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Noah Lechtzin

Publications and source records attributed to Noah Lechtzin.

16 recordsLinked to original sources

Measures of dyspnea in patients with amyotrophic lateral sclerosis.

Respiratory symptoms are not well characterized in amyotrophic lateral sclerosis (ALS). The baseline dyspnea index (BDI) and transition dyspnea index (TDI) are indices designed to measure change in dyspnea over time. They are easy to administer and do not inquire about specific physical tasks. The latter makes these scales particularly well suited for use in ALS. This study evaluated the ability of the BDI and TDI to measure dyspnea in 46 subjects with ALS. The BDI/TDI had excellent reproducibility. The TDI detected worsening dyspnea by 4 weeks and declined significantly more over the subsequent 8 weeks. The TDI was significantly associated with changes in forced vital capacity and appeared more sensitive to changes in dyspnea than the ALS functional rating scale-respiratory subscale (ALS-FRS R) and a visual analog scale of breathlessness. The BDI and TDI thus appear to be useful measures in ALS and may have both clinical and research applications.

Aged↗

Current treatment of adult Duchenne muscular dystrophy.

Patients with Duchenne muscular dystrophy (DMD) are living longer into adulthood due to a variety of improvements in health care practices. This growing patient population presents new therapeutic challenges. In this article, we review the literature on current treatment of adult DMD as well as our own experience as a multidisciplinary team actively caring for 23 men ages 19-38 years of age. Approximately one quarter of our adult DMD patients have remained on moderate dose corticosteroids. Daily stretching exercises are recommended, particularly of the distal upper extremities. Cardiomyopathy is anticipated, detected, and treated early with afterload reduction. Oxygen saturation monitoring, noninvasive positive pressure ventilation and cough assist devices are routinely used. Other medical issues such as osteoporosis, gastrointestinal and urinary symptoms are addressed. Current and future therapies directed at prolonging the lifespan of those with DMD will result in further increases in this adult population with special needs and concerns. These needs are best addressed in a multidisciplinary clinic.

Adult↗

Persistent cytomegalovirus-specific memory responses in the lung allograft and blood following primary infection in lung transplant recipients.

Primary CMV infection in lung transplant recipients (LTRs) is associated with increased mortality. We studied 22 donor CMV-positive, recipient-negative (D(+)R(-)) LTRs for the development of posttransplant CMV-specific immunity. We found that 13 of 22 D(+)R(-) LTRs (59.1%) seroconverted (CMV IgG Ab(+)). Using pooled peptides of the immunodominant CMV Ags pp65 and IE1, we detected CMV-specific CD8(+)IFN-gamma(+) T cells in the PBMC of 90% of seroconverted individuals following primary infection by intracellular cytokine staining. In contrast, few seroconverters had detectable CMV-specific CD4(+)IFN-gamma(+) T cells during viral latency. However, the majority of IgG(+) LTRs demonstrated CMV-specific CD4(+) and CD8(+) T cell proliferative responses from PBMC, with CD4(+)IFN-gamma(+) T cells detectable upon re-expansion. Examination of lung allograft mononuclear cells obtained by bronchoalveolar lavage revealed both CMV-specific CD4(+) and CD8(+)IFN-gamma(+) T cells, including patients from whom CD4(+)IFN-gamma(+) T cells were simultaneously undetectable in the PBMC, suggesting differential effector memory populations between these compartments. Moreover, both responses in the PBMC and lung allograft were found to persist, despite substantial immunosuppression, long after primary infection. Clinical correlation in this cohort demonstrated that the acquisition of CMV immunity was associated with freedom from CMV disease (p < or = 0.009) and preservation of allograft function (p < or = 0.02) compared with those who failed to develop CMV immunity. Together, our data reveal immunologic heterogeneity in D(+)R(-) LTRs, with the development and persistence of primary CMV responses that may provide clinical benefit.

Adolescent↗

Pulmonary predictors of survival in amyotrophic lateral sclerosis: use in clinical trial design.

