Nephrotic Syndrome Fix

8/19/2019 Nephrotic Syndrome Fix

1/79

NephroticSyndrome

Jewel Montolalu, MD


2/79

Identity


3/79

History of Present Illness


4/79

History of Present Illness


5/79

Review of Systems

– General : no dizziness, no headache, (+) edema

– Skin: no jaundice, non pallor, rashes (+)

– HEENT : No ear pain, no epistaxis, no nasal discharge, noblurring of vision

– Respiratory : no !", occasional cough

– Heart : No palpitations, no easy fatigability


6/79

Review of Systems

– GIT: no abdominal pain, no nausea, no vomiting, nodiarrhea, no constipation

– GUT: !liguria (+), no dysuria, no hematuria

– Musculoskeletal: No joint pains, no limitation ofmovement

– Nervous system: No seizure, no behavioral

changes, no headache


7/79

Past Medical History

–#revious hospitalizations

$ugust %&' Nephrotic Syndrome

– (*) asthma

– (*) allergy


8/79

Family History

aternal -idney disease.,

/ypertension#aternal 0nrecalled


9/79

Birth & Maternal History

– 1ull term to a 23#3 (3&&3) via

NS, non institutional delivery– No prenatal chec4 up

– No ne5born screening test


10/79

Nutritional History

– "reastfed for ' year old6

– Semi*solids introduced at 7months

– Not a pic4y eater


11/79


12/79


13/79

Immunization History

– "82'

– #;:

– !#


14/79

Physical Examination

– General: conscious, irritable, not inrespiratory distress,

– Vital Signs:– "# =&>7& mm/g

– /? ''& bpm

– ?? :% bpm

– ;emp :@°8

– Weigt: '767 4g

– !eigt: @7cm

– "MI: %&6= (z score &) "S# &6A


15/79

PHYSICAL EXAMINATION

S$INpustular rashes at upper andlo5er extremities, open 5ound at

left gluteal 5ith blood dischargeand foul smelling, no jaundice,no cyanosis, no pallor

!%%NT

$nicteric sclera, pin4 palpebralconjunctiva, no naso*auraldischarge, no cervical lymphadenopathy


16/79


!%#&T

$dynamic precordiumNormal rate?egular rhythmNo murmur

'!%ST #ND (UNGSSymmetrical chest expansionNo retractions?ales (+) 5heeze (*)


17/79


%)T&%MITI%S#itting edema,pustular lesion, fulland eBual pulses

#"D*M%N+U&IN#& 2lobular, distended,

(+) ascites, (+) Cuid5ave, Dsh mouthumbilicus


18/79


19/79

Admitting Diagnosis

Nephrotic SyndromePneumoniaPyoderma


20/79

Course in the

Ward


21/79

Admission Day

Su-.ective *-.ective #ssessme

nt /lan

1ebrile2eneralized

edemaS4in rashes,itchy

#eriorbital s5elling?ales (+)istension abdomen

Scrotal s5elling#ustular lesion atextremities

"# =&>7& mm/g/? ''& bpm?? :% cpm

; :76@*:@6% E8

NephroticSyndrome,#neumonia,#yoderma

edsF" atmaintenance8efuroxime F< G@('&Am4)#rednisone'&mg>ml, ml !1urosemide '&mg F<B'%Ho5 salt diet

011+23+145"UN 462'reatinine71 0(5'olesterol 869 0!5

#l-umin 1;6 0(5

'"'012+23+145

/ct ::/gb ''%I"8':6=&Hym 37ono 3

Jos ASeg3:

Urinalysis011+23+145

Sp6 2rav '6&%&p/ 76&Heu esterase *I"8 &*%?"8 @*'&ucus ++

"acteria +

''>%=>'"lood 8S no gro5th

'est )ray 011+23+145

;he lungs underaerated dueto elevated diaphragm6/aziness at the left mid toinner lung zones due to

#neumonia6 ;here is s5elling of the


22/79

Date Intake *ut


23/79

First hospitalization day


nt /lan

1ebrile2eneralize

edemaS4in rashes

#eriorbitals5elling

istensionabdomenSctrotal s5elling#ustular rashes atextremities

"# =&>7&*'&&>A&mm/g/? =&*''& bpm?? %@*:3 cpm

; :76A*:@6@ E80! '6Acc>4g>%3

hours + 'cc

NephroticSyndrome,

#neumonia,Scabies

edsF" at -

8efuroxime F< ;F('&Am4)#rednisone'&mg>ml, ml !1urosemide '&mg F<B'%

Ho5 salt diet#ermethrin


24/79

Date Intake *ut


25/79

Second to fourth Hospitalization Day


nt /lan

$febrile2eneralize

edemaS4in rashes

#eriorbitals5elling

istensionabdomenSctrotal s5elling#ustular rashesat extremities

"# =&>7&*''&>A& mm/g/? @@*''& bpm?? %@*:% cpm ; :76@*:A6' E8

0! 36Acc>4g>%3*

NephroticSyndrome,Scabies

edsF" at

maintenance ;ransfuse :$lbumin %&K8efuroxime F< ;F('&Am4)

