Antiphospholipid Syndrome (APS) yog kab mob autoimmune. Nws yog tsim los ntawm kev tsim cov tshuaj tiv thaiv rau phospholipids, uas yog cov khoom tseem ceeb ntawm cov cell membranes. Cov kab mob no tshwm sim los ntawm cov teeb meem ntawm cov hlab ntsha, lub plawv, lwm yam kabmob, nrog rau kev xeeb tub.
Nyob rau hauv tsab xov xwm, peb yuav xav txog qhov ua rau, cov tsos mob, txoj hauv kev rau kev kuaj mob antiphospholipid syndrome. Cov txheej txheem kho mob rau kev kho mob ntawm APS tau raug tshuaj xyuas pej xeem thiab tau pom zoo nyob rau lub Kaum Ob Hlis 2013. Cov lus qhia tseem muab cov lus piav qhia ntxaws txog dab tsi ua rau tus mob antiphospholipid. Cov ntaub ntawv muaj nyob hauv daim ntawv no yog cov lus qhia siv tau rau cov kws kho mob ua haujlwm nrog cov neeg mob kuaj mob APS.
cov ntaub ntawv dav dav
APS tuaj yeem raug hu ua cov tsos mob, suav nrog cov hlab ntsha rov qab thiab cov hlab ntsha venous, nrog rau obstetric pathology. Muaj ob hom kab mob:
- Y Primary antiphospholipid syndrome.
- Secondary API.
Pib raug kuaj pom thaum tus neeg mob tsis pom lwm yam kab mob tshwj tsis yog APS rau 5 xyoos.
Secondary yog ib yam kab mob uas tau tsim tawm tsam keeb kwm ntawm lwm yam kab mob (lupus erythematosus, scleroderma, mob caj dab rheumatoid thiab lwm yam).
Nyob rau hauv thawj qhov sib txawv, tus neeg mob tsis muaj erythema ntawm lub ntsej muag, daim tawv nqaij ua pob liab vog, stomatitis, o ntawm peritoneum, Raynaud's syndrome, thiab tsis muaj cov tshuaj tiv thaiv kab mob, tshuaj tiv thaiv rau haiv neeg DNA thiab cov tshuaj tiv thaiv rau Sm-antigen hauv kuaj ntshav.
Kev kuaj kab mob
Antiphospholipid syndrome tau kuaj pom thaum tib neeg muaj tsawg kawg yog ib qho chaw kho mob thiab kuaj ntsuas rau nws qhov tshwm sim. Yog tias tsuas muaj cov txheej txheem kho mob nkaus xwb, thiab tsis muaj qhov ntsuas ntsuas, ces qhov kev kuaj mob ntawm tus kab mob no tsis tau ua. Tsis tas li ntawd, kev kuaj mob ntawm APS tsis yog ua nyob rau hauv lub xub ntiag ntawm cov qauv kuaj xwb. Kev kuaj mob ntawm APS yog txiav txim siab yog tias ib tus neeg muaj cov tshuaj tiv thaiv kab mob antiphospholipid hauv lawv cov ntshav ntau tshaj tsib xyoos ua ke, tab sis tsis muaj cov tsos mob tshwm sim.
Kev kuaj mob ntawm antiphospholipid syndrome muaj nws tus kheej nuances.
Muab hais tias txhawm rau txiav txim siab qhov ntsuas qhov ntsuas ntawm APS, nws yuav tsum tau kuaj xyuas qhov concentration ntawm cov tshuaj tiv thaiv kab mob antiphospholipid hauv cov ntshav tsawg kawg ob zaug, nws tsis tuaj yeem kuaj xyuas qhov tseeb hauv ib qho kev kuaj mob. Tsuas yog thaum cov kev ntsuam xyuas cuam tshuam tau dhau ob zaug tuaj yeem ntsuas cov qauv ntsuas.
Qhov kev kuaj pom zoo yuav raug txiav txim tsuas yog tias tus nqi ntawm cov tshuaj tiv thaiv rau phospholipids hauv kev kuaj ntshav rau cov tshuaj tiv thaiv phospholipid tau nce ob zaug ua ke. Nyob rau hauv cov xwm txheej uas tsuas yog ib zaug cov tshuaj tiv thaiv antiphospholipid tau pom nyob rau hauv qhov nce ntxiv, thiab thaum rov kuaj xyuas lawv zoo li qub, qhov no suav tias yog qhov tsis zoo thiab tsis ua qhov cim ntawm tus kab mob no. Kev kuaj mob ntawm cov kab mob antiphospholipid yuav tsum tau ua los ntawm tus kws kho mob tshwj xeeb.
