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박지수,최윤정,김영태,박사미나,채종희,박준동,조연진,김우선,성문우,박성혜,권도희,정두현,서동인 대한의학회 2018 Journal of Korean medical science Vol.33 No.22
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.
서민석,강연구,하승균,김용일,팽진철,천기정,박사미나,김영태,이동수,E. Edmund Kim,정준기 대한핵의학회 2017 핵의학 분자영상 Vol.51 No.2
Purpose Until now, there was no single standardized regionalsegmentation method of planar lung perfusion scan.We comparedplanar scan based two segmentation methods, which arefrequently used in the Society of Nuclear Medicine, with referenceto the lung perfusion single photon emission computedtomography (SPECT)/computed tomography (CT) derivedvalues in lung cancer patients. Methods Fifty-five lung cancer patients (male:female, 37:18;age, 67.8 ± 10.7 years) were evaluated. The patientsunderwent planar scan and SPECT/CT after injection oftechnetium-99 m macroaggregated albumin (Tc-99 m-MAA). The % uptake and predicted postoperative percentageforced expiratory volume in 1 s (ppoFEV1%) derived fromboth posterior oblique (PO) and anterior posterior (AP)methods were compared with SPECT/CT derived parameters. Concordance analysis, paired comparison, reproducibilityanalysis and spearman correlation analysis were conducted. Results The % uptake derived from PO method showedhigher concordance with SPECT/CT derived % uptake in everylobe compared to AP method. Both methods showed significantlydifferent lobar distribution of%uptake compared toSPECT/CT. For the target region, ppoFEV1% measured fromPO method showed higher concordance with SPECT/CT, butlower reproducibility compared to AP method. Preliminarydata revealed that every method significantly correlated withactual postoperative FEV1%, with SPECT/CT showing thebest correlation. Conclusion The PO method derived values showed betterconcordance with SPECT/CT compared to the AP method. Both PO and AP methods showed significantly different lobardistribution compared to SPECT/CT. In clinical practice suchdifference according to different methods and lobes should beconsidered for more accurate postoperative lung functionprediction.