Abstract:
:This report describes a patient with biliary atresia (BA) associated with polysplenia syndrome who showed a rapid progression of intrapulmonary arteriovenous shunting (IPS), resulting in a fatal outcome. Intrauterine ultrasonography at 36 weeks of gestation revealed fetal abnormalities, including situs inversus, absent retrohepatic inferior vena cava, and azygous connection. She was diagnosed postnatally as BA because of persistent acholic stool and neonatal jaundice. She underwent hepatic portoenterostomy at age 158 days. The gallbladder and the hepatic ducts were hypoplastic, and the common bile duct was absent. Magnetic resonance image and operative findings also identified polysplenia and an absent portal trunk. Liver histology showed cirrhotic changes and bile duct proliferation. Postoperatively, she achieved good bile secretion, with gradual decrease of total bilirubin. However, she had repeated febrile episodes, and computerized tomography at age 7 months showed multiple liver cysts. Thereafter, she presented with exertional dyspnea. Contrast-enhanced echocardiography showed IPS with a degree of 2/III at age 8 months and 3/III at 10 months. (99m)Technetium-labeled macroaggregated albumin ((99m)Tc-MAA) scintigraphy revealed a shunt ratio of 25.5% at 9 months and 39.7% at 10 months. Percutaneous transhepatic drainage of the bile cysts was performed without success. Sludged bile was obtained. However, respiratory distress rapidly progressed, and she died at age 11 months. In the present patient, the association of polysplenia syndrome and absent portal vein with BA, as well as liver cirrhosis, seemed to be contributing factors to rapid progression of IPS in early life.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Kimura T,Hasegawa T,Sasaki T,Okada A,Mushiake Sdoi
10.1002/ppul.10285subject
Has Abstractpub_date
2003-06-01 00:00:00pages
494-8issue
6eissn
8755-6863issn
1099-0496journal_volume
35pub_type
杂志文章abstract:OBJECTIVE:To determine reference values of sniff nasal inspiratory pressure (SNIP) in healthy children. METHODS:This cross-sectional observational study included healthy children aged 6 to 11 years of both sexes. The volunteers underwent a pulmonary function test to rule out respiratory disorders. Respiratory muscle s...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24591
更新日期:2020-02-01 00:00:00
abstract:OBJECTIVE:To carry out a cost-effectiveness analysis of omitting chest radiography in the diagnosis of infant bronchiolitis. HYPOTHESIS:Omitting chest radiographs in the diagnosis of typical bronchiolitis was expected to reduce costs without adversely affecting the detection rate of alternate diseases. STUDY DESIGN:A...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20948
更新日期:2009-02-01 00:00:00
abstract::"Pulmonary Interstitial Glycogenosis: Diagnostic Evaluation and Clinical Course," written by Liptzin et al is a timely and insightful phenotypic summary of a rare pediatric interstitial lung disease. Twenty-four infants with biopsy-proven pulmonary interstitial glycogenosis (PIG) were reviewed at their center. Genetic...
journal_title:Pediatric pulmonology
pub_type: 评论,杂志文章
doi:10.1002/ppul.24167
更新日期:2018-12-01 00:00:00
abstract:OBJECTIVE:To describe and analyze the characteristics and the early risk factors for mortality of noninvasive ventilation (NIV) in critically ill children. STUDY DESIGN:A multicenter, prospective, observational 2-year study carried out with critically ill patients (1 month - 18 years of age) who needed NIV. Clinical d...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.25246
更新日期:2020-12-31 00:00:00
abstract:OBJECTIVES:a: To evaluate the long-term outcome of parapneumonic effusions (PPE) in children regarding lung function and exercise tolerance, (b) to investigate the role of bronchial asthma in the outcome of PPE. METHODS:The design of the study included 51 children with PPE, at least 2 years after the initial infection...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23054
更新日期:2015-06-01 00:00:00
abstract::Forced oscillatory respiratory resistance was measured in 138 healthy children aged 2- to 16-years-old using a commercial unit. Regression analysis was performed using the resistance measurements obtained at frequencies of 6 Hz and 26 Hz and averages of measurements obtained at frequencies of from 6-26 Hz, 6-10 Hz, an...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950010305
更新日期:1985-05-01 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: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
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::Pulmonary hemorrhage (PH) is a serious complication causing acute respiratory distress in the premature infant, and it is associated with significant mortality and morbidity. The role of inflammatory mediators in this condition is largely undefined. Serial tracheal aspirates (TA) were obtained at intervals from 65 mec...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10141
更新日期:2002-08-01 00:00:00
abstract::Some studies have suggested that lung clearance index (LCI) is age-independent among healthy subjects early in life, which implies that ventilation distribution does not vary with growth. However, other studies of older children and adolescents suggest that ventilation becomes more homogenous with somatic growth. We d...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21553
更新日期:2012-04-01 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
pub_type: 杂志文章
doi:10.1002/ppul.1950150604
更新日期:1993-06-01 00:00:00
abstract:BACKGROUND/OBJECTIVE:Our objective was to obtain follow-up pulmonary function testing and assessment of clinical respiratory outcomes, at 1-2 years, in preterm infants whose mothers were randomized to a single rescue course of antenatal steroids (AS) versus placebo. METHODS:Follow-up of a randomized, double-blinded tr...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ppul.23711
更新日期:2017-09-01 00:00:00
