Jacobs W H, Kanarek J
Am J Gastroenterol. 1981 Oct;76(4):342-6.
Twenty-eight patients with cystic fibrosis, who survived to over 18 years of age are reported. The ages at the time of diagnosis ranged from and two and half months to over 21 years. Manifestations of cystic fibrosis in adults and youngsters are compared and contrasted. A review of the patients surviving shows that steatorrhea is under control. There is no evidence of biliary cirrhosis in any of these patients. Rectal prolapse has not been an important factor. Pseudomonas and Staphylococci were the major infecting organisms causing lung disease. Two-thirds of the patients do not use prophylactic antibiotics and one-third do not practice regular pulmonary care. Two female patients died; one of Pseudomonas lung infection and respiratory failure, after a pregnancy that was carried to term (live baby); one, of respiratory failure, also with severe Pseudomonas respiratory tract infection, two years after a pregnancy that was terminated in the first trimester.
报告了28例存活至18岁以上的囊性纤维化患者。诊断时的年龄从两个半月到21岁以上不等。对成人和青少年囊性纤维化的表现进行了比较和对比。对存活患者的回顾显示,脂肪泻得到了控制。这些患者中没有任何一例有胆汁性肝硬化的证据。直肠脱垂不是一个重要因素。铜绿假单胞菌和葡萄球菌是导致肺部疾病的主要感染病原体。三分之二的患者不使用预防性抗生素,三分之一的患者不进行常规肺部护理。两名女性患者死亡;一名在足月妊娠(活产婴儿)后死于铜绿假单胞菌肺部感染和呼吸衰竭;另一名在孕早期终止妊娠两年后死于呼吸衰竭,也伴有严重的铜绿假单胞菌呼吸道感染。