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Case Report

CHRONIC KIDNEY DISEASE


Presenter : Agus Salim (100100027)
Seprima Yenti (100100029)
Supervisor : dr. ell! Rosdiana" Sp.A (#)
INTRODUCTION
C$roni% #idne! &isease (C#&) is a patop$!siologi% pro%ess 'it$ various etiolog! '$i%$
%ause de%rease renal (un%tion progressivel!. )$is is a %lini%al %ondition '$ere t$e renal
d!s(un%tion is irreversi*le and lasting until renal repla%ement t$erap! (dial!sis" renal
transplantation) is needed.
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C#& is a serious pu*li% $ealt$ pro*lem 'it$ national surve!s s$o'ing a %ondirea*l!
$ig$er prevalen%e t$an appre%iated previousl!.
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Re%ent eviden%e indi%ates t$at t$e out%omes %an
*e improved *! earl! diagnosis and treatment.
+
,arlier stages o( C#& %an *e dete%ted t$roug$
routine la*orator! measurements. )$e presen%e o( C#& s$ould *e esta*lis$ed" *ased on presen%e
o( -idne! damage and level o( -idne! (un%tion (glomerular (iltration rate ./0R1).
2

C$ildren '$o $ave C#& ma! present to %lini%ians 'it$ a %om*ination o( pro*lems
involving gro't$" nutrition" ele%trol!te distur*an%es" renal osteod!strop$!" anemia"
immuni3ations" $!pertension" and renal transplantation. C$ildren 'it$ C#& need a
%ompre$ensive treatment. A team'or- %onsist o( pediatri% nep$rologist"nutritionist" psi-ologist
or ps!%$iatri% and patient (amil!.
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EPIDEMIOLOGY
/lo*all!" t$e prevalen%e o( %$roni% -idne! disease (C#&) stage 55 or lo'er in %$ildren is
reported to *e appro6imatel! 17.4847.+ per million %$ildren.
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&ata (rom t$e 5tal#id stud!
reported a mean in%iden%e o( 12.1 %ases per !ear per million in t$e age8related population (age
range" 7.781+.9 !) and a prevalen%e o( 72.7 per million in t$is population.
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)$e (re:uen%! o(
%$roni% -idne! disease in%reases 'it$ age and is mu%$ more %ommon in adults t$an %$ildren.
Among %$ildren" %$roni% -idne! disease is more %ommon in %$ildren older t$an 9 !ears t$an in
t$ose !ounger t$an 9 !ears. )$e per%entages in t$e APR)CS %o$ort 'ere 19; in %$ildren aged
081 !ears< 17; in t$ose aged 9812 !ears< ++; in %$ildren aged 284 !ears< and +1; in t$ose older
t$an 12 !ears.
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5n 5ndonesia" t$ere is no prevalen%e o( C#& in %$ildren nationall!. =ased on stud! in 7
$ospital in 5ndonesia" 2779 %$ildren '$o 'ere treated 'it$ renal disease (!ears 197281977)" 2;
o( t$em are diagnosed 'it$ C#&.RSC> ?a-arta (ound 2.9; (rom 997 o( %$ildren '$o are
$ospitali3ed 'it$ -idne! disease are diagnosed 'it$ C#&. )$e in%iden%e in t$e /eneral @ospital
&r. Soetomo (or 4 !ears (197781992) is 0.07; o( all patients $ospitali3ed in t$e pediatri% 'ard.
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DEFINITION AND STAGING
C$roni% -idne! disease (C#&) is t$e presen%e o( -idne! damage lasting (or at least +
mont$s 'it$ or 'it$out a de%reased /0R or an! patient '$o $as a /0R o( less t$an 90 mABmin
per 1.7+ m
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lasting (or + mont$s or more" irrespe%tive o( diagnosis ()a*el 1).
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)a*el 1. Criteria (or de(inition o( C#&
A patient $as C#& i( eit$er o( t$e (ollo'ing %riteria are present :
1. #idne! damage (or C+ mont$" as de(ined *! stru%tural or (un%tional a*normalities o( t$e
-idne!" 'it$ or 'it$out de%rease /0R" mani(ested *! 1 or more o( t$e (ollo'ing (eatures:
A*normalities in t$e %omposition o( t$e *lood or urine
A*normalities in imaging tests
A*normalities on -idne! *iops!
2. /0R D90 mA.minB1.7+ m
2
(or C+ mont$" 'it$ or 'it$out t$e ot$er signs o( -idne! damage
des%ri*ed a*ove.
#idne! &isease Eut%omes &isease 5nitiative" 2012. Clinical Practice Guideline for Chronic
Kidney Disease : Evaluation, Clasification and Stratification.
)$e normal level o( /0R varies a%%ording to age" gender" and *od! si3e. )$e normal
/0R in !oung adults is 120 to 1+0 mABminB1.7+ m
2
" '$ereas t$e normal level o( /0R is mu%$
lo'er t$an t$is in earl! in(an%!" even '$en %orre%ted (or *od! sur(a%e area" and su*se:uentl!
in%reases in relations$ip to *od! si3e (or up to 2 !ears.
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Staging o( C$roni% #idne! &isease *ased
on #&EF5 is s$o'n in ta*el 2.
)a*el 2. #&EF5 Classi(i%ation o( t$e stage o( C#&
Stage GFR
(mL/min/1.! m
"
#
De$%&i'ti(n A%ti(n P)an
1 C90 #idne! damage 'it$ normal or
in%reased /0R
)reat primar! and %omor*id %onditions"
slo' C#& progression" CG& ris-
redu%tion
2 90879 #idne! damage 'it$ mild
redu%tion o( /0R
,stimate rate o( progression
+ +0849 >oderate redu%tion o( /0R ,valuate and treat %ompli%ations
2 14829 Severe redu%tion o( /0R Prepare (or -idne! repla%ement t$erap!
4 D14 ,nd Stage renal (ailure #idne! repla%ement t$erap!
#idne! &isease Eut%omes &isease 5nitiative" 2012. Clinical Practice Guideline for Chronic
Kidney Disease : Evaluation, Clasification and Stratification.
ETIOLOGY
C#& $as a prevalen%e o( 1.4 to +.0 per 1.000.000 among %$ildren !ounger t$an t$e age
o( 19 !ears. 5n %$ildren" C#& ma! *e t$e result o( %ongenital" a%:uired" in$erited" or meta*oli%
renal disease" and t$e underl!ing %ause %orrelates %losel! 'it$ t$e age o( t$e patient at t$e time
'$en t$e C#& is (irst dete%ted. C#& in %$ildren D4 !ears old is most %ommonl! a result o(
%ongenital a*normalities su%$ as renal $!poplasia" d!splasia" or o*stru%tive uropat$!. Additional
%auses in%lude %ongenital nep$roti% s!ndrome" prune *ell! s!ndrome" %orti%al ne%rosis" (o%al
segmental glomerulos%lerosis" pol!%!sti% -idne! disease" renal vein t$rom*osis" and $emol!ti%
uremi% s!ndrome. A(ter 4 !ears o( age" a%:uired diseases (various (orms o( glomerulonep$ritis
in%luding lupus nep$ritis) and in$erited disorders ((amilial Huvenile nep$ronop$t$isis" Alport
s!ndrome) predominate. C#& related to meta*oli% disorders (%!stinosis" $!pero6aluria) and
%ertain in$erited disorders (pol!%!sti% -idne! disease) %an o%%ur t$roug$out t$e %$ild$ood !ears.
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Everall" t$e most %ommon %auses o( C#& in %$ildren are urologi% a*normalities (+0; to
++;) and glomerulopat$ies (24; to 27;). )$ese t'o a*normalities a%%ount (or more t$an 40;
o( t$e reported %auses o( end8stage renal disease in %$ildren. )$e ot$er maHor %auses are
$ereditar! nep$ropat$ies (19;) and renal $!poplasia and d!splasia (11;). &ata (rom t$e ort$
Ameri%an Pediatri% Renal )rial and Colla*orative Studies 2009 demonstrate ver! similar
in(ormation ()a*le +).
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)a*el +.Common Cause o( C$roni% #idne! &isease in C$ildren
Diagn($i$ In%i*en%e
E*stru%tive uropat$! 22;
AplasiaB$!poplasiaBd!splasia 17;
/lomerulonep$ritis 10;
Re(lu6 nep$ropat$! 7;
&il!s A. I$!te and Ri%$ard 0ine" 2007. C$roni% #idne! &isease in C$ildren.
PATOPHYSIOLOGY
Renal (un%tion de%rease progressivel! despite t$e primar! disease $as *een resolved or
$ave *e%ome ina%tive. )$is %ondition indi%ate a me%$anism o( se%ondar! adaptation t$at $as a
role in t$e ongoing damage in %$roni% -idne! disease. Ene o( t$e eviden%e a*out t$e me%$anism
is t$e presen%e o( renal $istologi% (eature o( C#& t$at %aused *! an! primar! disease. C$ange
and adaptation o( t$e remaining nep$ron (ollo'ing renal damage in t$e *eginning lead to
(ormation o( %onne%tive tissue and (urt$er damage o( t$e surviving nep$rons. )$is %ondition 'ill
%ontinue until t$e end stage o( renal (ailure.
