Fundamentals of Children's Applied Pathophysiology E-Book

Ian Peate

• Herausgeber: John Wiley & Sons
• Kategorie: Fachliteratur
• Serie: Fundamentals
• Sprache: Englisch

Seitenzahl: 815

Veröffentlichungsjahr: 2018

Beschreibung

Fundamentals of Children's Applied Pathophysiology introduces nursing and healthcare students to the pathophysiology of the child, and offers an applied full-colour visual approach throughout. Explaining the anatomy of the human body, and the effects of disease or illness on normal physiology, it enables the reader to develop the understanding, knowledge, and skills required to know how to respond and provide safe and effective high-quality care to children and their families. Key features: * Written by an experienced author team * Filled with superb full-colour illustrations * Packed with learning features, including key words, test-your-knowledge, exercises, further reading, and learning outcomes * Includes case studies to help readers understand how to apply the knowledge in clinical practice * Contains links to clinical observations, vital signs to look out for, investigations boxes, red flags to indicate essential information to be aware of when providing care, and medication alerts Fundamentals of Children's Applied Pathophysiology is an ideal book for pre-registration nursing students, including child and adult nurses, as well as for all healthcare professionals who come into contact with children and their families.

Leseprobe

Table of Contents

Cover

Preface

Reference

Chapter 1: The cell and body tissue

Aim

CELLS

TISSUES

References

Chapter 2: Genetics

Aim

The cell nucleus and DNA

Introduction

Genes

The double helix

From DNA to proteins

Genetic mutations

The transference of genes

Mendelian genetics: the role of genetics in inheritance

Dominant genes and recessive genes

Autosomal dominant inheritance and ill health

Autosomal recessive inheritance and ill health

Morbidity and mortality of dominant versus recessive disorders

X‐linked recessive disorders

Spontaneous mutation

Conclusion

References

Chapter 3: Cancer

Aim

Introduction

Biology of cancer

Pathogenesis of cancers

Aetiology of cancers

Signs and symptoms

Staging cancers

Central nervous system tumour staging

Treatment of cancer

Prevention of cancer

Examples of cancers

Outcomes and survivorship

Conclusion

References

Chapter 4: Homeostasis

Aim

Introduction

Dehydration

Dehydration due to gastrointestinal fluid loss

Dehydration due to blood loss

The child requiring surgery

The child with respiratory impairment

The child with renal impairment

The child with an infection

The diabetic child

Escalating your concerns as a nurse

Conclusion

References

Chapter 5: Inflammation, immune response and healing

Aim

Introduction

The immune system

Acquired immunity

Alterations in immunity and inflammation

Care of a child with anaphylaxis

Autoimmune disease

Care and management of a child with primary immunodeficiency

Acquired immunodeficiency syndrome

Conclusion

References

Chapter 6: Shock

Aim

Introduction

Distributive shock

Anaphylactic shock

Hypovolaemic shock

Cardiogenic shock

A common pathway to the pathophysiology of shock

Strategies for caring for and managing the child in shock

Monitoring end‐organ perfusion

A systematic approach to assessing the patient in shock

Summary and conclusion

References

Chapter 7: Pain and pain management

Aim

Introduction

Anatomy and physiology of pain in children

Gate control theory

Common myths about anatomy and physiology of children’s pain

Common approaches to assessing pain in infants, children and young people

Cognitive development and the perception of pain

Gender and cultural aspects of pain in children, young people and their families

Methods of pain assessment (see Table 7.3)

Pain management

Management of procedural pain

Conclusion

References

Chapter 8: Disorders of the nervous system

Aim

Introduction

Structure of the nervous system

Disorders of the nervous system

Causes of neurological dysfunction

Congenital causes

Acquired causes

Cerebral dysfunction

Cerebral trauma

Spinal trauma

Motor dysfunction

Neurological tumours

Intracranial infection

Seizure disorders

Epilepsy

Conclusion

References

Chapter 9: Disorders of the cardiac system

Aim

Introduction

Anatomy of the heart

Congenital disorders of the heart

Classification of congenital heart defects

Cardiac investigations

ACYANOTIC CONGENITAL HEART DEFECTS

CYANOTIC CONGENITAL HEART DEFECTS

Conclusion

References

Chapter 10: Disorders of the respiratory system

Aim

Introduction

Respiratory assessment

Respiratory noises

Measuring oxygen saturations

Skin colour

Heart rate

Mental status

Oxygen therapy

Clearance of nasal and oral secretions

Infection control precautions

Bronchiolitis

Upper airway disorders

Altered physiology

Conclusion

References

Chapter 11: Disorders of the endocrine system

Aim

Introduction

Endocrine anatomy and function

Endocrine disorders

Disorders of the hypothalamus and pituitary gland

Disorders of the pineal gland

Disorders of the thyroid gland

Parathyroid disorders

Disorders of the thymus gland

Disorders of the adrenal glands

Disorders of the pancreas

Disorders of the gonads

Conclusion

References

Chapter 12: Disorders of the digestive system

Aim

Introduction

Motility disorders

Inflammatory conditions

Obstructive disorders

Malabsorption syndromes

Structural defects

Hernias

Paediatric early warning system – related to a case study

References

Chapter 13: Disorders of the renal system

Aim

Introduction

Antenatal detection of renal anomalies

Assessment of the renal system

Medical disorders of the upper urinary tract

Chronic kidney disease

Acute kidney injury

Haemolytic uraemic syndrome

Wilms’ tumour

General medical renal nursing principles

Surgical disorders of the upper urinary tract

General surgical renal nursing principles

Medical disorders of the lower urinary tract

Surgical disorders of the lower urinary tract

Conclusion

References

Chapter 14: Disorders of the reproductive systems

Aim

Introduction

The male reproductive system

Male reproductive issues

Female reproductive health issues

Sexual health: both girl and boys

Sexual activity

Contraception

Female genital mutilation

Conclusion

References

Chapter 15: Disorders of the musculoskeletal system

Aim

Introduction

Fractures

Developmental dysplasia of the hip

Conclusion

References

Chapter 16: Disorders of the skin

Aim

Introduction

Type of lesion

Epidermolysis bullosa

Wounds

Epidermal stripping

Burns

Pediculosis (lice)

