Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Paeds > Haematology > Flashcards

Haematology Flashcards

1
Q

How is haemophilia inherited

A

X-linked recessive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Blood findings of haemophilia

A

Prolonged APTT
PT, bleeding time, thrombin time all normal`
Low of respective factors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

If a female has haemophilia what do they likely have

A

Turners

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What must be avoided in haemophilia

A

NSAIDS and aspirin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What advice should be given to parents with haemophilia

A

Present ASAO to hospital if slight trauma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

4 complications of haemophilia

A

Chronic arhtropathy
Compartment syndrome
Haematuria
HBV infection related to transfusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Male versus female with reduced factor 8

A

Male- haemophilia A
Female- vWD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Traetment of haemophilia chronically

A

Done at haemophilia centre- MDT approach
Mild to moderate haemophilia A- desmopressin
Severe- prophylactic factor replacement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Why is desmopressin used to treat haemophilia A

A

Stimulates F8 and VWF release

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Treatment of acute haemophilia

A

IV infusion of respective factor
Minor bleeds- raise to 30 % of normal
Major- raise to 100% and maintain at 30% for 2 weeks to prevent haemorrhage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

How is haemophilia graded

A

Mild will bleed after surgery (>5% of factor)
Moderate will bleed after minor trauma (1-5% of factor)
Severe will bleed spontaneously (<1% of factor)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is cows milk protein allergy

A

An allergic reaction to cows milk protein in formula fed milk but can occur in breatfed if protein present in mothers milk

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

How can IgE mediated allergy present

A

Urticaria
Facial swelling
Nausea
D&V
Itching
Sneezing
Rhinorrhoea

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

How can non-IgE mediated food allergy present

A

Erythema
Atopic eczema
GORD
Change in frequency of stools
Aversion of food
Infantile colic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Investigations for cows milk allergy and food allergy

A

Allergy focussed history- avoidance etc, have they trialled re-introducing allergen
Test 1- skin prick test
Test 2- total IgE or specific IgE (RAST)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is RAST

A

Another term for allergen specific IgE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Management of Cows milk allergy

A
  1. Avoid trigger
    If formula fed use hypoallergenic formula
    If breast fed exclude from diet (takes2-3 weeks tho)
    Can give calcium and vit D supplements
  2. must regularly monitor growth
  3. Reintroduce in 6-12 months using Milk Ladder
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Difference in formula given to formula fed babies CMPA

A

Mild and moderate- hydrolysed
Severe- amino acid based formula

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What can be given in CMPA to breast fed children

A

Give mum calcium and vit d supplements

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is immune thrombocytopenia

A

Immune mediated disease against glycoprotein IIb/IIIa or Ib-V-IX-complex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

How does ITP present in children

A

Recent infection/vaccine
Brusing
Petechial/purpuric rash
Rarely epistaxis or gingival bleeding

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Investigations for ITP

A

FBC- isolated thrombocytopenia
Blood film
Bone marrow examination if lymph node enlargement, splenomegaly, failure to respond to treatment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Management of ITP

A

Mainly benign and self limiting- resolves in 6-8 weeks
Treat only if major bleeding or persistent minor bleeding

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Managment of ITP if major bleeding

A

IVIG
Corticosteroids
Anti-Rhd
Plt transfusion if life threating

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Traetment for chronic ITP 1st line and second

A

Mycophenolate motefil
Rituximab
Eltrombopag
Second- splenectomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What is eltrombopag

A

A thrombopoietin agonist

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What is strawberry tongue seen in

A

Scarlet fever

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Presentation of scarlet fever

A

Tonsilitis
Fever
Malaise
Maculopapular rash

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What is rash like in scarlet fever

A

fine punctate erythema (‘pinhead’) which generally appears first on the torso and spares the palms and soles
Has rough sandpaper texture
Spares feet and hands

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Treatment for scarlet fever

A

Oral penicillin V for 10 days
Azithromycin if penicillin allergic
Can go back to school 24 hours after infection
Notifiable disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Most common complication of scarlet fever and other ones

A

Otitis media
Rheumatic fever
Acute glomerulonephritis
Invasive complications- bacteraemia, meningitis, necrotising fasciitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What are the different types of alpha thalassaemia and outcomes

A

Major with 4 alpha globulin missing- hydrops fetalis
HbH disease with 3 missing- mild/moderate anaemia with occasional transfusion
Alpha thalassaemia with 1 or 2 deleted- asymptomatic with mild/no anaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

How does HbH disease present

A

Hypochromic and microcytic anameia with splenomegaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What is thelarche

A

First stage of breast development

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

What is adrenarche

A

First stage of pubic development

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

How is precocious defined

A

The development of secondary sexual characteristics before 8 years in females and 9 years in males
Much more common in females

