Laron chirwere: zvinokonzera, chirwere, kurapwa, fungidziro

Laron chirwere - mamiriro pathological umo muviri hausi kukwanisa kugamuchira uye kushandisa kukura kemikari. Vakawanda chiratidzo pakati kutyora - dwarfism. Zvimwewo zviratidzo vangazivikanwa neutera tsandanyama richikwidibira nesimba, mwana yomuropa, kunonoka kurara kukura, muzvidimbu kupfupikisa, uyewo kufutisa. Kakawanda kukonzera kupera kuchinja (kunotouraya) ane Gene mhosva kukura. Zvinonzi nhaka ive autosomal recessive unhu.

zviratidzo

Laron chirwere haiwanzowaniki chirwere. Panyaya iyi, nechirwere pamusoro vachangoberekwa, kazhinji vane avhareji dziine kukura uye uremu, asi mune ramangwana kukura kwavo mumuviri DZEMUNHU DZINODZORA zvinoshamisa. Untreated murume mukuru kusvika maximal kukura 130 cm. Growth vakadzi idukusa masendimita 120.

Kuwedzera kune yakaderera kukura pamberi zvokurwara anotevera zviratidzo:

• Tsandanyama simba, kushaya kutsungirira;
• Yomuropa yomwana;
• kunonoka chipotera;
• sikarudzi disproportionately diki;
• dzakaonda, bvudzi simba;
• kutyora maumbirwo kwemeno;
• Wakafupisa Mbabvu;
• kufutisa;
• nevamwe chiso zvinhu (akakurumbira huma, mungarava mhino, zvitema sclera chirwere).

zvikonzero

Laron chirwere kukonzera mutation dzine kukura kemikari Kanotokonya - mapuroteni dzawanikwa wokunze membranes nemasero rose muviri. Kanotokonya kukura kemikari zvichiitirwa kuziva uye Activate ari somatotropin, nokudaro kutanga kukura uye weboka masero. Kunotouraya of Gene ruchavhiringidza dzose kushanda zvipenyu mashandiro uye kukonzera mhirizhonga foni kukura. Zviratidzo zvirwere zvakawanda zvinokonzerwa nekusagona vaunze hormone yechipiri - jekiseni-kufanana kukura chinhu.

nhodzera

Kazhinji, Laron chirwere kuvana iri nhaka munzvimbo autosomal recessive nenzira. Izvi zvinoreva kuti kukundikana kunokonzerwa kunotouraya mune zvose maBhaibheri njodzi Gene muchitokisi chimwe nechimwe. Kazhinji vabereki varwere zvinetso vari vatakuri imwe kopi mutated Gene, uye kashoma kutambura kubva hwokufananidzira zvinoratidza kukanganisa. Apo vatakuri maviri recessive autosomal chirwere akaberekwa mwana nhaka chirwere pangozi ndiyo 25%; zvichida kuva pusa mutakuri sei nechimwe vabereki - 50%; mukana kwete nhaka chero kanganisika kana mutated Gene - 25%.

Maererano nedzimwe mishumo, pane mhuri umo Laron chirwere iri nhaka munzvimbo autosomal kureva nenzira. Muzviitiko zvakadaro, nokuti kupararira kwezvirwere chinhu mutation mune imwe kopi njodzi Gene muchitokisi chimwe nechimwe. Dzimwe nguva izvi hazvisati zvave adopts mwana kubva mubereki zvokurwara murwere. Anozivikanwa uye nyaya itsva, kwete nhaka nemagene kunotouraya, kana kukanganisa kunokwanisa kuonekwa mune vana vari mumhuri vakanga vasina nyaya kuroorwa chirwere. Munhu ane yasimbiswa zvokurwara mishumo mutated Gene vana vavo 50% nyaya.

Diagnostics

Pachapera tinyatsonzwisisa zviratidzo pachena uye zviratidzo nyanzvi inogona rokuongorora Laron syndrome (mapikicha vanhu vane zvokurwara cm. Munyaya). mamwe miedzo yaitwa kusimbisa ichi chirwere uye inoshanda Kusiyanisa, kusanganisira muedzo chaiwo mahormone ropa. Most kazhinji vanhu pamwe chirwere akasangana yepamusoro kwevari kukura kemikari panguva iyoyo pamwe vasanyanye jekiseni-kufanana kukura chinhu. Mune zvimwe zviitiko, vanachiremba akarayira masero zvidzidzo kusimbisa kuti majini mutation. chirwere matanho zvabuda kubvumira kuona chaizvoizvo Laron syndrome.

mabatiro

Chose kuporesa ichi pathological ezvinhu mushonga ano haisati mune simba. Innovative kurapa achinyanya nezvezvaiitwawo kumhanyisa kukura, iyo inopiwa pamusoro subcutaneous jekiseni insulin yakakodzera asipo kemikari. Fuma ude Linear kukura (kureba), izvozvo Inowedzera kukura uropi uye kunodzivisa panowanikwa metabolic zvirwere zvinokonzerwa kwenguva refu kushaikwa zvakakodzera mapurotini. Uyezve, majekiseni dzakadaro kusimudza ropa shuga, kutadzisa kuunganidza remafuta akawandisa uye yekudyidzana toned tsandanyama tishu. Mabatirwo jekiseni-kufanana kukura chinhu chinofanira kuitwa pasi kuramba pokudzora vanachiremba, sezvakanga nekumwisa runodiwa chipimo chemushonga kungaita zvoutano.

mataridzikiro

Long-rokuti fungidziro zvinokonzerwa chaizvo tariro. Laron chirwere hazvirevi kukanganisa upenyu anotarisirwa. Uyezve, mutakuri nhaka chirwere ndoda mukana kenza uye asiri jekiseni chirwere cheshuga mellitus. Pari taiita pokutsvakurudza kuziva Links pakati Laron urwere, nemagene kunotouraya, kemikari kukura nyanyisa anomera. Zvinogoneka kuti mune izvi Gene kuchinja ndiyo musimboti akundwe kenza.