Cystic fibrosis
Article Abstract:
Cystic fibrosis is an inherited disease that affects the pancreas, respiratory system, and sweat glands. The disease usually starts in infancy and is characterized by chronic respiratory infection, impaired function of the pancreas, and increased levels of electrolytes in sweat. The primary defect causing cystic fibrosis is not known. The average age of death from cystic fibrosis increased from six months in 1959 to 17 years in 1986. Prolonged survival from this disease results from improvements in antibiotics; nutrition; pancreatic enzyme supplements; management of meconium ileus, or bowel obstruction by feces; diagnosis of milder forms of the disease; and the development of specialized centers. However, the effectiveness of current methods of managing cystic fibrosis has not been adequately studied. The diagnosis, genetics, and screening of cystic fibrosis are discussed. Patients must be continuously monitored to evaluate their progress, detect complications or noncompliance of treatment, and to continue parent and patient education in the management of the disease. Patients should be vaccinated against measles, pertussis, influenza, and possibly varicella and pseudomonas, to prevent worsening of infections. Management of cystic fibrosis with antibiotics, bronchodilators (drugs that dilate the airways and improve airflow into the lungs), steroids, improved nutrition, and physiotherapy are described. Some complications of cystic fibrosis include pneumothorax, the collection of air or gas in the lung cavity; gallstones; meconium ileus; nasal disorders; arthritis; and various social and psychiatric problems. Regional centers are beneficial because they provide better laboratory and paramedical facilities, wards that are specialized for patients with cystic fibrosis, support from other patients with cystic fibrosis and their families, and a team approach. The current methods for managing cystic fibrosis have increased survival and improved health in childhood of patients with cystic fibrosis, but require further evaluation. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Archives of Disease in Childhood
Subject: Health
ISSN: 0003-9888
Year: 1990
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Haemophilus infection in cystic fibrosis
Article Abstract:
Strains of Hemophilus influenzae (HI) occur in both encapsulated and nonencapsulated forms. The nonencapsulated forms are primarily responsible for local mucosal disease of the respiratory tract and are among the most important bacterial pathogens in childhood diseases, causing otitis media (inflammation of the middle ear) and sinusitis (inflammation of the sinuses). These organisms and strains of H. parainfluenzae frequently colonize the lower respiratory tracts of patients with cystic fibrosis (CF). This group of organisms is divided into biotypes on the basis of their biochemical characteristics (indole production, ornithine decarboxylase, and urease). Biotype 1 of HI was positive for the three biochemical tests, and was isolated more frequently from CF patients than from healthy control subjects. The significance of HI isolation rates and the distribution of biotypes in CF patients under the age of 12 was studied. Children with asthma were used as controls. Twenty-seven children, matched for age and sex, were in each group. HI biotype 1 was isolated more frequently from the lower respiratory tract in cases of CF than from the asthma controls. Isolation rates of HI increased preceding exacerbations of CF, and clinical improvement was accompanied by a reduction in isolation rates after antibiotic therapy. HI occurs more frequently than Staphylococcus aureus or Pseudomonas aeruginosa in these patients. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Archives of Disease in Childhood
Subject: Health
ISSN: 0003-9888
Year: 1990
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Pathogenesis and management of aspergillosis in cystic fibrosis
Article Abstract:
Cystic fibrosis (CF) is an inherited disease characterized by inadequate function of the pancreas and chronic lung infection. A major complication in CF is invasion by Aspergillus fumigatus, a mold that commonly grows on rotting vegetables and plants, into diseased airways, which causes allergic bronchopulmonary aspergillosis. Initially described in 1965, aspergillosis may be affecting up to 11 percent of people with CF, and is diagnosed if six of the following criteria are met: (1) elevated levels of serum immunoglobulin E (IgE) and specific IgE to Aspergillus fumigatus, (2) reversible constriction of the bronchial airways, (3) abnormally high levels of eosinophils (a type of white blood cell) in the blood, (4) positive immediate skin test, sputum culture or serum precipitins test for A. fumigatus, (5) chronic dilation of bronchial passages, (6) brown plugs in sputum, and (7) late skin test reaction. Aspergillosis is usually recognized when antibiotic treatment is ineffective for improving affected areas on the chest X-ray. A collapsed lung may result due to bronchial obstruction, and in some cases, invasive aspergillosis may be fatal. Trials using antifungal drugs have been done, but serious liver damage was reported. Oral steroids appear to be the most effective treatment for preventing lung damage, however, long-term steroid therapy is controversial because of well-known side effects of these drugs. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Archives of Disease in Childhood
Subject: Health
ISSN: 0003-9888
Year: 1990
User Contributions:
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