Many defense mechanisms are located in the respiratory tract, since they are constantly exposed to a variety of pathogens from both inside and outside the body. Among the various defense mechanisms, the immune system is so potent that immunodeficiency results in proneness to infections and repeated and/or intractable infection.
It is not uncommon to encounter patients with congenital immunodeficiency in clinical practice because of recent advances in supportive therapy. This paper describes cases of primary immunodeficiency that developed respiratory infections, together with the results of investigations of their immune status. Doctors should suspect the possibility of immunodeficiency in patients with repeated or intractable respiratory infections.
Intractable respiratory infections, however, have been seen with increasing frequency in the elderly because of the increase in the elderly population. One reasons for this is the physiologic immunodeficiency associated with aging, which is characterised by decreased antigen-induced lymphocyte proliferation, IL-2 production, IL-2R expression, hsp 70mRNA production, and demethylation of T lymphocytes, and delayed degradation of c-myc mRNA.

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Aspiration pneumonia is the fourth cause of death in Japanese, of which 92% occurs in elderly people aged more than 65 years. Pneumonia in the elderly is mostly due to aspiration.
Aspiration is caused by disturbance of the pharyngeal reflex and cough mechanisms are due to disturbances of brain activity.
Respiratory diseases including bronchial asthma must be treated with consideration of brain activity.

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The relationship between histological type and immunohistological findings was studied in total 141 cases of resected lung cancer. Adenocarcinoma was cytologicaly subtyped according to the ultrastructural findings. Immunohistochemical staining was performed on paraffin-embeded tissue using the avidin-biotin-peroxidase complex method for carcinoembryonic antigen (CEA), keratin, secretory component (SC), neuron specific enolase (NSE), lysozyme (Ly) and lactoferrin (La).
Adenocarcinoma stained strongly positive with antibody against CEA and SC. There was no statistical difference among the different subtypes of adenocarcinoma, but in the cases of clara cell type, CEA staining was less intense and in goblet cell type, the intensity of SC staining was greates. Goblet cell type characteristically stained positively with anti-Ly antibody, and Ly was a specific marker for differentiating adenocarcinoma of goblet cell type. La was positive not only in bronchial gland cell type, but also in other subtypes in adenocarcinoma. Squamous cell carcinoma showed more intense staining with anti-keratin antibody than other histological types. Small cell carcinoma extensively stained with anti-NSE antibody, but some of the other histological types also stained positively. NSE was a relatively good marker for small cell carcinoma but was not specific.
It is concluded that immunohistochemical examination is a useful method for differentiation of different histological types of lung cancer.

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Respiratory resistance and dynamic compliance in guinea pigs were measured by pulmonary mechanics analyzer Buxco Model 6. Changes induced by intravenous injection of histamine were studied.
Administration of histamine caused dose-dependent increase in respiratory resistance and decrease in dynamic compliance in guinea pigs. The increase in respiratory resistance by repeated injections of the same dose of histamine was maintained at the same level 5 hours after the first injection. However, the decrease in dynamic compliance by histamine administration decreased significantly from 1 to 4 hours after the first injection.
The baseline resistance increased from 1 to 5 hours after the first injection of histamine. The baseline compliance decreased from 1 to 5 hours after the first injection.

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Twenty-five patients who underwent upper sleeve lobectomy for lung cancer were studied by spirometric examination to evaluate postoperative pulmonary function, including Vital Capacity (VC), %Vital Capacity (%VC), Forced Expiratory Volume in one second (FEV_1.0), and Forced Expiratory Volume in one second as a percentage of the forced vital capacity (FEV_1.0%). The results of the patients with bronchoplasty were compared with those of patients with lobectomy alone and of patients with pneumonectomy. Lung function was periodically examined postoperatively following bronchoplasty.
VC, %VC and FEV_1.0 were decreased postoperatively in the patients with upper sleeve lobectomy, while FEV_1.0% was increased. There were no differences in postoperative %VC or FEV_1.0% between the patients with bronchoplasty and those with upper lobectomy alone. However, the postoperative %VC of the patients with pneumonectomy was significantly decreased compared with patients who underwent bronchoplasty or lobectomy alone. %VC in the patients with bronchoplasty was decreased at 3 months after operation, but it to gradually returned to the preoperative value by 13 to 24 months after operation.

