A Nationwide Survey of Lymphangioleiomyomatosis in Korea: Recent Increase in Newly Diagnosed Patients

In 2007, the Korean Interstitial Lung Disease Society had collected clinical data of patients who have diagnosed as Lymphangioleiomyomatosis (LAM) since 1990 through nationwide survey, which showed that LAM patients had increased sharply after 2004. The present study was performed to show the clinical features of Korean patients with LAM, and to establish the reason for the recent increase in the diagnosis. All 63 patients were women and the mean age at diagnosis was 36 yr. The most common presenting symptom was dyspnea and 8 patients had tuberous sclerosis complex. The survival rate at 5 yr after diagnosis was 84%. Compared with patients diagnosed after 2004 (n=34), the patients diagnosed before 2004 (n=29) complained with dyspnea more (P=0.016) and had lower FEV1% predicted (P=0.003), and DLco% predicted (P=0.042). The higher proportion of patients diagnosed after 2004 showed the normal chest radiography, and they were detected by routine chest CT screening (P=0.016). This study showed that clinical features of Korean patients with LAM were not different from those reported elsewhere. It is concluded that the reason for the increase of newly diagnosed patients is the result of increase in detection of the early stage LAM by the widespread use of chest CT screening.

Treatment Outcomes and Prognostic Factors in Patients with Multidrug-Resistant Tuberculosis in Korean Private Hospitals / 결핵및호흡기질환

BACKGROUND: The increasing rate of drug-resistant tuberculosis (TB) is a threat to the public health and TB control. In Korea, about 75~80% of TB patients are treated in private hospitals and the rate has been continuously increasing since 2000. METHODS: On a retrospective basis, we enrolled 170 newly diagnosed with or retreated for multidrug-resistant TB (MDR-TB) in 2004 from 21 private hospitals. We extracted the following demographics and treatment history from patient medical records: initial treatment outcomes, cumulative survival rates, treatment outcomes, and prognostic factors. RESULTS: Of the 170 patients, the majority were male (64.1%), the mean age was 44.5 years old, and mean body-mass-index was 20.2 kg/m2. None of the patients tested positive for HIV. Eleven (6.5%) were confirmed to have extensively drug-resistant TB (XDR-TB) at treatment initiation. Treatment success rates were not different between XDR-TB (36.4%, 4/11) and non-XDR MDR-TB (51.6%, 82/159). Default rate was high, 21.8% (37/170). Far advanced disease on X-ray was a significant negative predictor of treatment success; advanced disease and low BMI were risk factors for all-cause mortality. CONCLUSION: In private hospitals in Korea, the proportion of XDR-TB in MDR-TB was comparable to previous data. The treatment success rate of MDR-/XDR-TB remains poor and the failure rate was quite high. Adequate TB control policies should be strengthened to prevent the further development and spread of MDR-/XDR-TB in Korea.

Clinical Features of Pulmonary Langerhans Cell Histiocytosis in Korea / 결핵및호흡기질환

BACKGROUND: Pulmonary Langerhans cell histiocytosis (PLCH) is characterized by a proliferation of Langerhans cells and this results in granulomas that involve multiple organs of the body. Because the incidence of PLCH is very low in Korea and worldwide, collecting the clinical data of patients with PLCH nationwide is needed to determine the clinical features of Korean patients with PLCH. METHODS: The patients with PLCH confirmed by biopsy at any body site were included and the patients should have lung lesions present. A questionnaire that had items on the symptoms, lung function tests, the roentgenographic findings and the treatment was collected retrospectively at a Korean ILD Research Meeting. RESULTS: A total of 56 cases were collected. The number of males and females was 48 and 8, respectively, and their median age was 43 years (range: 18~67 years). The patients were current or ex-smokers in 79% of the cases. The most frequent symptom was coughing (39%), followed in decreasing order by dyspnea (38%), sputum (20%) and chest pain (20%). Pneumothorax was observed in 16 (29%) patients. Lung function tests showed a normal, restrictive, mixed or obstructive pattern in 26 (61%), 7 (16%), 7 (16%) and 3 patients (7%), respectively. Nodular-cystic lesion was most frequently observed in 59% of the patients on HRCT. The lung lesions were located in the middle and upper lobes in almost the cases. The median follow-up period was 90 months (range: 1~180 months) and only two patients died during this period. CONCLUSION: This study provides a national survey of the patients with PLCH during a long follow-up period.

