Chest and spinal disease in patients with progressive neuromuscular disease.

The chest and spine deformity in neuromuscular disease (NMDz) can impact respiratory mechanics and pulmonary function by changing the orientation of the muscles and joints of the respiratory system and placing them in a mechanically unfavorable position. This increases mechanical load on weak respiratory muscles and eventually can cause respiratory failure. Therefore, chest and spine deformity in NMDz will both lead to increased respiratory "load" and decreased respiratory muscle "pump", an exceptionally bad combination.

In-office Maximal Voluntary Ventilation Testing Demonstrates Pulmonary Improvement Following Posterior Spinal Fusion for Adolescent Idiopathic Scoliosis.

Objective: Pulmonary function can be impaired in patients with adolescent idiopathic scoliosis (AIS). Maximal voluntary ventilation (MVV) has been shown to be more strongly correlated with major coronal curve, and a more easily obtained measurement of pulmonary function, than forced vital capacity (FVC). We evaluated changes in pulmonary function using these 2 measures in patients with AIS in relation to changes in major coronal curves over time.

Elevated periodic limb movement index is associated with hypoxemia in children with early onset scoliosis.

Purpose: Patients with early onset scoliosis are at high risk of sleep disordered breathing, sleep disruption, and adverse consequences of poor sleep. In this study, we aim to assess the prevalence of periodic limb movements of sleep in a cohort of children with early onset scoliosis and identify factors that correlate with the presence of periodic limb movements.

Methods: This is a retrospective chart review of 40 patients with EOS (ages 1-17 years) who underwent a PSG from 2003 through 2019.

Paediatric syndromic scoliosis: proceedings of the half-day course at the 57th annual meeting of the Scoliosis Research Society.

There are some syndromes that present with unique manifestations pertaining to the spinal column. A good working understanding of these common syndromes is useful for the spinal deformity surgeons and related healthcare providers. This review attempts to encompass these unique features and discuss them in three broad groups: hypermobility syndromes, muscle pathology-related syndromes, and syndromes related to poor bone quality.

Obstructive Apnea and Hypopnea Length in Normal Children and Adolescents.

(1) Background: Breathing is an essential function that requires both metabolic (or au-tomatic) and voluntary (behavioral) control during wakefulness but during sleep depends on metabolic control via peripheral and central chemoreceptors. Breathing during sleep disordered breathing also depends on the maturity of the neural centers and the strength of the respiratory muscles. We do not know if the response to apnea varies with age.

A Comparison of Maximal Voluntary Ventilation and Forced Vital Capacity in Adolescent Idiopathic Scoliosis Patients.

Study Design: Prospective, single center.

Objective: To compare maximal voluntary ventilation (MVV) and forced vital capacity (FVC) testing in the outpatient scoliosis clinic to determine their relative feasibility and sensitivity in assessing pulmonary function in the AIS population.

Summary Of Background Data: Evidence of compromised pulmonary function in patients with severe AIS is well established.

Complications in using the vertical expandable prosthetic titanium rib (VEPTR) in children.

Purpose: This report describes complications using the vertical expandable prosthetic titanium rib (VEPTR) for thoracic insufficiency syndrome (TIS) at a single center.

Methods: This is a prospective cohort evaluating 65 patients with rib-rib and rib-spine VEPTR devices for TIS placed between 10/2001 and 11/2014, for children with spinal or chest wall deformity. Patients were classified using the early onset scoliosis classification system (C-EOS).

Obstructive lung disease in children with idiopathic scoliosis.

Objective: To measure the prevalence of obstructive lung disease (OLD) among patients undergoing preoperative pulmonary assessment for idiopathic scoliosis.

Study Design: This was a retrospective, descriptive review from clinical data in a tertiary care pediatric hospital in the US. Patients (n = 176) with idiopathic scoliosis with Cobb angles of ≥ 40 degrees who performed acceptable and repeatable preoperative pulmonary function testing were included.

Obstructive lung disease is common in children with syndromic and congenital scoliosis: a preliminary study.

