case nephrotic syndrome

49
CASE REPORT Non-Infection Unit NEPHROTIC SYNDROME Presentator : Edwin B. Saragih Sejahtera Surbakti Supervisor : dr. Yazid Dimyati, Sp.A Date of presentation : 12 th April 2010 I. INTRODUCTION Definition NS is a common chronic disorder, characterized by alterations of permselectivity at the glomerular capillary wall, resulting in its inability to restrict the urinary loss of protein. Nephrotic range proteinuria is defined as proteinuria exceeding 1000 mg/m² per day or spot (random) urinary protein- tocreatinine ratio exceeding 2 mg/ml. The proteinuria in childhood nephrotic syndrome is relatively selective, constituted primarily by albumin. 1 The diagnosis of nephrotic syndrome requires the presence of edema, severe proteinuria (> 40 mg/m2/h, or a protein:creatinine ratio > 2.0), hypoalbuminemia (< 2.5 g/dl), and hyperlipidemia. 2 Table I. Common definition to define the course of nephrotic syndrome. 3 Nephrotic Oedema ; nephrotic range proteinuria (> 1

Upload: tera-surbakti

Post on 08-Nov-2014

58 views

Category:

Documents


6 download

DESCRIPTION

laporan kasus sindroma nefrotik

TRANSCRIPT

Page 1: Case Nephrotic Syndrome

CASE REPORT

Non-Infection Unit

NEPHROTIC SYNDROME

Presentator : Edwin B. Saragih

Sejahtera Surbakti

Supervisor : dr. Yazid Dimyati, Sp.A

Date of presentation : 12th April 2010

I. INTRODUCTION

Definition

NS is a common chronic disorder, characterized by alterations of permselectivity at the

glomerular capillary wall, resulting in its inability to restrict the urinary loss of protein.

Nephrotic range proteinuria is defined as proteinuria exceeding 1000 mg/m² per day or spot

(random) urinary protein-tocreatinine ratio exceeding 2 mg/ml. The proteinuria in childhood

nephrotic syndrome is relatively selective, constituted primarily by albumin.1 The diagnosis of

nephrotic syndrome requires the presence of edema, severe proteinuria (> 40 mg/m2/h, or a

protein:creatinine ratio > 2.0), hypoalbuminemia (< 2.5 g/dl), and hyperlipidemia.2

Table I. Common definition to define the course of nephrotic syndrome.3

Nephrotic syndrome Oedema ; nephrotic range proteinuria (> 40mg/m2/h on timed

sample, spot albumin to creatine ration > 2mg/mg ) ;

hypoalbuminaemia (<2.5g/dl)

Relapse Urinary protein excretion > 40mg/mg/m2/h ; ≥3+ by dipstick

for 3 consecutive days

Remission Urinary protein excretion < 4 mg/m2/h ; nil or trace by dipstick

on spot sample for 3 consecutive days

Frequent relapses Two or more relapses in 6 months of initial response ; 4 or

more relapses in any 12 month period

Steroid dependence Occurrence of 2 consecutive relapses during steroid therapy or

within 2 weeks of its cessation

1

Page 2: Case Nephrotic Syndrome

Steroid resistance Failure to achieve remission after 4 weeks of daily therapy

with oral prednisolone at a dose of 2mg/kg/day

Epidemiology

The annual incidence of nephrotic syndrome has been estimated to range from 2 to 7 new

cases per 100 000 children, and the prevalence is about 16 cases per 100 000 children, or 1 in

6000 children.2 In Jakarta Indonesia, Wila Wirya reported 6 new cases per 100.000 children

younger than 14 years old, making it a relatively common major disease in pediatrics.4 In

younger children, boys are about twice as likely to develop nephrotic syndrome as girls, but this

imbalance disappears by adolescence such that the incidence in adolescents and adults is equal

among males and females. The histologic lesion associated with nephrotic syndrome also differs

between genders. In a large multicenter study of childhood nephrotic syndrome carried out by

the International Study of Kidney Disease in Children (ISKDC), females represented 39.9% of

children with minimal change nephrotic syndrome (MCNS) and 30.6% of those with focal

segmental glomerulosclerosis (FSGS). In marked contrast, females represented 64.1% of those

with membranoproliferative glomerulonephritis (MPGN).2

Familial occurrence of idiopathic nephrotic syndrome is also a well-recognized

phenomenon and the disorder has been reported in identical twins. In a report of 1877 children

with idiopathic nephrotic syndrome in Europe, 3.3% of children were found to have affected

family members, most often siblings. The disorder tended to occur in the siblings at the same

ages, and with similar biopsy findings and clinical outcomes. At least one locus for SSNS has

been mapped to chromosome 1q25, which is near, but distinct from, the NPHS2 gene locus

encoding the podocyte protein podocin.2

The peak age of presentation of nephrotic syndrome is 2 years, and 70–80% of cases of

nephrotic syndrome occur in children less than 6 years of age. Age of onset may also be

predictive of the underlying histologic lesion causing nephrotic syndrome. MCNS is seen in 80%

of children diagnosed with nephrotic syndrome before 6 years of age. In comparison, only 50%

of those with FSGS, and 2.6% of those with MPGN present before 6 years.12 In the same study,

the median age at presentation of MCNS was 3 years, compared with 6 years for FSGS, and 10

years for MPGN. These findings suggest that the likelihood of having MCNS decreases with

2

Page 3: Case Nephrotic Syndrome

increasing age at onset, whereas the likelihood for having the less-favorable diagnoses of FSGS

or MPGN increases.2

Failure to respond to steroid treatment (SRNS) has an important ramification for the risk

of developing progressive renal failure later in life. Within 5 years of diagnosis, 21% of children

with FSGS developed ESRD and another 23% developed CKD. Thus, in a child diagnosed as

having FSGS, the risk of developing CKD or ESRD within 5 years is almost 50%.2

Although the overall prevalence of nephrotic syndrome has remained relatively stable

over the last 20 years, a dramatic increase in the incidence of FSGS and decrease in the incidence

of MCNS has been reported. The reported increased incidence of FSGS needs, however, to be

interpreted cautiously, since renal biopsies are generally obtained only in a preselected group of

children with atypical presentations, or those exhibiting steroid resistance. It is possible that an

increased incidence of FSGS merely represents the fact that a larger percentage of children with

SRNS undergo renal biopsies in the present era. This is in marked contrast to an early ISKDC

study where all children underwent a renal biopsy at the time of presentation, prior to the

institution of treatment. Because renal biopsies are no longer routinely performed in steroid-

responsive patients, it is unlikely that the ISKDC observations can be replicated.2

Pathogenesis

One of the kidney’s most important functions is filtration of the blood by glomeruli,

allowing excretion of fluid and waste products, while retaining all blood cells and the majority of

blood proteins within the bloodstream. Each glomerulus is composed of numerous capillaries

which have evolved to permit ultrafiltration of the fluid that eventually forms urine. The

capillary walls are composed of an inner endothelial cell cytoplasm, with pores known as

‘fenestrations’, the glomerular basement membrane (GBM), and outer glomerular epithelial cells

(podocytes) whose distal ‘foot’ processes are attached to the GBM. Under normal conditions,

molecules greater than 42 Å in diameter, or more than 200 kDa, are unable to cross the filtration

barrier.2

The role played by podocytes in glomerular function in renal disease and pathogenesis is

evolving. Several morphologic changes have been reported in podocytes in nephrotic syndrome.

