B&l: Test 2: 6. Showalter : Pediatric Anemias I

Front 1

Most common form of iron deficiency in kids?

**

Back 1

Iron deficiency (hypochromic, microcytic)

Front 2

6 months and 18-24 months is when we see the most iron deficiency in kids, why?

Back 2

time of rapid growth, generally still on formula/breast milk and running out of iron stores...time to start thinking about switching to solid food

Front 3

Before 12 months, what can be a huge cause of iron deficiency?

***

Back 3

drinking too much cows milk

leads to occult blood loss/iron deficiency

Front 4

pallor, fatigue, weakness, SOB, decreased appetite, pica, headache...

all buzzwords for what problem in kids?

Back 4

iron deficiency anemia

Front 5

What are the levels of

Hematocrit
Hemogolibn
MCV
Serum Ferritin
Serum Iron
TIBC

for iron deficiency anemia

Back 5

Hematocrit: decrease
Hemogolibn: decrease
MCV: low
Serum Ferritin: low
Serum Iron: low
TIBC: high/increased

Front 6

anemia leading to problem with DNA

Back 6

Macrocytic anemia (B12 deficiency)

Front 7

goats milk and vegetarian mothers can lead to what pediatric anemia?

Back 7

B12/folate (macrocytic)

Front 8

what are the 2 types of hereditary hemolytic anemia?

Back 8

Hereditary spherocytosis
Hereditary elliptocytosis

Front 9

a northern European newborn is brought to your office with hyperbilirubinemia, spelnomegaly, pallor, and fatigue. You note intermittent exacerbations of anemia...what are you thinking?

Back 9

Hereditary spherocytosis

Front 10

Numerous microspherocytes- are the hallmark of the disease

Back 10

Hereditary spherocytosis

Front 11

direct Coombs test does what?

Back 11

tells us that mom has anti-bodies that can attack the babies RBCs and lead to jaundice (aka ABO incompatability)

Front 12

Hereditary spherocytosis vs. elloptocytosis

DAT (direct antiglobin test) positive

Back 12

Spherocytosis

Front 13

what kind of anemia is hereditary spherocytosis

Back 13

Normocytic and hyperchromic

Front 14

what will the retic count, unjconjugated bilirubin lvls, and osmotic fragility be like in hereditary spherocyosis?

Back 14

Retic: up

Unconj bili: up

Osomotic fragility: increased **

Front 15

describe Alpha thalassemia- trait

Back 15

mild anemia

Children with alpha-thalassemia trait do not have thalassemia disease

Front 16

what can be diagnostic for beta thalassemia minor?

Back 16

Elevated level of hemoglobin A2

Front 17

on electrophoresis, what will be findings in beta thalassemia major?

Back 17

Increased A2 and F hemoglobin at an increased age (which is not normal)

Front 18

what physical finding can be a giveaway for beta thalassemia major?

Back 18

bone involvement

Facial deformities: The eyes appear slanted and the cheekbones and maxillary areas become prominent

Front 19

in beta thalassemia lifelong transfusion is often needed...what problem can this cause? Solution?

Back 19

Hemosiderosis (Hemochromatosis) - an overload of iron
Over time, people with thalassemia accumulate deposits of iron in their bodies due transfusions

solution: iron chelator

Front 20

how long do blood cells live in sickle cell disease?

Back 20

10-20 days

120 is normal

Front 21

30% of patients experience one or more episodes of acute splenic sequestration in what problem?

Back 21

sickle cell anemia

get a sudden enlargement of the spleen due to pooling irregular red blood cells, get an acute exacerbation of anemia and in more severe cases shock can result

Front 22

Dactylitis can be a symptom of what?

Back 22

sickle cell disease

Front 23

Pleuritic chest pain
Fever
Pulmonary infiltrates with hypoxemia
Life threatening

are all known as what? What disease are they a complication of?

Back 23

Acute Chest Syndrome

sickle cell disease

Front 24

in sickle cell disease what can cause aplastic crisis?

***

Back 24

Human parvovirus
“Fifth disease”- common childhood illness
Usually self limiting infection with mild fever, “slapped cheek” appearance of the face and classic rash.

Front 25

male pt has ingested fava beans....you think

Back 25

Glucose-6-phosphate dehydrogenase (G6PD) deficiency

it is X linked

Front 26

Most patients have episodic hemolysis at times of exposure to an oxidant stress rather than chronic hemolytic anemia

Back 26

G6PD deficiency

Front 27

this is a chronic hemolytic anemia that can have echinocytes on peripheral smear....

