Anatomy and Physiology of Pediatric Differences Anemias Iron Deficiency Anemia P516 G-6-PD

Hematologic disorders in childhood－Chapter 15 P513 • Anatomy and Physiology of Pediatric Differences • Anemias • Iron Deficiency AnemiaP516 • G-6-PD • β-thalassemiaP526 • Clotting Disorders • HemophiliaP528 • Idiopathic thrombocytopenic PurpuraP532

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 P516 • Etiology and pathophysiology第1段 • Causes 第2行 • 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 iron-binding capacity） • Diet history and analysis TreatmentP517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） • 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2β2α＋2γ 第2段第4行

Clinical manifestations 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 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％

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 plateletcount 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 managementP530 • 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第4行 • 容易瘀傷 • 黏膜出血 • 下肢血腫

History Physical findinfing Lab. Finding Platelet count and antiplatelet antibodies（Platelets<20,000/mm3） Diagnostic tests第4行

支持療法 限制活動 Treatment • 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）

Anatomy and Physiology of Pediatric Differences Anemias Iron Deficiency Anemia P516 G-6-PD