CASE REPORT

NEPHROTIC SYNDROME

ARRANGED BY:
Agung Dwi Saputro 2011730118

PRESEPTOR :
dr Ihsanil Husna, Sp.PD

STASE ILMU PENYAKIT DALAM RSIJ CEMPAKA PUTIH

PROGRAM STUDI KEDOKTERAN
FAKULTAS KEDOKTERAN DAN KESEHATAN
UNIVERSITAS MUHAMMADIYAH JAKARTA
2016
1

KATA PENGANTAR

AssalamualaikumWr. Wb.
Alhamdulillah, Puji syukur penyusun panjatkan kehadiran ALLAH SWT atas
terselesaikannya tugas Laporan Kasus Sindrom Nefrotik.
Makalah refreshing

ini disusun dalam rangka untuk dapat lebih mendalami dan

memahami mengenai Sindrom Nefrotik . Tujuan khususnya adalah sebagai pemenuhan tugas
kepaniteraan Stase Ilmu Penyakit Dalam.
Semoga dengan adanya laporan kasus ini dapat menambah khasanah ilmu pengetahuan
dan berguna bagi penyusun maupun peserta didik lainnya.
Penyusun menyadari bahwa laporan kasus ini masih jauh dari kesempurnaan, oleh karena
itu penyusun sangat membutuhkan saran dan kritik untuk membangun laporan kasus yang lebih
baik di masa yang akan datang.
Terimakasih.
WassalamualaikumWr. Wb

Jakarta, April 2016

Penulis

BAB I
PATIENT STATUS

A. Patients identity
Name

: Ms. A

Age

: 27nd years old

Education

: Junior High school

Marital status

: Not Married

Occupation

: Shopkeeper

Religion

: Moslem

Date of admission

: 08 April 2016

MR number

: 00930613

B. Anamnesis
a. Chief complaint :
Patient complained of swelling in the legs since 3 weeks ago.
Another complaint :
Body weakness, dizziness, decreased appetite, and there are abrasions on both of her
ankles.
b. History of present illness
Patients come to the hospital with complaints of swelling in both legs since three
weeks ago. Swelling felt arise gradually, initially swelling occurs in the legs and face
when she wakes up, then at noon swelling in the face disappeared, but the swelling in
both legs still have not disappeared, on waking the patient feel a little breathless, then
3

after that breathless disappeared. The more swelling in her legs getting bigger. Patients
admitted when pressed with a finger in the second leg swelling will form a concave.
Patients also complained of decreased appetite, body felt weak and a little nauseous.
normal defecated, urination 3 times a day, a little frothy, no pain, sand during urinary
and blood during urination. There is no fever and no vomiting. There were abrasions on
both her ankles that arise 3 days ago.
c. History of past illness
Have history of same problem 3 months ago.
No history of Hypertension
No history of DM
No history of urinary or kidney disease
No history of asthma
No history of allergic
No history of hematologic disease
d. History of family
None of his family has same problem
No history of hypertension
No history of DM
No history of allergic
No history of hematologic disease
e. History of allergy
Patient has no allergy to food, drugs and weather.
f. History of treatment
When complaints swelling, which first appeared in December 2016, the patient
went to the hospital and given medication by a doctor, the patient drink it regularly
and diligently control to the hospital every two weeks, then the complaint is not there
anymore.
When the control back in the patient not to buy one of the drugs prescribed by the
doctor is ramipril. After 2 weeks, complaints swelling reappeared in March 2016 until
now.
g. Habits
Smoking habits
Drinking alcohol

: Denied
: Denied
4

Taking any medication or herbal : patient was consumed a herbal medicine to reduce
the swelling, but the swelling is not reduced
C. Physical Examination
- Generalis status : Mild ill
- Conciusness
: composmentis

Vital sign
-

blood pressure : 110/90 mmHg

Heart rate
: 86x/minute
Respiratory rate: 20x/minute
Temperature : 36.6 C

Body weight : 65 kg
Body height : 160 cm

D. General physical examination

Head
: normocephal, deformity (-)
Faces
: symmetric
Eyes
: anemic conjungtiva (-/-), icteric sclera (-/-)
Mouth
: the oral mucosa moist
Neck
: not palpable mass, lymphadenopathy (-)
Thorax
Inspection

