A systematic approach to eye muscle surgery for infantile nystagmus syndrome: results in 100 patients.

PURPOSE: To report a systematic approach to, and the visual and electrophysiological effect of, eye muscle surgery in 100 patients with infantile nyustagmus syndrome (INS). METHODS: Prospective, interventional case cohort analysis of clinical and eye movement data in 100 patients with INS who had virgin extraocular eye muscles operated on for nystagmus with or without combinations of strabismus and an anomalous head posture. All patients were followed at least 9 months after surgery. Outcome measures, part of an IRB approved study, included binocular visual acuity, head position, strabismic deviation, and eye movement recordings, from which waveform types and an Automated Nystagmus Acuityn Function (ANAF) was calculated. Computerized parametric and non-parametric statistical analysis of data were performed using standard software on both individual and group data. RESULTS: There were 9 consistent surgical procedures used with the most common being that for a horizontal head posture alone (22%). Age at surgery averaged 14 years with 11 months followup. Sixty-eight percent had associated eye disease (optic nerve, retinal, amblyopia, cataracts). Group means in binocular acuity, strabismic deviation, head posture, abd ANAF measures from eye improved for all procedures. There were 12 (12%) reoperations without any serious surgical complications. Individual analysis revealed only age and head posture differences in outcome measures between the 9 procedures. CONCLUSIONS: Using this approach, surgery on the extraocular muscles in patients with INS results in improvements in multiple aspects of ocular motor and visual function.

Healthcare proxies of nursing home residents with advanced dementia: decisions they confront and their satisfaction with decision-making.

OBJECTIVES: To describe the medical decisions confronting healthcare proxies (HCPs) of nursing home (NH) residents with advanced dementia and to identify factors associated with greater decision-making satisfaction. DESIGN: Prospective cohort study. SETTING: Twenty-two Boston-area NHs. PARTICIPANTS: Three hundred twenty-three NH residents with advanced dementia and their HCPs. MEASUREMENTS: Decisions made by HCPs over 18 months were ascertained quarterly. After making a decision, HCPs completed the Decision Satisfaction Inventory (DSI) (range 0-100). Independent variables included HCP and resident sociodemographic characteristics, health status, and advance care planning. Multivariable linear regression identified factors associated with higher DSI scores (greater satisfaction). RESULTS: Of 323 HCPs, 123 (38.1%) recalled making at least one medical decision; 232 decisions were made, concerning feeding problems (27.2%), infections (20.7%), pain (12.9%), dyspnea (8.2%), behavior problems (6.9%), hospitalizations (3.9%), cancer (3.0%), and other complications (17.2%). Mean DSI score +/- standard deviation was 78.4 +/- 19.5, indicating high overall satisfaction. NH provider involvement in shared decision-making was the area of least satisfaction. In adjusted analysis, greater decision-making satisfaction was associated with the resident living on a special care dementia unit (P=.002), greater resident comfort (P=.004), and the HCP not being the resident's child (P=.02). CONCLUSION: HCPs of NH patients with advanced dementia can most commonly expect to encounter medical decisions relating to feeding problems, infections, and pain. Inadequate support from NH providers is the greatest source of HCP dissatisfaction with decision-making. Greater resident comfort and care in a special care dementia unit are potentially modifiable factors associated with greater decision-making satisfaction.

Wrongful life and wrongful birth: legal aspects of failed genetic testing in oocyte donation.

The field of reproductive medicine has all but exploded in recent years, allowing more people access to novel treatments. New technologies have been introduced so rapidly that ethics and the law have been unable to respond appropriately. A particularly promising reproductive therapy, oocyte donation, and the wide variety of prenatal genetic tests already available, will theoretically present the legal world with a variety of new challenges. How should the law respond to potential wrongful birth and wrongful life suits resulting from failed genetic testing, if the child in question was conceived using a donor egg? Under what circumstances should the physician be held liable? Under what circumstances might the donor be culpable? This paper reviews scholarly opinions and existing case law in order to answer these questions.

Manipulation of kinetic profiles in 2-aryl propionic acid cyclooxygenase inhibitors.

The nonsteroidal anti-inflammatory drugs flurbiprofen and ibuprofen were modified in an attempt to alter the kinetics of inhibitor binding by COX-1. Contrary to prior predictions, a halogen substituent is not sufficient to confer slow tight-binding behavior. Conversion of the carboxylate moiety of flurbiprofen to an ester or amide abolishes slow tight-binding behavior, regardless of halogenation state.

Understanding syncope. The pathophysiology & assessment of a challenging condition that EMS crews frequently encounter.

Syncope is a transient loss of postural tone and consciousness following which, by definition, the patient has a spontaneous recovery without intervention. The differential diagnosis of syncope is broad and spans benign to life-threatening conditions. Identifying and treating serious conditions that may mimic syncope and identifying patients at high risk (cardiac syncope) are challenges that face prehospital providers. A comprehensive history and physical provide the most useful information in making these distinctions. Syncope is a symptom, not a disease; therefore, treatment must be targeted to the underlying disease process.