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Haematology Flashcards

1
Q

How is haemophilia inherited

A

X-linked recessive

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2
Q

Blood findings of haemophilia

A

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

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3
Q

If a female has haemophilia what do they likely have

A

Turners

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4
Q

What must be avoided in haemophilia

A

NSAIDS and aspirin

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5
Q

What advice should be given to parents with haemophilia

A

Present ASAO to hospital if slight trauma

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6
Q

4 complications of haemophilia

A

Chronic arhtropathy
Compartment syndrome
Haematuria
HBV infection related to transfusion

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7
Q

Male versus female with reduced factor 8

A

Male- haemophilia A
Female- vWD

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8
Q

Traetment of haemophilia chronically

A

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

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9
Q

Why is desmopressin used to treat haemophilia A

A

Stimulates F8 and VWF release

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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

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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)

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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

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13
Q

How can IgE mediated allergy present

A

Urticaria
Facial swelling
Nausea
D&V
Itching
Sneezing
Rhinorrhoea

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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

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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)

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16
Q

What is RAST

A

Another term for allergen specific IgE

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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
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18
Q

Difference in formula given to formula fed babies CMPA

A

Mild and moderate- hydrolysed
Severe- amino acid based formula

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19
Q

What can be given in CMPA to breast fed children

A

Give mum calcium and vit d supplements

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20
Q

What is immune thrombocytopenia

A

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

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21
Q

How does ITP present in children

A

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

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22
Q

Investigations for ITP

A

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

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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

