Anemia in Children PDF

} Anemia is a significant global health problem affecting children } Definition : - Reduction of the hemoglobin concentration, hematocrit or RBC volume below normal values. - Normal values vary with age and sex (according to following table) } Clinical findings generally...

} Anemia is a significant global health problem affecting children } Definition : - Reduction of the hemoglobin concentration, hematocrit or RBC volume below normal values. - Normal values vary with age and sex (according to following table) } Clinical findings generally do not become apparent until the hemoglobin level falls to < 8 g/dL } Weakness , Sleepiness , irritability } Decreased exercise tolerance } Exertional dyspnea } Pallor ; can involve tongue , nail beds , conjunctiva , palms } A hemic murmur is often present. } Tachypnea , tachycardia } cardiac dilation and high-output heart failure in severe anemia o initial laboratory testing should include hemoglobin, hematocrit, and RBC indices as well as a white blood cell (WBC) count and differential, platelet count, reticulocyte count, and peripheral blood smear. 1. According to RBC size , (mean corpuscular volume (MCV) and microscopic appearance 2. According to underlying physiology.  According to RBC size , (mean corpuscular volume (MCV) and microscopic appearance: } Microcytic : iron deficiency anemia , thalassemia } Normocytic : hemolytic anemia's , chronic disease , acute hemorrhage , malignancy. } Macrocytic : folic acid and vitamin B12 deficiency , aplastic anemia  According to underlying physiology into : - Decreased production : BM failure or erythropoiesis defects as congenital pure red aplasia. - Increased destruction or loss : hemolytic anemia or hemorrhage. - A progressive decrease in Hb level due to decreased erythropoietin level. - Begins within the 1st wk of life and persists for 6-8 wk..may extend till 3 months - Hb levels : decrease down to (9–11 g/dL) in full-term neonates and - in preterm infants may reach 7–9 g/dL and presented earlier at 3–6 weeks. } Treatment : - In full-term infant : no therapy required , just ensuring that the infant’s diet contains essential nutrients for normal erythropoiesis - Premature infants who are feeding well and growing normally treatment only if there is poor weight gain , respiratory difficulties or abnormal heart rate; tachycardia. - Treatment; blood transfusion versus recombinant erythropoietin (EPO) treatment - Iron deficiency is the most common nutritional disorder in the world. - It is estimated that 30–50% of the global population has iron-deficiency anemia, most are in developing countries. - Breastfed infants are at risk of developing iron deficiency without regular intake of iron-fortified foods by 6 mo of age. o Decrease iron intake o Under nutrition o Excessive cow milk intake o Parasitic infections ankylostoma o Chronic blood loss ; as in peptic ulcer, Meckel diverticulum, polyp, hemangioma, or inflammatory bowel disease. o Decrease iron absorption : as in celiac disease - Manifestations of anemia as previously mentioned - Fainting attacks - Iron deficiency : o impaired neurocognitive function in infancy , loss of concentration o Pica : the desire to ingest nonnutritive substances o Pagophagia : desire to ingest ice - CBC : reveals reduced RBC count , Hb levels , hematocrit value , variable RBC shape and size o Microcytic anemia o High RDW o normal WBC count , normal or elevated platelet count. o reduced serum ferritin , reduced serum iron, and increased totaliron-binding capacity (TIBC) o stool analysis : for parasites o stool occult blood : for suspected GIT blood loss - Elemental iron : o a daily dose of 3-6 mg/kg/day in 1 or 2 doses. o Maximum dose : 150-200 mg of elemental iron / day - Within 72–96 hours—peripheral reticulocytosis and increase in HB over 4–30 days - Continue iron for 8 weeks after blood values normalize; repletion of iron in 1–3 months after start of treatment - Dietary counseling : - Increase intake of iron containing foods (iron from heme sources as meat is 10 times more bioavailable than iron from non heme sources as vegetables). - limit excessive intake of cow’s milk , Calcium , Tea , fibers as they may decrease the absorption of iron. - Co-administration of vitamin C is helpful. - Breastfeeding should be encouraged. - Add supplemental iron at 4 mo of age : 2 mg/kg/day. - Infants who are not breastfed should only receive iron- fortified formulas - Due to deficiency of folic acid or vitamin B12 (cobalamin) ; vitamins essential for DNA synthesis } Causes : - Mainly nutritional as Only animal sources (humans cannot synthesize) - Vitamin B 12 deficiency :from low vitamin B 12 levels in breast milk of B12-deficient mothers or Inadequate intake in strict vegetarian diet (vegan) o Decrease absorption : as in pernicious anemia, GIT disorders as H. pylori infection , celiac disease, Crohn disease , gastric bypass surgery (terminal ileum), o treatment with PPIs - Clinical features of anemia. - folate-deficient Peaks at 4-7 months , infants and children may manifest with irritability, chronic diarrhea , poor weight gain › Vitamin B12 deficiency (Sufficient stores in older children and adults for 3–5 years; but in infants born to mothers with deficiency, will see signs in first 4–5 months) - often present with: o nonspecific manifestations such as weakness, lethargy, feeding difficulties, failure to thrive and irritability. o pallor, glossitis, vomiting , diarrhea, and icterus. o Neurologic symptoms include paresthesia , sensory deficits , hypotonia , seizures , developmental delay, developmental regression , neuropsychiatric change. - The anemia is macrocytic (mean corpuscular volume >100 fL.) - Variable RBC shape and size. - Neutropenia and thrombocytopenia may be present in longstanding severe deficiencies. - Neutrophils are large , some with hypersegmented nuclei. hypercellular bone marrow with megaloblastic changes - In folic acid deficiency ; folic acid is 1,000 ng/ mL and/or a liver iron >5,000 µg/g dry weight. - Not used in children 25% } marked microcytosis, anemia, mild splenomegaly , occasionally jaundice or cholelithiasis. 