Antiphospholipid syndrome - APS

Antiphospholipid Syndrome (APS) waa cillad isdifaac ku dhacda oo ku lug leh xinjirowga dhiigga ee joogtada ah (thromboses).Markaad xaaladan leedahay, nidaamkaaga difaaca jirka ayaa sameeya borotiino aan caadi ahayn oo weerara unugyada dhiigga iyo xuubka marinnada dhiigga. Joogitaanka unugyadan difaaca jirka waxay sababi karaan dhibaatooyin xagga socodka dhiigga ah waxayna u horseedi karaan xinjiro halis ah xididdada dhiigga ee jirka oo dhan.

Sababta dhabta ah ee APS lama oga. Labadaba hiddo-wadaha qaarkood iyo arrimo kale (sida infekshan) ayaa laga yaabaa inay dhibaatada u koraan.

Badanaa waxaa laga helaa dadka qaba cudurada kale ee isdifaaca jirka, sida systemic lupus erythematosus (SLE). Xaaladda ayaa ah dumarka ugu badan marka loo eego ragga, Inta badan waxaa laga helaa haweenka taariikh u leh soo dhicinta soo noqnoqda.

Dadka qaar waxay wataan unugyada difaaca jirka ee aan kor kusoo xusnay, laakiin malaha APS. Waxyaabaha kiciya qaarkood waxay sababi karaan inay dadkan yeeshaan xinjirowga dhiigga, oo ay ku jiraan:

• Sigaar cabid
• Nasasho sariir dheer
• Uurka
• Daaweynta hormoonka ama kaniiniyada xakameynta dhalmada
• Kansarka
• Cudurka kilyaha

Ma yeelan kartid wax calaamado ah, in kasta oo aad leedahay unugyada difaaca jirka. Astaamaha dhici kara waxaa ka mid ah:

• Xinjirowga dhiigga ee lugaha, gacmaha ama sambabaha. Xinjirowgu wuxuu ku jiri karaa xididdada dhiigga ama xididdada dhiigga.
• Dhicitaan soo noqnoqda ama wali dhalasho.
• Rash, dadka qaarkood.

Marar dhif ah, xinjirowga ayaa si lama filaan ah ugu soo baxa halbowlayaasha badan muddo maalmo ah. Tan waxaa lagu magacaabaa catastrophic anti-phospholipid syndrome (CAPS). Waxay u horseedi kartaa istaroog iyo xinjirooyinka kelyaha, beerka, iyo xubnaha kale ee jirka oo dhan, iyo qanjirka 'gangrene' ee addimada.

Tijaabooyinka lupus anticoagulant iyo antiphospholipid antibodies ayaa la samayn karaa markii:

• Xinjirow dhiig oo lama filaan ah ayaa ku dhaca, sida dhalinyarada ama kuwa aan lahayn waxyaabo kale oo qatar u ah xinjirowga dhiigga.
• Haweeney taariikh ayey leedahay soo noqnoqoshada uurka.

Tijaabooyinka lupus ee xinjirowga xinjirowga waa baaritaan dhiig xinjirowga. Unugyada difaaca jirka ee antiphospholipid (aPL) waxay sababaan in baaritaanka uusan caadi ku aheyn shaybaarka.

Noocyada baaritaanka xinjirowga waxaa ka mid noqon kara:

• Waqtiga tromboplastin qayb ahaan firfircoon (aPTT)
• Xilliga sunta jilicsan ee Russell
• Tijaabada 'Thromboplastin inhibition'

Tijaabooyin ku saabsan ka hortagga jirka antiphospholipid (aPL) ayaa sidoo kale la sameyn doonaa. Waxaa ka mid ah:

• Tijaabooyinka antibody ee Anticardiolipin
• Kahortagayaasha beta-2-glypoprotein I (Beta2-GPI)

Bixiyaha xanaanada caafimaadkaaga ayaa baari doona antiphospholipid antibody syndrome (APS) hadii aad leedahay baaris togan oo loogu talagalay aPL ama lupus anticoagulant, iyo mid ama in kabadan oo kamid ah dhacdooyinka soosocda:

• Xinjirowga dhiigga
• Dhicis soo noqnoqoshada

Tijaabooyinka wanaagsan waxay u baahan yihiin in la xaqiijiyo 12 toddobaad kadib. Haddii lagaa helo baadhitaan hufan oo aan lahayn astaamo kale oo cudurka ah, laguma ogaan doono cudurka APS.

Daaweynta APS waxaa loogu talagalay ka hortagga dhibaatooyinka ka yimaada xinjirowga dhiigga cusub ee sameysmaya ama xinjirooyinka jira ee sii weynaanaya. Waxaad u baahan doontaa inaad qaadato nooc ka mid ah dawooyinka dhiigga khafiifiya. Haddii aad sidoo kale qabtid cudur isdifaaca jirka, sida lupus, waxaad u baahan doontaa inaad xaaladaas xakameyso sidoo kale.

Daaweynta saxda ah waxay ku xirnaan doontaa sida daran ee xaaladaadu tahay iyo dhibaatooyinka uu keenayo.

ANTIPHOSPHOLIPID XANUUNKA ASTAANTA (APS)

Guud ahaan, waxaad u baahan doontaa daaweyn dhiig khafiifiya muddo dheer haddii aad leedahay APS. Daaweynta bilowga ah waxay noqon kartaa heparin. Daawooyinkaan waxaa lagu bixiyaa cirbad.

Xaaladaha badankood, warfarin (Coumadin), oo afka laga siiyo, ayaa markaa la bilaabaa. Waa lagama maarmaan in si joogta ah loola socdo heerka xinjirowga xinjirowga. Tan waxaa badanaa lagu sameeyaa iyadoo la adeegsanayo baaritaanka INR.

