1. Hematology 2
  2. Basic Iron Metabolism
    • fetal life
    • birth
    • early wks life
    • 2mos of life
    • premature infants
    • Reminder
  3. Fetal Life:
    transplancental transfer against conc grad, mostly during 3rd trimester.
  4. Birth:
    • typically polycythemic w/total body iron=circ RBC�s
    • Above 65 count=could be due to positon
  5. Early Weeks of Life:
    dec in hematocrit b/c fetal rbc�s short live & little erythropoesis occurs. Recovered iron stored bone marrow
  6. Two Months of Life:
    erythropoesis inc & uses the stored iron in the form new rbc�s. initial iron stores will last until the child is 2.5birth wt. child must take in iron.
  7. Premature Infants:
    become iron def sooner=smaller rbc mass & faster growth rate
  8. Reminder:
    early life, iron loss=minimal
  9. Iron Requirements (Elemental)
    • Full term infants need approximately 1mg/kg/day
    • Premature infants need approximately 2mg/kg/day=b4 37wks
  10. Breast fed infants do not need supplemental iron while the child is exclusively breast fed!
    Add Iron between 4 and 6 months=ferisol drops
  11. If iron deficient:Premature infant:
    2-4mg/kg/day after 2 mos
  12. If iron deficient:Full term infant:
    1-2mg/kg/day after 2 mos
  13. If iron def max dose
    • is 15mg of elemental iron in 24 hrs
    • Children:3-6mg/kg/day
  14. Anemias
    • Iron deficiency=most common
    • beta-thalassemia
    • hereditary spherocytosis (congenital hemolytic)
    • sickel cell
  15. Iron deficiency definition
    hypochromic, microcytic
  16. Etiology/Implications:iron deficiency
    hematologic, gi, cns ( dec conc, irritability), musc growth
  17. Pertinent Historical Findings/Clinical manifestations:Iron def
    asymptomoatic, pallor, fatigue, irritable, dec musc tone, mot development delay & pagophagia (chewing on ice)
  18. Diagnostic Evaulations: Iron Def
    Labs: hypochromic, microcytic RBCs, low MCV, low MCH, low serum ferritin, low serum iron, low transferrin saturation, normal to higher reticulocyte count.
  19. Differential Diagnosis:Iron Def
    othalassemia, lead poisoning, & chronic inflammation or infection, iron def
  20. Medical Management:Iron def
    Treatment: iron supplem
  21. Thalassemia (congenital hemolytic anemia)=Alpha-Thalassemia:definiton
    microcytic, hypochromic anemia of variable severity=chromosome 16
  22. Etiology/Pathogenesis:Thalassemia
    inc severity w/each deletion of the four alpha globin genes
  23. Pertinent Historical Findings/clinical symptoms:Thalassemia
    • 1 deletion: no symptoms,
    • 2 deletions: mild anemia,
    • 3 deletions: hemolytic anemia,
    • 4 deletions: stillborn � hydrops fetalis
  24. Medical Management=Thalassemia
    • in more sever ecases folic acid is rec & transfusion may be needed
    • TIP: iron supplementation is useless for thalassemia
  25. Beta �Thalassemia:Definition=Minor:
    mild microcytic, hypochromic anemia, unresp to iron tx, elevated hemoglobin A2, asymptomatic=chromosome 11
  26. Beta-thalassemia:Definition=Major
    mod to severe microcytic, hypochromic anemia developoing in 1st yr life as fetal hgb production dec, elevated fetal & hgb A2, HSM, skeletal abnormalities.
  27. Medical Management:Beta Thalassemia
    • bone marrow transplantation
    • chronic transfusion with iron chelation to prevent iron from getting to high
    • Emerging treatments: pharmacologic treatment to manipulate fetal hemoglobin & gene therapy
    • Note: if not treated, most children will die w/in the 1st decade of life. It is the most common cause of transfusion dependent anemia in childhood.
  28. Hereditary Spherocytosis (congenital hemolytic anemia):Definition:
    normocytic, hyperchromic anemia w/spherocytosis & inc reticulocytes
  29. Etiology/Background:Hereditary spherocytosis
    oprimarily autosomal dominant with a partial deficiency of spectrin, a protein of the red cell membrane=common in European descent
  30. Pertinent historical findings/Clinical manifestations:Hereditary spherocytosis
    anemia, jaundice & splenomegaly. Other: gallstones, weakness, fever, abdominal pain & CHF.
