Pediatric Nephrology

Question 1

3 stages of development of the kidney

Answer 1

Pronephros (nonfunctional), Mesonephros, Metanephros

Question 2

True/ False: Kidney produces amniotic fluid at a rate up to 1 ml/kg/h.

Answer 2

False: 10 ml.kg/h

Question 3

At what weight (kg) do you get adult like composition of fluids

Answer 3

15 kg- Water content decreases from 96% at EGA 8 weeks to 78% at term

Question 4

In a preterm baby, you have fewer production of glomeruli and because of the diluting and concentration capacity, baby is at risk for…

Answer 4

hyponatremia

Question 5

No cows milk up until 1 y/o because limited ability to excrete a high solute load: why is this

Answer 5

• Low GFR
• Shorter loops of Henle
• Increased medullary/cortical blood flow ratios

Question 6

One of the most common pediatric infections.

Answer 6

UTI

Question 7

Age 0-3 mos.: Boy/Girls have more UTIs

Answer 7

Boys: After 1 yr. girls more likely

Question 8

Most UTIs in the first year are Pyelonephritis/Cyctitis

Answer 8

Pyelonephritis (upper tract)

Question 9

WHAT REDUCES UTI incidence in boys in 1st year of life

Answer 9

Circumcision

Question 10

AAP: Infant with fever without focus. What do you obtain

Answer 10

Urine specimen by catheterization for both culture and UA. Treat constipation

Question 11

T/F: Grade III or less VUR may resolve without intervention

Answer 11

True

Question 12

What is hypoplasia, what is it proportional to, and what do you get?

Answer 12

Small kidneys, decreased glomeruli.

• Directly proportional to BW.
• Oligomeganephronia- Hyperfiltration injury

Question 13

Dysplasia:

Answer 13

Maturational arrest
-Abnormal histology- primitive tubules and cartilage
Ex: Multicystic dysplastic kidney, obstructive dysplasia

Question 14

ARPKD: Autosomal recessive, infantile form

Answer 14

Fibrocystin, Chromosome 6

• Hepatic fibrosis w/ portal HTN
• Systemic HTN
• Kidney Failure
• Respiratory distress

Question 15

ARPKD: Autosomal Dominant, more frequent in adults

Answer 15

Polycystin

• Bilateral macrocysts
• Hepatic, splenic, and pancreatic cysts
• Aneurysms of Circle of Willis

Question 16

Water excretion in the fetal and neonatal kidney

Answer 16

• Diminished capacity to rapidly excrete water.
• Lower GFR
• Elevated hormone levels suggest decreased ability of the neonatal kidney to respond to renin, aldosterone, atrial natriuretic peptide, and antidiuretic hormone.

Question 17

Increased renal mass after glomerular development is complete is due to tubular growth and maturation. So this means Preterm GFR is greater than what?

Answer 17

Tubular absorption- Fractional excretion of Na+ is greater in preterm- Hyponatremia

Question 18

Bicarbonate reabsorption threshold is directly related to ____ and most bicarbonate is reabsorbed in the ____.

Answer 18

Age, Proximal tubule- Same with glucose!

Question 19

Glucosuria can increase osmotic diuresis, risk for _________

Answer 19

Dehydration

Question 20

1) Phosphorous reabsorption is _________ in infants.

2) ___________ related to age and GFR.

Answer 20

Higher, Inversely

Question 21

Chronic kidney disease stages

Answer 21

I: GFR>90
II: GFR 60-90
III: GFR 30-60
IV: GFR 15-30
V: GFR <15

Question 22

What is this: Many AR mutations, retinal degeneration, hepatic fibrosis, Brain and skeletal abnormalities.

Answer 22

Juvenile Nephronophthisis

Question 23

What is this: AD, typically presents later. HTN. Polyuria, hematuria in adults.

Answer 23

Medullary cystic disease

Question 24

Congenital nephrotic syndrome presents in the first ___ months. Fetal _____________ —-> elevated AFP. Placenta >25% birth weight. It is resistant to __________ treatment. Leads to end stage renal disease so need a __________. Abnormality in making ________.

Answer 24

1) 3 months
2) Proteinuria
3) Steroid
4) Transplant
5) Nephrin

Question 25

____________ nephrotic syndrome presents in the first 12 months. It is resistant to steroid treatment. ESRD, Transplant. An abnormality in _________.

Answer 25

Infantile, podocin

Question 26

A form on infantile nephrotic syndrome that presents with HTN, gonadoblastoma, wilms tumor, and male pseudohermaphroditism.

Answer 26

Denys-Drash

Question 27

Nephrotic Cystinosis: Renal fanconi syndrome

Answer 27

Proximal tuble: glucosuria, amino aciduria, phosphaturia, proximal RTA, secondary hypercalciuria.
-AR- prototypic lysosomal membrane transport disease: cystine accumulates in lysosomes, causing crystal formation and cell death. Untreated leads to ESKD, CORNEAL crystals, hypothyroidism, and growth retardation.
Rx: Cysteamine

Question 28

Basement membrane disease when it is less than 100nm in thickness

Answer 28

Thin basement membrane lesion. Normal is 100-300nm

Question 29

Abnormally split and laminated GBM

Answer 29

Alport syndrome- Problem with Collagen IV

Question 30

Posterior urethral valves

Answer 30

Obstructive Uropathy- Dilated ureters and prostatic urethra- Treat with catheter

Question 31

Early obstructive uropathy

Answer 31

10-12 weeks

• Failure of UB branching, blastema induction
• Dysplasia

Question 32

Late obstructive uropathy

Answer 32

• VUR
• Inflammation
• Fibrosis

Question 33

Triad of Prune belly syndrome (aka Eagle-Barrett sundrome)

Answer 33

1) Deficient abdominal musculature
2) Bilateral Cryptorchidism
3) Dilated nonobstructed urinary tract

Question 34

Symptoms of Prune belly syndrome

Answer 34

Dysplasia, VUR, UTIs common.

• Pectus excavatum, hip dysplasia
• 97% male

Question 35

UPJ obstruction signs and symptoms

Answer 35

1) Dilated renal pelvis
2) Flank pain with high urine flow
3) May be bilateral-lead to renal failure
4) Nephrostomy tube replacement

Question 36

A horseshoe kidney is fusion of the _______ poles and the ascent is blocked by the ______. The kidney is functional and malrotated.

Answer 36

Caudal, Inferior mesenteric artery

Question 37

What is this: Microangiopathic hemolytic anemia, thrombocytopenia, acute renal failure. What 2 types

Answer 37

Hemolytic Uremic Syndrome

1) D +HUS: Hemorrhagic colitis, shigella, EHEC,
2) Atypical: Pneumococcus, complement abnormalities