FINAL TESIS JADI ALHAMDULILLAH.docx

7/25/2019 FINAL TESIS JADI ALHAMDULILLAH.docx

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PERBEDAAN KADAR SGOT, SGPT DAN GAMMA GT PADA PASIEN

TALASEMIA BETA MAYOR DENGAN KELASI BESI DEFERASIROX

DAN DEFERIPRONE

TESIS

Disusun untuk Memenuhi Sebaian Pe!s"a!atan Men#a$ai De!a%at

Maiste!

P!&!am Stu'i Ke'&kte!an Ke(ua!a

Minat )tama I(mu Bi&me'ik

OLE* +

Rea Ab'ussa(am

S -./0.0.-1

PROGRAM PAS2ASAR3ANA

)NI4ERSITAS SEBELAS MARET

S)RAKARTA

1


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1

PERBEDAAN KADAR SGOT, SGPT DAN GAMMA GT PADA PASIEN

TALASEMIA BETA MAYOR DENGAN KELASI BESI DEFERASIROX DAN

DEFERIPRONE

Disusun oleh:

Rez A!"ussl#

S $%1&%&%$'

Telh "ise(u)ui oleh Ti# Pe#!i#!in*

De+n Pe#!i#!in* N# Tn" Tn*n Tn**l

Pe#!i#!in* I Po-. D. B. Soe!*/o, ", S0.AK2 .................... ...........

NIP. 13451&16 13'6%5 1 %%1

Pe#!i#!in* II Gnun* 7sono, ",S0.AK2 ................... ...........

NIP.13$1%&1'13'8%11%%1


3/73

PERNYATAAN ORISINALITAS DAN P)BLIKASI TESIS

Penulis #en/(9n "en*n se!enn/ !h+:

1. Tesis /n* !e)u"ul PERBEDAAN KADAR SGOT, SGPT DAN GAMMA

GT PADA PASIEN TALASEMIA BETA MAYOR DENGAN KELASI BESI

DEFERASIROX DAN DEFERIPRONE; ini "lh 9/ 0eneli(in 0enulis

sen"ii "n !e!s 0l*i(, se( (i"9 (e"0( 9/ il#ih /n* 0enh

"i)u9n oleh on* lin un(u9 #e#0eoleh *el 9"e#i9 se( (i"9

(e"0( 9/ (u 0en"0( /n* 0enh "i(ulis (u "i(e!i(9n oleh on*

lin 9e


4/73

KATA PENGANTAR

Se*l 0u)i s/u9u 9eh"i( Allh S@T (s se*l h#( "n hi"/h=

N/ sehin** 0enulis "0( #en/elesi9n (esis "en*n )u"ul PERBEDAAN

KADAR SGOT, SGPT DAN GAMMA GT PADA PASIEN TALASEMIA

BETA MAYOR DENGAN KELASI BESI DEFERASIROX DAN

DEFERIPRONE;.

Tesis ini #eu09n 0es/(n +)i! un(u9 #e#0eoleh "e)( #*is(e

9eseh(n Pese( Po*# S(u"i Il#u Keseh(n An9 F9ul(s Ke"o9(en>niesi(s Se!els Me( Su9(. P" 9ese#0(n ini 0enulis in*in

#en*uniesi(s Se!els Me( /n* (elh

#e#!ei9n 9ese#0(n 9e0" 0enulis un(u9 #en*i9u(i 0o*# M*is(e "i

Po*# Psniesi(s Se!els Me(.

&. Po-. D. M. Fuon 7i"/(ullh, M.P", sel9u Die9(u Po*# Psniesi(s Se!els Me( /n* (elh #e#!ei9n 9ese#0(n 9e0" 0enulis

un(u9 #en*i9u(i 0o*# M*is(e "i Po*# Psniesi(s Se!els

Me(.

5. Po-. D. ". 7(ono, Msi, sel9u De9n F9ul(s Ke"o9(en >niesi(s Se!els

Me( Su9( /n* (elh #e#!ei9n 9ese#0(n 9e0" 0enulis se!*i

PPDS I Il#u Keseh(n An9 F9ul(s Ke"o9(en >NSCRS>D ". Moe+"i "n

Po*# M*is(e "i Po*# Psniesi(s Se!els Me(.

4. Po-. D. ". AA Su!i/n(o, MS, sel9u Ke(u Po*# S(u"i M*is(e Ke"o9(en

Kelu* /n* (elh #e#!ei9n 9ese#0(n "n !i#!in*n 9e0" 0enulis

un(u9 #en*i9u(i 0o*# M*is(e "i Po*# Psniesi(s Se!els

Me(.

$. ". En"n* De+i Les(i, S0.AK2, MP7, sel9u Ke0l B*in Il#u Keseh(n

An9 F9ul(s Ke"o9(en >NSCRS>D ". Moe+"i /n* (elh #e#!ei9n

9ese#0(n "n "u9un*n un(u9 #en*i9u(i PPDS I Il#u Keseh(n An9

F9ul(s Ke"o9(en >NSCRS>D ". Moe+"i "n 0o*# M*is(e Po*#

Psniesi(s Se!els Me( Su9(.

5


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6. ". Muh##" Riz S0.A M9es, sel9u Ke(u Po*# S(u"i Pen"i"i9n Do9(e

S0esilis Il#u Keseh(n An9 F9ul(s Ke"o9(en >NSCRS>D ". Moe+"i

/n* (elh #e#!ei9n 9ese#0(n "n "u9un*n un(u9 #en*i9u(i PPDS I Il#u

Keseh(n An9 F9ul(s Ke"o9(en >NSCRS>D ". Moe+"i "n 0o*#

M*is(e Po*# Psniesi(s Se!els Me( Su9(.

'. Po-. D. ". B. Soe!*/o, S0.A K2, sel9u 0e#!i#!in* #e(o"olo*i /n* (elh

!ese"i #elun*9n +9(u un(u9 #e#!i#!in* 0enulis "en*n 0enuh 9es!n.

8. ". Gnun* 7sono, S0. AK2, sel9u 0e#!i#!in* su!s(nsi, /n* !ese"i

#elun*9n +9(u un(u9 #e#!i#!in* 0enulis "en*n 0enuh 9es!n.

