NEPHROTIC SYNDROME

DEFINITION

NEPHROTIC SYNDROME (NS) IS
CHARACTERIZED BY PROTEINURIA,
HYPOALBUMINEMIA,
EDEMA, HYPERCHOLESTEROLEMIA.
CLASSIFICATION OF NS

A. ETIOLOGY
1. PRIMARY NS
2. SECONDARY NS

B. STEROID THERAPY
1. STEROID RESPONSE
2. NON STEROID RESPONSE

c. HISTOPATHOLOGY
1. MINIMAL CHANGE
2. GLOMERULOSCLEROSIS
- FOCAL SEGMENTAL
- FOCAL GLOBAL

3. MESANGIAL PROLIFERATIF GLOMERULONEFPHITIS
DIFUS
4. KRESENTIK GLOMERULONEPHRITIS
5. MEMBRANOUS PROLIFERATIF GLOMERULONEPHRITIS
6. MEMBRANOUS GLOMERULOPATHY
7. CHRONIC GLOMERULO NEPHRITIS
ETIOLOGY
ETIOLOGY AND PATHOGENESIS IS
REMAIN UNCLEAR
IMMUNOLOGIC MECHANISMS

PATHOPHYSIOLOGY
PROTEINURIA INCREASE OF
GLOMERULAR PERMEABILITY


PROTEIN EXCRETION > 40 MG/HOUR/M2
LOSS OF VARIOUS PROTEINS
MINIMAL NS --> SELECTIVE LOSS OF
NEGATIVE CHARGE --> DECREASE OF T CELL
FUNCTION
NON MINIMAL NS --> LOSS OF NEGATIVE
CHARGE AND ALTERATION OF PORE SIZE
NEGATIVE CHARGE --> PROTEOGLICANS
SIALOPROTEIN


HYPOALBUMINEMIA
FILTRATION OF ALBUMIN
IMPAIRMENT OF ALBUMIN TUBULER
REABSORPTION
ALBUMIN < 3,0 G/DL --> HYPOALBUMINEMIA
< 2,7 G/DL --> EDEMA
< 1,2 G/DL --> SEVERE HYPOVOLEMIA

ORTOSTATIC HYPOTENSI :
ABDOMINAL DISCOMFORT,
VOMITE, DIARRHOEA
Ig G & -1 GLOBULIN Ig M, -2-GLOBULIN,
GLOBULIN, IG E,
FIBRINOGEN
EDEMA
EDEMA IS THE MAIN SIGN AND SYMPTOMS
EDEMA OF EYELID IN THE MORNING
PITTING EDEMA ON FOOT, ASCITES, PLEURAL
EFFUSION, SCROTAL & LABIA EDEMA
(ANASARCA EDEMA) --> ALBUMIN < 1,5 GR/DL
80% ONCOTIC PRESSURE PLASMA --> ALBUMIN
UNDERFILLED & OVERFILLED THEORY
HYPERLIPIDEMIA

ESPECIALLY MINIMAL CHANGE LESSION
CORRELATION OF HYPERLIPIDEMIA &
HYPOALBUMINEMIA
LIPOPROTEIN SYNTESIS HYPOALBUMINEMIA
ONCOTIC PRESSURE
LIPID CLEARENS
CLINICAL MANIFESTATION

EDEMA
ACUTE RESPIRATORY TRACT INFECTION
DIURESIS , TURBID
NORMOTENSION
TRANSIENT MICROSCOPIC HEMATURIA
SEVERE HYPOVOLEMIA & PERITONITIS -->
ABDOMEN DISCOMFORT
LABORATORY
A. URINE
MASSIVE PROTEINURIA : > 40 MG/HOUR/M2
> 50 MG/ 24 HOURS/KG
> 2 GR/24 HOURS
PROT CREAT RATIO > 2,5
PROTEIN SELECTIVITY:
RATIO (U TRANSFERRIN) : (P Ig G)
(U Ig G) : (P TRASNFERRIN)
< 0,1 SELECTIVE
> 0,2 NON SELECTIVE
HEMATURIA 15%


B. BLOOD : HYPOALBUMINEMIA,
HYPERLIPIDEMIA

DIAGNOSE
A. EDEMA
B. SEVERE PROTEINURIA ( PROTEINURIA +3)
C. HYPOALBUMINEMIA < 2,5 G/DL

THERAPY
DIETETICS
ALBUMIN & DIURETICS
SEVERE HYPOVOLEMIA, DISPNEA, SEVERE
EDEMA ON SCROTUM & LABIA
DOSE 0,5 - 1 G/KG WEIGHT IV ON 2-4 HOUR,
FOLLOWED BY FUROSEMIDE 1-2 MG/KG
WEIGHT.
ALBUMIN CAN BE GIVEN 2 X


