Sudden Atelectasis and Respiratory Failure in a Neutropenic Patient: Atypical Presentation of Pseudomembranous Necrotizing Bronchial Aspergillosis

Pseudomembranous necrotizing bronchial aspergillosis (PNBA) is a rare form of invasive aspergillosis with a very poor prognosis. The symptoms are non-specific, and the necrotizing plugs cause airway obstruction. Atelectasis and respiratory failure can be the initial manifestations. Recently, we treated an immunocompromised patient with PNBA, who presented with a sudden onset of atelectasis and acute respiratory failure. There were no preceding signs except for a mild cough and one febrile episode. Bronchoscopy revealed PNBA, and Aspergillus nidulans was cultured from the bronchial wash.

Ubiquitous-Severance Hospital Project: Implementation and Results / 대한의료정보학회지

OBJECTIVES: The purpose of this study was to review an implementation of u-Severance information system with focus on electronic hospital records (EHR) and to suggest future improvements. METHODS: Clinical Data Repository (CDR) of u-Severance involved implementing electronic medical records (EMR) as the basis of EHR and the management of individual health records. EHR were implemented with service enhancements extending to the clinical decision support system (CDSS) and expanding the knowledge base for research with a repository for clinical data and medical care information. RESULTS: The EMR system of Yonsei University Health Systems (YUHS) consists of HP integrity superdome servers using MS SQL as a database management system and MS Windows as its operating system. CONCLUSIONS: YUHS is a high-performing medical institution with regards to efficient management and customer satisfaction; however, after 5 years of implementation of u-Severance system, several limitations with regards to expandability and security have been identified.

ANCA-negative limited Wegener's granulomatosis / 대한내과학회지

Wegener's granulomatosis is a very rare autoimmune disease that forms inflammatory granulomas of the upper and lower respiratory tract, and causes necrotizing vasculitis by invading small vessels. Its etiology is uncertain, but antineutrophil cytoplasmic antibody (ANCA) is thought to play an important role in causing the inflammatory granuloma formation and vasculitis. The detection of ANCA is a valuable finding in diagnosing Wegener's granulomatosis. However, in the limited type of Wegener's granulomatosis, which lacks accompanying constitutional symptoms, the diagnostic value of ANCA is minimal, requiring careful interpretation of ANCA-negativity. We report a case diagnosed as limited-type Wegener's granulomatosis through repeated biopsies despite ANCA negativity.

Clinical Benefits and Complications of Cryotherapy in Advanced Lung Cancer with Central Airway Obstruction / 결핵및호흡기질환

BACKGROUND: The efficacy of the use of the interventional bronchoscope for palliation of patients with central airway obstruction has been established. In the palliative setting to alleviate central airway obstruction, the use of laser resection, electrocautery, argon plasma coagulation, photodynamic therapy and cryotherapy can provide relief of an airway obstruction. Cryotherapy is the therapeutic application of extreme cold for the local destruction of living tissue. Recently, this technique has been used for endoscopic management of central airway obstructions in Korea. We report the role and complications of the use of cryotherapy for airway obstructions in patients with advanced lung cancer. METHODS: We used a flexible cryoprobe for cryotherapy using nitrous oxide as a cryogen. The cryoprobe was applied through the working channel of a flexible fiberoptic bronchoscope. The temperature of the tip was approximately -89degrees C, and the icing time was 5~20 seconds. RESULTS: Four patients with a central airway obstruction from advanced lung cancer were treated with cryotherapy. Three of the four patients were treated successfully and the airway obstruction was improved after the cryotherapy procedure. Dyspnea, hypoxia and atelectais were improved in three cases. Two patients experienced complications-one patient experienced pneumomediastinum and the other patient experienced massive hemoptysis during the cryotherapy procedure. However, these complications resolved and did not influence mortality. CONCLUSION: This technique is effective and relatively safe for palliation of inoperable advanced lung cancer with a central airway obstruction.

