Fibroblasts positive for meflin have anti-fibrotic properties in pulmonary fibrosis.

The prognosis of elderly individuals with idiopathic pulmonary fibrosis (IPF) remains poor. Fibroblastic foci, in which aggregates of proliferating fibroblasts and myofibroblasts are involved, are the pathological hallmark lesions in IPF to represent focal areas of active fibrogenesis. Fibroblast heterogeneity in fibrotic lesions hampers the discovery of the pathogenesis of pulmonary fibrosis. Therefore, to determine the pathogenesis of IPF, identification of functional fibroblasts is warranted. The aim of this study was to determine the role of fibroblasts positive for meflin, identified as a potential marker for mesenchymal stromal cells, during the development of pulmonary fibrosis.We characterised meflin-positive cells in a single-cell atlas established by single-cell RNA sequencing (scRNA-seq)-based profiling of 243 472 cells from 32 IPF lungs and 29 normal lung samples. We determined the role of fibroblasts positive for meflin using bleomycin (BLM)-induced pulmonary fibrosis.scRNA-seq combined with in situ RNA hybridisation identified proliferating fibroblasts positive for meflin in fibroblastic foci, not dense fibrosis, of fibrotic lungs in IPF patients. A BLM-induced lung fibrosis model for meflin-deficient mice showed that fibroblasts positive for meflin had anti-fibrotic properties to prevent pulmonary fibrosis. Although transforming growth factor-ß-induced fibrogenesis and cell senescence with the senescence-associated secretory phenotype were exacerbated in fibroblasts via the repression or lack of meflin, these were inhibited in meflin-deficient fibroblasts with meflin reconstitution.These findings provide evidence to show the biological importance of meflin expression on fibroblasts and myofibroblasts in the active fibrotic region of pulmonary fibrosis.

Secondary organizing pneumonia following viral pneumonia caused by severe influenza B: a case report and literature reviews.

BACKGROUND: Some reported that organizing pneumonia (OP) may occur after influenza A infections including swine-origin influenza A (H1N1). However, OP associated with influenza B infection has never been reported. We report the first case of secondary OP associated with viral pneumonia caused by influenza B. CASE PRESENTATION: A 23-year old woman was diagnosed as viral pneumonia caused by type B influenza. Despite of antiviral therapy, abnormal chest shadows were not improved. Bronchoscopy and transbronchial lung biopsy showed organizing pneumonia due to viral pneumonia caused by influenza B. Corticosteroid therapy was started at 30 mg daily (0.5 mg/kg), and the dose was reduced to 25, 20, 15 or 10 mg per day every month with symptomatic and radiological resolution. Even after corticosteroid therapy was discontinued, we did not confirm disease recurrence. CONCLUSIONS: Physicians should be aware of the possibility for SOP and severe viral pneumonia even in case of type B as well as type A influenza infections.

Case of cholangiocellular carcinoma in a patient with glycogen storage disease type Ia.

Glycogen storage disease (GSD) type Ia is caused by a deficiency in glucose-6-phosphatase. Long-term complications, including renal disease, gout, osteoporosis and pulmonary hypertension, develop in patients with GSD type Ia. In the second or third decade, 22-75% of GSD type Ia patients develop hepatocellular adenoma (HCA). In some of these patients, the HCA evolves into hepatocellular carcinoma. However, little is known about GSD type Ia patients with HCA who develop cholangiocellular carcinoma (CCC). Here, we report for the first time, a patient with GSD type Ia with HCA, in whom intrahepatic CCC was developed.

New diagnostic technique for rapid fluorescence immunocytochemical staining of adenocarcinoma and mesothelial cells using liquid-based cytology.

