Chronic Kidney Disease Stage IV

8/20/2019 Chronic Kidney Disease Stage IV

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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!sun%tion is irreversi*le and lasting until renal repla%ement t$erap! (dial!sis" renal

transplantation) is needed.1

C#& is a serious pu*li% $ealt$ pro*lem 'it$ national surve!s s$o'ing a %ondirea*l!

gi$ger prevalen%e t$an appre%iated previousl!.2 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 /R).

C$ildren '$o $ave C#& ma! present to %lini%ians 'it$ a %om*ination o pro*lemsinvolving 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!.4


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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! 1.484.+ per million %$ildren. &ata rom t$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" .81+.9 !) and a prevalen%e o 7.7 per million in t$is population. 7 $e reuen%! 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 !ears t$an in

t$ose !ounger t$an !ears. $e per%entages in t$e APRCS %o$ort 'ere 19; in %$ildren aged

081 !ears< 17; in t$ose aged 812 !ears< ++; in %$ildren aged 284 !ears< and +1; in t$ose older

t$an 12 !ears.

5n 5ndonesia" t$ere is no prevalen%e o C#& in %$ildren nationall!. =ased on stud! in 7

$ospital in 5ndonesia" 29 %$ildren '$o 'ere treated 'it$ renal disease (!ears 19819)" 2;

o t$em are diagnosed 'it$ C#&.RSC> ?a-arta ound .9; rom 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 (1981992) is 0.07; o all patients $ospitali3ed in t$e pediatri% 'ard.4

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$out a de%reased /R or an! patient '$o $as a /R o less t$an 0 mBmin

per 1.7+ m2 lasting or + mont$s or more" irrespe%tive o diagnosis (a*el 1).

a*el 1. Criteria or deinition o C#&

A patient $as C#& i eit$er o t$e ollo'ing %riteria are present :

1. #idne! damage or + mont$" as deined *! stru%tural or un%tional a*normalities o t$e

-idne!" 'it$ or 'it$out de%rease /R" maniested *! 1 or more o t$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. /R D0 m.minB1.7+ m2 or + 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.


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$e normal level o /R varies a%%ording to age" gender" and *od! si3e. $e normal

/R in !oung adults is 120 to 1+0 mBminB1.7+ m 2" '$ereas t$e normal level o /R is mu%$

lo'er t$an t$is in earl! inan%!" even '$en %orre%ted or *od! sura%e area" and su*seuentl!

in%reases in relations$ip to *od! si3e or up to 2 !ears. Staging o C$roni% #idne! &isease *ased

on #&EF5 is s$o'n in ta*el 2.

a*el 2. #&EF5 Classii%ation o t$e stage o C#&

Stage GFR

(mL/min/1.! m"#

De$%&i'tin A%tin P)an

1 90 #idne! damage 'it$ normal or

in%reased /R

reat primar! and %omor*id %onditions"

slo' C#& progression" CG& ris-

redu%tion

2 089 #idne! damage 'it$ mild

redu%tion o /R

,stimate rate o progression

+ +0849 >oderate redu%tion o /R ,valuate and treat %ompli%ations

14829 Severe redu%tion o /R 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 1 !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. Ater 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


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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. 9

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 (1;) and renal $!poplasia and d!splasia (11;). &ata rom t$e ort$

Ameri%an Pediatri% Renal rial and Colla*orative Studies 200 demonstrate ver! similar

inormation (a*le +).10

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 1;

/lomerulonep$ritis 10;

Relu6 nep$ropat$! ;

&il!s A. I$!te and Ri%$ard ine" 200. 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 $as arole in t$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.11


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igure 1. C!%le o t$e %$roni% -idne! disease

Iassner S?" =aum > (1999). C$roni% Renal ailure. P$!siolog! and >anagement. 5n: =arrat

>" Avner ,&" @armon I," ,ditors. Pediatri% ep$rolog!" t$ edition. =altimore: ippin%ott

Iilliams J Iil-ins" pp. 11448112.

