MRCPH/FCPS PEDIATRIC MCQS: IMMUNE DEFICIENCY DISORDERS

MRCPH/FCPS PEDIATRIC MCQS

1. A 5-year-old girl attention-deficit/hyperactivity disorder and autism, with repair of tetralogy of Fallot in past, having frequent upper respiratory tract infections, and recurrent tinea capitis: Diagnosis?
2. A 6-year-old girl with severe eczema, recurrent staphylococcal abscesses, mild facial dysmorphism, and scoliosis: What will be the initial test?
3. A 1-month-old infant  with absent thymic shadow on chest radiograph; most appropriate next step in the care of this infant?
4. A 30-month-old boy with recurrent Klebsiella pneumoniae, as well as recurrent fevers and pyoderma; most likely diagnosis in this patient?
5. Newborn screening results positive for severe combined immunodeficiency (SCID). Which of the following is the most appropriate next step in the management of this patient?

MRCPH/FCPS PEDIATRIC MCQS

PEDIATRIC MCQS: IMMUNE DEFICENCY DISORDERS

Quiz

 

1. A 5-year-old girl, new to your practice, is brought to the clinic by her parents with concerns for attention-deficit/hyperactivity disorder and autism because of her cognitive and behavioral problems. Her medical history is significant for tetralogy of Fallot, which was repaired at 5 months of age, frequent upper respiratory tract infections, and recurrent tinea capitis. Which of the following is the most likely diagnosis to consider in this patient?
   1.   Aicardi-Goutières syndrome.
   2.   Chédiak-Higashi syndrome.
   3.   Chronic granulomatous disease.
   4.   DiGeorge syndrome.
   5.   Wiskott-Aldrich syndrome
2. A 6-year-old girl in your practice has a history of severe eczema, recurrent staphylococcal abscesses, mild facial dysmorphism, and scoliosis. Her older sister has similar symptoms and her mother has a history of recurrentskin and lung abscesses. Which of the following laboratory tests is the most appropriate initial test that is likely to be useful in making a diagnosis in this patient?
   1.   Total serum hemolytic complement level.
   2.   Dihydrorhodamine-1,2,3 flow cytometry.
   3.   Platelet count.
   4.   Quantitative immunoglobulins.
   5.   T-cell flow cytometry.
3. A 1-month-old infant, followed in your practice, is found to have an absent thymic shadow when a chest radiograph was obtained during an episode of bronchiolitis. A complete blood cell count demonstrates marked lymphopenia. Which of the following is the most appropriate next step in the care of this infant?
   1.   Initiate antibiotic prophylaxis with azithromycin.
   2.   Measure quantitative immunoglobulins.
   3.   Obtain a complete blood cell count with a manual differential count twice weekly for 6 to 8 weeks.
   4.   Refer to an immunology specialist
   5.   Test for human immunodeficiency virus infection using a nucleic acid–based test
4. A 30-month-old boy was hospitalized for operative drainage of bilateral cervical adenitis due to Staphylococcus aureus infection. His medical history includes a previous hospitalization for pneumonia with blood culture positive for Klebsiella pneumoniae, as well as recurrent fevers and pyoderma. Dihydrorhodamine-1,2,3 assay results are abnormal. Which of the following is the most likely diagnosis in this patient?
   1.   Ataxia-telangiectasia.
   2.   Chronic granulomatous disease.
   3.   Common variable immunodeficiency.
   4.   Cyclic neutropenia.
   5.   Leukocyte adhesion deficiency
5. Newborn screening results for an infant in your practice have just returned positive for severe combined immunodeficiency (SCID). Which of the following is the most appropriate next step in the management of this patient?
   1.   Measure quantitative immunoglobulin levels.
   2.   Order a gene sequencing panel for SCID.
   3.   Reassure the parents that this is likely a false-positive result.
   4.   Repeat the newborn screen.
   5.   Seek immediate specialty consultation