|HUN00EF|8|EN8| PR|HER

(|P}
M. Sjabaroeddin Loebis, LiIy Irsa, Rita EvaIina
AIIergy ImmunoIogy Division
Pediatrics Departement
MedicaI FacuIty Sumatera Utara University
PEN0AhULUAN
lrurodel|s|ers|, 1953, 8rulor : r|pogarag|oou||rer|a pada
ara| us|a 8 larur
0aroarar |||r|s : |rle|s| re|urer, |ror|s dergar c|r| seoao
l|da| o|asa (oporlur|sl||), l|ora rorra|, r||rooa ||rg|urgar
o|asa respors je|e| lerradap arl|o|ol||
0eja|a yarg ser|rg : d|are |ror|s, repalosp|erorega||,
auloarl|ood| alau perya||l aulo|rur
Terd|r| alas :
- Pr|rer : dasar gerel||a, jararg
- 3e|urder : ser|rg, |arera oeroaga| la|lor sele|ar |ar|r
eo|r dar| 100 oerlu| lP lerdala sarpa| se|ararg
T|rg|al oeral perya||l oervar|as|
Kara|ler|sl|| : perya||l oeru|arg, pers|sler, daya larar
rerurur, dar |ror|s, respors je|e| lerradap arl|o|ol||
A3 : 1 jula, dur|a : 10 jula
Pr|a, War|la, serua urur
8erlu| oeral ser|rg pada us|a ara|
0|agros|s dar perala|a|saraar aWa| perl|rg urlu| cegar
|rle|s| oeru|arg dar |erusa|ar perrarer
PENDAHULUAN......
ET|0L0C|
0ele| gerel||
0oal alau lo|s|r (|rurosupresar, arl||orvu|sar)
Perya||l rulr|s| dar relaoo||| (ra|rulr|s|, del. v|lar|r, del.
r|rera|)
Ke|a|rar |rorosor (de|es| 22q11, lr|sor| 18)
lrle|s| (lrars|er, perrarer)
T|pe, |o|as| , dar orgar|sre peryeoao |rle|s| o|sa
reroarlu urlu| rerelu|ar lerpal |e|a|rar.
1 area ulara ada|ar :
- se| T
- se| 8
- se| PVN
- |orp|erer
CANCCUAN FUNC8| 8|8TEH |HUN YANC 8ER|NC
0EF|8|EN8|
FUNC8| ERLE|hAN
0EF|8|EN8|
0el|s|ers| se| 8 : |rle|s| oa|ler| re|urer seperl| ol|l|s
red|a, preror|a re|urer
0el|s|ers| se| T : |ererlarar rer|rg|al lerradap v|rus,
jarur dar prolozoa
0el|s|ers| lagos|l : |rle|s| oa|ler|, aulo|rur|las
CANCCUAN FUNC8| 8|8TEH |HUN .....
FUNC8| ERLE|hAN
3e| 8 : garopal| roro|ora|
3e| T : |e|eo|rar se| Ts yarg rer|rou||ar |rle|s|
dar perya||l ||rlopro||leral|l
Fagos|l : r|persers|l|v|las, oeoerapa perya||l
aulo|rur
Korp|erer : eder arg|oreurol|| a||oal l|da|
adarya |rr|o|lor eslerase C1
PEHAC|AN 0EF|8|EN8| |HUN
0EF. |HUN N0N 8PE8|F|K
0EF. |HUN 8PE8|F|K
0EF|8|EN8| |HUN N0N 8PE8|F|K
A. 0el|s|ers| |orp|erer ; |orger|la|, l|s|o|og||, d|dapal
8. 0el|s|ers| |rlerleror dar ||soz|r ; |orger|la|, d|dapal
C. 0el|s|ers| se| NK ; |orger|la|, d|dapal
0. 0el|s|ers| s|sler lagos|l : |uarl|lal|l, |ua||lal|l
PEHAC|AN 0EF|8|EN8| |HUN......
0EF|8|EN8| |HUN 8PE8|F|K
A. 0el|s|ers| |orger|la| alau pr|rer
8. 0el|s|ers| |rur l|s|o|og|| ; |erar||ar, us|a larur
perlara, us|a |arjul
C. 0el|s|ers| d|dapal / se|urder : ra|rulr|s|, |rle|s|,
ooal, lraura, l|rda|ar |aleler|sas| dar oedar,
pery|rarar, perya||l oeral, |er||argar lg/|e|os|l,
slres, agarag|oou||rer|a dergar l|rora
0. Al03
A. 0EF|8|EN8| |HUN N0N8PE8|F|K
1. 0EF|8|EN8| K0HPLEHEN
- oerruourgar dergar |rle|s| dar 3
- lurgs| |orp|erer : ourur |urar, opsor|sas|, |erola|s|s,
