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Glomerulopati
Glomerulopati
Mahasiswa FK Undip
CLINICAL MANIFESTATIONS
Isolated proteinuria
Proteinuria + edema (i.e.Nephrotic syndrome)
Isolated haematuria
Hypertension +/- proteinuria/haematuria
Renal failure
Classification
Alport Syndrome
Congenital Congenital Nephrotic Syndrome
fetoprotein
before 20 weeks gestation
Classification :
Primary
Finnish type
Diffuse mesangial sclerosis
Minimal changes NS
Secondary
congenital syphilis, toxoplasmosis,
cytomegalovirus
XY gonadal dysgenesis and Wilms tumour
nephroblastoma
CONGENITAL NEPHROTIC SYNDROME
the majority of cases
Prognosis : infaust
SECONDARY NEPHROTIC SYNDROME
Causes of secondary nephrotic syndrome
Hepatitis B Syphilis
2. Infections Malaria Filariasis
Leprosy Schistosomiasis
1.PROTEINURIA
2.PERMEABILITY IMPAIRMENT
MOLECULE IONIC CHARGE
MOLECULE SIZE
Glomerular Capillary Membranes
Mechanism of Proteinuria
A SIZE-SPECIFIC BARRIER
A CHARGE-SPECIFIC BARRIER
CLINICAL MANIFESTATIONS
Oedema (uptill 40% BW), ascites, hydrothorax,
scrotal oedema
Secondary infections : skin, peritonitis
Anaemia
Growth disturbances
Tetany (hypocalcaemia)
Hypovolemic shock
Vein thrombosis
Acute renal failure: oliguria/anuria,
metabolic acidosis,potassium
Nephrotic syndrome
Generelised edema
(anasarca)
Older child with
nephrotic syndrome
Pitting peripheral
oedema
Nephrotic Syndrome
Ascites
Nephrotic syndrome
Urinary analysis:
specific gravity , pH
proteinuria massive
(selective - albumin 85-95%)
qualitative/semiquantitative > 2+
quantitative : Esbach
leukocyturia
haematuria
double refractile lipoid bodies
hyaline cast
Plasma :
Hb , Ht
hypoalbuminaemia, reverse ratio alb/glob
hypercholesterolaemia
normal: ureum, creatinine
IDIOPATHIC NEPHROTIC SYNDROME
1. Minimal change
2. Focal segmental glomerulosclerosis
3. Proliferative glomerulonephritis
Mesangial
With crescent formation
Focal
Diffuse exudative
Mesangiocapillary (membrano-proliferative)
4. Membranous glomerulonephritis
5. Advance chronic glomerulonephritis
EPIDEMIOLOGY
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
3. OPTIMIZING CONDITION
(physic,psychology,social)
- Activity : not limited
- Immunization: as scheduled
- Psychological support : the child + parents
FOLLOW UP
OUT PATIENT CLINIC:
- Symptomatic : weekly - monthly
- Asymptomatic : every 3-6 months (renal
function evaluation)
ADMISSION :
generelized oedema, severe hypertension,
severe infection, shock, acute renal failure
STANDARD TREATMENT
CORTICOSTEROID (PREDNISON)
INITIAL TREATMENT
4 MINGGU 4 MINGGU
Prednison FD: 60 mg/m 2/day
Prednison AD: 40 mg/m 2/day
STEROID RESISTANT
IMMUNOSUPRESSIVE AGENTS
CICLOPHOPHAMIDE
2-3 mg/kg/day for 8 12 weeks in combination with
steroid intermittent
CHLORAMBUCILE
0,1-0,2 mg/kg/day in divided dose with steroid AD
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)
rapid, about 5
10 years
SECONDARY
GLOMERULONEPHRITIS
DEFINITION
GROUP A
Beta-hemolytic
Respiratory tract M 1,2,4,12,18,25
Skin M 49, 55, 57, 60
GROUP C
Streptococci
Streptococcus zooepidermicus
Site of infection:
upper respiratory tract: pharynx, tonsilles, middle ear
skin
Upper Respiratory
Sore Throat
Tonsillar exudate
Fever
Chills
20% school children
carriers
Skin
Impetigo
Lesions on
extremities
Commonly on face
Pustular and crusty
INFECTIONS PRECEDING ACUTE GN
BACTERIAL
Group A, Beta-hemolityc streptococci
