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• W2078957057 abstract "Cystic fibrosis is a systemic disorder transmitted by autosomal recessive genetics which is still characterized as the most common fatal genetic disease in whites. Approximately 5 percent of the US white population are carriers of the CF gene. Carriers of the CF mutation do not manifest symptoms, a heterozygote advantage has not been demonstrated, and there is at present no carrier test that can be applied to the general population. The basic biochemical abnormality in CF is not known, although rapid progress is being made toward that end. Molecular biologists using recombinant DNA techniques have located the CF gene on a small portion of the long arm of chromosome 7, providing a major step toward isolating the gene itself and finally understanding the biochemical pathways involved in the clinical manifestations of CF. All fields of medicine may be touched by this disease, and it is possible for the astute physician to find adult patients with CF while evaluating chronic sinusitis, asthma, pancreatic insufficiency, sprue-like symptoms, azoospermia, or cirrhosis and portal hypertension. Similarly, there is great variability in the clinical course of this disease, and deterioration does not occur at the same rate in all patients. Although CF is a systemic disorder, it is the pulmonary disease, recurrent pulmonary bacterial infections superimposed on chronic colonization of the airways and resultant inflammation leading to destructive disease of the airways (bronchiectasis), which causes most of the morbidity. Ninety percent of CF patients die of respiratory failure. Despite the promise that molecular biology holds for the ultimate therapy for CF, standard care for pulmonary disease due to CF today remains based on prompt initiation of effective treatment of the disease of the airways. In 1988, widespread diagnostic and therapeutic programs have made possible an increased median survival to almost 27 years in CF centers. In the paragraphs that follow, we will present a limited summary of both new and old therapies that remain controversial. The purpose of such a, necessarily, circumscribed review is to highlight therapeutic modalities of promise and to expose common treatments based on inadequate data, in an attempt to provide debate between colleagues, to stimulate clinical trials, and to suggest directions for future clinical investigation. The lungs in CF are morphologically normal at birth, and the onset of pulmonary pathology may occur anytime after birth, with varying severity of the resulting respiratory signs and symptoms. By the time patients with CF enter adulthood, only 2 percent lack evidence of pulmonary disease by history, by chest radiographic examination, and by pulmonary function tests. Recent morphometric work performed on lungs from patients with CF obtained at autopsy indicate that the airway disease and pulmonary remodeling are irregularly distributed and that the upper lobe segments are disproportionately involved.1Tomashefski JF Bruce M Goldberg HI Dearborn DG. Regional distribution of macroscopic lung disease in cystic fibrosis.Am Rev Respir Dis. 1986; 133: 535-540PubMed Google Scholar Careful light-microscopic and electron microscopic studies have failed to identify a single lesion specific for CF. In contradistinction to the usual pseudomonas pneumonia, the lesion in CF is largely confined to the airway, with destruction of the wall leading to bronchiolitis and eventually bronchiectasis. As the airway disease becomes established, patchy areas of parenchymal involvement (bronchopneumonia) may become more apparent (Fig 1). Many adults will have a long history of respiratory infections which started in childhood. Pulmonary disease due to CF may present acutely with staphylococcal pneumonia or insidiously with persistent cough following an apparent viral upper respiratory infection. The acquisition of mucoid Pseudomonas aeruginosa in respiratory secretions marks the beginning of a slow decline in pulmonary function. Respiratory infection ‡“Respiratory infection” will be used throughout this text and refers to the early colonization of the airways, the destructive disease of the airways, and the patchy pneumonic parenchymal