Lap or ank as u s

SEORANG PENDERITA CALCIPHYLAXIS PADA GAGAL GINJAL KRONIK

Ar v an i t a, J od i S L Di v i s i Gi n j al d anHi p e r t e n s i Bag i an /S M FI l muP e n y ak i t Dal am F K Un u d /RS UPS an g l ahDe n p as ar Emai l : ar v an i t as b@ y ah oo.com
ABS TRACT Cal ci p h y l ax i s , al s o cal l e dcal ci c u r e mi c ar t e r i ol op at h y , i sa r ar eabn or mal i t ywh i chch ar act e r i z e dbyme d i al cal ci cat i on oft h es mal l ar t e r i e san di s ch e mi a oft h es u bcu t an e ou st i s s u e , of t e nl e ad i n gt on e cr os i sofs u bcu t an e ou sf at an ds k i n .I t af f e ct s mai n l ywomanwi t hES RD. A 48 y e arol dwomanwi t hES RD h ada h i s t or yofl on gs t an d i n gES RD wh i chwasbe i n gt r e at e dby h e mod i al y s i sf or 1 3y e ar san dp e r i t on e al d i al y s i sf or 1 2 mon t h s .T wo we e k sp r i or t o ad mi s s i on , s h ecomp l ai n e dof a con s t an t d u l l ach i n gp ai naccomp an i e dbymu l t i p l ecu t an e ou su l ce r sonbot hd i s t al e x t r e mi t i e s . Th es y mp t omsd i dn ot r e s ol v ewi t han t i bi ot i c an dan al g e s i ct r e at me n t . Th es k i nof t h el e gh admu l t i p l ev i ol ace ou sl e s i on swh i chwe r et e n d e r onp al p at i on . Th e r ewasmu l t i p l e p ai n f u l l e s i on swi t hd i ame t e r 6 8 cm, n e cr ot i cu l ce r at e dl e s i on ss u r r ou n dbye r y t h e ma. Labor at or yd at ar e v e al e ds e r u m cal ci u m was1 0 .4 mg /d l ,p h os p h at ewas8.5 mg /d lan dp ar at h y r oi dh or mon el e v e l2, 1 64. P l ai nP h ot o ofabd ome n an d bot he x t r e mi t y r e v e al e dcal ci cat i ons of t t i s s u e .S k i nbi op s ywascon s i s t e n t ofCal ci c Ur e mi c Ar t e r i ol op at h y( CUA) . Hi s t op at h ol og ys t u d i e s s h owe dme t as t at i c cal ci cat i ons u b cu t i s( cu t an e ou scal ci p h y l ax i s )an dar t e r i ol os cl e r os i swi t h i ns u p e r ci al d e r mi s . Th ep at i e n t s wast r e at e dwi t hl ocal wou n dcar e , f os r e n ol 3 x5 0 0mgan du s u al t r e at me n t f orCKD we r econ t i n u e d . Re s p on s ecal ci p h y l ax i s af t e rt h et r e at me n t wasg e t t i n gbe t t e r . Ke y wor d s : cu t an e ou s , cal ci p h y l ax i s , CKD

P ENDAHULUAN Calciphylaxis me r u p ak an man i f e s t as ik e l ai n an k u l i td e n g an k ar ak t e r i s t i kt e r j ad i n y ap r os e sk al s i k as i mi k r ov as k u l e r , t r ombos i sd ann e k r os i sp ad ak u l i t s e r t a 2 j ar i n g anl e mak .1, Br y an t d an Wh i t e me l ap or k ank e j ad i an i n ip e r t ama k al ibe r h u bu n g an d e n g an k e ad aan u r e mi a p ad at ah u n1 898. P ad at ah u n1 981d i l ap or k ank e j ad i an calciphylaxis s e k i t ar5 0k as u sd id u n i a. Calciphylaxis me r u p ak ank e j ad i any an gs an g at j ar an gt e r j ad i d e n g an an g k ai n s i d e n14 % p ad ag ag al g i n j al s t ad i u m ak h i r y an gr u t i n me n j al an ih e mod i al i s at au t r an s p l an t as i 4 g i n j al s e t i ap t ah u n n y a.3, Be be r ap a p e n e l i t i an j u g a me l ap or k ancalciphylaxis d ap at t e r j ad i p ad ak ar s i n oma 42

p ay u d ar a,s i r os i sh e p at i s ,k ol an g i ok ar s i n oma,ar t h r i t i s r e mat oi d , h i p e r p ar at i r oi d p r i me r , systemic lupus erythematous d e n g anat aut an p ad i s e r t ai l u p u sn e f r i t i s . De n g ans e mak i ns e r i n g n y ak e j ad i ancalciphylaxis p ad a p e n d e r i t au r e mi ad al am d u ad e k ad et e r ak h i r mak a ol e h Coat e sd i s e bu t j u g a calcic uremic arteriolopathy.2 M an i f e s t as ik u l i ts an g at k h as be r u p a livedo reticularis like eruption l u asy an g ce p atbe r k e mban g me n j ad ig an g r e n . M an i f e s t as iawalbe r u p al e s is an g at n y e r i ,be r i n d u r as id an be r k e mban g me n j ad iu l s e r as i , n e k r ot i k , d i s e r t ai i n f e k s i s e k u n d e r y an gs e r i n gme n j ad i p e n y e bab u t ama k e mat i an .3 P at og e n e s i s calciphylaxis mas i h be l u m d ap at d i j e l as k an s amp ai s aat i n i . Hal i n i l ah y an g
J Peny Dalam, Volume 12 Nomor 1 Januari 2011

me n y e babk an t i mbu l n y a k on t r ov e r s i d al am h al p e n g obat an s e h i n g g a me n i mbu l k an an g k a mor t al i t as y an gcu k u pt i n g g i .3 Ad an y ap e n i n g k at ank ad ar k al s i u m d anf os f at me r u p ak ans al ahs at uf ak t or r i s i k ot e r j ad i n y a 6 calciphylaxis.5, P r og n os i sbu r u kd an an g k a mor t al i t as d i l ap or k ancu k u pt i n g g i s e ban y ak80 % be be r ap a bu l an s e t e l ah on s e t . P e n y e bab p r i me rk e mat i an k ar e n a 3 i n f e k s is e k u n d e rd ar iu l k u sd an s e p s i s . Be r i k u tak an d i l ap or k an s e bu ah k as u sp e n d e r i t a calciphylaxis p ad a g ag al g i n j al s t ad i u m ak h i r . KAS US S e or an g wan i t a, S u k uJ awa, u mu r 48 t ah u n mas u kr u mah s ak i td e n g an k e l u h an l u k a mu l t i p e l d i s e r t ai n y e r i p ad ak e d u at u n g k ai bawahs e j ak2 bu l an s e be l u m M RS . Lu k ap ad a awal n y ad i k at ak an t amp ak bi r us e p e r t il e bam mak i nl ama d i r as ak an me l e bar , me me r ah d an me n g h i t am. Lu k a d i r as ak an n y e r i s e p e r t i t e r bak ard ar i t u n g k ai bawahs amp ai u j u n gk ak i be r s amaand e n g ant i mbu l n y al u k a. Du a mi n g g u s e be l u m mas u k r u mah s ak i t p e n d e r i t a be r obat k ed ok t e rk ar e n a k e l u h an y an g s ama d an d i k at ak an me n d e r i t ad e r mat i t i ss e r t a al e r g i mak an an . P e mbe r i an s al e p d an an t i bi ot i k t i d ak me n u n j u k k anp e r bai k an . P e n d e r i t a me mi l i k i r i way at g ag al g i n j al s t ad i u m V et causa GNC y an gme n j al an i cu ci d ar ahr u t i ns e j ak t ah u n1 996 d anCAP Ds e j ak1t ah u nt e r ak h i r . Ri way at h i p e r t e n s is e j ak t ah u n1 996 t e r k on t r old e n g an t e r ap i . P e n g obat ant e r ak h i r be r u p a as am f ol at 2 x2 mg , CaCO3 3 x5 0 0mg , ad al at or os1x30mg , cap t op r i l 2 x25mg , p e n g g u n aan h e p ar i ns e j ak h e mod i al i s i sd an CAP D. Ri way atd i abe t e sme l l i t u s ,p e r i t on i t i s ,al e r g imak an an d an t r au ma l ok ald i s an g k alp e n d e r i t a. P e n y ak i ty an g s ama t i d ak d i d ap at k an d al am k e l u r g a. P e mak ai an s t e r oi d , al k oh ol d i s an g k al p e n d e r i t a. P ad a p e me r i k s aan s i ks aatM RS t an g g al23 S e p e t e mbe r 20 0 9 d i d ap at k an k e s ad ar an k omp os me n t i s ,t e k an an d ar ah 1 40 /90 mmHg ,n ad i 82 k al i /

