In mucopolysaccharidosis I, deficiency of alpha-L-iduronidase can cause spinal cord compression (SCC) due to storage of glycosaminoglycans (GAGs) within the cervical meninges. As intravenous enzyme replacement therapy (ERT) is not likely to provide enzyme across the blood-brain barrier, standard treatment for this complication is usually surgical, which has a high morbidity and mortality risk. We report on the use of intrathecal (IT) laronidase in a MPS I patient with SCC who refused the surgical treatment. Assessments were performed at baseline, with clinical and biochemical evaluations, 4-extremity somatosensory evoked potentials, 12 min walk test and MRI studies of the CNS. Changes on these parameters were evaluated after 4 IT infusions of laronidase administered monthly via lumbar puncture. To our knowledge, this was the first MPS patient who received IT ERT. No major adverse events were observed. There were no clinically significant changes in serum chemistries. CSF GAG results revealed pretreatment values slightly above normal standards: 13.3 mg/L (NV < 12 mg/L) which after IT laronidase infusions were within normal levels (10.3 mg/L). 12MWT presented a 14% improvement, with better performance on stability and gait control. Maximum voluntary ventilation showed 55.6% improvement considering the percentage of predicted (26.7% at baseline compared to 41.9%); Maximum Inspiration Pressure improved 36.6% of predicted (26.8% at baseline to 36.7%); Pulmonary diffusion improved 17.6% of predicted %. In conclusion, although the improvement observed in this case with IT laronidase should be confirmed in further patients, this procedure seems to be a safe treatment for SCC in MPS I.

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These results evidence the possibility to inform collectively the research subjects when getting the Informed Consent in those projects where this choice is available.

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Age is probably not an adequate variable to measure decision-making capacity, because questions of medical consent relate to participants' own personal health. Decision-making capacity should be viewed as a continuous function. While the age of the person whose consent is needed should be considered, age alone, from a moral perspective, is not the main determinant of this capacity.

ObjectiveA major barrier to the adoption of an approach that integrates spirituality into palliative care is the lack of preparation/education of healthcare professionals on the topic. This study aimed to evaluate the effectiveness of a continuing education activity for healthcare professionals addressing spirituality and spiritual care provision to patients and families within palliative care.MethodWe conducted an intervention study using a quantitative pre- and posttest design in a convenience sample of 52 healthcare professionals. Participants completed the Brazilian version of the Spiritual Care Competence Scale before and after attending a four-hour continuing education activity.ResultSignificant differences were observed between pre- and postintervention scores in the following dimensions: assessment and implementation of spiritual care, professionalization and improving the quality of spiritual care, personal support, and patient counseling (p < 0.001), and referral (p = 0.003).Significance of resultsThe results of this study provide preliminary evidence of a positive effect of this educational intervention on the development of the competences needed by healthcare professionals to deliver a comprehensive approach centered on the patient/family, which includes attention to spirituality and spiritual care in the decision-making process.

O artigo objetiva discutir a atenção em saúde sob uma perspectiva intercultural e laica. A proposição da bioética como interdisciplina, desde Potter até autores contemporâneos, traz consigo forte estímulo à reflexão sobre a atenção em saúde e a premente necessidade de diálogo entre os saberes envolvidos nesta área. A interculturalidade, como proposta de abordagem em saúde, remete ao pensamento de que não é possível exercer a atenção em saúde sem relacioná-la com outros elementos envolvidos além dos biológicos, tais como históricos, sociais, políticos, econômicos, religiosos, culturais, entre outros. Um modelo de atenção em saúde pode ser construído a partir da interpretação de uma realidade, que por sua vez também pode ser construída. Atualmente, os distintos modelos de atenção em saúde existentes se deparam com o desafio de integrar-se entre si, com base em seus saberes diversos, legitimando uma real atenção integral em saúde.

This is a cross-sectional validation study following the stages proposed by Beaton et al.: translation into Portuguese, back translation into English, expert committee review for semantic equivalence, assessment of the clarity of the pre-final version, and evaluation of the psychometric properties of the final version in Portuguese. Health professionals working at a public hospital in South Brazil participated in the different stages of this study.ResultRegarding internal consistency, total Cronbach's alpha was 0.92 and the mean inter-item correlation was 0.29. The test-retest procedure showed no statistically significant differences in the six subscales. The intraclass correlation coefficient ranged from 0.67 to 0.84, demonstrating the stability of the scale.Significance of resultsThe results support the psychometric quality of the scale and indicate that the adapted instrument is a valid and reliable scale with good internal consistency for measuring spiritual care competencies of health professionals in Brazilian healthcare settings.