process. Because we believe that the presence of P aeruginosa in airway secretions or specimens of sputum always represents a pathogen, mandating therapy and heralding a poor prognosis, the term, “colonization,” will be avoided. Pseudomonas does not exist as a benign commensal in this disease. in CF follows a smoldering course punctuated by acute exacerbations (in part caused by viral agents) superimposed upon a baseline of chronic productive cough and bacterial colonization, most commonly caused by mucoid P aeruginosa. Curiously, during these airway infections, temperature elevations are uncommon, routine blood cell counts are generally not helpful, and positive cultures of blood are practically unknown. Greater than 90 percent of the deaths due to CF occur during one of these pulmonary infections, and it is a rare occurrence that pseudomonas is eradicated from the sputum, regardless of the antibiotic regimen selected. Although P aeruginosa is the most common bacterial isolate obtained from the airways of adult patients with CF, recently new multiresistant organisms have emerged. One nonaeruginosa strain, P cepacia, has been associated with increased morbidity and premature death in a subgroup of patients with CF. During acute flare-ups of pulmonary symptoms, 5 to 15 percent of the bacterial strains isolated from samples of sputum may be nonaeruginosa pseudomonas. In large measure, improvement in the survival of patients with CF reflects timely use of newer, more potent antibiotics. The mechanism of action and indications for these antibiotics have been adequately reviewed in recent publications;2Hata JS Fick RB. Pseudomonas aeruginosa and the destructive airways disease of cystic fibrosis. In: Fick RB, ed. Inflammatory disorders of the airways.Clinics Chest Med. 1988; 9: 679-691Google Scholar, 3Krilor LR Blumer JL Stern RC. Imipenem/cilastatin in acute pulmonary exacerbation of cystic fibrosis.Rev Infect Dis. 1985; 7: S482-S489Crossref Google Scholar, 4Walker RC Wright AJ. Symposium on antimicrobial agents: 3. the quinolines.Mayo Clin Proc. 1987; 62: 1007-1012Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar, 5Kelly HW Lovato C. Antibiotic use in cystic fibrosis.Drug Intell Clin Pharm. 1984; 18: 772-783Crossref PubMed Scopus (28) Google Scholar however, important clinical questions remain unanswered. What is the most effective protocol for administration of these potent antibiotics? Should they be administered orally, by nebulizer, or parenterally? If the parenteral route is selected, should the antibiotics be given only to inpatients, or can these antibiotics be safely administered in the outpatient home setting? Should antibiotics be prescribed continuously or for the first week of each month? Should the choice of antimicrobials be dictated by the results of cultures of sputum? Good answers do not exist for many of these commonly asked clinical questions. Collaborative multicenter studies are needed to quickly provide answers which, with confidence, provide guidance to those caring for patients with CF. Often the limiting factor between in-hospital and at-home treatment is parenteral antibiotics. With improvements in the availability of at-home health care, parenteral antibiotic therapy can be easily achieved at home with success equal to that of in-hospital treatment in selected patients.6Donati MA Guenetti G Auerbach H. Prospective controlled study of home and hospital therapy of cystic fibrosis pulmonary disease.J Pediatr. 1987; 111: 28-33Abstract Full Text PDF PubMed Scopus (81) Google Scholar, 7Winter RJD Deacock SJ George RJD. Self-administered home intravenous antibiotic therapy in bronchiectasis and adult cystic fibrosis.Lancet. 1984; 1: 1338-1339Abstract PubMed Scopus (45) Google Scholar, 8Gilbert J Robinson T Littlewood JM. Home intravenous antibiotic treatment in cystic fibrosis.Arch Dis Childhood. 1988; 63: 512-517Crossref PubMed Scopus (46) Google Scholar This can be achieved with either peripheral intravenous access or indwelling central venous access. It should be noted that antibiotics without appropriate attention to concomitant chest physiotherapy and nutrition will have less than optimal results. In a controlled, prospective trial, Donati et al6Donati MA Guenetti G Auerbach H. Prospective controlled study of home and hospital therapy of cystic fibrosis pulmonary disease.J Pediatr. 