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Hanan FathyAss.lecturer Pediatric Nephrology Unit 
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Male patient 13 years old (DOB:18/2/96), living in Alexandria.Presented at the age of six months by: marked pallor and generalized weakness. Blood  picture revealed severe anemia.He was given blood transfusion,and he received blood 
 
 
Parents are consanguinous.No history of previous hospital admission.No history of similar condition in the family.
 
He has icthyosis vulgarissince birth.
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