Brainscape's Knowledge GenomeTM

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


See full index

Year 4 Paeds Conditions > Haematological/Oncological > Flashcards

Haematological/Oncological Flashcards

1
Q

why is paeds pt more prone to iron deficiency anaemia?

A

iron is required for growth and so early childhood will require a large amount of iron - children might not eat enough iron to cover the iron requirement

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

how can cow’s milk cause iron deficiency anaemia in children?

A

if whole cow’s milk is given too early - induce microscopic bleeding due to allergic reaction

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

aetiology of iron - deficiency in children

A

dietary iron deficiency

whole cow’s milk allergy - leads to microscopic bleeding

infections - malaria, TB, HIV and helmith infection

inherited conditions eg sickle cell, diamond-blackfan syndrome, beta thalassaemia

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

what is the range of HB in order to be considered to be anaemic in 1/2 - 5 yrs old

A

<11

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

what is the range of HB in order to be considered to be anaemic in 5- 11 yrs old

A

< 11.5

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

what is the range of HB in order to be considered to be anaemic in 12- 14 yrs old

A

< 12

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

symptoms and signs of iron-deficiency of anaemia

A

can be asymptomatic

pallor 
fatigue 
SOB/IWOB 
Tachycardia 
gallop rhythm 
systolic flow murmur 
cardiomegaly and hepatomegaly

failure to thrive

affect growth

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

things to consider asking during history taking ?

A

ethnic origin - african people - inc risk of sickle cell disease, malaria, diamond-blackfan syndrome, mediterranean people - inc risk of beta thalassemia

recent travel - infection and parasitic infection

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

investigation for iron-deficiency anaemia

A

FBC - shows microcytic, hypochromic and poikilocytosis

ferritin - reduced
folate, B12 - for macrocytic anaemia
coombs test - haemolytic anaemia
blood film - to exclude other types of anaemia eg sickle cells

TFT - hypothyroidism

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

management of iron deficiency anaemia

A

PO iron salts over 2-3 months - sytron (sodium feredetate)

iron transfusion

inc dietary intake

limit cow’s milk to 1 pint only

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

how would you prevent iron deficiency anaemia?

A

breast milk somewhat protective - low iron

no unmodified cow’s milk in first year of life

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

complications or IDA

A

cognitive impairment - cognitive and behavioural impairment, attention deficit

impaired muscle performance

developmental delay
high output heart failure
tran

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

what is the most common solid brain tumor in children?

A

70-80% infratentorial (glial tumors, medulloblastoma) or mid-line (Germ Cell tumor, craniopharyngioma - tumor of the pituitary gland

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

what are some clinical features for childhood brain tumour?

A 
headache 
N+V 
visual distrubanec - double vision, papilloedema, squint 
hydorcephalus 
diabetes inspidius 
seizures 
gorwth restictions 
gait/co-ordination problems 
CN palsies
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

ix for childhood brain tumor

A

urgent referral 48 hours if suspected brian or CNS tumours

MRI and CT - MRI preferred, better imaging for soft tissue but takes longer and may require sedation, so CT is more often used first

biopsy

others ix –> hearing test and pituitary function, CSF analysis

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

what brain tumor could inc hCG and AFP in CSF suggest ?

A

pineal tumours

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

mx of childhood brain tumours

A 
urgent referral 
surgical resection 
radio
chem 
follow up - every 6 m for first 2 years
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what is haemophilia A

A

it is factor 8 deficiency - can be congenital or acquired

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

what is haemophilia B

A

it is factor 9 deficiency - can be congenital or acquired

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

how is haemophilia pass down genetically

A

X-linked recessive

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

what is considered a mild hemophilia A or B

A

it is when factor 8/9 is between 5-40% of normal level

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

what is considered a moderate hemophilia A or B

A

it is when factor 8/9 is between 1-5% of normal level

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

what is considered a severe hemophilia A or B

A

it is when factor 8/9 is <1% of normal level

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

what are the symptoms of haemophilia

A

mild - bleeding after surgery eg circumcision

moderate - bleeding after minor trauma –> after hell stick test

severe - spontaneous bleeding into joints/muscle or intra-cranial bleeding

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

Investigation for haemophilia

A

• Activated partial Thromboplastin time (aPTT) – inc
o use to test intrinsic pathway
• Factor 8 assay or 9 – level dec
• normal prothrombin time
o use to test extrinsic and common pathway
• plasma von Willebrand factor assay – normal to exclude VWF deficiency
• other factors assay – to exclude problems elsewhere
• FBC – normal, low after bleeding
• LFT – factor deficit can be caused by liver disease
• FHx or carrier – test baby’s gender from 9 wks, if male: plan safe delivery
o can do amniocentesis or CVS to confirm diagnosis

