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Sample records for bronchopulmonary dysplasia

  1. Bronchopulmonary dysplasia

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/001088.htm Bronchopulmonary dysplasia To use the sharing features on this page, please enable JavaScript. Bronchopulmonary dysplasia (BPD) is a long-term ( chronic ) lung condition ...

  2. Bronchopulmonary dysplasia

    National Research Council Canada - National Science Library

    Deakins, Kathleen M

    2009-01-01

    Bronchopulmonary dysplasia (BPD) is the most common chronic respiratory disease that results from complications related to the lung injury during the treatment of respiratory distress syndrome, or develops in older infants when...

  3. [Bronchopulmonary dysplasia].

    Science.gov (United States)

    Monte, Luciana F Velloso; Silva Filho, Luiz Vicente F da; Miyoshi, Milton Harumi; Rozov, Tatiana

    2005-01-01

    To present a wide-ranging review of the literature on broncopulmonary dysplasia, covering new definitions, pathophysiology, prevention, treatment, prognosis and progression. The most relevant articles published on the subject since it was first described in 1967 were selected from MEDLINE search results. Bronchopulmonary dysplasia is considered one of the primary causes of chronic lung disease among infants. It is associated with frequent and prolonged hospital admissions, in particular for pulmonary diseases, with high rates of mortality and alterations to neuropsychomotor development and pondero-statural growth. Pathogenesis is complex, being primarily influenced by prematurity, infection, supplementary oxygen and mechanical ventilation. Prevention involves appropriate prenatal care, the prevention of premature delivery, prenatal corticosteroids, surfactant replacement therapy and "protective" ventilatory strategies. Treatment of bronchopulmonary dysplasia patients demands a multidisciplinary team. When indicated, oxygen supplementation is extremely important. Despite increased risk of morbidity and mortality during the first years of life, long term progress is favorable in the majority of cases. Bronchopulmonary dysplasia has been and continues to be studied in great depth with the objective of identifying its causes and possible prevention and treatment strategies. Controversies remain with respect of these issues and also about the prognosis of these patients, in particular when the subject is long-term progress of "new" bronchopulmonary dysplasia patients.

  4. Bronchopulmonary dysplasia: a review

    DEFF Research Database (Denmark)

    ali, Zarqa; Peter, Schmidt; Dodd, James Keith

    2013-01-01

    Introduction The prevalence of bronchopulmonary dysplasia (BPD), one of the most frequently occurring complications following preterm birth, is increasing due to increased survival of preterm infants. Methods Systematic literature review. Conclusion The etiology is multifactorial, with prematurity...... being a prerequisite for the development of BPD. Over time, there have been many different and new treatment modalities, some of them have reduced the severity of the disease, but none of them have been able to impact upon the increasing incidence of BPD....

  5. Epidemiology of bronchopulmonary dysplasia.

    Science.gov (United States)

    Jensen, Erik A; Schmidt, Barbara

    2014-03-01

    Bronchopulmonary dysplasia (BPD) is among the most common and serious sequelae of preterm birth. BPD affects at least one-quarter of infants born with birth weights less than 1500 g. The incidence of BPD increases with decreasing gestational age and birth weight. Additional important risk factors include intrauterine growth restriction, sepsis, and prolonged exposure to mechanical ventilation and supplemental oxygen. The diagnosis of BPD predicts multiple adverse outcomes including chronic respiratory impairment and neurodevelopmental delay. This review summarizes the diagnostic criteria, incidence, risk factors, and long-term outcomes of BPD. Copyright © 2014 Wiley Periodicals, Inc.

  6. Displasia broncopulmonar Bronchopulmonary dysplasia

    Directory of Open Access Journals (Sweden)

    Luciana F. Velloso Monte

    2005-04-01

    Full Text Available OBJETIVO: Apresentar uma ampla revisão da literatura sobre displasia broncopulmonar, abordando novas definições, fisiopatologia, prevenção, tratamento, prognóstico e evolução. FONTE DOS DADOS: Foram selecionados os artigos mais relevantes sobre o tema, desde a sua descrição inicial, em 1967, pesquisados na MEDLINE. SÍNTESE DOS DADOS: A displasia broncopulmonar é considerada uma das principais causas de doença pulmonar crônica em lactentes. Está associada a hospitalizações freqüentes e prolongadas, especialmente por doenças pulmonares, altos índices de mortalidade e alterações no desenvolvimento neuropsicomotor e no crescimento pôndero-estatural. A patogênese é complexa e influenciada principalmente por prematuridade, infecção, oxigênio suplementar e ventilação mecânica. A prevenção envolve o acompanhamento pré-natal adequado, a prevenção do parto prematuro, o uso pré-natal do corticosteróide, a terapia de reposição de surfactante e o uso de estratégias ventilatórias "protetoras". O tratamento do paciente com displasia broncopulmonar demanda uma equipe multidisciplinar. Quando indicada, a suplementação de oxigênio é de extrema importância. Apesar de maior risco de morbimortalidade nos primeiros anos de vida, a evolução em longo prazo é favorável na maioria das vezes. CONCLUSÕES: A displasia broncopulmonar vem sendo profundamente estudada na tentativa de identificação das suas causas e possibilidades de prevenção e de tratamento. Ainda existem controvérsias quanto a esses assuntos e também em relação ao prognóstico desses pacientes, especialmente quando se trata da evolução tardia da "nova" displasia broncopulmonar.OBJECTIVE: To present a wide-ranging review of the literature on bronchopulmonary dysplasia, covering new definitions, pathophysiology, prevention, treatment, prognosis and progression. SOURCES OF DATA: The most relevant articles published on the subject since it was first

  7. Advances in bronchopulmonary dysplasia.

    Science.gov (United States)

    Strueby, Lannae; Thébaud, Bernard

    2014-06-01

    Bronchopulmonary dysplasia (BPD) is a chronic respiratory condition primarily affecting infants born less than 28 weeks gestational age. BPD and the diagnostic criteria that define it have evolved since the initial description of the disease more than four decades ago. BPD is one of the most common and serious complications of extreme premature birth. Despite advances in neonatal care and continued research into therapeutic strategies the incidence of BPD remains unchanged. Pharmacologic approaches to the management of BPD include methylxanthines, corticosteroids, and vitamin A supplementation. Supportive therapies including the increased use of non-invasive ventilation and careful oxygen delivery strive to reduce injury inflicted on the developing lung. Stem cell-based therapies are a new investigational strategy showing promise for the prevention or treatment of BPD. The goal of this review is to highlight the evolution of BPD and review current and potential future therapeutic strategies for BPD.

  8. Predictors of Bronchopulmonary Dysplasia Formation

    Directory of Open Access Journals (Sweden)

    Ye.N. Okhotnikova

    2013-12-01

    Full Text Available The authors studied and analyzed a group of prenatal, intra-, and neonatal factors leading to the development of bronchopulmonary dysplasia (BPD. Nnegative impact of BPD on the development of chronic bronchopulmonary pathology in children in the first three years of life was determined.

  9. [Magnesium and bronchopulmonary dysplasia].

    Science.gov (United States)

    Fridman, Elena; Linder, Nehama

    2013-03-01

    Bronchopulmonary dysplasia (BPD) is a chronic lung disease that occurs in premature infants who have needed mechanical ventilation and oxygen therapy. BPD is defined as the presence of persistent respiratory symptoms, the need for supplemental oxygen to treat hypoxemia, and an abnormal chest radiograph at 36 weeks gestational age. Proinflammatory cytokines and altered angiogenic gene signaling impair prenatal and postnatal lung growth, resulting in BPD. Postnatal hyperoxia exposure further increases the production of cytotoxic free radicals, which cause lung injury and increase the levels of proinflammatory cytokines. Magnesium is the fourth most abundant metal in the body. It is commonly used for the treatment of preeclamsia, as well as for premature labor alleviation. Magnesium's role in BPD development is not clear. A significant association between high magnesium levels at birth and respiratory distress syndrome (RDS), pulmonary interstitial emphysema in the extremely low birth weight, respiratory failure, and later development BPD was found. Conversely, low magnesium intake is associated with lower lung functions, and hypomagnesemia was found in 16% of patients with acute pulmonary diseases. Magnesium is used for the treatment of asthmatic attacks. Magnesium deficiency in pregnant women is frequently seen due to low intake. Hypomagnesemia was also found among preterm neonates and respiratory distress syndrome (RDS). Experimental hypomagnesemia evokes an inflammatory response, and oxidative damage of tissues. These were accompanied by changes in gene expression mostly involved in regulation of cell cycle, apoptosis and remodeling, processes associated with BPD. It is rational to believe that hypomagnesemia can contribute to BPD pathogenesis.

  10. Ureaplasma and bronchopulmonary dysplasia.

    Science.gov (United States)

    Gancia, Paolo; Delogu, Antonio; Pomero, Giulia

    2014-03-01

    Advances in neonatal intensive care have greatly improved survival rates for children born in a very early stage of lung development (i.e. less than 26 weeks of gestation). In these premature babies, even low levels of oxygen and methods of minimally invasive ventilation may disrupt the growth of the distal airways, a condition described as "new" bronchopulmonary dysplasia (BPD). Ureaplasma infection can occur in utero or in the perinatal period in premature infants, in some of which the infection with these organisms triggers an important lung pro-inflammatory and pro-fibrotic response, and may increase the risk of developing BPD. The inflammation may be worsened by exposure to oxygen and mechanical ventilation. At present, clinical studies have not clarified the role of Ureaplasma in the pathogenesis of BPD and there is insufficient evidence to determine whether antibiotic treatment of Ureaplasma has influence on the development of BPD and its comorbidities. Future research in the context of well-designed and controlled clinical trials of adequate statistical power should focus on how to determine whether the treatment of Ureaplasma decreases lung inflammation, reduces rates of BPD, and improves long-term neurodevelopment. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  11. Managing Children with Bronchopulmonary Dysplasia

    Directory of Open Access Journals (Sweden)

    A. A. Baranov

    2016-01-01

    Full Text Available Bronchopulmonary dysplasia is one of the most significant early childhood chronic respiratory diseases. The article features modern approaches to preventing, diagnosing and treating broncho-pulmonary dysplasia, as well as ways of preventing complications and undesirable disease outcomes in patients older than 3 years. Members of professional associations — Union of Pediatricians of Russia and Russian Association of Perinatal Medicine Specialists — have summarized the experience of managing this category of patients at leading Russian pediatric centers according to the principles of evidence-based medicine and have provided scientific and practical data corresponding to the world level of knowledge with regard to the present problem.

  12. [Bronchopulmonary dysplasia: definitions and classifications].

    Science.gov (United States)

    Sánchez Luna, M; Moreno Hernando, J; Botet Mussons, F; Fernández Lorenzo, J R; Herranz Carrillo, G; Rite Gracia, S; Salguero García, E; Echaniz Urcelay, I

    2013-10-01

    Bronchopulmonary dysplasia is the most common sequelae related to very low birth weight infants, mostly with those of extremely low birth weight. Even with advances in prevention and treatment of respiratory distress syndrome associated with prematurity, there is still no decrease in the incidence in this population, although a change in its clinical expression and severity has been observed. There are, however, differences in its frequency between health centres, probably due to a non-homogeneously used clinical definition. In this article, the Committee of Standards of the Spanish Society of Neonatology wishes to review the current diagnosis criteria of bronchopulmonary dysplasia to reduce, as much as possible, these inter-centre differences. Copyright © 2013 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  13. Pulmonary hypertension in bronchopulmonary dysplasia.

    Science.gov (United States)

    Ambalavanan, Namasivayam; Mourani, Peter

    2014-03-01

    Pulmonary hypertension is common in bronchopulmonary dysplasia and is associated with increased mortality and morbidity. This pulmonary hypertension is due to abnormal microvascular development and pulmonary vascular remodeling resulting in reduced cross-sectional area of pulmonary vasculature. The epidemiology, etiology, clinical features, diagnosis, suggested management, and outcomes of pulmonary hypertension in the setting of bronchopulmonary dysplasia are reviewed. In summary, pulmonary hypertension is noted in a fifth of extremely low birth weight infants, primarily those with moderate or severe bronchopulmonary dysplasia, and persists to discharge in many infants. Diagnosis is generally by echocardiography, and some infants require cardiac catheterization to identify associated anatomic cardiac lesions or systemic-pulmonary collaterals, pulmonary venous obstruction or myocardial dysfunction. Serial echocardiography and B-type natriuretic peptide measurement may be useful for following the course of pulmonary hypertension. Currently, there is not much evidence to indicate optimal management approaches, but many clinicians maintain oxygen saturation in the range of 91 to 95%, avoiding hypoxia and hyperoxia, and often provide inhaled nitric oxide, sometimes combined with sildenafil, prostacyclin, or its analogs, and occasionally endothelin-receptor antagonists. Copyright © 2014 Wiley Periodicals, Inc.

  14. Genetic predisposition to bronchopulmonary dysplasia.

    Science.gov (United States)

    Lal, Charitharth Vivek; Ambalavanan, Namasivayam

    2015-12-01

    The objective of this study is to review the candidate gene and genome-wide association studies relevant to bronchopulmonary dysplasia, and to discuss the emerging understanding of the complexities involved in genetic predisposition to bronchopulmonary dysplasia and its outcomes. Genetic factors contribute much of the variance in risk for BPD. Studies to date evaluating single or a few candidate genes have not been successful in yielding results that are replicated in GWAS, perhaps due to more stringent p-value thresholds. GWAS studies have identified only a single gene (SPOCK2) at genome-wide significance in a European White and African cohort, which was not replicated in two North American studies. Pathway gene-set analysis in a North American cohort confirmed involvement of known pathways of lung development and repair (e.g., CD44 and phosphorus oxygen lyase activity) and indicated novel molecules and pathways (e.g., adenosine deaminase and targets of miR-219) involved in genetic predisposition to BPD. The genetic basis of severe BPD is different from that of mild/moderate BPD, and the variants/pathways associated with BPD vary by race/ethnicity. A pilot study of whole exome sequencing identified hundreds of genes of interest, and indicated the overall feasibility as well as complexity of this approach. Better phenotyping of BPD by severity and pathophysiology, and careful analysis of race/ethnicity is required to gain a better understanding of the genetic basis of BPD. Future translational studies are required for the identification of potential genetic predispositions (rare variants and dysregulated pathways) by next-generation sequencing methods in individual infants (personalized genomics). Copyright © 2015 Elsevier Inc. All rights reserved.

  15. Rates of bronchopulmonary dysplasia in very preterm neonates in Europe

    DEFF Research Database (Denmark)

    Gortner, Ludwig; Misselwitz, Björn; Milligan, David

    2011-01-01

    A considerable local variability in the rate of bronchopulmonary dysplasia (BPD) has been recorded previously.......A considerable local variability in the rate of bronchopulmonary dysplasia (BPD) has been recorded previously....

  16. Association of TLR polymorphisms with bronchopulmonary dysplasia.

    Science.gov (United States)

    Malash, Amr Hosny; Ali, Aliaa Adel; Samy, Rania Mohamed; Shamma, Radwa Ahmed

    2016-10-30

    Bronchopulmonary dysplasia (BPD) remains a leading cause of morbidity and mortality during infancy. Evidence suggests that the Toll-like receptor (TLR) signaling pathway plays an integral role in lung inflammation and injury. This study aimed to detect single nucleotide polymorphisms (SNPs) in TLR pathway genes [TLR5 and Toll-interleukin 1 receptor domain-containing adaptor protein (TIRAP)] among preterm neonates and to determine their association with the development and severity of bronchopulmonary dysplasia. Copyright © 2016 Elsevier B.V. All rights reserved.

  17. Ureaplasma urealyticum colonization, prematurity and bronchopulmonary dysplasia

    NARCIS (Netherlands)

    vanWaarde, WM; Brus, F; Okken, A; Kimpen, JLL

    The aim of the present study was to determine the association between the presence of Ureaplasma urealyticum in endotracheal aspirates and bronchopulmonary dysplasia (BPD). In addition, a review of similar studies from the English literature is presented. During the period February 1990 until March

  18. Bronchopulmonary dysplasia: incidence and risk factors.

    Science.gov (United States)

    Brener Dik, Pablo H; Niño Gualdron, Yeimy M; Galletti, María F; Cribioli, Carolina M; Mariani, Gonzalo L

    2017-10-01

    Bronchopulmonary dysplasia is the most common chronic pulmonary sequela among very low birth weight infants. The objective of this study was to estimate its incidence in our Neonatal Unit over the past 5 years and analyze associated risk factors. An observational and analytical study was conducted in a retrospective cohort, using data obtained from a prospective database of infants born at Hospital Italiano de Buenos Aires with a birth weight of less than 1500 grams between January 2010 and December 2014. The incidence of bronchopulmonary dysplasia and its association with several secondary outcome measures were studied. Two hundred and forty-five patients were included. The incidence of moderate/severe bronchopulmonary dysplasia was 22%, and it was associated with a younger gestational age and lower birth weight. A significant association was observed with surfactant use, mechanical ventilation requirement, and length of mechanical ventilation. Patients with moderate/severe bronchopulmonary dysplasia had a higher incidence of patent ductus arteriosus and late-onset sepsis. A lower birth weight (adjusted odds ratio |-#91;aOR|-#93;: 0.99, 95% confidence interval |-#91;CI|-#93;: 0.991-0.997, pdysplasia in our unit was associated with a lower birth weight and the length of mechanical ventilation. Among infants born at less than 32 weeks of gestation, intrauterine growth restriction accounted for an additional risk.

  19. Novel treatment options for bronchopulmonary dysplasia

    NARCIS (Netherlands)

    Chen, X.

    2017-01-01

    Bronchopulmonary dysplasia is the most common complication when premature birth occurs at less than 28 weeks gestational age. The general aim of this thesis is to explore the therapeutic potential of interventions in signaling pathways, involved in lung development and oxidative

  20. Animal models of bronchopulmonary dysplasia. The term mouse models

    National Research Council Canada - National Science Library

    Berger, Jessica; Bhandari, Vineet

    2014-01-01

    The etiology of bronchopulmonary dysplasia (BPD) is multifactorial, with genetics, ante- and postnatal sepsis, invasive mechanical ventilation, and exposure to hyperoxia being well described as contributing factors...

  1. Early (bronchopulmonary dysplasia in preterm infants.

    Science.gov (United States)

    Doyle, Lex W; Cheong, Jeanie L; Ehrenkranz, Richard A; Halliday, Henry L

    2017-10-24

    Bronchopulmonary dysplasia remains a major problem in neonatal intensive care units. Persistent inflammation in the lungs is the most likely underlying pathogenesis. Corticosteroids have been used to prevent or treat bronchopulmonary dysplasia because of their potent anti-inflammatory effects. To examine the relative benefits and adverse effects of systemic postnatal corticosteroids commenced within the first seven days of life for preterm infants at risk of developing bronchopulmonary dysplasia. For the 2017 update, we used the standard search strategy of Cochrane Neonatal to search the Cochrane Central Register of Controlled Trials (CENTRAL; 2017, Issue 1); MEDLINE via PubMed (January 2013 to 21 February 2017); Embase (January 2013 to 21 February 2017); and the Cumulative Index to Nursing and Allied Health Literature (CINAHL) (January 2013 to 21 February 2017). We also searched clinical trials databases, conference proceedings, and reference lists of retrieved articles for randomised controlled trials (RCTs) and quasi-randomised trials. For this review, we selected RCTs examining systemic postnatal corticosteroid treatment within the first seven days of life (early) in high-risk preterm infants. Most studies evaluated the use of dexamethasone, but we also included studies that assessed hydrocortisone, even when used primarily for management of hypotension. We used the GRADE approach to assess the quality of evidence.We extracted and analysed data regarding clinical outcomes that included mortality, bronchopulmonary dysplasia, death or bronchopulmonary dysplasia, failure to extubate, complications during primary hospitalisation, and long-term health outcomes. We included 32 RCTs enrolling a total of 4395 participants. The overall risk of bias of included studies was probably low, as all were RCTs, and most trials used rigorous methods. Investigators reported significant benefits for the following outcomes overall: lower rates of failure to extubate, decreased

  2. Impact of Nutrition on Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Poindexter, Brenda B; Martin, Camilia R

    2015-12-01

    Bronchopulmonary dysplasia (BPD) remains a common morbidity of prematurity. Although the pathogenesis of BPD is recognized to be both multifactorial and complex, the role of nutrition in the pathophysiology of BPD is typically limited to management after a diagnosis has been made. Infants born small for gestational age and those who experience postnatal growth failure are more likely to have BPD. Therapies for lung disease, such as fluid restriction, diuretics, and corticosteroids, can negatively impact postnatal growth. Future research is needed to optimize nutritional strategies in the neonatal intensive care unit and following hospital discharge. Copyright © 2015 Elsevier Inc. All rights reserved.

  3. Airway Disease and Management in Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Amin, Raouf S; Rutter, Michael J

    2015-12-01

    This article presents an overview of the diagnosis and management of airway problems encountered in infants with severe bronchopulmonary dysplasia (BPD). Respiratory failure in premature infants develops as a result of parenchymal and airway diseases. The survival of increasingly premature infants and the ventilatory support required by premature lungs may result in airway disease. The management of respiratory failure depends on whether it is primarily caused by parenchymal versus airway diseases. Continuous airway pressure early in the neonatal period has favorably changed the incidence of BPD. This article discusses the indications, timing, and guidelines for care of tracheotomy. Copyright © 2015 Elsevier Inc. All rights reserved.

  4. Impaired Pulmonary Vascular Development in Bronchopulmonary Dysplasia

    Science.gov (United States)

    Baker, Christopher D.; Abman, Steven H.

    2015-01-01

    Bronchopulmonary dysplasia (BPD), the chronic lung disease associated with preterm birth, results from disruption of normal pulmonary vascular and alveolar growth. Though BPD was once described as primarily due to postnatal injury from mechanical ventilation and oxygen therapy after preterm birth, it is increasingly appreciated that BPD results from antenatal and perinatal factors that interrupt lung development in infants born at the extremes of prematurity. The lung in BPD consists of a simplified parenchymal architecture that limits gas exchange and leads to increased cardiopulmonary morbidity and mortality. This review outlines recent advances in the understanding of pulmonary vascular development and describes how disruption of these mechanisms results in BPD. We point to future therapies that may augment postnatal vascular growth to prevent and treat this severe chronic lung disease. PMID:26044102

  5. Altered lung development in bronchopulmonary dysplasia.

    Science.gov (United States)

    Hadchouel, Alice; Franco-Montoya, Marie-Laure; Delacourt, Christophe

    2014-03-01

    Bronchopulmonary dysplasia (BPD) is the main respiratory sequela of extreme prematurity. Its pathophysiology is complex, involving interactions between host and environment, likely to be significantly influenced by genetic factors. Thus, the clinical presentation and histological lesions have evolved over time, along with the reduction in neonatal injuries, and the care of more immature children. Impaired alveolar growth, however, is a lesion consistently observed in BPD, such that it is a key feature in BPD, and is even the dominant characteristic of the so-called "new" forms of BPD. This review describes the key molecular pathways that are believed to be involved in the genesis of BPD. Much of our understanding is based on animal models, but this is increasingly being enriched by genetic approaches, and long-term respiratory functional studies. Copyright © 2014 Wiley Periodicals, Inc.

  6. Initial respiratory management in preterm infants and bronchopulmonary dysplasia

    Directory of Open Access Journals (Sweden)

    Ester Sanz López

    2011-01-01

    Full Text Available BACKGROUND: Ventilator injury has been implicated in the pathogenesis of bronchopulmonary dysplasia. Avoiding invasive ventilation could reduce lung injury, and early respiratory management may affect pulmonary outcomes. OBJECTIVE: To analyze the effect of initial respiratory support on survival without bronchopulmonary dysplasia at a gestational age of 36 weeks. DESIGN/METHODS: A prospective 3-year observational study. Preterm infants of 26 weeks (sensitivity =89.5% and specificity = 67%. The need for prolonged mechanical ventilation could be an early marker for the development of bronchopulmonary dysplasia. This finding could help identify a target population with a high risk of chronic lung disease. Future research is needed to determine other strategies to prevent bronchopulmonary dysplasia in this high-risk group of patients.

  7. Tracheobronchomalacia Is Associated with Increased Morbidity in Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Hysinger, Erik B; Friedman, Nicholas L; Padula, Michael A; Shinohara, Russell T; Zhang, Huayan; Panitch, Howard B; Kawut, Steven M

    2017-06-16

    Tracheobronchomalacia is a common comorbidity in neonates with bronchopulmonary dysplasia. However, the effect of tracheobronchomalacia on the clinical course of bronchopulmonary dysplasia is not well-understood. We sought to assess the impact of tracheobronchomalacia on outcomes in neonates with bronchopulmonary dysplasia in a large, multi-center cohort. We preformed a cohort study of 974 neonates with bronchopulmonary dysplasia admitted to 27 neonatal intensive care units participating in the Children's Hospital Neonatal Database who had undergone bronchoscopy. In hospital morbidity for neonates with bronchopulmonary dysplasia and tracheobronchomalacia (N=353, 36.2%) was compared to those without tracheobronchomalacia (N=621, 63.8%) using mixed-effects multivariate regression. Neonates with tracheobronchomalacia and bronchopulmonary dysplasia had more comorbidities, such as gastroesophageal reflux (OR=1.65, 95%CI 1.23- 2.29, P=0.001) and pneumonia (OR=1.68, 95%CI 1.21-2.33, P=0.002) and more commonly required surgeries such as tracheostomy (OR=1.55, 95%CI 1.15-2.11, P=0.005) and gastrostomy (OR=1.38, 95%CI 1.03-1.85, P=0.03) compared with those without tracheobronchomalacia. Neonates with tracheobronchomalacia were hospitalitized (118 ± 93 vs 105 ± 83 days, P=0.02) and ventilated (83.1 ± 91.1 vs 67.2 ± 71.9 days, P=0.003) longer than those without tracheobronchomalacia. Upon discharge, neonates with tracheobronchomalacia and BPD were more likely to be mechanically ventilated (OR=1.37, 95CI 1.01-1.87 P=0.045) and possibly less likely to receive oral nutrition (OR=0.69, 95%CI 0.47-1.01, P=0.058). Tracheobronchomalacia is common in neonates with bronchopulmonary dysplasia who undergo bronchoscopy and is associated with longer and more complicated hospitalizations.

  8. Bronchopulmonary dysplasia: an old and new disease

    Directory of Open Access Journals (Sweden)

    Caterina Franco

    2014-06-01

    Full Text Available Bronchopulmonary dysplasia (BPD is one of the most common and significant medical complications associated with prematurity. It is made more serious by its morbidity and mortality rates. Although recent advances in clinical practice (prenatal steroids, surfactants, new ventilatory strategies, nutritional support have contributed to improving the clinical course and outcomes of neonates with BPD, its overall incidence has not changed in the last decade owing to a concomitant increase in survival of prematures. The incidence of BPD is in fact inversely proportional to birth weight: 30% for neonates weighing less than 1,000 g, with different percentages in the single centres depending on clinical management and the ventilation criteria applied. However, to date, BPD represents not only a chronic pulmonary pathology in infancy that prevalently affects premature neonates who undergo mechanical ventilation and oxygen therapy for respiratory distress syndrome (RDS, but also prematures with minor signs of initial pulmonary pathology or term neonates requiring aggressive ventilatory support due to an acute and severe lung pathology. Proceedings of the International Course on Perinatal Pathology (part of the 10th International Workshop on Neonatology · October 22nd-25th, 2014 · Cagliari (Italy · October 25th, 2014 · The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken

  9. [Premature infants bronchopulmonary dysplasia: past and present].

    Science.gov (United States)

    Hadchouel, A; Delacourt, C

    2013-08-01

    Bronchopulmonary dysplasia (BPD) is the most common chronic respiratory disease in premature infants. BPD was first described by Northway in 1967 as a chronic respiratory condition that developed in premature infants exposed to mechanical ventilation and high oxygen supplementation. DBP is currently defined by the need for supplemental oxygen at 28 days of life (mild BPD) and at the 36 weeks of post-menstrual age (moderate and severe BPD). With the advances of neonatal care, epidemiological characteristics and mechanisms of the disease as well as pathological characteristics and clinical course have profoundly changed within the last two decades, but still no effective curative treatment exists and BPD continue to occur among 10 to 20% of premature infants. Furthermore, BPD is a significant source of respiratory and neuro-cognitive morbidities. Thus, its treatment makes a considerable demand on health services. Regarding its pathophysiological mechanisms, it is now established that BPD is a complex disease combining genetic susceptibility and environmental injuries. The identification of genetic variants involved in BPD is a potential source of innovative development in terms of diagnosis and treatment. Indeed, no curative or effective prophylactic therapeutic exists and BPD treatment is currently symptomatic. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  10. Pathogenesis and Treatment of Bronchopulmonary Dysplasia

    Science.gov (United States)

    Gien, Jason; Kinsella, John P.

    2012-01-01

    Purpose of review Bronchopulmonary dysplasia (BPD) is a chronic lung disease of infancy affecting mostly premature infants with significant morbidity and mortality. Improved survival of very immature infants has led to increased numbers of infants with this disorder. Acute and chronic lung injury and impaired postnatal lung growth are thought to be responsible for the development of BPD. While changes in clinical practice have improved the clinical course and outcomes for infants with BPD, over the last decade, the overall incidence of BPD has not changed. This review will describe the pre and postnatal factors that contribute to the pathogenesis of BPD as well as current and experimental therapies for treatment of BPD. Recent findings The factors that contribute to the pathogenesis of BPD are well described, however recent studies have better defined how these factors modulate lung growth. Inflammation, proinflammatory cytokines and altered angiogenic gene signaling contribute to lung injury and impair pre and postnatal lung growth resulting in BPD, however to date no therapy has been identified that potently and consistently prevents or reverses their effects on lung growth. We will discuss the cell signaling pathways affected in BPD and current therapies available for modulating these pathways. Summary Despite current advances in neonatal care, BPD remains a heavy burden on health care resources. New treatments directed either at reducing lung injury or improving lung growth are under study. PMID:21494147

  11. The genetic predisposition to bronchopulmonary dysplasia

    Science.gov (United States)

    Yu, Kun-Hsing; Li, Jingjing; Snyder, Michael; Shaw, Gary M.; O’Brodovich, Hugh M.

    2016-01-01

    Purpose of review Bronchopulmonary dysplasia (BPD) is a prevalent chronic lung disease in premature infants. Twin studies have shown strong heritability underlying this disease; however, the genetic architecture of BPD remains unclear. Recent findings A number of studies employed different approaches to characterize the genetic aberrations associated with BPD, including candidate gene studies, genome-wide association studies, exome sequencing, integrative omics analysis, and pathway analysis. Candidate gene studies identified a number of genes potentially involved with the development of BPD, but the etiological contribution from each gene is not substantial. Copy number variation studies and three independent genome-wide association studies did not identify genetic variations significantly and consistently associated with BPD. A recent exome-sequencing study pointed to rare variants implicated in the disease. In this review, we summarize these studies’ methodology and findings, and suggest future research directions to better understand the genetic underpinnings of this potentially life-long lung disease. Summary Genetic factors play a significant role in the development of BPD. Recent studies suggested that rare variants in genes participating in lung development pathways could contribute to BPD susceptibility. PMID:26963946

  12. Pulmonary Hemosiderosis in Children with Bronchopulmonary Dysplasia

    Directory of Open Access Journals (Sweden)

    David Kurahara

    2014-01-01

    Full Text Available We describe a possible association between pulmonary hemosiderosis (PH and a history of bronchopulmonary dysplasia (BPD. Both patients were born at 28-week gestation and presented with PH at ages 22 months and 6 years, respectively. Both initially presented with cough and tachypnea, and bronchoalveolar lavage showed evidence of hemosiderin-laden macrophages. Initial hemoglobin levels were < 4 g/dL and chest radiographs showed diffuse infiltrates that cleared dramatically within days after initiation of intravenous corticosteroids. In the first case, frank pulmonary blood was observed upon initial intubation, prompting the need for high frequency ventilation, immediate corticosteroids, and antibiotics. The mechanical ventilation wean was made possible by the addition of mycophenolate mofetil (MMF and hydroxychloroquine. Slow tapering off of medications was accomplished over 6 years. These cases represent a possible correlation between prematurity-associated BPD and PH. We present a review of the literature regarding this possible association. In addition, MMF proved to be life-saving in one of the PH cases, as it has been in pulmonary hemorrhage related to systemic lupus erythematosus. Further studies are warranted to investigate the possible association between PH and prematurity-related BPD, as well as the use of MMF in the treatment of PH.

  13. Docosahexaenoic Acid and Bronchopulmonary Dysplasia in Preterm Infants.

    Science.gov (United States)

    Collins, Carmel T; Makrides, Maria; McPhee, Andrew J; Sullivan, Thomas R; Davis, Peter G; Thio, Marta; Simmer, Karen; Rajadurai, Victor S; Travadi, Javeed; Berry, Mary J; Liley, Helen G; Opie, Gillian F; Tan, Kenneth; Lui, Kei; Morris, Scott A; Stack, Jacqueline; Stark, Michael J; Chua, Mei-Chien; Jayagobi, Pooja A; Holberton, James; Bolisetty, Srinivas; Callander, Ian R; Harris, Deborah L; Gibson, Robert A

    2017-03-30

    Studies in animals and in humans have suggested that docosahexaenoic acid (DHA), an n-3 long-chain polyunsaturated fatty acid, might reduce the risk of bronchopulmonary dysplasia, but appropriately designed trials are lacking. We randomly assigned 1273 infants born before 29 weeks of gestation (stratified according to sex, gestational age [bronchopulmonary dysplasia, defined on a physiological basis (with the use of oxygen-saturation monitoring in selected infants), at 36 weeks of postmenstrual age or discharge home, whichever occurred first. A total of 1205 infants survived to the primary outcome assessment. Of the 592 infants assigned to the DHA group, 291 (49.1% by multiple imputation) were classified as having physiological bronchopulmonary dysplasia, as compared with 269 (43.9%) of the 613 infants assigned to the control group (relative risk adjusted for randomization strata, 1.13; 95% confidence interval [CI], 1.02 to 1.25; P=0.02). The composite outcome of physiological bronchopulmonary dysplasia or death before 36 weeks of postmenstrual age occurred in 52.3% of the infants in the DHA group and in 46.4% of the infants in the control group (adjusted relative risk, 1.11; 95% CI, 1.00 to 1.23; P=0.045). There were no significant differences between the two groups in the rates of death or any other neonatal illnesses. Bronchopulmonary dysplasia based on a clinical definition occurred in 53.2% of the infants in the DHA group and in 49.7% of the infants in the control group (P=0.06). Enteral DHA supplementation at a dose of 60 mg per kilogram per day did not result in a lower risk of physiological bronchopulmonary dysplasia than a control emulsion among preterm infants born before 29 weeks of gestation and may have resulted in a greater risk. (Funded by the Australian National Health and Medical Research Council and others; Australian New Zealand Clinical Trials Registry number, ACTRN12612000503820 .).

  14. Pathogenesis of bronchopulmonary dysplasia: when inflammation meets organ development.

    Science.gov (United States)

    Shahzad, Tayyab; Radajewski, Sarah; Chao, Cho-Ming; Bellusci, Saverio; Ehrhardt, Harald

    2016-12-01

    Bronchopulmonary dysplasia is a chronic lung disease of preterm infants. It is caused by the disturbance of physiologic lung development mainly in the saccular stage with lifelong restrictions of pulmonary function and an increased risk of abnormal somatic and psychomotor development. The contributors to this disease's entity are multifactorial with pre- and postnatal origin. Central to the pathogenesis of bronchopulmonary is the induction of a massive pulmonary inflammatory response due to mechanical ventilation and oxygen toxicity. The extent of the pro-inflammatory reaction and the disturbance of further alveolar growth and vasculogenesis vary largely and can be modified by prenatal infections, antenatal steroids, and surfactant application.This minireview summarizes the important recent research findings on the pulmonary inflammatory reaction obtained in patient cohorts and in experimental models. Unfortunately, recent changes in clinical practice based on these findings had only limited impact on the incidence of bronchopulmonary dysplasia.

  15. POSTNATAL INFLAMMATION IN THE PATHOGENESIS OF BRONCHOPULMONARY DYSPLASIA

    Science.gov (United States)

    Bhandari, Vineet

    2014-01-01

    Exposure to hyperoxia, invasive mechanical ventilation and systemic/local sepsis are important antecedents of postnatal inflammation in the pathogenesis of bronchopulmonary dysplasia (BPD). This review will summarize information obtained from animal (baboon, lamb/sheep, rat and mouse) models that pertain to the specific inflammatory agents and signaling molecules that predispose a premature infant to BPD. PMID:24578018

  16. Speech and Language Outcomes of Children with Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Lewis, Barbara A.; Singer, Lynn T.; Fulton, Sarah; Salvator, Ann; Short, Elizabeth J.; Klein, Nancy; Baley, Jill

    2002-01-01

    A study of very low birth weight babies with (n=89) and without (n=71) bronchopulmonary dysplasia (BPD) and term controls was conducted at age 8. The BPD group demonstrated reduced articulation, receptive language skills, performance IQ, and gross and fine motor skills, and almost half were enrolled in speech-language therapy. (Contains…

  17. Roentgenographic aspects of idiopathic respiratory distress syndrome and bronchopulmonary dysplasia

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    Arlart, I.P.

    1982-03-01

    The present article gives a review about roentgenographic findings of the chest in indiopathic respiratory distress syndrome of the newborn and in bronchopulmonary dysplasia. In addition to etiological factors particularly a systematic of typical signs in both diseases and possibility of complications and differential diagnosis is presented.

  18. Echocardiographic evaluation of right ventricular function in preterm infants with bronchopulmonary dysplasia.

    Science.gov (United States)

    Bokiniec, Renata; Własienko, Paweł; Borszewska-Kornacka, Maria; Szymkiewicz-Dangel, Joanna

    2017-04-01

    To evaluate right ventricular function in preterm infants with and without bronchopulmonary dysplasia. Eighty-nine preterm infants (bronchopulmonary dysplasia (n=32); (2) mild-bronchopulmonary dysplasia (n=35); (3) severe-bronchopulmonary dysplasia (n=15). Right ventricular echocardiographic parameters included the following: (1) pulsed-wave Doppler through the tricuspid valve (E/A ratio), pulmonary artery acceleration time, right ventricular ejection time, right ventricular velocity-time integral; (2) tissue Doppler measurements of myocardial velocities and atrioventricular conduction times; (3) pulsed-wave Doppler and tissue Doppler evaluation of myocardial performance index and E/E' ratio; and (4) M-mode detection of right ventricular end-diastolic wall diameter. The severe-bronchopulmonary dysplasia group had higher mean right ventricular myocardial performance index (on the 28th day of life by pulsed-wave Doppler) than the no-bronchopulmonary dysplasia (P=.014) or mild-bronchopulmonary dysplasia (P=.031) groups; no differences were found between no-bronchopulmonary dysplasia and mild-bronchopulmonary dysplasia groups (P=.919). A reduction in right ventricular myocardial performance index at later time points was observed in all three groups (Pbronchopulmonary dysplasia severity in other right ventricular echocardiographic parameters. Right ventricular myocardial performance index measured by pulsed-wave Doppler indicates impaired right ventricular function in preterm infants with severe bronchopulmonary dysplasia. © 2017, Wiley Periodicals, Inc.

  19. Reviewing the use of corticosteroids in bronchopulmonary dysplasia

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    Fernanda Aparecida de Oliveira Peixoto

    2016-04-01

    Full Text Available Abstract Objective: Review the risks and benefits of postnatal corticosteroid use for the treatment of bronchopulmonary dysplasia, considering that there is not a more effective therapy. Data sources: The literature review was carried out in the BIREME database, using the terms "bronchopulmonary dysplasia and corticosteroid" in the LILACS, IBECS, MEDLINE, Cochrane Library, and SciELO databases, selecting the most relevant articles on the subject, with emphasis on recent literature published in the last five years. Summary of the data: In preterm infants, bronchopulmonary dysplasia is still a common problem and remains without a specific therapy, despite knowledge of the several risk factors. The treatment essentially consists of supportive measures, but in the past, corticosteroids were widely used, as they are the only medications that have an impact on disease progression. However, the emergence of cerebral palsy associated with the indiscriminate use of corticosteroids has prevented the prescription of this drug in the last 15 years. Since then, no new measures have been taken, and the incidence of the disease tended to increase during this period, creating the need for a review of corticosteroid use and, possibly, more restricted indications. Conclusions: The association between risks and benefits of corticosteroid use in preterm infants needs to be considered due to the fact that some infant subpopulations may show more benefits than risks, such as those using mechanical ventilation with difficult weaning.

  20. Reviewing the use of corticosteroids in bronchopulmonary dysplasia.

    Science.gov (United States)

    de Oliveira Peixoto, Fernanda Aparecida; Costa, Paulo Sérgio Sucasas

    2016-01-01

    Review the risks and benefits of postnatal corticosteroid use for the treatment of bronchopulmonary dysplasia, considering that there is not a more effective therapy. The literature review was carried out in the BIREME database, using the terms "bronchopulmonary dysplasia and corticosteroid" in the LILACS, IBECS, MEDLINE, Cochrane Library, and SciELO databases, selecting the most relevant articles on the subject, with emphasis on recent literature published in the last five years. In preterm infants, bronchopulmonary dysplasia is still a common problem and remains without a specific therapy, despite knowledge of the several risk factors. The treatment essentially consists of supportive measures, but in the past, corticosteroids were widely used, as they are the only medications that have an impact on disease progression. However, the emergence of cerebral palsy associated with the indiscriminate use of corticosteroids has prevented the prescription of this drug in the last 15 years. Since then, no new measures have been taken, and the incidence of the disease tended to increase during this period, creating the need for a review of corticosteroid use and, possibly, more restricted indications. The association between risks and benefits of corticosteroid use in preterm infants needs to be considered due to the fact that some infant subpopulations may show more benefits than risks, such as those using mechanical ventilation with difficult weaning. Copyright © 2015 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

  1. Respiratory outcomes and atopy in school-age children who were preterm at birth, with and without bronchopulmonary dysplasia

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    Hercília Guimarães

    2011-01-01

    Full Text Available OBJECTIVE: To assess pulmonary function and the prevalence of atopy in school-age children who were very low birth weight as infants and to compare those who had bronchopulmonary dysplasia to those who did not. METHOD: We studied 85 (39 male and 46 female at a mean age of 84 (range, 62 to 107 months who were very low birth weight infants. Bronchopulmonary dysplasia was defined as oxygen dependency at 36 weeks gestational age. We excluded 8 patients (4 for cerebral palsy and 4 for no collaboration. Detailed perinatal and clinical data were collected. Lung function was evaluated using conventional spirometry. Atopy (assessed by the allergy skin-prick test was considered when at least one positive skin test occurred in a panel of the most common environmental allergens in the local region. Comparisons between the bronchopulmonary dysplasia and no bronchopulmonary dysplasia groups were performed using the Mann-Whitney, x2 and Fisher's exact tests. RESULTS: We compared the bronchopulmonary dysplasia (n = 13 and no bronchopulmonary dysplasia (n = 64 groups. Atopy was observed in 4 (30.8% of the bronchopulmonary dysplasia patients and in 17 (26.6% of the no bronchopulmonary dysplasia patients (p = 0.742. Two (15.4% patients with bronchopulmonary dysplasia had a family history of atopy vs. 17 (26.6% in the no bronchopulmonary dysplasia group (p = 0.5. Lung function tests showed airway obstruction in 2 (15.4% of the bronchopulmonary dysplasia patients and in 10 (15.6% of the no bronchopulmonary dysplasia patients (p = 1.0. Four (33.3% of the bronchopulmonary dysplasia patients had small airway obstruction vs. 14 (22.2% of the no bronchopulmonary dysplasia patients (p = 0.466. CONCLUSION: Our data showed no significant differences in lung function between bronchopulmonary dysplasia and no bronchopulmonary dysplasia patients at school age and no evidence of an association between atopy and bronchopulmonary dysplasia.

  2. Early Inhaled Budesonide for the Prevention of Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Bassler, Dirk; Plavka, Richard; Shinwell, Eric S; Hallman, Mikko; Jarreau, Pierre-Henri; Carnielli, Virgilio; Van den Anker, Johannes N; Meisner, Christoph; Engel, Corinna; Schwab, Matthias; Halliday, Henry L; Poets, Christian F

    2015-10-15

    Systemic glucocorticoids reduce the incidence of bronchopulmonary dysplasia among extremely preterm infants, but they may compromise brain development. The effects of inhaled glucocorticoids on outcomes in these infants are unclear. We randomly assigned 863 infants (gestational age, 23 weeks 0 days to 27 weeks 6 days) to early (within 24 hours after birth) inhaled budesonide or placebo until they no longer required oxygen and positive-pressure support or until they reached a postmenstrual age of 32 weeks 0 days. The primary outcome was death or bronchopulmonary dysplasia, confirmed by means of standardized oxygen-saturation monitoring, at a postmenstrual age of 36 weeks. A total of 175 of 437 infants assigned to budesonide for whom adequate data were available (40.0%), as compared with 194 of 419 infants assigned to placebo for whom adequate data were available (46.3%), died or had bronchopulmonary dysplasia (relative risk, stratified according to gestational age, 0.86; 95% confidence interval [CI], 0.75 to 1.00; P=0.05). The incidence of bronchopulmonary dysplasia was 27.8% in the budesonide group versus 38.0% in the placebo group (relative risk, stratified according to gestational age, 0.74; 95% CI, 0.60 to 0.91; P=0.004); death occurred in 16.9% and 13.6% of the patients, respectively (relative risk, stratified according to gestational age, 1.24; 95% CI, 0.91 to 1.69; P=0.17). The proportion of infants who required surgical closure of a patent ductus arteriosus was lower in the budesonide group than in the placebo group (relative risk, stratified according to gestational age, 0.55; 95% CI, 0.36 to 0.83; P=0.004), as was the proportion of infants who required reintubation (relative risk, stratified according to gestational age, 0.58; 95% CI, 0.35 to 0.96; P=0.03). Rates of other neonatal illnesses and adverse events were similar in the two groups. Among extremely preterm infants, the incidence of bronchopulmonary dysplasia was lower among those who received early

  3. Evaluation of left ventricular function in preterm infants with bronchopulmonary dysplasia using various echocardiographic techniques.

    Science.gov (United States)

    Bokiniec, Renata; Własienko, Paweł; Borszewska-Kornacka, Maria; Szymkiewicz-Dangel, Joanna

    2017-04-01

    Echocardiographic evaluation of left ventricular function in preterm infants with and without bronchopulmonary dysplasia. In 82 preterm infants (32 in no-bronchopulmonary-dysplasia group, 35 in mild-bronchopulmonary-dysplasia group, and 15 in severe-bronchopulmonary-dysplasia group), echocardiography was performed on the first day of life, at 28 days of life, and at 36 weeks postconceptional age. The mean E/A ratio at 36 PCA was 0.94±0.31 and 0.73±0.12 in the mild- and severe-bronchopulmonary-dysplasia groups, respectively (P=.037). The mean E'-wave velocity was 5.62±1.61 cm/s vs 4.32±1.11 cm/s at 1 day of life (P=.006) and 6.40±1.39 cm/s vs 5.34±1.37 cm/s at 28 days of life (P=.030) in the no-bronchopulmonary-dysplasia and mild-bronchopulmonary-dysplasia groups, respectively. This measure tended to be lower in the severe-bronchopulmonary-dysplasia group compared to the no-bronchopulmonary-dysplasia group (5.25±1.29 cm/s at 28 days of life; P=.081). The E/E' ratio differed between the no-bronchopulmonary-dysplasia (7.21±1.85) and mild-bronchopulmonary-dysplasia groups (9.03±2.56; P=.019) at 1 day of life. The left ventricle myocardial performance index decreased between 1 day of life and 36 postconceptional age in infants without bronchopulmonary dysplasia and those with mild bronchopulmonary dysplasia, but not in those with severe bronchopulmonary dysplasia. E/A and E/E' ratios are the most sensitive indicators of impaired left ventricle diastolic function in preterm infants with bronchopulmonary dysplasia. © 2017, Wiley Periodicals, Inc.

  4. Attenuation of miR-17∼92 Cluster in Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Rogers, Lynette K; Robbins, Mary; Dakhlallah, Duaa; Yang, Zhaogang; Lee, L James; Mikhail, Madison; Nuovo, Gerard; Pryhuber, Gloria S; McGwin, Gerald; Marsh, Clay B; Tipple, Trent E

    2015-10-01

    Bronchopulmonary dysplasia remains a significant cause of neonatal morbidity; however, the identification of novel targets to predict or prevent the development of bronchopulmonary dysplasia remains elusive. Proper microRNA (miR)-17∼92 cluster is necessary for normal lung development, and alterations in expression are reported in other pulmonary diseases. The overall hypothesis for our work is that altered miR-17∼92 cluster expression contributes to the molecular pathogenesis of bronchopulmonary dysplasia. The current studies tested the hypothesis that alterations in miR-17∼92 cluster and DNA methyltransferase expression are present in bronchopulmonary dysplasia. miR-17∼92 cluster expression, promoter methylation, and DNA methyltransferase expression were determined in autopsy lung samples obtained from premature infants who died with bronchopulmonary dysplasia, or from term/near-term infants who died from nonrespiratory causes. Expression of miR-17∼92 cluster members miR-17 and -19b was measured in plasma samples collected in the first week of life from a separate cohort of preterm infants at a second institution in whom bronchopulmonary dysplasia was diagnosed subsequently. Autopsy tissue data indicated that miR-17∼92 expression is significantly lower in bronchopulmonary dysplasia lungs and is inversely correlated with promoter methylation and DNA methyltransferase expression when compared with that of control subjects without bronchopulmonary dysplasia. Plasma sample analyses indicated that miR-17 and -19b expression was decreased in infants who subsequently developed bronchopulmonary dysplasia. Our data are the first to demonstrate altered expression of the miR-17∼92 cluster in bronchopulmonary dysplasia. The consistency between our autopsy and plasma findings further support our working hypothesis that the miR-17∼92 cluster contributes to the molecular pathogenesis of bronchopulmonary dysplasia.

  5. Antibiotic Exposure and Risk for Death or Bronchopulmonary Dysplasia in Very Low Birth Weight Infants.

    Science.gov (United States)

    Cantey, Joseph B; Huffman, Landon W; Subramanian, Abirami; Marshall, Anderson S; Ballard, A Rebecca; Lefevre, Cassandra; Sagar, Malvika; Pruszynski, Jessica E; Mallett, Lea H

    2017-02-01

    We assessed the association between antibiotic exposure in the first 2 weeks of life and development of bronchopulmonary dysplasia in a cohort of very low birth weight infants. After controlling for the severity of illness, each additional day of antibiotic therapy was associated with both an increased risk for and severity of bronchopulmonary dysplasia. Copyright © 2016 Elsevier Inc. All rights reserved.

  6. Biomarkers, Early Diagnosis, and Clinical Predictors of Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Lal, Charitharth Vivek; Ambalavanan, Namasivayam

    2015-12-01

    The pathogenesis of bronchopulmonary dysplasia (BPD) is multifactorial, and the clinical phenotype of BPD is extremely variable. Several clinical and laboratory biomarkers have been proposed for the early identification of infants at higher risk of BPD and for determination of prognosis of infants with a diagnosis of BPD. The authors review available literature on prediction tools and biomarkers of BPD, using clinical variables and biomarkers based on imaging, lung function measures, and measurements of various analytes in different body fluids that have been determined to be associated with BPD either in a targeted manner or by unbiased omic profiling. Copyright © 2015 Elsevier Inc. All rights reserved.

  7. Genetic Determination of Bronchopulmonary Dysplasia Formation: Pros and Cons

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    V. K. Pozharishchenskaya

    2017-01-01

    Full Text Available Currently, researches are being actively carried out to identify genetic risk factors for the development of bronchopulmonary dysplasia (BPD in premature infants, including genetic polymorphism encoding surfactants, matrix metalloproteinases, cytokines, growth factors, and components of the body’s antioxidant defence. The review presents the results of foreign and domestic genetic trials in this field aimed at predicting the possible formation of BLD in premature infants and providing a personalized approach to the management of such patients.

  8. Pulmonary Hypertension and Vascular Abnormalities in Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Mourani, Peter M; Abman, Steven H

    2015-12-01

    Despite advances in the care of preterm infants, these infants remain at risk bronchopulmonary dysplasia (BPD), which results in prolonged need for supplemental oxygen, recurrent respiratory exacerbations, and exercise intolerance. Recent investigations have highlighted the important contribution of the developing pulmonary circulation to lung development, showing that these infants are also at risk for pulmonary vascular disease (PVD), including pulmonary hypertension (PH) and pulmonary vascular abnormalities. Several epidemiologic studies have delineated the incidence of PH in preterm infants and the impact on outcomes. These studies have also highlighted gaps in the understanding of PVD in BPD. Copyright © 2015 Elsevier Inc. All rights reserved.

  9. Cognitive performance of premature infants: association between bronchopulmonary dysplasia and cognitive skills. Cross-sectional study.

    Science.gov (United States)

    de Mello, Rosane Reis; Rodrigues Reis, Ana Beatriz; da Silva, Kátia Silveira

    2017-01-01

    Children born prematurely often have worse cognitive performance than those born at term regarding skills such as memory, attention and processing speed. Bronchopulmonary dysplasia may compromise cognitive development. The aims here were: a) To describe the cognitive performance of preterm infants with very low birth weight; b) To investigate its association with bronchopul-monary dysplasia adjusted for sociodemographic, neonatal and post-neonatal factors. Cross-sectional study developed in a public tertiary-care hospital. To evaluate cognition among 112 children, we applied an intelligence scale (Wechsler scale). The average scores for children with and without bronchopulmonary dysplasia were compared across the fve domains of the scale. Associations with bronchopulmonary dysplasia were investigated for domains that showed signifcant diferences between the two groups. Associations between exposure and outcome were estimated via multivariate logistic regression. There were no diferences in averages for the full-scale intelligence quotient, verbal intelligence quotient, performance intelligence quotient and general language composite domains. The processing speed quotient was the only domain that presented a signifcant diference between the two groups (P = 0.02). Among the children with bronchopulmonary dysplasia, low full-scale intelligence quotient was observed in 28.1%. In the multivariate analysis, bronchopulmonary dysplasia (odds ratio: 3.1; 95conf-dence interval: 1.1-8.7) remained associated with the outcome of processing speed quotient. Bronchopulmonary dysplasia was an independent risk factor for alteration of the processing speed quotient.

  10. Trends of urinary beta-2-microglobulin levels and bronchopulmonary dysplasia in preterm infants.

    Science.gov (United States)

    Shima, Yoshio; Kumasaka, Sakae; Nishimaki, Shigeru

    2017-08-18

    The developmental process of bronchopulmonary dysplasia is not identical between very preterm infants born small-for-gestational age and those born appropriate-for-gestational age. In this study, we compared the pattern of the inflammatory response in infants of each group, by measuring urinary beta-2-microglobulin as an alternative, concise, and less- invasive biomarker. Urinary beta-2-microglobulin levels and clinical details were examined at birth and 4 weeks of age in 146 very preterm infants. Of the 57 infants diagnosed with bronchopulmonary dysplasia, 18 were small-for-gestational age, and 39 were appropriate-for-gestational age. Urinary beta-2-microglobulin levels at birth were significantly lower in small-for-gestational age with bronchopulmonary dysplasia than in appropriate-for-gestational age with bronchopulmonary dysplasia, but they increased with time. The prevalence of chorioamnionitis was significantly lower in small-for-gestational age with bronchopulmonary dysplasia than in appropriate-for-gestational age with bronchopulmonary dysplasia, while that of pregnancy-induced hypertension was the opposite. Exposure to prenatal factors other than chorioamnionitis may sensitize fetal lungs to become vulnerable to postnatal inflammation in very preterm small-for-gestational age infants with bronchopulmonary dysplasia. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  11. Frequency of broncho-pulmonary dysplasia in the course of the respiratory distress syndrome in neonates

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    Tosch, U.; Becker-Gaab, C.; Dachs, C.

    1984-08-01

    The chest radiographs of 54 neonates with respiratory distress syndrome have been reviewed. 24% fell into stages I and II, 65% into stages III and IV and in 11% bronchopulmonary dysplasia developed. Infiltrations due to pneumonia or aspiration, persistent ductus arteriosus and pneumothorax can be differentiated radiologically. There was a strong correlation between the level of ventilatory pressure and the development of bronchopulmonary dysplasia. After reducing ventilation pressure to below 15 cm of water with a frequency of 66 per minute, bronchopulmonary dysplasia dropped from 14.4% (35 cases) to 5.3% (19 cases). 7 figs.

  12. Bronchopulmonary Dysplasia Early Changes Leading To Long Term Consequences

    Directory of Open Access Journals (Sweden)

    Anne eHilgendorff

    2015-02-01

    Full Text Available Neonatal Chronic Lung Disease, i.e. Bronchopulmonary Dysplasia (BPD is characterized by impaired pulmonary development. Triggered by different risk factors including infections, hyperoxia and mechanical ventilation of the immature lung, remodeling of the extracellular matrix, apoptosis as well as altered growth factor signaling characterize the disease. The immediate consequences have been studied in different animal models supported by in vitro approaches leading to the successful application of these findings to the clinical setting in the past. Nonetheless, existing information about long-term consequences of the identified early and most likely sustained changes to the developing lung is limited. Interesting results point towards a tremendous impact on the pulmonary repair capacity as well as aging related processes in the adult lung.

  13. Current Pharmacologic Approaches for Prevention and Treatment of Bronchopulmonary Dysplasia

    Directory of Open Access Journals (Sweden)

    Kristen Tropea

    2012-01-01

    Full Text Available Bronchopulmonary dysplasia (BPD is a major complication of preterm birth and has serious adverse long-term health consequences. The etiology of BPD is complex, multifactorial, and incompletely understood. Contributing factors include ventilator-induced lung injury, exposure to toxic oxygen levels, and infection. Several preventive and therapeutic strategies have been developed with variable success. These include lung protective ventilator strategies and pharmacological and nutritional interventions. These strategies target different components and stages of the disease process and they are commonly used in combination. The purpose of this review is to discuss the evidence for current pharmacological interventions and identify future therapeutic modalities that appear promising in the prevention and management of BPD. Continued improved understanding of BPD pathogenesis leads to opportunities for newer preventive approaches. These will need to be evaluated in the setting of current clinical practice in order to assess their efficacy.

  14. Pulmonary Hypertension in Preterm Infants with Bronchopulmonary Dysplasia

    Science.gov (United States)

    Abman, Steven H.; Mourani, Peter M.

    2014-01-01

    Bronchopulmonary dysplasia (BPD), the chronic lung disease of prematurity, is a significant contributor to perinatal morbidity and mortality. Premature birth disrupts pulmonary vascular growth and initiates a cascade of events that result in impaired gas exchange, abnormal vasoreactivity, and pulmonary vascular remodeling that may ultimately lead to pulmonary hypertension (PH). Even infants who appear to have mild BPD suffer from varying degrees of pulmonary vascular disease (PVD). Although recent studies have enhanced our understanding of the pathobiology of PVD and PH in BPD, much remains unknown with respect to how PH should be properly defined, as well as the most accurate methods for the diagnosis and treatment of PH in infants with BPD. This article will provide neonatologists and primary care providers, as well as pediatric cardiologists and pulmonologists, with a review of the pathophysiology of PH in preterm infants with BPD and a summary of current clinical recommendations for managing PH in this population. PMID:24669351

  15. Advances in paediatric pulmonary vascular disease associated with bronchopulmonary dysplasia.

    Science.gov (United States)

    Rossor, Thomas; Greenough, Anne

    2015-02-01

    Pulmonary hypertension (PH) is a common finding in infants with bronchopulmonary dysplasia (BPD). The aim of this review is to describe recent advances in the diagnosis and treatment of PH and discuss whether they will benefit infants and children with BPD related PH. Echocardiography remains the mainstay of diagnosis but has limitations, further developments in diagnostic techniques and identification of biomarkers are required. There are many potential therapies for PH associated with BPD. Inhaled nitric oxide has been shown to improve short term outcomes only. Sidenafil in resource limited settings was shown in three randomized trials to significantly reduce mortality. The efficacy of other therapies including prostacyclin, PDE3 inhibitors and endothelin receptor blockers has only been reported in case reports or case series. Randomized controlled trials with long term follow up are required to appropriately assess the efficacy of therapies aimed at improving the outcome of children with PH.

  16. Bronchopulmonary Dysplasia Early Changes Leading to Long-Term Consequences

    Science.gov (United States)

    Hilgendorff, Anne; O’Reilly, Michael A.

    2015-01-01

    Neonatal chronic lung disease, i.e., bronchopulmonary dysplasia, is characterized by impaired pulmonary development resulting from the impact of different risk factors including infections, hyperoxia, and mechanical ventilation on the immature lung. Remodeling of the extracellular matrix, apoptosis as well as altered growth factor signaling characterize the disease. The immediate consequences of these early insults have been studied in different animal models supported by results from in vitro approaches leading to the successful application of some findings to the clinical setting in the past. Nonetheless, existing information about long-term consequences of the identified early and most likely sustained changes to the developing lung is limited. Interesting results point towards a tremendous impact of these early injuries on the pulmonary repair capacity as well as aging related processes in the adult lung. PMID:25729750

  17. Course of bronchopulmonary dysplasia. A radiographic follow-up

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    Mortensson, W.; Lindroth, M.

    Forty-one low weight premature infants treated with intermittent positive pressure ventilation in infancy were followed clinically and with chest radiography for 4 to 6 years. One child died during the period (sudden infantile death) and 2 others were not available for follow-up examination. The abnormal chest pattern of bronchopulmonary dysplasia (BPD) resolved completely or improved during the period; residual changes were found in 34 per cent of the cases. The main part of the resolution occurred during the first 2 years. Mild BPD was more prone to heal. The persisting parenchymal changes - interstitial fibrosis or areas of hyperinflation or both - were generally slight. The frequency of infection of the lower respiratory tract was increased during the first 2 years of life and was positively correlated to the severity of the pulmonary abnormalities. The frequency of infection dramatically decreased during the subsequent 2 years.

  18. Late (>= 7 days) inhalation corticosteroids to reduce bronchopulmonary dysplasia in preterm infants

    NARCIS (Netherlands)

    Onland, Wes; Offringa, Martin; van Kaam, Anton

    2012-01-01

    Background Bronchopulmonary dysplasia (BPD), defined as oxygen dependence at 36 weeks postmenstrual age (PMA), remains an important complication of prematurity. Pulmonary inflammation plays a central role in the pathogenesis of BPD. Attenuating pulmonary inflammation with postnatal systemic

  19. Development of Sucking Patterns in Pre-Term Infants with Bronchopulmonary Dysplasia

    NARCIS (Netherlands)

    da Costa, Saakje P.; van der Schans, Cees P.; Zweens, Mar J.; Boelema, Sarai R.; van der Meij, Eva; Boerman, Mieke A.; Bos, Arend F.

    2010-01-01

    Background: Pre-term infants with bronchopulmonary dysplasia (BPD) are at risk of acquiring brain abnormalities. Combined with ongoing breathing difficulties, this may influence the development of their sucking patterns. Objective: To determine the longitudinal development of sucking patterns from

  20. HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY AS A SIDE-EFFECT OF DEXAMETHASONE TREATMENT FOR BRONCHOPULMONARY DYSPLASIA

    NARCIS (Netherlands)

    BRAND, PLP; VANLINGEN, RA; BRUS, F; TALSMA, MD; ELZENGA, NJ

    1993-01-01

    We report three infants who developed hypertrophic obstructive cardiomyopathy during dexamethasone treatment for bronchopulmonary dysplasia. In all three infants, echocardiography had ruled out cardiac abnormalities prior to the dexamethasone course. The hypertrophic obstructive cardiomyopathy

  1. Surfactant phosphatidylcholine half-life and pool size measurements in premature baboons developing bronchopulmonary dysplasia

    NARCIS (Netherlands)

    D.J. Janssen; V.P. Carnielli (Virgilio); P.E. Cogo (Paola); S.R. Seidner; I.H.I. Luijendijk; J.L.D. Wattimena (Josias); A.H. Jobe (Alan); L.J.I. Zimmermann (Luc)

    2002-01-01

    textabstractBecause minimal information is available about surfactant metabolism in bronchopulmonary dysplasia, we measured half-lives and pool sizes of surfactant phosphatidylcholine in very preterm baboons recovering from respiratory distress syndrome and developing

  2. Postnatal corticosteroids to prevent or treat bronchopulmonary dysplasia - Who might benefit?

    Science.gov (United States)

    Doyle, Lex W; Cheong, Jeanie L Y

    2017-10-01

    Newborn infants born very preterm are at high risk of developing bronchopulmonary dysplasia, which is associated with not only mortality but also adverse long-term neurological and respiratory outcomes in survivors. Postnatal corticosteroids might reduce the risk of developing bronchopulmonary dysplasia, or reduce its severity. However, it is important to minimize exposure to the potentially harmful effects of corticosteroids, particularly on the developing brain. Systemic corticosteroids started after the first week of life have shown the most benefit in infants at highest risk of developing bronchopulmonary dysplasia, whereas inhaled corticosteroids have little effect in children with established lung disease. Systemic corticosteroids in the first week of life are not recommended, but inhaled corticosteroids, or corticosteroids instilled into the trachea using surfactant as a vehicle to distribute the corticosteroids through the lungs, offer promise with respect to prevention of bronchopulmonary dysplasia. Copyright © 2017 Elsevier Ltd. All rights reserved.

  3. Bombesin-like peptide mediates lung injury in a baboon model of bronchopulmonary dysplasia

    NARCIS (Netherlands)

    Sunday, ME; Yoder, BA; Cuttitta, F; Haley, KJ; Emanuel, RL

    1998-01-01

    The etiology of bronchopulmonary dysplasia (BPD), a chronic lung disease of infants surviving respiratory distress syndrome, remains fundamentally enigmatic. BPD is decreasing in severity but continues to be a major problem in pediatric medicine, being especially prevalent among very premature

  4. INFANTILE CHOREA IN AN INFANT WITH SEVERE BRONCHOPULMONARY DYSPLASIA - AN EMG STUDY

    NARCIS (Netherlands)

    HADDERSALGRA, M; BOS, AF; MARTIJN, A; PRECHTL, HFR

    Recently a new movement disorder has been described which develops in some preterm infants with bronchopulmonary dysplasia. The present report provides a detailed description (including polyelectromyographical and serial cranial ultrasound findings) of this syndrome in a single infant. The authors

  5. Oxygen weaning after hospital discharge in children with bronchopulmonary dysplasia.

    Science.gov (United States)

    Yeh, Jennifer; McGrath-Morrow, Sharon A; Collaco, Joseph M

    2016-11-01

    In the United States, approximately 12,000 preterm infants are diagnosed with bronchopulmonary dysplasia (BPD) and many of these infants require supplemental oxygen after initial hospital discharge. In children with BPD we sought to identify factors associated with supplemental oxygen use after initial hospital discharge, factors associated with duration of supplemental oxygen use, and methods used to wean off supplemental oxygen in the home environment. All subjects (n = 420) with the diagnosis of BPD were recruited from a single center Bronchopulmonary Dysplasia Clinic between 2008 and 2013. Subject information was obtained from patient history records, patient demographics, and caregiver questionnaires. Younger gestational age and having a Nissen fundoplication were associated with home supplemental oxygen use in subjects with BPD. Of the 154 subjects who received supplemental oxygen at home, 38% received flows ≤1/8 LPM, 30% received flows >1/8 LPM and ≤1/4 LPM, 21% received flows >1/4 LPM and ≤1/2 LPM, and 11% received flows >1/2 LPM. Among subjects receiving ≤1/8 LPM of oxygen, the median age of weaning off oxygen was 10.1 months, but increased depending on level of oxygen flow at initial outpatient visit. Of the 137 subjects weaned off of oxygen during the study period, weaning was not supervised by a physician in 32.1% of subjects. Home supplemental oxygen use is common in infants diagnosed with BPD. In this study, the median age of weaning off supplemental oxygen was 10.1 months after initial hospital discharge. Unsupervised weaning of supplemental oxygen occurred in 32.1% of subjects with BPD. Pediatr Pulmonol. 2016;51:1206-1211. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  6. Comparisons and Limitations of Current Definitions of Bronchopulmonary Dysplasia for the Prematurity and Respiratory Outcomes Program.

    Science.gov (United States)

    Poindexter, Brenda B; Feng, Rui; Schmidt, Barbara; Aschner, Judy L; Ballard, Roberta A; Hamvas, Aaron; Reynolds, Anne Marie; Shaw, Pamela A; Jobe, Alan H

    2015-12-01

    Bronchopulmonary dysplasia is the most common morbidity of prematurity, but the validity and utility of commonly used definitions have been questioned. To compare three commonly used definitions of bronchopulmonary dysplasia in a contemporary prospective, multicenter observational cohort of extremely preterm infants. At 36 weeks postmenstrual age, the following definitions of bronchopulmonary dysplasia were applied to surviving infants with and without imputation: need for supplemental oxygen (Shennan definition), National Institutes of Health Workshop definition, and "physiologic" definition after a room-air challenge. Of 765 survivors assessed at 36 weeks, bronchopulmonary dysplasia was diagnosed in 40.8, 58.6, and 32.0% of infants, respectively, with the Shennan, workshop and physiologic definitions. The number of unclassified infants was lowest with the workshop definition (2.1%) and highest with the physiologic definition (16.1%). After assigning infants discharged home in room air before 36 weeks as no bronchopulmonary dysplasia, the modified Shennan definition compared favorably to the workshop definition, with 2.9% unclassified infants. Newer management strategies with nasal cannula flows up to 4 L/min or more and 0.21 FiO2 at 36 weeks obscured classification of bronchopulmonary dysplasia status in 12.4% of infants. Existing definitions of bronchopulmonary dysplasia differ with respect to ease of data collection and number of unclassifiable cases. Contemporary changes in management of infants, such as use of high-flow nasal cannula, limit application of existing definitions and may result in misclassification. A contemporary definition of bronchopulmonary dysplasia that correlates with respiratory morbidity in childhood is needed. Clinical trial registered with www.clinicaltrials.gov (NCT01435187).

