The clinical manifestations of cystic fibrosis are typically categorized as pulmonary and non-pulmonary.

Pulmonary manifestations

The pulmonary manifestations of cystic fibrosis are encountered in the majority of the cases. They are often the presenting complaints of the patient.

• Recurrent pulmonary infections are very characteristic of cystic fibrosis. The most common pathogen responsible for pulmonary infection in cystic fibrosis is Staphylococcus aureus. Other pathogens commonly encountered in this disease include Pseudomonas aeruginosa, Hemophilus influenza and Burkholderia cepacia.
• The patient also suffers from persistent (or recurrent) productive cough. In addition, dyspnea and wheezing are also present.
• With time, the damage resulting from these recurrent infections and coughing accumulates and leads to progressive bronchiectasis. There is also scarring of the airways which may result in airflow obstruction.

Other manifestations

The non-pulmonary manifestations of cystic fibrosis cover several organ systems and are summarized below.

• General physical manifestations: A number of findings will be seen in the advanced cases of cystic fibrosis upon general physical examination. Clubbing of the fingernails is often present. The anteroposterior diameter of the chest is also increased and upon percussion, the chest is found to be hyper-resonant.
• Upper respiratory tract manifestations: The patients of cystic fibrosis often suffer from sinusitis. The upper respiratory tract may produce purulent secretions in large amount. Nasal polyps also occur frequently in cases of cystic fibrosis.
• Gastrointestinal tract manifestations: In newborns, the distal gastrointestinal tract can become filled with thick, sticky intestinal secretions, causing meconium ileus and obstruction of the small intestine. The meconium also contains a higher amount of carbohydrates and proteins [5]. Although much less pronounced in adults, advanced cases can present with similar findings.
• Pancreatic insufficiency: Both the exocrine and endocrine functions of the pancreas can be affected. Dysfunction of the exocrine part can cause some degree of indigestion. This mostly comes to notice as steatorrhea due to the high undigested lipid content in the stool. Pancreatic insufficiency also leads to decreased levels of insulin. As a result, insulin dependent diabetes mellitus can occur.
• Reproductive system manifestations: Almost all the male patients suffering from cystic fibrosis are infertile. This is due to the inability of the vas deferens to develop on both sides.

• Infertility

  Case 1 patient is a 39-year-old infertile man presenting with congenital unilateral absence of the vas deferens and recurrent episodes of epigastric pain. [ncbi.nlm.nih.gov]

  Other signs and symptoms include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in males, among others. Different people may have different degrees of symptoms. [dbpedia.org]

  Almost all men and most women with cystic fibrosis are infertile. The lives of people with cystic fibrosis are usually shortened by the disorder, but they can lead happy and productive lives well into middle age. [healthdirect.gov.au]

• Malnutrition

  Diet-induced protein calorie malnutrition in C57BL/6J mice resulted in impaired pulmonary clearance of P. aeruginosa. [ncbi.nlm.nih.gov]

  These results indicate that malnutrition impairs pulmonary clearance of P. aeruginosa. Table 1. [doi.org]

  This leads to problems with digestion (e.g. diarrhea, fatty stools) and to malnutrition. Children do not grow properly and do not gain sufficient weight. In rare cases, newborns may be afflicted by intestinal blockage (meconium ileus). [aoporphan.com]

  Most people with cystic fibrosis have: chest problems such as cough, wheeze and recurrent chest infections digestive problems and bulky, fatty stools very salty sweat They may also have lung damage, malnutrition, poor growth and diabetes. [healthdirect.gov.au]

• Anemia

  Additional resources: American Sickle Cell Anemia Association Genetics Home Reference: Sickle Cell Anemia MEDLINEplus: Sickle Cell Anemia UK : NHS Sickle Cell & Thalassaemia Screening Programme Haemophilia Haemophilia is a hereditary bleeding disorder [who.int]

  Female sex and chronic anemia were independent predictors of readmission. The most common causes of readmission were pulmonary exacerbation (31%), lung transplant complications (5.2%), and septicemia (3.4%). [ncbi.nlm.nih.gov]

  Cystic fibrosis can cause other health problems, such as: Weak bones Low number of red blood cells (anemia) Bleeding problems Heart problems Liver problems Problems getting pregnant or fathering children (infertility) In the United States, doctors do [merckmanuals.com]