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

management of haemophilia

A

o Avoid NSAIDs and IM injections
o Regular or prophylactic factor replacement therapy  to those with severe haemophilia
 Haemophilia A every 48hrs, for haemophilia B 2x week
 Implantable port (from 2yrs) at home

o Minor bleeding – eg minor surgery/dential extraction
 Pressure, elevation
 IV desmopressin (0.3µg/kg/12hrs over 20min) to factor 8

o Major bleeding (haemarthrosis etc.)
 Aim to factor 8 to 50% of normal

o Life-threatening bleeding (e.g., airway obstructing)
 Need 100% levels factor 8 (recombinant factor 8)

IV desmopressin does not work for hemophilia B

27
Q

what is Henoch-Schoenlein Purpura

A

small vessel vasculitis - characterized by tissue deposition of IgA containing immune complexes within affected organs

28
Q

pathophysiology of Henoch-Schoenlein Purpura

A

small vessel leukocytoclastic (neurophil debris) vasculitis

  • characterized by tissue deposition of IgA containing immune complexes within affected organs
  • skin biopsy of lesions contains neutrophils and monocytes
  • fluorescence microscopy of kidney –> IgA, C3, fibrin mainly
29
Q

clinical features of Henoch-Scholenin Purpura

A

Tetrad = rash (palpable purpura and non-blanching, usually lower extremities), abdo pain, arthritis/arthralgia, glomerulonephritis

other signs of renal disease - asymptomatic haematuria +/- proteinurua, if severe –> rapid progressive nephritis, nephrotic syndrome and renal failure

scrotal pain or swelling

headache

seizures

pulmonary haemorrhage

30
Q

investigation of HSP

A

urinalysis - assess renal involvment

24 hour urine for protein - raised due to renal involvement

U&Es + creatinine –> kidney function

serum IgA - maybe elevated

coag - should be normal in HSP, used to exclude other causes

skin biopsy - will show IgA involvement

Renal biopsy - will show IgA involvement

31
Q

management. of HSP

A

mid + no renal involvment –> analgesia + supportive

if severe oedema/severe abdo pain/scortal swelling –> prednisolone

if kidney involvement –> prednisolone

if rapid progressive nephritis –> IV methyprednisolone + cyclophosphamide/azathioprine

if severe kidney damage –> renal transplant

32
Q

definition of leukaemia

A

• malignant proliferation of white cell precursors in bone marrow

33
Q

what is the most common childhood cancer

A

Acute lymophoblastic leukaemia

34
Q

pathology of leukaemia and lymphoma?

A

• WBC (T, B etc), RBC and platelets are all made form bone marrows in long bones
• stem cells produce cell precursors which can either mature through lymphoid cell line  makes T and B cells or myeloid cell line which matures the other WBCs, RBCs and platelets etc.
• in both leukaemia and lymphoma, genetics changes occurs in the precursors leading to malignant proliferations of cells.
o leukaemia  2 types as can affect both lymphoid and myeloid cell lines  lymphoblastic or myelogenous
o lymphoma  only affect lymphoid cell line  T and B cells

35
Q

clinical features of ALL?

A
  • insidious onset
  • anorexia, irritability and lethargy
  • bone pain
  • mucous membrane bleeding
  • Petechiae
  • as bone marrow fails due to malignant tumours replacing normal stem cells  Pallor, fever, Petechiae
  • lymphadenopathy
  • splenomegaly
36
Q

Ix for leukaemia?

A
  • FBC with differentials  inc WCC, dec Hb (anaemia), neurorpenia,
  • peripheral blood smear  thrombocytopenia +/- blast cells
  • U&Es  elevated Ca2+, K+, phosphorus, uric and lactic acid
  • LFT  liver can be affected
  • diagnosis  examination of bone marrow (bone marrow aspiration + trephine biopsy  replaced by leukaemic lymphoblasts
37
Q

mx for leukaemia

A
  • chemo, prophylactic Radiotherapy to CNS and systemic radiotherapy for 2-3 yrs
  • prophylactic ABx + antiviral
  • prophylactic colony-stimulating factors (to stimulate neutrophiles)
  • follow for relapse  intensive retreatment
38
Q

which cells are affected in lymphoma?

A

B cells

39
Q

what is associated to inc risk for lymphoma?

A
  • EBV infection associated to both

* NHL associated to other autoimmune disorders

40
Q

clinical features for lymphoma?

A
  • most commonly present with painless cervical +/- supraclavicular lymphadenopathy in a young adult/ adolescence/late childhood
  • B symptoms  fever, night sweats, weight loss occurs in 30% of pts
  • often less common presentation  dyspnoea &cough (mediastina adenopathy), SVC obstruction, abdo pain
41
Q

ix for lymphoma

A
  • FBC with differentials  low Hb and platelets, WCC can be high or low
  • LFT  can be affected
  • U&Es + creatinine  can be affected
  • ESR  elevated
  • CXR  if dyspnoea and maybe able to see mediastinum adenopathy
  • PET-CT scan  affected area will light up
  • contrast CT neck, chest and abdo/pelvis  may show enlarged lympho nodes and other sites of disease
  • excisional lymph node biopsy  Hodgkin’s cell (Reed-Sternberg cell)
  • bone marrow biopsy can be consider
  • for NHL  skin biopsy shows cutaneous T cells
42
Q

mx for lymphoma

A
  • chemo +/- radiotherapy for both

* NHL  R-CHOP-21 (rituximab + cyclophosphamide+ doxorubicin + vincristine + prednisolone for 21 days)