The rapidity of progression of amyotrophic lateral sclerosis (ALS) to death or respiratory failure impacts patients, clinicians, and clinical investigators. This study compared the abilities of various pulmonary function tests to predict tracheostomy-free survival. We evaluated 95 ALS patients by determining upright and supine forced vital capacity (FVC), maximal inspiratory (MIP) and expiratory (MEP) pressures, arterial partial pressure of carbon dioxide (PaCO2), and transdiaphragmatic sniff pressures (Pdi-sniff). Tracheostomy-free survival time was measured from the date of spirometry. Supine FVC, upright FVC, MIP, MEP, and Pdi-sniff were significantly associated with tracheostomy-free survival after controlling for nonpulmonary factors, whereas PaCO2 was not. A normal supine FVC, MIP, or MEP was highly predictive for one-year survival. These tests are well suited to predict survival for trial enrollment and patient counseling. Supine FVC's simplicity of use and availability to ALS investigators makes it a particularly attractive predictor of one-year survival in ALS.

Amyotrophic Lateral Sclerosis↗

Supramaximal inflation improves lung compliance in subjects with amyotrophic lateral sclerosis.

RATIONALE: Lung compliance has been found to be low in patients with chronic diaphragmatic weakness or paralysis but has not been well-studied in patients with amyotrophic lateral sclerosis (ALS). Noninvasive positive-pressure ventilation (NPPV) prolongs survival in ALS patients but may also have additional beneficial effects. OBJECTIVES: This study evaluated static expiratory lung compliance (CL) in subjects with ALS and determined the effect of lung inflation with supramaximal inflation on CL. DESIGN: This was a prospective trial comparing CL before and after supramaximal lung inflation via mouthpiece-delivered positive pressure. SETTING: A single university medical center with an multidisciplinary ALS center. PARTICIPANTS: Fourteen subjects with ALS were compared to 4 healthy volunteers. INTERVENTIONS: Subjects underwent a battery of pulmonary function tests including for CL. Then they used positive pressure administered via a mouthpiece set to 10 cm H2O above their maximal static recoil pressure for 5 min. The CL measurement was then repeated. RESULTS: The mean (+/- SD) baseline CL was reduced (164.1 +/- 82.1 mL/cm H2O) in subjects with ALS and was significantly lower than that in healthy volunteers (237.5 mL/cm H2O; p = 0.04). CL increased significantly in subjects with evidence of diaphragm weakness (change in CL, 11.3 +/- 16.7 mL/cm H2O; p = 0.03). Healthy volunteers did not have an increase in CL. CONCLUSIONS: Patients with ALS and diaphragmatic weakness have reduced CL, and brief supramaximal inflation increases CL. These findings suggest that atelectasis or increased alveolar surface forces are present in ALS patients and that these patients will have increased work of breathing. Some of the beneficial effects demonstrated with NPPV therapy may be through its effects on CL and the work of breathing.

Adult↗

Respiratory effects of amyotrophic lateral sclerosis: problems and solutions.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease. Most patients die from respiratory complications. Fortunately, there are a growing number of treatment options that can improve both survival and quality of life for patients with ALS. This review discusses the respiratory evaluation and treatment of patients with ALS, about which a great deal is known. It also includes material on related problems, such as speech and swallowing difficulties and end-of-life care.

Amyotrophic Lateral Sclerosis↗

Outcomes of adults with cystic fibrosis infected with antibiotic-resistant Pseudomonas aeruginosa.

BACKGROUND: Although Pseudomonas aeruginosa is the most common bacterial infection in adults with cystic fibrosis and frequently develops resistance to multiple classes of antibiotics, it has not been determined whether patients with multiple antibiotic-resistant Pseudomonas aeruginosa have worse clinical outcomes than patients with more susceptible strains. OBJECTIVES: This study assessed the impact of multiply-resistant P. aeruginosa on lung function, hospitalizations, antibiotic use, lung transplantation and survival in adults with cystic fibrosis. METHODS: In a cohort study at a university-based adult cystic fibrosis program, 75 consecutive adult cystic fibrosis patients who had P. aeruginosa isolated from sputum cultures were studied over a 4-year period. Outcomes included decline in FEV1, clinic visits, hospitalizations, courses and days of intravenous antibiotics, and lung transplantation. Multiple linear and Poisson regression for repeated measures were used to assess the outcomes. RESULTS: In comparison to patients with susceptible strains, patients with resistant P. aeruginosa had more severe baseline lung disease, more rapid decline in FEV1 (160 ml/year, p = 0.003) and were significantly more likely to undergo lung transplantation (17.6 vs. 0%, p = 0.005). CONCLUSIONS: Infection with multiple-antibiotic-resistant P. aeruginosa is associated with accelerated progression of cystic fibrosis, and has important implications for infection control strategies, antibiotic use and lung transplantation.

