Abstract:
:Sudden unexplained deaths have been reported in 13% [corrected] of Familial Dysautonomia (FD) subjects. To characterize cardiorespiratory dysregulation in children with FD that might contribute to potential sudden death, respiratory inductance plethysmography (chest/abdomen), ECG, hemoglobin saturation, and pulse waveform (VivoMetrics, Inc.) were recorded in the home during daytime wakefulness and overnight sleep in 25 children with IKBKAP mutation-confirmed FD and 25 age-, and gender-matched controls. Breath-to-breath and beat-to-beat characterization of breathing, hemoglobin saturation, and heart rate was conducted. Children with FD had more frequent, prolonged, and severe episodes of hypoxemia than matched controls, awake and asleep. Though a small percent of the study time revealed bradycardia and apnea, the hypoxemia was the most prevalent pattern in FD and rarely occurred with related bradycardia. Though infrequent with desaturation or bradycardia, apnea was more prevalent in FD subjects than controls, and more apparent during sleep than wakefulness. Children with FD have cardiorespiratory dysregulation during wakefulness and sleep, likely representing alveolar hypoventilation. We hypothesize that the related repeated hypoxemia (and presumed related hypercarbia) may render individuals with FD more vulnerable to sudden death.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Weese-Mayer DE,Kenny AS,Bennett HL,Ramirez JM,Leurgans SEdoi
10.1002/ppul.20764subject
Has Abstractpub_date
2008-03-01 00:00:00pages
251-60issue
3eissn
8755-6863issn
1099-0496journal_volume
43pub_type
杂志文章abstract::Malacoplakia is an unusual inflammatory condition with distinctive histologic features. Involvement of the lung is quite uncommon and is rarely described in paediatrics. We report on a case of pulmonary malacoplakia in a teenage girl. ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10386
更新日期:2003-11-01 00:00:00
abstract::Multiple breath inert gas washout (MBW) is gaining popularity for measurements of resting lung volume and ventilation inhomogeneity. Test reproducibility is an important determinant of the clinical applicability of diagnostic tests. The between-test reproducibility of variables derived from MBW tests in newborn infant...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21113
更新日期:2010-01-01 00:00:00
abstract::Randomized trials of ribavirin therapy have used clinical scores to assess illness severity. Little information on agreement for these findings between observers has been published. We decided to determine interobserver agreement for (1) a history for apnea or respiratory failure; (2) assessment of cyanosis, respirato...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1002/(SICI)1099-0496(199607)22:1<23::AID-PPUL4>
更新日期:1996-07-01 00:00:00
abstract::A continuous subcutaneous infusion of terbutaline (CSIT) was used to treat 8 children with chronic severe asthma who continued to experience frequent symptoms, despite treatment with regular oral prednisolone. Five patients experienced a symptomatic improvement from CSIT, leading to a reduction in regular medication. ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10081
更新日期:2002-05-01 00:00:00
abstract::Traumatic injuries to the pediatric trachea are uncommon events that require prompt diagnosis and management. When they do occur, tracheal injuries often arise in the setting of high impact trauma and are accompanied by major injuries to other organ systems. In this report, we present the diagnosis and conservative ma...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23507
更新日期:2017-01-01 00:00:00
abstract::To determine whether doses of ipratropium bromide (IB) greater than those usually administered by aerosol (75-250 micrograms) give a greater degree of protection from exercise-induced asthma (EIA) in children, 12 patients with chronic asthma, ages 7-13 yr, were challenged with methacholine and exercise after inhalatio...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950060205
更新日期:1989-01-01 00:00:00
abstract:BACKGROUND:Magnetic Resonance Imaging (MRI) techniques to image the larynx have evolved rapidly into a promising and safe imaging modality, without need for sedation or ionizing radiation. MRI is therefore of great interest to image pediatric laryngeal diseases. Our aim was to review MRI developments relevant for the p...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24250
更新日期:2019-04-01 00:00:00
