Paediatric nephrology, Nephrotic syndrome

1. NEPHROTIC
SYNDROME
P R E S E N T E D BY A H M E D A L M A K R A M I ( M 6 )

2. OBJECTIVE
• Proteinuria in children.
• Nephrotic syndrome overview.
• Types.
• Pathophysiology.
• Diagnosis.
• Management (Treatment).

3. PROTEINURIA IN CHILDREN
• Definition/ protein excretion more than 150 mg/hr or more than 4 mg/𝑚2/ℎ𝑟.
• Normal daily protein excretion in a febrile child is around 100-150 mg/hr.
Method of detection of proteinuria:
• Qualitative method/ Dipstick urine test.
• Quantitative method/ urine collection 24 hours, U.Pr/U.Cr ratio.

4. DIPSTICK URINE TEST
• Detect only albumin.
Frothy Urine

5. QUANTITATIVE MEASUREMENT

6. TYPE
• Transient/ fever, exercise, dehydration, cold exposure, stress, CHF, seizures.
• Persistent like Orthostatic and glomerular and tubular and selective.
• Orthostatic/ MC persistent proteinuria in school age children and adolescent, and
should be ruled out at beginning.
Dx: check proteinuria in morning –ve and +ve in evening only.
• If fixed is glomerular or tubular disorder.

8. DIFFERENTIAL DIAGNOSIS
(OEDEMA)

9. A 3-year-old child presents to the physician with a chief complaint of
bilateral puffy eyes. On physical examination, there is no erythema or
evidence of trauma, insect bite, cellulitis, conjunctival injection, or
discharge.

10. NEPHROTIC SYNDROME
Features
• Proteinuria (>40 mg/m2/hour)
• Hypoalbuminemia (<2.5 g/dL)
• Oedema
• Hyperlipidaemia

11. • Hypoalbuminemia due to selective or non selective proteinuria.
• Generalized Oedema due to decrease plasma osmotic pressure and Na and water
retention.
• Hyperlipidaemia due to reactive to loss of protein and loss of lipoprotein lipase
• Intravascular hypovolaemia due to hypalbuminaemia

12. CAUSE
• Idiopathic in 90% of cases.
Minimal change Nephrotic syndrome (85%), Focal segmental glomerulosclerosis (10%),
Mesangeal proliferative (5%)
• Secondary nephrotic syndrome.
Any glomerulonephritis with heavy proteinuria:
 Systemic lupus nephritis, Henoch Schonlein purpura, Infection like HBV, HCV,
schistosomiasis, falciparum malaria.
 Drugs like D penicillamine & heavy metals (e.g. gold ), Phenytoin & procainamide
 Tumors e.g. Hodgkin lymphoma and carcinoma
• Congenital nephrotic syndrome.

14. IDIOPATHIC NEPHROTIC SYNDROME
MINIMAL CHANGE DISEASE (85–90%)
• Presentation/
1. 2-6 years old
2. After minor infections
3. Dependent edema: more generalized edema and anasarca (Common scenario
morning periorbital edema decrease during the day)
4. Gastro intestinal mucosal oedema/ Diarrhea, Abdominal pain, anorexia.
5. Uncommon/ HTN, gross hematuria.
No changes seen under normal microscope and immunofluorescence but podocyte
foot process fusion on electron microscopy

16. DIAGNOSIS
• UA/ proteinuria +3 or more.
• 24 hours urine > 40 mg/𝑚2
/ℎ𝑟.
• Normal serum creatinine or slight increase.
• Serum albumin < 2.5 g/dl.
• Elevated serum cholesterol and TAG.
• Normal C3 and C4.
• Renal biopsy (in specific case)/ age less than 1 years or more than 12, gross hematuria,
hypertension, high creatinine, low C3.

17. FEATURES SUGGESTING A DIAGNOSIS
OTHER THAN IDIOPATHIC MCNS
• Age <1 year or >12 year.
• Positive family history
• Extrarenal disease (eg, arthritis, rash, anaemia) Chronic disease
• Symptoms due to intravascular volume expansion (eg, hypertension, pulmonary
oedema)
• Renal failure
• Active urine sediment (red blood cell casts)

18. TREATMENT
• Hospitalize & monitor
Daily weight, 24 hr urine protein, Urine out put, Blood pressure, Monitor intravascular
volume.
• Oral corticosteroids – 60 mg/m2/day (2 mg/kg/day) for at least 4 weeks, until urine
free protein.
• Avoid infections: Perform tuberculin test, Oral penicillin prophylaxis, vaccine.
• Avoid thrombosis: low dose aspirin.
• Diet: Salt restriction, Fluid restriction in progressive weight gain, Increase proteins
intake.

20. RESPOND TO THERAPY
80% to 90% will respond to steroid in 1 month.
Relapse of proteinuria in alternative day or after termination less than 28 day (steroid
dependant).
• Groupe 1: Remission.
• Groupe 2: Remission but with frequent relapse (less than 4 in 12 months)
• Groupe 3: Remission but with frequent relapse (more than 4 in 12 months)
• Groupe 4: about 10 to 20% no Remission after 8 week of steroid, (steroid resistant)

21. COMPLICATION
• Relapse
• Protein depletion: muscle wasting, osteoporosis, short stature
• Steroids side effect: Cataract, Ulcers, Hypertension, Infections, Necrosis of bone,
Growth retardation, Osteoporosis, increase Intracranial tension, Diabetes Mellitus,
Myopathy, Adipose tissue hypertrophy (moon face, buffio hump, truncal obesity),
Pancreatitis.
• Intra vascular thrombosis: due to dehydration, loss of Anti thrombin III, protein C and
S.
• Infection: Pneumococci (peritonitis), E.coli (urinary tract infections), H.influenza
(pneumonia), Staph aureus (cellulitis), and Varicella.
• Acute renal failure ( Nephrotic crisis ): due to pre renal failure due to dehydration.

22. PROGNOSIS
• Majority of children have repeated relapses; decrease in number with age.
• Those with steroid resistance and who have focal segmental glomerulosclerosis have
much poorer prognosis (progressive renal insufficiency).

23. REFERENCE
• Nephrotic syndrome and proteinuria Lecture By Dr. Mohammed Huneif.
• Manual of Clinical Pediatric By Dr. Mansour Al-Howasi.
• Baby nelson.
• USMLE Step 2 CK Lecture note 2018 Pediatric.
• Easy Paediatrics.