These changes include cell swelling; retraction and effacement of the podocyte foot processes,

resulting in the formation of a diffuse cytoplasmic sheet along the GBM; vacuole formation;

3

Page 4: Case Nephrotic Syndrome

occurrence of occluding junctions with displacement of slit diaphragms; and detachment of the

podocyte from the GBM.8–10,12,20 These structural alterations in podocytes, often associated

with detachment from the underlying GBM have been shown to result in proteinuria.2

Proteinuria occurs due to increased glomerular permeability of proteins resulting from the

loss of fixed negative charges and inability of the proximal tubules to reabsorb all of the filtered

proteins. Mean glomerular pore size or density may be altered due to lack of electrostatic

interaction between glomerular capillaries and polyionic plasma proteins, such as albumin. [25] In

addition, the type of proteinuria appears to correlate with response to therapy. Patients with

highly selective proteinuria respond better to corticosteroids and are more likely to have minimal

change disease than those with nonselective proteinuria. In highly selective proteinuria, only

intermediate-sized proteins (< 100 kD), such as albumin and transferrin, leak through the

glomerulus; in nonselective proteinuria, a large range of proteins leak through the glomerulus. [9]

In the modern era, use of protein selectivity to predict response to therapy has been replaced by

observation of the response to corticosteroids in favorable populations, and renal biopsy and

electron microscope results in patients in less favorable subgroups.3

Clinical Features

The idiopathic nephrotic syndrome is more common in males than in females (2:1) and

most commonly appears between the ages of 2 and 6 yr. It has been reported as early as 6 mo of

age and throughout adulthood. The initial episode and subsequent relapses may follow minor

infections and, occasionally, reactions to insect bites, bee stings, or poison ivy. Children usually

present with mild edema, which is initially noted around the eyes and in the lower extremities.

Nephrotic syndrome may initially be misdiagnosed as an allergic disorder because of the

periorbital swelling that decreases throughout the day. With time, the edema becomes

generalized, with the development of ascites, pleural effusions, and genital edema. Anorexia,

irritability, abdominal pain, and diarrhea are common; hypertension and gross hematuria are

uncommon.5

a. Edema

Edema is the most profound symptom of the nephrotic syndrome. It may present in a

mild localized form or in a generalized fashion and also may be mobile, presenting as puffiness

of the eyelids that is worsened with lying down, especially in the morning upon awakening, and

4

Page 5: Case Nephrotic Syndrome

as lower extremity edema that is worse at the end of the day. Moreover, excess fluid may collect

internally and present as pleural or pericardial effusions and ascites. Subungual edema may

manifest as parallel white lines in the fingernail beds. In cases of severe edema, the patient may

have generalized swelling, or anasarca, which is usually pitting and worse in dependent areas of

the body such as the genitalia and lower extremities. Because of the excess body fluid, patients

generally report unexplained weight gain and fatigue. Edema of the nephrotic syndrome may be

a manifestation of hypoalbuminemia with increased water and salt retention, or it may be due to

a primary defect within the collecting tubule of the nephron that leads to uncontrolled water and

salt retention. Regardless of the pathophysiology of edema, it is important to consider other

conditions that present with edema and/or hypoalbuminemia.(Mira)

b. Hypoalbuminemia

In nephrotic syndrome, the serum concentration of albumin often is significantly low as it

is one of the smaller proteins and therefore is easily lost in the urine. When albumin appears in

the urine, the patient may complain of urine frothiness. Albumin synthesis is upregulated by the

liver because of the urinary losses, but the body’s compensatory mechanisms to maintain

albumin homeostasis are insufficient as urinary losses exceed hepatic production. (Mira)

c. Hyperlipidemia

The other characteristic findings in the nephrotic syndrome may present clinically as

medical complications. The hyperlipidemia associated with the syndrome is primarily due to

abnormal lipoprotein homeostasis that results in an increase in synthesis and decrease in

catabolism. Patients usually have elevations of total plasma cholesterol, triglyceride, very-low-

density lipoprotein (VLDL), and low-density lipoprotein (LDL). Dyslipidemia increases the risk

of atherosclerosis and cardiovascular disease in patients with the nephrotic syndrome, and

patients may present with complications of these diseases.6

d. Hypercoagulability

The hypercoagulable state associated with the nephrotic syndrome is caused by an

increased urinary loss of antithrombin III, altered activity and levels of proteins C and S,

increased hepatic synthesis of fibrinogen, and increased platelet aggregation. Clearly, these

5

Page 6: Case Nephrotic Syndrome

conditions predispose patients to an increased risk of spontaneous thrombosis and embolism. In

adults, most thromboses are venous, while in children arterial thromboses are more common.

Renal vein thrombosis is present in approximately 30% of patients with the nephrotic syndrome,

and the rate is highest in patients with membranous glomerulopathy. The patient with acute renal

vein thrombosis can present with sudden onset of flank or abdominal pain, gross hematuria, and

an acute decline in renal function, but most patients are asymptomatic. In addition to renal vein

thrombosis, 20% to 30% of nephrotic patients develop pulmonary emboli. Strokes and

myocardial infarctions are also potential complications that can occur as a result of the

hypercoagulable state associated with the nephrotic syndrome.6

e. Immunocompromised State

The nephrotic syndrome is associated with increased urinary loss of immunoglobulins,

especially IgG, as well as defects in the complement cascade. Each of these defects weakens the

immune system and increases susceptibility to infections. The pneumococcal vaccine, commonly

given to patients with nephritic syndrome, may have limited efficacy due to a rapid decline of

antipneumococcal antibody levels.6

f. Anemia

Patients with nephrotic-range proteinuria have a tendency to lose different types of

proteins in the urine, including binding proteins. With transferrin loss due to proteinuria, patients

present with an iron-resistant microcytic hypochromic anemia. With progressive renal failure,

anemia may result from decreased renal synthesis of erythropoietin.6

Pathophysiology

The basic functional defect in NS is an increased permselectivity of the glomerular

filtration barrier (GFB) to molecules that ordinarily would not be filtered. Numerous factors

affect the filtration of molecules, such as properties of molecules themselves, the properties of

the filtration barrier, and hemodynamic factors. Properties of molecules themselves include the

size and the charge of the molecule. The GFB has a negative charge imparted to it by a heparin

sulfate proteoglycan that retards the transport of negatively charged molecules across the GFB

while facilitating the passage of positively charged molecules. The increase of permeability of

6

Page 7: Case Nephrotic Syndrome

the GFB can be caused by damage of many macromolecules, including degradation of heparin

sulfate. This damage is suggested to be caused at least in part, by free radicals . The extremely

short half life of free radicals seems that they cannot be measured directly and that oxidative

activity must be measured indirectly by the levels of antioxidant substances in the body.7

