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Irreversible Respiratory Failure in a Full-Term Infant with Features of Pulmonary Interstitial Glycogenosis as Well as Bronchopulmonary Dysplasia.

ABSTRACT: Pulmonary interstitial glycogenosis (PIG) is a rare interstitial lung disease in the newborns. We report on the clinical presentation and pathological findings of a full-term male infant with pulmonary hypertension requiring extracorporeal membrane oxygenation (ECMO). An open lung biopsy demonstrated interstitial changes resembling pulmonary interstitial glycogenosis as well as bronchopulmonary dysplasia (BPD), without convincing evidence of maturational arrest, infection, alveolar proteinosis, or alveolar capillary dysplasia. The boy was treated with glucocorticoids and, after a few days, was weaned from ECMO. A few hours later, the patient died due to acute severe pulmonary hypertension with acute right ventricular failure. The etiology and underlying pathogenic mechanisms of PIG are unknown. The clinical outcomes are quite varied. Deaths have been reported when PIG exists with abnormal lung development and pulmonary vascular growth and congenital heart disease. No mortality has been reported in PIG together with BPD in full-term infants. In this article, we reported on a full-term infant with interstitial changes resembling PIG and BPD who expired despite no convincing evidence of an anatomical maturational arrest or congenital heart disease.

SUBMITTER: Jiskoot-Ermers ME

PROVIDER: S-EPMC4603843 | biostudies-other | 2015 Oct

REPOSITORIES: biostudies-other

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Biomarkers for Bronchopulmonary Dysplasia in the Preterm Infant.

Project description:Bronchopulmonary dysplasia (BPD) is a chronic inflammatory lung disease of very-low-birth-weight (VLBW) preterm infants, associated with arrested lung development and a need for supplemental oxygen. Over the past few decades, the incidence of BPD has significantly raised as a result of improved survival of VLBW infants requiring mechanical ventilation. While early disease detection is critical to prevent chronic lung remodeling and complications later in life, BPD is often difficult to diagnose and prevent due to the lack of good biomarkers for identification of infants at risk, and overlapping symptoms with other diseases, such as pulmonary hypertension (PH). Due to the current lack of effective treatment available for BPD and PH, research is currently focused on primary prevention strategies, and identification of biomarkers for early diagnosis, that could also represent potential therapeutic targets. In addition, novel histopathological, biochemical, and molecular factors have been identified in the lung tissue and in biological fluids of BPD and PH patients that could associate with the disease phenotype. In this review, we provide an overview of biomarkers for pediatric BPD and PH that have been identified in clinical studies using various biological fluids. We also present a brief summary of the information available on current strategies and guidelines to prevent and diagnose BPD and PH, as well as their pathophysiology, risk factors, and experimental therapies currently available.

| S-EPMC4814627 | biostudies-literature

Microbiome features of bronchopulmonary dysplasia

Project description:Microbiome features of bronchopulmonary dysplasia

| PRJNA1105631 | ENA

Interstitial pneumonia pattern on day 7 chest radiograph predicts bronchopulmonary dysplasia in preterm infants.

Project description:BackgroundEarly identification of infants at higher risk of developing bronchopulmonary dysplasia (BPD) may enable a targeted approach to reduce BPD. We aimed to evaluate the hypothesis that the interstitial pneumonia pattern on the day 7 chest radiograph predicts BPD or death before 36 weeks postmenstrual age (PMA).MethodsA retrospective cohort study was performed on 336 preterm infants (birth weight < 1500 g and gestational age < 32 postmenstrual weeks) who were admitted to a single tertiary academic center between January 2008 and December 2014. Day 7 chest radiographs were independently reviewed by two pediatric radiologists who were unaware of the clinical information regarding each individual infant.ResultsData from 304 infants who survived more than 7 days after birth were collected. The interstitial pneumonia pattern on the day 7 chest radiograph was independently associated with BPD or death before 36 weeks PMA (odds ratio [OR] 4.0, 95% confidence interval [CI] 1.1-14.4). The interstitial pneumonia pattern on the day 7 chest radiograph predicted BPD or death with a specificity of 98%. Histologic chorioamnionitis was a preceding factor that was independently associated with the interstitial pneumonia pattern on the day 7 chest radiograph (OR 3.7, 95% CI 1.3-10.3).ConclusionsThe interstitial pneumonia pattern on the day 7 chest radiograph has a high specificity for predicting BPD or death and can be utilized to select high-risk preterm infants who will benefit from potentially preventive interventions against BPD.

| S-EPMC5433188 | biostudies-literature

Bronchopulmonary dysplasia in extremely premature infant with congenital lobar emphysema: a case report.

