Nephrotic syndrome
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Transcript of Nephrotic syndrome
Nephrotic Syndrome
Prof. Imran IqbalDepartment of Paediatrics
Children’s Hospital & Institute of Child Health, Multan, Pakistan
Nephrotic Syndrome
Nephrotic Syndrome ?Usual Presenting feature is Oedema
D/D of Oedema
Nephrotic Syndrome
Acute Glomerulonephritis
Congestive Heart Failure
Kwashiorkor
Chronic Liver Disease
Protein Losing Enteropathy
Angioedema
OPD Urine Examination
• Protien = 2 + OR 3 +
• Urine protein/creatinineratio > 2
• Nephrotic Syndrome likely
Proteinuria
Spot urine
Trace = 15mg/dl
+ = 30mg/dl
++ = 100mg/dl
+++ = 300mg/dl
++++ = 2000mg/dl
Dipstick
Boiling
P/C ratio
Heavy proteinuria ( > 40mg/m2 /hour
or > 50mg/kg/day)
Hypoalbuminaemia (<2.5g/dl)
Oedema
Hyperlipidaemia (>220mg/dl)
Nephrotic Syndrome
Typical Clinical Features
• Age 2-6 years
• Boys 70 %
• Facial Puffiness / marked edema
• Mild Diarrhea
• Oliguria
• Blood Pressure normal or low
• Anorexia
Other PRESENTATIONS of NS
• GENERALIZED BODY SWELLING- EDEMA, OLIGURIA
• Abdominal pain
• Acute kidney injury
• Infections
• Shock
• Thrombo-embolism
• Hypertension
• Routine urinalysis
EXAMINATION
Clinical Assessment of Circulating Volume
•Toe Temp.
•Capillary Refill time
• B.P
•Pulse Rate, Volume
Extent of Edema
Infections
Systemic Examination
Weight, Height, Ideal weight for Height, Surface area
COMPLICATIONS
• Gross edema
• Hypovolemia
• Infections – peritonitis, cellulitis, UTI
• Pneumonia
• Tuberculosis
• Arterial / venous thrombosis
Atypical Clinical Features
• Age < 1 year or > 10 years
• Hematuria
• Hypertension
• Azotemia
Nephrotic Syndrome in Children
Congenital/Infantile NS
Primary Secondary
•Finnish Type
•DMS
•Deny –Drash Syndrome
•MCNS
•FSGS
•MN
•Galloway-Mowat
Syndrome
•Nail-Petella Syndrome
• Congenital Infections
• HIV
• Malaria
• SLE
Childhood NS
Secondary
• SLE
• HSP
•Hep B
•Others
Primary
(Idiopathic)
• MCD
• FSGS
• DMP
• MPGN
• MN
Nephrotic Syndrome in Children
• Primary Nephrotic Syndrome - 95%
• Minimal Change Disease - 80 %
CAUSES OF SECONDARY Nephrotic Syndrome
• SLE
• HSP
• Hepatitis B, C
• HIV
• Malaria
• Lymphoma
• Drugs (NSAIDS)
SLE
Secondary NS - Causes
• Lupus nephritis (LN)
• HSP nephritis (HSPN)
• Hepatitis C nephritis - MPGN
• Hepatitis B nephritis – MN
• Malaria
• HIV nephritis
• Lymphoma
• Drugs
Minimal Change Disease
• Genetic factors
• T – cell activation
• Cytokines – IL 4 & IL 13
MCD - pathology
Albuminuria
PATHOPHYSIOLOGYUnder-fill Hypothesis
Heavy Albuminuria
Hypoalbuminaema
Reduced Plasma Oncotic Pressure Continuing Salt& Water Intake
OEDEMA
Renal Salt & Water retention
HypovolaemiaShift of fluid from
plasma to interstitium
Reduced GFR
Increased proximal tubular salt &water reabsorption
Activation of the RAAS
Release of ADH
Inhibition of atrial natriuretic peptide
Labs for Nephrotic Syndrome
INITIAL INVESTIGATIONS
• Urine Exam
• Serum albumin
• Urea , Creatinine
• Lipid profile
• Na, K, Ca,
• CBC
• PT, APTT
Subsequent Investigations
• HBsAg
• HCV antibody
• ASO titre
• USG abdomen
• Tuberculin test
• X ray Chest
• C3 , C4
• Anti ds DNA antibodies
Management of Nephrotic Syndrome
• Patient COUNSELING
• Disease
• Urine Examination
• Medicines
• Complications
• Immunisations
MONITORING
• Daily home monitoring of proteinuria(PU) by dipsticks or boiling method and keeping diary
