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anemia

2. anaemia
PE29.1 Discuss the etio-pathogenesis, clinical features,
classification and approach to a child with anaemia
PE29.2 Discuss the etio-pathogenesis, clinical features
and management of Iron Deficiency anaemia
PE29.3 Discuss the etiopathogenesis, clinical features
and management of VIT B12, Folate deficiency anaemia

3. Anemia— Definition
It is a condition in which hemoglobin (Hb)
concentration and/or reduced absolute
number of circulating RBCs, lower than the
age-adjusted reference range for healthy
children and insufficient to meet an
individual’s physiological needs.

4. Haemoglobin levels to diagnose anaemia
WHO
Nathan and Oski's hematology of
infancy and childhood

5. the etio-pathogenesis
I Impaired red cell formation
A/ Deficiency
•Decreased dietary intake, Increased demand ,Decreased absorption
B/ Bone marrow failure
•Failure of a single or all cell lines, Infiltration
II Blood loss
III Hemolytic anemia
•Corpuscular (membrane, enzymatic or hemoglobine
defects)Extracorpuscular (immune, idiopathic)

7. Symptoms of Anemia

8. ØTiredness, easy fatigability and generalized
muscular weakness are
Ø poor feeding, irritability, inadequate
school performance; pallor -
.

9. CVS symptoms
•(Common)
Dyspnea on exertion, tachycardia,palpitation
•Hemic murmurs (severity) midsystolic flow murmurs
the pulmonary area, reflecting increased velocity of
blood passing through the valves.
•moderate to severe anemia -Systolic bruits, postural
hypotension and congestive heart failure
•Severe anemia - a high output state with elevated
pulse pressure and a 'collapsing' character.

10. SKIN
Café au lait spots Fanconi anemia
Jaundice Hemolysis, hepatitis
Petechiae, purpura Bone marrow infiltration,
autoimmune hemolysis with autoimmune
thrombocytopenia, hemolytic-uremic syndrome
Erythematous rash Parvovirus, Epstein-Barr virus
Butterfly rash Systemic lupus erythematosus

11. HEAD Frontal bossing Thalassemia major, severe iron
deficiency, chronic subdural hematoma Fanconi anemia
Eyes Microcephaly & Microphthalmia Fanconi anemia
Retinopathy Hemoglobin SS, SC disease Kayser-Fleischer
ring Wilson disease Blue sclera Iron deficiency
Mouth Glossitis Vitamin B12 deficiency, iron deficiency
Angular stomatitis Iron deficiency
Chest Murmur Endocarditis; prosthetic valve hemolysis

12. Hepatomegaly Hemolysis, infiltrative tumor,
chronic disease, Splenomegaly Hemolysis, sickle
cell disease (early), thalassemia, malaria,
Nephromegaly/Absent kidney Fanconi anemia
Spoon nails Iron deficiency
Beau line (nails) Heavy metal intoxication, severe
illness

13. Conjenital Anaemia
Diamond-Blackfan anemia a congenital form of Pure red cell
aplasia usually presents at 3-month or earlier and shows a
consistently low reticulocyte count and absence of erythroid
precursors in the marrow.
Fanconi anemia It is the most common cause of inherited
bone marrow failure characterized by pancytopenia.variable
onset, with children presenting at 3-4 years of age or even in
adulthood. a rare genetic disorder, involving all three blood
cell lines. Additionally, it affects almost all organs of the body

24. The red cell distribution width (RDW)
RDW results help your provider understand how much your red blood
cells vary in size and volume.
Low ROW = all the red blood cells uniform in size,
High RDW shows = the cells vary in size greatly
a varied size distribution of red blood cells is a hallmark of
, and as such shows an increased RDW in virtually all
cases.
In the case of both iron and B12 deficiencies, there will normally be a
mix of both large cells and small cells, causing the RDW to be elevated.

25. I ,
•Low Iron body store - premature infants, low-
birthweight infants, or perinatal blood loss
•undernutrition - excessive consumption of cow's milk
(low iron content, blood loss from milk protein colitis)
•Blood loss -occult bleeding may be caused by a lesion
of the gastrointestinal (GI) tract, such as peptic ulcer,
paracytic infections Meckel diverticulum, polyp,
hemangioma, or inflammatory bowel disease.

26. Pathophysiology
Iron is essential for multiple metabolic processes,
including oxygen transport, DNA synthesis, and
electron transport.
In severe iron deficiency, iron containing enzymes
are low and can affect immunee and tissue
function. Iron defidencv anemia can result in
diminished growth and leantlng and have serious
consequences in children.

