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60 Cards in this Set

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Name 3 congenital anomalies of the kidney.
Renal Agenesis: the complete absence of renal tissue; the mother usually suffers from oligohyramnios = Potter syndrome.

Horseshoe kidney: fusion of the 2 kidneys.

Ectopic Kidney: abnormal location of the kidney, usually in the pelvis.
Define renal dysplasia.
Cystic renal dysplais is characterized by the presence of numerous cysts in all or part of a kidney together with the persistence of abnormal structures, such as cartilage and undifferentiated mesenchyem.

Often an associated ureteral malformation.
Define "polycystic kidney diseases"
A heterogeneous group of congenital and acquired disorders that are characterized by distortion of the renal parenchyma because of numerous cysts.

Includes:
Autosomal dominant (adult)
Autosomal recessive (infantile)
Nephronophthisis-Medullary Cystic Disease Complex
Simple Renal Cysts
Autosomal Dominant (Adult) Polycystic Kidney Disease
An autosomal dominant trait that is characterized by progressively EXPANDING CYSTS.

Defective gene (AKPKD-1) has been localized to the short arm of chromosome 16.

Kidneys enlarged bilaterally, weighing alot.

External contour distorted by numerous cysts, which are filled with a clear to straw-yellow fluid.

1/3 have hepatic cysts.
15% have a cerebral aneurysm.

Need long-term dialysis or kidney transplant.
Autosomal Recessive (Infantile) Polycystic Kidney Disease
An uncommon autosomal recessive condition in which dilatation of cortical and medullary collecting ducts leads to a cystic kidney.

SMOOTH external surface. Always BILATERAL.

Liver changes = congenital hepatic fibrosis

Candidate gene may reside on the short arm of chromosome 6.

Most infants die- large kidneys compromise expansion of lungs.
Nephronophthisis-Medullary Cystic Disease Complex
A group of related, autosomal recessive diseases that are characterized by renal medullary cysts, sclerotic kidneys, and renal failure.

85% familial.
Ages 1-20.
Kidneys small, multiple cysts @ the corticomedullary j(n). The cysts accumulate --> atrophic parenchyma --> deteriorating tubular function.
Simple Renal Cysts
Very common lesions, found in 1/2 the population >50 years.

No clinical symptoms unless huge.

Found in outer cortex, bulging the capsule, or in the medulla.
ACUTE renal failure
refers to an acute decline in the GFR, with aresultant increase in BUN (blood urea nitrogen) and serum creatinine values.

Can result from damage to any part of the kidney or blood vessels. Can also result from extra-renal lesions and is a frequent complication of HYPOTENSIVE SHOCK.
CHRONIC renal failure
Refers to end-stage renal disease, which is characterized clinically by UREMIA- the clinical syndrome associated with a severe and chronically elevated level of BUN = azotemia.

Characterized by marked sclerosis of both glomeruli and the interstitium and by tubular atrophy.
Define nephrotic syndrome.
Characterized principally by heavy proteinuria together with hypoproteinemia (hypoalbuminemia), peripheral edema, and hyperlipidemia.

Most result from NON-inflammatory glomerulopathies.
Define Nephritic Syndrome.
Characterized by:
1. hematuria
2. proteinuria
3. oliguria (not enough urine)
4. decreased GFR (with resulting elevations in BUN and serum creatinine values).

salt + water rention --> hypertension + edema

Often associated with INFLAMMATORY glomerular diseases.
Minimal Change Glomerulopathy
A glomerular disorder that is characterized clinically by nephrotic syndrome and pathologically by fusion of the visceral epithelial foot processes.

a.k.a. epithelial cell disease or lipoid nephrosis.

disorder of CHILDREN (boys <6)

Association with an allergic history-involvement of immune system.

Loss of foot processes.

No tendency to progress into chronic renal failure.
Focal Segmental Glomerulosclerosis
Refers to a malady in which some (focal) glomeruli exhibit segmental areas of sclerosis in the capillary tufts whereas others appear to be normal.

