Abstract:
OBJECTIVE:While pulmonary arterial hypertension (PAH) is rare in infants and children, it results in substantial morbidity and mortality. In recent years, prognosis has improved, coinciding with the introduction of new PAH-targeted therapies, although much of their use in children is off-label. Evidence to guide the treatment of children with PAH is less extensive than for adults. The goal of this review is to discuss the treatment recommendations for children with PAH, as well as the evidence supporting the use of prostanoids, endothelin receptor antagonists (ERAs), and phosphodiesterase type 5 inhibitors (PDE5i) in this setting. DATA SOURCES:Nonsystematic PubMed literature search and authors' expertise. STUDY SELECTION:Articles were selected concentrating on the nitric oxide (NO)-soluble guanylate cyclase (sGC)-cyclic guanosine monophosphate (cGMP) pathway in PAH. The methodology of an ongoing study evaluating the sGC stimulator riociguat in children with PAH is also described. RESULTS:Despite recent medical advances, improved therapeutic strategies for pediatric PAH are needed. The efficacy and tolerability of riociguat in adults with PAH have been well trialed. CONCLUSION:The pooling of data across trials, supplemented by registry data, will help to confirm the safety and tolerability of prostanoids, ERAs, and PDE5i in children. Ongoing studies will clarify the place of sGC stimulators in the treatment strategy for pediatric PAH.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Beghetti M,Gorenflo M,Ivy DD,Moledina S,Bonnet Ddoi
10.1002/ppul.24442subject
Has Abstractpub_date
2019-10-01 00:00:00pages
1516-1526issue
10eissn
8755-6863issn
1099-0496journal_volume
54pub_type
杂志文章,评审abstract::The consequences of poor quality and/or inadequate sleep in children and adolescents have become a major public health concern, and one in which pediatric health care professionals have become increasingly involved. In particular, insufficient and/or fragmented sleep resulting from primary sleep disorders such as obst...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.20981
更新日期:2009-05-01 00:00:00
abstract:AIM:Children with respiratory conditions benefit from care provided by pediatric pulmonologists. As these physicians are a small portion of the overall pediatric workforce, it is necessary to understand the practices and career plans of these specialists. METHODS:An internet survey was developed by the American Academ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24253
更新日期:2019-04-01 00:00:00
abstract::A group of 153 children (51 with a history of bronchiolitis and 102 matched controls) were evaluated in a historical cohort study at a mean age of 8 years and again at 13 years to test the primary hypothesis that mild bronchiolitis, far more common than severe (hospitalized) bronchiolitis, predicts wheezing. A seconda...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950060303
更新日期:1989-01-01 00:00:00
abstract::Exercise challenge tests are helpful in the diagnosis and management of childhood asthma, but methodology is complex and time-consuming. The aim of this study was to investigate whether exercise-induced bronchoconstriction (EIB) can be predicted by the results of different surrogate tests in a pediatric population. Ei...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20556
更新日期:2007-03-01 00:00:00
abstract:OBJECTIVE:To determine the interpretative consequences of adopting the Global Lungs 2012 (GLI-2012) spirometric prediction equations in a pediatric hospital population. MATERIAL:Spirometric records from 2,192 white boys and 1,842 white girls, and 412 and 334 African-American boys and girls, respectively, aged 6.0-18.0...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22876
更新日期:2014-02-01 00:00:00
abstract:AIM:The aim of this study was to investigate the status and effects of vitamin D supplement as adjuvant therapy in the management of respiratory distress syndrome (RDS) in preterm infants. PATIENT AND METHODS:Vitamin D was prospectively studied in 196 preterm neonates, 96 of them developed RDS, which further subdivide...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24552
更新日期:2020-01-01 00:00:00
abstract:OBJECTIVE:To validate the bronchiolitis score of Sant Joan de Déu (BROSJOD) and to examine the previously defined scoring cutoff. PATIENTS AND METHODS:Prospective, observational study. BROSJOD scoring was done by two independent physicians (at admission, 24 and 48 hr). Internal consistency of the score was assessed us...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23546
更新日期:2017-04-01 00:00:00
abstract:OBJECTIVE:Obstructive sleep apnea/hypopnea syndrome (OSAHS) is a common sleep problem. The aim of this study is to investigate the association between OSAHS and blood pressure (BP) in snoring children. METHODS:Snoring children were recruited from January 2009 to December 2010. Clinical history was taken accompanied by...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22595
更新日期:2013-03-01 00:00:00
