Adrenogenital syndrome - tag nrho cov nta ntawm pathology

Rau cov thawj thiab theem ob hom kev sib deev, cov tshuaj hormones yog lub luag haujlwm, qee yam uas tau ua nyob rau hauv cov qog ntawm qog. Muaj tus kab mob yug hauv lub cev los ntawm qhov kawg ntawm qog qhov qog ntawm qhov cov qog thiab ntau qhov kev tso tawm ntawm androgens. Tshaj ntawm txiv neej cov tshuaj hormones hauv lub cev ua rau cov kev hloov loj hauv cov qauv ntawm lub cev.

Adrenogenital Syndrome - Ua rau

Cov kab mob hauv kev xav tau tshwm sim los ntawm qhov kev hloov noob caj noob ces. Nws tsis tshua pom tau tias, qhov tshwm sim ntawm adrenogenital syndrome yog 1 case per 5000-6500. Cov kev hloov hauv cov cai caj dab provokes kev nce rau hauv qhov loj me thiab deterioration ntawm adrenal cortex. Qhov tshwj xeeb ntawm cov enzymes tshwj xeeb uas koom hauv kev tsim cov cortisol thiab aldosterone txo. Lawv tsis muaj peev xwm ua rau kom muaj kev nce rau ntawm cov txiv neej cov tshuaj hormones.

Adrenogenital syndrome - kev faib tawm

Nyob ntawm seb qhov kev loj hlob ntawm adrenocortical thiab qhov hnyav ntawm cov tsos mob, tus kab mob piav qhia muaj nyob rau hauv ntau yam kev hloov. Cov ntaub ntawv ntawm adrenogenital syndrome:

• ntsev-poob;
• viril (yooj yim);
• tom qab-pubertal (nonclassical, atypical).

Adrenogenital syndrome - ntsev daim ntawv

Cov hom kab mob kheev muaj ntau tshaj plaws, uas tau kuaj pom hauv cov me nyuam mos los yog cov me nyuam ntawm thawj xyoo ntawm lub neej. Nrog lub ntsev-poob daim ntawv ntawm adrenogenital syndrome, qhov hormonal tshuav yog ntxhov siab thiab cov kev ua ntawm adrenal cortex yog tsis txaus. Hom kab mob no yog nrog los ntawm kev tsis sib haum xeeb ntawm aldosterone. Nws yog ib qho tsim nyog los tswj xyuas dej-ntsev seem hauv lub cev. Qhov hu ua adrenogenital syndrome ua rau muaj kev ua txhaum kev mob plawv thiab dhia hauv ntshav siab. Qhov no tshwm sim tawm tsam tom qab ntawm tsub zuj zuj ntawm salts nyob rau hauv ob lub raum.

Adrenogenital syndrome yog ib daim kab mob viril

Ib qho yoojyim los sis txawv ntawm hom kev kawm ntawm pathology yog tsis nrog tshwm sim ntawm kev tsis muaj qab hau adrenal. Cov kab mob hu ua adrenogenital syndrome (ACS viril daim ntawv) ua rau kev hloov pauv hauv lwm qhov chaw mos. Hom kab mob no kuj tau kuaj pom thaum ntxov los yog tom qab yug me nyuam tas. Sab hauv kev tsim kho me nyuam nyob tsis zoo.

Postpubertate daim ntawv ntawm adrenogenital syndrome

Hom kab mob no kuj hu ua atypical, tau txais thiab tsis yog-classical. Xws li tus mob adrenogenital tshwm sim hauv cov poj niam uas muaj kev sib deev hauv lub neej. Qhov ua rau ntawm kev loj hlob ntawm pathology yuav ua tau ob qho tib si yog ib qho kev sib hloov ntawm cov noob, thiab ib lub qog ntawm adrenal cortex . Tus kab mob no feem ntau yog nrog cov infertility, thiaj li tsis muaj kev kho kom haum, lub plawv adrenogenital thiab cev xeeb tub yog cov ntsiab lus tsis sib xws. Txawm tias muaj kev vam meej, qhov kev pheej hmoo ntawm qaub ncaug yog siab, tus fetus raug tua txawm nyob rau theem ntxov (7-10 lub lis piam).

Adrenogenital syndrome - cov tsos mob

Daim duab saib ntawm qhov piav qhia txog genetic anomaly sib raug rau lub hnub nyoog thiab daim ntawv ntawm tus kab mob. Adrenogenital syndrome nyob rau hauv cov me nyuam mos muaj qee zaum kuj tsis raug txiav txim siab, vim tias qhov kev sib deev ntawm tus me nyuam tuaj yeem txheeb xyuas tau qhov tsis raug. Cov cim tshwj xeeb ntawm pathology tshwm los ntawm 2-4 xyoo, nyob rau hauv qee zaus nws tshwm sim tom qab, nyob rau hauv kev loj hlob los yog kom loj hlob.

Adrenogenital syndrome hauv cov tub

Nrog ntsev-poob daim ntawv ntawm tus kab mob, cov tsos mob ntawm dej-ntsev kom sib npaug kev cuam tshuam yog pom:

• zawv plab;
• ntuav heev;
• ntshav siab;
• kev sib ceg;
• tachycardia;
• poob phaus.

Ib qho yooj yim adrenogenital syndrome hauv cov txiv neej muaj cov tsos mob nram qab no:

• tsam noov;
• ntau daim tawv pigmentation ntawm scrotum;
• tsaus epidermis ncig lub qhov quav.

