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Flashcards in Kidney Deck (379)
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1

Infantile polycystic kidney disease: Inheritance.

Autosomal recessive.

2

Infantile polycystic kidney disease:

A. Gene and its location.
B. Encoded protein.

A. PKHD1 on chromosome 6p21-23.

B. Polyductin / fibrocystin.

3

Infantile polycystic kidney disease: Presentation (2).

Abdominal distention.

Stillbirth or early neonatal death in most cases.

4

Infantile polycystic kidney disease: Associated abnormalities (2).

Pulmonary hypoplasia.

Congenital hepatic fibrosis.

5

Infantile polycystic kidney disease: Gross pathology.

Massive enlarged kidneys with smooth surface.

6

Infantile polycystic kidney disease:

A. Location and arrangement of cysts.
B. Calyceal system.

A. Cortex and medulla; oriented perpendicular to the renal capsule.

B. Normal.

7

Infantile polycystic kidney disease: Histopathology (2).

Dilated collecting ducts lined by bland cuboidal cells.

Nephrons appear normal.

8

Medullary cystic disease: Presentation.

Renal dysfunction in childhood:

− Polyuria and polydipsia.
− Uremia.
− Growth retardation.

9

Medullary cystic disease: Gross pathology.

Kidneys are small; both are affected.

10

Medullary cystic disease: Number, size, and location of cysts.

Many.

Up to 2 cm in diameter.

Corticomedullary junction.

11

Medullary sponge kidney: Presentation.

Found in children or adolescents.

12

Medullary sponge kidney: Gross pathology.

Kidneys are typically of normal size.

13

Medullary sponge kidney: Size and location of cysts.

Less than 0.5 cm.

Medullary pyramids, renal papillae; communication with collecting ducts.

14

Medullary sponge kidney: Prognosis.

Renal function is usually normal.

Progression to end-stage renal disease occurs rarely.

15

Renal dysplasia: Clinical significance (2).

Dysplastic kidneys usually do not function.

Nearly always accompanied by other abnormalities of the urinary tract.

16

Renal dysplasia: Presentation (2).

If large: Palpable mass.

If small: May remain asymptomatic for many years.

17

Renal dysplasia: Possible outcomes in utero (2).

Oligohydramnios with Potter's sequence.

Death from pulmonary hypoplasia.

18

Renal dysplasia: Gross pathology.

Distorted kidneys with variable of cysts.

19

Renal dysplasia: Histopathology (3).

Disorganized renal parenchyma containing cartilage.

Dysplastic ducts lined by columnar epithelium and surrounded by spindle cells.

Cystic spaces lined by flat epithelium.

19

Adult polycystic kidney disease: Type of inheritance.

Autosomal dominant.

20

Adult polycystic kidney disease: Genes, locations, proteins (2).

PKD1, chromosome 16, polycystin 1.

PKD2, chromosome 4, polycystin 2.

21

Presentation of adult polycystic kidney disease:

A. Age of patient.
B. Laboratory abnormalities (2).

A. Fourth or fifth decade.

B. Hematuria or proteinuria.

22

Adult polycystic kidney disease: Extrarenal manifestations (4).

Berry aneurysms.

Colonic diverticula.

Cysts in pancreas, liver.

Cardiac valvular abnormalities.

23

Adult polycystic kidney disease: Gross pathology (2).

Early: Normal-sized kidneys with a few cysts.

Later: Markedly enlarged kidneys with irregular contour due to cysts.

24

Adult polycystic kidney disease: Size and location of cysts.

From a few millimeters to several centimeters in diameter.

Cortical and medullary.

25

Adult polycystic kidney disease: Histopathology (2).

Cysts: Flat or cuboidal epithelial lining.

Parenchyma: Fibrosis, atrophy.

26

Adult polycystic kidney disease: Complications (2).

End-stage renal disease.

Renal-cell carcinoma.

27

Acquired cystic disease: Presentation (2).

Usually asymptomatic.

Cysts develop in the setting of chronic renal failure, usually dialysis dependent.

28

Acquired cystic disease:

A. Inheritance.
B. Extrarenal manifestations.

A,B. None.

29

Acquired cystic disease:

A. Gross pathology.
B. Size and location of cysts.

A. Kidneys are moderately enlarged at the most.

B. Variable as in ADPKD; cortical.