Applied Knowledge in Paediatrics: MRCPCH Mastercourse

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Applied Knowledge in Paediatrics: MRCPCH Mastercourse
Copyright
Contributors
Acknowledgements
Preface
1 - Preparing for the AKP exam
Examination structure
Question journey
Question types
Single best answer (SBA)
Multiple best answer (MBA)
Multipart question (MPQ)
Extended matching questions (EMQ)
Detailed advice
Evidence-­based paediatrics
2 - Neonatology
Antenatal assessment of fetal growth
Definition and classification
Screening for intrauterine growth restriction
Fundal height (FH) measurement
Selective ultrasonography
Growth indices
Perinatal events and birth injury
Hypoxic-­ischaemic encephalopathy
Investigations
Treatment and managment
Important sequelae
Brachial plexus injury
Investigations
Treatment and management
Antenatal management of preterm labour
Management of preterm labour
Management of the high-­risk pregnancy
Following birth
Medical conditions in the preterm neonate
Respiratory distress syndrome
Differential diagnosis
Investigations
Treatment and management
Mechanical respiratory support
Bronchopulmonary dysplasia
Treatment and management
Patent ductus arteriosus
Treatment and management
Necrotising enterocolitis (NEC)
Investigations
Treatment and management
Germinal matrix and intraventricular haemorrhage (GM-­IVH)
Treatment and management
Neurodevelopmental outcome
Periventricular leukomalacia
Treatment and management
Neurodevelopmental outcome
Retinopathy of prematurity (ROP)
Treatment and management
Medical conditions in the term neonate
Meconium aspiration syndrome
Transient tachypnoea of the newborn (TTN)
Treatment and management
Persistent pulmonary hypertension of the newborn (PPHN)
Investigations
Treatment and management
Neonatal hypoglycaemia
Clinical presentation
Investigations
Treatment and management
Hyperbilirubinemia
Unconjugated hyperbilirubinaemia
Treatment and management
Complications
Conjugated hyperbilirubinemia
Investigations
Treatment and management
Neonatal sepsis
Investigations
Treatment and management
Neonatal seizures
Clinical presentation
Investigations
Treatment and management
Neonatal abstinence syndrome
Investigations
Treatment and management
Neonatal thrombocytopenia
Treatment and management
Common surgical conditions affecting the term neonate
Malrotation with or without midgut volvulus
Treatment and management
Meconium ileus
Duodenal atresia and duodenal web
Tracheo-­oesophageal fistula and oesophageal atresia
Investigations
Treatment and managment
Anorectal malformations
Vacterl association
Exomphalos
Gastroschisis
Treatment and management
Congenital diaphragmatic hernia
Treatment and managment
3 - Adolescent medicine
Adolescent health needs
Legal framework
Risk-­taking behaviour
Substance abuse
Alcohol
Alcohol use disorders
Tobacco and vaping
Nicotine replacement therapy
E-­cigarettes and vaping
Illicit drugs
Cannabis
Other drugs of abuse
Inhalants
Self-­harm
Assessment of risk
Safety planning
Outcomes
Eating disorders
Anorexia nervosa
Bulimia nervosa
Clinical presentation
Investigations
Treatment and management
Potential complications
Important sequelae
Sexual health
Teenage pregnancy
Risk factors
Consequences of teenage parenthood
Health outcomes of child born to young parents
Reducing teenage pregnancy
Sexually transmitted infection
Acne
Management
4 - Growth and puberty
Disorders of growth
Short stature
Clinical presentation
Investigations
Important sequelae
Specific conditions
Familial short stature
Small for gestational age (SGA)
Chronic conditions
Endocrine causes
Growth hormone deficiency
Clinical presentation
Investigations
Treatment and management
Pharmacological agents used
Hypothyroidism
Cushing syndrome or disease
Syndromic causes
Turner syndrome
SHOX mutations
Noonan syndrome
Silver Russell syndrome (SRS)
Fetal alcohol spectrum disorder
Skeletal dysplasias
Tall stature
Syndromic causes with disproportionate long-­limbed tall stature
Marfan syndrome
Klinefelter syndrome
Syndromic causes without disproportionate long-limbed tall stature
Beckwith–Wiedermann syndrome
Sotos syndrome
Disorders of puberty
Sexual precocity
Clinical presentation
Gonadotropin-­dependent sexual precocity
Investigations
Treatment and management
Potential complications
Important sequelae
Gonadotrophin-­independent sexual precocity
Premature thelarche
Exaggerated adrenarche
McCune–Albright syndrome
Delayed puberty
Clinical presentation
History
Examination
Central delayed puberty
Primary gonadal failure
Primary ovarian failure
Testicular failure
Investigations
Treatment and management
