中枢性甲状腺功能减退症应采用甲状腺素替代治疗,同时评估和处理垂体病变。[35]Persani L. Clinical review: central hypothyroidism: pathogenic, diagnostic,
and therapeutic challenges. J Clin Endocrinol Metab. 2012;97:3068-3078.http://press.endocrine.org/doi/full/10.1210/jc.2012-1616http://www.ncbi.nlm.nih.gov/pubmed/22851492?tool=bestpractice.com[52]Grunenwald S, Caron P. Central hypothyroidism in adults: better understanding for better care. Pituitary. 2015;18:169-175.http://www.ncbi.nlm.nih.gov/pubmed/24554165?tool=bestpractice.com应紧急寻求内分泌专科医生的意见。
并发的肾上腺功能减退症的治疗
开始进行甲状腺激素替代治疗前,务必对肾上腺激素缺乏程度进行评估并治疗。用于治疗肾上腺功能减退症的药物有氢化可的松、可的松以及泼尼松龙。这些药物主要用于纠正糖皮质激素缺乏。在肾上腺功能减退的情况下行甲状腺激素替代治疗可能会诱发急性肾上腺危象。
甲状腺素替代
通常可以在治疗开始就使用预计需要的全剂量;但对于年龄较大和有已知冠状动脉疾病的患者,建议从低剂量开始,然后根据游离 T4 水平,逐渐调整到计算出的全剂量。[49]Garber JR, Cobin RH, Gharib H, et al. Clinical practice guidelines for hypothyroidism in adults: cosponsored by the American Association of Clinical Endocrinologists and the American Thyroid Association. Endocr Pract. 2012;18:988-1028.http://www.thyroid.org/thyroid-guidelines/hypothyroidism/http://www.ncbi.nlm.nih.gov/pubmed/23246686?tool=bestpractice.com[53]Clarke N, Kabadi UM. Optimizing treatment of hypothyroidism. Treat Endocrinol. 2004;3:217-221.http://www.ncbi.nlm.nih.gov/pubmed/16026104?tool=bestpractice.com[54]Beck-Peccoz P. Treatment of central hypothyroidism. Clin Endocrinol (Oxf). 2011;74:671-672.http://www.ncbi.nlm.nih.gov/pubmed/21521306?tool=bestpractice.com接受生长激素和/或雌激素治疗的患者可能需要进一步调整剂量。[55]Yamada M, Mori M. Mechanisms related to the pathophysiology and management of central hypothyroidism. Nat Clin Pract Endocrinol Metab. 2008;4:683-694.http://www.ncbi.nlm.nih.gov/pubmed/18941435?tool=bestpractice.com[56]Lania A, Persani L, Beck-Peccoz P. Central hypothyroidism. Pituitary. 2008;11:181-186.http://www.ncbi.nlm.nih.gov/pubmed/18415684?tool=bestpractice.com[57]Alexopoulou O, Beguin C, De Nayer P, et al. Clinical and hormonal characteristics of central hypothyroidism at diagnosis and during follow-up in adult patients. Eur J Endocrinol. 2004;150:1-8.http://eje-online.org/cgi/reprint/150/1/1http://www.ncbi.nlm.nih.gov/pubmed/14713273?tool=bestpractice.com甲状腺素治疗的主要潜在不良反应为用药过量。
腹部疾病、炎症性肠病和乳糖不耐受之类的吸收障碍性疾病可能会阻碍甲状腺素的吸收。可阻碍甲状腺素吸收的药物有铁剂、含铝的抑酸剂、碳酸钙、磷酸盐结合剂、胆酸螯合剂和质子泵抑制剂。服用甲状腺素时,应与可能影响其吸收的药物相隔至少 4 小时。[58]Biondi B, Wartofsky L. Treatment with thyroid hormone. Endocr Rev. 2014;35:433-512.http://www.ncbi.nlm.nih.gov/pubmed/24433025?tool=bestpractice.com
替代治疗不应同时应用甲状腺素和三碘甲腺原氨酸。[59]Jonklaas J, Bianco AC, Bauer AJ, et al. Guidelines for the treatment of hypothyroidism: prepared by the American Thyroid Association Task Force on Thyroid Hormone Replacement. Thyroid. 2014;24:1670-1751.http://online.liebertpub.com/doi/full/10.1089/thy.2014.0028http://www.ncbi.nlm.nih.gov/pubmed/25266247?tool=bestpractice.com
垂体肿瘤的治疗
对垂体肿瘤治疗有效的药物包括拟多巴胺药物或生长抑素类似物。对于分泌催乳素的垂体腺瘤主要使用多巴胺受体激动剂进行治疗。[60]Melmed S, Casanueva FF, Hoffman AR, et al. Diagnosis and treatment of hyperprolactinemia: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2011;96:273-288.http://press.endocrine.org/doi/10.1210/jc.2010-1692?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%3dpubmedhttp://www.ncbi.nlm.nih.gov/pubmed/21296991?tool=bestpractice.com[61]Casanueva FF, Molitch ME, Schlechte JA, et al. Guidelines of the Pituitary Society for the diagnosis and management of prolactinomas. Clin Endocrinology (Oxf). 2006;65:265-273.http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2265.2006.02562.x/fullhttp://www.ncbi.nlm.nih.gov/pubmed/16886971?tool=bestpractice.com
对于不分泌催乳素的垂体腺瘤,可以根据肿瘤的大小、范围和分泌功能进行手术治疗。可以采用经蝶手术或经额手术。[62]Buchfelder M, Schlaffer S. Surgical treatment of pituitary tumours. Best Pract Res Clin Endocrinol Metab. 2009;23:677-692.http://www.ncbi.nlm.nih.gov/pubmed/19945031?tool=bestpractice.com[63]Melmed S, Colao A, Barkan A, et al; Acromegaly Consensus Group. Guidelines for acromegaly management: an update. J Clin Endocrinol Metab. 2009;94:1509-1517.http://jcem.endojournals.org/cgi/content/full/94/5/1509http://www.ncbi.nlm.nih.gov/pubmed/19208732?tool=bestpractice.com
对于术后残留或复发性垂体腺瘤,放疗是一种有效的治疗方法,其对肿瘤的控制和过量激素分泌都有很好疗效。放疗实施途径、立体定向适形放疗 (SCRT) 以及立体定向放射外科 (SRS) 等领域的技术进步可以减少受到大剂量照射的正常大脑的面积。[64]Minniti G, Gilbert DC, Brada M. Modern techniques for pituitary radiotherapy. Rev Endocr Metab Disord. 2009;10:135-144.http://www.ncbi.nlm.nih.gov/pubmed/18787957?tool=bestpractice.com
实验室评估
内分泌科诊所对治疗进行监测。甲状腺素剂量的调整应以服用最后一剂药物 24 小时后空腹测量的游离 T4 值为依据。一般在剂量调整后的 4 至 8 周通过临床和游离T4水平重新评估该剂量是否足够。TSH 的测定对中枢性甲状腺功能减退症的治疗没有任何价值。甲状腺替代治疗后,游离T4水平的恢复比临床症状的缓解要早。游离 T4 水平应调整到正常范围内的中上水平。达到稳定后,可以每年随访一次游离 T4 水平。