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Review
. 2013 Mar;9(1):66-70.
doi: 10.17925/EE.2013.09.01.66. Epub 2013 Mar 15.

Extra-hepatic Acromegaly

Sanne E Franck  1 , Aart Jan van der Lely  1 , Sebastian Neggers  1
Affiliations
Review

Extra-hepatic Acromegaly

Sanne E Franck et al. Eur Endocrinol. 2013 Mar.

Abstract

After the introduction of somatostatin analogs (LA-SMSA) and the growth hormone (GH) receptor antagonist, pegvisomant (Peg-v) normal serum insulin-like growth factor-1 (IGF-1) concentrations in virtually every patients with acromegaly is possible. The impact of these products on the GH-IGF1 axis is completely different. We advocate that LA-SMSA may normalize serum IGF1 levels in the presence of elevated GH actions in extra-hepatic tissues. This results in persistent peripheral disease activity that we call 'extra-hepatic acromegaly'. Peg-v competitively blocks systemic GH action and results in a GH serum level increase. Therefore high doses of Peg-v are necessary to control IGF-1. Since the mode of action differs between these products, it is questionable if identical IGF-1 levels, during Peg-v or LA-SMSA are really identical representations of the biochemical situation. With the traditional biomarkers medical treatment is therefore difficult to monitor with the traditional biomarkers. Additionally, Peg-v and LA-SMSA could be ideal combination since they have different mode of actions. We believe that the time has come to challenge the existing concepts of treatment and monitoring of patients with acromegaly.

Keywords: Acromegaly; IGF-1; Pegvisomant; extra-hepatic acromegaly; growth hormone deficiency; growth hormone receptor antagonist; growth hormone sensitivity; somatostatin analogs.

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Conflict of interest statement

Disclosure: Aart-Jan van der Lely and Sebastian Neggers have received grants from Novartis Pharma, Ipsen Pharma, and Pfizer Corporation. Sanne E Franck has no conflicts of interest to declare.

Figures

Figure 1:
Figure 1:. Effects of Somatostatin Analogs (SMSAs) in SMSA-sensitive Acromegalic Subjects
Red arrows indicate inhibitory effects; green arrows indicate stimulatory effects, while thickness of arrow indicates level of inhibition. GH = growth hormone; IGF-I = insulin-like growth factor–I; SMSAs = somatostatin analogs; WAT = white adipose tissue.
Figure 2:
Figure 2:. Effects of Pegvisomant in Acromegalic Subjects
Red arrows indicate inhibitory effects; green arrows indicate stimulatory effects, while thickness of arrow indicates level of inhibition. GH = growth hormone; IGF-I = insulin-like growth factor–I; WAT = white adipose tissue. Adapted from Neggers S. et al., Eur J Endocrinol, 2011;164:11–6.
See this image and copyright information in PMC

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