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    Kidney Disorders 03.27.2019

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    Emily Eresuma
    Pediatric Morning Report

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    Kidney Disorders 03.27.2019

    Uploaded by

    Emily Eresuma
    2K views33 pages
    Pediatric Morning Report

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    Pediatric Morning Report

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    2K views33 pages

    Kidney Disorders 03.27.2019

    Uploaded by

    Emily Eresuma
    Pediatric Morning Report

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    of 33

    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

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