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Advances in Pediatrics

Contents

        Associate Editors v
        Contributors vii

        Preface: Emerging from the Pandemic: Taking Up Where We Left Off with Newer Modalities, Assessment, and Treatment xxv

        Carol D. Berkowitz

        Pediatric Telemedicine: Lessons Learned During the Coronavirus Disease 2019 Pandemic and Opportunities for Growth

        Sarah C. Haynes and James P. Marcin
        The accelerated uptake of telemedicine during the coronavirus disease 2019 pandemic has resulted in valuable experience and evidence on the delivery of telemedicine for pediatric patients. The pandemic has also highlighted inequities and opportunities for improvement. This review discusses lessons learned during the pandemic, focusing on provider-to-patient virtual encounters. Recent evidence on education and training, developing and adapting clinical workflows, patient assessment and treatment, and family-centered care is reviewed. Opportunities for future research in pediatric telemedicine are discussed, specifically with regard to engaging pediatric patients, improving and measuring access to care, addressing health equity, and expanding the evidence base.
         Introduction 1
         Models of care within pediatric telemedicine 2
         Lessons learned 3
        Education and training 3
        Developing and adapting clinical workflows 4
        Assessment and treatment 5
        Family-centered care 6
         Opportunities 7
        Improving engagement with pediatric patients 7
        Improving and measuring access to care 7
        Addressing health equity 8
        Expanding the evidence base for pediatric telemedicine 8
         Summary 8
        Disclosure 9

        Helping Our Toddlers, Developing Our Children’s Skills: Innovative Behavioral Management Training for Pediatric Residents

        Heather Agazzi, Sarah Dickinson, and Rebecca M. Plant
        This article describes the implementation of a behavioral management training program into pediatric and combined medicine-pediatric residencies at a large urban academic medical center in southwest Florida. We describe 2 modalities for training residents in effective behavioral modification strategies immediately useable in pediatric practice. Results indicate that residents significantly increased their knowledge of effective, evidence-based strategies and continued to use them 6 to 12 months following completion of the training.
         Introduction 13
         General content 15
        Program development 15
        Research 16
        Modality 1 17
         Modality 2 18
         Summary 20
        Disclosure 20

        Childhood Hearing Health and Early Language Exposure: A Culturally Sensitive Approach

        Parul Bhatia, Julie Rems-Smario, Katrin Jaradeh, and Dylan Chan
        Implementation of the universal newborn hearing screening has changed the landscape for children born deaf or hard of hearing, and with this, pediatricians must be knowledgeable about the screening and referral process, state protocols, and how to support patients and their families in their unique journeys. Early access to language—spoken, signed, both—forms the foundation for successful outcomes for the development of fluent language. For children using spoken language, early access to sound is critical and can often be achieved with the assistance of hearing devices, even in the newborn period. For all language modalities, state-funded Deaf mentor programs allow families to have meaningful exposure to the Deaf community. The authors come from different professions in the health care and education fields, including General Pediatrics, Otolaryngology Head and Neck Surgery, and the state Department of Education, and use different languages, including spoken and American Sign Language.
         Terminology 24
         Background 25
         Early hearing detection and intervention goals 25
         Hearing screening and audiometry 26
         Language opportunities 27
         Deaf community 29
         Non-audiologic evaluation 30
        Genetic 30
        Infectious 31
        Imaging 31
         Intervention for access to sound 33
         Cultural intersectionality 35
         The medical home 36
         Summary 36
        Acknowledgments 37
        Disclosure 37

        A Pediatrician’s Guide to Working with Children on the Autism Spectrum in Coronavirus Disease 2019 and Beyond: Retrospect and Prospect

        Thusa Sabapathy, Megan Goss, Jessica L. Borelli, and Robin Steinberg-Epstein
        The COVID-19 pandemic is an unprecedented event with devastating effects on children and families, highlighting and broadening disparities in the care of children with developmental disabilities, while simultaneously catalyzing innovation. Children are vulnerable to the impacts of COVID-19, resulting in increased stress, anxiety, isolation, and health challenges, further amplified in autistic children and children with other neurodevelopmental disabilities. These children are uniquely vulnerable due to communication impairments, comorbid medical disorders, reduced adaptability, and reliance on therapeutic interventions. Abrupt reduction in services and access to care during the pandemic compromised physical and mental health and led to missed intervention opportunities at critical times. It is important to examine the effects that the pandemic triggered, address deficiencies, and recognize new opportunities to improve systems of care to prepare for unforeseen futures.
         Introduction 41
         Caveats about the state of the science 42
         Medical care 43
         Telehealth 44
         Autism screening and diagnosis 45
         Services 46
         Education 47
         Mental health 49
         Family relationships 50
         Consideration of further exacerbations from disparities and adverse childhood events 50
         The pediatrician’s toolbox for autism care in coronavirus disease 2019 and beyond 51
         Disclosure 53
         Clinics care points 53
        Acknowledgments 53

