Treatment and prevention of vitamin D insufficiency in cystic fibrosis patients: comparative efficacy of ergocalciferol, cholecalciferol, and UV light.

BACKGROUND: The optimal treatment for correcting or preventing vitamin D insufficiency in cystic fibrosis (CF) patients has not been established. OBJECTIVE: The aim of the study was to assess the relative efficacy of three modes of vitamin D therapy: cholecalciferol (D3), ergocalciferol (D2), and UV light in raising or maintaining 25(OH)D levels above 30 ng/ml. DESIGN: Thirty adult CF subjects with vitamin D insufficiency were randomized into one of three treatment arms: D3, D2, or UV light. Subjects randomized to D3 or D2 ingested 50,000 IU of vitamin D weekly, and those randomized to UV exposed their skin to UV light from a lamp five times a week. Serum was collected for 25(OH)D and PTH at baseline and at 12 wk. RESULTS: Treatment with D3 and D2 raised 25(OH)D levels significantly, from a mean of 21.2 +/- 10.18 to 47.1 +/- 20.5 ng/ml (P < 0.001) and 24.4 +/- 10.3 to 32.7+/- 9.7 ng/ml (P = 0.01), with 100% and 60% reaching 25(OH)D levels above 30 ng/ml, respectively. Treatment with UV did not raise 25(OH)D levels significantly; however, only 55% of subjects were adherent with UV therapy. CONCLUSION: This study demonstrates that CF subjects are able to achieve or maintain optimal vitamin D status (>30 ng/ml) with two oral regimens of either D3 or D2 treatment, the former being more efficacious. A confounding variable for this observation is the fact that the D3 and D2 capsules contained different carriers, powder-based vs. oil-based, respectively. UV therapy did not alter vitamin D status, possibly due to poor adherence to UV therapy.

Genetic and non-genetic determinants of outcomes in cystic fibrosis.

The treatment of cystic fibrosis has improved significantly over the past three decades. Median survival has improved by decades and is now estimated to be 37 years. Many factors contribute to improvements in disease severity and outcome. This paper reviews the current evidence of three groups of important factors: genetic, environmental and healthcare related.

Vitamin D and bone health in adults with cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF) patients have chronic pancreatic insufficiency leading to malabsorption of fat-soluble vitamins, including vitamin D which can contribute to poor skeletal health and respiratory function. OBJECTIVE: This study evaluated the prevalence of vitamin D insufficiency and its impact on bone and respiratory health in adults with CF. DESIGN AND MEASUREMENTS: This was a retrospective study in which data were collected from medical records over a 2-year period. Data included patient demographics, lung function, biochemical data, bone mineral densities, X-rays and ascertainment of use of vitamin supplements. Data were collected from medical records at a single accredited CF Center. Serum 25-hydroxyvitamin D [25(OH)D] levels and bone mineral density studies were also collected. PATIENTS: A total of 185 adults with CF were identified with a mean age of 29 +/- 9 years. RESULTS: The prevalence of vitamin D insufficiency [25(OH)D < 75 nmol/l] was 76%. Mean serum 25(OH)D concentrations were 58.8 +/- 30 nmol/l. Use of specific vitamin D supplementation was protective against vitamin D insufficiency whereas use of multivitamins was not. There was a small, but significant, positive association between serum 25(OH)D and FEV(1) per cent predicted after controlling for age, gender, BMI and race (R(2) = 0.30, P < 0.001). A high prevalence (27%) of vertebral fractures was detected on lateral chest X-ray. CONCLUSIONS: The prevalence of vitamin D insufficiency and poor skeletal health is high in the US CF population. Vitamin D status appears to be positively associated with lung function. Prospective studies to examine the impact of correction of vitamin D insufficiency on skeletal and lung health in adult CF are warranted.

Treatment of vitamin D deficiency with UV light in patients with malabsorption syndromes: a case series.

BACKGROUND: Cystic fibrosis (CF) and short bowel syndrome (SBS) patients are unable to absorb vitamin D from the diet and thus are frequently found to be severely vitamin D deficient. We evaluated whether a commercial portable ultraviolet (UV) indoor tanning lamp that has a spectral output that mimics natural sunlight could raise circulating 25-hydroxyvitamin D [25(OH)D] levels in subjects with CF and SBS. METHODS: In initial pilot studies, two SBS subjects came to the outpatient clinic twice weekly for 8 weeks for UV light sessions of 6 min each. In a follow-up study, five CF subjects exposed their lower backs in a seated position to the sunlamp at a distance of 14 cm for 5-10 min depending on the skin type five times a week for 8 weeks. Blood samples for 25(OH)D and parathyroid hormone (PTH) measurements were performed at baseline and at the end of the study. RESULTS: In our study, with two SBS subjects, the indoor lamp increased or maintained circulating 25(OH)D levels during the winter months. We increased the UV lamp frequency and found an improved response in the CF patients. Serum 25(OH)D levels in CF subjects at baseline were 21 +/- 3 ng/ml, which increased to 27 +/- 4 ng/ml at the end of 8 weeks (P=0.05). PTH concentration remained largely unchanged in both population groups. CONCLUSION: A UV lamp that emits ultraviolet radiation similar to sunlight and thus produces vitamin D(3) in the skin is an excellent alternative for CF, and SBS patients who suffer from vitamin D deficiency due to fat malabsorption, especially during the winter months when natural sunlight is unable to produce vitamin D3 in the skin. This UV lamp is widely available for commercial home use and could potentially be prescribed to patients with CF or SBS.

