Kepada Yth :
Joint Conference / Laporan Kasus Sulit Unit Non Infeksi
1. 2. . #. %. (. *.
Divisi Divisi Bedah Bedah Sara Saraff RS HAM/FK HAM/FK USU Depart Departeme emen n Radiol Radiologi ogi RS RS HAM/F HAM/FK K USU USU Divisi Divisi Hema Hemato to !n"olo !n"ologi gi Ana" Ana" RS HAM/F HAM/FK K USU Depart Departeme emen n Reha$i Reha$ilit litasi asi Medi" Medi" RS HAM Divisi Divisi &e'rolo &e'rologi gi Ana" Ana" RS HAM/FK HAM/FK USU )nst )nstal alas asii Farm Farmas asii RS HAM HAM Dire"t Dire"t'r 'r +ela +ela,an ,anan an Medi Medi" " RS RS HAM HAM
PERDARAHAN INTRAKRANIAL PADA ANAK HEMOFILIA B SEDANG DENGAN FOKAL SYMPTOMATIC EPILEPSY Penyaji
: i!a"an "an Si!e#a!
Ha!i Ha!i $ T#% T#%
:
K*n+,%tan
: P!*-. d!. Hj. Bida+a!i L,/i+0 SpA1K2
$ Ma!e Ma!ett &'() &'() d!. Ne%%y R*+diana0 M.Ked1Ped20 SpA1K2 d!. Se%3i Na-ianti0 M.Ked1Ped20 SpA1K2 d!. O%#a R. Si!e#a!0 M.Ked1Ped20 M.Ked1 Ped20 SpA
PENDAH4L4AN Hemofi Hemofili liaa didefi didefinis nisi"a i"an n se$agai se$agai pen,a"i pen,a"itt ata' ata' gangg'a gangg'an n perdar perdaraha ahan n ,ang ,ang $ersif $ersifat at herediter a"i$at "e"'rangan fa"tor pem$e"'an -))) ata' ) ata' dise$a$"an oleh m'tasi gen fa"tor -))) 0F. 12 +ada saat ini di"enal 2 $ent'" hemofilia ,ait' hemofilia A "arena "e"'rangan fa"tor -))) 0anti-h 0anti-hemophi emophilic lic factor dan hemofilia B "arena "e"'rangan fa"tor ) 0Christmas 0Christmas factor . .1 Di Asia 3enggara ang"a "e4adian $erdasar"an risi"o adalah 15%6.666 penderita. Ke4adian di )ndonesia se7ara tepat $el'm di"etah'i nam'n diper"ira"an dengan pop'lasi 266 4'ta le$ih terdapat se"itar 2.666 penderita hemofilia B.2 +ada hemofilia B dengan $er$agai ma7am manifestasi perdarahan salah sat'n,a adalah perdarahan intra7ranial dengan prevalensi 128 dengan deng an se$agian "as's se7ara "linis asimtomati". Fa"tor Fa"tor risi"o risi"o ,ang ,ang $erh'$' $erh'$'ngan ngan dengan dengan ter4ad ter4adii hemofi hemofilia lia B ,ait' ,ait' h'$'nga h'$'ngan n ter4adin,a ter4adin,a per"em$angan per"em$angan pengham$at seperti seperti ras ri9a,at "el'arga "el'arga adan,a penderita pen,a"it ,ang sama dan adan,a m'tasi gen fa"tor )# ata' ata' adan,a adan,a fa"tor fa"tor geneti" geneti" 0m'tasi gen fa"tor ) ras ri9a,at "el'arga dan fa"tor non geneti" 0fa"tor im'nologi pem$edahan dan tra'ma fa"tor:fa"tor ,ang $erh'$'ngan dengan pengo$atan 5 'sia pem$erian fa"tor ) se7ara $er"elan4'tan 4enis pem$erian fa"tor ) per'$ahan "onsentrasi fa"tor ). 2 Selain it' pera9atan intensif 0prosed'r 0prosed'r $edah saat p'n7a" a9al pengo$atan dan dosis tinggi fa"tor ) ter"ait dengan risi"o ,ang le$ih tinggi.#
1
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Hemofilia dapat di7'rigai pada ana" la"i:la"i dengan "e4adian perdarahan ,ang s'lit s'lit 'nt'" 'nt'" $erhent $erhenti. i. ;e4ala ;e4ala "linis "linis pada pada hemofi hemofilia lia A dan B s'lit s'lit 'nt'" 'nt'" di$eda" di$eda"an. an. Fre"'en Fre"'ensi si dan $eratn, $eratn,aa perdar perdarahan ahan pada pada hemofi hemofilia lia selal' selal' $erh'$ $erh'$'nga 'ngan n dengan dengan nilai nilai plasma dari fa"tor -))) ata' ). Diagnosa pada hemofilia $erdasar"an gam$aran "linis dan pemeri"saan la$oratori'm.%
Tujuan penulisan ini adalah untuk melaporkan satu kasus perdarahan intracranial pada seorang anak laki-laki berusia 2 tahun 7 bulan dengan hemofilia B sedang.
