence and Western blotting. Mitogenic activity and migratory capacity of PASMC and PAEC have been investigated in vitro. Results PAEC express CD31 on their surface, von Willebrand factor in Weibel-Palade bodies and take up acetylated LDL. PASMC express various differentiation markers including α-smooth muscle actin (α-SMA), desmin and smooth muscle myosin heavy chain (SMMHC). In vascular tissue from CTEPH and non-thromboembolic PH patients, expression of α-SMA and desmin is down-regulated compared to lung donors; desmin expression is also down-regulated in vascular tissue from CTEPH compared to non-thromboembolic PH patients. A low proportion of α-SMA positive cells express desmin and SMMHC in the neointima of proximal pulmonary arteries from CTEPH patients. Serum-induced mitogenic activity of PAEC and PASMC, as well as migratory capacity of PASMC, were increased in CTEPH only. Conclusions Modified proliferative and/or migratory responses of PASMC and PAEC in vitro, associated to a proliferative phenotype of PASMC suggest that PASMC and PAEC could contribute to proximal vascular remodeling in CTEPH.

Abstract Background Fibromyalgia is a chronic syndrome with no cure. A thorough understanding of the illness experience is therefore key in the palliative care of patients with this condition. In search for supportive treatments fibromyalgia patients often attend a chiropractor or other manual therapist. Knowledge of the meaning and reality of living with this condition to the patient could be considered essential to any health care practitioner playing a role in the management. This study aimed to gain a better understanding of the subjective experience of fibromyalgia, focusing on the personal, occupational and social impact of the condition on patients' lives. This included exploring the patients' views about the future. Methods This study employed descriptive phenomenology and adopted Husserl's concept of transcendental subjectivity or "bracketing". This qualitative study involved semi-structured interviews and was undertaken to obtain rich data that reflected the essence of the participants' experience. Participants consisted of six female volunteers, diagnosed with fibromyalgia by the University Hospital Gent, Belgium. Data were analysed using a thematic framework. Results Fibromyalgia pervaded all aspects of life. Four main themes arose from data analysis, namely; the impact of fibromyalgia on patients' occupational and personal life, the impact on their future and aspects of social interaction. Nearly all participants had stopped working, giving rise to feelings of uselessness and loss of identity. Leisure activities were also greatly affected. Fibromyalgia was said to alter family bonds, some of which were reinforced, others were broken. The diagnosis was seen as a relief, marking an end to a period of uncertainty. Participants reported ambivalence in interaction. Despite some positive encounters, frustration arising from perceived incomprehension dominated. Consequently patients preferred not to share their experiences. Conclusions The study revealed the negative impact of fibromyalgia on patients' lives as comprising of great complexity and individuality. Several implications for health care practitioners can be extrapolated, including the need of a more efficient diagnostic process and increased education about the fibromyalgia experience. Further studies are required to better clarify the multifaceted nature of living with the condition.