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Waterhouse-Friderichsen syndrome

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Waterhouse-Friderichsen syndrome

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Questions

USMLE® Step 1 style questions USMLE

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A 21-year-old female is brought to the emergency department by EMS after being found unresponsive in her apartment. An accompanying friend recalls the patient was at home sick for several days. The patient is unable to provide any medical history. Temperature is 38.9°C (102°F), pulse is 130/min, respirations are 22/min, blood pressure is 76/52 mmHg, and oxygen saturation saturation is 93% on room air. Physical examination is notable for a diffuse purpuric rash and cool, clammy skin. The patient’s IV, which was established en route, begins to bleed spontaneously. She is aggressively resuscitated with IV fluids, antibiotics, and peripheral vasopressors without improvement in her clinical status. Initial laboratory findings are demonstrated below:  



Which of the following organisms is most likely implicated in this patient’s presentation?

Memory Anchors and Partner Content

External References

First Aid

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Adrenal hemorrhage p. 722

Waterhouse-Friderichsen syndrome p. 353

Disseminated intravascular coagulation (DIC) p. 433

Waterhouse-Friderichsen syndrome p. 353, 722

Fever

Waterhouse-Friderichsen syndrome p. 140

Hypotension

Waterhouse-Friderichsen syndrome p. 722

Neisseria meningitidis

Waterhouse-Friderichsen syndrome p. 353

Septicemia

Waterhouse-Friderichsen syndrome p. 353

Shock p. 317

Waterhouse-Friderichsen syndrome and p. 353

Waterhouse-Friderichsen syndrome p. 353

meningococci p. 140

presentation p. 722

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Transcript

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Content Reviewers

Rishi Desai, MD, MPH

Waterhouse-Friderichsen syndrome occurs when the blood vessels in the adrenal gland rupture during a severe bacterial infection, turning the adrenal glands into sacks of blood.

This results in adrenal crisis, or acute adrenal insufficiency, which is when the adrenal gland suddenly stops producing hormones.

The syndrome is named after two physicians - Waterhouse and Friderichsen who separately described the syndrome back in the early 1900’s.

Now, there are two adrenal glands, one above each kidney, and each one has an inner layer called the medulla and an outer layer called the cortex.

The adrenal cortex is subdivided into three more layers, the zona glomerulosa, zona fasciculata, and the zona reticularis.

The adrenal cortex secretes hormones under the control of adrenocorticotropic hormone, released by pituitary gland.

The outermost layer is the zona glomerulosa, which makes the hormone aldosterone.

Aldosterone acts on the nephrons of the kidney, and decrease potassium levels, increase sodium levels, and increase blood volume and blood pressure.

The middle layer is the zona fasciculata, which makes the hormone cortisol, as well as other glucocorticoids.

The main job of glucocorticoid is to increase blood glucose levels, especially when there’s emotional and physical stress.

Finally, the innermost layer is the zona reticularis, which makes a group of sex hormones called androgens.

In men, androgen stimulates development of male reproductive tissues and secondary sex characteristics like facial hair and a large Adam’s apple.

In women, androgen causes a growth spurt, and growth of underarm and pubic hair during puberty.

The adrenal gland gets blood through three main arteries- the superior, middle, and inferior suprarenal arteries.

All three divide into branches that supply the adrenal cortex and the adrenal medulla.

Summary

Waterhouse-Friderichsen syndrome is a rare and potentially life-threatening condition that occurs when the adrenal glands fail and produce insufficient amounts of cortisol and other hormones. This condition is usually caused by an overwhelming bacterial infection, most commonly meningococcal septicemia, but it can also be caused by other bacterial infections.

The characteristic symptom of the syndrome is a petechial rash throughout the body, which can turn into large purpura over time. Another potential complication of this syndrome is an Addisonian crisis, which can cause sudden lower back, abdominal or leg pain, vomiting, diarrhea, and low blood pressure leading to loss of consciousness. Other symptoms include fever and chills, which can be a sign of sepsis.

Waterhouse-Friderichsen syndrome is a medical emergency and requires immediate treatment with antibiotics to treat the underlying bacterial infection, as well as glucocorticoids to help support the person's blood pressure and metabolic needs. Other supportive care measures may also be necessary, such as IV fluids, oxygen therapy, and monitoring of vital signs.

Sources

  1. "Robbins Basic Pathology" Elsevier (2017)
  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
  5. "<i>Staphylococcus aureus</i>Sepsis and the Waterhouse–Friderichsen Syndrome in Children" New England Journal of Medicine (2005)
  6. "Waterhouse-Friderichsen syndrome without purpura due to Haemophilus influenzae group B." Postgraduate Medical Journal (1985)