Clinical Appilication of Endobornchial Cryoablation That's Performed through Flexible Bronchoscope for Treating Tracheobronchial Ostruction / 대한흉부외과학회지

BACKGROUND: Emergency airway access is essential when a patient has dyspnea that's due to tracheal or bronchial obstruction. Such methods as laser therapy and PDT are now being used for the treatment of tracheal obstruction that's due to benign diseases or nonsurgical malignant diseases. Cryotherapy is a method that uses extreme hypothermia for freezing a tumor to cause necrosis. In this study, we have evaluated the clinical effectiveness of performing endobronchial cryoablation through a flexible bronchoscope. MATERIAL AND METHOD: 10 patients with tracheal obstruction that was due to endotracheal tumors were evaluated between May 2005 and May 2007. Eight were male and the mean age of the 10 patients was 59.4+/-18.4 years. Three cases of tracheal obstruction were due to benign tumors and 7 were due to malignant tumors. The obstruction sites were 3 at the trachea, 3 at the carina and 4 at the bronchus. A flexible bronchoscope was inserted and the tumor was eliminated using a flexible cryoprobe. Follow up bronchoscopy was performed at 1 week and 1 month after cryoablation, and then we evaluated the decrease of dyspnea, the improvement of the performance and the complications of the procedures. RESULT: Complete remission was achieved in 4 patients and partial remission was achieved in 6 patients. Complications such as hemoptysis (100%), and cough (50%) were noted. Hemoptysis was spontaneously resolved in 3 to 8 days (mean: 4.9 days). A decrease in dyspnea and improvement in the performance was noted in all patients. CONCLUSION: Endobronchial stenosis plays a detrimental role in the life quality of a terminal cancer patient. Due to its simplicity and effectiveness for controlling bleeding, endobronchial cryoablation is considered to be a safe method that is clinically applicable to a wide range of tumors, including the removal of large tumors. We concluded that endobronchial cryoablation through a flexible bronchoscope is a safe, effective method for treating tracheobroncheal obstructions.

Sarcoidosis in Korea: Revisited

Sarcoidosis is a multisystem disorder of unknown origin characterized by noncaseating granulomatous inflammation. This disorder has variable clinical course, ranging from benign self - limited recovery to life-long disability. Sarcoidosis is found worldwide; however, the incidence and clinical course of the disease vary among different ethnicity and geographic regions. Especially in Korea, sarcoidosis had been known as a very rare disease until the 1st and the 2nd nationwide surveys performed in 1993 and 1998 by the Korean Academy of Tuberculosis and Respiratory Diseases. Those surveys revealed gradually increasing incidence of biopsy-proven sarcoidosis in Korea (0.027/100,000 in 1993 to 0.125/100,000 in 1998), but still very rare, and the peak age was the 30's with a female predominance (64.6%). The respiratory symptom was the most common (42%) symptom and thoracic lesion including mediastinum was the most frequently (87%) involved organ. On the other hand, cardiac involvement of this disease was very rare (0.7%). In conclusion, sarcoidosis in Korea, is a still uncommon disorder, and clinical manifestations were similar to those of western countries. Additional nationwide survey should be performed and maintained in order to investigate the correct incidence and the course of clinical manifestations of sarcoidosis in near future.

Atypical Radiological Manifestation of Pulmonary Metastatic Calcification

Metastatic pulmonary calcification refers to calcium deposition in the normal pulmonary parenchyma and this deposition is secondary to abnormal calcium metabolism. The most common radiologic manifestation consists of poorly-defined nodular opacities that are mainly seen in the upper lung zone. We present here a case of metastatic pulmonary calcification that manifested as atypical, dense, calcium deposition in airspaces within the previously existing consolidation in the bilateral lower lobes, and this process was accelerated by pneumonia-complicated sepsis in a patient with hypercalcemia that was due to hyperparathyroidism.

Two Cases of Chemical Pneumonitis Caused by Hydrogen Sulfide / 결핵및호흡기질환

Chemical pneumonitis is an occupational lung disease that's caused by the inhalation of chemical substances. Its severity depends on the characteristics of the substances, the exposure time and the susceptibility of the patients. Hydrogen sulfide is not only emitted naturally, but it also frequently found in industrial settings where it is either used as a reactant or it is a by-product of manufacturing or industrial processes. Inhalation of hydrogen sulfide causes various respiratory reactions from cough to acute respiratory failure, depending on the severity. Two pharmaceutical factory workers were admitted after being rescued from a waste water disposal site that contained hydrogen sulfide. In spite that they recovered their consciousness, they had excessive cough and mild dyspnea. The simple chest radiographs and high resolution computed tomography showed diffuse interstitial infiltrates, and hypoxemia was present. They were diagnosed as suffering from chemical pneumonitis caused by hydrogen sulfide. After conservative management that included oxygen therapy, their symptoms, hypoxemia and radiographic abnormalities were improved.

