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Index
Title page
Table of Contents
Copyright
Contributors
Preface
Acknowledgements
Section 1: Organisation, Delivery and Outcome of Neonatal Care
Chapter 1: Epidemiology
Introduction
Births and birth rates
The incidence of preterm birth and low birthweight
Multiple births
Mortality in the first year of life
Geographical variations and international comparisons in mortality
Classification of clinical causes of death
Confidential enquiries
Morbidity in childhood in relation to circumstances at birth
The increasing demand for neonatal care
The need for better data
Chapter 2: Organisation and evaluation of perinatal care
Organisation of care
Relationship of organisational factors to outcome
Philosophy of care and survival
Service evaluation
Definitions – mortality and morbidity
Neonatal data recording
Guidelines and evidence-based care
Conclusions
Chapter 3: Outcome following preterm birth
History: early neonatal care
Reporting outcomes and study design
Outcome in early infancy
School-age outcomes
The ex-preterm young adult
Chapter 4: Developmental care
Introduction
Brain development in relation to developmental care
Developmental care
Newborn Individualized Developmental Care and Assessment Program
Discussion of evidence for developmental care
Summary
Chapter 5: Counselling and support for parents and families
Introduction
The relationship with parents
Consent to treatment
Life and death decision-making
The dying baby
Conflict
Dealing with complaints
Special situations
The parent who is a healthcare professional
The mother with threatened extreme preterm labour
Preserving your own health
Concluding thoughts
Chapter 6: Ethical and legal aspects of neonatology
Introduction
Plurality of moral beliefs and ethical approaches
The value of life of the newborn
Recent philosophical challenges
Best interests
Balancing burdens and benefits
Withholding and withdrawing life-sustaining treatment
Neonatal euthanasia
Quality of life and disability perspectives
Resuscitation of extreme preterm
Conclusion
Introduction
Clinicians and litigation
Situations with a high risk of litigation in neonatology
Relations with parents
Discontinuation of futile treatment
Negligence
Criminal cases
Child protection law
Chapter 7: Science and the emergence of neonatal medicine
Introduction
Emerging care of the premature infant
Feeding the premature infant
Thermoregulation and the premature infant
Respiratory distress syndrome
Intraventricular haemorrhage
Periventricular leukomalacia
Necrotising enterocolitis
Retinopathy of prematurity
Birth asphyxia
Rhesus haemolytic disease
Section 2: Prenatal Life
Chapter 8: Basic genetics
The nature and structure of a gene
Decoding the information in DNA
Chromosomes and cell division
Chromosome analysis
Patterns of inheritance
Molecular genetic analysis for single gene disorders
New diagnostic genetic technology
Glossary
Chapter 9: Antenatal diagnosis and fetal medicine
Introduction
Fetal physiology
Antenatal diagnosis
Fetal medicine
Fetal surgery
Chapter 10: Fetal growth, intrauterine growth restriction and small-for-gestational-age babies
Introduction and definitions
Incidence and recurrence rate
The aetiology of IUGR
Physiological sequelae of IUGR
The consequences of IUGR
Primary prevention of IUGR
Antenatal screening and diagnosis
Management of IUGR and timing of delivery
Fetal therapy for IUGR
Chapter 11: Maternal illness in pregnancy
Thromboembolic disease
Hypertension
Pre-eclampsia
HELLP syndrome
Eclampsia
Heart disease
Diabetes Mellitus (see Ch. 22 for management of the infant of a diabetic mother)
Thyroid disease
Neurological disorders in pregnancy
Antiepileptic drugs
Renal disease and pregnancy
Renal transplantation and pregnancy
Haematology
Malignant disease and pregnancy
Respiratory disease
Rheumatological disorders
Liver disease
Gasteroentrology
Psychiatric diseases
Drugs of misuse
How are pharmacological agents handled in pregnancy and breastfeeding?
