endocrine and metabolic disorders
Gigantism and Acromegaly
Symptoms and Signs of Gigantism and Acromegaly
This rare condition occurs if GH hypersecretion begins in childhood, before closure of the epiphyses. Skeletal growth velocity and ultimate stature are increased, but little bony deformity occurs. However, soft-tissue swelling occurs, and the peripheral nerves are enlarged. Delayed Puberty or hypogonadotropic hypogonadism is also frequently present, resulting in a eunuchoid habitus (ie, a body build that is tall and slender with long extremities). In acromegaly, GH hypersecretion usually starts when the patient is between 20 and 40 years old. When GH hypersecretion begins after epiphyseal closure, the earliest clinical manifestations are coarsening of the facial features and soft-tissue swelling of the hands and feet. Appearance changes, and larger rings, gloves, and shoes are needed. Photographs of the patient are important in delineating the course of the disease. This photo shows a patient with frontal bossing, prognathism, nasal bone hypertrophy, and thickened skin. © Springer Science+Business Media Left image shows a 64-year-old woman who presented with acromegaly due to a pituitary adenoma. Right image shows the same patient 11 years earlier. Note the change in facial features compared with the left image. By permission of the publisher. From Newman C. InAtlas of Clinical Endocrinology: Neuroendocrinology and Pituitary Disease. Edited by SG Korenman (series editor) and ME Molitch. Philadelphia, Current Medicine, 2000. Frontal and lateral images of a patient with acromegaly. Coarse facial features are evident, including prognathism and prominence of the malar eminences and supraorbital ridges. By permission of the publisher. From Conrad C, Pro B, Prabhu S, et al. InAtlas of Cancer. Edited by M Markman and MR Gilbert. Philadelphia, Current Medicine, 2002. This photo shows a normal hand (on the left) compared to the enlarged hand (on the right) of a patient with acromegaly. BIOPHOTO ASSOCIATES/SCIENCE PHOTO LIBRARY In adults with acromegaly, coarse body hair increases, and the skin thickens and frequently darkens. The size and function of sebaceous and sweat glands increase, such that patients frequently complain of excessive perspiration and offensive body odor. Overgrowth of the mandible leads to protrusion of the jaw (prognathism) and malocclusion of teeth. Cartilaginous proliferation of the larynx leads to a deep, husky voice. The tongue is frequently enlarged and furrowed. In long-standing acromegaly, costal cartilage growth leads to a barrel chest. Articular cartilaginous proliferation occurs early in response to GH excess, with the articular cartilage possibly undergoing necrosis and erosion. Joint symptoms are common, and crippling degenerative arthritis may occur. Peripheral neuropathies occur commonly because of compression of nerves by adjacent fibrous tissue and endoneural fibrous proliferation. Headaches are common because of the pituitary tumor. Bitemporal hemianopia may develop if suprasellar extension compresses the optic chiasm. The heart, liver, kidneys, spleen, thyroid gland, parathyroid glands, colon, and pancreas are larger than normal; thyroid enlargement may be generalized or multinodular. Cardiac disease (eg, Overview of Coronary Artery Disease , cardiomegaly, valvular insufficiencies, sometimes Snoring is a common symptom, and Obstructive Sleep Apnea (OSA) occurs in 40 to 50% of patients. Colonic polyps are increased as a result of GH excess. The risk of cancer, particularly of the gastrointestinal tract, increases 2-fold to 3-fold. GH increases tubular reabsorption of phosphate and leads to mild hyperphosphatemia. Impaired glucose tolerance occurs in nearly half the patients with acromegaly and in gigantism, but clinically significant Diabetes Mellitus (DM) occurs in only about 10% of patients. Prolactinoma occurs in some women with acromegaly, usually in association with 