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1. 嚢胞性線維症：実験的治療cystic fibrosis investigational therapies [show details]
… The survival rate of patients with cystic fibrosis (CF) continues to improve. In 1985, the median predicted survival was 25 years of age, while in 2017, it had increased to 46 years . In an effort to … very little mature F508del CFTR protein is trafficked to its targeted location on the apical surface of cells .…
2. 嚢胞性線維症：肝胆道系疾患cystic fibrosis hepatobiliary disease [show details]
… be useful to assess for fibrosis: Magnetic resonance elastography (MRE) may be the most helpful modality in assessing the extent of fibrosis in this very heterogeneous… CF transmembrane conductance regulator (CFTR) is located on the apical membrane of the biliary epithelium,…
3. 肝線維症の病因pathogenesis of hepatic fibrosis [show details]
… elastin may mark the conversion of reversible to irreversible fibrosis, and its degradation by macrophages may be an important determinant of elastic accumulation . As the liver becomes fibrotic, significant …
4. 肝線維症の非侵襲的評価：超音波エラストグラフィnoninvasive assessment of hepatic fibrosis ultrasound based elastography [show details]
… predicting significant fibrosis . Initially reported intraobserver variability and interobserver agreement for the assessment of liver fibrosis were relatively low . More recently, the elastic ratio of measurements …
5. 心筋症の定義および分類definition and classification of the cardiomyopathies [show details]
… scar/fibrosis, focal hypertrophy, left ventricular (LV) apical aneurysm,… characterized by diffuse thickening of the LV endocardium secondary to proliferation of fibrous and elastic tissue. Two forms have been described: a dilated form (DCM phenotype), in which the LV is enlarged …

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掌蹠角化症｜皮膚疾患症状対策治療｜品川シーサイド皮膚 ...

3）尖端角化類弾力線維症 (Acrokeratoelastoidosis) 本症は通常孤発性ですが、常染色体優性遺伝性のこともあります。 20-30歳代に発症し、卵円形ないし多角形のクレータ状の角化性丘疹が、掌蹠辺縁部、手指足趾の辺縁、下腿前面、ナックル部位や爪囲に見られます。

疾患詳細 - UR-DBMS / Syndrome Finder

・Acrokeratoelastoidosis (端角化類弾力線維症) = punctate PPK type III, focal acral hyperkeratosis, acrokeratoelastoidosis of Costa. 通常は散発性であるが, 家系例は常染色体優性を示唆する 10~20 歳以前に発症

疾患詳細

・Acrokeratoelastoidosis (端角化類弾力線維症) = punctate PPK type III, focal acral hyperkeratosis, acrokeratoelastoidosis of Costa. 通常は散発性であるが, 家系例は常染色体優性を示唆する 10~20 歳以前に発症