Mauriac's syndrome yog ib yam kab mob uas tshwm sim los ntawm kev kho mob ntshav qab zib tsis raug thaum tseem hluas. Tus kab mob no tau piav qhia thawj zaug hauv xyoo 1930 los ntawm tus kws kho mob Fabkis yug Pierre Mauriac. Nws tau piav qhia txog cov duab tshwj xeeb hauv kev kho mob uas cov menyuam yaus muaj ntshav qab zib mellitus uas tau txais kev kho tshuaj insulin nrog kev siv tshuaj tsis raug qhia qee yam tsos mob sab nraud. Nws pom tias txhua tus menyuam yaus sab nraud muaj qhov zoo sib xws, uas tshwm sim nws tus kheej hauv qhov qis qis, rog rog, lag luam hauv kev sib deev.
Kev txhim kho kab mob
Qhov laj thawj tseem ceeb ntawm kev mob hnyav yog kev kho mob ntshav qab zib mellitus tsis raug. Hauv tus kab mob no, tus txiav ua haujlwm tsis zoo, tsis tsim cov tshuaj insulin txaus. Nrog rau pathologydeficiency ntawm cov piam thaj hauv cov hlwb vim qhov tseeb tias nws muaj ntau ntau hauv cov ntshav.
Kev txhim kho ntawm Mauriac's syndrome hauv hom 1 mob ntshav qab zib mellitus yog txuam nrog kev kho tsis txaus. Ib tus menyuam muaj mob tau txais koob tshuaj insulin tsis txaus rau lub sijhawm ntev, lossis siv cov tshuaj tsis zoo thiab tsis huv tau siv, uas ua rau tsis muaj insulin ntev.
Ntev tsis muaj insulin hauv lub cev ua rau cov txheej txheem hauv qab no:
- Metabolic ntshawv siab, tshwj xeeb, kev cuam tshuam ntawm carbohydrate metabolism.
- Npaj qhov loj ntawm daim siab thiab nws cov rog degeneration vim muaj kev puas tsuaj ntawm glycogen.
- Hloov pauv hauv cov ntshav - nce ntshav qabzib, roj cholesterol, fatty acids.
Tib lub sijhawm, cov no nyob deb ntawm tag nrho cov txheej txheem pathological uas tshwm sim nrog lub cev hauv Mauriac's syndrome, thiab cov kev sib txawv hauv qab no kuj tau pom hauv cov menyuam mob:
- Tsis txaus tsim cov tshuaj hormones tseem ceeb - cortisol, somatotropin, glucagon thiab, vim li ntawd, cuam tshuam cov txheej txheem kev loj hlob.
- Kev tawg ntawm cov protein thiab tshem tawm ntau ntawm calcium thiab phosphorus los ntawm cov pob txha, nws thiaj li ua rau kev txhim kho pob txha thiab atrophy ntawm qee cov leeg.
- Tsis muaj peev xwm nqus cov vitamins hauv cov hnyuv.
Tus kab mob feem ntau tshwm sim nws tus kheej thaum muaj hnub nyoog 15-18, tab sis cov txheej txheem ua haujlwm tsis zoo pib ua ntej. Tam sim no, cov tshuaj niaj hnub thiab ua tib zoo ntxuav tau siv los kho cov ntshav qab zib thaum ntxov.uas xyaum tsis suav nrog kev txhim kho ntawm Mauriac's syndrome.
Symptoms of disease
Mauriac's syndrome hauv ntshav qab zib mellitus muaj ntau yam tshwm sim:
- Tus menyuam muaj kev loj hlob qeeb thiab kev loj hlob inhibition. Kev rog rog thiab luv luv stature feem ntau pom. Nyob rau tib lub sijhawm, tus menyuam muaj mob yuav poob qab cov phooj ywg hauv kev loj hlob ntawm 10-30 centimeters.
- Kev sib deev retardation (tsis muaj kev sib deev yam ntxwv thiab tsis muaj poj niam cev xeeb tub).
- Kev rog, tshwj xeeb tshaj yog nyob rau hauv lub ntsej muag thiab lub cev sab sauv nrog cov ceg tawv nyias. Cov menyuam yaus muaj mob zoo ib yam, lawv muaj lub ntsej muag "lub hli", lub caj dab luv, cov rog rog hauv caj npab, xub pwg, thiab plab. Nyob rau tib lub sijhawm, qhov qis ntawm lub cev tseem nyias nyias.
- daim siab loj nrog lub voj voog ncig venous.
- Kev txhim kho pob txha (kev txhim kho pob txha qeeb).
- kab mob qhov muag, suav nrog cov kab mob ntawm retina, thiab tom qab ntawd kev loj hlob ntawm cataracts.
Moriac's syndrome feem ntau tshwm sim hauv cov menyuam yaus, daim duab ntawm tus menyuam muaj mob qhia meej tias qhov tshwm sim sab nraud tus kab mob no muaj li cas.