3. Se#u s(- 0en*) B*in Il#u 9eseh(n An9 F9ul(s Ke"o9(en

>NSCRS>D ". Moe+"i /n* (elh #e#!i#!in*, #e#!ei9n "oon*n,

#su9n, "n se#n*( 9e0" 0enulis.

1%. Is(i "n n9 0enulis /n* (elh #ene#ni, #e#0eh(i9n, #en"o9n,

"n #e#o(isi.

Penulis #en/"i !h+ (esis ini (i"9 le0s "i 9e9un*n, un(u9 i(u

9i(i9 "n sn "i 0e#!


6/73

DAFTAR ISI

K( Pen*n(........................................................................................................i

D-( Isi.................................................................................................................i

D-( G#!......................................................................................................iii

D-( T!el............................................................................................................i

D-( Sin*9(n......................................................................................................

A!s(9....................................................................................................................i

A. L( Bel9n* Mslh...................................................................................1

B. Ru#usn Mslh.............................................................................................4

. Tu)un Peneli(in..............................................................................................4

D. Mn-( Peneli(in............................................................................................$

BAB II Tin)un Pus(9.........................................................................................'

A. K)in Teoi.......................................................................................................'

1. Tlse#i M/o........................................................................................'

&. Te0i Kelsi Besi....................................................................................14

5. Pen*uh Penu#0u9n Besi Teh"0 Fun*si 7(i.................................&1

B. Ken*9 Konse0............................................................................................5'

. 7i0o(esis Peneli(in........................................................................................58

BAB III Me(o"e Peneli(in....................................................................................53

A. Desin Peneli(in............................................................................................53

B. Te#0( Dn @9(u..........................................................................................53

. Po0ulsi Dn Pe#ilihn Su!/e9............................................................53

D. ?u#lh Su!/e9................................................................................................53

$


7/73

E. I"en(i-i9si i!el Peneli(in Dn De-inisi O0esionl.............................41

F. Ke).......................................................................................................45

G. Izin Su!/e9 Peneli(in....................................................................................45

7. Alu Peneli(in................................................................................................4$

I. Pen*olhn D(.............................................................................................45

BAB I. 7ASIL PENELITIAN DAN PEMBA7ASAN......................................46

A. 7sil Peneli(in.................................................................................................46

B. Pe#!hsn.......................................................................................................48

. Ke(e!(sn Peneli(in.....................................................................................$&

BAB . PEN>T>P................................................................................................$5

A. Si#0uln............................................................................................................$5

B. Sn..................................................................................................................$5

. I#0li9si Peneli(in...........................................................................................$4

D-( Pus(9........................................................................................................$$

L#0in

6


8/73

DAFTAR GAMBAR

G#! &.1. Mu(si *en 0" (lse#i..................................................................3

G#! &.&. Al*oi(# 0ene*9n "i*nosis (lse#i........................................11

G#! &.5. 9o#0li9si n9 "en*n (hlsse#i................................................1&

G#! &.4. Al*oi(# (e0i 9elsi !esi 0" (hlsse#i..................................18

G#! &.$. F#9o9in(ei9 "e-ei0one "n "e-eio9s#in...............................&%

G#! &.6. ?lu Re*ulsi 7e0


9/73

DAFTAR TABEL

T!el &.1.K9(eis9(i9 Kelsi Besi HHHHHHHHHHHH................1$

T!el &.&. Me9nis#e /n* #un*9in (e)"i se!*i 9i!( 0enu#0u9n !esi 0"

he0(i(is ius 9oni9 .................................................................................&3

T!el 4.1. K9(eis9(i9 Ds Kelo#0o9......................................................46

T!el 4.& K" SGOT, SGPT "n GAMMA GT 0" 9e"u 9elo#0o9 .4'

T!el 4.5 9oelsi SGOT "en*n 9" -ei(in HHHHHHHHHHH48

T!el 4.4 9oelsi SGPT "en*n 9" -ei(in HHHHHHHHHHH43

T!el 4.5 9oelsi G## GT "en*n 9" -ei(in HHHHHHHHHHH43

8


10/73

DAFTAR SINGKATAN

MRI :Magnetic resonance imaging

EKG : Ele9(o9"io*-i

TM : Tlse#i M/o

ALT :Alanine Aminotransferase

AST :Aspartate Aminotransferase

LI :Liver Iron Concentration

SGOT : Serum Glutamic Oxaloacetic Transaminase

SGPT : Serum Glutamic Pyruvic Transaminase

GAMMA GT : Gamma Glutamil Transpeptidase

PR :Paced !ed Cell

7I : 7u#n I##uno"e-i


11/73

>(# Il#u Bio#e"i9, Po*# Psniesi(s Se!els Me(

Su9(

ABSTRAK

Lata! Be(akan5 Ti#!unn !esi 0" h(i 0en"ei( (lse#i #/o /n* su"h

#en"0(9n (ns-usi !euln* #en/e!!9n *n**un -un*si h(i. Di0elu9n

9elsi !esi un(u9 #en*un*i *n**un -un*si h(i (ese!u(.

Tu%uan5 Peneli(in ini "il9u9n un(u9 #en*e(hui 09h (e"0( 0e!e"n

9" SGOT, SGPT "n GAMMA GT 0" 0sien (lse#i #/o "en*n

9elsi !esi "e-ei0one "n "e-esio.

Met&'e5 Peneli(in ini #eu09n 0eneli(in nli(i9 "en*n #e(o"e 0o(on*lin(n* "en*n 0e#ei9sn 9" SGOT, SGPT "n GAMMA GT 0"

9elo#0o9 "e-ei0one "n "e-esio /n* #e#enuhi 9i(ei in9lusi "n

e9s9lusi. Pe!e"n 9" enzi# (ese!u( "i!n"in*9n "i n( "u 9elo#0o9

"n "inlisis "en*n u)i Mnn=@hi(ne/.

