Psychometric Evaluation of the Union Physio-Psycho-Social Assessment Questionnaire / 中国医学科学院学报

Objective To validate the Union Physio-Psycho-Social Assessment Questionnaire(UPPSAQ-70)and test its validity and reliability.Methods From April,2013 to July,2018,patients were asked to finish the computer evaluation of UPPSAQ-70 and Symptom Checklist 90(SCL-90)in Peking Union Medical College Hospital(PUMCH).Confirmatory factor analysis(CFA)was conducted on the SPSS 17.0,and the number of fixed factors was 8 factors and 3 factors.Amos 23.0 was used to verify the original 8-factor model,8-factor revision model,3-factor model,3-factor revision model,and single-factor model.Each factor of SCL-90 was used as the calibration standard to calculate the correlation coefficient between factors.The retest reliability was tested by the outpatients in PUMCH in July,2018.Results Exploratory factor analysis indicated that the 8-factor revised model included:depression,anxiety and fatigue,sleep,physical discomfort,sexual function,happiness and satisfaction,hypochondria,and social anxiety.The 3 factors revised model included that:psychological,physiological and social dimension.Confirmatory factor analysis indicated that the 8-factor modified model was superior to the 3-factor model and the single-factor model: =10 410.4,=1862,RMSEA=0.07,CFI=0.753,and NFI=0.715.With SCL-90 as the standard criteria,except the low correlation coefficient between emotional scale and depression(=0.600)and anxiety(=0.520),the correlation coefficients of other symptoms were below 0.5.The chronbach's between each factor and total score of UPPSAQ-70 was between 0.823 and 0.904,and the Chronbach's coefficient of the whole scale was between 0.954 and 0.956 after each item was deleted.The retest reliability of the scale of 32 participants Chronbach's was 0.847.Each item of the scale measured between one week was significantly correlated(<0.05). Conclusion UPPSAQ-70 is a good scale for evaluating overall health status and is especially feasible in general hospitals.

Pathological and high resolution CT findings in Churg-Strauss syndrome / 中国医学科学杂志(英文版)

<p><b>OBJECTIVE</b>To investigate the Churg-Strauss syndrome (CSS) associated lung involvement, concentrating on clinical characteristics, pathological findings of lung involvements, response to treatment, and prognosis.</p><p><b>METHODS</b>We retrospectively analyzed the characters of the clinical manifestations, thin-section CT and pathological findings of CSS. The study involved 16 patients. Clinical data were obtained by chart review. All patients underwent transbronchial lung biopsy (TBLB). Six of them underwent surgical lung biopsy as well.</p><p><b>RESULTS</b>The patients included 7 men and 9 women, aged from 14 to 61 years (median, 47.5 years). Extrathoracic organs involved included nervous system (7/16) and skin (5/16). Respiratory symptoms included cough (12/16), exertional dyspnea (11/16), hemoptysis (4/16), and chest pain (3/16). CT findings included bilateral ground-glass opacities (12/16), bilateral patchy opacities (12/16), and centrilobular nodules (6/16). The pathological findings of TBLB demonstrated increased eosinophils (3/16), vasculitis (3/16), and interstitial pneumonia (16/16). The pathological findings of surgical lung biopsy of 6 cases showed necrotizing vasculitis in 4 cases, capillaries in 5, eosinophilic pneumonia in 3, granulomas in 2, and airway abnormalities in 3. All patients improved in symptoms after therapy during the study period (range, 3 to 51 months; median, 15 months).</p><p><b>CONCLUSIONS</b>Asthma may be present in CSS patient when there is bronchial involvement. Ground-glass opacities and consolidation seen on high-resolution CT reflect the presence of eosinophilic pneumonia, vasculitis, and pulmonary alveolar hemorrhage. TBLB has significant limitations for the diagnosis of CSS. Early diagnosis and therapy can result in satisfactory prognosis.</p>

