Renal System-Proteinuria By Ryan

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Renal System-Proteinuria by Ryan

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Renal System-Proteinuria by Ryan

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how much and what kind of protein do you see in normal youngsters?

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Protein can be found in the urine of normal children
-4 mg/m2/hr in young children
-150 mg/24 hr in adolescents

Albumin is most common
-Remainder is Tamm-Horsfall protein from the distal tubule

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what is proteinuria? how can you subdivide it?

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Proteinuria is defined as >150 mg/day

Proteinuria is subdivided into:
-Pathologic
-Non-Pathologic (usually <1g/day)
-Postural (orthostatic)
-Fever (inflammatory response)
-Exercise (exercise can give you protein pee)

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Why do you repeat UA tests?

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-Make sure it is persistent and not transient
-Supine and upright to rule out orthostatic proteinuria

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ok, fine, so what is nephrotic syndrome

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Proteinuria >50mg/kg/day or >3.5g/day
Serum albumin < 3mg.dL (since you're peeing it out)
Edema
Hypercholesterolemia

-Cellular casts are absent
-May bee oval fat bodies & granular casts
-Infections: 50% of infections are due to encapsulated organisms (vaccinate!)
-Thrombotic disease
-Almost all have a hypercoagulable state and 20% have a clinically silent event
-Renal vein and saggital sinus are most common locations

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What's the most common type of nephrotic syndrome in childhood? What's the cause?

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Minimal change nephrotic syndrome.
-90% <10 yrs old
-almost always idiopathic
-Has been associated with Hodgkin’s lymphoma, NSAIDs, and systemic immune-mediated diseases
-Atopy occurs in 30-60% of kids

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Clinical and Lab findings of Minimal Change?

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Clinical:
-gravity-dependent edema (early morn eyelid swelling, edema)
-triggered by preceding infection

Lab:
-Protenuria is universal
-Oval fat bodies (maltese crosses) & waxy or hyaline casts are common
-Creatinine may be elevated
-Hyponatremia is common (dilutional)
-C3 is normal or high
-Treat empirically without a renal biopsy

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Treatment of Minimal Change

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Supportive and symptomatic care
-Manage edema by salt restriction
-Can restrict fluids, but not usually necessary
-Avoid diuretics
Most patients have a chronic relapsing course
-Triggered by URI
Give pneumococcal vaccine and avoid NSAIDs
Corticosteroids
-Prednisone 2mg/kg/day (max 60 mg)
-may be steroid resistant

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When do you do a renal biopsy?

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Renal biopsy indications:
-< 1 yr
-Gross hematuria
-Hypocomplementemia
-Renal failure (not due to dehydration)
-No remission in 8-10 weeks

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What is the most common GLOMERULAR cause of End Stage Renal Disease in childhood?

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Focal Segmental Glomerulosclerosis.
-it's idiopathic

Histology: Segment of the glomerulus collapses with mesangial sclerosis

-Most children present with nephrotic syndrome that does not respond to steroids

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if you see HTN and nephrotic syndrome in an adolescent, what is more likely to be, FSGN or Minimal Change?

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FSGN.

Most commonly affects those between 2-7 years old
-Higher incidence in boys
-Higher incidence in African-Americans

Some cases occur in families
-Recessive: chromosome 1
-Dominant: chromosome 11

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Diagnosis & Treatment of FSGN

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Need a biopsy to diagnose FSGS

Treatment is difficult
-Only 25% achieve remission with steroids
-Usually high dose IV steroids and cyclophosphamide are used for prolonged durations

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Characterized by immune deposits (IgG and C3) in the basement membrane or along the subepithelial area of the glomerular capillary wall

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Membranous nephropathy (idiopathic)

50% are due to a secondary cause in children
-SLE, HBV, congenital syphilis, malaria, medications, CLL, NHL, neuroblastoma, sickle cell

Most present with nephrotic syndrome

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Membranous nephropathy... needed for dx? tx?

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-Renal biopsy required for diagnosis
-Always look for secondary causes
-Treat severe disease or persistent nephrotic syndrome with steroids
-In children, its more an indolent disease with < 5% developing ESRD 5 yrs after diagnosis

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Hereditary glomerulopathies. Name 5.

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Alport Syndrome
Thin basement membrane disease
Denys-Drash Syndrome
Fabry Disease
Nail-Patella Syndrome

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What do you see with Denys-Drash Syndrome?

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-Very rare
-**Wilms tumor, XY gonadal dysgenesis with ambiguous genitalia & nephropathy
-Infantile onset of nephrotic syndrome
-Renal failure by age 3 yrs
-Consider bilateral nephrectomy to prevent Wilms tumor

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Fabry Disease. it's a deficiency is WHAT?!

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-**X-linked deficiency of alpha-galactosidase A
-In boys, causes structural and functional abnormalities in the kidney, heart and CNS
-Nephrotic range proteinuria is rare
-Progresses to ESRD in the 40s
-Enzyme replacement is now available

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Nail-Patella Syndrome. Which chromosome? Presentation?

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-***Autosomal dominant (Chromosome 9)
-Hypoplasia or absence of patellae
-Dystrophic nails
-Dysplasia of elbows, iliac horns
-Renal disease
-Microhematuria & mild proteinuria
-10% progress to ESRD

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Hypocomplementemia always seen in? frequently seen in?

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ALWAYS occurs in POSTINFECTIOUS and frequently in MEMBRANOPROLIFERATIVE GN
-Also seen in cryoglobulinemic GN and SLE flares (which can cause any type of GN)

Other causes include:
-endocarditis, shunt nephritis and atheroembolic renal disease

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Hypocomplementemia usually seen in? never seen in?

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The following USUALLY have normal complement (only occasionally low):
-RPGN, mesangial proliferative GN, Wegener’s & Goodpasture

NEVER occurs in:
-Minimal change, focal sclerosis, membranous nephropathy, diabetic nephropathy or amyloid nephropathy

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Nephritic sediment.. what do you see if you have nephritis? What is it usually seen in?

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Nephritis (= active; = casts of WBCs, RBCs & granules)

Urine sediment is USUALLY SEEN in:
-IgA nephropathy, early postinfectious GN, SLE, RPGN

Nephritic sediment DOES NOT exclude nephrosis

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Nephritic sediment is NEVER seen in

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-Minimal change, focal sclerosis, membranous nephropathy, diabetic nephropathy & amyloid

-THESE ARE THE SAME DISEASES IN WHICH HYPOCOMPLEMENTEMIA NEVER OCCURS!!!

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Urine Sediment in Renal Disease, Pre-renal failure:

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granular & hyaline casts, but most often these are normal

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Urine Sediment in Renal Disease, post-renal:

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BLOOD
-bc the stone is scratching all the way down
-WBC casts if due to papillary necrosis

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Urine Sediment in Renal Disease, Renal:

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ATN: large muddy brown granular casts

GNs:
-Nephritic: hematuria with RBC casts, sometimes pyuria with WBC casts
-Nephrotic: oval fat bodies, Maltese crosses

Allergic interstitial Nephritis: eosinophils, RBCs, WBCs, and WBC casts