An autopsy case of pneumococcal Waterhouse-Friderichsen syndrome with possible functional asplenia/hyposplenia.

We report an autopsy case of rapid progressive Waterhouse-Friderichsen syndrome (WFS) associated with Streptococcus pneumonia infection in a previously healthy man. Although he once visited a hospital about 6 hours before death, the both physical and serological examination did not show any sign of overwhelming infection. Autopsy showed massive adrenal hemorrhage without inflammation, and showed proliferation of gram positive cocci and microthrombosis in the vessels of many organs. The pathological change of respiratory tract was extremely minimal. Size and weight of the spleen possible decreased than normal. However, histological examination showed that obscuration of germinal center and decreasing the immunological cells of mantle and marginal zone. Immunohisitochemically, marked decreasing the marginal zone macrophages, which are positive for specific intercellular adhesion molecule grabbing nonintegrin receptor-1 (SIGN-R1) and macrophage receptor with collagenous structure (MARCO), were decreased comparing with age-matched control case. Polymerase chain reaction (PCR) assay using each DNA, extraction from formalin-fixed paraffin-embedded specimen (FFPE) samples of lung, adrenal gland, heart, spleen, and kidney showed positive the ply gene and the lytA gene specific for Streptococcus pneumonia. Present case showed possible acquired atrophy of spleen, especially decreasing marginal zone macrophage may correlate with rapid progression of sepsis of Streptococcus pneumonia with massive adrenal hemorrhage. In addition, present case showed the usefulness of PCR using FFPE for the postmortem diagnosis of WFS.

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

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

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

Fatal Waterhouse-Friderichsen syndrome due to serotype C Neisseria meningitidis in a young HIV negative MSM (men who have sex with men).

Waterhouse-Friderichsen syndrome (fulminant meningococcaemia) is a fulminating infection, often leading to mortality in a matter of hours. In the past 3 years there has been a rise in cases of Neisseria meningitis in the men who have sex with men (MSM) population in the USA and sporadic cases over the world especially in those who are HIV positive. We describe a case of a 34-year-old Caucasian man who presented with acute fulminant meningococcaemia, which proved fatal over a period of hours. This case report emphasises the need for further vigilance, prompt diagnosis and treatment of fulminant meningococcaemia without signs of meningitis especially in the MSM population and the need to expand vaccination recommendations of Meningococcaemia in the MSM population across the USA in both HIV positive and negative males.

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.

Prevalência de sepse e fatores de risco em neonatos de unidade de terapia intensiva de referência em Palmas,Tocantins, Brasil / Prevalence of sepsis and risk factors in newborns from intensive care unit of reference in Palmas, Tocantins, Brasil

As infecções microbianas neonatais, especialmente as sepses, correspondem a uma das causas mais importantes de morbimortalidade em unidades de terapia intensiva e sua prevalência é maior naqueles pacientes expostos a procedimentos invasivos. Objetivo: identificar a prevalência de sepse neonatal hospitalar, bem como os principais fatores de risco associados a esta infecção e os microrganismos envolvidos. Métodos: foi realizado um estudo retrospectivo revisando as fichas de investigação epidemiológica do Serviço de Controle de Infecção Hospitalar com dados dos recém-nascidos internados em unidade de terapia intensiva de referência estadual do Tocantins, no período de julho de 2007 a junho de 2009. Observou-se que 25% dos pacientes analisados desenvolveram sepse neste período, dos quais 60% foram do gênero masculino, 67% prematuros e 65% com peso ao nascer menor que 2500 gramas. Entre os fatores de risco de importância clínica, observou-se a prematuridade, baixo peso ao nascimento, ventilação mecânica, cateterismo venoso central e utilização de nutrição parenteral. A prematuridade e o peso ao nascimento foram fatores de menor risco relativo quando comparados aos demais fatores analisados. Resultados: foram identificados 111 pacientes com sepse, com taxa de mortalidade de 26%. Os principais microrganismos associados à sepse neonatal foram Klebsiella pneumoniae (28%), os Staphylococcus coagulase negativa - CoSN (20%) e Candida albicans (14%). Conclusão: os achados neste estudo reforçam a importância de ações de prevenção de infecções neonatais, bem como o manejo asséptico durante a instalação e a manutenção de procedimentos invasivos necessários aos cuidados intensivos neonatais

