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Specialties > Paeds Written - HAEM > Flashcards

Paeds Written - HAEM Flashcards

1
Q

Epidemiology of ALL

A

Peak age 2-5 yo
Boys slightly > girls
Trisomy 21 increases risk!!

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2
Q

Presentation of ALL

A 
Bone pain
Splenomegaly
Hepatomegaly
Testicular swelling
Fever
Leukaemia cutis - petechial rash on face & trunk
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3
Q

Poor prognostic factors in ALL

A 
Age <2 or >10
WBC >20 at diagnosis
T or B cell surface markers
Not White
Male
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4
Q

Ix findings in ALL

A

BM biopsy >20% blasts

Film –> peripheral blasts

FBC, clotting –> anaemia, thrombocytopenia, neutropenia +/- DIC

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5
Q

Biochemical evidence of tumour lysis syndrome

A

High K+
High phosphate
High uric acid
High LDH

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6
Q

Pathophysiology of Idiopathic Thrombocytopenic Purpura

A

Immune-mediated (type 2 hypersensitivity)

Abs directed against glycoprotein IIb/IIIa or Ib-V-IX complex

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7
Q

Triggers for ITP

A

Viral infection

Live vaccine

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8
Q

Presentation of ITP

A

NO FEVER
Petechiae or purpuric rash
Bleeding e.g. epistaxis, gingival bleeding - less common

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9
Q

Management of ITP

A

Usually no tx needed
Advise to avoid activities that may cause trauma e.g. contact sports

If very low Plts (<10) or bleeding:

  • oral/IV corticosteroids
  • IV Ig
  • platelet transfusion? - only in emergency, will soon be destroyed
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10
Q

Signs & symptoms of Hodgkin’s lymphoma

A

Painless lymphadenopathy (neck)
Painful after EtOH
B symptoms - fever, night sweats, weight loss

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11
Q

Lymph node biopsy findings in Hodgkin’s lymphoma

A

Reed-Sternberg Cells - Owl’s eyes appearance

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12
Q

Staging for lymphoma

A

Ann Arbor

1 = one group of nodes
2 = >1 group of nodes, same side of diaphragm
3 = node above and below diaphragm
4 = extra nodal spread

A = no B Sx, B = B Sx

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13
Q

Diagnostic markers for Hodgkin’s lymphoma

A

CD30

CD15

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14
Q

Hodgkin’s lymphoma chemotherapy regime

A

ABVD

Adriamycin
Bleomycin
Vincristine
DTIC (Dacarbazine)

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15
Q

Specific types of Non Hodgkin’s lymphoma to know

A

H. Pylori associated gastric MALToma

Enteropathy associated T cell lymphoma - coeliac disease

HIV - adult T cell lymphoma

Transpant & EBV - post transplant lymphoproliferative disease

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16
Q

Histopathology of Burkitt’s lymphoma

A

Starry sky appearance

Germinal centre cells

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17
Q

Types of Burkitt lymphoma

A

Endemic - EBV infection, children in malaria endemic regions, involves jaw or fatal bones

Sporadic - EBV infection, ‘Western’ world

Immunodeficiency - HIV infection or post transplant

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18
Q

Presentation of Ewing’s sarcoma

A

Mass or swelling
Bone pain
Malaise, fever
Paralysis - may precipitate osteomyelitis

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19
Q

Where does Ewing’s sarcoma occur in the body?

A

Long bones of arms, legs

chest, skull & trunk

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20
Q

Radiological finding in Ewing’s sarcoma

A

Bone destruction

Onion skinning - overlying periosteal bone formation

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21
Q

Histology of Ewing’s sarcoma

A

Small round blue cells

22
Q

Most common primary bone malignancy of children?

A

Osteosarcoma

23
Q

Presentation of osteosarcoma

A

Relatively painless
Mass/swelling
Restricted movement
Rapid mets to lung

24
Q

Radiological findings in Osteosarcoma

A

Sunburst appearance - soft tissue calcification

Codman’s triangle - elevated periosteum

25
Q

Origin of neuroblastoma

A

Neural crest tissue

anywhere in sympathetic chain - most commonly adrenal medulla

26
Q

Presentation of neuroblastoma

A

Abdominal mass - depends on site of tumour
Systemic - weight loss, pallor, hepatomegaly, bone pain, limp
Spinal cord compression Sx

27
Q

Ix findings in neuroblastoma

A

Raised urinary catecholamine metabolites - VMA / HVA

+ confirmatory biopsy from BM
+ MIBG radioisotope scan (highlights areas e.g. phaeo, neuroblastoma)

28
Q

Management of neuroblastoma

A

May spontaneously regress - very young infant

Localised primary = surgery

Metastatic = chemotherapy + radiotherapy (with autologous stem cell rescue) + surgery

29
Q

Pathophysiology of Sickle cell disease

A

Point mutation in codon 6 of beta globin gene on Chr11
Causes presence of structurally abnormal haemoglobin - HbS
Low oxygen conditions (or acidosis) –> aggregation of HbS –> occlusion of vessels

30
Q

Forms of sickle cell disease

A

Sickle cell anaemia = homozygous HbSS

Sickle cell trait = heterozygous for HbAS
- mild anaemia

Hb C disease
- milder sickling than HbSS

31
Q

Presentation of sickle cell anaemia

A

chronic haemolysis - anaemia, splenomegaly, gallstones, aplastic crises (parvovirus B19)

Vaso-occlusive painful crises - dactylitis, acute chest syndrome, stroke, priapism, splenic sequestration, retinopathy, renal dysfunction

Infection - encapsulated organisms esp.

