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Peds > Peds HemOn > Flashcards

Peds HemOn Flashcards

1
Q

A child is born with a congenital disease linked to defective DNA repair. What is the condition and how will it be manifest? Tx?

A

Dx: Congenital aplastic DNA aka Fanconi
Presentation: thrombocytopenia or neutropenia –> pancytopenia; short height, cafe-au lait,mental retardation
tx: Bone marrow transplant

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2
Q

What differential dx should be included in a child with congenital aplastic anemia (Fanconi)?

A

ddx: idiopathic thrombocytopenic purpura (ITP)

* congenital aplastic anemia presents with thrombocytopenia or neutropenia –> pancytopenia

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3
Q

A kid comes in with peripheral pancytopenia and hypocellular bone marrow. You order a lab test and see a low reticulocyte count. Dx? Causes? Tx?

A

Dx: Acquired aplastic anemia
Etiology: 50% idiopathic (unknown), some due to drug or VIRAL infection (usually Hepatitis)
Tx: stop offending drug, antibiotic if infection, blood transfusion in severe cases, bone marrow transplant potentially

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4
Q

What is the most common aplastic anemia and what are some complications associated with this anemia?

A

ACQUIRED aplastic anemia

Complications: overwhelming infection bc low WBC and severe hemorrhage bc low platelet

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5
Q

A child’s lab work reveals microcytic hypochromic anemia (low Hct, Hbg) combined with low serum iron and elevated TIBC. Physical manifestations include glossitis, fatigue, angular stomatitis. Dx? tx? cause?

A

dx: iron deficiency (most common cause of anemia in kids*)
tx: iron supplement
cause: usually poor intake

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6
Q

What other ddx must you consider with an iron deficiency dx? and how would you r/o this ddx?

A

Thalassemia specifically in African, Asian, or Mediterranean descent. Confirm with a Hbg electrophoresis lab test

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7
Q

Megaloblastic anemia… ddx? what labs to determine dx? what is most important to treat

A

ddx: B12 or folate deficiency anemia
labs: order serum vitamin B12 and serum folic acid
* always treat B12 deficiency due to neurologic risk

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8
Q

A child presents with jaundice, an enlarged spleen and lab peripheral smear revealing spherocytes and an elevated reticulocyte count. What is the dx and underlying cause?

A

dx: hereditary spherocytosis anemia
cause: inherited hemolytic anemia due to problems in the spleen

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9
Q

What are the three different types of thalassemia, which is most severe, and what will the lab results reveal. Any treatment?

A

alpha (usually no sx), beta minor, and beta major (most severe)
LABS: microcytic cells out of proportion to degree of anemia (microcytic level doesn’t reflect severity of low Hbg levels)
TX: for beta major may need bone marrow transplant

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10
Q

G6PD etiology and presentation?

A

most common RBC enzyme defect causing hemolytic anemia

Presentation: hyperbilirubinemia, Heinz bodies, Low Hbg, high reticulocyte count

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11
Q

a young african girl comes in with her mom complaining of severe pain. You note jaundice and decide to order labs. What specific study should you order and what is your primary ddx?

A

ddx: sickle cell anemia
lab: Hbg electrophoresis lab (would reveal low HbgS and Hct), elevated retic count
* note: low retic count due to hemolytic anemia

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12
Q

What is the cause of the severe pain associated with sickle cell anemia. How should you treat this anemia and what are some risks/complications?

A

Cause of pain: Vasooclusion
TX: educate patient and manage acute problems, blood transfusion, narcotic for pain (controlled), oxygen for vasooclusion

RISKS: high risk bacterial sepsis

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13
Q

peripheral blood smear reveals basophilic stippling.. dx, tx?

A

dx: lead poisoning
tx: chelation therapy (from pharm remember it is edetate calcium disodium)

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14
Q

this form of increased RBC is caused by hypoxemic disorder that may be congenital and affects only one red cell line. Dx, tx?

A

Primary polycythemia

tx: phlebotomy to decrease amount of RBC

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15
Q

Secondary polycythemia is distinguishable from primary how? tx?

A

Secondary polycythemia is due to cyanotic congenital hear disease (tetralogy of fallot and transposition of great BV), primary is potentially congenital hypoxemic disorder affecting one red cell line

tx of secondary: fix underlying disorder/heart condition

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16
Q

anemia of chronic disease lab findings

A

normochromic, normocytic

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17
Q

tests to consider for anemia

A

first: Hbg (determine normo or hypochromic)
second: MCV (determine micro vs macro vs normocytic)

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18
Q

signs of anemia

A

increased pulse (80-120 in kids), headache, sleepiness, poor feeding

19
Q

This is the most common bleeding disorder in kids ages 2-5 and is always considered a ddx for congenital aplastic anemia (fanconi). What is it?

