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Unit 8 – Immunodeficiencies Questions & Answers
What type of immunodeficiencies disorders are these?:
- DiGeorge syndrome
- Severe combined immune deficiency
- Chronic mucocutaneous candidiasis - ANS-Cellular immunodeficiencies
What type of immunodeficiencies disorders are these?:
- Ataxia-telangiectasia
- Wiskott-Aldrich syndrome - ANS-T-cell immunodeficiencies
What type of immunodeficiencies disorders are these?:
- Bruton x-linked agammaglobulinemia
- Common variable immunodeficiency
- Hyper IgM - ANS-B-cell and antibody immunodeficiency
What type of immunodeficiency disorder is Hyper IgE syndrome? - ANS-Innate immune disorders
What disorder is this?:
- Congenital T-cell defect
- Aplasia of parathyroid and thymus gland
- Pharyngeal pouch syndrome
- Heart defects at birth - ANS-DiGeorge syndrome
DiGeorge syndrome is usually fatal..... - ANS-in the first year of life
What disorder is this?:
- Genetic causes
- Autosomal recessive or X-linked recessive forms
- Bacterial, fungal and viral diseases
- Respiratory and intestinal issues - ANS-Severe combined immunodeficiency
Severe combined immunodeficiency is usually deadly by age _________. - ANS-
What disorder is this?:
- T-cell defect
- Fungal infections of scalp, skin and nails, also Thrush
- T-cells don't recognize Candida antigen
- Deadly from adrenal insufficiency
- Treatable with anti-Candida antisera - ANS-Chronic mucocutaneous candidiasis
Chronic mucocutaneous candidiasis is treated with what? - ANS-anti-Candida antisera
In what immunodeficiency disorder are T-cells unable to recognize Candida antigen? - ANS-Chronic mucocutaneous candidiasis
What disorder is this?:
- Disturbance of gait and posture
- Superficial abnormalities of the blood vessels
- Lymphs decreased
- Chronic respiratory infections
- IgA secretory component abnormal
What disorder is this?:
- Seen in very young to young adults
- One or many Ig's are absent or decreased
- Patients have sinus and pulmonary infections
- B-cells fail to become plasma cells
- Excessive numbers of T-regulator cells - ANS-Common variable immunodeficiency
What disorder is this?:
- X chromosome mutation
- Increased IgM, extremely low IgA
- Recurrent infections
- Neutropenia
- Aplastic anemia - ANS-Hyper-IgM
In Hyper-IgM what immunoglobulin is increased? - ANS-IgM
In Hyper-IgM what immunoglobulin is extremely low? - ANS-IgA
What is Job syndrome also known as? - ANS-Hyper-IgE syndrome
What disorder is this?:
- Aka as Job syndrome
- Recurrent Staphylococcal abscesses
- Treatable with bone marrow transplant or IgG replacement therapy - ANS-Hyper-IgE syndrome
What are the 2 ways to treat Hyper-IgE syndrome? - ANS-bone marrow transplant or IgG replacement therapy
What are the 4 categories of the primary immunodeficiency disorders? - ANS-1. cellular immunodeficiencies
- T-cell immunodeficiencies
- B-cell and antibody immunodeficiencies
- Innate immune disorders
What disorder is a congenital T-cell defect where there is lost genetic material chromosome 22? - ANS- DiGeorge syndrome
What is DiGeorge syndrome also known as? - ANS-congenital thymic hypoplasia
What is a facial and vascular abnormality found in newborns that presents either as the absence of the thymus and parathyroid glands or a malformation of the heart and blood vessels? - ANS-Pharyngeal pouch
What 3 infections are people with DiGeorge syndrome prone to? - ANS-1. acid-fast bacillus
- listeria
- pneumocystis
A life-threatning infection that begins at about six months would be a sign/symptom of what disease? - ANS-Severe combined immune deficiency
What disorder is this?:
- neisseria infections
- respiratory infections from pneumocystis
What is an X-chromosome mutation that occurs in the first or second year of life? - ANS-Hyper-IgM immunodeficiency
What 3 recurrent infections do people with Hyper-IgM Immunodeficiency have? - ANS-1. otitis media
- sinusitis pneumonia
- tonsillitis
Due to what disorder do patients often die before puberty from opportunistic infections B-cell lymphomas? - ANS-Hyper-IgM immunodeficiency
Patients with which disorder generally have aplastic anemia? - ANS-Hyper IgM disorder
Which disorder is characterized by a disturbance of gait and posture? - ANS-Ataxia-telangiectasia
Which yeast is the cause of issues with the chronic mucocutaneous disease? - ANS-Candida sp.
Which disease is characterized by the hosts pharyngeal pouch syndrome? - ANS-DiGeorge syndrome
People with which disorder have more sinus and pulmonary infections than is normal? - ANS-Common variable immunodeficiency
Patients with this syndrome tend to also have thrombocytopenia, identify the disease. - ANS-Wiskott- Aldrich syndrome
This disorder is seen around 6 months of age, the patient has multiple bacterial infections and their B- cell populations are absent. - ANS-Bruton x-linked agammaglobulinemia
