Faculdade de Medicina
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Browsing Faculdade de Medicina by Subject "Acromegaly"
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- Acromegaly in humans and cats: pathophysiological, clinical and management resemblances and differencesPublication . Lopes-Pinto, Mariana; Marques, Patrícia Lunet; Lacerda-Nobre, Ema; Miceli, Diego; Leal, Rodolfo Oliveira; Marques, PedroObjective: Acromegaly is a disorder associated with excessive levels of growth hormone (GH) and insulin-like growth factor-1 (IGF-1). In general, GH/IGF-1 excess leads to morphologic craniofacial and acral changes as well as cardiometabolic complications, but the phenotypic changes and clinical presentation of acromegaly differ across species. Here, we review the pathophysiology, clinical presentation and management of acromegaly in humans and cats, and we provide a systematic comparison between this disease across these different species. Design: A comprehensive literature review of pathophysiology, clinical features, diagnosis and management of acromegaly in humans and in cats was performed. Results: Acromegaly is associated with prominent craniofacial changes in both species: frontal bossing, enlarged nose, ears and lips, and protuberant cheekbones are typically encountered in humans, whereas increased width of the head and skull enlargement are commonly found in cats. Malocclusion, prognathism, dental diastema and upper airway obstruction by soft tissue enlargement are reported in both species, as well as continuous growth and widening of extremities resulting in osteoarticular compromise. Increase of articular joint cartilage thickness, vertebral fractures and spine malalignment is more evident in humans, while arthropathy and spondylosis deformans may also occur in cats. Generalized organomegaly is equally observed in both species. Other similarities between humans and cats with acromegaly include heart failure, ventricular hypertrophy, diabetes mellitus, and an overall increased cardiometabolic risk. In GH-secreting pituitary tumours, local compressive effects and behavioral changes are mostly observed in humans, but also present in cats. Cutis verticis gyrata and skin tags are exclusively found in humans, while palmigrade/plantigrade stance may occur in some acromegalic cats. Serum IGF-1 is used for acromegaly diagnosis in both species, but an oral glucose tolerance test with GH measurement is only useful in humans, as glucose load does not inhibit GH secretion in cats. Imaging studies are regularly performed in both species after biochemical diagnosis of acromegaly. Hypophysectomy is the first line treatment for humans and cats, although not always available in veterinary medicine. Conclusion: Acromegaly in humans and cats has substantial similarities, as a result of common pathophysiological mechanisms, however species-specific features may be found.
- Diagnosis and management of acromegaly: a consensus statement of the pituitary study group of the portuguese society of endocrinology, diabetes and metabolismPublication . Cardoso, Luís Miguel; Marques, Pedro; Pereira, Maria Teresa; Agapito, Ana; Almeida, Rui; Amaral, Sara; Cattoni, Maria Begona; Bugalho, Maria João; Cortez, Luísa; Duarte, Diana Borges; Duarte, João Sequeira; Fonseca, Fernando; Gomes, Leonor; Manique, Inês; Marques, Olinda; Martins, Teresa; Oliveira, Maria João; Paiva, Isabel; Pereira, Josué; Santos, Ana Paula; Silva, Vânia Benido; Torres, Isabel; Amaral, Cláudia; Carvalho, DavideAcromegaly is characterised by hypersecretion of growth hormone and presents diagnostic and therapeutic challenges that require consensus and guidelines for effective management. The Pituitary Study Group of the Portuguese Society of Endocrinology, Diabetes and Metabolism used a modified Delphi methodology to develop consensus recommendations for the diagnosis and management of acromegaly. A multidisciplinary panel of experts in acromegaly collaborated through this process to establish consensus-based statements. The authors did not receive any corporate funding or remuneration. The methodology employed to achieve these consensus recommendations included a literature review of specific topics, development of consensus statements, survey and interactive discussions, and a subsequent comprehensive analysis of the results to converge on key consensus statements for diagnosis, treatment, and monitoring strategies. By synthesising the available evidence and integrating expert opinion, this consensus document offers valuable insights for healthcare professionals, facilitating timely diagnosis, personalised treatment strategies developed by multidisciplinary teams, and enhanced patient care.
