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Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

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Page 1: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Approach to Nephrotic & Nephritic Syndromes

Academic Half-DayAug 2, 2012

Page 2: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Nephrology Olympics!

Page 3: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Name two NBA athletes with

Nephrotic Syndrome

Page 4: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Proteinuria• Some protein in urine is normal (< 4mg/m2/hr or < 100

mg/m2/24h)

• Non-pathologic

• Postural (orthostatic)

• Febrile

• Exercise-induced

• Pathologic

• Tubular

• Inherited (cystinosis, Wilson ds, Lowe syndrome)

• Acquired (antibiotic, AIN, ATN, heavy metal poisoning)

• Glomerular

Page 5: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Hematuria• Gross

• Trauma - perform imaging and cystoscopy

• Pain - rule out renal stones, mass, UTI

• Painless - consider stones, mass, HSP, familial (thin BM), hematologic, other glomerular disease, systemic disease

• Microscopic

• Symptomatic - symptoms direct investigation

• Isolated hematuria - rarely have significant renal disease (benign familial hematuria or hypercalciuria)

• Asymptomatic hematuria and proteinuria - more worrisome, requires thorough evaluation by nephrology.

Page 6: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Edema• Differential diagnosis

• Loss of oncotic pressure

• decreased protein synthesis

• increased protein loss

• Increased capillary permeability

• viral/bacterial

• immune mediated

• mechanical/thermal trauma

• drugs

• Increased hydrostatic pressure

• congestive heart failure

• lymphatic blockage

Page 7: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

What is the term for extreme,

generalized edema?ANASARCA

Page 8: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Golmerular Disease

•Presents in one of 4 ways:

•Acute Nephritic Syndrome

•Nephrotic Syndrome

•Rapidly Progressive Glomeruloneprhitis

•Asymptomatic

Page 9: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Nephrotic Syndrome

•Nephrotic range proteinuria

• 3+ or 4+

• > 250 mg/mmol creatinine

• > 40 mg/m^2/hr

•Hypoalbuminemia

•Hyperlipidemia

•Hypercoagulable

Page 10: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Pathophysiology

Glomerular Filtration Barrier

Page 11: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

What is this fictional doc’s specialty?

NEPHROLOGY AND INFECTIOUS DISEASE!

Page 12: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Etiology

•Primary or idiopathic

• Minimal change disease

• FSGS

• MPGS

• Membranous nephropathy

Page 13: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Minimal Change Disease

85% of cases

Page 14: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

FSGS

10-15% of cases

Page 15: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Membranous Nephropathy

4% of cases

Page 16: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

In what event did this athlete win a bronze medal?

100m freestyle

Page 17: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Etiology• Secondary causes

• Genetic

• Metabolic

• Infections

• Drugs

• Immunologic/Allergic

• Malignancy

• Glomerular hyperfiltration

Page 18: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Idiopathic Nephrotic Syndrome

•90% of childhood nephrotic syndrome

• 85% of all cases due to MCD

•Consider diagnosis other than MCD in:

• Children < 1 year and > 10 years

• Family history nephrotic syndrome

• Extra-renal findings

• More than minimal hematuria

Page 19: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Idiopathic Nephrotic Syndrome

• Clinical manifestations:

• Most common between age 2 and 6.

• Often follows minor infection.

• Mild edema progressing to generalized.

• Ascites

• Pleural effusions

• Genital edema

• Anorexia, abdo pain, diarrhea, irritability common.

Page 20: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

What food item is known scientifically as Phaseolus

vulgaris?

Page 21: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Diagnosis• Laboratory findings

• Low serum albumin

• High cholesterol, triglycerides, lipoproteins

• Low serum sodium

• Nephrotic range proteinuria

• Indications for biopsy:

• Age > 10, gross hematuria, hypertension

• Renal insufficiency

• Low C3 complement value

• Persistent proteinuria following 4 week course of steroids

Page 22: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Treatment•Prednisone drug of choice.

