Pituitary dwarfism: akpata, mgbaàmà, ọgwụgwọ nke ọrịa

Pituitary dwarfism (dwarfism) - a ọrịa ji ibu iberibe na a anụ ahụ mmepe ọrịa n'ihi secretion nke ihu n'ọnụ ọnụ nke pituitary somatotropin - ibu hormone. Ọ bụ nke a hormone bụ maka na usoro nke cell nkewa ahụ mmadụ.

Types of pituitary dwarfism

Dị ka etiological akpata pituitary dwarfism (23.0 mb) koodu bụ:

1. Primary, mmepe nke nke pụtara dị ka a n'ihi nke pituitary ọrịa, na dị ka a na ya pụta, Mbelata ibu hormone etoju.
2. Hypothalamic, mmepe nke nke ruru a ọnụ ke larịị nke hypothalamic atọhapụ homonụ, e mere iji nwere mmetụta na pituitary gland.
3. Esonyere anụ ahụ iguzogide ibu hormone. Ya mmepe bụ n'ihi na enweghi ike anabata na iche na nsụkọrọ ime ọma na ihe nke hormone.

etiopathogenesis

Akpata pituitary dwarfism nwere ike a mmeri nke pituitary: akpụ (meningioma, craniopharyngioma, chromophobe adenoma), trauma, na-egbu egbu, na-efe efe mmebi mezhutochno- pituitary region ma ọ bụ dysregulation nke pituitary gland site hypothalamus. Ma, ọtụtụ ọrịa forms akọ a mkpụrụ ndụ ihe nketa ụdị.

Ihe kasị ekesa nwere pangipopituitarnaya dwarfism, nke nēche na-ketara tumadi recessive ụdị. E ntule na e nwere ihe abụọ na ụdị nnyefe iche nke a na ọrịa - autosomal na site X-chromosome. Yana secretion nke ibu hormone ọrịa na nke a n'ụdị dwarfism na emee Ọtụtụ na aghara secretions nke thyroid-akpali akpali hormone na gonadotropins.

Na na na naa esogbu secretion nke ACTH. Dị ka e gosiri site ọtọ ọmụmụ eduziri atọhapụ hormone ọrịa (ọmụmụ na-gụnyere na sịntetik ibu hormone-atọhapụ hormone, esịnede 29, 40 44 amino acid residues), ọtụtụ n'ime ndị ọrịa nwere hypothalamic ọrịa, na underdevelopment nke ihu pituitary gland bụ ugbua abụọ mma. Primary daa ọrịa nke pituitary ozugbo eme adịkarịghị.
E nwere ọrịa na-enwe a lesion nke Central ụjọ usoro n'ihi enweghị oxygen, e.g., na otutu ime.

Ime mkpọchị anụ ahụ mmepe na-dwarfism ike ihe ndị dị ka ya erughị eru na-ezighị ezi bịara oriri na-edozi, ji a protein erughi, zinc na ndị ọzọ na mineral, oghom gburugburu ebe obibi ihe, ọnụnọ nke concomitant somatic ọrịa.

mgbaàmà

Backlog nke mmadụ ibu arụmọrụ ma anụ ahụ mmepe si nkezi ụkpụrụ - na na-egosipụta pituitary dwarfism. The foto n'okpuru egosi na mgbaàmà nke ọrịa.

Ọzọkwa, nwa ọhụrụ nwere a nkịtị ahu arọ na ogologo. Stunting na-achọpụtakarị na na 2-4 afọ nke ndụ: uto ọnụego nwa nwere ahụ ike nke afọ bụ 7-8 cm kwa afọ, ebe ụmụaka na pituitary dwarfism eto site na nkezi nke 2-3 cm Tupu iwebata mmiri ọgwụ ọgwụ. Dwarf atụle uto n'ihi na ndị inyom na-erughị 120 cm n'ihi na ndị ikom - na-erughị 130 cm.

The adakarị foto nke pituitary dwarfism

Emekarị nnọọ mfe ịmata ọnụnọ nke a onu maara dị ka pituitary dwarfism. Gịnị ka ọ bụ na otú ọ na-emetụta ndị mmadụ na-ahụ anya, anyị na-akọwa n'ụzọ zuru ezu n'okpuru.