• Weight Loss

  An oral glucose tolerance test is recommended annually for patients > or = 10 years of age and for any patients presenting unexplained weight loss or symptoms of diabetes. Patients hospitalized with severe diseases should also be screened. [ncbi.nlm.nih.gov]

  Contact you doctor if you experience new or worsening symptoms, such as more mucus than usual or a change in the mucus color, lack of energy, weight loss, or severe constipation. [mayoclinic.org]

  Increased gas, bloating, or a belly that appears swollen (distended) Nausea and loss of appetite Stools that are pale or clay-colored, foul smelling, have mucus, or that float Weight loss Symptoms related to the lungs and sinuses may include: Coughing [nlm.nih.gov]

• Recurrent Respiratory Infections

  digestive and reproductive tracts: although lungs are normal at birth, recurrent respiratory infection is a major presenting feature 85% of CF patients have pancreatic insufficiency 10% of CF newborns suffer meconium ileus patients are also prone to [gpnotebook.co.uk]

  Presentation As normal digestive function is possible with The most common presentation is with respiratory problems - usually recurrent lower respiratory tract infection (LRTI) with chronic sputum production. [patient.info]

  It is characterized by the triad of chronic bronchopulmonary disease (with recurrent respiratory infections), pancreatic insufficiency (which leads to malabsorption and growth retardation) and elevated sweat electrolytes. [malacards.org]

• Cough

  Numerical pain rating scores from his ribs were lower post-operatively and he was able to tolerate chest physical therapy and vigorous coughing. [ncbi.nlm.nih.gov]

  This makes it easier to cough the mucus out of the lungs. [nlm.nih.gov]

• Hemoptysis

  Abstract Massive hemoptysis occurs in a minority of patients with cystic fibrosis, with an annual incidence of 1%. Although rare, massive hemoptysis can be a severe and potentially fatal complication of this disease. [ncbi.nlm.nih.gov]

  Hemoptysis and pneumothorax are complications commonly reported in patients with cystic fibrosis. Objectives : This document presents the CF Foundation's Pulmonary Therapies Committee recommendations for the treatment of hemoptysis and pneumothorax. [doi.org]

• Chronic Cough

  Respiratory organs The viscous mucus causes chronic coughing and regular infections of the respiratory organs with germs such as staphylococcus aureus and pseudomonas aeruginosa. [aoporphan.com]

  Chronic coughing weakens the bladder muscles. Almost 65% of women with CF have stress incontinence. This means that you leak urine when you cough, sneeze, laugh, or lift something. Though more common in women, men can have it, too. Kidneys. [webmd.com]

  Other symptoms include a chronic cough, frequent wheezing, pneumonia, or a chronic cough with thick mucus. Infants and babies with Cystic Fibrosis may also have vomiting, dehydration, salty-tasting skin, and intestinal blockages. [disabled-world.com]

  These infections cause chronic coughing, wheezing, and inflammation. Over time, mucus buildup and infections result in permanent lung damage, including the formation of scar tissue (fibrosis) and cysts in the lungs. [ghr.nlm.nih.gov]

  Chronic cough, recurrent pneumonia, and the progressive loss of lung function are the major manifestations of lung disease, which is the most common cause of death of persons with cystic fibrosis. [britannica.com]

• Sputum Production

  production, and recurrent respiratory infections. [ncbi.nlm.nih.gov]

  Periods in which the lung infections worsen are called exacerbations, which are associated with more coughing and sputum production, and may require treatment with the use of intravenous antibiotics. [healthychildren.org]

  Acute or subacute clinical deterioration (cough, wheeze, exercise intolerance, exercise-induced asthma, change in pulmonary function, or increased sputum production) not attributable to another etiology. 2. [doi.org]

  Presentation As normal digestive function is possible with The most common presentation is with respiratory problems - usually recurrent lower respiratory tract infection (LRTI) with chronic sputum production. [patient.info]

• Dyspnea

  The patient was admitted to our department with fever, cough, and dyspnea at rest unresponsive to antibiotics CONCLUSIONS: Late diagnosis of cystic fibrosis in uncommon. [ncbi.nlm.nih.gov]

  Shortness of breath Other names Dyspnea, dyspnoea, breathlessness, difficulty of breathing, respiratory distress Pronunciation Dyspnea: /dɪspˈniːə/ Specialty Pulmonology Shortness of breath (SOB), also known as dyspnea, is a feeling of not being able [en.wikipedia.org]

