Journal articles: 'Cystic Fibrosis in adulthood'

1

Simmonds, Nicholas J. "Cystic Fibrosis Presenting in Adulthood." Clinical Pulmonary Medicine 20, no. 1 (January 2013): 1–5. http://dx.doi.org/10.1097/cpm.0b013e31827a292e.

Full text

APA, Harvard, Vancouver, ISO, and other styles

2

Hota, Partha, and Rachna Madan. "Cystic Fibrosis from Childhood to Adulthood." Radiologic Clinics of North America 58, no. 3 (May 2020): 475–86. http://dx.doi.org/10.1016/j.rcl.2019.12.003.

Full text

APA, Harvard, Vancouver, ISO, and other styles

3

Palmer, Marykay Lannon, and Laura S. Boisen. "Cystic Fibrosis and the Transition to Adulthood." Social Work in Health Care 36, no. 1 (November 12, 2002): 45–58. http://dx.doi.org/10.1300/j010v36n01_04.

Full text

APA, Harvard, Vancouver, ISO, and other styles

4

Tješić Drinković, Dorian, and Duška Tješić-Drinković. "Clinical presentations of cystic fibrosis in adulthood." Paediatria Croatica 59, no. 2 (June 26, 2015): 74–80. http://dx.doi.org/10.13112/pc.2015.12.

Full text

APA, Harvard, Vancouver, ISO, and other styles

5

Baran, E., N. Granero, B. Hendriksen, F. Butti, V. D'Ascenzo, S. Ibarra, V. Pistorio, L. Volta, and G. García. "22 Misdiagnosis of cystic fibrosis in adulthood." Journal of Cystic Fibrosis 12 (June 2013): S54. http://dx.doi.org/10.1016/s1569-1993(13)60165-4.

Full text

APA, Harvard, Vancouver, ISO, and other styles

6

Bourgani, E., C. Kosti, E. Stagaki, and F. Diamantea. "P027 Diagnosis of cystic fibrosis in adulthood." Journal of Cystic Fibrosis 19 (June 2020): S62. http://dx.doi.org/10.1016/s1569-1993(20)30364-7.

Full text

APA, Harvard, Vancouver, ISO, and other styles

7

Nick, Jerry A., and David M. Rodman. "Manifestations of cystic fibrosis diagnosed in adulthood." Current Opinion in Internal Medicine 5, no. 1 (February 2006): 68–73. http://dx.doi.org/10.1097/01.mcp.0000183052.56728.76.

Full text

APA, Harvard, Vancouver, ISO, and other styles

8

Radlovic, Nedeljko. "Cystic fibrosis." Srpski arhiv za celokupno lekarstvo 140, no. 3-4 (2012): 244–49. http://dx.doi.org/10.2298/sarh1204244r.

Full text

Abstract:

Cystic fibrosis (CF) is a multisystemic autosomal recessive disease caused by a defect in the expression of CFTR protein, i.e. chloride channel present in the apical membrane of respiratory, digestive, reproductive and sweat glands epithelium. It primarily occurs in the Caucasians, while being considerably or exceptionally rare in persons of other races. Absence, deficit or structural and functional abnormalities of CFTR protein lead to mucosal hyperconcentration in the respiratory, digestive and reproductive systems and malabsorption of chloride and sodium in the sweat glands. Thus, the clinical features of patients? with CF are predominated by respiratory, digestive and reproductive disorders, as well as the tendency to dehydration in the condition of increased sweating. Beside genotype variations, the degree of disease manifestation is also essentially influenced by various exogenous factors, such as the frequency and severity of respiratory infections, the level of aero-pollution, quality of immunoprophylaxis, patients? nutritional condition and other. Chloride concentration of over 60 mmol/L in sweat, a high level of immunoreactive chymotrypsinogen in blood and the verification of homozygous mutation of CFTR gene are the basic methods in the diagnostics of the disease. CF belongs to the group of severe and complex chronic diseases, and therefore requires multidisciplinary therapeutic approach. Owing to the improvement of healthcare provision, most patients with CF now survive into adulthood. In addition, their quality of life is also considerably improved.

APA, Harvard, Vancouver, ISO, and other styles

9

Harriss, Patrick B., Anusha Jogimahanti, Vincent Herlevic, Hrishikesh Samant, James D. Morris, and Paul Jordan. "2439 Cystic Fibrosis Liver Disease: A Curious Case of Atypical Cystic Fibrosis Diagnosed in Adulthood." American Journal of Gastroenterology 114, no. 1 (October 2019): S1350—S1351. http://dx.doi.org/10.14309/01.ajg.0000599288.13732.20.

Full text

APA, Harvard, Vancouver, ISO, and other styles

10

Phillipson, Greg. "Cystic fibrosis and reproduction." Reproduction, Fertility and Development 10, no. 1 (1998): 113. http://dx.doi.org/10.1071/r98044.

Full text

Abstract:

Cystic fibrosis (CF) is the most common autosomal recessive disease. CF and congenital bilateral absence of the vas deferens (CBAVD) share a genetic and embryological background. Since the 1960s, medical therapy to reduce the progressive obstructive lung disease and nutritional deficiencies has resulted in most CF patients reaching adulthood. With the improved life expectancy of CF patients, new issues in reproductive health and pregnancy management have arisen. Puberty is delayed, with menarche often occurring eighteen months later than the average. Almost all men with CF are azoospermic. In both CF and CBAVD, the vas deferens is absent and the seminal vesicles are often hypoplastic. Many women with CF are subfertile, and if pregnancy is achieved there is an observed increase in maternal morbidity and mortality. The understanding of the molecular basis of CF and CBAVD has evolved, with the identification of hundreds of CF gene mutations and discovery of an associated intron polymorphism of the CF gene. The concept of severe and mild mutations has been introduced to explain the severe and mild phenotype variations such as the pancreatic insufficient and pancreatic sufficient patient. This paper reviews the above issues to assist with the management of infertile couples with CF or CBAVD.

APA, Harvard, Vancouver, ISO, and other styles

11

Nespoli, P., D. Parlatano, G. Cortese, B. Messore, C. Bena, M. Chiesa, and A. Veltri. "201 CT findings in cystic fibrosis diagnosed in adulthood." Journal of Cystic Fibrosis 12 (June 2013): S99. http://dx.doi.org/10.1016/s1569-1993(13)60342-2.

Full text

APA, Harvard, Vancouver, ISO, and other styles

12

Orlandi, Richard R., and Richard H. Wiggins. "Radiological Sinonasal Findings in Adults with Cystic Fibrosis." American Journal of Rhinology & Allergy 23, no. 3 (May 2009): 307–11. http://dx.doi.org/10.2500/ajra.2009.23.3324.

