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Waterhouse-Friderichsen Syndrome

MedGen UID:
53065
•Concept ID:
C0043068
•
Disease or Syndrome
Synonyms: Purpura Fulminans (Waterhouse-Friderichsen Syndrome); Waterhouse Friderichsen Syndrome; Waterhouse Friederichsen Syndrome; Waterhouse-Friederichsen Syndrome

Definition

A serious disorder characterized by massive adrenal gland hemorrhage secondary to a bacterial infection, most often Neisseria meningitidis infection. It is manifested with decreased blood pressure, shock, disseminated intravascular coagulation, and adrenocortical insufficiency. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVWaterhouse-Friderichsen Syndrome
Follow this link to review classifications for Waterhouse-Friderichsen Syndrome in Orphanet.

Professional guidelines

PubMed

The diagnosis and treatment of adrenal insufficiency.
McMILLAN CD, POWELL WN, HAINES RD
Med Clin North Am 1955 Jul;Cleveland No.:1111-25. doi: 10.1016/s0025-7125(16)34745-9. PMID: 14393163
Norepinephrine in the treatment of the Waterhouse-Friderichsen syndrome; report of a case.
CHOU TC, ANAGNOS SG
Med Ann Dist Columbia 1953 Nov;22(11):604-7. PMID: 13098452
Nor-epinephrine in the treatment of acute meningococcemia with shock (Waterhouse-Friderichsen syndrome) and an evaluation of adrenocortical function.
UHL HS
N Engl J Med 1953 Aug 6;249(6):229-32. doi: 10.1056/NEJM195308062490603. PMID: 13072798

Recent clinical studies

Etiology

A rare case of Waterhouse- Friderichsen syndrome during primary Varicella zoster infection.
Heitz AFN, Hofstee HMA, Gelinck LBS, Puylaert JB
Neth J Med 2017 Oct;75(8):351-353. PMID: 29219830
Overwhelming postsplenectomy infection syndrome in adults - a clinically preventable disease.
Okabayashi T, Hanazaki K
World J Gastroenterol 2008 Jan 14;14(2):176-9. doi: 10.3748/wjg.14.176. PMID: 18186551Free PMC Article
Waterhouse-Friderichsen syndrome after infection with group A streptococcus.
Karakousis PC, Page KR, Varello MA, Howlett PJ, Stieritz DD
Mayo Clin Proc 2001 Nov;76(11):1167-70. doi: 10.4065/76.11.1167. PMID: 11702906
Treatment of thromboembolic complications of fulminant meningococcal septic shock.
Mele JA 3rd, Linder S, Capozzi A
Ann Plast Surg 1997 Mar;38(3):283-90. doi: 10.1097/00000637-199703000-00017. PMID: 9088469
Postoperative adrenal hemorrhage.
Clark OH
Ann Surg 1975 Aug;182(2):124-9. doi: 10.1097/00000658-197508000-00007. PMID: 1211987Free PMC Article

Diagnosis

Waterhouse-Friderichsen syndrome, septic adrenal apoplexy.
Rijal R, Kandel K, Aryal BB, Asija A, Shrestha DB, Sedhai YR
Vitam Horm 2024;124:449-461. Epub 2023 Nov 8 doi: 10.1016/bs.vh.2023.06.001. PMID: 38408808
Illustrated Forensic Pathology: Fatal Waterhouse-Friderichsen Syndrome.
Abouzahir H, Nya S, Benyaich H
Am J Forensic Med Pathol 2020 Sep;41(3):e58-e60. doi: 10.1097/PAF.0000000000000581. PMID: 32796209
Waterhouse-Friderichsen Syndrome in an Infant.
Hawi N, Liodakis E, Sasse M
Dtsch Arztebl Int 2020 Feb 28;117(9):147. doi: 10.3238/arztebl.2020.0147b. PMID: 32234194Free PMC Article
Fatal pneumococcal Waterhouse-Friderichsen syndrome.
Doherty S
Emerg Med (Fremantle) 2001 Jun;13(2):237-9. doi: 10.1046/j.1442-2026.2001.00219.x. PMID: 11482865
Waterhouse Friderichsen syndrome.
Zavell PM
Mich Med 1975 Dec;74(36):758. PMID: 1207489