OBJECTIVE: To evaluate the expression of antibodies against calretinin, cytokeratin 5/6, desmin, D2-40, HBME-1, mesothelin, thrombomodulin, WT1, Ber-EP4, CEA, EMA and MOC-31 individually and to compare it with a new rapid procedure for fluorescence immunocytochemistry (ICC) using liquid-based cytology (LBC). STUDY DESIGN: Sixty-four peritoneal cell specimens prepared with the LBC method were stained with these markers to evaluate their usefulness and develop a rapid fluorescence immunostaining method using Ber-EP4 that is applicable to intraoperative cancer cytodiagnosis. RESULTS: The adenocarcinoma markers were positive in 92% of adenocarcinoma cases, 57% of cases with suspicion of adenocarcinoma, and 5% of negative cases (reactive mesothelial cells). On the other hand, the mesothelial cell markers were positive in 8-15% of adenocarcinoma cases, 43-57% of cases with suspicion of adenocarcinoma, and 93-95% of negative cases. The rapid new fluorescence ICC procedure clearly stained only the adenocarcinoma cells within 20 min. CONCLUSION: Immunocytochemical examination with the LBC method is a powerful ancillary technique for discriminating adenocarcinoma cells from mesothelial cells. This rapid new fluorescence ICC procedure can be used as an ancillary technique for accurate detection of adenocarcinoma cells in the intraoperative cytological examination of peritoneal or pleural washing fluid.

A case of estrogen receptor positive secretory carcinoma in a 9-Year-old girl with ETV6-NTRK3 fusion gene.

The patient was a 9-year-old premenarcheal pediatric female, whose chief complaint was a well-circumscribed palpable right breast mass without nipple discharge. Although the patient had noticed the lump 2 years prior to hospital admission, its size (1.5 × 1.3 cm) had been stable. There was no family history or previous history of malignancies. Physical examination showed a well-delimited, elastic-firm and movable tumor just beneath the nipple and areolar complex. Regional lymph nodes were not palpable. Ultrasonography and breast computed tomography revealed a subareolar oval-shaped tumor exhibiting homogeneous echogenicity with clear margins. Distant metastases could not be detected using whole-body computed tomographic scans. A fine-needle aspiration cytology specimen showed atypical cells with prominent nucleoli and abundant intracellular secretory material, suggesting the possibility of secretory carcinoma. Histopathological analysis of the core needle biopsy specimen revealed that the tumor was a secretory carcinoma. The patient underwent total mastectomy with sentinel lymph node biopsy. Metastases were not observed in the removed lymph nodes. Estrogen receptor was weakly positive and progesterone receptor was negative. Human epidermal growth factor receptor 2 expression was also negative. In addition, the ETV6 (exon 5) and NTRK3 (exon 13) fusion gene was detected using the reverse transcription-polymerase chain reaction method. This gene is considered specific for secretory carcinoma. Immunohistochemistry revealed weak basal differentiation [cytokeratin 5/6(CK5/6)(+), vimentin(+) and epidermal growth factor receptor(+)]. The patient has received no adjuvant therapy and is currently disease free at 12 months after surgery.

Significant prognostic factor of immunohistochemical HER-2 expression using initial prostate biopsy specimens with M1b prostate cancer.

BACKGROUND: We examined whether human epidermal growth factor-2(HER-2) overexpression could be a useful marker of outcome after hormone therapy in patients with M1b prostate cancer (PC). SUBJECTS AND METHODS: The subjects were 102 patients who were diagnosed with M1b PC at Aichi Medical University Hospital. HER-2 expression was determined by immunohistochemical (IHC) staining using initial needle biopsy specimens for diagnosis. The results were classified into four grades (0, 1+, 2+, 3+), and scores of 1+ or greater were considered overexpression and defined as positive. RESULTS: The results showed a rating of 0 in 72 subjects, 1+ in 10, 2+ in 14, and 3+ in 6; 30 subjects (29.4%) were classified as HER-2 positive. Comparison of clinical data of HER-2 positive and negative subjects obtained at baseline revealed many of the subjects with high-grade tumors by Gleason score were HER-2 positive (P = 0.030). The prostate-specific antigen (PSA) relapse was observed in 76 subjects and cause-specific death occurred in 44. A significant difference was observed only in the item HER-2 (negative vs. positive) by multivariate Cox proportional hazard analysis. The 5-year PSA relapse-free rate was 0% in subjects with HER-2 positive (26/30), and 43.9% in subjects with HER-2 negative (50/72, P = 0.0192). The 5-year cause-specific survival rate was 40.9% in subjects with HER-2 positive (30/102), and 67.3% in subjects with HER-2 negative (72/102, P = 0.0301). CONCLUSION: HER-2 overexpression as determined by IHC staining using needle biopsy specimens for diagnosis with M1b PC is a significant prognostic factor for PSA relapse after hormone therapy and unfavorable outcome.

