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Question:

A 3-year-old boy is brought to the emergency department due to high fever, vomiting, and lethargy.  His symptoms started 1 day ago but rapidly worsened over the last few hours.  Physical examination shows hypotension, tachycardia, neck stiffness, and a petechial rash over the trunk and lower extremities.  Laboratory results are as follows:

Complete blood count
    Hemoglobin12.0 g/L
    Platelets80,000/mm3
    Leukocytes34,500/mm3
        Neutrophils66%
        Band forms20%
       Lymphocytes14%
Chemistry panel
    Blood urea nitrogen30 mg/dL
    Serum creatinine1.8 mg/dL
    Blood glucose50 mg/dL

While being evaluated, the patient develops apnea and asystole.  Despite aggressive resuscitation efforts, the patient dies.  Autopsy would most likely reveal which of the following?

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Explanation:

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Meningococcal meningitis

Epidemiology

  • Neisseria meningitidis
  • Most common in children and young adults

Pathophysiology

  • Nasopharyngeal colonization
  • Epithelial cell invasion
  • Immune system evasion (eg, capsule, variable pili)

Clinical features

  • Fever, headache, vomiting, severe myalgias
  • Petechial/purpuric rash
  • Altered mental status, neck stiffness

Complications

  • Septic shock, multiorgan failure
  • Adrenal hemorrhage/insufficiency
  • Disseminated intravascular coagulation

This patient's rapidly worsening fever, vomiting, altered mental status, and nuchal rigidity are consistent with meningococcal meningitis due to infection with Neisseria meningitidis.  Meningococcal infection usually occurs in children and young adults and can present acutely with rapid progression (within hours) to meningococcemia and septic shock.  These patients frequently have thrombocytopenia and a petechial rash and are at risk for development of disseminated intravascular coagulation.

A serious complication of meningococcemia is bilateral hemorrhagic infarction of the adrenal glands, resulting in acute adrenal insufficiency (Waterhouse-Friderichsen syndrome).  Manifestations include hypoglycemia due to cortisol deficiency as well as hyponatremia and hyperkalemia due to aldosterone deficiency.  Loss of cortisol and aldosterone also contributes to worsening shock and can result in rapid deterioration and death despite aggressive intervention.

(Choices B and E)  Both cardiac tamponade and a saddle pulmonary embolus can cause obstructive shock that results in multiorgan failure.  However, shock due to these conditions is not usually associated with adrenal insufficiency, and this patient's other clinical features (eg, neck stiffness, rash) suggest meningococcal meningitis as the primary cause.

(Choice C)  Intracerebral hemorrhage with increasing intracranial pressure can present with severe headache, vomiting, altered mental status, loss of consciousness, and death.  Neck stiffness may be present if the hemorrhage extends into the subarachnoid space and irritates the meninges.  However, this patient's fever, petechial rash, and adrenal insufficiency make meningococcal meningitis more likely.

(Choice D)  Necrotizing pancreatitis typically presents with worsening abdominal pain and vomiting, and if infected can lead to septic shock and death.  However, it is not associated with neck stiffness and a petechial rash.

Educational objective:
Meningococcal meningitis is a rapidly progressive infection that can spread systemically (ie, meningococcemia) and lead to septic shock, multiorgan failure, and disseminated intravascular coagulation.  Meningococcemia is also associated with bilateral hemorrhagic infarction of the adrenal glands, which can result in acute adrenal insufficiency (Waterhouse-Friderichsen syndrome) with worsening shock and rapid clinical deterioration.