Yet, in the nephrotic syndrome, the pathogenesis of edema is still a matter of controversy,

as several studies have given results that are inconsistent with the postulate that

hypoalbuminemic patiens have a contracted (‘under filled’) intravascular space. Although the

interpretation of these investigations has been challenged, the fact remains that only some

patients, especially children with minimal-change nephrotic syndrome (MCNS), as evidenced by

decreased blood volume, stimulated rennin-angiotensin-aldosteron secretion, and head-out water

immersion. In contrast, most patients with nephritic syndrome have evidence for a renal defect in

sodium excretion and thus have evidence of an expanded (‘overfilled’) plasma volume. Indeed, a

reduction in urinary sodium excretion can be shown to occur in the incipient stage of adults with

relapsing MCNS and in experimental nephritic syndrome coincident with the proteinuria, but

prior to fall in plasma albumin and PCOP.8

The interstitial inflamation of the kidney has a key role in the pathogenesis of nephrotic

edema by inducing primary sodium retention. The generation of vasoconstrictive substances in

the interstitium, driven by the inflammatory cell infiltrate, may influence sodium handling at

both the glomerular and tubular level, resulting in both decreased sodium filtration and increased

net sodium reabsorption. It should be recognized that tubulointerstitial mononuclear cell

infiltration is not always associated with sodium retention. For example, natriuresis follows the

release of urinary obstruction, an effect that may be mediated by the effect of interleukin-1 (IL-1)

in a collecting duct. IL-1 is a well-known macrophage product; therefore, the net effect of the

inflammatory infiltrate in urinary sodium excretion would depend on the secretory phenotype of

the infiltrating cells.8

Clearly, there is also a key role for the reduction in PCOP in the pathogenesis of edema.

Thus, hypoalbuminemia effectively buffers the hemodynamic effects of acute increments in

blood volume as the fluid overload is sequestered into the tissues and is responsible for the fact

that while patients with acute glomerulonephritis show a steep relationship between weight gain

and the humoral response, indicating plasma expansion, the patients with nephrotic syndrome do

not. This could help explain why many nephrotic subjects do not show hypertension, especially

7

Page 8: Case Nephrotic Syndrome

those with severe proteinuria and hypoalbuminemia. Low PCOP and primary sodium retention

combine to overwhelm the mecanisms protecting from changes in interstitial volume and drive

the development of edema.8

Investigation

Investigations at Initial Presentation9

a) Full blood count, blood levels of albumin,Cholesterol.

b) Renal profile: urea & creatinine.

c) Blood level of antistreptolysin O and C3.

d) Urinalysis and Quantification for urinary protein excretion and culture and sensitivity

e) X-ray chest, Montaux test, Hepatitis B surface antigen.

Other investigations depends on the clinical features and the physician in charge.

The international study of kidney Disease in children (ISKDC) had found that at the initial

presentation of children with minimal change nephrotic syndrome.

a. 20.7% of children had systolic blood pressure above 98th percentile for age ,

b. 22.7% had microscopic haematuria,

c. 32.5% had transiently raised plasma creatinine concentration.

Methods Available to Test for Proteinuria10

Method Indications Normal Range Comments

Dipstick testing Routine screening for proteinuriaperformed in the

office

Negative or trace in aconcentrated urine specimen

False–positive test can occur ifurine is very alkaline (pH<.8.0) or very concentrated(specific gravity:

<.1.025)

24-h urine for proteinand creatinineexcretion

Quantitation of proteinuria (aswell as creatinine

clearances)

,100 mg/m2/24 h in adocumented 24-h

collection

More accurate than spot urineanalysis. Inconvenient forpatient. Limited use inpediatric practice

8

Page 9: Case Nephrotic Syndrome

Spot urine for protein/creatinine ratio—preferably on firstmorning urinespecimen

Semiquantitative assessment ofProteinuria

,.2 mg protein/mg creatinine inchildren .2 y old,.5 mg protein/mg creatinine inthose 6–24 mo old

Simplest method to quantitateproteinuria. Less accurate thanmeasuring 24-h

proteinuria

Microalbuminuria Assess risk of progressiveglomerulopathy in patientswith diabetes mellitus

,30 mg urine albumin/gcreatinine on first morningurine

Therapy should be intensified indiabetics withmicroalbuminuria

Treatment of Initial Presentation of Nephrotic Syndrome

Since proteinuria is the main manifestation of nephritic syndrome and the cause of its

complications, several measures should be implemented to help reduce the proteinuria. The use

of angiotensin-converting enzyme inhibitors is the most important intervention, even in

normotensive patients. In addition, a low-protein diet has been shown to help reduce the

proteinuria, with a recommended daily protein intake of 0.7 g/kg/day. Patients should be

carefully monitored, however, to avoid malnutrition.6

a. Prednisolone

When the diagnosis of nephrotic syndrome has been made, prednisolone treatment can be

started in children with typical features. Children with atypical features should be referred to

paediatric nephrology for consideration of renal biopsy.

There is increasing evidence that longer initial courses of prednisolone are associated

with a lower incidence of relapse, and therefore a 12-week initial course is recommended. The

dose of prednisolone is based on surface area.11,12

• 60 mg/m2/day for 4 weeks (maximim 80 mg)

• 40 mg/m2/on alternate days for 4 weeks (maximum 60mg)

• Reduce dose by 5-10mg/m2 each week for another 4 weeks then stop

Prednisolone can be given as a single dose in the morning with food, or as divided doses

during the day. Patients should be issued with a steroid warning card, and they should be aware

of the side effects and risks of steroid treatment. Varicella status should be documented clearly in

9

Page 10: Case Nephrotic Syndrome

the casenotes and on HISS. If prednisolone causes gastric irritation, start ranitidine 2mg/kg bid

for the duration of steroid treatment.11

b. Albumin

As discussed above the clinical indications for albumin are

• Clinical hypovolaemia

• Symptomatic oedema

A low serum albumin alone is not an indication for intravenous albumin.

If there is evidence of hypovolaemia, give 1 g/kg 20% albumin (5ml/kg) over 4 - 6 hours. Give

2mg/kg of iv frusemide mid-infusion. If clinically shocked give 10ml/kg 4.5% albumin. Children

should be closely monitored during albumin infusions, and where possible they should be

administered during working hours.11

c. Penicillin Prophylaxis

Whilst nephrotic, children are at increased risk of infection, particularly with

encapsulated organisms such as pneumococcus. There is no evidence that antibiotic prophylaxis

is of benefit, and some centres do not use prophylaxis. Penicillin V can be given while there is

proteinuria and discontinued when the child goes into remission. Grossly oedematous children

are at risk of cellulitis and may benefit from antibiotic prophylaxis.

Dose: Under 5 yrs 125 mg bid

5yrs or above 250 mg bid11

d. Salt/Fluid Restriction

A low salt diet is used to try to prevent further fluid retention and oedema.11 Patients are

advised to limit their sodium chloride intake to 2 g/day.6 Fluid restriction may also be helpful.