  7. INHALANT GLUCOCORTICOID APPLICATION EXPERIENCE AMONG INFANTS WITH BRONCHOPULMONARY DYSPLASIA IN THE FIRST HALF YEAR OF LIFE

    Directory of Open Access Journals (Sweden)

    I.V. Davydova

    2008-01-01

    Full Text Available The article describes the modern views on the application of the inhalant glucocorticoids (budesonide suspension among infants, who underwent artificial pulmonary ventilation at the neonatal stage and who had bronchopulmonary dysplasia formed. The authors performed the clinical and functional evaluation of their state owing to the bronchophonography along with the follow up observation until 6 month of life.Key words: artificial pulmonary ventilation, bronchopulmonary dysplasia, extremely low body weight, bronchophonography, acoustic work of breathing.

  8. [PREDICTORS OF FORMATION OF NEW FORMS--OF BRONCHOPULMONARY DYSPLASIA AT THE PRENATAL STAGE].

    Science.gov (United States)

    Loginova, O L; Senatorova, G S

    2014-01-01

    The analysis of history in 116 premature infants, of which 74 children formed a new form of bronchopulmionary dysplasia. The analysis revealed that predictors of the formation of new forms of bronchopulmonary dysplasia are the pregnant woman, accompanied by hypoxia (jeopardy throughout pregnancy, fetoplacental insufficiency, hypotension, pregnancy, anemia) or infectious inflammation (presence of IgG to a pregnant ureaplasma, chorioamnionitis, polyhydramnios).

  9. The relationship between eosinophilia and bronchopulmonary dysplasia in premature infants at less than 34 weeks' gestation.

    Science.gov (United States)

    Yang, Joo Yun; Cha, Jihei; Shim, So-Yeon; Cho, Su Jin; Park, Eun Ae

    2014-04-01

    Eosinophilia is common in premature infants, and its incidence increases with a shorter gestation period. We investigated the clinical significance of eosinophilia in premature infants born at 3% of the total leukocytes. Perinatal parameters and clinical parameters were also analyzed. Of the 261 infants born at bronchopulmonary dysplasia, nephrocalcinosis, intraventricular hemorrhage, and sepsis were associated with a higher eosinophil percentage. The eosinophil percentage was significantly higher in infants with bronchopulmonary dysplasia from the first postnatal week and the percentage was the highest in the fourth postnatal week, with the maximal difference being 4.1% (Pbronchopulmonary dysplasia patients from the first postnatal week. Severe eosinophilia was significantly associated with the incidence of bronchopulmonary dysplasia even after adjusting for other variables.

  10. Nutrition of preterm infants in relation to bronchopulmonary dysplasia

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    Tschirch Edda

    2011-02-01

    Full Text Available Background The pathogenesis of bronchopulmonary dysplasia (BPD is multifactorial. In addition to prenatal inflammation, postnatal malnutrition also affects lung development. Methods A retrospective study was performed to analyse during the first two weeks of life the total, enteral and parenteral nutrition of premature infants ( Results Ninety-five premature infants were analysed: 26 with BPD (27 ± 1 weeks and 69 without BPD (28 ± 1 weeks. There was no statistical significant difference in the total intake of fluids, calories, glucose or protein and weight gain per day in both groups. The risk of developing BPD was slightly increased in infants with cumulative caloric intake below the minimal requirement of 1230 kcal/kg and a cumulative protein intake below 43.5 g/kg. Furthermore, the risk of developing BPD was significantly higher when infants had a cumulative fluid intake above the recommended 1840 ml/kg. In infants who developed BPD, the enteral nutrition was significantly lower than in non-BPD infants [456 ml/kg (IQR 744, 235 vs. 685 (IQR 987, 511]. Infants who did not develop BPD reached 50% of total enteral feeding significantly faster [9.6 days vs. 11.5]. Conclusions Preterm infants developing BPD received less enteral feeding, even though it was well compensated by the parenteral nutrient supply. Data suggest that a critical minimal amount of enteral feeding is required to prevent development of BPD; however, a large prospective clinical study is needed to prove this assumption.

  11. Executive functioning deficits in young adult survivors of bronchopulmonary dysplasia.

    Science.gov (United States)

    Gough, Aisling; Linden, Mark A; Spence, Dale; Halliday, Henry L; Patterson, Christopher C; McGarvey, Lorcan

    2015-01-01

    To assess long-term impairments of executive functioning in adult survivors of bronchopulmonary dysplasia (BPD). Participants were assessed on measures of executive functioning, health-related quality of life (HRQoL) and social functioning. Survivors of BPD (n = 63; 34 males; mean age 24.2 years) were compared with groups comprising preterm (without BPD) (executive functioning relating to problem solving (OR: 5.1, CI: 1.4-19.3), awareness of behavior (OR: 12.7, CI: 1.5-106.4) and organization of their environment (OR: 13.0, CI: 1.6-107.1). Birth weight, HRQoL and social functioning were predictive of deficits in executive functioning. This study represents the largest sample of survivors into adulthood of BPD and is the first to show that deficits in executive functioning persist. Children with BPD should be assessed to identify cognitive impairments and allow early intervention aimed at ameliorating their effects. Implications for Rehabilitation Adults born preterm with very-low birth weight, and particularly those who develop BPD, are at increased risk of exhibiting defects in executive functioning. Clinicians and educators should be made aware of the impact that BPD can have on the long-term development of executive functions. Children and young adults identified as having BPD should be periodically monitored to identify the need for possible intervention.

  12. [Guidelines for the follow up of patients with bronchopulmonary dysplasia].

    Science.gov (United States)

    Pérez Tarazona, S; Rueda Esteban, S; Alfonso Diego, J; Barrio Gómez de Agüero, M I; Callejón Callejón, A; Cortell Aznar, I; de la Serna Blázquez, O; Domingo Miró, X; García García, M L; García Hernández, G; Luna Paredes, C; Mesa Medina, O; Moreno Galdó, A; Moreno Requena, L; Pérez Pérez, G; Salcedo Posadas, A; Sánchez Solís de Querol, M; Torrent Vernetta, A; Valdesoiro Navarrete, L; Vilella Sabaté, M

    2016-01-01

    Bronchopulmonary dysplasia (BPD) is the most common complication of preterm birth, and remains a major problem in pediatric pulmonology units. The decision of discharging from the Neonatal Unit should be based on a thorough assessment of the condition of the patient and compliance with certain requirements, including respiratory and nutritional stability, and caregiver education on disease management. For proper control of the disease, a schedule of visits and complementary tests should be established prior to discharge, and guidelines for prevention of exacerbations and appropriate treatment should be applied. In this paper, the Working Group in Perinatal Respiratory Diseases of the Spanish Society of Pediatric Pulmonology proposes a protocol to serve as a reference for the follow up of patients with BPD among different centers and health care settings. Key factors to consider when planning discharge from the Neonatal Unit and during follow up are reviewed. Recommendations on treatment and prevention of complications are then discussed. The final section of this guide aims to provide a specific schedule for follow-up and diagnostic interventions to be performed in patients with BPD. Copyright © 2015 Asociación Española de Pediatría. Published by Elsevier España, S.L.U. All rights reserved.

  13. The extracellular matrix in bronchopulmonary dysplasia: target and source

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    Ivana eMižíková

    2015-12-01

    Full Text Available Bronchopulmonary dysplasia (BPD is a common complication of preterm birth that contributes significantly to morbidity and mortality in neonatal intensive care units. BPD results from life saving interventions such as mechanical ventilation and oxygen supplementation used to manage preterm infants with acute respiratory failure, which may be complicated by pulmonary infection. The pathogenic pathways driving BPD are not well delineated, but include disturbances to the coordinated action of gene expression, cell cell communication, physical forces, and cell interactions with the extracellular matrix (ECM, which together guide normal lung development. Efforts to further delineate these pathways have been assisted by the use of animal models of BPD which rely on infection, injurious mechanical ventilation, or oxygen supplementation, where histopathological features of BPD can be mimicked. Notable amongst these are perturbations to ECM structures, namely the organization of the elastin and collagen networks in the developing lung. Dysregulated collagen deposition and disturbed elastin fiber organization are pathological hallmarks of clinical and experimental BPD. Strides have been made in understanding the disturbances to ECM production in the developing lung, but much still remains to be discovered about how ECM maturation and turnover are dysregulated in aberrantly developing lungs. This review aims to inform the reader about the state-of-the-art concerning the ECM in BPD, to highlight the gaps in our knowledge and current controversies, and to suggest directions for future work in this exciting and complex area of lung development (pathobiology.

  14. Reliability of CXR for the diagnosis of bronchopulmonary dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Moya, M.P.; Auten, R.L. Jr. [Duke University Medical Center, Dept. of Pediatrics, Durham, NC (United States); Bisset, G.S. III; Miller, C.; Hollingworth, C.; Frush, D.P. [Dept. of Radiology, Duke Univ. Medical Center, Durham, NC (United States)

    2001-05-01

    Background. Bronchopulmonary dysplasia (BPD) continues to be prevalent, despite new treatment, in part because of increased survival in less mature infants. Investigations of new treatments have been hampered by a lack of universally accepted diagnostic criteria. Radiographic scoring systems have been developed to provide objective assessment of lung injury and risk for chronic lung disease. Objective. We sought to test the reliability of a recently reported system using chest radiography as the main tool for diagnosis of BPD. Materials and methods. One hundred chest radiographs, half demonstrating BPD and the other half without BPD, were analyzed by pediatric radiologists and by a neonatologist, using the Weinstein score (1-6, depending on increasing radiographic severity). The reliability of this scoring system was tested by kappa (k) statistics. Results. Reliability at the lowest threshold (dividing score 1 from score {>=} 2) was unacceptably low in this population. Reliability increased with inclusion of higher BPD scores in the comparison groups: 1-3 versus 4-6. Conclusion. Using the chest radiograph for the prediction of BPD is not reliable between different observers except at the two extremes of the disease. (orig.)

  15. Aberrant Pulmonary Vascular Growth and Remodeling in Bronchopulmonary Dysplasia

    Science.gov (United States)

    Alvira, Cristina M.

    2016-01-01

    In contrast to many other organs, a significant portion of lung development occurs after birth during alveolarization, thus rendering the lung highly susceptible to injuries that may disrupt this developmental process. Premature birth heightens this susceptibility, with many premature infants developing the chronic lung disease, bronchopulmonary dysplasia (BPD), a disease characterized by arrested alveolarization. Over the past decade, tremendous progress has been made in the elucidation of mechanisms that promote postnatal lung development, including extensive data suggesting that impaired pulmonary angiogenesis contributes to the pathogenesis of BPD. Moreover, in addition to impaired vascular growth, patients with BPD also frequently demonstrate alterations in pulmonary vascular remodeling and tone, increasing the risk for persistent hypoxemia and the development of pulmonary hypertension. In this review, an overview of normal lung development will be presented, and the pathologic features of arrested development observed in BPD will be described, with a specific emphasis on the pulmonary vascular abnormalities. Key pathways that promote normal pulmonary vascular development will be reviewed, and the experimental and clinical evidence demonstrating alterations of these essential pathways in BPD summarized. PMID:27243014

  16. Biomarkers in neonatology: the new "omics" of bronchopulmonary dysplasia.

    Science.gov (United States)

    Piersigilli, Fiammetta; Bhandari, Vineet

    2016-01-01

    Bronchopulmonary dysplasia (BPD) is a complex disorder resulting from gene-environmental interactions. An improved understanding of the pathogenesis of this most common chronic lung disease in infants has been made by utilizing animal models and correlating with human data. Currently, while some (vitamin A, caffeine) pharmacotherapeutic options are being utilized to ameliorate this condition, there is still no specific or effective treatment for BPD. It would be helpful for prognostication and targeted potential novel therapeutic strategies to identify those babies accurately who are at risk for developing this disease. A reliable biomarker would have the capacity to be detected in the initial phase of the disease, to allow early interventions to avoid or minimize the detrimental effects of the disease. This review will focus on human studies performed with the "omic" techniques, specifically genomics, epigenomics, microbiomics, transciptomics, proteomics and metabolomics, and summarize the information available in the literature, as it pertains to biomarker identification for BPD. Using "omics" technologies, investigators have reported markers that have the potential to be used as biomarkers of BPD: SPOCK2, VEGF -624C > G, VEGF -460T > C, mast cells specific markers, miR-219 pathway, miR-152, -30a-3p, -133b, -206, -7, lactate, taurine, trimethylamine-N-oxide, gluconate, myoinositol and alterations in surfactant lipid profile.

  17. The Extracellular Matrix in Bronchopulmonary Dysplasia: Target and Source

    Science.gov (United States)

    Mižíková, Ivana; Morty, Rory E.

    2015-01-01

    Bronchopulmonary dysplasia (BPD) is a common complication of preterm birth that contributes significantly to morbidity and mortality in neonatal intensive care units. BPD results from life-saving interventions, such as mechanical ventilation and oxygen supplementation used to manage preterm infants with acute respiratory failure, which may be complicated by pulmonary infection. The pathogenic pathways driving BPD are not well-delineated but include disturbances to the coordinated action of gene expression, cell–cell communication, physical forces, and cell interactions with the extracellular matrix (ECM), which together guide normal lung development. Efforts to further delineate these pathways have been assisted by the use of animal models of BPD, which rely on infection, injurious mechanical ventilation, or oxygen supplementation, where histopathological features of BPD can be mimicked. Notable among these are perturbations to ECM structures, namely, the organization of the elastin and collagen networks in the developing lung. Dysregulated collagen deposition and disturbed elastin fiber organization are pathological hallmarks of clinical and experimental BPD. Strides have been made in understanding the disturbances to ECM production in the developing lung, but much still remains to be discovered about how ECM maturation and turnover are dysregulated in aberrantly developing lungs. This review aims to inform the reader about the state-of-the-art concerning the ECM in BPD, to highlight the gaps in our knowledge and current controversies, and to suggest directions for future work in this exciting and complex area of lung development (patho)biology. PMID:26779482

  18. Animal models of bronchopulmonary dysplasia. The preterm baboon models.

    Science.gov (United States)

    Yoder, Bradley A; Coalson, Jacqueline J

    2014-12-15

    Much of the progress in improved neonatal care, particularly management of underdeveloped preterm lungs, has been aided by investigations of multiple animal models, including the neonatal baboon (Papio species). In this article we highlight how the preterm baboon model at both 140 and 125 days gestation (term equivalent 185 days) has advanced our understanding and management of the immature human infant with neonatal lung disease. Not only is the 125-day baboon model extremely relevant to the condition of bronchopulmonary dysplasia but there are also critical neurodevelopmental and other end-organ pathological features associated with this model not fully discussed in this limited forum. We also describe efforts to incorporate perinatal infection into these preterm models, both fetal and neonatal, and particularly associated with Ureaplasma/Mycoplasma organisms. Efforts to rekindle the preterm primate model for future evaluations of therapies such as stem cell replacement, early lung recruitment interventions coupled with noninvasive surfactant and high-frequency nasal ventilation, and surfactant therapy coupled with antioxidant or anti-inflammatory medications, to name a few, should be undertaken. Copyright © 2014 the American Physiological Society.

  19. Biomarkers for Bronchopulmonary Dysplasia in the Preterm Infant

    Science.gov (United States)

    Rivera, Lidys; Siddaiah, Roopa; Oji-Mmuo, Christiana; Silveyra, Gabriela R.; Silveyra, Patricia

    2016-01-01

    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. PMID:27065351

  20. S-Nitrosoglutathione Attenuates Airway Hyperresponsiveness in Murine Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Raffay, Thomas M; Dylag, Andrew M; Di Fiore, Juliann M; Smith, Laura A; Einisman, Helly J; Li, Yuejin; Lakner, Mitchell M; Khalil, Ahmad M; MacFarlane, Peter M; Martin, Richard J; Gaston, Benjamin

    2016-10-01

    Bronchopulmonary dysplasia (BPD) is characterized by lifelong obstructive lung disease and profound, refractory bronchospasm. It is observed among survivors of premature birth who have been treated with prolonged supplemental oxygen. Therapeutic options are limited. Using a neonatal mouse model of BPD, we show that hyperoxia increases activity and expression of a mediator of endogenous bronchoconstriction, S-nitrosoglutathione (GSNO) reductase. MicroRNA-342-3p, predicted in silico and shown in this study in vitro to suppress expression of GSNO reductase, was decreased in hyperoxia-exposed pups. Both pretreatment with aerosolized GSNO and inhibition of GSNO reductase attenuated airway hyperresponsiveness in vivo among juvenile and adult mice exposed to neonatal hyperoxia. Our data suggest that neonatal hyperoxia exposure causes detrimental effects on airway hyperreactivity through microRNA-342-3p-mediated upregulation of GSNO reductase expression. Furthermore, our data demonstrate that this adverse effect can be overcome by supplementing its substrate, GSNO, or by inhibiting the enzyme itself. Rates of BPD have not improved over the past two decades; nor have new therapies been developed. GSNO-based therapies are a novel treatment of the respiratory problems that patients with BPD experience. Copyright © 2016 by The Author(s).

  1. Potential Nutrients for Preventing or Treating Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Ma, Liya; Zhou, Ping; Neu, Josef; Lin, Hung-Chih

    2017-03-01

    Bronchopulmonary dysplasia (BPD) is a frequent complication occurring in extremely preterm infants. Despite recent advances in newborn medicine, the incidence of BPD does not appear to have changed markedly, and specific treatments and prevention strategies are still lacking. Nutrition plays an important role in normal lung development and maturation. Malnutrition may delay somatic growth and new alveoli development, thus aggravating pulmonary injury involved in the pathogenesis of BPD. However, few nutrients have been investigated for their potential to mitigate the pathogenesis of BPD. In this article, we reviewed the recent progress in research on potential nutrients useful for the prevention or treatment of BPD, including glutamine, cysteine and N-acetylcysteine, L-arginine and L-citrulline, long chain polyunsaturated fatty acids (LCPUFAs), inositol, selenium, and some antioxidant vitamins including vitamin A. Current evidence shows that vitamin A and LCPUFA can prevent BPD, and that L-citrulline might provide a new method to treat chronic pulmonary hypertension associated with BPD in premature infants. The effects of other nutrients on BPD prevention need to be further studied. Copyright © 2016 Elsevier Ltd. All rights reserved.

  2. Drug therapy for the prevention and treatment of bronchopulmonary dysplasia

    Science.gov (United States)

    Iyengar, Anjali; Davis, Jonathan M.

    2015-01-01

    Introduction: As more infants are surviving at younger gestational ages, bronchopulmonary dysplasia (BPD) remains as a frequent neonatal complication occurring after preterm birth. The multifactorial nature of the disease process makes BPD a challenging condition to treat. While multiple pharmacologic therapies have been investigated over the past two decades, there have been limited advances in the field. Often multiple therapies are used concurrently without clear evidence of efficacy, with potential for significant side effects from drug-drug interactions. Methods: Systematic literature review. Conclusion: Although there is physiologic rationale for the use of many of these therapies, none of them has single-handedly altered the incidence, severity, or progression of BPD. Future research should focus on developing clinically significant end-points (short and long term respiratory assessments), investigating biomarkers that accurately predict risk and progression of disease, and creating appropriate stratification models of BPD severity. Applying a multi-modal approach to the study of new and existing drugs should be the most effective way of establishing the optimal prevention and treatment regimens for BPD. PMID:25762933

  3. Animal models of bronchopulmonary dysplasia. The term mouse models

    Science.gov (United States)

    Berger, Jessica

    2014-01-01

    The etiology of bronchopulmonary dysplasia (BPD) is multifactorial, with genetics, ante- and postnatal sepsis, invasive mechanical ventilation, and exposure to hyperoxia being well described as contributing factors. Much of what is known about the pathogenesis of BPD is derived from animal models being exposed to the environmental factors noted above. This review will briefly cover the various mouse models of BPD, focusing mainly on the hyperoxia-induced lung injury models. We will also include hypoxia, hypoxia/hyperoxia, inflammation-induced, and transgenic models in room air. Attention to the stage of lung development at the timing of the initiation of the environmental insult and the duration of lung injury is critical to attempt to mimic the human disease pulmonary phenotype, both in the short term and in outcomes extending into childhood, adolescence, and adulthood. The various indexes of alveolar and vascular development as well as pulmonary function including pulmonary hypertension will be highlighted. The advantages (and limitations) of using such approaches will be discussed in the context of understanding the pathogenesis of and targeting therapeutic interventions to ameliorate human BPD. PMID:25305249

  4. Peculiarities of rickets of children who were born prematurely and suffered bronchopulmonary dysplasia

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    Yablon О.S.

    2016-05-01

    Full Text Available Objective: to establish the clinical and metabolic peculiarities of rickets of children who were born prematurely and suffered bronchopulmonary dysplasia and to evaluate the effectiveness of specific and nonspecific prevention of rickets of these children. Materials and methods. 15 infants with clinical manifestations of rickets who were born preterm (gestational age 28,87 0,56 weeks, weight 1214,0077 ,91 g and transferred to the neonatal bronchopulmonary dysplasia (first group investigated serum total calcium, the level of ionized calcium, inorganic phosphorus in serum, activity of alkaline phosphatase in serum. The results were compared with those of 25 premature babies of the same age and severity of rickets without bronchopulmonary dysplasia (second group and 10 term infants of the same age and severity of rickets (group. Results. Children with rickets and osteomalacia bronchopulmonary dysplasia symptoms prevailed, particularly kraniotabes (60.00% and availability Harissonovoyi grooves (33.33% (p<0.05, deformity of the sternum (93.33% and expanding the lower aperture (100.00% (p<0.01. The data indicate that the depth of metabolic disorders in premature babies with rickets and bronchopulmonary dysplasia fairly similar as prevailing without bronchopulmonary dysplasia of premature babies and of children who were born full-term. The children of the first group all indicators biochemistry blood were significantly lower than similar indicators in children with group comparison, the level of total calcium — 1.84±0.04 mmol/l and inorganic phosphorus — 1.44±0.02 mmol/l (p<0.01, and ionized calcium — 1.06±0.03 mmol/l. The activity of alkaline phosphatase (663.53±55.10 U/L significantly prevailing rate of term infants (p<0,05. In premature infants antenatal and postnatal prevention of rickets was broken. Conclusion. It has been established that rickets in premature infants in the background bronchopulmonary dysplasia began earlier, had mostly

  5. Bronchopulmonary dysplasia. X-ray patterns and grading according to stages

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    Ponhold, W.; Coradello, H.

    1980-12-01

    Bronchopulmonary dysplasia, first described by Northway in 1967 as a separate entity, was graded into four stages. Basing on thoracic X-ray films of 14 newborn and prematurely born infants an attempt has been made to analyse the X-ray patterns of signs characterising bronchopulmonary dysplasia and to establish a relation between the X-ray signs and the four stages. With few exceptions the various radiologic signs were seen in all stages, although with varying incidence. It follows that X-ray visualisation of the thorax yields limited pointers only to the actual stage of the disease. Roentgenography is the method of choice (in connection with the pattern of clinical signs) for diagnosing bronchopulmonary dysplasia and should be also employed for controlling the course of the disease and for the identification of eventual complications.

  6. Gene expression profiling in preterm infants: new aspects of bronchopulmonary dysplasia development.

    Science.gov (United States)

    Pietrzyk, Jacek J; Kwinta, Przemko; Wollen, Embjorg J; Bik-Multanowski, Mirosław; Madetko-Talowska, Anna; Günther, Clara-Cecilie; Jagła, Mateusz; Tomasik, Tomasz; Saugstad, Ola D

    2013-01-01

    Bronchopulmonary dysplasia is one of the most serious complications observed in premature infants. Thanks to microarray technique, expression of nearly all human genes can be reliably evaluated. To compare whole genome expression in the first month of life in groups of infants with and without bronchopulmonary dysplasia. 111 newborns were included in the study. The mean birth weight was 1029 g (SD:290), and the mean gestational age was 27.8 weeks (SD:2.5). Blood samples were drawn from the study participants on the 5th, 14th and 28th day of life. The mRNA samples were evaluated for gene expression with the use of GeneChip® Human Gene 1.0 ST microarrays. The infants were divided into two groups: bronchopulmonary dysplasia (n=68) and control (n=43). Overall 2086 genes were differentially expressed on the day 5, only 324 on the day 14 and 3498 on the day 28. Based on pathway enrichment analysis we found that the cell cycle pathway was up-regulated in the bronchopulmonary dysplasia group. The activation of this pathway does not seem to be related with the maturity of the infant. Four pathways related to inflammatory response were continuously on the 5(th), 14(th) and 28(th) day of life down-regulated in the bronchopulmonary dysplasia group. However, the expression of genes depended on both factors: immaturity and disease severity. The most significantly down-regulated pathway was the T cell receptor signaling pathway. The results of the whole genome expression study revealed alteration of the expression of nearly 10% of the genome in bronchopulmonary dysplasia patients.

  7. Refining anti-inflammatory therapy strategies for bronchopulmonary dysplasia.

    Science.gov (United States)

    Rudloff, Ina; Cho, Steven X; Bui, Christine B; McLean, Catriona; Veldman, Alex; Berger, Philip J; Nold, Marcel F; Nold-Petry, Claudia A

    2017-06-01

    Bronchopulmonary dysplasia (BPD) is a severe lung disease of preterm infants, which is characterized by fewer, enlarged alveoli and increased inflammation. BPD has grave consequences for affected infants, but no effective and safe therapy exists. We previously showed that prophylactic treatment with interleukin-1 receptor antagonist (IL-1Ra) prevents murine BPD induced by perinatal inflammation and hyperoxia. Here, we used the same BPD model to assess whether an alternative anti-inflammatory agent, protein C (PC), is as effective as IL-1Ra against BPD. We also tested whether delayed administration or a higher dose of IL-1Ra affects its ability to ameliorate BPD and investigated aspects of drug safety. Pups were reared in room air (21% O2 ) or hyperoxia (65% or 85% O2 ) and received daily injections with vehicle, 1200 IU/kg PC, 10 mg/kg IL-1Ra (early or late onset) or 100 mg/kg IL-1Ra. After 3 or 28 days, lung and brain histology were assessed and pulmonary cytokines were analysed using ELISA and cytokine arrays. We found that PC only moderately reduced the severe impact of BPD on lung structure (e.g. 18% increased alveolar number by PC versus 34% by IL-1Ra); however, PC significantly reduced IL-1β, IL-1Ra, IL-6 and macrophage inflammatory protein (MIP)-2 by up to 89%. IL-1Ra at 10 mg/kg prevented BPD more effectively than 100 mg/kg IL-1Ra, but only if treatment commenced at day 1 of life. We conclude that prophylactic low-dose IL-1Ra and PC ameliorate BPD and have potential as the first remedy for one of the most devastating diseases preterm babies face. © 2016 The Authors. Journal of Cellular and Molecular Medicine published by John Wiley & Sons Ltd and Foundation for Cellular and Molecular Medicine.

  8. Bronchopulmonary dysplasia: a longitudinal study of 23 cases.

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    Lambert, I; Matthews, T G; Clarke, T A

    1991-10-01

    Bronchopulmonary dysplasia (B.P.D.) is a condition reflecting the reaction of the immature lung to the intensive support (barotrauma from mechanical ventilation and oxygen toxicity) required for survival in critically ill newborn infants. This study examines all infants who developed B.P.D. over a 2 year period in the Rotunda Hospital. Between 1st January 1986-31st December 1987 there were 1,360 N.I.C.U. admissions, 198 with respiratory problems and 76 requiring assisted ventilation (I.P.P.V.); 23 infants developed B.P.D. with a mean gestational age of 28.7 weeks (SD 2.5), mean birth weight 1,243 g. (SD 523 g.). One infant died at 4 months from S.I.D.S. and one was lost to follow-up (both had been clinically normal). At one year post term the weight was 7,843 g. (SD 1,134) (normal population mean 9.75 Kg. third percentile 8 Kg.) and head circumference 46 cm. (SD 2.5) (normal population mean 47 cm., third percentile 45 cm). During the 1st year of life 11 infants required re-hospitalisation (5 bronchiolitis, 2 urinary tract infections, 2 failure to thrive, 2 myringotomies/grommets) and a further 8 attended hospital with respiratory infections. Only 6/21 received 3 in 1 vaccine (all in hospital O.P.D.) and 14/21 received 2 in 1 vaccine. At one year 15 infants were normal, 2 had cerebral palsy, 2 mild motor delay (one with arrested hydrocephalus), 1 sensorineural deafness and 1 arrested hydrocephalus with mild motor delay. Five infants developed retinopathy of prematurity but none required treatment.

  9. Impact of bronchopulmonary dysplasia on brain and retina

    Directory of Open Access Journals (Sweden)

    Annie Wing Hoi Poon

    2016-04-01

    Full Text Available Many premature newborns develop bronchopulmonary dysplasia (BPD, a chronic lung disease resulting from prolonged mechanical ventilation and hyperoxia. BPD survivors typically suffer long-term injuries not only to the lungs, but also to the brain and retina. However, currently it is not clear whether the brain and retinal injuries in these newborns are related only to their prematurity, or also to BPD. We investigated whether the hyperoxia known to cause histologic changes in the lungs similar to BPD in an animal model also causes brain and retinal injuries. Sprague Dawley rat pups were exposed to hyperoxia (95% O2, ‘BPD’ group or room air (21% O2, ‘control’ group from postnatal day 4–14 (P4–14; the rat pups were housed in room air between P14 and P28. At P28, they were sacrificed, and their lungs, brain, and eyes were extracted. Hematoxylin and eosin staining was performed on lung and brain sections; retinas were stained with Toluidine Blue. Hyperoxia exposure resulted in an increased mean linear intercept in the lungs (P<0.0001. This increase was associated with a decrease in some brain structures [especially the whole-brain surface (P=0.02], as well as a decrease in the thickness of the retinal layers [especially the total retina (P=0.0008], compared to the room air control group. In addition, a significant negative relationship was observed between the lung structures and the brain (r=−0.49, P=0.02 and retina (r=−0.70, P=0.0008 structures. In conclusion, hyperoxia exposure impaired lung, brain, and retina structures. More severe lung injuries correlated with more severe brain and retinal injuries. This result suggests that the same animal model of chronic neonatal hyperoxia can be used to simultaneously study lung, brain and retinal injuries related to hyperoxia.

  10. Circulating Fibrocytes Are Increased in Neonates with Bronchopulmonary Dysplasia.

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    Chun Li

    Full Text Available Bronchopulmonary dysplasia (BPD is characterized by the aberrant remodeling of the lung parenchyma, resulting from accumulation of fibroblasts or myofibroblasts. Circulating fibrocytes are implied in pulmonary fibrosis, but whether these cells are associated with the development of BPD or the progressive fibrosis is unknown. The aim of the present study was to investigate the occurrence of fibrocytes in peripheral venous blood and explore whether these cells might be associated with severity of BPD.We investigated circulating fibrocytes in 66 patients with BPD, 23 patients with acute respiratory distress syndrome(ARDS and 11 normal subjects. Circulating fibrocytes were defined and quantified as cells positive for CD45 andcollagen-1 by flow cytometry. Furthermore, serum SDF-1/CXCL12 and TGF-β1 were evaluated using ELISA methods. We also investigated the clinical value of fibrocyte counts by comparison with standard clinical parameters.The patients with BPD had significantly increased numbers of fibrocytes compared to the controls (p < 0.01. Patients with ARDS were not different from healthy control subjects. There was a correlation between the number of fibrocytes and pulmonary hypertension or oxygen saturation (p < 0.05. Fibrocyte numbers were not correlated with other clinical or functional variables or radiologic severity scores. The fibrocyte attractant chemokine CXCL12 increased in plasma (p < 0.05 and was detectable in the bronchoalveolar lavage fluid of 40% of the patients but not in controls.These findings indicate that circulating fibrocytes are increased in patients with BPD and may contribute to pulmonary fibrosis in BPD. Circulating fibrocytes, likely recruited through the CXCR4/CXCL12 axis, might contribute to the production of TGF-β1 for the expansion of fibroblast/myofibroblast population in BPD.

  11. Influence of own mother's milk on bronchopulmonary dysplasia and costs.

    Science.gov (United States)

    Patel, Aloka L; Johnson, Tricia J; Robin, Beverley; Bigger, Harold R; Buchanan, Ashley; Christian, Elizabeth; Nandhan, Vikram; Shroff, Anita; Schoeny, Michael; Engstrom, Janet L; Meier, Paula P

    2017-05-01

    Human milk from the infant's mother (own mother's milk; OMM) feedings reduces the risk of several morbidities in very low birthweight (VLBW) infants, but limited data exist regarding its impact on bronchopulmonary dysplasia (BPD). To prospectively study the impact of OMM received in the neonatal intensive care unit (NICU) on the risk of BPD and associated costs. A 5-year prospective cohort study of the impact of OMM dose on growth, morbidity and NICU costs in VLBW infants. OMM dose was the proportion of enteral intake that consisted of OMM from birth to 36 weeks postmenstrual age (PMA) or discharge, whichever occurred first. BPD was defined as the receipt of oxygen and/or positive pressure ventilation at 36 weeks PMA. NICU costs included hospital and physician costs. The cohort consisted of 254 VLBW infants with mean birth weight 1027±257 g and gestational age 27.8±2.5 weeks. Multivariable logistic regression demonstrated a 9.5% reduction in the odds of BPD for every 10% increase in OMM dose (OR 0.905 (0.824 to 0.995)). After controlling for demographic and clinical factors, BPD was associated with an increase of US$41 929 in NICU costs. Increased dose of OMM feedings from birth to 36 weeks PMA was associated with a reduction in the odds of BPD in VLBW infants. Thus, high-dose OMM feeding may be an inexpensive, effective strategy to help reduce the risk of this costly multifactorial morbidity. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  12. Bronchopulmonary dysplasia: correlation of radiographic and clinical findings

    Energy Technology Data Exchange (ETDEWEB)

    Breysem, L. [Department of Radiology, University Hospitals, K. U. Leuven, Herestraat 49, B-3000 Leuven (Belgium); Smet, M.H. [Department of Radiology, University Hospitals, K. U. Leuven, Herestraat 49, B-3000 Leuven (Belgium); Lierde, S. van [Department of Pediatrics, University Hospitals of Leuven, Leuven (Belgium); Devlieger, H. [Department of Pediatrics, University Hospitals of Leuven, Leuven (Belgium); Boeck, K. de [Department of Pediatrics, University Hospitals of Leuven, Leuven (Belgium); Marchal, G. [Department of Radiology, University Hospitals, K. U. Leuven, Herestraat 49, B-3000 Leuven (Belgium)

    1997-08-01

    Background and purpose. Abnormalities of the chest wall have been described in bronchopulmonary dysplasia (BPD). Clinical, radiographic and pulmonary function variables were evaluated in 1-year-old children ventilated because of neonatal lung disease in order to quantify these thoracic changes and to evaluate the lung disease. Methods. The pulmonary status of 51 infants with neonatal lung disease requiring artificial ventilation was reevaluated clinically and radiographically at the age of 1 year. Twenty-two of these infants had developed BPD. Thoracic depth and width were measured clinically and on chest X-ray. The Toce score evaluated the presence of cardiomegaly, hyperinflation, emphysema and interstitial lung disease. Lung function was measured after sedation using previously reported methods. In BPD patients, Toce score and lung function were determined and compared at 1 month and at 1 year of age. Results. In BPD patients, chest depth was significantly smaller when measured clinically as well as on chest radiograph (P < 0.05; Mann-Whitney U-test). There was a statistically significant correlation between chest depth measured clinically and on chest X-ray. Toce score was significantly higher in BPD patients (P < 0.05). In BPD patients intersitial abnormalities and decreased lung compliance were more frequent at the age of 1 month than at the age of 1 year. At the age of 1 year, hyperinflation was more frequent and at that time increased airway resistance was still noted. Thus the type of X-ray abnormality reflects the type of lung function disturbance. Conclusion. The flatness of the chest is most likely a consequence of the long-standing lung function abnormalities. (orig.). With 3 figs., 6 tabs.

  13. Nutrition of preterm infants with bronchopulmonary dysplasia after hospital discharge – Part II

    Directory of Open Access Journals (Sweden)

    Hercília Guimarães

    2014-01-01

    Full Text Available Preterm infants with bronchopulmonary dysplasia often present with severe growth failure at discharge from the neonatal intensive care unit. Catch-up growth accelerates after hospital discharge, nevertheless, feeding problems may need a specialized approach. Following the revision of the scientific literature on the most relevant aspects on nutrition of patients with bronchopulmonary dysplasia after hospital discharge in Part I, in this article the Authors present and discuss important issues such as catch up growth, swallow dysfunction, gastroesophageal reflux, and how to improve feeding competences.

  14. Regional Variation on Rates of Bronchopulmonary Dysplasia and Associated Risk Factors

    Science.gov (United States)

    Rojas, María Ximena; Rojas, Mario Augusto; Lozano, Juan Manuel; Rondón, Martín Alonso; Charry, Laura Patricia

    2012-01-01

    Background. An abnormally high incidence (44%) of bronchopulmonary dysplasia with variations in rates among cities was observed in Colombia among premature infants. Objective. To identify risk factors that could explain the observed high incidence and regional variations of bronchopulmonary dysplasia. Study Design. A case-control study was designed for testing the hypothesis that differences in the disease rates were not explained by differences in city-of-birth specific population characteristics or by differences in respiratory management practices in the first 7 days of life, among cities. Results. Multivariate analysis showed that premature rupture of membranes, exposure to mechanical ventilation after received nasal CPAP, no surfactant exposure, use of rescue surfactant (instead of early surfactant), PDA, sepsis and the median daily FIO2, were associated with a higher risk of dysplasia. Significant differences between cases and controls were found among cities. Models exploring for associations between city of birth and dysplasia showed that being born in the highest altitude city (Bogotá) was associated with a higher risk of dysplasia (OR 1.82 95% CI 1.31–2.53). Conclusions. Bronchopulmonary dysplasia was manly explained by traditional risk factors. Findings suggest that altitude may play an important role in the development of this disease. Prenatal steroids did not appear to be protective at high altitude. PMID:22830042

  15. Serum surfactant protein D as a marker for bronchopulmonary dysplasia.

    Science.gov (United States)

    Vinod, Suja; Gow, Andrew; Weinberger, Barry; Potak, Debra; Hiatt, Mark; Chandra, Shaku; Hegyi, Thomas

    2017-10-26

    Lung epithelial cells express surfactant protein D (SP-D), a calcium-dependent lectin that plays an important role in antibody-independent pulmonary host defense. Previous studies have shown that it is found in the peripheral circulation in patients with pulmonary disease, likely because of translocation into the blood when lung epithelial barriers are disrupted by inflammation or acute injury. In adults, serum SP-D levels are biomarkers for the progression and severity of chronic lung disease. In neonates, elevated SP-D levels in cord blood and on day 1 have been associated with prenatal risk factors and with an increased risk of respiratory distress syndrome and infections. It is not known whether serum SP-D during the first week of life is a marker for bronchopulmonary dysplasia (BPD), a form of chronic lung disease of prematurity that is associated with lung parenchymal maldevelopment and injury. The goal of this study is to determine whether serum SP-D on days 3 and 7 of life are associated with the development of BPD in preterm infants. Serum samples were obtained on postnatal days 3 and 7 from 106 preterm infants (500-2000 g birth weight, 23-32-week gestation). SP-D was quantified by Western blot. BPD was determined at 36 weeks PMA using NICHD criteria. The mean birth weight was 1145 ± 347 g and gestational age 29.2 ± 7.4 weeks. BPD was diagnosed in 7 and "BPD or death" in 16 infants. Days 3 and 7 values tracked significantly (r = 0.648), and did not correlate with birth weight or gestational age. Contrary to expectations, serum SP-D was not associated with BPD. Significant gender differences were noted, with SP-D dropping from day 3 to day 7 in males, while increasing in females (p D does not appear to be a useful marker for BPD. Decreasing serum SP-D levels in males, as compared to females, during the first week of life are likely related to gender differences in lung maturation, consistent with the higher incidence of BPD in males.

  16. Postnatal Cytomegalovirus Infection and the Risk for Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Kelly, Matthew S; Benjamin, Daniel K; Puopolo, Karen M; Laughon, Matthew M; Clark, Reese H; Mukhopadhyay, Sagori; Benjamin, Daniel K; Smith, P Brian; Permar, Sallie R

    2015-12-01

    Postnatally acquired cytomegalovirus (CMV) is typically benign in term infants but in very low-birth-weight (VLBW) infants can cause pneumonitis and sepsislike illness. Whether postnatal CMV infection results in long-term pulmonary sequelae in these infants is unknown. To investigate the association between postnatal CMV infection and bronchopulmonary dysplasia (BPD) and mortality in a large multicenter cohort of VLBW infants. Conducted between October 2014 and June 2015, this propensity-matched retrospective cohort study involved 101,111 hospitalized VLBW (<1500 g) infants at 348 neonatal intensive care units in the United States from 1997 to 2012. We matched infants with postnatal CMV infection 1:1 to comparison infants using propensity scores, and we used Poisson regression to examine the effect of postnatal CMV on the combined risk for death or BPD at 36 weeks' postmenstrual age. To describe features of postnatal CMV infection, we extracted clinical and laboratory data from 7 days before until 7 days after infants met criteria for postnatal CMV. Postnatal CMV infection was defined as a diagnosis of CMV or detection of CMV from blood, urine, cerebrospinal fluid, or respiratory secretions on or after day of life 21. Infants with a CMV diagnosis or virologic detection of CMV prior to day of life 21 were not considered to have postnatal infection. The primary outcome was death or BPD at 36 weeks' postmenstrual age. Of 101,111 infants, 328 (0.3%) had postnatal CMV infection. We matched a comparison infant to 303 CMV-infected infants (92%) for a final cohort of 606 infants. The median gestational age and birth weight of this cohort were 25 weeks and 730 g, respectively. Postnatal CMV infection was associated with an increased risk for death or BPD at 36 weeks' postmenstrual age (risk ratio, 1.21; 95% CI, 1.10-1.32) and BPD (risk ratio, 1.33; 95% CI, 1.19-1.50). Changes in cardiorespiratory status associated with postnatal CMV infection included a new requirement for

  17. Postnatal Cytomegalovirus Infection and the Risk of Bronchopulmonary Dysplasia

    Science.gov (United States)

    Kelly, Matthew S.; Benjamin, Daniel K.; Puopolo, Karen M.; Laughon, Matthew M.; Clark, Reese H.; Mukhopadhyay, Sagori; Benjamin, Daniel K.; Smith, P. Brian; Permar, Sallie R.

    2015-01-01

    Importance Postnatally acquired cytomegalovirus (CMV) is typically benign in term infants but, in very low birth weight (VLBW) infants, can cause pneumonitis and sepsis-like illness. Whether postnatal CMV infection results in long-term pulmonary sequelae in these infants is unknown. Objective To investigate the relationship between postnatal CMV infection and bronchopulmonary dysplasia (BPD) and mortality in a large, multicenter cohort of VLBW infants. Design Propensity-matched retrospective cohort study. Setting 348 neonatal intensive care units in the United States from 1997–2012. Participants Hospitalized VLBW (<1500 g) infants. Exposures Postnatal CMV infection was defined as a diagnosis of CMV or detection of CMV from blood, urine, cerebrospinal fluid, or respiratory secretions on or after day of life 21. Infants with a CMV diagnosis or virologic detection of CMV prior to day of life 21 were not considered to have postnatal infection. Main Outcomes and Measures We matched infants with postnatal CMV infection 1:1 to comparison infants using propensity scores, and used Poisson regression to examine the effect of postnatal CMV on the combined risk of death or BPD at 36 weeks postmenstrual age. To describe features of postnatal CMV infection, we extracted clinical and laboratory data from 7 days before until 7 days after infants met criteria for postnatal CMV. Results Of 101,111 infants, 328 (0.3%) had postnatal CMV infection. We matched a comparison infant to 303 (92%) CMV-infected infants for a final cohort of 606 infants. The median gestational age and birth weight of this cohort were 25 weeks and 730 g, respectively. Postnatal CMV infection was associated with an increased risk of death or BPD at 36 weeks postmenstrual age (risk ratio [RR]: 1.21, 95% confidence interval [CI]: 1.10–1.32) and BPD (RR: 1.33, 95% CI: 1.19–1.50). Changes in cardiorespiratory status associated with postnatal CMV infection included a new requirement for vasopressor medications

  18. Tracheostomy placement in infants with bronchopulmonary dysplasia: safety and outcomes.

    Science.gov (United States)

    Mandy, George; Malkar, Manish; Welty, Stephen E; Brown, Rachel; Shepherd, Edward; Gardner, William; Moise, Alicia; Gest, Alfred

    2013-03-01

    Optimizing the timing and safety for the placement of a tracheostomy in infants with bronchopulmonary dysplasia (BPD) has not been determined. The purpose of the present study was to describe the data from a single institution about the efficacy and safety of tracheostomy placement in infants with BPD needing long-term respiratory support. We established a service line for the comprehensive care of infants with BPD and we collected retrospective clinical data from this service line. We identified patients that had a trachostomy placed using the local Vermont-Oxford database, and obtained clinical data from chart reviews. We identified infants who had a tracheostomy placed for the indication of severe BPD only. Safety and respiratory efficacy was assessed by overall survival to discharge and the change in respiratory supportive care from just before placement to 1-month post-placement. Twenty-two patients (750 ± 236 g, 25.4 ± 2.1 weeks gestation) had a tracheostomy placed on day of life 177 ± 74 which coincided with a post-conceptual age of 51 ± 10 weeks. At placement these infants were on high settings to support their lung disease. The mean airway pressure (MAP) was 14.3 ± 3.3 cmH(2) O, the peak inspiratory pressure was 43.7 ± 8.0 cmH(2) O, and the FiO(2) was 0.51 ± 0.13. The mean respiratory severity score (MAP × FiO(2) ) 1 month after tracheostomy was significantly (P = 0.03) lower than prior to tracheostomy. Survival to hospital discharge was 77%. All patients with tracheostomies that survived were discharged home on mist collar supplemental oxygen. In conclusion, the high survival rate in these patients with severe BPD and the decreased respiratory support after placement of a tracheostomy suggests that high ventilatory pressures should not be a deterrent for placement of a tracheostomy. Future research should be aimed at determining optimal patient selection and timing for tracheostomy placement in infants

  19. Bronchopulmonary dysplasia: understanding of the underlying pathological mechanisms

    Directory of Open Access Journals (Sweden)

    Daniela Fanni

    2014-06-01

    Full Text Available Bronchopulmonary dysplasia (BPD is a chronic lung disease occurring in preterm infants, typically before 28 weeks of gestational age, characterized by a prolonged need for supplemental oxygen or positive pressure ventilation. The normal stages of lung development and their relation to the timing of preterm birth is strategic in order to understand the pathogenesis of BPD. In embryonic and pseudoglandular stages the lungs arise from the anterior foregut as a bud where the branching morphogenesis generate a tree-like network of airways. The canalicular stage is characterized by increasing proliferation of distal lung epithelial cells and rapid expansion of the intra-acinar capillaries. The complexity of the airways increases, secondary crests begin to form and full maturation of the alveolus occurs during the saccular and the alveolar stages. Mesechyme components, expecially elastin and myofibroblast, display a major role in normal lung development. BPD is thought to result after an acute insult to the neonatal lung following therapy with oxygen supplementation and mechanical ventilation. Chorioamnionitis, infections and genetic susceptibly are hypothesized to contribute to the injury that affect the normal human lung development. Abnormalities in the mesenchyme were consistently seen in association with inhibition of alveolarization. The pathological features that characterize BPD are complex and differ according with the disease progression. Alveolar simplification, interstitial fibrosis, septal thickness, large airways, smooth muscle hypertrophy, fetal artery persistance and decrease in the arterial number can be histologically observed. In conclusion, in order to reach a complete clinical-pathological diagnosis, the correlation of the pathological features with the fundamental steps of lung morphogenesis and a strict dialogue between the neonatologist and the perinatal pathologist are required. Given these conditions, in our experience, a

  20. Endothelin 1 as a predictor marker for bronchopulmonary dysplasia in preterm neonates with respiratory distress syndrome.

    Science.gov (United States)

    El Shemi, M S; Tawfik, S; Khafagy, S M; Hamza, M T; Youssef, A M A

    2017-01-01

    We aimed to investigate if endothelin 1 concentration at day 3 postnatal age could be used as a predictive marker for development of bronchopulmonary dysplasia in preterm neonates with respiratory distress syndrome. This prospective observational study was done on 69 preterm neonates with gestational ages between 28 and 34 weeks and diagnosed as having respiratory distress syndrome. Serum concentrations of endothelin 1 was measured for all patients at day 3 of life and they were divided into BPD and No-BPD groups according to whether they developed bronchopulmonary dysplasia or not. A total of 17 infants were in the BPD group and 52 infants were in the No-BPD group. Serum endothelin 1 was significantly higher in the BPD group (435.39±172.88) compared with the No-BPD group (302.65±49.32) (p bronchopulmonary dysplasia with a sensitivity of 88.24%, and specificity of 61.54%. Serum endothelin 1 is significantly increased at day 3 of life in preterm neonates with respiratory distress syndrome who later develop bronchopulmonary dysplasia (BPD). It seems to be a promising predictive marker for BPD but further studies are needed to find the appropriate time for its measurement.

  1. Inhaled nitric oxide for prevention of bronchopulmonary dysplasia in premature babies (EUNO) : a randomised controlled trial

    NARCIS (Netherlands)

    Mercier, Jean-Christophe; Hummler, Helmut; Durrmeyer, Xavier; Sanchez-Luna, Manuel; Carnielli, Virgilio; Field, David; Greenough, Anne; Van Overmeire, Bart; Jonsson, Baldvin; Hallman, Mikko; Baldassarre, James

    2010-01-01

    Background In animal models, inhaled nitric oxide improved gas exchange and lung structural development, but its use in premature infants at risk of developing bronchopulmonary dysplasia remains controversial. We therefore tested the hypothesis that inhaled nitric oxide at a low concentration,

  2. Calcium absorption in very low birth weight infants with and without bronchopulmonary dysplasia

    Science.gov (United States)

    Our objective was to evaluate the effects of early bronchopulmonary dysplasia (BPD) on calcium (Ca) metabolism and growth in very low birth weight (VLBW) infants. A dual-tracer, stable isotope method was used to assess Ca absorption in VLBW infants. Infants with early BPD received energy-dense feedi...

  3. Role of serine proteases in the regulation of interleukin-877 during the development of bronchopulmonary dysplasia in preterm ventilated infants.

    Science.gov (United States)

    Chakraborty, Mallinath; McGreal, Eamon P; Williams, Andrew; Davies, Philip L; Powell, Wendy; Abdulla, Salima; Voitenok, Nikolai N; Hogwood, John; Gray, Elaine; Spiller, Brad; Chambers, Rachel C; Kotecha, Sailesh

    2014-01-01

    The chemokine interleukin-8 is implicated in the development of bronchopulmonary dysplasia in preterm infants. The 77-amino acid isoform of interleukin-8 (interleukin-877) is a less potent chemoattractant than other shorter isoforms. Although interleukin-877 is abundant in the preterm circulation, its regulation in the preterm lung is unknown. To study expression and processing of pulmonary interleukin-877 in preterm infants who did and did not develop bronchopulmonary dysplasia. Total interleukin-8 and interleukin-877 were measured in bronchoalveolar lavage fluid from preterm infants by immunoassay. Neutrophil serine proteases were used to assess processing. Neutrophil chemotaxis assays and degranulation of neutrophil matrix metalloproteinase-9 were used to assess interleukin-8 function. Peak total interleukin-8 and interleukin-877 concentrations were increased in infants who developed bronchopulmonary dysplasia compared to those who did not. Shorter forms of interleukin-8 predominated in the preterm lung (96.3% No-bronchopulmonary dysplasia vs 97.1% bronchopulmonary dysplasia, p>0.05). Preterm bronchoalveolar lavage fluid significantly converted exogenously added interleukin-877 to shorter isoforms (pbronchopulmonary dysplasia infants (pbronchopulmonary dysplasia, due to conversion of interleukin-877 by neutrophil serine proteases and thrombin. Processing of interleukin-8 provides an attractive therapeutic target to prevent development of bronchopulmonary dysplasia.

  4. Functional genetic variation in NFKBIA and susceptibility to childhood asthma, bronchiolitis, and bronchopulmonary dysplasia.

    Science.gov (United States)

    Ali, Salman; Hirschfeld, Aaron F; Mayer, Matthew L; Fortuno, Edgardo S; Corbett, Nathan; Kaplan, Maia; Wang, Shirley; Schneiderman, Julia; Fjell, Christopher D; Yan, Jin; Akhabir, Loubna; Aminuddin, Farzian; Marr, Nico; Lacaze-Masmonteil, Thierry; Hegele, Richard G; Becker, Allan; Chan-Yeung, Moira; Hancock, Robert E W; Kollmann, Tobias R; Daley, Denise; Sandford, Andrew J; Lavoie, Pascal M; Turvey, Stuart E

    2013-04-15

    Respiratory diseases are the most frequent chronic illnesses in babies and children. Although a vigorous innate immune system is critical for maintaining lung health, a balanced response is essential to minimize damaging inflammation. We investigated the functional and clinical impact of human genetic variants in the promoter of NFKBIA, which encodes IκBα, the major negative regulator of NF-κB. In this study, we quantified the functional impact of NFKBIA promoter polymorphisms (rs3138053, rs2233406, and rs2233409) on promoter-driven protein expression, allele-specific and total NFKBIA mRNA expression, IκBα protein expression, and TLR responsiveness; mapped innate immune regulatory networks active during respiratory syncytial virus infection, asthma, and bronchopulmonary dysplasia; and genotyped and analyzed independent cohorts of children with respiratory syncytial virus infection, asthma, and bronchopulmonary dysplasia. Genetic variants in the promoter of NFKBIA influenced NFKBIA gene expression, IκBα protein expression, and TLR-mediated inflammatory responses. Using a systems biology approach, we demonstrated that NFKBIA/IκBα is a central hub in transcriptional responses of prevalent childhood lung diseases, including respiratory syncytial virus infection, asthma, and bronchopulmonary dysplasia. Finally, by examining independent pediatric lung disease cohorts, we established that this immunologically relevant genetic variation in the promoter of NFKBIA is associated with differential susceptibility to severe bronchiolitis following infection with respiratory syncytial virus, airway hyperresponsiveness, and severe bronchopulmonary dysplasia. These data highlight the importance of negative innate immune regulators, such as NFKBIA, in pediatric lung disease and begin to unravel common aspects in the genetic predisposition to bronchopulmonary dysplasia, bronchiolitis, and childhood asthma.

  5. Metabolic perturbations of postnatal growth restriction and hyperoxia-induced pulmonary hypertension in a bronchopulmonary dysplasia model

    Science.gov (United States)

    Introduction: Neonatal pulmonary hypertension (PH) is a common manifestation of bronchopulmonary dysplasia (BPD) and contributes to increased morbidity and mortality of preterm birth. Postnatal growth restriction and hyperoxia are independent contributors to PH development, as indicated by our previ...

  6. Histologic Evidence of Intrapulmonary Anastomoses by Three-Dimensional Reconstruction in Severe Bronchopulmonary Dysplasia

    Science.gov (United States)

    Sims-Lucas, Sunder; Abman, Steven H.

    2013-01-01

    Rationale: Bronchopulmonary dysplasia (BPD) is the chronic lung disease of infancy that occurs in premature infants after oxygen and ventilator therapy for acute respiratory disease at birth. Despite improvement in current therapies, the clinical course of infants with BPD is often characterized by marked hypoxemia that can become refractory to therapy. Preacinar anatomic and functional communications between systemic and pulmonary vascular systems has been established in fetal lungs, but whether increased intrapulmonary anastomotic vessels or their failure to regress after birth contributes to hypoxemia in preterm infants with BPD is unknown. Objectives: We sought to find histologic evidence of intrapulmonary anastomotic vessels in lungs of patients who died of severe BPD. Methods: We collected lung tissues from fatal BPD cases and performed histology, immunohistochemistry, and high-precision three-dimensional reconstruction techniques. Measurements and Main Results: We report histologic evidence of intrapulmonary vessels that bridge pulmonary arteries and veins in the distal lungs of infants dying with severe BPD. These prominent vessels appear similar to “misaligned pulmonary veins” described in the lethal form of congenital lung disorder, alveolar capillary dysplasia. Conclusions: We found striking histological evidence of precapillary arteriovenous anastomotic vessels in the lungs of infants with severe bronchopulmonary dysplasia. We propose that persistence or expansion of these vessels after premature birth provides the anatomic basis for intrapulmonary shunt and hypoxemia in neonates with severe bronchopulmonary dysplasia and may play a significant role in the morbidity and mortality of BPD. PMID:23987309

  7. Longitudinal assessment of the lung mechanics of very low birth weight preterm infants with and without bronchopulmonary dysplasia.

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    Mello, Rosane Reis de; Silva, Kátia Silveira da; Costa, Anniele Medeiros; Ramos, José Roberto de Moraes

    2015-01-01

    Prematurity has been correlated with altered lung mechanics. Some infants develop lung injury as a consequence of lung immaturity, invasive mechanical ventilation and exposure to oxygen, thus resulting in bronchopulmonary dysplasia. The aim here was to compare the lung mechanics of preterm infants with and without bronchopulmonary dysplasia during the first year of life. Prospective cohort study in a tertiary-level hospital. This study included premature infants at a public hospital who underwent two pulmonary function tests: one at discharge and the other at the corrected age of 4 to 8 months. Tidal volume, lung compliance and lung resistance were measured. Statistical tests were used for comparisons between infants with and without bronchopulmonary dysplasia. 102 children with mean gestational age of 29 ± 2.0 weeks were studied; 17 with bronchopulmonary dysplasia. Lung compliance (0.84 ± 0.29 versus 1.28 ± 0.46; P bronchopulmonary dysplasia than in those without the disease, but no differences were observed at the second test (compliance: 1.53 ± 0.77 versus 1.94 ± 1.01; P = 0.12; and tidal volume: 6.9 ± 1.4 versus 7.3 ± 1.6; P = 0.42). Differences in lung mechanics were observed between infants with and without bronchopulmonary dysplasia at hospital discharge but these differences were no longer detected at the final follow-up. The lung mechanics of all the infants improved over this period of time.

  8. MicroRNA in late lung development and bronchopulmonary dysplasia: the need to demonstrate causality

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    Nardiello, Claudio; Morty, Rory E.

    2016-01-01

    MicroRNA are emerging as powerful regulators of cell differentiation and tissue and organ development. Several microRNA have been described to play a role in branching morphogenesis, a key step in early lung development. However, considerably less attention has been paid to microRNA as regulators of the process of secondary septation, which drives lung alveolarization during late lung development. Secondary septation is severely perturbed in bronchopulmonary dysplasia (BPD), a common complica...

  9. Targeting Inflammation to Prevent Bronchopulmonary Dysplasia: Can New Insights Be Translated Into Therapies?

    Science.gov (United States)

    Kirpalani, Haresh

    2011-01-01

    Bronchopulmonary dysplasia (BPD) frequently complicates preterm birth and leads to significant long-term morbidity. Unfortunately, few therapies are known to effectively prevent or treat BPD. Ongoing research has been focusing on potential therapies to limit inflammation in the preterm lung. In this review we highlight recent bench and clinical research aimed at understanding the role of inflammation in the pathogenesis of BPD. We also critically assess currently used therapies and promising developments in the field. PMID:21646264

  10. Nutrition in extremely low birth weight infants: impact on bronchopulmonary dysplasia.

    Science.gov (United States)

    Uberos, José; Lardón-Fernández, Marita; Machado-Casas, Irene; Molina-Oya, Manuel; Narbona-López, Eduardo

    2016-12-01

    Bronchopulmonary dysplasia (BPD) is a chronic lung disease that affects premature infants with multifactorial etiology. Some authors have considered malnutrition to be a major factor promoting BDP. The aim of our study was to examine the contribution of enteral and parenteral nutritional intake in the first 14 days of life to the development of bronchopulmonary dysplasia in a sample of preterm infants. A prospective cohort study was conducted on all preterm infants born between 1 January 2008 and 31 December 2013. The nutritional parameters compiled included the cumulative amount of fluids, calories, proteins, carbohydrates and lipids consumed. Statistical analysis of the data consisted of a descriptive analysis, Mann-Whitney pairwise comparison test and logistic regression. The total caloric intake in the infants studied was significantly lower in patients with subsequent bronchopulmonary dysplasia (76.1 kCal/kg, 95% CI: 71.2-81.1 vs. 91.1 kCal/kg, 95% CI: 87.5-94.8). The intake of carbohydrate and fat was significantly lower in the patients with BPD (11.6 g/kg, 95% CI: 11.1-12.0 vs. 12.6 g/kg, 95% CI: 12.1-13; and 2.5 g/kg, 95% CI: 2.3-2.7 vs. 3.4 g/kg, 95% CI: 2.9-3.9, respectively). Our study shows that infants who develop bronchopulmonary dysplasia receive a lower enteral intake of calories and total lipids during the first 14 days of life.

  11. Severe bronchopulmonary dysplasia improved by noninvasive positive pressure ventilation: a case report

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    Mann Christian

    2011-09-01

    Full Text Available Abstract Introduction This is the first report to describe the feasibility and effectiveness of noninvasive positive pressure ventilation in the secondary treatment of bronchopulmonary dysplasia. Case presentation A former male preterm of Caucasian ethnicity delivered at 29 weeks gestation developed severe bronchopulmonary dysplasia. At the age of six months he was in permanent tachypnea and dyspnea and in need of 100% oxygen with a flow of 2.0 L/minute via a nasal cannula. Intermittent nocturnal noninvasive positive pressure ventilation was then administered for seven hours daily. The ventilator was set at a positive end-expiratory pressure of 6 cmH2O, with pressure support of 4 cmH2O, trigger at 1.4 mL/second, and a maximum inspiratory time of 0.7 seconds. Over the course of seven weeks, the patient's maximum daytime fraction of inspired oxygen via nasal cannula decreased from 1.0 to 0.75, his respiratory rate from 64 breaths/minute to 50 breaths/minute and carbon dioxide from 58 mmHg to 44 mmHg. Conclusion Noninvasive positive pressure ventilation may be a novel therapeutic option for established severe bronchopulmonary dysplasia. In the case presented, noninvasive positive pressure ventilation achieved sustained improvement in ventilation and thus prepared our patient for safe home oxygen therapy.

  12. Hospital variation and risk factors for bronchopulmonary dysplasia in a population-based cohort.

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    Lapcharoensap, Wannasiri; Gage, Susan C; Kan, Peiyi; Profit, Jochen; Shaw, Gary M; Gould, Jeffrey B; Stevenson, David K; O'Brodovich, Hugh; Lee, Henry C

    2015-02-01

    Bronchopulmonary dysplasia (BPD) remains a serious morbidity in very low-birth-weight (VLBW) infants (Bronchopulmonary dysplasia was defined as continuous supplemental oxygen use at 36 weeks' postmenstrual age. A combined outcome of BPD or mortality prior to 36 weeks was used. Multivariable logistic regression accounting for hospital as a random effect and gestational age as a risk factor was used to assess individual risk factors for BPD. This model was applied to determine risk-adjusted rates of BPD across hospitals and assess associations between levels of care and BPD rates. The study cohort included 15,779 infants, of which 1534 infants died prior to 36 weeks' postmenstrual age. A total of 7081 infants, or 44.8%, met the primary outcome of BPD or death prior to 36 weeks. Combined BPD or death rates across 116 NICUs varied from 17.7% to 73.4% (interquartile range, 38.7%-54.1%). Compared with level IV NICUs, the risk for developing BPD was higher for level II NICUs (odds ratio, 1.23; 95% CI, 1.02-1.49) and similar for level III NICUs (odds ratio, 1.04; 95% CI, 0.95-1.14). Bronchopulmonary dysplasia or death prior to 36 weeks' postmenstrual age affects approximately 45% of VLBW infants across California. The wide variability in BPD occurrence across hospitals could offer insights into potential risk or preventive factors. Additionally, our findings suggest that increased regionalization of NICU care may reduce BPD among VLBW infants.

  13. Prenatal interventions to prevent bronchopulmonary dysplasia in animal models: a systematic review.

    Science.gov (United States)

    Jimenez, Julio; Richter, Jute; Toelen, Jaan; Deprest, Jan

    2016-01-01

    The objective of this study is to identify and systematically review in vivo animal studies on antenatal medical interventions to prevent bronchopulmonary dysplasia. An automated literature search was conducted using MEDLINE (Pubmed) and Embase including all studies using Medical Subject Headings (MeSH) and keywords following a step-by-step approach. All in vivo prenatal intervention studies in animal models mimicking key aspects of the pathophysiology of bronchopulmonary dysplasia were included. In view of relevance of the findings, an additional criterion was that outcomes at 48 h of life or beyond were available. The PRISMA statement concerning systemic reviews was applied and a quality checklist developed by the CAMARADES group was used. In total, 518 abstracts were identified yet only eight studies were eligible for further analysis. Four studies involved administration of glucocorticoids, the other studies described therapy with epidermal growth factor, interleukin 1b, beta-naphthoflavone, or vitamin D. Outcomes were survival, pulmonary histology, lung function, and/or biochemical analysis. Though many in vivo experimental studies in animal models for bronchopulmonary dysplasia have been done, only few have looked into the effect of prenatal interventions and measured outcomes after at least 48 h of life. Most involve the use of antenatal glucocorticoids, although still only four.

  14. [Bronchopulmonary dysplasia epidemic: incidence and associated factors in a cohort of premature infants in Bogotá, Colombia].

    Science.gov (United States)

    Ruiz-Peláez, Juan G; Charpak, Nathalie

    2014-01-01

    There is a perception that bronchopulmonary dysplasia incidence has increased in Bogotá since 2000. This study estimates its incidence, compares it with historical data and describes associated factors. We carried out a prospective analytical cohort of preterm newborns =34 weeks of gestational age without major malformations from 12 health facilities from Bogotá in 2004. The main outcomes were incidence and severity of bronchopulmonary dysplasia, which were compared with an historical cohort (1994-1999). Neonatal mortality was 80/496, and the bronchopulmonary dysplasia incidence was 54.3% (95% CI, 49.4-59.1). When controlling for type of institution (low and high mortality) it appeared that being born in an institution with low mortality decreased the risk for death (OR=0.308; 95% CI, 0.129-0.736) but increased the odds for moderate-severe bronchopulmonary dysplasia (OR=1.797; 95% CI, 1.046-3.088). The risk for bronchopulmonary dysplasia was higher than for the historical control cohort (RR=1.924; 95% CI, 1.686-2.196). Weight and gestational age at birth, mechanical ventilation, intrauterine growth restriction and type of institution (low vs. intermediate-high mortality) were independently associated with bronchopulmonary dysplasia of increasing severity or even death. The frequency of bronchopulmonary dysplasia in Bogotá has increased markedly, and this cannot be explained solely by better survival of more fragile infants. Survivors-irrespective from gestational age-- have more frequent and more severe respiratory sequels. Probably suboptimal aggressive respiratory care practices associated with a recent transition from restricted to almost universal access to mechanical ventilation in neonatal intensive care units in Bogota might be compromising the quality of neonatal respiratory care.

  15. Fluid and electrolyte balance during the first week of life and risk of bronchopulmonary dysplasia in the preterm neonate

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    Gustavo Rocha

    2010-01-01

    Full Text Available BACKGROUND: Early fluid and electrolyte imbalances may be associated with an increased risk of bronchopulmonary dysplasia. OBJECTIVE: We sought to establish an association between fluid and electrolyte balance in the first week of life and the risk of bronchopulmonary dysplasia. METHODS: Clinical charts of 205 neonates <32 weeks gestational age and/or <1,250 g birth weight (admitted to our NICU between 1997 and 2008 were analyzed. Clinical features, fluid and electrolyte balance were analyzed for the first 7 days of life using multivariate models of generalized estimation equations. A p value <0.05 was considered significant in all of the hypothesis tests. RESULTS: The prevalence of bronchopulmonary dysplasia was 22%. Lower gestational age and birth weight, male gender, less frequent use of antenatal steroids, respiratory distress syndrome, use of surfactant, patent ductus arteriosus, duration of invasive ventilation and NICU stay were significantly associated with bronchopulmonary dysplasia. The variation in serum values of potassium, phosphorus and creatinine during the first week of life also revealed an association with bronchopulmonary dysplasia. Higher mean plasma calcium values were associated with spontaneous closure of the patent ductus arteriosus. The use of indomethacin to induce patent ductus arteriosus closure was significantly higher in bronchopulmonary dysplasia patients. CONCLUSIONS: Differences in renal function and tubular handling of potassium and phosphorus are present during the first week of life among preterm neonates who will develop bronchopulmonary dysplasia. The higher rate of patent ductus arteriosus and indomethacin use may influence these differences. Serum levels of calcium also appear to play a role in spontaneous ductus arteriosus closure.

  16. Association between clinical variables related to asthma in schoolchildren born with very low birth weight with and without bronchopulmonary dysplasia.

    Science.gov (United States)

    Gonçalves, Emília da Silva; Mezzacappa-Filho, Francisco; Severino, Silvana Dalge; Ribeiro, Maria Ângela Gonçalves de Oliveira; Marson, Fernando Augusto de Lima; Morcilo, Andre Moreno; Toro, Adyléia Aparecida Dalbo Contrera; Ribeiro, José Dirceu

    2016-09-01

    to assess the prevalence, spirometry findings and risk factors for asthma in schoolchildren who were very low birth weight infants with and without bronchopulmonary dysplasia. Observational and cross-sectional study. The parents and/or tutors answered the International Study of Asthma and Allergies in Childhood questionnaire. The schoolchildren were submitted to the skin prick test and spirometry assessment. 54 schoolchildren who were very low birth weight infants were assessed and 43 met the criteria for spirometry. Age at the assessment (bronchopulmonary dysplasia=9.5±0.85; without bronchopulmonary dysplasia=10.1±0.86 years) and birth weight (bronchopulmonary dysplasia=916.7±251.2; without bronchopulmonary dysplasia=1,171.3±190.5g) were lower in the group with bronchopulmonary dysplasia (p<0.05). The prevalence of asthma among very low birth weight infants was 17/54 (31.5%), being 6/18 (33.3%) in the group with bronchopulmonary dysplasia. There was an association between wool blanket use in the first year of life (p=0.026) with the presence of asthma at school age. The skin prick test was positive in 13/17 (76.5%) and 23/37 (62.2%) of patients with and without asthma, respectively. The schoolchildren with asthma had lower z-score values of forced expiratory flow between 25% and 75% of forced vital capacity (n=16; -1.04±1.19) when compared to the group of patients without asthma (n=27; -0.38±0.93) (p=0.049). There was no difference between the spirometry variables in the groups regarding the presence or absence of bronchopulmonary dysplasia. Very low birth weight infants with and without bronchopulmonary dysplasia showed a high prevalence of asthma (33.3% and 30.6%, respectively). Pulmonary flow in the small airways was lower in children with asthma. Copyright © 2016 Sociedade de Pediatria de São Paulo. Publicado por Elsevier Editora Ltda. All rights reserved.

