REFERAT

NEPHROTIC SYNDROM FREQENT RELAPS

Created by:
Chriscahya Wibisana Candra
0861050169

Pembimbing :
Prof dr. Taralan Tambunan, SpA(K)

KEPANITERAAN KLINIK ILMU PENYAKIT ANAK

PERIODE 6 AGUSTUS 2012 6 OKTOBER 2012
FAKULTAS KEDOKTERAN
UNIVERSITAS KRISTEN INDONESIA
JAKARTA
2012

BAB I

INTRODUCTION
Praise and gratitude i give to God upon the blessings and of his so writer can
finish drafting a Referat with a title Nephrotic Syndrome Frequent Relapse. This
Referat is arranging by the writer to completing task in Pediatric Department at RS
FK-UKI.
Writer would give a million thanks to Prof. Dr. Taralan Tambunan, SpA (K),
who has been guiding and teaching the writer in process of arranging referat, so the
writer gets more understanding in Nephrotic Syndrome Frequent Relapse disease
Writer realizes there are still many deficiencies both on the content and format
of this Referat. Therefore, writer accepts all criticism and input with open arms and
apologizes if there are errors in the Referat task that writer created.
Writer hope that this referat could be a media for information and reference for
the reader when facing a case that have a link to a kidney disease in general and
especially in Nephrotic Syndrome
In final words writer would like to say a million thanks to all, may this referat
can give more information for us all

Jakarta, Agustus 2012

Chriscahya Wibisana Candra

BAB II

CONTENT
II.1 Preliminary
Syndrome Nephrotic is a chronical disease that usually found in child, with
incident 2-4 cases in 100.000 children under 16 every year. histopathology deviation is
most happen in syndrome nephrotic, syndrome nephrotic can attack every age,
especially child in 2-6 years, man is get nephrotic syndrome more frequent than
women in ratio 3:2 more than 90% case of syndrome nephrotic is idiopathic, and the
rest is secondary syndrome nephrotic that causes by many kind of disease1
Syndrome Nephrotic Frequent Relapse is when its happen more than 2 times
in 6 month or relapse more than 4 times in 1 years, usually after initial treatments, 94
% cases get total remission of the disease, and the rest 60-70% will become relapse 3040% will become frequent relapse2

II.2 Etiology2
Nephrotic syndrome can be primary, as part of a systemic disease, or secondary
due to several causes
1. Primary causes:
a. post infection
b. Collagen vascular disease (SLE, rheumatoid arthritis, polyarteritis
nodosa)
c. Henoch-Schnlein purpura
d. Hereditary nephritis
e. Sickle cell disease
f. Diabetes Mellitus
g. Amyloidosis
h. Malignancy (leukemia, lymphoma, Wilms Tumar, pheochromocytoma)
i. Toxin (bee stings, snake venom)
j. Drugs (probenecid, fenoprofen, catopril, lithium, wafarin, penicilamine,
mercury, gold, trimethadione, the metadione, NSAIDs)

2. Secondary causes
a. Group A beta-hemolytic streptococcus
b. Syphilis
c. Malaria
d. Tuberculosis
e. viral infection
Most (90%) of the children were suffering from nephrotic syndrome have
some form of idiopathic nephrotic syndrome, including; disease lesions of at least
about 85%, the proliferation of mesangium 5%, and 10% local sclerosis. In the
remaining 10% of children suffer nephroses include. Nephrotic syndrome is largely
mediated by some form of glomerulonephritis and most often affects the membranous
and membranoproliferatif.

II.3 Pathofisiology3
Proteinuria is a major symptom of nephrotic syndrome, proteinuria occurred
more severe than proteinuria in others kidney disease. The amount of protein in the
urine can got 40mg/jam /m2 body surface area (1 gram / m 2/day) or 2 to 3.5 grams / 24
hours. Proteinuria that occur due to changes in the selectivity of the protein, and
changes in the glomerular filter.
Changes in selectivity to proteins and glomerular filtration rate changes will
depend on the type of glomerular abnormalities. But the outline can be explained that,
in the normal child, filtration of low molecular weight plasma protein negatively
charged in glomerular basement membrane is normally maintained by the negative
charge filtration barrier. The negative charge of the molecule consists of heparan
sulfate proteoglycans. In people with nephrotic syndrome, the concentration of heparan
sulfate saccharide mucopoly at basal membrane is very low. So many proteins can pass
through the barrier. In addition there is also a change gap size (pores) on the barrier so
that the neutral charge protein can pass through the barrier.

