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48 Cards in this Set
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start lecture 29
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*Terms Used In Evaluation of Glomerular Diseases*
...: >50% of glomeruli are affected ...: <50% of glomeruli are affected ...: the entire glomerulus is affected ...: lesion involves only a portion of the glomerulus |
Diffuse
Focal Global Segmental |
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*Characteristics*
... syndrome – massive proteinuria (>3.5 gm/day for adults), hypoalbuminemia, edema, hyperlipidemia/-uria ... syndrome – hematuria, oliguria, azotemia, HTN |
nephrotic
nephritic |
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*Classification of Glomerular Diseases*
... -the disease process is confined to the kidney and does not involve other organs ... -the renal involvement is part of a systemic disease (i.e. diabetes) |
Primary
Secondary |
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...:
Clinical -Most frequent cause of nephrotic syndrome in children -Peak age ...-... years -No hypertension -Preserved renal function -Associations *Children - post infection, post allergen exposure *Adults - Hodgkin disease, ... use |
Minimal change disease (Nil disease, lipoid nephrosis)
2-6 NSAID |
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*Minimal Change Disease*
Biopsy Findings -LM - ... -IF - ... -EM - ... |
normal
negative effacement (flattening) of podocyte foot processes |
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*Minimal Change Disease*
Prognosis -Rapid remission with ... therapy -No progression to chronic disease |
steroid
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*Minimal Change Disease*
-#1 cause of nephrotic syndrome in ... -Loss of ... processes -Pathogenesis ... -... prognosis |
children
foot unknown Good |
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*Focal Segmental Glomerulosclerosis (FSGS)*
Clinical -10% of nephrotic syndrome in children -35% of nephrotic syndrome in adults -Most common cause of nephrotic syndrome in ... -Pathogenesis ... *Is associated with HIV infection, obesity, reflux, pamidronate, heroin use slide 32 |
African-Americans
unknown |
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*Focal Segmental Glomerulosclerosis*
Prognosis -Progressive decline in ... function -Spontaneous remission ... -20% progress to ESRD in less than 3 years (esp. ... pts) -Recurrence in 25-50% of patients after ... -... do not help in adults, may be worth a try in children |
renal
uncommon HIV kidney transplant Steroids |
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*Focal Segmental Glomerulosclerosis*
-Primary or secondary -Some (...) glomeruli show partial (...) hyalinization -... pathogenesis -... prognosis |
focal
segmental Unknown Poor |
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MC nephrotic syndrome in children is ...
MC in adults is ... |
minimal change disease
membranous nephropathy |
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*Membranous Nephropathy*
Pathogenesis -Deposition of immune complexes on the ... side of the GBM -In most cases the inciting antigen ... be identified |
epithelial
cannot |
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*Membranous Nephropathy*
Prognosis -... in 50% at 10-15 years -... remission in 25% -Indolent (prolonged) course in ...% -In idiopathic cases ... and cytotoxic drugs may help (controversial!) |
ESRD
Spontaneous 25 steroids |
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*Membranous Glomerulonephritis*
-... reaction against unknown antigen -Immune complexes (deposition of IgG and complement) -... GBM -... deposits/spikes |
Autoimmune
Thickened Subepithelial |
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start lecture 37
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*Diabetic Nephropathy*
Clinical -Patients typically have DM for ...+ yrs -... (urinary excretion of 30-300 mg/day of albumin) is earliest feature and then overt proteinuria develops -... that may be severe -Often have concurrent retinopathy and hypertension |
10
Microalbuminuria Edema |
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*Diabetic Nephropathy*: Biopsy Findings
LM -... arteriolosclerosis -... cells (cleared out cells in the tubules): glycogen in tubules of uncontrolled diabetes -capillary basement membrane ... -diffuse glomerulosclerosis -... glomerulosclerosis *AKA ... disease (very typical for diabetic nephropathy) -accumulation of hyaline material *in capillary loops = fibrin caps *adherent to Bowman's capsule = capsular drops |
Hyaline
Armani-Ebstein thickening nodular Kimmelstiel-Wilson |
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This is nodular glomerulosclerosis (the ... lesion) of diabetes mellitus. Nodules of pink hyaline material form in regions of glomerular ... in the glomerulus. This is due to a marked increase in mesangial matrix from damage as a result of non-enzymatic glycosylation of proteins.
