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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.