NEPHROTIC SYNDROME
Q&A COUNT: 33*
Relevant Findings to this case:
History:
1) C/C: Facial puffiness/swollen legs and decreased urine output
2) Facial puffiness is more prominent when child gets up in morning
3) Decreased urine output - Number of times/day, no hematuria* (rule out Nephritic syndrome which has hematuria)
4) H/O orthopnea, dyspnea, PND (in case of complication - CCF)
5) H/O recurrent respiratory tract infections (complication)
6) No H/O sore throat, No H/O skin infections
7) No H/O seizures or blurred vision or headache (ruled out hypertensive encephalopathy due to Glomerulonephritis)
8) H/O abdominal pain or distension (in Nephrotic syndrome)
Examination:
Bilateral pitting pedal edema
Normal BP* (BP elevated in Nephritic syndrome)
Anasarca
Penile/scrotal edema
Abdomen: Distended, Fluid thrill positive, Shifting dullness negative
Normal liver span
No renal angle tenderness
1) Heavy proteinuria (>50 mg/kg/day)
2) Hypoproteinemia (<5 g/dl) or Hypoalbuminemia (<2.5 g/dl)
3) Hyperlipidemia (>200 mg/dl) with or without edema
Q&A COUNT: 33*
Relevant Findings to this case:
History:
1) C/C: Facial puffiness/swollen legs and decreased urine output
2) Facial puffiness is more prominent when child gets up in morning
3) Decreased urine output - Number of times/day, no hematuria* (rule out Nephritic syndrome which has hematuria)
4) H/O orthopnea, dyspnea, PND (in case of complication - CCF)
5) H/O recurrent respiratory tract infections (complication)
6) No H/O sore throat, No H/O skin infections
7) No H/O seizures or blurred vision or headache (ruled out hypertensive encephalopathy due to Glomerulonephritis)
8) H/O abdominal pain or distension (in Nephrotic syndrome)
Examination:
Bilateral pitting pedal edema
Normal BP* (BP elevated in Nephritic syndrome)
Anasarca
Penile/scrotal edema
Abdomen: Distended, Fluid thrill positive, Shifting dullness negative
Normal liver span
No renal angle tenderness
QUESTIONS:
Definition of Nephrotic Syndrome1) Heavy proteinuria (>50 mg/kg/day)
2) Hypoproteinemia (<5 g/dl) or Hypoalbuminemia (<2.5 g/dl)
3) Hyperlipidemia (>200 mg/dl) with or without edema
HISTORY
EDEMA
Q1: Mechanisms of edema in Nephrotic syndrome and AGN
Nephrotic syndrome: Decreased osmotic pressure
AGN: Increased hydrostatic pressure
(Note that both disease follow different mechanisms to produce same edema)
Q2: How to differentiate Cardiac and Renal edema?
Cardiac edema occurs in Dependent parts like Pre-sacral edema (dependent part on sitting)
Q3: How to check Pre-sacral edema?
Same as how you check Pedal edema.
Done in sacral and coccyx regions**
Done in sacral and coccyx regions**
Q4: Till when, you can check pre-sacral edema?
Answer:
Till the child starts to walk (it will sit to make its pre sacral area as its dependent part)
Q5: Why facial puffiness is particularly seen in Renal disease edema, why not other edema's?
Cause is unknown**
Recurrent LRI History:
Recurrent LRI History:
What is recurrent Respiratory Tract infection? (or) Tell criteria to say it is recurrent RTI
6-8 episodes of Respiratory Infection in every year
EXAMINATION
Q1: Renal angle tenderness - Causes:
Answer:
Pyelonephritis
Pyonephrosis
Hydronephrosis
Any mass
Q2: Boundaries of Renal angle
Located between the lateral border of erector spinae muscle and inferior border of the twelfth rib
MANAGEMENT
Investigations for Nephrotic Syndrome (very important)
Basic investigations:
1) Three consecutive early morning samples - 3+ or 4+ proteinuria
2) 24 hour urinary protein >50 mg/kg/day
3) Spot Urine protein: Creatinine ratio (>3 in Nephrotic syndrome)
4) Serum protein (Total <5 g/dl or Albumin <2.5 g/dl)
5) Lipid profile
6) Complete hemogram
7) Renal function test
8) USG Abdomen and Xray KUB region
9) Before starting steroids:
a) Urine culture and sensitivity
b) Chest X ray
c) Mantoux test
Specific investigations:
1. ASO titre
2. Serum C3, C4 levels
3. Serum electrophoresis
4. ANA
5. HbsAg
Value of Spot Urine Protein: Creatinine ratio in Nephrotic Syndrome
>3 in Nephrotic syndrome
Which disease can flare up when steroid treatment is given in children?