Qhov tseeb yog qhov nce ib ntus ntawm cov tshuaj tiv thaiv kab mob antiphospholipid hauv cov ntshav tuaj yeem tshwm sim ntau zaus. Qhov tseeb, nws raug kho tom qab txhua tus kab mob sib kis, txawm tias tawm tsam keeb kwm ntawm banal otolaryngological ailments. Qhov nce ib ntus ntawm cov tshuaj tiv thaiv kab mob no tsis tas yuav kho thiab daws nws tus kheej li ob peb lub lis piam xwb.
Thaum qhov kev kuaj mob ntawm tus mob no tau lees paub lossis tsis lees paub, nws yuav tsum tsis txhob txiav txim siab tam sim ntawd, vim tias lawv qib tuaj yeem hloov pauv nyob ntawm ntau yam, xws li mob khaub thuas lossis kev ntxhov siab tsis ntev los no.
Kev sib txawv ntawm lwm yam kab mob
Antiphospholipid syndrome raws li ICD 10 muaj code D 68.6. Qhov kev hloov kho thib kaum tau muaj nyob rau xyoo 1989 hauv Geneva. Nws qhov kev tsim kho tshiab yog siv cov lej thiab cov tsiaj ntawv hauv kab mob. Ua ntej no, antiphospholipid syndrome raws li ICD 9 muaj cai 289.81 hauv chav kawm "Cov kab mob ntawm cov ntshav thiab cov kab mob hematopoietic". APS feem ntau tuaj yeem tsis meej pem nrog lwm cov kab mob. Yog li, tus kab mob yuav tsum muaj peev xwm paub qhov txawv ntawmCov kab mob hauv qab no nrog cov tsos mob zoo sib xws:
- Tus neeg mob tau kis los yog caj ces thrombophilia.
- Ntseeg ntawm fibrinolysis defects.
- Kev txhim kho ntawm cov qog qog nqaij hlav ntawm ib qho chaw hauv zos.
- Kev muaj atherosclerosis lossis embolism.
- Kev txhim kho myocardial infarction nrog thrombosis ntawm lub plawv ventricles.
- Kev txhim kho ntawm kev mob nkeeg.
- Tus neeg mob muaj thrombotic thrombocytopenic purpura lossis hemolytic uremic syndrome.
Yuav ua li cas xeem yuav tsum tau ua kom kuaj tau APS
Raws li ib feem ntawm kev kuaj mob ntawm tus kab mob xws li antiphospholipid syndrome, nws yog ib qho tsim nyog yuav tsum pub ntshav los ntawm cov hlab ntsha. Qhov no yog ua tiav ntawm lub plab khoob thaum sawv ntxov. Tib lub sijhawm, tus neeg yuav tsum tsis txhob mob khaub thuas.
Thaum tus neeg mob hnov mob tsis xis nyob, nws tsis tuaj yeem ua qhov kev soj ntsuam rau cov kab mob antiphospholipid. Nws yuav tsum tau tos kom txog thaum lub xeev zoo li qub, thiab tsuas yog tom qab ntawd ua qhov kev sim tsim nyog.
Tam sim ua ntej ua qhov kev xeem no, tsis tas yuav ua raws li kev noj haus tshwj xeeb, tab sis nws tseem ceeb heev kom txwv tsis pub haus cawv, haus luam yeeb thiab noj zaub mov tsis zoo. Kev ntsuam xyuas tuaj yeem ua tiav rau txhua hnub ntawm kev coj khaub ncaws, yog tias nws txhawj xeeb txog poj niam. Yog li, ua ib feem ntawm kev kuaj mob ntawm antiphospholipid syndrome, nws yog ib qho tsim nyog los ua cov kev tshawb fawb hauv qab no:
- Antibodies rau phospholipids zoo li "IgG" thiab "IgM".
- Antibody rau cardiolipin hom "IgG" thiab "IgM".