5 - Genetics and dysmorphology
Chromosomal abnormality and mutations
Numerical abnormalities
Structural abnormalities
Sex chromosome abnormalities
Genetic sequence variants (point mutations)
Autosomal dominant conditions
Autosomal recessive conditions
X chromosome linked conditions
Mitochondrial mutations
Imprinting conditions
Common genetic and dysmorphological conditions
Down syndrome
Patau syndrome
Edward syndrome
Turner syndrome
Klinefelter syndrome
Wiskott-Aldrich syndrome
Chromosome 22q11.2 deletion (Di George syndrome, Velo-cardio-facial syndrome)
Williams syndrome
Prader-Willi syndrome
Angelman syndrome
Peutz-Jeghers syndrome
Beckwith–Wiedemann syndrome
Noonan syndrome
Fragile X syndrome
Marfan syndrome
Silver Russell syndrome
Rett syndrome
Achondroplasia
Albright’s Hereditary Osteodystrophy
(also known as pseudohypoparathyroidism type 1a)
Genetic testing
Screening of individuals
Diagnostic genetic testing
Predictive genetic testing
Genetic carrier testing
Population screening
Population carrier screening
Prenatal screening
Newborn screening
Disease susceptibility screening
Effects of environmental factors affecting the foetus
Smoking
Drugs
Alcohol
6 - Nutrition
Breast feeding
Formula feeding
Alternative milks and their clinical indications
Complimentary feeding
Assessment of nutritional status
Growth charts
Malnutrition (undernutrition)
Investigation
Treatment and management
Obesity
Vitamin, mineral and micronutrient deficiencies
Vitamins
Vitamin A
Thiamine (vitamin B1)
Riboflavin (vitamin B2)
Niacin (vitamin B3)
Pantothenic acid (vitamin B5)
Pyridoxine (vitamin B6)
Folate (vitamin B9, folic acid)
Vitamin B12
Ascorbic acid (vitamin C)
Vitamin E
Vitamin K
Micronutrients
Zinc
Iodine
7 - Behavioural medicine and mental health
Sleep Problems
Bedtime resistance or refusal
Delayed sleep onset
Nightmares and night terrors
Delayed sleep-­wake cycle disorder
Relevant pharmacological agents used
Feeding difficulties
Avoidant and Restrictive Feeding Intake Disorder (ARFID)
Pica
Behavioural manifestations in physical disease
Impact of chronic disease
Somatic symptom disorders
Somatic symptom disorder
Illness anxiety disorder
Conversion disorder (functional neurological symptom disorder)
Antisocial behaviour and conduct disorder
Conduct disorders
Oppositional defiant disorder
Treatment and management
Challenging behaviour in children with intellectual disability
Child and adolescent mental health services (CAMHS)
Mental health conditions
Depressive disorder
Clinical presentation
Differential and comorbid diagnoses
Treatment and management
Relevant pharmacological agents used
Bipolar disorder
Clinical presentation
Differential and comorbid diagnoses
Treatment and management
Early onset psychosis and schizophrenia
Clinical presentation
Treatment and management
Anxiety disorders
Clinical presentation
Separation anxiety disorder
Generalised anxiety disorder
Social anxiety disorder
Panic disorder
Specific or simple phobias
Agoraphobia
Post-traumatic stress disorder
Differential and comorbid diagnoses
Treatment and management
Relevant pharmacological agents used
Obsessive compulsive disorders (OCD)
Clinical presentation
Treatment and management
Relevant pharmacological agents used
8 - Emergency medicine
Initial assessment of a collapsed or unconscious child
Paediatric cardiac arrest
Treatment and management
Shock
Clinical manifestations
Investigations
Treatment and management
Relevant pharmacological agents used
Anaphylaxis
Investigations
Management of acute episode
Management following acute episode
Relevant pharmacological agents used
Acute airway obstruction
Investigations
Acquired acute upper airway obstruction
Croup
Treatment and management
Peritonsillar abscess
Retropharyngeal abscess
Epiglottitis
Clinical presentation
Treatment and management
Bacterial tracheitis
Airway foreign bodies
Treatment and management
Acute seizures
Convulsive status epilepticus (CSE)
Investigations
Treatment and management
Medication
Acute neurological emergencies
Treatment and management
Stroke
Treatment and management
9 - Unintentional injuries and poisoning
Trauma
Primary survey
Drowning
Treatment and management
Investigations
Life-­threatening haemorrhage
Treatment and management
Head and spinal injuries
Head injury
Extradural
Subdural haemorrhage
Classification of head injury
Investigations
Treatment and management
Spinal injury
Treatment and management
Burns and scalds
Investigations
Treatment and management
Potential complications
Limb-­threatening injuries
Vascular injuries
Compartment injuries
Neurological injuries