: the movement of the chest symmetrical

Palpation

: same vocal fremitus in dextra and sinistra

Percussion

: Sonor

Auscultacion : vesicular breath sounds + / +, ronkhi - / -, wheezing - / -

Heart
Inspection

: ictus cordis not seen

Palpation

: ictus cordis palpable in ICS 5

Percussion

: margin left heart midclavicula ICS 5, margin the right heart linea right

parasternalis ICS 4
Auscultation : Regular 1st & 2nd heart sounds, murmur (-), gallop (-)
Abdomen
Inspection

: looked flat

Auscultation : bowel (+) sounds, 7x/minutes

Palpation

: pressure pain (-), ascites (-)

Percussion

: timpani, shifting dullness (-)

Extremities
Superior : Edema (- / -), warm akral(+ / +), RCT <2 seconds (+ / +)
Inferior : Edema (+/ +), warm akral (+ / +), RCT <2 seconds (+ / +)

Laboratory examination
12th Desember 2015
Examination
Kimia Klinik
Cholesterol
Protein total

Albumin
Ureum blood
Kreatinin blood
Urinalisis
Color
Purity
BJ

pH
Protein
Glukosa

Value

Units

Normal

H 696
L 3.9

g/dL
g/dL

13,2-17,3
6.0 8.0

L 1.3
39

g/dL
mg/dL

4.0 5.2
10-50

1.1

mg/dL

< 1.4

Yellow
Sligty turbid
1.047

7.0
Positive 2 (150mg/dl)
Negative

1.015 1.025

4.8 7.4
Negative
Negative
7

Keton

Positive 1 (5mg/dl)

Negative

Bilirubin
Urobilinogen

Negative
Normal

Negative
<1

Nitrit

Negative

Negative

Darah

Positive 1 (50/uL)

Negative

Leukosit

Negative

Negative

Sedimen

Eritosit

3-5

/lp

0-2

Leukosit

5-7

/lp

0-5

Epitel

15-20

/lp

5-15

Silinder

Negative

Kristal

Negative

Others

Bakteri (+)

Fungsi hati
SGOT

27

0-31

SGPT

26

0-35

Laboratory examination
11th Maret 2016
8

Examination
Profil Lemak
Cholesterol
Trigliserida

Value

Units

Normal

734
381

mg/dL
mg/dL

< 200
< 150

HDL
LDL

61
549

mg/dL
mg/dL

40 60
< 100

Ratio LDL/HDL

9.0

Cardio risk index

(CRI)
<3 : low risk
3.5 : moderat
>5 : high risk

Urinalisis
Color
Purity
BJ
pH
Glukosa
Protein
Keton
Bilirubin
Urobilinogen
Nitrit
Blood
Lekosit
Sedimen
Eritrosit
Lekosit
Epitel
Silinder
Kristal
Others

Yellow
Limpid
1,013
5,0
Negative
Negative
Negative
Negative
Normal
Negative
Negative
Negative
0-1
1-2
1-2
Smooth granule 1-2
Negative
Negative

1,015 1,025
4,8 7,4
Negative
Negative
Negative
Negative
<1
Negative
Negative
Negative
/lp
/lp
/lp
/lp

0-2
0-5
5-15

Resume :

Ms. A, 27nd years old, came to the hospital with complaints of swelling in both
legs since three weeks ago. Swelling felt arise gradually, initially swelling occurs in the
legs and face when she wakes up, then at noon swelling in the face disappeared, but the
swelling in both legs still have not disappeared, on waking the patient feel a little
breathless, then after that breathless disappeared. The more swelling in her legs getting
bigger. Patients admitted when pressed with a finger in the second leg swelling will form
a concave. Patients also complained of decreased appetite, body felt weak and a little
nauseous. urination 3 times a day, a little frothy. History of past illness : have history of
same problem 3 months ago. Physical examination : Blood pressure: 110/90 mmHg,
Heart rate: 86x/minute, Respiratory rate: 20x/minute, Temperature : 36.6 C, Extremities
Inferior : Edema (+/ +). Laboratory examination:
12th Desember 2015