• Follow up every 4 weeks- check weight, BP, PU, edema, side effects of steroids and other medicines
• Height – every 6 months
Immunisations
• Avoid live vaccines
• Give Pneumococcal vaccine
Pneumococcal conjugate vaccine
Pneumococcal polysaccharide vaccine
SUPPORTIVE TREATMENT
Diet Hypovolemia Diuretics Mucosal protectives Antihypertensives Infections
Gross Edema
DIURETICS- WHEN TO USE ? Very cautious and judicious use when :
• Generalized anasarca
• Massive ascities or pleural effusion causing respiratory difficulty
• Massive scrotal swelling with imminent skin rupture
• Concomitant albumin infusions are required to mobilize edema
• Persistent hypertension
DIURETICS-RISKS
• Intavascular volume depletion
• Thromboembolism
• Acute kidney injury
• Severe electrolyte imbalance
• Steroid Sensitive Nephrotic Syndrome: 90%
• Steroid Resistant Nephrotic Syndrome: 10%
Primary/ Idiopathic NS
Steroid Induction of Remission therapy
• Prednisolone 60 mg / m2 for 4 wks
( usual remission time 7 – 10 days )
• If no remission:
Continue same dose for another 4 wks
OR
MethylPrednisolone IV 30 mg / m2 alternate day for 3 doses
Continuation Steroid therapy
If remission achieved:
• Prednisolone 40 mg / m2 alternate day for 4 wks
• Reduce dose by 5 – 10 mg every 4 weeks
• Stop treatment by 3 – 6 mo
Relapses in Nephrotic Syndrome
• Relapses 40 – 70 %
Treatment of Relapse
• Prednisolone 60 mg / m2 till remission
• Reduce dose by 5 – 10 mg every 4 weeks
Terminology Remission = urine albumin free for 3 days
Relapse = urine albumin 3+ for 3 days
Frequent Relapser = > 2 relapses in 6 mo or
> 3 relapses in 12 mo
Steroid Dependant = relapse within 14 days of reducing steroid dose
Steroid Resistant = No response to initial steroid therapy in 4 – 8 weeks
Types of NEPHROTIC SYNDROME Steroid Sensitive NS (SSNS)
– Non Relapsing NS (NRNS)
– Infrequently Relapsing NS (IRNS)
– Frequently Relapsing NS (FRNS)
– Steroid Dependant NS (SDNS)
Steroid Resistant NS (SRNS)Atypical NSSecondary NS
Treatment of Frequent Relapses
• Prednisolone 10 – 15 mg / m2 in Alternate day doses continued for 1 – 2 years
• Cyclophosphamide 2mg /kg for 12 weeks
• Mycophenolate Mofetil 30 mg / kg / day
Adverse effects of steroid therapy
• Infections
• Cushingoid appearance
• Emotional problems
• Hypertension
• Acid peptic disease
• Osteoporosis
• Impaired glucose tolerence
• Growth retardation
Iatrogenic Cushing’s Syndrome
MANAGEMENT OF SSNS
First Episode of NS
Trial of Steroids
Response
Withdraw Steroids over 6 months Relapse Repeat short Steroid course
Infrequent relapse
Frequent relapses Intermittent short steroid
courses
or Steroid dependency
No Relapse, discharge
Alternate day Steroids
Steroids well toleratedContd. AD Steroids
Steroids not well tolerated Consider Alternatives
Renal Biopsy
• Steroid Resistance • Age < 1 year >10 years
• Prolonged mild proteinuria
• Macroscopic haematuria
• Marked persistent HTN
• Persistent renal insufficiency
• low C3 or C4
• Cyclosporine therapy
Prognosis of SSNS
• Relapses decreased after few years
• In some relapsing patients , Relapses may continue upto 14 – 20 years
• Renal insufficiency is rare
Predictors of prolonged course
• Early onset of disease
• Severity of proteinuria
• Frequent Relapses
150 Beds Extension (New Block)