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Immunoglobulin J

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The dimeric IgA molecule The dimeric Iga molekul ! "#chain ! "#rantai $ L#chain $ L#rantai % J#chain % J#rantai & secretor' component & 'g mengeluarkan komponen

Ig A Ig J Immunoglobulin A ( IgA ) is an anti(od' pla'ing a critical role in mucosal immunit' Immunoglobulin A (Iga) adalah anti(odi 'ang (ermain peran penting dalam imunitas mucosal )ore IgA is produced in mucosal linings than all other t'pes of anti(od' com(ined* + ! , (et-een % and .g is secreted into the intestinal lumen each da' + $ , Lagi Iga diproduksi dalam mucosal linings dari semua /enis anti(odi ga(ungan* +!, di antara % dan .g adalah secreted ke dalam lumen usus setiap hari +$, "o-ever, sources are correct -hen the' indicate immunoglo(ulin 0 as the most common form of immunoglo(ulin in the human (od' 1amun, sum(er 'ang (enar ketika mereka menun/ukkan immunoglo(ulin 0 se(agai 'ang paling umum (erupa immunoglo(ulin dalam tu(uh manusia In its secretor' form, IgA is the main immunoglo(ulin found in mucous

secretions , including tears , saliva , colostrum , intestinal /uice , vaginal fluid and secretions from the prostate and respirator' epithelium 2g mengeluarkan dalam (entuk, Iga adalah utama immunoglo(ulin ditemukan di mucous secretions, termasuk air mata, air liur, kolostrum, /us usus, cairan vaginal dan secretions dari prostata dan respirator' epithelium It is also found in small amounts in (lood "al ini /uga ditemukan dalam /umlah kecil dalam darah 3ecause it is resistant to degradation (' en4'mes, secretor' IgA can survive in harsh environments such as the digestive and respirator' tracts, to provide protection against micro(es that multipl' in (od' secretions + % , 5arena tahan terhadap degradasi oleh en4'mes, 'g mengeluarkan Iga dapat hidup di lingkungan keras seperti pencernaan dan pernafasan tracts, untuk mem(erikan perlindungan terhadap mikro(a 'ang (er(iak di tu(uh secretions +%, IgA is a poor activator of the complement s'stem , and opsonises onl' -eakl' Iga adalah miskin activator 'ang melengkapi sistem, dan opsonises han'a sakitan Its heav' chains are of the t'pe 6 Its rantai (erat adalah dari /enis 6

Contents Isi
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! 7orms ! 7ormulir o ! ! IgA! vs IgA$ !,! IgA! vs IgA$ o ! $ 8erum vs secretor' IgA !,$ 8erum vs Iga 'g mengeluarkan $ IgA activit' $ Iga kegiatan % Transport % Transportasi & 9atholog' & 9atholog' . 8ee also . Lihat /uga : References : Referensi ; <=ternal links ; 9ranala luar

edit ! Forms "unting! Formulir


edit ! IgA# $s% IgA& "unting! IgA# $s IgA&
It e=ists in t-o isot'pes , IgA! (>?@) and IgA$ (!?@): Itu ada di dua isot'pes, IgA! (>?@) dan IgA$ (!?@):

IgA! is found in serum and made (' (one marro- 3 cells IgA! ditemukan dalam serum dan di(uat oleh sumsum tulang 3 sel In IgA$, the heav' and light chains are not linked -ith disulfide (ut -ith noncovalent (onds Dalam IgA$, 'ang (erat dan ringan rantai 'ang tidak terhu(ung dengan disulfide tetapi dengan noncovalent o(ligasi IgA$ is made (' 3 cells located in the mucosae and has (een found to secrete into colostrum , maternal milk, tears and saliva IgA$ di(uat oleh sel 'ang terletak di mucosae dan telah ditemukan rahasia men/adi kolostrum, susu i(u, air mata dan air liur

