Nephrotic Syndrome Flashcards by CM A

Nephrotic-range proteinuria is defined as

> 3.5g/24hr or UPCr >2g/g

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Triad of clinical findings associated with nephrotic syndrome arise from

Large urinary losses of protein

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Triad of clinical findings associated with nephrotic syndrome

1) Hypoalbuminemia (less than 2.5g/dL) 2) Edema 3) Hyperlipidemia (cholesterol >200mg/dL)

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Without treatment, nephrotic syndrome is associated with a high risk of death, most commonly from

Infections

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___% of children with nephrotic syndrome respond to corticosteroid therapy

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MC glomerular lesion associated with idiopathic nephrotic syndrome

Minimal change disease

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Underlying abnormality in nephrotic syndrome

Increased permeability of the glomerular capillary wall

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A highly differentiated epithelial cell located on the outside of the glomerular capillary loop

Podocyte

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Functions of the podocyte

1) Structural support of the capillary loop 2) Major component of the glomerular filtration barrier to proteins 3) Involved in the synthesis and repair of the GBM

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MC presenting symptom of children with nephrotic syndrome

Edema

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Postulates that nephrotic syndrome is associated with primary sodium retention, with subsequent volume expansion and leakage of excess fluid into the interstitium

Overfill hypothesis

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Postulates that sodium and water retention therefore occur as a consequence of intravascular volume depletion.

Underfill hypothesis

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Goal of nephrotic syndrome therapy

Gradual reduction of edema with 1) Judicious use of diuretics 2) Sodium restriction 3) Cautious use of albumin infusions

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Why are children with nephrotic syndrome especially susceptible to infections

1) Hypoglobulinemia as a result of the urinary losses of immunoglobulin (Ig) G 2) Impaired opsonization from urinary loss of complement factors (predominantly C3 and C5)

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Children with nephrotic syndrome are at significantly increased risk for infection with what kind of bacteria

Encapsulated, particularly pneumococcal

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Most frequent cause of peritonitis

Pneumococcus

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Peritoneal leukocyte counts of ____ are highly suggestive of SBP

> 250

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Nephrotic syndrome us a hypercoagulable state resulting from

1) Vascular stasis from hemoconcentration and intravascular depletion 2) Increased platelet number and aggregability 3) Changes in coagulation factor levels (increased production of fibrinogen, urinary losses of antithrombotic factors ATIII and protein S)

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Clinical risk of hypercoagulable state in children is

2-5%, low compared to adults

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Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Most commonly presents as nephrotic syndrome

MCNS

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Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Least hematuria

MCNS

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Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Asymptomatic proteinuria

MPGN

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Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Most hypertensive

MPGN

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Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Does not progress to renal failure

MCNS

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Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Associated with HIV and heroin use

FSGS

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Associated with sickle cell disease

FSGS

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Associated with RVT

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Associated with partial lipodystrophy

MPGN II

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Associated with reflux nephropathy

FSGS

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Low complement levels

MPGN I

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Steroid resistant

MN, MPGN

Approx ___% of children with nephrotic syndrome have idiopathic nephrotic syndrome

Approx 85% of total cases of nephrotic syndrome in children is ___ in pathology

MCNS

___% of children with MCD respond to corticosteroid therapy

FSGS: LM findings

Mesangial cell proliferation and segmental scarring

FSGS: IF findings

Positive for IgM and C3

FSGS: EM findings

Segmental scarring of the glomerular tuft with obliteration of the glomerular capillary lumen

Only ___% of patients with FSGS respond to prednisone

Idiopathic nephrotic syndrome is more common in what gender

Males

Idiopathic nephrotic syndrome appears between the ages of

2 and 6 years

MC cause of idiopathic nephrotic syndrome in children less than 6 y/o

MCNS

MC cause of idiopathic nephrotic syndrome in adolescents

FSGS

Nephrotic syndrome can initially be misdiagnosed as ___ because of the periorbital swelling that decreases throughout the day

Allergic disorder

Important features of minimal change idiopathic nephrotic syndrome

Absence of hypertension and gross hematuria

Diagnosis of nephrotic syndrome is confirmed by

1) Urinalysis with first morning urine protein:creatinine ratio 2) Serum electrolytes, BUN, Crea, albumin, cholesterol

Evaluation to rule out secondary forms of nephrotic syndrome (children >/10 yr)

C3, ANA, dsDNA, Hepatitis B and C, HIV

Evaluation for children >/12 yr with idiopathic nephrotic syndrome, who are less likely to have MCNS

Kidney biopsy

Nephrotic syndrome: UA protein

3+ or 4+

Microscopic hematuria is present in ___% of children with nephrotic syndrome

20%

Nephrotic syndrome: Spot urine protein:crea ratio

Nephrotic syndrome: Serum albumin level

Less than 2.5g/dL

Nephrotic syndrome: Serum complement

Normal

Idiopathic nephrotic syndrome is less likely to be MCNS if it occurs at what ages

Less than 1 year or >12 years old

Mainstay of therapy of MCNS

Corticosteroids

Response to steroids is defined as

Attainment of remission within the initial 4 weeks of corticosteroid therapy

MCNS: Remission consists of

UPCr <0.2 or <1+ protein on dipstick for 3 consecutive days

MCNS: Vast majority of children who respond to prednisone therapy do so within

First 5 weeks of treatment

Nephrotic syndrome: Indication for admission

Severe symptomatic edema (Large pleural eff, ascites, or severe genital edema)

