Exam 2 Flashcards
first line of defense cells in the immune system
Neutrophils
basophils
eosinophils
natural killer cells
dendritic cells
monocytes
macrophages
special proteins that are included in first line of defense
Complement proteins
Cytokines
anti microbial
foreign (to our body) proteins on pathogens are called
antigens
dendritic cells internalize the pathogens by
phagocytosis
which cell internalizes the pathogen by phagocytosis then displays the antigens on its surface
Dendritic cell
After the dendritic cells display antigens on its surface where are the dendritic cells carried
lymph nodes on spleen
After the dendritic cells display antigens on its surface, they are carried to the lymph nodes on the spleen where more specialized cells are made which are a part of the second line of defense
lymphocytes
what are the 2 main types of lymphocytes
B cells and T cells
B cells produce what
antibodies - proteins specifically directed at antigens on pathogens
Antibodies that are produced by B cells go where? what happens
they bind to the pathogens and then the macrophages clean these complexes up by phagocytosis
2 kinds of T cells
Helper cells
Cytotoxic cells
Helper T cells interact with ___ cells to get them to produce more ___
B cells
Antibodies
what does cytotoxic T cells do
they find the cells that pathogens are hiding in and kill these cells
what cells are made once an infection is cleared
Memory B cells
Memory T cells
JIA is arthritis of how many joints for at least ___ weeks in a child of what age?
1 or more joints for at least 6 weeks in a child younger than 16 yrs old
JIA in 4 or fewer joints
oligoarticular JIA
JIA in 5 or more joints
Polyarticular JIA
high spiking fevers of at least 2 weeks plus arthritis
what is systemic onset JIA
JIA with positive ANA increases risk of
iritis and uveitis
symptoms of systemic JIA
fatigue
anemia
fever
salmon-colored macular rash
hepatosplenomegaly
lymphadenopathy
ESR and CRP is typically normal in what type of JIA
Oligo
how often are optho eye screenings done in Systemic JIA vs Oligo or Poly JIA
Systemic JIA - every 12 mos
Oligo or poly JIA - every 6 mos
Oligo or Poly JIA if <7 yrs and Positive ANA - every 3-4 mos
Psoriatic arthritis symptoms
Arthritis in small and large joints
psoriatic rash
Dactylitis
Nail pitting
if no rash
-family h/o psoriasis in first degree relative
in psoriatic arthritis there is a risk of uveitis which requires
slit lamp exam every 6 months
psoriatic arthritis may have what positive markers
ANA
HLA B27
Enthesitis-related arthritis usually occurs in what age group
> 8 yrs
symptoms of Enthesitis-related arthritis
pain, stiffness and loss of mobility of the lower back
can present with arthritis in lower extremity joints
Positive HLA-B27
malar rash
photosensitivity (rash in reaction to sunlight)
oral ulcers
arthritis
serositis
Systemic Lupus erythematosus
Labs for SLE
CBC - potential cytopenias
CMP - Liver and renal function
ANA - positive ANA
Anti-dsDNA - at time of dx and to monitor activity
C3 and C4 low or undetectable levels during disease activity
Anti-Smith antibody - highly specific for SLE
what labs are used to monitor disease acitvity. SlE
Anti-dsDNA
C3 and C4
transplacental passage of maternal SSA or SSB antibodies
Neonatal lupus erythematosus
symptoms of Neonatal lupus erythematosus
fetal bradycardia
Congenital heart block in 30% (30-50% willl need pacemaker)
Rash will resolve 6 months later but heart block stays
Meds used in SLE
Hydroxychloroquine
Corticosteroids
Cyclophosphamide
other immunosuppressive
agents
NSAIDS
Calcium
Vit D
inflammation of sacroiliac joints (SI) and the axial skeleton
Ankylosing spondylitis (AS) and spondyloarthropathy
Low back pain characteristics for
Ankylosing spondylitis (AS) and spondyloarthropathy
worse in morning and improves with exercise
labs in Ankylosing spondylitis (AS) and spondyloarthropathy
High ESR and CRP
Positive HLA B27
Bamboo sign
This is Bridging syndesmophytes
characteristic of
Ankylosing spondylitis (AS) and spondyloarthropathy
recurrent parotitis
think sjogren syndrome
management of sjogren syndrome
artificial tears
pilocarpine tablets
antimalarial for skin rash and arthritis
sjogren syndrome has a risk of
lymphoma
Blood transfusions started in what years and why
maternal hemorrhage
1600s
A type lab tells you what
ABO and RH
Forward and reverse typing
what does this mean?
testing the antigens on the Red cell and they are testing what AB are in the plasma
Surface markers on blood typing is the presence or absence of what?
