Overview Of Nephrotic Syndrome
This document was uploaded by user and they confirmed that they have the permission to share it. If you are author or own the copyright of this book, please report to us by using this DMCA report form. Report DMCA
Overview
Download & View Overview Of Nephrotic Syndrome as PDF for free.
More details
- Words: 1,119
- Pages: 19
Overview of Nephrotic Syndrome (NS) BY CJ Lau 3 June 2009
1
Background 1. 2. 3. 4.
Is not a disease but a collection of symptoms Not be to confused with nephritic syndrome Also known as nephrosis Characterized by : Proteinuria Hypoalbuminemia Hyperlipidemia edema
2
Classification Nephrotic syndrome
Primary/idiopathic NS (gromerular diseases intrinsic to the kidney)
Secondary (etiology extrinsic to the kidney)
-Minimal change nephrotic syndrome (MCNS)-Hepatitis B & C -Focal segmental glomerulosclerosis (FSGS) -Diabetes mellitus -Syphilis -Membranous nephropathy (MN) -Systemic Lupus Erythematous -drug exposure (eg. lithium & mercury) 3
Pathophysiology
proteinuria- concentration of heparan sulfate(ve charged) in the basement membrane is lower in NS patients, filtration of plasma protein(-ve charged) through glomerular membrane is prevented by a negatively charged filtration barriers Hypoalbuminemia-glomerular capillary permeability to albumin is selectively increased, leads to hyperalbuminurea and eventually hypoalbuminemia
4
Pathophysiology
Edema- hypoalbuminemia lowers the plasma colloid osmotic pressure, causing transcapillary filtration of water and the development of edema Hyperlipidemia- hypoproteinemia stimulates generalized protein synthesis in liver including lipoprotein.
5
Epidemiology
9th leading cause of death in Sarawak general hospital in 2006 (3.41% of total death) Children at any age and adults can get it, but usually children between the age of 1 ½ -4 are more prone Boys are twice more susceptible than girls In adults, male-female prevalence is approximately equal
6
Clinical Presentation
Increasing edema over a few days or weeks, lethargy, poor appetite and occasional abdominal pain Edema is the predominant feature, initially develops around eyes & lower extremities, becomes generalized and leads to weight gain Decline in urine output and frothy urine Hematuria and hypertension are unusual
7
Diagnosis
First morning urine protein/creatinine value greater than 2-3mg/mg or 24hours urine protein level of more than 40mg/m2/hr Serum albumin level less than 2.5g/dL (normal 3.5-5g/dL) Elevated total cholesterol, low density lipoprotein (LDL) and triglycerides. Normal or low high density lipoprotein (HDL). Check creatinin and urea level to evaluate renal function
8
Treatment
Renal biopsy – not indicated for INS in children prior to starting corticosteroid therapy. It is indicated for steroid resistant NS (not achieving remission despite 4weeks of adequate corticosteroid therapy). Other indication would depends on presence of features to suggest other renal diseases such as persistent hypertension and haematuria. Decision is up to attending pediatrician in consultation with the pediatric nephrologists.
9
Management of edematous state
Antibiotics- penicillin V 125mg BD (1-5years old),250mg BD (6-12years old) and 500mg BD (>12years old) is recommended during relapse particularly with gross edema. Diet-A normal protein diet with adequate calories is recommended. No added salt to the diet during the edematous state. Fluid restriction- Not usually recommended except in chronic edematous state. Diuretics- Frusemide is usually not necessary in steroid responsive NS but if require should be used with caution as it can precipitate hypovolaemia.
10
Management of edematous state
Human albumin (20-25%) at 0.5-1.0g/kg can be used in symptomatic edematous state together with IV frusemide at 1-2mg/kg to produce a diuresis. Urine output and blood pressure should be closely monitored. Human albumin at 0.5-1.0g/kg of 5%, 20% or 25% (whichever is available) over one hour should be given in those suspected to have hypovolaemia. Do not give frusemide in this instant.
