Pituitary dwarfism (nanism) yog ib yam kab mob uas tshwm sim nws tus kheej hauv kev loj hlob qeeb thiab kev loj hlob ntawm lub cev vim yog kev ua txhaum ntawm kev tso tawm ntawm lub anterior pituitary somatotropin - kev loj hlob hormone. Nws yog cov tshuaj no uas yog lub luag haujlwm rau cov txheej txheem ntawm kev faib cell hauv tib neeg lub cev.
Types pituitary dwarfism
Raws li etiological yam, pituitary dwarfism (ICB code 23.0) tshwm sim:
- Pib, kev loj hlob uas tshwm sim los ntawm cov kab mob ntawm lub caj pas pituitary thiab, vim li ntawd, qhov txo qis ntawm cov tshuaj hormones somatotropic.
- Hypothalamic, kev loj hlob ntawm uas yog vim qhov txo qis hauv qib tso tawm cov tshuaj hormones ntawm hypothalamus, tsim los cuam tshuam kev ua haujlwm ntawm lub qog pituitary.
- Nrog rau cov ntaub so ntswg tiv thaiv kev loj hlob hormone. Nws txoj kev loj hlob yog txuam nrog qhov tsis muaj peev xwm ntawm receptors hauv cov ntaub so ntswg los teb kom raug rau qhov kev txiav txim ntawm cov tshuaj hormones.
Etiopathogenesis
Yog vim li cas rau kev loj hlob ntawm pituitary dwarfism tuaj yeem yog qhov swb ntawm pituitary caj pas nws tus kheej: cov txheej txheem qog (meningiomas, craniopharyngiomas, chromophobic adenomas), kev raug mob,tshuaj lom, kis kab mob rau thaj tsam interstitial-pituitary lossis dysregulation ntawm kev ua haujlwm ntawm pituitary caj pas los ntawm hypothalamus. Txawm li cas los xij, feem ntau hom kab mob yog hom caj ces.
Qhov kev nthuav dav tshaj plaws yog panhypopituitary dwarfism, uas zoo li yuav tau txais txiaj ntsig los ntawm hom recessive. Muaj kev xav tias muaj ob hom kev sib kis ntawm cov kab mob no - autosomal thiab dhau ntawm X chromosome. Nrog rau kev tsis sib haum xeeb ntawm cov tshuaj hormones somatotropic, nrog rau daim ntawv ntawm dwarfism, ib qho kev tsis sib haum xeeb ntawm cov thyroid-stimulating hormone thiab gonadotropins feem ntau pom.
Tsawg dua thiab tsawg dua, ACTH zais cia raug cuam tshuam. Raws li kev tshawb fawb ua haujlwm nrog cov neeg mob uas tso cov tshuaj hormones (kev tshawb fawb kuj suav nrog cov tshuaj somatotropin-tso cov tshuaj hormones uas muaj 29, 40 thiab 44 amino acid residues) qhia, feem ntau ntawm cov neeg mob no muaj pathologies ntawm lub hypothalamus, thiab underdevelopment ntawm lub anterior pituitary caj pas twb yog lawm. kos npe thib ob. Lub hauv paus pathology ntawm lub caj pas pituitary nws tus kheej tsis tshua muaj.
Muaj cov neeg mob uas muaj kev puas tsuaj rau lub hauv nruab nrab lub paj hlwb vim tsis muaj oxygen, piv txwv li, ntau lub cev xeeb tub. Cov yam xws li qis dua thiab cov khoom noj khoom haus tsis sib npaug uas pom los ntawm qhov tsis txaus ntawm cov protein, zinc thiab lwm yam kab mob, qhov tsis zoo ntawm ib puag ncig, muaj cov kab mob sib xyaw ua ke.
Symptoms of disease
Lagging qab qhov ntsuas ntawm ib tug neeg txoj kev loj hlob thiab nws lub cev kev loj hlob los ntawm qhov nruab nrab statistical qauv - qhov no yog dab tsi pituitary nanism manifests nws tus kheej nyob rau hauv. Daim duab hauv qab no qhia meej txog cov tsos mob ntawm tus kab mob.
