Endoscopic endonasal excision of nasal chondromesenchymal hamartoma with intracranial extension.

INTRODUCTION: Nasal chondromesenchymal hamartoma (NCMH) is an extremely rare benign hamartoma of the sinonasal tract, predominantly involving infants and young children. METHODS: We report the case of a 3-year-old boy of NCMH with extension to anterior skull base. RESULTS: The tumor was completely resected piece by piece via an endonasal endoscopic approach. There is no recurrence 3 years after operation. CONCLUSIONS: We reported the case of NCMH extending to skull base was successfully resected by endonasal endoscopic approach.

Dyskeratosis congenita showing usual interstitial pneumonia.

A 46-year-old man was admitted to our hospital with cough and dyspnea on exertion. A chest X-ray film showed diffuse interstitial shadows. He had hyperpigmentation forming a network pattern around his neck and dystrophy of the fingernails and toenails, and was diagnosed as having dyskeratosis congenita. Histological examination of the lung specimen obtained from the left S4b by open lung biopsy revealed usual interstitial pneumonia pattern with neither asbestos bodies nor silicotic nodules. Taken together with previously published findings, pulmonary involvement is considered to be an important complication of dyskeratosis congenita.

Weekly low-dose methotrexate therapy for sarcoidosis.

Low-dose methotrexate therapy has been used to treat a variety of chronic inflammatory diseases; a few studies have discussed the efficacy of this therapy for sarcoidosis. We present a patient with symptomatic sarcoidosis who was successfully treated with low-dose methotrexate after being refractory to steroids. This patient showed significant clinical improvement without development of any severe side effects. These findings suggest that low-dose methotrexate therapy may be a useful alternative treatment for sarcoidosis.

[Pulmonary tuberculosis with diabetes mellitus, presenting multiple nodular shadows].

An asymptomatic 52-year-old male, who was diagnosed as poorly controlled diabetes mellitus (DM), was referred to our hospital because of abnormal shadows on chest X-rays. Physical examinations and laboratory data showed no abnormal findings except increased values of blood sugar and HbA1c (12.1%). Chest X-ray and CT scan revealed multiple nodular shadows in both lung fields. The specimens from transbronchial biopsy showed epithelioid granuloma, and Mycobacterium tuberculosis was confirmed with bronchial wash fluid. The patient was then treated with antimycobacterial drugs and sulfonylurea along with diet therapy. The multiple nodular shadows gradually disappeared. It seemed that impaired host defense mechanisms caused by poorly controlled DM is responsible for the bronchogenic dissemination of M. tuberculosis, resulting in the extension into the multiple nodular shadows. Thus, a good control of DM is important for the prevention of an onset and excerbation of tuberculosis in patients with DM.

[Aspergillosis with non-cavitary lung cancer].

Growth of Aspergillus was observed in the necrotic tissue of non-cavitary lung cancer. Case 1 was a 60-year-old male complaining of cough and fever and was found to have left lower lobe atelectasis on chest X-ray. Bronchoscopic examination showed the yellow soft lesion occluding the left upper and lower lobe bronchus. Specimen from the lesion demonstrated adenocarcinoma and hyphae of Aspergillus in the necrotic tumor. Case 2 was a 53-year-old male who was found to have a left upper lobe mass by chest X-ray screening. Bronchoscopic examination showed the yellow polypoid lesion occluding the left upper division bronchus. Pneumonectomy was done and resected specimen of the lesion demonstrated adenocarcinoma and the hyphae of Aspergillus growing in both necrotic tissue and cancer tissue. As both patients had hyphae of Aspergillus in the necrotic tumor, it is considered that the presence of necrosis may cause the growth of Aspergillus.

[A case of tracheobronchomegaly].

We report a rare case of tracheobronchomegaly with crescent-type tracheobronchomalacia. A 77-year-old man with a chronic cough was referred to our hospital because of fever and dyspnea. Radiographic examination showed enlargement of the trachea and main bronchi. On chest radiography, the transverse diameter of the trachea was 31 mm, and consolidation shadows were seen in both upper lung fields. Tracheobronchomegaly with pneumonia was diagnosed. The pneumonia was improved by administration of PAPM/BP. On bronchoscopic examination, the trachea and main bronchi were extremely dilated on inspiration, and were collapsed on expiration. The biopsy specimen from the bronchial mucosa showed non-specific chronic inflammation.

[Compensatory growth of residual lung after pneumonectomy in childhood].

