anemia - كلية الطب...anemia anaemia is defined as an hb level below the normal range. the...
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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
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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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mohammadLinemost imp one
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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
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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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