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Referat Kelasi Besi (Lucky Bintang Kharismawati)

Mar 01, 2018

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    Referat

    MENGENAL KELASI BESI PADA TALASEMIA

    Oleh :

    Lucky Bintang Kharismaati! S"Ke#

    NIM" I$A%$%%&'

    Pem(im(ing :

    #r" )ulan#ei Marhaeni! S*" A

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    BAGIAN+SM, ILM- KESE.ATAN ANAK

    ,K -NLAM / 0S-D -LIN

    BAN1A0MASIN

    Agustus! &%$2

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    DA,TA0 ISI

    .alaman

    HALAMAN JUDUL................................................................................. i

    DAFTAR ISI.............................................................................................. ii

    DAFTAR GAMBAR................................................................................. iii

    DAFTAR TABEL...................................................................................... iv

    DAFTAR SINGKATAN............................................................................ v

    BAB I PENDAHULUAN.................................................................. 1

    BAB II TINJAUAN PUSTAKA

    A. Talasemia...................................................................................... 3

    B. Dampak Kelei!a" Bea" Besi Akia# T$a"%&si Da$a!............... '

    (. Te$api Kelasi Besi........................................................................ 13

    BAB III PENUTUP

    A" Kesimp&la".................................................................................. )*

    DAFTAR PUSTAKA

    2

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    LAMPIRAN

    3

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    BAB I

    PENDA.-L-AN

    Talasemia me$&paka" sala! sa#& pe"+aki# ,e"e#ik #e$a"+ak -i -&"ia.

    Pemaa si%a# #alasemia/0 -a" #alasemia/ me"2apai 1456 p7p&lasi -&"ia

    se-a",ka" pemaa si%a# !em7,l7i" E seki#a$ 89*6.1Pemaa si%a# #alasemia/

    0 -i I"-7"esia -i#em&ka" lei! #i",i +ai#& 36/186 pemaa si%a# #alasemia/

    )4/116 -a" pemaa si%a# !em7,l7i" E 1*/336. Di P&sa# Talasemia Jaka$#a

    pa-a ak!i$ &la" Ma$e# )885 #e$2a#a# 1)4: pasie" -e",a" '8/188 pasie" a$&

    se#iap #a!&". Kas&s #alasemia/ me$&paka" kas&s +a", #e$a"+ak -i-apa#ka" +ai#&

    *846 #alasemia 0/HE :456 -a" #alasemia/ ))6.)

    Pasie" #alasemia aka" se"a"#iasa me",alami a"emia akia# ,a",,&a"

    p$7-&ksi !em7,l7i" se!i",,a meme$l&ka" #$a"%&si -a$a!. T$a"s%&si -a$a!

    me$&paka" #a#a laksa"a s&p7$#i% ama +a", e$#&;&a" mempe$#a!a"ka" ka-a$

    !em7,l7i" 9/18 ,

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    e"-7k$i" +a", p$7,$esi% e$akia# #im&l"+a %i$7sis !a#i si$7sis !a#i ,a,al

    ;a"#&", -iae#es melli#&s !ip7,7"a-isme !ip7#i$7i-isme !ip7pa$a#i$7i-ime

    !i",,a kema#ia". U"#&k me"2e,a! #e$;a-i"+a k7mplikasi akia# kelei!a" esi i"i

    -ipe$l&ka" #e$api kelasi esi. T&;&a" ama #e$api kelasi esi a-ala! me"2apai

    ka-a$ esi #&&! +a", ama". Te$api kelasi esi +a", -ike"al saa# i"i -apa#

    -ie$ika" se2a$a pa$e"#e$al >De%e$7ksami"? -a" pe$7$al >De%e$ip$7" -a"

    De%e$asi$7@?.*4

    Meli!a# #e$;a-i"+a pe"i",ka#a" kas&s #alasemia pa-a a"ak -a" pe"#i","+a

    #e$api kelasi esi maka pa$a kli"isi pe$l& me",e#a!&i ;e"is/;e"is #e$api kelasi esi

    &"#&k me",e#a!&i ke&"#&",a" sekali,&s e%ek sampi","+a se!i",,a -apa# memili!

    -a" meme$ika" pe"a",a"a" +a", #e$aik.

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    BAB II

    TIN1A-AN P-STAKA

    A" Talasemia

    $" De3inisi

    Talasemia me$&paka" kelai"a" m7"7,e"ik +a", pali", se$i", -i#em&ka"

    -a" me$&paka" s&a#& kel7mp7k kelai"a" aaa" si"#esis !em7,l7i" -e",a"

    ka$ak#e$is#ik pe"&$&"a" p$7-&ksi sa#& a#a& lei! $a"#ai ,l7i" pa-a !em7,l7i"

    -easa. Talasemia -apa# -ikel7mp7kka" ses&ai -e",a" $a"#ai ,l7i" +a", #i-ak

    #e$e"#&k se2a$a e%ek#i% sea,ai #alasemia al%a >? e#a >0? -el#a e#a >0? -a"

    ,amma -el#a e#a >0?. Nam&" se2a$a kesel&$&!a" #alasemia al%a -a" e#a

    a-ala! +a", pali", pe"#i",. Sea,ia" esa$ #ipe #alasemia +a", pe"#i",

    -ia$iska" -e",a" 2a$a Me"-elia" $esesi% 7$a",#&a pemaa si%a# #a"pa ,e;ala

    memp&"+ai sa#& -ia"#a$a empa# kesempa#a" memp&"+ai a"ak -e",a" kelai"a"

    e$a#.45

    &" E*i#emi4l4gi

    Talasemia -i-is#$i&sika" se2a$a l&as pa-a -ae$a! Me-i#e$a"ia Tim&$

    Te",a! s&k7"#i"e" I"-ia -a" Asia Te",,a$a m&lai -a$i (i"a sela#a" melea#i

    seme"a";&", Mala+a sampai ke I"-7"esia >Gama$ ).1?. Pa-a a"+ak "e,a$a i"i

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    %$ek&e"si ,e" &"#&k #alasemia +a", e$e-a -a" va$iasi/va$iasi s#$&k#&$