TUBERCULOSIS - Chest X ray and Mantoux are done before giving steroids
DEFINITION FOR DIFFERENT CASE TERMINOLOGIES (very important)
(1) Remission of Nephrotic Syndrome
Urine Trace or negative for protein for 3 consecutive days
(2) Relapse of Nephrotic Syndrome
3 consecutive days - shows 3+ or 4+ proteinuria with edema
(3) Infrequent Relapse
3 or less relapse in a year
(4) Frequent Relapse
2 or more relapse in 6 months
(5) Steroid Dependent Nephrotic Syndrome
2 consecutive relapses while on alternate day steroids
(or)
2 consecutive relapses within 14 days of stopping steroids
(6) Steroid Resistant Nephrotic Syndrome
Proteinuria persists after 8 weeks of completing steroids
(or)
Proteinuria persists after 4 weeks of alternate day steroids followed by 3 doses of IV Methylprednisolone
(7) Early responders
Remission by 4 weeks of steroid therapy
(8) Late responders
Persistence of proteinuria by 4 weeks of steroid therapy, but urine becomes negative by 8 weeks of "daily" steroid therapy
TREATMENT FOR NEPHROTIC SYNDROME
1. High protein diet
2. Normal salt intake - no extra salt should be given
3. Screen for TB*
4. Diuretics for edema (Furosemide 1-4 mg/kg/day in 2 divided doses)
5. Steroids or Steroid sparing agent (Cyclosporine, Tacrolimus or MMF or Cyclophosphamide) (based on whether the disease steroid sensitive or steroid resistant)
MANAGEMENT PROTOCOL FOR GIVING STEROIDS IN NEPHROTIC SYNDROME
(1) For First episode
Prednisolone 2 mg/kg for 6 weeks (max 60 mg) followed by 1.5 mg/kg on single morning alternate days for 6 weeks
(2) In case of first time relapse or infrequent relapse after steroid treatment
If any H/O precipitating factors like UTI, URI, LRI - treat them
After treating it, test for Urine protein
(a) If urine protein is negative - It indicates spontaneous remission
(b) If urine protein is positive - STEROID is INDICATED
Prednisolone 2 mg/kg until remission (within 14 days) followed by 1.5 mg/kg on single morning alternate days for 6 weeks and tapered over 4 weeks
(3) Frequent relapses/ Steroid dependent
Tab.Prednisolone on single alternate morning days to maintain remission
Assess for steroid threshold
(a) If non toxic (<0.5 mg/kg) - continue for 9-18 months
(b) If steroid toxicity (>0.5 mg/kg) - add LEVAMISOLE 2-2.5 mg/kg alternate days with tapering dose of steroids to reach 0.3-0.5 mg/kg for 6 months
(4) In case of Steroid Resistance
RENAL BIOPSY
Indications for RENAL BIOPSY
1. Gross or persistent microscopic hematuria
2. Low C3 levels
3. Hypertension
4. Steroid resistance
5. Impaired renal function
6. Age of onset <1 year or >8 years
Side effects of Steroids
1. Hypertension
2. Neuropsychiatric disturbances
3. Severe infections, TB
4. Ophthalmology manifestations
MANAGEMENT
Investigations for Nephrotic Syndrome (very important)
Basic investigations:
1) Three consecutive early morning samples - 3+ or 4+ proteinuria
2) 24 hour urinary protein >50 mg/kg/day
3) Spot Urine protein: Creatinine ratio (>3 in Nephrotic syndrome)
4) Serum protein (Total <5 g/dl or Albumin <2.5 g/dl)
5) Lipid profile
6) Complete hemogram
7) Renal function test
8) USG Abdomen and Xray KUB region
9) Before starting steroids:
a) Urine culture and sensitivity
b) Chest X ray
c) Mantoux test
Specific investigations:
1. ASO titre
2. Serum C3, C4 levels
3. Serum electrophoresis
4. ANA
5. HbsAg
Value of Spot Urine Protein: Creatinine ratio in Nephrotic Syndrome
>3 in Nephrotic syndrome
Which disease can flare up when steroid treatment is given in children?
