Characteristics and treatment outcomes of micromegaly – acromegaly with apparently normal basal GH: A retrospective study and literature review

Q3 Medicine Endocrine regulations Pub Date : 2022-04-01 DOI:10.2478/enr-2022-0010
I. Inácio, L. Fonseca, A. Amado, V. Benido, I. Ribeiro, J. Dores, C. Amaral, M. H. Cardoso
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Abstract

Abstract Objective. Micromegaly describes a subgroup of patients with clinically evident acromegaly and elevated insulin-like growth factor I (IGF-I) with apparently normal basal growth hormone (bGH) and often a glucose-suppressed growth hormone (GH) of <1 ng/mL at diagnosis. It is controversial whether this condition is a distinct clinical entity or a classic acromegaly in early stages. The aim of the present article was to characterize the prevalence, clinical and biochemical characteristics, and therapeutic outcomes of micromegaly. Methods. A retrospective study of patients with an acromegaly followed ≥1 year at a tertiary center from 1995 to 2019. Patients without IGF-I or GH measurements at diagnosis were excluded. At diagnosis, bGH was considered normal if <2 ng/mL. Results. From 74 patients with acromegaly, 6 (8.1%) had normal bGH levels. There was no difference in the gender distribution, median diagnostic delay, and follow-up time between patients with normal bGH and elevated bGH. Patients with normal bGH were significantly older at time of the first acromegalic manifestation and diagnosis they had significantly lower nadir post-glucose GH and IGF-I levels, and tended to have a higher prevalence of obesity than patients with the elevated bGH. The frequency of acromegalic symptoms, signs, and other comorbidities than obesity was similar between groups. Five patients (83.3%) with normal bGH presented microadenomas. Post-operative remission and outcomes at last visit were comparable between patients with or without normal bGH. Conclusions. Normal bGH acromegaly is relatively uncommon in our patients. These patients showed differentiating characteristics from the classical acromegaly with elevated bGH. Further studies are needed to extend the knowledge about its clinical behavior, therapeutic outcomes, morbidity, and mortality.
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基底生长激素明显正常的微小型肢端肥大症的特征和治疗结果:一项回顾性研究和文献综述
摘要目标。Micromalegaly描述了一组临床明显肢端肥大症和胰岛素样生长因子I(IGF-I)升高的患者,其基础生长激素(bGH)明显正常,诊断时葡萄糖抑制生长激素(GH)通常<1 ng/mL。这种情况是一种独特的临床实体还是早期的典型肢端肥大症,目前仍存在争议。本文的目的是描述微小畸形的患病率、临床和生化特征以及治疗结果。方法。1995年至2019年在三级中心随访≥1年的肢端肥大症患者的回顾性研究。在诊断时没有IGF-I或GH测量的患者被排除在外。在诊断时,如果bGH<2 ng/mL,则认为bGH正常。后果74例肢端肥大症患者中,6例(8.1%)bGH水平正常。bGH正常和bGH升高患者的性别分布、中位诊断延迟和随访时间没有差异。bGH正常的患者在首次肢端肥大症表现和诊断时年龄明显较大,他们的血糖后最低点GH和IGF-I水平明显较低,并且往往比bGH升高的患者有更高的肥胖患病率。两组之间肢端肥大症症状、体征和其他合并症的频率与肥胖相似。5例(83.3%)bGH正常患者出现微腺瘤。bGH正常或不正常的患者术后病情缓解和最后一次就诊时的结果具有可比性。结论。正常bGH肢端肥大症在我们的患者中相对罕见。这些患者表现出与bGH升高的典型肢端肥大症不同的特征。需要进一步的研究来扩展对其临床行为、治疗结果、发病率和死亡率的了解。
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来源期刊
Endocrine regulations
Endocrine regulations Medicine-Endocrinology, Diabetes and Metabolism
CiteScore
2.70
自引率
0.00%
发文量
33
审稿时长
8 weeks
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