Adult↗

Failure of high-dose ergocalciferol to correct vitamin D deficiency in adults with cystic fibrosis.

RATIONALE: Treatment guidelines for vitamin D monitoring and supplementation in cystic fibrosis (CF) have recently been developed and published by a consensus committee, but have not been prospectively tested. OBJECTIVES: To use these guidelines to determine the percentage of adults with CF requiring vitamin D repletion therapy and to evaluate the effectiveness of the currently recommended high-dose oral ergocalciferol repletion protocol. METHODS: Prospective study of clinical outcomes after therapy with the recommended vitamin D repletion algorithm. RESULTS: Of 134 adults with CF, 109 (81.3%) were found to have 25-hydroxyvitamin D (25-OHD) levels below the recommended 30 ng/ml. Sixty-six of these adults completed the recommended course of 400,000 IU of oral ergocalciferol over 2 months, and only five (8%) responded with correction of their serum 25-OHD to the goal of 30 ng/ml or greater (mean change, +0.3 ng/ml; from 18.8 to 19.1 ng/ml). In the 33 adults with CF who also completed the recommended second course of 800,000 IU of ergocalciferol over 2 months, none demonstrated correction of their deficiency (mean change, -1.2 ng/ml). CONCLUSION: The results of this study demonstrate that a majority of adults with CF have serum 25-OHD levels below 30 ng/ml, and the currently recommended ergocalciferol repletion regimen often does not fully correct vitamin D deficiency and may need to be revised to include even higher dosing of ergocalciferol. Further work is needed to establish the ideal 25-OHD level for maximizing calcium absorption and bone health in CF.

Adult↗

Use of noninvasive ventilation in patients with amyotrophic lateral sclerosis.

INTRODUCTION: Noninvasive positive pressure ventilation (NIPPV) is associated with improved survival in amyotrophic lateral sclerosis (ALS) and has been widely recommended. The extent of NIPPV use in ALS patients and the factors associated with its use have not been studied. METHODS: A cross-sectional study using the ALS Patient Care Database. Analyses were performed to assess the association of patient and care characteristics with use of ventilatory support. RESULTS: 1458 patients were studied. 15.6% used NIPPV and 2.1% used invasive mechanical ventilation. Patients who used NIPPV were significantly more likely to be male and have higher income than those who did not. They were also more likely to have a gastrostomy tube, lower vital capacity, more severe disease, bulbar involvement and poorer general health status as measured by the SF-12 and Sickness Impact Profile. Multivariate analysis revealed that lower FVC, higher income and use of gastrostomy tube were independently associated with use of NIPPV. CONCLUSIONS: NIPPV is used more than seven times as frequently as invasive ventilation in ALS patients. Patients who use NIPPV have more severe disease than those who do not use any respiratory intervention. Patients with lower income are less likely to use NIPPV, which raises concerns about disparities in the care of patients with ALS.

Adolescent↗

In-hospital mortality following acute exacerbations of chronic obstructive pulmonary disease.

