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Three Children with Electrolyte Problems by Larry Greenbaum, MD, PhD Pediatric Nephrology
37

Three Children with Electrolyte Problems

Jan 20, 2016

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Three Children with Electrolyte Problems. by Larry Greenbaum, MD, PhD Pediatric Nephrology. 106. 147. 8. 32. 1.8. 0.4. Patient One. Two year old with failure to thrive Polyuria and polydipsia Blood pressure of 160/90. 90. 124. 30. 12. 7.7. 1.5. - PowerPoint PPT Presentation
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Page 1: Three Children with Electrolyte Problems

Three Children with Electrolyte Problems

by

Larry Greenbaum, MD, PhDPediatric Nephrology

by

Larry Greenbaum, MD, PhDPediatric Nephrology

Page 2: Three Children with Electrolyte Problems

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

Page 3: Three Children with Electrolyte Problems

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)

Page 4: Three Children with Electrolyte Problems

Patient Three

Four month old with fever and dehydration

Failure to thrive and decreased tone

120

2.6

59

41

18

0.6

Page 5: Three Children with Electrolyte Problems

Do you enjoy renal physiology?

Yes

No

10%

90%

95%

5%

Page 6: Three Children with Electrolyte Problems

Distal tubule

Page 7: Three Children with Electrolyte Problems

Na+

K+

Na+

K+

K+

H+

Na+

K+

Na+

K+

K+

Na+

Na+

Na+

Na+

H+

Na+

Na+

Na+

Na+

-

-

-

-

Page 8: Three Children with Electrolyte Problems

AdrenalGlandKidney

Aldosterone

Renin ATII

Volume Depletion

Regulation of Aldosterone

Page 9: Three Children with Electrolyte Problems

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

Page 10: Three Children with Electrolyte Problems

Excess Aldosterone Effect

AdrenalGlandKidney

Aldosterone

Adrenal adenoma

Page 11: Three Children with Electrolyte Problems

Na+

K+

Na+

K+

K+

H+

Na+

K+

Na+

K+

K+

Na+

Na+

Na+

Na+

H+

-

-

-

-

Page 12: Three Children with Electrolyte Problems

Absence of Aldosterone Effect

AdrenalGlandKidney

Renin ATII

Volume Depletion

21-Hydroxylase deficiency(CAH)

Page 13: Three Children with Electrolyte Problems

17-Hydroxypreg.

17-hydroxyprog.

11-deoxycortisol

Cortisol

Pregnenolone

Progesterone

DOC

Corticosterone

18-Hydroxycorticosterone

Aldosterone

DHEA

Androstendione

Testosterone

GlucocorticoidsMineralocorticoids Androgens

Page 14: Three Children with Electrolyte Problems

Na+

K+

Na+

K+

K+

H+

Na+

K+

Na+

K+

K+

H+

Na+

Na+

Na+

Na+

Page 15: Three Children with Electrolyte Problems

Physiologic Aldosterone Overproduction

AdrenalGlandKidney

Aldosterone

Renin ATII

Volume Depletion

Loop Diuretic

Page 16: Three Children with Electrolyte Problems

Distal tubule

Page 17: Three Children with Electrolyte Problems

Na+

K+

Na+

K+

K+

H+

Na+

K+

Na+

K+

K+

Na+

Na+

Na+

Na+

H+

-

-

-

-

Page 18: Three Children with Electrolyte Problems

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

Page 19: Three Children with Electrolyte Problems

Case One Diagnosis

Excess aldosterone effect

Absence of aldosterone effect

Physiologic aldosterone overproduction

Voting

85%

10%

5%

Page 20: Three Children with Electrolyte Problems

Na+

K+

Na+

K+

K+

H+

Na+

K+

Na+

K+

K+

Na+

Na+

Na+

Na+

H+

-

-

-

-

Page 21: Three Children with Electrolyte Problems

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

Page 22: Three Children with Electrolyte Problems

Na+

K+

Na+

K+

K+

H+

Na+

K+

Na+

K+

K+

Na+

Na+

Na+

Na+

H+

-

-

-

-

AA

A

A

TT

TT

Page 23: Three Children with Electrolyte Problems

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)

Page 24: Three Children with Electrolyte Problems

Case Two Diagnosis

Excess aldosterone effect

Absence of aldosterone effect

Physiologic aldosterone overproduction

Voting

5%

90%

5%

Page 25: Three Children with Electrolyte Problems

Na+

K+

Na+

K+

K+

H+

Na+

K+

Na+

K+

K+

H+

Na+

Na+

Na+

Na+

Page 26: Three Children with Electrolyte Problems

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

Page 27: Three Children with Electrolyte Problems

Patient Three

Four month old with fever and dehydration

Failure to thrive and decreased tone

120

2.2

59

41

18

0.6

Page 28: Three Children with Electrolyte Problems

Case Three Diagnosis

Excess aldosterone effect

Absence of aldosterone effect

Physiologic aldosterone overproduction

Voting

0

0

100%

Page 29: Three Children with Electrolyte Problems

Na+

K+

Na+

K+

K+

H+

Na+

K+

Na+

K+

K+

Na+

Na+

Na+

Na+

H+

-

-

-

-

Page 30: Three Children with Electrolyte Problems

Distal tubule

Page 31: Three Children with Electrolyte Problems

Bartter Syndrome

Hypokalemia and metabolic alkalosis

Failure to thrive and muscle weakness

Polyuria and polydipsia (polyhydramnios

and premature delivery)

Autosomal recessive

Page 32: Three Children with Electrolyte Problems

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

Page 33: Three Children with Electrolyte Problems

Na

K

Na

Aldosterone

Cortisol11HSDCortisone

K

11 -HydroxysteroidDehydrogenase

Page 34: Three Children with Electrolyte Problems

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

Page 35: Three Children with Electrolyte Problems

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

Page 36: Three Children with Electrolyte Problems

The End

Page 37: Three Children with Electrolyte Problems