Full text

Abstract:

Background Care of cystic fibrosis (CF) patients has extended the expected survival far into adulthood. The morphological changes of adult CF patients’ paranasal sinuses have not been thoroughly addressed. Methods A retrospective review was performed of computed tomography imaging of adult CF patients at an academic medical center. Developmental changes, bone sclerosis, mucoceles, and degree of inflammation (using a modified Lund-Mackay scoring system) were assessed. Results Forty-five patients were included in the study. The majority of frontal and sphenoid sinuses were either aplastic or hypoplastic. Maxillary hypoplasia was less commonly seen. Seven patients had mucoceles. Sclerosis of the sinuses was a common finding, affecting 84.4% of patients. Inflammation of the sinuses was also common, with a median modified Lund-Mackay score of 15.0 (out of a median 20 possible). Conclusion Poor development of the sinuses seen in pediatric CF patients persists into adulthood. Inflammation of the sinuses is a common finding. Bone sclerosis, likely caused by chronic inflammation, affects a large majority of these patients.

APA, Harvard, Vancouver, ISO, and other styles

13

Weeks, A. M., and M. R. Buckland. "Anaesthesia for Adults with Cystic Fibrosis." Anaesthesia and Intensive Care 23, no. 3 (June 1995): 332–38. http://dx.doi.org/10.1177/0310057x9502300310.

Full text

Abstract:

Cystic fibrosis is an autosomal recessive disorder which affects one in 2500 live births. It is a multisystem disease and has a variety of presentations. The major clinical features affect the gastrointestinal and respiratory tracts. Severe respiratory disease, diabetes and gastroesophageal reflux are common features of concern to anaesthetists. Improved care of young patients has allowed many to survive into adulthood. Lung transplantation has significantly improved the outlook for many patients. At Alfred Hospital, 74 patients with cystic fibrosis underwent 149 procedures from January 1978 to January 1994, with a mortality of 0.6% (95% CI 0.4%-0.8%). This retrospective cohort study describes the anaesthetic management and perioperative care of these patients. Most of the anaesthetics were for procedures related to cystic fibrosis but 12% were for unrelated conditions. Cystic fibrosis related procedures include diagnostic, venous access, enteral feeding procedures, treatment of complications of cystic fibrosis and lung transplantation. Despite extremely poor respiratory function, these patients can be managed with acceptably low postoperative mortality (1%). Pre- and postoperative care must be directed towards optimal clearance of viscous respiratory secretions. Procedures need to be planned so that optimal care can be given by each member of the team caring for cystic fibrosis patients.

APA, Harvard, Vancouver, ISO, and other styles

14

Huang, Ting, Caihong Li, and Daishun Liu. "Recurrent Cough and Expectoration for 10 Years: A Case Report." Global Pediatric Health 6 (January 2019): 2333794X1983372. http://dx.doi.org/10.1177/2333794x19833725.

Full text

Abstract:

Rationale. Most cases of cystic fibrosis occur in Europe, with only a few occurring in Asia. Pulmonary cystic fibrosis is not a rare disease, but in children it is a potentially life-threatening condition. Children suffering from pulmonary cystic fibrosis rarely survive to adulthood, and responses to treatment are generally poor. The most common cause of cystic fibrosis is a genetic mutation on chromosome 7. Patient concerns. A 15-year-old boy with healthy parents suffered from a recurrent cough and expectoration for nearly 10 years. Six years previously, a definitive diagnosis of pulmonary cystic fibrosis and hepatic cirrhosis was made at the Beijing Children’s Hospital. The first occurrence of hematemesis occurred 1 year ago. The main symptoms, which caused this period of hospitalization, were cough, expectoration, and hematemesis. Diagnoses. The underlying cause was finally determined to be the cystic fibrosis transmembrane conductance regulator gene (p.G970D). After genetic and sweat testing performed at the Beijing Children’s Hospital in 2012, a definitive diagnosis of cystic fibrosis was made. Interventions. The patient was administered hemostatic treatment, antibiotics, and cough relief and sputum reduction therapy. Outcomes. The patient’s condition rapidly improved and continued to remain stable, though future relapse is possible following respiratory tract infections. Lessons. This case indicates that in the case of any child that presents a recurrent cryptogenic cough and expectoration, whether accompanied by hematemesis or not, pulmonary cystic fibrosis should be considered. In order to determine underlying causes and prepare for cystic fibrosis transmembrane conductance regulator modulator therapy, genetic and sweat testing are recommended to be conducted if available.

APA, Harvard, Vancouver, ISO, and other styles

15

Opoka-Winiarska, Violetta, Szczepan Cofta, Henryk Mazurek, and Jerzy Kozielski. "Problems of patients with cystic fibrosis during transition to adulthood." Pneumonologia i Alergologia Polska 83, no. 5 (September 8, 2015): 394–400. http://dx.doi.org/10.5603/piap.2015.0064.

Full text

APA, Harvard, Vancouver, ISO, and other styles

16

Hamlett, Kim W., Mary Murphy, Risa Hayes, and Carl F. Doershuk. "Health independence and developmental tasks of adulthood in cystic fibrosis." Rehabilitation Psychology 41, no. 2 (1996): 149–60. http://dx.doi.org/10.1037/0090-5550.41.2.149.

Full text

APA, Harvard, Vancouver, ISO, and other styles

17

Anderson, A., M. Caraher, S. Johnston, K. Heslop, C. O'Brien, D. Spencer, and S. Bourke. "Transition to adulthood with cystic fibrosis: current characteristics and challenges." Journal of Cystic Fibrosis 9 (June 2010): S65. http://dx.doi.org/10.1016/s1569-1993(10)60252-4.

Full text

APA, Harvard, Vancouver, ISO, and other styles

18

Naehrlich, Lutz. "The Changing Face of Cystic Fibrosis and Its Implications for Screening." International Journal of Neonatal Screening 6, no. 3 (July 3, 2020): 54. http://dx.doi.org/10.3390/ijns6030054.

Full text

Abstract:

Early diagnosis, multidisciplinary care, and optimized and preventive treatments have changed the face of cystic fibrosis. Life expectancy has been expanded in the last decades. Formerly a pediatric disease, cystic fibrosis has reached adulthood. Mutation-specific treatments will expand treatment options and give hope for further improvement of quality of life and life expectancy. Newborn screening for CF fits perfectly into these care structures and offers the possibility of preventive treatment even before symptoms occur. Especially in countries without screening, newborn screening will fulfill that promise only with increased awareness and new care structures.