Conclusion

References

Index

End User License Agreement

List of Tables

Chapter 03

Table 3.1 Examples of congenital factors associated with childhood cancer

Table 3.2 UK risk stratification to the treatment of acute lymphoblastic leukaemia (ALL)

Table 3.3 Tumour grades

Table 3.4 Mandatory investigations for all new suspected cases of malignancy

Chapter 04

Table 4.1 Signs and symptoms of medical shock

Table 4.2 Blood tests used in dehydration

Chapter 05

Table 5.1 Different types of cells involved in the innate and adaptive immunity

Table 5.2 T‐cell functions

Table 5.3 Functions of antibodies

Table 5.4 Types of hypersensitivity reaction

Table 5.5 Allergic diseases that affect children

Chapter 06

Table 6.1 Classification of shock

Table 6.2 SIRS to septic shock

Table 6.3 Causes of cardiogenic shock in children

Table 6.4 Cardiogenic shock related to right ventricular failure

Table 6.5 Cardiogenic shock related to left ventricular failure

Table 6.6 Pathophysiology of the signs and symptoms of cardiogenic shock

Table 6.7 The goals of treatment of children with cardiogenic shock

Chapter 07

Table 7.1 The experience of individual and biopsychosocial factors

Table 7.2 Size and speed of first‐order sensory fibres

Table 7.3 Validated, age‐appropriate pain assessment tool suggestions

Table 7.4 Pain and behavioural cues

Table 7.5 Non‐opioid drugs

Table 7.6 Examples of opioid drugs

Table 7.7 Patient observations during PCA administration and for 4 hours following discontinuation

Table 7.8 Observations required for a child with a continuous epidural with levobupivacaine with/without fentanyl

Chapter 08

Table 8.1 Anatomical structure of the nervous system

Table 8.2 Vital signs and symptoms of a fractured skull

Table 8.3 Investigations to assess level of consciousness

Table 8.4 Vital signs and symptoms of paediatric brain tumours

Table 8.5 Vital signs and symptoms of meningitis and meningococcal disease

Chapter 09

Table 9.1 Possible causes for CHD

Table 9.2 Possible causes of CF not associated with congenital heart defects

Table 9.3 Drugs that may be used in the treatment of heart failure (Joint Formulary Committee, 2015)

Table 9.4 Patent ductus arteriosus in the premature infant

Table 9.5 Manifestations of rheumatic fever

Chapter 10

Table 10.1 Features of acute/severe and life‐threatening asthma

Chapter 11

Table 11.1 Overview of the endocrine system, its associated functions and disorders. (For full details of endocrine system function and specific hormones as relevant to children, see Petty, 2015)

Chapter 12

Table 12.1 Possible causes of acute and chronic diarrhoea

Table 12.2 Possible causes of vomiting

Table 12.3 Possible complications of gastro‐oesophageal reflux (GOR)

Table 12.4 Clinical manifestations of inflammatory bowel diseases (IBD)

Table 12.5 Symptoms of coeliac disease

Table 12.6 Pre‐ and post‐operative nursing care of the infant with a cleft lip and/or palate

Table 12.7 Causes of potential alteration in post‐operative vital signs

Chapter 13

Table 13.1 Antenatal urinary tract abnormalities

Table 13.2 Assessment of the renal system

Table 13.3 Some causes of proteinuria

Table 13.4 Possible signs and symptoms of glomerulonephritis

Table 13.5 Causes of CKD in children

Table 13.6 Consequences of CKD

Table 13.7 Stages of CKD and GFR

Table 13.8 Classification of AKI

Table 13.9 Staging Wilms’ tumour

Table 13.10 Symptoms and signs of UTI in infants and children

Chapter 14

Table 14.1 Some common male reproductive conditions

Table 14.2 Some common female reproductive conditions

Table 14.3 Examples of abdominal pain presentation in adolescent girls

Table 14.4 Teenage conception statistics

Table 14.5 Classification of female genital mutilation

Chapter 15

Table 15.1 Location and features of fractures

Table 15.2 Types of traction and examples of each

Table 15.3 Some causes of limp in childhood

Table 15.4 Radiological classification of SCFE

Table 15.5 Key terms associated with DDH

Chapter 16

Table 16.1 Skin conditions of childhood

Table 16.2 Harry’s vital signs

Table 16.3 Harry’s blood test results

Table 16.4 Age‐related factors to consider in skin care

Table 16.5 Classification of burns and presentation of injury.

Table 16.6 Burn severity classification

List of Illustrations

Chapter 01

Figure 1.1 Examples of different types of cells in the body.

Figure 1.2 Structure of the cell.

Figure 1.3 Cell membrane.

Figure 1.4 Facilitated diffusion.

Figure 1.5 Effect of solute concentration on a red blood cell.

Figure 1.6 Endoplasmic reticulum.

Figure 1.7 The cytoskeleton.

Figure 1.8 Simple epithelium.

Figure 1.9 Stratified epithelium.

Figure 1.10 Connective tissue reinforces epithelial tissue.

Figure 1.11 Simple cuboidal.

Figure 1.12 Simple columnar.

Figure 1.13 Where cartilage is found in the body.

Figure 1.14 Skeletal muscle cells.

Figure 1.15 Smooth muscle.

Figure 1.16 Peristalsis.

Figure 1.17 Nerve cell.

Chapter 02

Figure 2.1 The cell nucleus.

Figure 2.2 A pictorial representation of a portion of the double helix.

Figure 2.3 DNA from double helix to chromosome.

Figure 2.4 Male human chromosomes.

Figure 2.5 The separation of DNA and production of further DNA.

Figure 2.6 Brief summary of protein synthesis.

Figure 2.7 The cell cycle.

Figure 2.8 Mitosis.

Figure 2.9 Gene crossover.

Figure 2.10 Genetic inheritance.

Figure 2.11 X‐linked inheritance.

Figure 2.12 PEWS chart for William.

Chapter 03

Figure 3.1 Incidence rates for childhood cancer.

Figure 3.2 Possible signs and symptoms of solid and CNS tumours.

Chapter 04

Figure 4.1 Regulation of aldosterone by the renin‐angiotensin‐aldosterone pathway.

Figure 4.2 Locations of areas of the respiratory centre.

Figure 4.3 Regulation of breathing in response to changes in blood pCO

2

, pO

2

and pH (H

+

concentration) via negative feedback control.