- Cov tshuaj tiv thaiv rau glycoprotein hom "IgG" thiab"IgM".
- Tshawb nrhiav ntawm lupus anticoagulant. Nws raug pom zoo los txiav txim siab qhov ntsuas no hauv chav kuaj siv Russell kuaj siv viper venom.
- Tshuaj ntshav suav nrog platelet suav.
- Ua ib coagulogram.
Cov kev tshuaj ntsuam qhia tau txaus los ua lossis tsis lees paub qhov kev kuaj mob sib xws. Raws li kev pom zoo ntawm tus kws kho mob, koj tuaj yeem ua lwm yam kev ntsuas ntxiv rau cov ntsuas uas qhia txog lub xeev ntawm cov ntshav coagulation system. Piv txwv li, koj tuaj yeem nqa D-dimer, thromboelastogram, thiab lwm yam. Txawm li cas los xij, cov kev ntsuam xyuas ntxiv no yuav tsis tso cai kom paub meej qhov kev kuaj mob, tab sis raws li lawv lub hauv paus nws yuav muaj peev xwm txheeb xyuas qhov kev pheej hmoo ntawm thrombosis thiab coagulation system tag nrho.
Antiphospholipid syndrome thiab cev xeeb tub
Hauv cov poj niam, APS tuaj yeem ua rau nchuav menyuam (yog tias lub sijhawm luv luv) lossis kev ua haujlwm ua ntej.
Tus kab mob tuaj yeem ua rau kev loj hlob qeeb lossis tuag hauv plab. Kev tshem tawm ntawm cev xeeb tub feem ntau tshwm sim hauv 2nd thiab 3rd trimesters. Yog tias tsis muaj kev kho mob, qhov txiaj ntsig zoo li no yuav tshwm sim hauv 90-95% ntawm cov neeg mob. Nrog rau kev kho kom raug raws sijhawm, kev xeeb tub tsis zoo yuav tshwm sim hauv 30% ntawm cov neeg mob.
Kev xaiv rau kev xeeb tub:
- Kev tuag ntawm fetus noj qab nyob zoo yam tsis muaj laj thawj.
- Pre-eclampsia, eclampsia lossis placental insufficiency ua ntej 34 lub lis piam.
- Kev rho menyuam tawm mus txog li 10 lub lis piam, tsis muaj chromosomal txawv txav hauv cov niam txiv, nrog rau hormonal lossis anatomicalmaternal genital disorders.
Antiphospholipid syndrome tuaj yeem ua rau cev xeeb tub.
Kev kho mob tshwm sim tuaj yeem tsis tuaj. Hauv cov xwm txheej zoo li no, tus kab mob no tsuas yog kuaj tau thaum ua qhov kev kuaj sim. Acetylsalicylic acid txog li 100 mg ib hnub yog muab raws li kev kho mob, tab sis cov txiaj ntsig ntawm txoj kev kho no tsis tau tsim muaj tseeb.
Asymptomatic antiphospholipid syndrome yog kho nrog Hydroxychloroquine. Tshwj xeeb tshaj yog feem ntau nws yog sau rau concomitant connective cov ntaub so ntswg kab mob, xws li systemic lupus erythematosus. Yog tias muaj kev pheej hmoo ntawm thrombosis, "Heparin" tau muab tshuaj rau hauv koob tshuaj tiv thaiv.
Tom ntej no, peb yuav pom tias cov kab mob no tau kho li cas.
Kev kho mob
Tam sim no, hmoov tsis zoo, kev kho mob ntawm cov kab mob antiphospholipid yog ib txoj haujlwm nyuaj heev, txij li hnub no tseem tsis muaj cov ntaub ntawv raug thiab txhim khu kev qha ntawm cov txheej txheem thiab ua rau cov kab mob no.