APA, Harvard, Vancouver, ISO, and other styles

19

Kerr, Colm, David Morrissy, Mary Horgan, and Barry J. Plant. "Microbial clues lead to a diagnosis of cystic fibrosis in late adulthood." BMJ Case Reports 13, no. 4 (April 2020): e233470. http://dx.doi.org/10.1136/bcr-2019-233470.

Full text

Abstract:

Cystic fibrosis (CF) is the most common life-limiting autosomal recessive genetic disorder among Caucasian populations. The majority of CF cases are diagnosed in childhood; however, increasing numbers of adults are being diagnosed with the condition. We present the case of a 65-year-old Irish woman presenting with a chronic cough and a history of recurrent respiratory tract infections. Staphylococcus aureus, Scedosporium apiospermum and Stenotrophomonas maltophilia were grown from bronchoalveolar lavage raising suspicion for CF. Sweat testing was negative; however, genetic testing revealed the presence of ∆F508 and R117H CF mutations, the latter mutation conferring a milder form of CF. The patient commenced treatment with the cystic fibrosis transmembrane conductance regulator (CFTR) potentiator medication ivacaftor to good effect. Novel CFTR potentiators and modulators have significant potential to benefit morbidity and mortality in this group. In this case, the microbiological results were key in pursuing genetic testing and diagnosing CF.

APA, Harvard, Vancouver, ISO, and other styles

20

Elborn, J. Stuart, Scott C. Bell, Susan L. Madge, Pierre-Regis Burgel, Carlo Castellani, Steven Conway, Karleen De Rijcke, et al. "Report of the European Respiratory Society/European Cystic Fibrosis Society task force on the care of adults with cystic fibrosis." European Respiratory Journal 47, no. 2 (October 9, 2015): 420–28. http://dx.doi.org/10.1183/13993003.00592-2015.

Full text

Abstract:

The improved survival in people with cystic fibrosis has led to an increasing number of patients reaching adulthood. This trend is likely to be maintained over the next decades, suggesting a need to increase the number of centres with expertise in the management of adult patients with cystic fibrosis. These centres should be capable of delivering multidisciplinary care addressing the complexity of the disease, in addition to addressing the psychological burden on patients and their families. Further issues that require attention are organ transplantation and end of life management.Lung disease in adults with cystic fibrosis drives most of the clinical care requirements, and major life-threatening complications, such as respiratory infection, respiratory failure, pneumothorax and haemoptysis, and the management of lung transplantation require expertise from trained respiratory physicians. The taskforce therefore strongly reccommends that medical leadership in multidisciplinary adult teams should be attributed to a respiratory physician adequately trained in cystic fibrosis management.The task force suggests the implementation of a core curriculum for trainees in adult respiratory medicine and the selection and accreditation of training centres that deliver postgraduate training to the standards of the HERMES programme.

APA, Harvard, Vancouver, ISO, and other styles

21

Gilljam, Marita, Lynda Ellis, Mary Corey, Julian Zielenski, Peter Durie, and D. Elizabeth Tullis. "Clinical Manifestations of Cystic Fibrosis Among Patients With Diagnosis in Adulthood." Chest 126, no. 4 (October 2004): 1215–24. http://dx.doi.org/10.1378/chest.126.4.1215.

Full text

APA, Harvard, Vancouver, ISO, and other styles

22

Willsie, Sandra K. "Clinical Manifestations of Cystic Fibrosis Among Patients With Diagnosis in Adulthood." Yearbook of Pulmonary Disease 2006 (January 2006): 46–47. http://dx.doi.org/10.1016/s8756-3452(08)70042-2.

Full text

APA, Harvard, Vancouver, ISO, and other styles

23

Sharma, N., D. Harcourt, E. Jenkinson, and A. Pearce. "WS02.6 Living with cystic fibrosis: patients’ experiences of diagnosis in adulthood." Journal of Cystic Fibrosis 19 (June 2020): S4. http://dx.doi.org/10.1016/s1569-1993(20)30176-4.

Full text

APA, Harvard, Vancouver, ISO, and other styles

24

VandenBranden, Stacy L., Ann McMullen, Michael S. Schechter, David J. Pasta, Rory L. Michaelis, Michael W. Konstan, Jeffrey S. Wagener, Wayne J. Morgan, and Susanna A. McColley. "Lung function decline from adolescence to young adulthood in cystic fibrosis." Pediatric Pulmonology 47, no. 2 (August 24, 2011): 135–43. http://dx.doi.org/10.1002/ppul.21526.

Full text

APA, Harvard, Vancouver, ISO, and other styles

25

Filkins, Laura M., Jyoti A. Graber, Daniel G. Olson, Emily L. Dolben, Lee R. Lynd, Sabin Bhuju, and George A. O'Toole. "Coculture of Staphylococcus aureus with Pseudomonas aeruginosa Drives S. aureus towards Fermentative Metabolism and Reduced Viability in a Cystic Fibrosis Model." Journal of Bacteriology 197, no. 14 (April 27, 2015): 2252–64. http://dx.doi.org/10.1128/jb.00059-15.

Full text

Abstract:

ABSTRACTThe airways of patients with cystic fibrosis are colonized with diverse bacterial communities that change dynamically during pediatric years and early adulthood.Staphylococcus aureusis the most prevalent pathogen during early childhood, but during late teens and early adulthood, a shift in microbial composition occurs leading toPseudomonas aeruginosacommunity predominance in ∼50% of adults. We developed a robust dual-bacterialin vitrococulture system ofP. aeruginosaandS. aureuson monolayers of human bronchial epithelial cells homozygous for the ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) mutation to better model the mechanisms of this interaction. We show thatP. aeruginosadrives theS. aureusexpression profile from that of aerobic respiration to fermentation. This shift is dependent on the production of both 2-heptyl-4-hydroxyquinolineN-oxide (HQNO) and siderophores byP. aeruginosa. Furthermore,S. aureus-produced lactate is a carbon source thatP. aeruginosapreferentially consumes over medium-supplied glucose. We find that initiallyS. aureusandP. aeruginosacoexist; however, over extended cocultureP. aeruginosareducesS. aureusviability, also in an HQNO- andP. aeruginosasiderophore-dependent manner. Interestingly,S. aureussmall-colony-variant (SCV) genetic mutant strains, which have defects in their electron transport chain, experience reduced killing byP. aeruginosacompared to their wild-type parent strains; thus, SCVs may provide a mechanism for persistence ofS. aureusin the presence ofP. aeruginosa. We propose that the mechanism ofP. aeruginosa-mediated killing ofS. aureusis multifactorial, requiring HQNO andP. aeruginosasiderophores as well as additional genetic, environmental, and nutritional factors.IMPORTANCEIn individuals with cystic fibrosis,Staphylococcus aureusis the primary respiratory pathogen during childhood. During adulthood,Pseudomonas aeruginosapredominates and correlates with worse patient outcome. The mechanism(s) by whichP. aeruginosaoutcompetes or killsS. aureusis not well understood. We describe anin vitrodual-bacterial species coculture system on cystic fibrosis-derived airway cells, which models interactions relevant to patients with cystic fibrosis. Further, we show that molecules produced byP. aeruginosaadditively induce a transition ofS. aureusmetabolism from aerobic respiration to fermentation and eventually lead to loss ofS. aureusviability. Elucidating the molecular mechanisms ofP. aeruginosacommunity predominance can provide new therapeutic targets and approaches to impede this microbial community transition and subsequent patient worsening.

