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基础生长激素水平看似正常的巨大症-肢端肥大症的特征及治疗结果:一项回顾性研究及文献综述

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

作者信息

Inacio Isabel, Fonseca Liliana, Amado Ana, Benido Vania, Ribeiro Isabel, Dores Jorge, Amaral Claudia, Cardoso Maria Helena

机构信息

Endocrinology Department, Centro Hospitalar do Baixo Vouga, Aveiro, Portugal.

Endocrinology Department, Centro Hospitalar Universitario do Porto, Porto, Portugal.

出版信息

Endocr Regul. 2022 Apr 30;56(2):87-94. doi: 10.2478/enr-2022-0010.

Abstract

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. 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. 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. 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.

摘要

微巨人症描述了一组临床明显的肢端肥大症患者,其胰岛素样生长因子I(IGF-I)升高,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经典肢端肥大症存在争议。本文的目的是描述微巨人症的患病率、临床和生化特征以及治疗结果。对1995年至2019年在三级中心随访≥1年的肢端肥大症患者进行回顾性研究。排除诊断时未进行IGF-I或GH测量的患者。诊断时,bGH<2 ng/mL被认为正常。在74例肢端肥大症患者中,6例(8.1%)bGH水平正常。bGH正常和bGH升高的患者在性别分布、中位诊断延迟和随访时间上没有差异。bGH正常的患者在首次出现肢端肥大症表现和诊断时年龄明显更大,他们的葡萄糖后最低GH和IGF-I水平明显更低,并且肥胖患病率往往高于bGH升高的患者。两组之间肢端肥大症症状、体征和除肥胖外的其他合并症的发生率相似。5例(83.3%)bGH正常的患者出现微腺瘤。bGH正常和不正常的患者术后缓解情况及最后一次随访时的结果相当。bGH正常的肢端肥大症在我们的患者中相对少见。这些患者表现出与bGH升高的经典肢端肥大症不同的特征。需要进一步研究以扩展关于其临床行为、治疗结果、发病率和死亡率的知识。

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