The adult respiratory distress syndrome (ARDS) occurs in patients with various underlying illnesses. It has been suggested that complement activation may play a crucial role in the pathophysiology of ARDS by direct actions of complement fragments and/or by release of mediators including endotoxin, superoxide anion, and arachidonate metabolites. Therefore, complement evaluation was performed in patients with ARDS, with emphasis on SC5b-9 (Membrane Attack Complex).
Complement split products, Cod, Bb, and SC5b-9, respectively from the classical, alternative, and terminal complement pathway were measured by enzyme immunoassay (EIA) using respective monoclonal antibodies. Plasma S-protein (vitronectin) concentration was also evaluated by EIA.
We could not detect SMAC in normal controls or in a C5-deficient patient with abdominal infection. In contrast, most patients with ARDS and patients at risk showed elevated SMAC, and an increase in the values in association with exacerbation of the disease. However, two patients with ARDS exhibited lower values of SMAC in spite of deterioration and subsequent death. It was suggested that in these two ARDS patients, liver dysfunction may have contributed to the reduced SMAC formation. The finding that S-protein concentration (a MAC inhibitor) in the plasma of these two patients was reduced supports this speculation.
It is suggested that simultaneous evaluation of SMAC and S-protein in plasma is helpful in determining the prognosis of critically ill patients.

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We investigated the long-term (3-30 months) therapeutic effects of low-dose (300-600mg/day) erythromycin in 26 patients with diffuse panbronchiolitis (DPB).
Significant improvements of pulmonary functions especially in %VC and PaO₂ as well as respiratory symptoms were shown. However, erythromycin treatment was not associated with a significant change in surface phenotypes on peripheral blood lymphocytes (CD4, CD8, CD4/CD8).
It is well known that serum levels of tumor-associated carbohydrate antigens such as SLX (sialylated Lewis X-i) and CA19-9 (sialylated Lewis^a) are significantly elevated in patients with DPB.
In the present study, 68.4% (13/19) of DPB patients showed marked elevation of SLX and 52.9% (9/17) showed marked elevation of CA19.9 levels in serum. These positive ratios were significantly decreased by erythromycin treatment to 31.6% (6/19) in SLX and 23.4% (4/17) in CA19-9.
The mean values of each marker were also significantly decreased after erythromycin administration from 54.9±26.9U/ml to 39.5±22.1U/ml for SLX and from 70.5±77.4U/ml to 28.8±37.4U/ml for CA19-9.

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We investigated whether regular use of beta-agonist inhalation concomitantly with regular beclomethasone dipropionate (BDP) inhalation is necessary in chronic bronchial asthma. Twenty chronic asthmatic patients who were stable on regular BDP and beta-agonist inhalation were studied. After a 2 week observation period, the patients were randomly assigned to two groups. One group recieved BDP 400μg/day (2 puffs 4 times) and beta-agonist inhalation as required for 4 weeks. This period was followed by 4 weeks of treatment with BDP 400μg/day and regular beta-agonist inhalation. The other group received these treatments in the reverse order. No significant differences among the two groups were observed in attack score, ADL score, sleep score, and %PEFR. In addition, no differences were detected in these parameters between the periods of regular use of beta-agonist and the periods of use as required. The frequency of inhalation of beta-agonist during the use as required period correlated with the sleep score and difference in %PEFR between morning and night, and was significantly lower than the frequency of inhalation during the period of regular. From these results, we conclude that inhalation of beta-agonist on a regular basis is not necessary to achieve the same degree of relief of symptoms as treatment with β₂-agonist and BDP inhalation on regular basis in patients with chronic asthma.

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The measurement of serum SLX is thought to be a useful aid in the diagnosis of malignant diseases, particularly adenocarcinoma of the lung. In the present investigation, we measured and compared SLX values in BALF from affected and normal bronchi, obtained from 83 patients. They consisted of 64 males and 19 females, with mean age of 60 years, consisting of 8 normal controls, 19 cases of benign lung disease, and 56 cases of primary lung cancer. SLX value in BALF from normal bronchi was significantly higher in patients with lung cancer than in normal controls, but there was no significant difference in SLX value between lung cancer and benign lung disease. On the other hand, SLX value from affected bronchi was significantly higher in patients with lung cancer than in normal controls and patients with benign lung disease. The rate of elevated SLX in BALF from affected bronchi was significantly higher in patients with lung cancer than in those with benign lung disease. These results suggest that measurement of SLX levels in BALF from affected bronchi may be a useful method for differential diagnosis of primary lung cancer.

