Pediatrics Nephrotic and Nephritic Syndrome 7.ppt

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/hrin adults
or 40mg/m2 /hrin
children)
Hypoalbuminemia
<2.5gm/dl
Hyperlipidemia
Edema Nephrotic
Syndrome

8. PRESENTATION
New-onset oedema
Initially periorbital or peripheral
Later genitals, ascites, anasarca
Frothy urine
Generalisedsymptoms –
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/hrfor children
volume: oliguria (during stage of edema formation)
Microscopically:-
microscopic hematuria20%, 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
MonitorU&E,BP,fluidbalance,weight
Saltandfluidrestriction
Treatunderlyingcause

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,heavyhypertension 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/m
2
/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/m
2
/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
oMonitor U&E, BP, fluid balance, weight
oSalt and fluid restriction
oTreat underlying cause
Medical
oDiuretics
oTreat hypertension
Corticosteroids/immunosuppression
oDialysis
Surgical
oRenal 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….