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.

Lethal Waterhouse-Friderichsen syndrome caused by Capnocytophaga canimorsus in an asplenic patient.

BACKGROUND: Capnocytophaga canimorsus, a Gram-negative rod, belongs to the Flavobacteriaceae family and colonizes the oropharynx of dogs and cats. Infections with C. canimorsus are rare and can induce a systemic infection with a severe course of the disease. So far, only five case reports of C. canimorsus infections associated with Waterhouse-Friderichsen Syndrome (WFS) have been reported with only two of the patients having a history of splenectomy. CASE PRESENTATION: Here, we report a fatal case of WFS due to C. canimorsus bacteremia and mycetal superinfection in a 61-year-old female asplenic patient. Despite extensive therapy including mechanical ventilation, antibiotic coverage with meropenem, systemic corticosteroids medication, vasopressor therapy, continuous renal replacement therapy, therapeutic plasma exchange, multiple transfusions of blood products and implantation of a veno-arterial extracorporeal membrane oxygenation the patient died 10 days after a dog bite. The autopsy showed bilateral hemorrhagic necrosis of the adrenal cortex and septic embolism to heart, kidneys, and liver. Diagnosis of C. canimorsus was prolonged due to the fastidious growth of the bacteria. CONCLUSIONS: The occurrence of a severe sepsis after dog bite should always urge the attending physician to consider C. canimorsus as the disease-causing pathogen. A therapeutic regimen covering C. canimorsus such as aminopenicillins or carbapenems should be chosen. However, despite maximum therapy, the prognosis of C. canimorsus-induced septic shock remains very poor. Asplenic or otherwise immunocompromised patients are at higher risk for a severe course of disease and should avoid exposure to dogs and cats and consider antibiotic prophylaxis after animal bite.

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.

Manejo quirúrgico temprano en la purpura fulminans del lactante / Early surgical management of purpura fulminans in the infant

Introducción: La purpura fulminans (PF) es una patología grave, que asocia trastornos de la coagulación con afectación principalmente en zonas acrales. El objetivo de este trabajo es destacar la importancia de la sospecha diagnóstica, el manejo multidisciplinar y el tratamiento quirúrgico temprano. Caso: Se describe el caso de una paciente de 14 meses con presentación atípica de PF y síndrome compartimental de antebrazo izquierdo. Tras realizar fasciotomía y un tratamiento médico de soporte de la coagulación intravascular diseminada, se llegó a un diagnóstico etiológico infrecuente: déficit autoinmune de proteína S. Resultados: La purpura progresó en miembros inferiores, que tuvieron buena evolución con las curas locales. No hubo afectación de órganos internos. El miembro superior izquierdo sufrió necrosis seca y precisó amputación. Conclusiones: El manejo de la PF debe ser multidisciplinar para llegar a un diagnóstico certero e iniciar un tratamiento precoz. El diagnóstico y tratamiento rápido del síndrome compartimental es importante para no empeorar el pronóstico del miembro. Entre las opciones de tratamiento quirúrgico encontramos el desbridamiento de heridas, el injerto libre de piel, los colgajos cutáneos y la amputación, para lo cual debe esperarse,siempre que sea posible, a la estabilización del paciente y delimitación de la necrosis.(AU)

Introduction: Purpura fulminans is a serious pathology that associates coagulation disorders which mainly affects acral areas. The aim of this study is to highlight the importance of suspected diagnosis, multidisciplinary management and early surgical treatment. Case: It is described the case of a 14-months-old patient with an atypical presentation of purpura fulminans and a compartmental syndrome of the left forearm. After performing a fasciotomy and a supporting medical treatment for intravascular disseminated coagulation, it was reached to an infrequent diagnosis etiology: autoimmune protein S deficiency. Results: Purple injuries progressed in lower limbs, which had good evolution with local treating. There were no internal organs affection. The left upper limb suffered dry necrosis and it required amputation. Conclusion: The management of PF must be multidisciplinary to reach an ccurate diagnosis and to initiate an early treatment. Prompt diagnosis and treatment of the compartmental syndrome is necessary to prevent a worsening of the limb prognosis. Surgical treatment options include wound debridement, free skin graft, skin flaps and amputation, they should be postponed, as long as possible, until the patient gets stable and the necrosis gets delimitated.(AU)

Waterhouse-Friderichsen syndrome in a cat with Klebsiella spp. infection.

