Sex: M
Vaccine: HIBV
Symptoms: Ear tube insertion, Fatigue, Inappropriate schedule of product administration, Malaise, Tonsillectomy
Clinical narrative: Six months later, he received his second Hib vaccination; This literature marketed report has been received from the authors of a published article, titled as stated below, and refers to a boy, who was 1-year-old at the time of the report, with Zhu-Tokita-Takenouchi-Kim (ZTTK) syndrome (confirmed by genetic testing) due to a de novo mutation in the SON gene (c.2250_2280del31). He had a urinary tract infection diagnosed after birth requiring antibiotics for both treatment and subsequent prophylaxis for vesicoureteral reflux. His history was notable for multiple upper respiratory infections and recurrent acute otitis media. He did not have any confirmed pneumonia or skin infections. On an unknown date, the patient presented at an immunology clinic for evaluation of recurrent infections (reported as Francisella tularensis infection). On an unknown date, laboratory evaluation initially revealed low immunoglobulin (Ig) level across all classes (IgG, 308 mg/dL range: 389-1260 mg/dL; IgM, 42 mg/dL, range: 38-195 mg/dL; IgA, 9 mg/dL, range: 31-47 mg/dL). He was not vaccinated at the time of the initial encounter, so vaccine titers were not assessed. A lymphocyte subset panel result was normal. The patient was diagnosed with hypogammaglobulinemia given his deficiencies in all classes of Igs. On an unknown date (reported as subsequently), the patient received his first round of vaccinations: pneumococcal conjugate 13 and combined diphtheria (+) tetanus (+) pertussis (+) Haemophilus influenzae B (Hib) (+) inactivated polio vaccine, but developed a febrile seizure the next day. At baseline, pneumococcal immunization titers revealed 4 of 23 protective serotypes (more than or equal to 1.3 microgram/mL). On an unknown date (reported as 2 months after vaccination), it was reported that antibody titer to Hib was adequate (84.7 microgram/mL, range: more than to 0.10 microgram/mL).  On an unknown date (reported as six month later), the patient was vaccinated with a dose of Haemophilus b Conjugate Vaccine (Meningococcal Protein Conjugate) (manufacturer unknown) (reported as his second hib vaccination), formulation was defaulted to intramuscular Injection (dose, route of administration, anatomical location, lot number and expiration date were not reported) for prophylaxis (inappropriate schedule of product administration).  On an unknown date (reported as afterward [the vaccination]), the patient was fatigued and seemed unwell. The patient's mother declined further vaccinations. On an unknown date, the patient's diphtheria titer was 0.0 IU/mL (range: more than 0.10). It was reported that the patient had an adequate response to tetanus vaccination with a titer of 15.0 IU/mL (range: more than 0.10 IU/mL). Lymphocyte proliferation to mitogen stimulation was within normal limits, with maximum proliferation to pokeweed mitogen at 18.5% of CD45 (reference: more than or equal to 4.5%), 23.1% of CD3 (reference: more than or equal to 3.5%), and 85.8% of CD19 (reference: more than or equal to 58.5%). The patient had diminished candida response (maximum proliferation of candida for CD45 was 0.8% [reference: more than or equal to 5.7%] and for CD3 was 1.0% [reference: more than or equal to 3.0%]), but he had normal tetanus response on lymphocyte proliferation to antigens. Absolute CD values were reported as: CD3 of 2067 cells per microliter (range: 2100-6200 cells per microliter), CD4 of 1174 cells per microliter (range: 1300-4300 cells per microliter), CD8 of 8787 cells per microliter (range: 620-2000 cells per microliter), CD19 of 1031 cells per microliter (range: 720-2600 cells per microliter), CD56 of 130 cells per microliter (range: 126-729 cells per microliter) and CH50 of 59 cells per microliter (range: 101-300 cells per microliter). It was reported that diminished candida response can be related to his young age and that the patient had a humoral deficiency. The patient's Igs spontaneously recovered: repeat IgG, IgM and IgA were 429 mg/dL, 61 mg/dL and 24 mg/dL, respectively. This was noted at a follow-up after tonsillectomy, adenoidectomy, and bilateral tympanostomy tube placement. It was reported that based on the limited vaccinations received, he had adequate antibody response. It was reported that as such, no treatment was implemented.  A copy of the published article is attached as further documentation of the patient's experience.  This is one of several reports derived from the same literature article.  Lot number is being requested and will be provided if available.  Sender's Comments:  US-009507513-2407USA002561: