A 25-year-old woman with Hallopeau-Siemens recessive dystrophic epidermolysis bullosa had generalized blistering, scarring and milia since birth. In the course of the disease, acral pseudosyndactyly developed, and the patient suffered from corneal erosions, oesophageal strictures, malabsorption, recurrent severe pneumonias and nephrotic syndrome. In addition, she had severe anaemia, sideropaenia, hypocalcaemia, heavy proteinuria and hypoalbuminaemia. A rapidly growing skin squamous cell carcinoma developed on the neck that spread to axillary and cervical lymph nodes. Recurrent hypocalcaemic tetanic convulsions and dyspnoea and a pneumonia refractory to antibiotics led to the premature demise of the patient. Autopsy revealed extensive amyloidosis of the renal, hepatic and splenic tissues. AA type amyloid deposits were detected in the renal glomeruli and in the lung, explaining the patient's unusually severe pulmonary infections. In essence, the patient had severe recessive dystrophic epidermolysis bullosa, complicated by squamous cell carcinoma, recurrent pneumonias and nephrotic syndrome due to secondary amyloidosis of the kidney and lung. The possibility of secondary pulmonary amyloidosis should be considered in severe dystrophic epidermolysis bullosa patients with recurrent pulmonary infections.
High recurrence of the splice-site mutation 425A-->G in central European patients with DEB should be taken into account when designing COL7A1 mutation detection strategies. Reporting of three novel COL7A1 mutations in this study further emphasizes the molecular heterogeneity of DEB and provides more information for studies on genotype-phenotype correlations in different DEB subtypes.
Introduction Most people experience grief-related symptoms after losing a loved one. Approximately 9.8% of bereaved individuals’ symptoms persist over the first year post-loss, emphasizing the importance of research in prolonged grief. The role of self-conscious emotions in prolonged grief, such as self-compassion, state shame, guilt and pride has been proposed in previous studies. Objectives Our aim was to examine the mediating role of state shame, guilt and pride in the relationship between self-compassion and prolonged grief. Methods This cross-sectional study collected data via online questionnaires based on self-reports (N=177, mean age: 42.26 years [SD=12.32], 97.2% women). Demographic and loss-related variables were measured, and further instruments assessed levels of self-compassion, state shame, guilt, and pride, and prolonged grief. Correlation and mediation analyses were used. Results Two significant indirect effects were shown in the mediation model. First, lower levels of self-compassion predicted higher levels of state shame, which in turn predicted elevated levels of prolonged grief. Second, higher levels of self-compassion predicted higher levels of pride, which subsequently contributed to lower levels of prolonged grief. Guilt did not have a significant mediating role. Conclusions The results highlight the possible role of elevated levels of state shame and lower levels of self-compassion and state pride in the development of prolonged grief. It is important for researchers and clinicians to be attentive to the role of self-compassion, state shame and pride in grieving. Disclosure No significant relationships.
Összefoglaló. Bevezetés: A coeliakia és a kapcsolódó terhek befolyásolják az érintettek életminőségét. A krónikus betegségekkel járó stigmatizáció hozzájárul a fizikai tünetek és a lelki panaszok fokozódásához, valamint az egészségmagatartás csökkenéséhez. Mindez szükségessé teszi a krónikus megbetegedések kapcsán a stigmatizáció felismerését, felmérését és kezelését. Célkitűzések: Fő célunk a 8 tételes Stigmatizáció Krónikus Betegségekben Kérdőív (SSCI-8) magyar adaptációja és pszichometriai vizsgálata volt coeliakiások körében. További célunk volt megvizsgálni a szégyen közvetítő szerepét a stigmatizáció és a jóllét között. Módszerek: A kutatás önbeszámolós, online kérdőíves, keresztmetszeti vizsgálatként zajlott (n = 85, átlagéletkor: 37,64, 91,8% nő). Az SSCI-8 mellett felvételre kerültek a szégyenélményt, a coeliakiás életminőséget, a jóllétet mérő kérdőívek. A kutatási célok tesztelése megerősítő faktorelemzéssel, korrelációs és mediációs elemzésekkel történt. Eredmények: Az SSCI-8 faktorelemzése során az egydimenziós modell megfelelő illeszkedést mutatott magas belső konzisztenciaértékek mellett. A mediációs modellek szerint a magasabb stigmatizáció a magasabb szégyenélményen keresztül járulhat hozzá a pszichés jóllét (coeliakiás