DeVile C J, Grant D B, Hayward R D, Stanhope R
Institute of Child Health and Great Ormond Street Hospital for Children, NHS Trust, London.
Arch Dis Child. 1996 Aug;75(2):108-14. doi: 10.1136/adc.75.2.108.
The growth and endocrine sequelae of 75 children (33 girls and 42 boys) with craniopharyngioma, treated from 1973 to 1994, were studied by retrospective review and by follow up assessment in 66 survivors, with a mean time from initial surgery of 6.7 years (range 1.5 to 19.8 years). Although infrequently complained of, 71% of patients had symptoms to suggest an endocrinopathy at diagnosis. After surgery, multiple endocrinopathies were almost universal, such that 75% of children had panhypopituitarism at follow up. Hypoadrenal crises in association with intercurrent illness contributed significantly to morbidity and mortality, as did the metabolic consequences of concomitant antidiuretic hormone (ADH) insufficiency and absent thirst. Final height in 25 patients was significantly below genetic target height, particularly in the girls, with loss of height potential occurring during the pubertal years. The endocrine morbidity associated with craniopharyngioma and its treatment remains high but manageable with appropriate hormone replacement. However, the combination of ADH insufficiency and an impaired sense of thirst following aggressive surgery and severe hypothalamic injury remains one of the most complex management problems.
对1973年至1994年间接受治疗的75例颅咽管瘤患儿（33例女孩和42例男孩）的生长及内分泌后遗症进行了回顾性研究，并对66例幸存者进行了随访评估，从初次手术到随访的平均时间为6.7年（范围1.5至19.8年）。虽然很少有患儿抱怨，但71%的患者在诊断时有提示内分泌病的症状。手术后，多种内分泌病几乎很常见，以至于75%的患儿在随访时有全垂体功能减退。与并发疾病相关的肾上腺皮质功能减退危象对发病率和死亡率有显著影响，抗利尿激素（ADH）不足和无口渴感所导致的代谢后果也同样如此。25例患者的最终身高显著低于遗传目标身高，尤其是女孩，身高潜力在青春期出现丢失。与颅咽管瘤及其治疗相关的内分泌发病率仍然很高，但通过适当的激素替代治疗是可控的。然而，在积极手术和严重下丘脑损伤后，ADH不足和口渴感受损的组合仍然是最复杂的管理问题之一。
