Anemia - كلية الطب...Anemia Anaemia is defined as an Hb level below the normal range. The normal range varies with age, so anaemia can be defined as: • Neonate: Hb

Anemia

Anaemia is defined as an Hb level below the normal

range. The normal range varies with age, so anaemia

can be defined as:

• Neonate: Hb

Physiologic Anemia of the Newborn At one week postnatal all RBC indices begin declining to a minimum value reached at about 2 months of age.

decreased RBC production

plasma dilution associated with increasing blood volume

shorter life span on neonatal RBCs (50-70 days)

more fragile RBCs

switch from HbF to HbA HbF decreases about 3% per week

at 6 mo. HbF represents only 2% of total Hb

switch to HbA provides for greater unloading of oxygen to tissues d/t lower oxygen affinity of HbA relative to HbF.

seldom produces symptoms

not altered by nutritional supplements

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Anemia of Prematurity

Occurs in low birth weight infants w/ poor erythropoietin response Protein content of breast milk may not be sufficient

for hematopoiesis in the premature infant.

Hb level rapidly declines after birth to a low of 7-10 g/dl at 6 weeks of age.

Signs and Symptoms apnea

poor weight gain

pallor

decreased activity

tachycardia

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mohammadHighlightearlier than physiological anemia which reached by age of 9 weeks

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mohammadSticky Noteanisocytosis : variation in RBC size

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Fe++ deficiency

Tx– Fe++ replacement gives dramatic response

reticulocytosis in 72 hr, Hgb responds at ~1g/L per wk, iron stores us. replenished by 3 mo

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mohammadSticky Notewhen give iron the all symptoms improve

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mohammadSticky Noteantenatal..... gama is predominant beta is limited

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mohammadSticky Noteprecipitate in RBC which result in RBC destruction

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mohammadSticky Notebecause of B2 production remain normal

mohammadSticky Note4 (B2) globins

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mohammadSticky Notethis occur after neonatal period because the production of the Gama globin decrease naturally (which form Hg Barts)

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mohammadSticky Notecommon in Jordan

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mohammadSticky NoteIron def anemia not responding to iron .....do Hg elctrophresis >> elevated HbA2 and HbF

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mohammadSticky Notebecause the neonate dont have sufficient amount of B globin

we wait until age of 6 months until diagnosis to and do electrophosesis

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mohammadSticky Notethese two changes occur due to activation of extramedullary erythropoiesis

+splenic sequestration

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mohammadSticky NoteB thalasemia major

Beta Thalassemia Major

No production of Beta chains- Chromosome 11 Autosomal recessive

25 % chance with each pregnancy Pre-natal testing for carriers Chorionic villous sampling for diagnosis

Transfusion dependent-allows for normal development Pen Prophylaxis, Anti oxidants Splenectomy after age 5

Iron overload- inherent and transfusion Need chelators

BMT is a cure

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mohammadHighlightChelators are small molecules that bind very tightly to metal ions

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Management Blood transfusion lifelong monthly transfusions of red blood cells. Keep Hb anove 10 g/dl. iron chelation with subcutaneous desferrioxamine, or with an oral iron chelator drug, such as deferasirox, starting from 2 to 3 years of age. Patients who comply well with transfusion and chelation have a 90% chance of living into their forties Bone marrow transplantation, which is currently the only cure. It is generally reserved for children with an HLAidentical sibling as there is then a 90–95% chance of success (i.e. transfusion independence and long-term cure) but a 5% chance of transplant-related mortality.

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