NEPHRO
CASE
Reporters:
LUNA MAGHANOY MAYORMITA
PETILO PATES
GENERAL DATA
CJ
2.5 years old
Male
Filipino
CHIEF COMPLAINT
Generalized swelling of the body
for 5 days
HPI
5 DAYS PTA
Swelling of face
▪ Acute in onset
▪ Progressing towards abdomen & bilateral upper and lower limbs
▪ Painless
▪ Pitting
Overlying skin: normal
No history of itching, rash, sore throat and fast breathing
HISTORY OF PRESENT ILLNESS
No history of:
▪ Frothy urine
▪ Yellowish discoloration of urine
▪ Frank blood in urine
▪ Crying during micturition
No history of:
▪ Cough
▪ Chest Pain
▪ Abdominal pain
▪ Loss of appetite
▪ Vomiting
▪ Jaundice
HISTORY OF PRESENT ILLNESS
No history of:
▪ Skin infection
▪ Petechiae
▪ Purpura
✓
✓
✓
✓
Went to local hospital
Medication given
UTZ
Referred to VSMMC
p
PAST MEDICAL HISTORY
●
●
●
●
Throat infection 2-3 weeks back
No hx of similar illness
No TB, asthma, HTN, DM
No hx of fever with rash and neck swelling
ANTENATAL HISTORY
No ANC visits
No TT vaccines
No h/o fever, rashes,
lymphadenopathy, excessive No h/o PV bleeding,
spotting
vomiting, increased frequency
& urgency of micturition,
burning micturition
No Fe2+ & Ca2+ taken
No h/o HA, BOV,
epigastric pain
PERINATAL HISTORY
Term via NSD
(Parent not sure of
exact date)
Baby cried at birth
BW: 2.0 kg
Breastfeeding at 4
hours of life
POSTNATAL HISTORY
No excessive bleeding and other
complications.
FAMILY HISTORY
No h/o similar illness
in the family
No h/o
consanguineous
marriage
No h/o TB, DM, HTN,
& kidney diseases
DIETARY HISTORY
• ½ cup rice with fish, egg, occasional
pork and vegetables
DEVELOPMENTAL
HISTORY
IMMUNIZATION
HISTORY
Complete
immunization as per
EPI schedule
Explore drawers, runs up
and down the stairs
Vertical and circular
strokes
Asks for food and toilet,
2-3 word sentence
General Survey
Bilateral pitting edema of lower
extremities grade 2
Facial puffiness
No scar marks of
infection
VITAL SIGNS
BP 90/60 mmHg
R arm, sitting
PR 110 bpm
Regular, normal vol. & char.
RR 22 cpm
T 98 F or 36.6 C
PHYSICAL EXAMINATION
G.P.E.
No pallor, no icterus,
no lymphadenopathy,
no cyanosis, no
dehydration
Anthropometry
1
G.I.T.
4
2
Weight: 13.5 kg
Abdomen distended, umbilicus
central, all quadrant moving
symmetrically with respiration,
no venous prominence,
no scar marks, no hernia
Palpation: no local rise in temp,
no tenderness, no lump, and
organomegaly
Percussion: Shifting dullness (+)
5
C.V.S.
S1S2
No murmurs
Chest
3
Trachea central.
bilateral chest
pansion, normal
vesicular breath
sounds, no
adventitious sounds
WHO Growth Chart Interpretation
* shaded boxes= NORMAL
PHYSICAL EXAMINATION
G.P.E.
No pallor, no icterus,
no lymphadenopathy,
no cyanosis, no
dehydration
Anthropometry
1
G.I.T.
4
2
Weight: 13.5 kg
Abdomen distended, umbilicus
central, all quadrant moving
symmetrically with respiration,
no venous prominence,
no scar marks, no hernia
Palpation: no local rise in temp,
no tenderness, no lump, and
organomegaly
Percussion: Shifting dullness (+)
5
C.V.S.
