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CTLA-4 binds to ____, and serves to ____T-cell activation. _____binds to and inhibits CTLA-4.

A. B7, inhibit, Abatacept

B. CD28, stimulate, Etanercept

C. B7, stimulate, Ipilimumab

D. PD-1, inhibit, Adalimumab

E. B7, inhibit, Ipilimumab

E. CTLA-4 is a regulatory molecule expressed on T-cells that binds to B7 on antigen-presenting cells and downregulates T-cell activation. Ipilimumab is a monoclonal antibody that inhibits CTLA-4, inhibiting the inhibitor and increasing T-cell activation. Ipilimumab has been shown to have modest survival benefits in metastatic melanoma.

A patient presents with recurrent MRSA abscesses, thrush, and chronic lung infections. Physical examination reveals facial dysmorphism and retained deciduous teeth. The most likely mutation is in_____.

A. FoxP3

B. IL-23

C. STAT3

D. LFA-3

E. NFAT

C. Hyper IgE syndrome is characterized by patients with recurrent MRSA abscesses, thrush, and chronic lung infections leading to bronchiectasis and pneumatoceles. Hyper IgE syndrome can be caused by both STAT3 mutations and DOCK8 mutations, but patients with STAT3 mutations tend to have "coarse," dysmorphic facies and retained deciduous teeth, while patients with DOCK8 mutations tend to have viral infections and severe food allergies.

A patient presents with recurrent MRSA abscesses, thrush, and chronic lung infections. Laboratory examination reveals extremely elevated levels of IgE. Hyper IgE syndrome in DOCK8 deficiency can be distinguished from that occurring in STAT3 deficiency by:

A. Facial dysmorphism in cases of DOCK8 deficiency

B. Increased incidence of severe asthma and food allergies in STAT3 deficiency

C. Staphylococcal infections in STAT3 deficiency

D. Pneumatoceles in DOCK8 deficiency

E. Viral infections in DOCK8 deficiency

E. Hyper IgE syndrome is characterized by patients with recurrent MRSA abscesses, thrush, and chronic lung infections leading to bronchiectasis and pneumatoceles. Hyper IgE syndrome can be caused by both STAT3 mutations and DOCK8 mutations, but patients with STAT3 mutations tend to have "coarse," dysmorphic facies and retained deciduous teeth, while patients with DOCK8 mutations tend to have viral infections and severe food allergies.

A 6-month-old boy presents to the emergency department with fever and tachypnea, and is found to be in diabetic ketoacidosis. He has a history of intractable watery diarrhea ...

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