Total Cholesterol : 696 mg/dL (H), Total Protein : 3.9 g/dL (L), Albumin : 1.3 d/dL (L).
Urinalisis ; BJ : 1,047, Protein : Positive 2 (150mg/dl), Keton : Positive 1 (5mg/dl),
blood : Positive 1 (50/uL). Sediment ; Eritrosit : 3-5, Lekosit : 5-7, Bakteri Positive
11th Maret 2016
Cholesterol : 734 mg/dL, Triglisrida : 381 mg/dL, LDL : 549 mg/dL. Urinalisis ;
protein : Positive 3 (500mg/dl),

Problem List:

Edema
10

Proteinuria
Hipoalbuminema
Hiperkolesterolemia

Assessment :
Nephrotic Syndrome

Edema
Proteinuria
Hipoalbuminema
Hiperkolesterolemia

Differential Diagnosis :
1. Glomerulonefritis akut

S : Patient complaints of swelling in both legs since three weeks ago, Patients also
complained of decreased appetite, body felt weak and a little nauseous. urination 3 times
a day, a little frothy.
O: Blood pressure: 110/90 mmHg, Heart rate: 86x/minute, Respiratory rate: 20x/minute,
Temperature : 36.6 C, Extremities Inferior : Edema (+/ +).
Lab examination: 12th Desember 2015

11

Total Cholesterol : 696 mg/dL (H), Total Protein : 3.9 g/dL (L), Albumin : 1.3 d/dL (L).
Urinalisis ; BJ : 1,047, Protein : Positive 2 (150mg/dl), Keton : Positive 1 (5mg/dl),
blood : Positive 1 (50/uL). Sediment ; Eritrosit : 3-5, Lekosit : 5-7, Bakteri Positive
11th Maret 2016
Cholesterol : 734 mg/dL, Triglisrida : 381 mg/dL, LDL : 549 mg/dL. Urinalisis ;
protein : Positive 3 (500mg/dl),
A: Nephrotik Syndrome
P: Laboratory :
- Full peripheral blood
- Urinalisis
Renal Biopsy
Therapy :
-

Bedrest until until the swelling disappears

Protein intake is restricted from 0.8 to 1 g / kg / day
Diet low in salt 2 grams / day and low-fat
Prednisone 5 mg 3 x 8 tab
Furosemid 40 mg/day

BAB I
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LITERATURE REVIEW

A. Anatomy of Renal
Renal is bean-shaped organ located on both sides of the vertebral column. In
general, lower than the right kidney left kidney because of the liver and is closer to the
midline of the body. It is as high as XII thoracic vertebra, while the upper pole of the left
kidney is located as high as thoracic vertebra XI.
Renal parenchyma composed of two special areas: the renal cortex which is
located on the outside and looks granular, as well as the inner regions that form a triangle
striped (renal pyramids), which are collectively referred to as the renal medulla. Each
pyramid separated by columns Bertini. Basic pyramid was closed by the cortex, while the
peak (marginal papillae) forms of papillary ducts of Bellini enter into Calix minor. Some
minor Calix Calix unified into a major, which then coalesced into the renal pelvis and
ureter renal pelvis is out.
Each kidney is composed of about one million functional units (the smallest unit
that is capable of forming urine) microscopic called nephrons. Each nephron consists of
the Bowman's capsule and capillary glomerolus, proximal convoluted tubule, loop of
Henle, distal convoluted tubules which empties into the collecting tubules. Each kidney
obtain blood supply from the renal artery. On entering the kidney, renal artery branchingcabgang eventually become the afferent arterioles with each of these vessels provide
blood supply a nephron.

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A. Physiology of glomerulus
The glomerulus is a dominant part in the vascular component of the nephron. The
most important function of glomerolus is formed ultrafiltrate which can fit into the
tubules due to capillary hydrostatic pressure greater than the hydrostatic pressure
intrakapiler and colloid osmotic pressure.
The layers of the membrane to function as molecular sieves glomerolus holding
red blood cells and plasma proteins, but skip H2O and other solute molecular size is quite
small. Although plasma proteins can not be filtered because it can not pass through the
pores from above, the pore itself is large enough to pass the plasma protein albumin
which is the smallest. However, the basement membrane glycoprotein glomerolus highly
negatively charged so that it will refuse albumin and other plasma proteins also
negatively charged. Most disease characterized by the presence of albuminuria, is caused
by disturbances in the membrane negative charge glomerolus, which causes the
membranes more permeable to albumin although the pore size has not changed.
Under normal circumstances, the renals receive 20% to 25% of cardiac output or
1200-1250 mL / min (RBF). When considered normal hematocrit of 45%, then the renal