Ntxiv mus, tus me nyuam mos muaj qhov hnyav thiab lub cev ntev. Stunting, raws li txoj cai, tsis tshwm sim kom txog rau thaum muaj hnub nyoog 2-4 xyoos: qhov kev loj hlob ntawm tus me nyuam noj qab nyob zoo ntawm lub hnub nyoog no yog 7-8 cm nyob rau hauv ib xyoos, thaum cov me nyuam pituitary dwarfism loj hlob los ntawm qhov nruab nrab ntawm 2-3 cm. Ua ntej kev qhia txog kev kho tshuaj hormone qhov siab rau cov poj niam tau txiav txim siab tsawg dua 120 cm, rau cov txiv neej - tsawg dua 130 cm.
daim duab kho mob ntawm pituitary dwarfism
Nws feem ntau yog ib qho yooj yim heev los txiav txim seb muaj qhov tshwm sim li pituitary dwarfism. Nws yog dab tsi thiab nws cuam tshuam li cas rau tus neeg lub ntsej muag, peb yuav qhia hauv qab no.
Lub cev ntawm tus neeg mob pituitary dwarfism muaj cov yam ntxwv ntawm cov menyuam yaus. Kev ncua sij hawm ntawm ossification ntawm pob txha pob txha, nrog rau kev ncua sij hawm ntawm kev hloov ntawm cov hniav, kev loj hlob tsis zoo ntawm cov leeg nqaij thiab cov ntaub so ntswg subcutaneous adipose, pallor, dryness thiab wrinkling ntawm daim tawv nqaij, qee zaum muaj. cov rog ntau dhau ntawm lub hauv siab, lub duav thiab plab. Feem ntau tsis muaj plaub hau kev loj hlob theem nrab.
Lub eeb Turkish hauv 70-75% ntawm cov neeg mob tsis hloov qhov loj me (hauv qhov so nws raug txo), tab sis nws cov duab sib raug rau thaum yau thiab zoo li lub oval sawv. Nyob rau hauv cov ntaub ntawv ntawm cov qog pituitary, lub sella turcica yog loj thiab kev puas tsuaj ntawm nws cov phab ntsa yog pom.
Prolapse ntawm gonadotropicKev ua haujlwm ntawm lub caj pas pituitary provokes ncua kev loj hlob ntawm kev sib deev, feem ntau tsis muaj pathology. Raws li txoj cai, cov yam ntxwv ntawm kev sib deev theem nrab tsis tuaj. Cov txiv neej cov neeg mob muaj qhov noov thiab qog txo qis nyob rau hauv kev sib piv nrog rau cov hnub nyoog qauv, ib tug underdeveloped scrotum. Cov poj niam uas cuam tshuam feem ntau tsis coj khaub ncaws thiab muaj lub mis tsis zoo.
Kev txawj ntse ntawm cov neeg mob feem ntau yog khaws cia, qhov tshwj xeeb yog cov neeg mob kuaj pom muaj pituitary dwarfism, cov kab mob uas tshwm sim los ntawm cov kab mob organic ntawm lub paj hlwb ntawm cov qog los yog kev puas tsuaj. Splanchnomycria yog yam ntxwv. ntawm tus kab mob - ib tug txo nyob rau hauv lub cev nyob rau hauv loj, arterial hypotension, muffled lub plawv tones, hypotension, bradycardia yog nquag. Muaj peev xwm tshwm sim ntawm theem nrab hypothyroidism thiab hypocorticism.
Kev kuaj mob ntawm pituitary dwarfism
Feem ntau, kev kuaj mob ntawm "nanism" hauv cov neeg laus yog qhov tsis ntseeg. Cov ntaub ntawv ntawm anamnesis, nrog rau kev kuaj xyuas dav dav, kev kuaj mob, kuaj hluav taws xob thiab tshuaj hormonal yog lub hauv paus rau kev kuaj mob ntawm "hypophyseal dwarfism", code MKB 10 23.0.