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0igure 1. C!%le o( t$e %$roni% -idne! disease
Iassner S?" =aum > (1999). C$roni% Renal 0ailure. P$!siolog! and >anagement. 5n: =arrat
)>" Avner ,&" @armon I," ,ditors. Pediatri% ep$rolog!" 2
t$
edition. =altimore: Aippin%ott
Iilliams J Iil-ins" pp. 114481172.
@!per(iltration inHur! ma! *e an important (inal %ommonpat$'a! o( glomerular
destru%tion" independent o( t$e underl!ing %ause o( renal inHur!. As nep$rons are lost" t$e
remaining nep$rons undergo stru%tural and (un%tional $!pertrop$! %$ara%teri3ed *! an in%rease
in glomerular *lood (lo'. )$e driving (or%e (or glomerular (iltration is t$ere*! in%reased in t$e
surviving nep$rons. Alt$oug$ t$is %ompensator! $!per(iltration temporaril! preserves total renal
(un%tion" it %an %ause progressive damage to t$e surviving glomeruli" possi*l! *! a dire%t e((e%t
o( t$e elevated $!drostati% pressure on t$e integrit! o( t$e %apillar! 'all andBor t$e to6i% e((e%t o(
in%reased protein tra((i% a%ross t$e %apillar! 'all. Ever time" as t$e population o( s%lerosed
nep$rons in%reases" t$e surviving nep$rons su((er an in%reased e6%retor! *urden" resulting in a
vi%ious %!%le o( in%reasing glomerular *lood (lo' and $!per(iltration inHur!.
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Proteinuria itsel( %an %ontri*ute to renal (un%tional de%line" as eviden%ed *! studies t$at
$ave s$o'n a *ene(i%ial e((e%t o( redu%tion in proteinuria. Proteins t$at traverse t$e glomerular
%apillar! 'all %an e6ert a dire%t to6i% e((e%t on tu*ular %ells and re%ruit mono%!tes and
ma%rop$ages" en$an%ing t$e pro%ess o( glomerular s%lerosis and tu*ulointerstitial (i*rosis.
Kn%ontrolled %an $!pertension e6a%er*ate disease progression *! %ausing arteriolar
nep$ros%lerosis and *! in%reasing t$e $!per(iltration inHur!. @!perp$osp$atemia %an in%rease
progression o( disease *! leading to %al%ium p$osp$ate deposition in t$e renal interstitium and
*lood vessels. @!perlipidemia" a %ommon %ondition in C#& patients" %an adversel! a((e%t
glomerular (un%tion t$roug$ o6idant8mediated inHur!.
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CLINICAL MANIFESTATION
,arl! %lini%al mani(estation o( C#& doesnLt s$o' spe%i(i% s!mptoms su%$ as di33iness"
letargis" anore6ia" nausea.
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C#& patient (rom %$roni% glomerulonep$ritis %an present 'it$
edema" $!pertension" $ematuria" and proteinuria. 5n(ants and %$ildren 'it$ %ongenital disorders
su%$ as renal d!splasia and o*stru%tive uropat$! %an present in t$e neonatal period 'it$ (ailure to
t$rive" pol!uria de$!dration" urinar! tra%t in(e%tion" or overt renal insu((i%ien%!. Congenital
-idne! disease is diagnosed 'it$ prenatal ultrasonograp$! in man! in(ants" allo'ing earl!
diagnosti% and t$erapeuti% intervention. C$ildren 'it$ (amilial Huvenile nep$ronop$t$isis %an
$ave a ver! su*tle presentation 'it$ nonspe%i(i% %omplaints su%$ as $eada%$e" (atigue" let$arg!"
anore6ia" vomiting" pol!dipsia" pol!uria" and gro't$ (ailure over a num*er o( !ears.
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5n p$!si%al e6amination 'e (ound a pallor" limp" and $!pertension. )$e %ondition last (or
along time and more da! more severe. Kremia is a to6i% s!ndrome %aused *! severe glomerular
damage" tu*ular d!s(un%tion" and renal endo%rine (un%tion.
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Clini%al mani(estation is t$e result o( :
1. 0luid and ele%trol!te im*alan%e
2. overa%umulation o( uremi% to6in.
+. @ormonal d!s(un%tion (de%rease o( er!tropoeitin and vitamin &+)
2. unresponse o( end organ 'it$ gro't$ $ormone.
LA+ORATORY FINDING
Aa*orator! (indings %ould *e (ound :
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1. %omplete *lood %ount normo%$romi%" normo%!ti% anemia.
2. Renal 0un%tion )est serum ureum and %reatinine.
+. ,le%trolite $!per-alemia" $!ponatremia (i( volume overloaded)" a%idosis"
$!po%al%emia" $!perp$osp$atemia.
2. Krinalisis proteinuria and $ematuria i( %aused *! glomerulonep$ritis in $eav!
proteinuria %ould *e (ound $!poal*uminemia.
/lomerular (iltration rate (/0R) used (or staging o( C#&. 5n %$ildren" t$e (ormula to
estimate patientLs /0R are s$o'n *elo' :
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I$ere k is :
1. 0.++ (or lo'8*ir$t'ei$t in(ant D1 !ear old
2. 0.24 (or term in(ant D1 !ear old '$ose 'eig$t is appropriate (or gestational age
+. 0.44 (or %$ildren (181+ !ears old)
2. 0.47 (or adoles%ent girls (1+821 !ears old)
4. 0.70 (or adoles%ent *o!s (1+821 !ears old)
TREATMENT
Patients 'it$ C#& s$ould *e evaluated to determine:
1. &iagnosis (t!pe o( -idne! disease)
2. Comor*id %onditions (su%$ as $!perlipidemia)
+. Severit!" assessed *! level o( -idne! (un%tion
2. Compli%ations" related to level o( -idne! (un%tion
4. Ris- (or loss o( -idne! (un%tion
9. Ris- (or %ardiovas%ular disease
)reatment o( C#& s$ould in%lude:
1. Spe%i(i% t$erap!" *ased on diagnosis
2. ,valuation and management o( %omor*id %onditions
+. Slo'ing t$e loss o( -idne! (un%tion
2. Prevention and treatment o( %ardiovas%ular disease
4. Prevention and treatment o( %ompli%ations o( de%reased -idne! (un%tion (eg"
$!pertension" anemia" a%idosis" gro't$ (ailure)
9. Preparation (or -idne! (ailure t$erap!
7. Repla%ement o( -idne! (un%tion *! dial!sis and transplantation"
i( signs and s!mptoms o( uremia are present" a %lini%al a%tion plan s$ould *e developed
(or ea%$.
1. N,t&iti(n
C$ildren '$o $ave C#& $ave nutrition and protein de(i%ien%ies (or several reasons"
in%luding anore6ia" nausea and vomiting (rom t$e uremia" and an a*normal sense o( taste. Young
%$ildren" in parti%ular" need su((i%ient %alori% inta-e to gro'. Protein inta-e s$ould *e optimi3ed
to allo' (or maintenan%e o( nitrogen *alan%e and preservation o( lean *od! mass. Some patients
ma! re:uire supplemental nasogastri% or gastrotom! tu*e(eeding i( t$e! %annot maintain optimal
$eig$t and 'eig$t gain *! oral (eeding. @o'ever" i( protein inta-e ise6%essive" $!per(iltration
ma! o%%ur" leading to in%reased damage to t$e renal paren%$!ma. >i%ropun%ture studies
demonstrate an in%rease in t$e /0R ($!per(iltration) a(ter an amino a%id load due to redu%tion in
a((erent arteriolar resistan%e.
Prostaglandins" '$i%$ %an alter vas%ular tone and in%rease t$e /0R" re%entl! $ave *een
impli%ated in t$e development o( $!per(iltration *e%ause prostaglandin values $ave *een noted to
in%rease in response to an in%reased amino a%id load. Protein restri%tion 'as *elieved to slo' t$e
progression o( renal disease" *ut t$is e((e%t $as not *een veri(ied in %$ildren. Redu%ing protein
inta-e to 0.7 to 1.1 gB-g per da! $as not *een s$o'n to a((e%t linear gro't$ negativel!. =e%ause
man! vitamins are lost during dial!sis" pediatri% patients undergoing t$is t$erap! s$ould
supplement t$eir diets 'it$ vitamins" espe%iall! (oli% a%id" tra%e minerals" and = %omple6es.
Spe%iali3ed (ormulas t$at $ave $ig$ energ! %ontents and lo'er ele%trol!te %ontents $ave
*een developed (or in(ants and %$ildren '$o $ave C#&. )$ese are reasona*le (ormulas (or an
older %$ild alread! on dial!sis '$o ma! *e not *e meeting $is or $er nutritional goal or '$o is
e6perien%ing poor 'eig$t gain or gro't$.
