Adaptive behavior assessed by Vineland-3 as comprehensive outcome measure in vanishing white matter.

OBJECTIVES: Investigate the results and usability of the Vineland-3 as an outcome measure in vanishing white matter patients. METHODS: A cross-sectional investigation of the Vineland-3 based on interviews with caregivers, the Health Utilities Index, and the modified Rankin Scale in 64 vanishing white matter patients. RESULTS: Adaptive behavior measured with the Vineland-3 is impaired in the vast majority of vanishing white matter patients and significantly impacts daily life. Typically, the daily living skills domain is most severely affected and the socialization domain is the least affected. Based on the metric properties and the clinical relevance, the standard scores for the daily living skills domain and Adaptive Behavior Composite have the best properties to be used as an outcome measure. INTERPRETATION: The Vineland-3 appears to be a useful outcome measure to explore and quantify complex cognitive, behavioral, and psychiatric impairments affecting daily functioning in vanishing white matter. Further research should address the longitudinal evaluation of this tool and its additional value to standard neuropsychological and clinical examination.

The effect of natural growth on chin point deviation in patients with unilateral craniofacial microsomia: A retrospective study.

This study aimed to investigate the potential progressiveness of mandibular asymmetry and to study factors that influence chin point deviation in patients with unilateral craniofacial microsomia (CFM). Paediatric patients with unilateral CFM with available radiologic imaging and medical photographs were included. Chin point deviation was measured on clinical photographs. A Jonckheere-Terpstra test and linear mixed model for repeated measurements assessed the relation of chin point deviation on natural growth, Pruzansky-Kaban score, and soft tissue score. A total of 110 patients were included. The linear mixed model showed no statistically significant changes of chin point deviation during growth (effect estimate -0.006°, 95% CI -0.04° to -0.03°, p = 0.74). A statistical significant relation between both the Pruzansky-Kaban and soft tissue score on chin point deviation was found (effect estimate -5.10°, 95% CI -6.45° to -3.75°, p ≤ 0.001 and effect estimate -3.42°, CI -5.86° to -0.98°, p ≤ 0.001, respectively). Within the limitations of the study it seems that craniofacial microsomia may be a non-progressive disorder, because chin point deviation did not change over time.

Physicians' views on the usefulness and feasibility of identifying and disclosing patients' last phase of life: a focus group study.

OBJECTIVES: Accurate assessment that a patient is in the last phase of life is a prerequisite for timely initiation of palliative care in patients with a life-limiting disease, such as advanced cancer or advanced organ failure. Several palliative care quality standards recommend the surprise question (SQ) to identify those patients. Little is known about physicians' views on identifying and disclosing the last phase of life of patients with different illness trajectories. METHODS: Data from two focus groups were analysed using thematic analysis with a phenomenological approach. RESULTS: Fifteen medical specialists and general practitioners participated. Participants thought prediction of patients' last phase of life, i.e. expected death within 1 year, is important. They seemed to find that prediction is more difficult in patients with advanced organ failure compared with cancer. The SQ was considered a useful prognostic tool; its use is facilitated by its simplicity but hampered by its subjective character. The medical specialist was considered mainly responsible for prognosticating and gradually disclosing the last phase. Participants' reluctance to such disclosure was related to uncertainty around prognostication, concerns about depriving patients of hope, affecting the physician-patient relationship, or a lack of time or availability of palliative care services. CONCLUSIONS: Physicians consider the assessment of patients' last phase of life important and support use of the SQ in patients with different illness trajectories. However, barriers in disclosing expected death are prognostic uncertainty, possible deprivation of hope, physician-patient relationship, and lack of time or palliative care services. Future studies should examine patients' preferences for those discussions.