*asi(5 Pe!e"n /n* !e#9n n( 9e"u 9elo#0o9 0" 9" GAMMA

GT, /i(u e( &4,$ 14,%8 uCl 0" 9elo#0o9 "e-ei0one "n e( 16,'8

6,81 uCl 0" 9elo#0o9 "e-esio "en*n nili 0%,%11. Se"n*9n 9"

SGOT "n SGPT #enuun 0" 9elo#0o9 "e-esio "i!n"in* "e-ei0one (0i

(i"9 !e#9n se


12/73

Ba#k!&un'Ion


13/73


14/73


15/73

Sin"o# (lse#i 0en/9i( *ene(i9 /n* "i+is9n "ise!!9n oleh #u(si

0" *en l0h (u !e( *lo!in, /n* #en*9i!(9n sin(esis he#o*lo!in

!no#l. Me9nis#e 0(o-isiolo*isn/ "0( "i!*i #en)"i 0enuunn 0o"u9si

(e(en(u )enis he#o*lo!in Thlsse#i2 "n 0o"u9si s(u9(u !no#l )enis

he#o*lo!in &emoglo'inopathies2. 7l ini #en/e!!9n (i"9 hn/ #o-olo*i

ei(osi( !no#l, (e(0i )u* #e#0esin*9( #s hi"u0 ei(osi( 9en

#enin*9( "i 9e0uhn io "n 0en*hn


16/73

Selin i(u, 1,3& 0esen #e#!+ he#o*lo!in s!i(, %,3$ 0esen #e#!+

he#o*lo!in E, "n %,&3 0esen #e#!+ he#o*lo!in . Den*n "e#i9in,

(in*9( 9elhin "i seluuh "uni on*=on* /n* ho#ozi*o( (u sen/+

he(eozi*o( un(u9 *n**un *lo!in *e)l, (e#su9 l0h (lse#i "n !e(

(lse#i, "lh (i"9 9un* "i &,4 0e 1%%% 9elhin, "i#n 1,36 #e#ili9i

0en/9i( sel s!i( "n %,44 #e#ili9i (hlsse#i Se(inin*sih,&%%%2.

7e#o*lo!in #e#ili9i !en(u9 (e(#e(i9 /n* s#, (e"ii "i & 0sn*

n(i *lo!in /n* (ei9( "en*n he#e. 7e#o*lo!in -e(us "n "e+s #e#ili9i

n(i "n 7!A&, &&2, n(i Q 7!A&, &Q&2 "n n(i 7!A&, &&2.

M


17/73

G#! &.1. Mu(si *en 0" (lse#i Ku9e(i et al, &%%&2

Mu(si 0" Tlse#i Be( #eli0u(i "elesi *en *lo!in, #u(si "eh

0o#o(o, 0en*hen(in #u(si "n #u(si linn/. Te"0( el(i- se"i9i( #u(si

0" =Thlsse#i. Pen/e!! u(# "lh (e"0(n/ 9e(i"9sei#!n*n

n(i *lo!in. P" su#su# (uln* #u(si (lse#i #en**n**u 0e#(n*n sel

"h #eh, sehin** (i"9 e-e9(i-n/ ei(o0oiesis 9i!( hi0e9(i- su#su#

(uln*, (e"0( 0ul se"i9i( e(i9ulosi( "n ne#i !e(. P" (lse#i !e(

(e"0( 9ele!ihn n(i *lo!in =/n* el(i- (eh"0 = "n =*lo!in (e(#es=

*lo!in 42 (e!en(u9, "n ini !ein(e9si "en*n #e#!n ei(osi( sehin**

#e#0e0en"e9 hi"u0 ei(osi(, /n* #en*h 9e ne#i "n #enin*9(9n

0o"u9si e/(hoi". Rn(i *lo!in ="i0o"u9si "l# )u#lh /n* no#l,

sehin** #en/e!!9n 0enin*9(n 7! F & &2. Rn(i Q=*lo!in )u*

6


18/73

"i0o"u9si "l# )u#lh no#l, 7! A& #enin*9( & Q&2 "i =Tlse#i. P"

=(lse#i (e"0( le!ih se"i9i(=*lo!in n(i "n =!ele!ihn "n n(i =

*lo!in. Kele!ihn n(i ini #e#!en(u9 h! B( 42 "l# 9ehi"u0n )nin "n

7! 7 42 se(elh lhi. Te(#es !no#l ini (i"9 #e#(i9n (e(0i

#en*9i!(9n he#olisis e(s


19/73

0e(u#!uhn (eh#!(, 0e#!en*99n 0eu(, "n 0en/9i( 9unin*. Tn0

0en*o!(n, li#0, h(i, "n )n(un* #e#!es, "n (uln* "0( #en)"i (i0is,

0uh, "n


20/73

!en(u9 PR. Re*i#en (n-usi s*sen ,&%1%2.

G#! &.5. 9o#0li9si n9 "en*n (hlsse#i 7e#n hu"/. &%%$2

Psien (lse#i en(n (eh"0 in-e9si 9i!( -9(o 0en/9i(n/ #u0un

9i!( 0en*o!(n. Kele!ihn !esi /n* (e)"i 9i!( (ns-usi !euln*

#e#0en*uhi sis(i# i#un, #ene9n 9si 9e#o(9sis -*osi(osis, #i9o!iosi"l

leu9osi( #ononu9le "n 0oli#o-onu9le. Penuln in-e9si #ellui (ns-usi

se0e(i ius he0(i(is, 7I "n M #eu09n 9o#0li9si (ns-usi /n*

"i(9u(i. In-e9si ius he0(i(is /n* "i(ul9n #ellui (ns-usi n( lin

he0(i(is A, 7e0(i(is B, he0(i(is "n he0(i(is D. he0(i(is #un*9in

#eu09n 0en/e!! u(# siosis he0(i(is 0" 0sien (lse#i /n* #en"0(

3


21/73

(ns-usi. In-e9si !9(ei S( pneumoniae #eu09n 0en/e!! (e!n/9

!9(ee#i (eu(# 0" 0sien (lse#i /n* #en)lni o0esi s0lene9(o#i.

