A 6-year-old male child was admitted to the pediatric ward with hematuria and burning urination. Tests revealed protein in his urine and thickened bladder walls on ultrasound. This is consistent with chronic cystitis. The document goes on to define nephrotic syndrome, discuss its pathophysiology, incidence, etiology including genetic and secondary causes, clinical features, lab investigations, management including diet and steroids, complications, and nursing considerations like monitoring intake/output and administering medications.

1. Prepared by Amjad Ali
MS N, KMU Peshawar

2. CASE SCENARIO
A male child patient aged 6 yrs was admitted in pediatric ward with:
Present complains:
Hematuria
burning maturation
History of present illness:
Subject developed dyspnea 2 days back and on the present day
morning blood in urine was found.
Past family history:
Not significant (NO ONE HAD HEMATURIA)

3. Investigations
1. CUE (COMPLETE URINE EXAMINATION):
urine proteins: 420 (0-8 mg/dl)
creatinine: 39 (30-40 mg/dl)
P/C ratio : 10.70 (<0.2)
pus cells : 10-15
RBC’s: LOADED
2. Abdomen USG:
urinary bladder: walls are irregular and thickened
IMPRESSION: cystitis (chronic)
3. Culture Test: NEGATIVE

4. DEFINITION
 Manifestation of glomerular disease, characterized by
nephrotic range proteinuria and a triad of clinical
findings associated with large urinary losses of protein
: hypoalbuminaemia , edema and hyperlipidemia.
 Defined as
 protein excretion of > 40 mg/m2/hr
 First morning protein : creatinine ratio of > 2-3 : 1

5. PATHOPHYSIOLOGY

6. PATHOPHYSIOLOGY
 1. Edema

8. Incidence ( pediatric )
 2 – 7 cases per 100,000 children per year
 Higher in underdeveloped countries ( South east Asia )
 Occurs at all ages but is most prevalent in children
between the ages 1.5-6 years.
 It affects more boys than girls, 2:1 ratio
http://www.kidney.org/site/107/pdf/NephroticSyndro
me.pdf

9. Etiology
 Genetic
 Secondary
 Idiopathic or Primary

10. Genetic causes
 Finnish type Congenital Nephrotic Syndrome
 Focal Segmental Glomerulosclerosis
 Diffuse Mesangial Sclerosis
 Denys-Drash Syndrome
 Nail – Patella Syndrome
 Alport Syndrome
 Charcot-Marie-tooth disease
 Cockayne syndrome
 Laurence-Moon-Beidl-Bardet Syndrome
 Galloway-Mowat Syndrome

11. Secondary causes
 Congenital
 Oligomeganephronia
 Infectious
 Hepatitis (B,C) , HIV-1, Malaria, Syphilis, Toxoplasmosis
 Inflammatory
 Glomerulonephritis
 Immunological
 Castleman Disease, Kimura Disease, Bee sting, Food allergens
 Neoplastic
 Lymphoma, Leukemia
 Traumatic ( Drug induced )
 Penicillamine, Gold, NSAIDS, Pamidronate, Mercury, Lithium.

12. Idiopathic
 Minimal Change disease ( >80 % )
 Mesangial proliferation
 Focal segmental Glomerulosclerosis
 Membranous Nephropathy
 Membranoproliferative glomerulonephritis

13. Clinical Features
Edema
 Mild to start with – peri orbital puffiness, lower extremities
 Progression to generalized edema, ascites, pleural effusion,
genital edema
Decreased urine output
 Anorexia, Irritability, Abdominal pain and diarrhea
 Absence of
 Hypertension
 Gross hematuria

14. NO PEDAL EDEMA BUT FACIAL PUFFYNESS PRESENT

15. DIFFERENTIALS
 Protein losing enteropathy
 Hepatic failure
 Heart failure
 Acute/Chronic Glomerulonephritis
 Protein Malnutrition

16. Lab Investigations
 Urine Examination
 Complete Blood Count & Blood picture
 Renal parameters :
 Spot Urine Protein : Creatinine ratio
 Urinary protein excretion
 protein selectivity ratio
 Liver Function Test
 Renal Biopsy ???

