Fever and Rash.

Infectious causes of fever and rash pose a diagnostic challenge for the emergency provider. It is often difficult to discern rashes associated with rapidly progressive and life-threatening infections from benign exanthems, which comprise the majority of rashes seen in the emergency department. Physicians must also consider serious noninfectious causes of fever and rash. A correct diagnosis depends on an exhaustive history and head-to-toe skin examination as most emergent causes of fever and rash remain clinical diagnoses. A provisional diagnosis and immediate treatment with antimicrobials and supportive care are usually required prior to the return of confirmatory laboratory testing.

Meningococcemia in a vaccinated child receiving eculizumab and review of the literature.

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare and severe disease characterized by uncontrolled activation and dysregulation of the alternative complement pathway and development of thrombotic microangiopathy. Eculizumab, which is used as a first-line therapy in aHUS, blocks the formation of C5 convertase and inhibits the formation of the terminal membrane attack complex. It is known that treatment with eculizumab increases the risk of meningococcal disease by 1000-2000-fold. Meningococcal vaccines should be administered to all eculizumab recipients. CASE: We describe a girl with aHUS who was receiving eculizumab treatment and experienced meningococcemia with non-groupable meningococcal strains which rarely cause disease in healthy people. She recovered with antibiotic treatment and we discontinued eculizumab. CONCLUSIONS: In this case report and review, we discussed similar pediatric case reports in terms of meningococcal serotypes, vaccination history, antibiotic prophylaxis and prognosis of patients who experienced meningococcemia under eculizumab treatment. This case report highlights the importance of a high index of suspicion for invasive meningococcal disease.

Epidemiología de la enfermedad meningocócica en República Dominicana 2012-2022: bases para su prevención / Epidemiology of meningococcal disease in Dominican Republic 2012-2022: strategies for its prevention

Objetivo: La Enfermedad Meningocócica Invasiva es una causa frecuente de morbimortalidad mundial. El objetivo de este estudioes describir la epidemiología de la enfermedad meningocócica en República Dominicana desde 2012 a 2022 y proponer las basespara su prevención.Método: En el análisis se calcularon incidencias, tasas de hospitalización por cada 100.000 habitantes, tasas de letalidad y se hizouna comparación entre grupos de edad de los 325 casos de meningococcemia reportados en el Sistema Nacional de VigilanciaEpidemiológica Dominicano durante estos 10 años.Resultados principales: El 33% de los casos (103) tenía menos de 5 años. Este grupo de edad fue el que tuvo la tasa de hospitalizaciónmás alta, la mayor tasa de mortalidad en edad pediátrica la mayor letalidad.Conclusiones: Si la República Dominicana vacunara a su población, debería comenzar con los menores de 5 años, y así se reduciríanlas hospitalizaciones, muertes, complicaciones y secuelas producidas por el meningococo. (AU)

Objective: Invasive meningococcal disease is a frequent cause of morbidity and mortality worldwide. The aim of this study is todescribe the epidemiology of meningococcal disease in the Dominican Republic from 2012 to 2022 and to propose the basis for itsprevention.Methods: Incidences, hospitalization rates per 100,000 population, case fatality rates and a comparison between age groups ofthe 325 cases of meningococcemia reported in the Dominican National Epidemiological Surveillance System during these 10 yearswere calculated in the analysis.Main results: 33% of the cases (103) were less than 5 years old. This age group had the highest hospitalization rate, the highestmortality rate in pediatric age and the highest case fatality rate.Conclusions: If the Dominican Republic were to vaccinate its population, it should start with those under 5 years of age, and thiswould reduce hospitalizations, deaths, complications and sequelae caused by meningococcus. (AU)

Momificación natural de falanges como consecuencia de meningococemia fulminante. A propósito de un caso / Natural mummification of phalanges as a consequence of fulminant meningococcemia. About a case

Fundamento: Las infecciones del sistema nervioso central constituyen una importante causa de morbilidad y mortalidad neurológica. La púrpura fulminante o meningococemia es una enfermedad grave que evoluciona a severas complicaciones y secuelas, sin embargo, es infrecuente que ocurra momificación o amputación natural de los dedos de las manos y los pies. Objetivo: Describir el caso de un paciente con meningococemia fulminante, al cual se le momificaron de manera natural las falanges de las manos y los pies como consecuencia de la enfermedad. Presentación del caso: Se presenta un paciente de 18 años de edad que fue diagnosticado con meningococemia. Clínicamente presentó fiebre, cefalea intensa, lesiones purpúrico hemorrágicas, petequias y hematomas en piel, evolutivamente shock séptico y disfunción múltiple de órganos. Posterior a 16 días de estadía en sala egresó vivo del hospital, pero con secuelas caracterizadas por momificación de las falanges de las manos y los pies. Conclusiones: La meningococemia es una enfermedad aguda, potencialmente mortal y se reporta mayormente en la edad pediátrica. Entre los sobrevivientes es infrecuente que ocurra la momificación de las falanges de las manos y los pies, como ocurrió en el caso reportado.