APA, Harvard, Vancouver, ISO, and other styles

26

Gubergrits, N. B., N. Ye Monogarova, N. V. Byelyayeva, A. A. Zeynalova, P. G. Fomenko, S. A. Shpak, E. L. Bakhchevan, and A. A. Burka. "Idiopathic recurrent pancreatitis in a young woman: unexpected clue." Herald of Pancreatic Club 48, no. 3 (July 31, 2020): 54–69. http://dx.doi.org/10.33149/vkp.2020.03.08.

Full text

Abstract:

The article presents a complex clinical case of idiopathic recurrent pancreatitis in a young woman, 23 years old. The main clinical manifestation of the disease was acute, intense, recurrent abdominal pain. Despite ongoing symptomatic therapy, the patient’s condition had been worsened, and the intensity of abdominal pain increased. Adequate dosage of the enzyme replacement therapy allowed increasing the duration of remission. Differential diagnosis, additional laboratory and instrumental studies allowed determining the cause of recurrent pancreatitis and confirming the diagnosis of cystic fibrosis with a primary lesion of the pancreas. A distinctive feature of this clinical case is the diagnosis of a hereditary cystic fibrosis disease in adulthood, the absence of clinical symptoms of lungs’ involvement in the pathological process, as well as the absence of underdevelopment, malnutrition. The diagnosis of cystic fibrosis is confirmed by German specialists; currently, the patient continues treatment in a German clinic. The article presents current data on idiopathic pancreatitis, discusses a diagnostic algorithm for identification of the cause of the disease. The etiology, pathogenetic mechanisms of cystic fibrosis occurrence are considered, the known types of CFTR gene mutations, causing the development of the disease, as well as the principles of replacement enzyme therapy in patients with cystic fibrosis are described. Evidence-based medicine data are presented, revealing the efficacy and safety of taking enzyme preparations in the correction of the exocrine pancreatic insufficiency.

APA, Harvard, Vancouver, ISO, and other styles

27

Williams, Lindsey W., Eileen J. Burker, and Kelly Kazukauskas. "Cystic Fibrosis and Achieving Vocational Success: The Key Role of the Rehabilitation Counselor." Journal of Applied Rehabilitation Counseling 42, no. 4 (December 1, 2011): 12–18. http://dx.doi.org/10.1891/0047-2220.42.4.12.

Full text

Abstract:

In the past, individuals with cystic fibrosis (CF) were not expected to live to be adults, but now the expected lifespan is nearly forty years, with many living decades longer. As more persons with CF live into adulthood, the need for rehabilitation counselors familiar with the demands of this health condition will grow. Rehabilitation counselors are trained to assist those with CF, especially as they transition into adulthood, begin career exploration, attain and maintain employment, and balance life demands with health care needs. This paper provides specific vocational recommendations and examples to assist rehabilitation counselors working with individuals with CF.

APA, Harvard, Vancouver, ISO, and other styles

28

Marks, Margaret P., Sonya L. Heltshe, Arthur Baines, Bonnie W. Ramsey, Lucas R. Hoffman, and Michael S. Stalvey. "Most Short Children with Cystic Fibrosis Do Not Catch Up by Adulthood." Nutrients 13, no. 12 (December 10, 2021): 4414. http://dx.doi.org/10.3390/nu13124414.

Full text

Abstract:

Poor linear growth is common in children with cystic fibrosis (CF) and predicts pulmonary status and mortality. Growth impairment develops in infancy, prior to pulmonary decline and despite aggressive nutritional measures. We hypothesized that growth restriction during early childhood in CF is associated with reduced adult height. We used the Cystic Fibrosis Foundation (CFF) patient registry to identify CF adults between 2011 and 2015 (ages 18–19 y, n = 3655) and had height for age (HFA) records between ages 2 and 4 y. We found that only 26% CF adults were ≥median HFA and 25% were <10th percentile. Between 2 and 4 years, those with height < 10th percentile had increased odds of being <10th percentile in adulthood compared to children ≥ 10th percentile (OR = 7.7). Of HFA measured between the 10th and 25th percentiles at ages 2–4, 58% were <25th percentile as adults. Only 13% between the 10th and 25th percentile HFA at age 2–4 years were >50th percentile as adults. Maximum height between ages 2 and 4 highly correlated with adult height. These results demonstrate that low early childhood CF height correlates with height in adulthood. Since linear growth correlates with lung growth, identifying both risk factors and interventions for growth failure (nutritional support, confounders of clinical care, and potential endocrine involvement) could lead to improved overall health.

APA, Harvard, Vancouver, ISO, and other styles

29

Baran, E., V. D'Ascenzo, F. Butti, N. Granero, B. Hendriksen, D. Bossia, M. C. Calzona, et al. "298 Comparison of adults with cystic fibrosis (diagnosed in childhood vs adulthood)." Journal of Cystic Fibrosis 14 (June 2015): S134. http://dx.doi.org/10.1016/s1569-1993(15)30472-0.

Full text

APA, Harvard, Vancouver, ISO, and other styles

30

Sala, Valentina, Alessandra Murabito, and Alessandra Ghigo. "Inhaled Biologicals for the Treatment of Cystic Fibrosis." Recent Patents on Inflammation & Allergy Drug Discovery 13, no. 1 (August 5, 2019): 19–26. http://dx.doi.org/10.2174/1872213x12666181012101444.

