Oncology & Haematology : Prepare for the MRCPCH. Key Articles from the Paediatrics & Child Health journal

Oncology & Haematology is a new e-book in a collection of subject-themed e-books containing relevant key articles from Paediatrics & Child Health. The e-books provide a perfect source of revision for post-graduate exams in paediatrics and portfolio material for life-long learning. As well as mapping to the requirements of post-graduate training in paediatrics, these e-books also enable anyone with a short-term interest in a specific area to buy individual articles at a price-point that will give affordable access to all readers (from medical students to GPs and practitioners in related areas). The quality of user experience on mobiles, tablets and laptops will be an added bonus for learning on the move. About the journal The parent journal (http://www.paediatricsandchildhealthjournal.co.uk/) is a rolling, continuously updated review of clinical medicine over a 4-year cycle covering all the important topics for post-graduate exams in paediatrics. The journal’s articles are refreshed, updated, augmented or replaced as appropriate each time the subject is due for revision to provide a concise overview of knowledge and practice core to the curriculum. Each article is written by invited experts and overseen by the relevant subject specialist on the Board. A trainee representative on the Board ensures relevance and accessibility for exam candidates. Front Cover Oncology and Haematology: Prepare for the MRCPCH Copyright Page PREFACE Table of Contents Section 1: Oncology Wilms tumour — the surgical issues Introduction Diagnosis Differential diagnosis Pre-op investigations Staging of Wilms tumour Surgical exploration Tumour involving the renal vein, IVC Bilateral — stage V — Wilms tumour Contraindications for partial nephrectomy Lung metastases Jehovah’s witnesses Early complications from Wilms nephrectomy Surgical treatment of relapse Long term outcomes from surgery Conclusion FURTHER READING Bone tumours in childhood and adolescence Epidemiology and genetics Natural history and pattern of spread Clinical presentation Investigation and diagnosis Prognostic factors Treatment of bone tumours Following the survivors Rehabilitation FURTHER READING Germ cell tumours in children and adolescents Introduction Epidemiology Pathology Tumour markers Biology Clinical presentation Principles of management for GCTs Case-based clinical presentations and management of GCTs Follow-up in GCTs Extracranial GCTs Intracranial GCTs Late effects and quality of life FURTHER READING Current perspectives on childhood brain tumours: a review Introduction HeadSmart be brain tumour aware Difficulties in diagnosing congenital/infantile brain tumours Epilepsy in paediatric brain tumours Recent advances in paediatric brain tumours FURTHER READING Principles of chemotherapy Introduction Molecular basis of chemotherapy Resistance to chemotherapy Principles of combination chemotherapy FURTHER READING Symptom management during chemotherapy Introduction Assessment of symptoms Pain Mucositis Nausea and vomiting Weight loss and poor nutrition Fatigue Sleep disturbance Mood disturbance Discussion FURTHER READING Childhood leukaemia: an update Introduction Epidemiology Aetiology Pathogenesis Clinical features Differential diagnosis Laboratory features Classification AML ALL Prognosis Treatment AML Relapse Early and late toxicity Treatment in a resource poor setting Future strategies and conclusions FURTHER READING Update on non-Hodgkin lymphoma in children Introduction Epidemiology — overview Classification Clinical presentation Symptoms associated with localized disease NHL staging Investigation and diagnosis Treatment of non-Hodgkin’s lymphomas Case-based clinical presentations FURTHER READING Lymphadenopathy in Children and Young People Introduction History Examination Examination of the lymph nodes Occipital and post-auricular nodes Cervical and inguinal nodes Supraclavicular nodes Axillary nodes Inguinal nodes Investigation Practical differential diagnosis Main causes of lymphadenopathy Summary FURTHER READING Neuroblastoma Epidemiology, genetic predisposition and risk factors The clinical problem Clinical features Diagnostic markers Imaging Tissue diagnosis Pathology and genetic features Staging Role of screening Spontaneous regression and stage 4S disease Management Surgery Chemotherapy Radiotherapy Monitoring response to treatment Management