Long term - short stature, delayed puberty, heart failure, psychosocial issues

32
Q

Mechanisms of anaemia in sickle cell anaemia

A

Haemolysis

BUT also HbS is lower affinity Hb so more readily releases O2 to tissues –> lower EPO drive

33
Q

Ix for sickle cell disease

A

Family origins questionnaire

Guthrie/heelprick testing (neonate)

FBC & blood smear –> nucleated RBC, sickle cells, Howell-Jolly bodies

Solubility testing

Haemoglobin electrophoresis = gold standard

34
Q

Electrophoresis results in sickle cell disease

A

Normal beta globin (glutamine) = very +ve charge

Defective beta globin (HbS - valine) = neutral

Defective beta globin (HbC - lysine) = very -ve charge

35
Q

management of painful crises

A

Admit (unless only mid-mod pain + apyrexial)

Analgesia within 30 mins

  • regular paracetamol + NSAIDs
  • opioid

Oxygen if sats <95%

Corticosteroids NOT usually given in uncomplicated crises

36
Q

Long term management principles for sickle cell disease

A

Hydroxycarbamide - increases foetal Hb

Prophylactic penicillin V

Immunisations (encapsulated organisms - strep, pneumococcus, HiB)

Folic acid

Nutrition + fluid intake

Avoid triggers - cold, dehydration, stress

+/- Splenectomy
+/- HSCT

37
Q

Signs & Sx of beta thalassaemia

A

Major

  • extramedually haematopoeisis - bone expansion, hepatosplenomegaly, frontal bossing
  • aneamia
  • heart failure
  • growth retardation
  • iron overload –> HF, gonadal failure

Trait

  • asymptomatic
  • microcytic anaemia
38
Q

Ix in (beta) thalassaemia

A

Family origins questionnaire (FOQ) – Indian, Mediterranean, Middle East

Guthrie testing after antenatal screening

Blood smear –> microcytic red cells, tear drop cells, microspherocytes, target cells, shistocytes, nucleated RBCs

Haemoglobin electrophoresis (gold-standard)

39
Q

Management of beta thalassaemia major

A

Blood transfusion
+/- iron chelation (dexferrioxamine, deferiprone)

HSCT if HLA-identical sibling

40
Q

Pathophysiology of Haemophilia

A

X linked recessive

Deficiency of factor 8 (A) or 9 (B)

Causes intrinsic pathway defect

41
Q

When might a female have Haemophilia A/B?

A

Turner’s syndrome

  • only have 1 X chromosome
42
Q

Clinical features of haemophilia

A

Haemarthrosis
May seem like NAI

Mild (>5% factor levels) - bleeding after surgery

Moderate (1-5%) - bleeding after minor trauma

Severe (<1%) - spontaneous bleeding (joints, muscles)

43
Q

Clotting studies in Haemophilia

A

APTT (intrinsic) prolonged

PT (extrinsic) normal

44
Q

Management of Haemophilia

A

Mild Haemophilia A = desmopressin

Severe haemophilia - prophylactic factor replacement

  • 2-3 x per week
  • can be done at home
  • target = factor level baseline >2%

Active bleeding = IV infusion factor 8/9 concentrate

AVOID IM injections, aspirin, NSAIDs

45
Q

Presentation of iron deficiency anaemia in children

A

Asymptomatic - until ~60-70
Feed slowly
Tire easily
Pica - eating soil, ice etc.

46
Q

tx of iron deficiency anaemia

A

Dietary advise - leafy green veg, red meat

Oral ferrous sulphate 200mg TDS

  • until normal Hb
  • continue for at least 3 months after
  • reached 2-4wks after starting, Hb should ride 2g/100mL
  • SE = black stools, constipation
47
Q

Triggers in G6PD deficiency

A 
Antimalarials - quinine
Abx - nitrofurantoin
Naphthalene moth balls
Analgesics - aspirin
Fava beans
47
Q

Triggers in G6PD deficiency

A 
Antimalarials - quinine
Abx - nitrofurantoin
Naphthalene moth balls
Analgesics - aspirin
Fava beans
48
Q

Presentation of G6PD deficiency

A

Neonate: jaundice (<3 days old)

Acute intravascular haemolytic after trigger exposure

  • fever
  • abdo pain
  • malaise
  • dark urine
49
Q

Ix findings in G6PD deficiency

A

During acute episode:
Raised G6PD - higher enzyme concentration in reticulocytes
Blood film - Heinz bodies, bite cells

After episode:
G6PD levels low (gold standard is to repeat after 1 month)

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