Project description:BackgroundCongenital lobar emphysema (CLE) is a congenital pulmonary cystic disease, characterized by overinflation of the pulmonary lobe and compression of the surrounding areas. Most patients with symptoms need an urgent surgical intervention. Caution and alertness for CLE is required in cases of local emphysema on chest X-ray images of extremely premature infants with bronchopulmonary dysplasia (BPD).Case presentationHere, we report a case of premature infant with 27 + 4 weeks of gestational age who suddenly presented with severe respiratory distress at 60 days after birth. Chest X-ray and computed tomography (CT) indicated emphysema in the middle lobe of the right lung. The diagnosis of CLE was confirmed by histopathological examinations.ConclusionsAlthough extremely premature infants have high-risk factors of bronchopulmonary dysplasia due to their small gestational age, alertness for CLE is necessary if local emphysema is present. Timely pulmonary CT scan and surgical interventions should be performed to avoid the delay of the diagnosis and treatment.

| S-EPMC8256543 | biostudies-literature

Respiratory outcomes of "new" bronchopulmonary dysplasia in adolescents: A multicenter study.

Project description:ObjectiveLong-term respiratory consequences of bronchopulmonary dysplasia (BPD) in preterm infants born in the post-surfactant era ("new" BPD) remain partially unknown. The present study aimed to evaluate the respiratory outcomes of "new" BPD in adolescents who were born preterm.MethodsThis multicenter, cross-sectional study included 286 adolescents born between 2003 and 2005 (mean age: 14.2 years); among them, 184 and 102 were born extremely preterm (EP; <28 weeks' gestation) and moderate-late preterm (32 to <37 weeks' gestation), respectively. Among EP adolescents, 92 had BPD, and 92 did not. All participants underwent lung function tests, skin prick testing, and questionnaires on asthma symptoms and quality of life.ResultsEP adolescents with BPD had significantly lower forced expiratory volume in 1 s (FEV1 ), forced vital capacity (FVC), FEV1 /FVC ratio, and forced expiratory flow between 25% and 75% of FVC than other included adolescents. FEV1 /FVC ratios were below the lower limit of normal (z-score <-1.645) in 30.4% of EP adolescents with BPD, 13.0% of EP adolescents without BPD, and 11.8% of adolescents who were born moderate-late preterm. Bronchodilator response and air-trapping were significantly higher in BPD adolescents than in other adolescents. Diffusion capacity was significantly lower in EP adolescents than in moderate-late preterm adolescents. Asthma symptoms and quality-of-life scores were similar among groups.ConclusionEP adolescents with "new" BPD had poorer pulmonary function than EP adolescents without BPD or moderate-late preterm adolescents. Further studies are needed to determine whether "new" BPD is associated with early-onset chronic obstructive pulmonary disease in adulthood.

| S-EPMC8246560 | biostudies-literature

Predictors of Bronchopulmonary Dysplasia in 625 Neonates with Respiratory Distress Syndrome.

Project description:ObjectiveThis study was designed to investigate the predictors of bronchopulmonary dysplasia in neonates with respiratory distress syndrome.MethodsThis was a single-center retrospective cohort study conducted between 1 January 2015 and 31 December 2020. A total of 625 neonates with respiratory distress syndrome (RDS) were enrolled. Demographic data, clinical presentations, complications and related treatment information were collected and analyzed. We used bivariate and multivariate logistic-regression analyses to determine significant predictors of bronchopulmonary dysplasia (BPD) in RDS neonates.ResultsIn these 625 neonates, 102 (16.3%) of them developed BPD. Bivariate analysis and multivariate logistic-regression analyses revealed that birthweight, gestational age under 32 weeks, duration of oxygen therapy over 10 days, asphyxia, patent ductus arteriosus, transfusion of red blood cells (packed red blood cells) and surfactant use were significantly associated with the development of BPD.ConclusionBirthweight, gestational age <32 weeks, total duration of oxygen therapy >10 days, asphyxia, patent ductus arteriosus, need for red blood cell infusion, and the use of pulmonary surfactant were important predictors of BPD in neonates with RDS.

| S-EPMC9122647 | biostudies-literature

Novel biomarkers of bronchopulmonary dysplasia and bronchopulmonary dysplasia-associated pulmonary hypertension.