    !em7,l7i" sa",a# #i",,i. Sei$i", -e",a" pe$aika" k7"-isi s7sial pa-a "e,a$a/

    "e,a$a e$kema", -a" pe"&$&"a" m7$#ali#as 7le! ka$e"a i"%eksi -a" mal"$isi

    a"ak/a"ak -e",a" #alasemia +a", seel&m"+a aka" me"i",,al m&-a seka$a",

    e$#a!a" 2&k&p lama -a" meme$l&ka" pe$aa#a". Pa-a -ae$a!/-ae$a! i"i ;&,a

    -i#em&ka" pe"+ea -a$i #i",,i"+a %$ek&e"si #alasemia +ai#& m&"2&l sea,ai

    sek&ele -a$i i"%eksi mala$ia %alsipa$&m.1

    Gam(ar &"$ Peta *enye(aran talasemia #i #unia! *erhatikan (aha

    *enye(aran ini nam*aknya sesuai #engan *enye(aran

    malaria 3alsi*arum" $

    5" Genetik talasemia

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    Da$a! ma"&sia -easa me$&paka" 2amp&$a" -a$i ee$apa #ipe

    !em7,l7i". Hem7,l7i" +a", #e$a"+ak >HA? #e$-i$i -a$i -&a $a"#ai al%a -a"

    e#a $a"#ai/$a"#ai i"i -ik7-eka" 7le! : l7k&s ,e" al%a -a" ) e#a.

    Hem7,l7i"7pa#i me$&paka" kelai"a" as7mal $esesi% -a$i ,e"/,e" i"i. Lei!

    -a$i 488 va$iasi +a", e$e-a #ela! -i,ama$ka" -a" me$eka -apa# mempe",a$&!i

    aik s#$&k#&$ H >kelai"a" va$ia" sepe$#i sel sai#? a#a& me",&$a",i k&a"#i#as

    $a"#ai e#a a#a& al%a !em7,l7i" >#alasemia?.1*

    Talasemia -i"amaka" ses&ai -e",a" $a"#ai +a", me",alami -e%isie"si

    +ak"i al%a a#a& e#a. Kelai"a" $a"#ai al%a -iseaka" 7le! ka$e"a -elesi ,e"

    se-a",ka" $a"#ai e#a -iseaka" 7le! alel "7"/-elesi7"al -a" s&-a! -i#em&ka"

    lei! -a$i 188 kelai"a". Se2a$a kli"is kea-aa" +a", e$a# #im&l ila #e$;a-i

    kelai"a" pa-a ke-&a ,e" a#a& pa-a 3 sampai : $a"#ai al%a. Da$a! ma"&sia -easa

    &m&m"+a me",a"-&", )46 HA) >))? +a", me$&paka" !em7,l7i" $esi-&al

    -a" ;&,a se;&mla! H %e#al >HF ))? pa-a #i,a &la" pe$#ama ke!i-&pa".

    Ke-&a !em7,l7i" i"i #i-ak me",a"-&", $a"#ai e#a se!i",,a kee$a-aa"

    me$eka pa-a &sia +a", lei! #&a -apa# mema"#& -ia,"7sis a-a"+a

    !em7,l7i"7pa#i.*

    Pe$e-aa" !a$&s -i&a# a"#a$a pemaa si%a# >!a"+a memiliki se&a!

    l7k&s ,l7i" +a", #e$ke"a -a" #e$&s se!a# selama !i-&p "am&" memp&"+ai $isik7

    me"&$&"ka" pe"+aki# ke a"ak/a"ak"+a? -a" 7$a",/7$a", -e",a" kelai"a"

    !7m7=i,7# a#a& !e#e$7=i,7# ,a"-a se!i",,a me",alami kelai"a". Kelai"a"/

    kelai"a" i"i -i#&$&"ka" se2a$a $esesi% me"&$ ,e"e#ika Me"-elia" 7$a", #&a

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    memp&"+ai sa#& -ia"#a$a empa# kesempa#a" &"#&k mela!i$ka" a"ak pe"-e$i#a

    !em7%ilia apaila me$eka e$-&a me$&paka" pemaa si%a# >Gama$ ).)?.5'9

    Gam(ar &"&"

    Pearisan si3at talasemia mengikuti *4la *earisan si3at

    Men#elian! sehingga 4rang tua *em(aa si3at akanmem*unyai

    kesem*atan meariskan satu #ari em*at keturunannya"

    2" Mani3estasi Klinis #an Diagn4sis Talasemia

    a" Talasemia Beta

    Talasemia e#a !e#e$7=i,7# me$&paka" kelai"a" asimp#7ma#ik -e",a"

    ,ama$a" a"emia !ip7k$7m mik$7si#ik >M(H -a" M(C $e"-a!? se$#a memiliki

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    ka-a$ HA) -&a kali "7$mal. Talasemia e#a !7m7=i,7# a#a& me$eka +a",

    mea$isi ,e" #alasemia e#a +a", e$e-a -a$i ke-&a 7$a", #&a iasa"+a

    me",alami a"emia e$a# pa-a #a!&" pe$#ama ke!i-&pa".

    Kea-aa" i"i -i!asilka" -a$i kek&$a",a" $a"#ai ,l7i" e#a kelei!a" $a"#ai

    al%a +a", -ip$esipi#asi ke -alam p$ek&$s7$ sel -a$a! me$a! se!i",,a me"+eaka"

    ke$&saka" sel #e$se aik -alam s&ms&m #&la", a#a&p&" pem&l&! -a$a!

