Approaches to end-of-life decision-making in the NICU: insights from Dostoevsky's The Grand Inquisitor.

For many parents stopping life-sustaining medical treatment on their dying infant is psychologically impossible. Dostoevsky's insights into human behavior, particularly the fact that individuals do not want the anxiety and guilt associated with responsibility for making difficult decisions, might change the way physicians approach parents for permission to withdraw life-prolonging medical interventions on dying infants.     

Outcomes of low-vision services using optometric and multidisciplinary approaches: a non-randomized comparison

Consecutive patients (n = 215) who were referred to optometric (55%) or multidisciplinary (45%) low-vision services and above 50 years of age were recruited from four hospitals in the Netherlands. They completed two vision-related quality of life questionnaires, the Vision Quality of Life Core Measure (VCM1) and the Low Vision Quality of Life Questionnaire (LVQOL), before their first visit with low-vision services and 1 year later. At follow-up, patients referred to multidisciplinary low-vision services had lower scores on the mobility subscale of the LVQOL than patients referred to optometric low-vision services [5.3 points; 95% confidence interval (CI): 0.2-10.5]. Paired sample t-tests for the two groups of patients taken together show improvement for the VCM1 (3.1 points; 95% CI: 0.6-5.6) and deterioration for the basic aspects of vision (3.5 points; 95% CI: 1.1-5.9) and the mobility (6.6 points; 95% CI: 3.7-9.5) subscales of the LVQOL. In conclusion, people referred to optometric services showed less deterioration in mobility than those referred to multidisciplinary services. No differences were observed for any of the other subscales of the LVQOL and the VCM1. Future research in this field should include randomized controlled designs comparing low-vision services with no treatment or placebo.     

Periodontal status of orthodontic patients and the relationship between dental aesthetic index and community periodontal index of treatment need

This prospective clinic-based study evaluated the pretreatment periodontal status of the orthodontic patients seen at the University College Hospital, Ibadan, Nigeria, and assessed the relationship between dental aesthetic index (DAI) scores and periodontal status according to community periodontal index of treatment needs (CPITN) scores. One hundred forty five patients-70 (48.3%) males and 75 (51.7%) females from 6 to 45 years (mean 15.8 +/- 7.5)-were seen. World Health Organization (WHO) guidelines were followed in the examination and reporting of the periodontal status, and DAI scores were assessed based on WHO guidelines. The chi-square test was used to determine the association between the DAI and the CPITN scores. Most patients were in the 6-15 (55.9%) or 16-25 (35.9%) age groups. Based on the WHO preferred cumulative calculations of treatment need (TN), 35.2% of the patients had TN 0, 64.9% had TN 1, 24.9% had TN 2, and only 0.7% had TN 3. The relationship between DAI scores and periodontal treatment needs was not statistically significant (P >.05). Although many patients were yet to attain the WHO goal of no more than 1 sextant affected by bleeding or calculus at the age of 15, over one third had satisfactory periodontal health.     

Islet Xenotransplantation: Are We Really Ready for Clinical Trials?

Four clinical trials of porcine islet transplantation have been reported, and there are verbal reports that clinical trials on much larger scales are continuing in centers in China and Russia. The four reported trials are briefly reviewed and, in the light of the present status of experimental islet xenotransplantation, consideration is given to whether such trials are currently justified. The Ethics Committee of the International Xenotransplantation Association has (1) emphasized the need for encouraging studies in non-human primates before clinical trials should be undertaken, (2) mandatory monitoring for the transfer of porcine microorganisms, and (3) careful regulation and oversight by recognized bodies. Other aspects of the topic, such as the need for informed consent, are briefly discussed. We conclude that, at the present time, more data documenting convincing efficacy, focused on clinically applicable immunosuppressive regimens, are needed to justify the initiation of closely monitored clinical trials. A clinical trial may then be justified even though the potential risk to the patients, and possibly for society, will not be zero.     

Spinocerebellar ataxia types 2 and 3 segregating simultaneously in a single family.

Spinocerebellar ataxia (SCA) types 2 and 3 are autosomal-dominant neurodegenerative disorders caused by mutations in two different genes. We identified mutations for SCA2 and SCA3 segregating simultaneously in a single Brazilian family. The index patient had SCA2, whereas her two second-degree cousins had SCA3. Disease was more rapidly progressive in the SCA2 patient, who presented severe brainstem and pancerebellar atrophy, as opposed to the two SCA3 patients, who had only mild cerebellar vermian atrophy. In such situations, molecular confirmation of all patients may avoid misdiagnosis of SCA subtypes and eventual errors in predictive testing of unaffected family members.     

Pathological structure of the kidney from adult mice with mosaic mutation

Summary: The mosaic (Atp7a ^mo-ms ) is an X-linked, lethal mutation in mice. In mosaic mutant males, many clinical features characteristic of defective copper metabolism have been observed and they die at the age of 15 days, exhibiting strongsimilarities to the brindled and macular mutants. About 4% of the mutant males live to sexual maturity and some of them are fertile. In this paper, alterationsin the structure of the kidney from adult mutants are described. Owing to an inherited defect of efflux, copper is accumulated in the kidney of the mutants up to a toxic level and this leads to severe damage of the renal cortex. Pathological changes in the kidney mostly affected the structure of the renal corpuscle and renaltubules.