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A Case From The Clinic

A Case From The Clinic. Paul J. Scheel, Jr., MD Director Of Nephrology The Johns Hopkins University School of Medicine. Patient W.T. 56 year old AA male Hypertension x 28 years Hypokalemia past 2 years during annual physical. ( 2.8,3.1, 3.0) Past Medical History : Negative

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A Case From The Clinic

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  1. A Case From The Clinic Paul J. Scheel, Jr., MD Director Of Nephrology The Johns Hopkins University School of Medicine

  2. Patient W.T. • 56 year old AA male • Hypertension x 28 years • Hypokalemia past 2 years during annual physical. ( 2.8,3.1, 3.0) • Past Medical History : Negative • Past Surgical History: Absent

  3. Patient W.T. • Current Meds: • Procardia XL 90 mg twice daily • Amiloride 10 mg orally each day • Metoprolol 100 mg twice daily • Clonidine 0.2 three times daily

  4. Patient W.T. • Family History: Mother and Father both deceased ( 64,59) both with hypertension, One of 7 children all with hypertension • Social History: Recently retired from Federal Government. No Tob or Alcohol, No history of recreational drug use. • Review of Systems: Occasional fatigue and erectile dysfunction.

  5. Patient W.T.Physical Exam • General: Appeared Well • Vitals: BP 160/92, P 62, R 12 Wt 175 # • HEENT: Normal Fundi • Neck: No Bruits • Back: No Buffalo Humping • CV: Displaced PMI, S4, All peripheral pulses strong without bruits. • Abdomen: No masses No striae, No Bruits • Skin: No Echymoses

  6. Patient W.T.Labs 26 143 108 25 3.2 0.9 U/A: Dip negative , No Cells

  7. Hypertension and HypokalemiaDifferential Diagnosis • Mineralocorticoid Excess • Hyperaldosteronism • Excess deoxycorticosterone • Renal Vascular Disease • Cushing’s • Congenital Adrenal Hyperplasia • Renin Secreting tumors

  8. When to Evaluate • Unexplained Hypokalemia ? • Severe, Resistant Hypertension or a Change in BP Pattern ? • Adrenal Incidentaloma • Physical Exam Suggestive of Excess Cortisol. • Hypertension Alone ?

  9. Incidence Of HyperaldosteronismPAC/PRA > 30

  10. Primary HyperaldosternoismPrevalence by JNC VI • I: BP 140-159/90-99 • II: BP 160-179/100-109 • III BP > 180/>110

  11. Pathophysiology Na, K Circulating Blood Volume Renal Perfusion Pressure Aldosterone Release Renin Release Angiotensinogen Angiotensin II Angiotensin I

  12. Pathophysiology Tubular Lumen Peritubular Capillary Na 3Na 2K Aldosterone Receptor Aldosterone K

  13. Diagnosis • Plasma Renin Activity • Plasma Aldosterone • Plasma Aldosterone: Renin Ratio • 24 Hour Urine ( For What ?)

  14. Plasma Aldosterone: Renin • 8 am paired plasma Aldosterone + Renin • For Diagnosis of Hyperaldosteronism Plasma Aldosterone > 20 • Patients must be off Aldactone for 6 weeks • Calcium Channel Blockers, Alpha Blockers, Beta Blockers OK • ACEI : May falsely elevate renin

  15. Plasma Aldosterone : Renin • Interpretation of Results: • Normal - 4-10 • Hyperaldosteronism – 30-50 Must know lower limit of lab for plasma renin. Is is 0.6 or 0.1 ? May significantly affect ratios

  16. PAC/PRA • PAC > 20 and PAC/PRA > 30 • Sensitivity and Specificity of 90% for diagnosis of aldosterone producing adenoma

  17. 24 Hour Urine Collection • Historically used to document K+ Wasting • Now more useful to document other potential etiologies for low K + • 24 hour Urine should be sent for: • K + • Na + • Creatinine • Aldosterone