- Ecchordosis physaliphora: a rare and challenging clinical entity in a patient with acromegalyPublication . Marques, Pedro; Neto, Lia; Tortosa, Francisco; Sagarribay, Amets
- Predictors of therapeutic failure in GH and prolactin co-secreting pituitary adenomasPublication . Araujo-Castro, Marta; Biagetti, Betina; Menéndez, Edelmiro; Novoa-Testa, Iría; Cordido, Fernando; Berrocal, Víctor Rodríguez; Pascual-Corrales, Eider; Guerrero-Pérez, Fernando; Vicente, Almudena; García-Centeno, Rogelio; González, Laura; García, María Dolores Ollero; Echarri, Ana Irigaray; Rodríguez, María Dolores Moure; Novo-Rodríguez, Cristina; Calatayud, María; Villar-Taibo, Rocío; Bernabéu, Ignacio; Alvarez-Escola, Cristina; Jimenéz, Carmen Tenorio; Abellán-Galiana, Pablo; Venegas, Eva; González-Molero, Inmaculada; Iglesias, Pedro; Blanco, Concepción; Lara, Fernando Vidal Ostos de; Novoa, María Paz de Miguel; Torres, Elena López Mezquita; Hanzu, Felicia; Lamas, Cristina; Rodríguez, Silvia Aznar; Aulinas, Anna; Recio, José María; Aviles-Pérez, María Dolores; Núñez, Miguel Antonio Sampedro; Camara, Rosa; Fano, Miguel Paja; Fajardo, Carmen; Cardoso, Luís; Marques, Pedro; Martínez-Sáez, Elena; Ruz-Caracuel, Ignacio; Marazuela, Mónica; Puig-Domingo, ManelAim: To evaluate which factors are associated with a higher probability of failure to surgical and first-generation somatostatin receptor ligands (fgSRLs) treatment in patients with growth hormone and prolactin co-secreting pituitary adenomas (GH&PRL-PAs). Methods: Acromegaly patients with GH&PRL-PAs included in the ACRO-SPAIN study were enrolled. GH&PRL-PAs were defined as tumors with serum PRL levels above the upper limit of normal and positive immunostaining for GH and PRL, or with PRL levels ≥100 ng/mL when immunostaining data were not available. Results: A total of 126 acromegaly patients with GH&PRL-PAs who underwent transsphenoidal pituitary surgery were included, and 42.1% (n = 53) were biochemically cured at the immediate postoperative evaluation. Knosp grade >2 (odds ratio (OR) 3.48, 95% CI 1.28–9.38), higher serum GH (OR 1.01, 95% CI 1.01–1.08) and IGF-1 (OR 1.60, 95% CI 1.05–2.45) levels were associated with a lower probability of surgical cure. Sixty-eight patients received first-line medical therapy as follows: fgSRLs in monotherapy (n = 22), fgSRL plus cabergoline (n = 37), cabergoline in monotherapy (n = 7) and pegvisomant in monotherapy (n = 2). Among the cases treated with fgSRL in monotherapy, 18.2% (n = 4/22) were resistant. We identified as predictors of fgSRL resistance (in monotherapy and combined with cabergoline) a Knosp grade >2 (OR 8.75, P = 0.003), high GH levels at acromegaly diagnosis (OR 1.02, P = 0.031) and higher postoperative GH levels (OR 1.05, P = 0.006), but no predictors of response to fgSRL in monotherapy were identified. Conclusion: The clinical predictors of surgical failure and of fgSRL resistance in patients with GH&PRL-PAs are similar to those described in acromegaly without PRL, co-secretion.
- Pseudoacromegaly - a challenging entity in the endocrine clinic: a systematic reviewPublication . Marques, Pedro; Sapinho, Inês; Korbonits, MártaObjective: Pseudoacromegaly encompasses conditions with features of acromegaly/ gigantism, but no growth hormone (GH) or insulin‐like growth factor‐1 (IGF‐1) excess. We aimed to review published pseudoacromegaly cases evaluated due to clinical suspicion of acromegaly. Design/Patients: PubMed/Medline search was conducted to identify reported pseudoacromegaly cases, which were systematically reviewed to ensure they met eligibility criteria: (1) presentation suggestive of acromegaly; (2) acromegaly excluded based on normal GH, IGF‐1 and/or GH suppression on oral glucose tolerance test (OGTT‐GH); (3) diagnosis of the pseudoacromegaly condition was established. Data were retrieved from each case and analysed collectively. Results: Of 76 cases, 47 were males, mean ages at presentation and at first acromegaloid symptoms were 28 ± 16 and 17 ± 10 years, respectively. Most common conditions were pachydermoperiostosis (47%) and insulin‐mediated pseudoacromegaly (IMP) (24%). Acromegaloid facies (75%) and acral enlargement (80%) were the most common features. Measurement of random GH was reported in 65%, IGF‐1 in 79%, OGTT‐GH in 51%. GH excess was more frequently excluded based on two tests (53%). Magnetic resonance imaging (MRI) was performed in 30 patients, with pituitary adenoma or hyperplasia being reported in eight and three patients, respectively. Investigations differed between cases managed by endocrine and non‐endocrine specialists, the former requesting more often IGF‐1, OGTT‐GH and pituitary MRI. Conclusions: Pseudoacromegaly is a challenging entity that may be encountered by endocrinologists. Pachydermoperiostosis and IMP are the conditions most often mimicking acromegaly. Adequate assessment of GH/IGF‐1 is crucial to exclude acromegaly, which may be better performed by endocrinologists. Pituitary incidentalomas are common and require careful judgement to prevent unnecessary pituitary surgery.