". F),i* an* E)e%t&()ite Im-a)an%e
@!per-alemia is a %ompli%ation o( C#&. 5n t$e $ealt$! -idne!" potassium rea*sorption
o%%urs in t$e pro6imal tu*ules and t$e loop o( @enle" and se%retion o( up to 90; o( t$e dail!
inta-e o( potassium o%%urs in t$e distal tu*ules. As renal disease progresses" t$e distal tu*ules o(
t$e remaining nep$rons %ontinue to se%rete potassium. 5n%reased aldosterone also en$an%es
potassium se%retion *! stimulating sodium8potassium e6%$ange in t$e -idne!s and t$e %olon.
@o'ever" $!per-alemia develops (rom an in%rease in dietar! potassium t$at over'$elms t$e
%ompensator! me%$anisms or *! use o( medi%ations t$at alter potassium se%retion
(spironola%tone" amiloride" or angiotensin %onverting en3!me in$i*itors). )a*le 2 lists
approa%$es to treating $!per-alemia. @!po-alemia also %an o%%ur in %$ildren '$o $ave C#& *ut
tends to develop in patients '$o $ave tu*ular de(e%ts su%$ as seen 'it$ 0an%oni s!ndrome.
)a*el 2. )reatment o( $!per-alemia
Produ%t &ose Potential adverse e((e%ts
Sodium *i%ar*onate (.0.9 M ==1 M .*i%ar*onate
desired8*i%ar*onated o*served1) B2
0.481 m,:B-g 5G over 1 $our
@!po%al%emia
Cal%ium glu%onate (10;) 0.481 mAB-g 5G over 4814 min Arr$!t$mia
/lu%ose and insulin /lu%ose 0.4 gB-g 'it$ insulin 0.1
unitB-g 5G over +0 min
@!pogl!%emia
Sodium pol!st!rene
sul(onate
1gB-g per dose PR or PE ConstipationBdiarr$ea
=eta agonist 4810 mg aerosoli3ed )a%$!%ardia" $!pertension
5GN intravenous" PEN orall!" PRNre%tall!
&il!s A. I$!te and Ri%$ard 0ine" 2007. C$roni% #idne! &isease in C$ildren.
!. A%i*.-a$e im-a)an%e
>eta*oli% a%idosis develops in patients '$o $ave C#& *e%ause o( a*normall! de%reased
*i%ar*onate rea*sorption o( (iltered *i%ar*onate" redu%tion o( renal ammonia s!nt$esis" de%reased
a%idi(ied tu*ular (luid" and de%reased titrata*le a%id e6%retion. &e%line in /0R *elo' 40; o(
normal is a%%ompanied *! a de%line in *i%ar*onate rea*sorption. Redu%ed *i%ar*onate
rea*sorption leads to s!stemi% a%idosis" '$i%$ %auses protein degradation and e((lu6 o( %al%ium
(rom *one. Su%$ (a%tors pla! a role in t$e poor linear gro't$ o*served in %$ildren '$o $ave
C#&.
)$erap! s$ould target maintaining a serum *i%ar*onate %on%entration o( 20 to 22 m,:BA
(20 to 22 mmolBA). =i%ar*onate repla%ement %onsists o( administering sodium *i%ar*onate
supplements or p$osp$ate *inders. >ost availa*le *inders $ave a *ase %omponent su%$ as
%al%ium %ar*onate.
/. H0'e&ten$i(n
@!pertension is diagnosed in %$ildren '$o $ave C#& *! (inding an elevated *lood
pressure reading on t$ree or more separate o((i%e visits at least 1 'ee- apart. )$e diagnosis is
*ased on t$e %$ildLs age" se6" and $eig$t per%entile. /rades o( $!pertension are as (ollo's" *ased
on ta*les or grap$s o( normal values:
Pre$!pertension: Average s!stoli% or diastoli% pressuresare at t$e 90t$ per%entile or
greater *ut at or less t$an t$e 94t$ per%entile (or age" se6" and $eig$t
Stage 5 $!pertension: Average s!stoli% or diastoli% pressure is at or greater t$an t$e
94t$ per%entile (or age" se6" and $eig$t
Stage 55 $!pertension: Average s!stoli% or diastoli% pressure is more t$an 4mm@g
$ig$er t$an t$e 94t$ per%entile
@!pertensive urgen%! and emergen%!: Average s!stoli% or diastoli% pressure is more
t$an 4 mm @g $ig$er t$an t$e 94t$ per%entile and %lini%al s!mptoms o( $eada%$e"
vomiting" sei3ures" or en%ep$alopat$! are present
5n addition to determining t$e underl!ing %ause o( t$e $!pertension" %lini%ians s$ould
monitor patients at least annuall! 'it$ e%$o%ardiograp$! to assess (un%tion and le(t ventri%ular
status. >edi%ations are adHusted to improve %ardia% (un%tion. )$ose %$ildren '$o (all into t$e
$!pertensive urgen%! and emergen%! %ategor! re:uire intravenous medi%ations or a rapid8a%ting
oral medi%ation (ni(edipine or mino6idil) to redu%e *lood pressure.
1. Anemia
Anemia in C#& is %aused *! eit$er an insu((i%ient produ%tion o( er!t$ropoietin *! t$e
diseased -idne!s or *! iron de(i%ien%!. Anemia is de(ined as a redu%tion in red *lood %ell volume
or $emoglo*in %on%entration *elo' t$e normal range (or a $ealt$! person. >or*idit!" mortalit!"
and :ualit! o( li(e data (rom t$e #&EF5 guidelines suggest t$at maintaining t$e $emato%rit in
t$e range o( ++; to +9; (0.++ to 0.+9) and t$e $emoglo*in at 11.0 to 12.0 gBdA (110.0 to 120.0
gBA) is important (or %$ildren '$o $ave C#&. Prior to t$e development o( re%om*inant $uman
er!t$ropoietin" patients '$o $ad C#& $ad to undergo trans(usions to in%rease t$eir $emato%rit
values. )rans(usions not onl! e6posed patients to various in(e%tious agents *ut e6posed and
sensiti3ed t$em to $uman l!mp$o%!te antigens" putting t$em at in%reased ris- (or reHe%tion
s$ould t$e! undergo renal transplantation.
Iit$ improvement o( anemia" %$ildren demonstrate improvement in %ognitive
development" %ardia% (un%tion" and e6er%ise toleran%e" as 'ell as de%reased mortalit!. As stated"
anemia is %aused *! eit$er an insu((i%ient produ%tion o( er!t$ropoietin *! t$e diseased -idne!s or
*! iron de(i%ien%!. &ue to de%reased appetites" %$ildren '$o $ave C#& %annot in%rease t$eir
iron stores ade:uatel! t$roug$ an oral diet. Eral iron t$erap! s$ould *e administered at a dose o(
2 to + mgB-g per da! o( elemental iron in t'o or t$ree divided doses. 5ron s$ould *e %onsumed
on an empt! stoma%$ and not %on%omitantl! 'it$ p$osp$ate *inders *e%ause iron *inds to t$e
p$osp$ate *inders.
Parenteral iron %an *e provided to t$ose '$o %ontinue to lose *lood or '$o %annot
tolerate oral iron. Parenteral iron %an *e administered easil! to patients re%eiving $emodial!sis
*e%ause t$e! alread! $ave vas%ular a%%ess. 5ntravenous iron also %an *e used (or t$e peritoneal
dial!sis patient '$o is resistant to oral iron or is non%ompliant in ta-ing oral iron.
,r!t$ropoietin %an *e administered su*%utaneousl! to %$ildren '$o $ave C#&" in%luding
t$ose undergoing peritoneal dial!sis" or intravenousl! (or t$ose re%eiving $emodial!sis.
,r!t$ropoietin %an *e given one" t'o" or t$ree times per 'ee-. )$e initial dose ranges *et'een
+0 and +00 unitsB-g per 'ee-" 'it$ t$e usual maintenan%e dosage *et'een 90 and 900 unitsB-g
per 'ee-. )$e maintenan%e dose is determined and adHusted *ased on mont$l! $emoglo*in
values. A ne' (orm o( er!t$ropoietin" dar*epoetin al(a" '$i%$ $as a longer $al(8li(e and re:uires
dosing on%e ever! 2 'ee-s to on%e mont$l!" is *eing investigated (or use in %$ildren.
2. G&(3t4
/ro't$ retardation is one o( t$e maHor %ompli%ations o( a %$ild '$o $as C#&. )$e
degree o( gro't$ (ailure $as *een %orrelated 'it$ age o( onset o( C#&.
)$e %ause o( gro't$ (ailure is *elieved to *e multi(a%torial" in%luding :
gro't$ $ormone (/@) and insulin8li-e gro't$ (a%tor85 (5/085) (un%tion
nutritional status
a%id8*ase *alan%e
*one minerali3ation.
