ANEMIA IN CHILDREN Sumadiono Dept of Pediatric Fac

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

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

BLOOD a m s la P C B R C B W s t

BLOOD a m s la P C B R C B W s t e l late P 1 Blood flows. flv

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

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

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

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

ANEMIA Definition Reduction in blood Hb concentration WHO: - 6 month – 6 year:

ANEMIA Definition Reduction in blood Hb concentration WHO: - 6 month – 6 year: > 11 g% - > 6 year: > 12 g%

Causes of ANEMIA CLASSIFICATION Production Two broad categories Bone Marrow: - Aplastic Anemia -

Causes of ANEMIA CLASSIFICATION Production Two broad categories Bone Marrow: - Aplastic Anemia - Leukemia Nutrition: - IDA (Iron Def. Anemia) -Folate def. - B 12 def. Blood loss Bleeding: - Acute - Chronic Hemolytic: - Thallasemia - AIHA (Autoimmune Hemolytic anemia) - G 6 PD Def. - Sickle cell, Spherocytocis

Symptoms and Signs of Anemia General Tissue Hypoxia Paloor Fatique Low concentration Specific Tahycardia

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

(IDA): ANEMIA DEFESIENSI ZAT BESI (ADB) Most common anemia In 1 -3 year olds

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

Causes of IDA 1. Iron intake << Poor dietary sources of iron 2. Rapid

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

Symptoms Associated with low oxygenation of tissue: Pallor PICA Fatigue Shortness or breath Irritability

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

Signs Koilonychia Conjunctiva: Pallor Angular Stomatitis Glossitis

Signs Koilonychia Conjunctiva: Pallor Angular Stomatitis Glossitis

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

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

Blood smear Fe is needed for production If decreased: small cell Microcytic Low MCV

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

Management Treat the etiology Iron supplementation: · re-check retic & Hgb: in 1 week

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

Folic acid & B 12 is needed for Mitosis if decreased: large cell Megaloblastic

Folic acid & B 12 is needed for Mitosis if decreased: large cell Megaloblastic Hypersegmentation Teatment Folic Acid fisrt

 Acquired or inherited Production of blood cells: absent or decreased. Causes Exposure to:

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

 Pancytopenia Anemia Fever Leucopenia Thrombhocytopenia Reticulocyte low Without Organomegaly Pallor Bleeding/ Petechie

Pancytopenia Anemia Fever Leucopenia Thrombhocytopenia Reticulocyte low Without Organomegaly Pallor Bleeding/ Petechie

Bone marrow: Hypoplasia Fat cell >>

Bone marrow: Hypoplasia Fat cell >>

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

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

Inherited Defects in the red cell Providing membrane - Spherocytosis - Elliptocytosis - Sickle

Inherited Defects in the red cell Providing membrane - Spherocytosis - Elliptocytosis - Sickle cell Defects of red cell metabolism - G 6 PD def. - Pyruvate kinase Def. Hb Disorders - Thalassemias Acquired Immune-mediated Auto-immune hemolytic anemia Non-Immune Infection Drug-induced

Signs-Symptoms & Lab. of Hemolytic Anemia LABORATORY ANEMIA, NORMOCYTIC v Pallor/ v Anemia Icterus

Signs-Symptoms & Lab. of Hemolytic Anemia LABORATORY ANEMIA, NORMOCYTIC v Pallor/ v Anemia Icterus RETICULOCYTOSIS ERYTHROBLAST v SPLENOMEGALY BILIRUBIN > 2000 -2001 22

Spherocytosis · The RBCs: trapped in the splenic sinuses and cords destroyed & removed

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

Spherocytosis

Spherocytosis

Glucose-6 -Phosphate Dehydrogenase Deficiency · >> in males · Hemolysis: episodic following to drugs,

Glucose-6 -Phosphate Dehydrogenase Deficiency · >> 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

G 6 PD Deficiency Contd. · Diagnosis ·Hemolysis · Qualitative with a triggering agent

G 6 PD Deficiency Contd. · Diagnosis ·Hemolysis · Qualitative with a triggering agent and quantitative measurement of G 6 PD · Management · Supportive care · Blood transfusion ·Education to prevent further episodes.

Auto-immune Hemolytic Anemia (AIHA) Antibodies to the RBC antigens (autoantibodies). - Warm autoimmune hemolytic

Auto-immune Hemolytic Anemia (AIHA) Antibodies to the RBC antigens (autoantibodies). - Warm autoimmune hemolytic anemia: Ig. G: optimal at 370 C - Cold autoimmune hemolytic anemia: Ig. M: optimally below 370 C

. b La The direct & Indirect Coombs test t n e tm Glucocorticoids

. b La The direct & Indirect Coombs test t n e tm Glucocorticoids a e r T Splenectomy Immunosuppressive drugs.

Thalassemias Decreased or absent synthesis of globin chains · Autosomal recessive · Mediterranean, Middle

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

Haemoglobin Production Hb. A (Adult): α 2 2 Hb. F (Fetal): α 2 Gamma

Haemoglobin Production Hb. A (Adult): α 2 2 Hb. F (Fetal): α 2 Gamma 2 Hb. A 2 (Adult): α 2 Delta 2 2000 -2001 30

Haemoglobinopaty 2000 -2001 31

Haemoglobinopaty 2000 -2001 31

Beta Thalassemia Major The first signs & symptoms: first year of life ·pallor ·poor

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

2000 -2001 33

2000 -2001 33

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

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

RBC Characteristics Microcytosis = small in size Hypochromia = decrease hemoglobin Poikilocytosis = abnormal

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

2000 -2001 36

2000 -2001 36

Management · Transfusion: PRC · Splenectomy · for · Iron hypersplenism , increased blood

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

Bone Marrow Aspiration 2 CHild BMP. flv

Bone Marrow Aspiration 2 CHild BMP. flv

T H A N K Y O U

T H A N K Y O U