Nephrotic
syndrome ( SN ) is one of the clinical manifestations of
glomerulonephritis ( GN ) is characterized by anarsarka edema , massive
proteinuria ≥ 3.5 g / day , hypercholesterolemia and lipiduria . ( 1.2 )At the beginning of the process or SN light , to establish the diagnosis are not all symptoms are found . Massive proteinuria is a typical sign on SN SN but heavy with low albumin levels , urinary protein excretion was also reduced . Proteinuria also contribute to a variety of complications that occur in SN . ( 1.2 )Hypoalbuminemia
, hyperlipidemia and lipiduria , nitrogen balance disorders ,
hypercoagulability , disorders of calcium and bone metabolism and
thyroid hormone often found in SN . Generally , SN with normal renal function but most cases develop into end stage renal disease ( PGTA ) . In some episodes of SN can heal itself and shows a good respone to steroid therapy but others can develop into chronic . ( 1.2 )
etiologyNephrotic syndrome can be caused by primary and GN GN secondary to infection , malignancy , connective tissue disease (connective tissue disease) , caused by drugs or toxins and systemic disease as listed in Table 1 .Table 1 Classification and Causes of Nephrotic Syndrome :Primary glomerulonephritis-0 GN minimal lesions ( GNLM )-1 Focal glomerulosclerosis ( GSF )-2 Membranous GN ( GNMN )GN Membranoploriferatif -3 ( GNMP )Proliferative GN -4 anotherGlomerulonephritis secondary to :infection :-5 HIV , hepatitis B and C virus-6 Syphilis , malaria , skistosoma-7 Tuberculosis , leprosymalignancy :Adenocarcinoma of the lung , breast , colon , Hodgkin's lymphoma , multiple myeloma , and renal carcinoma .Connective tissue diseaseSystemic Lupus Erythematosus , Rheumatoid Artritia , MCTD (mixed connective tissue disease)Effects of drugs and toxinsNon - steroidal anti-inflammatory drugs , gold preparations , penisilinamin , probenecid , mercury , kaptpril , heroin .Other:Diabetes mellitus , amyloidosis , pre - eclampsia , chronic allograft rejection , vesicoureteric reflux , or bee stings .Primary or idiopathic glomerulonephritis is the most common cause of SN . In primary GN group , consisting of : ( a) GN minimal lesions ( GNLM ) often in children - children , ( b ) focal glomerulosclerosis ( GSF ) , ( c ) membranous GN ( GNMN ) often in adults and ( d ) GN Membranoproliferative ( GNMP ) . ( 1.2 )Glomerulonephritis secondary to ineksi frequently encountered for example in post- infectious GN streptococcus virus or hepatitis B virus infection , caused by medications such as nonsteroidal anti-inflammatory drugs or preparations organic gold , and due to systemic diseases such as systemic lupus erythematosus and in diabetes mellitus . ( 1.2 ) .pathophysiologyReaction of antigen - antibody causes increased permeability of the glomerular basement membrane followed by leakage of protein ( albumin ) .Proteinuri :Proteinuri a basic abnormality SN . Proteinuri largely derived from leakage glomerulus ( glomerular Proteinuri ) and only a fraction derived from tubular secretion ( Proteinuri tubular ) . Changes in glomerular basal membrane integrity causes increased glomerular permeability to plasma proteins and major protein excreted in the urine is albumin . Another protein that is excreted thyroid binding globulin , IgG , IgA , antithrombin III and protein binding of vitamin D. ( 1.2 )Proteinuri degree is not directly related to the severity of glomerular damage . Passage of plasma proteins larger than 70 kDa through the glomerular basal membrane is normally limited by the charge selective barrier ( a polyanionic glycosaminoglycan ) and size selective barrier . At minimal lesion nephropathy , Proteinuri caused mainly by the loss of charge selectivity whereas the membranous nephropathy caused mainly by the loss of size selectivity . ( 1.2 )HipoalbuminemiThis situation is caused by the loss of a number of proteins the body through the urine ( proteinuria ) and intestine ( protein loosing enteropathy ) , katabolisma albumin , a protein intake less kerana appetite decreased and utilization of amino acids accompanying decline in renal physiology . If compensation in the hepatic synthesis of albumin is inadequate , will happen hipoproteinemi . ( 1.2 )Plasma albumin concentration determined by protein intake , liver albumin synthesis and protein loss in the urine . On SN , hypoalbuminemia caused by the massive protenuria due to decreased plasma oncotic pressure . Thus , to maintain plasma oncotic pressure , the heart trying to increase albumin synthesis . Increased liver