43
Q

what is sickle cell anemia

A

a single autosomal recessive gene mutation in the beta chain of hemoglobin resulting in the production of sickle cell hemoglobin

44
Q

pathophysiology of sickle cell anaemia

A

in a hypoxic and acidotic environment, HbS becomes rigid and change to a crescent shape and so unable to carry O2 properly

the deformed HbS can also occlude small vessels or stick to the endothelium of large vessels to slow blood flow

45
Q

which race is most commonly carrier of the sickle cell gene?

A

black population - approx 8% of the entire black population

46
Q

what are some clinical features of sickle cell anaemia

A 
persistent pain in abdo, bone and chest 
dactylitis 
fever 
pneumonia-like symptoms 
chronic haemolytic anaemia 
visual floaters 
tachycardia, pallor, jaundice, tachypnoea, lethargy

can have acute sickle crisis

47
Q

ix for sickle cell anaemia

A
  • DNA-based assay  replacement of both beta haemoglobin subunits with HbS = diagnostics
  • cellulose acetate electrophoresis  sickle cell anaemia 75-95% HbS
  • high performance liquid chromatography
  • FBC  anaemia
  • peripheral blood smear  presence of nucleated RBC, sickle-shaped cells and Howell-Jolly bodies
48
Q

Mx of sickle cell anaemia

A

• treatment of crisis/ acute chest syndrome  analgesia, Abx, antihistamine, warmth, fluids + O2 if needed
o blood transfusion if life-threatening
• ongoing management  management of pain (hydroxycarbamide to dec frequency of pain episode) , pneumococcal immunisation, prophylactic Penicillin,

49
Q

what is neuroblastoma

A

tumour develop from nerve cells in foetus (neuroblast) which affects the sympathetic nervous system

50
Q

which organ does neuroblastoma normally affect?

A

mostly in adrenal gland (due to sympathetic nervous system)

but can start/spread to chest, abdo, spine, spinal cord

51
Q

clinical features of neuroblastoma

A

abdo mass, pain
FTT
bleeding
hard, painless neck mass
irritable, fever, dec appetite
leg weakness/numbness (spinal cord involvement)
if spread (50%) - bone pain, anaemia, weight loss, sweating, watery diarrhoea

52
Q

mx of nueroblastoma

A

chemo/radio
surgery (unless at spinal core)
stem cell transplant

53
Q

peak incidence of neuroblastoma

A

2 yrs old

54
Q

pathophysiology of thalassaemia

A

• inherited microcytic anaemia caused by mutations of the beta-globin gene leading to dec or absent synthesis of beta-globin  ineffective erythropoiesis.

55
Q

which races are most susceptible to thalassaemia

A

• ß-thalassaemia most common; Southeast Asian, middle east, Mediterranean backgrounds.

56
Q

clinical features of thalassaemia trait

A

• Heterozygous ß-thalassaemia = thalassaemia trait

o Mild anaemia or asymptomatic

57
Q

clinical features of thalassaemia major

A

• Homozygous thalassaemia
o Severe haemolytic anaemia
o Compensatory BM hyperplasia  characteristic overgrowth of facial + skull bones
o Haemosiderosis (iron overload) + cardiomyopathy, DM + skin pigmentation due to repeated blood transfusion.

o lethargy, abdo distension, FTT, low height and weight
o pallor
o spinal changes (iron overload osteopenia)
o hepatosplenomegaly

58
Q

ix for thalassaemia

A
  • Hypochromic, microcytic anaemia in thalassaemia trait may be confused with iron def.
  • Homozygous: severe hypochromic microcytic anaemia, bizarre fragmented poikilocytes and target cells.

• Diagnosis of type: Hb electrophoresis.
o ß-thalassaemia major: absence of HbA1, HbA2 + HbF

59
Q

mx of thalassaemia

A

• Thalassaemia trait requires no treatment.
• Thalassemia major: regular blood transfusions to maintain Hb levels.
o Haemosiderosis (iron overload) is inevitable consequence, can minimise by use of SC/IV infusions of chelating agent desferrioxamine.
o Or PO iron chelating agents

60
Q

what is Wilm’s tumour

A

it is nephroblastoma, most common renal malignancy in children

peaks at 5 yrs

61
Q

presentation of Wilm’s tumours

A

unilateral, painless, abdo mass, haematuria + lethargy, weight loss etc

62
Q

ix for Wil’s tumour

A

FBC, U&E, LFT, LFT, coag, CT or MRO +/- biopsy

63
Q

Mx for Wilm’s tumour

A

surgery +/- adjuvant chemo +/- radiotherapy

Year 4 Paeds Conditions (10 decks)

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