Full text

Abstract:

Background:Cystic Fibrosis (CF), one of the most frequent genetic diseases, is characterized by the production of viscous mucus in several organs. In the lungs, mucus clogs the airways and traps bacteria, leading to recurrent/resistant infections and lung damage. For cystic fibrosis patients, respiratory failure is still lethal in early adulthood since available treatments display incomplete efficacy.Objective:The objective of this review is to extend the current knowledge in the field of available treatments for cystic fibrosis. A special focus has been given to inhaled peptide-based drugs.Methods:The current review is based on recent and/or relevant literature and patents already available in various scientific databases, which include PubMed, PubMed Central, Patentscope and Science Direct. The information obtained through these diverse databases is compiled, critically interpreted and presented in the current study. An in-depth but not systematic approach to the specific research question has been adopted.Results:Recently, peptides have been proposed as possible pharmacologic agents for the treatment of respiratory diseases. Of note, peptides are suitable to be administered by inhalation to maximize efficacy and reduce systemic side effects. Moreover, innovative delivery carriers have been developed for drug administration through inhalation, allowing not only protection against proteolysis, but also a prolonged and controlled release.Conclusion:Here, we summarize newly patented peptides that have been developed in the last few years and advanced technologies for inhaled drug delivery to treat cystic fibrosis.

APA, Harvard, Vancouver, ISO, and other styles

31

LaBonte, Michelle Lynne. "Addressing Uncertainty: The Emergence of the CRMS/CFSPID Diagnostic Category Following Newborn Screening for Cystic Fibrosis." OBM Genetics 05, no. 03 (July 29, 2021): 1. http://dx.doi.org/10.21926/obm.genet.2103139.

Full text

Abstract:

This article uses cystic fibrosis as a case study to examine how physicians and scientists have navigated uncertainty following newborn screening. Despite the many benefits of newborn screening, including earlier diagnosis, therapeutic intervention, and a reduced diagnostic odyssey, this public health approach also comes with challenges. For example, physicians began to document infants with indeterminate diagnoses - those with a positive screen who did not clearly fit into the cystic fibrosis or “normal” categories - by the early twenty first century. As a means of addressing this uncertainty and to facilitate long-term follow up of such infants, the U.S. Cystic Fibrosis Foundation recommended in 2009 that a new diagnostic term, CFTR-related metabolic syndrome (CRMS), be used. However, the CRMS label was not favored in Europe and a different term, cystic fibrosis screen positive, inconclusive diagnosis (CFSPID), was adopted in 2015 instead. Efforts to address the uncertainty associated with indeterminate diagnoses have been complicated, as stakeholders have held differing views about whether the screening algorithms should aim to maximize or minimize CRMS/CFSPID cases. Many who favor the identification of babies with CRMS/CFSPID note that they are at increased risk of developing symptoms and signs consistent with a cystic fibrosis diagnosis. In contrast, those who support algorithms that reduce CRMS/CFSPID cases point to iatrogenic harms associated with the medicalization of children who may remain healthy into adulthood. Furthermore, investigators have grappled with how best to ensure equity in newborn screening among different racialized groups while concomitantly attempting to minimize false positive results. These issues are applicable beyond the context of cystic fibrosis, especially as programs contemplate the incorporation or expanded use of next generation sequencing in algorithms for a wide range of diseases, and this history highlights how efforts to reduce uncertainty in one setting can lead to new and persistent sources of uncertainty in other areas.

APA, Harvard, Vancouver, ISO, and other styles

32

Stahl, Mirjam, Eva Steinke, and Marcus A. Mall. "Quantification of Phenotypic Variability of Lung Disease in Children with Cystic Fibrosis." Genes 12, no. 6 (May 25, 2021): 803. http://dx.doi.org/10.3390/genes12060803.

Full text

Abstract:

Cystic fibrosis (CF) lung disease has the greatest impact on the morbidity and mortality of patients suffering from this autosomal-recessive multiorgan disorder. Although CF is a monogenic disorder, considerable phenotypic variability of lung disease is observed in patients with CF, even in those carrying the same mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene or CFTR mutations with comparable functional consequences. In most patients with CF, lung disease progresses from childhood to adulthood, but is already present in infants soon after birth. In addition to the CFTR genotype, the variability of early CF lung disease can be influenced by several factors, including modifier genes, age at diagnosis (following newborn screening vs. clinical symptoms) and environmental factors. The early onset of CF lung disease requires sensitive, noninvasive measures to detect and monitor changes in lung structure and function. In this context, we review recent progress with using multiple-breath washout (MBW) and lung magnetic resonance imaging (MRI) to detect and quantify CF lung disease from infancy to adulthood. Further, we discuss emerging data on the impact of variability of lung disease severity in the first years of life on long-term outcomes and the potential use of this information to improve personalized medicine for patients with CF.

APA, Harvard, Vancouver, ISO, and other styles

33

Krasovskiy, S. A., E. L. Amelina, U. V. Gorinova, A. V. Chernyak, M. V. Afanasjeva, N. A. Krilova, G. L. Shumkova, O. G. Zonenko, and A. R. Tatarskiy. "The dynamics of some health indicators in adult patients with cystic fibrosis, observed in the research institute of pulmonology in 2003-2018." Journal of Clinical Practice 9, no. 4 (December 15, 2018): 25–32. http://dx.doi.org/10.17816/clinpract9425-32.

Full text

Abstract:

Background: Evaluation of the main clinical and functional indicators and their relationship with the treatment and diagnostic care at different time periods can contribute to the development of further effective medical strategies and globally lead to improved care for cystic fibrosis patients. Aim: An assessment of the dynamics of some clinical and functional indicators in adult CF patients, who have been observed ed Research Institute of Pulmonology during the last 15 years(2003–2018). Methods: The comparative analysis was performed on the data of adult patients with cystic fibrosis, observed at 31.12.2003 and at the end of October of 2018. The group of patients in 2003 consists of 80 and in 2018 — of 667 patients. The comparative analysis between the groups was carried out according to the following indicators: age, survival, lung function, nutritional status, proportion of infection with pathogenic microflora, identification of mutations in the cystic fibrosis gene and diagnosis in adult patients. Results: An increase in patient’s age, survival rate, the proportion of identified mutations in cystic fibrosis gene and infection of Burkholderia cepacia complex in the group of patients in 2018 was revealed. There were no differences in the state of lung function, nutritional status, the ratio of the living and dead patients and proportion of patients diagnosed in adulthood between groups. Conclusion: The progress in the treatment through the introduction of modern methods and therapeutic programs leads to improved survival and an increase in the number of adult patients with cystic fibrosis.

APA, Harvard, Vancouver, ISO, and other styles

34

Marks, Steven C., and Dana G. Kissner. "Management of Sinusitis in Adult Cystic Fibrosis." American Journal of Rhinology 11, no. 1 (January 1997): 11–14. http://dx.doi.org/10.2500/105065897781446810.

