Zeitler P S, Travers S, Kappy M S
University of Colorado Health Sciences Center, Denver, USA.
Adv Pediatr. 1999;46:101-49.
Children with chronic illness live with the specific consequences of their illness, as well as secondary endocrine abnormalities that further compromise growth and pubertal development. These secondary abnormalities may significantly add to their physiologic and psychological burden. Although these endocrine abnormalities theoretically arise as adaptations to the chronic illness, they may have deleterious effects if they persist untreated. Children with HIV infection and other wasting disorders, for example, show growth suppression out of proportion to the severity of their primary illness as a result of growth hormone resistance and enhanced cortisol secretion. In hematologic conditions such as sickle cell anemia, thalassemia, or bone marrow transplant, damage to the hypothalamus and/or pituitary may lead to growth hormone deficiency, gonadal insufficiency, and hypothyroidism. Growth and pubertal delay are also common among children with cystic fibrosis, along with insulin-dependent diabetes mellitus caused by pancreatic fibrosis. Similarly, children receiving long-term steroid therapy have delays in growth and pubertal development, accompanied by risk for osteoporosis, whereas chronic renal disease is associated with growth and pubertal delay, as well as secondary hyperparathyroidism. Recognition of potential endocrinopathies in children with chronic illness is an important aspect of the care of these children because the disturbances are frequently amenable to treatment, permitting full or partial restoration of normal growth and development in these children. In this chapter, the endocrine consequences of common chronic conditions of childhood are reviewed, as well as the etiology of the endocrine disturbance, the clinical consequences, and recommendations for treatment.
患有慢性病的儿童不仅要承受疾病带来的特定后果,还要面对继发性内分泌异常,这进一步影响了他们的生长和青春期发育。这些继发性异常可能会显著增加他们的生理和心理负担。虽然这些内分泌异常理论上是对慢性病的一种适应性反应,但如果不加以治疗,可能会产生有害影响。例如,感染艾滋病毒和患有其他消耗性疾病的儿童,由于生长激素抵抗和皮质醇分泌增加,其生长抑制程度与原发性疾病的严重程度不成比例。在镰状细胞贫血、地中海贫血或骨髓移植等血液系统疾病中,下丘脑和/或垂体受损可能导致生长激素缺乏、性腺功能不全和甲状腺功能减退。生长和青春期延迟在囊性纤维化儿童中也很常见,同时还伴有胰腺纤维化导致的胰岛素依赖型糖尿病。同样,接受长期类固醇治疗的儿童生长和青春期发育会延迟,同时存在骨质疏松的风险,而慢性肾病则与生长和青春期延迟以及继发性甲状旁腺功能亢进有关。认识到慢性病患儿潜在的内分泌疾病是照顾这些儿童的一个重要方面,因为这些紊乱通常可以通过治疗得到改善,使这些儿童的生长和发育得以完全或部分恢复正常。在本章中,将回顾儿童常见慢性病的内分泌后果,以及内分泌紊乱的病因、临床后果和治疗建议。