These restrictions are lifted once the child goes into remission.11 For mild cases of edema, salt

restriction is coupled with a mild diuretic such as a thiazide.6 Loop diuretic like furosemide 1-3

mg/kgbw/day is given, if necessary, furosemide can be combined with spironolactone

(aldosteron antagonis, potassium thrifty diuretic) 2-4 mg/kgbw/day. Before giving diuretic,

10

Page 11: Case Nephrotic Syndrome

hypovolemia condition must be excluded. After using diuretic in 1-2 weeks, blood sodium and

potassium must be checked.12

e. Dietary Management

Although some benefit from dietary protein restriction has been described in a small

series of children with chronic renal insufficiency, a recent controlled study has not demonstrated

a significant impact of protein restriction on the rate of progression of renal disease. However, it

seems reasonable to avoid an excess of dietary protein in children with proteinuric renal diseases,

because high dietary protein intake may actually worsen proteinuria, at least in some patients

with nephrotic syndrome (NS), and does not result in a higher serum albumin. Thus, it is

recommended that children with proteinuria receive the recommended daily allowance of protein

for age.10 The necessity of protein diet according to RDA is 1,5-2 g/kgbw/day.12

f. Vaccination

Pneumococcal vaccination is recommended for children with NS. Consider giving at the

time of diagnosis. Varicella vaccination is only available on a named patient basis.11

g. Hyperlypidemia

The hyperlipidemia associated with the nephrotic syndrome may be managed with

nonpharmacologic interventions such as the use of soy protein diet to lower the total cholesterol

and LDL levels. The use of fish oil has been shown to lower triglycerides and VLDL. Statins are

the mainstay of treatment as they have been proven to reduce LDL levels.6

hs. Hypertension Management

● control blood pressure to 90th percentile of normal

● recommend low-salt diet, exercise, and weight reduction if obesity is present; and

● ACE-Is and/or ARBs for chronic pharmacologic management.

Hypertension is present in 13% to 51% of children with nephrotic syndrome. Blood

pressure generally improves with remission of nephrotic syndrome. When antihypertensive

11

Page 12: Case Nephrotic Syndrome

therapy is indicated, the expected reduction in proteinuria and blood pressure with ACE-I or

ARB agents make them first-line agents.13

Complications

a. Infection

Prophylaxis of S pneumoniæ with oral penicillin is often applied in patients during the

initial treatment with corticosteroids. Pneumococcal vaccine may be performed and is not

associated with an increased risk of relapse. In cases of peritonitis, antibiotics against both

S.pneumonia and gram-negative organisms are started after peritoneal liquid sampling. Varicella

is a serious disease in patients receiving immunosuppressive treatment or daily corticosteroids.

Varicella immunity status should be checked in these patients. In cases of exposure, early

preventive treatment by acyclovir must be instituted. Varicella vaccination is safe and effective if

the child is in remission even if he is on low-dose alternate day steroids.14

b. Acute renal failure

Acute renal failure (ARF) is another complication of nephrotic syndrome that occurs in a

small percentage of children.2 Renal function is usually within normal limits at presentation. A

reduction of the GFR, secondary to hypovolemia, infection or thrombosis is frequent. A reduced

GFR may be found in patients with normal effective plasma flow.14 Possible explanations for this

include: renal vein thrombosis, reduced renal perfusion, acute tubular necrosis, interstitial edema

within the renal parenchymal bed, and alterations in glomerular permeability. A recent report of

11 children with biopsy-proven MCNS with oliguric ARF found that alterations in glomerular

permeability played a greater role than that of reduced renal perfusion in these patients.2

Marked oliguria may occur in children. Oliguric renal failure may be the presenting

symptom. Renal failure may be secondary to bilateral renal vein thrombosis, which is recognized

by sonography or to interstitial nephritis which has been reported, especially with furosemide.

Skin rash and eosinophilia are suggestive of this diagnosis.14

Acute renal failure is usually reversible, often with high dose furosemide induced

diuresis, especially with intravenous infusion of albumin. In some cases, where glomerular

structure is normal on initial histology, renal failure may last for as long as a year and sometimes

be irreversible.14

12

Page 13: Case Nephrotic Syndrome

c. Thromboembolism

Thromboembolism is a potentially life-threatening complication of nephrotic syndrome.

The incidence of thromboembolism in children has been reported to range from 1.8 to 5%, with

the incidence being twice as high among children with SRNS as compared with SSNS. It is

possible that this complication is underestimated in children due to subclinical manifestations. In

one study of 26 children with SSNS who were systematically evaluated by ventilation–perfusion

scans to look for pulmonary emboli, findings consistent with pulmonary embolism were reported

in 28% of children.2

The majority of episodes of thrombosis in children are venous in origin, although arterial

thrombosis has been reported in 19–45% of cases. The most common sites for thrombosis are the

deep leg veins, inferior vena cava, and ileofemoral veins, although a variety of other veins and

arteries have been reported to be affected. In addition, central venous catheters, which are

sometimes used in the management of patients with refractory nephrotic syndrome with poor

vascular access, can further increase the risk of thrombosis.2

Pain and swelling of an extremity is suggestive of a deep venous thrombosis, and upper

extremity swelling accompanied by neck and facial swelling in the setting of a central venous

catheter should raise clinical suspicions for a central venous thrombosis. Similarly, development

of acute renal failure or gross hematuria should prompt a renal Doppler ultrasonographic

evaluation for possible renal vein or inferior vena cava thrombosis. Finally, development of

respiratory distress or cardiovascular symptoms should prompt evaluation by chest X-ray and

consideration of a ventilation–perfusion or chest CT scan to exclude possible pulmonary

embolus.2

d. Respiratory distress

In some patients, initiation of an albumin infusion in the setting of anasarca can lead to

the development of acute respiratory distress. Most often this is a result of rapid return of

interstitial fluid to the intravascular space, resulting in development of pulmonary edema. High-

risk patients include those with severe edema, those receiving albumin without adequate

diuretics, and those with compromised renal function. However, other causes for respiratory

distress, such as pleural effusion and pulmonary thromboembolism, should also be considered in

13

Page 14: Case Nephrotic Syndrome

any child with nephrotic syndrome who develops tachypnea or hypoxia. In the clinical setting of

pleural effusion with respiratory compromise, hospitalization for monitoring and diuresis (often

with albumin) is usually necessary. As noted above, in the setting of systematic screening of

asymptomatic children with nephrotic syndrome using ventilation perfusion scans, 28% of

children had findings consistent with pulmonary embolism.2

e. Anemia

Chronic nephrotic syndrome can also lead to the development of anemia. This is thought

to be due primarily to the loss of both erythropoietin and transferrin into the urine. These losses,

in combination with a reduced serum half-life for erythropoietin, and increased transferrin

catabolism, can result in the development of an erythropoietin-responsive anemia or iron

deficiency in patients who remain chronically nephrotic. Although treatable with erythropoietin,

induction of remission of the nephrotic syndrome is the most effective approach to correct the

anemia.2

Prognosis

Likelihood of achieving remission

It is generally accepted that the initial response to corticosteroids (i.e. induction of

complete remission) is the single best indicator of the long-term prognosis for a child presenting

with nephrotic syndrome, as children who fail to respond to an 8 week course of oral

corticosteroids have a guarded prognosis. Steroid response has been reported to correlate with

renal biopsy findings if done at disease outset prior to the institution of treatment. In these early

studies, while overall steroid responsiveness was seen in 78% of newly-diagnosed children

treated with corticosteroids, the likelihood of achieving remission varied greatly by histologic

diagnosis. Steroid responsiveness was 93% for MCNS, 30% for FSGS, 56% for mesangial

proliferative glomerulonephritis, 7% for MPGN, and 0% for membranous nephropathy. In

addition, the likelihood of steroid responsiveness was decreased in older children, possibly

related to the increasing incidence of the steroid-resistant glomerulopathies in later childhood.