Aru nke onye ọrịa nwere pituitary dwarfism e ji mara otosịrị iru nke ụmụ. E nwere a na-egbu oge na usoro iheomume nke osifikeshịọn nke ọkpụkpụ, nakwa dị ka ndị na-egbu oge na mgbanwe nke ezé, ogbenye mmepe nke mmiri nke nweekwa usoro na subcutaneous abụba, pallor, nkụ, na akpụkpọ wrinkling, mgbe ụfọdụ, e nwere ngafe abụba na obi, na apata na afo. Ọtụtụ mgbe, ọ dịghị nke abụọ hairiness.

Ephippium na 70-75% nke ọrịa na-agbanwe size (fọdụrụ - belata), ma ya ụdị kwekọrọ na ụmụ afọ na nwere n'ụdị a adade oval. N'ihe banyere pituitary etuto ephippium hụrụ ubara unwu ala nke mgbidi ya.

Loss nke gonadotropic pituitary ọrụ akpalite a na-egbu oge nke mmekọahụ development mgbe enweghị daa ọrịa. Dị ka a na-achị, ọ dịghị nke abụọ mmekọahụ mara. Nwoke ọrịa na-ebelata ke tụnyere afọ norm amụ na prostate, underdeveloped scrotum. Ọtụtụ n'ime ndị inyom na-ata ahụhụ site na ọrịa, adịghị noo nanso na nwere underdeveloped ara.

Intelligence ọtụtụ ndị ọrịa na-nọgidere na-enwe, wezụga ndị ọrịa na a nchoputa nke "pituitary dwarfism", pathogenesis nke bụ n'ihi na organic ụbụrụ mmebi akpụ ma ọ bụ na-akpasasị uche na okike.
E ji mara nke ọrịa bụ splanhnomikriya - Mbelata ke size nke esịtidem akụkụ, ọbara hypotension, muffled obi na-ada, Ugboro hypotension, bradycardia. Eleghị anya a ngosipụta nke abụọ hypothyroidism na hypocorticoidism.

Diagnosis nke pituitary dwarfism

Abụkarị nchoputa "dwarfism" okenye na-enweghị mgbagha. Anamnesis data na mgbagwoju laabu, adakarị na radiographic nnyocha bụ mmiri ọgwụ ndabere maka nchoputa "pituitary dwarfism", koodu MB 10 23.0.

Evaluation nke mmadụ ibu

Iji ịchọpụta, ọdịiche dị ma mata na a ọrịa dị ka pituitary dwarfism, mgbaàmà, na ọgwụgwọ na-ekpebi n'ụzọ ziri ezi, mere a mgbakọ na mwepụ nwale nke mmadụ ibu. N'ihi ya nwale, na mgbakwunye na nke zuru size nke ahụ, chọpụta ibu erughi na-anọchi anya ihe dị iche n'etiti uru nke ọrịa elu na nkezi ọnụego kwekọrọ ya afọ na mmekọahụ; ibu afọ dị ka abịa na ụkpụrụ nke ime uto nke ụfọdụ ụkpụrụ; uru nke normalized deviations:
N = P - PCP / δ,
ebe P - elu nke onye ọrịa ahụ;
Pcp - nkezi uru nke nkịtị ibu maka nnọchiteanya nke okike na afọ;
δ - ndiiche si PCP.
The lekwasịrị uru N <3 bụ ahụkarị ọrịa ndị dị ka pituitary dwarfism, dwarfism, N> 3 - maka gigantism. Nke a na egosi nwere ike ejikwara, na-amata na Ọnọdụ nke development.

Diagnosis nke pituitary dwarfism na ụmụ

Pituitary dwarfism na ụmụaka na-achọ a ọzọ enyocha ihe nchoputa nke ọrịa, dị ka na-abụkarị na-adakarị picture bụ edoghị. A nchoputa e nyere a oge nke ọnwa isii iji a afọ. N'oge a na nwa-eledo, ọ na-kenyere ume ọhụrụ ọgwụgwọ enweghị hormone ọgwụ, oriri na-edozi na-aba ụba anụmanụ protein, akwụkwọ nri na mkpụrụ osisi, calcium, site, na vitamin A na D. anọghị n'okpuru ọnọdụ ndị a zuru ezu ehihie na okwu nke na-eto ma na-ahụ mmepe bụ ihe ndabere maka ndị dị otú ahụ ọmụmụ, dị ka:

• ọbara ibu hormone;
• cranial radiography ịchọpụta daa ọrịa na cranium;
• X-ụzarị nke obi, ọkpụkpụ nke aka na wrists, nke na-ekpughe ọkpụkpụ afọ nke nwa;
• MRI;
• agbakọrọ tomography.

