Koj tus me nyuam puas muaj cov pob liab loj loj ntawm nws lub cev? Cia peb kawm txog PHACE Syndrome.

Cov niam thiab cov txiv, tej zaum nej yuav txhawj thiab ntshai thaum nej pom cov pob liab loj lossis cov pob me me ntawm nej tus menyuam lub cev, qee zaum ntawm lub ntsej muag thiab caj dab. Qee zaum cov no tsuas yog tej yam uas yuav kho tau, tab sis tsis tshua muaj, lawv tuaj yeem yog ib qho cim ntawm ib qho mob tsis tshua muaj thiab nyuaj xws li PHACE Syndrome . Yog li, tsis tas yuav hais ntxiv lawm, cia peb tham txog PHACE Syndrome yog dab tsi, nws ua rau dab tsi, thiab peb yuav tsum ua li cas yog tias nws yog ib yam dab tsi zoo li ntawd.

PHACE Syndrome yog dab tsi?

Zoo, ua ntej, cia peb saib seb PHACE syndrome yog dab tsi. Hais yooj yim xwb, nws yog ib qho mob tsis tshua muaj. Qhov ntawd txhais tau tias nws tsis tshwm sim rau txhua tus. Nws yog ib qho mob uas muaj txij thaum yug los. Qhov ntawd txhais tau tias nws tshwm sim thaum tus menyuam tseem nyob hauv plab. Qhov mob no tuaj yeem cuam tshuam rau ntau qhov ntawm tus menyuam lub cev. Nws feem ntau cuam tshuam rau daim tawv nqaij, lub hlwb, lub plawv, cov hlab ntsha loj, thiab qhov muag .

Feem ntau, tsuas muaj ob peb qhov tsis zoo hauv txhua qhov chaw no. Qee tus menyuam tsuas yog qhia ob peb yam tsos mob teev hauv qab no. Cov mob feem ntau pom hauv cov menyuam yaus uas muaj PHACE syndrome yog hemangiomas , cerebrovascular defects, thiab heart defects . Cov no yuav pom tau thaum cev xeeb tub, thaum yug los, lossis thaum tseem yog menyuam yaus.

PHACE yog ib lo lus luv rau ntau yam kab mob sib txawv. Nws piav qhia txog cov teeb meem loj ntawm kev loj hlob uas cuam tshuam nrog tus kab mob no. Qee zaum nws hu ua PHACES syndrome. Tus 'S' ntxiv sawv cev rau sternal clefting . Qhov no txhais tau tias pob txha nyob hauv nruab nrab ntawm lub hauv siab, lub sternum, tsis tau tsim kom zoo, lossis muaj qee qhov tsis zoo hauv nws.

Cov tsos mob ntawm tus mob PHACE yog dab tsi?

Yog li, cov tsos mob ntawm ib tug menyuam uas muaj tus kab mob PHACE yog dab tsi? Qhov no yuav txawv ntawm ib tug menyuam mus rau ib tug menyuam. Ib txhia menyuam muaj tsawg dua cov tsos mob, ib txhia muaj ntau dua. Thiab qhov hnyav ntawm cov tsos mob kuj txawv ntawm ib tug menyuam mus rau ib tug menyuam. Cia peb saib cov tsos mob tseem ceeb yog dab tsi:

• Hemangiomas: Cov no yog cov pob uas pom tseeb, tsis muaj xim ntawm daim tawv nqaij. Lawv tau tsim los ntawm kev loj hlob ntawm cov hlab ntsha ntxiv . Lawv feem ntau pom ntawm lub taub hau, lub ntsej muag, caj dab, lub cev, thiab caj npab. Lawv feem ntau loj heev thiab tuaj yeem kis mus rau lwm qhov. Txawm li cas los xij, qee zaum cov hemangiomas no kuj tuaj yeem cuam tshuam rau cov kab mob sab hauv ntawm tus menyuam. Yog tias qhov no tshwm sim, teeb meem nrog kev pom thiab kev hnov ​​​​lus yuav tshwm sim.

• Kev ua tsis zoo ntawm lub hlwb:Lub posterior fossa yog thaj chaw ntawm lub hlwb uas tswj cov haujlwm tseem ceeb xws li kev sib npaug, kev sib koom tes, thiab kev ua pa. Yog tias muaj qhov tsis zoo hauv thaj chaw no, tus menyuam yuav muaj kev txav mus los tsis raug cai thiab kev tsis taus ntawm lub hlwb . Kuj tseem yuav muaj kev hloov pauv hauv cov qauv ntawm lub hlwb nws tus kheej.

