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Growth hormone deficiency and growth hormone therapy in Ullrich-Turner-Syndrome

Wachstumshormon-Mangel und Wachstumshormon-Therapie beim Ullrich-Turner-Syndrom

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Zusammenfassung

Bei einer 7jährigen Patientin mit dem Chromosomensatz 45 XO wurde mit Hilfe von Stimulationstesten ein Mangel an Wachstumshormon nachgewiesen. Die Wachstumsrate von 2 cm/Jahr ließ sich mit. Hilfe von humanem Wachstumshormon auf 4,1 cm/Jahr (2 Behandlungsjahre) und unter zusätzlicher Gabe von Anabolica auf 5,7 bzw, 5,4 cm/Jahr (3. bzw. 4. Behandlungsjahr) steigern. Bei vier Patientinnen mit Ullrich-Turner-Syndrom (2mal 45X/, 1mal 46XXqi, 1mal 45XO/46 XX-Mosaik) ohne Wachstumshormon-Mangel konnte keine gleichartige Verbesserung der Wachstumsrate erzielt werden. Eine Therapie mit humanem Wachstumshormon ist somit nur dann gerechtfertigt, wenn ein Mangel an Wachstumshormon zusätzlich als Ursache einer verminderten Wachstumsrate beim Ullrich-Turner-Syndrom nachgewiesen werden kann.

Summary

In a girl with Ullrich-Turner-Syndrome (gonadal dysgenesis 45, XO) and growth hormone deficiency, 10 U of human growth hormone/m2 body surface area/week increased the growth rate from 2.0 to 4.1 cm/year. Doses of up to 36 U/m2/week did not improve the growth rate in 4 girls with Ullrich-Turner-Syndrome who had normal plasma growth hormone concentration and incretion. We conclude that growth hormone therapy is unsuccessful in dwarfism in Ullrich-Turner-Syndrome and should be reserved for patients with proven growth hormone deficiency.

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Supported by Deutsche Forschungsgemeinschaft, SFB 51/C10

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Butenandt, O. Growth hormone deficiency and growth hormone therapy in Ullrich-Turner-Syndrome. Klin Wochenschr 58, 99–101 (1980). https://doi.org/10.1007/BF01477194

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