-idiopathic
-decreased renal mass
-healed focal glomerular lesions

The frequent accumulation of a periodic acid-Schiff (PAS)-positive material in the affected areas produces a leion referred to as HYALINOSIS.
Membranous Glomerulopathy
A frequent cause of nephrotic syndrome in adults and results from the accumulation of immune complexes in the subepithelial zone of glomerular capillaries.

In adult, 1 of the most frequent associations is with CARCINOMAS, being found in as many as 10% of these patients.

Associated with hepatitits B and ~10% of patients with SLE present with a lesion of membranous nephropathy.
Diabetic Glomerulosclerosis
A.k.a. Kimmelstiel-Wilson disaese.
Embraces the glomerular changes seen in patients with diabetes, which result in proteinuria and progressive renal failure.

Pathogenesis related to severeity and duration of hyperglycemia.

Early thickening of the glomerular BM --> widening of mesangial areas --> accumulation of a PAS-positive matrix.

Manifested by hyaline arteriolosclerosis, which in patients with diabetes uniquely involves both the afferent and efferent arterioles.

LEADING CAUSE of end-stage renal disease in the US.
Diffuse glomerulosclerosis
enlarged glomeruli with expanded mesangial areas, with the accumulation of a PAS-positive matrix.

[diabetic]
Nodular glomerulosclerosis
describes single or multiple nodules in the glomeruli. THese are rounded, homogeneous, and eosinophilic masses in centrilobular areas. The nodules become acellular.

[diabetic]
Renal Amyloidosis
Refers to the deposition in the kidneys of diverse extracellular proteins that have common morphologic properties, stain with specific dyes, and have a characteristic appearance under polarized light when stained with certain dyes.
Hereditary Nephritis (Alport Syndrome) [non-inflammatory]
A proliferative and sclerosing glomerular disease that is often accmpanied by defects of the ears, or ocassionally the eyes, and that is caused by a genetic abnormality in type IV collagen.

X-linked, caused by a mutation in the gene COL4A5 that codes for type IV collagen.

No lesion.

1/3-1/2 of patients have a progressive hearing impairment. A smaller proportion suffers ocular defects.
Thin Glomerular Basement Membrane Nephropathy
A.k.a. Benign familial hematuria.

A hereditary, noninflammatory disorder that typically presents as recurrent hematuria in childhood or young adulthood and is characterized by reduced thickness of the glomerular capillary basement.

Doesn't preogress to renal failure.

Now recognized as being 1 of the most frequent causes of asymptomatic hematuria, even in patients wiht no family history of renal disease.
Define Glomerulonephritis.
Refers to INFLAMMATORY lesions of the glomerulus that are characterized histologically by hypercellularity of the glomeruli and clinically by nephritic syndrome.
Pathogenic Mechanisms of Glomerulonephritis.

Immunologic injury can be divided into 4 basic types:
1. trapping of circulating immune complexes.
2. in situ immune complex formation
3. activation of the alternative pathway of complement
4. cell-mediated processes
Acute Postinfectious Glomerulonephritis
Characterized by the sudden onset of nephritic syndrome and morphologically by diffuse hypercellularity of glomeruli.

Sequel to infection; most frequent = certain strains of group A beta-hemolytic streptococci (S. pyogenes)

Probably caused by glomerular localization of immune complexes.

Hypercellularity caused by proliferation of both endothelial and mesangial cells.

Complete recovery is the rule.
Membranoproliferative Glomerulonephritis
Characterized by the combination of glomerular BM thickening ("membrano") and mesangial cell proliferation ("proliferative").

Type I
Type II
Systemic Lupus Erythematosus (SLE)
70% of these patients will develop clinically significant renal disease.

Is the prototypical example of a human immune complex disease.

Heavy subendothelial deposits can be recognized as a marked thickening of the involved capillary wall, which results in a WIRE LOOP.