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Idiopathic pulmonary fibrosis (IPF) is thought to develop through slowly progressing lung injury, in which fibrosis occurs as a result of abnormal repair processes. Lung injury in emphysema, in which the normal extracellular matrix is destroyed, is considered to occur mainly because of protease-antiprotease imbalance. In order to examine whether the pathogenesis of IPF involves the proteolytic mechanism of enzymes as in emphysema, concentrations of plasma neutrophil elastase and serum α₁-protease inhibitor were measured in patients with IPF, and compared with the levels in patients with emphysema and in normal individuals. In some patients with IPF, the blood concentration of neutrophil elastase was much higher than normal and the degree of imbalance between neutrophil elastase and α₁-protease inhibitor was significantly greates than in patients with emphysema.
In these patients, many years had passed since the onset of the disease, the number of leukocytes and neutrophils and the concentration of LDH in peripheral blood were significantly higher than normal, and the concentration of CEA-II was slightly increased. These data suggest that chronic, massive lung injury had occurred. The blood concentration of neutrophile elastase and α₁-protease inhibitor ratio may be useful in assessing the degree of lung injury.

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In order to longitudinally study the pulmonary function development of children with a history of asthma, we measured the flow-volume curves of 441 fourth-grade children in seven primary schools randomly selected from the Kashima district in Ibaraki prefecture in 1980, and again in 1982 and 1984. Levels and slopes of pulmonary function indices for individual children were calculated in 325 children who performed acceptable forced expiratory maneuvers at all of the three surveys.
In FVC and FEV1, no significant difference of levels and slopes was observed between children with asthmatic history and control children. However, the level and slope of V₂₅ of children with asthmatic history were significantly lower than those of control children (0.5l/sec for the level, 0.08l/sec for the slope) even when only those who did not suffer from wheezing attack during the follow-up period were concerned. These results suggested that the functional change of peripheral airways in children with asthmatic history did not recover within a couple of years even when they were in remission. Moreover, the functional differences between those two groups of children might become larger in early adolescence.

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The authors previously reported that liposomes, when injected intravenously, produce transitory pulmonary hypertension with increased secretions of thromboxane A₂ from activated intravascular macrophages that phagositize liposomes in sheep. In the present study, we attempted to determine whether such responses were modified by the lipid compositions of the liposomes.
Five different types of liposomes were prepared by reverse-phase evaporation. The liposomes used were composed of phosphatidylcholine (PC), cholesterol (CHOL), and either phosphatidylglycerol (PG-liposomes), phophatidylserine (PS-liposomes), phosphatidylethanolamine (PE-liposomes), stearlyamine (SA-liposomes) or none (PC-liposomes). The net charges of PG and PS-liposomes were negative, SA-liposomes were positive, PE- and PC-liposomes were neutral.
Each liposome was injected intravenously to obtain a pulmonary arterial pressure response. Arterial blood was sampled before and after liposome injections to measure thromboxane B₂ concentrations. All liposomes, but not PC-liposomes, produced pulmonary arterial hypertension associated with increased arterial thromboxane B2₂ concentrations, irrespective of the net surface charge of the liposome. PG and PS-liposomes, both of which were negatively charged, showed different dose-response curves, the two different types of neutral liposomes showed different responses, and PC-liposomes produced a small increase in pulmonary arterial pressure. PE-liposomes produced marked increases in the pulmonary arterial pressure.
From these results, the authors concluded that pulmonary arterial pressure responses to the liposomes are modified by the lipid compositions of the liposomes, and that this is not caused by the difference in the net charge of each liposome.