Immunocytochemical analysis for differential diagnosis of thyroid lesions using liquid-based cytology.

Recently, liquid-based cytology (LBC) has been widely applied to various samples in diagnostic cytology and its usefulness has been reported. In this study, we investigated thyroid cytology that applied LBC and immunocytochemistry to achieve more objective diagnosis and greater diagnostic accuracy. This study included 125 cases (57 papillary carcinomas (PCs), 22 follicular tumors, 43 adenomatous goiters and 3 with Basedow's disease). After preparing the LBC slide, immunocytochemical staining was performed on each slide with six antibodies (HBME-1, cytokeratin 19 (CK19), high molecular weight cytokeratin (34JE12), galectin-3, CD15 and CA 19-9). All antibodies presented immunopositivity frequently in PCs, but only a few or some of them were positive in other cases. These antibodies were considered positive markers for PCs, and the most reliable marker was 34betaE12; its sensitivity, specificity and diagnostic accuracy were 82.5%, 100% and 92.0%, respectively. Relations of immunocytochemical profiles against these markers were assessed using panel 34betaE12, GAL-3 and CK19. More than or equal to two of these markers showed co-positive in 53 of 57 PCs, and negative for all markers was observed in only one case. In the other (non PC) cases, the former was 0 of 58 and the latter was 40 cases. In this panel, the sensitivity, specificity and diagnostic accuracy were 93.0%, 100% and 96.8%, respectively. All of these values were higher than or equal to single values of 34betaE12. We concluded that the panel in this study is useful for more objective and accurate diagnosis of thyroid cytology.

An aspiration material preparation system: application of a new liquid-based cytology technique for fine-needle aspiration of the breast.

OBJECTIVE: To evaluate the usefulness of our original liquid-based cell preparation system AMAPS (aspiration material preparation system) and to compare it with the AutoSmear system in breast aspiration cytology. STUDY DESIGN: A total of 487 specimens of fine-needle aspiration cytology of the breast were retrieved, of which 250 were processed with AMAPS and 237 with the AutoSmear method (before the introduction of AMAPS). A final histological diagnosis was obtained by an excisional biopsy or a surgical resection in 148 cases. RESULTS: Cell recovery rates were significantly improved with AMAPS (96.8 and 99.1% in Papanicolaou and Diff-Quik, respectively) compared with the AutoSmear method (40.9 and 42.3%, respectively; p<0.01). Within-run and day-to-day reproducibility of cell recovery was satisfactory, with coefficients of variations of 6.8 and 8.7%, respectively. Following the introduction of AMAPS in breast cytology, the unsatisfactory rate decreased significantly (from 16.0 to 8.8%; p<0.01), while the diagnostic sensitivity for malignancy did not change (97.8 to 98.1%). Moreover, the diagnostic specificity for benign lesions increased from 75 to 93.8%, thus decreasing the excision rate of fibrocystic disease. CONCLUSION: AMAPS may serve as an alternative to the conventional technique or commercially available liquid-based cytology systems.

RhoB enhances migration and MMP1 expression of prostate cancer DU145.