Back 27

Pyruvate kinase deficiency

(note: echinocytes on peripheral smear is a buzzword)

Front 28

in red cell aplasia, what will your retic count be like? (low or high)

Back 28

low

Front 29

Triphalangeal thumbs, renal anomalies, chronic anemia, and jaundice can all be seen in what problem?

Back 29

Diamond-Blackfan anemia

note: this is a red cell aplasia

Front 30

Severe macrocytic anemia
Marked reticulocytopenia
Bone marrow shows a marked decrease in erythroid precursors
Decreased levels of adenosine deaminase

seen in....

Back 30

levels for Diamond-Blackfan anemia

Front 31

early detection of Diamond-Blackfan anemia is important because we can treat with...

Back 31

corticosteroids

results in increased erythropoiesis in about 2/3 of patients, thus avoiding the difficulties and complications of long term chronic transfusion therapy

Front 32

this is a normocytic anemia that is due to decreased red cell production. It develops slow, the CV system can compensate, and Children with hemoglobins as low as 4 or 5 g/dL look remarkably well.

Back 32

TEC (Transient erythroblastemia of childhood)

Front 33

Pallor
No hepatospenomegaly
No jaundice
No peripheral smear evidence of destruction of cells
No lymphadenopathy
No evidence of chronic disease
No short stature
No congenital anomalies

Back 33

TEC (Transient erythroblastemia of childhood)

note: Transient
Self limited
Resolves in 4 to 6 weeks

Front 34

in this problem, erythropoietin production abruptly decreases in the first week of life, but then later will return to normal

Back 34

Physiologic anemia of infancy

Front 35

Definition of anemia

Back 35

Hemoglobin more than 2 standard deviations below the mean in healthy children.

Front 36

What is the RDW like in:

Iron deficiency anemia
Alpha/beta thalassemia
Congenital Hemolytic Anemia

Back 36

Iron deficiency anemia- high
Alpha/beta thalassemia- normal
Congenital Hemolytic Anemia- high

Front 37

What is the MCV like in:

Iron deficiency anemia
Alpha/beta thalassemia
Congenital Hemolytic Anemia

Back 37

Iron deficiency anemia- low
Alpha/beta thalassemia- low
Congenital Hemolytic Anemia-normal

Front 38

what can low and high retic counts tell you?

Back 38

low: may indicate bone marrow disorders or aplastic crisis

high:generally indicates a hemolytic process or a hemorrhagic process (active blood loss)

Front 39

Iron deficiency
Thalassemia trait
Hemoglobin EE
Lead poisoning
Chronic infection
Sideroblastic anemia

MCV?

Back 39

Low MCV (Microcytic anemia)

Front 40

Nutritional deficiency (folate, vitamin B12)
Bone marrow failure (Diamond-Blackfan anemia, drug-related: {anticonvulsants, immunosuppressive therapy}, myelodysplasia, leukemia)
Liver disease
Cyanotic heart disease
Hypothyroidism
Down syndrome

MCV?

Back 40

High MCV (Macrocytic anemia)

Front 41

RBC aplasia (transient erythroblastemia of childhood-TEC)
Malignancy (leukemia, metastatic disease) (if neutrophil and/or platelet count is low)
Aplastic anemia (early)

MCV & Retic?

Back 41

Normal MCV and Low reticulocyte count

Front 42

Acute or chronic infection or inflammation (anemia of inflammation) (wait until the child is well and obtain CBC again)
Malnutrition
Inflammatory bowel disease
Renal disease


MCV & Retic?

Back 42

Normal MCV and Normal reticulocyte count

Front 43

Peripheral smear- 5 Clues to the Etiology of anemia..what are the 5
?

Back 43

1. Cell size is not uniform (high RDW)
2. Cell shape is abnormal (poikilocytosis)
3. Pigmentation is abnormal
4. Polychromasia (elevated reticulocyte count-an indicator of regenerative anemia)
5. Inclusion bodies are present

Front 44

Basophilic stippling (ribosomes) should make you think?

Back 44

Lead poisoning

Front 45

Heinz bodies should make you think?

Back 45

G6PD or oxidant stress hemolysis

Front 46

what are the 3 common causes of anemia in the 1st year of life

Back 46

Blood loss (iron def)

Congenital hemolytic anemia

hemoglobinopathy

Front 47

What anemia should you consider gender as a diagnostic feature?

Back 47

G6PD deficiency...

X linked, so seen in boys

Front 48

family history of Gallstones, neonatal jaundice, enlarged
spleen, and transfusion in family members suggest.....

Back 48

a mechanism for an increased risk of a heritable form of anemia