Adults with Childhood Illnesses: Considerations for Practice (Health, Medicine and Human Development)

Just a few decades ago, children born with significant congenital anomalies or genetic and metabolic diseases perished at an early age and very few survived into their teens and even less into adulthood. Congenital heart disease, major errors in metabolism, cancer, cystic fibrosis and many other major diseases were fatal. Because of that many physicians in adult primary care did not have the opportunity to see patients with these problems and thus unable to learn how to care for them. In this book, we have recruited highly qualified and experienced physicians to compile what is to the best of our knowledge, the first book dealing entirely with the issue of children's diseases in adults. Our goal is to provide a resource for all health care providers in order to help with caring for such adult patients. We believe that it will be valuable to all health care providers who provide care to adults with children's diseases. To our knowledge, there is no such resource available for practitioners which will make this book desirable. Contents......Page 6 Preface......Page 14 Abbreviations......Page 16 Authors index......Page 20 INTRODUCTION......Page 24 1 Adults with childhood illnesses......Page 26 CHILDHOOD INTO ADULTHOOD......Page 28 2.1 Introduction......Page 30 2.2 Diagnostic criteria, epidemiology, comorbidities and associated features......Page 31 2.3 Assessment of ADHD in children and adolescents......Page 33 2.4 Treatment of ADHD in children and adolescents......Page 34 2.5 ADHD in adulthood: epidemiology, comorbidities and associated features......Page 37 2.6 Assessment of ADHD in adults......Page 38 2.7 Treatment of ADHD in adults......Page 39 2.8 Conclusions......Page 41 3.1 Introduction......Page 46 3.2 CF care paradigm......Page 47 3.3 Transition from pediatric to adult care......Page 48 3.4 Challenges of treatment burden and adherence to maintain lung function......Page 49 3.5 Diseases of aging in CF: diabetes, osteoporosis and malignancy......Page 51 3.7 Achieving a “normal” life......Page 55 3.8 Conclusions......Page 58 4.2 Pathogenesis and pathophysiology of asthma......Page 60 4.2.2 Host factors (genes and sex)......Page 62 4.3.1 Natural history of asthma symptoms......Page 63 4.3.2 Natural history of lung function and bronchial hyperreactivity......Page 65 4.4.1 Childhood predictors of asthma......Page 66 4.5 Clinical differences of asthma in childhood versus adulthood......Page 67 4.6 Predicting adult asthma......Page 68 4.8 Impact of environmental factors......Page 69 4.10 Impact of therapy upon the natural course of asthma......Page 70 4.11 Conclusions......Page 71 5.2 Presentation in adult years......Page 76 5.3 Complications of right-to-left shunts......Page 77 5.4 Patients who have had surgery for a right-to-left shunt......Page 79 5.5 Other cardiac complications in adults survivors of cyanotic heart defects in childhood......Page 80 5.6 Conclusion......Page 81 6.2.1 Aortic valve stenosis and bicuspid aortic valve......Page 82 6.2.2 Subvalvular aortic stenosis......Page 88 6.2.3 Supravalvular aortic stenosis......Page 89 6.3 Aortic valve regurgitation......Page 90 6.4 Coarctation of the aorta......Page 91 6.5 Mitral valve stenosis......Page 93 6.6.1 Mitral valve prolapse......Page 94 6.6.2 Postoperative mitral regurgitation......Page 95 6.8 Tricuspid valve regurgitation......Page 96 6.8.1 Ebstein anomaly of the tricuspid valve......Page 97 6.9 Pulmonary stenosis......Page 98 6.10 Pulmonary regurgitation......Page 100 7.2 Atrial septal defects......Page 104 7.2.1 Secundum ASDs......Page 105 7.2.2 Sinus venosus ASD with partial anomalous pulmonary venous return......Page 110 7.2.3 Endocardial cushion (atrioventricular canal) defects......Page 113 7.2.4 Primum ASD (partial atrioventricular canal defect)......Page 114 7.2.5 Complete atrioventricular canal defect......Page 117 7.2.6 VSDs......Page 120 7.2.7 PDA......Page 125 7.2.8 Pulmonary hypertension and Eisenmenger syndrome......Page 128 7.2.9 Future considerations......Page 129 8.2 TS......Page 132 8.2.2 Pediatric issues......Page 134 8.3 CAH......Page 138 8.3.1 Diagnostic