2 antigens (A and B) on RBC
Isohemaglutinins on blood typing is the presence or absence of what?
antibodies in the serum directed against missing A and B
Antibodies develop by what age so what does this mean if you are doing a type and screen in a baby <4 months
12 months of age
only need one type and screen test bc they only create one AB. immune system is still underdeveloped
The screen part of a type and screen is also known as
what does it do?
Indirect antiglobulin test or an “indirect coombs”
detects AB in the serum
Agglutination in the screen part of the type in screen is a ____ test
positive - must do further testing to evaluate for presence of AB and then identify the AB
how does the screen part of the type and screen work? (aka indirect antiglobulin test or indirect coombs)
you have antibodies in serum
They use a reagent
They are looking to see if the reagent causes the RBCs to clump
If It is positive then a Direct Antiglobulin Test (DAT) AKA Coombs is done
Direct Antiglobulin Test (DAT) is also known as
Coombs
Coombs is also known as
Direct Antiglobulin Test (DAT)
Patients can develop antibodies from
Pregnancy
Transfusion
Transplantation
Repeat type and screen every 72 hours is because
They need to re-screen the patient. This is to see if because they have been exposed to transfusion, have they now developed antibody
what is Allo vs Auto Antibody source
Allo - AB formed in response to
pregnancy
Transfusion
Transplantation
These antibodies are targeted against a antigen that is not present on the patients RBC
Auto
AB formed in response to patients own RBC
what type of Antibody source?
AB formed in response to
pregnancy
Transfusion
Transplantation
These antibodies are targeted against a antigen that is not present on the patients RBC
Allo
what type of antibody source?
AB formed in response to patients own RBC
Auto
How does AB formed after transfusion work
A pt is transfused. donor RBC can have antigens. You form antibodies against these antigens. Next time your transfused, you can develop hemolysis.
If the pt has developed antibodies against antigens from previous blood products we give them what
antigen negative blood product
(specifically Kell neg but can be other antigens)
Antigen vs Antibody
Where are these found
Antigen is on the RBC
Antibody are in the plasma
what is done to prevent bacterial contamination during blood product collection
the first 30 ml is separated into an alloquat that would contain the skin plug from puncture to help prevent bacterial contamination also for donor infectious disease contamination
Product is received to blood bank after testing usually by day
day 3
blood donors tested for Covid, why?
so they can use their plasma in very sick covid pt in hopes that they get their antibodies to help fight the infection
All blood products are filtered through ____ _____ filters
white cell - larger than red cells so these filters capture large amount of white cells (Leukoreduction)
term for
white cell - larger than red cells so these filters capture large amount of white cells
Leukoreduced
Leukoreduction helps to prevent
transfusion associated graft vs host disease
febrile non-hemolytic transfusion reactions (FNHTR)
transmission of disease - viruses live in white cells. The more you can decrease number of white cells, the more you decrease the risk such as CMV
HLA Alloimmunization -
when would leukoreduction not occur
in autologous (banking for self)
types of crossmatching
Serologic (Tube testing)- recipient plasma an donor RBC is spun to see if there is clumping = ABO incompatibility
Electronic - 2 confirmed ABO/Rh at TCH + no antibody history + negative current antibody screen
when is Serologic crossmatching used (tube testing)
if recipient is AB positive, otherwise electronic crossmatching is used
why is irradiation used
what circumstances?
Leukoreduction does not get rid of all white cells. Irradiation is used so remaining white cells cant replicate (leads to transfusion associated graft vs host disease - extremely rare but fatal)
Immunodeficient - don’t have the immune system to keep donor white cells at bay
-Transplant (pre and post bone marrow, solid organ)
-Immunocompromised (Malignancy, iatrogenic or acquired)
-Immunodeficiency (suspected/confirmed primary cellular or combined)
-Age consideration (Fetus, infant <3 months old)
-Other (Granulocytes, HLA matched platelets, directed donation from blood relatives)
_____% of donor population is CMV negative
10%
2 examples guest speaker gave for when they would use CMV negative blood product because it is so rare
SCID (severe combined immunodeficiency)
DiGeorge
why is the risk for CMV negative and CMV safe blood product nearly the same
due to the window period for CMV. You could have been exposed recent but not testing positive for it yet.
