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Paediatrics > Haematology/oncology > Flashcards

Haematology/oncology Flashcards

1
Q

What level of haemoglobin is equivalent to anaemia in a child?

A

<11g/dL (from birth until puberty)

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2
Q

Most common cause of iron deficiency anaemia?

A

Inadequate intake

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3
Q

Early signs of iron deficiency anaemia?

A

Dyspnoea and increasing tiredness
Slow/poor feeding
Pallor (pale conjunctiva, palmar creased, skin)

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4
Q

What is Pica?

A

This is a late sign of iron deficiency anaemia - it is inappropriate eating e.g. chalk, gravel, sand etc.

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5
Q

What blood tests would be performed and what would they show in iron deficiency anaemia?

A

FBC - decreased Hb, MCV, MCH and MCHC, increased platelet count
(WCC - eosinophils++ in hookworm infection)

Serum ferratin - reduced
Serum iron - <6-7 g/dl
Increased total iron binding capacity

Blood film = Microcytic and hypochromic RBC

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6
Q

Dietary advice given after diagnosis of iron deficiency anaemia?

A

Milk:
Breat milk - low Fe content but good absorption (50%)
Cows milk - high Fe content but low absorption (20%) - avoid if <1yr
Infant formula - supplement iron to increase intake

Eat vitamin C rich foods - fruit and dark green veg
Eat beef, lamb, liver, kidney (high in iron)

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7
Q

Further management of iron deficiency anaemia?

A

ferrous sulphate salt - 5mg/kg/day in 2-3 doses (do not exceed 200mg/day)

Continue for 3 months until Hb normalises - if not screen for thalassaemia

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8
Q

What type of brain tumour is most common in children?

A

Astrocytoma (40%)

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9
Q

What are the different types of brain tumour seen in children?

A

Astrocytoma = vary from benign to highly malignant (glioblastoma multiforme)

Medulloblastoma (20%) - form in the midline of the posterior fossa. May seed through CNS via CSF –> 20% have soinal metastases at presentation

Ependyoma (8%) - mostly in the posterior fossa and act like a medulloblastoma

Brainstem glioma (6%)

Craniopharyngoma - remnant of Rathke’s Pouch - not truly malgnnat but slowly invasive

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10
Q

Clinical presentation of a brain tumour?

A

Raised ICP +/- focal neurological signs

Spinal tumour mets –> back pain, paraesthesia, (chronic back pain should always be investigated by MRi in children)

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11
Q

Investigations and management for brain tumours

A

MRI head = gold standard

Surgery +/- radio/chemotherapy - primarily to reduce hydrocephalus, biopsy and maximal resection of tumour

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12
Q

What type of disorder is haemophilia?

A

An X-linked autosomal recessive of the clotting factors - 1/10,000 males
Haemophilia A = FVIII
Haemophilia B = FIX

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13
Q

Symptoms of Haemophilia?

A

Easily bruising, nose/gum bleeds
Bleeding in joints/muscle
Prolonged bleeding after trauma
Intracranial bleeds

Signs - haematuria, multiple bruises

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14
Q

Investigations for Haemophilia?

A

Bloods: FBC, U+E’s, LFTs, TFTs

CT head for intracranial bleeds

Clotting tests:
APTT = increased (intrinsic part of the clotting cascade)
PT/INR = normal
Thrombin time = normal
FVIII:C ratio = reduced (indicates severity)
vWF - important to differentiate from von Willebrand’s disease (similar results in clotting tests - not as severe)

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15
Q

Management of haemophilia?

A

Replace missing clotting factors when bleeds occur
Prophylactic clotting factors in severe disease

Desmopressin increases levels of vWF and FVIII:C therapeutically in haemophilia A (and von Willebrand disease)

Transexamic acid (TXA) for mouth bleeds

Lifestyle - avoid IM injections, contact sports, aspirin, NSAIDS

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16
Q

What is HSP?

A

Small vessel vasculitis often preceded by URTI (strep) - immune complexes IgG and IgA attacked by compliment cascade

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17
Q

Clinical features of HSP?

A
  1. Palpable papular non-blanching purpuric rash - usually buttocks and lower legs (trunk spared)
  2. Arthralgia/arthritis
  3. Abdominal pain (colicky)

Other features: Fever, UTI, oedema, melena,

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18
Q

Management of HSP?

A

Self-limiting - supportive
NSAIDS - for arthritic pain
Corticosteroids for arthritis/abdo pain

If severe - high dose steroids +/- cyclophosphamide

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19
Q

Complications of HSP?

A

Nephritic syndrome

Intussusception/appendicitis –> bowel perforation

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20
Q

What is the most common type of leukaemia in children?

A

Acute lymphoblastic leukemia (ALL) - 80% - peaks 2-5yrs

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21
Q

Clinical presentation of ALL?

A

General - anorexia, malaise
Bone marrow - anaemia (pallor, lethargy), infections (neutropenia), thrombocytopenia (bruising, rash) and bone pain
Reticulo-endothelial infiltration - hepatospenomegaly, mediastinal lymphadenopathy

22
Q

Symptoms typical of a relapse?

A

Organ filtration

  • -> CNS = headaches, nausea, vomiting
  • -> Testes = enlargement
23
Q

Diagnosis of leukaemia?

A

Bloods - FBC (low WCC, Hb, platelets +/- leukaemic blast cells)
Clotting sceen, ESR, U+Es

Bone marrow - leukaemic blast cells and leucocytosis = diagnostic, gives useful diagnostic info

24
Q

What management options are there before trying to reduce remission of leukaemia?

A

Correct underlying electrolyte/cellular inbalances i.e. blood transfusion for anaemia

Preventative measures:
Allopurinol - protects kidneys from rapid cell lysis
Prophylactic Co-trimoxozole to prevent pneumocystis carinii pneumonia

25
Q

Treatment course for ALL?

