Hematologic Peds

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Hematologic Peds
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2010-11-15 20:14:01
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Hematologic Peds
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  1. Most common anemai age
    • 6 mo to 2 years
    • 12-20 years
  2. Sympt of iron def. anemia
    • letheragy
    • irritability
    • muscle weakness
    • dizzyness
    • faiting w/exertion
    • SOB
    • Systolic heart murmor
    • heart failure
    • tachycard/tachypnea
    • pallor
    • imapired healing
    • thinning of hair
  3. Fatigue, lethargy, irritability
    iron defeciency anemia
  4. Muscle weakness, dizzyness, fainting with exertion
    iron defeciencyh anemia
  5. Sortness of breath, heart murmor, heart failure
    Iron def. anemia
  6. Tachycardia, tacypnea, pallor
    Iron def. anemia
  7. Impaired healing
    thinning of hair
    Iron def. anemia
  8. Iron supplement should not be given with
    milk
  9. Encouring what feeding for iron def. anemia
    breast feeding/iron fortified formula/iron fortified cereal
  10. Milk restrictions in toddlers
    • <1 qt of milk per day
    • No milk after a meal
    • don't carry cup of milk
  11. Increase what in diet to treat iron def. anemai
    • Vit C
    • Protein
    • Iron
  12. Iron rich foods are
    inadequate to treat iron def. anemai
  13. Use straw to
    prevent straining
  14. How iron is given
    IV, PO, IM
  15. Stool color in Iron def. anemia
    tarry green
  16. length of tx ffo Iron def. anemia
    3 mo
  17. monitor what labs with Iron def. anemia
    • rbc
    • hemat
    • hgb
  18. prolong anemia can cause
    growth retardation and developmental delays
  19. Signs of iron overdose?
    Vomiting, abd pain, bloody diarrhea, followe dby lethargy, dyspnea, shock
  20. Vomiting, abdominal pain and bloody diarrhea
    iron od
  21. lethargy, dyspnea, shock
    sign off iron OD
  22. overdose may appear better before
    metabolic alkalosis
  23. used to bind iron
    Deferal
  24. removes iron from body, _____________ agen
    chelating
  25. Inherited sickle cell anemia
    autosomal recessive
  26. SCA, genes come from
    each parent, 25% child will get
  27. Vaso occlusion is seen in
    SCA
  28. What causes vaso-occlusion
    increased RBC destruction, causes hypoxemia to adjacent tissues
  29. Painful crises associated with
    Vaso Occlusive crisis
  30. Prenantal screening for SCA
    8-10 weeks
  31. Newborn screening for SCA
    mandatory
  32. DX tests for SCA
    • Sickledex
    • Hemoglobin elecgtrophoresis
  33. Check for intracranial vascular flow and detect risk for CVA in what disease?
    SCA
  34. Cold and SCA
    makes it WORSE
  35. Transcranial doppler for what, and who?
    Tests for CVA in SCA, 2-16 year olds
  36. What vaccines in SCA
    pneumococcal, flu, mengiococcal
  37. Prophylactic what in SCA?
    PCN, prn bid
  38. 02 for SCA
    Short term with respiratory difficulty
  39. Fluids in SCA
    Hypotonic D5W or D5w/0.25NS
  40. Blood transfusions tx for
    SCA
  41. Splenectomy tx for
    SCA, life saving measure
  42. Goal in SCA
    prevent pain
  43. Manage pain in vaso occulive crisis
    • Motrin/tylenol opiods
    • PCA
  44. Contraindicated for pain in VO
    Meperidine/Demerol
  45. Pain in V O crisis r/t
    bone marrow ischemia
  46. Ischemia in hands and feet seen in
    hand foot syndrome ischemia in V-O crisis SCA
  47. Pain, cough, fever >102, retractions, decfreased 02 sats...think
    Fat embolism, Acute Chest Syndrome V O crisis, SCA
  48. Priapism seen in
    V O Crisis
  49. Hematuria, hyposthenuria seen in
    V O Crisis, SCA
  50. Hyposthenuria is
    low specific gravity
  51. Osteomyelitis see in
    V O crisis, SCA
  52. Retinopathy, blindness seen in
    SCA, V O Crisis
  53. Chronic skin ulcers seen in
    V O Crisis, SCA
  54. Hepatomegaly, splenomegaly seen in
    SCA, VO crisis
  55. Dacylitis seen in
    SCA, VO crisis