Well-differentiated fetal adenocarcinoma of the lung: cytomorphologic features on fine-needle aspiration with emphasis on use of beta-catenin as a useful diagnostic marker.

Well-differentiated fetal adenocarcinoma (WDFA), also known as low grade adenocarcinoma of the fetal lung type, is a rare pulmonary neoplasm now considered to be a variant of lung adenocarcinoma rather than a type of pulmonary blastoma. Upregulation of the Wnt signaling pathway with subsequent aberrant nuclear/cytoplasmic beta-catenin expression has been recently described in these tumors providing a possible pathogenetic role for this gene in WDFA. We describe the cytomorphologic findings of a case of WDFA in a 36-yr-old female patient and emphasize the diagnostic utility of aberrant nuclear/cytoplasmic expression of beta-catenin as an adjunct to the correct preoperative recognition of this tumor on aspiration cytology.

Relationship of physician estimate of underlying asthma severity to asthma outcomes.

BACKGROUND: Implementation of national guidelines for the treatment of asthma requires physician estimates of patients' underlying asthma severity. Asthma severity is commonly assigned based on respiratory symptoms. OBJECTIVE: To evaluate the relationship of guideline-based physician assessments to asthma control. METHODS: Data were collected by survey as part of a cohort study of adults with asthma. Physicians estimated the underlying severity of their patients' asthma as mild, moderate, or severe. We evaluated the relationship of these estimates to (1) general health status, asthma symptoms, and patient-reported emergency department (ED) visits and hospitalizations in the previous year and (2) outcomes in the following year. RESULTS: A total of 3,468 adults with asthma had physicians who completed assessments of their severity. Physician evaluation of severity was significantly associated with the patient's recent general health status, asthma symptom control, ED visits, and hospitalizations (P < .001 for all). Future outcomes, including hospitalizations and ED visits for asthma, increased with increasing severity rating (hospitalizations: 5% [mild] vs 11% [moderate] vs 19% [severe]; ED visits: 15% [mild] vs 22% [moderate] vs 32% [severe]; P < .001 for all). CONCLUSION: This study provides evidence of the validity of physician assessments of patients' underlying asthma severity using the strategy recommended by national guidelines.

Bronchoscope reprocessing and infection prevention and control: bronchoscopy-specific guidelines are needed.

BACKGROUND: It has been recommended that bronchoscopists familiarize themselves with national recommendations for bronchoscope reprocessing practices, but the extent of guideline awareness is unclear. METHODS: We distributed a survey to practicing bronchoscopists at two meetings. Questions addressed infection control issues related to bronchoscopy and specific reprocessing recommendations. RESULTS: A total of 46 surveys were completed by medical directors of bronchoscopy suites (26%) and attending bronchoscopists (74%) who had graduated from medical school a median of 22 years ago and performed a median of 19 procedures per month. Sixty-five percent of respondents, including 55% of directors, were not familiar with national reprocessing recommendations, and 39% did not know the approach to reprocessing at their own institution. Respondents who did > 20 procedures per month trended toward being more likely to be aware of guidelines (54% vs 26%, p = 0.09) and were less likely to answer "do not know" to more than one question about specific reprocessing details (25% vs 70%, p = 0.003). Seventy-eight percent of respondents did not know local practices for at least one of the reprocessing details. Forty-six percent of respondents stated their institutions followed recommendations that records be kept of the specific bronchoscope used in each case. CONCLUSIONS: Many experienced bronchoscopists are unfamiliar with national guidelines and local practices related to bronchoscope reprocessing. Publication of bronchoscope-specific, comprehensive reprocessing guidelines in the pulmonary literature may help increase familiarity with this crucial process.

An outbreak of Pseudomonas aeruginosa infections associated with flexible bronchoscopes.