Kev ntsuas qhov siab tib neeg
Txhawm rau kuaj xyuas, sib txawv thiab paub txog tus kab mob xws li pituitary dwarfism, cov tsos mob, thiab kev kho mob kom txiav txim siab kom raug, kev ntsuas lej ntawm tib neeg kev loj hlob yog tsim. Txhawm rau ntsuas nws, ntxiv rau qhov loj me ntawm lub cev, qhov kev loj hlob tsis txaus yog txiav txim siab, uas yog qhov sib txawv ntawm tus nqi ntawm tus neeg mob qhov siab thiab qhov nruab nrab tus qauv sib xws.nws poj niam txiv neej thiab hnub nyoog; lub hnub nyoog loj hlob raws li kev sau ntawv ntawm tus nqi ntawm tus neeg mob qhov siab rau qee cov qauv; tus nqi ntawm qhov kev sib txawv ntawm qhov qub:
N=Р - Рср / δ, qhov twg Р yog tus neeg mob qhov siab;
Рср - tus nqi nruab nrab ntawm kev loj hlob ib txwm rau tus neeg sawv cev ntawm ib qho muab poj niam txiv neej thiab hnub nyoog;
δ - square deviation from Рavg. Xav tau tus nqi N 3 - rau gigantism. Qhov ntsuas no kuj tseem siv tau los ntsuas qhov kev loj hlob ntawm kev loj hlob.
Kev kuaj mob pituitary dwarfism hauv menyuam yaus
Pituitary dwarfism nyob rau hauv cov menyuam yaus yuav tsum tau ua tib zoo mus rau kev kuaj mob, vim tias daim duab kho mob feem ntau tsis meej. Ib lub sij hawm ntawm rau lub hlis mus rau ib xyoos yog muab rau kev kuaj mob. Nyob rau lub sijhawm no, tus menyuam raug saib xyuas, nws raug sau tseg kev kho kom muaj zog ntxiv yam tsis muaj kev kho tshuaj hormone, kev noj zaub mov zoo uas muaj protein ntau, zaub thiab txiv hmab txiv ntoo, calcium, phosphorus, thiab vitamins A thiab D. Nyob rau hauv cov xwm txheej no, muaj. tsis muaj kev hloov pauv txaus hauv kev loj hlob thiab kev loj hlob ntawm lub cev yog lub hauv paus rau kev tshawb fawb xws li:
- ntshav qib ntawm kev loj hlob hormone;
- X-ray ntawm pob txha taub hau txhawm rau kuaj pom kab mob hauv pob txha taub hau;
- x-ray kuaj ntawm lub hauv siab, pob txha ntawm tes thiab dab teg, uas tso cai rau koj los txiav txim siab lub hnub nyoog ntawm tus menyuam;
- MRI;
- CT scan.
Raws li cov kev tshawb fawb no, nws tuaj yeem txiav txim siab qhov ua rau tus kab mob thiab pib kho tshuaj hormone.
txoj kev kho
Thaum kuaj pom tus kab mob xws li pituitary dwarfism, kev kho mob yog ua raws li kev qhia ntawm somatotropin rau hauv tib neeg lub cev nyob rau hauv daim ntawv ntawm kev kho mob tsis tu ncua. Qhov koob tshuaj ntawm kev txhaj tshuaj loj hlob yog nyob ntawm nws cov ntsiab lus thawj zaug hauv cov ntshav, nrog rau cov yam ntxwv ntawm tus neeg mob. Txhawm rau kom zoo dua qhov nqus ntawm cov tshuaj hormones, nws raug nquahu kom noj cov tshuaj tseem ceeb thaum sawv ntxov, uas sib haum rau cov yam ntxwv ntawm lub cev hormonal secretion. Kev txhawb kev loj hlob ntawm kev sib deev yog ua tiav tom qab cov txheej txheem kaw qhov chaw loj hlob nrog kev pab los ntawm kev sib deev hormones.
Qhov kev ntsuas tseem ceeb tshaj plaws hauv kev kho mob dwarfism kuj yog kev noj tshuaj anabolic steroids, phosphorus, zinc, calcium npaj, biostimulants thiab vitamin complexes, khoom noj khoom haus txaus los ntawm cov neeg mob.
Yuav tsum pib kho mob dwarfism thaum muaj hnub nyoog 5-7.
