Adrenoleukodystrophy (ALD) Chii? Zviratidzo, Zvinokonzera, Kurapa

Adrenoleukodystrophy (ALD) chirwere chisingawanzo chiri chose chinokanganisa hurongwa hwemasumbu ne adrenal glands. Ichi chirwere chinogona kuwanikwa kubva kuna baba kana amai chinoitika kana muviri wako usingakwanisi kuputsa mafuta akati anodanwa kuti very long-chain fatty acids, zvichiita kuti aunganidzwe uye kukuvadza zvikamu zvikuru zvemuviri wako. Kunyange ALD ichikanganisa vanhu vakasiyana nenzira dzakasiyana, kunzwisisa chirwere ichi kunogona kukubatsira kuziva zviratidzo pakutanga uye kutsvaga nzira dzekurapa dzingaderedza kufambira mberi kwaro.

Adrenoleukodystrophy Chii?

Adrenoleukodystrophy inokonzerwa nekushanduka kwe gene rinonzi ABCD1, rinowanzo kubatsira muviri wako kugadzirisa marudzi akati mafuta. Kana gene iri risina kushanda zvakanaka, zvinhu zvine mafuta zvinounganidzwa mubongo, spinal cord, ne adrenal glands.

Chirwere ichi chinowana zita rayo kubva kunzvimbo dzachinokanganisa zvikuru. "Adreno" inoreva adrenal glands dziri pamusoro peitsvo dzako, "leuko" inoreva chena chinhu mubongo, uye "dystrophy" inotsanangura kukanganisika kunoitika nekufamba kwenguva.

ALD chirwere cheX-linked, zvinoreva kuti gene inokonzera iri pachikamu cheX chromosome. Iyi nzira yekuwanikwa inotsanangura kuti sei chirwere ichi chikanganisa varume zvakanyanya kupfuura vakadzi, sezvo varume vane X chromosome imwe chete nepo vakadzi vane mbiri.

Marudzi eAdrenoleukodystrophy Ndeapi?

ALD inoratidzwa mumaitiro akasiyana-siyana, imwe neimwe ine nguva yayo uye kukomba. Rudzi rwawakagona kuwana kazhinji zvinoenderana nezera rako apo zviratidzo zvinoonekwa kekutanga uye zvikamu zvemuviri wako zvinokanganisika zvikuru.

Childhood cerebral ALD ndiyo fomu inonyanya kukomba, kazhinji ichitanga pakati pemakore 4 ne 10. Iyi fomu inofambira mberi nekukurumidza uye inokanganisa chena chinhu mubongo, ichitungamira kumatambudziko makuru eneurological. Vakomana vane iyi fomu vangaratidzika kuva vane utano zvakanaka usati watanga zviratidzo.

Adrenomyeloneuropathy (AMN) kazhinji inoitika pakuva mukuru, kazhinji mumakore ako ekuma20 kana ekuma30. Iyi fomu inofambira mberi zvishoma nezvishoma uye inonyanya kukanganisa spinal cord ne peripheral nerves. Vanhu vakawanda vane AMN vanogona kuchengetedza hupenyu hunowanzoita zvakanaka kwemakore akawanda nekugadzirisa kwakakodzera.

Addison-only disease inokanganisa adrenal glands chete pasina kukanganisa hurongwa hwemasumbu. Vanhu vane iyi fomu vangave nekushomeka kwehomoni asi havaiti zviratidzo zve neurological zvinoonekwa mune mamwe marudzi.

Vamwe vanhu vanoramba vasina zviratidzo, kunyanya vakadzi, vangave vasina zviratidzo zvinoonekwa asi vanogona kupasa chirwere ichi kuvana vavo.

Zviratidzo zveAdrenoleukodystrophy Ndeapi?

Zviratidzo zveALD zvinogona kusiyana zvakanyanya zvichienderana nerudzi rwaunaro uye makore ako apo zvinoonekwa kekutanga. Kuziva izvi zviratidzo pakutanga kunogona kuita musiyano mukuru mukuwana kurapwa nekukurumidza nekutsigirwa.