KAS4S RS la"i:la"i 2 tah'n * $'lan s'"' $ata" mas'" "e RSU+ HAM Medan pada tanggal 1 Fe$r'ari Fe$r'ari 261* dengan dengan "el'han le$am pada anggota anggota gera".
: 1 gelas "e7il tiap "ali m'ntah saat ini m'ntah s'dah mem$ai". Ri9a,at pen,a"it ,ang sama dialami pada sep'p' pasien 0"epona"an )$' nam'n $el'm $ero$at hingga saat ini. Ri9a,at le$am:le$am disadari )$' pasien se4a" pasien $er'sia $ er'sia 1 tah'n le$am ter4adi saat pasien ter4at'h ter4at'h dan ter$ent'r. Ri9a,at mimisan dan BAB hitam tida" di4'mpai. Ri9a,at im'nisasi "esan tida" leng"ap.+asien mer'pa"an r'4'"an dari RS S9asta dira9at selama hari se$el'm dir'4'" dengan sang"aan "elainan perdarahan ri9a,at pema"aian o$at 5)n4e"si -itamin K in4e"si seftria"son 2%6 mg/12 4am in4e"si 3ransamin 1%6 mg/ 4am in4e"si de"sametason 2 mg/ 4am m'ltivitamin sir'p oral dan domperidon sir'p oral.
Silsilah Kel'arga
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Keterangan 5 5 la"i:la"i 5 peremp'an 5 penderita hemofilia la"i:la"i 0pasien
PEMERIKSAAN FISIK Kesadaran "ompos mentis $erat $adan 16 "g tinggi $adan 1 7m BB/3B5 :2?@:s7ore?: temperat'r temperat'r (.' Keadaan 'm'm 5 sedang "eadaan pen,a"it 5 sedang "eadaan gi@i 5 "'rang. Di4'mpai anemia i"teri" 'dem dispn' dan sianosis. Stat's lo"alisata 5 Kepala Kepala
5 U"'ran U"'ran 5
5
mesosefal
U$'n:'$'n
5
tert't'p
Mata Mata
5 s"ler s"leraa tida" tida" i"te i"teri ri" " "on4' "on4'ngt ngtiv ivaa p'7at p'7at 0 0 refle refle"s "s 7aha, 7aha,aa / norma normal l p'pil normal iso"or '4i da,a penglihatan dengan $enda:$enda se"itar tida" ada "e7'rigaan gangg'an vis's
Hid'n Hid'ng g 5
tida" tida" ada dis7 dis7ha harg rgee
3elinga nga 5
tida tida" " ada ada dis dis7har 7harg ge $ent $ent'" '" teli teling ngaa norm normal al
M'l't
stomatitis 0: 7left 0:
5
3enggo nggorro"an o"an 5 ;igi
5
fari aring tida" ida" hipe hiperremi emis tonsi onsill tida tida" " hipe hiperrtrof trofii.
