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Addison's DiseaseAka: Addisonian Crisis, Adrenal Cortical Insufficiency, Adrenal Insufficiency, Adrenal Hypofunction, Illness Induced Adrenal Insufficiency, Stress Dose Steroid

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  1. See Also
    1. Autoimmune Polyglandular Syndrome
  2. Pathophysiology
    1. Hypothalamic-Pituitary-Adrenal Axis (HPA) Dysfunction
      1. Lab features: Cortisol low, ACTH Low
      2. Causes
        1. Severe illness
        2. Exogenous Corticosteroid (suppression lasts months)
          1. Prednisone >7.5 mg/day for >3 weeks
          2. Dexamethasone >0.75 mg/day for >3 weeks
        3. Pituitary Infarction
        4. CNS Infection or tumor invasion
        5. Head Injury
        6. HIV Infection
    2. Adrenal Insufficiency
      1. Lab features: Cortisol low, ACTH high
      2. Causes
        1. Addison's Disease
          1. Primary adrenal failure of adrenal glands
        2. Idiopathic adrenal hypoplasia or destruction
          1. Granulomatous disease (e.g. Tuberculosis, Fungus)
          2. Amyloidosis
          3. Hemochromatosis
          4. Tumor
          5. Autoimmune process
        3. Medication medicated cortisol inhibition
          1. Etomidate
          2. Ketoconazole
  3. Symptoms
    1. Weakness
    2. Fatigue or Lethargy
    3. Nausea or Vomiting
    4. Diarrhea
    5. Abdominal Pain
    6. Weight loss
    7. Salt craving
    8. Altered Level of Consciousness (e.g. Delirium)
  4. Signs
    1. Fever
    2. Hyperpigmentation
      1. Skin with mottled areas of increased pigmentation
      2. Pigment at Buccal mucosa (Caucasian)
      3. Increased pigment in lips, vagina, rectum
    3. Reduced Hair Growth
    4. Postural Hypotension
    5. Signs of dehydration
  5. Labs
    1. Complete Blood Count
      1. Normochromic Normocytic Anemia
      2. Eosinophilia (helps differentiate from other causes)
    2. Chemistry Panel
      1. Hyponatremia
      2. Hypochloremia
      3. Hyperkalemia
      4. Hypoglycemia (helps differentiate from other causes)
    3. Urine 17-ketosteroids low
    4. Urine 17-Hydroxysteroids low
  6. Diagnosis
    1. Corticotropin Stimulation Test
      1. Failed adrenal response
  7. Management
    1. Acute
      1. IV Cortisol
    2. Chronic Replacement
      1. Hydrocortisone (glucocorticoid) AND
      2. Mineralocorticoid (9-alpha-fluorocortisol)
    3. Stress Dose Steroid
      1. Indication: Acute severe illness
        1. Serum Cortisol <15 to 34 mcg/dl
        2. Cortisol <9 after Corticotropin Stimulation Test
      2. Management
        1. Hydrocortisone 50 mg IV or IM q6 hours
        2. Consider adding fludricortisone 50 mcg qd
  8. Resources
    1. Addison and Cushing International Federation (ACIF)
      1. http://www.nvacp.nl/page.php?main=5
  9. References
    1. Cooper (2003) N Engl J Med 348:727

Addison's disease (C0001403)

Definition (CSP)disease characterized by hypotension, weight loss, anorexia, weakness, and sometimes a bronze-like melanotic hyperpigmentation of the skin; due to tuberculosis or autoimmune induced disease (hypofunction) of the adrenal glands that results in deficiency of aldosterone and cortisol.
Definition (NCI)A hormonal disorder that occurs when the adrenal glands fail to release adequate amounts of glucocorticoids (cortisol), mineralocorticoids (aldosterone, 11-deoxycorticosterone), and androgens (dehydroepiandrosterone) to meet physiologic needs, despite release of ACTH from the pituitary.
Definition (MSH)An adrenal disease characterized by the progressive destruction of the ADRENAL CORTEX, resulting in insufficient production of ALDOSTERONE and HYDROCORTISONE. Clinical symptoms include ANOREXIA; NAUSEA; WEIGHT LOSS; MUSCLE WEAKNESS; and HYPERPIGMENTATION of the SKIN due to increase in circulating levels of ACTH precursor hormone which stimulates MELANOCYTES.
Definition (NCI)A rare endocrine or hormonal disorder that occurs when the adrenal glands do not produce enough of the hormone cortisol and in some cases, the hormone aldosterone. It may be due to a disorder of the adrenal glands themselves (primary adrenal insufficiency) or to inadequate secretion of ACTH by the pituitary gland (secondary adrenal insufficiency).
ConceptsDisease or Syndrome (T047)
ICD9255.41
MSHD000224
EnglishADDISON DIS, Addison Disease, Addison's disease, ADDISONS DIS, Addisons Disease, Chronic Adrenal Insufficiency, DISEASE ADDISON'S, Hypocortisolism, Primary adrenal deficiency, Primary Adrenal Insufficiency, Primary Adrenocortical Insufficiencies, Primary Adrenocortical Insufficiency, Primary Hypoadrenalism
Spanishenfermedad de Addison, insuficiencia adrenal cronica, insuficiencia adrenal primaria, insuficiencia adrenocortical primaria, insuficiencia corticosuprarrenal primaria, insuficiencia suprarrenal cronica, insuficiencia suprarrenal primaria
Parent ConceptsAdrenal cortical hypofunction (C0405580), Adrenal gland hypofunction (C0001623), Autoimmune Diseases (C0004364), Addison's disease (C0001403), Ambiguous concept (C1274012), Reason not stated concept (C1276325)
SourcesAOD, COSTAR, CSP, CST, DXP, LCH, MEDLINEPLUS, MSH, MTH, MTHICD9, NCI, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


