Pediatric Nephrology Flashcards
3 stages of development of the kidney
Pronephros (nonfunctional), Mesonephros, Metanephros
True/ False: Kidney produces amniotic fluid at a rate up to 1 ml/kg/h.
False: 10 ml.kg/h
At what weight (kg) do you get adult like composition of fluids
15 kg- Water content decreases from 96% at EGA 8 weeks to 78% at term
In a preterm baby, you have fewer production of glomeruli and because of the diluting and concentration capacity, baby is at risk for…
hyponatremia
No cows milk up until 1 y/o because limited ability to excrete a high solute load: why is this
- Low GFR
- Shorter loops of Henle
- Increased medullary/cortical blood flow ratios
One of the most common pediatric infections.
UTI
Age 0-3 mos.: Boy/Girls have more UTIs
Boys: After 1 yr. girls more likely
Most UTIs in the first year are Pyelonephritis/Cyctitis
Pyelonephritis (upper tract)
WHAT REDUCES UTI incidence in boys in 1st year of life
Circumcision
AAP: Infant with fever without focus. What do you obtain
Urine specimen by catheterization for both culture and UA. Treat constipation
T/F: Grade III or less VUR may resolve without intervention
True
What is hypoplasia, what is it proportional to, and what do you get?
Small kidneys, decreased glomeruli.
- Directly proportional to BW.
- Oligomeganephronia- Hyperfiltration injury
Dysplasia:
Maturational arrest
-Abnormal histology- primitive tubules and cartilage
Ex: Multicystic dysplastic kidney, obstructive dysplasia
ARPKD: Autosomal recessive, infantile form
Fibrocystin, Chromosome 6
- Hepatic fibrosis w/ portal HTN
- Systemic HTN
- Kidney Failure
- Respiratory distress
ARPKD: Autosomal Dominant, more frequent in adults
Polycystin
- Bilateral macrocysts
- Hepatic, splenic, and pancreatic cysts
- Aneurysms of Circle of Willis
Water excretion in the fetal and neonatal kidney
- 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.
Increased renal mass after glomerular development is complete is due to tubular growth and maturation. So this means Preterm GFR is greater than what?
Tubular absorption- Fractional excretion of Na+ is greater in preterm- Hyponatremia
Bicarbonate reabsorption threshold is directly related to ____ and most bicarbonate is reabsorbed in the ____.
Age, Proximal tubule- Same with glucose!
Glucosuria can increase osmotic diuresis, risk for _________
Dehydration
1) Phosphorous reabsorption is _________ in infants.
2) ___________ related to age and GFR.
Higher, Inversely
Chronic kidney disease stages
I: GFR>90 II: GFR 60-90 III: GFR 30-60 IV: GFR 15-30 V: GFR <15
What is this: Many AR mutations, retinal degeneration, hepatic fibrosis, Brain and skeletal abnormalities.
Juvenile Nephronophthisis
What is this: AD, typically presents later. HTN. Polyuria, hematuria in adults.
Medullary cystic disease
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 ________.
1) 3 months
2) Proteinuria
3) Steroid
4) Transplant
5) Nephrin
____________ nephrotic syndrome presents in the first 12 months. It is resistant to steroid treatment. ESRD, Transplant. An abnormality in _________.
Infantile, podocin
A form on infantile nephrotic syndrome that presents with HTN, gonadoblastoma, wilms tumor, and male pseudohermaphroditism.
Denys-Drash
Nephrotic Cystinosis: Renal fanconi syndrome
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
Basement membrane disease when it is less than 100nm in thickness
Thin basement membrane lesion. Normal is 100-300nm
Abnormally split and laminated GBM
Alport syndrome- Problem with Collagen IV
Posterior urethral valves
Obstructive Uropathy- Dilated ureters and prostatic urethra- Treat with catheter
Early obstructive uropathy
10-12 weeks
- Failure of UB branching, blastema induction
- Dysplasia
Late obstructive uropathy
- VUR
- Inflammation
- Fibrosis
Triad of Prune belly syndrome (aka Eagle-Barrett sundrome)
1) Deficient abdominal musculature
2) Bilateral Cryptorchidism
3) Dilated nonobstructed urinary tract
Symptoms of Prune belly syndrome
Dysplasia, VUR, UTIs common.
- Pectus excavatum, hip dysplasia
- 97% male
UPJ obstruction signs and symptoms
1) Dilated renal pelvis
2) Flank pain with high urine flow
3) May be bilateral-lead to renal failure
4) Nephrostomy tube replacement
A horseshoe kidney is fusion of the _______ poles and the ascent is blocked by the ______. The kidney is functional and malrotated.
Caudal, Inferior mesenteric artery
What is this: Microangiopathic hemolytic anemia, thrombocytopenia, acute renal failure. What 2 types
Hemolytic Uremic Syndrome
1) D +HUS: Hemorrhagic colitis, shigella, EHEC,
2) Atypical: Pneumococcus, complement abnormalities