o me n i t ,r e g u l e r ,s u h u ak s i l a 37 Cd an l aj up e r n ap as an 20 k al i /me n i t , be r at bad an 5 8 k i l og r am, t i n g g i 2 bad an 1 60 cm,BM I22, 5 6 k g /m . P ad a p e me r i k s aan mat a d i d ap at k an an e mi a d an THT t i d ak d i d ap at k an k e l ai n an . P ad a l e h e rt i d ak ad a p e mbe s ar an k e l e n j ar d anp e n i n g k at anJ VP .P e me r i k s aanj an t u n gd i d ap at k an i ct u scor d i st i d akt e r l i h at , s u ar aj an t u n g1d an2 r e g u l e r , mu r mu rt i d ak t e r d e n g ar , bat as k an an 3 s e n t i me t e r l at e r alp ar as t e r n alk an an d an bat as k i r i4 s e n t i me t e r mi d k l av i k u l ar i sk i r i . P e me r i k s aan p ar u d i d ap at k an s u ar an af asv e s i k u l e rk an an d an k i r i , t i d ak d i d ap at k an r on k imau p u n wheez ing .P ad ap e me r i k s aan abd ome n t i d ak ad ad i s t e n s i ,bi s i n gu s u sn or mal ,h e p ard an l i e n t i d ak t e r aba t i d ak d i d ap at k an n y e r it e k an , ballotement t i d ak t e r aba. P ad a p e me r i k s aan e k s t r e mi t as k an an bawah d i d ap at k an u l k u sd e n g an mak u l a e r i t e ma d i s e k i t ar n y a u k u r an be r v ar i as id e n g an d i ame t e r6 8 cm, bat asbe r i n d u r as i 0 , 51cm, d i t u t u p i k r u s t at e bal war n ak e h i t amand i s e r t ai n y e r i t e k an , t i d akd i d ap at k an n an ah d an d ar ah . P ad a p e me r i k s aan e k s t r e mi t as k i r i bawah d i d ap at k an u l k u sd e n g an mak u l a e r i t e ma d i s e k i t ar n y a u k u r an be r v ar i as id e g an d i ame t e r6 8 cm, bat asbe r i n d u r as i 0 , 51cm, d i t u t u p i k r u s t at e bal war n ak e h i t amand i s e r t ai n y e r i t e k an , t i d akd i d ap at k an n an ahd and ar ah .P ad ak e d u ae k s t r e mi t ast e r aba p u l s as i p e r i f e rd ane d e ma t i d akd i d ap at k an . P ad ap e me r i k s aan l abor at or i u mt an g g al23 J u n i 3 20 0 9, d i d ap at k anW BC 8, 4 x1 0 /l , Hb 7 , 6g /d l , Hct 22, 0 %,M CV 96, 9 ,M CH 33, 2p g ,t r ombos i t25 8x 3 1 0 /l ,BT 1 me n i t ,CT 8 me n i t , BUN 1 7 0 , 4 mg /d l , k r e at i n i n1 1 , 23 mg /d l , GDS 88, 4 mg /d l , n at r i u m1 31 , 2 mmol /l , k al i u m 4, 5 2 mmol /l , ch l or i d a 89, 7mmol /l , T3 1 , 37 n mol /l ,T4 38, 60 p mol /l ,F T4 4, 95 p mol /l ,TS H 0 , 5 5 , k ol e s t e r ol244mg /d l , HDL 31 , 8 mg /d l , LDL 1 84 mg /d l , t r i g l i s e r i d a1 5 3mg /d l . P ad a p e me r i k s aan t h or ak s AP d i d ap at k an k ar d i ome g al i , at e r os k l e r os i s aor t a d an k on g e s t i f p u l mon u m. Has i l p e me r i k s aan BOF d i d ap at k an k al s i k as i abd ome np ad ar e g i oh i p ok on d r i u mk i r i d an s u p r ap u bi k .

S e or an gP e n d e r i t a Calciphylaxis p ad a Gag al Gi n j al Kr on i k Arvanita, Jodi S L

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Gambar 1 . Has i lp e me r i k s aan f ot o cr u r i sd e k s t r as i n i s t r a AP /l at e r al d i d ap at k an k al s i k as i s of t t i s s u er e g i o cr u r i sk an and ank i r i s e r t a os t e op or os i s .

P ad as aatawalM RS p e n d e r i t ad i k on s u l k an k e bag i an k u l i td an k e l ami nd e n g an d i ag n os i ss u s p e k p i od e r ma g an g r e n os u m DD/ n e cr ot i z i n g v e n ol i t i s d an v as cu l i t i s .T e r ap iy an gd i an j u r k an p ar ace t amol3 x5 0 0 mg ,k l or amf e n i k old e n g an d e s ok s i me t h as on e cr e am 0 , 25 %. Re n can ap e me r i k s aanp e n u n j an gd e n g an bi op s id an g r am. Be r d as ar k an d at ad at a t e r s e bu td i bag i an p e n y ak i td al am p e n d e r i t a d i d i ag n os i s CKD s tV et causa GNC d e n g an CAP D,an e mi a s e d an g n or mok r omi k n or mos i t e rd an h i p e r t e n s it e r k on t r ol , obs e r v as ik ar d i ome g al iet causa s u s p e k HHD DD/ k ar d i omi op at iu r e mi k u m. P e mbe r i an t e r ap iu n t u k p as i e nd i e t230 0k k ald an 5 0g r am p r ot e i n /h ar i ,i n f u s NaCl 0 , 9% 8 t e t e s /me n i t , as am f ol at 2 x2mg , CaCO3 3 x5 0 0 mg , ad al at or os1x30 mg , cap t op r i l 2 x25 mgd an r awat be r s ama bag i ank u l i t d ank e l ami n . P ad ah ar i k e 7p e r awat anp e n d e r i t ad i k on s u l k an k eBag i an Be d ah Kar d i ov as k u l e rd an j awaban k on s u l 44