Chapter 12: Obstetrics for the neonatologist
Introduction
Intrapartum assessment and management
Diagnosis of labour
Interventions during labour
Electronic fetal heart rate monitoring
Fetal scalp blood sampling
Umbilical cord blood sampling
Delivery of the baby
Shoulder dystocia
Induction of labour
Breech presentation
Multiple pregnancy
Other high-risk situations for the fetus
Preterm infants
Section 3: Care around Birth
Chapter 13: Resuscitation and transport of the newborn
Epidemiology of neonatal resuscitation
The approach to newborn resuscitation
Assessment of the newborn
Techniques of resuscitation
Supporting the circulation
Drugs
Meconium-stained liquor
A baby born under general anaesthesia
Congenital anomalies
Babies still attached to the placenta
Ethics issues
Teaching and training
Research in neonatal resuscitation
Remit of neonatal transport
Clinical and physiological stresses and pretransport stabilisation
Clinical care during transport
Care at receiving hospital
Communication
Organisation of services
Section 4: General Neonatal Care
Chapter 14: Examination of the newborn
Introduction
Examination in the delivery room
Full routine examination
Systematic review
Gestational age
Chapter 15: Temperature control and disorders
Introduction
Body temperature control mechanisms
Normal heat transfer
Measurement of temperature
Body temperature and thermal neutrality
‘Normal’ temperature
Practical management of the thermal environment
Disorders of temperature control
Therapeutic hypothermia (see Ch. 40.4 for further details)
Chapter 16: Infant feeding
Nutritional programming
Nutrition during fetal life
Adaptations to extrauterine nutrition
Biological consequences of depriving babies of enteral feeds after birth
Non-nutritional consequences of enteral feeding
Individual nutrients: physiology and dietary needs of term and preterm infants
Breastfeeding
Artificial feeding for the normal term infant
Breast versus formula
General considerations
Choice of diet
Human milk
Types of breast milk
‘Term’ infant formulas
Special preterm infant formulas
Diets for preterm infants: overview
Route of administration of feeds
Feeding schedules
Psychosocial aspects
Postdischarge nutrition (Fewtrell 2003)
The term growth-retarded infant
Chapter 17: Parenteral nutrition
Composition of infusates
Techniques
Hazards
Transition to enteral nutrition
Conclusion
Appendix: How to write a total parenteral nutrition (TPN) prescription
Chapter 18: Fluid and electrolyte balance
Introduction
Postnatal alterations in body water distribution
Non-renal influences on water balance
Clinical implications of postnatal and developmental changes: constructing a fluid prescription
Monitoring fluid balance
Common clinical problems
Chapter 19: Intensive care monitoring and data handling
Introduction
Cardiovascular monitoring
Respiratory system monitoring
Neurological system monitoring
Temperature monitoring
Monitoring the sick baby
Clinical and laboratory monitoring
Data handling
Chapter 20: Care of the normal term newborn baby
Anticipatory care
Care immediately after birth
The normal neonate
Postnatal care
Routine neonatal clinical examination (Ch. 14)
Biochemical screening
Hearing screening
Feeding the normal term baby
Feeding problems
Chapter 21: Care of the normal small baby and convalescent NICU graduate
Introduction
The normal small baby
Care of the intensive-care graduate
Feeding the convalescent premature baby
Discharging small babies
Chapter 22: Neonatal complications following diabetes in pregnancy
Congenital abnormalities
Macrosomia
Hypertrophic cardiomyopathy
Intrauterine growth restriction
Effects of antenatal and intrapartum hypoxia–ischaemia
Respiratory complications
Hypocalcaemia and hypomagnesaemia
Disordered postnatal metabolic adaptation (see Ch. 34.1)
Iatrogenic complications
Outcome
Avoiding harm
Chapter 23: Twins
Epidemiology
Perinatal mortality
Zygosity
Chorionicity
Monoamniotic twins
Conjoined twins
Monochorionic, diamniotic twins
Postnatal determination of zygosity
Fetal growth in multiple pregnancy
Antenatal management
Chromosomal anomalies
Congenital anomalies
Death of one fetus
Intrapartum management
Postnatal management: routine
Co-bedding
Postnatal management: high-risk care
Bereavement
Social
Acknowledgement
Chapter 24: Pharmacology
Introduction
History of drug toxicity in neonates
Licensing
Drug handling
Drug prescribing and administration
Drug interactions
Drug therapy for the fetus
Drugs in breast milk
Therapeutic drug monitoring
Chapter 25: Neonatal analgesia
Introduction
Nociception in the infant
Effects of nociception
Measurement of pain/nociception in the neonate
Pharmacological considerations
Clinical analgesia
Chapter 26: The baby of a substance-abusing mother
Introduction
