Pediatrics

1. Nephrotic And Nephrotic
Syndrome

3. NEPHROTIC NEPHRITIC
• Loss of foot processes  Proliferative changes and
inflammation of the glomeruli
Bottom line- “increased permeability of the glomeruli”
Pathophysiology

4. What is Nephrotic syndrome
 Increased permeability of the glomerulus leading to loss of proteins into the
tubules

5. How many pathological types causes
nephrotic syndrome?

7. Proteinuria
(>3.5g/24/hr in adults
or 40mg/m2 /hr in
children)
Hypoalbuminemia
<2.5gm/dl
Hyperlipidemia
Edema Nephrotic
Syndrome

8. PRESENTATION
 New-onset oedema
Initially periorbital or peripheral
Later genitals, ascites, anasarca
 Frothy urine
 Generalised symptoms –
lethargy, fatigue, reduced
appetite

10. Further Possible Presentations
 Oedema
 BP normal/raised
 Leukonychia
 Breathlessness:
Pleural effusion, fluid overload,
AKI
 DVT/PE/MI
 Eruptive xanthomata/
xanthalosmata

11. Differential Diagnosis for Oedema
 Congestive Cardiac Failure
Raised JVP, pulmonary oedema, mild proteinuria
 Liver disease
Hypoalbuminaemia, ascites/oedema

12.  Most children (90%) with nephrotic syndrome have a form of
the idiopathic nephrotic syndrome.

13. Investigations
 Urine dipstick for protein
 Urine microscopy
 Bloods – the usual ones, plus renal screen
Immunoglobulins, electrophoresis (myeloma
screen), complement (C3, C4) autoantibodies (ANA,
ANCA, anti-dsDNA, anti-GBM)
 Renal ultrasound
 Renal biopsy (all adults)
Children generally trial of steroids first

14. Investigations:-
1-Urine analysis:-
 Proteinuria : 3-4 + SELECTIVE.
 Urine collection for protein
>40mg/m2/hr for children
 volume: oliguria (during stage of edema formation)
 Microscopically:-
microscopic hematuria 20%, large number of hyaline cast

15. Investigations:-
2-Blood:
 Serum protein: decrease >5.5gm/dL , Albumin levels are low
(＜2.5gm/dL).
 Serum cholesterol and triglycerides:
Cholesterol ＞5.7mmol/L (220mg/dl).
 ESR↑＞100mm/hr during activity phase
3.Serum complement: Vary with clinical type.
4.Renal function

16. Management
 Conservative
Monitor U&E, BP, fluid balance, weight
Salt and fluid restriction
Treat underlying cause

17. Management
 General (non-specific )
 *Corticosteroid therapy

18. General therapy:-
 Hospitalization:- for initial work-up and evaluation of
treatment.
 Activity: usually no restriction , except
massive edema,heavy hypertension and infection.
 Diet
Hypertension and edema: Low salt diet (<2gNa/ day) only
during period of edema or salt-free diet.
Severe edema: Restricting fluid intake
 Avoiding infection: very important.
 Diuresis: Hydrochlorothiazide (HCT) ：2mg/kg.d
Antisterone ： 2～4mg/kg.d
Dextran ： 10～15ml/kg , after 30～60m,
followed by Furosemide (Lasix) at 2mg/kg .

19. Induction use of albumin:-
Albumin + Lasix (20 % salt poor)
 1-Severe edema
 2-Ascites
 3-Pleural effusion
 4-Genital edema
 5-Low serum albumin

20. Corticosteroid—prednisone therapy:-
 Prednisone tablets at a dose of 60 mg/m2/day
(maximum daily dose, 80 mg divided into 2-3 doses) for
at least 4 consecutive weeks.
 After complete absence of proteinuria, prednisone dose
should be tapered to 40 mg/m2/day given every other
day as a single morning dose.
 The alternate-day dose is then slowly tapered and
discontinued over the next 2-3 mo.