@!periltration 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! $!periltration temporaril! preserves total renal

un%tion" it %an %ause progressive damage to t$e surviving glomeruli" possi*l! *! a dire%t ee%t

o t$e elevated $!drostati% pressure on t$e integrit! o t$e %apillar! 'all andBor t$e to6i% ee%t o

in%reased protein trai% a%ross t$e %apillar! 'all. Ever time" as t$e population o s%lerosed

nep$rons in%reases" t$e surviving nep$rons suer an in%reased e6%retor! *urden" resulting in a

vi%ious %!%le o in%reasing glomerular *lood lo' and $!periltration inHur!.9


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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 *enei%ial ee%t o redu%tion in proteinuria. Proteins t$at traverse t$e glomerular

%apillar! 'all %an e6ert a dire%t to6i% ee%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 $!periltration inHur!. @!perp$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! ae%t

glomerular un%tion t$roug$ o6idant8mediated inHur!.9

CLINICAL MANIFESTATION

,arl! %lini%al maniestation o C#& doesnLt s$o' spe%ii% s!mptoms su%$ as di33iness"

letargis" anore6ia" nausea.4 C#& patient rom %$roni% glomerulonep$ritis %an present 'it$

edema" $!pertension" $ematuria" and proteinuria. 5nants and %$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 ine%tion" or overt renal insui%ien%!. Congenital

-idne! disease is diagnosed 'it$ prenatal ultrasonograp$! in man! inants" 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%ii% %omplaints su%$ as $eada%$e" atigue" let$arg!"

anore6ia" vomiting" pol!dipsia" pol!uria" and gro't$ ailure over a num*er o !ears. 9

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!sun%tion" and renal endo%rine un%tion.4

Clini%al maniestation is t$e result o :

1. luid and ele%trol!te im*alan%e

2. overa%umulation o uremi% to6in.

+. @ormonal d!sun%tion (de%rease o er!tropoeitin and vitamin &+)

. unresponse o end organ 'it$ gro't$ $ormone.


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LA+ORATORY FINDING

a*orator! indings %ould *e ound :9

1. %omplete *lood %ount normo%$romi%" normo%!ti% anemia.

2. Renal un%tion est serum ureum and %reatinine.

+. ,le%trolite $!per-alemia" $!ponatremia (i volume overloaded)" a%idosis"

$!po%al%emia" $!perp$osp$atemia.

. Krinalisis proteinuria and $ematuria i %aused *! glomerulonep$ritis in $eav!

proteinuria %ould *e ound $!poal*uminemia.

/lomerular iltration rate (/R) used or staging o C#&. 5n %$ildren" t$e ormula to

estimate patientLs /R are s$o'n *elo' :12

I$ere k is :

1. 0.++ or lo'8*ir$t'ei$t inant D1 !ear old

2. 0.4 or term inant D1 !ear old '$ose 'eig$t is appropriate or gestational age

+. 0.44 or %$ildren (181+ !ears old)

. 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

. Compli%ations" related to level o -idne! un%tion

4. Ris- or loss o -idne! un%tion. Ris- or %ardiovas%ular disease

reatment o C#& s$ould in%lude:

1. Spe%ii% t$erap!" *ased on diagnosis

2. ,valuation and management o %omor*id %onditions


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+. Slo'ing t$e loss o -idne! un%tion

. 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)

. 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&itin

C$ildren '$o $ave C#& $ave nutrition and protein dei%ien%ies or several reasons"

in%luding anore6ia" nausea and vomiting rom t$e uremia" and an a*normal sense o taste. Young

%$ildren" in parti%ular" need sui%ient %alori% inta-e to gro'. Protein inta-e s$ould *e optimi3ed

to allo' or maintenan%e o nitrogen *alan%e and preservation o lean *od! mass. Some patients

ma! reuire supplemental nasogastri% or gastrotom! tu*eeeding i t$e! %annot maintain optimal

$eig$t and 'eig$t gain *! oral eeding. @o'ever" i protein inta-e ise6%essive" $!periltration

ma! o%%ur" leading to in%reased damage to t$e renal paren%$!ma. >i%ropun%ture studies

demonstrate an in%rease in t$e /R ($!periltration) ater an amino a%id load due to redu%tion in

aerent arteriolar resistan%e.

Prostaglandins" '$i%$ %an alter vas%ular tone and in%rease t$e /R" re%entl! $ave *een

impli%ated in t$e development o $!periltration *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 ee%t $as not *een veriied in %$ildren. Redu%ing protein

inta-e to 0. to 1.1 gB-g per da! $as not *een s$o'n to ae%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 inants 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$t gain or gro't$.


