Gigantism inokonzereswa nekuvanda kwakanyanya kwekukura kwehormone panguva yehucheche, izvo zvinoguma nehurefu hwakakura kwazvo. Ichi chiitiko chisingawanzoitika chinowanzo sunganidzwa nekukudziridzwa kwechunhu chisina kumira chepituitary gland, iyo pituitary adenoma. Mumakore achangopfuura, kutsvagurudza kwakaburitsa kuwanda kwekubatanidzwa kwemajini ezvinhu. Kurapa kwakaoma uye kazhinji multimodal.

Gigantism chimiro chisingawanzoitika cheacromegaly, mamiriro anokonzereswa nekuwandisa kwekuvanzika kwehormone yekukura, inonziwo GH (ye Kukura hormone), kana hormone somatotrope (STH). 

Kana zvikaitika pamberi pekuyaruka (vechidiki uye vacheche acromegaly), apo mapfupa emapfupa asati abatana, uku kusarongeka kwehomoni kunoenderana nekukura kwakanyanya uye nekukurumidza kwemapfupa pakureba pamwe nemuviri wese. uye inotungamira kune gigantism.

Vana vane mamiriro acho akareba zvisingaenzaniswi, nevakomana vanosvika 2 metres kana kupfuura mumakore avo ekupedzisira.

Kazhinji, mahormone ekukura anosunungurwa muropa nekanhu kadiki pazasi pehuropi inonzi pituitary gland. Muvana, basa rayo guru nderekusimudzira kukura. Iyo kugadzirwa kwekukura kwehomoni neiyo pituitary gland pachayo inodzorwa neGHRH (kukura hormone-kusunungura mahormone), Hormone inogadzirwa neiyo hypothalamus iri padyo.

Kukura kwehormoni hypersecretion muvana vane gigantism kunowanzo kuve nekuda kwekuonekwa kwechironda chisina kuchena muganda repituitary, rinonzi pituitary adenoma: kuwanda kwemasero anoburitsa mahormone kunotsanangura huwandu hwayo zvisina kujairika.

Mune isingasviki 1% yematambudziko, iyo pituitary gland yakanyanyisa nekuti iri pamusoro-inokurudzirwa neGHRH, inogadzirwa yakawandisa nebundu rinogona kuwanikwa chero mumuviri.

chirwere

Gigantism inofungidzirwa mukutarisana nekukurumidza kukurumidza kukura (iyo yekukura kukura curve inofananidzwa neavhareji curve), kana mwana akareba kwazvo achienzaniswa nedzimwe nhengo dzemhuri yake. Kuongororwa kwekiriniki kunozivisa zvimwe zvisina kujairika zvine chekuita negigantism (ona zviratidzo).

Iko kuongororwa kunosimbiswa nekuongororwa kweropa, uko kunosanganisira kudzokororwa zviyero zvekukura kwehomoni pamwe nekuyerwa kweglucose bvunzo - kukwira kwemazinga eruglucose eropa zvichitevera kunwa kweshuga chinodzvinyirira kunowanzoita kuderera kwekukura kwehomoni secretion, isina kucherechedzwa muzvidzidzo ne gigantism.

Kufungidzira bvunzo kunoitwa kutsvaga iro bundu rinokonzera gigantism:

• MRI (magnetic resonance imaging) ndiko kuongorora kwesarudzo yekufungidzira pituitary adenoma;
• iyo scanner inonyanya kushandiswa kutsvaga mamota anovharira GHRH mupancreas, ovaries kana adrenal gland;
• iyo radiografia inoita kuti zvikwanise kuratidza kusagadzikana kwekukura kwemapfupa.

Kuvapo kwepituitary adenoma kunogona kukanganisa mashandiro epituitary kumadhigirii akasiyana. Pamusoro pekukura kwehormone, inoburitsa prolactin (lactation hormone) pamwe nemamwe mahormone ane basa rekumutsa masevhisi kubva kune adrenal gland, iyo thyroid gland uye genital gland. Kuongorora kwakakwana kwehormonal saka kwakakosha.

Bundu iri rinogona zvakare kumanikidza mitsipa yemaziso uye kukurudzira kusagadzikana kwekuona, nekudaro kudiwa kwekunyatso kuongorora ophthalmologic.

