Name the major (1) signs, (2) serum changes, and (3) urine changes associated with nephrotic syndrome.
(1) Edema
(2) Hypoalbuminemia and hyperlipidemia
(3) Proteinuria
Nephrotic syndromes are characterized by a proteinuria of ___ 3.5 g/day.
Nephrotic syndromes are characterized by a proteinuria of > 3.5 g/day.
__________ syndrome is characterized by the following:
(1) Hypoalbuminemia
(2) Hypogammaglobulinemia
(3) Hypercoagulable state
(4) Hyperlipidemia and hypercholesterolemia
Nephrotic syndrome is characterized by the following:
(1) Hypoalbuminemia
(2) Hypogammaglobulinemia
(3) Hypercoagulable state
(4) Hyperlipidemia and hypercholesterolemia
Nephrotic syndrome is characterized by the following:
(1) Hypo_________emia
(2) Hypo_________emia
(3) Hypercoagulable state
(4) Hyperlipidemia and hypercholesterolemia
Nephrotic syndrome is characterized by the following:
(1) Hypoalbuminemia
(2) Hypogammaglobulinemia
(3) Hypercoagulable state
(4) Hyperlipidemia and hypercholesterolemia
Nephrotic syndrome is characterized by the following:
(1) Hypoalbuminemia
(2) Hypogammaglobulinemia
(3) Hyper_________ state
(4) Hyper_________emia and hyper_________emia
Nephrotic syndrome is characterized by the following:
(1) Hypoalbuminemia
(2) Hypogammaglobulinemia
(3) Hypercoagulable state
(4) Hyperlipidemia and hypercholesterolemia
What causes the S/Sy associated with nephrotic syndrome?
(I.e. hypoalbuminemia, hypogammaglobulinemia, hypercoagulable state, hyperlipidemia, and hypercholesterolemia)
Proteinuria due to a variety of glomerular disorders
(> 3.5 mg/day)
The following diseases are six causes of _________ _________:
(1) Minimal change disease
(2) Focal segmental glomerulosclerosis
(3) Membranous nephropathy
(4) Membranoproliferative glomerulonephritis
(5) Diabetes mellitus
(6) Systemic amyloidosis
The following diseases are six causes of nephrotic syndrome:
(1) Minimal change disease
(2) Focal segmental glomerulosclerosis
(3) Membranous nephropathy
(4) Membranoproliferative glomerulonephritis
(5) Diabetes mellitus
(6) Systemic amyloidosis
Name six causes of nephrotic syndrome:
(1) _________ _________ disease
(2) Focal ___________ _____________
(3) Membranous nephropathy
(4) Membranoproliferative glomerulonephritis
(5) Diabetes mellitus
(6) Systemic amyloidosis
Name six causes of nephrotic syndrome:
(1) Minimal change disease
(2) Focal segmental glomerulosclerosis
(3) Membranous nephropathy
(4) Membranoproliferative glomerulonephritis
(5) Diabetes mellitus
(6) Systemic amyloidosis
Name six causes of nephrotic syndrome:
(1) Minimal change disease
(2) Focal segmental glomerulosclerosis
(3) ____________ _______pathy
(4) ____________ ____________
(5) Diabetes mellitus
(6) Systemic amyloidosis
Name six causes of nephrotic syndrome:
(1) Minimal change disease
(2) Focal segmental glomerulosclerosis
(3) Membranous nephropathy
(4) Membranoproliferative glomerulonephritis
(5) Diabetes mellitus
(6) Systemic amyloidosis
Name six causes of nephrotic syndrome:
(1) Minimal change disease
(2) Focal segmental glomerulosclerosis
(3) Membranous nephropathy
(4) Membranoproliferative glomerulonephritis
(5) _________ _________
(6) Systemic _________
Name six causes of nephrotic syndrome:
(1) Minimal change disease
(2) Focal segmental glomerulosclerosis
(3) Membranous nephropathy
(4) Membranoproliferative glomerulonephritis
(5) Diabetes mellitus
(6) Systemic amyloidosis
What is the most common cause of nephrotic syndrome in children?