Open fractures
Common poisonings
Nonpharmaceuticals and household products
Advice to parents on unintentional injuries and their prevention
Head injuries and fractures
Falls
Foreign body aspiration or ingestion
Poisoning
Burns
Drowning
Road traffic impact
10 - Fluids and electrolyte management
Appropriate intravenous fluid
Ongoing losses
Monitoring
Assessment of hydration status
Management of dehydration
Oral rehydration therapy
Resuscitation
Risk of acute kidney injury
Electrolyte disturbance
Hyponatraemia
Causes of hyponatraemia
Clinical presentation
Investigations
Treatment and management
The child with normal or increased volume status
The child with moderate dehydration and serum sodium 130–135 mmol/l
The child with severe dehydration or dehydration with serum sodium less than 130 mmol/l
Acute hyponatraemic encephalopathy is a medical emergency
The hyponatraemic child with seizures or CNS depression
Hypernatraemia
Clinical presentation
Treatment and management
For hypernatraemic dehydration with serum sodium greater than 150 mmol/l
Hypokalaemia
Causes
Clinical presentation
Treatment and management
Oral supplementation
Intravenous supplementation of maintenance fluids
Hyperkalaemia
Causes
Clinical presentation
Treatment and management
Moderate hyperkalaemia (potassium over 5.9 mmol/l)
Severe hyperkalaemia (potassium over 7 mmol/l)
Relevant pharmacological agents used
11 - Safeguarding
Physical abuse
Bruises
Plausible explanations for bruises
Fractures
Plausible explanations for causes of fracture
Burns
Plausible explanations for causes of skin lesions
Head trauma
Neglect and emotional abuse
Perplexing presentation and fabricated or induced illness (FII)
Sexual abuse
Presentation
Assessment of a child where abuse is suspected
Organisation of child protection services
Long-­term effects of abuse
12 - Infectious diseases
Bacterial Infections
Bacterial Septicaemia
Investigations
Treatment and management
Potential complications
Bacterial meningitis
Investigations
Treatment and management
Pneumococcal infections
Meningococcal disease
Streptococcal disease
Investigations
Treatment and management
Potential complications
Staphylococcal infections
Pertussis
Alternative diagnoses and differing features
Investigations
Treatment and management
Potential complications
Important sequelae
Lyme disease
Investigations
Treatment and management
Pharmacological agents used
Tuberculosis
Investigations
Treatment and management
Relevant pharmacological agents used
Non tuberculous mycobacterium
Viral Infections
Rotavirus
Alternative diagnoses
Investigations
Treatment and management
Enterovirus and parechovirus
Alternative diagnoses
Investigations
Treatment and management
Human herpes virus
Herpes simplex virus (HSV)
Investigations
Treatment and management
Complications
Varicella-­zoster virus (VZV)
Treatment and management
Cytomegalovirus (CMV)
Investigations
Treatment and management
Epstein Barr virus (EBV)
Associations
Investigations
Treatment and management
Respiratory viruses
Influenza
Investigations
Treatment and management
Pharmacological agents used
Respiratory Syncytial Virus (RSV)
Investigations
Treatment and management
SARS-­CoV-­2 (COVID-­19)
Investigations
Treatment and management
Potential complications
Important sequelae
Hepatitis B (HBV)
Investigations
Treatment and management
Hepatitis C (HCV)
Investigations
Treatment and management
Human Immunodeficiency Virus (HIV)
Investigations
Treatment and management
Fungal infections
Candida albicans
Investigations
Treatment and management
Aspergillus
Investigations
Treatment and management
Pharmacological agents used
Infections acquired overseas
Travellers’ diarrhoea
Investigations
Treatment and management
Malaria
Investigations
Treatment and management
Typhoid fever/enteric fever
Investigations
Treatment and management
Potential complications
Kawasaki disease
Clinical presentation
Alternative diagnoses
Investigations
Treatment and management
Potential complications
13 - Infection control
Infection control
Modes of transmission
Droplet
Airborne
Contact
Infectious period
Standard precautions
Immunisation
Routine immunisation
Complications of vaccination
Contraindications to vaccinations
Vaccination in pregnancy
Vaccination of children with unknown or incomplete immunisation status
Immunising the immunocompromised child
Children with clinical conditions where vaccinations are contraindicated
Children with clinical conditions may need extra vaccinations
Children where some vaccinations may not be effective
Children where family members to be vaccinated
Antimicrobial use
