PediatricPediatric Renal Diseases Renal Diseases

Developmental andDevelopmental and

Physiological Aspects Physiological Aspects

1.1. Urine volume: Urine volume:

Newborns 1~3 ml/kg/h Newborns 1~3 ml/kg/h

3~10 d 100~300 ml/d 3~10 d 100~300 ml/d

~2 m 250~400 ml/d ~2 m 250~400 ml/d

~1 y 400~500 ml/d ~1 y 400~500 ml/d

~3 y 500~600 ml/d ~3 y 500~600 ml/d

~5 y 600~800 ml/d ~5 y 600~800 ml/d

~8 y 600~1000 ml/d ~8 y 600~1000 ml/d

~14 y 800~1400 ml/d ~14 y 800~1400 ml/d

>14 y 1000~1600 ml/d >14 y 1000~1600 ml/d

▲▲Oliguria (low urine output): Oliguria (low urine output):

Newborns < 1ml/kg/h Newborns < 1ml/kg/h

Infant & infancy <200ml/m Infant & infancy <200ml/m22/d/d

Pre-school age <300ml/m Pre-school age <300ml/m22/d /d

School age <400ml/m School age <400ml/m22/d /d

▲▲Anuria: < 50 ml/mAnuria: < 50 ml/m22/d /d

(newborns < 0.5 ml/kg/h) (newborns < 0.5 ml/kg/h)

2.2.  Routine urine test Routine urine test

2.1. 2.1. Urine color--normally yellow,Urine color--normally yellow,

color changes may be normal color changes may be normal

or abnormal or abnormal

2.2. 2.2. PH: normal range 5~7PH: normal range 5~7

2.3. 2.3. Specific gravity Specific gravity

newborns – 1.006~1.008 , newborns – 1.006~1.008 ,

>1 year old – 1.011~1.025 >1 year old – 1.011~1.025

2.4. 2.4. Urine analysis – freshly collectedUrine analysis – freshly collected

and centrifugal urine and centrifugal urine

● ● RBC < 3/hpfRBC < 3/hpf

● ● WBC < 5/hpfWBC < 5/hpf

● ● Casts–cellular (Casts–cellular (RBC, WBCRBC, WBC) and ) and

granular casts are abnormal, granular casts are abnormal,

hyaline casts can be normal hyaline casts can be normal

●● Crystals – phosphate andCrystals – phosphate and urate crystals may be normalurate crystals may be normal ●● Protein(Pro) – negative Protein(Pro) – negative ●● Sugar (Glu)Sugar (Glu) ●● Ketones (Ket)Ketones (Ket) ● ● Urobilinogen (Uro)Urobilinogen (Uro) ●● Bilirubin (Bil)Bilirubin (Bil)

3. 3. Addis countAddis count RBCRBC < 50,0000, < 50,0000, WBCWBC <1,000,000, <1,000,000, CastsCasts < 5000 < 5000 4. 4. 24h total urinary protein 24h total urinary protein less than 100 mg/m less than 100 mg/m22/d,/d, or <4 mg/m or <4 mg/m22/h, /h, or <100 mg/L, or <100 mg/L, or <150 mg/d or <150 mg/d

5. 5. Renal function tests: BUN, CrRenal function tests: BUN, Cr

6. 6. Imaging procedures Imaging procedures

X-ray, Ultrasound, VCUG, X-ray, Ultrasound, VCUG,

Nuclear medicine ( Nuclear medicine (99m99mTc DMSA, Tc DMSA,

99m99mTc DTPA),Tc DTPA),

IVP etc. IVP etc.