Adrenal gland hypofunction (C0001623)

Definition (CSP)abnormally diminished activity of the adrenal gland.
Definition (MSH)Conditions in which the production of adrenal CORTICOSTEROIDS falls below the requirement of the body. Adrenal insufficiency can be caused by defects in the ADRENAL GLANDS, the PITUITARY GLAND, or the HYPOTHALAMUS.
Definition (NCI)Adrenocortical hypofunction includes all conditions in which adrenal steroid hormone secretion falls below the requirements of the body. Adrenal insufficiency may be divided into two general categories: (1) those associated with primary inability of the adrenal to elaborate sufficient quantities of hormone and (2) those associated with a secondary failure due to a primary failure in the elaboration of adrenocorticotropin. (Harrison's Principles of Internal Medicine, 13th ed, p1970)
ConceptsDisease or Syndrome (T047)
MSHD000309
EnglishAdrenal failure, Adrenal gland hypofunction, Adrenal Gland Insufficiency, Adrenal hypofunction, Adrenal Insufficiencies, ADRENAL INSUFFICIENCY, hypoadrenalism, HYPOFUNCTION ADRENAL, INSUFFICIENCY ADRENAL
Spanishhipoadrenalismo, hipofuncion adrenal, hipofuncion suprarrenal
Parent ConceptsAdrenal Gland Diseases (C0001621), Non-Neoplastic Adrenal Gland Disorder (C1334993), Non-Neoplastic Endocrine Disorder (C1335007), Adrenal gland hypofunction (C0001623)
SourcesCOSTAR, CSP, CST, DXP, MSH, MTH, NCI, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


Addisonian crisis (C0151467)

ConceptsDisease or Syndrome (T047)
ICD9255.41
EnglishAcute adrenal insufficiency, Addisonian crisis, ADRENAL APOPLEXY, ADRENAL CRISIS, ADRENAL STORM, Adrenocortical crisis, CRISIS ADDISONIAN, CRISIS ADRENAL, Severe adrenal insufficiency
Spanishcrisis addisoniana, crisis suprarrenal, insuficiencia adrenal severa, insuficiencia suprarrenal aguda, insuficiencia suprarrenal severa
Parent ConceptsAdrenal Cortex Diseases (C0001614), ADRENAL DYSFUNCTION (C0549609), Adrenal gland hypofunction (C0001623), Ambiguous concept (C1274012), Duplicate concept (C1274013)
SourcesCST, DXP, MTH, MTHICD9, OMIM, QMR, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


Adrenal cortical hypofunction (C0405580)

Definition (NCI)The inability of the adrenal gland to produce adequate amounts of cortisol.
ConceptsDisease or Syndrome (T047)
ICD9255.4, 255.41
EnglishADRENAL CORTEX HYPOFUNCTION, ADRENAL CORTEX INSUFFIENCY, Adrenal cortical hypofunction, ADRENAL CORTICAL INSUFFICIENCY, Adrenal Insufficiency, Adrenocortical hypofunction, Corticoadrenal insufficiency, Hypoadrenocorticism, INSUFFICIENCY ADRENAL CORTEX
Spanishhipoadrenocorticismo, hipofuncion adrenocortical, hipofuncion de corteza adrenal, insuficiencia corticoadrenal, insuficiencia corticosuprarrenal, insuficiencia suprarrenal
Parent ConceptsAdrenal Cortex Diseases (C0001614), ADRENAL DYSFUNCTION (C0549609), Adrenal Gland Diseases (C0001621), Adrenal gland hypofunction (C0001623), Adrenal cortical hypofunction (C0405580), Ambiguous concept (C1274012)
SourcesAOD, CST, ICD9CM, MTH, MTHICD9, NCI, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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