Infection control for epidemic and pandemic infections
14 - Allergy
Clinical presentation
Treatment and management
Investigations
Relevant pharmacological agents used
Important sequelae
Food allergy
Clinical presentation
Investigations
Treatment and management
Acute and chronic urticaria
Clinical presentation
Investigations
Treatment and management
Relevant pharmacological agents used
Asthma and allergy
Clinical presentation
Investigations
Management of allergic aspect of asthma
Allergic rhinoconjunctivitis
Clinical presentation
Common allergens
Investigations
Treatment and management
Relevant pharmacological agents used
Important sequelae
Drug allergy
Clinical presentation
Investigation
Treatment and management
15 - Immunology
Antibody immunodeficiencies
Clinical presentation
Investigations
Treatment and management
X-­linked agammaglobulinaemia
Clinical presentation
Investigations
Treatment and management
Common variable immunodeficiency disorder (CVID)
Treatment and management
Selective IgA deficiency
Clinical presentation
Treatment and management
Specific antibody deficiency (SPAD)
Transient hypogammaglobulinemia of infancy (THI)
Secondary antibody deficiencies
Combined immunodeficiencies
Severe combined immunodeficiency (SCID)
Management
Combined immunodeficiencies
Combined immunodeficiencies with syndromic features
Complement deficiencies
Abnormalities of neutrophil function
Congenital neutropenia
Severe congenital neutropenia (SCN)
Cyclical neutropenia
Schwachman-­Diamond syndrome (SDS)
Chronic granulomatous disease (CGD)
Autoimmune disorders
Autoinflammatory disorders
Periodic Fever syndromes
Clinical presentation
Alternative diagnoses and differing features
Investigations
Treatment and management
16 - Cardiology
Chest radiograph
Electrocardiogram
Ambulatory ECG monitoring
Echocardiography
Cardiac catheterisation
Hyperoxia test
Congenital heart disease
Clinical features
Left to right shunt (table 16.3)
Atrial septal defect (secundum)
Investigations
Treatment and management
Partial atrio-­ventricular septal defect
Ventricular septal defect
Persistent ductus arteriosus
Investigations
Treatment and management
Right to left shunt (table 16.4)
Tetralogy of Fallot
Investigations
Treatment and management
Transposition of the great arteries
Investigations
Treatment and management
Common mixing (table 16.5)
Complete AV septal defect
Well children with obstruction
Pulmonary stenosis
Investigations
Treatment and management
Aortic stenosis
Investigations
Treatment and management
Vascular ring
Sick children with obstruction
Coarctation of the aorta
Investigations
Treatment and management
Cardiac symptoms and signs
Murmurs
Chest pain
Palpitations, cardiac arrhythmias
Supraventricular tachycardia (SVT)
Emergency management of acute arrhythmias
Supraventricular tachycardia (SVT)
Ventricular tachycardia (VT)
Further management of cardiac arrhythmias
Examples of abnormal ECG rhythms
Relevant pharmacological agents
Adenosine. Adenosine blocks conduction, primarily at the atrioventricular node, and has a very short half-­life of a few seconds...
Flecainide. Flecainide is contraindicated if heart failure, haemodynamically significant valvar heart disease or conduction defe...
Amiodarone. Amiodarone is contraindicated if bradycardia, thyroid dysfunction and iodine sensitivity are present. Side effects i...
Syncope
Heart failure
Investigations
Treatment and management
Relevant pharmacological agents used
Potassium sparing diuretics (amiloride, spironolactone). There is evidence that spironolactone can help the ventricle remodel, s...
Loop diuretics (bumetanide, furosemide). These inhibit the reabsorption of sodium, potassium and chloride in the kidney. Contrai...
Digoxin. Digoxin decreases the automaticity of sinoatrial node and reduces conductivity within the atrioventricular node. It is ...
Other cardiac conditions
Myocarditis
Treatment and management
Kawasaki disease
Pericardial disease
Rheumatic fever
Investigations
Treatment and management
Important sequelae
Cardiomyopathy
Dilated cardiomyopathy
Management
Hypertrophic cardiomyopathy
Hypertension
Systemic hypertension
Pulmonary hypertension
Investigations
Management
Relevant pharmacological agents used
Sildenafil. Promotes relaxation of vascular smooth muscle. It is contraindicated if the patient has sickle-­cell disease or rece...
Prostanoids—epoprostenol, iloprost, selexipag. Prostanoids produce a vasodilatation but are contraindicated in veno-­occlusive d...
Nitric oxide. Nitric oxide is a potent and selective pulmonary vasodilator as it is given by inhalation. Side effects include in...
