Waterhouse-friderichsen syndrome

Waterhouse-Friderichsen syndrome is a severe and often fatal condition characterized by acute adrenal gland failure due to hemorrhage into the adrenal glands. This syndrome is most commonly associated with severe bacterial infections, particularly those caused by Neisseria meningitidis.

Pathophysiology

Waterhouse-Friderichsen syndrome results from the overwhelming sepsis that leads to disseminated intravascular coagulation (DIC) and subsequent adrenal hemorrhage. The adrenal glands are crucial for producing cortisol, a hormone essential for stress response, and their failure leads to a rapid decline in blood pressure and shock.

Causes

The primary cause of Waterhouse-Friderichsen syndrome is severe bacterial infection, most notably meningococcal septicemia caused by Neisseria meningitidis. Other bacteria such as Streptococcus pneumoniae, Haemophilus influenzae, and Staphylococcus aureus can also be responsible.

Symptoms

The symptoms of Waterhouse-Friderichsen syndrome include:

• Sudden onset of fever
• Petechiae and purpura
• Hypotension
• Shock
• DIC
• Adrenal insufficiency

Diagnosis

Diagnosis is typically based on clinical presentation and confirmed by laboratory tests showing adrenal insufficiency, DIC, and the presence of the causative organism in blood cultures. Imaging studies such as CT scan or MRI may reveal adrenal hemorrhage.

Treatment

Immediate treatment is critical and includes:

• Broad-spectrum antibiotics
• Intravenous fluids and vasopressors to manage shock
• Corticosteroid replacement therapy to address adrenal insufficiency

Prognosis

The prognosis of Waterhouse-Friderichsen syndrome is poor, with a high mortality rate if not treated promptly. Early recognition and aggressive management are essential for improving outcomes.

History

The syndrome is named after Rupert Waterhouse and Carl Friderichsen, who first described the condition in the early 20th century.

Related Pages

• Adrenal gland
• Sepsis
• Disseminated intravascular coagulation
• Neisseria meningitidis
• Adrenal insufficiency
• Corticosteroid