Acromegaly caused by growth hormone-releasing hormone-producing tumors:: long-term observational studies in three patients
Acromegaly caused by growth hormone-releasing hormone-producing tumors:: long-term observational studies in three patients
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DOI：
10.1007/s11102-007-0045-7
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发表时间：
2007-09-01
期刊：
影响因子：
3.8
通讯作者：
Roelfsema, Ferdinand
中科院分区：
文献类型：
--
作者：
Biermasz, Nienke R.;Smit, Jan W. A.;Roelfsema, Ferdinand
我们报告了三个新诊断的颅外异位GHRH相关肢端肥大症患者，在原发肿瘤手术后进行了长期随访。一例胰腺肿瘤和两个甲状旁腺腺瘤患者是MEN-I综合征的一个大家族的指示病例。另外两名患者有一个大的支气管类癌。第一位患者在手术后近22年仍处于缓解期。在另外两名患者中，GHRH在手术后没有完全恢复正常，他们现在用缓释奥曲肽治疗。IGF-I在所有患者中恢复正常。在药物治疗期间，基础生长激素分泌保持（略）升高和分泌规律性下降，在24小时采血研究。我们没有观察到对药物的快速耐受性的发展或（生长）转移的放射学证据。我们建议在原发肿瘤切除后血清GHRH浓度持续升高的病例中使用生长抑素类似物进行终身抑制治疗。残留疾病的独立参数是基础（非脉动）GH分泌升高和GH分泌规律性降低。
We report on three newly diagnosed patients with extracranial ectopic GHRH-associated acromegaly with long-term follow-up after surgery of the primary tumor. One patient with a pancreatic tumor and two parathyroid adenomas was the index case of a large kindred of MEN-I syndrome. The other two patients had a large bronchial carcinoid. The first patient is still in remission now almost 22 years after surgery. In the two other patients GHRH did not normalize completely after surgery and they are now treated with slow-release octreotide. IGF-I normalized in all patients. During medical treatment basal GH secretion remained (slightly) elevated and secretory regularity was decreased in 24 h blood sampling studies. We did not observe development of tachyphylaxis towards the drug or radiological evidence of (growing) metastases. We propose life-long suppressive therapy with somatostatin analogs in cases with persisting elevated serum GHRH concentrations after removal of the primary tumor. Independent parameters of residual disease are elevated basal (nonpulsatile) GH secretion and decreased GH secretory regularity.