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首例接受人生长激素治疗患者之一的长期随访

LONG TERM FOLLOW-UP OF ONE OF THE FIRST PATIENTS TO RECEIVE HUMAN GROWTH HORMONE THERAPY.

作者信息

Hawco Cassandra, Houlden Robyn L

机构信息

Department of Medicine, Queen's University, Kingston, Ontario, Canada.

出版信息

AACE Clin Case Rep. 2020 Jun 23;6(5):e262-e264. doi: 10.4158/ACCR-2020-0278. eCollection 2020 Sep-Oct.

Abstract

OBJECTIVE

Treatment of growth hormone (GH) deficiency with GH extracts from human pituitary glands was introduced by Dr. Maurice Raben at Tufts New England Medical Center in 1956. We report long term follow-up of one of the first patients treated with GH.

METHODS

Clinical, radiographic, and genetic data are presented.

RESULTS

A 78-year-old man presented for follow-up. He was one of the first patients to receive GH therapy from Raben in 1958. Growth was reported as normal until age 3 and then decelerated. At age 17 years, he was 129.5 cm with absent sexual development and bone age of 7 years. Treatment was initiated with desiccated thyroid and cortisone acetate for 8 months. Human GH extract was then initiated with 2 mg 3 times/week for 2 years, then 3 mg 3 times/week for 6 months, resulting in a final height of 168.9 cm. Testosterone intramuscularly every 2 weeks was added with sexual maturation over 2 years. He remained on testosterone injections until the age of 40 years, and on transdermal testosterone until the age of 50 years. At age 27 years, he was treated by Raben with human chorionic gonadotropin and menotropins for spermatogenesis restoration with successful conception by his wife. At age 78 years, pituitary MRI revealed a tiny amount of pituitary tissue within normal-sized sella turcica with absent pituitary infundibulum. A combined pituitary hormone deficiency genetic panel did not reveal any mutations.

CONCLUSION

Sixty-two years later, the patient remains in good health and grateful to a pioneer in Endocrinology for groundbreaking therapy of short stature. The cause of his hypopituitarism remains unknown.

摘要

目的

1956年,塔夫茨新英格兰医疗中心的莫里斯·拉本医生开始用从人垂体中提取的生长激素(GH)治疗生长激素缺乏症。我们报告了首批接受GH治疗的患者之一的长期随访情况。

方法

展示了临床、影像学和基因数据。

结果

一名78岁男性前来接受随访。他是1958年首批接受拉本医生GH治疗的患者之一。据报告,他3岁前生长正常,之后生长速度减缓。17岁时,他身高129.5厘米,性发育未启动,骨龄为7岁。开始用干燥甲状腺片和醋酸可的松治疗8个月。随后开始使用人GH提取物,最初每周3次,每次2毫克,持续2年,然后每周3次,每次3毫克,持续6个月,最终身高达到168.9厘米。在2年的性成熟过程中,每2周肌肉注射一次睾酮。他一直注射睾酮直至40岁,之后使用经皮睾酮直至50岁。27岁时,拉本医生用人类绒毛膜促性腺激素和促卵泡素对他进行治疗,以恢复精子生成,其妻子成功受孕。78岁时,垂体MRI显示在正常大小的蝶鞍内有少量垂体组织,垂体柄缺失。联合垂体激素缺乏基因检测未发现任何突变。

结论

62年后,该患者仍健康状况良好,并感激一位内分泌学先驱对其矮小身材进行的开创性治疗。其垂体功能减退的病因仍不明。

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