Notes

Clinical and also molecular evaluation associated with smoothened inhibitors within Sonic Hedgehog medulloblastoma.
The OVAMA (
utcome
easures for
scular
lformations) project determined quality of life (QoL) as a core outcome domain for evaluating treatment effect in vascular malformations. To correctly evaluate treatment effect on QoL, patient-reported outcome measures (PROMs) are needed that are responsive to changes. In children with vascular malformations, we explored if two widely used PROMs were responsive to changes the Pediatric Quality of Life Inventory (PedsQL) and the Children's Dermatology Life Quality Index (CDLQI).

In an international multicenter prospective study, conservatively and invasively treated children completed the PedsQL and CDLQI at baseline and after follow-up of 6-8 weeks. At follow-up, change in health was assessed by a global rating of change (GRC) scale. Apamin in vivo Responsiveness was assessed by testing hypotheses on expected correlation strength between change scores of the PROMs and the GRC scale, and by calculating the area under the receiver operating characteristics curve (AUC). The PROMs were considered responsive if ≥75% of the hypotheses were confirmed or if the AUC was ≥0.7.

Twenty-nine children were recruited in three centers in the Netherlands and United States, of which 25 completed all baseline and follow-up measurements. For both the PedsQL and CDLQI, less than 75% of the hypotheses were confirmed and the AUC was <0.7.

The results suggest that these PROMs are not sufficiently responsive for evaluating treatment effect in peripheral vascular malformations. Our study emphasizes the need for assessing responsiveness before using a PROM in evaluating treatment effect.
The results suggest that these PROMs are not sufficiently responsive for evaluating treatment effect in peripheral vascular malformations. Our study emphasizes the need for assessing responsiveness before using a PROM in evaluating treatment effect.•We report the first case of a paraurethral mixed Müllerian cystadenoma.•The cystic lesion was lined by a mixture of three different types of epithelium.•All epithelial cells were positive for estrogen receptor and PAX8.•Complete gross resection as part of debulking surgery is crucial in advanced ovarian cancer.•Supradiaphragmatic lymph node resection may prolong survival in patients with ovarian cancer.•We report acute pericarditis after supradiaphragmatic lymph node resection and pericardotomy.This is a case of recurrent intravascular leiomyomatosis in a pre-menopausal woman of African-Caribbean heritage. She presented in 2006 with multiple uterine leiomyomata, tumour invading the inferior vena cava (IVC) extending into the right atrium, and pulmonary metastases. Her initial presentation was treated surgically. On recurrence she was treated by oestrogen suppression using a combination of goserelin and letrozole, with a substantial response. She subsequently reported further regression of disease following exposure to strong sunlight enabling her to discontinue oestrogen suppression without relapse. The hypothesis is that the benefit was due to vitamin D. The role of hypovitaminosis D in the pathogenesis of uterine leiomyomata is discussed, including epidemiology data demonstrating a link between ethnicity and risk and the proven mechanisms by which vitamin D controls oestrogen and progesterone receptor expression and influences other signalling pathways involved in the pathogenesis of leiomyomas. Data indicating the intermediate malignancy nature of intravascular leiomyomatosis, are discussed. We are not aware of other reports indicating a link between intravascular leiomyomatosis and a lack of vitamin D.
Chronic inflammatory diseases lead to glycation of protein, lipids and nuclear acids. One product generated in this context is pentosidine.

To study pentosidine levels in Systemic Lupus Erythematosus (SLE) and its possible association with disease activity and cumulative damage.

Pentosidine serum levels were measured in the serum by ELISA commercial kits in 79 patients with SLE. Disease activity index and cumulative damage were studied by SELENA-SLEDAI (Safety of Estrogen in Lupus National Assessment Systemic Lupus Erythematosus Disease Activity Index) and cumulative damage by SLICC/ACR DI (Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index for Systemic Lupus Erythematosus) respectively and simultaneously with determination of pentosidine levels. Epidemiological and clinical and serological profile were collected from the charts.

In the 79 studied patients, the SLEDAI ranged from 0 to 12 (median of 0) and the SLICC/ACR-DI from 0 to 4 (median of 0). Serum pentosidine levels did not correlate with SLEDAI neither with SLICC. Patients with discoid skin lesions and photosensitivity had lower levels than those without them, with p=0.04 in both.

In SLE, serum pentosidine levels did not reflect activity and cumulative damage. Patients with skin manifestations had lower levels of this biomarker.
In SLE, serum pentosidine levels did not reflect activity and cumulative damage. Patients with skin manifestations had lower levels of this biomarker.The dataset presented in this article is an examination of coronavirus (COVID-19) thought concern, employees' work performance, employees background, feeling about job, work motivation, job satisfaction, psychological state of mind and family commitment in two middle east countries. The data were collected one time of survey data during June and July 2020 targeting people from different sectors whom have job through a survey distributed via an online questionnaire. Coronavirus (COVID-19) thought concern (9 items), employees' work performance (5 items), employees background (6 items), feeling about job (5 items), work motivation (6 items), job satisfaction (6 items), psychological state of mind (2 items) and family commitment (3 items). A mixture of purposive and snowball techniques facilitated to choose the respondents via email. We distributed 950 questionnaires through email due to the current circumstances, we offer suggestions from actual time-surveys handled in two Arabic countries the Yemen and Saudi Aribia, with a total final correct sample of 307 respondents.
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