1987; 111: 28-33Abstract Full Text PDF PubMed Scopus (81) Google Scholar compared at-home and in-hospital antibiotic therapy. The patients (41 in each group) were matched for age, sex, pulmonary function, and arterial blood levels. Intravenous antibiotics, usually a semisynthetic penicillin and an aminoglycoside, were employed with IV catheters (“heparin locks”) maintained by registered nurses. The care at home compared favorably with hospitalization, with both groups demonstrating improvements in the results of pulmonary function tests with similar mean numbers of days of treatment. Moreover, there was no difference in the interval between exacerbations seen in the two groups or between the number of patients requiring further antibiotic therapy; however, care at home was significantly less costly and allowed the individual patient a greater opportunity to participate in work or school. Winter et al7Winter RJD Deacock SJ George RJD. Self-administered home intravenous antibiotic therapy in bronchiectasis and adult cystic fibrosis.Lancet. 1984; 1: 1338-1339Abstract PubMed Scopus (45) Google Scholar initiated parenteral therapy during hospitalization followed by continuing intravenous antibiotic therapy at home or provided therapy totally at home. There was no difference in mean time until relapse between conventional therapy (ie, antibiotics given entirely in the hospital) and therapy given entirely or partly at home. The major problems recorded with patients participating at home was blockage of the IV cannula, involving 14 cannulas during 116 days of care. No major complications were encountered, eg, thrombophlebitis, extravasation of the antibiotic, or bleeding. These studies suggest that home-based, intravenous antibiotic therapy can be an effective alternative for selected individuals with exacerbations of their CF-related pulmonary disease. Before implementation of outpatient parenteral antibiotics, the physician must consider the following: (1) the patient's degree of illness and ability for self-care; (2) the potential for ancillary support, including chest physical therapy and nutritional supplementation; (3) the availability of individuals to care for the IV catheters; and (4) the availability of close medical follow-up, as day-by-day contact with the physician is generally terminated when the patient returns home. Several relatively small clinical trials have demonstrated the safety, absence of untoward effects, low rate of development of bacterial resistance, and efficacy of aerosolized antibiotics (Table 1). This route has been used to deliver aminoglycosides, broad-spectrum penicillins, and cephalosporins. A particular advantage is that this therapy may be used at home. This presents an alternative to the rather short list of oral pseudomonicidal agents used in daily outpatient maintenance therapy.Table 1Aerosolized Antibiotic Treatment for Pseudomonas in CF AirwaysAntibiotic ClassnObservationsAminoglycosides149Loss of pseudomonas more commonly observed; improved spirometry; resistance same as systemic usage44Boxerbaum B Pittman S Doershuk CF Stern RC Matthews LW. Use of Gentamicin in children with cystic fibrosis.J Infect Dis. 1971; 124: S293-S295Crossref Scopus (12) Google Scholar, 45Baran D Dachy A Klastersky J. Concentration of gentamicin in bronchial secretions of children with cystic fibrosis or tracheostomy.Int J Clin Pharmacol. 1975; 12: 336-341Google Scholar, 46Stephens D Garey N Isles A Levison H Gold R. Efficacy of inhaled tobramycin in the treatment of pulmonary exacerbations in children with cystic fibrosis.Pediatr Infect Dis. 1983; 2: 209-211Crossref PubMed Scopus (88) Google Scholar, 47Kun P Landau LI Phelan PD. Nebulized gentamicin in children and adolescents with cystic fibrosis.Aust Paediatr J. 1984; 20: 43-45PubMed Google ScholarBroad-spectrum penicillins26Subjective improvement; weight gain and fewer hospitalizations; gains in spirometry equivocal; resistance equal to placebo48Hodson ME Penketh ARL Batten JC. Aerosol carbenicillin and gentamicin treatment of Pseudomonas aeruginosa infection in patients with cystic fibrosis.Lancet. 