slide 8 (lecture 37) |
Kimmelstiel-Wilson
capillary loops |
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*Diabetic Nephropathy*
Prognosis -Most common cause of ... -Renal failure develops in 40% of Type ... diabetics and 20% of Type ... diabetics after an interval of 20-30 years after the onset of diabetes -Keeping blood sugar and blood pressure under control ... the progression of diabetic nephropathy |
ESRD
I II delays |
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*Amyloidosis* - Clinical
AL (amyloid light chain) amyloidosis (AKA ... amyloidosis) - most common cause of ... amyloidosis in US -due to plasma cell dyscrasia with ... deposition of amyloid and mild increase in bone marrow plasma cells that are monoclonal Renal involvement seen in amyloidosis secondary to -chronic ... - particularly ... & leprosy -degenerative disease (rheumatoid arthritis, ankylosing spondylitis, lupus-rarely) -malignancy Renal failure develops at variable rate |
primary
renal systemic infections tuberculosis |
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*Amyloidosis*
Biopsy Findings -LM *deposition of amyloid in ... walls and ... *stain positively with ... stain (characteristic ... under polarized light) -IF *negative -EM *deposits of ... fibrils 7-10 nm (intermediate) in diameter |
capillary
mesangium Congo Red apple-green birefringence non-branching |
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*... *
Clinical -Seen with a wide variety of infections -Classically seen after beta hemolytic Group A streptococcal infection -Children ... Adults -Develops ...-... weeks after recovery from Group A strep infection elsewhere in the body (throat, skin) -Abrupt onset of hematuria, renal insufficiency, hypertension, proteinuria, edema “sore throat, face bloat (edema), pee coke (dark urine w/ blood and protein in it)” |
Postinfectious Glomerulonephritis
> 1-4 |
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*Postinfectious Glomerulonephritis*
Clinical -... serum complement (complement consumption) -Elevated ... titers -Pathogenesis *... mediated, circulating or planted antigen -Treat conservatively *... management -No evidence of benefit for steroids, immunosuppressive agents |
Low
ASO (antistreptolysin O) antibody fluid |
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*Postinfectious Glomerulonephritis*
Prognosis -... *complete recovery in >95% of cases *2-3% develop rapidly progressive ... or chronic renal disease -... *complete recovery in 60% of cases *40% develop rapidly progressive glomerulonephritis or chronic renal disease |
Children
glomerulonephritis Adults |
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*Post-Infectious Glomerulonephritis*
... after strep throat ... complexes ... glomeruli ... humps on EM |
Child
Immune Hypercellular Subepithelial |
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*... *
Clinical -AKA Berger disease -... renal disease -Lack of ... manifestations distinguishes from Henoch-Schonlein purpura (skin, GI involvement) -Most common cause of ... worldwide -Affects children and young adults -Males ... Females -Onset often follows an upper respiratory, urinary, or gastrointestinal tract ... |
IgA nephropathy
Isolated systemic glomerulonephritis > infection |
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*IgA Nephropathy*
Common! Child with ... after URI IgA deposition in ... ... prognosis |
hematuria
mesangium Variable |
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*... *
Clinical -Rapid loss of renal function and oliguria -Death in weeks to months if not treated -Three subcategories Characterized histologically by accumulation of cells in Bowman's space in the form of ... |
Rapidly Progressive Glomerulonephritis (RPGN)
crescents |
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*RPGN*
-Rapid and progressive loss of renal ... -Characterized by ... -... subtypes -... prognosis |
function
crescents Three Poor |
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*Systemic Lupus Erythematosus*
Clinical -Women ... Men (9:1 ratio) -... incidence in African-Americans -Age ...’s typically but can occur at any age -Multisystem involvement *skin rashes ( ... rash classic) *joint involvement *anemia *oral ulcers *serosal involvement (pleuritis, pericarditis) *CNS involvement *Renal involvement *Libman-Sacks endocarditis |
>
Increased 20's malar |
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*Systemic Lupus Erythematosus (SLE)*
Clinical -... (an auto immune disease) *...: >95% *Anti-dsDNA: 40-60% *Anti-Smith (20-30%) -Type ... and Type ... hypersensitivity reactions -Treatment with ..., other immunosuppressive agents |
Autoantibodies
Antinuclear antibody (ANA) II III steroids |
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If a patient presents w/ hematuria, eye problems, and deafness, what should you think of?