Kev kho mob tam sim no tau qhia rau kev tshem tawm thiab tiv thaiv thrombosis. Yog li, kev kho mob yog qhov tseem ceeb ntawm cov tsos mob thiab tsis tso cai kom ua tiav qhov kev kho mob rau tus kab mob no. Qhov no txhais tau hais tias cov kev kho mob zoo li no tau ua tiav rau lub neej, vim nws ua rau txo qis kev pheej hmoo ntawm thrombosis, tab sis tib lub sijhawm tsis tshem tawm cov kab mob. Ntawd yog, nws hloov tawm tias, raws li lub xeev cov tshuaj thiab kev paub txog kev tshawb fawb niaj hnub no, cov neeg mob yuav tsum tshem tawm cov tsos mob ntawm APS rau lub neej. Hauv kev kho rau qhov noCov kab mob, ob lub ntsiab lus tseem ceeb yog qhov txawv, uas yog qhov kev daws teeb meem ntawm frolicking thrombosis, nrog rau kev tiv thaiv rov ua dua ntawm thrombosis.
Kev kho mob xwm txheej ceev
Tawm tsam keeb kwm yav dhau los ntawm kev puas tsuaj antiphospholipid syndrome, kev kho mob ceev tau ua rau cov neeg mob, uas tau ua hauv kev saib xyuas hnyav. Txhawm rau ua qhov no, siv txhua txoj hauv kev los tiv thaiv kab mob thiab mob hnyav, piv txwv li:
- Tshuaj tua kab mob uas tshem tawm foci ntawm kev kis kab mob.
- Kev siv "Heparin". Tsis tas li ntawd, cov tshuaj uas tsis muaj molecular li Fraxiparin yog siv nrog Fragmin thiab Clexane. Cov tshuaj no pab txo cov ntshav txhaws.
- Kho nrog glucocorticoids hauv daim ntawv ntawm "Prednisolone", "Dexamethasone" thiab lwm yam. Cov tshuaj no tso cai rau koj tso tseg cov txheej txheem inflammatory.
- Kev siv ib txhij ntawm glucocorticoids nrog "Cyclophosphamide" rau kev kho mob ntawm cov txheej txheem mob hnyav.
- Kev txhaj tshuaj ntawm immunoglobulin rau ntawm keeb kwm ntawm thrombocytopenia. Qhov kev ntsuas no tsim nyog nyob rau hauv qhov muaj pes tsawg tus platelets hauv cov ntshav.
- Yog tias tsis muaj txiaj ntsig los ntawm kev siv glucocorticoids, immunoglobulin thiab Heparin, kev sim tshuaj genetic engineered tshuaj xws li Rituximab thiab Eculizumab tau qhia.
- Plasmapheresis tsuas yog ua nrog qhov siab heev ntawm cov tshuaj tiv thaiv phospholipid.
Qee cov kev tshawb fawb tau ua pov thawj qhov ua tau zoo ntawm Fibrinolysin nrog rau Urokinase, Alteplase thiab"Antisreplaza" raws li ib feem ntawm qhov kev pab cuam ntawm kev puas tsuaj ntawm tus kab mob. Tab sis kuv yuav tsum hais tias cov tshuaj no tsis raug sau tseg tas li, vim tias lawv siv tau cuam tshuam nrog kev pheej hmoo los ntshav ntau.
Tshuaj kho mob thrombosis
Raws li ib feem ntawm kev tiv thaiv ntawm thrombosis, cov neeg uas muaj tus mob no yuav tsum tau noj cov tshuaj tas mus li uas txo cov ntshav txhaws. Ncaj qha kev xaiv ntawm cov tshuaj txiav txim cov yam ntxwv ntawm kev kho mob chav kawm ntawm tus kab mob no. Txog niaj hnub no, cov kws kho mob tau pom zoo kom ua raws li cov lus qhia hauv qab no thaum tiv thaiv thrombosis hauv cov neeg mob uas muaj cov kab mob antiphospholipid:
- Thaum APS nrog cov tshuaj tiv thaiv rau phospholipids, tiv thaiv qhov tsis muaj qhov chaw kho mob ntawm thrombosis, koj tuaj yeem txwv koj tus kheej rau acetylsalicylic acid hauv cov koob tshuaj qis ntawm 75 milligrams ib hnub. "Aspirin" nyob rau hauv cov ntaub ntawv no yog coj mus rau lub neej los yog kom txog thaum ib tug hloov nyob rau hauv kev kho mob tactics. Yog tias qhov mob no yog theem nrab (piv txwv li, nws tshwm sim tawm tsam keeb kwm ntawm lupus erythematosus), cov neeg mob raug pom zoo kom siv Hydroxychloroquine ib txhij nrog Aspirin.
