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.

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.

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.

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.

Waterhouse-Friderichsen syndrome secondary to Capnocytophaga canimorsus septicemia and demonstration of bacteremia by peripheral blood smear.

Waterhouse-Friderichsen syndrome caused by Capnocytophaga canimorsus septicemia was fatal in a previously healthy 47-year-old woman. The patient died suddenly in less than 12 hours after presentation, in spite of supportive measures, including ventilation, antibiotic coverage, pressor therapy, and multiple transfusions of blood products. The diagnosis of infection due to an unusual organism was suspected earlier in the course of management after review of the peripheral blood smear. The importance of the findings in the blood smear and their correlation with infection due to this organism are discussed.

OPSI (overwhelming postsplenectomy infection) syndrome: a case report.

Splenectomized patients are likely to suffer from severe infections, such as sepsis and meningitis. This syndrome is called overwhelming postsplenectomy infection (OPSI) in Europe and America. We present an adult case of OPSI syndrome, which occurred as respiratory insufficiency, and thrombocytopenia. The course is rapid, the clinical symptoms are serious, and the prognosis is very poor. Clinical examination showed cyanosis, mandibular hypertonia, psychomotor anxiety and purpura. Laboratory findings were thrombocytopenia, leukocytosis, hypoglycemia and altered coagulation parameters. A chest X-ray showed right pulmonary aspecific thickening. The autopsy findings occurred as Waterhouse-Friderichsen syndrome.

[A fulminant course of infection after splenectomy]. / Foudroyant verlaufende Sepsis nach Splenektomie.

A report is given of two patients with a history of splenectomy many years previously due to traumatic rupture. No vaccination was given to either patient. From a state of good health, both patients developed fulminant, therapy-resistant sepsis with proof of Streptococcus pneumoniae in the blood culture. Autopsy findings were similar to Water-house-Friderichsen-syndrome. In conjunction with the history of splenectomy, the final pathological diagnosis was a so-called OPSI syndrome. This postsplenectomy sepsis is discussed further.

[Changes in the adrenal glands in menigococcemia]. / Izmeneniia nadpochechnikov pri meningokoktsemii

Post-mortem morphological, histochemical, and morphometric investigations of the adrenals in 46 deceased patients who suffered from meningococcemia with and without lesions of the meninges were carried out. The control group was composed of 7 practically healthy persons who had perished suddenly as a result of craniocerebral trauma. It was shown that the adrenals might often be seriously damaged in meningococcemia without clinical and morphological signs of meningitis. Hemodynamic disorders, in particular, involvement of the vessels of the microcirculatory bed, contributed considerably to the lesions of the adrenals. However, even in meningococcemia free from signs of meningitis, in a number of cases individual areas of the cortex retained a high functional activity. In meningococcemia combined with lesions of the meninges, despite considerable damage of the cortical layer, in the majority of cases there were evidences of the tense functioning of the adrenal cells.

[Current problems in the pathological anatomy of acute neuroinfections in children]. / Aktual'nye voprosy patologicheskoi anatomii ostrykh neiroinfektsii u detei.

The analysis of the autopsy material of children dying from acute neuroinfections in a specialized department of the Research Institute of Children's Infections from 1954 to 1984 is presented. The increase of the importance of hypertoxic forms of meningococcal infection, purulent meningoencephalitis (particularly those of pneumococcal etiology) as well as acute viral encephalitis (particularly herpetic ones) is noted at present. The results of the materials studied by the author from 182 children dying of those infections at the period from 1971 to 1984, the problems of their patho- and tanatogenesis are discussed. Special attention is drawn to the necessity of considering the type of an etiological agent, its properties, the features of tissue response and those of the immunological resistance in the brain for the diagnosis and epicritic assessment of acute neuroinfections.

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

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