TUBERCULOSIS - Chest X ray and Mantoux are done before giving steroids
DEFINITION FOR DIFFERENT CASE TERMINOLOGIES (very important)
(1) Remission of Nephrotic Syndrome
Urine Trace or negative for protein for 3 consecutive days
(2) Relapse of Nephrotic Syndrome
3 consecutive days - shows 3+ or 4+ proteinuria with edema
(3) Infrequent Relapse
3 or less relapse in a year
(4) Frequent Relapse
2 or more relapse in 6 months
(5) Steroid Dependent Nephrotic Syndrome
2 consecutive relapses while on alternate day steroids
(or)
2 consecutive relapses within 14 days of stopping steroids
(6) Steroid Resistant Nephrotic Syndrome
Proteinuria persists after 8 weeks of completing steroids
(or)
Proteinuria persists after 4 weeks of alternate day steroids followed by 3 doses of IV Methylprednisolone
(7) Early responders
Remission by 4 weeks of steroid therapy
(8) Late responders
Persistence of proteinuria by 4 weeks of steroid therapy, but urine becomes negative by 8 weeks of "daily" steroid therapy
TREATMENT FOR NEPHROTIC SYNDROME
1. High protein diet
2. Normal salt intake - no extra salt should be given
3. Screen for TB*
4. Diuretics for edema (Furosemide 1-4 mg/kg/day in 2 divided doses)
5. Steroids or Steroid sparing agent (Cyclosporine, Tacrolimus or MMF or Cyclophosphamide) (based on whether the disease steroid sensitive or steroid resistant)
MANAGEMENT PROTOCOL FOR GIVING STEROIDS IN NEPHROTIC SYNDROME
(1) For First episode
Prednisolone 2 mg/kg for 6 weeks (max 60 mg) followed by 1.5 mg/kg on single morning alternate days for 6 weeks
(2) In case of first time relapse or infrequent relapse after steroid treatment
If any H/O precipitating factors like UTI, URI, LRI - treat them
After treating it, test for Urine protein
(a) If urine protein is negative - It indicates spontaneous remission
(b) If urine protein is positive - STEROID is INDICATED
Prednisolone 2 mg/kg until remission (within 14 days) followed by 1.5 mg/kg on single morning alternate days for 6 weeks and tapered over 4 weeks
(3) Frequent relapses/ Steroid dependent
Tab.Prednisolone on single alternate morning days to maintain remission
Assess for steroid threshold
(a) If non toxic (<0.5 mg/kg) - continue for 9-18 months
(b) If steroid toxicity (>0.5 mg/kg) - add LEVAMISOLE 2-2.5 mg/kg alternate days with tapering dose of steroids to reach 0.3-0.5 mg/kg for 6 months
(4) In case of Steroid Resistance
RENAL BIOPSY
Indications for RENAL BIOPSY
1. Gross or persistent microscopic hematuria
2. Low C3 levels
3. Hypertension
4. Steroid resistance
5. Impaired renal function
6. Age of onset <1 year or >8 years
Side effects of Steroids
1. Hypertension
2. Neuropsychiatric disturbances
3. Severe infections, TB
4. Ophthalmology manifestations
Q: Differences between Nephrotic syndrome and AGN
 |
| This picture is taken from Aruchamy textbook of Paediatrics |
Mantoux Test:
1. About Mantoux test
Dosage - 0.1 ml of Purified Protein Derivative (PPD) injected intra-dermally
After 24-48 hours
Measure vertical INDURATION (not horizontal erythema - as lymphatic spread is possible)
Induration measurement:
>10 mm - Definite TB
6-9 mm - Possible TB
5 mm or less - TB ruled out
Dosage - 0.1 ml of Purified Protein Derivative (PPD) injected intra-dermally
After 24-48 hours
Measure vertical INDURATION (not horizontal erythema - as lymphatic spread is possible)
Induration measurement:
>10 mm - Definite TB
6-9 mm - Possible TB
5 mm or less - TB ruled out
2. False positive Manxtoux test - conditions:
a) Prior BCG vaccination
b) Atypical mycobacteria infection
c) Hypersensitivity (Allergy)
a) Prior BCG vaccination
b) Atypical mycobacteria infection
c) Hypersensitivity (Allergy)
3. False negative Manxtoux test - conditions:
a) Malnutrition
b) Steroids
c) Sarcoidosis
d) Infectious Mononucleosis
e) AIDS