  31. Differential Diagnosis:hereditary spherocytosis
    • ABO, or Rh incompatibility,
    • autoimmune hemolytic anemaia
  32. Medical Management:hereditary spherocytosis
    • folic acid,
    • transfusions,
    • splenectomy
  33. Sickle Cell Anemia (congential hemolytic anemia)Definition:
    deoxygenation leads to polymerization of hemoglobin into long rods that deform the cell into sickle shape.
  34. Sickle cell anemia: Etiology/Pathogenesis
    • This leads to a decrease in RBC life span, increase in blood viscosity and may lead to vaso-occlusion.
    • Hypoxia, dehydration, hypertonicity all predispose
    • Mutation/deletion of hemoglobin gene. Globin genes typically cluster at chromosome 11( Beta-globin) and Chromosome 16 (alpha-globin) HbAS � Trait; HbAC � mild anemia, HbSS or HbS � disease
  35. Sickel Cell Anemia Background:
    African, Mediterranean, Middle Eastern or Indian. 1/400 African-American infants are affected. Eight percent of all African-Americans are considered to have the trait.
  36. Sickle cell anemia Pertinent Historical Findings/Clinical Manifestations
    • normal birth wt until 3-4mos of life due to high levels of fetal hgb.
    • Anemia=present by 1yr.
    • most clinical problems are 2nd to chronic hemolytic anemia or the vaso-occlusion.
  37. Central Nervous System:findings w/SCA
    CVA (dizz, faint, headaches may be warn signs.strokes have recurrent problems=silent infarctio
  38. Cardiac:findings w/SCA
    cardiomegaly-systolic ejection, murmur, CHF
  39. pulmonary: findings w/SCA
    pneumonia, pulm hpt & infarction (acute chest syndrome)=sickle cell crisis
  40. GI: findings w/sca
    hepatomegaly, inc LFT�s, jaundice, gallstones
  41. GU: findings w/sca
    hypostheuria, polyuria, hematuria, salt losing nephropathy, nephritic syndrome, UTI�s priapism (prolonged erection)
  42. Ortho: findings w/sca
    avascualr necrosis of femoral head, osteomyelitis=salmonella
  43. Derm: SCA findings
    skin ulcers of low extr=teens typically
  44. Immune: SCA findings
    pneumococcus, haemophilus influenzae, and encapsulated organisms are ususally responsible. Bacterial sepsis and meningitis are the most life threatening.
  45. Crises: findings w/sca
    • vasoocclusive
    • dactylitis
    • acute chest syndrome
    • CVA
    • acute splenic sequestration
    • aplastic

    • Vaso-occlusive �
    • painful, transient, ischemic tissue damage which may be triggered by dehydration, infection, stress
  46. Dactylitis (hand-foot syndrome) �
    often 1st sign, fever may be present, Abdominal and musculoskeletal pain are also common=occurs in 50% in age of children b4=3yo
  47. Acute chest syndrome, CVA
  48. Acute Splenic Sequestration �
    massive enlargement of spleen due to pooling of rbc�s rapid drop hgb/hct=shock or death. Seen in infants & young children following febrile illness.
  49. Aplastic �
    sudden shutdown of bone marrow & drop in hct. May be life threating & follows parvovirus b19 infection.
  50. Diagnostic Evaluations:SCA
    • Labs: normocytic or macrocytic anemia, increased platelets, reticulocytes. Other findings: sickle cells, Howell-Jolly bodies, target cells
    • Dx: hemoglobin electrophoresis
  51. Medical Management:SCa
    Treatment a. Health Maintenance: nutrition, baseline lab studies, immunizations, education & referreals=folic acid 1mgperday, pneumonocal, infleuza vaccines; delayed puberty, short stature & recognize disease sx=fever & pallor. Opthalomogy for retinopathy, echo for tricuspid regurg; nephrology based upon renal fx tests & pulmonalgy for tests
  52. Infections: prevention & early recognition (prophylactic abx)=3mo�s to 2yo they should receive pencillin=125mg ; 2-5 should be 250mg.
