Kuliah Glomerulopati

pada anak
Muhammad Heru
Muryawan,dr,SpA(K)

Pokok bahasan
Glomerulopati
1. Sindrom nefrotik
2. Glomerulonefritis

Gejala gangguan ginjal anak yang

sering dijumpai
Edema

Renal
Kardial
Hepatal
Nutrisional

Hematuria (ginjal/di luar ginjal)

Proteinuria
Hipertensi (primer/sekunder)
Penurunan Laju filtrasi glomerulus.

GLOMERULOPATHY
MAIN CAUSE OF KIDNEY FAILURE IN CHILDREN

DEFINITION:
inflamatory changes in glomerulus due to
immunologic mechanism
CLINICAL MANIFESTATIONS
Isolated proteinuria
Proteinuria + edema (i.e.Nephrotic
syndrome)
Isolated haematuria
Hypertension +/- proteinuria/haematuria
Renal failure

Classification
Congenital

Primary/
idiopathic

Acquired
Secondary

Alport Syndrome
Congenital Nephrotic Syndrome

1. Minimal change (MCNS)

2. Focal segmental
glomerulosclerosis
3. Mesangial proliferative
glomerulonephriti
4. Membrano-proliferative
glomerunephritis
5. Membranous glomerulonephritis
6. IgA Nephropathy
7.
Glomerulonephritis
Post Infection Post others
streptococcal

glomerulonephritis
Mulisystem diseases: LE, HUS
Intoxicaation: drugs, metal
Neoplasms

Nephrotic Syndrome
Massive Proteinuria
- 50 mg/kg body weight /day,
or
- 40 mg/m2/hour, or
- Urine protein/creatinin ratio > 2
mg/mg, - dipstick +2)

Heavy Hypoalbuminemia
< 2,5 g/dL
Edema
Hyperlipidemia (>200mg%)
Roth KS. Nephrotic syndrome: Pathogenesis and management. Ped in Rev 2002;23(7):237-47

Epidemiology
Incidence
Incidence 2-7 new cases per 10,000
Prevalence 15.7 cases per 10,000

Age
MCD 2.5 years median age
FSGS 6 years median age

Sex
3:2 Boys; Girls in children <6 yo
Equal ratio in those older

Classification
1. Congenital.
2. Primary nephrotic syndrome
The term applied to disease limited to
the kidney
Minimal change, lipoid disease, nil
disease
Focal segmental schlerosis
Membranous nephropathy
Proliferative nephritis (mesangial,
focal, diffuse)
3. Secondary nephrotic syndrome
Lupus nephritis
Henoch-Schonlein purpura

Classification of Nephrotic syndrome

Response to steroid

Barratt TM. Steroid responsive nephrotic syndrome. In: Barratt TM, editor. Pediatric nephrology. 4th edition. Baltimore:
Lippincot Wiliams & Wilkins;1999. p. 732.

CONGENITAL NEPHROTIC SYNDROME

clinical onset in the first 3 months of life

proteinuria in utero or at birth
elevated amniotic fluid level of alpha-fetoprotein
before 20 weeks gestation
Classification :

Primary
Finnish type
Diffuse mesangial sclerosis
Minimal changes NS
Focal segmental glomerulosclerosis
Secondary
congenital syphilis, toxoplasmosis, cytomegalovirus
XY gonadal dysgenesis and Wilms tumour
nephroblastoma
etc

IDIOPATHIC/PRIMARY
NEPHROTIC SYNDROME

Etiologi
90 % idiopathic nephrotic syndrome
75 % minimal change nephrotic syndrome
(MCNS)
10 % focal segmental glomerulosclerosis
(FSGS)
< 5 % membranous nephropathi

10 % Membrano proliferative
glomerulonephritis

EPIDEMIOLOGY

Pathophysiology
The underlying pathogenetic abnormality
of NS is proteinuria due to an increase in
glomerular capillary wall permeability.
1. The capillary wall loss the negative
charge
glycoprotein barries
2. Increase glomerular permeability to
proteins

Gambar 1. Penampang ginjal

Patofisiologi SN
Kehilangan muatan negatif
Di membran basalis
Proteinuria
Hipoalbunemia
hiperkolesterolemia
edema

In principle edema may develop

by two mechanism :
A. The capillary hydraulic pressure
increases as a result of constant
elevation of plasma volume :
overflow concep (nehpritic
edema)
B. The colloid osmotic pressure in
plasma drops : underfilling
theory (nephrotic edema

Nephritic edema
edema

Nephrotic

Renal salt and water

Alteration of
Starling forces
retention
(Capillary colloid osmotic
pressure )
Expansion of
circulatory volume

Edema formation

Alteration of Starling forces

Volume contraction
(Capillary hydraulic pressure )
Edema formation
Renal salt and water
retention
Proposed
scheme of edema formation in

patiens with glomerular disease

Clinical manifestation
Idiopathic nephrotic syndrome
Prevalency male : female = 2 : 1
Most commonly between the age of 2
6 ys
Edema, initially noted around the
eyes, and in the lower extremities is
pitting. It becomes generalized