Characteristics of Mechanical Ventilation Employed in Intensive Care Units: A Multicenter Survey of Hospitals

A 1D point-prevalence study was performed to describe the characteristics of conventional mechanical ventilation in intensive care units (ICUs). In addition, a survey was conducted to determine the characteristics of ICUs. A prospective, multicenter study was performed in ICUs at 24 university hospitals. The study population consisted of 223 patients who were receiving mechanical ventilation or had been weaned off mechanical ventilation within the past 24 hr. Common indications for the initiation of mechanical ventilation included acute respiratory failure (66%), acute exacerbation of chronic respiratory failure (15%) (including tuberculosis-destroyed lung [5%]), coma (13%), and neuromuscular disorders (6%). Mechanical ventilation was delivered via an endotracheal tube in 68% of the patients, tracheostomy in 28% and facial mask with noninvasive ventilation (NIV) in 4%. NIV was used in 2 centers. In patients who had undergone tracheostomy, the procedure had been performed 16.9+/-8.1 days after intubation. Intensivists treated 29% of the patients. A need for additional educational programs regarding clinical practice in the ICU was expressed by 62% of the staff and 42% of the nurses. Tuberculosis-destroyed lung is a common indication for mechanical ventilation in acute exacerbation of chronic respiratory failure, and noninvasive ventilation was used in a limited number of ICUs.

Bilateral Pulmonary Infiltrate with Milky BAL Fluid / 결핵및호흡기질환

Exogenous lipoid pneumonia is an uncommon disease that's caused by aspirating lipid formulations. Squalene, obtained from shark liver oil, is one of the causative agent and this is commonly used by some Koreans as a health promoting medication. We report here on a case of exogenous lipoid pneumonia that developed after ingestion of squalene capsules. The case showed milky BAL fluid and multiple pulmonary consolidations.

Successful Application of Extracorporeal Membrane Oxygenation for 3 Patients in Medical Intensive Care Unit: Case Report / 대한구급학회지

Extracorporeal membrane oxygenation (ECMO) is a life-sustaining salvage therapy applied to the patient with acute heart failure or respiratory failure which is considered curable, but uncorrectable by conventional means. Recently, accumulating data has shown the survival benefit of ECMO in patients with acute fatal cardiopulmonary decompensation. Here, we report a series of cases of successful ECMO treatment in patients with acute cardiopulmonary insufficiency. Case 1: A patient with progressive respiratory failure on mechanical ventilation after pneumonectomy was managed satisfactorily using a veno-venous ECMO. Case 2: A veno-arterial ECMO was used to support a patient with vasopressor refractory septic shock. After 5 days of treatment, the patient was successfully weaned from ECMO. Case 3: A patient in cardiac arrest after the orthopedic surgery was resuscitated using a veno-arterial ECMO. Pulmonary angiography on ECMO revealed massive pulmonary thromboembolism and embolectomy was thoroughly performed under the support of ECMO.

Cyclooxygenase-2 (COX-2) Inhibitors Reduce Immune Tolerance through Indoleamine 2,3-dioxygenase (IDO)

