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.

Clinical Characteristics and Adherence of Patients Who Were Prescribed Home Oxygen Therapy Due to Chronic Respiratory Failure in One University Hospital: Survey after National Health Insurance Coverage / 결핵및호흡기질환

BACKGROUND: Despite the benefits of home oxygen therapy in patients suffering chronic respiratory failure, previous reports in Korea revealed lower compliance to oxygen therapy and a shorter time for oxygen use than expected. However, these papers were published before oxygen therapy was covered by the national insurance system. Therefore, this study examined whether there were some changes in compliance, using time and other clinical features of home oxygen therapy after insurance coverage. METHODS: This study reviewed the medical records of patients prescribed home oxygen therapy in our hospital from November 1, 2006 to September 31, 2008. The patients were interviewed either in person or by telephone to obtain information related to oxygen therapy. RESULTS: During study period, a total 105 patients started home oxygen therapy. The mean age was 69 and 60 (57%) were male. The mean oxygen partial pressure in the arterial blood was 54.5 mmHg and oxygen saturation was 86.3%. Primary diseases that caused hypoxemia were COPD (n=64), lung cancer (n=14), Tb destroyed lung (n=12) and others. After oxygen therapy, more than 50% of patients experienced relief of their subjective dyspnea. The mean daily use of oxygen was 9.8+/-7.3 hours and oxygen was not used during activity outside of their home (mean time, 5.4+/-3.7 hours). Twenty four patients (36%) stopped using oxygen voluntarily 7+/-4.7 months after being prescribed oxygen and showed a less severe pulmonary and right heart function. The causes of stopping were subjective symptom relief (n=11), inconvenience (n=6) and others (7). CONCLUSION: The prescription of home oxygen has increased since national insurance started to cover home oxygen therapy. However, the mean time for using oxygen is still shorter than expected. During activity of outside their home, patients could not use oxygen due to the absence of portable oxygen. Overall, continuous education to change the misunderstandings about oxygen therapy, more economic support from national insurance and coverage for portable oxygen are needed to extend the oxygen use time and maintain oxygen usage.

Two Cases of Hot Tub Lung in Bodyscrubbers Working in a Public Bath / 결핵및호흡기질환

Hot tub lung has been described as a pulmonary illness associated with exposure to nontuberculous mycobacteria, mainly hot bathtub water contaminated with Mycobacterium avium complex (MAC) and hence the name. Although not entirely clear, its etiology has been thought to involve either an infection or a hypersensitivity pneumonitis secondary to MAC. Herein, we describe 2 female patients (60 and 53 years old) admitted to our hospital with hot tub lung, and both of whom worked in a public bath. Both women were initially admitted following several months of exertional dyspnea and cough. The patients had been working as body-scrubbers in a public bath for several years. Their chest CT scans showed bilateral diffuse ground-glass opacities with multifocal air-trappings and poorly defined centrilobular nodules in both lungs. Pathological findings from lung specimens revealed small non-necrotizing granuloma in the lung parenchyme with relatively normal-looking adjacent alveoli. Discontinuation of working in the public bath led to an improvement in symptoms and radiographic abnormalities, without antimycobacterial therapy.

Congenital Cystic Adenomatoid Malformation of Lung in Adults: Clinical, Pathologic and Radiologic Evaluation of Six Patients / 결핵및호흡기질환