CORTICOSTEROID
TBC (-) --> PREDNISON :
4 WEEKS I : 60 MG/M2/DAY DIVIDED 3-4 DOSE.
MAX 80 MG/DAY
4 WEEKS II : 40 MG/M2/DAY SINGLE DOSE
INTERMITEN OR ALTERNATE
REMISSION --> TAPPERING OFF EVERY WEEK --
> 30 MG, 20 MG, 10 MG
ALTERNATIVE THERAPY
RELAPSES FREQUENT, STEROID RESISTANT,
STEROID DEPPENDENT OR STEROID TOXICITY


CYCLOPHOSPHAMIDE 2-3 MG/KG WEIGHT/DAY
SINGLE DOSE 8 - 12 WEEKS ~ PREDNISON
ALTERNATE DOSE
LEUCOPENIA < 3000/MM3 --> STOP
LEVAMISOLE 2 -3 MG.KG WEIGHT/DAY
INTERMITEN 6 - 18 MONTHS
CYCLOSPORIN A 4- 5 MG/KG WEIGHT/DAY MIN
1 YEAR
CHLORAMBUCIL 0,15-0,2 MG/KG WEIGHT/DAY 8
WEEKS
COMPLICATIONS
INFECTIONS
THROMBOSIS
ACUTE RENAL FAILURE

PROGNOSIS
STEROID RESPONSIVE NS --> RELAPSES
FREQWENT--> SPONTANEUS REMISSION IN 20
YEARS OLD
MINIMAL CHANGE
25% ~ 1 X RELAPS
1/3 ~ INFREQUENT RELAPSES
1/2 ~ STEROID DEPENDENT

RELAPSES ~ FIRST ATTACK > 6 YEARS
END STAGE RENAL DISEASE ~ FREQUENT
RELAPSES
ACUTE NEPHRITIS SYNDROME
DEFINITION
Syndrome : - Hematuria
- Hypertension
- Edema
- Renal insufficiency
ETIOLOGY & EPIDEMIOLOGY
- Immune complex
- Most common 2-10 years old
- Ratio M : F = 2 : 1
- Incidence : 1-20 %
- E/ : Post group A - hemolytic streptococcal infection
(nephritogenic) induced respiratory (M12),
skin lesion (M49)
- Other E/ : Streptococcus, Pneumococcus, Klebsiella,
Meningococcus, Mycoplasma pneumoniae,
HIV, Coxsackie virus, Hepatitis, Measles.
PATHOGENESIS
- Unknown
- maybe related immune complex
CLINICAL MANIFESTATION
- URTI (4-5 days laten periode - 3 weeks, mean 10 days)
- Skin infection/ pioderma ( 10 days laten periode)
- Edema
- Hematuria
- Fever, weakness, abdominal pain, anorexia, headache, pallor
- Hypertension (5 % H. Encephalopathy)
- Circulatory congestion
DIAGNOSTIC
Clinical manifestation and Laboratory
COMPLICATION
1. Acute Renal Failure
2. Congestive : heart failure, lung edema
3. Hypertension
4. Electrolyte impaired : hyperkalemia, hypocalcemia
5. Acidosis
6. Uremia : seizure, coma

