AimIn case of extremely rare diseases, case reports are often the only experience to draw from for evidence-based management. Carmi syndrome is a rare, mostly lethal combination of junctional epidermolysis bullosa and pyloric atresia. During an ethical board, there were differences in perception of mortality rate. We tested the hypothesis that the cumulative mortality of single case reports is lower than that of multiple case series.CaseA baby girl was born at 33 weeks gestation with Carmi syndrome. The treatment options discussed (...) in an interdisciplinary ethics board were a palliative approach versus surgical gastrojejunostomy. Because about one third of operated children described in case reports survived, we opted for surgical treatment. The patient died a painful death 4 weeks later.MethodsThe PubMed database was systematically searched for reports of Carmi syndrome. Single case reports were compared to case series in terms of outcome.ResultsA total of 102 cases of Carmi syndrome were identified in the literature. Mortality of single case reports was 17 out of 27 patients (63%), while that of case series was higher at 62 out of 74 patients (84%,p = 0.036).ConclusionsSelection and publication bias may lead to inflation of survival rates in single case reports because successful cases are more likely to be published in the literature. These biases may lead to inappropriately aggressive treatment in futile cases. Clinicians should be cautious when discussing prognosis and making decisions based on the cumulative experience of case reports of extremely rare or novel diseases. (shrink)

v. 1. Sŏngnihak pʻyŏn -- v. 2. Kyŏngse sasang pʻyŏn.

Purpose. Based on tracing dualistic tendencies in the history of the concept “self” formation, the paper aims to clarify in what way dualism – contradistinction of the self and sociality, in particular – is being overcome in phenomenological and hermeneutic approaches to the self. Methodology. The systematic and integrative approaches, hermeneutic, phenomenological and retrospective methods, comparative analysis, description and synthesis underlie the research conducted in this paper. Theoretical basis. The development of the concept “self” is traced based on historical retrospective (...) of its definitions. The paper shows the influence of Kantian interpretation of the self upon the contemporary approaches, presents the comparative analysis of the most recent definitions of the self and illustrates the study results on the relation of the self to the body, the world, the narration and the identity with reference to Martin Heidegger, Paul Ricoeur and Charles Taylor in particular. Originality. An integration of phenomenological and hermeneutic approaches to the self is introduced in the paper. It is argued that phenomenological and hermeneutic interpretations can be represented as complementing each other, since they share the anti-dualist perspective and interweave in description of the key aspects of the self, particularly sociality and language. Conclusion. Dualism is overcome in the contemporary interpretations of the self as it is clear from the phenomenological and hermeneutic approaches example. There is no more acute opposition between the self and the world, the self and the body, the self and the social life of a person. The self is considered as “being-in-the-world” which unfolds through intersection of different dimensions: subjectivity, body, temporality, language and sociality. Sociality provides the self with a moral framework through identity and enables conditions for person’s self-fulfillment, since the self reaches completeness only by transcending itself towards the others. The modern discovery of the self entails the need to invent a new type of solidarity. (shrink)