Secondary adrenal insufficiency

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Adrenal insufficiency
Adrenal gland
ICD-10 E27.1-E27.4
ICD-9 255.4
MeSH D000309

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: hypocortisolism; hypocorticism; adrenocortical hypofunction

Overview

Adrenal insufficiency is a condition in which the adrenal glands, located above the kidneys, do not produce adequate amounts of steroid hormones (chemicals produced by the body that regulate organ function), primarily cortisol, but may also include impaired aldosterone production (a mineralocorticoid) which regulates sodium, potassium and water retention.[1][2] Craving for salt or salty foods due to the urinary losses of sodium is common.[3]

Adrenal insufficiency can also occur when the hypothalamus or the pituitary gland, both located at the base of the skull, does not make adequate amounts of the hormones that assist in regulating adrenal function.[1][4][5] This is called secondary adrenal insufficiency and is caused by lack of production of ACTH in the pituitary or lack of CRH in the hypothalamus.[6]

Classification

There are three major types of adrenal insufficiency.

Pathophysiology

  • Primary adrenal insufficiency:

Here the defect lies in the adrenal glands. Autoantibodies, infections, drugs and mutations cause destruction of the adrenal glands leading to deficiency of adrenal hormones and thus causing symptoms.

  • Secondary adrenal insufficiency:

ACTH deficiency caused by diseases of the pituitary gland is the main cause for adrenal insufficiency. ACTH deficiency leads to loss of stimulus for the production of adrenal hormones. ACTH levels in the blood can differentiate primary adrenal insufficiency from the secondary form. In the primary form the ACTH levels are high because of the loss of negative feedback from the adrenal hormones.

  • Tertiary adrenal insufficiency:

The hypothalamus in the brain secretes a hormone called corticotropin releasing factor (CRF). This hormone provides the necessary stimulus to the pituitary for secreting ACTH which in turn activates the process of production of adrenal hormones. In diseases of the hypothalamus, the CRF is not produced leading to a decreased production of ACTH and in turn the adrenal hormones.

Causes

Common Causes

Causes by Organ System

Cardiovascular Arteritis, Hypotension, Hemorrhage, Infarction
Chemical / poisoning No underlying causes
Dermatologic POEMS syndrome
Drug Side Effect Aminoglutethimide, Corticosteroid withdrawal, Trilostane
Ear Nose Throat No underlying causes
Endocrine Allgrove syndrome, Achalasia-addisonian syndrome, Adrenal aplasia / adrenal hypoplasia, Adrenal metastases, After surgery of cortisol-secreting tumor, Autoimmune polyendocrine syndrome type 2, Bilateral adrenalectomy , Congenital adrenal hyperplasia, Glucocorticoid deficiency 1, IMAGE syndrome, POEMS syndrome, X-linked adrenal hypoplasia congenita
Environmental No underlying causes
Gastroenterologic Allgrove syndrome, Achalasia-addisonian syndrome, Hemochromatosis
Genetic Congenital adrenal hyperplasia, Cytochrome P450 oxidoreductase deficiency, Glycerol kinase deficiency, Hereditary ACTH resistance, IMAGE syndrome, X-linked adrenal hypoplasia congenita
Hematologic Anticoagulation, Coagulopathy , Embolus, Leukemia, Lymphoma , Thrombosis
Iatrogenic Iatrogenic, Radiation therapy
Infectious Disease AIDS, Blastomycosis, CMV, Coccidiomycosis, Cryptococcosis, Histoplasmosis , Mycobacterium avium intracellulaire (MAI), Sepsis, Syphilis, Toxoplasmosis, Tuberculosis, Waterhouse-Friderichson syndrome
Musculoskeletal / Ortho IMAGE syndrome
Neurologic Adrenoleukodystrophy, Coma, Craniopharyngioma, Panhypopituitarism, POEMS syndrome
Nutritional / Metabolic No underlying causes
Obstetric/Gynecologic No underlying causes
Oncologic Craniopharyngioma, Kaposi's sarcoma, Leukemia, Lymphoma
Opthalmologic No underlying causes
Overdose / Toxicity No underlying causes
Psychiatric No underlying causes
Pulmonary Sarcoidosis,
Renal / Electrolyte Uremia, Waterhouse-Friderichson syndrome
Rheum / Immune / Allergy Autoimmune, Autoimmune polyendocrine syndrome type 2, Sarcoidosis
Sexual No underlying causes
Trauma Trauma
Urologic No underlying causes
Dental No underlying causes
Miscellaneous Amyloidosis, Idiopathic, Iatrogenic, Radiation therapy, Surgery, Surgical removal of pituitary, Surgical removal of hypothalamus

Causes in Alphabetical Order


Risk Factors

The following conditions increase the risk of adrenal insufficiency:

Natural HIstory, Complications and Prognosis

Complications

Complications of adrenal insufficiency include:

Prognosis

The prognosis of adrenal insufficiency depends on:

  • Whether the patient has received hormone replacement or not.
  • When stress happens on the patients with adrenal insufficiency, whether the treatment protocols are adjusted or not.

