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PAEDS SC1 > Haem > Flashcards

Haem Flashcards

(56 cards)

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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

Study These Flashcards
A

HbSS

= most severe

24
Q

sickle cell thal

25
sickle cell trait
HbS
26
acute anaemia in sickle cell
infection= haemolytic parvo= aplastic sequestration crisis: HSM,abdo pain,ciruclatory collapse
27
long term complications of sickle cell
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
diagnosis of sickle cell
[UK newborn screening] - Hb electrophoresis - Metabisulfite/ Sickledex - O2 sats, CXR, PaO2 - cultures
29
Prophylaxis of sickle cells
- vaccines: MenC, HiB, PCV - daily oral penicillin - daily folic acid
30
Acute tx sickle crisis
``` = AHOE Analgesia Hydration Oxygen Exchange transfusion ```
31
Who to give exchange transfusion for sickle cell
- acute chest syndrome - stroke - priapism
32
Chronic problems in sickle cell
- hydroxyurea | - BMT
33
Who to give BMT for sickle cell
- no response to hydroxyurea | - stroke
34
causes of aplastic anaemia
1. viruses 2. drugs 3. benzene + glue
35
fanconi anaemia
AR - short stature - skin hyper pigmentation - renal abnormalities - abnormal radius and thumb - micropthalmia
36
shwachman diamond syndrome
AR - bone marrow F - skeletal abnormalities - exocrine pancreas - --> isolated neutropenia - --> mild pancytopenia
37
gene in diamond blackfan
ribosomal protein
38
gene in shwachman diamond
SBDS
39
most common cause of thrombocytopenia in childhood
ITP
40
increase destruction of platelets
immune: - ITP - SLE - alloimmune neonatal thrombo nonimmune - HUS - TTP - CHD - DIC - Kassabach merritt - hyperplenism
41
decrease production of platelets
congenital: - fanconi - wiskott acquired; - aplastic anaemia/ drugs - leukaemia
42
normal platelet count with PC thrombocytopenia
dysfunction: - glanzzman - bernard soulier - uraemia - CARDIOPULMONARY BYPASS vascular; - ED/ Marfans/ HHT - meningococcal - HSP, SLE - scurvy
43
treatment of DIC
ICU and underlying cause - FFP, cryoprecipitate - platelets - AT3, protein C
44
platelet levels
20-- spontaneous bleeding 50-- low spontaneous, risk with surgery/trauma 150--NOT spontaneous, low risk with surgery/trauma
45
PC haemophilia 1 yo
CAN'T WALK
46
PC haemophilia neonate
- ICH - post-circumicision bleeding - heel prick bleeding
47
severe haemophilia
48
moderate haemophilia
1-5% | post-trauma
49
mild haemophilia
5-20% | post-surgical
50
tx haemophilia
recombinant factor 8 | plasma derived products
51
complications of tx haemophilia
1. cf inhibitors-- thus increase the dose of recombo 2. transufsion infections-- HIV, hep B/C, prions 3. vascular access
52
age of VWD
adolescent/ adulthood
53
tx of VWD
1. desmopressin | 2. plasma derived factor 8 (severe)
54
TX NO NO's in VWD
NO DESMOPRESSIN KIDS--> cerebral edema, seizures NO CRYOPRECIPITATE
55
Dx of VWD
``` increase bleeding time increase PTT normal PT abnormal Ristocetin test Fe def anaemia ```
56
thrombosis in kids
UNCOMMON a/w the hyper coagulable states PC: STROKE

PAEDS SC1 (13 decks)

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