More CP Flashcards
1
Q
What is haematocrit?
A
Vol % rbcs in blood
2
Q
Is haematocrit higher or lower in children?
A
Higher
3
Q
Zeta + epsilon = what sort of Hb?
A
Hb Gower-1
4
Q
Zeta + gamma =?
A
Hb Portland
5
Q
Alpha + epsilon
A
Hb gower-2
6
Q
Hb gower 1 is ?
A
Zeta + epsilon
7
Q
Hb Portland is ?
A
Zeta + gamma
8
Q
Hb gower 2 is ?
A
Alpha + epsilon
9
Q
When are Hbs gower1, portland and gower 2 present?
A
4-14 weeks gestation
10
Q
When does HbF become present?
A
more than 14 weeks gestation
11
Q
Difference in WBCs in children?
A
More lymphocytes
12
Q
Which Ig crosses the placenta?
A
IgG
13
Q
Which Igs in breast milk?
A
All
14
Q
When do babies start producing their own antibodies?
A
2-3 months
15
Q
When can babies have their own satisfactory immune responses?
A
6 months
16
Q
When do platelets reach adult numbers?
A
18 weeks gestation
17
Q
What are 2 features of gestational platelets?
A
Larger initially but reduce to adult size by birth
Hyperresponsive to vWF
18
Q
Which clotting factors are normal at birth? What are the rest of them like?
A
Fibrinogen, FV, VIII, XIII
Rest are reduced
19
Q
When do haemostatic parameters reach adult values?
A
6 months
20
Q
What is vitamin k dependent other than factors 10, 7, 9, 2?
A
Proteins S and C
21
Q
What does routine neonatal vit k injections prevent?
A
Haemorrhagic disease of the newborn
22
Q
What are 5 features of neonatal haemostasis?
A
Pro-coagulant proteins reduced (e.g. all the other factors) Reduced conc. coagulation inhibitors unique forms fibrinogen, plasminogen Raised D dimers and vWF Platelet aggregation differs
23
Q
What is acute leukaemia?
A
Accumulation of early myeloid or lymphoid precursors in BM, blood and other tissues
24
Q
What are the two main subgroups of acute leukaemia
A
Acute myeloid leukaemia and acute lymphoblastic leukaemia
25
How does acute leukaemia present?
Anaemia
Infection
Easy bruising or haemorrhage
Possibly organ infiltration by the leukaemia cells
26
FAB classification of leukaemia is ?
Morphological
27
WHO classification of leukaemia is ?
Risk adapted- takes into account prognosis
28
6 poor prognostic factors in ALL?
```
Age
High wcc
Male
Cytogenetic abnormalities (some)
Poor response to Rx
T cell -ALL and null-ALL
```
29
2 tests for diagnosis?
MABs
| Fluorescence activated cell sorting
30
Treatment for acute leukaemia that is shared with ALL and AML
Induction therapy to obtain remission
Consolidation cheomtherapy
Possible BM transplant
31
Additional Rx in ALL
Prophylaxis of meningeal leukaemia with intrathecal methotrexate and cranial irradiation
32
Siblings have a ?% change of being a bone marrow donor match?
25%
33
What is neutropenic sepsis?
All pts with acute leukaemia receiving intensive chemotherapy will become neutropenic for 10-21 days.
Overwhelming gram negative or positive infection= sepsis.
34
What is neutropenic fever?
Pyrexia in the presence of neutrophil count of less than 1.0/10^9/L
35
What is the management of neutropenic sepsis?
Immediate broad spectrum IV Abx (often Tazocin and gentamicin) given empirically- within 30 mins of temperature
36
Prevention of neutropenic sepsis?
Isolation
Prophylactic Abx
Granulocyte colony stimulating factors
Strict hand hygeine
37
Which element of virchow's triad contributes to arterial thrombosis?
Endothelial damage
38
Which elements of virchow's triad contributes to venous thrombosis?
Venous stasis and hypercoagulable state
39
What are arterial thrombi made of?
Platelets
40
What are venous thrombi made of?
Fibrin
41
Definition of a hospital acquired VTE?
Within 90 days discharge
42
Having considered the risks of bleeding and VTE, if there is a risk of bleeding how can you still help prevent a VTE?
Mechanical methods eg stockings
43
When must you reassess risks of bleeding and VTE?
Within 24hrs admission and whenever the clinical situation changes
44
True of false antiplatelets are adequate prophylaxis against VTE alone?
False, also mobilisation and hydration
45
4 pharmacological methods of prevention
1. low molecular weight heparin (antithrombin and mostly anti-Xa)
2. Fondaparinux
3. Rivaroxaban/apixaban (direct factor Xa inhibitor)
4. Dabigatran (direct thrombin inhibitor)
46
What is the exclusion test for a VTE?
Use Wells score and D-dimer in an algorithm
47
What are D-dimers?
Breakdown product of fibrin clots
48
Scans for:
VTE
PE
VTE- US
| PE- CT, VQ- compare inhaled and injected isotopes. Mismatch is diagnostic of a PE
49
Advantages of Rivaroxaban/apixaban/dabigatran over heparin?