PURPOSE : Cyclooxygenase-2 (COX-2) and its metabolite, PGE2 affect multiple tumorigenesis, including angiogenesis, invasion, and tumor-induced immune suppression. Their overexpression is association with impaired immune cell function in many tumors. Indoleamine 2,3-dioxygenase (IDO) is an emerging immuno-regulatory enzyme that can catalyze the initial rate-limiting step in tryptophan catabolism, by causing tryptophan depletion can block T lymphocyte activation, and thus, enable tumor cells to escape from immune system. Although the potential of immunosuppression associated with tumorproduced COX-2 has been suggested, the mechanism of immunosuppression in tumor immunology is not yet well defined. Thus, we hypothesized that the tumor immunity of COX-2 could be partly due to IDO-dependent immune tolerance. To test this hypothesis, we evaluated IDO expression in cancer cells treated with selective COX-2 inhibitor. MATERIALS AND METHODS : The A549 human adenocarcinoma cell line, murine Lewis lung carcinoma (LLC) cell line and C57Bl/6 mice were used for in vitro and in vivo studies. In vitro studies, A549 cells were treated with various concentrations of COX-2 inhibitor (PTPBS) or PGE2. IDO enzyme activity and protein expression were checked by IDO enzyme activity assay and Western blotting. In vivo study, the 20 mice were randomized into normal control, LLC inoculated control, and low and high selective COX-2 inhibitor (celecoxib 25 or 250 mg/kg/day) treated LLL inoculated mice groups (n=5 per group). At one month, mice were sacrificed and tumor mass was isolated for quantification of IDO expression by immunohistochemical stain and western blotting. RESULTS : In vitro studies, PTPBS treated A549 cells showed a significant decreased in IDO enzyme activity and expression but PGE2 treated A549 cells showed increased in IDO expression. In vivo studies, the tumor mass and lung metastasis were attenuated by celecoxib (respectively, p<0.05, p<0.01). Compared with the LLC inoculated control group, mice treated with celecoxib had significant reductions in IDO expression of tumor mass (IDO immunohistochemical stain and western blotting ). CONCLUSION : The present study reveals that COX-2 inhibitor serves to restore the tumor-induced IDO expression and promotes antitumor reactivity in an immunocompetent murine lung cancer model. These findings further support the suggestion that COX-2 inhibitor is a potential pharmacological immunotherapy in cancer

Basal Serum Cortisol Levels are not Predictive of Response to Corticotropin but Have Prognostic Significance in Patients with Septic Shock

Because high levels of cortisol are frequently observed in patients with septic shock, low levels of serum cortisol are considered indicative of relative adrenal insufficiency (RAI). This study was performed to investigate whether pretest clinical characteristics, including basal serum cortisol levels, are predictive of serum cortisol response to corticotropin and whether basal cortisol levels have a prognostic significance in patients with septic shock. We performed a retrospective analysis of 68 patients with septic shock who underwent short corticotropin stimulation testing. RAI was defined as an increase in cortisol level or =30 microgram/dL) was significantly associated with in-hospital mortality. In conclusion, our data suggest that basal serum cortisol levels are not predictive of serum cortisol response to corticotropin but have a significant prognostic value in patients with septic shock.

A Case of Portopulmonary Hypertension Associated with Primary Biliary Cirrhosis / 결핵및호흡기질환

Portopulmonary hypertension (PPHTN) is a clinically and pathophysiologically distinct complication of advanced liver disease. PPHTN is characterized by the development of pulmonary arterial hypertension in association with advanced hepatic disease-related portal hypertension. A characteristic feature of PPHTN is an obstruction to the pulmonary artery flow caused by vasoconstriction, the proliferation of the endothelium and smooth muscle components of the vascular wall, as well as in situ thrombosis. This disorder is commonly underdiagnosed but the clinical implications are significant because it has substantial effects on survival and requires special treatment. We report a case of portopulmonary hypertension in a 53-year-old woman with primary biliary cirrhosis who presented with exertional dyspnea.

Potential use of an anticancer drug gefinitib, an EGFR inhibitor, on allergic airway inflammation

The EGFR plays an essential role in goblet cell hyperplasia and mucus hypersecretion. EGFR has an intrinsic tyrosine kinase activity that, when activated, induces the production of MUC5AC through the signaling kinase cascade in the airway epithelium. We have investigated the effects of an EGFR tyrosine kinase inhibitor, gefitinib, on ovalbumin (OVA)-induced, allergic inflammation in airway epithelia of mice. OVA-sensitized mice were pretreated with gefitinib at two different doses (12.5 and 50 mg/kg) and then challenged with OVA. The OVA challenge increased the total cell count and eosinophil count in bronchoalveolar lavage fluid (BALF), as well as the concentrations of T-helper2 (Th2) cytokines, such as IL-4 and IL-13, overall eosinophil recruitment in the lung tissue and airway hyperresponsiveness (AHR). Pretreatment with gefitinib reduced the inflammatory cell counts and released cytokine concentrations (IL-4 and IL-13) in BALF, as well as eosinophil recruitment in the lungs and AHR, in a dose-dependent manner. This was associated with decreased EGFR and Akt phosphorylation. We showed that gefitnib inhibits EGFR and phosphoinositol 3'-kinase (PI3K)/Akt activation which were activated in OVA sensitized mice. These findings suggest that inhibitors of the EGFR cascade may have a role in the treatment of asthma.