Minimal change disease
Minimal change disease is the most common cause of nephrotic syndrome in children. It is typically ___________ and is associated with __________ disease as well.
Minimal change disease is the most common cause of nephrotic syndrome in children. It is typically idiopathic and is associated with Hodgkin’s disease as well.
How do glomeruli on H&E appear in minimal change disease?
Normal
In minimal change disease, lipids can be found in what part(s) of the kidney?
The proximal tubule cells
What is noticable on electron microscopy in cases of minimal change disease?
Effacement of foot processes
How do the glomeruli appear on immunoflorescence in patients with minimal change disease?
No change
(no immune complex deposits)
True/False.
Minimal change disease presents with both albuminuria and hypogammaglobulinemia.
False.
Minimal change disease presents with albuminuria but no hypogammaglobulinemia (selective proteinuria).
Minimal change disease shows what changes on imaging?
Lipids in proximal tubule cells (light microscopy);
podocyte process effacement (electron microscopy)
How is minimal change disease treated?
How does it typically respond to treatment?
Steroids;
excellent response
The renal damage seen in minimal change disease is mediated by what cell type?
T cells
What is the most common cause of nephrotic syndrome in Hispanics and African-Americans?
Focal segmental glomerulosclerosis
Focal segmental glomerulosclerosis is usually ___________ but may be asssociated with HIV, heroin use, and sickle cell disease.
Focal segmental glomerulosclerosis is usually idiopathic but may be asssociated with HIV, heroin use, and sickle cell disease.
Focal segmental glomerulosclerosis is usually idiopathic but may be asssociated with _____, ________ use, and _________ ______ disease.
Focal segmental glomerulosclerosis is usually idiopathic but may be asssociated with HIV, heroin use, and sickle cell disease.
How does focal segmental glomerulosclerosis appear on microscopic exam with H&E stain?
Focal (some glomeruli) and segmental (involving only part of the glomerulus) sclerosis
(shocking)
What is noticeable on electron microscopy in cases of focal segmental glomerulonephritis?
Effacement of foot processes
How do the glomeruli appear on immunoflorescence in patients with focal segmental glomerulonephritis?
No change
(no immune complex deposits)
How is focal segmental glomerulosclerosis disease treated?
Steroids
How does the renal damage in focal segmental glomerulosclerosis respond to steroid treatment?
Poorly
(progresses to chronic renal failure)
What is the most common cause of nephrotic syndrome in Caucasians?
Membranous nephropathy
Membranous nephropathy is usually ___________ but may be asssociated with hepatitis B, hepatitis C, solid tumors, SLE and drugs (e.g. NSAIDs, penicillamine).
Membranous nephropathy is usually idiopathic but may be asssociated with hepatitis B, hepatitis C, solid tumors, SLE and drugs (e.g. NSAIDs, penicillamine).
Membranous nephropathy is usually idiopathic but may be asssociated with hepatitis ___, hepatitis ___, solid ______, SLE and drugs (e.g. ______, ______).
Membranous nephropathy is usually idiopathic but may be asssociated with hepatitis B, hepatitis C, solid tumors, SLE and drugs (e.g. NSAIDs, penicillamine).
How does membranous nephropathy appear on microscopic exam with H&E stain?
Thick glomerular basement membrane
How do the glomeruli appear on immunoflorescence in patients with membranous nephropathy?
Subepithelial deposits
(‘spike and dome’ appearance)
How is membranous nephropathy treated?
Steroids
How does the renal damage in membranous nephropathy respond to steroid treatment?
Poorly
(progresses to chronic renal failure)
Which causes of nephrotic syndrome are associated with immune complex deposits and will immunofluoresce on microscopy?
Membranous nephropathy;
membranoproliferative glomerulonephritis
Name the respective diseases that are the most common causes of nephrotic syndrome associated with each of the following demographics:
Children
African-Americans
Caucasians
Hispanics
Minimal change disease
Focal segmental glomerulonephritis
Membranous nephropathy
Focal segmental glomerulonephritis
How do glomeruli appear on microscopy with H&E in patients with membranoproliferative glomerulonephritis?