Figure 4.4 Cellular and humoral immune responses.

Figure 4.5 Flowchart of inflammatory events.

Figure 4.6 Negative feedback regulation of the secretion of glucagon (blue arrows) and insulin (orange arrows).

Chapter 05

Figure 5.1 Distribution of organs, tissues and lymph vessels and nodes.

Figure 5.2 Diagram showing development of blood cells.

Figure 5.3 Features of the innate immune system.

Figure 5.4 Stages of phagocytosis: (a) stage 1, (b) stage 2 and (c) stage 3.

Figure 5.5 Inflammation and the stages that occur.  

Figure 5.6 Flowchart of inflammatory events.  

Figure 5.7 Stages of wound healing.

Figure 5.8 Diagram to show pilonidal abscess.

Figure 5.9 Diagram to show systemic signs of infection.

Figure 5.10 Primary and secondary responses to infection.

Figure 5.11 Acquired (specific) immunity.

Figure 5.12 Diagram of the allergic march.

Figure 5.13 Diagram of allergic symptoms.

Figure 5.14 Emergency treatment of anaphylaxis.

Figure 5.15 Features of primary immunodeficiency.

Figure 5.16 Features of secondary immunodeficiency.

Chapter 06

Figure 6.1 The clinical features of warm and cold shock.

Figure 6.2 Life‐threatening complications of septic shock.

Figure 6.3 Paediatric code red.

Figure 6.4 Cardiac output and its variables.

Figure 6.5 Stroke volume and its variables.

Figure 6.6 Oxygen delivery, blood pressure and its variables.

Chapter 07

Figure 7.1 Pathway of pain transmission and interpretation.

Figure 7.2 The ascending pain pathway.

Figure 7.3 Basic structure of myelinated and unmyelinated nerve fibres.

Figure 7.4 The gate control theory of pain.

Chapter 08

Figure 8.1 Organisation of the nervous system.

Figure 8.2 Raised intracranial pressure (ICP).

Figure 8.3 Focal and diffuse brain injury types.

Figure 8.4 Anterior view of frontal section through the skull showing the cranial meninges.

Chapter 09

Figure 9.1 Diagrammatic representation of the blood flow through the heart.

Figure 9.2 Classification of CHD.

Figure 9.3 Diagram showing oxygen saturation levels within the heart.

Figure 9.4 Diagram showing pressure in mmHg within the heart.

Figure 9.5 Diagram of ASD showing oxygenated blood flowing from the left atrium to the right atrium.

Figure 9.6 Diagram of VSD showing oxygenated blood flowing from the left ventricle to the right ventricle.

Figure 9.7 Diagram of PDA showing oxygenated blood returning to the heart from the aorta.

Figure 9.8 Diagram of pulmonary stenosis with right ventricular hypertrophy.

Figure 9.9 Diagram showing (a) normal tricuspid aortic valve; (b) stenotic tricuspid aorta valve; (c) abnormal bicuspid aorta valve.

Figure 9.10 Diagram showing (a) preductal coarctation of the aorta; (b) postductal coarctation of the aorta.

Figure 9.11 Diagram of tetralogy of Fallot.

Figure 9.12 Diagram of tricuspid atresia.

Figure 9.13 Transposition of the great arteries. The aorta takes blood from the right ventricle and the pulmonary artery takes blood from the left ventricle.

Figure 9.14 PEWS chart.

Figure 9.15 Position of chest ECG leads.

Chapter 10

Figure 10.1 Choking algorithm.

Figure 10.2 Difference between a normal airway and an asthmatic airway.

Chapter 11

Figure 11.1 The endocrine system.

Chapter 12

Figure 12.1 Distended sigmoid colon in Hirschsprung disease.

Figure 12.2 Common location of pain in children and adolescents with appendicitis.

Figure 12.3 Hypertrophic pyloric stenosis. (a) Enlarged muscular tumour nearly obliterates pyloric canal. (b) Longitudinal surgical division of muscle down to submucosa establishes adequate passageway.

Figure 12.4 Ileocaecal valve (ileocolic) intussusception.

Figure 12.5 (a)‐(e) Five most common types of oesophageal atresia and tracheoesophageal fistula. (See text for discussion)

Figure 12.6 Cleft lip and cleft palate.

Figure 12.7 (a)‐(d) Stages in palatine development. (See text for discussion).

Figure 12.8 Development of inguinal hernias. (a) and (b), prenatal migration of processus vaginalis (c), Normal. (d), partially obliterated processus vaginalis. (e), Hernia, (f), Hydrocele.

Figure 12.9 PEWS chart for Paul in Case Study 1.

Chapter 13

Figure 13.1 Upper and lower urinary tract.

Figure 13.2 Duplex left kidney.

Figure 13.3 The nephron.

Figure 13.4 Peritoneal dialysis.

Figure 13.5 Haemodialysis.

Figure 13.6 Renal transplantation.

Figure 13.7 Ureteropelvic junction obstruction.

Figure 13.8 Male reproductive system.

Figure 13.9 Classification of hypospadias.

Figure 13.10 Ureterovesical junction obstruction.

Figure 13.11 Stages of vesicoureteric reflux.

Figure 13.12 Posterior urethral valves.

Figure 13.13 PEWS chart for Emily.

Chapter 14

Figure 14.1 The male reproductive system.

Figure 14.2 The development of the male reproductive tract.

Figure 14.3 The female reproductive system.

Figure 14.4 The uterus and associated structures.

Figure 14.5 Prevalence of FGM (Africa).

Figure 14.6 Prevalence of FGM (globally).

Chapter 15

Figure 15.1 Ross’s PEWS chart.

Figure 15.2 Fracture types.

Figure 15.3 Stages in bone healing.

Figure 15.4 Application of traction to maintain bone alignment.

Figure 15.5 Ilizarov frame.

Figure 15.6 Normal femur and femur with slipped capital femoral epiphysis.

Figure 15.7 Growth plates of the femur.

Figure 15.8 Avascularity of the femoral head.

Figure 15.9 Abduction of lower limb.

Figure 15.10 Trendelenburg gait. (a) Normal hip joints. (b) There is a dip on the opposite of the pelvis when weight bearing on the affected side.

Figure 15.11 Pavlik harness.

