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Nephrotic syndrome.pptx

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Nephrotic syndrome is a kidney condition characterized by heavy proteinuria, hypoalbuminemia, edema, and hyperlipidemia. It can be caused by primary kidney diseases like minimal change disease or secondary causes such as diabetic nephropathy. Patients present with edema, thromboembolic risks, infections, malnutrition, and bone fractures due to proteinuria. Diagnosis involves urine protein estimation through 24-hour urine collection or spot urine protein:creatinine ratio. Treatment focuses on managing the underlying cause, complications, and reducing proteinuria with ACE inhibitors.

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NEPHROTIC SYNDROME
WHAT IS IT? A glomerular disease characterized by heavy protreinuria (>3.5gm/day) with hypoalbuminaemia, oedema, hyperlipidaemia and lipiduria
CAUSES OF NEPHROTIC SYNDROME A)PRIMARY CAUSES Nephrotic syndrome due to kidney disease: 1. Minimal change disease (MCD) 2. Focal segmental GN(FSGN) 3. Membranoproliferative GN(MPGN) 4. Membranous GN (MGN B) SECONDARY CAUSES 1. Diabetic nephropathy 2. SLE 3. Amyloidosis 4. PSGN 5. Malignancy 6. Drugs (gold, NSAID, penecillamine) 7. HIV
CLINICAL PRESENTATION 1. OEDEMA 2. THROMBOEMBOLIC EVENT a) Deep venous thrombosis (DVT) b)Pulmonary embolism (PE) c) Renal vein thrombosis (RVT) 3. Infection 4. Malnutrition 5. Bone fracture 6. Uncommonly hypertension 7. Rarely uraemia 8. Primary disease causing NS
PROTEINURIA ESTIMATION NEPHROTIC SYNDROME Urinary protein excretion can be quantified by: A) 24hr urine collection, which is: 1. Difficult 2. Cumbersome and 3. Expensive B) Random spot urine 1. Protein:creatinine ratio 2. Albumin:creatinine ratio
24 HOUR URINE COLLECTION Ask the patient to: 1. Collect a special bottle from the laboratory 2. Chose a day for collection, say Sunday. 3. Must empty the bladder in the toilet on Sunday at 8am. 4. Thereafter, MUST, pass all the urine in the bottle. 5. Must empty the bladder into the bottle on Monday at 8am. 6. Any urine passed after 8am on Monday must be discarded.
RANDOM SPOT URINE 1. IT IS EASY AND CONVINIENT. 2. IT MEASURES URINARY PROTEIN IN RELATION TO CREATININE. 3. A URINE:CREATININE RATIO OF 5 IS CONSISTENT WITH 5G PER DAY PER 1.73M族
PROTEINURIA AND OEDEMA IN NEPHROTIC SYNDROME 1. Proteinuria leads to hypoalbuminaemia 2. Hypoalbuminaemia leads to reduction in intravascular oncotic pressure. 3. Low oncotic pressure leads to movement of fluid into the interstitial space. 4. Fall in the intravascular volume stimulates release of renin and aldosterone 5. This leads to sodium and water retention. 6. The oedema worsens
NEPHROTIC SYNDROME AND LIPIDS 1. IN INTRAVASCULAR ONCOTIC PRESSURE 2. LIVER SYNTHESIS OF PROTEINS INCLUDING LIPOPROTEINS 3. THESE ARE FILTERED IN THE GBM. 4. HENCE: a) Hyperlipidaemia leading to accelerated atherosclerosis, seen commonly in NS. b) Hyperlipiduria
THROMBOEMBOLISM AND NEPHROTIC SYNDROME Hypercoagulabilty is very common in nephrotic syndrome due to many factors: 1. Urinary loss of anticoagulants: a) Antithrombin 111. b) Protein S c) Protein C 2. Platelet hyperaggregability 3. liver synthesis of procoagulant proteins COMPLICATIONS: a) Deep venous thrombosis (DVT) b) Pulmonary embolism (PE) c) Renal vein thrombosis (RVT)
NEPHROTIC SYNDROME AND BONE FRACTURES 1. Urinary loss of 25(OH)D binding protein. 2. 25(OH)D 3. Deficiency of 1,25(OH)D 4. Secondary hyperparathyroidism (PTH) 5. Bone fracture.
