Dharan Shalini Sree, Kamaruddin Nor Azmi
Endocrinology and Diabetes Unit, Department of Medicine, National University of Malaysia.
J ASEAN Fed Endocr Soc. 2017;32(2):169-172. doi: 10.15605/jafes.032.02.12. Epub 2017 Sep 30.
Acromegaly is a rare disease with an annual incidence of 3 to 4 cases in a million. Diagnosis is often delayed due to the slow progression of the disease. Persistent elevation of growth hormone (GH) in acromegaly causes a reduction in life expectancy by 10 years. Aside from multiple cardiovascular, respiratory and metabolic co-morbidities, it has also been proven to cause an increased incidence of cancer. The main treatment of acromegaly is surgical excision of the functioning pituitary adenoma. Multiple comorbidities, including obstructive sleep apnea (OSA), left ventricular hypertrophy (LVH) and soft tissue swelling, make surgery complicated, if not impossible. Medical therapy to reduce comorbidities may be indicated in certain situations. Somatostatin receptor ligands (SRL) are able to reduce, and possibly normalize, IGF-1 levels. Reduction of insulin-like growth factor-1 (IGF-1), the main mediator of GH, is able to resolve headache, sweating, fatigue and soft tissue swelling, and also reduce ventricular hypertrophy. This case report illustrates the successful use of the SRL octreotide LAR in treating acromegaly. It also confirms the observation from several case series that thyroid cancer is the most common malignancy in acromegaly.
肢端肥大症是一种罕见疾病,年发病率为百万分之3至4例。由于疾病进展缓慢,诊断往往会延迟。肢端肥大症患者生长激素(GH)持续升高会导致预期寿命缩短10年。除了多种心血管、呼吸和代谢合并症外,还已证实其会导致癌症发病率增加。肢端肥大症的主要治疗方法是手术切除功能性垂体腺瘤。多种合并症,包括阻塞性睡眠呼吸暂停(OSA)、左心室肥厚(LVH)和软组织肿胀,即使手术并非不可能,也会使手术变得复杂。在某些情况下,可能需要进行减轻合并症的药物治疗。生长抑素受体配体(SRL)能够降低并可能使胰岛素样生长因子-1(IGF-1)水平正常化。降低GH的主要介质胰岛素样生长因子-1(IGF-1)能够缓解头痛、出汗、疲劳和软组织肿胀,还能减轻心室肥厚。本病例报告说明了SRL长效奥曲肽在治疗肢端肥大症方面的成功应用。它还证实了几个病例系列的观察结果,即甲状腺癌是肢端肥大症中最常见的恶性肿瘤。