edit ! "erum $s% secretory IgA "unting! "erum $s yg mengeluarkan Iga


It is also possi(le to distinguish forms of IgA (ased upon their location # serum IgA vs secretor' IgA Adalah mungkin /uga untuk mem(edakan (entuk Iga (erdasarkan lokasi mereka # serum 'g mengeluarkan Iga Iga vs IgA is found in secretions in a specific form called secretory IgA' , pol'mers of $#& IgA monomers linked (' t-o additional chains Iga ditemukan di secretions dalam (entuk tertentu 'ang dise(ut yg mengeluarkan Iga ', 9olimer dari $#& Iga monomers dihu(ungkan oleh dua tam(ahan rantai Ane of these is the J chain (/oining chain), -hich is a pol'peptide of molecular mass !.kD, rich -ith c'steine and structurall' completel' different from other immunoglo(ulin chains 8alah satun'a adalah rantai J ((erga(ung rantai), 'ang merupakan pol'peptide dari massa molekular !.kD, ka'a dengan c'steine dan struktural (er(eda dari immunoglo(ulin rantai This chain is formed in the IgA# secreting cells Ini adalah mem(entuk rantai di Iga secreting#sel The oligomeric forms of IgA in the e=ternal (mucosal) secretions also contain a pol'peptide of a much larger molecular mass (;? kD) called the secretor' component that is produced (' epithelial cells The oligomeric (entuk Iga di luar (mucosal) secretions /uga (erisi pol'peptide /auh le(ih (esar dari massa molekular (;? 5D) dise(ut 'g mengeluarkan komponen 'ang diproduksi oleh sel epithelial This molecule originates from the pol'#Ig receptor (!%? kD) that is responsi(le for the uptake and transcellular transport of oligomeric ((ut not monomeric) IgA across the epithelial cells and into secretions such as tears, saliva, s-eat and gut fluid )olekul ini (erasal dari pol'#ig receptor (!%? 5D) 'ang (ertanggung /a-a( atas uptake dan transcellular transportasi dari oligomeric (namun tidak monomeric) Iga di seluruh epithelial cells secretions dan men/adi seperti air mata, air liur, keringat dan cairan usus

edit ! IgA acti$ity "unting! Iga kegiatan


The high prevalence of IgA in mucosal areas is a result of a cooperation (et-een plasma cells that produce pol'meric IgA (pIgA), and mucosal epithelial cells that e=press an immunoglo(ulin receptor called the pol'meric Ig receptor (pIgR) Tinggin'a prevalensi Iga mucosal di daerah merupakan hasil ker/asama antara plasma sel 'ang memproduksi di(uat dr polimer Iga (pIgA), dan mucosal epithelial sel 'ang men'atakan se(uah immunoglo(ulin receptor dise(ut di(uat dr polimer ig receptor (pIgR) pIgA is released from the near(' activated plasma cells and (inds to pIgR pIgA dilepaskan dari dekat diaktifkan plasma ke sel dan mengikat pIgR This results in transportation of IgA across mucosal epithelial cells and its cleavage from pIgR for release into e=ternal secretions + & , Ini hasil transportasi Iga di mucosal epithelial dan pem(elahan sel dari pIgR untuk rilis ke luar secretions +&, In the (lood, IgA interacts -ith an 7c receptor called 7c6RI (or BDC> ), -hich is e=pressed on immune effector cells, to initiate inflammator' reactions + & , Ligation of 7c6RI (' IgA containing immune comple=es causes anti(od'#dependent cell#mediated c'toto=icit' (ADBB), degranulation of eosinophils and (asophils , phagoc'tosis ('

monoc'tes , macrophages , neutrophils and eosinophils , and triggering of respirator' (urst activit' (' pol'morphonuclear leukoc'tes + & , Di dalam darah, Iga (erinteraksi dengan 7B receptor dise(ut 7c6RI (atau BDC>), 'ang din'atakan pada keke(alan effector sel, untuk melakukan ko(aran reaksi +&, Ligation dari 7c6RI oleh Iga (erisi keke(alan kompleks men'e(a(kan anti(odi 'ang (ergantung pada sel#mediated c'toto=icit' ( ADBB), degranulation dari eosinophils dan (asophils, phagoc'tosis oleh monoc'tes, macrophages, neutrophils dan eosinophils, dan memicu aktivitas dari respirator' (urst oleh pol'morphonuclear leukoc'tes +&,

