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ویرایش: 1 نویسندگان: Dr. Martin Hewitt (editor), Dr. Roshan Adappa (editor) سری: ISBN (شابک) : 0702080373, 9780702080371 ناشر: Elsevier سال نشر: 2022 تعداد صفحات: 420 زبان: English فرمت فایل : PDF (درصورت درخواست کاربر به PDF، EPUB یا AZW3 تبدیل می شود) حجم فایل: 31 مگابایت
در صورت تبدیل فایل کتاب Applied Knowledge in Paediatrics: : MRCPCH Mastercourse به فرمت های PDF، EPUB، AZW3، MOBI و یا DJVU می توانید به پشتیبان اطلاع دهید تا فایل مورد نظر را تبدیل نمایند.
توجه داشته باشید کتاب دانش کاربردی در اطفال: : دوره کارشناسی ارشد MRCPCH نسخه زبان اصلی می باشد و کتاب ترجمه شده به فارسی نمی باشد. وبسایت اینترنشنال لایبرری ارائه دهنده کتاب های زبان اصلی می باشد و هیچ گونه کتاب ترجمه شده یا نوشته شده به فارسی را ارائه نمی دهد.
راهنمای ضروری برای قبولی در آزمون MRCPCH دانش کاربردی در عمل (AKP).
با برنامه درسی آزمون تئوری کالج سلطنتی اطفال و بهداشت کودک (RCPCH) هماهنگ است و همچنین منعکس کننده عملکرد فعلی انگلستان است. دانش کاربردی در اطفال باعث می شود خواننده با دانش کاملی از تمام موضوعات اصلی مرتبط با اطفال و سلامت کودک آشنا شود. همچنین یک کمک مطالعه مفید برای کسانی که امتحان MRCPCH دانش کاربردی در عمل را انجام می دهند ارائه می دهد.
این کتاب چگونگی و چرایی ایجاد بیماری ها در کودکان و نوجوانان، بررسی ها و درمان مناسب مورد نیاز، فوریت اجرا و تاثیر روند بیماری بر کودک و خانواده آنها.
این متن جامع توسط دکتر مارتین هویت، کارشناس ارشد تئوری برای معاینه AKP، و دکتر روشن آدپا، پزشک ارشد و مربی تحصیلات تکمیلی، با نظرات کارشناسان بیش از 60 متخصص اطفال. 33 فصل اطلاعات جامعی را در مورد تمام زمینههای تمرین اطفال ارائه میدهد.
The essential guide to passing the MRCPCH Applied Knowledge in Practice (AKP) exam.
Closely aligned to the Royal College of Paediatrics and Child Health (RCPCH) theory examination syllabus and also reflective of current UK practice. Applied Knowledge in Paediatrics will leave the reader with a sound knowledge of all the core topics relevant to paediatrics and child health. It will also provide a useful study aid for those undertaking the MRCPCH Applied Knowledge in Practice examination.
The book covers how and why illnesses develop in children and young people, the appropriate investigations and treatment required, the urgency of implementation and the impact of the disease process on the child and their family.
This comprehensive text is edited by Dr Martin Hewitt, a Senior Theory Examiner for the AKP examination, and Dr Roshan Adappa a Senior clinician and postgraduate trainer, with expert input from more than 60 paediatric specialists. 33 chapters provide comprehensive information of all areas of paediatric practice.
Cover 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