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The distribution of ferruginous bodies in the lung in cases of malignant pleural mesothelioma with definite occupational history of asbestos exposure was examined, and the length of ferruginous bodies and the kinds of asbestos fibers were also evaluated. Ferruginous bodies were most numerous in the upper lobes, especially in S¹, and were least numerous in the lower lobes. On the other hand, the longest ferruginous bodies were detected in the lower lobes. Amosite and crocidolite (amphibole groups) were found in almost all cases of malignant pleural mesothelioma, but chrysotile was found in only one case. The longer fiber length of asbestos in the amphibole group was considered to be important in the carcinogenicity of malignant mesothelioma, since almost all cases of malignant mesothelioma originated from the lower pleura.

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Fractional analysis of bronchoalveolar lavage (FABAL) fluid was performed in 6 control patients and 41 patients with various interstitial lung disease. The cell differential counts in the first 30ml fraction of BAL (FBAL-I), which is considered to be the bronchial lavage, differed from those of the 50ml second and third fraction (FBAL-III). Hypersensitivity pneumonitis, pulmonary tuberculosis, and sarcoidosis showed a high recovery of lymphocytes (52%); however, the former two disorders were occasionally, associated with neutrophil airway inflammation, whereas sarcoidosis was not. The percentage recovery of neutrophils in total FBAL was considerably high in patients with diffuse panbronchiolitis, and relatively high in those with collagen vascular disease, idiopathic pulmonary fibrosis, pneumoconiosis, and control smokers. However, these neutrophils were largely recovered from FBAL-I, suggesting the presence of airway inflammation. Thus, it is valuable to apply the FBAL method to determine the topographic distribution of inflammatory cells in the lungs. It was also found that the lymphocyte morphology in the lavage fluid was of value in establishing the diagnosis of hypersensitivity pneumonitis, and it is critical whether or not mast cells and basophils are present in BALF since they indicate the pathologic state of allergy or fibrosis. Although present in various fibrotic lung diseases in a limit number, langerhans cells are a diagnostic marker for histiocytosis X.

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The purpose of this study was to establish the optimal bronchodilating drug among therapies currently available for clinical treatment of the stable phase of chronic obstructive pulmonary disease (COPD). The efficacy of ipratropium bromide 40μg, salbutamol 200μg, and ipratropium bromide 40μg plus salbutamol 200μg was compared in 14 patients with COPD. Daily PEFR was obtained during the last seven days of a 2 week period incorporating drug inhalation four times daily. FEV₁ and FVC were assessed on the final day of the treatment period. In the absence of bronchodilating medication, FEV₁, was 1.27±0.13l (52.9±5.1% pred). With ipratropium bromide 40μg alone, FEV₁ was 1.43±0.13l (59.8±5.3% pred). A similar value was obtained for salbutamol 200μg: 1.45±0.14l (61.0±5.4% pred). However, FEV₁ following the administration of ipratropium bromide 40μg in combination with salbutamol 200μg was 1.51±0.13l (63.6±5.3% pred). The percent increase in FEV₁ (compared to the value obtained without medication) was significantly higher with combined ipratropium bromide 40μg plus salbutaml 200μg (122.2±3.8%) than with either ipratropium bromide 40μg (114.8±5.5%) or salbutamol 200μg (116.5±4.4%) alone. Furthermore, the daily post-dilator PEFR improved significantly more with the combined therapy four times a day (311±29l/min) than with either ipratropium bromide 40μg (296±30l/min) or salbutamol 200μg (303±29l/mm) therapy alone. There was no discernible difference between results obtained with ipratropium bromide 40μg versus salbutamol 200μg.
The additive effect of oral theophylline when compared with ipratropium bromide 40μg plus salbutamol 200μg was also studied in eight patients with stable COPD, whose serum theophylline was more than 10μg/ml. FEV₁ with ipratropium and salbutamol treatment, in the absence of oral theophylline administration, was 1.62±0.10l (69.9±5.2% pred). FEV₁ with combined ipratropium, salbutamol, and oral theophylline therapy was 1.71±0.10l (73.6±5.4% pred). The percent increase in FEV₁ (compared to the value obtained without bronchodilator) with the addition of oral theophylline (130.9±6.9%) was not significantly different from that observed without oral theophylline therapy (123.5±4.3%). Improvements in FEV₁, FVC, and daily PEFR were similarly insignificant. In conclusion, the combination of ipratropium bromide 40μg with salbutamol 200μg constitutes the most appropriate therapy for stable COPD.

