Renal Diseases

Question 1

Azotemia

Answer 1

Elevation of the blood urea nitrogen (BUN) and creatinine levels, due to decreased filtration of blood through the glomeruli (decreased glomerular filtration rate).

Question 2

Uremia

Answer 2

Association of azotemia with clinical signs and symptoms, including gastroenteritis, peripheral neuropathy, pericarditis, dermatitis, hyperkalemia, and metabolic acidosis. advanced form of liver disease

Question 3

Azotemia is due to ________

Answer 3

decrease glomerular filtrate

Question 4

you get increased levels of ____ and ____ in azotemia

Answer 4

nitrogen and creatinine

Question 5

Major Clinical Renal Syndromes

Answer 5

Acute nephritic syndrome
Nephrotic syndrome
Acute renal failure (rapidly)

Question 6

Acute nephritic syndrome

Answer 6

Results from glomerular injury and is characterized by acute onset of hematuria (blood in urine), mild to moderate proteinuria, azotemia, and hypertension.

Question 7

Nephrotic syndrome

Answer 7

Glomerular syndrome characterized by heavy

proteinuria (> 3.5 grams per day), hypoalbuminemia, severe edema, hyperlipidemia, and lipiduria.

Question 8

nephritic associated simply with ____ in urine

Answer 8

blood

Question 9

nephrotic associated simply with ____ in urine

Answer 9

protein

Question 10

Acute renal failure

Answer 10

Acute onset of azotemia with oliguria (or anuria). (inability to produce urine)

Question 11

CONGENITAL CYSTIC KIDNEY DISEASE (2)

Answer 11

Autosomal dominant (adult) polycystic kidney disease
Autosomal recessive (childhood) polycystic kidney disease

Question 12

auto dominant associated with ____ (age group)

Answer 12

adult

Question 13

auto recessive associated with ____ (age group)

Answer 13

children

Question 14

Autosomal dominant (adult) polycystic kidney disease clinical presentation

Answer 14

characterized by MULTIPLE EXPANDING CYSTS in BOTH kidneys. Gradual onset of renal failure in adult, urinary tract hemorrhage (hematuria), pain, hypertension, urinary tract infection.

Question 15

Autosomal dominant (adult) polycystic kidney disease etiology

Answer 15

defective gene is PKD1 (in 90% of families) located on chromosome 16. The gene encodes for polycystin-1

Question 16

Autosomal dominant (adult) polycystic kidney disease Extrarenal pathology

Answer 16

1/3 of patients have CYSTS IN LIVER; aneurysms

may develop in the circle of Willis (intracranial)

Question 17

Autosomal dominant (adult) polycystic kidney disease Pathology

Answer 17

very large (up to 4 kg) kidneys with numerous cysts that
arise in every part of the tubular system
BIG KIDNEYS

Question 18

Autosomal recessive (childhood) polycystic kidney disease clinical presentation

Answer 18

renal failure develops from infancy to several years of age – rare; seen in 1 in 20,000 live births.

Question 19

Autosomal recessive (childhood) polycystic kidney disease extrarenal pathology

Answer 19

almost all have liver cysts and progressive liver fibrosis

Question 20

Autosomal recessive (childhood) polycystic kidney disease pathology

Answer 20

numerous small uniform-size cysts from collecting tubules in cortex and medulla

Question 21

Autosomal recessive (childhood) polycystic kidney disease etiology

Answer 21

Due to mutations in the PKHD1 gene

Question 22

Mechanisms of glomerular injury

Answer 22

1. Immune complex deposits in glomerular basement membrane (GBM)
   or mesangium.
2. Epithelial and endothelial cell injury.

Question 23

Pathologic evaluation of kidney biopsies

Answer 23

1. Light microscopy
2. Immunofluorescence
3. Electron microscopy

Question 24

Nephrotic syndrome 4 manifestations

Answer 24

1. Minimal change disease
2. Focal and segmental glomerulosclerosis
3. Membranous nephropathy (glomerulonephritis)
4. Glomerular disease in diabetes mellitus

Question 25

Minimal change disease- nephrotic

Answer 25

1. most common nephrotic in KIDS 2. normal histology of glomeruli 3. good response to treatment (in kids)

Question 26

Focal and segmental glomerulosclerosis- nephrotic

Answer 26

1. one of the most common causes of nephrotic syndrome in ADULTS 2. may be primary (idiopathic) or secondary to other glomerular diseases, loss or scarring of other glomeruli, or genetic. 3. pathology - partial (segmental) sclerosis of some (focal) glomeruli characterized by increased mesangial matrix collagen with obliteration of capillary loops. The idiopathic form has NO immune complexes. 4. poor response to corticosteroid treatment – renal failure in 50% after 10 yrs.

