Presentators:

Presentator:
Ranap Hadiyanto Gultom
Day/Date : Tuesday / 29th june 2010
Supervisor:
Prof. Dr. Atan Baas Sinuhaji, SpA (K)
 Introduction
• Nephrotic syndrome (NS)  also known as
nephrosis
• Nephrotic syndrome is not a disease itself, but
the manifestation of many different
glomerular diseases
• Caused by various disorders that damage the
kidneys, particularly the basement membrane
of the glomerulus
 Definition

• Nephrotic syndrome is a kidney disorder in

which damage has occurred to the kidneys in
the kidney's filtering system called the
glomeruli
• Resulting:
– Proteinuria
– Hipoalbuminemia
– Edema
– Hipercolesterolemia
 Epidemiology

• Incidence rate is 2-7 cases per 100.000 children

younger than 16 years
• ISKDC found
– 76,6% of children with INS had MCNS
– 7% of cases associated with FSGS
– Children younger than 8 years  males:females (2:1 to
3:2)
– 66% of patients with either MCNS or FSGS are male, 65% of
individuals with MPGN are female
• Indonesia  6 cases per 100.000 children younger than 14
years, males to females is 2:1
 Etiology
1. Associated with glomerular diseases intrinsic to
the kidney and not related to systemic causes 
Primary nephrotic syndrome
2. Extrinsic to the kidney and related to systemic
causes  Secondary nephrotic syndrome
3. Defects in the nephrin gene (NPHS1),
phospholipase C epsilon 1 gene (PLCE1), and the
Wilms tumor suppressor gene (WT1)  Infantile
NS and congenital NS
4. Mutations in the podocin gene (NPHS2) 
autosomal-recessive form of FSGS, Mutations in
the α-actinin-4 gene (ACTN4) and the gene TRPC6
 autosomal-dominant forms of familial FSGS
 Predisposition factors

Multitude factors:
– Infection
– Drug exposure
– Hereditary disorders
– Immune disorders
– Diabetes
– Systemic lupus erythematosus
– Multiple myeloma
– Amyloidosis
– Etc
 Classification
Primary Nephrotic Secondary nephrotic syndrome Genetic nephrotic
Syndrome syndrome
MCNS Infections
FSGS Congenital syphilis, toxoplasmosis,
Finnish-type
MPGN cytomegalovirus, rubella
congenital nephrotic
Membranous Hepatitis B and C
syndrome (NPHS1,
glomerulonephritis (MGN) HIV/acquired immunodeficiency syndrome
nephrin)
IgA nephropathy (AIDS)
Denys-Drash
Idiopathic crescentic Malaria
syndrome (WT1)
glomerulonephritis Drugs
Frasier syndrome
Penicillamine
(WT1)
Gold
Diffuse mesangial
Nonsteroidal anti-inflammatory drugs (NSAIDs)
sclerosis (WT1, PLCE1)
Interferon
Autosomal recessive,
Mercury
familial FSGS (NPHS2,
Heroin
podocin)
Pamidronate
Autosomal dominant,
Lithium
familial FSGS (ACTN4,
Systemic disease
α-actinin-4; TRPC6)
Systemic lupus erythematosus
Nail-patella syndrome
Malignancy - Lymphoma, leukemia
(LMX1B)
Vasculitis -Wegener granulomatosis, Churg-
Pierson syndrome
Strauss syndrome, polyarteritis nodosa,
(LAMB2)
microscopic polyangiitis, Henoch-Schönlein
Schimke immuno-
purpura (HSP)
osseous dysplasia
Immune-complex–mediated -
(SMARCAL1)
Poststreptococcal glomerulonephritis
Galloway-Mowat
syndrome
Oculocerebrorenal
(Lowe) syndrome
 Pathophysiology

increase in permeability of the glomerular

capillary wall

Proteinuria and Hypoalbuminemia

 Pathophysiology
T-cell dysfunction
MCNS leads to alteration
of cytokines

increase
in Nephro
plasma factor capillary tic
FSGS produced by wall syndro
lymphocytes permeabi me
lity

