Polyarteritis nodosa and acute interstitial pneumonia.
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Acute Disease↗
Biomedical subjects
S Kira
Publications and source records attributed to S Kira.
Gene analysis of heterozygous protein C deficiency in a patient with pulmonary arterial thromboembolism.
A 16-yr-old male patient with heterozygous protein C deficiency developed acute pulmonary thromboembolism. The patient had low levels of plasma protein C antigen and activity (33 and 35% of normal, respectively). Analysis of the protein C gene by polymerase chain reaction (PCR) and direct sequencing revealed a nucleotide substitution (Arg169CGG----Trp169 TGG) in exon VII. This mutation is identical with protein C Tochigi, and the substituted amino acid is located at the cleavage site of the activation peptide of protein C. The mutant sequence was also detected in the mRNA transcripts of protein C gene. These results suggest that the possible mechanism of plasma protein C reduction is impaired stability or susceptibility to protein degradation during intracellular processing or after secretion into plasma. As this is a third independent case of protein C Tochigi with thromboembolism, the mutation of Arg169 (CGG) to Trp169 (TGG) in the protein C gene may be a "hot spot" and a common type of genetic lesion in congenital protein C deficiency with thromboembolic complications.
Adolescent↗
Evaluation of cor pulmonale on a modified short-axis section of the heart by magnetic resonance imaging.
Magnetic resonance imaging (MRI) of a modified short-axis section of the heart, in 36 patients with chronic pulmonary diseases, consisting of 19 patients with pulmonary hypertension (PH group; mean pulmonary arterial pressure > or = 20 mm Hg) and 17 patients without pulmonary hypertension (non-PH group) was evaluated to study the configuration of the right ventricle. Parameters of right ventricular hypertrophy, including right ventricular wall thickness (RVWT) and the ratio of RVWT to left ventricular posterior wall thickness (RVWT/LVPWT), with this method were significantly larger in the PH group than in the non-PH group (p < 0.01). RVWT and RVWT/LVPWT correlated well with mean pulmonary arterial pressure (r = 0.90, p < 0.001 and r = 0.89, p < 0.001), total pulmonary resistance (TPR; r = 0.88, p < 0.001 and r = 0.85, p < 0.001), and pulmonary arteriolar resistance (PAR; r = 0.83, p < 0.001 and r = 0.81, p < 0.001). This method of setting a patient in a supine position and slicing with single-oblique sections may seem overly simple compared with Dinsmore's double-oblique short-axis section of the heart, but it is more convenient in practice. These results suggest that a modified short-axis section of the heart by MRI provides valid clinical configurational information concerning the right ventricle on which to base a noninvasive diagnosis of cor pulmonale.
Adult↗
[Structures and functions of adhesion molecules--involvement of adhesion molecules in the pathogenesis].
Previous studies have revealed that adhesion molecules are involved in immune responses, such as the interaction between T cells and antigen presenting cells. Recent investigations demonstrated that one of these molecules, integrins, which was concerned with cell-cell adhesion and cell-extracellular matrix proteins, was prominently expressed on lymphocytes and neutrophils in inflammatory diseases. In addition, administration of monoclonal antibodies against integrins in vivo abrogated the inflammatory responses completely in rats. These findings suggest that adhesion molecules are involved in not only immune responses but also in the pathogenesis of inflammatory diseases. As shown here, integrins also play an important role in the metastasis of small cell lung cancers.
Animals↗
[Application of ultrasonography in chest diseases--usefulness of ultrasonography and physiologic evaluation of low pressure vascular system].
Air and bones, which limit echo penetration to the lesion, are the major components of the thorax. Recently, however, there have been many reports concerning the valid application of ultrasonography for evaluation of chest diseases. In this paper, we report the usefulness of ultrasonography to analyze thoracic lesions based on our experience. Generally, ultrasonography is a method providing high resolution and real-time images. Using these characteristics, we tried to obtain physiological information concerning the hemodynamics of the right heart system. We addressed the results of our studies using ultrasonography as follows; 1) relationship between ventilatory change of IVC and central venous pressure, 2) estimation of pulmonary arterial pressure by measuring the size of the pulmonary artery, 3) dynamic changes of SVC configuration.