Mu childhood cerebral ALD, ungatanga kuona kuchinja mumafambiro kana kuita kwakakosha kuchikoro. Izvi zviratidzo zvekutanga zvinogona kuva zvishoma uye zvinogona kusanganisira:

• Kunetseka kuisa pfungwa kuchikoro
• Kuchinja kunyora kana kugadzirisa
• Matambudziko ekutarisa kana kunzwa
• Kuwedzera kweh hyperactivity kana matambudziko emafambiro
• Kunetseka kunzwisisa kana kutevera mirairo

Sezvo childhood cerebral ALD ichifambira mberi, zviratidzo zvakakomba zve neurological zvinowanzoitika. Izvi zvinogona kusanganisira kuita zvekutambudzika, kunetseka kumira, kurasikirwa nekutaura, uye matambudziko ekufamba nekutonga.

Adult-onset AMN inowanzoitika nezviratidzo zvakasiyana zvinoitika zvishoma nezvishoma nekufamba kwenguva. Ungave uine:

• Kusimba nekushaya simba mumakumbo ako
• Kunetseka kufamba kana kuchengetedza kutonga
• Matambudziko ekudzora bhara kana matumbo
• Kusuruvara kana kupisa mumaoko nemakumbo
• Kushaya simba pabonde

Zviratidzo zve adrenal insufficiency zvinogona kuitika mune chero rudzi rweALD uye zvinogona kuva zviratidzo zvekutanga zvaunoona. Izvi zvinosanganisira kuneta kwenguva refu, kurasikirwa nehuremu, kuita rima reganda rako, kuderera kweropa, uye kuda zvinhu zvine munyu.

Vakadzi vanotakura gene yeALD vanogona kuwana zviratidzo zvishoma gare gare muhupenyu, kazhinji zvichienderana nekusimba kwemukumbo kana kuchinja kwemashure eneurological, kunyange vazhinji vachiramba vasina zviratidzo zvachose.

Zvinokonzera Adrenoleukodystrophy Ndeapi?

ALD inokonzerwa ne mutations mu ABCD1 gene, iyo inopa mirairo yekugadzira protein inobatsira kutakura fatty acids mune cellular structures inonzi peroxisomes. Kana gene iri risina kushanda zvakanaka, muviri wako haugone kuputsa very long-chain fatty acids zvakanaka.

Aya mafuta anounganidzwa mune dzakasiyana-siyana tishu mumuviri wako, kunyanya muchena chinhu mubongo ne adrenal glands. Funga nezvazvo sehurongwa hwekudzokorora hwakaputsika - zvinhu zvinoraswa zvinofanira kugadziriswa uye kubviswa panzvimbo pezvo zvinounganidzwa uye zvinokonzera kukuvara.

Chirwere ichi chinogona kuwanikwa kubva kuna baba kana amai muX-linked recessive pattern. Izvi zvinoreva kuti gene iri iri paX chromosome, uye sezvo varume vane X chromosome imwe chete, vanonyanya kukanganisika kana vakawana gene yakakanganisika.

Vakadzi vane X chromosomes mbiri, saka vanowanzotoda mutations mune zvese zvikamu kuti vakanganiswe zvakanyanya. Nekudaro, vakadzi vane imwe yakakanganisika copy vanotakura uye vanogona kuwana zviratidzo zvishoma gare gare muhupenyu nekuda kwechinhu chinonzi X-inactivation.

Kukomba uye rudzi rweALD zvinogona kusiyana kunyange mumhuri imwe chete, zvichiratidza kuti mamwe ma genetic kana environmental factors anogona kukanganisa kuti chirwere ichi chinofambira sei uye chinofambira mberi.

Rini Unoenda Kune Dokita neAdrenoleukodystrophy?

Unofanira kutsvaga kurapwa kana ukaona zviratidzo zve neurological kana zviratidzo zve adrenal insufficiency, kunyanya kana uine nhoroondo yemhuri yeALD. Kuongororwa pakutanga kunogona kubatsira kuziva chirwere ichi usati chafambira mberi zvakanyanya.

