Heffes Nahmod L A, Ortiz J, Cervetto J L, Guastavino E, Boffi A
Arq Gastroenterol. 1984 Jan-Mar;21(1):39-45.
Five patients with CF (cystic fibrosis) dead between 1974 to 1982 at ages ranging from one to six months are presented. All of them showed edema, hypoalbuminemia and anemia in a severely compromised clinical situation, and failure to gain weight in spite of being breast-fed in the first weeks of life, in four of them. All of them were second or third degree malnourished babies (Gomez classification) at admission. Five children presented edema, two severe, two moderate and one mild. Hematocrit values ranged from 19% to 39% (means 26.4%), and albuminemia from 1.60 to 3.00 g/% (means 2.14 g/%). Two patients presented antecedents of dead brothers. All of them received substitution therapy with pancreatic enzymes. The children dead within seven and seventeen days of admission (means ten days) of broncho-pulmonar disfunction. In this work, we wish to call the pediatrician's attention about the importance of making this diagnostic presumption in the first months of the life.
本文介绍了1974年至1982年间死亡的5例囊性纤维化(CF)患儿,年龄在1至6个月之间。他们在临床情况严重受损时均出现水肿、低白蛋白血症和贫血,其中4例在出生后的头几周尽管进行母乳喂养但体重未增加。入院时,他们均为二度或三度营养不良婴儿(戈麦斯分类法)。5例患儿出现水肿,2例严重,2例中度,1例轻度。血细胞比容值在19%至39%之间(平均26.4%),白蛋白血症在1.60至3.00 g/%之间(平均2.14 g/%)。2例患儿有兄弟死亡史。他们均接受了胰酶替代治疗。这些患儿在入院后7至17天(平均10天)死于支气管-肺功能障碍。在这项研究中,我们希望引起儿科医生对在生命最初几个月做出这种诊断推测的重要性的关注。