7. 7. Renal BiopsyRenal Biopsy

Glomerular Glomerular

Diseases Diseases

ClassifyClassify

▲▲Clinical classifyClinical classify 1. Primary glomerular diseases 1. Primary glomerular diseases

1.1. Glomerulonephritis 1.1. Glomerulonephritis

(Nephritis) (Nephritis)

﹉﹉ Acute glomerulonephritisAcute glomerulonephritis

  

﹉﹉ Rapidly progressive glomerulRapidly progressive glomerul

o-o-

nephritis (RPGN) nephritis (RPGN)

    ﹉﹉ Persistent glomerulonephritis Persistent glomerulonephritis

﹉﹉ Chronic glomerulonephritisChronic glomerulonephritis

  

﹉﹉ Rapidly progressive glomerulRapidly progressive glomerul

o-o-

nephritis (RPGN) nephritis (RPGN)

    ﹉﹉ Persistent glomerulonephritis Persistent glomerulonephritis

﹉﹉ Chronic glomerulonephritisChronic glomerulonephritis

1.2. Nephrotic syndrome (NS) 1.2. Nephrotic syndrome (NS)

﹉﹉ Simple tape NSSimple tape NS

﹉﹉ Nephritic tape NS Nephritic tape NS

1.3. Asymptomatic (isolated) 1.3. Asymptomatic (isolated)

hematuria or proteinuria hematuria or proteinuria

1.4. Familial nephritis 1.4. Familial nephritis

2. Secondary glomerular 2. Secondary glomerular

diseases– it is part of mul- diseases– it is part of mul-

tisystem disorder, e.g. – tisystem disorder, e.g. –

2.1. Hepatitis B virus related2.1. Hepatitis B virus related

glomerulonephritis (HBV-G glomerulonephritis (HBV-G

N)N)

2.2. 2.2. Purpuric nephritisPurpuric nephritis

2.3. 2.3. Lupus nephritis (LN)Lupus nephritis (LN)

▲▲ Pathologic classify Pathologic classify

▲▲Immunopathology classifyImmunopathology classify

Acute Acute

GlomerulonephritisGlomerulonephritis

(AGN) (AGN)

DefinitionDefinition Glomerulonephritis is aGlomerulonephritis is a

various group of diseases– acute various group of diseases– acute

nephritic syndrome. nephritic syndrome.

★★Acute poststreptococcal Acute poststreptococcal

glomerulonephritis, glomerulonephritis, APSGNAPSGN

(acute nephritis)(acute nephritis)

●● Incidence age: in 5 ~14 years oldIncidence age: in 5 ~14 years old

● ● peak age: 3~7 years old peak age: 3~7 years old

●● Boys > girls = 2:1 Boys > girls = 2:1

●● Incidence peak: Jan. Feb. Incidence peak: Jan. Feb.

Sep. and Oct. Sep. and Oct.

Etiology & PathogenesisEtiology & Pathogenesis

●● Bacterial: Bacterial: ▲ ▲ group Aβ- group Aβ-

hemolytic streptococci,hemolytic streptococci,

Staphylococci,Staphylococci,

Pneumococci, Pneumococci,

G Gˉ ˉ bacillibacilli

● ● Viral: influenza virus,Viral: influenza virus,

mumps virus , Coxsackie mumps virus , Coxsackie

virus, ECHO virus and virus, ECHO virus and

EBV EBV

● ● Other pathogens Other pathogens

fungi etc. fungi etc.

The immunoreaction caused bThe immunoreaction caused b

y group Aβ- hemolytic strep-y group Aβ- hemolytic strep-

tococcitococci －－ nephritogenic strannephritogenic stran

ss

  

  

  

  

  

Circulating immunecomplexesCirculating immunecomplexes

(CIC) (CIC)

Antigens+antibodiesAntigens+antibodies

  

In situ immunecomplexes In situ immunecomplexes

→ →deposited on glomerular deposited on glomerular

capillaries capillaries →→ complement complement

system activated system activated→→immuneimmune

mediators and inflammatory mediators and inflammatory

mediators mediators

PathologyPathology1. 1. The feature of pathological The feature of pathological

changes: Diffus, exudative and changes: Diffus, exudative and

proliferative inflammation of proliferative inflammation of

the glomerulus the glomerulus

2.2. Chief variety Chief variety

Endothelial and mesangial cellsEndothelial and mesangial cells

proliferation with leukocyte proliferation with leukocyte

infiltration; immunofluorescence infiltration; immunofluorescence

shows granular IgG & C shows granular IgG & C33 deposits deposits

Electron microscopy Electron microscopy ★★HumHum

p-like electron densep-like electron dense

deposits on epithelial side deposits on epithelial side

of GBM of GBM

Pathophysiology (Figure)Pathophysiology (Figure)

