Waterhouse-Friderichsen Syndrome Research Articles

Meningococcal infection in children in the period 2012–2021. Main results of a retrospective multicenter study, issues of the day

The heavy burden of meningococcal infection is associated not only with life-threatening complications in the acute period and high mortality in invasive forms of the disease, but also with severe consequences in survivors, who are not recorded in our country.The aim of study: to analyze clinical manifestations, complications of the acute period and outcomes of invasive forms of meningococcal disease in children in various regions of the Russian Federation.Materials and methods: an analysis of data from 1327 inpatient medical records of children with an invasive meningococcal infection from 14 regional centers of the Russian Federation for 2012-2021 was carried out (28.3% of cases of the disease in children in the represented federal districts).Results: it was found that young children predominated among the patients – the median was 27.4 (10.7-70.4) months. Complications of the acute period, often combined, were observed in 47.6% of cases. The development of septic shock was noted in 30.4%, Waterhouse-Friderichsen syndrome in 6.6%, carditis in 2.9%, cerebral edema in 15.7%, arthritis in 1.4% of cases; the formation of hydrocephalus, subdural effusion, sensorineural hearing loss in 1.8%, 0.6%, 1% of children, respectively. The presence of soft tissue necrosis requiring surgical intervention was noted in 3.5% of cases. Mortality rate was 10.1%. At the time of discharge from the hospital, 30% of children had complications associated with meningococcal infection: organ dysfunction/ failure in 13.2% of patients (severe in 1.3%), cerebral insufficiency in 19.6%; severe psycho-neurological deficits, sensorineural hearing loss, problems associated with the need for orthopedic/surgical interventions accounted for 0.7%, 0.6% and 0.8%, respectively.Conclusion. Considering the epidemiological features of meningococcal infection – the risk of a sharp increase in morbidity in short periods of time, the life-threatening nature of the disease itself, it is necessary to remain alert to these risks and take all possible measures to prevent the disease using all available means, the most effective of which is vaccine prevention.

FRI212 Adrenalitis Caused By Proteus Mirabilis In An Immunocompetent Patient

Abstract Disclosure: J.O. Abdelkarim: None. U. Tarabichi: None. M. Jakoby: None. Infectious adrenalitis with Mycobacterium tuberculosis remains the leading cause of primary adrenal insufficiency in developing countries, but the incidence of adrenalitis from infection with other bacterial species is quite low. We present a patient with radiographic evidence of adrenalitis during an infection with Proteus mirabilis that resolved after antimicrobial treatment.An 86-year-old male with an indwelling Foley catheter presented to the hospital for evaluation of abdominal pain and distension. The patient was afebrile and normotensive. Examination was notable for a distended bladder and obstructed urinary catheter. Computed tomography (CT) revealed a dense right lower lobe infiltrate and bilaterally enlarged, hypointense adrenal glands with irregular margins. Radiographic findings were new compared to a CT scan obtained approximately 10 weeks before admission to hospital. Turbid and malodorous urine was drained after replacement of the patient’s urinary catheter, and urine culture grew > 105 CFU/mL Proteus mirabilis. Antibiotics were dosed before blood cultures were drawn, and no organisms were grown from blood cultures. Since there was no clinical or biochemical evidence (e.g. hyponatremia or hyperkalemia) of primary adrenal insufficiency, an ACTH stimulation test was deferred. The patient responded well to replacement of his urinary catheter and antibiotics and was discharged to his extended care facility three days later. A CT scan obtained 10 days after treatment showed resolution of the changes to the patient’s adrenal glands observed on admission imaging.The radiographic changes are most plausibly due to P. mirabilis bacteremia and adrenalitis. The patient had urine culture confirmed infection with P. mirabilis and radiographic evidence of disseminated infection in the right lung and adrenal glands. Adrenal enlargement is well described in cases of acute M. tuberculosis adrenalitis, and radiographic changes of adrenalitis resolved after successful treatment of the patient’s infection. P. mirabilis has been documented as an etiology of Waterhouse-Friderichsen syndrome and cultured from an adrenal abscess. Blood cultures in this case were almost certainly negative due to timing relative to initial antibiotic doses. Injury to the adrenals in bacterial adrenalitis is thought to occur due to multiple mechanisms including direct infection of the glands, endotoxemia, vasculitis, and disseminated intravascular coagulation (DIC). Hemorrhage appears to occur in about 60% of cases. Elevated ACTH level is postulated as a factor predisposing to adrenal hemorrhage, and this prompted deferral of stimulation testing to evaluate adrenal function since there was no clinical evidence of primary adrenal insufficiency. Early management of the patient’s infection likely prevented the occurrence of adrenal insufficiency and deterioration of his clinical status. Presentation: Friday, June 16, 2023