Rho family protein regulates variety of cellular functions as cytoskeletal organization, cell proliferation and apoptosis. In the present study, we demonstrate that RhoB-overexpressed prostate cancer cells showed an enhanced cell motility and the administration of the GSK-3 inhibitors inhibited this increase in migration. Among the extracellular matrix and adhesion-related molecules, MMP1 RNA expression was increased in RhoB-overexpressed cells, administration of MMP inhibitor suppressed the collagen gel invasion in these cells. This is the first report evaluating RhoB function and the downstream signaling events in prostate cancer cell. Our results indicate that RhoB promotes cell motility and invasion in a metastatic prostate cancer cell.

Prognostic factors in rapidly progressive interstitial pneumonia.

UNLABELLED: Clinical and other features, as well as prognostic factors for survival, were examined in patients with rapidly progressive interstitial pneumonia. The disease entity included different histological patterns with diverse outcomes, and distinctions were not possible from baseline data. Histological diagnosis was the only significant prognostic determinant. BACKGROUND AND OBJECTIVE: The aim of the present study was to examine clinical and other features that might allow prognostic distinctions between histological patterns in presentations with rapidly progressive interstitial pneumonia (RPIP), and to assess prognostic factors for survival. METHODS: Patients with RPIP among 425 consecutive patients with diffuse lung disease, who underwent surgical lung biopsy, were studied retrospectively. The discriminatory value of clinical and investigative features for identifying disease with a better outcome was evaluated. An a priori comparison was made between diffuse alveolar damage (DAD)/usual interstitial pneumonia with DAD pattern (Group A), and organizing pneumonia/non-specific interstitial pneumonia pattern (Group B). RESULTS: Twenty-eight patients (6.6%) fulfilled the criteria for RPIP. The diagnosis was Group A disease in 15 (DAD in 10, usual interstitial pneumonia with DAD in 5), and Group B disease in 13 (organizing pneumonia in 8, non-specific interstitial pneumonia in 5). There were no significant differences in initial findings between the groups. Prognosis was significantly better for Group B patients than for Group A patients (P=0.021). Neither BAL nor parenchymal high-resolution CT score was indicative of therapeutic responsiveness or outcome. Distinction between Group A and Group B on the basis of disease pattern was the only significant determinant of prognosis. CONCLUSIONS: RPIP included varied histological patterns with different outcomes, and in many cases these could not be predicted using baseline clinical data. Histology was the only significant predictor of ultimate prognosis.

Acute Exacerbation of Pleuroparenchymal Fibroelastosis Secondary to Allogenic Hematopoietic Stem Cell Transplantation.

In this article, we report a case with pleuroparenchymal fibroelastosis (PPFE) following hematopoietic stem cell transplantation (HSCT) that developed acute respiratory failure with new bilateral ground glass opacity, which could not be explained by either a pulmonary infection, drug toxicity or extraparenchymal causes. Although combination therapy with multiple immunosuppressants was transiently effective, the patient died from a recurrent exacerbation. Autopsied lungs demonstrated diffuse alveolar damage superimposed on PPFE. There was no evidence of any coexisting interstitial pneumonia with the usual interstitial pneumonia (UIP) pattern. Our case suggests that acute exacerbation can occur in patients with post-HSCT PPFE, even when a coexisting UIP pattern is absent.

Up-regulation of surfactant protein production in a mouse model of secondary pulmonary alveolar proteinosis.

Although Pneumocystis infection might be one of the causes of secondary pulmonary alveolar proteinosis (PAP), the mechanism of its pathogenesis is uncertain. We analyzed a mouse model of secondary PAP resulting from Pneumocystis infection using mice deficient in CD40 (CD40KO), and evaluated the mechanism of the pathogenesis of secondary PAP from the viewpoint of surfactant-associated protein (SP) homeostasis, the overproduction of SP by type II alveolar epithelial cells, and the phagocytic function of alveolar macrophages (AMs). The effect of CD40 on SP production was also investigated in vitro using the H441 cell line, which has a phenotype similar to type II alveolar epithelial cells and primary alveolar epithelial cells. After long-term exposure to ovalbumin, CD40KO mice showed Pneumocystis infection and accumulation of surfactants in the alveoli (ApCD40KO). The amounts of SP production were up-regulated in ApCD40KO mice compared with wild-type mice treated using the same procedure. On the other hand, AMs from ApCD40KO mice did not show either phagocytic dysfunction or down-regulation of PU.1 expression. Furthermore, the stimulation of CD40-CD40 ligand (CD154) pathway regulated the production of SPs in H441 cells or primary alveolar epithelial cells. These results suggested that CD40KO mice could be one of the models useful for developing secondary PAP resulting from Pneumocystis infection. Surfactant accumulation was due to the overproduction in our model of secondary PAP. The CD40-CD154 interaction plays an important role in the regulation of surfactant-associated protein production.