In Nephrotic syndrome occurs hipoproteinemia mainly albumin, this is caused

by increased excretion of albumin in the urine and increased degradation in the renal
tubules that exceed power synthesis of the liver. Disruption of protein in plasma was
decreased -1 globulin. While 2globulin -, -globulin and fibrinogen increased
relative or absolute. -2globulin increased due to the selective retention of high
molecular weight proteins by the kidney, while the rate of synthesis is relatively
normal.
There are two theories regarding the pathophysiology of edema in nephrotic
syndrome; theory underfilled and the theory overfille. In underfill theory described
edema formation occurs because of decreased albumin (hipoalbuninemia), due to loss
of protein in the urine. Hypoalbuminemia causes a decrease in plasma oncotic
pressure, which allows the transudation of fluid from the inervaskular to interstisial
space. Intravakular volume decrease causes a decrease in renal perfusion pressure,
resulting in the activation of the renin-angiotensin-aldosterone system, which stimulate
sodium reabsorption distal tubulus. Decrease in intravascular volume underfilled also
stimulates the release of hormones that enhance antideuritik water absorption in the
collecting ducts. Due to the lack of oncotic pressure fluid and sodium that has been
reabsorbed back into the interstitial space so that aggravate edema.
In theory described overfill sodium and water retention caused by intra-renal
mechanisms of primary and independent of systemic peripheral stimulation. And the
presence of agents that increase the permeability of the capillary circulation throughout
the body and kidneys. Primary sodium retention caused by intra-renal defect is causing
the expansion of the plasma and extracellular fluid. Edema that occurs due to
overfilling of fluid into the interstitial space.

II.4 Diagnosis1
Syndrome Nephrotic can be diagnose based on 4 specific symptomps1
1. Masif Proteinuria or Proteinuria Nephrotic
Urine contain protein 40 mg/m2lpb/jam or > 50mg/kgBB/24hours,
ratio albumin/kreatinin in urine > 2 mg/mg, or dipstik 2 .
2. Hipoalbuminemia
Albumin serum < 2,5 g/dl. Normal albumin serum in child is 3,64,4g/dl. In syndrom nephrotic water retension and swelling can be seen
if albumin under 2,5-3,0 g/dl
3. Swelling
4. Hiperlipidemia
Pasien syndrome nephrotic mengalami hiperkolesterolemia ( cholesterol
more than 200mg/dl

II.5 Examination1,3
1. Urinalisis and Urine Culture
2. Quantitative protein urin, can be 24 hours urine, or ratio protein/kreatini
in first urine in the morning
3. Blood
a. Peripheral Blood (Hemoglobin, White Blood Cell,Diff Count,
Trombosit, Hematokrit
b. Levels of albunin and plasma cholestrol
c. Ureum and Creatinine and Creatinine Clearance
d. ASO titer and levels of complement C3 if have microscopic
hematuria presisten
4. Kidney Biopsy
Kidney biopsy usually not needed in most of child with nephrotic
syndrome, more than 80% child with nephrotic syndrome, is a nephrotic
syndrome with minimal lesion with typical characteristic when see in light
microscope the normal histological kidney, the indication for kidney biospy is

II.6 Treatment1
Initial treatment for nephrotic syndrom according to ISKDC (International
Study on Kidney Disease in Children), initial prednisone start with full dose
2mg/kgBB/day or 60mg/m2LPB/day (maximum dose 8o mg/hari) that divided into 3
times of administration, for induce remision, full dosage of prednisone is administrate
for 4 weeks, if there are remition after 4 weeks of treatment , the dosage of steroid for
further 4 weeks is 40 mg/m2LPB/day (2/3 of full dose), 1 times a day after breakfast, if
4 weeks early treatment not giving a remition patient will catogorize as resisten steroid

Today there are 4 option for treatment in syndrom nephrotic frequent relaps,
and that is :
1. Long Term Steroid
many eksperiment show that long term steroid can be try at first before
giveing siklofosfamid (CPA), knowing the side effect of steroid is small. If it
has been diagnose as syndrom nephrotic frequent relaps, after get to remision
with prednison full dosage, the threatment continued with altrenating steroid
that tamprering off slowly 0,2 mg/kgBB until it reach smallest dosage that no
causeing relaps 0,1-0,5 mg/kgBB , this dosage called, threshold dosage