Multiple dimensions of cardiopulmonary dyspnea / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>The current theory of dyspnea perception presumes a multidimensional conception of dyspnea. However, its validity in patients with cardiopulmonary dyspnea has not been investigated.</p><p><b>METHODS</b>A respiratory symptom checklist incorporating spontaneously reported descriptors of sensory experiences of breathing discomfort, affective aspects, and behavioral items was administered to 396 patients with asthma, chronic obstructive pulmonary disease (COPD), diffuse parenchymal lung disease, pulmonary vascular disease, chronic heart failure, and medically unexplained dyspnea. Symptom factors measuring different qualitative components of dyspnea were derived by a principal component analysis. The separation of patient groups was achieved by a variance analysis on symptom factors.</p><p><b>RESULTS</b>Seven factors appeared to measure three dimensions of dyspnea: sensory (difficulty breathing and phase of respiration, depth and frequency of breathing, urge to breathe, wheeze), affective (chest tightness, anxiety), and behavioral (refraining from physical activity) dimensions. Difficulty breathing and phase of respiration occurred more often in COPD, followed by asthma (R(2) = 0.12). Urge to breathe was unique for patients with medically unexplained dyspnea (R(2) = 0.12). Wheeze occurred most frequently in asthma, followed by COPD and heart failure (R(2) = 0.17). Chest tightness was specifically linked to medically unexplained dyspnea and asthma (R(2) = 0.04). Anxiety characterized medically unexplained dyspnea (R(2) = 0.08). Refraining from physical activity appeared more often in heart failure, pulmonary vascular disease, and COPD (R(2) = 0.15).</p><p><b>CONCLUSIONS</b>Three dimensions with seven qualitative components of dyspnea appeared in cardiopulmonary disease and the components under each dimension allowed separation of different patient groups. These findings may serve as a validation on the multiple dimensions of cardiopulmonary dyspnea.</p>

The St. George's Respiratory Questionnaire in lymphangioleiomyomatosis / 中国医学科学杂志(英文版)

<p><b>OBJECTIVE</b>To examine the correlation between the health-related quality of life measured by the St. George's Respiratory Questionnaire (SGRQ) and the commonly used physiological measures in lymphangioleiomyomatosis (LAM).</p><p><b>METHODS</b>This study retrospectively analyzed the SGRQ scores and other measures (the Borg scale of breathlessness at rest, 6-minute walking distance, blood oxygen levels, and pulmonary function) of patients diagnosed and confirmed with LAM. Altogether 38 patients between June 2007 and November 2009 were included.</p><p><b>RESULTS</b>The mean values of the SGRQ three components (symptoms, activity, and impacts) and total scores in the LAM patients were 46.95 +/- 28.90, 58.47 +/- 25.41, 47.89 +/- 29.66, and 51.11 +/- 26.35, respectively. The SGRQ total or component scores were correlated well with the Borg scale of breathlessness, 6-minute walking distance, partial pressure of oxygen in arterial blood, spirometry and diffusion capacity of lung. There were poor correlations between SGRQ score and residual volume or total lung capacity. In our preliminary observation, sirolimus improved the SGRQ total and three component scores and the Borg scale of breathlessness significantly after 101-200 days of treatment (n = 6).</p><p><b>CONCLUSIONS</b>The SGRQ score in LAM is correlated well with physiological measures (Borg scale of breathlessness, 6-minute walking distance, blood oxygen levels, and pulmonary function tests). The SGRQ could therefore be recommended in baseline and follow-up evaluation of patients with LAM. Treatment with sirolimus, an inhibitor of mammalian target of rapamycin, may improve the quality of life and patient's perception of breathlessness in LAM.</p>

Diagnostic specificity of dynamic assessment and monitoring of spirometry in asthma / 中国医学科学院学报

<p><b>OBJECTIVE</b>To evaluate the diagnostic specificity of dynamic assessment and monitoring using a portable spirometer in diagnosis and differential diagnosis of asthma.</p><p><b>METHODS</b>We retrospectively reviewed the results of dynamic monitoring of spirometry in 145 symptomatic patients with physician-diagnosed asthma. Flow-volume curve and simultaneous symptoms and mood were measured in a fixed-time thrice-daily assessment schedule for 2 weeks. Patients were allowed to make additional measurements in case of symptom exacerbations.</p><p><b>RESULTS</b>The clinical data of 51 males and 94 females with a mean age of (39.1 +/- 13.0) years (ranged from 10 to 65 years) were analyzed. Duration of asthma before study was (6.7 +/- 9.9) years. Of 145 patients with physician-diagnosed asthma, 126 (87%) could be conclusively confirmed for a diagnosis of asthma. Asthma was misdiagnosed in 14 patients (9.7%). Overdiagnosis of asthma was observed in 5 patients (3.4%).</p><p><b>CONCLUSION</b>Dynamic assessment and monitoring using a portable spirometer by revealing variability and reversibility of airway obstruction may provide an additional tool for diagnosis and differential diagnosis of asthma.</p>

Clinical features and prognosis in 21 patients with extrinsic allergic alveolitis / 中国医学科学杂志(英文版)