  In addition, dyspnea and wheezing are also present. With time, the damage resulting from these recurrent infections and coughing accumulates and leads to progressive bronchiectasis. [symptoma.com]

  If acute worsening of dyspnea or pleuritic chest pain develops – consider spontaneous pneumothorax-stat chest X-ray Minor hemoptysis is not uncommon in CF patients. If develops, discontinue chest physiotherapy until resolves. [cancertherapyadvisor.com]

  Strong recommendation, very low-quality evidence For patients with cystic fibrosis who have both mild chronic hypoxemia and dyspnea on exertion, we suggest that home oxygen therapy be prescribed. [doi.org]

• Constipation

  However, gastrointestinal problems such as constipation and distal intestinal obstruction syndrome (DIOS) are also important and well-recognised complications in CF. [ncbi.nlm.nih.gov]

  A person with cystic fibrosis may have constipation or blocked intestines. Medicines can help with constipation. The medicine may be one that your child drinks or one that you can place in your child's anus (where poop comes out) as an enema. [merckmanuals.com]

  The most common form of cystic fibrosis is associated with respiratory symptoms, digestive problems (steatorrhea and/or constipation) and staturoponderal growth anomalies. Mortality and morbidity depend on the extent of bronchopulmonary involvement. [orpha.net]

• Diarrhea

  Both adaptations would avoid severe diarrhea attacks following lactose ingestion. [nature.com]

  This leads to problems with digestion (e.g. diarrhea, fatty stools) and to malnutrition. Children do not grow properly and do not gain sufficient weight. In rare cases, newborns may be afflicted by intestinal blockage (meconium ileus). [aoporphan.com]

  […] the very high frequency of cystic fibrosis (CF) mutations in most populations of European descent, it has been proposed that CF heterozygotes have a survival advantage when infected with Vibrio cholerae or Escherichia coli, the toxins of which induce diarrhea [ncbi.nlm.nih.gov]

  Proposed diseases that the CF carrier state may protect against include diarrhea from cholera or lactose intolerance, typhoid fever, high blood pressure and tuberculosis. [nationaljewish.org]

• Abdominal Pain

  They share similar symptoms e.g. bloating, abdominal pain, but are distinct conditions. [ncbi.nlm.nih.gov]

  Forest plot of comparison: 2 ECM versus ECT, outcome: 2.3 Abdominal pain [% days]. [doi.org]

  Consequences are fatty stools, abdominal pain and, in the chronic phase, rectal prolapse. Gall bladder/liver: cholezystolithiasis, cholestasis, fatty liver Fertility: Male patients are infertile due to obliteration of the Vas deferens. [flexikon.doccheck.com]

  Complete blood count (CBC) with differential Basic Metabolic Profile (BMP) Lipase (if abdominal pain) Hepatic function panel (if abdominal pain) Urinalysis and culture, if indicated Arterial blood gas (ABG) if evidence of hypoxemia (room air sat <88%) [cancertherapyadvisor.com]

• Failure to Thrive

  Children with CF have the following symptoms: Failure to thrive (inability to gain weight despite having good appetite and taking in enough calories) Loose stools or oily stools Trouble breathing Recurrent wheezing Frequent lung infections (recurrent [my.clevelandclinic.org]

  We present the case of an exclusively breastfed, 2-month-old boy with known cystic fibrosis admitted with failure to thrive and erosive dermatitis. [ncbi.nlm.nih.gov]

  GI tract presentation in early infancy may be in the form of steatorrhea, failure to thrive, or both. [emedicine.medscape.com]

• Vomiting

  These small, hard mineral deposits can cause nausea, vomiting, and pain. If ignored, they can lead to a kidney infection. Reproductive organs. Excess mucus affects fertility in men and women. [webmd.com]

  Cough, haemoptysis, bronchospasm, pharyngolaryngeal pain and post-tussive vomiting were the most commonly reported side effects in both treatment groups. [ncbi.nlm.nih.gov]

  Cough is the most troublesome complaint, often accompanied by sputum, gagging, vomiting, and disturbed sleep. [merckmanuals.com]

• Chest Pain

  Acute chest pain is common in patients with cystic fibrosis (CF). Here we report the case of an adolescent who suffered acute chest pain in September after an history of acute abdominal pain and fever. [ncbi.nlm.nih.gov]

  Seek immediate medical care if you're coughing up blood, have chest pain or difficulty breathing, or have severe stomach pain and distention. [mayoclinic.org]

