Sex: F
Vaccine: UNK, UNK, UNK
Symptoms: Anion gap increased, Antibody test negative, Blood bicarbonate decreased, Blood chloride decreased, Blood potassium decreased
Clinical narrative: Immune evaluation resulted in low antibody titer to Hib despite appropriate vaccination/Immune evaluation resulted in low antibody titer to Hib despite appropriate vaccination; This literature marketed report has been received from the authors of a published article, titled as stated below, referred to 12-month-old female patient.   On an unknown dates, the patient was vaccinated with 3 doses of Haemophilus b Conjugate Vaccine (Meningococcal Protein Conjugate) (Manufacture Unknown), (dose, strength, frequency, vaccination scheme, route, anatomical location, lot number, and expiration date were not reported), administered as prophylaxis.  The 12-month-old female with no pertinent medical history on an unknown date, presented to the emergency room for fever and emesis, she was febrile to 101.4°F, tachycardic to 170 and respiratory rate of 50; her initial labs were notable for hyponatremia 127 mmol/L, hypokalemia 3 mmol/L, hypochloremia 90 mmol/L, bicarbonate 14 mmol/L, and anion gap 23 mmol/L. She was admitted to pediatrics for fluid resuscitation and electrolyte management. She became more somnolent and began having seizure-like activity, and posturing; so she was transferred to the Pediatric Intensive Care Unit (PICU) and intubated; an electroencephalogram (EEG) was conducted revealing seizures, and magnetic resonance imaging (MRI) brain revealed meningitis and ventriculitis with hydrocephalus; lumbar puncture findings of purulent cerebrospinal fluid (CSF), protein elevated 368?mg/dL and glucose resulted low less than 2mg/dL; CSF and blood cultures positive for nontypeable haemophilus influenzae (NTHi). Immunological studies later revealed that although she received three Hib vaccines, she had inadequately low levels of antibodies, less than 0.15 mcg/mL, she also had low CH50 complements of less than 12.5?U/mL. It was reported that her decreased complement was likely to be the cause of her susceptibility and lethality of her NTHi meningitis. Unfortunately, despite appropriate antimicrobials and aggressive neuroprotective strategies, she developed severe cerebral edema leading to herniation, underwent brain death testing and was declared brain dead.  Her immune evaluation resulted in low antibody titer to Hib despite appropriate vaccination (Vaccine impaired response), and total hemolytic complement (CH50) was undetectable; there was concern that this patient had inherited complete deficiency in either the classical complement pathway or the terminal complement pathway, both are important for protection from NTHi. She appeared to have a specific antibody deficiency to polysaccharide antigens, as seen with her low antibody levels to Hib and Streptococcus pneumoniae. The lack of response to Hib vaccines would not have offered protection against her NTHi.  At the time of reporting, the outcome of vaccination failure was unknown. The authors considered the event of vaccination failure as related to the Haemophilus b Conjugate Vaccine (Meningococcal Protein Conjugate) (Manufacture Unknown). A copy of the published article is attached as further documentation of the patient's experience  Additional information is not expected.   Literature Citation