LABORATORY
Urine : decrease amount, coca cola-colored
micros : ery (+), leukocyturia,proteinuria ( +2)
Renal function : GFR ,ureum & creatinin , azotemia
Blood : normochrome normocyter anemia
Culture nasopharynx
Serologic: ASTO
Immunologic : complement C3
TREATMENT
- Not specific
- Supportive and symptomatic :
1. Generally : rest, salt restriction
2. Antiobitics
3. Complication : heart failure/ Hypertension :
furosemide 1-2 mg/kg/dose iv
4. Seizure: diazepam 0,25-0,5 mg/kg/dose iv
5. Acute Renal failure : fluid restriction & diuretics
URINARY TRACT
INFECTION
UTI is the common term for conditions in
which there is growth of bacteria within the
urinary tract
Bacteriuria is presence of bacteria in
bladder urine
Growth of > 100.000 colony forming units
significant
AETIOLOGY
Escherichia coli (80-90% acute UTI)
(70-80% recurrent UTI)
Proteus, Staphylococcus epidermidis &
aureus, Enterococcus, Pseudomonas,
Klebsiella.
CLASSIFICATION
1. Clinical Problem
-Non complicated UTI (Non obstructive)
-Compilcated UTI (Obstructive)
- Abnormality urinary tract
- Abnormality immunology system
- Renal impairment
With or without symptom
- Symptomatic
Frequent syndrome
Acute pyelonephritis
Acute prostatitis
-Asymptomatic
PATHOGENESIS
1. Ascenderen (95%)
2. Hematogen (3%)
3. Lymphogen
LABORATORY
PYURIA
A. urine sedimen, leucocytes 5/HPF
B. urine leucocytes (non centrifuge)
2. BACTERIA IN URINE (non
centrifuge)
2 bact/10 HPF or 5 bact/HPF
3. CHEMICAL TEST
a. Nitrite test
b. Methylen blue reductase test
URINE SAMPLE LABORATORY
1. Perineal bag steril

2. Urine mid stream

3. Catheterization

4. Suprapubic aspiration
INTERPRETATION OF THE RESULT URINE
CULTURE
Sample Colony Culture UTI
Midstream >100.000 1 80%
>100.000 2 96%
Catheterization >100.000 1 95%
Supra pubic > 1 bact Gr - 1 99%
> 1000 bact Gr+ 1 99%
DIAGNOSIS
- SYMPTOMS

- PYURIA

- TRUE BACTERIURIA
CLINICAL PRESENTATIONS
- The symptoms of children with UTI
depend on the level of the infections as
well as the age of child
- Neonatal : anorexia, lethargy, feeding
difficults, body tenderness, hypothermia
- Infants : non toxic
- Childhood : classical symptoms

Management of Acute
Symtomatic
1. Eliminate infection
2. Establish clinical and microbiological
survailence to ensure
3. Prevent further infection
ANTI BACTERIAL
TREATMENT
Depend : culture & resistensi test

1. Bactericid & Bacteriostatic
2. No side effect
3.After treatment increase therapeutic level
4. Easy
5. No resistance
COMPLICATION
- Evaluate ---- Anomaly. VUR
and other obstruction

ren & CRF damage
If +
1. Prophylactic antibiotic
a. Complicated UTI, recurrence 3 X or
more in one year ( 1-2 years)
B. Uncomplicated UTI recurrence 3X or
more in one year (3-6 months)

Drugs : Nitrofurantoin, cotrimoxazol
Radiology : PIV, MSU, USG, Cyntigrahpy,
Cistography, Tomography computer, after
4-6 weeks no infections.
Urology intervention
HYPERTENSION
DEFINITION
BP = RVP X C
Normal BP = Syst & Diast < 90th percentile
Hypertension = Syst &/ Diast 95th percentile
Crisis Hypertension :
BP sudden , normotension/hypertension before
(Syst > 180 mmHg, Diast > 120 mmHg)
Hypertensive Encephalopathy :
BP sudden , intracranial pressure manifestation
result affected another organ, decline consciousness
CLASSIFICATION
Mild Hypertension :
Syst/ Diast < 10 mmHg over 95th percentile
(140/90 - 149/99)
Moderate Hypertension :
Syst/ Diast 10-20 mmHg over 95th percentile
(150/100 - 159/109)
Severe Hypertension :
Syst/ Diast > 20 mmHg over 95th percentile
(>160/110)


ETIOLOGY
- Primary H : underlying disease (-)
- Secondary H : underlying disease (+), 80 % children
PATHOGENESIS
Hypervolemia
Impaired of RAA System
Vasodilators deficiency
Cardiovascular disease
Endocrine impairment
Neurologic impairment
CLINICAL MANIFESTATION
Asymptomatic
Symptomatic
DIAGNOSTIC
- History
- Physical examination
- Supporting examination :
. Laboratory
. ECG
. IVP/ Renal ultrasound
TREATMENT
NON PHARMACOLOGIC :
- Dietary
- Weight loss
- Exercise
PHARMACOLOGIC :
- Diuretics
- Blocker
- Vasodilators
- ACE inhibitors
- Ca glukonate
PROGNOSIS
Essential H : adequate treatment incidence :
renal failure, stroke,
congestive heart failure
Secondary H : depends on underlying disease and
respons of therapy