        Brain Surgery for Medically Intractable Epilepsy

        Vincent Joris, Alexander G. Weil, and Aria Fallah
        This review covers the broad topic of brain surgery in the treatment of pediatric intractable epilepsy. The authors review the latest advancements in the presurgical workup as well as the mandatory tests needed to explore the epilepsy workup in these children. They describe the different types of epilepsy from a surgical standpoint (temporal, extratemporal, multifocal, and hemispheric epilepsies) and various surgical procedures that can be proposed depending on the clinical scenario: lesionectomies, lobectomies, hemispherectomies, neuromodulation, and palliative surgeries. They also describe the key differences of the pediatric patient as compared with the adult patient in such pathologic conditions.
         Introduction 59
         Presurgical workup 60
        Noninvasive investigations 60
        Timing of surgery 63
         Temporal lobe epilepsy 63
         Extratemporal lobe epilepsy 64
        Lesionectomy 64
        Frontal lobectomy 65
        Posterior quadrant resections 65
        Insulectomy 66
        Multiple subpial transection 66
        Neuroplasticity 67
         Multilobar and generalized epilepsy 67
        Palliative surgeries 67
        Neuromodulation procedures 69
         Summary 70
         Clinics care points 71
        Disclosure 71

        Chronic and Recurrent Sinusitis in Children, as Manifestation of Immune Dysfunction and Atopic Background

        Farn-Hsuan Tseng, Marissa Newman, and Charles H. Song
        Rhinosinusitis in children, as in adults, can be classified by duration (acute, recurrent, and chronic) and by cause (viral, bacterial, and inflammatory) and needs to be treated accordingly after careful investigation which include through clinical history, laboratory tests, and, if necessary, nasal endoscopy and imaging studies.
         Introduction 75
         A case history 76
         Clinical manifestation and diagnosis 76
         Epidemiology 78
         Classification 78
         Acute rhinosinusitis (ARS): post-viral & bacterial 79
         Chronic rhinosinusitis (CRS) 79
         Pathophysiology 79
        Microbiome of the sinus cavities 79
         Associated conditions 82
        Allergy (immunoglobulin Eâmediated) 82
        Immune deficiencies 82
        Cystic fibrosis 83
        Gastroesophageal/oropharyngeal reflux 83
        Primary ciliary dyskinesia (Kartagener syndrome) 83
         Lab studies 84
         Complications 84
        Preseptal/periorbital cellulitis 84
        Orbital cellulitis 84
        Intracranial complications 84
        Fungal involvement 84
        Nasal polyps 85
         Differential diagnosis 85
        Allergic rhinitis 85
        Protracted bacterial bronchitis 85
        Nasal foreign body 85
        Pertussis 85
        Migraine/facial pain syndrome 85
         Management 85
        Nasal irrigation 86
        Nasal steroids (inhaler and steroid solution rinse) 86
        Systemic corticosteroid 86
        Antibiotics 87
        Oral and nasal antihistamines, decongestants, and oral antileukotrienes 87
        Allergen and irritant control measures as an adjunctive therapy 87
        Biologics 87
        Vaccination with pneumococcal vaccines 88
        Nonresponders (specific antibody deficiency) 88
        Probiotics 88
         Surgery 88
        Adenoidectomy 88
        Sinus surgery: balloon sinuplasty (balloon ostial dilation) versus functional endoscopic sinus surgery 89
         Summary 89
        Disclosure 89

        Management of Pediatric Obstructive Sleep Apnea After Failed Tonsillectomy and Adenoidectomy