Full text

Abstract:

Until recently, cystic fibrosis was frequently fatal during childhood. However, with current medical management, many patients are living into adulthood. This has created a new population of patients with chronic sinusitis and severe medical problems. In this report, experience with 22 patients, eight of whom have undergone sinus surgery, is presented, and recommendations for management are proposed. Presenting symptoms are typical of sinusitis, but in a few patients, severe debilitating headaches predominate. Oral antibiotics are often of little use due to the numerous courses of high dose intravenous antibiotics used for resistant pulmonary infections. Topical nasal steroids and mucolytics have been of some benefit. Fourteen operative procedures were performed on eight patients. These procedures included 12 endoscopic sphenoethmoidectomies, four Caldwell-Luc procedures, two frontal sinus obliterations, and one transseptal sphenoidotomy (many of these were in combination.) Results from this experience indicate 1) Failure of endoscopic surgery to control frontal and maxillary sinus disease; 2) Delayed healing of the ethmoid cavity with persistent crusting; and 3) Significant, albeit short term, symptomatic relief following surgical intervention. Based on this limited series, we conclude that surgery should be delayed until absolutely necessary and that an aggressive approach should be adopted when surgery is performed. In our hands this included initial endoscopic sphenoethmoidectomy with open surgical techniques used for removal of trapped inspissated secretions. We recommend long term intravenous antibiotics postoperatively and frequent cleaning of the ethmoid cavity after surgery, continuing indefinitely, to optimize the benefit of surgery.

APA, Harvard, Vancouver, ISO, and other styles

35

Nasr, Samya Z., and Carol Campbell Welsch. "Disability and quality of life in cystic fibrosis from early age to adulthood." Journal of Adolescent Health 19, no. 6 (December 1996): 381–83. http://dx.doi.org/10.1016/s1054-139x(96)00158-9.

Full text

APA, Harvard, Vancouver, ISO, and other styles

36

Szczesniak, Rhonda D., Dan Li, Weiji Su, Cole Brokamp, John Pestian, Michael Seid, and John P. Clancy. "Phenotypes of Rapid Cystic Fibrosis Lung Disease Progression during Adolescence and Young Adulthood." American Journal of Respiratory and Critical Care Medicine 196, no. 4 (August 15, 2017): 471–78. http://dx.doi.org/10.1164/rccm.201612-2574oc.

Full text

APA, Harvard, Vancouver, ISO, and other styles

37

Office, Daniel, and Inge Heeres. "Transition from paediatric to adult care in cystic fibrosis." Breathe 18, no. 3 (September 2022): 210157. http://dx.doi.org/10.1183/20734735.0157-2021.

Full text

Abstract:

In the decades since cystic fibrosis (CF) was first clinically defined in the 1930s, there have been many advancements in the treatment and management of this disease. Initially it was considered a disease of childhood where the majority of those affected died before reaching adolescence. Now, through advancements in management and treatment, the vast majority of those affected will live into adulthood. Therefore, paediatric and adult CF services must collaborate to ensure that young people and their families experience a positive and supportive transition into adult services. Key aspects of transition will be discussed, including when to begin the transition process, who should coordinate this and how the transition process should be structured. Challenges of the transition process and potential pitfalls when transition does not run smoothly will also be discussed, as well as tools that may be used to support a positive transition for young people and their families.Educational aimsTo familiarise readers with factors that make the transition process positive.To make suggestions regarding the application of the transition process.To highlight factors which may impact on the success of the transition process and the risks associated with disengagement at the point of transition.To discuss tools which can be used by care teams to ensure a smooth transition process.

APA, Harvard, Vancouver, ISO, and other styles

38

FALANY, Josie L., Heather GREER, Timea KOVACS, Eric J. SORSCHER, and Charles N. FALANY. "Elevation of hepatic sulphotransferase activities in mice with resistance to cystic fibrosis." Biochemical Journal 364, no. 1 (May 8, 2002): 115–20. http://dx.doi.org/10.1042/bj3640115.

Full text

Abstract:

The severity of intestinal disease in the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) (-/−) mice has been reported to co-segregate with gene loci which contain the genes for hydroxysteroid sulphotransferase (SULT). Because of the potential involvement of steroid hormones in CF, we investigated levels of steroid SULT activity in the livers of CFTR mice to determine whether the levels of SULT activity correlate with the occurrence or severity of CF. To elucidate the possible role of SULT activity in ameliorating the deleterious effects of CF in CFTR (-/−) mice, we determined the levels of phenol SULT (PST), hydroxysteroid SULT [dehydroepiandrosterone (DHEA)-ST] and oestrogen SULT (EST) activity in control CFTR (+/+), heterozygous CFTR (+/−) and homozygous CFTR (-/−) mice, which survive to adulthood. The level of PST activity was not significantly different between any of the groups of mice, regardless of sex or genotype. Although DHEA-ST activity was significantly higher in female mice than in male mice, there was no difference in DHEA-ST activity that could be correlated with genotype. In contrast with PST and DHEA-ST activities, we found that some male and all female adult CFTR (-/−) mice had elevated, dramatically different levels of EST from both CFTR (+/+) and CFTR (+/−) mice. Results from these SULT activity experiments were confirmed by Northern-blot analysis of mouse-liver RNA. Subsequent studies with preweanling mice revealed no differences in the levels of EST that could be correlated with genotype. Thus this study indicates that EST is elevated significantly in CFTR (-/−) mice which survive to adulthood and provides important biochemical information that EST levels may be protective in CF.

APA, Harvard, Vancouver, ISO, and other styles

39

Cole, Kate H., Patrick R. Sosnay, Lonny B. Yarmus, and Jonathan B. Zuckerman. "The Novel CFTR Mutation A457P in a Male with a Delayed Diagnosis of Cystic Fibrosis." Case Reports in Medicine 2011 (2011): 1–4. http://dx.doi.org/10.1155/2011/903910.

Full text

Abstract:

Cystic fibrosis (CF) is an autosomal recessive disease that may be caused by more than 1000 different mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. We describe the case of a CF patient who was initially diagnosed at 16 years of age after presenting with mild respiratory compromise and pancreatic sufficiency. When genetic testing was first performed using a CF mutation panel, only a single F508del CFTR allele was identified. We subsequently performed testing, which revealed a previously unreported mutation: A457P (p.Ala457Pro, c.1369G>C). The patient's clinical course through adulthood is described, and genotype-phenotype correlation is discussed. The A457P mutation appears to confer a relatively mild phenotype, as is usually observed with CFTR class IV–VI defects. With the advent of more comprehensive and widely available genetic testing techniques, identification of CF genotypes in patients with milder disease variants may help stratify patients for targeted therapy and prevent late complications of the disease.