Impact of Radiology on the Early Identification of Waterhouse-friderichsen Syndrome: A Case Study

This study aims to analyze the relevance of radiology in the diagnosis of Waterhouse-Friderichsen Syndrome (WFS), a rare case of acute adrenal insufficiency secondary to hemorrhage. WFS is an extremely rare and serious medical condition, characterized by acute adrenal insufficiency due to hemorrhage in the adrenal glands. Studies have shown that imaging tools such as CT and MRI play a crucial role in confirming the diagnosis of WFS by visualizing bleeding in the adrenal glands. There in not a exact prevalence because of the low presentation of the disease. We present the case of a 40-year-old female patient, previously healthy, who presented symptoms 72 hours before hospital admission and subsequently had severe hemodynamic deterioration. A CT scan is performed and found bleeding in the adrenal gland, free fluid in the left retroperitoneum and important retroperitoneal hemorrhage and her health progressively worsening. She being in charge of the internal medicine service.

Development of the Waterhouse-Friderichsen syndrome after infection with SARS-CoV-2 in a patient with rheumatoid arthritis and secondary AA-adrenal amyloidosis

The COVID-19 pandemic has also affected rheumatic diseases. A clinical case of the development of the Waterhouse-Friderichsen syndrome after SARS-CoV-2 infection in a patient with secondary AA - adrenal amyloidosis is presented. Description of the case. Patient G.G., 57 years old, was hospitalized for several hours on May 20, 2023 at the Cheboksary Regional Hospital with a diagnosis of severe viral interstitial pneumonia. Secondary AA adrenal amyloidosis. Acute adrenal insufficiency. She was admitted with complaints of an increase in body temperature up to 38.20C. Computed tomography of the chest organs showed signs of bilateral viral interstitial pneumonia (the percentage of lung damage was 74%). The diagnosis of COVID-19 was based on a positive PCR test in nasopharyngeal swabs. Blood pressure - 80 / 40 mm Hg. Art. D - dimer - 786 ng / ml (not higher - 243 ng / ml). Despite treatment, the patient died. At autopsy in the lungs, dystelectasis, hyaline membranes, intraalveolar edema. In the adrenal glands - the deposition of amyloid masses, foci of necrosis of the cortical layer and hemorrhage. Signs of DIC - syndrome. Conclusion. The peculiarity of the case is the development of the Waterhouse-Frideriksen syndrome without signs of sepsis.

A clinical case of development of Waterhouse-Friderichsen syndrome after SARS-CoV-2 infection in a patient with rheumatoid arthritis and secondary adrenal AA-amyloidosis

We present a clinical observation of a patient whose cause of death was a new coronavirus infection COVID-19 complicated by bilateral viral interstitial pneumonia and adult acute respiratory distress syndrome. The presence of background diseases-rheumatoid arthritis and secondary AA-adrenal amyloidosis-worsened the prognosis. Waterhouse-Friderichsen syndrome with hemorrhage and foci of necrosis in the adrenal cortex developed against a background of SARS-CoV-2 infection. The unique feature of this case is the long-term favorable course of the disease and its rapid progression after infection with SARS-CoV-2. The associated syndrome of disseminated intravascular coagulation further aggravated the patient's condition.

