Mwana wako anowanzova nemapundu here? Kana kuti unoti majoini ake anorwadza here? Maziso ake dzimwe nguva anotsvuka here uye kuona kwake kuchiita sekusanyatsojeka? Kunyangwe chimwe chezvinhu izvi chingaitika, dzimwe nguva zvese zvinogona kuitika pamwe chete. Nhasi tichataura nezvechirwere chisingawanzoitiki asi chakakosha chekuziva. Ndiyo Blau Syndrome .

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Chii chinonzi Blau Syndrome?

Zvichitaurwa zviri nyore, Blau Syndrome chirwere chisingawanzoitiki chekuzvimba chinokanganisa ganda remwana wako, majoini, nemaziso . 'Kuzvimba' zvinoreva kuzvimba nekutsvuka mukati memuviri. Chikonzero chikuru chechirwere ichi kuchinja kwemajini uko mwana anozvarwa aine . Kazhinji, zviratidzo zvakaita semapundu eganda, kurwadziwa kwemajoini, kana arthritis zvinotanga mwana asati asvitsa makore mashanu . Zvinogonawo kukonzera chirwere chinonzi uveitis, chinokanganisa kuona.

Zita rekuti "Blau Syndrome" rinorevei?

Paunonzwa zita iri, ungangoshamisika kuti chii ichi. Ngatitarisei zvinorehwa nemashoko maviri aya:

Blau: Iri ndiro zita rechiremba. Muna 1985, Dr. Edward Blau, aimbova chiremba wevana muWisconsin, akaburitsa bepa rekutsvagisa nezvechirwere ichi. Akatsanangura mhuri yakanga yatambura nechirwere ichi kwezvizvarwa zvina.
Syndrome: Mukurapa, chirwere chirwere umo zviratidzo zvakawanda zvine chekuita nazvo zvinosangana pamwe chete uye zvinokanganisa nhengo dzakasiyana dzemuviri. Kureva, musanganiswa wezviratidzo kwete chirwere chimwe chete.

Ndezvipi zviratidzo zveBlau Syndrome?

Zviratidzo zveBlau syndrome zvinowanzotanga paucheche . Kazhinji, zviratidzo izvi zvinoonekwa pazera remakore mashanu. Zvinonyanya kukanganisa ganda remwana wako, majoini, nemaziso .

Zviratidzo zveganda

Chiratidzo chekutanga cheBlau syndrome chirwere cheganda chinonzi granulomatous dermatitis. Iri mapundu anoonekwa paganda. Anowanzoonekwa pamaoko, makumbo, kana dzimwe nzvimbo dzemuviri, dzakadai sechipfuva nedumbu, mukati megore rekutanga rehupenyu hwemwana . Rudzi urwu rwedermatitis runogona kukonzera zviratidzo zvakaita se:

Mapundu akaomarara kana mapundu aunonzwa pasi peganda remwana wako . Aya anonzi magranulomas.
Ganda rinoita sekorari .
Maronda matsvuku, eyero, kana mashava pamusoro peganda remwana, epidermis.Mapundu anoonekwa.

Zviratidzo mumajoini

Blau syndrome inogona kukonzera kuzvimba kwemukati memajoini emwana wako, kunonzi synovium. Mwana wako anogona kubatwa nearthritis munzvimbo dzakadai semaoko, maoko, tsoka, uye zvitsitsinho pakati pemakore maviri nemana. Zviratidzo zvearthritis zvinosanganisira:

Kurwadziwa kwemajoini .
Kurwadziwa kwetsandanyama , kunyanya mumatendo.
Kuzvimba kana kuomarara kwemajoini .

Fungidzira kana mwana wako akachema paanomuka mangwanani, asingakwanise kufambisa makumbo ake, kana kuti kana achigara achinyunyuta kuti majoini ake anorwadza paanoenda kunotamba, unofanira kunetseka nazvo.

Zviratidzo zvemaziso

Vana vanosvika 80% vanoonekwa vaine chirwere cheBlau syndrome vanowana chirwere chemaziso chinonzi uveitis. Uveitis kuzvimba kwechikamu chepakati cheziso, chinonzi uvea. Izvi zvinogona kukanganisa retinas yemwana netsinga dzemaziso. Mwana anogona kuva ne uveitis mumaziso ese , uye zvinogonawo kukonzera kurasikirwa nekuona . Zviratidzo zve uveitis zvinosanganisira:

Kuona zvishoma.
Unoona madotsi madiki matema (maziso anoyangarara ) achifamba-famba pamberi pemaziso ako.
Unonzwa kurwadziwa kana kumanikidzwa mumaziso ako .
Kutsvuka kwemaziso .
Kunzwa kwechiedza (photosensitivity) , zvichireva kuti zvinova zvakaoma kuona chiedza.
Kuzvimba kwemaziso .