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

How is precocious puberty classified

A

Gonadotrophin dependent (central or true)- premature activation of hypothalamic-pituitary-gonadal axis. FSH and LH high
Gonadotrophin independent (pseudo, false)- due to excess sex hormones. FSH and LH low

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

How to determine cause of precocious puberty from testes

A

Bilaterally enlarged- gonadotrophin dependent
Unilaterally enlarged- gonadal tumour
Small- adrenal cause

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Faractures associated with non-accidental injury

A

Metaphyseal
Posterior rib
Fractures at different stages of healing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

What is a bucket handle fracture

A

Fracture of the metaphyseal corner in tibia
Seen in NAI

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Risk factors for rickets

A

Dietary feeding of calcium
Prolonged breastfeeding
Lack of sunlight
Unsupplemented cows milk formula

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Presentation of rickets

A

Aching bones and joints
Waddling gait
Kyphoscoliosis
Forehead bossing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

What is kyphoscoliosis

A

Deviation of the spine laterally (scoliosis) and anteriorly (kyphosis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Joint findings of rickets

A

Widening of joints
Rickety rosary- bony prominences at costochondral joint

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Examination findings of rickets

A

Harrison sulcus- protrusion of lower ribs anteriorly
Forehead bossing
Bowed legs
Kyphoscoliosis

46
Q

Genu varum versus genu valgum

A

Genu varum is bowed legs and seen in toddlers
Genu valgum is knock knees seen in older children

47
Q

What would cause a prolonged PT in ALL

A

DIC

48
Q

Presentation of kawasakis

A

Can present with anything but typically FEVERand CRASH
C- conjunctival injection
R- polymorphous rash
A- lymphAdenopathy
S- strawberry tongue
E-dema of hands and feet which can peel

49
Q

What is main complication of kawasakis

A

Coronary artery aneurysms

50
Q

Investigations and diagnosis of kawasakis

A

Mainly a clinical diagnosis but
FBC- high WCC
CRP and ESR
ECG and echo

51
Q

Treatment of kawasakis

A

High-dose aspirin and IVIG

52
Q

What is a white coloured nodule at the roof of the mouth

A

Epsteins pearl

53
Q

What is measles

A

RNA paramyxovirus

54
Q

How does measles present

A

Prodromal phase of fever, ill and conjunctivitis
Koplik spots in mouth which are white pots
Then get a maculopapular rash- starts behind the ears and spreads to whole body
Can peel but spares the hands and feet

55
Q

How to manage measles

A

Largely supportive with pain relief and lots of fluids
Stay away from school until 4 days after development of the rash
Inform that needs to go to hospital if develop SOB or neuro symptoms
Admit to hospital if immunocompromised, pregnant or an infant

56
Q

What is the most common complication of measles

A

Otitis media

57
Q

Main complications of measles

A

Otitis media
Pneumonia
Encephalitis
Subacute sclerosing panencephalitis

58
Q

When are stages children get vaccines

A

Birth (sometimes)
2 months
3 months
4 months
1 year
3 years
12-13
13-18

59
Q

What vaccine is given at birth

A

BcG sometimes
- born in areas of uk where high rate (40 in 1000)
- parent or grandparent born in country where (40 in 1000)
- live with or close contact of someone with infectious TB

60
Q

Vaccinations done at 2 months

A

6 in 1
Oral rotavirus
Men B

61
Q

Vaccinations done at 3 months

A

6 in 1
Oral rotavirus
PCV

62
Q

Vaccinations done at 4 months

A

6 in 1
Men B

63
Q

Vaccinations done at 1 year

A

Hib/Men C
PCV
Men B
MMR

64
Q

Vaccinations done at 3 years and 4 months

A

4 in 1- diptheria, tetanus, pertussis, polio
MMR

65
Q

What is given annually to children between 2 and 10

A

In september give Live flu vaccine

66
Q

What should be given in 12-13 years

A

HPV

67
Q

What is given at age 14

A

Tetanus, diptheria, polio
Men ACWY

68
Q

What is in the 6 in 1 vaccine

A

Parents- polio
Will- whooping cough
Immunise- influenza (Hib)
Toddlers- tetanus
Because- Hep B
Death- diphteria

69
Q

What vaccine is being given to those starting uni or aged 25 or under who never had it

A

Men ACWY

70
Q

What is hereditary angioedema

A

Condition where get recurrent bouts of subcut and submucosa
Caused by deficiency in C1-esterase inhibitor
Autosomal dominant

71
Q

What is treatment for hereditary angioedema

A

C1-esterase inhibitor like Cinryze
Things like steroids and antihistamines have no effect

72
Q

What causes traumatic bruises

A

Excessive coughing/vomiting
Get them around the face and neck

73
Q

Pathophysiology and inheritance of G6PD

A

X linked
Cells become sensitive to haemolysis in response to triggers including infection, certain drugs and flava beans

