Chii Kalman chirwere? Kallman kuti Syndrome: symptomatology, chirwere uye mabatiro zvikuru

"Kurega" imwe chete Gene kungatungamirira zvirwere zvakakomba zvikuru, mushonga zvachose hazviiti. Mumwe pathologies izvi ndizvo Kallman kuti syndrome. chirwere ichi kunoitika runonyanya nevanhu, kunyange zvazvo dzimwe nguva vamiririri hadzi vanoiwana zvakare.

tsananguro mukuru chirwere

Kallman kuti chirwere - nemagene abnormality riri chinopfuudzwa sechinhu autosomal recessive, autosomal kuzivikanwa uye X-kwakabatana Gene.

Ichazivikanwa chirwere kusagadzikana pakati hypothalamus uye pituitary shwanu. Pakazoti wokutanga zvisiri unobereka kusunungura chinhu. Iyewo, kunobatsira kuderedza kugadzirwa gonadotropin ari pituitary shwanu. The chakaipa nokushanda kwenhengo gonads saka zvisisakwanisiki.

The zvinokonzera chirwere kukura

Kallman chirwere - chirwere majini kuti inogona kukonzerwa chinhu. Kukonzera chirwere ndiko kururama kubatana masero mukono nehadzi panguva pamuviri. Ndiko, muna imwe masero uye ane "wakaputsika" Gene chinopa refu ndingangokuvara mamwe zvinoratidza chirwere muvana.

Sezvinotarisirwa, Kushatiswa majini kunaka tsanga inogona kukanganiswa nezvinenge zvaitika. The chikuru kwavo mamiriro zvakatipoteredza. Kwenguva yakareba kunokuvadza namakemikari kukonzerawo kunotouraya siyana. Kallman chirwere chinogona kuratidza zvakajeka imene runoratidzwa, kana zvarakasiya diki kubva kwose kwose.

symptomatology chirwere

Dzaimiririrwa chirwere ane chaizvo zviratidzo, saka zviri nyore kuziva kusiyana nemamwe pathologies. Kana murwere akawanikwa Kallman kuti urwere, Zviratidzo zvingasanganisira:

• Kukwana yokuyaruka. Dzimwe nguva anenge chose vasipo. Somuenzaniso, varume yakaratidza shoma testicular - 3 chete Rine mamiririta (anowanzoita zvinofanira kuva 12 ML).
• Hyperplastic uye simba pigmentation yacho scrotum.
• A nemuumbi hypoplasia pakati yemukomana uye kweprostate gland.
• Kupiwa adipose zvinyama somukadzi.
• Full kana kukwana kusavapo kuti yechipiri dzepabonde zvatariswa. Somuenzaniso, vanhu vanogona kurasikirwa bvudzi riri mumuviri.
• Sexual passivity.
• System malformations: mumukaha mukanwa, mumukaha nomuromo gothic denga.
• Kutadza kunzwisisa kunhuwirira. Saka kuravidza maonero anoramba.

Zviratidzo izvi ringaratidzwa kana yekudyidzana simba. Zvose zvinoenderana ukuru abnormality ari hypothalamus uye pituitary.

Diagnostics Features

Kallman syndrome (mapikicha varwere kuzviisa pasi kushandiswa pfuma zvokurapa nyaya, ratidza zvakajeka kusiyana nemamiriro ngakuite muviri) hachisi chirwere nevakawanda zvikuru. Zvisinei, kubata chirwere chake chinofanira kuva vainyatsotarisirwa. It anopa Implementation yezviito zvakadaro:

1. Kuongorora dzinokosha kwevari mahormone pamuviri: estradiol, testosterone, prolactin, kukura kemikari. Zviongorore refu uye INDICATIVE. Zvinonzi inoitwa kanenge pavhiki.
2. Zvokuwedzera kuchabatsira nyanzvi kusiyanisa urwere kubva yepamutemo kunonoka kurara kukura.
3. Check aikanganiswa kunhuwirira. Izvi zvinoitwa chaizvo nyore, murwere anopiwa zvinhu (sipo, mafuta), ane simba zvakanaka. Maererano neongororo anopedzisa: chakaipa kunhuhwidza muvanhu kana kwete.
4. US itsvo uye testis.
5. MRI of pituitary uye hypothalamus. Uyezve, panguva kuongorora tomograph zvakafanira nehanya mapundu musoro kuti anogona kuonekwa zviratidzo pamusoro apa.
6. Kuunganidza nhoroondo yemhuri. Sezvo nechipiriso zvokurwara nhaka, kuwana ruzivo pamusoro Muzviitiko hosha iri murwere mhuri kuchaita zvigoneke kununura yakarurama zvikuru zvawanikwa.

Features kurapwa

Sezvo chirwere ari kuvabereki, ipapo kuchiparadza haangavi zvinobvira. Zvisinei, kurapwa kunofanira kuitwa. Pakarepo mushure nechirwere, murwere chinotarisirwa testosterone. The nzira zvinoitwa kwemwedzi mishomanana.

Nokuda mushonga nenyanzvi dzakadaro kunogona kuzadzisa kuvandudzika murwere mukuru ezvinhu utano, chinogonesa chido chokurara. Uyezve, verilizatsiya kubudirira. Zvadaro, vanachiremba kuramba kurapa, zvainanga kudzorera mukana chizvarwa dzisingaoneki kurei. Muchiitiko chino, hauchafambi kuiswa testosterone uye gonadotropins.

Ona kuti kana munhu yave chirwere "Kalman chirwere", mishonga achafanira kuitisa upenyu. It achabatsirawo kuti kwerunyararo yechipiri dzepabonde zvatariswa uye kudzorerwa pazera hwandaiva upenyu. Zvisinei, kana munhu murefu chirwere ichi angatsunga nevana, vanofanira nguva dzose kutanga genetics. Ngozi yokuva mwana akafanana zvokurwara anoramba yakakwirira chaizvo. In vakasara - ane zvishoma zviratidzo uye kurapwa - murwere anogona kurarama chaizvo zvinoitika muupenyu.

Panyaya kudzivirira, izvozvo anenge haako. Sezvingatarisirwa, zviri chinodiwa kuti tidzivise avo zvinogona kutungamirira "zororo" Gene. Stay utano!