Background: Infections of the central nervous system are a significant cause of neurological morbidity and mortality. Purpura fulminans or meningococcemia is a serious disease that evolves into severe complications and sequelae, however it is infrequent for mummification or natural amputation of fingers and toes to occur. Objective: To describe the case of a patient with fulminant meningococcemia, in whom hands and feet phalanges were naturally mummified as a consequence of the disease. Case report: An 18-year-old patient diagnosed with meningococcemia is presented. Clinically, he presented fever, intense headache, purpuric hemorrhagic lesions, petechiae and bruises on the skin, progressively septic shock and multiple organ dysfunction. After 16 days in hospital, he was discharged alive, but with some sequelae characterized by mummification of the hands and feet phalanges. Conclusions: Meningococcemia is an acute, life-threatening disease and is mostly reported in the pediatric age. Mummification of the hands and feet phalanges is uncommon among survivors, as occurred in the reported case.

CRYOGLOBULINEMIC VASCULITIS.

BACKGROUND: Cryoglobulinemia is a hematologic condition characterized by the presence of immunologic proteins in the blood, resulting from underlying malignancy to chronic viral processes. The recognition of this condition is critically vital, as patients can first present to the emergency department as their initial manifestation of disease. CASE REPORT: We present a case of cryoglobulinemia, discuss the clinically important types, their presentations, and then emergent complications that can be encountered in the emergency setting. Why Should an Emergency Physician Be Aware of This? Cryoglobulinemia comes in two clinically significant types, both of which can be indicative of an underlying hematologic malignancy, autoimmune, or viral process. The presentation can appear dramatic and can also mimic severe critical illness, for example, meningococcemia. Recognition and appropriate disposition is crucial for the best patient outcome.

Eculizumab's Unintentional Mayhem: A Systematic Review.

Eculizumab, first-line therapy for paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS), has infectious side effects in addition to its therapeutic benefits. This study aims to discuss the mechanism of development of infections, prevention, and timely treatment to prevent complications such as septic shock and mortality. The study was conducted using the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) checklist and reporting guidelines for systematic review. Inclusion and exclusion criteria were determined. A total of 10 research papers were extracted after exploring Pubmed and Google Scholar from 2001 to 2021. The New Castle Ottawa Questionnaire for non-randomized clinical trials and the National Institutes of Health (NIH) quality assessment tool for case reports and case series were used to assess the risk of bias. The studies included in this systematic review describe infections with Neisseria meningitidis, Neisseria gonorrhoeae, unusual Neisseria species, Moraxella lacunata, and Pseudomonas aeruginosa. The main goal of this review is to impress upon the seriousness of the infectious complications associated with eculizumab. Health care providers should maintain a high index of suspicion for early identification and treatment.

Genetic workup as a complementary tool for the diagnosis of primary complement component deficiencies: a multicenter experience.

Diagnosis of primary complement deficiencies requires a high index of suspicion. Thus, susceptible patients are often underdiagnosed and untreated. Here, we present a multicenter experience with two novel inborn errors of the classical complement system. This is a retrospective multicenter analysis of computerized medical records of children (<18 years) admitted in the period between 2012 and 2018 at Shaare Zedek Medical Center in Jerusalem and Edmond and Lily Safra Children's Hospital, Tel-Hashomer Medical Center, in Ramat Gan, Israel. Patients were genetically diagnosed by a complementary immune workup. We identified 5 patients (3 males) from four different families harboring two novel mutations in the complement components C6-C8. Genetic mutations were identified by whole-exome sequencing or by sequencing of the coding exons of a single gene based on the findings in the immune workup. Clinical manifestations consisted of meningitis with or without meningococcemia. The immune workup demonstrated nearly absent levels of CH50, compatible with a complement pathway defect. Diagnosis delay ranged between 0 and 30 years. CONCLUSION: Awareness of risk factors for primary complement deficiencies, even at the first infectious episode, should facilitate prompt immune and genetic workup, commencing diagnosis and proper treatment for the patient and family. WHAT IS KNOWN: • Deficiencies in the classical terminal complement components increase susceptibility to invasive meningococcal infections. • Recurrent meningococcal infections mandate a diagnostic workup of the complement system. WHAT IS NEW: • Genetic workup can be utilized for prompt diagnosis of complement deficiencies. • High rates of consanguinity, even in the presence of a single meningococcal infection, should promote immune and genetic workups.

