Bierich J R
Universitätskinderklinik, Tuebingen, F.R.G.
J Endocrinol Invest. 1989;12(8 Suppl 3):25-33.
The spontaneous secretion of GH which determines the growth in height as well as the GH increment in the provocation tests (the socalled pituitary reserve) is diminished in pituitary dwarfism. In numerous other growth disorders only the spontaneous GH secretion is impaired. Amongst others this is true of the syndrome of constitutional delay of growth and adolescence which is the most frequent growth disorder in childhood and of the growth retardation after X-ray irradiation of the skull. In all these cases treatment by hGH represents replacement therapy. In children with intrauterine growth retardation, familial short stature and skeletal dysplasias growth hormone deficiency cannot be proved. In most cases the growth velocity cannot be accelerated with hGH in the usual replacement doses. High doses of hGH may be successful. In endogenous and exogenous hypercortisolism the spontaneous GH secretion is suppressed and the effect of the somatomedins on the growing cartilage is inhibited. This is not only true of Cushing's Syndrome and corticosteroid therapy but also of cases of glycogenosis Type I. The resulting growth retardation can - at least partly - be overcome with hGH. Preliminary investigations show that the growth retardation in children with chronic renal failure can successfully be treated by hGH.
垂体性侏儒症患者中,决定身高增长的生长激素(GH)自发性分泌以及激发试验中的GH增量(即所谓的垂体储备)均减少。在许多其他生长障碍中,仅自发性GH分泌受损。其中包括体质性生长和青春期延迟综合征,这是儿童期最常见的生长障碍,以及头颅X线照射后的生长迟缓。在所有这些情况下,使用hGH治疗属于替代疗法。对于宫内生长迟缓、家族性身材矮小和骨骼发育异常的儿童,无法证实存在生长激素缺乏。在大多数情况下,以常规替代剂量使用hGH无法加快生长速度。高剂量的hGH可能会取得成功。在内源性和外源性皮质醇增多症中,自发性GH分泌受到抑制,生长介素对生长中的软骨的作用也受到抑制。这不仅适用于库欣综合征和皮质类固醇治疗,也适用于Ⅰ型糖原贮积症病例。由此导致的生长迟缓至少部分可以通过hGH克服。初步研究表明,hGH可以成功治疗慢性肾功能衰竭儿童的生长迟缓。