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囊性纤维化患者的皮肤坏死性静脉炎

Cutaneous necrotizing venulitis in patients with cystic fibrosis.

作者信息

Soter N A, Mihm M C, Colten H R

出版信息

J Pediatr. 1979 Aug;95(2):197-201. doi: 10.1016/s0022-3476(79)80650-2.

DOI:10.1016/s0022-3476(79)80650-2
PMID:448559
Abstract

Palpable purpura was noted to occur late in the course of some patients with cystic fibrosis. Skin biopsy specimens showed necrotizing venulitis characterized by a perivenular infiltrate composed of neutrophilic leukocytes, fibrin, hypogranulated mast cells, and endothelial cell necrosis. Circulating immune complexes were detected. Recurrent pulmonary infections and the chronic administration of therapeutic agents provide sources of potential antigens.

摘要

在一些囊性纤维化患者病程后期可出现可触及的紫癜。皮肤活检标本显示为坏死性小静脉炎,其特征为静脉周围有由嗜中性白细胞、纤维蛋白、颗粒减少的肥大细胞和内皮细胞坏死组成的浸润。检测到循环免疫复合物。反复的肺部感染和长期使用治疗药物提供了潜在抗原的来源。

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Cutaneous necrotizing venulitis in patients with cystic fibrosis.囊性纤维化患者的皮肤坏死性静脉炎
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Mesoglycan treatment restores defective fibrinolytic potential in cutaneous necrotizing venulitis.硫酸乙酰肝素治疗可恢复皮肤坏死性静脉炎中缺陷的纤溶潜能。
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Cutaneous necrotizing venulitis.皮肤坏死性静脉炎
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引用本文的文献

1
Vasculitis in Cystic Fibrosis.囊性纤维化中的血管炎
Front Pediatr. 2020 Nov 12;8:585275. doi: 10.3389/fped.2020.585275. eCollection 2020.
2
Chronic suppurative lung disease with associated vasculitis.伴有相关血管炎的慢性化脓性肺部疾病。
Postgrad Med J. 1995 Jan;71(831):24-7. doi: 10.1136/pgmj.71.831.24.
3
Arthritis in cystic fibrosis.囊性纤维化中的关节炎
Arch Dis Child. 1984 Apr;59(4):377-9. doi: 10.1136/adc.59.4.377.
4
Pathogenesis and management of arthropathy in cystic fibrosis.囊性纤维化关节病的发病机制与管理
J R Soc Med. 1986;79 Suppl 12(Suppl 12):44-50.
5
Rheumatoid factors in cystic fibrosis: associations with disease manifestations and recurrent bacterial infections.囊性纤维化中的类风湿因子:与疾病表现及反复细菌感染的关联
Clin Exp Immunol. 1989 Jul;77(1):52-7.
6
Vasculitis and arthropathy in cystic fibrosis.囊性纤维化中的血管炎和关节病。
J R Soc Med. 1992;85 Suppl 19(Suppl 19):38-40.