  56. Increased electrolytes in VO crisis....why?
    Damaged kidneys can't excrete electrolytes
  57. S/S of CVA
    • abnormal behhavior
    • seizures, slurred speach, wakness, vision chagnes, vomting, headache
  58. Blood transfusion how long following CVA?
    3-4 weeks
  59. CVA is a complication of
    VO Crisis, SCA
  60. Nursing intervention for VO crisis
    • Pain management,
    • Family ed (avoid stress)
    • Hydtration
    • Enuriesis
    • heat to affacted area
  61. Avoid stress or trauma
    VO, SCA
  62. Hydration
    SCA, VO
  63. May have enuriesiss
    SCA, VO
  64. Heat to affected area
    SCA, VO
  65. Bed Rest
    SCA, VO
  66. Prevent infection
    SCA, VO
  67. ROM
    SCA, VO
  68. Avoid exposure to cold
    SCA, VO
  69. Monitor labs
    SCA, VO
  70. Monitor liver function
    SCA, VO
  71. Inherited blood disorders
    Thalassemia (autosomal recessive)
  72. Who gets thalasemia
    African decendents or, italians, syrrians, greeks
  73. Types of Thallasemia
    Alpha, and beta
  74. Damages/hemolyzes RBC
    Beta Thalassemia
  75. 3 types of Betal Thallasemia
    • Minor Trait
    • Intermedia blood transfusions
    • Major - Coly'sanemai
  76. Severe Anemia
    Chronic hypoxia
    Growth retaradtion
    Fractures, skeletal deformities
    Coolye's anemai (Type III of Beta Thalassemia)
  77. Skeletal deformities
    Coolye's anemai (Type III of Beta Thalassemia)
  78. Chronic hypoxia
    Coolye's anemai (Type III of Beta Thalassemia)
  79. Severe anemai
    Coolye's anemai (Type III of Beta Thalassemia)
  80. Prominent and proruding forehead
    Coolye's anemai (Type III of Beta Thalassemia)
  81. Maxillary prominence
    Coolye's anemai (Type III of Beta Thalassemia)
  82. Wide set eyes
    Coolye's anemai (Type III of Beta Thalassemia)
  83. Flattened nose
    Coolye's anemai (Type III of Beta Thalassemia)
  84. Acccomodates hyperplastic marrow
    Coolye's anemai (Type III of Beta Thalassemia)
  85. Too much iron
    Hemosiderosis, Coolye's anemai (Type III of Beta Thalassemia)
  86. Bronze sin, leg ulceres
    hemosiderosis, Coolye's anemai (Type III of Beta Thalassemia)
  87. Splenomegaly, endocrine abnormalities
    Hemosiderosis, Coolye's anemai (Type III of Beta Thalassemia)
  88. Liver and gallbladder disease
    Hemosiderosis, Coolye's anemai (Type III of Beta Thalassemia)
  89. Osteopororiss
    Hemosiderosis, Coolye's anemai (Type III of Beta Thalassemia)
  90. DX for Cooley's anemia
    H&H, Iron levels
  91. What hemoglobing above what level in Cooley's anemia?
    9.5
  92. Deferoxamine
    Chealting therapy with transfusions
  93. Splenectomy in some children
    TX for Coolye's anemai (Type III of Beta Thalassemia)
  94. When spleet is removed be careful of....tx with....immunize for
    • infection
    • Prophylactic antibiots
    • pneumococcal, meningococcal
  95. Failure of bone marrow to prduce blood cells
    Aplastic anemia
  96. Bone marrow aplasia & pancytopenia
    Aplastic anemia
  97. Profound anemia
    Leukopenia
    Thrombocytopenia
    Pancytopenia, seen in aplstic anemia
  98. Fanconi's inerited
    type of aplastic anemia (autosomal recessive)
  99. Exposure to toxins, environement, viruses, myelosuppressive drugs, other drugs such as sulfanamides, anit inflamatory, gold salts, anticonvulsants, tridione, dilantin, tegretol, antimalarials, analgesics and radiotion can cause
    aplastic anemia
  100. Most cases are idiopathic
    aplastic anemia
  101. How to diagnose aplastic anemai
    • CBC
    • Guaiac postive stool
    • Hematuria
    • Bone marrow aspiration
  102. definistive dx of bone marrow aspiration
    DX of aplastic anemia
  103. Guaiac positive stool
    dx of aplastic anemai
  104. Hematuria
    DX of aplastic anemia
  105. Bone marrow transplant
    TX for aplastic anemia
  106. ALG
    Immnosuppresive therapy for TX for aplastic anemia
  107. ATG
    Immunosuppresive therepay for TX for aplastic anemia
  108. Cyclospirine
    Immunosuppresive therapy, TX for aplastic anemia
  109. Hypertension
    Excessive body hair
