JCHIMP 2000-9666 Journal of Community Hospital Internal Medicine Perspectives Vol. 1, January 2012, pp. 1–4 sici xxx PUBMED Abbreviation John Tabacco MPH MD

*John Patrick Tabacco, 1201 W. Mt. Royal Ave Baltimore MD 21217 USA (240) 498 8282

Suniega Elizabeth MSIII Sarabchi Fardad MD Mitsani Dimitra MD Union Memorial Hospital

Baltimore MD USA

Journal of Community Hospital Internal Medicine Perspectives CASE REPORT Fatal meningococcemia Within the past six years, a case of bothWaterhouse-Freidrichsen Syndrome and fulminant meningococcemia have presented to Union Memorial Hospital. Both cases presented in markedly different fashions, differed in microbiologic serogrouping, showed minimal histopathologic similarities; however achieved ultimately the same outcome through two different pathological pathways. The following case reports illustrate two mechanisms through which N. Meningitis may pathogenize a host, both leading to complete cardiovascular collapse in less than 12 hours. Infectious disease A 46-year-old male bus driver with past medical history of obstructive sleep apnea presented to an emergency department in Baltimore Maryland in early July, 2011 at approximately 1:00 pm, complaining of sudden onset severe, left sided chest pain, shortness of breath, back pain, chills, and one episode of a non-witnessed seizure with loss of consciousness as he was preparing to depart on his bus route early in the morning. The patient denied any prodrome to presenting symptoms, stating he had felt fine the day prior. He denied sick contacts. His temperature on admission was 101.1, blood pressure was 126/71 mmHg, and heart rate was 96 BPM. He was an obese, well developed male who was ill looking. He was alert and oriented with no nuchal rigidity. Lungs were clear and no cardiac murmurs were auscultated. No abdominal or flank tenderness was noted. No rashes, petechiae or purpura were noted. Neurological exam was non focal. Significant laboratory values were: WBC: 3.8 k/UL, PMN: 90.9%, troponin: 0.057 ng/ml, Hgb: 12.8 gm/dL, PLT: 198K/UL, BUN: 12, Cr: 0.68, CK: 189. ECG showed non-specific T wave abnormalities. Chest radiograph revealed questionable lower lobe infiltrate and piperacillin-tazobactam was started empirically. Chest CT was negative for pulmonary embolism. At 7:45 pm, while in the ER, the patient began to hemodynamically decompensate as blood pressure dropped to 80/60 mmHg. The patient was given two liters of normal saline and was moved to the Intensive Care Unit. Cardiopulmonary arrest occurred at approximately 8:45 pm. Cardiopulmonary resuscitation was started and intubation was attempted but failed and pulse was unable to be established. Approximately eight hours after presenting to the Emergency Room the patient was pronounced dead. At 8:00 am on the following morning, morphology of blood cultures returned as Gram negative diplococci, with speciation later to return as Neisseria meningitides serogroup Y. Later autopsy revealed myocarditis (Fig. 1) Final diagnosis was fulminant meningococcemia.

Myocarditis.

A 19-year-old male college student was brought into the emergency department by ambulance at approximately 1:00 am on 26 October 2005. The patient believed that he was having an ‘allergic reaction to Chinese food’ that he had consumed an hour prior to developing the presenting symptoms of hot flashes, shortness of breath, lightheadedness, generalized weakness, and myalgia. On physical exam he was alert, pale, clammy, tachycardic, normotensive, and afebrile. There was no rash. The patient was treated initially for a presumed allergic reaction. On subsequent re-evaluation at 7:00 am and 8:00 am, he remained tachycardic with no improvement of symptoms. Initial laboratory workup was significant for a WBC: 2.2 k/UL, Hgb: 12.1 gr/dl, PLT: 35 K/UL, bandemia of 36%, non-anion gap metabolic acidosis (CO2: 16 mmol/L); Urine toxicology screen was negative. Approximately five hours later, the patient developed restlessness, diaphoresis, and became disoriented. Blood cultures were sent and broad spectrum intravenous antibiotics were started. He was sent to the Intensive Care Unit. At 9:45 am he became hypotensive, failed to respond to fluid resuscitation and went into a state of pulseless electrical activity. Cardiopulmonary resuscitation continued for 45 min and patient was pronounced dead at 10:30 am, less than nine hours from time of presentation. The following day, blood cultures grew gram negative diplococci later speciated as Neisseria meningitidis serogroup B. Review of school records showed that he had been vaccinated with the meningococcal vaccine. On autopsy, there were macroscopic bilateral areas of adrenal hemorrhage consistent with Waterhouse-Friderichsen syndrome (Fig. 2) as well as florid conjunctival petechiae and bilateral subconjuntival hemorrhage. Myocarditis was noted.

Adenal cortex hemorrhage.

Neisseia meningitides is a gram negative aerobic diplococcus that colonizes human nasopharynx in up to 10% of the population 18. Humans are the sole reservoir for this pathogen which is spread by physical contact or by inhalation of respiratory droplets 18. Infections in industrialized countries occur sporadically or in outbreaks, tend to exhibit seasonality (late winter – early spring) and are observed mainly in crowded settings such as schools or military camps where transmission is readily facilitated. There are 13 antigenically distinct serogroups of N. meningitides. Serogroups B, C and Y account for the majority of disease in the United States whose annual incidence amounts to 1,000 cases 19. Neisseria meningitis is the second most common pathogen of community acquired meningitis 19 but meningococcal disease can present with extremely rapidly developing fatal outcomes in the absence of meningitis as in our two cases. Our second case is an example of Waterhouse-Friderichsen syndrome with extensive adrenal hemorrhages; a catastrophic development in a septic patient. Our first case had a similar clinical course but without the adrenal hemorrhage. This autopsy result surprised us, but serves to point out the unique virulence of this organism, as well as illustrating the important point that myocarditis during N. Meningococcus infections increases the mortality of this destructive pathogen. Meningococcus possesses an array of characteristics that serve to evade the host immune response, rendering havoc in individual cases 20. First, structural components of its service display molecular mimicry of host cells 20. Second, the organism employs genetic transformation- reuptake of DNA from other bacteria in the environment along with iron scavenging and chelation to a binding human lactoferrin that selects for survival and commensal state and allows for rapid multiplication and outer membrane proteins which promote attachment and host invasion 21. The unique structure of the outer membrane liposaccharide leads to bleb formation and release of potent endotoxin that quickly achieves high blood stream levels readily culminating in clinical sepsis 21. Finally, the inner bacterial capsule is well known to display resistance to complement phagocyte mediated lysis 22. One hundred years ago Waterhouse reported a disease that was humbling in its ferocity. A century has passed and our understanding is at a rewardable depth. We are equally impressed and humbled today. The authors have not received any funding or benefits from industry or elsewhere to conduct this study. Herrick W. 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