abstract::Better growth and nutritional status is strongly associated with better pulmonary function and survival in children with CF. Behavioral intervention is an efficacious treatment approach for improving calorie intake and weight gain in children with CF; and recently has been shown to facilitate maintenance of daily ener...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1002/ppul.21147
更新日期:2010-01-01 00:00:00
abstract:OBJECTIVE:To evaluate safety and efficacy of oral posaconazole and terbinafine for Lomentospora prolificans and Scedosporium apiospermum in children with cystic fibrosis. METHODS:Retrospective case review. RESULTS:There were five children (four girls), median age 15.0 years; three had S. apiospermum and two had L. pr...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24789
更新日期:2020-08-01 00:00:00
abstract:OBJECTIVE:Telomere length is associated with poorer lung health in older adults, possibly from cumulative risk factor exposure, but data are lacking in pediatric and population-based cohorts. We examined associations of telomere length with lung function in children and mid-life adults. METHODS:Data were drawn from a ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24489
更新日期:2019-12-01 00:00:00
abstract::Plasminogen deficiency is characterized by fibrin-rich pseudomembrane formation on all mucosal surfaces, particularly the conjunctiva. Respiratory system involvement is common; fibrin often obstructs the upper or lower respiratory tract, causing death. Although many treatments have been applied, no definitive treatmen...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24563
更新日期:2020-01-01 00:00:00
abstract:BACKGROUND:Population-based studies investigating health-related quality of life (HRQoL) among asthmatic adolescents are rare. Further, among subjects with asthma, HRQoL may be affected by asthma control and severity. AIM:To investigate HRQoL in relation to asthma control and asthma severity among adolescents. METHOD...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23723
更新日期:2017-07-01 00:00:00
abstract::Intrauterine inflammation generates inflammatory mediators that damage the developing bronchoalveolar epithelium, resulting in neonatal lung injury. Lung fluid transport disorders are the main reasons for the development of pulmonary edema, an important pathology of lung injury. Previous studies suggested that epithel...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24242
更新日期:2019-03-01 00:00:00
abstract::This paper assesses the effectiveness of aerosolized tobramycin (TOBI) on cystic fibrosis (CF) lung disease, using a radiologic tool. The published tool, the age-based severity curve (ABS), is derived from Brasfield scoring of chest X-rays (CXR). This study evaluates both the usefulness of the ABS as an assessment too...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20032
更新日期:2004-07-01 00:00:00
abstract::There is a subpopulation of asthmatic patients with a history of life-threatening asthma (LTA) who have a reduced perception of respiratory loads. The aim of this study was to determine if these patients have a reduced perception of both intrinsic and extrinsic loads. Children with asthma were classified into life-thr...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10199
更新日期:2002-12-01 00:00:00
abstract:OBJECTIVE:To determine whether non-invasive positive pressure ventilation (NIPPV) delivered via nasal prongs can alleviate the need for tracheal intubation and invasive ventilation in infants admitted to the pediatric intensive care unit (PICU) with impending respiratory failure, and to find predictive factors for succ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22561
更新日期:2012-10-01 00:00:00
abstract:BACKGROUND:Chronic colonization with Pseudomonas (P.) aeruginosa worsens the prognosis of cystic fibrosis (CF) patients. This study aims to analyze the functional properties of neutrophils in CF patients with P. aeruginosa colonization. METHODS:Patients with CF (n = 16) were grouped by positivity of P. aeruginosa in s...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25294
更新日期:2021-01-29 00:00:00
abstract::Dedicator of cytokinesis 8 (DOCK8) deficiency is an autosomal recessive type of combined immunodeficiency with elevated IgE. In this report, we describe a Japanese girl of non-consanguineous family suffering from acute eosinophilic pneumonia (AEP) as a presenting feature of DOCK8 deficiency. Although AEP was self-limi...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22814
更新日期:2014-03-01 00:00:00
abstract::We present a 9-month-old infant with persistent cough refractory to conventional asthma therapy. An extensive evaluation eventually revealed a Chiari I malformation with syringohydromyelia. His cough resolved one month after surgical decompression, suggesting that brainstem compression from the Chiari malformation dir...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20912
