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Biomedical subjects

Patrick A Flume

Publications and source records attributed to Patrick A Flume.

14 recordsLinked to original sources

Superior vena cava syndrome related to indwelling intravenous catheters in patients with cystic fibrosis.

Patients with cystic fibrosis (CF) often need long-term implanted vascular-access devices for intravenous antibiotics for chronic lower respiratory tract infections. These devices are not without complications, including infection, occlusion, and vascular thrombosis. Such thrombosis can result in superior vena cava (SVC) syndrome due to the position of the catheter proximal to the right atrium. SVC syndrome in CF patients, however, is rarely reported in the literature, suggesting that its incidence is uncommon. We describe three patients with SVC syndrome as a consequence of implanted vascular-access devices.

Adult↗

An unusual presentation of cystic fibrosis in an adult.

The diagnosis of cystic fibrosis (CF) generally is made within the first few years of life, although some cases will not be diagnosed until adulthood. For most patients the diagnosis is suggested by typical CF-related symptoms such as chronic respiratory infection or maldigestion. The authors describe an adult patient with newly diagnosed CF whose presenting abnormalities consisted of hypokalemia and metabolic alkalosis. These are known complications of CF but are not common presenting features that lead to the diagnosis of CF. The authors discuss their patient's presentation and review his metabolic manifestations of CF.

Acid-Base Equilibrium↗

Pneumothorax in cystic fibrosis.

BACKGROUND: Spontaneous pneumothorax is a complication that is commonly reported in patients with cystic fibrosis (CF). An understanding of the pathophysiology of this complication and its consequences is important for the management of patients with CF. OBJECTIVE: To identify risk factors associated with pneumothorax and to determine the prognosis of CF patients following an episode of pneumothorax. DESIGN: A retrospective observational cohort study of the National Cystic Fibrosis Patient Registry between the years 1990 and 1999. PATIENTS: The registry contained data on 28,858 patients with CF who had been followed up over those 10 years at CF centers across the United States. RESULTS: Pneumothorax occurred with an average annual incidence of 0.64% and in 3.4% of patients overall. There was no increased occurrence by sex, but CF was more prevalent in older patients (mean [+/- SD] age, 21.9 +/- 9.1 years) with more severe pulmonary impairment (nearly 75% of patients with FEV1 of < 40% predicted). The principal risks associated with an increased occurrence of pneumothorax included the presence of Pseudomonas aeruginosa (odds ratio [OR], 2.3), Burkholderia cepacia (OR, 1.8), or Aspergillus (OR, 1.3) in sputum cultures, FEV1 < 30% predicted (OR, 1.5), enteral feeding (OR, 1.7), Medicaid insurance (OR, 1.1), pancreatic insufficiency (OR, 1.4), allergic bronchopulmonary aspergillosis (OR, 1.5), and massive hemoptysis (OR, 1.4). There is an increased morbidity (eg, increased number of hospitalizations and number of days spent in the hospital) and an increased 2-year mortality rate following pneumothorax. CONCLUSION: Pneumothorax is a serious complication in CF patients, occurring more commonly in older patients with more advanced lung disease. Nearly 1 in 167 patients will experience this complication each year. There is an attributable mortality to the complication and considerable morbidity, resulting in increased health-care utilization and a measurable decline in lung function.

Adolescent↗

Massive hemoptysis in cystic fibrosis.

BACKGROUND: Massive hemoptysis is a complication commonly reported in patients with cystic fibrosis (CF). An understanding of the pathophysiology of this complication and its consequences is important for the management of patients with CF. OBJECTIVES: To identify risk factors associated with massive hemoptysis, and to determine the prognosis of patients following an episode of massive hemoptysis. DESIGN: A retrospective, observational cohort study of the National CF Patient Registry between the years 1990 to 1999. PATIENTS: The Registry contained data on 28,858 patients with CF observed over 10 years at CF centers across the United States. RESULTS: Massive hemoptysis occurred with an average annual incidence of 0.87% and in 4.1% of patients overall. There was no increased occurrence by sex, but it was more prevalent in older patients (mean age, 24.2 +/- 8.7 years [+/- SD]) with more severe pulmonary impairment (nearly 60% of patients who had an episode of massive hemoptysis had FEV1 < 40% predicted). The principal risks associated with an increased occurrence of massive hemoptysis included the presence of Staphylococcus aureus in sputum cultures (odds ratio [OR], 1.3) and diabetes (OR, 1.1). There was an increased morbidity (eg, increased hospitalizations and hospital days) and an increased 2-year mortality following massive hemoptysis. CONCLUSION: Massive hemoptysis is a serious complication in CF patients, occurring more commonly in older patients with more advanced lung disease. Nearly 1 in 100 patients will have this complication each year. There is an attributable mortality to the complication and considerable morbidity, resulting in increased health-care utilization and a measurable decline in lung function.