Background: It is well known that restrictive lung disease (RLD) is associated with scoliosis. This study identifies that obstructive lung disease (OLD) is associated with syndromic scoliosis and congenital scoliosis.

Methods: We searched a local database for patients with scoliosis who underwent a pulmonary function testing (PFT) from 2004 to 2009.

Longitudinal nasopharyngeal carriage and antibiotic resistance of respiratory bacteria in indigenous Australian and Alaska native children with bronchiectasis.

Background: Indigenous children in Australia and Alaska have very high rates of chronic suppurative lung disease (CSLD)/bronchiectasis. Antibiotics, including frequent or long-term azithromycin in Australia and short-term beta-lactam therapy in both countries, are often prescribed to treat these patients. In the Bronchiectasis Observational Study we examined over several years the nasopharyngeal carriage and antibiotic resistance of respiratory bacteria in these two PCV7-vaccinated populations.

Correlating Haller Index and cardiopulmonary disease in pectus excavatum.

Background: The Haller Index (HI) has become standard for determining the severity of pectus excavatum. We compared patterns of cardiopulmonary dysfunction and their relationship with HI in patients with pectus excavatum.

Methods: We performed cardiopulmonary exercise testing and chest computed tomography scans on 90 patients with pectus excavatum deformities at a regional pediatric hospital.

Strategic plan for pediatric respiratory diseases research: an NHLBI working group report.

The Division of Lung Diseases of the National Heart, Lung, and Blood Institute (NHLBI) recently held a workshop to identify gaps in our understanding and treatment of childhood lung diseases and to define strategies to enhance translational research in this field. Leading experts with diverse experience in both laboratory and patient-oriented research reviewed selected areas of pediatric lung diseases, including perinatal programming and epigenetic influences; mechanisms of lung injury, repair, and regeneration; pulmonary vascular disease; sleep and control of breathing; and the application of novel translational methods to enhance personalized medicine. This report summarizes the proceedings of this workshop and provides recommendations for emphasis on targeted areas for future investigation.

Strategic plan for pediatric respiratory diseases research: an NHLBI working group report.

The Division of Lung Diseases of the National Heart, Lung and Blood Institute (NHLBI) recently held a workshop to identify gaps in our understanding and treatment of childhood lung diseases and to define strategies to enhance translational research in this field. Leading experts with diverse experience in both laboratory and patient-oriented research reviewed selected areas of pediatric lung diseases, including perinatal programming and epigenetic influences; mechanisms of lung injury, repair, and regeneration; pulmonary vascular disease (PVD); sleep and control of breathing; and the application of novel translational methods to enhance personalized medicine. This report summarizes the proceedings of this workshop and provides recommendations for emphasis on targeted areas for future investigation.

Interdisciplinary leadership training outcomes of Maternal and Child Health-funded pediatric pulmonary centers.

Objectives: To describe career paths, leadership accomplishments, and extent of incorporation of Maternal and Child Health Bureau (MCHB) values into professional activities of all previous long-term trainees.

Method: In 1998 the Pediatric Pulmonary Centers (PPCs) completed a Leadership Training Outcomes Survey of all previous long-term trainees. The survey included 1) characteristics, 2) career paths, 3) current professional activities, 4) leadership roles and activities, and 5) career incorporation of MCHB values.

Sequelae of severe respiratory syncytial virus infection in infancy and early childhood among Alaska Native children.

Objective: In 1993-1996, we conducted a nested case-control study to determine risk factors for hospitalization with respiratory syncytial virus (RSV) infection among Alaska Native infants and young children. In the current study, we returned to former RSV case-patients and their control subjects during 1999-2001 to determine whether children who are hospitalized with RSV at <2 years of age are more likely to develop chronic respiratory conditions.

Methods: For each former RSV case-patient and control subject from remote villages in southwest Alaska, we reviewed medical records, interviewed parents, performed physical examinations and spirometry, collected sera, and analyzed chest radiographs.