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60 Cards in this Set
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anemia
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A reduction in oxygen carryng capacity resulting in decreased tissue oxygenation.
measured as a decrease in RBC, Hgb or Hct. |
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Symptoms of Anemia
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A reduction in oxygen carryng capacity resulting in decreased tissue oxygenation
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Clinical Manifestations of Mild Anemia
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Skin Mucosa: Normal
Heart: No Change CNS: No Change |
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Clinical Manifestations of Moderate Anemia
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Skin Mucosa: Pallor
Heart: Tachycardia, Murmur CNS: Headache |
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Clinical Manifestations of Severe Anemia
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Skin Mucosa: Marked Pallor
Heart: Tachycardia, Murmur, Heart failure CNS: Fainting, Syncope, seizures |
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Signs of hemolytic anemia or dyserythropoiesis
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jaundice
splenomegaly maxillary hyperplasia frontal "bossing" |
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What are anemia clues for iron deficiency?
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Excessive milk intake
Pagophagia Pica Koilonychia |
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What are anemia clues for hemolysis?
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- neonatal jaundice
- splenectomy - cholecystectomy |
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What are anemia clues for Vit. B12 deficiency?
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- Abdominal surgery (ileal resection)
- Glossitis - Decreased proprioception and vibratory perception |
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Pica
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A perverted appetite for substances not fit as food or of no nutritional value;
Dirt, cloth, clay... |
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Koilonychia
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spooning of nails...indicative of iron deficiency
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Skin Mucosa:
Heart: CNS: |
- COMPLETE BLOOD COUNT (CBC)
- RETICULOCYTE COUNT - PERIPHERAL BLOOD SMEAR |
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WHAT’S IN THE CBC ?
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1. HGB
2. RBC 3. MCV 4. RDW 5. HCT 6. MCH 7. MCHC |
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Microcytosis
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A reduction in the size of red cells (erythrocytes). Measured as a decrease in the mean cell volume (MCV) of red cells.
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What causes macrocytes to be so large?
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Defects in DNA synthesis
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What causes microcytes to be so small?
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Defects in Hgb synthesis
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What do microcytes look like?
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microcytic (low MCV)
hypochromic (low MCH) |
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What is the function of ferrochelatase?
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to combine protoporphyrin IX with Fe2+ ...making heme
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Causes of Microcytic Anemias
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1. Iron deficiency anemia
2. Disorders of iron metabolism 3. Sideroblastic anemias 4. Thalassemias 5. Hemoglobinopathies |
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CBC in Iron Deficiency
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LOW: RBC, Hb, Hct, MCV, MCH, MCHC
HIGH: RDW |
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Why is RDW high in iron deficiency?
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becuase there is not enough iron for all the cells and some cells get more iron than others
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Components of IRON PROFILE
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1. Serum Iron
2. TIBC / Transferrin 3. % Iron Saturation of TIBC 4. Serum Ferritin |
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Lab Tests in Iron Deficiency
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1. IRON PROFILE (standard)
2. Soluble Transferrin Receptor 3. Free Erythrocyte Protoporphyrin |
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TIBC
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Total Iron Binding Capactiy
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What must an iron profile show in order to be diagnostic of iron deficiency?
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low serum iron with high transferrin
(b/c transferrin is increased when there is a demand for iron) this dx is true except for pregnant individuals |
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What are the states from normal to iron deficient anemia?
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Normal
Iron Depletion Iron Deficient Erythropoiesis Iron Deficiency Anemia |
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Which of these is altered in iron depletion: Plasma Ferritin, Transferrin Saturation, RBC Protoporphyrin, Hemoglobin?
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plasma ferritin is low (<12)
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Which of these is altered in iron deficient erythropoiesis: Plasma Ferritin, Transferrin Saturation, RBC Protoporphyrin, Hemoglobin?
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plasma ferritin is low (<12)
transferrin saturation is low (<16) RBC Protoporphyrin is high (>100) |
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Which of these is altered in iron deficient erythropoiesis: Plasma Ferritin, Transferrin Saturation, RBC Protoporphyrin, Hemoglobin?
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Plasma Ferritin is low (<12)
Transferrin Saturation is low (<16) RBC Protoporphyrin is high (>100) Hemoglobin is low (<12) |
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What is the most common cause of iron deficiency in adults?