Dabere na ndị ọmụmụ nwere ike ikpebi na-akpata ọrịa na-amalite hormone ọgwụ.

therapies

Mgbe Ịchọpụta Otú Nsogbu ọrịa ndị dị ka pituitary dwarfism, dabere na omume ndinọ na mmadụ ibu hormone n'ụdị intermittent ọgwụgwọ ọmụmụ. The dose of ibu hormone ndinọ na-adabere na isi iyi nke ya ọdịnaya na ọbara, nakwa dị ka onye ọrịa. N'ihi na mma absorption nke hormone oriri isi dose na-advantageously rụrụ ke usenubọk awa, nke kwekọrọ na n'ahụ ji mara mmiri ọgwụ secretion. Mmetuta nke mmekọahụ development a rụrụ mgbe usoro nke mmechi nke ibu zones na-enyemaka nke mmekọahụ homonụ.

Ihe kacha mkpa ọtùtù nke ọgwụgwọ na ọgwụgwọ nke dwarfism na-anata ọrịa anabolic Steroid, ọgwụ ọjọọ site, zinc, calcium, na vitamin okụre biostimulants, ezigbo oriri na-edozi.

Iji malite usoro ọgwụgwọ ọ bụ ihe amamihe dwarfism na 5-7 afọ.

Nnọchi ọgwụ dịruru ọtụtụ afọ, Bilie elekọta ya mgbe nile nke irè nke ọgwụ ọjọọ e site ndidi ma ha nnọchi na rụọ ọrụ nke ọma, na-agwụ na mkpochapu na-akpata ọrịa ahụ. Ọ bụrụ na ị na-apụghị idozi nsogbu ahụ onye ọrịa na-kenyere nnọchi ọgwụ maka ndụ. Na a n'ụzọ kwesịrị ekwesị sized ọgwụgwọ enweta a uto ọnụego nke 8-10 cm kwa afọ, na ọrịa anaghị si dị iche na ahụ ike ụmụ.

Ọ bụ amanyere bụ iwu na-edebe ndụ nke ọrịa site na otu endocrinologist.

tikọta ọgwụgwọ

N'ihi na nke specificity nke mmadụ ibu hormone ifịk ke ọgwụgwọ nke dwarfism bụ naanị mmadụ ibu hormone mepụtara, ahụ dị ka nke ọma dị ka primates. Ọtụtụ-eji somatotropin emepụta site nje njikọ site nkà mgbanwe mkpụrụ ndụ iji Escherichia coli. E nwere a somatotropin, a sịntetik chemical pụtara, ma ọ na-fọrọ nke nta mgbe eji n'ihi na nke elu na-eri.

Ahụmahụ na-egosi na ụkpụrụ nke kacha nta irè dose nke mbụ ọgwụgwọ oge ederịta 0.03-0.06 mg kwa kilogram ahu aro. Oke arụmọrụ na-ewere 2-4 mg 3 ugboro a izu. Site na-amụba otu dose 10 mg ezigbo abawanye ibu mmetụta e hụrụ, ma gosiri ngwa ngwa guzobere-alụso ọrịa ọgụ na somatotropin. Otu ihe dị mkpa uru nke ọgwụgwọ nke ibu hormone nweghị osooso nke osifikeshịọn nke ọkpụkpụ na ya ndabere.

The ojiji nke anabolic Steroid

Iji otu n'ime ihe ndị kasị mkpa ngwaọrụ na ọgwụgwọ nke dwarfism akọ ndidi ojiji nke anabolic Steroid. Ha edinam dabeere mmetuta nke ibu site amụba protein njikọ ma na-amụba otú larịị nke endogenous ibu hormone. Nke a na ọgwụgwọ a rụrụ ọtụtụ afọ, obere ifịk ọgwụ ọjọọ na-eji nwayọọ nwayọọ dochie anya ọzọ nọ n'ọrụ ogige. The egosi na-agbanwe agbanwe anabolic ọgwụ ọjọọ bụ Mbelata 2-3 afọ uto mmetụta, nke, n'aka, nwere mmetụta nke n'ihu ime uto. Pituitary dwarfism agụnye ọgwụgwọ anabolic Steroid dị ka ọgwụgwọ N'ezie na oge ezumike, hà ọkara oge nke ọgwụgwọ. N'ihe gosiri Habituation na aba oge izu (ruo 4-6 ọnwa). Otu shot ndibọbọ agụnye naanị otu ụdị anabolic Steroid, na ijikọta multiple ọgwụ ọjọọ impractical n'ihi welie ha edinam adịghị ime. Dosing nke anabolic Steroid amalite na kacha nta irè na ha nwayọọ nwayọọ na-abawanye.

