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妊娠合并肺淋巴管平滑肌瘤病。病例报告。

Pulmonary lymphangiomyomatosis complicating pregnancy. A case report.

作者信息

Hughes E, Hodder R V

出版信息

J Reprod Med. 1987 Jul;32(7):553-7.

PMID:3625622
Abstract

A 32-year-old primigravida presented with cough and dyspnea at 16 weeks' gestation. Chest roentgenogram revealed a large pleural effusion and diffuse interstitial infiltrates. Moderate arterial hypoxemia and a significant reduction in vital capacity were present. Thoracentesis revealed sterile chyle with no evidence of malignancy. Spontaneous delivery of a healthy infant occurred at 38 weeks, but no change was seen in either the pulmonary infiltrates or chylothorax. Open lung biopsy confirmed the clinical impression of pulmonary lymphangiomyomatosis, and a pleurodesis was performed. Progesterone and estrogen receptor assays on the lung biopsy material revealed only minimal binding. Following two years of therapy with tamoxifen citrate and megestrol acetate, the chylothorax has not recurred, and there has been no other appreciable change in pulmonary function.

摘要

一名32岁初产妇在妊娠16周时出现咳嗽和呼吸困难。胸部X线片显示大量胸腔积液和弥漫性间质浸润。存在中度动脉低氧血症和肺活量显著降低。胸腔穿刺抽出无菌乳糜液,无恶性肿瘤证据。孕38周时自然分娩出一名健康婴儿,但肺部浸润或乳糜胸均无变化。开胸肺活检证实了肺淋巴管平滑肌瘤病的临床诊断,并进行了胸膜固定术。对肺活检材料进行的孕酮和雌激素受体检测仅显示微量结合。在用枸橼酸他莫昔芬和醋酸甲地孕酮治疗两年后,乳糜胸未再复发,肺功能也没有其他明显变化。

相似文献

1
Pulmonary lymphangiomyomatosis complicating pregnancy. A case report.妊娠合并肺淋巴管平滑肌瘤病。病例报告。
J Reprod Med. 1987 Jul;32(7):553-7.
2
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Nihon Kyobu Shikkan Gakkai Zasshi. 1987 Aug;25(8):918-23.
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Pulmonary lymphangiomyomatosis associated with tuberous sclerosis. Treatment with tamoxifen and tetracycline-pleurodesis.与结节性硬化症相关的肺淋巴管平滑肌瘤病。他莫昔芬和四环素胸膜固定术治疗。
Chest. 1985 Sep;88(3):473-5. doi: 10.1378/chest.88.3.473.
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Pulmonary lymphangiomyomatosis.肺淋巴管平滑肌瘤病
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Pregnancy in lymphangioleiomyomatosis: clinical and lung function outcomes in two national cohorts.淋巴管平滑肌瘤病患者的妊娠:两个国家队列的临床和肺功能结局。
Thorax. 2020 Oct;75(10):904-907. doi: 10.1136/thoraxjnl-2020-214987. Epub 2020 Aug 11.
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Estrogen activates pyruvate kinase M2 and increases the growth of TSC2-deficient cells.雌激素激活丙酮酸激酶 M2 并增加 TSC2 缺陷细胞的生长。
PLoS One. 2020 Feb 20;15(2):e0228894. doi: 10.1371/journal.pone.0228894. eCollection 2020.
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Clinical experience of lymphangioleiomyomatosis in the UK.英国淋巴管平滑肌瘤病的临床经验。
Thorax. 2000 Dec;55(12):1052-7. doi: 10.1136/thorax.55.12.1052.
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Rare diseases. 1. Lymphangioleiomyomatosis: clinical features, management and basic mechanisms.罕见病。1. 淋巴管平滑肌瘤病:临床特征、管理及基本机制
Thorax. 1999 Mar;54(3):254-64. doi: 10.1136/thx.54.3.254.
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Relation of pulmonary lymphangio-leiomyomatosis to use of the oral contraceptive pill and fertility in the UK: a national case control study.英国肺淋巴管平滑肌瘤病与口服避孕药使用及生育情况的关系:一项全国性病例对照研究。
Thorax. 1994 Sep;49(9):910-4. doi: 10.1136/thx.49.9.910.