1. Deletion of all 4 α-globin genes : profound anemia during fetal life, resulting in hydrops fetalis. - Abnormal Hb condition in which Hemoglobin HbS is the main form - Resulting from a change in β-globin gene that encodes valine instead of glutamine → exposure to deoxygenated state (hypoxia ,infection) HbS molecules interact with each other → rigid polymers → “sickled” RBCs → Intravascular sickling and hemolysis. - Hemolytic attacks - Sequestration crisis ; pooling of large amount of blood in spleen causing severe anemia and hypovolemic shock. - Aplastic crisis : induced by parvovirus B19 causing retculcytopenia , severe anemia - Vaso-occlusive crisis : o Recurrent painful crises (dactylitis) o Acute chest syndrome , pulmonary hypertension o Stroke o Recurrent fever and infections : due to functional asplenia → increased } risk of infection with encapsulated organisms such as Streptococcus pneumoniae, Haemophilus , influenzae type b, and Neisseria meningitidis. o Avascular necrosis of the hip ; with leg length discrepancy and limping. } Priapism (prolonged erection of penis> ischemic or low flow ) 1. Hb electrophoresis : In sickle cell anemia ; HbS is typically as high as 90% , Whereas in sickle cell disease ; HbS is 50% of all hemoglobin 2. Investigations for detecting complications : CT brain , chest x ray , - Hydroxyurea : ↓ frequency of acute painful episodes , vaso-occlusive crisis , rate of hospitalization , acute chest syndrome (ACS) and blood transfusion by 50%. - Hematopoietic Stem Cell Transplantation is Only curative therapy : - Red Blood Cell Transfusions to prevent progression of acute Complications such as aplastic crisis, splenic sequestration, and acute stroke, - Iron chelation therapy for iron overload - Prophylactic oral penicillin until at least 5 yr of age - immunizations against encapsulated organisms, including pneumococcal and meningococcal vaccinations besides annual influenza vaccine } 1. Glucose-6-Phosphate Dehydrogenase Deficiency. } 2. Pyruvate Kinase Deficiency } - G6PD is responsible for maintaining glutathione in a reduced state that prevent oxidants action on RBCs. } - G6PD Deficiency leads to hemolysis specially in response to exposure infections , drugs. } - X-linked disease; only in males , affects >400 million people worldwide } - The most common manifestations are: } o Neonatal jaundice } Recurrent acute hemolytic anemia which is induced by infections, certain drugs, and rarely, fava beans without organomegaly } - diagnosis depends ; decrease G6PD activity in RBCs ≤10% of normal } - plus indices of hemolysis (anemia , ↑ Reticulocyte count ↑ Indirect bilirubin …etc). } - in some individuals, ingestion of fava beans also produces an acute, severe } hemolytic syndrome, known as favism. } An autosomal dominant common cause of inherited hemolytic } - anemia with a prevalence of approximately 1 in 2,000-5,000 persons } - A membrane proteins defect (mostly ankyrin or spectrin) → loss of membrane Surface area without a proportional loss of cell volume → decreased erythrocyte deformability → RBCs become spherocyes → trapping and premature destruction of HS erythrocytes by spleen } Clinical picture : } - Recurrent hemolytic attacks } - Neonatal jaundice with hemolytic anemia } - Splenomegaly } - Gall stones } - Positive family history } Laboratory tests : } - Indices of hemolysis : as previous } - Blood smear : spherocytes } - Classic osmotic fragility test : poor sensitivity and specify } - Genetic diagnosis } Treatment : } Splenectomy is curative in most patients } Because spherocytes are destroyed almost exclusively in the spleen, } - Most significant extracellular factor of hemolytic anemia's } - Types : } 1. Isoimmune hemolytic anemia : hemolytic disease of the newborn (ABO or Rh incompatibility). } 2. Autoimmune hemolytic anemia } Causes : } 1. Idiopathic or } 2. Secondary to : } - Infections : (Epstein-Barr virus , cytomegalovirus and mycoplasma } - Immunologic diseases : SLE , rheumatoid arthritis. } - Immunodeficiency diseases : agammaglobulinemia , autoimmune lymphoproliferative disorder. } - Tumours : lymphoma, leukemia } - Drugs : penicillins, cephalosporins , L.dopa } Laboratory tests : } - Severe anemia ; hemoglobin levels < 6 g/dL } - Blood smear : spherocytes } - Marked reticulocytosis } - In some cases, a low reticulocyte count may be found, particularly early in the episode. } - platelet count is usually normal, but concomitant immune thrombocytopenic purpura sometimes occurs(Evans syndrome). } - Coombs test (direct and indirect Coombs test) : strongly positive, } Treatment : } - In mild cases : no specific treatment } - In severe cases : corticosteroids } - no adequate initial response : (IV) immunoglobulin. } - Refractory cases : monoclonal antibodies (rituximab) , Plasmapheresis } , Splenectomy thank you