[Analysis of fatal outcomes of meningococcal infection in adults].

Clinical characteristics and pathomorphological manifestations in 69 patients aged 18 to 86 years with a fatal outcome of the disease were examined in order to analyze the causes of severe course and high mortality of generalized forms meningococcal infection. It was found that the main clinical form was meningococcemia (90%), in the majority in combination with meningitis (52%). The fulminant course in 77% of patients with meningococcal sepsis manifested itself as a sudden onset, rapid development of typical symptoms. Hemorrhagic exanthema was detected on the first day of meningococcemia. The leading complications and critical conditions were infectious-toxic shock, disseminated intravascular coagulation and acute adrenal insufficiency (Waterhouse-Friederiksen syndrome). The severe course of meningitis (in 10%) led to the development of cerebral coma, the morphological substrate of which was edema - swelling of the brain.

Meningococcal disease and carriage in the Philippines: A review of recent data

@#This article reviews recent data on meningococcal disease and carriage in the Philippines. It aims to provide information on the epidemiology of meningococcal disease, its carriage, data on prevention, and the impact of vaccination on disease and carriage. The World Health Organization considers the Philippines as having low endemicity for meningococcal disease. However, current data underestimates the true burden in the country due to many factors. In recent years, data from the Philippines show a high case-fatality rate since only the septicemic form is being reported. Studies on asymptomatic meningococcal carriage rates are sparse, with one study by Gonzales, et al. investigating the prevalence of meningococcal nasopharyngeal carriage in Filipinos aged 5-24 years old living in an urban setting. The study showed that the overall prevalence of carriage was 3.7% and was highest (9%) among the 10-14 age group. Serogroup B was the most common isolate. Effective meningococcal vaccines are available. Although not included in the National Immunization Program, medical societies recommend giving vaccines to individuals at high risk of infection. Data on local epidemiology accounting for the disease and asymptomatic carriage are important to strengthen future programs on immunization and prevention of meningococcal disease.

Hospitalizations related to meningococcal infection in Spain from 1997 to 2018.

BACKGROUND: Baseline hospitalization, mortality, and in-hospital fatality rates for meningococcal infection are required to evaluate preventive interventions, such as the inclusion of the conjugated quadrivalent meningococcal vaccine and serogroup B based protein vaccines. METHODS: All meningococcal infection-related hospitalizations in any diagnostic position in Spain from 1st January 1997 through 31st December 2018 were analysed. The annual hospitalization rate, mortality rate and case-fatality rate were calculated. RESULTS: The average hospitalization rate for meningococcal infection was 1.64 (95% CI 1.61 to 1.66) hospitalizations per 100,000 inhabitants during the study period and significantly decreased from 1997 to 2018. Hospitalizations for meningococcal infection decreased significantly with age and were concentrated in children under 5 years of age (46%). The hospitalization rates reached 29 per 100,000 and 24 per 100,000 children under 1 and 2 years of age, respectively. The in-hospital case-fatality rate was 7.45% (95% CI 7.03 to 7.86). Thirty percent of the deaths occurred in children under 5 years of age, and more than half occurred in adults. The case fatality rate increased significantly with age (p < 0.001). CONCLUSION: It is necessary to maintain epidemiological surveillance of meningococcal infection to determine the main circulating serogroups involved, track their evolution, and evaluate preventive measures whose effectiveness must be assessed in all age groups.

Meningococcal disease epidemiology in Brazil (2005-2018) and impact of MenC vaccination.

