See also: Gigantism
Definition
Acromegaly is a chronic, rare and disabling syndrome caused by a "hypersecretion" of the hormone hGH during adulthood.
Symptoms and Complications
For further information: Acromegaly Symptoms
Gigantism: Robert Wadlow, 2 meters and 72 cm, next to his father, shows us the effects of a hyper-secretion of GH during development.
An excess of growth hormone during childhood and puberty is accompanied by exaggerated growth in height (gigantism). In adulthood, on the contrary - since any further increase in the length of the diaphysis is impossible - the disease causes a volumetric increase in the distal parts of the body (bones and soft tissues). The patient affected by acromegaly thus develops singular features, up to a deep subversion of his physiognomy. These alterations lead to a noticeable enlargement of the hands, feet, supraorbital arches and jaw (with inevitable dental diastasis and aggravation of malocclusion); therefore they electively affect the body extremities, as the same name reminds us of the disease (from the Greek akros "extremity" e megalos "great").
The characteristic symptoms of acromegaly evolve in an extremely slow and progressive way, so much so that generally neither the patient nor the people around him are able to realize it. Only in a fairly advanced stage can the subject, for example, realize that the phalanges are they swell because gloves, shoes or rings no longer fit; alterations to the skull make the hats too small, while food residues get stuck between the teeth.
At the same time the nose and lips increase in size, the cheekbones protrude and the characteristic prognathism appears; the tongue also undergoes abnormal growth (hyperglossia). More often, however, the acromegalic patient comes to the doctor complaining of the symptoms and complications that typically precede and accompany the physiognomic alterations, among which we remember: headache, carpal tunnel syndrome, arthrosis and joint pain, reduced joint mobility, muscle pain , asthenia, changes in the menstrual cycle up to "amenorrhea, erectile dysfunction up to" impotence, thickened and oily skin, hypertrichosis, hyperhidrosis and bad body odors, enlargement of some internal organs (including liver, heart, kidneys, spleen, intestine and viscera in general - visceromegaly -), nodular goiter, cardiomyopathies, arterial hypertension, hoarse and deep voice, intestinal polyposis, visual changes, diabetes, severe insulin resistance, visual disturbances and urolithiasis.
An early treatment of acromegaly can prevent the onset and worsening of the various complications, which in the absence of adequate medical and pharmacological intervention can become lethal.
Excessive drug treatment with hGH, for the purpose of increasing muscle mass, can also cause acromegaly.This phenomenon is noticed by comparing the youth photos and the competitive ones of some bodybuilding champions, which highlight the physiognomic alterations typical of this syndrome.
The diagnosis of acromegaly is based on physical examination, associated with various instrumental investigations (MRI and CT of the pituitary) and laboratory (GH, IGF-1, GHRH, prolactin, oral glucose tolerance test, measurement of the visual field and others).
Causes
The pituitary is a small gland located at the base of the brain, behind the upper edge of the nose. Among the various pituitary hormones, GH participates in the process of development and growth of the skeletal system during childhood and puberty, while in the " adult guarantees muscle and bone trophism. In this period of life, the hypersecretion of GH is generally due to a benign tumor affecting the pituitary; more rarely, acromegaly is caused by external tumors (located for example in the lungs, pancreas or adrenal glands), which directly produce GH (very rare occurrence) or stimulate the pituitary gland to do so by releasing the hormone GHRH.
Care and Treatment
For further information: Medicines for the treatment of Acromegaly
The aim of the treatment is to reduce GH production and any pituitary tumor mass. To this end it is possible to intervene by administering specific drugs, such as somatostatin analogues (octreotide and lanreotide, which exert a powerful inhibiting action on GH secretion), dopamine agonists (bromocriptine) and growth hormone receptor antagonists (pegvisomant). Surgical therapy is performed transphenoidally, through an operation performed through the nasal cavity with the aim of removing the abnormal mass. The treatment of acromegaly with radiation therapy is indicated when surgery is impractical or does not produce the desired effects; it is however a second choice treatment, both for the long time necessary to obtain a significant improvement, both for permanent damage and causes at the hypothalamic-pituitary level.