I$en renal (un%tion is redu%ed" /@ is in%reased *e%ause o( de%reased %learan%e *! t$e
-idne!s despite t$e normal pulsatile release o( t$e $ormone" '$i%$ %ontinues despite t$e
in%reased /@ %on%entrations. Resistan%e to /@ and to 5/085 also is *elieved to lead to gro't$
redu%tion. 5n some studies" serum /@ %on%entrations are in%reased in patients '$o $ave C#&"
*ut t$e %on%entrations o( /@ re%eptors are redu%ed. Anot$er %ause ma! *e t$e upregulation o(
intra%ellular in$i*itors" la*eled suppressors o( %!to-ine signaling (SECS). )$e SECS proteins
%an alter t$e p$osp$or!lation o( /@ re%eptors and ma! %ause /@ resistan%e.
Similarl!" 5/081 resistan%e pro*a*l! is due to several %auses. 5/08*inding proteins" o(
'$i%$ si6 are no' identi(ied" in%rease 'it$ renal (ailure and most li-el! in$i*it t$e a%tions o(
5/081 *! *inding 'it$ it" t$ere*! preventing 5/081 (rom *inding to its re%eptor. Et$er $ormones
t$at pla! a role in pu*ertal gro't$ and development $ave *een (ound to *e redu%ed in %$ildren
'$o $ave C#&" in%luding luteini3ing $ormone" plasma testosterone" and (ree testosterone.
)reatment o( gro't$ (ailure initiall! involves resolving nutritional de(i%ien%ies and
improving t$e a%id8*ase *alan%e o( %$ildren '$o $ave C#&. En%e t$ese tas-s are a%%omplis$ed"
a((e%ted %$ildren *egin /@ t$erap! i( gro't$ retardation persists. >ost patients '$o $ave C#&
gro' '$en given t$e re%ommended starting dose o( 0.04 mgB-g per da!" administered
su*%utaneousl! dail!. I$et$er patients in t$e pu*ertal age group re:uire additional /@ needs
additional investigation.
PROGNOSIS
A*out 70; o( %$ildren 'it$ %$roni% -idne! disease develop ,SR& *! age 20 !ears.
C$ildren 'it$ ,SR& $ave a 108!ear survival rate o( a*out 70; and an age8spe%i(i% mortalit! rate
o( a*out +0 times t$at seen in %$ildren 'it$out ,SR&. )$e most %ommon %ause o( deat$ in t$ese
%$ildren is %ardiovas%ular disease" (ollo'ed *! in(e%tion. E( t$e deat$s due to %ardiovas%ular
%auses" 24; 'ere attri*uted to %ardia% arrest (%ause un%ertain)" 19; to stro-e" 12; to
m!o%ardial is%$emia" 12; to pulmonar! edema" 11; to $!per-alemia" and 22; to ot$er
%ardiovas%ular %auses" in%luding arr$!t$mia. =ut %urrentl!" survival rate o( %$ildren 'it$ C#& is
more *etter. &ata (rom t$e Australia and e' Oealand (AO) registr! revealed t$at t$e ris- o(
deat$ 'as asso%iated 'it$ t$e !ear in '$i%$ renal repla%ement t$erap! 'as initiated" t$e age o(
patients at t$e start o( t$at t$erap!" and t$e t!pe o( dial!sis used.
11
CASE REPORT
ame : 0.A.O
Age : 14 )a$un
Se6 : >ale
&ate o( Admission : August" 12
t$
2012

C4ie5 C(m')aint : Aoss o( %ons%iousness
Hi$t(&0:
)$is $as *een $appened *! t$e patient P 2 da!s ago. Patient didnQt respond '$en (amil! as-ing
(or %onversation. Patient *e more :uite even t$e patient spo-e" t$at 'as in%o$erent. Patient is a
oldterm patient in nep$rolog! division pediaetri% departemen. Aast mont$" t$e patient 'as
re%ommended (or $ospitali3ation" *ut t$e patient re(used.
@istor! o( vomit su((ered *! patient P + da!" (re:uen%! (ive times per da!" volume P R spoon.
)$e %ontent is '$at patient eat and drin-. o'" t$e vomit 'as denied.
&iare 'asnQt (ound. last de(e%ation t$ree da!s ago. @istor! o( pale (ae%es 'as denied. Aast
urination 2 da!s ago. @istor! o( urine li-e a tea 'as denied.
0ever 'asnQt (ound" $istor! o( (ever also denied
@istor! o( sei3ure 'as (ound (ive mont$s ago" (re:uen%! on%e a da!. As soon as t$e patient
$ospitali3ed at RSKP @A>.
@istor! o( 'eig$t loss 'as (ound" $istor! o( appetite loss 'as (ound
Hi$t(&0 (5 '&e6i(,$ i))ne$$ : 8
Hi$t(&0 (5 *&,g$ 7 Spironola-ton" 0urosemide
P40$i%a) E8aminati(n
Generalized status
=od! 'eig$t: 20 -g" =od! lengt$: 194 %m
=od! 'eig$t a%%ording to age : 20B49 N 71;
=od! $eig$t a%%ording to age : 194B170 N97;
=od! 'eig$t a%%ording to *od! $eig$t : 20B41 N 77;
5nterpretation : Kndernutrition
Presens status
Cons%iousness: /CS : 1+ (,2G2>4)" =lood pressure 120B100 mm@g" @R: 97 *pm" RR: 22 *pm"
*od! temperature: +9"7
o
C" *od! 'eig$t : 20 -g" *od! $eig$t : 194 %m
Anemi% (P)< 5%teri% (8)< C!anosis (8)< ,dema (8). &!spnea (8).
Localized status
Head :
,!e: iso%$ori% pupil (+mmB+mm)" lig$t re(le6 (PBP) " %onHun%tiva palpe*ral in(erior pale (PBP)"
i%teri% s%lera (8).
ose: nasal septum : no deviation" mu%osa %olor dar- pin-" pol!ps (8)" sinus tenderness (8)"
dis%$arge (8).
,ar : ,6ternal auditor! %anal: no dis%$arge" t!mpani% mem*rant: inta%t" no in(lammation"
>out$: lips : %olor is red" (issures< =u%al : dar- pin-" glistening< tounge : dar- pin-" papilla
at$rop! (8)" tremor (8)
)eet$ and gums : t'ent! teet$" %aries (8).
)onsil : di((i%ult to assess
Neck :
A!mp$ node enlargement (8)" e%- rigidit! (8)
Thora:
5nspe%tion : S!mmetri%al (usi(ormis" epigastrial retra%tion (8)" RR: 20 6Bi" reguler
Palpation : S0 rig$t N le(t" normal
Per%ussion : Sonor
Aus%ultation : rales (8B8) " stridor (8B8).
!"do#en:
5nspe%tion : S!mmetri%al (usi(ormis
Palpation : Soepel" Aiver and spleen unpalpa*le
Per%ussion : )!mpani
Aus%ultation : ormoperistalti%
Etre#ities:
,6tremitas superior : Pulse 97 *pm" regular" ade:uate pressure and volume" 'arm"
CR) D +S" spasti% (8B8).
,6tremitas in(erior : Pulse 97 *pm" regular" ade:uate pressure per volume" 'arm"
CR) D +S" spasti% (8B8).
$ro%enital:
>ale" 'it$in normal limit.
La-(&at(&0 Fin*ing$ (A,g,$t 1"
t4
"91/# 7
Parameters Galue ormal Galue
Co#&lete 'lood Count
Hem(g)(-in ."9 g&: 1!." ; 1.! g&:
Hemat(%&ite 1./9 : /! ; /< :
E&it4&(%0te ".! 8 19
2
/mm
!
/."9 ; /.= 8 19
2
/mm
!