Hematoxylin bodies may be found.
IgG is the most frequent immunoglobulin.

Full house pattern = IgG, IgA, IgM, C3, C4, C1q
5 classes of lupus nephritis
1: histologically normal
2: pure mesangial lesion
3: focal and segmental glomerulonephritis
4. diffuse proliferative glomerulonephritis
5. diffuse membranous nephropathy

2,5 = benign course
IgA Nephropathy (Berger disease)
A glomerulonephritis caused by accumulation of immune complexes, composed of IgA.

Aggregates of IgA and fibronectin in the blood found in 70% of patients.

Known association with chronic liver disease.

C3 and properdin present with IgA.

Most common in young men.

Not responsive to therapy.
Crescentic Glomerulonephritis
An ominous morphologic pattern in which the majority of glomeruli are surrounded by an accumulation of cells in the Bowman space.

Fulminant glomerular damage, always leaves scarring.

Escape of fibrin into the Bowman space; fibrin can stimulate the migration of macrophages into glomeruli.
Anti-GBM antibody disease = anti-glomerular basement membrane antibody disease
a rare condition in which renal disease is mediated by an autoantibody directed against a component of the glomerular BM.

80% male, average age = 29.

Cross-reactivity of the antibodies with alveolar basement membrnaes --> pulmonary hemorrhages and recurrent hemoptysis.

Goodpasture syndrome when both lungs and kidneys involved.
Antineutrophil Cytoplasmic Autoantibody Glomerulonephritis
ANCA glomerulonephritis is an aggressive, neutrophil-mediated disease that is characterized by necrosis in glomerular crescents but not immunoglobulin deposits.

ANCAs are specific for proteins in the cytoplasm of neutrophils and monocytes.

Most common clinical presentation: rapidly progressive renal failure.

3/4 have systemic small vessel vasculitis --> pulmolnary hemorrhage.
Benign Nephrosclerosis (Hypertensive Nephrosclerosis)
Refers to renal vascular and glomerular sclerosis that occurs with mild to moderate hypertension (160/90).

Consequence of renal ischemia.

Kidneys are small and affected bilaterally.

Among blacks in the US, hypertension without any evidence of a malignant phase is the single leading cause of end-stage renal disease.
Malignant Nephrosclerosis
Refers to the renal changes associated with malignant hypertension, including a diastolic blood pressure of >125 mmHg, the presence of retinal changes and papilledema, and functional impairment of the kidney.

Can arise de novo in apparently healthy people - young black men.

"flea-bitten" kidney - cortical surface has petechiae.

Cut surface sometimes exhibits small cortical infarcts.
Small vessel vasculitis
Affects small arteries, arterioles, capillaries, and venules.

Glomerulonephritis is a frequent component.

Can be caused by immune complexes, anti-BM antibodies, or ANCAs
Henoch-Schonlein purpura
[renal vasculitis]

Most common type of childhood vasculitis.

Caused by vascular localization of immune complexes containing IgA.
Medium-size vessel vasculitis
involves only arteries.

polyarteritis nodosa: asults
Kawasaki disease: children
Large vessel vasculitis
Giant cell arteritis
Takayasu arteritis

affects the aorta and its major branches. May cause renovascular hypertension by involving the renal arteries.
Thrombotic Microangiopathy
Refers to a group of diseases sharing similar renal microvascular lesions, including thrombosis, arterial intimal expansion, arteriolar fibrinoid necrosis, and glomerular consolidation.

-hemolytic-uremic syndrome
-thrombotic thrombocytopenic purpura
Hemolytic-Uremic Syndrome
Describes a condition of acute renal failure, thrombocytopenia, and hemolytic anemia.

