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Recent Advances in the Diagnosis and Management of Pneumocystis Pneumonia

Tasaka S

In human immunodeficiency virus (HIV)-infected patients, Pneumocystis jirovecii pneumonia (PCP) is a wellk-nown opportunistic infection and its management has been established. However, PCP is an emerging threat to immunocompromised patients...
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Osteogenesis imperfecta and combined orthodontics and orthognathic surgery: a case report on two siblings

Kim DY, Baik U, Jeon JH

Osteogenesis imperfecta is a heterogeneous group of connective tissue diseases that is predominantly characterized by bone fragility and skeletal deformity. Two siblings with undiagnosed type I osteogenesis imperfecta underwent orthognathic...
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A Rare Case of Overlap Syndrome with Diffuse Systemic Sclerosis, Rheumatoid Arthritis, and Cutaneous Sarcoidosis

Ko KM, Moon SJ, Koh JH, Kim J, Min JK

Overlap syndrome is defined as a disease entity that satisfies the classification criteria of at least two connective tissue diseases occurring concurrently or separately in a single patient. Here, we...
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Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 5. Connective Tissue Disease Associated Interstitial Lung Disease

Koo SM, Kim SY, Choi SM, Lee HK, on behalf of Korean Interstitial Lung Diseases Study Group

Connective tissue disease (CTD) is a collection of disorders characterized by various signs and symptoms such as circulation of autoantibodies in the entire system causing damage to internal organs. Interstitial...
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Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 1. Introduction

Park SW, Baek AR, Lee HL, Jeong SW, Yang SH, Kim YH, Chung MP, on behalf of the Korean Interstitial Lung Diseases Study Group

Idiopathic interstitial pneumonia (IIP) is a histologically identifiable pulmonary disease without a known cause that usually infiltrates the lung interstitium. IIP is largely classified into idiopathic pulmonary fibrosis, idiopathic non-specific...
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Treatment of the wide open wound in the Ehlers-Danlos syndrome

Baik BS, Lee WS, Park KS, Yang WS, Ji SY

Ehlers-Danlos syndrome (EDS) is an inherited disorder of collagen biosynthesis and structure, characterized by skin hyperextensibility, joint hypermobility, aberrant scars, and tissue friability. Besides the skin, skeleton (joint) and vessels,...
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New Diagnostic Guidelines for Idiopathic Pulmonary Fibrosis

Kwon BS, Song JW

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease characterized by worsening lung function and dyspnea. The prognosis of IPF patients is poor, as median survival is approximately...
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Acute Hemodynamic Changes after Single Administration of Udenafil in Pulmonary Arterial Hypertension: a Phase IIa Study

Chang SA, Kim HK, Chang HJ, Kim DK

BACKGROUND AND OBJECTIVES: Udenafil, a new phosphodiesterase-5 inhibitor (PDE5i), has been used to treat erectile dysfunction. Given the proven benefit of PDE5i in pulmonary arterial hypertension (PAH), we evaluated serial...
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Early Detection of Pulmonary Hypertension in Connective Tissue Disease

Jeon CH

No abstract available.
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Pathological interpretation of connective tissue disease-associated lung diseases

Kwon KY

Connective tissue diseases (CTDs) can affect all compartments of the lungs, including airways, alveoli, interstitium, vessels, and pleura. CTD-associated lung diseases (CTD-LDs) may present as diffuse lung disease or as...
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Two cases of calcinosis cutis treated with surgical excision

Park HJ, Kim JP, Ahn SY

Calcinosis cutis is an uncommon disorder and defined as the deposit of insoluble calcium salts in the skin. It is classified as dystrophic, metastatic, idiopathic, and iatrogenic according to etiology....
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Case of Raynaud Syndrome after the Use of Methimazole

Kim Y, Tag HS, Kim GT, Lee SG, Park EK, Park JH, Kweon SM, Yang SI, Kim JH

Raynaud syndrome is a medical condition that causes pain, numbness, and changes in skin color at the distal extremities. Raynaud syndrome can be subdivided into primary Raynaud's and secondary Raynaud's....
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Pulmonary Hypertension in Connective Tissue Disease is Associated with the New York Heart Association Functional Class and Forced Vital Capacity, But Not with Interstitial Lung Disease

Jung JY, Lee CH, Kim HA, Choi ST, Lee JH, Yoon BY, Kang DR, Suh CH

OBJECTIVE: Pulmonary hypertension (PH) develops frequently in connective tissue diseases (CTD) and is an important prognostic factor. The aim of this study was to assess the prevalence of PH in...
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Two Cases of Neonatal Lupus and Literature Review

Kim DH, Lee SJ, Kim TH, Yoo GH, Lee JY, Lee SY, Chang SH, Lee HJ, Hong SA, Park JS

Neonatal lupus is a rare rheumatic disease. Clinical manifestations include characteristic annular or macular rashes, congenital heart block, cytopenias, and hepatitis. Neonatal lupus is caused by transmission of maternal immunoglobulin...
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Arterial Tortuosity Syndrome in a Neonate

Kim KM, Hur SM, Yoon JH, Lee EJ, Lee JY

Arterial tortuosity syndrome (ATS) is a very rare autosomal recessive connective tissue disease characterized by generalized elongation and tortuosity of the medium- to large-sized arteries, and other systemic connective tissue...
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Clinical Study of 52 Patients with Adult-onset Dermatomyositis

Jung IS, Yun SJ, Lee JB, Kim SJ, Lee SC, Won YH

  • KMID: 2436624
  • Korean J Dermatol.
  • 2018 Jan;56(1):24-29.
BACKGROUND: Dermatomyositis is one of the idiopathic inflammatory myopathies, characterized by unique cutaneous features. Unfortunately, there is little data regarding the clinical features of dermatomyositis in Korea patients. OBJECTIVE: The study...
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Augmentation Index in Connective Tissue Diseases

Choi JH, Kim J

Atherosclerosis and its complications are often reported in patients with connective tissue diseases (CTDs) showing chronic inflammation. Traditional cardiovascular risk factors do not account for accelerated atherosclerosis in patients with...
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Treatment of connective tissue disease-associated interstitial lung disease: the pulmonologist's point of view

Koo SM, Uh ST

Interstitial lung disease (ILD) occurs in 15% of patients with collagen vascular disease (CVD), referred to as connective tissue disease (CTD). Despite advances in management strategies, ILD continues to be...
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Differential Diagnosis of Juvenile Idiopathic Arthritis

Kim YD, Job AV, Cho W

Juvenile idiopathic arthritis (JIA) is a broad spectrum of disease defined by the presence of arthritis of unknown etiology, lasting more than six weeks duration, and occurring in children less...
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Expression profile of mitochondrial voltage-dependent anion channel-1 (VDAC1) influenced genes is associated with pulmonary hypertension

Zhou T, Tang H, Han Y, Fraidenburg D, Kim YW, Lee D, Choi J, Bang H, Ko JH

Several human diseases have been associated with mitochondrial voltage-dependent anion channel-1 (VDAC1) due to its role in calcium ion transportation and apoptosis. Recent studies suggest that VDAC1 may interact with...
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