A Case of Cholethorax following Percutaneous Transhepatic Cholangioscopy / 결핵및호흡기질환

Cholethorax (bilious pleural effusion) is an extravasation of bile into the thoracic cavity via a pleurobiliary fistula (and also a bronchobiliary fistula). It is an extremely rare complication of thoraco-abdominal injuries. It can be caused by congenital anomaly and also by hepatobiliary trauma, severe infection or iatrogenic procedures. The definitive diagnosis is made with aspiration of bilious fluid from the pleural space during thoracentesis, by finding a fistulous tract during endoscopic retrograde cholangiopancreatography (ERCP) or cholagioscopy, or with finding an abnormal pleural accumulation of radioisotope during hepatobiliary nuclear imaging. Its symptoms include coughing, fever, dyspnea and pleuritc chest pain. Herein we report on a case of cholethorax following performance of percutaneous transhepatic cholangioscopy (PTCS) to remove incidentally discovered common bile duct (CBD) stones.

A Case Report of Disseminated Extranodal Marginal Zone B-Cell Lymphoma of MALT Manifested by Solitary Pulmonary Nodule / 결핵및호흡기질환

Extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) is usually indolent. Although it was reported recently that about 20-30% cases of MALT lymphoma presented with a disseminated disease at diagnosis, it was described as a disease localized at diagnosis and remaining stable for a prolonged period. However, only a few cases of MALT lymphoma involved the lung and gastrointestinal tract all at once. We report a case of a 73-year-old man with disseminated MALT lymphoma. He presented with non-productive cough, initial chest radiograph showed a nodule in the right lower lobe. The diagnosis of stage IV MALT lymphoma was made by CT scan, video-assisted thoracoscopic excisional biopy, gastrofiberscopic biopsy and bone marrow biopsy. The lymphoma involved the lung, stomach and bone marrow at the time of diagnosis. Because he refused chemotherapy, he discharged after Helicobacter pylori eradication without chemotherapy. Regular follow-up examination did not show any evidence of disease progression over 22 months.

Numerous Bilateral Radiographically Dense Branching Opacities after Vertebroplasty with Polymethylmethacrylate / 결핵및호흡기질환

Percutaneous vertebroplasty consists of the percutaneous injection of polymethylmethacrylate (PMMA) cement into a collapsed vertebral body in order to obtain pain relief and mechanically strengthen the vertebral body. This procedure is now extensively used in treating osteoporotic vertebral compression fracture. It is an efficient treatment, but it is not free of complications. Most complications after vertebroplasty are associated with PMMA leakage. Pulmonary embolism of PMMA is rare, but this can occur when there is a failure to recognize venous migration of cement early during the procedure. We encountered a case of a patient with asymptomatic pulmonary embolism because of PMMA after percutaneous vertebroplasty. Chest X-ray and CT scanning revealed numerous tubular branching opacities that corresponded to the pulmonary vessels at the segmental and subsegmental levels.

Development of the Home-Based Pulmonary Rehabilitation Program for Patients with Chronic Lung Disease / 결핵

BACKGROUND:Even though it is well known that pulmonary rehabilitation (PR) improves exercise capacity, and the quality of life, in patients with chronic lung disease, not many patients can attend hospital based intensive PR in Korea. The purpose of this study was to develop a method for a home-based PR program, and study its effectiveness. METHODS:Twenty patients with chronic lung diseases were randomly divided into two groups : a home PR group comprising of 10 male patients, with a mean age of 70 years, and a control group comprisiong of 10 male patients, with a mean age of 65 years. We developed exercise programs, depending on the exercise capacity of each patient, which were easy to do at home. The PR program consisted of a 12 week period of enforced aerobic (mostly walking) and muscle strengthening exercises, as prescribed by the exercise specialist, in accordance with the functional capacity of the patient. In addition to the education, nutritional and psychiatric consultation was undertaken, and respiratory muscle training arranged. Patients visited hospital every 2 weeks for evaluation and exercise prescription. RESULTS: All patients finished the 12 week course of therapy. Following the home PR, the endurance times and work capacity of the upper and lower extremities were significantly increased in the treatment group in comparison to the controls. The six minute working (Eds note : should) 'working' read 'walking'?) distance was increased from 465+/-60m to 508+/-37m and the maximal inspiratory pressure from 72.8+/-27.2cmH2O to 91.4+/-30.9 cmH2O. The quality of life, as assessed by St Georges Respiratory Questionnaire (SGRQ), was also improved following PR. (Eds note : do you have figures for before and after, and a reference for the SGRQ? i.e. for the main paper.) CONCLUSION: The home PR program we developed seemed to be applicable, and effective, to most of the patients with chronic lung diseases in the study.