BACKGROUND: Meningococcal disease (MD) presents a substantial public health problem in Brazil. Meningococcal C conjugate (MenC) vaccination was introduced into the routine infant immunization program in 2010, followed by adolescent vaccination in 2017. We evaluated changes in national and regional MD incidence and mortality between 2005 and 2018, serogroup distribution and vaccine coverage. METHODS: Data were obtained from national surveillance systems from 2005 to 2018. Age-stratified incidence and mortality rates were calculated and a descriptive time-series analysis was performed comparing rates in the pre-(2005-2009) and post-vaccination (2011-2018) periods; MD due to specific meningococcal serogroups were analyzed in the pre-(2007-2009) and post-vaccination (2011-2018) periods. RESULTS: From 2005 to 2018, 31,108 MD cases were reported with 6496 deaths; 35% of cases and deaths occurred in children < 5 years. Incidence and mortality rates declined steadily since 2012 in all age-strata, with significantly lower incidence and mortality in the post-vaccine introduction period in children aged < 1-year, 1-4 years, 5-9 years and 10-14 years. A significant decline in MenC disease in children < 5 years was observed following MenC vaccine introduction; infants < 1 year, from 3.30/100,000 (2007-2009) to 1.08/100,000 (2011-2018) and from 1.44/100,000 to 0.42/100,000 in 1-4-year-olds for these periods. Reductions in MenB disease was also observed. MenW remains an important cause of MD with 748 cases reported across 2005-2018. While initial infant vaccination coverage was high (>95% nationwide), this has since declined (to 83% in 2018); adolescent uptake was < 20% in 2017/18). Regional variations in outcomes and vaccine coverage were observed. CONCLUSION: A substantial decline in incidence and mortality rates due to MD was seen following MenC vaccine introduction in Brazil, especially among children < 5 years chiefly driven by reductions in MenC serogroup. While these benefits are considerable, the prevalence of MD due to other serogroups such as MenW and MenB remains a concern. A video summary linked to this article can be found on Figshare: https://doi.org/10.6084/m9.figshare.13379612.v1.

Chronic meningococcemia in a vertically HIV-infected adolescent.

Chronic meningococcemia is a rare manifestation of meningococcal disease, characterized by a period of more than one week of intermittent or continuous fever, arthralgia and skin lesions without meningitis. It can occur both in previously healthy and immunocompromised patients. The gold standard for the diagnosis is culture isolation of Neisseria meningitidis in sterile material. We describe a case of a vertically HIV-infected adolescent with chronic meningococcal disease.

Neisseria meningitidis Induced Fatal Waterhouse-Friderichsen Syndrome in a Patient Presenting With Disseminated Intravascular Coagulation and Multiple Organ Failure.

Neisseria meningitidis-induced acute systemic meningococcal disease is an emergency and a fatal condition that has a high mortality rate. In patients with a fulminant infection, a maculopapular petechial eruption, purpura fulminans, or an ecchymotic lesion are worrisome signs reflecting disseminated intravascular coagulation (DIC) and hint at Waterhouse-Friderichsen syndrome (WFS). Here, we describe a rare case of a patient with a fulminant Neisseria meningitidis-induced acute systemic meningococcal disease presenting with high-grade fever without meningitis symptoms. Fatal septicemia with DIC and multiple organ failure was noted. WFS was chiefly suspected. We highlight the clinical features and pathogenesis of Neisseria meningitidis-induced meningococcemia and WFS. We propose that they should be kept in mind, especially in patients presenting with a petechial eruption and purpura fulminans.

First case report of fulminant septic shock from meningococcemia associated with Cryptococcus neoformans coinfection in an immunocompetent patient.

The meningococcal disease manifestation associated with the presence of Cryptococcus neoformans is rare. There are no reports in the literature about these simultaneous infections in immunocompetent patients. The aim of the present study is to describe the first case of fulminant septic shock by Neisseira meningitidis associated with Cryptococcus neoformans coinfection in an immunocompetent patient. We describe a case of an immunocompetent 74-year-old Caucasian woman who presented with fulminant acute meningococcemia associated with cryptococcal meningitis, which progressed to worsening general condition and died of septic shock and multiple organ dysfunctions in less than 48 hours. This case report demonstrates the possibility of coinfections related to Neisseria meningitidis and Cryptococcus neoformans, even in immunocompetent patients, which represent a diagnostic challenge for clinicians, thus encouraging further studies for a better understanding.

Purpura in the Emergency Room: Two Cases of Sepsis with Cutaneous Manifestations.

Purpura fulminans is a seldom seen manifestation of sepsis in the emergency department (ED). The morbidity and mortality of sepsis have been widely studied and reported; the hallmark of treatment is early recognition and intervention. In extreme cases, sepsis can cause widespread activation of the coagulation cascade further complicating the treatment and recovery from the causative pathogen. We report two cases and their differing outcomes after presentation to the ED with similar dermatologic findings on initial physical exam.

Epidemiological burden of meningococcal disease in Latin America: A systematic literature review.

