peds3.txt

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adamarine
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peds3.txt
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2011-09-19 23:41:59
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ped3
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  1. pediatric hematology
  2. General principles
    • normal ranges vary w/age & race
    • newborns=higher Hct (45-65%) & reticulocyte count=2 to 8%
  3. Hemotocrit
    • 42=females
    • 48=males
    • 45-65=kids
  4. high reticulocyte means what
    more of the factory producing=rbc's
  5. several days after birth RBC production
    decreases & Hgb/Hct levels begin to fall until 2-3mos (Hgb:10 Hct:30)=physiologic anemia of infancy
  6. newborns have a lrg rbc's w/a mean corpuscular vol >94, when does this fall
    6mos
  7. newborns can have an elevated wbc count at birth of what
    9.4 to 30K
  8. how is the wbc count in reference from infant/young child to teens/adults
    relatively higher
  9. what are mostly seen on a smear at birth & in the older child
    neutrophils
  10. what are mostly seen on semar (80%) btwn 1mos & 6yrs
    lymphocytes
  11. what is it when you put blood on a slide & count cells
    smear
  12. platelet count varies from what to what
    150-400K
  13. Increase #wbc's that is 2 standard deviations above the mean count for a particular age
    leukocytosis
  14. what happens if the wbc count is >50,000
    leukemoid reaction which is often seen in sepsis & bacterial
  15. what is defined as an inc in immature cells i:e greater than or equal 5% of immature to mature neutrophil cells
    left shift
  16. what might you see with a left shift
    inc in bands, metamyelocytes & myelocytes
  17. Left shifts may occur in what
    • bacterial inf
    • trauma
    • burns
    • surgery
    • acute hemolysis/hemorrhage=all hands on deck so to speak
  18. Absolute neutrophil count calculated as the wbc count/ul*%neutrophils & bands that is below 2 standard deviations below the normal mean standard for age & race
    neutropenia
  19. hemotological do characterized by an abnormally low # neutrophils
    neutropenia
  20. neutrophil count lower limits
    • white=1500
    • black=1200
  21. it is blank whether example above is netropenic
    tittering
  22. When can neutropenia occur=more common
    extrinsic factors affecting marrow myeloid cells
  23. when can neutropenia occur=less common
    acquired do myeloid & stem cells
  24. when can neutropenia occur=rarely
    intrinsic defect affecting proliferation & maturation of myeolod & stem cells
  25. what are some examples of causes of neutropenia
    • Infections=viral(HIV) Bacterial (sepsis)
    • drug induced-chemo
    • PCN
    • phenothiazines-given toxic amt
    • malignancy-neuroblastoma, rhabdomyosarcoma, ewings sarcoma
  26. Bone marrow failure
    failure to produce an adequated # circulating blood cells that may lead to pancytopenia (dec all types cells) as in aplastic anemia or cytopenia 1 cell line
  27. bone marrow failure causes
    • malignancy
    • drugs & toxins
    • associated=macrocytosis
  28. Hereditary clotting Do's
    Hemophilia VIII=A or IX=B
  29. Most common severe bleeding disorders
    hemophilia VIII or IX
  30. Do the factors VIII or IX cross the placenta
    no they dont
  31. When do the symptoms of hemophilia A or B present typically
    • birth
    • utero
  32. hemophilia typically will present as what
    intracranial bleed so 30 to 50% affected males bleed w/circumcision which may go undiagnosed
  33. when could hemophilia show its nasty self
    • when they begin to crawl or walk
    • sustains the typical minor injuries
  34. Even in severe cases only blank will have evidence of inc bleeding by 1yr
    90%
  35. Hallmark manifestations of hemophilia
    hemarthrosis
  36. hemarthrosis
    blood in ankle joint
  37. recurrent bleeding in same joint
    target joints
  38. where can life threatening bleeds occur into what structures
    • vital such as CNS, upper airway, exsanguitnation
    • external=gi, iliopsoas hemorrhage
  39. patients w/factor VIII or IX usually won't have spontaneous bleeding when
    >5U/dl
  40. patients w/factor VIII or IX may experience prolonged bleeding with what
    • dental work
    • surgery
    • mild-moderation trauma
  41. how do you dx hemophilia
    labs=ptt=affected & maybe 2-3 times normal
  42. What is the tx for hemophilia
    • injury prevention
    • hepb vaccine
    • pyshosocial counseling
    • clotting factor & fluid replacement=severe
    • fibrin glue=circumcision=mom carrier must wait
    • if no child is carrier=be careful w/circum
    • Desmopressin antifibrinolytic=tranexamic acid eposolon aminocarproic acid
  43. most common hereditary bleeding do chromosome 12
    von willebrand disease
  44. Etiology of von willebrand disease
    • varies but some reports suggest 1-2% gen pop affected
    • more women than men
    • vwf protein: 1=red but not absent; 2=qualatively; 3=abscent
  45. Symptoms of von willebran disease
    • mucocutaneous hemorrhage
    • stress
  46. excessive epistaxis, menorrhagia & post op bleeding
    mucocutaneous hemorrhage
  47. what increases the protein factor & therefore may not have significant bleeding w/childbirth & appendectomy
    stress
  48. how do you dx von willebrand disease
    • quanitative assay=VWF antigen, activity
    • determination of VWF structure & platelet count
    • labs will show inc bleeding & ptt
  49. how do you tx von willebrand disease
    • aim is to inc plasma level of VWF & factor VIII
    • DDAVP=desmopressin induces the release of VWF from endothelial cells=milder forms. Otherwise:replacement therapy
    • antifibrinolytic therapy=role too
  50. characterized by ineffective erythropoiesis where there is active erythropoiesis but with premature death of cells, a dec output of RBC from the bone marrow=anemia
    megaloblastic anemias
  51. megaloblastice anemias
    • folic acid def
    • vit B12 def
  52. what are some typical findings w/MA
    • rbc's larger inc mcv & freq oval
    • hypersegmented neutrophils=smear perhaps
    • vit def malnutrition is uncommon in us but problem worldwide
    • not problem in inner city kids b/c of easy access to food so they get their fortified vitamins
  53. Folic acid deficiency definiton
    folates are found in green veggies, fruits, liver & kidney. body stores for a short time. anemia may occur w/in 2-3mos of folate free diet.