d ar ibe d ah s ar an u n t u kd i l ak u k an d e br i d e me nd e n g an op t i mal i s as ik e ad aan u mu m p e n d e r i t a. P ad a h ar ik e 8p e r awat an p e n d e r i t ad i be r i k an t r an s f u s iP RC 1 k ol f u n t u kp e r s i ap and e br i d e me n .P ad ah ar i k e 9p e r awat an p e n d e r i t a d i l ak u k an d e br i d e me n . S e t e l ah d i l ak u k an d e br i d e me n s amp ai d e n g an h ar ik e 1 6 p e r awat an p e n d e r i t a mas i hd e n g an k e l u h an n y e r iy an gd i r as ak an s e p e r t it e r bak ar p ad a k e d u a t u n g k ai bawah t e r ap i d i t ambah k and e n g anci p r oox aci n2 x5 0 0mg .P ad ah ar i k e 1 6p e r awat anp e n d e r i t a mas i hd e n g ank e l u h ann y e r i d anh as i l d e br i d e me nd i d ap at k ans u at us t as i sd e r moi d . Be r d as ar k an k e ad aan p as i e n y an g t i d ak me mbai k s e r t a t i d ak d i d ap at k an r e s p on s e t e l ah p e mbe r i an t e r ap imak a d i l ak u k an e v al u as iu l an gd an d i cu r i g ai ad an y a p r os e s calciphylaxis et causa h i p e r p ar at i r oi d s e k u n d e rd e n g ani n f e k s i p ad ap e n d e r i t ai n i . Ke mu d i an d i l an j u t k an p e me r i k s aan p e n u n j an g me l i p u t ik al s i u m, f os f at d ank ad arh or monp ar at i r oi dd anr e n can a bi op s i
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k e e s ok an h ar i n y a. P e n d e r i t a d i l ak u k an bi op s id an r e n can a p e mbe r i an l an t h an u m 3 x5 0 0 mg k e e s ok an h ar i n y a. Dar ih as i lp e me r i k s aan d i d ap at k an k al s i u m 1 0 , 4 mg /d l , f os f at 8, 5mg /d l , h or monp ar at i r oi d2.1 64, p e me r i k s aanbi op s i d e n g ank al s i k as ime t as t as i kp ad a d ae r ahme d i u ms u bk u t i s( cutaneous calciphylaxis) d an ar t e r i ol os k l e r os i sp ad a d e r mi ss u p e r ci aly an gd ap at me r u p ak an man i f e s t as ih i p e r t e n s il ama, d i ag n os i s k l i n i sd e n g an calciphyloides et causa h i p e r t i r oi d i s m s e k u n d e r . P ad a h ar ik e 25 p e r awat an l u k a t amp ak me mbai k ,me n g e r i n gd an p e n d e r i t ad i bol e h k an u n t u k r awat j al and anUS G Dop p l e rd i l ak u k and i p ol i k l i n i k . Be r d as ar k an d at ad at a t e r s e bu t p e n d e r i t a d i d i ag n os i scalciphylaxis et causa s u s ph i p e r p ar at i r oi d s e k u n d e r ,CKD s tV et causa GNC on CAP D d an an e mi as e d an gn or mok r omi k n or mos i t e r d anh i p e r t e n s i t e r k on t r ol ,k ar d i ome g al ie c HHD d e n g an M R r i n g an . P e mbe r i an t e r ap ip ad ap as i e nd e n g an d i e t230 0k k al d an 5 0g r am p r ot e i n /h ar i ,i n f u s NaCl0 , 9% 8 t e t e s / me n i t ,p ar ace t amol3 x 5 0 0 mg , k omp r e sNaCl0 , 9% p ad al e s i , as am f ol at 2 x2 mg , ad al at or os1x30mg , cap t op r i l2 x25mg , l an t h an u mk ar bon at3 x5 0 0mg , 0 0mgd i h e n t i k an . CaCO3 3 x5 P EM BAHAS AN Calciphylaxis at aucalcic uremic arteriolopathy me r u p ak an s i n d r om y an g j ar an g t e r j ad id e n g an k ar ak t e r i s t i kk al s i k as imi k r ov as k u l e ry an gp r og r e s i f d an be r k e mban g me n j ad iu l s e r as imu l t i p e ld an l u as y an gs u k ars e mbu hd i s e r t aii n f e k s i ,n e k r os i sj ar i n g an d an g an g r e n . Ul k u sy an gt i mbu ls e r i n gd i s e r t ain y e r i 1 , 2, 7 y an gd i r as ak ans e p e r t i t e r bak ar . Calciphylaxis s e r i n gt e r j ad ip ad ak on d i s iu r e mi a y ai t up e n d e r i t ag ag al g i n j al k r on i ky an gr u t i nme n j al an i h e mod i al i s at aut r an s p l an t as i g i n j al d anl e bi hj ar an gt e r j ad i 4 p ad ap e n d e r i t ap e n d e r i t ag ag al g i n j al k r on i kt an p ad i al i s i s . Be be r ap ak on d i s i n onu r e mi ay an gd ap atme n y e babk an calciphylaxis ad al ah k an k e rp ay u d ar a ( p e n g obat an k e mot e r ap i ) , s i r os i sh e p at i s , k ol an g i ok ar s i n oma, Crohn s disease, ar t h r i t i s r e mat oi dd ansystemic lupus eritematous 5 ( d i s e r t ai l u p u sn e f r i t i smau p u nt i d ak ) . 3,
S e or an gP e n d e r i t a Calciphylaxis p ad a Gag al Gi n j al Kr on i k Arvanita, Jodi S L

T abe l 1 . Et i ol og i calciphylaxis p ad ak on d i s i n onu r e mi a Et i ol og i Hi p e r p ar at i r oi d i s mp r i me r Ke g an as an Alcoholic liver disease Connective tissue diseases Di abe t e s Chemotherapyinduced protein C and Sdeciency Crohn s disease Os t e omal as i ay an gme n d ap at t e r ap i n ad r op ar i nk al s i u m P OEM Ssyndrome De s i e n s i v i t ami nD Weig ht loss CKD ( bu k anES KD)

Kas u s me mi l i k ir i way at g ag al g i n j al k r on i k d e n g anh e mod i al i s i sr u t i ns e j ak1 2t ah u ny an gl al ud an CAP Ds e l ama 1t ah u nt e r ak h i r . Ul k u sy an gt i mbu l p ad a p e n d e r i t ad i r as ak ans an g at n y e r i d ans e p e r t i t e r bak ar . Ras i o i n s i d e n calciphylaxis p ad a wan i t a :p r i a = 3 :1 . Calciphylaxis d ap att e r j ad ip ad as e mu au mu r d e n g anu s i at e r mu d a 6 bu l and ant e r t u a 85t ah u n . On s e t k e l u h ancalciphylaxis be r ag am d ar i 1bu l ans amp ai 1 2 t ah u ns e t e l ahon s e t g ag al g i n j al s t ad i u m ak h i r ( me d i an2 t ah u n9 bu l an ) .P ad as at up e n e l i t i and i d ap at k ani n s i d e n t e r t i n g g ip ad au s i a mu d ad e n g ant e r ap id i al i s i sj an g k a l ama d e n g anr e r at au s i a 48 t ah u n .6 Kas u sad al ah s e or an g wan i t a be r u s i a 49 t ah u n d e n g an on s e t calciphylaxis y an gt i mbu l1 3 t ah u n s e t e l ahon s e t g ag al g i n j al s t ad i u m ak h i rd i t e g ak k an . Le s i calciphylaxis p al i n gban y akd i d ap at k anp ad a e k t r e mi t asbawah( 90 %) s e d an g k anl e s i e k t r e mi t asat as ( 1 0 %) . Le s i y an gme n g e n ai or g anp r ok s i mal me mp u n y ai p r og n os i sy an gl e bi hbu r u k( 68% mor t al i t as )d ar i p ad a d i s t al ( 23% mor t al i t as ) .T e t ap i We e n i g , et al.3 me n e l i t i t i d akd i t e mu k anad an y ah u bu n g anan t ar al ok as i d e n g an p r og n os i sp ad a 64 k as u s . Kas u s me mi l i k iu l k u s mu l t i p e lp ad a k e d u a e k s t r e mi t asbawah . Ul k u sd e n g an mak u l a e r i t e ma d i s e k i t ar n y ad e n g anu k u r anbe r v ar i as i d i ame t e r6 8cm, 45