Pediatric infectious emergencies-from febrile seizure to purpura fulminans

Febrile seizures, which are relatively common in young children, are often triggered by an infection and resolve quickly. Prompt presentation to a pediatric department is mandatory after any first seizure and every time for children ≤ 12months. Central nervous system (CNS) diseases in childhood are able to cause seizures or other neurological disorders. Even the slightest suspicion of aseizure with CNS involvement must be promptly treated. In case of doubt, both an antiviral and an antibacterial treatment are started in parallel, which can be stopped after detecting the pathogen. Lumbar puncture is strictly indicated unless there are contraindications. Meningococcal sepsis is asevere clinical feature comprising high fever, chills and disorders of consciousness. The first skin symptoms are petechiae as ared flag sign. With progression, potentially lethal purpura fulminans may develop. Waterhouse-Friderichsen syndrome is asevere complication of acute bacterial meningitis. Lethality rate is 35%. The pediatric assessment triangle and the ABCDE algorithm help to identify critically ill children in a standardized, structured, and rapid manner.

A fatal case of fulminant group: a streptococcal infection in a neonate

BackgroundFulminant hemolytic streptococcal infection is a condition of sudden onset and rapidly progressing septic shock caused by Streptococcus pyogenes. It causes beta (complete) hemolysis. Although type A Streptococcus occurs more frequently, all streptococci that cause beta-hemolysis are eligible as causes. This report describes a rare autopsy case of fulminant group A streptococcal infection in a neonate.Case presentationA 16-day-old girl, 3300 g, born by spontaneous delivery at 41 weeks 1 day, experienced a 1-day history of low-grade fever, malaise, and a few hours of cyanosis and anuria, prompting her parents to bring her to the hospital. Her eldest brother, who lived with her, had been infected with Streptococcus approximately one month earlier, and had been treated with ten days of antibiotics. The infant died three hours after presentation. Autopsy findings indicated her to be 52 cm in length, weighing 3585 g, with medium build and normal nutrition. Her lungs were slightly oligemic with decreased volumes. The liver and kidneys were mildly enlarged. The spleen was markedly enlarged. The adrenal glands showed diffuse cortical hemorrhage (Fig. 1). There was some thymic atrophy (thymus weight 7.4 g, < 1 SD below the mean). Histopathological findings included chronic and neutrophilic infiltration of the tonsils and multiple septic emboli containing cocci in the lungs. Perivascular inflammatory cell infiltrates were observed in the lungs, myocardium, kidneys, adrenal glands, brain, meninges, and liver, with micro-necrotic changes in the kidneys and liver. Fibrin thrombi were observed in multiple renal glomeruli. Hemophagocytosis by macrophages was observed in the spleen, liver, lymph nodes, bone marrow, and adrenal glands. Immunostaining showed positive results for group A Streptococcus antibody in the tonsils, with bacterial masses. Blood cultures were positive for group A Streptococcus pyogenes.ConclusionsBecause of the lower respiratory tract infection and suspected sibling transmission, in addition to the adrenal hemorrhages, a diagnosis of fulminant sepsis with group A Streptococcus pyogenes with associated Waterhouse–Friderichsen syndrome was made.

Waterhouse-Friderichsen syndrome following a dog bite in an asplenic patient: case report and review of the literature

The Waterhouse-Friderichsen syndrome represents a critical condition characterized by a septic shock associated with a disseminated intravascular coagulation causing the plugging of the microvascular network virtually all organs and systems, including the skin, the kidneys, the liver, and adrenal glands; the mortality rate is elevated, and survivors often must undergo multiple limb amputations. Here, we describe the uncommon case of an asplenic patient who developed this syndrome after a superficial wound caused by a dog bite causing an initial infection due to Capnocytophaga canimorsus that is part of the normal oral microbiome of pets. The clinical and pathological findings and the current and future therapeutic options are reviewed and discussed.

