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.
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.
Authors: W Wu; J D Cogan; R W Pfäffle; J S Dasen; H Frisch; S M O'Connell; S E Flynn; M R Brown; P E Mullis; J S Parks; J A Phillips; M G Rosenfeld Journal: Nat Genet Date: 1998-02 Impact factor: 38.330
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Authors: James L Mills; Lawrence B Schonberger; Diane K Wysowski; Paul Brown; Stephen J Durako; Christopher Cox; Fanhui Kong; Judith E Fradkin Journal: J Pediatr Date: 2004-04 Impact factor: 4.406