Flashcards in 14. Renal Pathology Deck (104):
What other congenital disorder might come with bilateral agenesis of the kidney?
What are three symptoms of Potter fascies on fetus with bilateral agenesis of the kidney?
Flattened nose, low-set ears, and recessed chin.
On unilateral agenesis, what happens to the kidney that made it?
What is the most common abnormal location of the kidney?
What is the most common fusion point of the horseshoe kidney?
Fusion of the lower pole.
What are three gross presentations of autosomal recessive polycystic kidney disease?
1. Bilaterally enlarged kidneys.
2. Multiple small cysts of the cortex and medulla.
3. The cyst are oriented in a radial fashion with their long axis at right angles to the capsules.
What are other two presentation that may come with autosomal recessive polyscystic kidney disease?
Multiple hepatic cysts and congenital hepatic fibrosis.
What gene and what chromosome is the one most commonly affected in adult polycystic kidney disease?
PKD1 gene on chromosome 16. The gene produces a transmembrane protein called polycystin 1.
Other mutations involve PKD2 and PKD3 genes.
What are three clinical presentations of adult polycystic kidney disease?
1. Asymptomatic with normal renal function till middle age; most patients develop end-stage renal failure by their seventh decade.
2. Presents with renal insufficiency, hematuria, and hypertension.
3. Abdominal masses and flank pain.
How is autosomal dominant polycystic kidney disease diagnosed?
Ultrasound and CT scan.
What are 2 gross presentation of autosomal dominant polycystic kidney disease?
1. Massive bilateral kidney enlargement with large budding cysts.
2. Cysts are filled with serous, turbid, or hemorrhagic fluid.
How come does autosomal dominant polycystic kidney disease has a long period of showing it symptoms with renal failure showing up at the third age?
The cysts involve less than 10% of the nephrons, but they gradually expand and compress the rest of the kidney, interfering with its function.
What are 4 extrarrenal manifestations of adult polycystic kidney disease?
1. Liver cysts.
2. Berry aneurysm of the circle of Willis.
3. Mitral valve prolapse.
4. Colonic diverticula.
What is the easiest way to distinguish between Nephritic syndrome and Nephrotic syndrome?
Nephretic is the only one that presents with hematuria.
What are 5 symptoms of Nephritic syndrome?
1. Hematuria (RBC casts).
5. Proteinuria (
What are 5 symptoms of Nephrotic syndrome?
1. Severe proteinuria (>3.5 g/day)
2. Hypoalbuminemia (
What are 6 primary nepritic glomerulopathies?
1. Acute poststreptococcal glomerulonephritis.
2. Goodpasture syndrome (anti-GM disease).
3. Rapidly progressive glomerulonephritis (RPGN).
4. IgA nephropathy (Berger disease)
5. Membranoproliferative glomerulonephritis (MPGN).
6. Alport syndrome.
What renal syndrome does acute poststreptococcal glomerulonephritis cause?
What is the most common organsim that cause poststreptococcal glomerulonephritis?
Beta-hemolytic group A Streptpcoccus.
What two things do we see in the light microscope in poststreptococcal glomerulonephritis?
1. Hypercellular glomerulo with neutrophils and monocytes.
2. Red cell casts in the renal tubules.
What do we see in the immunofluorescence in poststreptococcal glomerulonephritis?
Granular deposists of IgG, IgM and C3 throughout the glomerulus.
What do we see in the electron microscope in poststreptococcal glomerulonephritis?
Subepithelial (lumps) immune complex deposists.
What is the treatment for poststreptococcal glomerulonephritis?
Conservative fluid management.
Which gender is more likely to get Goodpasture syndrome?
What is the pathogenesis of Goodpasture syndrome?
Production of antibodies directed against basement membrane (anti-GBM antibodies), which result in damage of the lungs and kidney; the Goodpasture antigen is the noncollagenous component of type IV collagen.
What type of syndrome does Goodpasture syndrome cause?
What can Goodpasture syndrome develop to?
Rapidly progressive glomerulonephritis.
What do we see in the light microscope in Goodpasture syndrome?
Hypercellularity, cresecents and fibrin.
What can we see in the immunofluorescence in Goodpasture syndrome?
Smooth and linear pattern of IgG and C3 in the GBM.
What can we see in the electron microscope in Goodpasture syndrome?
No deposits, but there is glomerular basement membrane (GBM) disruption.
What organ is affected first in Goodpasture syndrome?
Pulmonary involvement is typically first than renal disease.
What is the treatment for Goodpasture syndrome?
