From the Guidelines
A salt-wasting crisis is a life-threatening medical emergency characterized by excessive loss of sodium and water due to adrenal insufficiency, requiring immediate treatment with intravenous fluids and hydrocortisone. This condition occurs when the adrenal glands fail to produce adequate amounts of cortisol and aldosterone, hormones that regulate sodium retention and water balance [ 1 ]. Without sufficient aldosterone, the kidneys excrete too much sodium and water, leading to severe dehydration, low blood pressure, and electrolyte imbalances. Symptoms include vomiting, diarrhea, rapid weight loss, lethargy, weakness, and if untreated, can progress to shock and death.
Causes and Risk Factors
The most common cause of salt-wasting crisis is congenital adrenal hyperplasia (CAH), but it can also occur in patients with primary adrenal insufficiency (PAI) [ 1 ]. Stress, illness, or injury can trigger a crisis by increasing the body's cortisol requirements beyond what the compromised adrenal glands can produce.
Treatment and Management
Treatment requires immediate medical attention with intravenous fluids containing sodium chloride (normal saline), along with hydrocortisone (typically 100 mg as an initial dose, as recommended in [ 1 ]). Long-term management involves daily oral hydrocortisone and fludrocortisone to replace missing hormones [ 1 ]. Patients with known adrenal insufficiency should carry emergency medication and wear medical alert identification.
Prevention of Future Crises
To prevent future adrenal crises, it is essential to diagnose the precipitating causes leading to adrenal crisis and educate patients to increase steroid doses during intercurrent illnesses, vomiting, injuries, or other stressors [ 1 ]. A low salt consumption and chronic under-replacement with mineralocorticoid could be causes of recurrent adrenal crises. Whether or not poor compliance and underlying psychiatric disorders may be involved should be investigated.
Key Recommendations
- Immediate treatment with intravenous fluids and hydrocortisone is crucial in a salt-wasting crisis [ 1 ].
- Patients should carry emergency medication and wear medical alert identification [ 1 ].
- Long-term management involves daily oral hydrocortisone and fludrocortisone [ 1 ].
From the FDA Drug Label
In the event transient hypertension develops as a consequence of therapy, the dose should be reduced to 0.05 mg daily. Salt-Losing Adrenogenital Syndrome The recommended dosage for treating the salt-losing adrenogenital syndrome is 0.1 mg to 0.2 mg of fludrocortisone acetate tablets daily.
A salt-wasting crisis is not directly defined in the provided drug label. However, it can be inferred that a salt-wasting crisis may be related to the salt-losing adrenogenital syndrome, which is mentioned as a condition treated with fludrocortisone acetate tablets.
- The condition is characterized by the loss of salt.
- The treatment involves administering fludrocortisone acetate tablets, preferably in conjunction with a glucocorticoid. 2
From the Research
Definition of Salt-Wasting Crisis
- A salt-wasting crisis is a life-threatening condition that occurs in individuals with congenital adrenal hyperplasia (CAH) or other adrenal disorders, characterized by severe electrolyte imbalance, dehydration, and potentially fatal complications 3, 4, 5.
- It is often caused by a deficiency of the enzyme 21-hydroxylase, which is necessary for the production of aldosterone, a hormone that regulates electrolyte balance in the body 3, 5.
Symptoms and Diagnosis
- Symptoms of a salt-wasting crisis include poor feeding, lethargy, dehydration, and electrolyte imbalances, such as hyponatremia (low sodium levels) and hyperkalemia (high potassium levels) 3, 4.
- Diagnosis is often made through a combination of physical examination, laboratory tests, and genetic studies 3, 4, 6.
- Laboratory tests may show elevated levels of 17-hydroxyprogesterone, a precursor to aldosterone, and low levels of aldosterone itself 5, 6.
Treatment and Management
- Treatment of a salt-wasting crisis typically involves correction of electrolyte imbalances, administration of hydrocortisone and fludrocortisone, and management of dehydration 3, 4.
- In some cases, patients may require ongoing mineralocorticoid therapy to prevent future salt-wasting crises 7.
- Genetic studies can help confirm the diagnosis and guide treatment decisions 6.