The Effects of Ethyl Pyruvate on Lipopolysaccharide-induced Acute Lung Injury / 결핵및호흡기질환

BACKGROUND: Ethyl pyruvate (EP) is a derivative of pyruvate that has recently been identified by both various in vitro and in vivo studies to have antioxidant and anti-inflammatory effects. The aim of this study was to determine the effect of EP on lipopolysaccharide (LPS)-induced acute lung injury (ALI). METHODS: 5 weeks old, male BALB/c mice were used. ALI was induced by an intratracheal instillation of LPS 0.5mg/Kg/50microliter of saline. The mice were divided into the control, LPS, EP+LPS, and LPS+EP groups. In the control group, balanced salt solution was injected intraperitoneally 30 minutes before or 9 hours after the intratracheal instillation of saline. In the LPS group, a balanced salt solution was also injected intraperitoneally 30 minutes before or 9 hours after instillation the LPS. In the EP+LPS group, 40mg/Kg of EP was injected 30 minutes before LPS instillation. In the LPS+EP group, 40mg/Kg of EP was injected 9 hours after LPS instillation. The TNF-alpha and IL-6 concentrations in the bronchoalveolar lavage fluid (BALF), and that of NF-KappaB in the lung tissue were measured in the control, LPS and EP+LPS groups at 6 hours after instillation of saline or LPS, and the ALI score and myeloperoxidase (MPO) activity were measured in all four groups 24 and 48 hours after LPS instillation, respectively. RESULTS: The TNF-alpha and IL-6 concentrations were significantly lower in the EP+LPS group than in the LPS group (p<0.05). The changes in the concentration of these inflammatory cytokines were strongly correlated with that of NF-kappaB (p<0.01). The ALI scores were significantly lower in the EP+LPS and LPS+EP groups compared with the LPS group (p<0.05). In the EP+LPS group, the MPO activity was significantly lower than the LPS group (p=0.019). CONCLUSION: EP, either administered before or after LPS instillation, has protective effects against the pathogenesis of LPS-induced ALI. EP has potential theurapeutic effects on LPS-induced ALI.

Two Cases of Transfusion Related Acute Lung Injury / 결핵및호흡기질환

Transfusion related acute lung injury (TRALI) is a serious, potentially life-threatening complication of transfusion therapy that is sometimes under diagnosed and under reported. Patients with TRALI present with dyspnea/respiratory distress and fever. The symptoms, signs and chest radiological findings in TRALI are similar to transfusion associated circulatory overload, which makes it is difficult to distinguish it from circulatory overload. Although the mortality rate in cases of TRALI is relatively low, TRALI is the third most common cause of fatal transfusion reactions next to ABO blood type incompatibility and hepatitis. Mild-to-moderate cases of TRALI may be misdiagnosed as volume overload. Recently, we encountered two cases where the patients suffered from dyspnea and fever after a transfusion. and review of the relevant literature.

The Effects of Intra-Abdominal Hypertension on the Prognosis of Critically Ill Patients in the Intensive Care Unit (ICU) / 결핵및호흡기질환