what is volume reduction for blood product
for platelets
plasma removed.
in fluid overload concerns and anaphylaxis
impacts quality of platelet product significantly
Adds 2 hrs to processing
Blood product washing
RBC and platelets
done for IgA deficiency and severe anaphylaxis
impacts quality of platelet product significantly
Adds 2 hrs to processing
word for partial unit of blood product
aliquot
1 apheresis unit for platelets is approx ___ mL
250
1 unit per “bag” of RBC is approx ____ -___ mL
250-400mL
1 unit increases hgb approx
1-2 g/dL
____ is the preservative now used in RBCs which means what
Adsol -
42 day storage
<50mL plasma
HCT 55-60%
less citrate
Platelets have a ___ day shelf life
5
Platelets increase count by
30k-60k
Jumbo unit of plasma is equal to how many regular
4-5
It takes about ____ minutes to thaw FFP
45
Plasma contains all of the _____ cascade
coagulation
Cryo contains factors
VIII
XIII
Fibrinogen
good fibrinogen level is >
200
most important in clotting
Fibrinogen - spiderweb for RBCs to attach to in order to clot
Concentrated amount of fibrinogen is found in what product
Cryo
most transfusion reactions occur in the first
15 min
however can start 4 hours later
what temp increase do you need to see to suspect a transfusion reaction and stop the transfusion
1 degree C or 2 degree F from pre-transfusion
differentials on temp increase for transfusion reactions
Acute hemolytic Transfusion Reaction (AHTR)
Bacterial contamination - maybe they are sick with another bacteria and just happen to spike
underlying condition
Febrile non-hemolytic transfusion Reaction (FNHTR)
Post transfusion purpura and transfusion associated graft versus host disease - rare but fatal
clinical presentation of Acute hemolytic Transfusion Reaction (AHTR)
-fever and chills (most common)
-Pain (back or infusion site)
-hypotension/shock
-hemoglobinuria
-impending doom
Work up for Acute hemolytic Transfusion Reaction (AHTR)
Positive DAT
Hemoglobinemia/uria
Elevated indirect and direct bilirubin
DIC findings (clot clot clot, bleed, bleed, bleed) -> plasma exchange these pt
RBC abnormalities
management for Acute hemolytic Transfusion Reaction (AHTR)
Stop the transfusion
hydration and diuresis
Plasma exchange?
Transfusion medicine
Prevention!
Febrile non-hemolytic transfusion Reaction (FNHTR) symptoms
This is a diagnosis of exclusion!
Fever (temp increase of about 2 degree F)
chills, shakes, rarely rigors
Mild dyspnea or tachypnea
usually within 4 hours of transfusion and resolves within 2 hours
Benign …uncomfortable but no long lasting deficits
Febrile non-hemolytic transfusion Reaction (FNHTR) usually occurs within ____ hours of transfusion and usually resolves within ___ hours
4
2
Febrile non-hemolytic transfusion Reaction (FNHTR) is from the ___ cells from a ____ release
White cells
cytokine release
Resp distress from transfusion reaction occurs ___ - ____ hours after transfusion
6-12
S/S of resp distress in transfusion reaction
Tachypnea
decreased oxygen saturation
What can cause respiratory distress in transfusion reactions
Anaphylaxis
TRALI (immune response)
TACO (volume intolerance)
TRALI is a _____ response
immune
TACO is a _____ intolerance
volume
difference in chest Xray in TRALI vs TACO
identical
What do you need order in resp distress after Transfusion reaction
BNP
Chest x ray
tell me about TACO
increased hydrostatic pressure (Pmv) from too fast of rate, too much volume or pt has congestive heart issues, renal issues or started the transfusion fluid overload.