NEPHROTIC SYNDROME AND INFECTION The urinary protein loss in nephrotic syndrome may include loss of: a) Immunoglobulins(IgG). b) Compliment proteins c) Impaired cell mediated immunity This leads to: 1. IgG deficiency 2. Poor bacterial opsonization. Increased risk of infection by encapsulating bacteria (Strep pneumonia, H. influenzae, E.Coli)
LABORATORY EVALUATION OF NEPHROTIC SYNDROME If the primary disease is know, like DM, further unnecessary tests are not beneficial. Some patients may require further evaluation: FBC Renal immunological studies including C3, C4 levels Serum immunoelectrophoressis Urinary immunoelectrophoresis CXR Stool for occult blood Hepatitis serology HIV Renal biopsy in adults
MANAGEMENT OF NEPHROTIC SYNDROME This depends on the cause. TREAT THE: a) Underlying disease (or secondary cause) b) Complications (oedema, hyperlipidaemia, infection, thrombosis, hypertenstion, renal failure) c) Proteinuria
TREATMENT OF PROTEINURIA 1. ACEI OR ARB. 2. LOW SALT DIET. 3. VITAMIN D SUPPLEMENT
THROMBOEMBOLISM IN NEPHROTIC SYNDROME 1. Use of anticoagulant remains contraversal. 2. Start with Heparin and warfarin. 3. Cease Heparin on the third day. 4. Continue with oral. 5. Monitor INR
OEDEMA MANAGEMENT IN NEPHROTIC SYNDROME 1. DAILY WEIGHT 2. INPUT OUTPUT CHART 3. USE HIGH DOSE LOOP DIURETICS I.V. 4. ADD THIAZIDE DIURETICS IF POOR RESPONSE. 5. MONITOR THE ELECTROLYTES 6. REPLACE POTASSIUM IF TOO LOW.
END
MINIMAL CHANGE DISEASE (MCD) Definition: Kidney disease characterized by nephrotic syndrome with normal histology on light microscopy and immunofluorescence but an effacement and widening of epithelial cell foot processes(podocytes)on electron microscopy.
CAUSES OF MCD 1. Not known 2. Viral or bacterial infection 3. Activation of immune response leading to formation of Immune-complex disease. 1. This explains why it has good response to treatment with steroids.
INCIDENCE OF MCD 1. COMMON CAUSE OF IDIOPATHIC NS: a) Adult 10 20% b) Children 70 90% 2. SEX: MALES > FEMALES (3:2 ratio) 3. Age group: 2 6 years
CLINICAL PRESENTATION OF MCD 1. OVERT NEPHROTIC SYNDROME 2. NORMAL BLOOD PRESSURE 3. MILDLY IMPAIRED RENAL FUNCTION 4. MINIMAL HAEMATURIA 5. HIGHLY SELECTIVE PROTEINURIA 6. NORMAL SERUM C3, C4
DIAGNOSIS OF MCD 1. Presents as nephrotic syndrome in children 2. Good response to therapy 3. Renal biopsy not necessary 4. Is uncommon in older children (>10years), presence of haematuria, AKI, hypertension, raised serum creatinine). In this situation, renal biopsy maybe warranted prior to initiation of therapy. 5. Renal biopsy is mandatory in adults with suspected MCD to make the diagnosis.
STANDARD TREATMENT OF MINIMAL CHANGE DISEASE 1. High dose prednisolone 20mg TID for 2- 6 weeks(to prevent relapse). 2. Reduce prednisolone to 40mg single dose on alternate days for 1 month. 3. Then start tapering over 2 months. 4. Cease the treatment at the end of the 2 months. 5. LSD is recommended
COMPLICATIONS OF MCD 1.Increased risk of infection with encapsulated organisms (Strep pneumonie, E. coli, H. influenzae) due to: 1.1 Loss of antibodies and complement proteins. 1.2 Defective cell mediated immunity. 2. Hypercoagulability 2.1 Venous thrombosis (DVT) 2.2 Arterial thrombosis 2.4 Just due to aggressive diuresis 3. Bone disease 3.1 Vitamin D deficiency 3.2 SHPTH
PROGNOSIS 1. About 75-80% go into remission within 2weeks 2. Approximately 60-75% of children with MCNS will at least have one relapse 3. Relapse rate is much higher in adults 4. Some patients may be steroid dependent ie go into remission, while on treatment but relapse when steroid is ceased.
TREATMENT OUTCOMES OF MCD 1. Complete remission: 1.1 May be spontaneous or 1.2 With steroids therapy. 2. Relapse: 2.1 Frequent relapses ( 2 relapses within 6months) 3. Steroid dependent: Patient goes into remission, but relapses when steroid dose is papered or ceased within 4 weeks.
Treatment of steroids in MCD 1. Repeat doses of steroids 60mg/day in divided doses for 3 days 2. steroid dose to 40mg/day on alternating days for 4 weeks. 3. Continue papering steroids until minimal dose, with no recurrence. 4. Refer to nephrologist for possible imunosuppressive therapy.