Kev kuaj kab mob
Kev kuaj mob ntawm "Mauriac's Syndrome" yog tsim los nrog cov cim qhia sab nraud ntawm kev loj hlob ntawm tus kab mob, xws li: kev loj hlob tsis txaus rau ib tus neeg muaj hnub nyoog, muaj kev rog, tshwj xeeb tshaj yog nyob rau hauv lub ntsej muag, kev sib deev tsis paub tab, pom meej o. lub siab.
Yuav kom paub meej qhov kev kuaj mob, kuaj ntshav tau ua, hauvRaws li qhov tshwm sim ntawm qhov tshwm sim hauv qab no tuaj yeem paub qhov txawv:
- qib qabzib tsis ruaj khov, tshwm sim los ntawm kev dhia tas li nce thiab nqis.
- Cov ntshav lipids ntau dhau (hyperlipemia).
- Qhov tseem ceeb ntawm cov roj cholesterol hauv cov ntshav (hypercholesterolemia).
Qee zaum, daim siab tau kawm ntxiv, rau qhov kev kuaj ntshav no tau ua kom pom cov rog
Kev mob nkeeg
Rau ib tug hluas lub cev, tus kab mob no nyuaj heev. Ua ib hom mob ntshav qab zib mellitus hnyav, nws nyuaj rau kev them nyiaj. Feem ntau ua rau acidosis thiab hyperglycemic coma.
Mauriac's syndrome tshwm sim hauv daim ntawv hnyav vim yog qhov sib ntxiv ntawm ntau yam kab mob sib kis, nrog rau kev ua kom rov zoo thiab ntev ntawm cov txheej txheem biochemical hauv tib neeg lub cev.
Mauriac thiab Nobecourt syndrome: zoo sib xws thiab sib txawv
Tus mob ntawm Mauriac thiab Nobekur feem ntau tshwm sim thaum yau, ob qho tib si kab mob yog ib qho teeb meem loj ntawm ntshav qab zib mellitus, uas tshwm sim tawm tsam keeb kwm ntawm kev kho tsis raug. Ob leeg syndromes muaj qhov tshwm sim zoo sib xws, lawv suav nrog kev loj hlob qeeb thiab kev loj hlob ntawm kev sib deev, kev puberty ntev, fatty degeneration ntawm daim siab. Nyob rau tib lub sijhawm, qhov sib txawv tseem ceeb ntawm Nobekur's syndrome yog qhov tsis muaj rog subcutaneous. Hauv qhov no, kev kho mob ntawm ob leeg mob yog tsom rau kev them nyiaj kho mob ntshav qab zib mellitus.
Kev Kho Mob ntawm Mauriac's Syndrome
Kev kho mob ncaj qha ntawm tustsis muaj ib yam dab tsi li syndrome. Txhua txoj kev kho yog txhawm rau tshem tawm cov hauv paus hauv paus ntawm tus kab mob thiab cov teeb meem tshwm sim los ntawm nws. Rau qhov no, tus neeg mob tau sau cov tshuaj insulin kom raug hauv cov koob tshuaj kom raug thiab cov tshuaj niaj hnub zoo.
Txhawm rau tiv thaiv lipodystrophy, ntau cov txheej txheem tiv thaiv tau ua tiav, rau lub hom phiaj no zaws, cov txheej txheem kho lub cev tau raug sau tseg thiab tsim cov phiaj xwm rau kev txhaj tshuaj insulin. Ntxiv nrog rau kev kho mob, kev noj zaub mov tshwj xeeb tuaj yeem raug sau tseg, tsis suav nrog kev noj zaub mov txhua yam khoom muaj roj tsiaj. Tib lub sijhawm, kev noj zaub mov tsom rau cov khoom noj muaj protein thiab carbohydrates.
Kev kho mob kuj tseem tsim los pab them rau cov teeb meem uas tau tsim tawm tsam keeb kwm ntawm tus kab mob hauv qab, rau qhov no tus neeg mob yuav raug tshuaj:
- Kev txais cov tshuaj hepatoprotectors txhawm rau txhawm rau txhim kho daim siab hlwb.
- B vitamins kom rov qab zoo li qub metabolism.
- Ib yam tshuaj uas tsom mus rau normalizing qib lipids thiab cov roj cholesterol hauv cov ntshav.
- tshuaj Steroid kom txhawb lub cev loj hlob.
- Kev noj tshuaj hormonal los kho cov haujlwm tseem ceeb ntawm kev sib deev.
Txhawm rau tiv thaiv kev txhim kho ntawm cov teeb meem txaus ntshai ntawm ntshav qab zib li Mauriac's syndrome, nws yuav tsum tau ua raws li kev kho mob ntawm tus kab mob hauv qab no kom raug thiab tiv thaiv cov tshuaj insulin tsis txaus.
Nyob rau hauv txoj kev kho mob ntawm tus mob uas twb muaj lawm nrog cov tshuaj niaj hnub, qhov kev tshwm sim yog heevtxaus siab, tab sis yuav tsum tau ua raws li tag nrho cov lus pom zoo ntawm tus kws kho mob. Kev noj tshuaj insulin kom raug thiab kev kho kom rov qab ua haujlwm ntawm tag nrho cov khoom nruab nrog cev tuaj yeem kho tus menyuam kom noj qab haus huv, sab nraud thiab sab hauv.