Ọgwụgwọ nke mmekọahụ obere nwa nke ụmụ nwoke

Ọgwụgwọ nke mmekọahụ obere nwa nke ụmụ nwoke chọpụtara na dwarfism na-rụrụ iji mmadụ chorionic gonadotropin. The eji nke a ọgwụ adịghị amalite tupu afọ 15, mgbe ọbụna mgbe e mesịrị na ndụ. Mmetụta nke ọgwụ na-iji na-akpali akpali nke Leydig mkpụrụ ndụ, ekele ya androgenic anabolic ọrụ accelerates na-enwe mmekọahụ na mmepe, na ibu. The onunu ogwu Usoro nke ọgwụ dose bụ 1000 - 1500 IU, intramuscularly ndinọ 1-2 ugboro n'izu, abụọ ma ọ bụ atọ ọmụmụ kwa afọ. Ọ bụrụ na ụmụ nwoke 16 afọ hụrụ a ele mmadụ anya n'ihu utịp ke ọgwụgwọ nke mmadụ chorionic gonadotropin, mepụtara nwetụ na obere doses nke androgens ( "Methyltestosterone" Sublingual 5-10 mg kwa ụbọchị ahụ). Ndinọ ogbo nke ọgwụgwọ nke nwoke ọrịa a na enweta na ogologo-eme androgen - ọgwụ ọjọọ "testenat", "Omnadren-250", "Sustanon-250."

Ọgwụgwọ nke mmekọahụ obere nwa na ụmụ agbọghọ

ụmụ agbọghọ, ọgwụgwọ nwere ike na-amalite na 16, simulating a ala dose of estrogen nkịtị ịhụ nsọ okirikiri. Ọ rụrụ n'ime izu atọ nke ọ bụla n'ọnwa. The abụọ na-adọ nke ịhụ nsọ okirikiri si atọ izu i nwere ike na-ekenye chorionic gonadotrophin onunu ogwu 1000-1500 IU na-enweta oge 3-5 ugboro n'izu. The ọzọ bụ ọgwụ ọjọọ progestin edinam ( "Progesterone," "Pregnin").

Dechara omume (na ezu eziokwu nke mmechi nke ibu zones) anata iche mgbe nile mmekọahụ homonụ na ọgwụgwọ doses. Nke a bụ mkpa iji mmekọahụ akụkụ na sekọndrị mmekọahụ ji mara ruru ntozu, iji hụ kwesịrị ekwesị larịị nke libido na potency. Female ọrịa na-kenyere jikọtara estrogen-progestin preparations, dị ka "Non-ovlon", "Infekundin", "Bisekurin", "Rigevidon".

ele ihe anya

Amụma ndụ na pituitary dwarfism na-adabere na ya udi. Ọ bụrụ na ị na-onwe-eche ihu dị otú ahụ a nsogbu, dị ka mkpụrụ ndụ ihe nketa pituitary dwarfism, ọ na-amụtawo n'onwe, na ọ ga-amasị ka obi sie gị - nke a, ndị prognosis dị mma. Na ọnụnọ nke pituitary etuto ahụ na ọnya nke CNS, mmerụ na na. E., The prognosis na-adabere na Ọnọdụ nke isi-enweghị nchịkwa na usoro.

N'ihi oge a ụzọ nke ọgwụgwọ nke anụ ahụ ike na-arụ ọrụ ike nke ndị mmadụ na-ata ahụhụ site na dwarfism, mụbara budata site amụba oge nke ndụ ha. N'oge nọ n'ọrụ ọgwụgwọ oge, bụchaghị rụrụ a Ịhazi nnyocha nke ndị ọrịa ọ bụla 2-3 ọnwa, mmezi ọgwụ - ule ọ bụla 6-12 ọnwa.

The isi ihe na-akpata ọrụ na -elekọta mmadụ mmegharị nke ọrịa na dwarfism bụ ha anụ ahụ na nke ndị nwere ọgụgụ isi ike. Ọ bụ ihe amamihe na-ahọrọ a ọrụ, ewepu mmega.