edit ! 'ransport "unting! 'ransportasi


9ol'meric IgA (mainl' the secretor' dimer) is produced (' plasma cells in the lamina propria ad/acent to mucosal surfaces Di(uat dr polimer Iga (terutama 'g mengeluarkan dimer) dihasilkan oleh plasma sel di lapisan tipis propria (erse(elahan dengan mucosal permukaan It (inds to the pol'meric immunoglo(ulin receptor on the (asolateral surface of epithelial cells and is taken up into the cell via endoc'tosis It mengikat kepada di(uat dr polimer immunoglo(ulin receptor di (asolateral permukaan sel epithelial dan diam(il ke dalam sel melalui endoc'tosis The receptor#IgA comple= passes through the cellular compartments (efore (eing secreted on the luminal surface of the epithelial cells, still attached to the receptor 9roteol'sis of the receptor occurs and the dimeric IgA molecule, along -ith a portion of the receptor kno-n as the secretor' component , are free to diffuse throughout the lumen + . , In the gut, it can (ind to the mucus la'er on top of the epithelial cells to form a (arrier capa(le of neutrali4ing threats (efore the' reach the cells 2ang kompleks receptor#Iga mele-ati selular compartments se(elum secreted pada permukaan luminal dari sel epithelial, masih melekat pada receptor 9roteol'sis dari receptor ter/adi dan dimeric Iga molekul, (ersama dengan (agian receptor dikenal se(agai 'g mengeluarkan komponen, (e(as mem(aur di seluruh lumen +., Di dalam usus, dapat mengikat ke lapisan lendir di (agian atas epithelial sel untuk mem(entuk se(uah penghalang mampu neutrali4ing ancaman se(elum mencapai sel

edit ! (athology "unting! (athology


Decreased or a(sent IgA, termed selective IgA deficienc' , can (e a clinicall' significant immunodeficienc' 3erkurang atau tidak hadir Iga, diistilahkan selektif Iga kekurangan, dapat men/adi klinis signifikan Immunodeficienc' Neisseria gonorrhae (causes gonorrhea ) releases a protease -hich destro's IgA Neisseria gonorrhae (pen'e(a( gonorea) merilis se(uah protease 'ang merusak Iga IgA nephropath' is caused (' IgA deposits in the kidne's Iga nephropath' dise(a(kan oleh Iga deposito dalam gin/al It is not 'et kno-n -h' IgA deposits occur in this chronic disease 5arena (elum diketahui mengapa ter/adi di Iga deposito ini pen'akit kronis 8ome theories suggest it is an a(normalit' of immune s'stem that results in these deposits 3e(erapa teori itu adalah men'arankan kea(normalan dari sistem keke(alan 'ang hasil dalam deposito

edit ! "ee also "unting! )ihat *uga (era+atan


(rehospital Care (rehospital Care
9aramedics should recogni4e the presence of severe fluid loss and should treat patients -ith 8tevens#Johnson s'ndrome (8J8) as the' -ould patients -ith thermal (urns 9aramedis harus mengakui ke(eradaan cairan kerugian parah dan harus mera-at pasien dengan sindroma 8tevens#Johnson (8J8) karena mereka akan pasien dengan luka (akar panas

,mergency Department Care Departemen (era+atan darurat


)ost patients present earl' and prior to o(vious signs of hemod'namic compromise 5e(an'akan pasien 'ang hadir a-al dan se(elum /elas tanda#tanda hemod'namic kompromi The single most important role for the <D ph'sician is to detect 8J8DT<1 earl' and initiate the appropriate <D and inpatient management Tunggal 'ang paling penting untuk <D dokter adalah untuk mendeteksi 8J8 D 8epuluh dini dan melakukan 'ang sesuai <D Ra-at Inap dan mana/emen Eithdra-al of the suspected offending agent is criticall' important 9enarikan 'ang diduga (ersalah agent kritis penting Timing of -ithdra-al has (een linked to outcome Eaktu penarikan telah terhu(ung ke hasil