<p><b>OBJECTIVE</b>To assess the spectrum of causes, clinical features, differences between disease phases, and prognosis of extrinsic allergic alveolitis (EAA).</p><p><b>METHODS</b>Patients with EAA diagnosed at Peking Union Medical College Hospital from August 1983 to May 2007 were analyzed retrospectively. Their medical records were examined to gather clinical, laboratorial, radiological, and histopathological data. Patients were divided to three phases (acute, subacute, and chronic) according to clinical presentations. Follow-up data regarding treatment response, subsequent radiological and pulmonary function studies, and clinical outcomes were collected.</p><p><b>RESULTS</b>A total of 21 cases were enrolled. Among them, 11 were subacute, 10 were chronic. The most common exposure was pet birds (6 cases, 28.6%). The primary abnormality of pulmonary function was restriction and/or reduction in diffusing capacity (12 cases, 63.2%). The most common findings on high-resolution computed tomography (HRCT) were ground-glass opacities (13 cases, 68.4%) and centrilobular nodules (8 cases, 42.1%). Airway obstruction in pulmonary function test, emphysema, lung cysts, and fibrosis on HRCT were more frequently seen in chronic than in subacute patients, though the differences were not statistically significant. Bronchoalveolar lavage fluid (BALF) showed lymphocytosis. The total cell count and the percentage of neutrophils were significantly higher in subacute than in chronic patients (P<0.05). Nonnecrotizing granulomas were seen in 8 (47.1%) cases. Improvement or normalization in symptoms, radiography, and pulmonary function test after treatment were seen in all 18 patients with available follow-up data. Five patients recurred.</p><p><b>CONCLUSIONS</b>The characteristic abnormalities of pulmonary function, findings on HRCT, and pathology are essential for all phases of EAA, and the atypical manifestations such as obstruction and fibrosis can also be present frequently, particularly in chronic cases. Differential cell counts of BALF are related to the phase of the disease. The treatment response and prognosis of EAA are good.</p>

Pathologic study of diffuse pulmonary interstitial fibrosis caused by chronic hypersensitivity pneumonitis / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the pathologic characteristics of chronic hypersensitivity pneumonitis, especially the pattern of pulmonary interstitial fibrosis; and to compare the histologic features with those of idiopathic interstitial pneumonitis.</p><p><b>METHODS</b>The HE-stained paraffin sections of 10 cases of chronic hypersensitivity pneumonitis encountered during the period from 2000 to 2008 were retrospectively analyzed.</p><p><b>RESULTS</b>There were altogether 6 males and 4 females, with age of patients ranging from 23 to 59 years (mean=47.2 years). Clinically, the patients presented with chronic cough and shortness of breath for 4 months to 6 years. Histologically, 7 cases showed usual interstitial pneumonitis (UIP)-like fibrosis. Patchy fibrosis was observed under the pleura, adjacent to interlobular septa and around bronchioles. In all of the 7 cases, foci of fibroblastic proliferation, as well as bronchiolar metaplasia of peribronchiolar alveoli and mild bronchiolitis, were noted. Three cases presented with mild honeycomb changes of lung and 3 cases showed non-specific interstitial pneumonitis (NSIP)-like fibrosis, in which the alveolar septa were expanded by fibrous tissue and collagen, with relative preservation of alveolar architecture. Bronchiolitis and lymphocytic infiltrates in alveolar septa were seen. Schaumann bodies were identified in 1 case. In general, patients with chronic hypersensitivity pneumonitis were younger than patients with idiopathic UIP. Computed tomography often showed upper and middle lobar involvement and mosaic attenuation. Compared with idiopathic UIP, the UIP-like fibrosis of chronic hypersensitivity pneumonitis often occurred not only under the pleura and adjacent to interlobular septa, but also around bronchioles and was accompanied by bronchiolar metaplasia.</p><p><b>CONCLUSIONS</b>Chronic hypersensitivity pneumonitis can mimic other types of lung conditions with interstitial fibrosis, especially UIP and NSIP. As a result, some cases of chronic hypersensitivity pneumonitis may be misdiagnosed as such.</p>