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Blau Syndrome inokanganisa sei dzimwe nhengo dzemuviri?

Kunyangwe izvi zvisingawanzoitiki, mwana wako ane Blau syndrome anogona kuva nezvirwere zvinogona kuisa upenyu pangozi munhengo idzi:

Mitsipa yeropa
Uropi
Mwoyo
Chiropa
Lymph nodes (system yelymphatic)
Chidendene

Ndezvipi zvinetso zvinogona kukonzera Blau Syndrome?

Kuzvimba kunokonzerwa neBlau syndrome kunogona kukonzera matambudziko akadai se:

Matumbu, glaucoma, cystoid macular edema, retinal detachment, uye kurasikirwa zvachose kwekuona.
Kuoma kwekufamba uye kugonya kwepfundo rakakanganiswa zvachose.
Chirwere cheitsvo uye kutadza kushanda zvakanaka kweitsvo .
Kuzvimba kwemoyo.
Chidende chakakura.
Neuropathy - matambudziko etsinga.
Kukwira kweropa mumapapu - kukwira kweropa mumapapu.
Vasculitis - kuzvimba kwetsinga dzeropa.

Chii chinokonzera Blau Syndrome?

Chikonzero chikuru cheBlau syndrome kuchinja kwejini reNOD2 . Muvanhu vazhinji vane hutano hwakanaka, jini iri reNOD2 rinogadzira puroteni inonzi NOD2. Puroteni iyi inobatsira masoja edu emuviri kurwisa utachiona nehutachiona. Zvisinei, kana mwana wako aine Blau syndrome, puroteni iyi yeNOD2 inova nesimba rakawandisa . Izvi zvinoshandura mashandiro anoita masoja emuviri, zvichikonzera kuzvimba kwakanyanya kunokanganisa maziso, ganda, nemapfundo emwana.

Ndiani ari panjodzi yekubatwa nechirwere cheBlau Syndrome?

Kana mumwe mubereki aine Blau syndrome (kana kuti gene mutation inoikonzera), mwana ane mukana we50% wekugara nhaka gene rakachinjwa uye kukura kwechirwere ichi . Mwana anofanira kugara nhaka rimwe remajini akachinjwa kuti awane chirwere ichi. Izvi zvinoreva kuti chirwere ichi chinobva muboka rinonzi autosomal dominant disorders. Dzimwe nguva, mwana anogona kugara nhaka iyi gene mutation asingazove neBlau syndrome. Zvisinei, mwana ane mukana we50% wekupa vana vake gene rakachinjwa mune ramangwana.

Vanachiremba verudzii vanoongorora uye vanorapa Blau Syndrome?

Zvichienderana nezviratidzo zvemwana wako, angangoda kurapwa nechikwata chenyanzvi, kusanganisira:

Chiremba wemapfupa (chiremba anonyanya kubata zvirwere zvemajoini) wearthritis nematambudziko ane chekuita nemajoini .
Chiremba weganda (nyanzvi yeganda) wezvirwere zveganda .
Chiremba wemaziso (nyanzvi yemaziso) wematambudziko ekuona .

Vanachiremba vanoongorora sei Blau Syndrome?

Kuongororwa kwechirwere cheBlau kunosiyana zvichienderana nezviratidzo zvemwana wako. Kuongororwa kwemajini (kuongororwa ropa) kunogona kuitwa kuti uone shanduko yemajini eNOD2 inokonzera Blau syndrome. Mwana wako anogonawo kuve nebvunzo imwe kana kupfuura dzinotevera:

Kuongororwa kwemaziso: Izvi zvinogona kusanganisira bvunzo dzakadai se optical coherence tomography (OCT) uye bvunzo dzekuona.
Kuongororwa kwemifananidzo : MRI scan, CT scan, ultrasound, kana X-rays yekutarisa majoini nedzimwe nhengo dzemuviri zvichienderana nezviratidzo.
Kuongororwa kweganda : Kutorwa kweganda diki kuti riongororwe.

Kuongororwa kwemwana asati aberekwa kunogona kuona Blau Syndrome here?

Kuongororwa kwemwana asati aberekwa kwakadai se chorionic villus sampling kana amniocentesis hakuedze zvakananga NOD2 gene mutation.

Ndeapi mamwe mazita eBlau Syndrome?

Chiremba wemwana wako anogonawo kudana Blau syndrome nerimwe remazita aya:

Arthritis yevana inonzi granulomatous arthritis
Granulomatosis yeganda reuvha rinobuda mumuromo
Granulomatosis yemhuri
Granulomatosis yevana vechidiki mumhuri
Arthritis ine granulomatous inflammatory, dermatitis uye uveitis

Blau Syndrome isingawanzoitiki sei?