considerations......Page 139 8.3.2 CAH therapeutic management......Page 140 8.4 DM......Page 141 8.4.1 Incidence and prevalence of T1DM......Page 142 8.4.3 Diagnostic considerations......Page 143 8.4.4 Management considerations......Page 144 8.4.5 The team approach......Page 145 8.4.6 Main components of DM management......Page 146 8.5 Conclusions......Page 152 9.1 Introduction......Page 158 9.3.1 PKU: the beginning......Page 161 9.3.3 PKU in the 1960s: newborn screening begins and improvements in dietary therapy......Page 162 9.3.6 PKU in 2007: cofactor therapy with BH4 in classic PKU and hyperphenylalaninemia......Page 163 9.4.2 Metabolic diseases are invariably hereditary......Page 164 9.6 Pediatric patients are surviving and thriving......Page 165 9.7.1 Treatment strategies in IEM......Page 166 9.8.1 Dietary challenges in the management of PKU......Page 167 9.9 Unique features of IEM that are helpful in the diagnosis and management of adolescents and adults......Page 169 9.10 Conclusion......Page 170 10.1 Introduction......Page 172 10.2 Spectrum of kidney diseases in children......Page 173 10.4 Mental health aspects of CKD in the child......Page 174 10.5 CKD: special implications related to the transitioning from pediatric to adult care......Page 175 10.7 Physical aspects of grownups with renal disease since childhood......Page 176 10.9 Conclusions......Page 178 11.1 Introduction......Page 182 11.3 Comprehensive long term follow-up care initiatives......Page 183 11.4 Overview of adverse late effects as a function of therapy type......Page 184 11.5 Secondary malignancies......Page 185 11.6 Cardiopulmonary complications......Page 187 11.7 Endocrine complications......Page 189 11.8 Adverse outcomes in the CNS......Page 190 11.9 Reproduction and fertility......Page 192 11.10 Other late effects......Page 193 11.11 Psychosocial support......Page 194 11.13.3 Textbooks and other publications......Page 195 12.2 Velo-cardio facial syndrome: deletion of chromosome 22q11.2......Page 200 12.3 TS......Page 201 12.4 Noonan syndrome......Page 203 12.5 Down syndrome (trisomy 21)......Page 205 12.6 Williams-Beuren Syndrome......Page 207 12.7 Marfan syndrome and Loeys-Dietz syndromes (LDS)......Page 209 12.8 Summary......Page 212 13.2 Upper urinary tract......Page 216 13.2.4 Post-treatment sequelae of chemotherapy......Page 217 13.2.7 Vesicoureteral reflux......Page 218 13.2.8 Cystic kidney disorders......Page 219 13.2.9 Duplicated urinary collecting systems......Page 221 13.2.10 UPJO......Page 222 13.2.12 Rotational/ascent/fusion/formation anomalies of the kidney......Page 223 13.2.13 Megatureter, ectopic ureter and ureterocele......Page 225 13.2.16 Testicular tumors......Page 226 13.2.20 Post-treatment sequelae of radiation therapy......Page 227 13.2.22 Posterior urethral valves......Page 228 13.2.23 Hypospadias......Page 229 13.2.24 Undescended testicles......Page 230 13.2.26 Neuropathic bladder voiding dysfunction and urinary reconstruction......Page 231 13.2.27 Prune belly syndrome, bladder exstrophy-epispadias, urachal cyst/patent urachus and congenital urethral strictures......Page 233 13.3 Abnormalities of sexual differentation......Page 234 14.2 Anemias through decreased production of RBCs......Page 238 14.3 Anemias through abnormal maturation of RBCs: thalassemia syndromes......Page 239 14.5 Sickle cell disease......Page 240 14.5.3 Renal complication......Page 243 14.5.8 Transfusion therapy......Page 244 14.6 Anemias due to membrane defects: hereditary spherocytosis......Page 245 14.8.1 Splenectomy......Page 246 14.8.2 Chronic transfusion therapy......Page 247 15.2 Transition......Page 250 15.3 Conclusions......Page 252 16.1 Introduction......Page 254 16.3 Women with physical disabilities......Page 255 16.3.3 Specific reproductive issues......Page 256 16.3.4 Reproductive health and mental illness......Page 258 16.4 Conclusions......Page 259 17.1 Introduction......Page 262 17.2 Hemophilia A and B......Page 263 17.4 Hemophilia Treatment Centers......Page 265 17.6 Orthopedic complications of hemophilia......Page 266 17.8 Cardiovascular disease......Page 267 17.12 Pregnancy......Page 268 17.13 Conclusion......Page 269 18.1 Introduction......Page 