Final Case Study - Cal State LA - Instructional Web Server最后的案例研究-加州大学洛杉矶-教学的Web服务器.pptVIP

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Final Case Study - Cal State LA - Instructional Web Server最后的案例研究-加州大学洛杉矶-教学的Web服务器.ppt

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Final Case Study - Cal State LA - Instructional Web Server最后的案例研究-加州大学洛杉矶-教学的Web服务器

Final Case Study Case # 4 Nancy Lee, Andrea Ustarez, Luis Castellon CSULA Case Summary A 3-year-old girl was diagnosed with pneumonia, caused by Streptococcus pneumoniae. She had fever, high respiratory rate, and low blood oxygen saturation. Her lymph nodes were enlarged and a chest X-ray showed diffused consolidation of the lower lobe of the left lung. She had pneumonia before, when she was 25 months old. She also had 10 episodes of otitis media that required antibiotic treatment. Her immunoglobulin levels were measured: high levels of IgM, undetectable levels of IgA, and low levels of IgG. She was previously vaccinated against tetanus and Haemophilus influenzae, but she did not express specific IgG against the tetanus toxoid or the polyribosyl phosphate (PAP) polysaccharide antigen of H. influenzae. She had blood type A so she was tested for anti- antibodies. Her IgM titer of anti-B antibodies was positive at the upper limit of normal, her IgG titer was undetectable. Her peripheral blood lymphocytes were analyzed showing normal expression of CD40 ligand on T cells activated by anti-CD3 antibodies, and normal expression of CD40 on B cells. Her blood cells completely failed to secrete IgG and IgE after stimulation with anti-CD40 antibody (to mimic the effects of engagement of CD40 ligand) and interleukin4 (IL-4). Key Information Pointing to Diagnosis CLINICAL Features 3 years old Recurrent infections Pneumonia 25 mo ago 10 episodes of otitis media Streptococcus pneumonia found in blood Enlarged lymph nodes in neck and armpits LABORATORY Features High IgM 470 mg/dl (normal 40-240 mg/dl) Low IgG 40 mg/dl (normal 639-1344 mg/dl) Undetectable IgA Upon vaccination no specific IgG antibodies Treatment w intravenous antibiotic showed improvement Failure to secrete IgG and IgE after stimulation Normal expression of: CD40 L on T cells CD40 on B cells The Diagnosis for Case # 4 HIGM2 - Hyper IgM syndrome type 2 (AID deficiency). Diagnostic Tests for Activation