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10 Cards in this Set
- Front
- Back
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Two components of hemoglobin:
What is the function of the globin chains? What is the 1st step in heme synthesis? |
tetra pyrrhole ring and 4 globin chains
make hydrophobic "cage" around the heme Delta-aminolevulineic acid (ALA) |
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Class of diseases with defects in heme synthesis:
What are the major symptoms of porphyria? Disease with defect in PBG deaminase: What is increased in the urine with AIP? |
porphyria
cutaneous photosensitivity, neuro S/S Acute Intermittent Porphyria ALA, PBG |
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What substance stops porphyrin formation when ingested?
What are the most common S/S of AIP? What are some drugs that can cause AIP? Treatment for AIP? |
hematin
Abd pain, N/V/ileus, tachycardia, HTN; also neuro sx (seizures, psychosis) Dilantin, Tegretol, Depakote, barbiturates, Sulfa, EtOH D5/D10 IVF, hematin, BB's for tachycardia/HTN, analgesics |
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Most common porphyria, usually seen after EtOH ingestion, defect in uroporphyrinogen decarboxylase:
Major symptom of PCT? Which system is not affected? What do you see on the skin in PCT after minor trauma? What are some other symptoms and lab values? Treatments? |
Porphyria Cutanea Tarda
cutaneous reactions, no neuro S/S bulla post trauma abnormal LFT's, increased Fe in liver, ALA in urine, porphyin in stool D/C exacerbating factor, phlebotomy for high iron, chloroquin |
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Diseases with defects in globin production:
What kind of anemia does B-thal cause? What types of cells do you see on PS? What are some skeletal changes you see? What are treatments for homozygous B-thal? |
Thalassemia
microcytic/hypochromic teardrop cells, target cells bone marrow hypertrophy primary treatment - transfusions; also desferrioxamine for Fe overload |
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Hgb Bart's is associated with what condition?
Fetal homozygous A-thal has what globin chains instead? Adult homozygous A-thal has what globin chains instead? |
A-Thal - hydrops fetalis
4 gamma chains adult - 4 B chains - Hgb H |
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In SCD, which form of Hgb is the oxygenated state? deoxygenated?
What is the mutation in SCD? What is the most common type of crisis in SCD? What is the most common symptom of a crisis? |
oxygenated - R
deoxy - T Glut-6-Val infarctive - sickling, aggregation, blocks small vessels Pain |
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What type of SCD crisis involves decreased erythropoiesis secondary to infection, such as Parvo B19?
Major cause of death in children with SCD: What are some clinical changes with SCD? |
aplastic
Sequestration RBC's pool in the spleen - Hgb < 6 bone changes - medullary thickening, infarcts - osteosclerosis, dactylitis; increased risk of infection in bone (Salmonella) |
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What type of anemia does SCD cause? What are the lab values for bili, retics, nucleated RBC's and H&H?
Treatments for SCD and crises? |
normocytic, normochromic - increased bili, retics, nucleated RBC's; decreased H&H
avoid O2 changes - surgery (tranfuse 1-2 weeks prior), airplanes treat infections aggressively - Abx crises - hydration, hospitalization, analgesics bone marrow transplant |
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Hgb S "wannabe", splenomegaly, more benign S/S:
Glut-121-Lysine mutation, minimal S/S: |
Hgb C
Hgb D |