272 18.3 Management of allergies after childhood......Page 273 18.4 Testing methods......Page 274 18.4.1 Skin testing......Page 275 18.4.2 In-vitro testing......Page 276 18.4.3 Positive and negative challenge testing......Page 277 18.5.2 Pharmacotherapy......Page 278 18.5.3 Immunotherapy......Page 281 18.6 Conclusion......Page 282 19.1 Introduction......Page 286 19.2 Trends in the aging in the population of persons with intellectual disability in residential care centers in Israel......Page 287 19.3 Our study of >2, 000 aging persons with intellectual disability......Page 289 19.3.2 General health and utilization/coordination of health services......Page 290 19.4.1 Mild and moderate intellectual disability......Page 291 19.4.2 Health concerns in severe and profound intellectual disability......Page 292 19.4.3 Health concerns in Down syndrome......Page 293 19.5 Intellectual disability and general practice......Page 294 19.6 Conclusions......Page 295 20.2 RS......Page 300 20.2.1 Stages of RS......Page 301 20.2.2 Longitudinal follow-up of adults with RS......Page 302 20.2.3 Recent studies......Page 303 20.2.4 Clinical manifestations of the aging person with RS......Page 304 20.2.5 Adults with RS......Page 306 20.3 Discussion......Page 307 ACKNOWLEDGEMENTS......Page 312 21 About the editors......Page 314 22 About the Kentucky Children’s Hospital at the University of Kentucky, Lexington, Kentucky, USA......Page 316 23.2 The vision......Page 318 23.3 Target areas of interests......Page 319 24.3 Research activities......Page 320 24.5 International collaborations......Page 321 24.6 Targets......Page 322 Index......Page 324 Attention-deficit/hyperactivity disorder : epidemiology, assessment, and treatment / John A. Yozwiak Adults with cystic fibrosis / Michael I. Anstead Childhood asthma into adult years / Don Hayes and Zoran Danov Cyanotic congenital heart defects in adulthood / J. Timothy Bricker and Jorge Alegria Obstructive and regurgitant cardiac lesions in adults who had childhood heart disease / Douglas J. Schneider Adults with left-to-right cardiac shunts and with shunts treated in childhood / Craig Alexander and Lou Bezold Transition of pediatric endocrine patients to adult care / Manmohan K. Kamboj ... [et al.] Adolescents and adults with inborn errors of metabolism / Carolyn Bay, Carol Reid, and Ashley Daub Grownups who had kidney disease in childhood / Aftab S. Chishti and Stefan G. Kiessling Adult survivors of childhood cancer / Sherry Bayliff and Jeffrey A. Moscow Adults with genetic syndromes / Jacqueline A. Noonan and Michelle A. Grenier Adult considerations of pediatric urologic care / Stephen E. Strup, Ali M. Ziada, and William B. Rogers Adults patients with childhood anemias / Vlad Calin Radulescu and Jeffrey A. Moscow Transition from pediatric to adult care : social and family issues / Jacqueline A. Noonan Disabled women and reproductive health care in the United States / Marlene Belew Huff Adults with congenital bleeding disorders / Susan M. Peterson, Vlad C. Radulescu, and Jeffrey A. Moscow Children with allergic disease as adults / Maria C. Veling, Abbas Younes, and Richard C. Haydon Aging with intellectual disability : current health issues / Joav Merrick ... [et al.] Rett syndrome into adulthood / Meir Lotan ... [et al.]. Just a few decades ago, children born with significant congenital anomalies or genetic and metabolic diseases perished at an early age and very few survived into their teens and even less into adulthood. Congenital heart disease, major errors in metabolism, cancer, cystic fibrosis and many other major diseases were fatal. Because of that many physicians in adult primary care did not have the opportunity to see patients with these problems and thus unable to learn how to care for them. This book provides a resource for all health care providers in order to help with caring for such adult patien Just a few decades ago, children born with significant congenital anomalies or genetic and metabolic diseases perished at an early age and very few survived into their teens and even less into adulthood. This book provides a resource for all health care providers in order to help with caring for such adult patients.