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Cutis marmorata telangiectatica congenita (CMTC) is characterized by the appearance of telangiectasia, phlebectasia, and a persistent reticular pattern of subcutaneous vasculature at or soon after birth. Up to 90% of cases are associated with systemic abnormalities, which include body asymmetry, cutaneous atrophy, neurological abnormalities, and vascular anomalies (nevus flammeus, Sturge-Weber syndrome, Klippel-Trenaunay syndrome, and capillary and cavernous hemangiomas). Glaucoma is the most commonly reported ocular association of CMTC, usually presenting in infancy. We report a case of CMTC associated with the previously unreported onset of glaucoma in mid-childhood that was managed by glaucoma drainage implant surgery and review the literature on glaucoma associated with this condition.  相似文献   
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PurposeAlthough quantitative benefits of mindfulness training have been demonstrated in youth, little is known about the processes involved. The aim of this study was to gain a detailed understanding of how young people engage with the ideas and practices known as mindfulness using qualitative enquiry.MethodsFollowing completion of a six-week mindfulness training program with a nonclinical group of 11 young people (age 16–24), a focus group (N = 7) and open-ended interviews (n = 5) were held and audio-recorded. Qualitative data, collected at eight time points over three months from the commencement of training, were coded with the aid of computer software. Grounded theory methodology informed the data collection process and generation of themes and an explanatory model that captured participants' experiences.ResultsParticipants described their daily lives as beset by frequent experiences of distress sometimes worsened by their unhelpful or destructive reactions. With mindfulness practice, they initially reported greater calm, balance, and control. Subsequently they commented on a clearer understanding of themselves and others. Mindfulness was then described as a “mindset” associated with greater confidence and competence and a lessened risk of future distress.ConclusionsParticipants demonstrated a sophisticated understanding of and engagement with mindfulness principles and practice. Their reported experience aligned well with qualitative research findings in adults and theoretical literature on mindfulness. An encouraging finding was that, with ongoing mindfulness practice and within a relatively short time, participants were able to move beyond improved emotion regulation and gain greater confidence in their ability to manage life challenges.  相似文献   
34.
Solid-cystic papillary tumor of the pancreas in children   总被引:4,自引:0,他引:4  
Solid-cystic papillary tumor (SCPT) of the pancreas is a rare neoplasm in children. The current study attempted to estimate the incidence and possible pathological origin of the tumor. Clinicopathologic features of the children under the age of 16 years with pancreatic tumors managed in a single institution between January 1993 and November 1999 were reviewed. Representative blocks of SCPT specimens were immunostained for neuron-specific enolase (NSE) chromogrannin, synaptophysin, insulin, glucagon, somatostatin, and gastrin. There were three SCPTs, two pancreatic endocrine tumors, and one acinar cell carcinoma during the study period. The estimated yearly incidence in the referral area of 2 million population is about 0.01 pediatric SCPT per year per 100,000 population. The children underwent surgical removal of the tumors. Postoperatively, they were followed up for 6 months to 4 years and were well. Immunostaining for NSE, chromogranin, synaptophysin, insulin, somatostatin, and gastrin was negative in all cases. SCPT may thus be the most common pancreatic tumor in the Asian pediatric population. The pathological origin of the tumor remains unclear and requires further investigations. Accepted: 30 October 2000  相似文献   
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BACKGROUND AND AIM: There is an increasing use of active-fixation leads for cardiac pacing, yet concerns remain regarding initial high stimulation thresholds. The aim was to perform a detailed analysis of pacing parameters at the time of implantation to determine when lead repositioning should be considered. METHODS: We performed a prospective observational study of consecutive new pacemaker implants. Detailed analysis of pacing parameters was collected at 2-minute intervals for 10 minutes, and at day 1 and week 8 following implant. RESULTS: Ninety-four patients underwent implantation of 79 dual-chamber and 15 single-chamber pacemakers using active-fixation leads in both chambers. An initial threshold of >1 V was demonstrated in 45/94 (48%) ventricular leads (mean threshold 1.5 +/- 0.3 V). This declined rapidly to 0.9 +/- 0.3 V at 4 minutes (P < 0.01), 0.7 +/- 0.3 V at 10 minutes (P < 0.01), and 0.6 +/- 0.3 V at day 1 (P < 0.01). At day 1, 43/45 leads were <1 V. There were 79 atrial leads. An initial threshold of >1 V (mean 1.7 +/- 0.6 V) was demonstrated in 41/79 (52%) leads falling significantly to 1.1 +/- 0.5 V at 4 minutes (P < 0.01), 0.9 +/- 0.4 V at 10 minutes (P < 0.01), and 0.6 +/- 0.2 V at day 1 (P < 0.01). At 10 minutes, 32 of 41 leads demonstrated a threshold of <1 V with all leads <1 V at day 1. Thresholds were maintained medium term. CONCLUSIONS: Active-fixation leads are commonly associated with initially high thresholds that fall rapidly. An initial threshold of 2 V should be provisionally accepted and retested at 4 minutes. The majority will have a threshold of <1 V the following day. A failure of a high threshold to decline at 4 minutes requires lead repositioning.  相似文献   
37.
Hemoglobin H disease (HbH) is a hemoglobinopathy peculiar to parts of the world with high incidence -thalassemia mutations. Among 90 HbH cases, 50 cases suffered from clinically significant jaundice (bilirubin >30 mmol/l), including 14 with severe jaundice (bilirubin >60 mmol/l). Cholelithiasis was found in 38 cases. The incidence is roughly eight times higher than that in background control population but 50% lower than that in -thalassemia. The risk of gallstones was related to higher bilirubin levels but not -globin genotype, sex, ferritin, and hemoglobin levels. Homozygotes or double heterozygotes for Gilbert alleles (17.2%), but not heterozgyotes (42.2%), were found to have a significantly increased risk of gallstones and jaundice. However, common Chinese Gilbert syndrome alleles do not completely explain the variable risks.  相似文献   
38.
OBJECTIVE: Attenuation changes on computed tomography (CT) in mediastinal lymph nodes (LN) may be related to lung alterations and functional impairment in silicosis. DESIGN: CT and clinical data of 41 (64.2 +/- 8.3 years) males with silicosis were retrieved. Attenuation type (calcified, hyperdense, normodense) and calcification pattern (central, eccentric, dense, eggshell, speckled) of mediastinal LN were evaluated; LN attenuation of uncalcified LNs quantified on CT in six LN stations. Nodular profusion (CT-NP) and progressive massive fibrosis (CT-PMF) were graded. Relationships between LN, CT, lung function and clinical parameters were determined. RESULTS: LN sites were paratracheal (n = 39), subcarinal (n = 39), tracheobronchial (n = 37), aortopulmonary (n = 37), hilar (n = 27), and peri-oesophageal (n = 21). LNs were calcified, hyperdense and normodense in 107, 85 and 54 LN stations, respectively. Uniformly calcified LN was most common, followed by speckled calcification. Central, eccentric and eggshell calcification was rare. CT-NP scores > or = 16 were associated with higher LN attenuation and number of calcified LN stations than CT-NP scores < 16. PMF had no influence over LN morphology or calcification pattern. LN attenuation correlated with CT-PMF (r = 0.36, P = 0.01), CT-NP (r = 0.54, P < 0.001) and DLCO/VA (r = -0.33, P = 0.02). CONCLUSION: Uniformly calcified and hyperdense LNs are common in silicosis, and eggshell LN calcification is rare. There are associations between LN attenuation and lung function impairment, and CT grades of nodular profusion and PMF.  相似文献   
39.
Ratneswaran  C.  Steier  J.  Reed  K.  Khong  T. K. 《Lung》2019,197(5):533-540
Introduction