Bare in the <D must (e directed to fluid replacement and electrol'te correction 9era-atan di <D harus diarahkan untuk penggantian cairan dan koreksi elektrolit 8kin lesions are treated as (urns 5ulit luka dianggap se(agai luka (akar 9atients -ith 8J8DT<1 should (e treated -ith special attention to air-a' and hemod'namic sta(ilit', fluid status, -oundD(urn care, and pain control 9asien dengan 8J8 D 8epuluh harus dira-at dengan perhatian khusus untuk Air-a' hemod'namic dan sta(ilitas, status cairan, luka D luka (akar pera-atan, kontrol dan sakit Treatment is primaril' supportive and s'mptomatic 9era-atan terutama adalah mendukung dan merupakan ge/ala 8ome have advocated c'clophosphamide, plasmapheresis, hemodial'sis, and immunoglo(ulin Ada (e(erapa advocated c'clophosphamide, plasmapheresis, hemodial'sis, dan immunoglo(ulin )ost authorities (elieve that corticosteroids are contraindicated 9aling (er-enang perca'a (ah-a corticosteroids 'ang contraindicated o )anage oral lesions -ith mouth-ashes )engelola luka mulut dengan mulut o Topical anesthetics are useful in reducing pain and allo-ing the patient to take in fluids Topical anesthetics sangat (erguna dalam mengurangi rasa sakit dan memungkinkan pasien untuk mengam(il dalam cairan

Areas of denuded skin must (e covered -ith compresses of saline or 3uro- solution 3idang denuded kulit harus ditutup dengan compresses dari saline atau 3uro- solusi Fnderl'ing diseases and secondar' infections must (e identified and treated 2ang pen'akit infeksi dan sekunder harus diidentifikasi dan dio(ati Affending drugs must (e stopped 3ersalah o(at harus dihentikan The use of s'stemic steroids is controversial 9enggunaan sistemik steroids adalah kontroversial 8ome authors (elieve that the' are contraindicated 3e(erapa penulis 'akin (ah-a mereka contraindicated Treatment -ith s'stemic steroids has (een associated -ith an increased prevalence of complications 9era-atan dengan sistemik steroids telah dihu(ungkan dengan peningkatan prevalensi komplikasi Address tetanus proph'la=is Alamat pencegahan pen'akit tetanus
o

Consultations -onsultasi
Bonsultants ma' help esta(lish the diagnosis and direct inpatient care 5onsultan dapat mem(antu mem(uat diagnosis dan langsung Ra-at Inap pera-atan A dermatologist is the most likel' clinician to esta(lish the diagnosis, -ith or -ithout (iops' J dermatolog kemungkinan (esar pen'e(a(n'a adalah clinician untuk menentukan diagnosa, dengan atau tanpa (iopsi

8evere cases ma' reGuire the involvement of a (urn specialist or plastic surger' specialist 9arah kasus mungkin memerlukan keterli(atan ter(akar spesialis (edah plastik atau spesialis Internal medicine, critical care, or pediatrics consultants direct inpatient care Internal o(at#o(atan, pera-atan kritis, pediatri atau konsultan langsung Ra-at Inap pera-atan Aphthalmolog' consultation is mandator' for those -ith ocular involvement Aphthalmolog' konsultasi adalah -a/i( (agi orang#orang dengan mata keterli(atan Depending on organ s'stem involvement, consultations -ith a gastroenterologist, pulmonologist, and nephrologist ma' (e helpful Tergantung pada sistem organ keterli(atan, konsultasi dengan gastroenterologist, pulmonologist, dan nephrologist dapat mem(antu

.edication /bat
1o specific drug treatment e=ists for 8tevens#Johnson s'ndrome Tidak ada perlakuan khusus o(at untuk 8tevens#Johnson s'ndrome The choice of anti(iotic depends on the associated infection 9ilihan tergantung pada anti(iotik terkait infeksi The use of s'stemic corticosteroids is controversial 9enggunaan sistemik corticosteroids 'ang kontroversial The' are useful in high doses earl' in the reaction, (ut mor(idit' and mortalit' actuall' ma' increase in association -ith corticosteroid use )ereka (erguna dalam dosis tinggi di a-al reaksi, sifat mudah kena sakit dan kematian tetapi se(enarn'a dapat meningkatkan (erkaitan dengan corticosteroid digunakan