11
Corticosteroids
Effective in inducing remission of NS Prednisolone dosage orally for children60mg/m2/day or 2mg/kg/day in divided dose 34times (maximum of 80mg/day) for 4 weeks. Followed by 40mg/m2/dose or 11.5mgmg/kg/alternate day for 4weeks. Reduce prednisolone dose by 25% monthly over next 4months. Monitor blood pressure, blood glucose. 95% of children will achieve remission defined as urine dipstick in trace or nil for 3 consecutive days within 28days . 12
Treatment of relapse
Relapse is defined by urine albumin excretion more than 40mg/m2/hour, dipstick of 2+ or more for 3 consecutive days. Prednisolone 60mg/m2/day until remission then 40mg/m2/dose for 4 weeks and off. Frequent relapse -2 or more relapses within 6 months of initial response. Treatment-Prednisolone 60mg/m2/day till nil albumin in urine for 3 days then 40mg/m2/alternate mornings for 4weeks. Taper prednisolone dose every 2weeks and keep on as low alternate day dose as possible for 6 months.
13
Management of steroid dependent
Defined as 2 consecutive relapses ocurring during the period of steroid taper or 14 days of its cessation. If a child is not steroid toxic (eg.cushing syndrome), reinduce with steroids and maintain on as low dose of alternate day prednisolone as possible as possible. If child is steroid toxic, consider cyclophosphomide therapy. 14
Steroid resistant NS
Refer for renal biopsy- specific treatment will depend on the histopathology.
15
Cyclophosphamide
An alkylating agent, also possess potent immunosuppressive activity. Indicated for the treatment of steroid dependent nephrotic syndrome with sigh of steroid toxicity. Dose- Oral: 2-3mg/kg/day everyday for up to 12weeks Monitoring parameters- Complete blood count with blood count, serum creatinine Adverse effects-hemorrhagic cystitis (drink plenty of fluids, avoid taking drug at night), leucopenia
16
Complication of NS
Infection - due to low IgG level and impaired T-cell functions in INS patients. Medications used to treat NS (corticosteroids) further suppress immune system. Patients are at risk of infection, especially with Streptococcus Pneumonia. Patients infected with varicella should be treated with acyclovir. Thromboembolism- renal vein thrombosis, deep vein thrombosis, and pulmonary thrombosis are common due to increase platelet activation and aggregation, decreased antithrombin III, protein C & S which leads to hypercoagulable state in INS. Following tromboembolism, warfarin is often prescribed for a period of 6 months. Some practitioners advocate the use of low dose aspirin in chronic NS patients, however, adequate clinical trials examining the use of aspirin have not been performed.
17
Patient education
Home monitoring of first morning urine protein with urine dipstick Weight should be checked and a home logbook should be kept (include the steroid dose taken) Consult doctor if edema, weight gain, or proteinurea persist for more than 2days Control fluid (urine volume+500ml) and salt intake Yearly influenza vaccination and pneumococcal vaccination (every 5years) are recommended.
18
References
http://emedicine.medscape.com/article/982920-overview http://www.myhealth.gov.my/myhealth/bm/dewasa_content.jsp?lang=dewas http://www.kidney.org/Atoz/atozItem.cfm?id=53 (national kidney foudation) http://www.hkl.gov.my/content/hfacts/death2007.htm Drug information Handbook, Lexi-Comp http://mpaeds.org.my/PaediatricProtocols/46.%20Nephrotic%20Syndrome.pd http://mpaeds.org.my/PaediatricProtocols/46.%20Nephrotic%20Syndrome.p Consensus of statement – Management of idiopathic nephrotic syndrome in childhood. Ministry of Health. Academy of Medicine Malaysia 1999. Hodson EM, Knight JF, Willis NS, Craig JC. Corticosteroid therapy for nephrotic syndrome in children. The Cochrane library, Issue 1,2003.Oxford Durkan A, Hodson E, Willis N, Craig J. Non corticosteroid treatment for nephrotic syndrome in children. The Cochrane library, Issue 1, 2003, Oxford.
