I-Gigantism idalwa yimfihlo eyeqile ye-hormone yokukhula ngesikhathi sobuntwana, okuholela ekuphakameni okukhulu kakhulu. Lesi simo esingajwayelekile sivame ukuxhumaniswa nokwakhiwa kwesimila esibi se-pituitary gland, i-pituitary adenoma. Eminyakeni yamuva nje, ucwaningo luveze ukubandakanyeka okuvamile kwezici zofuzo. Ukwelashwa kunzima futhi kuvame ukwenziwa ngezindlela eziningi.

I-Gigantism iyindlela engavamile kakhulu ye-acromegaly, isimo esibangelwa uketshezi olweqile lwe-hormone yokukhula, ebizwa nangokuthi yi-GH (ye i-hormone yokukhula), noma i-hormone somatotrope (STH). 

Lapho kwenzeka ngaphambi kokuthomba (i-juvenile ne-infantile acromegaly), lapho ama-cartilage amathambo engakahlangani, lokhu kungahambi kahle kwe-hormonal kuhambisana nokukhula ngokweqile nokushesha kwamathambo ngobude kanye nasemzimbeni wonke. futhi kuholele kugigantism.

Izingane ezinalesi sifo zinde ngokungajwayelekile, kanti abafana bafinyelela kumamitha ama-2 noma ngaphezulu sebeneminyaka eyevile kwengu-XNUMX.

Imvamisa, i-hormone yokukhula ikhishwa egazini yindlala encane engxenyeni yobuchopho ebizwa nge-pituitary gland. Ezinganeni, indima yalo enkulu ukukhuthaza ukukhula. Ukukhiqizwa kwe-hormone yokukhula ngendlala ye-pituitary uqobo kulawulwa yi-GHRH (i-hormone yokukhulula ekhipha i-hormone), i-hormone ekhiqizwa yi-hypothalamus eseduze.

I-hormone yokukhula i-hypersecretion ezinganeni ezine-gigantism imvamisa kakhulu ngenxa yokuvela kwesimila esibi ebusheni be-pituitary, esibizwa nge-pituitary adenoma: ukwanda kwamaseli akhiqiza ama-hormone kuchaza izinga lalo eliphakeme ngokungajwayelekile.

Ezimweni ezingaphansi kuka-1%, indlala yamapayipi iyasebenza ngokweqile ngoba ivuselelwa ngokweqile yi-GHRH, ekhiqizwa ngokweqile ngesimila esingatholwa noma ikuphi emzimbeni.

kwesifo

I-Gigantism isolwa ebusweni bokukhula okuphawuleka kakhulu (ijika lokukhula lokuphakama liqhathaniswa nejika elijwayelekile), lapho ingane yinde kakhulu uma iqhathaniswa namanye amalungu omndeni wayo. Ukuhlolwa komtholampilo kuveza okunye okungajwayelekile okuhambisana ne-gigantism (bona izimpawu).

Ukuxilongwa kuqinisekiswa ukuhlolwa kwegazi, okubandakanya izilinganiso eziphindaphindwayo ze-hormone yokukhula kanye nokuhlolwa kwe-glucose braking - ukukhuphuka kwamazinga eglucose egazini kulandela ukumuncwa kwesiphuzo esinoshukela kudala ukwehla kokucasha kwe-hormone yokukhula, okungabonwa ngezihloko gigantism.

Ukulingisa izivivinyo kwenziwa ukuze kutholakale isimila esibangela i-gigantism:

• I-MRI (imaging resonance imaging) wukuhlolwa kokukhetha ukubona ngeso lengqondo i-pituitary adenoma;
• isithwebuli sisetshenziselwa ukubheka izicubu ezifihla i-GHRH kuma-pancreas, ama-ovari noma izindlala ze-adrenal;
• i-radiography yenza kube nokwenzeka ukufakazela ukungajwayelekile kokukhula kwethambo.

Ukuba khona kwe-pituitary adenoma kungaphazamisa ukusebenza kwe-pituitary kumazinga ahlukahlukene. Ngaphezu kwe-hormone yokukhula, ikhiqiza iprolactin (i-lactation hormone) kanye namanye ama-hormone indima yawo ukudala uketshezi oluvela kwizindlala ze-adrenal, indlala yegilo nezindlala zangasese. Ukuhlolwa okuphelele kwamahomoni kuyadingeka.

Isimila singaphinde sicindezele izinzwa ze-optic futhi sidale ukuphazamiseka okubukwayo, yingakho kunesidingo sokuhlolwa ngokuphelele kwe-ophthalmologic.