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". 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 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 $ave tu*ular dee%ts su%$ as seen 'it$ an%oni s!ndrome.

a*el . reatment o $!per-alemia

Produ%t &ose Potential adverse ee%ts

Sodium *i%ar*onate (0. M == M *i%ar*onate

desired8*i%ar*onated o*served) B2

0.481 m,B-g 5G over 1 $our

@!po%al%emia

Cal%ium glu%onate (10;) 0.481 mB-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

sulonate

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 ine" 200. 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%idiied tu*ular luid" and de%reased titrata*le a%id e6%retion. &e%line in /R *elo' 40; o


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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 elu6 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,B

(20 to 22 mmolB). =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$in

@!pertension is diagnosed in %$ildren '$o $ave C#& *! inding an elevated *lood

pressure reading on t$ree or more separate oi%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 let ventri%ular

status. >edi%ations are adHusted to improve %ardia% un%tion. $ose %$ildren '$o all into t$e

$!pertensive urgen%! and emergen%! %ategor! reuire intravenous medi%ations or a rapid8a%ting

oral medi%ation (niedipine or mino6idil) to redu%e *lood pressure.


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. Anemia

Anemia in C#& is %aused *! eit$er an insui%ient produ%tion o er!t$ropoietin *! t$e

diseased -idne!s or *! iron dei%ien%!. Anemia is deined 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 lie data rom t$e #&EF5 guidelines suggest t$at maintaining t$e $emato%rit in

t$e range o ++; to +; (0.++ to 0.+) and t$e $emoglo*in at 11.0 to 12.0 gBd (110.0 to 120.0

gB) is important or %$ildren '$o $ave C#&. Prior to t$e development o re%om*inant $uman

er!t$ropoietin" patients '$o $ad C#& $ad to undergo transusions to in%rease t$eir $emato%rit

values. ransusions not onl! e6posed patients to various ine%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$er an insui%ient produ%tion o er!t$ropoietin *! t$e diseased -idne!s or

*! iron dei%ien%!. &ue to de%reased appetites" %$ildren '$o $ave C#& %annot in%rease t$eir

iron stores adeuatel! 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 0 and 00 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$ropoietin" dar*epoetin ala" '$i%$ $as a longer $al8lie and reuires

dosing on%e ever! 2 'ee-s to on%e mont$l!" is *eing investigated or use in %$ildren.


12/35

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 multia%torial" in%luding :

gro't$ $ormone (/@) and insulin8li-e gro't$ a%tor85 (5/85) 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 5/85 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!" 5/81 resistan%e pro*a*l! is due to several %auses. 5/8*inding proteins" o

'$i%$ si6 are no' identiied" in%rease 'it$ renal ailure and most li-el! in$i*it t$e a%tions o

5/81 *! *inding 'it$ it" t$ere*! preventing 5/81 rom *inding to its re%eptor. Et$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 dei%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"

ae%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 reuire additional /@ needs

additional investigation.


13/35

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 0; and an age8spe%ii% mortalit! rate

o a*out +0 times t$at seen in %$ildren 'it$out ,SR&. $e most %ommon %ause o deat$ in t$ese

%$ildren is %ardiovas%ular disease" ollo'ed *! ine%tion. E t$e deat$s due to %ardiovas%ular

%auses" 24; 'ere attri*uted to %ardia% arrest (%ause un%ertain)" 1; to stro-e" 1; 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


14/35

CASE REPORT

ame : .A.O

Age : 14 a$un

Se6 : >ale

&ate o Admission : August" 12t$ 201

C4ie5 Cm')aint : oss o %ons%iousness

Hi$t&0:

$is $as *een $appened *! t$e patient 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$rolog! division pediaetri% departemen. ast mont$" t$e patient 'as

re%ommended or $ospitali3ation" *ut t$e patient reused.

@istor! o vomit suered *! patient + da!" reuen%! ive times per da!" volume spoon.$e %ontent is '$at patient eat and drin-. o'" t$e vomit 'as denied.

&iare 'asnQt ound. last dee%ation t$ree da!s ago. @istor! o pale ae%es 'as denied. ast

urination 2 da!s ago. @istor! o urine li-e a tea 'as denied.

ever 'asnQt ound" $istor! o ever also denied

@istor! o sei3ure 'as ound ive mont$s ago" reuen%! on%e a da!. As soon as t$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" urosemide

P40$i%a) E8aminatinGeneralized status

=od! 'eig$t: 0 -g" =od! lengt$: 14 %m

=od! 'eig$t a%%ording to age : 0B4 N 71;

=od! $eig$t a%%ording to age : 14B170 N97;

=od! 'eig$t a%%ording to *od! $eig$t : 0B41 N 7;

5nterpretation : Kndernutrition

Presens status

Cons%iousness: /CS : 1+ (,G>4)" =lood pressure 10B100 mm@g" @R: 9 *pm" RR: 2 *pm"

*od! temperature: +"oC" *od! 'eig$t : 0 -g" *od! $eig$t : 14 %m

Anemi% ()< 5%teri% (8)< C!anosis (8)< ,dema (8). &!spnea (8).

