The role of primary pharmacological therapy in acromegaly

Monteros, Ana Laura Espinosa de los; Carrasco, Claudia; Albarran, A.; Gadelha, Mônica R.; Lomba, Alín Abreu; Mercado, Moisés

doi:10.1007/s11102-013-0530-0

Cited by 7 publications

(4 citation statements)

References 56 publications

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“…In patients with somatotropic adenoma, GH hypersecretion is directly from the neoplastic cells and the evolution of the endocrine disease is strictly related to tumour activity. Therefore, in this situation, surgical resection is the first line of treatment for both cancer and GH excess, while lifelong pharmacological therapy is used as adjuvant therapy in the setting of persistent disease despite surgical intervention or in patients who are poor surgical candidates [27,28]. On the other hand, the pathogenesis of GH excess in NF-1 patients with OPG is still unclear, but its reversed course unrelated to the cancer treatment may suggest another mechanism.…”

Section: Discussionmentioning

confidence: 99%

Reversible Growth Hormone Excess in Two Girls with Neurofibromatosis Type 1 and Optic Pathway Glioma

Bruzzi

Sani

Albanese³

2015

Horm Res Paediatr

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Background: A total of 12 children with neurofibromatosis type 1 (NF-1) with optic pathway glioma (OPG) and growth hormone (GH) excess are reported to date, but no data exist on the long-term outcome. We describe 2 girls with NF-1 with OPG and GH excess treated with somatostatin analogue (SSa) who maintained a normal GH axis after stopping SSa therapy. Methods: The diagnosis ofGH excess was established from auxological data, persistently high levels of insulin-like growth factor 1 (IGF-1) and a lack of GH suppression during an oral glucose tolerance test. Results: Both patients were started on SSa treatment. During treatment, growth deceleration and normal IGF-1 levels were documented. The first case stopped treatment following the development of SSa side effects. The second case interrupted SSa when, closed to her final height, a normal IGF-1 level was documented. While off treatment, both cases maintained normal IGF-1 levels and appropriate growth velocity for their age and development, with normal GH secretion on biochemical testing. Both cases received treatment for central precocious puberty. Conclusion: GH excess in NF-1 children with OPG can be reversed and only short-term SSa therapy may be required. The aetiology remains undetermined, but the course suggests a hypothalamic dysfunction.

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Section: Discussionmentioning

confidence: 99%

Reversible Growth Hormone Excess in Two Girls with Neurofibromatosis Type 1 and Optic Pathway Glioma

Bruzzi

Sani

Albanese³

2015

Horm Res Paediatr

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“…Fifty-seven percent of patients treated with OCT exhibit tumor shrinkage of at least 20%; the mean computed degree of change induced by OCT-LAR was around 50% (Giustina et al 2012). In isolated cases the shrinkage effect can be spectacular (Espinosa de los Monteros et al 2014). Tumor decrease is more frequent and significant in primary-treated patients and in large macroadenomas (Mazziotti & Giustina 2010, Colao et al 2011.…”

Section: Medical Treatmentmentioning

confidence: 99%

60 YEARS OF NEUROENDOCRINOLOGY: Acromegaly

Căpățînă¹,

Wass²

2015

Journal of Endocrinology

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Acromegaly (ACM) is a chronic, progressive disorder caused by the persistent hypersecretion of GH, in the vast majority of cases secreted by a pituitary adenoma. The consequent increase in IGF1 (a GH-induced liver protein) is responsible for most clinical features and for the systemic complications associated with increased mortality. The clinical diagnosis, based on symptoms related to GH excess or the presence of a pituitary mass, is often delayed many years because of the slow progression of the disease. Initial testing relies on measuring the serum IGF1 concentration. The oral glucose tolerance test with concomitant GH measurement is the gold-standard diagnostic test. The therapeutic options for ACM are surgery, medical treatment, and radiotherapy (RT). The outcome of surgery is very good for microadenomas (80-90% cure rate), but at least half of the macroadenomas (most frequently encountered in ACM patients) are not cured surgically. Somatostatin analogs are mainly indicated after surgical failure. Currently their routine use as primary therapy is not recommended. Dopamine agonists are useful in a minority of cases. Pegvisomant is indicated for patients refractory to surgery and other medical treatments. RT is employed sparingly, in cases of persistent disease activity despite other treatments, due to its long-term side effects. With complex, combined treatment, at least three-quarters of the cases are controlled according to current criteria. With proper control of the disease, the specific complications are partially improved and the mortality rate is close to that of the background population.

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“…Well-controlled patients can have their dose down titrated by increasing the injection interval, a strategy that can reduce costs significantly, while increasing convenience [5][6][7]. Although increasing the injection interval to every 6, 8, or more weeks is offlabel in the US, it is a common practice in Europe and in Latin America [5][6][7]. In some patients increasing the injection interval is medically necessary in order to avoid GH deficiency.…”

mentioning

confidence: 99%