Biomedical subjects

Joseph G Parambil

Publications and source records attributed to Joseph G Parambil.

11 recordsLinked to original sources

PubMed2006

Microscopic polyangiitis associated with thymoma, exacerbating after thymectomy.

A variety of autoimmune diseases has been associated with thymoma, and thymectomy does not always induce remission of these disorders. This case report describes a 50-year-old man who presented with migratory polyarthritis and an anterior mediastinal mass that proved to be a thymoma. Five months after thymectomy, the patient presented with worsening polyarthritis, hematuria, and azotemia. Based on elevated titers of antineutrophil cytoplasmic antibodies directed against myeloperoxidase and renal biopsy showing crescentic necrotizing glomerulonephritis, microscopic polyangiitis was diagnosed. After remission-induction therapy with prednisone and cyclophosphamide, articular symptoms and renal manifestations resolved. Microscopic polyangiitis was not associated previously with thymoma, and this case broadens the spectrum of autoimmune disorders seen with this tumor. Progressive disease seen after thymectomy in this patient has potential implications regarding the pathophysiological characteristics of microscopic polyangiitis and management of patients with this clinical association.

Antibodies, Antineutrophil Cytoplasmic↗

PubMed2006

Bronchogenic cyst causing atrial fibrillation by impinging the right inferior pulmonary vein.

Atrial fibrillation is the most common sustained cardiac arrhythmia and is usually associated with underlying structural heart disease, but may also occur in isolation--the entity of "lone" atrial fibrillation. Recently, attention has been directed to the pulmonary veins as a source of the arrhythmia through identification of rapidly firing ectopic foci within the covering myocardial sleeves. We describe a 38-year-old man who presented with treatment-resistant atrial fibrillation and a posterior mediastinal mass. Electrophysiological studies demonstrated abnormal foci of electrical activity at the entrance of the right inferior pulmonary vein into the left atrium. Surgical exploration revealed a bronchogenic cyst that distorted and stretched the right inferior pulmonary vein as it traversed the posterior mediastinum towards the left atrium. Restoration of sinus rhythm without recurrence of atrial fibrillation characterized the clinical course after surgical resection of the mass. This case demonstrates that a retro-cardiac bronchogenic cyst can cause atrial fibrillation by impinging on a pulmonary vein.

Adult↗

PubMed2006

Diffuse alveolar damage: uncommon manifestation of pulmonary involvement in patients with connective tissue diseases.

BACKGROUND: Diffuse alveolar damage (DAD) is a relatively common finding on surgical lung biopsy and can result from a variety of causes. METHODS: We studied nine consecutive patients with connective tissue disease (CTD) and DAD diagnosed on surgical lung biopsy to examine this association and clinical implications. RESULTS: The median age was 63 years (range, 35 to 76 years), and seven of the patients were women (78%). Underlying CTDs included rheumatoid arthritis in five patients, polymyositis in two patients, and one patient each with systemic sclerosis and mixed CTD. In seven patients (78%), CTD had been diagnosed before the onset of DAD; six of these patients had a preexisting interstitial lung disease (ILD) related to their CTD. DAD was the presenting manifestation leading to a new CTD diagnosis in two patients (22%). CT of the chest revealed ground-glass opacities and/or consolidation bilaterally with or without honeycombing. In all patients, surgical lung biopsy revealed DAD for which no cause could be identified other than the underlying CTD. Seven patients (78%) were receiving mechanical ventilatory support at the time of the surgical lung biopsy. Four patients (44%) survived to hospital discharge and included one patient with preexisting ILD and all three patients without chronic ILD. CONCLUSION: We conclude that DAD can complicate the clinical course of patients with CTD-related chronic ILD, or can occasionally occur as a presenting manifestation of CTDs. When DAD occurs in patients with CTDs, the outcome appears to be worse for those with preexisting chronic ILD compared to those without ILD.

Adult↗

PubMed2006

Nontraumatic disruption of the fibrocartilaginous trachea: causes and clinical outcomes.

BACKGROUND: Nontraumatic disruption of the fibrocartilaginous trachea is rare, and the appropriate management of this condition is not well-characterized. METHODS: Retrospective analysis of the clinical features, causes, and outcomes with surgical and nonsurgical management in nine adult patients with nontraumatic fibrocartilaginous tracheal disruption identified by bronchoscopy from January 1, 1975, to December 31, 2004, at a single institution. RESULTS: The most common cause was external beam radiotherapy (RT) in five patients. Other causes included postoperative complications of cervical and superior mediastinal operations in three patients and Aspergillus fumigatus-induced ulcerative tracheobronchitis in one patient post-lung transplantation. Four patients were treated surgically; three because of significant pneumomediastinum and one because the size of the tracheal defect made spontaneous healing seem unlikely. A silicone stent was placed in one patient for concomitant tracheal narrowing, and one patient was treated medically with antifungal agents. The remaining three patients were followed up serially without any intervention. With these treatments, only one patient died as a consequence of tracheal disruption. Repeat bronchoscopies were performed in seven of the remaining eight patients and confirmed healing of the necrotic defect in all. CONCLUSION: Nontraumatic disruption of the fibrocartilaginous trachea occurs most commonly as a consequence of external beam RT. It can also occur as a complication of cervical and superior mediastinal operations or from A fumigatus-induced ulcerative tracheobronchitis post-lung transplantation. Although surgical treatment has been generally recommended for patients with this condition, patients with contained disruptions without evidence of pneumomediastinum may be managed nonoperatively.