  54. Etiology of folic acid def
    inadequate dietary intake, dec absorption, acquired or cognential do of metabolism
  55. Findings associated w/folic acid def
    • VLBW infants, incidence peaks=4 to 7 mos
    • sym=similiar to other anemias w/addition of irritable, poor wt gain, chronic diarrhea. In most sever cases, thrombocytopenia may occur=hemorrhages
  56. how do you dx folic acid def
    labs: consistent w/megaloblastic anemia plus a dec in serum folic acids 3ng/ml. RBC folate levels are more indicative of chronic disease
  57. normal folic acid levels
    5-20 ng/dl
  58. how do you tx folic acid def
    • Folic acid po/IM 0.5 to 1.0mg/day
    • improve=72hrs
    • transfusion=ill or severe
    • long term=MV
  59. Derived from cobalamin in foods (animals) 2nd production by microrganisms. Human digestion=release & absorption of cobalamin
    vb12 def
  60. Etiology of VB12 def
    • inadequated dietary intake or problems w/in dig sys
    • vb12=3 to 5yrs in older child & adult; however if a child is born to mother w/def the baby may show signs of anemia=4 to 5mos
  61. what are the findings w/vb12 def
    • weakness
    • fatigue
    • FTT
    • irritability
    • palor
    • glossitis
    • V/D & jaundide
  62. What are the neuro findings w/vb12 def
    • paresthesias
    • sens deficits
    • hypotonia
    • seizures
    • development delay
    • neuropyshciatric changes
  63. can neuro changes occur w/out hematologic abnormalities
    yes they can
  64. how do you dx vb12 def
    • labs; consistent w/MA
    • serum vb12<100pg/ml
    • LDH=inc
    • bilirubin=inc
    • inc methylamalonic acid=urine
    • schilling test
  65. how do you tx folic acid def
    parenteral vb12 & oral, nasal formulations=wait to stabilized
  66. Hemolytic disease of the newborn
    • abo incompatibility
    • rh isoimmunization
  67. most common cause of hemolytic dis in NB
    ABO incompatibility
  68. Definition of ABO
    most common cause of HDNB, 20-25% preg, only 3rd coomb's pos & only 20% of these develop exc jaundice/hemolytic anemia
  69. Etiology of ABO incompatibiltiy
    • MOM is O & baby is either A or B however if mom=A she may have AB's against B. same principle is true if mom=B
    • Mild & present=jaundice
    • Also=kernicterus or hydrops fetalis but rare
  70. how do you tx ABO incompatibility
    • phototherapy
    • exchange transfusion
  71. a much less common but can be more severe, severity inc w/each preg, inc abortion-spontaneous or induced
    RH isoimmunization
  72. Etiology of Rh isoimmunization
    • 90%=D antigen
    • M=Rh neg & baby needs to be Rh pos
    • as the baby's antigen enters the maternal circ, maternal IgM Ab's formed=form IgG Ab's
    • w/exp/sens IGM, igg ab formation inc, maternal igg ab's can cross placenta=hemolytic disease
  73. Findings w/Rh isoimmunization
    1st preg rarely results in hemolytic disease as sensitization has not recoccured at this time. In severe cases, this may lead to erythoblastotosis fetalis or in less severe cases, hyperbilirubinemai & anemia
  74. how do you tx Rh isoimmunization=neonatal
    • phototherapy
    • exchange transfusions
  75. how do you tx rh isoimmunization=maternal
    • rhogam-28 to 32 wks & birth again
    • risk of initial sensitization Rh neg mothers has dropped from 10-20% to less than 1% w/rhogam.

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