Koj puas los ntshav ntau heev los ntawm qhov txhab me me? Cia peb tham txog Bernard-Soulier Syndrome (BSS)!

Koj puas tau pom tias muaj ib txhia neeg los ntshav ntau heev txawm tias yog los ntawm qhov khawb me me lossis qhov mob me me xwb, lossis qhov mob yooj yim thiab siv sijhawm ntev los kho? Qee zaum qhov no yuav yog vim muaj mob uas peb tsis tau hnov ​​​​​​dua txog, tab sis nws tseem ceeb heev uas yuav tsum paub txog. Ib qho mob zoo li no yog Bernard-Soulier Syndrome, lossis luv luv "(BSS)" . Cia peb tham txog qhov no kom ntxaws me ntsis hnub no.

Bernard-Soulier Syndrome (BSS) yog dab tsi?

Hais yooj yim xwb, Bernard-Soulier Syndrome '(BSS)' yog ib qho mob tsis tshua muaj. Qhov tshwm sim hauv qhov no yog tias cov txheej txheem ntawm cov ntshav txhaws hauv peb lub cev, uas txhais tau tias cov ntshav nres thaum peb raug mob, raug cuam tshuam. Xav txog, koj muaj ib qho qhov txhab me me ntawm koj txhais tes. Tom qab ib pliag, cov ntshav nres, puas yog? Zoo, qhov pab nrog qhov ntawd yog platelets, ib hom cell me me hauv peb cov ntshav, thiab plasma, uas yog cov kua ntawm cov ntshav.

Ib tug neeg uas muaj tus kab mob Bernard-Soulier Syndrome (BSS) muaj cov ntshav tsawg dua li qub. Cov kws kho mob hu qhov mob no ua thrombocytopenia. Tsis tas li ntawd, cov ntshav uas muaj nyob ntawd loj dua li cov ntshav ib txwm muaj. Yog li, vim li no, cov neeg uas muaj BSS los ntshav thiab mob nqaij yooj yim.

Tus mob no tsawg npaum li cas?

Qhov no yog ib qho mob tsis tshua muaj. Raws li cov kws kho mob hais, tus mob no tsuas cuam tshuam rau ib tus neeg hauv ib lab tus neeg thoob ntiaj teb. Qhov no txhais tau tias txawm tias nyob hauv Sri Lanka, muaj tsawg tus neeg uas raug tus mob no.

Cov tsos mob ntawm Bernard-Soulier Syndrome (BSS) yog dab tsi?

Feem ntau, cov menyuam mos uas muaj BSS pib qhia cov tsos mob no thaum ntxov hauv lub neej. Xyuas seb koj lossis ib tus neeg koj paub puas muaj cov tsos mob no:

• Koj yooj yim raug mob, thiab nws siv sijhawm ntev los kho: Txawm tias yog ib lub pob me me ntawm lub rooj zaum tuaj yeem ua rau muaj mob loj. Nws tuaj yeem siv sijhawm ntau lub lis piam rau qhov mob ntawd kom kho tau.
• Los ntshav ntau heev txawm tias yog los ntawm cov qhov txiav me me thiab cov qhov txhab: Txawm tias koj tau txais ib qho me me thaum txiav plaub hau lossis txiav koj txhais tes nrog riam thaum txiav zaub, nws nyuaj rau kom tsis txhob los ntshav.
• Ntshav ntswg ntau zaus: Ib txhia neeg hu qhov no ua '(epistaxis)'. Yog tias koj ntshav ntswg ntau zaus yam tsis muaj laj thawj, qhov ntawd kuj yog ib qho tsos mob.
• Los ntshav hauv qab daim tawv nqaij: Cov no zoo li cov me me liab lossis xim av ntawm qhov chaw ntawm daim tawv nqaij. Cov kws kho mob hu cov no "petechiae".
• Los ntshav ntau thaum lub sijhawm muaj hli rau cov poj niam: Yog tias los ntshav ntev dua li niaj zaus ob peb hnub, lossis yog tias nws los ntshav ntau (menorrhagia), qhov ntawd kuj yog qhov txhawj xeeb.

Tsis txhob ntshai BSS tsuas yog vim koj muaj ib lossis ob yam ntawm cov tsos mob no. Tab sis yog tias cov tsos mob no tseem muaj, nws yog qhov zoo tshaj plaws los nrhiav kev pab kho mob.