Therapy

When von Willebrand disease comes into age - a matter of change?
Miesbach W, Berntorp E
Eur J Haematol 2011 Jun;86(6):496-501. doi: 10.1111/j.1600-0609.2011.01611.x. PMID: 21418329
Overwhelming postsplenectomy infection syndrome in adults - a clinically preventable disease.
Okabayashi T, Hanazaki K
World J Gastroenterol 2008 Jan 14;14(2):176-9. doi: 10.3748/wjg.14.176. PMID: 18186551Free PMC Article
Community-acquired Acinetobacter meningitis in adults.
Chang WN, Lu CH, Huang CR, Chuang YC
Infection 2000 Nov-Dec;28(6):395-7. doi: 10.1007/s150100070013. PMID: 11139162
Treatment of thromboembolic complications of fulminant meningococcal septic shock.
Mele JA 3rd, Linder S, Capozzi A
Ann Plast Surg 1997 Mar;38(3):283-90. doi: 10.1097/00000637-199703000-00017. PMID: 9088469
Postoperative adrenal hemorrhage.
Clark OH
Ann Surg 1975 Aug;182(2):124-9. doi: 10.1097/00000658-197508000-00007. PMID: 1211987Free PMC Article

Prognosis

Waterhouse-Friderichsen syndrome, septic adrenal apoplexy.
Rijal R, Kandel K, Aryal BB, Asija A, Shrestha DB, Sedhai YR
Vitam Horm 2024;124:449-461. Epub 2023 Nov 8 doi: 10.1016/bs.vh.2023.06.001. PMID: 38408808
Illustrated Forensic Pathology: Fatal Waterhouse-Friderichsen Syndrome.
Abouzahir H, Nya S, Benyaich H
Am J Forensic Med Pathol 2020 Sep;41(3):e58-e60. doi: 10.1097/PAF.0000000000000581. PMID: 32796209
Waterhouse-Friderichsen Syndrome in an Infant.
Hawi N, Liodakis E, Sasse M
Dtsch Arztebl Int 2020 Feb 28;117(9):147. doi: 10.3238/arztebl.2020.0147b. PMID: 32234194Free PMC Article
Fatal pneumococcal Waterhouse-Friderichsen syndrome.
Doherty S
Emerg Med (Fremantle) 2001 Jun;13(2):237-9. doi: 10.1046/j.1442-2026.2001.00219.x. PMID: 11482865
Reminder--necrotizing fasciitis complicated by Waterhouse-Friderichsen syndrome.
Dunstan SP
Br J Oral Maxillofac Surg 1999 Jun;37(3):217-8. doi: 10.1054/bjom.1998.0155. PMID: 10454031

Clinical prediction guides

Immunoadsorption therapy for a meningococcemia patient with myocarditis, adrenal hemorrhage, and purpura fulminans: a case report.
Akcay N, Kihtir HS, Ozcelik G, Barlas UK, Petmezci MT, Sevketoglu E
Braz J Anesthesiol 2022 Nov-Dec;72(6):819-822. Epub 2021 Jul 17 doi: 10.1016/j.bjane.2021.06.021. PMID: 34284056Free PMC Article
Atriobronchial Fistula Complicated by Septic Cerebral Air Emboli After Pulmonary Vein Ablation.
Velghe D, Apers T, Devriendt S, Deblier I, Hendriks B, Nieuwendijk R, Rogiers P
Crit Care Med 2017 Aug;45(8):e867-e871. doi: 10.1097/CCM.0000000000002438. PMID: 28441232
Overwhelming postsplenectomy infection syndrome in adults - a clinically preventable disease.
Okabayashi T, Hanazaki K
World J Gastroenterol 2008 Jan 14;14(2):176-9. doi: 10.3748/wjg.14.176. PMID: 18186551Free PMC Article
Cardiac rescue with enoximone in volume and catecholamine refractory septic shock.
Ringe HI, Varnholt V, Gaedicke G
Pediatr Crit Care Med 2003 Oct;4(4):471-5. doi: 10.1097/01.PCC.0000074275.61913.72. PMID: 14525645
Sepsis-associated purpura fulminans in adults.
Gamper G, Oschatz E, Herkner H, Paul G, Burgmann H, Janata K, Röggla M, Laggner AN
Wien Klin Wochenschr 2001 Feb 15;113(3-4):107-12. PMID: 11253735

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