Question 27

Membranous nephropathy (glomerulonephritis)- nephrotic

Answer 27

a) most common in ADULTS age 30-50 b) may be primary (disease limited to the kidney) or secondary to infection, malignancy, SLE, or drugs. c) pathology - IMMUNE COMPLEXES in the epithelial side (subepithelial) of the GBM demonstrable by immunofluorescence and electron microscopy d) poor response to corticosteroid treatment, with 40% developing renal failure in 2-20 years

Question 28

Glomerular disease in diabetes mellitus - nephrotic

Answer 28

a) minimal proteinuria progresses, over 10-15 years, to severe proteinuria. b) thick glomerular basement membranes, diffuse increase in mesangial matrix and formation of mesangial nodules (the latter is nodular glomerulosclerosis or Kimmelstiel-Wilson lesion)

Question 29

nephrotic typically more chronic or acute?

Answer 29

chronic

Question 30

nephritic typically more chronic or acute?

Answer 30

acute

Question 31

basics of nephritic

Answer 31

acute blood in urine failure to produce urine

Question 32

two main causes of nephritic syndrome

Answer 32

1. Acute postinfectious (poststreptococcal) glomerulonephritis 2. IgA nephropathy

Question 33

Acute postinfectious (poststreptococcal) glomerulonephritis

Answer 33

a) CHILDREN mostly after strep throat (streptococcal pharyngitis) b) pathology - proliferation of endothelial and mesangial cells; infiltration of neutrophils and monocytes; immune complexes in GBM and sometimes in the mesangium. basically a immune reaction and INFLMMATORY cells c) progression to chronic renal disease is more likely in adults.

Question 34

IgA nephropathy

Answer 34

a) usually occurs in CHILDREN and young adults b) hematuria is noted 1-2 days after non-specific upper respiratory tract viral infection. c) pathogenesis may involve increased IgA IMMUNE COMPLEXES production d) when the glomerular disease is associated with systemic manifestations (purpuric skin rash and arthritis), this is called Henoch-Schönlein purpura. e) pathology - mesangial deposition of immune complexes containing IgA and variable proliferation of mesangial and endothelial cells. Occasionally there is epithelial cell proliferation with crescents. affects mainly central core of glomerulus

Question 35

Crescentic or Rapidly Progressive Glomerulonephritis

Answer 35

antibodies directed against a glomerular basement antigen, deposition of immune complexes, or lack of immune complex deposition (called pauci-immune glomerulonephritis). characteristic finding is crescentic glomerulonephritis due to proliferation of endothelial cells and histocytes around the edge.

Question 36

CHRONIC (END-STAGE) RENAL DISEASE

Answer 36

SCAR TISSUE, BECOMES NON FUNCTIONAL. Loss of glomeruli and tubules leads to fibrosis. Damaged glomeruli become completely sclerotic (global sclerosis). With advanced loss of tubules and glomeruli, the remaining glomeruli develop adaptive changes. These changes eventually lead to further injury and progressive renal failure.

Question 37

IgA nephropathy main points

Answer 37

1. children/ young adults 2. hematuria after non specific upper resp. viral infection 3. IgA immune complexes

Question 38

``` Acute postinfectious (poststreptococcal) glomerulonephritis main points ```

Answer 38

1. children after strep 2. proliferation of inflammatory cells and immune complexes 3. can progress to chronic renal diease in adults

Question 39

what is ACUTE PYELONEPHRITIS

Answer 39

renal disease affecting tubules, interstitium, and pelvis and is most often secondary to bacterial infection

Question 40

where does this secondary infection come from?

Answer 40

``` The infection may spread from the urinary bladder, up the ureters and into the renal pelvis and kidney (ascending) or hematogenous (blood) spread of bacteria.. ```

Question 41

ACUTE PYELONEPHRITIS clinical presentation

Answer 41

Sudden onset with pain at the costovertebral angle and systemic evidence of infection. Often there is accompanying dysuria, frequency and urgency.

Question 42

ACUTE PYELONEPHRITIS pathology

Answer 42

Patchy interstitial and tubular neutrophilic inflammation.

Question 43

ACUTE PYELONEPHRITIS etiology

Answer 43

Predisposing conditions, include urinary tract obstruction, instrumentation, vesicoureteral reflux, pregnancy, gender and age, diabetes mellitus and immunosuppression.

Question 44

Drug-Induced Interstitial Nephritis etiology

Answer 44

some antibiotics, non-steroidal anti-inflammatory drugs and others.