Aged↗

PubMed2006

Interstitial lung disease in primary Sjögren syndrome.

BACKGROUND: Primary Sjögren syndrome (pSS) has been associated with various histologic patterns of interstitial lung disease (ILD). METHODS: We retrospectively identified 18 patients with pSS and suspected ILD who underwent lung biopsies (14 surgical biopsies and 9 bronchoscopic biopsies) at our institution during a 13-year period from 1992 through 2004. Histopathologic findings were analyzed and correlated with radiologic features and outcome. RESULTS: Median age was 62 years (range, 34 to 78 years), and 15 patients (83%) were women. Most patients presented with dyspnea and cough. Chest radiographs demonstrated bilateral infiltrates, and high-resolution CT revealed abnormalities of various types including ground-glass, consolidation, reticular, and nodular opacities. The major histopathologic patterns included nonspecific interstitial pneumonia (NSIP) [five patients], organizing pneumonia (OP) [four patients], usual interstitial pneumonia (UIP) [three patients], lymphocytic interstitial pneumonia (three patients), primary pulmonary lymphoma (two patients), and diffuse interstitial amyloidosis (one patient). In four patients (three with OP and one with amyloidosis), the diagnosis was established on transbronchial biopsy results. Treatment commonly included prednisone with or without another immunosuppressive agent. During the follow-up period (median, 38 months), most patients improved or remained stable except three patients with UIP, one patient with NSIP, and one patient with amyloidosis. Seven patients (39%) died, including three deaths from acute exacerbation of interstitial pneumonia. CONCLUSIONS: A variety of histologic patterns can be seen in patients with pSS-associated ILD. Those with UIP tended to have progression of lung disease. Death from acute exacerbation of interstitial pneumonia may occur in patients with pSS-associated ILD.

Adrenal Cortex Hormones↗

PubMed2006

Effect of shifting costs to patients on specialty evaluation for sleep disorders.

OBJECTIVE: To determine whether the introduction of out-of-pocket expenses to medical center employees would lead to decreased use of sleep disorder services. PATIENTS AND METHODS: We retrospectively analyzed and compared the clinical and medical accounting data from visits by Mayo Clinic employees to the Sleep Disorders Center from January 1 to March 31, 2003, with that of January 1 to March 31, 2004, le, before and after a January 2004 increase in co-payments for evaluation and testing. RESULTS: The total number of new patients evaluated in the first quarters of 2003 and 2004 was similar (113 vs 119; P = .37). Snoring, restless legs symptoms, hypertension, atrial fibrillation, and prior overnight oximetry testing were more prevalent in 2004 than in 2003 (P = .05, P = .01, P < .001, P = .003, P = .02, respectively). In contrast, insomnia and parasomnia complaints were less common in 2004 (P < .001). The mean apnea-hypopnea index, minimum oxygen saturation, and percentage of time with oxygen saturation less than 90% were all more severe in 2004 (P = .01, P = .001, P < .001, respectively). Sleep-related breathing disorders were more commonly diagnosed in 2004 (83.2% vs 67.2%; P = .02), whereas the diagnoses of nonbreathing disorders declined. CONCLUSION: The insurance policy changes that resulted in larger employee co-payments shifted the spectrum of diagnoses seen at the Sleep Disorders Center toward more symptomatic patients, with more associated comorbidities, and patients who had more severe sleep-related breathing disorders. Total utilization did not decrease.

Academic Medical Centers↗

PubMed2005

Causes and presenting features of pulmonary infarctions in 43 cases identified by surgical lung biopsy.

BACKGROUND: Although pulmonary infarction is usually associated with pulmonary thromboembolism, it can occur with other disorders such as vasculitis, angioinvasive infections, sickle-cell disease, tumor embolism, and pulmonary torsion. STUDY OBJECTIVE: To identify causes and presenting features of pulmonary infarctions diagnosed by surgical biopsy in a consecutive series of patients encountered at a single institution. DESIGN: Retrospective review. SETTING: Tertiary care, referral medical center. PATIENTS: Forty-three patients with pulmonary infarction identified on surgical lung biopsy over a period of 7 years, January 1996 through December 2002. RESULTS: The median age of these 43 patients was 55 years (range, 22 to 85 years); 17 patients (40%) were women, and 26 patients (60%) were men. Thirty-five patients (81%) had a smoking history. Twenty-eight patients (65%) presented with solitary or multiple lung nodules/masses of undetermined etiology. The underlying cause was identifiable in 31 cases (72%) based on a review of clinical, laboratory, radiologic, and histopathologic data. The two most common causes were pulmonary thromboembolism (18 cases, 42%) and pulmonary infections (5 cases, 12%). Thromboembolic pulmonary infarctions typically presented as solitary or multiple nodules located in the subpleural regions. Other causes included diffuse alveolar damage in two cases (5%), pulmonary torsion in two cases (5%), and one case each of lung cancer, amyloidosis, embolotherapy, and catheter embolism. In 12 cases (28%), the underlying cause was not directly identifiable but was probably due to previous pulmonary thromboembolism. CONCLUSION: We conclude that although pulmonary thromboembolism is the most common cause of pulmonary infarction identified by surgical lung biopsy, a variety of other causes are clinically encountered, including infections, inflammatory or infiltrative lung diseases, pulmonary torsion, malignancy, and nonthrombotic embolism. Pulmonary infarction should be considered in the differential diagnosis of peripheral lung nodules or masses.