Thickened glomerular basement membrane
(‘tram-track’ appearance)
Type I membranoproliferative glomerulonephritis results in what form of immune deposits?
Subendothelial
(associated with hepatitis B and hepatitis C)
Type II membranoproliferative glomerulonephritis results in what form of immune deposits?
Intramembranous
(associated with C3 nephritic factor — C3 overactivation and depletion)
Type ___ membranoproliferative glomerulonephritis results in subendothelial immune deposits and is associated with hepatitis B and hepatitis C.
Type I membranoproliferative glomerulonephritis results in subendothelial immune deposits and is associated with hepatitis B and hepatitis C.
Type ___ membranoproliferative glomerulonephritis results in intramembranous immune deposits (associated with C3 nephritic factor — C3 overactivation and depletion).
Type II membranoproliferative glomerulonephritis results in intramembranous immune deposits (associated with C3 nephritic factor — C3 overactivation and depletion).
How is membranoproliferative glomerulonephritis treated?
Steroids
How does the renal damage in membranoproliferative glomerulonephritis respond to steroid treatment?
Poorly
(progresses to chronic renal failure)
Which etiologies of nephrotic syndrome respond well to steroid treatment?
Minimal change disease only
Which etiologies of nephrotic syndrome typically do not respond well to steroid treatment and eventually progress to chronic renal failure?
Focal segmental glomerulosclerosis;
membranous nephropathy;
membranoproliferative glomerulonephritis
Nephrotic syndrome is associated with what two systemic disorders?
Diabetes mellitus;
amyloidosis
Diabetes mellitus is associated with nephrotic syndrome.
High serum glucose levels lead to _______ _______ of the vascular basement membrane, resulting in _______ arteriolosclerosis.
Diabetes mellitus is associated with nephrotic syndrome.
High serum glucose levels lead to nonenzymatic glycosylation of the vascular basement membrane, resulting in hyaline arteriolosclerosis.
Which portion of the glomerular vasculature is most affected by nonenzymatic glycosylation in patients with diabetes mellitus?
Efferent arteriole
(> afferent arteriole; resulting in an increased GFR)
What is the characteristic urine finding of diabetes mellitus which results from hyperfiltration (following nonenzymatic glycosylation of the vascular basement membrane)?
Microalbuminuria
The kidney damage associated with diabetes mellitus can eventually progress to _________ syndrome.
The kidney damage associated with diabetes mellitus can eventually progress to nephrotic syndrome.
How does the kidney appear on microscopy in diabetic patients that have progressed to nephrotic syndrome?
(1) Mesangial sclerosis
(2) Formation of Kimmelstiel-Wilson nodules
What medication type can slow the progression of hyperfiltration-induced renal damage in diabetic patients?
ACE inhibitors
Which organ is the most commonly affected in patients with systemic amyloidosis?
The kidneys
Systemic amyloidosis leads to amyloid deposits in the _________ of the kidneys, leading to nephrotic syndrome.
Systemic amyloidosis leads to amyloid deposits in the mesangium of the kidneys, leading to nephrotic syndrome.
How do congo-red-stained amyloid deposits in the kidney appear under polarized light in patients with systemic amyloidosis (progressing to nephrotic syndrome)?
Apple-green birefringence
In patients with systemic amyloidosis, renal amyloid deposits in the mesangium show apple-green birefringence under ________ light after staining with ______ ______.
In patients with systemic amyloidosis, renal amyloid deposits in the mesangium show apple-green birefringence under polarized light after staining with congo red.
Anti-phospholipase A2 receptor antibodies are associated with what renal disorder?
Membranous nephropathy
Cresecent formation in glomeruli is characteristic of what category of renal disease?
Rapidly progressive glomerulonephritis
What glomerular disease is associated with hepatitis B, hepatitis C, and some solid tumors?
Membranous nephropathy