Age: 1.3
Sex: M
Vaccine: HIBV
Symptoms: Neurological examination abnormal, Neutrophil count, Neutrophil count decreased, Neutrophil morphology abnormal, Nuclear magnetic resonance imaging abnormal
Clinical narrative: Initial report retrieved from the scientific literature on 15 November 2010. It is a case report taken from the following source: The following is verbatim from the article: "A 15-month with no significant past medical history presented to the emergency department for 2 days Tmax of 102 degrees F, lethargy and decrease oral intake and urine output. There was vomiting the day before admission. He was seen by his physician who diagnosed otitis media and referred him to the ED because of a concern for the level of consciousness. Immunizations were up-to date (3 DTap and Hib vaccinations, with the last being 9 months ago) except for VARIAVX which he did not receive secondary to a manufacturer shortage. Family history included a 3-year-old brother with reactive airways disease. Social history was notable for attendance at a church daycare where many children were not immunized. On arrival to the ED, vital signs were as follows: temperature, 36.5 degrees C; heart rate, 133 beats per minute; blood pressure, 81/62 mm Hg; respiratory rate, 24 breaths per minutes; and pulse oximetry, 97% on room air. Physical examination revealed a child with a weak cry who did not maintain eye contact with the examiner. Stiffness of his neck was noted with flexion and extension. The right tympanic membrane was erythematous and bulging. Pulses and perfusion were normal. Leukocyte count was 3 x 10E9/L white blood cells (0.23 segmented neutrophils and 0.55 bands) with Dohle bodies and vacuolization noted on the smear. Two 20-mL/kg saline boluses were given with no improvement in mental status. A blood culture was obtained before ceftriaxone 100 mg/kg was given intravenously. Soft tissue radiographs and computed tomographic scans of the neck ruled out retropharyngeal abscess but revealed opacification of both maxillary sinuses. Cloudy cerebrospinal fluid was obtained from lumbar puncture. Immediately after the procedure and before laboratory results, vancomycin was given. Cerebrospinal fluid had the following: white blood cell count, 3.45 x 10E9/L (0.59 neutrophils); red cell count, 1 x 10E9/L; glucose, 0.6612 mmol/L, and protein, 2.98 g/L; CSF was also positive by Phadebact Haemophilus Co-Agglutination test, manufactured by BOULE Diagnostics, Foreign. When the Gram stain showed gram-negative rods, meropenem was added. The hospital microbiology laboratory serotyped the organism as type b, which was confirmed by the state laboratory. The patient was admitted to the pediatric intensive care unit with a diagnosis of meningitis and sepsis. Mental status improved on day 2. Dexamethasone was started, and antibiotics were switched to ceftriaxone alone when blood and CSF cultures returned positive for Hib sensitive to ampicillin. Evaluation for the immunologic response to Hib and immunodeficiency was initiated on day 3, but results were not available during the hospital stay. The patient was transferred to the floor after results of CT scan of the head were normal on day 4. A lumbar puncture was planned on day 7, but the parents refused the procedure. Day 8 was the last day that fever (Tmax, 38.8 degrees) was present. Blood and urine cultures were sent; vancomycin was added for a possible central venous line infection. Magnetic resonance imaging on day 9 demonstrated a thin dural hygroma over the left cerebral convexity that measured approximately 4mm in thickness but normal spleen for age. On day 12 of hospitalization, vancomycin was discontinued. The patient was discharged after 13 days of antibiotics with appointments scheduled with neurology and immunology. The pediatrician was to arrange audiologic with appointments scheduled with neurology and immunology. The pediatrician was to arrange audiologic evaluation and early intervention; further immunologic evaluation was planned. The patient returned to the ED 48 hours after discharge with focal seizure activity. On the day of admission, the patient was afebrile and was more active than in previous days. Parents described right side shaking that lasted several minutes. After the seizure, he was noted to have decreased movement of the right upper and lower extremities. Vital signs in the ED included the following: temperature, 37.9 degrees C; heart rate, 96 beats per minute; respiratory rate, 28 breaths per minute; pulse oximetry, 99% on room air (no blood pressure was recorded). Physical examination revealed an awake child in no immediate distress. Anterior fontanelle was open and flat; pupils were equally round and reactive. No papilledema was noted, and extraocular eye movements were intact. Neurological examination was significant for flaccid right lower extremity and a weak right upper extremity with normal reflexes and sensation. The remainder of the physical examination was unremarkable. There was improvement of the right hemiparesis during his ED stay. Workup in the ED included CT scan of the brain, which demonstrated a left-sided hygroma without midline shift. He then underwent a lumbar puncture that obtained clear fluid with opening pressure of 17 cm H20. Results of analysis were as follows: white blood cells, 0.064 x 10E9L (o.11 neutrophils and 0.77 lymphocytes)l red blood cells, 0.017 x 10E9/L; glucose, 3.94121 mmol/L; and protein, 1.14 g/L. Gram stain demonstrated rare white blood cell count and no organisms. Bacterial antigen panel was negative as was the final culture. White blood cell count was 24.7 x 10E9/L with 0.81 neutrophils, 0.11 lymphocytes, 0.06 monocytes, and 0.02 basophils. Electrolytes and liver profile were normal. Urine toxicology was negative. Neurology and infectious disease consultations were obtained. Ceftriaxone was given intramuscularly per recommendation of the infectious disease specialist because intravenous access was not obtained immediately. Shortly after the lumbar puncture was completed, the patient had generalized tonic-clonic seizure characterized by lip smacking for which he received 1 mg of lorazepam and 15 mg/kg of fosphenytoin intravenous with resolution. During transport to the PICU, he had recurrence of seizure activity. On transfer to the PICU bed, he became apneic and bradycardiac. At that time, his pupils were fixed and dilated. Cardiopulmonary resuscitation was initiated, and his trachea emergently intubated. Mannitol was given along with additional doses of lorazepam and resuscitation medications. Hypertonic saline was infused for presumed cerebral edema. The pediatric neurosurgeon performed a ventricular tap that produced 7 mL of purulent CSF. A ventricular catheter as then placed and yielded 40 mL of CSF, but resuscitation was unsuccessful. Autopsy demonstrated severe acute and chronic leptomenigitis described as multilayer exudates with neutrophils, lymphocytes, and proliferating capillaries. There was subdural empyema with organizing pachymeningitis of the left frontal region and diffuse brain edema with acute neuronal injury. No evidence of herniation was noted at autopsy. Immunologic workup from the first hospitalization included quantitative immunoglobulins with an IgG of 4.91 g/L (range, 0.40-2.50 g/L) both at the lower end of normal for age. No significant tetanus antitoxoid antibodies at 0.08 IU/mL (reference, 0.16 IU/mL) or Hib antibodies at 0.13 mg/L (reference, 0.15 mg/L) were detected. Diphtheria antitoxoid antibodies were present at 0.20 IU/ML (reference, 0.10 IU/mL). Varicella IgG was also negative at 0.81 mg/L (reference, 0.90 mg/L) with vaccination occurring approximately 3 weeks before assessment. There were only 2 increased pneumococcal antibody IgG serotypes despite the receipt of his fourth PREVNAR 4 months before presentation. In vitro mitogen and antigen stimulation studies, which were planned by the Immunologist on an outpatient basis, were never completed. DISCUSSION: The poor outcome may be related to the patient's immunologic deficiency, which, perhaps, did not completely eradicate the organism despite and adequate course of antibiotics..." Documents held by sender: None