Bierich J R
Monatsschr Kinderheilkd. 1983 Apr;131(4):180-92.
According to recent investigations more than half of all growth disorders are due to growth hormone (GH) deficiency. I. The cause of the classic hypothalamo-pituitary dwarfism is: 1. perinatally acquired lesions of the hypothalamus; 2. suprasellar tumours; 3. brain malformations; 4. hereditary disturbances of GH-synthesis. - II. Constitutional delay of growth and adolescent development is clinically characterised by harmonious delay of growth, skeletal development and sexual maturation. The typical aspect of the pituitary dwarf is missing. Whereas provocation tests for GH do not show reliable deviations, assessment of the spontaneous GH secretion during sleep usually demonstrates a significant GH-deficit. - III. Impairments of GH synthesis with production of a hormone which reacts in the RIA but exhibits reduced biological effectivity causes the same clinical picture as constitutional delay. The disorders I--III can successfully be treated by GH. - IV. Familial short stature is based on the summation of hereditary anlages of the parents. In principle, it is no endocrine disorder. However, the combination with disorders II and III occurs frequently ("Small/Delay" acc. to Tanner). - V. Intrauterine growth retardation is caused by maternal, placental or fetal factors. With most of the "small for dates" growth prognosis is good. However, if teratogenic agents, particularly alcohol is involved, and if the damage occurs in the first months of gestation, the prognosis is unfavorable. - VI. A great number of genetic disorders is connected with pre- and/or postnatal growth retardation, combined with typical malformations. Apart from few exceptions these syndromes are rare. Endocrine deviations are missing. - The therapeutic possibilities of GH and anabolics are thoroughly discussed.
根据最近的调查，超过一半的生长障碍是由生长激素（GH）缺乏引起的。一、经典下丘脑 - 垂体性侏儒症的病因是：1. 围产期获得性下丘脑病变；2. 鞍上肿瘤；3. 脑畸形；4. 生长激素合成的遗传性紊乱。——二、体质性生长和青春期发育延迟的临床特征是生长、骨骼发育和性成熟的协调性延迟。垂体侏儒的典型表现不存在。虽然生长激素激发试验未显示可靠的偏差，但睡眠期间自发性生长激素分泌的评估通常显示明显的生长激素缺乏。——三、生长激素合成受损，产生一种在放射免疫分析（RIA）中起反应但生物有效性降低的激素，会导致与体质性延迟相同的临床症状。I - III类疾病可用生长激素成功治疗。——四、家族性身材矮小是基于父母遗传特征的总和。原则上，它不是内分泌疾病。然而，它与II类和III类疾病的合并情况很常见（根据坦纳的“小/延迟”）。——五、宫内生长迟缓由母体、胎盘或胎儿因素引起。大多数“小于胎龄儿”的生长预后良好。然而，如果涉及致畸剂，特别是酒精，并且损伤发生在妊娠的头几个月，预后则不佳。——六、大量遗传性疾病与产前和/或产后生长迟缓有关，并伴有典型畸形。除少数例外，这些综合征很少见。不存在内分泌偏差。——文中对生长激素和合成代谢药物的治疗可能性进行了全面讨论。