END
DIABETIC NEPHROPATHY Definition: Progressive kidney disease due to long standing diabetes mellitus (type 1 or 2), characterized by the presence of proteinuria or frank nephrotic syndrome.
DIABETIC NEPHROPATHY PATHOLOGY . Pathogenesis of diabetic kidney disease is complex and poorly understood. It is basically associated with persistent hyperglycaemia or poorly controlled diabetes mellitus. . It causes unique structural changes in the kidney culminating into nodular glomerulosclerosis, the Kimmelstiel-Wilson nodules. This classical histological finding is pathonognomic of DN, but is only found in 10-20%
RISK FACTORS OF DIABETIC NEPHROPATHY 1. FAMILIAL SUSCEPTIBILITY 2. HYPERGLYCAEMIA 3. PROTEINURIA 4. CIGARATTE SMOKING 5. DYSLIPIDAEMIA
OTHER ASSOCIATED CLINICAL PRESENTATION 1. Long standing history of DM 2. Good or poor glycaemic control. 3. Presence of diabetic retinopathy as in type 1 4. Detection of microscopic proteinuria (albumin) 5. Rarely, presence of microscopic haematuria.
COMORBID ASSOCIATION WITH DIABETIC NEPHROPATHY 1. Diabetic retinopathy 2. Peripheral neuropathy 3. Cardiovascular diseases(coronary heart disease) 4. Vascular diseases(Carotid, atherosclerotic renal artery stenosis)
PREVENTION OF DIABETIC NEPHROPATHY ABSOLUTE CONTROL OF RISK FACTORS: 1.HYPERTENSION 2.HYPERGLYCAEMIA 3.SMOKING 4.DYSLIPIDAEMIA 5.HIGH SALT INTAKE These are also risk factors for CVD
MANAGING DIABETIC NEPHROPATHY 1. HYPERTENSION; 1.1 Start with ACEi or ARB 1.2 Add on CCB (Nondihydropyridine) 1.3 Then beta blocker 2. HYPERGLYCAEMIA 2.1 Aim at HbAiC <6% 2.2 Remember as the GFR deteriorate, the insulin requirement drops. 3. PROTEINURIA 3.1 ACEi or ARB 3.2 Pentoxifylline (an old drug but new function)
END

More Related Content

Nephrotic syndrome.pptx

  • 2. WHAT IS IT? A glomerular disease characterized by heavy protreinuria (>3.5gm/day) with hypoalbuminaemia, oedema, hyperlipidaemia and lipiduria
  • 3. CAUSES OF NEPHROTIC SYNDROME A)PRIMARY CAUSES Nephrotic syndrome due to kidney disease: 1. Minimal change disease (MCD) 2. Focal segmental GN(FSGN) 3. Membranoproliferative GN(MPGN) 4. Membranous GN (MGN B) SECONDARY CAUSES 1. Diabetic nephropathy 2. SLE 3. Amyloidosis 4. PSGN 5. Malignancy 6. Drugs (gold, NSAID, penecillamine) 7. HIV
  • 4. CLINICAL PRESENTATION 1. OEDEMA 2. THROMBOEMBOLIC EVENT a) Deep venous thrombosis (DVT) b)Pulmonary embolism (PE) c) Renal vein thrombosis (RVT) 3. Infection 4. Malnutrition 5. Bone fracture 6. Uncommonly hypertension 7. Rarely uraemia 8. Primary disease causing NS
  • 5. PROTEINURIA ESTIMATION NEPHROTIC SYNDROME Urinary protein excretion can be quantified by: A) 24hr urine collection, which is: 1. Difficult 2. Cumbersome and 3. Expensive B) Random spot urine 1. Protein:creatinine ratio 2. Albumin:creatinine ratio
  • 6. 24 HOUR URINE COLLECTION Ask the patient to: 1. Collect a special bottle from the laboratory 2. Chose a day for collection, say Sunday. 3. Must empty the bladder in the toilet on Sunday at 8am. 4. Thereafter, MUST, pass all the urine in the bottle. 5. Must empty the bladder into the bottle on Monday at 8am. 6. Any urine passed after 8am on Monday must be discarded.