    Urea stays in body
    gingival hypertorphy
    immunodefici
    increased creat
    irreversable nephrotoxicity
    P. Carinni pneumonia
    Commmon side effects of cyclosporine
  110. More successful before multiple transfusions adminsitered
    Hematopoietic stem cell transplant, TX for aplastic anemia
  111. Hematopoeitic stem cell transplant
    TX for aplastic anemia
  112. 85% survival rate in untransfused
    TX for aplastic anemia, hematopoietic stem cell tx
  113. 70% survival rate in transused
    hematopoeitic stem cell transplant,TX for aplastic anemia
  114. Prevent complications
    assess for hemorrhage
    ecchymoses, petechiae, purpura
    tachycarida
    tachypnea
    stool softener to prevent anal fissures
    TX for aplastic anemia
  115. stool softener
    TX for aplastic anemia
  116. 80% X linked recessive
    Hemophilia
  117. Mothers to sons
    Hemophilia
  118. Factor VIII defienciey
    Classic hemophilia
  119. Facor IX deficiency
    heophilia B or christmas disease
  120. Factor XI defenciency
    Hemophilia C
  121. Affects men and women
    • Hemophilia C and
    • Von Willebrand disease
  122. Def of factor VIII and Von willebrand
    Von Willebrand disese
  123. Bleeds into sq and IM tissue
    Hemophilia A
  124. Hemarthroisis, bleeds into joint space
    Hemophilia A
  125. Swelling, warmth, redness, pain, loss of movement, bruising
    Hemophilia A
  126. Hx of bleeding episodes, epistaxis, bleeding gums, hematuria, tarry stools
    Hemophilia A
  127. Headahe, slurred speech, decrease LOC
    Hemophilia A
  128. Chest or abdominal pain
    Hemophilia A, consider internal injury
  129. Assess circulation
    inquire about any injury
  130. Factor levels, dx for
    Hemophilai
  131. DNA test
    DX for hemophilia
  132. PTT, dx for
    hemophilia
  133. hgb, hct dx for
    hemophilia
  134. Replace factor VIII
    txx hemophilia
  135. corticosteroids tx for
    hemophilia
  136. NSAIDS used in caution , tx for
    Hemophilia
  137. Phyiscal activity and exersise, tx for
    hemophilia
  138. Infusion of VIII prophylaxis of...before onset of
    hemophilia, joint damage
  139. Primary prophylaxis in hemophilia
    infusion of VIII on regular basis before onset of joint damage
  140. Secondary prophylaxis for hemophilia
    Regularly after the first joing bleed. Given 3 x a week
  141. Active bleed tx
    • Pressure
    • Rest
    • Ice
    • Compression
    • Elevation
    • Factor replacement
  142. No cure
    Hemophilia
  143. If older than 25, and blood transfusions
    HIV risk
  144. ITP
    Idiopathic thrombocytopenic purpura
  145. Excessive destruction of palteltes (thrombocytopenia)
    Idiopathic thrombocytopendc purpura
  146. Normal bone marrow with normial or increase number of immature platelets
    Idiopathic thrombocytopenic purpura
  147. Discoloration caused by petechiae beneath the skin
    Idiopathic thrombocytopenic purpura
  148. Acute, self limiting course...a form of
    ITP
  149. Chronic condition, greather than 6 months duration a form of
    ITP
  150. Acute ITP seen after
    • URI
    • MMR
    • Vaaricella
    • Parvovirus
  151. DX for ITP based on clinical manifestations
    • Easy bruising
    • petechiae
    • eccymosis
    • bleeding from mucous memebrane
    • epistaxis
    • bleeding gums
    • internal hemorrage
    • hematomas over LE
  152. Platelet count reduced to 20,000
    ITP
  153. WBC's, HGB, HCT in ITP
    stay normal
  154. Watch bleeding times in
    ITP
  155. No definitive test
    Idiopathic thrombocytopenic purpura
  156. May do tests to rule out other disorders
    Idiopathic thrombocytopenic purpura
  157. Restrict activty
    Idiopathic thrombocytopenic purpura
  158. IVIG or until paltelest up to 30000
    Idiopathic thrombocytopenic purpura
  159. Platelest transfusion in Idiopathic thrombocytopenic purpura only if
    life threatening
  160. Splenectoy for ITP if it lasts longer than
    1 year
  161. NO contact sports
    Idiopathic thrombocytopenic purpura