Adolescent↗

Transition programs in cystic fibrosis centers: perceptions of team members.

We previously surveyed cystic fibrosis (CF) center directors and adult patients with CF to assess their perceptions regarding transition from a pediatric to an adult setting. An important finding in those studies was a lack of standard programs for transfer of young adults with CF from pediatric to adult care settings. Patients with CF typically receive care from clinics utilizing a multidisciplinary approach, suggesting that every member of the CF team can impact the transition process. Our purpose in this study was to gain an appreciation for various team members' perspectives on transition. An Internet survey was offered to all CF centers across the country to be completed by team members, excluding physicians. We received 291 completed surveys, nearly half completed by nurses, but our respondents included social workers, nutritionists, respiratory therapists, and a few team members with other training. Nearly half of the respondents work for both pediatric and adult teams. The majority of respondents (71.8%) reported that their adult patients receive care from an internist in a separate adult program, but nearly 20% reported that a pediatrician follows their adult patients. A minority thought that age (37.4% of respondents), marriage (16.2%), and pregnancy (27.1%) were criteria for transfer, though most (86.2%) suggested that patients should be transferred by age 21 years. Criteria precluding transfer included patient/family resistance (45%), disease severity (34%), and developmental delay (31.3%). It was uncommon (11.4%) for an introduction to the concept of transition at the time of diagnosis. Over one-half of patients did not meet the adult team until time of transfer. Team members' perceptions of patients' concerns were similar to what we had previously measured in physicians, again far greater than what we have measured in patients themselves. In many ways, what we have measured here in team members reflects what we have reported by physicians, demonstrating slow development of standard transition programs and an overestimate of patients' concerns regarding transition. These differences may impede the successful transition of patients into an adult program. It is clear from this study that team members have an interest in and opinions on transition, and are likely play a vital role in the transition process. Standard programs of transition should be developed, and team members should be engaged in that process.

Adolescent↗

Linezolid pharmacokinetics in adult patients with cystic fibrosis.

The pharmacokinetics of many drugs are altered in patients with cystic fibrosis (CF), often necessitating different dosage requirements than those used in non-CF patients. The objective of this study was to determine the pharmacokinetics of linezolid, an antibiotic with good activity against gram-positive organisms such as methicillin-resistant Staphylococcus aureus, in patients with CF so that dosage requirements could be established. Twelve adult patients (6 male) ranging in age from 22 to 39 years were studied. A single 600-mg dose was administered intravenously over 0.5 h, and plasma samples were collected at 0 (predose), 0.5, 0.75, 1, 2, 4, 8, and 24 h. Linezolid concentrations were determined with a validated high-performance liquid chromatography assay. Pharmacokinetic parameters were estimated using standard noncompartmental methods. Blood chemistry and hematologic indices were determined before and after the study for safety purposes. All patients completed the study without encountering any adverse reactions. The pharmacokinetic parameters, while variable, with half-lives varying from 1.76 to 8.36 h, were similar to those previously described in other populations. Mean (+/- standard deviation) values for pharmacokinetic parameters of interest were as follows: elimination rate constant, 0.21 (0.11) h(-1); half-life, 4.41 (2.43); volume of distribution at steady state, 0.87 (0.19) liters/kg of body weight; and total body clearance, 0.12 (0.06) liters/h/kg. No patient would have achieved the pharmacodynamic target of an area under the concentration-time curve/MIC ratio of 83 h for pathogens for which the MIC was 4 micro g/ml. Patients with inadequate clinical responses to linezolid may require more frequent dosing.

Acetamides↗

Nebulizer use and maintenance by cystic fibrosis patients: a survey study.

INTRODUCTION: Patients with cystic fibrosis (CF) suffer from chronic infection of the airways, and typical CF therapies include aerosolized medications. There is recent evidence that home nebulizers become contaminated by bacteria, causing concern that nebulizers may be a source of bacterial infection of the lower airways. A recent consensus document on infection-control issues for the CF population included recommendations on cleaning and disinfecting nebulizers. METHODS: We surveyed 39 patients and their parents, as well as 54 respiratory therapists, regarding their routine nebulizer use and maintenance practices. RESULTS: All the patients used at least one nebulized medication, and they used a variety of nebulizers, obtained from a wide variety of sources. Thirty percent of the patients used nebulizers well beyond the manufacturer's recommended replacement date. Ninety percent of the patients rinsed their nebulizers following use, but only 15% performed any routine disinfection. The respiratory therapists' nebulizer cleaning methods were widely disparate, with only 70% performing some method of rinsing. The respiratory therapists' self-report of cleaning methods and their instructions to patients regarding frequency of cleaning were so diverse that no standard is evident. CONCLUSION: This study demonstrates that CF patients and their respiratory therapists should immediately address and improve their nebulizer cleaning methods and replacement practices. There should be more focus on teaching patients to regularly clean and replace nebulizers.