f) Hodgkin's disease
b) Steroids
c) Sarcoidosis
d) Infectious Mononucleosis
e) AIDS
f) Hodgkin's disease
*******
Annexure:
MODEL CASE SHEET
HISTORY:
1) Demography - Name, Age, Gender, Area, Order of Birth, Consanguinity, Informant and reliability
2) Chief complaints - Decreased Urine output and facial puffiness/swollen legs for past "x" days
3) History of presenting illness:
Ask H/O of hematuria, sore throat, skin infection, headache/blurred vision (hypertension) - to rule out glomerulonephritis*
Ask H/O of heart failure symptoms (dyspnoea, orthopnoea, PND) - complication of Nephrotic syndrome
4) Past History - H/O similar episodes in past along with any treatment given
5) Antenatal History - all 3 trimesters (no significant role in this case)
6) Birth History (no significant role in this case)
7) Postnatal History (breastfeeding/weaning) (no significant role in this case)
8) Developmental History - all 4 milestones (Milestones attained appropriate for age, Developmental quotient = 100%)
9) Diet History (no significant role in this case)
10) Family History - Not significant usually
11) Socio-environmental History (Socio economic class & Environmental history) (Overcrowding is a factor involved in post streptococcal glomerulonephritis)
12) Immunization History (no significant role in this case)
13) Contact History (no significant role in this case)
14) Allergy History (no significant role in this case)
Provisional Diagnosis
EXAMINATION:
1) General examination - Child is conscious, alert, active. Bilateral pitting pedal edema present
2) Vitals - Normal (BP is normal**)
3) Anthropometry - Normal
4) Head to Toe examination
1. Anasarca
2. Bilateral pitting pedal edema
3. Facial puffiness
4. Penile and scrotal edema
5) Systemic examination - all 4 systems
Abdomen - Fluid thrill positive and Shifting dullness negative in Abdominal percussion
No renal angle tenderness
Other systems - Normal
DIAGNOSIS
1. A case of Nephrotic Syndrome with no complications* (if there is no CCF, mention it as complication) with:
2. Normal Immunization status (or) not immunized upto date,
3. Normal Anthropometry (or) mention abnormal anthropometric measure,
4. Protein deficit in %, Calorie deficit in % (or) No protein or calorie deficit,
5. Normal Developmental status (or) Developmental delay with DQ of ___%,
6. (And Mention if any) poor socio-environmental conditions like _____
1. A case of Nephrotic Syndrome with no complications* (if there is no CCF, mention it as complication) with:
2. Normal Immunization status (or) not immunized upto date,
3. Normal Anthropometry (or) mention abnormal anthropometric measure,
4. Protein deficit in %, Calorie deficit in % (or) No protein or calorie deficit,
5. Normal Developmental status (or) Developmental delay with DQ of ___%,
6. (And Mention if any) poor socio-environmental conditions like _____
INVESTIGATIONS
Basic investigations:1) Three consecutive early morning samples - 3+ or 4+ proteinuria
2) 24 hour urinary protein >50 mg/kg/day
3) Spot Urine protein: Creatinine ratio (>3 in Nephrotic syndrome)
4) Serum protein (Total <5 g/dl or Albumin <2.5 g/dl)
5) Lipid profile
6) Complete hemogram
7) Renal function test
8) USG Abdomen and Xray KUB region
9) Before starting steroids:
a) Urine culture and sensitivity
b) Chest X ray
c) Mantoux test
Specific investigations:
1. ASO titre
2. Serum C3, C4 levels
3. Serum electrophoresis
4. ANA
5. HbsAg
TREATMENT
1. High protein diet
2. Normal salt intake - no extra salt should be given (you can simply tell salt restriction)
3. Diuretics for edema (Furosemide 1-4 mg/kg/day in 2 divided doses)
4. Steroids
2. Normal salt intake - no extra salt should be given (you can simply tell salt restriction)
3. Diuretics for edema (Furosemide 1-4 mg/kg/day in 2 divided doses)
4. Steroids