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In order to elucidate the background of candida infection in patients with pulmonary disease, we divided 100 patients into 2 groups according to their serum titer of CAND-TEC determined by latex agglutination test for candida antigen. Group C1 (83 patients) had CAND-TEC value less than 2:1, and Group C2 (17 patients) had CAND-TEC value greater than 4:1. There was a significantly higher incidence of respiratory failure, hypoalbuminemia, and fever unresponsive to antibiotics in Group C2 compared with Group C1. Regarding the sputum culture of candida, there were significantly more positive cases in Group C2 than in Group C1. Furthermore, Group C2 showed a significantly poorer prognosis than Group C1.
Generally, the candida antigen titer correlated well with clinical findings and with the course of infection and treatment with antifungal drugs.
These results suggest that administration of antifungal drugs may be indicated in patients with fever unresponsive to antibiotics when the candida antigen titer is greater than 4:1.

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We report the histological and histochemical features of core fibers present in respiratory muscles obtained from autopsy since 1980. Fifty autopsy cases of adults without neuromuscular disease were examined. “Cental core” structure was present in 38 cases and absent in 12 cases. The duration of D. O. E. was greater than 1 month in 81.8% of core positive cases. In core negative cases, the duration of D. O. E. was less than 3 months. “Central core” structure were present in 80.0% of chronic respiratory failure cases and 41.7% of non-chronic cases.
It is suggested that central core in respiratory muscles develops less than 3 months after the onset of D. O. E.

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We evaluated the bronchial hyperresponsiveness to methacholine in the two cases of eosinophilic pneumonia with infiltration of eosinophils into bronchial mucosa. Bronchial responsiveness was not increased in either case in spite of marked infiltration of eosinophils into the bronchial mucosa and submucosa. Hypodense eosinophils are reported in the sputum of patients with bronchial asthma. This suggests that infiltrations of activated eosinophils into the bronchial mucosa is an essential factor in bronchial hyperresponsiveness.

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The changes in pulmonary epithelial permeability during and following radiation therapy were studied in 10 patients with malignant diseases of the chest; 9 patients with bronchogenic carcinoma and one with thymoma. ^99mTc-DTPA aerosol was inhaled during tidal breathing with the patient in supine position, and radioactivity was measured anteriorly by a gamma camera and recorded on a computer. Half time clearance (t_1/2) was calculated from exponential fitting of time activity curves by regression analysis in various regions of interest in the initial 7min following completion of aerosol inhalation. Studies were made every two weeks.
In patients who developed radiation pneumonitis, t_1/2 values decreased and reached the nadir at the time of manifest pneumonitis, indicating increased pulmonary epithelial permeability. Increased pulmonary epithelial permeability was observed not only in the pneumonic regions but also in the contralateral normal lung regions. Steroid therapy reversed these changes. Increased pulmonary epithelial permeability was observed in 2 out of 5 patients who did not develop radiation pneumonitis.
In summary, pulmonary epithelial permeability changes occur not only in regions of radiation pneumonitis but also in non-irradiated lung regions following radiation therapy. We consider that the judicious use of this method enables detection of changes in pulmonary epithelial permeability prior to the development of clinical manifestations of radiation pneumonitis.

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A 50-year-old woman with occupational asthma, whose attacks were provoked by inhalation of Arrowhead scale (Unapsis Yanonensis Kuwana) attached to the leaves of mandarin oranges is reported. She experienced asthmatic attacks while picking leaves and harvesting mandarin oranges. Because Arrowhead scale-dust stuck to the leaves was suspected to be the allergen, tests of allergy were performed using an extract of the allergen prepared by Unger's method in our laboratory. Asthmatic attack was provoked 90 minutes after inhalation of the extract of cocoon. Histamine was not released, but leukotriene D4 production was induced by the addition of the extract to whole blood. Basophils were activated by addition of anti-IgG anti-sera as well as the extract. These data indicate that LT released from target cells in response to the worm elements, as well as histamine, is important as a chemical mediator.

