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

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Presentation on theme: "Three Children with Electrolyte Problems by Larry Greenbaum, MD, PhD Pediatric Nephrology by Larry Greenbaum, MD, PhD Pediatric Nephrology."— Presentation transcript:

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

2 Patient One u Two year old with failure to thrive u Polyuria and polydipsia u Blood pressure of 160/

3 Patient Two u One week old brought to the hospital for lethargy, poor feeding and dehydration (Bun and creatinine normalized after hydration)

4 Patient Three u Four month old with fever and dehydration u Failure to thrive and decreased tone

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

6 Distal tubule

7 Na + K+K+ K+K+ K+K+ H+H+ K+K+ K+K+ K+K+ H+H

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

9 Three Possible Problems u Excess aldosterone effect l Hypertension l Hypokalemia and metabolic alkalosis u Absence of aldosterone effect l Hypotension l Hyperkalemia, metabolic acidosis and hyponatremia u Physiologic aldosterone overproduction l Volume depletion l Hypokalemia and metabolic alkalosis

10 Excess Aldosterone Effect Adrenal Gland Kidney Aldosterone Adrenal adenoma

11 Na + K+K+ K+K+ K+K+ H+H+ K+K+ K+K+ K+K+ H+H

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

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

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

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

16 Distal tubule

17 Na + K+K+ K+K+ K+K+ H+H+ K+K+ K+K+ K+K+ H+H

18 Patient One u Two year old with failure to thrive u Polyuria and polydipsia u Blood pressure of 160/

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

20 Na + K+K+ K+K+ K+K+ H+H+ K+K+ K+K+ K+K+ H+H

21 Liddle Syndrome u Severe hypertension u Hypokalemia and metabolic alkalosis u Polyuria, polydipsia and muscle weakness u Low aldosterone and renin levels u Autosomal dominant u Blood pressure does not improve with Aldactone but does improve with triamterene or amiloride

22 Na + K+K+ K+K+ K+K+ H+H+ K+K+ K+K+ K+K+ H+H A A A A TT TT

23 Patient Two u One week old brought to the hospital for lethargy, poor feeding and dehydration (Bun and creatinine normalized after hydration)

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

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

26 Pseudohypoaldosteronism Type I u Dehydration and failure to thrive as neonates u Hyponatremia, hyperkalemia and metabolic acidosis u Elevated plasma renin and aldosterone u Aldosterone resistance in kidney, sweat and salivary glands, colonic mucosa u Autosomal recessive u Treatment with NaCl and Kayexalate

27 Patient Three u Four month old with fever and dehydration u Failure to thrive and decreased tone

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

29 Na + K+K+ K+K+ K+K+ H+H+ K+K+ K+K+ K+K+ H+H

30 Distal tubule

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

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

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

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

35 Differential of Physiologic Aldosterone Overproduction Urine Chloride <10 u Loss of gastric fluids u Chloride-losing diarrhea u Sweat (CF) u Dietary chloride deficiency u Remote diuretics Urine Chloride >20 u Current diuretics u Bartter syndrome u Gitelman syndrome

36 The End

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