1981; 2: 1137-1139Abstract PubMed Scopus (244) Google Scholar, 49Wall MA Terry AB Eisenberg J McNamara M Cohen R. Inhaled antibiotics in cystic fibrosis [letter].Lancet. 1983; 1: 1325Abstract PubMed Scopus (72) Google ScholarCephalosporins18Fewer hospitalizations; improved pulmonary function; resistance equivalent to nonaerosol controls30Sutton RP Gensel HG Innes N Davidson J Smith FW Legge JS et al.Use of nebulized saline and terbutaline as adjuncts to chest physiotherapy.Thorax. 1988; 43: 57-60Crossref PubMed Scopus (59) Google Scholar Open table in a new tab Inhaled antibiotics have been used for over a decade in the treatment of CF-related airway disease caused by P aeruginosa, and reported studies are of two types. They examine use of aerosolized antibiotics in the management of acute exacerbations of CF-related pulmonary disease, as well as application of this route on a long-term basis for maintenance therapy. If this mode of delivery is selected, it is advised that the aminoglycoside and broad-spectrum penicillin (carbenicillin; ticarcillin) be administered separately, as gentamicin has been shown to be chemically inactivated by carbenicillin or piperacillin. Many of these protocols have not been properly controlled, but the results document that the emergence of resistant strains is uncommon. It is interesting to note the generally favorable results of these protocols, despite the fact that P aeruginosa has been rarely eradicated from the airway secretions. This should not be surprising because we seldom sterilize the sputum with any treatment, including parenteral pseudomonicidal antibiotics delivered in the hospital. Sterilization of secretions in these patients with distorted airways is seldom possible and suggests that an appropriate goal of therapy in patients with CF may be simply reduction of the bacterial burden, rather than eradication of pseudomonas. Therapeutic clinical trials of aerosolized antibiotics in CF have yielded conflicting results because of the lack of uniformity in grading the severity of the pulmonary disease of the patients with CF enrolled, the variable sizes of particles generated, and the differing antibiotic dosing protocols in each of these studies. Recently, investigators have analyzed an inexpensive, commercially available aerosolizer (Centimist; Intec Corp) and, using an immunoenzymatic assay for gentamicin, calculated that 7.7 percent of the original amount of antibiotic placed in the chamber was deposited in airway secretions. The peak levels in the sputum (mean, 377µg/ml) greatly exceeded MIC and MBC levels for clinical isolates of P aeruginosa and, yet, serum levels of the antibiotic were not detected. Therefore, high concentrations of pseudomonicidal antibiotics may be safely and selectively delivered to the airways in CF. In contrast to earlier studies with inhaled antibiotics in the adult ICU, which showed no prevention of pneumonia and selection of resistant organisms,9Feeley TW du Moulin GC Hedley-Whyte J Bushnell LS Gilbert JP Feingold DS. Aerosol polymyxin and pneumonia in seriously ill patients.N Engl J Med. 1975; 293: 471-475Crossref PubMed Scopus (197) Google Scholar the emergence of resistant strains does not occur. Often, clinical improvement is reported when aerosolized antibiotics are used on a long-term basis in maintenance therapy. Our knowledge regarding the physics of the generation and delivery of these droplets has improved greatly in the last six to eight years. Nevertheless, before this mode of therapy can be widely recommended for patients with CF, additional carefully controlled clinical trials should be completed. Because of the ongoing bacterial load in the sputum, many have advocated either long-term continuous or intermittent administration of oral antibiotics. This approach is supported by a double-blind crossover study using cephalexin that demonstrated improved growth, fewer pulmonary exacerbations, and less non-pseudomonas organisms in the sputum in the treated group; however, more mucoid Pseudomonas species were isolated in the treated group, suggesting that there may be adverse long-term sequelae of long-term therapy.10Loening-Bauke VA Mischler E Myers MG. A placebo-controlled trial of cephalexin therapy in the ambulatory management of patients with cystic fibrosis.J Pediatr. 