  17. [BRONCHOPULMONARY DYSPLASIA--WHAT DO WE KNOW TODAY?].

    Science.gov (United States)

    Radulova, P; Vakrilova, L; Slancheva, B

    2015-01-01

    The survival of great number of extremely premature newborn babies is associated with increased risk of damage of the newborn lung and development of chronic lung disease/Broncopulmonary dysplasia. The lower the gestational age and weight, the greater the frequency of BPD. The disease leads to impairment of the normal alveolization and vascularization of the premature lung. There are new theories for the pathogenesis of BPD and new staging of the disease. These changes lead to new therapeutic strategies.

  18. Late (> 7 days) systemic postnatal corticosteroids for prevention of bronchopulmonary dysplasia in preterm infants.

    Science.gov (United States)

    Doyle, Lex W; Cheong, Jeanie L; Ehrenkranz, Richard A; Halliday, Henry L

    2017-10-24

    Many preterm infants who survive go on to develop bronchopulmonary dysplasia, probably as the result of persistent inflammation in the lungs. Corticosteroids have powerful anti-inflammatory effects and have been used to treat individuals with established bronchopulmonary dysplasia. However, it is unclear whether any beneficial effects outweigh the adverse effects of these drugs. To examine the relative benefits and adverse effects of late systemic postnatal corticosteroid treatment (> 7 days) for preterm infants with evolving or established bronchopulmonary dysplasia. For the 2017 update, we used the standard search strategy of Cochrane Neonatal to search the Cochrane Central Register of Controlled Trials (CENTRAL; 2017, Issue 1); MEDLINE via PubMed (January 2013 to 21 February 2017); Embase (January 2013 to 21 February 2017); and the Cumulative Index to Nursing and Allied Health Literature (CINAHL; January 2013 to 21 February 2017). We also searched clinical trials databases, conference proceedings, and reference lists of retrieved articles for randomised controlled trials and quasi-randomised trials. We selected for inclusion in this review randomised controlled trials (RCTs) comparing systemic postnatal corticosteroid treatment versus placebo or nothing initiated more than seven days after birth for preterm infants with evolving or established bronchopulmonary dysplasia. We used the GRADE approach to assess the quality of evidence.We extracted and analysed data regarding clinical outcomes including mortality, bronchopulmonary dysplasia, death or bronchopulmonary dysplasia, failure to extubate, complications during primary hospitalisation, and long-term health outcomes. Twenty-one RCTs enrolling a total of 1424 participants were eligible for this review. All were RCTs, but methods used for random allocation were not always clear. Allocation concealment, blinding of the intervention, and blinding of outcome assessments most often were satisfactory. Late steroid

  19. Rib enlargement in premature infants with bronchopulmonary dysplasia

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    Yoon, Hye Kyung; Han, Kim Bokyung; Chang, Yun Sil; Choo, In Wook [Samsung Medical Center, School of Medicine, Sungkyunkwan University, Seoul (Korea, Republic of); Kim, Kyeong Ah [Anyang General Hospital, Anyang (Korea, Republic of)

    2000-04-01

    The purpose of this study is to describe the rib changes seen in patients with brochopulmonary dysplasia (BPD). Serial chest radiographs of nine premature infants with BPD who showed diffuse rib enlargement were reviewed for hyperinflation, which was compared with the observed degree of rib enlargement. Vibrator chest physiotherapy was performed in all cases, and five infants underwent conventional ventilation plus high frequency oscillatory ventilation therapy. Their calcium level was normal whereas alkaline phosphatase and phosphate levels were high. In all infants except one, liver enzyme levels were normal. For the treatment of patent ductus arteriosus, infection, and BPD, medications including indomethacin, antibiotics, and dexamethasone were administered. Vitamin D was given to all patients with total parenteral nutrition. Rib enlargement was found to be severe (n=3D4), moderate (n=3D3), or mild (n=3D2) with undulating margins or posterior tapering (n=3D2). Hyperinflation was noted in eight patients, in seven of whom it was moderate to severe. Among these seven, rib enlargement was severe (n=3D2), moderate (n=3D3), or mild (n=3D2). In one infant with mild hyperinflation, rib enlargement was severe. Bilateral irregular infiltrates and atelectases were noted in all patients. In BPD patients, rib enlargement may be seen. In order to differentiate this process from systemic bone disease or bony dysplasia, an awareness of the rib changes occurring in patients with BPD may be important. (author)

  20. Nutrition of preterm infants with bronchopulmonary dysplasia after hospital discharge – Part I

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    Hercília Guimarães

    2014-01-01

    Full Text Available Bronchopulmonary dysplasia (BPD remains the most common severe complication of preterm birth. In addition to well known risk factors, nutrition plays an important role in normal lung development and maturation. Nutrition has a direct effect on the developing lung because it can modulate lung structure. Lung growth and maturation continue after hospital discharge and BPD patient’s nutritional requirements and feeding problems will need a specialized multidisciplinary approach during follow-up. Our aim is to review the scientific literature on the most relevant aspects of nutrition of BPD patients after hospital discharge.

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

    Science.gov (United States)

    Viscardi, Rose Marie; Kallapur, Suhas G

    2015-12-01

    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. Copyright © 2015 Elsevier Inc. All rights reserved.

  2. How to decrease bronchopulmonary dysplasia in your neonatal intensive care unit today and “tomorrow”

    Science.gov (United States)

    Nelin, Leif D.; Bhandari, Vineet

    2017-01-01

    Bronchopulmonary dysplasia, or BPD, is the most common chronic lung disease in infants. Genetic predisposition and developmental vulnerability secondary to antenatal and postnatal infections, compounded with exposure to hyperoxia and invasive mechanical ventilation to an immature lung, result in persistent inflammation, culminating in the characteristic pulmonary phenotype of BPD of impaired alveolarization and dysregulated vascularization. In this article, we highlight specific areas in current management, and speculate on therapeutic strategies that are on the horizon, that we believe will make an impact in decreasing the incidence of BPD in your neonatal intensive care units. PMID:28503300

  3. Revisiting the Definition of Bronchopulmonary Dysplasia: Effect of Changing Panoply of Respiratory Support for Preterm Neonates.

    Science.gov (United States)

    Isayama, Tetsuya; Lee, Shoo K; Yang, Junmin; Lee, David; Daspal, Sibasis; Dunn, Michael; Shah, Prakesh S

    2017-03-01

    Several definitions of bronchopulmonary dysplasia are clinically used; however, their validity remains uncertain considering ongoing changes in the panoply of respiratory support treatment strategies used within neonatal units. To identify the optimal definition of bronchopulmonary dysplasia that best predicts respiratory and neurodevelopmental outcomes in preterm infants. Retrospective cohort study at tertiary neonatal intensive care units. Preterm infants born at less than 29 weeks' gestation between 2010 and 2011 who were admitted to neonatal intensive care units participating in the Canadian Neonatal Network and completed follow-up assessments in a Canadian Neonatal Follow-Up Network clinic at 18 to 21 months. Various traditional bronchopulmonary dysplasia criteria based on respiratory status at different postmenstrual ages. Serious respiratory morbidity, neurosensory impairment at 18 to 21 months of age, and a composite outcome of respiratory or neurosensory morbidity or death after discharge. Adjusted odds ratios (AORs) and 95% CIs were calculated. Of 1914 eligible survivors, 1503 were assessed (mean gestational age was 26.3 weeks; 68% were white, 9% were black, and 23% were other race/ethnicity), 88 had serious respiratory morbidity, 257 infants had neurosensory impairment, and 12 infants died after discharge. Definitions using oxygen requirement alone as the criterion at various postmenstrual ages were less predictive compared with those using the criterion of oxygen/respiratory support (RS) (receiving supplemental oxygen and/or positive-pressure RS); among those, oxygen/RS at 36 weeks had the highest AOR and area under the curve (AUC) for all outcomes. Further analyses of oxygen/RS at each week between 34 and 44 weeks' postmenstrual age indicated that the predictive ability for serious respiratory morbidity increased from 34 weeks (AOR, 1.8; 95% CI, 0.9-3.4, AUC, 0.721) to 40 weeks (AOR, 6.1; 95% CI, 3.4-11.0; AUC, 0.799). For serious neurosensory

  4. Effects of the early administration of sivelestat sodium on bronchopulmonary dysplasia in infants: A retrospective cohort study.

    Science.gov (United States)

    Ogawa, Ryo; Mori, Rintaro; Iida, Koichi; Uchida, Yumiko; Oshiro, Makoto; Kageyama, Misao; Kato, Yuichi; Tanaka, Taihei; Nakata, Yusei; Nishimura, Yutaka; Hokuto, Isamu; Bonno, Motoki; Matsumoto, Naoko; Ito, Masato; Takahashi, Noriko; Namba, Fumihiko

    2017-09-23

    Chorioamnionitis, or infiltration of the chorioamnion by neutrophils, is a risk factor associated with the development of bronchopulmonary dysplasia. Increased neutrophil elastase levels are observed in the tracheal aspirates of these patients. To examine the effects of early administration of the selective neutrophil elastase inhibitor sivelestat, which is used to treat acute lung injury in adults, on bronchopulmonary dysplasia in extremely premature infants. Retrospective cohort study. This study included extremely low-birth-weight infants born at a gestational agebronchopulmonary dysplasia-free survival at a postmenstrual age of 36weeks, and the secondary outcomes included various clinically significant factors of neonatal mortality and morbidity and adverse events. Of the 1031 included neonates, 124 (12.0%) were treated with sivelestat. Significant differences between the groups were noted for gestational age, delivery method, fetal number, the frequency of chorioamnionitis, immunoglobulin M levels, and WBC counts. No differences were identified concerning the bronchopulmonary dysplasia-free survival rate at a postmenstrual age of 36weeks (adjusted odds ratio for sivelestat to control, 0.83; 95% confidence interval=0.53-1.30). Secondary outcomes did not significantly differ between the groups. In extremely premature infants, early sivelestat use was not associated with an improved rate of survival without bronchopulmonary dysplasia at a postmenstrual age of 36weeks. Copyright © 2017 Elsevier B.V. All rights reserved.

  5. Transition from gavage to nipple feeding for preterm infants with bronchopulmonary dysplasia.

    Science.gov (United States)

    McCain, Gail C; Del Moral, Teresa; Duncan, Robert C; Fontaine, Jessica Loucas; Pino, Lilia Diaz

    2012-01-01

    The transition from gavage to nipple feeding is difficult for preterm infants with bronchopulmonary dysplasia because of tachypnea and hypoxemia from chronic respiratory distress. The aim of this study was to test the hypothesis that preterm infants with bronchopulmonary dysplasia who transitioned from gavage to nipple feeding with the semidemand method would achieve nipple feeding sooner and be discharged from hospital sooner than control infants who received standard care. Forty-two infants were randomized to the control condition and 44 to the experimental protocol. Mean gestational ages and birth weights were 25 ± 1.5 weeks and 784 g for controls and 25 ± 1.4 weeks and 787 g for experimental infants. Control infants received standard care that included gradual increases in the number of nipple to gavage feedings per day. Experimental infants received the semidemand method that used infant behavioral and cardiorespiratory signs to regulate frequency, length, and volume of nipple feedings. General linear model procedures were used to compare study groups. Experimental infants achieved nipple feeding at M = 5.9 ± 0.7 days compared with control infants, M = 12.3 ± 0.8 (p < .0001). Length of hospitalization was not significantly different between groups. The semidemand method significantly shortened the time for infants to attain nipple feeding in a manner taking their respiratory distress into consideration.

  6. High-resolution CT findings in infants with bronchopulmonary dysplasia: preliminary report

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    Chung, Yoon Ho; Lee, Young Seok; Kim, Ji Hye; Han, Heon; Chung, Hyo Sun; Cha, Yoo Mi; Kim, Young Chae; Kim, Sang Hee [Chungang Gil Hospital, Inchon (Korea, Republic of)

    1996-07-01

    To evaluate high resolution CT(HRCT) findings in infants with bronchopulmonary dysplasia(BPD). In 13 infants(age range, 1-12 months;11 premature babies, two full-term babies; birth weight, 0.97-3.88kg;mean 2,03kg) with clinico-radiologically suggested BPD, HRCT findings of the lung were reviewed retrospectively. Spiral CT using ultra high bone algorithm, 1mm collimation with 5-8mm interval, and 0.7sec scan time was performed without regard to breathing-control of infants. Three radiologists each analysed the HRCT findings twice. HRCT findings of BPD were as follows:parenchymal bands(n=13), interlobular septal thickenings (n=12), multifocal hyperaeration involving lobar or segmental distribution(n=7), and involving lobular distribution or small cyst-like lesion(n=4), centrilobular nodules(n=7), consolidation and/or atelectasis(n=7), and bronchovascular bundle thickening(n=6). Parenchymal bands, interlobular septal thickenings, and multifocal hyperaerations were the major findings in cases of bronchopulmonary dysplasia whereas, centrilobular nodules, consolidation and/or atelectasis, and bronchovascular bundle thickenings were the minor findings. These findings may be used as basic data in the evaluation of BPD in future studies.

  7. INCIDENCE OF BRONCHOPULMONARY DYSPLASIA IN PRETERM NEWBORNS SUBMITTED TO MECHANICAL VENTILATION: A RETROSPECTIVE STUDY OF 1250 PRETERM NEWBORNS

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    Leilianna de Souza Vieira

    2014-03-01

    Full Text Available Objective: To determine the incidence of preterm newborn infants in mechanical ventilation who developed bronchopulmonary dysplasia in a public hospital at Fortaleza/CE. Method: Descriptive, retrospective and longitudinal quantitative analysis with 1250 preterm infants admitted to the Intensive Care Unit, Dr. César Cals General Hospital, at Fortaleza, from July 2006 to June 2007. Data collection occurred during two months, with visits to units twice a week, where the medical records were done. Were included in these sample newborns that were in mechanical ventilation and developed bronchopulmonary dysplasia. Then the gestational average was 28.6 weeks; the mean weight of infants was 1125.33 grams, born vaginally or cesarean section, of both sexes and with various primary diseases such as respiratory distress syndrome, jaundice and neonatal infection. Results: In the sample from the total admissions, 34.48% were for mechanical ventilation and 3.48% developed bronchopulmonary dysplasia. Conclusion: Despite the low prevalence, bronchopulmonary dysplasia is a important complication of prematurity, directly related to the duration of mechanical ventilation, thus the team must be committed on weaning and extubation of those as soon as possible, preferably within the first week of life.

  8. Anatomical Closure of Left-to-Right Shunts in Premature Infants with Bronchopulmonary Dysplasia and Pulmonary Hypertension: A Cautionary Tale

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    Narendra R. Dereddy

    2015-10-01

    Full Text Available Closure of a systemic to pulmonary shunt in premature infants with bronchopulmonary dysplasia may be beneficial, but in the presence of pulmonary hypertension is controversial. Here, we discuss two premature infants with pulmonary hypertension who developed acute pulmonary hypertensive crisis after closure of these shunts and hence advise caution.

  9. The Role of Chronic Hypoxia in the Development of Neurocognitive Abnormalities in Preterm Infants with Bronchopulmonary Dysplasia

    Science.gov (United States)

    Raman, Lakshmi; Georgieff, Michael K.; Rao, Raghavendra

    2006-01-01

    Bronchopulmonary dysplasia is the most common pulmonary morbidity in preterm infants and is associated with chronic hypoxia. Animal studies have demonstrated structural, neurochemical and functional alterations due to chronic hypoxia in the developing brain. Long-term impairments in visual-motor, gross and fine motor, articulation, reading,…

  10. Lung function following very preterm birth in the era of 'new' bronchopulmonary dysplasia.

    Science.gov (United States)

    Simpson, Shannon J; Hall, Graham L; Wilson, Andrew C

    2015-05-01

    One of the most significant complications of preterm birth is bronchopulmonary dysplasia (BPD). The pathophysiology of BPD has changed in recent years as advances in neonatal care have led to increased survival of smaller, more preterm, infants who display alterations to alveolar and pulmonary microvascular development. It is becoming clear that infants with 'new' BPD experience lung disease that persists into later childhood, however, the oldest of these children are just now entering young adulthood and therefore the longer term pulmonary implications remain unknown. The role of lung function testing in the identification and subsequent management of patients with lung disease resulting from a neonatal classification of BPD is reviewed based on the underlying pathophysiology of the disease. © 2015 Asian Pacific Society of Respirology.

  11. Understanding the Short- and Long-Term Respiratory Outcomes of Prematurity and Bronchopulmonary Dysplasia

    Science.gov (United States)

    Islam, Jessica Y.; Keller, Roberta L.; Aschner, Judy L.; Hartert, Tina V.

    2015-01-01

    Bronchopulmonary dysplasia (BPD) is a chronic respiratory disease associated with premature birth that primarily affects infants born at less than 28 weeks’ gestational age. BPD is the most common serious complication experienced by premature infants, with more than 8,000 newly diagnosed infants annually in the United States alone. In light of the increasing numbers of preterm survivors with BPD, improving the current state of knowledge of long-term respiratory morbidity for infants with BPD is a priority. We undertook a comprehensive review of the published literature to analyze and consolidate current knowledge of the effects of BPD that are recognized at specific stages of life, including infancy, childhood, and adulthood. In this review, we discuss both the short-term and long-term respiratory outcomes of individuals diagnosed as infants with the disease and highlight the gaps in knowledge needed to improve early and lifelong management of these patients. PMID:26038806

  12. PREVENTIVE STRATEGIES IN THE STAGES OF FORMATION AND COURSE OF BRONCHOPULMONARY DYSPLASIA

    Directory of Open Access Journals (Sweden)

    I. V. Davydova

    2014-01-01

    Full Text Available Prevention of development of bronchopulmonary dysplasia (BPD and possibility of preventing severe course of the disease are the first-priority areas of neonatal pulmonology. Use of modern medical technologies allows protecting a premature infant’s respiratory system from as early as antenatal period of development. Use of the newest neonatal resuscitation protocols may prevent development of BPD. International experience and clinical data gathered by the authors demonstrate that seasonal palivizumab immune prevention of severe course of a respiratory syncytial viral infection in children with BPD results in rarer cases of hospitalization, resuscitation measures and fatal outcomes. The article presents modern preventive strategies used in this category of patients. 

  13. The Future of Bronchopulmonary Dysplasia: Emerging Pathophysiological Concepts and Potential New Avenues of Treatment

    Science.gov (United States)

    Collins, Jennifer J. P.; Tibboel, Dick; de Kleer, Ismé M.; Reiss, Irwin K. M.; Rottier, Robbert J.

    2017-01-01

    Yearly more than 15 million babies are born premature (bronchopulmonary dysplasia (BPD), one of the most common adverse outcomes in these preterm neonates. BPD patients have an arrest in alveolar and microvascular development and more frequently develop asthma and early-onset emphysema as they age. Understanding how the alveoli develop, and repair, and regenerate after injury is critical for the development of therapies, as unfortunately there is still no cure for BPD. In this review, we aim to provide an overview of emerging new concepts in the understanding of perinatal lung development and injury from a molecular and cellular point of view and how this is paving the way for new therapeutic options to prevent or treat BPD, as well as a reflection on current treatment procedures. PMID:28589122

  14. Bottle-feeding behaviors in preterm infants with and without bronchopulmonary dysplasia.

    Science.gov (United States)

    Howe, Tsu-Hsin; Sheu, Ching-Fan; Holzman, Ian R

    2007-01-01

    This study compared bottle-feeding behaviors in preterm infants with and without bronchopulmonary dysplasia (BPD) during the initial hospitalization. Individual sucking characteristics and feeding transitional rates were compared in 41 preterm infants (22 boys, 19 girls) with BPD and 99 infants (44 boys, 55 girls) without BPD. Observations of the first bottle feeding and observations of the last feeding before discharge were obtained from medical records of all infants retrospectively. On discharge, infants with BPD, unlike those without BPD, continued to have an immature sucking pattern and required longer hospital stays to attain full oral feeding (p feeding and use of oral support. These results suggest that feeding transitional rate, rather than sucking pattern, may be a better discharge indicator for infants with BPD.

  15. Fetal Doppler velocimetry and bronchopulmonary dysplasia risk among growth-restricted preterm infants: an observational study.

    Science.gov (United States)

    Lio, Alessandra; Rosati, Paolo; Pastorino, Roberta; Cota, Francesco; Tana, Milena; Tirone, Chiara; Aurilia, Claudia; Ricci, Cinzia; Gambacorta, Alessandro; Paladini, Angela; Mappa, Ilenia; Buongiorno, Silvia; Zannoni, Gian Franco; Romagnoli, Costantino; Vento, Giovanni

    2017-07-20

    To investigate whether fetal growth restriction (FGR) diagnosis, based on pathological prenatal fetal Doppler velocimetry, is associated with bronchopulmonary dysplasia (BPD) independently of being small for gestational age (SGA) per se at birth among very preterm infants. Prospective, observational study. FGR was defined as failing fetal growth in utero and fetal Doppler velocimetry abnormalities. Policlinico Universitario Agostino Gemelli, Roma, Italy. Preterm newborns with gestational age ≤30 weeks and birth weight (BW) ≤1250 g. Bronchopulmonary dysplasia. In the study period, 178 newborns were eligible for the study. Thirty-nine infants (22%) were considered fetal growth-restricted infants. Among the 154 survived babies at 36 weeks postmenstrual age, 12 out of 36 (33%) of the FGR group developed BPD versus 8 out of 118 (7%) of the NO-FGR group (p<0.001). BPD rate was sixfold higher among the SGA-FGR infants compared with the SGA-NO-FGR infants. In a multivariable model, FGR was significantly associated with BPD risk (OR 5.1, CI 1.4 to 18.8, p=0.01), independently from BW z-score that still remains a strong risk factor (OR 0.5, CI 0.3 to 0.9, p=0.01). Among SGA preterm infants, BPD risk dramatically increases when placenta dysfunction is the surrounding cause of low BW. Antenatal fetal Doppler surveillance could be a useful tool for studying placenta wellness and predicting BPD risk among preterm babies. Further research is needed to better understand how FGR affects lung development. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  16. Placental Villous Vascularity Is Decreased in Premature Infants with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension.

    Science.gov (United States)

    Yallapragada, Sushmita G; Mestan, Karen K; Palac, Hannah; Porta, Nicolas; Gotteiner, Nina; Hamvas, Aaron; Grobman, William; Ernst, Linda M

    2016-01-01

    The development of pulmonary hypertension (PH) is a serious complication of bronchopulmonary dysplasia (BPD) among infants born at extremely low gestational ages. Bronchopulmonary dysplasia-associated PH is characterized by persistent pulmonary vasoconstriction, progressive right heart dysfunction, and an increased risk of death. We have shown previously that certain placental vascular lesions are associated with BPD-associated PH. Further evaluation of the villous and vascular morphometry of these placentas is warranted. Using digital image analysis (DIA), we compared villous and vascular morphometric parameters of placentas from infants with and without BPD-associated PH. We conducted a case-control study of placentas from 14 infants born at ≤28 weeks' gestational age (GA). Cases with PH (N=7) and non-PH controls (N=7) were identified using echocardiogram screening at 36 weeks' corrected GA. Central parenchymal sections from each placenta were stained for CD31. Digital image analysis was used to measure vessel and villous capillary number, perimeter, diameter, and area. Mean villous vascularity (number of vessels per villus) was calculated for each patient. Mean vessel and villous number as well as area were similar between the two groups. Villous vascularity was decreased in placentas from infants who ultimately had PH disease compared to non-PH controls (5.5±1.0 vs 7.1±1.6; P<0.05). Placental villous vascularity is decreased in infants with BPD-associated PH. Further studies should assess whether placental morphometric markers may allow clinicians to better predict BPD and provide earlier and more targeted management.

  17. Vitamin A Supplementation for Prevention of Bronchopulmonary Dysplasia: Cornerstone of Care or Futile Therapy?

    Science.gov (United States)

    Gawronski, Catherine A; Gawronski, Kristen M

    2016-08-01

    To review the evidence on vitamin A supplementation (VAS) and bronchopulmonary dysplasia (BPD) in extremely-low-birth-weight infants. We also discuss the impact of a vitamin A shortage on BPD rates. A PubMed search inclusive of dates 1946 to March 2016 was performed using the search terms bronchopulmonary dysplasia, chronic lung disease (CLD), and vitamin A STUDY SELECTION AND DATA EXTRACTION: All English-language studies were evaluated. Only those investigating VAS by intramuscular administration were included. A total of 6 studies were evaluated. Additionally, a report on the incidence of BPD during a national shortage was reviewed. Investigators found mixed results with VAS and incidence of CLD or death in a varying number of neonates. In the largest evaluation, investigators found a statistically significant decrease in the rate of death or BPD: 55% in the VAS group versus 62% in the placebo group. The number needed to treat to prevent 1 case of BPD was 15 infants. Few studies found an increased incidence of adverse events following VAS. A report over a 2-year shortage period found that whereas the rate of VAS declined dramatically, BPD rates remained stable. This large observational evaluation calls into question the place of vitamin A in BPD prevention. VAS has been identified as a strategy to decrease the incidence of BPD. Initial large-scale prospective evaluations have shown clear benefit of VAS in reducing the incidence of CLD or death. However, changing definitions of BPD and implementation of noninvasive ventilation strategies limit the application of early studies. During a drug shortage, VAS declined dramatically, but BPD rates remained stable. With concerns of sepsis and necrotizing enterocolitis in small-scale studies, and in light of the recent shortage evidence, further evaluations are necessary before VAS can be recommended as a cornerstone of BPD prevention. © The Author(s) 2016.

  18. [Incidence, pathomorphism and outcomes of the bronchopulmonary dysplasia associated with microaspiration of gastric contents].

    Science.gov (United States)

    Bryksina, E Y; Bryksin, V S; Pochivalov, A V

    2016-01-01

    Today the influence of the digestive tract functional violations followed by microaspiration of gastric contents (MAGC) on the incidence, features and outcomes of bronchopulmonary dysplasia BPD) remains little studied. Focusing on this aspect makes the research actual. determination of the nature of influence of MAGC on the progress and course of BPD. 373 newborns exposed to artificial pulmonary ventilation (APV) in the neonatal period were examined. In a tracheobronchial aspirate (TBA) the marker of MAGC-pepsin--was determined. Its activity was measured by extinction value with subsequent analysis of the incidence and nature of the course of bronchopulmonary dysplasia (BPD) in patients against MAGC and without it. During the three years follow-up period outcomes of BPD and features of combined pathology were established. it was revealed that in children suffered from MAGC the incidence of BPD was higher and grew in proportion to the increase of pepsin activity in TBA and the reduction of gestational age. The extinction increase in TBA values was followed by the increase in duration of APV and the subsequent oxygen therapy, and also the severity of BPD clinical course. By the end of 3 years clinical recovery was detected in 55.2% of children suffering from BPD without MAGC whereas in patients with BPD against MAGC this outcome occured only in 0.9% of cases. MAGC is clinically significant for the etiology, pathogenesis and pathomorphism of BPD. The proposed method of early detection of MAGC and algorithm of complex therapy can reduce its severity and improve the forecast accuracy of neonatal adaptation.

  19. Extrapulmonary Conditions, Concomitant of Bronchopulmonary Dysplasia, in Babies of the First 3 Years of Life: Results of a Retrospective Cross-Sectional Study

    Directory of Open Access Journals (Sweden)

    K. A. Kazakova

    2016-01-01

    Full Text Available Background: There are few data on co-occurring with bronchopulmonary dysplasia diseases but there is no single point of view on their mutual effect.Objective: Our aim was to learn the structure and frequency of extrapulmonary disease, concomitant of bronchopulmonary dysplasia, in children aged up to 3 years.Methods. A retrospective analysis of histories of 93 children with bronchopulmonary dysplasia with an analysis of the consequences of perinatal pathology structure was carried out.Results. On average, each patient with bronchopulmonary dysplasia accounted for 5 comorbidities. The most common (89; 96% were perinatal lesions of the nervous system and their consequences. In children with bronchopulmonary dysplasia at the age of 3 years there was a relatively low incidence of hydrocephalus and, on the contrary, high — of infantile cerebral palsy. Violations of the organs of vision were found in 58 (62% children, malnutrition and other violations of physical development — in 58 (62% and 27 (29%, respectively, and the cardiovascular system pathology — in 59 (63%.Conclusion. The most commonly, extrapulmonary pathology, co-occuring with bronchopulmonary dysplasia, includes neurological deficit with psychomotor retardation, violations of organs of vision, pathology of the cardiovascular system, malnutrition/delay in physical development.

  20. Asbestos bodies in children's lungs. An association with sudden infant death syndrome and bronchopulmonary dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Haque, A.K.; Kanz, M.F.

    1988-05-01

    Lungs from 46 autopsied children (age range, 1 to 27 months) were examined for asbestos bodies using a bleach-digestion extraction technique. Ten (21.7%) of 46 children had asbestos bodies in their lungs. Of these ten children, seven were diagnosed with sudden infant death syndrome, and three were diagnosed with bronchopulmonary dysplasia. Thus, 46.6% of children with sudden infant death syndrome and 42.8% of children with bronchopulmonary dysplasia had asbestos bodies. Impaired lung-clearing mechanisms due to either abnormal lung physiology or reorganization of pulmonary architecture may be significant in the formation of asbestos bodies. Additionally, children with asbestos bodies may have been exposed to higher ambient levels of asbestos and other pollutants.

  1. Donor Human Milk Protects against Bronchopulmonary Dysplasia: A Systematic Review and Meta-Analysis

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    Eduardo Villamor-Martínez

    2018-02-01

    Full Text Available Bronchopulmonary dysplasia (BPD is the most common complication after preterm birth. Pasteurized donor human milk (DHM has increasingly become the standard of care for very preterm infants over the use of preterm formula (PF if the mother’s own milk (MOM is unavailable. Studies have reported beneficial effects of DHM on BPD. We conducted a systematic review and meta-analysis of randomized controlled trials (RCTs and observational studies on the effects of DHM on BPD and other respiratory outcomes. Eighteen studies met the inclusion criteria. Meta-analysis of RCTs could not demonstrate that supplementation of MOM with DHM reduced BPD when compared to PF (three studies, risk ratio (RR 0.89, 95% confidence interval (CI 0.60–1.32. However, meta-analysis of observational studies showed that DHM supplementation reduced BPD (8 studies, RR 0.78, 95% CI 0.67–0.90. An exclusive human milk diet reduced the risk of BPD, compared to a diet with PF and/or bovine milk-based fortifier (three studies, RR 0.80, 95% CI 0.68–0.95. Feeding raw MOM, compared to feeding pasteurized MOM, protected against BPD (two studies, RR 0.77, 95% CI 0.62–0.96. In conclusion, our data suggest that DHM protects against BPD in very preterm infants.

  2. Respiratory Phenotypes for Preterm Infants, Children, and Adults: Bronchopulmonary Dysplasia and More.

    Science.gov (United States)

    Collaco, Joseph M; McGrath-Morrow, Sharon A

    2018-01-12

    Ongoing advancements in neonatal care since the late 1980's have led to increased numbers of premature infants surviving well beyond the neonatal period. As a result of increased survival, many individuals born preterm manifest chronic respiratory symptoms throughout infancy, childhood and adult life. The archetypical respiratory disease of prematurity, bronchopulmonary dysplasia (BPD), is the second most common chronic pediatric respiratory disease after asthma. However, there are several commonly held misconceptions. These misconceptions include that BPD is rare, that BPD resolves within the first few years of life, and that BPD does not impact respiratory health in adult life. This focused review article describes a spectrum of respiratory conditions that individuals born prematurely may experience throughout their lifespan. Specifically, this review provides quantitative estimates of the number of individuals with alveolar, airway, and vascular phenotypes associated with BPD as well as non-BPD respiratory phenotypes such as airway malacia, obstructive sleep apnea, and control of breathing issues. Furthermore, this review illustrates what is known about the potential for progression and/or lack of resolution of these respiratory phenotypes in childhood and adult life. Recognizing the spectrum of respiratory phenotypes associated with individuals born preterm and providing comprehensive and personalized care to these individuals may help to modulate adverse respiratory outcomes in later life.  .

  3. Continuous positive airway pressure titration in infants with severe upper airway obstruction or bronchopulmonary dysplasia.

    Science.gov (United States)

    Khirani, Sonia; Ramirez, Adriana; Aloui, Sabrina; Leboulanger, Nicolas; Picard, Arnaud; Fauroux, Brigitte

    2013-07-26

    Noninvasive continuous positive airway pressure (CPAP) is recognized as an effective treatment for severe airway obstruction in young children. The aim of the present study was to compare a clinical setting with a physiological setting of noninvasive CPAP in infants with nocturnal alveolar hypoventilation due to severe upper airway obstruction (UAO) or bronchopulmonary dysplasia (BPD). The breathing pattern and respiratory muscle output of all consecutive infants due to start CPAP in our noninvasive ventilation unit were retrospectively analysed. CPAP set on clinical noninvasive parameters (clinical CPAP) was compared to CPAP set on the normalization or the maximal reduction of the oesophageal pressure (Poes) and transdiaphragmatic pressure (Pdi) swings (physiological CPAP). Expiratory gastric pressure (Pgas) swing was measured. The data of 12 infants (mean age 10 ± 8 mo) with UAO (n = 7) or BPD (n = 5) were gathered. The mean clinical CPAP (8 ± 2 cmH₂O) was associated with a significant decrease in Poes and Pdi swings. Indeed, Poes swing decreased from 31 ± 15 cmH₂O during spontaneous breathing to 21 ± 10 cmH₂O during CPAP (P Poes swing 11 ± 5 cm H₂O; P Poes and Pgas, is superior to a clinical setting, based on clinical noninvasive parameters. Expiratory abdominal activity was present during spontaneous breathing and decreased in the physiological CPAP setting.

  4. Interleukin-4 and 13 concentrations in infants at risk to develop Bronchopulmonary Dysplasia

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    Kruger Thomas E

    2003-08-01

    Full Text Available Abstract Background An exaggerated inflammatory response occurs in the first few days of life in infants who subsequently develop bronchopulmonary dysplasia (BPD. The increase of inflammatory cytokines in many disease processes is generally balanced by a rise in anti-inflammatory cytokines. Interleukin-4 (IL-4 and interleukin-13 (IL-13 have been shown to inhibit production of several inflammatory cytokines important in the development of BPD. Methods We sought to determine if a correlation exists between the presence or absence of IL-4 and IL-13 in tracheal aspirates (TA during the first 3 weeks of life and the development of BPD in premature infants. Serial TAs were prospectively obtained from 36 very low birth weight infants and IL-4 and IL-13 concentrations were determined by ELISA. Results Infants who developed BPD (n = 19 were less mature (25.3 ± 0.02 wks vs. 27.8 ± 0.05 wks; p Conclusions TA concentrations of IL-4 and IL-13 do not increase significantly during acute lung injury in premature infants.

  5. A Time-Based Analysis of Inflammation in Infants at Risk of Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Leroy, Sandrine; Caumette, Elsa; Waddington, Chandra; Hébert, Audrey; Brant, Rollin; Lavoie, Pascal M

    2018-01-01

    To precisely delineate the timing and contribution of inflammation to bronchopulmonary dysplasia (BPD) in preterm infants during the neonatal period. Longitudinal study of blood inflammatory biomarkers (interleukin [IL]-6, IL-8, and granulocyte colony-stimulating factor) measured between birth and 42 days of age, at high temporal (daily) resolution, in infants born at or below 30 weeks of gestation. Cytokine predictors of BPD at 36 weeks postmenstrual age were adjusted for infant-specific and time-dependent factors, using hierarchical mixed effects regressions models. A total of 1518 data points were obtained in 62 infants (mean gestational age of 27 weeks). Infants who developed BPD later on presented increased inflammation after birth compared with infants without BPD. Inflammation was sustained, with gradual attenuation over 2 weeks (IL-8: OR: 6.5 [95% CI: 1.8-24]; granulocyte colony-stimulating factor: 3.3 [1.5-7.6]) and was higher in boys and in infants of lower birth weight. This inflammation preceded the clinical increased requirement in supplemental oxygen characteristic of BPD, and preceded the peak occurrence of neonatal sepsis or necrotizing enterocolitis. Systemic inflammation occurs early in the neonatal period and precedes clinical symptoms in infants with BPD. These data provide a discrete vulnerability window period, supporting a role for targeted intensive care interventions during the early phase of BPD. Copyright © 2017 Elsevier Inc. All rights reserved.

  6. Bronchopulmonary Dysplasia: Chronic Lung Disease of Infancy and Long-Term Pulmonary Outcomes

    Directory of Open Access Journals (Sweden)

    Lauren M. Davidson

    2017-01-01

    Full Text Available Bronchopulmonary dysplasia (BPD is a chronic lung disease most commonly seen in premature infants who required mechanical ventilation and oxygen therapy for acute respiratory distress. While advances in neonatal care have resulted in improved survival rates of premature infants, limited progress has been made in reducing rates of BPD. Lack of progress may in part be attributed to the limited therapeutic options available for prevention and treatment of BPD. Several lung-protective strategies have been shown to reduce risks, including use of non-invasive support, as well as early extubation and volume ventilation when intubation is required. These approaches, along with optimal nutrition and medical therapy, decrease risk of BPD; however, impacts on long-term outcomes are poorly defined. Characterization of late outcomes remain a challenge as rapid advances in medical management result in current adult BPD survivors representing outdated neonatal care. While pulmonary disease improves with growth, long-term follow-up studies raise concerns for persistent pulmonary dysfunction; asthma-like symptoms and exercise intolerance in young adults after BPD. Abnormal ventilatory responses and pulmonary hypertension can further complicate disease. These pulmonary morbidities, combined with environmental and infectious exposures, may result in significant long-term pulmonary sequalae and represent a growing burden on health systems. Additional longitudinal studies are needed to determine outcomes beyond the second decade, and define risk factors and optimal treatment for late sequalae of disease.

  7. An interdisciplinary bronchopulmonary dysplasia program is associated with improved neurodevelopmental outcomes and fewer rehospitalizations.

    Science.gov (United States)

    Shepherd, E G; Knupp, A M; Welty, S E; Susey, K M; Gardner, W P; Gest, A L

    2012-01-01

    Bronchopulmonary dysplasia (BPD) is a pulmonary disease associated with poor neurodevelopmental and medical outcomes. Patients with BPD are medically fragile, at high risk for complications and require interdisciplinary care. We tested the hypothesis that a chronic care approach for BPD would improve neurodevelopmental outcomes relative to the National Institute of Child and Human Development Neonatal Research Network (NICHD NRN) and reduce medical complications. Infants were followed as inpatients and outpatients. Bayley developmental exams were carried out at 18-24 months of age and compared with the NICHD NRN report. Finally, rates of readmission (a proxy for medical complications) were compared before and after implementation of the Comprehensive Center for BPD (CCBPD). Developmental scores obtained in 2007 and 2008 show that 12 and 10% of patients with moderate BPD (n=61) had Bayley Scores <70 for mental and motor indices respectively, whereas corresponding national rates were 35 and 26%. For patients with severe BPD (n=46), 15 and 11% of patients within the CCBPD vs 50 and 42% of national patients scored <70 for mental and motor indices, respectively. Finally, readmission rates dropped from 29% in the year before the implementation of the CCPD (n=269) to 5% thereafter (n=866, P<0.0001). The encouraging neurodevelopmental outcomes and readmission rates associated with a chronic care approach to BPD suggest these infants may be best served by a comprehensive interdisciplinary approach to care that focuses on neurodevelopment throughout the hospital stay.

  8. Pulmonary perfusion scintigraphy in the evaluation of the severity of bronchopulmonary dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Soler, C. [Neonatal Unit, Dept. of Pediatrics, Casa Maternitat Hospital, Clinical Hospital and Sant Joan de Deu Hospital, Univ. of Barcelona (Spain); Figueras, J. [Neonatal Unit, Dept. of Pediatrics, Casa Maternitat Hospital, Clinical Hospital and Sant Joan de Deu Hospital, Univ. of Barcelona (Spain)]|[Servicio de Neonatologia, Hospital Clinico, Barcelona (Spain); Roca, I. [Nuclear Medicine Unit, Autonomous Univ. of Barcelona (Spain); Perez, J.M. [Neonatal Unit, Dept. of Pediatrics, Casa Maternitat Hospital, Clinical Hospital and Sant Joan de Deu Hospital, Univ. of Barcelona (Spain); Jimenez, R. [Neonatal Unit, Dept. of Pediatrics, Casa Maternitat Hospital, Clinical Hospital and Sant Joan de Deu Hospital, Univ. of Barcelona (Spain)

    1997-01-01

    Objective. The objectives of this study were to analyze the changes in pulmonary perfusion in bronchopulmonary dysplasia (BPD) and to assess the advantages of this method in evaluating the severity of BPD. Patients and methods. The study group was made up of 10 children with BPD, matched with a control group of 12 children. The criteria for matching were birth weight, gestational age and need for ventilation for more than 3 days. Clinical and roentgenographic scoring systems were applied on the 21st day of life. At 6 months of corrected age, clinical evolutive severity was evaluated and a pulmonary perfusion scintigraphy using technetium-99 was performed in each child. The scintigraphic findings were classified in five categories ranging from normal to severely affected, depending on the degree and localization of perfusion abnormalities. Another score was obtained by assigning a value from 1 to 5 to each pulmonary lobe, depending on the concentration of the tracer. Results. The study of clinical, roentgenographic and evolutive scores always showed higher values in children with BPD, with good correlation between methods (P < 0.001). In the BPD group, abnormal lung perfusion patterns were more frequent and more severe (P < 0.05), the lobe scoring was higher (P < 0.05), and a lower count rate was found (P < 0.01). Conclusion. Pulmonary scintigraphy is a useful technique in evaluating the severity of BPD. (orig.). With 1 fig., 3 tabs.

  9. Understanding the impact of infection, inflammation and their persistence in the pathogenesis of bronchopulmonary dysplasia

    Directory of Open Access Journals (Sweden)

    Jherna eBalany

    2015-12-01

    Full Text Available The concerted interaction of genetic and environmental factors act on the preterm human immature lung with inflammation being the common denominator leading to the multifactorial origin of the most common chronic lung disease in infants – bronchopulmonary dysplasia or BPD. Adverse perinatal exposure to infection/inflammation with added insults like invasive mechanical ventilation, exposure to hyperoxia and sepsis causes persistent immune dysregulation. In this review article we have attempted to analyze and consolidate current knowledge about the role played by persistent prenatal and postnatal inflammation in the pathogenesis of BPD. While some parameters of the early inflammatory response (neutrophils, cytokines etc. may not be detectable after days to weeks of exposure to noxious stimuli, they have already initiated the signaling pathways of the inflammatory process / immune cascade and have affected permanent defects structurally and functionally in the BPD lungs. Hence translational research aimed at prevention / amelioration of BPD needs to focus on dampening the inflammatory response at an early stage to prevent the cascade of events leading to lung injury with impaired healing resulting in the pathologic pulmonary phenotype of alveolar simplification and dysregulated vascularization characteristic of BPD.

  10. The NLRP3 inflammasome is critically involved in the development of bronchopulmonary dysplasia.

    Science.gov (United States)

    Liao, Jie; Kapadia, Vishal S; Brown, L Steven; Cheong, Naeun; Longoria, Christopher; Mija, Dan; Ramgopal, Mrithyunjay; Mirpuri, Julie; McCurnin, Donald C; Savani, Rashmin C

    2015-11-27

    The pathogenesis of bronchopulmonary dysplasia (BPD), a devastating lung disease in preterm infants, includes inflammation, the mechanisms of which are not fully characterized. Here we report that the activation of the NLRP3 inflammasome is associated with the development of BPD. Hyperoxia-exposed neonatal mice have increased caspase-1 activation, IL1β and inflammation, and decreased alveolarization. Nlrp3(-/-) mice have no caspase-1 activity, no IL1β, no inflammatory response and undergo normal alveolarization. Treatment of hyperoxia-exposed mice with either IL1 receptor antagonist to block IL1β or glyburide to block the Nlrp3 inflammasome results in decreased inflammation and increased alveolarization. Ventilated preterm baboons show activation of the NLRP3 inflammasome with increased IL1β:IL1ra ratio. The IL1β:IL1ra ratio in tracheal aspirates from preterm infants with respiratory failure is predictive of the development of BPD. We conclude that early activation of the NLRP3 inflammasome is a key mechanism in the development of BPD, and represents a novel therapeutic target for BPD.

  11. Differential expression of long non-coding RNAs in hyperoxia-induced bronchopulmonary dysplasia.

    Science.gov (United States)

    Bao, Tian-Ping; Wu, Rong; Cheng, Huai-Ping; Cui, Xian-Wei; Tian, Zhao-Fang

    2016-07-01

    Bronchopulmonary dysplasia (BPD) is a common complication of premature birth that seriously affects the survival rate and quality of life among preterm neonates. Long non-coding RNAs (lncRNAs) have been implicated in many human diseases. However, the role of lncRNAs in the pathogenesis of BPD remains poorly understood. Here, we exposed neonatal C57BL/6J mice to 95% concentrations of ambient oxygen and established a mouse lung injury model that mimicked human BPD. Next, we compared lncRNA and messenger RNA (mRNA) expression profiles between BPD and normal lung tissues using a high-throughput mouse lncRNA + mRNA array system. Compared with the control group, 882 lncRNAs were upregulated, and 887 lncRNAs were downregulated in BPD lung tissues. We validated some candidate BPD-associated lncRNAs by real-time quantitative reverse-transcription polymerase chain reaction analysis in eight pairs of BPD and normal lung tissues. Gene ontology, pathway and bioinformatics analyses revealed that a downregulated lncRNA, namely AK033210, associated with tenascin C may be involved in the pathogenesis of BPD. To the best of our knowledge, our study is the first to reveal differential lncRNA expression in BPD, which provides a foundation for further understanding of the molecular mechanism of BPD development. Copyright © 2016 John Wiley & Sons, Ltd. Copyright © 2016 John Wiley & Sons, Ltd.

  12. Bronchopulmonary Dysplasia: Chronic Lung Disease of Infancy and Long-Term Pulmonary Outcomes

    Science.gov (United States)

    Davidson, Lauren M.; Berkelhamer, Sara K.

    2017-01-01

    Bronchopulmonary dysplasia (BPD) is a chronic lung disease most commonly seen in premature infants who required mechanical ventilation and oxygen therapy for acute respiratory distress. While advances in neonatal care have resulted in improved survival rates of premature infants, limited progress has been made in reducing rates of BPD. Lack of progress may in part be attributed to the limited therapeutic options available for prevention and treatment of BPD. Several lung-protective strategies have been shown to reduce risks, including use of non-invasive support, as well as early extubation and volume ventilation when intubation is required. These approaches, along with optimal nutrition and medical therapy, decrease risk of BPD; however, impacts on long-term outcomes are poorly defined. Characterization of late outcomes remain a challenge as rapid advances in medical management result in current adult BPD survivors representing outdated neonatal care. While pulmonary disease improves with growth, long-term follow-up studies raise concerns for persistent pulmonary dysfunction; asthma-like symptoms and exercise intolerance in young adults after BPD. Abnormal ventilatory responses and pulmonary hypertension can further complicate disease. These pulmonary morbidities, combined with environmental and infectious exposures, may result in significant long-term pulmonary sequalae and represent a growing burden on health systems. Additional longitudinal studies are needed to determine outcomes beyond the second decade, and define risk factors and optimal treatment for late sequalae of disease. PMID:28067830

  13. Membrane and Capillary Components of Lung Diffusion in Infants with Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Chang, Daniel V; Assaf, Santiago J; Tiller, Christina J; Kisling, Jeffrey A; Tepper, Robert S

    2016-04-01

    Autopsied lungs of infants with bronchopulmonary dysplasia (BPD) demonstrate impaired alveolar development with larger and fewer alveoli, which is consistent with our previous physiologic findings of lower pulmonary diffusing capacity of the lung for carbon monoxide (DL(CO)) in infants and toddlers with BPD compared with healthy controls born at full term (FT). However, it is not known whether the decreased DL(CO) in infants with BPD results from a reduction in both components of DL(CO): pulmonary membrane diffusing capacity (D(M)) and Vc. We hypothesized that impairment of alveolar development in BPD results in a decrease in both D(M) and Vc components of DlCO but that the D(M)/Vc ratio would not differ between the BPD and FT groups. DL(CO) was measured under conditions of room air and high inspired oxygen (90%), which enabled D(M) and Vc to be calculated. D(M) and Vc increased with increasing body length; however, infants with BPD had significantly lower D(M) and Vc than FT subjects after adjustment for race, sex, body length, and corrected age. In contrast to D(M) and Vc, the D(M)/Vc ratio remained constant with increasing body length and did not differ for infants with BPD and FT subjects. Our findings are consistent with infants with BPD having impaired alveolar development with fewer but larger alveoli, as well as a reduced Vc.

  14. Lung CT imaging in patients with bronchopulmonary dysplasia: A systematic review.

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    van Mastrigt, Esther; Logie, Karla; Ciet, Pierluigi; Reiss, Irwin K M; Duijts, Liesbeth; Pijnenburg, Mariëlle W; Tiddens, Harm A W M

    2016-09-01

    Bronchopulmonary dysplasia (BPD) is a common respiratory complication of preterm birth and associated with long-term respiratory sequelae. Chest computed tomography (CT) is a sensitive tool to obtain insight in structural lung abnormalities and may be a predictor for later symptoms. To give an overview of chest CT scoring methods that are used to evaluate chest CT scans of BPD patients. To review which structural lung abnormalities are described in children and adults with BPD and whether these are related to clinical outcomes. An extensive literature search was conducted for relevant studies on chest CT imaging in patients born preterm with BPD. We retrieved 316 original papers of which 16 articles and three abstracts fulfilled our inclusion criteria. Overall, we identified nine different semi-quantitative scoring methods. Chest CT scans revealed structural abnormalities in >85% of BPD patients. These abnormalities are decreased pulmonary attenuation, opacities, bronchial wall thickening, and consolidations. Some have been found to be negatively correlated with lung function and respiratory symptoms. None of the currently described scoring systems are appropriately validated or superior over another. Future studies are needed to generate a validated and universal chest CT quantitative scoring method for patients with BPD. Pediatr Pulmonol. 2016; 51:975-986. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  15. A Relationship between Epithelial Maturation, Bronchopulmonary Dysplasia, and Chronic Obstructive Pulmonary Disease

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    Roos, Abraham B.; Berg, Tove; Nord, Magnus

    2012-01-01

    Premature infants frequently develop bronchopulmonary dysplasia (BPD). Lung immaturity and impaired epithelial differentiation contribute together with invasive oxygen treatment to BPD onset and disease progression. Substantial evidence suggests that prematurity is associated with long term pulmonary consequences. Moreover, there is increasing concern that lung immaturity at birth may increase the risk of developing chronic obstructive pulmonary disease (COPD). The mechanisms contributing to this phenomenon remains unknown, largely as a consequence of inadequate experimental models and clinical follow-up studies. Recent evidence suggests that defective transcriptional regulation of epithelial differentiation and maturation may contribute to BPD pathogenesis as well as early onset of COPD. The transcriptional regulators CCAAT/enhancer-binding protein (C/EBP)α and C/EBPβ, SMAD family member (Smad)3, GATA binding protein (GATA)6, and NK2 homeobox (NKX)2-1 are reported to be involved in processes contributing to pathogenesis of both BPD and COPD. Increased knowledge of the mechanisms contributing to early onset COPD among BPD survivors could translate into improved treatment strategies and reduced frequency of respiratory disorders among adult survivors of BPD. In this paper, we introduce critical transcriptional regulators in epithelial differentiation and summarize the current knowledge on the contribution of impaired epithelial maturation to the pathogenesis of inflammatory lung disorders. PMID:23320163

  16. Recent advances in the mechanisms of lung alveolarization and the pathogenesis of bronchopulmonary dysplasia.

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    Silva, Diogo M G; Nardiello, Claudio; Pozarska, Agnieszka; Morty, Rory E

    2015-12-01

    Alveolarization is the process by which the alveoli, the principal gas exchange units of the lung, are formed. Along with the maturation of the pulmonary vasculature, alveolarization is the objective of late lung development. The terminal airspaces that were formed during early lung development are divided by the process of secondary septation, progressively generating an increasing number of alveoli that are of smaller size, which substantially increases the surface area over which gas exchange can take place. Disturbances to alveolarization occur in bronchopulmonary dysplasia (BPD), which can be complicated by perturbations to the pulmonary vasculature that are associated with the development of pulmonary hypertension. Disturbances to lung development may also occur in persistent pulmonary hypertension of the newborn in term newborn infants, as well as in patients with congenital diaphragmatic hernia. These disturbances can lead to the formation of lungs with fewer and larger alveoli and a dysmorphic pulmonary vasculature. Consequently, affected lungs exhibit a reduced capacity for gas exchange, with important implications for morbidity and mortality in the immediate postnatal period and respiratory health consequences that may persist into adulthood. It is the objective of this Perspectives article to update the reader about recent developments in our understanding of the molecular mechanisms of alveolarization and the pathogenesis of BPD. Copyright © 2015 the American Physiological Society.

  17. Persistent pulmonary abnormalities in newborns: The changing picture of bronchopulmonary dysplasia

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    Heneghan, M.A.; Baquero, J.M.; Sosulski, R.

    1986-03-01

    Significant changes in the radiographic features of bronchopulmonary dysplasia (BPD) have accompanied recent advances in treatment of neonatal respiratory distress syndrome. Retrospective study of 709 newborns showed atypical radiographic findings in many patients with clinical BPD. While 12/20 infants with clinical BPD showed changes identical to Northway's stage 4 disease, the remaining 8 (40% of patients with significant respiratory dysfunction) had diffuse, fine infiltrates without emphysema. Radiographic progression from RDS through all Northway stages was observed in only 4 patients. Diagnosis of stage 2 BPD was complicated by the presence of PDA in 9/17 cases. Stage 3 BPD was identified with certainty in only 5 infants, but may have coexisted with PIE in as many as 22 cases. Nevertheless, there was close agreement between the radiographic findings and clinical severity of chronic lung disease. Mild (type 1) infiltrates following RDS may be distinguished from chronic pulmonary insufficiency of prematurity (CPIP) or ''immature lung''. In patients who require only short-term supplemental 02, type 1 changes may reflect delayed resolution of RDS in an undeveloped lung. These same findings in infants with prolonged 02 dependence usually indicate a mild form of BPD. Coarse infiltrates and emphysema (type 2) are almost always associated with severe respiratory impairment.

  18. Ultrafast CT scoring system for assessing bronchopulmonary dysplasia. Reproducibility and clinical correlation

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    Kubota, Jun; Ohki, Yasushi; Inoue, Tomio; Mochizuki, Hiroyuki; Aoki, Jun; Morikawa, Akihiro; Endo, Keigo [Gunma Univ., Maebashi (Japan). School of Medicine; Sakurai, Minako; Shigeta, Makoto

    1998-05-01

    To evaluate the reproducibility of the Ultrafast CT (UFCT) scoring system and assess its usefulness in monitoring clinical severity in infants with bronchopulmonary dysplasia (BPD). UFCT scoring was done in 22 infants (15 boys and 7 girls aged 1 to 37 months) with BPD. A total of 258 lung fields were evaluated for the presence of hyperaeration, linear opacities, triangular subpleural opacities, and bronchovascular bundle distortion or thickening, and UFCT scores were given. Intraobserver and interobserver agreement and reproducibility of UFCT scores were statistically analyzed. In 12 patients, UFCT scores were linearly correlated with clinical severity scores based on respiratory dysfunction and complexity of care. `Hyperaeration,` which was the most frequent (18 of 22, 81.8%) finding, showed high concordance ({kappa}=0.73, p<0.001, {kappa}=0.59, p<0.001), and its UFCT scores significantly correlated with intraobserver and interobserver analyses (r=0.94, p<0.001, r=0.82, p<0.001, respectively). UFCT scores for hyperaeration significantly correlated with clinical scores (r=0.75, p<0.01), whereas those for the others did not. UFCT is useful for assessing BPD. Hyperaeration was the most common and reproducible finding, and its extent significantly correlated with clinical severity. (author)

  19. Hydrogen-rich water ameliorates bronchopulmonary dysplasia (BPD) in newborn rats.

    Science.gov (United States)

    Muramatsu, Yukako; Ito, Mikako; Oshima, Takahiro; Kojima, Seiji; Ohno, Kinji

    2016-09-01

    Bronchopulmonary dysplasia (BPD) is characterized by developmental arrest of the alveolar tissue. Oxidative stress is causally associated with development of BPD. The effects of hydrogen have been reported in a wide range of disease models and human diseases especially caused by oxidative stress. We made a rat model of BPD by injecting lipopolysaccharide (LPS) into the amniotic fluid at E16.5. The mother started drinking hydrogen-rich water from E9.5 and also while feeding milk. Hydrogen normalized LPS-induced abnormal enlargement of alveoli at P7 and P14. LPS increased staining for nitrotyrosine and 8-OHdG of the lungs, and hydrogen attenuated the staining. At P1, LPS treatment decreased expressions of genes for FGFR4, VEGFR2, and HO-1 in the lungs, and hydrogen increased expressions of these genes. In contrast, LPS treatment and hydrogen treatment had no essential effect on the expression of SOD1. Inflammatory marker proteins of TNFα and IL-6 were increased by LPS treatment, and hydrogen suppressed them. Treatment of A549 human lung adenocarcinoma epithelial cells with 10% hydrogen gas for 24 hr decreased production of reactive oxygen species in both LPS-treated and untreated cells. Lack of any known adverse effects of hydrogen makes hydrogen a promising therapeutic modality for BPD. Pediatr Pulmonol. 2016; 51:928-935. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  20. Animal models of bronchopulmonary dysplasia. The preterm and term rabbit models.

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    D'Angio, Carl T; Ryan, Rita M

    2014-12-15

    Bronchopulmonary dysplasia (BPD) is an important lung developmental pathophysiology that affects many premature infants each year. Newborn animal models employing both premature and term animals have been used over the years to study various components of BPD. This review describes some of the neonatal rabbit studies that have contributed to the understanding of BPD, including those using term newborn hyperoxia exposure models, premature hyperoxia models, and a term newborn hyperoxia model with recovery in moderate hyperoxia, all designed to emulate aspects of BPD in human infants. Some investigators perturbed these models to include exposure to neonatal infection/inflammation or postnatal malnutrition. The similarities to lung injury in human premature infants include an acute inflammatory response with the production of cytokines, chemokines, and growth factors that have been implicated in human disease, abnormal pulmonary function, disordered lung architecture, and alveolar simplification, development of fibrosis, and abnormal vascular growth factor expression. Neonatal rabbit models have the drawback of limited access to reagents as well as the lack of readily available transgenic models but, unlike smaller rodent models, are able to be manipulated easily and are significantly less expensive than larger animal models. Copyright © 2014 the American Physiological Society.

  1. Role of the Nrf2/HO-1 axis in bronchopulmonary dysplasia and hyperoxic lung injuries.

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    Amata, Emanuele; Pittalà, Valeria; Marrazzo, Agostino; Parenti, Carmela; Prezzavento, Orazio; Arena, Emanuela; Nabavi, Seyed Mohammad; Salerno, Loredana

    2017-07-15

    Bronchopulmonary dysplasia (BPD) is a chronic illness that usually originates in preterm newborns. Generally, BPD is a consequence of respiratory distress syndrome (RDS) which, in turn, comes from the early arrest of lung development and the lack of pulmonary surfactant. The need of oxygen therapy to overcome premature newborns' compromised respiratory function generates an increasing amount of reactive oxygen species (ROS), the onset of sustained oxidative stress (OS) status, and inflammation in the pulmonary alveoli deputies to respiratory exchanges. BPD is a severe and potentially life-threatening disorder that in the most serious cases, can open the way to neurodevelopmental delay. More importantly, there is no adequate intervention to hamper or treat BPD. This perspective article seeks to review the most recent and relevant literature describing the very early stages of BPD and hyperoxic lung injuries focussing on nuclear factor erythroid derived 2 (Nrf2)/heme oxygenase-1 (HO-1) axis. Indeed, Nrf2/HO1 activation in response to OS induced lung injury in preterm concurs to the induction of certain number of antioxidant, anti-inflammatory, and detoxification pathways that seem to be more powerful than the activation of one single antioxidant gene. These elicited protective effects are able to counteract/mitigate all multifaceted aspects of the disease and may support novel approaches for the management of BPD. © 2017 The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society.

  2. Mechanisms of cough provocation and cough resolution in neonates with bronchopulmonary dysplasia.

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    Jadcherla, Sudarshan R; Hasenstab, Kathryn A; Shaker, Reza; Castile, Robert G

    2015-10-01

    Cough and deglutition are protective mechanisms that defend against aspiration. We identified mechanisms associated with cough provocation as well as those associated with cough resolution in infants with bronchopulmonary dysplasia (BPD). Manometry signatures of cough were recognized in 16 premature infants with BPD undergoing concurrent esophageal manometry, respiratory inductance plethysmography, and nasal air flow measurements. Pretussive and post-tussive pharyngo-esophageal motility changes were analyzed. Mechanisms associated with cough and mechanisms that restored respiratory and esophageal normalcy were analyzed. We analyzed 312 cough events during 88 cough clusters; 97% were associated with recognizable manometric patterns. Initial mechanisms related with coughing included nonpropagating swallow (59%), upper esophageal sphincter (UES) reflex contraction (18%), and lower esophageal sphincter (LES) relaxation (14%). UES and LES dysfunction was present in 69% of nonpropagating swallow-associated cough clusters. Mechanisms restoring post-tussive normalcy included primary peristalsis (84%), secondary peristalsis (8%), and none recognized (8%). UES contraction reflex was associated with cough clusters more frequently in infants on nasal continuous positive airway pressure (NCPAP) (OR = 9.13, 95% CI = 1.88-44.24). Cough clusters in infants with BPD had identifiable etiologies associated with esophageal events; common initial mechanisms were of upper aerodigestive origin, while common clearing mechanisms were peristaltic reflexes.

  3. Disrupted Lung Development and Bronchopulmonary Dysplasia: Opportunities for Lung Repair and Regeneration

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    Baker, Christopher D.; Alvira, Cristina M.

    2014-01-01

    Purpose of review Advances of medical therapy have increased survival of extremely premature infants, and changed the pathology of bronchopulmonary dysplasia (BPD) from one of acute lung injury to a disease of disrupted lung development. With this evolution, new questions emerge regarding: (i) the molecular mechanisms that control postnatal lung development; (ii) the effect of early disruptions of postnatal lung development on long-term lung function; and (iii) the existence of endogenous mechanisms that permit lung regeneration after injury. Recent Findings Recent data demonstrate that a significant component of alveolarization, the final stage of lung development, occurs postnatally. Further, clinical and experimental studies demonstrate that premature birth disrupts alveolarization, decreasing the gas exchange surface area of the lung and causing BPD. BPD is associated with significant short-term morbidity, and new longitudinal, clinical data demonstrate that survivors of BPD have long-standing deficits in lung function, and may be at risk for the development of additional lung disease as adults. Unfortunately, current care is mainly supportive with few effective therapies that prevent or treat established BPD. These studies underscore the need to further elucidate the mechanisms that direct postnatal lung growth, and develop innovative strategies to stimulate lung regeneration. Summary Despite significant improvements in the care and survival of extremely premature infants, BPD remains a major clinical problem. While efforts should remain focused on the prevention of preterm labor and BPD, novel research aimed at promoting postnatal alveolarization offers a unique opportunity to develop effective strategies to treat established BPD. PMID:24739494

  4. Association of a FGFR-4 Gene Polymorphism with Bronchopulmonary Dysplasia and Neonatal Respiratory Distress

    Directory of Open Access Journals (Sweden)

    Milad Rezvani

    2013-01-01

    Full Text Available Background. Bronchopulmonary dysplasia (BPD is the most common chronic lung disease of premature birth, characterized by impaired alveolar development and inflammation. Pathomechanisms contributing to BPD are poorly understood. However, it is assumed that genetic factors predispose to BPD and other pulmonary diseases of preterm neonates, such as neonatal respiratory distress syndrome (RDS. For association studies, genes upregulated during alveolarization are major candidates for genetic analysis, for example, matrix metalloproteinases (MMPs and fibroblast growth factors (FGFs and their receptors (FGFR. Objective. Determining genetic risk variants in a Caucasian population of premature neonates with BPD and RDS. Methods. We genotyped 27 polymorphisms within 14 candidate genes via restriction fragment length polymorphism (RFLP: MMP-1, -2, -9, and -12, -16, FGF receptors 2 and 4, FGF-2, -3, -4, -7, and -18, Signal-Regulatory Protein α (SIRPA and Thyroid Transcription Factor-1 (TTF-1. Results. Five single nucleotide polymorphisms (SNPs in MMP-9, MMP-12, FGFR-4, FGF-3, and FGF-7 are associated ( with RDS, defined as surfactant application within the first 24 hours after birth. One of them, in FGFR-4 (rs1966265, is associated with both RDS ( and BPD (. Conclusion. rs1966265 in FGF receptor 4 is a possible genetic key variant in alveolar diseases of preterm newborns.

  5. Updates on functional characterisation of bronchopulmonary dysplasia – the contribution of lung function testing

    Directory of Open Access Journals (Sweden)

    Anne eGreenough

    2015-05-01

    Full Text Available Bronchopulmonary dysplasia (BPD is a chronic lung disease that predominantly affects prematurely born infants. Initially BPD was described in infants who had suffered severe respiratory failure and required high pressure, mechanical ventilation with high concentrations of supplementary oxygen. It now also occurs in very prematurely born infants who initially had minimal or even no signs of lung disease. These differences impact on the nature of the lung function abnormalities suffered by BPD infants, which is also influenced by the criteria used to diagnose BPD and the oxygen saturation level used to determine the supplementary oxygen requirement. Key also to interpreting lung function data in this population is whether appropriate lung function tests have been used and in an adequately sized population to make meaningful conclusions. It should also be emphasised that BPD is a poor predictor of long term respiratory morbidity. Bearing in mind those caveats, studies have consistently demonstrated that infants who develop BPD have low compliance and functional residual capacities and raised resistances in the neonatal period. There is, however, no agreement with regard to which early lung function measurement predicts the development of BPD, likely reflecting different techniques were used in different populations in often underpowered studies. During infancy, lung function generally improves, but importantly airflow limitation persists and small airway function appears to decline. Assessment of lung function has demonstrated that improvements in lung function following diuretics or bronchodilators have not translated into long term improvements in respiratory outcomes. In contrast, early differences in lung function related to different ventilation modes have led to investigation and demonstration that prophylactic, neonatal high frequency oscillation appears to protect small airway function.

  6. Special nutritional needs of infants for prevention of and recovery from bronchopulmonary dysplasia.

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    Atkinson, S A

    2001-03-01

    Extremely low birth weight infants who develop severe respiratory disease may have special nutrient requirements imposed by a combination of enhanced utilization of nutrients or the need for epithelial cell repair resulting from the disease process, as well as to support catch-up growth. Inositol, free fatty acids, vitamin E and vitamin A are proposed as nutrients for which infants at risk of chronic pulmonary insufficiency may have special requirements. Of these nutrients, only for vitamin A does suggestive evidence exist that high doses when given intramuscularly may reduce the incidence of death or chronic lung disease. Exogenous steroid therapy (dexamethasone), which is often used to improve pulmonary compliance in ventilated premature infants, may compromise vitamin A status and induce restricted somatic and bone mineral growth. Supplemental nutrition by means of enriched infant formulas has provided benefits in growth and bone mass accretion to infants recovering from bronchopulmonary dysplasia up to 3-mo corrected age. This growth advantage was not sustained over the subsequent 9 mo, suggesting that prolonged nutritional support is required until catch-up growth is complete. Further studies are required to delineate the needs for specific nutrients such as antioxidant vitamins and minerals or vitamin A that may play a role in preventing severe chronic lung disease in premature infants. As well, the role of supplemental nutrition (beyond the requirements of term infants) to support catch-up growth and maintenance during the critical stages of early development requires further investigation before evidence-based nutrient recommendations can be developed for this special population of infants.

  7. Psychoeducational outcome at school age of preterm infants with bronchopulmonary dysplasia.

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    Gray, P H; O'Callaghan, M J; Rogers, Y M

    2004-03-01

    To investigate the cognitive performance and educational attainment at school-age of children with bronchopulmonary dysplasia (BPD), compared with a preterm control group of children. Seventy preterm infants with BPD and 61 birth weight matched controls were prospectively followed-up to school-age. The Weschler Intelligence Scale for Children - III (WISC), the Wide Range Achievement Test (WRAT) and the Developmental Test of Visual Motor Integration (VMI) were administered. The results were compared between the two groups and multiple regression analyses were performed to determine the effect of confounding variables. The children in the BPD group performed less well on the Full Scale IQ (mean 86.7 vs 93.5; 95% CI, 1.9-11.7), Verbal IQ (mean 87.1 vs 94.1; 95% CI, 2.0-12.0) and the Performance IQ (mean 88.6 vs 95.2; 95% CI, 2.0-11.2) of the WISC, the reading component of the WRAT (mean 93.8 vs 98.9; 95% CI, 0.3-9.8) and the VMI (mean 88.9 vs 93.3; 95%, CI 1.1-7.8). Despite controlling for social and biological variables, statistical differences persisted for Full Scale and Verbal IQ and reading. A Verbal IQ >1 SD below the mean was found in 41% of BPD children compared to 21% of controls, while on the reading component of the WRAT a greater proportion of BPD children also had scores>1 SD below the mean. Impaired psychoeducational performance was found in preterm children with BPD compared to controls, especially in the areas of language abilities and reading skills. This supports a greater need for special educational services and counseling for parents for these children.