Kev hloov kho yuav kav ntev li ob peb xyoos, nrog rau kev saib xyuas tas li ntawm kev ua tau zoo ntawm cov tshuaj noj los ntawm tus neeg mob thiab lawv cov kev hloov pauv nrog cov txiaj ntsig zoo dua, thiab xaus thaum qhov ua rau tus kab mob raug tshem tawm. Yog tias nws tsis tuaj yeem tshem tawm nws, tus neeg mob tau muab tshuaj hloov kho rau lub neej. Nrog txoj kev kho kom raug, kev loj hlob ntawm 8-10 cm ib xyoos yog tiav, thiab tus neeg mob tsis txawv ntawm cov menyuam noj qab haus huv.
Kev ua raws li lub neej ntawm cov neeg mob nrog tus kws kho mob endocrinologist yog qhov yuav tsum tau ua.
Kho nrog kev loj hlob hormone
Vim qhov tshwj xeeb ntawm tib neeg kev loj hlob hormone, tib neeg somatotropin tsuas yog ua haujlwm hauv kev kho mob dwarfism, thiabkuj tsim los ntawm primates. Somatotropin, tau los ntawm cov kab mob sib txuas, los ntawm kev siv tshuaj genetic engineering siv Escherichia coli, yog siv dav. Muaj cov tshuaj ua ke somatotropin, tab sis nws siv tsis tau vim nws tus nqi siab.
Kev paub qhia tau hais tias qhov tseem ceeb ntawm qhov tsawg kawg nkaus koob tshuaj zoo rau thawj lub sijhawm ntawm kev kho mob sib raug rau 0.03-0.06 mg ib kilogram ntawm lub cev hnyav. Qhov zoo tshaj plaws yog 2-4 mg noj 3 zaug hauv ib lub lis piam. Nrog rau kev nce ntxiv hauv ib koob tshuaj rau 10 mg, tsis pom qhov nce ntxiv ntawm kev loj hlob zoo, tab sis kev tsim cov tshuaj tiv thaiv sai rau somatotropin tau sau tseg. Ib qho txiaj ntsig tseem ceeb ntawm kev kho somatotropin yog qhov tsis muaj qhov nrawm ntawm pob txha ossification tawm tsam nws keeb kwm yav dhau.
Siv anabolic steroids
Ib qho tseem ceeb tshaj plaws hauv kev kho mob dwarfism yog kev siv cov tshuaj anabolic steroids los ntawm tus neeg mob. Lawv qhov kev ua yog ua raws li kev txhawb nqa ntawm kev loj hlob los ntawm kev txhim kho cov protein synthesis thiab yog li nce qib ntawm endogenous somatotropic hormone hauv lub cev. Cov kev kho mob zoo li no tau ua tiav rau ntau xyoo, cov tshuaj tsis tshua muaj zog yog maj mam hloov los ntawm cov tshuaj nquag. Ib qho kev qhia txog kev hloov tshuaj anabolic yog qhov txo qis hauv kev loj hlob tom qab 2-3 xyoos, uas, nyob rau hauv lem, tsim cov nyhuv ntawm kev loj hlob ntxiv. Pituitary dwarfism cuam tshuam nrog kev kho mob nrog anabolic steroids nyob rau hauv daim ntawv ntawm cov kev kho mob nrog rau lub sij hawm so sib npaug rau ib nrab ntawm lub sij hawm ntawm kev kho mob. Thaum muaj kev quav yeeb quav tshuajntev so (txog 4-6 lub hlis) kuj qhia. Kev noj ib zaug suav nrog tsuas yog ib hom tshuaj anabolic steroids, kev sib xyaw ua ke ntawm ntau yam tshuaj tsis zoo, vim tias lawv cov nyhuv tsis nce. Kev noj cov tshuaj anabolic steroids pib nrog qhov tsawg kawg nkaus thiab maj mam nce lawv.