S1S2
No murmurs
Chest
3
Trachea central.
bilateral chest
pansion, normal
vesicular breath
sounds, no
adventitious sounds
LABORATORY
BIOCHEMICAL PARAMETERS
PARAMETER
RESULTS
REFERENCE RANGE
UREA
14 mg/dl
10-50
CREATININE
0.4 mg/dl
0.5-1.4
SODIUM
127 mmol/L
136-145
POTASSIUM
3 mmol/l
3.5-5.0
TOTAL PROTEIN IN 24
HOURS URINE
375 mg/ g/ day
•
•
URINE CULTURE AND
SENSITIVITY STERILE AFTER 24
HOURS
HBsAG, HCV AND HIV NEGATIVE
URINE RE
RESULT
Albumin
4+
Sugar
Nil
MICROSCOPIC TEST
WBCS
10-15/ hpf
RBCS
20-30/ hpf
Epithelial cells
3-5/ hpf
Others
Not seen
PARAMETERS
RESULTS
Blood group
O positive
REFERENCE
RANGE
CBC
Hematological
Parameters
Hemoglobin
12.8 gm/ dl
11-16 gm/ dl
PCV
35.9
36-48%
TLC
9400
4000- 11000
cell/mm
Neutrophil
25
40-75%
Lymphocyte
60
20-45%
Monocyte
6
2-10%
Eosinophil
9
1-6%
Platelet
324,00054
150,000400,000 cell/
mm cu
ESR
54
SALIENT FEATURES
Ancillary Exams
History
Acute generalized swelling
of the body for 5 days
• Previous throat infection
(2-3 weeks ago)
• No ANC visits
• BW 2.0 kg
Physical Examination
•
•
•
•
•
•
•
Facial puffiness
Bilateral pitting edema of
lower extremities (G2)
Shifting dullness on
abdomen
No cyanosis or pallor
No tachypnea or dyspnea
No murmurs
BIOCHEMICAL PARAMETERS
• Creatinine 0.4 mg/dl - dec
• Na 127 mmol/l - dec
• K 3 mmol- dec
URINALYSIS
• Albumin 4+
• WBC 10-15/HPF - inc
• RBC 20-30/HPF - inc
HEMATOLOGIC
• Neutrophil 25 - dec
• Lymphocytes 60 - inc
• Eosinophil 9 - inc
• ESR 54 (1st hr) - inc
DIFFERENTIAL DIAGNOSIS
Salient Features
2.5 y.o., Male
Acute onset
Focal Segmental
Glomerulosclerosis
Minimal Change
Syndrome
Acute Poststreptococc
al Glomerulonephritis
+
+
+
(2-10 yo; M>F)
(2-6 yo; M>F)
(all ages; M>F)
+
+
+ (periorbital)
+
+ (periorbital)
+
+
+
+
+ (severe)
+ (severe)
-
-
+
Generalized swelling
•
•
•
Facial puffiness
Bilateral pitting
edema
Shifting dullness and
distention
of abdomen
Previous throat infection
+++
DIFFERENTIAL DIAGNOSIS
Focal Segmental
Glomerulosclerosis
Minimal Change
Syndrome
Acute
Poststreptococcal
Glomerulonephritis
Creatinine 0.4 mg/dl dec
Na 127 mmol/l - dec
K 3 mmol- dec
Normal in early stages
+
+
+
+
+
+
+
+
Albumin 4+
WBC 10-15/HPF - inc
RBC 20-30/HPF - inc
+
-
+
+
+
+
+ (60-80%)
+ (10-20%)
+ (gross hematuria)
+/+/+/+/-
+/+/+/+/-
+
+
+
+
Salient Features
Neutrophil 25 - dec
Lymphocytes 60 - inc
Eosinophil 9 - inc
ESR 54 (1st hr) - inc
WORKING DIAGNOSIS:
Acute
Poststreptococcal
Glomerulonephritis
CASE
DISCUSSION
INTRODUCTION ETIOLOGY
EPIDEMIOLOGY PATHOLOGY & PATHOGENESIS
CLINICAL MANIFESTATIONS DIAGNOSIS TREATMENT
COMPLICATION PROGNOSIS PREVENTION
ACUTE POSTSTREPTOCOCCAL
GLOMERULONEPHRITIS
ACUTE GLOMERULONEPHRITIS
- inflammation and/or cellular proliferation of the glomeruli not caused by direct
infection of the kidneys
Nephritic
Syndrome
- hematuria
- proteinuria
- volume overload
Nephrotic
Syndrome
- severe proteinuria
- hypoalbuminemia
- edema
ACUTE
POSTSTREPTOCOCCAL
GLOMERULONEPHRITIS
• Primary disorder of the kidneys with
extrarenal manifestations being secondary
to renal dysfunction
• Caused by Group A Streptococcus
ETIOLOGY:
Group A Beta-Hemolytic
Streptococcus
• M proteins
- determines its virulence
• Serum Opacity Factor
- determinant of secondary sequelae of GAS infection
NEPHRITOGENIC STRAINS:
•
Pharyngitis: M-12 , 1, 4 and 25
•
Skin Infections: M- 49 , 2, 42, 56, 57 and 60
EPIDEMIOLOGY:
●
●
●
PSGN the most common
glomerulonephritis in children
worldwide.
