Nephrotic Syndrome

肾病综合征
Department of pediatrics
The first affiliated hospital
Sun Yat-Sen University
Yue Zhihui ( 岳智慧 )
Contents
1 Definition of NS

2 Pathophysiology of INS

3 Clinical manifestation & laboratory test

4 2012 年度市场推广策略
Complication

5 Treatment
2
 Key words
Proteinuria
Hypo-albuminemia
Hyper-cholesterolemia
Pitting edema
Minimal change nephropathy (MCN)
Corticosteroid ，
Contents
1 Definition of NS

2 Pathophysiology of INS

3 Clinical manifestation & laboratory test

4 2012 年度市场推广策略
Complication

5 Treatment
4
 Definition Glomerular permeability↑
Nephrotic
Syndrome

3 1
increase decrease

proteinuria Edema Hypo-

proteinemia
Hyper- 5

lipoproteinemia
 Classification
Secondary&
 Idiopathic NS 10% Congenital NS

 Secondary NS

SLE, HBV, anaphylactoi 1

2
d purpura
 Congenital NS 90%
Idiopathic NS
 Secondary NS : DIAMOND
Drug,Toxic,Allegy: mercury, snake venom, vaccine, pellicillamine,
Heroin,gold, NSAID, captopril, probenecid, volatile hydrocarbons
Infection: APSGN, HBV, HIV,shunt nephropathy, reflux nephropathy,
leprosy, syphilis, schistosomiasis, hydatid disease
Autoimmune or collagen-vascular diseases: SLE, Has
himoto’s thyroiditis, EMC, HSP, Vasculitis
Metabolic disease: Diabetes mellitus
Others: Chronic transplant rejection, congenital nephrosclerosis
Neoplasma: Hodgkin’s disease, carcinoma ( renal cell, lung, neurobla
stoma, breast, and etc)
Genetic Disease: Alport syn., Fabry syn., Nail-patella syn., Sickle c
ell disease, Amyloidosis, Congenital nephropathy
Congenital Nephrotic Syndrome
 Infants who develop nephrotic syndrome wi
thin the first 3 mo of life
Finnish type of congenital nephrotic syndro
me
Diffuse mesangial sclerosis
Primary nephrotic syndrome
Pathophysiology
Primary Nephrotic Syndrome
 Etiology
 Pathogenesis Something wrong
in the kidney
Contents
1 Definition of NS

2 Pathophysiology of INS

3 Clinical manifestation & laboratory test

4 2012 年度市场推广策略
Complication

5 Treatment
11
Pathophysiology of INS
Glomerular filtration barrier
 Size-selective (aperture) barrier
 Charge-selective barrier
Glomerular filtration barrier
aperture barrier
slit diaphragm

Endothelium
Charge-selective barrier

Endothelium
Charge-selective barrier

Endothelium
Absence of proteinuria
Selective proteinuria
Nonselective proteinuria
Nonselective proteinuria
 Disturbence of cellular immunity function
 IG & C deposit in glomeruli
 heredity
HLA-DR7 steroid sensitive
HLA-DR9 frequent relapse
 Pathophysiology
hyperlipidemia
massive proteinuria
Lipoprotein lipase↓
Lipoproteins synthesis↑
Hypoproteinemia
Intravascular volume↓

Plasma oncotic pressure↓

RAA(aldosterone)↑
ADH↑
Fluid

Interstitial space Edema Watersodium

retention
Pathological changes in INS
 minimal change disease (MCD)
 mesangial proliferative glomerulonephri
tis (MsPGN)
 focal segmental glomerulosclerosis (FSG
S)
 membranous nephrosis (MN)
 membranoproliferative glomerulonephri
tis (MPGN)
Pathological patterns of INS

1.4
2.3
1.5
6.9
7.5 76.4%
MCD
Focal segmental glomerulosclerosis (FSGS)
Clinical manifestation
Contents
1 Definition of NS

2 Pathophysiology of INS

3 Clinical manifestation & laboratory test

4 2012 年度市场推广策略
Complication

5 Treatment
35
 Clinical manifestation
Epidemiology
Incidence, sex and age
Main symptoms and signs
Genaral situation
Edema 、 ascites 、 pleural effusion
Urine, hematuria
Blood pressure
Renal function
 Laboratory tests
 Urinalysis
protein 3+-- 4+.
protein > 50
mg/kg.d
 Laboratory tests
Serum
albumin < 25mg/L cholesterol < 5.72mmol/L
 Laboratory tests
Urine
• Urinalysis, 24h urinary protein excret
ion, urinary Pro/Cr
Serum
• albumin, cholesterol, triglyceride
• IgG, IgA, IgM, C3
• BUN, Cr
• sodium, potassium, calcium
Ultrasonography
renal biopsy
 Clinical types
Simple type and Nephritic type
• hematuria
urinary RBC≥10/HPF
• hypertension:
preschool age child≥120/80mmHg
school age child≥130/90mmHg
• Azotemia: renal function insufficient
• hypocomplementemia
Contents
1 Definition of NS