        Abhay Varun Sharma, Tapan Padhya, and Sagarika Nallu
        Pediatric obstructive sleep apnea (OSA) represents a different entity from its adult counterpart and therefore requires a different therapeutic approach. Adenotonsillectomy (AT) is the primary treatment of pediatric OSA, and evidence shows it is very effective. However, there is a growing understanding that residual OSA is common, and next steps for patients who fail primary AT are less certain. This article reviews current methods of evaluating and treating these complex patients.
         Introduction 95
        Reasons for the failure of adenotonsillectomy 96
        Evaluation 96
        Drug-induced sleep endoscopy 97
        Cine magnetic resonance imaging 97
        Medical treatment 98
        Surgical treatment 98
        Craniofacial abnormalities 101
        Obesity 101
         Summary 102
         Clinics care points 103
        Disclosure 103

        The Antiphospholipid Syndrome in the Pediatric Population

        Elizabeth E. Sloan and Deborah McCurdy
        Pediatric antiphospholipid syndrome (APS) is characterized by autoantibodies directed against protein complexes on cellular membranes and leads to a prothrombotic, proinflammatory state. A child with APS may present with venous, arterial, or small vessel thrombosis. Other manifestations of APS include nonthrombotic manifestations, such as hematologic and neurologic symptoms. APS may be a primary condition or related to other autoimmune diseases. If APS-related thrombosis is unrecognized, the child may suffer recurrent thrombotic events after the withdrawal of anticoagulation. Thus, it is important to consider APS as a cause of thrombosis in children. Appropriate testing confirms the diagnosis and directs further care.
         Introduction 107
        Definitions and classification criteria 108
        Epidemiology 109
        Pathogenesis 109
        Spectrum of disease 110
         Manifestations 111
        Thrombosis 111
        Obstetric antiphospholipid syndrome 112
        Catastrophic antiphospholipid syndrome 112
        Microangiopathic disease 113
        Hematologic 113
        Acquired lupus anticoagulant-hypoprothrombinemia syndrome 114
        Skin manifestations 114
        Neurologic 114
        Other manifestations 114
         Laboratory investigations 115
         Imaging and biopsy 116
         Management 116
        Goals of management 116
        Anticoagulation and antiplatelet therapy 116
        Adjunctive therapies 117
        Primary prophylaxis 117
        Birth control and pregnancy considerations 118
         Summary 118
         Clinics care points 118
        Disclosure 119

        Pediatric Chronic Kidney Disease

        Valerie Panzarino, Jake Lesser, and Frank Ayestaran Cassaini
        Chronic kidney disease (CKD) in children has a significant impact on morbidity, mortality, and quality of life. The degree of renal dysfunction should be calculated using pediatric-specific formulas and the degree of CKD staged; this allows for appropriate dosing of medications based on renal function and monitoring for progression and comorbid conditions including metabolic acidosis, bone disease, anemia, cardiovascular complications, malnutrition and electrolyte abnormalities, growth failure, and psychosocial issues. Treatment strategies include treating the underlying disease and using general renal protective measures. Effective management of these complex issues requires a specialized multidisciplinary team approach.
         Clinics care points 129
        Disclosure 130

        Advances in Hemophilia A Management

        Sukjoo Cho, Ashley M. Perry, Anna M. Cheng, Carrie Wang, and Juan Felipe Rico
        Hemophilia A is an inherited insufficiency of Factor VIII (FVIII), one of the critical clotting factors. The gold standard for the management of moderate-to-severe hemophilia A is prophylaxis using regular replacement therapy with clotting factor concentrates. Compared with conventional treatment, extended half-life products reduce the burden of frequent factor replacement injections. Of note, up to 30% of patients with hemophilia A receiving prophylactic factor infusions develop “inhibitors,” neutralizing anti-FVIII autoantibodies. Therapeutic options for patients with hemophilia A and inhibitors include the immune tolerance induction (ie, eradication of inhibitors) and the management of acute bleeds with bypassing agents and/or emicizumab. Emicizumab is a biphasic monoclonal antibody mimicking activated FVIII, approved for patients with hemophilia A with/without inhibitors. Gene therapy is an emerging therapy for hemophilia A, essentially curing patients with hemophilia A or transforming them to a milder phenotype by establishing continuous endogenous expression of FVIII after one-time treatment.
         Introduction 134
        Comprehensive care 134
        Preventive care 136
        Gene therapy 141
         Clinics care points 143
         Summary 143
        Acknowledgments 143
        Disclosure 143

