pediatrics

1. Prepared by: -
M o h a m m a d A l i A l - s h e h r i
… . .
S u p e r v i s e d b y :
D r .
Nephrotic Syndrome..…(NS)

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

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

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. Causes of Nephrotic Syndrome
Most children (90%) with nephrotic syndrome have
a form of the idiopathic nephrotic syndrome.
Primary glomerulonephritis
 Minimal change disease (80% paeds cases)
 Focal segmental glomerulosclerosis (most common
cause in adults)
 Membranous glomerulonephritis

13. Systemic Causes
 Secondary glomerulonephritis
 Diabetic nephropathy
 Sarcoidosis
 Autoimmune: SLE, Sjogrens
 Infection: Syphilis, hepatitis B, HIV
 Amyloidosis
 Multiple myeloma
 Vasculitis
 Cancer
 Drugs: gold, penicillamine, captopril, NSAIDs

14. 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

15. 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

16. 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 complemen: Vary with clinical type.
4.Renal function

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

18. Management of NS:
 General (non-specific )
 *Corticosteroid therapy

19. 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 .

20.  3-ARF: pre-renal and renal
 4- cardiovascular disease :-Hyperlipidemia, may be a risk
factor for cardiovascular disease.
 5-Hypovolemic shock
 6-Others: growth retardation, malnutrition,
 adrenal cortical insufficiency

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

22. 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.

23. 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.

24. 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.

25. 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

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

27. Complications
Thromboembolism
Hyperlipidaemia
Increased
Susceptibility to
infection

28. What is nephritic syndrome?

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

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

32. 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

33. Investigations
 Urine dipstick and send sample to lab
 Urine microscopy – red cell casts
 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

34. Red Cell Casts

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!

38. pathogenesis of edema:-
 *Reduction plasma colloid osmotic pressure↓
secondary to hypoalbuminemia Edema and
hypovolemia
 *Intravascular volume↓ antidiuretic hormone (ADH
) and aldosterone(ALD)  water and sodium
retention Edema
 *Intravascular volume↓ glomerular filtration rate
 (GFR)↓ water and sodium retention  Edema

39. How many pathological types causes
nephrotic syndrome?

40. Clinical Manifestation:-
IN MCNS , The male preponderance of 2:1
: 1.Main manifestations:
Edema (varying degrees) is the common symptom
Local edema: edema in face , around eyes( Periorbital swelling) , in
lower extremities.
Generalized edema (anasarca), edema in penis and scrotum.
2-Non-specific symptoms:
Fatigue and lethargy
loss of appetite, nausea and vomiting ,abdominal pain , diarrhea
body weight increase, urine output decrease
pleural effusion (respiratory distress)

42.  THE END….
THANK YOU….