19
1
Background 1. 2. 3. 4.
Is not a disease but a collection of symptoms Not be to confused with nephritic syndrome Also known as nephrosis Characterized by : Proteinuria Hypoalbuminemia Hyperlipidemia edema
2
Classification Nephrotic syndrome
Primary/idiopathic NS (gromerular diseases intrinsic to the kidney)
Secondary (etiology extrinsic to the kidney)
-Minimal change nephrotic syndrome (MCNS)-Hepatitis B & C -Focal segmental glomerulosclerosis (FSGS) -Diabetes mellitus -Syphilis -Membranous nephropathy (MN) -Systemic Lupus Erythematous -drug exposure (eg. lithium & mercury) 3
Pathophysiology
proteinuria- concentration of heparan sulfate(ve charged) in the basement membrane is lower in NS patients, filtration of plasma protein(-ve charged) through glomerular membrane is prevented by a negatively charged filtration barriers Hypoalbuminemia-glomerular capillary permeability to albumin is selectively increased, leads to hyperalbuminurea and eventually hypoalbuminemia
4
Pathophysiology
Edema- hypoalbuminemia lowers the plasma colloid osmotic pressure, causing transcapillary filtration of water and the development of edema Hyperlipidemia- hypoproteinemia stimulates generalized protein synthesis in liver including lipoprotein.
5
Epidemiology
9th leading cause of death in Sarawak general hospital in 2006 (3.41% of total death) Children at any age and adults can get it, but usually children between the age of 1 ½ -4 are more prone Boys are twice more susceptible than girls In adults, male-female prevalence is approximately equal
6
Clinical Presentation
Increasing edema over a few days or weeks, lethargy, poor appetite and occasional abdominal pain Edema is the predominant feature, initially develops around eyes & lower extremities, becomes generalized and leads to weight gain Decline in urine output and frothy urine Hematuria and hypertension are unusual
7
Diagnosis
First morning urine protein/creatinine value greater than 2-3mg/mg or 24hours urine protein level of more than 40mg/m2/hr Serum albumin level less than 2.5g/dL (normal 3.5-5g/dL) Elevated total cholesterol, low density lipoprotein (LDL) and triglycerides. Normal or low high density lipoprotein (HDL). Check creatinin and urea level to evaluate renal function
8
Treatment
Renal biopsy – not indicated for INS in children prior to starting corticosteroid therapy. It is indicated for steroid resistant NS (not achieving remission despite 4weeks of adequate corticosteroid therapy). Other indication would depends on presence of features to suggest other renal diseases such as persistent hypertension and haematuria. Decision is up to attending pediatrician in consultation with the pediatric nephrologists.
9
Management of edematous state
Antibiotics- penicillin V 125mg BD (1-5years old),250mg BD (6-12years old) and 500mg BD (>12years old) is recommended during relapse particularly with gross edema. Diet-A normal protein diet with adequate calories is recommended. No added salt to the diet during the edematous state. Fluid restriction- Not usually recommended except in chronic edematous state. Diuretics- Frusemide is usually not necessary in steroid responsive NS but if require should be used with caution as it can precipitate hypovolaemia.
10
Management of edematous state
Human albumin (20-25%) at 0.5-1.0g/kg can be used in symptomatic edematous state together with IV frusemide at 1-2mg/kg to produce a diuresis. Urine output and blood pressure should be closely monitored. Human albumin at 0.5-1.0g/kg of 5%, 20% or 25% (whichever is available) over one hour should be given in those suspected to have hypovolaemia. Do not give frusemide in this instant.