In contrast to tobacco smoking, electronic cigarette (“vaping”) advertisement had been approved in the United Kingdom (UK) in January 2013. Currently, there are an estimated 3.2 million UK e-cigarette users. The impact of e-cigarette advertisement on tobacco use has not been studied in detail. We hypothesised that e-cigarette advertisement impacts on conventional smoking behaviour.

Methods

A cross-sectional structured survey assessed the impact of e-cigarette advertising on the perceived social acceptability of cigarette and e-cigarette smoking and on using either cigarettes or e-cigarettes (on a scale of 1 to 5/‘not at all’ to ‘a lot’). The survey was administered between January to March 2015 to London university students, before and after viewing 5 UK adverts including a TV commercial.

Results

Data were collected from 106 participants (22 ± 2 years, 66% male), comprising cigarette smokers (32%), non-smokers (54%) and ex-smokers (14%). This included vapers (16%), non-vapers (77%) and ex-vapers (7%). After viewing the adverts, smokers (2.6 ± 1.0 vs. 3.8 ± 1.1, p = 0.001) and non-smokers (3.2 ± 0.7 vs. 3.7 ± 0.8, p = 0.007) felt smoking was more socially acceptable, compared to before viewing them. Participants were more likely to try both e-cigarettes (1.90 ± 1.03 to 3.09 ± 1.11, p < 0.001) and conventional cigarettes (1.73 ± 0.83 to 2.27 ± 1.13, p < 0.001) after viewing the adverts compared to before. Vapers were less likely to smoke both an e-cigarette, and a conventional cigarette after viewing the adverts.

Conclusion

E-cigarette advertising encourages both e-cigarette and conventional cigarette use in young smokers and non-smokers. The adverts increase the social acceptability of smoking without regarding the importance of public health campaigns that champion smoking cessation.

  相似文献   
40.
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