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Pathoma: Renal- Questions and Answers
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Pathoma: Renal- Questions and Answers
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Pathoma: Renal- Questions and AnswersWhat are the two forms of PKD? Correct Ans-AR that presents in infants; AD that presents
in young adults
How does AR PKD present? Correct Ans-In infants as worsening renal failure and
hypertension; may present with Potter Sequence due to oligohydramnios. It is associated with
congenital hepatic fibrosis, which can lead to portal hypertension and hepatic cysts
What disease do you think about in an infant with portal hypertension? Correct Ans-AR
PKD
How does AD PKD present? Correct Ans-Young adults has hypertension (from increase
renin), hematuria, worsening renal failure
What is the mutation in AD PKD? Correct Ans-APKD1 or APKD2 genes (adult polycystic
kidney disease) [APKD1 is most common and is present in ~85% of cases]
Why does AD PKD present in adulthood? Correct Ans-Cysts develop over time
What is a common cause of death in those with AD PKD Correct Ans-Berry aneurysm -->
Occurs in the circle of willis
, Pathoma: Renal- Questions and Answers
What three things is AD PKD associated with? Correct Ans-Berry aneurysm, hepatic cysts,
mitral valve prolapse
Inherited AD defect leading to cysts in medullary collecting ducts of kidney Correct Ans-
Medullary cystic kidney disease
What does parenchymal fibrosis of medullary cystic kidney disease cause? Correct Ans-
Shrunken kidneys and worsening renal failure
Kidneys in PKD vs. medullary cystic kidney disease Correct Ans-PKD: enlarged
Medullary cystic kidney disease: shrunken
Acute, severe decrease in renal function that develops in days Correct Ans-Acute renal
failure
What is the hallmark of acute renal failure? Correct Ans-Azotemia (increased BUN and
creatinine) often with oliguria
What is the most common congenital renal abnormality? Correct Ans-Horseshoe kidney
Conjoined kidneys usually connected at lower poles Correct Ans-Horseshoe kidney
, Pathoma: Renal- Questions and Answers
Horseshoe kidney Correct Ans-Conjoined kidneys usually connected at lower poles
Where are kidneys located in horseshoe kidney? Correct Ans-Lower abdomen, because the
horseshoe gets caught on inferior mesenteric artery root as it ascends from pelvis to
abdomen
Where does the kidney develop in embryogenesis? Correct Ans-Pelvis
Absent kidney formation Correct Ans-Renal agenesis
Is renal agenesis unilateral or bilateral? Correct Ans-Can be either;
Risk of unilateral renal agenesis Correct Ans-Renal failure later in life due to hyperfiltration
of hypertrophied kidney
How does body compensate for unilateral renal agenesis Correct Ans-Hypertrophy of the
one kidney that is present
What does bilateral renal agenesis cause? Correct Ans-Oligohydramnios with lung
hypoplasia (bc there's not enough amniotic fluid), flat face with low set ears, and
developmental defects of extremities -- AKA potter sequence
, Pathoma: Renal- Questions and Answers
Potter Sequence Correct Ans-Pulmonary hypoplasia, flat face with low set ears,
developmental defects of the face (due to oligohydramnios!)
Is dyplastic kidney inherited or noninherited? Correct Ans-Noninherited
What is a Dyplastic kidney Correct Ans-Noninherited, congenital malformation of renal
parenchyma characterized by cysts and abnormal tissue (like cartilage)
Where is the problem in dyplastic kidney Correct Ans-Renal parenchyma; cysts and
abnormal tissue (cartilage)
cystic space on left, cartilage on right
Is dyplastic kidney usually unilateral or bilateral? Correct Ans-Usually unilateral, but on
exams will be bilateral and will need to differentiated from PKD
Inherited defect leading to bilateral enlarged kidneys with cysts in the renal cortex and
medulla Correct Ans-Polycystic kidney disease (PKD)
Is PDK inherited or no? Correct Ans-Inherited (in contrast to dysplastic kidney)
What is azotemia and how is it measured? Correct Ans-Increase in nitrogenous waste
products in the blood; measured with blood BUN and Cr concentrations
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