Child with Pallor Flashcards
eWhat are the three different modes which lead to anaemia? List some differentials under each?
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
If reticulocytes are elevated what would you consider to be amongst the differentials?
Blood loss or haemolysis
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?
- 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
How do you know there is impaired red blood cell production? Which investigation?
Check for low reticulocyte count Discriminating tests - Blood film: MCV - Serum iron - TIBC - Ferritin - Folic acid - B12 - Bone marrow aspirate
What is the aetiology of iron deficiency anaemia?
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
What is the are risk factors for iron deficiency anaemia?
- 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
What are the clinical manifestations of iron deficiency anaemia?
- 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
In iron deficiency anaemia? What investigations would you do and what would their results be?
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)
List some strategies to prevent iron deficiency anaemia in paediatrics?
- 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
Treatment of iron deficiency anaemia?
- Identify the cause
- Ferrous gluconate (10-12% elemental iron) 6 mg/kg/day
- Dietary advice: Red meat, white meat, legumes, green veges, egg yolk
What is the aetiology of B12 deficiency in the child?
- Undiagnosed maternal B12 deficiency in the breast fed child
- Inadequate intake of meat, chicken, eggs and milk
What are some investigations for B12 deficiency?
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
How is B12 deficiency treated.
IM B12
Ongoing oral supplementation
What is thalassemia minor? How does it present? What is diagnosis and management?
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
What is beta thalassemia major? How does it present? Explain the diagnosis and management?
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
Name causes of haemolysis extrinsic to the RBC and intrinsic to the RBC?
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
How do haemolytic anaemias present?
Anemia with pallor, fatigue, cardiac instability
Jaundice + Dark urine
Recent viral illness/ vomiting/ Diarrhoea
What is the key investigating in knowing that there is a haemolysis
Increased reticulocyte count & Jaundice
What are some discriminating investigations in haemolysis?
- Blood group and Rh typing
- Blood film (microspherocytosis in immune and HS)
- Coombs test
- G6PD assay
- Osmotic fragility
What is the most common platelet disorder in childhood?
Immune Thrombocytopaenic purpure, it peaks at ages 2-6
What is the aetiology/ pathophysiology of ITP?
Caused by antibodies which bind to platelet membranes –> Fc receptor mediated splenic uptake –> splenic destruction of platelets
What is the clinical presentation of ITP?
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)
What is the management of ITP?
• 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
HDN?
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
Haemophilia A is a deficiency of what factors? How does it manifest?
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
What is haemophilia B and how is it treated?
Factor 9 deficiency treated with recombinant factor 9
What is purpure fulminans?
- 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