Figure 15.12 Hip spica.

Figure 15.13 Barlow test.

Figure 15.14 Ortolani’s test.

Chapter 16

Figure 16.1 Cross‐section of the skin and primary sites of blister formation.

Figure 16.2 Wound healing by primary intention.

Figure 16.3 Healing by secondary intention‐infected wound.

Figure 16.4 Degree of burn injury.

Figure 16.5 Chart to determine extent of burn injury.

Figure 16.6 Jackson’s burns zones.

Figure 16.7 Systemic impact of burn injury.

Figure 16.8 The life‐cycle of head lice.

Guide

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Fundamentals of Children’s Applied Pathophysiology

An Essential Guide for Nursing and Healthcare Students

EDITED BY

LIZ GORMLEY‐FLEMING

Associate DirectorAcademic Quality Assurance at University of HertfordshireHatfield, UK

IAN PEATE OBE FRCN

Visiting Professor of NursingSt George’s University of London Kingston University London, UKEditor in Chief British Journal of NursingHead of School, School of Health StudiesUniversity of Gibraltar

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List of contributors

Ann L. Bevan RN, RSCN, PhD, MSc, FHEA, PGCEDSenior Lecturer, Children’s and Young People’s Nursing, Faculty of Health and Social Science, Bournemouth University, UKAnn is senior lecturer in the Children’s and Young People’s nursing programme in the Faculty of Health and Social Science at Bournemouth University. She has been a registered nurse for nearly 40 years, has qualifications in adult nursing, midwifery and children’s nursing, and is a qualified teacher. Ann has nursed in many areas in the UK and also in Hong Kong. She returned to the UK in January 2010 after nursing and teaching for 16 years in Canada. Research interests are children’s nutrition and childhood obesity, and also all aspects of child health. Her primary research methodology is action research.

Mary Brady RN, RSCN, CHSM, BSc, PGCHE, MSc, SHEASenior Lecturer, Child Field Cohort Lead, Exams and Assessment Tutor, Kingston University, UKMary is a senior lecturer and field lead for children’s nursing at Kingston University. She has a lengthy and extensive knowledge of the clinical care required for children in a variety of settings (neonatal units, paediatric intensive care and general paediatric wards). She has held posts as sister in neonatal/infant surgery, a neonatal unit and a general children’s ward. Since 2004, she has been teaching pre‐ and post‐registration nurses, midwives and paramedics. In 2016, she took on the role of Exams and Assessment Tutor for the School of Nursing. Mary is also an external examiner at Huddersfield University.In January 2015, she joined the RCN Children and Young People’s Professional Issues Forum and has contributed to RCN various publications.Mary is interested in all aspects of children’s nursing and more recently has researched the preparation of first‐year student child nurses for their first clinical practice placements.

Petra Brown RGN, DP, SN, BSc (Hons), MALecturer, Faculty of Health and Social Sciences, Bournemouth University, UKPetra began her nursing a career in 1988 at Salisbury School of Nursing, becoming a staff nurse. She has worked in a variety of clinical areas including recovery, telephone triage, intensive and coronary care. On completion of her degree in critical care, Petra started her career in nurse education as a practice educator for critical care, A & E and orthopaedics. On completion of a Master’s degree in health and social care practice education, she was appointed as a lecturer at Bournemouth University on the overseas and pre‐registration nursing courses. Her key areas of interest are nursing practice, anatomy and physiology, nurse education, practice development, respiratory and critical care.

Usha Chandran RN (Mental Health, Adult, Child), PGCEA, MSc Critical Care Nursing (Adults), MSc Health and DiseaseLecturer/Practitioner Paediatric Intensive Care Unit (PICU), St George’s Hospital, Tooting, UKSenior Lecturer, Child Health, Kingston University, UKUsha trained as a mental health, adult and children’s nurse, specialising in postnatal mental health disorders and eating disorders in mental health and intensive and critical care nursing in adult and children’s nursing.Usha has extensive experience both as an expert clinical nurse, nurse manager and nurse lecturer. She managed a mother and baby mental health unit and functioned as a practice development nurse for a nurse‐led eating disorder clinic (anorexia nervosa) at Springfield University Hospitals, Tooting, UK. Usha facilitated workshops at eating disorder nursing conferences delivered training as a nurse teacher for mental health nursing.Usha is an expert intensive and critical care nurse with expertise in adult and children’s critical care nursing and enjoys teaching this subject very much and helping new and novice nurses develop in this area of care. Her main specialities are in applied physiology and intensive care management and interventions. She worked in a combined cardiac and general adult intensive care unit in Melbourne, Australia for a period of eight months and presented a poster on sedation scales – the next generation at the British Association of Critical Care Nursing in York, UK. Usha is particularly interested in simulation training.Usha is the lead nurse for children’s critical care nurse training and development at St George’s Hospital, Tooting, London, UK, the nursing PI for a multi‐centre trial on sedation and weaning in children (SANDWICH) trial and the module leader for the PICU course modules at the joint faculty: Kingston University & St George’s, University of London.

Rosemary Court RGN, RSCN, RNT, BSc (Hons), MSc, FHEASenior Lecturer in Children’s Nursing, Sheffield Hallam University, Sheffield, UKRosemary began her nursing career in 1986 at Sheffield School of Nursing, becoming a registered general nurse and a registered sick children’s nurse working in a neonatal intensive care unit. She completed a course in special and intensive care of newborns and worked as a neonatal sister for 13 years. Rosemary went on to work in the community, becoming a community specialist practitioner children’s nurse and a nurse independent prescriber. She worked within a children’s complex care team for 5 years before taking up a position in nurse education. In 2012, Rosemary joined Sheffield Hallam University as a senior lecturer in children’s nursing, where she teaches on both pre‐ and post‐registration nursing courses. Her key areas of interest are children’s critical care nursing, children’s respiratory nursing, public health, advancing practice, and nurse education.

Susan Fidment RGN, RSCN, RNT, BSc, MSc, FHEASenior Lecturer in Children’s Nursing, Sheffield Hallam University, Sheffield, UKSue began her general nursing career in 1987 at Leeds General Infirmary. After she qualified as an RGN, she spent a brief period working in care of the elderly at Leicester General Hospital, before moving back to Leeds to commence a career in children’s nursing. She has worked in children’s orthopaedics and plastics at St James’s Hospital, moved on to commence RSCN education and then worked at Killingbeck Hospital in children’s cardiology. In 2012, after 16 years working within paediatric critical care both clinically and in education, she became a full‐time lecturer. Sue has a specialist interest in critical care of the child and teaches on advanced paediatric life support programmes. She is particularly interested in using simulation as a teaching methodology.