A

Idiopathic thrombocytopenia Pupura

*immune process of antibodies to own platelets often following a virus

20
Q

Tx for ITP

A

90% have spontaneous remission
tx with prednisone if bleeding

*remember, ITP is often preceded by a virus

21
Q

List the inherited bleeding disorders and their cause

A 
Hemophilia A (factor VIII deficiency)
Hemophilia B (factor IX deficiency)
22
Q

List the acquired bleeding disorders

A

DIC, liver disease (clotting factors made here) ,vitamin K deficiency

23
Q

What labs should be ordered for acquired bleeding disorders (vit K def, Liver disease, DIC)

A

if concern: PT (prothrombin time), aPPT (test of hoice for heparin), bleeding time, platelet count

*PT is what is used to monitor warfarin/coumadin

24
Q

Tx DIC, vit K def

A

DIC: treat underlying cause, often hospitalize bc this

25
Q

What are the inherited thrombotic disorders and the risks for them?

A

Inherited protein C, S deficiency, antithrombin III def, factor V leiden mutation
*risks: may see DVTs of lower extremities or pulmonary embolus, positive famility hx clots

26
Q

this is the most common vasculitis in kids age 2-5. What is it and how do you treat it?

A

Henoch-schonlein purpura. 66% have preceding URI. No tx needed

27
Q

Pt presents with LAD, fever. pain in pelvis,spine and legs and an enlarged spleen. Lab studies reveal WBC >200,999, elevated LDH and decrease in 2 cell lines. What is your primary dx and what should you do next to confirm dx?

A

dx: Acute lymphoblastic leukemia

confirm dx via bone marrow exam that would reveal leukemic blasts

28
Q

Lab results for DIC

A

elevated PT, aPTT, Ddimer, FDP

thrombocytopenia

29
Q

etiology of ALL?

A

most common malignancy of childhood

Proliferation of immature lymphocytes

30
Q

tx ALL and expectation?

A

chemo

anticipate tumor lysis syndrome hyperkalemia, hyperuricemia, hypocalcemia, renal failure

31
Q

this neoplastic disease is associated with cytotoxic agents, radiation, and down syndrome. Usually adults over 65 are affected by in children 80% have cytogenic clonal abnormality

A

Acute myeologenous leukemia

32
Q

which lab signs are consistent with Acute myeologenous leukemia?

A

anemia, thrombocytopenia, neutropenia BUT leukocytosis in 25%
*though it won’t be as high as in acute, this leukocytosis is a medical emergency! more so than acute lymphoblastic anemia

33
Q

CML!! etiology, tx?

A

Chronic Myelogenous leukemia is only %5 of childhood leukemias.–> philadelphia chromosome

tx: interferon alpha or bone marrow transplant
* *all leukemia leans toward bone marrow transplant

34
Q

Young girl comes in with a severe headache. She has been vomiting and appears lethargic with an unsteady gait. You measure her head circumference and it appears unsymmetrical. What is a likely dx and what do you need to do to rule it out

A

dx: brain tumor… r/o with MRI

* NOTE: other sx might include irritability and visual changes

35
Q

what is the most common childhood solid tumor, what are the different categories and what is the usual tx?

A

Brain tumors are most common childhood tumor.

  1. glial (astrocytoma and ependymoma)
  2. nonglial (medulloblastoma)
    tx: usually surgery, possibly chemo/radiation
36
Q

child has B symptoms (anorexia, wt. loss, fever, fatigue, night sweats) and painless cervical adenopathy. Upon palpation, you notice a mass in their mediastinum. You fear this ____. How do you confirm

A

ddx: Hodgkin’s lymphoma

* must stage with chest xray, CT, bone marrow biopsy and see Reed Sternberg (Owl eyes) cells

37
Q

What is the tx for Hodgkin’s lymphoma? What is the one good thing about a child having this cancer?

A

treat hogkin’s lymphoma with chemo

the good thing is kids usually have better response than adults

38
Q

child has B symptoms (anorexia, wt loss, fever, fatigue, night sweats) abdominal pain and is coughing, having trouble breathing especially when lying down. You note lymphadenopathy present. What should you do next? ddx? if confirmed, tx and concerns

A

BIOPSY

ddx: non-hodgkins lymphoma
tx: chemo
concern: unlike adult NHL, kid NHL is rapidly growing diffuse malignancy

39
Q

a 3 year old child exhibits B symptoms, bone pain and you notice an abnormal ABDOMINAL MASS upon palpation. dx? how to confirm? etiology, tx?

A

dx: NEUROBLASTOMA
etiology: SNS originating cancer
* confirm with bone scan
tx: surgery, radiation, chemo
* 90% dx before 5 yr

40
Q

most common soft tissue sarcoma? most aggressive bone cancer?

A 
rhabdosarcoma = most common soft tissue sarcoma
osteosarcoma = most aggressive bone cancer
41
Q

Young pt is found to have fever, HTN, hematuria and an asymptomatic abdominal mass. suspected dx? tx?

A

Nephroblastoma

tx: surgical removal/exploration of abdomen, chemo

42
Q

a child in the midst of adolescent growth spurt and develops legs of differing lengths. He is dx with bone cancer of long bones via bone scan. What is cancer specifically would this be? tx? risk?

A

Osteosarcoma

tx: surgery, chemo
* risk: 70% just receiving treatment develop pulmonary metastatic disease within 6 mth, this is why need to combine with chemo

43
Q

A child has an enlarged abdomen and so you check serum alpha fetal protein. What are you looking for? and what would be a concern if this was found

A

Hepatic tumor
concern bc 57% found in childhood are malignant

tx: chemo, surgery

Peds (8 decks)

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