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3a > Paed:Haem > Flashcards

Paed:Haem Flashcards

1
Q

General signs and symptoms of anaemia?

A 
Fatigue
Dyspnoea on exertion
Poor grwoth
Pallor
Anorexia
Rarely -> stomatitis, koilonychia
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2
Q

List some examples of microcytic anaemia?

A

Iron defiency anaemia, thalassaemias, anaemia of chronic disease

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3
Q

Examples of macrocytic anaemia

A

Liver disease, b12/folate defieciency, bone marrow failure syndromes

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4
Q

Examples of normocytic anaemia?

A

Combined iron and b12/folate deficiency, anaemia of chronic disease, recent bleeding

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5
Q

Pathopysiology of sickle cell?

A

AR sickling disorder in homozygous sickle haemoglobin. Single amino acid substituion (glutamine for valine) occurs in beta-globin chain from mutation on chromosome 11.

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6
Q

Clinical features of sickle cell?

A

Infancy: High HbF protective until 6months. Most common problems are dactylitis, infections, splenic sequestrations.
Young children: Infection, vaso-occlusive crises in long bones, upper airway obstruction, stroke
Older children: Vaso-occlusive crises, infections (pneumococcus and parvovirus)

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7
Q

Sickle crises/problems?

A 
Vasoocclusive crises
Acute chest syndrome
Sequestration
Stroke
Infection
Aplastic crises
Priapism
Avascular necrosis
Retinopathy
Leg ulcers
Ent problems
growth and development retardation
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8
Q

Diagnosis of sickle cell?

A

haematology: increased reticulocytes, sickled cells,

HB electrophoresis is definitive test

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9
Q

Management of sickle cell?

A

Hydration
Analgesia
Antibiotics
o2

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10
Q

What is Von Willebrands disease?

A

Autosomal dominant. Results from either quantative or qualitative defiecinecy of VWF. Defective platelet plug formation and defiecient in FVIII:C.

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11
Q

What is the role of VWF?

A

1: Facilitates adhesion to damaged endothelium
2: Acts as carrier protein to FVIII:C, protecting it from inactivation and clearance

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12
Q

clinical features of VWF disease?

A

Bruising
Excessive prolonged bleeding post surgery
Mucosal bleeding - epistaxis and menorrhagia

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13
Q

Management of VWF disease?

A

Avoid Nsaids
TXA if minor bleed
DDVAP

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14
Q

What is haemophilia A?

A

COngenital bleeding disorder due to the defective production of factor 8.

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15
Q

Presentation of haemophilia A?

A 
easy brusing
bleeding into joints
painful joints
intramuscular bleeds
intercranial bleeds
haematuria
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16
Q

investigations of haemophilia A?

A

Inr same. APTT raised. Factor 8 down. VwF and bleeding time same.
CT scan ?intercranial bleed
USS for muscle haematomas

17
Q

Management of haemophilia A?

A

Factor 8 concentrate to raise levels

analgesia

18
Q

What is christmas disease?

A

Haemophilia B. Same as A tbh pal but just factor 9. Treatment is just factor 9.

19
Q

What is Thalassaemia?

A

Inherited defect in synthesis of one or more globin chains. Ineffective erythropoieses, leads to haemolysis. Normal adult haemoglobin is Hb A2B2.

20
Q

what is beta thalassaemia?

A

Loss of beta globin gene, so less beta chains and excess alpha chains.

21
Q

Clinical presentation of major beta thal?

A

6-12months in homozygous children: FtT, recurrent bacterial infections. Anaemia. Skeletal deformity. hepatosplenomegaly.

22
Q

What is the hair on end sign?

A

Seen on skull x-ray in major beta thalassaemia due to increased marrow activity.

23
Q

Bloods for Beta major thal?

A

MCV very low (microcytic)
Serum ferritin normal
Over 90% HbF
Film: Large and small irregular hypochromic RBCs

24
Q

management of beta thallasamia?

A

Transfusions
Chelation also needed
-Excess iron causes: Liver fibrosis and cirrhosis
Splenectomy

25
Q

What is alpha thalassaemia?

A

Deletion of one or both alpha chains on c16.

26
Q

What is Fanconis anaemia?

A

Rare AR condition leading to progressive bone marrow failure affecting all three haemopoietic cell precursors.

27
Q

PResentation of fanconis?

A

Bruising and purpura
Insidious onset anaemia
Associations: short stature, skin hyperpigmentation, skeletal abnormalities, renal malformations, microcephaly, cryporchism, mental retardation, deafness, abnormal facies.

28
Q

Investigations in fanconis?

A

FBC shows pancytopenia

BM shows hypoplastic diserythropoeitc or megaloblastic changes

29
Q

Treatment of fanconis?

A

Supportive eg. RBC transfusion, hearing aids
Immunosuppression with corticosteroids and androgens (oxymetholone)
BM transpalnt

30
Q

What is Immune Thrombocytopenia?

A

Commonest cause of thrombocytopenia in childhood. Caused by destruction of platelets by antiplatelet IgG antibodies. Decreased platelet count may be accompanied by a compensatory icnrease of megkaryocytes in BM.

31
Q

Clinical features of ITP?

A

Usually 2-10yrs old. onset often 1-2weeks post viral infection.
Sx: Petechiae, purpura, +/- superficial brusiing, epistaxis and other mucosal bleeding. Rare: Intercranial bleeding

32
Q

Investigations for ITP?

A

Dx of exclusion

NEed BM exam

33
Q

management of ITp?

A

most self limiting.
Oral prednisolone, Iv anti-D, IV IG,
platelet transfusions only if life threateing bleeding as only transient increase.

34
Q

What is chronic ITP?

A

Platelet count remains low for 6months after diagnosis

35
Q

What is the tx for significant bleeding?

A

Retuximab (directed against b lymphocytes)
Thrombopoietic growth factors
splenectomy

36
Q

What is haemolytic disease of the newborn?

A

Rhesus haemolytic disease. Haemorrhage usually at birth of fetal blood of differing rhesus group into maternal circulation which leads to maternal antiD igG production.

37
Q

Presentation of rheus disease?

A

Antenatal: Fetal anemia, hydrops fetalis
Postnatal: Hydrops fetalis, early jaudice, kernicterus, cutraneous haemopieitc lesions (Blueberry muffin),hepatosplenomegaly

38
Q

investigations of rhesus?

A

maternal blood: Rhesus -ve, increaed anti Rh titre

39
Q

What do u give to Rhd-ve mothers?

A

anti D IgG

3a (25 decks)

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