Adult↗

PubMed2005

Lack of evidence for an association between neurofibromatosis and pulmonary fibrosis.

STUDY OBJECTIVES: To reassess the association between neurofibromatosis and pulmonary fibrosis. DESIGN: Retrospective single-center study with analysis of patients' chest radiographs, CT scans, and medical records. SETTING: Tertiary care, referral medical center. PATIENTS: One hundred fifty-six adult patients with neurofibromatosis seen over a 6-year period between 1997 and 2002. RESULTS: A review of chest radiographs revealed abnormal findings in 70 patients (44.9%). The most common radiographic abnormalities were extrapulmonary nodules or masses seen in 22 patients (14.1%), followed by skeletal abnormalities in 16 patients (10.3%). Bilateral interstitial infiltrates were noted in only three patients (1.9%), all of whom had potential causes other than neurofibromatosis for their lung infiltrates, including smoking-related interstitial lung disease, rheumatoid lung disease, recurrent pneumonias, and a history of ARDS. CT scans were available in two of these patients and revealed nonspecific patterns of abnormalities with no honeycombing. Six patients had bullae or cystic airspaces demonstrated on chest radiography or CT scan; all of these findings occurred in the context of smoking-related emphysema. Combined together, bilateral interstitial lung infiltrates or cystic airspaces were demonstrated in five patients (3.2%) by chest radiography, and in eight patients (5.1%) by chest radiography or CT scanning; one patient had both findings on the CT scan. CONCLUSIONS: We found little evidence to support an association between neurofibromatosis and pulmonary fibrosis or any other form of parenchymal lung disease. Interstitial lung disease and bullae described in association with neurofibromatosis in previous reports may have, in part, represented smoking-induced manifestations.

Adult↗

PubMed2005

Histopathologic features and outcome of patients with acute exacerbation of idiopathic pulmonary fibrosis undergoing surgical lung biopsy.

STUDY OBJECTIVES: To define the clinicopathologic features and outcome of acute exacerbation in patients with idiopathic pulmonary fibrosis (IPF) undergoing surgical lung biopsy. DESIGN: Retrospective, single-center study. SETTING: Tertiary care, referral medical center. PATIENTS: Seven patients with acute exacerbation of IPF who underwent surgical lung biopsy. RESULTS: The median age of these seven patients was 70 years (range, 59 to 74 years); two were women. Five patients had a smoking history and included two current smokers. All patients were experiencing an exacerbation of dyspnea for a median duration of 14 days (range, 7 to 28 days) prior to presentation. In three of these patients, the acute deterioration was the presenting feature of IPF, while in the remaining four patients the diagnosis of IPF had previously been established. Chest radiography demonstrated bilateral mixed alveolar-interstitial infiltrates in all of them. CT revealed ground-glass opacities and consolidation bilaterally in all patients with associated peripheral honeycombing in six of them. Echocardiography was performed in six patients and demonstrated pulmonary hypertension in all. BAL fluid was obtained in five patients and revealed neutrophilia in all. Surgical lung biopsy showed diffuse alveolar damage (DAD) in five patients with associated collagen fibrosis and honeycomb changes typical of usual interstitial pneumonia (UIP). One biopsy showed a combination of UIP and organizing pneumonia, while one biopsy showed only DAD. Despite treatment with lung-protective ventilation strategies and high-dose systemic corticosteroids, six patients (86%) died during their hospitalization. CONCLUSIONS: Although IPF is typically associated with an insidious, slowly progressive clinical course, acute exacerbations occur and may be the presenting manifestation in some patients. In either situation, current management strategies including high-dose corticosteroid therapy appear to be relatively ineffective for these patients with acute exacerbation undergoing surgical lung biopsy.

Aged↗

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↗

PubMed2005

Solitary pulmonary metastasis presenting 20 years after primary resection of Wilms tumor.

Wilms tumor is the most common renal malignancy of childhood. Relapse occurs most often within 4 years of initial diagnosis, and the most common site of metastasis is the lung. We describe a 22-year-old man who presented with hemoptysis and a solitary pulmonary lesion 20 years after primary resection of Wilms tumor. Computed tomography of the chest showed an indeterminate pulmonary mass of heterogeneous attenuation with no other intrathoracic abnormalities. Surgical resection revealed a solitary pulmonary metastasis from Wilms tumor. Further evaluation yielded no evidence of extrathoracic metastases. This case shows that late relapse in the form of a solitary pulmonary mass can occur in patients with Wilms tumor.

Adult↗