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.

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.

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.

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.

[Dyspnea and skin rash in a 49-year-old male patient]. / Dyspnoe und Hautausschlag bei einem 49-jährigen Patienten.

We report on the case of a 49-year-old man who presented with increasing dyspnea and a skin rash. The community-acquired pneumonia was initially treated with broad spectrum antibiotics. The patient's respiratory condition rapidly worsened and the clinical picture of Waterhouse-Friderichsen syndrome developed with disseminated intravasal coagulopathy and necrosis of the toes. An infection with Capnocytophaga canimorsus, which had been caused by an initially unmentioned dog bite was confirmed. In view of the fulminant course and the high risk of operative treatment of the ubiquitous necroses in all limbs, a joint decision for deescalation of therapy was made together with relatives. The patient died 14 days after admission to hospital.

A rare case of Waterhouse-Friderichsen syndrome caused by Capnocytophaga canimorsus in an immunocompetent patient.

A 53-year-old Caucasian male with hypertension and active tobacco abuse presented to a community hospital with a 2-day history of vague abdominal pain, myalgia and increased lethargy after being bitten on his right hand by the family dog while camping just 3 days prior to symptom onset. He expired within 90 min upon arrival to our intensive care unit. Pre-mortem blood cultures grew a fastidious Gram-negative aerobic rod that was identified as Capnocytophaga canimorsus. Autopsy findings showed multi-organ disseminated intravascular coagulopathy with microthrombi along with bilateral adrenal hemorrhage and necrosis of the adrenal glands consistent with Waterhouse-Friderichsen syndrome. This case contributes to the medical literature as a rare presentation of Capnocytophaga canimorsus infection in an otherwise immunocompetent patient and stresses the importance of a thorough history taking and physical examination by clinicians along with prompt administration of appropriate antibiotics.

Waterhouse Friderichsen syndrome complicating fulminant Enterobacter cloacae sepsis in a preterm infant: the unresolved issue of corticosteroids.

Bilateral adrenal hemorrhage can complicate severe sepsis in the neonate and is most commonly attributed to meningococcal disease; however, it can be caused by other etiologic agents as well. We report herein a fatal case of Enterobacter cloacae sepsis in a preterm infant, resulting in massive adrenal hemorrhages. This is the first documented case of adrenal hemorrhage following infection with this pathogen.

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.

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.

When von Willebrand disease comes into age - a matter of change?

The population of elderly patients with von Willebrand Disease (VWD) is growing because of the improvement of medical care. The increase in comorbidities and their treatment as well as standard preventive measures like antiplatelet or anticoagulation therapy constitutes a challenge in the overall treatment of patients with coagulation disorders. Because of the lack of literature on older patients with VWD, we discuss different aspects related to ageing in those patients. Plasma levels of von Willebrand factor and factor VIII, bleeding symptoms, treatment requirements and comorbidities are possibly changing with age. Development of an evidence-based approach to the management of ageing VWD patients is therefore necessary.

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.

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.

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.

Staphylococcus aureus sepsis and the Waterhouse-Friderichsen syndrome in children.

Staphylococcus aureus has increasingly been recognized as a cause of severe invasive illness. We describe three children who died at our institution after rapidly progressive clinical deterioration from this infection, with necrotizing pneumonia and multiple-organ-system involvement. The identification of bilateral adrenal hemorrhage at autopsy was characteristic of the Waterhouse-Friderichsen syndrome, a constellation of findings usually associated with fulminant meningococcemia. The close genetic relationship among the three responsible isolates of S. aureus, one susceptible to methicillin and two resistant to methicillin, underscores the close relationship between virulent methicillin-susceptible S. aureus and methicillin-resistant S. aureus isolates now circulating in the community.

Overwhelming postsplenectomy infection syndrome in adults - a clinically preventable disease.

Overwhelming postsplenectomy infection (OPSI) syndrome is a rare condition, but is associated with high mortality. However, recognition and clinical management of OPSI is not well established. The prevalence of splenectomy increased recently because it was a clinically effective treatment for hepatitis C virus-associated thrombocytopenia before the introduction of the interferon/ribavirin combination therapy. We reviewed the literature characterizing the clinicopathological features of OPSI and assessed the most effective and feasible administration of the condition. A Medline search was performed using the keywords 'overwhelming', 'postsplenectomy infection', 'postsplenectomy sepsis', 'chronic liver disease', and/or 'splenectomy'. Additional articles were obtained from references within the papers identified by the Medline search. Durations between splenectomy and onset of OPSI ranged from less than 1 wk to more than 20 years. Autopsy showed that many patients with OPSI also had Waterhouse-Friderichsen syndrome. Although the mortality rate from OPSI has been reduced by appropriate vaccination and education, the precise pathogenesis and a suitable therapeutic strategy remain to be elucidated. Protein energy malnutrition (PEM) is commonly observed in cirrhotic patients. Since the immune response in patients with PEM is compromised, a more careful management for OPSI should therefore be applied for cirrhotic patients after splenectomy. In addition, strict long-term follow up of OPSI patients including informed consent will lead to a better prognosis.