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PAEDS SC1 > Haem > Flashcards

Haem Flashcards

1
Q

blood cells in babies

A 
Hb-- high at first, decrease by 2 months, decrease more if prime
normal: Fe/ B12/folate
preme: lower Fe/folate
WBC: INCREASE
platelets= normal
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2
Q

elemental Fe intake kids

A

1mg/kg/day

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3
Q

Fe body stores in kids

A

2.5g,
75%= blood
25%= ferritin and haemosiderin

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4
Q

breast milk iron

A

low, but 50% absorbed

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5
Q

formula milk

A

ADDED iron

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6
Q

cows milk iron

A

greater iron than breast, but 10% absorbed

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7
Q

cereals + Fe but…

A

1% absorbed

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8
Q

important foods questions to ask in anaemia

A
  • breast or bottle
  • cereal/ high fibre foods
  • cows milk
  • tea
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9
Q

when do patients with Fe def anaemia present

A

Fe= LESS than 6-7g/dL

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10
Q

treatment of Fe deficiency anaemia

A

Fe oral and dietary device

— continue Fe 3 most after Hb normal

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11
Q

Diamond Blackfan

A

congenital red cell aplasia

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12
Q

PC diamond blackfan

A

short stature and thumb abnormalities

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13
Q

Labs diamond blackfan

A

decrease Hb
decrease reticulocytes
decrease red cell precursors in marrow
normal billirubin

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14
Q

HbH

A

beta tetramers

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15
Q

HbBarts

A

gamma tetramers– hydrops fetalis

alpha thal major

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16
Q

how can a baby with hydrops fatalis survive?

A

intrauterine transfusions,

immediate post-delivery transfusions

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17
Q

age of presentation of beta than major

A

3-6 months

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18
Q

age of presentation of hereditary spherocytosis

A

childhood

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19
Q

diagnosis of hereditary spherocytosis

A
  1. blood smear
  2. osmotic fragility test
  3. DCT– exclude autoimmune HA
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20
Q

tx of hereditary spherocytosis

A
  1. folic acid

2. splenectomy– 7 YEARS OLD

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21
Q

NB pain crisis questions for sickle cell

A
  1. bone pain
  2. hand swelling
  3. chest pain
  4. abdo pain
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22
Q

sickle cell disease

A

normal ish HbS
retinopathy
and osteonecrosis: shoulder and hips

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23
Q

sickle cell anaemia

A

HbSS

= most severe

24
Q

sickle cell thal

A

HbST

25
Q

sickle cell trait

A

HbS

26
Q

acute anaemia in sickle cell

A

infection= haemolytic
parvo= aplastic
sequestration crisis: HSM,abdo pain,ciruclatory collapse

27
Q

long term complications of sickle cell

A
  1. short stature and delayed puberty
  2. education/ cognition
  3. adenotonsilar hypertrophy
  4. obstructive sleep apnea
  5. heart failure
  6. sttroke
  7. billirubin gallstones
  8. renal – haematuria, renal papillary necrosis
28
Q

diagnosis of sickle cell

A

[UK newborn screening]

  • Hb electrophoresis
  • Metabisulfite/ Sickledex
  • O2 sats, CXR, PaO2
  • cultures
29
Q

Prophylaxis of sickle cells

A
  • vaccines: MenC, HiB, PCV
  • daily oral penicillin
  • daily folic acid
30
Q

Acute tx sickle crisis

A 
= AHOE
Analgesia
Hydration
Oxygen
Exchange transfusion
31
Q

Who to give exchange transfusion for sickle cell

A
  • acute chest syndrome
  • stroke
  • priapism
32
Q

Chronic problems in sickle cell

A
  • hydroxyurea

- BMT

33
Q

Who to give BMT for sickle cell

A
  • no response to hydroxyurea

- stroke

34
Q

causes of aplastic anaemia

A
  1. viruses
  2. drugs
  3. benzene + glue
35
Q

fanconi anaemia

A

AR

  • short stature
  • skin hyper pigmentation
  • renal abnormalities
  • abnormal radius and thumb
  • micropthalmia
36
Q

shwachman diamond syndrome

A

AR

  • bone marrow F
  • skeletal abnormalities
  • exocrine pancreas
  • –> isolated neutropenia
  • –> mild pancytopenia
37
Q

gene in diamond blackfan

A

ribosomal protein

38
Q

gene in shwachman diamond

A

SBDS

39
Q

most common cause of thrombocytopenia in childhood

A

ITP

40
Q

increase destruction of platelets

A

immune:

  • ITP
  • SLE
  • alloimmune neonatal thrombo

nonimmune

  • HUS
  • TTP
  • CHD
  • DIC
  • Kassabach merritt
  • hyperplenism
41
Q

decrease production of platelets

A

congenital:

  • fanconi
  • wiskott

acquired;

  • aplastic anaemia/ drugs
  • leukaemia
42
Q

normal platelet count with PC thrombocytopenia

A

dysfunction:

  • glanzzman
  • bernard soulier
  • uraemia
  • CARDIOPULMONARY BYPASS

vascular;

  • ED/ Marfans/ HHT
  • meningococcal
  • HSP, SLE
  • scurvy
43
Q

treatment of DIC

A

ICU and underlying cause

  • FFP, cryoprecipitate
  • platelets
  • AT3, protein C
44
Q

platelet levels

A

20– spontaneous bleeding
50– low spontaneous, risk with surgery/trauma
150–NOT spontaneous,
low risk with surgery/trauma

45
Q

PC haemophilia 1 yo

A

CAN’T WALK

46
Q

PC haemophilia neonate

A
  • ICH
  • post-circumicision bleeding
  • heel prick bleeding
47
Q

severe haemophilia

A
48
Q

moderate haemophilia

A

1-5%

post-trauma

49
Q

mild haemophilia

A

5-20%

post-surgical

50
Q

tx haemophilia

A

recombinant factor 8

plasma derived products

51
Q

complications of tx haemophilia

A
  1. cf inhibitors– thus increase the dose of recombo
  2. transufsion infections– HIV, hep B/C, prions
  3. vascular access
52
Q

age of VWD

A

adolescent/ adulthood

53
Q

tx of VWD

A
  1. desmopressin

2. plasma derived factor 8 (severe)

54
Q

TX NO NO’s in VWD

A

NO DESMOPRESSIN KIDS–> cerebral edema, seizures

NO CRYOPRECIPITATE

55
Q

Dx of VWD

A 
increase bleeding time
increase PTT
normal PT
abnormal Ristocetin test
Fe def anaemia
56
Q

thrombosis in kids

A

UNCOMMON
a/w the hyper coagulable states
PC: STROKE

PAEDS SC1 (13 decks)

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