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Unit 5: Path - The Kidney > Congenital Anomalies > Flashcards

Congenital Anomalies Flashcards

1
Q

What is Potter Syndrome?

A

Pathologic abnormalities caused by markedly reduced intrauterine urine production

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2
Q

What is the most life threatening component of Potter sequence?

A

Pulmonary Hypoplasia

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3
Q

What is the mechanism by which the Potter Sequence results in congenital abnormalities?

A

Lower urine output -> less amniotic fluid -> compression of fetus -> abnormalities

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4
Q

What is renal agenesis?

A

Complete absence of renal tissue

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5
Q

What is the term used to describe histologically normal kidneys with six or fewer renal lobes?

A

Renal Hypoplasia

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6
Q

What is renal ectopia? Difference betwixt simple and crossed?

A

Normal kidney in an abnormal location; Simple - Ureters drain into appropriate side of bladder; Crossed - ectopic ureter crosses the midline and drains into the contralateral side of the bladder

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7
Q

What is your Dx?

A

Horseshoe kidney

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8
Q

What is Renal Dysplasia?

A

Developmental disorder charac. by undifferentiated tubular structures surrounded by primitive mesenchyme

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9
Q

Renal dysplasia is shown. What are the histological hallmarks?

A

Undifferentiated tubules and ducts lined by cuboidal or columnar epithelium

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10
Q

What is the most common cause of an abdominal mass in newborns? Tx?

A

Multicystic renal dysplasia; Surgery Multicystic Renal Dysplasia

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11
Q

What is the most common congenital disease characterized by numerous cysts in the renal parenchyma?

A

Autosomal Dominant Polycystic Kidney Disease Adult Polycystic Disease

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12
Q

What are the genes commonly affected in ADPKD?

A

PKD1 and PKD2

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13
Q

What condition do 1/3 of patients with ADPKD also have?

A

Hepatic cysts

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14
Q

At what age does ADPKD generally manifest?

A

4th decade

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15
Q

What is Autosomal Recessive Polycystic Kidney Disease characterized by?

A

Cystic transformation of the collecting ducts

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16
Q

What gene is affected in ARPKD? What protein is it responsible for forming?

A

PKHD1, Fibrocystin

17
Q

What other condition usually manifests in a person with ARPKD?

A

Congential Hepatic Fibrosis

18
Q

A kidney of a patient with ARPKD is shown below. Describe the kidney and how it differs from a person with ADPDK.

A

Dilated cortical and medullary collecting ducts arranged radially; The external surface is SMOOTH

19
Q

What gene is commonly mutated in Glomerulocystic Disease? What is characteristic of this condition?

A

Hepatocyte Nuclear Factor-1Beta; Dilation of Bowman’s Capsule

20
Q

What are the genes mutated that can cause Nephronophthisis? Medullary Cystic Disease?

A

NPHP1-9; MCKD1-2

21
Q

A patient presents with flank pain, dysuria, and “gravel” in the urine. Cysts are found in the renal papillae. What is the Dx?

A

Medullary Sponge Kidney

Unit 5: Path - The Kidney (11 decks)

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