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In the present study, we investigated the effect of α1 protease inhibitor (α1PI) on the production of interleukin-1α (IL-1α, ELISA), IL-1β (ELISA), and tumor necrosis factor α (TNFα, ELISA) by alveolar macrophages (AM) recruited by broncholaveolar lavage (BAL) from patients with lung cancer and benign pulmonary diseases. Levels of BALF α1PI were significantly increased in patients with lung cancer compared with benign pulmonary diseases. When BALF AM were cultured and stimulated by KLPS (20μg/ml) for 48 hours, production of IL-1α and IL-1β was less marked in patients with lung production showed a similar tendency, but the difference between patients with lung cancer and benign pulmonary diseases was not significant. Addition of α1PI to the LPS-stimulated AM culture system inhibited production of these cytokines dose-dependently.
Thus, the local increase of α1PI in lung cancer may act as a humoral inhibitor against the production of IL-1 and TNF by AM.

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We assessed the period of administration of antibiotics required for cases of mycoplasmal pneumonia. The subjects were 38 patients with mycoplasmal pneumonia admitted to our hospital. These patients were treated with 100mg minocycline or 500mg erythromycin by intravenous infusion twice a day. They were divided into a 6 day-administration group (Group A; 16 cases) and a 9 day-administration group (Group B; 17 cases). Administration was discontinued on the 4th day or earlier in 5 cases due to side effects. A comparative assessment was made between Groups A and B with respect to body temperature, WBC, erythrocyte sedimentation rate, CRP, and chest X-ray on the 3rd, 6th, and 9th days of treatment, but no significant difference was observed. Residual shadows at the end of treatment were present in 100% of Group A and in 47% of Group B, but they disappeared gradually in both groups. No cases of recurrence were observed in either Group A or B within 1 month after the completion of treatment.
Regarding the treatment period for mycoplasmal pneumonia by intravenous infusion of minocycline or erythromycin, no significant clinical difference was observed between the 6 day-administration group and the 9 day-administration group, suggesting that 6 days of administration is sufficient for treatment.

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We clinically investigated 40 cases of respiratory infection due to B. catarrhalis by transtracheal aspiration (TTA). The cases consisted of acute bronchitis (13 cases), pneumonia (14 cases), and chronic lower respiratory tract infection (13 cases). The infection was monomicrobial in 17 cases and polymicrobial in 23 cases with respect to the organisms isolated from TTA. The principal organisms isolated in combination with B. catarrhalis in cases of polymicrobial infection were S. pneumoniae and H. influezae. All of the 6 cases of death were patients with pneumonia who had malnutrition and immunological hypofunction.
These results suggest that B. catarrhalis is an important pathogen in respiratory infection.

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A case of lymphangiomyomatosis (LAM) in a 35-year-old woman is reported. Because she was already severely dyspneic when she was admitted to our hospital, neither TBLB nor open lung biopsy was performed, and no accurate diagnosis was made. She died of respiratory failure three years after the development of exertional dyspnea, and autopsy revealed LAM. Pneumothorax, a well-known and frequent complication of LAM, did not occur until one month prior to her death. Although peeumothorax frequently complicates LAM, there are a few reports of cases in which pneumothorax did not occur during the course of the disease. We emphasize that LAM should be considered in the differential diagnosis of diffuse interstitial lung disease, even when the patient has no pneumothorax.
The retroperitoneal tumor (15×10×10cm), which was evident clinically, was later shown to be intraabdominal involvement of LAM. Although only a few cases of LAM with a large retroperitoneal tumor have been reported, minor intra-abdominal involvement is relatively common. This case also suggested that the finding of a retroperitoneal tumor may be a diagnostic clue in LAM.

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A 78-year-old man was admitted to hospital because of episodes of high grade fever and multiple nodular shadows on chest roentgenogram. He had a past history of percutaneous drainage and partial resection of the left lobe of the liver for liver abscess of unknown origin in 1987. The high grade fever was secondary to sepsis due to Citrobacter freundii. The sepsis improved with antibiotic therapy, but the abnormal shadows on chest roentgenogram did not improved. Immunoserological tests indicated a probable diagnosis of alveolar hydatid disease of the lung, which is very rare in the Kansai district of Japan. Open lung biopsy was performed and the diagnosis of alveolar hydatid disease of the lung was confirmed.

