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Cystic fibrosis.

作者信息

Welsh M J, Fick R B

机构信息

Department of Internal Medicine, University of Iowa College of Medicine, Iowa City 52242.

出版信息

J Clin Invest. 1987 Dec;80(6):1523-6. doi: 10.1172/JCI113237.

DOI:10.1172/JCI113237
PMID:3316277
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC442419/
Abstract
摘要
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b58e/442419/0fd6e04f9c93/jcinvest00096-0016-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b58e/442419/6421e2acb1bd/jcinvest00096-0016-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b58e/442419/0fd6e04f9c93/jcinvest00096-0016-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b58e/442419/6421e2acb1bd/jcinvest00096-0016-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b58e/442419/0fd6e04f9c93/jcinvest00096-0016-b.jpg

相似文献

1
Cystic fibrosis.囊性纤维化
J Clin Invest. 1987 Dec;80(6):1523-6. doi: 10.1172/JCI113237.
2
Studies of the function of the sweat gland, parotid gland and the pancreas in chronic bronchitis and heterozygous mucoviscidosis or cystic fibrosis.慢性支气管炎及杂合性黏液黏稠症或囊性纤维化患者汗腺、腮腺及胰腺功能的研究。
Ann Med Intern Fenn. 1967;56(50):Suppl 50:9-48.
3
[Mucoviscidosis in negative BM-meconium test].[骨髓-胎粪检测结果为阴性的黏液黏稠症]
Helv Paediatr Acta. 1974 Apr;29(1):51-4.
4
Pathogenesis of cystic fibrosis.囊性纤维化的发病机制。
Lancet. 1993 Apr 24;341(8852):1065-9. doi: 10.1016/0140-6736(93)92422-p.
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[Changes in the pancreatic and respiratory functions in cystic fibrosis. The influence of the time of the evolution of the disease].[囊性纤维化患者胰腺和呼吸功能的变化。疾病进展时间的影响]
Med Clin (Barc). 1993 Nov 20;101(17):650-2.
6
Modifier genetics: cystic fibrosis.修饰基因学：囊性纤维化
Annu Rev Genomics Hum Genet. 2005;6:237-60. doi: 10.1146/annurev.genom.6.080604.162254.
7
Biochemical studies of sweat secretion in cystic fibrosis.囊性纤维化中汗液分泌的生化研究。
Proc R Soc Med. 1968 Mar;61(3):297-300. doi: 10.1177/003591576806100343.
8
[Study of the sweating function in 2 cases of adult mucoviscidosis].[2例成人黏液黏稠病出汗功能的研究]
Schweiz Med Wochenschr. 1968 Sep 7;98(36):1388-92.
9
General discussion. Sweat--"First sample phenomenon".
Ciba Found Study Group. 1968;32:132-45.
10
[Efficiency of the respiratory system in children with cystic fibrosis].[囊性纤维化患儿呼吸系统的效率]
Pediatr Pol. 1973 May;48(5):551-6.

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End-stage cystic fibrosis lung disease is characterised by a diverse inflammatory pattern: an immunohistochemical analysis.终末期囊性纤维化肺病的特征是炎症模式多样：一项免疫组织化学分析。
Respir Res. 2017 Jan 10;18(1):10. doi: 10.1186/s12931-016-0489-2.
2
Communication-Microelectrode Detection of Cholesterol Efflux from the Human Buccel Mucosa.基于通信微电极检测人颊黏膜胆固醇流出情况
J Electrochem Soc. 2016;163(8):B453-B455. doi: 10.1149/2.1001608jes. Epub 2016 Jun 7.
3
Ibuprofen regulation of microtubule dynamics in cystic fibrosis epithelial cells.