21. Treatment of relapse in NS:
 Many children with nephrotic syndrome will
experience at least 1 relapse (3-4+proteinuria plus
edema).
 daily divided-dose prednisone at the doses noted earlier
(where he has the relapse) until the child enters
remission (urine trace or negative for protein for 3
consecutive days).
 The pred-nisone dose is then changed to alternate-day
dosing and tapered over 1-2 mo.

22. According to response to prednisone
therapy:
 *Remission: no edema, urine is protein free for 5 consecutive
days.
 * Relapse: edema, or first morning urine sample contains > 2 +
protein for 7 consecutive days.
 *Frequent relapsing: > 2 relapses within 6 months (> 4/year).
 *Steroid resistant: failure to achieve remission with
prednisolone given daily for 28 days.

23. Side Effects With Long Term Use of
Steroids “Steroid toxicity
 hyperglycemia
 myopathy
 peptic ulcer
 poor healing of wound.
 Hirsutism
 Thromboembolism
-Stunted growth
Cataracts
- Pseudotumor cerebri
-Psycosis
-Osteoporosis
- Cushingoid features
-Adrenal gland suppression

24. Alternative agent:-
 When can be used:
 Steroid-dependent patients, frequent relapsers, and steroid-
resistant patients.
 Cyclophosphamide Pulse steroids
 Cyclosporin A
 Tacrolimus
 Microphenolate

25. Complications
Thromboembolism
Hyperlipidaemia
Increased
Susceptibility to
infection

26. What is nephritic syndrome?
 Refers to a specific set of renal diseases in which an
immunologic mechanism triggers inflammation
and proliferation of glomerular tissue that result in
damage to the basement membrane, mesangium or
capillary endothelium
It is a syndrome associated with severe glomerular
injury, but does not denote a specific etiologic form of
glomerulonephritis

28. Pathophysiology
 Thin glomerular basement membrane with pores that allow protein and blood
into the tubule.

30. Hematuria
Red cell casts
Hypertension
Proteinuria
<3gm/day
Oliguria
Nephritic
Syndrome

31. Signs and Symptoms
 Haematuria (E.g. cola coloured)
 Proteinuria
 Hypertension
 Oliguria
 Flank pain
 General systemic symptoms
 Post-infectious = 2-3 weeks
after strep-throat/URTI

32. Investigations
 Urine dipstick and send sample to lab
 Bloods – the usual plus renal screen
Immunoglobulins, electrophoresis, complement (C3,
C4) autoantibodies (ANA, ANCA, anti-dsDNA, anti-
GBM); blood culture; ASOT (anti-streptolysin O titre)
 Renal ultrasound
 Renal biopsy
 Urine microscopy – red cell casts

33. Investigations
 U&E – ?Elevated urea ± creatinine
 Urinalysis – haematuria, Red cell casts present, 24
hour collection helpful
 ASOT (increased in 60-80%), anti-DNAse b
 ESR ?CRP
 Cultures (throat, blood, urine)Complement
(Decreased C3, normal C4

34. Management
 Correct electrolyte abnormalities if present
 Post Streptococcal – penicillin therapy
 Admission if oliguria and renal failure
 Fluid restriction with significant oedema

35. Management
 Conservative
o Monitor U&E, BP, fluid balance, weight
o Salt and fluid restriction
o Treat underlying cause
 Medical
o Diuretics
o Treat hypertension
Corticosteroids/immunosuppression
o Dialysis
 Surgical
o Renal transplant

36. NEPHROTIC NEPHRITIC
 Negligible RBC’s /
WBC’s
 Absence of cellular
casts
 Free lipid droplets
 Lipid laden
macrophages
 RBC’s abundant
 RBC casts
 Lipid elements usually
absent
URINANALYSIS

37. Summary
 Nephrotic syndrome = MASSIVE proteinuria
 Nephritic syndrome = haematuria/red cell casts
 May be a mixed presentation
 New oedema? Dipstick that urine!
 Haematuria? Exclude malignancy!

42.  THE END….
THANK YOU….