14

plasma flow (RPF) is equal to 660 mL / min. Under normal circumstances, about 20% of
plasma that went into glomerolus filtrated with a net filtration pressure 10 mmHg,
produces filtration rate glomerolus average (GFR) of 125 mL / min. As the filtrate flows
through the tubules, added or taken various substances from the filtrate, so that eventually
only about 1.5 L / day is excreted as urine.
B. Nephrotic Syndrome
Nephrotic syndrome is a set of clinical manifestations characterized by massive
proteinuria (greater than 3.5 g / 1.73 m2 body surface area per day), hypoalbuminemia
(less than 3.5 g / dl), edema, hyperlipidemia and lipiduria
C. Epidemiology
Incidence may affect all ages but most (74%) was found at the age of 2-7 years. The ratio
of male: female = 2: 1, whereas in adolescence and adulthood this ratio ranges from 1: 1.
Usually 1 of 4 patients with nephrotic syndrome are patients with age> 60 years. But in
exact incidence and prevalence of nephrotic syndrome in geriatrics is not known because
often misdiagnosed.

D. Etiology
The etiology of nephrotic syndrome can be divided into :
1. Primary glomerulonephritis with an unknown cause (idiopathic) with a wide
variety of histopathological abnormalities, include:
- minimal lesion glomerulonephritis
- focal glomerulosclerosis
- membranous glomerulonephritis
- glomerulonephritis membranoproliferative
- the other proliferative glomerulonephritis
2. Glomerulonephritis secondary to:
- infections, such as HIV infection, hapatitis virus B and C, syphilis, malaria,
Schistosomal, tuberculosis, and leprosy.

15

Malignancy, such as adenocarcinoma of the lung, breast, colon, Hodgkin's

lymphoma, multiple myeloma, and renal carcinoma.

connective tissue diseases, such as systemic lupus erythematosus, rheumatoid

arthritis, MCTD (mixed connective tissue disease)

The effects of drugs and toxins, such as non-steroidal anti-inflammatory drugs,

gold preparations, penicillamine, probenecid, mercury, captopril, and heroin.

Other, including diabetes mellitus, amyloidosis, pre-eclampsia, chronic allograft
rejection, vesicoureteric reflux, or a bee sting.

Currently immune disorders mediated by T cells suspected to be the cause of nephrotic

syndrome. This is supported by evidence of an increased concentration of serum
neopterin and the ratio of neopterin / creatinine urine and increased activation of T cells
in the peripheral blood of patients with nephrotic syndrome which reflect abnormalities T
cell-mediated immunity
E. Clinical Manifestations
Proteinuria
Proteinuria due to increased capillary permeability to proteins from damage
glomerolus. In nomal circumstances basement membrane barrier glomerolus have a
mechanism to prevent the leakage of protein. The first barrier mechanism based on
molecular size (size barrier) and the second by an electric charge (charge barrier). In
nephrotic syndrome, both mechanisms involved subject. In addition, the configuration of
the protein molecules also determine whether or not the protein passes through the
basement membrane glomerolus.
The degree of proteinuria is not directly related to the severity of glomerular
damage. At minimal lesion nephropathy, proteinuria caused mainly by the loss of charge
selectivity while in membranous nephropathy is caused mainly by the loss of size
selectivity.
16

Hypoalbuminemia
Plasma albumin concentration is determined by the intake of protein, albumin
synthesis of the liver, and loss of albumin in the urine. In nephrotic syndrome,
hypoalbuminemia caused by the loss of albumin in urine and increased catabolism of
albumin in the kidney. Protein synthesis in the liver normally increases as compensation
effort (but not sufficient to replace the loss of albumin in the urine), but may be normal or

