Acromegaly

Most often, there is a pituitary tumor, which in 20% of patients contains eosinophilic granules, and in the rest - chromophobe granules. Acromegaly can be a consequence of ependymomas of the third ventricle, glioma of the hypothalamus.

An erased form of acromegaly may occur with constitutionally determined hypothalamic-pituitary dysfunction. Signs of acromegaly may also be detected with the syndrome of the "empty" sella turcica.

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Causes of Acromegaly

The disease is mainly associated with hyperproduction of growth hormone, which can be primarily pituitary, caused by the development of an autonomous tumor, or has a hypothalamic origin, associated with excessive secretion of STH-releasing factor or insufficient secretion of somatostatin. The latter opinion is supported by the possibility of the formation of a pituitary tumor with hypersecretion of growth hormone as a result of long-term stimulation of STH-releasing factor.

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Symptoms of Acromegaly

Symptoms of acromegaly usually appear after age 20 and develop gradually. Early signs of acromegaly are swelling and hypertrophy of the soft tissues of the face and extremities. The skin thickens, and the appearance of skin folds increases. The increase in soft tissue volume makes it necessary to constantly increase the size of shoes, gloves, and rings.

Generalized hirsutism, increased pigmentation, the appearance of skin fibrous nodules, increased oiliness of the skin, and increased sweating are often encountered. Bone changes join later, develop more slowly, and consist of thickening of the cortical layer of bones, the formation of bone growths, and spikes at the end of the phalanges.

Hypertrophic arthropathy often results in arthralgia and deforming arthritis. The enlargement of the lower jaw leads to prognathism, protrusion of the lower incisors, and increased spaces between the teeth. There is an overgrowth of the facial skeleton, an increase in the size of the bone sinuses, and hypertrophy of the vocal cords, leading to a coarsening of the voice. If hypersecretion of growth hormone begins in childhood, then there is a proportional increase in growth with the development of true gigantism, which is usually accompanied by hypogonadism. A combination of gigantism and acromegaly is possible, which indicates a fairly long-standing onset of the disease. Patients often have signs of visceromegaly, most often in the form of cardio- and hepatomegaly. A fairly common symptom is various tunnel syndromes (usually carpal tunnel), which appear as a result of the proliferation of ligaments and fibrous tissue surrounding the nerves.

As a rule, acromegaly is combined with varying degrees of severity of signs of hypogonadism, obesity, and diabetes mellitus.

Among patients with typical acromegaly, it is necessary to distinguish individuals with acromegaloid condition, characterized by weakly expressed or transient signs of acromegaly. Acromegaloid condition, or, according to H. dishing, "fleeting acromegaly", is typical for periods of hormonal changes: puberty, pregnancy, menopause. Individual signs of acromegaly can be found in cerebral obesity, diabetes insipidus, idiopathic edema, and "empty" sella turcica syndrome.

Differential diagnosis of acromegaly

The possibility of ectopic production of growth hormone by bronchogenic lung cancer and carcinoid tumors should be kept in mind. Some of these tumors contain GH-releasing factor.

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Treatment of acromegaly

There are three approaches to therapy - surgical, radiation and pharmacological. The first two approaches are used in the presence of a tumor process. Pharmacotherapy with estrogens and progesterone, as a rule, does not give a sufficient effect. Drugs that affect the metabolism of neurotransmitters in the central nervous system are used (L-DOPA, parlodel, lisinil, metergoline, cyprogentadine). L-DOPA has the ability to quickly suppress the secretion of STH in acromegaly. Bromocriptine (parlodel) therapy is widely used at a dose of 10-15 mg / day, depending on tolerance for an indefinitely long time. There are reports that parlodel retains activity in suppressing hypersecretion of STH even with continuous administration for 7 years. Parlodel is not only able to suppress the secretion of STH, but also has antitumor activity. Treatment with parlodel should be recommended for tumor processes in the presence of contraindications to surgical intervention and radiation therapy. It is mainly used for hypersecretion of STH and the absence of a tumor process. Treatment of acromegaly with cyprogentadine (peritol, deseril) is carried out for a long time at a dose of 25 mg / day.