General effects of substance abuse in pregnancy
Approach to management of substance abuse in pregnancy
General management of infants of substance-abusing mothers
Specific drugs of abuse
Other drugs of abuse
Summary
Section 5: Disorders of the Newborn
Chapter 27: Pulmonary disease of the newborn
Introduction
Morphological development of the lung
Factors influencing lung growth and development
Pulmonary circulation
Cardiorespiratory adaptation at birth
Postnatal function
Gas exchange in the neonatal lung
Regulation of breathing
Responses to changes in oxygen tension (Fig. 27.4)
Response to carbon dioxide/acidosis
Respiratory reflexes
Lung mechanics (Table 27.3)
Surfactant
Respiratory distress syndrome
Transient tachypnoea of the newborn
Minimal respiratory disease
Pulmonary airleaks
Persistent pulmonary hypertension of the newborn
Meconium aspiration syndrome
Aspiration of amniotic fluid
Aspiration of fluid at delivery
Other aspiration syndromes (Table 27.8)
Pulmonary haemorrhage
Asphyxial lung disease/acute respiratory distress syndrome
Pleural effusions
Management of neonatal respiratory failure
Bronchopulmonary dysplasia
Other forms of chronic lung disease
Introduction
Definition
Incidence of clinical apnoea
Types of apnoea
Mechanisms of apnoea
Factors involved in apnoea
Bradycardia
Monitoring
Investigation of apnoea
Treatment of apnoea of prematurity
Stopping treatment, predischarge monitoring and discharge home
Prognosis
Pulmonary agenesis
Pulmonary hypoplasia
Small-chest syndromes
Congenital cystic adenomatoid malformation
Congenital lung cysts
Pulmonary sequestration
Congenital pulmonary lymphangiectasis
Pulmonary alveolar proteinosis (congenital alveolar proteinosis)
Congenital lobar emphysema
Ciliary abnormalities
Introduction
Congenital posterolateral diaphragmatic hernia
Anterior diaphragmatic hernias
Diaphragmatic eventration
Introduction
Development of the nose, larynx and trachea and lungs
Higher upper airway obstruction
Oropharyngeal airway obstruction and stertor
Laryngotracheal airway obstruction
Care of the intubated baby
Laryngotracheal airway obstruction: conditions and management
Chapter 28: Cardiovascular disease
Introduction
Fetal circulation
Fetal cardiology
Incidence and aetiology of fetal and neonatal heart disease
Clinical implications of adaptation to birth
History and examination
Investigations
Treatment
Persistent pulmonary hypertension of the newborn
Cardiovascular problems in the preterm infant
Structural heart disease in the newborn
Cardiac conditions likely to present in the asymptomatic newborn (Table 28.24)
Acknowledgement
Chapter 29: Gastroenterology
Introduction
Bilirubin biochemistry
Biochemical basis of phototherapy
The normal pattern of neonatal jaundice
Epidemiology and genetics
Jaundice in the healthy breastfed infant
Pathophysiology of bilirubin encephalopathy
Identifying the newborn at risk of bilirubin encephalopathy
Assessing the level of serum bilirubin
Identifying different types of jaundice
Clinical management of the jaundiced infant
Guidelines for the use of phototherapy and exchange transfusion
Introduction
Hepatitis syndrome of infancy
Identifying bile duct obstruction
Surgically correctable disorders
Structure and function of the developing gastrointestinal tract
Signs of gastrointestinal disease
Necrotising enterocolitis
Short bowel syndrome
Neonatal appendicitis
Intractable diarrhoea
Gastro-oesophageal reflux
Milk protein intolerance
Pancreatic disease
Schwachman–Diamond syndrome
Introduction
Mouth and nasopharynx
Oesophagus and stomach
Duodenum and small bowel
Large bowel
Anterior abdominal wall defects
Hernias
Acknowledgements
Chapter 30: Haematology
Developmental haemopoiesis
Neonatal anaemia and other red cell disorders
White cell disorders
Haemostasis and thrombosis in the newborn
Transfusion of blood and blood products in the newborn
Chapter 31: Malformation syndromes
Introduction
Basic terms in dysmorphology
Sequence
Clinical evaluation of a neonate with birth defects
Prenatal history
Perinatal history
Family history
Instant syndrome diagnosis by pattern recognition – gestalt
Investigating the malformed neonate in the absence of diagnosis
Genetic counselling and support for families
Chromosomal anomalies/microdeletion syndromes
Single-gene disorders identifiable in the neonatal period
Genetic conditions related to growth deficiency
Genetic conditions related to overgrowth
Chapter 32: Neonatal dermatology
Introduction
Structure and function of the neonatal skin
Pustules, blisters and erosions in the neonate
Other transient neonatal conditions
The red scaly baby
Birthmarks, congenital tumours and other naevoid conditions
Some congenital tumours
Aplasia cutis
Chapter 33: Ophthalmology
When does a neonatologist need an ophthalmologist?
