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Objectives: The MetabQoL 1.0 is the first disease-specific health related quality of life (HrQoL) questionnaire for patients with intoxication-type inherited metabolic disorders. Our aim was to assess the validity and reliability of the MetabQoL 1.0, and to investigate neuropsychiatric burden in our patient population. Methods: Data from 29 patients followed at a single center, aged between 8 and 18 years with the diagnosis of methylmalonic acidemia (MMA), propionic acidemia (PA) or isovaleric acidemia (IVA), and their parents were included. The Pediatric Quality of Life Inventory (PedsQoL) was used to evaluate the validity and reliability of MetabQoL 1.0.
Results: The MetabQoL 1.0 was shown to be valid and reliable (Cronbach's alpha: 0.64–0.9). Fourteen out of the 22 patients (63.6%) formally evaluated had neurological findings. Of note, 17 out of 20 patients (85%) had a psychiatric disorder when evaluated formally by a child and adolescent psychiatrist. The median mental scores of the MetabQoL 1.0 proxy report were significantly higher than those of the self report (p = 0.023). Patients with neonatal-onset disease had higher MetabQoL 1.0 proxy physical (p = 0.008), mental (p = 0.042), total scores (p = 0.022); and self report social (p = 0.007) and total scores (p = 0.043) than those with later onset disease.
Conclusions: This study continues to prove that the MetabQoL 1.0 is an effective tool to measure what matters in intoxication-type inherited metabolic disorders. Our results highlight the importance of clinical assessment complemented by patient reported outcomes which further expands the evaluation toolbox of inherited metabolic diseases.
Zusammenfassung: Eine umfassende Literaturrecherche (Comprehensive Literature Search, CLS) bezeichnet eine umfangreiche und aufwändige Recherche mit dem Ziel, möglichst alle Studien zur Beantwortung einer zugrundeliegenden Forschungsfrage im Rahmen einer systematischen Übersichtsarbeit zu identifizieren. Konkrete Empfehlungen oder eine Definition des Begriffs CLS gibt es jedoch nicht. Der vorliegende Beitrag führt in eine Rahmung der CLS ein, stellt die damit einhergehenden Herausforderungen dar und weist auf methodische Implikationen sowie mögliche zukünftige Forschungsbereiche hin.
The Convention on the Rights of the Child (CRC) is a human rights framework in the context of multi-level governance child protection policies central to social work education and practice (United Nations, 1989). In line with this statement, children’s rights-based education introduces undergraduate social work students to the principles of the CRC, namely participation, protection, harm prevention and provision, to facilitate knowledge acquisition by building core competencies for critical practice (IFSW, 2002). It equips social workers with analytical and advocacy skills that foster critical thinking and creativity in the juxtaposition between child protection, autonomy and self-determination.
This chapter provides insights for social work education to locate and analyse the underlying casualties of social problems using a problem and resource framework, the w-questions (Geiser, 2015). The framework is used to develop theory driven social work interventions as illustrated against the backdrop the anonymised case study, Amira, an accompanied child asylum seeker in Austria (Fritsche, Glawischnig, & Wolfsegg, 2019). Correspondingly, CRC is addressed along a continuum between human needs fulfilment and human right entitlements (Obrecht, 2009; IFSW, 2002; Ife, 2012). The concept of need is understood as tension in our concrete biological and psychological bio-values and states (Obrecht, 2009, p. 27). The assertion is that when children lack support or are obstructed from achieving their equal right to education due to social, cultural or economic barriers, this exacerbates social marginalisation because it deprives them of membership in the school social system. Social marginalisation thwarts the fulfilment of needs and weakens social cohesion by causing alienation and anomie (Mayrhofer, 2015). The tentative conclusion is that knowledge and practice models that link human needs and children’s rights equip social workers with the expertise to reduce children’s vulnerability whilst strengthening their protection, autonomy and self-determination.
Organic acidurias (OAs), urea-cycle disorders (UCDs), and maple syrup urine disease (MSUD) belong to the category of intoxication-type inborn errors of metabolism (IT-IEM). Liver transplantation (LTx) is increasingly utilized in IT-IEM. However, its impact has been mainly focused on clinical outcome measures and rarely on health-related quality of life (HRQoL). Aim of the study was to investigate the impact of LTx on HrQoL in IT-IEMs. This single center prospective study involved 32 patients (15 OA, 11 UCD, 6 MSUD; median age at LTx 3.0 years, range 0.8–26.0). HRQoL was assessed pre/post transplantation by PedsQL-General Module 4.0 and by MetabQoL 1.0, a specifically designed tool for IT-IEM. PedsQL highlighted significant post-LTx improvements in total and physical functioning in both patients' and parents' scores. According to age at transplantation (≤3 vs. >3 years), younger patients showed higher post-LTx scores on Physical (p = 0.03), Social (p < 0.001), and Total (p =0.007) functioning. MetabQoL confirmed significant post-LTx changes in Total and Physical functioning in both patients and parents scores (p ≤ 0.009). Differently from PedsQL, MetabQoL Mental (patients p = 0.013, parents p = 0.03) and Social scores (patients p = 0.02, parents p = 0.012) were significantly higher post-LTx. Significant improvements (p = 0.001–0.04) were also detected both in self- and proxy-reports for almost all MetabQoL subscales. This study shows the importance of assessing the impact of transplantation on HrQoL, a meaningful outcome reflecting patients' wellbeing. LTx is associated with significant improvements of HrQol in both self- and parentreports. The comparison between PedsQL-GM and MetabQoL highlighted that MetabQoL demonstrated higher sensitivity in the assessment of diseasespecific domains than the generic PedsQL tool.