The frequency of diagnosing bronchiectasis is increasing around the world. Cystic fibrosis is the most common inherited cause of bronchiectasis, but there is increasing recognition of significant numbers of patients with bronchiectasis from various causes. With increasing awareness of bronchiectasis, a significant number of research, concerning the causes and treatments, were published over the past few years. Investigation of the underlying cause of bronchiectasis is the most important key to effective management. The purpose of this report is to review the immunological abnormalities that cause bronchiectasis in those that the cystic fibrosis has been excluded, identify the available evidences of current management, and discuss several controversies in the treatment of this disorder.
Bronchiectasis ; Cystic Fibrosis ; Humans ; Immunologic Deficiency Syndromes

Herpes simplex encephalitis is known to cause intracranial hypertension and cerebral edema. When cerebral edema occurs, venous sinuses are compressed, resulting in congestion and thrombosis. An arteriovenous fistula may form as a result, particularly in conjunction with superior sagittal sinus thrombosis. In this report, the authors describe a patient with protein S and C deficiency who developed venous sinus thrombosis and dural arteriovenous fistula after experiencing herpes simplex encephalitis.

PURPOSE: Tuberculous pleurisy is the most frequent extrapulmonary manifestation of tuberculosis. In spite of adequate treatment, pleural fibrosis is a common complication, but the mechanism has not been elucidated. This study is to determine whether epithelial to mesenchymal transition (EMT) of mesothelial cells occurs in tuberculous pleurisy. MATERIALS AND METHODS: Normal pleural mesothelial cells, isolated from irrigation fluids during operations for primary spontaneous pneumothorax, were characterized by immunofluorescence and reverse transcription polymerase chain reaction (RT-PCR). These cells were treated in vitro with various cytokines, which were produced in the effluents of tuberculous pleurisy. The isolated cells from the effluents of tuberculous pleurisy were analyzed by immunofluorescence and RT-PCR analysis. RESULTS: The isolated cells from the irrigation fluid of primary spontaneous pneumothorax had epithelial characteristics. These cells, with transforming growth factor-beta1 and/or interleukin-1beta treatment, underwent phenotypic transition from epithelial to mesenchymal cells, with the loss of epithelial morphology and reduction in cytokeratin and E-cadherin expression. Effluent analysis from tuberculous pleurisy using immunofluorescence and RT-PCR demonstrated two phenotypes that showed mesenchymal characteristics and both epithelial & mesencymal characteristics. CONCLUSION: Our results suggest that pleural mesothelial cells in tuberculous pleurisy have been implicated in pleural fibrosis through EMT.
Cells, Cultured ; Epithelial Cells/*pathology ; Epithelial-Mesenchymal Transition/*physiology ; Fluorescent Antibody Technique ; Humans ; Pleura/*pathology ; Reverse Transcriptase Polymerase Chain Reaction ; Tuberculosis, Pleural/*pathology

Catamenial hemoptysis is a rare condition that's characterized by recurrent hemoptysis occurring in association with menstruation, and this is associated with the presence of intrapulmonary or endobronchial endometrial tissue. The diagnosis of pulmonary endometriosis can be made according to a typical clinical history and with exclusion of other causes of recurrent hemoptysis. Treatment of pulmonary endometriosis can be medical or surgical; however, the optimal management of this condition is still a matter of debate. Medical therapy may be problematic, due to recurrence of symptoms despite hormonal ablation, and adverse effects from long-term hormone therapy can also be a problem. We report here on a case of pulmonary endometriosis in a 23-year-old woman who presented with hemoptysis that occurred during the first 3 days of menstruation, and this happened over a 4 month period. She was successfully treated by video-assisted thoracoscopic surgery (VATS). No more hemoptysis was noted during 12 months of follow-up.
Endometriosis ; Female ; Follow-Up Studies ; Hemoptysis ; Humans ; Menstruation ; Recurrence ; Thoracic Surgery, Video-Assisted ; Young Adult