Infective endocarditis
Investigations
Treatment and management
Potential complications
Cardiac complications of other system disorders
17 - Respiratory
Blood gases
Chest radiograph
Pulse oximetry
Spirometry
Bronchodilator reversibility test
Bronchoprovocation test
Fraction of exhaled nitric oxide (FeNO)
Bronchoscopy
Whole body plethysmography (or the ‘body box’)
Transfer factor of the lung for carbon monoxide (TLCO)
Cough
Protracted bacterial bronchitis
‘Red flags’ for cough
Investigations
Treatment and management
Wheezing disorders
Asthma
Asthma in the acute setting
Associations
Investigations
Treatment and management of chronic asthma
Treatment and management of acute attack
Lower respiratory tract infections
Clinical presentation
Alternative diagnoses
Investigations
Treatment and management
Important sequelae
Respiratory failure or cardiovascular collapse
Pleural effusion and empyema
Bronchiectasis
Bronchiolitis obliterans
Other respiratory conditions
Primary ciliary dyskinesia
Chronic aspiration
Complication of foreign body inhalation
Cystic fibrosis
Clinical presentation
Investigations
Treatment and management
Nutritional management
Management of pulmonary exacerbations
Measurement of lung function
Respiratory complications
Allergic bronchopulmonary aspergillosis
Haemoptysis
Pneumothorax
End-­stage lung disease
Non respiratory complications
CF liver disease
CF-­related diabetes
CF-­related arthritis
Fertility
Annual assessment
Transition to adult care
CFTR modulator drugs
Sleep-­disordered breathing
Life-­threatening events and sudden infant death
Acute life-­threatening event (ALTE)
Sudden infant death in infancy (SUDI)
Long-­term ventilation
Surgical respiratory conditions
Eventration of diaphragm
Treatment and management
Congenital pulmonary airway malformation (CPAM)
Investigations
Treatment and management
Chest wall deformities
Treatment and management
Surgical emphysema
18 - Hearing and ENT
Hearing Testing
Newborn Hearing Screening Tests
Visual Reinforcement Audiometry
Play Audiometry
Pure Tone Audiometry
Tympanometry
Types of Hearing Loss
Conductive Impairment
Sensorineural Impairment (SNHL)
Mixed impairment
Treatment and management
Conditions Affecting Ears, Nose or Throat
Otitis Media
Otitis Media with Effusion
Treatment and management
Acute Otitis Media
Epistaxis
Tonsillar Hypertrophy
Pharyngotonsillitis
Clinical presentation
Investigations
Treatment and management
Sinusitis
Mastoiditis
19 - Gastroenterology and hepatology
Acute abdominal pain
Chronic abdominal pain
Organic causes of chronic abdominal pain
Gastric acid–related conditions
Treatment and management
Helicobacter gastritis
Investigations
Treatment and management
Potential complications
Functional causes of chronic abdominal pain
Treatment and management
Irritable bowel syndrome (IBS)
Treatment and management
Abdominal migraine
Treatment and management
Diarrhoea
Acute diarrhoea
Investigations
Treatment and management
Chronic diarrhoea
Investigations
Specific conditions causing chronic diarrhoea
Coeliac disease
Associations
Investigations
Treatment and management
Inflammatory bowel disease (IBD)
Investigations
Treatment and management
Relevant pharmacological agents used
Short bowel syndrome
Treatment and management
Protein-­losing enteropathy
Treatment and management
Lactose intolerance
Investigations
Constipation
Investigations
Treatment and management
Relevant pharmacological agents used
Encopresis
Vomiting
Investigations
Treatment and management
Infant regurgitation
Gastro-­oesophageal disease (GORD)
Treatment and management
Cyclical vomiting syndrome
Treatment and management
Gastrointestinal bleeding
Investigations
Treatment and management
Diseases of the pancreas
Pancreatitis
Investigations
Treatment and management
Diseases of the liver
Prolonged neonatal jaundice
Conjugated jaundice in neonatal period and in infancy
Biliary atresia
Investigations
Treatment and management
Alagille syndrome
Alpha-­1-­antitrypsin deficiency
Cystic fibrosis
Incidental finding of an elevated transaminases
Investigations
Autoimmune liver disease
Wilson’s disease
Investigations
Treatment and management
Nonalcoholic fatty liver disease (NAFLD)
Treatment and management
Acute liver disease
Investigations
Treatment and management
Acute liver failure
Treatment and management
Chronic liver disease
Treatment and management
Surgical conditions and congenital malformations
Oesophageal stricture
Achalasia
Treatment and management
Hiatus hernia
Treatment and management
Infantile hypertrophic pyloric stenosis
Treatment and management
Ingested foreign body
Treatment and management
Malrotation and volvulus
Treatment and management
Treatment and management
Meckel’s diverticulum
Intussusception
Treatment and management
Treatment and management
Hirschsprung disease
Treatment and management
Treatment and management
Rectal prolapse
Treatment and management
Treatment and management
20 - Nephro-­urology
Proteinuria
Haematuria
Radiological investigations
Congenital anomalies of the kidney and urinary tract
Hydronephrosis
Causes
Treatment and management
Pelviureteric junction obstruction
Posterior urethral valve
Polycystic kidney disease
Acute kidney injury