be r i n d u r as i0 , 5 1 cm,d i t u t u p ik r u s t a t e balwar n a k e h i t aman d i s e r t ain y e r it e k an s e r t at i d ak d i d ap at k an n an ahd and ar ah .P ad ak e d u ae k s t r e mi t asmas i ht e r aba p u l s as i p e r i f e r . Be be r ap a f ak t orr i s i k o t e r j ad i n y a calciphylaxis me l i p u t i k e g e mu k an , p e mak ai an d e x t r an , i mu n os u p r e s an , war f ar i n , k al s i u m k ar bon at , h i p e r p ar at i r oi dd an h i p oal bu mi n e mi a. P ad ap e n e l i t i an 25 p as i e nd i al i s i sd e n g an calciphylaxis d i t e mu k an be be r ap a f ak t or r i s i k o t e r ban y ak me l i p u t ir as k u l i t p u t i h , k e g e mu k an , p e n u r u n anbe r at bad an , p e n g g u n aan war f ar i nd anh i p oal bu mi n .P ad ap e n e l i t i ank as u s k on t r ol d i d ap at k anp e n i n g k at anf os f at d anal k al i f os f at as ep ad a calciphylaxis. P ad a an al i s i s mu l t i v ar i at d i d ap at k an p e mak ai an k al s i u m k ar bon at be r h u bu n g an d e n g an p e n i n g k at an calciphylaxis. Ke j ad i an calciphylaxis j u g a me n i n g k at p ad a wan i t a d an DM t i p e 1 . P e n e l i t i ant e r ak h i rme n y i mp u l k anp e mak ai anwar f ar i n me n g ak i bat k an t i mbu l n y a d e s i e n s ip r ot e i nC d an S y an g me n y e babk an hypercoag ulable state s e h i n g g a me mp e r mu d ah t r ombos i sp ad ap e mbu l u hd ar ah k e ci l . P e n e l i t i an e k s p e r i me n t alme n y i mp u l k an p e n g g u n aan p r e d n i s on p ad a t r an s p l an t as ig i n j alj u g a me r u p ak an s al ah s at uf ak t or r i s i k o. Be l u m ad a p e n e l i t i an y an g me n y e bu t k an bah wa calciphylaxis d i d as ar i ad an y a k e l ai n anme k an i s meau t oi mu n .
T abe l 2. F ak t orr i s i k ot e r j ad i n y a calciphylaxis10 S ensitiz ingag ents Hi p e r f os f at e mi a Hi p e r k al s e mi a P e n i n g k at anl e v e l p r od u kk al s i u m xp h os p h or u s P e n i n g k at anl e v e l h or monp ar at i r oi d Ak t i v i t asv i t ami nD Challeng ingag ents P r od u kd ar ah( p r ot e i n ) Metallic salts ( iron salts and others) Gl u cocor t i k os t e r oi d Obat s i t ot ox i c/i mmu n os u p p r e s s i v e T r au ma l ok al De s i e n s i p r ot e i nC

P ad a k as u sd i d ap at k an f ak t or r i s i k o me l i p u t i h i p oal bu mi n e mi a, h i p e r f os f at e mi a, h i p e r k al s e mi a, p e n i n g k at an k ad arp ar at i r oi d ,p e n u r u n an be r atbad an , p e mak ai an k al s i u mk ar bon ats e j ak 1 3t ah u ny ag l al u t e t ap it i d ak d i d ap at k an ad an y a r i way atk e g e mu k an , p e mak ai an d e k s t r an s e r t a i mu n os u p r e s an ,d i abe t e s me l l i t u s , p e n y ak i t au t oi mu nd anr i way at t r au ma l ok al .
T abe l 3. M an i f e s t as i k e l u h and ang e j al ap ad a calciphylaxis10 Hyperphospatemiaa Hypercacalcemiab I ncreased calcium x phosphorus productc Hyperparathyroidism S kin lesions on upper extremities S kin lesions on lower extremities S kin lesions on trunk L ocation of skin lesions of extremities Proximal involvement Distal involvement History of corticosteroid treatment History of kidney transplant
a b c

68% 20 % 33% 82% 42% 90 %

68% 32% 49% 38%

P h os p h or u s> 5mg /d l Kal s i u m >1 0 , 5mg /d l Kal s i u m xp h os p h or u s> 7 0mg /d l

