Baumann G
Center for Endocrinology, Metabolism, and Nutrition, Northwestern University Medical School, Chicago, Illinois.
Endocrinol Metab Clin North Am. 1987 Sep;16(3):685-703.
Acromegaly is caused by GH-secreting pituitary adenomas and, in rare cases, by ectopic production of GRH with resultant hypersecretion of GH. Important systemic manifestations include acral enlargement, swelling, disfigurement, glucose intolerance and diabetes, hypertension, nerve entrapment, arthropathy, and cardiac disease. Tumor-related major manifestations are visual impairment, oculomotor paralysis, and hypopituitarism. Morbidity is substantial, and mortality is increased. Diagnosis should be made as early as possible by measuring plasma GH after an oral glucose load and plasma somatomedin C levels. Assessment of a pituitary lesion is best made by CT scanning in the coronal plane. Therapy is mandatory and consists of surgical removal of the pituitary adenoma (usually by the transsphenoidal route) or of the ectopic source of GRH (carcinoids or islet cell tumors). Adjunctive radiation and/or drug therapy is often necessary if complete surgical ablation of the adenoma is not possible. Radiation therapy can be administered as conventional supervoltage x-ray treatment or in the form of heavy particle beams. Drugs effective in partially lowering GH levels are bromocriptine and (not yet released) somatostatin analogues. Long-term follow-up of treated patients is important to guard against recurrence, progression, or development of hypopituitarism.
肢端肥大症由分泌生长激素(GH)的垂体腺瘤引起,在罕见情况下,由异位分泌生长激素释放激素(GRH)并导致GH分泌过多所致。重要的全身表现包括肢端增大、肿胀、畸形、葡萄糖耐量异常和糖尿病、高血压、神经受压、关节病和心脏病。与肿瘤相关的主要表现为视力损害、动眼神经麻痹和垂体功能减退。发病率很高,死亡率增加。应通过口服葡萄糖负荷后测量血浆GH和血浆生长调节素C水平尽早做出诊断。垂体病变的评估最好通过冠状面CT扫描进行。治疗是必需的,包括手术切除垂体腺瘤(通常通过经蝶窦途径)或GRH的异位来源(类癌或胰岛细胞瘤)。如果无法完全手术切除腺瘤,通常需要辅助放疗和/或药物治疗。放射治疗可作为传统的超高压X线治疗或重粒子束形式进行。有效部分降低GH水平的药物有溴隐亭和(尚未上市的)生长抑素类似物。对接受治疗的患者进行长期随访对于预防复发、病情进展或垂体功能减退的发生很重要。