Dab tsi ua rau muaj tus mob Bernard-Soulier Syndrome (BSS)?

Qhov no yog qhov tseem ceeb tshaj plaws. `(BSS)` yog ib qho kev mob caj ces.. Ntawd yog, ib qho mob uas tshwm sim thaum peb yug los vim muaj kev hloov pauv (kev hloov pauv) hauv peb cov noob caj noob ces. Qhov no yog los ntawm noob caj noob ces , uas txhais tau tias cov menyuam yaus tuaj yeem tau txais qhov kev hloov pauv caj ces no los ntawm lawv niam lawv txiv.

Tus mob no (BSS) yog tshwm sim los ntawm kev hloov pauv hauv cov noob 'GP1BA', 'GP1BB' lossis 'GP9' tshwj xeeb. Tag nrho cov noob no yog qhov tseem ceeb heev rau cov txheej txheem ntshav txhaws hauv peb lub cev. Yog li thaum muaj teeb meem nrog cov noob no, cov ntshav txhaws tsis tshwm sim kom raug.

Koj paub li cas yog tias koj muaj tus mob Bernard-Soulier Syndrome (BSS)?

Yog koj muaj cov tsos mob no, thawj qhov uas kws kho mob yuav ua thaum koj pom lawv yog nug koj ib lo lus nug kom meej. Tom qab ntawd, lawv yuav xaj ntau zaus kuaj ntshav kom paub meej tias koj muaj mob.

• Kev kuaj suav cov platelet: Qhov no xyuas seb cov platelets hauv koj cov ntshav puas tsawg.
• Kev kuaj mob hloov pauv ntawm cov noob caj noob ces: Qhov no tuaj yeem ntes tau seb puas muaj kev hloov pauv hauv cov noob caj noob ces `GP1BA`, `GP1BB`, `GP9` uas tau hais ua ntej uas ua rau `(BSS)`.
• Kev kuaj xyuas cov platelets hauv lub tshuab microscopic: Ib qho qauv ntawm koj cov ntshav raug coj mus kuaj hauv qab lub tshuab microscope kom pom tias cov platelets loj tsis zoo li qub.
• Kev ntsuas cov ntshav ntws hauv cov ntshav (Platelet flow cytometry): Txawm hais tias nws muaj lub npe nyuaj, qhov kev kuaj no ntsuas qhov ntau ntawm cov glycoproteins hu ua 'GP1BA', 'GP1BB', 'GP9' ntawm qhov chaw ntawm cov ntshav. Yog tias cov no qis, nws yog ib qho cim ntawm '(BSS)'.

Nws yog raws li cov txiaj ntsig ntawm cov kev kuaj no uas tus kws kho mob yuav qhia rau koj paub meej tias koj puas muaj BSS lossis tsis muaj.

Cov kev kho mob rau Bernard-Soulier Syndrome (BSS) yog dab tsi?

Tam sim no tsis muaj tshuaj kho tau BSS, vim nws yog ib yam mob uas los ntawm noob caj noob ces. Txawm li cas los xij, muaj ntau yam uas koj ua tau los tiv thaiv kev los ntshav ntau dhau thiab txo lwm yam teeb meem uas yuav tshwm sim. Kev kho mob feem ntau yog tsom mus rau qhov ntawd.

Tej yam koj ua tau kom txo qhov kev los ntshav:

Cov no yog cov yam yooj yim, tab sis tseem ceeb heev uas koj tuaj yeem ntxiv rau hauv koj lub neej txhua hnub.