and

can be continued for 6 12 month, then tried to stop. In most cases schoolchild
can tolerate prednison 0,5 mg/kgBB and preschoolers can tolerate until 1
mg/kgBB/day in alternating1
2. Levamisol
Levamisol is drugs with effect immunomodulating selT. Consumptin of
levamisol on nephrotic syndrom still limited becasue the effect is till doubtful .
in Jakarta study show giving levamisol show bad result, side effect of levamisol
is nausea, regurgutate, amd reversible neutropenia, the dosage of levamisol is
2,5 mg/kgBB single dosage for 4-12 month
3. Sitostatik
Sitostatik drugs that usually used in treatment nephrotic syndrom in child is
siklofosfamid (CPA) with dosage 2-3mg/kgBB for 8 weeks. Stiostaika can
reduce relaps more than 50%, that is 67-93% in first years, and 36-66% for 5
years. APN report that giving CPA for 12 weeks can keep the remition longer
than giving CPA for 8 weeks, but this statment not confirmation by other
researcher
Administration of CPA on keeping remition more effectiveon nephrotic
syndrom frequent relaps (70%) than sindrom nephrotic steroid dependen
(30%). Side effect of sitosatika is bone marrow depressio, aloplesia, sistisis
hemorage, azospremia and on long trem can cause malignacy
4. Siklosporin (CyA)
On Nephrotic Syndrome that not responsif in steroid and sitostatik treatment
its recommended to giving siklosporin, that inhibit the calcineurin with dosage 56mg/kgBB/day to keep the consentration in blood. CyA can make and keep the

remition, so the administration of steroid can lowerer or stop, but if CyA is stoped,
usually will become relaps (dependen siklosporin)

II.7 Prognosis1,4
The majority of children with steroid-responsive nephrotic syndrome have
repeated relapses, which generally decrease in frequency as the child grows older.
Although there is no proven way to predict an individual child's course, those children
who respond to steroids rapidly and those who have no relapses during the first 6
mounth after diagnosis are likely to follow an infrequently relapsing course. It is
important to indicate to the family that the child with steroid-responsive nephrotic
syndrome is unlikely to develop chronic kidney disease, that the disease is generally
not hereditary, and that the child (in the absence of prolonged cyclophosphamide
therapy) will remain fertile. To minimize the psychological effects of the condition, the
physician should emphasize that the child should be considered normal when in
remission and may have unrestricted diet and activity, without the need for urine
testing for protein.
Children with steroid-resistant nephrotic syndrome, most often caused by
FSGS, generally have a much poorer prognosis. These children develop progressive
renal insufficiency, ultimately leading to end-stage renal disease requiring dialysis or
renal transplantation. Recurrent nephrotic syndrome develops in 3050% of transplant
recipients with FSGS. Plasmapheresis, plasma protein absorption onto protein Abased
columns, high-dose cyclosporine or tacrolimus, and ACE inhibitors may reduce
proteinuria in these patients.

II.7 Conclusion
Syndrome Nephrotic is a chronical disease that usually found in child, with
incident 2-4 cases in 100.000 children under 16 every year. Syndrome Nephrotic
Frequent Relapse is when its happen more than 2 times in 6 month or relapse more
than 4 times in 1 years, usually after initial treatments, 94 % cases get total remission
of the disease, and the rest 60-70% will become relapse 30-40% will become frequent
relapse.
Syndrome Nephrotic can be diagnose based on 4 specific symptomps that is
Masif Proteinuria or Proteinuria Nephrotic is when urine contain protein 40
mg/m2lpb/jam or > 50mg/kgBB/24hours, ratio albumin/kreatinin in urine > 2 mg/mg,
or dipstik 2 .hipoalbuminemia is when albumin serum < 2,5 g/dl. Normal albumin
serum in child is 3,6-4,4g/dl. In syndrom nephrotic water retension and swelling can be
seen if albumin under 2,5-3,0 g/dl , swelling, and hyperlipidemia patien with nephrotic
syndrome have hiperkolesterolemia ( cholesterol more than 200mg/dl.
Today there are 4 option for treatment in syndrom nephrotic frequent relaps,
and that is, Long Term Steroid, Levamisol, Sitostatik and Siklosporin (CyA)

DAFTAR PUSTAKA

1. Noer M Sjaifullah, 2011. Sindrom Nefrotik Idioatik. In Noer M Sjaifullah,

Soemyarso Ninik, Subandiyah Krisni, dkk. Kompendium Nefrologi Anak.
Jakarta : Badan Penerbit Ikatan Do; 142-163.
2. Lane C Jerome, Pediatric Nefrotik Syndrome. www.emedicine.com Last
Update: November 11, 2011..
3. Eddy A Allison, Symons M Jordan, 2003. Nephrotic Syndrome in Childhood.
Lancet. 2003. 362:629-639
4. Kliegman RM, et al. Nelson textbook of pediatrics. 18th ed Philadelphia :
Saunders Elsevier, 2007 : 1533-4.