Flashcards in Hogan- Primary immunodeficiency Diseases Deck (52):
What is the most likely failure of immunity?
What about second most likely?
Antibody defect (B cell)
T cell disorders
innate and complements
Leukocyte development has passed from (blank) to bone marrow BEFORE birth
Is phagocyte functionality complete in neonates?
Is complement function available to neonates?
yes via maternal passive transfer of IgG
(Blank) will lage until antibody production is initiated.
Antibody mediated complement fixation (self)
Is NK function available in neonates?
When is T cell function complete?
at birth :)
T cell maturation is required to complete B cell maturation to functional antibody function. What does this mean?
It means that T cell function is available at infancy, but B cell function lags for 6-24 months. (i.e if T cells to develop, B cell functionality will suffer)
Autoimmune problems in patient/family members suggest some difficulty with (blank) at possibly a genetic level.
B cell maturation/or tolerance issues
Does immunodeficiency develop before or after autoimmunity?
Most immune defenses are (blank) in nature as a significant number of autoimmue issues are failure of B cell maturation/isotope switiching/ or tolerance
What are some severe infections of the innate immunity?
lymphadenitis, osteomyelitis, pneumonia, and sepsis
Are phagocytes nondiscriminatory? WHat does this mean
they attack both gram + and gram - and yeast/fungal organisms are attacked
When you have a catalase producing organism, what kind of disorder should you be thinking about?
CGD (neutrophil disorder)
What are some CGD organisms?
What are common problems in B cell disorders?
What organisms are associated with B cell disorders?
When is getting otitis media worrisome?
after middle school
When you get an infection from a rar or odd organisms, what kind of problem should you suspect?
What are some of the weird organisms associated with T cell problems?
candida: invasive (lung/esophagus)
system viral illness (CMV etc.)
mycobacterial infections: systemic
What are the clinical characteristics of T cell deficiency?
onset before 6 months
no lymph nodes
increased chance of cancer
failure to thrive
GvH disease after transfusion
Severe fungal/viral infection
fatality after BCG
What are the clinical characteristics of B cell deficiency?
Famiy history (autoimmune/immunodeficiency)
onset after 6 months
recurrent virulent bacterial infections
failure to thrive
What are the clinical characteristics of phagocytic deficiencies?
severity range mild to severe
susceptible to low grade bacteria, fungus
severe infections (pneumonia/osteomyelitis)
skin infections/ furunculosis
delayed separation of the umbilical cord
What complement will be deficient if you get recurrent bacterial infections?
What complement will be deficient if you have nesseria infections?
What disease has neutrophils that have azurophilic granules?
What disease has bi-lobed nucleus in the neutrophils?
specific granule deficiency
If you have neutrophilia what should you consider?
LAD d/o or infection
If you have neutropenia what should you consider?
congenital absence, autoantibody, cyclic neutropenia
If you have small platelets, and decreased number of platelets what should you consider?
(Wiskott aldrich syndrome)
If you have an RBC abnormality what should you consider?
autoimmune anemia and associated G6PD deficiency
If you have lymphopenia what should you consider?
SCID in an infant
If you have a genetic deficiency somewhere in the complement cascade what will the CH50 be? how else can you get this number
profound sepsis with DIC: consumptive and will take 4-6 weeks to replenish cascade
If you have a genetic deficiency somewhere in the complement cascade what wil the AH50 be?
(could be consumption)
If you AH50 and CH50 are both zero. where could you deficiency be?
C3 or C5-9 because these are shared by both assays
What are the 5 immunology labs you want to order?
What does albumin tell you?
it determines if IgG loss is secondary
What IgA level is considered good?
any number greater than botom of measurable assay is good despite statistical reporting.
If a child has nasal polyposis what should you look for?
Before you look for a B cell deficiency in chidren, what 2 things might you look into?
immotile cilia syndrome
What can functional responses to vaccines tell you?
can tell you what antibodies arent being produced.
I.e if you respond to protein components but not polysaccharide componenets etc.
If you have IgM, what does this tell you?
that you have titer function
If you have failure in a vaccine you can do an advance test which is what?
its a booster dose that will check for memory cel function and plasma cell production.
If you give an advanced test and al the Igs are decreased what should you be thinking?
CD3, CD4, CD8, CD19, CD56
ie check the CDs
If CD19 (which is B cells) is low, what should you be thinking is the culprit?
( if all Bs and Cs are messed up think SCID)
If you are suspecting T cell disorder, what should you look into?
HIV (secondary immunodeficiency)
CD3 CD4 CD8 CD19 (B cell), CD56 (NK cells)
What are some ways you can check T cell function?
lymphocyte stimulation with mitogens
specific antigen studies (PPD if BCG vaccinated)
TREC analysis of thymic output
FISH: 22q11 (DiGeorge)
What is the sailboat that you see on a neonatal CXR?
the thymus :)
If your suspicious of a phagocyte disorder what do you look up?
CD11/18 flow cytometry
serial neutrophil counts
Bactericidal assay (function)
When does pneumonia in childhood become a red flag?
the third !!!
If someone says they had pneumonia what do you want to ask?
was there a chest xray done