BACKGROUND: Intra-abdominal hypertension (IAH) is defined as the presence of either an intra-abdominal pressure (IAP) > or = 12 mmHg or an abdominal perfusion pressure (APP = mean arterial pressure - IAP) or = 20 mmHg together with organ failure. The purpose of this study was to investigate the prevalence of IAH and ACS on the day of admission and the effects of these maladies on the prognosis of critically ill patients in the ICU. METHODS: At the day of admission to the ICU, the IAP was recorded by measuring the intravesicular pressure via a Foley catheter. The APACHE II and III scores were checked and SAPS II was also scored during the days the patients were in the ICU. The primary end point was the prevalence of IAH and ACS at the day of admission and the correlation between them with the 28-days mortality rate. The measurement of IAP continued until the 7th day or the day when the patient was transferred to the general ward before 7th day, unless the patient died or a Foley catheter was removed before 7th day. Patients were observed until death or the 28th day. RESULTS: A total of 111 patients were enrolled. At the day of admission, the prevalence of IAH and ACS were 47.7% and 15.3%, respectively and the mean IAP was 15.1+/-8.5 mmHg. The rates of IAH for the survivor and the non-survivor groups were 56.5% and 71.4%, respectively, and these were not significantly different (p=0.593). Yet the rates of ACS between these two groups were significantly different (4/62, 6.5% vs. 13/49, 26.5%; Odds Ratio = 5.24, 95% CI = 1.58-17.30, p=0.004). CONCLUSION: In the present study, the prevalence of IAH was 47.7% and the prevalence of ACS was 15.3% on the day of admission. ACS was associated with a poor outcome for the critically ill patients in the ICU.

Partial Interferon-gamma Receptor Deficiency in Patients with Disseminated Tuberculosis / 결핵및호흡기질환

BACKGROUND: Interferon-gamma (IFN-gamma) is essential in the immune response to mycobacterial infections, and a complete or partial deficiency in the IFN-gamma receptor 1 (IFNgammaR1) or the IFN-gamma receptor 2 (IFNgammaR2) have been reported to confer susceptibility to a disseminated infection with nontuberculous mycobacteria. However, similar mutations in the IFN-gamma receptor have not been specifically examined in the patients with clinical tuberculosis. METHODS: This study searched for mutations in the IFN-gamma receptor gene that resulted in a partial IFN-gamma receptor deficiency in six patients with disseminated tuberculosis. The previously identified IFNgammaR1 and IFNgammaR2 coding regions were sequenced after amplification. RESULTS: There was no partial IFNgammaR1 deficiency including a homozygous recessive missense mutation causing an amino-acid substitution in the extracellular domain of the receptor (I87T) and a hotspot for small deletions (818delT, 818del4, 818insA) found in any of the patients. In addition, a partial IFNgammaR2 deficiency of the homozygous missense mutation (R114C) was not found in any of the patients. CONCLUSION: Genetic defects causing a partial IFN-gamma receptor deficiency were not identified in our patients with disseminated tuberculosis.

Etiologic Distribution, Diagnostic Tests and Treatment in Prosp??ectively Registered Patients with DILD for Two Years in a Tertiary Medical Center / 결핵및호흡기질환

INTRODUCTION: Diffuse interstitial lung diseases (DILD) comprise of a large group of lung diseases with diverse etiologies. They are classified into four categories based on the etiology and pathological findings. In Korea, epidemiological data on DILD has never been reported in a prospective manner. METHOD: From May 2002 to April 2004, total 487 patients with DILD were prospectively registered at Samsung Medical Center. The prospective observational analysis of the etiologies, its classification based on 2002 ATS/ERS (American Thoracic Society/European Respiratory Society) guidelines, as well as diagnostic tests and the retrospective analysis of the treatment modalities were carried out. Any infectious and malignant causes were excluded. RESULTS: 1) The patients were classified into idiopathic interstitial pneumonia (IIP) in 269 patients (55.2%), known causes of DILD in 168 patients (34.5%), sarcoidosis in 27 patients (5.5%), other forms of DILD in 14 patients (2.9%), and undetermined DILD in 9 patients (1.9%). 2) The diagnostic test showed that most patients had undergone chest high resolution computed tomography (HRCT) and pulmonary function test (PFT) (97%, 89%). Transbronchial lung biopsy (TBLB) and surgical lung biopsy (SLB) were performed in limited patients (38%, 29%). 3) Among 269 patients with IIP, 220 (82%) had idiopathic pulmonary fibrosis (IPF) while 23 (9%) had nonspecific interstitial pneumonia. SLB was carried out in 36% of patients with IIP. 4) Symptomatic supportive care was given to 67% of IPF, but specific medical treatment including corticosteroids was administered to 89% of non-IPF patients. CONCLUSION: A nationwide registry of DILD patients is required to determine the annual incidence, etiology, and practice pattern of diagnosis and treatment in Korea.