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Cystic fibrosis is consistently accompanied by the ability to taste and smell salt, sweet, sour, and bitter substances in solution at concentrations much more dilute than those at which the substances are detectable by normal persons. These abnormal thresholds are not affected by the administration of carbohydrate-active steroids.
Cystic fibrosis, a disease thought to be transmitted as a recessive genetic trait, is found as a disease in about one in 1,000 to one in 10,000 births. It involves all of the exocrine glands with presenting symptoms dependent upon the extent of involvement of any group of glands. Many aspects of the disease can be corrected by substitution therapy. This applies particularly to the use of animal pancreas for the steatorrhea and salt for prevention of heat prostration. Unfortunately, the obstructive pulmonary disease with secondary bronchial infections can only be treated symptomatically by the use of mucus thinning agents, postural drainage, and antibiotics. Nevertheless, longevity can be increased and a great deal of hope offered to the families of these unfortunate children by careful supervision of their medical care.
Cystic fibrosis-related diabetes mellitus (CFRD) is the most frequent comorbidity in cystic fibrosis. Its clinical relevance is stressed by the association with increased mortality, and decreased pulmonary and nutritional status. An annual oral glucose tolerance test (OGTT) is recommended as a screening test for CFRD, but this is often not realised because of its time- and resource-consuming nature. Therefore, alternative approaches are welcome. In 2003, the American Diabetes Association (ADA) lowered the cut-off point separating normal from elevated fasting plasma glucose from <6.1 mmol x L(-1) to <5.6 mmol x L(-1), suggesting the performance of an OGTT only in those with impaired fasting glucose (IFG; range 5.6-6.0 mmol x L(-1)). The current authors tested whether this approach was reliable for the early identification of patients with CFRD. OGTTs from 1,128 patients (53% males; 47% females; median age 17.1 yrs) were available for analysis. A total of 101 (8.9%) OGTTs were classified as diabetic. The new ADA criteria for IFG increased the sensitivity to 82% (versus 65%) and decreased the specificity to 70% (versus 94%) compared with the old criteria used to identify patients with diabetic OGTTs. In conclusion, the American Diabetes Association approach of using impaired fasting glucose as an indication for performing selective oral glucose tolerance tests is definitely unsuitable when aiming at the early identification of patients with cystic fibrosis-related diabetes mellitus, and it cannot replace annual oral glucose tolerance tests.
Cystic fibrosis is a chronic, life-threatening inherited disease which poses severe physical, psychological and social problems for the sufferer and his or her family. Frances Duncan-Skingle and Fiona Foster present an overview of recent developments in the clinical management of this distressing condition.
Cystic fibrosis patients have an increased requirement for calories and probably for all the major nutrients. The newer, enteric-coated granular preparations of pancreatic enzyme are more effective than preceding preparations and should permit a normal fat intake. Recent work has emphasized the interdependence of respiratory disease and nutrition.
Cystic fibrosis is a devastating illness affecting the young, in particular, and for which no cure exists. Any new treatment which might improve quality of life and prolong survival is to be welcomed but, so far, there are still several questions to be answered concerning the role of dornase-alfa.
Cystic fibrosis is common genetic disease, inherited via an autosomal recessive pattern. The authors present state of knowledge, concerning genetic and diagnostic problems, clinical signs and treatment. Several clinically evident involvements of the eye such as xerophthalmia, deficiencies of tear film, functional changes of the optic nerve, papilloedema, nyctalopia, retinal hemorrhages, have been described.
Cystic fibrosis has been regarded as a disease of infants. Yet, with improved management, a high percentage of patients how survive into young adulthood. In addition, mild cases may not be detected until long after infance. The older patient often has minimal symptoms, and the majority are socially active and productive.
Cystic fibrosis (CF) is characterized by symptoms related to pulmonary dysfunction and pancreatic insufficiency. Constipation, though a frequent complaint of patients with CF, receives less attention. We report a case of acquired segmental megacolon and constipation necessitating surgical colonic resection in an adult patient with CF. The differential diagnosis and possible causes of megacolon in this setting are discussed.
Cystic fibrosis patients are exceptionally prone to colonisation by a narrow spectrum of pathogenic bacteria. Since pulmonary infection presently, and for the foreseeable future, plays such a major role in CF lung disease, we review the microbes that are classically associated with CF and the virulence, inflammatory potential and resistance mechanisms which contribute to the reduction in life expectancy for colonised CF patients.
Cystic fibrosis lung inflammation is early, sustained and severe and would justify an anti-inflammatory treatment. At present, the inhaled corticosteroid treatment did not give evidence of efficacy, contrary to the oral presentation, but at the cost of side effects. Azithromycin gives more encouraging results with a good tolerance. New molecules are in the process of validation.
Cystic fibrosis primarily affects tissues containing secretory epithelia, but not all of them become diseased. The distribution of clinical disease corresponds closely to the effect on net anion-linked water secretion, which is reduced across epithelia of endodermal and mesodermal origin but not those of ectodermal origin. Although the gene is expressed in all secretory epithelia, its effects on water secretion are bypassed in those of ectodermal origin.
Cystic fibrosis occurs in 1 in 2000 children and the majority now reach adulthood. The disease is a complex multisystem disorder which is likely to challenge anaesthetists with increasing frequency. In this review the presentation and genetics of the disease are briefly described, followed by a detailed account of the pathophysiology relevant to anaesthesia. The pre-operative assessment and conduct of anaesthesia are discussed and some suggestions made regarding management.
Cystic fibrosis patients suffer from recurrent and chronic lung infections mainly caused by Staphylococcus aureus, Haemophilus influenzae and Pseudomonas aeruginosa. The fluoroquinolones, notably ciprofloxacin and ofloxacin, represent an important addition to the therapy of P. aeruginosa infections. They offer the possibility of effective oral treatment for early colonisation as well as chronic infections, even in children. They are associated with only few and mild adverse effects. Development of resistance represents an increasing problem.
Cystic fibrosis (CF) is a monogenic disorder and is therefore a good candidate for gene therapy. Initial clinical trials provided proof-of-principle for gene transfer to the airways, but efficiency was low and likely to be insufficient for clinical benefit. Here, we review the progress in CF gene therapy over the last 12 months, including recent advances in viral and non-viral gene transfer agents and novel strategies, such as RNA repair and stem cell gene therapy.
Cystic fibrosis (CF) is one of the commonest lethal inherited conditions among Caucasians. It affects multiple organ systems and exhibits a range of clinical problems of varying severity. Life expectancy has improved in recent years as treatment regimes have become more intensive, but current treatments are expensive, often time consuming and may affect quality of life. This review summarises the treatments currently used in the management of patients with CF, and the evidence for these.
Cystic fibrosis (C.F.) is characterised by low serum levels of essential fatty acids (E.F.A.). However, the fatty-acid pattern does not totally resemble that of dietary E.F.A. deficiency. The differences suggest a reduction in the desaturation of E.F.S. It is not known whether this defect is the primary lesion in C.F. or is the result of tissue damage in the disease. It is proposed that C.F. patients might have increased linoleic-acid requirements, and possibly specific requirements for its desaturation products.
Cystic fibrosis is a genetic disorder of the exocrine glandular function leading to the formation of thick mucus in the nasal and paranasal cavities. Nearly all the patients develop sinus disease but not all have complaints. Two different sinus diseases can be found: chronic sinusitis and nasal polyposis. Only the symptomatic cases have to be treated. Medical treatment consists of antibiotherapy for chronic sinusitis and nasal steroid sprays for nasal polyps. Sinus surgery is frequently needed in case there is a persistence of symptoms (and before lung transplantation).