1979; 95: 630-637Abstract Full Text PDF PubMed Scopus (64) Google Scholar The change in the predominant flora from Staphylococcus to pseudomonas in patients with CF had been postulated to be due to oral antibiotics selecting for the pseudomonas.11Kulczycki LL Murphy TM Bellanti JA. Pseudomonas colonization in cystic fibrosis.JAMA. 1978; 240: 30-34Crossref PubMed Scopus (82) Google Scholar Currently, there is a multicenter trial of long-term oral antibiotic therapy underway to help answer the question of whether or not this is beneficial. There is compelling clinical evidence to suggest oral therapy is beneficial in acute pulmonary exacerbations of CF-related pulmonary disease, despite the fact that until recently there have been no oral antibiotics with strong antipseudomonas properties.5Kelly HW Lovato C. Antibiotic use in cystic fibrosis.Drug Intell Clin Pharm. 1984; 18: 772-783Crossref PubMed Scopus (28) Google Scholar While it is entirely possible that improvement is merely coincidental, it may be that suppression or elimination of other sensitive organisms helps decrease the volume and viscosity of the sputum, thereby facilitating mucociliary clearance, without having a primary effect on the pseudomonas.12Hodson ME Penketh ARL Batten JC. Aerosol carbenicillin and gentamicin treatment of P aeruginosa infection in patients with cystic fibrosis.Lancet. 1981; 2: 1127-1129Google Scholar Oral antiviral antibiotics such as amantadine should be considered when acute influenzal infections are prevalent in the communities. Several clinical studies have demonstrated that the bacterial species identified in the culture of specimens of expectorated sputum are representative of the bacteriologic findings obtained from resected pulmonary segments, protected catheter brushes, and percutaneous needle aspirates of the lungs of patients with CF. It is our belief that the selection of antimicrobials should be directed by the results of cultures of sputum and the in vitro sensitivities, despite the fact that it is well recognized that in vitro sensitivity does not necessarily predict success in clearing the airway of the particular pathogen(s) being treated or predict clinical improvement. In fact, some studies have documented improvement despite placebo or “inappropriate” antibiotics.13Gold R Carpenter S Hewter H Corey M Levinson H. Randomized trial of ceftazidime versus placebo in the management of acute respiratory exacerbations in patients with cystic fibrosis.J Pediatr. 1987; 111: 907-913Abstract Full Text PDF PubMed Scopus (63) Google Scholar Clinicians are best advised to prescribe antimicrobial chemotherapeutic agents at high doses because the serum-airway partition coefficient for many classes of antibiotics has reproducibly been shown to approximate 5:1; that is, the level in the airways will be only 20 to 30 percent of a simultaneously obtained serum concentration (Table 2). The newly released quinolones and the much older agent, chloramphenicol, appear to be exceptions to this formula. Finally, the value of quantitative cultures of sputum in many clinical settings has been argued, but may be of value in the patient with CF. Quantitating the bacterial burden is useful in following the response to therapy and confirming the clinical impressions of worsening or improving in patients with bronchiectasis.14Smith AL Redding G Doershuk C. Sputum changes associated with therapy for endobronchial exacerbation in cystic fibrosis.J Pediatr. 1988; 112: 547-554Abstract Full Text PDF PubMed Scopus (119) Google Scholar The presence of pseudomonas in the sputum has been related to the severity of pulmonary disease as shown by clinical deterioration and death and by pulmonary function tests and chest roentgenograms.11Kulczycki LL Murphy TM Bellanti JA. Pseudomonas colonization in cystic fibrosis.JAMA. 1978; 240: 30-34Crossref PubMed Scopus (82) Google Scholar Having said this, it is important to emphasize that the greatly distorted airways of these patients continuously harbor bacteria and will infrequently yield a sterile culture of sputum following aggressive antibiotic treatment, although there may be a fall in the density of organisms in the sputum.14Smith AL Redding G Doershuk C. Sputum changes associated with therapy for endobronchial exacerbation in cystic fibrosis.J Pediatr. 