APA, Harvard, Vancouver, ISO, and other styles

40

Morozova, N. L., M. M. Tkachenko, and A. A. Romanenko. "RADIOLOGIC DIAGNOSTIC'S CAPABILITIES OF LUNG INJURY IN ADULT PATIENTS WITH CYSTIC FIBROSIS." Medical Science of Ukraine (MSU) 15, no. 1-2 (December 6, 2019): 51–58. http://dx.doi.org/10.32345/2664-4738.1-2.2019.07.

Full text

Abstract:

Relevance. The viability of patients is determined by the degree of lung injury despite the fact that cystic fibrosis (CF) is a multiple organ disease. Early diagnosis and adequate treatment complex of cystic fibrosis prolong the lives of patients and improves its quality. Therefore, timely and detailed diagnosis of cystic fibrosis lung is particularly important problem. The central role for its solution belongs to radiological studies. Objective. To refine the radiology’s capabilities in detecting lung changes in case of cystic fibrosis in adult patients and to determine the role of the computed tomography (CT) in the diagnostic process. Materials and methods. The results of radiography and CT scans of 15 patients, for whom the diagnosis of CF was established in adulthood, were analyzed. Results. The most typical radiographic and CT signs of lung damage were determined in adult CF patients. It has been shown that the radiographic changes in the lungs are non-specific for CF patients and are determined by the secondary inflammatory process. The diagnostic capabilities of CT scans in detecting subtle structural changes in the lung tissue and bronchial tree are underlined, which allows to diagnose light and atypical forms of CF in adults, determinate the stage and activity of the pathological process and the effectiveness of the treatment. Findings. The radiation studies were able to define the CF’s specific symptoms of lung lesions. Radiography of the thoracic cavity’s organs is a necessary step in the preliminary diagnosis for adult patients with CF or in the medical emergency's cases. The CT has the greatest diagnostic value in detecting subtle structural and functional specific changes of CF. The regular monitoring using CT scans is necessary for improving the control of the respiratory organs’ state and determine the further tactics for the particular patient.

APA, Harvard, Vancouver, ISO, and other styles

41

De Lisle, Robert C., Matthew Petitt, Kathryn S. Isom, and Donna Ziemer. "Developmental expression of a mucinlike glycoprotein (MUCLIN) in pancreas and small intestine of CF mice." American Journal of Physiology-Gastrointestinal and Liver Physiology 275, no. 2 (August 1, 1998): G219—G227. http://dx.doi.org/10.1152/ajpgi.1998.275.2.g219.

Full text

Abstract:

The mucinlike glycoprotein MUCLIN, one of two protein products of the CRP-ductin gene, was used to study changes in the expression of sulfated glycoconjugates during the pathogenesis of cystic fibrosis, using the cystic fibrosis transmembrane conductance regulator (CFTR) knockout mouse (CF mouse). We assessed the appearance of dilated lumina containing protein or mucus plugs in pancreatic acini and crypts of the small intestine and quantified MUCLIN protein and CRP-ductin mRNA during postnatal development. In CF mice, the pancreatic acinar lumen was dilated by postnatal day 16( P16), but MUCLIN protein was first significantly increased by P23 and remained elevated through adulthood compared with normal mice. Similarly, intestinal crypts had CF-like mucus plugs by P16, but MUCLIN protein was first elevated by P23 and remained elevated through adulthood compared with normal mice. In both organs, MUCLIN labeling of the luminal surface was increased concomitantly with dilation and protein or mucus plugging but before upregulation of expression. The morphological changes were then followed by upregulation of MUCLIN protein and CRP-ductin mRNA expression. This is the first direct study of CF pathogenesis and the resultant increase in glycoconjugate gene expression. The data are consistent with CF pathogenesis progressing from an initial alteration in protein secretory dynamics (increased luminal MUCLIN and protein/mucus plugs) to an upregulation of glycoprotein/mucin gene expression, which is expected to exacerbate obstruction of the luminal spaces.

APA, Harvard, Vancouver, ISO, and other styles

42

GÖKÇE, Ali, Burcu DİKEÇ GÖKÇE, and Ahmet UYANIKOĞLU. "Tekrarlayan Pankreatit ile Erişkin Dönemde Tanı Alan Kistik Fibrozis Olgusu Cystic Fibrosis Case Diagnosed in Adulthood with Recurrent Pancreatitis." AYDIN TIP FAKÜLTESİ KLİNİKLERİ DERGİSİ 5, no. 1 (2018): 49–54. http://dx.doi.org/10.17932/iau.tfk.2018.008/tfk_v05i1006.

Full text

APA, Harvard, Vancouver, ISO, and other styles

43

Aris, Robert M. "Increased Rate of Fractures and Severe Kyphosis: Sequelae of Living into Adulthood with Cystic Fibrosis." Annals of Internal Medicine 128, no. 3 (February 1, 1998): 186. http://dx.doi.org/10.7326/0003-4819-128-3-199802010-00004.

Full text

APA, Harvard, Vancouver, ISO, and other styles

44

Mohan, Kamlesh, Vinay Lakshman, Joanne L. Fothergill, Martin J. Ledson, Craig Winstanley, and Martin J. Walshaw. "Empyema due to a highly transmissible Pseudomonas aeruginosa strain in an adult cystic fibrosis patient." Journal of Medical Microbiology 59, no. 5 (May 1, 2010): 614–16. http://dx.doi.org/10.1099/jmm.0.014696-0.

Full text

Abstract:

Chronic pulmonary infection with Pseudomonas aeruginosa occurs in up to 85 % of individuals with cystic fibrosis (CF) by the time they reach adulthood, and is the major cause of morbidity and mortality: nearly all patients die from progressive respiratory failure due to repeated pulmonary exacerbations. However, despite the predilection of this organism for the lungs of CF people, infection of the pleura is much less common and is not well described in the CF population. We describe what is believed to be the first case of pleural empyema due to a particularly pathogenic transmissible strain of P. aeruginosa (the Liverpool epidemic strain) in an adult CF patient.

APA, Harvard, Vancouver, ISO, and other styles

45

Elborn, J. Stuart, Patrick A. Flume, Donald R. Van Devanter, and Claudio Procaccianti. "Management of chronic Pseudomonas aeruginosa infection with inhaled levofloxacin in people with cystic fibrosis." Future Microbiology 16, no. 14 (September 2021): 1087–104. http://dx.doi.org/10.2217/fmb-2021-0150.