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Alport’s Syndrome
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*Alport’s Syndrome*
Mutations in the alpha-3, -4, or -5 chain of type ... collagen -... most common -> alpha-5 mutations -... -> alpha-3 or -4 mutations -... rarest -> alpha-3 or -4 mutations Symptoms appear age 5-... ... with progressive development of proteinuria and renal failure ... loss, ... disorders |
IV
X-linked Autosomal recessive Autosomal dominant 20 Hematuria Hearing ocular |
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A 12-year-old boy is a member of a family with a history of renal disease, with males more severely affected than females. He is found to have auditory nerve deafness, corneal dystrophy, and ocular lens dislocation. A urinalysis shows microscopic hematuria. A renal biopsy is performed and the glomeruli show glomerular capillaries with irregular basement membrane thickening and attenuation with splitting of the lamina densa. Which of the following is the most likely diagnosis?
1. Goodpasture syndrome 2. IgA nephropathy 3. Alport syndrome 4. Dominant polycystic kidney disease 5. Diabetes mellitus, type I |
3. Alport syndrome
Hereditary nephritis (Alport syndrome) is not associated with immune complexes, but with a genetic defect of type IV collagen production. In most families, it is inherited in an X-linked dominant pattern. Symptoms usually appear at ages 5 to 20, with overt renal failure between ages 20 to 50. |
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A 5-year-old boy is noted to have increased puffiness around his eyes for the past week, and he has been less active than normal. Vital signs include T 37 C, P 75/minute, RR 18/minute, and BP 140/90 mm Hg. A urinalysis reveals sp. gr. 1.010, pH 6.5, no glucose, 4+ protein, no blood, no casts, and no ketones. Microscopic urinalysis reveals oval fat bodies, but no WBC's or RBC's. He improves following a course of corticosteroid therapy. Which of the following renal lesions is most likely to have been present in this boy?
1. Glomerular crescents 2. Fusion of podocyte foot processes 3. Patchy tubular necrosis 4. Hyperplastic arteriolosclerosis 5. Mesangial immune complex deposition |
2. Fusion of podocyte foot processes
This is minimal change disease, the most common cause for nephrotic syndrome in children, and fusion of podocyte foot processes is the only pathologic finding present. Most patients respond to corticosteroid therapy |
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A clinical study is performed involving subjects with glomerulonephritis. One group of subjects has a diagnosis of crescentic glomerulonephritis and another group has membranous glomerulonephritis. Which of the following laboratory findings is most likely to be found in the absence of other findings in subjects with membranous glomerulonephritis?
1. Rapid onset 2. Red blood cell casts 3. Oliguria 4. Albuminuria 5. Hypertension |
4. Albuminuria
Proteinuria may be present with nephritic syndromes, but it is usually not marked, as in the nephrotic syndrome. Membranous glomerulonephritis typically produces nephrotic syndrome, while membranoproliferative glomerulonephritis leads to a nephritic syndrome. |
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A 20-year-old previously healthy man has been feeling tired for the past 5 days. He goes to his physician when he passes dark-colored urine. On physical examination his blood pressure is 155/90 mm Hg. Labortory studies show his serum creatinine is 4.4 mg/dL. A urinalysis reveals pH 6, specific gravity 1.011, 3+ blood, trace protein, no glucose, and no ketones. On urine microscopic examination there are numerous RBC casts. Which of the following pathologic findings on renal biopsy is most likely to be present in this man?
