Chambers Daniel C, Lutzky Viviana P, Apte Simon H, Godbolt David, Feenstra John, Mackintosh John
Qld Lung Transplant Service The Prince Charles Hospital Brisbane QLD Australia.
School of Clinical Medicine The University of Queensland Brisbane QLD Australia.
Respirol Case Rep. 2020 Jun 25;8(6):e00607. doi: 10.1002/rcr2.607. eCollection 2020 Aug.
We report the case of a 42-year-old female with a history of finger clubbing which improved during pregnancy, a history of unexplained hepatosplenomegaly, and subsequent non-specific interstitial pneumonia with respiratory failure. Given a personal and family history of early greying of the hair, the peripheral blood monocyte telomere length was measured and was confirmed to be <1st centile, explaining the multiorgan presentation. She was treated with prednisolone, mycophenolate mofetil, and the synthetic androgen danazol with a dramatic improvement in respiratory failure and lung function. After 18 months of danazol treatment, the peripheral blood monocyte telomere length had returned to the normal range.
我们报告了一例42岁女性病例，该患者有杵状指病史（孕期有所改善）、不明原因肝脾肿大病史，随后出现非特异性间质性肺炎并伴有呼吸衰竭。鉴于其个人及家族有早生白发史，遂检测外周血单核细胞端粒长度，结果证实其低于第1百分位数，这解释了多器官受累的表现。她接受了泼尼松龙、霉酚酸酯和合成雄激素达那唑治疗，呼吸衰竭和肺功能得到显著改善。达那唑治疗18个月后，外周血单核细胞端粒长度恢复至正常范围。