mutations in
SRNS NPHS2 (podocin)
and WT1 genes
 Clinical manifestation
History
• Edema  periorbital,
peripheral, scrotal, or abdominal
Physical
• The most common clinical
and can be massive (anasarca)
finding is edema, pitting edema
• A history of a respiratory tract
found in the lower extremities,
infection  Preceding the onset
face and periorbital regions,
of nephrotic syndrome
scrotum and labia, and
• Upper respiratory infections,
abdomen
otitis media, and other infections
• Tachypnea, Pulmonary edema
 INS
and effusions
• fever, lethargy, irritability, or
• Hypertension
abdominal pain due to sepsis or
• Abdominal tenderness might
peritonitis
indicate peritonitis
• Anorexia, irritability, fatigue,
abdominal discomfort, and
diarrhea
 Diagnosis
investigation of the patient’s history and physical
examination

diagnostic laboratory test

• The urinalysis reveals 3+ or 4+ proteinuria (Urinary protein
excretion exceeds 3,5 g/24 hr in adults and 40 mg/m2/hr in children)
• The serum albumin level is generally less than 2.5 g/dL
• The serum cholesterol and triglyceride levels are elevated

Nephrotic syndrome
Management
Management
 Complication
• Infection
• Thrombosis
• Hyperlipidemia
• Acute kidney failure (AKF)
• Side effects of drug therapy
– Corticosteroids  Behavioral changes, increased
appetite, and Cushingoid signs, infection, obesity, growth
delay, osteopenia, avascular hip necrosis, cataracts,
hypertension, hyperglycemia, nephrolithiasis, and
hyperlipidemia, etc
– Diuretics  hypokalemia and contraction alkalosis
– Albumin  pulmonary edema and congestive heart failure
– Calcineurin inhibitors  increased susceptibility to
infection, direct nephrotoxicity, hyperkalemia, and
hypertension
 Prognosis
• Most children with nephrotic syndrome
eventually go into remission
• Resistant INS has a good prognosis if
remission of proteinuria can be achieved by
other medications
• Failure to respond to treatment and kidney
insufficiency at presentation are predictors of
poor outcome and progression to ESKD
 Objective
The aim of this paper work is to report a case of
nephrotic Sindrome in a 4 years 4 month old
child.
 Case Report
• M, female, 4 years 4 month of age, body weight 18 Kg,
height 104 cm, was admitted to unit non infection unit of
Haji Adam Malik Hospital on May 25th 2010 at 02.00 AM.
The main complaint was edema all of body. This condition
had occurred for 3 month and become worst in 3 weeks
ago. Edema begin when she 3 years old. Breathing difficulty
and cough was not found. History of fever and trauma also
not found. Patient have micturation of difficulties volume
±30 cc each time of micturation. Frecuency 3 times a
day.Defecation was normal.
• History of previous disease: the patient wass referred from
RS Tamiang and was diagnose with nephrotic Sindrom.
• History of previous medication:Lasix, Prednison.
 Physical Examination
Sens : Compos Mentis, Temperature: 37,3o C,body weight 18 kg, Anemia (-), Icteric (-), Dyspnea
(-), Oedema (+), Cyanosis (-).
Head: Eye : Light reflexes (+/+), isochoric pupil, pale inferior conjunctive palpebra (-/-) 
,edema palpebra superior and inferior (+/+).
Ear/Nose/Mouth: Normal
Neck : Lymph node enlargement (-)
Chest : Symmetrical fusiformed, retraction (-)
HR : 112 bpm, regular, murmur (-) RR : 22 rpm, regular, rales (-)
Abdominal : Soepel, peristaltic (+) N, ascites (+) Sleeping abdominal circumference :61 cm,
Sitting abdominal circumference :63cm,shifting dullnes (+),
Hepar/ spleen : unpalpable
Extremities : Pulse was 112 bpm, regular, Pressure/Volume : adequate, edema (+/+), BP: 110/80
mmHg.
Differential diagnosis
• Nephrotic sindrom
• Acute Glomerulonephritis
Working diagnosis
Nephrotic sindrom
Management
IVFD D 0.5% 5gtt/I micro
Inj. Lasix 10 mg/8 hour
Inj. Ceftriaxone 500 mg/12 hour (at DS 0.5% 50 cc finished in 30 minutes)
Spironolacton 2 x 6.25
Diet MBRG 1400 with 36 gr protein
 
Further Examinations :
CBC
Urinalisa
C3
LFT
RFT
Electrolyte
Albumin
Lipid profile
ASTO
CRP
Mantoux test
 Laboratory Result : (Mayl 25th 2010)
Routine Blood Examination
Hemoglobin : 12.20 g% (11.3-14.1)
Leukocyte : 10.700 103/mm3 (4.5-13.5)
Hematocryte : 38.60 % (37-41)
Thrombocyte : 438.000 103/mm3 (150.000-450.000)
MCV : 84.40 fl (81-95)
MCH : 26.70 pg (25-29)
MCHC :31.60 g% (29-31)
RDW :13.00% (11,6-14,8)