This was supported by the findings that the median ages for clinical presentation with MCNS,

FSGS, and MPGN were 3 years, 6 years and 10 years old, respectively.2

14

Page 15: Case Nephrotic Syndrome

Relapse rate

Relapses of nephrotic syndrome occur commonly in SSNS. Only 30% patients with

SSNS will never experience a relapse, although the overall tendency to relapse decreases with

time. A large study of MCNS found that there was a gradual tendency toward an increase in the

number of non-relapsing patients over time, reaching 80% eight years after onset of disease.

Moreover, 75% of those patients who remained relapse-free for the initial six months after

treatment either continued in remission during their entire course or relapsed only rarely. Such

findings suggest that while the majority of children (60%) with nephrotic syndrome experience

one or more relapses, most patients experience a gradual decrease in the frequency of relapses

over time.2

ESRD and transplant recurrence risk

Non-responsiveness to corticosteroids clearly identifies those patients at high risk for

progressive kidney disease. In one large study of 389 children with nephrotic syndrome, 21% of

children with biopsy-proven MCNS who were unresponsive to the initial 8-week course of

steroids subsequently progressed to ESRD. Among children with nephrotic syndrome due to

FSGS who progress to ESRD, renal transplantation can also pose serious challenges. Nephrotic

syndrome recurs in the allograft in approximately 30% of such cases, and results in graft loss in

approximately one-half of those patients affected. Because FSGS is the most common

glomerulopathy associated with ESRD in children, this matter has received much attention in the

pediatric transplantation literature. Disease recurrence can be a devastating complication, and

efforts are ongoing to attempt to characterize patients at risk for disease recurrence. Clinical and

biopsy features of children at high risk for recurrence of FSGS are: those who reached ESRD

within 3–4 years following diagnosis, those with histologic features of mesangial proliferation,

and those with previous history of recurrence. Treatment strategies for recurrent nephrotic

syndrome post-transplant have included plasma exchange, cyclophosphamide, and intravenous

cyclosporine but none of these have proven to be uniformly effective.2

Mortality risk

Since the introduction of antibiotics and corticosteroids several decades ago, and the

further refinement of immunosuppressive agents in recent years, the mortality rate for nephrotic

15

Page 16: Case Nephrotic Syndrome

syndrome has been reduced to < 5% from 67% seen in the preantibiotic era. In a large ISKDC

series reported in 1984, the mortality rate was only 1.9%.339 Importantly, 9 of the 10 deaths in

this study occurred in children who either had SRNS or in those who relapsed within the first 8

weeks of steroid therapy, and six of these children died of infections, emphasizing the continued

importance of this complication of nephrotic syndrome. Thus, despite dramatic improvements in

the mortality risk for children with nephrotic syndrome over the last 50 years, it should be

remembered that children who prove to be steroid-resistant remain at increased risk for

potentially life-threatening complications of either nephrotic syndrome or its treatment.2

II.CASE

G, male,8 years old, 26,5 kg, 124 cm. was admitted to the non-infection unit of H.Adam

Malik Hospital on 18th March 2010 at18.45 pm. Main complaint was full body oedema. This

condition had occurred for 1 month. Oedema begin at abdomen, then to face and extremities.

Full body oedema subsides in 1 week after drug administration, but abdomen still enlarge.

History of meat-washed color urine was positive since 1 month . History of sandy urine was

negative. History of bloody urine was negative. Urinary pain was positive since 1 month. History

of trauma was positive at right side abdomen and happened at 1 month ago. No fever, no cough,

no vomiting, no diarrhea, no dyspnea. Family history of suffering renal problems was negative

History of immunization was unclear. History of recurrent fever was negative. History of birth,

spontaneous labor aided by midwife, spontaneous cry, birth weight : 3500gr. His past medical

history that this patient was referred from Pirngadi Hospital by a pediatrician with diagnosed as

Nephrotic Syndrome and had been taken care for 3 weeks. History of medication use, prednisone

4-4-3 and lasix 2x20 mg

Physical Examination

Generalized Status

Sens : compos mentis. Temperature : 36,7 C.

anemia (-), dyspnea (-), edema (+), cyanosis (-), icterus (-).

Localized Status

Head : Eye: light reflex +/+, pupil isocor right = left, pale inferior conjungtiva palpebra -/-

ear / nose/ mouth = within normal limits

16

Page 17: Case Nephrotic Syndrome

Neck : lymph node enlargement (-), stiff neck (-)

Thorax : fusiformed symmetrical, retraction (-)

HR = 92 bpm, regular, murmur (-)

RR = 26 rpm, regular, rales (-). Decreased breath sound on the lower region of right

and left lung

Abdomen : distention, collateral vein (+), ascites (+), shifting dullness (+)

Hepar/ spleen = difficult to be measured

Sleeping abdomen circumference: 80cm, Sitting abdomen circumference: 83 cm

Extremity : pulse 92 bpm, regular, Pressure/ Volume = adequate, acral warm

blood pressure = 100/70 mmHg, oedema (-)

Differential Diagnosis

Nephrotic Syndrome + Bilateral Effussion Pleura

Acute Glomerulonephritis + Bilateral Effussion Pleura

Working Diagnosis.

Nephrotic syndrome + Bilateral Effussion Pleura

Management

Spironolacton 2x25mg

Prednisone 4-4-3 tab

Captopril 2x12,5 mg tab

Amoxicillin 3x250 mg tab

Diet normal meal 1630 kkal with 53 gr of protein

Investigation plan.