Aeu%o%!te 11.+7 6 10
+
Bmm
+
2.4 T 11 6 10
+
Bmm
+
Platelet 220.000 Bmm
+
140 8 240Bmm
+
MC> !./9 5) =1 ; <1 5)
>C@ +0.20 pg 27 8 +2 pg
>C@C 21.20 gr; ++ 8 +4 gr;
R&I 1+.20 ; 11.9 T 12.7 ;
>PG 7.00 (l 7.0 T 10.2 (l
PC) 0.+2;
P&I 7.7;
@itung ?enis
eutro(il
79.90;
+7870
Aim(osit
9.70;
20820
>onosit
+.+0;
287
,osino(il
0.10;
189
=aso(il
0.000;
081
eutro(il a*solute
9.77
1"984"2
Aim(osit a*solute
1.10
+"7810"7
>onosit a*solute
0.+7
0"+80"7
,osino(il a*solute
0.01
0"280"4
=aso(il a*solute
0.00
080"1
Parameters Galue ormal Galue
Ana)i$a Ga$ Da&a4
p@ 7.+1 7.+4 T 7.24
PCE2 27.9 mm@g +7 T 22
PE2 141.7 mm@g 74 T 100
=i-ar*onat 29.4 mmolBA 22 T 29
)otal CE2 14.1 mmolBA 19 T 24
#ele*i$an =asa (=,) 84.0 mmoBA (82) T (P2)
Saturasi E2 99.+; 94 T 100
Car"ohydrate (eta"olis#
=lood /lu%ose ad random 129.00 mgBdA D 200
)enal *unction Test
U&e,m 1=.99 mg/*L ? 19
C&eatinine /.< mg/*L 9@1 ; 9@/"
Elektrolit
Ka)$i,m =.< mg/*L <." ; 11.9 mg/*L
Nat&i,m <= mEA/L 1!1 ; 111 meA/L
Ka)i,m 1. mEA/L !@2 ; 1@1 mEA/L
K)(&i*a 12 mEA/L <2 ; 192 mEA/L
Ra*i()(gi% imaging B,ni "=
t4
"91/
0igure 2. )$e %$est 68ra! o( t$e patient
C4e$t 8.&a0 inte&'&etati(nC
#G is too $ig$" good inspiration" middle tra%$ea" *ot$ %ostop$reni%us angels is s$arp" smoot$
diap$ragm" Cardio )$ora%i% Ratio is more t$an 40;" *ones and so(t tissues in normal %ondition"
Result : Cardiomegal!
D(&Eing Diagn($i$7
C$roni% #idne! disease Stage 5G
Management7@
E6!gen 1 T 2 ABi nasal %anule (P)" nasogastri% tu*e (8)
5G0& &4; 4 gttBi
Spironola-ton 2 6 24 mg
&iet 1994 --al P +2 g protein
Diagn($ti% P)anning7
C$est M8ra!
Complete *lood %ount
/lu%ose ad random
,le%trol!te (a" #" Cl)
=lood gas anal!sis
Krin anal!sis
ep$rolog! %onsult
KS/ &oppler E( #idne! and =ladder
F())(3 U' Patient$
A,g,$t@ 1" t4

"91/
S Aoss o( %ons%iousness
O Sens: /CS 12 " )emp: +9.7
o
C. Anemi% (P). 5%teri% (8). ,dema (8). C!anosis (8) &!spnoe
(8)
=od! 'eig$t: 20 -g" =od! lengt$: 194 %m
@ead Rig$t ,!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P).
Ae(t e!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P)..
e%- A!mp$ node enlargement (8)
)$ora6 Simetris (usi(ormis. Retra%tion (8) epigastrial< inter%ostals" suprasternal.
@R: 97 *pm" reguler< murmur (8)
RR: 27 6Bi" regular" rales (8)
A*domen Soepel" Rapid turgor. normoperistalti%. Aiver" spleen and renal
unpalpa*le.
,6tremities Pulse 97 6Bi" regular" ade:uate pBv" 'arm" CR) D +U. =lood Pressure
120B100 mm$g
/enital >ale< 'it$in normal limit.
A C$roni% #idne! &isease Stage 5G
P Management7
E2 182 ABi
5G0& &4; 4 gttB5 mi-ro
Spironola-ton 2624 mg
&iet 1994 --al P +2 g protein
@iponatremia %orre%tion
0ast Corre%tion N (120897) 6 20 6 0.9 N 401.9 m,: ((inis$ed in 9 $ours. Iit$ aCl
+; N 401.9B41+ N977%% N 190 gttB5 mi%ro
Slo' %orre%tion N (1+48124) 6 20 6 0.9 N 220 m,:BA
>aintanan%e N 2 82 m,:BA N 70 T 190 m,:BA
)otal N +20 T 200 m,:
Iit$ 5G0& &4; aCl 0.9; N +20B142 6 1000 N 2077 %% 144 gttB5 mi%ro"
(inis$ed in 17 $ours
@ipo%alemia %orre%tion
1.7 m,:BA 'it$ 0.74 m,:B-g==" +0 m,: #Cl in 90 %% &4;" (inis$ed in + $ours N
20 gttB5 mi%ro
Planning :
,le%trolit test repetition post %orre%tion" (ull *lood" and A/&
&ipsti%- result
KroB=ilB#etB=loodBProBitBAeuB/luBSgBp@
8BPB8B8BPB8BPB8B1.004B9.4
A,g,$t 1!
t4
"91/
S limp (P)" nausea and vomit (8)" 0ever (8)
O Alert : Compos >entis " )emp: +9"7
o
C. Anemi% (P). 5%teri% (8). ,dema (8). C!anosis (8)
&!spnoe (8)
=od! 'eig$t: 20 -g" =od! lengt$: 194 %m
@ead Rig$t ,!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P).
Ae(t e!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P)..
e%- A!mp$ node enlargement (8)
)$ora6 Simetris (usi(ormis. Retra%tion (8) epigastrial< inter%ostals" suprasternal.
@R: 77 *pm" reguler< murmur (8)
RR: 29 6Bi" regular" rales (8)
A*domen Soupel" Rapid turgor. normoperistalti%. Aiver" spleen and renal
unpalpa*le.
,6tremities Pulse 77 6Bi" regular" ade:uate pBv" 'arm" CR) D +U. =lood Pressure
120B100 mm$g
/enital >ale< 'it$in normal limit.
A C$roni% #idne! &isease Stage 5G
P E2 182 AB5
5G0& &4; 4 gttB5 mi-ro
Spironola-ton 2624 mg
&iet 1994 --al P protein +2 gram
Planning :
,le%trolit and A/&A repetition test ( i( %orre%tion o( $ipo%alemia and $iponatremia
(inis$ed).
Iater =alan%e
5nput :&iet N 1.400 %% Eutput : EKP N 900 %% = N 5 8 E
5G0& N +0 %% =A= N 0 N 220 %%
)otal N 1.4+0 %% 5IA N 190 %% #9?= N R @S P =C
)otal N 1.090 %% N +4 %%
A,g,$t@ 1/
t4
"91/
S limp (P)" nausea and vomit (8)"0ever (8)
E Alert : Compos >entis " )emp: +9"7
o
C. Anemi% (P). 5%teri% (8). ,dema (8). C!anosis (8)
&!spnoe (8)
=od! 'eig$t: 20 -g" =od! lengt$: 194 %m
@ead Rig$t ,!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P).
Ae(t e!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P)..
e%- A!mp$ node enlargement (8)
)$ora6 Simetris (usi(ormis. Retra%tion (8) epigastrial< inter%ostals" suprasternal.
@R: 97 *pm" reguler< murmur (8)
RR: 22 6Bi" regular" rales (8)
A*domen Soupel" Rapid turgor. normoperistalti%. Aiver" spleen and renal
unpalpa*le.
,6tremities Pulse 97 6Bi" regular" ade:uate pBv" 'arm" CR) D +U. =lood Pressure
1+0B90 mm$g
/enital >ale< 'it$in normal limit.
A C$roni% #idne! &isease Stage 5G
P Management7
E2 V 8 1 AB5 nasal -anul
5G0& &4; 4 gttB5 mi%ro
Spironola-ton 2624 mg
&iet 1994 --al P +2 g protein
Iater =alan%e
5nput : &iet N 900 %% Eutput : EKP N 900 %% = N 5 8 E
5G0& N 140 %% =A= N 0 N 820%%
)otal N 740 %% 5IA N 190 %% #9?= N R @S P =C
)otal N 790 %% N 274 P 20 N 414 %%
A,g,$t@ 11
t4
"91/
S limp (P)" nausea and vomit (8)"0ever (8)
O Alert : Compos >entis " )emp: +7
o
C. Anemi% (P). 5%teri% (8). ,dema (8). C!anosis (8)
&!spnoe (8)
=od! 'eig$t: 20 -g" =od! lengt$: 194 %m
@ead Rig$t ,!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P).
Ae(t e!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P)..
e%- A!mp$ node enlargement (8)
)$ora6 Simetris (usi(ormis. Retra%tion (8) epigastrial< inter%ostals" suprasternal.
@R: 110 *pm" reguler< murmur (8)
RR: 29 6Bi" regular" rales (8)
A*domen Soupel" Rapid turgor. normoperistalti%. Aiver" spleen and renal
unpalpa*le.
,6tremities Pulse 97 6Bi" regular" ade:uate pBv" 'arm" CR) D +U. =lood Pressure
1+0B90 mm$g
/enital >ale< 'it$in normal limit.
