Liver Disease in Adolescence (In Clinical Practice)

Preface
Contents
Part I: The Impact of Liver Disease on Adolescent Development
	Chapter 1: Impact of Chronic Liver Disease on Nutrition, Growth and Puberty in Adolescence
		Introduction
		Nutrition in Adolescence (Table 1.1)
			Chronic Liver Disease
			Liver Transplant
			Non-alcoholic Fatty Liver Disease (NAFLD)
		Growth in Adolescence (Table 1.2)
			Chronic Liver Disease
			Liver Transplant
			Non-alcoholic Fatty Liver Disease (NAFLD)
		Puberty (Table 1.3)
			Chronic Liver Disease
			Liver Transplant
			NAFLD
		References
	Chapter 2: Neurodevelopment During Adolescence
		Anatomy and Brain Functions
		Neuronal Maturation
			Neural Structure
			Brain Chemistry
		Brain Volumetric Changes
			Timing and Time Course
			Sex Differentiation
		Sex-steroid Hormones and Brain Development
		Functional Changes
			Social Cognitive Emotions
			Reward Processing
			Emotional Faces
		Key Findings
		Conclusion
		References
	Chapter 3: Impact of Liver Disease on Cognitive and Psychosocial Development
		Introduction
		Why Cognition?
		Summary of Available Literature
		Factors Relating to Poorer Cognition
		Monitoring Cognition in Our Population
		Suggestions for Clinicians
		What to do When Concerns Are Identified?
		Summary
		References
Part II: Specific Liver Diseases
	Chapter 4: Biliary Atresia in Transition Hepatology
		Introduction
		Medical Complications in Young People with BA
			Cholangitis
			Portal Hypertension
			Cholestasis and Pruritus
			Synthetic Function
			Hepatocellular Carcinoma
			Extrahepatic Medical Complications
				Pulmonary
				Small Intestinal Bacterial Overgrowth
				Others
		Growth Characteristics
		Puberty
		Pregnancy
		Education and Employment
		Quality of Life
		Lifestyle Advice
		Mental Health
		LT Listing/Timing
		Conclusion
		References
	Chapter 5: ALGS and Transition: The Prognosis into Adulthood
		Introduction
		Hepatic Prognosis
			Cholestasis and Liver Dysfunction
			Prognosis and Spontaneous Remission of Liver Disease
			Pruritus
			Partial External Biliary Diversion
			Liver Malignancies
			Liver Transplantation in ALGS
		CVS Involvement in ALGS
		Xanthomas and Hyperlipidaemia
		Renal Disease and Prognosis
			Structural Defects and Renal Dysplasia
			Glomerulopathy and Tubulopathy
			Renal Vascular Disease
			Renal Outcomes After Transition
		Immunology
		Neurological, Vascular and Eye Abnormalities
			Bleeding Diathesis and CNS Vascular Malformations
			Prognosis of Eye Abnormalities
			Cranial Synostosis
		Hypothyroidism
		Growth, Puberty and Fertility
		Bone Health
		Quality of Life, Education and Employment
		References
	Chapter 6: Alpha-1 Antitrypsin Deficiency in the Transition Period
		What the Paediatric Hepatologist Needs to Know About the Natural History?
		What the Adult Hepatologist Needs to Know When Taking Over a Young Person with A1ATD?