74
Q

Presentation of G6PD

A

Neonatal jaundice
Haemolysis after infection or new drugs
Dark urine and malaise

75
Q

Cause of HUS

A

E coli O157

76
Q

Presentation of HUS

A

Prodrome of fever, abdo pain and diarrhoea
Triad
- MAHA leading to pallor
- thrombocytopenia
- acute renal failure

77
Q

Presentation of TTP

A

MAHA
Thrombocytopenia
Acute renal failure
Fever
Neurological symptoms

78
Q

What is risk with influenza vaccine

A

If allergic to egg allergy

79
Q

What done if egg allergy and need the flu vaccine

A

If small allergy like HIVES do in Primary care
If anaphylaxis consider requirement

80
Q

Complications of SCD

A

Avascular necrosis in bones
Stroke
MI
Splenic sequestration
Chronic cholecystisis and gall stones

81
Q

What happens in splenic sequestration and long term consequences

A

Infarct in spleen leads to back up of blood->anaemia
Long term get auto-splenectomy as fibroses to become very small

82
Q

What are sickle cell crises

A

Get vaso occlusive crises due to acidosis, hypoxia or dehydration

83
Q

What are acute chest syndromes in children with SCD

A

Occlusion of pulmonary vessels results in build up of deoxygenated blood which sets up negative feedback loop as pulnomary arteries constrict in hypoxia

84
Q

Management of acute SCD crises

A

Analgesia
Oxygen
IV fluids

85
Q

What is given to SCD patients with recurrent admissions

A

Hydroxycarbamide/hydroxyurea

86
Q

Classify complications of SCD

A

Chronic haemolysis- anaemia, splenomegaly, chronic gallstones, aplastic crises
Vaso-occlusive crises- dactylitis, acute chest syndrome, priapism, stroke, splenic sequestration
Infection- susceptible to encapsulated bacteria
Long term problems - short stature, delayed puberty, HF

87
Q

What prophylactice antibiotics can be given in SCD

A

Penicillin V

88
Q

Blood findings of G6PD during crisis and not

A

Crisis
- increased haptoglobins
- increased LDH
- reticulocytosis
- hyperbilirbuninaemia
Normal
- all fine

89
Q

How is G6PD diagnosed

A

Enzyme assay 2-3 months after the crisis

90
Q

Differences between beta thalassaemia major and trait

A

Major is homozygous whereas trait is heterozygous
In major patients present age 3-6 months with faltering growth/signs of extramedullary haematopoiesis
In trait it is normally picked up on routine bloods

91
Q

Extramedullary haematopoieis signs

A

Frontal bossing
Maxillary overgrowth (protruding jaws)

92
Q

What are options if did not get MMR but want it at any time

A

Can have at any age
Get both doses 2 months apart

93
Q

What are risk factors for Vit K deficiency

A

Maternal anti-convulsant use
Breastfeeding

94
Q

What is vitmain K deficiency and why a problem

A

Children are born with very low vitamin K which can predispose to haemorrhagic disease of the newborn
As such children are given IM vit k at birth
Can present up to 8 weeks

95
Q

Alleles of SCD homozygous versus sickle cell trait

A

Homozygous- HbSS
Trait- HbAS

96
Q

Main way of differentiating between IgE and non-IgE mediated CMPA

A

IgE will present within 2 hours of milk

97
Q

Inheritance of mitochondrial disorders

A

Only via the maternal line

98
Q

What appears with red, ragged fibres on muscle biopsy

A

Mitochondrial disorder

99
Q

How do mitochondrial disorders present

A

Myopathy- muscle cramps and weakness

100
Q

What does PICA suggest in a child

A

IDA

101
Q

When does IDA present

A

Any time after 4 months as have stores for this long then you need supplements from pureed food etc
Risk factors are prolonged breastfeeding and delayed weaning

102
Q

Consequences of late weaning for kids

A

IDA
Vit D deficiency
Poor growth

103
Q

When treating AML what is most important thing to manage after starting chemo

A

TLS

104
Q

Differing between alpha beta thalassaemia trait

A

Beta- rise in HbA2 and HbF

105
Q

What vaccines is egg allergy a problem for

A

Influenza
Yellow fever

106
Q

Presentation of folate deficiency

A

Anaemia
Glossitis- smooth beefy red tongue
Angular stomatitis

107
Q

How can polycythaemia present in newborn

A

Blue peripheries
Lethary
Hypotonia
NEC
HCT over 0.65

108
Q

First intervention for SCD crisis

A

Analgesia

109
Q

When is exchange transfusion indicated for SCD crisis

A

Priapism
Stroke
Acute chest syndrome

110
Q

What is found on electrophoresis of sickle cell anaemia

A

HbS and HbF

Paeds (17 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions