Adrenal gland and paraganglia - Waterhouse-Friderichsen syndrome

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Adrenal gland and paraganglia

Adrenal insufficiency

Waterhouse-Friderichsen syndrome

Reviewer: Nat Pernick, M.D. (see Reviewers page)
Revised: 6 February 2013, last major update February 2005
Copyright: (c) 2002-2013, PathologyOutlines.com, Inc.

General
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● Hemorrhagic necrosis of adrenal glands, usually due to bacteremia, classically Neisseria meningitides; also Pseudomonas aeruginosa, pneumococci, staphylococcus and historically Haemophilus influenzae
● Less common causes are burns, cardiac failure, hypothermia and birth trauma

Clinical features
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● More common in children, particularly before age 2 years
● Usually bilateral; newborns may have unilateral hemorrhage, more commonly in right adrenal gland
● Symptoms: shock, disseminated intravascular coagulation and adrenal insufficiency; the shock may cause the hemorrhage
● Meningococcemia (Arch Pathol Lab Med 1977;101:6)

Case reports
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● 47 year old woman with Capnocytophaga canimorsus septicemia (Arch Pathol Lab Med 2000;124:859)
● Male newborn with Echovirus type 6 infection, otherwise healthy (Mod Pathol 2001;14:85)

Treatment
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● Treat underlying infection with antibiotics
● Also cortisol, electrolytes
● Must detect and treat quickly

Gross description
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● Glands are enlarged and hemorrhagic with extensive cortical and medullary necrosis

Gross images
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Red-brown adrenal glands due to hemorrhage

Fatal meningococcemia in 10 month old boy, courtesy Dr. Eric L. Vey, Erie County Coroner’s Officer, Erie, PA (USA):

Adrenal glands in situ Normal (top) and hemorrhagic (bottom) adrenal glands

Micro description
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● Hemorrhage, necrosis, fibrin deposition, neutrophilic infiltration of medulla and cortex
● Zona glomerulosa cells may be spared

Micro images
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Due to Capnocytophaga canimorsus

Differential diagnosis
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● Central adrenal vein thrombosis

End of Adrenal gland and paraganglia > Adrenal insufficiency > Waterhouse-Friderichsen syndrome

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