Clinicopathologic study of Churg-Strauss syndrome / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinical and pathologic features of Churg-Strauss syndrome (CCS).</p><p><b>METHODS</b>Three cases of Churg-Strauss syndrome, including 1 autopsy case and 2 cases with open thoracoscopic lung biopsy, were retrospectively reviewed. All the tissue samples were formalin-fixed, paraffin-embedded and stained with hematoxylin and eosin.</p><p><b>RESULTS</b>The first patient was a 68-year-old man who had history of asthma for 4 years, with recent exacerbation and chest pain for 2 weeks. Patient died 1 day after admission due to myocarditis and myocardial infarction. He did not have peripheral eosinophilia, skin or paranasal sinus pathology. CSS represented an incidental autopsy finding and he had never been treated with corticosteroid before. The other 2 patients were a 58-year-old male and a 12-year-old female, respectively. Both had history of asthma, peripheral eosinophilia and lung consolidations on computed tomographic examination. Pathologically, all cases showed vasculitis, perivascular allergic-type granulomas, eosinophilic pneumonia and asthmatic bronchitis.</p><p><b>CONCLUSIONS</b>Thorough understanding of the clinical and pathologic criteria is essential for arriving at a correct diagnosis of CSS. Although some patients may present with atypical symptoms, lung biopsies often reveal the classic histologic findings which include vasculitis and perivascular allergic granuloma formation.</p>

Clinicopathologic study of primary marginal zone B-cell lymphoma of MALT type and lymphoid hyperplasia of lung / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features, immunohistochemical findings and immunoglobulin heavy chain (IgH) gene rearrangement results of primary pulmonary mucosa-associated lymphoid tissue lymphoma (MALToma) and reactive lymphoid hyperplasia.</p><p><b>METHODS</b>Twenty cases, included 13 cases of pulmonary MALToma and 7 cases of pulmonary lymphoid hyperplasia, encountered during the period from 1989 to 2007, were retrospectively analyzed. The samples were paraffin-embedded and stained with hematoxylin and eosin. Immunohistochemical study and semi-nested polymerase chain reaction for IgH gene rearrangement were performed.</p><p><b>RESULTS</b>The 13 cases of primary pulmonary MALToma were composed of a spectrum of lymphoid cells, including lymphocyte-like cells, centrocyte-like cells and mononuclear B cells with plasmacytoid differentiation. They often had diffuse or marginal zone growth patterns. Lymphoid follicles with neoplastic colonization were apparent. The lymphoma cells spread along alveolar septa and bronchovascular bundles. Vascular invasion was noted in 9 cases, pleura involvement in 6 cases and nodal involvement in 2 cases. Lymphoepithelial lesions (LEL) were identified in 9 cases of pulmonary MALToma. Immunohistochemically, the lymphocytes in LEL were CD20-positive and CD3-negative. On the other hand, LEL was also present in 2 of the 7 cases of lymphoid hyperplasia studied, with a mixture of CD20-positive B cells and CD3-negative T cells. Eight of the 9 cases of primary pulmonary MALToma were positive for IgH gene rearrangement, while all of the 7 cases of lymphoid hyperplasia were negative.</p><p><b>CONCLUSIONS</b>Histologically, the cell population of primary pulmonary MALToma is similar to that of extranodal MALToma occurring in other organs. LEL, though commonly observed in pulmonary MALToma, are not specific and can also be seen in cases of reactive lymphoid hyperplasia. The immunophenotype of intraepithelial lymphocytes in pulmonary MALToma and reactive lymphoid hyperplasia is different. The presence of a monotonous population of CD20-positive intraepithelial lymphocytes supports a diagnosis of MALToma. IgH gene rearrangement study is also useful in differentiating both entities.</p>

Fearful imagery induces hyperventilation and dyspnea in medically unexplained dyspnea / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Medically unexplained dyspnea refers to a condition characterized by a sensation of dyspnea and is typically applied to patients presenting with anxiety and hyperventilation without underlying cardiopulmonary pathology. We were interested to know how anxiety triggers hyperventilation and elicits subjective symptoms in those patients. Using an imagery paradigm, we investigated the role of fearful imagery in provoking hyperventilation and in eliciting symptoms, specifically dyspnea.</p><p><b>METHODS</b>Forty patients with medically unexplained dyspnea and 40 normal subjects matched for age and gender were exposed to scripts and asked to imagine both fearful and restful scenarios, while end-tidal PCO(2) (PetCO(2)) and breathing frequency were recorded and subjective symptoms evaluated. The subject who had PetCO(2) falling more than 5 mmHg from baseline and persisting at this low level for more than 15 seconds in the imagination was regarded as a hyperventilation responder.</p><p><b>RESULTS</b>In patients with medically unexplained dyspnea, imagination of fearful scenarios, being blocked in an elevator in particular, induced anxious feelings, and provoked a significant fall in PetCO(2) (P < 0.05). Breathing frequency tended to increase. Eighteen out of 40 patients were identified as hyperventilation responders compared to 5 out of 40 normal subjects (P < 0.01). The patients reported symptoms of dyspnea, palpitation or fast heart beat in the same fearful script imagery. Additionally, PetCO(2) fall was significantly correlated with the intensity of dyspnea and palpitation experienced during the mental imagery on one hand, and with anxiety symptoms on the other.</p><p><b>CONCLUSIONS</b>Fearful imagery provokes hyperventilation and induces subjective symptoms of dyspnea and palpitation in patients with medically unexplained dyspnea.</p>