Though pneumonectomy proved to be a potent stimulus to compensatory growth in the animal model, the literature on this subject in human patients is sparse. We report a rare case of compensatory growth after pneumonectomy in childhood. A 17-year-old man was admitted to our hospital because of chest pain. He had been capable of normal exercise despite a history of pneumonectomy at the age of 4. Chest radiography showed pneumothorax and a displaced heart. In a chest CT scan, both pleural spaces were filled with the enlarged left lung. Since thoracoscopic examination showed a bulla at the surface of S3, bullectomy was performed. The specimen resected from the lung showed slight dilatation of the alveoli. It is considered that the enlargement of the residual lung had occurred as a result of alveolar multiplication rather than by dilatation of the existing alveoli. This case demonstrates lung regeneration in childhood.

[Analysis of T cell receptor V beta gene expression in bronchoalveolar lavage fluid lymphocytes from patients with idiopathic interstitial pneumonia].

T lymphocytes in bronchoalveolar lavage (BAL) from idiopathic interstitial pneumonia (IIP) patients are considered to recognize unknown antigens, such as dust, fume, virus or degenerated autoantigens. To analyse the nature of these T lymphocytes, we investigated T cell receptor (TCR) V beta gene usage (22 kinds) in BAL Lymphocytes and Peripheral blood lymphocytes from 10 11P patients and 9 normal controls, using reverse transcriptase-polymerase chain reaction method. In BAL lymphocytes, predominant usage of V beta genes (> 15% of the sum of all V beta transcripts) was recognized in 7 of 10 IIP patients, which appeared to vary in individuals (Case 1: V beta 14, case 2: V beta 3, V beta 5.1, case 5: V beta 2, case 6: V beta 6, case 7: V beta 8, case 8: V beta 7, V beta 20, case 9: V beta 6), whereas no predominant V beta usage was demonstrated in normal controls. It remains to be elucidated whether the BAL lymphocytes expressing predominant V beta genes are involved in the activation of alveolar macrophages, fibroblasts, thereby inducing the production of autoantibodies.

[Lung cancer obscured by aspergillus hyphae].

A 66-year-old man was referred to our hospital because of cough. Chest X-ray films showed complete atelectasis of the left lung. Serum CYFRA was elevated. Bronchoscopic examination disclosed a white polypoid lesion occluding the left main bronchus. A biopsy specimen from the lesion revealed numerous aspergillus hyphae. Oral itraconazole (100 mg) and weekly endobronchial instillation of fluconazole were administered. Three months later, on the 12th bronchoscopic examination, the tumor occluding the left main bronchus was detected after the removal of aspergillus, and the biopsy findings yielded a diagnosis of squamous cell carcinoma. Aspergillosis complicated by lung cancer without cavity formation is very rare, and compounded the difficulty of diagnosing lung cancer in this case.

[Pyothorax associated lymphoma treated by chemotherapy after thoracostomy].

A 62-year-old man with a medical history that included artificial pneumothorax therapy at the age of 18 was admitted to our hospital because of persistent cough. Chest computed tomographic scans disclosed atelectasis in the right lung and pyothorax surrounded by calcifications. Radiographic examination failed to disclose any tumors. After admission, high grade fever developed due to aggravated pyothorax infection. Because antibiotic therapy and drainage failed, open window thoracostomy was performed. Tumors were found along the wall of the pyothorax cavity, and examination of resected specimens yielded a diagnosis of non-Hodgkin's lymphoma, diffuse large cell type (B-cell lineage). It was difficult to close the pyothorax cavity due to infection and lymphoma. Therefore, with the thoracic window open, the patient was given combination chemotherapy including CHOP (6 courses) and DeVIC (7 courses). He died of disseminated intravascular coagulation 17 months after thoracostomy. In patients with pyothorax associated lymphoma, chemotherapy is sometimes difficult to perform because of persistent pyothorax infection. Although edema and ascites due to protein loss from the tumor complicated the treatment of our patient, we concluded that open window thoracostomy is effective in managing pyothorax prior to and during chemotherapy.

[A case of myxedema heart with serial endomyocardial biopsy].

We report a case of middle-aged woman with myxedema heart who presented congestive heart failure due to myocardial damage caused by myxedema, and showed reversible and irreversible myocardial change proven by serial endomyocardial biopsy. She was admitted to our hospital because of facial, peripheral edema and dyspnea. On admission, chest X-P revealed severe cardiomegaly (CTR 70%) and bilateral pleural effusion. Electrocardiogram showed low voltage. Echocardiogram and chest CT revealed a large amount of pericardial effusion. No significant stenosis was observed on a coronary arteriogram. Laboratory data showed elevated TC, CPK, LDH values and immunological test indicated high titer of thyroid test and microsome test. Thyroid function test revealed primary hypothyroidism with low T3, low T4 and high TSH levels. Replacement therapy for hypothyroidism in addition to digitalis and diuretics++ has been started. Seven months after initial evaluation, this therapy has resulted in dramatic clinical improvement. Transvenous right ventricular endomyocardial biopsy demonstrated vacuolated degeneration on admission and improvement of vacuolated degeneration with a slight degree of fibrosis after therapy. This pathological finding suggests that myxedema heart is able to produce both reversible and irreversible myocardial damage.