Dose uniform rather than done by weight so monitoring not needed
Rapid onset of action
Oral route
50
Management for a first episode of DVT or PE
Treat for 3-6 months if there is a temporary risk factor such as pregnancy
51
What is warfarin target INR?
2.5
52
Management for recurrent episodes of cardiothoracic emboli?
Long term anticoagulation
53
Management of a proximal DVT or PE that has occured in the absence of a reversible risk factor?
Consider long term anticoagulation
54
Management if they have recurrent VTEs even on therapeutic anticoagulants?
Increase target INR to 3.5 for warfarin
55
What is a thrombophilia (2 definitions)
1. familial/acquired predisposition to thrombosis
| 2. patients who develop VTE spontaneously, extra bad or recurrently at an early age
56
What is the action of protein C and what is it activated by?
Thrombin-thrombomodulin complex activates it (thrombomodulin is on the vessel wall), protein C-protein S complex breaks down factor Va and VIIIa resulting in coagulation.
57
In factor V leiden, factor V is resistant to what?
Protein C
58
When is factor V leiden asymptomatic?
Heterozygous
59
Are screening tests valuable in factor V leiden?
Debatable as often wouldn't change management. Doesn't increase risk of recurrent thrombosis.
60
What is prothrombin 20210A?
Increased prothrombin levels, 3 fold increase in VTE
61
What is the dx criteria for antiphospholipid syndrome?
Presence of antiphospholipid antibodies on at least 2 occasions 8-12 weeks apart in assoc with VT, AT or recurrent foetal loss (more than 2)
62
What might antiphospholipid syndrome be secondary to?
SLE, conn tissue disorders etc.
63
When should you screen for thrombophilia?
In younger patients, positive Fhx and if management will change.
64
Folate and B12 deficiency cause what type of anaemia?
Megaloblastic macrocytic
65
Main cause and other 4 causes of folate deficiency?
```
Dietary main cause
Alcohol
Malabsorption
Increased usage
Drugs
```
66
What is iron transported in?
Transferritin
67
What is iron stored in?
Ferritin/haemosiderin
68
2 causes of microcytic anaemia
Fe deficiency
| Hb disorders
69
3 causes of iron deficiency
```
Blood loss
Increased demand (pregnancy,growth)
Redued intake (diet, malabsorption)
```
70
Rx for iron deficiency
```
Oral iron (although constipation and abdo pain)
IM iron (although painful and skin colour change)
IV iron probably best
```
71
How do B12 and folate deficiencies cause anaemia?
B12 required for cell folate generation, folate required for nitrogenous base synthesis which make up DNA
72
What foods is B12 from?
Animal sources
73
Describe the absorption of B12
Gastric parietal cells absorb
Bound to intrinsic factor
Receptors in terminal ileum
74
How long are B12 stores sufficient for?
Years
75
3 causes of B12 deficiency
```
Nutritional (vegan)
Gastric problems (pernicious anaemia, gastrectomy)
Small intestine problems (resection, crohns, stagnant loops, jejunal diverticulitis, tropical sprue, fish tapeworm)
```
76
Severe B12/folate deficiency can result in...?
pancytopenia
77
What neurological problem can B12 deficiency result in?
Sub acute degeneration of the spinal cord
78
Rx B12+/folate deficiency?
Give B12 and folate until B12 deficiency is excluded as folic acid in isolation can make B12 def worse.
79
7 cases haemolytic anaemia
```
Haemoglobinopathy
Enzyme defects (G6PD)
Hereditary spherocytosis/elliptocytosis
Antibodies to RBC
Drubs/toxins
Heart valves
Vascular
```
80
What is the name of haemolytic anaemia in its own right?
autoimmune haemolytic anaemia (AIHA)
81
Rx AIHA
steroids and immune suppression
82
What sort of aneamia is anaemia of chronic disease?
Normocytic
83
What is the MCV in anaemia of chronic disease?
Normal
84
3 reasons anaemia of chronic disease happens?
Abnormal Fe metabolism
Poor EPO response
Poor BM response
85
11 causes of thrombocytopenia
```
drugs alcohol toxins
infection (sepsis, EBV, HIV)
Other autoimmune
DIC
Liver disease
Hypersplenism
Congenital
Pregnancy
Haematological/marrow diseases
Immune thrombocytopenic purpura
Thrombotic thrombocytopenic purpura
```
86
Immune thrombocytopenic purpura cause in children
Post viral- self limiting
87
Rx immune throbocytopenic purpura
Steroids
Thrombomimetics
IV Igs (blocks spleen)
Splenectomy
88
What is the role of the spleen in terms of platelets?
Removes them
89
How does Immune thrombocytopenic purpura present?
Bruising, petechiae, bleeding
| Splenomegaly
90
Cause of thrombotic thrombocytopenic purpura
Immune (ADAMTS-13, vWF)
91
Presentation of thrombotic thrombocytopenic purpura
Thrombocytopenia +
| Fever, neurological, haemolysis
92
Rx thrombotic thrombocytopenic purpura
Plasma exchange
| Steroids
