Early diagnosis of ataxia-telangiectasia using radiosensitivity testing

OBJECTIVES: To utilize radiosensitivity testing to improve early diagnosis of patients with ataxia-telangiectasia (A-T). STUDY DESIGN: We established normal ranges for the colony survival assay (CSA) by testing cells from 104 patients with typical A-T, 29 phenotypic normal patients, and 19 A-T heterozygotes. We also analyzed 61 samples from patients suspected of having A-T and 25 patients with related disorders to compare the CSA with other criteria in the diagnosis of A-T. RESULTS: When cells were irradiated with 1.0 Gy, the mean survival fraction (microSF +/- 1 SD) for patients with A-T was 13.1% +/- 7.2% compared with 50.1% +/- 13.5% for healthy control patients. These data served to define a diagnostic range for the CSA (ie, <21%), a normal range (>36%), and a nondiagnostic intermediate range of 21% to 36%. The mutations of patients with A-T with intermediate radiosensitivity tended to cluster around the functional domains of the ATM gene. CONCLUSIONS: The CSA is a useful adjunctive test for confirming an early clinical diagnosis of A-T. However, CSA is also abnormal in other chromosomal instability and immunodeficiency disorders.     

Nursing at ground zero: experiences during and after September 11 World Trade Center attack

The purpose of this study is to discover shared perceptions, feelings, and common experiences of nurses after the September 11th World Trade Center terrorist attack through interpretive analysis of narrative stories of seventeen nurses. Six themes and one constitutive pattern describe the experiences: (a) Loss of a symbol and regaining new meaning, (b) Disaster without patients, (c) Coordinating with and without organizations, (d) Rediscovering the pride in nursing, (e) Traumatic Stress, and (f) Preparing for the future. The constitutive pattern is that nursing enables a humanitarian disaster response.     

Introducing a simple,weight-based,color-coded,medication dosing device

OBJECTIVE: To compare the time required to withdraw various pediatric resuscitation medications using traditional techniques and the Per-Kilo Doser (PKD), a new weight-based dosing device. METHODS: Seven emergency department nurses were each videotaped as they withdrew medications for four different pediatric resuscitation scenarios. In random order, each nurse performed a total of eight timing trials-four with traditional techniques and four with the PKD. The videotapes were later reviewed and timed by two reviewers who were unaware of the study purpose. The time to drug withdrawal (announcement of medication and dose to be withdrawn until the nurse completed medication withdrawal) was measured in seconds. Nonparametric and exact techniques were used to determine statistical significance. RESULTS: Using traditional techniques, the mean time to drug withdrawal was 71.8 seconds; using the PKD, the mean time to drug withdrawal was 43.1 seconds. The mean time difference between the PKD and traditional techniques was 29.2 seconds (95% CI, 16.7-41.7). CONCLUSION: The PKD decreases medication withdrawal time.     

Information technology and the National Centers of Excellence in Women's Health

Women are increasingly turning to the Internet for health information. The National Centers of Excellence in Women's Health (U.S. Dept. of Health and Human Services) have as one goal the use of information technology to improve care of women. Telemedicine, the Internet, and Resource Centers were used for patient consultation, patient support, support of women in academics, and clinician education regarding women's health. Access of the lay public to high-quality health information was achieved using the Internet as well as Resource Centers employing Internet educational modalities. Telemedicine, distance learning for clinicians and patient consultations for those far from medical care, has been used successfully. For clinicians, continuing medical education regarding women's health and calendars of opportunities for education in women's health were made available on-line. Creative new uses of information technology have been developed by the Centers of Excellence in Women's Health. These modalities may be adopted, tested, and adapted by others seeking to improve the care and health of women.     

Solitary fibrous tumor of the orbit in a child

PURPOSE: Solitary fibrous tumor is a rare spindle-cell tumor that usually is seen in the pleura. The orbit is one of the most common extrapleural sites. It is frequently misdiagnosed as hemangiopericytoma and is seen in older patients. We present the youngest case of this tumor, which was apparent in family photographs by age 10 and removed at age 15. The first reported echography findings are presented. METHODS: Case report and literature review. RESULTS: Solitary fibrous tumor was diagnosed by microscopy and immunohistochemical study that showed cells reactive with vimentin and CD34. CONCLUSIONS: Solitary fibrous tumor of the orbit has been diagnosed with increasing frequency in recent years as the result of improved methods of pathologic examination. It is important to be aware of this tumor and recognize that it must be included in the differential diagnosis of highly vascular spindle-cell tumors even in young children.     