1. Glomerular crescents 2. Widened proximal tubules 3. Polymorphonuclear infiltrates 4. Lipiduria 5. IgA deposited in glomerular capillaries |
1. Glomerular crescents
Crescents are characteristic for a rapidly progressive glomerulonephritis; they form when there is leakage of fibrinogen into Bowman's space, with proliferation of epithelial cells to form the crescent. |
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A 43-year-old man has had increasing malaise for the past 3 weeks. On physical examination he has a blood pressure of 150/95 mmHg and 1+ pitting edema of the lower extremities to the knees. Dipstick urinalysis shows no glucose, blood, ketones, nitrite, or urobilinogen, and the microscopic urinalysis reveals no RBCs and no WBCs. Additional laboratory studies show a 24 hour urine protein of 4.1 gm. His serum creatinine is 2.2 mg/dL with urea nitrogen of 40 mg/dL. His hepatitis B surface antigen is positive. Which of the following is the most likely diagnosis?
1. Membranous nephropathy 2. Systemic lupus erythematosus 3. Acute tubular necrosis 4. Diabetic nephropathy 5. Post-streptococcal glomerulonephitis |
1. Membranous nephropathy
Membranous GN is the most common cause for nephrotic syndrome in adults. Some cases are associated with underlying infections or malignancies, but in most cases the cause is unknown. |
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A 50-year-old man was diagnosed at age 15 with type 1 diabetes mellitus. His disease has been poorly controlled, as evidenced by elevated hemoglobin A1C levels. He develops a non-healing ulcer of his foot at age 35. At age 45, he has an increasing serum urea nitrogen and a urinalysis shows sp gr 1.012, pH 6.5, 1+ protein, no blood, 1+ glucose, negative leukocyte esterase, negative nitrite, and no ketones. Which of the following renal diseases is he most likely to have?
1. Nodular glomerulosclerosis 2. Hyperplastic arteriolosclerosis 3. Papillary necrosis 4. Crescentic glomerulonephritis 5. Pyelonephritis |
1. Nodular glomerulosclerosis
This is a typical complication of long-standing diabetes mellitus. Microalbuminuria may preceed development of other abnormalities. |
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A 15-year-old girl has had increasing lethargy following a bout of the "flu" 3 weeks ago. On physical examination there are no abnormal findings. Her condition does not improve after 3 weeks on corticosteroid therapy, so a renal biopsy is performed. Microscopic examination shows segmental sclerosis of 3 of 10 glomeruli identified in the biopsy specimen. What is the most appropriate advice to give the girl's parents regarding her condition?
1. She may require a renal transplant in 10 years 2. She will probably improve with additional corticosteroid therapy 3. She will likely develop a restrictive lung disease 4. She has an underlying malignancy 5. She will improve if she loses weight |
1. She may require a renal transplant in 10 years
The findings point to focal segmental glomerulosclerosis (FSGS), which leads to chronic renal failure in half of cases. The lack of resolution with corticosteroid therapy and the progression to chronic renal failure is what sets FSGS apart from minimal change disease. |
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A 10-year-old girl is brought to the physician because of increasing lethargy and passing dark-colored urine for the past week. She had a sore throat two weeks prior to this. On physical examination she is afebrile with blood pressure 140/90 mm Hg. Laboratory studies show her serum creatinine is 2.8 mg/dL and urea nitrogen 24 mg/dL. Urinalysis shows 2+ blood, 1+ protein, no glucose, and no ketones. Microscopic urinalysis shows dysmorphic RBC's. A renal biopsy shows glomerular hypercellularity, with PMNs present. EM shows subepithelial electron dense "humps". Which of the following laboratory test findings is most likely to be present in this girl?
