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Clinical Features and Outcomes of Idiopathic Pulmonary Alveolar Proteinosis in Korean Population

Cited 17 time in Web of Science Cited 15 time in Scopus
Authors
Byun, Min Kwang; Kim, Dong Soon; Kim, Young Whan; Chung, Man Pyo; Cha, Seung Ick; Park, Choon Sik; Park, Yong Bum; Park, Moo Suk; Lee, Hong Lyeol; Jeong, Sung Hwan; Uh, Soo-Taek; Shim, Jae Jeong
Issue Date
2010-03
Publisher
KOREAN ACAD MEDICAL SCIENCES
Citation
JOURNAL OF KOREAN MEDICAL SCIENCE; Vol.25 3; 393-398
Keywords
Pulmonary Alveolar ProteinosisTreatment OutcomeIrrigationKoreans
Abstract
Idiopathic pulmonary alveolar proteinosis (PAP) is a rare disorder in which lipoproteinaceous material accumulates within alveoli. There were few reports on Asian populations with idiopathic PAP. We retrospectively reviewed 38 patients with idiopathic PAP in Korea. We assessed clinical features, therapeutic efficacy and outcomes of whole lung lavage in patients with idiopathic PAP. The mean age at diagnosis was 52 yr. Eighty six percent of patients were symptomatic at diagnosis. Dyspnea and cough were the most common symptoms. Crackles were the most common physical examination finding. On pulmonary function test, a mild restrictive ventilatory defect was common, with a predicted mean forced vital capacity (FVC) of 77% and forced expiratory volume in one second (FEV(1)) of 84.6%. Diffusing capacity was disproportionately reduced at 67.7%. Arterial blood gas analysis revealed hypoxemia with a decreased PaO(2) of 69.0 mmHg and an increased D(A-a)O(2) of 34.2 mmHg. After whole lung lavage, PaO(2), D(A-a)O(2) and DL(CO) were significantly improved, but FVC and total lung capacity (TLC) were not different. This is the first multicenter study to analyze 38 Korean patients with idiopathic PAP. The clinical features and pulmonary parameters of Korean patients with idiopathic PAP are consistent with reports in other published studies. Whole lung lavage appears to be the most effective form of treatment.
ISSN
1011-8934
Language
English
URI
http://hdl.handle.net/10371/76539
DOI
https://doi.org/10.3346/jkms.2010.25.3.393
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College of Medicine/School of Medicine (의과대학/대학원)Internal Medicine (내과학전공)Journal Papers (저널논문_내과학전공)
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