Full text

Abstract:

People with cystic fibrosis (CF) are highly susceptible to bacterial infections of the airways. By adulthood, chronic Pseudomonas aeruginosa ( Pa) is the most prevalent infective organism and is difficult to eradicate owing to its adaptation to the CF lung microenvironment. Long-term suppressive treatment with inhaled antimicrobials is the standard care for reducing exacerbation frequency, improving quality of life and increasing measures of lung function. Levofloxacin (a fluoroquinolone antimicrobial) has been approved as an inhaled solution in Europe and Canada, for the treatment of adults with CF with chronic P. aeruginosa pulmonary infections. Here, we review the clinical principles relating to the use of inhaled antimicrobials and inhaled levofloxacin for the management of P. aeruginosa infections in patients with CF.

APA, Harvard, Vancouver, ISO, and other styles

46

Zorcec, Tatjana, Nada Pop-Jordanova, Stojka Fustik, Tatjana Jakovska, and Lidija Spirevska. "Coping with Cystic Fibrosis in the Republic of Macedonia–Parent Perspective." PRILOZI 40, no. 3 (December 1, 2019): 69–75. http://dx.doi.org/10.2478/prilozi-2020-0006.

Full text

Abstract:

Abstract Introduction: Cystic fibrosis (CF) is a progressive, life-threatening, genetic disease which mainly damages the lungs and the digestive system. It’s a complex medical condition, with several individual forms and variation in the symptoms severity. Few factors such as age of establishing the diagnosis, the number and the type of infections and their management, best treatment options, comorbid conditions etc. can influence the patient’s overall health, disease progression and quality of life. Many CF patients will reach adulthood, so coping with the chronic disease is very important for the overall health and everyday living. Aim of the study: To screen the quality of life in CF patients in the Republic of Macedonia, from the parent perspective. Subjects and methods: In the study we have included 55 parents of CF patients. We have created a questionnaire, specially designed for this survey, with questions related to their everyday coping with CF and quality of life. Results: The majority of the parents refer to the overall typical social and emotional life of their children, addressing some difficulties concerning the financial aspect of the disease and still significantly having fear from the stigma in the society. Conclusion: CF patients and their families in the Republic of Macedonia must overcome many obstacles on daily basis. Despite that, they can still have full and meaningful lives.

APA, Harvard, Vancouver, ISO, and other styles

47

Chandler, Joshua D., Camilla Margaroli, Hamed Horati, Matthew B. Kilgore, Mieke Veltman, H. Ken Liu, Alexander J. Taurone, et al. "Myeloperoxidase oxidation of methionine associates with early cystic fibrosis lung disease." European Respiratory Journal 52, no. 4 (September 6, 2018): 1801118. http://dx.doi.org/10.1183/13993003.01118-2018.

Full text

Abstract:

Cystic fibrosis (CF) lung disease progressively worsens from infancy to adulthood. Disease-driven changes in early CF airway fluid metabolites may identify therapeutic targets to curb progression.CF patients aged 12–38 months (n=24; three out of 24 later denoted as CF screen positive, inconclusive diagnosis) received chest computed tomography scans, scored by the Perth–Rotterdam Annotated Grid Morphometric Analysis for CF (PRAGMA-CF) method to quantify total lung disease (PRAGMA-%Dis) and components such as bronchiectasis (PRAGMA-%Bx). Small molecules in bronchoalveolar lavage fluid (BALF) were measured with high-resolution accurate-mass metabolomics. Myeloperoxidase (MPO) was quantified by ELISA and activity assays.Increased PRAGMA-%Dis was driven by bronchiectasis and correlated with airway neutrophils. PRAGMA-%Dis correlated with 104 metabolomic features (p<0.05, q<0.25). The most significant annotated feature was methionine sulfoxide (MetO), a product of methionine oxidation by MPO-derived oxidants. We confirmed the identity of MetO in BALF and used reference calibration to confirm correlation with PRAGMA-%Dis (Spearman's ρ=0.582, p=0.0029), extending to bronchiectasis (PRAGMA-%Bx; ρ=0.698, p=1.5×10−4), airway neutrophils (ρ=0.569, p=0.0046) and BALF MPO (ρ=0.803, p=3.9×10−6).BALF MetO associates with structural lung damage, airway neutrophils and MPO in early CF. Further studies are needed to establish whether methionine oxidation directly contributes to early CF lung disease and explore potential therapeutic targets indicated by these findings.

APA, Harvard, Vancouver, ISO, and other styles

48

Di Cicco, Maria E., Francesca Bizzoco, Elena Morelli, Veronica Seccia, Vincenzo Ragazzo, Diego G. Peroni, and Pasquale Comberiati. "Nasal Polyps in Children: The Early Origins of a Challenging Adulthood Condition." Children 8, no. 11 (November 2, 2021): 997. http://dx.doi.org/10.3390/children8110997.

Full text

Abstract:

Nasal polyps (NPs) are benign inflammatory masses causing chronic nasal obstruction, usually associated with underlying chronic rhinosinusitis (CRS), which are rarely reported in childhood. The interest in NPs has recently increased due to new therapeutic options, namely biological agents, such as dupilumab, and an update of the European position paper on this topic was released in 2020, providing a detailed classification for these lesions and also discussing diagnostic and therapeutic approaches also in children. In childhood, NPs usually represent red flags for systemic diseases, such as cystic fibrosis and immunodeficiencies. This review outlines the recent data on NPs in childhood, focusing on predisposing factors for CRS as well as on the potential endotypes in this particular age group, for which further studies are required in order to better clarify their pathogenesis and to identify molecular biomarkers that could help achieve more personalized treatments.

APA, Harvard, Vancouver, ISO, and other styles

49

Miller, R., K. Askew, J. Bamford, N. Hudson, J. Moratelli, A. Anderson, S. Doe, and SJ Bourke. "P182 Current characteristics, coping strategies and outcomes of young people with cystic fibrosis transitioning to adulthood." Thorax 71, Suppl 3 (November 15, 2016): A182.3—A183. http://dx.doi.org/10.1136/thoraxjnl-2016-209333.325.

Full text

APA, Harvard, Vancouver, ISO, and other styles

50

Cobanoglu, N., S. Pekcan, N. Kiper, U. Ozcelik, D. Dogru, E. Yalcin, A. Gocmen, and M. Kose. "346 Clinical manifestations of cystic fibrosis among patients diagnosed in childhood and patients diagnosed in adulthood." Journal of Cystic Fibrosis 6 (June 2007): S84. http://dx.doi.org/10.1016/s1569-1993(07)60317-8.

Full text

APA, Harvard, Vancouver, ISO, and other styles

Journal articles on the topic 'Cystic Fibrosis in adulthood'

Create a spot-on reference in APA, MLA, Chicago, Harvard, and other styles