questions 8

Primary Nephrotic Syndrome

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Topic updated on 02/23/17 11:15am

Snapshot
  • A 3-year-old boy presents with puffy eyes to the ER. His physician had treated him for allergies but the symptoms did not improve. On PE the patient is noted to have lower extremity edema. He has a serum albumin of 2.5 g/dL, and 4+ proteinuria on urinalysis. (Minimal Change Disease)
Introduction
  • Primary nephrotic syndrome refers to disease limited to kidney
  • Secondary nephrotic syndrome is a multisystem disease
Presentation
  • Generalized edema (anasarca)
    • caused by loss of albumin / decreased oncotic pressure
Evaluation
  • Labs show
    • hypoalbuminemia (serum albumin of <3.0 g/dL)
    • proteinuria > 3.5 g/day
    • hyperlipidemia with lipiduria
      • low albumin stimulates liver to produce cholesterol
  • Diagnosis made by renal biopsy
Differential

 
Nephrotic Syndrome
"o for protein"
Nephritic Syndrome
Chronic Disease
Proteinuria
+ + +
+ / -
+ / -
Hematuria
+ / _
+ + +
+ / -
Cells
+ / _
+ RBCs / + WBCs
+ / -
Casts
Fatty Casts
RBC and granular casts
Waxy/pigmented/granular casts
Lipids
Free fat droplets
Oval fat bodies
   

Generalized treatment
  • Protein restriction
  • Salt restriction
  • Diuretic therapy for edema
  • HMG-CoA reductase inhibitor for hyperlipidema
Minimal Change Disease (MCD)
  • Most common cause of nephrotic syndrome in young children 
  • Results from loss of polyanions (neg charge) in the GBM
  • Associated with allergic diseases
    • treating allergy often improves nephrotic syndrome
    • also the most common manifestation of nephrotic syndrome in Hodgkin's lymphoma
    • electron microscopy findings show effacement of podocytes 
  • Treatment  
    • prednisone
      • disease very responsive with excellent prognosis
  • Complications
    • at risk for encapsulated organism infection especially strep. pneumonia
Focal Segmental Glomerulonephritis
  • Clinically similar to MCD, but occurs in adults with refractory HTN
  • Usually idiopathic
  • Can be associated with:
    • heroin
    • HIV   
    • DM
    • Sickle cell anemia
    • Obesity
    • Most common cause of proteinuria in adults
  • Typical presentation of idiopathic type is young, black, hypertensive male 
  • Treatment
    • prednisone
    • cyclophosphamide
  • Prognosis very poor due to refractory disease
Membraneous Glomerulonephritis
  • Most common cause of nephrotic syndrome in adults
    • unique finding of anti-phospholipase A2 antibodies
  • Causes include
    • infection (HBV, HCV, syphilis, malaria) 
    • drugs (gold, penicillamine)
    • occult malignancy
    • SLE (found in 10% of patients)
  • Treatment
    • prednisone
    • +/- cyclophosphamide
  • Slowly progressive disorder with decreasing response to steroids
Membranoproliferative Glomerulonephritis
  • Two forms
    • Type I is slowly proliferative 
    • Type II more aggressive
      • have autoantibody against C3
      • leads to decreased serum C3 levels
  • Treatment
    • prednisone
    • +/- plasmapheresis or interferon-alpha
  • Prognosis very poor


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Qbank (4 Questions)

TAG
(M2.RL.7) A seven-year-old girl presents to general pediatrics condition with swelling as shown in Figure A. She has never had symptoms like this before. She has no significant past medical history. Her vital signs are stable. Her physical examination is notable for 2+ pitting edema of the bilateral lower extremities. The remainder of her physical examination is within normal limits. She has no costovertebral angle tenderness. A dipstick urinalysis is significant for 3+ proteinuria. On renal biopsy and on microscopic urinalysis, what would one likely expect to find in this patient? Topic Review Topic
FIGURES: A          

1. Apple-green birefringence and no casts
2. Podocyte effacement and fatty casts
3. Spike and dome appearance with subepithelial deposits and red blood cell casts
4. Positive Congo red stain and waxy casts
5. Kimmelstiel-Wilson lesions and waxy casts

PREFERRED RESPONSE ▶
TAG
(M2.RL.50) A 37-year-old male with HIV presents to infectious disease clinic for follow-up. He contracted HIV 15 years ago and has been compliant with his anti-viral medications. His viral load is undetectable and CD4 count is within normal limits. He has no other comorbidities and no complaints at today's visit. Vital signs are BP 150/95 mmHg, HR 75 bpm, RR 12, SpO2 99% on room air and T 37 C. Physical examination is unremarkable. A urinalysis reveals proteinuria. Given this patient's illness, what is the most likely glomerulopathy causing this finding? Topic Review Topic

1. A highly corticosteroid responsive disease that is also the most common cause of nephrotic syndrome in children
2. A cause of nephropathy that is likely to be found in patients with multiple myeloma and rheumatoid arthritis
3. A glomerulopathy that is secondary to nonenzymatic glycosylation of the glomerular basement membrane resulting in Kimmelstiel-Wilson lesions
4. A glomerulopathy that is also found in patients with Goodpasture syndrome, Wegener's granulomatosis, and microscopic polyangiitis
5. A disease that is the most common cause of nephrotic syndrome in adults and African American patients

PREFERRED RESPONSE ▶
TAG
(M2.RL.64) A 3-year-old girl is brought to her pediatrician by her mother, who is concerned that her daughter has developed puffy eyes and swelling in her feet. Physical examination confirms bilateral lower extermity edema to the mid-calf. The patient's medical history is significant for acute hepatitis B infection at the age of 9 months that was acquired through perinatal transmission. Laboratory studies are obtained and show a serum albumin level of 2.2 g/dL and 4+ protein and no blood on urinalysis. Hepatitis B serologies are also obtained and show positive HBsAg and positive anti-HBc IgM antibodies. Which of the following is the most likely diagnosis in this patient? Topic Review Topic

1. Minimal change disease
2. Focal segmental glomerulosclerosis
3. Membranous glomerulonephritis
4. IgA nephropathy
5. Poststreptococcal glomerulonephritis

PREFERRED RESPONSE ▶
TAG
(M2.RL.168) A concerned mother brings her previously healthy 5 year child to the family care physician after 3 mornings of significant swelling around the child's eyes and feet. The mother is concerned since she feels that the child has not been urinating at his normal frequency, and that his urine has been "thick and frothy." The physician requests a 24hr urine protein collection which reveals nephrotic range proteinuria. What is the next step in the management of this patient? Topic Review Topic

1. Echocardiogram
2. Abdominal X-Ray
3. Empiric Steroid Therapy
4. Renal Biopsy
5. Renal Ultrasound

PREFERRED RESPONSE ▶
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