- commonly affects 5-12 y.o.
Global burden: 450,000 /year
- developing countries
PNSP Renal Census
(1995-2007)
- 7973 cases (25%)
PATHOPHYSIOLOGY
ANATOMY
Podocytes
KIDNEY
GLOMERULUS
GLOMERULAR BASEMENT
MEMBRANE
HISTOLOGY
Normal Glomerulus
Poststreptococcal
Glomerulonephritis
NEPHRITIC SYNDROME
Characteristics:
✓
Peripheral & Orbital edema
✓
Hematuria (Cola colored urine)
✓
Arterial Hypertension
✓
Oliguria
Lab tests: ↑ Creatinine & BUN
Urine test: Hematuria, Proteinuria, RBC casts
NEPHRITIC SYNDROME
Trapped in the Glomerulus
TYPE III HYPERSENSITIVITY
* Immune Complexes*
Antigens
Antibodies
IgG and IgM
Carried through the blood
KIDNEY
Glomerular Basement Membrane (GBM)
GLOMERULAR BASEMENT MEMBRANE
Hematuria
Activation of:
C3 complement
Cytokines
Oxidants
Proteases
Proteinuria
<3.5g/day
Oliguria
↑ Blood Pressure
Salt and Water
retention
INFLAMMATION
Damage podocytes
Cola colored urine
Allow larger molecules to pass:
✓ RBC
✓ Proteins
Peripheral edema &
Periorbital edema
HISTOLOGY
NEPHROTIC SYNDROME
Characteristics:
✓
Proteinuria >3.5g / day
✓
Dyslipidemia (Hypercholesterolemia)
✓
Hypoalbuminemia
✓
Peripheral Edema
Urine test: Frothy urine (Fatty casts)
NEPHROTIC SYNDROME
↑ risk for thromboembolism
Liver compensates
Albumin
Produce
more CHON
Hypercoagulable state
(↓ Antithrombin III)
↑ Cholesterol
↓ Albumin
(Hypoalbuminemia)
↓ Plasma Oncotic
Pressure
Proteinuria
>3.5g/day
H20 & Electrolytes
Move into interstitium
↓ Immunoglobulins
Lipids
Frothy urine
(Fatty casts)
HYPERCHOLEST
-EROLEMIA
Increased risk
for infection
PERIPHERAL
EDEMA
NEPHROTIC
NEPHRITIC
ONSET
Insidious
Abrupt
EDEMA
++++
Generalized, periorbital
++
Normal
Increased
Normal or low
Increased
CLINICAL MANIFESTATIONS
BLOOD PRESSURE
JVP
NEPHROTIC
NEPHRITIC
Usually normal
Low
Normal
High
++++
>3-3.5 g/24h
++
<3 g/24h
+/-
+++
Fatty casts
RBC casts; cola/smoky urine
Mostly normal
Elevated (severe)
URINE
VOLUME
SPEC. GRAVITY
PROTEINURIA
HEMATURIA
URINE CASTS
UREA/CREA
NEPHROTIC
NEPHRITIC
Low
Normal or slightly reduced
Normal
May increase
POTASSIUM
Decreased
May increase
UREA/CREA
Normal (early)
High (RF)
May increase
OTHERS
Hyperlipidemia
BLOOD
SERUM ALBUMIN
SODIUM
CLINICAL
MANIFESTATIONS
1-2 wk
Streptococcal
pharyngitis
ACUTE NEPHRITIC
SYNDROME
Streptococcal
pyoderma
3-6 wk
Normal
renal fxn
Asymptomatic
microscopic
hematuria
Gross
hematuria
Acute renal
failure
ACUTE NEPHRITIC
SYNDROME
Peripheral
Edema
Oliguria
Hypertension
Pulmonary
Heart