cegar pery.aulo|rur, e||r|ras| |orp|e|s Ag-Ao
- |eoarya|ar rered|ler
a. 0el|s|ers| |orp|erer |orger|la|
- del|s|ers| |rr|o|lor eslerase C1 arg|oedera rered|ler
- a|l|v|las C1 l|da| ler|orlro|, produ|s| ||r|r perreo|||las
|ap||er
C2a dar C1a d||epas se| rasl |epas r|slar|r d|daerar
de|al lraura
A. 0EF|8|EN8| |HUN N0N8PE8|F|K...
- del|s|ers| C2 dar C1 pery.serupa 3
- del|s|ers|C3 lragrer C5 |erola|l|| l|da| d|produ|s|
|orp|e|s Ag-Ao l|da| dapal d|erdap|ar d|rerorar
gargguar opsor|sas| |rle|s| oeral dar lala|
- del|s|ers| C5 gargguar |erola|s|s rerlar lrdp
|rle|s| oa|ler|
- del|s|ers| C, CZ dar C8 rerlar lrd sepl||er|a
rer|rgo|o| dar goro|o|, derajal |rle|s| oeral lrdp
Ne|ser|a, seps|s, dar arlr|l|s, juga rer|rg|al|ar 0lC
A. 0EF|8|EN8| |HUN N0N8PE8|F|K...
o. 0el|s|ers| |orp|erer l|s|o|og|| : rarya pada reoralus,
|arera |adar C3, C5, dar la|lor 8 ras|r rerdar
c. 0el|s|ers| |orp|erer d|dapal :
- del. C1q,r,s aulo|rur / 3, rerlar |rle|s|
- del. C1 3
- del. C2 ser|rg, geja|a (-), 3
- del. C3 |rle|s| re|urer, 0N |ror||
- del. C5-8 rerlar |rle|s| lerulara Ne|ser|a
- del. C9 jararg, |rle|s| re|urer (-)
A. 0EF|8|EN8| |HUN N0N8PE8|F|K...
2. 0EF|8|EN8| |NTERFER0N 0AN L|80Z|H
a. Korger|la| |rle|s| rororu||eos|s yarg lala|
o. 0|dapal pada ra|rulr|s| prole|r / |a|or|
. 0EF|8|EN8| 8EL NK
a. Korger|la| perder|la osleopelros|s, lg0, lgA dar
|e|erapar auloarl|ood|
o. 0|dapal a||oal |rurosupres| / rad|as|
A. 0EF|8|EN8| |HUN N0N8PE8|F|K...
4. 0EF|8|EN8| 8|8TEH FAC08|T
- R|s||o |rle|s| oeru|arg
- oerruourgar dergar jur|ar relrol||
r|s||o rer|rg|al o||a jur|ar lagos|l < 500/rr
3
- del|s|ers| d|le|ar|ar lerradap se| PVN
a. del|s|ers| |uarl|lal|l : produ|s| alau deslru|s|
produ|s| relrol|| : |erolerap|, |e|er|a, d|sgeres|s
rel||u|er
- deslru|s| : lerorera aulo|rur a||oal ooal lerlerlu
o. del|s|ers| |ua||lal|l
- Crror|c 0raru|oralous 0|sease (C00) : |rle|s| re|urer, X
||r|ed reses|l, 2 larur perlara, dele| relrol||, l|da| rarpu
reroerlu| pero|s|d r|droger / relaoo||l o|s|ger lo|s||
|a|rrya
- 0el. 0|ucose--prosprale derydrogerase (0--P0) : X
||r|ed, |||r|s spl C00, arer|a rero||l||, geja|a ru|a| us|a <
2 lrr, ||rladeropal|, repalosp|erorega||, K08 lerus
rererus |e|uar ca|rar
- 0el. r|e|opero|s|dase (0VP) : |erarpuar ourur relrol||
- 3|rdror Cred|a|-l|gasr| (3Cl) : jararg, |rle|s| re|urer,
p|oger||, progros|s ouru|, relrol|| rergardurg ||sosor
oesar aororra| oersalu dgr lagosor re|epas |s|
lergarggu le|ar & rarcur|ar r||rooa ler|aroal
- 3|rdror Joo : p||e| oeru|arg (l|da| lerjad| |rl|aras|
rorra|, aoses slal||o|o|, e|s|r |ror|s, ol|l|s red|a,
|erola|s|s lergarggu, lg sargal l|rgg|, eos|rol|||a
- 3|rdror |e|os|l ra|as (|azy |eucocyle) : rerlar |rle|s|,
jur|ar relrol|| , respor |erola|s|s dar |rl|aras|
lergarggu