OBJECTIVE: To evaluate the epidemiological profile of invasive meningococcal disease (IMD), meningococcal meningitis, and Neisseria meningitidis carriers in Latin America. METHODS: A systematic review was conducted to identify and analyze studies published in 2008-2018. Incidence rates, case fatality rates (CFRs), and the relative distribution of cases per serogroup by country were assessed. RESULTS: Meningococcal surveillance in Latin America differs among countries, and most systems are based on passive sentinel surveillance. Thirty-nine studies were selected. In 2006, the incidence rate of IMD per 100 000 inhabitants was highest in Brazil (1.9), followed by Uruguay (1.3), Chile (0.8), Argentina (0.7), Colombia and Venezuela (0.3 each), and Mexico (0.06). Brazil reported the highest CFR (20%), followed by Uruguay (15%), Chile (11%), and Venezuela and Argentina (10% each). In 2012, the CFR in Chile increased to approximately 27%. The most frequent serogroups among IMD cases were C in Brazil (2007-2010) and Mexico (2005-2016), W in Chile (2012-2018), and B in Argentina (2012-2015). However, the true burden of IMD in Latin America is probably underestimated due to underreporting of cases. CONCLUSIONS: Improvements in IMD notification, IMD registration, national surveillance programs (including active surveillance systems), diagnostic tools, and characterization of isolates may better elucidate the true epidemiological burden of IMD in Latin America.

First Report of a Disease by Rhazes 10 Centuries Ago.

INTRODUCTION: Abu Bakr Mohammad Ibn Zakariya Al-Razi (865-925 CE), who was known as "Rhazes" in the west, was a famous scientist of medieval ages. He has more than 200 books and treatises. His masterpiece on medicine "Kitab Al-Hawi Fi Al-Tibb" contains around 900 case reports. Some of the diseases which seem to be recently reported have been stated previously, but not well described. Considering symptoms of the patient described at that time, differential diagnosis will be discussed. CASE PRESENTATION: Rhazes described a patient with bilious fever. He had developed bloody urine and stool on the fourth day and fatigue. Subsequently, the patient's urine and stool color turned into dark and black, respectively, and died the following day. According to Rhazes attitude, it was malignant measles. Meyerhof in his book has referred to post-measles acute glomerulonephritis, but more appropriate differential diagnoses are compatible with this patient. DISCUSSION: One of the best diagnoses for this case can be Weil's syndrome. Presence of fever, icterus, hemorrhage and renal injury, all suggest Weil's syndrome without pulmonary involvement. The other probable diagnosis is thrombotic thrombocytopenic purpura (TTP). Meningococcal sepsis is the other possible diagnosis. CONCLUSION: To sum up, as three compatible diseases with the case; have been described more than a thousand years after Rhazes (Weil's syndrome 1886, TTP 1925 and meningococcemia 1805); if the case is either Weil's or TTP or meningococcal sepsis, it is the first report of the disease in the world by Rhazes.

Chronic meningococcemia.

The spectrum of Neisseria meningitidis-associated clinical entities involves mild forms of disease, without neurological involvement or sepsis, and asymptomatic carrier states. Rarely, N. meningitidis bacteremia can be associated with a prolonged fever with or without arthritis, which we designate as chronic meningococcemia. Chronic meningococcemia is an uncommon entity, usually associated to serogroup B N. meningitidis. Diagnosis is frequently delayed as blood cultures collected outside febrile periods can be negative. We present a case of chronic meningococcemia in a 22-year-old woman with no relevant clinical background, presenting with fever, arthralgia and exanthem. Due to the potential for progression to more severe disease and the risk of N. meningitidis transmission and development of secondary cases, a high degree of clinical suspicion is required to ensure prompt recognition and adequate treatment. Our patient had a favorable outcome probably due to early recognition and adequate treatment, which is critical for the resolution of the disease without complications.

Chronic meningococcemia: a report of 26 cases and literature review.

Chronic meningococcemia is defined by blood culture(s) positive for Neisseria meningitidis, symptoms duration > 7 days, and neither meningitis nor shock on admission. This series of 26 consecutive cases illustrates that this is a rare disease (< 5% of meningococcemia, < 0.05 cases per 100,000 inhabitants per year), mostly affecting young adults, males, with no predisposing condition. Major symptoms include fever, rash, and arthralgia. Median time between symptoms onset, and diagnosis is 28 days. Most patients fully recover with a 1-week course of parenteral betalactams.