BACKGROUND: Endoscopes, including bronchoscopes, are the medical devices most frequently associated with outbreaks of nosocomial infections. We investigated an outbreak of Pseudomonas aeruginosa infections after bronchoscopic procedures. METHODS: Microbiologic results were reviewed to determine the rates of recovery of P. aeruginosa from bronchoalveolar-lavage specimens. Environmental samples from endoscopes and the endoscopy suite were cultured. Medical records were reviewed to identify infections in the 14 days after a bronchoscopy. RESULTS: The rate of recovery of P. aeruginosa from bronchoalveolar-lavage specimens obtained with use of endoscopy-suite bronchoscopes increased from 10.4 percent at base line to 31.0 percent during the outbreak (relative risk, 2.97; 95 percent confidence interval, 2.28 to 3.90). Cultures of samples from three bronchoscopes grew P. aeruginosa, whereas cultures of samples from the environment, instrument-cleaning machines, and gastrointestinal endoscopes did not. The three bronchoscopes had been part of a nationwide recall. A total of 414 patients underwent bronchoscopy during the outbreak, and there were 48 respiratory tract and bloodstream infections among 39 of these patients (9.4 percent). In 32 infections (66.7 percent), P. aeruginosa was confirmed as a potentially causative organism. Exposure to a potentially contaminated bronchoscope may have had a role in the death of three patients. The rate of recovery of P. aeruginosa returned to base line after the instruments were removed from service. CONCLUSIONS: This large outbreak of P. aeruginosa infections related to bronchoscopy was apparently caused by a loose biopsy-port cap in the bronchoscopes. Instrument safety and surveillance methods for bronchoscopy must be improved, and better recall procedures are needed for medical devices.

Lower physician estimate of underlying asthma severity leads to undertreatment.

BACKGROUND: Asthma undertreatment has been linked to poor outcomes. National guidelines recommend that physicians classify asthma severity based on pretreatment symptoms and titrate care as the disease changes in the individual patient. This study evaluated the extent to which the physician estimate of underlying severity affects a patient's asthma care. METHODS: Data used were collected from a cohort of adults with asthma enrolled in managed care. Eligible patients were adults enrolled in managed care with medical encounters coded for asthma. Physicians were eligible if they were main asthma providers. The patient survey covered demographics, symptoms, asthma treatment, and self-management knowledge. Physicians were asked to assess the underlying severity of their patients' asthma. RESULTS: There were 4005 patients with asthma with physician estimates of underlying severity. Of the patients, 70.1% were female (mean age, 44.8 years) and 83.5% were white. Most patients' current asthma symptoms were moderate (39.4%) and severe (50.1%). Most physician estimates of underlying severity were mild (44.6%) and moderate (44.5%). Among those patients reporting moderate symptoms, daily inhaled corticosteroid use was reported in 35.2% when physician estimates were mild, 53.0% when moderate, and 68.1% when severe (P =.001). Rates of peak flowmeter ownership, allergy testing, and self-management knowledge tracked similarly with physician estimates of underlying severity. CONCLUSIONS: Physician estimates of underlying asthma severity appear to determine asthma care. For patients with inadequate symptom control, lower physician estimates of underlying severity were associated with care that is less consistent with national guidelines. To improve the quality of asthma care, physicians need to update treatment based on their patients' current symptoms and adapt care accordingly.

Gaps in asthma care of the oldest adults.

OBJECTIVES: To assess the adequacy of asthma care reported by a group of older adults who were subsequently hospitalized for their asthma. DESIGN: Prospective cohort study. SETTING: Fifteen managed care organizations in the United States. PARTICIPANTS: Adults with asthma, enrolled in managed care. MEASUREMENTS: Patient survey of demographics, asthma symptoms, health status, comorbid conditions, asthma treatment, asthma knowledge, and asthma self-management at baseline and 1 year later. RESULTS: Of 254 older adults, 38 (15.0%) reported being hospitalized for asthma at 1-year follow-up. Of these, 22.9% owned a peak flow meter (PFM). Of those with allergies, only about half (56.0%) had been told how to avoid allergens and had been referred for formal allergy testing. Adrenergic drug use was high in some patients. Nearly all (94.6%) used beta-agonist metered-dose inhalers (MDIs); 60.0% reported theophylline; 17.1% reported beta-agonist MDI overuse (>8 puffs per day); 10.5% reported beta-agonist MDI over-use and theophylline; and 13.2% reported both beta-agonist MDI over-use and oral beta-agonist use. Only 18.4% of respondents rated their overall asthma attack knowledge as excellent. Compared with nonhospitalized older adults, the hospitalized group reported care that was more consistent with guidelines, but also higher rates of potentially toxic combinations of adrenergic drugs. Compared with younger hospitalized adults, older hospitalized adults had clear deficiencies, including lower use of PFMs (55.3% vs 22.9%) and worse asthma self-management knowledge. CONCLUSIONS: There are many opportunities to improve both the pharmacologic and non-pharmacologic care of older adults with asthma. Overuse of and potentially toxic combinations of inhaled and oral sympathomimetics should probably be avoided. Older asthmatics may also benefit from increased specialty referral, PFM use, allergy testing, and asthma teaching.