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In order to investigate the etiology of usual interstitial pneumonia (UIP) and UIP with lung cancer (LC), autopsy findings in 18 cases of UIP with LC and 11 cases of uncomplicated UIP were clinicopathologically compared with the environmental factors of smoking habits and occupation. UIP with LC was highly correlated with smoking, especially heavy smoking and with occupations in which dust is inhaled, such as electrical installation and ceramic production, indicating that these environmental factors are important background factors in the complication of UIP with LC.
Pathologic examination of cases of UIP with LC (6 squamous cell carcinomas, 5 small cell carcinomas, 4 adenocarcinomas, and 3 large cell carcinomas, 2 of which showed pulmonary double carcinoma revealing a slight correlation between fibrosis and primary site of LC and a slightly greater correlation of squamous cell carcinoma and small cell carcinoma to smoking habits and inhalation of dust. In terms of the correlation between UIP and LC among autopsy cases, the environmental factors proved to be more significant than the fibrotic findings. These environmental factors are thought to merit consideration as commonpredisposing factors in the development of LC and its complication with UIP.

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The relationship between the quantity of silver-binding nucleolar organizer regions (AgNORs), nuclear DNA content, and proliferative activity was studied in 61 patients with adenocarcinoma of the lung. The proliferative activity of adenocarcinoma was estimated by tumor volume doubling time based on chest X-ray findings.
There was a high, inverse correlation between the AgNORs and the tumor doubling time (p<0.001, r=-0.815), and the contribution rate was high value (²=0.664). However, the AgNORs value was an independent prognostic factor for survival time.
A better 5-year survival rate was observed in patients with DNA diploidy than in DNA aneuploidy, but there was no statistical difference between the two groups. There was an inverse correlation between the DNA index and tumor doubling time (p<0.05, r=-0.565), but the contribution rate had a low value (r²=0.319).
These results indicate that the AgNORs value is important in providing an estimate of the proliferative activity of adenocarcinoma.

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Paraganglioma is a tumor arising from the extra-adrenal paraganglion system and is rarely observed in the mediastinum. The authors experienced a case of nonfunctioning paraganglioma of the upper posterior mediastinum. The patient was a 40-year-old female in whom abnormal shadows were noted in chest X-ray films taken in March, 1989. She was examined at our hospital with a diagnosis of mediastinal tumor in June of the same year. CT of the chest revealed a tumor in the upper posterior mediastinum and it was shown to be hypervascular by a DSA study. Urinary hormone levels were normal. The tumor was resected in May, 1990. The tumor, measuring 6.8×4.0×3.5cm, was encapsulated, appeared dark red and had relatively clear borders. A histopathologic diagnosis of paraganglioma was made. The patient remains disease-free at present, 6 months after operation. Problems concerning diagnosis and treatment of mediastinal paraganglioma were evaluated with a review of cases reported in Japan.

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A 68-year-old woman had pulmonary aspergilloma in the right upper lobe with old cavitary pulmonary tuberculosis. Despite intravenous fluconazole there was no change in the size of the fungus ball. Endobronchial instillation of fluconazole also failed. Subsequently percutaneus instillation of fluconazole was attempted with localized drainage. The fungus ball decreased in size after 8 weeks. Although slight hemoptysis and subcutaneus emphysema occurred in this case, these condition required no treatment. This method of treatment appears to be useful for inoperable cases of pulmonary aspergilloma.

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An 18-year-old man was admitted to Hamamatsu University Hospital on February 15, 1985, with high fever, vesicular and papular rash involving the skin and mouth, conjunctivitis, productive cough and dyspnea. A diagnosis of Stevens-Johnson syndrome was made by skin biopsy, and chest X-ray showed an infiltrate in the right lower lung filed. Despite treatment with corticosteroids and antibiotics, the mucocutaneous lesions did not heal, and the pneumonia progressed to both lung fields. Because the patient had developed dyspnea, a tracheotomy was performed, mechanical ventilatory support was instituted, and high-dose corticosteroid therapy was started. However, jaundice due to intrahepatic cholestasis, hematuria, hematochezia, sepsis, and subcutaneous and mediastinal emphysema ensued, and the patient died of respiratory failure on March 1. Postmortem examination of the lung demonstrated diffuse alveolar damage. The complement-fixation titer for Mycoplasma was 1:64, compared with a level of less than 1:4 on admission. This case was thought to be one of fulminant Mycoplasma pneumoniae infection presenting with Stevens-Johnson syndrome, respiratory failure and other extra-pulmonary complications.

