Three Children with Electrolyte Problems

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

2. 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

3. 90 124 30 12 7.7 1.5 (Bun and creatinine normalized after hydration) Patient Two • One week old brought to the hospital for lethargy, poor feeding and dehydration

4. 59 120 18 41 2.6 0.6 Patient Three • Four month old with fever and dehydration • Failure to thrive and decreased tone

5. 95% 10% Yes 5% 90% No Do you enjoy renal physiology?

6. Glomerulus Distal tubule Proximal tubule Collecting duct Loop of Henle

7. Na+ Na+ Na+ - Na+ K+ - Na+ Na+ Na+ Na+ - Na+ - Na+ Na+ K+ K+ K+ K+ Na+ K+ H+ H+

8. Volume Depletion Adrenal Gland ATII Renin Kidney Aldosterone Regulation of Aldosterone

9. 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

10. Adrenal Gland Kidney Aldosterone Excess Aldosterone Effect Adrenal adenoma

11. Na+ Na+ Na+ - K+ - Na+ Na+ - - Na+ Na+ K+ K+ K+ K+ Na+ K+ H+ H+

12. Volume Depletion Adrenal Gland Kidney ATII Renin Absence of Aldosterone Effect 21-Hydroxylase deficiency (CAH)

13. 17-Hydroxypreg. 17-hydroxyprog. 11-deoxycortisol Cortisol Mineralocorticoids Glucocorticoids Androgens Pregnenolone Progesterone DOC Corticosterone 18-Hydroxycorticosterone Aldosterone DHEA Androstendione Testosterone

14. Na+ Na+ K+ Na+ Na+ Na+ Na+ Na+ K+ K+ K+ K+ Na+ K+ H+ H+

15. Volume Depletion Adrenal Gland Kidney ATII Renin Aldosterone Physiologic Aldosterone Overproduction Loop Diuretic

16. Glomerulus Distal tubule Proximal tubule Collecting duct Loop of Henle

17. Na+ Na+ Na+ - K+ - Na+ Na+ - - Na+ Na+ K+ K+ K+ K+ Na+ K+ H+ H+

18. 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

19. Case One Diagnosis Voting • Excess aldosterone effect • Absence of aldosterone effect • Physiologic aldosterone overproduction 85% 10% 5%

20. Na+ Na+ Na+ - K+ - Na+ Na+ - - Na+ Na+ K+ K+ K+ K+ Na+ K+ H+ H+

21. 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

22. A A T Na+ Na+ T Na+ - K+ - A A T T Na+ Na+ - - Na+ Na+ K+ K+ K+ K+ Na+ K+ H+ H+

23. 90 124 30 12 7.7 1.5 (Bun and creatinine normalized after hydration) Patient Two • One week old brought to the hospital for lethargy, poor feeding and dehydration

24. Case Two Diagnosis Voting • Excess aldosterone effect • Absence of aldosterone effect • Physiologic aldosterone overproduction 5% 90% 5%

25. Na+ Na+ K+ Na+ Na+ Na+ Na+ Na+ K+ K+ K+ K+ Na+ K+ H+ H+

26. 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

27. 59 120 18 41 2.2 0.6 Patient Three • Four month old with fever and dehydration • Failure to thrive and decreased tone

28. Case Three Diagnosis Voting • Excess aldosterone effect • Absence of aldosterone effect • Physiologic aldosterone overproduction 0 0 100%

29. Na+ Na+ Na+ - K+ - Na+ Na+ - - Na+ Na+ K+ K+ K+ K+ Na+ K+ H+ H+

30. Glomerulus Distal tubule Proximal tubule Collecting duct Loop of Henle

31. Bartter Syndrome • Hypokalemia and metabolic alkalosis • Failure to thrive and muscle weakness • Polyuria and polydipsia (polyhydramnios and premature delivery) • Autosomal recessive

32. Differential of ExcessAldosterone Effect High Aldosterone Low Renin • Primary aldosteronism • Glucocorticoid-remediable aldosteronism • High Aldosterone • High Renin • Renovascular disease • Renin-secreting tumor • Malignant hypertension • Birth control pills • Low Aldosterone • Low Renin • Congenital adrenal hyperplasia • Liddle syndrome • Apparent mineralocorticoid excess • Licorice

33. 11 -Hydroxysteroid Dehydrogenase Cortisone 11HSD Cortisol K Aldosterone K Na Na

34. Aldosterone deficiency Adrenal insufficiency CAH Aldosterone synthetase Aldosterone resistance Pseudo-hypoaldosteronism Obstructive uropathy Pyelonephritis Sickle cell disease Chronic renal failure Differential of Absence of Aldosterone Effect

35. 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 Differential of Physiologic Aldosterone Overproduction

36. The End