NEPHROTIC SYNDROME

NEPHROTIC SYNDROME MADE EASY ...A OVERVIEW

1. A CASE PRESENTATION NIKHIL.V, Doctor Of Pharmacy, MGMH, Wrangal .

2. A male child of age 6 years was admitted into Pediatric ward with IP number 27289 0n 28/12/2015 SUBJECTIVE FINDINGS presented with c/o cough since 2days facial puffines since 10days abdominal distension since 10days Decreased urine output

3. GUESS THE DIAGNOSIS…..?

4. Haemoglobin - 11.5 gm% Sr.creatinine - 0.6 mg/dl Urinary protein - 3.8 gm% Total cholestrol - 404 mg/dl LDL - 300 mg/dl HDL - 60 mg/dl Triglycerides - 243 mg/dl VLDL - 49 mg/dl Urine output - 1.5lit normal :1ml / kg/hr OBJECTIVE FINDINGS

5. 28/12/2015 INJ.CEFOTAXIM 560 mg/iv/ tid Salt restriction diet Tab.MVT 02/01/2016 ADD TAB. PREDNISOLONE 5mg 3-2-3 04/01/2016 ADD.INJ.AMPICILLIN 400mg/iv/bid TREATMENT GIVEN

6. NEPHROTIC SYNDROME

7. DIFFERENTIAL DIAGNOSIS….? Also consider HEART FAILURE CIRRHOSIS CHRONIC GLOMERULONEPHRITIS DIABETIC NEPHROPATHY MINIMAL CHANGE NEPHROPATHY FOCAL SEGMENTAL GLOMERULOSCLEROSIS HIV ASSOCIATED NEPHROPATHY RADIATION NEPHROPATHY SICKELCELL NEPHROPATHY

8. UNDERSTAND NEPHROTIC SYNDROME PHYSIOLOGY PATHOGENESIS DIAGNOSIS TREATMENT COUNSELLING INTERACTIONS INTERVENTIONS AS A PHARMACIST

9. NEPHROTIC ? NEPHRITIC NEVER MISPELL ME…..!!!!!!!!!!

10. CROSSSECTION OF A KIDNEY CAPILLARY

11. NEPHROTIC VS NEPHRITIC NEPHROTIC >LEAKED PODOCYTES>LOSS OF PROTIENS>3.5gm/day> FROTHY URINE>PROTEINURIA>ALBUMIN KEEPS FLUID IN LUMEN> ALBUMIN LOSS>FLUID LEAKS>LEGS,LUNGS,FACE EDEMA. NEPHRITIC >ANTIGEN ANTIBODY COMPLEX>LODGE IN CAPPILERIES >ELLICT IMMUNE RESPONSE>RECRUIT WBC>INFLAMMATION> BREAKDOWN OF BARRIER>PROTIENS, RBC,WBC LEAK> HEMATURIA INFFLAMATION

12. The nephrotic syndrome is a clinical complex characterised by a number of reanal and external features , the most prominent are Albumin decrease Hypercoagulability . Hyperlipidemia Edema Lipiduria Proteinuria of ›3.5g/day DEFINATION

13. When the components of the filtration barrier Are disrupted leads to protein loss and worsens renal function Complictions : Albumin : edema Transferin : anemia Vit.D binding globulin : hypocalcemia >convulsions GLOMERULAR PROTIENURIA

14. Due to urinary loss of albumin , liver tries to Compensate this protein loss by increasing the synthesis of albumin as well as other molecules like VLDL nd LDL contributing to development of Hperlipidemia . Protien synthesis and lipid accumulation: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2224672/ HYPOALBUMINEA nd HYPERLIPIDEMIA

15. It results from the loss of inhibitors of coagulation in the urine and increased synthesis of procoagulatory factors by the liver. COMPLICATIONS: Renal vein thrombosis Peripheral vein thrombosis Cerebral vein thrombosis HYPERCOAGUBALITY

16. It is due to a combination of a decrease in oncotic pressure from the hypoalbuminaemia as well a primary renal sodium retention in the collecting tubules. OEDEMA

17. Patients with nephrotic syndrome are at increased risk of infections due to loss of immunoglobulins and complement being lost in the urine. RISK OF INFECTIONS

18. Minimal change disease- tissue normal under microscope Focal segmental glomerulosclerosis - scarring of glomeruli Membraneous nephropathy-thickened membrane Diabetic kidney disease Systemic lupus erythematosus Amyloidosis-amyloid build up Blod clot in renal vein CHF are responsible for increasing the glomerular permiability and protienuria . PATHOGENESIS

19. MANAGEMENT Bed rest in edema Regulate body water balance by Salt and fluid restriction depending upon edema Replace the protein loss by having intake of protiens , avoid high intake of protiens to prevent any tubular damage. Fat intake should also be low Calcium intake of 800mg/day either by diet or a tablet with VIT-D is necessary. Iron supplementation

20. Diuretics is a double edge weapon, so use with caution Use spironolactone if given with hydrochlorothiazide @ 4mg/kg NSIDS, ACE inhibitors reduce protienuria (selective COX2inhibition decreses protienuria : www.medscpe.com/viewarticle/704078 ) Vitamin D Lipid lowering drugs Anticoagulants Pharmcolocgical treatment

21. Prednisolone 1mg/kg in children as a single or divided doses for 4 weeks and then single dose in the morning on alternate days. If proteinuria relapses then the child is steroid dependent , continue prednisolone for 8 weeks and taper the dose, so that the entire course lasts for 6 months If resistant to prednisolone , cyclophosphamide 2mg/kg, cyclosporin,chlorambucil are used to retard progression. INITIAL THERAPY

22. Blocks the action of migratory inhibiting factor (MIF) and chemotactic factor, inhibits the endothelial adherence of macrophages and leukocytes , blocks the antigen processing function of macrophages, stablises lysosomal membrne and prevents the increase in capillary permiability and diapedesis . What does glucocorticoids do....?

23. NATURAL HUMAN STEROID HARMONES: GLUCOCORTICOIDS Cortisol MINERALOCORTICOIDS Aldosterone SEX STEROIDS Androgens Estrogens progestagens

24. COMPLICATIONS OF PREDNISOLONE

25. Nephrotic Syndrome is associated with immune complexes depositing in the kidneys. When you get fever, cold or infection, excessive immune complexes come into being and deposit in the kidneys, triggering the relapse of Nephrotic Syndrome. Antibiotics can kill the bacteria invading into your body so as to reduce their damage to your body. ANTIBIOTICS FOR NEPHROTIC SYNDROME

26. If you do not want to use antibiotics, you should improve your immunity to fight against infections. In the daily, you should do some gentle exercise, such as walking, jogging, yoga and tai chi to strengthen your immunity. You should also add fresh vegetables and fruits into your diet, which is packed with rich vitamins, which can also boost your immune system SKIP ANTIBIOTIC USE…?

27. Gracias……