Download Anatomy and Physiology of Pediatric Differences

Hematologic disorders in childhood －Chapter 15
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
P513
Anatomy and Physiology of Pediatric
Differences
Anemias
 Iron Deficiency Anemia P516
 G-6-PD
 β-thalassemia P526

Clotting Disorders
 Hemophilia P528
 Idiopathic thrombocytopenic Purpura P532
Anatomy and Physiology of Pediatric Differences
• Blood cell production P515第1段
– RBC：2 week of gestation
– WBC & platelet：8 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
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 )
Iron Deficiency Anemia
 Etiology and pathophysiology
 Causes 第2行
P516
第1段
 Blood loss
－Chronic blood loss is always a potential cause 第3段
 Internal demands increased
 Adolescents
 Milk baby
 Poor nutritional intake
 High risk 第2段
 Adolescents
 Infants >6 months
 Pregnant mother whose nutritional status is inadequate
 Premature or multiple births
 Had bleeding in the neonatal period、hemophilia or parasitic
GI illness、menorrhagia。
Clinical manifestations
第4段
 Pallor, Fatigue, Irritability
→nailbed deformities, growth
retardation & developmental delay,
tachycardia, systolic heart murmur
 腦部損傷
Diagnostic tests 第5段
 Laboratory studies（Hb, mean
corpuscular volume－MCV,
microscopic analysis, serum ironbinding capacity）
 Diet history and analysis
Treatment P517第2段
Oral elemental iron preparation
A diet high in iron
Identify and treat the cause
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
Glucose-6-phosphatase dehydrogenase（G-6-PD）
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俗稱蠶豆症
Normocytic anemia P517
X染色體性聯遺傳
地中海沿海居民、亞洲(中國以廣東人居多，中
東地區如:猶太人、阿拉伯人)及非洲案例較多
 客家人
資料來源：陳月枝等合著（2003），實用兒科護理，台北：匯華， P96-98。
Etiology and pathophysiology
 G-6-PD為紅血球分解代謝葡萄糖的一種酵素。
 主要功能是維持紅血球的正常代謝及球膜的完整性。
 分子結構變異造成溶血現象。
誘發因子
感染
氧化性藥物(解熱劑、 抗生素等)
Glutathione(G-SH)
蠶豆(favabean)
吸入樟腦丸之蒸氣
臨床表徵
 貧血
 黃疸
 血尿
 脾腫大
診斷檢查
G-6-PD活性試驗
Acethylphenyl
hydrazine
(Heinz bodies)
醫療措施
預防
症狀治療
輸血治療
護理措施:
（1）家族史
（2）教導照顧者預防措施
（3）教導照顧者觀察溶血症狀
（4）G-6-PD缺乏症備忘卡
β-thalassemia
P526
Etiology and pathophysiology
 A group of inherited blood disorders of hemoglobin
synthesis 第1行
 Mediterranean; middle Eastern, Asian, and African
populations 第3行
 Three types：minor or trait, intermedia, major 第2段
 Cooley’s anemia（most common type）（major） 第
1段第1行
 Defective synthesis of hemoglobin, structurally
impaired RBC, shortened life span of the RBC
2α2β
2α＋2γ
第2段第4行
Clinical manifestations
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第6行
Pallor
Failure to thrive
Hepatosplenomegaly
Severe anemia（Hb＜6g/dL）
骨髓腔擴大→顱骨變大、顴骨隆起、
塌鼻及頭大
 自發性骨折
 血鐵質沈著症（hemosiderosis）
 黃銅色皮膚
Diagnostic tests
第7行
Hemoglobin electrophoresis
Treatment
第3段
Supportive
Blood transfusion （Hb>10gm/dl；Hct>27%）
 Iron overload, transfusion reaction and
alloimmunization（antiboby formation）
Iron-chelating（Deferoxamine）
Bone marrow transplantation （ BMT）
脾臟切除
Nursing management
Observing for complications of
transfusion therapy（Table 15-6）
Providing emotional support
Genetic counseling
Clotting Disorders － Defects in hemostasis
血液凝固三階段
• Vascular
• Platelet
• Plasma Phase
Hemophilia
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A deficiency of factor VIII, IX
Hemophilia A
－classic hemophilia
－a deficiency of factor VIII
－80％
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P528
Hemophilia B
－a deficiency of factor IX
－15％
Etiology and pathophysiology
• X-linked recessive trait
• One-third of hemophiliacs have no family
members with a history of clotting disorders
Clinical manifestations
 Bleeding tendencies－may have bleeding after
circumcision, ecchymosis, nosebleeds, hematuria, and
bleeding after tooth extraction, minor trauma, or minor
surgical procedures 第2段
 Spontaneous bleeding, hemarthrosis, deep tissue
hemorrhage pain, tenderness, swelling limited
motion bone change, contractures and disabling
deformities 第1段第4行
 Large subcutaneous and intramuscular hemorrhages
 Potential for airway obstruction
 Retroperitoneal and intracranial bleeding
 脊髓血腫
Diagnostic tests
（clinical therapy之第1段）
 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.
Medical management
p529第2段
• Control bleeding－replacement therapy
– Replacing the missing clotting factor
• DDAVP（desmopressin acetate）
• Gene therapy
Nursing assessment
• Physiologic assessment
• Psychologic assessment
• Development assessment
Nursing management P530
• Prevent and control bleeding episodes
• Limit joint involvement and manage pain
• Provide emotional support
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
Limit joint involvement and manage pain
 Elevating and Immobilizing the joint
 Applying ice packs
 Administer analgesics（Scanol、
Codeine、Demerol）
 ROM
 Appropriate weight
Idiopathic thrombocytopenic Purpura
P532
ITP；原發性血小板減少性紫斑症
Etiology and pathophysiology
Autoimmune thrombocytopenic purpura
Increased destruction of platelets
Most frequently in children 2~10years of age
分為急性自限性（self-limited）及慢性緩解性
Clinical manifestations
Multiple ecchymoses and petechiae
容易瘀傷
黏膜出血
下肢血腫
第4行
Diagnostic tests
第4行
 History
 Physical findinfing
 Lab. Finding
 Platelet count and antiplatelet antibodies
（Platelets<20,000/mm3）
Treatment
 支持療法
 限制活動
急性
Corticosteroids
Intravenous immunoglobulins
Anti-D antibody (Box 35-2)
慢性
Splenectomy
Steroids
免疫抑制療法
γ球蛋白
Ascorbate
BT PRBC
Progress
Spontaneous remission: 90％
Nursing management
Control bleeding episodes
Pain control（acetaminophen）