  8. Modelling bronchopulmonary dysplasia in animals: arguments for the preterm rabbit model.

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    Salaets, Thomas; Gie, Andre; Tack, Bieke; Deprest, Jan; Toelen, Jaan

    2017-09-26

    Bronchopulmonary dysplasia (BPD) remains a frequent and disabling consequence of preterm birth, despite the recent advances in neonatal intensive care. There is a need to further improve outcomes and many novel therapeutic or preventive strategies are therefore investigated in animal models. We discuss in this review the aspects of human BPD pathophysiology and phenotype, which ideally should be mimicked by an animal model for this disease. Prematurity remains the common denominator in the heterogeneous spectrum of human BPD, and preterm animal models thus have a clear translational advantage. Additional factors, like excessive oxygen, mechanical ventilation and infection, which frequently have been studied in animal models, can contribute to preterm lung injury however are not indispensable to develop BPD. The phenotype of human BPD is characterized by alveolar developmental arrest with extracellular matrix remodeling, signs of obstructive airway disease and pulmonary vascular disease. Many animal models mimic this phenotype and have their place in BPD research, but results should be interpreted bearing in mind the specific advantages and disadvantages of the model. Term mice and rats are well suited for basic explorative research on specific disease mechanisms, essential for the generation of new hypotheses, while the larger ventilated preterm baboons and lambs provide a good platform for the ultimate translation of these strategies towards clinical application. The preterm rabbit model seems a promising model as it the smallest model that includes a factor of prematurity and has a unique position between the small and large animal models. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  9. Persistently elevated right ventricular index of myocardial performance in preterm infants with incipient bronchopulmonary dysplasia.

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    Christoph Czernik

    Full Text Available OBJECTIVES: Elevated pulmonary vascular resistance occurs during the first days after birth in all newborn infants and persists in infants at risk for bronchopulmonary dysplasia (BPD. It is difficult to measure in a non-invasive fashion. We assessed the usefulness of the right ventricular index of myocardial performance (RIMP to estimate pulmonary vascular resistance in very low birth weight infants. STUDY DESIGN: Prospective echocardiography on day of life (DOL 2, 7, 14, and 28 in 121 preterm infants (median [quartiles] gestational age 28 [26]-[29] weeks, birth weight 998 [743-1225] g of whom 36 developed BPD (oxygen supplementation at 36 postmenstrual weeks. RESULTS: RIMP derived by conventional pulsed Doppler technique was unrelated to heart rate or mean blood pressure. RIMP on DOL 2 was similar in infants who subsequently did (0.39 [0.33-0.55] and did not develop BPD (0.39 [0.28-0.51], p = 0.467. RIMP declined steadily in non-BPD infants but not in BPD infants (DOL 7: 0.31[0.22-0.39] vs. 0.35[0.29-0.48], p = 0.014; DOL 14: 0.23[0.17-0.30] vs. 0.35[0.25-0.43], p<0.001; DOL 28: 0.21[0.15-0.28] vs. 0.31 [0.21-0.35], p = 0.015. CONCLUSIONS: In preterm infants, a decline in RIMP after birth was not observed in those with incipient BPD. The pattern of RIMP measured in preterm infants is commensurate with that of pulmonary vascular resistance.

  10. Azithromycin and other macrolides for prevention of bronchopulmonary dysplasia: a systematic review and meta-analysis.

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    Nair, Vrinda; Loganathan, Prakash; Soraisham, Amuchou Singh

    2014-01-01

    Ureaplasma spp. infection has been associated with bronchopulmonary dysplasia (BPD) in preterm infants. Macrolides have been used for the treatment of Ureaplasma spp. infection, with an intention to prevent BPD. The objective of this meta-analysis is to evaluate the use of macrolides in the prevention of BPD in preterm infants. We searched MEDLINE, EMBASE, and Cochrane Central Register of Controlled Trials, abstracts of the major pediatric society meetings and bibliographies of retrieved articles. We included randomized controlled trials assessing the effects of macrolides therapy on BPD in preterm infants. A random/fixed-effect model was used to synthesize predefined outcomes. Six studies involving 469 preterm infants were eligible for the analysis. Macrolides when used prophylactically (4 studies) did not show significant reduction in BPD (risk ratio, RR, 0.88, 95% confidence interval, CI, 0.75-1.03), death (RR 0.89, 95% CI 0.79-1.01) or in the composite outcome of BPD/death. Similarly, there was no significant reduction in BPD (RR 0.64, 95% CI 0.31-1.31) or the composite outcome of BPD/death (RR 0.41, 95% CI 0.05-3.13), when macrolides were used in Ureaplasma-positive infants. However, prophylactic azithromycin therapy (3 studies) was associated with significant reduction in BPD (RR 0.83, 95% CI 0.71-0.97; number needed to treat, NNT, 10) and composite outcome of BPD/death (RR 0.86, 95% CI 0.77-0.97; NNT 10). This meta-analysis demonstrates prophylactic azithromycin therapy was associated with statistically significant reduction in BPD and the composite outcome of BPD/death in preterm infants. However, given the limited information on pharmacokinetics and potential harmful effects, further studies should be done before routine use of azithromycin in the neonatal population.

  11. MicroRNA in late lung development and bronchopulmonary dysplasia: the need to demonstrate causality.

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    Nardiello, Claudio; Morty, Rory E

    2016-12-01

    MicroRNA are emerging as powerful regulators of cell differentiation and tissue and organ development. Several microRNA have been described to play a role in branching morphogenesis, a key step in early lung development. However, considerably less attention has been paid to microRNA as regulators of the process of secondary septation, which drives lung alveolarization during late lung development. Secondary septation is severely perturbed in bronchopulmonary dysplasia (BPD), a common complication of preterm birth characterized by blunted alveolarization. A number of studies to date have reported microRNA microarray screens in animal models of BPD; however, only two studies have attempted to demonstrate causality. Although the expression of miR-150 was altered in experimental BPD, a miR-150(-/-) knockout mouse did not exhibit appreciable protection in a BPD animal model. Similarly, while the expression of miR-489 in the lung was reduced in clinical and experimental BPD, antagomiR and over-expression approaches could not validate a role for miR-489 in the impaired alveolarization associated with experimental BPD. This mini-review aims to highlight microRNA that have been revealed by multiple microarray studies to be potential causal players in normal and pathological alveolarization. Additionally, the challenges faced in attempting to demonstrate a causal role for microRNA in lung alveolarization are discussed. These include the tremendous variability in the animal models employed, and the limitations and advantages offered by the available tools, including antagomiRs and approaches for the validation of a specific microRNA-mRNA interaction during lung alveolarization.

  12. Ascorbylperoxide Contaminating Parenteral Nutrition Is Associated With Bronchopulmonary Dysplasia or Death in Extremely Preterm Infants.

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    Mohamed, Ibrahim; Elremaly, Wesam; Rouleau, Thérèse; Lavoie, Jean-Claude

    2017-08-01

    Ascorbylperoxide (AscOOH) is a hydrogen peroxide-dependent by-product of ascorbic acid that contaminates parenteral nutrition. In a guinea pig model, it caused oxidized redox potential, increased apoptosis, and decreased alveolarization. AscOOH detoxification is carried out by glutathione peroxidase (GPX). We hypothesize that extremely preterm infants have limited capacity for AscOOH detoxification. Our objective was to determine if there is an association between an early level of urinary AscOOH and later development of bronchopulmonary dysplasia (BPD) or death. This prospective cohort study included 51 infants at <29 weeks of gestation. Baseline clinical characteristics and clinical outcomes data were collected. Urine samples were collected on days 3, 5, and 7 of life for urinary AscOOH. Blood samples on day 7 were collected for total plasma glutathione, GPX, and glutathione reductase. χ(2), Student's t test, Spearman correlation ( r), linear regression (adjusted r(2)), and repeated-measure analysis of variance were used as appropriate. P < .05 was considered significant. Urinary AscOOH increased over time ( P = .001) and was higher in infants who later developed BPD or died ( P = .037). Compared with adults and full-term infants, total plasma glutathione concentration was low (median, 1.02 µmol/L; 25th-75th percentiles, 0.49-1.76 µmol/L), whereas GPX and glutathione reductase activities were sufficient (3.98 ± 1.25 and 0.36 ± 0.01 nmol/min/mg of protein, respectively). Extremely preterm infants have low glutathione levels, which limit their capacity to detoxify AscOOH. Higher first-week urinary AscOOH levels are associated with an increased incidence of BPD or death.

  13. IFN-γ and IP-10 in tracheal aspirates from premature infants: relationship with bronchopulmonary dysplasia.

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    Aghai, Zubair H; Saslow, Judy G; Mody, Kartik; Eydelman, Riva; Bhat, Vishwanath; Stahl, Gary; Pyon, Kee; Bhandari, Vineet

    2013-01-01

    Interferon-gamma (IFN-γ) and interferon-inducible protein of 10 kDa (IP-10) are potent inflammatory mediators and contribute to acute lung injury in adults. Recently, a potential role for IFN-γ and IP-10 in the pathogenesis of bronchopulmonary dysplasia (BPD) has been reported in animal models. To study the association between IFN-γ and IP-10 in tracheal aspirate (TA) and the development of BPD in premature infants. TA samples collected within 48 hr after birth from 79 mechanically ventilated premature neonates [gestational age (GA) IP-10 was determined using a commercially available ELISA kit. Total protein in TA was measured by Bradford assay to correct for sampling related dilution. BPD was defined as the need of supplemental oxygen at 36 weeks postmenstrual age (PMA). Twenty infants (GA 26.4 ± 1.9w, BW 860 ± 201 g) survived without BPD at 36 weeks PMA and 59 infants (GA 25.5 ± 1.5w, BW 751 ± 163 g) died before 36 weeks PMA or developed BPD. The mean IFN-γ level was higher in infants who died or developed BPD (9.7 ± 2.8 vs. 3.1 ± 1.1 pg/ml, P = 0.03). Similarly, the mean IP-10 level was higher in infants who died or developed BPD (63.4 ± 17.5 pg/ml) compared to those who survived without BPD (18.5 ± 7.5 pg/ml, P = 0.02). Higher IFN-γ and IP-10 levels in TA samples are associated with the development of BPD or death in premature infants. Copyright © 2012 Wiley Periodicals, Inc.

  14. Treatment strategies for bronchopulmonary dysplasia with postnatal corticosteroids in Europe: the EURAIL survey.

    Science.gov (United States)

    Truffert, P; Empana, J P; Bréart, G; Saugstad, O D; Goelz, R; Halliday, H L; Anceschi, M

    2003-08-01

    To survey practices in 14 European countries and describe strategies for the prevention and treatment of bronchopulmonary dysplasia with postnatal steroids (PNS). In 1999-2000 questionnaires covering the use of PNS were sent to every neonatal unit taking very preterm newborns in charge, in population-based areas covering at least 20000 births annually. One questionnaire was sent to surveyed unit. The participating areas were chosen by an expert from each country participating in the Europe Against Immature Lung (EURAIL) study group. Responses to 331 questionnaires were received; the mean response rate by countries was 84% (range 64-100%). Teaching hospitals accounted for 19% of the responding units. The number of extremely premature newborns (less than 28 wk of gestation) admitted yearly to these units was 0 in 16%, 39 in 11%. Overall, 67% of the centres used PNS: 48% initiated treatment in non-intubated infants and 53% at 7-14 d. Treatment duration was 4-15 d in 62% and > 15 d in 21%. PNS administration was limited to intubated infants less often in smaller units [odds ratio (OR) 0.2, 95% confidence interval (95% CI) 0.1-0.6] and more often in non-teaching hospitals (OR 2.5, 95% CI 2.5-5.0). Although PNS have important side effects, they were still widely used in 1999 to treat or prevent chronic lung disease. Surprisingly, steroids are still prescribed in non-ventilated infants. PNS use should be based on guidelines derived from the evidence from randomized controlled trials. This evidence should be regularly updated and disseminated.

  15. Quantitative Magnetic Resonance Imaging of Bronchopulmonary Dysplasia in the Neonatal Intensive Care Unit Environment.

    Science.gov (United States)

    Walkup, Laura L; Tkach, Jean A; Higano, Nara S; Thomen, Robert P; Fain, Sean B; Merhar, Stephanie L; Fleck, Robert J; Amin, Raouf S; Woods, Jason C

    2015-11-15

    Bronchopulmonary dysplasia (BPD) is a prevalent yet poorly characterized pulmonary complication of premature birth; the current definition is based solely on oxygen dependence at 36 weeks postmenstrual age without objective measurements of structural abnormalities across disease severity. We hypothesize that magnetic resonance imaging (MRI) can spatially resolve and quantify the structural abnormalities of the neonatal lung parenchyma associated with premature birth. Using a unique, small-footprint, 1.5-T MRI scanner within our neonatal intensive care unit (NICU), diagnostic-quality MRIs using commercially available sequences (gradient echo and spin echo) were acquired during quiet breathing in six patients with BPD, six premature patients without diagnosed BPD, and six full-term NICU patients (gestational ages, 23-39 wk) at near term-equivalent age, without administration of sedation or intravenous contrast. Images were scored by a radiologist using a modified Ochiai score, and volumes of high- and low-signal intensity lung parenchyma were quantified by segmentation and threshold analysis. Signal increases, putatively combinations of fibrosis, edema, and atelectasis, were present in all premature infants. Infants with diagnosed BPD had significantly greater volume of high-signal lung (mean ± SD, 26.1 ± 13.8%) compared with full-term infants (7.3 ± 8.2%; P = 0.020) and premature infants without BPD (8.2 ± 6.4%; P = 0.026). Signal decreases, presumably alveolar simplification, only appeared in the most severe BPD cases, although cystic appearance did increase with severity. Pulmonary MRI reveals quantifiable, significant differences between patients with BPD, premature patients without BPD, and full-term control subjects. These methods could be implemented to individually phenotype disease, which may impact clinical care and predict future outcomes.

  16. Exome Sequencing of Neonatal Blood Spots and the Identification of Genes Implicated in Bronchopulmonary Dysplasia

    Science.gov (United States)

    Li, Jingjing; Yu, Kun-Hsing; Oehlert, John; Jeliffe-Pawlowski, Laura L.; Gould, Jeffrey B.; Stevenson, David K.; Snyder, Michael; Shaw, Gary M.

    2015-01-01

    Rationale: Bronchopulmonary dysplasia (BPD), a prevalent severe lung disease of premature infants, has a strong genetic component. Large-scale genome-wide association studies for common variants have not revealed its genetic basis. Objectives: Given the historical high mortality rate of extremely preterm infants who now survive and develop BPD, we hypothesized that risk loci underlying this disease are under severe purifying selection during evolution; thus, rare variants likely explain greater risk of the disease. Methods: We performed exome sequencing on 50 BPD-affected and unaffected twin pairs using DNA isolated from neonatal blood spots and identified genes affected by extremely rare nonsynonymous mutations. Functional genomic approaches were then used to systematically compare these affected genes. Measurements and Main Results: We identified 258 genes with rare nonsynonymous mutations in patients with BPD. These genes were highly enriched for processes involved in pulmonary structure and function including collagen fibril organization, morphogenesis of embryonic epithelium, and regulation of Wnt signaling pathway; displayed significantly elevated expression in fetal and adult lungs; and were substantially up-regulated in a murine model of BPD. Analyses of mouse mutants revealed their phenotypic enrichment for embryonic development and the cyanosis phenotype, a clinical manifestation of BPD. Conclusions: Our study supports the role of rare variants in BPD, in contrast with the role of common variants targeted by genome-wide association studies. Overall, our study is the first to sequence BPD exomes from newborn blood spot samples and identify with high confidence genes implicated in BPD, thereby providing important insights into its biology and molecular etiology. PMID:26030808

  17. Posttranslational modification of β-catenin is associated with pathogenic fibroblastic changes in bronchopulmonary dysplasia.

    Science.gov (United States)

    Sucre, Jennifer M S; Vijayaraj, Preethi; Aros, Cody J; Wilkinson, Dan; Paul, Manash; Dunn, Bruce; Guttentag, Susan H; Gomperts, Brigitte N

    2017-02-01

    Bronchopulmonary dysplasia (BPD) is a common complication of premature birth. The histopathology of BPD is characterized by an arrest of alveolarization with fibroblast activation. The Wnt/β-catenin signaling pathway is important in early lung development. When Wnt signaling is active, phosphorylation of β-catenin by tyrosine kinases at activating sites, specifically at tyrosine 489 (Y489), correlates with nuclear localization of β-catenin. We examined fetal lung tissue, lung tissue from term newborns, and lung tissue from infants who died with BPD; we found nuclear β-catenin phosphorylation at Y489 in epithelial and mesenchymal cells in fetal tissue and BPD tissue, but not in the lungs of term infants. Using a 3D human organoid model, we found increased nuclear localization of β-catenin phosphorylated at Y489 (p-β-catenin(Y489)) after exposure to alternating hypoxia and hyperoxia compared with organoids cultured in normoxia. Exogenous stimulation of the canonical Wnt pathway in organoids was sufficient to cause nuclear localization of p-β-catenin(Y489) in normoxia and mimicked the pattern of α-smooth muscle actin (α-SMA) expression seen with fibroblastic activation from oxidative stress. Treatment of organoids with a tyrosine kinase inhibitor prior to cyclic hypoxia-hyperoxia inhibited nuclear localization of p-β-catenin(Y489) and prevented α-SMA expression by fibroblasts. Posttranslational phosphorylation of β-catenin is a transient feature of normal lung development. Moreover, the persistence of p-β-catenin(Y489) is a durable marker of fibroblast activation in BPD and may play an important role in BPD disease pathobiology. Copyright © 2017 the American Physiological Society.

  18. Stem cells as therapeutical option for the treatment of bronchopulmonary dysplasia

    Directory of Open Access Journals (Sweden)

    Dominik Monz

    2015-12-01

    Full Text Available During the past decades clinical results in neonatology have improved dramatically and increased the survival rate of preterm infants significantly. However, the short and long term outcome of these high-risk preterm infants is mainly influenced by respiratory diseases and neurological damages. Despite great advances in perinatal medicine, there is still no satisfactory treatment for bronchopulmonary dysplasia (BPD and current approaches are only supportive, have strong adverse effects or only show small benefits. Stem cell based therapies as well as other modes of regenerative strategies are applied as standard therapy in childhood predominantly in paediatric oncology. To date, such therapies have successfully been applied to treat immunodeficiency disorders and aplastic anaemia. But regenerative medicine might be an option for the treatment of BPD in preterm infants. According to some first preclinical results stem cell administration appears as a promising tool to improve the clinical outcome in high-risk infants. For severe neonatal diseases, e.g. hypoxic-ischemic encephalopathy (HIE in term neonates or BPD in preterm infants, a number of animal models have been established. Although these studies showed positive effects of stem cells in animal models of BPD several questions still remain. Further studies with appropriate preclinical neonate models and carefully controlled clinical trials are needed to assess the significance of regenerative therapies. In this review, we summarize recent results of some experimental and clinical studies that used stem cells to treat BPD associated with impairment of lung development. Proceedings of the 2nd International Course on Perinatal Pathology (part of the 11th International Workshop on Neonatology · October 26th-31st, 2015 · Cagliari (Italy · October 31st, 2015 · Stem cells: present and future Guest Editors: Gavino Faa, Vassilios Fanos, Antonio Giordano

  19. Acute kidney injury is associated with bronchopulmonary dysplasia/mortality in premature infants.

    Science.gov (United States)

    Askenazi, David; Patil, Neha R; Ambalavanan, Namasivayam; Balena-Borneman, Jessica; Lozano, David J; Ramani, Manimaran; Collins, Monica; Griffin, Russell L

    2015-09-01

    Acute kidney injury (AKI) impairs electrolyte balance, alters fluid homeostasis and decreases toxin excretion. More recent data suggest it also affects the physiology of distant organs. We performed a prospective cohort study which invloved 122 premature infants [birth weight (BW) ≤1200 g and/or gestational age (GA) bronchopulmonary dysplasia (BPD)/mortality. Days until oxygen discontinuation was compared between those with and without AKI in survivors who received oxygen for ≥24 h. Acute kidney disease, defined by a rise in serum creatinine (SCr) of ≥0.3 mg/dl or an increase in SCr of ≥150%, occurred in 36/122 (30%) of the premature infants. Those with AKI had a 70% higher risk of oxygen requirement or of dying at 28 days of life [relative risk (RR) 1.71, 95% confidence interval (CI) 1.22-2.39; p < 0.002]. This association remained after controlling for GA, pre-eclampsia, 5 min Apgar score and percentage maximum weight change (max % weight Δ) in the first 4 days (RR 1.45, 95% CI 1.07-1.97); p < 0.02). Similar findings were noted for receipt of mechanical ventilation/death by day 28 (adjusted RR 1.53, 95% CI 1.05-2.22; p < 0.03). Those without AKI were 2.5-fold more likely to come off oxygen [hazard ratio (HR) 1.3-5; p < 0.02) than those with AKI, even when controlling for GA, pre-eclampsia, 5 min Apgar and max % weight Δ (multivariate HR 2.0, 95% CI 0.9-4.0; p < 0.06). In premature infants, AKI is associated with BPD/mortality. As AKI could lead to altered lung physiology, interventions to ameliorate AKI could improve long-term BPD.

  20. A systematic review of randomized controlled trials for the prevention of bronchopulmonary dysplasia in infants

    Science.gov (United States)

    Beam, Kristyn S.; Aliaga, Sofia; Ahlfeld, Shawn K.; Cohen-Wolkowiez, Michael; Smith, P. Brian; Laughon, Matthew M.

    2014-01-01

    Objective Bronchopulmonary dysplasia (BPD) is the most common cause of pulmonary morbidity in premature infants and is associated with life-long morbidities. Developing drugs for the prevention of BPD would improve public health. We sought to determine characteristics of favorable randomized controlled trials (RCTs) of drugs for BPD prevention. Evidence review We searched MEDLINE and EMBASE from 1992–2014 using the MeSH terms “BPD” and “respiratory distress syndrome, newborn.” We included a Cochrane Library search to ensure inclusion of all available RCTs. We identified RCTs with BPD as a primary or secondary outcome and determined the definition of BPD used by the study. We determined whether a phase I or phase II study—to determine drug safety, efficacy, or optimal dose—was performed prior to the RCT. Finally, we searched the Cochrane Library for meta-analyses for each drug and used the results of available meta-analyses to define a favorable versus unfavorable RCT. Findings We identified 2026 articles; 47 RCTs met our inclusion criteria encompassing 21 drugs; 5 of the drugs reduced the incidence of BPD. We found data from phase I or II studies for 16 of the drugs, but only 1 demonstrated a reduction of BPD. Conclusions and relevance The majority of the drugs studied in RCTs failed to reduce the incidence of BPD. Performing early-phase studies prior to phase III trials might provide necessary information on drugs and drug doses capable of preventing BPD, thus informing the development of future RCTs. PMID:25010224

  1. Early pulmonary vascular disease in preterm infants at risk for bronchopulmonary dysplasia.

    Science.gov (United States)

    Mourani, Peter M; Sontag, Marci K; Younoszai, Adel; Miller, Joshua I; Kinsella, John P; Baker, Christopher D; Poindexter, Brenda B; Ingram, David A; Abman, Steven H

    2015-01-01

    Pulmonary hypertension (PH) is associated with poor outcomes among preterm infants with bronchopulmonary dysplasia (BPD), but whether early signs of pulmonary vascular disease are associated with the subsequent development of BPD or PH at 36 weeks post-menstrual age (PMA) is unknown. To prospectively evaluate the relationship of early echocardiogram signs of pulmonary vascular disease in preterm infants to the subsequent development of BPD and late PH (at 36 wk PMA). Prospectively enrolled preterm infants with birthweights 500-1,250 g underwent echocardiogram evaluations at 7 days of age (early) and 36 weeks PMA (late). Clinical and echocardiographic data were analyzed to identify early risk factors for BPD and late PH. A total of 277 preterm infants completed echocardiogram and BPD assessments at 36 weeks PMA. The median gestational age at birth and birthweight of the infants were 27 weeks and 909 g, respectively. Early PH was identified in 42% of infants, and 14% were diagnosed with late PH. Early PH was a risk factor for increased BPD severity (relative risk, 1.12; 95% confidence interval, 1.03-1.23) and late PH (relative risk, 2.85; 95% confidence interval, 1.28-6.33). Infants with late PH had greater duration of oxygen therapy and increased mortality in the first year of life (P < 0.05). Early pulmonary vascular disease is associated with the development of BPD and with late PH in preterm infants. Echocardiograms at 7 days of age may be a useful tool to identify infants at high risk for BPD and PH.

  2. Deregulation of the lysyl hydroxylase matrix cross-linking system in experimental and clinical bronchopulmonary dysplasia.

    Science.gov (United States)

    Witsch, Thilo J; Turowski, Pawel; Sakkas, Elpidoforos; Niess, Gero; Becker, Simone; Herold, Susanne; Mayer, Konstantin; Vadász, István; Roberts, Jesse D; Seeger, Werner; Morty, Rory E

    2014-02-01

    Bronchopulmonary dysplasia (BPD) is a common and serious complication of premature birth, characterized by a pronounced arrest of alveolar development. The underlying pathophysiological mechanisms are poorly understood although perturbations to the maturation and remodeling of the extracellular matrix (ECM) are emerging as candidate disease pathomechanisms. In this study, the expression and regulation of three members of the lysyl hydroxylase family of ECM remodeling enzymes (Plod1, Plod2, and Plod3) in clinical BPD, as well as in an experimental animal model of BPD, were addressed. All three enzymes were localized to the septal walls in developing mouse lungs, with Plod1 also expressed in the vessel walls of the developing lung and Plod3 expressed uniquely at the base of developing septa. The expression of plod1, plod2, and plod3 was upregulated in the lungs of mouse pups exposed to 85% O2, an experimental animal model of BPD. Transforming growth factor (TGF)-β increased plod2 mRNA levels and activated the plod2 promoter in vitro in lung epithelial cells and in lung fibroblasts. Using in vivo neutralization of TGF-β signaling in the experimental animal model of BPD, TGF-β was identified as the regulator of aberrant plod2 expression. PLOD2 mRNA expression was also elevated in human neonates who died with BPD or at risk for BPD, compared with neonates matched for gestational age at birth or chronological age at death. These data point to potential roles for lysyl hydroxylases in normal lung development, as well as in perturbed late lung development associated with BPD.

  3. Inhibition of β-catenin signaling improves alveolarization and reduces pulmonary hypertension in experimental bronchopulmonary dysplasia.

    Science.gov (United States)

    Alapati, Deepthi; Rong, Min; Chen, Shaoyi; Hehre, Dorothy; Hummler, Stefanie C; Wu, Shu

    2014-07-01

    Bronchopulmonary dysplasia (BPD) is the most common and serious chronic lung disease of preterm infants. The development of pulmonary hypertension (PH) significantly increases the mortality and morbidity of this disease. β-Catenin signaling plays an important role in tissue development and remodeling. Aberrant β-catenin signaling is associated with clinical and experiment models of BPD. To test the hypothesis that inhibition of β-catenin signaling is beneficial in promoting alveolar and vascular development and preventing PH in experimental BPD, we examined the effects of ICG001, a newly developed pharmacological inhibitor of β-catenin, in preventing hyperoxia-induced BPD in neonatal rats. Newborn rat pups were randomized at postnatal day (P)2 to room air (RA) + DMSO (placebo), RA + ICG001, 90% FiO2 (O2) + DMSO, or O2 + ICG001. ICG001 (10 mg/kg) or DMSO was given by daily intraperitoneal injection for 14 days during continuous exposure to RA or hyperoxia. Primary human pulmonary arterial smooth muscle cells (PASMCs) were cultured in RA or hyperoxia (95% O2) in the presence of DMSO or ICG001 for 24 to 72 hours. Treatment with ICG001 significantly increased alveolarization and reduced pulmonary vascular remodeling and PH during hyperoxia. Furthermore, administering ICG001 decreased PASMC proliferation and expression of extracellular matrix remodeling molecules in vitro under hyperoxia. Finally, these structural, cellular, and molecular effects of ICG001 were associated with down-regulation of multiple β-catenin target genes. These data indicate that β-catenin signaling mediates hyperoxia-induced alveolar impairment and PH in neonatal animals. Targeting β-catenin may provide a novel strategy to alleviate BPD in preterm infants.

  4. Occurrence and severity of bronchopulmonary dysplasia and respiratory distress syndrome after a preterm birth

    Science.gov (United States)

    Landry, Jennifer S; Menzies, Dick

    2011-01-01

    BACKGROUND: Despite notable advances in neonatal care, bronchopulmonary dysplasia (BPD) remains an important complication of preterm birth, frequently resulting in prolonged hospital stay and long-term morbidity. METHODS: A historical cohort study of all preterm infants (gestational age younger than 37 weeks) admitted to the Montreal Children’s Hospital (Montreal, Quebec) between January 1, 1980, and December 31, 1992, was conducted. Information collected included demographic data, maternal and perinatal history, and main neonatal outcomes. Independent risk factors associated with BPD were identified by univariate analysis using one-way ANOVA, t tests or Mantel-Haenszel χ2 testing. Severity of disease was studied using an ordinal multinomial logistic regression model. RESULTS: In total, 1192 preterm infants were admitted, of whom 551 developed respiratory distress syndrome and 322 developed BPD. For each additional week of prematurity, the risk of developing BPD increased by 54% (adjusted OR 1.54/week [95% CI 1.45 to 1.64]). For each point subtracted on the 1 min Apgar score, the risk of developing BPD was increased by 16% (OR 1.16 [95% CI 1.1 to 1.3]). BPD was also associated with the presence of patent ductus arteriosus (OR 3.5 [95% CI 2.1 to 6.0]), pneumothorax in the first 48 h (OR 9.4 [95% CI 3.6 to 24.8]) or neonatal pneumonia/sepsis in the neonatal period (OR 1.9 [95% CI 1.1 to 3.2]). Severity of BPD was associated with gestational age, 1 min Apgar score, very low birth weight and the presence of neonatal pneumonia/sepsis. CONCLUSION: Factors associated with BPD following a preterm birth were the degree of prematurity, birth weight, Apgar score at 1 min, and the presence of patent ductus arteriosus, pneumothorax or neonatal pneumonia/sepsis. PMID:22851893

  5. Genome-Wide Transcriptional Profiling Reveals Connective Tissue Mast Cell Accumulation in Bronchopulmonary Dysplasia

    Science.gov (United States)

    Bhattacharya, Soumyaroop; Go, Diana; Krenitsky, Daria L.; Huyck, Heidi L.; Solleti, Siva Kumar; Lunger, Valerie A.; Metlay, Leon; Srisuma, Sorachai; Wert, Susan E.; Pryhuber, Gloria S.

    2012-01-01

    Rationale: Bronchopulmonary dysplasia (BPD) is a major complication of premature birth. Risk factors for BPD are complex and include prenatal infection and O2 toxicity. BPD pathology is equally complex and characterized by inflammation and dysmorphic airspaces and vasculature. Due to the limited availability of clinical samples, an understanding of the molecular pathogenesis of this disease and its causal mechanisms and associated biomarkers is limited. Objectives: Apply genome-wide expression profiling to define pathways affected in BPD lungs. Methods: Lung tissue was obtained at autopsy from 11 BPD cases and 17 age-matched control subjects without BPD. RNA isolated from these tissue samples was interrogated using microarrays. Standard gene selection and pathway analysis methods were applied to the data set. Abnormal expression patterns were validated by quantitative reverse transcriptase–polymerase chain reaction and immunohistochemistry. Measurements and Main Results: We identified 159 genes differentially expressed in BPD tissues. Pathway analysis indicated previously appreciated (e.g., DNA damage regulation of cell cycle) as well as novel (e.g., B-cell development) biological functions were affected. Three of the five most highly induced genes were mast cell (MC)-specific markers. We confirmed an increased accumulation of connective tissue MCTC (chymase expressing) mast cells in BPD tissues. Increased expression of MCTC markers was also demonstrated in an animal model of BPD-like pathology. Conclusions: We present a unique genome-wide expression data set from human BPD lung tissue. Our data provide information on gene expression patterns associated with BPD and facilitated the discovery that MCTC accumulation is a prominent feature of this disease. These observations have significant clinical and mechanistic implications. PMID:22723293

  6. Correlation of radiographic thoracic area and oxygenation impairment in bronchopulmonary dysplasia.

    Science.gov (United States)

    Dassios, Theodore; Curley, Anna; Krokidis, Miltiadis; Morley, Colin; Ross-Russell, Robert

    2016-01-01

    We hypothesized that radiographically-assessed hyperinflation in bronchopulmonary dysplasia (BPD) is related to the degree of oxygenation impairment. Our objective was to explore the relation of chest radiographic thoracic area (CRTA) with right-to-left shunt, right shift of the oxyhemoglobin dissociation curve and ventilation/perfusion ratio (VA/Q) in infants with BPD. Twenty-two infants born at median (IQR) gestation of 26 (24-28) weeks with BPD were prospectively studied at 39 (30-69) days. Inspired oxygen (FiO2) was varied to obtain transcutaneous oxygen saturation (SpO2) values between 85 and 96%. Shunt, shift and VA/Q were derived by plotting and analysing pairs of SpO2 and FiO2. CRTA was measured by free hand-tracing the perimeter of the thoracic area in anterio-posterior chest radiographs. Median (IQR) shunt was 8 (1-14)%, shift was 13 (11-19)kPa and VA/Q 0.42 (0.30-0.48). Median (IQR) CRTA/kg was 2495 (1962-2838)mm(2) and was significantly related to shift (r=0.674, p<0.001), VA/Q (r=-0.633, p<0.001), weight at study (r=-0.457, p=0.003) and day of life (r=-0.406, p=0.009), but not to shunt. CRTA in BPD is significantly related to oxygenation impairment as quantified by shift and VA/Q. CRTA can be used as a simple radiographic test to quantify BPD severity. Copyright © 2015 Elsevier B.V. All rights reserved.

  7. Antenatal Determinants of Bronchopulmonary Dysplasia and Late Respiratory Disease in Preterm Infants.

    Science.gov (United States)

    Morrow, Lindsey A; Wagner, Brandie D; Ingram, David A; Poindexter, Brenda B; Schibler, Kurt; Cotten, C Michael; Dagle, John; Sontag, Marci K; Mourani, Peter M; Abman, Steven H

    2017-08-01

    Mechanisms contributing to chronic lung disease after preterm birth are incompletely understood. To identify antenatal risk factors associated with increased risk for bronchopulmonary dysplasia (BPD) and respiratory disease during early childhood after preterm birth, we performed a prospective, longitudinal study of 587 preterm infants with gestational age less than 34 weeks and birth weights between 500 and 1,250 g. Data collected included perinatal information and assessments during the neonatal intensive care unit admission and longitudinal follow-up by questionnaire until 2 years of age. After adjusting for covariates, we found that maternal smoking prior to preterm birth increased the odds of having an infant with BPD by twofold (P = 0.02). Maternal smoking was associated with prolonged mechanical ventilation and respiratory support during the neonatal intensive care unit admission. Preexisting hypertension was associated with a twofold (P = 0.04) increase in odds for BPD. Lower gestational age and birth weight z-scores were associated with BPD. Preterm infants who were exposed to maternal smoking had higher rates of late respiratory disease during childhood. Twenty-two percent of infants diagnosed with BPD and 34% of preterm infants without BPD had no clinical signs of late respiratory disease during early childhood. We conclude that maternal smoking and hypertension increase the odds for developing BPD after preterm birth, and that maternal smoking is strongly associated with increased odds for late respiratory morbidities during early childhood. These findings suggest that in addition to the BPD diagnosis at 36 weeks, other factors modulate late respiratory outcomes during childhood. We speculate that measures to reduce maternal smoking not only will lower the risk for preterm birth but also will improve late respiratory morbidities after preterm birth.

  8. Quality improvement study of effectiveness of cue-based feeding in infants with bronchopulmonary dysplasia in the neonatal intensive care unit.

    Science.gov (United States)

    Davidson, Elizabeth; Hinton, Diana; Ryan-Wenger, Nancy; Jadcherla, Sudarshan

    2013-01-01

    The effectiveness and safety of experimental cue-based versus health care provider-driven (baseline) feeding strategies were evaluated in infants with bronchopulmonary dysplasia. The experimental group (n = 55) and the control group(n = 60) included infants who had been previously diagnosed with varying levels of severity of bronchopulmonary dysplasia and were identified retrospectively. Previous research was used to derive an Oral Feeding Readiness Scale as well as an Oral Feeding Quality Scale. Results validated both scales as well as the cue-based feeding strategy. 2013 AWHONN, the Association of Women's Health, Obstetric and Neonatal Nurses.

  9. Follow-up of school-age children with bronchopulmonary dysplasia.

    Science.gov (United States)

    Giacoia, G P; Venkataraman, P S; West-Wilson, K I; Faulkner, M J

    1997-03-01

    To investigate the outcome of school-age children with bronchopulmonary dysplasia (BPD) in terms of nutrition, pulmonary function, and intelligence, and to compare the results with a preterm cohort matched for gestational age and birth weight, and with a term control group. Cross-sectional. Follow-up clinic at level III neonatal intensive care unit, university-affiliated hospital, Children's Hospital. Twelve children who had BPD as infants and 2 control groups of 12 children each. Anthropometric measurements, dietary intake, resting energy expenditure, pulmonary function, body composition measurements by dual energy x-ray absorptiometry, and Weschler intelligence test scores. Children with BPD had decreased forced expiratory volume at 1 second, decreased forced expiratory flow between 25% and 75% of vital capacity, and decreased maximal expiratory flow velocity at 50% of vital capacity compared with age-matched normal inborn subjects (p = 0.025, p = 0.005, and p = 0.0013, respectively). Both children with BPD and matched preterm control children were shorter than infants in the term control group (p = 0.018). There were no significant differences in the other anthropometric parameters studied. The groups did not differ in resting energy expenditure. Lean body mass was lower in the BPD group compared with the term control groups (p = 0.017). Bone mineral content was lower in the BPD group compared with both the preterm and term control infants (p = 0.050 and p = 0.059, respectively). The mean performance intelligence quotient (IQ) and full-scale IQ scores in the BPD group were lower than in the term control group (p = 0.011 and p = 0.029, respectively). The proportion of children with borderline or intellectually deficient scores was significantly higher in the preterm group compared with the term group for verbal, performance, and full-scale IQ scales (p = 0.046, p = 0.018, and p = 0.048 respectively). The proportion of children with BPD who had borderline or

  10. Prediction of bronchopulmonary dysplasia by chest radiographic scoring system at seven days of age

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    Choi, Yun Sun; Kim, Woo Sun; Kim, In One; Choi, Jung Hwan; Yeon, Kyung Mo; Yun, Chong Ku [Seoul National Univ. College of Medicine, Seoul (Korea, Republic of)

    1997-03-01

    Recent trials of preventive dexamethasone therapy in preterm neonates at high risk of developing bronchopulmonary dysplasia (BPD) have required the objective criteria for prediction of BPD in the early neonatal period. The purpose of this study is to determine whether a chest radiographic scoring system at 7 days of age can be used to predict BPD. Chest radiographs taken at 7 days and 28 days of age in 59 preterm neonates(gestational age of less than 33 weeks) were scored prospectively according to the consensus of two radiologists. The 7-day radiographs were scored according to a system derived from Yuksel's method:endotracheal tube insertion, degree of lung inflation, lung opacification, interstitial changes and cardiomegaly were measured. The radiographs taken at 28 days were scored according to a modification of Toce's method. The BPD group was defined as consisting of patients who needed oxygen therapy for more than 28 days and showed abnormality on chest radiographs. Scores were analysed to determine whether there were any statistical differences between the BPD and non-BPD groups, whether there was a significant correlation between scores at 7 days and 28 days, and whether there was any relationship between scores at 7 days of age and the development of BPD. We tried to determine which factors, as indicated by the scores at 7 days, significantly contributed to the development of BPD. The mean scores at 7 days of age in the BPD group (n=18) were 4.3{+-}1.5 (2-7), and those in the non-BPD group (n=41) were 2.2{+-}1.2 (0-4). The differences were statistically significant (p<.0001). Significant correlation was found between scores at 7 days and at 28 days of age(r:0.57, p<.0001). Analysis showed that endotracheal tube insertion, cardiomegaly, and degree of interstitial change, as seen on radiographs at 7 days, were factors which significantly contributed to the development of BPD (p<0.05 each). All neonates with a score of 5 or more developed BPD (7

  11. Standardisation of oxygen exposure in the development of mouse models for bronchopulmonary dysplasia

    Science.gov (United States)

    Nardiello, Claudio; Mižíková, Ivana; Silva, Diogo M.; Ruiz-Camp, Jordi; Mayer, Konstantin; Vadász, István; Herold, Susanne; Seeger, Werner

    2017-01-01

    ABSTRACT Progress in developing new therapies for bronchopulmonary dysplasia (BPD) is sometimes complicated by the lack of a standardised animal model. Our objective was to develop a robust hyperoxia-based mouse model of BPD that recapitulated the pathological perturbations to lung structure noted in infants with BPD. Newborn mouse pups were exposed to a varying fraction of oxygen in the inspired air (FiO2) and a varying window of hyperoxia exposure, after which lung structure was assessed by design-based stereology with systemic uniform random sampling. The efficacy of a candidate therapeutic intervention using parenteral nutrition was evaluated to demonstrate the utility of the standardised BPD model for drug discovery. An FiO2 of 0.85 for the first 14 days of life decreased total alveoli number and concomitantly increased alveolar septal wall thickness, which are two key histopathological characteristics of BPD. A reduction in FiO2 to 0.60 or 0.40 also caused a decrease in the total alveoli number, but the septal wall thickness was not impacted. Neither a decreasing oxygen gradient (from FiO2 0.85 to 0.21 over the first 14 days of life) nor an oscillation in FiO2 (between 0.85 and 0.40 on a 24 h:24 h cycle) had an appreciable impact on lung development. The risk of missing beneficial effects of therapeutic interventions at FiO2 0.85, using parenteral nutrition as an intervention in the model, was also noted, highlighting the utility of lower FiO2 in selected studies, and underscoring the need to tailor the model employed to the experimental intervention. Thus, a state-of-the-art BPD animal model that recapitulates the two histopathological hallmark perturbations to lung architecture associated with BPD is described. The model presented here, where injurious stimuli have been systematically evaluated, provides a most promising approach for the development of new strategies to drive postnatal lung maturation in affected infants. PMID:28067624

  12. Leukotriene receptor blockade as a life-saving treatment in severe bronchopulmonary dysplasia.

    Science.gov (United States)

    Rupprecht, Thomas; Rupprecht, Christian; Harms, Dieter; Sterlacci, William; Vieth, Michael; Seybold, Kathrin

    2014-01-01

    Bronchopulmonary dysplasia (BPD) is a major cause of mortality and morbidity in infants with an extremely low birth weight. Because there is no effective therapy, the mortality of this condition in severely affected patients is high. Therapeutic blocking of the leukotriene system seems to be a logical approach due to the known pathophysiology of BPD. The aim of this study was to examine the therapeutic effect of montelukast in preterm children suffering from severe BPD. We performed an unblinded, prospective trial including infants born between 23 and 27 weeks of gestation suffering from severe BPD. The study drug was montelukast (1 mg/kg of body weight as a single dose daily in the 1st week of therapy, increasing to 1.5 mg/kg of body weight in the 2nd week and finally to 2 mg/kg of body weight in the 3rd week). Treatment was continued until the radiological signs and the clinical symptoms of BPD disappeared or the patient was discharged from the hospital. Each patient included in this study was matched for gestational age, birth weight, and pulmonary severity score to a control. Until March 2014, a total of 22 infants were enrolled into the study. The rates of the primary outcome differed significantly between the montelukast-treated group and the control group. All but 1 of the children in the treatment group survived (91%), whereas 7 of the 11 children in the control group died (survival rate 36%; p = 0.002 using Fisher's exact test). The mean mechanical ventilation time (41.2 ± 25.3 vs. 103.7 ± 90.6 days) was significantly shorter and the mean preterm complication score (3.0 ± 1.7 vs. 5.6 ± 1.4) was significantly lower in treated patients compared to the control group. (p = 0.05 for both items; Wilcoxon's matched-pairs test). Based on the clinical observations, the statistical results, and the relatively low risk of the study drug montelukast, we recommend using this treatment in severe cases of BPD for infants facing a high risk of death.

  13. Endoglin in Amniotic Fluid as a Risk Factor for the Subsequent Development of Bronchopulmonary Dysplasia

    Science.gov (United States)

    KIM, Sun Kwon; ROMERO, Roberto; SAVASAN, Zeynep ALPAY; XU, Yi; DONG, Zhong; LEE, Deug-Chan; Yeo, Lami; HASSAN, Sonia S; CHAIWORAPONGSA, Tinnakorn

    2014-01-01

    Objective Cross-talk between inflammation and angiogenesis pathways has been recently reported. The objective of this study was to: 1) examine whether amniotic fluid (AF) concentrations of soluble endoglin (sEng), a protein with anti-angiogenic properties, changes during pregnancy, parturition or intraamniotic infection and/or inflammation (IAI); 2) determine whether an elevation of sEng in the AF of patients with preterm labor (PTL) and preterm prelabor rupture of membranes (PROM) is associated with adverse neonatal outcomes; and 3) investigate potential sources of sEng in AF. Study Design A cross-sectional study was conducted to include patients in the following groups: 1) midtrimester (n=20); 2) PTL with term delivery (n=95); 3) PTL leading to preterm delivery with (n=40) and without IAI (n=46); 4) preterm PROM with (n=37) and without IAI (n=37); 5) term in labor (n=48) and not in labor (n=44). AF concentrations of sEng were determined by ELISA. Chorioamniotic membranes, umbilical cord blood and AF macrophages were examined for the expression of endoglin. Results 1) Patients with IAI had a higher median AF concentration of sEng than those without IAI (p=0.02 for PTL; and 0.06 for preterm PROM); 2) AF concentrations of sEng in the 3rd and 4th quartiles were associated with IAI (OR 2.5 and 7.9 respectively); 3) an AF sEng concentration ≥779.5 pg/ml was associated with bronchopulmonary dysplasia (BPD) (OR 7.9); 4) endoglin was co-localized with CD14+ macrophages in AF pellets of patients with IAI by immunofluorescence and flow cytometry; and 5) the concentration of sEng in the supernatant was significantly increased after treatment of macrophages with endotoxin or TNF-α. Conclusions sEng participates in the host response against IAI. Activated macrophages may be a source of sEng concentrations in the AF of patients with IAI. An increase of sEng in the AF is associated with BPD and adverse neonatal outcomes. PMID:23279628

  14. Fgf10 deficiency is causative for lethality in a mouse model of bronchopulmonary dysplasia.

    Science.gov (United States)

    Chao, Cho-Ming; Yahya, Faady; Moiseenko, Alena; Tiozzo, Caterina; Shrestha, Amit; Ahmadvand, Negah; El Agha, Elie; Quantius, Jennifer; Dilai, Salma; Kheirollahi, Vahid; Jones, Matthew; Wilhem, Jochen; Carraro, Gianni; Ehrhardt, Harald; Zimmer, Klaus-Peter; Barreto, Guillermo; Ahlbrecht, Katrin; Morty, Rory E; Herold, Susanne; Abellar, Rosanna G; Seeger, Werner; Schermuly, Ralph; Zhang, Jin-San; Minoo, Parviz; Bellusci, Saverio

    2017-01-01

    Inflammation-induced FGF10 protein deficiency is associated with bronchopulmonary dysplasia (BPD), a chronic lung disease of prematurely born infants characterized by arrested alveolar development. So far, experimental evidence for a direct role of FGF10 in lung disease is lacking. Using the hyperoxia-induced neonatal lung injury as a mouse model of BPD, the impact of Fgf10 deficiency in Fgf10(+/-) versus Fgf10(+/+) pups was investigated. In normoxia, no lethality of Fgf10(+/+) or Fgf10(+/-) pups was observed. By contrast, all Fgf10(+/-) pups died within 8 days of hyperoxic injury, with lethality starting at day 5, whereas Fgf10(+/+) pups were all alive. Lungs of pups from the two genotypes were collected on postnatal day 3 following normoxia or hyperoxia exposure for further analysis. In hyperoxia, Fgf10(+/-) lungs exhibited increased hypoalveolarization. Analysis by FACS of the Fgf10(+/-) versus control lungs in normoxia revealed a decreased ratio of alveolar epithelial type II (AECII) cells over total Epcam-positive cells. In addition, gene array analysis indicated reduced AECII and increased AECI transcriptome signatures in isolated AECII cells from Fgf10(+/-) lungs. Such an imbalance in differentiation is also seen in hyperoxia and is associated with reduced mature surfactant protein B and C expression. Attenuation of the activity of Fgfr2b ligands postnatally in the context of hyperoxia also led to increased lethality with decreased surfactant expression. In summary, decreased Fgf10 mRNA levels lead to congenital lung defects, which are compatible with postnatal survival, but which compromise the ability of the lungs to cope with sub-lethal hyperoxic injury. Fgf10 deficiency affects quantitatively and qualitatively the formation of AECII cells. In addition, Fgfr2b ligands are also important for repair after hyperoxia exposure in neonates. Deficient AECII cells could be an additional complication for patients with BPD. Copyright © 2016 Pathological Society of

  15. Standardisation of oxygen exposure in the development of mouse models for bronchopulmonary dysplasia

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    Claudio Nardiello

    2017-02-01

    Full Text Available Progress in developing new therapies for bronchopulmonary dysplasia (BPD is sometimes complicated by the lack of a standardised animal model. Our objective was to develop a robust hyperoxia-based mouse model of BPD that recapitulated the pathological perturbations to lung structure noted in infants with BPD. Newborn mouse pups were exposed to a varying fraction of oxygen in the inspired air (FiO2 and a varying window of hyperoxia exposure, after which lung structure was assessed by design-based stereology with systemic uniform random sampling. The efficacy of a candidate therapeutic intervention using parenteral nutrition was evaluated to demonstrate the utility of the standardised BPD model for drug discovery. An FiO2 of 0.85 for the first 14 days of life decreased total alveoli number and concomitantly increased alveolar septal wall thickness, which are two key histopathological characteristics of BPD. A reduction in FiO2 to 0.60 or 0.40 also caused a decrease in the total alveoli number, but the septal wall thickness was not impacted. Neither a decreasing oxygen gradient (from FiO2 0.85 to 0.21 over the first 14 days of life nor an oscillation in FiO2 (between 0.85 and 0.40 on a 24 h:24 h cycle had an appreciable impact on lung development. The risk of missing beneficial effects of therapeutic interventions at FiO2 0.85, using parenteral nutrition as an intervention in the model, was also noted, highlighting the utility of lower FiO2 in selected studies, and underscoring the need to tailor the model employed to the experimental intervention. Thus, a state-of-the-art BPD animal model that recapitulates the two histopathological hallmark perturbations to lung architecture associated with BPD is described. The model presented here, where injurious stimuli have been systematically evaluated, provides a most promising approach for the development of new strategies to drive postnatal lung maturation in affected infants.

  16. Altered Right Ventricular Mechanical Properties Are Afterload Dependent in a Rodent Model of Bronchopulmonary Dysplasia

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    Jitandrakumar R. Patel

    2017-10-01

    Full Text Available Infants born premature are at increased risk for development of bronchopulmonary dysplasia (BPD, pulmonary hypertension (PH, and ultimately right ventricular (RV dysfunction, which together carry a high risk of neonatal mortality. However, the role alveolar simplification and abnormal pulmonary microvascular development in BPD affects RV contractile properties is unknown. We used a rat model of BPD to examine the effect of hyperoxia-induced PH on RV contractile properties. We measured in vivo RV pressure as well as passive force, maximum Ca2+ activated force, calcium sensitivity of force (pCa50 and rate of force redevelopment (ktr in RV skinned trabeculae isolated from hearts of 21-and 35-day old rats pre-exposed to 21% oxygen (normoxia or 85% oxygen (hyperoxia for 14 days after birth. Systolic and diastolic RV pressure were significantly higher at day 21 in hyperoxia exposed rats compared to normoxia control rats, but normalized by 35 days of age. Passive force, maximum Ca2+ activated force, and calcium sensitivity of force were elevated and cross-bridge cycling kinetics depressed in 21-day old hyperoxic trabeculae, whereas no differences between normoxic and hyperoxic trabeculae were seen at 35 days. Myofibrillar protein analysis revealed that 21-day old hyperoxic trabeculae had increased levels of beta-myosin heavy chain (β-MHC, atrial myosin light chain 1 (aMLC1; often referred to as essential light chain, and slow skeletal troponin I (ssTnI compared to age matched normoxic trabeculae. On the other hand, 35-day old normoxic and hyperoxic trabeculae expressed similar level of α- and β-MHC, ventricular MLC1 and predominantly cTnI. These results suggest that neonatal exposure to hyperoxia increases RV afterload and affect both the steady state and dynamic contractile properties of the RV, likely as a result of hyperoxia-induced expression of β-MHC, delayed transition of slow skeletal TnI to cardiac TnI, and expression of atrial MLC1. These

  17. A randomized, placebo-controlled GH trial in very preterm infants who were at risk for bronchopulmonary dysplasia and were treated with dexamethasone

    NARCIS (Netherlands)

    Huysman, MWA; Hop, WCJ; Cromme-Dijkhuis, AH; Sauer, PJJ; Hokken-Koelega, ACS

    2005-01-01

    Very preterm infants who develop bronchopulmonary dysplasia are often treated with dexamethasone (DEXA) to wean them from the ventilator. As DEXA has growth-suppressive and catabolic effects, which might have long-term consequences on growth and organ development, we investigated whether high-dose

  18. Angiopoietin-1 and endostatin levels in cord plasma predict the development of bronchopulmonary dysplasia in preterm infants.

    Science.gov (United States)

    Mohamed, Walid Abdel Wahab; Niyazy, Walid H; Mahfouz, Ahmed A

    2011-10-01

    To determine whether angiopoietin-1 and endostatin levels in the cord blood could predict the subsequent development of bronchopulmonary dysplasia (BPD) in preterm infants. A total of 102 preterm (gestational age ≤ 32 weeks) infants (28 infants developed BPD and 74 had no BPD) were enrolled in the study. Cord plasma levels of angiopoietin-1 and endostatin were measured by enzyme-linked immunosorbent assay. Preterm infants who subsequently developed BPD had significantly lower cord plasma levels of angiopoietin-1 than those who did not (p cord plasma levels of endostatin were significantly higher in infants with BPD than in those without (p cord plasma correlated negatively with endostatin (r = -0.48; p = 0.008). In preterm infants, low-angiopoietin-1 and high-endostatin levels in cord plasma at birth predict the subsequent development of BPD.

  19. Bronchopulmonary dysplasia: Clinical practices in five Portuguese neonatal intensive care units

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    H. Guimarães

    2010-03-01

    Full Text Available With the advent of surfactant, prenatal corticosteroids (PNC and advances in technology, the survival rate of extremely low birth weight (ELBW infants has improved dramatically. Rates of bronchopulmonary dysplasia (BPD vary widely among neonatal intensive care units (NICUs and many studies using multiple interventions have shown some improvement in BPD rates. Implementing potentially better practices to reduce BPD has been an effort made over the last few decades. Aim: To compare five Portuguese NICUs in terms of clinical practices in very low birth weight (VLBW infants, in order to develop better practices to prevent BPD. Patients and methods: 256 preterm neonates, gestational age (GA < 30 weeks and/or birthweight (BW < 1250g admitted to five Portuguese NICUs (centers 1 to 5 between 1st January 2004 and 31st December 2006, were studied. VLBW infants with major malformations, grade IV intraventricular haemorrhage in the first week of life and metabolic or neuromuscular disease were excluded. BPD was defined as oxygen dependency at 36 weeks of postconceptional age. We considered a practice to be improved as clinically significant whenever a decrease greater than 10% in the prevalence of BPD adjusted for the practice, GA and BW was achieved compared to BPD prevalence adjusted only for GA and BW. Results: The overall prevalence of BPD was 12.9%. Our results revealed that PNC use should be improved in centers 4 and 5; fluid policy in center 4; oxygen therapy and sepsis prevention in centers 1 and 2. Patent ductus arteriosus (PDA treatment should be improved in center 2. Conclusion: The implementation of potentially better practices to reduce lung injury in neonates in Portuguese NICUs, according to each NICU, must be addressed to increase the prescription of PNC, to use a lower FiO2, to be careful with fluid administration in the first weeks of life and to prevent PDA and sepsis. It is necessary to follow guidelines, recommendations or

  20. The influence of gender on respiratory outcomes in children with bronchopulmonary dysplasia during the first 3 years of life.

    Science.gov (United States)

    Collaco, Joseph M; Aherrera, Angela D; McGrath-Morrow, Sharon A

    2017-02-01

    Since premature males are more likely to be diagnosed with bronchopulmonary dysplasia we hypothesized that differences in respiratory outcomes after initial hospital discharge and during the first 3 years of life would exist between females and males diagnosed with BPD. Subjects with the diagnosis of BPD were recruited from the Johns Hopkins Bronchopulmonary Dysplasia Clinic between 2008 and 2014. Clinical features were assessed through chart review (n = 482). Respiratory morbidities were assessed by caregiver questionnaires at clinic visits (n = 429), including emergency department visits, hospital admissions, systemic steroid use, and antibiotic use for respiratory reasons since the last BPD clinic visit or after initial hospital discharge if assessed at the first visit. Male infants weighed significantly more at birth, had higher birth weight percentiles and were more likely to be non-white compared to female infants. The frequency of ever acute care use was 36.9% for emergency department visits, 27.4% for hospital admissions, 36.9% for systemic steroid use, and 40.5% for antibiotic use for a respiratory illness. No differences in respiratory morbidities were found between males and females. Females however, tended to be weaned from supplemental oxygen over 3 months later than males. Compared to females with BPD, males were more likely to weigh more, have higher birth weight percentiles and be non-white. After initial hospital discharge, there were no difference in respiratory morbidities between males and females with BPD. Female infants however were more likely to be weaned from supplemental oxygen at a later age than male infants. Pediatr Pulmonol. 2017;52:217-224. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

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

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    Maresa E. C. Jiskoot-Ermers

    2015-10-01

    Full Text Available 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.

  2. Sphingosine kinase 1 deficiency confers protection against hyperoxia-induced bronchopulmonary dysplasia in a murine model: role of S1P signaling and Nox proteins.

    Science.gov (United States)

    Harijith, Anantha; Pendyala, Srikanth; Reddy, Narsa M; Bai, Tao; Usatyuk, Peter V; Berdyshev, Evgeny; Gorshkova, Irina; Huang, Long Shuang; Mohan, Vijay; Garzon, Steve; Kanteti, Prasad; Reddy, Sekhar P; Raj, J Usha; Natarajan, Viswanathan

    2013-10-01

    Bronchopulmonary dysplasia of the premature newborn is characterized by lung injury, resulting in alveolar simplification and reduced pulmonary function. Exposure of neonatal mice to hyperoxia enhanced sphingosine-1-phosphate (S1P) levels in lung tissues; however, the role of increased S1P in the pathobiological characteristics of bronchopulmonary dysplasia has not been investigated. We hypothesized that an altered S1P signaling axis, in part, is responsible for neonatal lung injury leading to bronchopulmonary dysplasia. To validate this hypothesis, newborn wild-type, sphingosine kinase1(-/-) (Sphk1(-/-)), sphingosine kinase 2(-/-) (Sphk2(-/-)), and S1P lyase(+/-) (Sgpl1(+/-)) mice were exposed to hyperoxia (75%) from postnatal day 1 to 7. Sphk1(-/-), but not Sphk2(-/-) or Sgpl1(+/-), mice offered protection against hyperoxia-induced lung injury, with improved alveolarization and alveolar integrity compared with wild type. Furthermore, SphK1 deficiency attenuated hyperoxia-induced accumulation of IL-6 in bronchoalveolar lavage fluids and NADPH oxidase (NOX) 2 and NOX4 protein expression in lung tissue. In vitro experiments using human lung microvascular endothelial cells showed that exogenous S1P stimulated intracellular reactive oxygen species (ROS) generation, whereas SphK1 siRNA, or inhibitor against SphK1, attenuated hyperoxia-induced S1P generation. Knockdown of NOX2 and NOX4, using specific siRNA, reduced both basal and S1P-induced ROS formation. These results suggest an important role for SphK1-mediated S1P signaling-regulated ROS in the development of hyperoxia-induced lung injury in a murine neonatal model of bronchopulmonary dysplasia. Copyright © 2013 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.

  3. The Role of Growth Factors (VEGF, TGF-β1 and Cyclic Guanosine Monophosphate in the Formation of Pulmonary Hypertension in Children with Bronchopulmonary Dysplasia

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    A.S. Senatorova

    2013-11-01

    Full Text Available In 82 children with bronchopulmonary dysplasia (from 1 to 36 months of corrected age we investigated the level of VEGF, TGF-β1 in blood and cyclic guanosine monophosphate (cGMP in sputum. It was revealed that children with bronchopulmonary dysplasia had a significant increase in TGF-β1 (p < 0.05 and cGMP (p < 0.01–0.001, reduced VEGF (p < 0.05, indicating inhibition of angiogenesis, activation of fibrosis factors and endothelium-dependent vasodilation. Reliable direct dependence of activation of TGF-β1 in blood and cGMP in sputum, as well as inverse correlation between VEGF in blood and rLA had been proved, which gave reason to think of pulmonary hypertension as an adverse factor in fibrosis activation and angiogenesis inhibition in children with bronchopulmonary dysplasia. Reduced oxygen saturation and oxygen partial pressure moderately activated cGMP, but did not provide a sufficient reduction of pressure in the pulmonary artery.

  4. Policy of feeding very preterm infants with their mother's own fresh expressed milk was associated with a reduced risk of bronchopulmonary dysplasia.

    Science.gov (United States)

    Dicky, Odile; Ehlinger, Virginie; Montjaux, Nathalie; Gremmo-Féger, Gisèle; Sizun, Jacques; Rozé, Jean-Christophe; Arnaud, Catherine; Casper, Charlotte

    2017-05-01

    Since 2005, the French Food Safety Agency has recommended that very preterm or low-birthweight babies should be fed with pasteurised, expressed breastmilk, and feeding policies on this vary widely in French neonatal units. We investigated the differences between using a mother's expressed milk, in fresh or pasteurised forms, for very preterm infants. This observational multicentre study analysed data on 926 very preterm infants: 636 from neonatal units who used the mother's own fresh milk and 290 who used the mother's milk after pasteurisation. We analysed necrotising enterocolitis, bronchopulmonary dysplasia, in-hospital mortality, late-onset sepsis, weight gain, length of hospital stay, the duration of parenteral nutrition and the duration of enteral feeding with a nasogastric tube. Multivariate analyses were conducted to assess the impact of maternal milk policies. After adjustment, there was a reduced risk of bronchopulmonary dysplasia in the fresh milk group with an odds ratio of 0.40 and 95% confidence interval of 0.27-0.67 (p bronchopulmonary dysplasia, and further investigations are needed to evaluate the clinical impact of this practice. ©2017 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

  5. B-type natriuretic peptide is a biomarker for pulmonary hypertension in preterm infants with bronchopulmonary dysplasia

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    Cuna A

    2013-05-01

    Full Text Available Alain Cuna,1 Jegen Kandasamy,1 Naomi Fineberg,2 Brian Sims1 1Department of Pediatrics, Division of Neonatology, 2Department of Biostatistics, University of Alabama at Birmingham, Birmingham, AL, USA Background: B-type natriuretic peptide (BNP is a cardiac biomarker useful in screening for pulmonary hypertension (PH in adults. It is possible that BNP may also be useful in detecting PH among preterm infants with bronchopulmonary dysplasia (BPD. Objective: To determine the utility of BNP for identification of PH among preterm infants with BPD. Methods: We retrospectively identified preterm infants with BPD who underwent screening echocardiography for suspected PH and had serum BNP levels measured within 10 days before or after echocardiography. Eligible infants were classified based on echocardiographic diagnosis of either PH or no PH. Median and interquartile ranges (IQR of BNP values were compared, and area under the curve (AUC of receiver operator characteristic (ROC analysis was used to determine the optimum threshold value for detection of PH. Results: Twenty-five preterm infants with BPD (mean gestational age 26.5 ± 1.7 weeks, mean birth weight 747 ± 248 g were identified. The median difference in days between echocardiography and BNP measurement was 1 day (IQR 0–3, range 0–10 days. Based on echocardiography, 16 were diagnosed with PH and nine without PH. No significant difference in terms of gestational age, birth weight, sex, race, or respiratory support was found between the two groups. Median (IQR BNP values of those with PH were higher than those without PH (413 [212–1178] pg/mL versus 55 [21–84] pg/mL, P < 0.001. AUC of ROC analysis showed that a BNP value of 117 pg/mL had 93.8% sensitivity and 100% specificity for detecting PH. Conclusion: BNP estimation may be useful for screening of PH in infants with BPD. Keywords: B-type natriuretic peptide, pulmonary hypertension, bronchopulmonary dysplasia, biological markers

  6. Vitamin A supplementation for prevention of bronchopulmonary dysplasia in very-low-birth-weight premature Thai infants: a randomized trial.

    Science.gov (United States)

    Kiatchoosakun, Pakaphan; Jirapradittha, Junya; Panthongviriyakul, M Charnchai; Khampitak, Tueanjit; Yongvanit, Puangrat; Boonsiri, Patcharee

    2014-10-01

    Bronchopulmonary dysplasia (BPD) is one ofthe most significant complications among very-low-birth-weight (VLBW) premature infants. Vitamin A deficiency increases the risk of BPD in VLBWinfants. To assess the effect of vitamin A supplementation for prevention of bronchopulmonary dysplasia in VLBW premature Thai infants. Randomized control trial. Eighty premature infants weighing supplementation at 24 hours ofage-admitted to Neonatal units ofSrinagarind Hospital, Khon Kaen University, Khon Kaen, Thailand-were assigned to receive either intramuscular vitaminA 5, 000 IU3 times/week (treatment group) or sham procedure (control group) for four weeks. Serum vitamin A levels were measured before and after administration of the vitamin A. The baseline of mean serum vitamin A levels were similar in both groups. The mean serum level of vitamin A was significantly higher in the vitamin A supplemented infants than in the control infants on day 7 (1.41 +/- 0.48 vs. 0.92+0.38 pmol/ L, psupplementation. None of the infants in the vitamin A supplemented group, compared to 5% of the infants in the control group, had vitamin A level supplemented group required oxygen supplementation at 36 weeks postmenstrual age than in the control group albeit not statistically significant (22.5 vs. 35% relative risk 0.71; 95% CI 0.40 +/- 1.26; p = 0.21). Supplementation with vitamin A was also associated with a significant reduction in the duration ofintubation (10.8 +/- 3.1 days vitamin A supplemented group vs. 26.1 +/- 6.4 days control group, p = 0.03), days on oxygen therapy (29.8 +/- 5.1 days vitamin A supplemented group vs. 58.2 +/- 9.1 days control group, p = 0.01) and length of hospital stay (61.9 +/- 4.2 days vitamin A supplemented group vs. 88.3 +/- 7.2 days control group, p = 0.002). The dose of vitamin A used in this study reduced biochemical evidence of vitamin A deficiency and, without complications, resulted in reducing duration of intubation, days of oxygen therapy, and length of

  7. Medical closure of patent ductus arteriosus does not reduce mortality and development of bronchopulmonary dysplasia in preterm infants

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    Demet Terek

    2014-01-01

    Full Text Available Background: Although, patent ductus arteriosus (PDA is associated with significant morbidity due to hemodynamic instability in preterm infants, the effect of ductus closure on mortality and morbidity is a controversial issue. The aim is to evaluate the efficacy of oral and intravenous (IV ibuprofen treatment on ductal closure and effects on mortality and bronchoplumonary dysplasia. Materials and Methods: The medical records of 292 premature infants treated at Ege University Neonatal Intensive Care Unit were retrospectively evaluated. Patients were classified into 3 groups as; No PDA, hemodynamically insignificant PDA (hiPDA and hemodynamically significant PDA (hsPDA according to the presence and hemodynamical significance of PDA by echocardiography. hsPDA group was treated with IV or oral ibuprofen. Results: Patent ductus arteriosus was diagnosed by routine echocardiography in 145 patients, of whom 78 (53.7% had hsPDA. All 65 infants with hiPDA had spontaneous PDA closure. Echocardiographic measurements were similar to those patients treated with oral or IV ibuprofen, as in the response rate to treatment without serious adverse effects. The presence of respiratory distress syndrome, surfactant therapy, late sepsis, bronchopulmonary dysplasia (BPD and mortality rates were significantly higher in patients with hsPDA. However, with stepwise logistic regression; 5th min Apgar score (odds ratio [OR], 1.321, 95% confidence interval [CI], 1.063-1.641, P = 0.012 and gestational age (OR, 1.422, 95% CI, 1.212-1.662, P < 0.001 were the only significant variables associated with mortality. Gestational age (OR, 0.680, 95% CI, 0.531-0.871, P = 0.002 was the only significant variable associated with BPD shown with logistic regression. Conclusion: Ibuprofen treatment is effective for hsPDA closure with minimal side effects. HiPDA can close spontaneously; therefore treatment decision should be individualized. However, medical treatment of PDA does not reduce

  8. Bronchopulmonary dysplasia: clinical grading in relation to ventilation/perfusion mismatch measured by single photon emission computed tomography.

