Haem Flashcards
blood cells in babies
Hb-- high at first, decrease by 2 months, decrease more if prime normal: Fe/ B12/folate preme: lower Fe/folate WBC: INCREASE platelets= normal
elemental Fe intake kids
1mg/kg/day
Fe body stores in kids
2.5g,
75%= blood
25%= ferritin and haemosiderin
breast milk iron
low, but 50% absorbed
formula milk
ADDED iron
cows milk iron
greater iron than breast, but 10% absorbed
cereals + Fe but…
1% absorbed
important foods questions to ask in anaemia
- breast or bottle
- cereal/ high fibre foods
- cows milk
- tea
when do patients with Fe def anaemia present
Fe= LESS than 6-7g/dL
treatment of Fe deficiency anaemia
Fe oral and dietary device
— continue Fe 3 most after Hb normal
Diamond Blackfan
congenital red cell aplasia
PC diamond blackfan
short stature and thumb abnormalities
Labs diamond blackfan
decrease Hb
decrease reticulocytes
decrease red cell precursors in marrow
normal billirubin
HbH
beta tetramers
HbBarts
gamma tetramers– hydrops fetalis
alpha thal major
how can a baby with hydrops fatalis survive?
intrauterine transfusions,
immediate post-delivery transfusions
age of presentation of beta than major
3-6 months
age of presentation of hereditary spherocytosis
childhood
diagnosis of hereditary spherocytosis
- blood smear
- osmotic fragility test
- DCT– exclude autoimmune HA
tx of hereditary spherocytosis
- folic acid
2. splenectomy– 7 YEARS OLD
NB pain crisis questions for sickle cell
- bone pain
- hand swelling
- chest pain
- abdo pain
sickle cell disease
normal ish HbS
retinopathy
and osteonecrosis: shoulder and hips
sickle cell anaemia
HbSS
= most severe
sickle cell thal
HbST
sickle cell trait
HbS
acute anaemia in sickle cell
infection= haemolytic
parvo= aplastic
sequestration crisis: HSM,abdo pain,ciruclatory collapse
long term complications of sickle cell
- short stature and delayed puberty
- education/ cognition
- adenotonsilar hypertrophy
- obstructive sleep apnea
- heart failure
- sttroke
- billirubin gallstones
- renal – haematuria, renal papillary necrosis
diagnosis of sickle cell
[UK newborn screening]
- Hb electrophoresis
- Metabisulfite/ Sickledex
- O2 sats, CXR, PaO2
- cultures
Prophylaxis of sickle cells
- vaccines: MenC, HiB, PCV
- daily oral penicillin
- daily folic acid
Acute tx sickle crisis
= AHOE Analgesia Hydration Oxygen Exchange transfusion
Who to give exchange transfusion for sickle cell
- acute chest syndrome
- stroke
- priapism
Chronic problems in sickle cell
- hydroxyurea
- BMT
Who to give BMT for sickle cell
- no response to hydroxyurea
- stroke
causes of aplastic anaemia
- viruses
- drugs
- benzene + glue
fanconi anaemia
AR
- short stature
- skin hyper pigmentation
- renal abnormalities
- abnormal radius and thumb
- micropthalmia
shwachman diamond syndrome
AR
- bone marrow F
- skeletal abnormalities
- exocrine pancreas
- –> isolated neutropenia
- –> mild pancytopenia
gene in diamond blackfan
ribosomal protein
gene in shwachman diamond
SBDS
most common cause of thrombocytopenia in childhood
ITP
increase destruction of platelets
immune:
- ITP
- SLE
- alloimmune neonatal thrombo
nonimmune
- HUS
- TTP
- CHD
- DIC
- Kassabach merritt
- hyperplenism
decrease production of platelets
congenital:
- fanconi
- wiskott
acquired;
- aplastic anaemia/ drugs
- leukaemia
normal platelet count with PC thrombocytopenia
dysfunction:
- glanzzman
- bernard soulier
- uraemia
- CARDIOPULMONARY BYPASS
vascular;
- ED/ Marfans/ HHT
- meningococcal
- HSP, SLE
- scurvy
treatment of DIC
ICU and underlying cause
- FFP, cryoprecipitate
- platelets
- AT3, protein C
platelet levels
20– spontaneous bleeding
50– low spontaneous, risk with surgery/trauma
150–NOT spontaneous,
low risk with surgery/trauma
PC haemophilia 1 yo
CAN’T WALK
PC haemophilia neonate
- ICH
- post-circumicision bleeding
- heel prick bleeding
severe haemophilia
moderate haemophilia
1-5%
post-trauma
mild haemophilia
5-20%
post-surgical
tx haemophilia
recombinant factor 8
plasma derived products
complications of tx haemophilia
- cf inhibitors– thus increase the dose of recombo
- transufsion infections– HIV, hep B/C, prions
- vascular access
age of VWD
adolescent/ adulthood
tx of VWD
- desmopressin
2. plasma derived factor 8 (severe)
TX NO NO’s in VWD
NO DESMOPRESSIN KIDS–> cerebral edema, seizures
NO CRYOPRECIPITATE
Dx of VWD
increase bleeding time increase PTT normal PT abnormal Ristocetin test Fe def anaemia
thrombosis in kids
UNCOMMON
a/w the hyper coagulable states
PC: STROKE
