Immune Flashcards

(52 cards)

1
Q

Sever combined immunodeficiency disorder (SCID)

primary or secondary?

How is it passed on?

A

Severe deficiency of T & B cells ; so immune response is inhibited bc no memory

Primary due to being born with it

Autosomal & sex linked

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2
Q

Sever combined immunodeficiency disorder prognosis without tx

A

Will probably die from frequent infections if not treated

- can get treated and have another immediately bc of no T & B cells

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3
Q

Treatment for Sever combined immunodeficiency disorder focus?

Two medicines to do this?

the most invasive form of treatment?

A

Lower risk to infections

prophylactic antibiotics
IVIG or pooling of immunoglobulins

stem cells transplant for leukocyte (T &B cells) production

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4
Q

IVIG concerns & preparation with Sever combined immunodeficiency disorder

How fast should infusion be?

A

Could be receiving a large amount & risk factor for anaphylactic response so give

  • Tylenol or Nsaid
  • histamine
  • steroids

Start infusion very slow
- .01 mL/kg/min
- monitor it and in about 30 min bump it up .02-.05 until you are at .08
(if they’ve done the infusion before, you’ll probably know how to react)

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5
Q

Body system to be aware of with IVIG for patient with Sever combined immunodeficiency disorder ?

What other forms of IMG are there?

A
  • be aware of fluid volume & kidney function

SubC and IM which is more concentrated

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6
Q

Best match for stem cell transfusion? What else?

A

A sibling
- bc has genes from parents too

Can get from parents

Unrelated donors may have a match too for bone marrow registry

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7
Q

How will the stem cell transplant go in Sever combined immunodeficiency disorder patient?

Is it always successful?

A

Wipe out abnormal bone marrow and then introduce new stem cells

NO, there can be rejections.

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8
Q

T/F

You can give only give irritated blood products and non-activated vaccines for those with immunodeficiency

A

true

- no MMR, polio etc

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9
Q

HIV is a retrovirus. what does this mean

A

transmitted through body secretions

- blood, semen, vagina, breast milk

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10
Q

HIV pathophysiology

A

T-cells called CD4 lymphocytes are affected and so the immune response can’t be launched

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11
Q

Is HIV a death sentence?

A

Nope it is more of a chronic illness now for adults and children

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12
Q

Diagnosis of HIV

A

About 15 days after exposure

But will want to follow up 30 days later on.

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13
Q

Most recommended testing of HIV

A

PCR and proviral DNA
- type of testing done will be based on likelihood of infection

  • needle stick test of two negative patients won’t be using a high sensitivity test
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14
Q

HIV stats of kids in US

nationally?

A

Dramatic decline, so less than 150-200 in US
Especially in maternal fetus passage.
- we have education, screening, and treatment

Way bigger international problem bc more adults have HIV

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15
Q

What will symptoms be like of HIV if on meds?

A

Symptoms related to medication like diarrhea

  • need dosage adjustment to fix
  • make sure to double check symptoms are even related to the meds or if there’s another issue going on
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16
Q

Lymph, liver, spleen symptoms of HIV

A

enlarges and swells

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17
Q

Mouth symptoms related to HIV

A

Oral candidiasis shown as white patches

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18
Q

Gi symptoms related to HIV

A

Diarrhea

  • could be from meds or the disease
  • know the right diet ; low bacteria for prevention of infection of issues
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19
Q

treatment meds for HIV

How long will they need this?

A

antiretroviral meds
- suppresses replication of HIV so the CD4 t cells aren’t affected

  • will be lifelong bc no cure
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20
Q

how do they protect HIV patients from pneumocytisis pneumonia?
what other meds do HIV kids need

A

Prophylactic antibiotics

immunoglobulin

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21
Q

What is the concern nationally with HIV treatment?

A

We have the meds to manage HIV but poverty governments may not have resources

22
Q

Nursing goals with HIV

A

1) protect them from infection and support their nutrition
- do not give them live viruses

2) take care of symptoms
3) monitor their growth

23
Q

Galactosemia

A

Not enough of galactose 1 phosphate enzyme so the “galactose” cannot be converted to glucose
- malnutrition and no energy causing failure to thrive

24
Q

When is Galactosemia tested for?

A

Newborn screening so we can catch it early on

25
Can Galactosemia pateints drink diary?
Nope - no milk or breast milk. anything with lactose - replace with specialty formula. - educate on solid foods to for when kid gets older
26
How often are blood levels drawn for galactosemia
serial to check for Galactosemia
27
What needs to be monitored in Galactosemia patients
liver function and bleeding
28
What are Galactosemia patients prone to
E. coli sepsis
29
Longterm Galactosemia disease consequences
cognitive delays cataracts at a young age female ovarian dysfunction
30
why is education is important with Galactosemia
bc follow up and diet are important
31
Phenylketonuria (PKU)
Inability to metabolize phenylalanine so it builds up and causes brain damage due to not being able to excrete it as an amino acid
32
What do Phenylketonuria or PHU kids look like
Blond hair, blue eyes, fair skin with dermatologic problems | - eczyma or ceriasis
33
What status are most Phenylketonuria or PKU kids
failure to thrive
34
Urine smell of Phenylketonuria
musty
35
Behavior of PKU or Phenylketonuria kids
irritable hyperactive mental retardation Uncontrolled activities in older kids
36
Diagnosis of Phenylketonuria or PKU
Newborn screening and done in every state with Guthrie blood test
37
What must be restricted for Phenylketonuria or PKU patients How much is ok?
Restrict phenylalanine foods bc it can't be eliminated as an essential amnio acid - but we need to make sure they're still getting adequate nutrition 2-6, 2-10 levels are acceptable
38
Phenylketonuria formula?
Must be careful =- make it into paste and then add fluid
39
long term effects of PKU
lower IQ Decreased bone density Behavior probelms
40
Trisomy 21 is what
Down syndrome
41
Main cause of trisomy 21? | Other causes?
Extra 21 chromosome Translocation of chromosome 15 Mosacism mix from different cell type
42
Diagnosis triggers that lead to testing of down syndrome
Facial features are big indicator like wide flat nasal bridge and ears are below eyes and no muscle, short neck, brush fed spots on eyes, dry skin - poor eating can happen Defects like ASD
43
What are Trisomy patients prone to IQ? muscle? endocrine
infections URI/otitis IQ can be altered hypotonicity of chest and abdomen hypothyroid
44
t/f | everyone with down syndrome has cardiac defect trisomy 21
false
45
t/f | increased risk of hypothyroid states trisomy 21
true
46
t/f | decrease risk of AML or acute myelnoid leukemia in trisomy 21
false - increased risk
47
what does the mouth do in trisomy 21
open mouth with tongue that is too large for mouth high narrow pallate ears small in size small genitalia stimian crease short stature; can lead to obesity
48
how to manage infections of down syndrome
immunizations up to date
49
trisomy 21 feeding concers
mouth and tongue issue can make feeding hard exhaustion is easy too
50
Constipation risk for trisomy 21 explained
decreased activity
51
why is early dx of down syndrome important
access to therapists and therapy
52
planning for down sydrome
who is gonna take care of the child as they grown into an adult