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Biomedical subjects

James P Utz

Publications and source records attributed to James P Utz.

12 recordsLinked to original sources
PubMed2006

Organizing pneumonia in patients with hematologic malignancies: a steroid-responsive lesion.

BACKGROUND: Organizing pneumonia (OP) is a distinct histopathologic lesion that occurs in a variety of clinical contexts. There have been occasional reports of OP occurring in patients with hematologic malignancies. STUDY OBJECTIVES: To examine the association of OP with hematologic malignancies and to assess the clinical course of affected patients. DESIGN: A retrospective review of our institutional experience of unexplained OP developing in patients with hematologic malignancies. SETTING: Tertiary care, referral medical center. PATIENTS: We identified 6 patients with a verified histopathologic diagnosis of OP and antecedent or concomitant diagnosis of a hematologic malignancy from the Mayo Clinic database (1995-2003). Clinical, radiologic, and outcome data were abstracted from records. RESULTS: Underlying hematologic disorders included lymphoma (2), acute leukemia (2), cutaneous T-cell lymphoma (1), and myelodysplastic syndrome (1). OP was diagnosed by surgical lung biopsy in 4 and bronchoscopic biopsy in 2. Four of the 6 patients had previously been exposed to chemotherapeutic agents, two had not. Three of the 6 patients had bone marrow transplantation prior to development of OP. Five patients were treated with prednisone and all experienced symptomatic improvement with documented radiologic resolution in 4. One patient experienced symptomatic and radiologic resolution with observation alone. Three patients ultimately died from complications of their underlying hematologic disorder and 1 patient died of unknown causes. Two patients were alive without respiratory complaints more than 1 year after lung biopsy. CONCLUSIONS: OP occurs in patients with underlying hematologic malignancies who may or may not have been treated with chemotherapy and responds favorably to corticosteroid therapy.

Acute Disease↗
PubMed2006

Genetic association analysis of functional impairment in chronic obstructive pulmonary disease.

RATIONALE: Patients with severe chronic obstructive pulmonary disease (COPD) may have varying levels of disability despite similar levels of lung function. This variation may reflect different COPD subtypes, which may have different genetic predispositions. OBJECTIVES: To identify genetic associations for COPD-related phenotypes, including measures of exercise capacity, pulmonary function, and respiratory symptoms. METHODS: In 304 subjects from the National Emphysema Treatment Trial, we genotyped 80 markers in 22 positional and/or biologically plausible candidate genes. Regression models were used to test for association, using a test-replication approach to guard against false-positive results. For significant associations, effect estimates were recalculated using the entire cohort. Positive associations with dyspnea were confirmed in families from the Boston Early-Onset COPD Study. RESULTS: The test-replication approach identified four genes-microsomal epoxide hydrolase (EPHX1), latent transforming growth factor-beta binding protein-4 (LTBP4), surfactant protein B (SFTPB), and transforming growth factor-beta1 (TGFB1)-that were associated with COPD-related phenotypes. In all subjects, single-nucleotide polymorphisms (SNPs) in EPHX1 (p < or = 0.03) and in LTBP4 (p < or = 0.03) were associated with maximal output on cardiopulmonary exercise testing. Markers in LTBP4 (p < or = 0.05) and SFTPB (p = 0.005) were associated with 6-min walk test distance. SNPs in EPHX1 were associated with carbon monoxide diffusing capacity (p < or = 0.04). Three SNPs in TGFB1 were associated with dyspnea (p < or = 0.002), one of which replicated in the family study (p = 0.02). CONCLUSIONS: Polymorphisms in several genes seem to be associated with COPD-related traits other than FEV(1). These associations may identify genes in pathways important for COPD pathogenesis.

Aged↗
PubMed2005

Usual interstitial pneumonia complicating dyskeratosis congenita.

Dyskeratosis congenita (DC) is a rare disorder characterized by skin hyperpigmentation, nail dystrophy, and leukoplakia of mucous membranes. Pulmonary complications occur in approximately 20% of patients, although the specific histopathologic features, the temporal relationship between the diagnosis of DC and the development of pulmonary fibrosis, and the response to specific treatment are largely undefined. We describe 2 patients with DC who developed usual interstitial pneumonia. Pulmonary fibrosis developed 18 and 38 years after the original manifestations of DC. Both patients died of respiratory failure, 4 and 6 months after lung biopsy. Pulmonary fibrosis in patients with DC may be linked to underlying abnormalities of fibroblast function.

Adult↗
PubMed2005

Alkaptonuria diagnosed by flexible bronchoscopy.