Generelised edema
(anasarca)

Older child with

nephrotic syndrome
Pitting peripheral
oedema

Nephrotic Syndrome

Ascites

Nephrotic syndrome

SCROTAL EDEMA

LABIAL EDEMA

Laboratory Test in N S
To confirm NS
Serum (albumin, globulin, cholesterol)
Urine protein : qualitative (dipstick : albumin)
quantitative (24-hr collection)
To distinguish primary from secondary NS
Urinalysis
Screening test for sickle cell anemia
Serum C3 complement
Serum antinuclear antibody
Hepatitis B surface antigen
Management test
Complete Blood Counts , serum
electrolytes,serum creatinine, BUN

Diagnosis
Edema
Proteinuria

on the dipstick +3
(approximately 300 mg/dl).
Serum albumin levels is generally less than
2 gram/dl
Serum cholesterol and triglycerides levels
are elevated
Renal function may be normal or reduced

Management
A. General Principles
No sistematic dietary advice is necessary
in simple cases of SRNS
Antibiotic is indicated in cellulitis, peritonitis, septicemia,etc.
Diuretic: Edematous child in the absence of hypovolemia
diuretic : furosemide (1-2 mg /kgBW/day)
Albumin infusions : Expensive & can hazardous but may be
life saving, its indications include :
Hypovolemia (abdominal pain, hypotension, oliguria)
Renal insufficiency
Complication :
Infections : S.pneumoniae, chickenpox and measles
Thrombocytosis : Hypercoagulable state.

TREATMENT
1. Medication
1. STEROID
2. DIURETICS
3. IMMUNOSUPRESSIVE AGENTS
2. Dietary (nephrotic diet)
LOW SALT (1-2 g/day)
PROTEIN 2-3 g/kg/day

B.Corticosteroid

Prednisolon an active metabolit of

prednison.

Both have been widely used but

remains unclear whether their mode
of action is - anti-inflamatory,
- immunosupressive,
- or both

Initial Treatment
a. Introduction of remission
Prednisolon 60 mg/m2/day or 2 mg/kgBW/
day at least 4 weeks daily being required
b. Withdrawal : There are two alternative :
- Modified ISKDC regimen :
40 mg/m2 BSA on alternate days for 4
weeks
- Withdraw prednisolon gradually :
over 6-8 weeks or longer

STANDARD TREATMENT
CORTICOSTEROID (PREDNISON)
INITIAL TREATMENT
FULL DOSE

ALTERNATING

4 MINGGU

4 MINGGU

REMISSION (+)

REMISSION (-)

Prednison FD: 60 mg/m2/day

Prednison AD: 40 mg/m2/day

STEROID
SENSITIVE

STEROID RESISTANT

IMMUNOSUPRESSIVE AGENTS

THE INTERNATIONAL COMMITTEE OF KIDNEY DISEASE IN CHILDREN (1967)

Acute complication of nephrotic syndrome

Without steroid therapy

Bacterial infection
Hypovolemia
Hypercoagulability (thromboembolic
phenomena)
Respiratory embarrassment

With steroid therapy

Bacterial infection
Hypovolemia
Hypercoagulability
Respiratory embarrassment
Hypertension
Altered behavior
Steroid withdrawal (benign intracranial
hypertension)

Indications for hospital admission or

patient with nephrotic syndrome
Newly diagnosed patiens
Severe dehydrations (poor intake,
persistent vomiting)
Unexplained fever (suspected bacterial
infection)
Refractory edema (respiratory distress)
Peritonitis
Renal insufficiency (elevated serum
creatinine)

Outcome
Mortality
The mortality rate for SRNS is 1 to 2,5 %
usually from sepsis, hypovolemia, and
thrombocytosis.
Relapses
In most cases the relapses eventually
cease
The earlier the onset of SRNS, the more
likely that the disease will be protracted

Definitions
Remission
Urinary protein < 4 mg/ m2hr or Albustix =
0/Trace for 3 consecutive days

Steroid Responsive
Remission with steroids alone

Relapse
Urinary protein > 40 mg/m2*hr or Albustix
> 2+ for 3 consecutive days

Frequent Relapses
Two or more relapses within 6 months of
initial response or 4 or more relapses within
any 12 month period

 Steroid Dependence
Two consecutive relapses occurring
during corticosteroid treatment or within
14 days of its cessation

Steroid Resistance
Failure to achieve response in spite of 4
weeks of prednisone 60 mg/m2*day

THE CLINICAL RESPONS OF MINIMAL CHANGES

PATIENTS TO STEROID (ISKDC)

Minimal Change 100%

Responsive 93%

Norelaps
36%

Infrequent
Relapser
18%

Frequent
Relapser
39%

Non responsive
5%

Early Non responsive 7%

Late responsive
5%

Non-responsive
2%

(Kidney Int. 13-43, 1978)

PROGNOSIS
RENAL FUNCTION
failure

rapid, about
5 10 years

gradually