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A 57-year-old woman was admitted to our hospital for the evaluation of roentgenological micronodular shadows of the bilateral middle and lower lung fields. Physical findings were normal except for left axillary lymph adenopathy. The level of tumor marker NCC-ST-439 was markedly elevated (2800U/ml). Histological examination of the transbronchial biopsy and the swollen lymph node biopsy specimens showed proliferation of identical tumor cells. Breast or lung carcinoma was suspected as the primary site. Echogram of the left breast showed microcalcification without a mass. The biopsy specimen obtained from the calcified lesion indicated occult breast carcinoma. From these findings the diffuse micronodular shadows on the chest roentgenogram were considered to be the metastatic lesions of occult breast carcinoma.
This case indicates that occult breast carcinoma should be considered as a possible primary site for diffuse pulmonary metastatic lesions.

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A 44-year-old woman was admitted to hospital with a history of high fever, cough, and weight loss. Her chest X-ray film showed a giant solitary pulmonary mass in the left lower lobe. After admission, she developed bilateral eyeache and left exophthalmos. CT film of the head showed a massive lesion in the sinuses. Biopsy specimens obtained from both massive lesions showed either epithelioid granulomas with Langhans' giant cells or necrotising vasculitis. Antineutrophil cytoplasmic antibodies, which have high sensitivity and specificity for active WG, were found. The diagnosis of WG was thus made.

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We describe a 58-year-old female with BOOP associated with polymyositis. Four months prior to the appearance of distinctive manifestation of polymyositis, she presented with a two-week history of cough, dyspnea on exertion, and fever. Chest roentgenogram demonstrated bilateral basal infiltrative shadows. The patient was treated with prednisolone, 30mg/day because of progressive hypoxemia. Open lung biopsy revealed organizing masses of granulation tissue extending from the respiratory bronchioles into the intra-alveolar spaces, which was consistent with BOOP. She developed muscle pain in her legs, fever, dry cough, hypoxemia, and high CPK value in the course of tapering of steroid dose. The findings of biopsy from the left rectus femoris muscle were compatible with polymyositis. Retrospective study of the patient's serum on admission showed positive anti Jo-1 antibody.

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We report a 39-year-old male with pulmonary alveolar proteinosis. Although no abnormal shadows were seen on regular check-up chest roentgenogram one year previously, diffuse alveolar filling shadows were noted on admission, suggesting fairly acute progression of the disease. He was treated with bronchoalveolar lavage of each segment of both lungs by flexible fiberoptic bronchoscope under local anesthesia. Following this treatment, the shadows on X-ray film and shortness of breath resolved. Oxygen tension of arterial blood, pulmonary function, and serum CEA recovered to almost within normal ranges. The shadows deteriorated twice, but therapeutic lavage performed at the outpatient clinic was effective on each occasion. He thus received 3 series of bronchoalveolar lavage over a period of more than 3 years. We followed the clinical course of this patient for 6 years, and he remained well 3 years after the final treatment.

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A 65-year-old female suffering from lumbago, headache, and hypertension had been treated with nonsteroidal anti-inflammatory drugs (NSAIDs) and antihypertensive drugs. On June 13, 1990, 2 weeks after the commencement of loxoprofen administration, she developed cough and low grade fever. She was treated with antibiotics and NSAIDs without improvement. Laboratory data showed marked eosinophilia (2200/mm³), elevation of IgE (3090IU/ml), and liver dysfunction. Her chest X-ray revealed no active lesion, but the percentage of eosinophils in BALF was elevated (38%). Because drug-induced eosinophilic pneumonia was suspected, all drugs were discontinued. Her symptoms improved and the abnormalities of laboratory data normalized. The lymphocyte stimulation test was weakly positive with three NSAIDs (loxoprofen, pranoprofen, and alminoprofen). The challenge test by loxoprofen reproduced eosinophilia and liver dysfunction, suggesting that she had loxoprofen-induced eosinophilic pneumonia. To our knowledge, this is the first reported case of loxoprofen-induced lung injury.