Pancreatic islets as a SARS-CoV-2 target

Aim. The study is aimed at describing pancreatic islets as one of the SARS-CoV-2 targets.Materials and methods. The object of the study was the pancreas. The study involved histological staining.Results and discussion. The autopsy study revealed the signs typical of bilateral viral interstitial pneumonia. Histological examination revealed alveoli with disatelectasis, intra-alveolar edema, hyaline membranes in a part of alveoli. The pancreas histological examination showed hemorrhages, necrotic areas of the islets and detected the following DIC signs: small hyaline thrombi in the heart cavities and large vessels, petechial hemorrhages in the internal organs and adrenal glands, foci of adrenal cortex necrosis with perifocal infiltration (Waterhouse—Friderichsen syndrome); as well as signs of necronephrosis with fibrinoid areas of necrosis in the glomerular capillary network and perivascular lymphoid infiltrates.Conclusion. SARS-CoV-2 was revealed to target human pancreatic islets, where areas of necrosis developed.

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.

Острая надпочечниковая недостаточность и инфекционно-токсический шок как непосредственные причины смерти при менингококковой инфекции

The purpose of the work is to clarify the criteria for the diagnosis of acute adrenal insufficiency and infectious-toxic shock as the direct causes of death in meningococcal infection. The study is based on a clinical and morphological analysis of 27 archival cases of fatal outcomes of patients with meningococcal infection. Histological examination of the archival preparations stained with hematoxylin and eosin was carried out. The specific volume of capillaries in the lungs, hemorrhages in the medullary and cortical substance of the adrenal glands, necrosis in the cortical and medullary layer of the adrenal glands, as well as preserved adrenal cells were studied quantitatively. According to the results of the study, in Waterhouse-Friderichsen syndrome, hemorrhages involving more than 1/3 of the adrenal cortex lead to fatal cases. In meningococcal infection with the development of infectious-toxic shock, capillaries occupy more than ½ of the volume of the interalveolar septa of the lung. The analysis of specific volumes of hemorrhages in the cortical substance of the adrenal glands and capillaries in the interalveolar septa of the lungs can be used during clinical and anatomical analysis for differential diagnosis of Waterhouse-Friderichsen syndrome and infectious-toxic shock.

Fatal Waterhouse-Friderichsen syndrome due to Neisseria meningitidis group B in a paediatric patient during the phone consultation era

ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Serafin M, Czerwińska E, Koźbiał M, Leszczyński M, Szenborn L. Fatal Waterhouse-Friderichsen syndrome due to Neisseria meningitidis group B in a paediatric patient during the phone consultation era. Pediatria Polska - Polish Journal of Paediatrics. 2023. doi:10.5114/polp.2023.125063. APA Serafin, M., Czerwińska, E., Koźbiał, M., Leszczyński, M., & Szenborn, L. (2023). Fatal Waterhouse-Friderichsen syndrome due to Neisseria meningitidis group B in a paediatric patient during the phone consultation era. Pediatria Polska - Polish Journal of Paediatrics. https://doi.org/10.5114/polp.2023.125063 Chicago Serafin, Marta, Ewa Czerwińska, Marian Koźbiał, Mariusz Leszczyński, and Leszek Szenborn. 2023. "Fatal Waterhouse-Friderichsen syndrome due to Neisseria meningitidis group B in a paediatric patient during the phone consultation era". Pediatria Polska - Polish Journal of Paediatrics. doi:10.5114/polp.2023.125063. Harvard Serafin, M., Czerwińska, E., Koźbiał, M., Leszczyński, M., and Szenborn, L. (2023). Fatal Waterhouse-Friderichsen syndrome due to Neisseria meningitidis group B in a paediatric patient during the phone consultation era. Pediatria Polska - Polish Journal of Paediatrics. https://doi.org/10.5114/polp.2023.125063 MLA Serafin, Marta et al. "Fatal Waterhouse-Friderichsen syndrome due to Neisseria meningitidis group B in a paediatric patient during the phone consultation era." Pediatria Polska - Polish Journal of Paediatrics, 2023. doi:10.5114/polp.2023.125063. Vancouver Serafin M, Czerwińska E, Koźbiał M, Leszczyński M, Szenborn L. Fatal Waterhouse-Friderichsen syndrome due to Neisseria meningitidis group B in a paediatric patient during the phone consultation era. Pediatria Polska - Polish Journal of Paediatrics. 2023. doi:10.5114/polp.2023.125063.