• Kev puas tsuaj ntawm lub plawv: Qee qhov teeb meem ntawm lub plawv tuaj yeem ua rau muaj cov tsos mob xws li ua tsis taus pa , cov leeg tsis muaj zog , ntshav siab , ntxhov siab ntau zaus, thiab tawm hws. Qee zaum, lub plawv ntawm tus menyuam tsis tau loj hlob tag nrho, thiab tej zaum yuav muaj qhov ntawm cov chav ntawm lub plawv (ventricular septal defect) . Lossis, tej zaum yuav muaj kev nqaim ntawm aorta (coarctation of the aorta).

• Kev tsis zoo ntawm cov hlab ntsha: Yog tias cov hlab ntsha uas nqa cov ntshav mus rau lub hlwb thiab caj dab nqaim lossis muaj lwm yam kev tsis zoo, lub hlwb yuav tsis tau txais cov ntshav txaus. Qhov no tuaj yeem ua rau muaj cov mob txaus ntshai xws li aneurysms , qaug dab peg , mob hlwb , lossis tsis hnov ​​​​​​lus ntawm ib sab ntawm lub cev.

• Qhov muag tsis zoo: Tus menyuam lub qhov muag, cov hlab ntsha ntawm lub qhov muag, lossis cov hlab ntsha hauv qhov muag tej zaum yuav tsis tau loj hlob zoo. Qhov no tuaj yeem ua rau pom kev tsis zoo, tsis pom kev zoo , qhov muag me me txawv txav , tsis tswj tau qhov muag txav, lossis qhov muag poob qis .

• Kev tsis zoo ntawm pob txha hauv siab: Raws li tau hais ua ntej lawm, pob txha hauv siab (sternum) nyob nruab nrab ntawm tus menyuam lub hauv siab tej zaum yuav ploj lawm lossis tej zaum yuav tsis loj hlob zoo. Qhov no tuaj yeem ua rau muaj kab nrib pleb, pob, thiab qhov tshwm sim hauv thaj chaw ntawd.

Tej yam teeb meem uas yuav tshwm sim ntawm PHACE syndrome

Cov menyuam yaus uas muaj tus kab mob PHACE yuav muaj feem ntau dua los tsim qee yam teeb meem vim yog cov teeb meem no hauv lawv lub cev thiab cov hlab ntsha. Nov yog lawv yog dab tsi:

• Cov teeb meem sib npaug
• Cov teeb meem hniav, piv txwv li , enamel hypoplasia thiab cov hauv paus hniav tsis zoo.
• Nyuaj nqos
• Kev ua haujlwm tsis zoo ntawm lub cev endocrine . Piv txwv li, txo qis kev ua haujlwm ntawm cov thyroid thiab txo qis kev tsim cov tshuaj hormones.
• Mob taub hau ntau zaus, qee zaum mob migraines .
• Kev loj hlob tsis zoo
• Kev qeeb ntawm kev hais lus thiab kev hais lus
• Kev pheej hmoo ntawm mob stroke nce ntxiv.
• Cov noob caj noob ces tsis loj hlob zoo

Dab tsi ua rau muaj tus mob PHACE?

Cov kws tshawb nrhiav tseem tsis tau paub meej tias vim li cas qee tus menyuam yaus thiaj li muaj tus mob PHACE syndrome. Kev tshawb fawb qhia tias nws yuav yog los ntawm ob qho tib si caj ces thiab ib puag ncig. Txawm li cas los xij, qhov kev hloov pauv caj ces tseeb uas ua rau nws tseem tsis tau meej. Qee tus kws tshaj lij ntseeg tias nws yog los ntawm kev hloov pauv caj ces (de novo) uas tshwm sim thaum lub sijhawm xeeb tub. Qhov no txhais tau tias nws feem ntau tsis tau txais los ntawm niam txiv.

Yuav kuaj mob PHACE li cas?

Zoo, yog li cov kws kho mob kuaj mob PHACE li cas? Feem ntau, tus kws kho mob yuav kuaj lub cev kom huv si thiab ua qee qhov kev kuaj mob . Lawv muaj cov qauv kuaj mob tshwj xeeb, uas yog cov lus qhia. Yog li ntawd lawv txiav txim siab seb tus menyuam puas muaj mob PHACE.

Qee zaum, cov kws kho mob tuaj yeem tau txais lub tswv yim ntawm tus mob los ntawm kev luam theej duab uas koj muaj thaum koj cev xeeb tub. Tab sis lwm lub sijhawm, nws yuav tsis pom tseeb kom txog thaum tom qab tus menyuam yug los, lossis tom qab ntawd thaum tseem yog menyuam yaus.

Feem ntau, tus kws kho mob yuav xub nrhiav seb puas muaj hemangioma loj ntawm tus menyuam lub cev, tshwj xeeb tshaj yog ntawm lub ntsej muag, caj dab, lossis taub hau. Qhov muaj ib qho hemangioma thiab tsawg kawg yog ib qho tseem ceeb ntxiv uas cuam tshuam nrog PHACE yuav txaus rau kev kuaj mob.

