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Background: Patients with Gaucher disease (GD), a rare lysosomal storage disorder, have reduced health-related quality of life (HRQOL). A patient-reported outcome measure (PROM) for HRQOL developed for type 1 GD (GD1) is not appropriate for patients with neuronopathic GD (nGD) types 2 (GD2) and 3 (GD3). In this study, we developed a new PROM for use in all GD types. We previously reported the qualitative analysis of interviews with Japanese patients with nGD, which was used to create nGD-specific PROM items. Here we evaluated the full PROM combining the type 1 questionnaire with the new nGD-specific items. Methods: Patients with confirmed GD were recruited (Association of Gaucher Disease Patients in Japan or leading doctors) for pre-testing (May 2021) or the main survey (October–December 2021). The PROM had three parts: Parts 1 and 2 were translated into Japanese from the pre-existing GD1 PROM, whereas Part 3 was newly developed. Patients (or their caregivers, where necessary) completed the PROM questionnaire on paper and returned it by mail. Mean scores were determined overall and by GD type. Inter-item correlations, content consistency (Cronbach's alpha), and test–retest reliability (Cohen's kappa; main survey only, taken 2 weeks apart) were calculated. Results: Sixteen patients (three with GD1; six with GD2; seven with GD3) and 33 patients (nine with GD1; 13 with GD2; 11 with GD3) participated in the pre-test and main survey, respectively. All GD2 patients and one-third (6/18) of GD3 patients required caregivers to complete the questionnaire. Mean scores indicated that the burden was highest in GD2 and lowest in GD1. In the main survey, internal consistency was high (Cronbach's alpha = 0.898 overall, 0.916 for Part 3), and test–retest reliability was high for Part 3 (kappa > 0.60 for 13/16 items) but low for Part 1 (kappa < 0.60 for 12/15 items). Conclusions: We have developed a flexible and reliable PROM that can be tailored for use in all types of GD and propose using Parts 1 and 2 for GD1, Parts 2 and 3 for GD2, and Parts 1, 2, and 3 for GD3. [ABSTRACT FROM AUTHOR]

Objective. On the basis of inheriting the classical aesthetic orientation of Japanese literature, contemporary Japanese literature changes the creative methods of traditional Japanese writers, strives to transcend the national limitations of Japanese literature, and strives to capture literary materials with a "modern feel." Combined with modern western literary techniques to express the "modern self," and integrating popular literature with pure literature, Japanese writers created a "healing" literature that is popular all over the world. There is some value in exploring the correlation between Japanese literature and the influence of psychotherapy. Method. In this paper, an equation diagnosis algorithm based on iterative learning for a class of linear discrete systems is studied, and the existing discrete iterative learning diagnostics are improved. We exchange the order of time domain and iterative domain to achieve the purpose of point-by-point equation diagnosis. On this basis, a virtual influence initial value estimation algorithm with a sliding window is proposed. The impact that has not been diagnosed is estimated and used as the initial value of the impact, so as to reduce the number of iterations and improve the efficiency of equation diagnosis. This paper takes the public and readers as different perspectives, based on the use and satisfaction theory and prospect theory, and uses interviews and questionnaires to study the communication effect of Japanese literature from the three dimensions of cognition, emotion, and behavior. The cognitive part includes the public's understanding and readers' understanding and reading; the emotional part includes the public's and readers' impressions and emotional attitudes; the behavioral part includes the public's reading situation and readers' behavior changes. Results/Discussion. In the survey of loyal readers, although readers' understanding of Japanese literature has improved compared with the general public, it is not high, and their liking and influence on behavior are slightly lower than the preset level. This paper believes that the existing problems of Japanese literature dissemination are mainly concentrated in three aspects: the market is somewhat biased, which narrows the cognitive scope of users; as a cross-cultural literary work, Japanese literature still makes readers feel that it exists; there is some sense of estrangement, which will affect their enjoyment in reading; as a niche interest, readers lack timely communication and sharing objects after reading, so it is difficult to enhance their sense of identity. [ABSTRACT FROM AUTHOR]