  At Children’s, we treat kids and teens who have a range of respiratory problems, including: Asthma Bronchiolitis Bronchopulmonary dysplasia Chest pain Chronic cough Chronic respiratory failure and ventilator dependence Congenital abnormalities of the [choa.org]

  Patients may experience the following when using Pulmozyme: change in or loss of their voice, discomfort in the throat, rash, chest pain, red watery eyes, runny nose, lowering of lung function, fever, indigestion, and shortness of breath. [pulmozyme.com]

• Cyanosis

  用 語 対 訳 cutaneous 皮膚の【形】 cutdown カットダウン、動(静)脈切開法 cutis laxa 弛緩性皮膚 cut-off method カットオフ法 CVP (central venous pressure) 中心静脈圧 CVR (coronary vascular resistance) 冠[状]血管抵抗 CVVH (continuous veno-venous hemofiltration) 持続的静脈静脈血液濾過[法] cyanosis チアノーゼ、紫色症 cyanotic [j-circ.or.jp]

  Intercostal retractions, use of accessory muscles of respiration, a barrel-chest deformity, digital clubbing, cyanosis, and a declining tolerance for exercise occur with disease progression. [merckmanuals.com]

  […] polyps Findings related to the pulmonary system may include the following: Tachypnea Respiratory distress with retractions Wheeze or crackles Cough (dry or productive of mucoid or purulent sputum) Increased anteroposterior diameter of chest Clubbing Cyanosis [emedicine.medscape.com]

  क्यान्सरPathological Processes and Terms CellKoshikaकोसिकाPathological Processes and Terms ChronicPuranoपुरानोPathological Processes and Terms CigaretteChurotचुरोटPathological Processes and Terms CongenitalJanmajaatजन्मजातPathological Processes and Terms Cyanosis [virtualnepali.com]

• Osteoporosis

  Effect of oral alendronate on bone mineral density and the incidence of fractures in postmenopausal osteoporosis. The Alendronate Phase III Osteoporosis Treatment Study Group. [ncbi.nlm.nih.gov]

  Osteoporosis may occur during the early years of adulthood. [aoporphan.com]

  They are used to treat osteoporosis caused by menopause or the use of corticosteroid drugs. [doi.org]

• Amenorrhea

  Amenorrhea may occur in females with severe nutritional or pulmonary involvement. [emedicine.medscape.com]

  In severe cases, malnutrition disrupts ovulation and causes amenorrhea. [20] गंभीर मामलों में, कुपोषण बाधित ovulation और कारणों amenorrhea. 20 [] Cause कारण Cystic fibrosis has an autosomal recessive pattern of inheritance. [helpebookhindi.wikifoundry.com]

• Headache

  Chronic sinusitis may cause deformities of the external nasal skeleton, a loss of the sense of smell, and headaches. [ncbi.nlm.nih.gov]

  Presenting symptoms are typical of sinusitis, but in a few patients, severe debilitating headaches predominate. [doi.org]

Diagnosis of cystic fibrosis can easily be made on clinical grounds but a number of examinations are still very useful for confirming the diagnosis. Investigations can also be performed routinely in patients with an affected first degree relative. The following investigations are of diagnostic importance in cystic fibrosis.

Sweat sodium or chloride concentration

The amount of salt in the sweat is increased in the patients suffering from cystic fibrosis. Values of sweat sodium or chloride greater than 60 mmol/L are diagnostic of cystic fibrosis.

DNA analysis

The patient’s DNA can be analyzed in order to locate defects in the gene corresponding to this disease.
Pulmonary function tests: Pulmonary function tests may indicate an obstructive and/or restrictive disease pattern. There may be hypoxemia and in advanced stages, respiratory acidosis.

Imaging

Features of cystic fibrosis can be identified on imaging. Common findings in cystic fibrosis include hyperinflation of the lungs, peribronchial cuffing, mucus plugging, bronchiectasis and peripheral opacities.

