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11.
Visual-evoked potential evidence of chiasmal hypoplasia   总被引:3,自引:0,他引:3  
PURPOSE: To show that chiasmal hypoplasia or aplasia need not be an isolated developmental anomaly and to examine the spectrum of associated clinical findings to explore the possibility that these patients may represent a phenotypic manifestation of a developmental gene anomaly. DESIGN: An observational case series. PARTICIPANTS: Five infants, between several weeks and 7 months of age, in whom the electrophysiologic characteristic of chiasmal hypoplasia had been noted were included. METHODS: Flash electroretinography and flash and pattern visual-evoked potentials (VEPs) were elicited from all patients. Clinical ophthalmologic examinations, including funduscopy, were performed, and all patients had magnetic resonance imaging (MRI) brain scans. MAIN OUTCOME MEASURES: The occipital distribution of monocular VEP response peaks was studied. The symmetry of lateral channel responses was compared for monocular stimulation. RESULTS: All five patients had a crossed asymmetry in the monocular VEP occipital distribution, which is consistent with a paucity of fibers crossing at the chiasm. The MRI findings supported this electrophysiologic observation, illustrating degrees of chiasmal hypoplasia and variable coincidence of other midline abnormalities of the brain. Optic disc appearances varied from normal to hypoplastic and colobomatous. CONCLUSIONS: The ophthalmologic and MRI findings of five patients who showed a crossed asymmetry in monocular flash VEPs are consistent with a paucity of axons crossing at the chiasm. The similarities between achiasmia in humans and mice due to a Pax2 gene anomaly are discussed.  相似文献   
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Background

Solid organ transplantation is associated with a higher risk of Epstein-Barr virus (EBV)–related lymphoproliferative disease due to immunosuppressive regimen. Little evidence is currently available on post-transplant lymphoproliferative disorders (PTLDs) in the lung transplant (LuTx) setting, particularly in cystic fibrosis (CF) recipients.

Methods

We retrospectively analyzed all the cases of PTLDs that occurred in our LuTx center between January 2015 and December 2017. We reviewed clinical and radiologic data, donor and recipient EBV serostatus, immunosuppressive therapy, histologic data, and follow-up of these patients.

Results

A total of 77 LuTxs were performed at our center in the study period; 39 (50.6%) patients had CF; 4 developed EBV-related PTLDs. They were all young (17–26 years) CF patients with high serum EBV DNA load. Disease onset was within the first 3 months after LuTx. In 3 cases presentation was associated with fever and infection-like symptoms, whereas in 1 case radiologic suspicion arose unexpectedly from a CT scan performed for different clinical reasons. Diagnosis was reached through lung biopsy in all cases. All patients received rituximab,?cyclophosphamide, doxorubicin hydrochloride (hydroxydaunomycin), vincristine sulfate (Oncovin), and prednisone with variable response and complications.

Conclusion

In our experience, the early development of EBV-related PTLD was a highly aggressive, life-threatening condition, which exclusively affected young CF patients in the early post-transplant period. The rate of this complication was relatively high in our population.Diagnosis with lung biopsy is crucial in all suspected cases and regular monitoring of EBV DNA levels is of utmost importance given the high correlation with PTLDs in patients at increased risk.  相似文献   
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Background

The Veterans Health Administration (VHA) faces challenges in providing comprehensive, gender-sensitive care for women. National policies have led to important advancements, but local leadership also plays a vital role in implementing changes and operationalizing national priorities. In this article, we explore the notions of ideal women veterans' health care articulated by women's health leaders at local VHA facilities and regional networks, with the goal of identifying elements that could inform practice and policy.

Methods

We conducted semistructured interviews with 86 local and regional women's health leaders at 12 VHA medical centers across four regions. At the conclusion of interviews about women's primary care, participants were asked to imagine “ideal care” for women veterans. Interviews were transcribed and coded using a hybrid inductive/deductive approach.

Results

In describing ideal care, participants commonly touched on whether women veterans should have separate primary care services from men; the need for childcare, expanded reproductive health services, resources, and staffing; geographic accessibility; the value of input from women veterans; the physical appearance of facilities; fostering active interest in women's health across providers and staff; and the relative priority of women's health at the VHA.

Conclusions

Policy and practice changes to care for women veterans must be mindful of key stakeholders' vision for that care. Specific features of that vision include clinic construction that anticipates a growing patient population, providing childcare and expanded reproductive health services, ensuring adequate support staff, expanding mechanisms to incorporate women veterans' input, and fostering a culture oriented towards women's health at the organizational level.  相似文献   
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The Institutional Graduate Partnerships Program (GPP) offered by the National Institute of Nursing Research (NINR) provides an exceptional opportunity for students who are enrolled in any PhD program in nursing across the nation to complete dissertation research on the premier research campus of the National Institutes of Health, Bethesda, MD. The goal of this doctoral fellowship program, which is up to 3 years in length, is to train promising doctoral students in basic and clinical research. This knowledge and skill set is necessary for the next generation of nurse scientists to ultimately conduct translational research. In this article, the authors describe the program, eligibility requirements, application procedures, and selection criteria for NINR-supported GPP nursing students. Also provided are tips for interested students and outcomes of current and former NINR-supported GPP students (NINR-GPP).  相似文献   
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BackgroundFruit consumption is known to be beneficial to health. However, the health benefits of fruit juice are controversial due to its high sugar content.ObjectivesTo examine the associations of frequency of consumption of whole fruit and fruit juice with obesity and metabolic syndrome.DesignThis cross-sectional study used data from the 2012-2015 Korea National Health and Nutrition Examination Survey.ParticipantsA total of 10,460 adults (4,082 men and 6,378 women) aged 19 to 64 years were included in the study.Main outcome measuresFrequency of consumption of whole fruit and fruit juice was evaluated using a food frequency questionnaire, and dietary sugar intake was calculated using a 24-hour recall. Obesity and abdominal obesity were determined using body mass index and waist circumference, respectively. Metabolic syndrome was defined on the basis of the National Cholesterol Education Program Adult Treatment Panel III.Statistical analyses performedMultiple logistic regression analysis was performed to examine the associations between frequency of whole fruit or fruit juice consumption and obesity and metabolic syndrome abnormalities.ResultsThe percentage of participants who consumed whole fruit daily was 32.6%, whereas 52.3% consumed fruit juice rarely. The average intake of total sugars was 14.9% of total energy, which was within the recommend range (<20% of total energy) for Koreans. Consuming whole fruit ≥1 time/day was associated with reduced prevalence of obesity, abdominal obesity, and elevated blood pressure compared with consuming whole fruit ≤1 time/wk. However, frequency of fruit juice consumption showed no association with obesity, abdominal obesity, and metabolic syndrome.ConclusionsFrequency of whole fruit consumption was associated with reduced prevalence of obesity and metabolic syndrome abnormalities among Korean adults with average total sugar intake within the recommended range.  相似文献   
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