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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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- 학술저널
- 저자정보
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- 2018.1
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Mutations of the surfactant protein (SP)-C gene (SFTPC) have been associated with neonatal respiratory distress syndrome (RDS) and childhood interstitial lung disease (ILD). If accurate diagnosis and proper management are delayed, irreversible respiratory failure demanding lung transplantation may ensue. A girl was born at term but was intubated and given exogenous surfactant due to RDS. Cough and tachypnea persisted, and symptoms rapidly progressed at 16 months of age despite treatment with antibiotics, oral prednisolone, methylprednisolone pulse therapy, and intravenous immunoglobulin. At 20 months, she visited our hospital for a second opinion. A computed tomography scan showed a diffuse mosaic pattern with ground-glass opacity and subpleural cysts compatible with ILD. A video- assisted thoracoscopic lung biopsy revealed ILD with eosinophilic proteinaceous material and macrophages in the alveolar space. Bilateral lung transplant from a 30-month-old child was done, and she was discharged in room air without acute complications. Genetic analysis revealed a novel c.203T>A, p.Val68Asp mutation of SP-C, based on the same exon as a known pathogenic mutation, p.Glu66Lys.
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참고문헌 (20)
참고문헌 신청
Hawgood S / 1989 / Pulmonary surfactant apoproteins: a review of protein and genomic structure / Am J Physiol 257(2):L13~L22
Weaver TE / 2001 / Functions of surfactant proteins B and C / Annu Rev Physiol 63(1):555~578
Beers MF / 2005 / Surfactant protein C biosynthesis and its emerging role in conformational lung disease / Annu Rev Physiol 67(1):663~696
Nogee LM / 2001 / A mutation in the surfactant protein C gene associated with familial interstitial lung disease / N Engl J Med 344(8):573~579
Brasch F / 2004 / Interstitial lung disease in a baby with a de novo mutation on the SFTPC gene / Eur Respir J 24(1):30~39
Weaver TE / 2001 / Functions of surfactant proteins B and C / Annu Rev Physiol 63(1):555~578
Beers MF / 2005 / Surfactant protein C biosynthesis and its emerging role in conformational lung disease / Annu Rev Physiol 67(1):663~696
Nogee LM / 2001 / A mutation in the surfactant protein C gene associated with familial interstitial lung disease / N Engl J Med 344(8):573~579
Brasch F / 2004 / Interstitial lung disease in a baby with a de novo mutation on the SFTPC gene / Eur Respir J 24(1):30~39
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