A C$roni% #idne! &isease Stage 5G
P >anagement
E2 V 8 1 AB5 nasal -anul
5G0& &4; 4 gttB5 mi%ro
Spironola-ton 2624 mg
&iet pediasure mil- P &iet 1994 --al P +2 g protein
Planning
KS/ &oppler o( -idne! and *ladder
,le%trolit %orre%tion result
CaBaB#BCl N 7.7B121B1.7B9+ m,:BA
0ull *lood %ount result
@*B@tBR=CBI=CBPlt N 9.7B17.9B2.+1610
9
B9.4610
+
B2+1610
+
Iater =alan%e
5nput : &iet N 900 %% Eutput : EKP N 900 %% = N 5 8 E
5G0& N 140 %% =A= N 0 N 820%%
)otal N 740 %% 5IA N 190 %% #9?= N R @S P =C
)otal N 790 %% N ++4 %%
A,g,$t@ 12
t4
"91/
S limp (P)" nausea and vomit (8)"0ever (8)
O Alert : Compos >entis " )emp: +7
o
C. Anemi% (P). 5%teri% (8). ,dema (8). C!anosis (8)
&!spnoe (8)
=od! 'eig$t: 20 -g" =od! lengt$: 194 %m
@ead Rig$t ,!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P).
Ae(t e!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P)..
e%- A!mp$ node enlargement (8)
)$ora6 Simetris (usi(ormis. Retra%tion (8) epigastrial< inter%ostals" suprasternal.
@R: 110 *pm" reguler< murmur (8)
RR: 29 6Bi" regular" rales (8)
A*domen Soupel" Rapid turgor. normoperistalti%. Aiver" spleen and renal
unpalpa*le.
,6tremities Pulse 110 6Bi" regular" ade:uate pBv" 'arm" CR) D +U. =lood Pressure
1+0B90 mm$g
/enital >ale< 'it$in normal limit.
A C$roni% #idne! &isease Stage 5G
P >anagement
E2 V 8 1 AB5 nasal -anul
Spironola-ton 2624 mg
&iet 1994 --al P +2 g protein
Planning
KS/ &oppler o( -idne! and *ladder
)ran(ussion PRC + %%B-g** in 22 $ours
Iater =alan%e
5nput : &iet N 200 %% Eutput : EKP N +00 %% = N 5 8 E
5G0& N 0 %% =A= N 0 N 200 T 290 %% N 890%%
)otal N 200 %% 5IA N 190 %% #9?= N R @S P =C
)otal N 290 %% N 274 P 90 N 494 %%
A,g,$t@ 1
t4
"91/
S limp (P)" nausea and vomit (8)"0ever (8)
O Alert : Compos >entis " )emp: +7
o
C. Anemi% (P). 5%teri% (8). ,dema (8). C!anosis (8)
&!spnoe (8)
=od! 'eig$t: 20 -g" =od! lengt$: 194 %m
@ead Rig$t ,!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P).
Ae(t e!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P)..
e%- A!mp$ node enlargement (8)
)$ora6 Simetris (usi(ormis. Retra%tion (8) epigastrial< inter%ostals" suprasternal.
@R: 110 *pm" reguler< murmur (8)
RR: 20 6Bi" regular" rales (8)
A*domen Soupel" Rapid turgor. normoperistalti%. Aiver" spleen and renal
unpalpa*le.
,6tremities Pulse 110 6Bi" regular" ade:uate pBv" 'arm" CR) D +U. =lood Pressure
120B70 mm$g
/enital >ale< 'it$in normal limit.
A C$roni% #idne! &isease Stage 5G
P >anagement
Spironola-ton 2624 mg
&iet 1994 --al P +2 g protein
Planning
KS/ &oppler o( -idne! and *ladder
)ran(ussion PRC + %%B-g** in 22 $ours
Iater =alan%e
5nput : &iet N 2+0 %% Eutput : EKP N +00 %%
5G0& N 0 %% =A= N 0
)otal N 2+0 %% 5IA N 190 %%
)otal N 290 %%
= N 5 8 E
N 2+08290 %% N 890 %%
#9?= N R @S P =C
N 274 P 90 %% N 4+4 %%
A,g,$t@ 1=
t4
"91/
S limp (P)" nausea and vomit (8)"0ever (8)
O Alert : Compos >entis " )emp: +7
o
C. Anemi% (P). 5%teri% (8). ,dema (8). C!anosis (8)
&!spnoe (8)
=od! 'eig$t: 20 -g" =od! lengt$: 194 %m
@ead Rig$t ,!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P).
Ae(t e!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P)..
e%- A!mp$ node enlargement (8)
)$ora6 Simetris (usi(ormis. Retra%tion (8) epigastrial< inter%ostals" suprasternal.
@R: 110 *pm" reguler< murmur (8)
RR: 29 6Bi" regular" rales (8)
A*domen Soupel" Rapid turgor. normoperistalti%. Aiver" spleen and renal
unpalpa*le.
,6tremities Pulse 110 6Bi" regular" ade:uate pBv" 'arm" CR) D +U. =lood Pressure
120B70 mm$g
/enital >ale< 'it$in normal limit.
A C$roni% #idne! &isease Stage 5G
P >anagement
5G0& &4; 4 gttB5 mi-ro
Spironola-ton 2624 mg
&iet 1994 --al P +2 g protein
Planning
KS/ &oppler o( -idne! and *ladder
)ran(ussion PRC + %%B-g** in 22 $ours
Repeat ele%trolit and A/&A
Iater =alan%e
5nput : &iet N 900 %% Eutput : EKP N 70 %% = N 5 8 E
5G0& N +00 %% =A= N 200 %% N 200 %%
)otal N 1200 %% 5IA N 100 %% #9?= N R @S P =C
)otal N 1.000 %% N 274 8 200 %%
F 274 %%
&ipsti-
KroB=ilB#etB=loBProBitBAeuB/luBS/Bp@
8BP1B8B8BP2BP+B8B8B1.010B7.0
A,g,$t@ 1<
t4
"91/
S limp (P)" nausea and vomit (8)"0ever (8)
O Alert : Compos >entis " )emp: +9"7
o
C. Anemi% (P). 5%teri% (8). ,dema (8). C!anosis (8)
&!spnoe (8)
=od! 'eig$t: 20 -g" =od! lengt$: 194 %m
@ead Rig$t ,!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P).
Ae(t e!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P)..
e%- A!mp$ node enlargement (8)
)$ora6 Simetris (usi(ormis. Retra%tion (8) epigastrial< inter%ostals" suprasternal.
@R: 120 *pm" reguler< murmur (8)
RR: 20 6Bi" regular" rales (8)
A*domen Soupel" Rapid turgor. normoperistalti%. Aiver" spleen and renal
unpalpa*le.
,6tremities Pulse 120 6Bi" regular" ade:uate pBv" 'arm" CR) D +U. =lood Pressure
120B70 mm$g
/enital >ale< 'it$in normal limit.
A C$roni% #idne! &isease Stage 5G
P >anagement
5G0& &4; 4 gttB5
Spironola-ton 2624 mg
&iet 1900 --al P +2 g protein
Planning
)ran(ussion PRC + %%B-g** in 22 $ours
Iater =alan%e
5nput : &iet N +00 %% Eutput : EKP N 200 %% = N 5 8 E
5G0& N 40 %% =A= N 0 N +408200 %% N 840 %%
)otal N +40 %% 5IA N 200 %% #9?= N R @S P =C
)otal N 200 %% N 274 P 40 N 424 %%
A,g,$t@ "9
t4
"91/
S limp (P)" nausea and vomit (8)"0ever (8)
O Alert : Compos >entis " )emp: +7.4"7
o
C. Anemi% (P). 5%teri% (8). ,dema (8). C!anosis (8)
&!spnoe (8)
=od! 'eig$t: 20 -g" =od! lengt$: 194 %m
@ead Rig$t ,!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P).
Ae(t e!e: Pupil diameter + mm. 5n(erior palpe*ra %onHun%tiva pale (P).
5%teri% s%lera (8). Aig$t re(le6 (P)..
e%- A!mp$ node enlargement (8)
)$ora6 Simetris (usi(ormis. Retra%tion (8) epigastrial< inter%ostals" suprasternal.
@R: 100 *pm" reguler< murmur (8)
RR: 20 6Bi" regular" rales (8)
A*domen Soupel" Rapid turgor. normoperistalti%. Aiver" spleen and renal
unpalpa*le.
,6tremities Pulse 100 6Bi" regular" ade:uate pBv" 'arm" CR) D +U. =lood Pressure
120B70 mm$g
/enital >ale< 'it$in normal limit.
A C$roni% #idne! &isease Stage 5G
P >anagement
5G0& &4; 4 gttB5
Spironola-ton 2624 mg
&iet 1900 --al P +2 g protein
Iater =alan%e
5nput : &iet N 900 %% Eutput : EKP N 70 %% = N 5 8 E
5G0& N +00 %% =A= N 200 %% N 8200 %%
)otal N 1200 %% 5IA N 100 %% #9?= N R @S P =C
)otal N 1.000 %% N 274 P 200 %%
F 274 %%
&ipsti-
KroB=ilB#etB=loBProBitBAeuB/luBS/Bp@
8BPB8B8BPB8BPB8B1.004B9.4
Di$%,$$i(n
C$roni% -idne! disease" is a slo' progressive de%line o( -idne! (un%tion. 5tQs usuall! a
result o( a %ompli%ation (rom anot$er serious medi%al %ondition. Knli-e a%ute renal (ailure"
'$i%$ $appens :ui%-l! and suddenl!" %$roni% -idne! disease $appens graduall! T over period o(
'ee-s" mont$s" or !ears T as t$e -idne! slo'l! stop 'or-ing" leading to end8stage renal disease
(,SR&).