Haddii aad leedahay APS oo aad uur yeelato, waxaad u baahan doontaa inuu si dhow kuu raaco bixiye khabiir ku ah xaaladdan. Ma qaadan doontid warfarin inta aad uurka leedahay, laakiin waxaa lagaa siin doonaa tallaallo heparin ah.

Haddii aad leedahay SLE iyo APS, adeeg bixiyahaagu sidoo kale wuxuu kugula talin doonaa inaad qaadato hydroxychloroquine.

Xilligan, noocyada kale ee dawooyinka khafiifiya dhiigga laguma talinayo.

CATASTROPHIC ANTIPHOSPHOLIPID XANUUNKA (CAPS)

Daaweynta CAPS oo ku lug leh iskudhafka daaweynta xinjirowga xinjirowga, qiyaaso badan oo corticosteroids ah, iyo isdhaafsiga plasma ayaa waxtar u leh dadka badankood. Mararka qaarkood IVIG, rituximab ama eculizumab ayaa sidoo kale loo isticmaalaa kiisaska daran.

IMTIXAANKA FUDUD EE LUPUS ANTICOAGULANT AMA APL

Uma baahnid daaweyn haddii aadan lahayn astaamo, uur lumis, ama haddii aadan waligaa dhiig xinjirow qabin.

Qaad tallaabooyinka soo socda si aad uga hortagto xinjirowga dhiigga inuu sameeyo:

• Ka fogow inta badan kiniinka ka hortagga uurka ama daaweynta hoormoonka dhalmo-dayska (haweenka).
• SIGAAR ha cabin ama ha u isticmaalin waxyaabaha kale ee tubaakada.
• Kac oo soco inta lagu guda jiro duulimaadyada dhaadheer ee diyaaradda ama waqtiyo kale oo ay tahay inaad fadhiisato ama aad jiifsatid muddo dheer.
• Qoobabkaaga kor iyo hoos u dhaqaaji markii aadan dhaqaaqi karin.

Waxaa laguu qori doonaa daawooyinka khafiifiya dhiiga (sida heparin iyo warfarin) si looga hortago xinjirowga dhiigga:

• Qalliinka kadib
• Jab jab lafta kadib
• Oo leh kansar firfircoon
• Markaad u baahan tahay inaad fadhiisato ama jiifsato waqti dheer, sida intaad isbitaalka ku jirto ama aad guriga ku soo kabanayso

Waxa kale oo laga yaabaa inaad u baahato inaad qaadatid waxyaabaha khafiifiyeyaasha dhiigga 3 ilaa 4 toddobaad qalliinka kadib si loo yareeyo halista xinjirowga dhiiggaaga.

Daaweyn la'aan, dadka qaba APS waxay yeelan doonaan xinjirowga soo noqnoqda. Waqtiga intiisa badan, natiijada waxay kufiican tahay daaweynta saxda ah, oo ay kujirto daaweynta xinjirowga xinjirowga dheer ee muddada dheer. Dadka qaar ayaa laga yaabaa inay yeeshaan xinjiro dhiig oo ay adag tahay in la xakameeyo inkasta oo daaweyntu ay jirto. Tani waxay u horseedi kartaa CAPS, oo nafta halis gelin kara.

Wac bixiyahaaga haddii aad dareento astaamaha xinjirowga dhiigga, sida:

• Barar ama casaan lugta ah
• Neefta oo kugu yaraata
• Xanuun, kabuubyo, iyo midabka midabka cirro leh ee gacanta ama lugta

Sidoo kale la hadal daryeel bixiyahaaga haddii aad lumiso uur soo noqnoqda (dhicis dhicis).

Unugyada difaaca jirka ee Anticardiolipin; Hughes syndrome

• Nidaamka lupus erythematosus finanka wajiga
• Xinjirowga dhiigga

Amigo MC, Khamashta MA. Cudurka 'Antiphospholipid Syndrome': cudurada keena, ogaanshaha, iyo maaraynta. Gudaha: Hochberg MC, Gravallese EM, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatology. 7aad ed. Philadelphia, PA: Elsevier; 2019: cutubka 148.

Cervera R, Rodríguez-Pintó I, Colafrancesco S, iyo al. Shirweynaha 14aad ee Caalamiga ah ee 'Antiphospholipid Antibodies Antibodies Task Force' ayaa warbixin ka diyaariyay cilladda masiibada antiphospholipid syndrome. Autoimmun Rev. 2014; 13 (7): 699-707. PMID: 24657970 www.ncbi.nlm.nih.gov/pubmed/24657970.

Dufrost V, Risse J, Wahl D, Zuily S. Daawada xinjirowga lidka ku ah ee tooska ah ee loo isticmaalo antiphospholipid syndrome: Daawooyinkaani ma yihiin kuwo wax ku ool ah oo badbaado u leh warfarin? Dib-u-eegis nidaamsan ee suugaanta: jawaab celinta faallooyinka. Curr Rheumatol Rep. 2017; 19 (8): 52. PMID: 28741234 www.ncbi.nlm.nih.gov/pubmed/28741234.

Erkan D, Salmon JE, Lockshin MD. Cudurka anti-phospholipid syndrome. Gudaha: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. Kelley iyo Firestein's Buugga Buugga Rheumatology. 10aad ed. Philadelphia, PA: Elsevier; 2017: cutubka 82.

Websaydhka Machadka Wadnaha, Sambabka, iyo Dhiigga. Antiphospholipid antibody syndrome. www.nhlbi.nih.gov/health-topics/antiphospholipid-antibody-syndrome. La helay Juun 5, 2019.