ECFS standards of care on CFTR-related disorders: Updated diagnostic criteria

This paper is the first in a series providing updated guidance on the definition, evaluation and management of people with a Cystic Fibrosis Transmembrane conductance Regulator (CFTR)-Related Disorder (CFTR-RD). The need for this update relates to more precise characterisation of CFTR gene variants and improved assessment of CFTR protein dysfunction. The exercise is co-ordinated by the European CF Society Standards of Care Committee and Diagnostic Network Working Group and involves stakeholder engagement. This first paper was produced by a core group using an extensive literature review and papers graded for their quality. Subsequent wider stakeholder agreement was achieved.The definition of a CFTR-RD remains “a clinical condition with evidence of CFTR protein dysfunction that does not fulfil the diagnostic criteria for CF”. Clearer guidance on CFTR dysfunction and relevant CFTR variants will be provided. Thresholds for clinical presentations are presented and the paradigm that pathobiological processes may be evident in more than one organ is agreed. In this paper we reflect on the early patient journey, highlighting that CF specialists as well as other relevant specialists should be involved in the care of people with a CFTR-RD.     

Malnutrition Diagnosis during Adult Inpatient Hospitalizations: Analysis of a Multi-Institutional Collaborative Database of Academic Medical Centers

Background

Malnutrition is a significant problem for hospitalized patients. However, the true prevalence of reported malnutrition diagnosis in real-world clinical practice is largely unknown. Using a large collaborative multi-institutional database, the rate of malnutrition diagnosis was assessed and used to assess institutional variables associated with higher rates of malnutrition diagnosis.

Study on self-consciousness of children with learning disabilities and related factors

To study the self-consciousness of children with learning disabilities (LD) and to identify related factors. Methods Five hundred and sixty pupils graded from 1 to 6 in an elementary school were investigated. According to the pupil rating scale revised screening for learning disabilities (PRS), combined Raven‘s test (CRT) and achievement of main courses, 35 of 560 pupils were diagnosed as LD children. Thirty-five children were selected from the average children and 35 from advanced children in academic achievement equally matched in class, gender, and age with LD children as control groups. The three groups were tested by Piers-Harris children‘s self-concept scale. Basic information of each subject was collected by self-made questionnaire. Results Compared with the average and advanced children, LD children got significantly lower scores in self-concept scale. Based on logistic regression analysis, 3 factors were identified, including family income per month, single child and delivery model. Conclusion The results suggest that self-consciousness of children with LD is lower than that of normal children.     

Eagle's综合征的诊断和治疗——附7例分析

茎突过长是一种较少见的解剖异常，偶而可伴随有口咽，头颈部的不适或疼痛，并被命名为Ｅａｇｌｅ＇ｓ综合征。本文对７例因茎突过长而致口腔、咽、颈部不适的患者，从发病原因、病理机制、临床表现和体征，诊断及治疗等各方面进行了回顾分析；总结了两种手术途径的适应征和优缺点。作者首次将３维ＣＴ重建成像技术用于Ｅａｇｌｅ＇ｓ综合征的诊断，认为与以前常用的Ｘ线检查方法相比，３维ＣＴ重建成像技术具有更为明显的优点     

Langerhans cell disease associated with pathological fracture of the mandible

A 27-year-old male suffered a fractured mandible following extraction of a tooth. It was subsequently found that the fracture occurred in an area pathologically weakened by a localized lesion of Langerhans cell disease. Since lesions of the jaws may be seen either as the first manifestation or as a complication of widespread Langerhans cell disease, the dentist has a major role in the diagnosis and management of such cases.     

Cone-beam computed tomography in assessment of periodontal ligament space: in vitro study on artificial tooth model

The aim of this in vitro study was to compare cone-beam computed tomography (CBCT) to conventional radiography (RG) in the assessment of the periodontal ligament space. A phantom with a variable "artificial" periodontal ligament space (0, 100, 200, 300, and 400 microm) was used as a model. The examinations were performed simultaneously with RG and NewTom 9000 digital volume tomograph. Assorted after increasing widths, 15 RGs and 15 CBCT images were presented for judgment to 20 dentists (DD), 20 dental assistants, and 20 dental students. Several weeks later, the same images were randomly mixed and presented to the same 20 DD again. The trial shows that RG gaps wider than 200 microm could be correctly identified by all participants with an accuracy of nearly 100%. A significant difference was observed between the modalities (p<0.05 and p<0.001) where conventional RGs performed better than CBCT for assessment of periodontal ligament space. Interobserver variation in relation to each technique was evaluated and no significant difference was found (p>0.05). In subjective evaluations of image quality with CBCT, the results were basically inferior for images of artificial periodontal ligament space, regardless of the experience of the observers.     

肝炎肝化病人抑郁状况调查及护理对策

目的 研究肝炎肝硬化病人抑郁状况及相关因素。方法 采用Zung抑郁自评量表（SDS）和问卷表对120例住院肝硬化病人进行调查。结果 65％肝硬化病人存在不同程度的抑郁状况；在分组比较中，中年和文化程度在大专以上者抑郁发生率偏高，肝硬化病人抑郁发生与性别无相关性。结论 肝炎肝硬化病人发生抑郁关况，临床护理人应给予足够重视，采取相应的护理措施，以维护病人的心理健康。