"aries 0:
5 pende" pem$esaran "elen4ar getah
$ening 0: Dada Dada
5 Ben Bent' t'" " nor norm mal al sim simet etri ris s tida" da" ada ada "et "etingg inggal alan an gera gera" " tida" da" ada ada retr etra"s a"si dinding dada hematom pada p'ngg'ng 0 Fre". Eant'ng 5 16# /mnt reg'ler reg'ler desah 0: 0: Fre". nafas 5 26 /mnt reg'ler reg'ler ron"hi 0:
+er't
5 perm'"aan dinding per't le$ih tinggi dari dinding dada $ising 's's terdengar tiap 16:26 deti" s'pel t'rgor dan elastisitas normal tida" tera$a massa/s"i$ala tida" ada n,eri te"an timpani
Anogenital Anogenital
Hati
5 tida" tera$a
5 tida" tera$a
5 An's 5 tida" ada hemoroid hemoroid ata' fis'ra fis'ra
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3D 6/(6 6/(6 mmHg mmHg tampa" tampa" hematom hematom pada dorsal dorsal man's "iri "iri dan "anan "anan hematom pada fossa ante$ra"hii "anan 0 hematom pada t'ng"ai tangan dan "a"i 0
Dia#n*+a Bandin#
::
Hemofilia A ;i@i "'rang
:
Hemofilia B ;i@i "'rang
5
+en,a"it von Gille$rand ;i@i "'rang
Ha+i% La/*!at*!i,6 :
H$ 5 (.# g/dl Ht 5 26 8
:
/B/ /B/&/ &/
:
Ga"t' prot protrom rom$in $in 5 12.I 12.I 05 1#I 1#I a+33 a+33 5 *.%I *.%I 05 05 2.I 2.I
:
Ga"t' "t' tro trom$ m$in in 5 1. 1.I I 051 051. .6I 6I
Dia#n*+a Ke!ja
: Hemofilia A ;i@i "'rang
TERAPI : :
)-FD )-FD D%8 D%8 &a &all 6# 6#%8 %8 6 tet tetes es/m /mnt nt mi"r mi"ro o
:
)n4. )n4. Asam Asam trane" trane"sam samat at 16 16 mg/Kg mg/KgBB BB 0166 0166 mg/ mg/ 4am 4am / )-
:
3ransf ansf's 'sii +R +R ses' ses'ai ai "e$ "e$'t 't'h 'han an
:
Diet Diet MB 1166 1166 ""al ""al denga dengan n 26 26 gr gr pro prote tein in
Ren7ana : :
+emer +emeri" i"sa saan an "ada "adarr fa" fa"to torr -)) -)))) dan dan )
:
Kons Kons'l 'l Div Divis isii Hema Hemato to:o :on" n"ol olog ogii
:
Kons Kons'l 'l Divi Divisi si &'t &'tri risi si dan dan +en,a +en,a"i "itt Meta$ Meta$ol oli" i"
PEMANTA4AN PEMANTA4AN TGL (85&' Fe/!,a!i Fe/!,a! i &'() S5
3 5 (6
BB 5 16 "g
5 Ga Ga4ah $'l $'lat atdag' rang rang" "ap mat mata 5 R / / p'p p'pil iso iso"or "an "anan J "iri "on4'ngtiva palpe$ra inferior p'7at :/: 3elinga 3elinga / Hid'ng 5 dalam $atas normal M'l't 5 darah pada g'si 0:
5 pende" pem$esaran "elen4ar $etah $ening 0:
Dada ada
5 simetris f'siform ormis re retra"si 0: FE 5 / 1 1 reg des desah 0: 0: F+ 5 26 / 1 reg ron"hi 0:
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Anggota gera" 5 nadi 5 / 1 reg t/v t/v 7'"'p R3 ?I tampa" l'"a pada digiti ) pedis "iri perdarahan 0 hematom pada t'ng"ai tangan dan "a"i digiti )) man's "iri 0: A 5 Hemofilia A ;i@i "'rang +5 :
3erpasang 3erpasang three9a,
:
)n4. )n4. Asa Asam m tra trane ne"s "sam amat at 166 166 mg mg / 4am 4am / )-
:
Diet Diet MB 1166 1166 ""al ""al denga dengan n 26 26 gr gr pro prote tein in
Ha+i% La/*!at*!i,6 :
Fa"t Fa"tor or -))) -))) 5 .2 .2 8 0&5 0&5 %%:1 %%:1%6 %68 8
:
Fa"tor )
5 1# 8 0&5 *6:1#68