Olunye uvivinyo olwengeziwe lungacelwa ukuthi kuhlolwe ukungasebenzi kahle okuhlukahlukene okungahlotshaniswa ne-gigantism.

Abantu abathintekayo

I-Gigantism iyivelakancane kune-acromegaly ethinta abantu abadala, yize lesi simo ngokwaso singavamile (amacala amasha ayi-3 kuye kwangama-5 kwisakhamuzi ngasinye ngonyaka). E-United States, kutholakale amacala ayikhulu kuphela we-gigantism.

I-Gigantism ibusa kakhulu kubafana, kepha ezinye izinhlobo zakuqala kakhulu ikakhulukazi ezabesifazane

Izici zengozi

I-Gigantism ngokuvamile iziveza njenge-hormone ehlukile futhi engajwayelekile, okusho ukuthi yenzeka ngaphandle kwanoma yimuphi umbandela wefa. Kepha kunezigameko ezingavamile ze-pituitary adenomas yomndeni, i-gigantism nayo ingaba enye yezakhi zama-syndromes amafa amaningi, njengeMcCune-Albrigh syndrome, thayipha i-1 endocrine neoplasia (NEM1) noma i-neurofibromatosis. .

Ukuthambekela okuningana kwezakhi zofuzo nokwenziwa genomic okuhlobene ne-pituitary gigantism, ifa noma cha, kutholakele eminyakeni yamuva. Ucwaningo olukhulu lwasemuva olwaluqondiswa yisazi se-endocrinologist saseBelgium u-Albert Beckers, oluhlanganisa amacala angama-208 we-gigantism, ngaleyo ndlela lwaqhakambisa ukubandakanyeka kwezici zofuzo kumacala angama-46%. 

Ngaphezu kwesiqu sabo esikhulu, izingane nentsha ene-gigantism ingaletha okunye ukubonakaliswa okuhlobene nokugula kwabo:

• ukukhuluphala okuphakathi (okuvamile),
• ukukhula okunehaba kwevolumu yogebhezi (i-macrocephaly), ehambisana noma hhayi enezici ezithile zobuso (i-prognathism, i-frontal bumps, njll.)
• ukuphazamiseka okubukwayo njengokushintsha emkhakheni wokubona noma ukubona kabili,
• izandla nezinyawo ezinkulu ngokungajwayelekile, eneminwe emincane,
• i-neuropathies yomngcele,
• ukuphazamiseka kwenhliziyo,
• izimila ezinobungozi,
• ukuphazamiseka kwamahomoni…

Ukuphathwa kwezingane ezine-gigantism kuhlose ukulawula uketshezi lwazo olweqile lwe-hormone yokukhula, okudinga kakhulu ukusebenzisa izindlela zokwelashwa eziningana.

Ukwelapha okuhlinzwa

Ukususwa kokuhlinzwa kwe-pituitary adenoma kukhethwa njengokwelashwa komugqa wokuqala. Lokhu kungumsebenzi onzima, ovame ukwenziwa ngamakhala yize kudingeka ukuvula i-cranium lapho i-adenoma inkulu (macroadenoma).

Lapho isimila sikhulu kakhulu noma sisondele kakhulu ezakhiweni ezibalulekile ebuchosheni, asikwazi ukuhlinzwa.

Radiotherapy

Kunganconywa imisebe ye-X-ray ngaphezu kokuhlinzwa ukubhubhisa noma yimaphi amangqamuzana e-tumor asalayo futhi welaphe izicubu eziphindaphindayo (cishe izikhathi ezingamashumi amathathu). Le ndlela ayinabuhlungu kepha ingadala ukungalingani kwe-hormonal okubhekene nokuphazamiseka okuhlukahlukene.

Muva nje, kwethulwe inqubo ye-Gamma Knife radiosurgery. Esikhundleni se-scalpel, isebenzisa imisebe ye-gamma, enamandla kakhulu futhi eqonde kakhulu kune-x-ray, ukuqeda isimila ngasikhathi sinye. Kugcinelwe izicubu ezincane.

Ama-molecule asebenza ekunciphiseni uketshezi lwe-hormone yokukhula angabekwa ngokuhlangana nokuhlinzwa nokwelashwa ngemisebe, ikakhulukazi uma ukususwa kwesimila kungaphelele. Isikhali sokwelashwa sibandakanya okufana ne-somatostatin ne-dopamine, esebenza kahle kepha ingaba nemiphumela emibi kakhulu.