Plasma exchange, steroids and cytotoxic drugs.
What is another name for rapidly progressive glomerulonephritis?
What is clinical feature of rapidly progressive glomerulonephritis?
Rapid progression to severe renal failure in weeks or months.
What 2 things do we see in light microscopy in rapidly progressive glomerulonephritis?
1. Hypercellular glomeruli.
2. Crescent formation in Bowman space.
What do we see in immunofluorescence in rapidly progressive glomerulonephritis?
Variable; may show granular or linear deposits of immunoglobulin an complement.
What do we see in electron microscopy in rapidly progressive glomerulonephritis?
Variable; may or may not have electron-dense deposits. GBM disruption and discontinuity is commonly seen.
What is another name for IgA nephropathy?
What is the most common cause of glomerulonephritis in the world?
What type of renal syndrome does rapidly progressive glomerulonephritis cause?
What type of renal syndrome does IgA nephropathy cause?
What 2 other pathologies are related with IgA nephropathy?
Celiac sprue and Henoch-Schonlein purpura.
What is the pathogenesis of IgA nephropathy?
Mechanism unknown. There is a possible entrapment of circulating immune complex with activation of the alternate complement pathway. There is also a possible genetic predisposition.
What do we see in the light microscope in IgA nephropathy?
Variable; normal or mesangial proliferation.
What do we see in the electric microscopy in IgA nephropathy?
Mesangial immune complex deposits.
What do we see in immunofluorescence in IgA nephropathy?
Mesangial deposits of IgA and C3.
What type of renal syndrome does membranoproliferative glomerulonephritis cause (MPGN)?
May be nephritic, nephrotic or mixed.
How many types of membranoproliferative glomerulonephritis are there?
Type I and type II.
Which type of membranoproliferative glomerulonephritis causes dense deposit disease?
What two things do we see in lab in membranoproliferative glomerulonephritis?
1. Decreased C3.
2. C3 nephritic factor (MPGN type II).
What do we see in the light microscopy of membranoproliferative glomerulonephritis?
1. Lobulated apperance of the glomeruli.
2. Mesangial proliferation and basement-membrane thickening.
3. Splitting of the basement membrane ("tram-tracking") may be seen with a silver or periodic acid-Schiff (PAS) stain. This is due to the splitting of the mesangial cell processes into the capillary loop.
What do we see in immunofluorescense in type I membranoproliferative glomerulonephritis?
Granular pattern of C3 often with IgG, C1q, and C4.
What do we see in immunofluorescence in type II membranoproliferative glomerulonephritis?
Granular and linear pattern of C3.
What do we see in electron microscopy in type I membranoproliferative glomerulonephritis?
Subendothelial and mesangial immune complex deposits.
What do we see in electron microscopy in type II membranoproliferative glomerulonephritis?
Dense deposits within GBM.
Describe Alport syndrome.
A rare X-linked disorder caused by a defect in type 4 collagen that is characterized by hereditary nephritis, hearing loss and ocular abnormalities.
Which gene is the most commonly affected in Alport syndrome?
COL4A5 gene coding for the alpha-5 chain of type 4 collagen.
What are three clinical presentations of Alport syndrome?
1. Gross or microscopic hematuria begins in childhood.
2. Hearing loss leading to sensorineural deafness.
3. Various ocular abnormalities of the lens and cornea can occur.
What do we see in electron microscopy in Alport syndrome?
Alternating thickening and thinning of basement membrane is seen with splitting of the lamina densa.
What are 3 pathologies that causes Nephrotic syndrome?
1. Membranous glomerulonephritis.
2. Minimal change disease.
3. Focal segmental glomerulosclerosis.
What is the most common cause of nephrotic syndrome in adults?
What is the most common etiology of membranous glomerulonephritis?
What 2 things do we see in light microscopy in membranous glomerulonephritis?
1. There is a diffuse membrane-like thickening of the capillary walls.
2. Basement membrane projections ("spikes") are seen on silver stains.
What do we see in the immunofluorescence of membranous glomerulonephritis?
Granular and linear pattern of IgG and C3.
What do we see in immunofluorescence in membranous glomerulonephritis?
Granular and linear pattern of IgG and C3.
What two things do we see in electron microscopy in membranous glomerulonephritis?
1. Subepithelial deposits along the basement membranes.
2. Effacement of podocytes foot processes.
What is another name for minimal change disease?
Lipoid nephrosis, Nil disease.
What is the most common nephrotic syndrome in children?
Minimal change disease.
What do we see in light microscopy in minimal change disease?
