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Pituitary
Published in: Pituitary 3/2007

Open Access 01-09-2007

Acromegaly caused by growth hormone-releasing hormone-producing tumors: long-term observational studies in three patients

Authors: Nienke R. Biermasz, Jan W. A. Smit, Alberto M. Pereira, Marijke Frölich, Johannes A. Romijn, Ferdinand Roelfsema

Published in: Pituitary | Issue 3/2007

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Abstract

We report on three newly diagnosed patients with extracranial ectopic GHRH-associated acromegaly with long-term follow-up after surgery of the primary tumor. One patient with a pancreatic tumor and two parathyroid adenomas was the index case of a large kindred of MEN-I syndrome. The other two patients had a large bronchial carcinoid. The first patient is still in remission now almost 22 years after surgery. In the two other patients GHRH did not normalize completely after surgery and they are now treated with slow-release octreotide. IGF-I normalized in all patients. During medical treatment basal GH secretion remained (slightly) elevated and secretory regularity was decreased in 24 h blood sampling studies. We did not observe development of tachyphylaxis towards the drug or radiological evidence of (growing) metastases. We propose life-long suppressive therapy with somatostatin analogs in cases with persisting elevated serum GHRH concentrations after removal of the primary tumor. Independent parameters of residual disease are elevated basal (nonpulsatile) GH secretion and decreased GH secretory regularity.
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Metadata
Title
Acromegaly caused by growth hormone-releasing hormone-producing tumors: long-term observational studies in three patients
Authors
Nienke R. Biermasz
Jan W. A. Smit
Alberto M. Pereira
Marijke Frölich
Johannes A. Romijn
Ferdinand Roelfsema
Publication date
01-09-2007
Publisher
Springer US
Published in
Pituitary / Issue 3/2007
Print ISSN: 1386-341X
Electronic ISSN: 1573-7403
DOI
https://doi.org/10.1007/s11102-007-0045-7

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WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

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