Os(eo#ieli(is /n* (e)"i 9i!( in-e9si (uln* /n* #en*l#i in-9 !isn/

"ise!!9n oleh s0esies Sl#onell. Kuli(s hi"u0 /n* en"h 0" 0sien

(hlsse#i #/o !e9i(n "en*n !e!*i #


22/73

"i(ns-usi9n #en*n"un* &%%=&$% #* !esi 0e uni(. Oleh 9en i(u, 0sien

"en*nTlse#i #/o TM2 #enei# &=4 uni( "h 0e !uln #e#ili9i

su0n (hunn $%%%=1%.%%% #* "i !esi (u %,5=%,6 #* C 9* 0e hi. Tu!uh

#e#ili9i #e9nis#e un(u9 #e#!un* 9ele!ihn z( !esi ini. Selin i(u, 0sien

"en*n Tlse#i M/o "i(n"i "en*n ei(o0oiesis (i"9 e-e9(i- #en/e0

9ele!ihn z( !esi. Se)u#lh !esi "i hsil (ns-usi /n* (i"9 "io!(i, 9n

#en/e!!9n 9eus9n 0" h(i, o*n en"o9in, "n /n* 0lin* 0en(in* 9e

)n(un*. P" n9 "en*n Tlse#i M/o, (n0 9elsi !esi /n* e-e9(i-,

9e#(in (e)"i "i **l )n(un* (u i(#i, !isn/ 0" 9hi #s 9n9=

9n9 (u 0" usi e#) An*elu


23/73

Kompleks

Besi-kelasi

Klirens

plasma

Absorpsi

Ekskresi besi

Dosis terapi

Rute

Percobaan

Efek

samping

20 menit

Tidak ada

Urin dan fekal

40mg/kgbb

Parenteral

40 ta!"n

#t$t$k%i%ita%&

t$k%ik retina&

defek

'ert"mb"!an&

abn$rmalita%

t"lang dan

kartilag$

53(166 menit

P"n)akn*a 45

menit

Urin

75(100

mg/kgbb

#ral

20 ta!"n

+gran"l$%it$%i

%& artr$'ati&

de,%ien%i

%eng&

gangg"an

ga%tr$inte%tina

l

1(16 -am

P"n)akn*a 1(

2-am

.ekal

20(30

mgkgbb

#ral

5 ta!"n

"am k"lit&

gangg"an

ga%tr$inte%tin

al& kenaikan

"re"m

kreatinin

Seu# -ei(in se


24/73

!esi 9e "l# )in*n. Di9en9n !esi !ei9(n "en*n 0o(ein in i, #9

)in*n (ese!u( 9n (elin"un*i "i e-e9 (o9si9 !esi (ese!u(. Psien "en*n

(n-susi "h !euln* #enei# le!ih !n/9 !esi /n* !is "ii9( "en*n

(ns-ein. Sehin** 9" !esi !e!s "l# "h 9n #enin*9(. Besi !e!s ini

"ise!u( "en*n NTBI "on#Transferrin $ound Iron2 /n* se


25/73

"lh e9(n 0" -se 9u( /n* !isn/ 9n #u"h ni9 0" 0en/9i( h(i,

in-e9si "n 0oses in-l#si. P" (i0 0sien, seu# -ei(in sehusn/ "i0ei9s

se


26/73

0" 0sien "en*n (hlsse#i /n* (e*n(un* 0" (ns-usi, "e-ei0one

#e#!n(u #enin*9(9n 0o*nosis 9eseluuhn 0sien (ese!u(. 7l ini el(i-

!i9 "i(olensi, "0un e-e9 s#0in* "en*n *e)l *s(oin(es(inl #en)"i e-e9

s#0in* /n* 0lin* u#u#. A*nulosi(osis %,$2, neu(o0eni 32,

(o#!osi(o0eni hin** 4$2 "n (o0(i &%2 "lh e-e9 s#0in* /n*

0lin* 0en(in* "n #un*9in #e#elu9n 0en*hen(in (e0i. Pe#n(un !e9l

)u#lh "h "ie9o#en"si9n un(u9 0sien 0" (e0i "e-ei0one. De-ei0one

"lh o!( 9elsi ol 0e(# /n* "i0e9enl9n un(u9 9e0en(in*n 9linis.

O!( ini "0( #en*i9( !esi 0" 9e"n !esi /n* #enu#0u9 "n ##0u

#en*elu9n sis !esi 9elu "i (u!uh. Pen**unn 0" #nusi

"i0e9enl9n 0e(# 9li (hun 138' ?#u,&%1&2.

De-ei0one "lh o!( "en*n s(u9(u !i"en(( h/"o/0/i"inone /n*

se


27/73

1%% #* C 9* C hi. Dosis hus "isesui9n !e"s9n es0on 0sien "n (u)un

(e0i. Be!e0 s(u"i #enun)u99n !h+ "osis '$ #* C 9* C hi "e-ei0one

"lh se!n"in* "en*n $% #* C 9* C hi "e-eo#ine "l# #en


28/73

G#! &.$. F#9o9ine(i9 "e-ei0one "n "e-eo9si#in 7o--!n", &%1&2.

De-ei0one


29/73

"i0e9i9n "l# )n*9 0en"e9 "en*n #en*u9u 9esei#!n*n !esi !esih,

"n "l# )n*9 0n)n* "en*n #en*u9u 9onsen(si -ei(in seu# "n

9onsen(si !esi "l# !e!*i o*n, (eu(# "i h(i "n #io9"iu#.

De-ei0one (elh (e!u9(i e-e9(i- 0" 0enuunn (u s(!ilissi 9onsen(si

seu# -ei(in. 9onsen(si -ei(in seu# #oni(oin* "i +9(u 9e +9(u (elh

(e!u9(i !e*un un(u9 (en" es0on (eh"0 (e0i 9helsi 0" 0sien. Magnetic

resonance imaging )n(un* MRI2 T& W (elh (e!u9(i #en)"i 0en**n(i /n*

!i9 un(u9 #en*u9u 9onsen(si !esi )in*n. Telh (e!u9(i "i !e!e0 s(u"i

/n* "e-ei0one se


30/73

9elsi "n 0enuunn )u#lh


31/73

se#en( 9e(inin seu#, n/ei 0un**un* "n u#. De-esio "lh 9elsi

!esi ol /n* "i#uinu# se9li sehi /n* !n/9 "i*un9n "l# 0en*eloln

0sien "en*n he#osi"eosis (ns-usionl. D( "i !e!*i u)i 9linis

#enun)u99n !h+ "osis "e-esio "i &% #* C 9* C hi #ens(!il9n 9"

