SM_217a: Pediatric Nephrology

Question 1

Prenatal ultrasound reveals that the fetus has 2 kidneys that are both measured large for gestational age and are echogenic. Most likely diagnosis is _____

Answer 1

Prenatal ultrasound reveals that the fetus has 2 kidneys that are both measured large for gestational age and are echogenic. Most likely diagnosis is ARPKD

Question 2

A 16 year old boy with no past medical history presents with clearly chronic ESRD of unknown etiology. Most likely underlying diagnosis is ______

Answer 2

A 16 year old boy with no past medical history presents with clearly chronic ESRD of unknown etiology. Most likely underlying diagnosis is renal dysplasia

Question 3

Complication of CKD unique to pediatric population is ______

Answer 3

Complication of CKD unique to pediatric population is growth impairment

Question 4

A 7 year old boy presents with brown urine, hematuria, and proteinuria and urinalysis, and hypertension. Complement level C3 is low. Most likely diagnosis is _____

Answer 4

A 7 year old boy presents with brown urine, hematuria, and proteinuria and urinalysis, and hypertension. Complement level C3 is low. Most likely diagnosis is Post-infectious Glomerulonephritis

Question 5

Cutoff for normal protein:creatinine spot ratio in urine is ____

Answer 5

Cutoff for normal protein:creatinine spot ratio in urine is 0.2

Question 6

Protein:creatine is considered to be in nephrotic range above _____

Answer 6

Protein:creatine is considered to be in nephrotic range above 2-3

Question 7

Micoscopic hematuria is defined as microscopic urinalysis with ____ RBC/hpf

Answer 7

Micoscopic hematuria is defined as microscopic urinalysis with > 5 RBCs/hpf

Question 8

Most common cause of CKD/ESKD in kids is _____ group of abnormalities followed by _____

Answer 8

Most common cause of CKD/ESKD in kids is CAKUT group of abnormalities (aplasia/hypoplasia/dysplasia, obstructive uropathy/reflux nephropathy) followed by FSGS

(NOT hypertension or diabetes)

Question 9

Describe the CAKUT abnormalities in order of decreasing frequency

Answer 9

CAKUT abnormalities

• Most common: hydronephrosis ± obstructive, vesicoureteral reflux
• Renal cysts
• Renal dysplasia ± hydronephrosis and/or cysts
• Renal agenesis

Question 10

Describe complications of CKD in kids and associated treatments

Answer 10

Complications of CKD in kids and associated treatments

• Growth failure: treat with rHGH
• Mineral and bone disorder: dietary phosphorus restriction, phosphorus binders, treatment of secondary hyperparathyroidism with activated Vitamin D
• Anemia: higher doses of rHuEPO than adults
• Hypertension: intensified BP control
• CVD: address CVD and CKD risk factors
• Cognitive and psychosocial development

Question 11

_____ is leading cause of death in pediatric CKD population

Answer 11

Cardiovascular disease is leading cause of death in pediatric CKD population

Question 12

Describe causes of UTI in kids

Answer 12

UTI in kids

• Urinary obstruction
• Vesicoureteral reflux: retrograde flow of urine flrom bladder into ureters (Grade I-V)
• Voiding dysfunction

Question 13

Painful hematuria is _____

Answer 13

Painful hematuria is urologic

Question 14

Imaging is used when patient has hematuria to rule out _____ and _____

Answer 14

Imaging is used when patient has hematuria to rule out mass (Wilm’s tumor) and nephrolithiasis

Question 15

Describe the differential diagnosis for nephrotic syndrome in kids

Answer 15

Differential diagnosis for nephrotic syndrome in kids

• Minimal change disease (most kids)
• FSGS: idiopathic, familial, HIV, hepatitis, heroim, NSAIDs, obesity, drugs
• Membranous nephropathy
• Congenital nephrotic syndrome: nephrin, Denys-Drash syndrome, others
• Familial nephrotic syndrome: podocin, alpha actinin, others

Question 16

Glomerulonephritis with low complement is most often _____

Answer 16

Glomerulonephritis with low complement is most often PIGN (low C3, C4 usually normal)

Question 17

_____, _____, and _____ are causes of glomerulonephritis with normal complement levels

Answer 17

IgA nephropathy, Henoch Schonlein purpura, and pauci-immune GN (ANCA-associated GN) are causes of glomerulonephritis with normal complement levels

Question 18

PIGN has ____ C3 level and ____ C4 level

Answer 18

PIGN has low C3 level and normal C4 level

Question 19

IgA nephropathy has _____ complement levels

Answer 19

IgA nephropathy has normal complement levels

Question 20

Henoch Schonlein Purpura has ____ complement levels, while lupus nephritis has ____ complement levels

Answer 20

Henoch Schonlein Purpura has normal complement levels, while lupus nephritis has low complement levels

Question 21

ARPKD involves _____ and _____

Answer 21

ARPKD involves congenital hepatic fibrosis and portal hypertension

Question 22

ADPKD is associated with ____

Answer 22

ADPKD is associated with intracranial aneurysms