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Biomedical subjects

Jay H Ryu

Publications and source records attributed to Jay H Ryu.

At least 37 records · Page 2Linked to original sources
PubMed2005

Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung disease.

BACKGROUND: Desquamative interstitial pneumonia (DIP) and respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) are uncommon forms of interstitial lung disease and have been incompletely characterized. STUDY OBJECTIVES: To further characterize the clinical features and course of subjects with DIP and RB-ILD. DESIGN: Retrospective study. SETTING: Tertiary care, referral medical center. PATIENTS: Twenty-three subjects with DIP and 12 subjects with RB-ILD seen over a 12-year period between 1990 and 2001. INTERVENTIONS: None. RESULTS: The study population included 19 men (54%) and 16 women (46%). The mean (+/- SD) age at diagnosis was 46 +/- 10 and 43 +/- 7 years, respectively, for patients with DIP and RB-ILD. All subjects were either current or previous smokers except for three subjects with DIP. The diagnosis was confirmed in all cases by surgical lung biopsy. Bronchoscopy with transbronchial lung biopsy had been performed in 12 patients and was nondiagnostic in all. The most common pulmonary function abnormality was a reduced diffusing capacity of the lung for carbon monoxide. A CT scan of the chest revealed ground-glass opacities bilaterally in most patients who had DIP and RB-ILD. No differences were observed between subjects with DIP and RB-ILD with respect to clinical features, radiologic findings, or pulmonary function test results. The clinical course was characterized by relative stability in the majority of patients in both groups and a partial response to corticosteroid therapy. Five deaths were observed, including three resulting from progressive diffuse lung disease, all in subjects with DIP. CONCLUSIONS: We concluded that DIP and RB-ILD are chronic disease processes that in most patients are related to smoking. Persistent abnormalities can be seen on pulmonary function testing and radiologic studies despite smoking cessation and corticosteroid therapy. Corticosteroid therapy appeared to be associated with modest clinical benefit but usually not with resolution of disease. Progressive disease with eventual death can occur in subjects with DIP, especially with continued cigarette smoking.

Aged↗
PubMed2005

Low yield of microbiologic studies on pleural fluid specimens.

BACKGROUND: It is generally recommended that pleural fluid samples from pleural effusions of unknown cause be cultured for bacteria, mycobacteria, and fungi. However, the utility of this practice has been not been adequately assessed. DESIGN: Retrospective review. SETTING: Tertiary care, referral medical center. PATIENTS: Five hundred twenty-five patients undergoing diagnostic thoracentesis at Mayo Medical Center, Rochester, MN, over a 12-month period from July 1, 2001, to June 30, 2002. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: Among 525 patients undergoing diagnostic thoracenteses, 476 patients (91%) had one or more cultures performed on their pleural fluid specimens. Thirty-nine positive results (3.0% of 1,320 cultures) occurred in 35 of these 476 patients (7.4%). After excluding likely contaminants, true pathogens were identified in only 19 of 1,320 pleural fluid cultures (1.4%) belonging to 15 patients (3.2% of those who had cultures performed on their pleural fluid specimen). These positive results included 2.3% of aerobic bacterial, 1.2% of anaerobic bacterial, 1.4% of fungal, and 0% of mycobacterial cultures. Microbiologic smears performed on these pleural fluid samples included 357 Gram stains, 109 fungal smears (potassium hydroxide), and 232 acid-fast smears with positive yields of 2.5%, 0%, and 0%, respectively. These positive findings represented 1.3% of all smears performed. Of the specimens obtained from outpatient thoracenteses, only one had a true-positive result (0.8%). Only 1.1% (four specimens) of the cultures performed on free-flowing effusions demonstrated true pathogens; three of these four specimens grew fungi. CONCLUSIONS: The positive yield of microbiologic smears and cultures on pleural fluid specimens is low, particularly in the outpatient setting and in patients with free-flowing effusions. Microbiologic testing of pleural fluid specimens should be ordered more selectively.

Adolescent↗
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

Familial idiopathic pulmonary fibrosis: clinical features and outcome.

