National family health survey (NFHS-III) in 2005-06 found that the prevalence of anemia under 5 children approach about 70%.it is highest in children between 6-35 months of age.

PRESENTED BY

DR. VIJAY KR. SINGH(DNB PED)

UNDRE GUIDENCE OF

DR. T. K. MONDAL MD(PED)

M R BANGUR HOSPITAL

TOLLYGUNJ

KOLKATA

Decreased erythropoesis either due to deficiency of nutrition or defect in erythipoesis

Increased blood loss.

Diminished red cell survival as a result of immune disorder and chronic diseases.

Inadequate iron supply at birth like- prenatal or maternal nutrition deficiency, prematurity, low birth weight, multiple birth.

Inadequate absorption of iron-Gluten induced entrepathy, atrophic gastritis, post –gasterectomy, presence of phytats in diet and calcium, presence of recurrent diarrhea.

A. disorder of red cell production in which the rate of red cell production is less than expected.

1. marrow faillure

- Aplatic anemia( congenital and aquired)

2. Pure red cell aplasia

congenital

Diamond- Blackfan Syndrome

Aese Symdrome

Transient erthroblastopenia of childhood

C. Bone marrow replacement

malignancy

Ostipetrosis

Myelofibrosis

Chronic renal disesas

Vitamin D

Chronic renal disease

Hypothyriodism

Chronic infection

Protein energy malnutrition

Hemoglobin mutant with decreased affinity of O2

Abnormalities in cytoplasmic maturation

Iron deficiency

Thalassemia syndrome

Lead poisoning

Abnormalities in nuclear maturation

Vitamin B12

folic acid

Thiamin respond megaloblastic anemia

hereditary abnormalities in folatemetabolism

Orotic aciduria

Iron deficiency anemia is the most wide spread micronutrient deficiency and affect nearly 1.5 billion people globally.

Infant,pre school children, adolescent, and growing children are at greater risks.

According to NFHS-III in 2005-06, the prevalence of anemia under -5 yrs of children is about 70%.

Normal infant have 75mg of iron of per kg body weight.

Two third present in RBC.

The majority of body iron is in the form of Hb with about 10% in iron containing protien like myoglobinant cytochrome.

Pulses

green leafy vegetable

Dal

Bajra, dates, Nuts,

Jaggery

Meat and fish

Administration of Vitamin- C increased the absorption of iron

A. Decreased iron store Preterm

Small for date babyTwins

B. Decreased intake assimilation

Delayed intake of complementary feedingMalnutritionPoor iron diet

Mal absorptionChronic diarrhoea/ infectionGastero- intestinal surgery

C. Increased lossGastro intestinal bleedingMalaria

Hook worm infestation, trichuristrichuria.

H. pylori.Peptic ulcer.Diveticulutis, bleeding diathesis,

feto- femoral haemorrahagem, repeated venous sampling.

Prematurity

LBW Baby

Recovery from mal nutrition

Adolescence

Mostly are asymptomatic. Pallor is most important clinical sign but

usually not visible until Hb fall to 7-8gm/ dl.

Initial manifestation irritability, anorexia, pallor, hyper dynamic circulation leads to palpitation.

Shortness of breath, easy fatigability, exercise intorelerance, heart failure.

Sign- koilonychias, glossittis, angular chellitis, these are more common in long standing Anemia.

Hb 6 month-5yrs < 11gm/dl

6- 11yrs <11.5gm/dl

non pregnant <12gm/dl

pregnant < 11gm/DL

MCV <82

Transferring saturation <16%

Serum ferritin < 5yrs<12

>5yrs 15

in infection < 30

erythrocytes Zinc protoporphyrin < 5yrs >70

> 5yrs >80

Serum ferritin

decreased

Serum iron

decreased

Iron binding

capacity increased

RDW – Increased>19

RBC- Decreased

Peripheral smear shows-Hypochoromic microcytic red cell with sustacial variation in cell size, elliptocytic or cigar shaped red cell are seen.

Study Iron deficiency anemia

thalassemia Anemia of chronic diseases

Hemoglobin Decreased Decreased decreased

MCV Decreased Decreased Decreased-Normal

RDW Increased Decreased Normal-Decreased

RBC Decreased Normal-Increased

Normal-Decreased

Serum ferritin Decreased Normal Increased

Total iron binding capacity

Increased Normal Dcreased

ReticulocyteHemoglobin

Decreased Normal Normal -Decreased

Daily doses of 3-6 mg of elemental iron per kg of body weight in two or three divided doses

Parental iron prepation is usually used only in malabsorption,poor compliance.

Parental iron sucrose and gluconoate complex have a lower risk of serious reaction than dextran.

Doses of parental iron-2.3x body weight( 15-patient Hb in gm/dl) +500 to 1000 mg, given in divided doses.

Excessive intake of milk should be avoided

Repeat blood count after 4weeks Hb begins to increased 0.1 to 0.4 gm/ dl per day.

Iron medication should be continue for 8weeks after blood value normalized to re-establishment a response to therapy.

Time after iron administration

Response

12-24 hrs Replacement of intracellular iron enzyme , subjective improvement, decreased irritability, increased appetite

36- 48 hrs Initial bone marrow response, erythroid hyperplasia

48-72hrs Reticulocytisis, peaking at 5-7 days

4- 30 days Increased in Hb level

1- 3 months Repletion of store

Poor compliance.

Incorrect dose or administration.

Malabsorption of absorbed iron.

On going blood loss.

Concurrent vitamin B 12 deficiency or fIoatedeficiency.

Diagnosis other than iron deficiency like

Thalassemias

Hb C and E disorder

Anemia of chronic diseases

lead poisoning, sickle cell anemia

Sideroblastic anemia result from disorder of heme synthesis. Characterized by microcytic, mixed with normal RBC a picture of dimorphic anemia with high RDW, serum iron elevated and trasferrinsaturation increased. Elevated iron deposited in mitochondria.