    pe$i%e$. Hipe$#$7%i -a$i s&ms&m #&la", +a", #i-ak e%ek#i% me"+eaka" pe$&a!a"

    skele#al -a" ;&,a !epa#7sple"7me,ali +a", e$va$iasi. Ka-a$ HF selal&

    me"i",ka#. Pa-a saa# a"ak/a"ak i"i -ie$ika" #$a"s%&si s&ms&m #&la", -apa#

    me",!e"#ika" p$7-&ksi"+a se!i",,a #e$;a-i pe$#&m&!a" -a" pe$kema",a" +a",

    "7$mal. Meskip&" -emikia" -apa# #e$;a-i ak&m&lasi esi -a" me"+eaka"

    kema#ia" akia# ke$&saka" pa-a mi7ka$-i&m pa"k$eas a#a& !a#i. Me$eka ;&,a

    $e"#a" #e$!a-ap i"%eksi -a" me"-e$i#a -e%isie"si asam %7la#. Be"#&k/e"#&k

    #alasemia e#a +a", lei! $i",a" >#alasemia i"#e$me-ia? ala&p&" #i-ak selal&

    #e$,a"#&", pa-a #$a"s%&si #e$ka-a", ;&,a -ikai#ka" -e",a" pe$&a!a" #&la",

    a"emia &lk&s #&",kai aa! -a" ,a",,&a" pe$#&m&!a".1811

    Ca$iasi kli"is -a$i #alasemia e#a +a", -i"amaka" #alasemia i"#e$me-ia

    e$a-a -ia"#a$a spek#$&m #alasemia ma+7$ -a" pemaa si%a# asimp#7ma#ik.

    Si"-$7m i"i melipi kelai"a"/kelai"a" -e",a" spek#$&m -isaili#as +a", l&as

    pa-a sisi +a", e$a# pasie" -apa# -a#a", -e",a" a"emia lei! lama#

    -ia"-i",ka" -e",a" #alasemia e#a #e$,a"#&", #$a"s%&si -a" !a"+a mamp&

    mempe$#a!a"ka" !em7,l7i" k&$a", lei! 4 ,

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    Se2a$a &m&m ,ama$a" kli"is #alasemia e#a i"#e$me-ia mi$ip -e",a"

    #alasemia e#a ma+7$. Pa-a spek#$&m +a", e$a# k!&s&s"+a pa-a kas&s/kas&s

    ,a",,&a" pe$#&m&!a" pasie" !a$&s -i#e$api -e",a" #$a"s%&si $i". Meskip&"

    -emikia" ee$apa k7mplikasi pe"#i", sepe$#i !ipe$sple"isme p$7,$esi% #im&l

    pa-a pasie" -e",a" e"#&k/e"#&k +a", lei! $i",a". Ge;ala kli"is kelei!a"

    ea" esi akia# pe"i",ka#a" as7$psi a!ka" -apa# -ili!a# pa-a pasie" -e",a"

    #$a"s%&si +a", #i-ak $i".91)

    (" Talasemia Al3a

    H7m7=i,7# #alasemia 7 Si"-$7m !i-$7ps H Ba$#s i"i iasa"+a #e$;a-i -alam

    $a!im. Bila !i-&p !a"+a -alam ak#& pe"-ek. Gama$a" kli"is"+a a-ala!

    !i-$7ps %e#alis -e",a" e-em pe$ma,"a -a" !epa#7sple"7me,ali. Ka-a$ H 4/'

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    brilliant cresil blue+a", aka" me"+eaka" pe",e"-apa" -a" peme"#&ka"

    a-a" i"kl&si. Se#ela! sple"ek#7mi e"#&ka" i"i maki" a"+ak pa-a e$i#7si#.

    B" Dam*ak Kele(ihan Be(an Besi Aki(at Trans3usi Darah

    Besi me$&paka" l7,am +a", pe"#i", &"#&k si"#esis !em7,l7i" $eaksi

    7ksi-asi $e-&ksi -a" p$7li%e$asi sel se-a",ka" kelei!a" esi aka" me"+eaka"

    -is%&",si 7$,a" lea# p$7-&ksi spesies 7ksi,e" $eak#i%. J&mla! ka-a$ esi -i

    -alam #&&! e$kisa$ a"#a$a 3/: , -&a pe$#i,a e$a-a -i -alam sel -a$a! me$a!

    -a" -i-a&$ &la", -e",a" pe",!a"2&$a" e$i#$7si# sisa"+a -isimpa" -alam e"#&k

    %e$$i#i"

    ,as#$7i"#es#i"al -a" e$e-a$ -i -alam -a$a!. Me#a7lisme esi #&&! me$&paka"

    s&a#& sis#em se#e",a! #e$#&p -a" se2a$a k$i#ikal -i$e,&lasi 7le! ee$apa %ak#7$

    #e$mas&k !ep2i-i" +a", a$& sa;a -i#em&ka".Dalam pe$e-a$a" -a$a! esi

    iasa"+a #e$ika# pa-a #$a"s%e$$i" -a" kea"+aka" esi #e$ika# #$a"s%e$$i" -i,&"aka"

    7le! s&ms&m #&la", &"#&k e$i#$7p7iesis. le! ka$e"a #i-ak a-a"+a meka"isme

    ak#i% -i -alam #&&! &"#&k me",eksk$esika" esi s&a#& ak&m&lasi p$7,$esi% esi

    #&&! aka" m&-a! #e$;a-i sea,ai akia# -a$i #$a"s%&si e$kepa";a",a" pa-a

    pasie" -e",a" #alasemia.1813

    Se#iap &"i# e$i#$7si# +a", -i#$a"s%&si me",a"-&", )88/)*8 m, esi sea,ai

    a,ia" -a$i pi,me" !eme. Pa-a saa# e$i#$7si# -ipe2a! 7le! sis#em mak$7%a, esi

    -ilepaska" -a$i !eme -a" -isimpa" -i -alam #&&!. le! ka$e"a pe",el&a$a" esi

    !a$ia" 7le! #&&! -alam ke$i",a# -a" pelepasa" sel epi#el !a"+a e$kisa$ 1 m,

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    maka peme$ia" sa#& &"i# PR( e$!&&",a" -e",a" seki#a$ )88 !a$i sikl&s esi

    !a$ia" #&&!. le! ka$e"a kelei!a" esi #i-ak -apa# -ielimi"asi -a$i #&&! maka

    #$a"s%&si k$7"ik aka" me"+eaka" keseima",a" esi #&&! sa",a# ;a&! -a$i

    ek&li$i&m. T7ksisi#as esi -alam -7sis #i",,i -iseaka" 7le! ka$e"a

    kemamp&a""+a e$eaksi -e",a" m7lek&l 7ksi,e" memi"-a!ka" elek#$7" ke

    -alam"+a -a" me",!asilka" spesies 7ksi,e" a"#a$a +a", kem&-ia" -e",a"

    a-a"+a esi aka" me"+eaka" #e$e"#&k"+a $a-ikal/$a-ikal +a", lei! $eak#i%

    la,i. Ra-ikal $eak#i% i"i aka" me"+e$a", lipi- p$7#ei" -a" DNA me"+eaka"