• Atelectasis

  Postoperative complications, management, and outcomes of bronchiectasis Complications after surgical resection for lung abscess are infrequent and include atelectasis, bronchopleural fistula, and wound infection. [web.archive.org]

  (in English) The acapella® vibratory PEP system is a single patient use device that provides Positive Expiratory Pressure (PEP) Therapy for patients who have Cystic Fibrosis, COPD, asthma, and lung diseases with secretory problems, and patients with atelectasis [msa.sm.ee]

  Subsequent changes include areas of infiltrate, atelectasis, and hilar adenopathy. With advanced disease, segmental or lobar atelectasis, cyst formation, bronchiectasis, and pulmonary artery and right ventricular hypertrophy occur. [merckmanuals.com]

• Pulmonary Infiltrate

  Allergic bronchopulmonary aspergillosis (ABPA) is an allergic disease characterized clinically by wheezing, pulmonary infiltrates, bronchiectasis, and fibrosis that affects patients with asthma and cystic fibrosis (CF). [ncbi.nlm.nih.gov]

  However, when A. fumigatus does cause clinical symptoms they are most often along the spectrum of ABPA, which is characterized clinically by wheezing, pulmonary infiltrates, bronchiectasis, and pulmonary fibrosis. [doi.org]

• Bilateral Atelectasis

  Axial CT image of a 16-year-old adolescent with group A streptococcal mediastinitis, demonstrating rim-enhancing fluid collections in the mediastinum, bilateral pleural fluid collections, pericardial fluid collections, and bilateral atelectasis. [web.archive.org]

• Left Pleural Effusion

  CT scan of the thorax of a sixteen-year-old with Lemierre's syndrome and anterior mediastinitis revealing pneumomediastinum posterior to and to the left of the trachea, free fluid surrounding the trachea, and left pleural effusion. [web.archive.org]

• Hypercapnia

  Patients with FEV1 > 30% predicted had a higher risk of death only when their Pa(CO2) was > or = 50 mm Hg (HR, 7.0; 95% CI, 1.5-32), while the increased risk of death with FEV1 < or = 30% was not further influenced by the presence of hypercapnia. [ncbi.nlm.nih.gov]

  Patients with FEV 1 > 30% predicted had a higher risk of death only when their Pa CO 2 was ⩾ 50 mm Hg (HR, 7.0; 95% CI, 1.5–32), while the increased risk of death with FEV 1 ⩽ 30% was not further influenced by the presence of hypercapnia. [dx.doi.org]

• Hyponatremia

  Laboratory analysis on admission showed hyponatremia (115-133 mmol/L, mean 122.4 mmol/L), high plasma renin activity (229-500 pg/ml, mean 324 pg/ml) and metabolic alkalosis (pH 7.5-7.6, mean 7.56) in all the patients, and in four of them high blood level [ncbi.nlm.nih.gov]

  […] prolapse, esophageal varices Sex organs ♂ infertility due to blocked vas deferens; ↓ fertility in ♀ Endocrine system Abnormal glucose tolerance, type 2 DM Musculoskeletal Bone pain due to hypertrophic osteoarthropathy Signs hypoproteinemia, salty taste, hyponatremia [medical-dictionary.thefreedictionary.com]

• Pseudomonas

  Respiratory tract infection with Pseudomonas aeruginosa occurs in most people with cystic fibrosis. [ncbi.nlm.nih.gov]

  Pseudomonas putida was grown from the left and right hands of the same member of staff. All other staff hand samples were negative for growth of Pseudomonas species. [doi.org]

• Staphylococcus Aureus in the Sputum

  Staphylococcus aureus Two studies reported on this outcome ( Alton 2015 ; Moss 2004 ). Alton 2015 reported no clinically relevant changes in sputum microbiology of Staphylococcus aureus; no additional data were provided for analysis ( Alton 2015 ). [doi.org]

Treatment of cystic fibrosis focuses on the removal of respiratory tract infections, reduction of secretions and reversal of bronchoconstriction coupled with interventions to combat pancreatic and nutritional insufficiency.

• In cases of respiratory tract infections, the sputum should be cultured and appropriate antibiotics are then administered. Short term antibiotics are used against the infections caused by Staphylococcus aureus, Hemophilus influenza, Burkholderia cepacia and Stenotrophomonas maltophilia. Long term antibiotics are required against Pseudomonas aeruginosa [6].
• Inhaled bronchodilators are effective in reducing respiratory symptoms in most patients.
• Mucolytics are given to reduce the viscosity of the secretions.
  Pancreatic enzyme supplements are given in order to make up for pancreatic insufficiency.
• Nutritional supplements and multivitamins are required.
• A new oral drug named ivacaftor has recently become available for 5% of the cystic fibrosis patients with G551D mutation. This drug reverses the dysfunction of the chloride membrane channel [7]. This treatment is therefore referred to as CFTR corrector therapy.
• Lung transplantation is the most definite treatment in severe respiratory disease [8].
• Adopting an exercise regimen can improve the quality of life of the patient [9].

