Kuliah Glomerulopati

pada anak

dr. Muhammad Heru Muryawan, SpA(K)

Divisi Nefrologi Dept. IKA

FK Undip-RSUP dr Kariadi Semarang
 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

• Alport Syndrome
Congenital
• Congenital Nephrotic Syndrome

1. Minimal change (MCNS)

2. Focal segmental glomerulosclerosis
Primary/ 3. Mesangial proliferative glomerulonephriti
4. Membrano-proliferative glomerunephritis
idiopathic
5. Membranous glomerulonephritis
6. IgA Nephropathy
7. Glomerulonephritis others
Acquired
• Post Infection – Post streptococcal
glomerulonephritis
Secondary • Mulisystem diseases: LE, HUS
• Intoxicaation: drugs, metal
• Neoplasms
Nephrotic Syndrome
 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
• Heriditery nephritis
• Drugs,toxins,infections
Classification of Nephrotic syndrome

Response to
Histopathology:
steroid

Sensitive Minimal

Non minimal
Resistant  Mainly
FSGS
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

(MPGN)
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 Nephrotic edema
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
Prednison FD: 60 mg/m2/day
Prednison AD: 40 mg/m2/day

REMISSION (+) STEROID SENSITIVE

REMISSION (-)

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% Early Non responsive 7%

No- Infrequent Frequent Late responsive Non-responsive

relaps Relapser Relapser 5% 2%
36% 18% 39%

Non responsive
5%

(Kidney Int. 13-43, 1978)

PROGNOSIS

RENAL FUNCTION gradually

failure

rapid, about
5 – 10 years