Zorn W, Bartel M
Z Erkr Atmungsorgane. 1976;145(3):379-87.
The Pringle's Syndrome (Synon. adenoma sebaceum) is a very variable illness, based on a congenital neuroectodermal genetic defect. The hereditary transmission is dominant and it can be very various. The pulmonary involvement in Pringle's Syndrome is very rare. A case of a 25 year old female patient is demonstrated. She suffered on spontaneous pneumothoraces in both sides and had many typical alterations in other organs. Further examinations revealed disseminated small cystic alterations in the lung. Histologically these changes are based on a congenital defect of bronchioli. The patient's cardiopulmonal efficiency is assessed regarding to its prognosis. Finally the necessity of genetic care for patients with Pringle's Syndrome is pointed out.
普林格尔综合征（同义词：皮脂腺瘤）是一种基于先天性神经外胚层遗传缺陷的高度可变疾病。遗传传递为显性，且表现形式多样。普林格尔综合征的肺部受累非常罕见。本文展示了一例25岁女性患者的病例。她双侧自发性气胸，其他器官有许多典型改变。进一步检查发现肺部有弥漫性小囊性改变。组织学上，这些变化基于细支气管的先天性缺陷。对患者的心肺功能进行了预后评估。最后指出了对普林格尔综合征患者进行遗传护理的必要性。
