Question

Bill Grignard was well for the first 10 months of his life. In the next year he had pneumonia once, several episodes of otitis media (inflammation of middle ear) and on once occasion developed erysipelas (skin streptococcal infection) on his right cheek. These infections were all treated successfully with antibiotics but seemed to his mother, a nurse, that he was constantly on antibiotics.

His mother had two brothers who all died, 30 years prior to Bill’s birth, from pneumonia in their second year of life, before antibiotics were available. She also had two sisters who were well; one had a healthy son and daughter and the other a healthy daughter.

Bill was a bright and active child who gained weight, grew and developed normally but he continued to have repeated infections of the ears and sinuses and twice again pneumonia. At 2 years 3 months his local pediatrician tested his serum immunoglobulin is. He found 80 mg/ dl IgG ( normal is 600-1500 mg/dl), no IgA (normal is 50-125 mg/dl) and only 10 mg/dl IgM (normal is 75-150 mg/dl).

Bill was started on monthly intramuscular injections of gamma globulin; his serum IgG level was maintained at 200 mg/dl. He started school at age 5 years and performed well despite prolonged absences because of recurrent pneumonia and other infections.

At age 9 he was referred to the Children’s Hospital because of atelectasis (partial lung collapse) and a chronic cough. On physical examination he was found to be a well-developed alert boy. He weighed 33.5 kg and was 146 cm tall (normal). The doctor noted he had no visible tonsils (he never had a tonsillectomy). With a stethoscope the doctor also heard rales (moist crackles) at both lung bases.

Further family history revealed that Bill had one younger sibling, John, a 7 year old brother who also had contracted pneumonia on 3 occasions. John had a serum IgG of 150 mg/dl.

Laboratory studies at the time of Bill/s visit to the Children’s Hosptial gave a white blood cell count of 5100/ ul (normal) of which 45% were neutrophils (normal), 43% were lymphocytes (normal), 10% were monocytes (elevated) and 2% were eosinophils (normal).

Flow cytometry showed of lymphocytes, 85% were T cells ( 55% CD4, 29% CD8), however there was a complete lack of B cells (CD19+, normal 12%). Serum IgG remained low at 155 mg/dl and serum IgA and IgM were still undetectable.

3. Read the description paragraph below and identify/diagnose what the patient is experiencing (ie disease or immunological problem).

2. Once you identify the problem, think over what we have covered in class and describe what the outcomes of the disease or immunological problem is in the context of the immune system and where might there be deficiencies or problems with the normal immune system function.

3. Lastly, describe how the immune system would be working in a healthy individual. This could include a brief description of how the specific part of the immune system identified as being deficient normally works in a stepwise pathway that we cover in class. You can either use words to describe these pathways or you can draw pictures well annotated with high detail and include them as pictures with your word document you submit.

Answer 1

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