of minimal residual disease (MRD) and relapse ‘Late effects’ and long-term outcomes FURTHER READING Advances in the understanding and management of histiocytic disorders 2015 Introduction Langerhans Cell Histiocytosis Non-Langerhans Cell Histiocytoses (Non-LCH) Haemophagocytic Lymphohistiocytosis (HLH) Histiocyte Lineage-related Malignancies Conclusion FURTHER READING Section 2: Haematology Leukaemias: a review Definition Incidence/100 000 under 16, UK and worldwide Management FURTHER READING Haematological assessment in non-accidental injury Introduction Normal haemostasis History and examination Examination Laboratory investigations Haematological disorders that can present with abnormal bleeding and/or bruising Conclusion FURTHER READING Newborn screening for haematological disorders Introduction Clinical importance of haemoglobinopathies Other haematological conditions in the newborn period Implementation of newborn screening for sickle cell disease Results from the newborn sickle cell screening programme Genetic issues raised by the programme Clinical standards and care of haemoglobinopathies Recent developments of clinical care Conclusion FURTHER READING Sickle cell disease, update on management Definition Epidemiology and distribution Physiology Genotypes Neonatal screening and routine management Diagnosis and management of complications Febrile children Stroke Transcranial Doppler scanning (TCD) in stroke prevention Acute splenic sequestration (ASS) Aplastic crisis Chest syndrome Priapism Gall stones Avascular necrosis Further treatments Other considerations FURTHER READING Diagnosis and management of childhood aplastic anaemia Introduction Pathogenesis Diagnosis Management Treatment choices in idiopathic SAA Non-severe aplastic anaemia Management of inherited bone marrow failure syndromes (IBMFS) Conclusion FURTHER READING Management of haemophilia Clinical features of haemophilia Genetic and molecular basis of haemophilia Treatment of haemophilia Inhibitors Carriers of haemophilia Management of pregnancy and care of affected neonates FURTHER READING Von Willebrand Disease: diagnosis and management Introduction Pathophysiology Classification of VWD FURTHER READING Diagnosis and management of thalassaemia Definition How common is thalassemia? Pathology, pathogenesis and applied physiology Molecular and cellular pathology Phenotypic diversity in beta thalassaemia Dominantly inherited beta thalassaemia Alpha thalassaemia Clinical effects of alpha thalassaemia Clinical course Thalassaemia intermedia (TI) Thalassaemia minor (thalassaemia trait) Diagnosis of thalassaemia History and physical examination Investigation of suspected thalassaemia Beta thalassaemia Beta thalassaemia trait Three gene deletion α thalassaemia One or two gene deletion alpha thalassaemia Outpatient management Ongoing management for transfusion dependent thalassaemia (see Table 1) Iron chelation Caring for thalassaemia patients in the community Care of thalassaemia patients in hospital Providing information and support Prognosis and explanation to the patient Prevention — of the primary disease Prevention — of disease complications Stem cell transplant (HSCT) Summary FURTHER READING Management of sickle cell disease: management of acute episodes in the community and in hospital Introduction Dactylitis Acute splenic sequestration (ASS) Acute painful crisis (APC) Acute chest syndrome (ACS) Acute stroke Abdominal crises Acute anaemic episodes Priapism Acute visual problems FURTHER READING Management of sickle cell disease: out-patient and community aspects Epidemiology Genetics Diagnosis and laboratory monitoring Neonatal screening Out-patient management Prevention of invasive pneumococcal disease (IPD) Prevention of stroke Silent cerebral ischaemia (SCI) Other common paediatric out-patient problems Schooling issues Therapeutic interventions to prevent acute and long-term complications of SCD Hydroxycarbamide (hydroxyurea, OHC) Blood transfusion Role of the GP General care and the multi-disciplinary approach FURTHER READING Hereditary spherocytosis Definition Epidemiology Pathology Course of the disease Diagnosis History Physical examination Investigations Differential diagnosis Management Iron and folate Vaccinations Anaemia management Iron monitoring and management of iron overload Management of splenomegaly Management of gallstones Transition to adult services Prognosis and explanation to patient Follow up FURTHER READING