    #e$;a-i"+a ke$&saka" sel +a", pa-a ak!i$"+a aka" #im&l sea,ai -is%&",si

    7$,a".181:

    Ha#i me$&paka" 7$,a" #e$pe"#i", &"#&k pe"+impa"a" esi -e",a"

    kapasi#as #e$esa$ &"#&k me"sek&es#$asi kelei!a" esi.Pe$&a!a" pe$i7-ik

    -is%&",si 7$,a" #ela! -ipela;a$i pa-a pasie" -e",a" #alasemia e#a !7m7=i,7#.

    Biasa"+a -alam ak#& ) #a!&" #$a"s%&si a"7$mali#as %&",si !a#i sepe$#i

    pe"i",ka#a" e"=im #$a"sami"ase #i-ak #e$lal& "+a#a -a" iasa"+a e$a-a -alam

    a#as "7$mal a#a& !a"+a se-iki# me"i",ka#. Selama pe$i7-e i"i peme$iksaa"

    i7psi !a#i aka" me"&";&kka" %i$7sis $i",a" -e",a" i"%lamasi $i",a" -a"

    -ep7sisi esi. Se2a$a kli"is !a#i me";a-i esa$ -a" -apa# -ipalpasi -a"

    peme$iksaa" %&",si !a#i lai""+a -alam $e"#a", "7$mal a#a& se-iki# me"i",ka#.

    le! ka$e"a i#& pe"#i", &"#&k pasie"/pasie" #e$,a"#&", #$a"s%&si -i"ilai se2a$a

    me"+el&$&! &"#&k memas#ika" a-a"+a kelai"a" !a#i %i$7#ik a#a& si$7sis -e",a"

    peme$iksaa" (T/S2a" MRI -a" a"alisis i7kimia #e$mas&k peme$iksaa"

    #$a"sami"ase se$&m.18

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    Pe"+ea pali", pe"#i", -a$i #$a"s%&si ;a",ka pa";a", a-ala! kema#ia"

    me"-a-ak 7le! ka$e"a ,a,al ;a"#&",. Dilap7$ka" k&$a", lei! 586 kema#ia" pa-a

    pasie" #alasemia e#a -iseaka" 7le! sea ka$-i7,e"ik. Ta"-a/#a"-a -is%&",si

    ka$-iak #e$mas&k !ipe$#$7%i ;a"#&", a$i#mia -a" e"-7ka$-i#is +a", pa-a ak!i$"+a

    aka" me"+eaka" ,a,al ;a"#&",. Ga",,&a" ve"#$ikel ki$i sa",a# me"7";7l -a"

    -i,ama$ka" 7le! pe"&$&"a" %$aksi e;eksi ve"#$ikel lea# peme$iksaa"

    e2!7ka$-i7,$am. le! ka$e"a pe"&$&"a" %$aksi e;eksi ve"#$ikel i"i #im&l seel&m

    #a"-a/#a"-a kli"is ,a,al ;a"#&", -a" ;&,a seel&m pemesa$a" a+a",a" ;a"#&",

    pa-a $7"#,e" -a-a e2!7ka$-i7,$am me$&paka" peme$iksaa" pali", e$,&"a &"#&k

    pema"#a&a" ke$&saka" mi7ka$-ial 7le! kelei!a" ea" esi.1811

    E2!7ka$-i7,$am +a", -isa$a"ka" &"#&k -e#eksi ke,a,ala" ;a"#&", +a",

    -iseaka" 7le! -ep7sisi esi -i mi7ka$-ial a-ala! -e",a" D7pple$ ;a$i",a".

    Pemi"-aia" -ilak&ka" lea# ;e"-ela ak&s#ik empa#/ilik apikal.La;& mi7ka$-ial

    kem&-ia" -ipe$iksa se2a$a #e$&s me"e$&s -a$i asal ke apeks -i -alam -i"-i",

    eas ve"#$ikel ki$i -a" ka"a" ;&,a -i sep#&m i"#e$ve"#$ik&la$ >Gama$ ).3?.

    Pe"eli#ia" 7le! C7,el me"em&ka" a!a se"si#ivi#as e2!7ka$-i7,$am

    D7pple$ &"#&k me"em&ka" -ep7sisi esi a"7$mal seesa$ ''6 -e",a"

    spesi%isi#as 4*6 >me",,&"aka" T) MRI sea,ai ak& emas?.111)

    11

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    Gam(ar &"5 Suatu *emeriksaan ech4car#i4gram D4**ler 6aringan

    menun6ukkan *eru(ahan arna #ari (iru ke merah *a#a

    se*tum mi4kar#ial se(agai tan#a a#anya #e*4sisi

    (esi"Ter(aliknya gel4m(ang s #an e *a#a a*eks menan#akan

    a#anya a(n4rmalitas gerakan #in#ing *a#a sist4lik #an

    #iast4lik"Kea#aan ini #a*at tim(ul (ahkan *a#a *asien

    talasemia #engan 3raksi e6eksi #alam (atas n4rmal"

    12

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    MRI ;&,a me$&paka" peme$iksaa" +a", e$,&"a &"#&k me"ilai %&",si

    ve"#$ik&la$ -a" -ep7sisi esi pa-a 7#7# ;a"#&", -apa# -i-e#eksi -e",a"

    pe"i",ka#a" i"#e"si#as si"+al. Lei! ;a&! la,i pe$!i#&",a" T) -a" R) -e",a"

    MRI mem&",ki"ka" pe"ilaia" k7"se"#$asi semi/k&a"#i#a#i% pa-a 7#7# ;a"#&",

    a!ka" pa-a ka-a$ +a", $ela#i% $e"-a!. Pe"eli#ia" 7le! C7,el ;&,a me"em&ka"