abstract:BACKGROUND:Lung involvement in children with Niemann-Pick disease has rarely been studied systematically. OBJECTIVE:To assess the involvement of the lung and the value of bronchoalveolar lavage in children with Niemann-Pick diseases. DESIGN:Retrospective analysis of patient records. PATIENTS:Thirteen patients, with ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20725
更新日期:2007-12-01 00:00:00
abstract::Lung diseases caused by surfactant protein C (SFTPC) mutations are inherited as autosomal traits with variable penetrance and severity or as sporadic disease caused by a de novo mutation on one allele. Here, we report the case of a child surviving with a homozygous surfactant protein C mutation after aggressive clinic...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22976
更新日期:2014-03-01 00:00:00
abstract::Our purpose was to identify and further characterize physiologic mechanisms relevant to autoresuscitation from hypoxic apnea in infants dying suddenly and unexpectedly. We studied cardiorespiratory recordings of 24 infants (age range, 0.8-21 months) who died suddenly while being monitored at home. These recordings wer...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10287
更新日期:2003-08-01 00:00:00
abstract::Ten children with mild bronchial asthma underwent bronchial challenge with carbachol on three consecutive days; using a double-blind technique, they were given inhaled saline placebo on the first day and placebo or sodium cromoglycate (SCG) on the second and third days 15 min prior to the bronchial provocation test wi...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.1950030309
更新日期:1987-05-01 00:00:00
abstract:RATIONALE:Increasing evidence suggests the forced oscillation technique (FOT) has the capacity to provide non-invasive monitoring and diagnosis of respiratory disease in young children. However, which FOT outcomes provide the most pertinent clinical information is currently unknown. The aim of this study was to determi...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24286
更新日期:2019-06-01 00:00:00
abstract:BACKGROUND:Multiple breath washout (MBW) in infants presents a number of technical challenges. Conventional MBW is based on simultaneous measurement of flow and gas concentrations. These two signals are aligned and combined to derive expired gas volumes from which lung volumes and measures of ventilation inhomogeneity ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23348
更新日期:2016-05-01 00:00:00
abstract::During the final prenatal period of fetal lung development in humans, important maturational processes occur, including the production of surfactant necessary to decrease surface tension at the air-liquid interface of the alveoli. During early gestation, the glucocorticoid receptor is expressed in the fetal lung, and ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.1092
更新日期:2001-07-01 00:00:00
abstract::The long-term efficacy and safety of aminoglycoside aerosol therapy for Pseudomonas aeruginosa colonization/infection in cystic fibrosis has not been fully investigated. In the present study, 14 patients with cystic fibrosis, ages 8-19 years (mean: 13.3 years), received tobramycin aerosol therapy for a mean duration o...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950060207
更新日期:1989-01-01 00:00:00
abstract::Previous research has demonstrated a significant reciprocal relationship between psychosocial factors and asthma morbidity in children. The National Cooperative Inner-City Asthma Study investigated both asthma-specific and non-specific psychosocial variables, including asthma knowledge beliefs and management behavior,...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
doi:10.1002/(sici)1099-0496(199710)24:4<263::aid-ppul5
更新日期:1997-10-01 00:00:00
abstract::Our aim was to determine whether antenatal corticosteroids improve perinatal adaptation of the pulmonary circulation in lambs with lung hypoplasia (LH). LH was induced in 12 ovine fetuses between 105 and 140 days gestation (term approximately 147 days); in 6 of these the ewe was given a single dose of betamethasone (1...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20453
更新日期:2006-09-01 00:00:00
abstract:INTRODUCTION:The prognosis of postinfectious bronchiolitis obliterans (PIBO) has many implications, ranging between reduced quality of life and life-threatening complications. We evaluated the prognostic factors for PIBO using the baseline clinical characteristics of patients and built a prediction model for determinin...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25220
更新日期:2020-12-11 00:00:00
abstract:OBJECTIVES:Sildenafil, tezosentan, and prostacyclin reduce pulmonary vascular pressures in pulmonary hypertension, but have potential to vasodilate the systemic circulation. Nebulized vasodilators allow targeted drug delivery, high local drug concentrations, less systemic hypotension, and better matching of the lung's ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20431
更新日期:2006-07-01 00:00:00
abstract::Assessment of dynamic inspiratory function may provide valuable information about the degree and progression of pulmonary involvement in patients with Duchenne muscular dystrophy (DMD). The aims of this study were to characterize inspiratory function and to assess the efficacy of idebenone on this pulmonary function o...