decreased .
Edema
Edema in nephrotic syndrome can be explained with the theory of underfill and
overfill. Underfill theory explains that hypoalbuminemia causes a decrease in plasma
oncotic pressure so that the fluid shift from the intravascular to the interstitial tissue and
edema. As a result of a decrease in plasma oncotic pressure and plasma fluid shifts occur
hypovolemia. Kidneys compensate by increasing sodium and water retention. The
compensation mechanism will improve intravascular volume, but also exacerbates the
occurrence of hypoalbuminemia so edema increasingly continue.
However, in the early 1940s, especially in the last two decades, many studies
support the concept that is contrary to the theory of underfill. Although some patients
with nephrotic syndrome minimal lesions have plasma volume endah, many patients with
nephrotic syndrome do not have clinical manifestations as theory underfill (decreased
plasma volume, increased sodium-retaining hormones, etc.)
Other researchers theoriy of overfill. This theory explains that renal retention of
sodium is a major defect. Retention of sodium by the kidneys, causing increased
extravascular fluid, causing edema. Glomerolus filtration rate decreased due to damage to
the kidneys will increase sodium retention and edema.
Some explanations trying to combine these two theories, for example, noted that
the edema formation is a dynamic process. It was found that the plasma volume

17

decreased significantly during the formation of edema and increased during phase

diuresis.
Hyperlipidemia
Cholesterol levels generally increased, while triglycerides varies from normal to
slightly elevated. Increased due to increased LDL cholesterol levels. High triglyceride
levels are associated with increased VLDL. Also found an increase in IDL (intermediate
density lipoprotein) and lipoprotein (Lp) a, whereas HDL tend to be normal or low .
This situation is due to increased lipid synthesis in the liver and decreased
catabolism in peripheral (decrease lipoprotein, VLDL, intermediate density lipoproteins
and chylomicrons from the blood). Increased lipoprotein lipid synthesis is stimulated by a
decrease in serum albumin and decrease in oncotic pressure.

F. Diagnosis
1. Anamnesis
It should be noted the problem of drug use, the possibility of various infections, and
the history of other systemic diseases.
2. Physical examination
There anasarca edema. Not infrequently eyes closed due to edema of the eyelids.
3. Supporting investigation
- Examination of urine, including urine protein, urinalysis, hamaturia, dipstick
urine specific gravity of urine and sediment examination. Volume is usually less
-

than 400 ml / 24 hours.

Blood tests, including serum albumin, serum cholesterol, triglycerides,
hemoglobin, hematocrit, erythrocyte sedimentation rate (ESR), and serum

electrolytes.
Serology and renal biopsy is often needed to confirm the diagnosis and rule out
possible causes of nephrotic syndrome secondary. Serology is often not a lot of
information and it is expensive because it should only be done by a strong
indication.

18

The diagnosis of nephrotic syndrome is made on clinical and laboratory examinations

in the form of massive proteinuria (> 3.5 g / 1.73 m2 body surface area / day),
hypoalbuminemia (<3 g / dL), edema, hyperlipidemia, and hypercoagulability lipiduria.
Additional examinations such as venography is required to make the diagnosis of venous
thrombosis which can occur as a result of hypercoagulability. In primary nephrotic
syndrome to determine the type of renal histopathologic abnormalities that determine
prognosis and response to therapy, needed a kidney biopsy.

G. Treatment
Treatment of nephrotic syndrome consists of a specific treatment against basic
diseases and non-specific treatment to reduce proteinuria, edema control, and treat
complications. Some definitions / limitations used in SN :
o Remission: negative or trace proteinuria (proteinuria <4 mg / m2 LPB / h) 3
consecutive days in one week
o Relapse: 2+ proteinuria (proteinuria 40 mg / m2 LPB / h) 3 consecutive days
in the first week 3
o Relapse rare: relapses occurred less than two times in the first 6 months after the
initial response to or less than 4 times per year of observation
o Relapses often (frequent relapses): relapses occurred 2 times in the first 6
months after the initial response or 4 times in a period of 1 year
o Dependent steroids relapses occurred during steroid doses lowered or within 14
days after treatment was stopped, and this occurs two times in a row
o Steroid Resistant: no remission in the treatment of full-dose prednisone (full dose)
2 mg / kg / day for 4 weeks.
a. Non- Pharmacology

19

Diet for patients with nephrotic syndrome is 35 cal / kg / day, consisting mostly of
carbohydrates. Proteinuria may improve hypoalbuminemia control and reduce the
risk of complications caused. Normal protein diet recommended 0.8-1.0 g / kg /
day. In patients with dietary protein 0.6 g / kg / day plus the number of grams of
protein according to the number Proteinuri result Proteinuri reduced, increased
blood levels of albumin and fibrinogen levels decreased. To reduce edema given a
low salt diet (1-2 grams of sodium / day) along with diuretics and bedrest.

b. Pharmacology
Corticosteroids
Minimal lesion nephropathy and membranous nephropathy are two disorders that
respond well to steroid therapy. Other researchers have found that focal segmental
glomerulosclerosis up to 40% of patients respond well to steroids with a complete
remission. In most patients with idiopathic membranous nephropathy,
symptomatic therapy with better kidney function for a longer period and can heal
spontaneously. Hence their use of glucocorticoids and immunosuppressants at
nephropathy type is not recommended.

