Cystic Fibrosis An Issue Of Pediatric Clinics Of North America

Cystic Fibrosis (CF) is a multisystem disease whose symptoms and signs involve the gastrointestinal tract (thus affecting nutritional status), endocrine system, reproductive system and the respiratory tract (nose, sinuses and lungs). Despite new treatments, the median survival for patients with CF is less than optimal, primarily due to complications of obstructive lung disease. Currently there are approximately 60,000-80,000 people worldwide with CF. The clinical manifestations of CF are caused by dysfunction of CFTR (cystic fibrosis transmembrane conductance regulator), a multifunctional cyclic-AMP regulated ion channel protein. Over time, there has been dramatic improvement in CF patient life expectancy, in large part related to earlier diagnosis (newborn screening), better understanding of molecular genetics and underlying pathophysiology, the integrated and highly specialized Cystic Fibrosis Foundation Accredited Care Centers, and development of a wide range of new treatments and therapies, some of which target the basic CFTR defect. This edition of Pediatric Clinics of North America will offer general pediatricians and family physicians, as well as subspecialists, an update of the extraordinary progress made in the understanding and treatment of Cystic Fibrosis.

In collaboration with Consulting Editor, Dr. Bonita Stanton, Guest Editor Dr. Nelson L. Turcios has created a comprehensive issue on the multiple pulmonary symptoms associated with pediatric disease. In this issue of Pediatric Clinics of North America, expert authors have contributed clinical review articles that are current and timely. Articles are devoted to the following topics: Pulmonary Manifestations of Genetic Disorders in Children; Pulmonary Manifestations of Congenital Heart Disease in Children; Pulmonary Manifestations of Gastrointestinal, Pancreatic & Liver Diseases in Children; Pulmonary Manifestations of Hematologic and Oncologic Diseases in Children; Pulmonary Manifestations of Endocrine and Metabolic Diseases in Children; Pulmonary Manifestations of Immunosuppressive Diseases Other than HIV in Children; Pulmonary Manifestations of Human Immunodeficiency Virus (HIV) Infection in Children; Pulmonary Manifestations of Rheumatoid Diseases in Children; Pulmonary Manifestations of Systemic Vasculitis in Children; Pulmonary Manifestations of Neuromuscular Diseases in Children; Pulmonary Manifestations of Renal Disorders in Children; Pulmonary Manifestations of Parasitic Diseases in Children; Pulmonary Manifestations of Skin Disorders in Children; Adverse Environmental Exposure and Childhood Respiratory Health in Children; Functional Respiratory Disorders in Children; and Healthcare Inequalities in Pediatric Respiratory Diseases in Children. Pediatricians will come away with the clinical information they need to treat pulmonary conditions and manage outcomes in children with pediatric diseases.

Cystic Fibrosis (CF) is a multisystem disease whose symptoms and signs involve the gastrointestinal tract (thus affecting nutritional status), endocrine system, reproductive system and the respiratory tract (nose, sinuses and lungs). Despite new treatments, the median survival for patients with CF is less than optimal, primarily due to complications of obstructive lung disease. Currently there are approximately 60,000-80,000 people worldwide with CF. The clinical manifestations of CF are caused by dysfunction of CFTR (cystic fibrosis transmembrane conductance regulator), a multifunctional cyclic-AMP regulated ion channel protein. Over time, there has been dramatic improvement in CF patient life expectancy, in large part related to earlier diagnosis (newborn screening), better understanding of molecular genetics and underlying pathophysiology, the integrated and highly specialized Cystic Fibrosis Foundation Accredited Care Centers, and development of a wide range of new treatments and therapies, some of which target the basic CFTR defect. This edition of Pediatric Clinics of North America will offer general pediatricians and family physicians, as well as subspecialists, an update of the extraordinary progress made in the understanding and treatment of Cystic Fibrosis.

Pediatricians care for children and families from all walks of life. Some are children known from neighborhoods. Others are children from distant lands. Pediatric focus does not stop with the physical care of children but extends to include their mental and social-emotional health and concern about their families. Pediatricians care about how children are doing at home, at school, and in their communities. In this era, children and their families are impacted by social and political changes in their homes (social media and screen time), in their communities (refugee populations and children requiring palliative supports at school), in their health care networks (EMR in every tertiary pediatric center), and in the larger world (multiple military deployments of fathers and mothers). This issue explores the impact of contemporary public health challenges for pediatric care, promising models for caring for chronically ill children, and state of the art therapies for complex childhood conditions.

Dr. Jon Koff has assembled and expert team of authors of the topic of Cystic Fibrosis. Articles include: Epidemiology and Pathobiology, Genetics and genetic medicine in Cystic Fibrosis, Innate and Adaptive Immunity in Cystic Fibrosis, Microbiome in Cystic Fibrosis, Diagnostic Testing in Cystic Fibrosis, Treating Pseudomonas in Cystic Fibrosis, Diagnosis of Adult Patients with Cystic Fibrosis, Transition from Pediatrics to Adult Care, Lung Transplant in Cystic Fibrosis, and more!

Dr. Kliegman and Dr. Bordini have written a primer on Undiagnosed and Rare Diseases in Children. Leading experts have presented the current knowledge in the following areas: How Doctor’s Think: Common Diagnostic Errors in Clinical Judgment; Team-Based Approach to Undiagnosed and Rare Diseases; Ending a Diagnostic Odyssey: Family Education Counselling and Their Response to Eventual Diagnosis; Eczema and Urticaria as Manifestations of Undiagnosed and Rare Diseases; Usual and Unusual Manifestations of Familial Hemophagocytic and Langerhans Cell Histiocytosis Syndromes; When Autistic Behavior Suggests a Disease Other than Classic Autism; Non-classic Inflammatory Bowel Disease in Young Infants; IPEX and Other Disorders; Usual and Unusual Presentation of Mitochondrial Disorders; When to Suspect Auto-inflammatory/Recurrent Fever Syndromes; Primary and Secondary Causes of Autonomic Dysfunction; Usual and Unusual Manifestations of Systemic and CNS Vasculitis; Fever of Unknown Origin; Differentiating Familial Neuropathies from Guillain-Barre Syndrome; and Munchausen by Proxy: A Factitious Undiagnosed Disease. Readers will come away with cutting-edge information to use immediately in their clinical management of patients.

The Guest Editors have tapped top key opinion leaders to provide current reviews on the clinical diagnosis and treatment of gastrointestinal disease in children. They have focused on topics related to GERD; Celiac Disease and Gluten Sensitivity; IBD; Motility Disorders; GI Bleeding and Management; Pancreatic Disorders; Hepatitis B and C; Liver Transplant; Abdominal Pain; Complications from Obesity; and Neonatal Cholestasis.These articles will provide a state-of-the-art overview of diagnosis and treatment of GI diseases that are impactful for the general pediatrician.

In this issue, guest editors bring their considerable expertise to this important topic. Provides in-depth reviews on the latest updates in the field, providing actionable insights for clinical practice. Presents the latest information on this timely, focused topic under the leadership of experienced editors in the field. Authors synthesize and distill the latest research and practice guidelines to create these timely topic-based reviews.