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Saturday, April 27, 2013

Pediatrics in focus

Neoplasia in Pediatrics
Wiskott aldrich syndrome- X -inked recessive eczema thrombocytopenia immunodeficiuncy syndrome
Hodgkin disease-malignant lymph node disorder of unknown etiology
Nonhodgkin lymphoma-heterogenous group of lymphoid  tumors
                             Causes: Viral infxn and immunodeficiency
What is tumor lysis syndrome? overload of lysed tumor material in the blooidstream leading to hyperuricemia compromising kidney function   Tx: hydration is important, allopurinol and sodium bicarbonate
 What is Bloom syndrome? aka Bloom–Torre–Machacek syndrome, a rare autosomal recessive disorder characterized by short stature and predisposition to the development of cancer. Cells from a person with Bloom syndrome exhibit a striking genomic instability that includes excessive homologous recombination. 
 What is the most common leukemia in children? ALL (acute lymphoblastic leukemia)
In leukemia, what is induction? 4 to 6 weeks initial treatment phase for reduction of leukemic burden
Usual Rx: corticosteroid, vincristine, asparaginase with or without anthracycline
What constitutes remission? decreased of blast cells in bone marrow by 5%.
Commonly used maintenance drugs in ALL? methothrecate, 6-mercaptourine, corticosteroid, vincristine
What is Fanconi Anemia?
genetic defect in a cluster of proteins involved in DNA repair, most common in Ashkenazi Jews, frequently leds to  the development of acute myelogenous leukemia 
Treatment for AML? 
ARA-C-it kill cells by interfering DNA synthesis, used in the treatment of AML and nonHodgkin Lymphoma, aka as cytrabine or ARA-C Arabinofuranosyl cytidine 
Daunorubicin-