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黑人患者的囊性纤维化病程。

Course of cystic fibrosis in black patients.

作者信息

Stern R C, Doershuk C F, Boat T F, Tucker A S, Primiano F P, Matthews L W

出版信息

J Pediatr. 1976 Sep;89(3):412-7. doi: 10.1016/s0022-3476(76)80538-0.

Abstract

Seventeen black patients showed typical sweat gland, gastrointestinal, and pulmonary manifestations of cystic fibrosis. There was an unusually high incidence of meconium ileus equivalent (35%). Three infant siblings of patients in this study had died of cystic fibrosis prior to referral of a family member to this center. One patient died at age four years after emotional problems interfered with the treatment program; status asthmaticus played a major role in his terminal respiratory failure. The remainder of the patients have had substantially less morbidity from pulmonary disease than a comparable, much larger group of white patients. Only one patient, age 28, who is married and employed full-time has severe pulmonary involvement as assessed by pulmonary function studies and chest roentgenogram. The 16 surviving patients have a mean age of 13 1/2 years after a mean follow-up period of 10 years. If black patients with cystic fibrosis survive infancy, they then, as a group, may have a relatively good prognosis.

摘要

17名黑人患者表现出典型的囊性纤维化汗腺、胃肠道和肺部表现。胎粪性肠梗阻等效征的发病率异常高(35%)。本研究中患者的3名婴儿兄弟姐妹在家庭成员转诊至本中心之前死于囊性纤维化。一名患者在4岁时因情绪问题干扰治疗方案而死亡;哮喘持续状态在其终末期呼吸衰竭中起主要作用。其余患者的肺部疾病发病率明显低于规模大得多的可比白人患者组。通过肺功能研究和胸部X光检查评估,只有一名28岁、已婚且全职工作的患者有严重的肺部受累。16名存活患者在平均随访10年后的平均年龄为13.5岁。患有囊性纤维化的黑人患者如果在婴儿期存活下来,那么作为一个群体,他们可能有相对较好的预后。

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