• Clinical and Experimental Pediatrics
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• v.53 no.6
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• pp.711-717
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• 2010

Purpose: Orphan lung diseases are defined as lung diseases with a prevalence of 1 or less in 2,000 individuals. Despite an increase in the numbers of patients with such diseases, few studies on Korean children have appeared. To obtain epidemiologic and demographic data on these diseases, we systematically reviewed reports on pediatric orphan lung diseases in Korea over the last 50 years. Methods: We reviewed 223 articles that have appeared since 1958 on orphan lung diseases in Korean children. These articles described a total of 519 patients aged between 0 and 18 years. We classified patients by year of publication, diagnosis, geographic region, and journal. Results: Of 519 patients, 401 had congenital cystic lung diseases and 66 had bronchiolitis obliterans. About 80% of patients were described in reports published in three journals, Pediatric Allergy and Respiratory Disease (Korea), the Korean Journal of Pediatrics, and the Korean Journal of Thoracic and Cardiovascular Surgery, in which papers on 157 (30.2%), 138 (26.6%), and 111 (21.4%) patients appeared, respectively. The frequency of publication of case reports has increased since 1990. Of the 519 patients, 401 (77.3%) were from Seoul/Gyeonggi-do and 72 (13.9%) from Busan/Gyeongsangnam-do. Conclusion: The prevalence of pediatric orphan lung disease has increased since 1990, and some provinces of Korea have a higher incidence of these diseases than do others. Studies exploring the incidence of pediatric orphan lung diseases in Korea are needed for effective disease management.

• Journal of Chest Surgery
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• v.49 no.3
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• pp.157-164
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• 2016

Background: Heart-lung transplantation (HLT) has provided hope to patients with end-stage lung disease and irreversible heart dysfunction. We reviewed the clinical outcomes of 10 patients who underwent heart-lung transplantation at Asan Medical Center. Methods: Between July 2010 and August 2014, a total of 11 patients underwent HLT at Asan Medical Center. After excluding one patient who underwent concomitant liver transplantation, 10 patients were enrolled in our study. We reviewed the demographics of the donors and the recipients' baseline information, survival rate, cause of death, and postoperative complications. All patients underwent follow-up, with a mean duration of $26.1{\pm}16.7months$. Results: Early death occurred in two patients (20%) due to septic shock. Late death occurred in three patients (38%) due to bronchiolitis obliterans (n=2) and septic shock (n=1), although these patients survived for 22, 28, and 42 months, respectively. The actuarial survival rates at one year, two years, and three years after HLT were 80%, 67%, and 53%, respectively. Conclusion: HLT is a procedure that is rarely performed in Korea, even in medical centers with large heart and lung transplant programs. In order to achieve acceptable clinical outcomes, it is critical to carefully choose the donor and the recipient and to be certain that all aspects of the transplant procedure are planned in advance with the greatest care.

• Journal of Chest Surgery
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• v.51 no.1
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• pp.22-28
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• 2018

Background: Lung transplantation is a life-saving procedure in patients with end-stage lung disease, and is increasingly performed in Korea. Methods: We retrospectively evaluated the outcomes of patients who received a lung transplant at Asan Medical Center between January 2008 and December 2016. Thirteen of 54 patients experienced multiorgan transplantation; the remaining 41 who received only lung grafts were included. Results: The mean age of the lung transplant recipients was 44.6 years; 27 were men and 14 were women. The most frequent reasons were idiopathic interstitial pneumonia (21 of 41 patients, 51.2%), interstitial lung disease (9 of 41, 22.0%), and bronchiolitis obliterans after bone marrow transplantation (7 of 41, 17.1%). The median waiting time was 47 days, and many patients received preoperative intensive care (27 of 41, 65.9%), ventilator support (26 of 41, 63.4%), or extracorporeal life support (19 of 41, 46.3%). All 41 patients received bilateral lung grafts. Ten deaths occurred (24.3%), including 5 cases of early mortality (12.2%) and 5 cases of late mortality (12.2%). The 1-, 3-, and 5-year survival rates were 78.9%, 74.2%, and 69.3%, respectively. Conclusion: Despite a high percentage of patients who required preoperative intensive care, the transplantation outcomes were acceptable.

