Three Children with Electrolyte Problems
Post on 20-Jan-2016
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Three Children with Electrolyte Problems
by
Larry Greenbaum, MD, PhDPediatric Nephrology
by
Larry Greenbaum, MD, PhDPediatric Nephrology
Patient One
Two year old with failure to thrive Polyuria and polydipsia Blood pressure of 160/90
147
1.8
106
32
8
0.4
Patient Two
One week old brought to the hospital for lethargy, poor feeding and dehydration
124
7.7
90
12
30
1.5
(Bun and creatinine normalized after hydration)
Patient Three
Four month old with fever and dehydration
Failure to thrive and decreased tone
120
2.6
59
41
18
0.6
Do you enjoy renal physiology?
Yes
No
10%
90%
95%
5%
Distal tubule
Na+
K+
Na+
K+
K+
H+
Na+
K+
Na+
K+
K+
Na+
Na+
Na+
Na+
H+
Na+
Na+
Na+
Na+
-
-
-
-
AdrenalGlandKidney
Aldosterone
Renin ATII
Volume Depletion
Regulation of Aldosterone
Three Possible Problems Excess aldosterone effect
Hypertension Hypokalemia and metabolic alkalosis
Absence of aldosterone effect Hypotension Hyperkalemia, metabolic acidosis and
hyponatremia Physiologic aldosterone overproduction
Volume depletion Hypokalemia and metabolic alkalosis
Excess Aldosterone Effect
AdrenalGlandKidney
Aldosterone
Adrenal adenoma
Na+
K+
Na+
K+
K+
H+
Na+
K+
Na+
K+
K+
Na+
Na+
Na+
Na+
H+
-
-
-
-
Absence of Aldosterone Effect
AdrenalGlandKidney
Renin ATII
Volume Depletion
21-Hydroxylase deficiency(CAH)
17-Hydroxypreg.
17-hydroxyprog.
11-deoxycortisol
Cortisol
Pregnenolone
Progesterone
DOC
Corticosterone
18-Hydroxycorticosterone
Aldosterone
DHEA
Androstendione
Testosterone
GlucocorticoidsMineralocorticoids Androgens
Na+
K+
Na+
K+
K+
H+
Na+
K+
Na+
K+
K+
H+
Na+
Na+
Na+
Na+
Physiologic Aldosterone Overproduction
AdrenalGlandKidney
Aldosterone
Renin ATII
Volume Depletion
Loop Diuretic
Distal tubule
Na+
K+
Na+
K+
K+
H+
Na+
K+
Na+
K+
K+
Na+
Na+
Na+
Na+
H+
-
-
-
-
Patient One
Two year old with failure to thrive
Polyuria and polydipsia
Blood pressure of 160/90
147
1.8
106
32
8
0.4
Case One Diagnosis
Excess aldosterone effect
Absence of aldosterone effect
Physiologic aldosterone overproduction
Voting
85%
10%
5%
Na+
K+
Na+
K+
K+
H+
Na+
K+
Na+
K+
K+
Na+
Na+
Na+
Na+
H+
-
-
-
-
Liddle Syndrome
Severe hypertension Hypokalemia and metabolic alkalosis Polyuria, polydipsia and muscle
weakness Low aldosterone and renin levels Autosomal dominant Blood pressure does not improve with
Aldactone but does improve with triamterene or amiloride
Na+
K+
Na+
K+
K+
H+
Na+
K+
Na+
K+
K+
Na+
Na+
Na+
Na+
H+
-
-
-
-
AA
A
A
TT
TT
Patient Two
One week old brought to the hospital for lethargy, poor feeding and dehydration
124
7.7
90
12
30
1.5
(Bun and creatinine normalized after hydration)
Case Two Diagnosis
Excess aldosterone effect
Absence of aldosterone effect
Physiologic aldosterone overproduction
Voting
5%
90%
5%
Na+
K+
Na+
K+
K+
H+
Na+
K+
Na+
K+
K+
H+
Na+
Na+
Na+
Na+
Pseudohypoaldosteronism Type I
Dehydration and failure to thrive as neonates
Hyponatremia, hyperkalemia and metabolic acidosis
Elevated plasma renin and aldosterone Aldosterone resistance in kidney, sweat
and salivary glands, colonic mucosa Autosomal recessive Treatment with NaCl and Kayexalate
Patient Three
Four month old with fever and dehydration
Failure to thrive and decreased tone
120
2.2
59
41
18
0.6
Case Three Diagnosis
Excess aldosterone effect
Absence of aldosterone effect
Physiologic aldosterone overproduction
Voting
0
0
100%
Na+
K+
Na+
K+
K+
H+
Na+
K+
Na+
K+
K+
Na+
Na+
Na+
Na+
H+
-
-
-
-
Distal tubule
Bartter Syndrome
Hypokalemia and metabolic alkalosis
Failure to thrive and muscle weakness
Polyuria and polydipsia (polyhydramnios
and premature delivery)
Autosomal recessive
Differential of ExcessAldosterone Effect
High AldosteroneLow Renin
Primary aldosteronism
Glucocorticoid-remediable aldosteronism
Low AldosteroneLow Renin
Congenital adrenal hyperplasia
Liddle syndrome
Apparent mineralocorticoid excess
Licorice
High AldosteroneHigh Renin
Renovascular disease
Renin-secreting tumor
Malignant hypertension
Birth control pills
Na
K
Na
Aldosterone
Cortisol11HSDCortisone
K
11 -HydroxysteroidDehydrogenase
Differential of Absence of Aldosterone Effect
Aldosterone deficiency
Adrenal insufficiency CAH Aldosterone
synthetase
Aldosterone resistance
Pseudo-hypoaldosteronism
Obstructive uropathy Pyelonephritis Sickle cell disease Chronic renal failure
Differential of Physiologic Aldosterone
Overproduction
Urine Chloride <10
Loss of gastric fluids Chloride-losing
diarrhea Sweat (CF) Dietary chloride
deficiency Remote diuretics
Urine Chloride >20
Current diuretics Bartter syndrome Gitelman
syndrome
The End
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