Clinical case of disseminated intravascular coagulation and Waterhouse-Friderichsen syndrome development after the SARS-CoV-2 infection

Clinical case of disseminated intravascular coagulation and Waterhouse-Friderichsen syndrome development after the SARS-CoV-2 infection

Multimodality Imaging Findings in Waterhouse-Friderichsen Syndrome: A Case Report and a Short Literature Review

Waterhouse-Friderichsen syndrome is a rare disease characterized by bleeding into the adrenal glands, and its symptoms may vary from non-specific abdominal pain to multi-organ failure. Commonly related to a variety of infectious diseases among which sepsis from Neisseria meningitidis is the most frequent, many other etiologies have been described, and in some rare cases, even no apparent cause can be identified. We report a rare case of an idiopathic bilateral adrenal hemorrhage in a 70-year-old male who presented to our emergency department with non-specific abdominal pain. Ultrasonography Scan (US) and Computed Tomography (CT) findings were crucial for making an early diagnosis, which resulted in a short hospitalization time and, eventually, a complete recovery of the adrenal function. After discharge, Magnetic Resonance Imaging (MRI) was also performed, providing a more complete ensemble of multimodality imaging findings that can be associated with this rare syndrome.

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

BackgroundCapnocytophaga 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 presentationHere, 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.ConclusionsThe 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.

MULTISYSTEM-INFLAMMATORY SYNDROME (MIS-C) IN NEONATE ASSOCIATED WITH SARS-COVID19

Background: Multisystem-Inflammatory Syndrome in Children (MIS-C) is a rare complication of COVID-19 infection in the pediatric population which greatly enhances the risk for thrombotic complications including disseminated intravascular coagulation (DIC). Furthermore, treating complications of DIC is complex as treating one complication can exacerbate the other, and the underlying COVID-19 infection and MIS-C as a whole. This makes choosing the most appropriate treatment difficult.Aim: To report on case of MIS-C in neonate who developed DIC with multiple thrombotic and hemorrhagic events.Methods: In our study, we analyzed a clinical case of a neonate diagnosed with MIS-C who developed DIC with damage to the heart, kidneys, lungs, liver and adrenal glands.Results: After infection with SARS-CoV2, MIS-C developed which led to systemic inflammation that triggered DIC, as proven by laboratory markers. DIC led to thrombotic and hemorrhagic events that affected multiple organs, which led to the following: Pre-renal Acute Kidney Injury-renal failure, acute adrenal insufficiency (Waterhouse-Friderichsen syndrome), ileofemoral thrombosis, right atrial thrombosis, Left ventricle thrombosis and fixed aortic thrombosis. Furthermore, the treatment of MIS-C and that of the complications of DIC were contraindicated on several occasions.Conclusion: This case demonstrated that MIS-C and DIC modulated each other which worsened the prognosis for this neonate initially. With many critical comorbidities the treatment for this case became complex, treating one complication would exacerbate the other, especially noted when the neonate suffered adrenal hemorrhage. Although prognosis was poor, the neonate recovered due to precise control of treatment tactics by the multidisciplinary team.

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

Patient: Male, 58-year-old Final Diagnosis: MRSA infection • Waterhouse-Friderichsen syndrome Symptoms: Shock • weakness Medication: — Clinical Procedure: — Specialty: Critical Care Medicine • Endocrinology and Metabolic • Infectious Diseases Objective: Unusual clinical course 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.

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.

Meningococcal Meningitis with Waterhouse-Friderichsen Syndrome.