failure
Respiratory
distress
Orthopnea
Cough
Encephalopathy
BOV,
severe HA
Altered
mental stat
New
seizure
ATYPICAL CLINICAL
MANIFESTATIONS
Nephrotic
syndrome
Subclinical
disease
Purpuric rash
Severe disease but
(-) initial UA
abnormalities
CLINICAL MANIFESTATIONS
6-8 weeks
Acute phase resolves
4-6 weeks
Urinary protein excretion and hypertension
normalize
1-2 years
Persistent microscopic hematuria
DIAGNOSTIC MODALITIES
•
History & PE
o Recent GAS infection
•
Urinalysis
o RBCs, RBC casts, proteinuria, & PMNs.
DIAGNOSTIC MODALITIES
•
Blood workup
o mild normochromic anemia
•
Antistreptolysin O
(ASO) titers
o confirms a recent streptococcal infection.
DIAGNOSTIC MODALITIES
•
Serum C3
o ↓ in the acute phase
•
MRI
o Patients with severe neurologic symptoms
COMPLICATIONS
HYPERTENSION
Hypertensive encephalopathy
Intracranial bleeding
HEART FALURE
ELECT. IMBALANCE
↑K, ↑PO4, ↓Ca, Acidosis
SEIZURES
UREMIA
ACUTE RENAL FAILURE
Dialysis
PREVENTION
•
Family members of patients with acute GN, should be
considered at risk and be cultured for GAS.
•
Dogs have been reported as carriers.
TREATMENT AND
MANAGEMENT
Antibiotics
❑
❑
Early systematic antibiotic does
not eliminate the risk of PSGN
10-day course of penicillin to
limit the spread of nephritogenic
organisms
Fluids
❑
❑
Sodium restriction
400mL x BSA +
urine output in 24
hours
Diuretics
❑
Furosemide 12mg/ kg / dose IV
q 6-12hours
Antihypertensives
❑
❑
Calcium channel
blockers combined
with a diuretic
ACE inhibitors
Indications for
Dialysis
❑
❑
❑
Uncontrollable
hyperkalemia
Fluid overload
Increased BUN (>100
mg/dL)
PROGNOSIS
❑
Overall prognosis is Good
❑
Volume overload resolves in 10 days
❑
❑
Serum creatinine levels returns in 3 to 4
weeks
0. 02 to 0.04 deaths/ 100,000
TREATMENT AND
MANAGEMENT
OF NEPHROTIC
SYNDROME
The goals of treatment of Nephrotic
Syndrome:
1. Relieve the symptoms
2. Prevent complications
3. Delay progressive kidney damage
Treatment and Management
●
●
Children with mild to moderate edema may be managed as
outpatient.
Parents may be taught how to use a dipstick and interpret
the results.
Use of Corticosteroids
●
Mainstay therapy for MCNS
Treatment of Initial Episode of Nephrotic Syndrome
●
Prednisone or Prednisolone single daily dose of 60mg/ m/ day or 2 mg/kg/day, to
maximum of 60mg daily for 4-6 wk followed by alternate prednisone (starting at
40mg/m2 qod or 1.5 mg/kg qod) for a period ranging from 8wk to 5mon with
tapering of dose.
