Clinical presentation of Churg-Strauss syndrome in children.

Churg-Strauss syndrome is an uncommon multisystem disorder characterized by asthma, eosinophilia and vasculitis. We report on a 12-year-old boy with asthma and deterioration of his general condition, who was eventually diagnosed with an ANCA-negative Churg-Strauss syndrome. The propositus included, 50 cases of childhood Churg-Strauss syndrome have been reported. The patient characteristics and clinical characteristics of these children are summarized. The respiratory tract is most frequently involved with pulmonary infiltrates, asthma and sinusitis. Early recognition of childhood Churg-Strauss syndrome is important as delayed diagnosis can lead to severe organ involvement, and possible fatal outcome

Gender differences in partners of patients with COPD and their perceptions about the patients

BACKGROUND/OBJECTIVES: Chronic obstructive pulmonary disease (COPD) not only affects patients but also their partners. Gender-related differences in patients with COPD are known, for instance regarding symptoms and quality of life. Yet, research regarding gender differences in partners of patients with COPD has been conducted to a lesser extent, and most research focused on female partners. We aimed to investigate differences between male and female partners of patients with COPD regarding their own characteristics and their perceptions of patients’ characteristics. DESIGN: Cross-sectional study. SETTING: Four hospitals in the Netherlands. PARTICIPANTS: One hundred and eighty-eight patient–partner couples were included in this cross-sectional study. MEASUREMENTS: General and clinical characteristics, health status, care dependency, symptoms of anxiety and depression, social support, caregiver burden, and coping styles were assessed during a home visit. RESULTS: Female partners had more symptoms of anxiety and a worse health status than male partners. Social support and caregiver burden were comparable, but coping styles differed between male and female partners. Female partners thought that male patients were less care dependent and had more symptoms of depression, while these gender differences did not exist in patients themselves. CONCLUSION: Health care providers should pay attention to the needs of all partners of patients with COPD, but female partners in particular. Obtaining an extensive overview of the patient–partner couple, including coping styles, health status, symptoms of anxiety, and caregiver burden, is necessary to be able to support the couple as effectively as possible

Changes in problematic activities of daily living in persons with COPD during 1 year of usual care

Introduction Persons with chronic obstructive pulmonary disease (COPD) are often limited in performing their activities of daily living (ADLs). However, it remains unknown whether and to what extent problematic ADLs change over time and whether exacerbation-related hospitalisations affect problematic ADLs. Therefore, we investigated self-reported problematic ADLs of persons with COPD during 1 year of usual care (i.e. without a specific experimental intervention). Methods Stable persons with moderate to very severe COPD (n = 137) were included in this longitudinal study (registered in the Dutch Trial Register [NTR 3941]). Participants were visited at home at baseline and after 1 year. Participants with an exacerbation-related hospitalisation during follow-up were visited additionally within 2 weeks after hospital discharge. During all visits, participants' personalised problematic ADLs were assessed using the Canadian Occupational Performance Measure (COPM), and perceived performance and satisfaction of important problematic ADLs were rated on a 10-point scale. Results In total, 90% of the participants reported at least one new important problematic ADL after 1 year. In the subgroup of participants with an exacerbation-related hospitalisation (n = 31), 92% of the participants reported new problematic ADLs 2 weeks after discharge and 90% reported new problematic ADLs again after 1 year. Only the satisfaction score of problematic ADLs as mentioned during baseline improved after 1-year follow-up in all participants (p = .002) and in participants without an exacerbation-related hospitalisation (n = 106; p = .014). Conclusion Problematic ADLs changed during 1 year of usual care, which underlines the need for regular assessment of problematic ADLs and referral to treatment options like monodisciplinary occupational therapy and/or a comprehensive pulmonary rehabilitation programme

Patient versus proxy-reported problematic activities of daily life in patients with COPD

Background and objective: Loved ones (proxies) of patients with COPD are confronted with the patients' limitations in activities of daily living (ADLs). However, it remains unknown whether proxies are able to correctly estimate the problematic ADLs of the patient. Therefore, we aimed to investigate the level of agreement between patient-reported and proxy-reported problematic ADLs of the patient.Methods: Stable outpatients with moderate to very severe COPD (n = 194) and their resident proxies (n = 194) were included in this cross-sectional study. Patients' problematic ADLs were assessed in the domains 'self-care', 'mobility', 'productivity' and ;'leisure' using the Canadian Occupational Performance Measure (COPM) in both patients and resident proxies. Furthermore, the perceived performance and satisfaction for important problematic ADLs were rated on a 10-point scale.Results: In total, 830 problematic ADLs were reported by patients, and 735 by proxies. Agreement in reporting problematic ADLs within a domain was poor (productivity and leisure; kappa = 0.20 and 0.16, respectively) to fair (self-care and mobility; kappa = 0.32 and 0.22, respectively). Similar performance and satisfaction scores, for equally reported problematic ADLs, were given by 24.0% and 17.6% of the pairs, respectively.Conclusion: Proxies were often not able to identify the patients' most important problematic ADLs. Moreover, when patient and proxy agreed about the presence of a specific problematic ADL, the perception of the performance and the satisfaction with that performance differed within most pairs. This emphasizes the importance of involving proxies, besides patients alone, in identifying patients' problematic ADLs.</p