		Therapeutic Approaches
			Blocking Polymerisation
			Chemical Chaperoning
			Augmentation of Autophagy
			iRNA Interference Techniques
			Transfer of Genetic Material
		References
	Chapter 7: Inherited Metabolic Diseases
		Introduction
		Overview of IMDs
			Disorders of Carbohydrate Metabolism
				Galactosaemia
				Glycogen Storage Disorders (GSDs)
				Hereditary Fructose Intolerance
				Transaldolase Deficiency
		Disorders of Protein Metabolism
			Urea Cycle Disorders (UCD)
			Citrin Deficiency
			Lysinuric Protein Intolerance (LPI)
			Hereditary Tyrosinaemia Type 1 (HT1)
			Organic Acidaemias
		Disorders of Fatty Acid Oxidation
		Mitochondrial Disorders
		Lysosomal Storage Disorders (LSDs)
		Peroxisomal Disorders
		Acute Management and Novel Therapies
		Outcomes for Adolescents with IMD
			Long Term Outcomes in IMDs
			Pregnancy, Fertility and Labour Management
		Conclusions
		References
	Chapter 8: Autoimmune Liver Disease
		Pathogenesis
		Types of AIH
		Diagnosis
		Treatment
		Difficult-to-Treat Patients
		Links with IBD
		De Novo AIH
		Medication Adherence
		What Adult Hepatologist Needs to Know
		Future
		References
	Chapter 9: Wilson Disease
		Introduction
		Pathophysiology
			Genetics
			Copper Metabolism and Homeostasis in Hepatocytes
		Clinical Presentations
			Hepatic Manifestations
			Neurological Manifestation
		Diagnosis
			Serum Copper and Exchangeable Copper (CuEXC) Determination
			Urinary Copper and Penicillamine Challenge Test
			Liver Copper
			Serum Caeruloplasmin
			Mutational Analysis
		Treatment
			Pharmacological Treatment
			Liver Transplantation
			Future Therapeutic Strategies: Liver Cell Transplantation and Gene Therapy
		Follow-Up and Prognosis
		Prevention and Screening
		Specific Issues in Young Adults Compared to Children
			Fertility and Pregnancy
		References
	Chapter 10: Nonalcoholic Fatty Liver Disease
		Top Tips for the Clinician
		Introduction
		Prevalence and Risk Factors
		Differences Between Adult and Paediatric NAFLD
			Noninvasive Biomarkers in NAFLD
				Serum Biomarkers
				Non-invasive Biomarkers and Imaging
				Non-hypothesis Driven Search for Novel Biomarkers Using New Technologies
		Natural History and Treatment
			Psychosocial Aspects
		Conclusions
		References
	Chapter 11: Portal Hypertension
		Introduction
		PHT Complications
		Quality of Life
		Endoscopic Therapy
		Primary Prophylaxis
		Pharmacological Therapy
		Shunt Surgery
		Acute Variceal Bleeding
		Pharmacotherapy for Acute Variceal Bleeding
		Endoscopic Treatment Pathway
		Secondary Prophylaxis
		Sports and Travelling with Portal Hypertension
		Fertility and Pregnancy
		Non-invasive Prediction Tools of Portal Hypertension
			Serum Biomarkers
			Variceal Prediction Scores
			Imaging Techniques
			FibroScan
		Summary
		References
	Chapter 12: Cystic Fibrosis and Liver Disease
		Spectrum of Liver Involvement in Adolescents and Young Adults
			Hepatobiliary Involvement
			Obliterative Portal Venopathy (OPV)
			Steatosis
		Outcome Related to Liver Disease
		Specific Features for Paediatricians Looking After Adolescents with CF
			Assessment of Liver Disease
			Management of CF Adolescents with Liver Disease
		Specific Features for Hepatologists Looking After Adolescents with CF
			Management of PH
			Timing for LT
			Novel Therapies
			Fertility and Risks of Pregnancy
			Transition to Adult Care
		References
	Chapter 13: Sickle Cell Disease
		Introduction
		Treatment
		Hydroxy-urea
		Blood Transfusion
		Haemopoietic Stem Cell Transplantation (HSCT)
		Liver Function Tests in Sickle Cell Disease
		Liver Disease in SCD
		Gallstones (Cholelithiasis)
		Cholangiopathy
		Acute Hepatic Sequestration and Intrahepatic Sickling (Acute Hepatic Crisis)
		Autoimmune Liver Disease (AILD) in Sickle Cell Disease
		Liver Transplantation in SCD
		New and Emerging Treatments
		Fertility and Pregnancy in SCD
		What Adult Hepatologist Needs to Know?
