[Case report about a 35 year old patient with cystic fibrosis and metastatic pancreatic cancer]. / Fallbericht eines 35 Jahre alt gewordenen Patienten mit Mukoviszidose und einem metastasierten Pankreaskarzinom.

Cystic fibrosis is the most common lethal genetic disease in Caucasian population. End-stage lung disease is the most frequent cause of death, however since therapeutic options have improved the life expectancy of patients with cystic fibrosis, increased incidences of gastrointestinal tumors including pancreatic carcinoma have been reported in several cohort studies from the USA and Europe. We report about a 35-year-old male patient who died from cystic fibrosis with a pancreatic carcinoma, which was histologically confirmed by autopsy. Additionally a low grade appendiceal mucinous neoplasm (LAMN) was diagnosed.

[Sporadic lymphangioleiomyomatosis. Clinical and lung functional characteristics of 32 female patients]. / Sporadische Lymphangioleiomyomatose. Klinische und lungenfunktionelle Charakteristika von 32 Patientinnen.

BACKGROUND AND OBJECTIVE: Lymphangioleiomyomatosis (LAM) is a rare and progressive multi-systemic disorder almost exclusively of women. Clinical and lung functional data of a substantial number of German patients have not so far been published. PATIENTS AND METHODS: In 32 women with sporadic LAM we performed the following investigations: medical history, clinical examination, lung function (spirometry, bodyplethysmography, diffusion capacity for carbon monoxide), ECG, echocardiography, and abdominal ultrasound. RESULTS: The mean age at the time of investigation was 48 (25 - 66) years, while the mean age at time of diagnosis had been 45 (21 - 61) years. The median time between onset of first symptoms and diagnosis was 8 years (1 months - 30 years). Most frequently stated first symptom was dyspnea on exertion (12/32 patients) and the most frequently stated first clinical sign was pneumothorax (12/32). Changes of hormonal status (contraception, treatment for fertility, pregnancy) occurred in 31 of the 32 patients before onset of first symptoms or clinical signs. Hormonal (16/32) and antiobstructive therapy (16/32) were the most frequently applied forms of treatment. Airway obstruction (26/32 patients), hyperinflation (20/32) and reduced diffusion capacity for carbon monoxide (24/32) were found on lung function test. Abnormal auscultation (6 patients), ECG signs of chronic cor pulmonale (4/32) and an elevated systolic pulmonary artery pressure (2 patients) were rare findings. Abdominal lesions (angiomyolipoma, 11/32 patients; lymphangioleiomyoma, 4 patients) were common. CONCLUSION: Lymphangioleiomyomatosis should be considered in women with spontaneous pneumothorax, unexplainable dyspnoea or angiomyolipoma.