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黄甲综合征:41例连续病例分析

Yellow nail syndrome: analysis of 41 consecutive patients.

作者信息

Maldonado Fabien, Tazelaar Henry D, Wang Chih-Wei, Ryu Jay H

机构信息

Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN.

Division of Anatomic Pathology, Mayo Clinic, Scottsdale, AZ.

出版信息

Chest. 2008 Aug;134(2):375-381. doi: 10.1378/chest.08-0137. Epub 2008 Apr 10.

Abstract

BACKGROUND

Yellow nail syndrome (YNS) is a rare condition defined by the presence of yellow nails associated with lymphedema and/or chronic respiratory manifestations. Several aspects of this disorder remain poorly defined.

METHODS

We sought to clarify the clinical features and course associated with YNS by analyzing 41 consecutive cases evaluated at a tertiary referral medical center.

RESULTS

There were 20 men and 21 women; median age at diagnosis was 61 years (range, 18 to 82 years). None had a family history of YNS. All but one patient had chronic respiratory manifestations that included pleural effusions (46%), bronchiectasis (44%), chronic sinusitis (41%), and recurrent pneumonias (22%); 26 patients (63%) had lymphedema. Treatment included rotating antibiotic therapy for bronchiectasis, thoracenteses, oral vitamin E, and corticosteroid therapy. Eight patients underwent surgical management of recurrent pleural effusions including pleurodesis and decortication; two additional patients underwent pleurodesis via tube thoracostomy. The yellow nails improved or resolved in 14 of 25 patients (56%) for whom relevant data were available. Median survival of this cohort using the Kaplan-Meier method was 132 months, significantly lower than (p = 0.01) the control population. Among those still alive (20 patients), the disease appeared stable.

CONCLUSIONS

In most cases, YNS is an acquired disorder and associated respiratory manifestations are generally manageable with a regimen of medical and surgical treatments. Yellow nails improve in about one half of patients, often without specific therapy.

摘要

背景

黄甲综合征(YNS)是一种罕见疾病,其特征为黄色指甲伴有淋巴水肿和/或慢性呼吸系统表现。该疾病的几个方面仍未明确界定。

方法

我们通过分析在一家三级转诊医疗中心评估的41例连续病例,试图阐明与YNS相关的临床特征和病程。

结果

有20名男性和21名女性;诊断时的中位年龄为61岁(范围18至82岁)。无人有YNS家族史。除1例患者外,所有患者均有慢性呼吸系统表现,包括胸腔积液(46%)、支气管扩张(44%)、慢性鼻窦炎(41%)和反复肺炎(22%);26例患者(63%)有淋巴水肿。治疗包括针对支气管扩张的轮换抗生素治疗、胸腔穿刺、口服维生素E和皮质类固醇治疗。8例患者接受了复发性胸腔积液的手术治疗,包括胸膜固定术和剥脱术;另外2例患者通过胸腔闭式引流术进行了胸膜固定术。在有相关数据的25例患者中,14例(56%)的黄色指甲有所改善或消退。使用Kaplan-Meier方法计算,该队列的中位生存期为132个月,显著低于(p = 0.01)对照人群。在仍存活的患者(20例)中,疾病似乎稳定。

结论

在大多数情况下,YNS是一种后天性疾病,相关的呼吸系统表现通常通过药物和手术治疗方案得以控制。约一半患者的黄色指甲有所改善,通常无需特殊治疗。

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