- Warfarin tau pom zoo rau APS nrog ntu ntawm venous thrombosis. Ntxiv rau Warfarin, lawv tuaj yeem sau tshuaj Hydroxychloroquine.
- Nyob rau hauv cov kab mob ntawm cov tshuaj tiv thaiv antiphospholipid nrog rau lub sijhawm ntawm cov hlab ntsha, nws kuj pom zoo kom siv Warfarin thiab Hydroxychloroquine. Thiab ntxiv rau "Warfarin" thiab "Hydroxychloroquine", yog tias muaj kev pheej hmoo siab ntawm thrombosis, lawv kuj tau sau ntawv. Aspirin thaum noj tsawg.
tshuaj ntxiv rau kev kho mob
Ntxiv rau ib qho ntawm cov txheej txheem saum toj no, qee cov tshuaj yuav raug sau tseg txhawm rau kho cov kab mob uas twb muaj lawm. Piv txwv li, tiv thaiv keeb kwm yav dhau los ntawm qhov nruab nrab thrombocytopenia, kev siv tshuaj tsawg ntawm glucocorticoids - Metipred, Dexamethasone, Prednisolone, thiab lwm yam. Nyob rau hauv lub xub ntiag ntawm kev kho mob tseem ceeb thrombocytopenia, glucocorticoids yog siv, thiab Rituximab. "Immunoglobulin" kuj tseem siv tau.
Yog tias qhov kev kho mob tsis tso cai kom cov platelets hauv cov ntshav nce ntxiv, ces kev phais tshem tawm ntawm tus po yog ua. Nyob rau hauv lub xub ntiag ntawm lub raum pathologies tawm tsam keeb kwm ntawm tus kab mob no, cov tshuaj los ntawm qeb ntawm inhibitors yog siv, piv txwv li, Captopril los yog Lisinopril.
tshuaj tshiab
Tsis ntev los no, tau muaj kev txhim kho cov tshuaj tshiab uas tiv thaiv thrombosis, uas suav nrog heparinoids, thiab ntxiv rau, receptor inhibitors, xws li "Ticlopidine" nrog rau "Tagren", "Clopidogrel" thiab "Plavix".
Raws li cov ntaub ntawv ua ntej, tau tshaj tawm tias cov tshuaj no muaj txiaj ntsig zoo hauv cov tshuaj tiv thaiv phospholipid. Nws yog qhov zoo heev uas lawv yuav sai sai no rau hauv cov qauv kev kho mob pom zoo los ntawm cov zej zog thoob ntiaj teb. Txog niaj hnub no, cov tshuaj no tau siv los ua ib feem ntawm kev kho mob antiphospholipid syndrome, tab sis txhua tus kws kho mob sim sau lawv raws li nws tus kheej cov tswv yim.
Yog xav tau kev phais mob hauv qhov mob no, ib tus yuav tsumntev li ntev tau kom noj cov tshuaj tiv thaiv coagulants hauv daim ntawv ntawm "Warfarin" thiab "Heparin". Lawv yuav tsum tau muab tso tseg rau lub sijhawm tsawg kawg nkaus ua ntej ua haujlwm. Nws yog ib qho tsim nyog yuav rov pib noj Warfarin tom qab phais.
Ntxiv rau, cov neeg uas raug kev txom nyem los ntawm cov kab mob antiphospholipid yuav tsum tau tawm ntawm txaj thiab pib txav sai li sai tau tom qab kev phais. Nws yuav tsis yog superfluous rau hnav cov ris tsho ua los ntawm cov khoom tshwj xeeb compression, uas yuav tso cai rau kev tiv thaiv ntxiv ntawm kev pheej hmoo ntawm thrombosis. Es tsis txhob tshwj xeeb compression ris tsho hauv qab, ib qho yooj yim qhwv ntawm ob txhais ceg nrog elastic bandages yuav ua.
Cov lus qhia kho mob rau cov kab mob antiphospholipid yuav tsum tau ua raws nruj me ntsis.
Yuav siv tshuaj dab tsi
Kev kho tus kab mob no, siv tshuaj los ntawm pab pawg hauv qab no:
- Kho nrog cov tshuaj tiv thaiv kab mob thiab cov tshuaj tiv thaiv tsis ncaj. Hauv qhov no (ntxiv rau cov tshuaj Aspirin thiab Warfarin), Pentoxifylline feem ntau siv.
