Endokrinologi Anak

PENGANTAR
PEDIATRIC ENDOCRINOLOGY

RUANG LINGKUP ENDOKRINOLOGI ANAK
Satriono, M.Sc., Dr., SpA(K)
Terakhir diperbaharui pada / Page updated :10/10/00!
TUJUAN INTRUKSIONAL UMUM:
Maha"i"#a $e$aha$i pen%akit&pen%akit endokrino'ogi anak %ang "ering dite$ukan.
TUJUAN INSTRUKSIONAL KHUSUS
1.Men%ebutkan ruang 'ingkup endokrino'ogi anak.
.Ma$pu $enggunakan (ro#th chart untuk $eni'ai pertu$buhan anak.
).Me$buat diagno"i" hipotiroidi"$e pada anak dan ba%i. *erda"arkan ri#a%at pen%akit,
ge+a'a k'inik, 'aboratoriu$, pe$erik"aan penun+ang 'ainn%a.
,.Me$buat diagno"i" di-eren"ia' hipotiroidi"$e dengan Do#n." "%ndro$e.
*erda"arkan ri#a%at pen%akit, ge+a'a k'inik, 'aboratoriu$, pe$erik"aan penun+ang
'ainn%a.
/.Men%ebutkan da"ar&da"ar penata'ak"anaan a"uhan $edik gangguan pertu$buhan pada
anak.
!. Men%ebutkan da"ar&da"ar penata'ak"anaan a"uhan $edik hipotiroidi"$e pada anak.
0.Men%ebutkan da"ar&da"ar penata'ak"anaan a"uhan $edik "tru$a pada anak.
1.Men%ebutkan da"ar&da"ar penata'ak"anaan a"uhan $edik diabete" $e''itu" pada anak.
2. Men%ebutkan $aca$&$aca$ ke'ainan genita'ia pada anak.

Ruang Lingku P!n"aki#
1.Pato'ogi Pertu$buhan
.3ipo-i"e
3ipopituitari"$e
De-i"ien"i (ro#th 3or$one
TS3
A4T3
5S3 , 63
AD3
.3ipo-i"e
3iperpituitari"$e
(angguan 3ipota'a$u" 3ipo-i"e

).Tiroid
3ipotiroidi"$e
3ipertiroidi"$e
(78D7K pada Anak
Tiroiditi"
Kar"ino$a tiroid *edah
,.Suprarena'i"
3ipo-ung"i
Pen%akit Addi"on
9n"u-i"ien"i Suprarena'i" Akut
3iper-ung"i:
3iperp'a"ia "uprarena'i" kongenita'
Sindro$a 4u"hing
/.(onad
Puberta" ter'a$bat : 3ipogonadi"$e
Puberta" Prekok"
Statu" 9nter"e;ua' (P"eudo 3er$a-roditi"$e)
3er$a-roditi"$e
Di"gene"i" (onad
Ke'ainan Kro$o"o$ "e; Sindro$a Turner
Kriptorkidi"$e (<nde"cended Te"ti")
Mikropeni"
!.Pankrea"
Diabete" Me''itu"
3ipog'i"e$ia "pontan
0.Pato'ogi Paratiroid
Metabo'i"$e 4a'ciu$ =it D
1. 7be"ita"
>ang akan dibaha" untuk S1 Ked
1.3ipotiroidi"$e
.DD n%a Do#n." S%ndro$e
).Diabete" Me''itu" pada Anak
,.Stru$a pada Anak.
/.(angguan pertu$buhan , Penggunaan (ro#th 4hart.
!.Ke'ainan (enita'ia pada anak.
0.3iperp'a"ia adrena' kongenita'.
1.7be"ita" anak
Endokrinologi Anak
HIPOTIROIDISME
Satriono, M.Sc., Dr., SpA(K)
Terakhir diperbaharui pada / Page updated :10/10/00!

D?5989S9 :
De-i"ien"i hor$on tiroid.
T, @ tirok"in
T) @ triiodo tironin
K6AS959KAS9
9. 39P7T9A79D9SM? K78(?89TA6
39P7T9A7D9SM? D9DAPAT (A4B<9A?D)
9"ti'ah KA?T989SM?C &&& dipakai untuk hipotiroidi"$e kongenita' di daerah ende$ik
(AK9
99. & 3 ?8D?M9K (AK9
& 3 SP7AAD9K
999. & 3 *A>9 &&&& 3 K78(?89TA6
& 3 A8AK &&&& 3 D<=?896
Pen%ebab 3 Kongenita'
1.Di"gene"i" /Atireo"i" : ap'a"i, hipop'a"i, Ma'de"cent E Ke'en+ar ektopik
.D%"hor$ogene"i"
).7bat&obatan (KD, (oitrogen)
,.De-i"ien"i 9odiu$
/.Th%roid 3or$on? <nre"pon"iFene""
!.De-i"i"en"i TS3

Pen%ebab 3 didapat (AcGuired)
1.7pera"i
.Auto i$$une Di"ea"e& Tiroidit" 6i$-o"itik Kronik (3AS39M7T7)
).9n-ek"i
,.7bat&obatan
/.De-i"ien"i 9odiu$
1.3 Pri$er & Tiroid
.3 Sekunder & 3ipo-i"i" ).3 Ter"ier &3ipota'a$u"
,.3 &&H?nd 7rganH

HIPOTIROIDISME $AYI
9n"iden" 1:,000 ()0/0 E10000)
Pere$puan : 6aki&'aki @ ): 1
(e+a'a baru $uncu' 1&) bu'an "e"udah 'ahir
Si$to$
1.7b"tipa"i
.Ma'a" $enetek (Minu$ "u"u)
).6etargi & $au tidur "a+a
,.9kteru" neonatoru$ Pro'ongatu"
/.3ipoter$i
Sign" ((e+a'a K'inik)
1.<<* 6ebar I)
.Aa$but kering
).3iperte'ori"$e
,.Makrog'o"i
/.Muka Se$bab (a Pu--% 5ace)
I) 5ontane'a po"t terbuka #aktu 'ahir 3ipotiroidi"$e *a%i
!.6eher Pendek
0.Ku'it: teba', kering, Kuning, Pucat, $ik"ede$a
1.Suara "erak
2.3ipotoni
10. Di"tenden Abdo$en
11.3ernia u$bi'ika"'i"
1.(ondok bi"a ada / tidak
1). Dantung :
Kardio$ega'i
*i"ing J
(AM*AA 39P7T9A79D9SM? K78(?89TA6
1.<<* 6ebar
.Aa$but kering
).3iperte'ori"$e
,.Makrog'o"i
/.Muka Se$bab
(a Pu--% 5ace)

HIPOTIROIDISME ANAK
Si$to$
1.Pertu$buhan ter'a$bat
& Menta' & 5i"ik & Denti"i
.(e$uk : Muka "e$bab
).Stru$a
,.*odoh
/.4o'd 9nto'erance
!.Meno$etrorhagi
(pada anak pere$puan
puberta")
Sign" ((e+a'a K'inik)
1.*radikardi
.Pendek
Aatio <pper:6o#er Seg$ent
K8or$a'
).7Fer#eight
,.Mik"ede$a
/.(oiter bi"a ada / tidak
!.Ku'it: Pucat, Teba', Karotine$ik,
Dingin, Mik"ede$a
0.3ipotoni E 7tot 6e$bek
1.APA , KPA E 6a$bat
(Laktu re-'ek" $e$an+ang)
Pe$erik"aan
DAAA3:
& 3b $enurun
& A'ka'i -o"-ata"e $enurun
& 3iperko'e"tero'e$ia
M Aa%
& di"gene"i" epi-i"i" (Li'kin)
& *one Age ter'a$bat
Pe$erik"aan
*one Age ter'a$bat
Pe$erik"aan 3or$ona'
5ree T, $enurun
TS3 "en"iti- $eningkat
Da$an Du'u : T, tota' $enurun , TS3 $eningkat
Da$an du'u "eka'i :
P*9 (protein *ound 9odine)
P!%!rik&aan Lain
Scanning (Sidik Tiroid)
??( 6o# =o'tage
?K( 6o# =o'tage
?M( (?'ectro$%ogra$) $e$an+ang
*MA $enurun
Tak di'akukan pada Anak
P!%!rik&aan Lain
<+i TA3
<ntuk bedakan 3 Pri$er dan 3 Sekunder

Diagno&#ik H
Sign" dan S%$pto$" 3
6aboratoriu$: du'u 3iperko'e"tero'e$ia 3b rendah
*one Age ter'a$bat
3or$ona' :
TS3 "en"iti- $eningkat
5ree T, $enurun
D<6< Diagno"i" e; +uFantibu"

PENGO$ATAN H
Du'u : obat de""icated th%roid e;tract
4ontoh Th%ranon 1 tab'et 100 $g
Do"i" opti$a' 100 $g / $
Sekarang : Sodiu$ 6eFo Th%ro;in
0,1 $g 6 Th%ro;in @ !0 E 100 ?;tract Th%roid
4ontoh: Th%ra; (7rganon)
?uth%ro; (Merck)
Do"i" /&1 ug/kg **
Diagno&i& $anding
Do#n." S%ndro$e
Anak Pendek:
1.Kondrodi"tro-i (Akondrop'a"ia)
.D#ar-i"$ E 3ipopituitari"$e E De-i"ien"i (ro#th 3or$one
).Turner S%ndro$e pada anak pere$puan
Progno&i&
*i'a terapi ter'a$bat / tanpa terapi
1.Ke$atian o.k.:
& 7b"truk"i "a'uran na-a"
& 9n-ek"i
.(ro#th Aetardation
).Matura"i otot ter'a$bat
,.Menta' Aetardation
/.SeGue'e 8ero'ogik :
& inkoordina"i
& atak"i
& "pa"tik
& "trabi"$u" , d''

Progno&i&
Bu ad =ita$
*ona$
Dubia
Ma'a$ & 9n-au"t
Bu ad "anatione$
*ona$
Dubia
De'ek
Progno"i"
N 4epatn%a Diagno"i"
O ) bu'an rata&rata 9B 12
) E ! bu'an rata&rata 9B 00
K 0 bu'an rata&rata 9B /,
N ?tio'ogi 9BK1/
Atierotik ,1P
Di"hor$ogene"i" ,,P
?ktopik 01P

MAK98 D989 T?AAP9
Poten"i inte'ek $akin tinggi
Ke'aianan nero'ogik $akin kurang
P?A6< <D9 SAA98( (S4A??898( T?ST) PADA 8?78AT<S
dipakai TS3