Streptococcus viridans
Streptococcus pneumoniae
Streptococcus grup C (Streptococcus zooepidermicus)
Staphylococcus aureus
Staphylococcus epidermidis
Salmonella typhosa
Gonococcus
Mycoplasma
Staphylococcus albus
Treponema pallidum
Corynebacterium bovis
Klebsiella pneumoniae
Diplococcus pneumonia
Brucella suis
Meningococcus
Leptospira
Propionibacterium acnes
Mycobacterium leprae
Actinobacillus
INFECTIONS PRECEDING ACUTE GN
VIRAL
Varicella-zantu - Coxsackie B
Rubella - Echovirus
Cytomegalovirus - Enterovirus
Epstein-Barr - Picornavirus
Hepatitis B - Onconavirus
Measles - Influenza
Mumps - HIV
PARASITIC
Plasmodium falsiparum - leishmanias
Plasmodium malariae - tripanosomes
Toxoplasma gondii - trichinosis
Schistosoma mansoni - filaria
FUNGAL RICKETSIAL
Coccidiodes immitus - Scrub typhus
ASSOCIATION BETWEEN
AGN STREPTOCOCCI INFECTION
Latent period
# pharyngitis associated : 10 d
# impetigo associated : 21 d
Pathogenesis
HYPOTHESIS:
Gross hematuria 25 33 %
Volume overload
oedema 85 %
hipertension 60 80 %
circulatory congestion 20 %
CNS symptoms 10 %
LABORATORY FEATURES
URINALYSIS :
proteinuria 1 - 4+
hematuria
abnormal sediment:
dysmorphic RBCs, WBCs, cellular casts,
granular casts, RBC casts
SERUM :
- BUN/ureum , creatinine
- K , acidosis, hyperphosphatemia, Ca
- Hypocomplementemia
in first week, normal in 8 10 wks
- Properdin level
- Evidence of a recent streptococcal infection
antistreptozyme, ASO, antihyaluronidase, anti-
DNase B
Clinical and laboratory evaluation in acute GN
Light microscopy
DIFFUSE ENDOCAPILLARY PROLIFERATIVE
GLOMERULONEPHRITIS
- diffuse mesangial cell and matrix proliferation
- endothelial cell proliferation
- infiltration polymorphonuclear cells and monocytes
- occlusion capillary lumens
Immunofluorescence microscopy
irregular fine coarse granular staining in mesangium
and along capillary loop for IgG, C3, IgM, IgA,
Electron microscopy
- electron-dense deposits in mesangium
- HUMPS (large deposits in subepithelial location)
pathognomonic
DIAGNOSIS
Sudden onset of gross hematuria, oedema, hypertension
and acute renal failure following a recent streptococcal infection
DIFFERENTIAL DIAGNOSIS
IgA Nephropathy synpharyngetic hematuria
Associated with systemic disease
Chronic glomerulonephritis
TREATMENT (1)
1. Bed rest
2. Antibiotic for eradicating streptococci
- Procain Penicillin 10 days
- Erythromicyn
3. Dietetic (fluid & salt restriction)
- low protein 1 g/kgBW/day
- low salt 1 g/day
- IVFD as necesarry
4. Prolonged anuria dialysis
- peritoneal dialysis
- haemodialysis
TREATMENT (2)
5. Diuretics
Furosemide 1 mg/kgBW/dose 2x/ day
6. Symptomatic treatment
hypertension
hypertensive encephalopathy
congestive heart failure
acute renal failure
TREATMENT (3)
Treatment of hypertension associated with APSGN
Mild Severe
A clinicopathologic entity
Clinical manifestations:
similar to APSGN severe
rapidly deteriorating
Management:
steroid / immunosupressive agents : controversial
CHRONIC GLOMERULONEPHRITIS (1)
Laboratory findings
Urine : isostenuria
proteinuria
hematuria
leukocyturia
PATHOLOGY ANATOMY
MACROSCOPICALLY
- SHRINKED KIDNEY
- CONTRACTED KIDNEY
MICROSCOPICALLY
- HYALINE DEGENERATION
- TUBULUS ATROPHY
- NEPHRON REPLACED WITH FIBROID TISSUE
+ LYMPHOCYTE INFILTRATION
TREATMENT STRATEGY FOR ACUTE GLOMERULONEPHRITIS
Angiotensin II
PRA =plasma renin activity; AVP = arginine vasopressin; ANP=atrial natriuretic peptide