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A 19-year-old psychologically disturbed female was admitted to our hospital because of intractable dyspnea and wheezing for twenty two months. She had been diagnosed as having asthma and had received several medications including steroids before admission to our hospital.
According to our clinical observations, she was considered not to have asthma because neither FEV_1.0 nor PaO₂ was decreased during the periods of wheezing and FEV_1.0 was not changed by inhalation of bronchodilators, although she had a family history of allergy and false bronchial hyperreactivity by asthograph.
On physical examination, diffuse wheezing was heard mainly over the larynx during inspiration at the times of dyspnea. There was flattening of the inspiratory flow-volume loop during wheezing. Bronchoscopy performed during an attack confirmed that wheezing was due to adduction of the vocal cords throughout the respiratory cycle.
Therefore, this case was diagnosed as vocal cord dysfunction. Her symptom was considered to be a form of conversion reaction derived from her unhappy past history.
Following psychosomatic therapy, all of her medications became unnecessary. She understood the mind-body relationship of her condition, and learned to achieve self-control.

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A 23-year-old male with complete collapse of the right lung due to spontaneous pneumothorax was admitted 11 days after its onset. Paying close attention to the re-expansion pulmonary edema (REPE), water seal drainage was performed. Following couple episodes of persistent severe cough, four hours later, he developed dyspnea and began to expectorate frothy massive sputum. Chest X-ray revealed pulmonary edema of the entire right lung field. Measurement of total proteins and neutrophil elastase in airway exudates showed 5.5g/dl (ratio to plasma, 0.89) and 7000μg/l, respectively. Because of marked difference of compliance between bilateral lungs, management with right and left-separated mechanical ventilation and PEEP applied only to the right lung was performed. Although transient mediastinal deviation to the left was observed, successful management was achieved by the maneuver.
High concentrations of total proteins and neutrophil elastase in edema fluid suggest that increased vascular permeability due to endothelial cell injury via activated neutrophils is mainly responsible for REPE. In the present case, rapid expansion of the collapsed lung accelerated by severe cough seems to be a predisposing factor of REPE. In patient with prolonged pneumothorax, suppression of cough is thought to be important for the prevention of REPE even with water seal drainage.

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A 67-year-old female was admitted to our hospital because of fever, dry cough, and exertional dyspnea. The findings of chest X-ray, transbronchial lung biopsy, and bronchoalveolar lavage were compatible with the diagnosis of idiopathic interstitial pneumonia. Prednisolone was administered and she felt better for a while. However, she developed severe dyspnea, and marked diffuse infiltrative shadows were observed on chest X-ray after 3 months of steroid therapy. In spite of pulse therapy with methylprednisolone, she died of severe respiratory failure. Complement fixation test and IgG antibody enzyme immunoassay for cytomegalovirus were positive, but there was no change the titers between admission and death. IgM antibody was negative. The lung findings at autopsy compatible with usual interstitial pneumonia and diffuse alveolar damage, moreover, cytomegalovirus infection was observed. We consider that recurrent cytomegalovirus pneumonia had been present due to secondary immunodeficiency caused by administration of steroid hormones.

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A 69-year-old male with bronchial asthma was admitted to a hospital with fever, dyspnea, and productive cough. Arterial blood gas analysis revealed sever hypoxemia (PaO₂ 54.8torr, PaCO₂ 28.8torr). Chest roentgenogram showed diffuse reticulonodular shadows predominantly in the upper filed and a small amount of bilateral pleural effusion. CT image of the lung showed nodular opacities at the peripheral branches of the pulmonary arteries and bronchi, some of which had become confluent. The bronchoarterial bundle had become thicker compared with a CT taken 18 months before this admission. Three days treatment with antibiotics and gamma globulin did not change the symptoms or radiologic findings.
After commencing methylprednisolone therapy, the pneumonia showed rapid improvement. Based upon the significant elevation of serum influenza B (B/Singapore/79) virus antibody titer, the patient was diagnosed as having influenza B viral bronchopneumonia. Twenty-three days after initiation of steroid therapy, slight nodular opacities were observed on CT. This finding suggests that bronchiolitis has a relatively prolonged course in influenza viral bronchopneumonia.

