肢端肥大症的综合治疗(附43例临床分析)
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摘要
目的分析总结肢端肥大症的诊断和治疗特点,提高肢端肥大症的治疗效果。
     方法回顾分析2003年8月到2006年2月收治的43例肢端肥大症,采取以经口鼻蝶手术治疗为中心的综合治疗。
     结果本组病例男23例,女20例,平均年龄36.7岁,随访时间为3月~2.5年。肢端肥大症的治愈标准,随机血GH<2.5μg/L (或口腹糖耐量试验可抑制<1.0μg/L)和与年龄一致的IGF-1水平。手术前GH值平均33.5μg/L,术后33例降至正常,仍有10例>10μg/L,3例辅以药物治疗,7例给予伽玛刀治疗,GH逐渐下降并得以控制。并有糖尿病者10例血糖恢复正常;5例并有肢端肥大性心肌病术后心脏全部恢复正常,全组无死亡率。
     结论经口鼻蝶手术治疗仍是首选的治疗方法,随着药物和放射治疗的进步能使肢端肥大症得到更好的控制。单一疗法常难取得满意疗效,我们提倡以手术治疗为主的综合治疗。
Objective To study the diagnostic and therapeutiic features of acromegaly and improve the effect of acromegaly treatment.
     Method 43 cases of acromegaly from August 2003 to February 2006 were analyzed retrospectively, who received treatment consists of surgery, medical treatment and radiotherapy. The follow-up time was 3-30 months.
     Results The cohort consisted of 43 patients(23 men, 20 women, average age 36.7years.)who had been followed for 0.3–2.5 years (mean 2 years). Remission criteria were a normalized IGF-I concentration, a nadir GH level during oral glucose load of<1.0μg/L, and a random GH value of <2.5μg/L. Average serum GH was 33.5μg/L, after transsphenoidal surgery, the average serum GH decreased to normal ,but 10 of which GH>10μg/L, 3 had assisted of medical and 7 assisted of radiotherapy, then GH was to be controlled. The concentration of blood glucose returned to normal in 10 patients accompanied by diabetes. The cardiac function came to normal in all 5 cases with acromegalic cardiomy opathy after operation. There was no death in this series.
     Conclusion Transsphenoidal surgery remains the first-line reference treatment, Advances in pharmacological treatment (i.e. use of pegvisomant and selective somatostatin receptor agonists) and radiotherapy (radiosurgery) may result in further improvement of disease control. Results of a single therapy were with less satisfaction, We advocate integrated therapy around operation.
引文
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    3 Lei T, Adams EF, Buchfelder M, et al. Relationship between protein kinase C and adenylyl cyclase activity in the regulation of growth hormone secretion by human pituitary somatotrophinomas. Neurosurgery. 1996, 39(3):569-575
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    9 Ben-Shlomo A, Melmed S. Acromegaly. Endocrinol Metab Clin North Am. 2001, 30(3):565-583
    10 Kreutzer J, Vance ML, Lopes MB,et al. Surgical management of GH-secreting pituitary adenomas: an outcome study using modern remission criteria. J Clin Endocrinol Metab.2001, 86(9):4072-4077
    11 Peacey SR, Shalet SM. Insulin-like growth factor 1 measurement in diagnosis and management of acromegaly. Ann Clin Biochem. 2001, 38(Pt 4):297-303
    12 Newman CB. Medical therapy for acromegaly. Endocrinol Metab Clin North Am. 1999, 28(1):171-190
    13 Davies PH, Stewart SE, Lancranjan L, et al. Long-term therapy with long-acting octreotide (Sandostatin-LAR) for the management of acromegaly. Clin Endocrinol (Oxf). 1998, 48(3):311-316
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    1 Orme SM, McNally RJ, Cartwright RA, et al. Mortality and cancer incidence in acromegaly: a retrospective cohort study. United Kingdom Acromegaly Study Group. J Clin Endocrinol Metab.1998, 83(8):2730-2734
    2 Buchfelder M, Fahlbusch R, Merz T, et al. Clinical correlates in acromegalic patients with pituitary tumors expressing GSP oncogenes. Pituitary. 1999, 1(3-4):181-185