27. three stages
First stage: Iron stores are depleted. ...
Second stage: When iron stores are low, the
normal process of making red blood cells is
altered.
Third stage: Iron-deficiency anemia develops
because there isn't enough iron to make
hemoglobin for red blood cells

28. Clinlcal
Most children with iron-deficiency anemia are asymptomatic
Hb 7-8 g/dL -visible Pallor is the most recognized clinical sign
Older cold intolerance, fatigue, exercise-induced dyspnea, or
decreased mental acuity.
<5 g/dL - irritability, anorexia,lethargy and systolic flow
murmurs often heard, Congestive heart failure
severe angular stomatitlS, glosiitis,
splenomegaly koilonychia pica pagophagia (desire to ingest
ice)

29. Lab evaluation
• smear microcytic and hypochromic, poikilocytosis
•Hemoglobin(g/dL) <11.0 age <4 yr, RDW - increased
• MCV (µm3 ) <70 for age 6-24 mo
•ferritin (µg/L) ≤5 yr: <12 ; >5 yr: <15 infection: <30- 100
•Transferrin saturation <16% normal 25-50%
•total iron binding capacity (TIBC) is increased
•High free erythroprotoporphyrin (FEP) before anemia

31. The mos.t rost-cifectir.e oral preparati.m !s Z=-ii.o:is rulfate
(20'1!.I elemental iron). The dose for rreaane:t! cl anemia. is
3-6 mg/kg/c6-y eleme:nttl iron.. T~..e ~....ru!°'-.,-te count
incre::o...xs ·within 72-%hcnns afterinifuting ttte-.a.py. A...~
rorrection of an._""IJtia, oral iron should b= ronfurued for
4-6 months to replenish iron stores.

32. Treatment
therapeutic dose calculated elemental iron daily
total dose of 3-6 mg/kg of elemental iron maximum dose is
150-200 mg elemental iron daily
Ferrous sulfate is 20% elemental iron by weight ideally given
between meals with vitamin C–containing juice,
oral iron 4-6 months to replenish iron stores.
intravenous low-molecular-weight iron dextran, parenteral
iron sucrose, ferric carboxymaltose, and ferric gluconate
complex are available, only LMW iron dextran is FDA
approved for use in children for iron deficiency

33. parenteral iron
Indiacations
(i) intolerance oral iron.
(ii) malabsorptive states, and
(iii) oral replacement cannot meet ongoing blood loss
intravenous low-molecular-weight iron dextran, parenteral iron sucrose,
ferric carboxymaltose, and ferric gluconate complex are available, only
LMW iron dextran is FDA approved for use in children for iron
deficiency. parenteral iron sucrose use in India
Total dose (mg)= [Target Hb - Acutal Hb] x weight (kg) x 24 + (15 x
weight (kg)]

34. 12-24 hr subjective improvement; decreased
irritability; increased appetite; increased serum iron
36-48 hr Initial bone marrow response; erythroid
hyperplasia
48-72 hr Reticulocytosis, peaking at 5-7 days
4-30 days Increase in hemoglobin level; increase in
mean corpuscular volume; increase in ferritin
1-3 mo Repletion of stores

35. MEGALOBLASTIC ANEMIA
It is characterized by macrocytic red blood cells
and erythroid precursors,caused by impaired DNA
synthesis.
The peripheral blood smear large, oval RBCs with
increased mean corpuscular volume.
Neutrophils characteristically hypersegmented,
having >5 lobes.

37. Causes of Folate Deficiency
•Inadequate Nutrition Poor diet Poor food preparation
methods Exclusive feeding with goat's milk
•Defects in Absorption Gastric achlorhydria, Diseases
of the upper small intestine (Tropical sprue Celiac
disease Dermatitis herpetiformis) Inflammatory
bowel disease, Hereditary folate malabsorption
• Increased Requirements or Losses Prematurity
Chronic hemolytic anemia Dialysis Hyperthyroidism

38. Causes of Folate Deficiency
•Disorders of Transport
Cerebral folate deficiency (genetic or acquired) ¶
•Disorders of Cellular Metabolism
Drugs inhibiting folate metabolism Antifolates (e.g., methotrexate)
Pyrimethamine ; trimethoprim Sulfasalazine Valproic acid ,
Inherited defects
Methylenetetrahydrofolate reductase (MTHFR) deficiency
Dihydrofolate reductase deficiency

39. Causes Vitamin B12 deficiency
1. Nutritional Deficiency (Inadequate Vitamin B12 Intake)- infants mostly
nutritional, breast milk of B12 -deficient mothers, a strict unsupplemented
vegetarian
2. Gastric Abnormalities gastric acid dissociates protein-bound vitamin B12,
and decline gastric acid and intrinsic factors in Gastritis, histamine H2
receptor antagonists and proton pump inhibitors
Autoimmune gastritis/juvenile pernicious anemia
adolescence.autoimune antibody against IF and the proton pump.
cutaneous candidiasis, hypoparathyroidism,
3. Fish tapeworm infestation Dibothriocephalus latus, which competes with
the host for vitamin B12 absorption