• Tsis txhob noj cov tshuaj uas ua rau muaj kev pheej hmoo ntawm kev los ntshav: Qee cov tshuaj tua kab mob, xws li cov tshuaj nonsteroidal anti-inflammatory drugs (NSAIDs), aspirin, thiab qee cov tshuaj antihistamines tuaj yeem ua rau muaj kev pheej hmoo ntawm kev los ntshav. Yog li ntawd, tsis txhob noj cov tshuaj no yam tsis tau sab laj nrog kws kho mob.
• Siv txhuam hniav uas muaj cov plaub mos mos thaum txhuam koj cov hniav: Qhov no tuaj yeem txo cov pos hniav uas los ntshav.
• Kawm txoj kev kom raug los tiv thaiv kev ntshav ntswg: nug koj tus kws kho mob txog yuav ua li cas tuav koj lub qhov ntswg thiab yuav ua li cas tuav koj lub taub hau.
• Txwv qee yam khoom noj thiab dej haus: Cov khoom noj uas muaj cawv ntau thiab cov suab thaj ntau tuaj yeem cuam tshuam rau koj cov ntshav platelet, yog li nws yog qhov zoo tshaj plaws kom txwv lawv ntau li ntau tau.
• Sau npe rau ntawm ib lub chaw uas muab kev kho mob thaum muaj xwm txheej ceev 24 teev: Qhov no yuav pab koj tau txais kev kho mob sai sai thaum muaj xwm txheej ceev, xws li kev los ntshav loj.
• Noj cov tshuaj ntxiv hlau yog tias koj tsis muaj hlau txaus: Kev los ntshav ntau zaus tuaj yeem ua rau tsis muaj hlau txaus. Noj cov tshuaj ntsiav hlau raws li koj tus kws kho mob qhia.
• Hnav ib txoj hlua tes lossis daim ntawv cim qhia txog tus kheej: Nws yog ib qho tseem ceeb heev thaum muaj xwm txheej ceev kom hnav ib txoj hlua tes lossis daim ntawv cim uas qhia meej tias koj muaj '(BSS)'.

Kev kho mob:

Muaj tej lub sij hawm, tshwj xeeb tshaj yog thaum los ntshav sai sai los yog thaum npaj phais, cov kws kho mob yuav muab kev kho mob xws li:

• Kev hloov ntshav platelet: Qhov no feem ntau yog thawj txoj kev kho mob rau qhov xwm txheej ceev uas los ntshav. Cov platelets los ntawm tus neeg pub dawb raug muab rau hauv koj lub cev los ntawm txoj kab IV. Cov platelets no pab koj cov ntshav txhaws.
• Kev kho mob Antifibrinolytic: Cov no yog cov tshuaj uas pab kom cov ntshav khov. Piv txwv li, tranexamic acid, uas tej zaum koj yuav tau hnov ​​​​​​txog, kuj muaj nyob rau hauv lub npe lag luam "Cyklokapron®". Qhov no pab tswj kev los ntshav.

Puas tiv thaiv tau tus kab mob Bernard-Soulier Syndrome (BSS)?

Tu siab kawg li, tsis muaj ib txoj hauv kev los tiv thaiv BSS kom tsis txhob loj hlob vim nws yog ib qho mob caj ces.

Txawm li cas los xij, yog tias ib tug neeg hauv koj tsev neeg muaj BSS, lossis yog tias koj xav tias koj muaj qhov kev hloov pauv ntawm cov noob caj noob ces no, tej zaum koj yuav xav txog kev kuaj noob caj noob ces . Nws tseem ceeb heev uas yuav tsum nrhiav kev pab tswv yim txog noob caj noob ces . Tus kws pab tswv yim txog noob caj noob ces tuaj yeem qhia koj ntxiv txog qhov mob thiab piav qhia txog qhov kev pheej hmoo ntawm kev kis tus kab mob mus rau koj cov menyuam.

Muaj tej yam dab tsi uas yuav tsum paub thaum nyob nrog Bernard-Soulier Syndrome (BSS)?

Feem ntau cov neeg uas muaj BSS tuaj yeem ua neej nyob li qub. Txawm li cas los xij, lawv yuav tsum ceev faj me ntsis kom txo qhov kev pheej hmoo ntawm kev raug mob.

Piv txwv li, nws yog qhov zoo tshaj plaws kom tsis txhob ua si kis las uas sib chwv, vim tias lawv muaj kev pheej hmoo siab dua ntawm kev raug mob thiab los ntshav loj.

Nws tseem ceeb heev uas yuav tsum mus ntsib koj tus kws kho mob tsis tu ncua kom koj noj qab haus huv tag nrho. Yog tias koj muaj kev phais, tej zaum yuav tsum tau teem sijhawm ua ntej thiab tej zaum yuav tsum tau hloov ntshav ua ntej txoj kev phais.

Yog koj muaj tus mob Bernard-Soulier Syndrome (BSS), koj yuav tsum nug koj tus kws kho mob li cas?