Endokrinologi Anak
DD Hio#iroidi&%!:
Terakhir diperbaharui pada / Page updated :10/10/00!
K<69A3 00,
DO'N(S SYNDROME )MONGOLISME)
Satriono,M.Sc.,Dr.,SpA(K)
*9KA 5K <83AS, Maka""ar
D7L8.S S>8DA7M? (DS)
11!! D.6.3. Do#n : Do#n." S%ndro$e
9n"iden" : 1& /1000 ke'ahiran
?T9767(9 :
12/2 6e Deune dkk TA9S7M9 1
TA9S7M9 1
Tri"o$i Aegu'er 2/ P
Tran"'oka"i ,&/ P
Mo"aik Q & 1 P
Patogene"i" diFi"ion
"truktur
4ontoh Kar%ot%pe pa"en

(e+a'a K'inik DS
Aetarda"i Menta'
9B 0&0 9D97T
9B 1 E /0 9M*?496
Aetarda"i Motorik :
(<$ur ) tahun perta$a)
Muka : 5A49?S M78(7679D
(e+a'a K'inik DS
Kepa'a
*raki"e-a'
<<* 6ebar, Ter'a$bat $enutup
*e'akang Kepa'a datar
(e+a'a K'inik DS
MATA
A'i" tipi"
4e'ah $ata $iring
?picanthu"
Katarak
8i"tag$u"
Strabi"$u"
3iperte'ori"$e
*ru"-ie'd." Spot
(e+a'a K'inik DS
39D<8(
Pe"ek
69DA3
Makrog'o"i
6ingua Skrota'i"
AA3A8( 3ipopa'"ia
PA6AT<M 6etak tinggi
(9(9 Abnor$a'
T?698(A Abnor$a'
6?3?A pendek
T37AAKS:
Ke'ainan *entuk
DA8T<8( KD* & =SD
A*D7M?8
3ernia <$bi'ika'i"
Ke'ainan (9 : Megako'on
7T7T 3ipotoni
P?6=9S Keci'
Der$atog'i-ik



Der$atog'i-ik DS
TA8(A8: Si$ian 'ine, S%dne% 'ine
(ari" -'ek"i di"ta' +ari = $enghi'ang
<D<8( DAA9: 6engkung <'na
T?6APAK TA8(A8:
Sudut atd K ,/R (po"i"i t., t.., t...)
T?6APAK KAK9:
3a'u; : *u"ur tibia
6engkung di"ta' keci'



M Aa% Panggu' DS
Sudut i'iu$ (i) O !0R
Sudut a"etabu'u$ (a) O
1! R
9'iac inde; @ ika J iki
J aka J aki


8or$a' K 10
M Aa% Panggu' DS
Diagno"tik DS
A.(?DA6A K6989K
*.(?DA6A >( DPAT D9K<A8T959KAS9:
Anthropo$etrik : **, T*, 6K
<pper:'o#er bod% "eg$entK8
<K<AA8 PA8((<6
D?AMAT7(6959K
4.P?M?A9KSAA8 KA7M7S7M

Diagno"tik DS
D?AMAT7(6959K
S47A? 4AAD :
La'ker
*eck$an (<pp"a''a)

Aeed (<niF.9ndiana):
1.Po'a ha'u; kanan
.Sudut atd kanan
).Po'a te'un+uk kanan
,.Po'a te'un+uk kiri
P!n*!ga+an DS
(?8?T94 47<8S?6698(
Tipe tran"'oka"i ok E $uta"i
& -a$i'ia'
Per'u pe$erik"aan kro$o"o$ anggota ke'uarga
9*< E *a'anced tran"'ocation
Per'u diagno"tik antenata'
Progno"i" DS
6?*93 *A9K daripada +a$an du'u
K?MAT9A8 ok :
Peneu$onia
43D
Ke'ainan kongenita'
6eke$ia
9n-ek"i
T!rai DS
Tak ada o'eh karena kongenita'
Terapi "i$pto$atik
in-ek"i
Korek"i ke'ainan
ST9M<6AS9 D989
Endokrinologi Anak
DIA$ETES MELLITUS PADA ANAK
Satriono, M.Sc., Dr., SpA(K)
Terakhir diperbaharui pada / Page updated :10/10/00!
Dia,!#!& M!lli#u& in C+ildr!n
Diabete" $e''itu" i" a "%ndro$e o- di"turbed energ% ho$eo"ta"i" cau"ed b% a de-icienc%
o- in"u'in or o- it" action and re"u'ting in abnor$a' $etabo'i"$ o- carboh%drate, protein,
and -at. 9t i" the $o"t co$$on endocrine&$etabo'ic di"order o- chi'dhood and
ado'e"cence #ith i$portant con"eGuence" -or ph%"ica' and e$otiona' deFe'op$ent.


TABLE XXVI-1 Summary of Classification of Diabetes Mellitus in
Chilren anAolescents!
Classification Criteria
1" Insulin#e$enent
%IDDM& ty$e I'
Ty$ical manifestations( )lucosuria&
*etonuria& ranom
$lasma )lucose %+,' -.// m)0L
OSTendi-UK
." 1on#insulin-e$enent
%1IDDM& ty$e II'
2+, -13/ m)0L an .-hr 4alue
-.// m)0L 5,TT
on more than one occasion an in
absence of
$reci$itatin) factors
6" 5ther ty$es Ty$e I or II criteria in association 7ith
certain )enetic
synromes %incluin) cystic fibrosis'&
other
isorers& an ru)s %see te8t'
Im$aire )lucose tolerance
%I,T'
2+, 913/ m)0L 7ith .-hr 4alue
-13/ m)0L urin)
5,TT
,estational iabetes %,DM' T7o or more of follo7in)
abnormalities urin) 5,TT(
2+, -1/: m)0L; 1 hr& -1</ m)0L;
.-hr& -1=:
m)0L; 6 hr& -13: m)0L"
Statistical ris* classes
1" +re4ious abnormality of
)lucosetolerance
1ormal 5,TT follo7in) a $re4ious
abnormal one&
s$ontaneous hy$er)lycemia or
)estational iabetes
." +otential abnormality of
)lucose
tolerance
,enetic $ro$ensity %e")"& ientical
noniabetic t7in of a iabetic
siblin)'; islet cell antiboies
!+ro$ose by 1ational Diabetes Data ,rou$ %Diabetes .>(1/6<&
1<?<' an enorse
by 4arious iabetes associations 7orl7ie"
+, @$lasma )lucose; 2+, @ fastin) $lasma )lucose; @ oral )lucose
tolerance test"

?P9D?M9767(>

5igure MM=9E1. 9ncidence o- in"u'in&dependent diabete" $e''itu" b% countr%. (Adapted
-ro$ 6aPorte A, et a': PreFenting in"u'in dependent diabete" $e''itu": The enFiron$enta'
cha''enge. *r Med D 2/:,02, 1210.)

Ma'e" and -e$a'e" are a'$o"t eGua''% a--ectedV
there i" no apparent corre'ation #ith "ocioecono$ic "tatu".
Peak" o- pre"entation occur in t#o age group": at /E0 %r o- age and at the ti$e o-
pubert%

?T9767(> A8D PAT37(?8?S9S

5igure MM=9E. Propo"ed "che$e o- natura' hi"tor% o- *&ce'' de-ect. (Adapted -ro$
Sper'ing MA TedU: Ph%"icianW" (uide to 9n"u'in&Dependent TT%pe 9U Diabete" Me''itu":
Diagno"i" and Treat$ent. 4op%right (1211) b% the A$erican Diabete" A""ociation.
Aeprinted #ith per$i""ion.)

PAT37P3>S9767(>

TA*6? MM=9& 9n-'uence o- 5eeding (3igh 9n"u'in) or o- 5a"ting(6o# 9n"u'in) on So$e
Metabo'ic
Proce""e" in 6iFer, Mu"c'e, and Adipo"e Ti""ueI
3ighP'a"$a 9n"u'in
(Po"tprandia'
State)
6o# P'a"$a 9n"u'in
(5a"ted State)
6iFer: ('uco"e <ptake ('uco"e production
('%cogenS%nthe"i" ('%cogeno'%"i"
Ab"ence o- ('uconeogene"i" ('uconeogene"i"
6ipogene"i" Ab"ence o- 'ipogene"i"
Ab"enceo-ketogene"i" Ketogene"i"
Mu"c'e: ('uco"e <ptake Ab"ence o- g'uco"euptake
('uco"e7;idation 5att% acid and ketone o;idation
('%cogenS%nthe"i" ('%cogeno'%"i"
ProteinS%nthe"i" Proteo'%"i" and a$ino Acid re'ea"e
Adipo"e ti""ue: ('uco"e <ptake Ab"ence o- g'uco"e uptake
6ipid S%nthe"i" 6ipo'%"i" and -att% acid re'ea"e
Trig'%ceride <ptake Ab"ence o- trig'%ceride uptake
I9n"u'in i" con"idered to be the $a+or -actor goFerning the"e $etabo'ic proce""e". Diabete"
$e''itu"$a% be Fie#ed a" a per$anent 'o#Ein"u'in "tate that, untreated, re"u't" in e;aggerated
-a"ting.


469894A6 MA895?STAT978S.
The c'a""ic pre"entation o- diabete" in chi'dren i"
a hi"tor% o- po'%uria, po'%dip"ia, po'%phagia, and #eight 'o"".
The duration o- the"e "%$pto$" Farie" but i" o-ten 'e"" than 1 $o.
A c'ue to the e;i"tence o- po'%uria $a% be the on"et o- enure"i" in a preFiou"'% toi'et&
trained chi'd.
An in"idiou" on"et characteriXed b% 'etharg%, #eakne"", and #eight 'o"" i" a'"o Guite
co$$on.
The 'o"" o- #eight in "pite o- an increa"ed dietar% intake i" readi'% e;p'icab'e b% the
-o''o#ing i''u"tration:
The aFerage hea'th% 10&%r&o'd chi'd ha" a dai'% ca'oric intake o- ,000 or $ore ca'orie",
o- #hich appro;i$ate'% /0P are deriFed -ro$ carboh%drate.
Lith the deFe'op$ent o- diabete", dai'% 'o""e" o- #ater and g'uco"e $a% be a" $uch a" /
6 and /0 g, re"pectiFe'%.
Thi" repre"ent" 1,000 ca'orie" 'o"t in the urine, or /0P o- aFerage dai'% ca'oric intake.
There-ore, de"pite the chi'dW" co$pen"ator% increa"ed intake o- -ood and #ater, the
ca'orie" cannot be uti'iXed, e;ce""iFe ca'oric 'o""e" continue, and increa"ing catabo'i"$
and #eight 'o"" en"ue.
P%ogenic "kin in-ection" and $oni'ia' Faginiti" in teenage gir'" are occa"iona''% pre"ent at
the ti$e o- diagno"i" o- diabete".
The% are rare'% the "o'e c'inica' $ani-e"tation" o- diabete" in chi'dren, and a care-u'
hi"tor% #i'' inFariab'% reFea' the coe;i"tence o- po'%uria and po'%dip"ia.
Ketaocido"i" i" re"pon"ib'e -or the initia' pre"entation o- $an% (appro;i$ate'% /P)
diabetic chi'dren.
The ear'% $ani-e"tation" $a% be re'atiFe'% $i'd and con"i"t o- Fo$iting, po'%uria, and
deh%dration.
9n $ore pro'onged and "eFere ca"e", Ku""$au' re"piration" are pre"ent, and there i" an
odor o- acetone on the breath.
Abdo$ina' pain or rigidit% $a% be pre"ent and $a% $i$ic appendiciti" or pancreatiti".
4erebra' obtundation and u'ti$ate'% co$a en"ue.

