Waterhouse-Friderichsen syndrome, septic adrenal apoplexy.

Waterhouse-Friderichsen syndrome is a rare but potentially fatal disorder of the adrenal gland characterized by bilateral adrenal hemorrhage. It is classically a result of meningococcal sepsis and presents acutely with features of shock, petechial rashes, abdominal pain, and non-specific symptoms such as headache, fatigue, and vomiting. Treatment consists of fluid resuscitation, corticosteroid replacement, and possibly surgery. The prognosis is poor despite treatment. This chapter will review the etiology, pathogenesis, clinical features, and management of the disease.

Three Autopsy Cases of Non-Meningococcal Waterhouse-Friderichsen Syndrome with Hypoplastic Spleen or Post-Splenectomy Status.

We report three cases of Waterhouse-Friderichsen syndrome (WFS) that were confirmed during forensic autopsies. Case 1 involved a man in his 50s post-splenectomy. Bacteriological examination revealed Streptococcus pneumoniae (S. pneumonia). The patient was considered to have died of asphyxiation after aspirating vomit. Case 2 involved a man in his 40s. Bacteriological examination again revealed S. pneumoniae. Histopathological examination showed hypoplasia of the spleen. This patient was considered to have died of multiple-organ failure due to sepsis, disseminated intravascular coagulation, and WFS. Case 3 involved a post-splenectomy woman in her 60s with a history of systemic lupus erythematosus. Bacteriological examination revealed Streptococcus oralis. This patient was considered to have died of multiple-organ failure due to sepsis, disseminated intravascular coagulation, and WFS. These three cases were included among forensic autopsies conducted in the last 5 years. WFS has been considered a rare disease, but may be more frequent than previously assumed. If a mildly ill patient displays a sudden change in status and dies within a short period of time, we consider it necessary to perform not only bacteriological examinations, but also histopathological examination of the spleen during autopsy.

Waterhouse-Friderichsen Syndrome with Bilateral Adrenal Hemorrhage Associated with Methicillin-Resistant Staphylococcus aureus (MRSA) Bacteremia in an Adult Patient with History of Intravenous Drug Use.

BACKGROUND Waterhouse-Friderichsen syndrome, also known as acute adrenal insufficiency due to adrenal gland hemorrhage, is an uncommon and frequently fatal condition classically presenting with fever, shock, rash, and coagulopathy. Although most often associated with Meningococcemia, many other etiologies have been implicated, including reports of Staphylococcus aureus infection on autopsy examinations. This report details an adult intravenous drug user with adrenal hemorrhage associated with methicillin-resistant Staphylococcus aureus (MRSA) bacteremia. CASE REPORT A 58-year-old man with a history of intravenous drug use presented to the hospital with weakness. Vitals were initially normal and exam findings were notable for decreased right-sided motor strength. Magnetic resonance imaging (MRI) revealed a cervical epidural abscess with spinal cord compression. Despite initiation of broad-spectrum antibiotics and intravenous fluids, the patient progressed to shock, requiring vasopressor administration, and his blood cultures later grew MRSA. Further imaging of the abdomen/pelvis was completed, revealing bilateral adrenal hemorrhage. Random cortisol at that time was 5.6 µg/dL, confirming a diagnosis of critical illness-related corticosteroid insufficiency in addition to likely septic and spinal shock. The patient was initiated on hydrocortisone with improvement in his hypotension. He was transitioned to prednisone and fludrocortisone in addition to 8 weeks of antibiotics after achieving clinical stability. CONCLUSIONS This report brings to attention the risk of adrenal hemorrhage and acute adrenal insufficiency as a sequela of the relatively common illness of Staphylococcus aureus bacteremia. As symptoms of adrenal insufficiency can overlap with septic shock related to the primary condition, this diagnosis requires a high index of suspicion in the critically ill patient.

Fatal Waterhouse-Friderichsen syndrome in an adult due to serogroup Y Neisseria meningitidis.

