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haem Flashcards

1
Q

sickle cell crisis mx?

A 
analgesia
oxygen
may admit (low threshold for admission - esp if chest symptoms present)
increase fluid intake!!!
avoid other triggers

if Hb v low, blood transfusion

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2
Q

Triggers of crises in sickle cell??

A

dehydration
cold weather
excessive exercise
viral illness

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3
Q

Sickle cell anaemia complications?

A

dactylitis
splenic sequestration
gall stones
chronic pain

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4
Q

sickle cell anaemia prophylaxis?

A

immunisations
abx prophylaxis due to splenic dysfunction
folate supplementation due to rapid cell turnover

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5
Q

SCA medication to prevent crises?

A

hydroxycarbamide

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6
Q

immune thrombocytopenia cause?

A

often 1-2 wks after viral infection

  • anti platelet igG autoantibodies
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7
Q

autoantibodies in immune thrombocytopenia?

A

anti platelet igG

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8
Q

immune thrombocytopenia presentation?

A

purpura, superficial bruising
petechiae
epistaxis or other mucosal bleeding

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9
Q

immune thrombocytopenia mx?

A

in 80% - self limiting and will self resolve

oral prednisolone or IVIG

platelet transfusion if severe

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10
Q

what is considered thrombocytopenia in children?

A

platelets <150 x 10^9/L

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11
Q

causes of low pl due to increased pl destruction / consumption?

A 
ITP, SLE
HUS 
TTP
DIC
hypersplenism
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12
Q

haemophilia presentation?

A

most frequently bleeding into joints or muscles

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13
Q

bleeding post-circumcision?

A

haemophilia

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14
Q

what to avoid in all pts with haemophilia?

A

IM injections
NSAIDS
aspirin

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15
Q

haemophilia mx?

A

replace missing factor

MDT- specialist centres, specialist nurse, physiotherapist, psychosocial support

self help / support groups

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16
Q

B thal major features?

A

severe anaemia - transfusion dependent from 3-6 months of age
jaundice
FTT
extramedullary haemopoiesis - heaptosplenomegaly
classical facies - maxillary overgrowth and skull bossing

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17
Q

B thal major mx?

A

regular blood transfusions
to reduce growth failure and bone deformities

or bone marrow transplant

+ iron chelation (desferrioxamine) to prevent chronic iron overload

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18
Q

cardiac failure, liver cirrhosis, diabetes, infertility -?

A

chronic iron overload

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19
Q

B thal trait presentation?

A 
usually asymptomatic
mild anaemia (hypo chromic, microcytic)

Raised HbA2 and HbF

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20
Q

haemolytic disease of newborn tx?

A

IVIG + phototherapy/ exchange transfusion
+ oral folate for 6 months
+ supportive e.g. if coagulopathy

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21
Q

pica - eating non food materials

A

IDA

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22
Q

cows milk in excess

A

IDA

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23
Q

inheritance of G6PD deficiency?

A

x linked

24
Q

what pathway is implicated in G6PD deficiency?

A

pentose phosphate pathway

25
Q

some triggers of acute haemolysis in G6PD deficiency

A

fava beans
moth balls
acute infection
certain drugs e.g. antimalarials, antibiotics

26
Q

G6PD deficiency ix?

A

FBC, WCC, CRP

G6PD activity in RBC

27
Q

Sickle cell anaemia susceptible to which infections?

A 
encapsulated organisms 
strep pneumoniae
h influenza
meningococcus
osteomyelitis from salmonella
28
Q

acute chest syndrome in SCA mx?

A

severe hypoxia- need for mechanical ventilation

emergency transfusion

29
Q

complications of SCA

A 
avascular necrosis of femoral head
hand - foot syndrome in late infancy
sickle cell crises
aplastic crises 
sequestration crises (sudden splenic enlargement, abdo pain and shock)
30
Q

parvovirus B19 in SCA?

A

aplastic crisis

31
Q

splenic sequestration in SCA presentation?

A

sudden splenic and hepatic enlargement, abdo pain and shock

32
Q

general malaise, pallor lethargy, recurrent infections, easy brusing/ petechiae, bone pain

+ * hepatosplenomegaly, possible lymphadenopathy + testicular enlargement

A

ALL likely

immunophenotype to further subclassify into T cell or B cell

33
Q

Ix in suspected ALL?

A

FBC, WCC,
Blood film
Bone marrow biopsy
CXR to identify mediastinal mass characteristic of T cell ALL.

34
Q

what are the high risk features of ALL

A

Age <1 or >10
Tumour load > 50 x 10^9/L
Cytogenetic/ molecular abnormalities in tumour cells,
persistence of leukaemic blasts in BM despite chemo,

35
Q

DDx of pancytopenia in child

A

Inherited - e,g, fanconi’s anaemia.
Acquired - e.g. viral (EBV/ parvovirus/ herpes), drugs e.g chemo, chloramphenicol, carbamazepine. >80% are idiopathic aplastic anaemia.
BM infiltration- leukaemia, neuroblastoma, myelofibrosis, myelodysplasia.

36
Q

Pancytopenia Ix?

A 
Blood film.
MCV. FBC.
Reticulocytes.
Viral titres.
BM aspirate and trephine.
37
Q

Aplastic anaemia mx?

A

mx pending a diagnosis is supportive.
Platelet infusions if platelet count <5 x10^9 or actively bleeding.
Regular monitoring of temperature.
Decision to embark on definitive tx depends upon severity.
Bone marrow transplant offers best chance to those w matched sibling donor.
Otherwise, immunosuppressive tx w antithymocyte globulin and cyclosporin.

