Maldonado Fabien, Tazelaar Henry D, Wang Chih-Wei, Ryu Jay H
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN.
Division of Anatomic Pathology, Mayo Clinic, Scottsdale, AZ.
Chest. 2008 Aug;134(2):375-381. doi: 10.1378/chest.08-0137. Epub 2008 Apr 10.
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.
We sought to clarify the clinical features and course associated with YNS by analyzing 41 consecutive cases evaluated at a tertiary referral medical center.
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.
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.
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。
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。
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。
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。