- 0el. adres| |eu|os|l : |rle|s| oa|ler| dar jarur re|urer,
gargguar peryerourar |u|a
. 0EF|8|EN8| |HUN 8PE8|F|K
1. 0EF|8|EN8| K0NCEN|TAL | PR|HER
a. 0el. |rur pr|rer se| 8 (r|pogaragoou||rer|a)gargguar
per|eroargar se| 8, serua lg / |e|as / suo|e|as l|da| ada
- X-||r|ed r|pogarrag|oou||rer|a : 8rulor 1952, rarya
pada oay| larpa| us|a 5- ou|ar saal lg0 |ou ru|a| ,
jar|rgar ||rpo|d rergec||
- r|pogarrag|oou||rer|a sererlara : s|rles|s ler|aroal,
seoao l|da| je|as, del. sererlara se| Tr
- corror var|ao|e rypogarrag|oou||rer|a (Cvl) ; r|r|p
8rulor, |rs|der aulo|rur l|rgg|, jur|ar se| 8 & lg rorra|
lap| |erarpuar produ|s| dar re|epas lergarggu, o|sa
/, o|asarya ru|a| us|a 15-35 larur, serua lg |urarg
- del. lg se|e|l|l (d|sgara-g|oou||rer|a) : salu alau |eo|r
|adar lg , yarg |a|r rorra|
o. 0el. |rur pr|rer se| T rerlar |rle|s|, d|serla| gargguar
produ|s| lg, l|da| ada respors lerradap |rur|sas|
- ap|as| l|rus |orger|la| (s|rdrora 0|0eorge) : seoao (?),
|e|erjar paral|ro|d ler|era, r|po|a|ser|a 21 jar perlara
d|||ul| |e|a|rar jarlurg dar g|rja|, l|da| d|lurur|ar,
pergooalar dergar lrarsp|arlas| l|rus lela|, progros|s
ouru| o||a l|da| d|ooal|
- |ard|d|as|s ru|o|ular |ror|| (KVK) : gargguar lurgs|
se| T se|e|l|l, o|asarya d|serla| d|slurgs| oeroaga|
|e|erjar erdo|r|r, lrarsp|arlas| lrus reroer||ar ras||
oervar|as|
c. 0el. |oro|ras| se| 8 dar se| T yarg oeral
- severe coro|red |rrurodel|c|ercy d|sease (3Cl0)
- s|rdror Neze|ol
- s|rdror w|s|oll-A|dr|cr (wA3) : l|da| rarpu respor
lerradap Ag po||sa|ar|da, rerlar lrdp |e|er|a,
lroroos|loper|, |rle|s| re|urer, lgV , lg0 rorra|, lgA
dar lg , lerap| dgr arl|o|ol|| dar lrarsp|arlas| sulu|
- ala|s|a le|earg|e|las| : aulosora| reses|l, rergera|
saral, erdo|r|r dar vas|u|er. 0era|ar olol l|da|
ler|oord|ras|, d||alas| perou|ur darar |ec||, ||rloper|a,
lgA/lg/lg0 , l|rou| pada us|a < 2 larur
- del|s|ers| aderos|r dear|rase
2. 0EF|8|EN8| |HUN 8PE8|F|K F|8|0L0C|K
- Kerar||ar
- us|a larur perlara
- us|a |arjul
. 0EF|8|EN8| |HUN 0|0APAT | 8EKUN0ER
- Va|rulr|s|
- lrle|s| / 0oal, lraura, l|rda|ar |aleler|sas| dar oedar
- Pery|rarar
- Perya||l oeral
- Ker||argar |rurog|oou||r
- 3lres
- Agarag|oou||rer|a dergar l|rora
Def.kompIemen 2%
Def. granuIosit 18%
Def. seI B & T Def. seI T 10% Def. seI B 50%
The 10 warn|ng 8|gns of P|
1. |grl or rore reW ear |rlecl|ors W|lr|r a year.
2. TWo or rore ser|ous s|rus |rlecl|ors W|lr|r a year.
3. TWo or rore rorlrs or arl|o|ol|cs W|lr ||ll|e ellecl.
1. TWo or rore preuror|as W|lr|r a year.
5. Fa||ure ol ar |rlarl lo ga|r We|grl or groW rorra||y.
. Recurrerl deep s||r or orgar aoscesses.
Z. Pers|slerl lrrusr |r roulr or e|seWrere or s||r, aller
age 1.
8. Need lor |rlraverous arl|o|ol|cs lo c|ear |rlecl|ors.
9. TWo or rore deep-sealed |rlecl|ors sucr as
