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68wm6 p2 Ped Hem Dis

Pediatric Hematologic Disorders

QuestionAnswer
What are the formed elements of blood? *Erythrocytes *Leukocytes *Thrombocytes
How does anemia lead to heart murmurs in children? Severe anemia decreases peripheral resistance. --> Increased amount of blood returned to heart. --> Murmur
Supportive Tx of anemia: *Supplemental oxygen *Bedrest *IV fluids
What children are at highest risk of iron deficiency anemia? 12 to 36 months
What is the major staple for Tx and prevention of iron deficiency anemia in children? Cow's milk
True or False: If an infant is severely anemic, whole blood is the best Tx. False. No whole blood, can cause circulatory overload. Use packed RBC's!
What can iron to do the patients stools? Turn stools tarry green
Why must liquid iron be given with a syringe, straw or dropper? Can stain teeth
What must be done if iron is given I.M.? Z-track method
True or False: Newborns with sickle cell anemia are at higher risk than healthy adults. False. The newborn with sickle cell are generally asymptomatic due to fetal Hbg
List four S/Sx of Sickle Cell Anemia: *Enlarged spleen *Pain in left abdomen *Appears pale *Unusual sleepiness *Rapid heart beat
What diagnostic tests are used to screen for sickle cell anemia? *Sickledex *Hemoglobin eletrophoresis
What pain medication is NOT to be given for pain associated with sickle cell anemia? Demerol
What is the most common type of sickle cell crisis? Vaso-occlusive.
What is beta-thalassemia? Refers to inherited blood disorders characterized by deficiencies in the rate of production of specific globin chains in Hgb (Partial or complete deficiency in the synthesis of hemoglobin)
Who does beta-thalassemia occur most often in? Occurs most often in persons leaving near the Mediterranean sea.
What are the four forms of Beta-Thalassemia? *Minor *Trait *Intermedia *Major a.k.a. Cooley anemia
How does thalassemia intermedia manifest? Splenomegaly and moderate to severe anemia
Which form of beta-thalessemia is asymptomatic? Thalassemia minor
Which form of Beta-Thalassemia results in severe anemia that can lead to cardiac failure and death in early childhood without treatment? Thalassemia major / Cooley anemia
What is aplastic anemia? : a bone marrow failure condition in which the formed elements of blood are simultaneously depressed (pancytopenia)
What is fanconi syndrome? pancytopenia, hypoplasia of bone marrow and patchy brown discolor
How does the discoloration of fanconi syndrome appear? Patient shows characteristic café-au-lait skin pigmentation. Generalized hyperpigmentation, increasing with age, and areas of hypopigmentation may occur
If antithymocyte globulin (ATG) is given in Tx of Aplastic Anemia, how must it be given? through a central vein
What is hemophilia? a disorder where the blood does not clot normally and even the slightest injury can cause severe bleeding
Fill in the blanks: The inheritance pattern of hemophilia is demonstrated as X-linked recessive so it affects _____ almost exclusively, but is transmitted by symptom free _____ via a defective gene on the X chromosome Males, Females
If a father with hemophilia and a mother who is a non-carrier have children, what are the odds that their male children will have hemophilia? Zero. Hemophilia is carried on the X-chromosome. Men get the Y-chromosome from father and X-chromosome from mother, so if father has hemophilia and mother doesnt carry, sons will not carry the gene. ALL daughters, however, will be asymptomatic carriers.
What form of hemophilia affects males and females? Von Willebrand disease
Hemophilia-A is a Factor VIII deficiency hemophilia. What is factor VIII needed for? Factor VIII is needed for forming thromboplastin (1st phase of coagulation)
What are common S/Sx of Hemophilia? Prolonged bleeding. Hemarthrosis. Hematomas. Spontaneous hematuria.
What is leukemia? a malignant disease of the blood forming organs of the body that results in an uncontrolled growth of immature white blood cells (blasts or stem cells)
What is a definitive test for Hodgkin's lymphoma? A blood test that identifies Reed-Sternberg cells
Created by: Shanejqb