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35382.ppt

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35382.ppt

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

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