Diagnosis

Symptoms

There is often the slow insidious onset of the following symptoms:

Physical Examination

Appearance

Vital Signs

Skin

  • There can be tanning of the skin. Tanning of the skin that may be patchy or even all over the body. Characteristic sites of tanning are skin creases (e.g. of the hands) and the inside of the cheek (buccal mucosa).
  • Vitiligo may also be present.
  • Absence of axillary and pubic hair in females as a result of loss of adrenal androgens.
  • Pallor may be present.

Neck

Extremities

Neurologic

Laboratory Findings

ACTH Stimulation Test

If the person is in adrenal crisis, the ACTH stimulation test[25] may be given. If not in crisis, cortisol, ACTH, aldosterone, renin, potassium and sodium are tested from a blood sample before the decision is made if the ACTH stimulation test needs to be performed. X-rays or CT of the adrenals may also be done.[1] The best test for adrenal insufficiency of autoimmune origin, representing more than ninety percent of all cases in a Western population, is measurement of 21-hydroxylase autoantibodies.

Source of pathology CRH ACTH DHEA DHEA-S cortisol aldosterone renin Na K Causes5
hypothalamus
(tertiary)1
low low low low low3 low low low low tumor of the hypothalamus (adenoma), antibodies, environment (i.e. toxins), head injury
pituitary
(secondary)
high2 low low low low3 low low low low tumor of the pituitary (adenoma), antibodies, environment, head injury,
surgical removal6, Sheehan's syndrome
adrenal glands
(primary)7
high high high high low4 low high low high tumor of the adrenal (adenoma), stress, antibodies, environment, Addison's Disease, trauma, surgical removal (resection), miliary tuberculosis of the adrenal
1 Automatically includes diagnosis of secondary (hypopituitarism)
2 Only if CRH production in the hypothalamus is intact
3 Value doubles or more in stimulation
4 Value less than doubles in stimulation
5 Most common, does not include all possible causes
6 Usually because of very large tumor (macroadenoma)
7 Includes Addison's disease

Routine Investigations

Treatment

  • Adrenal crisis
    • Intravenous fluids
    • Intravenous steroid (Solu-Cortef/injectable hydrocortisone) later hydrocortisone, prednisone or methylpredisolone tablets
    • Rest
  • Mineralocorticoid deficiency (low aldosterone)
    • Fludrocortisone Acetate

(To balance sodium, potassium and increase water retention)

Prevention

Acute adrenal insufficiency is fatal. When patients with adrenal insufficiency experience the following stress, they must be very careful.

  • Surgery: People who are not currently taking glucocorticoids but who have taken long-term glucocorticoids in the past year, and people with chronic adrenal insufficiency should tell their doctor before surgery. These patients must be treated with intravenous glucocorticoids and saline before surgery and continues until the patient is fully awake after surgery and able to take medication by mouth.
  • Illness: Patients with adrenal insufficiency should know the essentiality of increasing medication during such periods of stress. During illness, oral dosing of glucocorticoid needs to be adjusted to mimic the normal response of the adrenal glands on the body. When significant fever or injury happen, the patients may require triple oral dosing. Once recovery from the stress event, doses can be returned to maintenance levels. Be careful, when the conditions of severe infections, vomiting, or diarrhea occur, these signs suggest the possibility of addisonian crisis.
  • Pregnancy: If nausea and vomiting in early pregnant woman with adrenal insufficiency interfere with taking medication by mouth, injections of the hormone may be necessary. During delivery, treatment is similar to that of people needing surgery.