Treatment and management
Haemolytic uraemic syndrome
Chronic kidney disease
Investigations
Treatment and management
Urinary tract infection
Investigations
Treatment and management
Renal tubular disorders
Renal tubular acidosis (RTA)
Distal RTA (type I)
Bartter syndrome
Investigation
Treatment and management
Nephrogenic diabetes insipidus
Hypertension
Investigations
Treatment and management
Phaeochromocytoma
Acute glomerulonephritis
Causes
Investigations
Treatment and management
Henoch-­Schoenlein purpura
IgA Nephropathy
Investigations
Treatment and management
Alport syndrome
Nephrotic syndrome
Treatment and management
Potential complications
Renal calculi
Investigations
Treatment and management
Enuresis
Investigations
21 - Haematology
Investigations
Impaired red cell production
Iron deficiency anaemia
Investigations
Treatment and management
Relevant pharmacological agents used
Bone marrow failure
Investigations
Inherited bone marrow failure syndromes
Fanconi anaemia
Diamond Blackfan anaemia
Schwachman-­Diamond syndrome
Acquired bone marrow failure
Aplastic anaemia
Treatment and management
Haemoglobin disorders
Thalassaemia
α-­thalassaemia
Treatment and management
HbH
Hb Barts Hydrops fetalis
β-­thalassaemia
Investigations
Treatment and management
Potential complications
Relevant pharmacological agents used
Increased red cell destruction or loss
Haemolytic anaemia
Investigations
Treatment and management
Specific haemolytic conditions
Spherocytosis
Autoimmune haemolytic anaemia
Paroxysmal nocturnal haemoglobinuria (PNH)
Sickle cell disease
Clinical features
Investigations
Treatment and management of acute vaso-­occlusive episodes
Treatment and management of chronic problems
Important sequelae
Transfusion of blood products
Coagulation disorders
Investigations
Prothrombin time
Activated partial thromboplastin time
Thrombin Time
Fibrinogen levels
D-­dimers
Relevant pharmacological agents used
Bleeding disorders
Haemophilias
Investigations
Treatment and management
Relevant pharmacological agents used
Potential complications
von Willibrand Disease
Investigations
Treatment and management
Disseminated intravascular coagulation (DIC)
Investigations
Treatment and management
Potential complications
Thrombophilia
Platelet disorders
Acquired thrombocytopenias
Treatment and management
Relevant pharmacological agents used
Kasabach-­Merritt phenomenon
Neutropenia
Congenital neutropenia
Acquired neutropenia
Congenital neutropenia
Treatment and management
22 - Oncology
Clinical presentation
Alternative diagnoses
Aplastic anaemia
Investigations
Treatment and management
Important sequelae
Lymphoproliferative disorders
Non-­Hodgkin Lymphoma (NHL)
Clinical presentation
Alternative diagnoses
Associations
Investigations
Treatment and management
Potential complications
Important sequelae
Hodgkin Lymphoma (HL)
Clinical presentation
Investigations
Treatment and management
Important sequelae
Central nervous system tumours
Clinical presentation
Associations
Investigations
The common types of tumours include
Treatment and management
Potential complications
Solid tumours
Neuroblastoma
Clinical presentation
Alternative diagnoses and differing features
Investigations
Treatment and management
Nephroblastoma (Wilms tumour)
Clinical presentation
Associations
Investigations
Treatment and management
Important sequelae
Hepatoblastoma
Clinical presentation
Investigations
Treatment and management
Osteosarcoma
Clinical presentation
Investigations
Treatment and management
Potential complications
Ewing Sarcoma
Clinical presentation
Investigations
Treatment and management
Important sequelae
Soft tissue sarcomas
Langerhans cell histiocytosis (LCH)
Clinical presentation
Investigations
Treatment and management
Syndromes with propensity to malignancy
Oncological emergencies
Neutropenic fever
Clinical presentation
Treatment and management
Tumour lysis syndrome
Clinical presentation
Investigations
Treatment and management
Important sequelae
Superior vena caval obstruction
Radiotherapy
Peripheral stem cell harvest and transplantation
Treatment and management
Potential complications
Shared care services
Common pharmacological agents used
23 - Dermatology
Associations
Alternative diagnoses and differing features
Investigations
Treatment and management
Relevant pharmacological agents used
Bacterial skin infections
Staphylococcal scalded skin syndrome
Investigations
Treatment and management
Impetigo
Treatment and management
Cellulitis
Treatment and management
Scarlet fever
Treatment and management
Potential complications
Viral skin infections
Systemic viral infections
Localised viral skin infections
Fungal skin infections
Tinea (dermatophyte) infections
Candida (yeast) infections
Infestation
Clinical features
Urticaria
Clinical presentation
Investigations
Treatment and management
Cutaneous drug eruptions
Clinical presentation
Diagnosis and management
Systemic disorders involving the skin
Connective tissue disease
Clinical presentation
Lupus erythematosus
Juvenile dermatomyositis
Scleroderma
Cutaneous vasculitis
Henoch-­Schönlein purpura
Kawasaki disease
Endocrine disorders
Diabetes-­related disorders
Autoimmune disorders
Other disorders