11
Corticosteroids
Effective in inducing remission of NS Prednisolone dosage orally for children60mg/m2/day or 2mg/kg/day in divided dose 34times (maximum of 80mg/day) for 4 weeks. Followed by 40mg/m2/dose or 11.5mgmg/kg/alternate day for 4weeks. Reduce prednisolone dose by 25% monthly over next 4months. Monitor blood pressure, blood glucose. 95% of children will achieve remission defined as urine dipstick in trace or nil for 3 consecutive days within 28days . 12
Treatment of relapse
Relapse is defined by urine albumin excretion more than 40mg/m2/hour, dipstick of 2+ or more for 3 consecutive days. Prednisolone 60mg/m2/day until remission then 40mg/m2/dose for 4 weeks and off. Frequent relapse -2 or more relapses within 6 months of initial response. Treatment-Prednisolone 60mg/m2/day till nil albumin in urine for 3 days then 40mg/m2/alternate mornings for 4weeks. Taper prednisolone dose every 2weeks and keep on as low alternate day dose as possible for 6 months.
13
Management of steroid dependent
Defined as 2 consecutive relapses ocurring during the period of steroid taper or 14 days of its cessation. If a child is not steroid toxic (eg.cushing syndrome), reinduce with steroids and maintain on as low dose of alternate day prednisolone as possible as possible. If child is steroid toxic, consider cyclophosphomide therapy. 14
Steroid resistant NS
Refer for renal biopsy- specific treatment will depend on the histopathology.
15
Cyclophosphamide
An alkylating agent, also possess potent immunosuppressive activity. Indicated for the treatment of steroid dependent nephrotic syndrome with sigh of steroid toxicity. Dose- Oral: 2-3mg/kg/day everyday for up to 12weeks Monitoring parameters- Complete blood count with blood count, serum creatinine Adverse effects-hemorrhagic cystitis (drink plenty of fluids, avoid taking drug at night), leucopenia
16
Complication of NS
Infection - due to low IgG level and impaired T-cell functions in INS patients. Medications used to treat NS (corticosteroids) further suppress immune system. Patients are at risk of infection, especially with Streptococcus Pneumonia. Patients infected with varicella should be treated with acyclovir. Thromboembolism- renal vein thrombosis, deep vein thrombosis, and pulmonary thrombosis are common due to increase platelet activation and aggregation, decreased antithrombin III, protein C & S which leads to hypercoagulable state in INS. Following tromboembolism, warfarin is often prescribed for a period of 6 months. Some practitioners advocate the use of low dose aspirin in chronic NS patients, however, adequate clinical trials examining the use of aspirin have not been performed.
17
Patient education
Home monitoring of first morning urine protein with urine dipstick Weight should be checked and a home logbook should be kept (include the steroid dose taken) Consult doctor if edema, weight gain, or proteinurea persist for more than 2days Control fluid (urine volume+500ml) and salt intake Yearly influenza vaccination and pneumococcal vaccination (every 5years) are recommended.
18
References
http://emedicine.medscape.com/article/982920-overview http://www.myhealth.gov.my/myhealth/bm/dewasa_content.jsp?lang=dewas http://www.kidney.org/Atoz/atozItem.cfm?id=53 (national kidney foudation) http://www.hkl.gov.my/content/hfacts/death2007.htm Drug information Handbook, Lexi-Comp http://mpaeds.org.my/PaediatricProtocols/46.%20Nephrotic%20Syndrome.pd http://mpaeds.org.my/PaediatricProtocols/46.%20Nephrotic%20Syndrome.p Consensus of statement – Management of idiopathic nephrotic syndrome in childhood. Ministry of Health. Academy of Medicine Malaysia 1999. Hodson EM, Knight JF, Willis NS, Craig JC. Corticosteroid therapy for nephrotic syndrome in children. The Cochrane library, Issue 1,2003.Oxford Durkan A, Hodson E, Willis N, Craig J. Non corticosteroid treatment for nephrotic syndrome in children. The Cochrane library, Issue 1, 2003, Oxford.
19
Related Documents
Overview Of Nephrotic Syndrome
June 2020 10
Nephrotic Syndrome
July 2020 9
Nephrotic Syndrome
July 2020 9
Nephrotic Syndrome
December 2019 14
Pathogenesis Of Nephrotic Syndrome
May 2020 22