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68wm6 p2 Ped Hem Dis
Pediatric Hematologic Disorders
| Question | Answer |
|---|---|
| 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
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