Infection of streptococciInfection of streptococci

Immune complexesImmune complexes

Local immune inflammation Local immune inflammation in glomerular capillaries in glomerular capillaries

Stenosis of blood Glomerular filtration Stenosis of blood Glomerular filtration capillary cavity membrane injury capillary cavity membrane injury GFR ↓ Hematuria GFR ↓ Hematuria Proteinuria Proteinuria Oliguria Cylindruria Oliguria Cylindruria Blood volume↑ Blood volume↑ Venous pressure↑ Venous pressure↑

Edema Circulatory load↑ Edema Circulatory load↑ Hypertension Hypertension

Clinical ManifestationsClinical Manifestations●● Prodromal infections Prodromal infections

pharyngitis, scarlet fever, pharyngitis, scarlet fever,

Angina, and pyoderma Angina, and pyoderma● ● Incubation period: about 10Incubation period: about 10

days for pharyngitis, 14~20 days for pharyngitis, 14~20

days for skin infection days for skin infection

1.1. Typical findings (general Typical findings (general

case) case)

1.1. 1.1. Ordinary symptoms:Ordinary symptoms:

low grade fever, nausea, low grade fever, nausea,

debility, malaise, anorexia debility, malaise, anorexia

and vomiting, etc. and vomiting, etc.

1.21.2 Principal symptoms Principal symptoms

(nephric signs) (nephric signs)

a. Edemaa. Edema (nonpiting (nonpiting

edema, nephritic edema) edema, nephritic edema)

Edema is the most common Edema is the most common

initial sign– initial sign– Periorbital edemaPeriorbital edema

OliguriaOliguria may be present may be present

b. Hematuriab. Hematuria

Microscopic ~Microscopic ~ (most of cases) – (most of cases) –

>5/hpf, >5/hpf,

Gross~Gross~ (1/3~1/2 cases) – usually (1/3~1/2 cases) – usually

tea or cola colored (brownish) tea or cola colored (brownish)

urine, continue 1~2 w urine, continue 1~2 w

肉眼血尿肉眼血尿

c.Hypertension:c.Hypertension: 1/3~2/3 cases1/3~2/3 cases

Pre-school age>120/80mmHg Pre-school age>120/80mmHg

School age>130/90 mmHg School age>130/90 mmHg

Headache may be present Headache may be present

d. d. Proteinuria:Proteinuria: <3+ <3+

2.2. Severe findings (Severe case) Severe findings (Severe case)

Appear the following symp- Appear the following symp-

toms within 2 w of the onset. toms within 2 w of the onset.

a. Circulatory congestiona. Circulatory congestion

RR↑, HR↑, fidget , hepa-RR↑, HR↑, fidget , hepa-

tomegaly tomegaly→→→→dyspnea,dyspnea,

jugular phlebectasia, jugular phlebectasia,

pulmonary edema, gallop pulmonary edema, gallop

rhythm and cardiac dilation rhythm and cardiac dilation

Chest X-ray:

Enlarged cardiac silhouette,

lung markings coarsen

(pulmonary vascular

congestion)

b.b. Hypertensive encepha-Hypertensive encepha-

lopathy lopathy

BP↑ BP↑→→brain hypoxiabrain hypoxia

and edema and edema

Smart headache, nausea, Smart headache, nausea,

vomiting and d vomiting and d

iplopia or transient blindnessiplopia or transient blindness

→→

convulsion, comaconvulsion, coma

●●Hypertensive crisisHypertensive crisis

c. Acute renal insufficiencyc. Acute renal insufficiency

Severe oliguria or anuria→ Severe oliguria or anuria→

temporary azotemia, distur- temporary azotemia, distur-

bance of electrolytes and bance of electrolytes and

metabolic acidosis metabolic acidosis

3.3. Atypical findings Atypical findings

(Atypical case) (Atypical case)