[T cell receptor heterodimer (alpha beta/gamma delta) expressions in diffuse panbronchiolitis].

Several reports have indicated that T cells bearing gamma delta T cell receptor (TCR) are major effector cells in mucosal immunity of the murine intestinal tract. In humans, abnormal distribution of gamma delta + T cells has also been found in inflammatory bowel diseases. We studied TCR heterodimer expressions in lymphocytes infiltrating the peripheral airways in patients with diffuse panbronchiolitis (DPB), because DPB is histologically characterized by chronic inflammation in bronchioles and could be a model for investigation of the significance of gamma delta + T cells in persistent inflammation of the human respiratory tract. Open lung biopsy specimens were obtained from 7 patients and peripheral blood lymphocytes (PBL) from 9 patients. Immunohistochemically, we detected alpha beta and gamma delta TCR by avidin-biotin complex immunoperoxidase method, utilizing BMA 031 and TCR delta 1 monoclonal antibody, respectively, PBL were analyzed by flow cytometry. Histological analysis revealed that DPB lesions mainly involved the bronchiolar walls with marked infiltration of small round cells. Many T cells were distributed in the submucosal area, although few T cells infiltrated the epithelium. Most of the T cells in the bronchioles expressed alpha beta TCR (98.7%), whereas only 1.3% expressed gamma delta TCR. There was no significant difference in the percentage of gamma delta + T cells in PBL between DPB and age- and sex-matched controls (6.7% vs. 9.1%). These results suggest that the increase of T cells bearing alpha beta TCR played a major role. However, the possibility that the decrease of T cells bearing gamma delta TCR caused reduced mucosal defense against external antigens, leading to persistent inflammation of the lower respiratory tracts in DPB, is not excluded.

[Nasopharyngeal myiasis during mechanical ventilation].

We report a case of myiasis caused by Phaenicia sericata during mechanical ventilation. An 86-year-old woman with bronchiectasis was admitted to our hospital with severe respiratory failure. Treatment with mechanical ventilation and sedatives was initiated. On the 10th day of hospitalization, about 20 white larvae were found in the patient's oral or nasal cavities. The larvae were removed and identified as Phaenicia sericata. No mucosal injury was found in the patient's oral or nasal cavity by endoscopic examination. The patient died of multiple organ failure caused by sepsis that had no association with myiasis. From the clinical course and the fly's life cycle, it is considered that the fly laid eggs in the patient's oral or nasal cavity while she was sedated during mechanical ventilation. Myiasis can occur even in a hospital.

[Restricted T cell receptor V beta gene expression in bronchoalveolar lavage lymphocytes of patients with sarcoidosis].

T lymphocytes in bronchoalveolar lavage (BAL) fluid from patients with sarcoidosis are activated. They are thought to recognize as yet unknown antigens and to play an important role in the pathogenesis of the disease. We studied the use of the T cell receptor V beta gene of lymphocytes obtained by BAL and lymphocytes obtained from peripheral blood of 11 patients with sarcoidosis and 9 normal controls, using the reverse transcriptase-polymerase chain reaction. As compared to the normal controls, V beta 2 and V beta 6 genes were predominantly expressed (> 15% of the sum of all V beta transcripts) on lymphocytes obtained from BAL fluid in 4 and 7 of 11 patients with sarcoidosis, respectively, but no specific V beta gene was predominantly expressed on lymphocytes obtained from peripheral blood. These results imply that those lymphocytes that are obtained from BAL fluid and that express V beta 2 and V beta 6 genes are involved in the pathogenesis of sarcoidosis.

Green tea-induced asthma: relationship between immunological reactivity, specific and non-specific bronchial responsiveness.