    3 Lei T, Adams EF, Buchfelder M, et al. Relationship between protein kinase C and adenylyl cyclase activity in the regulation of growth hormone secretion by human pituitary somatotrophinomas. Neurosurgery. 1996, 39(3):569-575
    4 雷霆, 薛德麟, Adama EF 等. PKC 在人垂体肿瘤增生中的作用研究. 同济医科大学学报, 1998, 27:39-41
    5 Freda PU. How effective are current therapies for acromegaly? Growth Horm IGF Res. 2003, 13 Suppl A:S144-151
    6 Abe T, Ludecke DK. Transnasal surgery for prolactin-secreting pituitary adenomas in childhood and adolescence. Surg Neurol. 2002, 57(6):369-378
    7 Derome PJ, Visot A. Surgery of pituitary adenoma. Rev Prat. 1996, 15;46(12):1515-1519
    8 Jho HD, Alfieri A. Endoscopic endonasal pituitary surgery: evolution of surgical technique and equipment in 150 operations. Minim Invasive Neurosurg. 2001, 44(1):1-12
    9 Nomikos P, Buchfelder M, Fahlbusch R. The outcome of surgery in 668 patients with acromegaly using current criteria of biochemical 'cure'. Eur J Endocrinol. 2005, 152(3):379-387
    10 De P, Rees DA, Davies N, et al. Transsphenoidal surgery for acromegaly in wales: results based on stringent criteria of remission. J Clin Endocrinol Metab. 2003, 88(8):3567-3572
    11 Muller AF, Van Der Lely AJ. Pharmacological therapy for acromegaly: a criticalreview. Drugs. 2004, 64(16):1817-1838
    12 Abs R, Verhelst J, Maiter D,et al. Cabergoline in the treatment of acromegaly: a study in 64 patients. J Clin Endocrinol Metab. 1998, 83(2):374-378
    13 Hofland LJ, Lamberts SW. The pathophysiological consequences of somatostatin receptor internalization and resistance. Endocr Rev. 2003, 24(1):28-47
    14 Ben-Shlomo A, Melmed S. Acromegaly. Endocrinol Metab Clin North Am. 2001, 30(3):565-583
    15 Kreutzer J, Vance ML, Lopes MB,et al. Surgical management of GH-secreting pituitary adenomas: an outcome study using modern remission criteria. J Clin Endocrinol Metab.2001, 86(9):4072-4077
    16 Peacey SR, Shalet SM. Insulin-like growth factor 1 measurement in diagnosis and management of acromegaly. Ann Clin Biochem. 2001, 38(Pt 4):297-303
    17 Newman CB. Medical therapy for acromegaly. Endocrinol Metab Clin North Am. 1999, 28(1):171-190
    18 Davies PH, Stewart SE, Lancranjan L, et al. Long-term therapy with long-acting octreotide (Sandostatin-LAR) for the management of acromegaly. Clin Endocrinol (Oxf). 1998, 48(3):311-316
    19 Attanasio R, Barausse M, Cozzi R. GH/IGF-I normalization and tumor shrinkage during long-term treatment of acromegaly by lanreotide. J Endocrinol Invest. 2001, 24(4):209-16
    20 Colao A, Marzullo P, Lombardi G. Effect of a six-month treatment with lanreotide on cardiovascular risk factors and arterial intima-media thickness in patients with acromegaly. Eur J Endocrinol. 2002, 146(3):303-309
    21 Trainer PJ, Drake WM, Katznelson L,et al. Treatment of acromegaly with the growth hormone-receptor antagonist pegvisomant. N Engl J Med. 2000, 20;342(16):1171-1177
    22 van der Lely AJ, Hutson RK, Trainer PJ, et al. Long-term treatment of acromegaly with pegvisomant, a growth hormone receptor antagonist. Lancet. 2001, 24;358(9295):1754-1759
    23 Castinetti F, Taieb D, Kuhn JM, et al. Outcome of gamma knife radiosurgery in 82patients with acromegaly: correlation with initial hypersecretion. J Clin Endocrinol Metab. 2005, 90(8):4483-4488
    24 Gutt B, Wowra B, Alexandrov R, et al. Gamma-knife surgery is effective in normalising plasma insulin-like growth factor I in patients with acromegaly. Exp Clin Endocrinol Diabetes. 2005, 113(4):219-224

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