▲▲Extrarenal symptomatic Extrarenal symptomatic

nephritis nephritis

▲▲ Acute nephritis with neph- Acute nephritis with neph-

rotic manifestation rotic manifestation

▲▲ Asymptomatic AGN Asymptomatic AGN

Laboratory investigationsLaboratory investigations1.1. Routine urinalysisRoutine urinalysis

RBC↑, 2 RBC↑, 2++~ 3~ 3++, > 5/hpf,, > 5/hpf,

protein 1 protein 1++ ~ 3 ~ 3++, may occur , may occur

hyaline (or granular or red hyaline (or granular or red

cell) casts, +/- WBC cell) casts, +/- WBC

2.2. Blood exam Blood exam

2.1.2.1. Hemogram: initial mild  Hemogram: initial mild

anemia ( due to hemodilution), anemia ( due to hemodilution),

WBC↑ or normal WBC↑ or normal

2.2.2.2. ESR↑ ESR↑

3.3. Renal functions: BUN andRenal functions: BUN and

Cr are normal or slight increase Cr are normal or slight increase

4.4. Immunologic examImmunologic exam

Evidence of recent Evidence of recent

streptococcal infection— streptococcal infection—

4.1.4.1. ASO↑: 70%~80% of ASO↑: 70%~80% of

patients, 10~14 days after patients, 10~14 days after

infected, incidence peak at infected, incidence peak at

3~5w , normal after 3~6 m 3~5w , normal after 3~6 m

4.2.4.2. ADNase-B: positive rate ADNase-B: positive rate

is high (more than 90% is high (more than 90%

cases ) cases )

4.3.4.3. ADPNase ADPNase

4.4.4.4. Ahase Ahase

5.5. Serum complementSerum complement

80%~90% cases– low CH 80%~90% cases– low CH50 50

and low C and low C33 (within 2 w of the (within 2 w of the

onset), normalized in 6~8 w onset), normalized in 6~8 w

●●If CIf C33 still low after 8 w — still low after 8 w —

other etiology? other etiology?

Course & PrognosisCourse & Prognosis ▲▲Course: About 2 wCourse: About 2 w

▲▲Routine urine test: returns Routine urine test: returns

to normal within 4~6 w to normal within 4~6 w

▲▲ESR: returns to normalESR: returns to normal within 2~3 mwithin 2~3 m

▲ ▲ Addis count: 4~8 mAddis count: 4~8 m

▲ ▲ Microscopic hematuria may Microscopic hematuria may

persist for 6 m~ 1 y persist for 6 m~ 1 y

▲ ▲ Prognosis: most children Prognosis: most children

( (9595%)have a complete recover,%)have a complete recover,

recurrences are rare recurrences are rare

DiagnosisDiagnosis

Clinical diagnosis —Clinical diagnosis —AcuteAcute

Nephritic SyndromeNephritic Syndrome

▲▲ Diagnostic pointDiagnostic point

1.1. Prodromal infections, Prodromal infections,

evidence of streptococcal evidence of streptococcal

infection infection

2. 2. Urine exam: RBC, protein Urine exam: RBC, protein

and casts and casts

3. 3. Low CLow C33

Differential DiagnosisDifferential Diagnosis 1.1.Other AGN: e.g. MPGN , Other AGN: e.g. MPGN ,

IgA nephropathy ( IgAN) IgA nephropathy ( IgAN)

2.2. Acute exacerbation of Acute exacerbation of

chronic nephritis chronic nephritis

3.3. Rapidly progressive GN  Rapidly progressive GN

4.4. Nephrotic syndrome Nephrotic syndrome

5.5. Secondary GN, e.g. HSP Secondary GN, e.g. HSP

nephritis nephritis

TherapyTherapy There is no specific treatmentThere is no specific treatment

for typical cases. for typical cases.

