14 Anaemia power point presentation .ppt

This is the presentation of childhood anemia causes pathophsiology diagnosis and managmnet of anemia in children

1. GIZESHWORK (MD)
Anaemia

2. Overview
Definition
Physiology
Classification
Approach to the patient
Physiologic anemia
Iron deficiency anemia
Megaloblastic anemia(folic & B12def)
Hemolytic anemia

3. Defn .
Reduction of Rbc volume ,hgb concentration below
the range of value.
Few clinical disturbance occurs when hgb level falls
below 7-8 gm/dl.

4. Physiologic adjustment in anaemia
Increase cardiac out put
Increase oxygen extraction
Increase erythropotin
Shunting of blood flow toward vital organ and tissue
Increase 2,3 DPG conc with in Rbc (shift to right O2
dissociation curve

5. Classification of anaemia
A. Based on mean cell volume(MCV)
1-Microcytic _iron deficiency
_lead poisoning
_sideroblastic
_thalassemia
_chronic diseases
2-Normocytic_decreased production
_aplastic anemia
_BM replacement
(leukemia,tumor,storage disease)

6. Con.
normocytic _blood loss
_sequestration
_hemolysis
3-Macrocytic _vit B12 deficiency
_folate deficiency
_liver dieases
_Drugs(AZT, Anticonvulsants)

7. Cont,,
B.Based on the reticulocyte count and other findings
Mechanism of anemia
–decreased RBC production
-haemolysis
-blood loss

8. Cont..
Cause –under nutrition
-infection
-leukemia
-chronic diseases

9. Physiologic anemia &infacy
New born have higher hgb &hct with large Rbc.
With in 1
st
week of life aprogressive decline in hgb
begin.
6-8wk reaching its nadir called physiologic anemia of
infancy.

10. REASONS
1. Erythropoiesisceases abruptly with onset of respiration
2. EPO has decreased half life in newborns
3. Shortened survival of RBCs in newborns
4. Rapid expansion of blood volume which
accompanies rapid weight gain during the 1
st
three
months adds the need for rapid RBC production.
There is resumption of erythropoiesisat the age of
two to three months when Hgbfalls to 9-11 gm/dl

11. In Preterm
Hemoglobin declines to 7-9 dl/gm
Occur between age of 3-6 weeks
Exaggerated physiologic anemia
Inability to produce compensatory amount of EPO
Frequent phlebotomy in sick infant

12. Management
Part of developmental process , require no specific
therapy
Ensure essential nutrient for normal hematopoiesis-
folic acid and iron

13. Approach to the child with anaemia
History
dietary habits,growth &development
symptoms of chronic illness,malabsorption
history of blood loss,jaundice,familly history of
anemia
gallbladder dieases ,splenomegally or splenectomy
age and ethinic group
history of malaria attack
history of bleeding disorder

14. Physical examination
A/P
Pallor in mucus membrane ,nail bed & palm
Petechia & purpura
Lymphadenopathy & hepato splenomegally
Congenital anomaly
Evidence of chronic infection

15. Lab.
CBC & reticulocyte count
Peripheral morphology
Blood film for malaria
Bone marrow examination
S/E
Additional investigations

16. Iron deficiency anemia
Most common nutritional deficiency
Iron metabolism-
75% of Fe bound in heme protien(hgb ,myoglobin)
remainder bound in storage protein eg ferritin &
hemossiderin
3% bound in critical enzyme (catalaze &
cytochromase)
Most Fe recycled from break down of old Rbc

17. IDA
Iron balance is achieved by control of intestinal
absorptionwhich is a function of 3 factors ;
-body iron store
-erythropoietic rate
-bioavailability of dietary iron
Heme dietary source ( fish, poultry, meat) have high
bioavailability than do non heme sources like vegetables.
Iron is absorbed 2-3 times more efficiently from human
milk than from cow’s milk.
Infants breast fed exclusively should receive iron
supplementation from 4 months of age.
Ascorbic acid enhances absorption of iron from non heme
sourceswhere as tea and phosphates inhibit

18. Risk groups
Preterms, low birth weights, -decreased Iron store
Administration of EPO for physiologic anemia of
infancy
Fetomaternal hemorrhage
Twin –Twin Transfusion Syndrome
Other perinatal hemorrhage incidences

19. Risk groups
Age 6-24 months-increased demand
Early introduction of cow`s milk
Blood loss
Insufficient dietary intake
Decreased absorption

20. C/f
Occur 9months-24months of age
Pallor
Irritability
Poor feeding
Tachypneas
Cardiomegally
Impaired psychomotor & or mental dev`t
Decreased work capacity & school performance
Pica, pago phagia ,blue sclera ,cheliosis

21. C/F
Anorexia, tachycardia, cardiac dilatation and
systolic murmur is usually present when Hgb falls
below 5 gm/dl.
Anorexia and irritability w/c are signs of advanced
deficiency reflect depletion of tissue stores.
It has effect on neurologic and intellectual
function.
Some of the clinical manifestations may be related
to the role of iron in certain enzymes e.g.. MAO in
CNS manifestations.