"uman intravenous immunoglo(ulin has (een descri(ed as (oth treatment and proph'la=is Darah manusia immunoglo(ulin dideskripsikan se(agai pengo(atan dan pencegahan pen'akit In the latter setting, "e(ert and 3ogle used IHI0 in a patient -ho under-ent cardiac catheteri4ation (ut -ho had & previous 8J8 episodes after intravenous contrast in/ection . Dalam pengaturan 'ang kedua, "e(ert dan hantu IHI0 digunakan dalam pasien 'ang mengalami pen'akit /antung 'ang telah catheteri4ation tetapi & episode se(elumn'a 8J8 setelah suntikan darah kontras .

'indak lan*ut
Further Inpatient Care "elan*utnya (era+atan 0a+at Inap

8aline compresses ma' (e applied to the e'elids, lips, and nose 8aline compresses mungkin diterapkan pada e'elids, (i(ir, dan hidung Bareful dail' inspection is necessar' to monitor for secondar' superinfections "ati harian inspeksi diperlukan untuk memonitor superinfections kedua 9roph'lactic s'stemic anti(iotics are not useful, especiall' in the current era of multiple#drug resistance 9enangkal sistemik anti(iotik tidak (ermanfaat, terutama di era sekarang ini (an'ak o(at#perla-anan Antimicro(ials are indicated in cases of urinar' tract or cutaneous infections, either of -hich ma' lead to (acteremia Antimicro(ials ditun/ukkan dalam kasus# kasus urinar' tract 'g (erhu(ung dgn kulit atau infeksi, (aik 'ang dapat mengaki(atkan (acteremia

Further /utpatient Care "elan*utnya (era+atan 0a+at Jalan

Although patients -ith er'thema multiforme minor ma' (e treated as outpatients -ith topical steroids, those -ith er'thema multiforme ma/or (ie, 8J8) must (e hospitali4ed Ealaupun pasien dengan er'thema multiforme kecil dapat diperlakukan se(agai outpatients hangat dengan steroids, mereka 'ang er'thema multiforme (esar ('aitu, 8J8) harus hospitali4ed Bases of er'thema multiforme minor must (e follo-ed closel' 5asus#kasus er'thema multiforme minor harus diikuti erat 8ome authors recommend dail' follo-#up 3e(erapa penulis merekomendasikan harian tindak lan/ut

'ransfer 'ransfer

9atients -ith 8J8 are often criticall' ill* therefore, the' must (e admitted to hospitals capa(le of delivering critical care 9asien dengan 8J8 sering sakit kritis, karena itu harus masuk rumah sakit kritis mampu mem(erikan pera-atan 8ome patients ma' reGuire the services of a (urn unit 3e(erapa pasien mungkin memerlukan pela'anan 'ang ter(akar unit

Transfer criteria -ould (e the same as for patients -ith thermal (urns Transfer kriteria akan sama seperti untuk pasien dengan luka (akar panas

Deterrence1(re$ention (enangkisan 1 (encegahan

9atients must avoid an' future e=posure to agent(s) implicated in the occurrence of 8J8 9asien harus menghindari hu(ungan ke depan agen (s) implicated dalam ter/adin'a 8J8 Recurrences are possi(le Recurrences adalah mungkin

Complications -omplikasi

Aphthalmologic # Borneal ulceration, anterior uveitis, panophthalmitis, (lindness Aphthalmologic # Borneal pem(orokan, anterior uveitis, panophthalmitis, ke(utaan 0astroenterologic # <sophageal strictures 0astroenterologic # <sophageal strictures 0enitourinar' # Renal tu(ular necrosis, renal failure, penile scarring, vaginal stenosis 0enitourinar' # (er(entuk ta(ung ke(ekuan gin/al, gagal gin/al, penile scarring, stenosis vaginal 9ulmonar' # Tracheo(ronchial shedding -ith resultant respirator' failure 9ulmonar' # Tracheo(ronchial peluruhan dengan hasil kegagalan pernafasan Butaneous # 8carring and cosmetic deformit', recurrences of infection through slo-#healing ulcerations 2g (erhu(ung dgn kulit # 8carring kelainan (entuk dan kosmetik, recurrences infeksi melalui#lam(at pen'em(uhan ulcerations

