GIANTISM (gigantismus; grech, gigas, gigant[os] the giant) — the clinical syndrome which is developing owing to hyperproduction somatotropic) hormone and characterized by excessive, rather proportional (fig. 1) or disproportionate growth.
Meets concerning 1 — 3 on 1000 people
of G. can be at the adenomas of a hypophysis and other endocrine diseases connected with dysfunction of gipotalamo-pituitary system — acromegalias (see), hypogonadism (see), Klaynfelter's syndrome (see. Klaynfeltera syndrome ), a dispituitarizm of youthful age (see. Dispituitarizm pubertal and youthful ).
Partial G., at Krom separate parts or a half of a body (fig. 2) increase, is considered as anomaly of development; infections, injuries can be an incitement to its emergence.
Etiology and pathogeny
Hyperproduction somatotropic hormone (see) it is more often connected with a hyperplasia of eosinophial cells hypophysis (see). Also infectious processes in interstitial and pituitary area can be the cause of this hyperproduction. Consider as age option of an acromegalia. At young people with unfinished ossification of epiphyseal cartilages hyperproduction of a growth hormone can lead to the strengthened periosteal and epiphyseal growth. Excess of this hormone at adults strengthens the periosteal growth of bones and leads to disproportionate increase in separate parts of a skeleton. Such point of view does not explain, however, the reason of development of an acromegalia at youthful and children's age (including at newborns). Suggest about various sensitivity of peripheral fabrics to a growth hormone.
Develops usually for a row of years. Increase in growth is shown in the prepubertatny and pubertal periods, at the age of 10 — 15 years. Over time akromegaloidny enlargement of features, a thickening of flat bones, specific elongation of extremities can join.
The pathological anatomy
Pathological anatomy at G. is similar to changes at an acromegalia: insufficient ossification of seams, growth cartilage, sometimes kyphoscoliosis of a backbone, hypoplasia of the sexual device; the splanchnomegaly is found (more often in the presence of signs of akromegaloidny growth). Find an eosinophilic hyperplasia or adenoma of a hypophysis, the tumor of malignant character is more rare.
A clinical picture
Patients note deterioration in the general state, headaches, weakness, bystry fatigue, extremity pains. Animal force can be raised in the beginning, but further sharply decreases. Memory worsens, infantilism of mentality is often observed. From the very beginning of a disease note a hypogenitalism. Men sometimes can have in the beginning a raised functional activity of gonads.
At women primary amenorrhea or the early termination of periods, infertility is observed. There can be also other hormonal disturbances (symptoms of not diabetes mellitus, hypo - or a hyperthyroidism, decrease in tolerance to glucose and even symptoms of a diabetes mellitus).
At increase in the sizes of adenoma of a hypophysis at patients the bitemporal hemianopsia amplifies; at a research of an eyeground note congestive nipples, narrowing of fields of vision; at rentgenol, a research define increase in the sizes and destruction of the Turkish saddle. Cystous regeneration of adenoma is clinically shown by a picture of a hypopituitarism: there is a sharp weakness, a hypothermia, arterial hypotonia, spontaneous hypoglycemic states.
the Main method of treatment is radiation therapy. At G. which is not connected with tumoral defeat, the total exposure dose makes 1500 — 3000 r, a dose of one-time radiation (daily or every other day) — 100 — 140 r; radiation is made from three fields — two temporal and frontal (daily or every other day). At adenoma of a hypophysis the total dose is raised to 9000 — 10 000 r (one-time 150 — 200 r), radiation is made from three or five (frontal, two temporal and two maxillary and malar) fields. At repeated courses the total dose is reduced to 5000 — 8000 rubles. Use also telegamma therapy and implantation of drugs 198Au and 90Y in fabric of a tumor.
To girls and young men with G. which developed in the prepubertatny period appoint sex hormones for the purpose of suspension of growth. At secondary dysfunctions of sexual, thyroid, pancreatic glands carry out the corresponding hormonal therapy.
the Forecast cannot be considered favorable. Patients perish usually from intercurrent diseases.
See also Hyperpituitarism .
Bibliography: Zhukovsky M. A. Children's endocrinology, page 279, M., 1971; To Milk Sh.-M. Therapy of endocrine diseases, the lane from Romanians., page 60, Bucharest, 1966; The Multivolume guide to internal diseases, under the editorship of E. M. Tareeva, t. 7, page 341, L., 1966, bibliogr.; Physiology of the child of early age, under the editorship of 3. I. Kolarova (Biryukova) and V. Ga-teva, the lane with bolg., page 328, Sofia, 1970; Havin I. B. iislambekovr. K. Basic endocrine diseases and their treatment, Tashkent, 1975, bibliogr.; Asgo-m6galie-gigantisme, publ. par J. Hardy e. a., P *, 1973, bibliogr.
E. P. Tikhonova.