)$e -idne!s pla! t$ree ma!or roles :
Removing 'aste produ%t (rom t$e *od!" -eeping to6in (rom *uilding up in t$e
*loodstream
Produ%ing $ormones t$at %ontrol ot$er *od! (un%tions" su%$ as regulating *lood
pressure produ%ting red *lood %ells
Regulating t$e levels o( t$e minerals or ele%trol!tes (e.g." sodium" %al%ium" and
potassium) and (luid in t$e *od!
)$e most %ommon %auses o( %$roni% -idne! disease in ort$ Ameri%a are dia*etes
mellitus (t!pe 1 or t!pe 2 dia*etes ) and $ig$ *lood pressure. )$e most %ommon %ause o( end
stage renal (ailure 'orld'ide is 5gA nep$ropat$! (an in(lammator! disease o( -idne!s).
Et$er %ommon %auses o( %$roni% renal (ailure in%lude :
Re%urring p!elonep$ritis (-idne! in(e%tion)
Pol!%!sti% -idne! disease (multiple %!sts in t$e -idne!s)
Autoimmune disorders su%$ as s!stemi% lupus er!t$emastosus
@ardening o( t$e arteries" '$i%$ %an damage *lood vessels in t$e -idne!s
Krinar! tra%t *lo%-ages and re(lu6" due to (re:uent in(e%tions" stones" or an
anatomi%al a*normalit! t$at $appened at *irt$
,6%essive use o( medi%ations t$at are meta*oli3ed t$roug$ t$e -idne!
C$roni% -idne! disease %an *e present (or man! !ears *e(ore !ou noti%e an! s!mptoms.
Patients 'it$ %$roni% -idne! disease (C#&) stage 18+ (glomerular (i-tration rate C+0
mABminB1.7+ m
2)
are (re:uentl! as!mptomati% < in termso( possi*le WnegativeU s!mptoms related
simpl! to t$e loss o( glomerular (iltration rate (/0R)" t$e! do not e6perien%e %lini%all! evident
distur*an%es in 'ater or ele%trol!te *alan%e or endo%rineBmeta*oli% derangements.
/enerall!" t$ese distur*an%es *e%ome %lini%all! mani(est 'it$ C#& stages 284 (/0R D +0
mABminB1.7+ m
2
). Patients 'it$ tu*ulointerstitial disease" %!sti% diseases" nep$roti% s!ndrome"
and ot$er %onditions asso%iated 'it$ Wpositive s!mptomsU (eg" pol!uria" $ematuria" edema) are
more li-el! to develop sign o( disease at earlier stages. 5n patients 'e (ound /0R 2+"22
mABminB1.7+ m
2.
5t s$o's %$roni% -idne! disease stage 2.
N 0.70 6 190 %m
2.97mgBdA
N 2+.22 mmABminB1.7+ m
2

Anemia" '$i%$ in C#& develops primar! as a result o( de%reased renal s!nt$esis o(
er!t$ropoietin" mani(ests as (atigue" redu%ed e6er%ise %apa%it!" impaired %ognitive and immune
(un%tion" and redu%ed :ualit! o( li(e. Anemia is also asso%iated 'it$ t$e development o(
%ardiovas%ular disease" t$e ne' onset o( $eart (ailure" t$e development o( more severe $eart
(ailure" and in%reased %ardiovas%ular mortalit!. 5n patients 'e (ound o( la*oratorium result 'it$
@* 7.20 g;. 5t s$o's anemia.
)!pi%al p$!si%al (indings in persons 'it$ uremia are t$ose asso%iated 'it$ (luid retention"
anemia" and a%idemia. Severe malnutrition %an %ontri*ute to mus%le 'asting" '$ile ele%trol!te
a*normalities ma! %ause mus%le %ramping" %ardia% arr$!t$mias" and mental status %$anges.
Et$er mani(estations o( uremia in end stage renal disease (,SR&)" man! o( '$i%$ are more
li-el! in patients '$o are inade:uatel! dial!3ed" in%lude t$e (ollo'ing :
Peri%arditis : %an *e %ompli%ated *! %ardia% tamponade" possi*l! resulting in deat$
,n%ep$alopat$! : %an progress to %oma and deat$
Perip$eral neuropat$!
Restless leg s!ndrome
/astrointestinal s!mptoms : Anore6ia" nausea" vomiting" diarr$ea
S-in mani(estation : &r! s-in" pruritus" e%%$!mosis
0atigue" in%reased somnolen%e" (ailure to t$rive
>alnutrition
,re%tile dis(un%tion" de%reased li*ido" amenorr$ea
Platelet dis(un%tion 'it$ tenden%! to *leed

)reat t$ese pat$ologi% mani(estations o( %$roni% -idne! disease (C#&) as (ollo's :
Anemia : I$en t$e $emoglo*in level is *elo' 10 gBdA" treat 'it erit$ropoiesis8simulating
agents (,SAs) su%$ as epoetin al(a or dar*epoetin al(a
@!perp$osp$atemia : )reat 'it$ dietar! p$osp$ate *inders and dietar! p$osp$ate restri%tion
Golume overload : )reat 'it$ loop diureti% or ultra(iltration
>eta*oli% a%idosis : )reat 'it$ oral al-ali supplementation
Kremi% mani(estation : )reat 'i$ long T term renal repla%ement t$erap! ($emodial!sis"
peritoneal dial!sis" or renal transplantation)
Cardivas%ular %ompli%ation : )reat as appropriate
/ro't$ (ailure in %$ildren : )reat 'it$ gro't$ $ormone
Corre%tion @iponatremia
(ast Corre%tion N (120897) 6 20 6 0.9 N 401.9 m,: ((inis$ed in 9 $ours. Iit$ aCl +; N
401.9B41+ N977%% N 190 gttB5 mi%ro
Slo' %orre%tion N (1+48124) 6 20 6 0.9 N 220 m,:BA
>aintanan%e N 2 82 m,:BA N 70 T 190 m,:BA
)otal N +20 T 200 m,:
Iit$ 5G0& &4; aCl 0.9; N +20B142 6 1000 N 2077 %% 144 gttB5 mi%ro" (inis$ed in
17 $ours
Corre%tion @ipo-alemia +0 m,: in 90 %% &4;" (inis$ in + $ours N 20 gttB5 mi-ro
1.7 m,:BA 'it$ 0.74 m,:B-g==" +0 m,: #Cl in 90 %% &4;" (inis$ed in + $ours N 20 gttB5
mi%ro
@!ponatremia is de%rease in serum a %on%entration D 1+9 m,:BA %aused *! an e6%ess o(
'ater relative to solute. Common %auses in%lude diureti% use" diarr$ea" $eart (ailure" and renal
disease. Clini%al mani(estation are primaril! neurologi% (due to an osmoti% s$i(t o( 'ater into
*rain %ells %ausing edema)" espe%iall! in a%ute $!ponatremia" and in%lude $eada%$e" %on(usion"
and stupor" sei3ures and %oma ma! o%%ur. S!mptoms o( $!ponatremia in%lude nausea and
vomiting" $eada%$e" s$ort8term memor! loss" %on(usion" let$arg!" loss o( appetite" restlessness
and irrita*ilit!" mus%le 'ea-ness" spasms or %rumps" sei3ure" and de%reased %ons%iousness or
%oma. &iagnosis is *! measuring serum a. Serum and urine ele%trol!tes and osmolalit! $elp
determine t$e %ause. )reatment involves restri%ting 'ater inta-e and promoting its loss" repla%ing
an! a de(i%it" and %orre%ting t$e underl!ing %ause.
P&in%i'a) Ca,$e$ (5 H0'(nat&emia
Me%4ani$m Categor! ,6amples
H0'(6()emi%
40'(nat&emia
De%&ea$e* T+D
an* Na@ 3it4 a
&e)ati6e)0 g&eate&
*e%&ea$e in Na
/5 lossesX &iarr$ea
Gomiting
+rd8spa%e lossesX =urns
Pan%reatitis
Peritonitis
R$a*dom!ol!sis
Small8*o'el o*stru%tion
Renal losses &iureti%s
>ineralo%orti%oid de(i%ien%!