abstract::With dual energy X-ray absorptiometry (DEXA), it is possible to quantitate important aspects of growth in children with cystic fibrosis (CF), supplementing the usual measures of height and weight. Of particular concern during growth is the accumulation of bone mineral, since osteoporosis and fractures are well-recogni...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199902)27:2<80::aid-ppul3>
更新日期:1999-02-01 00:00:00
abstract:OBJECTIVES:To investigate the adherence and the self-reported barriers to general and respiratory exercises reported by individuals with cystic fibrosis (CF). STUDY DESIGN:An exploratory, experimental study. METHODS:Community-dwelling individuals aged 16 years and over, diagnosed with CF, who were accompanied in refe...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24912
更新日期:2020-10-01 00:00:00
abstract::Tracheal bronchus (TRB) has been generally considered an anatomical variant of the tracheobronchial tree without a precise pathological effect. Its prevalence is estimated to be between 0.2% to 3% of all children undergoing bronchoscopy and scientific information has been limited to case reports or small case series. ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
doi:10.1002/ppul.24435
更新日期:2019-10-01 00:00:00
abstract:OBJECTIVE:The study was designed to assess the use of newer sidestream microstream end tidal carbon dioxide (ETCO(2) ) device in predicting blood carbon dioxide (PCO(2) ) measurements in very low birth weight (VLBW = birth weight <1,500 g) and non-VLBW NICU neonates. STUDY DESIGN:Sidestream microstream ETCO(2) detecto...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22593
更新日期:2013-03-01 00:00:00
abstract::We describe a method for measuring carbon monoxide diffusing capacity (DL(CO)) and alveolar volume (V(A)) in sleeping infants, using a single 4-sec breath-hold technique. The breath-hold maneuver is obtained by inducing a respiratory pause of the respiratory system. Several inflations of the respiratory system with ro...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20403
更新日期:2006-06-01 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::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::Twenty patients with cystic fibrosis (CF) were trained to mobilize intrabronchial secretions by a new method of positive expiratory pressure mask (PEP mask) physiotherapy (PT). Patients repeatedly expired forcefully through the PEP mask; expiratory resistance was varied by eight different internal diameter resistors a...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950020608
更新日期:1986-11-01 00:00:00
abstract:INTRODUCTION:Peripheral muscle dysfunction is increasingly recognized as complicating respiratory disease, but this is difficult to measure non-invasively. RESEARCH QUESTION:Can skeletal muscle function and efficiency be measured during exercise non-invasively using respiratory mass spectrometry (RMS); and is the know...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20899
更新日期:2009-03-01 00:00:00
abstract::Multiresistance in Stenotrophomonas maltophilia limits the effectiveness of antimicrobial therapy for infections due to this organism. It can be of special concern in cystic fibrosis (CF) patients due to frequent antimicrobial administration. The in vitro activity of 41 antimicrobial agents against 76 epidemiologicall...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10216
更新日期:2003-02-01 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::In past decades, several chest radiograph scoring systems for cystic fibrosis were developed. This study was performed to compare interobserver variability of six different radiograph scores and to correlate them with clinical parameters. Thirty chest radiographs of 30 patients with cystic fibrosis were scored accordi...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10280
更新日期:2003-06-01 00:00:00
abstract::A thorough understanding of the early natural history of cystic fibrosis (CF) lung disease is critical for the development of effective interventions in the youngest patients. We assessed the evolution of pulmonary infection, inflammation, and clinical course among 40 infants over a 2-year period through annual bronch...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1144
更新日期:2001-11-01 00:00:00
abstract:BACKGROUND:Gastro-esophageal reflux (GOR) may contribute to lung disease in children with cystic fibrosis (CF). There is conflicting evidence regarding the effect of chest physiotherapy (CPT) in the head-down position on GOR. Furthermore, there is currently no evidence on the impact of physiotherapy on GOR as assessed ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ppul.21524
更新日期:2012-02-01 00:00:00
abstract::Bronchoalveolar lavage (BAL) performed with a fiberoptic bronchoscope (FOB) is a useful method for sampling alveolar contents. Since the smallest FOB with a channel has a diameter of 3.6 mm, BAL is difficult to accomplish through artificial airways (AA) less than 5.0 mm I.D. We used a 4F balloon wedge pressure cathete...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950130110
更新日期:1992-05-01 00:00:00
abstract::We report a case of fungal pleural effusion secondary to presumed valproate induced pancreatitis with pseudocyst and stricture formation. A child with dyskinetic cerebral palsy who had been on sodium valproate for several years was transferred for drainage of a left sided pleural effusion. Pleural fluid culture consis...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20982
更新日期:2009-06-01 00:00:00
abstract::Nine young asthmatic children aged 2-5 years underwent methacholine challenge after placebo or albuterol administered by metered dose inhaler through a spacer device (Babyhaler) with a face mask in a double-blind, cross-over, randomized study. The methacholine challenge was performed using chest auscultation to define...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/ppul.1950170503
更新日期:1994-05-01 00:00:00