infections in immunocompromised

Question 1

identify 3 immunocompromised infections?

Answer 1

Pneumocystis jirovecii
Cryptococcus neoformans
Fungal infections other than tinea and thrush

Question 2

Absence of neutrophils (

Answer 2

Kostmann’s, congenital neutropenia)

Question 3

immunocompromised often infected with?

Answer 3

Characterized by recurrent bacterial infections and Aspergillus infection

Question 4

defects in phagocyte function:

often infected with 3 bugs here:

Answer 4

Characterized by recurrent bacterial infections (especially S. aureus and other catalase positives), Aspergillus, and Candida sp. Infections

Question 5

missing C5-C9 what are you going to be infected with?

Answer 5

neisseria

Question 6

x-linked agammaglobulinemia- due to defect in what?

Answer 6

X-linked agammaglobulinemia – due to defect in Bruton tyrosine kinase

Question 7

when does CVID present?

Answer 7

Common variable immunodeficiency – presents age 2 to 20, typically early teens, due to various genetic defects; may have autoimmune disorders

Question 8

what is the most common immunodeficiency?

Answer 8

IgA deficiency – most common immunodeficiency

Question 9

all manifesations of:

Clinical manifestations
Recurrent bacterial (especially encapsulated organisms like S. pneumoniae, H. influenzae) and viral infections
Recurrent sinopulmonary infections or otitis
Giardiasis
Some may be associated with autoimmune disorders or increased risk of malignancy

Answer 9

immunoglobulin deficiencies

Question 10

what is defect in SCID?

how do you tx?

Answer 10

absence of T cells; impacts B cell function and B cells may also have functional deficiencies
Treated with stem cell transplantation

Question 11

what is DiGeorge?

Answer 11

Congenital thymic hypoplasia, heart disease, hypoparathyroidism, neonatal hypocalcemia
May develop autoimmune disorders in later life

Question 12

what is the disorder in Wiscott-Aldridge syndrome?

TIE

Answer 12

X-linked: in WAS gene on X-chromosome–>T cells unable to reorganize actin cytoskeleton

Thrombocytopenia, severe eczema
Treated with stem cell transplantation
High risk to develop malignancies (especially lymphoma)

Question 13

phagocyte deficiencies: which organisms

you need PLACESS for you cats.

Answer 13

Catalase positive bacteria and fungi

S. aureus, Nocardia species, P. aeruginosa, Serratia species, enteric gram-negative bacilli, Candida species, Aspergillus species

Question 14

complement deficiencies:

which organsism?

Answer 14

Neisseria infections

S. pneumoniae, H. influenzae, Neisseria species

Question 15

Antibody deficiencies
 (B cell) which organsisms? 

SHiN

SKiS

Answer 15

Organisms with polysaccharide capsules

Streptococcus pneumoniae, Haemophilus influenzae, Staphylococcus aureus, Pseudomonas aeruginosa, Mycoplasma species, Salmonella species, Shigella species, Campylobacter species, rotavirus, enteroviruses, Giardia species

Question 16

which organisms commonly infect?

Cellular and combined immuno-deficiencies

Answer 16

Opportunistic infections, fungi, viruses

Question 17

what are 3 aquired immunodeficiencies?

Answer 17

Chemotherapy
Bone marrow and solid organ transplant
Immunosuppressive therapies

Question 18

Splenectomy and Sickle Cell Disease

increased risk for which two organsims?

Answer 18

Increased risk for sepsis with encapsulated bacteria, especially S. pneumoniae and H. influenzae type b

Antibiotic prophylaxis and empiric therapy indicated

Question 19

3 diseases of pregnancy?

Answer 19

UTIs
Hepatitis E: 15 -25% mortality
H1N1 influenza associated with increased morbidity and mortality

Question 20

chemotherapy-induced neutropenia/immunodeficiency

what are the clinical manifestations? (4 bugs here)

Answer 20

clinical manifesations

Bacterial and candidal sepsis
Aspergillosis
Pneumocystic pneumonia
Disseminated varicella

Question 21

3 ways to manage chemotherapy induced neutropenia/immunodeficiency?

Answer 21

Prophylaxis – bacterial and fungal, based on risk assessment for type of underlying malignancy

Aggressive use of empiric antimicrobial therapy at first sign of infection

G-CSF

Question 22

which procedure brings these about?