Esmoti% diuresis (glu%ose"
urea" mannitol )
Salt8losing nep$ropat$ies (eg"
interstitial nep$ritis"
medullar! %!sti% disease"
partial urinar! tra%t
o*stru%tion" pol!%!sti%
-idne! disease)
E,6()emi%
40'(nat&emia
In%&ea$e* T+D
3it4 nea&.n(&ma)
t(ta) -(*0 Na
&rugs &iureti%s" *ar*iturates"
%ar*ama3epine "
%$lorpropamide " %lo(i*rate "
opioids" tol*utamide "
vin%ristine
Possi*l! %!%lop$osp$amide "
SA5&s" o6!to%in
&isorders Adrenal insu((i%ien%! as in
Addison disease
@!pot$!roidism
S!ndrome o( inappropriate
A&@ se%retion
5n%reased inta-e o(
(luids
Primar! pol!dipsia
States t$at in%rease ,motional stress
nonosmoti% release
o( A&@
Pain
Postoperative states
H0'e&6()emi%
40'(nat&emia
In%&ea$e* t(ta)
-(*0 Na 3it4 a
&e)ati6e)0 g&eate&
in%&ea$e in T+D
,6trarenal disorders Cirr$osis
@eart (ailure
Renal disorders A%ute -idne! d!s(un%tion
C$roni% -idne! disease
ep$roti% s!ndrome
GGI an* !&*.$'a%e )($$e$ %a,$e 40'(nat&emia i5 &e')a%ement 5),i*$
a&e 40'(t(ni% %(m'a&e* 3it4 )($$e$.
T+D F t(ta) -(*0 3ate&.
5n patients" natrium %on%entration is 97 m,:BA. 5t is severe $!ponatremia (serum a D 109
m,:BA). )reatment is more %ontroversial '$en neurologi% s!mptoms (eg" %on(usion" let$arg!"
sei3ure" %oma) are present. )$e de*ate primar! %on%ern t$e pa%e and degree o( $!ponatremia
%orre%tion. @!pertoni% (+;) saline (%ontaining 41+ m,: aBA) ma! *e used" *ut onl! 'it$
(re:uent (: 2 to 2 $) ele%trol!te determination. 0or patients 'it$ sei3ures or %oma" Y 100 mAB$
ma! *e administrated over 2 to 9 $ in amounts su((i%ient to raise t$e serum a 2 to 9 m,:BA.
)$is amount (in m,:) ma! *e %al%ulated using t$e a de(i%it (ormula as
(&esired C$ange in a) 6 )=I " '$ere )=I is 0.9 6 *od! 'eig$t in -g in men and 0.4 6
*od! 'eig$t in -g in 'omen.
@iponatremia %orre%tion in patients :
0ast Corre%tion N (120897) 6 20 6 0.9 N 401.9 m,: ((inis$ed in 9 $ours. Iit$ aCl +; N
401.9B41+ N977%% N 190 gttB5 mi%ro
Slo' %orre%tion N (1+48124) 6 20 6 0.9 N 220 m,:BA
>aintanan%e N 2 82 m,:BA N 70 T 190 m,:BA
)otal N +20 T 200 m,:
Iit$ 5G0& &4; aCl 0.9; N +20B142 6 1000 N 2077 %% 144 gttB5 mi%ro" (inis$ed in
17 $ours
#alium %on%entration in patient is 1.7 m,:BA. )$e normal potassium level is +.484.0
m,:BA. Ao' potassium is de(ined as a potassium level *elo' +.4 m,:BA. Ao' potassium levels
($!po-alemia)" %an %ause 'ea-ness as %ellular pro%esses are impaired. Ao' potassium %an o%%ur
(or man! reasons. Kse o( 'ater pills (diureti%s)" diarr$ea" and %$roni% la6ative a*use are t$e most
%ommon %auses o( (lo' potassium levels .
12
Et$er %auses o( $!po-alemia in%lude :
12
Ki*ne0 )($$e$
Certain -idne! disorder su%$ as renal tu*ular a%idosis ((or e6ample" %$roni% -idne! (ailure and
a%ute -idne! disease)
>agnesium de(i%ien%!
Aeu-emia
Cus$ingQs disease (and ot$er adrenal disorder)
L($$ (5 '(ta$$i,m t4&(,g4 $t(ma%4 an* inte$tine$
Gomiting
,nemas or e6%essive la6ative use
&iarr$ea
A(ter ileostom! operation
E55e%t (5 me*i%ine
Iater pills (diureti%s)
>edi%ined used (or ast$ma or emp$!sema (*eta8adrenergi% agonist t!pe o( drugs su%$ as
*ron%$odilators" steroids" or t$eop$!lline)
Aminogl!%osides ( a t!pe o( anti*ioti% used (or treating %ertain serious in(e%tions)
S4i5ting (5 '(ta$$i,m int( an* (,t (5 %e))$ %an )(3e& t4e %(n%ent&ati(n (5 '(ta$$i,m
mea$,&e* in t4e -)((*
Kse o( insulin
Certain meta*oli% states (su%$ as al-alosis)
De%&ea$e* 5((* intaEe (& ma)n,t&iti(n
Anore6ia
=ulimia
=ariatri% surger!
Al%o$olism
@!po%alemia %orre%tion:
D 2.4 m,:BA : 0.74 m,:B-g *od! 'eig$t 'it$ ratio &4; 1 : + and (inis$ed in + $.
2.4 T + m,:B A : 0.4 m,: B-g *od! 'eig$t 'it$ ratio &4; 1 : +" and (inis$ed in 2 $.
+.4 m,:BA : 0.24 m,:B-g *od! 'eig$t 'it$ ratio &4; 1 : +" and (inis$ed in 1 $.
5n patients potassium %on%entration is 1.7 m,:BA 'it$ %al%ulated potassium %orre%tion :
1.7 m,:BA 'it$ 0.74 m,:B-g==" +0 m,: #Cl in 90 %% &4;" (inis$ed in + $ours N 20 gttB5 mi%ro
C(n%),$i(n$
A *o! age 14 !ears su((ering (rom stage %$roni% -idne! disease stage 2 'it$ /0R 2+.22
mABminB1.7+ m
2.
REFERENCE
Su'itra #. Pen!a-it /inHal #roni-. 5n: Sudo!o AI" Seti!o$adi =" Al'i 5" # >S Setiati S.
2009. =u-u AHar 5lmu Pen!a-it &alam. ,d 5G. ?a-arta: Pusat Pener*itan &epartemen 5lmu
Pen!a-it &alam< 2009. P 470847+.
Cores$ ?" Astor =C" /reene )" ,lno!an /" Aeve! AS. Prevalen%e o( C$roni% #idne! &isease
And &e%reased #idne! 0un%tion 5n )$e Adult KS Population: )$ird ational @ealt$ and
utrition ,6amination Surve!. Am ? #idne! &is. 200+<21<1812
Remu33i /" Ruggenenti P" Peri%o " 2001. C$roni% renal diseases: renoprote%tive *ene(its o(
renin8angiotensin s!stem in$i*ition. !nn +ntern (ed.2002<1+9:9028914
Aeve! AS" Cores$ ?" =olton #" et al. #idne! &isease Eut%omes &isease 5nitiative" 2012.
Clinical Practice Guideline for Chronic Kidney Disease : Evaluation, Clasification and
Stratification.
oer >S" 2009. C$roni% Renal 0ailure in C$ildren. +n:old &ediatrik.
Sei-al! >/" @o PA" ,mmett A" et al. C$roni% renal insu((i%ien%! in %$ildren: t$e 2001
Annual Report o( t$e APR)CS. Pediatr Ne&hrol. Aug 200+<17(7):7998702.
Ardissino /" &a%%o G" )esta S" et al. ,pidemiolog! o( %$roni% renal (ailure in %$ildren: data
(rom t$e 5tal#id proHe%t. Pediatrics. Apr 200+<111(2 Pt 1):e+7287.
Ati!e$ =A" &a**ag$ SS" /rus-in A=" 2001. ,valuation o( renal (un%tion during %$ild$ood.
Pediatr )ev. 2001<17:1748179
Sreed$aran R" Avner ,&" 2011. C$roni% Renal 0ailure. +n: Nelson Tet"ook of Pediatric. ,d.
19
t$
:172181724.
&il!s A. I$!te and Ri%$ard . 0ine" 2007. C$roni% #idne! &isease in C$ildren. Pediatr.
)ev. 2007<29<++48+21.
#ari ?A" /on3ales C" Aedermann S," S$a' G" Rees A (2000). Eut%ome and gro't$ o( in(ants
'it$ severe %$roni% renal (ailure. #idne! 5nt 47: 197187.
Pardede SE" C$unnaed! S" 2009. Pen!a-it /inHal #roni- pada Ana-. +n: Sari Pediatri.Gol.
11 (+):199820+.
Craven A>" @a'le! C>" >%&onald SP" et al. Predi%tors o( renal re%over! in Australian and
e' Oealand end8stage renal (ailure patients treated 'it$ peritoneal dial!sis. Perit Dial +nt.
>ar8Apr 2007<27(2):172891
?o$n P.Cun$a" &E" 0ACE,P" 2012. Ao' Potassium (@!po%alemia).

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