P ad ak as u sd i d ap at k an ad an y ah i p e r f os f at e mi a, h i p e r k al s e mi a,p e n i n g k at an Cax P ,h i p e r p ar at i r oi dd an l e s i mu l t i p e l p ad ak e d u ae k s t e r mi t asbawah . P at og e n e s i s calciphylaxis s amp ai s aat i n i be l u mj e l asd anbe l u m ad al abor at or i u mt e sy an gbi s a d i g u n ak an u n t u kd i ag n os i s . Wal au p u n ban y ak p as i e n y an gme mi l i k i p e n i n g k at ank al s i u m, f os f at d anh or mon p ar at i r oi dt e t ap it i d ak me n j ad if u n d ame n t alt e r j ad i n y a calciphylaxis ol e hk ar e n a calciphylaxis d ap att e r j ad i bai kp ad a k e ad aan k ad ark al s i u m d an f os f atn or mal at au p u nme n i n g k at .S e bag i anbe s ar k as u sbe r h u bu n g an d e n g anh i p e r f os f at e mi ak ar e n ak e ad aanh i p e r p ar at i r oi d s e k u n d e rme l al u i me k an i s mep e n i n g k at ank al s i u md an f os f atd ar it u l an g . P e n e l i t i an t e r ak h i rme n y i mp u l k an bah wa calciphylaxis be r h u bu n g and e n g anp e n i n g k at an h or mon p ar at i r oi d . Ad an y a g ag al g i n j al k r on i k
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me n y e babk an f os f at t i d ak d ap at d i e k s r e s id e n g an bai ks e h i n g g ak ad arf os f atme n i n g k atd id al am d ar ah . Kad ar v i t ami nD y an gr e n d ah p ad a g ag al g i n j al k r on i kd an ak i batp e n y e r ap an u s u sy an gt e r g an g g u me n y e babk an t u l an g me n j ad ir e s i s t e np ad a h or mon p ar at i r oi ds e h i n g g ak e l e n j arp ar at i r oi dbe r u s ah au n t u k me n g h as i l k an h or mon u n t u k me n i n g k at k an k ad ar k al s i u m d al am s i r k u l as id ar ah . Kal s i k as it e r j ad i p ad ap e mbu l u hd ar ahk e ci l y an gbe r k e mban gme n j ad i n e k r os i sy an gl u as . P e mbu l u hd ar ah k e ci lt e r s u mbat ol e h be k u an d ar ah y an g me n y e babk an t e r j ad i n y a ar e a 7 , 8 n e k r ot i ky an gl u as . P e n e l i t i an J an n i g an , et al. me n y e bu t k an d u a t i n g k at ant e r j ad ip ad ap r os e scalciphylaxis be r u p al e s i p r i me rt e r j ad ik ar e n ak al s i k as ip ad ap e mbu l u hd ar ah s u bk u t an e u sd anl e s i s e k u n d e r t e r j ad i p ad aj ar i n g and an k u l i t . M as i h be l u md i k e t ah u ibe r ap al ama l e s ip r i me r be r k e mban g bi l a as i mp t omat i s . Le s ip r i me rh an y a be r h u bu n g and e n g anp e r k e mban g anl e s i s e k u n d e r .8 P ad a s t ad i u m awal l e s i calciphylaxis s an g at t i d ak s p e s i k s e h i n g g a me n y e r u p ai g ambar an v as k u l i t i s , systemic lupus erythematous, cr y og l obu l i n , s k l e r od e r ma,disseminated intravascular coag ulation, e mbol i l e makat aue n d ok ar d i t i s bak t e r i al . Calciphylaxis p e r l ud i be d ak an d e n g an u l s e r as iak r alat au g an g r e n e k ar e n ap ad a calciphylaxis p u l s as ip e r i f e rmas i hd ap at d i r as ak an . Di ag n os i s calciphylaxis be r d as ar k an g e j al a k l i n i sbe r u p al e s i k u l i t y an gd i d ap at k anp ad ap e n d e r i t a g ag alg i n j alk r on i k .P e me r i k s aan l abor at or i u ms an g at d i p e r l u k an be r u p a BUN, cr e at i n i n ,k al s i u m, f os f at , al k al i n ef os f at as e ,al bu mi n ,k ad arp ar at i r oi d ,p e n an d a i n amas i ES R d an CRP . P e me r i k s aan r ad i ol og i cr u r i s AP /l at e al me n u n j u k k an g ambar an p r os e s k al s i k as ij ar i n g an be r u p a pipelike artery wal au p u n s e r i n g k al ig ambar an n y at i d aks p e s i k u n t u kp e n d e r i t a calciphylaxis. P e me r i k s aanbone scanningme mp u n y ai s e n s i t i v i t as97 %d al am me n d i ag n os i sk al s i k as i .

P e me r i k s aan bi op s ik u l i ts an g at d i p e r l u k an u n t u kme n e g ak k and i ag n os i scalciphylaxis. Gambar an p at ol og i s y an g k h as d i t e mu k an k al s i u m p ad a p e mbu l u hd ar ah k u l i td an d i t e mu k an k al s i k as ip ad a p e mbu l u hd ar ah k e ci l . P e me r i k s aan h i s t ol og i sd ar i l e s ik u l i t me n u n j u k k an k al s i k as i mi k r ov as k u l e r d e n g an e p i d e r mol i s i si s k e mi k . Ad an y a k al s i k as i p ad a p e mbu l u hd ar ah k e ci ld an r e k an al i s as it r ombi n 9, 1 3 me mp e r k u atd u g aan calciphylaxis.1, P ad a be be r ap a k as u sbi op s i i n s i s i on al s an g at d i p e r l u k ank ar e n a punch biopsi s e r i n gk u r an g ad e k u at me n u n j u k k an p r os e s k al s i k as ik ar e n a k e d al aman j ar i n g an y an g s u k ar d i cap ai .T e t ap i bi op s i k u l i t p ad ap e n d e r i t a calciphylaxis me mp u n y ai r i s i k oy an gt i n g g i k ar e n ad ap at me mp e r l u as i n f e k s i . Di ag n os i scalciphylaxis p ad ak as u sd i t e g ak k an d ar i an amn e s i s k e l u h ant i mbu l l u k a mu l t i p e l p ad ak e d u a t u n g k ai bawahd e n g anr i way at g ag al g i n j al k r on i kr u t i n me n j al an i h e mod i al i s i sd e n g anf ak t or r i s i k op e mak ai an k al s i u mk ar bon atd anh e p ar i n .P e me r i k s aans i kp ad a k e d u a e k s t r e mi t as bawah d i d ap at k an u l k u sd e n g an mak u l ae r i t e ma d i s e k i t ar n y ad e n g anu k u r anbe r v ar i as i d i ame t e r6 8 cm,be r i n d u r as i0 , 5 1 cm,d i t u t u p i k r u s t a t e bal war n a k e h i t aman d i s e r t ai n y e r it e k an s e r t a t i d ak d i d ap at k an n an ah d an d ar ah . P ad a k e d u a e k s t r e mi t asmas i ht e r aba p u l s as ip e r i f e r .P e me r i k s aan p e n u n j an g l abor at or i u m d i d ap at k an p e n i n g k at an k ad ark al s i u m 1 0 , 4 mg /d l( n or mal :8, 2 1 0 , 20 p g / ml ) , f os f at :8, 5 mg /d l( k ad arn or mal2, 7 4, 5 mg /d l ) , h or monp ar at i r oi d2.1 64 ( k ad ar n or mal 1 069 p g /ml ) . P e me r i k s aanr ad i ol og i sBOFs e r t a cr u r i sD/SAPl at e r al me n u n j u k k an ad an y a p r os e sk al s i k as i . P e me r i k s aan bi op s i me n u n j u k k anp r os e sk al s i k as ime t as t as i kp ad a d ae r ahme d i u ms u bk u t i s( cutaneous calciphylaxis) d an ar t e r i ol os k l e r os i sp ad a d e r mi ss u p e r ci aly an gd ap at me r u p ak anman i f e s t as i h i p e r t e n s i l ama. P e n an g an anp ad a calciphylaxis s an g at d i p e r l u k an s e j akd i n i d anbe r s i f at mu l t i d i s i p l i n .P e n an g an anbe r u p a me d i k ame n t os ad anp e mbe d ah an .9

S e or an gP e n d e r i t a Calciphylaxis p ad a Gag al Gi n j al Kr on i k Arvanita, Jodi S L

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T abl e4. T e r ap i p i l i h anp ad a calciphylaxis Nor mal i s as i k al s i u md anf os f at s e r u m P ar at h y r oi d e k t omi T at al ak s an a ag r e s i fp ad ai n f e k s i Me n g h i l an g k ansensittiz ing / challeng ingag ents S y mp at e k t omi Di f os f on at e Ok s i g e nh i p e r bar i k

