Handzel Z T, Elitsur Y, Algrimawi S, Moses S, Keynan A, Amitai I, Pecht M, Burstein Y, Buchner V, Trainin N
Isr J Med Sci. 1986 May;22(5):387-92.
Five infants with congenital immune defects are presented. Three had various combined immune deficiencies (CID) and two had thymic deficiencies only. As bone marrow or thymus transplantations were not feasible in these patients, we attempted treatment with thymic humoral factor (THF), a thymic hormone, by daily i.m. injections during biweekly courses. In one CID patient, a partial improvement in immune indices and temporary clinical improvement were achieved. In the other two, THF did not arrest the patients' demise. The two patients with thymic dysplasia benefitted repeatedly from THF treatment, as exemplified by the disappearance of wasting, diarrhea and infections and by reconstitution of T cell parameters. Nevertheless, the patients relapsed after prolonged periods without THF administration. We therefore propose the administration of long-term, continuous or intermittent thymic hormone replacement therapy in infants with congenital thymic defects. Early diagnosis and immediate institution of treatment will probably improve prognosis.
本文介绍了五名患有先天性免疫缺陷的婴儿。其中三名患有各种联合免疫缺陷(CID),两名仅患有胸腺缺陷。由于这些患者无法进行骨髓或胸腺移植,我们尝试使用胸腺体液因子(THF)(一种胸腺激素)进行治疗,在每两周的疗程中每天进行肌肉注射。在一名CID患者中,免疫指标得到了部分改善,临床症状也有了暂时的改善。在另外两名患者中,THF未能阻止患者死亡。两名患有胸腺发育不良的患者多次从THF治疗中获益,表现为消瘦、腹泻和感染消失,T细胞参数恢复正常。然而,在停止使用THF较长时间后,患者病情复发。因此,我们建议对患有先天性胸腺缺陷的婴儿进行长期、持续或间歇性的胸腺激素替代治疗。早期诊断和立即治疗可能会改善预后。