Medicine Notes Paediatrics Notes
Paediatric notes based upon current NICE guidance, The Illustrated Textbook of Paediatrics by Lissauer and Clayden in conjunction with the Oxford Handbook of Paediatrics...
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Haematology and Oncology
Types of Hb
Embryonic Hb Gower 1, Hb Gower 2, Hb Portland
Fetal HbF (holds onto oxygen better than HbA oxygen dissociation curve shifts to the right at birth [offloads oxygen more readily])
Adult HbA (97%), HbA2
Anaemia
Hb below the normal range
Neonate <14g/dl
1-12 months <10g/dl
1-12 years <11g/dl
Anaemia may be due to:
Reduced RBC production
Red cell aplasia
Ineffective erythropoiesis
Fe deficiency
Folid acid deficiency
Haemolysis
Red cell membrane disorders hereditary spherocytosis
Red cell enzyme disorders glucose6 phosphate dehydrogenase deficiency
Haemoglobinopathies thalassaemias, sickle cell
Immune haemolytic disease of the newborn, autoimmune
Blood loss
Anaemia Due To Reduced Red Cell Production
Ineffective erythropoiesis differentiation and survival of RBCs is defective
Reticuloctye count will be normal
MCV will be abnormal
Low in iron deficiency
High in folate deficiency
Complete absence of RBC production in red cell aplasia
Low or absent reticulocyte count
Causes
Congenital – Diamond-Blackfan anaemia
Transient erythroblastopenia of childhood
Parvovirus B19 (only in those with inherited haemolytic anaemias)
Iron Deficiency
Term newborn iron stores of 250mg
Adult iron stores of 4-5g
Causes
Inadequate intake common in infantc
1 year old requires 8mg/day
Malabsorption
Blood loss
Iron sources
Breast milk – low content but 50% of iron is absorbed
Infant formula
Cow’s milk – higher content than breast but only 10% absorbed
Solids introduced at weaning
Increase absorption with Vitamin C
Clinically
Asymptomatic until Hb drops below 7g/dl
Tire easily
Feed slowly
Pallor (esp. conjunctivae, tongue or palmar creases)
Pica
Diagnosis
Microcytic, hypochromic anaemia (low MCV and MCH)
; B/a-thallassaemia, anaemia of chronic disease
Low serum ferritin
Management
Dietary advice
Oral supplementation
Continue until Hb is normal and for a further 3 months
Blood transfusion should NEVER be necessary for dietary iron deficiency
Increased Red Cell Destruction
Increased destruction either in the circulation (intravascular) or in the liver/spleen (extravascular)
Haemolysis only leads to anaemia when the bone marrow can no longer compensate
Main cause in children is intrinsic abnormalities of the RBC
Red cell mebrane disorders (hereditary spherocytosis)
Red cell enzyme disorders (G6PD deficiency)
Haemoglobinopathies (thalassaemia, sickle cell)
Haemolysis leads to:
Anaemia
Hepatomegaly
Splenomegaly
Increased blood levels of unconjugated bilirubin
Excess urinary urobilinogen
Diagnosis
Raised reticulocyte count (polychromasia)
Unconjugated bilirubinaemia and increased urinary urobilinogen
Abnormal appearance of red cells on blood film
Positive direct antiglobulin test (Coomb’s) [only in immune cause]
Increased red cell precursors in the bone
Hereditary Spherocytosis
1 in 5000 caucasian births
Autosomal dominant
Mutations in genes coding for red cell membrane proteins spectrin, ankyrin or band 3
Reduction in surface to volume ratio spheroidal destruction in the spleen
Clincally
Suspected due to family history
Jaundice intermittent during childhood OR severe jaundice in the first few days of life
Anaemia mild (9-11g/dl)
Mild/moderate splenomegaly
Aplastic crisis transient due to parvovirus B19
Gallstones due to increased bilirubin
Diagnosis
Blood film
- Autoimmune haemolytic anaemia
Excluded with a direct antibody test in the absence of a family history of hereditary spherocytosis
Management
Oral folic acid
Splenectomy is indicated for poor growth or troubelsome symptoms of anaemia
Defer until 7 years old
Vaccinated against Hib, Meningitis C and strep. Pneumoniae beforehand
Lifelong daily oral penicillin prophylaxis
Aplastic crisis
1 or 2 blood tranfusions over 3-4 weeks when no RBCs are produced
Glucose-6 Phosphate Dehydrogenase Deficiency
High prevalence in those originating from central Africa, the Mediterranean, the Middle East and the Far East
Rate limiting enzyme in the pentose phosphate pathway and prevents oxidative damage to RBCs
X linked and therefore largely affects males
Clinically
Neonatal jaundice
Acute haemolysis precipitated by:
Infection
Drugs antimalarials, sulphonamides, quinolones, nitrofuratoin
Fava bean
Intravascular haemolysis fever, malaise
Dark urine – urobilinogen and Hb
Diagnosis
Measure G6PD activity in RBCs
During a crisis the G6PD levels may be mistakingly elevated due to higher enzyme concentration in reticulocytes
Management
Info regarding signs of acute haemolysis (jaundice, dark urine)
And list of druge, chemicals and food to avoid
Haemoglobinopathies
Reduced or absent production of HbA (thalasaemia) or production of abnormal Hb (sickle cell)
Sickle Cell
1 in 2000 live births
Most common in patients who have black parents and originate from tropical Africa or the Caribbean
Mutation in codon 6 of the B globin chain HbS forms
Clincal manifestation is delayed until 6 months when the HbF has all but disappeared
Three main forms of disease and the sickle cell trait
Sickle Cell Anaemia (HbSS)
Homzygous for HbS
HbSC Disease
HbS from one parent and HbC from the other
HbC forms due to a different point mutation in B globin
No HbA – as no normal B globin genes
Develop proliferative retinopathy
Osteonecrosis of hips and shoulders
Sickle B thalassaemia
HbS from one parent and B thalassaemia trait from the other
No normal B globin genes
Sickle Trait
HbS from one parent and a normal B globin chain from the other
40% of Hb is HbS
Carriers
Asymptomatic
Clinical Features
Anaemia moderate anaemia with detectable jaundice
Infection
Esp. from encapsulated organisms (pneumococci, Hib)
Osteomyelitis due to Salmonella
Due to hyposplenism secondary to microinfarction in the spleen
Painful Crises
Vaso-occlusive crisis
Dactylitis with pain and swelling in the hands and feet in late infancy
...
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Paediatric notes based upon current NICE guidance, The Illustrated Textbook of Paediatrics by Lissauer and Clayden in conjunction with the Oxford Handbook of Paediatrics...
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