Seems to result from glomerular injury produced by toxins elaborated by a virulent serotype of Escherichia coli. The toxin injures endothelial cells.
Thrombotic Thrombocytopenic Purpura
o Is a microangiopathic disease that differs from adult hemolytic-uremic syndrome in that it
1. Generally occurs among an older age group
2. Has a lesser involvement of the kidney
3. Affects many organs, particularly the CNS
4. Has a worse prognosis
o Clinical features include a low platelet count, generalized bleeding, hemolytic anemia, neurologic abnormalities, fever, renal disease.
o Women>men
Scleroderma (progressive systemic sclerosis)
• A disease in which excess collagen is deposited, typically in the skin but also in other organs such as the GI tract, lungs, and heart.
• Vascular changes, particularly in the fingers and the kidney, always develop.
• Interlobular arteries and afferent arterioles are the sites of the lesions.

grave prognosis.
Renovascular Hypertension
Refers to elevated systemic blood pressure secondary to renal ischemia cuased by narrowing of a renal artery.

90-95% of cases caused by lesions of atherosclerosis = men, older age group.

The remaining cases reflect fibromuscular dysplasia.

hyperplasia of the juxtaglomerular apparatus --> increase in renin, angiotensin II, aldosterone.
Bilateral Cortical Necrosis
Refers to necrosis of part, or all, of the renal cortex, with sparing of the medulla.

Lesion is ischemic in origin.

Historically, most common clinical circumstance was premature separation of the placenta.
Describe diseases of the tubules and interstitium.
Characterized by injury to the renal tubule and an inflammatory infilrate in the interstitium.

patients demonstrate defects in tubular function, including
-inability to concentrate urine
-salt wasting
-metabolic acidosis
Define Pyelonephritis
A combined inflammation of the parenchyma, calyces, and renal pelvis that is caused by a bacterial infection.
Development of ascending ACUTE pyelonephritis depends on what 4 factors?
a source of pathogenic micro-organisms
infection of the urine
reflux of the infected urin up the reters and into the renal pelvis and calyces
entry of the bacteria through the papillae and into the renal parenchyma.

most common offending organisms: e. coli
CHRONIC pyelonephritis
a chronic tubulointerstitial disorder with gross, irregular, often asymmetric scarring together with deformation of the calyces and the overlying parenchyma.

End-stage kidney: a shrunken and fibrotic kidney that is insufficient to maintain renal function.

Relationship to TUBULOINTERSTITIAL DAMAGE!!!

Most of the cases without any form of obstruction are associated with REFLUX NEPHROPATHY.

Parenchyma is uniformly thinned = hydronephrosis.

Thyroidization pattern
Acute Tubular Necrosis (ATN)
• Is a severe, but potentially reversible, impairment of tubular epithelial function that is caused by ischemia or toxic injury and that results in acute renal failure.
3 types of acute renal failure
Pre-renal acute renal failure: most often results from a fall in renal blood flow caused by systemic hypotension or hypovolemia.

Post-renal acute renal failure: relates to urinary tract obstruction, which must involve both ureters or the common lower urinary outflow tract.

Intra-renal acute renal failure: may result from a lesion in any part of the kidney, including
1. Glomeruli
2. Blood vessels
3. Interstitium and tubules
Ischemic ATN
occurs most often in resposne to shock or dehydration.

o Tubules show these changes:
 “Distalization” (simplification) of proximal tubules: they are dilated and loss of brush border.
 Single Cell Necrosis with Denudation of the Basement Membrane
 Granular Casts
 Nucleated cells in the vasa recta: mononuclear leukocytes.
 Regenerative Changes of the Tubular Epithelium: appearance of mitoses, increased size of cells and nuclei, and cell crowding.
Toxic ATN
toxins include heavy metals, antibiotics, certain radiolabeled contrast agents, organic solvents, and cyclosporin A.
Drug-Induced (Hypersensitivity) Acute Tubulointerstitial Nephritis
An acute inflammation of the tubules and interstitium that is characterized by infiltrating lymphocytes and eosinophils.

Drugs include NSAID's and certain antibiotics.

Type IV cell-mediated immune response.