Ntxiv mus, yuav muaj ntau yam kev kuaj mob uas yuav ua tau los saib xyuas lub hlwb, lub plawv, cov hlab ntsha, thiab qhov muag ntawm tus menyuam mos kom zoo dua. Cov no yog cov kev kuaj mob uas feem ntau ua:

• Kev luam theej duab CT
• Echocardiograms - Qhov no saib ntawm cov qauv thiab kev ua haujlwm ntawm lub plawv.
• Kev kuaj qhov muag - los ntawm tus kws kho qhov muag.
• Kev ntsuam xyuas kev hnov ​​lus
• Kev thaij duab MRI - Thaij cov duab kom ntxaws, tshwj xeeb yog lub hlwb thiab cov hlab ntsha.
• Kev kuaj mob ultrasound

Yuav kho tus mob PHACE li cas?

Thaum kho tus mob PHACE syndrome, qhov tseem ceeb tshaj plaws yog tiv thaiv cov teeb meem uas yuav tshwm sim thiab pab tus menyuam kom nyob tau zoo li qub nrog lawv cov tsos mob. Cov txheej txheem kho mob sib txawv nyob ntawm seb cov kabmob lossis qhov twg ntawm lub cev uas tus mob cuam tshuam.Qhov ntawd txhais tau tias tsis yog txhua tus menyuam tau txais kev kho mob zoo ib yam.

Hom kev kho mob no feem ntau yog ua tiav:

• Kev kho mob siv laser rau cov hemangiomas ntawm daim tawv nqaij, lossis muab tshuaj rau lawv (piv txwv li, beta-blockers xws li propranolol) kom lawv me dua.
• Muab kev kawm tshwj xeeb , kev kho kev hais lus, thiab kev kho lub cev kom pab tau kev kawm hauv tsev kawm ntawv.
• Qee zaum yuav tsum tau phais mob kom kho qhov tsis zoo hauv lub plawv, cov hlab ntsha, lossis lub hlwb.
• Muab ntau yam tshuaj los tswj kev qaug dab peg, mob taub hau, thiab kev tsis sib npaug ntawm cov tshuaj hormones.
• Muab cov khoom siv txhawb nqa rau kev hnov ​​​​​​lus tsis zoo thiab tsom iav rau kev pom kev tsis zoo.

Muaj ntau tsev neeg uas tau nrog tus kws pab tswv yim txog noob caj ces tham kom paub ntau ntxiv txog kev kho mob rau lawv tus menyuam, thiab yuav pab lawv li cas thaum lawv loj hlob tuaj. Qhov no yuav pab tau heev.

Qhov no puas tiv thaiv tau?

Tu siab kawg li, tsis muaj ib txoj kev twg los tiv thaiv tus mob PHACE syndrome. Vim tias qhov ua rau mob no tsis paub meej, tsis muaj ib yam dab tsi uas koj ua tau los txo qhov kev pheej hmoo ntawm kev muaj menyuam nrog tus mob no.

Tab sis yog tias koj xav paub ntxiv txog koj txoj kev muaj feem yuav muaj menyuam uas muaj mob caj ces, koj tuaj yeem tham nrog koj tus kws kho mob txog kev kuaj caj ces .

Yog tias kuv tus menyuam muaj tus mob PHACE syndrome, yuav ua li cas?

Yog tias koj tus menyuam muaj tus mob no, lawv yuav tsum tau mus ntsib kws kho mob thiab kws tshwj xeeb hauv ntau qhov chaw (piv txwv li, kws kho mob plawv, kws kho mob hlwb, kws kho qhov muag) kom paub tseeb tias lawv cov tsos mob tsis cuam tshuam rau lawv lub neej. Qhov no yog ib qho mob uas xav tau kev tswj hwm mus sij hawm ntev.

Qhov mob no tsis yog tsuas cuam tshuam rau tus menyuam xwb, tab sis kuj cuam tshuam rau koj thiab lwm tus neeg hauv tsev neeg. Yog li ntawd, nws yog ib qho tseem ceeb heev uas yuav tsum xav txog koj txoj kev noj qab haus huv ntawm lub hlwb. Ib txhia neeg pom tias muaj kev nplij siab thiab lub zog los ntawm kev tham nrog tus kws kho mob hlwb .

Yog li ntawd, ib tug neeg uas muaj tus mob no yuav muaj sia nyob ntev npaum li cas? Nws nyob ntawm seb tus menyuam cov tsos mob hnyav npaum li cas thiab cov kab mob twg tau cuam tshuam.

Cov menyuam yaus uas muaj kev puas tsuaj rau lub hlwb thiab lub plawv hnyav yuav muaj lub neej luv dua. Txawm li cas los xij, yog tias ib tug menyuam muaj cov tsos mob me me thiab lawv tau txais kev kho mob zoo, lawv tuaj yeem ua neej nyob li qub.

