peds36

Question 1

isoimmune neutropenia

Answer 1

passive transfer of antineutrophil antibodies from mom to fetus; resolves by 8 weeks of life

Question 2

leading cause of death from disease in childhood

Answer 2

cancer

Question 3

most common childhood cancers

Answer 3

leukemia, brain tumors, lymphoma, neuroblastoma, soft tissue sarcomas, Wilms tumor, and bone tumor

Question 4

wiskott-aldrich syndrome is associated with what cancers?

Answer 4

leukemia and lymphoma; w-a is characterized by B and T cell dysfunciton

Question 5

EBV associated with what cancer?

Answer 5

lymphoma

Question 6

most common malignant abdominal tumors

Answer 6

wilms tumor and neuroblastoma

Question 7

down syndrome associated with what type of cancer?

Answer 7

leukemia

Question 8

turner syndrome associated with what cancer

Answer 8

gonadoblastoma

Question 9

trisomy 13 associated with what cancer

Answer 9

leukemia, teratoma

Question 10

trisomy 18 associated with what cancer

Answer 10

wilms tumor, neurogenic tumors

Question 11

klinefelter syndrome associated with what tumor

Answer 11

leukemia, germ cell tumors, breast cancer

Question 12

fanconi anemia associated with what kind of cancer

Answer 12

leukemia

Question 13

xeroderma pigmentosa associated with what

Answer 13

basal and squamous cell carcinoma, melanoma

Question 14

ataxia telangiectasia assoc with what cancer

Answer 14

lymphoma, leukemia, sarcoma

Question 15

bloom syndrome associated with what cancer

Answer 15

leukemia, lymphoma, GI malignancies, solid tumors

Question 16

beckwith-wiedemann syndrome associated with what cancers

Answer 16

wilms tumor, hepatoblastoma, rhabdomyosarcoma, adrenocortical carcinoma

Question 17

NF type 1 associated with what cancer

Answer 17

brain tumors, lymphoma, leukemia, malignant schwannoma

Question 18

NF type 2 associated with what cancers

Answer 18

acoustic neuroma

Question 19

acute lymphocytic leukemia (ALL) more common in what age and gender

Answer 19

2-6 years olds, boys

Question 20

most common childhood cancer

Answer 20

ALL

Question 21

ALL classification

Answer 21

by cell mophology; L1, L2, L3; L1 is most common;

Question 22

ALL- t cells or b cells or both?

Answer 22

t cell phenotype in 25%, B cell in less than 5%; pre-B cell in 70%

Question 23

ALL on CBC?

Answer 23

anemia with thrombocytopenia; the bone marrow is overwhelmed by producing these abnormal B or T cells; total WBC can be high, normal or low

Question 24

does a normal CBC rule out leukemia?

Answer 24

nope! Confirmation by bone marrow evaluation

Question 25

management of leukemia involves what?

Answer 25

three stages: induction, consolidation, and maintenance

Question 26

indiction for leukemia treatment

Answer 26

aims to destory as many cancer cell as possible; drugs vary; intrathecal methotrexate is given to all children during induction; remission is induced in 95% of kids

Question 27

consolidation for leukemia treatment

Answer 27

systemic chemo and prophylaxis to prevent CNS involvement

Question 28

what treatments are used for consolidation

Answer 28

intrathecal metotrexate is continued; cranial radiaion may be given to high risk kids but avoided in kids less than 5

Question 29

maintenance therapy for leukemia

Answer 29

involves daily and periodic chemo during remission for up to 3 years

Question 30

what to do for leukemia patients who relapse after remission?

Answer 30

bone marrow transplant

Question 31

metabolic complications from spontaneous or therapy induced cell lysis (leukemia was example)

Answer 31

aka tumor lysis syndrome; hyperuricemia may lead to renal insuff; hyperkalemia may lead to cardiac dysrhythmias, hyperphosphatemia my lead to hypocalcemia with tetany

Question 32

longterm survival for ALL patients

Answer 32

Eighty five percent

Question 33

acute myelogenous leukemia (AML)- how diff from ALL

Answer 33

ALL is b and t cells; AML is grnaulocytes like neutrophils, eosinophils, mast cells

Question 34

how do they classify AML?

Answer 34

M1 through M7; based on cell morphology; M7 is assoc with down syndrome; auer rods in M3

Question 35

Clinical features of AML vs ALL?

Answer 35

CNS involvment more common in AML

Question 36

auer rods

Answer 36

think AML

Question 37

management of AML

Answer 37

very intensive myeloblative therapy to induce remission; bone marrow transplant is recommended once patient is in remission

Question 38

prognosis for AML

Answer 38

chemo works in 50%; BMT works in 70%; therapy more effective in Down syndrome for unknown reason

Question 39

chronic myelogenous leukemia (CML)

Answer 39

least common type of leukemia

Question 40

how is CML classified

Answer 40

adult type and juvenile type

Question 41

adult ctype CML

Answer 41

twice as common as juvenile; older kids; philadelphia chromosome

Question 42

juvenile CML

Answer 42

infants and kids; no philly chrom

Question 43

phily chorm

Answer 43

reciprocal translocation between the long arms of choms 9 and 22, leading to fusion of BCR/ABL1 that produces BCR-ABL fusion protein

Question 44

clinical features of adult CML

Answer 44

nonspec symptoms; masssive splenomegaly leading to abdominal distension; extremely high WBC

Question 45

clinical features of juvenile CML

Answer 45

fever, chronic eczemalike facial rash, suppurative lymphadenopathy, petechiae and purpura; moderate leuocytosis, anemia, and thrombocytopenia

Question 46

treatment of CML

Answer 46

bone marrow transplant in both adult and juvenile forms; imatinib; radiation therapy is not effective

Question 47

prognosis of adult CML

Answer 47

biphasic course, which chemo working initially but then patient deteriorates and is not responsive

Question 48

prognosis of juvenile CML

Answer 48

often fatal; relapse occures in 50% of cases, even with bone marrow transplan

Question 49

hodgkin's disease

Answer 49

cancer of the antigen processing cells in the lymph nodes or spleen

Question 50

hodgkins disease is associated with what infection?

Answer 50

EBV; if you get EBV-assoc mono, you have a 2-4 times greater risk of hodgkins

Question 51

presenting features in hodgkins

Answer 51

painless lymphadenopathy; most commonly in supraclavicular or cervical regions