“Micromegaly”: an update on the prevalence of acromegaly with apparently normal GH secretion in the modern era

Butz, Laura; Sullivan, Stephen E.; Chandler, William F.; Barkan, Ariel L.

doi:10.1007/s11102-016-0735-0

Cited by 40 publications

(53 citation statements)

References 17 publications

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“…It has long been noted that older patients with acromegaly can have milder disease features and hormonal abnormalities ( van der Lely et al 1992 ). More recently, it has been noted that a group of patients with ‘normal’ GH and elevated IGF-1 exists, that are older and have smaller tumors than acromegaly patients with typically raised GH and IGF-1 parameters ( Dimaraki et al 2002 , Butz et al 2016 ). It may be that the wider access to MRI and greater awareness noted above is also leading to increased pick-up of a milder phenotype of acromegaly in an older population.…”

Section: Discussionmentioning

confidence: 99%

Acromegaly at diagnosis in 3173 patients from the Liège Acromegaly Survey (LAS) Database

Pétrossians¹,

Daly²,

Natchev³

et al. 2017

Endocrine-Related Cancer

200

152

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Acromegaly is a rare disorder caused by chronic growth hormone (GH) hypersecretion. While diagnostic and therapeutic methods have advanced, little information exists on trends in acromegaly characteristics over time. The Liège Acromegaly Survey (LAS) Database, a relational database, is designed to assess the profile of acromegaly patients at diagnosis and during long-term follow-up at multiple treatment centers. The following results were obtained at diagnosis. The study population consisted of 3173 acromegaly patients from ten countries; 54.5% were female. Males were significantly younger at diagnosis than females (43.5 vs 46.4 years; P < 0.001). The median delay from first symptoms to diagnosis was 2 years longer in females (P = 0.015). Ages at diagnosis and first symptoms increased significantly over time (P < 0.001). Tumors were larger in males than females (P < 0.001); tumor size and invasion were inversely related to patient age (P < 0.001). Random GH at diagnosis correlated with nadir GH levels during OGTT (P < 0.001). GH was inversely related to age in both sexes (P < 0.001). Diabetes mellitus was present in 27.5%, hypertension in 28.8%, sleep apnea syndrome in 25.5% and cardiac hypertrophy in 15.5%. Serious cardiovascular outcomes like stroke, heart failure and myocardial infarction were present in <5% at diagnosis. Erythrocyte levels were increased and correlated with IGF-1 values. Thyroid nodules were frequent (34.0%); 820 patients had colonoscopy at diagnosis and 13% had polyps. Osteoporosis was present at diagnosis in 12.3% and 0.6–4.4% had experienced a fracture. In conclusion, this study of >3100 patients is the largest international acromegaly database and shows clinically relevant trends in the characteristics of acromegaly at diagnosis.

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Section: Discussionmentioning

confidence: 99%

Acromegaly at diagnosis in 3173 patients from the Liège Acromegaly Survey (LAS) Database

Pétrossians¹,

Daly²,

Natchev³

et al. 2017

Endocrine-Related Cancer

200

152

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“…Patients with clinically active acromegaly and elevated IGF-1 may still have ‘suppressible’ GH after OGTT using both cut-offs of 1 and 0.4 ng/dL. These discordant findings were observed in 18–45% of treatment-naïve patients with acromegaly, 33 , 34 and in 17–35% of patients with acromegaly after treatment (surgery, radiotherapy and/or medication). 35 – 37 Recent studies showed that a large number of patients with acromegaly can have a very different clinical, biochemical and radiological presentation compared with what is considered a ‘classical’ one.…”

Section: Screening and Diagnosismentioning

confidence: 98%

Updates in Diagnosis and Treatment of Acromegaly

Zahr

Fleseriu

2018

European Endocrinology

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Acromegaly is a rare disease, caused largely by a growth hormone (GH) pituitary adenoma. Incidence is higher than previously thought. Due to increased morbidity and mortality, if not appropriately treated, early diagnosis efforts are essential. Screening is recommended for all patients with clinical features of GH excess. There is increased knowledge that classical diagnostic criteria no longer apply to all, and some patients can have GH excess with normal GH response to glucose. Treatment is multifactorial and personalised therapy is advised.

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“…In a recent series report, 31% of 157 treatment-naive patients with acromegaly had elevated IGF-1 and normal 24 hours mean plasma GH levels. The percentage of GH/IGF-1 discrepancy has increased to 47% in more recent years [27]. Newer GH assays, which are based on monoclonal antibodies with higher specificity, tend to be more sensitive when compared with older polyclonal antisera.…”

Section: Discussionmentioning

confidence: 99%

Acromegaly with congenital generalized lipodystrophy – two rare insulin resistance conditions in one patient: a case report

et al. 2020

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Background: Lipodystrophies are a group of diseases which are characterized by abnormal adipose tissue deposition and are frequently associated with metabolic changes. Congenital generalized lipodystrophy is an autosomal recessive syndrome, with a prevalence < 1:10 million. Acromegaly is a rare disease, secondary to the chronic hypersecretion of growth hormone and insulin-like growth factor-1, with characteristic metabolic and somatic effects. "Acromegaloidism" is a term used for patients who manifest clinical features of acromegaly, but do not present a demonstrable hormone growth hypersecretion. The extreme shortage of subcutaneous adipose tissues and muscle hypertrophy confer an acromegaloid-like appearance in these patients. Case presentation: We describe a case of a patient with the rare combination of Berardinelli-Seip congenital lipodystrophy and acromegaly; our patient is a 63-year-old white man, who was referred to an endocrinology consultation for suspected lipodystrophy. He had lipoatrophy of upper and lower limbs, trunk, and buttocks, with muscular prominence, acromegaloid facial appearance, large extremities, and soft tissue tumescence. In addition, he had dyslipidemia and prediabetes. His fat mass ratio (% trunk fat mass/% lower limbs fat mass) was 1.02 by densitometry and he also had hepatomegaly, with mild steatosis (from an abdominal ultrasound), and left ventricular hypertrophy (from an electrocardiogram). His first oral glucose tolerance test had growth hormone nadir of 0.92 ng/mL, and the second test, 10 months afterwards, registered growth hormone nadir of 0.64 ng/mL (growth hormone nadir < 0.3 ng/mL excludes acromegaly). Pituitary magnetic resonance imaging identified an area of hypocaptation of contrast product in relation to a pituitary adenoma and he was subsequently submitted to transsphenoidal surgical resection of the mass. A pathological evaluation showed pituitary adenoma with extensive expression of growth hormone and adrenocorticotropic hormone, as well as a rare expression of follicle-stimulating hormone and prolactin. A genetic study revealed an exon 3/exon 4 deletion of the AGPAT2 gene in homozygosity. Conclusions: Congenital generalized lipodystrophy is a rare disease which occurs with acromegaloid features. As far as we know, we have described the first case of genetic lipodystrophy associated with true acromegaly. Although this is a rare association, the presence of congenital generalized lipodystrophy should not exclude the possibility of simultaneous acromegaly.

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“Micromegaly”: an update on the prevalence of acromegaly with apparently normal GH secretion in the modern era

Cited by 40 publications

References 17 publications

Acromegaly at diagnosis in 3173 patients from the Liège Acromegaly Survey (LAS) Database

Acromegaly at diagnosis in 3173 patients from the Liège Acromegaly Survey (LAS) Database

Updates in Diagnosis and Treatment of Acromegaly

Acromegaly with congenital generalized lipodystrophy – two rare insulin resistance conditions in one patient: a case report

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