2 Pathophysiology of INS

3 Clinical manifestation & laboratory test

4 Complication
2012 年度市场推广策略

5 Treatment
41
 Complications
• Infection
• Electrolyte disorder, Hypovolemia
• Hypercoagulability and thrombosis
• Acute renal failure
• Renal tubular function disorder
•Growth retardation
 Infection
• Manifestations
• URI, spontaneous peritonitis, tuberculosis, cellul
itis, urinary tract infection
• Cause
• Immunoglobulin and complement factor↓
• protein malnutrition, edema,
• immunosuppressive therapy
• Management and Prophylaxis
• high index of suspicion, prompt evaluation
• early initiation of therapy
• polyvalent neumococcal vaccine
 Electrolytes disorder & hypovolemia
• Manifestations
• Hyponatremia, hypokalemia, Hypocalcemia
• Hypovolemic shock
• Cause
• salt intake restriction
• diuretic treatment
• vomit, diarrhea, intestinal reabsorbtion
• Loss of calcium binding protein
• Prophylaxis
• Avoiding aggressive diuretic therapy
• Inappropriate salt intake restriction
 Hypercoagulability and thrombosis

Manifestations
• thrombosis within kidney, extremities, brain an
d lung
Cause
• coagulation factorsⅡ , Ⅴ , Ⅶ , Ⅷ , Ⅹ ↑, platelet a
ggregation↑, antithrombin Ⅲ↓
• Hyperlipidemia, diuretic and steroid therapy
prophylaxis
• Avoiding puncture of deep veins
• Prophylactic anticoagulation drugs
 Acute renal failure
Manifestations
• Oliguria or anuria ， hypertension
• Elevated serum Cr and BUN levels
Cause
• Intravascular blood volume↓
• Obstruction, crescent formation
• Acute interstitial nephritis, drugs
Prophylaxis
• Avoiding use of renal toxic drugs
• Avoiding aggressive diuretic therapy
 Renal tubular function disorder

Manifestations
• polyuria, nocturia, Glucosuria, aminoaci
duria, Fanconi syndrome
Cause
• Progress of the glomerular disease
• Persistent massive proteinuria
prophylaxis
• avoiding excessive albumin transfusion
 Diagnosis sequence

 Nephrotic syndrome ？
 Primary, secondary or congenital ？
 Simple type or nephritic type ？
Contents
1 Definition of NS

2 Pathophysiology of INS

3 Clinical manifestation & laboratory test

4 2012 年度市场推广策略
Complication

5 Treatment
50
 General treatment
 Rest
 Diet sodium, protein, calcium and vita
min D
 Prevention and treat infection
 Diuresis
 Education of the family
Anticoagulant therapy
 Heparin, Persantine
Steroid
 Prednisone, prednisolone
Immunosupressive drugs
 CTX, CsA, FK506
Others
 ACEI, Immunologic regulators, Ch
inese herb medicine
Corticosteroid therapy
 Scheme
 Short range
 Intermediate range

 Long range
Prednisone 1.5-2 mg/kg/d*6-8w

Prednisone 2mg/kg qod*4w

Prednisone dose (every 2-4w)

Course of treatment

6m 9m
Intermediate long
Side effects of corticosteroid
 Metabolic disturbance
 Hypertension

 Infection, peptic ulcer

 Euphoria, lunacy, induce epilepsy, inso

mnia
 Osteoporosis, growth retardation

 Cataract

 abuse syndrome and adrenal insufficien

cy
classification on curative effects
steroid responsive
steroid resistant / insensitive
steroid dependent
frequent relapse
Indications for cytotoxic drugs
 frequent relapse
 steroid dependent

 steroid resistant

 unable to tolerate steroid treatment

 Side effects of CTX
 Leukopenia
 Alopecia

 Hepatic function disorder

 Hemorrhagic cystitis

 Sterility

 Disseminated varicella infection

 Inappropriate ADH secretion

Others
 ACEI, Immunologic regulators, Chinese med
icine
 Prognosis
• Relapse and resolve

• Prognosis is depend on pathologic

patterns
Summary

3 increase
1 decrease
 Nephrotic
Syndrome

3 1
increase decrease

Hypo-
proteinuria Edema proteinemia
Hyper-
62
lipoproteinemia
 Case presentation
A 5 years old male child was admitted to our hospital for
progressed edema and foamy urine for 1 week. 3 days bef
ore edema appearance, he suffered from fever, cough and r
unning nose. On examination patient had generalized anas
arca. Vitals were normal. Systemic examination revealed a
scites and pleural effusion. Biochemical investigations sho
wed serum cholesterol8.7mmol/L, total serum proteins 43
g/l, Albumin 16 g/l, Globulins 27 g/l. Urine proteinwas ++
+, RBC +++,electrolytes and urea were normal.
Diagnosis? Differential diagnosis? Treatment?
 Questions
1.The definition of nephrotic syndrome.
2.Describ the main clinical types of INS .
3.the diagnostic criteria of INS.
4.The most common pathological types of INS .
5.The major complications of INS.
6.The general treatment principles of INS .
7.How to evaluate the response of steroid therapy
？
8. Both NS and APGN patients suffer from edema,
what is the difference?
 Reference Books & website
 Brenner & Rector's: The Kidney, 2000 Edited by Barry M. Bren
ner
 Kliegman RM. Nephrotic Syndrome. From: KliegmanRM ， et
al. Nelson Textbook of Pediatric. 18th ed. Philadelphia ： WB S
aunders ， 2007.
 杨霁云 白克敏主编 . 小儿肾脏病基础和临床 . 人民卫生
出版社， 2002
 Pub Med http://www.ncbi.nlm.nih.gov
 中国知识资源总库 http://dlib.cnki.net
 岳智慧 yuezhihui810@ya
hoo.com.cn
 儿科教研室 http://jpkc.sysu.e
du.cn/erke/
Energy and persistence
conquer all things.
-- Benjamin Franklin