BACKGROUND: Congenital cystic adenomatoid malformation of the lung (CCAM) is a rare congenital developmental anomaly of the lower respiratory tract. Most cases are diagnosed within the first 2 years of life, so adult presentation of CCAM is rare. We describe here six adult cases of CCAM and the patients underwent surgical resection, and all these patients were seen during a five and a half year period. The purpose of this study was to analyze the clinical, radiological and histological characteristics of adult patients with CCAM. METHODS: Through medical records analysis, we retrospectively reviewed the clinical characteristics, the chest pictures (X-ray and CT) and the histological characteristics. RESULTS: Four patients were women and the mean age at diagnosis was 23.5 years (range: 18~39 years). The major clinical presentations were lower respiratory tract infection, hemoptysis and pneumothorax. According to the chest CT scan, 5 patients had multiseptated cystic lesions with air fluid levels and one patient had multiple cavitary lesions with air fluid levels, and these lesions were surrounded by poorly defined opacities at the right upper lobe. All the patients were treated with surgical resection. 5 patients underwent open lobectomy and one patient underwent VATS lobectomy. On the pathological examination, 3 were found to be CCAM type I and 3 patients were CCAM type II, according to Stocker's classification. There was no associated malignancy on the histological studies of the surgical specimens. CONCLUSION: As CCAM can cause various respiratory complications and malignant changes, and the risks associated with surgery are extremely low, those patients who are suspected of having or who are diagnosed with CCAM should go through surgical treatment for making the correct diagnosis and administering appropriate treatment.

A Case of Recurrent Pneumonitis Caused by Bojungikgitang(Bu-Zhong-Yi-Qi-Tang) / 결핵및호흡기질환

Many classes of drug, such as antineoplastic drugs and antiarrhythmic drugs, have potential to induce interstitial lung disease. Herbal medicines are also believed to have the potential to induce pneumonitis. However, to our knowledge, there are no reports of pneumonitis caused by herbal medications in the Korean medical database. We report a case of recurrent pneumonitis caused by a self rechallenge of the Herbal medicine Bojungikgitang (Bu-Zhong-Yi-Qi-Tang : Hochu-ekki-to).

A Case of Acute Respiratory Distress Syndrome Caused By Zinc Fume Inhalation / 결핵및호흡기질환

The Inhalation of certain freshly formed metal oxides can cause metal fume fever, which is an acute, self-limiting, flu-like illness. The most common cause of this syndrome is the inhalation of zinc oxide. The inhalation of zinc oxide can lead to tracheobronchiolitis, chemical pulmonary edema or to respiratory failure and acute respiratory distress syndrome(ARDS). We encountered a 43-years-old man who developed severe dyspnea after inhaling of zinc oxide while working for 5 hours in a closed space. He was diagnosed with ARDS and was treated successfully with glucocorticoid. We report a case of ARDS caused by the inhalation of zinc fumes.

A Case of Tuberculous Pleural Effusion Developed after Percutaneous Needle Biopsy of a Solitary Pulmonary Nodule / 결핵및호흡기질환

A tuberculous pleural effusion may be a sequel to a primary infection or represent the reactivation of pulmonary tuberculosis. It is believed to result from a rupture of a subpleural caseous focus in the lung into the pleural space. It appears that delayed hypersensitivity plays a large role in the pathogenesis of a tuberculous pleural effusion. We encountered a 52 years old man with pleural effusion that developed several days after a CT guided percutaneous needle biopsy of a solitary pulmonary nodule. He was diagnosed with TB pleurisy. It is believed that his pleural effusion probably developed due to exposure of the parenchymal tuberculous focus into the pleural space during the percutaneous needle biopsy. This case might suggest one of the possible pathogeneses of tuberculous pleural effusion.

A Case of Pulmonary Edema by Idiopathic Rupture of Mitral Chordae Tendinae / 결핵및호흡기질환

The typical radiographic findings of pulmonary edema from the increased hydrostatic pressure shows centrally localized consolidation, which is known as a "butterfly or bat's wing" pattern. These terms describe the anatomic distribution of edema that uniformly involve the hilum and medulla of the lung but not the peripheral region of the lung parenchyma (cortex). We present a case of butterfly wing-like pulmonary edema on a chest radiograph by mitral regurgitation due to an idiopathic chordal rupture.

A Case of Alpha-Fetoprotein Producing Adenocarcinoma of the Lung / 결핵및호흡기질환

Alpha-fetoprotein(AFP) is a plasma protein produced in the fetal liver, yolk sac and gastrointestinal tract. The plasma level of AFP decreases markedly 1 year after birth. The AFP level is usually increased in hepatocellular carcinoma and yolk sac tumor but is rare in a primary lung cancer. We report a case of primary adenocarcinoma of lung producing high levels of AFP.