Abstract:
:Cystic fibrosis (CF) is one of the most common autosomal recessive and multisystemic diseases. CF affects many systems. One of these systems is the endocrine and exocrine functions of the pancreas, causing cystic fibrosis-related diabetes, which is extremely complex and has unique pathogenesis. Maturity-onset diabetes of the young (MODY) is a rare type of diabetes with autosomal dominant inheritance and is not expected in patients with CF. In this study, we present MODY due to a novel glucokinase gene mutation, which is an unexpected form of diabetes in patients with CF. This is previously unreported in the literature.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Hangül M,Erdoğan M,Hatipoğlu N,Köse Mdoi
10.1002/ppul.24746subject
Has Abstractpub_date
2020-05-01 00:00:00pages
E5-E7issue
5eissn
8755-6863issn
1099-0496journal_volume
55pub_type
abstract:BACKGROUND:Esophageal atresia (EA) is a congenital anomaly associated with substantial pulmonary morbidity throughout childhood. AIM:The aim of this study was to evaluate pulmonary complications among 59 five to 15-year-old children and adolescents with surgically corrected congenital EA. METHODS:Participants underwe...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23477
更新日期:2017-01-01 00:00:00
abstract:BACKGROUND:Acquiring sputum cultures from infants is considered challenging. We describe their yield in infants with cystic fibrosis (CF) and other chronic suppurative lung diseases (CSLDs). METHODS:Retrospective medical record review over a 4-year period, for infants aged 0-2 years with ≥2 airway bacterial cultures a...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25103
更新日期:2020-12-01 00:00:00
abstract:BACKGROUND:Chest computerized tomography (CT) scores are associated with the frequency of future pulmonary exacerbations in people with cystic fibrosis (CF). However, cut-off values to identify children with mild lung disease with different risks for frequent future pulmonary exacerbations have not been identified. ME...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ppul.24144
更新日期:2018-10-01 00:00:00
abstract:BACKGROUND:Patient-reported outcomes (PROs) are increasingly used to evaluate the efficacy of new treatments and the progression of chronic diseases. The Cystic Fibrosis Questionnaire-Revised (CFQ-R) is a disease-specific, PRO measure of health-related quality of life (HRQOL). We evaluated associations between changes ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21325
更新日期:2011-01-01 00:00:00
abstract::Pediatric lung transplantation has advanced over the years, providing a potential life-prolonging therapy to patients with cystic fibrosis. Despite this, many challenges in lung transplantation remain and result in worse outcomes than other solid organ transplants. As CF lung disease progresses, children and their car...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.23748
更新日期:2017-11-01 00:00:00
abstract:OBJECTIVE:To define the mortality and long-term outcomes of children undergoing tracheostomy. DESIGN:Retrospective chart and Texas Department of Health Bureau of Vital Statistics review of patients admitted to a Pediatric Intensive Care Unit who underwent a tracheostomy between 2001 and 2011. Mortality and decannulati...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23657
更新日期:2017-07-01 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::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: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::To develop and validate a questionnaire scale that can be used as a screening tool to investigate for the presence of childhood obstructive sleep apnea syndrome (OSAS) in Hong Kong Chinese children. Subjects suspected to have OSAS and controls were recruited. Parents completed a Hong Kong children sleep questionnaire ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20505
更新日期:2006-12-01 00:00:00
abstract::Diffuse panbronchiolitis is a progressive fatal respiratory disease of unknown cause seen predominantly in Southeast Asian adults. We report this condition in a 10-year-old child of Korean birth because of the uncommon presentation at this age and the favorable outcome associated with early diagnosis. Our objective wa...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23176
更新日期:2015-09-01 00:00:00
abstract:OBJECTIVES:Forced expiratory volume in 1 second (FEV1) is an important predictor of outcome in cystic fibrosis (CF). This study aimed to describe the change in lung function, nutritional status, and mortality of children with CF at a single center in Cape Town, South Africa, and identify factors associated with poor lu...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24726
更新日期:2020-06-01 00:00:00
abstract::Tracheal mucosal damage has been reported in autopsy specimens of cases with proximal esophageal atresia and distal tracheoesophageal fistula (EA-TEF) (Gross classification type C). Such changes have not been reported for isolated EA (Gross classification type A). Our hypothesis is that passage of amniotic fluid (AF) ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20490
更新日期:2006-11-01 00:00:00
abstract::Three adults with cystic fibrosis (one after lung transplantation) presented with fever, chest pain, and acute radiographic changes. The changes included a cavitary lesion of the lung, acute dense infiltrates, and lobar collapse. After failing conventional antibiotic therapy, the patients underwent flexible bronchosco...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20348
更新日期:2006-02-01 00:00:00
abstract:OBJECTIVES:We evaluated safety and efficacy of recombinant human growth hormone (rhGH) for improving growth, lean body mass (LBM), pulmonary function, and exercise tolerance in children with cystic fibrosis (CF) and growth restriction. STUDY DESIGN:Multicenter, open-label, controlled clinical trial comparing outcomes ...