6A*7AAT7A> 598D98(S :
g'uco"uria,
ketonuria,
h%perg'%ce$ia,
ketone$ia,
and $etabo'ic acido"i".
6eukoc%to"i" i" co$$on, and non"peci-ic "eru$ a$%'a"e $a% be e'eFatedV "eru$ 'ipa"e
i" u"ua''% not e'eFated.

D9A(87S9S.
three genera' categorie":
(1) tho"e #ho haFe a hi"tor% "ugge"tiFe o- diabete", e"pecia''% po'%uria #ith po'%dip"ia
and -ai'ure to gain #eight or a 'o"" o- #eight in "pite o- a Foraciou" appetiteV
() tho"e #ho haFe a tran"ient or per"i"tent g'uco"uriaV and
()) tho"e #ho haFe c'inica' $ani-e"tation" o- $etabo'ic acido"i" #ith or #ithout "tupor or
co$a.
9n a'' in"tance" the diagno"i" o- diabete" $e''itu" i" dependent on the de$on"tration o-
N h%perg'%ce$ia
N in a""ociation #ith g'uco"uria
N #ith or #ithout ketonuria.
N decrea"ed 4&peptide
N increa"ed 3bA1c
Lhen c'a""ic "%$pto$" o- po'%uria and po'%dip"ia are a""ociated #ith h%perg'%ce$ia
and g'uco"uria,
the g'uco"e to'erance te"t i" not needed to "upport the diagno"i"


TREATMENT-
Y 98S<698
Y 8<TA9T978 S<PP7AT
Y ?M?A49S?
Y ?D<4AT978 A8D 47<8S?698(

98S<698 TA?ATM?8T.
The $anage$ent o- in"u'in&dependent diabete" $e''itu" $a% be diFided into three pha"e"
depending on
the initia' pre"entation: that o- ketoacido"i"V
the po"tacidotic or tran"ition period -or e"tab'i"h$ent o- $etabo'ic contro'V and
the continuing pha"e o- guidance o- the diabetic chi'd and hi" or her -a$i'%.
The techniGue o- in+ection o- in"u'in "hou'd be taught to the parent" and to the patient
#hen he or "he i" read% -or it. 9n+ection" are giFen "ubcutaneou"'%, rotating "ite" on ar$",
thigh", buttock", and abdo$en in a regu'ar "eGuence. An appropriate rotation he'p" to
en"ure adeGuate ab"orption o- in"u'in, preFent -ibro"i", and $ini$iXe 'ipod%"trophic
change". Lith thi" rotation and the aFai'abi'it% o- the purer, "ing'e&peak in"u'in",
'ipoatroph% and 'ipoh%pertroph% are Guite unu"ua'. >ounger chi'dren $a% -ind in+ection"
in the abdo$ina' #a'' di--icu't or pain-u'. Depending on their ph%"ica' and p"%cho'ogic
$aturit%, chi'dren oFer the range o- 10EZenda"h[1 %r "hou'd be encouraged to
ad$ini"ter their o#n in"u'in and to $onitor their o#n re"pon"e" to it.



47MP694AT978
The $a+or 'i-e&threatening co$p'ication in chi'dren treated -or DKA i" cerebra' ede$a.
4'inica''%, cerebra' ede$a deFe'op" "eFera' hour" a-ter the in"titution o- therap%, #hen
c'inica' and bioche$ica' indice" $a% "ugge"t i$proFe$ent. The $ani-e"tation" are tho"e
o- rai"ed intracrania' pre""ure and inc'ude headache, a'teration and deterioration in
a'ertne"" and con"ciou" "tate, \de'iriou" outbur"t",\ brad%cardia, Fo$iting, di$ini"hed
re"pon"iFene"" to pain-u' "ti$u'i, and di$ini"hed re-'e;e". There $a% be a change in
pupi''ar% re"pon"iFene"" #ith uneGua' pupi'" or -i;ed di'ated pupi'". Po'%uria, "econdar%
to deFe'op$ent o- diabete" in"ipidu", $a% be erroneou"'% attributed to o"$otic diure"i"
"econdar% to h%perg'%ce$ia, a'though diabete" $e''itu" and diabete" in"ipidu" coe;i"t.
Pro$pt recognition o- the condition a" it eFo'Fe", and pro$pt therap% #ith $annito' and
h%perFenti'ation, can be 'i-e"aFing. 9ncrea"ing'%, eFidence point" to the conc'u"ion that
"ubc'inica' cerebra' ede$a occur" in the $a+orit% o- patient" treated #ith -'uid" and
in"u'in -or DKA, and that in on'% a $inorit% doe" it beco$e c'inica''% $ani-e"t a" a
$edica' e$ergenc%.
8?<A7=AS4<6AA A8D 7T3?A 47MP694AT978S: A?6AT978 T7 (6>4?M94
478TA76.
The increa"ing'% pro'onged "urFiFa' o- the diabetic chi'd i" a""ociated #ith an increa"ing
preFa'ence o- co$p'ication" that a--ect the $icrocircu'ation o- :
N the e%e (retinopath%),
N the kidne% (nephropath%),
N the nerFe" (neuropath%),
N the 'arge Fe""e'" (athero"c'ero"i"),
N and the 'en" (cataract").
Aetinopath% i" pre"ent in ,/EZenda"h[!0P o- in"u'in&dependent diabetic" a-ter 0 %r o-
kno#n di"ea"e and in 0P a-ter 10 %rV 'en" opacitie" are pre"ent in at 'ea"t /P o- tho"e
under 12 %r o- age.
Diabetic nephropath% i" a'"o co$$onV it i" pre"ent in about ,0P o- patient" a-ter / %r o-
in"u'in&dependent diabete" #ho"e on"et occurred in chi'dhoodV thi" co$p'ication $a%
account -or about /0P o- death" in 'ong&ter$ in"u'in&dependent diabetic".
7ther co$p'ication" de"cribed in diabetic chi'dren inc'ude d#ar-i"$ a""ociated #ith a
g'%cogen&'aden en'arged 'iFer (Mauriac "%ndro$e), o"teopenia, and a "%ndro$e o-
'i$ited +oint $obi'it% a""ociated #ith tight, #a;% "kin, gro#th i$pair$ent, and
$aturationa' de'a%. The Mauriac "%ndro$e i" c'ear'% re'ated to underin"u'iniXationV it i"
no# rare becau"e o- the aFai'abi'it% o- the 'onger&acting in"u'in".
Another rare "%ndro$e a""ociated #ith diabete" $e''itu" i" the Lo'-ra$ "%ndro$e, a'"o
kno#n a" the D9DM7AD "%ndro$e becau"e o- it" $a+or cardina' $ani-e"tation" o-
diabete" in"ipidu", diabete" $e''itu", optic atroph%, and dea-ne"". The di"ea"e i" -a$i'ia'
#ith an auto"o$a' rece""iFe pattern o- inheritance.
PA7(87S9S.
T%pe 9 diabete" $e''itu" i" not a benign di"ea"e. 9n one "tud% o- the 'ong&ter$ outco$e o-
,/ chi'dren under 1 %r o- age at the ti$e o- diagno"i", three #ere "eFera' death" #ithin
10E/ %r o- diagno"i": there #ere direct'% attributab'e to diabete", and t#o #ere due to
"uicideV three patient" atte$pted "uicide un"ucce""-u''%.
=i"ua', rena', neuropathic, and other co$p'ication" #ere re'atiFe'% -reGuent.
5urther$ore, a'though diabetic chi'dren eFentua''% attain a height #ithin the nor$a'
adu't range, pubert% $a% be de'a%ed, and the -ina' height $a% be 'e"" than the genetic
potentia'
Endokrinologi Anak
STRUMA PADA ANAK-
Satriono, M.Sc., Dr., SpA(K)
Terakhir diperbaharui pada / Page updated :10/10/00!
Stru$a (goiter,gondok) $erupakan "etiap pe$be"aran ke'en+ar tiroid. Anak dengan
pe$be"aran ke'en+ar tiroid bi"a $e$per'ihatkan -ung"i tiroid %ang nor$a' (eutiroidi"$e),
-ung"i tiroid %ang kurang (hipotiroidi"$e), atau ke'ebihan produk"i hor$on tiroid
(hipertiroidi"$e).
(AADAS9
Secara pe$erik"aan -i"ik ke'en+ar tiroid di"ebut $e$be"ar bi'a ukurann%a 'ebih be"ar
daripada rua" terakhir ibu +ari penderita.
(rada"i pe$be"aran ke'en+ar tiroid pada "urFe% (AK9 di 9ndone"ia Dera+at 7, 1 A, 1* ,
dan ).
(AADAS9 L37 0 , 1 ,
Stru$a bi"a digo'ongkan ke da'a$ :
1.Stru$a kongenita' dan didapat.
.Stru$a ende$ik dan "poradik.
).Stru$a intratrakea' dan Stru$a ek"tratrakea'
PAT759S9767(9 T?ADAD98>A STA<MA:
1.Seringka'i Stru$a ti$bu' akibat $eningkatn%a TS3 "ebagai reak"i terhadap
$enurunn%a hor$on tiroid %ang ber"irku'a"i.
.Stru$a bi"a $uncu' akibat pro"e" in-i'tra"i berupa peradangan ataupun neop'a"$a.
).Pada anak %ang $enderita tirotok"iko"i" Stru$a di"ebabkan o'eh th%rotropin receptor
"ti$u'ating antibodie" (TASAb).


