Child with Pallor Flashcards

1
Q

eWhat are the three different modes which lead to anaemia? List some differentials under each?

A

Inadequate Production:

  • Bone marrow failure/ replacement: transient erythroblastopaenia of childhood, aplastic anaemia, leukaemia/ tumour infiltration, metabolic disorders
  • Haematinic deficiency ( eg. fe, B12, folate)
  • Haemoglobinopathies: e.g. SCA/ thalassaemia

Excessive Destruction

  • Hemolysis
  • Bleeding
  • Sequestration into an enlarged spleen
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2
Q

If reticulocytes are elevated what would you consider to be amongst the differentials?

A

Blood loss or haemolysis

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3
Q

If the McV is low and it is a microcytic anaemia? What are the differentials? What further tests would you conduct to differentiate between the differentials?

A
  • Further test to conduct would be a serum ferritin
  • If it is low then iron deficiency anaemia
  • If high , do a further hb electrophoresis to determine if thalassemia minor

Differentials for Hypochromic Microcytic anaemia

  • Iron deficiency anaemia
  • Thalassemia minor ( check the family history/ ethnicity)
  • Lead poisoning (Rare, PICA, irritability/ coloic)
  • Sideroblastic anaemia

Normocytic anaemia (reticulocyte count) - if increased you have haemolysis or blood loss, if no increased or abnormal consider marrow hypoplasia, leukaemia and infiltration

  • Anaemia of chronic disease
  • Acute blood loss/ haemolysis ( bleeding history
  • TEC: Malabsorption
  • Mixture nutritional deficiency (dietary restrictions)
  • Aplastic anaemia/ marrow infiltration

Macrocytic disease - do serum folate, RBC folate, Vit B12 levels

If low

  • Folate deficiency
  • B12 deficiency

If normal

  • Myelodysplasia
  • Fanconi’s anaemia
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4
Q

How do you know there is impaired red blood cell production? Which investigation?

A
Check for low reticulocyte count
Discriminating tests
- Blood film: MCV
- Serum iron
- TIBC
- Ferritin
- Folic acid
- B12
- Bone marrow aspirate
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5
Q

What is the aetiology of iron deficiency anaemia?

A

Increased requirements

  • Low birth weight
  • Rapid growth (first year, adolescents)
  • Feto-maternal, feto-fetal, placental, umbilical bleed

Inadequate intake
- Diet rick in milk and cereals but poor in meat and vegetables, lack of iron supplementation

Blood loss

  • faecal blood loss ( cows milk protein intolerance colitis)
  • Meckels Diverticulum
  • Recurrent epistaxis in olde rchildren
  • Periods: adolsecnet girls
  • Hookworm

Malabsroption

  • Chronic diarrhoea
  • Coeliacs Disease
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6
Q

What is the are risk factors for iron deficiency anaemia?

A
  • Exclusive breast feeding beyond 6/12, iron stores decline
  • Delayed introduction of iron containing solids
  • Excessive cows milk particularly < 1 yo.
  • Vegetarian/ Vegan Diet
  • Exclusion diets with allergic diseases
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7
Q

What are the clinical manifestations of iron deficiency anaemia?

A
  • Generally subclinical until marked anaemia
  • Pallor
  • Fatigue, reduced exercise tolerance, lethargy
  • Anorexia, postural hypotension and fainting
  • Poor growth
  • PICA ( eating non food materials)
  • Cardiac: Tachycardia, flow murmur, S3, SOB
  • Angular chelitis
  • ## Koilonychias
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8
Q

In iron deficiency anaemia? What investigations would you do and what would their results be?

A

FBE: Low Hb, low mCV and low MCH
Reticulocyte count: normal or high (absolute no is low)
If MCV is low - do ferritin - if low, confirms iron deficiency anaemia. Remember that ferritin is also an acute phase reactant, in the short term setting.
If high do a Hb electrophoresis - could be thalassemia minor

Blood film MCV < 65 fl, Microcytic, hypochromic, poikilocytosis ( distorted shape and variation)

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9
Q

List some strategies to prevent iron deficiency anaemia in paediatrics?

A
  • Introduce iron containing solids from 4-6 months
  • Avoid cows milk in the first 12 months ( aside from small amounts in cereals)
  • For breast fed infants, start iron supplements at 4-6 months
  • Premature infants - give iron supplements from 1 month to 1 year 2mg/kg/day
  • E.g. consider supplementation in a high risk group
  • Formulas and cereals should be iron fortified
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10
Q

Treatment of iron deficiency anaemia?

A
  • Identify the cause
  • Ferrous gluconate (10-12% elemental iron) 6 mg/kg/day
  • Dietary advice: Red meat, white meat, legumes, green veges, egg yolk
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11
Q

What is the aetiology of B12 deficiency in the child?

A
  • Undiagnosed maternal B12 deficiency in the breast fed child
  • Inadequate intake of meat, chicken, eggs and milk
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12
Q

What are some investigations for B12 deficiency?

A

FBE- macrocytic anaemia
Blood film - Hypersegmented neutrophils
Serum B12 - Low
Serum homocysteine and urinary methylmalonic acid allows monitoring of response to therapy
Ferritin, common to have coexistent iron deficiency
Maternal investigations: FBE, Active B12, Serum homocysteine, urinary MMA

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13
Q

How is B12 deficiency treated.