Development of the eye
Development of vision
Amblyopia
Eye conditions that require screening examinations
Congenital abnormalities of the eyes: unusual appearances seen by the neonatologist
The infant with poor vision
Disorders of the eyes and related tissues
The infant with unusual eye movements
Chapter 34: Metabolic and endocrine disorders
Introduction
Glucose homeostasis in the healthy fetus and neonate
Hypoglycaemia
Hyperglycaemia
Summary
Acknowledgement
Hypothalamus and anterior pituitary
Posterior pituitary (Robertson 2001)
Thyroid
Disorders of calcium metabolism
Adrenal
Disorders of sex development
Introduction
The organisation of clinical and laboratory services
Pathogenesis of inborn errors of metabolism
Clinical presentation of inborn errors of metabolism
Investigation of a baby who may have an inborn error of metabolism
Consideration of specific inborn errors of metabolism
Background
Diagnosing metabolic bone disease in preterm infants
Aetiology
Treatment: prevention is better than cure
Inherited metabolic bone disease
Chapter 35: Disorders of the kidney and urinary tract
Morphological development of the kidney
Assessing renal function
Chronic renal failure
Polycystic kidney disease
Congenital nephrotic syndrome
Tubulopathies
The renal sequelae of preterm birth
Urinary tract anomalies
Abnormal formation, migration and fusion of the kidney
Genitourinary tract anomalies
Chapter 36: Neonatal malignancy
Introduction
Incidence and survival
Neuroblastoma
Teratoma and other germ cell tumours
Acute leukaemia
Transient myeloproliferative disorder and Down syndrome
Renal tumours
Brain tumours
Retinoblastoma
Sarcomas
Liver tumours
Histiocytosis
Other rare tumours
Chapter 37: Orthopaedic problems in the neonate
Introduction
Lower limb abnormalities
Lower limb deficiencies
Upper limb abnormalities
Upper limb deficiencies
Abnormalities of the spine and axial skeleton
Fractures in the newborn
Vascular conditions associated with orthopaedic problems in the newborn
Nerve and muscle conditions associated with orthopaedic problems in the newborn
Infectious disease associated with orthopaedic problems in the newborn
Chapter 38: Neonatal gynaecology
The physiology of the fetal hypothalamopituitary axis
Neonatal breast development
Vulval problems
Abnormalities of the hymen
Vaginal bleeding problems
Uterine prolapse
Urethral prolapse
Paraurethral cysts
Ovarian cysts
Chapter 39: Neonatal infection
Introduction
The immune system
The development of the immune system
The maternofetal immunological relationship
Immune development and the neonatal gut (written with Alison Bedford Russell)
Immunodeficiency disorders: general principles
Diagnosis and investigation of immunodeficiency
Specific primary immunodeficiencies
DNA repair defects and immunodeficiency
Immunoregulation disorders
Other immunodeficiencies
Defects of antibody production
Disorders of phagocytic cells
Treatment of immunodeficiency
The origins of nosocomial infection related to feeding practices
Neonatal skin and infection
Incidence of infection and causative organisms
Clinical presentation, investigation and management of neonatal sepsis
Treatment of neonatal sepsis
Treatment of fungal infection
Treatment of ‘very sick septic neonates’
Additional (adjuvant) treatments for infection
Prevention of infection – infection control
Care bundles in the reduction of nosocomial infection
Focal bacterial infections in the newborn
Specific neonatal lung infections
Superficial infections
Other neonatal bacterial infections
Perinatal viral infections
Parasitic infections
Non-bacterial congenital infections
Congenital infections in the developing world
Other congenital infections
Chapter 40: Neurological problems in the newborn
Introduction
Examination systems
Scheme of neonatal neurological examination
Neonatal neurological alarm signals
Special senses
Imaging
Examination of cerebrospinal fluid and intracranial pressure
Electroencephalography
Neurological examination before discharge, and follow-up
Introduction
Pathophysiology
Incidence
Time of onset
Diagnosis and classification
Physiological changes during seizures
EEG diagnosis of seizures
Electrographic seizures
Electroclinical dissociation
Aetiology
Metabolic causes
Congenital malformations of the brain
Inborn errors of metabolism
Syndromic neonatal seizures
Epileptic encephalopathies
Early myoclonic epilepsy (EME)
Introduction
Incidence and prevalence of intracranial haemorrhage
Risk factors for intracranial haemorrhage
Clinical presentation of intracranial haemorrhage
Investigations
Pathogenesis of subdural haemorrhage
Mechanism of subdural haemorrhage
Clinical features of subdural haemorrhage
Clinical management and prognosis following subdural and intracranial haemorrhage
Extradural haemorrhage
Subarachnoid haemorrhage
Intraventricular, intraparenchymal, thalamic and cerebellar haemorrhage
Subgaleal (subaponeurotic) haemorrhage
Perinatal stroke
Thrombophilic state and the newborn
Perinatal arterial ischaemic stroke
Cerebral sinovenous thrombosis
Introduction
Pathophysiology of hypoxia–ischaemia
Pathology
Clinical aspects
The preterm infant with hypoxic–ischaemic injury
Neuroprotective treatments
Neurodevelopmental outcome
Preterm cerebral haemorrhage
Preterm brain injury: white-matter disease of prematurity
Conclusion
Introduction
Disorders of myelin and myelination
Neuroaxonal dystrophies
Neuronal degeneration
Pontocerebellar hypoplasias
Intracerebral calcification
Vasculopathies
Introduction
Clinical presentation
Obstetric history
Clinical investigations
Congenital myotonic dystrophy
Congenital muscular dystrophy
Forms of congenital muscular dystrophy with structural brain changes and eye involvement
Motor neuron disorders
Congenital myopathies
Myasthenia gravis
Introduction
Epidemiology of CNS malformations
Cephaloceles
Neural tube defects
Disorders of prosencephalic development
Malformation of cortical development
Ventriculomegaly
Posterior fossa abnormalities
Arachnoid cysts
Cerebrovascular malformations
Section 6: Pathology, Radiology and Biochemistry
Chapter 41: The perinatal postmortem examination
Introduction
Role of the perinatal autopsy
Contribution of autopsy findings
Factors influencing the value of the perinatal autopsy
Changes in perinatal autopsy rates
Attitudes to the perinatal postmortem examination
Consented versus coronial autopsies
Consent
The postmortem examination
The minimally invasive autopsy
Placental examination
Specific neonatal autopsy scenarios and common associated autopsy findings
Conclusion
Chapter 42: The clinical biochemistry laboratory
Introduction
Specimen collection
Reference ranges
Alert limits
Quality control, accreditation and clinical governance
Point-of-care testing
Inherited metabolic disease
Neonatal screening
Conclusion
Chapter 43: Neonatal imaging
Introduction
Radiation safety
Chest imaging
Chest ultrasonography
Magnetic resonance imaging of the chest
Abdominal imaging
Genitourinary imaging
Musculoskeletal imaging
Central nervous system imaging
Section 7: Practical Procedures
Chapter 44: Procedures and iatrogenic disorders
Introduction
Consent for procedures
Pain management (Ch. 25)
Aseptic technique and skin preparation
Procedures and their complications
Appendices
Appendix 1: Haematological values in the newborn
Appendix 2: Coagulation values in term and preterm infants
Appendix 3: Normal ranges for commonly assessed ECG values in the newborn
Appendix 4: Normal values for neonatal blood pressure
Appendix 5: UK-WHO chart source data
Appendix 6: Neonatal biochemical reference ranges
Appendix 7: Normal blood gas values
Appendix 8: Normal cerebrospinal fluid values
Appendix 9: Oxyhaemoglobin dissociation curve
Appendix 10: The Siggaard-Andersen nomogram
Instructions for online access
Index
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