Kune vana, taura nachiremba wako kana ukaona kuchinja kwakashamisira mumafambiro, kudzikira kwekuita kwakakosha kuchikoro, matambudziko ekutarisa kana kunzwa, kana matambudziko ekugadzirisa. Izvi zviratidzo zvingaratidzika kusina hukama pakutanga, asi pamwe chete zvinogona kuratidza chirwere che neurological chiri pasi.

Vakuru vanofanira kuona chiremba nekuda kwekushaya simba kwemukumbo, kunetseka kufamba, matambudziko ekudzora bhara kana matumbo, kana zviratidzo zve adrenal insufficiency senge kuneta kwakashamisira nekurasikirwa nehuremu. Usamirire kuti zviratidzo zvive zvakakomba usati watanga kutsvaga rubatsiro.

Kana uri kuronga kuva nevana uye une nhoroondo yemhuri yeALD, genetic counseling inogona kukubatsira kunzwisisa njodzi dzako uye sarudzo. Kuongororwa kuripo kuti uone kana uine gene mutation.

Vakadzi vanozikanwa sevatakuri vanofanira kukurukura nezvekuongororwa nesarudzo dzavo dzehutano, kunyange kana vachinzwa vane utano zvakanaka, sezvo zviratidzo zvinogona kuitika gare gare muhupenyu.

Zvinokonzera Njodzi dzeAdrenoleukodystrophy Ndeapi?

Chinokonzera njodzi huru yekuwana ALD ndeyekuva nenhoroondo yemhuri yechirwere ichi, sezvo iri chirwere chinogona kuwanikwa kubva kuna baba kana amai. Kunzwisisa nhoroondo yekurapa yemhuri yako kunogona kubatsira kuziva njodzi dzinogona kuitika pakutanga.

Kuve murume kunowedzera njodzi yako yekuwana marudzi akakomba eALD nekuda kwenzira yekuwanikwa yeX-linked. Varume vanowana gene yakakanganisika vanozowana chirwere ichi, kunyange kukomba nenguva zvinogona kusiyana.

Kuva neamai vanotakura gene yeALD kunokupa njodzi, sezvo amai vanogona kupasa chirwere ichi kuvana vavo. Mwana woga woga wemai vanotakura ane mukana we50% wekuwana gene mutation.

Zera rinogona kukanganisa rudzi rweALD rwawakagona kuwana. Childhood cerebral ALD kazhinji inoonekwa pakati pemakore 4 ne 10, nepo AMN kazhinji inoonekwa pakuva mukuru. Nekudaro, aya maitiro haasi echokwadi, uye zviratidzo zvinogona kuonekwa kunze kweizvi zera.

Mamwe genetic variations asingawanzoitika anogona kukanganisa kuti ALD inokanganisa sei, kunyange vatsvakurudzi vachiri kudzidza izvi zvinhu. Zvinhu zvinokonzera chirwere hazvina kuzivikanwa zvakajeka, zvichiita kuti genetic inheritance ive chinokonzera njodzi huru chinozivikanwa.

Matambudziko Anogona Kuitika eAdrenoleukodystrophy Ndeapi?

ALD inogona kutungamira kumatambudziko akakomba anokanganisa masisitimu akawanda emuviri, kunyange matambudziko chaiwo aunogona kusangana nawo zvinoenderana nerudzi rweALD rwaunaro uye nekukurumidza kwainofambira mberi. Kunzwisisa izvi zvinogona kukubatsira kugadzirira kurapwa kwakakwana.

Matambudziko eneurological kazhinji ndiwo anonyanya kunetseka kweALD. Mu childhood cerebral ALD, izvi zvinogona kusanganisira:

• Kurasikirwa kwenguva refu kwehunhu hwepfungwa nekuyeuka
• Kuita zvekutambudzika izvo zvingave zvakaoma kudzora
• Kurasikirwa nekutarisa nekunzwa
• Kunetseka kumira, izvo zvinogona kutungamira kumatambudziko ekudya
• Kurasikirwa kwakakwana kwekuita kwemuviri nekufamba

Aya matambudziko akakomba anowanzoitika nekukurumidza mu childhood cerebral ALD, kazhinji pamwedzi kusvika kune makore mashoma, zvichiita kuti kurapwa pakutanga kuve kwakakosha.