22. Cont…
Iron deficiency anemia is associated with mild to
moderate defects in leukocyte function leading to
infection
Koilonychias(spooning of nails)
Blue sclera(other differentials include
osteogenesis imperfecta, RVI, Fanconi anemia)
Cheliosis

23. Lab findings
The first thing to be detected is decreased serum ferritin
level
Next serum iron level decreases
Iron binding capacity of the serum (serum transferin)
increases
Percent saturation of transferin decreases
Free erythrocyte protoporphorines accumulate
In a third of patients occult blood is detected in stool

24. Cont…
Hemoglobin and hematocrit decreases
RBC morphology becomes microcytic and hypochromic
Poikilocytosis and anisocytosis(increased RDW)
Corrected reticulocyte index is low
Occasionally nucleated RBCs may be seen in the peripheral
blood
Thrombocytopenia or thrombocytosis with normal
leukocyte count may be found

25. Diagnosis
Increase RDW –earliest sign
Decreased serum ferritin
Low Hgb
Hypochromic microcytic
Decrease retic count
Increased total Iron binding capacity
Decreased serum Iron

26. DDX
1.Anemia of chronic disease (ACD) usually
normocyticoccasionally microcytic. Serum Fe level
and TIBC are decreased. Serum ferritinlevel is
normal or elevated
2.Lead poisoning
3.Alpha and beta thalasemia
4.Hemoglobin H disease
5.Sideroblastosis

27. Rx
1. Oral iron administration
6mg/kg/day -2-3 doses, 2-3 month
Response to reticulocyte count with in 48-72 hrs
2. Blood transfusion indicated when the anemia is severe
(<4mg/dl) or when the patient decompensated
3 . Parentraliron therapy has limited role due to
risk of anaphylaxis
4.Follow up-exclusive breast feed
iron fortified cereals
NB. Treatment should continue for 8 wks after blood values are
normal

28. Response
Time after Fe
administration
Response
12-24 hr Decreased irritability and
increased appetite
36-48 hr Erythroid hyperplasia
48-72 hr Reticulocytosis (peak at 5-7 days)
4-30 days Increase in hemoglobin
0.5gm/dl/day
1-3 months Replacement of stores

29. Causes of failure to respond (causes of
refractory IDA)
1.Compliance failure
2.Problems of absorption
3.Ongoing blood loss
4.Chronic inflammatory diseases
5.Incorrect diagnosis

30. Megaloplastic anemia
Megaloplastic anemia is amacrocytic anemia caused
by deficiencies of vit B12 ,folic acid (FA) or both
Both folic acid or B12 are cofactor required in the
synthesis of nucleoprotein
Almost all cases of childhood megaloblastic anemia
result from deficiency of folic acid or cobalamin
w/c are very important in synthesis of DNA and to
lesser extent RNA.

31. Megaloblasticanemia is x’zedby
1.Ineffective erythropoiesis
2.Large RBCs( increased MCV)
3.Hypersegmentedneutrophils
4.Asynchrony b/n nuclear and cytoplasmicmaturation

32. Folic acid deficiency
Folates are abundant in many foods including green
vegetables, fruits and animal organs like liver and kidney
Naturally occurring folates are polyglutamated w/c are
changed to monoglutamates by folate conjugase in the
intestinal brush boarder aiding easy absorption
Biologically active form is THF (produced by dihydrofolate
reductase mediated reaction)
Folic acid is absorbed throughout the small
intestine
Megaloblastic anemia occurs after 2-3 months of folate free
diet

33. Etiology
1.Inadequate folateintake -anemia manifested in
pregnant woman, growth in infancy and chronic
hemolysis). Goats milk is deficient .
2.Decreased folateabsorption-in cases of chronic
diarrheal and diffuse inflammatory Ds . In Pts taking
phenytoinand Phenobarbital
3.Congenital abnormalities in folatemetab.
Congenital dihydrofolatereductasedeficiency
4.Drug induced abnormalities in folatemetab.
Methotrexate, pyrimethamine, trimethoprim

34. C/F
Peak age is 4-7 monthsof age
More common in VLBW
Besides the usual c/f Pts are irritable, fail to gain
weight and have chronic diarrhea
Hemorrhages from thrombocytopenia occur in
advanced cases
Folic acid deficiency accompany Kwashiorkor,
marasmus or sprue

35. Lab finding
Macrocytic anemia (MCV >100 fl)
Variations in RBC shape and size
Low retic. Count with nucleated RBCs
Neutropeniaand thrombocytopenia
Hypersegmented neutrophils
Low serum and RBC folate level
Elevated or normal cobalamin and Fe level
Markedly elevated LDH
Hypercellular marrow

36. Treatment
Folic acid 0.5-1 mg/day for 3-4 wks till definite
hematologic response
If specific Dx is in doubt , smaller doses of
folate(0.1mg/day)may be used for a week b/c
hematologic response is expected with low dose and
within 72 hours.
Larger doses (>0.1mg) can correct anemia of
cobalamin deficiency but may aggravate any
associated neurologic abnormality.

