Glossary of sickle cell disease
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- Hemoglobin A (HgA) is replaced by ? in sickle cell disease.
- (HgS)abnormal sickled Hemoglobin
- The 4 most common diseases related to sickle cell disease in the US are?
- Sickle cell anemia, Sickle cell C disease, Sickle cell hemoglobin E disease, Sickle cell thalassemia disease.
- This, the most common form, is said to the result of selective protection afforded trait carriers against one type of malaria.
- Sickle Cell Anemia
- HgS is on an autosome & is always detectable(when present) and dominant. People who have both HgA & HgS are considered?
- to be carriers of the sickle cell trait
- If both parents have the sickle cell trait there is a ___% chance for each preg to have SCA.
- The HgS is usually not apparent until later in infancy because of ___ that is given by the mother in utero.
- HbF- fetal hemoglobin & it takes the place of the HbS so there is less to cause problems.
- The HbF will last about ___ and then the infant may begin to show s/s of SCA.
- about a year
- SCA is considered a disease of A.obstruction/destruction B.construction/destruction C.destruction/instruction
- Obstruction & destruction
- The sickle shaped cells get all tangled up and cause vaso-occlusion. Results are 3 major issues.
- Hypoxia/Ischemia/Infarction (cell death)
- The sequence of events following occlusion to organs are:123
- 1.Stasis with enlargement 2.Infarction w/ischemia & destruction 3.Replacement with fibrous tissue (scarring)
- The most acute symptom of SCA is?
- exacerbation called "crisis"
- The 5 types of SCA crisis are?
- Vaso-occlusive, Sequestration, Aplastic, Hyperhemolytic, Chest Syndrome (like pneumonia)
- Lehann said to remember that the blood can filter on through, its just tangled up. So it's not considered a ?
- Clot- it is blood & it is an occlusion but not a clot
- Which is the main or #1 crisis & the more often cause of going to ER w/SCA?
- The FIRST symptom in kids is usually?
- Pain in the extremities
- The syndrome noted for symmetrical swelling of hands/feet, painful joints, abdominal pain & you may think they have appendicitis
- Hand and Foot syndrome (clinical name is dactylitis)
- vaso-occlusion where? 1.causing vision chgs & stroke 2.episodes of pulmonary disease/pneumonia
- 1.Cerebrum 2.Acute chest syndrome 3.liver
- VO crisis where? if: 1.hematuria 2.priapism
- 1.kidneys 2.genitals
- Crisis of pooling of large amounts of blood in the liver & spleen, <blood volume, poss shock, poss spleenectomy
- Sequestration crisis
- Dont produce RBC's, severe anemia, Hgb is low
- Aplastic crisis
- accelerated rate of destruction of RBC's- faster than you can make them, anemia, jaundice
- Hyperhemolytic crisis
- Most frequent problem occuring w/SCA
- vaso-occlusive pain asstd w/vaso-occlusive crisis
- SCA diagnostics include
- Hgb electrophoresis-newborns b/c presence of HgF;Blood smear shows sickled cells
- If not diagnosed in infancy its usually dx when
- young child following a URI or GI infection
- Physical exam; growth chart can indicate
- slow growth, puberty delayed- will eventually catch up- but hard time through middle/high school
- Common findings-
- tachycardia, murmurs, pallor, scleral icterus, jaundice, hepatosplenomegaly
- If SCA gets hypoxia what will happen
- hypoxia=metabolic acidosis=sickling=severe crisis
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