Evaluation and management of pulmonary disease in ataxia-telangiectasia.

abstract：BACKGROUND:In cystic fibrosis (CF), the spectrum and frequency of CFTR variants differ by geography and race/ethnicity. CFTR variants in White patients are well-described compared with Latino patients. No studies of CFTR variants have been done in patients with CF in the Dominican Republic or Puerto Rico. METHODS:CFTR...

journal_title：Pediatric pulmonology

authors： Zeiger AM,McGarry ME,Mak ACY,Medina V,Salazar S,Eng C,Liu AK,Oh SS,Nuckton TJ,Jain D,Blackwell TW,Kang HM,Abecasis G,Oñate LC,Seibold MA,Burchard EG,Rodriguez-Santana J

更新日期：2020-02-01 00:00:00

abstract：BACKGROUND:Patients with bronchopulmonary dysplasia (BPD) may require tracheostomy for long-term mechanical ventilation. Polysomnography (PSG) may predict successful decannulation in children, however it is unclear how this success compares with children without a PSG. To better evaluate this role, we compared decannul...

journal_title：Pediatric pulmonology

authors： Quinlan C,Piccione J,Kim JY,Beck SE,Brooks L,Chandy-Patel R,Escobar E,Afolabi-Brown O

更新日期：2019-11-01 00:00:00

abstract：OBJECTIVES:To review 15-year trends in respiratory care of extremely preterm infants managed in a tertiary perinatal center; to identify the factors contributing to their evolution; and to determine whether these changes had an impact on infant mortality, severe morbidity, and growth. METHODS:Retrospective cohort stud...

journal_title：Pediatric pulmonology

authors： Habas F,Durand S,Milési C,Mesnage R,Combes C,Gavotto A,Picaud JC,Cambonie G

更新日期：2020-08-01 00:00:00

abstract：:We assessed the safety and use of induced sputum (IS) in children with cystic fibrosis (CF). Forty-eight children (19 males) with CF, mean age 12.6 (range, 7.3-17.0) years and median forced expired volume in 1 sec (FEV(1)) 48% (range, 14-77%) predicted were recruited. Patients spontaneously expectorated sputum and the...

journal_title：Pediatric pulmonology

authors： Suri R,Marshall LJ,Wallis C,Metcalfe C,Shute JK,Bush A

更新日期：2003-04-01 00:00:00

abstract：:During the past five decades there has been increasing interest in the potential anti-inflammatory effects of macrolide antibiotics. Low-dose macrolide therapy has dramatically increased survival in patients with diffuse panbronchiolitis, a disease with many similarities to cystic fibrosis (CF). This has led to furthe...

journal_title：Pediatric pulmonology

authors： Jaffé A,Bush A

更新日期：2001-06-01 00:00:00

abstract：:Pertussis carries a high risk of mortality in very young infants. The mechanism of refractory cardio-respiratory failure is complex and not clearly delineated. We aimed to examine the clinico-pathological features and suggest how they may be related to outcome, by multi-center review of clinical records and post-morte...

journal_title：Pediatric pulmonology

authors： Sawal M,Cohen M,Irazuzta JE,Kumar R,Kirton C,Brundler MA,Evans CA,Wilson JA,Raffeeq P,Azaz A,Rotta AT,Vora A,Vohra A,Abboud P,Mirkin LD,Cooper M,Dishop MK,Graf JM,Petros A,Klonin H

更新日期：2009-10-01 00:00:00

abstract：:We compared the performance of selected ultrasonic and jet nebulizers when aerosolizing several antibiotic formulations to determine optimum combinations for delivery of a respirable antibiotic aerosol. Three ultrasonic devices were tested: the UltraNeb 99/100, the UltraAIR and the Aerosonic. The reusable jet nebulize...

journal_title：Pediatric pulmonology

authors： Weber A,Morlin G,Cohen M,Williams-Warren J,Ramsey B,Smith A

更新日期：1997-04-01 00:00:00

abstract：BACKGROUND:Premature neonates frequently require oxygen supplementation as a therapeutic intervention that, while necessary, also exposes the lung to significant oxidant stress. We hypothesized that hyperoxia has a deleterious effect on alveolar epithelial barrier function rendering the neonatal lung susceptible to inj...