A 68-year-old white man was evaluated for failure to wean from mechanical ventilation after cardiac surgery. Bronchoscopy performed prior to percutaneous dilatational tracheotomy revealed circumferential strikingly dark-colored airways, most prominent in the trachea and mainstem bronchi, extending distally into all airways with overlying desiccated black secretions. Histologic examination of bronchial mucosal biopsy samples and the desiccated secretions showed acute bronchitis and necrotic debris, respectively. This finding and the patient's history led to testing for plasma homogentisic acid, which was found to be elevated at 12.6 mug/mL, establishing the first diagnosis of alkaptonuria made using flexible bronchoscopy.

Aged↗
PubMed2004

Pulmonary embolization of 2-octyl cyanoacrylate after endoscopic injection therapy for gastric variceal bleeding.

N-butyl-2-cyanoacrylate, a tissue adhesive that polymerizes on contact with weak bases such as blood, is being used widely outside the United States to obliterate gastric varices. Embolization of this material can occur via portosystemic shunts. We report a case of pulmonary embolization of 2-octyl cyanoacrylate (an analogue of N-butyl-2-cyanoacrylate) that occurred after endoscopic injection therapy for gastric variceal bleeding. Cyanoacrylate embolism is difficult to diagnose with computed tomographic angiography because radiopaque emboli are masked by the contrast material. It is important to distinguish these emboli from conventional thromboemboli because "glue emboli" require only symptomatic treatment. Clinicians should have a high index of suspicion for embolism in the setting of tachycardia, chest pain, or hypoxia after a patient undergoes endoscopic injection therapy with cyanoacrylate glue for gastric variceal bleeding. The radiologist should be alerted so that the appropriate radiographic settings are used to make the diagnosis.

Cyanoacrylates↗
PubMed2004

Thoracic surgical procedures in patients with rheumatoid arthritis.

OBJECTIVE: Rheumatoid arthritis (RA) is associated with a variety of pleuropulmonary manifestations, some of which require surgical intervention. We investigated the spectrum of indications and results, as well as outcome associated with thoracic surgical procedures in rheumatoid patients. METHODS: Identification and retrospective review of medical records of 100 patients with RA at a tertiary-referral medical center undergoing thoracic surgical procedures over a 24-year period from January 1, 1976, to December 31, 1999. RESULTS: Sixty-four patients underwent surgical lung biopsy for localized lung lesions, 57 of which were nodules or masses; 24 lesions proved to be malignant. Surgical lung biopsy was undertaken in 40 patients for diffuse interstitial lung disease, 4 cases of which were found to be infectious. Five other patients underwent surgery for pleural disease, 3 of which proved to be benign pleuritis. CONCLUSION: Patients with rheumatoid disease undergo thoracic surgical procedures for a variety of indications, including benign and malignant localized lesions and pleural disease as well as diffuse parenchymal lung disease. The overall in-hospital mortality rate was low. However, the subgroup with diffuse interstitial lung disease was found to have a 20% in-hospital death rate following surgical lung biopsy.

Aged↗
PubMed2003

Etanercept for the treatment of stage II and III progressive pulmonary sarcoidosis.

BACKGROUND: Tumor necrosis factor (TNF)-alpha is produced by macrophages and other cells, and is believed to participate in granulomatous inflammation. Targeted antagonism of TNF-alpha has been proposed as a novel treatment strategy for sarcoidosis. Etanercept is a dimeric fusion protein that binds specifically to TNF-alpha, rendering it biologically inactive. OBJECTIVE: To assess whether etanercept has potential efficacy in the treatment of progressive pulmonary sarcoidosis. DESIGN: Prospective, open-label, phase-2 treatment trial. SETTING: Mayo Clinic, Rochester, MN. PATIENTS: Stage II or III progressive pulmonary sarcoidosis. INTERVENTION: Etanercept, 25 mg subcutaneously twice weekly. MEASUREMENTS: Pulmonary function, chest radiographs, dyspnea, and TNF-alpha levels in serum and BAL fluid. RESULTS: The study was terminated after the enrollment of 17 patients due to an early-stop clause of the pretrial study design related to excessive treatment failures. Neither absolute levels of TNF-alpha nor TNF-alpha activity in the serum, BAL fluid, or alveolar macrophages were able to predict which patients would respond to etanercept. CONCLUSIONS: In patients with progressive stage II or III pulmonary sarcoidosis, etanercept was frequently associated with early or late treatment failure. These data would not support the design of a large multicenter randomized trial comparing etanercept with conventional corticosteroid therapy.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed2003

Interferon gamma-1b therapy for advanced idiopathic pulmonary fibrosis.

