Abstract:
:Cystic fibrosis (CF) is an autosomal recessive disease. It affects multiple body organs. The lungs and pancreas are the most affected which results in progressive lung damage and pancreatic insufficiency. Due to the disease process, CF patients require significantly higher caloric intake than recommended for other individuals. The nutritional goal for CF patients is to achieve normal growth and development and, once genetic potential is reached, to maintain good nutritional status throughout life. Evidence has shown that lung function is closely associated with nutritional status in CF and that nutritional status is an independent predictor of survival. Most CF patients are on a high calorie diet to help achieve normal growth and development and maintain good lung function. Inadequate caloric intake in CF can lead to malnutrition. Malnutrition in CF requires careful, multidisciplinary history taking, physical exam, and overall patient/family assessment. Only by determining the actual cause of the malnutrition can appropriate and safe therapies be used to treat it. Appetite stimulants, although efficacious in treating malnutrition in CF, should only be prescribed if decreased food intake secondary to inadequate appetite is the principal cause of the malnutrition and all other contributing factors have been assessed, ruled-out or treated. In this review, we attempted to summarize the use of several appetite stimulants used in CF and other diseases to improve appetite and maximize caloric intake.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Nasr SZ,Drury Ddoi
10.1002/ppul.20766subject
Has Abstractpub_date
2008-03-01 00:00:00pages
209-19issue
3eissn
8755-6863issn
1099-0496journal_volume
43pub_type
杂志文章,评审abstract:BACKGROUND:In young children with cystic fibrosis (CF) the forced expiratory volume in 1 second (FEV1 ) is often normal and a more sensitive measure to detect early obstructive lung disease is needed. AIM:To evaluate the progression of selected spirometry parameters with age in a cohort of CF patients and healthy chil...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22777
更新日期:2013-11-01 00:00:00
abstract:RATIONAL:This study aims at describing the use of bilevel positive airway pressure (BiPAP) in infants with severe bronchiolitis. WORKING HYPOTHESIS:The use of BiPAP in infants with bronchiolitis may be associated with a worst outcome. STUDY DESIGN:A single-center retrospective study performed from October 2013 to Apr...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25033
更新日期:2020-11-01 00:00:00
abstract:OBJECTIVE:Extremely low gestational age neonates (ELGANs) are at risk for pulmonary hypertension (PH). We hypothesized that PH, defined by echocardiogram at 36 weeks gestational age (GA), would associate with respiratory morbidity, increased oxidant stress, and reduced nitric oxide production. STUDY DESIGN:ELGANs in t...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23913
更新日期:2018-03-01 00:00:00
abstract::Antimicrobial proteins are important in lung defense and are potential therapeutic agents in chronic airways infection such as seen in cystic fibrosis (CF). In preparation for future clinical studies, we sought (1) to determine levels of three antimicrobial proteins [lactoferrin, lysozyme, and secretory leukoprotease ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21028
更新日期:2009-04-01 00:00:00
abstract::Bronchiolitis in infancy is a risk factor for development of asthma in the first decades of life, although the majority may be asymptomatic at school age. Respiratory symptoms are common in early life, and prediction of later asthma may be challenging. We aimed to study if simple clinical variables assessed at 2 years...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22675
更新日期:2013-06-01 00:00:00
abstract:OBJECTIVES:First, create a clinical severity score for patients with chronic lung disease of infancy (CLDi) following neonatal intensive care unit (NICU) stay. Second, using California wide population-based data, identify factors associated with clinical severity of CLDi at 4-9 months corrected gestational age (CGA). ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23148
