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pediatrics > hematology > Flashcards

hematology Flashcards

1
Q

infants - iron

A

full term infants are born with adequate iron stores to prevent anemia for the first 4-6 months. Preterm infants are at significantly increased risk for iron-def anemia –> iron supplementation should be started at birth in exclusively breastfed preterm infants and continued until age 1

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2
Q

risk for iron def anemia in infants

A

prematurity
iron def mother
cow’s milk before 1 year
Delay start of solid food

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3
Q

timeline of infant nutrition

A 
exclusive beastfeeding until 6 months
introduction of pureed foods at 6 months
introduction of cow's milk at 1 year
\+ VIT D
\+ IRON if premature
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4
Q

management of sickle cell anemia

A

acute pain: fluids, analgesia, +/- transfusion

maintenance: vaccination, PENICILLIN (until 5), folic acid, hydroxyurea (if reccurent vaso-occlusive crises)

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5
Q

osteonecrosis - ESR, CRP

A

both normal (and no leukocytosis)

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6
Q

MCC of pediatric stroke - how to diagnose

A

SCC

- confirm with electrophoresis

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7
Q

Common etiologies of pediatric stroke

A
  1. SCC (MC)
  2. prethrombotic disroders
  3. Congenital cardiac disease
  4. bact meningitis
  5. vasculitis
  6. Focal cerebral arteriopathy
  7. head + neck trauma
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8
Q

hereditary spherocytosis - how to confirm diagnosis

A

eosin-5-maleimide biding and acidified glycerol test

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9
Q

hereditary spherocytosis - treatment

A

floci acid, transfusion, splenectomy

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10
Q

hereditary spherocytosis - complications

A

pigment gallstones

aplastic crisis from B19

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11
Q

reticulocytes in hemolytic anemia in infancts

A

not very high (babies are not able to produce)

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12
Q

characteristics of splenic sequestration in SC anemia

A
  • rapidly enlarged spleen

- high reticulocytes

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13
Q

suscpicion of iron def anemia in baby

A

iron for 1 month –> recheck –> if Hb rise 1, continue for 2-3 more months
if not –> check Hb, colonoscopy, renal studies

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14
Q

Fanconi’s anemia

A

pancytopenia + characteristic congenital anomalies such as: hyperpigmentation on the trunk, neck and interginous areas, cafe au lait spots, short stature, upper limb abnormalities, hypogonadism, skeletal anomalis, eye or eyelid changes, renal malformations
DIAGNOSIS AT 8 YEARS OLD

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15
Q

osteomyelitis vs avascular necrosis in SCA - main difference

A

osteomyelitis has signs of inflammation

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16
Q

the earliest manifestation of vaso-occlusive disease in SC anemia

A

dactylitis

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17
Q

medulobastoma - another symptom

A 
truncal ataxia (vermis location)
rare --> hemisperes of celebelum --> intenional tremor
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18
Q

tumor near hypophisis with calcifucation

A

craniopharyngeoma

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19
Q

SC disease vs trait regarding Hemoglobins percentages

A

trait: HbA: 50-60 / HbF: less than 2 / HbS: 45-45%
disease: HbS: 85-95 / HbF: 5-15

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20
Q

SC disease with hydroxyurea - HbF %?

A

more than 15%

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21
Q

anemia of prematurity - etiology

A
  • impaired EPO production
  • short red blood cell life span
  • Iatrogenic blood sampling
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22
Q