STUDY OBJECTIVES: Familial idiopathic pulmonary fibrosis (FIPF) has been defined as idiopathic pulmonary fibrosis (IPF) occurring in two or more members of a family. The clinical course of FIPF has not been fully defined. Accordingly, the current study was undertaken to establish clinical, radiologic, and histologic features, and survival in a consecutive series of patients with FIPF. DESIGN: Retrospective analysis of clinical, radiologic, and pathologic data from a consecutive series of patients with FIPF who were seen at Mayo Medical Center. Survival in patients with FIPF was contrasted to that of previously characterized patients with nonfamilial IPF who were evaluated at our institution. SETTING: Tertiary referral medical center. PATIENTS: We screened 47 patients and family members with FIPF from 15 families who were identified between the years 1992 and 2002. We further analyzed the subgroup of FIPF patients that was composed of 27 patients from 15 families in whom the complete clinical course was monitored at our institution. MEASUREMENTS: All patients exhibited clinical features that were compatible with IPF and either compatible high-resolution CT (HRCT) scan findings or histologic evidence of usual interstitial pneumonia. Clinical data, including symptoms, physical findings, HRCT scan findings, lung function test results, biopsy results, and survival were abstracted from the clinical records. RESULTS: Compared to patients with nonfamilial IPF, patients with FIPF did not demonstrate any notable differences in clinical, radiologic, or pathologic features. We observed that the total number of affected members in a family with FIPF was a significant risk factor for earlier mortality (p = 0.0157; hazard ratio, 1.434). Overall, however, patients with FIPF had a statistically similar outcome to those patients with nonfamilial IPF. CONCLUSIONS: Although uncommon, FIPF represents a distinct syndrome, which has clinical features and patient survival rates that are similar to those of nonfamilial IPF.

Adult↗
PubMed2005

Septic pulmonary embolism: presenting features and clinical course of 14 patients.

BACKGROUND: Septic pulmonary embolism (SPE) is an uncommon disorder with an insidious onset and is difficult to diagnose. STUDY OBJECTIVES: To characterize the presenting features and clinical course of patients with SPE. DESIGN: Retrospective study. SETTING: Tertiary care, referral medical center. PATIENTS: Fourteen subjects with SPE diagnosed during a 6-year period between 1996 and 2002. INTERVENTIONS: None. RESULTS: The median age of these patients was 37.5 years (range, 14 to 81 years) and included five women. Presenting symptoms included fever (93%), dyspnea (36%), pleuritic chest pain (29%), cough (14%), and hemoptysis (7%). The median duration of symptoms before diagnosis was 18 days (range, 5 to 180 days). A potential source or underlying condition that predisposed to SPE was identified in all 14 patients and included Lemierre syndrome (4 patients), central venous catheter infection (3 patients), prosthetic cardiac valve (2 patients), and pacemaker infection (2 patients). Two patients had a focal extrapulmonary infection, and one patient was an IV drug user. Most common pathogens were staphylococcal species (eight patients) and fusobacterium (four patients). Chest radiographic presentation was usually nonspecific, but CT was more helpful and revealed multiple nodular opacities peripherally, often with cavitation. Transesophageal echocardiography was performed in eight patients and demonstrated infectious vegetations in four cases. Aside from antimicrobial therapy and removal of infected devices, the management of these patients included cardiac surgery (two patients), thoracoscopic surgery with decortication (one patient), and tube thoracostomy (one patient). All 14 patients recovered from their illness. CONCLUSIONS: We conclude that SPE presents with variable and often nonspecific clinical and radiographic features. The diagnosis is usually suggested by the presence of a predisposing factor, febrile illness, and CT findings of multiple, nodular lung infiltrates peripherally, with or without cavitation.

Adolescent↗
PubMed2005

Interferon-gamma1b therapy in idiopathic pulmonary fibrosis: a metaanalysis.