Thaum twg koj yuav tsum mus ntsib kws kho mob?

Yog koj tus menyuam muaj ib qho ntawm cov hauv qab no, nco ntsoov mus ntsib kws kho mob:

• Yog tias koj muaj teeb meem noj mov lossis haus dej
• Yog tias tus me nyuam tsis ua tau raws li cov theem kev loj hlob uas tsim nyog rau nws lub hnub nyoog, uas yog, yog tias lawv loj hlob lig (piv txwv li, tsis tsa lawv lub caj dab raws sijhawm, tsis taug kev, tsis hais lus).
• Yog tias koj tsis teb rau tej yam uas pom, uas yog, yog tias koj pom qee yam txawv txawv hauv koj lub qhov muag.
• Yog koj pom ib qho chaw loj, tiaj tus, liab ntawm koj lub cev (zoo li hemangioma).

Thaum twg koj yuav tsum coj koj tus menyuam mus rau tsev kho mob sai sai?

Yog koj pom ib yam dab tsi zoo li no, coj koj tus menyuam mus rau chav kho mob ceev ze tshaj plaws tam sim ntawd , lossis hu rau 1990:

• Yog tias koj qhia tias koj muaj cov cim qhia tias koj muaj mob stroke (piv txwv li, ib sab ntawm lub ntsej muag poob qis, tsis ua haujlwm ntawm tes, tsis hais lus tau).
• Yog koj muaj qaug dab peg.
• Yog tias koj ua pa nyuaj, yog tias koj xav tias koj ua tsis taus pa.
• Yog tias lub plawv dhia tsis xwm yeem, lossis yog tias tus menyuam tig xiav.

Cov lus nug uas yuav tsum nug tus kws kho mob/mrs.

Koj tuaj yeem nug koj tus menyuam tus kws kho mob lossis tus nais maum cov lus nug zoo li no. Cov no yuav pab koj nkag siab txog qhov xwm txheej:

• Puas yog kuv tus menyuam muaj tus mob PHACE tiag? Kuv puas yuav tsum tau ua lwm yam kev kuaj mob kom paub tseeb tias nws muaj?
• Puas yog kuv tus menyuam yuav tsum tau phais ? Yog tias yog, vim li cas thiab thaum twg?
• Puas muaj kev kho mob kom tshem tau kuv tus menyuam cov hemangioma ? Puas yuav kho tau tag nrho?
• Puas muaj tej yam tshwm sim ntawm cov kev kho mob no? Lawv yog dab tsi?
• Tej yam twg peb yuav tsum tau ceev faj tshwj xeeb rau tus menyuam?
• Kuv tus menyuam lub neej yuav ntev npaum li cas?
• Tus me nyuam puas yuav muaj peev xwm ua neej nyob li qub nrog tus mob no?

Thaum kawg, ib yam dab tsi yuav tsum nco ntsoov

Thaum koj pom tias koj tus menyuam muaj ib yam mob tsis tshua muaj thiab koj tsis paub ntau txog nws, koj tuaj yeem xav tsis thoob, tu siab, thiab ntshai heev. Nws nyuaj rau ntseeg. Qhov no tuaj yeem muaj kev cuam tshuam loj heev rau koj thiab koj tsev neeg.

Muaj ib txhia menyuam yaus uas muaj tus mob no nyob tau zoo mus txog thaum lawv loj hlob. Txawm li cas los xij, rau lwm tus, lub neej ntev yuav luv dua vim muaj cov tsos mob hnyav. Kev koom nrog ib pawg neeg txhawb nqa, lossis tham nrog tus kws pab tswv yim txog noob caj noob ces lossis tus kws kho mob hlwb , tuaj yeem yog qhov chaw zoo rau kev nplij siab thiab lub zog rau koj thiab koj tsev neeg lub sijhawm no. Yog li ntawd, tsis txhob yig nrhiav kev pab ntawd.

Nco ntsoov tias, koj tsis yog tib tug xwb. Muaj cov kws kho mob, cov kws tu mob, thiab ntau lwm tus uas tuaj yeem pab koj kov yeej cov teeb meem no, mloog koj cov lus nug, thiab muab cov ntaub ntawv uas koj xav tau rau koj. Yog li ntawd, nyob ruaj khov thiab sim muab qhov zoo tshaj plaws rau koj tus menyuam uas lawv xav tau.

` PHACE syndrome, hemangioma, cov kab mob uas muaj txij thaum yug los, cov teeb meem ntawm lub hlwb, cov teeb meem ntawm lub plawv, cov teeb meem ntawm cov hlab ntsha, cov teeb meem ntawm qhov muag, cov kab mob caj ces, cov kab mob tsis tshua muaj, kev noj qab haus huv ntawm menyuam yaus