Immunoglobulin G subclass deficiency (IgGSCD) is a relatively common primary immunodeficiency disease (PI) in adults. The biological significance of IgGSCD in patients with chronic airway diseases is controversial. We conducted a retrospective study to characterize the clinical features of IgGSCD in this population. This study examined the medical charts from 59 adult patients with IgGSCD who had bronchial asthma or chronic obstructive pulmonary disease (COPD) from January 2007 to December 2012. Subjects were classified according to the 10 warning signs developed by the Jeffrey Modell Foundation (JMF) and divided into two patient groups: group I (n = 17) met ≥ two JMF criteria, whereas group II (n = 42) met none. IgG3 deficiency was the most common subclass deficiency (88.1%), followed by IgG4 (15.3%). The most common infectious complication was pneumonia, followed by recurrent bronchitis, and rhinosinusitis. The numbers of infections, hospitalizations, and exacerbations of asthma or COPD per year were significantly higher in group I than in group II (P < 0.001, P = 0.012, and P < 0.001, respectively). The follow-up mean forced expiratory volume (FEV1) level in group I was significantly lower than it was at baseline despite treatment of asthma or COPD (P = 0.036). In conclusion, IgGSCD is an important PI in the subset of patients with chronic airway diseases who had recurrent upper and lower respiratory infections as they presented with exacerbation-prone phenotypes, decline in lung function, and subsequently poor prognosis.
Adult* ; Asthma ; Bronchitis ; Follow-Up Studies ; Forced Expiratory Volume ; Hospitalization ; Humans ; Immunoglobulin G* ; Immunoglobulins* ; Lung ; Phenotype ; Pneumonia ; Prognosis ; Pulmonary Disease, Chronic Obstructive ; Respiratory Tract Infections ; Retrospective Studies

BACKGROUND/AIMS: We investigated the incidence and prognosis of disseminated intravascular coagulation (DIC) using DIC scoring system in patients with severe sepsis or septic shock. METHODS: Patients admitted to the intensive care unit (ICU) in a tertiary hospital for severe sepsis or septic shock were enrolled from Mar. 2008 to Feb. 2009. Using the International Society on Thrombosis and Haemostasis (ISTH) criteria, we calculated DIC score at three time points (Day 0, Day 1, and Day 2). RESULTS: Among 111 patients with severe sepsis or septic shock, 89 (severe sepsis, 8; septic shock, 81) were enrolled. Mean DIC score at ICU admission was 3.3+/-1.3 and the incidence of overt DIC (DIC score > or = 5) during the first 48 hours was 33.8% (27/89). Sequential Organ Failure Assessment (SOFA) score was well correlated with DIC score and was higher in patients with overt DIC than in those without. The ICU, hospital and 28-day death rates in patients with overt DIC were 63.0%, 66.7%, and 63.0%, respectively, which were significantly higher than in those without overt DIC. In multivariate analysis, Simplified Acute Physiology Score (SAPS) II was significantly associated with hospital death (p=0.002), and the occurrence of overt DIC showed a borderline significance (p=0.053). CONCLUSIONS: Using the ISTH criteria for DIC, the incidence of overt DIC was 33.8% in patients with severe sepsis or septic shock. The occurrence of overt DIC was associated with a higher organ failure score and hospital outcomes.
Dacarbazine ; Disseminated Intravascular Coagulation ; Humans ; Incidence ; Intensive Care Units ; Multivariate Analysis ; Organ Dysfunction Scores ; Prognosis ; Sepsis ; Shock, Septic ; Tertiary Care Centers ; Thrombosis

Sarcoidosis is a systemic granulomatous disease that primarily affects the lung and lymphatic system of the body. Since Brincker first noted a statistically significant increase of malignant tumors among sarcoidosis patients, there have been several reports on simultaneously developed sarcoidosis and malignancy. A 30-year-old man was admitted to our hospital because of multiple enlarged mediastinal lymph nodes. The patient had been well until approximately 10 days before admission, when he developed a cough. Chest X-ray and computed tomography (CT) of the chest that were performed at the outpatient department revealed multiple enlarged mediastinal lymph nodes. Cervical lymph node biopsy revealed both non-caseating granuloma and metastatic papillary carcinoma, whereas the mediastinal lymph node showed only non-caseating granuloma. The thyroid gland surgical specimen showed papillary carcinoma. We report here on a case of a 30-year-old man who had sarcoidosis and thyroid cancer, and we include a review of the literature.
Adult ; Biopsy ; Carcinoma, Papillary ; Cough ; Granuloma ; Humans ; Lung ; Lymph Nodes ; Lymphatic System ; Outpatients ; Sarcoidosis ; Sarcoidosis, Pulmonary ; Thorax ; Thyroid Gland ; Thyroid Neoplasms