életminőség, jóllét) csökkenéséhez. Megbeszélés: Az SSCI-8 rövid, átfogó kérdőívként megbízhatóan és érvényesen mérte a krónikus betegségben tapasztalt stigmatizációt a jelen coeliakiás mintán. Eredményeink alátámasztják, hogy a stigmatizáció és a szégyen fontos szerepet tölt be a jóllét csökkenésében. Következtetés: A nemzetközileg széles körben alkalmazott SSCI-8 adaptálására került sor, mely hazai kutatásokban és az egészségügyi ellátásban is hasznos mérőeszköz lehet. Az eredmények rávilágítanak, hogy a stigmatizáció és a szégyenélmény további kutatása szükséges, különösen a hatékony pszichoszociális intervenciók kifejlesztését megcélozva. Hatékony pszichológiai segítség révén a szégyen és a stigmatizáltság mérséklésével javulhat a coeliakiával élők fizikai és lelki állapota, ami a gluténmentes diéta betartása révén hozzájárulhat a betegség okozta tünetek és szövődmények mérsékléséhez. Orv Hetil. 2021; 162(49): 1968–1976. Summary. Introduction: Coeliac disease can detrimentally affect well-being. Stigmatization related to a chronic disease can enhance physical and psychological symptoms and negatively influence health behaviour, hence, stigma in chronic diseases needs to be addressed. Objectives: Our main goal was to psychometrically evaluate the Hungarian adaptation of the Stigma Scale for Chronic Illness-8 (SSCI-8). Further aim was to examine the mediating role of shame on the relationship between stigmatization and well-being aspects among individuals with coeliac disease. Methods: This cross-sectional study collected data using online questionnaires based on self-reports (n = 85, mean age: 37.64 years, 91.8% women). Instruments assessed levels of stigmatization, shame experience, quality of life in coeliac disease and well-being. Confirmatory factor, correlation and mediation analyses were used. Results: Factor analysis showed adequate fit for a unidimensional model with high internal consistency. Mediation models showed that higher levels of stigmatization can contribute to decreased levels of quality of life in coeliac disease and well-being via increased levels of shame. Discussion: The SSCI-8 is a short, valid, reliable instrument measuring stigmatization in the current sample of people with coeliac disease. The results highlight the role of stigma and shame in the decrease of well-being. Conclusion: The adapted version of the SSCI-8 can be a useful tool in Hungarian research and healthcare. The results suggest that stigmatization and shame need further attention to develop effective intervention which can reduce their effect and enhance adherence to gluten-free diet and improve physical and psychological well-being. Orv Hetil. 2021; 162(49): 1968–1976.
ObjectivesThe current global health crisis of the COVID-19 pandemic has drastically affected the whole population, but healthcare workers are particularly exposed to high levels of physical and mental stress. This enormous burden requires both the continuous monitoring of their health conditions and research into various protective factors.DesignCross-sectional surveys.Setting and participantsSelf-administered questionnaires were constructed assessing COVID-19-related worries of health workers in Hungary. The surveys were conducted during two consecutive waves of the COVID-19 pandemic (N-first wave=376, N-second wave=406), between 17 July 2020 and 31 December 2020.Primary and secondary outcome measuresCOVID-19-related worry, well-being and distress levels of healthcare workers. We also tested whether psychological resilience mediates the association of worry with well-being and distress. Multiple linear regression analyses were performed.ResultsThe results indicated that healthcare workers had high levels of worry and distress in both pandemic waves. When comparing the two waves, enhanced levels of worry (Wald’s χ2=4.36, p=0.04) and distress (Wald’s χ2=25.18, p<0.001), as well as compromised well-being (Wald’s χ2=58.64, p<0.001), were found in the second wave. However, not all types of worries worsened to the same extent across the waves drawing attention to some specific COVID-19-sensitive concerns. Finally, the protective role of psychological resilience was shown by a mediator analysis suggesting the importance of increasing resilience as a key factor in maintaining the mental health of healthcare workers in the burden of the COVID-19 pandemic.ConclusionsOur results render the need for regular psychological surveillance in healthcare workers.RegistrationHungarian Scientific and Research Ethics Committee of the Medical Research Council (IV/5079-2/2020/EKU).
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