LE WE PMID CA
Cystic fibrosis2981Zystische Fibrose

Airways (Clearance)

Alginate

Antiinflammatory compounds

Antimicrobial peptides

Asthma (BASKET)

Aztreonam

BOOP

Cell stress (ER stress)

Cell volume

CFTR ABCC7

Constipation

COPD

Cystic fibrosis

Deoxyribonuclease dornase

Diabetes mellitus (BASKET)

Inhalers

Interferon gamma

Laboratory medicine

Liver (Transplantation)

Lung (Development)

Lung (Physiology)

Lung (Transplantation)

Lung and bronchial system (Malformation)

Macrolide

Mucolytic agents

Nucleoside triphosphate diphopshohydrolases

Pancreas (Neoplasia)

Proline glycine proline PGP

Proteasome (BASKET)

Pseudomonas aeruginosa

Pulmonary edema

Sweat testing

TRPV4

1998  
1
Pathology of pancreatic and intestinal disorders in cystic fibrosis.
[9709387] J R Soc Med 91 Suppl 34(-): 40-9 (1998)
2008  
2
Review of recombinant human deoxyribonuclease (rhDNase) in the management of patients with cystic fibrosis.
[19707442] Biologics 2(4): 611-7 (2008)
1996  
3
Microbial pathogenesis in cystic fibrosis: mucoid Pseudomonas aeruginosa and Burkholderia cepacia.
[8840786] Microbiol Rev 60(3): 539-74 (1996)
2004  
4
Infection control in cystic fibrosis.
[14726455] Clin Microbiol Rev 17(1): 57-71 (2004)
2002  
5
Lung infections associated with cystic fibrosis.
[11932230] Clin Microbiol Rev 15(2): 194-222 (2002)
1991  
6
Microbiology of airway disease in patients with cystic fibrosis.
[1900735] Clin Microbiol Rev 4(1): 35-51 (1991)
2007  
7
Microbial ecology of the cystic fibrosis lung.
[17379702] Microbiology 153(Pt 4): 917-23 (2007)
2005  
8
Understanding the control of Pseudomonas aeruginosa alginate synthesis and the prospects for management of chronic infections in cystic fibrosis.
[15813726] Mol Microbiol 56(2): 309-22 (2005)
2009  
9
Cystic fibrosis and innate immunity: how chloride channel mutations provoke lung disease.
[19068098] Cell Microbiol 11(2): 208-16 (2009)
2010  
10
Proline-Glycine-Proline (PGP) and High Mobility Group Box Protein-1 (HMGB1): Potential Mediators of Cystic Fibrosis Airway Inflammation.
[20448817] Open Respir Med J 4(-): 32-8 (2010)
1999  
11
Inflammation and CFTR: might neutrophils be the key in cystic fibrosis?
[10704083] Mediators Inflamm 8(1): 7-11 (1999)
1992  
12
Cystic fibrosis transmembrane conductance regulator and the etiology and pathogenesis of cystic fibrosis.
[1378801] FASEB J 6(10): 2775-82 (1992)
1995  
13
Liver disease in cystic fibrosis.
[7539244] Arch Dis Child 72(4): 281-4 (1995)
1965  
14
2005  
15
Diabetes: a major co-morbidity of cystic fibrosis.
[16142013] Diabetes Metab 31(3 Pt 1): 221-32 (2005)
2004  
16
New concepts of the pathogenesis of cystic fibrosis lung disease.
[14738247] Eur Respir J 23(1): 146-58 (2004)
2003  
17
Chronic pancreatitis and cystic fibrosis.
[12651880] Gut 52 Suppl 2(-): ii31-41 (2003)
1987  
18
Cystic fibrosis--a gastroenterological cornucopia.
[3322958] Gut 28(11): 1531-47 (1987)
2005  
19
Guide to bone health and disease in cystic fibrosis.
[15613415] J Clin Endocrinol Metab 90(3): 1888-96 (2005)
1996  
20
Fibrosing colonopathy in children with cystic fibrosis.
[8731700] Postgrad Med J 72(845): 129-30 (1996)
1984  
21
2009  
22
Mechanisms of the noxious inflammatory cycle in cystic fibrosis.
[19284656] Respir Res 10(-): 23 (2009)
2003  
23
State of the art: why do the lungs of patients with cystic fibrosis become infected and why can't they clear the infection?
[14511398] Respir Res 4(-): 8 (2003)
2007  
24
Cystic fibrosis in children and young adults: findings on routine abdominal sonography.
[17579157] AJR Am J Roentgenol 189(1): 89-99 (2007)
2006  
25
Review of the abdominal manifestations of cystic fibrosis in the adult patient.