(a$bar 1.
Seorang anak pere$puan dengan
"tru$a ende$ik di Su'a#e"i
Se'atan %ang $e$per'ihatkan
ge+a'a hipotiroidi"$e
P?8(7*ATA8 STA<MA D9 DA?AA3 ?8D?M9K
Pe$berian iodiu$ da'a$ $in%ak "ecara intra $u"ku'er pada ibu akan $encegah
de-i"ien"i 9odiu$ keha$i'ann%a %ang akan dating "e'a$a / tahun.
Terapi "e$aca$ ini pada anak beru$ur kurang , tahun %ang $enderita kretini"$e
dengan $%;ede$a akan $e$buat euth%roidi"$e da'a$ #aktu / bu'an.
Tetapi re"ponn%a "angat "edikit pada anak %ang 'ebih tua dan tidak "a$a "eka'i pada
orang de#a"a, ha' ini $enun+ukkan ketidak $a$puan ke'en+ar tiroid $eng"inte"i"V
Penderita ini $e$er'ukan pengobatan dengan T, . (8e'"on 1!)

P?8(7*ATA8 STA<MA SP7AAD9K
T, treat$ent i" indicated in patient" #ith high "eru$ TS3 concentration", in #ho$ it
$a% re"u't in a decrea"e in goiter "iXe.
Patient" #ho are euth%roid can a'"o be treated #ith T, in an atte$pt to reduce the goiter,
but it i" not o-ten e--ectiFe. (6a5ranchi, 001)

Endokrinologi Anak
GANGGUAN PERTUM$UHAN . PENGGUNAAN GRO'TH CHART-
Satriono, M.Sc., Dr., SpA(K)
Terakhir diperbaharui pada / Page updated :10/10/00!
SPM PERA'AKAN PENDEK
Pendahu'uan
Pera#akan pendek atau "hort "tature $erupakan "uatu ter$ino'ogi $engenai
pan+ang/tinggi badan %ang berada di ba#ah per"enti' ) atau ESD pada kurFa
pertu$buhan %ang ber'aku pada popu'a"i ter"ebut.
Pera#akan pendek dapat $erupakan Faria"i nor$a' pera#akan pendek dan dapat
di"ebabkan berbagai ke'ainan endokrin $aupun non endokrin. Ada beberapa k'a"i-ika"i
pera#akan pendek %aitu :
a. =arian nor$a' (u$u$n%a -a$i'ia' atau pen%ebab tidak diketahui)
b. pri$er / in"trin"ic ( ke'ainan pada "e' atau "truktur dari gro#th p'ate )
c. "ekunder / e;terna' (ke'ainan karena pengaruh 'uar dari gro#th p'ate)
d. Pera#akan pendek 9diopatik
a. =arian 8or$a'
Merupakan Faria"i nor$a' pera#akan pendek :
1. 5a$i'ia'/genetic "hort "tature
Tanda :
N pertu$buhan "e'a'u diba#ah p.)
N Kecepatan pertu$buhan nor$a' ("ekitar ./th centi'e )
N <$ur tu'ang "e"uai u$ur krono'ogi"
N Ai#a%at ke'uarga pendek teruta$a "a'ah "atu atau kedua orang tua pendek ( genetica''%
"hort)
N Tinggi akhir diba#ah p.) tetapi $a"ih da'a$ range poten"i tinggi genetik
N 7n"et puberta" nor$a'
. 4on"titutiona' de'a% o- gro#th and pubert%/$aturation
Tanda :
N Pera#akan pendek "aat $a"a anak
N Per'a$batan pertu$buhan 'inier pada ) tahun perta$a kehidupan
N Pertu$buhan 'inier nor$a' atau ha$pir nor$a' pada "aat prepuberta" dan "e'a'u berada
diba#ah p.)
N <$ur tu'ang ter'a$bat tetapi $a"ih "e"uai dengan height age
N 7n"et puberta" ter'a$bat
N Tinggi akhir da'a$ bata" nor$a'
N *ia"an%a ada ri#a%at puberta" ter'a$bat da'a$ ke'uarga
b. Pera#akan pendek pri$er / in"trin"ik
1.Sindro$&"indro$ %ang dihubungkan
dengan ke'ainan kro$o"o$
N Sindro$ Turner
N Sindro$ Do#n
.Sindro$ E"indro$ 'ain, $i"a'n%a:
N Sindro$ 8oonan
N Sindro$ Prader&6abhart&Li''i
N Sindro$ Au""e'' Si'Fer
N Sindr$e Secke'
N Sindro$ 3utchin"on (i'-ort
N Sindro$ 4ocka%ne
).9<(A, %ang di"ebabkan
N genetik atau ke'ainan $etabo'ik
N Adan%a ke'ainan "aat da'a$ kandungan o'eh in-ek"i, obat&obatan, a'koho',d''
N Di"-ung"i p'a"enta berat
,.Ske'eta' d%"p'a"ia/o"teochondrod%"p'a"ia
N Achondrop'a"ia
N 3%pochondrop'a"ia
N 3%popho"phate$ic ricket"
/. Storage di"order" (+arang)
N Mucopo'%"accharido"e"
N ('%cogen "torage di"ea"e
N 7"teogene"i" i$per-ecta
c. Pera#akan pendek "ekunder / e;trin"ik
1. Pen%akit / ke'ainan "i"te$ik
(chronic di"ea"e)
Mi"a'n%a ke'ainan +antung, paru, 'iFer, inte"tina', rena', he$ato'ogi, 48S dan genera'iXed
in-'a$$ator% di"ea"e, in-ek"i kronik, ane$ia kronik,$a'ab"orb"i
. Ma'nutri"i
). ke'ainan endokrin
N 3ipotiroid
N (ro#th hor$on de-i"ien"i
N 9(5&1 de-ect
N P"eudoh%poparath%roidi"$
N The cu"hing "indro$
N Mauriac "%ndro$e (karena regu'a"i g'uko"a %ang +e'ek pada pa"ien diabete" $e''itu")
N 3%pogonadi"$
N Aicket"
). Metabo'ik di"order"
*eberapa inborn error" o- $etabo'i"$ $i"a'n%a "indro$ *artter
,. 9atrogenic "hort "tature
Terapi "teroid, radia"i
/. P"%cho"o"ia' "hort "tature atau e$otiona' ( P"%cho"ocia' d#ar-i"$)
d. Pera#akan pendek idiopatik
Tidak di+u$pai ke'ainan

]Pe$antauan kecepatan pertu$buhan "angat dibutuhkan untuk $eni'ai nor$a' tidakn%a
pertu$buhan anak
Detek"i dini pen%i$pangan pertu$buhan diper'ukan untuk pe$berian terapi 'ebih a#a'
"ehingga $e$beri ha"i' %ang opti$u$H
6angkah Pro$oti- / PreFenti-
Peni'aian pertu$buhan $erupakan ha' penting bagi dokter anak dan ke"ehatan anak
ko$unita". *eberapa kondi"i 'oka' atau "i"te$ik dapat berakibat buruk terhadap
pertu$buhan . Ana'i"i" pro"e" pertu$buhan $e$pun%ai peran penting "ebagai "uatu
'angkah a#a' dari eFa'ua"i.
Pengukuran tinggi badan $erupakan ha' %ang $udah di'akukan dan tidak $e$er'ukan
pera'atan canggih dan dapat di'ak"anakan "ecara rutin "e+ak $u'ai ba%i "eperti ha'n%a
berat badan.
N Anak 0&1 bu'an "etiap bu'an
N Anak 1& tahun "etiap ) bu'an
N Anak &1 tahun "etiap ! bu'an
N 1 tahun&akhir puberta" "etiap tahun
9nterpreta"i ha"i' pengukuran :
1. *i'a tinggi badan diantara ESD dan E)SD, 10P $erupakan Farian nor$a'. *i'a
diba#ah )SD 10P $erupakan pato'ogi".
. Penurunan kecepatan pertu$buhan anak antara u$ur & 1 tahun ($e$otong
beberapa gari" per"enti') haru" dianggap pato'ogi" kecua'i dibuktikan 'ain.
). Aatio T* dan ** $ungkin bi"a $e$pun%ai ni'ai diagno"tik da'a$ $enentukan
etio'ogi. (Pada ke'ainan endokrin u$u$n%a tidak $engganggu ** "ehingga anak ter'ihat
ge$uk.Ke'ainan "i"te$ik u$u$n%a 'ebih $engganggu ** dibanding T* "ehingga anak
'ebih ter'ihat kuru")

6angkah Diagno"tik
9.Ana$ne"i" ('ihat tabe')
&Po'a pertu$buhan anak (berat badan dan tinggi badan $u'ai ba%i)
&Ai#a%at keha$i'an ibu
&Ai#a%ata ke'ahiran dan perke$bangan -i"i"
&Ai#a%at pen%akit kroni" , opera"i dan obat E obatan
&Ai#a%at pen%akit da'a$ ke'uarga
&Ai#a%at puberta" orang tua
&Ai#a%at nutri"i/ diet
&A"pek p"iko"o"ia'
&Target height / $id parenta' height :
6aki E 'aki @ ZT* a%ah J (T* 9bu J 1) )[ ; Q
Pere$puan @ ZT* 9bu J (T* a%ah E 1) )[ ; Q
&Prakiraan tinggi de#a"a ( poten"i tinggi genetik) dapat dihitung dari $idparenta'
height dengan ru$u" :
Poten"i tinggi genetik @ $id parenta' height ^ 1,/ c$
( Poten"i tinggi genetik ada'ah: Aentang ni'ai tinggi badan akhir "e"eorang akibat dari
kedua orang tua bio'ogi" )
99.Pe$erik"aan -i"i"
&Tinggi *adan,*erat *adan, rentang 'engan, tinggi duduk ( propor"i tubuh ), 'ingkar
kepa'a
Tubuh %ang tidak propor"iona' dapat ter'ihat pada beberapa ke'ainan tu'ang, ke'ainan
di"$or-ik "eperti "indro$ ttt
&Ada tidakn%a "tig$ata di"$or-ik /"indro$
&Ada tidakn%a ke'ainan tu'ang
&Ada tidakn%a ke'ainan (9T, paru, +antung, urogenita' ,ku'it dan organ 'ain
&Ada tidakn%a ke'ainan /ge+a'a neuro'ogi
&Statu" puberta"/ tingkat $atura"i ke'a$in
&Pe$erik"aan -i"ik 'ain
999.Pe$erik"aan penun+ang: ('ihat tabe' )
&6ab rutin $encari pen%ebab in-ek"i "i"te$ik :
D6 / <6 / 56
&*one age / u$ur tu'ang
Kriteria a#a' untuk $e'akukan pe$erik"aan 'an+utan anak dengan pera#akan pendek:
1. T* diba#ah per"enti' ) atau ESD
. Kecepatan tu$buh diba#ah per"enti' /
). Prakiraan tinggi de#a"a diba#ah target height
,. <$ur tu'ang (bone age) ter'a$bat
Pe$erik"aan 'an+utan
&5ung"i tiroid
&Ana'i"i" kro$o"o$ ( pada #anita) : untuk diagno"i" "indro$ Turner
&<+i "ti$u'a"i / proFoka"i untuk hor$on pertu$buhan (haru" di'akukan o'eh dokter di
"ub.endokrino'ogi anak)