Kev kho mob ntawm cov menyuam yaus hnub nyoog qis dua
Kev kho mob ntawm kev sib deev immaturity nyob rau hauv cov tub hluas uas kuaj pom muaj dwarfism yog ua nrog tib neeg chorionic gonadotropin. Kev siv cov tshuaj no pib tsis pub dhau 15 xyoo, thiab feem ntau thaum muaj hnub nyoog tom qab. Qhov kev txiav txim ntawm cov tshuaj yog tsom rau kev txhawb nqa Leydig hlwb, uas, vim yog cov haujlwm anabolic ntawm nws cov androgens, accelerates ob qho tib si kev sib deev thiab kev loj hlob. Cov txheej txheem ntawm cov tshuaj yog ib koob ntawm 1000 - 1500 IU, muab tshuaj intramuscularly 1-2 zaug hauv ib lub lis piam, ob lossis peb chav kawm ib xyoos. Yog tias qhov ua tsis tiav ntawm kev kho mob nrog chorionic gonadotropin pom nyob rau hauv cov tub hluas hnub nyoog 16 xyoo, ib qho kev sib hloov yog ua nrog me me ntawm androgens ("Methyltestosterone" sublingually ntawm 5-10 mg ib hnub twg). Cov theem kawg ntawm kev kho cov txiv neej cov neeg mob yog kev noj cov tshuaj androgens ntev - kev npaj "Testenat", "Omnadren-250", "Sustanon-250".
Kev kho mob ntawm cov menyuam ntxhais hluas
Kev kho cov ntxhais tuaj yeem pib txij li hnub nyoog 16 xyoo, simulating ib txwm coj khaub ncaws nrog cov tshuaj estrogens tsawg. Nws tau tuav peb lub lis piam txhua lub hlis. Nyob rau theem thib ob ntawm kev coj khaub ncaws los ntawm lub lim tiam thib peb, nws muaj peev xwm muab tshuaj rau chorionicgonadotropin nrog ntau npaum li cas ntawm 1000-1500 IU nrog lub sijhawm ntawm 3-5 zaug hauv ib lub lis piam. Lwm txoj hauv kev rau nws yog cov tshuaj progestogen ("Progesterone", "Pregnin")
Kev kho mob xaus (nrog rau kev ua tiav ntawm kev kaw ntawm thaj chaw loj hlob) los ntawm kev noj cov tshuaj hormones sib deev sib txuas hauv cov tshuaj kho mob. Qhov no yog qhov tsim nyog txhawm rau txhawm rau qhov chaw mos thiab cov yam ntxwv sib deev theem nrab kom ncav cuag txoj kev loj hlob, kom ntseeg tau tias qib libido thiab potency. Cov poj niam cov neeg mob tau muab tshuaj estrogen-gestagenic ua ke, xws li Non-ovlon, Infekundin, Bisekurin, Rigevidon.
Huab cua
Kev ntsuas rau lub neej hauv pituitary dwarfism nyob ntawm nws daim ntawv. Yog tias koj tus kheej ntsib teeb meem xws li caj ces pituitary dwarfism, nws yog dab tsi koj tau kawm ua ntej, peb maj nrawm kom paub tseeb tias qhov no qhov kev tshwm sim yog qhov zoo. Nyob rau hauv lub xub ntiag ntawm cov qog pituitary thiab qhov txhab ntawm lub hauv paus paj hlwb, kev raug mob, thiab lwm yam, qhov kev tshwm sim nyob ntawm qhov kev loj hlob ntawm cov txheej txheem tseem ceeb ntawm cov txheej txheem pathological.
Ua tsaug rau cov txheej txheem niaj hnub ntawm kev kho mob, lub cev muaj peev xwm thiab kev ua haujlwm muaj peev xwm ntawm cov neeg raug mob dwarfism tau nce ntau, lawv lub neej ntev tau ntev. Thaum lub sijhawm ua haujlwm ntawm kev kho mob, kev kuaj xyuas cov neeg mob yuav tsum tau ua txhua 2-3 lub hlis, kev kho mob - kev kuaj txhua 6-12 lub hlis.
Qhov tseem ceeb hauv kev ua haujlwm thiab kev sib raug zoo ntawm cov neeg mob dwarfism yog lawvlub cev thiab kev txawj ntse muaj peev xwm. Nws raug nquahu kom xaiv cov haujlwm uas tsis suav nrog kev ua lub cev.
