(B Gaspar & K C. Gilmour)

• Identification of lymphocyte subsets is an important first line investigation for:

1. SCID.

2. XLP.

3. XLA.

4. WASP.

5. XHM.

• Protein based diagnosis can be used as an effective screening tool because:

6. it identifies the genetic defect.

7. it is rapid.

8. it always identifies the carrier.

9. it is more cost effective.

10. it can direct other investigations.

• T-B+ SCID can be caused by defects in the:

11. common gamma chain.

12. the common alpha chain.

13. JAK1.

14. JAK2.

15. JAK3.

• The following abnormalities in protein expression conclusively indicate a disease causing abnormality

16. protein size is aberrant.

17. protein fails to be expressed.

18. protein is tyrosine phosphorylated after stimulation.

19. multiple non-specific bands are seen on immunoblot analysis.

20. over 78% of cells express the protein by flow cytometry analysis.

(S Deacock)

• In individuals with immediate /Type I hypersensitivity reactions to latex:

21. co-existent food allergies are rare.

22. clinical symptoms are always present if sIgE to latex is demonstrable.

23. low-protein latex gloves may always be used safely.

24. powdered gloves may cause more severe symptoms than non-powdered gloves because rubber additives bound to the cornstarch particles may be inhaled.

25. immunotherapy is widely used as treatment.

• Type IV hypersensitivity reactions to latex are:

26. due to T cells sensitised to chemicals added to the latex during manufacturing.

27. best diagnosed by measurement of sIgE.

28. a recognised risk factor for the development of Type I hypersensitivity.

29. the commonest type of skin reaction caused by rubber gloves.

30. a frequent cause of respiratory symptoms in latex-allergic subjects.

• In the diagnosis of latex allergy:

31. Hundreds of different polypeptides and proteins have been shown to stimulate sIgE production.

32. Commercially available in vitro assays for sIgE to latex proteins give very similar sensitivity and specificity rates.

33. Skin prick testing is the single most reliable diagnostic method for Type I hypersensitivity.

34. Patch testing may aid in diagnosis of “rubber glove eczema”.

35. Anaphylaxis has never been reported after skin prick testing.

(M Helbert & C Morgan)

• The following statements are true

36. HLA typing has a strong positive predictive value in Behçet’s disease in Caucasian patients.

37. Neuro radiology is non specific in Behçet’s disease.

38. Gut biopsies are non specific in Behçet’s disease.

39. Children with oral ulcers and raised IgD are unlikely to have Behçet’s disease.

40. Clinical diagnostic criteria are reliable in diagnosing Behçet’s disease.

• The following statements are true

41. TH1 cytokines drive granuloma formation in Behçet’s disease.

42. Antibodies are implicated in Behçet’s pathogenesis.

43. TH1 cytokines increase expression of endothelial cell antigens.

44. Antibodies to endothelial cell antigens characteristically bind to small vessels in Behçet’s disease.

45. There is clinical evidence for the efficacy of anti TNF monoclonal antibodies in Behçet’s disease.

• The following statements are true

46. Cyclophosphamide is useful in CNS and eye disease in Behçet’s disease.

47. Cyclosporin is useful in CNS and eye disease in Behçet’s disease.

48. Topical steroids are useful in Behçet’s disease.

49. Immunosuppressive drugs are often continued after clinical remission.

50. Immunosuppressive regimes should be continued in Behçet’s disease until the ESR has been normal for several months.

(R Pumphrey)

• The first treatment for a severe acute allergic reaction in a shocked but conscious 11-year-old should be

51. intravenous injection of 5mL 1:10,000 epinephrine over 5 minutes.

52. intramuscular injection 0.5mL 1:1000 epinephrine.

53. subcutaneous injection of 0.25 mL 1:1000 epinephrine.

54. intramuscular injection of 0.25mL 1:1000 epinephrine.

55. intramuscular injection 300microgram by epinephrine autoinjector.

• Which of the following give information useful for forecasting a future fatal allergic reaction to peanuts?

56. strongly positive skin prick test.

57. high level of specific IgE.

58. positive open challenge test.

59. positive double blind challenge test.

60. history of previous severe reactions.

61. none of the above.

• Which of the following statements about food allergy management strategies is true?

62. Anyone with peanut allergy can be trained to avoid peanuts completely.

63. Randomised controlled trials have proved the effectiveness of epinephrine in managing anaphylactic reactions.

64. Improving daily asthma management will save more lives than giving epinephrine for self-treatment.

65. Epinephrine self-treatment has been prescribed so widely that now most have it who might need it.

66. Epinephrine is the most important first treatment for food anaphylaxis because shock is the commonest cause of death.

(AR Gennery & M Abinun)

• Which statement is True or False:

67. A lymphocyte count of 1.5 x 10⁹/L is the lower limit of normal in an infant.

68. A lymphocytosis excludes SCID.

69. SCID immunophenotype is of research value only and has no prognostic significance.

70. BMT for SCID is successful less than 60% of the time.

71. In utero BMT is the treatment of choice when there is a positive foetal diagnosis.

72. JAK3 deficiency has been successfully treated by gene therapy.

(M Peakman)

• Which of the following statements is true or false about Type 1 diabetes:

73. Autoantibodies to glutamic acid decarboxylase are the best predictive marker of diabetes

74. Radioligand binding assays with recombinant islet autoantigens are the assays of choice for islet cell autoantibodies

75. Although islet-reactive T cells are thought to cause diabetes, it is impossible to detect them in peripheral blood.

76. Islet cell autoantibodies are rare in patients with long-standing (>20 years) disease.

77. The strongest genetic association with Type 1 diabetes is HLA-DR4 (B1*0401)

78. Rubella infection is associated with autoantibodies to insulin and phogrin.