CONTEXT: Despite the investigation of multiple therapeutic options, idiopathic pulmonary fibrosis (IPF) remains a devastating, progressively fatal disease. Much interest has focused on the use of interferon (IFN)-gamma1b therapy, but the efficacy of this treatment has not been proven. OBJECTIVE: To determine whether IFN treatment reduces mortality in patients with IPF. DESIGN: A meta-analysis of randomized controlled trials evaluating the use of IFN-gamma1b as treatment for IPF. MAIN OUTCOME MEASURE: Mortality in patients treated with IFN-gamma1b was compared to mortality in patients treated with control therapies. RESULTS: A total of three studies involving 390 patients was included in the analysis. IFN-gamma1b therapy was associated with reduced mortality (hazard ratio [HR], 0.418; 95% confidence interval [CI], 0.253 to 0.690; p = 0.0003). A comparison of mortality at different time points revealed that IFN-gamma1b therapy was associated with significantly reduced mortality at 1 year (0.0861; 95% CI, 0.0244 to 0.1478; p = 0.0063), 18 months (0.1682; 95% CI, 0.1065 to 0.2299; p < 0.0001), 650 days (0.1939; 95% CI, 0.1386 to 0.2492; p < 0.0001), and 2 years (0.2652; 95% CI, 0.1652 to 0.3652; p < 0.0001). CONCLUSION: When the results of multiple studies are combined in a meta-analysis, IFN-gamma1b therapy is associated with reduced mortality.

Humans↗
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

Pulmonary hypertension in patients with idiopathic pulmonary fibrosis.

STUDY OBJECTIVES: To determine the impact on survival and clinical correlates of pulmonary hypertension (PH) occurring in patients with idiopathic pulmonary fibrosis (IPF). DESIGN: Retrospective study. SETTING: Tertiary care, referral medical center. PATIENTS: Among 487 consecutive patients with IPF, we identified 136 patients who underwent transthoracic echocardiography within 3 months of their initial evaluation at our institution. Patients with left ventricular dysfunction, valvular heart disease, incomplete follow-up, and those in whom pulmonary artery pressures could not be assessed were excluded; the remaining 88 patients were included in this study. Correlations were performed between echocardiographic measures of PH and clinical variables including survival. MEASUREMENTS AND RESULTS: The mean (+/- SD) estimated systolic pulmonary artery pressure (SPAP) for the 88 patients was 48 +/- 16 mm Hg (range, 28 to 116 mm Hg). Among pulmonary function parameters, SPAP correlated best with diffusing capacity of the lung for carbon monoxide (D(LCO)), to which it was inversely related. For survival analysis, patients were stratified into three groups: < or = 35 mm Hg (14 patients), 36 to 50 mm Hg (47 patients), and > 50 mm Hg (27 patients). Using the Kaplan-Meier method, the median survival rates for these three groups were 4.8 years, 4.1 years, and 0.7 years, respectively. Those patients with SPAP > 50 mm Hg had significantly worse survival compared to other subgroups (p = 0.009). CONCLUSION: In patients with IPF, PH correlates inversely with D(LCO) and has a significant adverse impact on survival, particularly when SPAP is > 50 mm Hg.

Aged↗
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

Chronic nitrofurantoin-induced lung disease.

OBJECTIVE: To reassess the clinical and radiological features of chronic nitrofurantoin-induced lung disease and eventual clinical outcome. PATIENTS AND METHODS: We retrospectively reviewed the medical records of 18 patients with chronic nitrofurantoin-induced lung disease who were seen at the Mayo Clinic in Rochester, Minn, from January 1, 1997, to December 31, 2002. RESULTS: The median age of the 18 patients was 72 years (range, 47-90 years) at the time of diagnosis; 17 (94%) were women. Onset of symptoms occurred after a median interval of 23 months (range, 10-144 months) following the initiation of nitrofurantoin therapy for the prevention of recurrent urinary tract infections. All patients presented with persistent dyspnea and cough associated with lung infiltrates detected on chest radiography. Ten computed tomograms were available for review and revealed bilateral areas of ground-glass opacities in all cases and showed subpleural Irregular linear opacities and patchy consolidation in some cases. Nitrofurantoin therapy was discontinued in all patients, and most improved subsequently; 9 patients received corticosteroid therapy. CONCLUSIONS: Chronic nitrofurantoin-induced lung disease is seen predominantly in older women who present with respiratory symptoms after a year or more of nitrofurantoin therapy. Associated radiological features are relatively nonspecific but usually include bilateral areas of ground-glass opacities on computed tomography of the chest. Cessation of nitrofurantoin therapy leads to improvement and suffices in the management of some patients, although corticosteroid therapy may be helpful in those more severely affected.