#rednisone'&mg>ml, ml!1urosemide '&mgF< B'%#ermethrin lotion


26/79

Date Intake *ut


27/79

Fifth to Ninth Day Hospitalization Day

Su-.ective *-.ective #ssessment

/lan

1ebrile2eneralizeedema

8ough (+)

ecreaseperiorbital,abdomen andscrotal s5ellingNo pustularrashes"ibasal rales (+)Iheezing (+)

'63 from '6@4g"# =&>7&*''&>A&mm/g/? '&*'%& bpm?? %@*:3 cpm

; :76@*:@6 E80!%6%cc>4g>%3hrs

NephroticSyndrome,#neumoniaScabies

edsF" at maintenance

;ransfuse : $lbumin%&K

8iproCoxacin (%m4)F< G'%$zithromycin !('&m4)#rednisone '&mg>ml, ml !1urosemide '&mg F<

B'%Salbutamol G7

?epeat 8"8, 0rinalysis,Jlectrolyte, 8hest Lray

'"'012+=+145

/ct :&/gb '&&I"8 @6%AHym 3@ono %Jos &

Seglt '@

'est )ray 012+=+145

Fncreased bilateral conCuentpneumonic inDltrate

Urinalysis012+=+145

Sp6 2rav '6&'&p/ A6&Heu esterase *I"8 %*3?"8 @*'%ucus +

"acteria ++#rotein ++

012+8+145Na 17;$ 762

''>%=>'

"lood 8S no gro5th


28/79

Date Intake *ut


29/79

Tenth to twelfth Hospitalization Day


nt /lan

$febrileecrease

edemaNo cough

ecreaseperiorbital,

abdomen andscrotal s5ellingNo pustularrashes"ibasal rales (*)Iheezing (*)

'63 frm '6@4g"# =&>7&*''&>A&mm/g/? '&*'%& bpm?? %@*:3 cpm

; :76@*:A E80!

NephroticSyndrome,

#neumoniaresolvedScabiesresolved

edsF" at maintenance

;ransfuse ' $lbumin

%&K8iproCoxacin shift tooral#rednisone increase to% m4Salbutamol G'%Spironolactone at 'm4


30/79

Date Intake *ut


31/79

Thirteenth Hospitalization Day


nt /lan

1ebrileNo edema

No cough

ecreaseperiorbital,

abdomen andscrotal s5ellingNo pustularrashes"ibasal rales (*)Iheezing (*)

'63 frm'6@4g"# =&>7&*''&>A& mm/g

/? '&*'%&

NephroticSyndrome,

#neumoniaresolvedScabiesresolved

ischarged6/ome eds• 8iproCoxacin &&

mg>tab, M tab "F(:&m4)

• #rednisone%&mg>ml, 3 ml "Ffor 3 5ee4s (%m4)

• Fsoniazid%&&mg>ml, :6 mlfor : months

• Salbutamol neb B'%


32/79

Date Intake *ut


33/79


34/79


35/79


36/79


37/79


38/79

Discussion


39/79


40/79

What are the kidneys and what do

they do?

;5o bean*shaped organs,

each about the size of aDst

1ilter '%& to '& Buarts ofblood O ' to % Buarts of

urineillion Dltering units callednephrons O Dlters a small

amount of blood

;he nephron includes a Dlter,called the glomerulus and a

tubule


41/79

2lomerulus lets Cuid and

5aste products passthrough, prevents bloodcells and largemolecules, mostly

proteins from passing6

;ubule sends needed

minerals bac4 to thebloodstream andremoves 5astes


42/79

DefinitionNS is a glomerular disorder characterised by atetrad of:

– #roteinuria more than 'g>m%>%3hr

–/ypoproteinemia (albumin less than%6gm>dl)

– /ypercholesterolemia more than%%&mg>dl

– Jdema


43/79

Epidemiology

– Iorld5ide approximately '7 cases per '&&,&&& children 5ithan incidence of % to A per '&&,&&& children6

– 0nited States %*A cases per '&&,&&& children younger than '7years6

– #hilippines %:7@ out of %A7@%3: cases (##S %&''*%&')

– ales more aPected than females at a ratio of %' in children,but this predominance fails to persist in adolescence


44/79

Classification

1. Idiopathic nephrotic syndrome

(90% of cases)– Minimal cange @isease

– Aocal segmental glomerulosclerosis isscarring in scattered regions of the 4idney

– Mem-rano


45/79


46/79


47/79

Classification

3. Congenital nephrotic syndrome !

presenting at "irth or during the 1st# months

–Fnherited genetic defects, 5hich areproblems passed from parent to childthrough genes

–


48/79


49/79

Etiology and Pathogenesis

21?


50/79


51/79

Clinical Manifestation

$he initial attac and su"se&uent relapsesmay follo' a iral upper respiratory infection.

dema.

*eight gain.

+iminished urinary output.