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BLOOD LOSS
- tusually from large bowel especially males and post-menopausal females |
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What are causes of iron deficiency in adults?
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Blood loss
Pregnancy and Lactation (increased requirements) Decreased Absorption - decreased gastric acidity - small bowel disease Excessiver menstrual blood loss |
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What percent of toddlers and adolescent girls have iron deficiency (with anemia)?
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9% iron deficient
2% with anemia |
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How much of dietary iron is from supplemental feedings in breast fed infants from 6-34 months?
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>95% is from supplements
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How many people have nutritional iron deficiency worldwide?
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2 billion
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How much iron does breast milk have?
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0.2-0.4mg/L
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How much iron does cow milk have?
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0.7mg/L
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Why is breast milk the best iron source?
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because breast milk is more readily absorbed that cow's milk
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What makes an infant at Risk for Iron Deficiency?
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1. Low birthweight/prematurity
2. Perinatal bleeding 3. Low hemoglobin at birth 4. High growth rate 5. Early cow’s milk and solid food intake 6. Frequent tea intake by mom (tea chelates iron) |
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What are diagnostic clues for infants with iron deficiency anemia?
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-EXCESSIVE MILK INTAKE
-PICA |
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Pagophagia
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Compulsive and repeated ingestion of ice
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Where is Iron absorbed in GI tract?
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proximal portion of jejunum
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Hemoglobin E / Beta Thalassemia
(compound heterozygotes) |
MODERATELY SEVERE ANEMIA
SPLENOMEGALY GROWTH DELAY TRANSFUSION REQUIREMENT |
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Hemoglobin E
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1. B-CHAIN MUTATION (B26lys..glu)
2. PREVALENT IN S.E. ASIA 3. MICROCYTIC, HYPOCHROMIC 4. INTERACTS WITH B-THALASSEMIA |
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Beta Thalassemia Major
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a.k.a. Cooley's anemia
- VERY LOW HEMOGLOBIN - TRANSFUSION DEPENDENT - AUTOSOMAL RECESSIVE - BONE MARROW TRANSPLANT CAN BE CURATIVE |
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Beta Thalassemia Minor
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a.k.a. B thalassemia trait
heterozygote - MILD MICROCYTIC HYPOCHROMIC ANEMIA. - ELEVATED Hb. A2 (basically diagnostic) - AUTOSOMAL DOMINANT - Low MCV - Normal RDW |
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Beta Thalassemias are because of mutations or deletions?
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MUTATIONS
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Alpha Thalassemias are because of mutations or deletions?
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DELETIONS
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Hemoglobin H Disease
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- 3 alpha genes deleted
>moderately sever hemolysis >elevated reticulocytes >red cell inclusions on supravital staining |
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Alpha Thalassemia Trait
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Thalassemia Minor (2 alpha genes deleted)
> microcytic, hypochromic RBCs >Target cells >Decreased MCV and MCH >Normal RDW >Mild anemia |
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What is your classification is you have one alpha hemoglobin gene deletion?
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Silent Carrier
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What is your classification is you have four alpha hemoglobin gene deletions?
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Hydrops fetalis
>fatal in utero because of heart failure |
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alpha 0 trait
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when one chromosome has two alpha deletions, but the other has two alpha genes
(thalassemia minor) |
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alpha + trait
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when there is one alpha gene deletion on a chromosome
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homozygous alpha + trait
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when both chromosomes have one alpha gene deletion
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Hemoglobin Barts
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gamma chain tetramer
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Hemoglobin H
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beta chain tetramer
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thalassemias are the result of globin chain synthesis _____
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imbalance
quantitative disease |
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Sideroblastic anemia
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- microcytic, hypochromic anemia
- iron accumulated in mitochondria |
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Congenital causes of Sideroblastic anemia
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X-linked
Autosonal Recessive Mitochondrial Disease (mutation/deletion in mitochondrial DNA, defect in enzyme fxn) |
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Acquired causes of sideroblastic anemia
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1. Drugs (isoniazid)
2. Toxins (lead...interferes with protoporphyrin synthesis) 3. Neoplastic (myelodysplastic syndrome) |