"tubulitis": proximal and distal tubules are focally invaded by leukocytes.
Analgesic Nephropathy
is chronic inflammation and scarring of the renal parenchyma caused by the use of analgesic drugs = phenacetin.

both kidneys shrunken equally.
Multiple Myeloma Cast Nephropathy
• Refers to renal injury caused by monoclonal immunoglobulin-like chains in the urine that produce tubular epithelial injury and numerous tubular casts.
• Circulating LIGHT CHAINS filtered by the glomeruli probably cause this.
o At the acidic pH of urine, light chains can precipitate and cause tubular damage and obstruction.
• Characteristic tubular lesion: numerous dense, lamellated, and fractured casts in the distal and collecting tubules. These are eosinophilic and refractile and sometimes surrounded by giant cells.
• The casts contain light-chain material, usually K light chains.
Urate Nephropathy
• Refers to renal disease caused by the deposition of uric acid crystals in the tubules and interstitium.
• Any condition associated with high levels of uric acid in the blood can lead to this.
o Classic disease: primary gout
• Chemotherapy can also increase the blood uric acid level.
Hydronephrosis
Is defined as dilatation of the renal pelvis and calyces, flattening of the papillae, and in chronic cases, atrophy of the renal cortex.

Always the result of urinary tract obstruction, from tumors, stones, etc.

Most prominent microscopic finding: dilatation of the collecting ducts, followed by dilatation of the proximal and distal convoluted tubules --> loss of tubules is common.

Glomeruli SPARED.

Atrophy of an obstructed kidney is inevitable.
Renal stones (nephrolithiasis and urolithiasis)
• Refer to stones within the collecting system of the kidney (nephrolithiasis) or elsewhere in the collecting system of the urinary tract (urolithiasis).
• Pelvis and calyces are common sites for formation/accumulation of calculi.
• Men>women
• Hypercalciuria is found in the absence of hypercalcemia.
• Idiopathic hypercalciuria: has been linked to increased INTESTINAL absorption of calcium and decreased reabsoprtion of calcium in the proximal tubules.
• Passage of a stone into the ureter causes excruciating flank pain = RENAL COLIC.
• Ultrasonic disintegration and endoscopic removal are often effective treatments.
3 types of renal stones
• Calcium stones: the most common stone contains calcium complexed with oxalate, phosphate, or a mixture of these anions.

• Magnesium Ammonium Phosphate Stones: in the presence of infection with urea-splitting bacteria; the resulting alkaline urine favors precipitation of magnesium ammonium phosphate stones.
o These stone fill the pelvis and calyces to form a cast of these spaces= STAGHORN CALCULI

• Uric Acid Stones: occurs in 25% of patients with gout; stones are smooth, hard, and yellow.
Wilms Tumors (nephroblastoma)
• Is a malignant neoplasm of embryonal nephrogenic elements that is composed of mixtures of blastemal, stromal, and epithelial tissues.
• One of the most common solid tumors in very young children (<4).
• 99% of the cases are sporadic.
o ~10% of these seem to be related to loss of suppressor gene activity.
o The gene (WT-1) is located on chromosome 11 (11p13).
o Another gene (WT-2) may also be related.
Wilms tumors are composed of elements that resemble what 3 types of normal tissue?
1. Metanephric blastema—small ovoid cells, growing in nests and trabeculae.
2. Immature stroma (mesenchymal tissue)—composed of undifferentiated spindle cells.
3. Immature epithelial elements—small tubular structure and immature elements resembling glomeruli.
Renal cell carcinoma
• The most important neoplasm of the kidney
• A malignant tumor derived from the epithelial cells of the renal tubules.

Cigarette smoking, obesity (esp. women), analgesic nephropathy.

Associated with von Hippel-Lindau disease.

Small tumors that appear to be well-differentiated renal cell carcinomas are called RENAL CELL ADENOMAS.

All tumors considered potentially malignant.

Hematuria = #1 sign.

Distant metastases in lung/bone.