Terapi
9.Medika$ento"a
Pengobatan anak dengan pera#akan pendek haru" "e"uai dengan da"ar etio'ogin%a. Anak
dengan Faria"i nor$a' pera#akan pendek tidak $e$er'ukan pengobatan, "edang dengan
ke'ainan pato'ogi" terapi "e"uai dengan etio'ogin%a. :
N nutri"i
N pen%akit organik
N hor$ona'
N $echanika'/pe$bedahan

<ntuk terapi hor$on pertu$buhan
( di'akukan ata" adFi" dan penga#a"an dokter di "ub.endokrino'ogi anak ) :
Sebe'u$ terapi di$u'ai , kriteria anak dengan de-i"ien"i hor$on pertu$buhan haru"
ter'ebih dahu'u ditetapkan :
1. T* diba#ah per"enti' ke) atau ESD
. Kecepatan tu$buh diba#ah p./
). <"ia tu'ang ter'a$bat K tahun
,. Kadar (3 O 10 ng/$' pada u+i proFoka"i
/. Tidak ada di"$or-ik, ke'ainan tu'ang $aupun "indro$ tertentu.
Di"a$ping terapi untuk anak dengan de-i"ien"i hor$on pertu$buhan, terapi ini
diberikan +uga untuk anak dengan "indro$ Turner, "indro$ 8oonan, anak dengan 9<(A,
gaga' gin+a' kronik, "indro$ Prader Li''i, "indro$ 6eri&#ei''
3or$on pertu$buhan ini diberikan "ecara "c dengan do"i" 9</$/hari atau 0,0/
$g/kg/hari pada de-i"ien"i hor$on pertu$buhan dan 0,01 $g/kg/hari untuk "indro$a
Turner dan kronik rena' in"u--i"ien"i
Pe$berian diberikan "eban%ak ! ka'i per$inggu
Ko$p'ika"i terapi hor$on pertu$buhan :
N P"eudotu$or "erebri karena reten"i air dan natriu$ ( idiopathic intracrania'
h%perten"ion) : "angat +arang
N 5T, rendah ( tran"ient)
N 9n"u'in re"i"tance (+arang)
Kontraindika"i terapi hor$on pertu$buhan
N *'oo$ "%ndro$e
99.*edah
Pada ka"u" tertentu $i"a'n%a "ke'eta' d%"p'a"ia diper'ukan korek"i $echanica' /
pe$bedahan.(bone 'engthening)
Duga pada ka"u" karena tu$or
999.Suporti-
N p"%cho"ocia'

9=.6ain&'ain (ru+ukan "ub"pe"ia'i", ru+ukan "pe"ia'i"a"i 'ainn%a d'')
Se"uai etio'ogi

Pe$antauan (]MonitoringH)
9.Terapi
N Monitoring tinggi badan dan e-ek "a$ping
N Terapi hor$on dihentikan bi'a 'e$peng epi-i"i" te'ah $enutup atau re"pon terapi tidak
adekuat. 4iri re"pon terapi %ang tidak adekuat bi'a perta$bahan kecepatan pertu$buhan
'ebih keci' dari c$ da'a$ ! bu'an
N *i'a ada e-ek "a$ping p"eudotu$or cerebri karena reten"i air dan natriu$ ( pada
u$u$n%a di bu'an perta$a) dengan ke'uhan "akit kepa'a, $ua', pu"ing, ata;ia atau
gangguan peng'ihatan terapi "e$entara dihentikan
99.Tu$buh Ke$bang
Pera#akan pendek pato'ogi" pa"ti akan berpengaruh pada tu$buh ke$bang anak.
Diagno"i" dini dan terapi dini akan $e$perbaiki tu$buh ke$bang anak



6a$piran ta$bahan khu"u" untuk tabe', ga$bar, a'gorit$a.



/- Ta,!l P!r,!daan nor%al u&ia
kronologi& dan u&ia #ulang
U&ia kronologi& )#a+un0
/ SD
Laki1laki !r!%uan
)&! bu'an 0&1 0&1
1&1,/ tahun )&, &)
tahun 0&11 !&10
K tahun 1)&1, 1&1)
(Dikutip dari : 3ung Le''ington. (ro#th and deFe'op$ent: nor$a'
and Fariant .9n :Moore LT, ?a"t$an. Diagno"i"
?ndocrino'og%,
nd
ed,Mo"b%,122!,/&!)




3.Tabel :Important historical features in the evaluation of short stature
Hi&#ori*al 2!a#ur!& Diagno&#i* i%li*a#ion&
Materna' 3i"tor%
6ength o- ge"tation, preFiou" abortion",
co$p'ication" o- pregnanc%, "$ooking,
a'coho' and drug u"e
5eta' a'coho' "%ndro$e, 5eta' h%dantoin
"%ndro$e, T7A43 in-ection
Anthropo$etric Fa'ue"
*irth 'ength and #eight, d%"$orpho'i"$
9<(A, Turner "%ndro$e, other "hort "tature
"%ndro$e
8eonata' and deFe'op$enta' hi"tor%
8eonata' h%pog'%ce$ia, pro'ong neonata'
+aundice, deFe'op$enta' $i'e"tone"
3%popituitari"$, 3%poth%roidi"$
8utritiona' hi"tor%
9nadeGuate ca'oric intake
5ai'ure to thriFe
P"%cho"ocia' hi"tor%
4hi'd abu"e or neg'ect
P"%cho"ocia'"hort "tature
5a$i'% hi"tor%
(enetic "%ndro$e
Ske'eta' d%"p'a"ia, inborn error" o-
$etabo'i"$
3a%il" +!ig+#&. ag! o2 on&!# o2
u,!r#". ag! o2 %!nar*+!
5a$i'ia'"hort "tature, con"titutiona' de'a%ed
gro#th, "e;ua' $aturation
AeFie# o- "%"te$"
Spe"i-ic chronic organic di"order"
4ardiac, pu'$onar%, hepatic, rena' di"order"
Medica' hi"tor%
('ucocorticoid, "ti$u'ant
Drug&induced gro#th retardation
(Dikutip dari : 3ung Le''ington. (ro#th and deFe'op$ent: nor$a' and Fariant .9n: Moore LT, ?a"t$an. Diagno"i"
?ndocrino'og%, nded, Mo"b%,122!,/&!)



4- Ta,!l !%!rik&aan !nun5ang dan k!lainan klini&
P!%!rik&aan klini& K!lainan klini&
*one age
Ana'i"i" chro$o"o$
5S3
Sindo$a Turner
Skrining pen%akit "i"te$ik
pe$erik"aan darah
'engkap
'a+u endap darah
a'bu$in, creatinin, 8a,K,
ana'i"i" ga" darah
TS3 dan 5ree T,
4a'ciu$,pho"hor,a'ka'ine
Pho"phat
<rine rutin dan cu'tur
Ane$ia
Tuberku'o"i"
(aga' gin+a' kronik, Aena' tubu'ar a"ido"i"
3ipotiroid
=it D de-i"ien"i, Aicket",
h%popho"phate$ia
9n-ek"i gin+a'
(3 / 9(5&1 a;i"
9(5&1 dan 9(5*P&)
Te" "ti$u'a"i hor$on
De-i"ien"i hor$on pertu$buhan
Pencitraan
*one "urFe%
<S( kepa'a
Ske'eta' d%"p'a"ia
Adan%a de-ek "truktura' %ang dihubungkan
dengan de-i"ien"i hor$on pertu$buhan
atau de-i"ien"i hor$on hipo-i"i" $u'tip'e
pada ba%i
4T "can atau MA9 ?tio'ogi de-i"ien"i hor$on pertu$buhan
GRO'TH CHART
Satriono, M.Sc., SpA(K)
Terakhir diperbaharui pada / Page updated :10/10/00!