Cathepsin S, a new serum biomarker of sarcoidosis discovered by transcriptome analysis of alveolar macrophages.

BACKGROUND: Development of reliable new biomarkers remains crucial to improve diagnosis and assessing disease activity in sarcoidosis. The objective of this study was to seek such markers from the gene expression signature of alveolar macrophages by transcriptome analysis. METHODS: Pooled RNA extracted from alveolar macrophages from patients with active sarcoidosis and control patients was subjected to transcriptome analysis using microarrays. Expressed gene intensity in sarcoidosis relative to that in control was calculated. We measured serum cathepsin S (CTSS) concentrations in 89 healthy volunteers, 107 patients with sarcoidosis, 26 with interstitial pneumonia, 150 with pneumoconiosis, and 76 with pulmonary mycobacteriosis by the enzyme-linked immunosorbent assay. RESULTS: Among 12 genes with ratios higher than that of a housekeeping gene, we selected CTSS for scrutinizing protein expression in serum because of the feasibility of the protein assay. CTSS concentrations were significantly increased in sarcoidosis compared with not only controls but also all the other lung diseases. Receiver operating characteristics curve for sarcoidosis and parenchymal lung diseases revealed an area under the curve of 0.800 (95% confidence interval, 0.751-0.850; p=1.4 x 10-18) with 70% sensitivity and 78% specificity at a CTSS concentration of 15.5 ng/ml. A significant trend was identified between CTSS concentrations and the number of affected organs. Serum CTSS concentrations varied in parallel with clinical courses both spontaneously and in response to corticosteroid therapy. Epithelioid cells in granulomas were positive for immunohistochemical staining with CTSS. CONCLUSIONS: CTSS has the potential to be a useful biomarker in sarcoidosis.

Matrix metalloproteinase-9 associated with heparan sulphate chains of GPI-anchored cell surface proteoglycans mediates motility of murine colon adenocarcinoma cells.

Using murine colon adenocarcinoma-derived clones with different metastatic potentials, the cellular localization of matrix metalloporteinase-9 (MMP-9) and its role in the cell motility were examined. Highly metastatic LuM1 clone aggressively invaded into adjacent tissue in vivo, but low metastatic NM11 clone did not. As compared with the NM11 clone, the LuM1 clone expressed and secreted a remarkably large amount of MMP-9, and exhibited higher abilities of cell migration and invasion in vitro, which were suppressed by MMP-2/MMP-9 inhibitor IV. MMP-9, exhibiting high affinity to heparin, was demonstrated to be condensed on tips of cellular podia. Treatment of the cells with heparitinase-I or heparin resulted in release of MMP-9 from the cell surface, which caused concomitant suppression of their motility to a similar level to that with the MMP inhibitor. Immunoprecipitation of a LuM1 cell lysate with an anti-MMP-9 antibody resulted in co-precipitation of phosphatidylinositol-specific phospholipase C-susceptible heparan sulphate proteoglycans having 66 and 64 kDa core proteins. Taken together, the present results demonstrate that secreted MMP-9 associates with glypican-like proteoglycans through their heparan sulphate chains, and plays a crucial role in cell motility of LuM1 cells.

TH1/TH2 immune response in lung fibroblasts in interstitial lung disease.