Risk of neutropenia plus profound lymphocytic dysfunction plus, in some patients, graft-versus-host
Pattern of infection varies with the length of time from transplant

Answer 22

Bone Marrow and Solid Organ Transplantation

Question 23

child w/albinism +

a phagocyte functional disorder characterized by large leukocyte granules

Answer 23

Chediak-Higashi syndrome

Question 24

what type of disorder is chronic mucocutaneous candidiasis?

Answer 24

T-cell disorder

Question 25

in myeloperoxidase deficiency, which bug commonly afflicts patient?

Answer 25

candida

Question 26

in complement deficiency, what do ppl with C1-C4 suffer from?

Answer 26

recurrent bacterial infections, autoimmune disorders

Question 27

how do you manage complement deficiency pts?

Answer 27

immunizations + prohylactic antibiotics if needed

Question 28

immunoglobulin deficiencies: 1. which infections? 2. are they at increased risk for cancer/ autoimmune disorder? 3. how do you dx? how do you manage?

Answer 28

1. recurrent bacerial infection ( S. pneumo + H. influenza) + viral infections , recurrent sinopulmonary infections or otitis, giradiasis 2. yes, at increased risk for autoimmune disorders or increased risk for malignancy 3. dx: quantitaive immunoglobulin measurement 4. IgG replacement if deficient (do NOT use in IgA defiinecy), give prophylacit Abx.

Question 29

what is SCID and how do you tx?

Answer 29

SCID = absece of T cells; impacts B cell function and B cells may also have functional deficiencies tx: stem cell transplantation

Question 30

what is DiGeorge? what may these kids develop later in life?

Answer 30

-congenital thymic hypoplasia, heart disease, hypoparathyroidism, neonatal hypocalcemia may develop autoimmune disorders later in life

Question 31

what do kids with wiscott-aldridge syndrome present with? how do you tx? what are they at risk of developing?

Answer 31

1. TIE: thrombocytopenia, eczema, infections 2. tx w/stem cell transplantation 3. high risk to develop malingancies (esp. lymphoma)

Question 32

what are kids with lymphocytic disorders at risk for? (3)

Answer 32

1. opportunistic infections (pneumocystis pneumonia; fungal infections, protozoa; disseminated viral) 2. recurrent bacterial infection: 3. increased risk for malignancy

Question 33

KNOW: kid with phagocyte def at risk for??

Answer 33

catalase + and bacteria and fungi

Question 34

kids with complement deficiencies at risk for?

Answer 34

neiserria infections

Question 35

kids with antibody deficiencies (B cell)

Answer 35

organisms with polysaccharide capsule

Question 36

cellular and combined immuno deficiencies at risk for?

Answer 36

opportunistic infections, fungi, viral infections

Question 37

what are the five indications for immmune evaluation?

Answer 37

1. opportunistic infection 2. recurrent infection other than URIs in the young child at a day care center 3. failure to thrive 4. persistent infection despite therapy 5. hx of recurrent infections w/a defined cause is more meaningful than a hx of frequent, self-limiting infections of presumed viral cause

Question 38

what are the three evaluations for possible immunodef: 1

Answer 38

1. history 2. CBC, differential and platelet count (neutropenia and thrombocytopenia) 3. Hgb electrophoresis (sickle cell disease), HIV, serology/PCR, sweat test (cystic fibrosis) 4. addl testing based on prelim ddx

Question 39

what is basic work up of possible immunodef

Answer 39

genomic analysis (for 120+ genes\_

Question 40

2 bugs with big impact on ppl w/splenectomy and sickle cell disease

Answer 40

sespis w/encapuslaed bacteria esp: s. pneumo and H. influenza type B antibiotic prophylaxis and empiric therapy indicated

Question 41

3 infections with pregnancys

Answer 41

UTIs hepatitis E: 15-25% mortality H1N1 influenza associated with increased morbidity and mortality

Question 42

what is the risk of infection proprotional to in ppl w/chemo induced neutropenia?

Answer 42

proportional to degree and duration of neutropenia

Question 43

t/f. in ppl wht chemo induced neutropenia, lymphocytic dysfunction is present and may also manifest as infection

Answer 43

true

Question 44

what are 4 bugs that affect ppl with chemo induced neutropenia/immunodef

Answer 44

bacteria and candidal sespis aspergillosis pneuomycsitc pneumonia disseminated varicella