Liz Gormley‐Fleming RGN, RSCN, RNT, PGCert (Herts), PGDip HE (Herts), BSc (Hons), MA (Keele), SFHEAAssociate Director of Academic Quality Assurance, Centre for Academic Quality Assurance, University of Hertfordshire, UKLiz commenced her nursing career in Ireland where she qualified as an RGN and RSCN. Initially working in paediatric oncology and bone marrow transplant, Liz moved to London and has held a variety of senior clinical nursing and leadership roles across a range of NHS Trusts both in the acute care setting and in the community. Liz has worked in education since 2001, initially as a clinical facilitator before moving into full‐time higher education in 2003.Liz has held a number of senior leadership positions in higher education: Head of Nursing (Children’s, Learning Disability, Mental Health), Social Work Associate Dean of School, and Principal Lecturer in Learning and Teaching. Her areas of interest are care of the acutely ill child, healthcare law and ethics, the use of technology in higher education, curriculum development, and work‐based learning.

Kate Heaton‐Morley RNMH, RGN, RSCN, BSc (Hons), RNT, MSc, FHEASenior Lecturer Children’s Nursing, Sheffield Hallam University, Sheffield, UKKate’s career began in 1982 undertaking RNMH training (now RNLD) at Whittington Hall Hospital, Chesterfield. Subsequently training as a RGN and RSCN, Kate’s practice since 1991 has been dedicated to children’s nursing. She has worked and trained in the Sheffield Hospitals as a staff nurse, then sister, in acute and A & E settings. In 1998, Kate took up a senior sister post at Chesterfield Royal Hospital and, successively, positions of practice development advisor, matron, and senior matron. In 2007, to pursue her interest in nurse education, Kate joined Sheffield Hallam University as a senior lecturer in children’s nursing. She maintains her clinical skills and passion for children’s nursing through working as a staff nurse on the acute paediatric ward at Chesterfield Royal Hospital.

Sarah McDonald RSCN, BA (Hons), PGCert, RNT, FHEASenior Lecturer in Children’s Nursing, Sheffield Hallam University, Sheffield, UKSarah completed her RSCN Honours degree at Sheffield Hallam University in 1995. She consolidated her degree as a staff nurse in acute medicine at Rotherham District General Hospital. This was followed by positions as staff nurse then senior staff nurse at Sheffield Children’s Hospital in trauma, orthopaedics and plastics. Her other positions include sister in outpatient theatre at the Charles Clifford Dental Hospital and senior staff nurse in post‐anaesthetic care. Sarah became a clinical nurse educator at Sheffield Children’s Hospital in 2012. In 2014, she commenced a secondment as a lecturer in children’s nursing and is now a full‐time senior lecturer at Sheffield Hallam University. Sarah’s teaching interests include pain, tissue viability, and practical nursing skills. She has published guidelines for clinicalskills.net.

Elizabeth Mills RGN, RSCN, RNT, BSc (Hons), PGCert, PGDip, MSc, FHEASenior Lecturer in Children’s Nursing, Sheffield Hallam University, Sheffield, UKElizabeth began her nursing career in 1992 at West Yorkshire School of Nursing, Halifax, becoming a RGN and then a RSCN. After working in acute paediatric respiratory care, Elizabeth changed career direction and moved into neonatal intensive care, completing a course in the special and intensive care of newborns. Elizabeth worked in both district general and subregional neonatal intensive care units, and in 2005 she became a clinical educator within a large NHS Trust. In 2008, Elizabeth joined Sheffield Hallam University as a senior lecturer in children’s nursing, where she teaches on both pre‐ and post‐registration nursing courses. Her key areas of interest are children’s critical care nursing, children’s respiratory nursing, evidence‐based practice, and nurse education.

Helen Monks RGN, RSCN, BSc (Hons), RNT, MSc, FHEASenior Lecturer in Children’s Nursing, Sheffield Hallam University, Sheffield, UKHelen started her career in 1986 and gained her RGN qualification at Bradford and a few years later her RSCN qualification at Manchester. She has experience in nursing children and families within the fields of general surgery, plastic surgery and general medicine. During her practice, Helen has sought to empower both families and nurses via education and partnership approaches. Her career progressed to sister and ward manager where she became more interested in education, took up project nurse roles within Bradford’s nursing development unit, and subsequently worked as a practice development nurse. She then commenced lecturing at the University of Bradford in 1997 where she was part of a team to set up the first children’s nursing course there. Helen moved to Sheffield Hallam University in 2008 bringing her experience of course leadership and curriculum development. She has a research interest in the subjective nature of assessment of student nurses in the practice environment, and is currently undertaking a PhD in this subject.

Alison Mosenthal RGN, RSCN, Dip N (London), Dip Nursing Education, MScSenior Lecturer in Children’s Nursing, School of Health and Social Work, University of Hertfordshire, UKAlison began her nursing career at St Thomas’ Hospital London before undertaking her RSCN training at Great Ormond Street in 1979. After qualifying, she worked in the respiratory intensive care unit and then moved into nurse education in the School of Nursing at Great Ormond Street in 1983.After a career break raising her family Alison returned to clinical nursing working as a clinical nurse specialist in paediatric immunology nursing at St George’s Healthcare NHS Trust in 1996. She remains in clinical practice part‐time and in 2010 returned to teaching in higher education at the University of Hertfordshire, where she currently works part‐time as a senior lecturer in paediatric nursing.

Michele O’Grady RGN, RSCN, MSc, PGCertSenior Lecturer, University of Hertfordshire, UKMichele trained in Dublin and worked as a qualified nurse until she went to Sudan with the voluntary agency GOAL, running a primary care programme. She moved to the UK in 1987 where she worked in several hospitals before moving to Oxford where she became the HIV liaison officer for 5 years. Michele returned to the NHS where she was a senior sister and an emergency nurse practitioner in the children’s emergency department at Watford General Hospital. Michele joined the child nursing lecturing team at the University of Hertfordshire in 2015.Michele has had an interest in sexual health and health promotion since her time in Sudan.