A 5 Hemofilia B ;i@i "'rang +5 :
terpasang 3hree9a,
:
)n4. )n4. Asa Asam m tra trane ne"s "sam amat at 166 166 mg mg / 4am 4am / )-
:
)n4. )n4. Kofa7 Kofa7tt 0re" 0re"om$i om$inan nan fa"tor fa"tor ) %6 )U/K )U/KgBB gBB/2# /2# 4am
:
Diet Diet MB 1166 1166 ""al ""al denga dengan n 26 26 gr gr pro prote tein in
%66 )U/2# 4am/)-
PEMANTA4AN PEMANTA4AN TGL &( 9 & Fe/!,a!i Fe/!, a!i &'() S5
3 5 ( = * 6
BB 5 16 "g
Kepala 5 Ga4ah Ga4ah $'lat dag' rang"ap mata 5 R / p'pil iso"or "anan J "iri "on4'ngtiva palpe$ra inferior p'7at :/: 3elinga 3elinga / Hid'ng 5 dalam $atas normal
M'l't 5 darah pada g'si 0:
5 pende" pem$esaran "elen4ar $etah $ening 0:
Dada ada
5 si simetris f' f'siform ormis re retra"si 0: 0: FE FE 5 : 166 / 1 1 re reg de desah 0: 0: F+ 5 1: 26 / 1 reg ron"hi 0:
+er't
5 inpe"si 5 pendulous abdomen +alpasi 5 soepel peristalti" 0 normal Hepar /
Anggota gera" 5 nadi 5 : 166 / 1 reg t/v 7'"'p R3 ?I tampa" l'"a pada digiti ) pedis "iri perdarahan 0 hematom pada t'ng"ai tangan dan "a"i digiti )) man's "iri 0: A 5 Hemofilia B ;i@i "'rang
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:
)n4. Kof Kofa7t a7t %6 )U/ )U/Kg$$/2# 4am 4am
%66 )U/2# 4am/) 4am/)- di$eri"an di$eri"an selama selama hari
$ert'r't :
Diet Diet MB 1166 1166 ""al ""al denga dengan n 26 26 gr gr pro prote tein in
Ha+i% La/*!at*!i,6 :
H$ 5 11.2 g/dl Ht 5 %8
:
/B/ /B/&/ &/
:
Kalsi' Kalsi'm m ion 5 1.2 U/< U/< a a 5 .# mg/d< mg/d< &a 5 1( 1( mL/< mL/< K 5 .( mL/ mL/< < l 5 161 mL/<
Ren7ana 5 : Kons'l &e'rologi Ana"
;a"a/an K*n+,% Ne,!*%*#i Ana< 1tan##a% & Fe/!,a!i &'()2 S5
!5 Sens 5 M Kepa epala
BB 5 #% "g
BB/3B 5 1(8
5 Ga Ga4ah $'l $'lat dag dag' ran rang"a g"ap mat mata 5 R / / p'p p'pil iso iso"or "a "anan J "ir "iri "on4'ngtiva palpe$ra inferior p'7at :/: 3elinga 3elinga / Hid'ng / M'l't 5 dalam $atas normal
5 pende" pem$esaran "elen4ar $etah $ening 0: "a"' "'d'" 0:
Dada Dada
5 sime simettris f'si f'sifform ormis ret retra"s ra"sii 0: FE 5 /mnt /mnt reg'l eg'ler er desa desah h 0: F+ 5 26 /mnt reg'ler ron"hi 0:
+er't
5 inspe"si 5 pendulous abdomen +alpasi 5 soepel peristalti" peristalti" 0 normal Hepar /
Anggota gera" 5 nadi 5 /mnt /mnt reg t/v 7'"'p R3 ?I hematom hematom pada t'ng"ai t'ng"ai tangan "a"i dan dahi. 3'ng"ai "anan
"iri
$e$as
$e$as
;era"an
5
$e$as
Ke"'atan
5
normal 0%
normal 0%
normal 0% normal 0%
3rofi
5
e'trofi
atrofi
e'trofi
3on's
5
normal
normal
Klon's
5
:
:
normal
normal
Refle"s Fisiologis5
ter$atas
"anan
no normal
normal
e'trofi normal
normal
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A 5 Hemofilia B ;i@i "'rang sang"aan epilepsi Advise 5
5 +emeri"saan ; Do"'mentasi "e4ang MR) 0tida" tersedia
Head 3 S7an
PEMANTA4AN PEMANTA4AN TGL &= Fe/!,a!i &'() S5
!5 Sens 5 M Kepa epala
BB 5 16 "g