Idiopathic pulmonary fibrosis (IPF) comprises an aggregate of mesenchymal cells. However, the cellular origin of these mesenchymal phenotypes remains unclear. Transforming growth factor beta1 (TGF-beta1) has been known as the main cytokine involved in the pathogenesis of IPF. We examined whether the potent fibrogenic cytokine TGF-beta1 could induce the epithelial-to-mesenchymal transition (EMT) in the human alveolar epithelial cell line, A549, and determined whether snail expression is associated with the phenotypic changes observed in the A549 cells. EMT was investigated with cells morphology changes under phase-contrast microscopy, western blotting, and indirect immunofluorescence stains. E-cadherin and transcription factor, snail, were also evaluated by measuring mRNA levels using reverse transcriptase-polymerase chain rection (RT-PCR) analysis. The data showed that TGF-beta1 induced A549 cells with epithelial cell characteristics to undergo EMT in a concentration-dependent manner. Following TGF-beta1 treatment, A549 cells induced EMT characterized by cells morphological changes, loss of epithelial markers Ecaherin and cytokeratin, increased stress fiber reorganization by F-actin, and cytokeratin replacement by vimentin. Although IL-1beta failed to induce A549 cells to undergo EMT, the combination of TGF-beta1 and IL-1beta showed synergy effects in cells morphology changes and the expression of mesenchymal markers. The snail expression study using RT-PCR analysis provided that loss of E-cadherin expression was associated with snail expression. Stimulation of A54 cells with TGF-beta1 plus IL-1beta revealed a higher level of snail expression. Our data showed that EMT of A549 cells might be closely associated with snail expression.
Actins/metabolism ; Cadherins/metabolism ; Cell Differentiation ; Cell Line, Tumor ; Dose-Response Relationship, Drug ; Epithelium/*metabolism ; Fluorescent Antibody Technique, Indirect ; *Gene Expression Regulation, Neoplastic ; Humans ; Keratins/metabolism ; Mesoderm/*metabolism ; Microscopy, Fluorescence ; Reverse Transcriptase Polymerase Chain Reaction ; Transcription, Genetic ; Transforming Growth Factor beta1/metabolism/*physiology ; Vimentin/metabolism

BACKGROUND: Recently, multidrug-resistant (MDR) A. baumannii has been implicated for a significant proportion of nosocominal pneumonia in many intensive care units (ICUs), and its acquisition may increase mortality and the length of stay in the ICU. Aerosolized colistin has been successfully used in patients with cystic fibrosis, but there is a lack of data regarding the use of aerosolized colistin in patients with nosocomial pneumonia. METHODS: We conducted the present study to assess the effectiveness of aerosolized colistin for the treatment of MDR A. baumannii nosocomial pneumonia. We retrospectively reviewed the medical records of 10 patients who had been hospitalized in the medical ICU and had received aerosolized colistin as a therapy for MDR A. baumannii pneumonia. RESULTS: The mean duration of aerosolized colistin therapy was 12.7+/-2.4 days. Nine (90%) of 10 patients showed a favorable response to the therapy. Follow-up cultures were available for all patients, and the responsible pathogen was completely eradicated. One patient suffered from bronchospasm, which resolved after treatment with nebulized salbutamol. CONCLUSION: Our results corroborate previous reports that aerosolized colistin may be an effective and safe choice for the treatment of nosocomial pneumonia caused by MDR A. baumannii. Larger prospective controlled clinical studies are warranted to validate further the effectiveness and safety of aerosolized colistin therapy.
Acinetobacter ; Acinetobacter baumannii ; Albuterol ; Bronchial Spasm ; Colistin ; Cystic Fibrosis ; Follow-Up Studies ; Humans ; Intensive Care Units ; Length of Stay ; Medical Records ; Pneumonia ; Retrospective Studies