        Genital Herpes Simplex Virus—An Updated Review

        Saida Omarova, Aileen Cannon, Wendy Weiss, Adrienne Bruccoleri, and Joseph Puccio
        The herpes virus was named by the Greek physician Hippocrates who called it herpes because the lesions appeared near each other and were vesicular. Alphaherpesvirinae, Betaherpesvirinae, and Gammaherpesvirinae are subfamilies of the human herpes virus family. The Alphaherpesvirinae subfamily includes the simplex viruses—HSV-1 and HSV-2—and varicellovirus—varicella zoster virus. There are more than 200 members of the Herpesviridae family capable of infecting different species, 8 of which are known to cause disease in humans. The simplex viruses can cause lifelong genital infections, and despite the prevalence of HSV-1 and HSV-2 infections in the United States decreasing in the past 20 years, infections with these viruses continue to contribute to significant clinical and psychological morbidities.
         Introduction 149
         Epidemiology/statistics 150
         Herpes clinical course 151
         Diagnostic testing for herpes simplex virus 152
         Screening for asymptomatic herpes simplex virus infection 154
         Emotional/psychosocial implications and counseling 154
         Prevention 156
         Treatment 156
         Pregnancy and herpes simplex virus 158
         Summary 160
         Clinics care points 160

        Vaccine Hesitancy in Pediatrics

        Andrea Lafnitzegger and Claudia Gaviria-Agudelo
        Vaccine hesitancy is a growing complex and multifaceted phenomenon. It encompasses a wide spectrum of context-dependent attitudes and beliefs. Multiple factors influence parental decision-making including knowledge, sources of information, risk perception, trust, and individual experiences among others. This review focuses on describing the most common reasons that contribute to vaccine hesitancy among parents. Social media and the Internet have been described as major elements that can negatively influence parental decision-making regarding vaccines. The next focus is describing effective interventions that clinical providers can apply. Nonconfrontational and open discussions along with trusting and strong relationships between parents and providers seem to create a solid foundation toward vaccine acceptance. In addition, motivational interviewing is a helpful tool that has proven to be effective during these discussions. Ultimately, an individualized approach tailored to a specific community will likely be most effective in addressing vaccine hesitancy.
         Introduction 163
         Defining vaccine hesitancy 164
        The spectrum of vaccine acceptance 164
         Major reasons for vaccine hesitancy 165
         Parental decision-making 165
        Common reasons for vaccine refusal 165
         Individual factors 168
        Conspiracy theory 168
        Contextual influences/social pressure 169
        Pain perception 169
         Role of social media and the Internet 169
         Interventions: increasing vaccination and decreasing hesitancy and refusal 170
        The power of a multimodal and tailored approach 170
        The power of the medical provider 171
        Communication tools 171
         Summary 173
         Clinics care points 173
         Future directions 174
        Disclosure 174

        Turner Syndrome: An Update

        Margaret Steiner and Paul Saenger
        Turner syndrome is the most common sex chromosome abnormality in women. Infertility and short stature are the most striking findings seen in these patients. Unfortunately, many girls are still being diagnosed too late and therefore early diagnosis and treatment key. Turner syndrome affects many systems of the body; therefore, a comprehensive approach is key for therapeutic intervention.
         Introduction 177
         Incidence 178
         Pathogenesis 178
         Clinical manifestations 180
         Management 187
         Summary 196
         Clinics care points 196
        Disclosure 197

        Clinical Indications for Growth Hormone Therapy

        Melinda Danowitz and Adda Grimberg
        Growth hormone (GH) is an injectable medication originally used to replace the deficiency of the hormone, but has expanded to treating conditions that may reduce growth and adult height even when the body maintains endogenous GH production. In the United States, there are 8 Food and Drug Administration (FDA)-approved indications for pediatric GH therapy: GH deficiency, Prader–Willi Syndrome, small for gestational age (SGA) without catch-up growth, idiopathic short stature, Turner syndrome, SHOX gene haploinsufficiency, Noonan Syndrome, and chronic renal insufficiency. We characterize the growth patterns and effects of GH treatment in each of these indications. We also review patterns of growth that warrant referral to a pediatric endocrinologist, as well as safety updates. This review is intended to guide practitioners on the initial evaluation and management of patients with short stature, and the indications for GH therapy.
         Introduction 204
         Food and Drug Administration-approved indications for growth hormone therapy 204
        Growth hormone deficiency 204
        Prader Willi syndrome 205
        Small for gestational age without catch-up growth 206
        Idiopathic short stature 207
        Turner syndrome 208
        SHOX gene haploinsufficiency 209
        Noonan syndrome 210
        Chronic renal insufficiency 211
         When to refer to a pediatric endocrinologist? 212
         Side effects of growth hormone treatment 213
         Summary 213
         Clinical care points 214
        Disclosure 214

