Study of lung involvement in patients with cystic fibrosis

2000 ◽  
Vol 76 (4) ◽  
pp. 295-9 ◽  
Author(s):  
Eduardo C. Dornelas ◽  
Maria I.M. Fernandes ◽  
Lívia C. Galvão ◽  
Geruza A. Silva
Keyword(s):  
Cystic Fibrosis ◽  
Lung Involvement

High Resolution CT in Children with Cystic Fibrosis

1992 ◽  
Vol 33 (6) ◽  
pp. 548-553 ◽  
Author(s):  
R. Stiglbauer ◽  
H. Schurawitzki ◽  
I. Eichler ◽  
M. Götz
Keyword(s):  
Cystic Fibrosis ◽  
High Resolution ◽  
Motion Artifacts ◽  
Wall Thickening ◽  
Lung Involvement ◽  
Bronchial Wall ◽  
High Resolution Ct ◽  
Valuable Method ◽  
Frequent Finding ◽  
Pulmonary Changes

High resolution CT (HRCT) was performed in 24 children (median age 57.9 months) suffering from cystic fibrosis (CF). In 23 patients (one examination unacceptable because of motion artifacts) the most frequent finding was bronchial wall thickening, shown in 21 patients (91%), followed by bronchiectasis in 15 patients (65%). Less frequent findings were mucus plugging and patchy consolidations, which could be demonstrated in 11 patients each (48%). Findings were classified using a CT scoring system and including only irreversible pulmonary changes; a statistically significant correlation with lung function tests (FEV1/FVC; MEF50) could be established. HRCT to date seems to be the most valuable method to determine extent and severity of lung involvement in children with CF and should therefore be routinely used for the staging of this disease.

Lung involvement, the ΔF508 mutation and DNA haplotype analysis in cystic fibrosis

1992 ◽  
Vol 88 (6) ◽  
pp. 639-641 ◽  
Author(s):  
F. Santamaria ◽  
D. Salvatore ◽  
O. Castiglione ◽  
V. Raia ◽  
G. de Ritis ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Haplotype Analysis ◽  
Lung Involvement

scholarly journals Risk factors for Pseudomonas aeruginosa colonization in non-cystic fibrosis bronchiectasis and clinical implications

2021 ◽  
Author(s):  
Wang Chun Kwok ◽  
James Chung-man Ho ◽  
Chi Chun Terence Tam ◽  
Sau Man Mary Ip ◽  
David Chi-Leung Lam
Keyword(s):  
Risk Factors ◽  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Cross Sectional Study ◽  
Chinese Patients ◽  
Rapid Decline ◽  
Clinical Implications ◽  
Lung Involvement ◽  
Cross Sectional ◽  
Poor Outcomes

Abstract Background: Pseudomonas aeruginosa is one of the commonest bacteria colonizing the airway in patients with non-cystic fibrosis bronchiectasis. Pseudomonas aeruginosa colonization is associated with poor outcomes in patients with bronchiectasis, including rapid decline in lung function, exacerbation frequency and hospitalization.Methods: A cross-sectional study in Queen Mary Hospital, Hong Kong that included 350 Chinese patients with non-cystic fibrosis bronchiectasis to investigate the risk factors for Pseudomonas aeruginosa colonization and clinical implications on disease outcomes.Discussion: Pseudomonas aeruginosa colonization was more commonly found in patients with longer duration of bronchiectasis and those on proton pump inhibitors (PPIs) with adjusted ORs of 1.066 (95% CI = 1.036 – 1.096, p < 0.001) and 2.815 (95% CI = 1.307 – 6.064, p = 0.008) respectively. Patients with Pseudomonas aeruginosa colonization have more extensive lung involvement and higher risks of exacerbation requiring hospitalization with adjusted ORs of 2.445 (95% CI = 1.283 – 4.657, p = 0.007) and 2.745 (95% CI = 1.012 – 7.449, p = 0.047) respectively. Pseudomonas aeruginosa colonization is more common among patients with longer duration of bronchiectasis and those on PPI. Pseudomonas aeruginosa colonization is associated with more extensive lung involvement and higher risks of exacerbation requiring hospitalization.

scholarly journals Identification of a D579G homozygote cystic fibrosis patient with pancreatic sufficiency and minor lung involvement

1999 ◽  
Vol 13 (2) ◽  
pp. 173-173 ◽  
Author(s):  
Luigi Picci ◽  
Marilena Cameran ◽  
Paola Olante ◽  
Franco Zacchello ◽  
Maurizio Scarpa
Keyword(s):  
Cystic Fibrosis ◽  
Cystic Fibrosis Patient ◽  
Lung Involvement

scholarly journals Risk factors for Pseudomonas aeruginosa colonization in non-cystic fibrosis bronchiectasis and clinical implications

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Wang Chun Kwok ◽  
James Chung Man Ho ◽  
Terence Chi Chun Tam ◽  
Mary Sau Man Ip ◽  
David Chi Leung Lam
Keyword(s):  
Risk Factors ◽  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Cross Sectional Study ◽  
Chinese Patients ◽  
Rapid Decline ◽  
Clinical Implications ◽  
Lung Involvement ◽  
Cross Sectional ◽  
Poor Outcomes