5 Ga Ga4ah $'l $'lat dag dag' ran rang"a g"ap mat mata 5 R / / p'p p'pil iso iso"or "a "anan J "ir "iri "on4'ngtiva palpe$ra inferior p'7at :/: 3elinga linga / Hid'n Hid'ng g 5 dalam dalam $ata $atass norma normall
M'l' M'l'tt 5 dara darah h pada pada g'si g'si 0: 0:
5 pende" pem$esaran "elen4ar $etah $ening 0:
Dada ada
5 simetris f'siform ormis re retra"si 0: FE 5 6 6 / 1 1 reg des desah 0: 0: F+ 5 1 / 1 reg ron"hi 0:
+er't
5 inspe"si 5 pendulous abdomen +alpasi 5 soepel peristalti" peristalti" 0 normal Hepar /
Anggota gera" 5 nadi 5 6 / 1 reg t/v 7'"'p R3 R3 ?I tampa" l'"a pada digiti ) pedis "iri mengering perdarahan 0: digiti )) man's "iri 0: A 5 Hemofilia B ;i@i "'rang Fo7al sei@'re + 5 : 3erpasang 3erpasang three9a, :
)n4. )n4. Asam Asam tran trane" e"sam samat at 1 gr gr / 4am 4am / ))-
:
)n4. Kof Kofa7t a7t %6 )U/ )U/Kg$$/2# 4am 4am
%66 )U/2# 4am/) 4am/)- di$eri"an di$eri"an selama selama hari
$ert'r't 0H :
Diet Diet MB 1166 1166 ""al ""al denga dengan n 26 26 gr gr pro prote tein in
Hasil ; 5 : 3ampa" 3ampa" gelom$ang irama dasar dengan fre"'ensi # = spd $er7amp'r = .% spd. : Ditem'"an Ditem'"an gelom$ang pa"' = om$a" di +arietal "iri 0+ dan Frontal "anan 0F# : 3ida" ditem'"an asimetri ,ang $erarti : +S tida" menim$'l"an per'$ahan ,ang $erarti Kesan 5 ; A$normal Ditem'"an gelom$ang epileptiform di + dan !1
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5 pende" pem$esaran "elen4ar $etah $ening 0:
Dada ada
5 simetris f'siform ormis re retra"si 0: FE 5 6 6 / 1 1 reg des desah 0: 0: F+ 5 1 / 1 reg ron"hi 0:
+er't
5 inspe"si 5 pendulous abdomen
+alpasi 5 soepel peristalti" peristalti" 0 normal Hepar /
)n4. )n4. Asam Asam tran trane" e"sam samat at 1 gr gr / 4am 4am / ))-
:
Diet Diet MB 1166 1166 ""al ""al denga dengan n 26 26 gr gr pro prote tein in
Hasil Head 3: S7an 5
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DISK4SI Hemofilia B adalah "elainan resesif "romosom ,ang dise$a$"an oleh adan,a defe" ata' defisiensi plasma fa"tor ) dan ter4adin,a ins'fisiensi pem$e"'an sehingga ter4adi perdarahan.* +eremp'an $iasan,a se$agai pem$a9a sifat sedang"an la"i:la"i se$agai penderita.1 +ada "as's ini penderita adalah ana" la"i:la"i $er'sia 2 tah'n * $'lan. 3anda dan ge4ala "linis hemofilia B $er'pa perdarahan ter'tama adalah hematom dapat dapat $er'pa $er'pa "e$ir' "e$ir'an an pada pada $er$aga $er$agaii $agian $agian t'$'h dan hemarthrosis hemarthrosis ata' perdarahan ,ang s'"ar $erhenti.1 Unt'" mem'dah"an diagnosis ada $e$erapa tempat perdarahan ,ang ,ang dapa dapatt di4'm di4'mpai pai pada pada hemo hemofi fili lia a ,ait ,ait' ' hemarthrosis hemarthrosis hematom hematomaa setela setelah h tra'm tra'ma a hemat'ria perdarahan pada mem$ran m'"osa 0m'l't gigi epista"sis gastrointestinal perdarahan risi"o tinggi pada sistem saraf p'sat retrofaringeal retroperitoneal perdarahan ,ang dise$a$"an "arena sindroma "ompartemen/"ompresi saraf masa pem$e"'an
meman4ang
masa
protrom$in