Julia Petty RGN, RSCN, MSc, PGCert, MASenior Lecturer in Children’s Nursing, School of Health and Social Work, University of Hertfordshire, UKJulia began her children’s nursing career at Great Ormond Street Hospital. After a period in clinical practice and education, she moved into higher education and worked as a senior lecturer at City University, London for 12 years before commencing her current post in 2013. Her key interests are neonatal health, outcome of early care and most recently, the development of digital learning resources in neonatal/children’s nursing care. Julia has a considerable publication portfolio and is on the editorial board of the Journal of Neonatal Nursing. She is a newborn life support instructor for the UK Resuscitation Council, executive member and chair of the Neonatal Nurses Association Special Interest Education and Research Group. Her recent research interest involves exploring the narratives and experiences of parents in neonatal care for the development and evaluation of a digital storytelling resource for children’s nurses.

Cathy Poole RGN, RSCN, MSc, Public Sector Management PGDip Ed ENB 147Training and Education Manager, Fresenius Medical Care, Birmingham, UKCathy started her career as a nursery nurse not knowing that her career would lead on to nursing and the private health education sector. With over 35 years’ experience in a variety of health and education positions, Cathy has published several times and presented at local, national and international conferences. Her main area of interest is renal nursing. Her current post allows her to combine her two passions of renal nursing and education. Not wanting to abandon her third passion of children’s nursing, Cathy maintains her clinical practice as a bank staff nurse at Acorns Children’s Hospice.

Sheila Roberts RGN, RSCN, RNT, BA (Hons), MASenior Lecturer in Children’s Nursing, University of Hertfordshire, UKSheila began her nursing career in 1979 at the Queen Elizabeth School of Nursing in Birmingham, working primarily at Birmingham Children’s Hospital. She moved to general paediatrics at Kidderminster, Ipswich and finally to Bedford Hospital where she became ward sister. Sheila moved into nurse education in 2006. Her areas of interest include nursing practice and teaching nursing skills to students, along with an interest in the cardiac and respiratory systems and child development. More recently, Sheila has been taking part in projects involving children as service users within the pre‐registration nursing curriculum.

Tanya Urquhart‐Kelly RGN, RSCN, MSc, NMP, Dip H OncChild Field Nursing Lecturer, Sheffield Hallam University, Sheffield, UKTanya graduated from Sheffield School of Nursing and Midwifery in March 1993 as an RGN/RSCN and has worked in a variety of roles within the field of paediatric oncology/haematology nursing since qualifying. Most recently she worked as a Macmillan clinical nurse specialist in paediatric and teenage and young adult late effects at Sheffield Children’s NHS Foundation Trust. She has recently taken a substantive child field nursing lecturer post at Sheffield Hallam University. Her key areas of interest are teenagers and young adults with cancer, transition and survivorship; particularly the endocrine care for survivors of childhood cancer. She was awarded a distinction and the faculty prize for her contemporary Master’s degree in the care of teenagers and young adults with cancer from Coventry University, and holds certificates in endocrine nursing and research studies. She is recognised in the international arena of late effects following cancer in childhood, and has presented at numerous international symposiums. She was the previous chair of the CAN UK Nurses group (Cancer Aftercare Nurses group) and an active member of the CCLG (Children’s Cancer & Leukaemia Group) Late Effects group.

Peter S. Vickers Cert Ed, DipCD, RGN, RSCN, BA, PhDFollowing several years as a schoolteacher, Peter began his nursing career in 1980 at York District Hospital, before specialising in paediatric nursing at The Hospital for Sick Children, Great Ormond Street. His nursing specialties were paediatric immunology and immunodeficiency, infectious diseases, and genetics. In 1999, he was awarded his PhD following his study of children with severe combined immunodeficiency who had survived bone marrow transplants in the UK and Germany (which was later published as a book). Following award of his PhD, Peter entered nurse education as a senior lecturer in paediatric nursing at the University of Hertfordshire, where he first began writing, and has gone on to publish widely in nursing textbooks and journals. He has also undertaken research into adult hospice care and written computer programmes on immunology for distance learning. He has also presented at conferences in many European countries, as well as North Africa. In 2012, Peter was elected President of INGID (the international organisation for immunology nurses), and in 2014, upon stepping down as INGID President, he was presented with a life‐time achievement award in immunology nursing by INGID.

Preface

In order to provide safe and effective care to children and families, it is essential that those who are providing that care are able to understand the pathophysiology that underpins the child’s condition.

The overall aim of this text is to help make the sometimes complex subject of pathophysiology accessible and exciting, and to enable the reader to apply their knowledge to various contexts of care. The body has an extraordinary ability to respond to disease in a variety of physiological and psychological ways. It is able to compensate for the changes that come about as a result of the disease process – the pathophysiological processes. The text can assist you in advancing your critical thinking; it fosters innovation and creativity in relation to the health and wellbeing of those to whom you have the privilege to offer care.

The text adopts a user‐friendly approach – inviting you to delve deeper, discover new facts, and to engender curiosity. There are many illustrations, which are used in such a way as to explain and assist in understanding and appreciating the complex disease patterns that are being discussed. Applying a fundamental approach will provide you with a crucial understanding of applied pathophysiology, while emphasising that at all times the child and the family must be at the centre of all that is done.

A series of activities are provided, which are intended to help you learn in an engaged way and support you as you apply your learning in the various care settings, wherever these may be. This text offers an up‐to‐date overview of pathophysiology and the key issues associated with care provision.

The need to constantly consider the wider context of care provision, supplementing a nursing focus and recognising the broadening of the professional base, is emphasised. In providing care that is contemporary, safe and effective, an integrated, multidisciplinary approach is a key requirement. Healthcare students are important members of any multidisciplinary care team. It should also be acknowledged that contemporary care provision is delivered in ever‐changing environments to a range of children, families, communities and circumstances.

Most chapters provide case studies that are related to chapter content. The chapters will stimulate reflection and further thought. In all case studies the names used are pseudonyms, in order to maintain confidentiality. Nurses owe a duty of confidentiality to all those who are receiving care (NMC, 2015). The majority of case studies have been extended further and include data concerning the patient’s vital signs and blood analysis. This can help you to relate important concepts to care, offering you further insight into the patient’s condition and therefore their needs. A selection of case studies include a Paediatric Early Warning Score (PEWS).

