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Self-Assessment Questions


Immunodeficiency Disorders in Children: Review Questions

C. Lucy Park, MD

Dr. Park is an associate professor, Department of Pediatrics, University of Chicago Chicago, IL.

Choose the single best answer for each question.

1. A 7-month-old girl presents with chronic cough for 3 months and post-tussive vomiting for 3 weeks. The infant was born full-term with no neonatal complications, and she has never been exposed to tobacco smoke or pets. There is no history of asthma or recurrent infection in the family. Physical examination reveals a small, emaciated infant (weight at < 5th percentile, height at 10th percentile), extensive oral thrush, generalized lymphadenopathy (including cervical, axillary, inguinal, and suboccipital), bilateral intercostal and subcostal retraction, bilateral crackles, hepatosplenomegaly, and extensive diaper rash. Serum IgG, IgA, and IgM levels are all above normal. What is this patient’s most likely diagnosis?
  1. Adhesion molecule deficiency
  2. Asthma
  3. Bruton’s agammaglobulinemia
  4. HIV infection
  5. Severe combined immunodeficiency (SCID)
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2. A 20-month-old boy presents with a history of recurrent bruising, bilateral otitis media, pneumonia, and severe eczema. Complete blood count with differential count reveals thrombocytopenia (50 × 103/mm3), low mean platelet volume (5 fL), low serum IgM, and increased IgA and IgE levels. What is this patient’s most likely diagnosis?

  1. Ataxia-telangiectasia
  2. DiGeorge syndrome
  3. Hyperimmunoglobulinemia E syndrome
  4. Nezelof syndrome
  5. Wiskott-Aldrich syndrome (WAS)
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3. In which of the following conditions is regular intravenous immunoglobulin (IVIg) replacement therapy an absolute indication?
  1. AIDS
  2. Chronic granulomatous disease
  3. IgG2 subclass deficiency
  4. SCID
  5. None of the above
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4. A 12-year-old girl presents with history of recurrent pneumonia, otitis media, and sinusitis. She has had bilateral perforated tympanic membranes and mild hearing loss. Chest radiograph reveals bronchiectasis at the left lower and right middle lobes and infiltration at right lower and upper lobes. Total serum IgG, IgA, and IgM levels are normal. Her immunizations are current. Which of the following laboratory tests is the most appropriate next step in this patient’s evaluation?

  1. Total complement (CH50) and C3 and C4 levels
  2. HIV serology
  3. IgG subclass levels
  4. Serum antibodies to tetanus toxoid and pneumococcal antigens
  5. CD4/CD8 ratio
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5. Which of the following tests evaluates cell-mediated immune function in vivo?

  1. Antibodies to isohemagglutinins and Haemophilus influenzae
  2. Boyden chamber test
  3. Delayed hypersensitivity skin test (DHST) with candida antigen
  4. Nitroblue tetrazolium test
  5. T- and B-cell enumeration
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