Daily urinary dipstick

Fluid balance per 6 hours

Renal Function Test

Liver Function Test

Electrolyte

17

Page 18: Case Nephrotic Syndrome

Lipid Profile

Urinalysis

C3

Culture Urine and Sensitivity Test

Urine Esbach

Laboratory results on February 27 th 2010

Full Blood Count

WBC : 16,2.103 cell/mm3

RBC : 4,35.106 /mm3

Hb : 13,1 mg/dl

Ht : 41, 8 %

PLT : 541.103cell/mm3

Thorax and Abdomen Photo Results on March 2 nd 2010

Consolidation on right ang left lung were seen

Heart border was not clear

Air distribution on intestine was common

Intra abdominal organ was not clear

Conclusion: right and left effusion, abdomen was common

USG results on March 2 nd 2010

Hepar and Lien were normal

Right kidney 87cm, left kidney 84 cm, kidney was echo

Cortex and medulla were common, not specific to nephritic

Ascites was seen

Conclusion: ascites

Laboratory results on March 3 rd 2010

Urinalysis

Colour : yellow

18

Page 19: Case Nephrotic Syndrome

Turbidity : turbid

Protein : ++

Reduction : -

Sedimen

Erythrocyte : -

Leukocyte : 2-4

Renal epithel : -

Blaas epitel : -

Vag/Urethra Ep : 0-2

Cristal

Ca oxalate : -

T. phosphate : -

Cysteine : -

Uric : -

Cylindric : 1-2 leucocyte / wide field view

Urobilin : -

Bilirubin : -

Urobilinogen : +

pH : 6

SG : 1,020

Laboratory results on March 6 th 2010

Serum protein electrophoresis

Albumin : 11,0

Alpha 1 : 1,6

Alpha 2 : 53,0

Beta : 12,1

Gamma : 22,3

Laboratory results on March 9 th 2010

Full Blood Count

WBC : 8,5.103 cell/mm3

19

Page 20: Case Nephrotic Syndrome

RBC : 3,75.106 /mm3

Hb : 11,7 mg/dl

Ht : 36,6 %

PLT : 355.103cell/mm3

Laboratory results on March 18 th 2010

Full Blood Count

WBC : 17,9.103 cell/mm3

Hb : 14,9 mg/dl

Ht : 45,7 %

PLT : 510.103cell/mm3

Renal Function

Ureum : 70 mg/dl

Creatinin : 0,4 mg/dl

Liver Function

SGOT : 52 U/L

SGPT : 54 U/L

Electrolyte

Sodium : 136 mEq/L

Potassium : 3,1 mEq/L

Chloride : 94 mEq/L

Dipstick urine

Leu :70 Blood : -

Nit : +1 SG : 1,020

Uro : 0,2 Keton : -

Pro : +3 Bil : +

pH : 6 Glu : -

Counsult Thorax Photo to Radiology

Results can be interpretated as lung oedema

20

Page 21: Case Nephrotic Syndrome

Follow up March 19 th 2010

S : distention of abdomen (+)

O : Sens : compos mentis. Temperature : 37,1 C. BW: 26kg

Head : light reflex +/+, pupil isocor right = left, pale inferior conjungtiva palpebra -/-

ear / nose/ mouth = within normal limits

Neck : lymph node enlargement (-), stiff neck (-)

Thorax : fusiformed symmetrical, retraction (-)

HR = 98 bpm, regular, murmur (-)

RR = 22 rpm, regular, rales (-). Decreased breathing sound on the lower region of

right and left lung

Abdomen : distention, collateral vein (+), ascites (+), shifting dullness (+)

Hepar/ spleen = difficult to be measured

Sleeping abdomen circumference: 78 cm, Sitting abdomen circumference: 79 cm

Extremity : pulse 98 bpm, regular, Pressure/ Volume = adequate, acral warm,

blood pressure = 110/70 mmHg, oedema (-)

A: DD/- Nephrotic Syndrome + Bilateral Effussion Pleura

- Acute Glomerulonephritis + Bilateral Effussion Pleura

P: - Prednisone 4-4-3

- Lasix 2x20 mg

Dipstick urine

Leu : - Blood : -

Nit : - SG : 1,020

Uro : 0,2 Keton : -

Pro : +2 Bil : +

pH : 6 Glu : -

Follow up March 20 th 2010

S : distention of abdomen (+)

O : Sens : compos mentis. Temperature : 36,9 C. BW: 25 kg

21

Page 22: Case Nephrotic Syndrome

Head : light reflex +/+, pupil isocor right = left, pale inferior conjungtiva palpebra -/-

ear / nose/ mouth = within normal range

Neck : lymph node enlargement (-), stiff neck (-)

Thorax : fusiformed symmetrical, retraction (-)

HR = 90 bpm, regular, murmur (-)

RR = 24 rpm, regular, rales (-). Decreased breathing sound on the lower region of

right and left lung

Abdomen: distention, ascites (+), shifting dullness (+), collateral vein (+)

Hepar/ spleen = difficult to be measured

Sleeping abdomen circumference: 73 cm, Sitting abdomen circumference: 78 cm

Extremity: pulse 98 bpm, regular, Pressure/ Volume = adequate, acral warm,

blood pressure = 130/70 mmHg, oedema (-)

A: DD/- Nephrotic Syndrome + Bilateral Effussion Pleura

- Acute Glomerulonephritis + Bilateral Effussion Pleura

P: - IVFD D5% + Meylon 25 mEq 6 gtt/i mikro

- Inj. Ceftriaxone 750 mg diluted in D5% 20cc / 12 hours

- Inj. Lasix 30mg / 8h / iv

- Spironolactone 2 x 25 mg

- Prednisone 4-4-3

- Captopril 2x12,5 mg

- KSR 3 x 500mg

- Diet normal meal low salt 1630 kkal with 26 gr protein

- Nephrisol ad lib

Dipstick urine

Leu : - Blood : -

Nit : - SG : 1,005

Uro : - Keton : -

Pro : - Bil : -

pH : 8 Glu : -

22

Page 23: Case Nephrotic Syndrome

Counsult to Nephrology Division

Advise:

- IVFD D5% + Meylon 25 mEq 6 gtt/i mikro

- Inj. Ceftriaxone 750 mg diluted in D5% 20cc / 12 hours

- Inj. Lasix 30mg / 8h / iv

- Spironolactone 2 x 25 mg

- Prednisone 4-4-3

- Captopril 2x12,5 mg

- KSR 3 x 500mg

- Diet normal meal low salt 1630 kkal with 26 gr protein

- Nephrisol ad lib

- Daily urinary dipstick

- Fluid balance per 6 hours

- Abdomen and Urinary USG

- Blood Culture and Sensitivity Test

- Urine Culture and Sensitivity Test

- Urine Esbach

- ASTO, CRP, C3

Follow up March 21 th 2010

S : distention of abdomen (+)

O : Sens : compos mentis. Temperature : 36,8 C. BW: 25 kg

Head : light reflex +/+, pupil isocor right = left, pale inferior conjungtiva palpebra -/-

ear / nose/ mouth = within normal limits

Neck : lymph node enlargement (-), stiff neck (-)

Thorax : fusiformed symmetrical, retraction (-)

HR = 100 bpm, regular, murmur (-)

RR = 32 rpm, regular, rales (-).

Abdomen: distention, shifting dullness (+), ascites (+), collateral vein (+)

Sleeping abdomen circumference: 66 cm, Sitting abdomen circumference: 75 cm

Hepar/ spleen = difficult to be measured

23

Page 24: Case Nephrotic Syndrome

Extremity: pulse 100 bpm, regular, Pressure/ Volume = adequate, acral warm,

blood pressure = 90/60 mmHg, oedema (-)

A: Nephrotic Syndrome

P: - IVFD D5% + Meylon 25 mEq 6 gtt/i mikro

- Inj. Ceftriaxone 750 mg diluted in D5% 20cc / 12 hours

- Inj. Lasix 30mg / 8h / iv

- Spironolactone 2 x 25 mg

- Prednisone 4-4-3

- Captopril 2x12,5 mg

- KSR 3 x 500mg

- Diet normal meal low salt 1630 kkal with 26 gr protein

- Nephrisol ad lib

Dipstick urine

Leu : + Blood : -

Nit : + SG : 1,005

Uro : - Keton : -

Pro : - Bil : -

pH : 7 Glu : -

Follow up March 22 th 2010

S : distention of abdomen (+) , heartburn (+), tenderness on right abdominal (+)

O : Sens : compos mentis. Temperature : 36,80 C. BW: 21 kg

Head : light reflex +/+, pupil isocor right = left, pale inferior conjungtiva palpebra -/-

ear / nose/ mouth = within normal range

Neck : lymph node enlargement (-), stiff neck (-)

Thorax : fusiformed symmetrical, retraction (-)

HR = 118 bpm, regular, murmur (-)

RR = 30 rpm, regular, rales (-).