In England, nearly every hospital uses a different PEWS chart and calculates PEWS in varied ways. The PEWS charts included in this text are only there to demonstrate how they may be used. It must be remembered that infants (0–11months), preschool children (1–4 years), school‐age children (5–12 years), and teenagers (13–18 years) will all require a PEWS chart that is specific to their age. You should familiarise yourself with the PEWS chart used in the organisation where you work.

Where appropriate, significant information related to the chapter appears in boxed format to focus the reader, for example, red flags and medicines management. This can help you when you are offering care to children and families who may be vulnerable and scared.

A feature found in most chapters is the investigations box. One investigation has been chosen related to chapter content. This contains details about the test or investigation encouraging the reader to think about the pre‐, peri‐ and post‐procedural care that the child and family may require.

All chapters begin and end with questions, which are there to test your pre‐ and post‐knowledge. A range of learning resources are included at the end of the chapters, such as word searches, ‘fill in the blanks’, crosswords, and label the diagram activities. A list of further resources that you may wish to access with the intention of increasing and advancing your learning is provided at the end of each chapter. Each chapter also has a glossary of terms.

Pathophysiology is concerned with the cellular and organ changes that take place when disease is present, and the effects that these changes have on a person’s ability to function. When something happens that interrupts the normal physiological functioning of the body, for example, disease, it becomes a pathophysiological issue. It must always be acknowledged that normal health is not and cannot be exactly the same in any two children, and thus when the term ‘normal’ is used, it must be treated with caution. An understanding of pathophysiology ‘normal’ and ‘abnormal’ can assist the healthcare student in helping the child and family in a kind, sensitive, compassionate, caring, safe and holistic way.

This text is a foundation text providing support to the reader as you grow personally and professionally in relation to the provision of care. The text is primarily intended for nursing students who come into contact with children who may have a number of physically related healthcare problems, in the hospital and community setting. Illness and disease are discussed explicitly, highlighting the fact that children do become ill and they experience disease.

It is not imagined that you will read the text from cover to cover – we would encourage you to dip in and out of it. However, it may assist in your learning if you first read Chapter 1 (The cell and body tissue) and Chapter 2 (Genetics), as these provide a good starting point – they set the scene. The aim is to entice and encourage you, to whet your appetite, and inspire you to read further, and in so doing we hope to instill a sense of curiosity in you.

Reference

Nursing & Midwifery Council (NMC) (2015).

The Code. Professional standards of practice and behaviour for nurses and midwives

. Available at:

https://www.nmc.org.uk/globalassets/sitedocuments/nmc‐publications/nmc‐code.pdf

(last accessed April 2018).

Liz Gormley‐Fleming, HertfordshireIan Peate, Gibraltar

Acknowledgements

I would like to thank my family, Kieran my husband and my girls, Kate and Eilis. Thank you all for being you and providing me with real‐life case studies.

Liz Gormley‐Fleming

I would like to thank my partner Jussi Lahtinen for his support and encouragement and Mrs Frances Cohen who, without hesitation, provides me with her help and inspiration.

Ian Peate

About the companion website

This book is accompanied by a companion website:

www.wileyfundamentalseries.com/childpathophysiology

The website includes:

Multiple‐choice questions

Further resources

Word‐search exercises

Glossaries

Crosswords

‘Fill in the blanks’ exercises

True or false questions

How to use your textbook

Features contained within your textbook

Learning outcome boxes give a summary of the topics covered in a chapter.

Keyword boxes give a summary of the keywords covered in a chapter.

Every chapter contains ‘Test your prior knowledge’ questions.

Case studies

give an up‐close, in‐depth, and detailed examination of a subject.

Red flags

provide quick summaries of alert signs and symptoms.

Your textbook is full of illustrations and tables.

Chapter 1The cell and body tissue

Peter S. Vickers

Aim

The aim of this chapter is to introduce the reader to the various cells and tissues of the body in order to develop their insight and understanding.

Learning outcomes

On completion of this chapter, the reader will be able to:

Outline the structure and function of a human cell.

Name and describe the functions of the organelles.

Explain the cellular transport system.

Describe the structures and functions of the various tissues of the body, namely: epithelial, connective, muscle and nervous tissues.

Keywords

cytoplasm

plasma membrane

organelles

nucleus

passive transport

active transport

epithelial tissue

muscle tissue

connective tissue

mitochondria

Test your prior knowledge

What are the characteristics of human cells?

Describe the ways in which substances can pass through the cell membrane.

What is the role of the cell nucleus?

What are the four main roles of connective tissue?

How many different types of muscle tissue are there?

Where is epithelial tissue to be found within the body?

CELLS

Introduction

What is a cell? Put simply, a cell is a building block for the formation of all life and, particularly in this case, for the formation and development of the human body. There are many different types of cells and they play different roles in both the structure and functioning of the body. For example, certain cells come together to form skin (a tissue), which acts as a cover and protector for our internal organs (tissues). Other cells combine to form bone (tissue) and hence our skeleton. Then there are other different cells which combine to make up the brain and neurological tissue (nerves). Outside the cells that form our structure are the cells that help to keep us functioning, for example, the cardiac cells, which combine to make the heart (tissue), which in turn keeps blood (cells and a tissue) flowing around our body carrying nutrients to all our cells and tissues and removing waste products from them. Some cells are involved in protecting us from infectious organisms, whilst others form muscles (tissues) which allow us to work and move. So, it can be seen that cells are the basic building blocks of our bodies – indeed, our very ‘being’.

All these different types of cells are actually produced from just two cells – ovum and sperm – which fuse together at the moment of conception. Within those two cells are all the plans and schemata for producing the number and diversity of cells that make a human body – truly a miracle! Once they fuse together at conception, they begin to multiply and divide into the different types of cells. This manufacture and diversification of cells is dictated by the genes carried in all of our cells (see Chapter 2, Genetics).

This chapter will give a brief overview of the structure of cells and their roles within the body. In addition, it will discuss some of the problems that can occur and how these can affect the working and health of the body, commencing with the common characteristics of cells (Fig. 1.1).

Figure 1.1 Examples of different types of cells in the body.