    "ilai T) +a", "7$mal e$kisa$ a"#a$a )8 -a" '3 ms "ilai T) -i aa! )8 ms

    me"a"-aka" a-a"+a -ep7sisi esi mi7ka$- a"7$mal >,ama$ '?. A"-e$s7" -a"

    kaa"/kaa" ;&,a me"&";&kka" a!a MRI T) -apa# me"-e#eksi a-a"+a

    -ep7sisi esi mi7ka$-ial a!ka" seel&m #a"-a -a" ,e;ala ,a,al ;a"#&", #im&l

    se$#a seel&m #e$api kelasi se2a$a &m&m -ipe$#ima",ka".1:1*

    Be$-asa$ka" s&a#& s#&-i k7!7$# pasie" -e",a" #alasemia e#a -is%&",si

    7$,a" 7le! ka$e"a kelei!a" ea" esi #im&l pe$#ama kali -i !a#i pa-a saa# ka-a$

    %e$$i#i" melei!i 1.888 ",

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    Selai" -ep7sisi esi -i ;a"#&", -a" !a#i sel e#a pa"k$eas me$&paka" sala!

    sa#& #a$,e# pe"#i", &"#&k #7ksisi#as esi +a", -apa# me"+eaka" i"#7le$a"si

    ,l&k7sa -a" -iae#es melli#&s. Fak#7$ #ama!a" +a", me"+eaka" i"#7le$a"si

    ,l&k7sa a-ala! ,a",,&a" ilisasi i"s&li" 7le! !a#i +a", me",aksele$asi

    ke$&saka" sel e#a 7le! ka$e"a !ipe$i"s&li"emia. Di#i";a& -a$i pe$spek#i%

    peme$iksaa" se$ial ,&la -a$a! &$i" -a" ,lik7al&mi" e$,&"a &"#&k mema"#a&

    #e$;a-i"+a -iae#es melli#&s se-a",ka" peme$iksaa" ,lik7!em7,l7i" #i-ak

    e$,&"a 7le! ka$e"a -ampak #$a"s%&si e$&la",.1:1*

    E"-7k$i"7pa#i 7le! ka$e"a #$a"s%&si ;a",ka pa";a", -apa# -iama#i

    #e$mas&k ,a",,&a" pe$#&m&!a" p&e$#as i"k7mpli# -a" -is%&",si #i$7i-.Pa-a

    pasie" -e",a" #alasemia -a" a"emia sel sai# pe$!a#ia" k!&s&s !a$&s -ie$ika"

    kepa-a #a"-a -a" ,e;ala aal sepe$#i ,a",,&a" pe$#&m&!a" -a" ima#&$i#as

    seks&al.1811

    7" Tera*i Kelasi Besi

    T&;&a" ama #e$api kelasi esi a-ala! me"2apai ka-a$ esi #&&! +a",

    ama". Peme$ia" #e$api kelasi esi +a", a-ek&a# -a" kepa#&!a" pasie" sa",a#

    me"e"#&ka" kee$!asila" #e$api i"i. Te$api kelasi esi -im&lai apaila ka-a$

    %e$i#i" se$&m me"2apai 1888 ",

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    S#aili#as k7mpleks esi/kela#7$

    E%isie"si kelasi #i",,i

    La;& me#a7lisme $e"-a!

    Be$a# m7lek&l #i-ak #e$lal& esa$

    Pe"e#$asi ;a$i",a" -a" sel

    S7l&ili#as -alam ai$ -a" lipi-

    Ti-ak a-a $e-is#$i&si esi

    Rela#i% "7"/#7ksik

    Pe"2apaia" keseima",a" esi "e,a#i%

    M&$a!

    Dapa# -ie$ika" se2a$a 7$al

    Pe"eli#ia" &"#&k me"i",ka#ka" k&ali#as #e$api kelasi esi #ela! -ilak&ka"

    -alam 38 #a!&" #e$ak!i$. De%e$7ksami" me$&paka" kelasi esi +a", pali", lama

    -ipakai -i -&"ia. Peme$ia" -e%e$7ksami" se2a$a s&ka" #e$"+a#a meme$ika"

    pe$&a!a" +a", esa$ -alam me"i",ka#ka" &sia !a$apa" !i-&p -a" me",&$a",i

    k7mplikasi akia# kelei!a" esi se!i",,a #e$api i"i -i;a-ika" #e$api s#a"-a$

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    &"#&k pasie" #!alasemia -i sel&$&! -&"ia. Te#api peme$ia" -e",a" 2a$a s&ka"

    me"+eaka" ke#i-akpa#&!a" +a", 2&k&p #i",,i se!i",,a pa$a a!li -&"ia

    me"27a me"2a$i al#e$"a#i% lai" -e",a" me"em&ka" %7$m&lasi kelasi esi se2a$a

    7$al.131:

    $" 1enis81enis Kelasi Besi

    Je"is/;e"is #e$api kelasi esi +a", -ike"al saa# i"i a-a #i,a ;e"is +ai#& +a",

    -ie$ika" se2a$a pa$e"#e$al >De%e$7ksami"? -a" +a", -ie$ika" se2a$a 7$al

    >De%e$ip$7" -a" De%e$asi$7@?.

    a" De3er4ksamin

    De%e$7ksami" >DF? me$&paka" kela#7$ esi -e",a" e"#&k m7lek&l

    !eksa-e"#a# +a", pali", lama -a" a"+ak -ipakai +ai#& se;ak #a!&" 1954.

    De%e$7ksami" a-ala! m7lek&l e$e"#&k !eksa-e"#a# -e",a" e$a# m7lek&l *48

    kDa -e",a" -emikia" -e%e$7ksami" s&li# -ias7$psi -i sal&$a" 2e$"a.

    De%e$7ksami" me$&paka" s&a#& asam #$i!i-$7ksama# +a", -ip$7-&ksi 7le!

    Streptomyces pilosus. a# i"i memp&"+a spesi%isi#as &"#&k esiferric>esi 7ksi-a#

    -alam k7mpleks p$7#ei" %e$$i#i"?. De%e$7ksami" s&li# -ias7$psi pe$7$al -a" 2epa#

    -ime#a7lisme se!i",,a kek&$a",a" ama"+a a-ala! ke&!a" &"#&k -ie$ika"

    se2a$a i"%&s pa$e"#e$al k7"#i"+&.