        Update on Pediatric Hyperthyroidism

        Priya Vaidyanathan
        Typical symptoms which should lead to suspicion of hyperthyroidism are unintentional weight loss, tachycardia, and palpitations, heat intolerance, and hyperactivity. It is diagnosed by suppressed thyroid-stimulating hormone (TSH) with elevated thyroid hormone (TH) levels. Graves’ disease (GD) due to antibodies stimulating the TSH receptor is the leading cause, and first-line treatment is with methimazole (MMI). Emerging data suggest MMI treatment, up to 8 years is effective and safe in improving the rate of remission. Radioactive iodine (RAI) and thyroidectomy offer definitive treatment and induce permanent hypothyroidism. Thyroid storm is a life-threatening condition with systemic decompensation and hyperpyrexia. Neonates of mothers with current or past GD are at risk for neonatal hyperthyroidism (NH). Appropriate identification and follow-up of at-risk neonates will reduce complications.
         An update on hyperthyroidism in children 219
         Introduction 219
         Clinical manifestations of hyperthyroidism 220
         Differential diagnosis of hyperthyroidism and its key features 221
         Laboratory diagnosis of hyperthyroidism 221
         Management of Graves’ disease 221
         Management of other forms of hyperthyroidism 223
         Thyroid storm 223
         Predictors and management of neonatal hyperthyroidism 223
         A simplified guide and interpretation are as follows 227
         Summary 227
         Clinics care points 228
        Disclosure 228

        Updates on the Management of Pilonidal Disease

        Amelia T. Collings and Beth Rymeski
        This article reviews the current practices and evidence on the management of pilonidal disease in the pediatric population. Medical management, use of laser epilation, and minimally invasive surgical options are highlighted with a brief review of more invasive surgical options for refractory disease.
         Introduction 231
        Medical management 232
        Surgical treatment 234
         Summary 239
        Acknowledgments 239
        Disclosure 239

        Current Management of Adhesive Small Bowel Obstructions in Children

        Nathan S. Rubalcava and K. Elizabeth Speck
        Adhesive small bowel obstructions are a common cause of morbidity in children who underwent prior abdominal surgery. The concept of partial versus complete bowel obstruction is outdated and lacks precision to be clinically useful. Identifying patients with indications for immediate operative intervention is critical and must be recognized to limit morbidity. Clinical protocols and contrast challenge algorithms have attempted to identify patients that will resolve their bowel obstruction nonoperatively; there has been slow uptake in the pediatric patient population versus adults until recently. Incorporating predictive models and standardized contrast challenge protocols will help reduce interpractitioner variability and improve clinical outcomes.
         Introduction 243
         Initial decision tree 244
        Epidemiology 245
        Pathophysiology 246
         Diagnosis 246
        Clinical presentation 247
        Laboratory investigations 247
        Imaging 247
         Management 248
        Resuscitation and initial management 248
        Operative management 249
        Nonoperative management 250
        Other considerations: Prevention 253
        Other considerations: Recurrence 253
         Summary 254
         Clinics care points 254
        Disclosure 255

        Postoperative Opioid Prescribing, Use, and Disposal in Children

        Marjorie Odegard and Lorraine I. Kelley-Quon
        This article provides an overview of postoperative opioid prescribing, use, and disposal patterns in children and also identifies gaps in knowledge and areas for improvement. We present evidence that there is a need to tailor prescriptions to specific procedures to reduce the number of excess, unused prescription opioid pills in the home. We also explain the need to provide culturally competent care when managing a child’s pain after surgery. Finally, we discuss the need for widespread provider and caregiver education about safe prescription opioid use, storage, and disposal.
         Introduction 259
         Opioid prescribing patterns 260
         Prescription opioid use 263
         Prescription opioid disposal 264
         Summary 266
         Clinical care points 267
        Disclosure 267