Liver function
AST/SGOT :16 U/L (<32)
ALT/SGPT:10 U/L (<31)
Renal function
Ureum :61.00 mg/dl(<50)
Creatinin :0.80 mg/dl(0.40-0.60)
Met carbohidrat : 93.00 mg/dl (<200)
Blood Electrolic
Na : 133 mEq/L (135-155)
K : 4.2 mEq/L (3.6-5.5)
 
• Thorax foto was normal
 Follow Up may 25/26/27 2010
Patient Phisycal diagnosatic Diagnosis Treatment
complain

S : Edema SP: Sens : CM/CM/CM Nephrotic IVFD D 0.5% 5 gtt/I micro→6gtt/i→5 gtt/I
in all T : 37.3˚C/37.10 C/37.10 C BW=18 kg syndrome
body (+), Head : Eye : Light reflexes (+/+), isochoric pupil, pale inferior Lasix Inj. 10 mg/8 hour→Furosemide Inj.10 mg/8
fever (-) conjunctive palpebra -/-. Conjungtiva palpebra superior hour→Lasix Inj.20mg/8hour/iv
Edema (+/+).
Ear/Nose/Mouth: Normal Ceftriaxone Inj.500 mg/12 hour→Ceftriaxone
Neck : Lymph node enlargement (-) Inj.500 mg/12 hour→Ceftriaxone Inj.500 mg/12
hour
Chest : Fusiformed symmetrical, retraction (-)
HR :104 /98/98 bpm, regular, murmur (-) Spinolacton 2 x 6.25→Spinolacton 2 x
RR :28/28/24 tpm, regular, rales (-) 6.25→Spinolacton 2 x 12.5 mg

-/Captopril 2 x 6.25 mg/captopril 2 x 6.25

Abdominal : Soepel, peristaltic (+) N

Hepar/spleen : unpalpable Diet MBRG 1400 with 36 gr protein /Diet
MBRG//Diet MB 1640 kkal + 36 gr protein
Extremities : Pulse was /104/98/98 bpm, regular,
Pressure/Volume : adequate, edema pretibial (+/+), edema
dorsum pedis (+/+). Fluids balance/ 6 hour
Dipsticks urine every morning

Albumin level 1.1 g/dl ( june 26 th 2010)

Cholesterol total 774 mg/dl ( june 26 th 2010)
 Urine dipstic may 25/26/27 2010

(May 25th 2010) (May 26th 2010) (May 27th 2010)

Leu: - Leu: - -
Nit: - Nit: -
Uro: 0.2 Uro: 0.2
Pro: +2 Pro: 2000 (20) ++++
pH:6,0 pH:6,0
Blo:± Blo: -
Ket: 5 Sb: 1.015
Bil: 1 Ket: 5
Glu: - Bil: 1
Glu: -
 Follow Up may 28/29/30 2010
Patient Phisycal diagnosatic Diagnosis Treatment
complain

S : Edema in all SP: Sens : CM/CM/CM Nephrotic IVFD D 0.5% 5 gtt/I micro→5gtt/i→4 gtt/I
body (+), fever (-) T : 36.9˚C/37.10 C/T : 36.5˚C, BW : 18 Kg syndrome
BSA:0,74/m² Lasix Inj. 25 mg/6hour/iv→Lasix Inj.25 mg/6
hour→Lasix Inj.25mg/6hour/iv
Head : Eye : Light reflexes (+/+), isochoric pupil,
pale inferior conjunctive palpebra -/-. Ceftriaxone Inj.500 mg/12 hour→Ceftriaxone
Conjungtiva palpebra superior Edema (+/+). Inj.500 mg/12 hour→Ceftriaxone Inj.500
Ear/Nose/Mouth: Normal mg/12 hour
Neck : Lymph node enlargement (-)
Spinolacton 2 x 12.5→Spinolacton 2 x
Chest : Fusiformed symmetrical, 12.5→Spinolacton 2 x 12.5 mg
retraction (-)
HR :100 /110/80 bpm, regular, Captopril 2 x 6.25/Captopril 2 x 6.25
murmur (-) mg/captopril 2 x 6.25
RR :28/26/24 tpm, regular,
rales (-)
Diet MB 1640 kkal + 36 gr protein/Diet MB
1640 kkal + 36 gr protein/Diet MB 1640 kkal +
36 gr protein
Abdominal : Soepel, peristaltic (+) N
Hepar/spleen : unpalpable Prednisone tab 3-2-2/Prednisone tab 3-2-2
/Prednisone tab 3-2-2
Extremities : Pulse was /100/110/80 bpm,
regular, Pressure/Volume : adequate, edema Fluids balance/ 6 hour
pretibial (+/+), edema dorsum pedis (+/+). Dipsticks urine every morning
 Urine dipstic may 28/29/30 2010