A

IM B12

Ongoing oral supplementation

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14
Q

What is thalassemia minor? How does it present? What is diagnosis and management?

A

Defect in single allele of beta gene, common in medeterrian and Asian people . No clinical features, and a palpable spleen is rare. Investigations will show a microcytic anaemia, with normal RBC count
Blood Film - microcytosis basophilic

Hb electrophoresis:

  • Specific HbA2 increased to 3.5-5%
  • Non specific: 50 % have slight increase in HbF

Treatment is not rqeuired

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15
Q

What is beta thalassemia major? How does it present? Explain the diagnosis and management?

A

Defect in both alleles of beta gene, leads to ineffective chain synthesis leading to ineffective erythropoiesis, hemolysis of RBC’s and increase in HbF

Clinical Features

  • PResents at 6-12 months when fetal haemoglobin is replaced
  • Severe anaemia and jaundice occurs
  • Can get an iron overload due to repeated infusions and ineffective erythropoiesis
  • Leads to iron induced organ damage ( liver, spleen, anterior pituitary, pancreas, and heart affected)
  • Stunted growth and development (hypogonadal dwarf)
  • Hepatosplenomegally, frontal bossing and severe anaemia

Thalmajor: Erythroblastosis
Hb electrophoresis - elevated HbA2 +/- elevated Hbf

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16
Q

Name causes of haemolysis extrinsic to the RBC and intrinsic to the RBC?

A

Extrinsic to RBC

  • Immune mediated (AIHA), drug induced, ABO/rhesus
  • Consumptive (DIC)
  • Fragmentation/ Mechanical - Cardiac valve, HUS, TTP
  • Infection : malaria, severe sepsis, Ecoli, group B strep

Intrinsic to RBC

  • RBC membrane disorders e.g. spherocytosis
  • Haemoglobinopathies
  • Enzymopathies - G6PD
17
Q

How do haemolytic anaemias present?

A

Anemia with pallor, fatigue, cardiac instability
Jaundice + Dark urine
Recent viral illness/ vomiting/ Diarrhoea

18
Q

What is the key investigating in knowing that there is a haemolysis

A

Increased reticulocyte count & Jaundice

19
Q

What are some discriminating investigations in haemolysis?

A
  • Blood group and Rh typing
  • Blood film (microspherocytosis in immune and HS)
  • Coombs test
  • G6PD assay
  • Osmotic fragility
20
Q

What is the most common platelet disorder in childhood?

A

Immune Thrombocytopaenic purpure, it peaks at ages 2-6

21
Q

What is the aetiology/ pathophysiology of ITP?

A

Caused by antibodies which bind to platelet membranes –> Fc receptor mediated splenic uptake –> splenic destruction of platelets

22
Q

What is the clinical presentation of ITP?

A

50% present 1-3 wk after viral illness (URTI, chicken pox)
• sudden onset of petechiae, purpura, epistaxis in an otherwise well child
• clinically significant bleed in only 3% (severe bleed more likely with platelet count <10) with
<0.5% risk of intracranial bleed
• no lymphadenopathy, no hepatosplenomegaly
• labs: thrombocytopenia with normal RBC, WBC
• bone marrow aspirate only if atypical presentation (≥1 cell line abnormal, hepatosplenomegaly)
• differential diagnosis: leukemia, drug-induced thrombocytopenia, HIV, infection (viral),
autoimmune (SLE, ALPS)

23
Q

What is the management of ITP?

A

• observation vs. pharmacologic intervention highly debated; spontaneous recovery in >70% of
cases within 3 mo
• treatment with IVIg or prednisone if mucosal or internal bleeding, platelets <10, or at risk of
significant bleeding (surgery, dental procedure, concomitant vasculitis or coagulopathy)
• life-threatening bleed: additional platelet transfusion ± emergency splenectomy
• persistent (>3-12 mo) or chronic (>12 mo): re-evaluate; treat if symptoms persist
• supportive: avoid contact sports and ASA/NSAIDs

24
Q

HDN?

A

Deficiency of vitamin- K dependent coagulation factors
Factors 2,7,9,10 decline in the first 2-3 days of life
PT and PTT are prolonged
Melena, umbilical cord bleeding, haematuria
Intercranial haemorrhage
All newborns thus receive vitamin K

25
Q

Haemophilia A is a deficiency of what factors? How does it manifest?

A

Haemophilia A is an X linked - disorder
- Deficiency of factor VIII
Causes the PTT to be prolonged
It manifests in bleeding into joints, muscle, subcutaneous tissue, intercranial, umbilical
- Resolved by replacement of missing factors

26
Q

What is haemophilia B and how is it treated?

A

Factor 9 deficiency treated with recombinant factor 9

27
Q

What is purpure fulminans?

A
  • PT, PTT prolonged, fibrinogen and platelets low, D dimers increased
  • Meningococcaemia
  • DIC from other bacterial septicaemia
  • Varicella
  • Meaasles
  • Congenital C & S deficiency ( neonatal purpure filminans)
  • Acquired or congenital deificnecy fo protein C
    Hypercoagulable state. Protein C when attached to a vessel surface by protein S, inactivates factor V & VIII