-ei(in seu# "n 9onsen(si !esi h(i, se"n*9n "osis 5%=4% #* C 9* C hi

#en*un*i 0#e(e ini "n #en


32/73


33/73

#/oi(s #en*hiln* se


34/73

25 Pena!uh Penum$ukan Besi Te!ha'a$ Funsi *ati

7(i "lh (e#0( 0en/i#0nn u(#


35/73

"en*n in-e9si 7 se


36/73

#enun)u99n 0enin*9(n isi9o 9o#0li9si Tin*9( LI 1$ #* Fe C * 9ein*

!e( /n* "i9i(9n "en*n 0enin*9(n isi9o 9e#(in "ini, (in*9( enzi# h(i

/n* !no#l, "n 0e9e#!n*n -i!osis h(i. Mni-es(si in-e9si 7 0"

(hlsse#i #en/eu0i *#!n he0(i(is 0" n9 se


37/73

Pen"r"nan f"ng%i im"nita% %el"lar

Pen"r"nan f"ng%i makr$fag Peng!ambatan e,%ien%i kl$n T! 1 dan T

Pen"r"nan mat"ra%i 'r$lifera%i %el T limf$%it

Pen"r"nan f"ng%i im"nita% !"m$ral

Tlse#i "0( !e9e#!n* #en)"i -i!osis h(i 9en 0enu#0u9n

9" !esi "l# h(i "n in-e9si ius he0(i(is . S(u"i


38/73

"n !is, (e#su9 (in**in/ )u#lh 0us( /n* "i0ilih 0sien "l# s(u"i

#ul(i


39/73

!esi (u!uh 0" 0sien (lse#i #/o "n 0sien he#o9o#(osis hee"i(e "n

"i)"i9n in"i9(o /n* le!ih 9u( un(u9 #enili 0enu#0u9n !esi. Penilin

/n* 9u( 9" !esi (u!uh "0( #e#!n(u 0e#n"un (e0i 9elsi, sehin**

#en*un*i (o9sisi(s "es-eio9s#in. A9n (e(0i !io0si h(i #eu09n

0ose"u insi- ,"n !e!e0 0sien #un*9in (i"9 se(u)u "en*n 0ose"u

!io0si. Men*i"en(i-i9si 0sien /n* !eisi9o (in**i (e9en 9o#0li9si /n* -(l

"i 9ele!ihn z( !esi #eu09n (u)un 0en(in*. Pe#ei9sn his(olo*is h(i

#e#!ei9n in-o#si "n/ 9e"n he#osi"eosis "n -i!osis he0(is.

Se#en( 0e#ei9sn LI Liver Iron Concentration2 (i"9 (ese"i "i !e!e0

(e#0(, 0e#!*in (in*9( he#osi"eosis "lh 0en*u9un /n* !e*un "l#

hu!un*n /n* !i9 "en*n 9" LI. 7e#osi"eosis "e)( 4 sein*

"ihu!un*9n "en*n 9e"n LI /n* le!ih "i 1% #*C * !e( 9ein* "n

0sien (ese!u( #e#!u(uh9n (e0i 9elsi !esi. Ke"n -i!osis he0(is "lh

9un


40/73

u#u 5=15 !uln2. Di#uli "ispatium +isse"n !eln)u( ne9osis he0(osi(.

Fi!o*enesis +l ini (i"9 "0( "ilih( "en*n #i9os9o0


41/73

+9(u (e(en(u, NTBI non Transferin $inding Iron2 #en)"i )lu l(en(i- /n*

0en(in* s( " z( !esi #ele!ih 90si(s "i (ns-ein un(u9 #en*i9(.

7e0(osi( sen"ii #e#ili9i 90si(s !es un(u9 #en/i#0n 9ele!ihn z( !esi.

Ke!n/9n !esi "isi#0n "l# !en(u9 -ei(in, /n* "0( "i#o!ilissi !il

"i0elu9n (e#0( lin "l# (u!uh. A9hin/, hsil 9ele!ihn z( !esi !es=

!esn "i h(i #en/e!!9n he0(o(o9sisi(s /9ni he0(i(is #en/e!!9n

-i!osis "n siosis. O*n h(i (e9en NTBI non Transferin $inding Iron2 le!ih

"hulu "i0" )in*n lin 9en si9ulsi 0o(l 0e(# 9li #ele+(i h(i.

N#un, )in*n lin #e#ili9i 0en/e0n NTBI 9e(i9 NTBI " "i "l#

0ls#. Pe0(i" h(i /n* !en# hepcidin#en*(u 0en/e0n z( !esi usus

"n 0ele0sn !esi "i sel=sel 0en/i#0nn "en*n #en*i9( -eo0o(in

#en/e!!9n in(enlissi "n "e*"si, sehin** (e)"i e-e9 0en*h#!(n

0" 0en*elun !esi "i (u!uh. P" 9on"isi -isiolo*is, 0o"u9si hepcidin"i(u

se


42/73

G#! &.6. ?lu e*ulsi sis(e#i9 he0


43/73

+9(u $ s#0i 1% #eni( "i 0e#!ein ol. Konsen(si seu# 0un


44/73

Pa%ien Tala%emia ma*$r 81 ta!"n

Tran%f"%i %el dara! mera! r"tin

Pen"r"nan ke)e'atan eritr$'$ei%i%

Penimb"nan be%i di!e'ar

adar !e'%idin meningkat

i%$%$mal !em$%iderin meningkat 'ada %el k"'fer dan !e'at$%it

ier ;n-"r*

Deferiprone

Berat m$lek"l < 139

;katan kela%i be%i < 1:3

Deferasirox

Berat m$lek"l < 373

;katan kela%i be%i < 1:2

Penignkatan =>#T& =>PT

dan >amma >T

ama min"m $bat

e'at"!an min"m $bat

=tat"% >i?i

@"mla! tran%f"%i

eterangan

: ana! 'enelitian

: Aariabel 'eran)"

D. Ke!anka k&nse$

Dl# 0eneli(in ini (e"0( i!el !e!s /i(u o!( 9elsi !esi

De-esio "n De-ei0one, se"n*9n i!el (e*n(un* n/ "lh

55


45/73

9" SGOT% SGPT "nGamma GT(F9(o 0en


46/73

0eneli(in "i#n 0eneli(i #el9u9n o!sesi (u 0en*u9un i!el 0"

s(u s( (e(en(u Al(s et al, &%112.

