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NEPHROTIC SYNDROME
Soumya Ranjan ParidaBasic B.Sc. Nursing 4th year
Sum Nursing College
Nephrotic syndrome
Nephrotic syndrome is 15 times more common in childre than adults.
Incidence – 2-3 / 1lac children /yr
It is characterized by –
- Heavy proteinuria ( > 3.5gm/day or 40mg/m²/hr ),
- Hypoalbuminemia (< 2.5gm/dl ),
- Edema ,
- Hyperlipidemia.
Hematuria , hypertension and impaired renal function are occasionally associated.
Nephrotic SyndromeEtiology –• 90% Idiopathic nephrotic syndrome - Minimal change disease ( 85%), - Mesangial proliferation (5%) , - Focal segmental glorulosclerosis (10%)• 10% Secondary nephrotic syndrome - Membranuous nephropathy - Membranoproliferative GN
Pathophysiology –• T cell dysfunction• Altration of cytokines• Loss of negative charged glycoproteins
Pathophysiology
Heavy proteinuria
Hypoalbuminemia
Reduced plasma oncotic pressure
Extravasation of intravascular fluid
hypovolemia
Aldosterone Antidiuretic hormone
Distal Na & water reabsorption
Edema
Hyperlipidemia –
• Increased hepatic protein synthesis
• Decreased lipoprotein lipase activity
• LDL and VLDL are increased
• HDL may increase, normal or decrease
• Cholesterol and triglyceride are increased
Pathophysiology
Idiopathic Nephrotic SyndromePathology – • Minimal change disease ( 85% ) Minimal increase in mesangium and matrix IFMC – normal EMC – Effacement of epithelial cell foot processes Steriod sensitive – 90%• Mesangial proliferative ( 5% ) LMC - Diffuse increase in mesangium and matrix. IFMC – Mesangial IgM and IgA staining. EMC - Mesangium cell and matrix increased. Effacement of epithelial cell foot processes Steriod sensitive – 50%• Focal segmental glomerulosclerosis ( 10% ) LMC – mesangial proliferation and segmental scarring IFMC – IgM and C3 staing in the area of scarring
Clinical features –
• Sex – M:F 2 : 1
• Age – 2 – 6 yrs
Common –
• Periorbital edema
• Generalized edema
• Oliguria
• Irritability
• Abdominal pain
• Diarrhoea
Uncommon –
• Hypertension
• Gross hematuria
Idiopathic Nephrotic Syndrome
Diagnosis –
Urine examination –
• Proteinuria
• Hematuria
• 24 hour urinary protein
• Spot urine - protein mg% / creatinine mg %
Blood investigations –
• Sreum creatinine
• Total protein
• Serum albumin
• Serum cholesterol and triglyceride level
• C3-C4 levels – N
• IgG- low, IgM - increased
Idiopathic Nephrotic Syndrome
• Glomerular Proteinuria – Selective – LMW & albumin Nonselective – HMW IgG Selectivity = IgG / Transferrin ratio 1gm – 30 gm / day Hematuria , hypertension ,renal insufficiency• Tubular proteinuria – <1gm / day Low molecular proteinuria Little or no albumin • Transient proteinuria• Orthostatic proteinuria.R/o infections – Urine r/m , Urine c/s, Hb ,CBC, ESR , MT test Chest x- ray , USG abdomen.
Idiopathic Nephrotic Syndrome
Treatment
1st episode mild to moderate edema – • Out patient management• Sodium intake restricted initially
• Oral diuretics judiciously.
Severe edema –
• Hospitalized• Fluid restrction• IV diuretics
• 25% human albumin – 0.5gm/kg/12hour
Renal biopsy – • Hematuria, hypertension• Renal insufficiency• Hypocomplementemia
• Age beyond 1-8 yrs
• RBC casts
Prednisone - 60 mg/m²/day divided into 2-3 doses for at least 4wksAfter 4-6 wks - 40 mg/m²/day EOD single morning dose after mealsAfter 3 months – gradually tapered and stopped• Steroid responsive – Urine protein negative, trace or 1+ for 3 consecutive days• Steroid resistant – Urinary protein 2+ or more after 8 wks of therapy.• Steroid dependent – Relapse while on EOD therapy or within 28 days of stopping of steroids.• Relapse – Reappearance of proteinuria 3+ or 4+ and edema.• Infrequent relapse – 3 or less in a year.• Frequent relapse – 4 or more in a year
Treatment
Frequent relapses or steroid dependent – • Prednisolone alternate day – - 0.3 – 0.7 mg/kg: - Duration – 9-12 months• Levamisole – - 2-2.5mg/kg and prednisolone 1.5 mg/kg on alternate days. - Duration: 1 – 2yrs. - Prednisolone dose tapered and discontinued• Cyclophosphamide – - 2 mg/kg daily and prednisolone 1.5 mg/kg alternate days. - Duration : 12 wks• Cyclosporine – - 5 mg/kg daily and prednisolone 1-1.5 mg/kg alternate days. - Duration : 1-3 yrs. - Prednisolone dose tapered
Treatment
Complications
• Edema
• Infection –
- Urinary loss of Ig, properdin factor B
- Defective cell mediated immunity
- Malnutrition , edema, ascitis
- Spontaneous bacerial peritonitis ( Strept. Peumoniae, E.coli )
- Vaccination – pneumococcal, varicella, Infuenza.
• Thrombotic complications –
- Increased prothrombotic factors ( Fibrinogen, thrombocytosis,
hemoconcentration, relative immobilization.
- Decreased fibrinolytic factors ( urinary loss of AT3, protein C, S.
• Acute renal failure
• Steroid toxicity
Secondary Nephrotic Syndrome
• Glomerular diseases –
Membranous GN, MPGN, Lupus & HSP nephritis.
• Infection –
Malaria, Schistosomiasis, HBV, HCV, Filariasis, Leprosy, HIV.
• Malignancy –
Lung and GIT carcinoma, Hodgkins lymphoma
• Druga –
- Membranous GN – Penicillamine, captropil ,gold, NSAIDS,
mercury .
- Minimal change disease – Probencid, ethosuximide,
methimazole, lithium.
- MPGN – Procainamide, chlorpropamide, phenytoin,
trimethadion, paramethadione
• < 3 months
• Finnish type, AR
• Mutation in NPHS1 gene, ch 19, Nephrin
• Pathology –
Dilatation of proximal tubules, mesangial hypercellularity,
glomerular sclerosis
• Clinical features –
Massive proteinuria, large placenta, marked edema,
prematurity, RDS, seperation of cranial sutures
• Treatment -
ACE inhibitors, indomethacin, unilateral nephrectomy
• Denys-Drash syndrome –
Diffuse mesangial sclerosis, Male pseudohermaphroditism,,
Wimls tumor. Mutation in WT1 chromosome
Congenital Nephrotic Syndrome
THANKS