Low dose pirfenidone suppresses transforming growth factor beta-1 and tissue inhibitor of metalloproteinase-1, and protects rats from lung fibrosis induced by bleomycina / 中国医学科学杂志(英文版)

<p><b>OBJECTIVE</b>To investigate the optimal dosage of pirfenidone for the treatment of pulmonary fibrosis induced by bleomycin in Wistar rats, and the alteration of expressions of transforming growth factor beta-1 (TGF-beta 1), tissue inhibitor of metalloproteinase-1 (TIMP-1), and matrix metalloproteinase-13 (MMP-13) in lung tissue.</p><p><b>METHODS</b>Male Wistar rats were endotracheally instilled with bleomycin or normal saline. Pirfenidone (25-800 mg x kg(-1) x d(-1)), dexamethasone (3 mg/kg), or 1% carboxymethylcellulose sodium were given daily by feed 2 days before instillation of bleomycin. Groups T7 and T14 were fed pirfenidone 50 mg x kg(-1) x d(-1) at 7 days or 14 days after bleomycin instillation. Lungs were harvested at 28 days after bleomycin instillation. Patholological changes in lung tissues were evaluated with HE staining. Lung collagen was stained by sirius red and measured by content of hydroxyproline. Expression of proteins of TGF-beta 1, TIMP-1, and MMP-13 were detected by Western blotting.</p><p><b>RESULTS</b>At doses of 25, 50, and 100 mg x kg(-1) x d(-1), pirfenidone had significant anti-fibrotic effects for bleomycin-induced rat pulmonary fibrosis, and these effects were most significantly attenuated at the dosage of 50 mg x kg(-1) x d(-1) (HE: P < 0.01, P < 0.01, and P = 0.064; sirius red: P < 0.05, P < 0.01, and P < 0.05; hydroxyproline: P = 0.595, P < 0.01, and P = 0.976). Pirfenidone at a dosage of 50 mg x kg(-1) x d(-1) inhibited protein expression of TGF-beta1 and TIMP-1 in lung tissue in the early phase (0.79 and 0.75 times of control group), but had no effect on expression of MMP-13.</p><p><b>CONCLUSION</b>Low dose pirfenidone, especially at dosage of 50 mg x kg(-1) x d(-1), has significant anti-fibrotic effects on bleomycin-induced rat pulmonary fibrosis. Pirfenidone partially inhibits the enhancement of the expression of TGF-beta 1 and TIMP-1 in lung tissue.</p>

Clinicopathologic manifestation of amiodarone-induced lung injury / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinical, pathologic and radiologic features of amiodarone-induced lung injury.</p><p><b>METHODS</b>The clinical, pathologic and radiologic features, including treatment and follow-up information of three cases diagnosed as amiodarone-induced lung injury from October 2004 to October 2005 in the Peking Union Medical College Hospital were reviewed.</p><p><b>RESULTS</b>All the patients were males, with age ranging from 35 to 64 years. The duration of symptoms varied from 20 days to 3 years. All presented with cough. Worsening dyspnea and inspiratory crackles were noted in two patients. Computerized tomography showed bilateral patchy infiltration, ground-glass appearance and accentuation of bronchovascular markings. The most common pathologic manifestations included cellular interstitial pneumonia associated with intra-alveolar collections of macrophages and type II pneumocyte hyperplasia. Some of the macrophages contained finely vacuolated cytoplasm. An organizing pneumonia pattern was seen in one patient.</p><p><b>CONCLUSIONS</b>Amiodarone-induced lung injury has characteristic pathologic features which may provide clues to diagnosis. Correlation with clinical and radiologic findings is also important.</p>