P e n an g an an me d i k ame n t os a calciphylaxis y an gt e r p e n t i n g ad al ah me n g u r an g if ak t or p e n ce t u s , d i e tr e n d ah k al s i u m d an f os f at ,p e n g g u n aan d i al i s at r e n d ahk al s i u m, p e mbe r i analumunium based phosphat binder, me n or mal k an k ad ark al s i u m d an f os f ats e r t a me n g on t r ol k e ad aanh i p e r f os f at e mi ay an gbe r h u bu n g an d e n g an g ag al g i n j al k r on i k . P e n e l i t i an t e r bar u me mbu k t i k an p e n ambah an f r e k u e n s ih e mod i al i s i s ak an me n g op t i mal i s as iclearance d ap atme n g u r an g i 9 k on s e n t r as i k al s i u md anf os f at . Di e t r e n d ah k al s i u m d an f os f at s e r t a me mi n i mal k anp e n g g u n aand i al i s at d e n g ank on s e n t r as i y an gr e n d ahk al s i u ms an g at d i p e r l u k anp ad ap e n d e r i t a d e n g anh e mod i al i s i s . Kor e k s i abn or mal i t as k al s i u md an f os f at d ap at d i l ak u k and e n g ancar a me n u r u n k ank ad ar 2 Cax Pd i bawah5 5mg/d l .P ad ap e n d e r i t ad i al i s i sk ad ar f os f at y an gd i an j u r k an3, 5s amp ai 5 , 5mg /d l .P e mbe r i an non calcium phosphat binder s e p e r t is e l e v ame rd an l an t h an u m d ar i p ad a calcium phosphat binder d an i n t e r mi t e nh e mod i al i s i sd e n g and i al i s at r e n d ahk al s i u m d i d ap at k ans an g at be r man f aat p ad a be be r ap ap e n d e r i t a h i p e r f os f at e mi a. P e n e l i t i anj u g a me n u n j u k k anp e mbe r i anok s i g e n h i p e r bar i ks an g at be r g u n ap ad ap e n d e r i t a calciphylaxis s e t e l ah d e br i d e me ny an gl u asd an s e be l u md i l ak u k an s k i ng r af t i n g . Ok s i g e nh i p e r bar i k( HBO2) me n g an d u n g O2 1 0 0 % d e n g an t e k an an y an gl e bi ht i n g g id ar i p ad a t e k an anat mos f e r abs ol u t . Me n j ag a ok s i g e n as i j ar i n g an s an g at me mban t ume ce g ahp e r k e mban g anbak t e r i d an me mp e r ce p at p e n y e mbu h anl u k ad e n g anme n i n g k at k an t e k an an ok s i g e np ad a ar t e r id ar ah d an j ar i n g an s e r t a d i f u s i ok s i g e nd ar i j ar i n g ank es e l .P e mbe r i anok s i g e n h i p e r bar i kd ap at me n i n g k at k ant e k an anok s i g e ns amp ai 48

40 0 mmHg ( d e l ap an k al il i p atd i ban d i n g k an t e k an an ok s i g e nr u an g an ) . T e k an an ok s i g e ny an gd i be r i k an d i p e r t ah an k an d i bawah 30 0 at m d e n g an d u r as i d i bawah 2 j am u n t u k me mi n i mal i s as ie f e ks amp i n g . S e mak i n t i n g g it e k an an ok s i g e n y an g d i be r i k an s e mak i nce p atp r os e sp e n y e mbu h anl u k ay an gt e r j ad i . Ke u n t u n g and ar i p e mbe r i anok s i g e nh i p e r bar i kme l i p u t i me mp e r ce p atp r os e s an g i og e n e s i sd an p e n y e mbu h an l u k a, me mp e r ce p at p r os e sf ag os i t os i s , me mbu n u h or g an i s mean ae r ob d anme n s t i mu l as i s i n t e s i sk ol ag e n . Ef e k s amp i n g p e mbe r i an HBO mi op i a r e v e r s i be l k ar e n at ok s i s i t as l e n s a, s ak i t k e p al a, k e j an g , bar ot r au ma d an g e j al ap u l mon al . Vas s a,et al. me r e k ome n d as i k an p e mbe r i anok s i g e nh i p e r bar i ks e bag ai s al ahs at ut e r ap i 1 1 calciphylaxis.10, T i n d ak an p e mbe d ah an me l i p u t i p e r awat an l u k a,d e br i d e me n ,s k i ng r af t i n g at au r e v as k u l ar i s as i . P e r awat an l u k a d ap at be r u p a me n j ag a k e be r s i h an l u k a, p e mbe r i an an t i bi ot i k a s i s t e mi kd an t i n d ak an d e br i d e me nu n t u k me n g h i l an g k an j ar i n g an n e k r ot i k d al am h al i n i t i n d ak anamp u t as i k ad an gbi s ad i l ak u k an . P ad a be be r ap a k as u st i n d ak an d e br i d e me np ad a s aat awal d i p e r l u k anu n t u kme n g h i n d ar i i n f e k s i d ans e p s i s . P ad ap e n e l i t i an64 p as i e nd i d ap at k an1 2 years survival rate s e ban y ak 65 % p ad a p as i e ny an g me n d ap at k an d e br i d e me nd ans e ban y ak27 %p ad ap as i e ny an gt i d ak 1 2 me n d ap at k and e br i d e me n . P ar at i r oi d e k t omi mas i hme r u p ak ant i n d ak any an g k on t r ov e r s i al .P e n e l i t i anme n u n j u k k anh as i l y an gmas i h e k u i v ok al . Be be r ap a p e n e l i t i an me n u n j u k k an ad an y a p e r bai k an r as a n y e r i , me mp e r ce p at p e n y e mbu h an l u k a d an mamp e r p an j an g survival rate s e t e l ah k e l e n j arp ar at i r oi dd i an g k at . P e n e l i t i an y an gt e r ak h i r me n y e bu t k an t i d ak ad a p e r be d aan an g k a s u r v i v al p ad ap e n d e r i t ay an gt e l ahme l ak u k anp ar at i r oi d e k t omi mau p u n t i d ak . P ad a p as i e n d e n g an p e n i n g k at an k ad ar h or mon p ar at i r oi dy an gt i d ak bi s a t e r k on t r ol d e n g an k on s e r v at i f mak a t i n d ak an p ar at i r oi d e k t omi d ap at d i p e r t i mban g k an . P ar at i r oi d e k t omi d ap at me mp e r ce p atp e n y e mbu h an l u k a t e r u t ama p ad a f as e awal . P ar at i r oi d e k t omih ar u sd i h i n d ar ip ad a k on d i s i
J Peny Dalam, Volume 12 Nomor 1 Januari 2011