BACKGROUND: The relationships between immunological reactivity and bronchial responsiveness to allergen and non-specific bronchial responsiveness are unclear in occupational asthma caused by low molecular weight substances. OBJECTIVE: We assessed the above relationships in green tea-induced asthma, an occupational asthma of green tea factory workers, in which epigallocatechin gallate (EGCg), a low molecular weight component of green tea leaves, is the causative agent. METHODS: Subjects consisted of 21 patients suspected of having green tea-induced asthma, on whom skin test and inhalation challenge with EGCg were performed. The skin sensitivity or end-point titration to EGCg as a measure of immunological reactivity, together with the provocative concentrations causing a 20% or greater fall in forced expiratory volume in 1 s (PC20) of EGCg and methacholine, were determined. RESULTS: We found that 11 patients had green tea-induced asthma, with immediate asthmatic reactions in eight and dual asthmatic reactions in three. We also found that 11 of 13 patients (85%) with immunological reactivity and bronchial hyper-responsiveness to methacholine experienced an asthmatic reaction and that no subject without immunological reactivity reacted. There were significant correlations among skin sensitivity, EGCg PC20 and methacholine PC20. Multiple linear regression analysis showed the relationship: log (EGCg PC20)=0.42 log (skin sensitivity)+1.17 log (methacholine PC20)+0.93 (r=0.796, P<0.05). CONCLUSION: It is concluded that bronchial responsiveness to EGCg can be highly satisfactorily predicted by skin sensitivity to EGCg and bronchial responsiveness to methacholine.

[Acute exacerbation following bronchoalveolar lavage in idiopathic interstitial pneumonia].

We report two cases of idiopathic interstitial pneumonia (IIP) with acute deterioration after bronchoalveolar lavage (BAL). Case 1 was a 54-year-old woman, and case 2 was a 75-year-old man. Both were diagnosed as having IIP, and hospitalized with complaints of high fever and dyspnea. After BAL, the degree of dyspnea increased. White blood cell count and lactate dehydrogenase were elevated, and PaO2 was decreased. Chest X-ray revealed consolidation of the upper lung fields and reticulo-granular shadows spreading through both lungs. BAL fluid examination showed an elevated neutrophil fraction (case 1: 6.5%, case 2: 35.2%), suggesting respiratory tract infection although bacteria could not be detected. Case 1 died of respiratory failure despite corticosteroid therapy. Autopsy revealed diffuse alveolar damage with focal pneumonia in the right S6 corresponding to the upper lobe consolidation. Case 2 improved after antibiotic therapy. These findings suggest that aspiration of infected fluid during BAL can cause acute exacerbation of IIP. It is important to recognize that the BAL procedure can induce an acute exacerbation of IIP.

Biased T cell receptor Vbeta gene expression in bronchoalveolar lavage fluid from Japanese patients with sarcoidosis.

OBJECTIVE: Sarcoidosis is believed to be one of the T cell-mediated granulomatous diseases with unknown aetiology. We attempt to search for the causative T cell clones of sarcoidosis. METHODS: We study T cell receptor beta-chain variable region (Vbeta) repertoire in peripheral blood (PB) and bronchoalveolar lavage fluid (BALF) from patients with sarcoidosis, using semi-quantitative reverse transcriptase-polymerase chain reaction method. The expression of 22 kinds of Vbeta genes is examined in 17 patients with sarcoidosis and nine normal subjects. RESULTS: Compared with control subjects, the group with sarcoidosis exhibits significantly high expressions of the Vbeta2 (P < 0.005, Wilcoxon's test) and Vbeta6 (P = 0.005) genes in BALF. In each BALF sample, the Vbeta2 (P < 0.01, chi2 test) and Vbeta6 (P < 0.01) genes were overexpressed (> 2 SD above the mean value for each Vbeta observed in control subjects) in 11 and 10 of 17 patients with sarcoidosis, respectively. Furthermore, the amino acid sequences of Vbeta6+ complementarity determining region 3 were conserved in one of three patients. There is, however, no disposition of Vbeta gene usage in PB from patients with sarcoidosis compared with control subjects. CONCLUSIONS: The T lymphocytes with Vbeta2 and/or Vbeta6 are associated with the pathogenesis of sarcoidosis. The possibility exists that these T lymphocytes might be capable of recognizing the restricted antigens, thereby inducing oligoclonal expansion.

[Two cases of intrapulmonary lymph node associated with either progressive systemic sclerosis or idiopathic pulmonary fibrosis].

We encountered 2 cases of intrapulmonary lymph node. Case 1 was in a 48-year-old woman who had been given a diagnosis of progressive systemic sclerosis. Case 2 was in a 49-year-old woman with idiopathic pulmonary fibrosis. In each patient, a follow-up chest CT scan showed a pulmonary nodule, which could not be seen on chest X-ray film. Each nodule has an irregular margin and a linear shadow resembling pleural indentation. Histological examination of the open biopsy specimens revealed that these nodules were intrapulmonary lymph nodes surrounded by interstitial pneumonia. Previous case reports and these 2 cases indicate that unless a thoracotomy is done intrapulmonary lymph nodes are difficult to distinguish from lung cancers.