(May 28th 2010) (May 29th 2010) (May 30th 2010)

Leu: - Leu: 15 -
Nit: - Nit: -
Uro: 0.2 Uro: 0.2
Pro: +3 Pro: 300 ++++
pH:6,0 pH:5,0
Blood:± Blo: -
SG:1.020 SG: 1.010
Ket: - Ket: -
Bil: - Bil: -
Glu: - Glu: -
 Follow Up may 31/01/02 2010
Patient Phisycal diagnosatic Diagnosis Treatment
complain
S : Edema in SP: Sens : CM/CM/CM Nephrotic IVFD D 0.5% 4 gtt/I micro→4gtt/i→4 gtt/I
all body (+), T : 36.8˚C/37.10 C/T : 36.5˚C, BW : 18 Kg BSA:0,74/m² syndrome
fever (-) Lasix Inj. 30 mg/6hour/iv→Furosemide Inj.10
Head : Eye : Light reflexes (+/+), isochoric pupil, pale mg/8 hour→Furosemide Inj.10mg/8hour/iv
inferior conjunctive palpebra -/-. Conjungtiva palpebra
superior Edema (+/+). Ceftriaxone Inj.500 mg/12 hour→Ceftriaxone
Ear/Nose/Mouth: Normal Inj.500 mg/12 hour→Ceftriaxone Inj.500
Neck : Lymph node enlargement (-) mg/12 hour

Chest: Fusiformed symmetrical, retraction (-) Spinolacton 2 x 12.5→Spinolacton 3 x

HR :80 /110/109 bpm, regular, murmur 12.5→Spinolacton 3 x 12.5 mg
(-)
RR :24/28/24 tpm, regular, rales (-) Captopril 2 x 6.25/Captopril 2 x 6.25
mg/captopril 2 x 6.25

Abdominal : Soepel, peristaltic (+) N Diet MB 1640 kkal + 36 gr protein/Diet MB

Hepar/spleen : unpalpable 1640 kkal + 36 gr protein/Diet MB 1640 kkal +
36 gr protein
Extremities : Pulse was 80/110/109 bpm, regular,
Pressure/Volume : adequate, edema pretibial (+/+) /(-/-) Prednisone tab 3-2-2/Prednisone tab 3-2-2
-/- , edema dorsum pedis(+/+) / (+/+) / (↓/↓). /Prednisone tab 3-2-2

Fluids balance/ 6 hour

Dipsticks urine every morning
 Urine dipstic may 31/01/02 2010
(May 31th 2010) (June 01st 2010) (June 02nd 2010)

Leu: ±15 Leu: - Leu: -

Nit: - Nit: - Nit: -
Uro: 0.2 Uro: 0.2 Uro: 0.2
Pro: 300 (+++) Pro: +++ Pro: +++
pH:5,0 pH:6 pH:6
Blo:- Blood: - Blood: -
SG:1.020 SG: 1.020 SG: 1.020
Ket: - Keton: - Keton: -
Bil: - Bil: Bil:
Glu: - Glu: - Glu: -
 Follow Up may 03/04/05 2010
Patient Phisycal diagnosatic Diagnosis Treatment
complain
S : Edema in SP: Sens : CM/CM/CM Nephrotic IVFD DS 0.5% + Meylon 10 mg 4 gtt/I
all body (+) T : 36.3˚C/36 0 C/T : 36.9˚C, BW : 18 Kg/15 Kg/15 Kg syndrome micro→4gtt/i→4 gtt/I
/(+)/ (+), fever BSA:0,74/m²
(-) / (-) / (-) Furosemide Inj. 10 mg/8hour/iv→Furosemide
Head : Eye : Light reflexes (+/+), isochoric pupil, pale Inj.10 mg/8 hour→Furosemide
inferior conjunctive palpebra (-/-). Inj.10mg/8hour/iv