normal
masa
trom$oplastin parsial
meman4ang dan masa pem$e"'an trom$oplastin a$normal.1% +ada "as's ini se7ara "linis "linis didapati hematoma setelah tra'ma ,ait' pada saat perdarahan ,ang s'"ar $erhenti setelah tinda"an pengam$ilan sampel darahi. +asien mengala mengalami mi hemato hematom m pada pada $e$era $e$erapa pa $agian $agian t'$'h. t'$'h. +ada pemeri pemeri"sa "saan an la$orat la$oratori ori'm 'm di4'mpai masa protrom$in ,ang normal a+33 ,ang sedi"it meman4ang dan did'"'ng dengan rendahn,a nilai fa"tor ) dalam plasma. Klasifi"asi $eratn,a hemofilia $ergant'ng pada "adar fa"tor ) dalam plasma terdiri dari512 :
Hemofi Hemofilia lia $era $eratt $ila $ila "adar "adar fa"tor fa"tor ) ) "'rang "'rang dari dari 18 dari dari normal normal denga dengan n ge4ala ge4ala perdarahan spontan dapat ter4adi pada sendi dan otot dan $iasan,a di diagnosis saat 'sia 1 tah'n pertama "ehid'pan
:
Hemofi Hemofilia lia seda sedang ng $ila $ila "adar "adar fa"tor fa"tor ) ) $er"isa $er"isarr 1 = %8 "adang "adang ter4adi ter4adi perd perdara arahan han spontan spontan perdarahan perdarahan $erat dengan tra'ma dan pem$edahan pem$edahan $iasan,a $iasan,a didiagnosis se$el'm 'sia %:( tah'n
:
Hemofi Hemofilia lia ring ringan an $ila $ila "adar "adar fa"tor fa"tor ) le$ih le$ih dari dari % = #6 8 ter4adi ter4adi perd perdara arahan han $erat $erat
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+ada "as's dig'na"an hemofilia A dan pen,a"it von Gille$rand Gille$rand se$agai diagnosis $anding terhadap hemofilia B. Hemo Hemofi fili liaa A ,ang ,ang ringa ringan n $ias $iasan, an,aa dite ditera rapi pi denga dengan n desmo desmopr pres essi sin n denga dengan n mening"at"an nilai fa"tor ). +engo$atan optimal pada hemofilia $erat har's men7a"'p fa"tor ) 'nt'" men7egah ter4adin,a perdarahan spontan dan mengem$ali"an f'ngsi normal pem$e"'an setelah ter4adin,a tra'ma dan diperl'"an profila"sis 4ang"a pan4ang. Selain Selain menggan mengganti ti fa"tor fa"tor pem$e"' pem$e"'an an ,ang ,ang "'rang "'rang pera9a pera9atan tan dan reha$i reha$ilit litasi asi 4'ga 4'ga diperl'"an ed'"asi $agi penderita ma'p'n "el'arga.1 +em$erian fa"tor ) se7ara intravena dapat men7a"'p se7ara ade"'at pem$e"'an darah darah sehing sehingga ga men7ega men7egah h ter4ad ter4adin, in,aa perdara perdarahan han sponta spontan. n.* Keefe"tivit Keefe"tivitas as pem$erian pem$erian fa"tor ) dengan $er"em$angn,a alloanti$odi ata' pengham$at ,ang menetralisasi"an a"tivit a"tivitas as fa"tor fa"tor ) 'nt'" 'nt'" menggan mengganti ti"an "an protei protein. n. +en7egahan +en7egahan ter4adin,a ter4adin,a artropati mer'pa"an t'4'an 'tama dari pengo$atan hemofilia dengan mengganti"an defisiensi fa"tor pem$e"'an dengan "onsentrasi $ai" se7ara episodi" ma'p'n r'tin. 3erapi 4ang"a pan4ang terat'r dan r'tin dengan pem$erian melal'i inf's d'a ata' tiga mingg' se"ali mer'pa"an metode ,ang paling efe"tif dan optimal 'nt'" men7egah perdarahan dan "ompli "ompli"as "asi i dan ini digam$ digam$ar" ar"an an se$agai se$agai profil profila"s a"sis. is.*16 +em$erian +em$erian profila"si profila"siss 4'ga mem$eri"an efe" pelind'ng terhadap ter4adin,a inhi$itor ,ang mer'pa"an "ompli"asi $erat ter"ait dengan pengo$atan fa"tor ).*