更新日期:2015-09-01 00:00:00
abstract:BACKGROUND:Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) and endoscopic ultrasound with an echobronchoscope-guided fine needle aspiration (EUS-B-FNA) are useful modalities in the evaluation of mediastinal pathologies in adults. However, sparse data are available in children. OBJECTIVE:To...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24313
更新日期:2019-06-01 00:00:00
abstract::Indoor aeroallergen exposures increased asthma symptoms in Caucasians, but their determinants and relationship to asthma and allergy in Asians are unclear. This study investigated exposures to cat, cockroach, and Blomia tropicalis allergens in 115 Hong Kong families with asthmatic children. Patients underwent exhaled ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21391
更新日期:2011-07-01 00:00:00
abstract::Inhalation therapy for wheezy infants with either a nebulizer or a pressurized metered-dose inhaler (pMDI) through a spacer is common practice. The aim of our study was to compare aerosol delivery to wheezy infants from a nebulizer and from a pMDI via two small volume spacers. Twenty wheezy infants (aged 4-12 months) ...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/(sici)1099-0496(199703)23:3<212::aid-ppul7
更新日期:1997-03-01 00:00:00
abstract:OBJECTIVES:We sought to describe the phenotype for patients with P.I.G. including presentation, evaluation, cardiac co-morbidities, high resolution computed tomography findings, and outcomes. METHODS:With institutional review board approval, we performed a retrospective review of patients with biopsy-proven P.I.G. Bio...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24123
更新日期:2018-12-01 00:00:00
abstract::Changes in the relative contribution of ribcage and abdomen during augmented breaths were assessed in two groups of infants (less than 2 weeks and 4-12 weeks old). In both groups ribcage and abdominal movements increased during augmented breaths in quiet sleep. In the older infants the relative increase was greater fo...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950040303
更新日期:1988-01-01 00:00:00
abstract:BACKGROUND:Treatment regimens for patients with cystic fibrosis (CF) are time-consuming and complex, resulting in consistently low adherence rates. To date, few studies have evaluated innovative technologies to improve adherence in this population. Current infection control guidelines for patients with CF seek to minim...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21164
更新日期:2010-02-01 00:00:00
abstract:OBJECTIVES:A pilot study to compare pulmonary mechanics in a neonatal intensive care unit (NICU) cohort, specifically, comparing lung elastance between male and female infants in the NICU. HYPOTHESIS:Anecdotally, male infants are harder to ventilate than females. We hypothesize that males have higher model-based elast...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24538
更新日期:2020-01-01 00:00:00
abstract::Airway inflammation of eosinophilic asthma (EA) attributes to Th2 response, leaving the role of Th17 response unknown. Signal transducer and activator of transcription 3 (STAT3) induce both suppressors of cytokine signaling 3 (SOCS3) and retinoic acid receptor-related orphan nuclear receptor γ (RORγt) to initiate Th17...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24759
更新日期:2020-06-01 00:00:00
abstract:BACKGROUND:Juvenile dermatomyositis (JDM) is the main cause of chronic idiopathic inflammatory myopathy of autoimmune origin in children. The aim of this multicenter prospective study was to describe respiratory status and treatment of children followed for JDM. METHODS AND PATIENTS:Clinical manifestations, pulmonary ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
doi:10.1002/ppul.22742
更新日期:2013-10-01 00:00:00
abstract::IL-10 is an anti-inflammatory cytokine that may have a protective role in acute lung injury. IL-10 expression is affected by a single-nucleotide polymorphism (SNP) located at position -1082 (G to A). The A allele is associated with lower IL-10 production. Low IL-10 production has been linked to the development of BPD....