Megaloblastic anemia is characterized by macrocytic red cells and erthroid precursor which show nuclear dymaturity.

The common causes are Vitamin B12 and Folic acid.

Incidence

incidence of folate deficiency as 6.8% and vitamin B12 32% and combined 20%.

Megaloblastic anemia affect all hem poetic cell line resulting anemia, thrombocytopenia, and leucopenia.

DNA synthesis impaired because of lack of methytetrahydrofolate, vit B12 plays an important role as co factor which is necessary for DNA synthesis.

Folate deficiency caused by

Decreased ingestion

celiac disease

Malabsorption state

Drugs like

Methotrexate

6- mercaptopurine

Trimithprine, azathiprine, phynatoin and other anticonvulsent and increased requirement infancy, hyperthyroidism, chronic hemolytic disease.

Vitamin B 12 deficiency causesd by

Decreased ingestion

intestinal parasite

Intritrisic intestinal diseas

intrinsic factor deficiency

Oritic aciduria

HIV infection

H. pylori infection

Pallor

Anorexia

Irritability

Thrombocytopenia, neurtopenia,

Glossitis, stomatitis, hyper pigmentation over skin on knuckles

Enlarged liver and spleen in 30- 40% cases.

loss of position sense, vibration sensation, memory loss, confusion, and neuron psychiatric symptoms.

MCV>110

Hyper segmented neutrophil.

Bone marrow shows one or more cell line affected

Bone marrow is cellular and nuclear –cytoplasmic asynchrony.

Elevated lactate and billirubin.

Folate- 1-5 mg/day for 3-4 weeks

Vitamin B 12 500-1000micro gram for 2-3 weeks then 100-250 micro gram once in a month to prevent recurrence.

Treatment with folic acid alone can produce hematological response in vitamin B 12 deficiency but does not currect neurological impairment caused by Vitamin B12

Difinition- A term infant have higher hemoglobin and hematocrit and larger RBC than older child and adult, within the 1st week of life a progressive decline of Hb level begins then persist for 6-8wks of life called physiological anemia of infancy.

Causes – there is gradual , normal development switch from Hb F to adult Hb after birth which is capable of delivering of more oxygen to the tissue.The increased in blood oxygen content and delivery result in the down regulation of erythropoietin production leading to suppression of eryththropoesis.

Tratment- No therapy required

Hb may decreased to 8gm /dl at 4-8 weeks of life in premature neonate with birth weight less than 1.5 kg.

Factors which contributes development of anemia

Reduced hematopoietic activity as evident by decreased reticulocytes countsReduced red cell survival.

Treatment – recombinant human erythropoietin with or without transfusion.with adequate protein vitamin E, iron to

achieve full benefit of medication.

DEFINITION

A plastic anemia is defined by as presence of any two peripheral blood criteria ANC(Absolute neutrophil count) < 1500/mm platelet count < 40,000/cumm.

CongenitalFanconi anemiaDyskeratotic congenitaReticular dysgenesisShwachman- Diamond syndrome

AcquiredIdiopathicRadiationViruses- hepatitis, epstein – Bar virus, Parvo virus

pregnancy,Hypogamaglubenumia, thymoma, Enophilic fascitis,pre- leukemic state

Chemical- benzene,sniffing insectiside

Infection

hepatitis, parvo virus,Epsttien bar virus

Drugs

sulfa, anticancer drugs chlotramphenical,gold,carbamazepin .indomethacin .

Radiation.

Thrombocytopenia, with bleeeding in skin, mucasal, GI tract, hematuria, menorrhagia.

Neutropenia leads to fever, infection.

Anemia appears last with breathlessness, puffiness of face, edema of feet, congestive cardiac failure

Peripheral smear- normocyticnormochromic RBC,leukopenia, decreased ANC, Thrombocytopenia.

Coagulation parametre will be normal.

Bone marrow biopsy

hypocellular marrow with empty spicule, increased fat space, hypoplasia.

Curative-stem cell transplant

Criteria for stem cell transplant

1. Patient who are young

2. Severe a plastic anemia

3. Matched related sibling donor

Other treatment

Antithymocyte globin(ATG)

G-CSF

CyclosporineA

condition Inheritancce

Associated features Risk of malignancy

Falconianemia

AR Absent thumb,absent radius, microcephaly,renalanomalies,short stature,skinpigmentation

AML, Myelodysplasia,oral and liver cancer

Dyskeratosiscongenita

X–linked,AD, AR

Nail dustrophy, leukoplakia, Squamous cell calcinoma, myelodysplasia

Diamond Blackman

AD, AR Short stuture,congenitalanomalies , elevated Hb F, Raised adenosine deaminase,

Leukemia, myelodypkasia

Diamond blackman syndrome is isolated erythroid hypoplasia occurs in early child hood and associated with congenital anomalies like stabismus, webbed neck, deformed thumb, bony abnormalities of finger and thumb, double ureter, hydronephrosis.

Bone marrow shows profound erythroid hypoplasia, macrocytosis. Inceased Hb F

Treatment-transfusion therapy, and steroid

A rare disorder characterized by diminised megakaryocytic tithstem cell defect without associated physical defect or abnormal defect.

A variety of cells are abnormally sensitive by complement, due to defect in glycosylphosphatidylinositol anchor, which bind protein to the cell membrane.

Chronic hemolytic is more common than sleep- induced hemoglobinuria.

Diagnosis – molecular testing of CD-56

It is characterized by presence of vacuoles in bone marrow precursor cells along with sideroblastic anemia with pancreatic exocrine abnormalities.