    Science.gov (United States)

    Kjellberg, Malin; Björkman, Karin; Rohdin, Malin; Sanchez-Crespo, Alejandro; Jonsson, Baldvin

    2013-12-01

    Bronchopulmonary dysplasia (BPD) is a significant cause of morbidity in the preterm population. Clinical severity grading based on the need for supplemental oxygen and/or need for positive airway pressure at 36 weeks postmenstrual age does not yield reproducible predictive values for later pulmonary morbidity. Single photon emission computed tomography (SPECT) was used to measure the distribution of lung ventilation (V) and perfusion (Q) in 30 BPD preterm infants at a median age of 37 weeks postmenstrual age. The V and Q were traced with 5 MBq Technegas and Technetium-labeled albumin macro aggregates, respectively, and the V/Q match-mismatch was used to quantify the extent of lung function impairment. The latter was then compared with the clinical severity grading at 36 weeks, and time spent on mechanical ventilation, continuous positive airway pressure (CPAP) and supplemental oxygen. Of those with mild and moderate BPD 3/9 and 3/11 patients, respectively, showed significant V/Q mismatches. By contrast, 4/10 patients with severe BPD showed a satisfactory V/Q matching distribution. An unsatisfactory V/Q match was not correlated with time spent on supplemental oxygen or CPAP, but was significantly negatively correlated with time spent on mechanical ventilation. SPECT provides unique additional information about regional lung function. The results suggest that the current clinical severity grading can be improved and/or complemented with SPECT. © 2013 Wiley Periodicals, Inc.

  9. Transcatheter elimination of left-to-right shunts in infants with bronchopulmonary dysplasia is feasible and safe.

    Science.gov (United States)

    Wood, Amy M; Holzer, Ralf J; Texter, Karen M; Hill, Sharon L; Gest, Alfred L; Welty, Stephen E; Cheatham, John P; Yates, Andrew R

    2011-01-01

    To test the hypothesis that transcatheter elimination of left-to-right (L-R) cardiac shunts in former premature infants with bronchopulmonary dysplasia (BPD) is feasible, safe, and is associated with an improvement in respiratory status. Retrospective case review. Twelve patients with BPD who underwent an attempt at transcatheter closure of an L-R shunt lesion within the first year of life at a single center. Median weight was 5.4 kg and median age was 6 months. Fifteen L-R shunt lesions included patent ductus arteriosus (n = 1), atrial septal defect (ASD) (n = 9), and aortopulmonary collaterals (n = 5). Echocardiographic and clinical markers were collected before and after intervention as well as procedural variables including successful elimination of the shunt and procedural complications. The L-R shunts were successfully occluded in 11/12 (91.6%) patients without any significant procedural adverse event. The ASD closure group demonstrated a decrease in right heart size after the procedure. All patients required respiratory support prior to, and 1 month after, the procedure while only 5/10 (50%) required respiratory support at 12 months of age (P = .0129). There was no change in the median weight percentile over time. Transcatheter occlusion of L-R shunts can be performed safely and effectively in children with BPD. Further studies may clarify the role of such therapy in improvement in respiratory physiology over time. © 2011 Copyright the Authors. Congenital Heart Disease © 2011 Wiley Periodicals, Inc.

  10. The Relationship of Nosocomial Infection Reduction to Changes in Neonatal Intensive Care Unit Rates of Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Lapcharoensap, Wannasiri; Kan, Peiyi; Powers, Richard J; Shaw, Gary M; Stevenson, David K; Gould, Jeffrey B; Wirtschafter, David D; Lee, Henry C

    2017-01-01

    To examine whether recent reductions in rates of nosocomial infection have contributed to changes in rates of bronchopulmonary dysplasia (BPD) in a population-based cohort. This was a retrospective, population-based cohort study that used the California Perinatal Quality Care Collaborative database from 2006 to 2013. Eligible infants included those less than 30 weeks' gestational age and less than 1500 g who survived to 3 days of life. Primary variables of interest were rates of nosocomial infections and BPD. Adjusted rates of nosocomial infections and BPD from a baseline period (2006-2010) were compared with a later period (2011-2013). The correlation of changes in rates across periods for both variables was assessed by hospital of care. A total of 22 967 infants from 129 hospitals were included in the study. From the first to second time period, the incidence of nosocomial infections declined from 24.7% to 15% and BPD declined from 35% to 30%. Adjusted hospital rates of BPD and nosocomial infections were correlated positively with a calculated 8% reduction of BPD rates attributable to reductions in nosocomial infections. Successful interventions to reduce rates of nosocomial infections may have a positive impact on other comorbidities such as BPD. The prevention of nosocomial infections should be viewed as a significant component in avoiding long-term neonatal morbidities. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. The levels of the neutrophil elastase in the amniotic fluid of pregnant women whose infants develop bronchopulmonary dysplasia.

    Science.gov (United States)

    Ikeda, Sayako; Kihira, Kana; Yokoi, Akira; Tamakoshi, Koji; Miyazaki, Ken; Furuhashi, Madoka

    2015-03-01

    To clarify the association between amniotic neutrophil elastase levels and the development of bronchopulmonary dysplasia (BPD). The database between July 2001 and December 2012 was reviewed for women with amniocentesis on admission for amniotic fluid neutrophil elastase levels and with singleton deliveries between 22 + 0 and 31 + 6 weeks of gestation. Following deliveries, placentas were examined for histologic chorioamnionitis. The peripheral blood of the neonates was analyzed for acute phase reactants. Among 294 infants, no, mild, moderate or severe BPD was observed in 126, 89, 40 and 39 infants, respectively. The medians of gestational age on admission, at premature rupture of membranes and at delivery were significantly smaller in BPD (+) when compared with BPD (-) (p < 0.001). The median level of amniotic neutrophil elastase on admission was significantly greater in BPD (+) than that in BPD (-). Histologic chorioamnionitis and funisitis were both detected more frequently in BPD (+) patients than in BPD (-) patients. In a logistic regression model, the only variable that affected an increased chance of BPD was the gestational age at delivery (odds ratio, 0.58; 95% confidence interval, 0.36-0.92; p = 0.021). The level of amniotic neutrophil elastase cannot be a definitive risk factor for BPD.

  12. Angiogenesis-related genes may be a more important factor than matrix metalloproteinases in bronchopulmonary dysplasia development.

    Science.gov (United States)

    Yang, Min; Chen, Bo-Lin; Huang, Jian-Bao; Meng, Yan-Ni; Duan, Xiao-Jun; Chen, Lu; Li, Lin-Rui; Chen, Yan-Ping

    2017-03-21

    We characterized the expression profile of angiogenesis-related genes (ARG) and matrix metalloproteinase (MMP) genes in preterm infants, with and without bronchopulmonary dysplasia (BPD). We reanalyzed a gene expression dataset for preterm infants from the Gene Expression Omnibus database using the Gene-Cloud of Biotechnology Information platform. A total of 1,652 genes were differentially (1.2-fold change) expressed: 811 were highly expressed in infants with BPD, and 841 were highly expressed in those without BPD. Twenty-eight and 11 ARGs were upregulated in infants with and without BPD, respectively. Among 27 detected MMPs and TIMPs, MMP8, MMP9, MMP25, TIMP2 and TIMP3 were differently expressed. Levels of THBS1, MMP8, MMP9, MMP25, TIMP2 and TIMP3 increased as severity of BPD and retinopathy of prematurity (ROP) increased, whereas ETS1, LEF1 and SPOCK2 exhibited the opposite trend. Expression of ETS1 and LEF1 had a fitting rate of R2 = 0.849 and P < 0.001. ELISAs showed a positive correlation between THBS1 and CD36 (receptor of THBS1) levels in serum samples from preterm infants. Our study indicates that the upregulation of THBS1 and downregulation of ETS1, LEF1 promotes BPD in preterm infants by disrupting blood vessel formation rather than by dysregulation of MMPs and TIMPs.

  13. Macrolides do not affect the incidence of moderate and severe bronchopulmonary dysplasia in symptomatic ureaplasma-positive infants.

    Science.gov (United States)

    Anbu Chakkarapani, Aravanan; Paes, Bosco; Shivananda, Sandesh

    2015-10-01

    The aim of this study was to compare the incidence of bronchopulmonary dysplasia (BPD) in symptomatic ureaplasma-positive treated preterm infants and asymptomatic preterm infants not tested or treated for ureaplasma. A retrospective matched cohort study was conducted in a tertiary, neonatal unit between January 2007 and December 2012. Infants ≤29 completed weeks with signs and symptoms suggesting ureaplasma pneumonia who received macrolides comprised the study group. Infants ≤29 weeks without signs and symptoms not tested or treated with macrolides were the controls. Infants were mandatorily matched for gestational age ± one week or birthweight ± 100 grams. There were 31 infants in the study group and 62 in the control group. The baseline demographic data of both groups were similar on the whole. The incidence of moderate and severe BPD, defined by oxygen dependency or the need for continuous positive airway pressure at 36 weeks of postconceptual age, was 45.2% in the study group and 40.3% in the controls (p = 0.65). There was no significant difference in morbidities or mortality between the groups. A selective approach of treating symptomatic ureaplasma-positive preterm infants with macrolides did not affect the incidence of moderate and severe BPD. ©2015 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

  14. Neonatal bronchopulmonary dysplasia increases neuronal apoptosis in the hippocampus through the HIF-1α and p53 pathways.

    Science.gov (United States)

    Yin, Rong; Yuan, Lin; Ping, Lili; Hu, Liyuan

    2016-01-01

    Neonatal bronchopulmonary dysplasia (BPD) might lead to an increased risk for brain injury. The present study aims to investigate the effects of neonatal BPD on neuronal apoptosis in the hippocampus and cognitive function and to explore the underlying mechanisms. The results revealed that BPD model rat pups exhibited more apoptotic cells in the hippocampus and longer escape latencies in the Morris maze test. Both the caspase-dependent and caspase-nondependent signal pathways were activated. Further examinations showed an elevated p53 level by BPD via HIF-1α induction, while the caspase-3 in the hippocampus was suppressed by both HIF-1α and p53 inhibitor. These findings suggested that neonatal BPD caused impaired cognitive function and neuron apoptosis in hippocampus via p53 and HIF-1α. Although the precise mechanism requires further investigation, this study provided new evidence for and an explanation of the impaired CNS developmental outcomes of BPD. Copyright © 2015 Elsevier B.V. All rights reserved.

  15. Bone marrow-derived mesenchymal stem cells protect against lung injury in a mouse model of bronchopulmonary dysplasia.

    Science.gov (United States)

    Luan, Yun; Ding, Wei; Ju, Zhi-Ye; Zhang, Zhao-Hua; Zhang, Xue; Kong, Feng

    2015-03-01

    The aim of the present study was to investigate the effect of bone marrow‑derived mesenchymal stem cells (BMSCs) in the treatment of lung injury in a mouse model of bronchopulmonary dysplasia (BPD) and examine the underlying mechanisms. A mouse model of BPD was created using continuous exposure to high oxygen levels for 14 days. BMSCs were isolated, cultured and then labeled with green fluorescent protein. Cells (1x106) were subsequently injected intravenously 1 h prior to high oxygen treatment. Animals were randomly divided into three groups (n=5 in each): Control group, BPD model group and BMSC injection group. At two weeks post‑treatment, the expression of transforming growth factor‑β1 (TGF‑β1), vascular endothelial growth factor (VEGF) and von Willebrand factor (vWF) was detected using immunohistochemical staining and immunofluorescence. Compared with the BPD model group, the body weight, airway structure and levels of TGF‑β1 and VEGF were significantly improved in the BMSC‑treated group. Immunofluorescence observations indicated that BMSCs were able to differentiate into cells expressing vWF and VEGF, which are markers of vascular tissues. The present study demonstrated that intravenous injection of BMSCs significantly improved lung damage in a neonatal mouse model of BPD at 14 days following hyperoxia‑induced injury. This provides novel information which may be used to guide further investigation into the use of stem cells in BPD.

  16. The airway microbiome of intubated premature infants: characteristics and changes that predict the development of bronchopulmonary dysplasia.

    Science.gov (United States)

    Lohmann, Pablo; Luna, Ruth A; Hollister, Emily B; Devaraj, Sridevi; Mistretta, Toni-Ann; Welty, Stephen E; Versalovic, James

    2014-09-01

    Bronchopulmonary dysplasia (BPD) is associated with perinatal inflammatory triggers. Methods targeting bacterial rRNA may improve detection of microbial colonization in premature infants. We hypothesize that respiratory microbiota differs between preterm infants who develop BPD and those unaffected and correlates with inflammatory mediator concentrations. Twenty-five infants, born at ≤32 wk of gestation and intubated in the first 24 h, were enrolled. Tracheal aspirates were obtained at intubation and on days 3, 7, and 28. Bacterial DNA was extracted, and 16S rRNA genes were amplified and sequenced. Concentrations of interleukins (IL-1β, IL-6, IL-8, IL-10, and IL-12), tumor necrosis factor-α, interferon-γ, lipopolysaccharide (LPS), and lipoteichoic acid (LTA) were measured. Chorioamnionitis was diagnosed by histology. BPD was defined as an oxygen requirement at 36 wk postmenstrual age. Acinetobacter was the predominant genus in the airways of all infants at birth. Ten infants developed BPD and showed reduced bacterial diversity at birth. No differences were detected in bacterial diversity, cytokines, LPS, and LTA from infants with and without exposure to chorioamnionitis. The airways of premature infants are not sterile at birth. Reduced diversity of the microbiome may be an important factor in the development of BPD and is not associated with differences in inflammatory mediators.

  17. Progressive Vascular Functional and Structural Damage in a Bronchopulmonary Dysplasia Model in Preterm Rabbits Exposed to Hyperoxia.

    Science.gov (United States)

    Jiménez, Julio; Richter, Jute; Nagatomo, Taro; Salaets, Thomas; Quarck, Rozenn; Wagennar, Allard; Wang, Hongmei; Vanoirbeek, Jeroen; Deprest, Jan; Toelen, Jaan

    2016-10-24

    Bronchopulmonary dysplasia (BPD) is caused by preterm neonatal lung injury and results in oxygen dependency and pulmonary hypertension. Current clinical management fails to reduce the incidence of BPD, which calls for novel therapies. Fetal rabbits have a lung development that mimics humans and can be used as a translational model to test novel treatment options. In preterm rabbits, exposure to hyperoxia leads to parenchymal changes, yet vascular damage has not been studied in this model. In this study we document the early functional and structural changes of the lung vasculature in preterm rabbits that are induced by hyperoxia after birth. Pulmonary artery Doppler measurements, micro-CT barium angiograms and media thickness of peripheral pulmonary arteries were affected after seven days of hyperoxia when compared to controls. The parenchyma was also affected both at the functional and structural level. Lung function testing showed higher tissue resistance and elastance, with a decreased lung compliance and lung capacity. Histologically hyperoxia leads to fewer and larger alveoli with thicker walls, less developed distal airways and more inflammation than normoxia. In conclusion, we show that the rabbit model develops pulmonary hypertension and developmental lung arrest after preterm lung injury, which parallel the early changes in human BPD. Thus it enables the testing of pharmaceutical agents that target the cardiovascular compartment of the lung for further translation towards the clinic.

  18. Protective effect of chorioamnionitis on the development of bronchopulmonary dysplasia triggered by postnatal systemic inflammation in neonatal rats.

    Science.gov (United States)

    Choi, Chang Won; Lee, Juyoung; Oh, Joo Youn; Lee, Seung Hyun; Lee, Hyun Ju; Kim, Beyong Il

    2016-02-01

    Prenatal or postnatal systemic inflammation can contribute to the development of bronchopulmonary dysplasia (BPD). We investigated whether prenatal intra-amniotic (i.a.) inflammation or early postnatal systemic inflammation can induce BPD in a rat model. One microgram of lipopolysaccharide (LPS) or vehicle was injected into the amniotic sacs 2 d before delivery (E20). After birth, 0.25 mg/kg of LPS or vehicle was injected into the peritoneum of pups on postnatal day (P)1, P3, and P5. On P7 and P14, peripheral blood (PB), bronchoalveolar lavage fluid (BALF), and lung tissue were obtained and analyzed. Postnatal i.p. injections of LPS significantly increased neutrophil counts in PB and BALF on P7 and P14. Similarly, proinflammatory cytokine and angiogenic factor transcript levels were increased in the lung by i.p. LPS on P7. Alveolar and pulmonary vascular development was markedly disrupted by i.p. LPS on P14. However, pretreatment with i.a. LPS significantly negated the detrimental effects of postnatal i.p. LPS on PB and BALF neutrophil counts and on lung proinflammatory cytokine expression and histopathological changes. Exposure to early postnatal systemic LPS induces BPD, an arrest in alveolarization, in neonatal rats. Preceding exposure to i.a. LPS protects the lungs against BPD triggered by postnatal systemic inflammation.

  19. The Natural History of Bronchopulmonary Dysplasia: The Case for Primary Prevention.

    Science.gov (United States)

    McEvoy, Cindy T; Aschner, Judy L

    2015-12-01

    Brochopulmonary dysplasia (BPD) is the most common form of chronic lung disease in infancy. At present, BPD primarily occurs in extremely premature infants (23-28 weeks of gestation) born during the late canalicular/early saccular stage of lung development. This article summarizes the current knowledge of the life course of BPD by emphasizing recent or key articles notating its natural history from the newborn period through adulthood and building the case for a continued focus on its primary prevention. Copyright © 2015 Elsevier Inc. All rights reserved.

  20. Effect of early low-dose hydrocortisone on survival without bronchopulmonary dysplasia in extremely preterm infants (PREMILOC): a double-blind, placebo-controlled, multicentre, randomised trial.

    Science.gov (United States)

    Baud, Olivier; Maury, Laure; Lebail, Florence; Ramful, Duksha; El Moussawi, Fatima; Nicaise, Claire; Zupan-Simunek, Véronique; Coursol, Anne; Beuchée, Alain; Bolot, Pascal; Andrini, Pierre; Mohamed, Damir; Alberti, Corinne

    2016-04-30

    Bronchopulmonary dysplasia, a major complication of extreme prematurity, has few treatment options. Postnatal steroid use is controversial, but low-dose hydrocortisone might prevent the harmful effects of inflammation on the developing lung. In this study, we aimed to assess whether low-dose hydrocortisone improved survival without bronchopulmonary dysplasia in extremely preterm infants. In this double-blind, placebo-controlled, randomised trial done at 21 French tertiary-care neonatal intensive care units (NICUs), we randomly assigned (1:1), via a secure study website, extremely preterm infants inborn (born in a maternity ward at the same site as the NICU) at less than 28 weeks of gestation to receive either intravenous low-dose hydrocortisone or placebo during the first 10 postnatal days. Infants randomly assigned to the hydrocortisone group received 1 mg/kg of hydrocortisone hemisuccinate per day divided into two doses per day for 7 days, followed by one dose of 0·5 mg/kg per day for 3 days. Randomisation was stratified by gestational age and all infants were enrolled by 24 h after birth. Study investigators, parents, and patients were masked to treatment allocation. The primary outcome was survival without bronchopulmonary dysplasia at 36 weeks of postmenstrual age. We used a sequential analytical design, based on intention to treat, to avoid prolonging the trial after either efficacy or futility had been established. This trial is registered with ClinicalTrial.gov, number NCT00623740. 1072 neonates were screened between May 25, 2008, and Jan 31, 2014, of which 523 were randomly assigned (256 hydrocortisone, 267 placebo). 255 infants on hydrocortisone and 266 on placebo were included in analyses after parents withdrew consent for one child in each group. Of the 255 infants assigned to hydrocortisone, 153 (60%) survived without bronchopulmonary dysplasia, compared with 136 (51%) of 266 infants assigned to placebo (odds ratio [OR] adjusted for gestational age

  1. Early respiratory management of respiratory distress syndrome in very preterm infants and bronchopulmonary dysplasia: a case-control study.

    Directory of Open Access Journals (Sweden)

    Arjan B Te Pas

    Full Text Available BACKGROUND: In the period immediately after birth, preterm infants are highly susceptible to lung injury. Early nasal continuous positive airway pressure (ENCPAP is an attempt to avoid intubation and may minimize lung injury. In contrast, ENCPAP can fail, and at that time surfactant rescue can be less effective. OBJECTIVE: To compare the pulmonary clinical course and outcome of very preterm infants (gestational age 25-32 weeks with respiratory distress syndrome (RDS who started with ENCPAP and failed (ECF group, with a control group of infants matched for gestational age, who were directly intubated in the delivery room (DRI group. Primary outcome consisted of death during admission or bronchopulmonary dysplasia (BPD. RESULTS: 25 infants were included in the ECF group and 50 control infants matched for gestational age were included in the DRI group. Mean gestational age and birth weight in the ECF group were 29.7 weeks and 1,393 g and in the DRI group 29.1 weeks and 1,261 g (p = NS. The incidence of BPD was significantly lower in the ECF group than in the DRI group (4% vs. 35%; P<0.004; OR 12.6 (95% CI 1.6-101. Neonatal mortality was similar in both groups (4%. The incidence of neonatal morbidities such as severe cerebral injury, patent ductus arteriosus, necrotizing enterocolitis and retinopathy of prematurity, was not significantly different between the two groups. CONCLUSION: A trial of ENCPAP at birth may reduce the incidence of BPD and does not seem to be detrimental in very preterm infants. Randomized controlled trials are needed to test whether early respiratory management of preterm infants with RDS plays an important role in the development of BPD.

  2. Normal pulmonary gas exchange efficiency and absence of exercise-induced arterial hypoxemia in adults with bronchopulmonary dysplasia.

    Science.gov (United States)

    Lovering, Andrew T; Laurie, Steven S; Elliott, Jonathan E; Beasley, Kara M; Yang, Ximeng; Gust, Caitlyn E; Mangum, Tyler S; Goodman, Randall D; Hawn, Jerold A; Gladstone, Igor M

    2013-10-01

    Cardiopulmonary function is reduced in adults born very preterm, but it is unknown if this results in reduced pulmonary gas exchange efficiency during exercise and, consequently, leads to reduced aerobic capacity in subjects with and without bronchopulmonary dysplasia (BPD). We hypothesized that an excessively large alveolar to arterial oxygen difference (AaDO2) and resulting exercise-induced arterial hypoxemia (EIAH) would contribute to reduced aerobic fitness in adults born very preterm with and without BPD. Measurements of pulmonary function, lung volumes and diffusion capacity for carbon monoxide (DLco) were made at rest. Measurements of maximal oxygen consumption, peak workload, temperature- and tonometry-corrected arterial blood gases, and direct measure of hemoglobin saturation with oxygen (SaO2) were made preexercise and during cycle ergometer exercise in ex-preterm subjects ≤32-wk gestational age, with BPD (n = 12), without BPD (PRE; n = 12), and full term controls (CONT; n = 12) breathing room air. Both BPD and PRE had reduced pulmonary function and reduced DLco compared with CONT. The AaDO2 was not significantly different between groups, and there was no evidence of EIAH (SaO2 group preexercise or at any workload. Arterial O2 content was not significantly different between the groups preexercise or during exercise. However, peak power output was decreased in BPD and PRE subjects compared with CONT. We conclude that EIAH in adult subjects born very preterm with and without BPD does not likely contribute to the reduction in aerobic exercise capacity observed in these subjects.

  3. Addition of SNAP to perinatal risk factors improves the prediction of bronchopulmonary dysplasia or death in critically ill preterm infants.

    Science.gov (United States)

    Li, Yanhong; Yan, Jie; Li, Mengxia; Xiao, Zhihui; Zhu, Xueping; Pan, Jian; Li, Xiaozhong; Feng, Xing

    2013-09-10

    Bronchopulmonary dysplasia (BPD) is the most common serious pulmonary morbidity in premature infants. The score for neonatal acute physiology (SNAP) is a physiologic severity index for neonatal intensive care and correlates well with neonatal mortality and clinical outcomes. The prognostic value of the SNAP score for BPD in preterm infants remains to be clarified. The aim of the study was to determine whether SNAP can predict the development of BPD or death, and to investigate the contribution of SNAP to the predictive accuracy of other potential perinatal risk factors for the adverse outcome in critically ill preterm infants. We conducted a study in 160 critically ill preterm infants with less than 33 gestational weeks. The original SNAP score was prospectively calculated based on 28 items collected during the first 24 hours of admission. The potential perinatal risk factors were assessed during the first 72 hours of life. Major outcome measures were BPD and mortality before the time of BPD screening. Of the 160 infants, 17 died and 41 developed BPD. The SNAP score was significantly associated with BPD or death (odds ratio [OR] =1.28; 95% confidence interval [CI], 1.16-1.41; p death (area under the curve [AUC] =0.78; 95% CI, 0.70-0.85; p perinatal risk factors. Multivariate regression analysis resulted in a final model, including SNAP, gestational age, apnea of prematurity, patent ductus arteriosus, and surfactant use as independent risk factors, with a higher predictive accuracy compared with individual components (AUC =0.92; 95% CI, 0.87-0.96; p death. High SNAP scores are predictive of BPD or death in critically ill preterm infants, and add prognostic value to other perinatal risk factors.

  4. Trends in Survival and Incidence of Bronchopulmonary Dysplasia in Extremely Preterm Infants at 23–26 Weeks Gestation

    Science.gov (United States)

    2016-01-01

    The aim of this study was to investigate the relationship between survival and incidence of bronchopulmonary dysplasia (BPD) in extremely premature infants, and identify clinical factors responsible for this association. Medical records of 350 infants at 23–26 weeks gestation from 2000 to 2005 (period I, n = 137) and 2006 to 2010 (period II, n = 213) were retrospectively reviewed. The infants were stratified into 23–24 and 25–26 weeks gestation, and the survival, BPD incidence, and clinical characteristics were analyzed. BPD was defined as oxygen dependency at 36 weeks postmenstrual age. The overall survival rate was significantly improved in period II compared to period I (80.3% vs. 70.0%, respectively; P = 0.028), especially in infants at 23–24 weeks gestation (73.9% vs. 47.4%, respectively; P = 0.001). The BPD incidence in survivors during period II (55.0%) was significantly decreased compared to period I (67.7%; P = 0.042), especially at 25–26 weeks gestation (41.7% vs. 62.3%, respectively; P = 0.008). Significantly improved survival at 23–24 weeks gestation was associated with a higher antenatal steroid use and an improved 5-minute Apgar score. A significant decrease in BPD incidence at 25–26 weeks gestation was associated with early extubation, prolonged use of less invasive continuous positive airway pressure, and reduced supplemental oxygen. Improved perinatal and neonatal care can simultaneously lead to improved survival and decreased BPD incidence in extremely premature infants. PMID:26955244

  5. Trends in Survival and Incidence of Bronchopulmonary Dysplasia in Extremely Preterm Infants at 23-26 Weeks Gestation.

    Science.gov (United States)

    Kim, Jin Kyu; Chang, Yun Sil; Sung, Sein; Ahn, So Yoon; Yoo, Hye Soo; Park, Won Soon

    2016-03-01

    The aim of this study was to investigate the relationship between survival and incidence of bronchopulmonary dysplasia (BPD) in extremely premature infants, and identify clinical factors responsible for this association. Medical records of 350 infants at 23-26 weeks gestation from 2000 to 2005 (period I, n = 137) and 2006 to 2010 (period II, n = 213) were retrospectively reviewed. The infants were stratified into 23-24 and 25-26 weeks gestation, and the survival, BPD incidence, and clinical characteristics were analyzed. BPD was defined as oxygen dependency at 36 weeks postmenstrual age. The overall survival rate was significantly improved in period II compared to period I (80.3% vs. 70.0%, respectively; P = 0.028), especially in infants at 23-24 weeks gestation (73.9% vs. 47.4%, respectively; P = 0.001). The BPD incidence in survivors during period II (55.0%) was significantly decreased compared to period I (67.7%; P = 0.042), especially at 25-26 weeks gestation (41.7% vs. 62.3%, respectively; P = 0.008). Significantly improved survival at 23-24 weeks gestation was associated with a higher antenatal steroid use and an improved 5-minute Apgar score. A significant decrease in BPD incidence at 25-26 weeks gestation was associated with early extubation, prolonged use of less invasive continuous positive airway pressure, and reduced supplemental oxygen. Improved perinatal and neonatal care can simultaneously lead to improved survival and decreased BPD incidence in extremely premature infants.

  6. Histologic Chorioamnionitis and Bronchopulmonary Dysplasia in Preterm Infants: The Epidemiologic Study on Low Gestational Ages 2 Cohort.

    Science.gov (United States)

    Torchin, Héloïse; Lorthe, Elsa; Goffinet, François; Kayem, Gilles; Subtil, Damien; Truffert, Patrick; Devisme, Louise; Benhammou, Valérie; Jarreau, Pierre-Henri; Ancel, Pierre-Yves

    2017-08-01

    To investigate the association between histologic chorioamnionitis (HCA) and bronchopulmonary dysplasia (BPD) in very preterm infants, both in a general population and for those born after spontaneous preterm labor and after preterm premature rupture of membranes (pPROM). This study included 2513 live born singletons delivered at 24-31 weeks of gestation from a national prospective population-based cohort of preterm births; 1731 placenta reports were available. HCA was defined as neutrophil infiltrates in the amnion, chorion of the membranes, or chorionic plate, associated or not with funisitis. The main outcome measure was moderate or severe BPD. Analyses involved logistic regressions and multiple imputation for missing data. The incidence of HCA was 28.4% overall: 38% in cases of preterm labor, 64% in cases of pPROM, and less than 5% in cases of vascular disorders. Overall, the risk of BPD after adjustment for gestational age, sex, and antenatal steroids was reduced for infants with HCA (HCA alone: aOR 0.6 [95% CI 0.4-0.9]; associated with funisitis: aOR 0.5 [95% CI 0.3-0.8]). This finding was explained by the high rate of BPD and low rate of chorioamnionitis among children with fetal growth restriction. HCA was not associated with BPD in the preterm labor (13.4% vs 8.5%; aOR 0.9; 95% CI 0.5-1.8) or in the pPROM group (12.9% vs 12.1%; aOR 0.6; 95% CI 0.3-1.3). In homogeneous groups of infants born after preterm labor or pPROM, HCA is not associated with BPD. Copyright © 2017 Elsevier Inc. All rights reserved.

  7. Airway Remodeling and Hyperreactivity in a Model of Bronchopulmonary Dysplasia and Their Modulation by IL-1 Receptor Antagonist.

    Science.gov (United States)

    Royce, Simon G; Nold, Marcel F; Bui, Christine; Donovan, Chantal; Lam, Maggie; Lamanna, Emma; Rudloff, Ina; Bourke, Jane E; Nold-Petry, Claudia A

    2016-12-01

    Bronchopulmonary dysplasia (BPD) is a chronic disease of extreme prematurity that has serious long-term consequences including increased asthma risk. We earlier identified IL-1 receptor antagonist (IL-1Ra) as a potent inhibitor of murine BPD induced by combining perinatal inflammation (intraperitoneal LPS to pregnant dams) and exposure of pups to hyperoxia (fraction of inspired oxygen = 0.65). In this study, we determined whether airway remodeling and hyperresponsiveness similar to asthma are evident in this model, and whether IL-1Ra is protective. During 28-day exposure to air or hyperoxia, pups received vehicle or 10 mg/kg IL-1Ra by daily subcutaneous injection. Lungs were then prepared for histology and morphometry of alveoli and airways, or for real-time PCR, or inflated with agarose to prepare precision-cut lung slices to visualize ex vivo intrapulmonary airway contraction and relaxation by phase-contrast microscopy. In pups reared under normoxic conditions, IL-1Ra treatment did not affect alveolar or airway structure or airway responses. Pups reared in hyperoxia developed a severe BPD-like lung disease, with fewer, larger alveoli, increased subepithelial collagen, and increased expression of α-smooth muscle actin and cyclin D1. After hyperoxia, methacholine elicited contraction with similar potency but with an increased maximum reduction in lumen area (air, 44%; hyperoxia, 89%), whereas dilator responses to salbutamol were maintained. IL-1Ra treatment prevented hyperoxia-induced alveolar disruption and airway fibrosis but, surprisingly, not the increase in methacholine-induced airway contraction. The current study is the first to demonstrate ex vivo airway hyperreactivity caused by systemic maternal inflammation and postnatal hyperoxia, and it reveals further preclinical mechanistic insights into IL-1Ra as a treatment targeting key pathophysiological features of BPD.

  8. The development of lung biochemical monitoring can play a key role in the early prediction of bronchopulmonary dysplasia.

    Science.gov (United States)

    Fabiano, Adele; Gavilanes, Antonio W D; Zimmermann, Luc J I; Kramer, Boris W; Paolillo, Piermichele; Livolti, Giovanni; Picone, Simonetta; Bressan, Katia; Gazzolo, Diego

    2016-05-01

    Despite advances in perinatal management, there is a flat trend in incidences of respiratory distress syndrome (RDS) and bronchopulmonary dysplasia (BPD) in preterm infants. The main feature of BPD development in preterm infants is an imbalance between increased exposure to free radicals and inadequate antioxidant defences. We investigated the associations between BPD and lipid hydro-peroxide (LOOH) and glutathione (GSH) concentrations in bronchoalveolar lavage fluid (BALF). In this prospective study, BALF samples were collected from 44 preterm infants with RDS and oxidative stress markers were measured in 11 with BPD and 33 controls without BPD. LOOH levels were significantly higher (p < 0.01) in the BPD group (median 16.35; 25th-75th centile 13.75-17.05 nmol/mL) than in the no BPD group (median 13.18; 25th-75th centile 12.92-13.63 nmol/mL). Conversely, GSH levels were significantly lower in the BPD group (p < 0.01) (median 11.52; 25th-75th centile 6.95-13.85 μmol/mg) than the no BPD group (median: 18.69; 25th-75th centile: 13.89-23.64 μmol/mg). Multiple regression analysis showed significant correlations between BPD and mechanical ventilation time (p < 0.01) and LOOH levels (p < 0.05). Early LOOH level increases in preterm infants developing BPD suggest that lung biochemical monitoring of sick infants might be possible and BPD could be predicted early by evaluating biomarkers. ©2015 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

  9. Gestational Exposure to Sidestream (Secondhand) Cigarette Smoke Promotes Transgenerational Epigenetic Transmission of Exacerbated Allergic Asthma and Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Singh, Shashi P; Chand, Hitendra S; Langley, Raymond J; Mishra, Neerad; Barrett, Ted; Rudolph, Karin; Tellez, Carmen; Filipczak, Piotr T; Belinsky, Steve; Saeed, Ali I; Sheybani, Aryaz; Exil, Vernat; Agarwal, Hemant; Sidhaye, Venkataramana K; Sussan, Thomas; Biswal, Shyam; Sopori, Mohan

    2017-05-15

    Embryonic development is highly sensitive to xenobiotic toxicity and in utero exposure to environmental toxins affects physiological responses of the progeny. In the United States, the prevalence of allergic asthma (AA) is inexplicably rising and in utero exposure to cigarette smoke increases the risk of AA and bronchopulmonary dysplasia (BPD) in children and animal models. We reported that gestational exposure to sidestream cigarette smoke (SS), or secondhand smoke, promoted nicotinic acetylcholine receptor-dependent exacerbation of AA and BPD in mice. Recently, perinatal nicotine injections in rats were reported to induce peroxisome proliferator-activated receptor γ-dependent transgenerational transmission of asthma. Herein, we show that first generation and second generation progeny from gestationally SS-exposed mice exhibit exacerbated AA and BPD that is not dependent on the decrease in peroxisome proliferator-activated receptor γ levels. Lungs from these mice show strong eosinophilic infiltration, excessive Th2 polarization, marked airway hyperresponsiveness, alveolar simplification, decreased lung compliance, and decreased lung angiogenesis. At the molecular level, these changes are associated with increased RUNX3 expression, alveolar cell apoptosis, and the antiangiogenic factor GAX, and decreased expression of HIF-1α and proangiogenic factors NF-κB and VEGFR2 in the 7-d first generation and second generation lungs. Moreover, the lungs from these mice exhibit lower levels of microRNA (miR)-130a and increased levels of miR-16 and miR-221. These miRs regulate HIF-1α-regulated apoptotic, angiogenic, and immune pathways. Thus the intergenerational effects of gestational SS involve epigenetic regulation of HIF-1α through specific miRs contributing to increased incidence of AA and BPD in the progenies. Copyright © 2017 by The American Association of Immunologists, Inc.

  10. How to decrease bronchopulmonary dysplasia in your neonatal intensive care unit today and “tomorrow” [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Leif D. Nelin

    2017-04-01

    Full Text Available Bronchopulmonary dysplasia, or BPD, is the most common chronic lung disease in infants. Genetic predisposition and developmental vulnerability secondary to antenatal and postnatal infections, compounded with exposure to hyperoxia and invasive mechanical ventilation to an immature lung, result in persistent inflammation, culminating in the characteristic pulmonary phenotype of BPD of impaired alveolarization and dysregulated vascularization. In this article, we highlight specific areas in current management, and speculate on therapeutic strategies that are on the horizon, that we believe will make an impact in decreasing the incidence of BPD in your neonatal intensive care units.

  11. Fatores maternos e neonatais na incidência de displasia broncopulmonar em recém-nascidos de muito baixo peso Maternal and neonatal factors affecting the incidence of bronchopulmonary dysplasia in very low birth weight newborns

    Directory of Open Access Journals (Sweden)

    Gicelle S. Cunha

    2003-11-01

    Full Text Available OBJETIVO: Obter a incidência de displasia broncopulmonar (DBP; avaliar os fatores maternos e neonatais associados com a doença; determinar a correlação entre DBP e a evolução dos recém-nascidos. MÉTODOS: Os dados foram coletados prospectivamente de 153 recém-nascidos com peso de nascimento inferior a 1.500 g, nascidos em Campinas de setembro de 2000 a abril de 2002 e tratados no Hospital Universitário. Foram utilizados razão de taxas de incidências com intervalo de confiança de 95% (IC 95%, regressão Breslow-Cox, teste t de Student, regressão linear e teste exato de Fisher. RESULTADOS: Entre os 124 recém-nascidos que sobreviveram aos 28 dias de vida, 33 (26,6% apresentavam DBP. Peso de nascimento OBJECTIVE: To determine the incidence of bronchopulmonary dysplasia, to identify maternal and neonatal factors associated with the disease, and to determine the correlation between bronchopulmonary dysplasia and the progress of newborns. METHODS: Data were prospectively collected on 153 infants born in Campinas (state of São Paulo, Brazil from September 2000 to April 2002 weighing less than 1,500 g and treated at the University Hospital. The ratio of incidence rates with 95% CI, Breslow-Cox regression, Student's t test, linear regression and the Fisher's exact test were utilized. RESULTS: Among the 124 babies who survived until 28 days of age, 33 (26.6% developed bronchopulmonary dysplasia. Birthweight < 1,000 g (5.6; 95% CI 3.0, 10.4 and gestational age < 30 weeks (4.0; 95% CI 2.1, 7.2 were correlated with increased incidence of bronchopulmonary dysplasia. Breslow-Cox regression showed that other factors including gender, Apgar score, hyaline membrane disease, antenatal steroid therapy, pregnancy-induced hypertension, delivery route and maternal age were not associated with bronchopulmonary dysplasia. Mean duration of hospitalization and ventilator therapy in newborns with and without bronchopulmonary dysplasia was 78.8 days (SD = 26

  12. An official American Thoracic Society workshop report: optimal lung function tests for monitoring cystic fibrosis, bronchopulmonary dysplasia, and recurrent wheezing in children less than 6 years of age.

    Science.gov (United States)

    Rosenfeld, Margaret; Allen, Julian; Arets, Bert H G M; Aurora, Paul; Beydon, Nicole; Calogero, Claudia; Castile, Robert G; Davis, Stephanie D; Fuchs, Susanne; Gappa, Monika; Gustaffson, Per M; Hall, Graham L; Jones, Marcus H; Kirkby, Jane C; Kraemer, Richard; Lombardi, Enrico; Lum, Sooky; Mayer, Oscar H; Merkus, Peter; Nielsen, Kim G; Oliver, Cara; Oostveen, Ellie; Ranganathan, Sarath; Ren, Clement L; Robinson, Paul D; Seddon, Paul C; Sly, Peter D; Sockrider, Marianna M; Sonnappa, Samatha; Stocks, Janet; Subbarao, Padmaja; Tepper, Robert S; Vilozni, Daphna

    2013-04-01

    Although pulmonary function testing plays a key role in the diagnosis and management of chronic pulmonary conditions in children under 6 years of age, objective physiologic assessment is limited in the clinical care of infants and children less than 6 years old, due to the challenges of measuring lung function in this age range. Ongoing research in lung function testing in infants, toddlers, and preschoolers has resulted in techniques that show promise as safe, feasible, and potentially clinically useful tests. Official American Thoracic Society workshops were convened in 2009 and 2010 to review six lung function tests based on a comprehensive review of the literature (infant raised-volume rapid thoracic compression and plethysmography, preschool spirometry, specific airway resistance, forced oscillation, the interrupter technique, and multiple-breath washout). In these proceedings, the current state of the art for each of these tests is reviewed as it applies to the clinical management of infants and children under 6 years of age with cystic fibrosis, bronchopulmonary dysplasia, and recurrent wheeze, using a standardized format that allows easy comparison between the measures. Although insufficient evidence exists to recommend incorporation of these tests into the routine diagnostic evaluation and clinical monitoring of infants and young children with cystic fibrosis, bronchopulmonary dysplasia, or recurrent wheeze, they may be valuable tools with which to address specific concerns, such as ongoing symptoms or monitoring response to treatment, and as outcome measures in clinical research studies.

  13. Enteral vitamin A for reducing severity of bronchopulmonary dysplasia in extremely preterm infants: a randomised controlled trial.

    Science.gov (United States)

    Rakshasbhuvankar, Abhijeet; Patole, Sanjay; Simmer, Karen; Pillow, J Jane

    2017-12-16

    Intramuscular vitamin A supplementation decreases the risk of bronchopulmonary dysplasia (BPD) in very-low-birth-weight preterm infants without significant adverse effects. However, intramuscular vitamin A supplementation is not widely accepted because of the discomfort and risk of trauma associated with repeated injections. Enteral vitamin A supplementation has not been studied adequately in the clinical trials. Enterally administered water-soluble vitamin A is absorbed better than the fat-soluble form. We hypothesised that enteral administration of a water-soluble vitamin A preparation will decrease severity of BPD compared with a control group receiving placebo. We plan a double-blind randomised placebo-controlled trial at a tertiary neonatal-perinatal intensive care unit. Eligibility criteria include infants born at less than 28 weeks' gestational age and less than 72 h of life. Infants with major congenital gastrointestinal or respiratory tract abnormalities will be excluded. After parental consent, infants will be randomized to receive either enteral water-soluble vitamin A (5000 IU once a day) or placebo. The intervention will be started within 24 h of introduction of feeds and continued until 34 weeks' post-menstrual age (PMA). The primary outcome is severity of BPD at 36 weeks' PMA. Severity of BPD will be assessed objectively from the right-shift of the peripheral oxyhaemoglobin saturation versus partial pressure of inspired oxygen (SpO 2 -PiO 2 ) curve. We require 188 infants for 80% power and 5% significance level based on an expected 20% decrease in the right shift of the SpO 2 -PiO 2 curve in the vitamin A group (primary outcome) compared with control group at 36 weeks' PMA, and a 20% attrition rate. Secondary outcomes will be plasma and salivary concentrations of vitamin A on day 28 of the trial (first 30 infants), lung and diaphragm function, clinical outcomes at 36 week' PMA or before discharge/death, and safety of vitamin A. BPD poses a

  14. Positive end expiratory pressure for preterm infants requiring conventional mechanical ventilation for respiratory distress syndrome or bronchopulmonary dysplasia.

    Science.gov (United States)

    Bamat, Nicolas; Millar, David; Suh, Sanghee; Kirpalani, Haresh

    2012-01-18

    Conventional mechanical ventilation (CMV) of neonates has been used as a treatment of respiratory failure for over 30 years. While CMV facilitates gas exchange, it may simultaneously damage the lung. Positive end expiratory pressure (PEEP) has received less attention than other ventilation parameters when considering this balance of benefit and possible harm. While an appropriate level of PEEP may exert substantial benefits in ventilation, both inappropriately low or high levels may lead to harm. An appropriate level of PEEP for neonates may also be best achieved by an individualized approach. 1. To compare the effects of different levels of PEEP in preterm newborn infants requiring CMV for respiratory distress syndrome (RDS).2. To compare the effects of different levels of PEEP in preterm infants requiring CMV for bronchopulmonary dysplasia (BPD).3. To compare the effects of different methods for individualizing PEEP to an optimal level in preterm newborn infants requiring CMV for RDS. The search was performed in accordance with the standard search strategy for the Cochrane Neonatal Review Group. The Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library), Ovid MEDLINE, EMBASE, study references and experts were utilized for study identification. All randomized and quasi-randomized controlled trials studying preterm infants (less than 37 weeks gestational age) requiring CMV with endotracheal intubation and undergoing randomization to either different PEEP levels (RDS or BPD) or two or more alternative methods for individualizing PEEP levels (RDS only) were included. Cross-over trials were included but we limited the findings to those in the first cross-over period. Data collection and analysis were performed in accordance with the recommendations of the Cochrane Neonatal Review Group. An initial evaluation identified 10 eligible articles. Ultimately, a single study met our inclusion criteria. The study addressed the effects of different

  15. Systematic Review of Inhaled Bronchodilator and Corticosteroid Therapies in Infants with Bronchopulmonary Dysplasia: Implications and Future Directions.

    Directory of Open Access Journals (Sweden)

    Brian J Clouse

    Full Text Available There is much debate surrounding the use of inhaled bronchodilators and corticosteroids for infants with bronchopulmonary dysplasia (BPD.The objective of this systematic review was to identify strengths and knowledge gaps in the literature regarding inhaled therapies in BPD and guide future research to improve long-termoutcomes.The databases of Academic Search Complete, CINAHL, PUBMED/MEDLINE, and Scopus were searched for studies that evaluated both acute and long-term clinical outcomes related to the delivery and therapeutic efficacy of inhaled beta-agonists, anticholinergics and corticosteroids in infants with developing and/or established BPD.Of 181 articles, 22 met inclusion criteria for review. Five evaluated beta-agonist therapies (n = 84, weighted gestational age (GA of 27.1(26-30 weeks, weighted birth weight (BW of 974(843-1310 grams, weighted post menstrual age (PMA of 34.8(28-39 weeks, and weighted age of 53(15-86 days old at the time of evaluation. Fourteen evaluated inhaled corticosteroids (n = 2383, GA 26.2(26-29 weeks, weighted BW of 853(760-1114 grams, weighted PMA of 27.0(26-31 weeks, and weighted age of 6(0-45 days old at time of evaluation. Three evaluated combination therapies (n = 198, weighted GA of 27.8(27-29 weeks, weighted BW of 1057(898-1247 grams, weighted PMA of 30.7(29-45 weeks, and age 20(10-111 days old at time of evaluation.Whether inhaled bronchodilators and inhaled corticosteroids improve long-term outcomes in BPD remains unclear. Literature regarding these therapies mostly addresses evolving BPD. There appears to be heterogeneity in treatment responses, and may be related to varying modes of administration. Further research is needed to evaluate inhaled therapies in infants with severe BPD. Such investigations should focus on appropriate definitions of disease and subject selection, timing of therapies, and new drugs, devices and delivery methods as compared to traditional methods across all modalities of

  16. Systematic Review of Inhaled Bronchodilator and Corticosteroid Therapies in Infants with Bronchopulmonary Dysplasia: Implications and Future Directions

    Science.gov (United States)

    Clouse, Brian J.; Jadcherla, Sudarshan R.; Slaughter, Jonathan L.

    2016-01-01

    Background There is much debate surrounding the use of inhaled bronchodilators and corticosteroids for infants with bronchopulmonary dysplasia (BPD). Objective The objective of this systematic review was to identify strengths and knowledge gaps in the literature regarding inhaled therapies in BPD and guide future research to improve long-termoutcomes. Methods The databases of Academic Search Complete, CINAHL, PUBMED/MEDLINE, and Scopus were searched for studies that evaluated both acute and long-term clinical outcomes related to the delivery and therapeutic efficacy of inhaled beta-agonists, anticholinergics and corticosteroids in infants with developing and/or established BPD. Results Of 181 articles, 22 met inclusion criteria for review. Five evaluated beta-agonist therapies (n = 84, weighted gestational age (GA) of 27.1(26–30) weeks, weighted birth weight (BW) of 974(843–1310) grams, weighted post menstrual age (PMA) of 34.8(28–39) weeks, and weighted age of 53(15–86) days old at the time of evaluation). Fourteen evaluated inhaled corticosteroids (n = 2383, GA 26.2(26–29) weeks, weighted BW of 853(760–1114) grams, weighted PMA of 27.0(26–31) weeks, and weighted age of 6(0–45) days old at time of evaluation). Three evaluated combination therapies (n = 198, weighted GA of 27.8(27–29) weeks, weighted BW of 1057(898–1247) grams, weighted PMA of 30.7(29–45) weeks, and age 20(10–111) days old at time of evaluation). Conclusion Whether inhaled bronchodilators and inhaled corticosteroids improve long-term outcomes in BPD remains unclear. Literature regarding these therapies mostly addresses evolving BPD. There appears to be heterogeneity in treatment responses, and may be related to varying modes of administration. Further research is needed to evaluate inhaled therapies in infants with severe BPD. Such investigations should focus on appropriate definitions of disease and subject selection, timing of therapies, and new drugs, devices and delivery

  17. Compare severity of bronchopulmonary dysplasia in neonates with respiratory distress syndrome treated with surfactant to without surfactant.

    Science.gov (United States)

    Chotigeat, Uraiwan; Ratchatanorravut, Sombat; Kanjanapattanakul, Wiboon

    2011-08-01

    The utilization of surfactant replacement therapy had been limited in treatment of respiratory distress syndrome (RDS) due to the high cost especially in developing countries. Nowadays, the National Health Insurance Policy has covered the cost of surfactant for the patients. Therefore, bronchopulmonary dysplasia (BPD) may be found increasing due to increased survival in patients with severe RDS. To compare immediate treatment outcome of severity of BPD and outcome after hospital discharge in neonates with RDS who were treated with or without surfactant. Retrospective cohort study. The data of 54 infants who developed BPD after RDS at Queen Sirikit National Institute of Child Health between January 1st, 2003 and December 31th, 2005 were kept in database format. The database was analyzed for difference between groups and the outcome of immediate treatment, severity of BPD and outcome after hospital discharge were compared. The study group was BPD cases from RDS treated with surfactant compared to control (BPD cases from RDS treated without surfactant) groups. Forty-three (80%) from fifty-four cases had completed data and were included into the present study. There was no statistically significant difference in maternal conditions and neonatal conditions between groups. Antenatal steroid was prescribed more often in RDS without surfactant group than surfactant group. The mean birth weight and gestational age in surfactant and without surfactant groups were 1,179.1 +/- 274.3 gm vs. 1,114.4 +/- 338.3 gm and 29 +/- 1.6 weeks vs. 29.2 +/- 2.7 weeks respectively, but no significant differences were observed between groups. To compare the severity of RDS, only 17.6% of moderate to severe RDS in the control group was found, whereas 100% was found in the study group. Moderate to severe BPD cases were found more often in the control group (70.6%) than in the study group (61.6%), but no statistically significant difference was shown. The immediate complications, e

  18. Azithromycin in the extremely low birth weight infant for the prevention of Bronchopulmonary Dysplasia: a pilot study

    Directory of Open Access Journals (Sweden)

    Anstead Michael I

    2007-06-01

    Full Text Available Abstract Background Azithromycin reduces the severity of illness in patients with inflammatory lung disease such as cystic fibrosis and diffuse panbronchiolitis. Bronchopulmonary dysplasia (BPD is a pulmonary disorder which causes significant morbidity and mortality in premature infants. BPD is pathologically characterized by inflammation, fibrosis and impaired alveolar development. The purpose of this study was to obtain pilot data on the effectiveness and safety of prophylactic azithromycin in reducing the incidence and severity of BPD in an extremely low birth weight (≤ 1000 grams population. Methods Infants ≤ 1000 g birth weight admitted to the University of Kentucky Neonatal Intensive Care Unit (level III, regional referral center from 9/1/02-6/30/03 were eligible for this pilot study. The pilot study was double-blinded, randomized, and placebo-controlled. Infants were randomized to treatment or placebo within 12 hours of beginning mechanical ventilation (IMV and within 72 hours of birth. The treatment group received azithromycin 10 mg/kg/day for 7 days followed by 5 mg/kg/day for the duration of the study. Azithromycin or placebo was continued until the infant no longer required IMV or supplemental oxygen, to a maximum of 6 weeks. Primary endpoints were incidence of BPD as defined by oxygen requirement at 36 weeks gestation, post-natal steroid use, days of IMV, and mortality. Data was analyzed by intention to treat using Chi-square and ANOVA. Results A total of 43 extremely premature infants were enrolled in this pilot study. Mean gestational age and birth weight were similar between groups. Mortality, incidence of BPD, days of IMV, and other morbidities were not significantly different between groups. Post-natal steroid use was significantly less in the treatment group [31% (6/19] vs. placebo group [62% (10/16] (p = 0.05. Duration of mechanical ventilation was significantly less in treatment survivors, with a median of 13 days (1–47

  19. What Is Bronchopulmonary Dysplasia?

    Science.gov (United States)

    ... Later, babies may be given breast milk or infant formula through feeding tubes that are passed through their noses or mouths ... child's long-term care and make treatment recommendations. Infants who need ... It allows a breathing tube to be placed directly into the windpipe, rather ...

  20. Living with Bronchopulmonary Dysplasia

    Science.gov (United States)

    ... Later, babies may be given breast milk or infant formula through feeding tubes that are passed through their noses or mouths ... child's long-term care and make treatment recommendations. Infants who need ... It allows a breathing tube to be placed directly into the windpipe, rather ...

  1. N-Terminal Pro-B Type Natriuretic Peptide as a Marker of Bronchopulmonary Dysplasia or Death in Very Preterm Neonates

    DEFF Research Database (Denmark)

    Sellmer, Anna; Hjortdal, Vibeke Elisabeth; Bjerre, Jesper Vandborg

    2015-01-01

    increase of natural log NT-proBNP day three when adjusted for gestational age at birth (OR = 1.7, 95% CI 1.3; 2.3). The association was found both in neonates with and without a PDA. Adjusting for GA, PDA diameter, LA:Ao-ratio, or early onset sepsis did not change the estimate. CONCLUSION: We found NT...... three and bronchopulmonary dysplasia (BPD) or death and further to assess the impact of patent ductus arteriosus (PDA) on this association in neonates born before 32 gestational weeks. METHODS: A cohort study of 183 neonates born before 32 gestational weeks consecutively admitted to the Neonatal......-proBNP to be associated with BPD or death in very preterm neonates. This association was not only explained by the PDA. We speculate that NT-proBNP may help the identification of neonates at risk of BPD as early as postnatal day three....

  2. Tempo de ventilação mecânica e desenvolvimento de displasia broncopulmonar Duration of mechanical ventilation and development of bronchopulmonary dysplasia

    Directory of Open Access Journals (Sweden)

    Ana Damaris Gonzaga

    2007-02-01

    Full Text Available OBJETIVO: Verificar a associação entre o tempo de uso da ventilação mecânica e o desenvolvimento de displasia broncopulmonar em recém-nascidos com peso de nascimento 15 dias. Foi calculada a razão de chance para o desenvolvimento de displasia broncopulmonar em cada período de utilização da ventilação mecânica. RESULTADOS: Dos 216 prontuários avaliados, 121 preencheram os critérios de inclusão. As médias do peso de nascimento e idade gestacional foram de 1199,8 g e 31,8 semanas. No período de 1 a 7 dias de uso da ventilação mecânica, 15,5% dos recém-nascidos evoluíram com displasia broncopulmonar; no período de 8 a 14 dias, 60%; e no período > 15 dias, 88,2%; com razão de chance de 0,16, 11,25 e 16,36, respectivamente. CONCLUSÃO: A possibilidade de um recém-nascido com peso de nascimento OBJECTIVE: Verify the association between duration of mechanical ventilation and development of bronchopulmonary dysplasia in neonates weighting at birth less than 1500g. METHODS: Retrospective study conducted with neonates weighting less than 1500g at birth submitted to mechanical ventilation. Neonates presenting major birth defects, transferred to other services or died before the 28th day of life were excluded from the study. Three groups were analyzed according to duration of mechanical ventilation: 1 to 7 days, 8 to 14 days and more than 15 days. The chance ratio of developing bronchopulmonary dysplasia was calculated for each group. RESULTS: From the 216 clinical histories assessed, 121 met the criteria for inclusion in the study. Mean birth weight and gestational age were 1199.8 g and 31.8 weeks. Of all neonates submitted to mechanical ventilation from 1 to 7 days, 15.5% developed bronchopulmonary dysplasia; from 8 to 14 days 60% and from more than 15 days, 88.2%; chance ratios were equal to 0.16; 11.25 and 16.36, respectively. CONCLUSION: The chance of a neonate weighting less than 1500 g developing bronchopulmonary dysplasia

  3. N-Terminal Pro-B Type Natriuretic Peptide as a Marker of Bronchopulmonary Dysplasia or Death in Very Preterm Neonates: A Cohort Study.

    Science.gov (United States)

    Sellmer, Anna; Hjortdal, Vibeke Elisabeth; Bjerre, Jesper Vandborg; Schmidt, Michael Rahbek; McNamara, Patrick J; Bech, Bodil Hammer; Henriksen, Tine Brink

    2015-01-01

    Bronchopulmonary dysplasia (BPD) is a serious complication of preterm birth. Plasma N-terminal pro-B type natriuretic peptide (NT-proBNP) has been suggested as a marker that may predict BPD within a few days after birth. To investigate the association between NT-proBNP day three and bronchopulmonary dysplasia (BPD) or death and further to assess the impact of patent ductus arteriosus (PDA) on this association in neonates born before 32 gestational weeks. A cohort study of 183 neonates born before 32 gestational weeks consecutively admitted to the Neonatal Intensive Care Unit, Aarhus University Hospital, Denmark. On day three plasma samples were collected and echocardiography carried out. NT-proBNP was measured by routine immunoassays. The combined outcome BPD or death was assessed at 36 weeks of postmenstrual age. Receiver operator characteristic (ROC) analysis was performed to determine the discrimination ability of NT-proBNP by the natural log continuous measure to recognize BPD or death. The association of BPD or death was assessed in relation to natural log NT-proBNP levels day three. The risk of BPD or death increased 1.7-fold with one unit increase of natural log NT-proBNP day three when adjusted for gestational age at birth (OR = 1.7, 95% CI 1.3; 2.3). The association was found both in neonates with and without a PDA. Adjusting for GA, PDA diameter, LA:Ao-ratio, or early onset sepsis did not change the estimate. We found NT-proBNP to be associated with BPD or death in very preterm neonates. This association was not only explained by the PDA. We speculate that NT-proBNP may help the identification of neonates at risk of BPD as early as postnatal day three.

  4. Ruptura prematura das membranas amnióticas no pré-termo: fatores associados à displasia broncopulmonar Preterm premature rupture of the fetal membranes: factors associated with bronchopulmonary dysplasia

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    Danieli Dias Gonçalves

    2010-10-01

    Full Text Available OBJETIVO: identificar os fatores obstétricos e neonatais associados ao desfecho de displasia broncopulmonar em pacientes com amniorrexe prematura no pré-termo. MÉTODOS: foram analisados 213 prontuários do Instituto Fernandes Figueira, entre 1998 e 2002, cujas pacientes evoluíram com quadro de amniorrexe prematura 10 dias (OR: 54,00 [11,55-278,25] p=0,000; idade gestacional 10 dias (p=0,001 e "uso de surfactante" (p=0,040 permaneceram independentemente associadas ao desfecho. CONCLUSÕES: observou-se que os fatores associados à displasia broncopulmonar são de natureza neonatal, sendo que a ventilação mecânica duradoura e o uso de surfactante neonatal influenciaram no desenvolvimento dessa doença.PURPOSE: to analyze obstetric and neonatal factors associated with bronchopulmonary dysplasia outcome in patients with preterm premature amniorrhexis. METHODS: we analyzed 213 medical records of patients of Fernandes Figueira Institute who suffered premature amniorrhexis (10 days (p=0.001 and "use of a surfactant" (p=0.040 remained independently associated with bronchopulmonary dysplasia. CONCLUSIONS: the factors associated with bronchopulmonary dysplasia are related to neonatal features, asprolonged mechanical ventilation and the use of a surfactant influencethe development of thedisease.

  5. Early postnatal additional high-dose oral vitamin A supplementation versus placebo for 28 days for preventing bronchopulmonary dysplasia or death in extremely low birth weight infants.

    Science.gov (United States)

    Meyer, Sascha; Gortner, Ludwig

    2014-01-01

    Prematurity and the associated risk for bronchopulmonary dysplasia (BPD) remain a significant threat to extremely low birth weight (ELBW) infants. Vitamin A has been considered a therapeutic alternative in reducing the rate of BPD and mortality. To investigate whether early postnatal, additional high-dose oral vitamin A supplementation for 28 days is more efficient in reducing BPD or death in ELBW infants than placebo treatment. This is a multicenter, double-blind RCT comparing postnatal high-dose oral vitamin A supplementation (5,000 IU vitamin A/kg/day vs. placebo) for 28 days in ELBW neonates requiring mechanical ventilation, noninvasive ventilatory support or supplemental oxygen at 24 h of age. The primary end point is the proportion of children who died before 36 weeks' gestational age or developed moderate or severe BPD. BPD is defined as the need for supplemental oxygen to maintain SaO2 of ≥92% at rest at 36 weeks' postmenstrual age (PMA). Clinical secondary end points include the following: BPD (including mild form), intraventricular hemorrhage, periventricular leukomalacia, retinopathy of prematurity, necrotizing enterocolitis, total number of days of mechanical ventilation and oxygen supplementation, and safety and tolerability of high-dose vitamin A supplementation. The results of the NeoVitaA trial will provide robust data with regard to the efficacy of high-dose oral vitamin A supplementation in reducing the incidence of BPD or death at 36 weeks' PMA in ELBW infants. © 2014 S. Karger AG, Basel.

  6. Role of vitamin A supplementation in prevention of bronchopulmonary dysplasia in extremely low birth weight neonates: a systematic review of randomized trials.

    Science.gov (United States)

    Garg, Bhawan Deep; Bansal, Anju; Kabra, Nandkishor S

    2018-02-22

    Bronchopulmonary dysplasia (BPD) is one of the most common consequence of extreme prematurity (birth weight (ELBW) neonates. The aim of this systematic review is to evaluate the role of vitamin A supplementation in prevention of BPD in ELBW neonates. The literature search was done for various randomized control trial (RCT) by searching the Cochrane Central Register of Controlled Trials (CENTRAL), PubMed, EMBASE, and ongoing clinical trials. This review included two RCTs that fulfilled inclusion criteria. There were statistically significant reduction in the incidence of BPD (oxygen requirement at 36 weeks of postmenstrual age (PMA)) (relative risk (RR) 0.88; 95%CI 0.77-0.99; p = .04; NNTB 14) and borderline significant reduction in combined outcomes of mortality/BPD (oxygen requirement at 36 weeks of PMA) (RR 0.90; 95%CI 0.82-1.00; p = .05). However, oxygen requirement at 28 days of life and combined outcome of mortality/BPD (oxygen requirement at 28 days of life) were not statistically significant. The role of vitamin A supplementation in the prevention of BPD is supported by the current evidences. However, due to limited number of studies, current evidences are not sufficient which can translate into routine clinical practice. We need large high-quality trials, with sufficient power to reliably assess clinically relevant differences in outcomes.

  7. Development of left ventricular longitudinal speckle tracking echocardiography in very low birth weight infants with and without bronchopulmonary dysplasia during the neonatal period.

    Directory of Open Access Journals (Sweden)

    Christoph Czernik

    Full Text Available OBJECTIVES: In preterm infants, postnatal myocardial adaptation may be complicated by bronchopulmonary dysplasia (BPD. We aimed to describe the development of left ventricular function by serial 2D, Doppler, and speckle tracking echocardiography (2D-STE in infants with and without BPD during the neonatal period and compare these to anthropometric and conventional hemodynamic parameters. STUDY DESIGN: Prospective echocardiography on day of life (DOL 1, 7, 14, and 28 in 119 preterm infants 10% were seen for the apical segment. While anthropometric parameters show rapid development during the first 4 weeks of life, the speckle tracking parameters did not differ statistically significantly during the neonatal period. Infants with and without BPD differed significantly (p<0.001 in the development of anthropometric parameters, conventional hemodynamic parameters except for heart rate, and 2D-STE parameters: global longitudinal systolic strain rate (GLSSR and longitudinal systolic strain for the mid left wall (LSSR. The largest differences were seen at DOL 1 and 7 in GLSSR (p<0.001 and in LSSR (p<0.01. CONCLUSIONS: Reproducible 2D-STE measurements are possible in preterm infants <1500 g. Cardiac deformation reveals early (DOL 1 and 7 ventricular changes (GLSSR and LSSR in very low birth weight infants who develop BPD.

  8. Exome sequencing and pathway analysis for identification of genetic variability relevant for bronchopulmonary dysplasia (BPD) in preterm newborns: A pilot study.

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    Carrera, Paola; Di Resta, Chiara; Volonteri, Chiara; Castiglioni, Emanuela; Bonfiglio, Silvia; Lazarevic, Dejan; Cittaro, Davide; Stupka, Elia; Ferrari, Maurizio; Somaschini, Marco

    2015-12-07

    Bronchopulmonary dysplasia (BPD) is the most common chronic lung disease in infancy, affecting preterm children with low birth weight. The disease has a multifactorial aetiology with a significant genetic component; until now published association studies have identified several candidate genes but only few of these data has been replicated. In this pilot study, we approached exome sequencing aimed at identifying non-common variants, which are expected to have a stronger phenotypic effect. We performed this study on 26 Italian severely affected BPD preterm unrelated newborns, homogeneously selected from a large prospective cohort. We used an Illumina HiSeq 2000 for sequencing. Data analysis was focussed on genes previously associated to BPD susceptibility and to new candidates in related pathways, highlighted by a prioritization analysis performed using ToppGene Suite. By exome sequencing, we identified 3369 novel variants, with a median of 400 variations per sample. The top candidate genes highlighted were NOS2, MMP1, CRP, LBP and the toll-like receptor (TLR) family. All of them have been confirmed with Sanger sequencing. Potential candidate genes have been discovered in this preliminary study; the pathogenic role of identified variants will need to be confirmed with functional and segregation studies and possibly with further methods, able to evaluate the collective influence of rare variants. Moreover, additional candidates will be tested and genetic analysis will be extended to all affected children. Copyright © 2015 Elsevier B.V. All rights reserved.

  9. Deferoxamine Improves Alveolar and Pulmonary Vascular Development by Upregulating Hypoxia-inducible Factor-1α in a Rat Model of Bronchopulmonary Dysplasia.

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    Choi, Chang Won; Lee, Juyoung; Lee, Hyun Ju; Park, Hyoung-Sook; Chun, Yang-Sook; Kim, Beyong Il

    2015-09-01

    Fetal lung development normally occurs in a hypoxic environment. Hypoxia-inducible factor (HIF)-1α is robustly induced under hypoxia and transactivates many genes that are essential for fetal development. Most preterm infants are prematurely exposed to hyperoxia, which can halt hypoxia-driven lung maturation. We were to investigate whether the HIF-1α inducer, deferoxamine (DFX) can improve alveolarization in a rat model of bronchopulmonary dysplasia (BPD). A rat model of BPD was produced by intra-amniotic lipopolysaccharide (LPS) administration and postnatal hyperoxia (85% for 7 days), and DFX (150 mg/kg/d) or vehicle was administered to rat pups intraperitoneally for 14 days. On day 14, the rat pups were sacrificed and their lungs were removed and examined. A parallel in vitro study was performed with a human small airway epithelial cell line to test whether DFX induces the expression of HIF-1α and its target genes. Alveolarization and pulmonary vascular development were impaired in rats with BPD. However, DFX significantly ameliorated these effects. Immunohistochemical analysis showed that HIF-1α was significantly upregulated in the lungs of BPD rats treated with DFX. DFX was also found to induce HIF-1α in human small airway epithelial cells and to promote the expression of HIF-1α target genes. Our data suggest that DFX induces and activates HIF-1α, thereby improving alveolarization and vascular distribution in the lungs of rats with BPD.

  10. Pulmonary outcome in former preterm, very low birth weight children with bronchopulmonary dysplasia: a case-control follow-up at school age.

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    Vom Hove, Maike; Prenzel, Freerk; Uhlig, Holm H; Robel-Tillig, Eva

    2014-01-01

    To assess and compare long-term pulmonary outcomes in former preterm-born, very low birth weight (VLBW) children with and without bronchopulmonary dysplasia (BPD) born in the surfactant era. Pulmonary function tests (ie, spirometry, body plethysmography, and gas transfer testing) were performed in children with a history of VLBW and BPD (n = 28) and compared with a matched preterm-born VLBW control group (n = 28). Medical history was evaluated by questionnaire. At time of follow-up (mean age, 9.5 years), respiratory symptoms (36% vs 8%) and receipt of asthma medication (21% vs 0%) were significantly more frequent in the preterm-born children with previous BPD than in those with no history of BPD. The children with a history of BPD had significantly lower values for forced expiratory volume in 1 second (z-score -1.27 vs -0.4; P = .008), forced vital capacity (z-score -1.39 vs -0.71 z-score; P = .022), and forced expiratory flow rate at 50% of forced vital capacity (z-score -2.21 vs -1.04; P = .048) compared with the preterm control group. Preterm-born children with a history of BPD are significantly more likely to have lung function abnormalities, such as airway obstruction and respiratory symptoms, at school age compared with preterm-born children without BPD. Copyright © 2014 Mosby, Inc. All rights reserved.

  11. Lack of EC-SOD worsens alveolar and vascular development in a neonatal mouse model of bleomycin-induced bronchopulmonary dysplasia and pulmonary hypertension.