journal_title：Pediatric pulmonology

authors： Vyas-Read S,Vance RJ,Wang W,Colvocoresses-Dodds J,Brown LA,Koval M

更新日期：2018-01-01 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

authors： Opipari-Arrigan L,Powers SW,Quittner AL,Stark LJ

更新日期：2010-01-01 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

authors： Sidell DR,Wood RE,Hart CK

更新日期：2017-01-01 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

authors： Vogelberg C,Hirsch T,Rösen-Wolff A,Kerkmann ML,Leupold W

更新日期：2003-10-01 00:00:00

abstract：:Pneumogram (PG) recordings were performed in 87 very low birthweight (VLBW) asymptomatic infants just prior to hospital discharge to determine the relationships between: 1) a prior history of apnea of prematurity (AOP) and cardiorespiratory pattern; and 2) cardiorespiratory pattern at hospital discharge and postconcep...

journal_title：Pediatric pulmonology

authors： Hageman JR,Holmes D,Suchy S,Hunt CE

更新日期：1988-01-01 00:00:00

abstract：:The analysis of exhaled breath condensate (EBC) is a promising new method to measure airway inflammation. So far only limited data exist about methodological issues of EBC sampling in infants and young children. We evaluated 18 children with acute wheezy bronchitis (median age 24.3 months (min-max: 4-89.9)), 54 childr...

journal_title：Pediatric pulmonology

authors： Vogelberg C,Würfel C,Knoetzsch A,Kahlert A,Range U,Leupold W

更新日期：2007-12-01 00:00:00

abstract：BACKGROUND:Night-time symptoms and hypoxemia during sleep may affect sleep quality in children with chronic lung disease such as bronchiectasis. Poor sleep quality may impair growth, learning, and emotional development of children. Our aim was to assess the sleep quality and associated factors in children with non-cyst...

journal_title：Pediatric pulmonology

authors： Erdem E,Ersu R,Karadag B,Karakoc F,Gokdemir Y,Ay P,Akpinar IN,Dagli E

更新日期：2011-09-01 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

authors： Standaert TA,Boitano L,Emerson J,Milgram LJ,Konstan MW,Hunter J,Berclaz PY,Brass L,Zeitlin PL,Hammond K,Davies Z,Foy C,Noone PG,Knowles MR

更新日期：2004-05-01 00:00:00

abstract：:Short-acting β2-adrenergic receptor agonists are commonly used bronchodilators for symptom relief in asthmatics. Recent evidence demonstrated that prolonged exposure of cultured airway smooth muscle cells to β2 agonists directly augments procontractile signaling pathways with the change in expression of regulator of G...

journal_title：Pediatric pulmonology

authors： Labuda M,Laberge S,Brière J,Bérubé D,Krajinovic M

更新日期：2013-10-01 00:00:00

abstract：BACKGROUND:Pulmonary function testing is commonly performed for diagnosis and clinical management of respiratory diseases. It is important to use appropriate reference equations from healthy subjects for interpretation of data from infants with lung disease. This study aimed to determine if published reference equation...

journal_title：Pediatric pulmonology

authors： Lu Z,Foong RE,Kowalik K,Moraes TJ,Dubeau A,Lefebvre D,Davis SD,Balkovec S,Becker A,Mandhane P,Turvey SE,Lou W,Sears MR,Ratjen F,Subbarao P

更新日期：2018-07-01 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

authors： Boner AL,Vallone G,De Stefano G

更新日期：1989-01-01 00:00:00

abstract：BACKGROUND:Child exposure to cigarette smoke is harmful. It should be reduced through parental smoking cessation interventions. The aim of our study was to determine the impact of simple advice provided by the pediatrician on the smoking habits of parents of children with cystic fibrosis (CF), diabetes mellitus (DM) an...

journal_title：Pediatric pulmonology

authors： Lafont M,Morin C,Arrouy A,Rabeau A,Labouret G,Roditis L,Michelet M,Mittaine M

更新日期：2021-01-22 00:00:00

abstract：:Prader-Willi syndrome (PWS) is a genetic disorder, with hypotonia being the predominant feature in infancy, and developmental delay, obesity, and behavioral problems becoming more prominent in childhood and adolescence. Children with this disorder frequently suffer from excessive daytime sleepiness and have a primary ...

