Insuffisance surrénale aiguë. Authors; Authors and affiliations. Aude Mariani Ecochard. Aude Mariani Ecochard. There are no affiliations available. Chapter. l’insuffisance surrénale aiguë. Jérôme Bertherat, Paris. 10hh Diagnostic de l’insuffisance surrénale: facile ou difficile? Antoine Tabarin, Bordeaux. Request PDF on ResearchGate | On Oct 1, , M. Molimard and others published Insuffisance surrénalienne aiguë et hypercorticisme induits par des.

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Acute adrenal insufficiency AAI is a rare but severe condition caused by a sudden defective production of adrenal steroids cortisol and aldosterone. Clinical description The disease may occur at any age. The disease may occur at any age. If untreated, shock and bilateral adrenal hemorrhage can rapidly lead to death. AAI may also result from corticotroph insufficiency, either isolated or more often resulting from complete anterior pituitary insufficiency.

Acute adrenal failure Acute adrenocortical insufficiency Addisonian crisis Adrenal crisis Adrenocortical crisis Prevalence: Laboratory exams show signs of adrenal insufficiency hypoglycemia, hyponatremia and elevated natriuresis, hyperkaliemia, hemoconcentration, hypochloremic metabolic acidosis and functional renal failure confirmed by hypocortisolemia, increased ACTH, and an insufficient response to rapid ACTH stimulation testing that leads to the diagnosis of absolute and peripheral AAI.

Disease definition Acute adrenal insufficiency AAI is a rare but severe condition caused by a sudden defective production of adrenal steroids cortisol and aldosterone. Prognosis varies depending on the etiologies, but is generally correlated with the rapidity of diagnosis and medical assistance.


Administration of mg hydrocortisone i. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted.

It represents an emergency, thus the rapid recognition and prompt therapy are critical for survival even before the diagnosis is made.

Death is rare when the patients receive appropriate medical assistance. Management and treatment Immediate treatment in an intensive care unit inzuffisance necessary. Check this box if you wish to receive a copy of your message. Adrenal destruction may occur in the absence of CPAI history and may be due to bilateral massive adrenal hemorrhage BMAH; see this term as seen in Waterhouse-Friderichsen syndrome see this term.

During this time cardiac monitoring is essential. Other search option s Alphabetical list.

Affections surrénaliennes aiguës – EM|consulte

The documents contained in this web site are presented for information purposes only. The initial presentation may be limited to abdominal pain, nausea, vomiting and fever. Peritonitis is often a differential diagnosis as well as other causes of adrenal destruction such as bilateral adrenalectomy, Waterhouse-Friderichsen syndrome see this termautoimmune adrenalitis, infectious adrenalitis and tumour infiltration.

For all other comments, please send your remarks via contact us. Summary Epidemiology Exact prevalence of acute adrenal insufficiency is unknown. Patients and their families should also be educated on what to do during an adrenal crisis. Specialised Social Services Eurordis directory. The mineralocorticoid insufficiency, when present, can be confirmed by low aldosterone levels and high surrenalidnne renin activity PRA.


Health care resources for this disease Expert centres Diagnostic tests 0 Patient organisations 5 Orphan drug s 4. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.

In case of anterior pituitary insufficiency, ACTH is low. Differential diagnosis Secondary adrenal insufficiency needs to be eliminated.

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Antibiotics, vasopressors, heparin, packed red blood cells, platelets, cryoprecipitates and fresh frozen plasma are also administered if needed. Secondary adrenal insufficiency needs to be eliminated.

Preventive strategies include dosage increase of steroid hormones during times of stress in those with CPIA. Diagnostic methods The clinical signs are nonspecific but the diagnosis of AAI is suspected if a patient presents with hypotonia or shock that responds poorly to catecholamines. Steroid withdrawal is the most common cause of AAI in patients with chronic adrenal insufficiency. Adrenal destruction may be associated with autoimmune adrenalitis Addison disease; see this termisolated or in the context of autoimmune polyendocrinopathy type 1, 2 or 4 see these terms.

Immediate treatment in an intensive care unit is necessary.

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