OBJECTIVE: To report on observations made in a group of patients with idiopathic pulmonary fibrosis/usual interstitial pneumonia (IPF/UIP) who were treated with interferon gamma-1b. PATIENTS AND METHODS: We reviewed the clinical records, radiological studies, and pulmonary function data of all patients treated with interferon gamma-1b between January 2000 and March 2002 at the Mayo Clinic in Rochester, Minn. RESULTS: Twenty-one patients (mean age, 68 years; range, 52-80 years) were treated with interferon gamma-1b for a mean duration of 82 months (range, 3-21 months). All patients had been diagnosed previously as having IPF/UIP based on clinical, pulmonary function, and chest high-resolution computed tomographic scan criteria; 12 patients had also undergone video-assisted thoracoscopic surgical lung biopsy. Baseline pulmonary function data (mean +/- SEM percent predicted) were as follows: total lung capacity, 57.3 +/- 2.5; vital capacity, 55.0 +/- 3.3; diffusing capacity of lung for carbon monoxide, 39.7 +/- 2.8; and forced expiratory volume in 1 second, 58.1 +/- 3.6. Only 1 patient showed symptomatic and functional improvement, and 7 discontinued treatment because of a perceived lack of benefit. Eleven patients (52%) died after a mean of 6.4 months of treatment, and follow-up pulmonary function data suggested continued worsening in all but 1 patient. CONCLUSION: These observations do not support the use of interferon gamma-lb therapy for patients with advanced IPF/UIP.

Aged↗
PubMed2002

Relapsing polychondritis.

Relapsing polychondritis (RP) is a rare disease causing inflammation and destruction of cartilage and other connective tissues. Specific laboratory aberrations are lacking. Predominant clinical manifestations include auricular chondritis, polyarthritis, nasal chondritis, ocular inflammation, audiovestibular damage, and respiratory tract chondritis. A relapsing course is characteristic. Airways are involved in 50% of patients and may cause dyspnea, stridor, wheezing, hoarseness, aphonia, and laryneal or tracheal tenderness. Airflow obstruction may result from RP involving the tracheobronchial tree; there is no interstitial or pulmonary vascular component. Collapse or failure of the trachea to dilate during inspiration is a key feature. Fast computed tomographic (CT) scanners can visualize dynamic airway collapse. Randomized, controlled trials of therapy have not been done. Corticosteroids and nonsteroidal anti-inflammatory drugs (NSAIDs) are used most commonly, but optimal regimens and duration of therapy have not been elucidated. Endobronchial stents or tracheostomy may be required for severe stenoses refractory to medical therapy.

Journal Article↗
PubMed2002

Flexible bronchoscopic management of airway foreign bodies in children.

OBJECTIVES: To evaluate experience with the flexible bronchoscopic management of tracheobronchial foreign bodies (TFBs) in children (age < or = 16 years). DESIGN: All pediatric bronchoscopies performed by the bronchoscopy section at Mayo Clinic Rochester from 1990 through June 2001 for the suspicion of TFBs were reviewed. Information analyzed included the types of bronchoscope (rigid vs flexible) and techniques used, success rates of extraction of TFBs, and complications. RESULTS: Of the 94 children suspected of having TFBs, 39 children (28 boys and 11 girls; mean age, 47.3 months) were found to have 40 TFBs. The flexible bronchoscope was used exclusively to extract TFBs in 24 patients, and in 2 patients in whom the rigid bronchoscopic procedure was unsuccessful. Flexible bronchoscopy was performed through an endotracheal tube in 19 children. In the other five children, the procedure was accomplished through a laryngeal mask airway (LMA). In two additional patients in whom the rigid bronchoscopic procedure was unsuccessful, the instrument served as a conduit for the passage of the flexible bronchoscope. The extraction instruments employed included ureteral stone baskets and stone forceps. Since 1994, all extractions of TFBs were successfully accomplished with the flexible bronchoscope. Complications occurred in four patients who underwent rigid bronchoscopy, and included postbronchoscopy laryngeal edema manifested by stridor, cough, and respiratory distress. These resolved quickly with medical therapy. CONCLUSIONS: Flexible bronchoscopic extraction of pediatric TFBs can be performed safely with minimal risks and complications. In our experience, it was successful in all children in whom it was employed. Nevertheless, we caution that provisions be made to provide immediate rigid bronchoscopic management, should the attempts at flexible bronchoscopic extraction fail.

Adolescent↗
PubMed2002

Combined tracheal and esophageal stenting for palliation of tracheoesophageal symptoms from mediastinal lymphoma.

Mediastinal lymphoma as a cause of tracheobronchial obstruction is uncommon, and a malignant tracheoesophageal fistula in the setting of mediastinal lymphoma is rare. Malignant tracheoesophageal fistulas are associated with pronounced morbidity and mortality. We describe a patient with mediastinal lymphomatous infiltration resulting in tracheal obstruction, esophageal obstruction, and tracheoesophageal fistula that were successfully palliated with combined airway and esophageal stent placement.

Esophagoscopy↗