anemia of prematurity - clinical manifestation and labs

A
  • asymptomatic, tachycardia, apnea, poor weight gain

- low reticulocyte count, normocytic, normochromic

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23
Q

anemia of prematurity - treatment

A

minimize blood draws, iron, transfusion

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24
Q

ALL - how to confirm diagnosis

A

more than 25% of blasts in BM biopsy

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25
Clinical features of pineal gland masses
Parinaud syndrome: limited upward gaze, Upper eyelid retraction (Collier sign), pupils not reactive to light, reactive to accommodation Obstruction hydrocephalus: papilledema, headache, vomiting, ataxia
26
Diamond Blackfan anemia
macrocytic pure red aplasia associated with several congenital anomalies such as short stature, webbed neck, cleft lip, shielded chest and triphalangeal thumbs
27
diamond Blackfan vs fanconi anemaia regarding age of diagnosis
fanconi: 8 Blackdan: 1
28
ITP - treatment
children: skin manifestation only: observe, if bleeding, IVIG or glucocortic adults: platelets more than 30000 and cutaneous symptoms, without bleeding: observe, less than 30 or bleeding: IVIG or glucocort
29
are platelet affected in SC disease?
no | only in sequestration
30
Lymphoblast cytolasma - stain
pedioedi acid schiff
31
how to differentiate iron def anemia from thalassemia
iron def has increased RDW (more than 20%)
32
iron def in cow milk - mechanism
low iron low bioavailability GI bleeding
33
SC trait - splenic infractions ?
rare (at high altitudes)
34
hemarthroses - next step
CBC and coagulation studies
35
histology of cells in ALL
lack peroxidase positive granules but contain cytoplasmic aggregates of PAS (+) material + TDT
36
Wiskott-Aldrich syndrome - clinical manifestation
1. eczema 2. reccurent infections 3. microthrombocytopenia (small and few platelets)
37
Wiskott-Aldrich syndrome - treatment
stem cell transplant
38
Wiskott- Aldrich syndrpme - etiology
XR defect in WAS protein gene --> impaired cytoskeleton changes in leukocytes + platelets
39
what to see in cells in people with splenectomy
Howel jolly bodies
40
neonatal polyceythemia - causes definition
1. increased erythropoiesis from intrauteine hypoxia (Maternal diabetes, hypertenson or smoking) 2. IUGR 3. Transfusion (twin-twin, delayed cord clamping) 4. Geneticmetabolic disease definition: more than 65%
41
Neonatal polycythemia - clinical presentation
1. asymptomatic (MC) 2. Ruddy skin 3. hypoglycemia, hyperbilirubinemia 4. resp distress, cyanosis, apnea, 5. irritability 6. abd distention
42
neonatal polycythemia - treatment
1. IV fluids 2. glucose 3. partial exchange transfusion (if symptomatic)
43
iron def anemia in children blood transfusion
rare (even if Hb is 4)
44
Cow milk - when and amount
introduction at 1 years old | no more than 24 ounces / d
45
characteristic of avascular necrosis' pain
chronic no fever no signs of inflammtion
46
hydroxyyrea is used in patients with SC disease to decrease
1. pain crisis 2. need for transfusion 3. episodes of acute chest syndrome (increase in fetal Hb, which dilutes the amount of sickle hb)
47
Low folic or B12 in infants
no anemia in infancy
48
vit K def - PT, PTT
increased PT | normal PTT, (increased if severe def)
49
thalassemia β minor - reticulocytes
high
50
Mentzer index
MCV/RBC | in thalassemia minor is less than 13 (very low MCV and normal to high RBC)
51
RBC in thalassemia minor
normal to high
52
tilateral rb
bilateral rb PLUS pineal gland tumor
53
MCC of macrocytic anemia in SC disease
follate def
54
Diamond black .. - treatment
Blood transfusion | Steroids
55
SC disease - sequestration vs aplastic crisis
seq: elevated reticulocytes, rapidly enlarging spleen | aplastic crisis:, low reticulocytes, transient arrest of erythropoiesis, 2ry to infection
56
HUS treatment
1. fluid 2. blood transfusion 3. dialysis
57
another symptom of vaso-occlusive crisis in SC anemia
low grade fever
58
neuroblastoma - periorbital ecchymoses
due to orbital metastasis
59
harlequin sign
absent facial flushing (Horner syndrome)
60
SSA - sequestration treatment
Fluids Small volume transfusion Splenectomy
61
How to prevent pmeumonococcal sepsis in SCA
Vaccination and penicillin

pediatrics (16 decks)

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