Mimwe bvunzo dzekuwedzera dzinogona kukumbirwa kuti dziongorore madhisikirwo akasiyana siyana anogona kuve nechokuita negigantism.

Vanhu vane hanya

Gigantism yakanyanya kushomeka kupfuura acromegaly inobata vanhu vakuru, kunyangwe ichi chiitiko pachacho chisingawanzoitika (3 kusvika ku5 nyowani nyowani pamamiriyoni vagari pagore). MuUnited States, zana chete nyaya dzegigantism dzakazivikanwa.

Gigantism inotungamira zvakanyanya muvakomana, asi mamwe mafomu ekutanga zvakanyanya vakadzi

Zvinhu zvinotyisa

Gigantism inowanzozviratidza seyakavanzika uye inowanzoitika hormonal pathology, ndiko kuti kunoitika kunze kwenzvimbo yenhaka. Asi pane zviitiko zvisingawanzoitika zvemhuri pituitary adenomas, gigantism inogona zvakare kuve chimwe chezvinhu zvekugara nhaka multitumor syndromes, senge McCune-Albrigh syndrome, mhando 1 yakawanda endocrine neoplasia (NEM1) kana neurofibromatosis. .

Akawanda magene uye genomic kukanganisika kwakabatana nepituitary gigantism, nhaka kana kwete, zvakaonekwa mumakore apfuura. Chidzidzo chikuru chekudzokorodza kwepasirese chakarongedzwa neBelgian endocrinologist Albert Beckers, ichifukidza mazana makumi maviri neshanu ezviitiko zvegigantism, nekudaro yakaratidza kubatanidzwa kwemajini zvinhu mu208% yematambudziko. 

Pamusoro pehukuru hwavo, vana nevachiri kuyaruka vane gigantism vanogona kuunza zvimwe zviratidzo zvine chekuita nehurwere hwavo:

• kuenzanisa (kazhinji) kuneta,
• kuwedzeredza kukura kweiyo vhoriyamu dehenya (macrocephaly), inosanganisirwa kana kwete nehumwe hunhu maficha (kufungidzira, kumberi mapundu, nezvimwewo)
• kukanganiswa kunoonekwa sekushandurwa mumunda wekuona kana kuona kaviri,
• maoko nemakumbo zvakakura zvisina kujairika, neminwe matete,
• peripheral neuropathy,
• matambudziko emwoyo,
• mamota akanaka,
• kusagadzikana kwehomoni ...

Iwo manejimendi evana vane gigantism anovavarira kudzora kwavo kwakawandisa kusevha kwehormone yekukura, iyo inowanzo inoda kuita maitiro akati wandei ekurapa.

Kuvhiya kurapa

Kuvhiya kubviswa kwepituitary adenoma inosarudzika senzira yekutanga kurapwa. Uku kwakaoma kuvhiya, uko kunogona kazhinji kuitwa nemhino kunyangwe zvichidikanwa kuvhura iyo cranium kana adenoma yakakura (macroadenoma).

Kana bundu racho rakanyanya kukura kana kuswedera padhuze nezvivakwa zvakakosha muuropi, harigone kuvhiyiwa.

Radiotherapy

X-ray irradiation inogona kukurudzirwa mukuwedzera kune kuvhiyiwa kuti uparadze chero zvasara maseru emota uye kurapa matomu anodzokororazve (angangoita makumi matatu zvikamu). Maitiro aya haarwadzi asi anogona kukonzera kusaenzana kwehomoni kunokonzera kusagadzikana kwakasiyana.

Munguva pfupi yapfuura, iyo Gamma Knife redhiyo maekisheni akaunzwa. Panzvimbo pechikwiriso, inoshandisa gamma radiation, ine simba rakanyanya uye yakanyatsojeka kupfuura x-ray, kuparadza bundu mune imwechete. Inochengeterwa tumamota tudiki.

Mamorekuru anoshanda mukuderedza kukura kwehormoni secretion inogona kupihwa pamwe chete nekuvhiya uye radiation radiation, kunyanya kana bundu kubvisa risina kukwana. Iyo yekurapa arsenal inosanganisira zvakafanana zve somatostatin uye dopamine, ayo anoshanda chaizvo asi anogona kuve neakakosha mhedzisiro.