$$ 6 T$ ANAK LAKI1LAKI UMUR 77 1 89 $ULAN

$$ 6 T$ ANAK PEREMPUAN UMUR 77 1 89 $ULAN
$MI UNTUK ANAK LAKI1LAKI




$MI UNTUK ANAK PEREMPUAN
Endokrinologi Anak
KELAINAN GENITALIA PADA ANAK
Satriono, M.Sc., Dr., SpA(K)
Terakhir diperbaharui pada / Page updated :10/10/00!
(?89TA6 D9S7AD?AS 98 4396DA?8
MICROPENIS
The 'ength o- the nor$a' ne#born peni" i" )./ ^ 0.0 c$.
Micropeni" &&&&& O ) c$ (Age 1 E 11 %r)
?tio'og%
Micropeni" re"u't" -ro$ pri$ar% or "econdar% te"ticu'ar -ai'ure during -eta' 'i-e a-ter
$orphogene"i" i" co$p'ete.
Secondar% congenita' te"ticu'ar -ai'ure i" "een in anencepha'%, pituitar% agene"i", and
Ka''$ann, 8oonan, Prader&Li''i, and other "%ndro$e".
7ther ca"e" $a% be due to the pre"ence o- rudi$entar% te"te", d#ar-i"$, or $aterna'
hor$one ad$ini"tration".
E#iolog"
(onadotropin de-icienc%
(3 de-icienc% &&& Micropeni" Jh%pog'%ce$ia
3%popituitari"$e, pituitar% agene"i",_d#ar-i"$
Anencepha'%,
Anorchia,
Audi$entar% te"te"
Ka''$ann, 8oonan, Prader&Li''i "%ndro$e".
Materna' hor$one ad$ini"tration".
Tr!a#%!n#
Treat$ent option" inc'ude
a tria' o- hor$ona' "ti$u'ation (te"to"teron) , or
rearing a" -e$a'e, #ith 'ater genita' recon"truction.
Ad+u"t$ent to the $a'e gender ro'e and "e;ua' "ati"-action i" po""ib'e in "o$e o- the"e
patient".
Ag!n!&i& o2 #+! !ni&
Agene"i" o- the peni" i" rare and u"ua''% a""ociated #ith anorecta' and rena' ano$a'ie".
9- the chi'd i" 'ike'% to "urFiFe the a""ociated ano$a'ie", rearing a" a -e$a'e i"
reco$$ended, #ith 'ater genita' recon"truction.
<8D?S4?8D?D T?ST?S
<8D?S4?8D?D A8D ?4T7P94 T?ST?S.
5ai'ure to -ind one or both te"te" in the "crotu$ $a% indicate an% o- a Fariet% o-
congenita' or acGuired condition", inc'uding true unde"cended te"te", ectopic or
$a'de"cended te"te", retracti'e te"te", and ab"ent te"te".
True unde"cended te"te" and $a'de"cended or ectopic te"te" can be di--erentiated -ro$
each other on'% b% "urgica' e;p'oration, and both condition" u"ua''% are re-erred to a"
cr%ptorchidi"$ or hidden te"te".
The true unde"cended te"ti" i" -ound a'ong the nor$a' path o- de"cent, and the proce""u"
Fagina'i" i" u"ua''% patent. The ectopic te"ti" ha" co$p'eted it" de"cent through the
inguina' cana' but end" up in a "ubcutaneou" 'ocation other than the "crotu$, the $o"t
co$$on being a point 'atera' to the e;terna' inguina' ring, be'o# the "ubcutaneou" -a"cia.
CRYPTORCHIDISM
4r%ptorchidi"$ i" pre"ent in 0.0P o- chi'dren a-ter 1 %r o- age and in adu't". The
incidence i" high in -u''&ter$ ne#born" ().,P) and increa"e" #ith pre$aturit% (to 10P in
in-ant" #ith birth#eight" bet#een ,000 and ,/00 g and to 100P in tho"e under 200 g).
Thi" re-'ect" the -act that te"ticu'ar de"cent -ro$ the inguina' cana' into the "crotu$ take"
p'ace in the 0th $o o- ge"tation. Spontaneou" te"ticu'ar de"cent doe" not occur a-ter the
age o- 1 %r.
The con"eGuence" o- cr%ptorchidi"$ inc'ude in-erti'it% in adu'thood, tu$or deFe'op$ent
in the unde"cended te"te", a""ociated hernia", tor"ion o- the cr%ptorchid te"ti", and the
po""ib'e p"%cho'ogic e--ect" o- an e$pt% "crotu$. 4r%ptorchidi"$ i" bi'atera' in up to
)0P o- ca"e". 9n-erti'it% i" the ru'e in adu't" #ith untreated bi'atera' cr%ptorchidi"$, and
o- tho"e treated in chi'dhood 'e"" than one third #i'' be -erti'e. Lith uni'atera'
unde"cended te"ti", the rate o- in-erti'it% i" probab'% "i$i'ar to that in the genera'
popu'ation.
The unde"cended te"ti" i" o-ten hi"to'ogica''% nor$a' at birth, but -ai'ure o- deFe'op$ent
and atroph% are detectab'e b% the end o- the 1"t %r o- 'i-e, and b% the end o- the nd %r the
nu$ber o- ger$ ce''" in the a--ected te"ti" i" "eFere'% reduced. Surgica' correction at an
ear'% age re"u't" in a greater probabi'it% o- -erti'it% in adu'thood. The patient #ith
cr%ptorchidi"$ ha" a 0Zenda"h[E,,P increa"e in ri"k o- deFe'oping a $a'ignant
te"ticu'ar tu$or in the )rd or ,th decade o- 'i-e. Patient" #ith untreated intra&abdo$ina'
cr%ptorchidi"$ or tho"e #ho under#ent "urgica' correction during or a-ter pubert% are at
greate"t ri"k. A'though "urgica' correction o- the cr%ptorchidi"$ $a% not change the
oFera'' ri"k o- $a'ignant tran"-or$ation, Fer% -e# ca"e" o- tu$or" haFe been reported in
patient" #ho"e operation" #ere per-or$ed be-ore 1 %r o- age. 4arcino$a in "itu i"
occa"iona''% di"coFered #hen the te"ti" i" biop"ied at the ti$e o- orchiope;% or during
eFa'uation -or in-erti'it% 'ater in 'i-eV it" "igni-icance i" unc'ear.
The $o"t co$$on tu$or deFe'oping in unde"cended te"te" i" the "e$ino$a (!0P)V in
contra"t, "e$ino$a" repre"ent on'% )0P o- tu$or" occurring in nor$a''% de"cended
te"te".
9ndirect inguina' hernia" a'#a%" acco$pan% true unde"cended te"te" and are co$$on
#ith ectopic te"te". Tor"ion and in-arction o- the unde"cended te"ti" can occur becau"e o-
e;ce""iFe $obi'it% o- "uch te"te". The treat$ent o- the uni'atera' cr%ptorchid te"ti" i" be"t
undertaken ear'% in the nd %r o- 'i-e. Mo"t te"te" 'ocated e;tra&abdo$ina''% can be
brought do#n to the "crotu$ and the a""ociated hernia corrected #ith an operation
(orchiope;%). Thi" can o-ten be per-or$ed #ithout ho"pita'iXation. Lhen the te"ti" i" not
pa'pab'e, preoperatiFe 'aparo"cop% i" u"ed to deter$ine it" 'ocation. 9n the $a+orit% o-
ca"e", orchiope;% o- the intra&abdo$ina' te"ti" 'ocated i$$ediate'% in"ide the interna'
inguina' ring o--er" 'itt'e di--icu't%, but orchiecto$% "hou'd be con"idered in the $ore
di--icu't ca"e" or #hen the te"ti" appear" to be "eFere'% atrophied. T#o&"tage orchidope;%
i" "o$eti$e" needed in high abdo$ina' te"te". Te"ticu'ar pro"the"e" are aFai'ab'e -or
o'der chi'dren and ado'e"cent" #hen the ab"ence o- the gonad in the "crotu$ $a% haFe an
unde"irab'e p"%cho'ogic e--ect but, the adFi"abi'it% o- u"ing "i'icone i$p'ant" ha" been
Gue"tioned.
Treat$ent o- bi'atera' unde"cended te"te" i" identica' to the treat$ent o- uni'atera'
unde"cended te"ti" #hen the te"te" are pa'pab'e. Lhen te"te" are not pa'pab'e, ho#eFer,
di--erentia' diagno"i" $u"t be $ade -ro$ ab"ent te"te" b% $ea"uring "eru$ te"to"terone
'eFe'" be-ore and a-ter "ti$u'ation #ith hu$an chorionic gonadotropin (h4(). 9- the
te"to"terone 'eFe' ri"e", an abdo$ina' e;p'oration and orchiope;% "hou'd be undertaken.
A negatiFe re"pon"e doe" not ru'e out the po""ib'e e;i"tence o- intra&abdo$ina' te"ticu'ar
ti""ue. An atte$pt i" $ade to pre"erFe the"e gonad" -or hor$ona' production a-ter
pubert%V the 'ike'ihood o- pre"erFing -erti'it% i" Fer% 'o#.
Hor%onal #r!a#%!n#
3or$ona' treat$ent #ith
h4( or
'uteiniXing hor$onere'ea"ing hor$one (63&A3) .
So$e be'ieFe that preoperatiFe treat$ent #ith h4( -aci'itate" "urger%.
Aecent report" on the adFantage" o- hor$ona' treat$ent #ith 63&A3 -o''o#ed b% 34(
-or nonre"ponder", and ear'% "urger% -or the !0P o- te"te" that -ai' to de"cend, need to be
#eighed again"t potentia' detri$enta' e--ect" on puberta' peni'e gro#th o- ear'% e;po"ure
o- the peni'e receptor" to te"to"terone.

A?TAA4T96? T?ST?S.
The"e te"te" retract into the inguina' cana' in re"pon"e to an e;aggerated cre$a"teric
re-'e;. The cre$a"teric re-'e; i" #eak or ab"ent at birth. 4on"eGuent'%, #hen te"te" that
#ere pa'pab'e at birth beco$e nonpa'pab'e 'ater, retracti'e te"te" "hou'd be "u"pected.
Aetracti'e te"te" can be brought do#n b% care-u' pa'pation #hen the chi'd i" re'a;ed in a
#ar$ roo$, and "crota' e;a$ination i" -aci'itated i- the chi'd i" in a "Guatting po"ition.
7-ten $ore than one e;a$ination i" reGuired to e"tab'i"h the diagno"i". The retracti'e
te"ti" u"ua''% adopt" a per$anent "crota' po"ition during pubert% and ha" none o- the
co$p'ication" co$$on'% a""ociated #ith the true unde"cended or ectopic te"ti".
A*S?8T T?ST?S.
Appro;i$ate'% 0P o- nonpa'pab'e te"te" are ab"ent. 4ongenita' ab"ence o- the te"ti" i"
po""ib'e, but it i" Guite rare and $a% be a""ociated #ith "o$e degree o- -e$iniXation o-
the interna' organ" on the ip"i'atera' "ide. More co$$on'%, the -eta' te"ti" di"appear"
"o$e ti$e a-ter the di--erentiation o- the interna' and e;terna' genita'ia ha" occurred. Thi"
Fani"hing o- the te"ti" i" u"ua''% attributed to a Fa"cu'ar accident that ha" taken p'ace
prenata''% or a-ter birth but #a" not recogniXed c'inica''%. At e;p'oration, the "per$atic
Fe""e'" and the Fa" de-eren" end b'ind'%, u"ua''% "o$e#here in the inguina' region or in
the "crotu$. *ecau"e thi" condition i" ana'ogou" to te"ticu'ar tor"ion, "o$e author"
adFocate -i;ation o- the contra'atera' te"ti" to preFent tor"ion -ro$ occurring in the
re$aining gonad. 9n the"e ca"e", p'ace$ent o- a te"ticu'ar pro"the"i" can be con"idered a"
#e''.


Endokrinologi Anak
HIPERPLASIA ADRENAL KONGENITAL-
Satriono, M.Sc., Dr., SpA(K)
Terakhir diperbaharui pada / Page updated :10/10/00!
Cong!ni#al Adr!nal H"!rla&ia
PAT37(?8?S9S.
Lhen the adrenogenita' "%ndro$e i" a""ociated #ith congenita' adrena' h%perp'a"ia, it i"
cau"ed b% a -a$i'% o- auto"o$a' rece""iFe di"order" o- adrena' "teroidogene"i" 'eading to
a de-icienc% o- corti"o' .
The de-icienc% o- corti"o' re"u't" in increa"ed "ecretion o- corticotropin, #hich 'ead" in
turn to adrenocortica' h%perp'a"ia and oFerproduction o- inter$ediar% $etabo'ite".
SeFere and $i'd -or$" o- the"e di"order", cau"ed b% Fariation" in the "eFerit% o- the
genetic $utation", haFe been reported.
4ongenita' Adrena' 3%perp'a"ia
De-icienc% o- 1&h%dro;%'a"e account" -or 2/P o- a--ected patient".

469894A6 MA895?STAT978S
Mo"t patient" #ith congenita' adrena' h%perp'a"ia haFe the de-ect in 1&h%dro;%'ation
and e;hibit the c'a""ic -or$ o- the di"ea"e.
#ith "creening 0/P o- in-ant" are "a't 'o"er",
#ithout "creening /0P o- c'inica''% diagno"ed in-ant" are "a't 'o"er", pre"u$ab'% becau"e
o- undiagno"ed neonata' death".


