Gigantism yana haifar da ɓarkewar ɓarna na hormone girma yayin ƙuruciya, wanda ke haifar da tsayi sosai. Wannan yanayin da ba a saba gani ba galibi yana da alaƙa da haɓaka ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar cuta, pituitary adenoma. A cikin 'yan shekarun nan, bincike ya bankado yawan shiga cikin abubuwan halittar kwayoyin halitta. Jiyya yana da wahala kuma galibi yana da yawa.

Gigantism wani nau'in acromegaly ne wanda ba a saba gani ba, yanayin da ke haifar da ɓarkewar ɓarkewar hormone mai girma, wanda kuma ake kira GH (don Girma hormone), ko hormone somatotrope (STH). 

Lokacin da yake faruwa kafin balaga (ƙaramin yaro da ƙaramin acromegaly), lokacin da ƙusushin ƙashi har yanzu ba a haɗa su ba, wannan ɓarna na hormonal yana tare da wuce kima da sauri na kasusuwa cikin tsayi har da na jiki duka. kuma yana haifar da gigantism.

Yaran da ke da cutar suna da tsayi sosai, tare da yara maza ke kaiwa mita 2 ko fiye a ƙarshen shekarunsu.

A yadda aka saba, ana fitar da hormone girma cikin jini ta ƙaramin gland a gindin kwakwalwa da ake kira gland. A cikin yara, babban aikinsa shine inganta haɓaka. GHRH ne ya tsara samar da hormone mai girma ta pituitary gland.girma hormone-sakewa hormone), hormone wanda hypothalamus na kusa ya samar.

Haɓakar haɓakar hormone girma a cikin yara masu gigantism galibi saboda bayyanar ƙwayar cuta mara kyau a cikin glandon pituitary, wanda ake kira pituitary adenoma: yaduwar sel masu samar da hormone yana bayyana matakinsa mara kyau.

A kasa da kashi 1% na lamuran, glandon pituitary yana da yawan motsa jiki saboda GHRH ya fi ƙarfafawa, wanda ƙwayar cuta ke iya samarwa a ko'ina cikin jiki.

bincike

Ana tuhumar Gigantism a gaban ci gaban da aka ba da alama (ana kwatanta ƙimar girma idan aka kwatanta da matsakaicin lanƙwasa), lokacin da yaron yake da tsayi sosai idan aka kwatanta da sauran danginsa. Binciken asibiti yana bayyana wasu abubuwan da ke da alaƙa da gigantism (duba alamun).

An tabbatar da ganewar asali ta hanyar gwajin jini, wanda ya haɗa da maimaita ma'aunin girma na hormone har ma da gwajin birki na glucose - hauhawar matakan glucose na jini bayan sha abin sha yana haifar da raguwar ɓoyayyen hormone girma, ba a lura da shi a cikin batutuwa da gigantism.

Ana yin gwajin hoto don gano ƙwayar da ke haifar da gigantism:

• MRI (hoton maganadisu na maganadisu) shine gwajin zaɓin don ganin adenoma na pituitary;
• na'urar daukar hotan takardu galibi ana amfani da ita don neman ciwace -ciwacen da ke ɓoye GHRH a cikin pancreas, ovaries ko adrenal gland;
• rediyo yana ba da damar ƙin abubuwan da ke haifar da ci gaban kashi.

Kasancewar pituitary adenoma na iya tsoma baki tare da aikin pituitary zuwa digiri daban -daban. Baya ga hormone girma, yana samar da prolactin (hormone lactation) kazalika da sauran homonin wanda rawar su shine haifar da ɓoyewa daga glandan adrenal, glandar thyroid da glands na al'aura. Cikakken kima na hormonal ya zama dole.

Ciwon kansa kuma yana iya damun jijiyoyin ido da haifar da rikicewar gani, saboda haka buƙatar yin cikakken bincike na ophthalmologic.

Za a iya buƙatar ƙarin ƙarin gwaje -gwajen don tantance ayyukan daban -daban waɗanda na iya haɗawa da gigantism.