  53. Pain: analgesics, hydration, oxygen, abx, transfusion? Problem at capillary level
  54. Transfusion therapy: acute splenic sequestration, aplastic
  55. Other: hydroxyurea (inc fetal hgb), bone marrow transplantation, chemotherapy
  56. Upcoming:SCA
    • Gene Therapy
    • Increasing expression of Hb F
    • RNA repair
  57. Transient Erythroblastopenia of Childhood=Definition:
    thought to be an autoimmunce disorder resulting in a normocytic anemia w/reticulowyctopenia
  58. Transient Erythroblastopenia:Etiology/Background:
    fairly common affecting children=6mos to 4yo
  59. transient erythroblastopenia:Pertinent Historical Findings/Clinical Manifestations:
    pallor or incidental finding (draw from for another reason & something else comes back)
  60. transient erythroblastopenia:Differential Diagnosis:
    Diamond-Blackfan, chronic disorders like hypothyroidism, renal failure, malignancy
  61. transient erythroblastopenia=Medical Management
    Treatment: self-limiting=4-8wks after dx
  62. Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD)=definition
    osporadic hemolysis assocatied w/infection or ingestion of fava beans or oxidant drugs (aspirin, sulfa, antimalarials)=always comes back****
  63. Glucose6phosphate dehydrogenase def= Etiology/Background:
    most common red cell enzyme disorder resulting in hemolysis. X-linked recessive in Africans, Mediterraneans and Asians
  64. G6Dehy=Pertinent Historical Findings/Clinical Manifestations:
    hyperbilirubinemia, hemolytic episodes-pallor, jaundice, hemoglobinuria & can be life threatening
  65. G6Deh=Diagnostic Evualations
    Labs:normal initially, hemolysis -> fall Hgb/Hct, Heinz bodies, reticulocytosis, decrease in G6PD activity in erythrocytes, hemogloniuria
  66. G6Deh=Medical Management
    • Treatment: prevention: gene therapy trials underway
    • Other: Folic Acid, Transfusion of packed RBCs if hemolysis occurs
  67. Idiopathic Thrombocytopenia Purpura=Definition
    dec in platelets, acute vs chronic=ITP
  68. Idiopathic thrombocytopenia=Etiology/Background:
    most common bleeding do in children- 2 to 5 yo & often follows viral infection=varicella, rubella, measles, Epstein barr (mono). Spleen forms antibodies which results in thrombocytopenia
  69. Idiopathic thrombocytopenia=Pertinent Historical Findings/Clinical Manifestations:
    otherwise healthy child w/petechia, ecchymosis & epistaxis.
  70. Idiopathich thrombo=Complications
    hemorrhage into vital organs esp intracranial=major worry
  71. Idiopathic thrombo=Diagnostic Evulations
    Labs: drop in platelets, platelets are often larger, other tests normal unless hemolyisis has occured
  72. Idiopathci thrombo=Medical Management:
    • 1. General � avoid trauma, stop aspirin products, spontaneous recovery w/in 6mos (70 to 80% in acute form)
    • 2. Medications � Corticosteroids, Immune Globulin Intravenous, Rho(D) Immune Globulin, platelet transfusion may be indicated
    • 3. Surgical: splenectomy only in severe chronic form (pharm support 12mos after dx) or life-threating hemorrhagic complications.
  73. Plumbism � Lead Poisoning=definition
    an elevated serum lead level greater than 9mcg/dl; less than or equal 5yo=still developmenting neurologically which impacts
  74. plumbism=Etiology/Background:
    ingestion occurs typically from environmental exposures such as lead-based paint, renovations, nearby industry, food cans (ethnic), leaded crystal, water pipes, certain insecticides
  75. plumbism=Pertinent Historical Findings/Clinical Manifestations:
    • 1. Most are asymptomatic
    • 2. GI �anorexia, constipation, abd pain, vomiting, diarrhea, FTT, pica (eating non-food)
    • 3. Neuro � irritability (school performance), overactivity, lethargy, ataxia, encephalopathy, coma & death=levels typically over 100
    • 4. Heme � microcytic anemia, increased free erythrocyte protoporphyrin (FEP) or zinc protoporphyrin
    • 5. Skeletal � lead lines on radiographs of long bones
  76. plumbisim= Prevention
    • 1. Screening � all children below 6 yo should be screened at least one time. Screening should begin at 9-12 mos and be more regimented if child is high risk. Capillary (finger) vs. Venous blood (might have to stick twice). Below 9 for cap=nothing above 9 confirm w/venous
    • 2. Nutrition �balanced diet, foods rich in iron, VC, Ca2+, low fat
    • 3. Hygiene � reg hand washing, hand to mouth activities, clean the toys & stuff animals
    • 4. House Cleaning �damp mopping w/high phosphate cleaner
    • 5. Abatement=city or state helps to mandate the lead in the home.
  77. plumbism=Medical Management Intervention/Treatment
    • 1. Based on classification of VENOUS lead
    • 2. Fe supplementation
    • 3. Chelation � oral/IV/IM usually indicated for venous lead greater 45mcg/dl=greater than 70 do in hosp setting; encepatholopathy if suspect lead poising.
    • 4. New housing is needed
    • 5. Intensive follow-up w/specialist
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