Sodium restriction

<1500mg/day

Management of hyponatremia in children with nephrotic syndrome

Fluid restriction

Management of swollen scrotum in nephrotic syndrome

Elevated with pillows to enhance fluid removal by gravity

Indications for administration of IV albumin in children with nephrotic syndrome

Severe generalized edema with evidence of intravascular volume depletion (hemoconcentration, hypotension, tachycardia, etc)

Nephrotic syndrome: Albumin is administered at what dose

0.5-1.0g/kg) followed by furosemide (1-2mkdose IV)

Potential complications of parenteral albumin therapy

1) Volume overload 2) Hypertension 3) Heart failure 4) Pulmonary edema

Nephrotic syndrome: Management of dyslipidemia

1) Limit dietary fat intake to <30% of calories 2) Limit saturated fat intake to <10% of calories 3) Limit dietary cholesterol intake to <300mg/day

Nephrotic syndrome: Empiric antibiotic therapy should be broad enough to cover

1) Pneumococcus 2) G- bacteria (3rd gen cephalosporin is a common choice)

Nephrotic syndrome: Management of thromboembolism

1) Imaging studies to confirm presence of a clot 2) Studies to delineate a specific underlying hypercoagulable state 3) Heparin, LMWH, or Warfarin

Nephrotic syndrome: Relapse is defined as

1) UPCr >2 or 2) >/3+ protein on urine dipstick testing for 3 consecutive days

Nephrotic syndrome: Relapses are common, especially in younger children and are often triggered by

URTI or GI infections

Nephrotic syndrome: How are relapses treated

Usually in a similar manner to the initial episode except that daily prednisone courses are shortened

Nephrotic syndrome: Steroid resistance is defined as

Failure to achieve remission after 8 weeks of corticosteroid therapy

SRNS further eval includes

1) Kidney biopsy 2) KFT 3) Quantitation of urine protein excretion on top of urine dipstick testing

SRNS is usually caused by

FSGS in 80% of cases, MCNS, or MPGN

SRNS, specifically FSGS, is associated with a ___% risk for end-stage kidney disease within 5 years of diagnosis if patients do not achieve a partial or complete remission

Reduces the number of relapses in children with frequently relapsing and steroid-dependent nephrotic syndrome

Cyclophosphamide therapy

Potential side effects of Cyclophosphamide therapy

1) Neutropenia 2) Disseminated varicella 3) Hemorrhagic cystitis 4) Alopecia 5) Sterility 6) Increased risk of future malignancy

During cyclophosphamide therapy, what blood parameter should be monitored weekly?

WBC count, if below 5,000/mm3, withhold drug

Cumulative threshold of Cyclophosphamide above which oligospermia or azoospermia occurs in boys is

>250mg/kg

Recommended as initial therapy for children with steroid-resistant nephrotic syndrome

Calcineurin inhibitors (cyclosporine or tacrolimus)

Side effects of calcineurin inhibitors

1) Hypertension 2) Nephrotoxicity 3) Hirsutism 4) Gingival hyperplasia

An antihelmintic agent with immunomodulating effects that has been shown to reduce the risk of relapse in comparison to prednisone

Levamisole

Rituximab is a chimeric monoclonal antibody against ___

CD20

___ and ___ may be helpful as adjunct therapy to reduce proteinuria in steroid-resistant patients

Angiotensin-converting enzyme inhibitors and angiotensin II receptor blockers

Live vaccination should be deferred until prednisone dose is

Either below 1mkday or 2mkday on alternate days

T/F Live virus vaccines are contraindicated in children receiving corticosteroid- sparing agents such as cyclophosphamide or cyclosporine

Guideline for children on immunosuppresives following close contact with varicella infection

VZ immune globulin

T/F Most children with steroid-responsive nephrotic syndrome have repeated relapses

T/F Relapses in children with steroid-responsive NS decrease in frequency as the child grows older

Children with nephrotic syndrome with these characteristics are likely to follow an infrequently relapsing course

Children who respond rapidly to steroids and those who have no relapses during the first 6 mo after diagnosis

Infectious agents associated with nephrotic syndrome

1) Malaria 2) Schistosomiasis 3) Hepatitis B 4) Hepatitis C 5) Filaria 6) Leprosy 7) HIV

Proposed mechanism of nephrotic syndrome in lymphoma

Lymphoma produces a lymphokine that increases permeability of the glomerular capillary wall

Drugs associated with Membranous glomerulopathy

Penicillamine, captopril, gold, nonsteroidal antiinflammatory drugs, mercury compounds

Drugs associated with MCNS

Probenecid, ethosuximide, methimazole, lithium

Drugs associated with Proliferative GN

Procainamide, chlorpropamide, phenytoin, trimethadione, paramethadione

Congenital nephrotic syndrome is defined as

Nephrotic syndrome manifesting at birth or within the 1st 3 mo of life

Caused by mutations in the WT1 gene, which results in abnormal podocyte function

Denys-Drash syndrome

Manifestations of Denys-Drash syndrome

1) Early-onset nephrotic syndrome 2) Progressive renal insufficiency 3) Ambiguous genitalia 4) Wilms tumor

Syndrome caused by mutation in LAMB2 gene, leading to abnormalities in β2-laminin, a critical component of glomerular and ocular basement membranes.

Pierson syndrome

Congenital nephrotic syndrome presenting with bilateral microcoria (fixed narrowing of the pupil)

Pierson syndrome

T/F Secondary congenital nephrotic syndrome can resolve with treatment of the underlying cause

Definitive treatment of congenital nephrotic syndrome

Renal transplantation

Nephrotic Syndrome Flashcards

(101 cards)