Blau syndrome chirwere chisingawanzoitiki . Pasi rose, inobata vana vasingasviki mumwe pavana miriyoni .

Vanachiremba vanorapa sei Blau Syndrome?

Chikwata chevanachiremba chemwana wenyu chichaedza kurapa zvirwere zvakasiyana-siyana kuderedza zviratidzo uye kudzivirira kuputika kwechirwere. Nzira dzekurapa dzinosiyana zvichienderana nechirwere chacho uye kuoma kwacho. Dzinogona kusanganisira:

Mishonga inodzvinyirira muviri : Mishonga yakaita se corticosteroids, methotrexate, uye tumor necrosis factor (TNF) inhibitors.
Mishonga inorwisa kuzvimba : Mishonga yakaita semishonga isingarwisi kuzvimba (NSAIDs).
Mishonga yemaziso uye/kana kuvhiyiwa kwemaziso e cataract ne glaucoma .
Kurapa kwemuviri uye kurapwa kwebasa .

Ramangwana remunhu ane Blau Syndrome rakaita sei?

Kunyange hazvo pasina mushonga chaiwo weBlau syndrome, kurapwa kunogona kudzora zviratidzo uye kupa mwana wako hupenyu hwakanaka kusvika akura.Zvinogona kubatsira. Mabatiro anoita chirwere ichi munhu wese akasiyana. Imwe ongororo yakawana kuti 40% yevana vane Blau syndrome vaiva nezviratidzo zvishoma uye vaikwanisa kushanda sevamwe vana vezera ravo. Zvisinei, vanenge 10% yevana vanova nezviratidzo zvakakomba. Kana Blau syndrome ikakanganisa nhengo huru mumuviri, inogona kupfupisa makore ekurarama kwemunhu .

Blau Syndrome inogona kudzivirirwa here?

Kana iwe kana mumwe wako muine chirwere chekuchinja kwemajini chinokonzera Blau syndrome, zvakanaka kuti muone chiremba wezvemajini musati mava nevana. Nyanzvi iyi inogona kutaura nemi nezvenjodzi yekuti vana venyu vemangwana vagare nhaka jini reNOD2 rakachinjwa.

Ndinofanira kuona chiremba rini?

Enda kuna chiremba nekukasika kana mwana wako aine chimwe chezvinotevera:

Kana uchinetseka kubata zvinhu, kukotamisa majoini ako, kana kufamba-famba .
Kana paine kurwadziwa kwakanyanya .
Kana uine matambudziko ekuona .

Chii chandinofanira kubvunza chiremba wangu?

Unogona kubvunza chiremba wako mibvunzo yakaita seiyi:

Chii chinokonzera mwana wangu kuti aite Blau Syndrome?
Mishonga ipi uye mishonga inogona kubatsira mwana wangu?
Ini nemurume/mukadzi wangu tinofanira kuongororwa majini here?
Ndinofanira kungwarira zviratidzo zvematambudziko here?

Ndeupi musiyano uripo pakati peBlau Syndrome neEarly-Onset Sarcoidosis?

Blau syndrome uye early-onset sarcoidosis chirwere chimwe chete , chine zviratidzo zvakafanana. Zvisinei, vana vane Blau syndrome vanogara nhaka shanduko yemajini inokonzera chirwere ichi. Vana vane early-onset sarcoidosis havana nhoroondo yemhuri yeBlau syndrome. Izvi zvinoreva kuti gene reNOD2 rinochinja kana kuchinja nguva nenguva , pasina chikonzero chakajeka. Izvi zvinonzi de novo gene mutation.

Pakupedzisira, zvinhu zvekurangarira

Kuchengeta mwana ane chirwere chisingaperi chakadai seBlau Syndrome kunogona kuoma. Kana uine Blau Syndrome, unogona kushandisa ruzivo rwako pachako kuti utsigire mwana wako zviri nani. Unogonawo kushandisa ruzivo rwako kubatsira mwana wako kurarama nechirwere ichi chehupenyu hwake hwese. Chinhu chakakosha kutsvaga kurapwa kubva kuna nyanzvi inonyatsoziva nezvearthritis, uveitis, uye mamiriro eganda ane chekuita neBlau Syndrome. Vanogona kubatsira mwana wako kugadzirisa zviratidzo zvake uye kumubatsira kuti ave nehudiki hwakanaka. Kana ukaona chero zviratidzo, usazviregeredza. Enda kuna chiremba nekukasika.Blau Syndrome, Vana, Zvirwere zveGanda, Arthritis, Uveitis, Zvirwere zveMajini, NOD2 Gene

Mwana wenyu mudiki ane zviratidzo izvi here? Ngatikurukurei nezve Blau Syndrome.