Acromegaly (from the Greek. Ἄκρος - limb and Greek. Μέγας - large) - a disease associated with impaired function of the anterior pituitary ( adenohypophysis ); accompanied by an increase (expansion and thickening) of the hands, feet , skull , especially the front part, etc. Acromegaly usually occurs after the completion of the growth of the body; develops gradually, lasts for many years. It is caused by the production of an excessive amount of growth hormone . A similar violation of the pituitary gland at an early age causes gigantism (in the absence of treatment, gigantism may combine with acromegaly over time). With acromegaly, headaches, fatigue, weakening of mental abilities, visual impairment, often sexual impotence in men and the cessation of menstruation in women are noted. Treatment - a surgical operation on the pituitary gland, x-ray therapy, the use of hormonal drugs that reduce the production of STH (bromocriptine, lanreotide).
| Acromegaly | |
|---|---|
 | |
| ICD-10 | E 22.0 |
| ICD-9 | 253.0 |
| Omim | 102200 |
| Diseasesdb | 114 |
| Medlineplus | 000321 |
| eMedicine | med / 27 derm / 593 |
| Mesh | D000172 |
Content
Etiology and pathogenesis
The disease is caused by overproduction of GH. In almost all patients, STH-secreting pituitary adenomas originating from somatotropic cells are detected. In the vast majority of cases, tumor transformations of these cells are provoked by activating mutations of the Gsalfa protein gene. The mutant Gsalfa protein continuously stimulates adenylate cyclase, which leads to increased proliferation of somatotropic cells and to increased production of GH. In rare cases, STH hypersecretion is caused by pituitary hyperplasia or excessive secretion of somatoliberin.
Clinical picture
Hypersecretion of GH in adults causes acromegaly; hypersecretion of STH in children usually leads to gigantism, and hypersecretion of STH in the late stages of sexual development (before the closure of the pineal gland growth zones) - to tall.
- The main signs of acromegaly
- Coarsening of facial features (an increase in the superciliary arches, cheekbones, overgrowth of soft tissues).
- Disproportionate increase in the size of the hands and feet.
- Other symptoms
- Excessive sweating; due to an increase in sweat glands and increased metabolism.
- Carpal tunnel syndrome; due to compression of the median nerve with hypertrophic fibro-cartilaginous tissue of the wrist joint.
- Deforming osteo-arthrosis; it is especially pronounced in large joints, it develops a second time due to the growth of bone tissue and joint deformities (under the influence of weight).
- Arterial hypertension; apparently due to sodium retention by the kidneys under the influence of GH
- Impaired carbohydrate tolerance or non-insulin-dependent diabetes mellitus; caused by the contra-action of STH.
- Hypercalciuria : apparently due to the stimulating effect of STH on the production of 1.25 (OH) 2D3.
- Galactorrhea; due to either its own lactogenic activity of STH, or a mixed adenoma, secreting not only STH, but also prolactin.
- Sleep apnea ; can be caused by both obstruction of the airways and inhibition of the respiratory center.
- Headache ; due to increased ICP.
- Causes of increased mortality in acromegaly: damage to the vessels of the brain, heart disease and lungs. In addition, with acromegaly, the risk of polyposis and colon cancer is increased .
Diagnostics
The characteristic appearance and analysis allows you to immediately suspect acromegaly. To confirm the diagnosis, the following laboratory tests are performed:
- Determine the basal level of GH in serum on different days (twice or thrice) to exclude accidental fluctuations in the level of GH.
- Sample with glucose. This is the most reliable confirmatory test. The patient is prescribed a solution containing 50-100 g of glucose, administered orally and after 60-90 minutes an analysis is performed.
Persons with Acromegaly
In chronological order
- Rondo Hatton (1894-1946) - American soldier, journalist and film actor. Most likely (though not exactly) the impetus for the disease was caused by mustard gas poisoning during the First World War. Becoming an actor, played the ugly villains in horror films [1] [2] .
- Tiye, Maurice (1903−1954) - French wrestler , born in the Urals in a French family; prototype of Shrek [3] .
- Keel, Richard (1939–2014) - American actor with a height of 2.18 m [4] .
- Andre Giant (1946-1993) - a professional wrestler and actor of Bulgarian-Polish origin [5] .
- Streuken, Karel (born 1948) is a Dutch character actor 2.13 m tall [6] .
- Igor and Grishka Bogdanova ( Igor et Grichka Bogdanoff ; born 1949) are French twins of Russian origin, television presenters-popularizers of space physics [7] .
- The Big Show (born 1972) is an American wrestler and actor with a height of 2.13 m.
- McGrory, Matthew (1973-2005) - American actor with a height of 2.29 m [4] .
- Valuev, Nikolai Sergeyevich (b. 1973) - Russian professional boxer and politician [8] .
- The Great Kali (born 1972) is an Indian wrestler, actor, powerlifter with a height of 2.16 m.
- Antoniu Silva (born 1979 ) is a Brazilian mixed martial artist .
- Choi Hong Man The Techno Goliath, Korean Colossus, Che Man (born 1980) - South Korean athlete, kickboxing , mixed martial arts (MMA) fighter .
- Mary Ann Bevann - Winner of "The Most Man-Like Woman in the World"
See also
- Swyer Syndrome
Notes
- ↑ Rondo Hatton on the Internet Movie Database
- ↑ Rondo Hatton on tcm.com
- ↑ "Maurice Tillet, catcheur professionnel et joueur de rugby atteint d'acromégalie (maladie rare qui perturbe la croissance des os) était surnommé„ l'ogre du ring ". Il aurait servi d'inspiration aux artistes de Dreamworks pour créer le personnage de Shrek. ”/ Shrek, inspiré par Maurice Tillet
- ↑ 1 2 "Actors Richard Kiel (" Jaws "in James Bond movies) and Matthew McGrory (" Karl the Giant "in Big Fish) both had acromegaly." / Misbehaving pituitaries
- ↑ Box Brown, “André The Giant: Life and Legend, First Second”, 2014, 240 pp .; pg. 51-55 ( ISBN 9781596438514 )
- ↑ Dennis McLellan for the Los Angeles Times. December 27, 1992 Shedding Light on a Rare Disease: Woman Hopes OC Group Will Increase Awareness of Life-Threatening Effects of Excessive-Growth Illness
- ↑ Le magazine Marianne laisse entendre que les deux frères auraient ingéré de la déhydroépiandrostérone, une hormone stéroïdienne réputée pour ses effets antivieillissement, ou bien de l'hormone de croissance, ayant pour effet secondaire lieacéme fil des années / Nathalie Gathié, Le vrai visage des Bogdanov // Marianne (magazine) , 16-22 octobre 2010; c. 67
- ↑ Valuev serait atteint d'acromégalie, un dérèglement hormonal qui provoque une augmentation anormale de la taille des pieds, des mains et des os crâniens.
Literature
- Baranov V.G., Diseases of the endocrine system and metabolism, 2nd ed., M., 1957
- Guidelines for Clinical Endocrinology, ed. E. A. Vasyukova, M., 1958
- I.I.Dedov, G. A. Melnichenko, VV Fadeev., Endocrinology textbook "GOOETAR-Media .2012.
- Acromegaly // Great Soviet Encyclopedia : in 66 volumes (65 volumes and 1 additional) / Ch. ed. O. Yu. Schmidt . - M .: Soviet Encyclopedia , 1926-1947.
Links
- Acromegaly / The site of the medical directory "Healthy Topic"
- Interregional Charitable Public Organization of Patients with Pituitary Pathology "Giant"
- Giant's Online Schools for Patients with Pituitary Pathology