1.1. General measuresGeneral measures

1.1.1.1. Frequent Frequent measurementmeasurement ofof

BPBP

1.2.1.2. Rest treatment Rest treatment

Bed rest:Bed rest: within 2 w of onset within 2 w of onset

Slight activities:Slight activities: edema sub- edema sub-

sided, BP be normal and sided, BP be normal and

gross hematuria disappeared gross hematuria disappeared

Continue to attend school:Continue to attend school: ESRESR

returns to normal returns to normal

Normal activities:Normal activities: 3 m after the 3 m after the

routine urine test be normal routine urine test be normal

1.3.1.3. DietDiet

★★ Edema, hypertension– re- Edema, hypertension– re-

strict sodium – low salt strict sodium – low salt

diet (sodium chloride 60 diet (sodium chloride 60

mg/kg/d ), or salt-free diet mg/kg/d ), or salt-free diet

★★ Azotemia: proteins 0.5 g/Azotemia: proteins 0.5 g/

kg/d kg/d

★★ Severe oliguria, BP↑or Severe oliguria, BP↑or

circulatory congestion: re- circulatory congestion: re-

stricting fluid intake, stricting fluid intake, chartchart

to record intake and output to record intake and output

2. 2. AntibioticsAntibiotics

ObjectObject : to eradicate remnant : to eradicate remnant

bacteria in the focuses, but bacteria in the focuses, but

does not alter natural history does not alter natural history

of AGN of AGN

PG im , for 10 ~14 days PG im , for 10 ~14 days

3. 3. Symptomatic treatmentSymptomatic treatment

3.1.3.1. Diuretics Diuretics

HCT 1~2 mg/kg/d, HCT 1~2 mg/kg/d,

Lasix 1~2 mg/kg/time , Lasix 1~2 mg/kg/time ,

q6~8 h (prn) q6~8 h (prn)

3.2.3.2. Antihypertensive medica Antihypertensive medica

-tion -tion

Systolic pressure>140 mmHg Systolic pressure>140 mmHg

Diastolic pressure>90 mmHg Diastolic pressure>90 mmHg

★★Nifedipine 0.2~0.3 mg/kg/dNifedipine 0.2~0.3 mg/kg/d

(Max. 1 mg/kg/d ), bid~tid, (Max. 1 mg/kg/d ), bid~tid,

po/sublingual po/sublingual

★★Reserpine 0.07 mg/kg/time, Reserpine 0.07 mg/kg/time,

po/im , (Max. 1.5 po/im , (Max. 1.5 ～～ 2 mg/tim2 mg/tim

e)e)

→0.02 mg/kg/d , po →0.02 mg/kg/d , po

★★Captopril 0.3~0.5 mg/kg/d,Captopril 0.3~0.5 mg/kg/d,

po, (Max. 5~6 mg/kg/d), bid po, (Max. 5~6 mg/kg/d), bid

or tid or tid

4.4. Deal with serious symptomsDeal with serious symptoms

4.1.4.1. Hypertensive encephalo- Hypertensive encephalo-

pathy pathy

▲▲Treatment must be givenTreatment must be given

promptly promptly

UseUse sodium nitroprussidesodium nitroprusside

infusioninfusion － － 5~10 mg+10%GS 5~10 mg+10%GS

100ml(50~100 ug/ml) , 1ug/ 100ml(50~100 ug/ml) , 1ug/

kg/min, >W8 ug/kg/min kg/min, >W8 ug/kg/min

AttentionAttention: Survey BP: Survey BP

4.2.4.2. Serious circulatory congestionSerious circulatory congestion

a.a. Restrict the intake of water Restrict the intake of water

and sodium and sodium

b.b. Treatment of hypertension Treatment of hypertension

c.c. Diuretics Diuretics

4.3.4.3. Acute renal insufficiencyAcute renal insufficiency

Lasix 5 mg/kg/time, Lasix 5 mg/kg/time,

Fluid 400ml/m Fluid 400ml/m22/24h,/24h,

Dialytic treatment Dialytic treatment

5.5. Follow-up: measure BP, Follow-up: measure BP,

blood test (C blood test (C33, BUN, Cr) ,, BUN, Cr) ,

urine test urine test

PreventionPrevention

Proper treatment of Proper treatment of

pharyngitis and skin pharyngitis and skin

infections; less crowded infections; less crowded

living conditions. living conditions.

       

Think you!Think you!