Localized status

Head :

,!e: iso%$ori% pupil (+mmB+mm)" lig$t rele6 (B) " %onHun%tiva palpe*ral inerior pale (B)"

i%teri% s%lera (8).


15/35

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 inlammation"

>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 : dii%ult to assess

Neck :

!mp$ node enlargement (8)" e%- rigidit! (8)

Thora:

5nspe%tion : S!mmetri%al usiormis" epigastrial retra%tion (8)" RR: 20 6Bi" reguler

Palpation : S rig$t N let" normal

Per%ussion : Sonor

Aus%ultation : rales (8B8) " stridor (8B8).

!"do#en:

5nspe%tion : S!mmetri%al usiormis

Palpation : Soepel" iver and spleen unpalpa*le

Per%ussion : !mpani

Aus%ultation : ormoperistalti%

Etre#ities:

,6tremitas superior : Pulse 9 *pm" regular" adeuate pressure and volume" 'arm"

CR D +" spasti% (8B8).

,6tremitas inerior : Pulse 9 *pm" regular" adeuate pressure per volume" 'arm"

CR D +" spasti% (8B8).

$ro%enital :

>ale" 'it$in normal limit.


16/35

La-&at&0 Fin*ing$ (A,g,$t 1"t4 "91# 7

Parameters Galue ormal Galue

Co#&lete 'lood Count

Hemg)-in ."9 g&: 1!." ; 1.! g&:

Hemat%&ite 1.9 : ! ; < :

E&it4&%0te ".! 8 19

2

/mm

!

."9 ; .= 8 19

2

/mm

!

eu%o%!te 11.+7 6 10+ Bmm+ .4 T 11 6 10+Bmm+

Platelet 20.000 Bmm+ 140 8 40Bmm+

MC> !.9 5) = ; C@ +0.0 pg 2 8 +2 pg

>C@C 1.0 gr; ++ 8 +4 gr;

R&I 1+.20 ; 11. T 1. ;

>PG .00 l 7.0 T 10.2 l

PC 0.+;

P&I 7.;

@itung ?enis

eutroil.90;

+780

imosit9.70;

2080

>onosit+.+0;

28

,osinoil0.10;

18

=asoil0.000;

081

eutroil a*solute9.

1"984"

imosit a*solute1.10

+"7810"7

>onosit a*solute 0.+ 0"+80"

,osinoil a*solute 0.01 0"280"4

=asoil a*solute0.00

080"1

Parameters Galue ormal Galue

Ana)i$a Ga$ Da&a4

p@ 7.+1 7.+4 T 7.4

PCE2 27.9 mm@g + T 2

PE2 141.7 mm@g 4 T 100

=i-ar*onat 29.4 mmolB 22 T 2otal CE2 14.1 mmolB 19 T 24

#ele*i$an =asa (=,) 84.0 mmoB (82) T (2)

Saturasi E2 99.+; 94 T 100

Car"ohydrate (eta"olis#

=lood /lu%ose ad random 19.00 mgBd D 200

)enal *unction Test


17/35

U&e,m 1=.99 mg/*L ? 9

C&eatinine .


18/35

Management7@

E6!gen 1 T 2 Bi nasal %anule ()" nasogastri% tu*e (8)

5G& &4; 4 gttBi

Spironola-ton 2 6 24 mg&iet pediasure mil- &iet 1994 --al 0 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


19/35

F))3 U' Patient$

A,g,$t@ 1" t4 "91

S oss o %ons%iousness

O Sens: /CS 1 " emp: +.oC. Anemi% (). 5%teri% (8). ,dema (8). C!anosis (8) &!spnoe

(8)


@ead Rig$t ,!e: Pupil diameter + mm. 5nerior palpe*ra %onHun%tiva pale ().

5%teri% s%lera (8). ig$t rele6 ().

et e!e: Pupil diameter + mm. 5nerior palpe*ra %onHun%tiva pale ().

5%teri% s%lera (8). ig$t rele6 ()..

e%- !mp$ node enlargement (8)$ora6 Simetris usiormis. Retra%tion (8) epigastrial< inter%ostals" suprasternal.

