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
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