causes leak of protein poor edema
diuresis and they usually do great
Tell me about TRALI
immunologic issue
Neutrophils live in lungs to keep them healthy. Occasionally a donor will form neutrophil antibody and when recipient has matching neutrophil antigen. Immune response -> capillary leak syndrome
This causes protein rich edema fluid to leak inside alvoli
they may respond to diuresis but will continue to be sick for the next 24-72 hours
Treat with resp support: may need to be intubated
TRALI vs ARDS
TRALI gets better in 2-3 days. ARDS takes much longer
TRALI type I vs type II
in type one the pt had no resp symptoms at all. In type II, they had some resp symptoms before the transfusion and now much worse
Allergic reaction to transfusion
stepwise approach to premedication
Stepwise approach
Premedicate
1. Benadryl (H1)
2. Benadryl (H1) + Hydrocortisone
3. Benadryl (H1) + Pepcid (H2) + Hydrocortisone and Zyrtec qhs
General transfusion threshold is Hgb of ___
7
Transfusion ___-___mL/kg
10-15mL /kg and only 1 unit at a time
Our platelet threshold is
20,000 but they are working on adjusting down to 10,000 in non-bleeding patients
TCH is moving to a massive transfusion protocol Plasma:RBC ratio
1:1
remember, anemia is a ___ diagnosis
secondary….its a symptom
s/s for anemia
pallor
DOE dyspnea on exertion)
SOB
Fatigue
Bounding pulses
pallor
Tachycardia
Severe
dizziness
palpitations
chest pain
CHF symptoms
hypotension
lethargy
confusion
arrhythmia
CHF signs
anemia definition
reduced RBC measures (Hgb, HCT, RBC count) - normal depends on age, race, gender
reasons for anemia
Bleeding
diet related (are they strict vegan and not getting any B12)
Family history of bleeding disorders?
Bone marrow suppression
Meds - NSAIDs, ASA, Bactrim or other antimicrobials
cells responsible for transportation of O2/CO2 around the body
RBCs
iron containing protein with the RBC that carries O2
hemoglobin
the percentage of total blood volume occupied by RBCs
Hematocrit
the average volume of a single RBC
Mean Corpuscular Volume (MCV)
a measurement of the variability of red cell side
Red Cell distribution width (RDW)
Higher Red cell distribution width (RDW) indicates
Greater variation in size
small cells that assist with clotting
platelets
blood cells that make up the immune system
WBC
Onc labs
CBC with diff
Chem
Tumor Lysis labs (LDH, uric acid, phos level)
Blood cultures
DIC panel
Consider CRP, ESR
Infectious workup as indicated (UA, UC, resp panel, viral titers, Quantiferon if concerned for TB)
Tumor lysis labs
LDH
uric acid
phos level
most common and second most common childhood cancer
ALL
AML
AML and ALL are caused by ____ mutations
sporadic not hereditary
other siblings and future children are not at higher risk and do not need to be tested
Very elevated WBC, what level and what are you concerned for
> 191
high risk for
tumor lysis syndrome
vaso-occlusive crisis
T cell ALL and Lymphoma can have a ___ on x ray
Mediastinal mass on chest x ray
electrolyte disturbances associated with tumor lysis syndrome
Hyperkalemia
Hyperphosphatemia
hypocalcemia
hyperuricemia
destruction may be due to a spontaneous or treatment related causes, resulting in electrolyte and metabolic imbalances
(oncology)
tumor lysis syndrome
onc emergency associated with cytotoxic chemotherapy, including corticosteroids
Tumor lysis syndrome
what organs are highest concern in tumor lysis syndrome
Kidneys: uric acid deposits in the kidney and causes injury
Liver: Uric acid deposits and can cause liver injury
Heart: elevated K+ can cause arrhythmia with v fib or v tach
what medication is used to help keep uric acid from accumulating in tumor lysis syndrome
Allopurinol
Gold standard for diagnosis for leukemia
Bone marrow biopsy
time line for bone marrow biopsy in new onc diagnosis
within 72 hours
To diagnose leukemia you need > ___% blasts in bone marrow
20%
in flow cytometry
Lower CD numbers (CD4, CD8) are typically ____ cells
T cells
in flow cytometry
Higher CD numbers (CD19, CD20) are typically ____ cells
B cells
ALL induction treatment
Day 1: Lumbar puncture to look for CNS involvement
Intrathecal cytarabine: Day 1
Vincristine: Day 1, 8, 15, 22
Dexamethasone: Days 1-28
Pegasparagase: Day 4
Intrathecal Methotrexate: Day 8 and 29
Bone marrow biopsy is done again on day 29 to look for Minimal Residual Disease (MRD)
After ALL induction there is a bone marrow biopsy to look for Minimal Residual Disease (MRD) -what is the hope?