更新日期:2008-10-01 00:00:00
abstract::In children, pulmonary sequelae contribute to early and late morbidity after bone marrow transplantation (BMT). Between 1975-1999, we performed 152 BMTs in 138 pediatric patients with malignant and nonmalignant diseases. Allogenic bone marrow was used from 99 HLA identical siblings and from 23 other related or unrelat...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/1099-0496(200011)30:5<393::aid-ppul5>3.0.c
更新日期:2000-11-01 00:00:00
abstract::We herein report a case of antiphospholipid syndrome (APS) primarily presenting with asthma-like symptoms that had been misdiagnosed as severe asthma. Patients presenting with severe asthma symptoms along with systemic thrombosis should be systematically evaluated for APS. ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23079
更新日期:2015-02-01 00:00:00
abstract::In this article, we highlight cystic fibrosis (CF) research published in Pediatric Pulmonology during 2014, as well as related articles published in other journals. ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.23309
更新日期:2015-11-01 00:00:00
abstract::A 16-year-old female with cystic fibrosis (CF) presented with an acute respiratory exacerbation during which black flecks were observed in the spontaneously expectorated sputum. The production of this pigmented sputum was subsequently attributed to Exophiala dermatitidis hyphae. Treatment with antibiotics, corticoster...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21187
更新日期:2010-05-01 00:00:00
abstract:OBJECTIVE:To describe the clinical course and treatment of an infant with acute pulmonary toxicity following intralesional administration of bleomycin for a lymphovenous malformation. DESIGN:Case report. SETTING:A tertiary care University-affiliated hospital. PATIENT, INTERVENTION, AND RESULTS:An 8-month-old girl de...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21139
更新日期:2010-02-01 00:00:00
abstract::Allergic bronchopulmonary aspergillosis (ABPA) is a complication commonly encountered in patients with CF that produces significant respiratory morbidity. Chronic airway colonization with Aspergillus induces strong inflammatory responses with high IgE levels. Current guidelines for therapy include prolonged courses of...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20804
更新日期:2008-06-01 00:00:00
abstract::Respiration rate is an important indicator of a person's health, and thus it is monitored when performing clinical evaluations. There are different approaches for respiration monitoring, but generally they can be classed as contact or noncontact. For contact methods, the sensing device (or part of the instrument conta...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.21416
更新日期:2011-06-01 00:00:00
abstract::A new method that permits the measurement of adult-type maximal expiratory flow-volume curves and fractional lung volumes in sedated infants was recently described. The purpose of this study was to define the normal range for these new measures of pulmonary function in infants and young children. Measurements of force...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/1099-0496(200009)30:3<215::aid-ppul6>3.0.c
更新日期:2000-09-01 00:00:00
abstract::The goal of this research was to begin the process of evaluating acceptability of infection control (IC) recommendations to CF patients and their families, determine whether compliance with IC guidelines differs from compliance with traditional CF medical treatment with respect to the variables predictive of complianc...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20781
更新日期:2008-05-01 00:00:00
abstract::While many factors that contribute to the occurrence of lower respiratory tract infections (LRTI) in Canadian indigenous children have been described, the role of aspiration during swallowing has not been explored in these children. Because of an increase in referrals of indigenous children from our catchment area (Ma...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21489
更新日期:2011-12-01 00:00:00
abstract:INTRODUCTION:Interpregnancy interval (IPI) is defined as the period between a live birth and the conception of a subsequent fetus. Both short (IPI < 6 months) and long IPI (IPI > 60 months) have been shown to increase the risk for adverse perinatal outcomes, some of which, are known risk factors for obstructive sleep a...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25240
更新日期:2020-12-31 00:00:00