Coeliac disease
Erythema multiforme
Erythema nodosum
Langerhans cell histiocytosis
Ectodermal dysplasias
Epidermolysis bullosa
Epidermolysis bullosa simplex
Junctional epidermolysis bullosa
Dystrophic epidermolysis bullosa
Birthmarks
Vascular malformations
Capillary malformations
Port wine stain (also known as naevus flammeus)
Venous malformations
Lymphatic malformations
Vascular tumours
Infantile haemangiomas
Associations
Treatment
Pigmented birthmarks
Neurocutaneous syndromes
Neurofibromatosis
Clinical presentation
Investigations and management
Tuberous sclerosis complex
Clinical presentation
Sturge Weber syndrome
Incontinentia pigmenti
Ataxia telangiectasia
24 - Diabetes mellitus
Clinical presentation
Investigations
Treatment and management
Potential complications
Type 2 diabetes mellitus
Clinical presentation
Treatment and management
Relevant pharmacological agents used
Monogenic diabetes
Types of monogenic diabetes
Neonatal diabetes
Monogenic diabetes in children and adolescents
Investigations
Treatment and management
Diabetic ketoacidosis (DKA)
Clinical presentation
Investigations
Treatment and management
Fluid resuscitation and fluid deficit
Maintenance fluids
Metabolic acidosis
Intravenous insulin
Potential complications
Cerebral oedema
Other complications
Hypoglycaemia
25 - Endocrinology
Anterior pituitary hormone deficiency
Clinical presentation
Investigations
Associations
Treatment and management
Relevant pharmacological agents used
Inappropriate or excess anterior pituitary hormone production
Posterior pituitary hormone deficiency
Investigations
Treatment and management
Posterior pituitary hormone excess
Syndrome of inappropriate antidiuretic hormone (SIADH)
Associated conditions
Investigations
Management and complications
Adrenal disorders
Adrenal insufficiency
Adrenal insufficiency in the neonate
Adrenal insufficiency in the older child and adolescent
Alternative diagnoses and differing features
Investigations and expected results in primary adrenal insufficiency
Treatment and management
Cushing syndrome
Alternative diagnoses
Investigations
Treatment and management
Phaeochromocytoma
Thyroid disorders
Hypothyroidism
Investigations
Treatment and management
Hyperthyroidism
Investigations
Treatment and management
Antithyroid medication
Radioiodine
Thyroidectomy
Potential complications
Disorders affecting calcium regulation
Disorders resulting in hypocalcaemia
Investigations
Alternative diagnoses
Treatment and management
Disorders resulting in hypercalcaemia
Treatment and management
Other specific measures include
Rickets
Investigations
Treatment and management
Endocrine complications of other diseases
Chronic disease
Complications of steroid treatment
Endocrine problems in childhood cancer
26 - Neurology
Neurophysiological investigations
Electroencephalogram (EEG)
Cerebral function monitoring
Nerve conduction studies and electromyography
Neuroimaging
Cranial ultrasound
Computed tomography (CT) imaging
Magnetic resonance imaging (MRI)
Abnormal head shape
Microcephaly
Macrocephaly
Plagiocephaly
Craniosynostosis
Epilepsy
Neonatal seizures
Epilepsies of infancy
Epileptic spasms (ES)
Epilepsies in young children
Childhood absence epilepsy
Myoclonic atonic and myoclonic absence epilepsy
Childhood epilepsy with centrotemporal spikes
Lennox Gastaut syndrome
Epilepsies of adolescence
Juvenile absence epilepsy
Juvenile myoclonic epilepsy
Structural focal epilepsies (temporal, frontal and occipital lobe epilepsy)
Outcomes
Treatment and management
Seizure-­like disorders
Normal and abnormal paroxysmal events
Headache
Primary headache disorders
Treatment and management
Secondary headache disorders
Ataxia
Acute ataxia
Episodic ataxias
Chronic ataxia
Progressive ataxias
Neurocutaneous syndromes
Neurofibromatosis type 1 (NF1)
Treatment and management
Neurofibromatosis type 2 (NF2)
Treatment and management
Tuberous sclerosis
Treatment and management
Sturge Weber syndrome (SWS)
Treatment and management
Neuromuscular disorders
Floppy infant syndrome
Central hypotonia
Peripheral hypotonia
Spinal muscular atrophy (SMA)
Treatment and management
Duchenne muscular dystrophy (DMD)
Becker muscular dystrophy (BMD)
Investigation
Treatment and management
Progressive muscular conditions
Metabolic myopathies
Myasthenia
Peripheral neuropathies
Guillain Barre syndrome (GBS)
Investigations
Treatment and management
Rare causes of peripheral neuropathy
Hereditary sensory and motor neuropathy (HMSN)
Spina bifida
Treatment and management
Stroke
Perinatal arterial ischaemic stroke
Arterial ischaemic stroke
Treatment and management
Haemorrhagic stroke
27 - Neurodevelopmental medicine
Associations
Investigations
Treatment and management
Development
Medical
Social and well-­being
Cerebral palsy
Classification
Alternative diagnoses
Associations
Investigations
Treatment and management
Relevant pharmacological agents used
Complications and sequelae
Specific syndromes
Down syndrome
Complications and sequelae
Fetal alcohol syndrome disorder
Alternative diagnoses and differing features
Rett syndrome
Williams syndrome
Alternative diagnoses and differing features
Chromosome 22q11.2 deletion (Di George syndrome, velocardiofacial syndrome)