  • 7. RANDOM SPOT URINE 1. IT IS EASY AND CONVINIENT. 2. IT MEASURES URINARY PROTEIN IN RELATION TO CREATININE. 3. A URINE:CREATININE RATIO OF 5 IS CONSISTENT WITH 5G PER DAY PER 1.73M族
  • 8. PROTEINURIA AND OEDEMA IN NEPHROTIC SYNDROME 1. Proteinuria leads to hypoalbuminaemia 2. Hypoalbuminaemia leads to reduction in intravascular oncotic pressure. 3. Low oncotic pressure leads to movement of fluid into the interstitial space. 4. Fall in the intravascular volume stimulates release of renin and aldosterone 5. This leads to sodium and water retention. 6. The oedema worsens
  • 9. NEPHROTIC SYNDROME AND LIPIDS 1. IN INTRAVASCULAR ONCOTIC PRESSURE 2. LIVER SYNTHESIS OF PROTEINS INCLUDING LIPOPROTEINS 3. THESE ARE FILTERED IN THE GBM. 4. HENCE: a) Hyperlipidaemia leading to accelerated atherosclerosis, seen commonly in NS. b) Hyperlipiduria
  • 10. THROMBOEMBOLISM AND NEPHROTIC SYNDROME Hypercoagulabilty is very common in nephrotic syndrome due to many factors: 1. Urinary loss of anticoagulants: a) Antithrombin 111. b) Protein S c) Protein C 2. Platelet hyperaggregability 3. liver synthesis of procoagulant proteins COMPLICATIONS: a) Deep venous thrombosis (DVT) b) Pulmonary embolism (PE) c) Renal vein thrombosis (RVT)
  • 11. NEPHROTIC SYNDROME AND BONE FRACTURES 1. Urinary loss of 25(OH)D binding protein. 2. 25(OH)D 3. Deficiency of 1,25(OH)D 4. Secondary hyperparathyroidism (PTH) 5. Bone fracture.
  • 12. NEPHROTIC SYNDROME AND INFECTION The urinary protein loss in nephrotic syndrome may include loss of: a) Immunoglobulins(IgG). b) Compliment proteins c) Impaired cell mediated immunity This leads to: 1. IgG deficiency 2. Poor bacterial opsonization. Increased risk of infection by encapsulating bacteria (Strep pneumonia, H. influenzae, E.Coli)
  • 13. LABORATORY EVALUATION OF NEPHROTIC SYNDROME If the primary disease is know, like DM, further unnecessary tests are not beneficial. Some patients may require further evaluation: FBC Renal immunological studies including C3, C4 levels Serum immunoelectrophoressis Urinary immunoelectrophoresis CXR Stool for occult blood Hepatitis serology HIV Renal biopsy in adults
  • 14. MANAGEMENT OF NEPHROTIC SYNDROME This depends on the cause. TREAT THE: a) Underlying disease (or secondary cause) b) Complications (oedema, hyperlipidaemia, infection, thrombosis, hypertenstion, renal failure) c) Proteinuria
  • 15. TREATMENT OF PROTEINURIA 1. ACEI OR ARB. 2. LOW SALT DIET. 3. VITAMIN D SUPPLEMENT
  • 16. THROMBOEMBOLISM IN NEPHROTIC SYNDROME 1. Use of anticoagulant remains contraversal. 2. Start with Heparin and warfarin. 3. Cease Heparin on the third day. 4. Continue with oral. 5. Monitor INR
  • 17. OEDEMA MANAGEMENT IN NEPHROTIC SYNDROME 1. DAILY WEIGHT 2. INPUT OUTPUT CHART 3. USE HIGH DOSE LOOP DIURETICS I.V. 4. ADD THIAZIDE DIURETICS IF POOR RESPONSE. 5. MONITOR THE ELECTROLYTES 6. REPLACE POTASSIUM IF TOO LOW.
  • 18. END
  • 19. MINIMAL CHANGE DISEASE (MCD) Definition: Kidney disease characterized by nephrotic syndrome with normal histology on light microscopy and immunofluorescence but an effacement and widening of epithelial cell foot processes(podocytes)on electron microscopy.
  • 20. CAUSES OF MCD 1. Not known 2. Viral or bacterial infection 3. Activation of immune response leading to formation of Immune-complex disease. 1. This explains why it has good response to treatment with steroids.
  • 21. INCIDENCE OF MCD 1. COMMON CAUSE OF IDIOPATHIC NS: a) Adult 10 20% b) Children 70 90% 2. SEX: MALES > FEMALES (3:2 ratio) 3. Age group: 2 6 years
  • 22. CLINICAL PRESENTATION OF MCD 1. OVERT NEPHROTIC SYNDROME 2. NORMAL BLOOD PRESSURE 3. MILDLY IMPAIRED RENAL FUNCTION 4. MINIMAL HAEMATURIA 5. HIGHLY SELECTIVE PROTEINURIA 6. NORMAL SERUM C3, C4
  • 23. DIAGNOSIS OF MCD 1. Presents as nephrotic syndrome in children 2. Good response to therapy 3. Renal biopsy not necessary 4. Is uncommon in older children (>10years), presence of haematuria, AKI, hypertension, raised serum creatinine). In this situation, renal biopsy maybe warranted prior to initiation of therapy. 5. Renal biopsy is mandatory in adults with suspected MCD to make the diagnosis.
