Replacement Therapies in Adrenal Insufficiency

1. Why do we need to get hydrocortisone replacement right?

2. Mortality and morbidity data

3. Side effects and survey results of quality of life

4. Oral Glucocorticoid replacement

5. What is dosing regimen

6. How to calculate daily dose

7. How to distribute the dose

8. Drug interactions including drugs that alter liver metabolism

9. Specific problems with hormone replacement - oral or transdermal estrogens

10. Interplay of cortisol and vasopressin - diagnosis of diabetes insipidus and DDAVP replacement

11. Pituitary replacement that may modify hydrocortisone dosing - thyroxine and growth hormone

12. Monitoring replacement

13. What's missing what are we replacing what should we check

14. Ways of checking 17OHP, ACTH cortisol

15. Single sample versus multiple samples

16. Around dose sampling

17. Effects of OCP

18. Adjusting doses for events

19. Illness and the boxes

20. Stressful events

21. Surgery

22. Dental and the boxes

23. Travel and time zones

24. Adjustments and effects on other hormones e.g. DDAVP

25. Glucocorticoid replacement using pump therapy

26. Pump therapy to mimic circadian rhythm

27. Principles of the pump

28. Setting the system up, clearance studies

29. Monitoring replacement

30. Quality of life

31. Managing an Adrenal Crisis

32. Presentation as new diagnosis or following diagnosis

33. What it is and what it is not

34. Biochemical changes

35. How to manage a crisis - at home, in A&E after admission

36. Hydrocortisone requirements in a crisis

37. Glucose requirements during a crisis

38. Sodium and fluid balance - the importance of subacute or chronic hyponatraemia

39. Hyperkalaemia

40. Weaning Glucocorticoids

41. Factors influencing adrenal suppression dose, type of steroid, how long given, potency and whether given during day or night

42. The role of alternate day treatments

43. Principles in weaning

44. How to wean depending on duration of exposure, disease activity steroid used

45. What tests to use to determine axis recovery

Replacement Therapies in Adrenal Insufficiency provides a thorough understanding of the conditions which result in adrenal insufficiency. Never before has one source of information combined all updates on current causes and mechanisms of adrenal sufficiency to allow for quick reference and subsequent treatment decisions. Scientific data on this broad condition includes specific disease coverage of Addison's disease, hypopituitarism, congenital adrenal hypoplasia and adrenalectomy.

 

Practical points in diagnosis, dosing, drug interactions, replacement therapies and emergency situations are also provided as guidance for overall management.

 

Authors

Peter Hindmarsh is Professor of Pediatric Endocrinology at University College London and Consultant in Pediatric Endocrinology and Diabetes at University College London Hospitals and Great Ormond Street Hospital for Children. Currently, he is applying Chronic Care Models to the delivery of care for patients with Congenital Adrenal Hyperplasia. His research interests are in the physiology and pharmacology of drugs used to manage Congenital Adrenal Hyperplasia and novel ways of delivery Hydrocortisone using pump therapy to mimic the circadian rhythm. As the parent of a young adult with Salt Wasting Congenital Adrenal Hyperplasia,

 

Kathy Geertsma became Chair of the Congenital Adrenal Hyperplasia Parent and Support Group. She has extensive contact with parents and patients from all over the world who struggle with confirming a CAH diagnosis and getting proper treatment. As a result, she offers a unique perspective about how practitioners can better serve the needs of their patient population.