• Tuberculosis and Respiratory Diseases
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• v.62 no.6
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• pp.540-544
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• 2007

The patient is a 62-year-old man with known diabetes mellitus who presented with a two-weeks-history of dyspnea, cough, and fever. He was diagnosed with a lung abscess in the right upper lobe and was treated with intravenous antibiotics. The patient's clinical and radiological findings improved within seven days after medical treatment. However, newly developed ground-glass opacity and infiltrations were observed in the right lower lung. Fourteen days after admission, the patient's symptoms and imaging finding became aggravated despite trestment with susceptible antibiotics for lung abscess. Trans-bronchial lung biopsy (TBLB) was performed in the lateral basal segment of the right lower lobe of the lung. A histologic photomicrograph showed organizing pneumonia, also called bronchiolitis obliterans with organizing pneumonia(BOOP), that became more definite as the terminal bronchioles and alveoli became occluded with masses of inflammatory cells and fibrotic tissue. The clinical symptoms and radiograph findings resolved quickly with prednisone treatment. We report a case of secondary organizing pneumonia diagnosed after TBLB following lung abscess treatment and provide a review of the literature.

• Allergy, Asthma & Respiratory Disease
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• v.6 no.6
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• pp.295-302
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• 2018

Purpose: It is thought that Mycoplasma pneumoniae infection is more prevalent and causes more severe pneumonia in school-age children and young adults than in preschool children; however, recent studies suggest that the infection may be underdiagnosed and more severe in preschool children. This study investigated the clinical characteristics of Mycoplasma pneumoniae pneumonia (MPP) and the risk factors of refractory MPP (RMPP) by age. Methods: We retrospectively reviewed the medical records of 353 children admitted due to MPP from January 2015 to December 2016. Demographics, clinical information, laboratory data and radiological findings were collected from all patients in this study. The patients were divided into 2 groups by the age of 6 years. Also, both preschool (< 6 years old) and school-age (${\geq}6$ years old) children were divided into RMPP and non-RMPP patients. Results: Total febrile days, febrile days before admission and the duration of macrolide antibiotic therapy were significantly longer in school-age children than in preschool children. School-age children had significantly greater risk of lobar consolidation (P=0.036), pleural effusion (P=0.001) and extrapulmonary complications (P=0.019). Necrotizing pneumonia and bronchiolitis obliterans tended to occur more frequently in preschool children than in school-age children. In both preschool and school-age children, lactate dehydrogenase (LDH) levels were significantly higher in RMPP patients than in non-RMPP patients. In preschool children, LDH > 722 IU/L (odds ratio [OR], 3.02; 95% confidence interval [CI], 1.44-6.50) and ferritin > 177 ng/mL (OR, 5.38; 95% CI, 1.61-19.49) were significant risk factors for RMPP, while LDH > 645 IU/L (OR, 4.12; 95% CI, 1.64-10.97) and ferritin > 166 ng/mL (OR, 5.51; 95% CI, 1.59-22.32) were so in school-age children. Conclusion: Clinical features of MPP were significantly different between preschool and school-age children. LDH and ferritin may be significant factors of RMPP in preschool and school-age children.

• Journal of the Korean Society of Radiology
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• v.81 no.4
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• pp.756-769
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• 2020

Among the various emergency diseases in children, acute pulmonary and airway disease are common clinical conditions encountered by radiologists, and the first imaging modality is chest radiography. Therefore, it is important to be familiar with these diseases and their imaging findings. In this article, we review pneumonia and mimickers of acute pulmonary disease. For acute airway disease, we reviewed croup, acute epiglottitis, tracheomalacia, asthma, postinfectious bronchiolitis obliterans, and foreign body aspiration. We hope this review of special diseases can help the diagnosis and treatment in children.

• Tuberculosis and Respiratory Diseases
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• v.60 no.5
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• pp.564-570
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• 2006

Toxic gases and soot deposition as a consequence of smoke inhalation can cause direct injury to the upper and lower airways and even to the lung parenchyma. A delay in proper and prompt therapy can be detrimental to critically ill burn patients with an inhalation injury. Therefore, serial chest radiography is an important diagnostic tool for pulmonary complications during treatment. The radiographic findings of the chest include normal, consolidation, interstitial and alveolar infiltrates, peribronchial thickening, atelectasis, cardiogenic and non-cardiogenic pulmonary edema, and a pneumothorax as acute complications of smoke inhalation. In addition, bronchiectasis, bronchiolitis obliterans and pulmonary fibrosis can occur as late complications. We encountered a case of 44-year-old male who presented with acute lung injury after an inhalation injury. He required endotracheal intubation and mechanical ventilation due to respiratory failure. He was managed successfully with conservative treatment. Later, a cavitary lesion of the left upper lobe was observed on the chest radiography and computed tomography, which was complicated by massive hemoptysis during the follow-up. However, the cavitary lesion disappeared spontaneously without any clinical consequences.

