Pediatric Kidney & Urinary Tract Flashcards
Define embryologic basis and clinical presentation of renal agenesis.
- incidence of unilateral renal agenesis is 1/1000. The opposite kidney hypertrophies to compensate. Left kidney more commonly affected. May be associated with single umbilical artery.
- incidence of bilateral renal agenesis is 1/3000 births. Incompatible with postnatal life.
- due to failure of metanephric diverticulum to develop or its early degeneration.
Define embryologic basis and clinical presentation of renal hypoplasia
-the under development of a kidney with contralateral compensatory hypertrophy
Define embryologic basis and clinical presentation of renal dysplasia
-abnormal metanephric tissue differentiation of the kidney tissue with cysts and heterotopic tissues such as cartilage due to pleuripotent potential of renal anlage.
Define embryologic basis and clinical presentation of horseshoe kidney
- incidence is 1/500.
- the anlage of the kidneys is fused (90% of the time at the lower pole).
- since they are linked together, they are often ectopic and fail to rotate medially.
- increased incidence of urolithiasis.
Define embryologic basis and clinical presentation of renal ectopia
- failure of the kidney to rise out of the pelvis or to rotate medially.
- may result in ureteral obstruction.
- kidneys may be discoid in shape.
What is the most common renal lesion?
Simple cysts, they’re usually asymptomatic.
T or F?
In medullary sponge kidney, there are at least 2 enlarged and pale renal pyramid and is usually unilateral
False
Only needs to be at least one large pale pyramid, and it’s typically bilateral (70%)
Bob is a 65 year old man on dialysis. He has multiple etiologies that have led to his ESRD. He used to be asymptomatic but now he complains of gross hematuria, flank pain, renal colic, and has a palpable mass. What does Bob most likely have?
Acquired renal cystic disease. He pretty much covers the main demographic.
Joshua is a nice man. He is 45 and comes in complaining of flank pain and intermittent hematuria. You do a CT scan and notice bilaterally large kidneys that look like they weigh 5kg. On further examination, you find that he has cysts on the liver and pancreas, mitral valve prolapse, and diverticulosis. He also complains of headaches, which you conclude to be brain aneurysms. What disease does he have and with what proteins is it associated?
He has AD PKD (Autosomal dominant polycystic kidney disease). This is associated with the polycystin proteins 1 and 2. Look at the other side, all of the bolded items are the buzzwords.
Shaneequa gives birth to her daughter who she names Hunnet Dollabill. Poor Hunnet isn’t doing too well. Upon examination that her kidneys are 10-20 times their expected size. Her liver is also enlarged, and she goes on to develop pretty severe hypertension. As she grows, she experienced growth retardation. She also has periportal fibrosis of the liver. Shaneequa demands a diagnosis, and you tell her it is ________ which is associated with the ________ protein.
Autosomal Recessive Polycystic Kidney Disease.
Associated with the fibrocystin protein.
This disease is the most common cause of ESRD in the first 2 decades of life. Its histological findings are cuboidal epithelium with a thickened basement membrane. It presents with bilateral cortical atrophy with a thickened surface. What is it?
NMCD (Nephronophthisis-medullary cystic kidney disease complex).
A patient comes in having developed bilateral cysts of the kidney that are filled with glycogen rich cells. They also have retinal and cerebellar hemangioblastomas, and you suspect pheochromocytoma and renal cell carcinoma as well. Their poor epididymis has a cyst too, which is why the patient came in complaining of poor sexual performance. What do you think they have?
Von Hippel Lindau,
VHL 3p25
Nothing original here. Give general characteristics of Tuberous Sclerosis.
- phenotype includes facial nevi, cardiac rhabdomyomas, epilepsy, angiofibromas, and mental retardation and multiple renal angiomyolipomas although diffuse renal cystic disease is rare.
- renal cysts are present in 20-25% of these patients.
- cysts vary in size from several millimeters to 3 cm lined by large eosinophilic cells with enlarged, hyperchromatic nuclei.
What’s the most common cystic malformation of the kidney in infancy?
Hint: It’s ALWAYS associated with uteretal or uteropelvix atresia (failure of something to close)
Double hint: It looks like grapes filled with clear or yellow liquid.
Multicystic Dysplasia of the Kidney (MCDK)
-MCDK results from an abnormal induction of the metanephric blastema by the ureteral bud. This abnormal induction might be due to a problem with the formation of the mesonephric duct, malformation of the ureteral bud, or degeneration of the ureteral bud at an early stage.
A baby comes in to your clinic with a note from the OB/GYN that saw his mother saying that he noticed a “ring sign” on imaging during the pregnancy. On your imaging of the child you notice solitary firm round infiltrating fibrous mass composed of bland spindle cells around the kidney. You say to the mother, “your child has the most common kidney tumor at birth to 6 months of age, called _________” Fortunately it’s generally benign if completely resected.
Congenital Mesoblastic Nephroma