Hope is the blast cells are <0.01% (down from >20% on initial bone marrow biopsy)
If > 0.01%, they are classified under high risk and will need more intensive chemotherapy moving forward
Tumor lysis syndrome treatment
Stabilize cardiac membrane if evidence of heart involvement - Calcium Gluconate, lasix, Kayexalate, Insulin, albuterol
Elevated uric acid: Allopurinol (TID) or Rasburicase (once daily)
Hyperphosphatemia: AlternaGel (binds to phosphorous) q 4 hours
Onc patients who present with fever:
What antibiotic choices for both
If immunocompromised and
not immunocompromised
not immunocompromised
Ceftriaxone - empiric
Immunocompromised
Vanc and Cefepime for empiric
fever threshold for oncology pt
100.4
Rasburicase helps lower uric acid by helping with ____
excretion of uric acid by turning uric acid into Allantoin
Immature blasts are sticky and can cause similar issues seen in patients with sickle cell such as
-priapism, stroke, kidney inury
Vaso-occlusive issues
what intervention can worsen or induce vaso occlusive crisis in a Leukemia pt
RBC transfusion
criteria that makes you high risk in leukemia
Initial WBC at presentation >50k
Age <1 or >10 yrs
Testicular involvement (usually unilateral, large and firm)
CNS involvement
Also high risk if
-unfavorable genetics (such as Philadelphia chromosome
-Not under remission at end of induction (day 29)
most common cause of anemia
blood loss
Nutrient deficiencies leading to anemia
Iron
B12
Folate
leads to decreased effective RBC production
Bone marrow disorders that lead to decreased effective RBC production causing anemia
SAA (severe aplastic anemia)
pure RBC aplasia
Marrow infiltration
name 3 things that cause bone marrow suppression leading to decreased effective RBC production causing anemia
Drugs
chemotherapy
irradiation
what can cause decreased bone marrow stimulation leading to decreased effective RBC production causing anemia
Decreased EPO
decreased T4
Decreased androgens
Name 3 things that can lead to anemia of chronic disease leading to decreased effective RBC production causing anemia
Infection
inflammation
malignancy
What 3 things can cause ineffective erythropoiesis leading to decreased effective RBC production causing anemia
Alpha/beta thalassemia
Sideroblastic anemias
MDS (myelodysplastic syndromes)
what can cause intravascular hemolysis that causes increased RBC destruction/hemolysis which leads to anemia
Microangiopathic processes (TTP, HUS, aortic stenosis)
ABO incompatibility
infection
PNH (Paroxysmal nocturnal hemoglobinuria)
Venom
CU poisoning in wilson disease
Where does extravascular hemolysis occur that causes increased RBC destruction/hemolysis which leads to anemia
liver
spleen
what causes intrinsic hemolysis that causes increased RBC destruction/hemolysis which leads to anemia
enzyme deficiencies
hemoglobinopathies
membrane defects
What causes extrinsic hemolysis that that causes increased RBC destruction/hemolysis which leads to anemia
AIHA (autoimmune hemolytic anemia)
IVIG infusions
liver disease
infections
toxins
Low MCV
Normal MCV
High MCV
in anemia classification
Microcytic
Normocytic
Macrocytic
MCV in Microcytic anemia
<80
MCV in Normocytic anemia
80-96
MCV in Macrocytic anemia
> 100
Anemia differential?
MCV <80
Low Fe
High TIBC
Low ferritin
Iron deficiency anemia -> need to determine cause
Anemia differential?
MCV <80
Low Fe
Normal or low TIBC
Normal or high ferritin
Anemia of chronic disease:
infection
inflammation
malignancy
Anemia differential?
MCV <80
Normal to high Fe
Any TIBC
Normal to high ferritin
Iron overload present
Siderocytes on peripheral smear
Sideroblasts on bone marrow
Sideroblastic anemia: need to determine cause
Anemia differential?
MCV <80
Normal to high Fe
Any TIBC
Normal to high ferritin
Teardrop red cells
Target cells
Splenomegaly
Positive family history
Alpha or Beta thalassemia: Perform hemoglobin electrophoresis
anemia differential? with MCV 80-96
no blood loss
increased LDH
Increased indirect bilirubin
decreased haptoglobin
presence of hemolysis - determine why
anemia differential? with MCV 80-96
low reticulocyte response
bone marrow suppression…determine why?
anemia differential? with MCV 80-96
no blood loss
elevated creatinine
renal insufficiency
anemia differential?