  18. 24 Hour Urine CollectionResults • In setting of hypokalemia • Inappropriate K + Wasting > 30 meq/day • < 30 meq /day suggest extra renal losses • Aldosterone > 14μg/day ( 39nmol/day) • 24 hour urine sodium must be > 200 meq/day • Must be accurate 24 hour collection (creatinine) • Woman 10-12 mg/kg body wt/24 hrs • Men: 12-15 mg/kg/body wt/24 hrs

  19. Hypertension and Hypokalemia Plasma Renin and Plasma Aldosterone PRA PRA PRA PAC PAC PAC Secondary Hyperaldosteronism Hyperaldosteronism Work Up CAH DOC-Tumor Cushings Syndrome Renovasular Disease Diuretic Use Renin Tumor

  20. HyperaldosteronismConfirmatory Evaluation • Increased PAC:PRA • Confirmatory Testing Requires • High Sodium Diet followed by 24 hr urine • Saline Suppression Test with repeat of PAC:PRA • Fludrocortisone Suppression ( 0.2 mg b.i.d. x 2 days) Aldosterone level on day 3 > 5 confirmatory OR OR

  21. HyperaldosteronismClassification • Adrenal Hyperplasia • Adrenal Adenoma • Adrenal Carcinoma • Familial Hyperaldosteronism I + II

  22. Radiologic Testing • CT or MRI • Unilateral Adrenal Mass > 5 cm Carcinoma • Can Identify Adenomas > 1 cm • Bilateral Abnormal Glands or Normal Bilateral Glands Suggest Hyperplasia

  23. Radiologic Testing • Adrenal Vein Sampling: • Selective Catheterization of Adrenal Veins • > 5x PAC From One Side Unilateral Disease • Must Also Measure After ACTH Stimulation Measuring both Aldosterone and Cortisol. • Cortisol Should be 10x Cortisol From Peripheral Vein

  24. Patient W.T • Plasma Aldosterone 25, PRA 0.63 Ratio 40 • Saline Suppression PAC 21, PRA 0.4 Ratio 52.5 • CT Scan: No abnormality • Dexamethasone Suppression PAC 17, PRA 0.4 , Ratio 42.5

  25. Confirmed Hyperaldosteronism Negative CT Empiric Treatment Aldactone 100 mg- 200mg Adrenal Vein Sampling

  26. Medical Therapy • Aldactone: Usual therapeutic dose is 100-200mg in divided doses per day. • Amiloride or Triamtene, ? Eplerenone • Lifestyle Modification • Ideal Body Wt • Exercise • Smoking Cessation • Moderation of Alcohol Consumption • Sodium Restriction ( < 100 mEq/day)

  27. Negative CT • Adenomas < 1 cm will be missed • Sensitivity compared to adrenal vein sampling with subsequent surgery and histologic confirmation of adenoma as low as 53 % .

  28. Confirmed Hyperaldosteronism Negative CT Empiric Treatment Aldactone 100 mg- 200mg Adrenal Vein Sampling Adrenalectomy

  29. Adrenal Vein SamplingPatient W.T. Aldosterone 3229 ng/dl Aldosterone 39 ng/dl Cortisol 1062 mcg/dl Cortisol 598 mcg/dl

  30. Confirmed Hyperaldosteronism Adrenal Adenoma Laparoscopic Adrenalectomy Adrenal Vein Sampling Medical Therapy

  31. Patient W.T.

  32. Patient W.T. • Patient Now 3 months S/p Adrenalectomy • Bp 127/71 on Atenolol 50 mg once daily

  33. Conclusions: • Hyperaldosteronism suspected in a patient with hypertension and unexplained hypokalemia or Severe Hypertension alone • Screen with PAC:PRA • Confirmatory Testing with Saline Suppression Test or Salt loading followed by 24 hr Urine.

  34. Conclusions: • CT or MRI can detect lesions > 1 cm • Normal CT or MRI does not rule out microadenoma • Adrenal Vein sampling is difficult to perform but is crucial to differentiating unilateral vs bilateral disease • Surgical Therapy = Adrenalectomy • Medical Therapy = Aldactone, ? Eplerenone

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