Background: Lipoedema is a chronic disorder of adipose tissue typically involving an abnormal build-up of fat cells in the legs, thighs and buttocks. Occurring almost exclusively in women, it often co-exists with obesity. Due to an absence of clear objective diagnostic criteria, lipoedema is frequently misdiagnosed as obesity, lymphoedema or a combination of both. The purpose of this observational study was to compare muscle strength and exercise capacity in patients with lipoedema and obesity, and to use the findings to help distinguish between lipoedema and obesity. Design: This cross-sectional, comparative pilot study performed in the Dutch Expertise Centre of Lymphovascular Medicine, Drachten, a secondary-care facility, included 44 women aged 18 years or older with lipoedema and obesity. Twenty-two women with lipoedema (diagnosed according the criteria of Wold et al, 1951) and 22 women with body mass index ≥30kg/m2 (obesity) were include in the study. No interventions were undertaken as part of the study. Results: Muscle strength of the quadriceps was measured with the MicroFET™, and functional exercise capacity was measured with the 6-minute walk test. The group with lipoedema had, for both legs, significantly lower muscle strength (left: 259.9 Newtons [N]; right: 269.7 N; p < 0.001) than the group with obesity. The group with lipoedema had a non-significant, but clinically relevant lower exercise-endurance capacity (494.1±116.0 metres) than the group with obesity (523.9±62.9 metres; p=0.296). Conclusions: Patients with lipoedema exhibit muscle weakness in the quadriceps. This finding provides a potential new criterion for differentiating lipoedema from obesity. We recommend adding measuring of muscle strength and physical endurance to create an extra diagnostic parameter when assessing for lipoedema.
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Subcutaneous emphysema, pneumothorax and pneumomediastinum are well-known complications of invasive ventilation in patients with acute hypoxemic respiratory failure. We determined the incidences of air leaks that were visible on available chest images in a cohort of critically ill patients with acute hypoxemic respiratory failure due to coronavirus disease of 2019 (COVID-19) in a single-center cohort in the Netherlands. A total of 712 chest images from 154 patients were re-evaluated by a multidisciplinary team of independent assessors; there was a median of three (2–5) chest radiographs and a median of one (1–2) chest CT scans per patient. The incidences of subcutaneous emphysema, pneumothoraxes and pneumomediastinum present in 13 patients (8.4%) were 4.5%, 4.5%, and 3.9%. The median first day of the presence of an air leak was 18 (2–21) days after arrival in the ICU and 18 (9–22)days after the start of invasive ventilation. We conclude that the incidence of air leaks was high in this cohort of COVID-19 patients, but it was fairly comparable with what was previously reported in patients with acute hypoxemic respiratory failure in the pre-COVID-19 era.
Introduction: Throughout life, a patient with severe haemophilia is confronted with many treatment-related challenges. Insight into self-management and non-adherence could improve the quality of care for these patients. The aim of this study was to provide an overview of the current evidence on self-management and adherence to prophylaxis in haemophilia. Method: Based on series of studies and published literature, aspects of treatment were explored: learning and performing self-infusion, achieving self-management skills in adolescence, adherence issues and coping with haemophilia. Evidence-based and age-group-specific recommendations for haemophilia professionals were formulated. Results: Nearly, all severe haemophilia patients and parents were able to perform self-infusion and the quality level of infusion skills was acceptable. Learning self-infusion was generally initiated before the onset of puberty and full self-management was obtained 10 years later. Adherence was defined using a Delphi consensus procedure and was determined by skipping, dosing and timing of infusions. Adherence levels varied according to age, with highest levels in children (1–12 years) and the lowest among 25–40 years. Adherence to prophylaxis was acceptable (43%), yet 57% of the population struggled with prophylaxis. Qualitative research showed that the position of prophylaxis in life is the main driver of adherence. This position is influenced by acceptance and self-management skills. Regarding coping with haemophilia, the majority of patients used a problem-focused approach. Conclusion: Self-management and adherence to prophylaxis vary during the life span. Acceptance of the disease and self-management skills were important aspects that may require tailored professional support.