•Dose: 60 mg/m^2 per day divided TID

•Continue daily for 4 weeks, then 40mg/m^2 as single dose on alternate days for 4 weeks.

•90% of MCD is steroid responsive

•Of those, 60% relapse

•Frequent relapsers can be treated with cyclophosphamide

Page 23: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Steroid-resistance

•Up to 10% of all children with NS

•Poor prognosis

•Eventually leads to dialysis and renal transplant

Page 24: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Complications of Nephrotic Syndrome

• Thromboembolic events

• Loss of antithrombin III and protein S

• Increase in fibrinogen concentration

• Infection

• Loss of factor B, low IgG, impaired Tcell function

• Most common infection is peritonitis

• Most common organisms are Strep pneumo or gram-negatives

• Steroid side effects

Page 25: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Where did this duo rank in badminton at the 2012

Olympics?DISQUALIFIED for CHEATING!

Page 26: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Nephritic Syndrome

•Results from inflammation within glomerulus

•Characterized by

• Hematuria, RBC casts, dysmorphic RBCs

• Mild to moderate proteinuria

• Azotemia, oliguria

• Hypertension

Page 27: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

EtiologyLow complement

Normal complement

PrimaryPostinfectious GN

Membranoproliferative GN

IgA nephropathyAnti-GBM disease

Secondary

SLEEndocarditis

AbscessCryoglobulinemia

Polyarteritis nodosa

Wegener’sHSP

Goodpasture’s

Page 28: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Rapidly Progressive Glomerulonephritis• Subset of nephritic syndrome

• Medical emergency

• Characterized by renal failure in days or weeks (months)

• Classfication:

• Type I - anti-GBM (idiopathic, Goodpasture’s)

• Type II - immune complex (PIAGN, SLE, HSP)

• Type III - pauci-immune (idiopathic, Wegener’s, microscopic polyangitis, drugs)

• Treatment

• Induction of remission (pulse steroids, cyclophosphamide 3-6 months)

• Maintenance therapy (azathioprine)

• Newer agents - Rituximab, MMF

Page 29: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Crescent

BAD NEWS.

Page 30: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

IgA Nephropathy• Most common cause of GN

• Idiopathic or secondary (HSP, rheumatic ds, HIV, Celiac ds, chronic liver ds)

• Variable presentation:

• Asymptomatic hematuria and mild proteinuria

• Recurrent episodes of gross hematuria

• Nephrotic range proteinuria or RPGN

• Hematuria coincident with URTI

• Treatment depends on prognostic indicators

• Watch & wait vs. immunosuppressive agents

Page 31: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

Poststreptococus glomerulonephritis

• Prototypic acute nephritic syndrome

• Occurs 2-3 weeks after pharyngitis or skin infection with GAS (nephritogenic strain)

• Typical history plus low C3, normal C4, and high ASOT, positive anti-DNase B

• Self-limited disease - three phases

• latent phase

• acute phase

• recovery phase

• Supportive management of hypertension and edema

• Indications for biopsy include normal complement level, failure to document strep infection, GFR < 30ml/min/1.73 m2

Page 32: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

How much does the average adult

kidney weigh?

120-140 grams!

Page 33: Approach to Nephrotic & Nephritic Syndromes Academic Half-Day Aug 2, 2012

References• Gordillio, R. and Spitzer, A. The Nephrotic Syndrome. Pediatr Rev, 2009;30: 94-105.

• Beck, L. and Salant, D. Glomerular and Interstitial Diseases. Prim Care Clin Office Pract 2008;35:265-296.

• Bergstein, J. A Practical Approach to Proteinuria. Pediatr Nephrol 1999;13:697-700.

• Massengill, S. Hematuria. Pedatr Rev 2008;29:342-348.

• Pais, P. and Avner, E. Nephrotic Syndrome. Nelson’s Textbook of Pediatrics, Ch 52:1801-1806.

• Eison, T., Ault, B., and Jones, D. Post-streptococcal cute glomerulonephritis in children: clinical features and pathogenesis. Pedatr Nephrol 2011;16:165-180.