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1.
Sarah E. Sheppard Laura Elizabeth Anderson Cathryn Sibbald Colleen Cotton Elizabeth Bhoj Marissa J. Perman Leslie Castelo‐Soccio 《Pediatric dermatology》2019,36(6):1007-1009
Epidermolysis bullosa simplex (EBS) is a skin fragility disorder resulting from mutations of structural proteins in the epidermis. We provide a brief report of long‐term survival and reproduction in a mother with EBS due to keratin 5 (KRT5) c.1429G > A (p.E477K) mutation, which causes a particularly severe form of the disease. 相似文献
2.
Joseph D. Westaby Susanna T. E. Cooper Khari A. Edwards Robert H. Anderson Mary N. Sheppard 《Clinical anatomy (New York, N.Y.)》2020,33(3):394-404
Congenital heart disease is a rare but important finding in adults who experience sudden death. Examination of the congenitally malformed heart has historically been considered esoteric and best left to those with expertise. The Cardiac Risk in the Young cardiovascular pathology laboratory based at St George's University of London has now received over 6,000 cases. Of these, 21 congenitally malformed hearts were retained for research and educational purposes. Hearts were assessed using sequential segmental analysis, and causes of death were adjudicated based on thorough macroscopic examination and histology. Congenital malformations that were encountered included atrial septal defects, ventricular septal defects, tetralogy of Fallot, and transposition of the great arteries in both its regular and congenitally corrected variants. Findings also included hearts with mirror-imaged and isomeric atrial appendages. Direct causes of death included myocardial fibrosis, pulmonary hypertension, and hemorrhage. A small but notable proportion did not reveal a substrate for arrhythmia, raising the question of whether the terminal event was due to the congenital heart disease itself, or an underlying channelopathy. Here, we demonstrate the value of simple sequential segmental analysis in describing and categorizing the cases, with the concept of the “morphological method” serving to identify the distinguishing features of the cardiac components. Clin. Anat. 33:394–404, 2020. © 2019 Wiley Periodicals, Inc. 相似文献
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We examined weight changes over 1 and 2 y in 303 women enrolled in a low-fat dietary-intervention trial. Participants were randomly assigned to an intervention group that received intensive instruction in maintaining a low-fat diet or to a control group. After 1 y intervention-group women had decreased fat intake by 45.3 g (from 39.2% to 21.6% energy from fat) and weight by 3.1 kg (all P less than 0.0001); control-group women decreased fat intake by 8.8 g (from 38.9% to 37.3% energy from fat) and weight by 0.4 kg. In both univariate analyses and multivariate models, weight loss was more strongly associated with change in percent energy from fat than with change in total energy intake. These data, which are consistent with both epidemiologic and clinical studies, suggest that body adiposity is a function both of energy balance and the proportion of energy derived from fat. 相似文献
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The first 150 words of the full text of this article appear below. Key points Coronary artery disease accounts for >30% ofdeaths in Western society. The diagnosis of myocardial infarctionshould be qualified by size, causation and time from occurrence. Mortalityis reduced by immediate or primary percutaneouscoronary intervention or thrombolysis within the first 24 hof onset of ST-segment elevation myocardial infarction. Strategiesto reduce platelet activation (glycoprotein IIb/IIIa receptorantagonists, or clopidogrel) are now recommended in the treatmentof high-risk non-ST-segment myocardial infarction/unstable angina. Elevatedserum troponins may be the result of non-ischaemic myocardialdamage, especially in critical illness.
Pathophysiology
Changes in the definition of terms relating to the diagnosisof myocardial infarction (MI) have evolved by better understandingof the pathophysiology culminating in the new term of acutecoronary syndrome (ACS). Figure 1 illustrates the processesthat occur in the development of an acute coronary event. 相似文献
7.
James C Slaughter Thomas Lumley Lianne Sheppard Jane Q Koenig Gail G Shapiro 《Annals of allergy, asthma & immunology》2003,91(4):346-353
BACKGROUND: Exposure to air pollutants has been investigated as a possible cause of asthma attacks in children. OBJECTIVE: To investigate the short-term effects of air pollutants on a panel of 133 children with asthma who enrolled in the Childhood Asthma Management Program. METHODS: During screening, the children completed daily diary cards for an average of 58 days to indicate their medication use and asthma severity. We used ordinal logistic regression to compare the odds of a more serious relative to a less serious asthma attack, and we used a Poisson model to analyze medication use. In both analyses we accommodate dependence in the data and different periods of observation for study subjects. RESULTS: Our results indicate that a 10-microg/m3 increase in particulate matter less than or equal to 2.5 microm (PM2.5) lagged 1 day was associated with a 1.20 times increased odds of having a more serious asthma attack [95% confidence interval (CI), 1.05 to 1.37] and a 1.08-fold increase in medication use (95% CI, 1.01 to 1.15). A 10-microg/m3 increase in particulate matter less than or equal to 10 microm (PM10) increased the odds of a more serious asthma attack (odds ratio = 1.12; 95% CI, 1.04 to 1.22) and also increased medication use (relative risk = 1.05; 95% CI, 1.00 to 1.09). CONCLUSIONS: Increases in PM2.5 and PM10 are significantly associated with an increased risk of more severe asthma attacks and medication use in Seattle area children with asthma. We also found associations with carbon monoxide, but we believe that carbon monoxide is a marker for exposure to combustion byproducts. 相似文献
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Mahrdad Farahmand M.D. Brett C. Sheppard M.D. Clifford W. Deveney M.D. Karen E. Deveney M.D. Richard A. Crass M.D. 《Journal of gastrointestinal surgery》1997,1(2):182-187
Between 1989 and 1995 we performed completion gastrectomy for non-malignant disease in 21 patients (11 men and 10 women, mean
age 48.4 years). These patients had undergone a total of 48 prior gastric operations. Indications for completion gastrectomy
in this group were anastomotic ulceration with stricture in eight patients, alkaline reflux gastritis and/or esophagitis in
eight, postsurgical gastroparesis in two, gastroesophageal necrosis in two, and gastrocutaneous fistula in one. Major preoperative
symptoms included nausea and vomiting in 16 cases, abdominal pain in 15, dysphagia in 14, heartburn in seven, and weight loss
in five. Following completion gastrectomy, five patients (24%) had serious complications and there was one postoperative death
(5%). Five patients were lost to follow-up. For the remaining 15 patients, mean follow-up has been 30 months with a range
of 1 to 70 months. These patients were all interviewed and eight (53%) report significant improvement, two (13%) report moderate
improvement, and four (27%) report no improvement; one patient (7%) has had worsening of symptoms since undergoing completion
gastrectomy. The average body weight index was essentially unchanged after completion gastrectomy. We conclude that completion
gastrectomy with Roux-en-Y esophagojejunostomy results in a favorable outcome in the majority of selected patients with diseases
of the foregut who are unresponsive to less radical treatment.
Presented at the Thirty-Seventh Annual Meeting of The Society for Surgery of the Alimentary Tract, San Francisco, Calif.,
May 19–22, 1996. 相似文献
10.
H S Pall A C Williams D A Heath M Sheppard R Wilson 《Postgraduate medical journal》1987,63(742):665-667
A well-nourished alcoholic patient developed a subacute myopathy which responded rapidly to correction of severe hypomagnesaemia. The finding of profound hypocalcaemia prompted the measurement of serum magnesium. Magnesium deficiency should be looked for in any alcoholic patient with a myopathy as the prognosis seems better than in many other forms of alcoholic myopathy. Correction of the magnesium deficiency corrects the hypocalcaemia without the need for calcium supplementation. 相似文献