congenital kidney

Question 1

horseshoe kidney description

Answer 1

kidneys that are connected at the lower pole. this is the most common renal malformation.

Question 2

where is the horseshoe kidney located?

Answer 2

in the abdomen due to hanging up on the IMA.

Question 3

renal agenesis description

Answer 3

absent kidney formation.

Question 4

is renal agenesis unilateral or bilateral?

Answer 4

can be either. (bilateral is incompatible with life.

Question 5

unilateral renal agenesis

Answer 5

leads to hypertrophy of the existing kidney. this hyper filtration leads to increased risk of renal failure later in life,

Question 6

bilateral renal agenesis

Answer 6

leads to oligohydraminos with lung hypoplasia. the fetus will have a flat face and low ears. there will also be developmental abnormalities of the extremities

Question 7

what is potter sequence?

Answer 7

flat face. low ears, extremities malformed, oligohydraminos and lung hypoplasia.

Question 8

is dysplastic kidney inherited?

Answer 8

no.

Question 9

dysplastic kidney

Answer 9

congenital malformation of the kidney parenchyma characterized by cysts and abnormal tissue.

Question 10

is dysplastic kidney unilateral or bilateral?

Answer 10

usually unilateral, but can be bilateral and needs to be distinguished from PCK

Question 11

polycystic kidney disease description

Answer 11

inherited disorder that causes bilateral enlargement of the of the kidneys with cysts in the medulla and cortex. there are two types: autosomal recessive and dominant

Question 12

autosomal recessive polycystic kidney disease

Answer 12

this occurs in children, and causes worsening renal failure and HTN. newborns present with potters sequence.

Question 13

what is associated with autosomal recessive polycystic kidney disease

Answer 13

neonatal hepatic fibrosis leading to portal HTN. also hepatic cysts.

Question 14

autosomal dominant polycystic kidney disease

Answer 14

this occurs in adults and presents as HTN (due to renin), hematuria, and worsening renal failure. the cysts develop over time.

Question 15

autosomal dominant polycystic kidney disease mutations

Answer 15

APKD1, 2.

Question 16

autosomal dominant polycystic kidney disease associations

Answer 16

berry aneurysm, hepatic cysts, and mitral valve prolapse.

Question 17

medullary cystic disease description

Answer 17

inherited autosomal dominant, leads to cysts in the medulla collecting ducts. parenchymal fibrosis results in shrunken kidney and worsening renal failure,

Question 18

renal dysplasia key words

Answer 18

primitive mantles of undiff mesenchyme surrounding undifferentiated tubules and ducts lined with cuboidal or columnar epithelium. typically contains some ectopic tissue islands of CARTILAGE. tubules form cysts. highly associated with other abnormalities that cause renal obstruction. palpable flank mass of the newborn

Question 19

key words for autosomal recessive polycystic kidney disease

Answer 19

occurs in infants, transformation of the collecting ducts. RARE. 25% die in neonatal due to pulmonary hypoplasia survivors will have the triad of portal hypertension, hepatic fibrosis, and renal insufficiency. grossly will have dilated medullary and cortical collecting ducts. PKHD1 gene involved. fibrocytin gene. incredibly large kidneys, bilaterally.
the dilated ducts are radially oriented and perpendicular to the capsule.