[Pulmonary changes in a female patient with Pringle's syndrome (author's transl)].
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.
D000328:Adult; D003560:Cysts / Q000209:etiology; D005153:Facial Neoplasms / Q000235:genetics / Q000503:physiopathology*; D005260:Female; D006222:Hamartoma / Q000235:genetics / Q000503:physiopathology*; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D008171:Lung Diseases / Q000209:etiology; D011030:Pneumothorax / Q000209:etiology; D013577:Syndrome; D014402:Tuberous Sclerosis / Q000235:genetics / Q000503:physiopathology*
Pulmonale Veränderungen bei einer Patientin mit Pringle-Syndrom