Pathoma Chapter 12 Exam Study
Guide
Latest Updated 2024/2025
What is the most common congenital renal abnormality? -
ansHorseshoe kidney
Conjoined kidneys usually connected at lower poles -
ansHorseshoe kidney
Horseshoe kidney - ansConjoined kidneys usually connected at
lower poles
Where are kidneys located in horseshoe kidney? - ansLower
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? - ansPelvis
Absent kidney formation - ansRenal agenesis
Is renal agenesis unilateral or bilateral? - ansCan be either;
Risk of unilateral renal agenesis - ansRenal failure later in life
due to hyperfiltration of hypertrophied kidney
How does body compensate for unilateral renal agenesis -
ansHypertrophy of the one kidney that is present
What does bilateral renal agenesis cause? - ansOligohydramnios
with lung hypoplasia (bc there's not enough amniotic fluid), flat
face with low set ears, and developmental defects of extremities
-- AKA potter sequence
Is bilateral renal agenesis compatible with life? - ansNo
Potter Sequence - ansPulmonary hypoplasia, flat face with low
set ears, developmental defects of the face (due to
oligohydramnios!)
Is dyplastic kidney inherited or noninherited? - ansNoninherited
,Pathoma Chapter 12 Exam Study
Guide
Latest Updated 2024/2025
Noninherited, congenital malformation of renal parenchyma
characterized by cysts and abnormal tissue (like cartilage) -
ansDyplastic kidney
Where is the problem in dyplastic kidney - ansRenal
parenchyma; cysts and abnormal tissue (cartilage)
Is dyplastic kidney usually unilateral or bilateral? - ansUsually
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 - ansPolycystic kidney disease
(PKD)
Is PDK inherited or no? - ansInherited (in contrast to dysplastic
kidney)
What are the two forms of PKD? - ansAR that presents in
infants; AD that presents in young adults
How does AR PKD present? - ansIn 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? - ansAR PKD
How does AD PKD present? - ansYoung adults has
hypertension (from increase renin), hematuria, worsening renal
failure
, Pathoma Chapter 12 Exam Study
Guide
Latest Updated 2024/2025
What is the mutation in AD PKD? - ansAPKD1 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? - ansCysts develop
over time
What is a common cause of death in those with AD PKD -
ansBerry aneurysm --> Occurs in the circle of willis
What three things is AD PKD associated with? - ansBerry
aneurysm, hepatic cysts, mitral valve prolapse
Inherited AD defect leading to cysts in medullary collecting
ducts of kidney - ansMedullary cystic kidney disease
What does parenchymal fibrosis of medullary cystic kidney
disease cause? - ansShrunken kidneys and worsening renal
failure
Kidneys in PKD vs. medullary cystic kidney disease - ansPKD:
enlarged
Medullary cystic kidney disease: shrunken
Acute, severe decrease in renal function that develops in days -
ansAcute renal failure
What is the hallmark of acute renal failure? - ansAzotemia
(increased BUN and creatinine) often with oliguria
What is azotemia and how is it measured? - ansIncrease in
nitrogenous waste products in the blood; measured with blood
BUN and Cr concentrations
What are the three categories of acute renal failure based on
etiology? - ansPre-renal, post-renal, intrarenal azotemia
Guide
Latest Updated 2024/2025
What is the most common congenital renal abnormality? -
ansHorseshoe kidney
Conjoined kidneys usually connected at lower poles -
ansHorseshoe kidney
Horseshoe kidney - ansConjoined kidneys usually connected at
lower poles
Where are kidneys located in horseshoe kidney? - ansLower
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? - ansPelvis
Absent kidney formation - ansRenal agenesis
Is renal agenesis unilateral or bilateral? - ansCan be either;
Risk of unilateral renal agenesis - ansRenal failure later in life
due to hyperfiltration of hypertrophied kidney
How does body compensate for unilateral renal agenesis -
ansHypertrophy of the one kidney that is present
What does bilateral renal agenesis cause? - ansOligohydramnios
with lung hypoplasia (bc there's not enough amniotic fluid), flat
face with low set ears, and developmental defects of extremities
-- AKA potter sequence
Is bilateral renal agenesis compatible with life? - ansNo
Potter Sequence - ansPulmonary hypoplasia, flat face with low
set ears, developmental defects of the face (due to
oligohydramnios!)
Is dyplastic kidney inherited or noninherited? - ansNoninherited
,Pathoma Chapter 12 Exam Study
Guide
Latest Updated 2024/2025
Noninherited, congenital malformation of renal parenchyma
characterized by cysts and abnormal tissue (like cartilage) -
ansDyplastic kidney
Where is the problem in dyplastic kidney - ansRenal
parenchyma; cysts and abnormal tissue (cartilage)
Is dyplastic kidney usually unilateral or bilateral? - ansUsually
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 - ansPolycystic kidney disease
(PKD)
Is PDK inherited or no? - ansInherited (in contrast to dysplastic
kidney)
What are the two forms of PKD? - ansAR that presents in
infants; AD that presents in young adults
How does AR PKD present? - ansIn 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? - ansAR PKD
How does AD PKD present? - ansYoung adults has
hypertension (from increase renin), hematuria, worsening renal
failure
, Pathoma Chapter 12 Exam Study
Guide
Latest Updated 2024/2025
What is the mutation in AD PKD? - ansAPKD1 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? - ansCysts develop
over time
What is a common cause of death in those with AD PKD -
ansBerry aneurysm --> Occurs in the circle of willis
What three things is AD PKD associated with? - ansBerry
aneurysm, hepatic cysts, mitral valve prolapse
Inherited AD defect leading to cysts in medullary collecting
ducts of kidney - ansMedullary cystic kidney disease
What does parenchymal fibrosis of medullary cystic kidney
disease cause? - ansShrunken kidneys and worsening renal
failure
Kidneys in PKD vs. medullary cystic kidney disease - ansPKD:
enlarged
Medullary cystic kidney disease: shrunken
Acute, severe decrease in renal function that develops in days -
ansAcute renal failure
What is the hallmark of acute renal failure? - ansAzotemia
(increased BUN and creatinine) often with oliguria
What is azotemia and how is it measured? - ansIncrease in
nitrogenous waste products in the blood; measured with blood
BUN and Cr concentrations
What are the three categories of acute renal failure based on
etiology? - ansPre-renal, post-renal, intrarenal azotemia