Abdomen : soepel, peristaltic (+), tenderness on hypochondrium dextra (+)

Hepar = difficult to be measured,

24

Page 25: Case Nephrotic Syndrome

spleen: unpalpable

Sleeping abdomen circumference: 59 cm, Sitting abdomen circumference: 65 cm

Extremity : pulse 118 bpm, regular, Pressure/ Volume = adequate, acral warm,

blood pressure = 100/60 mmHg, oedema (-)

A: Nephrotic Syndrome

P: - IVFD D5% NaCl 0,45% 20gtt/i micro

- Inj. Ceftriaxone 750 mg diluted in D5% 20cc / 12 hours

- Inj. Lasix 2 x 20mg

- Spironolactone 2 x 12,5 mg

- Prednisone 4-4-3

- Captopril 2x12,5 mg

- KSR 3 x 500mg

- Diet normal meal low salt 1630 kkal with 26 gr protein

- Nephrisol ad lib

Dipstick urine

Leu : + Blood : -

Nit : + SG : 1,005

Uro : 0,2 Keton : -

Pro : - Bil : -

pH : 7 Glu : -

Chest X-Ray conclusion

Sign of peritonitis was not found

USG conclusions

Abnormalities of renal and gallbladder were not confirmed

Follow up March 23 th 2010

S : distention of abdomen (+) , heartburn (+), tenderness on right abdominal (+)

O : Sens : compos mentis. Temperature : 36,6 C. BW: 21 kg

25

Page 26: Case Nephrotic Syndrome

Head : light reflex +/+, pupil isocor right = left, pale inferior conjungtiva palpebra -/-

ear / nose/ mouth = within normal range

Neck : lymph node enlargement (-), stiff neck (-)

Thorax : fusiformed symmetrical, retraction (-)

HR = 108 bpm, regular, murmur (-)

RR = 30 rpm, regular, rales (-).

Abdomen : soepel, peristaltic (+)

Hepar = difficult to be measured, tenderness on hypochondrium dextra (+)

spleen: unpalpable

Sleeping abdomen circumference: 57 cm, Sitting abdomen circumference: 61 cm

Extremity : pulse 108 bpm, regular, Pressure/ Volume = adequate, acral warm,

blood pressure = 100/60 mmHg, oedema (-)

A: Nephrotic Syndrome

P: - IVFD D5% NaCl 0,45% 20gtt/i micro

- Inj. Ceftriaxone 750 mg diluted in D5% 20cc / 12 hours

- Inj. Lasix 2 x 20mg

- Spironolactone 2 x 12,5 mg

- Prednisone 4-4-3

- Captopril 2x12,5 mg

- KSR 3 x 500mg

- Diet normal meal low salt 1630 kkal with 26 gr protein

- Nephrisol ad lib

Dipstick urine

Leu : - Blood : -

Nit : + SG : 1,015

Uro : 0,2 Keton : -

Pro : - Bil : +

pH : 7 Glu : -

Laboratorium results March 23 th 2010

26

Page 27: Case Nephrotic Syndrome

Full Blood Count

WBC : 25,1.103 cell/mm3

Hb : 13,3 mg/dl

Ht : 41,8 %

PLT : 425.103cell/mm3

Renal Function

Ureum : 70 mg/dl

Creatinin : 0,4 mg/dl

Uric Acid : 3,5

Glucose ad random : 91,8 mg/dl

Electrolyte

Sodium : 132 mEq/L

Potassium : 2,9 mEq/L

Chloride : 99 mEq/L

CRP +

ASTO < 200

Follow up March 24 th 2010

S : distention of abdomen (+) , heartburn (+), tenderness on right abdominal (+)

O : Sens : compos mentis. Temperature : 36,8 C. BW: 21 kg

Head : light reflex +/+, pupil isocor right = left, pale inferior conjungtiva palpebra -/-

ear / nose/ mouth = within normal range

Neck : lymph node enlargement (-), stiff neck (-)

Thorax : fusiformed symmetrical, retraction (-)

HR = 118 bpm, regular, murmur (-)

RR = 30 rpm, regular, rales (-).

Abdomen : soepel, peristaltic (+)

Hepar = difficult to be measured, tenderness on hypochondrium dextra (+)

spleen: unpalpable

Sleeping abdomen circumference: 56 cm, Sitting abdomen circumference: 59 cm

Extremity : pulse 118 bpm, regular, Pressure/ Volume = adequate, acral warm,

27

Page 28: Case Nephrotic Syndrome

blood pressure = 100/60 mmHg, oedema (-)

A: Nephrotic Syndrome

P: - IVFD D5% NaCl 0,45% 20gtt/i micro

- Inj. Ceftriaxone 750 mg diluted in D5% 20cc / 12 hours

- Prednisone 4-4-3

- Captopril 2x12,5 mg

- KSR 3 x 500mg

- Omeprazole 1x10mg

- Antasid

- Diet normal meal low salt 1630 kkal with 26 gr protein

- Nephrisol ad lib

Dipstick urine

Leu : - Blood : -

Nit : + SG : 1,010

Uro : 0,2 Keton : -

Pro : - Bil : -

pH : 6 Glu : -

Patient was discharged in March 24th 2010 and given :

Amoxicillin oral 3 x 250 mg

Prednisone 4-4-3

Captopril 2 x 12,5mg

KSR 3 x 500 mg

Omeprazole 1x10mg

This patient was referred to the outpatient clinic to continue the treatment.

III. DISCUSSION

28

Page 29: Case Nephrotic Syndrome

Nephrotic syndrome (NS), also known as nephrosis, is defined by the presence of

nephrotic-range proteinuria, edema, hyperlipidemia, and hypoalbuminemia. Based on those

definition, the diagnose nephrotic syndrome can be made based on history of edema and

laboratoies finding. These conditions were found in this patient. The patient came to Adam

Malik Hospital with abdomen swelling which had been experienced in the last month. The

laboratories finding in this patient were proteinuria (++), hyperlipidemia (Cholesterol total was

571 mg/dL), and hypoalbuminemia (1,42 g/dL). So, the patient was diagnosed as nephrotic

syndrome. The other laboratories investigation which can do in nephrotic syndrome suspect are

C3, C4, ANA (Anti Nuclear Antibody), and anti ds-DNA. But, in this patient, those

investigations were not done because the investigations were expensive and whether they were

not done, the diagnose nephrotic syndrome can made in this patient.