  162. Avoid ASA and NSAIDS, use acetaminophne instead
    Idiopathic thrombocytopenic purpura
  163. Is a secondary condition
    DIC
  164. activation of coagulation
    DIC
  165. Thrombin generated, fibrin deposited in circualtion, platelets are consumed
    DIC
  166. Deficiency of coag and anticoag pathways occur
    DIC
  167. Results in hemorrage and organ/tissue damage
    DIC
  168. Septic shock is a trigger of
    DIC
  169. Endotoxins and viruses are triggers of
    DIC
  170. tissue necrosis or injury is a common trigger of
    DIC
  171. cancner treatment is a trigger of
    DIC
  172. Hemolytic transfusion reactions (anaphylaxis) can be a tigger for
    DIC
  173. Hypotension or hypoxia is a trigger of
    DIC
  174. Snake bites trigger
    DIC
  175. Pancreatitis, and liver disease can trigger
    DIC
  176. Suspect when there is a tendency to bleed
    DIC
  177. Bleeding from venipuncture and surgical sites, clinical manifestation of
    DIC
  178. Petechiae and purpura are manifestations of
    DIC
  179. Tx DIC
    • Control couase
    • anticoagulants
    • clotting factors
    • platelets, fresh forzen plastma
    • cryoprecipitates
    • labs
    • respiratory status
    • assess loc
  180. anticoagulants and clotting factors are treatmetns for
    DIC
  181. clotting factors, platelets and fresh frozen plasma are tx for
    DIC
  182. cryoprecipitates
    rich in factor VIII, frozen, thawed, tx for DIC
  183. Epistaxis tx
    • lean forward
    • contininous pressure
    • ice or cold if persists
    • calm quiet child
  184. malignant disease of bone marrow an lymph system. all cell types are effected
    leukemia
  185. normal elements in bone marrow are repalced with abnormal white blood cells
    Leukemia
  186. 2 types of Leukemia
    • ALL
    • AML
  187. most common type of Leukemia
    ALL
  188. Leukemia in children 2-8
    ALL
  189. less than 1 year old with Leukemia
    poor prognosis
  190. cancer seen in caucasions
    Leukemia ALL
  191. Abnormal lymphocytes, no abilityt ofight infection, crown out normal cells
    Leukemia ALL
  192. Cure rate 65-75%
    Leukemia ALL
  193. Cancer peaks during adolescense
    AML Leukemia
  194. Poor prognosis for this cancer
    AML Leukemia
  195. Presenting signs of leukemia (TEST)
    • 1.)Anemia
    • 2.)Infection
    • 3.)Bleeding
  196. Hepatosplenomagaly more comman than lymphandenopathy in
    Leukemia
  197. Can have increase ICP from CNS involvement
    Leukemia
  198. Originates in lymphoid system
    Hogdkins disease
  199. primary involves lumph
    Hogdkins disease
  200. Metastisises to non lodal istes such as spleen, liver, bone marrow and lungs
    Hogdkins disease
  201. Ann arbor staging for
    Hogdkins disease
  202. A in ann arbor staging
    Asymptomatic
  203. B in Annn Arbor staging
    Fever of 100.4 or great for 3 conseccutive days, night sweats, weigh tloss over past 6 mo.
  204. Enlarged cervical or supraclavicular lymphandenopathy, and is the most common presentation
    Hogdkins disease
  205. Multiple organ invoelvement
    Hogdkins disease
  206. Lymph node biopsy for staging and histology diagnosis
    Hogdkins disease
  207. Presense of reed sternburg cells
    Hogdkins disease
  208. Giant cells with huge nuclei
    Hogdkins disease, reed sternberg cells
  209. painless enlarged lyph nodes, ervical most common
    Hogdkins disease
  210. Nonproductive cough (mediastinal nodes)
    Hogdkins disease
  211. Abdominal pain (retoperi toneal nodes)
    Hogdkins disease
  212. Radiation and chemotherpay, tx for
    Hogdkins disease
  213. Long term survival rates excellent
    Hogdkins disease
  214. Mutation B & T cells
    Non-Hogdkins lymphoma
  215. Agressive malignancy
    Non-Hogdkins lymphoma
  216. Responseive to treatment
    Non-Hogdkins lymphoma
  217. managed like hodgkins
    Non-Hogdkins lymphoma
  218. Spreads easy to CNS
    Non-Hogdkins lymphoma
  219. Progresses quickly
    Non-Hogdkins lymphoma

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