MCV >100
dysplastic features present
Cytopenias present
Myelodysplastic disorder
anemia differential?
MCV >100
low B12
elevated methylmalonate
elevated homocysteine
B12 deficiency: determine cause
anemia differential?
MCV >100
low folate
normal methylmalonate
elevated homocysteine
folate deficiency:
determine cause
anemia differential?
MCV >100
increased reticulocytes
liver disease
hypothyroidism
drugs
lab test?
uses venous blood to measure iron molecules that are bound to transferrin
serum iron
lab test?
the sum of all iron binding sites on transferrin
-serves as an indirect measurement of transferrin
Total Iron Binding Capacity (TIBC)
TIBC serves as an indirect measurement of
trasferrin
Lab test?
extent to which sites on transferrin are filled by iron ions
Transferrin Saturation % Sat
Lab test?
an intracellular protein that stores iron; correlates with total iron body stores. An acute phase reactant
Ferritin
most common cause of blood loss in men and women
men: GI bleeding
women:Menstrual bleeding
during Menstruation women lose average of about ___mg of iron /day
1 mg of iron/day
causes of iron deficiency
blood loss
diet low in iron
malabsorption of iron
name 3 problems that can cause malabsorption of iron
Celiac disease
H. pylori
autoimmune gastritis
Treatment for iron deficiency
stabilize pt
stop blood loss
Replace with supplements PO/IV
-Ferrous salts: Ferrous sulfate( drug of choice), fumarate, gluconate, ect
what ferrous salt is drug of choice for iron replacement
Ferrous sulfate
When is iron best absorbed
before meals but best tolerated after meals
Ferrous sulfate is best absorbed when taken with
OJ or ascorbic acid
Take 2 hrs before or 4 hours after antacids
Don’t take with calcium
Don’t take ferrous sulfate with
calcium
Take ferrous sulfate either ___ hrs before or __ hrs after antacids
2 before
4 after
Side effects of ferrous sulfate
metallic taste
n/v
flatulence
constipation
diarrhea
epigastric pain
black/green stools
Length of therapy for ferrous sulfate in iron def treatment
3 months to build up hgb/hct, another >=3 months to build up stores. Chronic therapy for chronic bleeding
Acute iron poisoning causes
GI necrosis
CNS depression
Shock
Emergency treatment for iron tox
Deferoxamine chelation
any type of chronic disease >1-2 months duration can cause ___ due to inflammatory, infectious, malignancy
anemia
low serum iron
low or normal transferrin/TIBC
high or low % saturation
High or normal ferritin
Anemia of chronic disease
Low serum iron
high transferrin/TIBC
low % sat
Low ferritin
Iron deficiency anemia
appetite for substances not fit for food
Pica
Pica for ice
Pagophagia - can be seen in iron deficiency state
symptoms for microcytic anemia
Pica
glossal pain
dry mouth
tongue papillary atrophy
Atrophic glossitis
Koilonychias (spoon nails)
Diagnosis for Sideroblastic anemia
ringed sideroblasts on prussian blue reaction of BMA
increased serum iron
increased ferritin
Common causes for Sideroblastic anemia
Drug induced
lead poisoning
chronic alcoholism
Treatment for sideroblastic anemia
Treat cause
Remove offending agent
Most common causes of macrocytic anemia
B12 and folate deficiency
Macrocytic anemia is a _____ _____ disorder
DNA replication disorder - cells are arrested at an immature stage and do not divide correctly leading to larger cells
classes of drugs that can cause macrocytic anemia
antiviral drugs
chemotherapeutic agents
long term use of antacids
what foods are B12 (cobalamin) in
Meat
milk
cheese
eggs
causes of B12/cobalamin deficiency
poor absorption
strict vegans
Alcoholism
Poor absorption (gastric bypass surgery, Crohn’s, Celiac)
Long term use of antacid
pancreatic insufficiency
Pernicious anemia (MCC): :lack of intrinsic factor in the gut to absorb B12
other name for B12
Cobalamin
B9/Folate deficiency causes
Poor intake
alcoholism
poor absorption
Medications (phenytoin, trimethoprim, sulfa drugs, pyrimethamine, methotrexate, OCPs (oral contraceptives)
Meds that cause B9/folate deficiency
phenytoin
trimethoprim
sulfa drugs, pyrimethamine, methotrexate
OCPs (oral contraceptives
B9 is
folate
foods with folate
vegetables (esp dark green leafy vegetables - spinach, asparagus, Brussel sprouts)
Fruits and fruit juices
nuts
beans
peas
dairy products
poultry
meat (liver)
Eggs
Seafood
Grains
dietary def that is a reversible cause of dementia
B12
which deficiency can cause neuro symptoms?
