Anatomy%20and%20Physiology%20of%20Pediatric%20Differences

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Hematologic disorders in childhood -Chapter 15
P513

• Anatomy and Physiology of Pediatric Differences
• Anemias
• Iron Deficiency Anemia P516
• G-6-PD
• ß-thalassemia P526
• Clotting Disorders
• Hemophilia P528
• Idiopathic thrombocytopenic Purpura P532

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Anatomy and Physiology of Pediatric Differences

• Blood cell production P515?1?
• RBC2 week of gestation
• WBC platelet8 week of gestation
• Liver ? bone marrow(20-24 week of
  gestation)?marrow of almost every bone(at
  birth)the flat bones(throughout life)
• Normal Blood Values in Children P515 Table 15-1

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Anemias
P516

• Anemia is defined as a reduction in the number of
  red blood cells, the quantity of hemoglobin, the
  volume of packed red cells to below-normal levels

• Causes
• 1. Loss or destruction of existing RBC
• 2. Impaired or decreased rate of RBC production
• 3. Underlying disorder
• Lead poisoning
• Hypersplenism(splenomegaly blood cell
  deficiencies )

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Iron Deficiency Anemia P516

• Etiology and pathophysiology ?1?
• Causes ?2?
• Blood loss
• -Chronic blood loss is always a potential cause
  ?3?
• Internal demands increased
• Adolescents
• Milk baby
• Poor nutritional intake
• High risk ?2?
• Adolescents
• Infants gt6 months
• Pregnant mother whose nutritional status is
  inadequate
• Premature or multiple births
• Had bleeding in the neonatal period?hemophilia or
  parasitic GI illness?menorrhagia?

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Clinical manifestations ?4?

• Pallor, Fatigue, Irritability ?nailbed
  deformities, growth retardation developmental
  delay, tachycardia, systolic heart murmur
• ????

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Diagnostic tests ?5?

• Laboratory studies(Hb, mean corpuscular
  volume-MCV, microscopic analysis, serum
  iron-binding capacity)
• Diet history and analysis

• Treatment P517?2?
• Oral elemental iron preparation
• A diet high in iron
• Identify and treat the cause

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Nursing management

• 9 months adolescence
• Developmental screening test
• Height and weight
• Low-income groups

• Screen

• Causes
• Dietary management
• Oral iron elemental
• Straw
• Side effects(black stools, constipation, and a
  foul aftertaste)

• Education

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Glucose-6-phosphatase dehydrogenase(G-6-PD)

• ?????
• Normocytic anemia P517
• X???????
• ??????????(????????,?????????????)???????
• ???

??????????(2003),??????,????, P96-98?
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Etiology and pathophysiology

• G-6-PD?????????????????
• ???????????????????????
• ??????????????

• ????
• ??
• ?????(???? ????)
• Glutathione(G-SH)
• ??(favabean)
• ????????

10

• ????
• ??
• ??
• ??
• ???

• ????
• G-6-PD????
• Acethylphenyl hydrazine (Heinz bodies)

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• ????
• ??
• ????
• ????

???? (1)??? (2)????????? (3)??????????? (4)G-6-PD
??????
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ß-thalassemia
P526

• Etiology and pathophysiology
• A group of inherited blood disorders of
  hemoglobin synthesis ?1?
• Mediterranean middle Eastern, Asian, and African
  populations ?3?
• Three typesminor or trait, intermedia, major
  ?2?
• Cooleys anemia(most common type)(major)
  ?1??1?
• Defective synthesis of hemoglobin, structurally
  impaired RBC, shortened life span of the RBC

2a?2ß 2a2? ?2??4?
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Clinical manifestations ?6?

• Pallor
• Failure to thrive
• Hepatosplenomegaly
• Severe anemia(Hblt6g/dL)
• ?????????????????????
• ?????
• ??????(hemosiderosis)
• ?????

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Diagnostic tests ?7? Hemoglobin
electrophoresis

• Treatment ?3?
• Supportive
• Blood transfusion (Hbgt10gm/dlHctgt27)
• Iron overload, transfusion reaction and
  alloimmunization(antiboby formation)
• Iron-chelating(Deferoxamine)
• Bone marrow transplantation ( BMT)
• ????

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Nursing management

• Observing for complications of transfusion
  therapy(Table 15-6)
• Providing emotional support
• Genetic counseling

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Clotting Disorders - Defects in hemostasis

• ???????
• Vascular
• Platelet
• Plasma Phase

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Hemophilia P528

• A deficiency of factor VIII, IX
• Hemophilia A
• -classic hemophilia
• -a deficiency of factor VIII
• -80
• Hemophilia B
• -a deficiency of factor IX
• -15

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Etiology and pathophysiology

• X-linked recessive trait
• One-third of hemophiliacs have no family members
  with a history of clotting disorders

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Clinical manifestations

• Bleeding tendencies-may have bleeding after
  circumcision, ecchymosis, nosebleeds, hematuria,
  and bleeding after tooth extraction, minor
  trauma, or minor surgical procedures ?2?
• Spontaneous bleeding, hemarthrosis, deep tissue
  hemorrhage? pain, tenderness, swelling? limited
  motion? bone change, contractures and disabling
  deformities ?1??4?
• Large subcutaneous and intramuscular hemorrhages
• Potential for airway obstruction
• Retroperitoneal and intracranial bleeding
• ????

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Diagnostic tests (clinical therapy??1?)

• Before birth
• Chorionic villus sampling or amniocentesis
• Genetic testing of family members
• History
• Physical examination
• Laboratory data
• Factor VIII, IX?, APPT prolonged
• PT, TT, fibrinogen and platelet count are normal.

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Medical management p529?2?

• Control bleeding-replacement therapy
• Replacing the missing clotting factor
• DDAVP(desmopressin acetate)
• Gene therapy

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Nursing assessment

• Physiologic assessment
• Psychologic assessment
• Development assessment

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Nursing management P530

• Prevent and control bleeding episodes
• Limit joint involvement and manage pain
• Provide emotional support

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Prevent and control bleeding episodes

• Superficial bleeding
• -pressure to the area for at least 15 minutes
• -Immobilize and elevate the affected area
• -Ice packs to promote vasoconstriction
• Significant bleeding
• -Supportive measures
• -Factor replacement therapy

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Limit joint involvement and manage pain

• Elevating and Immobilizing the joint
• Applying ice packs
• Administer analgesics(Scanol?Codeine?Demerol)
• ROM
• Appropriate weight

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Idiopathic thrombocytopenic Purpura P532
ITP????????????

• Etiology and pathophysiology
• Autoimmune thrombocytopenic purpura
• Increased destruction of platelets
• Most frequently in children 210years of age
• ???????(self-limited)??????

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Clinical manifestations

• Multiple ecchymoses and petechiae ?4?
• ????
• ????
• ????

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Diagnostic tests ?4?

• History
• Physical findinfing
• Lab. Finding
• Platelet count and antiplatelet antibodies?
  (Plateletslt20,000/mm3)

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Treatment

• ????
• ????

• ??
• Corticosteroids
• Intravenous immunoglobulins
• Anti-D antibody (Box 35-2)

• ??
• Splenectomy
• Steroids
• ??????
• ????
• Ascorbate
• BT PRBC

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• Progress
• Spontaneous remission 90

• Nursing management
• Control bleeding episodes
• Pain control(acetaminophen)