Nephrotic Syndrome intro, pathophysiology & complications

this ppt shows the important things in nephrotic syndrome

1. Nephrotic Syndrome

2. Think about a normal urine, urine tests what does it tell you about the kidney ? Colour Ph Blood Protein albumin Cells Urobilirubin Specific gravity

3. Causes of proteinuria

4. Definitions

5. Definitions Nephrotic Syndrome: triad of heavy proteinuria ( protein/creatinine ratio > 200mg/mmol), hypoalbuminemia (<30g/L) and generalized edema. Congenital Nephrotic Syndrome: NS presenting < 3 months of age Infantile Nephrotic Syndrome: NS presenting 4 -12 months Idiopathic Nephrotic Syndrome: Nephrotic syndrome in the absence of other glomerular pathology mediated by systemic disease, vasculitis, immune complex deposition, structural glomerular changes

6. Epidemiology Incidence 1.15 to 16.9 per 100,000 children and varies by ethnicity and region 1-5 years Male predominance

7. Etiology NS can be divided into three groups: Primary(Idiopathic):85 ~ 90%, associated with immune disorder, but unclear. Secondary:10 ~ 15%,due to infections, drugs or toxins, autoimmune diseases , tumors. Infantile: rare

8. Most (90%)children with primary nephrotic syndrome have some form of idiopathic nephrotic syndrome: 1. Minimal-change diseases (MCD): 85% 2. Focal segmental glomerulo - Sclerosis(FSGS): 10% 3. membranoproliferative glomerulonephritis (MPGN): 5% 4. Membranous Glomerulonephritis(MGN): rare Pathology

9. Congenital NS Primary : finish type , diffuse mesangial sclerosis ,FSGS, minimal change , membranous nephropathy Secondary: Infectious : TORCH infections Other : SLE, HUS, Nephroblastoma, Drug reaction, Mercury toxicity

10. Syndrome associated NS Denys- Drash syndrome Nail – platella syndrome Lowe’s syndrome Galloway Mowatt syndrome Frasier syndrome Pierson syndrome

11. Primary nephrotic syndrome: common diagnoses

12. Sequence of Events in Nephrotic Syndrome Pathophysiology Glomerular injury Increased permeability of glomerular basement membrane Albuminuria Decreased serum albumin Decreased plasma oncotic pressure Decreased peripheral capillary return Increased renal Na reabsorbption Increased interstitial fluid Clinical Manifestations Heavy Proteinuria Microhematuria(occasional) Hypoproteinemia Hypercholesterolemia Urinary sodium Edema (Periorbital, peripheral, ascites)

13. Pathophysiology of oedema production in nephrotic syndrome

14. Signs and Symptoms Heavy Proteinuria ： urine protein +++ / ++++ Hypoalbuminaemia : (low level of albumin in the blood) albumin concentration less than 30 g/L ; Hypercholestrolemia : due to hypoalbuminaemia,but detail is’nt clear Severe Edema : Edema usually begin around eyes and face , severe in morning and decrease in afternoon.With the developing of the edema, ankles swelling, swelling foot, swollen abdomen might occur. even entire body swelling . Clinical Manifestations

15. Diagnosis Urine protein : +++ / ++++, > 3.5g 24 hrs urine protein collection . Serum albumin : <30 g/L Elevated blood cholesterol Oedema

16. Investigations

17. Hallmark of Nephrotic Onset at age above 1 year Dependent edema Most often SSNS associated with MCD. The likelihood of MCD is highest between ages 2 and 7 Edema preceded by a URTI or insect bite Low C3 / C4 or arthritis & rash – SLE

18. Treatment ABCs Screen for infections Fluid balance & hypovolemia Do not routinely fluid restrict Fluid restriction- hyponatremia (<130 meq /L) and/or severe edema in a hospital setting Low-salt diet (suggested maximum dose of 2–3 meq /kg/day) Cautions with diuretics Drugs: steroids (1st line)

19. More definitions

20. Steroids 4 weeks at 60 mg/m2 or 2 mg/kg (maximum dose 60 mg/day), followed by alternate day PDN at 40 mg/m2 or 1.5 mg/kg (maximum dose of 40 mg on alternate days) for 4 weeks or 6 weeks at 60 mg/m2 or 2 mg/kg (maximum dose 60 mg/day), followed by alternate day PDN at 40 mg/m2 or 1.5 mg/kg (maximum dose of 40 mg on alternate days) for 6 weeks

21. Things to note Complete remission (UPCr≤20 mg/mmol (0.2 mg/mg) or negative or trace dipstick on 3 or more consecutive days) and then decreased to alternate day PDN

22. Daily PDN treatment at onset of infection to prevent relapse Do not routinely use of a short course of low-dose daily PDN at the onset of an upper respiratory tract infection (URTI) for prevention of relapses Consider a short course of low dose daily PDN at the onset of an URTI in children who are already taking low dose alternate day PDN and have a history of repeated infection-associated relapses

23. Management of Finnish-type congenital nephrotic syndrome The aim of treatment is to enable growth and development. Nephrectomy: early unilateral nephrectomy at around 3 months of age Angiotensin-converting enzyme and prostaglandin inhibitors’ with the second nephrectomy Replacement of albumin Albumin requirements Transplantation Diuretics Nutrition and growth — high calorie and high protein diet (4mg/kg/day) Thyroxine is necessary from birth

24. Adjuvant therapies Albumin or Fresh Frozen Plasma Antiproteinuric agents ;ACE inhibitors and or ARBs Antihypertensive agents Diuretics- caution Vitamin D supplementation Calcium supplementation Fluid management Vaccination Treat infections

25. Vaccination

26. Infections

27. Hypovolemia

28. Kidney biopsy Genetic testing Macroscopic hematuria Low C3 levels AKI not related to hypovolemia Sustained hypertension Arthritis and/or rash Infantile onset NS if genetic screening is not available >12 years of age Persistent microscopic hematuria –Ig A nephropathy SRNS Congenital NS Extra renal features Family history suggesting syndromic/hereditary SRNS consider in patients with Infantile onset NS recommended in patients diagnosed with SRNS

29. Hypovolemic shock Electrolyte disturbance Thrombosis Acute renal failure (ARF):majority associate with low blood volume Infections :respiratory infections, skin infections, peritonitis Chronic Renal Failure Death Complications

30. Thank you