BACKGROUND: Inflammatory response in pulmonary fibrosis closely resembles a T-helper (Th) 2 immune response. For recruitment to an inflammatory lesion, the majority of Th1 cells express CXC chemokine receptor 3, recognizing monokine induced by interferon-gamma (Mig), interferon gamma-inducible protein of 10 kD (IP-10), and interferon-inducible T-cell alpha chemoattractant (I-TAC). Th2 cells express CC chemokine receptor 4, recognizing thymus- and activation-regulated chemokine (TARC) and macrophage-derived chemokine (MDC). We investigated the Th1/Th2 chemokine production patterns by lung fibroblasts and their evaluation in bronchoalveolar lavage (BAL) fluid of interstitial lung disease. METHODS: The production pattern of Th1/Th2 chemokines by lung fibroblasts was examined in ELISA and quantitative reverse transcriptase polymerase chain reactions. Th1/Th2 chemokine levels in BAL fluid of idiopathic pulmonary fibrosis (IPF) and nonspecific interstitial pneumonia (NSIP) were examined to evaluate the clinical relevance of Th1/Th2 chemokines. RESULTS: The lung fibroblasts were polarized to produce Th1-type chemokines by the pro-inflammatory cytokine, tumor necrosis factor (TNF)-alpha and the anti-fibrotic cytokine, interferon (IFN)-gamma. However, the induction patterns of chemokines by these two cytokines were different, i.e., involving predominant induction of IP-10 and I-TAC by TNF-alpha and induction of Mig by IFN-gamma. Although Mig, IP-10, and I-TAC were produced within the BAL fluid of patients, TARC and MDC were at significantly low levels. CONCLUSIONS: Our results suggest that lung fibroblasts tend to induce a Th1-type immune response under normal conditions, and that a Th2-type immune response does not play a significant role in smoldering inflammation around the established lesions in IPF and NSIP.

The role of organic cation transporter-3 in methamphetamine disposition and its behavioral response in rats.

Organic cation transporter-3 (OCT3) is expressed in several tissues including the brain. We have previously demonstrated that rats with behavioral sensitization to methamphetamine (METH) increased the brain penetration of METH with decreased expression of OCT3 in brain. Considering the earlier in vitro studies demonstrating that 1) OCT3 could transport dopamine (DA) and 2) the specific transport via OCT3 could be inhibited by METH, these results suggest that decreased OCT3 might decrease the efflux of METH and/or DA from brain, subsequently causing the development of behavioral sensitization. Thus, in the present study, behavioral task related to DA and pharmacokinetic experiment were performed using rats treated with antisense against OCT3 (OCT3-AS) since no specific ligands for OCT3 are still available. The continuous infusion of OCT3-AS into the third ventricle significantly decreased the expression of OCT3 in choroid plexus (CP) epithelial cells. Both METH-induced hyperlocomotion and METH-induced extracellular DA levels in nucleus accumbens and prefrontal cortex were significantly increased in OCT3-AS-treated rats. Moreover, the concentrations of METH were significantly increased in cerebrospinal fluid as well as extracellular areas at the nucleus accumbens in OCT3-AS-treated rats. These results suggested that decreased OCT3 elevated the concentration of METH and/or DA in brain, subsequently enhancing dopaminergic neuronal transmission and increasing METH-induced hyperlocomotion. In summary, OCT3 at the CP could regulate the effect of METH by controlling the levels of METH and/or DA in brain. Thus, these results suggest that OCT3 may be a new molecular target to treat METH-related disorders such as drug abuse and schizophrenia.

Differential cardiovascular effects of endotoxin derived from Escherichia coli or Pseudomonas aeruginosa.

