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Morning

Report
Melanie Nelson, PGY-2
03.27.2019
2 month old ex-32 week female discharged at
6 weeks of life presents for f/u visit. Relatively
uncomplicated NICU course. Family has been
struggling with the feeding plan at home.
•PO + NG = 40 ml q3, usually takes 20 ml PO
•Neosure, 27 kcal/oz

Oral skills are not improving, uncomfortable


with swallowing, seems gassy. Has had some
projectile vomiting. Case
Parents would like to switch to Nutramigen to
see if that will help
Case
Na 140, K 6.7, Cl 111, CO2 21, BUN 21, Cr 0.27, Ca 9.9, Tot Prot 5, Alb 3.6, Tbili 0.3, Alk
Phos 469, AST 45, ALT 23, Phos 6.9

● NICU labs: intermittent hyperK (5.4-6.1)


Case
Normal swallow study,
s/p septic r/o Mild CLD, now on RA tolerating full volume
feeds enterally (NG + PO)

Since discharge, parents


Improved growth with 27 have been mixing 5
Is that right?
kcal formula scoops of Neosure with 5
oz of water
Side Note
https://m.intermountain.net/nutrition/Pages/Recipes.aspx
Case
● s/p septic r/o
● Mild CLD, now on RA
● Normal swallow study, tolerating full volume feeds enterally (NG + PO)
● Improved growth with 27 kcal formula
● Since discharge, parents have been mixing 5 scoops of Neosure with 5 oz of water
Is that right?
NO, currently mixing to ~50 kcal/oz
Correct recipe is 5 scoops of Neosure to 8 oz of water
Case
Admitted to PCH
Correctly mixed feeds continued
Received lasix x 1
Potassium levels continued to fluctuate, up to a max of 7.3, no cardiac abnormalities
Endocrinology and Nephrology were consulted
Discharged with K of 6.7
Case
F/u appointment - K 7.3
F/u appointment - K 7.3

Differential?

Case
Differential?
GI Endo
- Hyperconcentrated feeds - CAH
- Volume depletion → metabolic acidosis → - Primary adrenal insufficiency
transcellular buffering - Pseudohypoaldosteronism

Renal ID
- Rhabdomyolysis - Pyelonephritis
- Type 4 RTA
- CKD Other
- Pseudohyperkalemia
Hem/Onc - Normal K in a ex-preterm infant
- Tumor lysis
- Hemolysis
- Sickle cell
Differential?

Mismanagement
[transcellular Not enough
Too much intake
movement, lysis, excretion
etc.]
Differential?
GI Endo
- Hyperconcentrated feeds - CAH
- Volume depletion → metabolic acidosis → - Primary adrenal insufficiency
transcellular buffering
ID
Renal - Pyelonephritis
- Rhabdomyolysis
- Type 4 RTA Other
- Pseudohypoaldosteronism - Pseudohyperkalemia
- CKD - Normal K in a ex-preterm infant

Hem/Onc
- Tumor lysis
- Hemolysis
- Sickle cell
Aldosterone
problems and other
kidney weirdness
RAAS
Aldosterone
Aldosterone Problems
● Decreased production
○ CAH
○ Primary adrenal
insufficiency
○ Hypoaldosteronism
● Resistance
○ Pseudohyopaldosteronism
Type 1
● Disruption of the gradient
○ Pseudohypoaldosteronism
Type 2
Pseudohypoaldosteronism

