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

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Salmonella Infections 沙门氏菌感染
Pub Date : 2015-05-01 DOI: 10.1542/9781581109276-salmonella
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引用次数: 0
Coarctation of Aorta 主动脉缩窄
Pub Date : 2007-01-01 DOI: 10.5005/JP/BOOKS/10463_27
V. Jose
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引用次数: 0
Cystic fibrosis 囊性纤维化
Pub Date : 1984-04-01 DOI: 10.1055/s-2007-1011472
R. Wood
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.
囊性纤维化(CF)是北美和欧洲大部分地区高加索人群中最常见的致命性遗传性疾病。据估计,多达5%的人群可能是CF基因的杂合子。CF尚未被定义为具有已知基因产物的特定遗传疾病。可能不止一种遗传缺陷导致了现在被认为是CF的综合征。尽管大多数观察结果与常染色体隐性遗传模式一致,但不同患者的临床严重程度甚至临床表现明显存在很大差异。目前,先天性CF患者的中位生存年龄约为21岁。临床上,CF以三个主要特征为特征:汗液中钠和氯化物浓度升高,慢性阻塞性肺疾病伴慢性细菌感染,90%的患者伴有外分泌胰腺功能不全。后者通常从出生时就表现出来,通常被认为是由胰腺导管阻塞引起的,随后胰腺分泌成分自身消化。CF的肺部部分是该疾病最重要的临床方面,至少95%的死亡是由它造成的。CF患者开始时肺部正常,但很快就出现粘液分泌过多的迹象。慢性细菌定植和感染,以金黄色葡萄球菌和/或铜绿假单胞菌为特征,很快就会发生。在大多数患者中,最初的病理过程始于细支气管,伴细支气管周围炎症和细支气管梗阻。在许多婴儿中,这一过程可由并发病毒感染引发或加重。一旦感染确定,病理生理事件的顺序就变得不那么清楚了。随着疾病进展,更大的气道受累,慢性炎症进展为支气管扩张。临床表现为支气管分泌物体积和黏度增加,常呈脓性。纤毛黏液运输减少,但并非没有,纤毛结构和搏动(至少在体外)看起来正常。患者形成气道阻塞的粘性循环,导致气道感染,进而导致进一步的阻塞。最终气道的破坏和阻塞发展到不可逆转的程度,患者通常死于呼吸衰竭和肺心病。
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引用次数: 1
Convergent Strabismus 内斜视
Pub Date : 1982-01-01 DOI: 10.1007/978-94-009-8024-2
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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。
H Ahari
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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。
A E Marans
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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。
J G Randolph
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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。
E A Torkington, H Isherwood
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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。
J Randolph
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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。
R H Parrott
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引用次数: 0
期刊
Clinical proceedings - Children's Hospital of the District of Columbia
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