    $9 Mekanisme Ker6a

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    Sa#& m7lek&l DF -apa# me",ika# 1 a#7m esi -a" memiliki s#aili#as

    +a", #i",,i #e$!a-ap Fe3. De%e$7ksami" me$&paka" s&a#& m7lek&l !i-$7%ilik

    se!i",,a amila" ke -alam sel -a" k7mpa$#eme" s&sel&la$ me";a-i lama#

    #e#api amila" ke -alam !epa#7si# 2&k&p 2epa#. Ka-a$ #e$api -i2apai -alam ak#&

    si",ka# +ai#& seki#a$ */18 me"i# -a" aka" !ila", se,e$a se#ela! pe",!e"#ia" #e$api

    +ai#& seki#a$ )8 me"i#. Eksk$esi 7a# #e$;a-i melal&i &$i" -a" %eses.19

    &9 Penggunaan

    Peme$ia" -e%e$7ksami"e -apa# -ilak&ka" se#ela! peme$iksaa" ka-a$

    %e$$i#i" a#a& se#ela! sa#& #a!&" -a" -ilak&ka" peme$iksaa" ka-a$ esi !a#i -e",a"

    i7psi -7sis aal #i-ak melei!i )*/3* m,

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    59 E3ek Sam*ing

    Bee$apa k7mplikasi akia# pe",,&"aa" DF a-ala! ,a",,&a"

    pe"-e",a$a" se"s7$i"e&$al %$ek&e"si #i",,i >1'6? ,a",,&a" pe",li!a#a")6? i"%eksi akia# e$si"ia

    >16? -a" "+e$i pa-a #empa# pemasa",a" >96?. (&""i",!am me"-apa#ka" a!a

    "+e$i pa-a #empa# pemasa",a" DF me$&paka" pe"+ea #e$se$i", pasie"

    #alasemia me",!e"#ika" pe",,&"aa" DF. livie$i -kk me$ek7me"-asika"

    -e#eksi -i"i #7ksisi#as DF &"#&k me",emalika" a"7$mali#as -e",a" 2a$a

    m7-i%ikasi #e$api >Tael ).1?.

    Ta(el &"$ Penuntasan T4ksisitas Pa#a Penggunaan D,O

    Ka-a$ vi#ami" ( +a", $e"-a! ;&,a #ela! -i#em&ka" pa-a pasie"/pasie"

    #alasemia -e",a" kelei!a" ea" esi s&pleme"#asi -apa# me"i",ka#ka"

    eksk$esi esi 7le! -e%e$7ksami" -e",a" mel&aska" 2a-a",a" esi +a", -apa#

    -ikelasi. Nam&" #e$api i"i !a$&s -ilak&ka" se2a$a !a#i/!a#i ka$e"a pel&asa"

    2a-a",a" esi i"i -apa# me"i",ka#ka" peme"#&ka" $a-ikal eas -a"

    mempe$e$a# #7ksisi#as esi. Ka$e"a e%ek sampi", i"i maka peme$ia" asam

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    ask7$a# !a"+a -ia";&$ka" ila #e$-apa# pe"&$&"a" e%isie"si peme$ia"

    -e%e$7ksami" -a" -ie$ika" -7sis 188/)88 m, !a"+a pa-a saa# -ie$ika" #e$api

    -e%e$7ksami" >38/48 me"i# ses&-a! #e$api -im&lai?.1)1*

    ala&p&" kepa#&!a" e$7a# -e",a" -e%e$7ksami" me$&paka"

    pe$masala!a" ama s#&-i/s#&-i #ela! me"&";&kka" a!a peme$ia"

    -e%e$7ksami" me"&";&kka" ke&"#&",a" ka$-i7p$7#ek#i% pa-a pasie"/pasie" +a",

    mela";ka" #e$api se2a$a #e$a#&$. Pe"eli#ia"/pe"eli#ia" #e$a$& ;&,a me"&";&kka"

    peme$ia" -e%e$7ksami" selama lei! -a$i 18 #a!&" pa-a pasie" #alasemia

    -ikai#ka" -e",a" masa eas k7mplikasi kelei!a" ea" esi +a", pa";a",.1)1:

    (" De3eri*r4n

    De%e$ip$i" >DFP? me$&paka" m7lek&l i-e"#a# -e",a" e$a# m7lek&l 139

    kDa. Pe"eli#ia" ;a",ka pa";a", -a$i -e%e$ip$7" pa-a #alasemia #ela! me"&";&kka"

    pe"&$&ka" ka-a$ %e$$i#i" se$&m se2a$a si,"i%ika" #e$ama pa-a pasie" -e",a"

    ka-a$ %e$$i#i" seel&m s#&-i lei! -a$i *888 ",papa$a" se#a$a -e",a" 5*8 #a!&"

    pasie" Tael ).)?.1*1'

    a# i"i ;&,a me"&";&kka" e%ek#ivi#as +a", lei! aik -ia"-i",ka"

    -e%e$7ksami" -alam memi"-a!ka" esi mi7ka$-ial pa-a pasie"/pasie"

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    asimp#7ma#ik. Se!i",,a -ia"-i",ka" -e",a" -e%e$7ksami" peme$ia"

    -e%e$ip$7" -ikai#ka" -e",a" p$7#eksi ka$-iak +a", lei! esa$.

    $9 Mekanisme Ker6a

    Pa-a -asa$"+a 2a$a ke$;a -a$i De%e$ip$7" >DFP? !ampi$ sama -e",a"

    De%e$7ksami" >DF? !a"+a sa;a peme$ia" kela#7$ +a", sa#& i"i melal&i 7$al.

    Ti,a m7lek&l DFP -apa# me",ika# 1 m7lek&l esi >31? -e",a" 7"se# p&"2ak

    seki#a$ :* me"i# se#ela! peme$ia" -a" -e",a" ak#& pa$&! seki#a$ *3/144 me"i#.

