Waterhouse-Friderichsen Syndrome
Classification according to ICD-10 | |
---|---|
A39.1 + | Waterhouse-Friderichsen syndrome in meningococcal infection |
E35.1 * | Diseases of the adrenal glands in diseases classified elsewhere |
ICD-10 online (WHO version 2019) |
The Waterhouse-Friderichsen syndrome , also known as adrenal apoplexy or Suprarenal apoplexy called, is an acute failure of the adrenal glands due to massive bacterial infections and a special form of coagulation .
The rare disease, which occurs mainly in children, was first described in 1886 by Felix Marchand and in 1894 by Arthur Francis Voelcker (1861-1946). It was described as an independent disease in 1911 by Rupert Waterhouse and worked out in 1918 by the Danish pediatrician Carl Friderichsen (1886–1979).
etiology
The Waterhouse-Friderichsen syndrome can occur in severe bacterial infections such as meningococci , Haemophilus influenzae or pneumococci and mostly affects children and young adults, but also patients after splenectomy as part of an OPSI syndrome .
Waterhouse-Friderichsen syndrome occurs in about 15% of patients with meningococcal sepsis and leads to death in up to 90% of cases ; if left untreated, it is always fatal.
Pathogenesis
Some of the pathogens (gram-negative pathogens such as meningococci) release endotoxins when they break down , which on the one hand lead to a circulatory shock with activation of the coagulation system and on the other hand also activate the coagulation directly via mediators . The result is a massive formation of thrombi in blood vessels with an undersupply of peripheral flow areas. ( Sanarelli-Shwartzman reaction )
During this coagulation, all of the coagulation factors in the blood are consumed, which leads to profuse bleeding in the skin ( purpura ), mucous membranes and internal organs . The adrenal cortex , which is destroyed, is particularly affected . This leads to an acute deficiency in the hormone cortisol .
The kidneys can also be affected by the thrombi and by products of fibrinolysis , so that patients may have to be dialyzed .
clinic
Signs of shock such as
as well as fever , diarrhea , confusion, circulatory collapse, respiratory failure, coma , neck stiffness and disseminated intravascular coagulation (DIC).
Diagnosis
- Blood count (regarding thrombocytopenia )
- Quick value / Partial Thromboplastin Time (PTT)
- D dimers
- Serum - cortisone -mirror
- Plasma - ACTH
- Sonography of the adrenal glands
- always consider this in case of fever and diarrhea and petechiae
therapy
- Immediate hydrocortisone therapy (continuous and high-dose, e.g. 100 mg hydrocortisone initial bolus , then 10 mg / h syringe pump )
- Mineralocorticoids
- Catecholamines (dobutamine, noradrenaline )
- Administration of penicillin G and cefotaxime
- ventilation if necessary
Without immediate therapy, almost 100% of patients die.
literature
- J. Varon, K. Chen, GL Sternbach: Rupert Waterhouse and Carl Friderichsen: adrenal apoplexy. In: J Emerg Med. Volume 16, No. 5, July-August 1998, pp. 643-647, PMID 9696186 .
- Ludwig Weissbecker: Adrenal Insufficiency. In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition ibid. 1961, pp. 1016-1021, here: pp. 1019 f. ( The Waterhouse-Friderichsen Syndrome and other acute adrenal gland damage ).
Web links
- whonamedit.com
- Young woman dies shortly after examination. Großhadern Clinic in Munich. In: merkur-online.de , February 23, 2012
- When fate makes a point . Radio feature, Saarländischer Rundfunk, October 29, 2017