Mu AMN, matambudziko anowanzoitika zvishoma nezvishoma asi anogona kukanganisa hupenyu hwako. Ungave uine kunetseka kwenguva refu kufamba, pakupedzisira uchida zvinhu zvekubatsira kufamba kana chigaro chemagurukota. Kushaya simba kwematumbo nembara kunogona kukanganisa mabasa ezuva nezuva uye kunoda kugadziriswa kwenguva refu.

Adrenal insufficiency chirwere chakakomba chinogona kuitika mune chero rudzi rweALD. Pasina kurapwa kwakakodzera kwehomoni, izvi zvinogona kutungamira kune adrenal crisis inotyisa, inoratidzwa nekuderera kwakanyanya kweropa, kushaya mvura, uye kukuvara.

Matambudziko epfungwa nesangano zvakakoshawo kufunga nezvazvo. Kugara uchinetseka nechirwere che neurological chinogona kutungamira kukuzvidya mwoyo, kunetseka, uye kusurukirwa kwevanhu nemihuri. Aya matambudziko emumoyo mhinduro dzakajairwa dzinoshamisa kutarisirwa nekutsigirwa.

Mu zviitiko zvisingawanzoitiki, vamwe vanhu vane ALD vanogona kuwana matambudziko akawedzerwa senge zviratidzo zvepfungwa kana inflammatory responses mubongo, kunyange izvi zvisingawanzoitiki kupfuura matambudziko makuru eneurological nemahomoni.

Adrenoleukodystrophy Inoziviswa Sei?

Kuziva ALD kazhinji kunotanga nekuziva zviratidzo uye kunzwisisa nhoroondo yemhuri yako, ichiteverwa nekuongororwa kwakakosha kweropa nekuongororwa kwemuviri. Chiremba wako achakutanga nenhoroondo yakakwana yekurapa nekuongororwa kwemuviri kuti aone basa re neurological uye atsvake zviratidzo zvematambudziko e adrenal.

Kuongororwa kwakakosha kwekutanga kunoyera very long-chain fatty acids muropa rako. Kuwanda kwezvinhu izvi kunoratidza ALD zvakasimba, sezvo muviri wako usingakwanisi kuzvipatsanura zvakanaka kana ABCD1 gene isina kushanda zvakanaka.

Genetic testing inogona kusimbisa kuongororwa nekutsvaga mutations mu ABCD1 gene. Kuongororwa uku kunokosha zvikuru kune nhengo dzemhuri dzinoshuvira kuziva kana vachitakura gene, kunyange kana vasingaone zviratidzo zvakare.

Brain MRI scans inobatsira madokota kuona kuchinja muchena chinhu chinoratidza ALD. Aya mafirimu anogona kuratidza huwandu hwekukanganisika kwemubongo uye kubatsira kuona rudzi rweALD rwawakagona kuwana. Nzira yekuchinja pa MRI inogonawo kubatsira kufanotaura kuti chirwere ichi chinofambira sei.

Adrenal function tests inotarisa mahomoni kuti aone kana adrenal glands dzako dziri kushanda zvakanaka. Aya maonero anogona kusanganisira kuyera cortisol, ACTH, nemamwe mahomoni anoratidza hutano hwe adrenal.

Kuongororwa kuwedzerwa kunogona kusanganisira nerve conduction studies kuti aone basa re peripheral nerve, kunyanya kana uine zviratidzo zveAMN. Chiremba wako anogonawo kukurudzira kuongororwa kwenguva dzose kuti atevedze kufambira mberi kwechirwere nekufamba kwenguva.

Kurapa kweAdrenoleukodystrophy Chii?

Kurapa kweALD kunotarisa pakugadzirisa zviratidzo, kuderedza kufambira mberi kana zvichibvira, uye kuchengetedza hupenyu hwakanaka. Kunyange pasina mushonga, nzira dzakati wandei dzinogona kukubatsira kurarama zvakanaka nechirwere ichi uye zvichida kuderedza kufambira mberi kwaro.

