Medical disese of kidneys seen commonly in children

3. Nephrotic Syndrome
Prof. Imran Iqbal
Department of Paediatrics
Children’s Hospital & Institute of Child Health,
Multan, Pakistan

4. Nephrotic Syndrome

7. 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

9. OPD Urine Examination
• Protien = 2 + OR 3 +
• Urine protein/creatinine
ratio > 2
• Nephrotic Syndrome
likely

10. Proteinuria
Spot urine
Trace = 15mg/dl
+ = 30mg/dl
++ = 100mg/dl
+++ = 300mg/dl
++++ = 2000mg/dl
Dipstick
Boiling
P/C ratio

11.  Heavy proteinuria ( > 40mg/m2 /hour
or > 50mg/kg/day)
 Hypoalbuminaemia (<2.5g/dl)
 Oedema
 Hyperlipidaemia (>220mg/dl)
Nephrotic Syndrome

13. Typical Clinical Features
• Age 2-6 years
• Boys 70 %
• Facial Puffiness / marked edema
• Mild Diarrhea
• Oliguria
• Blood Pressure normal or low
• Anorexia

14. Other PRESENTATIONS of NS
• GENERALIZED BODY SWELLING- EDEMA, OLIGURIA
• Abdominal pain
• Acute kidney injury
• Infections
• Shock
• Thrombo-embolism
• Hypertension
• Routine urinalysis

15. 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

16. COMPLICATIONS
• Gross edema
• Hypovolemia
• Infections – peritonitis, cellulitis, UTI
• Pneumonia
• Tuberculosis
• Arterial / venous thrombosis

19. Atypical Clinical Features
• Age < 1 year or > 10 years
• Hematuria
• Hypertension
• Azotemia

21. Nephrotic Syndrome in Children
Congenital/Infantile NS
Primary Secondary
•Finnish Type
•DMS
•Deny –Drash Syndrome
•MCNS
•FSGS
•MN
•Galloway-Mowat
Syndrome
• Congenital Infections
• HIV
• Malaria
• SLE
Childhood NS
Secondary
• SLE
• HSP
•Hep B
•Others
Primary
(Idiopathic)
• MCD
• FSGS
• DMP
• MPGN
• MN

23. Nephrotic Syndrome in Children
• Primary Nephrotic Syndrome - 95%
• Minimal Change Disease - 80 %

24. CAUSES OF
SECONDARY Nephrotic Syndrome
• SLE
• HSP
• Hepatitis B, C
• HIV
• Malaria
• Lymphoma
• Drugs (NSAIDS)

25. SLE

26. Secondary NS - Causes
• Lupus nephritis (LN)
• HSP nephritis (HSPN)
• Hepatitis C nephritis - MPGN
• Hepatitis B nephritis – MN
• Malaria
• HIV nephritis
• Lymphoma
• Drugs

27. Minimal Change Disease
• Genetic factors
• T – cell activation
• Cytokines – IL 4 & IL 13

28. MCD - pathology

29. Albuminuria

30. PATHOPHYSIOLOGY
Under-fill Hypothesis
Heavy Albuminuria
Hypoalbuminaema
Reduced Plasma Oncotic Pressure Continuing Salt
& Water Intake
OEDEMA
Renal Salt &
Water retention
Hypovolaemia
Shift 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

31. 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

32. Management of Nephrotic Syndrome
• Patient COUNSELING
• Disease
• Urine Examination
• Medicines
• Complications
• Immunisations

33. 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

34. Immunisations
• Avoid live vaccines
• Give Pneumococcal vaccine
Pneumococcal conjugate vaccine
Pneumococcal polysaccharide vaccine

35. SUPPORTIVE TREATMENT
 Diet
 Hypovolemia
 Diuretics
 Mucosal protectives
 Antihypertensives
 Infections

36. Gross Edema

37. 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

38. DIURETICS-RISKS
• Intavascular volume depletion
• Thromboembolism
• Acute kidney injury
• Severe electrolyte imbalance

39. • Steroid Sensitive Nephrotic Syndrome: 90%
• Steroid Resistant Nephrotic Syndrome: 10%
Primary/ Idiopathic NS

40. 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

41. 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

42. Relapses in Nephrotic Syndrome
• Relapses 40 – 70 %

43. Treatment of Relapse
• Prednisolone 60 mg / m2 till remission
• Reduce dose by 5 – 10 mg every 4 weeks

44. 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

45. 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 NS
Secondary NS

46. 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

47. Adverse effects of steroid therapy
• Infections
• Cushingoid appearance
• Emotional problems
• Hypertension
• Acid peptic disease
• Osteoporosis
• Impaired glucose tolerence
• Growth retardation

49. Iatrogenic Cushing’s Syndrome

50. 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 tolerated Contd. AD Steroids
Steroids not well tolerated Consider Alternatives

51. 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

53. Prognosis of SSNS
• Relapses decreased after few years
• In some relapsing patients , Relapses may continue
upto 14 – 20 years
• Renal insufficiency is rare

54. Predictors of prolonged course
• Early onset of disease
• Severity of proteinuria
• Frequent Relapses

56. 150 Beds Extension (New Block)