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A 63-year-old man with pulmonary sarcoidosis, diagnosed by mediastinal lymph node biopsy in 1977, was admitted in Feb. 1987 because of shortness of breath and cough. Chest X-ray showed bilateral hilar lymphadenopathy and a tumor shadow in the right lung field. Histological examination of specimens biopsied from the right lung revealed small cell carcinoma (S. C. C.). Bronchoalveolar lavage was performed to evaluate the disease activity of sarcoidosis, and the total number of cells and T-lymphocytes; the ratio of CD4⁺ cells to CD8⁺ cells was not increased. He was treated with combination chemotherapy, however, he died of respiratory failure after 7 months. An autopsy was performed, and the lesions were examined histologically. The sarcoid lesion in a lymph node obtained at autopsy was not active, in contrast to that obtained by mediastinal lymph node biopsy.
Lung cancer and sarcoidosis are both common diseases, but their coexistence in the same patient is not common, and autopsied cases are rare. In this case, an autopsy was performed, and BAL had been performed prior to his death. The relationship between the BAL findings and the histology of sarcoidosis was examined. Based on the results of autopsy and BAL, the sarcoidosis was inactive prior to death, but had been histologically active 10 years previously. Therefore, this is a very interesting case, since we can examine the relationship between the two diseases, and the progression of each disease. This case also provides an interesting example of differentiation of sarcoidosis from S. C. C. Metastatic invasion of the hilar lymph nodes without bronchial stenosis and changes secondary to stenosis may often occur in patients with small cell lung cancer. Such metastatic invasion closely resembles the bilateral hilar lymphadenopathy of sarcoidosis; therefore, in some cases, it may be extremely difficult to differentiate the two diseases.

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A 56-year-old man was admitted to our hospital with right chest pain. Chest X-ray, CT scan and MRI revealed a chest wall tumor and enlarged mediastinal lymph nodes. Percutaneous lung biopsy was performed, and the pathological diagnosis of pleomorphic rhabdomyosarcoma was obtained.
The only significant abnormal laboratory finding was elevation of serum NSE (24.5ng/ml).
Although chemotherapy (VAC-ADM) and radiation therapy were performed, the patient died about 7 months after admission.
To our knowledge, only 17 cases of chest wall rhabdomyosarcoma have been reported in Japan.

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A 33-year-old male was admitted to our hospital for further investigation of unilateral hyperlucency of the left upper and middle lung fields on chest X-ray. A pulmonary scan showed decreased perfusion of the left upper and middle lung fields. Pulmonary arteriography demonstrated small left pulmonary arteries with diminished peripheral vascular branches. Left bronchogram showed almost normal distribution of main and subsegmental bronchi, but poor filling by contrast in the peripheral bronchi. From these findings, the patient was diagnosed as having Swyer-James syndrome. In order to demonstrate the alveoli in this syndrome, selective alveolobronchography (SAB) was performed. SAB revealed coarse ring shadows of B¹⁺².

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We report a 43-year-old male with hypereosinophilic syndrome. The patient has had eosinophilia since the age of 25. He developed bronchial asthma at 41 years of age. In the following year, he consulted a gastroenterologist because of fever and abdominal discomfort. Upper gastrointestinal study showed duodenitis. At the age of 43 years, there was slight fever, watery diarrhea, and eosinophilia. Examination in our department disclosed hypereosinophilic syndrome associated with duodenitis, endomyocarditis with an intraventricular thrombus, and bronchial asthma caused by house dust. Both prednisolone and aspirin were administered with improvement of eosinophilia and partial thrombolysis. Assays of IL-3, GM-CSF, and IL-5 revealed increased activity of IL-5, GM-CSF, and IL-3 in this order. Especially IL-5 has by far the strongest activity in these cytokines. IL-5 may play an important role in the development of hypereosinophilic syndrome.

