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Adrenal insufficiency

Adrenal insufficiency (also known as Addison's disease) is a rare long-term endocrine disorder characterized by inadequate production of the steroid hormones cortisol and aldosterone by the two outer layers of the cells of the adrenal glands, causing adrenal insufficiency

Prevalence

1-5 / 10 000

33,100-165,500

US Estimated

51,350-256,750

Europe Estimated

Age of Onset

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ICD-10

E27.1

Inheritance

This condition does not appear to have a clear pattern of inheritance.

Rare View

A condition where the adrenal glands do not produce adequate amounts of steroid hormones, particularly cortisol, leading to symptoms such as fatigue, muscle weakness, and low blood pressure. It requires lifelong hormone replacement therapy.

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5 Facts you should know

FACT

1

Symptoms generally come on slowly and may include abdominal pain and gastrointestinal abnormalities, weakness, and weight loss

FACT

2

Other major symptoms include fatigue, gastrointestinal abnormalities, and pigmentation

FACT

3

It occurs most frequently in middle-aged females

FACT

4

Increased excretion of water and hypotension can lead to dehydration, some people have cravings for salty foods due to the loss of sodium

FACT

5

Severe infections, vomiting, or diarrhea can precipitate an Addisonian crisis

Interest over time

Adrenal insufficiency is also known as...

Adrenal insufficiency is also known as:

  • Addison disease
  • Adrenal hypoplasia
  • Hypoadrenocorticism familial
     

What’s your Rare IQ?

Which of the following are thought to be triggers of on Addisonian disease crisis?

Common signs & symptoms

Fatigue

Loss of appetite

Abdominal pain

Mood or behavior changes

Dark patches of skin (hyperpigmentation)

Muscle weakness or pain

Dehydration

Low blood pressure

Current treatments

Treatment for Adrenal insufficiency is focused on managing the symptoms. Treatment may include daily medicines that replace the adrenal hormones. Treatment for an adrenal crisis may include intravenous hydrocortisone, fluids, and electrolytes, as well as drugs that normalize blood pressure. People with Adrenal insufficiency should carry a medical ID that states the disease and emergency instructions.  Specialists involved in the care of someone with Adrenal insufficiency may include:

Endocrinologist

Radiologist

Immunologist

Top Clinical Trials

TitleDescriptionPhasesStatusInterventionsMore Information
Once-daily Oral Modified Release Hydrocortisone in Patients With Adrenal InsufficiencyThis is a randomised, controlled, open, two-armed, two-period cross-over, multi-centre phase II/III study to assess the safety, tolerability and pharmacokinetics of once-daily oral modified-release hydrocortisone in comparison to conventional thrice-daily oral hydrocortisone tablets in patients with adrenal insufficiencyPHASE2|PHASE3COMPLETEDDRUG: hydrocortisone (modified release), oral tablet 20 and 5 mg|DRUG: Hydrocortisone, oral tablet, 10 mgMore info
Chronocort Versus Plenadren Replacement Therapy in Adults With Adrenal InsufficiencyThis study is a double-blind, double-dummy, two-way cross-over, randomised, Phase II study to be conducted at approximately 6 investigational sites in 2 countries. The study will compare the efficacy, safety and tolerability of twice daily Chronocort, a modified-release hydrocortisone, with once daily Plenadren, a modified-release hydrocortisone, over a treatment period of up to 2 months in participants aged 18 years and over, diagnosed with primary Adrenal Insufficiency (AI).PHASE2COMPLETEDDRUG: Chronocort|DRUG: PlenadrenMore info
A Clinical Study to Assess the Efficacy and Safety of Leriglitazone in Adult Male Subjects With Cerebral AdrenoleukodystrophyA Clinical Study to Assess the Efficacy and Safety of Leriglitazone in Adults Male Subjects with Cerebral Adrenoleukodystrophy.PHASE3RECRUITINGDRUG: Leriglitazone|DRUG: PlaceboMore info
Study to Assess PXL065 in Subjects With Adrenomyeloneuropathy (AMN) Form of X-linked Adrenoleukodystrophy (X-ALD or ALD)A randomized open-label Phase 2a study to assess the pharmacokinetics and pharmacodynamic parameters of PXL065 after 12 weeks of treatment in male subjects with adrenomyeloneuropathy (AMN).PHASE2NOT_YET_RECRUITINGDRUG: PXL065More info
A Study to Evaluate Administration of SBT101 Gene Therapy in Adult Patients With Adrenomyeloneuropathy (AMN)This is a Phase 1/2 randomized, blinded, dose-escalation study to evaluate the safety and efficacy of intrathecal (IT) administration of SBT101, a recombinant adeno-associated virus serotype 9 (AAV9) containing a functional copy of the human adenosine triphosphate (ATP)-binding cassette transporter subfamily D member 1 (ABCD1; hABCD1) gene, in adult patients with adrenomyeloneuropathy (AMN) aged 18-65 years.


Patients will receive a single dose of SBT101 via IT route (or an imitation procedure) and will be followed for safety and efficacy for 2 years. Patients receiving SBT101 will be followed for an additional 3 years (5 total) for Safety. Patients receiving an imitation procedure will be offered the opportunity to receive SBT101 after 2 years, as data indicate.
PHASE1|PHASE2RECRUITINGGENETIC: SBT101|PROCEDURE: Imitation ProcedureMore info
Study to Assess PXL770 in Subjects With Adrenomyeloneuropathy (AMN) Form of X-linked Adrenoleukodystrophy (X-ALD or ALD)A randomized open-label Phase 2a study to assess the pharmacokinetics and pharmacodynamic parameters of PXL770 after 12 weeks of treatment in male subjects with adrenomyeloneuropathy (AMN).PHASE2NOT_YET_RECRUITINGDRUG: PXL770More info