难治性胰腺疾病包括胰腺囊肿性纤维增生、慢性胰腺炎、胰腺的先天性畸形和重症胰腺炎等。其中某些疾病的死亡率较高。胰腺囊肿性纤维增生的流行病学调查胰腺囊肿性纤维增生以白人多发,在胰腺或肺脏发生先天性的囊肿性变化,幼儿期死亡率较高。1980年日本报告了44个家族内的发病病例46例,从而证实了本病在日本的存在。随后,又收集到2组、4例家族内发病病例,合计17例。由此至少可以明确胰腺囊肿性纤维增生在日本存在,而且一般认为同欧美相比,日本的重症型患者较多。本病的预后取决于呼吸系统感染的情况,在欧美,近年来呼吸系统感染的治疗得到了改 Refractory pancreatic diseases include pancreatic cystic fibrosis, chronic pancreatitis, pancreatic congenital malformations and severe pancreatitis. Some of these diseases have a higher mortality rate. Epidemiological investigation of pancreatic cystic fibrosis Pancreatic cystic fibrosis is predominantly white in whites and congenital cystic changes occur in the pancreas or lung with high infant mortality. Japan reported 46 cases of disease in 44 families in 1980, confirming the existence of this disease in Japan. Subsequently, another two groups were collected, 4 cases of family history of disease, a total of 17 cases. At least it can be clear that pancreatic cystic fibrosis in Japan exists, and generally believed that compared with Europe and the United States, Japan’s more severe cases of patients. The prognosis of this disease depends on the situation of respiratory infections, in Europe and the United States, in recent years, the treatment of respiratory infections have been changed