17. Continue………
• Urinalysis - 3+ to 4+ proteinuria
• Renal Function
 Spot UPC ratio > 2.0
 UPE > 40 mg/m2/hr
 Serum Creatinine – normal or elevated
 Serum albumin - < 2.5 gm/dl
 Serum Cholesterol/ TGA levels – elevated
 Serum Complement levels – Normal or low
- Nelson Textbook of Pediatrics, Vol 2, 19th Edition, page 1804

18. Additional Tests
 C3 and antistreptolysin
 Chest X ray and tuberculin test
 Hepatitis B surface antigen
Indications for Biopsy
 Age below 12 months
 Gross or persistent microscopic hematuria
 Low blood C3
 Hypertension
 Impaired renal Function
 Failure of steroid therapy

19. Management
(Initial Episode)
 High protein diet
 Salt moderation
 Treatment of infections
 If significant edema – diuretics Aldosterone
antagonist ( Fursemide, spironolactone )
 Corticosteroid therapy with Prednisolone or
prednisone
 ( 2mg/kg per day for 6 weeks followed by
1.5 mg/kg single morning dose on alternate days for
6 weeks )

20. Subsequent course
 Relapse
 Infrequent Relapsers : 3 or less relapses per year
 Frequent Relapsers : 4 or more relapses per year
 Steroid therapy
 Steroid dependant : relapse following dose reduction
or discontinuation
 Steroid resistant : Partial or no response to initial
treatment

21. Management of Relapse
 Parent Education
 Symptomatic therapy for infections in case of low
grade proteinuria
 Persistent proteinuria ( 3 - 4+ ) –
 Prednisolone
( 2mg/kg/day until protein is negative for 3 days )
1.5 mg/kg on alternate days for 4 weeks )
Ghai Essential Pediatrics,8th edition, page
479.

22. Complications
 Edema
 Infections
 Thrombotic complications
 Hypovolaemia and Acute renal Failure
 Steroid Toxicity

23. Nursing management
 Goals of nursing management
 Reducing edema
 Nutrition
 Administering medications
 Skin care
 Infection prevention
 Promoting psychosocial growth
 Parental teaching

24. Nursing management
Assessment
 Age, height and weight
 Vital signs
 Past hospital admissions
 Immunization status
 Edema
 Urinary output
 Presence of infections
 Dietary pattern
 Malnourishment

25. Nursing management
 Monitoring of Vital signs
 Intake output
 Urinary protein
 Diet
 Weight
 Abdominal girth

26. Nursing management
Diet
 High protein diet 2-3 gm/kg/day
 Calories- 50- 70 kcal/kg/day
 No Added salt
 Protein rich food items
 Egg
 Milk
 Fish
 Chicken
 Paneer
 Spourts
 Pulses & legumes

27. FLUID MANAGEMENT
 Strict intake output monitoring
 Hypovolemic symptoms
 persistent tachycardia
 Hypotension
 abdominal pain
 Capillary refill >2sec urine Na <10mmol/l being
indicative of severe hypovolaemia
 Previous day output + insensible water loss
 Urine output monitoring

28. Nursing management
 Immunization.
 Live vaccines should not be given to immunosuppressed
children.
 killed vaccines are safe to administer
 However live vaccine can be considered if the child is not
on steroids for minimum of 03 months.
 Recommended -varicella, pneumococcal vaccines during
remission.

29. References
 Lau, Keith, et al. "Steroid Responsive Nephrotic Syndrome
in IgA Nephropathy with FSGS." The
 Internet Journal of Nephrology 4 (2008): n. pag. Print.
 "Pediatric Nephrotic Syndrome." Pediatric Nephrotic
Syndrome. N.p., n.d. Web.
<http://emedicine.medscape.com>.
 USA. NIH. NIDDK. Childhood Nephrotic Syndrome. N.p.:
n.p., 2008. Print.
 Trachtman, Howard. “Common Diseases: Minimal Change
Nephrotic Syndrome.” Nephrology
 Self Assessment Program 11 (2012) 19-20. Print.
 Trachtman, Howard. “Common Diseases: Focal Segmental
Glomerulosclerosis.” Nephrology