5igure /2E. A, A !&%r&o'd gir' #ith congenita' Firi'iXing
adrena' h%perp'a"ia. The height age #a" 1./ %rV the bone age
#a" 1) %rV and urinar% 10&keto"teroid" #ere /0 $g/, hr.
*, 8otice the c'itora' en'arge$ent and 'abia' -u"ion.
4, 5iFe&%r&o'd brother o- gir' in A #a" not con"idered to be
abnor$a' b% the parent". The height age #a" 1 %rV the bone
age #a" 1./ %rV and the urinar% 10&keto"teroid" #ere )!
$g/, hr.



5igure /2E). Three -e$a'e p"eudohe$aphrodite" #ith
untreated congenita' adrena' h%perp'a"ia. A'' #ere
erroneou"'% a""igned $a'e "e; at birth, and each had nor$a'
-e$a'e "e;&chro$o"o$e co$p'e$ent. 9n-ant" A and * #ere
"a't 'o"er" and #ere diagno"ed in ear'% in-anc%. 9n-ant 4
#a" re-erred at 1 %r o- age becau"e o- bi'atera'
cr%ptorchidi"$.
8otice the co$p'ete'% peni'e urethraV "uch co$p'ete degree"
o- $a"cu'iniXation in -e$a'e" #ith adrena' h%perp'a"ia are
rareV $o"t o- the"e in-ant" are "a't 'o"er".
LATIHAN $ELAJAR HIPOTIROIDISME
Satriono, M.Sc., SpA(K)

1.D?5989S9 3ipotiroidi"$e:
1 .De-i"ien"i hor$on T,
.De-i"ien"i hor$on tirok"in
).De-i"ien"i hor$on T)
,.De-i"ien"i hor$on triiodo tironin
.Pen%ebab terban%ak 3ipotiroidi"$e Kongenita' dengan ge+a'a "tru$a.
A.Di"gene"i" /Atireo"i" tiroid.
*.D%"hor$ogene"i"
4.7bat&obatan (KD, (oitrogen)
D.Th%roid 3or$on? <nre"pon"iFene""
?.De-i"i"en"i TS3
). Si$pto$ 39P7T9A79D9SM? *A>9 "ebagai berikut, K?4<A69
A.7b"tipa"i
*.Ma'a" $enetek (Minu$ "u"u)
4.*a%i hiper akti-
D.9kteru" neonatoru$ Pro'ongatu"
?.3ipoter$i
,.Sign" ((e+a'a K'inik) 39P7T9A79D9SM? *A>9 %ang di"ebabkan tiroid ektopik:
1.3ipotoni
. Di"tenden Abdo$en
).3ernia u$bi'ika"'i"
,.(ondok
/.Sign" ((e+a'a K'inik) 39P7T9A79D9SM? *A>9 "ebagai berikut, K?4<A69
A.S%ndne% 6ine
*.(ondok
4. Dantung : Kardio$ega'i
D.Dantung: *i"ing J
?. 5ontane'a po"t terbuka #aktu 'ahir
!. Si$to$ 39P7T9A79D9SM? A8AK
1.Pertu$buhan Menta' ter'a$bat
.Pertu$buhan 5i"ik ter'a$bat
).Pertu$buhan Denti"i er'a$bat
,.Pertu$buhan Puberta" ter'a$bat
0.Sign" ((e+a'a K'inik) 39P7T9A79D9SM? A8AK
1.(oiter bi"a ada / tidak
.Ku'it: Pucat
).Ku'it: Teba',
,.Ku'it:Karotine$ik,
1. Pe$erik"aan M Aa% pada 39P7T9A79D9SM? A8AK :
1. di"gene"i" epi-i"i" (Li'kin)
. i'iac inde; $eningkat
). *one Age ter'a$bat
,. "udut a"etabu'u$ $enurun
2. Pe$erik"aan %ang tak ter'a'u per'u diker+akan pada hipotiroidi"$e ba%i/anak:
A.Scanning Tiroid
*.??(
4.?K(
D.?M( (?'ectro$%ogra$)
?. *MA
10.Diagno"tik 39P7T9A79D9SM? :
1.3iperko'e"tero'e$ia
.3b rendah
). *one Age ter'a$bat
,.TS3 "en"iti- $eningkat
11.Diagno"tik 39P7T9A79D9SM? :
1.3ipotoni
.9kteru" neonatoru$ Pro'ongatu"
). *one Age ter'a$bat
,.?K( 6o# =o'tage
1. Sekarang untuk terapi hipotiroidi"$e digunakan:
1. Sodiu$ 6eFo Th%ro;in
. Th%ra; (7rganon)
). ?uth%ro; (Merck)
,.Th%ranon
1).Pen%akit/Ke'ainan %ang haru" di Diagno"i" *anding dengan hipotiroidi"$e:
1.Kondrodi"tro-i
.D#ar-i"$
).Turner S%ndro$e
,.Do#n." S%ndro$e
1,.*i'a hipotiroidi"$e diterapi ter'a$bat / tanpa terapi akan berakibat
1.Ke$atian o.k.: 7b"truk"i "a'uran na-a"
.Ke$atian o.k.: 9n-ek"i
).Menta' Aetardation
,.SeGue'e 8ero'ogik : inkoordina"i
1/. <D9 SAA98( (S4A??898( T?ST) 39P7T9A79D9SM? K78(?89TA6 PADA
8?78AT<S dipakai :
A.TA3
*.TS3
4.T*(
D.T)
?.T,
1!. 3a'&ha' %ang benar $engenai "eorang anak 'aki&'aki %ang didiagno"i" hipotiroidi"$e
pada u$ur ) tahun ke$udian $endapat terapi hor$one tirok"in adekuat "e'a$a , tahun ,
keadaann%a pada u$ur 0 tahun:
1. 3eight Age (<$ur Pera#akan) @ 0 tahun.
. 9B rendah .
). *one Age (<"ia tu'ang) @ 0 tahun
,. *radikardi
10..K6AS959KAS9 39P7T9A79D9SM? berda"arkan ter+adin%a
A.. 39P7T9A79D9SM? K78(?89TA6 : 39P7T9A7D9SM? D9DAPAT
(A4B<9A?D)
*. 39P7T9A79D9SM? ?8D?M9K : 39P7T9A79D9SM? SP7AAD9K
4. 39P7T9A79D9SM? *A>9 : 39P7T9A79D9SM? A8AK
D. 39P7T9A79D9SM? PA9M?A,S?K<8D?A,T?AT9?A
?. 39P7T9A79D9SM? D?8(A8 STA<MA : 39P7T9A79D9SM? TA8PA
STA<MA
11.Pen%ebab terban%ak 3ipotiroidi"$e didapat (AcGuired) "poradik:
A.7pera"i
*.Auto i$$une Di"ea"e& Tiroidit" 6i$-o"itik Kronik (3AS39M7T7)
4.9n-ek"i
D.7bat&obatan
?.De-i"ien"i 9odiu$
12. Si$pto$ 39P7T9A79D9SM? *A>9 "ebagai berikut, K?4<A69
A.7b"tipa"i
*.Ma'a" $enetek (Minu$ "u"u)
4.6etargi & $au tidur "a+a
D.9kteru" neonatoru$ Pro'ongatu"
?.Makrog'o"i
0.Sign" ((e+a'a K'inik) 39P7T9A79D9SM? *A>9 :
1. Dantung : Kardio$ega'i
.Dantung: *i"ing J
). 5ontane'a po"t terbuka #aktu 'ahir
,.*ru"-ie'dW" "pot
1.Sign" ((e+a'a K'inik) 39P7T9A79D9SM? *A>9 "ebagai berikut, K?4<A69
A.3ernia u$bi'ika"'i"
*.$ik"ede$a
4.8%"tag$u"
D.3ipotoni
?.Muka Se$bab
. Si$to$ 39P7T9A79D9SM? A8AK
1.(e$uk
.Stru$a
).*odoh
,.Cold Intolerance
).Sign" ((e+a'a K'inik) 39P7T9A79D9SM? A8AK
1.Ku'it: Dingin,
.Mik"ede$a
).3ipotoni E 7tot 6e$bek
,.APA , KPA E 6a$bat
,. Pe$erik"aan 3or$ona' pada 39P7T9A79D9SM? A8AK :
1.5ree T, $enurun
. TS3 "en"iti- $enningkat
).T, tota' $enurun
,.TS3 $eningkat
/.<ntuk bedakan 3 Pri$er dan 3 Sekunder d'akukan :
A.<+i TS3
*.<+i TA3
4.<+i hau"
D.?M(
?.TSH Screening Test
!.Diagno"tik 39P7T9A79D9SM? :
1 5ree T, $enurun
.??( 6o# =o'tage
).?K( 6o# =o'tage
,.?M( (?'ectro$%ogra$) $e$an+ang
0.Diagno"tik 39P7T9A79D9SM? :
1.Muka Se$bab
.3ipoter$i
).TS3 "en"iti- $eningkat
,.?M( (?'ectro$%ogra$) $e$an+ang
1. Pengobatan hipotiroidi"$e kongenita':
1. Di'akukan untuk "eu$ur hidup.
.Menggunakan obat ek"trak tiroid.
).Menggunakan "odiu$ 'eFo th%ro;in
,.Menggunakan obat "teroid.
2.Pen%akit/Ke'ainan %ang haru" di Diagno"i" *anding dengan hipotiroidi"$e:
1.Akondrop'a"ia
.3ipopituitari"$e
).Turner S%ndro$e
,.Do#n." S%ndro$e
)0.*i'a hipotiroidi"$e diterapi ter'a$bat / tanpa terapi akan berakibat
1.Ke$atian o.k.: 7b"truk"i "a'uran na-a"
.Ke$atian o.k.: 9n-ek"i
).Menta' Aetardation
,.SeGue'e 8ero'ogik : atak"i,"pa"tik
)1. 6aborator% eFidence o- c'a""ic congenita' h%poth%roidi"$ in a "a$p'e o- b'ood taken
on the third da% o- 'i-e inc'ude"
A. e'eFated TS3, 'o# T,
*. e'eFated TS3, high T)
4. 'o# TS3, 'o# T,
D. 'o# T,, high T)
?. 'o# T*(, 'o# T,
).Pen%ebab 3ipotiroidi"$e Kongenita' dengan kadar -ree T, %ang nor$a'..
A.Di"gene"i" /Atireo"i" tiroid.
*.D%"hor$ogene"i"
4.7bat&obatan (KD, (oitrogen)
D.Th%roid 3or$on? <nre"pon"iFene""
?.De-i"i"en"i TS3
LATIHAN $ELAJAR HIPOTIROIDISME DD NYA DO'N:S SYNDROME
Satriono, M.Sc., SpA(K)
Terakhir diperbaharui pada / Page updated :10/10/00!

