This presentation was done by Dr. Julius P. Kessy,MD. An intern Doctor at Dodoma Regional Referral Hospital (DRRH) during pediatrics unit clinical meeting and supervised by Dr. Christina K. Galabawa,MD,Mmed2, Pediatrics and Child Health, University of Dodoma (UDOM) in November, 2017.
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Nephrotic Syndrome in Pediatrics
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)
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.
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
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.
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
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
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.
