nephrotic syndrome

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