Project description:ObjectiveTo quantify and compare levels of potential biomarkers in neonates with (i) Bronchopulmonary dysplasia (BPD); (ii) BPD-associated pulmonary hypertension (BPD-PH); (iii) PH without BPD; and (iv) neonates without lung disease at ~36 weeks postmenstrual age.Study designMultiple potential biomarkers were measured in plasma samples of 90 patients using a multi-spot enzyme-linked immunosorbent assay. Statistical tests done included one-way ANOVA to compare levels of biomarkers between different groups.ResultsHigher levels of ICAM-1 were present in infants with BPD and correlated with its severity. Infants with BPD have significantly higher levels of ANG-2 and lower levels of ANG-1. Infants with PH have higher levels of: IL-6, IL-8, IL-10, and TNF-α. Infants with BPD-PH have significantly lower levels of MCP-1 and higher levels of IL-1β than infants with PH without BPD.ConclusionICAM-1 may be used as a specific biomarker for diagnosis of BPD and its severity.

| S-EPMC7664991 | biostudies-literature

Predictors of Developmental and Respiratory Outcomes Among Preterm Infants With Bronchopulmonary Dysplasia.

Project description:Objectives: To examine the importance of perinatal and postnatal environmental factors on developmental and respiratory outcomes among preterm infants with bronchopulmonary dysplasia (BPD). Methods: Preterm infants (<32 weeks of gestation) born at a single tertiary medical center between 2012 and 2015 were included. Development was assessed at 12 months corrected age. Parents retrospectively completed a health and lifestyle questionnaire reviewing their child's health during the first 2 years of life. A linear regression model was applied to assess the effect of various perinatal and postnatal factors on development. A machine-learning algorithm was trained to assess factors affecting inhaler use. Results: Of 398 infants meeting the inclusion criteria, 208 qualified for the study: 152 (73.1%) with no BPD, 40 (19.2%) with mild BPD, and 16 (7.7%) with moderate-severe BPD. Those in the moderate-severe group were more likely to be male, have mothers who were less educated, and require longer ventilation periods and less time to regain birth weight. They were also more likely to have mothers with asthma/allergies and to have a parent who smoked. Those in the moderate-severe BPD group exhibited significantly lower developmental scores (85.2 ± 16.4) than the no-BPD group (99.3 ± 10.9) and the mild BPD group (97.8 ± 11.7, p < 0.008) as well as more frequent inhaler use (p = 0.0014) than those with no or mild BPD. In addition to perinatal factors, exposure to breast milk, income level and daycare attendance positively affected development. Exposure to cigarette smoke, allergies among family members and daycare attendance proved to be important factors in inhaler use frequency. Conclusions: Postnatal environmental factors are important in predicting and modifying early childhood outcomes among preterm infants.

| S-EPMC8667805 | biostudies-literature

Duration of noninvasive respiratory support and risk for bronchopulmonary dysplasia or death.

Project description:ObjectiveTo determine whether the duration of noninvasive respiratory support exposure is associated with bronchopulmonary dysplasia (BPD) or death in preterm infants.MethodsMulticenter, retrospective study of infants born at <29 weeks' gestation. The association between days on noninvasive respiratory support and BPD or death was determined using instrumental variable techniques and generalized propensity score matching to account for potential confounding by illness severity.ResultsAmong 6268 infants 36% developed BPD or died. The median duration of noninvasive respiratory support was 18 days. There was inconsistency in the association between noninvasive support and BPD or death when analyzed by instrumental variable techniques (Average Marginal Effect -0.37; 95% CI -1.23 to 0.50) and generalized propensity score matching (Average Marginal Effect 0.46; 95% CI 0.33 to 0.60).ConclusionFindings on the association between duration of exposure to noninvasive respiratory support and the development of BPD or death were inconclusive.ClinicaltrialsGov idGeneric Database:NCT00063063.

| S-EPMC9007818 | biostudies-literature

Role of Ureaplasma Respiratory Tract Colonization in Bronchopulmonary Dysplasia Pathogenesis: Current Concepts and Update.

Project description:Respiratory tract colonization with the genital mycoplasma species Ureaplasma parvum and Ureaplasma urealyticum in preterm infants is a significant risk factor for bronchopulmonary dysplasia (BPD). Recent studies of the ureaplasmal genome, animal infection models, and human infants have provided a better understanding of specific virulence factors, pathogen-host interactions, and variability in genetic susceptibility that contribute to chronic infection, inflammation, and altered lung development. This review provides an update on the current evidence supporting a causal role of ureaplasma infection in BPD pathogenesis. The current status of antibiotic trials to prevent BPD in Ureaplasma-infected preterm infants is also reviewed.

| S-EPMC4662049 | biostudies-literature

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