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Paediatrics > Haematology > Flashcards

Haematology Flashcards

1
Q

What are the Hb types in normal children?

A

At birth HbF, HbA, HbA2
HbF lowers at 6 months
At this point Hbpathies will manifest

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2
Q

How do cell counts change with age?

A 
At birth (Hb 14-21, WBC 10-25, MCV 100-130, volume 80 mL/kg, 100 in preterm)
2 months (Hb 10, WBC 6-18, MCV 80-100)
Preterms at 2 months (Hb 6-9)
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3
Q

What is anemia?

A

Neonat <14
Infant <10
Child <11

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4
Q

What are the causes of anemia?

A 
Reduced production
-Ineffective erythropoiesis (IDA, Folic, ACD, CKD, Pb, MDS)
-Red cell aplasia (Parvo B19, congenital, AA, ALL)
Haemolysis
-Membranopathy (HS)
-Enzymopathy (G6PD)
-Hbpathy (Thal, SCD)
-Immune (HDN, AIHA)
Bleeding
-Malena (mucked)
bleeding diatheses (vWD)
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5
Q

How to investigate anemia?

A 
FBC - low Hb, MCV - low or high
Blood film
Retic count - low or normal
SBR
S Ferritin
HPLC
Parvovirus serology
Bone marrow aspirate
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6
Q

How to differentiate HPLC?

A

SCD - HbS, no HbA
beta major - only HbA
beta minor - high HbA2

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7
Q

What are the causes of IDA?

A

Diet (<8 mg/day)
Malabsoprtion
Blood loss

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8
Q

What are the dietary sources of iron for infants?

A 
Breast milk (low gross but 50% bioavailability )
Formula milk (high gross)
Cow milk (high cross but 10% bioavailability)
Complementary food (high in vit c low in tannin and phytates)
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9
Q

What are the features of IDA?

A

Tiredness, lethargy, pallor, pica, behavioural and intellectual oddities

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10
Q

How to manage IDA?

A 
Classify severity
Supply 1 mg/kg/day elemental iron
Diet change, feeding habit change
Non teeth staining sodium iron edetate
Continue for 3 months after normal Hb
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11
Q

What type of anemia does Parvo B19 cause?

A

Red cell aplasia in children already having inherited haemolytic disease

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12
Q

What is the mechanism of haemolytic anemia?

A

Red cell life span is 120 days

Bon marrow production rate < destruction rate

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13
Q

What are the features of haemolytic anemia?

A 
Anemia
HSM
Unconjugated bilirubin
Urine urobilinogen
Raised retic count (polychromasia)
Abnormal cells on bloodfilm
DAT positive in AIHA
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14
Q

What are the features of HS?

A 
AD, 25% no FHx
Spectrin, Ankyrin, Band 3 mutations
Spheroidal non deformable RBC
Extravascular hemolysis in spleen
Jaundice and anemia
Sudden crisis from B19
Gallstones and splenomegaly
Blood film + osmotic fragility
DAT to rule out AIHA
Give oral folic acid
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15
Q

What are the feature of G6PD?

A 
G6PD prevents oxidative damage to RBC
X linked recessive
Neonatal jaundice
Acute hemolytic anemia from infection, fava, quinines
Dark urine, jaundice, pallor
No need to transfuse
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16
Q

What are the features of SCD?

A 
Beta globin mutation valine replaces glutamine
HbSS, HbSC both have no HbA
Asymptomatic sickle trait
Sickling from HbS polymers
Anemia and jaundice
Hyposplenism causing infection risk
Vasoocclusive dactylitis, desaturation
Acute anemia from Parvo B19
Priapism 
Splenomegaly
Stoke, short stature, delayed puberty, adenoid hypertrophy
heart failure and cardiomegaly
Pigment stones
AKI CKD
Leg ulcers
Mx
Vaccinate and penicillin prophylaxis life long
Oral folic acid
Avoid stressors, prevent cold and dehydration
Exchange transfusion and analgesia
Early death
17
Q

What are the 5 components of haemostasis?

A 
Coagulation factors
Platelets
Endothelium
Coagulation inhibitors
Fibrinolytics
18
Q

What are the factors that limit coagulation?

A 
NO
PGI2
Antithrombin
Protein S
Thrombomodulin
19
Q

What are the 2 coagulation pathways?

A

Extrinsic (TF>Ca>VII>X>V>Thrombin>Fibrin+XIII)

Intrinsic (XII>XI>IX>VIII>X+V>Thrombin>Fibrin

20
Q

When do bleeding disorders present?

A

Neonate (ICH, circumcision bleeds)
Toddler (Hemophilias)
Adolescent (menorrhagia in vWD)

21
Q

How do bleeding orders present?

A

Mucosal (platelet, vWD)

Joints (Hemophilias)

22
Q

How to investigate bleeding disorders?

A 
FBC with BPx
BT
PT
APTT
TT
LFT
23
Q

What are the features of haemophilia?

A 
Commonest inherited bleeding disorders
X linked recessive
A = VIII, B=IX, A is 6 times more common
Rx = raise factor by 100% or 30%, maintain 50% for 2 weeks
Avoid IM, NSAIDs, Aspirin
DDAVP for mild A only, NOT for B
24
Q

What are the acquired coagulation disorders?

A

Haemorrhagic disorders of newborn (vit K deficiency)
Liver disease
ITP

25
Q

What is ITP?

A

Commonest cause of thrombocytopenia in kids
AntiPLT Ab with increased megakaryocytes in marrow
2-10 years after 2 weeks of viral infection
Petechiae/purpura/bruises/mucosal bleeds/ICH
Spontaneous resolution in 8 weeks
Dx of exclusion (ALL, AA, SLE, HUS, TTP)
Do BMBx if going to give steroids
Mx (prednisolone, IgG, AntiD, PLT transfusions)
20% will get low PLT even for 6 months (Chronic ITP)

Paediatrics (12 decks)

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