calciphylaxis y an g t an p a d i s e r t ai h i p e r p ar at i r oi d . P ar at i r oi d e k t omi d i i n d i k as i k anu n t u kp e n d e r i t ad e n g an k ad ark al s i u md anf os f at y an gt i d akt e r k on t r ol d e n g an t e r ap ik on s e r v at i fd an d i al i s i s . M as i h d i p e r l u k an p e n e l i t i an y an gl e bi hl an j u tu n t u k me n i l ai e k as i 1 3 p ar at i r oi d e k t omi . P e mbe r i an an t i k oag u l an u n t u k me n ce g ah t i mbu l n y a be k u an t e r bu k t it i d ak ban y ak me mbe r i k anman f aat .14 P e mbe r i an bi f os f on at d i l ap or k an ban y ak me mbe r i k ank e be r h as i l an . Bi f os f on at me mp u n y ai e f e k i n h i bi t ort e r h ad ap osteoclast s e r t a ban y ak d i g u n ak an u n t u k t e r ap i os t e op or os i s d an Pag et s disesase. Be be r ap ap e n e l i t i anme l ap or k anbi f os f on at me mp u n y ai e f e ky an gme n g u n t u n g k anu n t u kme n ce g aht i mbu l n y a l e s in e k r os i sp ad a calciphylaxis d an calcinosis tumor d e n g an d i s e r t air e s p on i n amas is i s t e mi k . Bi f os f on at me mp u n y ai e f e k i n h i bi t or t e r h ad ap ak t i v i t as mak r of ag d an p r od u k s is i t ok i np r oi n amas is e h i n g g a me mp e r ce p at p e n y e mbu h an l u k a, me n g u r an g ir as a n y e r id an me n u r u n k an k ad ar CRP . M on n e y , et al. me n u n j u k k anbi f os f on at me mp u n y ai e f e kan t i i n amas i mas i h k u r an g j e l as s e l ai n be r p e n g ar u h t e r h ad ap r e abs or p s it u l an g . T e t ap ip e mbe r i an bi f os f on atp e r l u d i s e s u ai k an p ad a g ag alg i n j alk r on i kk ar e n a e f e k n y a 1 5 , 1 6 t e r h ad apme t abol i s met u l an g . P e mbe r i an p r e d n i s on s e bag ai an t ii n amas i mas i hd i p e r d e bat k an . F i n ed an Zach ar i a me l ap or k an bah wa s t e r oi dmu n g k i nbe r g u n au n t u kbe be r ap ap as i e n calciphylaxis t an p au l k u st e t ap is amp ais aati n ibe l u m ad a p e n u l i sy an g me n y ar an k an p e n g g u n aan s t e r oi d 1 7 s e bag ai p i l i h ant e r ap i calciphylaxis. P e n e l i t i an t e n t an g p e mbe r i an cal ci mi me t i k s e bag ai t e r ap i calciphylaxis mas i hs an g at t e r bat as . Di i n d i k as i k an p ad a p e n d e r i t a y an g me n ol ak p ar at i r oi d e k t omiat au p u nd e n g an i n t ol e r an s it i n d ak an p e mbe d ah an . Ef e k s amp i n g be r u p a h i p ok al s e mi a. Obat i n it e r g ol on g aman d an d i t ol e r an s id e n g an bai ks e l ama p e n g g u n aan 1 8 bu l an . Ve l as co, et al. me l ap or k an p e mbe r i an ci n acal ce ts e bag ai al t e r n at i f p ar at i r oi d e k t omi60 1 20 mg /h ar il ama p e r i od e9 bu l an t e r bu k t ime n u r u n k an k ad arh or mon p ar at i r oi d
S e or an gP e n d e r i t a Calciphylaxis p ad a Gag al Gi n j al Kr on i k Arvanita, Jodi S L

d i s e r t aip e r bai k an u l k u s . P ad a k as u sy an gl ai nj u g a d i l ap or k an p e mbe r i an d os i sk e ci lci n acal ce t( 30 mg / h ar i )d e n g an k ad arp ar at i r oi d awal264 p g /mld ap at me n u r u n k an k ad arp ar at i r oi d me n j ad in or mald al am wak t u 20 mi n g g ud i s e r t aid e n g an p e r bai k an u l k u s .18 Wal au p u np ar at i r od e k t omi d ap at me n u r u n k an l e v e l p ar at i r oi dh or mons amp ai n i l ai n ol at aume n d e k at i n ol t e t ap i be l u m ad ap e n e l i t i any an gme l ap or k ans e be r ap a ban y ak p e n u r u n an k ad arp ar at i r oi dd e n g an p e mbe r i an cal ci mi e t i k . Kad ar p ar at i r oi d h ar u sd i p e r t ah an k an k i r ak i r a 1 0 0p g /d l ,d i man a k ad ar p ar at i r oi dy an g t e r l al ur e n d ah be r h u bu n g an d e n g an adynamic bone s e d an g k an bi l a k ad ar p ar at i r oi dy an gt e r l al ut i n g g i ak an me n g h ambatp r os e sp e n y e mbu h an u l k u s . NKF KDOQI j u g a me l ap or k an t e r n y at a d e n g an k ad ar h or mon p ar at i r oi d1 5 0 30 0p g /ml mas i h ban y ak p e n d e r i t ay an gme n g al ami adynamic bone. P e mbe r i an s od i u m t h i os u l f at e i n t r av e n a me n u n j u k k an p e r bai k an d al am wak t u be be r ap a h ar i s amp ai 6 bu l an me n u n j u k k an p e r bai k an l e s id an me n g u r an g in y e r id an i n amas i . Wal au p u ns od i u m t h i os u l f at es e k ar an gs u d ah ban y ak d i g u n ak an t e t ap i mas i hd i p e r l u k anp e n e l i t i anp r os p e k t i f y an gd i p e r l u k an u n t u k k e aman an d an e k as is e bag ai p e n an g an an s t an d ar k ar e n a t e r d ap atp e r be d aan d os i sd an d u r as i p e n g g u n aan . Ci con e , et al. me l ap or k an p e n g g u n aan s od i u m t h i os u l f at e( 25 g i n t r av e n a s e l ama 60 me n i t s e t i ap d u a h ar i )p ad a p as i e n calciphylaxis d e n g an CAP Dd i man ad i d ap at k anp e r bai k ans e t e l ah2 mi n g g u . Dos i ss od i u mt h i os u l f at e ad al ah 25 g /1 , 7 3mp e r s e g i p e rd os i si n t r av e n as e t i ap3 k al i s e mi n g g us e t e l ahs e s i h e mod i al i s i s . Le s id i d ap at k an s an g atj au h be r k u r an g d anr as an y e r i h i l an gs e t e l ah8 bu l an .19 S e l ama p e r awat an k as u s me n d ap at k an t e r ap i me d i k ame n t os a be r u p ad i e tr e n d ahk al s i u md anf os f at d anp e mbe r i annon calsium phosphat binder. T i n d ak an p e mbe d ah an be r u p ap e r awat an l u k ad e n g an k omp r e s NaCl 0 , 9% s e r t ad e br i d e me n . Komp l i k as i calciphylaxis s e r i n g be r ak i bat f at al . Le s is e r i n g k al ibe r u p a u l k u sy an gs u k ars e k al i s e mbu hd an me n j ad ig an g g r e n me n g ak i bat k an s u at u 49

k on d i s is e p s i s . Le s ip ad a ak r alk ad an g me me r l u k an t i n d ak an amp u t as i . P r og n os i sk u r an g bag u sd e n g an an g k ak e mat i an6080% d anve years survival rate d i d ap at k anbe r k i s aran t ar a 3545 %.20 P r og n os i s p e n d e r i t a calciphylaxis s an g at bu r u kd e n g an an g k a mor t al i t as60 80 %. P e n e l i t i an me n y e bu t k an67 %d ar i 47p as i e nd i d ap at k anme n i n g g al . S e bag i an p as i e n me n i n g g alk ar e n a s e p s i sd ar il u k a i n f e k s imau p u nk e g ag al an or g an .P e n e l i t i an Ch an ,et al. me n y e bu t k an l ok as id an p r og r e s i v i t as l e s is an g at me n e n t u k an an g k ak e be r h as i l an . Ad an y ad e t e k s id i n i d an p e n an g an an y an gt e p ats an g atd i p e r l u k an u n t u k me n ce g ahp r og r e s i v i t asd anan g k ak e g ag al anp e n y ak i t i n i .I n f e k s i s e r i n gme n j ad i s u mbe r s e p s i sy an gme n j ad i p e n y e bab u t ama k e mat i anp ad a calciphylaxis.20 RI NGKAS AN

3.