Edema around eyes (-/-) Ceftriaxone Inj.500 mg/12 hour→Ceftriaxone

Inj.500 mg/12 hour→Ceftriaxone Inj.500
Ear/Nose/Mouth: Normal mg/12 hour
Neck : Lymph node enlargement (-)
Spinolacton 2 x 2.625→Spinolacton 2 x
6.25→Spinolacton 2 x 6.25 mg
Chest: Fusiformed symmetrical, retraction (-)
HR :100/106 /98 bpm, regular, murmur (-) Captopril 2 x 6.25/Captopril 2 x 6.25
RR :28/30/22 tpm, regular, rales (-) mg/captopril 2 x 6.25

Diet MB 1640 kkal + 36 gr protein/Diet MB

Abdominal : Soepel, peristaltic (+) N 1640 kkal + 36 gr protein/Diet MB 1640 kkal +
Hepar/spleen : unpalpable 36 gr protein

Extremities : Pulse was 100/106/98 bpm, regular, Prednisone tab 3-2-2/Prednisone tab 3-2-2
Pressure/Volume : adequate, edema pretibial (-/-) / /Prednisone tab 3-2-2
(-/-) /(-/-) , edema dorsum pedis(-/-) / (-/-) / (-/-).
Fluids balance/ 6 hour
Dipsticks urine every morning

Added medication:
P:-Amoxicilin 3x 200mg(oral)
-lasix tab 3x 10mg(oral)
 Urine dipstic may 03/04/05 2010
(May 03rd 2010) (June 04st 2010) (June 05st 2010)

Leu: - Leu: - Leu: -

Nit: - Nit: - Nit: -
Uro: 0.2 Uro: 0.2 Uro: 0.2
Pro: +++ Pro: +++ Pro: +++
pH:6.5 pH:7 pH:7
Blood: - Blood: - Blood: -
SG: 1.010 SG: 1.015 SG: 1.0
Keton: - Keton: - Keton: -
Bil: Bil: Bil:
Glu: - Glu: - Glu: -
 Discussion
Teori case

• Nephrotic syndrome can affect all age • The patient was diagnosed with
groups, most common from age 2 to 6 Nephrotic Syndrome when she was 3
years old

• Edema is the presenting symptom in about • The patient was admitted , with main
95% of children with NS complaint swelling (edema) of whole body

• In NS, occurs abnormal excretion of protein • The urinalysis reveals +2 - +4 proteinuria

in the urine (proteinuria)

• The serum concentration of albumin often is • Laboratory findings showed albumin 1,1
significantly low gr/dL

• The serum concentration of cholesterol and • Total cholesterol: 774 mg/dL,

trygliseride are increased significantly trygliserida: 560 mg/dL

• Corticosteroids is the first step in treatment • The treatment such as: prednisone, lasix,
of idiopathic nephrotic syndrome, the other captopril, ceftriaxone, spironolacton.
treatment are Diuretic, Antihypertensive, etc
 Discussion
Teori
• In Nephrotic Syndrome Monitoring of urine
protein and fluid status is an important aspect
of management
• A normal activity plan is recommended and
keeping children from respiratory tract
infections may be beneficial
• Patients who remain responsive to steroids
with remission of proteinuria, even with
frequent relapses, generally have a good
prognosis, steroid-resistant INS has a good
prognosis if remission of proteinuria can be
achieved by other medications
case
• Every morning urin dipstic and fluid
balance had been done
• The patient has been hospitalized (bed
rest) and has been given antibiotic to
prevent from infection
• Prognosis the patient was good because
occurred a decreasing of edema and the
patient has been treated effectively until
patient discharged from this hospital
 SUMMARY
• It has been reported a case of a 4 year 4 month girl with nephritic
syndrome. She was presented to our unit non-infection pediactric
department of Adam Malik General Hospital on 25 may 2010 at
02.00 am with main complaint swelling of whole body. This has
been experienced by the patient for 3 month before and got
worse in the 3 weeks. Edema begin when she 3 years old.
Breathing difficulty and cough was not found. History of fever
and trauma alswo not found. Patient have micturation of
difficulties volume ±30 cc each time of micturation. Frecuency 3
times a day.Defecation was normal

• The diagnosis was established based on anamnesis, clinical sign,

physical examination, and laboratory findinds. She was treated
accordingly to her symptoms. And she was discharged on 5th june
2010