The document presents an overview of nephrotic syndrome in children, detailing its causes, clinical features, and treatment options. It highlights key clinical characteristics such as nephrotic range proteinuria, hypoalbuminemia, and edema, and discusses epidemiology, with a greater prevalence in boys aged 1.5-6 years. Treatment involves high-dose steroids and supportive management, with prognosis varying based on the underlying condition and response to therapy.

1. Nephrotic Syndrome in
Pediatrics
Presenter: Kessy Julius P. Intern Dr.
Supervisor: Christina K. Galabawa, MD, Mmed 2, Pediatrics and Child Health, UDOM.
DODOMA REGIONAL REFERRAL HOSPITAL (DRRH)

2. Outline
1.
• Introduction
• Epidemiology
• Classification & Etiology
• Pathophysiology
• Clinical features
• Investigations & treatment
• Complications & prognosis

3. Introduction
2.
▰Syndrome caused by renal diseases that
increase the permeability across the glomerular
filtration barrier.
▰Characterized by a tetrad of clinical features

4. Contd…
3.
1. Nephrotic range proteinuria – Urinary protein
excretion > 50 mg/kg/day.
2. Hypoalbuminemia – Serum albumin
concentration < 3g/dl (30g/l).
3. Hyperlipidemia
4. Edema.

5. Epidemiology
4.
▰Occurs at all ages but most prevalent in
children 1.5-6 years.
▰Boys > girls, 2:1 ratio.
▰Higher in underdeveloped countries (South east
Asia and Africa).
▰Incidence worldwide, 2 – 7 cases per 100,000
children/year.

6. Classification & Etiology
5.
1. Primary
No identifiable systemic disease. These are
patients with;
I. Idiopathic NS – No glomerular inflammation
on renal biopsy.
II. Primary glomerulonephritis – Active
sediment and glomerular inflammation on
biopsy.

7. Contd…
6.
2. Idiopathic
I. Minimal Change Disease (MCD) - 85%
II. Focal Segmental Glomeruloscrelosis (FSGSS)
III. Membranoproliferative glomerulonephritis
IV. Mesangial proliferation
V. Membranous nephropathy
>80% of children with idiopathic NS are steroid
sensitive.

8. Contd…
7.
3. Secondary
• Infections – Viral (HBV, HCV & HIV), Bacterial
(Streptococcal) and parasites (Malaria). Others
are syphilis and toxoplasmosis.
• Systemic conditions – Autoimmune (SLE &
vasculitis), Metabolic (Diabetes & amyloidosis),
Malignancies (Leukemia & lymphomas),
Hematological (SCA), Obesity.

9. Contd…
8.
• Allergy – bee sting, cow’s milk, pollen, house
dust.
• Drugs – NSAIDs, Ampicillin, penicillamine, gold,
captopril and lithium.

10. Contd…
9.
4. Hereditary NS
• Below one year.

11. 6.

12. Clinical features
10.
▰Periorbital puffiness: more marked in the
morning and later generalized
▰Scrotal edema
▰Pleural effusion and Ascites are late features
▰Decreased urine output
▰Hypertension and hematuria are absent

13. Clinical features
11.
▰.

14. Clinical features
12.
▰.

15. Clinical features
13.
▰.

16. Clinical features
14.
▰.

17. Investigations
15.
▰Urinalysis
I. Proteinuria +3 or +4
II. Urinary protein excretion (>40mg/m2/hour)
III. 24 hours urinary protein : creatinine > 3
IV. Microscopic hematuria in 10%
V. Pus cells in underlying UTI
VI. Cellular casts in other forms not MCD

18. Contd…
16.
▰Serum
I. Albumin < 3g/dl
II. Cholesterol > 250mg/dl
▰Others
I. CBC usually normal, raised ESR
II. CXR to R/O pleural effusion

19. Contd…
17.
▰Renal biopsy (Indications)
I. SRNS
II. Frequent relapses
III. Steroid toxicity
IV. Secondary NS
V. Gross hematuria
VI. Hypertension
VII. Renal insufficiency

20. Treatment
18.
▰High dose steroids
-2mg/kg (max 60) daily for 6 weeks
-1.5mg/kg (max 40) alternating days for 6 weeks
(If attained remission)
▰Relapse management
-Precipitated by minor infections
-Treatment of infections often result in remission
for +1-2 proteinuria

21. Treatment
19.
▰Persistent +3-4 needs steroids
-2mg/kg (max 60) daily until remission, then 1.5
mg/kg on A/D for 4 weeks.
▰Steroid dependant and frequent relapses
-Long term steroid therapy
-0.3-0.7 mg/kg on A/D for 9-18 months.

22. Special definitions
20.
▰Remission: trace/no protein in urine on dipstick
for 3 consecutive days.
▰ Relapse: urine protein is 3+/4+ for three
consecutive days after having attained remission.
▰Frequent relapser: four or more relapses in 12
months.

23. Special definitions
21.
▰Steroid dependent: two consecutive relapse
while on A/D steroids or within 14 days of its
discontinuation.
▰Steroid resistant: proteinuria (+2 or more) on
daily steroid therapy after 8 weeks.

24. Treatment
22.
▰Supportive management:
▰Diet
- Adequate in proteins and calories
- Fluid and salt restriction in edema
▰Hospitalization
-Infections
-Marked edema
- Monitoring BP in hypovolemia

25. Treatment
23.
▰Diuretics
-massive ascites, pleural effusion and severe
genital edema
▰Ambulation & Anti coagulants for
thromboembolism
▰ACEI in hypertension
▰Calcium carbonate and vitamin D in
Osteoporosis

26. Complications
24.
▰Infections
-SBP, Pneumonia, UTI
-Sepsis
-Cellulitis
▰Arterial and Venous Thrombosis
▰Steroids and other drugs adverse effects

27. Prognosis
25.
▰Response time:
10% by the end of first week
70% by the end of second week
85% by the end of third week
92% by the end of fourth week

28. Contd…
26.
▰Steroid responders
90% Minimal change disease
50% Mesangial proliferation
20% Focal sclerosis

29. Contd…
27.
▰Poor prognostic factors
-Hematuria
-Hypertension
-Hypocomplementemia
-Focal segmental sclerosis
-Steroid resistance

30. THANK YOU!!!
28.
“To study the phenomenon of disease without
books is to sail an uncharted sea while to study
books without patients is not to go to the sea at
all”
- William Osler.