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↗
PubMed2005

Etiology of chylothorax in 203 patients.

OBJECTIVES: To characterize the etiology of chylothorax in patients encountered at a single tertiary referral center and to compare the findings with those from previous studies. PATIENTS AND METHODS: The medical records of all patients with chylothorax seen at the Mayo Clinic in Rochester, Minn, over a 21-year period, from January 1, 1980, to December 31, 2000, were retrospectively reviewed to ascertain the underlying cause of their condition. RESULTS: We identified 203 patients with chylothorax; 92 were females (male-female ratio, 1.21). The median age was 54.5 years (range, 21 weeks' gestation to 93 years). Dyspnea, the most common presenting symptom, occurred in 98 (56.6%) of 173 patients in whom initial symptoms were recorded, whereas 64 (37.0%) had no respiratory symptoms. Median duration of symptoms before diagnosis was 7.5 weeks (range, 1 day to 4.5 years). Causes of chylothorax included surgery or trauma in 101 patients (49.8%), various medical conditions in 89 (43.8%), and unknown in 13 (6.4%). Among surgical procedures, esophagectomy (29 patients) and surgery for congenital heart disease (28 patients) were the most common causes of chylothorax. Among medical conditions, lymphoma (23 patients), lymphatic disorders (19 patients), and chylous ascites (16 patients) were the most common causes. CONCLUSIONS: Chylothorax has numerous causes. In contrast to previous studies, surgery or trauma was the most common cause of chylothorax at our institution, accounting for nearly 50% of cases. Lymphoma and other malignancies caused chylothorax in only 16.7% of cases. These numbers are possibly related to the high volume of cardiothoracic surgical procedures performed at our tertiary referral center.

Adolescent↗
PubMed2005

Cough-induced rib fractures.

OBJECTIVES: To define the demographic, clinical, and radiological features of patients with cough-induced rib fractures and to assess potential risk factors. PATIENTS AND METHODS: For this retrospective, single-center study, we identified all cases of cough-induced rib fractures diagnosed at the Mayo Clinic in Rochester, Minn, over a 9-year period between January 1, 1996, and January 31, 2005. Bone densitometry data from patients' medical records were analyzed, and T scores were used to classify patients into bone density categories. RESULTS: The mean +/- SD age of the 54 study patients at presentation was 55+/-17 years, and 42 patients (78%) were female. Patients presented with chest wall pain after onset of cough. Rib fracture was associated with chronic cough (> or =3 weeks' duration) in 85% of patients. Rib fractures were documented by chest radiography, rib radiography, computed tomography, or bone scan. Chest radiography had been performed in 52 patients and revealed rib fracture in 30 (58%). There were 112 fractured ribs in 54 patients. One half of patients had more than one fractured rib. Right-sided rib fractures alone were present in 17 patients (26 fractured ribs), left-sided in 23 patients (35 fractured ribs), and bilateral in 14 patients (51 fractured ribs). The most commonly fractured rib on both sides was rib 6. The fractures were most common at the lateral aspect of the rib cage. Bone densitometry was done in 26 patients and revealed osteopenia or osteoporosis in 17 (65%). CONCLUSIONS: Cough-induced rib fractures occur primarily in women with chronic cough. Middle ribs along the lateral aspect of the rib cage are affected most commonly. Although reduced bone density is likely a risk factor, cough-induced rib fractures can occur in the presence of normal bone density.

Adult↗
PubMed2004

Pulmonary Langerhans' cell histiocytosis.