Sepsis is characterized by various symptoms, signs and underlying pathophysiology. To investigate possible mechanisms underlying this diversity, we compared the cardiovascular effects of lipopolysaccharide (LPS) derived from Escherichia coli (E-LPS) with those of LPS from Pseudomonas aeruginosa (P-LPS) in rats. We also examined the possible roles of tumor necrosis factor-alpha (TNF-alpha) and oxidative stress in LPS-induced cardiovascular damage. E-LPS (10 mg/kg body weight) or P-LPS (2 mg/kg body weight) was administered intravenously to Wistar rats. Echocardiography was serially performed. E-LPS induced an increase in left ventricular fractional shortening that persisted for at least 6 h, whereas P-LPS elicited an initial increase and a subsequent decrease in this parameter. Histological analysis revealed that P-LPS induced interstitial edema, congestion, intramyocardial bleeding, myocardial necrosis, infiltration of inflammatory cells, and formation of fibrin thrombi in the heart, whereas no pathological changes were apparent in the hearts of rats treated with E-LPS. Furthermore, the plasma concentration of TNF-alpha in rats treated with P-LPS was greater than that in rats treated with E-LPS, but the glutathione redox ratio in the heart was not affected by either type of LPS. In conclusion, E-LPS and P-LPS induced distinct patterns of functional and structural responses in the cardiovascular systems of rats. These differential responses may be attributable in part to the difference in the associated increases in the plasma concentration of TNF-alpha. The cardiovascular effects of LPS thus depend on the causative organisms.

A 63-Year-Old Woman With Progressive Dyspnea After Remission of Lymphoma.

CASE PRESENTAION: A 63-year-old woman visited our hospital for a further evaluation of progressive dyspnea. She had developed a progressive airflow obstruction after 3 years' remission of non-Hodgkin's lymphoma (follicular mixed cell type), which had been treated with chemotherapy (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone). The patient's primary care physician had diagnosed her as having COPD and bronchial asthma and had treated her with medications including inhaled corticosteroids, tiotropium, and oral erythromycin. Her dyspnea had gradually worsened, however, and she had a score of 4 on the modified Medical Research Council dyspnea scale at the time of admission to our hospital.

Acute exacerbation of interstitial pneumonia following surgical lung biopsy.

STUDY OBJECTIVES: Surgical lung biopsy (SLB) plays an important role in the diagnosis of interstitial pneumonia, however, the occurrence of acute respiratory failure following SLB remains largely unreported. We evaluated the incidence, clinical features, therapy and prognosis of acute exacerbation of interstitial pneumonia following SLB. DESIGN: Retrospective study of consecutive patients who underwent SLB to establish a diagnosis of diffuse lung disease between May 1989 and April 2000. Patients with an acute exacerbation following lung biopsy were studied, and the HRCT images of the chest before and after surgery were reviewed. MEASUREMENTS AND RESULTS: Among the 236 consecutive patients with interstitial pneumonia who underwent a surgical lung biopsy, five (2.1%) (IPF, 3; NSIP, 1; COP, 1) developed acute exacerbation of the diffuse lung disease in the course of 1-18 days after SLB. The extent of parenchymal involvement on HRCT before surgery was not significantly different between operated and contralateral nonoperated lung. Significantly increased regions of parenchymal involvement on HRCT were seen postoperatively compared with the preoperative CT in both the operated (20.7+/-12.5% versus 38.2+/-10.8%, P = 0.0431) and nonoperated lung (22.7+/-13.8% versus 70.5+/-24.4%, P = 0.0431), but the extent of the parenchymal involvement was significantly greater on the nonoperated side (P = 0.0251). Two of the 3 IPF patients died from the acute exacerbation. CONCLUSIONS: It is important to be aware of the possibility of acute exacerbation of interstitial pneumonia following SLB even after an apparently uneventful immediate postoperative course. The asymmetric image findings suggest that intraoperative respiratory management is a possible etiologic factor.

Pleuropulmonary blastoma in a 12-year-old boy presenting with pneumothorax.

Pleuropulmonary blastoma is a rare pulmonary neoplasm seen in the pediatric population. We report a purely cystic-type pleuropulmonary blastoma in a 12-year-old boy who presented with spontaneous pneumothorax. He underwent partial resection of the left lower lobe under video-assisted thoracoscopy.