A

Induced remission = combinant chemotherapy - 95% success rate
Intensification - block of intense chemo to consolidate remission
CNS - intrathecal chemo (systemic chemo in circulation crosses into CNS poorly)

Continuing therapy - 3yrs (but low modest intensity)

Relapses? - High dose chemo and total body irradiation
Bone marrow transplantation

26
Q

What is the prognosis of leukaemia?

A

~80% survival

Poorer if: male, high WCC at diagnosis, high minimal residual disease (MRD), age <2 or >10yrs

27
Q

When would a headache be worst in the day in raised ICP?

A

In the morning (vomiting also worse first thing in the morning)

28
Q

What type of lymphoma is the most common?

A

Non-Hodgkins lymphoma (80%)

29
Q

How do lymphomas usually present? How else might they present?

A

Usually present with painless lymphadenopathy that is much firmer than it’s benign counterpart
can cause obstruction in airways or SVC

Can also develop ‘B’ symptoms - fever, itching, night sweats, weight loss

30
Q

Investigations into lymphomas?

A

Lymph node biopsy
Radiological assessment for staging
Bone marrow biopsy

PET scans to monitor treatment response when giving combinant chemo (+/- radiotherapy) in Hodgkins

31
Q

Pathophysiology of sickle cell disease?

A

HbA–> HbS
Polymerises to rigid tubular spiral bodies
Decreased RBC lifespan +/- trapped in microcirculation –>vaso-oclusive crisis

32
Q

How does vaso-oclusive crisis present clinically?

A

Pain+++ most commonly in the hands and back

Iachemia +/- necrosis/end organ damage
Can lead to acute chest syndrome (SOB, hypoxia, yellow sputum)

33
Q

What are the different types of sickle cell disease?

A

HbSS = true form (two copies of disease) - 50% mortality by 40 yrs
HbSC = One copy of HbS + another abnormal Hb (progressive retinopathy of adolescence +/- osteoporosis of hips/shoulders)
Sickle trait = carrier

34
Q

Diagnosis of sickle cell disease?

A

Guthrie (heel prick test) at 5 days = screening - will develop symtpoms at 6 months

Hb will usually be between 6-10g/dL

35
Q

Management of sickle cell disease?

A

Full imunisation is a priority
Penicillin V as prophylaxis against encapsulated pathogens
Folic acid daily

Lifestyle - avoid dehydration, extreme cold

36
Q

Complications of sickle cell disease?

A 
Encapsulate organism infection:
Pneumococcal sepsis (>3yrs most at risk)
Aplastic crisis (after parvovirus B19)
Vaso-oclusive crisis
Avascualr necrosis (hips and humerus)
Sequestrian crisis - sickle cells in spleen - can lead to infarcts (abdo pain, circulatory collapse)
Retinopathy
Acute chest syndrome (SOB, hypoxia, yellow sputum)
Priaprism (painful erection)
Stroke (5-10yr olds)
Hepatic pain
37
Q

Management of sickle cell disease crisis?

A

O2
IV fluids - aim for 150% of maintenance
Abx if infection (cephalosporin)
analgesia

38
Q

Where does the neural crest tissue arise from in neuroblastoma?

A

Adrenal medulla or the sympathetic nervous system

39
Q

Who does neuroblastoma usually affect?

A

Children under five

40
Q

What are common signs that the disease has progressed metastatically?

A

Bone marrow suppression –> malaise, pallor and weight loss
Bone pain
Hepatomegaly

41
Q

What might be found upon urinalysis in neuroblastoma? What other investigations should be done?

A

Catecholamines

Bloods
MRI/CT
Positive I-MIBG radioisotope scan +/- bone scan
Tumour and bone marrow biopsy

42
Q

In localised neuroblastoma what is the management? What further management is required in metastatic disease?

A

Surgery is the only management needed in localised disease

Chemo/radiotherapy are used in metastatic followed by immunotherapy (e.g. retenoic acid) for maintenance

43
Q

Why do haemoglobinopathies that affect the beta Hb chain (B thalassaemia and sickle cell) only present after 3-6 months

A

This is when the HbF present at birth is replaced by HbA

44
Q

Symptoms of B-thalassaemia?

A

Severe anaemia at 3-6 months
FTT
Jaundice

45
Q

Why might heterozygous B-thalassamia or minor a-thalassaemia be confused with iron deficiency?

A

It can result in hypochromic RBCs

46
Q

How might a foetus survive the pregnancy if it has the most severe form of a-thalassaemia?

A

Intra-uterine blood exchange transfusions

47
Q

What is Hb barts and how is it diagnosed?

A

Most severe form of a-thalassaemia (deletion mutation of all 4 a-chains) - results in foetal hydrops (severe odema and jaundice)
Can be detected mid trimester - Hb electrophoresis or Hb HPLC

48
Q

Management of symptomatic thalassaemia (i.e. results in severe anaemia)

A

Monthly exchange blood transfusions from 3-6 months of age (in utero and from birth in Hb barts)
+ iron chelation tehrapy (desferrioxamine SC) after 2-3 years to prevent iron over load

49
Q

When do 80% of wilms tumours present?

A

before the age of 5

V. rare to present after 10 yrs

50
Q

Investigations into wilms tumour?

A

CT/MRI will show mass distorting usual architecture of kidney and may show any metastases

51
Q

Management of Wilm’s tumour?

A
  1. Initial chemo
  2. Neohrectomy + histology for staging
  3. Chemotherapy +/- radiotherapy (reserved fro severe disease)

80% cure rate (60% if metastatic)

Paediatrics (14 decks)

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