1. D7L8.S S>8DA7M? (DS)
1.Dipub'ika"i perta$a ka'i tahun 11!!
.Di"ebut +uga Mongo'i"$e
).9n"iden" : 1& /1000 ke'ahiran
,.?tio'ogi dike$ukanan o'eh 6e Deune dkk tahun 12/2

. TA9S7M9 1 pada do#n. "%ndro$e
1. Tri"o$i Aegu'er 2/ P
. Tran"'oka"i ,&/ P
).Mo"aik Q & 1 P
,.>ang $ene$ukan 6e Deune dkk tahun 12/2

).(e+a'a K'inik DS pada MATA
1.8i"tag$u"
.Strabi"$u"
).3iperte'ori"$e
,.*ru"-ie'd." Spot


,.(a$bar di "a$ping ini ini ada'ah :
A.AA4<S @ *<S<A
*.677P @ 6?8(K<8(
4. TA9 AAD9<S
D.P<SAAA8 (L37A6)
?. T?8T?D AA43
LATIHAN $ELAJAR DIA$ETES MELLITUS PADA ANAK
Satriono, M.Sc., SpA(K)
Terakhir diperbaharui pada / Page updated :10/10/00!


Diabete" $e''itu" in 4hi'dren:
1. i" a "%ndro$e o- di"turbed energ% ho$eo"ta"i" cau"ed b% a de-icienc% o- in"u'in and
re"u'ting in abnor$a' $etabo'i"$ o- carboh%drate, protein, and -at.
.9t i" the $o"t co$$on endocrine&$etabo'ic di"order o- chi'dhood and ado'e"cence .
).i$portant con"eGuence" -or ph%"ica' and e$otiona' deFe'op$ent.
, i" a "%ndro$e o- di"turbed energ% ho$eo"ta"i" cau"ed b% in"u'in action and re"u'ting
in abnor$a' $etabo'i"$ o- carboh%drate, protein, and -at.

. 4'a""i-ication o- Diabete" Me''itu" in 4hi'dren and Ado'e"cent" -or. 9n"u'inEdependent
(9DDM, t%pe 9):
ba"ed on criteria:
1g'uco"uria
. ketonuria,
).rando$ p'a"$a g'uco"e (P() K00 $g/d6
,.5P( O1,0 $g/d6 #ith &hr Fa'ue K1,0 $g/d6 during 7(TT

).Diabete" $e''itu" in 4hi'dren:
1.Ma'e" and -e$a'e" are a'$o"t eGua''% a--ectedV
.there i" no apparent corre'ation #ith "ocioecono$ic "tatu".
).Peak" o- pre"entation occur at /E0 %r o- age
,.Peak" o- pre"entation occur at the ti$e o- pubert%

,.The c'a""ic pre"entation o- diabete" in chi'dren :
1. po'%uria,
.po'%dip"ia,
).po'%phagia,
,.#eight gain


/. Ketaocido"i" in diabetic chi'dren.
1. appro;i$ate'% /P o- diabetic chi'dren
.The ear'% $ani-e"tation" con"i"t o- Fo$iting.
).The ear'% $ani-e"tation" con"i"t o- po'%uria..
,.The ear'% $ani-e"tation" con"i"t deh%dration.


/. Ketaocido"i" in diabetic chi'dren.
1. appro;i$ate'% 2/P o- diabetic chi'dren
.The ear'% $ani-e"tation" con"i"t o- Fo$iting.
).The ear'% $ani-e"tation" con"i"t o- d%"uria..
,.The ear'% $ani-e"tation" con"i"t deh%dration


/.4'inica' "ign 9n diabetic chi'dren #ith $ore pro'onged and "eFere ca"e" o- Ketaocido"i"
:
A.Fo$iting.
*. po'%uria..
4. deh%dration
D.Ku""$au' re"piration"
?. Di"pnoe d.e--ort.
.
!.The diagno"i" o- diabete" $e''itu" in chi'dren ba"ed on the de$on"tration o- :
1.h%perg'%ce$ia
.g'uco"uria #ith or #ithout ketonuria.
).decrea"ed 4&peptide
,.increa"ed 3bA1c

0.the princip'e o- treat$ent o- diabete" $e''itu" in chi'dren :
1.in"u'in
.nutrition "upport
).e;erci"e
,.education and coun"e'ing

1. 3a'&ha' %ang ada hubungann%a dengan PA7(87S9S DM pada anak:
1.Sering terdapat ko$p'ika"i =i"ua' dan $ata.
.Puberta" dapat ter'a$bat
).Di'aporkan adan%a ke$atian akibat bunuh diri.
,.Tinggi badan akhir $a"ih da'a$ bata" nor$a' .
LATIHAN $ELAJAR STRUMA PADA ANAK-
Satriono, M.Sc., SpA(K)
Terakhir diperbaharui pada / Page updated :10/10/00!

(rada"i pe$be"aran ke'en+ar tiroid $enurut L37:
A.Dera+at 7, 1 A, 1* , dan ).
*.Dera+at 7, 1 , dan ).
4.Dera+at 7, 1 A, 1* ,
D.Dera+at 7, 1 , .
?.Dera+at 7, 1 , .)


5966 98 :
(rada"i pe$be"aran ke'en+ar tiroid pada "urFe% (AK9 di 9ndone"ia
Dera+at ``..,````,````,````,```,``...

(AADAS9 M?8<A<T L37 ```. , ```.., ```..
Stru$a bi"a digo'ongkan ke da'a$ :
1```````````..
```````````..
)```````````..'
PAT759S9767(9 T?ADAD98>A STA<MA:
1`````````````
```````````
)````````.
LATIHAN $ELAJAR GANGGUAN PERTUM$UHAN -
Satriono, M.Sc., SpA(K)
Terakhir diperbaharui pada / Page updated :10/10/00!

P?AALAKA8 P?8D?K
1.Pera#akan pendek atau "hort "tature $erupakan "uatu ter$ino'ogi $engenai
pan+ang/tinggi badan %ang berada di ba#ah ```.. atau ```` pada kurFa
pertu$buhan %ang ber'aku pada popu'a"i ter"ebut.
A. per"enti' ke ) , E) Standard DeFiation
*. per"enti' ke ) , E Standard DeFiation
4. per"enti' ke ) , E1 Standard DeFiation
D. per"enti' ke / , E Standard DeFiation
?. per"enti' ke / , E) S Standard DeFiation
.Pera#akan pendek pri$er / in"trin"ik:
A. 3ipotiroidi"$e
*.Sindro$ Turner
4 .cu"hing "indro$
D.3%pogonadi"$
?.Aicket"
Ada beberapa k'a"i-ika"i pera#akan pendek %aitu :
a. .......................................
b. ......................................
c. ........................................
d. .....................................

b. Pera#akan pendek pri$er / in"trin"ik
1.Sindro$&"indro$ %ang dihubungkan dengan ke'ainan kro$o"o$
N Sindro$ Turner
N Sindro$ Do#n
.Sindro$ E"indro$ 'ain, $i"a'n%a:
N Sindro$ 8oonan
N Sindro$ Prader&6abhart&Li''i
N Sindro$ Au""e'' Si'Fer
N Sindr$e Secke'
N Sindro$ 3utchin"on (i'-ort
N Sindro$ 4ocka%ne
c. Pera#akan pendek "ekunder / e;trin"ik
1. Pen%akit / ke'ainan "i"te$ik
(chronic di"ea"e)
Mi"a'n%a ke'ainan +antung, paru, 'iFer, inte"tina', rena', he$ato'ogi, 48S dan genera'iXed
in-'a$$ator% di"ea"e, in-ek"i kronik, ane$ia kronik,$a'ab"orb"i
. Ma'nutri"i
). ke'ainan endokrin
N 3ipotiroid
N (ro#th hor$on de-i"ien"i
N 9(5&1 de-ect
N P"eudoh%poparath%roidi"$
N The cu"hing "indro$
N Mauriac "%ndro$e (karena regu'a"i g'uko"a %ang +e'ek pada pa"ien diabete" $e''itu")
N 3%pogonadi"$
N Aicket"
LATIHAN $ELAJAR KELAINAN GENITALIA PADA ANAK-
Satriono, M.Sc., SpA(K)
Terakhir diperbaharui pada / Page updated :10/10/00!

1.Micropeni" (Age 1 E 11 %r) i- 'ength o- the peni" :
A. O 1 c$
*. O c$
4.O ,/ c$
D. O ) c$
? O , c$
S7A6 5966 98
3or$ona' treat$ent -or cr%ptorchidi"$ b% u"ing :.....................................
or ..................................
S7A6 *?8AA/SA6A3
So$e be'ieFe that preoperatiFe treat$ent #ith h4( -aci'itate" "urger%.
DALA*: A.*?8AA
*.SA6A3
LATIHAN $ELAJAR HIPERPLASIA ADRENAL KONGENITAL-
Satriono, M.Sc., SpA(K)
Terakhir diperbaharui pada / Page updated :10/10/00!

1. Pen%ebab 4ongenita' Adrena' 3%perp'a"ia terban%ak:
A.De-icienc% o- 1&h%dro;%'a"e
*.?;ce"" o- 1&h%dro;%'a"e
4. Tri"o$% 1
D.Tri"o$% 11
?. De-i"ien"i (nA3


. Pada pe$erik"aan kro$o"o$e ketiga ba%i di ata" ,!, MM.
Ketiga ba%i t"b ada'ah: ``````. Dengan ``````````
A. $a'e p"eudohe$aphrodite" , <ntreated congenita' adrena' h%perp'a"ia
*. -e$a'e p"eudohe$aphrodite" , <ntreated congenita' adrena' h%perp'a"ia
4. $a'e p"eudohe$aphrodite" , cr%ptorchi"i$
D. he$aphrodite" , <ntreated congenita' adrena' h%perp'a"ia
?. he$aphrodite"
5966 98
Lhen the adrenogenita' "%ndro$e i" a""ociated #ith congenita' adrena' h%perp'a"ia, it i"
cau"ed b% a -a$i'% o- ``````````.. di"order" o- adrena' "teroidogene"i"
'eading to a de-icienc% o- ```````.. .
The de-icienc% o- corti"o' re"u't" in increa"ed "ecretion o- ``````., #hich 'ead" in
turn to adrenocortica' h%perp'a"ia and oFerproduction o- inter$ediar% $etabo'ite".
SeFere and $i'd -or$" o- the"e di"order", cau"ed b% Fariation" in the "eFerit% o- the
genetic $utation", haFe been reported.
4ongenita' Adrena' 3%perp'a"ia
De-icienc% o- ```````````` -or 2/P o- a--ected patient".