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Three Children with Electrolyte Problems

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.

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Three Children with Electrolyte Problems

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  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

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