Yog koj xav tias koj muaj (BSS) lossis tau kuaj pom tias muaj nws, nws yuav pab tau heev rau koj nug koj tus kws kho mob cov lus nug zoo li no:

• Cov tsos mob thawj zaug ntawm Bernard-Soulier Syndrome yog dab tsi?
• Kuv yuav tsum ua dab tsi los xyuas seb kuv puas muaj tus mob Bernard-Soulier Syndrome?
• Cov kev kho mob rau Bernard-Soulier Syndrome yog dab tsi?
• Yog tias kuv muaj tus mob Bernard-Soulier Syndrome, kuv yuav ua li cas thiaj li tiv thaiv tau kev los ntshav ntau dhau?
• Kuv yuav tsum tsis txhob ua dab tsi vim kuv muaj tus mob Bernard-Soulier Syndrome?
• Muaj feem ntau npaum li cas uas kuv cov menyuam yuav tau txais Bernard-Soulier Syndrome los ntawm kuv?

Ntxiv rau cov lus nug no, tsis txhob ntshai tham nrog tus kws kho mob txog txhua yam uas los rau hauv koj lub siab.

Puas muaj Bernard-Soulier Syndrome (BSS) thaum cev xeeb tub muaj kev pheej hmoo rau tus menyuam?

Yog tias koj cev xeeb tub thiab muaj BSS, koj yuav xav tau kev kho mob tshwj xeeb (kev kho mob poj niam thiab ntshav uas muaj kev pheej hmoo siab) . Koj cov ntshav platelet thiab kev noj qab haus huv tag nrho yuav tsum tau saib xyuas tsis tu ncua. Thaum lub sijhawm yug menyuam, koj yuav tsis tuaj yeem siv qee hom tshuaj loog uas ua rau muaj kev pheej hmoo ntawm kev los ntshav.

Txawm tias tom qab tus menyuam yug los lawm, yuav tsum kuaj xyuas tus menyuam cov ntshav platelet tsis tu ncua. Vim tias muaj feem ntau uas koj tus menyuam yuav tau txais qhov kev hloov pauv caj ces uas ua rau "(BSS)" los ntawm koj. Yog li ntawd, nws yog ib qho tseem ceeb uas yuav tsum tham nrog koj tus kws kho mob txog qhov no thiab ua cov kauj ruam tsim nyog.

Qhov teeb meem tseem ceeb uas tshwm sim los ntawm Bernard-Soulier Syndrome (BSS) yog dab tsi?

Hauv ntej, qhov teeb meem tseem ceeb uas tshwm sim los ntawm "BSS" yog qhov ntshav tsis tuaj yeem txhaws . Qhov no yog vim li cas los ntshav ntau dhau thiab yooj yim raug mob yog cov tsos mob feem ntau.

Muaj lwm yam mob dab tsi ua rau muaj cov tsos mob zoo ib yam li Bernard-Soulier Syndrome (BSS)?

Muaj ntau lwm yam mob uas ua rau muaj teeb meem los ntshav thiab cov tsos mob zoo ib yam li BSS. Nws yog ib qho zoo kom paub cov no los ntawm lub npe xwb:

• Kab mob ntshav qab zib
• `May-Hegglin anomaly`
• Kab mob ntawm lub pas dej cia khoom
• `Glanzmann thrombasthenia`
• Kab mob Von Willebrand

Raws li koj cov tsos mob, tsuas yog tus kws kho mob thiaj li paub tseeb tias nws yog tus kab mob twg.

Peb cov lus kawg

Bernard-Soulier Syndrome '(BSS)' yog ib yam mob tsis tshua muaj uas tau txais los ntawm koj niam koj txiv. Yog tias koj muaj tus mob no, koj yuav muaj teeb meem nrog kev ntshav txhaws. Txawm li cas los xij, nws tsis tau txhais hais tias koj tsis tuaj yeem ua lub neej ib txwm. Qhov tseem ceeb tshaj plaws yog kom tsis txhob muaj tej yam txaus ntshai uas tuaj yeem ua rau raug mob thiab ua raws li koj tus kws kho mob cov lus qhia tas li.

Mus ntsib koj tus kws kho mob tsev neeg tsis tu ncua. Nws tuaj yeem teb koj cov lus nug, pab koj tswj tus mob no, thiab ua haujlwm nrog koj kom tswj tau koj txoj kev noj qab haus huv tag nrho. Tsis txhob ntshai, kev paub thiab kev saib xyuas yog peb lub zog loj tshaj plaws thaum nyob nrog cov mob no.

` Bernard-Soulier Syndrome, BSS, ntshav txhaws, platelets, los ntshav ntau dhau, cov kab mob caj ces, mob nqaij tawv