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991.
Objective: To evaluate the reliability and validity of the PROMIS Pediatric Global Health scale, a 7-item measure of perceived physical, mental, and social health, in children with asthma. Methods: From February 2014 to February 2015, convenience samples of 8–17 year-old children (n = 182) and parents of 5–17 year-old children (n = 328) visiting an emergency department for treatment of asthma were enrolled. The Asthma Control Test was used to characterize children as controlled versus not controlled, and the PROMIS Asthma Impact Scale was used to assess asthma symptoms' impact on functional status. We conducted longitudinal analyses among 92 children and 218 parents at 3 weeks, and 74 children and 171 parents at 8 weeks after enrollment. Results: The PGH-7 reliability ranged from 0.66 to 0.81 for child-report and 0.76 to 0.82 for parent-proxy. In cross-sectional analyses, children with controlled asthma had PGH-7 scores 0.40–0.95 standard deviation units higher than those who were uncontrolled. The PGH-7 was responsive to changes in overall general health between time points, with moderate effect sizes (0.5–0.6 standard deviation units). In longitudinal analyses, PGH-7 scores were no different between those who stayed uncontrolled versus became controlled at 3 weeks of follow-up; however, by 8 weeks of follow-up, the differences between these groups were 0.7–0.8 standard deviation units, indicative of large effects. Conclusions: The PGH-7 is a reliable and valid patient-reported outcome for assessing general health among children with asthma. It is a useful complement to other asthma-specific outcome measures.  相似文献   
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Craniofacial microsomia (CFM) is most often described as a unilateral malformation of derivatives of the first and second branchial arches. The mandible has been classified using several classification systems. However, all are based on two-dimensional imaging. The aim of this study was to mathematically describe the deformed mandible based on principal component analysis (PCA) in a three-dimensional way. This may aid in defining the flaws in existing surgical corrections of the mandible through the identification of the differences in shape compared with a normal mandible in a holistic view with the help of videos. Forty-three homologous landmarks were defined to describe a mandible with CFM. Computed tomography scans of 22 patients and 30 controls were marked manually. The changes in shape between the mandibles were visualized using videos. A lateral rotation with increase in posterior rotation of the condyle due to shortening of the condyle–gonial height and a longitudinal rotation with outward bending of the mandibular angle were noted on the affected side, as well as an inward bending of the angle on the unaffected side. Due to the compensatory remodelling of the mandible on the unaffected side, one could suggest that CFM is never truly unilateral.  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 and Aims

In addition to overt stroke lesions, co-occurring covert lesions, including white matter hyperintensities (WMH) and covert lacunar infarcts (CLI), contribute to poststroke outcome. The purpose of this study was to examine the relationship between covert lesions, and motor and cognitive outcomes in individuals with chronic stroke.

Methods

Volumetric quantification of clinically overt strokes, covert lesions (periventricular and deep: pWMH, dWMH, pCLI, dCLI), ventricular and sulcal CSF (vCSF, sCSF), and normal appearing white (NAWM) and gray matter (NAGM) was performed using structural magnetic resonance imaging. We assessed motor impairment and function, and global cognition, memory, and other cognitive domains. When correlation analysis identified more than one MR parameter relating to stroke outcomes, we used regression modeling to identify which factor had the strongest impact.

Results

Neuropsychological and brain imaging data were collected from 30 participants at least 6 months following a clinically diagnosed stroke. Memory performance related to vCSF (r = ?0.52, P = .004). The strongest predictor of nonmemory domains was pCLI (r2 = 0.28, P = .004). Motor impairment and function were most strongly predicted by the volume of stroke and NAWM (r2 = 0.36; P = .001), and dWMH (r2 = 0.39; P = .001) respectively.

Conclusions

Covert lesion type and location have important consequences for post-stroke cognitive and motor outcome. Limiting the progression of covert lesions in aging populations may enhance the degree of recovery post-stroke.  相似文献   
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