[16702447] Radiographics 26(3): 679-90 (2006)
2000  
26
Hepatobiliary and pancreatic manifestations of cystic fibrosis: MR imaging appearances.
[10835127] Radiographics 20(3): 767-77 (2000)
2009  
27
Airway clearance therapy in cystic fibrosis patients.
[19848046] Acta Biomed 80(2): 102-6 (2009)
2009  
28
Defective organellar acidification as a cause of cystic fibrosis lung disease: reexamination of a recurring hypothesis.
[19329540] Am J Physiol Lung Cell Mol Physiol 296(6): L859-67 (2009)
2007  
29
2005  
30
The role of liver transplantation in cystic fibrosis re-examined.
[16315291] Liver Transpl 11(12): 1463-5 (2005)
2003  
31
Cystic fibrosis: current trends in respiratory care.
[12667274] Respir Care 48(3): 234-45; discussion 246-7 (2003)
2008  
32
Practice of noninvasive ventilation for cystic fibrosis: a nationwide survey in France.
[18957151] Respir Care 53(11): 1482-9 (2008)
2009  
33
Evaluating the evidence for airway-clearance therapy in cystic fibrosis.
[19327179] Respir Care 54(4): 458-60 (2009)
2009  
34
Cystic fibrosis and the respiratory therapist: a 50-year perspective.
[19393103] Respir Care 54(5): 587-94 (2009)
2009  
35
Cystic fibrosis: pathogenesis and future treatment strategies.
[19393104] Respir Care 54(5): 595-605 (2009)
2009  
36
Monitoring respiratory disease severity in cystic fibrosis.
[19393105] Respir Care 54(5): 606-17 (2009)
2009  
37
Pulmonary complications of cystic fibrosis.
[19393106] Respir Care 54(5): 618-27 (2009)
2009  
38
Bugs, biofilms, and resistance in cystic fibrosis.
[19393107] Respir Care 54(5): 628-40 (2009)
2009  
39
Infection control in cystic fibrosis: cohorting, cross-contamination, and the respiratory therapist.
[19393108] Respir Care 54(5): 641-57 (2009)
2009  
40
Aerosol antibiotics in cystic fibrosis.
[19393109] Respir Care 54(5): 658-70 (2009)
2009  
41
Mucus, phlegm, and sputum in cystic fibrosis.
[19467160] Respir Care 54(6): 726-32; discussion 732 (2009)
2009  
42
New aerosol delivery devices for cystic fibrosis.
[19467162] Respir Care 54(6): 754-67; discussion 767-8 (2009)
2009  
43
Respiratory care of the hospitalized patient with cystic fibrosis.
[19467163] Respir Care 54(6): 769-75; discussion 775-6 (2009)
2009  
44
2007  
45
Cystic fibrosis: lessons from the sweat gland.
[17557942] Physiology (Bethesda) 22(-): 212-25 (2007)
2010  
46
Chronic macrolide therapy in inflammatory airways diseases.
[21051396] Chest 138(5): 1202-12 (2010)
2007  
47
Lung development and adult lung diseases.
[17699136] Chest 132(2): 651-6 (2007)
2007  
48
Special considerations for patients with cystic fibrosis undergoing lung transplantation.
[17426231] Chest 131(4): 1224-31 (2007)
2008  
49
Airway inflammation in cystic fibrosis.
[18252915] Chest 133(2): 489-95 (2008)
2009  
50
Cystic fibrosis genotype and assessing rates of decline in pulmonary status.
[19952026] Radiology 253(3): 813-21 (2009)
2010  
51
Role of endoplasmic reticulum stress in cystic fibrosis-related airway inflammatory responses.
[21030518] Proc Am Thorac Soc 7(6): 387-94 (2010)
2009  
52
Lung transplantation for cystic fibrosis.
[20008865] Proc Am Thorac Soc 6(8): 619-33 (2009)
2010  
53
Proteasomal regulation of pulmonary fibrosis.
[20160152] Proc Am Thorac Soc 7(1): 77-83 (2010)
2007  
54
Acute renal failure in people with cystic fibrosis.
[17536029] Thorax 62(6): 472-3 (2007)
2007  
55
Exacerbations in cystic fibrosis: 2 . prevention.
[17687099] Thorax 62(8): 723-32 (2007)
2007  
56
Pulmonary exacerbations in cystic fibrosis and bronchiectasis.
[17387213] Thorax 62(4): 288-90 (2007)
2007  
57
Exacerbations in cystic fibrosis. 1: Epidemiology and pathogenesis.
[17387214] Thorax 62(4): 360-7 (2007)
2006  
58