37. Vitamin B12(cobalamin) deficiency
Vitamin B12 is mainly found in animal food
Humans can not synthesize vitamin B12.
Cobalamin combines with R protein and IF in the acidic
media of the stomach, traverses the duodenum w/r
pancreatic enzymes break down the R protein and are
absorbed in the distal ileum via specific IF-cobalamin
receptors
In the serum it is bound to TC IIw/c transports the
vitamin to liver, BM and other storage organs
Older children and adults have sufficient stores for at least
3 -5 yrs

38. Etiology
1.Inadequate intake-in vegans, breast fed infants born
from vegans
2.Lack of intrinsic factor-congenital pernicious
anemia or juvenile pernicious anemia, gastric surgery
3.Impaired absorption-regional enteritis, NEC,
surgery, bacterial over growth, D.latum, Imerslund
Grasbecksyndrome
4.Absence of transport protein-lack of or presence of
functionally defective TC II

39. C/F
Present with non specific manifestations like weakness,
fatigue, FTT or irritability
Other findings include pallor, glossitis, vomiting, diarrhea
and icterus
Neurologic manifestations include parasthesias, sensory
deficit, hypotonia, seizure, developmental delay,
developmental regression and neuropsychiatric changes
Hyporeflexia, Babinski responses and clonus
Neurologic problems can occur in the absence of any
hematologic abnormality

40. Lab. findings
Macroovalocytosis of RBCs
Hypersegmented neutrophils
Neutropeniaand thrombocytopenia
Serum vit. B12 level is <100pg/ml
Serum folate and iron level are normal
High level of LDH level
Moderate elevation of serum billirubin
Excessive excretion of methylmalonic acid in urine is
reliable and sensitive index

41. Diagnosis
If specific cause is apparent from history e.g.
History previous surgery it may be reasonable to
start treatment
If there is no obvious cause do “schilling
test”
1.Give radioactive vit. B12 PO
2.Give flushing dose (1mg)of non radioactive vit.
B12 parenthrallyafter 2hr
3.10-30%of previously absorbed radioactive is
excreted in urine in normal children but less
than 2%is excreted in patients with pernicious
anemia

42. Cont…..
4. To confirm the absence of intrinsic factorgive 30
mg of IF with second dose of radioactive vit.
B12.we expect a normal response in patients with
pernicious anemia
5. If the defect is due to absence of receptors there will
be no improvement in absobtion

43. Treatment
Prompt hematologic response follows parenteral
administration of vit. B12 (1mg)usually with
reticulocytosis in 2-4 days
If there is neurologic manifestation 1 mg should be injected
daily for 2 weeks
Monthly administration of 1 mg of vit. B12 IM is sufficient
as maintenance
Physiologic requirement is 1-5µg/day and mini dose of this
amount can be given as therapeutic trial when Dx is in
doubt.

44. Hemolytic Anemia
Premature RBC destruction
Anemia:
if destruction > Bm RBC production
1% RBCs (senescent) are removed daily, replaced by BM
new RBCs
In Hemolysis:
RBC survival is shortened
BM activity is increased with reticulocytosis
BM can increase it’s output 2-3fold acutely and 6-8fold if long-
standing hemolysis

45. Evidence of hemolysis
Expanded medullary spaces at the expense of cortex
Measurement of RBC survival
Oncreased fecal urobilinogen
Gall –stones (Ca bilirubinate)
Increased serum unconjugated bilirubin
Altered heme-binding proteins:
Decreased haptoglobin
Decreased hemopexin
Increased methemalbumin
Free Hb in the plasma (evidence of IV hemolysis)
Free Hb in the urine

46. Classification
I. Cellular
Cell membrane defect
 Hereditary spherocytosis
 Eliptocytosis,Stomatocytosis,PNH
Enzymatic defects
 G6PD deficiency
HemoglobinAbnormalities
 Sickle Cell disease
 Thalassemias
II. Extracellular
Antibodies
Mechanical factors
Plasma factors

47. Autoimmune HemolyticAnemias
I. Warm Reactive Autoantibodies
Active b/n 35-40oC
IgG
No need of complement for activity
Primary (Idiopathic)
Secondary:
•SLE, Ulcerative colitis
•Lymphoproliferative disorders
II. Cold Reactive Autoantibodies (Cryopathic Hemolytic Syndrome)
--Abs are active in <37oC, IgM, need complement
Primary
Secondary
•Infections (e.g. mycoplasma, EBV)
III. Drug-induced immune hemolytic Anemia
Hapton/drug adsorption (e.g. penicillin)
Ternary (immune) complex (e.g.quinine)
True autoantibody induction (e.g. methyldopa)

48. THANK YOU!