Two very different


Poorly named and unrelated
disorders
Pseudohypoaldosteronism
Type 1
● Aldosterone resistance
○ Na wasting
○ Decreased K excretion
○ Hypovolemia
● Treatment:
○ High Na diet
Pseudohypoaldosteronism
Type 2
● WNK mutation → overactive thiazide-sensitive Na-Cl transporter in the DCT
○ Increased Na/Cl reabsorption
■ Decreased electrical gradient
○ Volume expansion, decreased RAAS
■ Decreased aldosterone
○ Decreased filtrate volume
■ Decreased K excretion
● Treatment:
○ Hydrochlorothiazide
Renal Tubular Acidosis
Renal tubular dysfunction Non-anion gap
→ Acidosis (hyperchloremic)
• Abnormal retention of H+
• Abnormal excretion of
HCO3
Bicarbonate 90%
● Filtered at the glomerulus
● Re-absorbed 10%
○ PCT - 90%, via Na-H exchange
○ DCT - 10%, via H-ATPase and H-K
ATPase
Hydrogen Ions
● Must be excreted
○ Primarily in collecting tubule
● Require buffering
○ Ammonia
○ Phosphate

100%
RTA - Types
Distal RTA

Proximal RTA

Voltage Dependent RTA

Hypoaldosteronism
Type 1 RTA
● Blocked secretion of H+ via H-ATPase
and H-K-ATPase pump
○ Hypokalemia
○ Relatively alkaline urine (>5.5)
○ Ca-containing kidney stones
● Associated with
○ Sjogren’s syndrome
○ Amphotericin B
● Treatment
○ Alkali therapy (NaHCO3 or
NaCitrate)
Type 2 RTA
● Blocked reabsorption of HCO3
○ Many different enzymes and transporters
○ Often also lose phosphate, uric acid,
glucose, amino acids, others
○ Hypokalemia
○ Fanconi syndrome = global PCT dysfunction
caused by cystinosis, tyrosinemia, glycogen
storage disease
● Treatment
○ Alkali therapy (NaHCO3 or NaCitrate)
Voltage-Dependent RTA
● Decreased distal Na delivery
OR defective Na transport
○ Reduced electrical
gradient
○ Reduced potassium
excretion → Hyperkalemia
○ HyperK limits NH4
formation → acidosis
● Treatment: Improve Na
delivery
Voltage-Dependent RTA
● Decreased distal Na delivery
OR defective Na transport
○ Reduced electrical
gradient
○ Reduced potassium
excretion → Hyperkalemia
○ HyperK limits NH4
formation → acidosis
● Treatment: Improve Na
delivery
Type 4 RTA
● Hypoaldosteronism
○ Hyperkalemia
○ Acidemia 2/2 reduced H+
pump activity
● Causes
○ Any drug that interferes
with RAAS
○ Primary isolated hypoaldo
(present like CAH)
● Treatment
○ Fludrocortisone
Aldosterone
problems and other
kidney weirdness
How to tell the difference
HyperK: Voltage-dependent vs HypoK: Type 1 vs Type 2
hypoaldo vs pseudohypoaldo
● Urine pH
● Urine pH ○ <5.3 → appropriately acidic
○ >5.5 → voltage-dependent RTA urine, not an RTA
○ <5.5 → hypoaldo of some ○ >5.5 → Maybe an RTA
variety (probably distal)
● Plasma Renin ● Fractional excretion of HCO3
○ High → hypoaldo ○ >15% → proximal RTA
○ Low/normal → ● Urine anion gap (estimates
pseudohypoaldo urine ammonium)
● Aldosterone ○ >0 → Distal RTA
○ High → pseudohypoaldo I
○ Low/normal →
pseudohypoaldo II
Case
Plasma renin: 1.1 ng/mL/hr (normal)
Aldosterone: 42.8 ng/kL (normal)
Aldosterone/Renin ratio: 38.9 (HIGH)
Cortisol: 14.1 mcg/dL (normal)
ACTH: 29 pg/mL (normal)
Case
Normal Na, cortisol, and blood pressure (not CAH or adrenal insufficiency)

Mild acidosis (less likely to be RTA)

Normal aldosterone level (not pseudohypoaldo type 1)

So.... Pseudohypoaldosteronism Type 2.


UpToDate
Yaxley J, Pirrone C. Review of the diagnostic
Sources evaluation of renal tubular acidosis. Ochsner J.
2016;16:525–530