1 / 41

TOPIK KULIAH BLOK 14

TOPIK KULIAH BLOK 14. Hemolitik Intrakospuskuler G6PD, Sferositosis, Anemia Aplastik Thalasemia, HbPathy Def Zat Besi dan Megaloblastik anemia Leukemia Syok. TOPIK KULIAH BLOK 14. III. ANEMIA Hemolitik Intrakospuskuler G6PD, Sferositosis IV. Anemia Aplastik

lohman
Download Presentation

TOPIK KULIAH BLOK 14

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. TOPIK KULIAH BLOK 14 • Hemolitik Intrakospuskuler G6PD, Sferositosis, Anemia Aplastik • Thalasemia, HbPathy • Def Zat Besi dan Megaloblastik anemia • Leukemia • Syok

  2. TOPIK KULIAH BLOK 14 III. ANEMIA Hemolitik Intrakospuskuler G6PD, Sferositosis IV. Anemia Aplastik II. Anemia Hemolitik: Thalasemia, HbPathy I. ANEMIA NUTRISI: Def Zat Besi dan Megaloblastik anemia V. Leukemia VI. Syok

  3. ANEMIA IN CHILDREN Sumadiono Dept. of Pediatric Fac. Of Medicine GMU Yogyakarta

  4. BLOOD • Plasma • RBC • WBC • Platelets 1 Blood flows.flv

  5. Plasma consists of: • 90% water • 10 % solutes: albumin, globulin, clotting factors, antibodies, electrolytes

  6. RBC’s RBC’s  delivering oxygen Life cycle 120 days 1 Oksigenasi.flv

  7. ANEMIADefinition Reduction in blood Hb concentration WHO: - 6 month – 6 year: > 11 g% - > 6 year: > 12 g%

  8. Causes of ANEMIA CLASSIFICATION Two broad categories Production Blood loss • Bleeding: • Acute • Chronic • Hemolytic: • - Thallasemia • AIHA (Autoimmune • Hemolytic anemia) • G6PD Def. • Sickle cell, Spherocytocis • Bone Marrow: • Aplastic Anemia • Leukemia • Nutrition: • - IDA (Iron Def. Anemia) • Folate def. • B12 def.

  9. Symptoms and Signs of Anemia General Specific • Paloor • Fatique • Low concentration • Tahycardia • Respiratory rate >> • Apetite << • Growth << Tissue Hypoxia • Depend to the CAUSES

  10. IRONDEFECIENCY ANEMIA ANEMIA DEFESIENSI ZAT BESI (ADB) (IDA): Most common anemia In 1-3 year olds  50% of children in developing countries

  11. Causes of IDA 1. Iron intake << Poor dietary sources of iron 2. Rapid growth (infancy, adolescence, pregnancy) 3. Iron stores << - Premature, - Low birth weight, - Gemelli - Ante partum bleeding 4. Occult blood loss - Ankylostomiasis 5. Malabsorption

  12. Symptoms Associated with low oxygenation of tissue: Pallor Fatigue Shortness or breath Irritability Intolerance of physical work / exercise PICA

  13. Signs Conjunctiva: Pallor Koilonychia Angular Stomatitis Glossitis

  14. Laboratory Haemoglobin: low Serum iron (low) Serum ferritin (low) TIBC: Increase

  15. Blood smear Microcytic Low MCV Fe is needed for production If decreased:  small cell Hypochromic Low MCH, Low MCHC Normal

  16. Management Treat the etiology Iron supplementation: re-check retic & Hgb: in 1 week  until Hgb normal + 1-2 months Nutritional: Infants 12 months /> intake of milk << + Solid foods Children: meat, fish, poultry, fruit, green vegetables Transfusion: Decomp.Cordis

  17. Folic acid & B12 is needed for Mitosis if decreased:  large cell Folic Acid/B12 • Megaloblastic Defeciency Hypersegmentation • Teatment • Folic Acid fisrt

  18. APLASTIC ANEMIA Causes • Acquired or inherited • Production of blood cells:absent or decreased. • Decrease in: - RBC’s, WBC’s, Platelets. • Exposure to: • - drugs • - chemicals • - toxins • Infection • Idiopathic

  19. APLASTIC ANEMIA • Pancytopenia • Anemia • Leucopenia • Thrombhocytopenia Pallor Fever Bleeding/Petechie • Reticulocyte low • Without Organomegaly

  20. Bone marrow: Hypoplasia Fat cell >>

  21. 2 CHild BMP.flv Management • Immunosuppressive therapy • Antithymocyte globulin • Bone Marrow Transplant

  22. HEMOLYTICANEMIA

  23. HEMOLYTIC ANEMIA Classification: • Inherited • Defects in the red cell • membrane • - Spherocytosis • - Elliptocytosis • - Sickle cell • Defects of red cell metabolism • - G6PD def. • - Pyruvatekinase Def. • Hb Disorders • - Thalassemias • Acquired • Immune-mediated • Auto-immune hemolytic anemia • Non-Immune • Infection • Drug-induced Providing

  24. Signs-Symptoms & Lab. of Hemolytic Anemia • LABORATORY • ANEMIA, NORMOCYTIC • RETICULO-CYTOSIS • ERYTHROBLAST • BILIRUBIN > • Pallor/ Anemia • Icterus • SPLENOMEGALY

  25. Spherocytosis The RBCs: trapped in the splenic sinuses and cords  destroyed & removed Diagnosis - Anemia, splenomegaly, icterus - Spherocyte cell, bilirubin - Osmotic fragility test + Treatment: Splenectomy

  26. Spherocytosis

  27. >> in males • Hemolysis: episodic •  following to drugs, infection or ingestion of fava beans • The important drugs: • - anti-malarials • - sulfonamides • - some antipyretics/analgesics • - vitamin K, nalidixic acid Glucose-6-Phosphate Dehydrogenase Deficiency

  28. G6PD Deficiency Contd. • Diagnosis • Hemolysis with a triggering agent • Qualitative and quantitative measurement of G6PD • Management • Supportive care • Blood transfusion • Education to prevent further episodes.

  29. Auto-immune Hemolytic Anemia (AIHA) Antibodies to the RBC antigens (autoantibodies). • Warm autoimmune hemolytic anemia: IgG: optimal at 370 C • Cold autoimmune hemolytic anemia: IgM: optimally below 370 C

  30. The direct & Indirect Coombs test Lab. • Glucocorticoids • Splenectomy • Immunosuppressive drugs. Treatment

  31. Thalassemias Autosomal recessive Mediterranean, Middle East, India, South East Asia and Africa Decreased or absent synthesis of globin chains

  32. Haemoglobin Production • HbA (Adult): α22 • HbF (Fetal): α2Gamma2 • HbA2 (Adult): α2Delta2

  33. Haemoglobinopaty

  34. Beta Thalassemia Major The first signs & symptoms: first year of life pallor poor feeding failure to thrive hepatosplenomegaly skull bossing in the older child Icterus

  35. Laboratory Anemia Hypochromia & microcytosis Aniso-poikilocytosis Nucleated RBCs Bilirubin >> Hb electrophoresis Hb F >>

  36. RBC Characteristics • Microcytosis = small in size • Hypochromia = decrease hemoglobin • Poikilocytosis = abnormal shape

  37. Management • Transfusion: PRC • Splenectomy • for hypersplenism , increased blood transfusion • Iron chelation therapy • with desferrioxamine: SC, IV • Stem cell transplantation

  38. Bone Marrow Aspiration 2 CHild BMP.flv

  39. T H A N K Y O U

More Related