@R: 9 *pm" reguler< murmur (8)

RR: 2 6Bi" regular" rales (8)

A*domen Soepel" Rapid turgor. normoperistalti%. iver" spleen and renal

unpalpa*le.

,6tremities Pulse 9 6Bi" regular" adeuate pBv" 'arm" CR D +U. =lood Pressure

10B100 mm$g

/enital >ale< 'it$in normal limit.

A C$roni% #idne! &isease Stage 5G

P Management7

E2 182 Bi5G& &4; 4 gttB5 mi-ro

Spironola-ton 2624 mg

&iet >il- Pediasure &iet 1994 --al +2 g protein@iponatremia %orre%tion

ast Corre%tion N (12089) 6 0 6 0. N 401. m, (inis$ed in $ours. Iit$ aCl

+; N 401.B41+ N977%% N 10 gttB5 mi%ro

Slo' %orre%tion N (1+48124) 6 0 6 0. N 20 m,B

>aintanan%e N 2 8 m,B N 0 T 10 m,Botal N +20 T 00 m,

Iit$ 5G& &4; aCl 0.9; N +20B14 6 1000 N 2077 %% 144 gttB5 mi%ro"

inis$ed in 1 $ours

@ipo%alemia %orre%tion

1.7 m,B 'it$ 0.74 m,B-g==" +0 m, #Cl in 90 %% &4;" inis$ed in + $ours N

0 gttB5 mi%ro

Planning :,le%trolit test repetition post %orre%tion" ull *lood" and A/&

&ipsti%- result

KroB=ilB#etB=loodBProBitBeuB/[email protected]

A,g,$t 1!t4 "91


20/35

S limp ()" nausea and vomit (8)" ever (8)

O Alert : Compos >entis " emp: +"7oC. Anemi% (). 5%teri% (8). ,dema (8). C!anosis (8)

&!spnoe (8)







@R: *pm" reguler< murmur (8)


A*domen Soupel" Rapid turgor. normoperistalti%. iver" spleen and renal

unpalpa*le.

,6tremities Pulse 6Bi" regular" adeuate pBv" 'arm" CR D +U. =lood Pressure

10B100 mm$g/enital >ale< 'it$in normal limit.


P E2 182 B5

5G& &4; 4 gttB5 mi-ro


&iet 1994 --al 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

5G& N +0 %% =A= N 0 N + %%

otal N 1.42 %% 5I N 190 %% #?= N @S =C

otal N 1.090 %% N 1010 %%

A,g,$t@ 1t4 "91

S limp ()" nausea and vomit (8)"ever (8)

E Alert : Compos >entis " emp: +"oC. Anemi% (). 5%teri% (8). ,dema (8). C!anosis (8)

&!spnoe (8)

=od! 'eig$t: 0 -g" =od! lengt$: 14 %m@ead Rig$t ,!e: Pupil diameter + mm. 5nerior palpe*ra %onHun%tiva pale ().



5%teri% s%lera (8). ig$t rele6 ().. e%- !mp$ node enlargement (8)

$ora6 Simetris usiormis. Retra%tion (8) epigastrial< inter%ostals" suprasternal.


21/35




unpalpa*le.


1+0B90 mm$g/enital >ale< 'it$in normal limit.


P Management7

E2 V 8 1 B5 nasal -anul

5G& &4; 4 gttB5 mi%ro


&iet pediasure mil- &iet 1994 --al +2 g protein

Iater =alan%e

5nput : &iet N 00 %% Eutput : EKP N 00 %% = N 5 8 E

5G& N 140 %% =A= N 0 N 80%%

otal N 740 %% 5I N 190 %% #?= N @S =C

otal N 790 %% N ++4 %%

A,g,$t@ 1t4 "91


O Alert : Compos >entis " emp: +7oC. Anemi% (). 5%teri% (8). ,dema (8). C!anosis (8)

&!spnoe (8)










unpalpa*le.