Normal glomeruli, but lipid accumulation in proximal tubule cells (lipoid nephrosis).
What do we see in electron microscopy in minimal change disease?
Effacement of epithelial (podocyte) foot processes and microvillous transformation.
What is the treatment for minimal change disease?
Corticosteroids. Prognosis is excellent, dramatic response to steroids in children; majority have a complete recovery.
What type of renal syndrome does focal segmental glomerulonephritis cause?
Which drug abuse is associated with focal segmental glomerulonephritis?
What do we see in the light microscopy in focal segmental glomerulonephritis?
Focal segmental sclerosis and hyalinization of glomeruli. Initially affects the glomeruli along the medullary border.
What do we see in the immunofluorescence in focal segmental glomerulonephritis?
IgM and C3 deposits in the sclerotic segments.
What do we see in electron microscopy in focal segmental glomerulonephritis?
Nonsclerotic regions exhibit effacement of foot processes. Sclerotic segments show increased mesangial matrix.
What do we see in micro changes in chronic glomerulonephritis?
Hyalinization of glomeruli, interstitial fibrosis, atrophy of tubules and a lymphocytic infiltrate.
What are 3 clinical features of acute tubular necrosis?
1. Oliguria, elevation of blood urea nitrogen (BUN) and creatinine.
2. Metabolic acidosis and hyperkalemia.
3. Urinalysis shows dirty brown granular casts and epithelial casts.
What is the most common cause of acute tubular necrosis?
Ischemic acute tubular necrosis: due to decreased blood flow caused by severe hemorrhage, severe renal vasoconstriction, hypotension, dehydration, or shock.
What are 4 drugs that can cause acute tubular necrosis?
What is the definition of acute pyelonephritis?
Bacterial infection involving the renal pelvis, tubules, and interstitium.
Which type of bacterial are the most common cause of acute pylonephritis?
Gram-negative enteric bacilli.
E. Coli, proteus, klebsiella, enterobacterium.
It may be difficult to distinguish cystitis from pyelonephritis. What are three clinical presentations that may be helpful clues to the diagnosis of pyelonephritis?
2. Costovertebral angle tenderness
3. WBC casts in urine
Hamartomas composed of fat, smooth muscle, and blood vessels.
Patients with which pathologies are angiomyolipomas most commonly seen?
With tuberous sclerosis.
What is another name for renal cell carcinoma?
Which genetic disease is associated with renal cell carcinoma?
von Hippel-Lindau disease.
What is the gross presentation of renal cell carcinoma?
Large solitary yellow mass found most commonly in the upper pole. Areas of necrosis and hemorrhage are commonly present. The tumor often invades the renal vein and may extend into the vena cava and heart.
What is the most common type of renal cell carcinoma?
Clear cell carcinoma.
What is the classic triad of clinical features of renal cell carcinoma?
Hematuria, palpable mass and flank pain.
What do we find on someone with bilateral agenesis of the kidney on the ultrasound?
What type of syndrome can renal cell carcinoma cause?
Paraneoplastic syndrome from ectopic hormone production.
1. Polycethemia (erythropoietin production).
2. Hypertension (renin production).
3. Cushing syndrome (Corticosteriod production)
4. Hypercalcemia (PTH-like hormone)
5. Feminization or masculinization (gonadotropin release).
May cause amyloidosis, a leukemoid reaction or eosinophilia.
What is another name for Wilms tumor?
What are the syndromes that come with Wilms tumor?
WAGR syndrome an Beckwith-Wiedemann syndrome.
What are the clinical features of WAGR syndrome?
Wilms tumor, aniridia, genital anomalies, and mental retardation.
What are the 2 tumor suppressor genes and their chromosome involved int Wilms tumor?
What is the peak age of Wilms tumor and what is its presentation?
Peak age of 2 to 5 years. Presents as a large abdominal mass.
What is the gross presetation of a Wilms tumor?
Large solitary tan mass.
What are 3 micro presentations of a Wilms tumor?
1. Metanephric blastema.
2. Epithelial elements (immature glomeruli and tubules).
What is the treatment for Wilms tumor?
Surgery, chemotherapy and radiation.
What are 3 etiologies of cystitis?
1. Organisms such as fecal flora.
2. Radiation cystitis.
3. Chemotherapy agents such as cyclophosphamide (homrrhagic cystitis).
What are 4 clinical signs of cystitis?
4. Suprapubic pain
Systemic signs (fever, chills, malaise) are uncommon.
What is the most common type of bladder tumor?
Transitional cell carcinoma.