OBJECTIVE: To describe a case of Waterhouse-Friderichsen syndrome of adrenocortical failure in a cat with Klebsiella spp. infection. CASE SUMMARY: A 12-year-old male neutered domestic short-haired cat was referred for respiratory failure requiring mechanical ventilation. The cat remained comatose despite successful weaning from the ventilator and developed a Klebsiella pneumoniae pneumonia. On day 4 of hospitalization, the cat acutely deteriorated with profound hypotension, azotemia, and hyperkalemia, which rapidly progressed to cardiac arrest. Necropsy findings revealed massive adrenal hemorrhage and intralesional bacteria, termed Waterhouse-Friderichsen syndrome. Waterhouse-Friderichsen syndrome was suspected to have been the cause of acquired adrenocortical insufficiency and sudden death of the cat. NEW OR UNIQUE INFORMATION: To the authors' knowledge, this is the first report of sepsis causing Waterhouse-Friderichsen syndrome in a veterinary species.

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 importance of the postmortem interval for the diagnosis of Waterhouse­Friderichsen syndrome by Neisseria meningitidis in a series of forensic cases

The effective value of microbiological post-mortem examinations stands as fundamental in forensic cases involving microbiology. We ran these analyses on five victims, who suddenly died after showing persistent fever. The examinations were conducted between 48 hours and 10 days after death, and adrenal gland apoplexy was detected in all the cases. Microbiological examinations identified Neisseria meningitidis, which was accountable for Waterhouse­Friderichsen syndrome. Diplococci were isolated from three cadavers that underwent forensic dissection between 2 and 3 days after death. The remaining two cadavers showed polymicrobial contamination, and a polymerase chain reaction technique was necessary to identify the pathogen. We assumed that the microbial overlap could lead to diagnostic mistakes and conceal the identification of the lethal pathogen. Therefore, we suggest using molecular techniques for a postmortem interval (PMI) longer than 72 hours. Classical microbiological examination should be performed for PMI within 72 hours.

A unique fatal case of Waterhouse-Friderichsen syndrome caused by Proteus mirabilis in an immunocompetent subject: Case report and literature analysis.

INTRODUCTION: The Waterhouse-Friderichsen syndrome (WFS), also known as purpura fulminans, is a potentially lethal condition described as acute hemorrhagic necrosis of the adrenal glands. It is often caused by infection. Classically, Neisseriae meningitidis represents the main microorganism related to WFS, although, infrequently, also other infectious agents are reported as a possible etiologic agent. The authors report the first case of death due to Proteus mirabilis infection, with postmortem evidence of WFS. PATIENT CONCERNS: After a facial trauma that provoked a wound on the nose, the subject, a healthy 40-years old man, was conducted to the local hospital (in Sicily, Italy) after the primary care he was discharged. Subsequently, after 2 days of general malaise, he returned to the hospital due to the worsening of the clinical condition. During the hospitalization, hypotension, and neurological impairment appeared; the laboratory analysis showed leukocytosis and the alteration of renal, hepatic and coagulative parameters. Microbiological blood analysis resulted positive for a P mirabilis infection. DIAGNOSIS: Multiorgan failure (MOF) with disseminated intravascular coagulation (DIC) due to sepsis was diagnosed. INTERVENTIONS: The practitioners administered intensive support, antibiotic therapy, antithrombin III, vitamin K, and plasma. OUTCOMES: After 3 days the subject died. The autopsy and the microscopic investigation were performed revealing, also, the adrenal diffuse micronodular hyperplasia associated with a cortico-medullary hemorrhagic apoplexy. CONCLUSION: To our knowledge, this is the first case of MOF with WFS due to P mirabilis infection. This case report suggests that P mirabilis should be added to the list of unusual bacteria causing WFS. Furthermore, it supports the theory that any bacterium which causes DIC may cause adrenal hemorrhage and should suggest to clinicians the importance to consider a potential adrenal involvement in every patient with sepsis and DIC.

[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.

[Management of severe sepsis using a Cytokin-adsorber]. / Management einer schweren Sepsis unter Einsatz eines Zytokin-Adsorbers.

Based on a representative case, which was treated in our center for severe burn injuries, the severity of the Waterhouse-Friderichsen syndrome and the complexity of the prolonged course of treatment are illustrated. Special attention is focused on the new treatment paradigm using the CytoSorb® adsorber.

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.

La necrosis cortical: una causa infrecuente de fracaso renal agudo pero con un pésimo pronóstico / Cortical necrosis: An uncommon cause of acute renal failure with a very poor outcome

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