B12 or Folate
most common symptom?
B12
most common is sock and glove parasthesias
folic acid therapy
1mg PO daily for 1-4 months is sufficient
Parenteral form can be given for malabsorption
no established side effects
B12 therapy
Generally
1mg IM daily x 1 week
1 mg weekly x 1 month
1 mg q month
Can also do daily 1-2 mg PO daily however erratic absorption
Inexpensive, no known side effects
Treat reversible causes to prevent life long supplementation
Neurological side effects are usually reversible if treated within ____ months of onset
6
labs to order in Normocytic anemia
peripheral smear
LDH
indirect bilirubin
haptoglobin
retic
Creatinine
If you have low Hgb and low Hct with normal RDW and MCV
may be acute blood loss because body has had no time to compensate with increased production or fluid balance
hormone made in cortex of kidney to tell bone marrow to produce more blood
Erythropoietin (EPO
synthetic EPO
Epoetin or Darbepoetin
Black box warning for EPO
increased clotting risk (MI, CVA, DVT)
pure red cell aplasia
-immature RBCs
- a quantitative measure of bone marrow production for new RBCs, should rise in response to anemia
Reticulocytes
cells in Erythropoiesis
Proerythroblasts
Basophilic erythroblasts
Polychromatophilic erythroblasts
Orthochromatophilic erythroblasts
Reticulocytes
Erythrocytes
low ferritin is pathopneumonic for
iron deficiency anemia
Alcohol has a direct toxic effect on the ____ _____ (when talking about anemia)
bone marrow
Ethanol inhibits folate absorption in the
jejunum
for lupus, having a sibling with lupus increases your risk for developing by
8-20 fold higher risk
what genotypes have higher risk for SLE
Caucasians at higher risk with genotype by 2-3x
HLA-DR2 and HLA-DR3
African Americans
HLA-DR2 and DR7
gender higher risk for SLE
Equal in <5yo but increases 4-5x in females <16
what syndrome has increased risk for SLE
Males with Klinefelter syndrome have relative deficiency of androgens and increased frequency of SLE
drugs that cause medication induced lupus syndrome
Drugs
Most commonly Antiepileptic drugs (shipp)
SHIPP, where
S- Sulfonamide
H -Hydralazine
I -Isoniazid
P -Procainamide and Phenytoin.
factors (not genetic) that can induce lupus
Crystalline silica
cigarette smoking,
exogenous estrogens,
UV radiation,
viral infections and
industrial chemicals (industrial solvents, and pesticides)
Crystalline silica is a common mineral found in the earth’s crust. Materials like sand, stone, concrete, and mortar contain crystalline silica.