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    Delaney, Cassidy; Wright, Rachel H; Tang, Jen-Ruey; Woods, Crystal; Villegas, Leah; Sherlock, Laurie; Savani, Rashmin C; Abman, Steven H; Nozik-Grayck, Eva

    2015-12-01

    Pulmonary hypertension (PH) worsens clinical outcomes in former preterm infants with bronchopulmonary dysplasia (BPD). Oxidant stress disrupts alveolar and vascular development in models of BPD. Bleomycin causes oxidative stress and induces BPD and PAH in neonatal rats. Disruption in the vascular endothelial growth factor (VEGF) and nitric oxide signaling pathways contributes to BPD. We hypothesized that loss of EC-SOD would worsen PAH associated with BPD in a neonatal mouse model of bleomycin-induced BPD by disrupting the VEGF/NO signaling pathway. Neonatal wild-type mice (WT), and mice lacking EC-SOD (EC-SOD KO) received intraperitoneal bleomycin (2 units/kg) or phosphate-buffered saline (PBS) three times weekly and were evaluated at weeks 3 or 4. Lack of EC-SOD impaired alveolar development and resulted in PH (elevated right ventricular systolic pressures, right ventricular hypertrophy (RVH)), decreased vessel density, and increased small vessel muscularization. Exposure to bleomycin further impaired alveolar development, worsened RVH and vascular remodeling. Lack of EC-SOD and bleomycin treatment decreased lung total and phosphorylated VEGFR2 and eNOS protein expression. EC-SOD is critical in preserving normal lung development and loss of EC-SOD results in disrupted alveolar development, PAH and vascular remodeling at baseline, which is further worsened with bleomycin and associated with decreased activation of VEGFR2.

  12. Preterm Cord Blood CD4+ T Cells Exhibit Increased IL-6 Production in Chorioamnionitis and Decreased CD4+ T Cells in Bronchopulmonary Dysplasia

    Science.gov (United States)

    Fowell, Deborah; Wang, Hongyue; Scheible, Kristin; Misra, Sara; Huyck, Heidie; Wyman, Claire; Ryan, Rita M.; Reynolds, Anne Marie; Mariani, Tom; Katzman, Philip J.; Pryhuber, Gloria S.

    2015-01-01

    Background Chorioamnionitis (CA) is associated with premature delivery and bronchopulmonary dysplasia (BPD). We hypothesize that preterm infants exposed to CA have reduced suppressive regulatory T cells (Treg) and increased non-regulatory T cell pro-inflammatory cytokines, increasing risk for BPD. Objective To evaluate cord blood CD4+ T cell regulatory phenotype and pro-inflammatory cytokine production in CA and BPD groups. Study Design Cord blood mononuclear cells from infants (GA ≤32 weeks), with or without placental histological evidence of CA (hChorio), were analyzed by flow cytometry. Clinical information was collected by retrospective chart review. Numbers of putative Treg (CD4+FoxP3+CD25+CD127Dim), CD4+ non-Tregs, and CD4+ T cell intracellular cytokine content following in vitro stimulation were compared with CA status and oxygen requirement at 36 weeks postmenstrual age. Result Absolute Treg numbers were not different in CA and non-CA exposed samples. However, the infants who developed BPD had a significant decrease in Treg and non-regulatory T cell numbers. Greater IL-6 production was observed in hCA group. Conclusion A pro-inflammatory CD4+ T cell status is noted in CA and BPD but the later disease is also associated with decrease in Tregs, suggesting that the development of BPD is marked by distinct inflammatory changes from those of CA exposed infants. PMID:25797206

  13. Expression of transforming growth factor-β1 in neonatal rats with hyperoxia-induced bronchopulmonary dysplasia and its relationship with lung development.

    Science.gov (United States)

    Yan, B; Zhong, W; He, Q M; Zhang, S Y; Yu, J K; Pan, Y L

    2016-05-06

    The aim of this study was to detect the expression of transforming growth factor-ß1 (TGF-ß1) in neonatal rats with hyperoxia-induced bronchopulmonary dysplasia (BPD) and to explore its relationship with lung development. Forty-eight rats (2-3 days old) were randomly divided into a hyperoxia group and a control group (N = 24) which were then fed in ≥95% oxygen atmosphere and air, respectively. On the 1st, 3rd and 7th days of hyperoxia exposure, morphological changes of lung tissues were observed under an optical microscope. TGF-ß1 mRNA and protein levels in lung tissues were detected by real-time quantitative polymerase chain reaction and western blot, respectively. With increasing time of hyperoxia exposure, the hyperoxia group gradually suffered from pathological changes such as poor development of lung tissues, alveolar simplification, decrease in the number of alveoli, and hindered pulmonary microvascular development. On the 7th day of hyperoxia exposure, TGF-ß1 mRNA and protein levels (relative to b-actin) of the hyperoxia group (0.34 ± 0.19 and 0.21 ± 0.09, respectively) were significantly lower than those of the control group (0.83 ± 0.45 and 0.57 ± 0.45, respectively; P factor during pulmonary vascular development.

  14. Inhaled nitric oxide improves lung structure and pulmonary hypertension in a model of bleomycin-induced bronchopulmonary dysplasia in neonatal rats.

    Science.gov (United States)

    Tourneux, Pierre; Markham, Neil; Seedorf, Gregory; Balasubramaniam, Vivek; Abman, Steven H

    2009-12-01

    Whether inhaled nitric oxide (iNO) prevents the development of bronchopulmonary dysplasia (BPD) in premature infants is controversial. In adult rats, bleomycin (Bleo) induces lung fibrosis and pulmonary hypertension, but the effects of Bleo on the developing lung and iNO treatment on Bleo-induced neonatal lung injury are uncertain. Therefore, we sought to determine whether early and prolonged iNO therapy attenuates changes of pulmonary vascular and alveolar structure in a model of BPD induced by Bleo treatment of neonatal rats. Sprague-Dawley rat pups were treated with Bleo (1 mg/kg ip daily) or vehicle (controls) from day 2 to 10, followed by recovery from day 11 to 19. Treatment groups received early (days 2-10), late (days 11-19), or prolonged iNO therapy (10 ppm; days 2-19). We found that compared with controls, Bleo increased right ventricular hypertrophy (RVH), and pulmonary arterial wall thickness, and reduced vessel density alveolarization. In each iNO treatment group, iNO decreased RVH (P rats, and that early and prolonged iNO therapy prevents right ventricle hypertrophy and pulmonary vascular remodeling and partially improves lung structure.

  15. Complacência pulmonar com uma hora de vida e displasia broncopulmonar em recém-nascidos prematuros Early dynamic pulmonary compliance and bronchopulmonary dysplasia in preterm newborn infants

    Directory of Open Access Journals (Sweden)

    Lídia Mayrink de Barros

    2007-12-01

    Full Text Available OBJETIVOS: avaliar se a complacência pulmonar precoce é fator preditor da presença de displasia broncopulmonar aos 28 dias de vida, em prematuros. MÉTODO: coorte prospectiva de neonatos com idade gestacional OBJECTIVES: to evaluate whether early pulmonary compliance could be a predictor of the presence of bronchopulmonary dysplasia at 28 days of life in preterm infants. METHODS: a cohort study was carried out involving neonates with gestational age <32 weeks and a birth weight of 500-1250 g receiving prophylactic surfactant at 30 minutes of life. The lung mechanics was evaluated using a pneumotachograph connected to the ventilator circuit 60 minutes after birth. Flow signals and volume were measured using WinTracer® in order to determine the dynamic pulmonary compliance and the airway resistance. Variables associated with the need for oxygen or assisted ventilation at 28 days were assessed using logistic regression. RESULTS: 32 neonates were enrolled in the study and 25 survived until the 28th day, at which point 17 (68% needed assisted ventilation and/or oxygen (Group 1, and 8 did not (Group 2. The Group 1 infants were younger, had higher clinical risk index scores and frequency of patent ductus arteriosus. The lung mechanics in the 1st hour of life was similar in Groups 1 and 2. Regression analysis showed that bronchopulmonary dysplasia was associated with the presence of patent ductus arteriosus and lower gestational age. CONCLUSIONS: pulmonary compliance figures in the 1st hour of life did not predict the presence of bronchopulmonary dysplasia in the 28th day of life of the studied population.

  16. Bubble nasal CPAP, early surfactant treatment, and rapid extubation are associated with decreased incidence of bronchopulmonary dysplasia in very-low-birth-weight newborns: efficacy and safety considerations.

    Science.gov (United States)

    Friedman, Charles A; Menchaca, Robert C; Baker, Mary C; Rivas, Clarissa K; Laberge, Raymond N; Rios, Enrique H; Haider, Syed H; Romero, Edgar J; Eason, Elizabeth B; Fraley, J Kennard; Woldesenbet, Mesfin

    2013-07-01

    Current literature has been inconsistent in demonstrating that minimizing the duration of mechanical ventilation in very-low-birth-weight (VLBW) newborns reduces lung damage. To determine if introduction of bubble nasal CPAP (bnCPAP), early surfactant treatment, and rapid extubation (combined bnCPAP strategy) in our community-based neonatal ICU reduced bronchopulmonary dysplasia (BPD). This was a 7-year retrospective,single-institution review of respiratory outcomes in 633 VLBW babies before and after introduction of the combined bnCPAP strategy. Coincident changes in newborn care were taken into account with a logistic regression model. The average percentage of VLBW newborns with BPD decreased to 25.8% from 35.4% (P = .02), reaching a minimum in the last post-bnCPAP year of22.1% (P = .02). When other coincident changes in newborn care during the study years were taken into account, VLBW babies in the post-bnCPAP years had a 43% lower chance of developing BPD(P = .003, odds ratio 0.43, 95% CI 0.25– 0.75). Decreases occurred in mechanical ventilation and the percentage of infants discharged on diuretics and on supplemental oxygen. Among the subset of extremely-low-birth-weight newborns, improved respiratory outcomes in the post-bnCPAP years,as compared to outcomes in the pre-bnCPAP years, included an increase in the percentage alive and off mechanical ventilation at 1 week postnatal age (P < .001), a more rapid extubation rate(P < .03), a decrease in the median days on mechanical ventilation (P = .002), and a decrease in the percentage with BPD plus died (P = .01). Post-bnCPAP extremely-low-birth-weight babies had a statistically significant decrease in retinopathy of prematurity, an increase in low-grade intraventricular hemorrhage, and a decrease in ductal ligations. A combined BnCPAP strategy may contribute to a reduction of BPD, after adjusting for concurrent treatments.

  17. Risk factors for bronchopulmonary dysplasia in five Portuguese neonatal intensive care units Factores de risco de displasia broncopulmonar em cinco unidades portuguesas de cuidados intensivos neonatais

    Directory of Open Access Journals (Sweden)

    Hercília Guimarães

    2010-06-01

    Full Text Available The pathogenesis of bronchopulmonary dysplasia (BPD is clearly multifactorial. Specific pathogenic risk factors are prematurity, respiratory distress, oxygen supplementation, mechanical ventilation (MV, inflammation, patent ductus arteriosus (PDA, etc. Aim: To evaluate BPD prevalence and to identify risk factors for BPD in five Portuguese Neonatal Intensive Care Units in order to develop better practices the management of these newborns. Material and methods: 256 very low birth weight infants with gestational age (GA 0.30 (85 vs 5 days, respectively in BPD and no BPD patients, pA displasia broncopulmonar (DBP é multifactorial. Prematuridade, doença da membrana hialina, oxigénio, ventilação mecânica, inflamação e canal arterial são alguns dos factores na sua patogénese. Objectivo: Avaliar a prevalência da DBP e seus factores de risco em cinco unidades portuguesas, para implementar boas práticas no tratamento deste doentes. Material e métodos: 256 recém-nascidos (RN com idade gestacional (IG 0,30 (85 vs 5 dias, respectivamente nos doentes com e sem DBP, p<0,001. Comentários: Os factores de risco de DBP mais relevantes foram o baixo peso, a doença da membrana hialina grave, a duração da ventilação mecânica e da oxigenoterapia e a sépsis. A implementação das boas práticas para reduzir a lesão pulmonar nos RN deve ser dirigida para melhorar as práticas que reduzem estes factores de risco.

  18. Delayed extubation to nasal continuous positive airway pressure in the immature baboon model of bronchopulmonary dysplasia: lung clinical and pathological findings.

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    Thomson, Merran A; Yoder, Bradley A; Winter, Vicki T; Giavedoni, Luis; Chang, Ling Yi; Coalson, Jacqueline J

    2006-11-01

    Using the 125-day baboon model of bronchopulmonary dysplasia treated with prenatal steroid and exogenous surfactant, we hypothesized that a delay of extubation from low tidal volume positive pressure ventilation to nasal continuous positive airway pressure at 5 days (delayed nasal continuous positive airway pressure group) would not induce more lung injury when compared with baboons aggressively weaned to nasal continuous positive airway pressure at 24 hours (early nasal continuous positive airway pressure group), because both received positive pressure ventilation. After delivery by cesarean section at 125 days (term: 185 days), infants received 2 doses of Curosurf (Chiesi Farmaceutica S.p.A., Parma, Italy) and daily caffeine citrate. The delay in extubation to 5 days resulted in baboons in the delayed nasal continuous positive airway pressure group having a lower arterial to alveolar oxygen ratio, high PaCO2, and worse respiratory function. The animals in the delayed nasal continuous positive airway pressure group exhibited a poor respiratory drive that contributed to more reintubations and time on mechanical ventilation. A few animals in both groups developed necrotizing enterocolitis and/or sepsis, but infectious pneumonias were not documented. Cellular bronchiolitis and peribronchiolar alveolar wall thickening were more frequently seen in the delayed nasal continuous positive airway pressure group. Bronchoalveolar lavage levels of interleukin-6, interleukin-8, monocyte chemotactic protein-1, macrophage inflammatory protein-1 alpha, and growth-regulated oncogene-alpha were significantly increased in the delayed nasal continuous positive airway pressure group. Standard and digital morphometric analyses showed no significant differences in internal surface area and nodal measurements between the groups. Platelet endothelial cell adhesion molecule vascular staining was not significantly different between the 2 nasal continuous positive airway pressure groups

  19. Modelo preditivo para displasia broncopulmonar ao final da primeira semana de vida Bronchopulmonary dysplasia prediction modelfor 7-day-old infants

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    Carlos A. Bhering

    2007-04-01

    Full Text Available OBJETIVO: Desenvolver um modelo preditivo capaz de identificar, ao final da primeira semana de vida, os recém-nascidos prematuros com maior probabilidade de evoluir para displasia broncopulmonar (DBP. MÉTODOS: Os dados foram coletados retrospectivamente entre janeiro de 1998 e julho de 2001, e prospectivamente de agosto de 2001 a julho de 2003. Foram incluídas todas as crianças nascidas na Instituição, com idade gestacional 2 dias e perda de > 15% do peso de nascimento no sétimo dia de vida. Nos pacientes com todas as variáveis presentes, o modelo permitiu uma probabilidade de acerto de 93,7%. Valores semelhantes foram obtidos com as 61 crianças utilizadas na validação do modelo. CONCLUSÕES O modelo preditivo desenvolvido em nossa população foi capaz de identificar com elevado grau de sensibilidade, ao final da primeira semana de vida, os recém-nascidos sob maior risco de evoluir para DBP.OBJECTIVE: To develop a predictive model capable of identifying which premature infants have the greatest probability of presenting bronchopulmonary dysplasia (BPD, based on assessment at the end of their first week of life. METHODS: Data were collected retrospectively from January 1998 to July 2001, and prospectively from August 2001 to July 2003. All children born at the institution with gestational age 2 days and loss of > 15% of birth weight on the seventh day of life. Where patients exhibited all of these variables, the model had a 93.7% probability of being correct. The model was further validated when using another sample of 61 newborns; similar figures were obtained. CONCLUSIONS: At the end of the first week of life, the predictive model developed from our population was capable of identifying newborn infants at increased risk of developing BPD with a high degree of sensitivity.

  20. Vitamin D treatment improves survival and infant lung structure after intra-amniotic endotoxin exposure in rats: potential role for the prevention of bronchopulmonary dysplasia.

    Science.gov (United States)

    Mandell, Erica; Seedorf, Gregory; Gien, Jason; Abman, Steven H

    2014-03-01

    Vitamin D (vit D) has anti-inflammatory properties and modulates lung growth, but whether vit D can prevent lung injury after exposure to antenatal inflammation is unknown. We hypothesized that early and sustained vit D treatment could improve survival and preserve lung growth in an experimental model of bronchopulmonary dysplasia induced by antenatal exposure to endotoxin (ETX). Fetal rats (E20) were exposed to ETX (10 μg), ETX + Vit D (1 ng/ml), or saline (control) via intra-amniotic (IA) injections and delivered 2 days later. Newborn pups exposed to IA ETX received daily intraperitoneal injections of vit D (1 ng/g) or saline for 14 days. Vit D treatment improved oxygen saturations (78 vs. 87%; P < 0.001) and postnatal survival (84% vs. 57%; P < 0.001) after exposure to IA ETX compared with IA ETX alone. Postnatal vit D treatment improved alveolar and vascular growth at 14 days by 45% and 25%, respectively (P < 0.05). Vit D increased fetal sheep pulmonary artery endothelial cell (PAEC) growth and tube formation by 64% and 44%, respectively (P < 0.001), and prevented ETX-induced reductions of PAEC growth and tube formation. Vit D directly increased fetal alveolar type II cell (ATIIC) growth by 26% (P < 0.001) and enhanced ATIIC growth in the presence of ETX-induced growth suppression by 73% (P < 0.001). We conclude that antenatal vit D therapy improved oxygenation and survival in newborn rat pups and enhanced late lung structure after exposure to IA ETX in vivo, which may partly be due to direct effects on vascular and alveolar growth.

  1. The Impact of Atrial Left-to-Right Shunt on Pulmonary Hypertension in Preterm Infants with Moderate or Severe Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Choi, Eui Kyung; Jung, Young Hwa; Kim, Han-Suk; Shin, Seung Han; Choi, Chang Won; Kim, Ee-Kyung; Kim, Beyong Il; Choi, Jung-Hwan

    2015-10-01

    Bronchopulmonary dysplasia (BPD)-associated pulmonary hypertension (PH) is a well-known complication of prematurity; however, the additional impact of a left-to-right interatrial shunt on this condition remains poorly understood. The aim of the present study was to identify the significance of atrial left-to-right shunt lesions in PH infants with moderate or severe BPD. The medical records of 383 preterm infants (gestational age of < 32 weeks) who were diagnosed with BPD between 2005 and 2013 were retrospectively reviewed. Baseline characteristics such as interatrial shunts and outcomes were compared between the infants who developed PH (n = 50) and infants who did not (n = 144). Infants with hemodynamically significant residual patent ductus arteriosus were excluded. Among the infants diagnosed with PH (n = 50), the outcomes were compared between the patients with (n = 21) and without atrial shunts (n = 29) at 36 weeks corrected postmenstrual age. Fifty (15%) preterm infants with BPD were diagnosed with PH. The number of infants with a history of atrial shunt lesions was significantly higher in the PH group than in the non-PH group (42% vs. 15.3%, respectively). The adjusted odds ratio for PH in the atrial shunt group was 3.8 (95% confidence interval, 1.8-8.0), compared to PH-BPD infants without an atrial shunt. The presence of an atrial left-to-right shunt was associated with PH in preterm infants with moderate or severe BPD. Close follow up is needed for infants with interatrial shunts, and a more tailored prognostic evaluation and treatment are recommended. Copyright © 2015. Published by Elsevier B.V.

  2. BNP, troponin I, and YKL-40 as screening markers in extremely preterm infants at risk for pulmonary hypertension associated with bronchopulmonary dysplasia.

    Science.gov (United States)

    König, Kai; Guy, Katelyn J; Nold-Petry, Claudia A; Barfield, Charles P; Walsh, Geraldine; Drew, Sandra M; Veldman, Alex; Nold, Marcel F; Casalaz, Dan M

    2016-12-01

    Bronchopulmonary dysplasia (BPD) is often complicated by pulmonary hypertension (PH). We investigated three biomarkers potentially suitable as screening markers for extremely preterm infants at risk of BPD-associated PH. In this prospective observational cohort study conducted in a tertiary neonatal intensive care unit, 83 preterm infants with BPD born <28-wk gestation and still inpatients at 36-wk corrected age received an echocardiogram and blood tests of B-type natriuretic peptide (BNP), troponin I, and YKL-40. Infants were analyzed according to echocardiographic evidence of tricuspid regurgitation (TR). Thirty infants had evidence of TR on echocardiogram at 36-wk corrected age. Infants with or without TR had similar baseline demographics: mean ± SD gestational age 26(1) ± 1(2) vs. 26(1) ± 1(1) wk and birth weight 830 ± 206 vs. 815 ± 187 g, respectively. There was no difference in duration of respiratory support. The right ventricular systolic pressure of infants with evidence of TR was 40 ± 16 mmHg. BNP was the only biomarker that proved to be significantly higher in infants with evidence of TR: median (interquartile range) serum level 54.5 (35-105) vs. 41.5 (30-59) pg/ml, P = 0.043. Subgroup analysis of infants with severe BPD requiring discharge on home oxygen or BPD-related mortality revealed similar results. There was no difference between groups for troponin I and YKL-40. In conclusion, increased serum levels of BNP were associated with evidence of TR at 36-wk corrected gestational age in extremely preterm infants, suggesting a potential role as a screening biomarker for BPD-associated PH. Copyright © 2016 the American Physiological Society.

  3. Two-Year Follow-Up Outcomes of Premature Infants Enrolled in the Phase I Trial of Mesenchymal Stem Cells Transplantation for Bronchopulmonary Dysplasia.

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    Ahn, So Yoon; Chang, Yun Sil; Kim, Ji Hye; Sung, Se In; Park, Won Soon

    2017-06-01

    To determine the long-term safety and outcomes of mesenchymal stem cells (MSCs) for bronchopulmonary dysplasia in premature infants enrolled in a previous phase I clinical trial up to 2 years of corrected age (CA). We assessed serious adverse events, somatic growth, and respiratory and neurodevelopmental outcomes at visit 1 (4-6 months of CA), visit 2 (8-12 months of CA), and visit 3 (18-24 months of CA) in a prospective longitudinal follow-up study up to 2 years' CA of infants who received MSCs (MSC group). We compared these data with those from a historical case-matched comparison group. One of 9 infants in the MSC group died of Enterobacter cloacae sepsis at 6 months of CA, the remaining 8 infants survived without any transplantation-related adverse outcomes, including tumorigenicity. No infant in the MSC group was discharged with home supplemental oxygen compared with 22% in the comparison group. The average rehospitalization rate in the MSC group was 1.4/patient because of respiratory infections during 2 years of follow-up. The mean body weight of the MSC group at visit 3 was significantly higher compared with that of the comparison group. No infant in the MSC group was diagnosed with cerebral palsy, blindness, or developmental delay; in the comparison group, 1 infant was diagnosed with cerebral palsy and 1 with developmental delay. Intratracheal transplantation of MSCs in preterm infants appears to be safe, with no adverse respiratory, growth, and neurodevelopmental effects at 2 years' CA. ClinicalTrials.gov: NCT01632475. Copyright © 2017 The Author(s). Published by Elsevier Inc. All rights reserved.

  4. Altered vasoreactivity in neonatal rats with pulmonary hypertension associated with bronchopulmonary dysplasia: Implication of both eNOS phosphorylation and calcium signaling.

    Directory of Open Access Journals (Sweden)

    Eric Dumas de la Roque

    Full Text Available Bronchopulmonary dysplasia (BPD consists of an arrest of pulmonary vascular and alveolar growth, with persistent hypoplasia of the pulmonary microvasculature and alveolar simplification. In 25 to 40% of the cases, BPD is complicated by pulmonary hypertension (BPD-PH that significantly increases the risk of morbidity. In vivo studies suggest that increased pulmonary vascular tone could contribute to late PH in BPD. Nevertheless, an alteration in vasoreactivity as well as the mechanisms involved remain to be confirmed. The purpose of this study was thus to assess changes in pulmonary vascular reactivity in a murine model of BPD-PH. Newborn Wistar rats were exposed to either room air (normoxia or 90% O2 (hyperoxia for 14 days. Exposure to hyperoxia induced the well-known features of BPD-PH such as elevated right ventricular systolic pressure, right ventricular hypertrophy, pulmonary vascular remodeling and decreased pulmonary vascular density. Intrapulmonary arteries from hyperoxic pups showed decreased endothelium-dependent relaxation to acetylcholine without any alteration of relaxation to the NO-donor sodium nitroprusside. This functional alteration was associated with a decrease of lung eNOS phosphorylation at the Ser1177 activating site. In pups exposed to hyperoxia, serotonin and phenylephrine induced exacerbated contractile responses of intrapulmonary arteries as well as intracellular calcium response in pulmonary arterial smooth muscle cells (PASMC. Moreover, the amplitude of the store-operated Ca2+ entry (SOCE, induced by store depletion using a SERCA inhibitor, was significantly greater in PASMC from hyperoxic pups. Altogether, hyperoxia-induced BPD-PH alters the pulmonary arterial reactivity, with effects on both endothelial and smooth muscle functions. Reduced activating eNOS phosphorylation and enhanced Ca2+ signaling likely account for alterations of pulmonary arterial reactivity.

  5. Altered vasoreactivity in neonatal rats with pulmonary hypertension associated with bronchopulmonary dysplasia: Implication of both eNOS phosphorylation and calcium signaling.

    Science.gov (United States)

    Dumas de la Roque, Eric; Smeralda, Gwladys; Quignard, Jean-François; Freund-Michel, Véronique; Courtois, Arnaud; Marthan, Roger; Muller, Bernard; Guibert, Christelle; Dubois, Mathilde

    2017-01-01

    Bronchopulmonary dysplasia (BPD) consists of an arrest of pulmonary vascular and alveolar growth, with persistent hypoplasia of the pulmonary microvasculature and alveolar simplification. In 25 to 40% of the cases, BPD is complicated by pulmonary hypertension (BPD-PH) that significantly increases the risk of morbidity. In vivo studies suggest that increased pulmonary vascular tone could contribute to late PH in BPD. Nevertheless, an alteration in vasoreactivity as well as the mechanisms involved remain to be confirmed. The purpose of this study was thus to assess changes in pulmonary vascular reactivity in a murine model of BPD-PH. Newborn Wistar rats were exposed to either room air (normoxia) or 90% O2 (hyperoxia) for 14 days. Exposure to hyperoxia induced the well-known features of BPD-PH such as elevated right ventricular systolic pressure, right ventricular hypertrophy, pulmonary vascular remodeling and decreased pulmonary vascular density. Intrapulmonary arteries from hyperoxic pups showed decreased endothelium-dependent relaxation to acetylcholine without any alteration of relaxation to the NO-donor sodium nitroprusside. This functional alteration was associated with a decrease of lung eNOS phosphorylation at the Ser1177 activating site. In pups exposed to hyperoxia, serotonin and phenylephrine induced exacerbated contractile responses of intrapulmonary arteries as well as intracellular calcium response in pulmonary arterial smooth muscle cells (PASMC). Moreover, the amplitude of the store-operated Ca2+ entry (SOCE), induced by store depletion using a SERCA inhibitor, was significantly greater in PASMC from hyperoxic pups. Altogether, hyperoxia-induced BPD-PH alters the pulmonary arterial reactivity, with effects on both endothelial and smooth muscle functions. Reduced activating eNOS phosphorylation and enhanced Ca2+ signaling likely account for alterations of pulmonary arterial reactivity.

  6. Azithromycin To Prevent Bronchopulmonary Dysplasia in Ureaplasma-Infected Preterm Infants: Pharmacokinetics, Safety, Microbial Response, and Clinical Outcomes with a 20-Milligram-per-Kilogram Single Intravenous Dose

    Science.gov (United States)

    Othman, Ahmed A.; Hassan, Hazem E.; Eddington, Natalie D.; Abebe, Elias; Terrin, Michael L.; Kaufman, David A.; Waites, Ken B.

    2013-01-01

    Ureaplasma respiratory tract colonization is associated with bronchopulmonary dysplasia (BPD) in preterm infants. Previously, we demonstrated that a single intravenous (i.v.) dose of azithromycin (10 mg/kg of body weight) is safe but inadequate to eradicate Ureaplasma spp. in preterm infants. We performed a nonrandomized, single-arm open-label study of the pharmacokinetics (PK) and safety of intravenous 20-mg/kg single-dose azithromycin in 13 mechanically ventilated neonates with a gestational age between 24 weeks 0 days and 28 weeks 6 days. Pharmacokinetic data from 25 neonates (12 dosed with 10 mg/kg i.v. and 13 dosed with 20 mg/kg i.v.) were analyzed using a population modeling approach. Using a two-compartment model with allometric scaling of parameters on body weight (WT), the population PK parameter estimates were as follows: clearance, 0.21 liter/h × WT(kg)0.75 [WT(kg)0.75 indicates that clearance was allometrically scaled on body weight (in kilograms) with a fixed exponent of 0.75]; intercompartmental clearance, 2.1 liters/h × WT(kg)0.75; central volume of distribution (V), 1.97 liters × WT (kg); and peripheral V, 17.9 liters × WT (kg). There was no evidence of departure from dose proportionality in azithromycin exposure over the tested dose range. The calculated area under the concentration-time curve over 24 h in the steady state divided by the MIC90 (AUC24/MIC90) for the single dose of azithromycin (20 mg/kg) was 7.5 h. Simulations suggest that 20 mg/kg for 3 days will maintain azithromycin concentrations of >MIC50 of 1 μg/ml for this group of Ureaplasma isolates for ≥96 h after the first dose. Azithromycin was well tolerated with no drug-related adverse events. One of seven (14%) Ureaplasma-positive subjects and three of six (50%) Ureaplasma-negative subjects developed physiologic BPD. Ureaplasma was eradicated in all treated Ureaplasma-positive subjects. Simulations suggest that a multiple-dose regimen may be efficacious for microbial clearance

  7. Analysis of the Functional State of the Gastrointestinal Tract in Children with Bronchopulmonary Dyspasia

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    H.S. Senatorova

    2015-09-01

    Full Text Available The features of the functional state of the gastrointestinal tract in children with bronchopulmonary dysplasia have been analyzed. It has been shown that children with bronchopulmonary dysplasia have significantly lower indicators of physical development than apparently healthy children. The dependence between hypoproteinemia in the blood serum and pathological changes in stool test of children with bronchopulmonary dysplasia was established that should be taken into account during the clinical management of these patients.

  8. How Is Bronchopulmonary Dysplasia Treated?

    Science.gov (United States)

    ... Later, babies may be given breast milk or infant formula through feeding tubes that are passed through their noses or mouths ... child's long-term care and make treatment recommendations. Infants who need ... It allows a breathing tube to be placed directly into the windpipe, rather ...

  9. Influência de fatores maternos e neonatais no desenvolvimento da displasia broncopulmonar Influence of maternal and neonatal factors on bronchopulmonary dysplasia development

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    Marcela Raquel de Oliveira Lima

    2011-08-01

    Full Text Available OBJETIVO: Analisar as características epidemiológicas da displasia broncopulmonar (DBP e suas relações com condições maternas e neonatais em uma unidade neonatal. MÉTODOS: Estudo transversal, descritivo e analítico, sendo os dados coletados através da análise de prontuários envolvendo recém-nascidos (RNs pré-termo com peso ao nascimento inferior a 1.500 g e idade gestacional abaixo de 37 semanas internados em uma unidade neonatal. RESULTADOS: Foram estudados 323 recém-nascidos com média do peso ao nascimento de 1.161 g (± 231 g, idade gestacional entre 24 e 36,5 semanas com incidência da DBP de 17,6%. Entre os RNs que desenvolveram DBP, a média de dias de uso de assistência ventilatória mecânica invasiva (AVMI, ventilação não invasiva (VNI e oxigênio foi, respectivamente, 17,6 dias, 16,2 dias e 46,1 dias, sendo significativamente maior naqueles RNs que desenvolveram a DBP (p < 0,001. A ocorrência da DBP foi significativamente maior nos RNs com diagnóstico de persistência do canal arterial (PCA. CONCLUSÃO: A incidência da DBP neste estudo foi semelhante à encontrada na literatura mundial. Não houve associação entre a presença de infecção materna e o uso de corticoide antenatal com a DBP. Os RNs que fizeram uso de surfactante tiveram maior incidência da DBP porque tinham menor PN e menor IG. A ocorrência da PCA e DBP simultaneamente está associada ao maior tempo de uso de AVMI, VNI e oxigênioOBJECTIVE: To review epidemiological features of bronchopulmonary dysplasia (BPD and its relationship with maternal and neonatal conditions in a neonatal unit. METHODS: Cross-sectional, descriptive and analytical study involving preterm newborns (NBs with a birth weight lower than 1,500 g and gestational age under 37 weeks. Data was collected through a review of medical records of these newborns admitted to a neonatal unit. RESULTS: The study included 323 newborns with a mean birth weight of 1,161 g (± 231 g

  10. Bronchopulmonary dysplasia: Clinical practices in five Portuguese neonatal intensive care units Displasia broncopulmonar: Práticas clínicas em cinco unidades de cuidados intensivos neonatais

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    H Guimarães

    2010-04-01

    Full Text Available With the advent of surfactant, prenatal corticosteroids (PNC and advances in technology, the survival rate of extremely low birth weight (ELBW infants has improved dramatically. Rates of bronchopulmonary dysplasia (BPD vary widely among neonatal intensive care units (NICUs and many studies using multiple interventions have shown some improvement in BPD rates. Implementing potentially better practices to reduce BPD has been an effort made over the last few decades. Aim: To compare five Portuguese NICUs in terms of clinical practices in very low birth weight (VLBW infants, in order to developbetter practices to prevent BPD. Patients and methods: 256 preterm neonates, gestational age (GA Com o advento do surfactante, dos corticosteróides prénatais e dos avanços na tecnologia, a sobrevida dos recém-nascidos de extremo baixo peso tem melhorado dramaticamente. As taxas de displasia broncopulmonar (DBP variam amplamente entre unidades, e vários estudos, avaliando resultados de múltiplas intervenções, têm mostrado alguma melhoria na prevalência da DBP. A implementação de potenciais boas práticas na DBP tem sido adoptada por muitos serviços nas últimas décadas. Objectivo: Comparar cinco unidades portuguesas de cuidados intensivos neonatais no que se refere as práticas clínicas no tratamento dos recém-nascidos de muito baixo peso, para desenvolver e melhorar as boas práticas na prevenção da DBP. População e métodos: Foram estudados 256 recém-nascidos com a idade gestacional inferior a 30 semanas e/ou peso ao nascer inferior a 1250 g, admitidos nas cinco unidades portuguesas (centros 1 a 5 entre 1 de Janeiro de 2004 e 31 de Dezembro de 2006. Foram excluídos os recém-nascidos com malformações major, hemorragia intraventricular grau IV na primeira semana de vida e com doença metabólica ou neuromuscular. Definimos DBP como a dependência do oxigénio às 36 semanas de idade pós-concepcional. A ecessidade de melhorar

  11. Avaliação tomográfica pulmonar tardia em prematuros com displasia broncopulmonar e persistência de canal arterial Late pulmonary tomography assessment in premature infants with bronchopulmonary dysplasia submitted to patent ductus arteriosus managemnent

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    Lilian Beani

    2007-12-01

    Full Text Available OBJETIVO: Avaliação tomográfica do parênquima pulmonar de crianças nascidas prematuras de muito baixo peso, com persistência do canal arterial submetidas a tratamento clínico ou cirúrgico e que apresentaram displasia broncopulmonar. MÉTODOS: Entre dezembro de 2006 e janeiro de 2007, 14 crianças foram submetidas à tomografia computadorizada de alta resolução (TCAR, que nasceram prematuras, peso inferior a 1500 gramas, com displasia broncopulmonar (DBP e persistência do canal arterial (PCA, os quais necessitaram tratamento para oclusão do canal, sendo divididos em dois grupos: A - clínico (n = 6 e B - cirúrgico (n = 8. Nove pacientes eram do sexo masculino e cinco, do feminino, com idade média de 36,5±4,3 meses. As TCAR foram analisadas por dois observadores independentes e as lesões quantificadas em cada paciente. Para análises estatísticas, foi utilizado o teste de Mann-Whitney e considerados significantes valores de pOBJECTIVE: To assess through high-resolution computed tomography the pulmonary parenchyma of children prematurely born with both very low birth weight and patent ductus arteriosus submitted to medical or surgical treatment that developed bronchopulmonary dysplasia. METHODS: Between December 2006 and January 2007, 14 children prematurely born with a weight less than 1500g with bronchopulmonary dysplasia (BPD and patent ductus arteriosus (PDA were submitted to high-resolution computed tomography (HRCT. All of them underwent surgical closure of the canal divided into two groups: A - medical (n=6 and B - surgical (n=8. The pool of patients comprised 9 baby boys and 5 girls who were 36.5±4.3 month-old. The HRCT were analyzed by two independent observers and quantified in each patient. The statistical analyses were assessed using the Mann-Whitney test, and p<0.05 was considered statistically significant. RESULTS: Three patients presented normal tomographies, being two of A group and one of B. In A, the most

  12. Cervical dysplasia

    Science.gov (United States)

    ... squamous cells - dysplasia; Pap smear - dysplasia; HPV - dysplasia; Human papilloma virus - dysplasia; Cervix - dysplasia; Colposcopy - dysplasia Images Female reproductive anatomy Cervical neoplasia Uterus Cervical dysplasia - series References American ...

  13. Bronchopulmonary dysplasia as a predictor factor for motor alteration at 6 months corrected age in premature infants Displasia broncopulmonar como fator predisponente para alterações motoras aos 6 meses em prematuros

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    Priscila Silveira Martins

    2010-10-01

    Full Text Available OBJECTIVE: The study aimed to assess bronchopulmonary dysplasia (BPD as a predisposing factor for alteration in the psychomotor development index (PDI in premature infants and verify the incidence of neuromotor alterations at 6 months corrected age. METHOD: This was a prospective cohort study that followed the neuromotor development of 152 very low birth weight premature infants, with psychomotor development index as the outcome. The study used the Bayley Scale of Infant Development at 6 months corrected age, and neurological examination. RESULTS: Incidence of BPD was 13.2% (n=20. Logistic regression analysis showed an association between BPD and altered psychomotor development index (OR 3.98; 95%CI: 1.04-15.1 after adjusting for confounding variables. Neurological examination was altered in 67.1% of the 152 infants. CONCLUSION: Bronchopulmonary dysplasia acted as an independent predisposing factor for alteration in the psychomotor development index in premature infants at 6 months corrected age.OBJETIVO: Avaliar a displasia broncopulmonar (DBP como fator predisponente para alteração no índice de desenvolvimento psicomotor em prematuros e verificar a incidência das alterações neuromotoras aos 6 meses de idade corrigida. MéTODO: Estudo de coorte prospectivo que acompanhou o desenvolvimento neuromotor de 152 prematuros de muito baixo peso, cujo desfecho foi o desenvolvimento psicomotor. Utilizou-se a Bayley Scale of Infant Development aos 6 meses de idade corrigida e exame neurológico. RESULTADOS: A incidência de DBP foi de 13,2% (n=20. A análise de regressão logística mostrou associação entre a DBP e alteração no índice de desenvolvimento psicomotor (RC 3,98 IC 95%:1,04-15,1 após ajuste para as variáveis de confundimento. O exame neurológico apresentou-se alterado em 67,1% das 152 crianças. CONCLUSão: A displasia broncopulmonar atuou como fator predisponente independente para alteração no índice de desenvolvimento

  14. Phenotypic assessment of pulmonary hypertension using high-resolution echocardiography is feasible in neonatal mice with experimental bronchopulmonary dysplasia and pulmonary hypertension: a step toward preventing chronic obstructive pulmonary disease.

    Science.gov (United States)

    Reynolds, Corey L; Zhang, Shaojie; Shrestha, Amrit Kumar; Barrios, Roberto; Shivanna, Binoy

    2016-01-01

    Bronchopulmonary dysplasia (BPD) and chronic obstructive pulmonary disease (COPD) are chronic lung diseases of human infants and adults, respectively, that are characterized by alveolar simplification. One-third of the infants with severe BPD develop pulmonary hypertension (PH). More importantly, PH increases morbidity and mortality in BPD patients. Additionally, COPD is a common respiratory morbidity in former BPD patients. The lack of an appropriate small animal model wherein echocardiography (Echo) can demonstrate PH is one of the major barriers to understand the molecular mechanisms of the disease and, thereby, develop rational therapies to prevent and/or treat PH in BPD patients. Thus, the goal of this study was to establish a model of experimental BPD and PH and investigate the feasibility of Echo to diagnose PH in neonatal mice. Since hyperoxia-induced oxidative stress and inflammation contributes to the development of BPD with PH, we tested the hypothesis that exposure of newborn C57BL/6J mice to 70% O2 (hyperoxia) for 14 days leads to lung oxidative stress, inflammation, alveolar and pulmonary vascular simplification, pulmonary vascular remodeling, and Echo evidence of PH. Hyperoxia exposure caused lung oxidative stress and inflammation as evident by increased malondialdehyde adducts and inducible nitric oxide synthase, respectively. Additionally, hyperoxia exposure caused growth restriction, alveolar and pulmonary vascular simplification, and pulmonary vascular remodeling. At 14 days of age, Echo of these mice demonstrated that hyperoxia exposure decreased pulmonary acceleration time (PAT) and PAT/ejection time ratio and increased right ventricular free wall thickness, which are indicators of significant PH. Thus, we have demonstrated the feasibility of Echo to phenotype PH in neonatal mice with experimental BPD with PH, which can aid in discovery of therapies to prevent and/or treat BPD with PH and its sequelae such as COPD in humans.

  15. Avaliação da aptidão cardiorrespiratória de crianças com displasia broncopulmonar Cardiorespiratory capacity assessment on children with bronchopulmonary dysplasia

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    LR Abreu

    2007-04-01

    Full Text Available OBJETIVO: Avaliar a aptidão cardiorrespiratória e verificar a presença de broncoespasmo induzido pelo exercício (BIE em crianças com displasia broncopulmonar (DBP. MÉTODO: Foram realizadas prova de função pulmonar e análise de gases em um teste cardiopulmonar, em 46 crianças com idade entre 7 a 10 anos, formando três grupos: crianças nascidas pré-termo com DBP, (DBP, n= 13; crianças nascidas pré-termo sem DBP, (RNPT, n= 13; e crianças saudáveis nascidas a termo, (Controle, n= 20. RESULTADOS: A duração dos testes foi 7,70 ± 1,49; 9,1 ± 2,02 e 8,4 ± 2,12 min; o VO2máximo foi 35,98 ± 5,33; 38,99 ± 6,73 e 34,91 ± 6,09 ml/kg/min; e a VE foi 28,54 ± 7,39; 28,84 ± 5,98 e 28,96 ± 6,96 l/min para os grupos DBP, RNPT e Controle, respectivamente. Não foram encontradas diferenças significantes entre os grupos (p> 0,05. A FCmáxima foi 188 ± 9,37; 196 ± 5,15 e 197 ± 10,90 bpm; a taxa de troca gasosa máxima (R foi 1,21 ± 0,22; 1,10 ± 0,06 e 1,05 ± 0,05 para os grupos DBP, RNPT e Controle, respectivamente, sendo esses valores diferentes entre o grupo Controle e DBP (pOBJECTIVE: To assess cardiorespiratory capacity and investigate the presence of exercise-induced bronchospasm among children with bronchopulmonary dysplasia. METHOD: Pulmonary function tests and gas analyses were performed in a cardiopulmonary test on 46 children aged 7-10 years. Three groups were formed: children born prematurely with bronchopulmonary dysplasia (BPD; n= 13, children born prematurely without bronchopulmonary dysplasia (Preterm; n= 13 and healthy children born at full term (Control; n= 20. RESULTS: The test duration was 7.70 ± 1.49; 9.1 ± 2.02 and 8.4 ± 2.12 min; VO2max was 35.98 ± 5.33; 38.99 ± 6.73 and 34.91 ± 6.09 ml/kg/min; and VE was 28.54 ± 7.39; 28.84 ± 5.98 and 28.96 ± 6.96 l/min for the BPD, Preterm and Control groups respectively. There were no significant differences between the groups (p> 0.05. The maximum heart rate was

  16. Displasia broncopulmonar: incidência, fatores de risco e utilização de recursos em uma população sul-americana de recém-nascidos de muito baixo peso Bronchopulmonary dysplasia: incidence, risk factors and resource utilization in a population of South-American very low birth weight infants

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    Jose L. Tapia

    2006-02-01

    .OBJECTIVE: To determine the incidence of bronchopulmonary dysplasia, its risk factors and resource utilization in a large South American population of very low birth weight infants. METHODS: Data were prospectively collected from infants weighing 500 to 1,500 g born at 16 NEOCOSUR Network centers from 10/2000 through 12/2003. Multivariate relative risk and 95% confidence intervals were estimated by Poisson regression with robust error variance to find factors that affected the risk of bronchopulmonary dysplasia. RESULTS: 1,825 very low birth weight infant survivors were analyzed. Mean birth weight and gestational age were 1085+279 g and 29+3 weeks respectively. Bronchopulmonary dysplasia incidence averaged 24.4% and survival without bronchopulmonary dysplasia augmented with increasing gestational age. Higher birth weight and gestational age and a female gender all decreased the risk for bronchopulmonary dysplasia. Factors that independently increased that risk were surfactant requirement, mechanical ventilation, air leak, patent ductus arteriosus, late onset sepsis and necrotizing enterocolitis. Bronchopulmonary dysplasia infants had more days of hospitalization (91±27 vs. 51±19, on mechanical ventilation (19±20 vs. 4±7 and oxygen therapy (72±30 vs. 8±14 in comparison with non BPD infants. CONCLUSIONS: Bronchopulmonary dysplasia incidence was 24.4% in a large South American population and is related to greater resource utilization. Risk factors for bronchopulmonary dysplasia in this study were: surfactant requirement, mechanical ventilation, air leak, patent ductus arteriosus, late onset sepsis and necrotizing enterocolitis. These studies may provide information useful to the design of effective preventive perinatal strategies.

  17. Transição alimentar em recém-nascidos com displasia broncopulmonar Transition time for full oral feeding in newborns with bronchopulmonary dysplasia

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    Daiana Evangelista

    2009-03-01

    Full Text Available OBJETIVO: verificar o tempo de transição da sonda para a via oral plena nos lactentes com Displasia Broncopulmonar (DBP e descrever as intercorrências observadas durante os períodos de alimentação. MÉTODOS: revisão de prontuários do setor de Fonoaudiologia de 32 lactentes nascidos no Hospital Universitário Pedro Ernesto (HUPE/UERJ divididos em 2 grupos. Grupo 1: 18 lactentes com diagnóstico de DBP; grupo 2: 18 lactentes com idade gestacional entre 29 e 32 semanas sem complicações clínicas relevantes que foram utilizados como grupo controle. Foram registrados o tempo de transição alimentar, as intercorrências durante a alimentação e via oral na alta hospitalar de ambos os grupos. RESULTADOS: média e desvio padrão do tempo de transição alimentar dos grupos 1 e 2 respectivamente: 18,22 dias e 14,79; 6,50 dias e 3,68, com p=0,002. Nos broncodisplásicos foram comuns intercorrências respiratórias, dificuldade de coordenação sucçãoXrespiraçãoXdeglutição, dificuldades no padrão oral, além de sinais de retraimento e rebaixamento do estado de consciência durante a alimentação. Apesar disso, 10 lactentes (52,63% tiveram alta em aleitamento materno exclusivo e 3 (15,78% em aleitamento misto. CONCLUSÃO: pacientes com DBP precisaram de um período maior de treino de VO (18 dias, além de serem passíveis de intercorrências durante a alimentação. No entanto, constata-se que foi possível o aleitamento materno exclusivo.PURPOSE: to check the time of transition from the feeding tube to oral feeding in newborns with Bronchopulmonar Dysplasia (BPD; observe the difficulties occurred during the oral feeding./ METHODS: the speech pathology department reviewed thirty-two past cases of newborns born in the Pedro Ernesto Hospital of Rio de Janeiro State University. The first group was composed of eighteen newborns with BPD, while a second group was composed of eighteen healthy newborns born between 29 and 32 weeks used as a

  18. Fatores associados à displasia broncopulmonar em prematuros sob ventilação mecânica precoce Factors associated with bronchopulmonary dysplasia in premature infants under early mechanical ventilation

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    Paula Eylla Cristina Rodrigues Duarte

    2012-06-01

    Full Text Available OBJETIVOS: descrever os fatores neonatais e de assistência ventilatória associados à displasia broncopulmonar (DBP, e verificar sua frequência em recém-nascidos prematuros submetidos à ventilação mecânica (VM na primeira semana de vida. MÉTODOS: coorte retrospectiva, realizada em Unidade de Terapia Intensiva Neonatal. Foram analisados prontuários de 86 prematuros, sob VM na primeira semana de vida e registrados dados neonatais, parâmetros da VM e sua relação com a DBP. Para verificar a associação entre as variáveis do estudo e a DBP utilizou-se o teste do qui-quadrado e o Exato de Fisher quando indicado. O teste t e o Kruskal Wallis foram utilizados para a comparação das médias das variáveis contínuas. RESULTADOS: a DBP ocorreu em 17,4%. Foram relacionados à doença: menor peso ao nascer e idade gestacional, Apgar OBJECTIVES: Objectives: to describe the neonatal and assisted ventilation factors associated with bronchopulmonary dysplasia (BPD and verify their frequency in premature newborns undergoing mechanical ventilation (MV in the first week of life. METHODS: retrospective cohort study carried out at the Neonatal Intensive Care Unit. The medical records of 86 premature infants under MV in the first week of life were analyzed and neonatal data, MV parameters and their relationship with BPD registered. To verify the association between the variables of the study and BPD, the chi-square test and the Fisher exact test were used as appropriate. The t-test and Kruskal Wallis test were used to compare the means of the continuous variables. RESULTS: BPD occurred in 17.4% of cases. Factors related to the illness were: lower birth weight and gestational age, Apgar <7 at 1 and 5 minutes, greater time under antibiotic therapy, parenteral nutrition and MV, higher values of the fraction of inspired oxygen (FiO2, MV as the first respiratory aid, lower volume of enteral nutrition and ponderal gain. No difference was found in the level

  19. Avaliação neurológica de recém-nascidos pré-termo de muito baixo peso com displasia broncopulmonar Neurological assessment of very low birth weight infants with bronchopulmonary dysplasia

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    Tathiana Ghisi de Souza

    2009-03-01

    Full Text Available OBJETIVO: Descrever e comparar a avaliação neurológica e comportamental de recém-nascidos pré-termos com e sem displasia broncopulmonar (DBP. MÉTODOS: Recém-nascidos prematuros com peso ao nascer inferior a 1500g e idade gestacional menor de 32 semanas foram avaliados com 40 semanas de idade gestacional corrigida, no Centro de Atenção Integral à Saúde da Mulher da Universidade Estadual de Campinas. Utilizou-se a Avaliação Neurológica de Dubowitz, com 29 itens divididos em seis categorias: tônus, padrões de tônus, reflexos, movimentos, sinais anormais e comportamento. O estado de consciência do recém-nascido foi graduado segundo Brazelton (1973. Utilizaram-se os testes do qui-quadrado e exato de Fischer para variáveis qualitativas e o de Mann-Whitney para as numéricas não-paramétricas, com nível de significância de 5%. RESULTADOS: No período de janeiro de 2005 a setembro de 2007, 24 recém-nascidos, 12 com DBP e 12 controles, com idade gestacional ao nascer de 28±1 semana e peso de 884±202g no grupo com DBP e 31±1 semana e 1156±216g no Grupo Controle foram avaliados. Dos 29 itens avaliados, 18 foram homogêneos entre os grupos e a pontuação geral dos dois grupos não apresentou diferença (p=0,30. Observou-se maior anormalidade neurológica no grupo com DBP em oito itens e, no Grupo Controle, em três itens. CONCLUSÕES: A comparação da avaliação neurológica de Dubowitz de recém-nascidos pré-termos com e sem DBP não apresentou diferença significante com 40 semanas de idade gestacional corrigida. Nas categorias reflexos e postura/tônus, observou-se tendência a anormalidade no grupo DBP.OBJECTIVE: To compare the neurological assessment of preterm newborn infants with and without bronchopulmonary dysplasia (BPD. METHODS: Preterm newborn infants with birth weight less than 1,500g and gestational age less than 32 weeks were evaluated by Dubowitz Method at 40 weeks of corrected gestational age. All infants

  20. Use of corticosteroids and the outcome of infants with bronchopulmonary dysplasia Uso de Corticosteróides e evolução de recém-nascidos com displasia broncopulmonar

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    Marta M. G. B. Mataloun

    1999-12-01

    Full Text Available Ventilator-dependent premature infants are often treated with dexamethasone. Several trials showed that steroids while improve pulmonary compliance and facilitate extubation, some treated infants may have adverse effects, such as alterations of growth curves. We conducted this retrospective study to evaluate the effects of steroids on mechanical ventilation, oxygen therapy, hospital length stay and mortality, in ventilator-dependent infants with bronchopulmonary dysplasia (BPD (defined as the need of oxygen supplementation at 28 days of life. Twenty-six newborns with BPD were evaluated during 9 -- 42 days postpartum (mean = 31 days and were divided into two groups: Group I - 14 newborns that did not receive dexamethasone, and Group II - 12 newborns that received dexamethasone at 14 --21 days of life. Dexamethasone was given at a dose of 0.25 mg per kilogram of body weight twice daily intravenously for 3 days, after which the dose was tapered. RESULTS: There were no statistically significant differences in the mean length of mechanical ventilation (Group I - 37 days, Group II - 35 days; oxygen supplementation (Group I - 16 days, Group II - 29 days; hospital stay (Group I - 72 days, Group II - 113 days; mortality (Group I - 35.7%, Group II - 41.6%. At birth, Group II was lighter (BW: Group I - 1154 grams ± 302, Group II - 791 grams ± 165; p Recém-nascidos (RN pré-termo dependentes de ventilação mecânica são frequentemente, tratados com corticosteróides. Vários estudos demonstraram que o uso de corticosteróides melhora a complacência pulmonar e facilita a extubação. No entanto, o uso de corticosteróides é associado , também, a alguns efeitos colaterais, como alterações na curva de crescimento dos recém-nascidos. Nós realizamos um estudo retrospectivo, para avaliar os efeitos dos corticosteróides sobre a duração da ventilação mecânica, oxigenoterapia como também sobre o tempo de internação e mortalidade, em rec

  1. Fatores de risco para a doença por refluxo gastroesofágico em recém-nascidos de muito baixo peso portadores de displasia broncopulmonar Risk factors for gastroesophageal reflux disease in very low birth weight infants with bronchopulmonary dysplasia

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    Thaís B. Mendes

    2008-04-01

    Full Text Available OBJETIVO: Conhecer os fatores de risco para a doença por refluxo gastroesofágico (DRGE em recém-nascidos de muito baixo peso com displasia broncopulmonar. MÉTODOS: Realizou-se um estudo caso-controle incluindo 23 casos e 23 controles com displasia broncopulmonar, sendo realizada investigação por monitorização prolongada do pH esofágico no período de janeiro de 2001 a outubro de 2005. Para cada caso, selecionou-se um controle, e foram comparados pela idade gestacional, peso ao nascimento, gênero, uso de corticóide pré-natal, tempo de ventilação assistida, tempo de oxigenoterapia, tempo de uso de sonda gástrica, uso de xantinas, idade pós-conceptual e peso durante a monitorização do pH esofágico. Realizou-se a análise por regressão logística múltipla para estabelecer o odds ratio (OR com intervalo de confiança de 95% (IC95%. RESULTADOS: Os dois grupos (com e sem DRGE não apresentaram diferenças significativas em relação às variáveis demográficas e de evolução pós-natal, uso de corticóide pré e pós-natal, bem como ao tempo de uso de cafeína, ventilação mecânica e oxigenoterapia. Entretanto, as variáveis intolerância alimentar (OR = 6,55; IC95% 1,05-40,8 e tempo de uso de sonda gástrica (OR = 1,67; IC95% 1,11-2,51 comportaram-se como fatores de risco para DRGE. A variável idade pós-conceptual ao exame de monitorização do pH (OR = 0,02; IC95% OBJECTIVE: To assess risk factors for gastroesophageal reflux disease (GERD in very low birth weight infants with bronchopulmonary dysplasia. METHODS: A case-control study was carried out in 23 cases and 23 control subjects with bronchopulmonary dysplasia submitted to 24-hour esophageal pH monitoring between January 2001 and October 2005. Cases and controls were compared for gestational age, birth weight, gender, use of antenatal steroids, duration of assisted ventilation, duration of oxygen therapy, length of gastric tube use, administration of xanthines

  2. [Pseudotumoral allergic bronchopulmonary aspergillosis].

    Science.gov (United States)

    Otero González, I; Montero Martínez, C; Blanco Aparicio, M; Valiño López, P; Verea Hernando, H

    2000-06-01

    Allergic bronchopulmonary aspergillosis (ABPA) develops as the result of a hypersensitivity reaction to fungi of the genus Aspergillus. Clinical and radiological presentation can be atypical, requiring a high degree of suspicion on the part of the physician who treats such patients. We report the cases of two patients with APBA in whom the form of presentation--with few asthma symptoms, images showing lobar atelectasia and hilar adenopathy--led to an initial suspicion of lung cancer.

  3. The significance of IL-1β +3953C>T, IL-6 -174G>C and -596G>A, TNF-α -308G>A gene polymorphisms and 86 bp variable number tandem repeat polymorphism of IL-1RN in bronchopulmonary dysplasia in infants born before 32 weeks of gestation

    Directory of Open Access Journals (Sweden)

    Dawid Szpecht

    2017-06-01

    Full Text Available Introduction : Bronchopulmonary dysplasia (BPD is a chronic lung disease that affects primarily preterm infants. Genetic factors are also taken into consideration in the pathogenesis of BPD. Genetic predispositions to higher production of inflammation mediators seem to be crucial. Material and methods: The aim of this study was to evaluate the possible relationship between polymorphisms: interleukin-1β +3953 C>T, interleukin-6 -174 G>C and -596 G>A, tumour necrosis factor -308 G>A and interleukin-1RN VNTR 86bp and the occurrence of BPD in a population of 100 preterm infants born from singleton pregnancy, before 32+0 weeks of gestation, exposed to antenatal steroids therapy, and without congenital abnormalities.  Results : In the study population BPD was diagnosed in 36 (36% newborns. Among the studied polymorphisms we found the higher prevalence for BPD developing of the following genotypes: 1/2 (OR 1.842 [0.673-5.025] and 2/2 IL-1RN (OR 1.75 [0.418-6.908] 86bpVNTR; GC (2.222 [0.658-8.706] and CC IL-6 -174G>C (1.6 [0.315-8.314] and GA (2.753 [0.828-10.64] and AA (1.5 [0.275-8.067] IL-6 -596G>A, GA 1.509 (0.515-4.301 TNF-α -308G>A. However, these finding were not statistically significant.  Conclusions : Genetic factors are undeniably involved in the pathogenesis of BPD. In the times of individualised therapy finding genes responsible for BPD might allow the development of new treatment strategies. A new way of specific therapy could ensure the reduction of complications connected with BPD and treatment costs.

  4. Metastasizing Bronchopulmonary Leiomyosarcoma

    Directory of Open Access Journals (Sweden)

    Speros Livieratos MD

    2015-04-01

    Full Text Available An otherwise healthy 55-year-old female, nonsmoker, was seen in pulmonary consultation for progressively worsening shortness of breath. She had undergone a complete hysterectomy 7 years prior for bleeding leiomyomas. On presentation, her initial chest X-ray showed a large right-sided pleural effusion with multiple pulmonary nodules. Two thoracenteses failed to reveal any cytologic abnormalities. Bronchoscopy revealed smooth, round, endobronchial lesions. Histologic examination showed features consistent with leiomyosarcoma. We present a rare case of a patient that initially had possible leiomyomas of the uterus surgically removed and years later presented with bronchopulmonary leiomyosarcoma.

  5. Ectodermal dysplasia

    Science.gov (United States)

    ... a wig and dentures to improve appearance. Use artificial tears to replace normal tearing and prevent drying ... dysplasia while the baby is still in the womb. Alternative Names Anhidrotic ectodermal dysplasia; Christ-Siemens-Touraine ...

  6. Imaging Bronchopulmonary Dysplasia—A Multimodality Update

    Science.gov (United States)

    Semple, Thomas; Akhtar, Mohammed R.; Owens, Catherine M.

    2017-01-01

    Bronchopulmonary dysplasia is the most common form of infantile chronic lung disease and results in significant health-care expenditure. The roles of chest radiography and computed tomography (CT) are well documented but numerous recent advances in imaging technology have paved the way for newer imaging techniques including structural pulmonary assessment via lung magnetic resonance imaging (MRI), functional assessment via ventilation, and perfusion MRI and quantitative imaging techniques using both CT and MRI. New applications for ultrasound have also been suggested. With the increasing array of complex technologies available, it is becoming increasingly important to have a deeper knowledge of the technological advances of the past 5–10 years and particularly the limitations of some newer techniques currently undergoing intense research. This review article aims to cover the most salient advances relevant to BPD imaging, particularly advances within CT technology, postprocessing and quantitative CT; structural MRI assessment, ventilation and perfusion imaging using gas contrast agents and Fourier decomposition techniques and lung ultrasound. PMID:28725645

  7. Bronchopulmonary dysplasia in a premature infant case report and ...

    African Journals Online (AJOL)

    She was successively managed with intermittent oxygen, dexamethasone, salbutamol and antibiotics (ceftriaxone). She was nursed in the incubator for 3 months. There was no episode of apneic attack throughout admission. She responded to treatment and was discharged home on intermittent oxygen therapy and ...

  8. Predictors of bronchopulmonary dysplasia and pulmonary hypertension in newborn children

    DEFF Research Database (Denmark)

    Ali, Zarqa; Peter, Schmidt; Dodd, James Keith

    2013-01-01

    %. Infants with BPD differed significantly from infants without BPD with regard to the following characteristics: Infants with BPD more frequently had a lower gestational age and BW, intubation at birth, mechanical ventilation within 24 hours of birth, a lower ­Apgar score at one minute and five minutes...

  9. Digital Chest Images For Bronchopulmonary Dysplasia In Children

    Science.gov (United States)

    Wang, Yen; Gross, George W.; D'Adamo, Arthur J.

    1989-05-01

    The clinical application of digital radiographic imaging has been the subject of several recent investigations (1-7). The digital radiographic image possesses certain advantages over the conventional analog radiographic image, such as wider dynamic range, potential for image manipulation, transfer, and storage, and radiation exposure reduction. However, the quality of image remains a valid concern to radiologists because they are accustomed to viewing conventional radiographs with a higher imaging quality.

  10. Ectodermal dysplasia

    Directory of Open Access Journals (Sweden)

    Sonia Saggoo

    2009-01-01

    Full Text Available Hereditary hypohidrotic ectodermal dysplasia, also called the Christ-Siemens-Touraine Syndrome is characterized by congenital dysplasia of one or more ectodermal structures and is manifested by hypohidrosis, hypotrichosis and hypodontia. It is usually an X-linked recessive mendelian character which is rarely seen in males. It results from abnormal morphogenesis of cutaneous and oral embryonic ectoderm. Patients with this disorder exhibit smooth , thin and dry skin, fine and blond scanty hair. Intra-orally anodontia or hypodontia, with impaired development of alveolar process is seen. A case report of a rare case of this disorder in a female patient aged 18 years is hereby presented.

  11. Frontofacionasal Dysplasia

    African Journals Online (AJOL)

    rme

    palpebral fissures, deformed nostrils, hypoplastic nasal wing, cleft lip, cleft palate and meningeocele. This association of anomalies suggests the diagnosis of frontofacionasal dysplasia and in our case is associated with facial heamangioma. To our knowledge, facial heamangioma in association with. FFND have not been ...

  12. Pseudoachondroplastic dysplasia.

    Directory of Open Access Journals (Sweden)

    Khungar A

    1993-04-01

    Full Text Available Pseudoachondroplasia is a heterogeneous inherited skeletal dysplasia in which dwarfism is a major feature. We report here a case of a 7 year old girl misdiagnosed as rickets, who presented with short stature, lordosis, genu varum and flexion deformities at both the elbows. Skeletal survey revealed epiphyseal and metaphyseal irregularities. A review of literature is also presented.

  13. Pictorial essay: Allergic bronchopulmonary aspergillosis

    Directory of Open Access Journals (Sweden)

    Ritesh Agarwal

    2011-01-01

    Full Text Available Allergic bronchopulmonary aspergillosis (ABPA is the best-known allergic manifestation of Aspergillus-related hypersensitivity pulmonary disorders. Most patients present with poorly controlled asthma, and the diagnosis can be made on the basis of a combination of clinical, immunological, and radiological findings. The chest radiographic findings are generally nonspecific, although the manifestations of mucoid impaction of the bronchi suggest a diagnosis of ABPA. High-resolution CT scan (HRCT of the chest has replaced bronchography as the initial investigation of choice in ABPA. HRCT of the chest can be normal in almost one-third of the patients, and at this stage it is referred to as serologic ABPA (ABPA-S. The importance of central bronchiectasis (CB as a specific finding in ABPA is debatable, as almost 40% of the lobes are involved by peripheral bronchiectasis. High-attenuation mucus (HAM, encountered in 20% of patients with ABPA, is pathognomonic of ABPA. ABPA should be classified based on the presence or absence of HAM as ABPA-S (mild, ABPA-CB (moderate, and ABPA-CB-HAM (severe, as this classification not only reflects immunological severity but also predicts the risk of recurrent relapses.

  14. Glutathion-S-Transferase P1 polymorphisms association with broncopulmonary dysplasia in preterm infants

    OpenAIRE

    Karagianni, P; Rallis, D; Fidani, L; Porpodi, M; Kalinderi, K; Tsakalidis, C.; Nikolaidis, N

    2013-01-01

    Background: Oxidative stress, characterized by the excretion of pre-oxidative and anti-oxidative proteases, has a key role in the pathogenesis of bronchopulmonary dysplasia (BPD). One of the many host anti-oxidant enzymes is glutathione-S-transferase P1 (GSTP1), with three polymorphic alleles having been identified: homozygous ile, heterozygous ile/val and homozygous val isomorph. The aim of this study was to examine the genetic predisposition to BPD in the GSTP1 polymorphisms.

  15. Fibromuscular dysplasia

    Directory of Open Access Journals (Sweden)

    Jeunemaitre Xavier

    2007-06-01

    Full Text Available Abstract Fibromuscular dysplasia (FMD, formerly called fibromuscular fibroplasia, is a group of nonatherosclerotic, noninflammatory arterial diseases that most commonly involve the renal and carotid arteries. The prevalence of symptomatic renal artery FMD is about 4/1000 and the prevalence of cervicocranial FMD is probably half that. Histological classification discriminates three main subtypes, intimal, medial and perimedial, which may be associated in a single patient. Angiographic classification includes the multifocal type, with multiple stenoses and the 'string-of-beads' appearance that is related to medial FMD, and tubular and focal types, which are not clearly related to specific histological lesions. Renovascular hypertension is the most common manifestation of renal artery FMD. Multifocal stenoses with the 'string-of-beads' appearance are observed at angiography in more than 80% of cases, mostly in women aged between 30 and 50 years; they generally involve the middle and distal two-thirds of the main renal artery and in some case also renal artery branches. Cervicocranial FMD can be complicated by dissection with headache, Horner's syndrome or stroke, or can be associated with intracerebral aneurysms with a risk of subarachnoid or intracerebral hemorrhage. The etiology of FMD is unknown, although various hormonal and mechanical factors have been suggested. Subclinical lesions are found at arterial sites distant from the stenotic arteries, and this suggests that FMD is a systemic arterial disease. It appears to be familial in 10% of cases. Noninvasive diagnostic tests include, in increasing order of accuracy, ultrasonography, magnetic resonance angiography and computed tomography angiography. The gold standard for diagnosing FMD is catheter angiography, but this invasive procedure is only used for patients in whom it is clinically pertinent to proceed with revascularization during the same procedure. Differential diagnosis include

  16. Allergic Bronchopulmonary Aspergillosis: A Perplexing Clinical Entity

    OpenAIRE

    Shah, Ashok; Panjabi, Chandramani

    2016-01-01

    In susceptible individuals, inhalation of Aspergillus spores can affect the respiratory tract in many ways. These spores get trapped in the viscid sputum of asthmatic subjects which triggers a cascade of inflammatory reactions that can result in Aspergillus-induced asthma, allergic bronchopulmonary aspergillosis (ABPA), and allergic Aspergillus sinusitis (AAS). An immunologically mediated disease, ABPA, occurs predominantly in patients with asthma and cystic fibrosis (CF). A set of criteria, ...

  17. Analysis of two cases with bronchopulmonary neurofibromatosis

    Directory of Open Access Journals (Sweden)

    Yuan Ting

    2012-07-01

    Full Text Available Abstract Neurogenic tumor of lung is very rare. Only few cases have been reported in the literature. We present here two cases of bronchopulmonary neurofibromatosis in two adults. In both cases, attempts at imaging failed to diagnose the case, and it was the histological study that ensured the diagnosis of neurofibromatosis. Biopsy specimens showed bundles of spindle-shaped cells mixed with collagen, and on immunohistochemistry some cells were positive for S-100 protein.

  18. Arrhythmogenic right ventricular dysplasia

    OpenAIRE

    Bockeria O.L.; Lе T.G.

    2015-01-01

    Arrhythmogenic right ventricular dysplasia is a hereditary cardiomyopathy characterized by structural and functional disorders in the right ventricle, which results in ventricular arrhythmias. Arrhythmogenic right ventricular dysplasia is one of the important causes of sudden cardiac death in young people and athletes. Structural disorders in arrhythmogenic right ventricular dysplasia are associated with fibrosis and fatty infiltration of the right ventricular myocardium. These changes lead t...

  19. The skeletal dysplasias

    National Research Council Canada - National Science Library

    Krakow, Deborah; Rimoin, David L

    2010-01-01

    The skeletal dysplasias (osteochondrodysplasias) are a heterogeneous group of more than 350 disorders frequently associated with orthopedic complications and varying degrees of dwarfism or short stature...

  20. Allergic bronchopulmonary mycosis caused by Penicillium luteum

    Directory of Open Access Journals (Sweden)

    Chiyako Oshikata

    2017-03-01

    Full Text Available A 65-year-old Japanese male had severe bronchial asthma had increased mold-containing sputum. Serum total IgE level had increased to 798 IU/mL and antigen-specific precipitating antibodies to P. luteum and P. notatum were present but not those reactive toward any species of Aspergillus. Chest computed tomography revealed central bronchiectasis and bronchial wall thickness. After antigen-specific provocation with 10 mg/mL of P. luteum, the patient developed asthma exacerbation, but not with A. fumigatus. We present a rare case of Penicillium-induced allergic bronchopulmonary mycosis caused by P. luteum.