Waterhouse-Friderichsen syndrome (WFS), defined as severe adrenal insufficiency due to bilateral adrenal gland haemorrhagic necrosis, occurred in a 59-year-old woman. An underlying serogroup Y Neisseria meningitidis (NM) infection was diagnosed, with a rapid progression to purpura fulminans, disseminated intravascular coagulation and WFS. Intensive treatment including fluid resuscitation, broad-spectrum antibiotic therapy, ventilatory support, platelet and factor replacement were administered. The meningococcaemia in the presence of WFS had a fulminant progression, leading to a fatal outcome within 24 hours of symptom onset. This case details the diagnosis and management challenges of the WFS, a rare complication of NM septicaemia, and describes the identification of a NM serogroup that is rare in Portugal in middle-aged patients.

[The Waterhouse-Friderichsen syndrome]. / Het syndroom van Waterhouse-Friderichsen.

BACKGROUND: The Waterhouse-Friderichsen syndrome (WFS) is a serious illness associated with a high mortality rate and characterized by septic shock and signs of adrenocortical insufficiency. CASE DESCRIPTION: A 33-year-old male was seen in the emergency department with severe abdominal and back pain with diffuse mottled skin and rapidly progressive petechiae all over his body. Laboratory results showed severe lactate acidosis with renal dysfunction and indications of diffuse intravascular coagulation. Because he had signs of progressive septic shock, the patient was admitted to the ICU. There he subsequently developed hypoglycaemia (glucose < 0.1 mmol/l) and CPR had to be performed twice - the patient died shortly afterwards. Autopsy showed bilateral necrosis and haemorrhage of the adrenal glands, indicative of the diagnosis of WFS. Streptococcus pneumoniae was identified. CONCLUSION: In case of sepsis, with fever, rapidly expanding petechiae and purpura the Waterhouse-Friderichsen syndrome should be considered. Intensive therapy with antibiotics, fluids, vasopressors, and corticosteroids should be initiated immediately.

A case report of spontaneous staphylococcal meningitis in a cynomolgus monkey.

This report describes a suppurative meningitis in a young cynomolgus. The animal had neutrophil aggregation in the subarachnoid space and hemorrhage in bilateral adrenal glands. Staphylococcus was identified by FISH in brain. To our knowledge, this is the first case of staphylococcal meningitis with Waterhouse-Friderichsen syndrome in a cynomolgus monkey.

A rare case of Waterhouse- Friderichsen syndrome during primary Varicella zoster infection.

Primary Varicella zoster virus infection in adults is associated with a higher risk of complications when compared with the benign disease course of primary infection during childhood. We present a rare complication of adult primary Varicella zoster in the form of acute, irreversible adrenal insufficiency due to bilateral adrenal haemorrhage, which is also known as the WaterhouseFriderichsensyndrome.

Streptococcus Pneumoniae Detection Long Time After Death in a Fatal Case of Waterhouse-Friderichsen Syndrome.

We report a fatal case of Waterhouse-Friderichsen syndrome in a 64-year-old man. The diagnosis, suspected during the autopsy (performed 63 hours after death), was confirmed through the successful detection of Streptococcus pneumoniae DNA and antigens in samples (blood and liver) collected during the autopsy. These results conformed with blood cultures performed antemortem, which became available only the day after the autopsy. The case underlines the need to collect biological material (liver and blood samples) during autopsy for microbiological investigations, although the collection is performed a long time after the death, suggesting that a liver sample works for DNA and liver and blood work for Streptococcus pneumoniae antigen detection.

Púrpura y necrosis de rápida instauración en paciente con fiebre / Purpura and necrosis of sudden onset in a patient with fever

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Muerte súbita por sepsis meningocócica: diagnóstico post mórtem / Sudden death from meningococcal sepsis: postmortem diagnosis

