Liver Disease in Adolescence (In Clinical Practice)

Description

This book discusses the clinical management of adolescents with liver disease bridging the gap between pediatric and adult hepatology patient management. Expert views on liver disease in adolescence are discussed and specific differences for this patient subset are highlighted and examined in depth. The reader will gain the specific knowledge required on pathophysiology and treatment, but also about additional aspects such as psychological difficulties and cultural differences including use of social media. Information is presented in a manner relevant to clinical practice, and is fully up-to-date in terms of development and integration of new communication technologies in the society. This book offers a fresh insight to this transitional period which will be a great benefit to hepatologists both in pediatric and adult setting. Edited by two experts in the field and authored by international experts in the area using a multidisciplinary approach, this book is a helpful tool in managing these complex patients and will help minimise their complication rates and improve outcomes by the end of their transition periods. 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