4.

5 .

6.

7 . T e l ah d i l ap or k an k as u ss e or an g p e n d e r i t a g ag alg i n j alk r on i kd e n g an calciphylaxis. Di ag n os i s d i t e g ak k an be r d as ar k an an amn e s i s r i way at g ag al g i n j al d e n g an h e mod i al i s i sr u t i nd an f ak t or r i s i k o, p e me r i k s aan s i k ,p e me r i k s aan p e n u n j an g . S e l ama p e r awat an p e n d e r i t a me n d ap at k an p e n g obat an d i e t35 k k al d an1 , 4g r am p r ot e i n /k g BB/h ar i , i n f u sNaCl 0 , 9% 8t e t e s /me n i t , as am me f e n amat3 x 5 0 0 mg , k omp r e s NaCL 0 , 9% p ad al e s i , as am f ol at 2 x2mg , ad al at or os 1x30mg , cap t op r i l 2 x25mg , l an t h an u m 3 x5 0 0mg s e r t a cal ci u m car bon at 3 x5 0 0mgd i h e n t i k an .S e l ama p e r awat an d i l ak u k an p e man t au an k e ad aan k l i n i s , k e l u h ans e r t al abor at or i u m. P ad ah ar i k e 25p e r awat an k e ad aan k l i n i sp e n d e r i t a me mbai ks e h i n g g ad i i j i n k an r awat j al an . DAF T AR RUJ UKAN 1 . An d r ag u eHJ ,F r az i e rM R,S e l u f fB,S u k iW N. S y s t e mi c cal ci p h y l ax i sr e v i s i t e d . AM JNe p h r ol 1 981 ; 1 : 1 7 7 83. Coat e sT , Ki r k l an dGS , Dy mockRB. Cu t an e ou s n e cr os i sf r om cal ci c u r e mi c ar t e r i ol op at h y . Am JKi d n e yDi s1 998; 32: 38491 .

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We e n i gRH. S e we l l LD, Dav i dM DP , M cCar t h y J T , P i t t e l k ow M R. Cal ci p h y l ax i s : n at u r al h i s t or y , r i s kf act oran al y s i s an d ou t come . JAm Acad De r mat ol 20 0 7 ; 5 6: 5 697 9. Bu d i s av l j e v i c M N, Ch e e k D, P l ot h DW. Cal ci p h y l ax i si nch r on i cr e n al f ai l u r e . JAm S oc Ne p h r ol 1 996; 7 : 97 882. Le v i nA, Me t h a R, Gol d s t e i nM B. M at h e mat i cal f or mu l at i on t o h e l pi d e n t i f yt h ep at i e n tatr i s k ofi s ch e mi c t i s s u en e cr os i s a p ot e n t i al l yl e t h al comp l i cat i on of ch r on i c r e n al f ai l u r e . AM J Ne p h r ol 1 993; 1 3: 4485 3. M az h arAR,J oh n s on RJ ,Gi l l e n D,S t i v e l man J ,Ry an M J ,Dav i sCL,e tal . Ri s kf act or san d mor t al i t y as s oci at e d wi t h cal ci p h y l ax i si ne n d s t ag er e n al d i s e as e . Ki d n e yI n t 20 0 1 ; 60 : 32432. Camp i s t ol J M ,Al mi r al lJ , M ar t i n E, T or r as A, Re v e r t L. Cal ci u mcar bon at e i n d u ce d cal ci p h y l ax i s . Ne p h r on1 989; 5 1 : 5 495 0 . Du h QY,Li m RC,Cl ar k OH. Cal ci p h y l ax i s i ns e con d ar yh y p e r p ar at h y r oi d i s m. Ar ch S u r g 1 991 ; 1 26: 1 21 39. Roe S M , Gr ah am LD, Br ock W B, Bar k e r DE. Cal ci p h y l ax i s : e ar l y r e cog n i t i on an d man ag e me n t . AM S u r g1 994; 60 : 81 6. Le ach RM ,Re e sP J ,W i l mh u r s tP . Hy p e r bar i c ox y g e nt h e r ap y . BM J1 998; 31 7 : 1 1 40 3. De anS M, We r manH. Cal ci p h y l ax i s : af av ou r abl e ou t come wi t hh y p e r bar i c ox y g e n . Vas c Me d 1 998; 3: 1 1 5 20 . Rog e r sNM , T e u be rDJ O, Coat e sP TH. Cal ci c u r e mi c ar t e r i ol op at h y : ad v an ce si np at h og e n e s i s an dt r e at me n t .S e mi nDi al 20 0 7 ; 20 : 1 5 0 7 . Haf n e r J . Ke u s chG, Wah l C, S au t e r B, Hu r l i man n A, Von We i z s ack e rF ,e t al . Ur e mi c s mal l ar t e r yd i s e as e wi t h me d i al cal ci cat i on an d i n t i malh y p e r p l as i a( s ocal l e d cal ci p h y l ax i s ) :A comp l i cat i onofch r on i cr e n al f ai l u r ean dbe n e t f r om p ar at h y r oi d e ct omy . JAm Acad De r mat ol 1 995 ; 33: 95 462.
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Don BR,Ch i nAI .As t r at e g yf ort h et r e at me n t ofcal ci c u r e mi c ar t e r i ol op at h y( cal ci p h y l ax i s ) e mp l oy i n g a combi n at i on of t h e r ap i e s . Cl i n Ne p h r ol 20 0 3; 5 9: 4637 0 . Ce cch i n iM G,F e l i x R,F l e i s ch H,Coop e rP H. Ef f e ct of bi s p h os p h on at e s on p r ol i f e r at i on an dv i abi l i t y of mou s e bon e mar r owd e r i v e d macr op h ag e s . J Bon e Mi n e r Re s1 987 ; 2: 1 35 42. Han af u s aT , Yamag u ch i Y, T e n i M, Ume g ak i N, Ni s h i mu r a Y,Kat ay ama I . I n t r act abl e wou n d s cau s e sby cal ci c u r e mi c ar t e r i ol op at h yt r e at e d wi t h bi s p h os p h on at e s . J Am Acad De r mat ol 20 0 7 ; 5 7 : 1 0 21 5 . F i n e A, Zach ar i as J . Cal ci p h y l ax i s i s u s u al l yn on u l ce r at i n g :r i s kf act or s ,ou t come an dt h e r ap y . Ki d n e yI n t 20 0 2; 61 : 221 0 7 .

1 8.

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Wi l me r WA, M ag r o CM . Cal ci p h y l ax i s : e me r g i n g con ce p t si np r e v e n t i on , d i ag n os i san d t r e at me n t .S e m Di al 20 0 2; 1 5 : 1 7 286. Ci con eJ S ,P e t r on i sJ B,Embe r tCD;S p e ct or DA. S u cce s s f u lt r e at me n tofcal ci p h y l ax i swi t h i n t r av e n ou ss od i u m t h i os u l f at e . Am J Ki d n e y Di s20 0 4; 43: 1 1 0 48. Haf n e r J , Ke u s ch G, Wah l C, Bu r g G. Cal ci p h y l ax i s :a s y n d r omeofs k i nn e cr os i san d acr al g an g r e n ei n ch r on i c r e n al f ai l u r e . Vas a 1 998; 27 : 1 37 43.

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