1. Elevated serum glucose 2. Antibody to double stranded DNA 3. Antiglomerular basement membrane antibody 4. Positive rheumatoid factor 5. Elevated antistreptolysin O titer |
5. Elevated antistreptolysin O titer
The hypercellular glomerulus with neutrophils is characteristic for a post-infectious glomerulonephritis, for which a strain of Streptococcus is a likely etiology |
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A 28-year-old man has the sudden onset of hemoptysis and hematuria. On physical examination his blood pressure is 150/95 mm Hg. Laboratory studies show a serum urea nitrogen of 64 mg/dL. A renal biopsy is performed. By immunofluorescent staining there is linear deposition of IgG along the glomerular basement membrane. He has antiglomerular basement membrane antibody in his serum. Which of the following is the most likely diagnosis?
1. Alport's syndrome 2. Membranous nephropathy 3. Diabetic glomerulosclerosis 4. Systemic lupus erythematosus 5. Goodpasture syndrome |
5. Goodpasture syndrome
In Goodpasture syndrome there is an autoantibody directed against glomerular basement membrane. |
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A 49-year-old woman has had increasing malaise for the past 6 months. Her physical examination is unremarkable except for diminished sensation to pinprick and light touch in her lower legs and feet. She is afebrile and normotensive. Laboratory studies show serum creatinine 4.5 mg/dL, urea nitrogen 42 mg/dL, glucose 130 mg/dL, and hemoglobin A1C 7.9%. A urinalysis shows 1+ glucose, 1+ protein, no blood, and no ketones. Urine microscopic examination shows 1 RBC/hpf and 1 WBC/hpf. Which of the following pathologic abnormalities is she most likely to have in her kidneys?
1. Acute pyelonephritis 2. Acute tubular necrosis 3. Hydronephrosis 4. Membranous glomerulonephritis 5. Nodular glomerulosclerosis |
5. Nodular glomerulosclerosis
The classic lesion with diabetes mellitus is nodular glomerulosclerosis, which gradually reduces renal function. Diffuse glomerulosclerosis may also be present. |
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A 5-year-old child has been noted by his mother to be lethargic for 2 weeks. On physical examianation he has periorbital edema. He is afebrile. Dipstick urinalysis reveals no glucose, ketones, or blood, but he has 4+ proteinuria present. Microscopic urinalysis reveals no casts, but oval fat bodies are seen. He is treated with corticosteroid therapy and his condition improves. Which of the following renal electron micrographic findings is most characteristic for this child's disease?
1. Fusion of foot processes 2. Subepithelial electron dense deposits 3. Duplication of glomerular capillary basement membranes 4. Irregular thickening of the glomerular basement membranes 5. Mesangial cell proliferation |
1. Fusion of foot processes
This is the typical (and only) pathologic finding for minimal change disease, the most common cause for nephrotic syndrome in children. |
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A 25-year-old woman has the sudden onset of fever, malaise and nausea. On physical examination she is afebrile. Her blood pressure is 160/90 mm Hg. She has presacral edema. A routine urinalysis shows 1+ protein, no ketones, no glucose, and 4+ blood, with RBC casts seen on urine microscopic examination. A renal biopsy is performed and by light microscopy the glomeruli are hypercellular, with neutrophils present. By electron microscopy there are electron dense subepithelial "humps". Infection with which of the following most likely preceded development of her renal disease?
1. Hepatitis B 2. Schistosoma hematobium 3. Group A streptococcus 4. Mycobacterium tuberculosis 5. Candida albicans |
3. Group A streptococcus
The hypercellular glomerulus with neutrophils is characteristic for a post-infectious glomerulonephritis, for which a strain of Streptococcus is a likely etiology |
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What is the most common cause of glomerulonephritis in the world?
1. Minimal change disease 2. Membranous nephropathy 3. IgA nephropathy 4. Diabetic nephropathy 5. Membranoproliferative glomerulonephritis |
3. IgA nephropathy
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What is the most common cause of end stage renal disease?
1. Minimal change disease 2. Membranous nephropathy 3. IgA nephropathy 4. Diabetic nephropathy 5. Membranoproliferative glomerulonephritis |
4. Diabetic nephropathy
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