Würdinger H, Feuerbacher H J
Rofo. 1978 Nov;129(5):633-6. doi: 10.1055/s-0029-1231173.
The features of dysporia entero-broncho-pancreatica familiaris (mucoviscidosis) are described and the course of the disease over ten years is recounted. The cases are probably patients with true mucoviscidosis but in whom not all the symptoms develop in early childhood (Doerr). In the present case the symptoms only developed at the age of five years. From this time on there was increasing pancreatic insufficiency, chronic recurring bronchitis, with increasing destructive bronchitis and resulting respiratory insufficiency, dyspnoea and orthopnoea, right heart strain, sodium and chloride loss in the sweat, changes in the duodenum and the development of aganglionic megacolon.
描述了家族性肠-支气管-胰腺功能不良（黏液黏稠症）的特征，并讲述了该疾病十年的病程。这些病例可能是真正患有黏液黏稠症的患者，但并非所有症状都在幼儿期出现（多尔）。在本病例中，症状直到五岁时才出现。从那时起，胰腺功能不全逐渐加重，慢性复发性支气管炎，伴有进行性破坏性支气管炎并导致呼吸功能不全、呼吸困难和端坐呼吸、右心劳损、汗液中钠和氯流失、十二指肠改变以及无神经节巨结肠的发展。