Mutanen da abin ya shafa

Gigantism ya fi raɗaɗi fiye da acromegaly da ke shafar manya, kodayake wannan yanayin da kansa ba shi da yawa (sabbin lamura 3 zuwa 5 a cikin miliyoyin mazauna kowace shekara). A Amurka, lokuta ɗari ne kawai na gigantism aka gano.

Gigantism ya mamaye gaba ɗaya a cikin samari, amma wasu nau'ikan farkon farkon galibi mata ne

hadarin dalilai

Gigantism gabaɗaya yana gabatar da kansa azaman ɓacin rai da ɓarna na jijiyoyin jiki, wato yana faruwa a waje kowane mahallin gado. Amma akwai ƙarancin lokuta na adenomas na pituitary na iyali, gigantism na iya kasancewa ɗaya daga cikin abubuwan da ke tattare da cututtukan cututtukan da yawa, kamar su cutar McCune-Albrigh, rubuta 1 da yawa endocrine neoplasia (NEM1) ko neurofibromatosis. .

An gano abubuwa da yawa na ƙwayoyin cuta da ƙwayoyin cuta da ke da alaƙa da gigantism na pituitary, na gado ko a'a, a cikin 'yan shekarun nan. Babban binciken baya -bayan nan na kasa da kasa wanda masanin ilimin endocrinologist na Belgium Albert Beckers ya jagoranta, wanda ya shafi shari'o'i 208 na gigantism, don haka ya nuna shigar abubuwan kwayoyin halitta cikin kashi 46% na lokuta. 

Baya ga girman su, yara da matasa masu gigantism na iya gabatar da wasu alamomin da suka danganci cututtukan su:

• matsakaici (m) kiba,
• haɓaka girman girman kwanyar (macrocephaly), mai alaƙa ko a'a tare da takamaiman fuskokin fuska (tsinkaye, kumburin gaba, da sauransu)
• rikicewar gani kamar canji a fagen hangen nesa ko hangen nesa biyu,
• manyan hannaye da ƙafa mara nauyi, tare da yatsun hannu,
• neuropathies na gefe,
• cututtukan zuciya,
• ciwace -ciwacen daji,
• matsalolin hormonal…

Gudanar da yara masu gigantism yana da niyyar sarrafa ɓoyayyen ɓoyayyen hormone girma, wanda gabaɗaya yana buƙatar aiwatar da hanyoyin magani da yawa.

Jiyya na tiyata

An fi son cire tiyata na pituitary adenoma a matsayin magani na farko. Wannan aiki ne mai wahala, wanda galibi galibi ana iya yin shi cikin nutsuwa kodayake ya zama dole a buɗe cranium lokacin da adenoma yayi girma (macroadenoma).

Lokacin da ƙari ya yi yawa ko kusa da mahimman sifofi a cikin kwakwalwa, ba za a iya aiki da shi ba.

Radiotherapy

Za a iya ba da shawarar yin amfani da hasken X-ray ban da tiyata don lalata duk wasu ƙwayoyin tumor da suka rage da kuma magance ciwace-ciwacen ƙima (kusan zaman talatin). Wannan dabarar ba ta da zafi amma tana iya haifar da rashin daidaituwa na hormonal wanda ke da alhakin rikice -rikice daban -daban.

Kwanan nan, an bullo da dabarun aikin tiyata na Gamma Knife. Maimakon fatar kan mutum, yana amfani da gamma radiation, mafi ƙarfi kuma ya fi madaidaiciya fiye da haskoki, don lalata tumor a zama ɗaya. An keɓe shi ga ƙananan ciwace -ciwacen daji.

Molecules masu tasiri don rage ɓoyayyen ɓoyayyen hormone na iya yin umurni tare da aikin tiyata da farmaki, musamman idan cire ƙwayar cuta bai cika ba. Arsenal na warkarwa ya haɗa da analogues na somatostatin da dopamine, waɗanda ke da tasiri sosai amma suna iya samun manyan sakamako masu illa.