Hormone replacement therapy inokosha kana uine adrenal insufficiency. Kutora synthetic cortisol uye dzimwe nguva mamwe mahomoni kunogona kutsiva zvakanaka zvinhu zvisingagoni kugadzirwa ne adrenal glands. Kurapa uku kazhinji kunogara kwenguva refu asi kunogona kuvandudza simba rako, chido chekudya, uye hupenyu hwako hwese.

Kune childhood cerebral ALD, hematopoietic stem cell transplantation (bone marrow transplant) inogona kuva sarudzo mumakore ekutanga. Kurapa uku kunogona kumisa kufambira mberi kwechirwere chemubongo, kunyange zvine njodzi huru uye kunoda kuongororwa kwakanyanya kuti uone kana uri munhu akanaka wekurapa uku.

Lorenzo's oil, musanganiswa we fatty acids chaiyo, yaimbovimbiswa kubatsira neALD, asi tsvakurudzo yakaratidza mabhenefiti mashoma kune vanhu vazhinji. Vamwe madokota vangachiri kukurukura nezvazvo sesarudzo yekuwedzera, kunyange zvisiri kurapa kukuru.

Gene therapy iri kurapwa kunobuda kunoratidza tariro mune clinical trials. Nzira iyi inosanganisira kuisa kopi inoshanda ye ABCD1 gene mumasero ako, zvichida ichivabvumira kugadzirisa fatty acids zvakajairwa zvakare.

Kutsigira kurapa kunotamba basa rinokosha mukugadzirisa zviratidzo zveALD. Izvi zvinogona kusanganisira physical therapy kuchengetedza simba, occupational therapy kugadzirisa mabasa ezuva nezuva, speech therapy kumatambudziko ekukurukura, uye rutsigiro rwekudya kana kumira kuchiva kuoma.

Mishonga inogona kubatsira kugadzirisa zviratidzo zvakakosha senge kuita zvekutambudzika, kusimba kwemhasuru, kana kurwadziwa. Timu yako yekurapa ichaita newe kuti uwane musanganiswa wakakodzera wekurapa kwemamiriro ako chaiwo.

Nzira Yekugadzirisa Adrenoleukodystrophy Kumba?

Kugadzirisa ALD kumba kunosanganisira kugadzira nharaunda inotsigira inogadzirisa kune zvinodiwa zvinoshanduka ichichengetedza kuzvimiririra nekufara sezvinobvira. Nzira yako ichaenderana nezviratidzo zvaunonzwa uye kuti chirwere ichi chinofambira sei.

Kana uri kutora hormone replacement therapy ye adrenal insufficiency, kuenderera mberi kwakakosha. Tora mishonga yako sezvakarayirwa, uye gara uine emergency hydrocortisone newe. Dzidza kuziva zviratidzo zve adrenal crisis uye uzive kana uchifanira kushandisa mushonga wekukurumidza kana kutsvaga kurapwa nekukurumidza.

Kuita zvemuviri mukati mekumhanya kwako kunogona kubatsira kuchengetedza simba nekufamba. Shanda ne physical therapist kuti ugadzire chirongwa chekurovedza muviri chinokodzera mamiriro ako. Kunyange mabasa ari nyore senge kuwedzera kana kurovedza muviri mumvura zvinogona kubatsira.

Kugara uchichengeteka kumba kunova kwakakosha sezvo kufamba kuchichinja. Funga nezvekuisa grab bars mumabhafu, kubvisa zvinhu zvinogona kukonzera kukuvara, uye kuve nechiedza chakanaka mumba mako. Occupational therapists vanogona kukupa mazano chaiwo anogona kukubatsira kufamba nemabasa ezuva nezuva nyore nyore.

Rutsigiro rwekudya runogona kudiwa kana kumira kuchiva kuoma. Shanda ne dietitian kuti uve nechokwadi chekuti uri kudya zvakakwana, uye dzidza nezvekuchinja kwekuumbwa kana nzira dzakasiyana dzekudya kana zvichidiwa. Kugara uine mvura yakakwana kwakakosha zvikuru kana uine adrenal insufficiency.