38
Q

purpura in child.

ddx?

A

infections. -e.g. meningococcal septicaemia.
thrombocytopenia - ITP, ALL.
Vasculitis- e.g. HSP.
trauma.
clotting disorders - e.g vwd, haemophilia a/b.
drugs e.g. steroids.

39
Q

purpura in child Ix?

A

FBC (Hb, WCC, Pl), CRP, U+Es.
Blood film.
urine dip.
clotting profile.

40
Q

Immune thrombocytopenia complications

A

most severe is intracranial haemorrhage, severe epistaxis, or GI bleeding.

41
Q

Immune thrombocytopenia mx?

A

dependent on symptoms.
if asymptomatic, most docs won’t treat. Lifestyle advice e.g. avoid activities w high risk of trauma.
90% self-resolve in 6 months.
if bleeding -> admit + prednisolone + IVIG.
platelet transfusions only indicated in the case of an intracranial haemorrhage or to tx life-threatening bleeding.

42
Q

what is acute sickle chest syndrome?

A

thrombosis, infection and fat embolism to the lung produces a syndrome of pleuritic chest pain, SOB, fever. hypoxia is.a frequent feature.

43
Q

acute sickle chest syndrome mx?

A

admit. adequate analgesia (IV morphine), supplemental oxygen and hyper hydration. Physiotherapy to help pt to breathe deeply enough to ventilate the affected areas of the lungs. empirical abx tx started e.g. coamoxiclav and clarithromycin.
Discuss mx w senior paediatrician and haemotologist.
If deteriorate-> ventilatory support (CPAP) or intubation.
exchange or top up blood transfusion may be needed to reduce the % of sickling RBCs.

44
Q

complications of sickle cell disease in children.

A 
painful crises.
avascular necrosis of hips/ shoulders.
chest syndrome, abdo syndrome.
splenic sequestration, aplastic anaemia.
stroke, retinal vein occlusion.
priapism, haematuria, enuresis, chronic renal failure.
pigment gallstones, cholecystitis, biliary colic.
hyposlenism, osteomyelitis.
delayed puberty.
45
Q

Main differential for Wilms tumour/ nephroblastoma?

A

Neuroblastoma- an embryonal cancer of the peripheral SNS. most arise in the abdomen either in the adrenal gland or in the retroperitoneal sympathetic ganglia.
the mass usually causes discomfort, and usually present with symptoms of metastatic spread.
95% of NB patients have raised urine VMA (vanilylmandelic acid) and HVA (homovanilic acid).

46
Q

Wilms tumour tx?

A

surgery with pre and post surgery chemo and radiotherapy depending on the stage.
prognosis favourable, stage 1-3 pts have an overall cure rate of 88-98%.
refer to specialist unit. care of MDT - oncologist, paediatrician, nephrology, specialist nurses.

47
Q

Suspected Wilms tumour Ix?

A

BP - usually raised.
Urine dip/ MCS.
Abdo USS.
FBC, WCC, CRP, U+Es. Creatinine.

48
Q

ataxia - broad based unsteady gait in a child who had previously normal gait?

A

disorder of the cerebellum and or sensory pathways in the posterior columns of the spinal cord.
- heel shin test, dysdiadochokinesia, dysmetria.

49
Q

Diagnosis of ALL

A

FBC. Blood film. analysis of BM aspirate.

50
Q

Mx of ALL

A

remission induction: 4 wks of combination chemo.
Consolidation and CNS protection: oral chemotherapy. a course of weekly intrathecal drugs given to prevent later CNS relapse.
Delayed intensification: intense chemo w blocks of maintenance chemo.
continuing maintenance: chemotherapy continues for a total of 2 yrs in girls and 3 yrs in boys. patients are immunosuppressed and at risk of opportunistic infections. co-trimoxazole given to prevent PCP infection.

51
Q

poor prognostic factors of ALL

A

outside age range 2-10 yrs. male sex.
total WCC >100 x 10^9 at presentation.
Certain chromosome abnormalities, CNS disease at presentation, slow response to tx.

52
Q

Ddx of structural scoliosis

A

idiopathic,
neuromuscular e.g. cerebral palsy, Duchenne muscular dystrophy,
congenital structural abnormalities of the vertebrae e.g. hemivertebrae - high risk of assoc spinal cord defects, esp genitourinary.
syndromic e.g. neurofibromatosis and Marfan syndrome.

53
Q

Ix of scoliosis

A

bend forward to touch toes - if scoliosis is postural, this will disappear.
if structural scoliosis found, thorough neurological examination.

54
Q

Mx of scoliosis

A

all should be monitored regularly. (esp premenarchal girls- most at risk of causing unacceptable deformities)
bracing and surgical correction are treatment options. surgery is recommended earlier in those w neuromuscular or congenital scoliosis.

55
Q

Child just diagnosed with AML. what is your first mx priority?

A

Prevent tumour lysis syndrome.

As tx begins, a large no of cells break down -> hyperK, hyperPO4, gout, fluid overload/ dehydration, raised Ur/Cr.

56
Q

what is part of the prophylactic mx of tumour lysis syndrome?

A

hyperhydration, allopurinol/ rasburicase and monitoring of electrolytes from time of diagnosis.

57
Q

von willebrands disease. inheritance?

A

auto dominant

paeds (12 decks)

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