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.23547
更新日期:2017-04-01 00:00:00
abstract::Our objective was to compare the effectiveness of maximum forced expiratory flow measured at functional residual capacity (V'maxFRC) and the ratio of flow at 75% of the forced expiratory volume to peak forced expiratory flow (FEF(75)/FEF(peak)) for detecting bronchodilator-related changes in wheezy infants. In 55 infa...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.10325
更新日期:2003-09-01 00:00:00
abstract::Mucus plugging constitutes a nutrient-rich nidus for a bacterial infection that has long been recognized as a potent stimulus for neutrophilic airway inflammation driving progressive lung damage in people with cystic fibrosis (CF). However, mucus plugging and neutrophilic inflammation are already present in many infan...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.24462
更新日期:2019-11-01 00:00:00
abstract::A study was designed to determine the sensitivity and specificity of a cold air bronchial provocation test. A total of 18 children with asthma (mean age 12 years) and 18 normal children (mean age 14 years) were studied. The cold air challenge consisted of a 4 min period of isocapnic hyperventilation of subfreezing air...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950030410
更新日期:1987-07-01 00:00:00
abstract::Neutrophil elastase is present at high levels in airway fluid of patients with cystic fibrosis (CF), and is responsible for considerable inflammatory damage. Human monocyte/neutrophil elastase inhibitor (MNEI), a 42-kDa serpin protein, is an effective inhibitor of neutrophil elastase, cathepsin G, and proteinase-3, re...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20167
更新日期:2005-02-01 00:00:00
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:OBJECTIVES:The present pilot study was performed to evaluate the HPA axis and ANS activity by measuring salivary cortisol and α-amylase diurnal trajectory and production, respectively, in mild or moderate-to-severe (MS) OSA-affected, but otherwise healthy, children. Moreover, a correlative analysis was performed betwee...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22972
更新日期:2014-11-01 00:00:00
abstract::Patients with cystic fibrosis (CF) can be discriminated from healthy subjects by measurement of the nasal potential difference, which has become a useful outcome measure for therapies directed toward correcting defective electrolyte transport in CF. A standard operating procedure was developed by a CF Foundation clini...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
doi:10.1002/ppul.10448
更新日期:2004-05-01 00:00:00
abstract::Between October, 1985 and May 1987, 29 children (mean age 22 +/- 22 months, range 2-54 months) with AIDS or ARC developed acute respiratory illness. The initial diagnostic procedure was flexible fiberoptic bronchoscopy, with bronchoalveolar lavage (BAL). BAL was positive for Pneumocystis carinii in 14 and for respirat...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950030609
更新日期:1987-11-01 00:00:00
abstract:BACKGROUND:The pH of exhaled breath condensate (EBC) of adolescent athletes engaged in vigorous physical activity is low compared to healthy controls; however, the ionic determinants of EBC pH and the acute effects of exercise on those determinants have not been definitively established. OBJECTIVES:This study had two ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21055
更新日期:2009-08-01 00:00:00