Source: Tortora & Derrickson 2009, in: Peate & Gormley‐Fleming 2015. Reproduced with permission of Wiley.

Characteristics of cells

Cells are active – carrying out specific functions.

Cells require nutrition to survive and function. They use a system known as endocytosis in order to catch and consume nutrients – they surround and absorb organisms such as bacteria and then absorb their nutrients. These nutrients are used for the storage and release of energy, as well as for growth and for repairing any damage to themselves.

Cells can reproduce themselves by means of asexual reproduction in which they first develop double the number of organelles (the organs of a cell) and then divide, with the same number and types of organelle and structure present in each half. This is known as simple fission.

Cells excrete waste products.

Cells react to things that irritate or stimulate them – for example, in response to threats from chemicals and viruses.

The structure of the cell

There are four main compartments of the cell:

cell membrane

cytoplasm

nucleus

nucleoplasm.

Within these compartments are many organelles (or small organs). These organelles perform numerous roles to keep cells alive and functioning.

The cell membrane

As can be seen in Fig. 1.2, the various structures of the cell are contained within a cell membrane (also known as the plasma membrane). This cell membrane is a semi‐permeable biological membrane separating the interior of the cell from the outside environment, and protecting the cell from its surrounding environment. It is semi‐permeable because it allows only certain substances to pass through it for the benefit of the cell itself. For example, it is selectively permeable to certain ions and molecules (Alberts et al., 2014). Inside the cells are the cytoplasm and the organelles, which include, for example, the lysosomes, mitochondria, and the nucleus of the cell.

Figure 1.2 Structure of the cell.

Source: Tortora & Derrickson 2009, in: Peate & Gormley‐Fleming 2015. Reproduced with permission of Wiley.

The cell membrane, which can vary in thickness from 7.5 nm (nanometres) to 10 nm (Vickers, 2009) is made up of a self‐sealing double layer (bilayer) of phospholipid molecules with protein molecules interspersed amongst them (Fig. 1.3). A phospholipid molecule consists of a polar ‘head’, which is hydrophilic (mixes with water), and a tail that is made up of non‐polar fatty acids, which are hydrophobic (repel water). In the bilayer of the cell membrane, all the heads of each phospholipid molecule are situated on the outer and inner surfaces of the cell facing outwards, whilst the tails point into the cell membrane; it is this central part of the cell membrane consisting of hydrophobic tails that makes the cell impermeable to water‐soluble molecules (Marieb, 2014). In addition to the phospholipid molecules, the cell membrane contains a variety of molecules, mainly proteins and lipids, and these are involved in many different cellular functions, such as communication and transport. The proteins inserted within the cell membrane are known as plasma member proteins (PMPs), which can be either integral or peripheral. Integral PMPs are embedded amongst the phospholipid tails whilst others completely penetrate the cell membrane. Some of these integral PMPs form channels for the transportation of materials into and out of the cell, others bind to carbohydrates and form receptor sites (e.g., attaching bacteria to the cell so they can be destroyed). Other examples of integral PMPs include those that transfer potassium ions in and out of cells, receptors for insulin, and types of neurotransmitters (Vickers, 2015). On the other hand, peripheral PMPs bind loosely to the membrane surface, and so can be easily separated from it. The reversible attachment of proteins to cell membranes has been shown to regulate cell signalling, as well as acting as enzymes to catalyse cellular reactions through a variety of mechanisms (Cafiso, 2005).

Figure 1.3 Cell membrane.

Source: Tortora & Derrickson 2009, in: Peate & Gormley‐Fleming 2015. Reproduced with permission of Wiley.

Functions of the cell membrane

Briefly, the two major physiological functions of the cell membrane are endocytosis and exocytosis. These are both concerned with the transport of fluids and other essential particulates and waste matter into and out of the cell.

Endocytosis

is the passing of fluids and small particles into the cell. There are three types of endocytosis, namely:

Phagocytosis

– the ingestion of large particulates, such as microbial cells

Pinocytosis

– the ingestion of small particulates and fluids

Receptor‐mediated

– involving large particulates, such as protein. It is also highly selective as to which particulates are taken up.

Endocytosis involves part of the cell membrane being drawn into the cell interior, along with particulates or fluid, in order to facilitate their ingestion. This part of the membrane is then ‘pinched off’ to form a vesicle within the cell. At the same time, the cell membrane reseals itself. Once inside the cell, the fate of this vesicle depends upon the type of endocytosis involved and the material that is contained within the cell membrane surrounding it. In some cases, the vesicle may ultimately fuse with a lysosome (an organelle), following which the ingested material can be processed. Endocytosis is also the means by which many simple organisms – such as amoeba – obtain their nutrients.

The cell membrane and transport

Selective permeability, as mentioned in the previous section, is very important to the process of transporting materials into and out of the cell, allowing certain materials to pass through the membrane, whilst preventing others that could harm the cell. This process depends upon the hydrophobicity of some of its molecules (as mentioned earlier). Because the phospholipid molecule tails are composed of hydrophobic fatty acid chains, it is difficult for hydrophilic (water‐soluble) molecules to penetrate the membrane. Hence it forms an effective barrier for these types of molecules, which can only be penetrated by means of specific transport systems that control what can enter or leave the cell. For example, the membrane controls the process of metabolism by restricting the flow of glucose and other water‐soluble metabolites into and out of cells – as well as between subcellular compartments. In addition, the cell stores energy in the form of transmembrane ion gradients by allowing high concentrations of particular ions to accumulate on one side of the membrane. Ions can pass through the membrane from inside the cell to the outside – or vice versa – so that there are more supplies of these ions just outside the cell or inside it. The membrane controls the speed/rate at which these ions pass through the membrane. The controlled release of such ions on the gradients can be used for:

extracting nutrients from the fluids around the cells

passing electrical messages (nerve excitability)

controlling the volume of the cell.

Cell membrane permeability

There are four factors involved in the degree of permeability of a cell membrane, namely:

The size of molecules – large molecules cannot pass through the integral membrane proteins, whilst small molecules (e.g., water, amino acids) can.

Solubility in lipids (fats) – substances that easily dissolve in lipids (e.g., oxygen, carbon dioxide, steroid hormones) can pass through the membrane more easily than non‐lipid soluble substances can.