Diagnosing CF: sweat, blood and years.
[16807389] Thorax 61(7): 556-7 (2006)
2006  
59
Cystic fibrosis: terminology and diagnostic algorithms.
[16384879] Thorax 61(7): 627-35 (2006)
2008  
60
2010  
61
The changing microbial epidemiology in cystic fibrosis.
[20375354] Clin Microbiol Rev 23(2): 299-323 (2010)
2006  
62
Immunopathophysiologic mechanisms of cystic fibrosis lung disease.
[16450752] Isr Med Assoc J 8(1): 44-8 (2006)
2010  
63
2003  
64
Pathophysiology and management of pulmonary infections in cystic fibrosis.
[14555458] Am J Respir Crit Care Med 168(8): 918-51 (2003)
2003  
65
Emergence of nontuberculous mycobacteria as pathogens in cystic fibrosis.
[12623856] Am J Respir Crit Care Med 167(6): 810-2 (2003)
2007  
66
Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health.
[17761616] Am J Respir Crit Care Med 176(10): 957-69 (2007)
2009  
67
Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations.
[19729669] Am J Respir Crit Care Med 180(9): 802-8 (2009)
2007  
68
2008  
69
Inhibition of airway proteases in cystic fibrosis lung disease.
[18757703] Eur Respir J 32(3): 783-95 (2008)
2008  
70
Cystic fibrosis in adults: diagnostic and therapeutic aspects.
[18345455] J Bras Pneumol 34(2): 107-17 (2008)
2008  
71
Cystic fibrosis-related dyslipidemia.
[19009217] J Bras Pneumol 34(10): 829-37 (2008)
2010  
72
Sweat testing: can the conductivity analysis take the place of the classic Gibson and Cooke technique?
[20361128] J Pediatr (Rio J) 86(2): 89-91 (2010)
2008  
73
Effective improvement of cystic fibrosis care by application of elementary measures: essential lessons not only for developing countries.
[18923794] J Pediatr (Rio J) 84(5): 383-5 (2008)
2008  
74
Improvements in lung function of a pediatric cystic fibrosis population in a developing country.
[18923789] J Pediatr (Rio J) 84(5): 403-9 (2008)
2008  
75
Hyperinflammation in airways of cystic fibrosis patients: what's new?
[18598217] Expert Rev Mol Diagn 8(4): 359-63 (2008)
2010  
76
Cystic fibrosis: exploiting its genetic basis in the hunt for new therapies.
[19903491] Pharmacol Ther 125(2): 219-29 (2010)
2007  
77
Microvascular complications in cystic fibrosis-related diabetes.
[17322485] Diabetes Care 30(5): 1056-61 (2007)
2008  
78
Front-runners for pharmacotherapeutic correction of the airway ion transport defect in cystic fibrosis.
[18468487] Curr Opin Pharmacol 8(3): 292-9 (2008)
2002  
79
2002  
80
New therapeutic approaches for cystic fibrosis lung disease.
[12216276] J R Soc Med 95 Suppl 41(-): 58-67 (2002)
2004  
81
Physiotherapy in infants and young children with cystic fibrosis: current practice and future developments.
[15239290] J R Soc Med 97 Suppl 44(-): 8-25 (2004)
2004  
82
Understanding cystic-fibrosis-related diabetes: best thought of as insulin deficiency?
[15239291] J R Soc Med 97 Suppl 44(-): 26-35 (2004)
2005  
83
The role of abdominal ultrasound in the diagnosis, staging and management of cystic fibrosis liver disease.
[16025764] J R Soc Med 98 Suppl 45(-): 17-27 (2005)
2005  
84
Musculoskeletal and neuromuscular interventions: a physical approach to cystic fibrosis.
[16025768] J R Soc Med 98 Suppl 45(-): 55-66 (2005)
2009  
85
New microbicidal functions of tracheal glands: defective anti-infectious response to Pseudomonas aeruginosa in cystic fibrosis.
[19399182] PLoS One 4(4): e5357 (2009)
2004  
86
Swelling-activated Ca2+ entry via TRPV4 channel is defective in cystic fibrosis airway epithelia.
[15489228] J Biol Chem 279(52): 54062-8 (2004)
2007  
87
Patterns of GI disease in adulthood associated with mutations in the CFTR gene.
[17446304] Gut 56(8): 1153-63 (2007)
2007  
88
Risk of pancreatic cancer in patients with cystic fibrosis.