20

Figure 1. The initial treatment with corticosteroids

Information:
- The full dose prednisone (full dose) 60 mg / mLPB / day (2mg /
kg / day) divided into 3 doses given daily for 4 weeks, followed by
prednisone 40 mg / mLPB / day (2/3 full dose), can given
intermittently (3 consecutive days in the first week) or alternating
-

(every other day) for 4 weeks.

When remission occurs within the first 4 weeks, then intermittent
prednisone / alternating 40 mg / mLPB / day administered for 4
weeks. When remission does not occur in the first 4 weeks, then
the patient is diagnosed as a steroid-resistant nephrotic syndrome.

Figure 2. Treatment of nephrotic syndrome relapse

Information:

21

Prednisone full dose every day until remission (maximum of 4

weeks) followed by intermittent prednisone / alternating 40 mg /

mLPB / day for 4 weeks.

When you get a full dose treatment for 4 weeks did not also occur
revision, the patient was diagnosed as a steroid-resistant SN and
should be given other immunosuppressive therapy

Figure 3. Treatment of nephrotic syndrome relapsed frequently

Information:
- Full dose prednisone daily until remission (maximum of 4 weeks)
followed by intermittent prednisone / alternating 40 mg / mLPB / day and
immunosuppressive / oral cytostatic (cyclophosphamide 2-3 mg / kg / day)
dose for 8 weeks
22

Monitoring of Hb, leukocytes, platelets every week

Leukocytes <3000 / ml CPA stopped first
Leukocytes> 5000 / ml CPA awarded again
Figure 4. The treatment of steroid dependent nephrotic syndrome
Information:
- Prednisone full dose every day until remission (maximum 4 weeks),
followed by cyclophosphamide puls with a dose of 500-750 mg / mLPB
given by infusion once a month for 6 consecutive months and intermittent
prednisone / alternating 40 mg / mLPB for 12 Sunday. Then tapering off
prednisone at a dose of 1 mg / kg / day for 1 month, followed by 0.5 mg /
kg / day for 1 month (long tapering off: 2 months)

23

Or, Prednisone full dose every day until remission (maximum of 4

weeks) , followed by oral cyclophosphamide 2-3 mg / kg / day dose for 12
weeks and alternating prednisone 40 mg / mLPB.hari for 12 weeks. Then
tapering off prednisone at a dose of 1 mg / kg / day for 1 month, followed
by 0.5 mg / kg / day for 1 month (long tapering off: 2 months)

Figure 5. Treatment of steroid-resistant nephrotic syndrome.

Information:
-

oral cytostatic: cyclophosphamide 2-3 mg / kg / day dose for 3-6 months

Prednisone dose of 40 mg / mLPB / alternating days during

administration of oral cyclophosphamide.

Then prednison in tapering-off with a dose of 1 mg / kg / day for 1 month,
followed by 0.5 mg / kg / day for 1 month (long tapering off 2 months).

Or, Cyclophosphamide puls with a dose of 500-750 mg / mLPB

dibertikan via intravenous infusion once a month for six months, may be
continued depending on the patient's condition.

24

Prednisone alternating doses of 40 mg / mLPB / day during

administration of cyclophosphamide puls (5 months). Then tapering off
prednisone at a dose of 1 mg / kg / day for 1 month, followed by 0.5 mg /
kg / day for 1 month (long tapering off 2 months).

