I-ATTR Amyloidosis: Iimpawu, isifo, kunye nonyango

Umxholo

• Ushwankathelo
• Oonobangela
• Iindidi ze-ATTR amyloidosis
• Iimpawu
• Ukuxilongwa kwe-ATTR
• Unyango
• Imbonakalo

Ushwankathelo

I-Amyloidosis sisifo esinqabileyo esenzeka xa kukho iiproteyini ezakhayo emzimbeni. Ezi proteni zinokwakha kwimithambo yegazi, amathambo, kunye nakwizitho eziphambili, ezikhokelela kuluhlu olubanzi lweengxaki.

Le meko inzima ayinyangeki, kodwa inokulawulwa ngonyango. Ukufumanisa isifo kunye nonyango kunokuba ngumceli mngeni kuba iimpawu kunye nezizathu ziyahluka phakathi kweentlobo ezahlukeneyo ze-amyloidosis. Iimpawu zinokuthatha ixesha elide ukuzibonakalisa.

Funda ukuze ufunde ngolunye lweentlobo eziqhelekileyo: i-amyloid transthyretin (ATTR) i-amyloidosis.

Oonobangela

I-ATTR amyloidosis inxulumene nemveliso engaqhelekanga kunye nokwakhiwa kohlobo lwe-amyloid ebizwa ngokuba yi-transthyretin (TTR).

Umzimba wakho wenzelwe ukuba ube nesixa sendalo se-TTR, esenziwa ikakhulu sisibindi. Xa ingena kwigazi, i-TTR inceda ukuhambisa iihomoni ze-thyroid kunye nevithamini A kuwo wonke umzimba.

Olunye uhlobo lwe-TTR lwenziwa kwingqondo. Inoxanduva lokwenza ulwelo lwe-cerebrospinal.

Iindidi ze-ATTR amyloidosis

I-ATTR lolunye uhlobo lwe-amyloidosis, kodwa kukwakho nee-subtypes ze-ATTR.

Ilifa, okanye usapho i-ATTR (hATTR okanye i-ARRTm), ibaleka kwiintsapho. Kwelinye icala, ifunyenwe (ayisiyofuzo) i-ATTR yaziwa ngokuba “luhlobo lwasendle” ATTR (ATTRwt).

I-ATTRwt ihlala inxulunyaniswa nokwaluphala, kodwa akunjalo kwezinye izifo zemithambo-luvo.

Iimpawu

Iimpawu ze-ATTR ziyahluka, kodwa zinokubandakanya:

• ubuthathaka, ngakumbi kwimilenze yakho
• umlenze neqatha ukudumba
• ukudinwa kakhulu
• ukuphuthelwa
• ukubetha kwentliziyo
• ukuhla ukusinda
• iingxaki zamathumbu kunye nokuchama
• libido ephantsi
• isicaphucaphu
• Isifo seCarpal tunnel

Abantu abane-ATTR amyloidosis nabo bathambekele ngakumbi kwisifo sentliziyo, ngakumbi uhlobo lwe-ATTR yasendle. Unokuqaphela ezongezelelweyo iimpawu ezinxulumene nentliziyo, ezinje:

• iintlungu zesifuba
• ukubetha kwentliziyo okungaqhelekanga okanye okukhawulezayo
• isiyezi
• ukudumba
• ukuqhawukelwa ngumphefumlo

Ukuxilongwa kwe-ATTR

Ukuchonga i-ATTR kunokuba ngumceli mngeni ekuqaleni, ngakumbi kuba uninzi lweempawu zayo zifana nezinye izifo. Kodwa ukuba umntu kusapho lwakho unembali ye-ATTR amyloidosis, oku kuya kunceda ukuhambisa ugqirha wakho ukuba avavanye iintlobo ze-amyloidosis. Ukongeza kwiimpawu zakho kunye nembali yempilo yakho, ugqirha unokuyalela ukuvavanywa kofuzo.

Iindidi ze-ATTR zasendle kunokuba nzima ngakumbi ukuzifumanisa. Esinye isizathu kukuba iimpawu ziyafana nokusilela kwentliziyo.

Ukuba i-ATTR ikrokrelwa kwaye awunayo imbali yosapho yesi sifo, ugqirha wakho kuya kufuneka afumane ubukho bee-amyloid emzimbeni wakho.

Enye indlela yokwenza oku kukuskena kwenyukliya. Olu scan lujonga iidipozithi ze-TTR emathanjeni akho. Uvavanyo lwegazi lunokumisela ukuba ngaba kukho iidipozithi kwigazi. Enye indlela yokufumanisa olu hlobo lwe-ATTR kukuthatha isampulu encinci (i-biopsy) yethishu yentliziyo.

Unyango

Zimbini iinjongo zonyango lwe-ATTR amyloidosis: yeka ukuqhubela phambili kwesifo ngokunqanda iidipozithi ze-TTR, kunye nokunciphisa ifuthe i-ATTR elinalo emzimbeni wakho.

Kuba i-ATTR ikakhulu ichaphazela intliziyo, unyango lwesi sifo luye lujolise kule ndawo kuqala. Ugqirha wakho unokumisela i-diuretics ukunciphisa ukudumba, kunye nezicoci gazi.

Ngelixa iimpawu ze-ATTR zihlala zilinganisa ezo zesifo sentliziyo, abantu abanale meko abanakuthatha ngokulula amayeza enzelwe ukusilela kwentliziyo.

Oku kubandakanya i-calcium block blockers, i-beta-blockers, kunye ne-ACE inhibitors. Ngapha koko, la mayeza anokuba yingozi. Esi sesinye sezizathu zokuba kutheni ukuxilongwa ngokufanelekileyo kubalulekile kwasekuqaleni.

Ukufakelwa kwentliziyo kunokucetyiswa kwiimeko ezinzima ze-ATTRwt. Oku kunjalo ngakumbi ukuba unengozi enkulu yentliziyo.

Ngamatyala okuzalwa, ukufakelwa kwesibindi kunokunceda ukumisa ulwakhiwo lwe-TTR. Nangona kunjalo, oku kuluncedo kuphela kuvavanyo lwakwangoko. Ugqirha wakho unokujonga kwakhona unyango lwezofuzo.

Ngelixa lungekho unyango okanye unyango olulula, uninzi lwamayeza amatsha okwangoku kwizilingo zeklinikhi, kwaye inkqubela phambili kunyango ikufuphi. Thetha nogqirha wakho ukuze ubone ukuba ulingo lweklinikhi lulungile na kuwe.

Imbonakalo

Njengakwezinye iintlobo ze-amyloidosis, akukho lunyango lwe-ATTR. Unyango lunokunceda ukunciphisa ukukhula kwesifo, ngelixa ulawulo lweempawu lunokuphucula umgangatho wobomi.

I-hATTR i-amyloidosis ine-prognosis engcono xa ithelekiswa nezinye iintlobo ze-amyloidosis kuba iqhubeka kancinci.

Njengayo nayiphi na imeko yezempilo, kwangethuba kuvavanywa kwaye kufunyaniswe i-ATTR, kokukhona kungcono ukubonwa ngokubanzi. Abaphandi baqhubeka ngokufunda ngakumbi malunga nale meko, ke kwixa elizayo, kuya kubakho iziphumo ezingcono kuzo zombini ii-subtypes.