BACKGROUND: Acute exacerbations of chronic obstructive pulmonary disease (COPD) are a frequent cause of hospitalization in the United States. Previous studies of selected populations of patients with COPD have estimated in-hospital mortality to range from 4% to 30%. Our objective was to obtain a generalizable estimate of in-hospital mortality from acute exacerbation of COPD in the United States and to identify predictors of in-hospital mortality using administrative data. METHODS: We performed a cross-sectional study utilizing the 1996 Nationwide Inpatient Sample, a data set of all hospitalizations from a 20% sample of nonfederal US hospitals. The study population included 71 130 patients aged 40 years or older with an acute exacerbation of COPD at hospital discharge. The primary outcome assessed was in-hospital mortality. RESULTS: In-hospital mortality for patients with an acute exacerbation of COPD was 2.5%. Multivariable analyses identified older age, male sex, higher income, nonroutine admission sources, and more comorbid conditions as independent risk factors for in-hospital mortality. CONCLUSIONS: Mortality during hospitalization in this nationwide sample of patients with acute exacerbations of COPD was lower than that of previous studies of select populations. This estimate should provide optimism to both clinicians and patients regarding prognoses from COPD exacerbations requiring hospitalization. Our results indicate that the use of administrative data can help to identify subsets of patients with acute exacerbations of COPD that are at higher risk of in-hospital mortality.

Acute Disease↗

The relationship between age and process of care and patient tolerance of bronchoscopy.

OBJECTIVES: To determine whether patient age is associated with differences in flexible bronchoscopy technique and tolerance. DESIGN: Prospective cohort study. SETTING: University hospital system. PARTICIPANTS: One thousand three hundred fifty-eight adults, including 219 (16.1%) aged 70 and older, undergoing bronchoscopy. MEASUREMENTS: Indications, sampling procedures, medication doses, patient reports of pain, willingness to return, and adverse events associated with bronchoscopy. RESULTS: Indications for bronchoscopy varied with age, with solitary pulmonary nodule (P <.001), mass (P <.001), or lymphadenopathy (P <.001) being more common in older patients. Invasive sampling methods were used more often with increasing age, but variation in disease processes between age groups accounted for the difference in sampling method performed. Mean doses of midazolam and fentanyl given for sedation decreased with increasing age (P <.001). There was no significant difference between older and younger patients in reported very good to excellent pain control (50% of patients >/=70 vs 64% of patients <40; P =.56) or in willingness to return for repeat bronchoscopy (98% vs 92%, respectively; P =.324). Overall risk for an adverse event increased with increasing patient age (P <.01), but adverse events were uncommon and generally not severe. Hypotension and pneumothorax were rare but occurred more often in older persons (1.9% and 3.4% in patients >/=70 vs 0.5% and 0.7% in patients <40, respectively). CONCLUSION: Despite more-invasive sampling methods and less sedation during bronchoscopy, elderly patients tolerate bronchoscopy as well as younger patients. There is increased risk for adverse events with increasing age, but the absolute frequency is low, suggesting that chronological age should not be a contraindication for bronchoscopy in older persons.

Adolescent↗

Distraction therapy with nature sights and sounds reduces pain during flexible bronchoscopy: a complementary approach to routine analgesia.

STUDY OBJECTIVES: To determine whether distraction therapy with nature sights and sounds during flexible bronchoscopy (FB) reduces pain and anxiety. DESIGN: Randomized controlled trial. SETTING: Teaching hospital in Baltimore, MD. PATIENTS: Consecutive adult patients (n = 80) undergoing FB with conscious sedation. INTERVENTION: Nature scene murals were placed at the bedside, and patients were provided a tape of nature sounds to listen to before, during, and after the procedure. Patients assigned to the control group were not offered either the nature scene or the sounds. MEASUREMENTS AND RESULTS: The primary outcomes were patient ratings of pain control (a 5-point scale ranging from poor to excellent) and anxiety. In a multivariate ordinal logistic regression model, the odds of better pain control were greater in the intervention patients than in the control patients (odds ratio [OR], 4.76; 95% confidence interval [CI], 1.35 to 16.7), after adjustment for age, gender, race, education, health status, and dose of narcotic medication. Older patients and patients with better health status reported significantly less pain. There was no difference in patient-reported anxiety between the two groups (OR, 0.87; 95% CI, 0.39 to 1.96). CONCLUSIONS: Distraction therapy with nature sights and sounds significantly reduces pain in patients undergoing FB. Although the precise mechanism of this beneficial effect requires further investigation, clinicians should consider this nonintrusive strategy in addition to standard analgesic medications in patients undergoing painful, invasive procedures.