Development of the Home-Based Pulmonary Rehabilitation Program for Patients with Chronic Lung Disease / 결핵

BACKGROUND:Even though it is well known that pulmonary rehabilitation (PR) improves exercise capacity, and the quality of life, in patients with chronic lung disease, not many patients can attend hospital based intensive PR in Korea. The purpose of this study was to develop a method for a home-based PR program, and study its effectiveness. METHODS:Twenty patients with chronic lung diseases were randomly divided into two groups : a home PR group comprising of 10 male patients, with a mean age of 70 years, and a control group comprisiong of 10 male patients, with a mean age of 65 years. We developed exercise programs, depending on the exercise capacity of each patient, which were easy to do at home. The PR program consisted of a 12 week period of enforced aerobic (mostly walking) and muscle strengthening exercises, as prescribed by the exercise specialist, in accordance with the functional capacity of the patient. In addition to the education, nutritional and psychiatric consultation was undertaken, and respiratory muscle training arranged. Patients visited hospital every 2 weeks for evaluation and exercise prescription. RESULTS: All patients finished the 12 week course of therapy. Following the home PR, the endurance times and work capacity of the upper and lower extremities were significantly increased in the treatment group in comparison to the controls. The six minute working (Eds note : should) 'working' read 'walking'?) distance was increased from 465+/-60m to 508+/-37m and the maximal inspiratory pressure from 72.8+/-27.2cmH2O to 91.4+/-30.9 cmH2O. The quality of life, as assessed by St Georges Respiratory Questionnaire (SGRQ), was also improved following PR. (Eds note : do you have figures for before and after, and a reference for the SGRQ? i.e. for the main paper.) CONCLUSION: The home PR program we developed seemed to be applicable, and effective, to most of the patients with chronic lung diseases in the study.

The Effect of Steroid Therapy in Patients with Late ARDS / 결핵

BACKGROUND: The mortality from acute respiratory distress syndrome(ARDS) in the late stage, which is characterized by progressive pulmonary fibroproliferation, is >or=80%. Although previous prospective trials failed to show a survival benefit of steroid therapy in early ARDS, recently, a few of reports have described the survival benefit of the long-term use of steroid in patients with late ARDS. In this study, we analyzed the effect of steroid therapy on patietns with late ARDS. In this study, we analyzed the effect of steroid therapy on patients with late ARDS retrospectively in a single. Medral intensive care unit. METHODS: Over a 3-year period, the medical records of 48 ARDS patients who had veen on mechanical ventilation more than 8 days were reviewed. 14 patients were treated by the long-term use of methylprednisolone and another 34 patients served as a control. Both groups were comparable regarding clinical and physiologic data lung injury score(LIS), multiple organ failure score, APACHE III and SAPS II score. Because steroid was instituted after 8 days of advanced mechanical ventilatory support in average, we arbitrarily defined the 8th day of ARDS as first day of the study. RESULTS: Initially, the group had similar PF(PaO2/FiO2)ratio, LIS, APA CHE III and SAPS II score. By 7th day after the start of steroid therapy, there were significant improvements in PF ratio, LIS, APACHE III and SAPS II score. The mortality in the steroid treated group was significantly lower(42.9% vs 73.5%, p<0.05). CONCLUSIONS: Although the data of this study was retrospective and was not randomized, in order to improve the patients's outcomes, steroid therapy should be considered in late ARDS patients. However, prospective trials are needed to define the indication and the effect of steroid therapy in late ARDS.

A case of Idiopathic CD4+ T-Lymphocytopenia with disseminated Mycobacterium kansasii infection and Pulmonary alveolar proteinosis / 결핵

Idiopathic CD4+ T-lymphocytopenia is defined as a depletion of CD4+ lymphocytes below 300/mm 3 in the absence of HIV infection or other known causes of immunodeficiency. Many infectious diseases have been reported to be associated with idiopathic CD4+ T-lymphocytopenia, and there have also been a few cases of mycobacterial infection in idiopathic CD4+ T-lymphocytopenia. Until now, it has been unclear as to whether CD4+ T-lymphocytopenia is a predisposing factor for or a consequence of the mycobacterial infection. Pulmonary alveolar proteinosis is an uncommon disease characterized by the intraalveolar deposition of amorphous granular material that stains positive with PAS, and its association with mycobacterial infection has rarely been reported. Recently, we experienced a previously healthy young man who had been diagnosed as idiopathic CD4+ T-lymphocytopenia with disseminated mycobacterium kansasii infection and pulmonary alveolar proteinosis, and report this case.

TNF-alpha, TGF-beta, and fibrinolytic parameters in tuberculous and malignant pleural effusions / 결핵