1+0B90 mm$g

/enital >ale< 'it$in normal limit.A C$roni% #idne! &isease Stage 5G

P >anagement




&iet pediasure mil- &iet 1994 --al +2 g protein


22/35

Planning

KS/ &oppler o -idne! and *ladder

,le%trolit %orre%tion result

CaBaB#BCl N 7.7B121B1.B9+ m,B

ull *lood %ount result

@*B@tBR=CBI=CBPlt N .7B1.9B2.+1610B9.4610+B+1610+

Iater =alan%e


5G& N 140 %% =A= N 0 N 80%%

otal N 740 %% 5I N 190 %% #?= N @S =C

otal N 790 %% N ++4 %%

A,g,$t@ 12

t4

"91S limp ()" nausea and vomit (8)"ever (8)


&!spnoe (8)








RR: 2 6Bi" regular" rales (8)A*domen Soupel" Rapid turgor. normoperistalti%. iver" spleen and renal

unpalpa*le.,6tremities Pulse 110 6Bi" regular" adeuate pBv" 'arm" CR D +U. =lood Pressure

1+0B90 mm$g/enital >ale< 'it$in normal limit.


P >anagement




&iet 1994 --al +2 g protein

Planning


ranussion PRC + %%B-g** in 2 $ours

Iater =alan%e


23/35

5nput : &iet N 100 %% Eutput : EKP N +00 %% = N 5 8 E

5G& N 0 %% =A= N 0 N 00 %%

otal N 100 %% 5I N 200 %% #?= N @S =C

otal N 400 %% N 74 %%

A,g,$t@ 1t4 "91

S limp ()" nausea and vomit (8)"ever (8)O Alert : Compos >entis " emp: +7oC. Anemi% (). 5%teri% (8). ,dema (8). C!anosis (8)

&!spnoe (8)








RR: 20 6Bi" regular" rales (8)A*domen Soupel" Rapid turgor. normoperistalti%. iver" spleen and renal


120B0 mm$g/enital >ale< 'it$in normal limit.


P >anagement

5G& &4; 4 gttB5

Spironola-ton 2624 mg&iet 1994 --al +2 g protein

Planning



A,g,$t@ 1=t4 "91



&!spnoe (8)


@ead Rig$t ,!e: Pupil diameter + mm. 5nerior palpe*ra %onHun%tiva pale ().5%teri% s%lera (8). ig$t rele6 ().






24/35




120B0 mm$g

/enital >ale< 'it$in normal limit.A C$roni% #idne! &isease Stage 5G

P >anagement

5G& &4; 4 gttB5 mi-ro



Planning



Repeat ele%trolit and A/&A

Iater =alan%e


5G& N +00 %% =A= N 200 %% N 200 %%

otal N 1200 %% 5I N 100 %% #?= N @S =C

otal N 1.000 %% N 74 200 %%

74 %%

&ipsti-

KroB=ilB#etB=loBProBitBeuB/luBS/Bp@

8B1B8B8B2B+B8B8B1.010B7.0

A,g,$t@ 1ale< 'it$in normal limit.


25/35


P >anagement

5G& &4; 4 gttB5



Planning


Iater =alan%e

5nput : &iet N 1+00 %% Eutput : EKP N 00 %% = N 5 8 E

5G& N 40 %% =A= N 0 N 8 240 %%

otal N +40 %% 5I N 200 %% #?= N @S =C

otal N 00 %% N 724 %%

A,g,$t@ "9t4 "91


O Alert : Compos >entis " emp: +7.4"oC. Anemi% (). 5%teri% (8). ,dema (8). C!anosis (8)

&!spnoe (8)






e%- !mp$ node enlargement (8)





unpalpa*le.


120B0 mm$g

/enital >ale< 'it$in normal limit.


P >anagement

5G& &4; 4 gttB5



Iater =alan%e


5G& N +00 %% =A= N 200 %% N 200 %%

otal N 1200 %% 5I N 100 %% #?= N @S =C

otal N 1.000 %% N 74 200 %%

74 %%

&ipsti-


26/35

KroB=ilB#etB=loBProBitBeuB/luBS/Bp@

8BB8B8BB8BB8B1.004B.4


27/35

Di$%,$$in

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 inlammator! disease o -idne!s).

Et$er %ommon %auses o %$roni% renal ailure in%lude :

Re%urring p!elonep$ritis (-idne! ine%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 relu6" due to reuent ine%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 *eore !ou noti%e an! s!mptoms.

Patients 'it$ %$roni% -idne! disease (C#&) stage 18+ (glomerular i-tration rate +0

mBminB1.7+ m2) are reuentl! as!mptomati% < in termso possi*le WnegativeU s!mptoms related

simpl! to t$e loss o glomerular iltration rate (/R)" t$e! do not e6perien%e %lini%all! evident

distur*an%es in 'ater or ele%trol!te *alan%e or endo%rineBmeta*oli% derangements.