Clinical application of portable spirometry in asthma / 中国医学科学院学报

<p><b>OBJECTIVE</b>To investigate the clinical applications of portable spirometry in asthma.</p><p><b>METHODS</b>Twenty patients with asthma were recruited from Peking Union Medical College Hospital. Flow-volume loop, simultaneous asthma symptoms, and mood were monitored three times a day for consecutive 14 days.</p><p><b>RESULTS</b>In patients with a normal daytime spirometry, marked decline of forced expiratory volume in one second (FEV1) and peak expiratory flow (PEF) were observed at night and/or in the early morning. A within subject correlation analysis between FEV1, PEF, and asthma symptoms showed that the correlation between symptoms and airway obstruction was found only in seven out of twenty patients (35%). Four patients (20%) reported many symptoms with nearly normal portable spirometry. Accordingly, their symptoms were not correlated with FEV1 and PEF. This group of patients was defined as over-perceivers. On the contrary, another two patients (10%) did not report any symptoms while obvious airways obstruction was recorded by a portable spirometry. These patients were defined as under-perceivers.</p><p><b>CONCLUSIONS</b>Dynamic monitoring of flow-volume loop with a portable spirometry is more accurate than routine lung function test in assessment of asthma severity. In addition, combined with simultaneous monitoring of symptoms, it would be of particularly helpful in identifying two specific types of asthma patients, e.g. over-perceivers and under-perceivers.</p>

Diagnosis and differential diagnosis of airway-centered interstitial fibrosis / 中国医学科学院学报

<p><b>OBJECTIVE</b>To describe a form of interstitial lung disease pathologically characterized by small airway-centered interstitial fibrosis (ACIF).</p><p><b>METHODS</b>We analyzed the clinical, pulmonary functional, radiographic, and histologic characteristics of one ACIF case in Peking Union Medical College Hospital and reviewed 12 cases in literatures.</p><p><b>RESULTS</b>Clinically, patients presented with chronic cough and progressive dyspnea. Pulmonary function tests showed restrictive ventilatory pattern. Bronchoalveolar lavage showed a mild increase in lymphocytes in most cases. Chest radiography revealed diffuse reticulonodular infiltrates, with thickening of the bronchial walls and surrounding fibrosis. The key finding in histopathology was a distinctive pattern of ACIF centered on membranous and respiratory bronchioles.</p><p><b>CONCLUSIONS</b>ACIF is a disease that do not fit into any known category of interstitial lung disease. Whether it is a unique disease remains to be determined.</p>

Respiratory manifestations in amyloidosis / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Amyloidosis is a collection of diseases in which different proteins are deposited. Amyloid deposits occur in systemic and organ-limited forms. In both systemic and localized forms of the disease, lung can be involved. The aim of this study was to explore the different respiratory manifestations of amyloidosis.</p><p><b>METHODS</b>Chest radiology, clinical presentations, bronchoscopic/laryngoscopic findings and lung function data of 59 patients with amyloidosis involving respiratory tract collected during January 1986 to March 2005, were analysed.</p><p><b>RESULTS</b>Of the 16 cases with localized respiratory tract amyloidosis, 8 had the lesions in the trachea and the bronchi, 2 in the larynx and the trachea, 5 in the larynx and/or the pharynx, and 1 in the lung parenchyma. Of 43 systemic amyloidosis with respiratory tract involvement, 3 had the lesions in bronchi, 13 in lung parenchyma, 33 in pleura, 8 in mediastina, 1 in nose and 1 in pharynx. Chest X-rays were normal in most cases of tracheobronchial amyloidosis. CT, unlike chest X-rays, showed irregular luminal narrowing, airway wall thickening with calcifications and soft tissue shadows in airway lumen. Localized lung parenchymal amyloidosis presented as multiple nodules. Multiple nodular opacities, patch shadows and reticular opacities were the main radiological findings in systemic amyloidosis with lung parenchymal involvement. In pleural amyloidosis, pleural effusions and pleural thickening were detected. Mediastinal and/or hilar adenopathy were also a form of lung involvement in systemic amyloidosis. The major bronchoscopic findings of tracheobronchial amyloidosis were narrowing of airway lumen, while nodular, 'tumour like' or 'bubble like' masses, with missing or vague cartilaginous rings, were detected in about half of the patients.</p><p><b>CONCLUSIONS</b>Localized respiratory tract amyloidosis mostly affects the trachea and the bronchi. Chest X-rays are not sensitive to detect these lesions. Systemic amyloidosis often involves lung parenchyma and the pleura. Open lung biopsy or pleural biopsy should be performed for the diagnosis.</p>