    Kela#7$ i"i me",eksk$esika" esi melal&i &$i".19

    &9 Penggunaan

    Peme$ia" -e%e$ip$7" -apa# -im&lai -e",a" -7sis )* m,

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    Be$a,ai pe"eli#ia" #ela! me"&";&kka" a!a DFP e$!asil me"&$&"ka"

    ka-a$ %e$i#i" se2a$a e$mak"a. Ke&"#&",a" lai" me",,&"aka" DFP a-ala! e%ek

    p$7#eksi #e$!a-ap ;a"#&", +a", me$&paka" pe"+ea kema#ia" #e$esa$ pa-a

    pasie" #alasemia. E%ek ka$-i7p$7#eksi -ilap7$ka" lei! s&pe$i7$ -ia"-i",ka"

    DF. A"-e$s7" -kk me"&";&kka" a!a pasie" #alasemia +a", me",,&"aka"

    -e%e$ip$7" memp&"+ai ka"-&",a" esi -i ;a"#&", +a", lei! $e"-a! -ia"-i",ka"

    -e",a" me",,&"aka" -e%e$7ksami". Pe""ell -kk me"em&ka" a!a DFP

    m7"7#e$api selama 1 #a!&" lei! e%ek#i% se2a$a e$mak"a -ia"-i",ka" DF

    -alam mempe$aiki si-e$7sis mi7ka$-ial asimp#7ma#ik pa-a #alasemia/0 ma+7$.

    59 E3ek Sam*ing

    E%ek sampi", +a", m&",ki" #e$;a-i akia# pe",,&"aa" DFP a-ala!

    a,$a"&l7si#7sis >8*6? "e$7pe"ia >'86? a$#$7pa#i >1*86? kel&!a"

    ,as#$7i"#es#i"al >33)6? -a" pe"i",ka#a" e"=im #$a"sami"ase !a#i.

    A,$a"&l7si#7sis -a" "e$7pe"ia lei! se$i", #im&l pa-a pasie" +a", me",alami

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    !ipe$sple"isme -a" me$&paka" e%ek sampi", se$i&s. Meskip&" -emikia" Pe""el

    -kk me"em&ka" -alam pe"eli#ia" selama 1 #a!&" a!a a,$a"&l7si#7sis i"i

    e$si%a# seme"#a$a -a" a!ka" -apa# memaik #a"pa pe",!e"#ia" pe",7a#a".

    A$#$7pa#i aal"+a -i-&,a #e$kai# -e",a" ka-a$ %e$i#i" se$&m pasie" "am&"

    pe"eli#ia" #i-ak -apa# mem&k#ika" a-a"+a ke#e$kai#a" #e$se. Kel&!a"

    ,as#$7i"#es#i"al -a" a$#$7pa#i aka" maki" me"&$&" sei$i", -e",a" maki" lama

    pe",,&"aa" DFP. E%ek sampi", +a", -apa# #e;a-i #e$mas&k ke!ila",a"

    pe"-e",a$a" %$ek&e"si #i",,i a"7$mali#as $e#i"al -a" kelai"a" me#a%isis se$#a

    spi"al +a", -apa# me"+eaka" pe"&$&"a" #i",,i a-a".1:

    Peme$ia" DFP pa-a #alasemia pe$"a! -ikai#ka" -e",a" pe"i",ka#a"

    e"=im ala"i" #$a"s%e$ase >ALT? -a" ke;a-ia" %i$7sis !a#i. Se;a&! i"i el&m a-a

    pe"eli#ia" +a", -apa# me"&";&kka" a-a"+a kai#a" e$mak"a a"#a$a pe",,&"aa"

    DFP -a" %i$7sis !a#i. a"less -kk mem&k#ika" -e",a" melak&ka" i7psi

    seel&m -a" ses&-a! peme$ia" DFP pa-a pasie" #alasemia -a" #i-ak a-a sa#&p&"

    pasie" +a", me",alami %i$7sis !a#i. Fi$7sis !a#i pa-a peme$ia" DFP lei!

    se$i", #e$;a-i pasie" #alasemia +a", me"-e$i#a !epa#i#is ( se!i",,a -i-&,a k&a#

    a!a ke;a-ia" %i$7sis #e$se lei! -iseaka" 7le! i"%eksi !epa#i#is (.15

    c" De3erasir433)6? -a" pe"i",ka#a" e"=im #$a"sami"ase !a#i.1:

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    BAB III

    PEN-T-P

    A" Kesim*ulan

    Talasemia saa# i"i me$&paka" pe$masala!a" kese!a#a" +a", semaki" esa$

    -i "e,a$a/"e,a$a e$kema",. Sei$i", -e",a" pe$aika" #a$a% ke!i-&pa" -a"

    la+a"a" kese!a#a" maka aka" semaki" a"+ak pe"-e$i#a #alasemia +a", me"2apai

    &sia -easa. Peme$ia" #e$api #$a"s%&si se2a$a me"a!&" aka" me",akia#ka"

    pe"-e$i#a #alasemia me"-e$i#a kelei!a" ea" esi -e",a" se,ala akia# -a"

    k7mplikasi"+a.

    Te$api -e",a" kelasi esi aik se2a$a 7$al ma&p&" i"#$ave"a #ela!

    me"&";&kka" ma"%aa# +a", aik -alam me"&$&"ka" m7$#ali#as -a" m7$i-i#as

    25

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    pasie" -e",a" !em7si-e$7sis sek&"-e$. Ti-ak a-a pe$e-aa" e$mak"a a"#a$a

    e%ek#ivi#as #e$api kelasi esi +a", e$e-a se!i",,a pemili!a" -i-asa$ka" pa-a

    p$e%e$e"si -a" ke#e$se-iaa" 7a# -i masi",/masi", #empa#.

    Upa+a pe"2e,a!a" kelei!a" esi pa-a pasie" #alasemia ma+7$ +a",

    a"+ak -ipakai saa# i"i a-ala! -e%e$7ksami" s&ka". (a$a i"i e%ek#i% -alam

    me"&$&"ka" ka-a$ esi ila -ilak&ka" #e$a#&$ -e",a" kepa#&!a" +a", #i",,i.

