Chapter 20 Study Guide Flashcards

1
Q

A nephrectomy specimen reveals a yellow lesion with large amounts of lipids in the kidney of a 63-year-old man. The lesion is most likely related to

a. a deletion of von Hippel-Lindau (VHL) tumor suppressor gene
b. a mutation of myc oncogene
c. an amplification of ras oncogene
d. a loss of bcl-2 proapoptotic gene
e. an aflatoxin related carcinogen

A

A
The kidney contains a large renal cell carcinoma. These tumors contain large amounts of lipids, which accounts for their yellow color on gross examination and the clear appearance of cells in routine microscopic slides. Deletions of the von Hippel-Lindau mutations are found in most clear renal cell carcinomas.

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2
Q

The normal glomerular basement membrane is composed of which type of collagen?

a. Type I
b. Type II
c. Type III
d. Type IV
e. Type V

A

D
Similar to other basement membranes, the glomerular basement membrane is composed of collagen type IV. It is a helical molecule composed of six genetically distinct α chains (α1 to α6). Mutation of the gene for the α5 chain of collagen type IV causes hereditary glomerulopathy of Alport syndrome.

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3
Q

A 6-year-old boy is taken to the pediatrician because of generalized facial edema and somnolence. He has been passing dark brown urine. Physical examination reveals hypertension. Urinalysis shows oliguria, hematuria, proteinuria, and red blood cell casts. Which of the following is the most likely diagnosis?

a. Nephritic syndrome
b. Nephrotic syndrome
c. Rapidly progressive acute renal failure
d. Chronic uremia
e. Nephrolithiasis

A

A
These clinical findings are typical signs and symptoms associated with nephritic syndrome, which is most likely caused by acute glomerulonephritis.

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4
Q

The PKD1 gene encoding polycystin-1 is a large integral membrane protein. It is typically found in persons who have which of the following diseases?

a. Childhood polycystic kidney disease
b. Adult polycystic disease
c. Adult onset renal medullary cystic disease
d. Glomerulocystic disease
e. Acquired renal cystic disease

A

B
The PKD1 gene encoding polycystin-1 is mutated in most persons who have autosomal dominant polycystic kidney disease (PKD), which is also known as adult PKD. This disease is frequently associated with polycystic liver disease and berry aneurysms of the circle of Willis.

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5
Q

A 30-year-old heroin addict who is positive for the human immunodeficiency virus (HIV) is hospitalized because of generalized edema. Laboratory studies show proteinuria, hypoalbuminemia, hyperlipidemia, and lipiduria. Which of the following diseases is most likely to be diagnosed by a biopsy of the kidney?

a. Lipoid nephrosis
b. Focal segmental glomerulosclerosis
c. Membranous nephropathy
d. IgA nephropathy
e. Amyloidosis

A

B
Clinical findings indicate that this patient has nephrotic syndrome. The most likely cause of nephrotic syndrome in a patient who is HIV-positive is focal segmental glomeruloscleros

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6
Q

Which kidney disease characterized by nephrotic syndrome, microscopic hematuria, and glomerular deposits of C3 complement and properdin is caused by an autoantibody called C3 nephritic factor?

a. Membranoproliferative glomerulonephritis type II
b. Focal segmental glomerulosclerosis
c. Membranous nephropathy
d. IgA nephropathy
e. Acute postinfectious glomerulonephritis

A

A
Deposits of C3 complement and properdin in the glomerular basement membranes, visible by electron microscopy in the form of ribbon-like densities and associated with low serum complement, are typical of membranoproliferative glomerulonephritis type II. This kidney disease is caused by C3 nephritic factor, an autoantibody binding to the alternative complement pathway regulating enzyme C3 convertase.

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7
Q

Membranoproliferative glomerulonephritis caused by mixed cryoglobulinemia is most often secondary to which of the following diseases?

a. Diabetes mellitus
b. Multiple myeloma
c. Membranous nephropathy
d. Amyloidosis
e. Human immunodeficiency virus infection

A

C
Membranoproliferative glomerulonephritis caused by mixed cryoglobulinemia is most often a complication of viral hepatitis C. The glomeruli contain deposits of IgG-IgM cryoglobulins, which are also found in the small blood vessels of the skin and joints, resulting in cutaneous vasculitis with petechial hemorrhages and synovitis.

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