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A 33-year-old man was referred to our department because of left cervical lymphadenopathy for one month which did not respond to antibiotics. Histological examination of the lymph node biopsy revealed eosinophilic granuloma. However, repeated bronchoscopic examinations performed to evaluate the pulmonary lesions appearing on chest roentgenograms and chest CT scans revealed no evidence of eosinophilic granuloma. Steroid therapy resulted in resolution of the cervical lymphadenopathy, but the pulmonary lesions showed no response. The possible relationship between eosinophilic granuloma and pulmonary cystic lesions in our patient is discussed.

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We reported a case of Kearns-Sayre syndrome with impaired respiratory regulation. A 55-year-old male was admitted to our hospital complaining of chronic progressive external ophthalmoplegia, limb muscle weakness and dyspnea. On admission, because arterial blood gas analysis showed marked alveolar hypoventilation, ventilatory response was measured and diminished chemosensitivity to both hypoxia and hypercapnia was found. His vital capacity and forced expiratory volume in 1 second were slightly decreased, and a chest X-ray film revealed a moderate degree of elevation of the bilateral diaphragm. Therefore, we considered that the diminished response to hypoxia and hypercapnia in this case was caused by an impairment of the respiratory center, as well as chemoreceptors and also the presence of respiratory muscular weakness.

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A 76-year-old man, whose carotid bodies had been resected for treatment of bronchial asthma 40 years previously was admitted for evaluation of abnormal arterial blood gases and exertional dyspnea. The case was diagnosed clinically as chronic pulmonary emphysema. His peripheral chemoreceptor function, estimated by hypoxic ventilatory and P_0.1 response tests and withdrawal test was non-functioning. His PaCO₂ value tended to rise over 50 Torr either after light exercise or during airway infection, though it was normal at rest. In addition his dyspnea had continued for 40 years in spite of carotid body resection. It was concluded that the effect of carotid body resection lasts more than 40 years and it does not have a good effect on COPD.

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A 26-year-old woman with a history of recurrent spontaneous abortion was admitted because of superior vena cava syndrome. Chest computed tomography and digital subtraction angiography revealed obstruction of the superior vena cava by a thrombus. After admission she also developed oral aphthous ulceration, erythema nodosum, and positive cutaneous hypersensitivity reaction to intradermal injection of saline. Laboratory investigations revealed HLA-B51 and positive lupus anticoagulant. We made the diagnosis of Vasculo-Behçet's disease and treated her with heparin and urokinase with no clinical improvement. The fever subsided after prednisolone administration.
Two years later, superior vena cava syndrome had slightly improved, although RI venography showed complete obstruction of the superior vena cava with development of collateral circulation. Endoscopic examination showed downhill esophageal varices.
It is of note that the initial symptom in this case of Vasculo-Behçet's disease was superior vena cava syndrome, and that lupus anticoagulant may have contributed to the thrombus formation.

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A case of pulmonary varices in a 73-year-old female is presented. Routine chest X-ray revealed a mass in the right hilar region. CR tomogram showed a round, clearly defined mass at the right hilum. Computed tomography demonstrated marked enlargement of the proximal portion of the pulmonary vein at the entrance of the left atrium, which was suspected to be pulmonary varices. The diagnosis was confirmed by pulmonary angiography. During the arterial phase no abnormal findings were seen, but during the venous phase the veins of the right upper and middle lobes were found to be draining into the dilated pulmonary vein and then into the left atrium. Thus, the diagnosis of pulmonary varices was established. CT and angiography are the most useful methods for definitive diagnosis of pulmonary varices.

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