BACKGROUND: Residual pleural thickening(RPT) develops in about 50% of tuberculous pleurisy(PLTB). Some reports have suggested that elevated TNF-α and impaired fibrinolysis could be the cause of RPT, but until now, the mechanism and predictors of RPT have not been well known. TGF-β has been known to promote fibrogenesis and is increased in tuberculous pleural fluid(PF). PLTB and malignant pleurisy(PLMAL) manifest lymphocyte-dominant exudative pleural effusion, and it has clinical implications in the differentiation of the two diseases, based on the findings of pleural effusion. We performed this study to compare pleural fluid TNF-α, TGF-β, and fibrinolytic parameters between PLTB and PLMAL, and to find the predictors of RPT in PLTB. METHODS: Thirty-five PLTB and 14 PLMAL patients who were admitted to the Asan Medical Center from February 1997 to August 1999 were enrolled. All PLTB patients were prescribed a primary, short-course, anti-tuberculosis regimen. TNF-α, tissue plasminogen activator (tPA), plasminogen activator inhibitor 1 (PAI-1), plasminogen, α2-antiplasmin, and D-dimer were measured in both PF and PB, TGF-β was measured only in PF. Clinical characteristics, TNF-α, TGF-β, and fibrinolytic parameters were compared between patients with RPT less than 2 mm and patients with more than 2 mm of the thirty patients who completed the anti-tuberculosis treatment. RESULTS: The levels of TNF-α, tPA, PAI-1, plasminogen, α2-antiplasmin, and D-dimer in PF were higher than those in peripheral blood (PB) in PLTB, whereas only plasminogen, α2-antiplasmin, and D-dimer were higher in PF than in PB in PLMAL. Pleural fluid TNF-α, TGF-β, PAI-1, plasminogen, α2-antiplasmin were increased in PLTB compared with PLMAL, but these factors did not show any further advantages over ADA in differentiation between PLTB and PLMAL. TNF-α, TGF-β, and fibrinolytic parameters did not show any differences between patients with RPT less than 2 mm and patients with RPT more than 2 mm. CONCLUSION: Our data suggest that TNF-α, TGF-β, and fibrinolytic parameters may play some role for the development of RPT in PLTB, but they failed to predict the occurrence of RPT in PLTB. Also these parameters did not seem to have any advantages over ADA in differentiating between two diseases.

Lung complications after allogenic bone marrow transplantation / 결핵

BACKGROUND: The occurrence of lung complications after allogenic bone marrow transplantation(BMT) has been reported as 40-60 percent. The risk factors for lung complications are whole body irradiation, high dose chemotherapy, graft versus host disease, old age and CMV infection. The prevalence of graft versus host disease is less in Korea than in Western countries, but frequency of CMV infection is higher. Therefore, the pattern of lung complications may be different in Korea from those in Western countries. METHODS: A retrospective cohort study was performed on one hundred consecutive adult patients who underwent allogenic bone marrow transplantation from December, 1993 to May, 1999 at Asan Medical Center. Lung complications were divided into two groups by the time of development, within 30days (pre-engraftment) and beyond 30 days (post-engraftment), and then subdivided into infectious and non-infectious complication. Infectious complications were defined as having the organism in blood, BAL fluid, pleural fluid or sputum, or compatible clinical findings in patients, which improved with antibiotics or an anti-fungal therapy. RESULT: 1) Eighty three episodes of lung complications had occurred in 54 patients. 2) Within thirty days after BMT, non-infectious complications were more common than infetions, but this pattern was reversed after 30days. After one year post-BMT, there was no infectious complication except in cases of recurrence of underlying disease or development of chronic GVHD. 3) Among the non-infectious complication, pleural effusion (27 episodes) was most common, followed by pulmonary edema(8 episodes), bronchiolitis obliterans(2 episodes), diffuse alveolar hemorrhage(1 episode) and bronchiloitis obliterans with organizing pneumonia(1 episode), 4) The infectious complications were pneumonia(bacterial:9 episodes, viral:4 episodes, fungal:5 episodes, pneumocystis carinii:1 episode), pulmonary tubercoulosis(3 episodes) and tuberculous pleurisy(3 episodes). 5) Lung complications were more frequent in CMV positive patients and in patients with delayed recovery of neutrophil count. 6) The mortality was higher in the patients with lung complications. CONCLUSION: Lung complications developed in 54% after allogenic BMT and were associated with higher mortality.

A case of Behcet's bisease with pulmonary infarction / 대한내과학회지

Behcet's disease is a nonspecific vasculitis characterized by exacerbations and remissions of unpredictictable duration. The commom clinical features in most patients are orogenital ulcers, uveitis and skin lesions-especially erythema nodosum-like lesions or positive pathergy test. Among the systemic manifestations of Behcet's disease, pulmonary involvement is very rare and only a few cases have been documented. The main event of pulmonary involvement is vasculitis forming multilocular aneurysms and thrombosis of the pulmonary artery. The commom pulmonary manifestations are hemoptysis, dyspnea, pleuritic chest pain, cough and fever. The prognosis of pulmonary vasculitis is very poor. A 30-year-old male patient with Behcet's disease presented with fever and dyspnea for 10 days. In the past years, his vision of the left eye was lost due to chorioretinitis. He took right hemicolectomy because of the perforation of colonic ulcer. Also he has been suffered from left hemiparesis due to multiple cerebral infarction. We describe a case with Behcet's disease with pulmonary infarction improved with prednisolone and cyclosporine.