  1. Fibrous dysplasia and cherubism

    National Research Council Canada - National Science Library

    Bhattacharya, Surajit; Mishra, R K

    2015-01-01

    ... and ultimately converting them into fibro-osseous cystic tissue. Cherubism is a hereditary form of fibrous dysplasia in which the causative factor is transmission of autosomal dominant SH3BP2 gene mutation...

  2. Septo-Optic Dysplasia

    Science.gov (United States)

    ... children with SOD have normal intelligence, others have learning disabilities. Most, however, are developmentally delayed due to vision impairment or neurological problems. × Definition Septo-optic dysplasia (SOD) is a rare disorder ...

  3. HIP DYSPLASIA IN TORNJAK

    Directory of Open Access Journals (Sweden)

    Hrvoje Milošević

    2012-07-01

    Full Text Available Canine hip dysplasia (CHD is a hereditary developmental anomaly, most frequent in large dog breeds. Clinical confirmation of the disorder is based on hip X-ray imaging. Twenty Tornjak dogs aged between 9-36 months, and weighted from 35-42 kg were examined for CHD. Scoring was performed according to six clearly defined radiographic parameters by Flueckiger method (5. Dysplastic changes of various severity were observed in 11 dogs, while in 9 dogs changes were absent. The study describes 4 CHD cases of varying degrees of severity. The results indicate the presence of CHD in Bosnian Tornjak. Determining the incidence of dysplasia in this autochthonous breed requires a more detailed study, which will enable determination of the prevalence of dysplasia and analysis of the relationship to other dog breeds. Key words: canine hip dysplasia, Bosnian Tornjak

  4. Mandibulo-acral dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Hoeffel, J.C.; Mainard, L. [Dept. of Radiology, Children' s Hospital, Vandoeuvre (France); Chastagner, P. [Dept. of Medicine, Children' s Hospital, Vandoeuvre (France); Hoeffel, C.C. [UFR Faculte de Medecine Cochin, Paris (France)

    2000-11-01

    We report on a 7 year-old-girl with mandibulo-acral dysplasia. When she was 3 years of age it mimicked scleroderma because of skin atrophy and later on a Hutchinson-Gilford progeria syndrome (HGP). Acro-mandibular dysplasia was diagnosed because of facial hypoplasia and mandibular hypoplasia. The bilateral proximal mid-humeral notch seen in this case is unusual. (orig.)

  5. Gracile bone dysplasias

    Energy Technology Data Exchange (ETDEWEB)

    Kozlowski, Kazimierz [Department of Medical Imaging, The Children' s Hospital at Westmead, Locked Bag 4001, Westmead 2145, NSW (Australia); Masel, John [Department of Radiology, Royal Children' s Hospital, Brisbane (Australia); Sillence, David O. [Department of Paediatrics and Child Health, The University of Sydney (Australia); Arbuckle, Susan [Department of Anatomical Pathology, The Children' s Hospital at Westmead, NSW (Australia); Juttnerova, Vera [Oddeleni Lekarske Genetiky, Hradec Kralove (Czech Republic)

    2002-09-01

    Gracile bone dysplasias constitute a group of disorders characterised by extremely slender bones with or without fractures. We report four newborns, two of whom showed multiple fractures. Two babies had osteocraniostenosis and one had features of oligohydramnios sequence. The diagnosis in the fourth newborn, which showed thin long bones and clavicles and extremely thin, poorly ossified ribs, is uncertain. Exact diagnosis of a gracile bone dysplasia is important for genetic counselling and medico-legal reasons. (orig.)

  6. APPLYING OF GAS ANALYSIS IN DIAGNOSIS OF BRONCHOPULMONARY DISEASES

    Directory of Open Access Journals (Sweden)

    Ye. B. Bukreyeva

    2014-01-01

    Full Text Available Bronchopulmonary system diseases are on the first place among the causes of people's death. Most of methods for lung diseases diagnosis are invasive or not suitable for children and patients with severe disease. One of the promising methods of clinical diagnosis and disease activity monitoring of bronchopulmonary system is analyzing of human breath. Directly exhaled breath or exhaled breath condensate are using for human breaths analyzing. Analysis of human breath can apply for diagnostic, long monitoring and evaluation of efficacy of the treatment bronchopulmonary diseases. Differential diagnostic between chronic obstructive lung disease (COPD and bronchial asthma is complicated because they have differences in pathogenesis. Analysis of human breath allows to explore features of COPD and bronchial asthma and to improve differential diagnostic of these diseases. Human breaths analyzing can apply for diagnostic dangerous diseases, such as tuberculosis, lung cancer. The analysis of breath air by spectroscopy methods is new noninvasive way for diagnosis of bronchopulmonary diseases.

  7. Anaesthetic management of bilateral alveolar proteinosis for bronchopulmonary lavage.

    Directory of Open Access Journals (Sweden)

    Dixit R

    1998-01-01

    Full Text Available The most hazardous manifestation of pulmonary alveolar proteinosis is progressive hypoxia for which bronchopulmonary lavage (BPL is the single most effective treatment. Unfortunately this procedure under general anesthesia itself increases the risk of hypoxia due to the need for one lung ventilation. It was therefore considered interesting to report the successful anaesthetic management of a patient with pulmonary alveolar proteinosis for Bronchopulmonary lavage.

  8. Genetics Home Reference: mandibuloacral dysplasia

    Science.gov (United States)

    ... a lack of fatty tissue under the skin (lipodystrophy) in certain regions of the body. The two ... of this disorder, mandibuloacral dysplasia with type A lipodystrophy (MADA) and mandibuloacral dysplasia with type B lipodystrophy ( ...

  9. TREATMENT OF HIP DYSPLASIA

    Directory of Open Access Journals (Sweden)

    Iulian ICLEANU

    2015-11-01

    Full Text Available In this thesis, our purpose is to show that using physiotherapy on patients with hip dysplasia from the very beginning, in the first months of life, helps treating them faster. Common literature proposes to use physiotherapy on patients with hip dysplasia either after their recovery or in the terminal phase of recovery, claiming that any earlier intervention will prolong the hip recovery. The effects of hip dysplasia reflect over the whole musculoskeletal system, while it hinders the knees (genu valgum, the ankles (ankle valgus, calcaneal valgus and the spine (scoliosis especially at the lumbar level. The most spectacular are at the hip level, that is why we made an analytical evaluation only for this joint. To show the importance of physiotherapy for children with hip dysplasia we started from the hypothesis: untimely treatment for children with hip dysplasia has improved results in functional recovery and in obtaining a better stability, without the necessity of orthopedics or surgical interventions. The research methods used in this study are: the observation method, the bibliographic study method, the experimental method, the graphics method and the statistical mathematical method to process the data and to represent the results graphically. In the end, the results obtained are significantly different from the initial evaluations and we came to the conclusion that starting an untimely analytical kinetic treatment and globally personalizing it to every patient improves stability and biomechanical parameters for the hip.

  10. Histiocytosis X and Bronchopulmonary Adenocarcinoma: A Rare Coexistence

    Directory of Open Access Journals (Sweden)

    Akýn Kaya

    2002-01-01

    Full Text Available There exists a rarely observed association between pulmonary histiocytosis X and bronchopulmonary cancer. However, the frequency of bronchopulmonary cancer in these patients is higher than in the general population. A 28-year-old patient who currently smokes ten packs of cigarettes a year came to our department of pneumology with complains of cough and hemoptysis. An x-ray of the thorax revealed bilateral cysts and a shadow in the upper part of the right pulmonary field. In addition, a chest tomography showed multiple cysts dispersed throughout the two pulmonary fields and an irregular mass with a diameter of four centimetres in the upper right lobe. Bronchopulmonary adenocarcinoma was diagnosed during a cytologic exam of the bronchial washing. We decided to perform a thoracotomy on the patient, since there was no far metastasis. An upper lobectomy and wedge resection of the upper segment of the lower right lobe, which had been invaded by the tumour, were performed. Histology confirmed the diagnosis of adenocarcinoma. A pulmonary biopsy was carried out on the tumour-free site and showed the presence of histiocytosis X. There is a hypothesis that a neoplasm developed on the pulmonary fibrosis could be an epiphenomenon of bronchopulmonary cancer in patients who smoke and have pulmonary histiocytosis X. It is interesting to note that histiocytosis X and bronchopulmonary cancer were diagnosed at the same time, since the bronchopulmonary cancer may have occurred within a few years following the diagnosis of histiocytosis X, even if she was a smoker. Hemoptysis, which is found in 5% of patients with histiocytosis X, may suggest cancer. This young patient, a smoker, who complained of hemoptysis, is a particularly rare case of the association between pulmonary histiocytosis X and bronchopulmonary cancer whose pathogenesis is not clear cut. It is thus important to note that smoking can have major consequences, even in young people.

  11. [Bronchopulmonary pathology with hypereosinophilia of fungal origin (excluding allergic bronchopulmonary aspergillosis)].

    Science.gov (United States)

    Lahoute, C; Tonnel, A B; Fournier, E; Ramon, P; Voisin, C

    1983-01-01

    Five cases of eosinophil lung are reported in which the fungus responsible for the affection was not Aspergillus. Documented data include reports on 19 similar cases with a clinical picture suggestive of allergic bronchopulmonary aspergillosis but with negative tests for Aspergillus. The various fungal species isolated included Candida albicans, Penicillium, Geotrichum candidum, Stemphylium lanuginosum, Culvularia lunata, and Drechsleria hawaïensis. Diagnostic criteria are discussed, with particular emphasis on the importance of the inhalation provocation test, as well as possible efficacy of antifungal treatment.

  12. Genetics Home Reference: cleidocranial dysplasia

    Science.gov (United States)

    ... Oct 24. Citation on PubMed OMIM: CLEIDOCRANIAL DYSPLASIA Cohen MM Jr. Biology of RUNX2 and Cleidocranial Dysplasia. J Craniofac Surg. 2013 Jan;24(1):130-3. doi: 10.1097/SCS.0b013e3182636b7e. Review. Citation on PubMed D'Alessandro G, Tagariello T, Piana G. Cleidocranial dysplasia: etiology and ...

  13. Nasal CPAP and surfactant for treatment of respiratory distress syndrome and prevention of bronchopulmonary dysplasia

    DEFF Research Database (Denmark)

    Verder, Henrik; Bohlin, Kajsa; Kamper, Jens

    2009-01-01

    CPAP) and surfactant treatment. The approach may be supplemented with caffeine citrate and non-invasive positive pressure ventilation for apnoea. The low incidence of BPD seen as a consequence of the treatment strategy is mainly due to a reduced need for mechanical ventilation (MV). Conclusion: Early...

  14. Glucocorticoid Induced Cerebellar Toxicity in the Developing Neonate: Implications for Glucocorticoid Therapy during Bronchopulmonary Dysplasia

    Directory of Open Access Journals (Sweden)

    Kevin K. Noguchi

    2014-01-01

    Full Text Available Prematurely born infants commonly suffer respiratory dysfunction due to the immature state of their lungs. As a result, clinicians often administer glucocorticoid (GC therapy to accelerate lung maturation and reduce inflammation. Unfortunately, several studies have found GC therapy can also produce neuromotor/cognitive deficits and selectively stunt the cerebellum. However, despite its continued use, relatively little is known about how exposure to this hormone might produce neurodevelopmental deficits. In this review, we use rodent and human research to provide evidence that GC therapy may disrupt cerebellar development through the rapid induction of apoptosis in the cerebellar external granule layer (EGL. The EGL is a transient proliferative region responsible for the production of over 90% of the neurons in the cerebellum. During normal development, endogenous GC stimulation is thought to selectively signal the elimination of the EGL once production of new neurons is complete. As a result, GC therapy may precociously eliminate the EGL before it can produce enough neurons for normal cerebellar function. It is hoped that this review may provide information for future clinical research in addition to translational guidance for the safer use of GC therapy.

  15. Bronchopulmonary dysplasia and perinatal glucocorticoids in preterm infants: changing practice based on evidence

    NARCIS (Netherlands)

    Onland, W.

    2011-01-01

    Te vroeg geboren kinderen hebben een verhoogd risico op longproblemen (bronchopulmonale dysplasie). Het medicijn dexamethason is gunstig voor de longen, maar verhoogt de kans op neurologische schade. Om nadelige effecten te verminderen, begint men later met deze therapie, is de dosis verlaagd en de

  16. Genetic Variants of Surfactant Proteins A, B, C, and D in Bronchopulmonary Dysplasia

    Directory of Open Access Journals (Sweden)

    J. Pavlovic

    2006-01-01

    Full Text Available BPD_28D (O2 dependency at 28 days of life and BPD_36W (O2 dependency at 36 wks post-menstrual age are diseases of prematurely born infants exposed to mechanical ventilation and/or oxygen supplementation. In order to determine whether genetic variants of surfactant proteins (SPs-A, B, C, and D and SP-B-linked microsatellite markers are risk factors in BPD, we performed a family based association study using a Greek study group of 71 neonates (<30 wks gestational age from 60 families with, 52 BPD_28D and 19 BPD_36W, affected infants. Genotyping was performed using newly designed pyrosequencing assays and previously published methods. Associations between genetic variants of SPs and BPD subgroups were determined using Transmission Disequilibrium Test (TDT and Family Based Association Test (FBAT. Significant associations (p ≤ 0.01 were observed for alleles of SP-B and SP-B-linked microsatellite markers, and haplotypes of SP-A, SP-D, and SP-B. Specifically, allele B-18_C associated with susceptibility in BPD_36W. Microsatellite marker AAGG_6 associated with susceptibility in BPD_28D/36W group. Haplotype analysis revealed ten susceptibility and one protective haplotypes for SP-B and SP-B-linked microsatellite markers and two SP-A-SP-D protective haplotypes. The data indicate that SP loci are linked to BPD. Studies in different study groups and/or of larger sample size are warranted to confirm these observations and delineate genetic background of BPD subgroups.

  17. The efficacy and safety of Montelukast sodium in the prevention of bronchopulmonary dysplasia.

    Science.gov (United States)

    Kim, Sang Bum; Lee, Jang Hoon; Lee, Juyoung; Shin, Seung Han; Eun, Ho Sun; Lee, Soon Min; Sohn, Jin A; Kim, Han Suk; Choi, Byung Min; Park, Min Soo; Park, Kook In; Namgung, Ran; Park, Moon Sung

    2015-09-01

    The purpose of this study was to evaluate the efficacy and safety of Montelukast sodium in the prevention of bronchopulmonarydysplasia (BPD). The Interventional study was designed as a multicenter, prospective, and randomized trial, with open labeled and parallel-experimental groups, 66 infants were enrolled and allocated to either the case group (n=30) or the control group (n=36) based on gestational age (GA). Infants in the case group were given Montelukast sodium (Singulair) based on their body weight (BW). Zero week was defined as the start time of the study. The incidence of moderate to severe BPD was not different between the groups (case group: 13 of 30 [43.3%] vs. control group: 19 of 36 [52.8%], P=0.912). Additionally, secondary outcomes such as ventilation index, mean airway pressure and resort to systemic steroids were not significantly different. There were no serious adverse drug reactions in either group, and furthermore the rate of occurrence of mild drug related-events were not significantly different (case group: 10 of 42 [23.8%] vs. control group: 6 of 48 (15.8%), P=0.414). Montelukast was not effective in reducing moderate or severe BPD. There were no significant adverse drug events associated with Montelukast treatment.

  18. Immunopathology and Immunogenetics of Allergic Bronchopulmonary Aspergillosis

    Directory of Open Access Journals (Sweden)

    Alan P. Knutsen

    2011-01-01

    Full Text Available Allergic bronchopulmonary aspergillosis (ABPA is a Th2 hypersensitivity lung disease in response to Aspergillus fumigatus that affects asthmatic and cystic fibrosis (CF patients. Sensitization to A. fumigatus is common in both atopic asthmatic and CF patients, yet only 1%–2% of asthmatic and 7%–9% of CF patients develop ABPA. ABPA is characterized by wheezing and pulmonary infiltrates which may lead to pulmonary fibrosis and/or bronchiectasis. The inflammatory response is characterized by Th2 responses to Aspergillus allergens, increased serum IgE, and eosinophilia. A number of genetic risks have recently been identified in the development of ABPA. These include HLA-DR and HLA-DQ, IL-4 receptor alpha chain (IL-4RA polymorphisms, IL-10 −1082GA promoter polymorphisms, surfactant protein A2 (SP-A2 polymorphisms, and cystic fibrosis transmembrane conductance regulator gene (CFTR mutations. The studies indicate that ABPA patients are genetically at risk to develop skewed and heightened Th2 responses to A. fumigatus antigens. These genetic risk studies and their consequences of elevated biologic markers may aid in identifying asthmatic and CF patients who are at risk to the development of ABPA. Furthermore, these studies suggest that immune modulation with medications such as anti-IgE, anti-IL-4, and/or IL-13 monoclonal antibodies may be helpful in the treatment of ABPA.

  19. Cervical dysplasia - slideshow

    Science.gov (United States)

    ... GO GO About MedlinePlus Site Map FAQs Customer Support Health Topics Drugs & Supplements Videos & Tools Español You Are Here: Home → Medical Encyclopedia → Cervical dysplasia - series—Normal anatomy URL of this page: //medlineplus.gov/ency/presentations/ ...

  20. Intraerythrocyte Non-Protein-Bound Iron in Children with Bronchopulmonary Pathology

    Directory of Open Access Journals (Sweden)

    E.M. Vasilyeva

    2014-12-01

    Full Text Available A total of 230 children having bronchopulmonary pathology (BPP were examined. Patients were divided into 4 groups according to their intraerythrocyte non-protein- bound iron (IE-NPBI levels. We investigated the relationship of the IE-NPBI level with parameters of respiratory function (RF tests, the severity of comorbidities, and level of other free intracellular ions, such as copper, zinc, and magnesium. The pronounced increase in IE-NPBI level was typical for patients with the connective tissue dysplasia, often accompanied by mitral valve prolapse, osteopenia, and mineral metabolism violation. The severe comorbid diagnoses were typical for patients with reduced levels of IE-NPBI (chronic cor pulmonale, tuberculosis infection. The largest number of comorbidities, aggravating the underlying disease, took place in the group of patients with a significant reduction in IE-NPBI level. A significant increase in IE-NPBI level, as well as a marked reduction of IE-NPBI level, was an unfavorable factor for the underlying disease. We found a correlation between IE-NPBI level and parameters of RF-test in patients with moderate increase in IE-NPBI level.

  1. Lumbar-sacral dysplasias

    Energy Technology Data Exchange (ETDEWEB)

    Schumacher, M.; Thron, A.

    1984-09-01

    By means of some selected examples, the myelographic and CT characteristics are presentated of different lumbar-sacral dysplasias. The advantage of the different methods of examination (CT, CT myelography and myelography) and the improved presentation of pathological-anatomical details by means of a combination of these methods in the diagnosis of hyperplasia of the filum terminale, diastematomyelia, tethered conus, intracorporal and anterior sacral meningocele have been shown.

  2. Canine Hip Dysplasia: Breed Effects

    OpenAIRE

    Martin, S W; Kirby, K.; Pennock, P. W.

    1980-01-01

    This paper is a refinement of previous studies in that only suitably radiographed dogs were included in the data base. The rate of hip dysplasia varied widely by breed from five percent in siberian huskies to eighty-three percent in english bulldogs. There was a significant difference in the prevalence of dysplasia within at least two breeds; golden retrievers and old english sheepdogs. Physical size per se did not appear to be an important determinant of hip dysplasia.

  3. Bronchopulmonary sequestration: Improving practice by evaluating for a missed diagnosis.

    Science.gov (United States)

    Estes, Mary Ellen Zator

    2017-06-16

    Bronchopulmonary sequestration (BPS) is a lung mass that does not communicate with the tracheobronchial tree or the pulmonary arterial vasculature, and thus does not play a role in oxygenation. This article discusses the etiology of BPS, as well as its pathophysiology, signs and symptoms, imaging studies used to diagnose, and treatment options in both pediatric and adult patients.

  4. Allergic bronchopulmonary aspergillosis as a cause of bronchial ...

    African Journals Online (AJOL)

    Background: Allergic bronchopulmonary aspergillosis (ABPA) occurs in patients with asthma and cystic fibrosis. When aspergillus fumigatus spores are inhaled they grow in bronchial mucous as hyphae. It occurs in non immunocompromised patients and belongs to the hypersensitivity disorders induced by Aspergillus.

  5. Diagnosis of allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis

    DEFF Research Database (Denmark)

    Skov, M; Koch, C; Reimert, C M

    2000-01-01

    The diagnosis of allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF) patients may be difficult to establish because ABPA shares many characteristics with coexisting atopy or other lung infections in these patients. This study aimed to evaluate the sensitivity and specificity...

  6. Genetics Home Reference: Kniest dysplasia

    Science.gov (United States)

    ... Resources (1 link) National Institute of Arthritis and Musculoskeletal and Skin Diseases: Heritable Disorders of Connective ... Skeletal Dysplasia Registry, UCLA Little People of America Little ...

  7. Genetics Home Reference: Czech dysplasia

    Science.gov (United States)

    ... of Arthritis and Musculoskeletal and Skin Diseases: Heritable Disorders of Connective Tissue National Institute of ... International Skeletal Dysplasia Registry, UCLA Resource list from the University ...

  8. Familial ectodermal dysplasia: a peers’ agony

    Science.gov (United States)

    Hegde, Karthik; Kashyap, Roopashri Rajesh; Nair, Gopakumar; Nair, Preeti P

    2013-01-01

    Ectodermal dysplasias include a various group of inherited disorders which share primary defect in the development of two or more tissues of embryonic ectodermal origin. Though there are many subtypes, ectodermal dysplasias are mainly hidrotic ectodermal dysplasia and hypohidrotic ectodermal dysplasia, among which the most common variety is X linked hypohidrotic ectodermal dysplasia. We report a rare case of X linked hypohidrotic ectodermal dysplasia occurring in a family with various skin, hair and oral abnormalities. PMID:23880572

  9. Familial ectodermal dysplasia: a peers' agony.

    Science.gov (United States)

    Hegde, Karthik; Kashyap, Roopashri Rajesh; Nair, Gopakumar; Nair, Preeti P

    2013-07-23

    Ectodermal dysplasias include a various group of inherited disorders which share primary defect in the development of two or more tissues of embryonic ectodermal origin. Though there are many subtypes, ectodermal dysplasias are mainly hidrotic ectodermal dysplasia and hypohidrotic ectodermal dysplasia, among which the most common variety is X linked hypohidrotic ectodermal dysplasia. We report a rare case of X linked hypohidrotic ectodermal dysplasia occurring in a family with various skin, hair and oral abnormalities.

  10. Cloverleaf skull with generalised bone dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Kozlowski, K.; Warren, P.S.; Fisher, C.C.

    1985-09-01

    A case of cloverleaf skull with generalised bone dysplasia is reported. The authors believe that bone dysplasia associated with cloverleaf is neither identical with thanatophoric dysplasia nor achondroplasia. Until identity of thanatophoric dysplasia and cloverleaf skull with generalised bone dysplasia is proved the diseases should be looked upon as separate entities and the wording ''thanatophoric dysplasia with cloverleaf skull'' should be abolished.

  11. Dysplasia in Ulcerative Colitis

    OpenAIRE

    RH Riddell

    1990-01-01

    Patients at highest risk for developing cancer in ulcerative colitis are those with ‘extensive’ or total involvement of the large bowel who have had the disease for at least seven years. Dysplasia is used as a marker bur has many problems including those of sampling, reproducibility and management. The risk in patients with colitis is unclear particularly in those with left-sided or distal ulcerative colitis. In countries at high risk from colorectal cancer about 4 to 6% of the population can...

  12. Childhood allergic bronchopulmonary aspergillosis presenting as a middle lobe syndrome

    OpenAIRE

    Shah, Ashok; Gera, Kamal; Panjabi, Chandramani

    2016-01-01

    Allergic bronchopulmonary aspergillosis (ABPA) is infrequently documented in children with asthma. Although collapse is not uncommon, middle lobe syndrome (MLS) as a presentation of ABPA is rather a rarity. A 9-year-old female child with asthma presented with increase in intensity of symptoms along with a right midzone patchy consolidation on a chest radiograph. In addition, an ill-defined opacity abutting the right cardiac border with loss of cardiac silhouette was noted. A right lateral vie...

  13. Increase in bronchopulmonary infection due to branhamella catarrhalis.

    OpenAIRE

    McLeod, D T; Ahmad, F; Capewell, S.; Croughan, M J; Calder, M A; Seaton, A

    1986-01-01

    In a six month prospective study during the winter Branhamella catarrhalis was isolated from the sputum of 63 patients with symptoms of bronchopulmonary infection: 49 isolates were in pure culture and 14 were with another pathogen, Haemophilus influenzae being the commonest (found with 10 of the 14 B catarrhalis isolates). Of 36 patients infected in the community, 26 required admission to hospital. The remaining 27 patients were infected while in hospital. Forty four of the 63 isolates produc...

  14. Fibrous dysplasia and cherubism

    Directory of Open Access Journals (Sweden)

    Surajit Bhattacharya

    2015-01-01

    Full Text Available Fibrous dysplasia (FD is a non-malignant fibro-osseous bony lesion in which the involved bone/bones gradually get converted into expanding cystic and fibrous tissue. The underlying defect in FD is post-natal mutation of GNAS1 gene, which leads to the proliferation and activation of undifferentiated mesenchymal cells arresting the bone development in woven phase and ultimately converting them into fibro-osseous cystic tissue. Cherubism is a hereditary form of fibrous dysplasia in which the causative factor is transmission of autosomal dominant SH3BP2 gene mutation. The disease may present in two distinct forms, a less severe and limited monostotic form, and a more aggressive and more widespread polyostotic form. Polyostotic form may be associated with various endocrine abnormalities, which require active management apart from the management of FD. Management of FD is not free from controversies. While total surgical excision of the involved area and reconstruction using newer micro-vascular technique is the only definitive treatment available from the curative point of view, but this can be only offered to monostotic and very few polyostotic lesions. In polyostotic varieties on many occasions these radical surgeries are very deforming in these slow growing lesions and so their indication is highly debated. The treatment of cranio-facial fibrous dysplasia should be highly individualized, depending on the fact that the clinical behavior of lesion is variable at various ages and in individual patients. A more conservative approach in the form of aesthetic recontouring of deformed bone, orthodontic occlusal correction, and watchful expectancy may be the more accepted form of treatment in young patients. Newer generation real-time imaging guidance during recontouring surgery adds to accuracy and safety of these procedures. Regular clinical and radiological follow up is required to watch for quiescence, regression or reactivation of the disease process

  15. Fibrous dysplasia and cherubism

    Science.gov (United States)

    Bhattacharya, Surajit; Mishra, RK

    2015-01-01

    Fibrous dysplasia (FD) is a non-malignant fibro-osseous bony lesion in which the involved bone/bones gradually get converted into expanding cystic and fibrous tissue. The underlying defect in FD is post-natal mutation of GNAS1 gene, which leads to the proliferation and activation of undifferentiated mesenchymal cells arresting the bone development in woven phase and ultimately converting them into fibro-osseous cystic tissue. Cherubism is a hereditary form of fibrous dysplasia in which the causative factor is transmission of autosomal dominant SH3BP2 gene mutation. The disease may present in two distinct forms, a less severe and limited monostotic form, and a more aggressive and more widespread polyostotic form. Polyostotic form may be associated with various endocrine abnormalities, which require active management apart from the management of FD. Management of FD is not free from controversies. While total surgical excision of the involved area and reconstruction using newer micro-vascular technique is the only definitive treatment available from the curative point of view, but this can be only offered to monostotic and very few polyostotic lesions. In polyostotic varieties on many occasions these radical surgeries are very deforming in these slow growing lesions and so their indication is highly debated. The treatment of cranio-facial fibrous dysplasia should be highly individualized, depending on the fact that the clinical behavior of lesion is variable at various ages and in individual patients. A more conservative approach in the form of aesthetic recontouring of deformed bone, orthodontic occlusal correction, and watchful expectancy may be the more accepted form of treatment in young patients. Newer generation real-time imaging guidance during recontouring surgery adds to accuracy and safety of these procedures. Regular clinical and radiological follow up is required to watch for quiescence, regression or reactivation of the disease process. Patients must be

  16. Evaluation of Ectodermal Dysplasia

    Directory of Open Access Journals (Sweden)

    Zelal Baskan

    2006-04-01

    Full Text Available This case series report outlines possible cranio-maxillofacial deformation consequences associated with ectodermal dysplasia (ED and embryonic malformations, including dental agenesis. Also described are the oral aspects and rehabilitation. A total of 14 ED patients (7 males and 7 females, aged 5-45 years underwent clinical examination before assessment and treatment. Lateral cephalometric radiography, Steiner's analysis, and respiratory capacity tests were performed. Most of the patients had sparse or absent hair, a short face with an unusual facial concavity, a maxillary retrusion, and a relative mandible protrusion. Depending on age and orthopedic abnormalities, patients were treated with prosthodontic and orthodontic approaches or implant treatment. Therapists should take a comprehensive and multidisciplinary approach with these patients to improve their dental, masticatory, growth, and orthognathic conditions, as well as esthetic appearance.

  17. Arrhythmogenic right ventricular dysplasia.

    Science.gov (United States)

    Kinsara, A J; Zaman, L; Gorgels, A

    2001-01-01

    Right ventricular dysplasia (RVD) is a disease entity of unknown cause that is characterised by partial or total replacement of RV-muscle by adipose or fibrous tissue. It is a well-recognized cause of arrhythmia and premature sudden death, but usually underdiagnosed. Several noninvasive and invasive diagnostic modalities have been used, however, all may not be positive in a given case. Drug therapy with class 1c, beta-blocker, and amiodarone in variable combination produce varying success rates in preventing recurrent ventricular tachycardia. Failure of the above measures calls for insertion of implantable cardioverter defibrillator. The attention of emergency physicians is drown to this disease as they are the first medical personnel to be presented with this disease as an emergency. Hence their recognition of RVD will ensure early and proper management.

  18. Genetics Home Reference: frontonasal dysplasia

    Science.gov (United States)

    ... 2 Genetic Testing Registry: Frontonasal dysplasia 3 Other Diagnosis and Management Resources (4 links) KidsHealth from Nemours: Cleft Lip and Palate MedlinePlus Encyclopedia: Head and Face Reconstruction Mount Sinai ...

  19. Oral epithelial dysplasia classification systems

    DEFF Research Database (Denmark)

    Warnakulasuriya, S; Reibel, J; Bouquot, J

    2008-01-01

    At a workshop coordinated by the WHO Collaborating Centre for Oral Cancer and Precancer in the United Kingdom issues related to potentially malignant disorders of the oral cavity were discussed by an expert group. The consensus views of the Working Group are presented in a series of papers....... In this report, we review the oral epithelial dysplasia classification systems. The three classification schemes [oral epithelial dysplasia scoring system, squamous intraepithelial neoplasia and Ljubljana classification] were presented and the Working Group recommended epithelial dysplasia grading for routine...... use. Although most oral pathologists possibly recognize and accept the criteria for grading epithelial dysplasia, firstly based on architectural features and then of cytology, there is great variability in their interpretation of the presence, degree and significance of the individual criteria...

  20. Cleidocranial dysplasia: A case report

    Directory of Open Access Journals (Sweden)

    Hemalatha R

    2008-03-01

    Full Text Available Cleidocranial dysplasias is an autosomal dominant disorder that presents with skeletal dysplasia. The dental manifestations are mainly delayed exfoliation of primary teeth and delayed eruption of permanent teeth, with multiple impacted supernumeraries. This report addresses the complex nature of the treatment modalities. In our patient, surgical exposure of unerupted teeth was done with orthodontic traction. Post-surgical follow-up was uneventful.

  1. Pediatric aspects of skeletal dysplasia.

    Science.gov (United States)

    Ozono, Keiichi; Namba, Noriyuki; Kubota, Takuo; Kitaoka, Taichi; Miura, Kohji; Ohata, Yasuhisa; Fujiwara, Makoto; Miyoshi, Yoko; Michigami, Toshimi

    2012-10-01

    Skeletal dysplasia is a disorder of skeletal development characterized by abnormality in shape, length, a number and mineral density of the bone. Skeletal dysplasia is often associated with manifestation of other organs such as lung, brain and sensory systems. Skeletal dysplasias or dysostosis are classified with more than 400 different names. Enchondral bone formation is a coordinated event of chondrocyte proliferation, differentiation and exchange of terminally maturated chondrocyte with bone. Impaired enchondral bone formation will lead to skeletal dysplasia, especially associated with short long bones. Appropriate bone volume and mineral density are achieved by balance of bone formation and bone resorption and mineralization. The gene encoding fibroblast growth factor receptor 3 is responsible for achondroplasia, representative skeletal dysplasia with short stature. The treatment with growth hormone is approved for achondroplasia in Japan. Osteogenesis imperfecta is characterized by low bone mineral density and fragile bone. Data on the beneficial effect of bisphosphonate for osteogenesis imperfecta are accumulating. Osteopetrosis has high bone mineral density, but sometimes show bone fragility. In Japan as well as other countries, pediatrician treat larger numbers of patients with skeletal dysplasia with short stature and fragile bones compared to 20 years ago.

  2. Progressive pseudorheumatoid dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Mampaey, S.; De Schepper, A. [Dept. of Radiology, University Hospital Antwerp, Edegem (Belgium); Vanhoenacker, F. [Dept. of Radiology, University Hospital Antwerp, Edegem (Belgium); Dept. of Radiology, St. Maarten Hospital, Duffel (Belgium); Boven, K. [Dept. of Pediatrics, University Hospital Antwerp, Edegem (Belgium); Hul, W. van [Dept. of Medical Genetics, University of Antwerp (Belgium)

    2000-11-01

    A rare case of progressive pseudorheumatoid dysplasia (PPD) in a 9-year-old girl is presented. Clinically, chronic painless swollen joints, accompanied by progressive motion restriction and progressive walking difficulties, were found. Radiologically, there was enlargement of the epimetaphyseal portions of the large joints, metacarpal heads, and phalanges, and generalized platyspondyly with irregular delineation of the endplates of the vertebral bodies. The radioclinical features at the peripheral joints were originally misdiagnosed as juvenile rheumatoid arthritis (JRA), and the structural spinal abnormalities were neglected and interpreted as Scheuermann's disease. However, the absence of active inflammatory parameters argues against JRA, whereas the low age of onset of the irregularities at the vertebral endplates is an argument against the diagnosis of Scheuermann's disease. The combination of the dysplastic abnormalities of the spine, with platyspondyly and Scheuermann-like lesions at an unusually low age of onset, and radiological features mimicking JRA of the peripheral joints, is the clue to the diagnosis of this rare autosomal-recessive disease. This case is the first to document the MRI features of PPD of the spine. (orig.)

  3. An Unusual Left Upper Quadrant Mass: A Bronchopulmonary Foregut Malformation

    Directory of Open Access Journals (Sweden)

    R. L. McDermott

    2013-01-01

    Full Text Available We report a case of a lady who presented with epigastric discomfort. Physical examination revealed a large left upper quadrant mass filling the left upper quadrant. Following extensive preoperative evaluation, she underwent resection of this centimeter mass with en bloc excision of a portion of the left hemidiaphragm. She made an uneventful postoperative recovery. Histopathology revealed a bronchopulmonary foregut malformation with pulmonary sequestration. This developmental anomaly of the foregut typically occurs in the thoracic cavity; however, it can occur below the diaphragm. Herein we report a case and a detailed review of the embryology, clinical features, and management of these extremely rare clinical entities.

  4. Esophageal lung – A rare bronchopulmonary foregut malformation

    Directory of Open Access Journals (Sweden)

    S.V. Parelkar

    2014-11-01

    Full Text Available Esophageal lung is a rare variety of communicating bronchopulmonary foregut malformation characterized by a fistula between an isolated portion of respiratory tissue and esophagus or stomach. It may involve the entire lung or one of the pulmonary lobes. Only 20 cases have been reviewed in 2011. Fifty percent of cases are associated with a tracheoesophageal fistula. We report a case of a 6 month old girl who was previously operated for TEF repair, with esophageal lobe which was successfully excised. The relevant literature is reviewed.

  5. Schimke immuno-osseous dysplasia: two cases

    Energy Technology Data Exchange (ETDEWEB)

    Tylki-Szymanska, Anna; Rokicki, Dariusz [Department of Metabolic Diseases, The Children' s Memorial Health Institute, Al. Dzieci Polskich 20, 04730, Warsaw (Poland); Pyrkosz, Antoni [Department of Genetics, Silesian Medical Academy, Katowice (Poland); Krajewska-Walasek, Malgorzata [Department of Genetics, The Children' s Memorial Health Institute, Warsaw (Poland); Michalkiewicz, Jacek [Department of Immunology, The Children' s Memorial Health Institute, Warsaw (Poland); Kowalska, Aleksandra [Department of Radiology, The Children' s Memorial Health Institute, Warsaw (Poland)

    2003-03-01

    We report two patients with Schimke immuno-osseous dysplasia (SIOD). SIOD is characterised by growth retardation, renal failure, spondylo-epiphyseal dysplasia, specific phenotype and defective cellular immunity. These two children demonstrated a bone dysplasia with characteristic radiographic appearances. We postulate that SIOD should be considered in all cases of growth failure with an unclassifiable bone dysplasia. Repeated urine tests for proteinuria could be helpful in reaching the correct diagnosis. (orig.)

  6. Prenatal diagnosis of asphyxiating thoracic dysplasia.

    Science.gov (United States)

    Lipson, M; Waskey, J; Rice, J; Adomian, G; Lachman, R; Filly, R; Rimoin, D

    1984-06-01

    A fetus at risk for asphyxiating thoracic dysplasia was studied with ultrasound examination at 16, 18, and 23 weeks of pregnancy. Definite shortness of fetal femora was observed. Radiographic and histologic examinations after pregnancy termination confirmed the diagnosis. Asphyxiating thoracic dysplasia appears to be one of an increasing number of skeletal dysplasias that can be diagnosed prenatally with ultrasound.

  7. Chondroectodermal dysplasia: a rare syndrome.

    Directory of Open Access Journals (Sweden)

    Dana Tahririan

    2014-06-01

    Full Text Available Chondroectodermal dysplasia (Ellis-Van Creveld syndrome is a rare autosomal recessive congenital abnormality. This syndrome is characterized by a spectrum of clinical findings, among which chondrodystrophy, polydactyly, ectodermal dysplasia, and congenital cardiac anomalies are the most common. It is imperative to not overlook the cardiac complications in patients with this syndrome during dental procedures. The case presented here, although quite rare, was detected under normal conditions and can be alarming for dental care providers. Clinical reports outline the classical and unusual oral and dental manifestations, which help health care providers diagnose chondroectodermal dysplasia, and refer patients with this syndrome to appropriate health care professionals to receive treatment to prevent further cardiac complications and bone deformities.

  8. Florid osseous dysplasia in Orientals.

    Science.gov (United States)

    Loh, F C; Yeo, J F

    1989-12-01

    Florid osseous dysplasia has been described as a condition that characteristically affects the jaws of middle-aged black women. It usually manifests as multiple radiopaque cementum-like masses distributed throughout the jaws. This condition has also been classified by various authors as sclerosing osteomyelitis, sclerosing osteitis, sclerotic cemental masses, gigantiform cementoma, and various other terms. In this series, we want to document eight cases of florid osseous dysplasia in middle-aged, female ethnic Chinese and one case in an Indian subject on the basis of radiographic and histopathologic findings. On the basis of this series, incidence is estimated to be 0.01 case per year 100,000 population. Florid osseous dysplasia constitutes 11% of cemental lesions in our files.

  9. Allergic bronchopulmonary aspergillosis: a rare cause of pleural effusion.

    LENUS (Irish Health Repository)

    O'Connor, T M

    2012-02-03

    Aspergillus fumigatus is one of the most ubiquitous of the airborne saprophytic fungi. Allergic bronchopulmonary aspergillosis (ABPA) is a syndrome seen in patients with asthma and cystic fibrosis, and is characterized by hypersensitivity to chronic colonization of the airways with A. fumigatus. We report the case of a patient with ABPA presenting with pleural effusion. A 27-year-old male was referred with recurrent right pleural effusion. Past medical history was remarkable for asthma, allergic sinusitis, and recurrent pleurisy. Investigations revealed peripheral eosinophilia with elevated serum immunoglobulin E and bilateral pleural effusions with bilateral upper lobe proximal bronchiectasis. Precipitating serum antibodies to A. fumigatus were positive and the A. fumigatus immediate skin test yielded a positive reaction. A diagnosis of ABPA associated with bilateral pleural effusions was made and the patient was commenced on prednisolone. At review, the patient\\'s symptoms had considerably improved and his pleural effusions had resolved. ABPA may present with diverse atypical syndromes, including paratracheal and hilar adenopathy, obstructive lung collapse, pneumothorax and bronchopleural fistula, and allergic sinusitis. Allergic bronchopulmonary aspergillosis is a rare cause of pleural effusion and must be considered in the differential diagnosis of patients presenting with a pleural effusion, in particular those with a history of asthma.

  10. Mutations in LTBP3 cause acromicric dysplasia and geleophysic dysplasia.

    Science.gov (United States)

    McInerney-Leo, Aideen M; Le Goff, Carine; Leo, Paul J; Kenna, Tony J; Keith, Patricia; Harris, Jessica E; Steer, Ruth; Bole-Feysot, Christine; Nitschke, Patrick; Kielty, Cay; Brown, Matthew A; Zankl, Andreas; Duncan, Emma L; Cormier-Daire, Valerie

    2016-07-01

    Acromelic dysplasias are a group of disorders characterised by short stature, brachydactyly, limited joint extension and thickened skin and comprises acromicric dysplasia (AD), geleophysic dysplasia (GD), Myhre syndrome and Weill-Marchesani syndrome. Mutations in several genes have been identified for these disorders (including latent transforming growth factor β (TGF-β)-binding protein-2 (LTBP2), ADAMTS10, ADAMSTS17 and fibrillin-1 (FBN1) for Weill-Marchesani syndrome, ADAMTSL2 for recessive GD and FBN1 for AD and dominant GD), encoding proteins involved in the microfibrillar network. However, not all cases have mutations in these genes. Individuals negative for mutations in known acromelic dysplasia genes underwent whole exome sequencing. A heterozygous missense mutation (exon 14: c.2087C>G: p.Ser696Cys) in latent transforming growth factor β (TGF-β)-binding protein-3 (LTBP3) was identified in a dominant AD family. Two distinct de novo heterozygous LTPB3 mutations were also identified in two unrelated GD individuals who had died in early childhood from respiratory failure-a donor splice site mutation (exon 12 c.1846+5G>A) and a stop-loss mutation (exon 28: c.3912A>T: p.1304*Cysext*12). The constellation of features in these AD and GD cases, including postnatal growth retardation of long bones and lung involvement, is reminiscent of the null ltbp3 mice phenotype. We conclude that LTBP3 is a novel component of the microfibrillar network involved in the acromelic dysplasia spectrum. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  11. Epithelial dysplasia in Caroli's disease.

    OpenAIRE

    Fozard, J. B.; Wyatt, J I; Hall, R.I.

    1989-01-01

    We report a young patient with a solitary intrahepatic cyst without demonstrable connection with the biliary tree. The operative appearances suggested hydatid disease but histological examination of the resected cyst showed that it was the result of Caroli's disease already complicated by severe dysplasia. This case provides further evidence for the premalignant nature of Caroli's disease.

  12. Genetics Home Reference: geleophysic dysplasia

    Science.gov (United States)

    ... from the University of Kansas Medical Center: Heart / Cardiology Conditions The MPS Society (UK) ... for This Page Giray O, Kýr M, Bora E, Saylam G, Ugurlu B, Gürel D. Clinical and morphological phenotype of geleophysic dysplasia. Ann Trop ...

  13. Allergic bronchopulmonary and sinus aspergillosis: the roentgenologic spectrum.

    Science.gov (United States)

    Shah, Ashok

    2003-01-01

    Allergic bronchopulmonary aspergillosis (ABPA) and allergic Aspergillus sinusitis (AAS) are the best recognized manifestations of Aspergillus-associated hypersensitivity respiratory disorders. These conditions occur predominantly in atopic individuals. Roentgenologic techniques play a pivotal role in the diagnosis of these two conditions. ABPA, on imaging, is characterized by fleeting pulmonary infiltrates often confused with pulmonary tuberculosis. However, central bronchiectasis on computed tomography (CT) is considered to be the hallmark of the disease. Though the diagnosis of AAS is primarily based on histopathology, roentgenology is essential for the diagnosis. Haziness of one or more paranasal sinuses is almost always seen on plain roentgenograms. However, CT proffers more reliable information with characteristic features that include heterogeneous densities and serpiginous areas of increased attenuation on non-contrast scans. Early diagnosis, with the help of roentgenologic techniques, and appropriate therapy could alter the natural history of these diseases.

  14. [Changes in time of the microbial flora in bronchopulmonary infections].

    Science.gov (United States)

    Fekete, T; Pop, A; Puriş, M

    1977-01-01

    The authors carried out a study on 100 cases with broncho-pulmonary infections in two different periods: 1963--1964 and 1973--1974. Changes in the microbial flora were investigated, as well as the sensitivity of germs to antibiotics. An increase was noted in the number of chronic bronchitis and a decrease in the number of pneumonia cases. In the microbial flora there was a constant proportion of staphylococcus, streptococcus and coli strains. The proportion of micrococci decreased with time and pneumococci practically disappeared, being replaced by klebsiella germs. Sensitivity to penicilin remained almost identical while that to chloramphenicol decreased significantly, as well as sensitivity to tetracycline and neomycine. The practical conclusion is that in some cases the application of penicilin treatment, before the results of the antibiogram are available appears to be justified.

  15. Clinical Characteristics of 118 Cases of Chronic Obstructive Pulmonary Disease Complicated with Primary Bronchopulmonary Carcinoma

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    Songlin ZHAO

    2017-08-01

    Full Text Available Background and objective The aim of this study is to investigate the clinical characteristics of patients with primary bronchopulmonary carcinoma complicated with chronic obstructive pulmonary disease (COPD, and to optimize the early diagnoses in the coexistence of COPD and primary bronchopulmonary carcinoma. Methods The clinical data of 118 patients with COPD complicated with primary bronchopulmonary carcinoma were analyzed retrospectively, including age, sex, smoking history, smoking index, clinical symptoms and signs, pathological type, staging, metastasis site and lung function index. 120 patients with simple COPD were selected as control. Results The smoking rate (55.1% and smoking index ≥400 branch /year (90.8% of the patients with COPD complicated with primary bronchopulmonary carcinoma were higher than the simple COPD group (20.8%, 48.0%. The difference between the two groups was statistically significant (P0.05, while the incidence of hemoptysis, weight loss, chest pain, hoarseness, pleural effusion and atelectasis were significantly higher than those in simple COPD group (P0.05, but the diffusing capacity of carbon monoxide (DLCO of COPD patients complicated with primary bronchopulmonary carcinoma was lower than that of simple COPD patients (P<0.05 . In the COPD patients with primary bronchopulmonary carcinoma, squamous cell carcinoma was the most common pathological type (51.7%. Male patients were mainly squamous cell carcinoma (60.7%, while female patients with adenocarcinoma (69.0%. Conclusion COPD combined with primary bronchopulmonary carcinoma occurs in male smokers more. There is higher incidence of squamous cell carcinoma. When they are first diagnosed, most of them are advanced or located late, due to no specific clinical symptoms at the early stages. Periodic chest CT examination for COPD patients can help early diagnoses of primary bronchopulmonary carcinoma.

  16. Allergic bronchopulmonary aspergillosis in Japan: A nationwide survey.

    Science.gov (United States)

    Oguma, Tsuyoshi; Taniguchi, Masami; Shimoda, Terufumi; Kamei, Katsuhiko; Matsuse, Hiroto; Hebisawa, Akira; Takayanagi, Noboru; Konno, Satoshi; Fukunaga, Koichi; Harada, Kazuki; Tanaka, Jun; Tomomatsu, Katsuyoshi; Asano, Koichiro

    2018-01-01

    Allergic bronchopulmonary aspergillosis (ABPA) is an allergic pulmonary disease characterized by a hypersensitivity reaction to Aspergillus species colonizing the airways. The clinical characteristics of ABPA may differ depending on genetic and environmental background. We performed a nationwide survey to determine the clinical characteristics of ABPA in Japan. In 2013, a questionnaire on physician-diagnosed ABPA/allergic bronchopulmonary mycosis was sent to 903 medical centers specializing in respiratory or allergic diseases. Cases fulfilling the following criteria were categorized as possible ABPA-central bronchiectasis (ABPA-CB): 1) presence of specific serum immunoglobulin E (IgE) antibodies or a positive skin reaction to Aspergillus, and 2) bronchiectasis or mucoid impaction in the central bronchi. Of 499 physician-diagnosed cases reported by 132 clinical centers, 358 cases met the criteria for possible ABPA-CB. Median age of ABPA-CB onset was 57 (interquartile range, 44-68) years; later-onset disease, developing ≥50 years of age, accounted for 66% of the cases and was associated with female sex, delayed onset of asthma, and lower levels of serum IgE. A third of the patients (120 patients, 34%) exhibited low levels of serum total IgE (<1000 IU/mL). Aspergillus species were isolated from sputum in 126/213 cases (59%), and Schizophyllum commune was identified in 12 (6%) patients. During the course of the treatment, ABPA recurred in 169 (48%) cases. This nationwide survey identified several unique clinical characteristics of ABPA in Japan, such as late-onset, relatively lower serum IgE levels, and frequent recurrences/flares. Copyright © 2017 Japanese Society of Allergology. Production and hosting by Elsevier B.V. All rights reserved.

  17. Anal dysplasia in kidney transplant recipients.

    Science.gov (United States)

    Ogilvie, James W; Park, Ina U; Downs, Levi S; Anderson, Kristin E; Hansberger, Jamie; Madoff, Robert D

    2008-12-01

    Although the risk of anal cancer in immunosuppressed individuals is significantly higher than in the general population, methods for detecting precancerous anal dysplasia have not been well studied in solid-organ transplant recipients. We sought to identify the incidence of anal dysplasia in kidney transplant recipients and associated factors that increase the likelihood of dysplasia. We prospectively analyzed kidney transplant recipients who had been immunosuppressed for at least 6 months with a functioning allograft. We interviewed willing participants and performed anal cytology sampling and high-resolution anoscopy; if we found microscopic abnormalities, we performed biopsies. We used univariate analysis to measure the association between variables and anal dysplasia. Of the 40 participants, 15 were women and 25 were men; their mean age was 61 years. Their median duration of immunosuppression was 5.6 years; 23 (59%) had fewer than 5 lifetime sexual partners, and 2 (5%) reported ever practicing anal intercourse. Of all cytology specimens, 35 (88%) had sufficient cells for interpretation and 2 (6%) demonstrated dysplasia. We performed biopsies in 11 patients; 6 had dysplasia (4 low-grade, 2 high-grade). Of these patients, five had normal anal cytology. The sensitivity of cytology to predict histologic evidence of dysplasia was 17%. Overall, seven (18%) had dysplasia according to either cytology or histology specimens; two (5%) had high-grade dysplasia. We found no significant associations between the tested variables and the presence of dysplasia. A significant proportion of kidney transplant recipients harbor anal dysplasia. One time anal cytology sampling was not predictive of histologic findings. Although these findings confirm their high risk for dysplasia, a larger sample is required to more accurately quantify risk factors for dysplasia and progression to cancer.

  18. Impact of Diuretic Therapy in the Treatment of Bronchopulmonary Dysplasia and Acute Kidney Injury in the Neonatal Population.

    Science.gov (United States)

    Johnson, Alexandra Kesler; Lynch, Natalie; Newberry, Desi; Jnah, Amy J

    2017-10-01

    Diuretics are among the most frequently prescribed medications in the neonatal intensive care unit (NICU), despite minimal data regarding the safety and efficacy of their use in the neonatal population. Off-label diuretic therapy is used in preterm and full-term infants to both optimize kidney function and improve respiratory status. This article examines the literature specific to the impact of diuretic therapy in the NICU and compares the benefits versus risks of utilization as they pertain to the prevention and treatment of renal and pulmonary dysfunction in this population. A comprehensive literature search of online databases was performed, utilizing: CINAHL via EBSCO, PubMed, and ProQuest. Full-text, peer-reviewed, clinical trials, and review articles published in the English language between 2005 and 2015 were searched. Diuretics rank as the seventh most frequently prescribed medication in the NICU. More than 8% of all NICU patients and 37% of infants born at less than 32 gestational weeks and weighing less than 1500 g are exposed to diuretics. Benefits include lung fluid resorption acceleration, improved urine output, fluid retention counteraction, and augmentation of physiologic weight loss. Diuretics are currently utilized in the NICU at an alarming rate, without adequate clinical trials regarding their safety and efficacy of use. Updated studies are needed regarding short- and long-term outcomes of diuretic use, as well as overall general outcome data regarding the impact and evaluation of diuretic usage in the NICU population.

  19. Complex orthopaedic management of patients with skeletal dysplasias

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    A. G. Baindurashvili

    2014-01-01

    Full Text Available Skeletal dysplasias are challenging for diagnostics and treatment. We present a series of fifteen patients with different forms of skeletal dysplasias with age ranged from 6 to 17 years with variable clinical presentations managed as a part of the project of scientific cooperation between Turner Paediatric Orthopaedic Institute and Orthopaedic Hospital Vienna-Speising. The spectrum of diagnoses included multiple epiphyseal dysplasia, spondyloepiphyseal dysplasia congenita, diastrophic dysplasia, metaphyseal dysplasia, spondylometaphyseal dysplasia, Stickler syndrome, Kniest dysplasia, and anauxetic dysplasia. Complex treatment, which included axial correction and juxta-articular realignment, was performed as a single-stage, or consecutive surgery. Surgical techniques included corrective osteotomies with internal fixation, guided growth technique and external fixation devices. Best results (full axial correction, normal alignment of the joint were achieved in 8 patients, including 2 patients with metaphyseal dysplasia, 2 patients with multiple epyphyseal dysplasia, 2 patients with spondyloepyphyseal dysplasia, patient with Stickler syndrome and patient with spondylometaphyseal dysplasia. Good results (partial correction at the present time were seen in 4 patients (2 patients with Kniest dysplasia, 1 - with multiple epyphyseal dysplasia and 1 - with anauxetic dysplasia. Satisfactory results (non-progressive condition in previous progression were obtained in 2 patients with diastrophic dysplasia, and poor results (progression of the deformity - in 1 patient with diastrophic dysplasia. Positive results in most of the cases of our series make promising future for usage of complex approach for orthopedic management of children with skeletal dysplasias; advanced international cooperation is productive and helpful for diagnostics and management of rare diseases.

  20. Autosomal recessive hydrotic ectodermal dysplasia.

    Science.gov (United States)

    Fried, K

    1977-04-01

    First cousins, a male and a female, with a new type of hidrotic ectodermal dysplasia are described. They were each the result of first cousin marriage from the Egyptian Karaite community. They both had partial adontia, conical peg-shaped teeth, fine hair that did not grow long, normal sweating, eversion of lips, and pronounced facial similarity. The male had cleft lip on the right side while the female had a branchial cyst on the left side of the neck. The parents of both the cases were completely normal. The patients had distinct clinical similarity to the condition described by Witkop (1965) as 'Autosomal dominant dysplasia of nails and hypodontia' but the nails were less affected and the mode of inheritance was completely different.

  1. Craniofacial features of cleidocranial dysplasia

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    Chin-Yun Pan

    2017-12-01

    Full Text Available Cleidocranial dysplasia (CCD is an autosomal-dominant malformation syndrome affecting bones and teeth. The most common skeletal and dental abnormalities in affected individuals are hypoplastic/aplastic clavicles, open fontanelles, short stature, retention of primary teeth, delayed eruption of permanent teeth, supernumerary teeth, and multiple impacted teeth. Treatment of CCD requires a multidisciplinary approach that may include dental corrections, orthognathic surgery and cranioplasty along with management of any complications of CCD. Early diagnosis of this condition enables application of the treatment strategy that provides the best quality of life to such patients. Notably, Runx2 gene mutations have been identified in CCD patients. Therefore, further elucidation of the molecular mechanism of supernumerary teeth formation related to Runx2 mutations may improve understanding of dental development in CCD. The insights into CCD pathogenesis may assist in the development of new treatments for CCD. Keywords: cleidocranial dysplasia, mutation, Runx2, supernumerary teeth

  2. Cleidocranial dysplasia: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Han, Jin Won [Kangnung National University College of Medicine, Kangnung (Korea, Republic of)

    2005-12-15

    Cleidocranial dysplasia is a rare, autosomal dominant congenital disorder. A 12-year-old female visited with chief complaint of unerupted permanent teeth. Also her father showed severe class III malocclusion. The extraoral radiography and computed tomography showed delayed closure of the cranial sutures and underdevelopment of maxilla, maxillary sinuses, and frontal sinus. Both clavicles were underdeveloped and thoracic rib cage was bell-shaped. Both zygomatic process appeared as hypoplastic feature. There were many unerupted permanent and supernumerary teeth in the maxilla and mandible. We examined location and number of the unerupted teeth using 3D CT. Finally we could conclude this case was cleidocranial dysplasia based on the clinico-radiologic findings.

  3. Cleidocranial dysplasia: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jae Duk; Lee, Chang Yul; You, Choong Hyun [Chosun University College of Medicine, Gwangju (Korea, Republic of)

    2004-03-15

    Cleidocranial dysplasia is a rare and autosomal dominant disorder characterized by aplasia or hypoplasia of the clavicles, an open fontanelle, dental abnormalities, and short stature. A 17-year-old female who presented with short stature and subsequent delay in eruption of permanent teeth is described. she showed the abnormal hypermobility of the shoulder, ocular hypertelorism and concave nasal bridge. Radiographs revealed the underdeveloped maxilla, defect of the cranium in the fontanelle region, and aplasia of the clavicles. Characteristically, panoramic view revealed near parallel-sided borders of the ascending ramus and downward curvature of the zygomatic arch with hypoplasia. The prolonged retention of deciduous teeth with delayed eruption of permanent teeth and multiple embedded supernumerary teeth were striking. Radiographic and clinical investigations revealed Cleidocranial dysplasia.

  4. Skeletal dysplasia in ancient Egypt.

    Science.gov (United States)

    Kozma, Chahira

    2008-12-01

    The ancient Egyptian civilization lasted for over 3000 years and ended in 30 BCE. Many aspects of ancient Egyptian culture, including the existence of skeletal dysplasias, and in particular achondroplasia, are well known through the monuments and records that survived until modern times. The hot and dry climate in Egypt allowed for the preservation of bodies and skeletal anomalies. The oldest dwarf skeleton, the Badarian skeleton (4500 BCE), possibly represents an epiphyseal disorder. Among the remains of dwarfs with achondroplasia from ancient Egypt (2686-2190 BCE), exists a skeleton of a pregnant female, believed to have died during delivery with a baby's remains in situ. British museums have partial skeletons of dwarfs with achondroplasia, humeri probably affected with mucopolysaccharidoses, and a skeleton of a child with osteogenesis imperfecta. Skeletal dysplasia is also found among royal remains. The mummy of the pharaoh Siptah (1342-1197 BCE) shows a deformity of the left leg and foot. A mummified fetus, believed to be the daughter of king Tutankhamun, has scoliosis, spina bifida, and Sprengel deformity. In 2006 I reviewed the previously existing knowledge of dwarfism in ancient Egypt. The purpose of this second historical review is to add to that knowledge with an expanded contribution. The artistic documentation of people with skeletal dysplasia from ancient Egypt is plentiful including hundreds of amulets, statues, and drawing on tomb and temple walls. Examination of artistic reliefs provides a glance of the role of people with skeletal dysplasia and the societal attitudes toward them. Both artistic evidence and moral teachings in ancient Egypt reveal wide integration of individuals with disabilities into the society. Copyright (c) 2008 Wiley-Liss, Inc.

  5. [Chlamydia trachomatis and cervix dysplasia].

    Science.gov (United States)

    Alaniz Sánchez, A; Flores Martínez, A; León Vistrain, M C; Castañeda Cano, E

    1995-09-01

    Fifty patients between 18 and 70 years of age from Gynecology and Obstetrics Department, Hospital General "Gonzalo Castañeda" ISSSTE, were studied. Patients were referred for bearing positive cytology with mild, moderate and severe dysplasia; also intentional search for Chlamydia trachomatis was made, both in cytology as well as with the immunofluorescence method, and also directed biopsy. A positive association was found in 10 patients (20%) proving that Chlamydia trachomatis is a promotor and modifier of cervical atypia.

  6. Epithelial dysplasia in Caroli's disease.

    Science.gov (United States)

    Fozard, J B; Wyatt, J I; Hall, R I

    1989-01-01

    We report a young patient with a solitary intrahepatic cyst without demonstrable connection with the biliary tree. The operative appearances suggested hydatid disease but histological examination of the resected cyst showed that it was the result of Caroli's disease already complicated by severe dysplasia. This case provides further evidence for the premalignant nature of Caroli's disease. Images Fig. 1 Fig. 2 Fig. 3 PMID:2767513

  7. Thanatophoric Dysplasia: A Case Report.

    Science.gov (United States)

    Sharma, Manisha; Jyoti; Jain, Rekha; Devendra

    2015-11-01

    Thanatophoric Dysplasia (TD) is a congenital, sporadic and the most lethal skeletal dysplasia caused by new mutation in the FGFR3 gene. At birth, it is characterized by shortening of the limbs (micromelia), small conical thorax, platyspondyly (flat vertebral bodies) and macrocephaly. TD is divided into two clinically defined subtypes: type I and II with some clinical overlap between the two subtypes. They can be differentiated by the skull shape and femur morphology. Ultrasound examination in the second trimester is often straight forward in diagnosing the congenital anomaly. We report a case of pre term fresh stillborn baby with dysmorphic facies, macrocephaly, micromelia with short stubby fingers and deep skin creases, narrow thorax and protuberant abdomen which delivered at our hospital. The ultrasound examination showed shortening of long bones with femur shaped like telephone receiver. Dysmorphic facial features and skeletal abnormalities in the baby lead us to make the diagnosis of TD type I. Because of the rarity of this condition we report this case of thanatophoric dysplasia with a short review of literature.

  8. Pelvic radiograph in skeletal dysplasias: An approach

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    Manisha Jana

    2017-01-01

    Full Text Available The bony pelvis is constituted by the ilium, ischium, pubis, and sacrum. The pelvic radiograph is an important component of the skeletal survey performed in suspected skeletal dysplasia. Most of the common skeletal dysplasias have either minor or major radiological abnormalities; hence, knowledge of the normal radiological appearance of bony pelvis is vital for recognizing the early signs of various skeletal dysplasias. This article discusses many common and some uncommon radiological findings on pelvic radiographs along with the specific dysplasia in which they are seen; common differential diagnostic considerations are also discussed.

  9. Presentation of hypohidrotic ectodermal dysplasia in two siblings

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    Uday Ginjupally

    2015-01-01

    Full Text Available Ectodermal dysplasias are a large hereditary group of disorders which are usually manifested as X-linked recessive disorders and have a full expression in males, whereas females show little to no signs of the disorder. The two most common types of ectodermal dysplasias are hypohidrotic ectodermal dysplasia (Christ-Siemens-Touraine syndrome and hidrotic ectodermal dysplasia (Clouston syndrome. Hypohidrotic ectodermal dysplasia is characterized by hypodontia, hypotrichosis, and hypohidrosis. Here, we present two female sibling cases of hypohidrotic ectodermal dysplasia.

  10. Prediction of exacerbation chronic bronchopulmonary diseases in children with influenza

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    O. I. Afanaseva

    2015-01-01

    Full Text Available The objective: To develop a method for predicting exacerbation of chronic illness in children with asthma and cystic fibrosis, patients with influenza, based on the study of the dynamics of cytokines. Materials and methods: Were examined 52 patients with bronchial asthma and 45 children with cystic fibrosis at the age from 1 year to 12 years, located in infectious pulmonary Department at the planned treatment of underlying pathology, in which influenza was in-hospital infection. Control group observations included 40 patients with the flu, without concomitant pulmonary disease. The etiology of viral infection was established by detection of viral RNA in nasopharyngeal swabs by PCR. Among the influenza viruses were identified influenza АH1N1, АH3N2, influenza B, and in 2009–2010 the predominant antigen was the pandemic influenza virus АH1N1pdm09. Determination of the concentration of serum interleukins IL-1β, IL-4, IL-8, IL-10, ТNF-α, IFN-γ was performed in the 1st and 3rd day of hospitalization cytokines by the solid-phase immune-enzyme assay. Analysis of the results performed using statistical package SPSS 17.0 EN for Windows. Results: The flu caused the aggravation associated bronchopulmonary pathology in 2/3 of children, as MV patients, and patients with BA (65,4%-66,7%, respectively. With an increase of the ratio of IL-4 / IFN-γ and IL-10/IFN-γ, at least 5-6 times, influenza can be considered a trigger of exacerbation of chronic bronchopulmonary pathologies that require amplification of the therapy of bronchial asthma and of сystic fibrosis. The growth of prognostic coefficients in 2-3 times allows using for treatment of influenza in these patients only antiviral agents. Conclusion: The study has shown a method for predicting exacerbation of bronchial asthma and cystic fibrosis in children at an early stage of influenza by calculating the ratio of IL-4/IFN-γ and IL-10/IFN-γ in children aged from 1 year to 12 years. 

  11. Developmental Dislocation (Dysplasia) of the Hip (DDH)

    Science.gov (United States)

    ... bone. In babies and children with developmental dysplasia (dislocation) of the hip (DDH), the hip joint has not formed normally. ... the American Academy of Orthopaedic Surgeons. .org Developmental Dislocation (Dysplasia) of the Hip cont. • Family history of DDH (parents or siblings) • ...

  12. Allergic bronchopulmonary aspergillosis in patients with cystic fibrosis

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    Ibrahim Ahmed Janahi

    2017-01-01

    Full Text Available Allergic bronchopulmonary aspergillosis (ABPA is a pulmonary disorder that often occurs in patients with asthma or cystic fibrosis (CF and is characterized by a hypersensitivity response to the allergens of the fungus Aspergillus fumigatus. In patients with CF, growth of A. fumigatus hyphae within the bronchial lumen triggers an immunoglobulin E (IgE-mediated hypersensitivity response that results in airway inflammation, bronchospasm, and bronchiectasis. In most published studies, the prevalence of ABPA is about 8.9% in patients with CF. Since the clinical features of this condition overlap significantly with that of CF, ABPA is challenging to diagnose and remains underdiagnosed in many patients. Diagnosis of ABPA in CF patients should be sought in those with evidence of clinical and radiologic deterioration that is not attributable to another etiology, a markedly elevated total serum IgE level (while off steroid therapy and evidence of A. fumigatus sensitization. Management of ABPA involves the use of systemic steroids to reduce inflammation and modulate the immune response. In patients who do not respond to steroids or cannot tolerate them, antifungal agents should be used to reduce the burden of A. fumigatus allergens. Recent studies suggest that omalizumab may be an effective option to reduce the frequency of ABPA exacerbations in patients with CF. Further randomized controlled trials are needed to better establish the efficacy of omalizumab in managing patients with CF and ABPA.

  13. Craniometadiaphyseal dysplasia, wormian bone type.

    Science.gov (United States)

    Santolaya, J M; Hall, C M; García-Miñaur, S; Delgado, A

    1998-05-18

    We report on a 4-year-old boy with craniometadiaphyseal dysplasia (CMDD), wormian bone type. Component manifestations include a large head with prominent forehead, skull changes showing multiple wormian bones, wide long tubular bones without the usual metaphyseal flare, wide and short tubular bones without the normal diaphyseal constriction, and wide ribs and clavicles. In addition to these findings, the propositus, his brother, his father, and a paternal aunt all have parietal protuberances, which seem not related to CMDD. Parental consanguineity supports the autosomal recessive transmission of the condition.

  14. Conservative Management of Hip Dysplasia.

    Science.gov (United States)

    Harper, Tisha A M

    2017-07-01

    Hip dysplasia (HD) is a common orthopedic condition seen in small animal patients that leads to osteoarthritis of the coxofemoral joint. The disease can be managed conservatively or surgically. The goals of surgical treatment in the immature patient are to either prevent the clinical signs of HD or to prevent or slow the progression of osteoarthritis. In mature patients surgery is used as a salvage procedure to treat debilitating osteoarthritis. Conservative management can be used in dogs with mild or intermittent clinical signs and includes nutritional management and weight control, exercise modification, physical rehabilitation, pain management and disease-modifying agents. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. Ectodermal dysplasia in identical twins

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    Gurkar Haraswarupa Puttaraju

    2013-01-01

    Full Text Available Hereditary hypohidrotic ectodermal dysplasia (HED is typically inherited as an X-linked recessive trait, characterized by deformity of at least two or more of the ectodermal structures - hair, teeth, nails and sweat glands. Two cases of hereditary HED involving identical male twins, is being documented for the rarity of its occurrence with special attention given to genetics, pathophysiology, clinical, intraoral manifestations and to the methods to improve the masticatory function, the facial esthetics and psychology of patients affected by this disease.