Hemodynamics↗
[A case of hypersensitivity pneumonitis due to isocyanate exposure showing progression even two months after removal of the antigen].
A 68-year-old male developed dry cough and exertional dyspnea after handling paint spray containing isocyanates (TDI, MDI) for three months. Initially, the symptoms fluctuated according to whether he was at work or not. He was admitted to our hospital on February 7, 1990, because of progressive worsening of symptoms. In spite of admission to hospital and cessation of exposure to isocyanates, there was no improvement of symptoms. His chest X-ray film showed diffuse small nodular and reticular shadows. Transbronchial lung biopsy revealed thickening of the alveolar walls and formation of Masson's bodies associated with mononuclear cell infiltration in alveolar spaces. High titers of TDI-HSA and MDI-HSA specific IgG antibodies were detected by ELISA, and a high level of serum soluble IL2 receptor was also detected. From these results, we diagnosed hypersensitivity pneumonitis due to exposure to isocyanates. One week administration of prednisolone caused dramatic improvement of his symptoms, chest X-ray findings, and laboratory data. His clinical course and response to prednisolone therapy indicated that long-term steroid administration could not be avoided. The prolonged symptoms and the necessity for long-term steroid therapy are discussed.
Aged↗
[Japanese clinical statistical data of patients with chronic obstructive pulmonary disease].
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Adolescent↗
[Three cases of surgical treatment of dumbbell neurogenic tumor of the mediastinum--evaluation of intraspinal extension in cases without clinical symptoms].
Three cases of dumbbell neurogenic tumor of the posterior mediastinum are reported. None of these cases had any clinical symptoms, and an abnormal mass shadow of the mediastinum had been detected on routine chest roentgenogram. In these three cases, however, myelogram and/or CT findings were suggestive of tumor extension to the spinal canal in various degrees. Two tumors were resected in two-stage operations with combined thoracic and neurosurgical approaches, and the other case was treated by one-stage operation with the combined two approaches. All tumors were completely resectable without serious complications such as hemorrhage, leakage of spinal fluid, or neurologic deficit. Even in asymptomatic patients with neurogenic tumor located in the paravertebral region, it should be determined whether or not the tumor extends to the spinal canal through the intervertebral foramen, prior to planning the surgical procedure.
Adult↗
[An autopsy case of Marfan syndrome with bronchiectasis and multiple bullae].
We report an autopsy case (27-year-old male) with Marfan syndrome, who died of chronic respiratory failure due to bronchiectasis and multiple bullae in both lungs. He had suffered from expectoration of massive amounts of sputum since the age of 15 years. At this time, chest roentgenogram had revealed bronchiectatic changes in the bilateral lower lung fields. Seven years later at the age of 22 years, the formation multiple bullae in both lungs were added to the bronchiectatic changes on chest roentgenogram. Administration of erythromycin (400 mg/day) was started in February, 1987, and the massive sputum volume markedly decreased according to appearance of bullous formation. He was admitted to our department because of deterioration with chronic respiratory failure and right heart failure at 26 years in December, 1989. Although various therapy was performed, he died of chronic respiratory failure in February, 1990. Autopsy findings were as follows: (1) cyclindrical bronchiectatic changes in bilateral lower lobes and (2) extensive multiple bullae in the subpleural areas with bronchiectatic changes in the middle and bilateral lower lobes, with no bronchiectatic changes in the bilateral upper lobes. Several pulmonary disorders accompanying Marfan syndrome have been reported, especially in children. However, the present case demonstrated that fetal pulmonary involvement by Marfan syndrome may not present until adulthood, and affect both airways and lung parenchyma.
Adult↗
[Graphic evaluation of the significance band for hypercapnia in pulmonary disorders].
Alveolar hypoventilation due to the chronic obstruction of the airway such as pulmonary emphysema, or severe restrictive dysfunction due to sequela of pulmonary tuberculosis causes chronic hypercapnia (chronic respiratory acidosis). Ninety-five percentile of significance band of chronic and acute hypercapnia of both experimental and clinical setting is introduced in the graphic display of the acid-base balance. On acute exacerbation of these disorders, examination of arterial blood gas in series are usually plotted along the significance band of hypercapnia. With clinical improvement, the plot will gradually drop down to the chronic stable area of the band. Although cases with metabolic disorders complicate the interpretation, evaluation of the acid-base status using the graphic display will be of help at bedside assessment.