Neonatal bacterial infections, especially sepsis, are one of the most important causes of morbidity and mortality in intensive care units and they are more prevalent among patients who have undergone invasive procedures. Objective: to identify the prevalence of hospital neonatal sepsis as well as the associated risks factors for this infection and the involved microorganisms. Method: a retrospective study was done going over the epidemiological investigation forms of the Service of Hospital Infection Control with data from newborns admitted in a State reference intensive care unit in Tocantins, from July 2007 to June 2009. We noticed that 25% of the analyzed patients developed sepsis and 60% were males, 67% premature and 65% with a weight at birth below 2500 grams. Among the risks factors of clinical importance we noticed, the prematurity, low birth weight, mechanical ventilation, central venous catheterization and use of parenteral nutrition. The prematurity and birth weight were lower relative risks factors in comparison with the other analyzed factors. Results: 111 patients with sepsis were found, with a mortality rate of 26%. The main microorganisms associated with neonatal sepsis were Klebsiella pneumoniae (28%), Staphylococcus coagulase negative - CoSN (20%) and Candida albicans (14%). Conclusion: the findings on this study emphasize the importance of actions for prevention of neonatal infections as well as aseptic handling during set up and maintenance of invasive procedures needed in neonatal intensive care

[Puerperal septic shock due to beta-hemolytic Streptococcus and Waterhouse-Friderichsen syndrome]. / Choque séptico puerperal por Streptococcus beta-hemolítico e síndrome de Waterhouse-Friderichsen.

An exceptional case of a 15-year-old puerpera with septic shock caused by Group A beta-hemolytic Streptococcus and Waterhouse-Friderichsen syndrome is reported. The findings were observed at the necropsy. The characteristics of the diagnosis, pathogenesis and evolution of this puerperal infection (sepsis), associated with adrenal hemorrhage and insufficiency are reviewed in this paper.

Choque séptico puerperal por Streptococcus β-hemolítico e síndrome de Waterhouse-Friderichsen / Puerperal septic shock due to β-hemolytic Streptococcus and Waterhouse-Friderichsen syndrome

É relatado caso excepcional de puérpera de 15 anos com choque séptico pelo Streptococcus beta-hemolítico do grupo A e síndrome de Waterhouse-Friderichsen, observado à necropsia. São revistos aspectos do diagnóstico, patogênese e evolução da infecção (sepse) puerperal associada à hemorragia e insuficiência das supra-renais.

An exceptional case of a 15-year-old puerpera with septic shock caused by Group A β-hemolytic Streptococcus and Waterhouse-Friderichsen syndrome is reported. The findings were observed at the necropsy. The characteristics of the diagnosis, pathogenesis and evolution of this puerperal infection (sepsis), associated with adrenal hemorrhage and insufficiency are reviewed in this paper.

[Waterhouse-Friderichsen syndrome associated to a Morganella morganii and Enterococcus faecium peritonitis]. / Syndrome de Waterhouse-Friderichsen au cours d'une péritonite à Morganella morganii et Enterococcus faecium.

About fifty to sixty percent of patients with septic shock acquire acute adrenal insufficiency. This insufficiency is most often relative, but can sometimes be absolute. Bilateral adrenal haemorrhage is a rare aetiology of absolute acute adrenal insufficiency. It is classically described in patients with severe meningococcemia (purpura fulminans), who commonly present many of the risk factors associated with bilateral adrenal haemorrhage (shock, coagulation disorders, sepsis). We report a case of bilateral adrenal haemorrhage during a peritonitis complicated by a septic shock, with no coagulation disorder. This observation shows up that this bilateral adrenal haemorrhage can complicate severe sepsis of various origins, and not only severe meningococcemia. It can be suspected in face of a septic shock with an unfavourable evolution despite adequate treatment.

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.

[Waterhouse-Friderichsen cases in the archives of ZMS PAM--epidemiological conditions and diagnostic difficulties]. / Przypadki zespolu Waterhouse'a-Friderichsena w materialach ZMS PAM--uwarunkowania epidemiologiczne oraz trudnosci diagnostyczne.

15 cases of meningococcal infections from 2003-2004 were discussed in the study. Their therapy ended in failure and they had been typically autopsied in our Forensic Med. Department. During the autopsy material for classical microbiological investigations was collected. That type of death is characterised with pathognomonic and autopsy image. The problem is the microbiological identification of the pathogen group and type. It is connected with the characteristic of the analysed material and the biological features of Neisseria meningitidis. The molecular and genetic methods show their usefulness in this type of diagnostic of the properly selected and collected material which typically are: cerebrospinal fluid, brain tissue with the arachnoid membranes and spleen tissue.

Purpura fulminans -- soft tissue damage as a manifestation of bacterial sepsis.

We present here two cases of purpura fulminans (PF), a rare complication of septicemia. Its fulminant course usually prompt the surgeon into quick and aggressive action. Although sometimes it may lead to mutilation of a patient, nevertheless it is a life-saving procedure. A decision to amputate all four extremities is very difficult, both for the parents and for the surgeon. Therefore one must be absolutely sure that this management strategy is the only way to save the patient's life. Two cases of meningococcal and staphylococcal sepsis treatment are described in this paper. The surgical approach is discussed together with a review of the literature.