Prader Willi syndrome (PWS)
Alternative diagnoses and differing features
Angelman syndrome
Complications and sequelae
Communication disorders
Treatment and management
Complications and sequelae
Neurodevelopmental disorders
Executive function
Autism spectrum disorder (ASD)
Treatment and management
Complications and sequelae
Attention deficit hyperactivity disorder (ADHD)
Associations
Investigations
Treatment and management
Relevant pharmacological agents used
Complications and sequelae
Developmental coordination disorder (DCD)
Investigations
Complications and sequelae
28 - Ophthalmology
Eye and vision screening programmes
Visual development and visual impairment
Testing visual function
Assessment of the red reflex
Abnormal ophthalmic findings
Proptosis
Causes of proptosis
Treatment and management
Ptosis
Causes of ptosis
Clinical presentation
Treatment and management
Strabismus (squint)
Causes of strabismus
Treatment and management
Nystagmus
Causes of nystagmus
Specific ophthalmic disorders
Optic nerve head swelling
Causes of optic nerve head swelling
Treatment and management
Cataract
Causes of childhood cataract
Treatment and management
Glaucoma
Causes of childhood glaucoma
Treatment and management
Optic nerve hypoplasia (ONH)
Investigations
29 - Musculoskeletal disorders
Septic arthritis
Investigations
Management and treatment
Reactive arthritis
Treatment and management
Juvenile idiopathic arthritis (JIA)
Investigations
Treatment and management
Oligoarticular JIA
Treatment and management
Systemic onset JIA
Polyarticular JIA
Psoriatic JIA
Enthesitis-­related JIA
Relevant pharmacological agents used
Vasculitic disorders
Juvenile systemic lupus erythematosus (jSLE)
Investigations
Treatment and management
Other systemic rheumatic diseases
Rheumatic fever
Henoch-­Schonlein purpura (HSP)
Treatment and management
Common inherited bone disorders
Osteogenesis imperfecta (OI)
Clinical presentation
Investigation
Treatment and management
Achondroplasia
Marfan syndrome
Developmental bone disorders
Scoliosis
Treatment and management
Developmental dysplasia of the hip (DDH)
Investigations
Treatment and management
Perthes disease
Investigation
Treatment and management
Slipped femoral caput epiphysis
Investigation
Treatment and management
30 - Metabolic medicine
Hypoglycaemia
Investigations
Treatment and management
Metabolic acidosis
Investigations
Treatment and management
Metabolic diseases
Idiopathic ketotic hypoglycaemia
Treatment and management
Glycogen storage disease (GSD)
Investigations
Treatment and management
Galactosaemia
Investigations
Treatment and management
Medium chain acyl-­CoA dehydrogenase deficiency (MCADD)
Investigations
Treatment and management
Mucopolysaccharidoses
Investigations
Treatment and management
Urea cycle disorders
Investigations
Treatment and management
Phenylketonuria (PKU)
Investigations
Treatment and management
Homocystinuria
Alternative diagnoses
Investigations
Treatment and management
Organic acidaemias (acidurias)
Methylmalonic acidaemia and propionic acidaemia
Maple syrup urine disease
Treatment and management
Familial hypercholesterolaemia
Investigations
Treatment and management
Mitochondrial disease
Newborn blood screening programme
31 - Palliative care
The principle of double effect
Proportionality and directness
Proportionality
Directness
Withdrawing versus withholding
The process of making difficult decisions
End of life discussions: uncertainty and futility
Paediatric Palliative Care (PPC)
Children who need palliative care
Palliative care and disease-­directed treatment
Symptoms
Psychosocial aspects of the symptom experience
Symptoms as spiritual phenomena
Assessment of symptoms
Management of specific symptoms
Pain
Nausea and vomiting
Dyspnoea
Other symptoms
When treatment fails
32 - Ethics and law
Autonomy
Nonmaleficence
Beneficence
Justice
Confidentiality
Research ethics
Law
Definition of a child
Children in the family
Welfare of the child is paramount
Parental responsibility
Consent
Consent by a young person
Withholding treatment
Mental Capacity Act
The child under arrest
Discrimination and protected status
Duty of candour
33 - Patient safety and clinical governance
Practical aspects of clinical governance
Clinical effectiveness
Multidisciplinary teams
Clinical audit
Near misses and mistakes
Risk management
Effective communication
Engaging with public and users
Information governance
Apply patient safety thinking
Human factors in patient care
Health care institution
Communication
Team structure
Team leadership (supervisors)
Decision making
Stress
Fatigue
Work environment
Education and training
Safe and effective handover
34 - Evidence-­based paediatrics
Methods
Statistical and sample size
Bias
Data analysis
Statistical tests
Data outcomes
Interpretation of confidence intervals and p-­values
Confidence intervals
Explanation of answers for Example 1
Interpretation of confidence intervals: difference between the means
P-­values
Data interpretation
Odds
Odds ratio
Explanation of answers to Example 2
Relative risk (RR)
Explanation of answers to Example 3
Risk difference
Number needed to treat
35 - Exam questions and answers
Questions
ANSWERS