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+rod'" pengganti fa"tor -))) ini dapat men4adi profila"sis ,ang standar $agi ana" ,ang ,ang deng dengan an hemof hemofil ilia ia $era $eratt 'nt' 'nt'" " men7e men7ega gah h ter4 ter4ad adin, in,aa perd perdar araha ahan n spon sponta tan n dan dan deformitas dini pada sendi.12 Se$'ah st'di randomisasi mem$anding"an pem$erian fa"tor -))) se$agai terapi on demand dan dan profila"sis men,ata"an "eamanan dan "eefe"tifan pem$erian fa"tor -))) se$agai terapi profila"sis dapat meng'rangi ter4adin,a perdarahan di$anding"an se$agai terapi on demand se"itar (6 persen.#* +em$erian terapi fa"tor ) dapat di$eri"an dengan dosis 5 Dosis fa"tor -))) 0)U J 8 ,ang diingin"an 0"enai"an F-))) "gBB 3a$el 3a$el 1. Dosis pem$erian p em$erian fa"tor -))) $erdasar"an 4enis perdarahan12 Keadaan "emarthrosis "emarthrosis
Dosis #6 )U/"g hari 1 "em'di "em'dian an 26 )U/Kg hari 2% sampai f'ngsi sendi normal. Ei"a perl' di$eri"an selama *:16 hari/profila"sis hari/profila"sis
+erdarahan otot ata' s'$"'tan
26 )U/"g $o $oleh di$eri"an setiap hari sampai ada per$ai"an
+en7a$'tan gigi
26 )U/"g se$agai antifi$rinolisis
pista"sis
Dila"'"an pene"anan 1%:26 menit di$eri"an antifi$rinolisis 4i"a gagal $eri"an "onsentrat fa"tor -))) 26 )U/"g
+em$edahan +em$edahan
%6:*% %6:*% )U/"g )U/"g "em'dian "em'dian di$eri"an di$eri"an melal'i melal'i inf's 2:# )U/"g/hari sampai 166 )U/2# hari "em'di "em'dian an 2: )U/"g )U/"g/ha /hari ri selama selama %:* hari hari samp sampai ai %6 )U dan dan pena penam$ m$ah ahan an %:* %:* hari hari sampai 6 )U
+erdarahan iliopsoas
%6 )U/"g "em'dian 2% )U/"g setiap 12 hari
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+em$er +em$erian ian fa"tor fa"tor ) mer'pa" mer'pa"an an terapi terapi ,ang aman aman dan se$agai se$agai pen7ega pen7egahan han ter4adin,a ter4adin,a perdarahan pada hemofilia hemofilia B.* Re"om$inan fa"tor ) se7ara intravena dapat mening"at"an "'alitas hid'p penderita hemofilia A ,ang $erat dan meng'rangi $e$an "el'arga serta pengo$atan medis lainn,a. +ada "as's setelah pem$erian pengo$atan fa"tor ) tida" di4'mpai lagi hematom dan perdarahan lain,a
RINGKASAN 3elah dilapor"an s'at' "as's hemofilia B dengan perdarahan intra"ranial pada ana" la"i: la"i $er'sia 2 tah'n * $'lan. Diagnosa ditega""an $erdasar"an anamnesis pemeri"saan fisi" pemeri"saan la$oratori'm dan pemeri"saan lainn,a. +asien ini masih memerl'"an s'$stit'si fa"tor ) ,ang $er"esinam$'ngan.
