Repaso del acercamiento diagnóstico, terapeutico y de seguimiento en el paciente con insuficiencia suprarrenal Un poco de arte tambien, Dalí...

1. Insuficiencia
suprarrenal
Hospital General “Dr. Nicolás San Juan”
Servicio de Medicina Interna
Daniel Rebolledo García
R3 Medicina Interna
Diciembre 2018
Toluca, México

2. • Descrita por Thomas Addison en 1855.
(Debilidad, fatiga, anorexia, hipotensión ortostatica,
ingesta de sal).
• La causa mas frecuente en los casos descritos fue
la tuberculosis (siglo XIX).
Curr Opin Endocrinol Diabetes Obes. 2010 June ; 17(3): 217-223

4. Definición
• Es la falla de producción de todas las hormonas de la corteza suprarrenal.
• La causa más frecuente en países desarrollados es la autoinmune.
• Puede ocurrir de forma aislada, con otros cuadros autoinmunes (síndrome
poliglandular tipo 1 y 2).
• Mutaciones ligadas al gen AIRE.
• HLA genotipo DR3/4-DQB1*0302 (diabetes mellitus tipo 1).
• Adrenoleucodistrofia ligada al X.

5. Epidemiología
• Addison’s disease has a prevalence of 93-140 per million people and an
annual incidence of 4.7-6.2 per million people in Western populations.
• A survey of patients with Addison’s disease found that 60% had seen two or
more clinicians before the diagnosis of Addison’s disease was ever
considered.
• An observational study of children with Addison’s disease found that a delay in
diagnosis occurred in about a third of the cases, in whom the median duration
between the onset of first symptoms and the correct diagnosis was two years.

6. • Before the availability of synthetic glucocorticoid replacement therapy, the
disease resulted in a 2-year mortality above 80% and the death of all patients
within 5 years of diagnosis.

8. Lancet Diabetes Endocrinol 2014

9. Am Fam Physician. 2014;89(7):563-568

10. Lancet Diabetes Endocrinol 2014

11. Lancet Diabetes Endocrinol 2014

13. BMJ 2009;339:b2385

14. Am Fam Physician. 2014;89(7):563-568

16. Am Fam Physician. 2014;89(7):563-568

17. Am Fam Physician. 2014;89(7):563-568

18. Lancet Diabetes Endocrinol 2014

20. The American Journal of Medicine (2016) 129, 339.e1-339.e9

21. The American Journal of Medicine (2016) 129, 339.e1-339.e9

22. The American Journal of Medicine (2016) 129, 339.e1-339.e9

23. Manejo en urgencias…
• In patients in adrenal crisis after hospital admission, if acute adrenal
insufficiency is clinically suspected.
• Hydrocortisone should be given without delay (100 mg bolus injection,
followed by 200 mg per 24 h either as a continuous infusion or 50 mg every 6
h).
• Along with intravenous fluid resuscitation.

24. • Intravenous fluid resuscitation with isotonic sodium chloride 0.9% will correct
the hypovolemia and hyponatremia, while intravenous dextrose may also be
required to correct hypoglycemia.

25. • One liter of saline 0.9% should be given over the first hour, and further
replacement fluids should be guided by frequent hemodynamic monitoring
and measurement of serum electrolytes.

26. • Cortisol replacement can induce water diuresis and suppress antidiuretic
hormone (in secondary adrenal insufficiency), which together with sodium
replacement can lead to rapid correction of hyponatremia, and osmotic
demyelination syndrome.

27. • In the hospital, hydrocortisone can be given intravenously or intramuscularly
100 mg as a bolus, followed by 100-300 mg per day for another 2 to 3 days,
either as boluses every 6 hours or as continuous infusion until full recovery.

28. • At hydrocortisone doses of >50 mg/day, there is sufficient action at the
mineralocorticoid receptor, and it is generally accepted that additional
mineralocorticoid therapy is not required.

29. The American Journal of Medicine (2016) 129, 339.e1-339.e9

30. The American Journal of Medicine (2016) 129, 339.e1-339.e9

31. • Daily physiological production of cortisol is about 5–6 mg/m2 body surface
area.
• Recommended hydrocortisone doses are 15–25 mg, usually given in two to
three doses per day, with 50–66% given in the morning on awakening.
• If given two times a day, the second dose is usually given 6–8 h after the
morning dose.
• If given three times per day, the second dose is given 4–6 h after the early
morning dose and the third dose 4–6 h after this.

32. • A global survey of more than 1000 patients, mostly with primary adrenal
insufficiency.
• Hydrocortisone was the most common treatment (75%),
• Prednisone or prednisolone (11%),
• Cortisone (6%), and dexamethasone (4%).

33. • Cortisone
(1 mg hydrocortisone= 1.6 mg cortisone acetate)
• Prednisone
• (1.0 mg hydrocortisone=0.25 mg prednisone) need intrahepatic activation to
cortisol and prednisolone (1.0 mg hydrocortisone=0.2 mg prednisolone),
respectively, by 11β-hydroxy steroid dehydrogenase type 1, which might lead
to a higher pharmacokinetic variability compared with hydrocortisone.

34. Am Fam Physician. 2014;89(7):563-568

37. • Gracias!!!!