Savage M O, Lebrethon M C, Blair J C, Ho J T, Johnston L B, Lienhardt A, Clark A J, Chaussain J L
Department of Endocrinology, St Bartholomew's and Royal London School of Medicine and Dentistry, London EC1A 7BE, UK.
Horm Res. 2001;56 Suppl 1:19-23. doi: 10.1159/000048129.
Linear growth can be disturbed in paediatric adrenal disease associated with endocrine hypo- or hyperfunction. Tall stature is a feature in some patients with adrenocorticotropic hormone resistance syndromes and short stature is recognized in the IMAGe (intrauterine growth retardation, metaphyseal dysplasia, adrenal hypoplasia congenita and genital anomalies) association. In autoimmune Addison's disease, growth is usually normal. In congenital adrenal hyperplasia, height may be compromised by advanced skeletal maturation or by suppressed growth, particularly in the neonatal period due to excess glucocorticoid treatment. In virilizing adrenal tumours, height is increased at diagnosis, but after surgical cure final height is usually in the normal range. In Cushing's disease, height was abnormally short in 50% of patients at presentation. After successful treatment, spontaneous catch-up growth was not seen. This led to a diagnosis of growth hormone (GH) deficiency in 80% of patients. With GH replacement, catch-up growth and long-term benefit occurred. Disturbance of linear growth is an important feature of many patients with adrenal disorders in childhood. Assessment of its pathogenesis and careful management are necessary to ensure optimal final adult height.
与内分泌功能减退或亢进相关的儿科肾上腺疾病可能会干扰线性生长。身材高大是一些促肾上腺皮质激素抵抗综合征患者的特征,而身材矮小在IMAGe(宫内生长迟缓、干骺端发育异常、先天性肾上腺发育不全和生殖器异常)综合征中较为常见。在自身免疫性艾迪生病中,生长通常正常。在先天性肾上腺皮质增生症中,身高可能会因骨骼成熟提前或生长受抑制而受到影响,尤其是在新生儿期,由于糖皮质激素治疗过量。在男性化肾上腺肿瘤中,诊断时身高会增加,但手术治愈后最终身高通常在正常范围内。在库欣病中,50%的患者就诊时身高异常矮小。成功治疗后,未观察到自发的追赶生长。这导致80%的患者被诊断为生长激素(GH)缺乏。给予生长激素替代治疗后,出现了追赶生长并带来了长期益处。线性生长障碍是许多儿童肾上腺疾病患者的一个重要特征。评估其发病机制并进行仔细管理对于确保成年后的最佳最终身高至关重要。