• Tuberculosis and Respiratory Diseases
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• v.56 no.2
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• pp.203-209
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• 2004

Background : Methotrexate (MTX) has been used to treat a wide range of malignant and benign diseases including osteosarcoma, advanced stage non-Hodgkin's lymphoma, psoriasis, severe rheumatoid arthritis, sarcoidosis, and Wegener's granulomatosis. MTX-induced lung injury occurs in up to 10% of treated patients. Although both acute and chronic presentations have been described, typical manifestation of MTX-induced lung injury is subacute with symptoms usually developing within several months after starting therapy. Nonspecific interstitial pneumonia (NSIP) is the most common histopathologic manifestation of MTX-induced lung disease, while bronchiolitis obliterans organizing pneumonia (BOOP) and diffuse alveolar damage (DAD) are less common. Granuloma formation is reported in 34.7%. In Korea, Two reports of MTX pneumonitis have been published. The one presented with NSIP and the other with DAD. We recently experienced a case of MTX pneumonitis with presentation of hypersensitivity pneumonitis.

• Pediatric Infection and Vaccine
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• v.3 no.2
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• pp.145-153
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• 1996

The incidence of Adenovirus(AV) pneumonia in children is estimated at 8%. Long-term complications reported with type 7 include hyperleucent lung syndrome, bronchiolitis obliterans and may require several months to recover. We reviewed 8 case of AV pnemonia confirmed by AV culture and sero-diagnosis among selected 19 AV pneumonia suspicious cases who were admitted to the Sanggye Paik Hospital of Inje University due to clinical pneumonia from May to July 1996. From 19 children, nasal aspirates or trachial aspirates were collected: viral agents were detected by virus isolation and virus antigen was detected by indirect immunofluorescent staining. Serologic diagnosis for type-specific AV antibody was done by neutralisation test. The results are as follows: 1) Age of the patients ranged from 6 months to 47 months with a mean of $24{\pm}9$ months. 6 were male and 2 were female(M:F= 3:1). 2) Monthly distribution showed 4 cases on June, 2 cases on May and July, respectively. 3) The mean duration of admission was 20.4 days and mean duration of fever was 11.3 days 4) Peripheral leukocyte counts were elevated in 4 cases(50%) and findings of shift to left were showed in 4 cases(50%) and serum aminotransferase activities were elevated in 6 case(75%). 5) Radiologic findings include linear and streaky infiltration(1/8 cases), patchy and lobar consolidation (7/8 cases), pleural effusion(2/8 cases) and hyperleucency was seen in 3/8 cases at time of admission and increased to 6/8 cases on hospital discharge. 6) The AV serotypes were type 7, type 3, and type 1. 7) Oxygen supply was done in 4 cases(50%) and all 8 patients include 2 cases of type 7 treated with artificial ventilator were survived. In conclusion, the serotypes of AV pneumonia outbreaks in early summer of 1996 were type 7, 3, 1 and, as showed severe clinical course, continued follow up surveillance is necessary to the long term pulmonary complications.

• Tuberculosis and Respiratory Diseases
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• v.61 no.5
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• pp.479-483
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• 2006

Acute fibrinous and organizing pneumonia (AFOP) is a histological pattern consisting of prominent intra-alveolar fibrin and organizing pneumonia, with out hyaline membranes or prominent eosinophilia. The clinical manifestations of AFOP resemble those of acute lung injury such as acute interstitial pneumonia (AIP). However, the classic histological patterns of AFOP differ from diffuse alveolar damage (DAD), bronchiolitis obliterans with organizing pneumonia (BOOP) or acute eosinophilic pneumonia (AEP). The characteristic intra-alveolar fibrin ball and lack of classic hyaline membrane are the predominant histological features of AFOP. Although some reports suggest that its clinical course is less catastrophic than DAD, the clinical entity that distinguishes AFOP from DAD has not been established. We present a case of pathologically demonstrated AFOP in a 79-year-old man. The radiological findings of our case were similar to those of DAD, presented with diffuse bilateral lung infiltrations. However, despite the rapid development of respiratory failure, the patient had a better response and outcome to steroid therapy than what would be expected for DAD.