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| Case 1 Presentation |
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On physical examination, the child is comatose. Her temperature is normal, pulse is 135 beats/min, and respiratory rate is 50 breaths/min. Her pupils are 3 mm in diameter and respond sluggishly to light. There are no signs of head injury, her neck is supple, and the tympanic membranes and fundi appear normal. Copious bloody secretions are suctioned from her nose and mouth. Respirations are deep and noisy, with scattered rhonchi audible. Cardiac examination reveals tachycardia in what appears to be a normal rhythm. The abdomen is soft and nontender, with no palpable masses or organs and active bowel sounds. There is no skin rash.
The child passes a large, bloody stool and resumes seizure activity, developing severe respiratory distress that requires endotracheal intubation. Intravenous phenobarbital and midazolam are administered to control the seizures. She has a blood glucose level of 18.5 mmol/L (333 mg/dL). A complete blood count; determinations of sodium, potassium, calcium, magnesium, and ammonia levels; cerebrospinal fluid analysis; urine dipstick and toxicology testing; and computed tomography of the head all yield normal results. Three hours after arrival, still receiving mechanical ventilation and oxygen, her Glasgow Coma Score is 3 and blood pressure is 114/89 mm Hg.
| Case 2 Presentation |
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