Approximately 70–80% of cases of nephrotic syndrome occur in children less than 6

years of age. In this patient, he was 8 years old. More than 80 % patients with nephrotic

syndrome show minimal change disease (MCD) characterized by normal renal histology on light

microscopy. Based on the condition, renal biopsy is not routine investigation in most cases of

nephrotic syndrome. The indications of doing renal biopsy is: (1) the age of first attack was

under 1 year old or above 16 years old; (2) there are gross hematuria, persistent microscopy

hematuria, or low serum C3 complement; (3) persistent hypertention; (4) decreasing of renal

function that are not related with hypovolemia condition; (5) suspected with secondary nephrotic

syndrome; (6) resistent steroid nephrotic syndrome; (7) before starting cyclosporine therapy. In

this patient, the above conditions were not found, so we were not doing renal biopsy in this

patient.

Patient was diagnosed with Nephrotic Syndrome. Since heavy proteinuria is a predictor

of rapid progression of renal failure, therefore reduction of proteinuria is a therapeutic goal. To

reduce heavy proteinuria in this patient, renal function improvement was the goal of treatment.

Initial therapy as recommended by the ISKD is prednisone 60mg/m2/ day or 2mg/kgBW/day

(maximum dose 80mg/day), given in divided dose to induce remission. Dosage of prednisone is

calculated based on ideal body weight. Full dose of initial prednisone is given for 4 weeks, if

remission occurs in the first 4 weeks, continued therapy of alternating dose (once every 2 days)

of 40mg/m2/day (2/3 of the initial dose) or 1,5 mg/kgBW/day for the next 4 weeks. If after the

first 4 weeks of full dose, patient does not show any remission, patient is classified as steroid

29

Page 30: Case Nephrotic Syndrome

resistant. In this patient, the initial prednisone therapy given was 55 mg/day (based on body

surface area) for first 4 weeks, divided in 3 dose in a day. After using prednisone, remission was

experience in this patient. Proteinuria was lost during 3 days of prednisone using.

Fluid restriction is recommended while severe edema is still exists. Loop diuretic like

furosemide 1-3 mg/kgbw/day is given, if necessary, furosemide can be combined with

spironolactone (aldosteron antagonis, potassium thrifty diuretic) 2-4 mg/kgbw/day. Before

giving diuretic, hypovolemia condition must be excluded. After using diuretic in 1-2 weeks,

blood sodium and potassium must be checked. In this patient, furosemide and spironolactone

were given. And based on laboratory finding which blood potassium level was low (3,1 mEq/L),

KSR was given to this patient.

Dietary management is based on RDA (Recommended Daily Allowances) for normal

protein diet of 1,5-2 g/ day to maintain child normal growth without risking glomerolus function

overload. In this patient, the protein diet should be given is 36 grams, but in fact, protein diet was

given only 26 grams. The other dietary management in nephrotic syndrome is low sodium

dietary (1-2 gr/day) while patient has edema. In this patient, low sodium dietary was given since

he was admitted to the hospital.

Patient with nephrotic syndrome are at increased risk of infection. This condition is

associated with low immunoglobulin (Ig)G that is seem to be the result of impaired synthesis

pointing to a primary disorder in lymphocyte regulation in Idiopathic Nephrotic Syndrome.

Infection risk is mostly of cellulitis, primary peritonitis, pneumonia, and upper respiratory tract

infection. The use of prophylactic antibiotic is somewhat more controversial. Although a recent

review on this subject supported its use, others have noted that prophylactic antibiotic usage may

result in development of resistant organism. Therefore, antibiotic prophylactic may be given on

this patient, especially those who undergo steroid therapy.The 3rd generation of cephalosporin

such as cefotaxim or ceftriaxone can be given for 10 to 14 days. This patient was given

ceftriaxone injection 750mg/12 hours/ iv in 20 cc of D 5 % in 30 minutes.

Hypertension can be find in SN patient related with steroid toxicity. The treatment of

hypertension is started with ACE-Inhibitor, ARB (angiotensin receptor blocker), β blocker, until

the blood tension of patient is under 90th percentil. In this patient, Captopril 3 x 12,5 mg was

given, because blood tension of patient was 130/70 mmHg.

The prognose of this patient is good according to the sensitivity of steroid.

30

Page 31: Case Nephrotic Syndrome

IV. REFERENCES

1. Bagga A, Mantan M. Nephrotic Syndrome in Children. Indian J Med. 2005; 122: 13-28

2. Valentini RP and Smoyer WE. Nephrotic Syndrome. Kheev KK, Schnaper HW, Mahkev SP.

Clinical Pediatric Nephrology. UK: Informa Health Care 2007; 155-194.

3. Lane CJ. Nephrotic Syndrome. eMedicine Pediatrics. 2009. Available from:

http://emedicine.medscape.com/article/982920-overview [Accessed March 20th 2010]

4. Noer MJ. Long Versus Standard Initial Steroid Therapy for Children with Idiopathic

Nephrotic Syndrome. Folia Medica Indonesiana. 2005; 41: 205-211.

5. Bergstein JM. Sindrom Nefrotik. Behrman, Kliegman, Arvin. Ilmu Kesehatan Anak Nelson.

Jakarta: EGC. 2000; 1828-1829

6. Keddis Mira T and Kamath BM. The Nephrotic Syndrome. Hospital Physician. 2007; 25-31

7. Ghodale SR, Suryakar AN, Ankesh RD, et all. Role of Reactive Oxigen Species in

Pathogenesis of Nephrotic Syndrome. Indian Journal of Clinical Biochemistry. 2010; 25: 82-

85.

8. Iturbe BR, Acosta JH, Johnson RJ. Interstial Inflammation, Sodium Retention, and the

Pathogenesis of Nephrotic Edema: An Unifying Hypothesis. Kidney International. 2002; 62:

1379-1384.

9. Uddin GM. Management of Steroid Sensitive Idiopathic Nephrotic Syndrome in Children.

Journal of Bangladesh College of Physicians and Surgeons. 2007; 25: 38-42.

10. Mogg RJ, Portman RJ. Milliner Daun, et all. Evaluation and Management of Proteinuria and

Nephrotic Syndrome in Children: Recommendations from a Pediatric Nephrology Panel

Established at the National Kidney Foundation Conference on Proteinuria, Albuminuria Risk,

Assesment, Detection and Elimination (PARADE) Pediatrics. 2000; 105: 1242-1249.

11. Beattie J. Guideline for the Management of Nephrotic Syndrome. Renal Unit Royal Hospital

for Sick Children Yorkhill Division. 2007; 1-8.

12. Trikono PP, Tambunan T, Pudjiastuti F, et all. Konsensus Tatalaksana Sindrom Nefrotik

Idiopatik pada Anak. Jakarta: Badan Penerbit IDAI. 2008: 1-22.

13. Gipson DS, Massengill SF, Yao L, et all. Management of Childhood Onset Nephrotic

Syndrome. Pediatrics. 2009; 124: 747-757.

31

Page 32: Case Nephrotic Syndrome

14. Niaudet P & Boyer O. Idiopathic Nephrotic Syndrome in Children: Clinical Aspects. Avner

ED, Haman WE, Niaudet P, et all. Pediatric Nephrology. Berlin Heidelberg: Springer. 2009;

662-702.

32