Acromegaloidism: description of fifteen cases
Wilfredo Guanipa Sierra1,4, Concepción Páramo Fernández2, Pablo Fernández Catalina1, Elías Álvarez García2, Amalia Andrade Olivié2, Eduardo Pena3 & Federico Mallo Ferrer4
Acromegaloidism is a very rare entity (have been 54 cases reported worldwide) that presents with clinical acromegaly and normal somatotropic axis, without findings of pituitary or extrapituitary tumour. Its origin has not been clearly established, although it is associated with several pathophysiological conditions. The local ethics committee approved to perform an observational and multicenter study in three hospitals from a region with approximately 800 000 people over 18 years, in order to determine the clinic, demographic, analytic and karyotypic features from patients in whom acromegaly was discarded. Was determined somatotropic hormonal profile by chemiluminescent immunoassay in all subjects, as well as routine biochemical parameters. Also calculated the HOMA index, according TurnerMatthews considering insulin-resistance values above the 75th percentile. Was performed fifteen metaphases karyotype from peripheral blood in twelve subjects. At twenty months had been evaluated 15 patients, of whom 5 (33%) were male and 10 were women (67%) with mean age 49±17.85 years (CI95: 39.11 −58.88). Was estimated prevalence of period in 18.75 cases per million. The most prominent symptoms were arthralgia (93.3%), acral growth (86.7%), paresthesia, headache and fatigue (66.7% each). The most common signs were acromegaloid facial appearance (93.3%), prognathism (73.3%) and frontal wrinkles with broad forehead (53.3%). CI95 BMI was 28.6333.82 kg/m2 with mean of 31.22±4.68 kg/m2. CI95 GH levels was 0.133.65 ng/ml with mean of 1.89±3.18 ng/ml and CI95 IGF1 was 146.75231.11 ng/ml with mean of 188.93±76.17 ng/ml. The most frequent associated conditions were hyperlipidemia (60%), obesity (53.3%), insulin resistance (40%), impaired fasting glycemia (26.6%), hypertension (20%), and DM2 (13.4%). Was detected one Trisomy X (8.3%). These results support the statement that acromegaloidism is more common than thought, more frequently in women and mostly associated with metabolic disorders than karyotypic abnormalities.