    U"#&k me"i",ka#ka" kepa#&!a" -apa# -i,&"aka" 7a# kelasi esi pe$7$al +ai#&

    -e%e$ip$7" >L1? a#a& -e%e$asi$7@ >I(L 458? +a", -apa# -ie$ika" se2a$a

    m7"7#e$api a#a&p&" k7mi"asi. Te$api kelasi esi pe$7$al -i!a$apka"

    me"i",ka#ka" kepa#&!a" pasie" se!i",,a ke$&saka" 7$,a" akia# !em7k$7ma#7sis

    -apa# -i$e-&ksi. Pe$masala!a" +a", masi! m&"2&l a-ala! !a$,a kela#7$ #e$se

    el&m -apa# -i;a",ka& 7le! sea,ia" esa$ mas+a$aka# -i "e,a$a e$kema",

    #e$mas&k I"-7"esia.1415

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    DA,TA0 P-STAKA

    1. M2Le7- ( et. al. De%e$asi$7@ %7$ #!e #$ea#me"# 7% i$7" 7ve$l7a- ass72ia#e-i#! $e,&la$ l77- #$a"s%&si7"s >#$a"s%&si7"al !em7si-e$7sis? i" pa#ie"#ss&%%e$i", i#! 2!$7"i2 a"emia a s+s#ema#i2 $evie a"- e27"7mi2aleval&a#i7". Heal#! Te2!"7l7,+ Assesme"# )889 13 1)3/1)4.

    ). Ae#= L Bala-i JF J7"es P et.al. T!e impa2# 7% i$7" 7ve$l7a- a"- i#s #!e$ap+7" &ali#+ 7% li%e $es&l# %$7m a li#e$a#&$e $evie. Heal#! a"- &ali#+ 7% Li%emes )884 : 1/4.

    3. N7$e$# G. T$ea#me"# 7% Se27"-a$+ Hem72!$7ma#7sis. Des2!es O$=#ela##I"#e$"a#i7"al )889 184 :99/*8:.

    :. Davies S( et. al. S2$ee"i", %7$ si2kle 2ell -isease a"- #alasemia as+s#ema#i2 $evie i#! s&ppleme"#a$+ $esea$2!. Heal#! Te2!"7l7,+Assesme"# )888 :.

    *. G$ee"e$, PL et al. Ma;7$ !ema#7l7,i2 -isease i" #!e -evel7pi", 7$l-Ne aspe2# 7% -ia,"7sis a"- ma"a,eme"# 7% #alasemia mala$ial a"emia a"-a2e le&kaemia. Hema#7l7,+ )881 :59 / :9'.

    4. (e2i A Ma",ia$i"i L Fellsi M et al. T!e Ma"a,eme"# 7% I$7" (!ela#i7"T!e$ap+P$elimi"a$+ Da#a %$7m a Na#i7"al Re,is#$+ 7% Talasemia Pa#ie"#s.Hi"-ai P&lis!i", (7$p7$a#i7" )811 1 1/5.

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    5. ea#!e$all DJ. T!e !e$e-i#a$+ a"emias. e-.Q D$e P$7va". AB( 7% (li"i2alHema#7l7,+ )"- e-. s.l. BMJ B77ks )883.

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    18. Jaa$ DA Davis7" G M&sli" AJ. Ge##i", #!e i$7" 7 P$eve"#i", a"-#$ea#i", !ea$# %ali&$e i" #$a"s%&ssi7" -epe"-e"# #alasemia. (levela"- (li"i2J7&$"al 7% Me-i2i"e )885 5 '85/'14.

    11. Aess7p7s A Be$-7&kas C Tsi$7"i M. T!e !ea$# i" #$a"s%&si7" -epe"-e"#!7m7=+,7&s#!alassaemia #7-a+ p$e-i2#i7" p$eve"#i7" a"- ma"a,eme"#.

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    13. B7$,"a/Pi,"a##i ( et al. (a$-ia2 m7$i-i#+ a"- m7$#ali#+ i" -e%e$7@ami"e/ 7$-e%e$ip$7"e/#$ea#e- pa#ie"#s i#! #alasemia ma;7$. Bl77- )884 185 3533/

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    1:. (7!e" AR. Ne A-va"2es i" I$7" (!ela#i7" T!e$ap+. Hema#7l7,+ )884 :)/:5.

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    1*. S!a$ma RN Pa"2!7li SS. $al I$7" (!ela#7$s A Ne Ave"&a %7$ #!eMa"a,eme"# 7% Talasemia Ma;7. J7&$"al 7% (&$$e"# P!a$ma2ei2al Resea$2!

    )818 1 1/5.

    14. Ta""e$ MA Gala"ell7 R Dessi ( et al. (7mi"a#i7" 2!ela#i7" #!e$ap+ i"#alasemia ma;7$ %7$ #!e #$ea#me"# 7% seve$e m+72a$-ial si-e$7sis i#! le%#ve"#$i2&la$ -+s%&"2#i7". J7&$"al 7% (a$-i7vas2&la$ Ma,"e#i2 Res7"a"2e)88' 18>1)? 1/9.

    15. B;!. S&22es%&l 2!ela#i7" #!e$ap+ i#! #!e 27mi"a#i7" 7% -e%e$asi$7@ a"--e%e$ip$7"e i" a pa#ie"# i#! #alasemia ma;7$ a"- pe$sis#i", seve$e i$7"7ve$l7a- a%#e$ si",le/a,e"# 2!ela#i7" #!e$apies. B$i#ies J7&$"al 7%Haema#7l7,+ )811 1*: 4*:/44*.

    1'. (e2i A Ma",ia$i"i L Felisi M et. al. T!e Ma"a,eme"# 7% I$7" (!ela#i7"T!e$ap+ P$elimi"a$+ Da#a %$7m a Na#i7"al Re,is#$+ 7% T!alassaemi2Pa#ie"#s. Hi"-ai P&lis!i", (7$p7$a#i7" )811 1 1/5.

    19. Ga#7# D. Pe"-eka#a" mak!i$ kelasi esi pa-a #!alassemia. Sa$i Pe-ia#$i)885 '>:? 5'/':.