See also

References

  1. 1.0 1.1 1.2 Eileen K. Corrigan (2007). "Adrenal Insufficiency (Secondary Addison's or Addison's Disease)". NIH Publication No. 90-3054.
  2. Adrenal+Insufficiency at the US National Library of Medicine Medical Subject Headings (MeSH)
  3. Ten S, New M, Maclaren N (2001). "Clinical review 130: Addison's disease 2001". J. Clin. Endocrinol. Metab. 86 (7): 2909–22. doi:10.1210/jc.86.7.2909. PMID 11443143.
  4. Brender E, Lynm C, Glass RM (2005). "JAMA patient page. Adrenal insufficiency". JAMA. 294 (19): 2528. doi:10.1001/jama.294.19.2528. PMID 16287965.
  5. "Dorlands Medical Dictionary:adrenal insufficiency".
  6. "Secondary Adrenal Insufficiency: Adrenal Disorders: Merck Manual Professional".
  7. "hypopituitary". 2006.
  8. http://www.endotext.org/adrenal/adrenal13/adrenal13.htm
  9. 9.0 9.1 Huebner A, Yoon SJ, Ozkinay F; et al. (2000). "Triple A syndrome--clinical aspects and molecular genetics". Endocr. Res. 26 (4): 751–9. PMID 11196451. Unknown parameter |month= ignored (help)
  10. Mosser J, Douar AM, Sarde CO; et al. (1993). "Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters". Nature. 361 (6414): 726–30. doi:10.1038/361726a0. PMID 8441467. Unknown parameter |month= ignored (help)
  11. Shashidhar PK, Shashikala GV (2012). "Low dose adrenocorticotropic hormone test and adrenal insufficiency in critically ill acquired immunodeficiency syndrome patients". Indian J Endocrinol Metab. 16 (3): 389–94. doi:10.4103/2230-8210.95680. PMC 3354846. PMID 22629505. Unknown parameter |month= ignored (help)
  12. Eledrisi MS, Verghese AC (2001). "Adrenal insufficiency in HIV infection: a review and recommendations". Am. J. Med. Sci. 321 (2): 137–44. PMID 11217816. Unknown parameter |month= ignored (help)
  13. Cocco C, Meloni A, Mariotti S; et al. (2012). "Novel neuronal and endocrine autoantibody targets in autoimmune polyendocrine syndrome type 1". Autoimmunity. 45 (6): 485–94. doi:10.3109/08916934.2012.680632. PMID 22506635. Unknown parameter |month= ignored (help)
  14. Osa SR, Peterson RE, Roberts RB (1981). "Recovery of adrenal reserve following treatment of disseminated South American blastomycosis". Am. J. Med. 71 (2): 298–301. PMID 6266250. Unknown parameter |month= ignored (help)
  15. Ardalan M, Shoja MM (2009). "Cytomegalovirus-induced adrenal insufficiency in a renal transplant recipient". Transplant. Proc. 41 (7): 2915–6. doi:10.1016/j.transproceed.2009.07.024. PMID 19765472. Unknown parameter |month= ignored (help)
  16. Mihalache A, Lamy O, Waeber G, Schneider A (2007). "[Adrenal insufficiency and hypercalcemia--an unusual presentation]". Praxis (Bern 1994) (in German). 96 (45): 1761–5. PMID 18050601. Unknown parameter |month= ignored (help)
  17. Kawamura M, Miyazaki S, Mashiko S; et al. (1998). "Disseminated cryptococcosis associated with adrenal masses and insufficiency". Am. J. Med. Sci. 316 (1): 60–4. PMID 9671046. Unknown parameter |month= ignored (help)
  18. Turan S, Hughes C, Atay Z; et al. (2012). "An atypical case of familial glucocorticoid deficiency without pigmentation caused by coexistent homozygous mutations in MC2R (T152K) and MC1R (R160W)". J. Clin. Endocrinol. Metab. 97 (5): E771–4. doi:10.1210/jc.2011-2414. PMC 3396854. PMID 22337906. Unknown parameter |month= ignored (help)
  19. Francke U, Harper JF, Darras BT; et al. (1987). "Congenital adrenal hypoplasia, myopathy, and glycerol kinase deficiency: molecular genetic evidence for deletions". Am. J. Hum. Genet. 40 (3): 212–27. PMC 1684111. PMID 2883886. Unknown parameter |month= ignored (help)
  20. Pedersen-Bjergaard U, Thorsteinsson B, Kirkegaard BC (1996). "[Pituitary function in hemochromatosis]". Ugeskr. Laeg. (in Danish). 158 (13): 1818–22. PMID 8650756. Unknown parameter |month= ignored (help)
  21. Pedreira CC, Savarirayan R, Zacharin MR (2004). "IMAGe syndrome: a complex disorder affecting growth, adrenal and gonadal function, and skeletal development". J. Pediatr. 144 (2): 274–7. doi:10.1016/j.jpeds.2003.09.052. PMID 14760276. Unknown parameter |month= ignored (help)
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ca:Insuficiència suprarenal de:Nebennierenrindeninsuffizienz he:תת פעילות בלוטת יותרת הכליה


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