Bicarbonates↗
[Alpha 1-antitrypsin genes in patients with alpha 1AT deficiency in Japan: mutational analysis and allelic background].
Deficiency of alpha 1-antitrypsin (alpha 1AT), a plasma serine protease inhibitor, increases the risk of precocious pulmonary emphysema. Patients with alpha 1AT deficiency in Japan are extremely rare and no Z type alpha 1AT deficiency, which is one of the most frequent genetic disorders among Caucasians, are reported in Japan at the level of gene analysis. It is not yet clear why Z type alpha 1AT is rare among Japanese. When Ala213(GCG)-Val213(GTG) mutation in the alpha 1AT gene was examined by restriction endonuclease BstPI, all of 156 Japanese samples were Val213(GTG) in contrast to the finding that 30% of U.S. Caucasians are Ala213(GCG), indicating that alpha 1AT genes among Japanese were diverted from M1(Val213) variant and are different from M1(Ala213) variant, from which Z variant was likely diverted. This may explain why Z type alpha 1AT deficiency is not found among Japanese. A new alpha 1AT deficient variant, Siiyama (Ser53(TCC)-Phe53(TTC)), was found in a 39-year-old male with pulmonary emphysema (Seyama K, et al, J Biol Chem, 266, 12627, 1991). Interestingly, 6 out of 10 families with alpha 1AT deficiency in Japan shared the identical substitution as Siiyama. This indicates that although Caucasian type Z alpha 1AT deficiency is not found, Siiyama variant may be relatively common in Japan and even in other oriental countries because of the historical migration of people.
Alleles↗
[A case of mediastinal lymphatic cyst possibly originating from the right thoracic duct].
A 33-year-old male was admitted to our hospital because of an abnormal shadow in the right superior mediastinum on chest X-ray, which was first noted about half a year before his admission. Several diagnostic procedures, including fiber bronchoscopy, CT scan and MRI; however, no final diagnosis could be made. Thoracotomy was performed with the presumed diagnosis of benign mediastinal cystic tumor. Entry of the right thoracic duct into the cyst was observed at operation. Histological examination revealed that the cyst was lined by mono-layered cuboidal epithelial cells with lymphocytic infiltration in the wall of the cyst. The mono-layered cuboidal epithelium of the thoracic duct gradually changed to the epithelium of the cyst. Twenty ml of colorless transparent fluid was aspirated from the cyst. Analysis of aspirated fluid revealed low concentration of several substances except LDH compared to the values of the previously reported normal standards and other reported cases. From these findings, the cyst was diagnosed as mediastinal lymphatic cyst. Entry of the thoracic lymphatic duct into the mediastinal lymphatic cyst is a rare occurrence, and it is of interest to speculate on its relationship to the origin of the lymphatic cyst.
Adult↗
[Mediastinitis with bilateral empyemas following neck phlegmon].
A 46-year-old male complaining of fever and neck swelling was transferred to our department because of bilateral empyemas in the pleural spaces following neck phlegmon. A culture of the right pleural effusion obtained at his local hospital revealed Gram-positive anaerobic cocci. Chest roentgenogram showed bilateral effusions and widening of the upper mediastinum. Neck roentgenogram confirmed widening of the retropharyngeal space. CT clearly demonstrated a continuous lesion from the neck to the mediastinum and occupying the pleural spaces bilaterally. The patient responded to intravenous antibiotics and tube drainage of the pleural cavities, and was discharged after 10 weeks of hospitalization. Because no mediastinal abscess requiring drainage was recognized on CT, surgical drainage of the mediastinal space was not performed. Sequential CT was used to follow the lesion in the retropharyngeal space, mediastinum and pleural spaces, to check and reposition the tubes for drainage of pleural exudate.
Cellulitis↗
[A case of recurrent pulmonary alveolar proteinosis treated by pulmonary lavage: were the remissions due to the natural course or due to the lavage?].
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.