B5 Tem$at 'an 7aktu

Peneli(in "il9u9n "i u#h s9i( ". Moe+"i Su9(. @9(u 0eneli(in

"il9u9n 0" !uln ?uni s#0i Dese#!e &%1$

25 P&$u(asi

/5 Po0ulsi (*e( : 0sien (lse#i #/o usi 18 (hun

/n* (elh #en"0(9n (ns-usi sel "h #eh u(in05 Po0ulsi (e)n*9u : 0sien (lse#i #/o usi 18 (hun

/n* (elh #en"0(9n (ns-usi u(in "i u#h s9i( ". Moe+"i

Su9( /n* #en**un9n 9elsi !esi "e-ei0one "n "e-esio

D5 Sam$e( 'an #a!a $emi(ihan sam$e(

Pen*#!iln s#0el "il9u9n se

&2. Nili -ei(in [ 1%%% n*C"l

!. On* (u 0sien #e#!ei9n 0ese(u)un


47/73

Pen*#!iln s#0el "l# 0eneli(in ini un(u9 nlisis #ul(ii(

"i0elu9n )u#lh s#0el "en*n sio (i"9 !oleh 9un* "i $:1.

Di9en9n #eli!(9n & i!el in"e0en"en "i0elu9n s#0el 0eneli(in $

9li )u#lh i!el in"e0en"en /9ni 1%% s#0el.

N#un, "i9en9n (e"0(n/ 9e(e!(sn )u#lh 0o0ulsi 0"

0eneli(in ini #9 0ehi(un*n !es s#0el #en**un9n u#us un(u9

nlisis !ii(, /i(u nlisis /n* #eli!(9n se!uh i!el "e0en"en "n

se!uh i!el in"e0en"en "en*n #en**un9n 0(o9n u#u# !ule of

Thum', /i(u "i*un9n u9un s#0el se!n/9 #ini#l 5% 0sien se(elh

"il9u9n es(i9si "en*n 9i(ei /n* (elh "i(en(u9n. Mu(i, &%1%2

E5 4a!iabe(

1. i!el !e!s : "e-ei0one "n "e-esio

&. i!el (ei9( : 9" SGOT,SGPT "n GAMMA GT

F5 De8inisi &$e!asi&na( 6a!iabe( $ene(itian

1. Ta(asemia beta ma"&!

"lh su(u 9elinn *ene(i9 "i#n (e)"i *n**un 0" sin(esis

n(i "en*n #ni-es(si (e)"in/ ne#i he#oli(i9 9oni9 /n*

#e#!u(uh9n (ns-usi sel "h #eh se0n)n* hi"u0.

0en*u9un : #e#ei9s 9" he#o*lo!in ele9(o-oesis 7!A& "n

7!F2

05 Ke(asi besiO!( un(u9 #enuun9n 9" !esi "l# (u!uh. "e-ei0one "en*n

#ee9 "*n* Fei0o \ "n "e-esio "en*n #ee9 "*n*

E)"e \

95 'i"er in#(ry

"lh su(u 9e"n /n* #en/e!!9n in-ury 0" he0 9en

"n/ 0eni#!unn z( !esi 9onis /n* #en/e!!9n 0enin*9(n

he#osi"ein 0" sel 9u00-e "n he0(osi(.

56


48/73

a& SGOT Serum Glutamate Oxaloacetate Transaminase2

SGOT #en*9(lissi (ns-e s# #ino L=As0(( #en)"i

9e(o*lu(( "n #en*hsil9n *lu(#(. SGOT se


49/73

A"lh 9e((n 0sien "l# 9onsu#si o!( 9elsi !esi. Dihi(un* "i

0esen(se n( )u#lh o!( /n* "i#inu# "i!*i "en*n )u#lh

o!( /n* "i!ei9n Po0e, 133$2.

G5 2a!a $enum$u(an 'ata

Pen*u#0uln "( "il9u9n se(elh se)u#lh s#0el /n* (e0ilih

#enn"(n*ni informed consent, 9e#u"in "il9u9n 0e#ei9sn 9"

SGOT, SGPT "n GAMMA GT 0" s( 0sien "(n* un(u9 (ns-usi.

Peneli(in "0( "il9sn9n se(elh #en"0( 0ese(u)un "i 9o#i(e e(i9 /n*

" "i RS>D ". Moe+"i Su9(.

*5 Iin sub"ek $ene(itian

Peneli(in ini "il9u9n (s 0ese(u)un on*(u (u +li "en*n


50/73

usi, )enis 9el#in, !e( !"n, (in**i !"n, s((us *izi, l# 9onsu#si

o!( 9elsi !esi, 9e((un #inu# o!(. i!el !e!s "i"es9i0si9n

"l# s9l no#inl "e-ei0one (u "e-esio2. i!el (e*n(un*

"in/(9n "l# s9l nu#ei9 9" SGOT,SGPT "n GAMMA GT2.

Pe!e"n 9" enzi# SGOT,SGPT "n GAMMA GT n( "inlisis

"en*n u)i ( in"e0en"en Tu#!el9 et al. &%112.