本文引用的文献

1
Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis.囊性纤维化患者呼吸上皮细胞跨膜生物电位差增加。
N Engl J Med. 1981 Dec 17;305(25):1489-95. doi: 10.1056/NEJM198112173052502.
2
Relative ion permeability of normal and cystic fibrosis nasal epithelium.正常和囊性纤维化鼻上皮的相对离子通透性。
J Clin Invest. 1983 May;71(5):1410-7. doi: 10.1172/jci110894.
3
Higher bioelectric potentials due to decreased chloride absorption in the sweat glands of patients with cystic fibrosis.囊性纤维化患者汗腺中氯化物吸收减少导致生物电位升高。
布洛芬对囊性纤维化上皮细胞微管动力学的调节作用
Am J Physiol Lung Cell Mol Physiol. 2016 Aug 1;311(2):L317-27. doi: 10.1152/ajplung.00126.2016. Epub 2016 Jun 17.
4
Inhaled hypertonic saline for cystic fibrosis: Reviewing the potential evidence for modulation of neutrophil signalling and function.吸入高渗盐水治疗囊性纤维化：回顾调节中性粒细胞信号传导和功能的潜在证据。
World J Crit Care Med. 2015 Aug 4;4(3):179-91. doi: 10.5492/wjccm.v4.i3.179.
5
New insights into the genetic basis of infertility.不孕不育遗传基础的新见解。
Appl Clin Genet. 2014 Dec 1;7:235-43. doi: 10.2147/TACG.S40809. eCollection 2014.
6
Cystic fibrosis-related diabetes: from CFTR dysfunction to oxidative stress.囊性纤维化相关糖尿病：从囊性纤维化跨膜传导调节因子功能障碍到氧化应激
Clin Biochem Rev. 2009 Nov;30(4):153-77.
7
Modulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial cultures.在原代人呼吸道上皮细胞培养物中调节 CFTR 的内吞运输和顶端稳定性。
Am J Physiol Lung Cell Mol Physiol. 2010 Mar;298(3):L304-14. doi: 10.1152/ajplung.00016.2009. Epub 2009 Dec 11.
8
Novel genetic determinants of low-level aminoglycoside resistance in Pseudomonas aeruginosa.铜绿假单胞菌中低水平氨基糖苷类耐药性的新型遗传决定因素。
Antimicrob Agents Chemother. 2008 Dec;52(12):4213-9. doi: 10.1128/AAC.00507-08. Epub 2008 Sep 29.
9
Neutrophil-derived Oxidants and Proteinases as Immunomodulatory Mediators in Inflammation.中性粒细胞衍生的氧化剂和蛋白酶作为炎症中的免疫调节介质。
Mediators Inflamm. 1994;3(4):257-73. doi: 10.1155/S0962935194000360.
10
Mechanisms of lipid malabsorption in Cystic Fibrosis: the impact of essential fatty acids deficiency.囊性纤维化中脂质吸收不良的机制：必需脂肪酸缺乏的影响。
Nutr Metab (Lond). 2005 May 3;2(1):11. doi: 10.1186/1743-7075-2-11.
N Engl J Med. 1983 May 19;308(20):1185-9. doi: 10.1056/NEJM198305193082002.
4
Chloride impermeability in cystic fibrosis.囊性纤维化中的氯离子不渗透性
Nature. 1983 Feb 3;301(5899):421-2. doi: 10.1038/301421a0.
5
The carbohydrate content of IgG from patients with cystic fibrosis.囊性纤维化患者免疫球蛋白G的碳水化合物含量。
Pediatr Res. 1983 Dec;17(12):931-5. doi: 10.1203/00006450-198312000-00001.
6
Defective beta adrenergic response of cystic fibrosis sweat glands in vivo and in vitro.囊性纤维化汗腺在体内和体外的β肾上腺素能反应缺陷。
J Clin Invest. 1984 Jun;73(6):1763-71. doi: 10.1172/JCI111385.
7
Abnormal ion permeation through cystic fibrosis respiratory epithelium.异常离子透过囊性纤维化呼吸道上皮。
Science. 1983 Sep 9;221(4615):1067-70. doi: 10.1126/science.6308769.
8
Improved patch-clamp techniques for high-resolution current recording from cells and cell-free membrane patches.用于从细胞和无细胞膜片进行高分辨率电流记录的改进膜片钳技术。
Pflugers Arch. 1981 Aug;391(2):85-100. doi: 10.1007/BF00656997.
9
Fibroblasts from patients with I-cell disease and pseudo-Hurler polydystrophy are deficient in uridine 5'-diphosphate-N-acetylglucosamine: glycoprotein N-acetylglucosaminylphosphotransferase activity.患有I型细胞病和假胡尔勒氏多营养不良症患者的成纤维细胞缺乏尿苷5'-二磷酸-N-乙酰葡糖胺：糖蛋白N-乙酰葡糖胺磷酸转移酶活性。
J Clin Invest. 1981 May;67(5):1574-9. doi: 10.1172/jci110189.
10
Development of a quantitative immunoassay for the cystic fibrosis gene.
Lancet. 1980 Feb 16;1(8164):330-1. doi: 10.1016/s0140-6736(80)90882-x.