Síndrome de Waterhouse-Friderichsen por Streptococcus pneumoniae / Fatal pneumococcal sepsis with Waterhouse-Friderichsen syndrome

Presentamos un caso de sepsis con síndrome de Waterhouse-Friderichsen, como forma de presentación de enfermedad neumocócica invasora en un niño previamente sano. El cuadro clínico evolucionó de forma fulminante y el paciente falleció a las 5 horas de su ingreso. El estudio necrópsico demostró apoplejía suprarrenal bilateral. Este caso demuestra que Streptococcus pneumoniae puede desencadenar un cuadro de sepsis fulminante con Síndrome de Waterhouse-Friderichsen en niños previamente sanos, siendo un cuadro clínico excepcional, muy poco descrito en la literatura científica. Debemos insistir en que es posible evitar la enfermedad neumocócica invasora en el niño, mediante la vacuna conjugada heptavalente, que ha demostrado ser segura y eficaz (AU)

Waterhouse-Friderichsen syndrome as a result of non-meningococcal infection.

Waterhouse-Friderichsen syndrome--massive adrenal haemorrhage in the setting of overwhelming clinical sepsis--is usually taken at necropsy to indicate meningococcal infection, and may be the only evidence of this pathogen. This report describes three fatal cases of the syndrome in which the causative organism proved to be a streptococcus. The organisms were detected during routine coroners' autopsies with histology and microbiological investigations. In two cases, the syndrome followed Streptococcus pneumoniae infection and in a third beta haemolytic streptococcus group A. Thus, adrenal haemorrhage alone cannot be taken to indicate meningococcal disease and other pathogens, particularly streptococcus, must be considered.

Fatal Waterhouse-Friderichsen syndrome due to Ewingella americana infection.

A fatal case of Waterhouse-Friderichsen syndrome resulting from infection in a previously healthy 74-year-old woman is reported. The patient died suddenly within 14 hours after presentation. The diagnosis of Waterhouse-Friderichsen syndrome as the cause of death was established post mortem based on autopsy findings, microscopic examination, measurement of serum procalcitonin concentration (113 ng/ml), and outcome of postmortem bacteriologic cultures that grew in heart and spleen blood samples. Since the introduction of as a new group in the family in 1983, more recent case studies have established its clinical significance and pathogenic potential to cause severe, life-threatening bacteremia and sepsis. is a rare pathogen that should be added to the list of unusual bacteria causing Waterhouse-Friderichsen syndrome.

Waterhouse-Friderichsen syndrome after infection with group A streptococcus.

We report a case of Waterhouse-Friderichsen syndrome associated with group A streptococcus (GAS) toxic shock syndrome in a previously healthy man. The patient presented with neck pain and fevers of 2 days' duration. Computed tomography of the neck revealed a mass in the retropharyngeal space, suggesting an abscess. Despite prompt treatment with appropriate antibiotics, the patient experienced a fulminant course and died within 8 hours of presentation. Antemortem blood cultures grew GAS positive for exotoxins A, B, and C. Postmortem examination revealed bilateral adrenal hemorrhage, consistent with Waterhouse-Friderichsen syndrome. Immunohistochemical analysis of the adrenal glands revealed the presence of GAS antigens. However, no disseminated intravascular coagulation was evident. This case demonstrates that adrenal hemorrhage can occur without associated coagulopathy and may result directly from the action of bacterial toxins.

Invasive disease due to group A beta-hemolytic streptococci: continued occurrence in children in North Carolina.

BACKGROUND: We have previously reported a significant increase beginning in the late 1980s in the incidence of invasive disease due to group A beta-hemolytic streptococci (GABS) in children admitted to our hospital. To determine subsequent trends in epidemiology, we have continued to monitor cases. METHODS: We prospectively monitored cases of invasive disease due to GABS at Brenner Children's Hospital during the 5 1/2 years (July 1, 1990, to December 31, 1995) since our last report. RESULTS: Twenty-five patients had GABS isolated from normally sterile sites. Their presentations were varied. One patient had necrotizing fasciitis and one had toxic shock-like syndrome. The one death was that of a newborn infant with sepsis and meningitis. The proportion of GABS infections associated with varicella was significantly greater during this period (7/25, 28%) than during the period 1983 to 1990 (1/22, 5%). Isolates were available tor study from 24 patients. Serotypes were M1 (4), M3 (4), M6 (2), M12 (3), M22 (3), M75 (1) and M-nontypeable (7). The number of cases of invasive disease seen annually from 1983 through 1995 also is reviewed. CONCLUSIONS: The resurgence of invasive disease due to GABS in children noted in the late 1980s continues through the first half of the 1990s. The clinical manifestations are varied as are the causative M-types. As almost one third of cases in this series were associated with varicella infection, widespread use of the varicella vaccine may lead to a decrease in the incidence of invasive GABS disease.