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.21546
更新日期:2012-03-01 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::Our objective was to determine the repeatability of bronchial responsiveness to mannitol dry powder (MDP) as an objective marker of asthma in children. MDP challenge was performed in children with stable asthma at the same time of the day on two separate occasions within a week. The test was terminated after a 15% fal...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.10378
更新日期:2003-12-01 00:00:00
abstract::Inadequate intake and suboptimal growth are common problems for patients with CF and a critical target for intervention. The purpose of this study was to compare the growth outcomes of children with CF who participated in a randomized clinical trial to improve energy intake and weight to children with CF receiving sta...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ppul.21322
更新日期:2011-01-01 00:00:00
abstract:BACKGROUND:Most methods used for the assessment of severe steatorrhea in cystic fibrosis (CF) are sensitive. In fact, the tests show their usefulness in a borderline zone of the results. Yet, the existing data related to acid steatocrit (AS) are still contradictory. Therefore, in the present study we have aimed to asse...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21149
更新日期:2010-03-01 00:00:00
abstract::The collection of sputum for microbiological examination in young cystic fibrosis patients can be very difficult. However, a knowledge of bacterial flora colonizing the patient's airways is of paramount importance for proper antimicrobial therapy. It is also known that cystic fibrosis patients colonized by Pseudomonas...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10352
更新日期:2003-10-01 00:00:00
abstract::For the last thirty years, oral chloral hydrate has been used for sedation of infants for lung function testing. Recently, however, availability of chloral hydrate became severely limited in the United States after two manufacturers discontinued manufacturing in 2012. Due to these limitations and the recent and ongoin...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23100
更新日期:2015-02-01 00:00:00
abstract::Four patients with severe cystic fibrosis lung disease, anorexia and weight loss, received Megestrol Acetate (MA), as an appetite stimulant. The initial dose was 400-800 mg daily and was continued for 6-15 months. Appetite was improved, with significant weight gain in all patients and an increase in their weight for a...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199911)28:5<380::aid-ppul1
更新日期:1999-11-01 00:00:00
abstract::Pulmonary hemangioma is a rare benign tumor of the lungs. Airway hemangiomas in particular may lead to feeding difficulties, barking cough, stridor, respiratory distress, and even acute airway obstruction. Hence, such hemangiomas usually require early and aggressive treatment; however, the treatment modalities employe...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23008
更新日期:2014-08-01 00:00:00
abstract:OBJECTIVE:The aim of this study was to assess pulmonary function and its predictors in very low birth weight (birth weight ≤1,500 g) children (VLBWc) with or without bronchopulmonary dysplasia (BPD), born at gestational age ≤32 weeks at a single tertiary center during 1996-1999, after the introduction of surfactant the...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22676
更新日期:2013-08-01 00:00:00
abstract::Neurally adjusted ventilatory assistance (NAVA) can overcome technical difficulties with synchronizing noninvasive ventilation breaths with the patient, a modality often used in very low birthweight infants (VLBW) with apnea of prematurity (AOP). This study is a retrospective single-center investigation into whether N...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24464
更新日期:2019-11-01 00:00:00
abstract:BACKGROUND:The diagnosis of community-acquired pneumonia (CAP) is based mainly on the patient's medical history and physical examination. However, in severe cases a further evaluation including chest X-ray (CXR) may be necessary. At present, lung ultrasound (LUS) is not included in the diagnostic work-up of pneumonia. ...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.22585
更新日期:2013-03-01 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::Viral colds are an important cause of respiratory symptoms in normal children. Studies in adults suggest that inflammation in the lower respiratory tract is associated with viral colds, but there are no data regarding inflammation and viral infection in the lower airway of normal children with colds. We, therefore, st...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199908)28:2<109::aid-ppul6
更新日期:1999-08-01 00:00:00
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::Bronchial responsiveness to isocapnic hyperventilation with cold air (CAH) and to inhaled methacholine (MCH) was compared in 17 children with bronchial asthma. The response to cold air was expressed as the percent drop in FEV1 from baseline at 4 min. after the challenge (delta % FEV1 CAH), and the response to methacho...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.1950190603
更新日期:1995-06-01 00:00:00