28/35

/enerall!" t$ese distur*an%es *e%ome %lini%all! maniest 'it$ C#& stages 84 (/R D +0

mBminB1.7+ m2 ). 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 /R 2+"2

mBminB1.7+ m2. 5t s$o's %$roni% -idne! disease stage .

N 0.70 6 10 %m

.97mgBd

N 2+.2 mmBminB1.7+ m2

Anemia" '$i%$ in C#& develops primar! as a result o de%reased renal s!nt$esis o

er!t$ropoietin" maniests as atigue" redu%ed e6er%ise %apa%it!" impaired %ognitive and immune

un%tion" and redu%ed ualit! o lie. 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$er maniestations o uremia in end stage renal disease (,SR&)" man! o '$i%$ are more

li-el! in patients '$o are inadeuatel! dial!3ed" in%lude t$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 maniestation : &r! s-in" pruritus" e%%$!mosis

atigue" in%reased somnolen%e" ailure to t$rive

>alnutrition

,re%tile disun%tion" de%reased li*ido" amenorr$ea


29/35

Platelet disun%tion 'it$ tenden%! to *leed

reat t$ese pat$ologi% maniestations o %$roni% -idne! disease (C#&) as ollo's :

Anemia : I$en t$e $emoglo*in level is *elo' 10 gBd" treat 'it erit$ropoiesi8simulating

agents (,SAs) su%$ as epoetin ala or dar*epoetin ala

@!perp$osp$atemia : reat 'it$ dietar! p$osp$ate *inders and dietar! p$osp$ate restri%tion

Golume overload : reat 'it$ loop diureti% or ultrailtration

>eta*oli% a%idosis : reat 'it$ oral al-ali supplementation

Kremi% maniestation : 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 @iponatremiaast Corre%tion N (12089) 6 0 6 0. N 401. m, (inis$ed in $ours. Iit$ aCl +; N

401.B41+ N977%% N 10 gttB5 mi%roSlo' %orre%tion N (1+48124) 6 0 6 0. N 20 m,B

>aintanan%e N 2 8 m,B N 0 T 10 m,B

otal N +20 T 00 m,Iit$ 5G& &4; aCl 0.9; N +20B14 6 1000 N 2077 %% 144 gttB5 mi%ro" inis$ed in

1 $oursCorre%tion @ipo-alemia +0 m, in 90 %% &4;" inis$ in + $ours N 0 gttB5 mi-ro

1.7 m,B 'it$ 0.74 m,B-g==" +0 m, #Cl in 90 %% &4;" inis$ed in + $ours N 0 gttB5mi%ro

@!ponatremia is de%rease in serum a %on%entration D 1+ m,B %aused *! an e6%ess o

'ater relative to solute. Common %auses in%lude diureti% use" diarr$ea" $eart ailure" and renal

disease. Clini%al maniestation are primaril! neurologi% (due to an osmoti% s$it o 'ater into

*rain %ells %ausing edema)" espe%iall! in a%ute $!ponatremia" and in%lude $eada%$e" %onusion"

and stupor" sei3ures and %oma ma! o%%ur. S!mptoms o $!ponatremia in%lude nausea and

vomiting" $eada%$e" s$ort8term memor! loss" %onusion" let$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 dei%it" and %orre%ting t$e underl!ing %ause.


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P&in%i'a) Ca,$e$ 5 H0'nat&emia

Me%4ani$m Categor! ,6amplesH0'6)emi%

40'nat&emia

De%&ea$e* T+

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 dei%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+

3it4 nea&n&ma)

tta) -*0 Na

&rugs &iureti%s" *ar*iturates"

%ar*ama3epine "

%$lorpropamide " %loi*rate "

opioids" tol*utamide "

vin%ristine

Possi*l! %!%lop$osp$amide "

SA5&s" o6!to%in&isorders Adrenal insui%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


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nonosmoti% release

o A&@

Pain

Postoperative statesH0'e&6)emi%

40'nat&emia

In%&ea$e* tta)

-*0 Na 3it4 a

&e)ati6e)0 g&eate&

in%&ea$e in T+

,6trarenal disorders Cirr$osis

@eart ailure

Renal disorders A%ute -idne! d!sun%tion

C$roni% -idne! disease

ep$roti% s!ndromeGI an* !&*$'a%e )$$e$ %a,$e 40'nat&emia i5 &e')a%ement 5),i*$

a&e 40'tni% %m'a&e* 3it4 )$$e$.

T+ tta) -*0 3ate&.