La presentación más común de la enfermedad meningocócica es la meningitis, con una tasa de incidencia de 0,92 por 100.000 habitantes y una letalidad del 40,3% en España. Un cuadro meníngeo puede cursar con síntomas inespecíficos como la cefalea, entre otros, de tal forma que en ocasiones progresa rápidamente en cuestión de horas y provoca la muerte del paciente antes de haber recibido una atención médica adecuada. Además de la meningitis clásica, el meningococo produce una enfermedad sistémica que incluye la sepsis meningocócica y la coagulopatía intravascular diseminada. En pacientes con sepsis meningocócica se ha descrito el síndrome de Waterhouse-Friderichsen, que se caracteriza por hemorragia suprarrenal bilateral, coagulación intravascular diseminada e hígado de shock, y que produce una bacteriemia grave. Esta combinación provoca unshock fulminante y, si no se trata, puede producir muerte súbita. Presentamos el caso de una paciente con odinofagia, fiebre, vómitos y deposiciones diarreicas de 24 horas de evolución, que a pesar de recibir asistencia médica ambulatoria y hospitalaria fallece como consecuencia de una sepsis fulminante por Neisseria meningitidis y síndrome de Waterhouse-Friderichsen no diagnosticado clínicamente (AU)

The most common presentation of meningococcal disease is meningitis, with an incidence rate of 0.92 per 100,000 inhabitants and a mortality rate of 40.3% in Spain. Meningeal disease may present with nonspecific symptoms such as headache, among others, sometimes progressing rapidly to a fatal outcome before the patient receives adequate medical care. Besides classical meningitis, Neisseria meningitis produces a systemic disease that includes meningococcal sepsis and disseminated intravascular coagulopathy. In patients with meningococcal sepsis the Waterhouse-Friderichsen syndrome is described, which is characterized by bilateral adrenal hemorrhage, intravascular coagulation and shock liver, producing a severe bacteremia. This combination causes a fulminant shock and, if untreated, it may cause sudden death. We report the case of a female patient with sore throat, fever, vomiting and diarrhea of 24 hours of evolution. Despite receiving inpatient and outpatient medical care, she died as a result of N. meningitidis fulminant sepsis and clinically undiagnosed Waterhouse-Friderichsen syndrome (AU)

A fatal case of streptococcal and meningococcal meningitis in a 2-years-old child occurring as Waterhouse-Friderichsen Syndrome.

We report a fatal case of streptococcal and meningococcal meningitis in a previously healthy 2-year-old child, a simultaneous co-infection of both pathogens that is poorly reported in the reviewed literature. The lack of a clinical diagnosis in addition to the medico-legal aspects arising from possible professional liability for the emergency service doctor who had failed to recognize the child's symptoms led to a forensic autopsy within 48 h after the death. After external and internal examination, Waterhouse-Friderichsen Syndrome (WFS) was suspected. Consequently, cerebrospinal fluid, whole blood, nasal and pharyngeal swab and pleural liquid samples were selected and collected for microbiological studies. All tested samples resulted Neisseria meningitidis DNA and Streptococcus pneumoniae DNA positive. The NM genotyping Real-Time PCR resulted positive for NM serotype C. Microscopic histological study confirmed these findings. We underline that when a patient presents fever and petechiae (50-60% of patients), WFS must be considered, even when the patient has a non-toxic appearance. Due to its rapid progression and often devastating consequences, therapy should be started as soon as WFS is suspected. Emphasis should also be placed on the importance of public education programs and on broadening protection against meningitis through new vaccines. In such cases, from a forensic point of view, there is a strong need for a robust, multidisciplinary approach in order to reach the correct post-mortem diagnosis.

The significance of adrenal hemorrhage: undiagnosed Waterhouse-Friderichsen syndrome, a case series.

A retrospective series of five cases of nontraumatic gross adrenal hemorrhage were identified in 800 consecutive forensic autopsies. All patients were males, of different ethnicities and with ages ranging from 2 to 48 years. All patients had a clinical history and autopsy findings suggestive of sepsis. Pre- or postmortem microbiological cultures were variably positive for Pseudomonas aeruginosa, Streptococcus pneumoniae, Staphylococcus aureus, and methicillin-resistant Staphylococcus aureus. The fifth case was positive for yeast and a coagulase negative staphylococcus; contamination of this culture medium cannot be excluded. No cases had a culture positive for Neisseria meningitidis. We find that the reviewed patients with grossly or microscopically identifiable adrenal hemorrhage were otherwise healthy individuals who died suddenly as a consequence of bacterial infection. In each case, signs and symptoms compatible with premortem adrenal insufficiency were reported; in no instance was the adrenal hemorrhage clinically identified.