Medically unexplained dyspnea in children: a review of 34 cases / 中华儿科杂志

<p><b>OBJECTIVE</b>Medically unexplained dyspnea is common in adult and accounts for 14% patients complaining of dyspnea. Its occurrence in children is seldom recognized. In the present paper, 34 children with medically unexplained dyspnea (age 10 to 18 years) seen in Peking Union Medical College Hospital from 1996 to 2002 are reported.</p><p><b>METHODS</b>The diagnosis of medically unexplained dyspnea was clinical: it was based on the presence of dyspnea and other complaints which cannot be explained by an organic disease. The patients answered Nijmegen questionnaire and state and trait anxiety (STAI), and performed hyperventilation provocation test. Twenty sessions of breathing therapy were applied and 13 out of 34 children were followed up after the therapy.</p><p><b>RESULTS</b>Among the children, 75% started to have symptoms at the age of 13 to 16 years, though the age of first episode could be as early as 8 years. In most of the cases, the course was chronic clinically. In addition to marked dyspnea, their clinical profile included symptoms of hyperventilation i.e. blurred vision, dizziness, tingling, stiff fingers or arm. The symptoms of anxiety were less frequent in children and accordingly the level of anxiety evaluated by means of STAI was lower in children compared to adult patients. The precipitating psychological factors appeared to be related to middle school competition. Pressure from exams, reprimand from stern and unsympathetic teachers coupled with high parental expectation could be emotionally damaging to psychologically susceptible children. Thirteen patients were followed up after 2-3 months of breathing therapy with emphasis on abdominal breathing and slowing down of expiration. After therapy, the sum score of the Nijmegen Questionnaire was markedly decreased. Dyspnea and symptoms of hyperventilation were improved. The level of anxiety was minimally modified.</p><p><b>CONCLUSION</b>The cases illustrated the need for careful diagnostic evaluation and treatment because of the high rate of chronicity of the disorder.</p>

Medically unexplained dyspnea: psychophysiological characteristics and role of breathing therapy / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Medically unexplained dyspnea occurs commonly in medical settings and remains poorly understood. This study was conducted to investigate the psychophysiological characteristics of medically unexplained dyspnea and the efficacy of breathing retraining for these patients.</p><p><b>METHODS</b>A group of patients with medically unexplained dyspnea were compared to patients with a variety of organic lung diseases and healthy subjects. In another group of patients, the influence of breathing therapy on complaints, anxiety, and breath-holding was evaluated for an average of 1.5 years.</p><p><b>RESULTS</b>Patients with medically unexplained dyspnea reported more intense dyspnea than patients with a variety of organic lung diseases. Additionally, they were anxious and presented a broad range of symptoms in daily life and under challenge, for instance voluntary hyperventilation. More than one third of them qualified for panic disorder. They had shorter breath-holding time at rest, less increase in breath-holding time and higher chances of showing a "paradoxical" decrease of breath-holding time after hyperventilation. A combination of PaO2, forced expiratory volume in one second (FEV1), and anxiety measures distinguished them from organic dyspnea. Breathing retraining profoundly improved their symptoms and decreased the level of state and trait anxiety. Moreover, they better tolerated the voluntary hyperventilation and the symptoms induced were also markedly decreased after therapy. Breath-holding time was prolonged and PetCO2 in a representative group of patients increased.</p><p><b>CONCLUSIONS</b>Patients with medically unexplained dyspnea appear to have the feature of a "psychosomatic" patient: an anxious patient with a wide variety of symptoms of different organ systems that do not have an organic basis. They can be distinguished from organic dyspnea using a small set of physiological and psychological measures. Breathing retraining turns out to be an effective therapy for those "difficult to treat patients".</p>

Diagnosis and treatment of medically unexplained dyspnea / 中国医学科学院学报

Medically unexplained dyspnea refers to a group of patients presenting marked dyspnea without structural alterations of organs/systems after thorough examinations. This clinically neglected group of patients accounts for about 14% of patients with dyspnea in secondary health care. They appear very difficult to manage clinically. In this paper an organized approach used to diagnose medically unexplained dyspnea is presented. Breathing re-training is recommended as a therapy for those "difficult to treat patients".

Inhibition of human lung fibroblast proliferation and the mitogen activated protein kinase pathway by dexamethasone / 中国医学科学院学报

<p><b>OBJECTIVE</b>To investigate the effects of dexamethasone on human lung fibroblast cell proliferation, cell cycles, and cell mitogen-activated protein kinases (MAPKs) passway.</p><p><b>METHODS</b>Dexamethasone was used at various concentration in culture medium. Cell number was counted using a hemacytometer. Whole cell propidium iodide staining and flow cytometric analysis were performed to determine cellular DNA content. MAPK proteins and activation were tested by Western blot analysis with antibodies to c-Jun N-terminal kinase (JNK), phospho-JNK, extracellular signal-regulated kinase (ERK), phospho-ERK, p38 and phospho-p38.</p><p><b>RESULTS</b>1x10(-7) mol/L and 1x10(-6) mol/L dexamethasone suppressed the proliferation of lung fibroblast cells by 34% and 72%, respectively, than that of control. This suppression was dose-dependant. Dexamethasone suppressed cell cycle with accumulation of cells in G1/G0 stage. It increased from 81.9% to 90.1% compared with that of control. We did not find any apoptosis induced by dexamethasone for lung fibroblast cells. Using Western blot analysis, we found that dexamethasone resulted in decreased activity of ERK, but had no effects on JNK and p38.</p><p><b>CONCLUSIONS</b>Dexamethasone may suppresses the proliferation of lung fibroblast cells, which is partly resulted from the facts that it can inhibit ERK activation in MAPK-signaling pathway but has little effect on JNK and p38 pathway. Dexamethasone may not induce lung fibroblast cell apoptosis directly.</p>