Induction and maintenance infliximab therapy for the treatment of moderate-to-severe Crohn's disease in children

BACKGROUND AND AIMS: The REACH study evaluated the safety and efficacy of infliximab in children with moderately to severely active Crohn's disease. METHODS: Patients (n = 112) with a Pediatric Crohn's Disease Activity Index (PCDAI) score >30 received infliximab 5 mg/kg at weeks 0, 2, and 6. Patients responding to treatment at week 10 were randomized to infliximab 5 mg/kg every 8 or 12 weeks through week 46. A concurrent immunomodulator was required. Clinical response (decrease from baseline in the PCDAI score > or =15 points; total score < or =30) and clinical remission (PCDAI score < or =10 points) were evaluated at weeks 10, 30, and 54. RESULTS: At week 10, 99 of 112 (88.4%) patients responded to infliximab (95% confidence interval: [82.5%, 94.3%]) and 66 of 112 (58.9%) patients achieved clinical remission (95% confidence interval: [49.8%, 68.0%]). At week 54, 33 of 52 (63.5%) and 29 of 52 (55.8%) patients receiving infliximab every 8 weeks did not require dose adjustment and were in clinical response and clinical remission, respectively, compared with 17 of 51 (33.3%) and 12 of 51 (23.5%) patients receiving treatment every 12 weeks (P = .002 and P < .001, respectively). CONCLUSIONS: Pediatric patients responding to an induction regimen of infliximab were more likely to be in clinical response and remission at week 54 without dose adjustment when their maintenance therapy was given every 8 weeks rather than every 12 weeks. Allowing for dose intensification in the case of relapse, remission rates, but not response rates, at week 54 were superior with every 8-week dosing compared with every 12-week dosing.     

Barriers to advancement in academic surgery: views of senior residents and early career faculty

Background

A significant faculty attrition rate exists in academic surgery. The authors hypothesized that senior residents and early-career faculty members have different perceptions of advancement barriers in academic surgery.

Methods

A modified version of the Career Barriers Inventory–Revised was administered electronically to surgical residents and early-career surgical faculty members at 8 academic medical centers.

Results

Residents identified a lack of mentorship as a career barrier about half as often as faculty members. Residents were twice as likely as faculty members to view childbearing as a career barrier.

Conclusions

Many early-career faculty members cite a lack of mentors as a limitation to their career development in academic surgery. Childbearing remains a complex perceived influence for female faculty members in particular. Female faculty members commonly perceive differential treatment and barriers on the basis of their sex. Faculty development programs should address both systemic and sex-specific obstacles if academic surgery is to remain a vibrant field.     

A limited comparison of apraclonidine's dose response in subjects with normal or increased intraocular pressure

We performed a multicentered, placebo-controlled, randomized, crossover study comparing the efficacy of 0.5% and 1.0% apraclonidine hydrochloride in 15 normal volunteers and 17 subjects with increased intraocular pressure. Apraclonidine 1% produced a maximum 30.4% +/- 14.0% (4.7 +/- 2.4 mm Hg) decrease in mean intraocular pressure in normal eyes and a 31.3% +/- 16.5% (7.6 +/- 4.2 mm Hg) decrease in eyes with increased pressure. Apraclonidine 0.5% produced a maximum 25.8% +/- 9.7% (4.0 +/- 1.7 mm Hg) decrease in mean intraocular pressure in normal eyes and a 27.4% +/- 16.0% (6.8 +/- 4.5 mm Hg) decrease in eyes with increased pressure. There was no statistically significant difference in mean percent intraocular pressure lowering effect between the 0.5% and 1.0% apraclonidine concentrations. Most subjects treated with apraclonidine had a greater than or equal to 20% reduction in intraocular pressure from baseline. Twelve hours after instillation of apraclonidine, nine of the normal volunteers had an intraocular pressure of 10 mm Hg or less. Apraclonidine produced the same percent intraocular pressure decrease regardless of the initial level of intraocular pressure.