Rutsigiro rwepfungwa rwakaenzana nekurapa kwemuviri. Bata nesangano rekutsigira, munhu kana online, kwaunogona kugovana zvakaitika nevamwe vanonzwisisa zvaunofamba nazvo. Usatya kutsvaga kurapwa kana uri kurwisana nezvinhu zvepfungwa zvekurarama neALD.

Chengeta rekodhi yakakwana yezviratidzo zvako, mishonga, uye chero kuchinja kwaunoona. Ruzivo urwu runobatsira timu yako yekurapa kuita sarudzo dzakachenjera nezve kurapwa kwako uye runogona kuva rinoshanda pakutevera kufambira mberi kwechirwere.

Ungagadzirira Sei Kune Appointmenti Yako Yechiremba?

Kugadzirira zvakanaka kune appointmenti yako yechiremba kunogona kukubatsira kuita zvakanyanya nguva yenyu pamwe chete uye kuve nechokwadi chekuti unowana ruzivo nekurapa kwaunoda. Tanga nekunyora mibvunzo yako yese nekunetseka usati wasvika, kuti usakanganwa chero chinhu chakakosha panguva yekusangana.

Chengeta diary yezviratidzo kwemvhiki dzinoverengeka usati wasvika, uchicherechedza chero kuchinja mumamiriro ako, zviratidzo zvitsva, kana kunetseka nezve kurapwa kwako kwazvino. Sanganisira ruzivo nezvekuti zviratidzo zvinoitika riini, zvakakomba sei, uye zvinobatsira kana kuwedzera zviratidzo.

Uyisa runyora rwakakwana remishonga yese yaunotora, kusanganisira zvikamu, zvinowedzerwa, nemishonga yekutengesa. Kana ukatora emergency hydrocortisone, ita shuwa kuti chiremba wako anoziva nezve chero zviitiko zvitsva zvawakashandisa.

Unganidza chero rekodhi dzekurapa dzakakosha, mhinduro dzekuongororwa, kana mashumo kubva kune vamwe vanopa kurapwa wawakaona kubva pakuona kwako kekupedzisira. Kana uine kuongororwa kwemuviri kana ropa rakaongororwa kune imwe nzvimbo, uyisa ma kopi kana uve nechokwadi chekuti chiremba wako anogona kuwana mhinduro.

Funga nezvekuuya nemhuri kana shamwari kune appointmenti, kunyanya kana uri kukurukura nezvesarudzo dzakaoma dzekurapa kana kana kuyeuka kana matambudziko ekutarisa zvichiita kuti zvive zvakaoma kuchengetedza ruzivo. Vanogona kukubatsira uye yeuka zvakakosha kubva mukukurukurirana.

Gadzirira mibvunzo chaiyo nezve mamiriro ako, senge kuti inogona kufambira sei, zviratidzo zvitsva zvekucherechedza, kana kuti kurapwa kwako kwazvino kuri kushanda sei. Usatya kukumbira kujeka kana usinganzwisisi chimwe chinhu chakatsanangurwa nechikurapa chako.

Chii Chinonyanya Kukosha Nezve Adrenoleukodystrophy?

Chinonyanya kukosha kunzwisisa nezve ALD ndechekuti kunyange iri chirwere chakakomba che genetic, kuongororwa pakutanga nekurapa kwakakodzera kunogona kukanganisa hupenyu hwako uye zvichida kuderedza kufambira mberi. Chiitiko chega chega chemunhu neALD chakasiyana, uye kuva nechirwere ichi hazvirevi kurasikirwa netariro yehupenyu hunoremekedzwa.

Adrenal insufficiency, iyo inogona kuitika nerudzi rweALD, inogona kurapwa zvachose ne hormone replacement therapy. Kugadzirisa chikamu ichi chechirwere zvinobudirira kunogona kukubatsira kunzwa zvakanaka uye kudzivirira matambudziko akakomba.