!espiratory di,culty.

+iarrhea.

Normal "lood pressure


52/79


53/79


54/79

Complications

Fnfection "lood clots /igh bloodcholesterol


55/79

Infection

– *hy are nephrotic patients more suscepti"le toinfection-

ecrease immunity Jdema Cuid is a good culture medium6

Fmmunosuppressive therapy6

– ost common organism/ Strep6 #neumoniae6

2ram negative organisms6

– ost common sites of infection #eritonitis (commonest )

Sepsis, pneumonia, cellulitis, urinary tract infection6

– anifestations of infection during steroid therapy


56/79

High blood cholesterol

– Ihen albumin lea4s into the urine, thealbumin levels in the blood drop6

– ;he liver ma4es more albumin to ma4e up

for the lo5 levels in the blood6 $t the sametime, the liver ma4es more cholesterol6


57/79

Laboratory

0rine test

– Di%>hr or R'&& mg>%>day

Nephrotic range 3& mg>%>hr or O'&&& mg>%>day

– Urine al-umin?to?creatinine ratio6

Normal R&6% mg protein>mg creatinine

Nephrotic % mg protein>mg creatinine


58/79


59/79

When to consider renal biopsy?


60/79


61/79

Treatment

$he t'o principlelines of treatmentare

JPort to reduce edema6

SpeciDc therapy 5ith prednisone


62/79

Effort to reduce edema.

iuretics

1urosemide

starting at '*% mg>4g>d F< may improve edema

inhibits reabsorption of Na8l O increased excretion of 5ater, -, Na,8l, g and 8al6

Spironolactone

'6*:6:mg>4g>day or 7&mg>"S$

Fncreasing sodium chloride and 5ater excretion 5hile conservingpotassium and hydrogen ions

;herapeutics #rescription in 8hildhood

?enal isease/e@iatric Ne


63/79

Effort to reduce edema.

$lbumin

– %K albumin at a dose of ' g>4g body 5eightgiven as a continuous infusion over %3 hours6

– Fntravenous albumin may be particularlyuseful in diuretic resistant edema and inpatients 5ith signiDcant ascites and>orscrotal, penile or labial edema6


64/79

Specific Therapy

– #rednisone

7&mg>m%>day given in : divided daily doses, for 35ee4s then start alternate day therapy6

– $lternate day therapy

prednisone 3&mg>m%>day ta4en as single morningdose 5ith brea4 fast 6 ;he alternate day therapy isthen tapered slo5ly and discontinued over the next: months6


65/79

Classification according to

response to steroids

Steroid responsive :

8hildren 5ho respond to

treatment 5ithin @ 5ee4s oftreatment6


66/79

Classification according to

response to steroids

Relapse :

– #roteinuria more than :+ and edema– aily steroids is given until proteinuria

is negative or only trace by dip stic4for : consecutive days ,then the

patient is shifted to alternate daytherapy and treatment is tapered over% months


67/79

hildren 5ith no relapses 8hildren 5ith relapses

FnfreBuent relapser1reBuent relapserSteroid dependent

Infre&uent relapser relapse less than 3 times in a'% months period6

re&uent relapser relapse more than 3 times 5ithin'% months periodteroid dependent relapse 5hile on alternate daytherapy or 5ithin '3*%@ days of stopping treatment


68/79

Classification according

to response to steroids

Steroid resistant:

#atients 5ho fail to

respond to treatment5ithin @ 5ee4s oftreatment6


69/79

Nephrotic Syndrome in Childhood

Complications of Steroid Therapy

–Behavior/sleep changes–Weight gain & distribution

–“Cushingoid facies”–Striae–Growth arrest

–Osteoporosis

–Hyperglycemia–Hypertension–Risk of ulcer–Hyperlipidemia


70/79

Cytotoxic drugs

(as cyclophosphamide)

Fndications

– 8orticosteroid toxicity in freBuentrelapses and steroid dependent,

– Steroid resistant nephrotic syndrome

(after renal biopsy )


71/79

Nephrotic syndrome in children

5 year status in various eras

Pre

antibiotic

Post

antibiotic

Early

steroid

Current

Survival 51% 62% 78% >95%

Persistentproteinuria

11% 2% !6% 2"%

#e$ission 8% 9% 9% 71%


72/79

Diet

$ sodium*restricted diet

#rotein restriction is notindicated

(except in cases of acute or chronic4idney failure 5hen severe azotemiais present)


73/79

Vaccines

#neumococcal and varicella

vaccines may be given oncethe child is in remission6


74/79

Prognosis

– Since the introduction of corticosteroids,

the overall mortality of FNS hasdecreased dramatically from over &Kto approximately %*K6

– Ft is important to remember that

children 5ith this disease have anexcellent long*term outloo4 and can livelong, healthy lives6


75/79

Summary

'il@oo@ ne


76/79

SummaryTe 7 ty


77/79

Summary

Te signs an@ sym


78/79


79/79

THANK YOU

Documents

Nephrotic Syndrome Fix