rer|rg|l|s, osleorye||l|s, ce||u||l|s, or seps|s.
10. A lar||y r|slory ol Pl
0|ACN08|8 |P
10 warr|rg 3|grs eva|uas| pas|er dgr |rle|s| re|urer
R|Wayal :
- orsel us|a
- l|pe |rle|s|
- |epas la|| pusal ler|aroal
- gargguar resp|ralory, 0l, CN3
- r|Wayal |e|uarga
- rea|s| adverse lrd va|s|r r|dup alau lrarslus|
0|ACN08|8 .....
Perer||saar l|s|| :
- Proo|er perralasar |ror|| : preror|a, Wreez|rg oeru|arg
- Fa||ure lo lrr|ve
- Proo|er ru|osa |u||l dar ru|ul
- 0VK
- Cl0
- lepalosp|erorega||
- Nodus ||rlal||us reroesar / rergec||
- Proo|er reurorus|u|ar
- Telar| / d|srorl||
0|ACN08|8....
Perer||saar darar rerurju||ar oag|ar dar| s|sler |rur yarg
l|da| ada alau l|da| oe|erja seresl|rya eva|uas| lurgs| se| 8
- |uarl|lal|l : lg0, lgA, lgV, lg, |uarl|lal|l lg0 suo|e|as
- Ao spes|l|| :
- |sorerag|ul|ras| lgV lrd go|orgar darar A80
- Ao lelarus loxo|d
- Ag prero|o|a| po||sa|ar|da
- Ao lrd Ag va|s|r var|se|a, l8v, reas|es
- Ao lrd ager resp|ras| : R3v, r||op|asra, para |rl|uersa,
|rl|uersa A / 8
- |uarl|lal|l se| 8 dar lerol|p : C020, C021, lg perru|aar se| 8
0HH0N VAR|ALE |HHUN00EF||ENY (V|0}
3e| 8 dar T
Predor|rar : r|pogarag|oou||rer|a
lrle|s| oeru|arg
lg oer|urarg, respors lerradap arl|o|ol|| je|e|
8erruourgar dergar ||rpora, arlr|l|s
Jar|rgar ||rpo|d r|perlrop|
lepalosp|erorega|| lroroos|loper| se|urder
0argguar aulo|rur ser|rg reralo|og|, reuro|og|, CAl,
erdo|r|ropal|
Arl|ood| spes|l|| l|da| ada / |urarg
Arggola |e|uarga rerpurya| |rs|ders l|rgg| lerradap del|s|ers|
lgA, perya||l aulo|rur, auloarl|ood|, |egarasar
|80LATE0 |gA 0EF|8|EN8|
0el|s|ers| Ao yarg ser|rg
1 : 100 s/d 1 : 3000
lgA < Z rg/d| dergar lg0 dar lgV rorra|
8erruourgar dergar alax|a-le|erg|a|lase
Aulosora|
8erruourgar dergar alop|
Pre|Wers| l|rgg| lerradap perya||l aulo|rur
TRAN8|ENT hYP0CAHACL0UL|NEH|A 0F |NFANY
3|rles|s lg ler|aroal
F|s|o|og|s lerjad| pada us|a 2 - 1 ou|ar
Kadarg oer|arjul sarpa| us|a 2 - 3 larur
lrle|s| sa|urar ralas oeru|arg
lg0 dar lgA rerdar, lap| lgV rorra|/l|rgg|
lg spes|l|| surlace ||rlos|l rorra|
3e|l ||r|led
Peroa||ar pada us|a 18 - 3 ou|ar
8erum |g |eve|s and age
b|rth
Age (years}
0
20 30
Cestat|on (weeks}
1 10
50
100
S
e
r
u
m

g

l
e
v
e
l
s

(
%

a
d
u
l
t

v
a
l
u
e
s
)
Maternal
Transfer gG
gM
gG
gA
Present|ng symptoms P|
0
30
100
P
e
r
c
e
n
t
a
g
e

o
f

p
a
t
i
e
n
t
s
10
20
80
90
90%
21%
15%
13%
6% 6% 6%
r
e
s
p
i
r
a
t
o
r
y
s
k
i
n
TERAP|
Arl|o|ol||
Perggarl| arl|ood| lvl0
A|lerral|l : oore rarroW lrarsp|arlal|or, erzyre
rep|acererl, or gere lrerapy
0ergar |rlervers|, pas|er lP d|rarap|ar o|sa lurour
|eroarg secara rorra| :
- o|sa oerparl|s|pas| da|ar |eg|alar |erja, oe|ajar,
|e|uarga a|l|v|las sos|a|
- |ejad|ar dar oeralrya |rle|s| oer|urarg
- sed|||l ele| sarp|rg dar| pergooalar
- rerasa ryarar dergar d|r|rya serd|r| dar
prograr pergooalar