Adult↗

Airway clearance techniques.

The airways of the patient with cystic fibrosis (CF) are chronically infected. There is excess mucus within the airways and, because of the chronic infection, the mucus also contains bacteria and inflammatory cells. Mucociliary clearance is impaired in patients with CF, and patients become dependent upon cough and other techniques to clear their airways of the thick sputum. This review discusses the various techniques and medications that have been demonstrated to be effective at assisting the clearance of the excess secretions in the CF airways. No single method has been proven to be better than the others, and not all patients will benefit, or use, the same techniques. Therefore, the methods of airway clearance must be tailored for each patient to assure effective therapy.

Journal Article↗

Pneumothorax in cystic fibrosis.

Spontaneous pneumothorax is a common complication in patients with cystic fibrosis (CF). It is thought to occur more frequently in patients with more advanced disease. Recommendations on the management of pneumothorax in CF are based on retrospective analyses and reports from CF centers. The following is a review of what has been published regarding the incidence and management of pneumothorax in this population, with some comment on the pathogenesis of the complication.

Cystic Fibrosis↗

Transition programs in cystic fibrosis centers: perceptions of patients.

There is a growing population of adults with cystic fibrosis (CF) and a need for development of adult CF programs. Recommendations for transfer of patients from pediatric care to an adult program include a transition program. The purpose of this study was to survey adult CF patients to assess their own concerns regarding this issue. A survey was sent to all 1,288 members of the International Association of Cystic Fibrosis Adults (IACFA), with a response rate of 25.9% (n = 334). The majority of patients (81.2%) received care from a CF center; the major difference between those seen at a CF center and those seen at another facility was proximity to a CF center. Nearly one-fourth of patients seen at a CF center continued to receive care from a pediatrician even though a CF-trained internist was available; though these patients were younger, their mean age was still about 30 years. Patients seen by a pediatrician were more like to be students and to live with their parents. Those patients seen in an adult program described a variety of criteria for their transfer to the adult pro-gram, but there were no consistent findings to suggest a standard transition program. Indeed, many patients did not meet the adult team until the time of the transfer. Most importantly, the patients reported their level of concern about transfer as minimal, far less than what CF physicians had perceived. These differences may impede the successful transition of patients into an adult program.

Adult↗

Pulmonary complications after esophagectomy.

BACKGROUND: Pulmonary complications are common in patients who have undergone esophagectomy. There are no good predictive variables for these complications. In addition, the role that preoperative treatment with chemotherapy and radiation may play in postoperative complications remains unclear. METHODS: We performed a retrospective review of all patients who underwent esophagectomy by a single surgeon at our institution over a 6-year period. Data were analyzed for a correlation between patient risk factors and pulmonary complications, including mortality, prolonged mechanical ventilation, and hospital length of stay. RESULTS: Complete data were available on 61 patients. Nearly all patients had some pulmonary abnormality (eg, pleural effusion), although most of these were clinically insignificant. Pneumonia was the most common clinically important complication, and 19.7% of patients required prolonged ventilatory support. Significant risk factors identified included impaired pulmonary function, especially for patients with forced expiratory volume in 1 second (FEV1) less than 65% of predicted, preoperative chemoradiotherapy, and age. CONCLUSIONS: Impaired lung function is a significant risk factor for pulmonary complications after esophagectomy. Patients with FEV1 less than 65% of predicted appear to be at greatest risk. There also seems to be an associated risk of preoperative chemoradiotherapy for pulmonary complications after esophagectomy.

Adenocarcinoma↗

A "story" of a woman with cystic fibrosis.

Munchausen syndrome is a pattern of abnormal behavior in which patients repeatedly present with symptoms that have no organic basis and are intentionally simulated or produced in order to assume the sick role and gain admission to the hospital. A 25-year-old white woman presented to our adult cystic fibrosis (CF) program and gave a detailed history consistent with a diagnosis of CF, a complicated disease with multiorgan involvement that is difficult to feign. However, discrepancies in her history were noted and objective measures of disease were inconsistent, including evidence of a fabricated sweat chloride result. We describe the classic characteristics of Munchausen syndrome that our patient exhibited, and report this unusual presentation of Munchausen syndrome to be consistent with the recommendation of reporting such cases to other physicians. Such knowledge is likely to result in less harm to the patient through unnecessary testing and treatment.

Adult↗