What is the most common congenital renal abnormality? Right Ans -
Horseshoe kidney
Conjoined kidneys usually connected at lower poles Right Ans - Horseshoe
kidney
Horseshoe kidney Right Ans - Conjoined kidneys usually connected at
lower poles
Where are kidneys located in horseshoe kidney? Right 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? Right Ans - Pelvis
Absent kidney formation Right Ans - Renal agenesis
Is renal agenesis unilateral or bilateral? Right Ans - Can be either;
Risk of unilateral renal agenesis Right Ans - Renal failure later in life due to
hyperfiltration of hypertrophied kidney
How does body compensate for unilateral renal agenesis Right Ans -
Hypertrophy of the one kidney that is present
What does bilateral renal agenesis cause? Right 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
Potter Sequence Right Ans - Pulmonary hypoplasia, flat face with low set
ears, developmental defects of the face (due to oligohydramnios!)
Is dyplastic kidney inherited or noninherited? Right Ans - Noninherited
,What is a Dyplastic kidney Right Ans - Noninherited, congenital
malformation of renal parenchyma characterized by cysts and abnormal
tissue (like cartilage)
Where is the problem in dyplastic kidney Right Ans - Renal parenchyma;
cysts and abnormal tissue (cartilage)
cystic space on left, cartilage on right
Is dyplastic kidney usually unilateral or bilateral? Right 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 Right Ans - Polycystic kidney disease (PKD)
Is PDK inherited or no? Right Ans - Inherited (in contrast to dysplastic
kidney)
What are the two forms of PKD? Right Ans - AR that presents in infants; AD
that presents in young adults
How does AR PKD present? Right 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?
Right Ans - AR PKD
How does AD PKD present? Right Ans - Young adults has hypertension
(from increase renin), hematuria, worsening renal failure
What is the mutation in AD PKD? Right 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? Right Ans - Cysts develop over
time
, What is a common cause of death in those with AD PKD Right Ans - Berry
aneurysm --> Occurs in the circle of willis
What three things is AD PKD associated with? Right Ans - Berry aneurysm,
hepatic cysts, mitral valve prolapse
Inherited AD defect leading to cysts in medullary collecting ducts of kidney
Right Ans - Medullary cystic kidney disease
What does parenchymal fibrosis of medullary cystic kidney disease cause?
Right Ans - Shrunken kidneys and worsening renal failure
Kidneys in PKD vs. medullary cystic kidney disease Right Ans - PKD:
enlarged
Medullary cystic kidney disease: shrunken
Acute, severe decrease in renal function that develops in days Right Ans -
Acute renal failure
What is the hallmark of acute renal failure? Right Ans - Azotemia
(increased BUN and creatinine) often with oliguria
What is azotemia and how is it measured? Right Ans - Increase 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?
Right Ans - Pre-renal, post-renal, intrarenal azotemia
What causes prerenal azotemia (ARF)? Right Ans - Decreased blood flow to
the kidneys (i.e. cardiac failure); common cause of ARF
How does prerenal azotemia affect GFR and other parameters? Right Ans -
Decrases GFR; azotemia and oliguria
What is BUN:Cr in prerenal azotemia and why? Right Ans - Over 15. RAAT
activated because not enough blood is getting to kidney; this causes
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