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Introduction: Autosomal dominant hyperimmunoglobulinemia E syndrome (HIES), also called Job's syndrome, is a primary immunodeficiency characterized by the triad of elevated immunoglobulin E levels, eczema, and infections. Its clinical course manifests as recurrent skin and pulmonary infections, and variable skeletal, connective tissue, and vascular abnormalities. There is evidence of abnormal tissue remodeling with pneumatocoeles frequently complicating pyogenic pneumonias and leading to secondary infections that cause the majority of morbidity and mortality. Complications are known to occur after lung surgery with a high frequency of bronchopleural fistulae, but little has been reported concerning abdominal surgeries., Discussion: Here, we report on the outcome and safety of two separate complex cases (hepatectomy and subtotal gastrectomy) and document our entire experience with abdominal surgical procedures performed on patients with HIES. Despite initial complications, all patients eventually made a full recovery., Conclusion: As HIES patients now frequently live beyond the third and fourth decade, surgical issues similar to those in the general population may increase. Complex surgical procedures can be performed safely and benefit select patients with HIES, but benefit strongly from multidisciplinary teams and awareness of complications related to abnormal healing. We discuss current treatment and potential complications post-operatively in patients with HIES.

Objective: To evaluate the relationship of race/ethnicity to Cognitive and Language scores on the Bayley Scales of Infant and Toddler Development, Third Edition in extremely preterm toddlers (<28 + 0 weeks' estimated gestational age)., Study Design: This cohort study included extremely preterm toddlers seen at Eunice Kennedy Shriver National Institute of Child Health and Human Development Neonatal Research Network centers evaluated at 18-22 months adjusted age from 3 race/ethnic groups (white, black, and Hispanic white). Multivariate regression modeling was used to identify race/ethnic differences, after adjusting for medical and psychosocial factors., Results: The study population comprised 369 whites, 352 blacks, and 144 Hispanic whites. Cognitive scores differed among the 3 groups on unadjusted analysis (P ≤ .001), but not after adjusting for medical and psychosocial factors (P = .13). Language scores differed on adjusted and unadjusted analyses. Whites scored higher than blacks and Hispanic whites, and blacks scored higher than Hispanic whites., Conclusions: A combination of medical variables and primary caretaker education accounted for differences in Bayley Scales of Infant and Toddler Development, Third Edition Cognitive scores among the 3 groups. Black and Hispanic white toddlers had lower Language scores than whites, even after adjustment. Early intervention should be targeted to these identified risk factors. Assessment of early language development in minority children may be warranted., (Copyright © 2012 Mosby, Inc. All rights reserved.)

Aspergillus viridinutans, a mold phenotypically resembling A. fumigatus, was identified by gene sequence analyses from 2 patients. Disease was distinct from typical aspergillosis, being chronic and spreading in a contiguous manner across anatomical planes. We emphasize the recognition of fumigati-mimetic molds as agents of chronic or refractory aspergillosis.

Relatively few articles discuss the ethical issues that accompany healthcare in rural areas. This article presents and discusses the key findings obtained from multi-method research studies conducted over a 9-year period of time in a multi-state rural area. It challenges the efficacy of current models for bioethics, shows what kinds of ethical issues develop in rural communities, and offers a framework for envisioning resources and approaches that may be more appropriate.

Recent reports from the Institute of Medicine and other sources have shown that far too many avoidable medical errors occur; other research has shown a strong association between patient outcomes and characteristics of nursing staff. The authors of this paper present findings from multimethod research conducted over three years in 29 small rural hospitals in nine Western states. They examined the organizational processes used to recognize medical errors and assign responsibility for them to resolve patient-safety issues. The research comprises seven substudies that used surveys, questionnaires, interviews, and case studies to gather data from nurses, physicians, administrators, pharmacists, and other health care workers.Generally, participants responded positively to questions about an institution's receptivity to communicating about errors and agreed on the most common kinds of errors that occur. But other data suggest that providers' understanding of patient safety is heavily conditioned by preconceived notions of what constitutes an error and of professional roles. Participants' analyses of case studies showed that they don't agree on what constitutes error or what kinds of events should be reported. And in one substudy, even when there was overwhelming agreement among participants (97%) that an error had occurred, only 64% would disclose the error to the patient affected. Physicians, administrators, and nurses tended to perceive patient safety as primarily a nursing responsibility. Only 22% of respondents to one survey said that physicians, nurses, pharmacists, and administrators should share responsibility equally for patient safety. The research was not designed to answer specific questions about the recruitment and retention of nurses, but the data collected suggest that institutional processes used to identify errors, assign responsibility for them, and resolve patient-safety issues may have unintended, harmful effects on nurse recruitment and retention. The authors propose that "a systems approach to patient safety" be adopted, one in which responsibility for safety is shared by all members of the health care team.