albumin synthesis could not deter the onset of hypoalbuminemia . High-protein diet can improve liver albumin synthesis . But still can encourage increased albumin excretion through the urine. Hypoalbuminemia may also occur due to increased reabsorption and catabolism of albumin by the proximal tubule . ( 1.2 )HiperlipidemiSerum cholesterol , very low density lipoprotein ( VLDL ) , low density lipoprotein ( LDL ) , triglycerides while increasing high-density lipoprotein ( HDL ) may be increased , normal or decreased . This is due to an increase in hepatic lipid synthesis and decreased catabolism in peripheral ( decrease spending lipoprotein , VLDL , intermediate -density lipoproteins and chylomicrons from the blood ) . ( 3 )Increased lipoprotein lipid synthesis is stimulated by a decrease in serum albumin and a decrease in oncotic pressure . ( 3 )LipiduriFat -free ( oval fat bodies) are often found in the urine sediment . The fat sources come from the filtrate glomerular basal membrane lipoproteins through the permeable . ( 3 )edemaUnderfil theory explains that hypoalbuminemia is a major contributing factor to the occurrence of edema in the SN . Hypoalbuminemia causes a decrease in plasma oncotic pressure so that fluid shifts from the intravascular to the network interstisium and edema . By kerana , the kidneys compensate by increasing sodium and water retention . This compensation mechanism will improve intravascular volume but will also exacerbate the occurrence of edema hypoalbuminemia so heavier. ( 1,2 , )Overfill theory explains that sodium retention as a primary renal defect . Retention of sodium by the kidneys, causing increased extracellular fluid , causing edema . Decline in glomerular filtration rate due to damage to the kidneys will increase sodium retention and edema . The second mechanism was found in SN patients . ( 1.2 )Some explanation is trying to combine these two theories , for example, stated that edema formation is a dynamic process . Found that plasma volume decreased significantly during the formation of edema and increased during the phase of diuresis . ( 1.2 )hypercoagulabilityThis situation is caused by the loss of antithrombin ( AT ) III , protein S , C and plasminogen activating factor in the urine and increased factor V , VII , VIII , X , platelets , fibrinogen , increased platelet aggregation , changes in endothelial cell function and reduced zimogen factor ( factor IX , XI ) . ( 3 )Susceptibility to infectionDecreased levels of immunoglobulin Ig G and Ig A due to loss through the kidneys , decreased synthesis and increased catabolism causes increased susceptibility to encapsulated bacterial infections such as Streptococcus pneumonia , Klebsiella , Haemophilus . At SN also disruption of T cell -mediated immunity Frequent bronkopneumoni and peritonitis . ( 3 )Clinical manifestations ( 1,2,3,4 )Protenuria : > 3.0 gr/24 hours . Changes on the basis of the glomerular membrane causes an increase in glomerular permebilitas to plasma proteins are albumin .Hypoalbuminemia : serum albumin 3.5 g / 1.73 m2 body surface area / day ) , hipoalbuminemi ( < 3 g / dl ) , edema , hiperlipidemi , lipiduri and hypercoagulability . Additional examinations such as venography is necessary to establish the diagnosis of venous thrombosis which can occur due to hypercoagulability . In the SN to determine the type of primary renal histopathologic abnormalities that determine prognosis and response to therapy , renal biopsy is necessary .Management ( 1,2,3 )SN treatment consists of specific treatment directed against basic diseases and non - specific treatment to reduce protenuria , control edema and treat complications . Secondary etiology of nephrotic syndrome should be sought and given therapy , da drugs are the cause removed .a) . diureticsAnsa Henle diuretics ( loop diuretics ) such as furosemide ( initial dose of 20-40 mg / day ) or a thiazide class with or without combination with a potassium -sparing diuretics ( spironolactone ) is used to treat edema and hypertension . Weight loss should not exceed 0.5 kg / day .b ) . Diet .Diet for patients SN is 35 cal / kg . / Day , consisting mostly of carbohydrates . Low-salt diet ( 2-3 g / day ) , low-fat should be given . Research has shown that in patients with certain kidney disease , a low intake of protein is safe , can reduce proteinuria and slow the loss of kidney function , possibly by lowering the pressure intraglomerulus . The degree of protein restriction will be recommended in patients who lack the protein due to nephrotic