1988; 112: 547-554Abstract Full Text PDF PubMed Scopus (119) Google ScholarTable 2Antibiotic Classes and Pulmonary Alveolar-Capillary Partition Coefficients*Studied in variety of patients with infectious or noninflammatory pulmonary diseases.AntibioticPercentage of Serum Concentration Achieved in SputumAminoglycosidesAmikacin10-30Gentamicin20-40Tobramycin20-67Netilmicin20Sisomicin40PenicillinsTicarcillin6-10Piperacillin10-14Azlocillin4-10Dicloxacillin2.5Cephalosporins(5-10)Ceftazidime2-15QuinolonesCiprofloxacin200* Studied in variety of patients with infectious or noninflammatory pulmonary diseases. Open table in a new tab Novel use of nonbactericidal antibiotics in an attempt to alter the synthesis and release of pseudomonas virulence factors may be a profitable area for future studies. Preliminary studies by Rennard et al15Rennard S Yam P Ertl R Fick RB Waldman R. Hartman PA Clindamycin inhibition of pseudomonas elastase production. American Society for Microbiology, Washington D.C.1986: A6Google Scholar indicate that subinhibitory levels of clindamycin block the ability of P aeruginosa to release an inflammatory and destructive bacterial metalloenzyme, elastase. Others have reported that subinhibitory concentrations of clindamycin inhibit production of hemolysin by Escherichia coli. Subinhibitory levels of aminoglycosides may have beneficial effects in CF by inhibiting excretion of alginate and the production of siderophores.16Morris G Brown MRW. Novel modes of action of aminoglycoside antibiotics against Pseudomonas aeruginosa.Lancet. 1988; 1: 1359-1360Abstract Scopus (22) Google Scholar These and other nontraditional potential mechanisms of antibiotic activity may help explain therapeutic successes. Corticosteroids have been used in patients with CF in an attempt to abrogate pulmonary inflammation thought to result from type 3 (immune complex-mediated) hypersensitivity reactions. A recently completed randomized, double-blind placebo-controlled study of alternate-day therapy with prednisone (2 mg/kg to maximum of 60 mg) in children with CF (ages 1 to 12 years) concluded after a four-year follow-up that the prednisone-treated group had significant advantages for height, weight, vital capacity, FEV1, peak flow, and number of required hospitalizations.17Auerbach HS Williams M Kirkpatrick JA Colter HR. Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis.Lancet. 1985; 2: 686-688Abstract PubMed Scopus (246) Google Scholar It remains unclear if the same long-term corticosteroid treatment protocol would prove beneficial in adolescent and adult patients with CF who have a great deal more permanent structural pulmonary damage. It is known that short-term therapy with prednisone (20 to 30 mg/day for three weeks) does not significantly increase pulmonary function in adult patients with CF18Pantin CFA Stead RJ Hodson ME Batten JC. Prednisolone in the treatment of air flow obstruction in adults with cystic fibrosis.Thorax. 1986; 41: 34-38Crossref PubMed Scopus (26) Google Scholar and may expose the patient with CF to an increased risk of developing pneumothorax. Although the use of corticosteroids in an alternate-day program appears to be free of potential corticosteroid side effects, it still remains unclear if all patients with CF might benefit from this therapy. An expanded multicenter trial of alternate-day steroids is underway. Future studies could examine other immunomodulators such as the cyclooxygenase inhibitors, eg, ibuprofen. This agent may be expected to decrease airway inflammation mediated by the cyclooxygenase pathway (prostaglandins and thromboxanes). Frequently, hospitalization is necessary despite use of antibiotics, exercise, and daily postural drainage performed as an outpatient. Nonemergent hospitalization may be warranted because of an insidious downhill clinical course including cough, dyspnea, anorexia, weight loss, and lack of energy, making daily activities of work or school impossible. There is no question that aggressive therapy combining antibiotics, chest percussion, and nutritional support leads to subjective improvement, improvement in gas exchange, improvement in pulmonary function, and radiographic improvement in most patients, unless there is severe fixed damage to the airways.14Smith AL Redding G Doershuk C. Sputum changes associated with therapy for endobronchial exacerbation in cystic fibrosis.J Pediatr. 