35 A(u! $ene(itian

BAB I4

*ASIL PENELITIAN DAN PEMBA*ASAN

53

Psien (lse#i #/o usi

18 /n* (elh #en"0(

Ki(ei in9lusi Ki(ei e9s9lusi

Informed concernt

De-ei0oneDe-esio

9"SGOT SGPT "n

G## GT

Anlisis 0e!e"n n( & 9elo#0o9


51/73

A5 *asi( Pene(itian

Su!/e9 0eneli(in (e"ii "i "u 9elo#0o9 /i(u 9elo#0o9 0sien

(lse#i #/o "en*n 9elsi !esi "e-ei0one "n 9elo#0o9 0sien

(lse#i #/o "en*n 9elsi !esi "e-esio. 9elo#0o9 0sien

(lse#i #/o "en*n 9elsi !esi "e-ei0one (e"ii "i 5& 0sien 15

lel9i "n 13 0ee#0un2 se"n*9n 9elo#0o9 0sien (lse#i #/o

"en*n 9elsi !esi "e-esio (e"ii "i 5& 0sien 18 lel9i "n 14

0ee#0un2. Su!/e9 0eneli(in "i#!il se


52/73

?enis 9el#in

Lel9i

Pee#0un

15 &%,52

13 &3,'2

18 &8,12

14 &1,32

%,185

Be( !"n K*2 &6,6 1%,' &5,$ 8,&3 %,586

Tin**i !"n


53/73

GAMMA GT uCl2 &4,$ 14,%8 16,'8 6,81 %,1&

&

%,%1

1

Di (!el 4.& #en**#!9n 9" SGOT, SGPT "n GAMMA

GT 0" 9elo#0o9 "e-ei0one "n "e-esio. K" GAMMA GT

!e!e" se


54/73

K" -ei(in R =%,463

P %,%%1

N 64T!el 4.$. #en**#!9n hsil nlisis 9oelsi S0e#n n(

9" -ei(in seu# 0en"ei( (lse#i #/o "en*n 9" GAMMA GT.

Di"0(9n 9oelsi ne*(i- /n* se"n* =%,4632 /n* se


55/73

in9lusi "n e9s9lusi. P" 0eneli(in ini #en*hsil9n "n/ 0e!e"n

/n* !e#9n n( 9e"u 9elo#0o9 0" 9" GAMMA GT, /i(u

e( &4,$ 14,%8 uCl 0" 9elo#0o9 "e-ei0one "n e( 16,'8 6,81

uCl 0" 9elo#0o9 "e-esio "en*n nili 0%,%11. Di"0(9n

0e!e"n 9" SGOT 0" 9elo#0o9 "e-ei0one "en*n e( $$,'&

4%,&5 uCl "n "e-esio "en*n 4%,$6 11,16 uCl, 9n (e(0i (i"9

!e#9n se


56/73

"en*n nili 0%,%$2. Koelsi 0osi(i- n( 9" seu# -ei(in "n

9onsen(si ALT, se"n*9n 9onsen(si IGF=I !e9oelsi ne*(i- "en*n

-ei(in. 7l ini #enun)u99n 0enu#0u9n !esi "i h(i /n* #en**n**u

-un*si h(i. K" 9onsen(si seu# -ei(in #eu09n 0enilin /n*

0en(in* un(u9 "i!n"in*9n "en*n 9" enzi# h(i, sehin** 9eni9n

9" -ei(in seu# #eu09n 0e"i9(o in"e0en"en 9eus9n h(i.

Be*unn un(u9 #en*i"en(i-i9si 0sien "en*n isi9o s(e(ohe0(i(is "n

-i!osis Soli#n et al%&%142.

Ke##0un !esi un(u9 (eli!( "l# e9si e"o9s "0(

#en*9i!(9n (o9sisi(s. Dl# 9e"n 0eni#!unn !esi "i h(i,

9eus9n sel h(i )u* "ise!!9n #!iln NTBI "on#Transferin $ound

Iron2 /n*


57/73

Den*n 0en*o!(n 9elsi !esi se


58/73

"e-eio#ine, "i!ei9n 9elsi !esi "e-esio &%#*C9*!!Chi. Se(elh

0e#9in sel# 1 (hun, "i0(9n 0enuunn si*ni-i9n 9" LI "i

se!elu#n/ e( 18,% J 3,1 #* FeC* !e( 9ein* #en)"i 5,4 #* FeC*

!e( 9ein*. 7l ini #enun)u99n e-e9(ii(s (e0i 9elsi !esi "e-esio

"l# #enuun9n 9" !esi "l# h(i Pi* &%%4, The &%%32.

Di 9oelsi n( 9" SGOT, SGPT "n GAMMA GT

(eh"0 9" -ei(in "l# "h, #enun)u99n 9oelsi se"n* "en*n

nili /n* si*ni-i9n. 7l ini #enun)u99n "n/ 9oelsi, )i9 9"

-ei(in #enin*9( #9 9" SGOT, SGPT "n GAMMA GT )u* i9u(

#enin*9(. 7l ini sesui "en*n 0eneli(in Soli#n (hun &%14, 7l ini

#enun)u99n 0enu#0u9n !esi "i h(i /n* #en**n**u -un*si h(i.

K" 9onsen(si seu# -ei(in #eu09n 0enilin /n* 0en(in* un(u9

"i!n"in*9n "en*n 9" enzi# h(i, sehin** 9eni9n 9" -ei(in

seu# #eu09n 0e"i9(o in"e0en"en 9eus9n h(i. Be*unn un(u9

#en*i"en(i-i9si 0sien "en*n isi9o s(e(ohe0(i(is "n -i!osis.

25 Kete!batasan Pene(itian

Ke(e!(sn 0eneli(in ini "lh 0eneli(in "il9u9n se


59/73

BAB 4

PEN)T)P

A5 Sim$u(an

P" 0eneli(in ini "i"0(9n hsil "i"0(9n 9" SGOT "n

SGPT /n* #enuun 0" 9elo#0o9 "e-esio "i!n"in* 9elo#0o9

"e-ei0one, n#un (i"9 !e!e" se


60/73

95 Pelu "il9u9n 0eneli(in ln)u(n se


61/73

DAFTAR P)STAKA

Ais/i M Tu#!el9 AR. &%%5. Pol Pen/9i( In-e9si P" Tlse#i. Sari

Pediatri, ol $ No.1. 7l# &'=55

Al(s, 7, K/o#n**olo, Mus, DA, Boe"iso, A, Oes#n, IN, I"is, NS.

&%11.+esain penelitian.00.1%4=1&3.'a(amS. Ss(os#oo, S. Is#ele"s2.

+asar#dasar metodologi penelitian linis. S*un* se(o, ?9(.

