Waterhouse-Friderichsen syndrome

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Waterhouse-Friderichsen syndrome

Pathology

Adrenal gland disorders

Congenital adrenal hyperplasia

Primary adrenal insufficiency

Waterhouse-Friderichsen syndrome

Hyperaldosteronism

Adrenal cortical carcinoma

Cushing syndrome

Conn syndrome

Thyroid gland disorders

Thyroglossal duct cyst

Hyperthyroidism

Graves disease

Thyroid eye disease (NORD)

Toxic multinodular goiter

Thyroid storm

Hypothyroidism

Euthyroid sick syndrome

Hashimoto thyroiditis

Subacute granulomatous thyroiditis

Riedel thyroiditis

Postpartum thyroiditis

Thyroid cancer

Parathyroid gland disorders

Hyperparathyroidism

Hypoparathyroidism

Hypercalcemia

Hypocalcemia

Pancreatic disorders

Diabetes mellitus

Diabetic retinopathy

Diabetic nephropathy

Pituitary gland disorders

Hyperpituitarism

Pituitary adenoma

Hyperprolactinemia

Prolactinoma

Gigantism

Acromegaly

Hypopituitarism

Growth hormone deficiency

Pituitary apoplexy

Sheehan syndrome

Hypoprolactinemia

Constitutional growth delay

Diabetes insipidus

Syndrome of inappropriate antidiuretic hormone secretion (SIADH)

Gonadal dysfunction

Precocious puberty

Delayed puberty

Premature ovarian failure

Polycystic ovary syndrome

Androgen insensitivity syndrome

Kallmann syndrome

5-alpha-reductase deficiency

Polyglandular syndromes

Autoimmune polyglandular syndrome type 1 (NORD)

Endocrine tumors

Multiple endocrine neoplasia

Pancreatic neuroendocrine neoplasms

Zollinger-Ellison syndrome

Carcinoid syndrome

Pheochromocytoma

Neuroblastoma

Opsoclonus myoclonus syndrome (NORD)

Endocrine system pathology review

Adrenal insufficiency: Pathology review

Adrenal masses: Pathology review

Hyperthyroidism: Pathology review

Hypothyroidism: Pathology review

Thyroid nodules and thyroid cancer: Pathology review

Parathyroid disorders and calcium imbalance: Pathology review

Diabetes mellitus: Pathology review

Cushing syndrome and Cushing disease: Pathology review

Pituitary tumors: Pathology review

Hypopituitarism: Pathology review

Diabetes insipidus and SIADH: Pathology review

Multiple endocrine neoplasia: Pathology review

Neuroendocrine tumors of the gastrointestinal system: Pathology review

Assessments

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?

External References

First Aid

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Adrenal hemorrhage p. 716

Waterhouse-Friderichsen syndrome p. 355

Disseminated intravascular coagulation (DIC) p. 435

Waterhouse-Friderichsen syndrome p. 355, 716

Fever

Waterhouse-Friderichsen syndrome p. 140

Hypotension

Waterhouse-Friderichsen syndrome p. 716

Neisseria meningitidis

Waterhouse-Friderichsen syndrome p. 355

Septicemia

Waterhouse-Friderichsen syndrome p. 355

Shock p. 319

Waterhouse-Friderichsen syndrome and p. 355

Waterhouse-Friderichsen syndrome p. 355

meningococci p. 140

presentation p. 716

External Links

Transcript

Content Reviewers

Rishi Desai, MD, MPH

Contributors

Brittany Norton, MFA

Jahnavi Narayanan, MBBS

Tanner Marshall, MS

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.

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)
Elsevier

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