1988; 112: 547-554Abstract Full Text PDF PubMed Scopus (119) Google Scholar, 19Redding GJ Restuccia R Cotton EK Brooks JG. Serial changes in pulmonary functions in children hospitalized with cystic fibrosis.Am Rev Respir Dis. 1982; 126: 31-36PubMed Google Scholar Traditionally, this aggressive therapy has been achieved in the hospital. Many CF centers advocate hospitalization only after progressive deterioration on vigorous outpatient regimens, whereas some centers plan routine elective admissions with hopes that this will have a prophylactic effect in slowing the progression of ongoing pulmonary damage. While in-hospital treatment may be considerably more expensive, especially in view of the recent successes with at-home management,6Donati MA Guenetti G Auerbach H. Prospective controlled study of home and hospital therapy of cystic fibrosis pulmonary disease.J Pediatr. 1987; 111: 28-33Abstract Full Text PDF PubMed Scopus (81) Google Scholar, 7Winter RJD Deacock SJ George RJD. Self-administered home intravenous antibiotic therapy in bronchiectasis and adult cystic fibrosis.Lancet. 1984; 1: 1338-1339Abstract PubMed Scopus (45) Google Scholar, 8Gilbert J Robinson T Littlewood JM. Home intravenous antibiotic treatment in cystic fibrosis.Arch Dis Childhood. 1988; 63: 512-517Crossref PubMed Scopus (46) Google Scholar supervised therapy in the hospital remains the “gold standard.” Patients whose condition fails to improve as outpatients should be given a chance to improve in the hospital. It may be that several factors besides parenteral antibiotics and chest percussion contribute significantly to recovery, such as rest, relaxation, rehydration, and compliance.13Gold R Carpenter S Hewter H Corey M Levinson H. Randomized trial of ceftazidime versus placebo in the management of acute respiratory exacerbations in patients with cystic fibrosis.J Pediatr. 1987; 111: 907-913Abstract Full Text PDF PubMed Scopus (63) Google Scholar Nevertheless, indications for this type of expensive hospitalization and the duration of hospitalization are imprecise. Empirically, hospitalizations have ranged between 10 and 21 days for most patients. The ten-day minimums were selected after repeated failures with stays of five to seven days (as would be customary procedure for an uncomplicated “pneumonia”). By 21 days, most patients have maximized any potential benefit and have made little or no progress in the two or three days just before discharge. Why the ten-day to 21-day period seems to be optimal is uncertain, but it may pertain to factors such as nutritional rehabilitation, muscle fatigue, and accumulation of antibiotics in the airways.20Mendelman PM Smith AL Levy J Weber A Ramsey B Davis RL. Aminoglucoside penetration, inactivation, and efficacy in cystic fibrosis sputum.Am Rev Respir Dis. 1985; 132: 761-765PubMed Google Scholar Increasingly, as the pulmonary status of patients with CF begins to improve in the hospital, preparations can be made to complete treatment comfortably at home, where usual activities may gradually be resumed. The cost savings for these home-care patients are considerable. Depending on the timing and intensity of the patients progressive pulmonary disease, hospitalization may provide long-lasting benefit (months to years) or may only provide a transient respite from ongoing damage. Clinical investigators need to continue studies in search of better parameters predictive of airway inflammation. Preliminary studies have indicated that clinical parameters of CF do not faithfully reflect the destructive inflammatory changes in pulmonary secretions and, hence, when used alone may not be sufficient to judge the adequacy of therapy.21Hornick DB Fick RB. Immunochemical studies of cystic fibrosis lung fluid: An important clinical tool to assess airways inflammation.Am Rev Respir Dis. 1987; 135: 465Google Scholar Assessment of the bronchoalveolar lavage fluid cellularity, elastolytic activity, IgG concent" @default.
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• W2078957057 magId "2078957057" @default.

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