Clinical proceedings - Children's Hospital of the District of Columbia最新文献

Salmonella Infections 沙门氏菌感染

Clinical proceedings - Children's Hospital of the District of Columbia Pub Date : 2015-05-01 DOI: 10.1542/9781581109276-salmonella

引用次数: 0

Coarctation of Aorta 主动脉缩窄

Clinical proceedings - Children's Hospital of the District of Columbia Pub Date : 2007-01-01 DOI: 10.5005/JP/BOOKS/10463_27

V. Jose

引用次数: 0

Cystic fibrosis 囊性纤维化

Clinical proceedings - Children's Hospital of the District of Columbia Pub Date : 1984-04-01 DOI: 10.1055/s-2007-1011472

R. Wood

{"title":"Cystic fibrosis","authors":"R. Wood","doi":"10.1055/s-2007-1011472","DOIUrl":"https://doi.org/10.1055/s-2007-1011472","url":null,"abstract":"Cystic fibrosis (CF) is the most common lethal inherited disease in causcasian populations in North American and most of Europe. It is estimated that as many as 5 percent of the population may be heterozygous for the CF gene. CF has not yet been defined as a specific genetic disorder with a known gene product. It is possible that more than one genetic defect may be responsible for the syndrome now recognized as CF. There is clearly a wide variation in clinical severity and even clinical presentation among different patients, although most observations are consistent with an autosomal recessive mode of inheritance. At present, the median survival age for patients born with CF is approximately 21 years. Clinically, CF is characterized by three major features: increased concentrations of sodium and chloride in the sweat, chronic obstructive lung disease with chronic bacterial infection, and, in 90 percent of the patients, exocrine pancreatic insufficiency. The latter is usually manifest from birth and is commonly thought to result from pancreatic ductal obstruction with consequent autodigestion of the secretory elements of the pancreas. The pulmonary component of CF is the most significant clinical aspect of the disorder and is responsible for at least 95 percent of the deaths. Patients with CF begin life with normal lungs, but soon develop evidence of hypersecretion of mucus. Chronic bacterial colonization and infection, characteristically with Staphylococcus aureus and/or Pseudomonas aeruginosa, soon follow. In most patients, the initial pathologic process begins in the bronchioles, with peribronchiolar inflammation and bronchiolar obstruction. In many infants, this process may be initiated or exacerbated by concurrent viral infection. Once infection has become established, the sequence of pathophysiologic events becomes less clear. As the disease progresses, larger airways become involved, with chronic inflammation progressing to bronchiectasis. Clinically, the bronchial secretions are increased in volume and viscosity, and are usually purulent. Mucociliary transport is decreased but not absent, and ciliary structure and beat (at least in vitro) appear normal. Patients develop a viscous cycle of airway obstruction leading to airway infection, which in turn leads to further obstruction. Eventually destruction and obstruction of airways progress to the point of irreversibility, and patients usually die in respiratory failure with cor pulmonale.","PeriodicalId":72622,"journal":{"name":"Clinical proceedings - Children's Hospital of the District of Columbia","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"1984-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1055/s-2007-1011472","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"58176860","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Convergent Strabismus 内斜视

Clinical proceedings - Children's Hospital of the District of Columbia Pub Date : 1982-01-01 DOI: 10.1007/978-94-009-8024-2

引用次数: 0

Progress in Iran's pediatric care in the past twenty years. 过去二十年伊朗儿科护理的进展。

Clinical proceedings - Children's Hospital of the District of Columbia Pub Date : 1970-10-01

H Ahari

引用次数: 0

Effects of child rearing patterns on the disadvantaged child. 儿童养育方式对弱势儿童的影响。

Clinical proceedings - Children's Hospital of the District of Columbia Pub Date : 1970-10-01

A E Marans

引用次数: 0

Surgical grand rounds: Hirschsprung's disease with enterocolitis. 外科查房:先天性巨结肠病合并小肠结肠炎。

Clinical proceedings - Children's Hospital of the District of Columbia Pub Date : 1970-10-01

J G Randolph

引用次数: 0

History of Children's Hospital of the District of Columbia. 哥伦比亚特区儿童医院的历史。

Clinical proceedings - Children's Hospital of the District of Columbia Pub Date : 1970-07-01

E A Torkington, H Isherwood

引用次数: 0

The department of surgery at Children's Hospital: past, present and future. 儿童医院外科:过去、现在和未来。

Clinical proceedings - Children's Hospital of the District of Columbia Pub Date : 1970-07-01

J Randolph

引用次数: 0

The emerging Children's Hospital National Medical Center: a decade of growth and change. 新兴的儿童医院国家医疗中心:十年的成长与变化。

Clinical proceedings - Children's Hospital of the District of Columbia Pub Date : 1970-07-01

R H Parrott

引用次数: 0