Adult↗

Patient satisfaction with bronchoscopy.

We conducted a cohort study to characterize patient satisfaction with flexible bronchoscopy (FB) and to determine patient characteristics, care factors, and patient evaluations of bronchoscopy that are associated with a patient's willingness to return for repeat FB. Physicians and patients completed surveys between February 1997 and June 1998. Data from 481 patients were analyzed. Over 80% of the patients rated physicians as very good or excellent, but they were least satisfied with the information that they were provided about FB, waiting time before and after FB, and the FB environment. Seventy-one percent of the patients would definitely return, and 22% would probably return for a repeat FB. Better health status (odds ratio [OR] 1.4; 95% confidence interval [CI], 1.1-1.7), not being bothered by scope insertion (OR 2.0; 95% CI, 1.2-3.3), better rating of information quality (OR 1.2; 95% CI, 1.0-1.3), and better rating of physician quality (OR 1.1; 95% CI, 1.0-1.2) were associated with patients reporting that they would definitely return for a repeat FB. Although patient satisfaction with providers is high, there are specific patient and process of care factors that should be improved. Sicker patients may be at a risk of dissatisfaction with FB. Process of care measures that could improve satisfaction with FB include providing better information to patients and optimizing the experience of bronchoscope insertion.

Adolescent↗

Amyotrophic lateral sclerosis: evaluation and treatment of respiratory impairment.

Patients with amyotrophic lateral sclerosis (ALS) invariably develop respiratory muscle weakness and most die from pulmonary complications. There are numerous tests available to evaluate respiratory status in ALS and it is important to understand their various advantages and limitations. Forced vital capacity (FVC) is commonly used but can remain normal despite substantial inspiratory muscle weakness. Maximal pressures measured at the mouth are useful for excluding weakness if they are normal but are difficult to interpret if abnormal. Invasive testing, such as measurement of transdiaphragmatic pressure, provides an accurate measure of inspiratory strength but is not readily available and is not practical for serial measures. There are supportive respiratory techniques that have been shown to benefit patients with ALS. Clinicians should be familiar with these interventions, including mechanically assisted coughing, non-invasive ventilation and tracheostomy with mechanical ventilation. Observational studies have demonstrated improved survival and quality of life with noninvasive ventilation. Tracheostomy with long-term mechanical ventilation is not frequently used but can be an important component of care for ALS. This review describes an approach to respiratory evaluation and care of patients with ALS.

Amyotrophic Lateral Sclerosis↗

Spirometry in the supine position improves the detection of diaphragmatic weakness in patients with amyotrophic lateral sclerosis.

STUDY OBJECTIVES: To determine which respiratory function tests best predicted diaphragmatic strength in patients with amyotrophic lateral sclerosis. PATIENTS AND METHODS: Patients referred for pulmonary evaluation were included (n = 25) if they underwent measurement of transdiaphragmatic pressure (Pdi) and one or more of the following on the same day: upright FVC, supine FVC, upright FEV(1), supine FEV(1), maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and PaCO(2). Abdominal paradox and use of accessory muscles were also assessed. Bivariate analyses were performed using simple linear regression. Sensitivity and specificity of the potential predictors to detect an abnormal Pdi (< 70 cm H(2)O) were calculated. SETTING: Pulmonary function laboratory of an academic medical center. RESULTS: Upright FVC, FEV(1), and MEP were all significantly correlated with Pdi, while MIP and PaCO(2) were not. Supine FVC was the most highly correlated predictor of Pdi (R(2) = 0.76). A cutoff of supine FVC that was < 75% predicted was 100% sensitive and specific for predicting an abnormally low Pdi. Accessory muscle use and abdominal paradox were both significantly associated with Pdi, and the presence of accessory muscle use had a sensitivity of 84% and a specificity of 100% for detecting a low Pdi. CONCLUSIONS: Our findings suggest that supine FVC is an excellent and simple test of diaphragmatic weakness.

Adult↗