journal_title：Pediatric pulmonology

authors： Nixon GM,Brouillette RT

更新日期：2002-09-01 00:00:00

abstract：:Two hundred twenty-six healthy school children, with a mean age of 8.8 years; 62 girls mean age 8.8, 48 boys mean age 12.6 and 51 girls mean age 12.6 years at the start, were enrolled in a longitudinal study of lung function and tested annually for 5 years. All were free of respiratory symptoms, and none smoked more t...

journal_title：Pediatric pulmonology

authors： Hibbert ME,Hudson IL,Lanigan A,Landau LI,Phelan PD

更新日期：1990-01-01 00:00:00

abstract：:A survey of 47 cystic fibrosis referral centers was conducted to study the use of exercise in the management of patients with cystic fibrosis (CF). A questionnaire with 5 brief questions concerning the prescription and application of exercise therapy was sent to the first 47 centers (38.5%) listed in the 1988 CF Found...

journal_title：Pediatric pulmonology

authors： Kaplan TA,ZeBranek JD,McKey RM Jr

更新日期：1991-01-01 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

authors： Wang EE,Law BJ,Stephens D,Langley JM,MacDonald NE,Robinson JL,Dobson S,McDonald J,Boucher FD,de Carvalho V,Mitchell I

更新日期：1996-07-01 00:00:00

abstract：:We previously demonstrated that infants with a history of bronchopulmonary dysplasia (BPD) exhibit airflow obstruction and air trapping. The purpose of this study was to assess longitudinal changes in pulmonary function in infants with a history of BPD over the first 3 years of life, and the relationship to somatic gr...

journal_title：Pediatric pulmonology

authors： Filbrun AG,Popova AP,Linn MJ,McIntosh NA,Hershenson MB

更新日期：2011-04-01 00:00:00

abstract：:Our study was aimed at elucidating the effect of strenuous maternal exercise (running at a speed of 20 m/min) on fetal somatic growth and lung development. Dams were separated into three groups: (1) exercising during the entire course of pregnancy; (2) exercising from the 16th to the 20th gestational day; and (3) cont...

journal_title：Pediatric pulmonology

authors： Nagai A,Sakamoto K,Konno K

更新日期：1993-06-01 00:00:00

abstract：:The structure, distribution, and frequency of neuroendocrine (NE) cells in human fetal lung from early stages of development to term are described. Neuroendocrine cells were studied by electron microscopy and immunostaining for serotonin and bombesin, recently identified markers of these cells in human lung. The diffe...

journal_title：Pediatric pulmonology

authors： Cutz E,Gillan JE,Bryan AC

更新日期：1985-05-01 00:00:00

abstract：:Two male patients with cystic fibrosis (CF), both 18 years old, developed frontal lobe brain abscesses. Both patients presented with histories of intermittent headache over several days and occasional vomiting. Headache was not more evident in the mornings and not associated with visual disturbance in either patient. ...

journal_title：Pediatric pulmonology

authors： Cooper DM,Russell LE,Henry RL

更新日期：1994-06-01 00:00:00

abstract：:Bronchial reactivity was assessed in 66 children with bronchial asthma (aged 8-15 years) by provocation with histamine-HCl during a symptom-free period. A significant bronchial reaction to histamine was defined as a 50% increase in the resistance of the respiratory system (Rrs) determined by the forced oscillation tec...

journal_title：Pediatric pulmonology

authors： Holmgren D,Engström I,Bjure J,Sixt R,Aberg N

更新日期：1993-03-01 00:00:00

abstract：:We developed a simple method to identify neonates at high risk of bronchopulmonary dysplasia (BPD) and determined whether early (8 hours) and late (14 days) risk assessment is equally useful. A retrospective cohort design was utilized of subjects enrolled in multi-dose surfactant trials to develop each risk identifica...

journal_title：Pediatric pulmonology

authors： Rozycki HJ,Narla L

更新日期：1996-06-01 00:00:00

abstract：:The tidal flow volume (TFV) loop ratios of (1) time to peak flow (tPTEF) to total expiratory time (tE) [tPTEF/tE] and (2) volume to peak flow (VPTEF) to expired volume (VE) [VPTEF/VE] are reported to decrease with age in early life, and to decrease in subjects with obstructive airways disease (OAD). However, the mecha...

journal_title：Pediatric pulmonology

authors： Lødrup Carlsen KC,Stenzler A,Carlsen KH

更新日期：1997-12-01 00:00:00