		References
	Chapter 14: Vascular Anomalies in the Liver
		Introduction
		Congenital Porto-Systemic Shunts
			Epidemiology
			Anatomy
			Consequences
			Clinical Features
				Adolescent Demography
				Associations
				Liver Tumours
				Pulmonary Hypertension
				Hepatopulmonary Syndrome
				Hepatic Encephalopathy
				Endocrine Abnormalities
			Management
		Arterioportal Fistulae
		Arterio-Venous Malformation
			Telangiectasia
		Hepatic Arterial Aneurysms, Pseudoaneurysms & Telangectasia
			Aneurysm & Pseudoaneurysm
		Hepatic Venous Anomalies
			Budd-Chiari Syndrome
			Veno-Occlusive Disease (Sinusoidal Obstruction Syndrome)
		Peliosis Hepatis
		Portal Vein Occlusion and Thrombosis
		Benign Vascular Tumours
			Focal Nodular Hyperplasia
		Malignant Vascular Tumours
			Hepatic Epithelioid Haemangioendothelioma
			Hepatic Angiosarcoma (HAS)
		References
Part III: Liver Transplantation in Adolescence
	Chapter 15: Patient and Graft Outcome in Paediatric Liver Transplantation
		Worldwide Practises and Long Term Outcome
		Graft Outcome Including Protocol Liver Biopsies at 5 and 10 Years Post LT
		Patient Outcome Including Medical Complications: Infections, Malignancies, Renal Failure, Nutrition, Social Aspects
		Infections
		Malignancy
		Renal Failure
		Nutrition
		Social Aspects
		Long Term Immunosuppression and Tolerance
		References
	Chapter 16: Liver Transplantation in Early Adulthood
		Indications
		Liver Transplant Waitlist
		Liver Transplant Outcomes and Re-transplantation
		Conclusion
		References
Part IV: Challenges for Adolescents and Young Adults with Liver Disease
	Chapter 17: Adherence to Treatment and Substance Misuse
		Scope of the Nonadherence Problem
		Measurement of Adherence
			Indirect Measures of Adherence
			Direct Measures of Adherence
		Adolescent Vulnerabilities and Interventions
			Mental Health
			Role of Caregivers
			Transition Readiness
			Transition Coordinators
			Programmatic Interventions
			Technology-based Interventions
			Limitations
			Recommendations
		Substance Use
			Alcohol Use
			Marijuana Use
			Opioid Use
			Other Substances
			Summary and Implications
		References
	Chapter 18: Mental Health
		Introduction
		Prevalence
		Factors Associated with Poorer Mental Health
			Sleep and Fatigue
			Illness Beliefs
			Cognition and Employment
		Screening and Management of Mental Health Problems (Difference in Access to Mental Health Services)
			Standardised Screening Questionnaires
		The HEEADDSS Tool
		Assessing Suicidality
		References
	Chapter 19: Health Related Quality of Life, Education and Employment
		Introduction
		Health-Related Quality of Life in the Adolescent with Liver Disease with Native Liver
		Health-Related Quality of Life in the Adolescent Liver Transplant Recipient
		Education and Employment
		Summary
		References
	Chapter 20: Fertility and Pregnancy
		Introduction
		Chronic Liver Disease and Cirrhosis
			Fertility
			Pregnancy
				Monitoring
				Complications
				Outcomes
		Liver Transplantation
			Fertility
				Pre-pregnancy Counselling
				Immunosuppression and Pregnancy
				Monitoring
				Outcomes
					Maternal Outcomes
					Fetal Outcomes
					Mode of Delivery
		Contraception/Breast Feeding
			Chronic Liver Disease
			Viral Hepatitis
			Post Transplantation
		Specific Considerations Dependent on Aetiology of Liver Disease
			Hepatitis B Virus
			Hepatitis C Virus (HCV)
			Autoimmune Hepatitis
				Fetal Outcomes
				Maternal Outcomes
			Primary Biliary Cholangitis
			Primary Sclerosing Cholangitis (PSC)
			Wilson Disease
			Biliary Atresia
			Alagille Syndrome
			Key Points for Adult Hepatologist
		References
	Chapter 21: Social Media and Technology
		Introduction
		Social Media in Healthcare
		Young People and Social Media
		Engaging Young People in Their Health Using Social Media
			Healthcare Delivery
			Health Education
		Social Media and Liver Disease
		Negative Impacts of Social Media
			Self-Esteem
			Cyber-Bullying
		Use of Technology in Young People’s Healthcare
			Transition, and Self-Management
			Adherence
		Mobile Technology and Liver Disease
		Summary
		References
	Chapter 22: The Social Aspects of the Adolescent in Transition
		The Patient Experience
		The Perspective of a Young Adult in Transition
		The Perspective of a Young Peer Mentor
		References
Index