NEPHROTIC SYNDROME2

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    NEPHROTIC SYNDROME

    BY,

    PUTRI NOOR ZULAIKHA

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    DEFINITION

    Triad of proteinuria

    Spot urine protein:creatinine ratio

    Hypoalbuminaemia

    Oedema

    Severe hyperlipidaemia

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    CAUSES

    CAUSES OF NEPHROTIC SYNDROME

    ALL GROMERULONEPHRITIESAND MINIMAL CHANGE OFGLOMERULUS LESIONS

    SYSTEMIC VASCULITIDES, MAINLYSYSTEMIC LUPUS ERYTHEMATOSUS

    DIABETIC GLOMERULOSCLEROSIS

    AMYLOIDOSIS

    DRUGS

    ALLERGIES

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    CLASSIFICATION

    Primary causes of nephrotic syndrome include, inapproximate order of frequency:

    Minimal-change nephropathy

    Focal glomerulosclerosis

    Membranous nephropathy

    Hereditary nephropathies

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    Secondary causes include, again in order ofapproximate frequency:

    Diabetes mellitus

    Systemic Lupus erythematosus

    Amyloidosis

    Viral infections (eg, hepatitis B, hepatitis C,human immunodeficiency virus [HIV] )

    Preeclampsia

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    CLINICAL

    History The first sign of nephrotic syndrome in children is

    usually swelling of the face; this is followed by swellingof the entire body.

    Adults can present with dependent edema. Foamy urine may be a presenting feature. A thrombotic complication, such as deep vein

    thrombosis of the calf veins or even a pulmonaryembolus, may be the first clue indicating nephrotic

    syndrome. Additional historical features that appear can be

    related to the cause of nephrotic syndrome. Thus, therecent start of a nonsteroidal anti-inflammatory drug(NSAID) or a 10-year history of diabetes may be veryrelevant.

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    Physical

    Edema is the predominant feature of nephrotic

    syndrome and initially develops around the eyes andlegs. With time, the edema becomes generalized andmay be associated with an increase in weight, thedevelopment of ascites, or pleural effusions.

    Hematuria and hypertension manifest in a minorityof patients.

    Additional features on exam will vary according tocause and as a result of whether or not renal function

    impairment exists. Thus, in the case of longstandingdiabetes, there may be diabetic retinopathy, whichcorrelates closely with diabetic nephropathy. If thekidney function is reduced, there may be

    hypertension and/or anemia.

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    LAB STUDIES..

    Urinalysis

    Time collection of urinary protein Serum tests for kidney function

    urine protein electrophoresis.

    The serum albumin

    Ultrasonographic

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    Granular cast

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    Waxy cast

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    Hyaline cast/ fatty cast

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    RBC cast

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    Acute management of childhood

    nephrotic syndrome has generalized edema severe enough to cause

    respiratory distress

    tense scrotal or labial edema, if he or she hascomplications (eg, bacterial sepsis, peritonitis,pneumonia, thromboembolism)

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    Diuretics will be needed; furosemide (1

    mg/kg/d) and spironolactone (2 mg/kg/d) willhelp when fluid retention is severe, provided nosigns of renal failure or volume contraction areevident. Achieving a satisfactory diuresis isdifficult when the patient's serum albumin levelis less than 1.5 g/dL. Albumin at 1 g/kg may begiven, followed by intravenous furosemide.Complications may occur, including pulmonaryedema. Some evidence suggests that albumin

    may delay the response to steroids and may eveninduce more frequent relapses, probably bycausing severe glomerular epithelial damage.Fluid removal and weight loss remain transient

    unless proteinuria remits

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    With regard to infection, oral penicillin can beprescribed as prophylaxis for children with grossedema. Abdominal paracentesis should beperformed if the patient develops signs ofperitonitis, and any bacterial infection should betreated promptly. A nonimmune patient with

    varicella should receive zoster immunoglobulintherapy if exposed to chickenpox, andacyclovir should be given if the patient developschickenpox

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    Acute management of adultnephrotic syndrome Diuretics will be needed; furosemide,

    spironolactone, and even metolazone may beused. Volume depletion may occur with diureticuse, which should be monitored by assessmentof symptoms, weight, pulse, and blood pressure

    Anticoagulation has been advocated by some for

    use in preventing thromboemboliccomplications, but its use in primaryprevention is of unproven value.

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    Hypolipidemic agents may be used, but if thenephrotic syndrome cannot be controlled, therewill be persistent hyperlipidemia

    In secondary nephrotic syndrome, such as that

    associated with diabetic nephropathy,angiotensin-converting enzyme (ACE) inhibitorsand/or angiotensin II receptor blockers are widelyused. These may reduce proteinuria by reducing

    the systemic blood pressure, by reducingintraglomerular pressure, and also by directaction on podocytes.

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    THANK YOU