Question 45

Drug-Induced Interstitial Nephritis pathology

Answer 45

- hypersensitivity reaction to the drugs. - consists of interstitial infiltration of mononuclear inflammatory cells, often with neutrophils and many eosinophils. - The glomeruli are not involved in the inflammation

Question 46

are the glomeruli inovled in drug induced interstitial nephritis

Answer 46

no

Question 47

rapid onset of renal failure, reduced urine output, electrolyte imbalances. The clinical manifestations are reversible over a period of weeks as the damaged tubular epithelium regenerates.

Answer 47

Acute Tubular Necrosis clinical

Question 48

injury to tubular epithelial cells from ischemia (ie. shock) or a toxin

Answer 48

Acute Tubular Necrosis

Question 49

dilation of tubules, interstitial edema, necrosis of epithelium (often very focal and subtle with ischemic injury, more diffuse with injury from a toxin)

Answer 49

Acute Tubular Necrosis

Question 50

Gross – kidneys are symmetrically atrophic, with moderate reduction in size. The kidney surface has an even fine granularity and the cortex is thin.

Answer 50

Arterionephrosclerosis- pathology

Question 51

Narrowing of the lumens of arterioles and arteries caused by hyaline type of arteriolosclerosis and fibroelastic hyperplasia of muscular arteries. In addition, there is (1) tubular atrophy and interstitial fibrosis and (2) global sclerosis of glomeruli.

Answer 51

Arterionephrosclerosis- histopathology

Question 52

Relatively rapid onset of renal failure with increased intracranial pressure leading to headache, nausea, vomiting, and visual impairment.

Answer 52

Arterionephrosclerosis associated with malignant hypertension clinical presentation

Question 53

Arterioles show hyperplastic arteriolosclerosis, reducing | blood flow and causing necrosis of glomeruli.

Answer 53

Arterionephrosclerosis associated with malignant hypertension pathology

Question 54

acquired defect in ADAMTS 13, a plasma protease that degrades vWF multimers. The large von Willebrand factor components activate platelets under certain conditions.

Answer 54

Thrombotic thrombocytopenia purpura (TTP)

Question 55

endothelial cell injury which, in most cases, is due to a Shiga-toxin from E. coli (reason for ground beef recalls in the news) or Shigella. The injury leads to platelet activation.

Answer 55

Hemolytic-uremic syndrome (HUS)

Question 56

similarities between TTP and HUS

Answer 56

microthrombus formation in capillaries.

Question 57

which disease has more renal involvement predominates and the disorder most often occurs in children.

Answer 57

Hemolytic-uremic syndrome

Question 58

which disease is there more widespread involvement of other organs

Answer 58

Thrombotic thrombocytopenia purpura

Question 59

Stones result in urinary tract obstruction, ulceration of the urothelial lining and bleeding. Small stones may migrate into the ureters and produce intense flank pain. Large stones remain in the pelvis and may be manifested by hematuria and superimposed infection.

Answer 59

Urolithiasis (Renal Stones) clinical

Question 60

Stones are unilateral in 80% of patients and most frequent site for their formation is within the calyces and pelvis. Stones may also develop in the bladder. Large stones are referred to as "staghorn calculi". These large stones form a cast of the pelvis and calyceal system.

Answer 60

Urolithiasis (Renal Stones) pathology

Question 61

Types of stones in urolithiasis

Answer 61

3. calcium - patients have increased concentration of calcium in the urine 2. magnesium ammonium phosphate - patients have persistently alkaline urine. Infection with Proteus predisposes to these stones. 1. Uric acid - occur in patients with gout, leukemia (high cell turnover) or persistently acid urine.

Question 62

2:1 male:female ratio. Hematuria, mass, pain, fever, polycythemia, paraneoplastic syndromes

Answer 62

renal cell carcinoma

Question 63

Risk factors include smoking, hypertension, obesity, | cadmium exposure and von Hippel-Lindau syndrome.

Answer 63

renal cell carcinoma

Question 64

Arise from tubular epithelium, often reach a large size prior to diagnosis, often invade the renal vein. Most common histologic subtype has cells with very pale or clear cytoplasm (clear cell carcinoma)

Answer 64

renal cell carcinoma

Question 65

Abdominal mass, risk is greatly increased in some inherited | syndromes, occurs in children ages 2 to 5 years

Answer 65

Wilm’s Tumor

Question 66

Triphasic pattern with epithelial structures resembling primitive tubules or glomeruli, stroma (mesenchymal component) and blastema which recapitulates early nephron formation. Thus the tumor illustrates abortive formation of renal structures in various stages of renal development.

Answer 66

Wilm's Tumor