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50 Cards in this Set

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  • Back

Why does renal agenesis happen?

ureteric bud fails to develop, eliminating induction of the metanephric vesicles and nephron formation

what are the two type of renal agenesis?

unilateral, bilateral

Which type of agenesis is more common in males?

unilateral

in unilateral renal agenesis, which kidney is usually absent?

L

what happens to the other kidney that is present in unilateral renal agenesis?

it becomes hypertrophic over making it compatible with life

overall, bilateral renal agenesis causes what?

oligohydraminos

with bilateral renal agenesis, what does that cause and result with?

potter sequence


deformed limbs, wrinkly skin, annl facial appearance

what is the congenital small kidney with no evidence of dyplasia

renal hypoplasia

what is the disorganization of renal parenchyma with abnormal developed immature nephrons

renal dysplasia

this is when one or both of the kidney fail to ascend and remain in the pelvis or lower lumbar area

ectopic kidney

what is the most common renal fusion?

horseshoe kidney

what causes a horseshoe kidney?

inferior poles of the kidney fused across the midline b/c they got caught behind the root of the IMA

are horseshoe kidneys symptomatic?

no

Which way do the hilum face in a horseshoe kidney?

ventrally

This anomaly is caused by the obstruction to urine flow from the renal pelvis to proximal ureter

ureteropelvic junction obstruction

What is the most common congenital obstruction of urinary tract?

ureteropelvic junction obstruction

what happens with UPJ and severe ureteropelvic atresia? and where is it found?

multicystic dysplastic kidney




dilated calyces

What does the multi cystic dysplastic kidney show as?

grapelike smooth walled cysts, dysplastic glomeruli and atrophic tubules found between cysts

Which urinary anomaly is autosomal recessive?

childhood polycystic kidney disease

what are the characteristics of the childhood polycystic kidney disease?

huge, spongy, dilation of the collecting ducts, with multiple cysts

child polycystic kidney disease is clinically associated with?

cysts of liver, pancreas and lungs

What is the tx for PCKD?

dialysis and kidney transplant

what is the most common renal malignancy of childhood

wilms tumor

This is the large, solitary, well-circumsicirbed mass on the cut sections is soft, homogenous, and tan-grey in color

wilms tumor

What are the 3 different histological areas of wilms tumor

stromal area, blastemal area or tightly packed embryonic cells, and tubular area

why does a ureteropelvic duplications happen?

ureteric bud prematurely divides before penetrating with the metanephric blastema

what does ureteropelvic duplications result in??

double kidney, duplicated ureter, renal pelvis

how can you Dx ureteropelvic duplication?

IVP, US, CT, MRI, excretory urogram

With an ectopic ureter in males, where does the ureter enter?

into neck of bladder or into the prostatic part of urethra

With an ectopic ureter in females, where does the ureter enter?

into the neck of bladder, urethra, vagina, or vestibule--> causes incontinence

This is where the posterior part of the bladder is exposed to there exterior?

exstrophy of bladder

Why does the exstrophy of the bladder happen?

failure of the anterior and wall and the anterior wall of the bladder to develop properly

Exstrophy of the bladder is clinically associated with?

urine drainage

this is where the cysts originate from the remnants of epithelial of the urachus?

urachal cysts

This is due to persistent inferior end of arches that dilates and opens into bladder?

urachal sinus

This is where the entire arches remains patent, allowing urine to escape from the umbilical orifice?

urachal fistula

this is where the distal end of the ureter has cyst like protrusion into submucosal layer of the urinary bladder

simple ureterocele

this is the distal end of the ureter has a cyst like protrusion into submucosal layer of the urinary bladder that is associated with an ectopic ureter and duplication

ectopic ureterocele

This is the most common extracranial neoplasm containing primitive neuroblasts or neurocrest origin?

neuroblastoma

neuroblastomas metastasize into?

bone marrow, bone, lymph nodes

labs findings of neuroblastomas?

VMA and elevated metanephric levels

clinically what is pheochromocytoma associated with?

paroxysmal HTN, anx, tremors, CP, sweating, and abdominal pain

lab findings of pehocromocytoma?

VMA, elevated metanephric, inability to suppress catechtoamines and hyperglycemia

this is a large urinary bladder due to the disorder with the metanephric diverticulum and dilation of the renal pelvis

congenital megacystis

what does congenital megacystis result in?

renal failure and pulmonary hypoplasia

this is because of the mutations in genes for enzymes involved in adrenocortical steroid biosynthesis

congenital adrenal hyperplasia

What does congenital adrenal hyperplasia result in?

excessive androgen production by suprarenal cortex




causes masculinization in females and mild clitoral enlargement

what does congenital adrenal hyperplasia present with?

hyponatremia, dehydration, hyperkalemia

tx of congenital adrenal hyperplasia

IV saline, steroid hormone replacement

is pheochromocytoma in adults or children?

adults