Pathogenesis of cigarette smoke-induced chronic obstructive pulmonary disease and therapeutic effects of glucocorticoids and N-acetylcysteine in rats / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>T lymphocytes and matrix metalloproteinase (MMP) play an important role in the pathogenesis of chronic obstructive pulmonary disease (COPD). However, the details of the mechanisms involved are unclear. The aims of this study were to investigate the changes in interferon-gamma (IFN-gamma), interleukin-4 (IL-4), MMP-9, MMP-12 and tissue inhibitor of metalloproteinase-1 (TIMP-1) levels in a smoke-induced COPD rat model and the therapeutic effects of glucocorticoids and N-acetylcysteine.</p><p><b>METHODS</b>Male Wistar rats were exposed to cigarette smoke for 3.5 months. Budesonide or N-acetylcysteine was given in the last month. Lung function was measured at the end of the study. IL-4 and IFN-gamma levels were then determined in bronchoalveolar lavage fluid and lung tissue samples by enzyme-linked immunosorbent assay. The expression of MMP-9, MMP-12 and TIMP-1 mRNA in lung tissue was determined by RT-PCR.</p><p><b>RESULTS</b>In comparison with the control group, rats exposed to smoke had a significant increase in IL-4 and MMP-12 levels and a significant decrease in IFN-gamma levels. In addition, the IL-4/IFN-gamma ratio and MMP-12/TIMP-1 ratio were both higher. At the same time, the ratio of forced expiratory volume in 0.3 second to forced vital capacity (FEV(0.3)/FVC) and dynamic compliance (C(dyn)) decreased and expiratory resistance (Re) increased. By measuring pulmonary mean linear intercept and mean alveolar numbers, obvious emphysematous changes were observed in the smoke exposed group. After treatment with budesonide, IL-4 and MMP-12 decreased and IFN-gamma increased. The IL-4/IFN-gamma ratio returned to normal, though the MMP-12/TIMP-1 ratio remained unchanged. FEV(0.3)/FVC was significantly higher and Re was significantly lower than that in untreated smoke exposed rats. No significant differences were found in pulmonary mean linear intercept and mean alveolar numbers. After treatment with N-acetylcysteine, IFN-gamma increased and the IL-4/IFN-gamma ratio decreased. The MMP-12/TIMP-1 ratio remained unchanged. Re and C(dyn) both improved obviously. No significant differences were found in pulmonary mean linear intercept and mean alveolar numbers. Correlation analysis indicated that IL-4 levels in lung tissue correlated negatively with FEV(0.3)/FVC (r = -0.53, P = 0.001), IFN-gamma levels in lung tissue correlated negatively with Re (r = -0.63, P = 0.000) and positively with C(dyn) (r = 0.44, P = 0.009), and that the IL-4/IFN-gamma ratio correlated negatively with FEV(0.3)/FVC (r = -0.44, P = 0.010) and C(dyn) (r = -0.42, P = 0.015) and positively with Re (r = 0.58, P = 0.000). Finally, MMP-12 correlated negatively with FEV(0.3)/FVC (r = -0.36, P = 0.026).</p><p><b>CONCLUSIONS</b>Cigarette smoke exposure increases IL-4 levels and decreases IFN-gamma levels. This may be the result of smoke-induced changes in lung function. Budesonide can mitigate the changes in IL-4 and IFN-gamma levels induced by smoke exposure. N-acetylcysteine has no effect on IL-4, but increases IFN-gamma levels and brings the IL-4/IFN-gamma ratio back to normal. Cigarette smoke can also promote MMP-12 gene expression and elevate the MMP-12/TIMP-1 ratio. This effect may play a role in smoke-induced emphysema. Budesonide and N-acetylcysteine do not alter the MMP-12/TIMP-1 ratio in this study when given in the late phase of smoke exposure.</p>