ACE inhibitors and angiotensin receptor blockers

In patients who are not responsive to corticosteroids, to reduce Proteinuri
used symptomatic therapy with angiotensin converting enzyme inhibitors (ACEI),
for example, captopril or enalapril low doses, and the dose is increased after 2
weeks, or anti-inflammatory drugs non-steroidal (NSAIDs), such as indomethacin
3x50 mg. Angiotensin converting enzyme inhibitors reduce glomerular
ultrafiltration protein with intrakapiler glomerular pressure and improve
glomerular size selective barrier. Antiproteinurik effect of this drug lasts longer
(approximately two months after the drug is stopped). Angiotensin receptor
blockers (ARBs) were also able to improve Proteinuri because it inhibits
inflammation and interstitial fibrosis, inhibiting the release of cytokines, growth
factors, adhesion molecules occupational local angiotensin II in the kidneys. The
combination of ACEI and ARB reported antiproteinuri greater effect on primary
glomerulonephritis than just the use of ACEI or ARB alone.

NSAIDs
Non-steroidal anti-inflammatory drugs (NSAIDs) may be used in patients
with membranous nephropathy and focal segmental glomerulosclerosis to
decrease the synthesis of prostaglandins. It causes renal vasoconstriction,
decreased glomerular capillary pressure, filtration surface area and reduces
proteinuria to 75%. Besides NSAIDs can reduce the levels of fibrinogen, fibrin-

25

related antigenic and prevent platelet aggregation. However, please note that
NSAIDs cause a progressive decline in kidney function in some patients. This
drug should not be given if creatinine clearance <50 ml / min.

Cyclophosphamide and Chlorambucil

In patients with frequent relapses with corticosteroids or corticosteroid
resistant to other therapies may be used with cyclophosphamide or chlorambucil.
Cyclophosphamide give remission longer than corticosteroids (75% over 2 years)
at a dose of 2-3 mg / kg / day for 8 weeks. The side effects of cyclophosphamide
is bone marrow depression, infections, alopecia, hemorrhagic cystitis and
infertility when given over 6 months. Chlorambucil given at a dose of 0.1-0.2
mg / kg / day for 8 weeks. The side effects of chlorambucil is azoospermia and
agranulocytosis.
In idiopathic membranous nephropathy, a combination of methylprednisolone and
chlorambucil induce remission for 6 months earlier and can retain kidney function
compared with methylprednisolone alone, but these differences diminish with
time (in four years this difference was not significant anymore). The regimen used
is methylprednisolone 1 g / day intravenously three days, then 0.4 mg / kg / day
orally for 27 days followed by chlorambucil 0.2 mg / kg / day of one month
alternately.

Another

alternative

therapy

membranous

nephropathy

is

cyclophosphamide 2 mg / kg / day plus 30 mg prednisolone every 2 days for

several months (up to 6 months).

Cyclosporine A
Cyclosporine A may be tried in patients who relapsed after being given
cyclophosphamide or to extend the period of remission after corticosteroid
administration. A dose of 3-5 mg / kg / day for 6 months to 1 year (after 6 months
26

the dose reduced by 25% every 2 months). Cyclosporine A may also be used in
combination with prednisolone at the failed cases of nephrotic syndrome in
combination with other therapies. The side effects of this drug are gingival
hyperplasia, hypertrichosis, hiperurisemi, hypertension and nefrotoksis

Diuretics
To reduce edema diuretics (furosemide 40 mg / day or class thiazides)
with or without combination with potassium-sparing diuretics (spironolactone).
When resistant with oral furosemide can be combined with thiazides, metalazon,
or acetazolamide.
In patients with nephrotic syndrome can occur resistance to diuretics (furosemide
500 mg and 200 mg spironolactone). Resistance to this diuretic is multifactorial.
Suspected hipoalbuminemi cause a reduction in drug transport to the workplace,
while binding by urinary protein is not the main mechanism of this resistance. In
such patients may be given intravenous salt-poor human albumin. It said this
therapy may increase plasma volume, increases in glomerular filtration rate, urine
flow, and sodium excretion. However, albumin infusion is still doubt its
effectiveness because albumin rapidly excreted through urine, but it can increase
blood pressure and even pulmonary edema in patients hipervolemi.