Pulmonary Langerhans' cell histiocytosis (PLCH) is an uncommon but important cause of interstitial lung disease, and it occurs predominantly in adult cigarette smokers. PLCH belongs to the spectrum of Langerhans' cell histiocytosis (LCH), diseases characterized by uncontrolled proliferation and infiltration of various organs by Langerhans' cells. Other clinical entities within this spectrum of LCH are seen in adults and children and vary in severity from mild disease that requires no therapy to severe disseminated forms with extensive organ involvement and high mortality. Organ systems involved by LCH may include skin, bone, pituitary gland, lymph nodes, and lungs. Although LCH is approximately three times more common in children than adults, pulmonary involvement is much more common in adults with LCH, in whom it frequently occurs as the sole organ involved with disease. This article summarizes recent advances and current understanding of PLCH.

Algorithms↗
PubMed2004

Histoplasmosis with tenosynovitis of the hand and hypercalcemia mimicking sarcoidosis.

We report on an otherwise healthy 47-year-old male who developed subacute tenosynovitis of the hand associated with night sweats and inguinal lymphadenopathy. He had a past history of granulomatous mediastinal lymphadenitis with positive histoplasmosis serology 11 years previously. Carpal tunnel exploration with biopsy demonstrated granulomatous inflammation. Granulomatous inflammation, hypercalcemia, and an elevated serum angiotensin converting enzyme (ACE) level suggested the diagnosis of sarcoidosis, however histoplasmosis infection could eventually be diagnosed. This unusual presentation of histoplasmosis underscores the fact that the diagnosis of sarcoidosis requires careful exclusion of other causes of granulomatous inflammation, particularly infectious agents. Even in the setting of an elevated ACE level and hypercalcemia, the possibility of an infectious etiology must be considered before establishing a diagnosis of sarcoidosis.

Journal Article↗
PubMed2004

Cryptococcal laryngitis: case report and review of the literature.

Infections caused by Cryptococcus neoformans may range from an asymptomatic illness to lethal systemic disease, especially in immunocompromised hosts. Although cryptococcal infection most commonly involves the lungs or the central nervous system, it can disseminate to virtually any organ. Laryngeal cryptococcal infections are extremely rare--only 5 cases have been reported in the literature. Herein, we describe cryptococcal laryngitis occurring in a 55-year-old man with asthma and allergic fungal sinusitis. He presented with hoarseness and cough. He was treated with itraconazole followed by fluconazole therapy with complete recovery from his laryngeal infection. The patient was disease-free when last seen 10 months after the diagnosis. We present a summary of all reported cases of cryptococcal laryngitis.

Asthma↗
PubMed2004

Pneumothorax in pulmonary Langerhans cell histiocytosis.

BACKGROUND: Pulmonary Langerhans cell histiocytosis (PLCH) is a smoking-related interstitial lung disease characterized by development of cystic changes that predispose to occurrence of pneumothorax. STUDY OBJECTIVES: To determine the frequency, recurrence rate, and optimal management of pneumothorax associated with PLCH. DESIGN: Retrospective study. SETTING: Tertiary care, referral medical center. PATIENTS: One hundred two adults >or= 18 years old with histologically confirmed PLCH seen at Mayo Clinic Rochester over a 23-year period from 1976 to 1998. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: Sixteen of 102 patients (16%) with PLCH had pneumothorax; mean age at the time of diagnosis was 29.4 years (range, 18 to 52 years), and all had smoked cigarettes. There were 37 episodes of pneumothoraces (1 to 5 episodes per patient); 10 patients (63%) had more than one episode. Median age at diagnosis of PLCH was significantly younger in patients with pneumothorax when compared to those without pneumothorax (27 years vs 41.5 years, p < 0.001), but pulmonary function parameters and survival after diagnosis were not significantly different. Rate of recurrent pneumothorax was 58% to the ipsilateral side when the episode was managed by observation or chest tube without pleurodesis, and 0% after surgical management with pleurodesis. CONCLUSIONS: These data support the early use of surgical therapy with pleurodesis in managing patients with PLCH and spontaneous pneumothorax.

Adolescent↗