syndrome have not been established . Restriction of intake of 0.8-1.0 g protein / kg / day can reduce proteinuria . Additional vitamin D can be given if the patient is deficient in vitamins .c ) Anticoagulant TherapyWhen diagnosed with the events of thromboembolism , anticoagulant therapy with heparin should be initiated . The number of heparin are required to achieve a partial thromboplastin time ( PTT ) therapeutic may increase due to decrease in number of antithrombin III . After intravenous heparin therapy , oral anticoagulation with warfarin was continued until the nephrotic syndrome can be overcome .d ) Drug TherapySpecific therapy for nephrotic syndrome are the corticosteroids prednisone of 1 - 1.5 mg / kg / day single dose of the morning during 4-6 weeks . Then reduced 5 mg / week to achieve maintenance dose ( 5-10 mg ) was then given 5 mg within a day and stopped in 1-2 weeks . If at the time of tapering off , the state of the patient deteriorated again ( arising edema , protenuri ) , given back full dose for 4 weeks and then tapering off again . Corticosteroid drugs of choice to deal with nephrotic syndrome ( prednisone , methyl prednisone ), especially on minimal glomerular lesions ( MGL ) , focal segmental glomerulosclerosis ( FSG ) and systemic lupus glomerulonephritis . Nonsteroidal anti-inflammatory drugs ( NSAIDs ) have been used in patients with membranous nephropathy and focal glomerulosclerosis to reduce the synthesis of prostaglandins that cause dilation . It causes renal vasoconstriction , reduction intraglomerulus pressure , and in many cases up to 75 % decrease in proteinuria .Sitostatica by administering prednisone is given when there is no response , relapse who repeatedly or the possible side effects of corticosteroids . Cyclophosphamide can be given 1.5 mg / kg / day. Lipid-lowering drugs known as statins such as simvastatin , pravastatin and lovastatin can lower LDL cholesterol , triglycerides and increase HDL cholesterol .Anti proteinurik eg ACE inhibitor ( captopril 3 * 12.5 mg ) , calcium antagonists ( Herbeser 180 mg ) or beta blockers . Angiotensin converting enzyme inhibitors ( angiotensin converting enzyme inhibitors ) and angiotensin II receptor antagonists can reduce blood pressure and the combination has an additive effect in reducing proteinuria .complicationHyperlipidemia is a condition that often accompanies SN . Cholesterol levels generally increased , while triglyceride levels vary from normal to slightly high . Increased cholesterol levels due to increased LDL (low density lipoprotein ) is a type of primary lipoprotein cholesterol transporter . High levels of LDL in the SN due to increased synthesis of liver without liver catabolism disorders . SN mechanism in hyperlipidemia associated with lipid and lipoprotein synthesis increased liver and decreased catabolism . ( 1.2 )Lipiduria often found in SN and is characterized by the accumulation of lipids in the cell debris and cast as oval fat bodies ( oval fat bodies) and fatty casts. Lipiduria more associated with protenuria than with hyperlipidemia . ( 3 )Thromboembolic complications often found in SN due to increased intravascular coagulation . In the SN due GNMP likelihood of renal vein thrombosis is quite high . Pulmonary embolism and deep venous thrombosis ( deep vein thrombosis ) often found in SN . ( 2.3 )Kerana infection by humoral immunity defects , cellular , and disorders of complement system . By the bacteria that are not encapsulated as Haemophilus influenzae and Streptococcus pneumoniae may cause an infection . Decrease in IgG , IgA and gamma globulin are often found in patients kerana SN by decreased synthesis or increased catabolism and increase the amount of waste through urine . ( 2 )Acute renal failure caused by hypovolemia . By kerana fluid accumulates in the tissues of the body , so less fluid in the blood circulation . Decreased blood flow to the kidneys causing kidney can not function properly and the incidence of acute tubular necrosis . ( 1,2,3 )Prognosis ( 5,6 )Prognosis depends on the movement of nephrotic syndrome . In the case of children , the prognosis is very good kerana minimal change disease ( MCD ) gave a very good response to steroid therapy and does not cause kidney failure (chronic renal failure) . But for other causes such as focal segmental glomerulosclerosis ( FSG ) often causes end stage renal disease occurs ( ESRD ) . Factors - other factors that aggravate again nephrotic syndrome is protenuria levels , control blood pressure and renal function .