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Two sisters with α₁-antitrypsin deficiency and bronchiectasis are reported.
The 53-year-old older sister (propositus) had pneumonia 3 times during her forties. She developed dyspnea on exertion in February, 1988, and a few months later she was seen at our hospital. Her serum α₁-antitrypsin level was 11mg/dl. Vascular markings on chest X-ray were mildly decreased. Bronchography showed generalized cystic bronchiectasis.
The younger sister was seen at our hospital at the age of 50. She had been in good health until one year previously when she had developed pneumonia, and thereafter she had suffered from productive cough and dyspnea on exertion. Her α₁-antitrypsin level was 22mg/dl. Chest X-ray showed ring-like shadows and tram lines. Chest CT scans of both sisters revealed cystic changes, dilatation of bronchi, and the connection of these lesions diffusely.
The α₁-antitrypsin phenotype of these sisters was found to be PiSiiyama (homozygote). Family study revealed that α₁-antitrypsin levels of 7 members were intermediate and no members had symptoms.
We consider that bronchiectasis may have been related to α₁-antitrypsin deficiency in these sisters.

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A 35-year-old man had been exposed to the vapor of dimethyl sulfate which was split over the bed of a truck from an accidentally broken container. After receiving treatment at the critical care unit of our university hospital for respiratory failure, he was transferred to our ward with dyspnea persisting for one month. Chest X-ray showed diffuse nodular and infiltrative shadows and shadows suggestive of bronchial wall thickening. Bronchoscopy demonstrated tracheobronchial stenosis due to severe inflammation, and transbronchial lung biopsy revealed erosion and granulation in tissues of the bronchiolar walls. These findings suggested extensive inflammatory involvement of the respiratory tract extending from the trachea to the bronchioles. Treatment with antibiotics, especially agents with antipseudomonal activity, was effective against secondary pneumonia. Administration of corticosteroid for stenotic lesions of the airway was extremely beneficial.
The clinical course of the respiratory lesion caused by dimethyl sulfate intoxication was following, including the bronchoscopic findings. The efficacy of treatment with corticosteroid and antibiotics was evaluated by successive bronchoscopic examinations of the lesions.

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We describe a case of hCG-producing large cell carcinoma of the lung in a 73-year-old man who had gynecomastia associated with high serum concentration of hCG. Serum hCG levels fluctuated in parallel with the response of the cancer to surgery, chemotherapy, and radiotherapy.
The patient was admitted to our hospital with a huge mass shadow in the right lung filed on chest X-ray film on July 31, 1989. Physical examination revealed bilateral gynecomastia. Serum hCG and beta-hCG were 1108.0mIU/ml (Normal<2.0) and 31.9ng/ml (Normal<1.0), respectively. Clinical staging was T2N1M0, determined by radioisotope scanning of bone, and CT scans of the chest, brain, and upper abdomen. Right upper and middle lobectomy with mediastinal lymph node dissection was performed on August 18, 1989. The tumor, 6×6×8cm in size, was located in the middle lobe and was histologically comfirmed to be large cell carcinoma of the lung. A few of small nodules found on the surface of the middle lobe at thoracotomy were histologically proved to be pleural dissemination. Metastatic involvement was present in the hilar and mediastinal lymph nodes. The pathological stage was concluded to be T4N2M0. Immunohistochemistry showed positive staining reaction for hCG within some of the tumor cells. Three weeks after the operation, serum hCG had decreased rapidly but did not reach the normal range. Two courses of CDDP, VDS, and MMC were given at four week intervals. Following chemotherapy, serum hCG decreased to the normal range. He was discharged from our hospital on November 29, 1989. Serum hCG remained within normal limits during follow-up at our outpatient clinic. On February 14, 1990, serum hCG and beta-hCG increased to 105.0mIU/ml and 1.8ng/ml, respectively. On February 20, he was readmitted because of nausea and weakness. A CT scan of the brain revealed multiple space-occupying lesions suggesting metastasis. Radioistope bone scanning and CT scans of the chest and upper abdomen showed no abnormalities. Whole brain irradiation (40Gy) was performed from February 26 to March 29. Serum hCG decreased temporarily but soon increased. He died on June 26, 1990.
Serum hCG levels appear to be a useful parameter for the evaluation of treatment and the prediction of prognosis.

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