40. Causes Vitamin B12 deficiency
4. gastrointestinal disorders Small Bowel Disease -malabsorption of vitamin B12.
Helicobacter pylori infection, sprue, Crohn disease. Celiac disease. pancreatic
insufficiency- Pancreatic enzymes are required to cleave vitamin B12 from salivary
proteins and transfer it to the intrinsic factor.
4. drug-induced - Metformin (calcium-dependent ileal absorption of the B12-
intrinsic factor complex),
6. Genetic abnormalities neonates with genetic abnormalities such as
transcobalamin deficiency and Imerslund Grasbeck syndromeselective vitamin B12
malabsorption in the ileum
7. Inborn Errors of Cobalamin Metabolism- conversion of Cbl to methylcobalamin
and adenosylcobalamin (infancy with megaloblastic anemia, vomiting,
anddevelopmental delay in homocystinuria)

41. •lnborn errors of cobalamln metabollsm
congenital intrinsic factor deficiency, defieiency of transcobalamln I and
II, cobalamin malabsorption due to defect in intestinal receptor
(lmerslUnd-Grasbeck syndrome) , Methylmalonic aciduria
Homocystinuria
•Inborn errors of folate metabolism
congenital folate malabsorption, Dihydrofolate reductase deficiency,
methyltetrahydrofolate homocysteine methyltransferase deficiency
•Other Inborn errors Hereditary erotic aciduria Lesch-Nyhan syndrome,
Thiamine responsive megaloblastic anemia

42. Clinical - Sign symptoms Of Pallor +
•H/O dietary medication and contributing medical disorders
•Pancytopenia and signs of thrombocytopenia (Petechiae and
hemorrhagic manifestation) and neutropenia
•hepatosplenomegaly
•hyperpigmentation skin on knucklesterminal phalanges,
•neurologic - loss of position and vibratory sensation (earliest
signs) Later other posterior and lateral column deficits
•Memory loss, confusion and neuropsychiatric symptoms

43. Subacute Combined Degeneration of the Spinal
Cord
vitamin B12 deficiency.
degeneration of the dorsal columns and the
lateral columns of the spinal cord due to
demyelination.
Age < 50 years Absence of sensory deficits
Absence of Romberg's sign Absence of Babinski's
signs Involvement of ≤ 7 spinal segments on MRI

44. •macrocytosis (>110 fl ) Hypersegmented neutrophils (nucleus
>6 lobes), Neutropenia and thrombocytopenia may found,
increased serum lactate dehydrogenase (ineffective
erythropoiesis)
•hypercellular bone marrow because erythroid hyperplasia,
megaloblast, giant metamyelocytes (abnormal neutrophil)

45. • Serum folic acid deficiency levels
<3 ng/mL (5-20 ng/mL N)
• Low RBC folate level (150-600
ng/mL of packed cells -normal)
(better indicator chronic deficiency.)

46. vitamin B12 deficiency
•elevations of serum bilirubin levels (2-3 mg/dL) also may be
found.
•increased methylmalonic acid in the urine (n: 0-3.5 mg/24
hr) Specific and sensitive index of vitamin B12 deficiency
•In the past the Schilling test, a measure of Cbl absorption,
was the gold standard, but it is no longer available, with no
comparable replacement.
•Anti-IF antibodies and anti–parietal cell antibodies for the
diagnosis of pernicious anemia.

47. Treatment Folate deficiency
•doubtful diagnosis - therapeutic trial- folate (0.1 mg/day) X 1
wk for hematologic response <72 hr
• >0.1 mg folate can correct vitamin B12 anemia but might
aggravate neurologic abnormalities
•Folic acid therapy (0.5-1.0 mg/day) for 3-4 wk for definite
hematologic response Maintenance a multivitamin
(containing 0.2 mg of folate)
•Only folate correct the anemia, not correct cobalamin
neurological disorder, result in progression neuropsychiatric
complications.

48. B12 - treatment
•Treatment regimens in children have not been well studied
•The cause B12 deficiency dictate treatment dosage route duration
•Cyanocobalamin is available a nasal spray andparenteral injection.
•Dose adjustments in response to clinical status and laboratory values.
•The physiologic requirement for vitamin B12 is about 1-3 µg/day.
start with a minidose as a therapeutic test observe Hematologic
responses
• the doubtful diagnosis or in severe anemia and higher initial doses
may causes severe metabolic disturbances

49. specialist treatment
•vitamin B 12 at a dose of 1mg (1000 µg) i.m. (lower
doses (250 µg) infants) - A decrease in MCV,
reticulocytosis and higher platelet and neuoophil
counts is observed
• pernicious anemia and malabsorption - vitamin B12
(1000 µg) IM daily X 2 weeks, then weekly until
hemntocrit value normal and then monthly for life.
•In neurological complications 1mg (1000 µg)
i.m.every day 2 weeks, then every 2 weeks for 6
months and monthly for life.