Kune mhuri dzakabatwa neALD, genetic counseling nekuongororwa kunogona kupa ruzivo rwakakosha kune kuronga mhuri nekuziva pakutanga mune mamwe nhengo dzemhuri. Kuziva nezve carrier status kunogona kubatsira vakadzi kuita sarudzo dzakachenjera nezvekuchengetedza hutano hwavo.

Tsvakurudzo mune kurapwa kwitsva kweALD inoramba ichifambira mberi, ne gene therapy ichiratidza tariro yakakosha mune clinical trials. Kugara uine hukama neALD treatment centers kunogona kukubatsira kuwana zvikuru zvichangobuda uye clinical trials.

Kuvaka network yakasimba yekutsigira, kusanganisira vanopa kurapwa vanonzwisisa ALD, mhuri inotsigira neshamwari, uye hukama nemamwe mhuri dzakabatwa nechirwere ichi, kunogona kuita musiyano mukuru mukukurira nematambudziko anounzwa neALD.

Mibvunzo Inowanzo Bvunzwa Nezve Adrenoleukodystrophy

Mubvunzo 1: Vakadzi vanogona kuwana zviratidzo zveALD here?

Hungu, vakadzi vanotakura gene yeALD vanogona kuwana zviratidzo, kunyange zviri zvishoma pane zvinoitika kune varume. Anenge 20% yevakadzi vanotakura vanowana zviratidzo, kazhinji zvichienderana nekusimba kwemukumbo kana kunetseka kufamba kunowanzoitika gare gare muhupenyu, kazhinji mushure memakore 40. Aya zviratidzo anowanzoenda zvishoma nezvishoma uye haawanzova akakomba sezvinetso zve neurological zvinoonekwa kune varume vane ALD.

Mubvunzo 2: Kune mushonga weALD here?

Parizvino, hapana mushonga weALD, asi kurapa kunogona kubatsira kugadzirisa zviratidzo uye zvichida kuderedza kufambira mberi. Bone marrow transplant inogona kumisa kufambira mberi kwechirwere chemubongo muvana vamwe vane childhood cerebral ALD, kunyange zvine njodzi huru. Gene therapy iri kuratidza tariro mune clinical trials uye inogona kuva sarudzo yakakosha yekurapa mune ramangwana.

Mubvunzo 3: ALD inosiyana sei nemamwe genetic neurological conditions?

ALD yakasiyana nekuti inokanganisa hurongwa hwemasumbu ne adrenal glands inogadzira mahomoni nekuda kwekushaya simba pakugadzirisa marudzi akati mafuta. Kusiyana nemamwe neurological conditions, chikamu che adrenal cheALD chinogona kurapwa zvachose ne hormone replacement therapy. Nzira yekuwanikwa yeX-linked inoitawo kuti ive yakasiyana nemamwe genetic neurological disorders.

Mubvunzo 4: Kuchinja kudya kunogona kubatsira neALD here?

Kunyangwe Lorenzo's oil yaimbovimbiswa kuva yakabatsira, tsvakurudzo yakaratidza mabhenefiti mashoma kune vanhu vazhinji vane ALD. Parizvino, hapana kudya kwakakosha kunoratidzwa kuti kunokanganisa kufambira mberi kweALD. Nekudaro, kuchengetedza kudya kwakanaka kwakakosha, kunyanya kana uine matambudziko ekumira kana uri kutora hormone replacement therapy.

Mubvunzo 5: Hupenyu hwekutarisa kune munhu ane ALD ndihupi?

Hupenyu hwekutarisa hunosiyana zvakanyanya zvichienderana nerudzi rweALD rwaunaro. Vanhu vane Addison-only disease kana AMN isingawanzoitika vanogona kuva nehupenyu hunowanzoita zvakanaka nekurapwa kwakakodzera. Avo vane AMN inofambira mberi zvishoma nezvishoma vanogona kuva nehupenyu hwakaderera asi vanogona kurarama kwemakore mazhinji nehupenyu hwakanaka. Childhood cerebral ALD inowanzoenda nekukurumidza, kunyange kurapwa pakutanga ne bone marrow transplant kunogona kuvandudza zvikuru mhinduro.