etiologyNephrotic syndrome can be caused by primary and GN GN secondary to infection , malignancy , connective tissue disease (connective tissue disease) , caused by drugs or toxins and systemic disease as listed in Table 1 .Table 1 Classification and Causes of Nephrotic Syndrome :Primary glomerulonephritis-0 GN minimal lesions ( GNLM )-1 Focal glomerulosclerosis ( GSF )-2 Membranous GN ( GNMN )GN Membranoploriferatif -3 ( GNMP )Proliferative GN -4 anotherGlomerulonephritis secondary to :infection :-5 HIV , hepatitis B and C virus-6 Syphilis , malaria , skistosoma-7 Tuberculosis , leprosymalignancy :Adenocarcinoma of the lung , breast , colon , Hodgkin's lymphoma , multiple myeloma , and renal carcinoma .Connective tissue diseaseSystemic Lupus Erythematosus , Rheumatoid Artritia , MCTD (mixed connective tissue disease)Effects of drugs and toxinsNon - steroidal anti-inflammatory drugs , gold preparations , penisilinamin , probenecid , mercury , kaptpril , heroin .Other:Diabetes mellitus , amyloidosis , pre - eclampsia , chronic allograft rejection , vesicoureteric reflux , or bee stings .Primary or idiopathic glomerulonephritis is the most common cause of SN . In primary GN group , consisting of : ( a) GN minimal lesions ( GNLM ) often in children - children , ( b ) focal glomerulosclerosis ( GSF ) , ( c ) membranous GN ( GNMN ) often in adults and ( d ) GN Membranoproliferative ( GNMP ) . ( 1.2 )Glomerulonephritis secondary to ineksi frequently encountered for example in post- infectious GN streptococcus virus or hepatitis B virus infection , caused by medications such as nonsteroidal anti-inflammatory drugs or preparations organic gold , and due to systemic diseases such as systemic lupus erythematosus and in diabetes mellitus . ( 1.2 ) .pathophysiologyReaction of antigen - antibody causes increased permeability of the glomerular basement membrane followed by leakage of protein ( albumin ) .Proteinuri :Proteinuri a basic abnormality SN . Proteinuri largely derived from leakage glomerulus ( glomerular Proteinuri ) and only a fraction derived from tubular secretion ( Proteinuri tubular ) . Changes in glomerular basal membrane integrity causes increased glomerular permeability to plasma proteins and major protein excreted in the urine is albumin . Another protein that is excreted thyroid binding globulin , IgG , IgA , antithrombin III and protein binding of vitamin D. ( 1.2 )Proteinuri degree is not directly related to the severity of glomerular damage . Passage of plasma proteins larger than 70 kDa through the glomerular basal membrane is normally limited by the charge selective barrier ( a polyanionic glycosaminoglycan ) and size selective barrier . At minimal lesion nephropathy , Proteinuri caused mainly by the loss of charge selectivity whereas the membranous nephropathy caused mainly by the loss of size selectivity . ( 1.2 )HipoalbuminemiThis situation is caused by the loss of a number of proteins the body through the urine ( proteinuria ) and intestine ( protein loosing enteropathy ) , katabolisma albumin , a protein intake less kerana appetite decreased and utilization of amino acids accompanying decline in renal physiology . If compensation in the hepatic synthesis of albumin is inadequate , will happen hipoproteinemi . ( 1.2 )Plasma albumin concentration determined by protein intake , liver albumin synthesis and protein loss in the urine . On SN , hypoalbuminemia caused by the massive protenuria due to decreased plasma oncotic pressure . Thus , to maintain plasma oncotic pressure , the heart trying to increase albumin synthesis . Increased liver albumin synthesis could not deter the onset of hypoalbuminemia . High-protein diet can improve liver albumin synthesis . But still can encourage increased albumin excretion through the urine. Hypoalbuminemia may also occur due to increased reabsorption and catabolism of albumin by the proximal tubule . ( 1.2 )HiperlipidemiSerum cholesterol , very low density lipoprotein ( VLDL ) , low density lipoprotein ( LDL ) , triglycerides while increasing high-density lipoprotein ( HDL ) may be increased , normal or decreased . This is due to an increase in hepatic lipid synthesis and decreased catabolism in peripheral ( decrease spending lipoprotein , VLDL , intermediate -density lipoproteins and chylomicrons from the blood ) . ( 3 )Increased lipoprotein lipid synthesis is stimulated by a decrease in serum albumin and a decrease in oncotic pressure . ( 3 )LipiduriFat -free ( oval fat bodies) are often found in the urine sediment . The fat sources come from the filtrate glomerular basal membrane lipoproteins through the permeable . ( 3 )edemaUnderfil theory explains that hypoalbuminemia is a major contributing factor to the occurrence of edema in the SN . Hypoalbuminemia causes a decrease in plasma oncotic pressure so that fluid shifts from the intravascular to the network interstisium and edema . By kerana , the kidneys compensate by increasing sodium and water retention . This compensation mechanism will improve intravascular volume but will also exacerbate the occurrence of edema hypoalbuminemia so heavier. ( 1,2 , )Overfill theory explains that sodium retention as a primary renal defect . Retention of sodium by the kidneys, causing increased extracellular fluid , causing edema . Decline in glomerular filtration rate due to damage to the kidneys will increase sodium retention and edema . The second mechanism was found in SN patients . ( 1.2 )Some explanation is trying to combine these two theories , for example, stated that edema formation is a dynamic process . Found that plasma volume decreased significantly during the formation of edema and increased during the phase of diuresis . ( 1.2 )hypercoagulabilityThis situation is caused by the loss of antithrombin ( AT ) III , protein S , C and plasminogen activating factor in the urine and increased factor V , VII , VIII , X , platelets , fibrinogen , increased platelet aggregation , changes in endothelial cell function and reduced zimogen factor ( factor IX , XI ) . ( 3 )Susceptibility to infectionDecreased levels of immunoglobulin Ig G and Ig A due to loss through the kidneys , decreased synthesis and increased catabolism causes increased susceptibility to encapsulated bacterial infections such as Streptococcus pneumonia , Klebsiella , Haemophilus . At SN also disruption of T cell -mediated immunity Frequent bronkopneumoni and peritonitis . ( 3 )Clinical manifestations ( 1,2,3,4 )Protenuria : > 3.0 gr/24 hours . Changes on the basis of the glomerular membrane causes an increase in glomerular permebilitas to plasma proteins are albumin .Hypoalbuminemia : serum albumin 3.5 g / 1.73 m2 body surface area / day ) , hipoalbuminemi ( < 3 g / dl ) , edema , hiperlipidemi , lipiduri and hypercoagulability . Additional examinations such as venography is necessary to establish the diagnosis of venous thrombosis which can occur due to hypercoagulability . In the SN to determine the type of primary renal histopathologic abnormalities that determine prognosis and response to therapy , renal biopsy is necessary .Management ( 1,2,3 )SN treatment consists of specific treatment directed against basic diseases and non - specific treatment to reduce protenuria , control edema and treat complications . Secondary etiology of nephrotic syndrome should be sought and given therapy , da drugs are the cause removed .a) . diureticsAnsa Henle diuretics ( loop diuretics ) such as furosemide ( initial dose of 20-40 mg / day ) or a thiazide class with or without combination with a potassium -sparing diuretics ( spironolactone ) is used to treat edema and hypertension . Weight loss should not exceed 0.5 kg / day .b ) . Diet .Diet for patients SN is 35 cal / kg . / Day , consisting mostly of carbohydrates . Low-salt diet ( 2-3 g / day ) , low-fat should be given . Research has shown that in patients with certain kidney disease , a low intake of protein is safe , can reduce proteinuria and slow the loss of kidney function , possibly by lowering the pressure intraglomerulus . The degree of protein restriction will be recommended in patients who lack the protein due to nephrotic syndrome have not been established . Restriction of intake of 0.8-1.0 g protein / kg / day can reduce proteinuria . Additional vitamin D can be given if the patient is deficient in vitamins .c ) Anticoagulant TherapyWhen diagnosed with the events of thromboembolism , anticoagulant therapy with heparin should be initiated . The number of heparin