Abstract Background Pseudomonas aeruginosa is one of the commonest bacteria colonizing the airway in patients with non-cystic fibrosis bronchiectasis. Pseudomonas aeruginosa colonization is associated with poor outcomes in patients with bronchiectasis, including rapid decline in lung function, exacerbation frequency and hospitalization. Methods A cross-sectional study in Queen Mary Hospital, Hong Kong that included 350 Chinese patients with non-cystic fibrosis bronchiectasis to investigate the risk factors for Pseudomonas aeruginosa colonization and clinical implications on disease outcomes. Discussions Pseudomonas aeruginosa colonization was more commonly found in patients with longer duration of bronchiectasis and those on proton pump inhibitors (PPIs) with adjusted ORs of 1.066 (95% CI = 1.036–1.096, p < 0.001) and 2.815 (95% CI = 1.307–6.064, p = 0.008) respectively. Patients with Pseudomonas aeruginosa colonization have more extensive lung involvement and higher risks of exacerbation requiring hospitalization with adjusted ORs of 2.445 (95% CI = 1.283–4.657, p = 0.007) and 2.745 (95% CI = 1.012–7.449, p = 0.047) respectively. Pseudomonas aeruginosa colonization is more common among patients with longer duration of bronchiectasis and those on PPI. Pseudomonas aeruginosa colonization is associated with more extensive lung involvement and higher risks of exacerbation requiring hospitalization.

scholarly journals The Evaluation of Lung Involvement in Patients with Cystic Fibrosis by Using Mediastinal Magnetic Resonance Imaging

2019 ◽  
Vol 20 (-1) ◽  
pp. 55-55
Author(s):  
Gokcen Dilsa Tugcu ◽  
◽  
Sanem Eryilmaz Polat ◽  
Mina Garibzadeh Hizal ◽  
Beste Karakaya Ozsezen ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cystic Fibrosis ◽  
Magnetic Resonance ◽  
Lung Involvement ◽  
Resonance Imaging

Cystic Fibrosis: CT Assessment of Lung Involvement in Children and Adults

Radiology ◽  
1999 ◽  
Vol 213 (2) ◽  
pp. 537-544 ◽  
Author(s):  
Thomas H. Helbich ◽  
Gertraud Heinz-Peer ◽  
Irmgard Eichler ◽  
Patrick Wunderbaldinger ◽  
Manfred Götz ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Involvement

Pregnancy in cystic fibrosis of the pancreas

JAMA ◽  
1966 ◽  
Vol 195 (12) ◽  
pp. 993-1000 ◽  
Author(s):  
R. J. Grand
Keyword(s):  
Cystic Fibrosis

Freeze-Fracture Examination of Tight Junctions of Human Eccrine Sweat Glands

1980 ◽  
Vol 38 ◽  
pp. 634-635
Author(s):  
J. V. Briggman ◽  
J. Bigelow ◽  
H. Bank ◽  
S. S. Spicer
Keyword(s):  
Cystic Fibrosis ◽  
Freeze Fracture ◽  
Sweat Glands ◽  
Hypertonic Solution ◽  
Dark Cells ◽  
Clear Cells ◽  
Junctional Complexes ◽  
Secretory Coil ◽  
Eccrine Sweat Glands ◽  
Eccrine Sweat

The prevalence of strands shown by freeze-fracture in the zonula occludens of junctional complexes is thought to correspond closely with the transepi-thelial electrical resistance and with the tightness of the junction and its obstruction to paracellular flow.1 The complexity of the network of junc¬tional complex strands does not appear invariably related to the degree of tightness of the junction, however, as rabbit ileal junctions have a complex network of strands and are permeable to lanthanum. In human eccrine sweat glands the extent of paracellular relative to transcellular flow remains unknown, both for secretion of the isotonic precursor fluid by the coil and for resorption of a hypertonic solution by the duct. The studies reported here undertook, therefore, to determine with the freeze-fracture technique the complexity of the network of ridges in the junctional complexes between cells in the secretory coil and the sweat ducts. Glands from a patient with cystic fibrosis were also examined because an alteration in junctional strands could underlie the decreased Na+ resorption by sweat ducts in this disease. Freeze-fracture replicas were prepared by standard procedures on isolated coil and duct segments of human sweat glands. Junctional complexes between clear cells, between dark cells and between clear and dark cells on the main lumen, and between clear cells on intercellular canaliculi of the coil con¬tained abundant anastomosing closely spaced strands averaging 6.4 + 0.7 (mean + SE) and 9.0 +0.5 (Fig. 1) per complex, respectively. Thus, the junctions in the intercellular canaliculi of the coil appeared comparable in complexity to those of tight epithlia. Occasional junctions exhibited, in addition, 2 to 5 widely spaced anastomosing strands in a very close network basal to the compact network. The fewer junctional complexes observed thus far between the superficial duct cells consisted on the average of 6 strands arranged in a close network and 1 to 4 underlying strands that lay widely separated from one another (Fig. 2). The duct epitelium would, thus, be judged slightly more "leaky" than the coil. Infrequent junctional complexes observed to date in the secretory coil segment of a cystic fibrosis specimen disclosed rela¬tively few closely crowded strands.

Chronic airway colonization by Penicillium emersonii in a patient with cystic fibrosis

1999 ◽  
Vol 37 (4) ◽  
pp. 291-293 ◽  
Author(s):  
B. Cimon ◽  
J. Carrere ◽  
J. P. Chazalette ◽  
J. F. Vinatier ◽  
D. Chabasse ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Airway Colonization ◽  
Chronic Airway
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