  • 24. STANDARD TREATMENT OF MINIMAL CHANGE DISEASE 1. High dose prednisolone 20mg TID for 2- 6 weeks(to prevent relapse). 2. Reduce prednisolone to 40mg single dose on alternate days for 1 month. 3. Then start tapering over 2 months. 4. Cease the treatment at the end of the 2 months. 5. LSD is recommended
  • 25. COMPLICATIONS OF MCD 1.Increased risk of infection with encapsulated organisms (Strep pneumonie, E. coli, H. influenzae) due to: 1.1 Loss of antibodies and complement proteins. 1.2 Defective cell mediated immunity. 2. Hypercoagulability 2.1 Venous thrombosis (DVT) 2.2 Arterial thrombosis 2.4 Just due to aggressive diuresis 3. Bone disease 3.1 Vitamin D deficiency 3.2 SHPTH
  • 26. PROGNOSIS 1. About 75-80% go into remission within 2weeks 2. Approximately 60-75% of children with MCNS will at least have one relapse 3. Relapse rate is much higher in adults 4. Some patients may be steroid dependent ie go into remission, while on treatment but relapse when steroid is ceased.
  • 27. TREATMENT OUTCOMES OF MCD 1. Complete remission: 1.1 May be spontaneous or 1.2 With steroids therapy. 2. Relapse: 2.1 Frequent relapses ( 2 relapses within 6months) 3. Steroid dependent: Patient goes into remission, but relapses when steroid dose is papered or ceased within 4 weeks.
  • 28. Treatment of steroids in MCD 1. Repeat doses of steroids 60mg/day in divided doses for 3 days 2. steroid dose to 40mg/day on alternating days for 4 weeks. 3. Continue papering steroids until minimal dose, with no recurrence. 4. Refer to nephrologist for possible imunosuppressive therapy.
  • 29. END
  • 30. DIABETIC NEPHROPATHY Definition: Progressive kidney disease due to long standing diabetes mellitus (type 1 or 2), characterized by the presence of proteinuria or frank nephrotic syndrome.
  • 31. DIABETIC NEPHROPATHY PATHOLOGY . Pathogenesis of diabetic kidney disease is complex and poorly understood. It is basically associated with persistent hyperglycaemia or poorly controlled diabetes mellitus. . It causes unique structural changes in the kidney culminating into nodular glomerulosclerosis, the Kimmelstiel-Wilson nodules. This classical histological finding is pathonognomic of DN, but is only found in 10-20%
  • 32. RISK FACTORS OF DIABETIC NEPHROPATHY 1. FAMILIAL SUSCEPTIBILITY 2. HYPERGLYCAEMIA 3. PROTEINURIA 4. CIGARATTE SMOKING 5. DYSLIPIDAEMIA
  • 33. OTHER ASSOCIATED CLINICAL PRESENTATION 1. Long standing history of DM 2. Good or poor glycaemic control. 3. Presence of diabetic retinopathy as in type 1 4. Detection of microscopic proteinuria (albumin) 5. Rarely, presence of microscopic haematuria.
  • 34. COMORBID ASSOCIATION WITH DIABETIC NEPHROPATHY 1. Diabetic retinopathy 2. Peripheral neuropathy 3. Cardiovascular diseases(coronary heart disease) 4. Vascular diseases(Carotid, atherosclerotic renal artery stenosis)
  • 35. PREVENTION OF DIABETIC NEPHROPATHY ABSOLUTE CONTROL OF RISK FACTORS: 1.HYPERTENSION 2.HYPERGLYCAEMIA 3.SMOKING 4.DYSLIPIDAEMIA 5.HIGH SALT INTAKE These are also risk factors for CVD
  • 36. MANAGING DIABETIC NEPHROPATHY 1. HYPERTENSION; 1.1 Start with ACEi or ARB 1.2 Add on CCB (Nondihydropyridine) 1.3 Then beta blocker 2. HYPERGLYCAEMIA 2.1 Aim at HbAiC <6% 2.2 Remember as the GFR deteriorate, the insulin requirement drops. 3. PROTEINURIA 3.1 ACEi or ARB 3.2 Pentoxifylline (an old drug but new function)
  • 37. END