are required to achieve a partial thromboplastin time ( PTT ) therapeutic may increase due to decrease in number of antithrombin III . After intravenous heparin therapy , oral anticoagulation with warfarin was continued until the nephrotic syndrome can be overcome .d ) Drug TherapySpecific therapy for nephrotic syndrome are the corticosteroids prednisone of 1 - 1.5 mg / kg / day single dose of the morning during 4-6 weeks . Then reduced 5 mg / week to achieve maintenance dose ( 5-10 mg ) was then given 5 mg within a day and stopped in 1-2 weeks . If at the time of tapering off , the state of the patient deteriorated again ( arising edema , protenuri ) , given back full dose for 4 weeks and then tapering off again . Corticosteroid drugs of choice to deal with nephrotic syndrome ( prednisone , methyl prednisone ), especially on minimal glomerular lesions ( MGL ) , focal segmental glomerulosclerosis ( FSG ) and systemic lupus glomerulonephritis . Nonsteroidal anti-inflammatory drugs ( NSAIDs ) have been used in patients with membranous nephropathy and focal glomerulosclerosis to reduce the synthesis of prostaglandins that cause dilation . It causes renal vasoconstriction , reduction intraglomerulus pressure , and in many cases up to 75 % decrease in proteinuria .Sitostatica by administering prednisone is given when there is no response , relapse who repeatedly or the possible side effects of corticosteroids . Cyclophosphamide can be given 1.5 mg / kg / day. Lipid-lowering drugs known as statins such as simvastatin , pravastatin and lovastatin can lower LDL cholesterol , triglycerides and increase HDL cholesterol .Anti proteinurik eg ACE inhibitor ( captopril 3 * 12.5 mg ) , calcium antagonists ( Herbeser 180 mg ) or beta blockers . Angiotensin converting enzyme inhibitors ( angiotensin converting enzyme inhibitors ) and angiotensin II receptor antagonists can reduce blood pressure and the combination has an additive effect in reducing proteinuria .complicationHyperlipidemia is a condition that often accompanies SN . Cholesterol levels generally increased , while triglyceride levels vary from normal to slightly high . Increased cholesterol levels due to increased LDL (low density lipoprotein ) is a type of primary lipoprotein cholesterol transporter . High levels of LDL in the SN due to increased synthesis of liver without liver catabolism disorders . SN mechanism in hyperlipidemia associated with lipid and lipoprotein synthesis increased liver and decreased catabolism . ( 1.2 )Lipiduria often found in SN and is characterized by the accumulation of lipids in the cell debris and cast as oval fat bodies ( oval fat bodies) and fatty casts. Lipiduria more associated with protenuria than with hyperlipidemia . ( 3 )Thromboembolic complications often found in SN due to increased intravascular coagulation . In the SN due GNMP likelihood of renal vein thrombosis is quite high . Pulmonary embolism and deep venous thrombosis ( deep vein thrombosis ) often found in SN . ( 2.3 )Kerana infection by humoral immunity defects , cellular , and disorders of complement system . By the bacteria that are not encapsulated as Haemophilus influenzae and Streptococcus pneumoniae may cause an infection . Decrease in IgG , IgA and gamma globulin are often found in patients kerana SN by decreased synthesis or increased catabolism and increase the amount of waste through urine . ( 2 )Acute renal failure caused by hypovolemia . By kerana fluid accumulates in the tissues of the body , so less fluid in the blood circulation . Decreased blood flow to the kidneys causing kidney can not function properly and the incidence of acute tubular necrosis . ( 1,2,3 )Prognosis ( 5,6 )Prognosis depends on the movement of nephrotic syndrome . In the case of children , the prognosis is very good kerana minimal change disease ( MCD ) gave a very good response to steroid therapy and does not cause kidney failure (chronic renal failure) . But for other causes such as focal segmental glomerulosclerosis ( FSG ) often causes end stage renal disease occurs ( ESRD ) . Factors - other factors that aggravate again nephrotic syndrome is protenuria levels , control blood pressure and renal function .
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