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24
Q

Managment of ITP if major bleeding

A

IVIG
Corticosteroids
Anti-Rhd
Plt transfusion if life threating

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25
Traetment for chronic ITP 1st line and second
Mycophenolate motefil Rituximab Eltrombopag Second- splenectomy
26
What is eltrombopag
A thrombopoietin agonist
27
What is strawberry tongue seen in
Scarlet fever
28
Presentation of scarlet fever
Tonsilitis Fever Malaise Maculopapular rash
29
What is rash like in scarlet fever
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
30
Treatment for scarlet fever
Oral penicillin V for 10 days Azithromycin if penicillin allergic Can go back to school 24 hours after infection Notifiable disease
31
Most common complication of scarlet fever and other ones
Otitis media Rheumatic fever Acute glomerulonephritis Invasive complications- bacteraemia, meningitis, necrotising fasciitis
32
What are the different types of alpha thalassaemia and outcomes
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
33
How does HbH disease present
Hypochromic and microcytic anameia with splenomegaly
34
What is thelarche
First stage of breast development
35
What is adrenarche
First stage of pubic development
36
How is precocious defined
The development of secondary sexual characteristics before 8 years in females and 9 years in males Much more common in females
37
How is precocious puberty classified
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
38
How to determine cause of precocious puberty from testes
Bilaterally enlarged- gonadotrophin dependent Unilaterally enlarged- gonadal tumour Small- adrenal cause
39
Faractures associated with non-accidental injury
Metaphyseal Posterior rib Fractures at different stages of healing
40
What is a bucket handle fracture
Fracture of the metaphyseal corner in tibia Seen in NAI
41
Risk factors for rickets
Dietary feeding of calcium Prolonged breastfeeding Lack of sunlight Unsupplemented cows milk formula
42
Presentation of rickets
Aching bones and joints Waddling gait Kyphoscoliosis Forehead bossing
43
What is kyphoscoliosis
Deviation of the spine laterally (scoliosis) and anteriorly (kyphosis)
44
Joint findings of rickets
Widening of joints Rickety rosary- bony prominences at costochondral joint
45
Examination findings of rickets
Harrison sulcus- protrusion of lower ribs anteriorly Forehead bossing Bowed legs Kyphoscoliosis
46
Genu varum versus genu valgum
Genu varum is bowed legs and seen in toddlers Genu valgum is knock knees seen in older children
47
What would cause a prolonged PT in ALL
DIC
48
Presentation of kawasakis
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
What is main complication of kawasakis
Coronary artery aneurysms
50
Investigations and diagnosis of kawasakis
Mainly a clinical diagnosis but FBC- high WCC CRP and ESR ECG and echo
51
Treatment of kawasakis
High-dose aspirin and IVIG
52
What is a white coloured nodule at the roof of the mouth
Epsteins pearl
53
What is measles
RNA paramyxovirus
54
How does measles present
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
How to manage measles
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
What is the most common complication of measles
Otitis media
57
Main complications of measles
Otitis media Pneumonia Encephalitis Subacute sclerosing panencephalitis
58
When are stages children get vaccines
Birth (sometimes) 2 months 3 months 4 months 1 year 3 years 12-13 13-18
59
What vaccine is given at birth
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
Vaccinations done at 2 months
6 in 1 Oral rotavirus Men B
61
Vaccinations done at 3 months
6 in 1 Oral rotavirus PCV
62
Vaccinations done at 4 months
6 in 1 Men B
63
Vaccinations done at 1 year
Hib/Men C PCV Men B MMR
64
Vaccinations done at 3 years and 4 months
4 in 1- diptheria, tetanus, pertussis, polio MMR
65
What is given annually to children between 2 and 10
In september give Live flu vaccine
66
What should be given in 12-13 years
HPV
67
What is given at age 14
Tetanus, diptheria, polio Men ACWY
68
What is in the 6 in 1 vaccine
Parents- polio Will- whooping cough Immunise- influenza (Hib) Toddlers- tetanus Because- Hep B Death- diphteria
69
What vaccine is being given to those starting uni or aged 25 or under who never had it
Men ACWY
70
What is hereditary angioedema
Condition where get recurrent bouts of subcut and submucosa Caused by deficiency in C1-esterase inhibitor Autosomal dominant
71
What is treatment for hereditary angioedema
C1-esterase inhibitor like Cinryze Things like steroids and antihistamines have no effect
72
What causes traumatic bruises
Excessive coughing/vomiting Get them around the face and neck
73
Pathophysiology and inheritance of G6PD
X linked Cells become sensitive to haemolysis in response to triggers including infection, certain drugs and flava beans
74
Presentation of G6PD
Neonatal jaundice Haemolysis after infection or new drugs Dark urine and malaise
75
Cause of HUS
E coli O157
76
Presentation of HUS
Prodrome of fever, abdo pain and diarrhoea Triad - MAHA leading to pallor - thrombocytopenia - acute renal failure
77
Presentation of TTP
MAHA Thrombocytopenia Acute renal failure Fever Neurological symptoms
78
What is risk with influenza vaccine
If allergic to egg allergy
79
What done if egg allergy and need the flu vaccine
If small allergy like HIVES do in Primary care If anaphylaxis consider requirement
80
Complications of SCD
Avascular necrosis in bones Stroke MI Splenic sequestration Chronic cholecystisis and gall stones
81
What happens in splenic sequestration and long term consequences
Infarct in spleen leads to back up of blood->anaemia Long term get auto-splenectomy as fibroses to become very small
82
What are sickle cell crises
Get vaso occlusive crises due to acidosis, hypoxia or dehydration
83
What are acute chest syndromes in children with SCD
Occlusion of pulmonary vessels results in build up of deoxygenated blood which sets up negative feedback loop as pulnomary arteries constrict in hypoxia
84
Management of acute SCD crises
Analgesia Oxygen IV fluids
85
What is given to SCD patients with recurrent admissions
Hydroxycarbamide/hydroxyurea
86
Classify complications of SCD
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
What prophylactice antibiotics can be given in SCD
Penicillin V
88
Blood findings of G6PD during crisis and not
Crisis - increased haptoglobins - increased LDH - reticulocytosis - hyperbilirbuninaemia Normal - all fine
89
How is G6PD diagnosed
Enzyme assay 2-3 months after the crisis
90
Differences between beta thalassaemia major and trait
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
Extramedullary haematopoieis signs
Frontal bossing Maxillary overgrowth (protruding jaws)
92
What are options if did not get MMR but want it at any time
Can have at any age Get both doses 2 months apart
93
What are risk factors for Vit K deficiency
Maternal anti-convulsant use Breastfeeding
94
What is vitmain K deficiency and why a problem
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
Alleles of SCD homozygous versus sickle cell trait
Homozygous- HbSS Trait- HbAS
96
Main way of differentiating between IgE and non-IgE mediated CMPA
IgE will present within 2 hours of milk
97
Inheritance of mitochondrial disorders
Only via the maternal line
98
What appears with red, ragged fibres on muscle biopsy
Mitochondrial disorder
99
How do mitochondrial disorders present
Myopathy- muscle cramps and weakness
100
What does PICA suggest in a child
IDA
101
When does IDA present
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
Consequences of late weaning for kids
IDA Vit D deficiency Poor growth
103
When treating AML what is most important thing to manage after starting chemo
TLS
104
Differing between alpha beta thalassaemia trait
Beta- rise in HbA2 and HbF
105
What vaccines is egg allergy a problem for
Influenza Yellow fever
106
Presentation of folate deficiency
Anaemia Glossitis- smooth beefy red tongue Angular stomatitis
107
How can polycythaemia present in newborn
Blue peripheries Lethary Hypotonia NEC HCT over 0.65
108
First intervention for SCD crisis
Analgesia
109
When is exchange transfusion indicated for SCD crisis
Priapism Stroke Acute chest syndrome
110
What is found on electrophoresis of sickle cell anaemia
HbS and HbF

Paeds (17 decks)

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