5n patients" natrium %on%entration is 9 m,B. 5t is severe $!ponatremia (serum a D 109

m,B). reatment is more %ontroversial '$en neurologi% s!mptoms (eg" %onusion" let$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, aB) ma! *e used" *ut onl! 'it$

reuent ( 2 to $) ele%trol!te determination. or patients 'it$ sei3ures or %oma" 100 mB$

ma! *e administrated over to $ in amounts sui%ient to raise t$e serum a to m,B.

$is amount (in m,) ma! *e %al%ulated using t$e a dei%it ormula as

(&esired C$ange in a) 6 =I " '$ere =I is 0. 6 *od! 'eig$t in -g in men and 0.4 6 *od! 'eig$t in -g in 'omen.

@iponatremia %orre%tion in patients :

ast Corre%tion N (12089) 6 0 6 0. N 401. m, (inis$ed in $ours. Iit$ aCl +; N

401.B41+ N977%% N 10 gttB5 mi%roSlo' %orre%tion N (1+48124) 6 0 6 0. N 20 m,B

>aintanan%e N 2 8 m,B N 0 T 10 m,Botal N +20 T 00 m,

Iit$ 5G& &4; aCl 0.9; N +20B14 6 1000 N 2077 %% 144 gttB5 mi%ro" inis$ed in

1 $ours

#alium %on%entration in patient is 1.7 m,B. $e normal potassium level is +.484.0

m,B. o' potassium is deined as a potassium level *elo' +.4 m,B. o' potassium levels

($!po-alemia)" %an %ause 'ea-ness as %ellular pro%esses are impaired. o' potassium %an o%%ur


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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 .1

Et$er %auses o $!po-alemia in%lude : 1

Ki*ne0 )$$e$

Certain -idne! disorder su%$ as renal tu*ular a%idosis (or e6ample" %$roni% -idne! ailure and

a%ute -idne! disease)>agnesium dei%ien%!eu-emia

Cus$ingQs disease (and ot$er adrenal disorder)

L$$ 5 'ta$$i,m t4&,g4 $tma%4 an* inte$tine$

Gomiting

,nemas or e6%essive la6ative use&iarr$ea

Ater ileostom! operation

E55e%t 5 me*i%ineIater 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 ine%tions)

S4i5ting 5 'ta$$i,m int an* ,t 5 %e))$ %an )3e& t4e %n%ent&atin 5 'ta$$i,m

mea$,&e* in t4e -)*

Kse o insulin

Certain meta*oli% states (su%$ as al-alosis)

De%&ea$e* 5* intae & ma)n,t&itin

Anore6ia=ulimia

=ariatri% surger!

Al%o$olism

@!po%alemia %orre%tion:

D 2.4 m,B : 0.74 m,B-g *od! 'eig$t 'it$ ratio &4; 1 : + and inis$ed in + $.

2.4 T + m,B : 0.4 m, B-g *od! 'eig$t 'it$ ratio &4; 1 : +" and inis$ed in 2 $.+.4 m,B : 0.24 m,B-g *od! 'eig$t 'it$ ratio &4; 1 : +" and inis$ed in 1 $.

5n patients potassium %on%entration is 1.7 m,B 'it$ %al%ulated potassium %orre%tion :1.7 m,B 'it$ 0.74 m,B-g==" +0 m, #Cl in 90 %% &4;" inis$ed in + $ours N 0 gttB5 mi%ro


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Cn%),$in$

A *o! age 14 !ears suering rom stage %$roni% -idne! disease stage 'it$ /R 2+.2

mBminB1.7+ m2.


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REFERENCE

Su'itra #. Pen!a-it /inHal #roni-. 5n: Sudo!o AI" Seti!o$adi =" Al'i 5" # >S Setiati S.

200. =u-u AHar 5lmu Pen!a-it &alam. ,d 5G. ?a-arta: Pusat Pener*itan &epartemen 5lmu

Pen!a-it &alam< 200. P 470847+.Cores$ ?" Astor =C" /reene " ,lno!an /" eve! AS. Prevalen%e o C$roni% #idne! &isease

And &e%reased #idne! un%tion 5n $e Adult KS Population: $ird ational @ealt$ and

utrition ,6amination Surve!. Am ? #idne! &is. 200+/" @o P" ,mmett " et al. C$roni% renal insui%ien%! in %$ildren: t$e 2001Annual Report o t$e APRCS. Pediatr Ne&hrol . Aug 200+" @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

Chronic Kidney Disease Stage IV

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