(rognosis (rognosa

Individual lesions t'picall' should heal -ithin !#$ -eeks, unless secondar' infection occurs )asing#masing (iasan'a harus men'em(uhkan luka dalam -aktu !#$ (ulan, kecuali ter/adi infeksi sekunder )ost patients recover -ithout seGuelae 8e(agian (esar pasien sem(uh tanpa seGuelae Development of serious seGuelae, such as respirator' failure, renal failure, and (lindness, determines prognosis in those affected 9engem(angan seGuelae serius, seperti kegagalan pernafasan, gagal gin/al, dan ke(utaan, prognosa dalam menentukan orang#orang terpengaruh Fp to !.@ of all patients -ith 8J8 die as a result of the condition "ingga !.@ dari semua pasien dengan 8J8 mati se(agai aki(at dari kondisi The 8BART<1 score looks at a num(er of varia(les and uses them to prognosticate risk factors for death in (oth 8J8 and T<1 8BART<1 skor 'ang terlihat di se/umlah varia(el dan menggunakan mereka untuk meramalkan faktor risiko untuk kematian pada kedua 8J8 dan sepuluh The varia(les include the follo-ing: Haria(el adalah se(agai (erikut: o Age I&? 'ears FsiaI &? tahun o )alignanc' 5eganasan o "eart rate I!$? Den'ut /antungI !$?

Initial percentage of epidermal detachment I!?@ A-al persentase (erhu(ung dgn kulit detasemenI !?@ o 3F1 level I!? mmolDL 3F1 tingkatI !? mmol D L o 8erum glucose level I!& mmolDL 8erum glucose tingkatI !& mmol D L o 3icar(onate level J$? mmolDL Tingkat (ikar(onat J$? mmol D L )ortalit' rates are as follo-s: Tingkat kematian adalah se(agai (erikut: o 8BART<1 ?#! I % $@ 8BART<1 ?#!I %,$@ o 8BART<1 $ I !$ !@ 8BART<1 $I !$,!@ o 8BART<1 % I %. %@ 8BART<1 %I %.,%@ o 8BART<1 & I .C %@ 8BART<1 &I .C,%@ o 8BART<1 . or more I >?@ 8BART<1 . atau le(ihI >?@
o

(atient ,ducation (endidikan pasien

7or e=cellent patient education resources, visit e)edicineKs 8kin, "air, and 1ails Benter 9endidikan 'ang sangat (aik untuk pasien terse(ut, kun/ungi e)edicine dari 5ulit, Ram(ut, dan 1ails 9usat Also, see e)edicineKs patient education article Life#Threatening 8kin Rashes 8elain itu, melihat dari pasien e)edicine pendidikan artikel#Life mengancam 5ulit Rashes

.iscellaneous .iscellaneous
.edicolegal (itfalls .edicolegal (itfalls

The gravit' of the diagnosis must (e recogni4ed 2ang (erat dari diagnosa harus diakui 3ecause patients -ith 8tevens#Johnson s'ndrome (8J8) -ho present earl' in the development of the disease ma' not 'et (e criticall' ill, the clinician ma' misdiagnose and discharge 5arena pasien dengan sindroma 8tevens#Johnson (8J8) 'ang hadir pada a-al perkem(angan pen'akit )ei (elum men/adi sakit kritis, 'ang clinician )ei misdiagnose dan keluarn'a 8J8 should (e considered in all patients -ith target lesions and mucous mem(rane involvement 8J8 harus dipertim(angkan pada semua pasien dengan target luka dan selaput lendir keterli(atan 9rovide close follo-#up and clear instructions Dekat mem(erikan tindak lan/ut dan petun/uk /elas Ehen discharging a patient home, clearl' document the degree (@) of skin involvement, the a(sence of mucous mem(rane lesions, and an' clinical signs of to=icit' 3ila pemakaian rumah pasien, /elas dokumen dera/at (@) dari kulit keterli(atan, tidak adan'a luka selaput lendir, dan ada tanda#tanda klinis racun

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