脑垂体前叶先天性缺如伴有脑正常是一种罕见病。作者报告2例女婴先天性脑垂体功能减退,伴有重度低血糖和甲状腺功能减退。可惜这种综合征的早期识别仍是困难的,且延误适当治疗可能造成致命的后果。虽然先天性垂体功能减退的突出临床病状为窒息、紫绀和低血糖。但这对许多新生儿疾病是非特异性的,在男婴常见,伴有阴茎小,再加上其他症状应想到此病。本文报告2例,临床表现主要是低血糖、甲状腺功能和肾上腺功能减退伴有其它各种症状和体征。虽经补充甲状腺素、氢化考的松等治疗,1例于生后13 Congenital absence of the anterior pituitary associated with brain normal is a rare disease. The authors report congenital pituitary dysfunction in 2 female infants with severe hypoglycemia and hypothyroidism. Unfortunately, the early identification of this syndrome is still difficult, and the delay in appropriate treatment may have fatal consequences. Although the prominent clinical symptoms of congenital hypopituitarism are asphyxia, cyanosis and hypoglycemia. However, this is nonspecific for many neonatal diseases, common in male infants, accompanied by small penis, plus other symptoms should be reminded of the disease. This article reports 2 cases, the main clinical manifestations of hypoglycemia, thyroid function and adrenal insufficiency accompanied by a variety of other symptoms and signs. Although supplemented with thyroxine, hydrocortisone and other treatment, 1 case after birth 13