[17698876] Gut 56(9): 1327-8 (2007)
2011  
89
Aztreonam (for inhalation solution) for the treatment of chronic lung infections in patients with cystic fibrosis: an evidence-based review.
[22022288] Core Evid 6(-): 59-66 (2011)
2011  
90
Intestinal obstruction syndromes in cystic fibrosis: meconium ileus, distal intestinal obstruction syndrome, and constipation.
[21384135] Curr Gastroenterol Rep 13(3): 265-70 (2011)
2007  
91
Too much salt, too little soda: cystic fibrosis.
[17700961] Sheng Li Xue Bao 59(4): 397-415 (2007)
2008  
92
Newborn screening for cystic fibrosis: a lesson in public health disparities.
[18718257] J Pediatr 153(3): 308-13 (2008)
2008  
93
Update on gene modifiers in cystic fibrosis.
[18812833] Curr Opin Pulm Med 14(6): 559-66 (2008)
2007  
94
Pseudomonas aeruginosa lipopolysaccharide: a major virulence factor, initiator of inflammation and target for effective immunity.
[17466590] Int J Med Microbiol 297(5): 277-95 (2007)
2004  
95
Newborn screening for cystic fibrosis: evaluation of benefits and risks and recommendations for state newborn screening programs.
[15483524] MMWR Recomm Rep 53(RR-13): 1-36 (2004)
2006  
96
E-NTPDases in human airways: Regulation and relevance for chronic lung diseases.
[18404479] Purinergic Signal 2(2): 399-408 (2006)
2006  
97
The ABC protein turned chloride channel whose failure causes cystic fibrosis.
[16554808] Nature 440(7083): 477-83 (2006)
2010  
98
Protein processing and inflammatory signaling in Cystic Fibrosis: challenges and therapeutic strategies.
[20205681] Curr Mol Med 10(1): 82-94 (2010)
2010  
99
Cystic fibrosis-related diabetes in childhood.
[20190536] Horm Res Paediatr 73(1): 15-24 (2010)
2007  
100
The limitations of sweat electrolyte reference intervals for the diagnosis of cystic fibrosis: a systematic review.
[17687417] Clin Biochem Rev 28(2): 60-76 (2007)
PMC   
101
Cystic fibrosis chronic rhinosinusitis: a comprehensive review.
[24119602] Am J Rhinol Allergy 27(5):387-95 (2013)
PMC   
102
Clinical insights from metagenomic analysis of sputum samples from patients with cystic fibrosis.
[24478471] J Clin Microbiol 52(2):425-37 (2014)
PMC   
103
Metagenomics and metatranscriptomics: Windows on CF-associated viral and microbial communities.
[22951208] J Cyst Fibros (): (2012)
PMC   
104
Cystic fibrosis bronchial epithelial cells are lipointoxicated by membrane palmitate accumulation.
[24586495] PLoS One 9(2):e89044 (2014)
PMC   
105
Improvement of chloride transport defect by gonadotropin-releasing hormone (GnRH) in cystic fibrosis epithelial cells.
[24586461] PLoS One 9(2):e88964 (2014)
PMC   
106
Treatment of pseudomonas and Staphylococcus bronchopulmonary infection in patients with cystic fibrosis.
[24489509] ScientificWorldJournal 2013():645653 (2013)
PMC   
107
Airway, but not serum or urinary, levels of YKL-40 reflect inflammation in early cystic fibrosis lung disease.
[24576297] BMC Pulm Med 14():28 (2014)
PMC   
108
Body composition and pulmonary function in cystic fibrosis.
[24783186] Front Pediatr 2():33 (2014)
PMC   
109
Patients with cystic fibrosis have inducible IL-17+IL-22+ memory cells in lung draining lymph nodes.
[22795370] J Allergy Clin Immunol 131(4):1117-29, 1129.e1-5 (2013)
PMC   
110
Early eradication of Pseudomonas aeruginosa in patients with cystic fibrosis.
[20692633] Paediatr Respir Rev 11(3):177-84 (2010)
PMC   
111
Amiloride-sensitive fluid resorption in NCI-H441 lung epithelia depends on an apical Cl(-) conductance.
[24744880] Physiol Rep 2(1):e00201 (2014)
PMC   
112
Shifting paradigms of nontuberculous mycobacteria in cystic fibrosis.
[24725650] Respir Res 15():41 (2014)

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