DAFTAR P4STAKA 1. ;atot ;atot D Moesli Moesli7ha 7han. n. +em$e"' +em$e"'an an darah. Dalam5+e Dalam5+ermo rmono no HB S'tar,o S'tar,o Ugrasena Ugrasena )D; Gindiast't Gindiast'tii A$d'lsalam A$d'lsalam M pen,'nting. pen,'nting. B'"' a4ar hematologi on"ologi ana". Ea"arta5 )"atan Do"ter Ana" )ndonesia 266(. h.1*#:**. 2. 3anta 3anta9, 9, AA;. Mole7'la Mole7'larr geneti7 geneti7ss of hemophi hemophilia lia A5 7lini7al 7lini7al perspe7t perspe7tive ive.. EMH;. EMH;. 2616N 11516%:11#. 11516%:11#. . Kno$e K Berntorp Berntorp . &e9 &e9 treatments treatments in hemophili hemophilia5 a5 insights insights for for the 7lini7ian. 7lini7ian. 3her 3her Adv Hematol. 2612N 51(%:*%. #. ;o'9 ;o'9 S S -an der der Born Born E; E; -an der der Berg Berg M. 3reat reatme ment nt:r :real ealte ted d ris" ris" fa7t fa7tor or of inhi$i inhi$itor tor develop developmen mentt in previo previo'sl 'sl, , 'ntreat 'ntreated ed patien patients ts 9ith 9ith hemophi hemophilia lia A5 the A&A< 7ohort st'd,. Blood. 266*N 165#(#:%#. %.
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1#. Dehghan M A"htar: A"htar:Danesh Danesh & Mer7hant Mer7hant A3. A3. hildhood o$esit, o$esit, prevalen7e prevalen7e and prevention. &'tr E 266%N #51:. 1%. S'$ard4 S'$ard4aa D ah,ono ah,ono HA Moel,o Moel,o A;. A;. !$esit !$esitas as pada ana". Dalam5 Dalam5 Bat'$ara Bat'$ara E 3rid4a4a B +'l'ngan AB pen,'nting. B'"' a4ar endo"rinologi ana". Eilid 1. Ea"arta5 )"atan Do"ter Ana" )ndonesia 2616.h.%:*2.
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B. C*6p*n C*6p*nent ent *- -*!e#!* -*!e#!*,nd ,nd ?,e+ti ?,e+ti*n *n 1PICO2 1PICO2 +atient 5 penderita hemofilia A ,ang mendapat d'a terapi profila"sis )ntervention/)nde5 pem$erian fa"tor -))) omparison 5 penderita hemofilia A ,ang mendapat terapi profila"sis dan on demand !'t7ome 5 pen'r'nan ge4ala pada penderita hemofilia A ,ang mendapat terapi profila"sis
C. Met* Met*de de pene%, pene%,!, !,+a +ann +enel's +enel's'ra 'ran n dila"' dila"'"an "an dengan dengan "ata "ata "'n7i "'n7i T factor factor #$$$ and hemophilia % and pre!iousl& treated I pada pada mesin mesin pen7ari pen7ari google. google. +en'lis +en'lis menem'"an menem'"an arti"e arti"ell dan A memi memili lih h 1 arti arti"el "el ,ang ,ang dapa dapatt men4a men4a9a 9a$ $ perta pertan,a n,aan an "lin "linis is denga dengan n 4'd' 4'd'll T A randoi!ed coparison of t"o prop#$la%is re&iens and a paired coparison of
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+ada peneli penelitia tian n ini dila"'" dila"'"an an '4i "linis "linis ter$'"a ter$'"a $ai" $ai" peneli peneliti ti ma'p'n ma'p'n s'$,e" s'$,e" mengetah'i oa$at ,ang di$eri"an
=. Apa
Peni%aian te!hadap ha+i% ,ji <%ini+
'n-demand +rofila"sis E'mlah
Sem$'h (( ## 116
Hasil 3ida" 22 2%
E'mlah ( (( 1%
xperimental e!ent rate () * #8 ata' 66# 0pada "elompo" on-demand Control e!ent rate 0R rate 0R J 68 ata' 6 0pada "elompo" profila"sis )elati!e risk reduction 0RRR RRR J 0R:R/R J 06:66#/6 J (8 0ARR %bsolute risk reduction reduction 0ARR ARR J R:R J 6:66# J 2(8 +umber needed to treat 03&3 03&3 &&3 J 1/ARR J 1/62( J # àsetiap mengo$ati # pasien dengan o$at profila"sis diperoleh tam$ahan 1 pasien ,ang sem$'h ata' menghindari tam$ahan 1 pasien tida" sem$'h
Peni%aian te!hadap
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