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20182
更新日期:2005-05-01 00:00:00
abstract:BACKGROUND:Prone sleeping position, use of soft mattresses and head covering by bedclothes are known risk factors for sudden infant death syndrome (SIDS). Rebreathing carbon dioxide (CO(2) ) may be a possible mechanism or a confounding factor of SIDS. OBJECTIVE:To compare the aeration properties of a new concept of in...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21351
更新日期:2011-02-01 00:00:00
abstract::The gasdermin A (GSDMA) and gasdermin B (GSDMB) genes are located at 17q21.2. The GSDM family genes have been studied in the gastrointestinal tract but recent reports suggest that GSDMB is associated with childhood asthma in several populations. We investigated the association of the GSDMA and GSDMB variants with asth...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21424
更新日期:2011-07-01 00:00:00
abstract::Neonatal exposure to intermittent hypoxia results in altered ventilatory response to subsequent hypoxia in animal models. The effect of similar exposure in human infants is unknown. Our objective was to determine the impact of sleep disordered breathing (SDB) in early infancy on ventilatory response in infants. We rec...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22568
更新日期:2013-03-01 00:00:00
abstract::Inhaled corticosteroids are commonly used in cystic fibrosis (CF), but there are few studies evaluating their safety in young children. We, therefore, prospectively administered beclomethasone diproprionate (BDP) to 12 clinically stable young children with CF to examine the safety of this therapy with respect to adren...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1122
更新日期:2001-10-01 00:00:00
abstract:BACKGROUND:Ivacaftor is a significant innovation in the treatment of cystic fibrosis (CF) with gating mutations. A substantial percentage of patients with CF have severe lung involvement, but these patients are usually excluded from phase III clinical trials. Thus, the effectiveness of ivacaftor in this population has ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24424
更新日期:2019-09-01 00:00:00
abstract::A 14-year-old Korean boy was admitted with cough, hemoptysis, and fever. A chest X-ray showed a solitary pulmonary mass and pneumonitis. Bronchial biopsy by fiberoptic bronchoscopy revealed a poorly differentiated small cell carcinoma. All of the staging information indicated that the patient had limited disease. Duri...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(200004)29:4<317::aid-ppul1
更新日期:2000-04-01 00:00:00
abstract:AIMS:To explore whether gender differences in the Scandinavian Cystic Fibrosis (CF) patients exist in the areas of key clinical parameters, complications, and medication. METHODS:Cross-sectional data on 890 (416 female) pancreatic insufficient CF patients were evaluated regarding chronic infection, body mass index, lu...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21265
更新日期:2010-10-01 00:00:00
abstract::Our objective was to review the clinical features and radiographic manifestations of chest wall lesions in a pediatric chest unit. Twenty-five patients (11 males and 14 females, aged 14 months to 15 years) were suspected of having a chest wall lesion on the basis of clinical examination and chest radiograph. A retrosp...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10440
更新日期:2004-03-01 00:00:00
abstract:BACKGROUND:Most international asthma guidelines recommend that children ≤5 years with asthma or recurrent wheezing be treated with daily low- moderate dose inhaled corticosteroids (ICS) as the preferred controller and leukotriene receptor antagonists (LTRA) as alternative therapy. There is no systematic review comparin...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24176
更新日期:2018-12-01 00:00:00
abstract::Drug reaction with eosinophilia and systemic symptom (DRESS) syndrome is a rare and severe side-effect, mainly described after intake of anticonvulsants, allopurinol, or antibiotics. It usually begins within 2 months after drug introduction. Symptoms include cutaneous rash, hematologic abnormalities, and internal orga...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23567
更新日期:2017-04-01 00:00:00
abstract::The efficacy and tolerability of high-dose salmeterol (100 mcg, BID) and albuterol (2.5 mg, BID) were compared with those of albuterol (2.5 mg, BID) in outpatients with cystic fibrosis in a randomized, double-blind, double-dummy, placebo-controlled, crossover study with both short- (4 weeks of each) and long-term (24 ...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/ppul.10162
更新日期:2002-10-01 00:00:00
abstract::Our objective was to determine whether a simple method of maintaining positive pressure ventilation during nonbronchoscopic bronchoalveolar lavage (NB-BAL) would successfully reduce the incidence and/or severity of desaturation events. Our design was a clinical trial with historical controls. Seventy ventilated pediat...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20082
更新日期:2004-09-01 00:00:00
abstract:INTRODUCTION:The prevalence of both childhood obesity and childhood asthma has increased dramatically over the past few decades. Little is known concerning the role of body composition and lifestyle influences on airway health in children. PURPOSE:To determine whether body composition, fruit and vegetable intake (FV) ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21393
更新日期:2011-05-01 00:00:00
abstract::In cystic fibrosis the bronchiectatic conducting airways have large numbers of neutrophils in their walls and in their luminal contents. The neutrophil's primary granule enzyme activities of elastase and peroxidase are increased in the sputum of these patients. It has been postulated that these enzymes--together or in...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199707)24:1<29::aid-ppul5>
更新日期:1997-07-01 00:00:00