An*elul*e ], N( D, Tezi A, e(ine N, Ellis G. &%%'. A Rn"o#ize"

on(olle" 1=Ye S(u"/ o- Dil/ De-ei0one Plus T+i


62/73

Gil P(&%%8. Ele(e" G##=Glu(#/l(ns-ese A


63/73

M*sen,. &%1%. Talasemiahl# 68=84. Dl# B. Pe#ono, Su(/o, I

>*sen, E @in"is(u(i, M A!"ulsl#. $uu a-ar hematologi ana.

Pene!i( IDAI, ?9(.

Se(inin*sih. &%%%. Mole


64/73

niSA.

$5


65/73

L#0in 1

FORM)LIR PERSET)3)AN MENGIK)TI PENELITIAN DAN

TINDAKAN MEDIS

Yn* !e(n" (n*n "i !+h ini:

N# : ______________________________

>#u :______________________________

Al#( :______________________________

Tele0on :______________________________

Pe9e)n :______________________________

A"lh on*(uC+li "i:N# :______________________________

>#u :______________________________

?enis Kel#in : LCP

Menen*9n !h+ se(elh #en"0(9n 9e(en*n /n* )els "n len*90

(en(n* (u)un 0eneli(in, #en/(9n !ese"i #en*i9u(i 0eneli(in "n (i"9

9e!e(n un(u9 "il9u9n 0e#ei9sn -isis "n 0en*#!iln "h un(u9

0e#ei9sn 9" SGOT, SGPT "n GAMMA GT

Su9(,

On*(uC+li 0ese( 0eneli(in Peneli(i

S9si ". Rez A!"ussl#

L#0in &

FORM)LIR ISIAN PENELITIAN

N# :__________________________________________

Tn**l lhiCu#u :__________________________________________

?enis 9el#in :__________________________________________

Al#( :__________________________________________

$4


66/73

BBCTBCS((us *izi :__________________________________________

Tlse#i !e( #/o : YC Ti"9

Tn**l "i+( :__________________________________________L# (ns-usi :__________________________________________

?u#lh 9n(on* (ns-usi :__________________________________________

Muli 0e#!ein 9elsi !esi : _________________________________________

?enis Kelsi Besi : De-esio E)"e2 C De-ei0one Fei0o2

An#nesis : Le#h : YC Ti"9

Penuunn BB : YC Ti"9

Te#o : YC Ti"9

Be9ein*( !n/9 : YC Ti"9

Kele#hn o(o( : YCTi"9

Gelish : YCTi"9

Tn" i(l : Te9nn "h :______________________ L)u n"i :______________________

L)u n0s :______________________

Suhu : ______________________

M( I9(ei9 : YC Ti"9

7e0 : Me#!esC(i"9

Lien : Me#!esC(i"9

L!o(oiu# Fei(in seu# : ______________________

GDS : ____________________

K" : SGOT : ______________________

SGPT : ______________________

G## GT : ____________________

MInu# O!( (i0 #in**u n/ :

Custom Tables

jenis chelasi

dferiprone deferasirox Total

Count Table N % Count Table N % Count Table N %

gizi baik 20 31.3% 23 3.!% "3 #$.2%

kurang 12 1.% ! 1".1% 21 32.%

$$


67/73

Custom Tables

jenis chelasi

dferiprone deferasirox Total

Count Table N % Count Table N % Count Table N %

kela&in laki'laki 13 20.3% 1 2.1% 31 "."%

pere&puan 1! 2!.$% 1" 21.!% 33 1.#%

Custom Tables

jenis chelasi

dferiprone deferasirox

(ean (axi&u& (ini&u&

)tandard

*e+iation (ean (axi&u& (ini&u&

u&ur 10.1! 1#.00 2.00 ".1 .!" 1#.00 3.00

jenis chelasi

deferasirox Total

)tandard *e+iation (ean (axi&u& (ini&u& )tandard *e+iation

u&ur 3.!# !.# 1#.00 2.00 ".0$

Custom Tables

$6


68/73

jenis chelasi



)tandard


bb 2#.0# 3.00 !.00 10.$0 23.0 3.00 13.00

jenis chelasi

deferasirox Total


bb .2! 2".$ 3.00 !.00 !.

Custom Tables

jenis chelasi



)tandard


tb 12$.1# 1#".00 #$.00 20.#1 121."" 1.00 $.00

jenis chelasi

deferasirox Total


tb 1$.3 12".30 1#".00 #$.00 1!.13

Custom Tables

jenis chelasi


$'


69/73


)tandard

*e+iation (ean (axi&u&

la&a pe&akaian obat 3.!" #.00 1.00 1.2 3.3" #.00

jenis chelasi

deferasirox

(ini&u&

)tandard


)tandard

*e+iation

la&a pe&akaian obat 1.00 1.21 3.#" #.00 1.00 1."0

Custom Tables

jenis chelasi



)tandard

*e+iation (ean (axi&u&

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jenis chelasi

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Custom Tables

jenis chelasi



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$8


70/73

feritin 33#.31 !#00.00 10.$0 211.2$ 3!"$.$$ $0!.00 1123.0

jenis chelasi

deferasirox Total


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Custom Tables

jenis chelasi



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),-T .$2 23#.00 1!.00 "0.23 "0.# #1.00 1.00

jenis chelasi

deferasirox Total


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Custom Tables

jenis chelasi



)tandard


),T ".31 1!.00 3.00 "1.!" 30.3 $2.00 .00

jenis chelasi

$3


71/73

deferasirox Total

)tandard *e+iation (ean (axi&u& (ini&u& )tandard *e+iation),T 1$.02 3!.3" 1!.00 3.00 33.01

Custom Tables

jenis chelasi



)tandard


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jenis chelasi

deferasirox Total


,/((/ ,T #.1 20.#" #$.00 !.00 11.#"

Custom Tables

ju&lahtransfusi


)tandard

*e+iation

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deferasirox 333.! 2.00 1$.00 1."$

Total 330.# 2.00 1$.00 $$.12

6%


72/73

Mann-Whitney TestRanks

jenis chelasi N (ean ank )u& of anks

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deferasirox 32 32.!2 103.0

Total #"

Test Statisticsa

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Correlations

Correlations

feritin ),-T

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61


73/73

Correlations

feritin ),T

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Correlations

feritin ,/((/ ,T

feritin earson Correlation 1 ."#!99


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