Anticoagulants
Risk of thromboembolism is increased in nephrotic syndrome and the need
to get treatment. Although the long-term administration is still controversial, but
in one study proved beneficial. To prevent complications hypercoagulable namely
thromboembolism occurring in approximately 20% of cases of nephrotic
syndrome (most often in membranous nephropathy), used dipyridamole (3 x 75
mg) or aspirin (100 mg / day) as an anti-platelet aggregation and deposition of
27

fibrin / thrombus. Besides these drugs can significantly reduce the decline in
kidney function and the occurrence of end stage renal failure. This therapy is
given for patients experiencing Proteinuri nephrotic albumin <2 g / dl or both. In
case of thromboembolism, should be given intravenous heparin / infusion for 5
days, followed by oral warfarin administration up to 3 months or after cure
nephrotic syndrome. Administration of heparin with monitoring activated partial
thromboplastin time (APTT) 1.5-2.5 times control, while the effect of warfarin
was evaluated by prothrombin time (PT) is usually expressed as the International
Normalized Ratio (INR) of 2-3 times normal.
Other diseases that may occur include hypertension, hypovolemic shock,
acute renal failure, chronic renal failure (after 5-15 years). Handling the same as
the handling in general. In case of chronic renal failure, in addition to
hemodialysis, renal transplantation can be performed. In patients with focal
segmental glomerulo-sclerosis who underwent a kidney transplant, 15% -55%
will be back to nephrotic syndrome. Recurrence may be caused by factors plasma
(circulating

factor)

or

factors

that

increase

glomerular

permeability.

Imunoadsorpsi plasma protein A decrease urinary protein excretion in patients

with nephrotic syndrome due to focal segmental glomerulosclerosis, membranous
nephropathy

and

nephrotic

syndrome

secondary

to

diabetes

mellitus.

Imunoadsorpsi allegedly releasing plasma factors that influence hemodynamic or

increase glomerular permeability.
H. Complication
1. Infection
28

Hipogamaglobulinemi, particularly immunoglobulin G (IgG), together with

factors B causes nephrotic syndrome sufferers are highly susceptible to infection. Much
more use of corticosteroids which we know as immunosuppression, susceptibility to
infection is greater. The most common infection is peritonitis, sepsis and cellulitis. The
main cause is Streptococcus pneumonia. Sometimes it can be caused by Gram-negative
bacteria (5%), such as E.coli, Klebsialla, H.influenza1.
2. Thrombosis
Thrombosis can occur in veins and arteries, especially the large vein in the liver,
pelvis, renal, mesenteric and pulmonary. The causes of thrombosis are:
- Hypovolemia: hemokonsentrasi and hyperviscosity
- Thrombocytosis
- Elevation concentrations of plasma coagulation factors: factor V, VII, VIII, X and
fibrinogen
- Decrease plasma concentration of antithrombin III
- Elevation platelet agregasi1

3. Acute Renal Failure

The cause of ARF is not known for sure, but there is evidence involving
hypovolemia and renal ischemia resulting in acute tubular necrosis and interstitial edema
ensued and elevation of pressure occurs in proximal tubules with consequent reduction in
filtration rate glomelurus.
H. Prognosis
Mortality and the prognosis of patients with nephrotic syndrome varies by etiology,
severe, extensive damage to the kidneys, the child's age, the underlying condition, and
response to treatment

29

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Sherwood, L. 2014. Fisiologi Manusia dari Sel ke Sistem. Edisi 2, Alih Bahasa: Brahm

U. Pendit. Jakarta, Penerbit Buku Kedokteran EGC. pp: 463-475.

Wilson, L. M. 2006. Anatomi dan Fisiologi Ginjal dan Saluran Kemih. Dalam:
Patofisiologi Konsep Klinis Proses-Proses Penyakit. Volume 2. Ed: Sylvia A. Price dan
Lorraine M. Wilson. Alih Bahasa: Brahm U. Pendit, dkk. Jakarta, Penerbit Buku

Kedokteran EGC. pp: 867-875.

Kidney Disease: Improving Global Outcomes (KDIGO) Glomerulonephritis Work
Group. KDIGO

Clinical Practice Guideline for Glomerulonephritis. Kidney inter.

2012;Suppl.2: 139 274

National Kidney and Urologic Disease Information Clearinghouse. Nephrotic Syndrome

in adults. National Institute of Health; 2010.

Hull RP., Goldsmith DJ., Nephrotic syndrome in adults. BMJ, 2011;336:1185-9

Siddall EC, Radhakrishnan J. The pathophysiology of edema formation in the nephrotic

syndrome. Kidney Int. 2012. 82:635642

Prodjosudjadi W., SindromNefrotik. Buku Ajar Ilmu Penyakit Dalam Ed VII. 2015; 2082
2088.

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