scholarly journals Yield From Flexible Bronchoscopy in Pediatric Cystic Fibrosis Patients

2008 ◽  
Vol 15 (4) ◽  
pp. 240-246 ◽  
Author(s):  
Ruben Boogaard ◽  
Johan C. de Jongste ◽  
Maarten H. Lequin ◽  
Annick S. Devos ◽  
Harm A. W. M. Tiddens ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Flexible Bronchoscopy

Flexible bronchoscopy in children and adolescents with cystic fibrosis

2018 ◽  
Author(s):  
Uros Krivec ◽  
Marina Praprotnik ◽  
Malena Aldeco ◽  
Dusanka Lepej ◽  
Ana Kotnik Pirs ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Children And Adolescents ◽  
Flexible Bronchoscopy

scholarly journals Therapeutic flexible bronchoscopy in child with cystic fibrosis

2008 ◽  
Vol 5 (2) ◽  
pp. 75-77
Author(s):  
Amina Selimović
Keyword(s):  
Cystic Fibrosis ◽  
Middle Lobe ◽  
Flexible Bronchoscopy ◽  
Sweat Glands ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Laboratory Findings ◽  
X Ray ◽  
Chest X Ray ◽  
Regulator Genes

The report deals with the case of a 10-year-old girl with chronic cystic fibrosis. She has been repeatedly treated at the hospital. She has been hospitalized due to respiratory deterioration. Cystic fibrosis is a rare disease, inherited autosomaly recessively, but is very complex in terms of diagnostic and treatment (2). The diagnosis is confirmed based on a clinical picture of the child, measure of Chloride in the sweat, chest X-ray, CT thorax, laboratory findings--genetic confirmation CFTR ( cystic fibrosis transmembrane conductance regulator) genes (3), which result in the production of hyper-viscous mucus and chloride malabsorption in the sweat glands ducts (5,6). Bronchial thickening and plugging and ring shadows suggesting bronchiectasis, segmental or lobar atelectasis are often. Computer tomography of the chest can be used to detect and localize thickening of bronchial airways walls, mucus plugging, hyperinflation and early bronchieactasiae. Pulmonary therapy: the object is to clear secretions from airways and to control infection (7). The diagnosis is originally set when she was 4 years old. She is now admitted due to a deterioration of the main disease. Day before admission in the hospital had a higher bodily temperature, cough and difficult breathing. She already treated conservatively (Ceftazidim, Ceftriakson, Kloksacillin) Since the girl is a chronic patient with bronchiectasie chronic walls of bronchi changes full of the mucus, who is not responding to conservative treatment (antibiotics), therapeutic and diagnostic flexible bronchoscopy had to be performed, resulting in a gram-negative bacteria pseudomonas aeruginosa--a typical bacteria for chronically sick C. F. PATIENT: A pseudomonas therapy was prescribed according to the sensitive antibiogram, during which bronchoscopy was given locally on changes mucous pulmozyme and garamycin. Flexible bronchoscopy was performed as therapeutic. Local bronchoscopy findings:by aspiration of tracheo-bronchal truncus it was found hyperemia and a lot of mucous sticky secretion inside of tracheobronchal tree, especially middle lobe right side, lingual and basals part of the lungs. It was performed broncho-alveolar lavage and given steroids on the place of changed inflamed mucous membrane of the bronchi. It was also given pulmozyme to destroid mucous and make better spontaneously expectorations. Control chest x ray was performed and it was better.

scholarly journals 491 Flexible bronchoscopy and oesophageal pH monitoring in children newly diagnosed with Cystic Fibrosis

2006 ◽  
Vol 5 ◽  
pp. S108
Author(s):  
T. Hilliard ◽  
S. Sukhani ◽  
J. Francis ◽  
M. Rosentha ◽  
I. Balfour-Lynn ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Ph Monitoring ◽  
Flexible Bronchoscopy ◽  
Newly Diagnosed

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

Heat stable toxin of E. coli (STa) stimulates duodenal mucosal bicarbonate secretion in cystic fibrosis knockout mice

2001 ◽  
Vol 120 (5) ◽  
pp. A137-A137
Author(s):  
D CHILDS ◽  
D CROMBIE ◽  
V PRATHA ◽  
Z SELLERS ◽  
D HOGAN ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Knockout Mice ◽  
Bicarbonate Secretion ◽  
E Coli ◽  
Heat Stable
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