In the past few decades, the prognosis of patients with cystic fibrosis has significantly improved. The life expectancy of these patients averages 40 years [10].

Modern treatment strategies enable more than 80% of the cystic fibrosis patients to reach adulthood. Deaths in these patients usually result from complications related to the lungs.

Cystic fibrosis is primarily a genetic disease and is inherited in an autosomal recessive pattern. The underlying genetic defects involve only a single gene on chromosome 7.

Currently, a total of 1965 different mutations have been identified in the aforementioned gene and up to 230 genes have been proven to cause clinical manifestations of cystic fibrosis [3].

The most common mutation in cystic fibrosis is ΔF508. It is characterized by a deletion of three nucleotides that code for the amino acid phenylalanine at position 508. It is present in over 65% of the clinical cases of cystic fibrosis worldwide [4].

Other common mutations of clinical significance include G542X, G551D, N1303K and W1282X.

Cystic fibrosis seems to be a rare disease on paper but practically, it is the most common fatal disease in the white population [1]. As expected from this statement, the disease is much more common in the western world, particularly in Europe; and much less common in the Asian and African countries.

In the United States, the prevalence of the disease varies among various races. The highest prevalence (one case per 3200 population) is present in the population of European descent. On the other hand, the lowest prevalence (one case per 31,000 population) is present in the Asian Americans. Hispanics and African Americans (at one case per 9200 and 15000 population, respectively) lie between these two extremes.

Ireland has the highest worldwide incidence of this disease with 1 new case per 1353 individuals per year [2].

The defective gene in this disease encodes a membrane chloride channel and regulatory protein known as Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). This membrane chloride channel is normally found in the apical epithelia of several organs of the body including the lungs, pancreas, stomach, intestines, endocrinal and reproductive organs.

Cystic Fibrosis Transmembrane Conductance Regulator encoded from the mutated gene has several functional defects. There is an abnormal transport of chloride ions and water molecules across the apical surface of the epithelia in the respective organs. The transport of sodium, bicarbonate and other ions may also be affected in some mutations.

As a result, the mucus produced at these epithelial surfaces is unusually thick, viscous and sticky causing clinical manifestations. In the respiratory tract, this viscid sputum prevents the adequate hydration of the mucociliary epithelium and also predispose the epithelium to recurrent infections. The surface epithelium starts acquiring damage and chronic inflammation results. Sloughing of the epithelium into the mucus causes the production of viscid sputum.

In endocrinal organs, the thick, sticky mucus gathers in the duct and causes obstruction. Similar pathologies occur in the gastrointestinal tract and reproductive organs. The salt content in sweat is also increased due to defects in Cystic Fibrosis Transmembrane Conductance Regulator.

There are no guidelines for prevention of cystic fibrosis.

Cystic fibrosis is an autosomal recessive disease resulting from mutations in the gene encoding a membrane chloride channel called Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) that is present in the epithelia of various tissues of the body, including the lungs, pancreas, stomach, intestines, endocrinal and reproductive organs.

Dysfunction of this channel causes the production of abnormal secretions in these organs, which ultimately leads to a multitude of pulmonary and extra-pulmonary manifestations.

With proper management, the life expectancy of most patients exceeds 40 years.

Cystic fibrosis results from a genetic defect that causes a malfunction in a protein present in tissues in many organs of the body including the lungs, pancreas, stomach, intestines, endocrinal and reproductive organs.

This causes the production of abnormal secretions and symptoms related to these organs result.

The management of cystic fibrosis has improved in recent years and the prognosis is fairly good.

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3. Consortium CFGA. Cystic fibrosis mutation database. CFMDB Statistics http://www.genet.sickkids.on.ca/cftr/StatisticsPage.html. Accessed Feb 21, 2014.
4. Mitchell K, Robbins, Abbas, Fausto, Nelson. Robbins Basic Pathology. Saunders/Elsevier; 2007.
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8. Yankaskas JR MGJ. Lung transplantation in cystic fibrosis: consensus conference statement. Chest. January 1998;113(1):217-226.
9. Moorcroft AJ DM, Webb AK. Exercise limitations and training for patients with cystic fibrosis. Disabil Rehabil.20(6-7):247-253.
10. Elborn JS SD, Britton JR. Cystic fibrosis: current survival and population estimates to the year 2000. Thorax. Dec 1991;46(12):881-885.