This scenario was developed to educate junior and senior emergency medicine (EM) residents. It can also be cut short to be used for 4th year EM bound medical students. Meningococcal meningitis is a devastating disease that can cause severe neurologic sequelae if not diagnosed early and treated appropriately. In 2017, Centers for Disease Control reports a rate of 350 cases (0.11 cases per 100,000) which makes it an extremely rare disease. The highest reported rate is under the age of 1 (0.69 in 100,000) with second peak in adolescents and young adults between the ages of 16 and 23 (0.29 in 100,000) and third peak in patients above the age of 80 (0.49 in 100,000).1 The presentation for bacterial meningitis includes neck stiffness, fever, vomiting, photophobia, positive Kernig and Brudzinski's sign, and lethargy. In addition, 80% patients with meningococcal meningitis have rash during some stage of their disease.2 A feared and rare complication of severe meningococcal disease is Waterhouse-Friderichsen Syndrome (WFS) which carries a high mortality rate of 20%. Therefore, early diagnosis and rapid management of meningococcal disease is highly imperative.3 This simulation case was written to demonstrate the presentation of meningococcal meningitis and to discuss the management of WFS. By the end of this simulation session, learners will be able to: (1) manage a patient with altered mental status (AMS) with fever while maintaining a broad differential diagnosis, (2) recognize the risk factors for meningococcal meningitis, (3) manage a patient with worsening shock and perform appropriate resuscitation, (4) develop a differential diagnosis for thrombocytopenia and elevated international normalized ratio (INR) in an altered febrile hypotensive patient with rash, (5) manage the bleeding complications from WFS, (6) discuss the complications of meningococcal meningitis including WFS, and (7) review when meningitis prophylaxis is given. This session was conducted using high-fidelity simulation. It was immediately followed by an in-depth debriefing session. The session was conducted on a total of 9 EM residents from various levels of training who actively participated during the case and 25 residents who were observers. There was 1 simulation instructor running the session and 1 simulation technician who acted as a nurse. After the simulation and debriefing session was complete, an online survey was sent via surveymonkey.com to all the learners. The survey collected responses to the following questions: (1) the case was believable, (2) the case had right amount of complexity, (3) the case helped in improving medical knowledge and patient care, (4) the simulation environment gave me a real-life experience and, (5) the debriefing session after simulation helped improve my knowledge. A ten-item Likert scale was used to collect the responses. Ten learners responded to the survey. One hundred percent of them either agreed or strongly agreed that the case was beneficial in learning and improving patient care. They also agreed that it helped in improving medical knowledge. The post-session debrief was found to be very helpful by all the learners.. This high-fidelity simulation case was not only cost-effective but also was very helpful in teaching EM residents how to manage a patient with meningococcal meningitis and WFS. The case was started with the patient presenting with altered mental status and fever, and as the case unfolded, mental status and shock worsened allowing the learners to intubate and resuscitate. Overall, learners also found the discussion of prophylaxis valuable. Meningitis, altered mental status, medical simulation, infectious disease, neurology, septic shock, Waterhouse-Friderichsen Syndrome, hematology.

Transient Waterhouse-Friderichsen Syndrome in a Child: A Case Report

Waterhouse-Friderichsen syndrome (WFS) is a rare but usually fatal disease characterized by adrenal insufficiency associated to bilateral adrenal hemorrhage. It can be associated both to non-infection and a variety of infectious diseases, among which sepsis from Neisseria meningitidis accounts for more than 80% of cases. We report the case of an otherwise healthy 2-year-old child who had a WFS as a consequence of Neisseria meningitidis infection and in which we witnessed a complete recovery of adrenal function a few months after the event. The case highlights not only the difficulty of diagnosing this syndrome but also, at the same time, the extreme importance of high suspicion, early treatment, a multidisciplinary approach and, most of all, the importance of verifying the restoration of adrenal function in order to achieve a better quality of life. Int J Clin Pediatr. 2021;10(1):28-33 doi: https://doi.org/10.14740/ijcp429