Adult↗
Siiyama (serine 53 (TCC) to phenylalanine 53 (TTC)). A new alpha 1-antitrypsin-deficient variant with mutation on a predicted conserved residue of the serpin backbone.
alpha 1-antitrypsin (alpha 1AT), a plasma serine protease inhibitor, increases the risk of precocious pulmonary emphysema in individuals when deficient. Although more than 25 years have passed since a deficiency in the serum level of alpha 1AT was reported, it is only recently that the consequence of the amino acid replacement which leads to the deficient state has been discussed in terms of the crystallographic structure of alpha 1AT and the amino acid residues conserved in the superfamily to which it belongs. Our case involved a 38-year-old Japanese male with alpha 1AT deficiency which was analyzed and identified as a new deficient variant. The serum alpha 1AT of the proband migrated to the S position of the reference serum which is more cathodal than M1, the predominant normal variant, when isoelectric focusing (pH 4.2-4.9) is performed by a combination of Western blotting and crossed immunoelectrophoresis. The new deficient variant is designated as Siiyama after his birthplace. Although liver biopsy specimen showed no apparent pathological findings, PAS-positive with diastase-resistant inclusion bodies and immunoreactive aggregates were detected in several hepatocytes. In addition, similar alpha 1AT mRNA transcript levels were observed in peripheral blood leukocytes from the proband and healthy subjects by Northern analysis. All the coding exons (exon Ic, II, III, IV, and V) of the alpha 1AT gene of the proband and his family were amplified by polymerase chain reaction and followed by direct sequencing. A single missense mutation, Ser53 (TCC) to Phe53 (TTC was identified in exon II of the proband's alpha 1AT gene. All his family examined were heterozygous at this base. Ser53 is one of the most conserved residues as predicted by Huber and Carrell (Huber, R., and Carrell, R. W. (1989) Biochemistry 28, 8951-8966) and is thought to contribute to the organization of the internal core element of the alpha 1AT molecule. The mutational matrix number of Ser to Phe substitution is -3, indicating that this change is evolutionally rare. In this regard, a possible explanation for the deficient state in alpha 1AT Siiyama is that the change from an uncharged polar to a nonpolar amino acid imposed on the conserved serpin backbone exerts severe effects on the integrity of the molecule, and hence alters the intracellular processing of alpha 1AT.
Adult↗
[Chronic respiratory failure].
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Animals↗
Respiratory nicotine absorption in non-smoking females during passive smoking.
The aim of this study was to measure nicotine concentrations in inspired and expired air so as to learn more about respiratory (nasopharyngeal cavity and lung) nicotine absorption from inspired air and to estimate the nicotine intake during passive smoking. A total of 17 young non-smoking women were exposed to experimental passive smoking. Inspired and expired air was sucked at a constant rate into samplers filled with acid-treated diatomite (Uniport-S) to absorb nicotine in the air. Absorbed nicotine was assayed by gas chromatography. The range of nicotine concentration in the inspired air was 40-200 micrograms/m3. In this setting, 47 samples obtained from the 17 subjects were assayed. Nicotine absorption, which was calculated as [(nicotine concentration in inspired air-nicotine concentration in expired air)/nicotine concentration in inspired air] x 100, remained at 60%-80% (mean +/- SD, 71.3% +/- 10.2%) without being affected by the nicotine concentration in the inspired air. From this result, it was estimated that the average intake of nicotine was 0.026 mg/h in a group of non-smokers exposed in a room containing a nicotine concentration of 100 micrograms/m3, which is equivalent to fairly severe involuntary tobacco smoking. This is the first report on the estimation of respiratory nicotine absorption and nicotine intake during passive smoking based on the direct measurement of nicotine concentrations in both inspired and expired air.
Adolescent↗
Enhanced neutrophil functions in a patient with colony-stimulating activity-producing lung cancer.
We studied neutrophil functions in a patient with colony-stimulating activity (CSA)-producing lung cancer. A 59-year-old man had an abnormal chest X-ray and leucocytosis, predominantly with neutrophils. Pneumonectomy was performed, and the histological diagnosis of the tumour was large-cell carcinoma of the lung. The tumour induced marked granulocytosis in tumour-transplanted nude mice, and the conditioned media of the tumour contained very strong human-active CSA. Superoxide release and membrane depolarization in neutrophils stimulated by the chemotactic peptide, N-formyl-methionyl-leucyl-phenylalanine, were markedly enhanced in the patient. These findings suggest that CSA produced by the tumour primed neutrophil functions in vivo in the patient.
Animals↗