When you see a limp, think
this may be a rheum issue
proximal muscle weakness
Heliotropic rash
Gottron’s papules
Dermatomyositis
Dermatomyositis revealed what elevated labs
CPK
Aldolase
LDH
AST, ALT
In Dermatomyositis
What will you see on MRI and muscle biopsy
MRI: diffuse muscle inflammation
Muscle biopsy: inflammation, atrophy
Purpura (and petechiae)
Joint complaints - soft tissue swelling (+/- arthritis)
ABd pain -> obstruction (intussusception)
Renal involvement -> hematuria, proteinuria, and HTN
Henoch-Schonlein Purpura (HSP)
Scleroderma has what other organs involved
heart
GI tract
lung involvement
types of scleroderma
Morphea
Linear scleroderma
coup de sabre
systemic sclerosis - tight shiny skin with distal tapering
KD symptoms
Conjunctivitis (limbic sparing)
Rash
Adenopathy (cervical)
Strawberry tongue
Hands and feet
Burn (fever for 5 days)
Diagnostic criteria for KD
fever for >= 5 days and 4/5 crash and burn
Bilat, nonpurulent limbic sparing conjunctivitis
Rash: polymorphic - trunk, extremities, groin
Adenopathy:
-cervical
-Unilateral
-Node >=1.5 cm
Strawberry tongue
-prominent papillae
-erythema of oral mucosa and lips
Hands and feet changes
-swelling
-erythema
-periungual desquamation 2 weeks after fever onset
incomplete KD criteria
Children with >= 5 days fever and 2 or 3 compatible clinical criteria or
infants with fever for >= 7 days without other explanation
Then
CRP >3 and or ESR >= 40
plus 3 or more lab findings
1. anemia
2. platelet count of >=450,000 after the 7th day of fever
3) albumin <3 g/dL
4) elevated ALT
5) WBC of >=15,000
6) Urine >= WBC
or positive echocardiogram
Then treat
other path
CRP <3.0 and ESR <40
Then
serial clinical and lab re-evaluation if fevers persist
echocardiogram if typical peeling develops
diff types of rash seen in KD
Maculopapular
Morbiliform
Targetoid
Scarlatiniform
Never vesicular
what kind of rash is this
maculopapular
describe rash
Morbiliform
describe rash
Targetoid
describe rash
Scarlatiniform
describe rash
vesicular
lab findings in KD
Anemia
leukocytosis
thrombocytosis,
elevated CRP
elevated ESR
Hypoalbuminemia
elevated ALT
sterile pyuria
pleocytosis
Mainstays of KD treatment
IVIG
ASA moderate high dose for the first 48-72 hours and after fever subsided. Low dose for 4-6 weeks or until aneurysms resolve
If develop fever after IVIG 72 hours then it is refractory
2nd dose IVIG
IVIG 2 gm/kg and high-dose ASA 80-100 mg/kg/day every 6 hours
ASA dose is decreased to 3-5 mg/kg/day after the pt is afebrile for 48-72 hours. It’s then continued for 6-8 weeks for antiPLT affect. However, if coronary artery abnormalities are present, then ASA therapy is indefinite and based on risk level.
“pulseless disease” primarily affects the aorta and its branches→ can result in stenosis and occlusion
Takayasu Arteritis (TA)
Takayasu Arteritis (TA) gender and age
Frequently seen in females 15-30 years
HTN followed by loss of pulses and (+) bruits
Malaise, fever, abd pain, elevations in acute inflammatory markers, wt loss, visual abnormalities, HA, dyarthrias, arthralgias, and seizure
Other PE findings= extremity systolic BP variance (10 mmHg), subclavian artery/abd aorta bruit, decreased or absent brachial artery pulse, or normochromic/normocytic anemia
Takayasu Arteritis (TA)
Takayasu Arteritis (TA) s/s
HTN followed by loss of pulses and (+) bruits
Malaise, fever, abd pain, elevations in acute inflammatory markers, wt loss, visual abnormalities, HA, dyarthrias, arthralgias, and seizure
Other PE findings= extremity systolic BP variance (10 mmHg), subclavian artery/abd aorta bruit, decreased or absent brachial artery pulse, or normochromic/normocytic anemia
dx Takayasu Arteritis (TA)
US, MRI, MRA, CT angio, biopsy of affected vessel
MRA may provide greater clarification of vessel changes but will not detect calcification, which can help determine chronicity of inflammation
tx Takayasu Arteritis (TA)
corticosteroids to decrease progression, immunosuppression prevents restenosis and improves control, and endovascular intervention can increase 5 year survival to 80-95%. Typical cytotoxic medications include cytoxan, tocilizumab, MTX, and anti-TNF agents
KD is predominantly in what age
5 yrs and younger
Very rare in children older than 12 or younger than 3 months
Phases in KD
ACUTE PHASE 10-14 days
1. fever and classic presentation (CRASH and BURN) plus acute myocarditis
● SUBACUTE PHASE 2-8 weeks
1. Resolution of acute symptoms
2. Coronary aneurysms develop
● CONVALESCENT PHASE
1. Last many months
2. Clinical symptoms and lab findings resolve
3. Coronary aneurysms develop, persist, resolve
In IgA deficiency has a high association with what type of reaction
anaphylaxis
lupus with a type ___ hypersensitivity reaction
III
Anaphylaxis is a type ___ hypersensitivity reaction
1
most common anaphylaxis triggers
food (e.g., eggs, shellfish, nuts), medications (e.g., antibiotics, nonsteroidal anti-inflammatories), and stinging.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1339). Wolters Kluwer Health. Kindle Edition.