Use of Corticosteroids
●
●
80-90% of children respond to steroid therapy
Remission consists of:
○
Protein: creatinine ratio of <0.2 or <1+ protein on
dipstick for 3 consecutive days
○
Majority of children respond within the first 5 wk of
treatment
Managing the Clinical Sequelae of Nephrotic
Syndrome
1. Edema
●
●
●
Children with severe symptomatic edema should be hospitalized
Sodium restriction (<1500mg daily)
Loop diuretics (Furosemide) orally or intravenously
Managing the Clinical Sequelae of
Nephrotic Syndrome
●
Patients with severe generalized edema with intravascular depletion
(hemoconcentration, hypotension, tachycardia)
○
○
IV administration of 25% albumin (0.5-1.0g albumin/kg) followed by
furosemide (1-2mg/kg/dose IV).
Should only be used in collaboration with pediatric nephrologist
Managing the Clinical Sequelae
of Nephrotic Syndrome
2. Dyslipidemia
●
●
●
Low fat diet
Dietary fat intake (< 30% cal, saturated fat < 10% cal)
Dietary cholesterol ( < 300mg/day )
Managing the Clinical Sequelae
of Nephrotic Syndrome
3. Infections
●
●
●
●
Counseled regarding the s/sx of Cellulitis, peritonitis and bacteremia
Blood culture prior to starting empiric antibiotic therapy
Paracentesis (bacterial peritonitis)
3rd generation cephalosporin IV
Managing the Clinical Sequelae
of Nephrotic Syndrome
4. Thromboembolism
●
●
Imaging studies for presence of clot
Anticoagulation (heparin, low molecular weight heparin, warfarin)
Managing the Clinical Sequelae
of Nephrotic Syndrome
5. Obesity and Growth
Dietary counseling
●
Managing the Clinical Sequelae
of Nephrotic Syndrome
6. Relapse of Nephrotic Syndrome
●
●
●
Defined as urine protein: creatinine ratio of >2 or >3+ protein on urine dipstick
for 3 days.
Triggered by UR and GI infections.
Treated similar to initial therapy, except daily prednisone is shortened
Managing the Clinical Sequelae
of Nephrotic Syndrome
●
●
Daily high dose prednisone until remission is achieved, then
switched to alternate day therapy.
Duration of alternate day therapy varies depending on
relapses of individual child
Managing the Clinical Sequelae
of Nephrotic Syndrome
7. Steroid resistance
●
●
●
Defined as failure to achieve remission after 8wk of corticosteroid therapy
Require further evaluation
Caused by FSGS (80%), MCNS or membranoproliferative glomerulonephritis
Alternative Therapies to Corticosteroids
●
●
Steroid- dependent patients, frequent relapsers and steroid resistant
patients (cushingoid appearance, hypertension, cataracts and growth
failure).
Cyclophosphamide - prolongs the remission and reduces the number of
relapses.
○ 2mg/kg single oral dose for 8-12wk
Alternative Therapies to Corticosteroids
●
Calcineurin inhibitors( cyclosporine or tacrolimus)
○
Recommended initial therapy for children with steroid resistant nephrotic
syndrome
Mycophenolate- can maintain remission in children with steroid dependent or
frequently relapsing nephrotic syndrome.
* Most children who respond to cyclosporine, tacrolimus or mycophenolate therapy tend to relapse
when medication is discontinued
Immunizations in Children with Nephrotic
Syndrome
●
●
●
●
Full Pneumococcal Vaccination (13-valent conjugant and 23-valent
polysaccharide vaccine)
Influenza vaccination annually
Defer vaccination with live vaccines until prednisone (< 1mg/kg daily or
2mg/kg on alternate days.
Varicella-zoster immune globulin for immunocompromised children on
immunosuppressive agents
PROGNOSIS
●
●
●
Children with steroid-responsive nephrotic syndrome have repeated
relapses, decreases in frequency as the child grows older.
Children who respond rapidly to steroids and those who have no relapses
during the first 6 mo after diagnosis are likely to follow and infrequently
relapsing course.
Children with FSGS, have much poorer prognosis
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