Cov me nyuam mos liab tshiab tsis tshua pom tau vim hais tias qhov kev kuaj mob ntawm lub hnub nyoog ntxov tsis zoo. Tom qab (ntawm 2 xyoos) Adrenogenital Syndrome no tshwm sim ntau dua:

• kev loj hlob ntawm cov plaub hau nyob rau hauv lub cev, nrog rau cov qoob loo;
• tsawg, lub suab gruff;
• hormonal pob txuv ( acne vulgaris);
• masculinization;
• acceleration ntawm pob txha tsim;
• luv luv stature.

Adrenogenital syndrome nyob rau hauv cov menyuam ntxhais

Txhawm rau txhais tau tias tus mob uas muaj tus kabmob ntawm cov pojniam yog yooj yim, nws muaj cov kev mob tshwm sim xws li:

• hypertrophic clitoris, uas zoo nkaus li zoo li tus qau;
• loj ua ke, zoo li scrotum;
• lub qhov ncauj thiab qhov zis yog tso ua ke nrog cov kab mob urogenital urogenital.

Tawm tsam cov keeb kwm ntawm cov me nyuam mos, cov me nyuam ntxhais qee zaum yuam kev rau cov tub thiab coj raws li txoj kev sib deev. Vim li no, nyob rau tsev kawm ntawv lossis thaum tiav hluas, cov menyuam no feem ntau muaj teeb meem ntawm lub hlwb. Hauv kev tsim kho me nyuam ntawm tus ntxhais kiag li sib raug rau cov poj niam genotype, yog vim li cas nws xav tias nws tus poj niam. Tus me nyuam pib ua kev tsis sib haum xeeb thiab muaj teeb meem nrog kev sib haum xeeb hauv zej zog.

Tom qab 2 xyoos, muaj tus kab mob hu ua adrenogenital syndrome yog cov tsos mob li nram qab no:

• kev loj hlob ntxov ntxov ntawm cov nqaij mos thiab quav;
• luv taw thiab caj npab;
• mob nqaij;
• pom cov plaub hau ntawm lub ntsej muag (thaum muaj hnub nyoog 8 xyoo);
• ib tug txiv neej lub cev (sab xub pwg, nqaim plab);
• tsis muaj kev loj hlob ntawm cov qog nqaij hlav hauv siab;
• tsawg kev loj hlob thiab loj heev;
• tsis paub lub suab;
• kab mob pob txuv;
• qhov pib qeeb ib ce (tsis pub dhau 15-16 xyoos);
• tsis hloov lub caij nyoog, nquag sij qeeb thaum ua pojniam;
• amenorrhea lossis oligomenorrhea;
• infertility;
• ntshav siab jumps;
• ntau pigmentation ntawm cov epidermis.

Adrenogenital syndrome - mob

Cov kev ntsuas thiab cov kev kuaj mob pab ua kom pom cov hyperplasia thiab cov teeb meem ntawm cov qoob loo ntawm adrenal cortex. Rau kev kuaj pom cov kab mob hauv lub cev adrenorital congenital syndrome hauv cov me nyuam mos, ib qho kev soj ntsuam ntawm cov qoob loo thiab cov tom tsev kawm tiav (los yog ultrasound). Kev kuaj mob kho mob yuav pom tau cov qe menyuam thiab lub tsev me nyuam hauv cov ntxhais nrog txiv neej qhov chaw mos.

Yuav kom paub tseeb tias qhov kev tshawb nrhiav liam, kuaj kev kuaj mob rau cov kab mob adrenogenital syndrome. Nws suav nrog txoj kev tshawb ntawm cov zis thiab ntshav ntawm cov ntsiab lus ntawm cov tshuaj hormones:

• 17-OH-progesterone;
• aldosterone;
• cortisol;
• 17-ketosteroids.

Tsis tas li ntawd:

• biochemistry ;
• kuaj ntshav;
• kev soj ntsuam ntawm cov zis.

Kev kho mob ntawm cov adrenogenital syndrome

Nws yog ib qho tsis yooj yim sua kom tshem tau ntawm cov tshuaj ntsuam genetic pathology, tab sis nws cov kev soj ntsuam tau raug tshem tawm. Adrenogenital syndrome - cov lus pom zoo:

1. Lub neej txais tos cov tshuaj hormonal. Rau normalize ua hauj lwm ntawm adrenal cortex thiab tswj lub tshuav endocrine, koj yuav tsum tau tas li haus glucocorticoids. Qhov kev xaiv dua yog Dexamethasone. Kev siv tshuaj yog suav ib qho zuj zus thiab qhov txawv ntawm 0.05 mus rau 0.25 mg ib hnub twg. Nrog ntsev-poob daim ntawv ntawm tus kab mob, nws yog ib qho tseem ceeb heev uas yuav tsum tau noj cov ntxhiab corticoids thiaj li tswj tau cov dej-ntsev seem.
2. Kho ntawm tsos. Cov neeg mob uas tau piav qhia yog pom zoo kom muaj cov plag yas, clitorectomy thiab lwm cov kev pabcuam hauv phais kom paub meej tias cov pojniam muaj qhov tseeb thiab loj.
3. Kev sab laj nrog ib tus kws kho mob (ntawm kev thov). Qee cov neeg mob xav tau kev pab hauv kev sib raug zoo thiab lees txais lawv tus kheej ua ib tus neeg muaj peev xwm.
4. Txaus siab rau ntawm ovulation. Cov poj niam uas xav ua kom cev xeeb tub yuav tsum tau noj cov tshuaj tshwj xeeb uas ua kom txoj kev kho ntawm lub cev ntas thiab qhov hluav taws xob ntawm kev ua tub rog. Glucocorticoids raug muab tshem tawm thoob plaws lub sij hawm gestation.