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Study Objective

To review our local experience with urogenital rhabdomyosarcoma (RMS) to determine the most common clinical presentation(s).

Design

Retrospective case series of all female patients with urogenital RMS who presented to a tertiary pediatric hospital between 1996 and 2016. All institutional electronic pathology reports were screened for RMS and those that were pelvic in origin and occurred in female patients were included for further analysis. Seventeen cases of urogenital RMS in female patients were identified and reviewed.

Setting

This study was conducted at The Royal Children's Hospital in Melbourne, Australia. This is a tertiary referral center for the state of Victoria and surrounding areas, which services more than 1.5 million pediatric patients.

Participants

Female pediatric patients (ages 0-18 years) who presented to The Royal Children's Hospital with eventual pathologic tissue diagnosis of urogenital RMS.

Main Outcome Measures

The cases were reviewed for clinical presentation, duration of symptoms before initial presentation, time to tissue diagnosis, and outcomes of treatment.

Results

Of the 17 cases reviewed, 5 (29%) presented with perineal mass, 4 (24%) presented with each of abdominal mass and grape-like lesions/hemorrhagic mass at the introitus, 3 (18%) with nonspecific symptoms only, and 1 (6%) with vulvar inflammation.

Conclusion

The clinical presentation of urogenital RMS in women is heterogeneous, and the classically described presentation of grape-like lesions at the introitus and vaginal bleeding represents only a small proportion of clinical presentations. Awareness of other presentations, which appear to be more common than previously recognized, needs to be increased to ensure timely diagnosis and treatment.  相似文献   
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In this study, we aimed to determine whether a postnatal trans fat diet (TFD) could aggravate prenatal bisphenol A (BPA) exposure effects on offspring’s small intestine and adulthood obesity, due to the relatively sparse findings on how the interaction between these two variables interrupt the small intestinal cells. Twelve pregnant rats were administered with either unspiked drinking water (control; CTL) or BPA-spiked drinking water throughout pregnancy. Twelve weaned pups from each pregnancy group were then given either a normal diet (ND) or TFD from postnatal week (PNW) 3 until PNW14, divided into control offspring on normal diet (CTL-ND), BPA-exposed offspring on normal diet (BPA-ND), control offspring on trans fat diet (CTL-TFD), and BPA offspring on trans fat diet (BPA-TFD) groups. Body weight (BW), waist circumference, and food and water intake were measured weekly in offspring. At PNW14, small intestines were collected for global DNA methylation and histological analyses. Marked differences in BW were observed starting at PNW9 in BPA-TFD (389.5 ± 10.0 g; p < 0.05) relative to CTL-ND (339.0 ± 7.2 g), which persisted until PNW13 (505.0 ± 15.6 g). In contrast, water and food intake between offspring were significantly different (p < 0.01–0.05) at earlier ages only (PNW4–6 and PNW7–9, respectively). Furthermore, substantial differences in the general parameters of the intestinal structures were exclusive to ileum crypt length alone, whereby both BPA-ND (150.5 ± 5.1 μm; p < 0.001), and BPA-TFD (130.3 ± 9.9 μm; p < 0.05) were significantly longer than CTL-ND (96.8 ± 8.9 μm). Moreover, BPA-ND (2.898 ± 0.147%; p < 0.05) demonstrated global small intestinal hypermethylation when compared to CTL-ND and CTL-TFD (1.973 ± 0.232% and 1.913 ± 0.256%, respectively). Prenatal BPA exposure may significantly affect offspring’s physiological parameters and intestinal function. Additionally, our data suggest that there might be compensatory responses to postnatal TFD in the combined BPA prenatal group (BPA-TFD).  相似文献   
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BackgroundRadial artery occlusion (RAO) is the most common complication following transradial approach (TRA) for cardiac catheterisation. Our aim was to assess if decreasing radial hemostatic device (RHD) time reduces the risk of RAO among individuals receiving small sheath sizes with no adjunctive heparin.MethodsWe randomised 450 individuals undergoing diagnostic cardiac catheterization via TRA to 3 durations of RHD time: 10, 20, or 30 minutes. After these time periods, the RHD was gradually released over 20 minutes. The primary efficacy end point was forearm hematoma grade ≥ 2 (5-10 cm) and the primary safety end point was RAO (as determined by Doppler ultrasound) 1 hour after RHD removal (before discharge).ResultsThe mean age was 66 years and 64% were male. Five-French sheaths were used in all patients. Hematoma grade ≥ 2 occurred in only 1 patient, who was in the 20-minute group (P = 0.39). RAO occurred in 6.7% of patients in the 10-minute group, 10.7% in the 20-minute group and 6% in the 30-minute group (P = 0.26).ConclusionsAmong patients receiving small-caliber sheaths without adjunctive heparin, the incidence of forearm hematoma and RAO are low. Shorter durations of RHD time did not further reduce the risk of these complications.  相似文献   
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Acute pancreatitis due to 5-aminosalicylate   总被引:1,自引:0,他引:1  
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We determined prospectively the clinical and electrophysiological progression of idiopathic, diabetic, paraproteinemic, alcoholic, and B12 deficiency neuropathy in 606 subjects over 3 years. We hypothesized that idiopathic peripheral neuropathy would demonstrate slower progression when compared with other etiologies. Laboratory assessments were used to determine the etiology of peripheral neuropathy at baseline and after 3 years. When compared with peripheral neuropathy related to type 1 or type 2 diabetes mellitus, subjects with idiopathic peripheral neuropathy progressed much slower, but demonstrated similar rates of progression to that of the other groups. Overall, detectable progression was minimal over 3 years. After 3 years, only 3% of cases of idiopathic peripheral neuropathy had any potentially identifiable causes discovered. Clinical and electrophysiological detection of very slow progression for these five types of peripheral neuropathy is possible using currently established clinical scales and standard electrophysiological techniques.  相似文献   
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