- Y Kev siv cov glucocorticoids. Hauv qhov no, cov tshuaj "Prednisolone" tuaj yeem siv tau. Hauv qhov no, nws tseem tuaj yeem ua ke nrog cov tshuaj tiv thaiv kab mob hauv daim ntawv ntawm "Cyclophosphamide" thiab "Azathioprine"..
- Kev siv tshuaj aminoquinoline, piv txwv li, Delagil lossis Plaquenil.
- Siv cov tshuaj uas tsis yog-steroidal anti-inflammatory siv nyob rau hauv daim ntawv ntawm Nimesulide, Meloxicam los yog Celecoxib.
- Raws li ib feem ntawm cov kab mob obstetric, "Immunoglobulin" yog siv rau hauv cov hlab ntsha.
- Kev kho mob nrog cov vitamins B.
- Kev siv cov polyunsaturated fatty acid npaj, piv txwv li, Omacora.
- Siv cov tshuaj tiv thaiv kab mob zoo li Mexicora.
Cov tshuaj hauv qab no tseem tsis tau siv dav, tab sis lawv tau txais txiaj ntsig zoo hauv kev kho mob antiphospholipid syndrome:
- Siv cov tshuaj tiv thaiv monoclonal rau platelets.
- Kho nrog anticoagulant peptides.
- Kev siv cov tshuaj apoptosis inhibitors.
- Kev siv cov tshuaj tiv thaiv kab mob enzyme, piv txwv li, Wobenzym lossis Phlogenzym.
- Kev kho mob nrog cytokines (feem ntau siv niaj hnub no yog Interleukin-3).
Raws li ib feem ntawm kev tiv thaiv ntawm thrombosis recurrent, indirect anticoagulants yog siv feem ntau. Hauv cov xwm txheej thib ob ntawm cov kab mob antiphospholipid, kev kho mob yog ua tawm tsam keeb kwm yav dhau los ntawm kev kho mob txaus ntawm tus kab mob.
Kev ntsuas tawm tsam keeb kwm ntawm pathology
Qhov kev cia siab rau qhov kev kuaj mob no tsis meej meej thiab feem ntau yog nyob ntawm lub sijhawm ntawm kev kho mob, nrog rau kev txaus ntawm cov txheej txheem kho. Qhov tseem ceeb sib npaug yog kev qhuab qhia ntawm tus neeg mob, ua raws li tag nrho cov tshuaj uas tsim nyog ntawm tus kws kho mob tuaj koom.
Puas muaj lwm cov lus pom zoo rau cov tshuaj tiv thaiv phospholipid? Cov kws kho mob qhia kom tsis txhob ua kev kho mob ntawm lawv tus kheej kev txiav txim siab los yog cov lus qhia ntawm "kev paub", tab sis tsuas yog nyob rau hauv kev saib xyuas ntawm tus kws kho mob. Nco ntsoov, xaiv cov tshuaj rau txhua tus neeg mob yog tus kheej. Cov tshuaj uas pab ib tus neeg mob tuaj yeem ua rau qhov xwm txheej ntawm lwm tus ua rau hnyav dua. Kuj cov kws kho mobqhia rau cov neeg uas muaj APS kom saib xyuas lawv qhov chaw kuaj tsis tu ncua. Qhov no muaj tseeb tshwj xeeb rau cov poj niam cev xeeb tub thiab rau cov poj niam uas npaj los ua niam.
Kuv yuav mus cuag kws kho mob twg?
Kev kho tus kab mob no yog ua los ntawm kws kho mob rheumatologist. Xav tias feem ntau ntawm cov kab mob no cuam tshuam nrog pathologies thaum cev xeeb tub, tus kws kho mob poj niam-gynecologist feem ntau kuj koom nrog hauv kev kho mob. Txij li tus kab mob no cuam tshuam rau ntau yam kabmob, nws yuav tsum tau sab laj nrog cov kws kho mob tshwj xeeb, piv txwv li, kev koom tes ntawm cov kws kho mob xws li kws kho mob hlwb, nephrologist, ophthalmologist, dermatologist, vascular surgeon, phlebologist, cardiologist.
