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28 Cards in this Set
- Front
- Back
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There must be a _:1 balance of production of a and B-like chains, or there will be production of _______ hemoglobins.
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1:1
abnormal hemoglobins. |
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Retics are what % at birth in a normal individual? Is there as much fat in the marrow?
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3-7%.
No, productive marrow fills all of the bones. |
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There is a precipitous [Hb] ____ in the first few days/months following birth in a normal individual. What is this called?
What is the etiology of this? |
drop. Physiologic anemia of infancy.
increase in environmental oxygen leads to a drop in EPO --> drop in retic + shortened lifespan of neonatal RBCs --> physiological anemia of infancy |
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Is the physiological anemia of infancy more of a concern in term babies or preterms?
Which sex sees a Hb spike (and an iron requirement spike) in the teenage years? |
preterms.
Both, but males are higher. |
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In a recent study, __% of american adolescent girls did not meet the recommended daily req. for iron.
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75%
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What are some childhood risk factors for iron def. anemia?
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low iron stores due to prematurity/low bw + very rapid growth in these infants.
exclusive breast feeding w/o iron supplementation for >6mo. Weaning foods low in iron (more of a concern in 3rd world) |
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Which has a higher risk of iron def. anemia by 9-12mo:
milk or non-fortified formula or exclusively breastfed |
milk/nonfort... @ 30-40%...
breastfed is still high @ 20% tho'. |
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What is the most common cause of anemia world-wide, in children and adults?
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iron def. anemia.
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What are some special risks to iron def. children?
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delayed psychomotor development / academic achievement
poor growth increased risk for lead toxicity due to pica and increased abs of led in the iron def state. |
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Are conduction times in nerves slower or faster w/o iron?
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slower due to effect of lack of iron on myelination.
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How do you Dx IDA in children?
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Upon focused lab assessment, first ask yourself:
is the child anemic based on the mean age-specific norms? Is there microcytosis based on age-specific norms? |
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Which two tests are able to detect iron def. anemia even in cases of infection?
What are the cutoffs? |
sTfR = soluble transferrin receptor
sTfR-F index = (sTfR / log Ferritin) >2 and >1.5, respectively. |
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Is iron def. bad for kids even w/o anemia?
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Yes.
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There is some data from developing countries that iron therpay may _____ morbidity from malaria and bacterial infections.
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increased, we still think it does more good than harm tho'.
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I'm gonna list phenotypes, you give me genotypes:
a-thalassemia-2 trait a-thalassemia 1 trait a-thalaseemia 2 homo. |
a - /aa
- - / aa a - / a - |
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In all of the a-thal disorders, what is present on newborn screen?
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Gamma4 Hb = Barts.
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Why is anemia related to acute infection problematic? What may lead to this confusion?
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it can be confused with IDA on the basic tests. sTfR can help distinguish tho.
It may be the action of hepcidin (remember back from AOCD), it is 'hiding' iron. |
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Here are the sickle hemoglobinopathies in order of commonality:
SCD-SS, SCD-SC, SCD-S+thal, and SCD-S0thal, with approximately two-thirds of the SCD cases being SCD-SS Order them by severity. |
SCD-SS and SCD-S0thal are considered to be equivalent, and more severe than SCD-SC, which is more severe than SCD-S+thal.
Importantly though, this is not absolute, and severity can overlap. |
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What does newborn screening for hemoglobinopathies accomplish?
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permits pneumococcal prophylaxis as well as parental education.
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Do factor VIII and IX cross the placenta? What does this mean re: how soon we can test newborns?
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no. can test 'em as soon as they're born... just note that K-dependent factors are lower in kids, may impair Hemophil B dx.
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There are at least 3 things you should know about a hemophilia patient before treating a bleed:
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A or B?
mild, moderate, or severe? inhibitor or not? |
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What is a "target joint" re: hemophilia? What can help prevent this?
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hemophilic arthropathy --> synovial atrophy --> target joint more prone to future bleeds.
use factor prophylaxis in addition to the classic "on demand" therapy. |
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Once pts develop antibodies to the administered factor (inhibitors), whatt can be done?
Why is gene therapy promising re: hemophilia? |
we can use bypassing agents, but the ultimate goal is to induce ITI (immune tolerance induction) in which we use regular, high-dose infusions of factor to desensitize immune system.
only a small bit of activated gene (like prophylaxis) would have the desired effect. |
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What is the difference between ITP in adults and that in kids?
How is it dx in kids? Likely etiology? What are things that should steer us AWAY from a classic childhood dx of ITP? |
80% of kids will resolve w/i 6 mo. Adults usually get is chronically.
Clincally - mucocutaneous (platelet type bleeding).. viral infection. histories of pallor, night sweats, weight loss... organomegaly. **all red/white blood cell morphology should be normal!** |
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Has it ever been shown that medical tx reduces the incidence of ICH (intracrainal hemorrhage) in childhood ITP?
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No...
**but maybe ask Dr. Ma about this, or take it w/ a grain of salt. this seems very strange**. A: kids are diff than adults, pt count of 2 in adults --> treatment... not necessarily so in kids. |
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Chronic ITP (lasting >6-12 mo) is ____ common in children.
Risk factors? Can resolution occur? Is splenectomy effective? |
less
age>10, absence of viral prodrome yes, can occur, sometimes spontaneously after years. Yes, it is effective in up to 80% of chronic ITP. |
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I'm gonna give the birth pattern and adult pattern, give me the interpretation:
FS --> S F A2 FS --> S F A2 FSA --> S A F A2 FSC --> S C F A2 |
SSA
Sickle-B0thal sickle-B+thal Sickle-HbC diz |
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differentiate b/t childhood ITP and Adult ITP.
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Childhood:
females = males abrupt onset infectious prodrome common <20% chronic Adult: Females > males (2:1) insidious onset infectious uncommon >50% |