  16. DYSPLASIA OF HIP DEVELOPMENT: UPDATE

    Science.gov (United States)

    Guarniero, Roberto

    2015-01-01

    The term “developmental dysplasia of the hip” (DDH) includes a wide spectrum of abnormalities that affect the hip during its growth, ranging from dysplasia to joint dislocation and going through different degrees of coxofemoral subluxation. The incidence of DDH is variable, and depends on a number of factors, including geographical location. Approximately one in 1,000 newborn infants may present hip dislocation and around 10 in 1,000 present hip instability. Brazil has an incidence of five per 1,000 in terms of findings of a positive Ortolani sign, which is the early clinical sign for detecting the disorder. The risk factors for DDH include: female sex, white skin color, primiparity, young mother, breech presentation at birth, family history, oligohydramnios, newborns with greater weight and height, and deformities of the feet or spine. Hip examinations should be routine for newborns, and should be emphasized in maternity units. Among newborns and infants, the diagnosis of DDH is preeminently clinical and is made using the Ortolani and Barlow maneuvers. Conventional radiography is of limited value for confirming the diagnosis of DDH among newborns, and ultrasound of the hip is the ideal examination. The treatment of DDH is challenging, both for pediatric orthopedists and for general practitioners. The objectives of the treatment include diagnosis as early as possible, joint reduction and stabilization of the hip in a secure position. Classically, treatment options are divided according to different age groups, at the time of diagnosis. PMID:27022528

  17. The other hip in unilateral hip dysplasia

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Rømer, Lone; Søballe, Kjeld

    2006-01-01

    We reviewed transverse pelvic computed tomography scans of 197 consecutively referred adult patients with hip pain thought to be secondary to developmental dysplasia. A center-edge angle of 20 degrees or less was considered the upper normal value. Four groups were identified: 69 patients with app......We reviewed transverse pelvic computed tomography scans of 197 consecutively referred adult patients with hip pain thought to be secondary to developmental dysplasia. A center-edge angle of 20 degrees or less was considered the upper normal value. Four groups were identified: 69 patients...... with apparently unilateral right developmental dysplasia (left hip center-edge angles greater than 20 degrees), 26 patients with apparently unilateral left developmental dysplasia (right hip center-edge angles greater than 20 degrees), 68 patients with bilateral developmental dysplasia, and 34 patients...... with bilateral borderline developmental dysplasia (bilateral center-edge angles less than or equal to 25 degrees). The pelvic computed tomography scans were compared with computed tomography scans of 41 control subjects with healthy hips. The joint anatomy of patients with developmental dysplasia differed from...

  18. Multiple Epiphyseal Dysplasia (MED: A Rare Type of Skeletal Dysplasia

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    Mohammad Imnul Islam

    2012-06-01

    Full Text Available Multiple epiphyseal dysplasia (MED is a congenital disorder of skeletal development that primarily affects the ends of long bones, causing progressive joint and bone inflammation and short stature. Mutations in several genes are responsible for pathogenesis of this disease. We are reporting a case of MED who presented with the complaints of multiple swelling of the joints which was associated with pain during movement for last seven years. The patient had flexion deformity of all the affected joints along with restriction of movement. These were associated with kyphosis, pectus carnitum, knock-knee and short stature. Radiological findings were suggestive of MED. Counseling was done with the parents regarding the etiology, progression and outcome of the disease.DOI: http://dx.doi.org/10.3329/bsmmuj.v5i1.11025 BSMMU J 2012; 5(1:57-60 

  19. Prevalence of hip dysplasia, elbow dysplasia and humeral head osteochondrosis in dog breeds in Belgium.

    Science.gov (United States)

    Coopman, F; Verhoeven, G; Saunders, J; Duchateau, L; van Bree, H

    2008-11-29

    The official screening results of the Belgian National Committee for Inherited Skeletal Disorders, an affiliate of the Belgian Kennel Club, have been used to estimate the prevalence of hip dysplasia, elbow dysplasia and humeral head osteochondrosis in the dog breeds in Belgium, and these have been compared with reported prevalence data from other countries. In some breeds, the prevalence of hip and elbow dysplasia is very high, both in Belgium and in other countries. Comparisons of the prevalence of hip dysplasia are not always feasible because different systems are used to evaluate the quality of the hips and because there is no strict consensus on what should be considered a diseased hip joint.

  20. Developmental hip dysplasia in adolescence

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    Vukašinović Zoran

    2009-01-01

    Full Text Available The authors define adolescence and developmental dysplasia of the hip (DDH. Special attention is paid to pathological findings characteristic of DDH in adolescence (unrecognized and untreated DDH; treated DDH, but non-terminated treatment; DDH diagnosed with delay, inadequately treated, with complications. The authors emphasise that DDH treatment has to be successfully terminated well before the adolescence; possibilities are explained on management modes at the time of adolescence, and possible persons guilty for the persistence of later hip problems are indicated. Based on the authors' experience and having in mind all surgical possibilities for the treatment (pelvic osteotomies, femoral osteotomies, trochanteroplasties, leg length equalization procedures the authors propose treatment protocols. The intention is to provide better treatment results and to prevent secondary hip arthrosis. Furthermore, how to improve the struggle against DDH is suggested.

  1. Ectodermal Dysplasia Skin Fragility Syndrome

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    Ayça Alan Atalay

    2014-06-01

    Full Text Available Ectodermal dysplasia-skin fragility syndrome (EDSFS is a rare autosomal recessive genodermatosis first described in 1997 by Mc Grath. EDSFS results from loss of function mutations in plakophilin-1 (PKP1. PKP1 is a structural component of desmosomes, cellcell adhesion complexes. It is also found as a nuclear protein in several cell types that are lack of desmosomes. In skin, however, PKP1 expression is confined mainly to suprabasal keratinocytes and the outer root sheath of hair follicules. Loss of function mutation in PKP1 leads to extensive skin fragility, bullae and erosions following minor trauma, focal keratoderma with painful fissures, alopecia, and nail dystrophy. In some patients hypohidrosis may also be seen. EDSFS is now considered as a specific suprabasal form of epidermolysis bullosa simplex. In this report we describe a 20 year old EDSFS case.

  2. Posttraumatic Cranial Cystic Fibrous Dysplasia

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    Arata Tomiyama

    2011-01-01

    Full Text Available A 14-year-old was girl admitted to our hospital with a subcutaneous mass of the occipital head. The mass had grown for 6 years, after she had sustained a head injury at the age of 6, and was located directly under a previous wound. Skull X-ray Photograph (xp, computed tomography (CT, and magnetic resonance imaging (MRI showed a bony defect and cystic changes in the skull corresponding to a subcutaneous mass. Bone scintigraphy revealed partial accumulation. The patient underwent total removal of the skull mass, and the diagnosis from the pathological findings of the cyst wall was fibrous dysplasia (FD. The radiographic findings for cystic cranial FD can be various. Progressive skull disease has been reported to be associated with head trauma, but the relationship between cranial FD and head trauma has not been previously reported. Previous studies have suggested that c-fos gene expression is a key mechanism in injury-induced FD.

  3. Renal infarction complicating fibromuscular dysplasia.

    Science.gov (United States)

    Gavalas, M; Meisner, R; Labropoulos, N; Gasparis, A; Tassiopoulos, A

    2014-01-01

    Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease that most commonly affects the renal and extracranial carotid arteries. We present 3 cases of renal infarction complicating renal artery FMD in 42-, 43-, and 46-year-old females and provide a comprehensive review of the literature on this topic. In our patients, oral anticoagulation therapy was used to treat all cases of infarction, and percutaneous angioplasty was used nonemergently in one case to treat refractory hypertension. All patients remained stable at 1-year follow-up. This is consistent with outcomes in previously published reports where conservative medical management was comparable to surgical and interventional therapies. Demographic differences may also exist in patients with renal infarction and FMD. A higher prevalence of males and a younger age at presentation have been found in these patients when compared to the general population with FMD. © The Author(s) 2014.

  4. Histogenesis of retinal dysplasia in trisomy 13

    National Research Council Canada - National Science Library

    Chan, Ada; Lakshminrusimha, Satyan; Heffner, Reid; Gonzalez-Fernandez, Federico

    2007-01-01

    .... Here, we describe the ocular findings in a case of trisomy 13 to better understand the histogenesis of the rosettes, or tubules, characteristic of the retinal dysplasia associated with this condition...

  5. Frontometaphyseal dysplasia; Dysplazja czolowo-przynasadowa

    Energy Technology Data Exchange (ETDEWEB)

    Bieganski, T.; Makowski, A. [Zaklad Radiologii Pediatrycznej and Oddzial Otiatrii i Laryngologii, Centrum Zdrowia Matki Polki, Lodz (Poland)

    1995-12-31

    The frequency of the features of frontometaphyseal dysplasia was calculated on the basis of literature data. Our own observation of this disorder is described with special reference to otologic changes. (author) 5 refs, 4 figs, 2 tabs

  6. Rootless teeth: Dentin dysplasia type I

    National Research Council Canada - National Science Library

    Fulari, Sangamesh G; Tambake, Deepti P

    2013-01-01

    A rare case of hereditary disturbance of dentine, Dentin dysplasia type I is presented, which is characterized by short or total absence of roots, obliterated pulp chambers, and peri-apical radiolucencies...

  7. Hip dysplasia in wrestlers: three lessons learned.

    Science.gov (United States)

    Byrd, J W Thomas; Clohisy, John C; Kim, Young-Jo; Gwathmey, F Winston; Jones, Kay S; Millis, Michael B

    2017-12-01

    Hip problems due to dysplasia are commonly associated with female athletes in sports demanding supraphysiologic motion, such as ballet, gymnastics and figure skating. However, hip problems are rarely mentioned among wrestlers, a male sport in which flexibility is advantageous. Dysplasia may have a mostly unrecognized prevalence among wrestlers that can lead to problems and benefit from reorientation periacetabular osteotomy (PAO). Study design in this research is Level 4 evidence case reports. Three consecutive intercollegiate wrestlers ages 20, 21 and 22 years underwent PAO for dysplasia and are reported. Two underwent concomitant arthroscopy. Each returned successfully to intercollegiate wrestling at 6, 8 and 11 months. There were no complications. This work concludes that dysplasia has an unknown but mostly unrecognized prevalence among wrestlers. With proper recognition and treatment with PAO, there is a reasonable expectation that they could return to wrestling.

  8. SPECIFIC SERUM IMMUNOPATTERNS IN CLINICAL-PHASES OF ALLERGIC BRONCHOPULMONARY ASPERGILLOSIS

    NARCIS (Netherlands)

    LESER, C; KAUFFMAN, HF; VIRCHOW, C; MENZ, G

    1992-01-01

    Immunoblotting, radioallergosorbent test (RAST), and enzyme-linked immunosorbent assay (ELISA) were performed to determine specific IgE and IgG responses to Aspergillus fumigatus (Af) allergens (IgE-Af; IgG-Af). Serology results were compared in patients with allergic bronchopulmonary aspergillosis

  9. Endobronchial valves in the management of bronchial fistulae caused by bronchopulmonary aspergillosis.

    Science.gov (United States)

    Williams, Rhys; Krishnadas, Rakesh; Patel, Bipen; Jarad, Nabil

    2015-12-01

    Following an aggressive episode of bronchopulmonary aspergillosis, a 54-year-old man developed a symptomatic air leak via a tunnel between the left upper lobe and an extra chest wall cavity. Following the failure of several surgical procedures to close the tunnel, endobronchial valves normally used in management of emphysema were used to successfully treat the air leak. 2015 BMJ Publishing Group Ltd.

  10. Dysplasia epiphysealis hemimelica of the tibial tubercle

    Energy Technology Data Exchange (ETDEWEB)

    Thacker, M.M.; Scully, S.P.; Pitcher, J.D.; Temple, H. Thomas [University of Miami, Department of Orthopedics and Rehabilitation, FL (United States); Azouz, E.M. [University of Miami, Department of Radiology, FL (United States)

    2006-03-15

    Dysplasia epiphysealis hemimelica (DEH) is a rare skeletal dysplasia with epiphyseal involvement first described by Mouchet and Belot in 1926. Lower extremity involvement is common and might involve a single or multiple epiphyses in the affected extremity. We report an unusual case of involvement of the tibial tubercle in a girl aged 4 years 8 months, and we present the clinical, radiographic and pathologic findings. We discuss the role of MRI in the diagnosis and treatment plan. (orig.)

  11. CONGENITAL RADIAL DYSPLASIA: A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Venkatram Reddy

    2015-08-01

    Full Text Available Congenital radial dysplasia, also referred to as radial club hand , means deficiency along the preaxial or radial side of the extremity. It ranges from hypoplasia of the thumb to variou s degrees of radial hypoplasia. We present one such rare case of type 4 congenital unilateral isolated radial dysplasia with carpel anomaly , reported to our department in SVS medical C ollege, Mahabubanagar, Telangana state

  12. Treatment of canine hip dysplasia: a review.

    OpenAIRE

    Remedios, A M; Fries, C L

    1995-01-01

    This article discusses the treatment approaches and recommendations for canine hip dysplasia. A search of the literature database MEDLINE (1969-1994) was conducted and relevant journal articles regarding the medical and surgical treatment of hip dysplasia were selected and reviewed. Dysplastic dogs can be divided, for treatment purposes, into those with no or minimal osteoarthrosis, and those with moderate to severe osteoarthrosis. In young animals with joint laxity and pain, but with no or m...

  13. Long-term results of PRRT in advanced bronchopulmonary carcinoid

    Energy Technology Data Exchange (ETDEWEB)

    Mariniello, Annapaola; Bodei, Lisa; Baio, Silvia Melania; Gilardi, Laura; Colandrea, Marzia; Papi, Stefano; Grana, Chiara Maria [European Institute of Oncology, Division of Nuclear Medicine, Milan (Italy); Tinelli, Carmine [IRCCS Foundation Policlinico San Matteo, Epidemiology and Biometric Unit, Pavia (Italy); Valmadre, Giuseppe [Presidio Ospedaliero E. Morelli AOVV, Sondalo (Italy); Fazio, Nicola [European Institute of Oncology, Unit of Gastrointestinal Medical Oncology and Neuroendocrine Tumors, Milan (Italy); Galetta, Domenico [European Institute of Oncology, Thoracic Surgery Division, Milan (Italy); Paganelli, Giovanni [Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Nuclear Medicine and Radiometabolic Units, Meldola (Italy)

    2016-03-15

    Peptide receptor radionuclide therapy (PRRT) for the treatment of neuroendocrine tumours (NET) has been explored for almost two decades, but there are still few trials that have exclusively investigated well-differentiated and moderately differentiated NET arising from the respiratory tree. Thus, the aim of this study was to explore the outcome in patients affected by bronchopulmonary carcinoid (BPC) following PRRT. We retrospectively analysed 114 patients with advanced stage BPC consecutively treated with PRRT at the European Institute of Oncology, Milan, from 1997 to 2012 and followed until October 2014. The objective responses, overall survival (OS) and progression-free survival (PFS) were rated, and three different PRRT protocols ({sup 90}Y-DOTATOC vs. {sup 177}Lu-DOTATATE vs. {sup 90}Y-DOTATOC + {sup 177}Lu-DOTATATE) were compared with regard to their efficacy and tolerability. The median OS (evaluated in 94 of the 114 patients) was 58.8 months. The median PFS was 28.0 months. The {sup 177}Lu-DOTATATE protocol resulted in the highest 5-year OS (61.4 %). Morphological responses (partial responses + minor responses) were obtained in 26.5 % of the cohort and were associated with longer OS and PFS. The {sup 90}Y-DOTATOC + {sup 177}Lu-DOTATATE protocol provided the highest response rate (38.1 %). Adverse events were mild in the majority of patients. However, haematological toxicity negatively affected survival. No severe (grade 3/4) serum creatinine increase was observed. Patients treated with {sup 90}Y-DOTATOC alone more frequently showed a mild/moderate decrease in renal function. In patients treated with chemotherapy before PRRT had a shorter OS and PFS, and a higher risk of developing nephrotoxicity. In a large cohort of patients with advanced BPC treated in a ''real-world'' scenario and followed up for a median of 45.1 months (range 2 - 191 months), PRRT proved to be promising in prolonging survival and delaying disease progression. Despite

  14. Retrospective Study of Clinicopathological Characteristics in Bronchopulmonary Carcinoid

    Directory of Open Access Journals (Sweden)

    Hongliang LIAO

    2010-06-01

    Full Text Available Background and objective Bronchopulmonary carcinoid (BPC account for less than 2% of all primary lung malignant tumors, but few related studies were reported. The aim of this study is to analyze this rare disease’s clinicopathological characteristics. Methods The clinical data of 28 patients with BPC in Cancer Center of Sun Yat-sen University, from January 1994 to June 2009, were enrolled into retrospective analysis. First, the corresponding paraffin blocks reexamined, slice up and stained, multiple pathologists re-consulted, and its subsets (typical carcinoid, TC; atypical carcinoid, AC defined. Second, the clinical characteristics and immunohistochemical markers and its relationship with prognosis were analyzed. Results First, the 5-year survival for overall and TC, AC was 56% and 70%, 41% respectively in 28 cases. The markers CD99, Bcl-2 and Ki-67 expression correlated significantly with the BPC subsets (P=0.017, P=0.043, and P=0.033 respectively. Further univariate analysis revealed that advanced TNM staging (P=0.037, lymph node metastasis (P=0.001 and Ki-67 nucleolus’s positive expression (P=0.009 are poor prognostic factors. Second, the overall, TC, AC 5-year survival rate was 73%, 83%, 57% respectively in 20 cases underwent the radically surgical resection. Further univariate analysis revealed that AC subset (P=0.013, lymph node metastasis (P=0.004 and Ki-67 nucleolus’s positive expression (P=0.006, advanced TNM staging (P=0.047 are poor prognostic factors in this 20 cases. Third, as univariate analysis, local recurrence and metastasis (n=4 correlate significantly with Ki-67 nucleolus’s and Bcl-2 positive expression (P=0.027, 0.045, respectively. Conclusion The prognosis of BPC was better than other types of primary lung cancer. Ki-67, bcl-2 high expression and advanced TNM staging are the poor recurrence and prognostic factors of BPC. The radical surgery remains the treatment of choice for resectable candidates in BPC as NSCLC.

  15. Glutathion-S-Transferase P1 polymorphisms association with broncopulmonary dysplasia in preterm infants.

    Science.gov (United States)

    Karagianni, P; Rallis, D; Fidani, L; Porpodi, M; Kalinderi, K; Tsakalidis, C; Nikolaidis, N

    2013-10-01

    Oxidative stress, characterized by the excretion of pre-oxidative and anti-oxidative proteases, has a key role in the pathogenesis of bronchopulmonary dysplasia (BPD). One of the many host anti-oxidant enzymes is glutathione-S-transferase P1 (GSTP1), with three polymorphic alleles having been identified: homozygous ile, heterozygous ile/val and homozygous val isomorph. The aim of this study was to examine the genetic predisposition to BPD in the GSTP1 polymorphisms. A prospective case-control study was carried out in the 2nd Neonatal Intensive Care Unit of Aristotle University in Thessaloniki, Greece during 2008. The genetic polymorphisms of GSTP1 in 28 preterms <32 weeks gestational age (GA) with BPD compared to 74 controls (33 preterms without BPD and 41 healthy terms) were examined. The homozygous ile isomorph was predominant in all groups (preterms with BPD: 82%, preterms without BPD: 70%, healthy terms: 78%), followed by the heterozygous ile/val (14%, 18% and 20% respectively) and the homozygous val isomorph (4%, 12% and 2% respectively). The homozygous ile isomorph was also identified in the majority of preterms with mild (80%), moderate (100%) and severe (73%) BPD. The GSTP1 genetic distribution did not differ between the groups and GSTP1 polymorphisms were not associated with the severity of BPD. This study could not confirm an association between GSTP1 polymorphisms and the development of BPD or the severity of the disease.

  16. Васкground development questionnaire quality of life for school age children with broncho-pulmonary disease

    Directory of Open Access Journals (Sweden)

    Nataliya Ivasyk

    2015-08-01

    Full Text Available Purpose: to prove feasibility of developing a questionnaire to assess quality of life for school-age children with acute broncho-pulmonary diseases. Material and Methods: analysis of scientific and methodological literature on the study of quality of life. Results: to assess quality of life using both general and specific questionnaires. The most of special are questionnaires designed for adults, and all pulmonary questionnaires designed for chronic diseases. There are of survey questionnaire for children with acute broncho-pulmonary diseases. Conclusions: the proposed of us a questionnaire for children with broncho-pulmonary diseases include questions to determine the effect impact of symptoms of diseasea on motor activity and quality of life. In future we plans to cheak effectiveness of the application of this questionnaire for determine quality of life of children with acute broncho-pulmonary diseases with goal to determine the effectiveness of the rehabilitation process

  17. Phrenic motor outputs in response to bronchopulmonary C‐fibre activation following chronic cervical spinal cord injury

    National Research Council Canada - National Science Library

    Lee, Kun‐Ze

    2016-01-01

    .... Supersensitivity of phrenic motor outputs to the inhibitory effect of bronchopulmonary C‐fibre activation is due to a shift of phrenic motoneuron types and slow recovery of phrenic motoneuron discharge in cervical spinal cord‐injured animals...

  18. Expansive focal cemento-osseous dysplasia.

    Science.gov (United States)

    Bulut, Emel Uzun; Acikgoz, Aydan; Ozan, Bora; Zengin, Ayse Zeynep; Gunhan, Omer

    2012-01-01

    To present a case of expansive focal cemento-osseous dysplasia and emphasize the importance of differential diagnosis. Cemento-osseous dysplasia is categorized into three subtypes on the basis of the clinical and radiographic features: Periapical, focal and florid. The focal type exhibits a single site of involvement in any tooth-bearing or edentulous area of the jaws. These lesions are usually asymptomatic; therefore, they are frequently diagnosed incidentally during routine radiographic examinations. Lesions are usually benign, show limited growth, and do not require further surgical intervention, but periodic follow-up is recommended because occasionally, this type of dysplasia progresses into florid osseous dysplasia and simple bone cysts are formed. A 24-year-old female patient was referred to our clinic for swelling in the left edentulous mandibular premolarmolar region and felt discomfort when she wore her prosthetics. She had no pain, tenderness or paresthesia. Clinical examination showed that the swelling in the posterior mandible that was firm, nonfluctuant and covered by normal mucosa. On panoramic radiography and computed tomography, a well defined lesion of approximately 1.5 cm in diameter of mixed density was observed. The swelling increased slightly in size over 2 years making it difficult to use prosthetics and, therefore, the lesion was totally excised under local anesthesia, and surgical specimens were submitted for histopathological examination. The histopathological diagnosis was focal cemento-osseous dysplasia. In the present case, because of the increasing size of the swelling making it difficult to use prosthetics, young age of the patient and localization of the lesion, in the initial examination, cemento-ossifying fibroma was suspected, and the lesion was excised surgically; the histopathological diagnosis confirmed it as focal cemento-osseous dysplasia. We present a case of expansive focal cemento-osseous dysplasia. Differential diagnosis

  19. [Relationship between congenital heart disease and bronchial dysplasia].

    Science.gov (United States)

    Zeng, Shuang-Lin; Li, Ya-Jun; Huang, Ting; Tan, Li-Hua; Mei, Xi-Long; Sun, Jian-Ning

    2011-11-01

    To study the relationship of the incidence of bronchial dysplasia (bronchial anomalous origin and bronchial stenosis) with congenital heart disease. A total of 185 children with congenital heart disease or bronchial dysplasia were enrolled. Bronchial dysplasia was identified by the 64-MSCT conventional scanning or thin slice scanning with three-dimensional reconstruction. Forty-five children (25.3%) had coexisting bronchial dysplasia and congenital heart disease. The incidence rate of bronchial dysplasia in children with congenital heart disease associated with ventricular septal defect was higher than in those without ventricular septal defect (33.7% vs 15.0%; Pincidence rate of bronchial dysplasia between the children with congenital heart disease who had a large vascular malformation and who did not. Bronchial dysplasia often occurs in children with congenital heart disease. It is necessary to perform a tracheobronchial CT scanning with three-dimensional reconstruction to identify tracheobronchial dysplasia in children with congenital heart disease, especially associated with ventricular septal defect.

  20. Progressive pseudorheumatoid dysplasia in North and West Africa ...

    African Journals Online (AJOL)

    Progressive pseudorheumatoid dysplasia is a rare autosomal recessive spondyloepiphyseal dysplasia characterized by predominant involvement of articular cartilage with progressive joint stiffness and enlargement in the absence of inflammation. Short stature, joint contractures, gait disturbance, and scoliosis and/or ...

  1. COMPLEX ORTHOPAEDIC MANAGEMENT OF PATIENTS WITH SKELETAL DYSPLASIAS

    OpenAIRE

    A.G. Baindurashvili; V. M. Kenis; E. V. Melchenko; Grill, F.; A. Al-Kaissi

    2014-01-01

    Skeletal dysplasias are challenging for diagnostics and treatment. We present a series of fifteen patients with different forms of skeletal dysplasias with age ranged from 6 to 17 years with variable clinical presentations managed as a part of the project of scientific cooperation between Turner Paediatric Orthopaedic Institute and Orthopaedic Hospital Vienna-Speising. The spectrum of diagnoses included multiple epiphyseal dysplasia, spondyloepiphyseal dysplasia congenita, diastrophic dysplas...

  2. Nonimmune idiopathic hydrops fetalis and congenital lymphatic dysplasia

    NARCIS (Netherlands)

    Bellini, Carlo; Hennekam, Raoul C. M.; Boccardo, Francesco; Campisi, Corradino; Serra, Giovanni; Bonioli, Eugenio

    2006-01-01

    Six newborns that presented at birth with nonimmune hydrops fetalis and for whom no cause could be found were investigated for the presence of lymphatic dysplasia. Careful analysis led to findings of some degree of lymphatic dysplasia in all patients. This suggests that lymphatic dysplasia may

  3. Variable manifestations of dysplasia epiphysealis hemimelica

    Energy Technology Data Exchange (ETDEWEB)

    Azouz, E.M.; Slomic, A.M.; Marton, D.; Rigault, P.; Finidori, G.

    1985-01-01

    Dysplasia epiphysealis hemimelica (DEH) is an osteocartilaginous overgrowth involving one or multiple epiphyses or ossification centers, usually in a lower extremity on one side of the body. Characteristically the involvement is hemimelic, i.e., either the medial or lateral part of the ossification center is involved. The authors have studied 24 patients with DEH and are adding 15 new cases to the literature. Because of the variable manifestations of the dysplasia and its different degrees of involvement in the affected children, they have subdivided it into localized, classical and generalized. In the generalized form, there is involvement of a whole lower extremity from the pelvis to the foot, and some of these patients show megaepiphyses with enlargement of a whole epiphyseal center, not only its medial or lateral part. The authors have also described and illustrated other special features of the dysplasia especially the advanced bone age and the metaphyseal and growth plate involvement.

  4. A novel syndrome resembling Desbuquois dysplasia.

    Science.gov (United States)

    Al Kaissi, Ali; Nessib, N; Ghachem, M B; Hammou, A; Guiddana, N; Kozlowski, K

    2005-01-01

    We report on three Tunisian siblings with a rare assortment of clinical and radiographic abnormalities closely resembling Desbuquois dysplasia. However, the siblings have had normal facies, normal hands, and were mentally normal. There were severe musculo-skeletal distinguishing features such as joint stiffness, severe kyphoscoliosis, and multiple large joint dislocations. Moreover, the patients had an additional remarkable radiographic feature not reported in Desbequois dysplasia-multiple carpal ossification centers. The diagnosis of Desbuquois dysplasia is more difficult in older children and adults as the characteristic facial features of early childhood may recede, and the metaphyseal growth plates obliterate. This condition of these patients represents a novel Desbuquois-like syndrome. (c) 2004 Wiley-Liss, Inc.

  5. The variable manifestations of dysplasia epiphysealis hemimelica.

    Science.gov (United States)

    Azouz, E M; Slomic, A M; Marton, D; Rigault, P; Finidori, G

    1985-01-01

    Dysplasia epiphysealis hemimelica (DEH) is an osteocartilaginous overgrowth involving one or multiple epiphyses or ossification centers, usually in a lower extremity on one side of the body. Characteristically the involvement is hemimelic i.e. either the medial or lateral part of the ossification center is involved. We have studied 24 patients with DEH and are adding 15 new cases to the literature. Because of the variable manifestations of the dysplasia and its different degrees of involvement in the affected children, we have subdivided it into localized, classical and generalized forms. In the generalized form, there is involvement of a whole lower extremity from the pelvis to the foot, and some of these patients show megaepiphyses with enlargement of a whole epiphyseal center, not only its medial or lateral part. We have also described and illustrated other special features of the dysplasia especially the advanced bone age and the metaphyseal and growth plate involvement.

  6. Intestinal epithelial dysplasia (tufting enteropathy

    Directory of Open Access Journals (Sweden)

    de Serres Natacha

    2007-04-01

    Full Text Available Abstract Intestinal epithelial dysplasia (IED, also known as tufting enteropathy, is a congenital enteropathy presenting with early-onset severe intractable diarrhea causing sometimes irreversible intestinal failure. To date, no epidemiological data are available, however, the prevalence can be estimated at around 1/50,000–100,000 live births in Western Europe. The prevalence seems higher in areas with high degree of consanguinity and in patients of Arabic origin. Infants develop within the first days after birth a watery diarrhea persistent in spite of bowel rest and parenteral nutrition. Some infants are reported to have associated choanal rectal or esophageal atresia. IED is thought to be related to abnormal enterocytes development and/or differentiation. Nonspecific punctuated keratitis was reported in more than 60% of patients. Histology shows various degree of villous atrophy, with low or without mononuclear cell infiltration of the lamina propria but specific histological abnormalities involving the epithelium with disorganization of surface enterocytes with focal crowding, resembling tufts. Several associated specific features were reported, including abnormal deposition of laminin and heparan sulfate proteoglycan (HSPG in the basement membrane, increased expression of desmoglein and ultrastructural changes in the desmosomes, and abnormal distribution of α2β1 integrin adhesion molecules. One model of transgenic mice in which the gene encoding the transcription factor Elf3 is disrupted have morphologic features resembling IED. Parental consanguinity and/or affected siblings suggest an autosomal recessive transmission but the causative gene(s have not been yet identified making prenatal diagnosis unavailable. Some infants have a milder phenotype than others but in most patients, the severity of the intestinal malabsorption even with enteral feeding make them totally dependent on daily long-term parenteral nutrition with a subsequent

  7. Technology of physical rehabilitation of children with bronchopulmonary diseases in the conditions of hospitalization

    Directory of Open Access Journals (Sweden)

    Nataliya Ivasyk

    2016-10-01

    Full Text Available Purpose: the development of technology of physical rehabilitation of children with bronchopulmonary diseases. Material & Methods: general scientific – analysis, conception and generalization, synthesis, comparison, abstraction. Results: technology of physical rehabilitation for children with bronchopulmonary diseases (BD, which is directed to the restoration and the development of physiological functions of a child, the prevention of synchronization of sharp processes, the elimination of negative changes in health of a child, the assistance in creation of conditions for adaptation to changes as a result of disease in life situations, is offered. Conclusions: the program and its activity including basic and variable components is the compound of the offered technology of physical rehabilitation for children with BD in the conditions of hospitalization.

  8. Early diagnosis of multiple epiphyseal dysplasia.

    Science.gov (United States)

    Ingram, R R

    1992-01-01

    In 20 children with multiple epiphyseal dysplasia, an objective assessment of epiphyseal size was made to determine its value in early diagnosis of this condition. All had wrist radiographs taken at age less than 15 years, and 16 children had knee radiographs. Before the epiphyses become fully ossified, abnormalities of size and shape may be difficult to assess, but carpal length/width ratios were abnormal in 60%, and distal femoral epiphyseal/metaphyseal ratios were abnormal in 56%. When both measurements were combined, 80% could be classified as abnormal. Objective radiologic assessment of epiphyses is of value in early diagnosis of multiple epiphyseal dysplasia.

  9. Cleidocranial dysplasia: Etiology, clinicoradiological presentation and management

    Directory of Open Access Journals (Sweden)

    Serhat Köseoğlu

    2012-03-01

    Full Text Available Cleidocranial dysplasia (CCD is an autosomal dominantskeletal dysplasia characterised by abnormal clavicles,patent sutures and fontanelles, supernumerary teeth,short stature, and a variety of other skeletal change. Cleidocranialdysplasia is caused by mutation in the geneon 6p21 encoding transcription factor CBFA1, i.e. runtrelatedtranscription factor 2 (RUNX2. Individuals withCCD should be followed by either a team of specialist orby individual specialist familiar with the problems that canbe associated with this condition. J Clin Exp Invest 2012;3(1: 133-136

  10. Camptomelic dysplasia: prenatal diagnosis by ultrasound.

    Science.gov (United States)

    Natasha, Gupta; Ghai, Rajeev; Shah, Dheeraj; Kiran, P S

    2006-09-01

    We report a case of camptomelic dysplasia, an extremely rare lethal congenital bony dysplasia with an incidence of about 2 per million live births. Diagnosis was made antenatally at a gestational age of about 25 weeks by sonographic demonstration of anterior bowing of long bones, hypoplastic scapulae, bilateral talipes equinovarus, and normally ossified unfractured ribs. The mother elected to terminate the pregnancy, and the diagnosis was confirmed on clinical and radiographic examination of the fetus. Radiological features and differential diagnoses of this entity are discussed.

  11. Therapy in Bronchopulmonary Diseases Associated with Increased Secretion of Viscous Sputum and Impaired Mucus Transport

    Directory of Open Access Journals (Sweden)

    Yu.V. Marushko

    2015-02-01

    Full Text Available The article deals with the problem of cough in bronchopulmonary diseases, in particular, the attention is paid to cough with increased secretion of viscous sputum, i.e., productive cough. It is noticed that derivative of vasicine alkaloid — ambroxol belongs to effective secretolytic mucoactive drugs. A review of researches on ambroxol efficacy in pediatrics is provided, the characteristics of Ambrolitin manufactured by European pharmaceutical company «Sopharma», which active ingredient is ambroxol, are given.

  12. The mathematical pathogenetic factors analysis of acute inflammatory diseases development of bronchopulmonary system among infants

    Directory of Open Access Journals (Sweden)

    G. O. Lezhenko

    2017-10-01

    Full Text Available The purpose. To study the factor structure and to establish the associative interaction of pathogenetic links of acute diseases development of the bronchopulmonary system in infants.Materials and methods. The examination group consisted of 59 infants (average age 13.8 ± 1.4 months sick with acute inflammatory bronchopulmonary diseases. Also we tested the level of 25-hydroxyvitamin D (25(ОНD, vitamin D-binding protein, hBPI, cathelicidin LL-37, ß1-defensins, lactoferrin in blood serum with the help of immunoenzymometric analysis. Selection of prognostically important pathogenetic factors of acute bronchopulmonary disease among infants was conducted using ROC-analysis. The procedure for classifying objects was carried out using Hierarchical Cluster Analysis by the method of Centroid-based clustering. Results. Based on the results of the ROC-analysis were selected 15 potential predictors of the development of acute inflammatory diseases of the bronchopulmonary system among infants. The factor analysis made it possible to determine the 6 main components . The biggest influence in the development of the disease was made by "the anemia factor", "the factor of inflammation", "the maternal factor", "the vitamin D supply factor", "the immune factor" and "the phosphorus-calcium exchange factor” with a factor load of more than 0.6. The performed procedure of hierarchical cluster analysis confirmed the initial role of immuno-inflammatory components. The conclusions. The highlighted factors allowed to define a group of parameters, that must be influenced to achieve a maximum effect in carrying out preventive and therapeutic measures. First of all, it is necessary to influence the "the anemia factor" and "the calcium exchange factor", as well as the "the vitamin D supply factor". In other words, to correct vitamin D deficiency and carry out measures aimed at preventing the development of anemia. The prevention and treatment of the pathological course of

  13. PROPHYLAXIS OF ACUTE RESPIRATORY TRACT INFECTIONS IN CHILDREN WITH RECURRENT BRONCHOPULMONARY DISORDERS

    Directory of Open Access Journals (Sweden)

    A. Yu. Simonova

    2013-01-01

    Full Text Available One of the most urgent problems of Russian pediatrics — high prevalence of acute respiratory infections — is analyzed in this article. The author characterizes a special group of «frequently ill children». Patients of this group are the most prone to recurrent bronchopulmonary diseases, due to special features of their immunological statuses. The article also contains a short literature review on pidotimod trials, which have proved this drug to be effective and safe in acute and recurrent bronchopulmonary diseases as well as in bronchial asthma. In the patients who were administered pidotimod the frequency of relapses of acute respiratory tract infections decreased, the duration of the disease course shortened significantly, as well as these patients required antibacterial and antifebrile agents more rarely and did not have complications of allergic diseases. Children with recurrent bronchopulmonary diseases and bronchial asthma receiving pidotimod were shown to have lower rate of relapses and normalization of immunological characteristics. It is important to mention that pidotimod do not affect results of peak flowmetry and improve results of «Asthma Control Test». Pidotimod usage during vaccination guaranteed uneventful course of post-vaccination period and stimulation of immune response. Long-term study of clinical efficacy and safety of pidotimod allowed to recommend this drug as preventive and medicinal measure in pediatric practice.

  14. Craniometaphyseal Dysplasia: A review and novel oral manifestation.

    Science.gov (United States)

    Martin, K; Nathwani, S; Bunyan, R

    2017-01-01

    Craniometaphyseal Dysplasia (CMD) is a sclerosing osseous dysplasia characterised by hyperostosis of craniofacial and long bones, resulting in distortion and cranial nerve palsies. We present a case report on the management of a 63 year old female with Craniometaphyseal Dysplasia. This report describes an additional clinical manifestation of hypercementosis, which although well recognised in other sclerosing osseous dysplasias, is not reported in the literature for Craniometaphyseal Dysplasia. We discuss established in vivo studies in mice which link the genetic mutations found in Craniometaphyseal Dysplasia to hypercementosis, and how this report describes the same manifestation in humans. This novel finding can aid the clinician in the management of patients with Craniometaphyseal Dysplasia, and complications that can arise in dentoalveolar surgery.

  15. Cleidocranial Dysplasia with Autosomal Dominant Inheritance Pattern

    African Journals Online (AJOL)

    Cleidocranial dysplasia (CCD) is an autosomal dominant disease with a wide range of expression, characterized by clavicular hypoplasia, retarded cranial ossification, delayed bone and teeth development, supernumerary teeth, stomatognathic, craniofacial and skeletal abnormalities. This paper presents a case of CCD in ...

  16. Arrythmogenic right ventricular dysplasia/cardiomyopathy | Scholtz ...

    African Journals Online (AJOL)

    Arrythmogenic right ventricular dysplasia/cardiomyopathy (ARVD) is a familial cardiomyopathy characterised clinically by right ventricular (RV) dysfunction as well ... can occur and appears to correlate with increased disease severity.3,4 Owing to the complexity of the disease, Task Force Criteria for diagnosis of ARVD were ...

  17. A new lethal sclerosing bone dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Kingston, H.M. (Saint Mary' s Hospital, Manchester (UK). Regional Genetics Centre); Freeman, J.S. (Tameside General Hospital, Ashton-under-Lyne (UK). Dept. of Paediatrics); Hall, C.M. (Hospital for Sick Children, London (UK). Dept. of Paediatric Radiology)

    1991-02-01

    A neonate is described with a lethal sclerosing bone dysplasia associated with prenatal fractures and craniofacial abnormalities including microcephaly, exophthalmos, hypoplastic nose and mid-face, small jaw and nodular hyperplasia of the gums. Parental consanguinity suggests that an autosomal recessive mutation is the likely aetiology. (orig.).

  18. Screening for Developmental Dysplasia of the Hip

    NARCIS (Netherlands)

    Boere-Boonekamp, Magdalena M.; Verkerk, Paul H.

    1998-01-01

    The success rates of screening programmes for Developmental Dysplasia of the Hip (DDH) vary widely. Studies on screening programmes for DDH based on a Medline search for the years 1966–1997 are reviewed. The percentage treated in most studies, especially those using ultrasound, are high and suggest

  19. Fibrous dysplasia of the jaws in Nigerians.

    Science.gov (United States)

    Daramola, J O; Ajagbe, H A; Obisesan, A A; Lagundoye, S B; Oluwasanmi, J O

    1976-09-01

    The clinicoradiologic features and surgical treatment of fibrous dysplasia of the jaws in forty-seven Nigerian patients are described. Marked facial deformity and functional disturbances were the main indications for operation. We noticed one case of malignant transformation. It is our opinion that a more-than-usual aggressive excision is indicated in Nigerian patients.

  20. Dietary vitamin C and uterine cervical dysplasia.

    Science.gov (United States)

    Wassertheil-Smoller, S; Romney, S L; Wylie-Rosett, J; Slagle, S; Miller, G; Lucido, D; Duttagupta, C; Palan, P R

    1981-11-01

    A case-control study of women with cervical abnormalities identified through Pap smears, was conducted in the Bronx, New York, to explore the relationship between nutritional intake and cervical dysplasia. Nutrient intake was estimated from computer analysis of three-day food records and 24-hour recall for 169 study participants (87 cases, 82 controls), including a subset of 49 pairs matched for age, race and parity. Mean vitamin C intake per day from three-day food record for controls was 107 mg, compared to 80 mg for cases (p less than 0.01). Analysis of matched pairs showed similar results; 29% of cases compared to 3% of controls in matched subset had vitamin C intake less than 50% of the recommended daily allowance, yielding a ten-fold increase in risk of cervical dysplasia as estimated by odds ratio (p less than 0.05). Younger age, greater frequency of sexual intercourse and younger age at first intercourse were associated with higher risk of cervical dysplasia. Multiple logistic analyses indicated that low vitamin C intake is an independent contributor to risk of severe cervical dysplasia when age and sexual activity variables are controlled. Approximately 35% of US women in their reproductive years have daily vitamin C intake below 30 mg, and 68% have vitamin C intake below 88 mg. If other studies confirm these findings, it may be important to explore a possible protective role of supplementary vitamin C for women at high risk of cervical cancer.

  1. Simplified Classification of Focal Cortical Dysplasia

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2002-09-01

    Full Text Available Sections of cortex from 52 of 224 (23% patients with cortical dysplasia, operated on for drug-resistant partial epilepsy, were retrospectively re-examined histologically at Niguarda Hospital, and Istituto Nazionale Neurologico ‘C. Besta’, Milan, Italy.

  2. Frontofacionasal dysplasia: another observation | Shawky | Egyptian ...

    African Journals Online (AJOL)

    ... deformed nostrils, hypoplastic nasal wing, cleft lip, cleft palate and meningeocele. This association of anomalies suggests the diagnosis of frontofacionasal dysplasia and in our case is associated with facial heamangioma. To our knowledge, facial heamangioma in association with FFND have not been described before

  3. An individual program planning model of physical rehabilitation/therapy of a child with a bronchopulmonary disease

    Directory of Open Access Journals (Sweden)

    Nataliya Ivasyk

    2017-04-01

    Full Text Available Purpose: create a model of planning an individual program of physical rehabilitation/therapy of children with bronchopulmonary diseases. Material & Methods: general scientific – analysis, interpretation and synthesis, synthesis, comparing, abstracting. Results: a model of planning an individual program of physical rehabilitation/therapy of children with bronchopulmonary disease, which consists of five interrelated functional subsystems. Conclusion: the proposed model takes into consideration the clinical and functional examination of the data, based on which are determined by the problem, the aim is and the problem of physical rehabilitation, according to which the selected means, forms and methods of influence, the methods of their implementation and dosing criteria in accordance with the individual data of a child with bronchopulmonary disease. This is accomplished by changes in the order, content and structure interference in accordance with changes in the patient's condition.

  4. Joint space width in dysplasia of the hip

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Sonne-Holm, Stig; Søballe, K

    2005-01-01

    . Neither subjects with dysplasia nor controls had radiological signs of ongoing degenerative disease at admission. The primary radiological discriminator of degeneration of the hip was a change in the minimum joint space width over time. There were no significant differences between these with dysplasia...... and controls in regard to age, body mass index or occupational exposure to daily repeated lifting at admission.We found no significant differences in the reduction of the joint space width at follow-up between subjects with dysplasia and the control subjects nor in self-reported pain in the hip......In a longitudinal case-control study, we followed 81 subjects with dysplasia of the hip and 136 control subjects without dysplasia for ten years assessing radiological evidence of degeneration of the hip at admission and follow-up. There were no cases of subluxation in the group with dysplasia...

  5. Ocular symptoms and signs in patients with ectodermal dysplasia syndromes.

    Science.gov (United States)

    Kaercher, T

    2004-06-01

    The ectodermal dysplasia syndromes are underestimated although precise inclusion criteria have been formulated. The purpose is to establish easily detectable ophthalmologic symptoms and signs as reliable criteria for ectodermal dysplasia syndromes. Thirty-six patients with confirmed ectodermal dysplasia syndromes were included in an observational case series: hypohidrotic ectodermal dysplasia (30), EEC syndrome (3), AEC syndrome (2), Gorlin-Goltz syndrome (1). Each patient was examined ophthalmologically. The principal outcome measures were ocular symptoms and signs in patients with different ectodermal dysplasia syndromes of varying severity. Some 94.4% of the patients suffered from dry eye symptoms. Reduction of eyebrows was seen in 94.4%; the lashes were altered in 91.6%. Changes of the meibomian glands were detected in 95.45%. Corneal changes such as pannus occurred later in life. Alterations of the meibomian glands, which were detected by meibomianoscopy, are the most reliable ocular sign of ectodermal dysplasia syndromes.

  6. Bronchopulmonary Neuroendocrine Neoplasms and Their Precursor Lesions in Multiple Endocrine Neoplasia Type 1.

    Science.gov (United States)

    Bartsch, Detlef K; Albers, Max B; Lopez, Caroline L; Apitzsch, Jonas C; Walthers, Eduard M; Fink, Ludger; Fendrich, Volker; Slater, Emily P; Waldmann, Jens; Anlauf, Martin

    2016-01-01

    The prevalence and clinical behavior of bronchopulmonary neuroendocrine tumors (bNET) associated with multiple endocrine neoplasia type 1 (MEN1) are not well defined. This study aimed to determine the prevalence, potential precursor lesions and prognosis of bNET in patients with MEN1. A database of 75 prospectively collected MEN1 cases was retrospectively analyzed for bNET. Patient characteristics, imaging and treatment were evaluated. Resection specimens of operated patients were reassessed by two specialized pathologists. Available CT scans of the whole cohort were reviewed to determine the prevalence of bronchopulmonary nodules. Five of the 75 MEN1 patients (6.6%; 2 male, 3 female) developed histologically confirmed bNET after a median follow-up of 134 months. The median age at diagnosis of bNET was 47 years (range 31-67), and all patients were asymptomatic. Four patients underwent anatomic lung resections with lymphadenectomy; the remaining patient with multiple lesions had only a wedge resection of the largest bNET. Tumor sizes ranged from 7 to 32 mm in diameter, and all bNET were well differentiated. Two patients had lymph node metastases. Two of 4 reevaluated resection specimens revealed multifocal bNET, and 3 specimens showed tumorlets (up to 3) associated with multifocal areas of a neuroendocrine cell hyperplasia within the subsegmental bronchi. One bNET-related death (1.3%) occurred during long-term follow-up. Review of the available CT scans of the patients without proven bNET revealed small bronchopulmonary lesions (≥3 mm) in 16 of 53 cases (30.2%). bNET in MEN1 might be more common than previously recognized. Their natural course seems to be rather benign. Multifocal tumorlets and multifocal neuroendocrine cell hyperplasia might represent their precursor lesions. © 2015 S. Karger AG, Basel.

  7. Chest radiographic staging in allergic bronchopulmonary aspergillosis: relationship with immunological findings.

    LENUS (Irish Health Repository)

    Kiely, J L

    2012-02-03

    The question of whether a chest radiographic severity staging system could be correlated with standard blood\\/serum diagnostic indices in allergic bronchopulmonary aspergillosis (ABPA) was addressed in 41 patients. Asthma and positive Aspergillus fumigatus (AF) serology were considered essential diagnostic inclusion criteria. Eosinophil count, serum immunoglobulin (Ig)E and immediate skin hypersensitivity were also tested to grade patients as "definite" or "likely" ABPA. Definite cases had all five of these factors present, whereas likely cases had three or more. Chest radiographs were examined by experienced radiologists blinded to the clinical data. The six-stage radiographic score (0-5) was based on the severity and duration of changes seen: stage 0: normal; stage 1: transient hyperinflation; stage 2: transient minor changes; stage 3: transient major changes; stage 4: permanent minor changes; and stage 5: permanent major changes. Significant positive correlations (p<0.05) were observed between peak AF titres (expressed as an index), peak eosinophil count and radiographic severity stage. When considered as subgroups, these correlations approached, but did not reach, significance for the group with "likely" ABPA (n=28), but in the group with definite ABPA (n=13), there was a high correlation between radiographic score and peak AF index (r=0.59), as well as peak eosinophil count (r=0.62). This study suggests that the peak Aspergillus fumigatus index and eosinophil counts correlate best with the severity of radiographic stages in allergic bronchopulmonary aspergillosis. This chest radiographic staging system may be useful in the clinical assessment and management of patients with allergic bronchopulmonary aspergillosis, particularly in those patients with more severe radiographic stages.

  8. Incidental Finding of Bronchopulmonary Sequestration in a 64-Year-Old Female.

    Science.gov (United States)

    Tunsupon, Pichapong; Arshad, Ayesha; Patel, Sumit; Mador, M Jeffery

    2017-01-01

    Bronchopulmonary sequestration is a congenital abnormality of the primitive foregut. In adults, the typical age at presentation is 20-25 years. A 64-year-old female was referred for evaluation of an 8 × 6-cm right lower lobe cystic lesion. Her medical history was significant for recurrent right lower lobe pneumonia requiring multiple hospitalizations. Her physical examination was significant for crackles at the right lung base. Computed tomography (CT) of the chest with contrast showed cystic changes with thickened septation of the medial segment of the right lower lobe lacking distinct visceral pleura and with arterial supply from the anomalous branch of the thoracic aorta arising near the celiac trunk. Pulmonary angiography confirmed the diagnosis of intralobar pulmonary sequestration. The patient underwent celiac endovascular coil embolization of the anomalous artery to lessen the risk of hemorrhage prior to video-assisted thoracoscopic surgery (VATS) resection of the right lower lobe. She recovered well and was discharged home 1 week after VATS lobectomy. Follow-up CT of the chest 2 months later showed normal postsurgical changes related to right lower lobe lobectomy. The patient remained asymptomatic and resumed her daily activities. Pulmonary sequestration can present with recurrent pneumonia in late adulthood. Physicians must review any previous imaging studies of the chest to identify the structural abnormality and be cognizant of differential diagnoses such as infected cystic bronchiectasis, bronchogenic cyst, congenital diaphragmatic hernia, or cystic adenomatoid malformation that can occur in conjunction with bronchopulmonary sequestration. Pulmonary angiogram is the gold standard to confirm the diagnosis of bronchopulmonary sequestration. Surgical resection is the standard of care.

  9. Prosthodontic management of anhidrotic ectodermal dysplasia

    Directory of Open Access Journals (Sweden)

    Shilpy Gupta

    2011-01-01

    Full Text Available Ectodermal dysplasia is characterized by the absence or defects of two or more ectodermally derived structures. Anodontia or hypodontia is the most striking dental manifestation. In severe hypodontia, there is lack of alveolar development with consequent protrusion and eversion of the lips. Patients with anhidrotic forms suffer from heat intolerance due to lack of sweat glands and mild infections may lead to death in infancy from hyperthermia. A case of a 4-year-old child with anhidrotic ectodermal dysplasia with partial anodontia is presented. Dental, oral, and physical features were taken into consideration in diagnosis and treatment planning for this patient. Clinical management consisted of removable partial prosthesis in maxillary arch and complete denture prosthesis in mandibular arch. The main aim of the treatment was to improve psychological development and to promote better functioning of the stomatognathic system.

  10. Arrhythmogenic right ventricular dysplasia: A case report

    Directory of Open Access Journals (Sweden)

    Tessa Negrín Valdés

    2015-10-01

    Full Text Available Arrhythmogenic right ventricular dysplasia is a heart muscle disease that predominantly affects the right ventricle, bringing about the replacement of normal myocardium with fatty or fibrofatty tissue and causing sudden death in young individuals. Ventricular tachycardia is an important clinical manifestation, although there are reports of right or global heart failure. The diagnosis is confirmed by echocardiography and magnetic resonance imaging. The case of a 65-year-old former smoker, with hypertension and ischemic heart disease, a history of effort syncope symptoms and proven non-sustained ventricular tachycardia, with morphology of left bundle branch block, is reported. Relevant diagnostic studies were performed, and echocardiographic elements which were compatible with arrhythmogenic right ventricular dysplasia were found. Therefore, an implantable cardioverter defibrillator was implanted, after which the patient has had a favorable outcome.

  11. Cleidocranial Dysplasia: Report of a Case

    Energy Technology Data Exchange (ETDEWEB)

    Jeong, Seon Jin; Hong, Soon Ki [Dept. of Oral and Maxillofacial Radiology, College of Dentistry, Chosun University, Kwangju (Korea, Republic of)

    2000-09-15

    Cleidocranial dysplasia (previously known as cleidocranial dysostosis) is a well-known, rare and hereditary skeletal disorder characterized by a variety of dental abnormalities and as its name implies, striking involvement of the cranial vaults and clavicles. A 17-year-old female who presented with short stature and prolonged retention of deciduous teeth, subsequent delay in eruption of permanent teeth is described. She could touch her shoulders together at the midline anteriorly. Diagnostic procedures showed hypoplasia of the maxillary and zygomatic bones, open fontanelles and sutures, and aplasia of the clavicles. The paranasal sinuses were absent or underdeveloped. Characteristically, she had near parallel-sided borders in the ascending ramus of the mandible and abnormal-shaped, the slender pointed coronoid process. The zygomatic arches had a downward bend and discontinuity at the zygomaticotemporal suture area. Radiographic and clinical investigations of her cranial and skeletal abnormalities revealed features of cleidocranial dysplasia.

  12. Short rib polydactyly syndrome: lethal skeletal dysplasia

    OpenAIRE

    Huertas, Erasmo; 1 Instituto Nacional Materno Perinatal. Lima, Perú. 2 Facultad de Medicina, Universidad Nacional Mayor de San Marcos. Lima, Perú.; Íngar, Jaime; 1 Instituto Nacional Materno Perinatal. Lima, Perú. 2 Facultad de Medicina, Universidad Particular San Martín de Porres. Lima, Perú.; Gutiérrez, Guiselle; 1 Instituto Nacional Materno Perinatal. Lima, Perú. 2 Facultad de Medicina, Universidad Nacional Mayor de San Marcos. Lima, Perú.; Quiñones, Eva María; Facultad de Medicina, Universidad Particular San Martín de Porres. Lima, Perú.

    2011-01-01

    Short rib-polydactyly syndrome is a descriptive category for a group of lethal skeletal dysplasias characterized by hypoplastic thorax, short ribs, short limbs, polydactyly, and visceral abnormalities. The 4 established variants are SRPS I (Saldino-Noonan type), SRPS II (Majewski type; 263520), SRPS III (Verma-Naumoff type; 263510), and SRPS IV (Beemer-Langer type; 269860). All variants are thought to be inherited in autosomal recessive pattern. Because of the frequent phenotype overlap there...

  13. MR imaging features of craniodiaphyseal dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Marden, Franklin A. [Mallinckrodt Institute of Radiology, Washington University Medical Center, 510 South Kingshighway Blvd., MO 63110, St. Louis (United States); Department of Radiology, St. Louis Children' s Hospital, Children' s Place, MO 63110, St. Louis (United States); Wippold, Franz J. [Mallinckrodt Institute of Radiology, Washington University Medical Center, 510 South Kingshighway Blvd., MO 63110, St. Louis (United States); Department of Radiology, St. Louis Children' s Hospital, Children' s Place, MO 63110, St. Louis (United States); Department of Radiology/Nuclear Medicine, F. Edward Hebert School of Medicine, Uniformed Services University of the Health Sciences, MD 20814, Bethesda (United States)

    2004-02-01

    We report the magnetic resonance (MR) imaging findings in a 4-year-old girl with characteristic radiographic and computed tomography (CT) features of craniodiaphyseal dysplasia. MR imaging exquisitely depicted cranial nerve compression, small foramen magnum, hydrocephalus, and other intracranial complications of this syndrome. A syrinx of the cervical spinal cord was demonstrated. We suggest that MR imaging become a routine component of the evaluation of these patients. (orig.)

  14. Fetal MR imaging of Kniest dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Yazici, Zeynep [Uludag University, Faculty of Medicine, Department of Radiology, Gorukle (Turkey); Kline-Fath, Beth M.; Laor, Tal [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Tinkle, Bradley T. [Cincinnati Children' s Hospital Medical Center, Division of Human Genetics, Cincinnati, OH (United States)

    2010-03-15

    We present a case of Kniest dysplasia, a rare form of the type II collagenopathies, with prenatal MRI. Sonography revealed only short limbs in the fetus. Fetal MRI findings included enlarged hyaline cartilaginous structures with abnormally high T2 signal intensity, delayed ossification of the pubic and ischial bones, and platyspondyly. By delineating the cartilaginous abnormalities, fetal MRI can contribute to the prenatal diagnosis of chondrodysplasias. (orig.)

  15. Distinctive skeletal dysplasia in Cockayne syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Silengo, M.C.; Franceschini, P.; Bianco, R.; Biagioli, M.; Pastorin, L.; Vista, N.; Baldassar, A.; Benso, L.

    1986-03-01

    Cockayne syndrome is a well-known autosomal recessive form of dwarfism with senile-like appearance. Skeletal changes such as flattening of vertebral bodies, ivory epiphyses and thickening of cranial vault, have been observed in some patients with this condition. We describe here a 5.5-year-old girl with the typical clinical signs of Cockayne syndrome and a distinctive form of bone dysplasia with major involvement of the spine.

  16. Epilepsy, Acquired Aphasia with Focal Cortical Dysplasia

    Directory of Open Access Journals (Sweden)

    Girija A.S

    1999-01-01

    Full Text Available A six year old boy having complex partial seizures with secondary generalization of four months duration developing isolated expressive dysphasia, later progressing to global aphasia is being reported. His awake EEG showed a left temporal spike wave discharge and sleep EEG showed continuous spike and ware discharges. MR imaging demonstrated focal cortical dysplasia in the left frontal and opercular region, a combination that has not been reported earlier.

  17. DNA content in human uterine cervical dysplasias.

    Science.gov (United States)

    Palan, P R; Romney, S L

    1979-01-01

    The DNA content of nuclei extracted from separate biopsies of normal cervical epithelium and from dysplastic sites in the same patient was determined biochemically. Mean percentage increase in the DNA content of dysplasias (mild: 143.3 +/- 14.9; moderate: 225.8 +/- 51.1; and severe: 359.8 +/- 45.8) was found to be statistically significant (F-Test: p less than 0.001) over the normal values in control cervical tissues.

  18. Osteochondral Allograft Transplantation for Femoral Trochlear Dysplasia

    Science.gov (United States)

    Vansadia, Dharmpal V.; Heltsley, James R.; Montgomery, Scott; Suri, Misty; Jones, Deryk G.

    2016-01-01

    Background: The risk factors for patellofemoral joint instability include laxity of medial patellar restraints, abnormal limb geometry, femoral and tibial malrotation, patella alta, and trochlear dysplasia. Femoral trochlear dysplasia is characterized by a hypoplastic or shallow trochlear groove. Case Report: We report the case of a 31-year-old female with trochlear dysplasia and recurrent patella dislocations, laxity of the medial patellofemoral ligament (MPFL), and high-grade chondromalacia of the trochlea and the patella. Surgical treatment goals were to re-create a trochlear groove, restore bony restraint, and realign and offload the patella. First, a triplane tibial tubercle osteotomy (TTO) was performed, and the patella was everted 360° with a subvastus approach. The MPFL was reconstructed using a gracilis allograft. A fresh osteochondral allograft transplant trochlea was sized, and a 35-mm diameter graft was transplanted to re-create the groove. The TTO was secured in a new anterior, medial, and distal position. The patient was braced for 6 weeks and completed a rehabilitation protocol. At 9-month follow-up, she had made significant gains in range of motion (0°-140°) and activity compared to her preoperative status. She reported no pain or recurrent dislocations. Conclusion: This case demonstrates a viable surgical option for treatment of instability resulting from trochlear dysplasia with patellofemoral chondromalacia. The osteochondral allograft transplantation surgery technique allows patients to have a stable, pain-free knee joint and participate in activities compared to nonoperative management. However, the long-term outcomes of this procedure are unknown. PMID:27999505

  19. Intralobar bronchopulmonary sequestration evaluated by contrast-enhanced three-dimensional MR angiography

    Energy Technology Data Exchange (ETDEWEB)

    Kouchi, K.; Yoshida, H.; Matsunaga, T.; Ohtsuka, Y.; Kuroda, H.; Hishiki, T.; Satou, Y.; Terui, K.; Mitsunaga, T.; Ohnuma, N. [Department of Paediatric Surgery, School of Medicine, Chiba Univ. (Japan)

    2000-11-01

    Bronchopulmonary sequestration (PS) is characterized by non-functioning lung tissue fed from one or several aberrant systemic arteries. The condition is diagnosed by visualizing the feeding arteries using non-invasive CT, MRI, colour Doppler sonography or conventional angiography. We present a 5-year-old boy in whom intralobar sequestration was diagnosed using contrast-enhanced 3D MR angiography, which visualised fine blood vessels in the thoraco-abdominal region without arterial puncture. This technique is useful for diagnosing PS. (orig.)

  20. Voriconazole in the treatment of allergic bronchopulmonary aspergillosis in cystic fibrosis.

    LENUS (Irish Health Repository)

    Glackin, L

    2009-01-01

    Allergic bronchopulmonary aspergillosis (ABPA) can cause a significant clinical deterioration in patients with cystic fibrosis. There is very little research in the current literature with regard to alternatives for treatment, apart from long courses of steroids. We conducted a retrospective review of all our patients with ABPA treated with the antifungal voriconazole and found there was a significant drop in IgE levels post treatment as well as a decrease in steroid dosing. The improvement in FEV was not statistically significant; however there was a very wide variation in pre-treatment levels.

  1. Ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome

    OpenAIRE

    Reema Sharma Dhar; Amitava Bora

    2014-01-01

    Ectrodactyly-ectodermal dysplasia-cleft (EEC) syndrome is an autosomal dominant disorder characterized by the triad of ectrodactyly-ectodermal dysplasia, and facial clefting along with some associated features. Presence of all the three major features in a single individual is extremely rare. We report a case of 4 year 11 months old child with EEC syndrome having ectodermal dysplasia-cleft lip and cleft palate and ectrodactyly with some associated features. Clinical features, diagnosis and ro...

  2. Florid osseous dysplasia of the jaws

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Su Beom; Koh, Kwang Joon [Dept. of Oral Radiology, College of Dentistry, Chonbuk National University, Chonju (Korea, Republic of)

    1995-02-15

    Few cases of florid osseous dysplasia has been described as a condition that characteristically affects the jaws. It usually manifests as multiple radiopaque masses distributed throughout the jaws. Confusion exists about the relationship of florid osseous dysplasia, gigantiform cementoma, chronic sclerosing osteomyelitis, sclerosing osteitis or multiple enostosis. Authors experienced a case of florid osseous dysplasia of the jaws in 52-year-old female on the basis of clinical, radiographic and histopathologic findings. The characteristic features are as follows: 1. In clinical examination, there was no clinical sign and symptoms except extrated area. And there was no facial asymmetry. 2. Radiograms show round or lobular dense radiopaque masses surrounded by radiolucent bands in lower molar teeth area bilaterally. And slight increased radiopacities in maxillary molar teeth area bilaterllay. There was no expansion or thinning of buccal and lingual cortical bones. There is no displacement or resorption of involved teeth. In right side of mandible, mandibular canal is displaced inferiorly due to mass. 3. Photomicrograms show densely mineralized sclerotic acellular masses with empty lacunae. Pattern is suggestive of cementum, although it could be considered sclerotic bone. In the periphery, lesion consisting of moderately cellular fibrous tissue in calcified products are deposited.

  3. Bizarre cell dysplasia of the cervix.

    Science.gov (United States)

    Ondič, Ondrej; Ferko, Radoslav; Kašpírková, Jana; Švajdler, Marián; Rýchly, Boris; Talarčík, Peter; Bouda, Jiří; Michal, Michal

    2017-02-01

    The aim of this study was the characterization of a new subtype of high-grade cervical squamous intraepithelial lesion (HSIL) with enlarged cells containing bizarre nuclei: so-called bizarre cell dysplasia (BCD). A total of 29 cervical cone biopsy samples of this type of dysplasia were studied. Multi-target polymerase chain reaction and in situ hybridization human papillomavirus (HPV) detection was performed in all cases. BCD was defined as a subtype of HSIL characterized by the presence of large dysplastic cells with abnormal, large pleomorphic nuclei or multinucleation causing nucleomegaly. This results in bizarre nuclear shapes. Bizarre cells are scattered throughout the whole thickness of the dysplastic squamous epithelium. The BCD lesions arise within the conventional/classic high grade or "bland" type squamous dysplasia HSIL. Statistically they were significantly associated with HVP type 16. A significant association with other studied viruses (Herpes simplex virus [HSV]1, HSV2, Varicella zoster virus, Epstein-Barr virus, cytomegalovirus, human herpesvirus 6, and human polyomaviruses BK and JC) was not confirmed. BCD involves cytologically characteristic morphologic changes that are recognizable, but which may pose some risk of misdiagnosis as low-grade squamous intraepithelial lesion due to the enlargement of dysplastic cells and multinucleation. Based on the unique histological, cytological and biological features of BCD including strong association with HPV 16 infection, we believe that this is a specific, and so far unrecognized variant of HSIL. © 2017 Japan Society of Obstetrics and Gynecology.

  4. Thanatophoric Dysplasia; a Rare Case Report on a Congenital Anomaly

    Directory of Open Access Journals (Sweden)

    Maria Francis Yuvaraj

    2017-01-01

    Full Text Available The rare form of skeletal dysplasia is thanatophoric dysplasia. The meaning for thanatophoric dysplasia is death bearing which is derived from Greek word. It occurs 1in 20,000 to 50,000. It is mainly due to mutations in the fibroblast growth factor receptor 3gene. Features of thanatophoric dysplasia are frontal bossing, prominent eyes, narrow thorax, protruded abdomen and bowed legs. The knowledge about this condition is useful in the fields of Anatomy, Paediatrics, Obstetrics and Gynaecology, Ultrasonagraphy and Genetics, for future research purpose.

  5. Focal cemento-osseous dysplasia: review and a case report.

    Science.gov (United States)

    Salem, Y M Y; Osman, Y I; Norval, E J G

    2010-10-01

    Focal cemento-osseous dysplasia is a benign fibro-osseous condition that can be seen in dentate and edentulous patients. It is an asymptomatic lesion and needs no treatment; however follow-up is essential due to the possibility that focal cemento-osseous dysplasia can progress to a condition called florid osseous dysplasia that involves multiple sites. A case report is presented here, along with a review of the differential diagnoses considered in order to reach a final diagnosis of focal cemento-osseous dysplasia.

  6. The Ectodermal Dysplasias : Severe Palmoplantar Hyperkeratosis And Chronic Angular Cheilitis

    Directory of Open Access Journals (Sweden)

    Mahajan Vikram K

    2003-01-01

    Full Text Available The ectodermal dysplasias are congenital, non-progressive and diffuse disorders affecting primarily the tissues derived from ectoderm. Over a period, their classification has become confusing due to indiscriminate use of them “ectodermal dysplasia” for numerous syndromes with a defect in one or more epidermal defect in each element of skin; their precise classification appears difficult as yet. Only X-linked recessive ectodermal dysplasia (Christ-Siemens-Touraine syndrome remains best defined. This paper describes three cases of ectodermal dysplasias highlighting their overlapping features.

  7. Osseous dysplasia (cemento-osseous dysplasia) of the jaw bones in western Pennsylvania patients: analysis of 35 cases.

    Science.gov (United States)

    Owosho, Adepitan A; Potluri, Anitha; Bilodeau, Elizabeth A

    2013-01-01

    The purpose of this study is to analyze the demographic, clinical, and radiographic presentations of osseous dysplasia of the jaws in western Pennsylvania patients and its associated complications. The clinical records and radiographs of patients diagnosed with osseous (cement-osseous) dysplasia were retrieved from the electronic health record of the University of Pittsburgh, School of Dental Medicine from 2007 to 2012. All cases were reviewed; the WHO criteria and classification for osseous dysplasia was used. Clinical and demographic data, radiographic findings, and final diagnoses were collected and analyzed. 35 cases of osseous dysplasia were retrieved over the six-year period.The majority (33) were females [94.3%], with ages ranging from 26 to 89 years, with a mean age of 53.9 years +/- standard deviation of 15.6 years, 32 [91.4%] were African Americans and 3 [8.6%] were Caucasians. 17 [48.6%] were florid osseous dysplasia, 13 [37.1%] periapical osseous dysplasia and 5 [14.3%] focal osseous dysplasia. Of the 35 patients only 8 [22.9%] patients were symptomatic. All florid osseous dysplasia patients were African American females, with 7 of the patients being symptomatic and the commonest symptom being pain. Also, all periapical osseous dysplasia patients were African Americans (12 females and 1 male), with 1 of the patients presenting with widening of the diastema. Of the focal osseous dysplasia patients, 3 were Caucasians and 2 African American (4 females and 1 male). The cases occurred mostly in African American females with a peak incidence in the fifth and sixth decades of life; most cases occurred in the mandible. The commonest form of osseous dysplasias was the florid osseous dysplasia which is most likely to present with symptoms.

  8. Bronchopulmonary nematode infection of Capra pyrenaica in the Sierra Nevada massif, Spain.

    Science.gov (United States)

    Alasaad, S; Morrondo, P; Dacal-Rivas, V; Soriguer, R C; Granados, J E; Serrano, E; Zhu, X Q; Rossi, L; Pérez, J M

    2009-10-14

    The present investigation examined the prevalence and abundance of bronchopulmonary nematodes in 213 randomly hunted Iberian ibexes (Capra pyrenaica) (87 females and 126 males) in the Sierra Nevada mountain range in Spain between 2003 and 2006. Post mortem examination revealed an overall prevalence of 72% for adult nematodes (Cystocaulus ocreatus 44%, Muellerius capillaris 44%, Protostrongylus sp. 40%, and Dictyocaulus filaria 4%). The abundances were 13.45+/-3.97, 5.18+/-2.49, 6.36+/-2.16, and 2.27+/-0.46, respectively. Protostrongylid adults showed similar infection rates, which were statistically different from that of D. filaria. 20% of the examined Iberian ibexes were infected by three protostrongylid nematodes species, 24% of C. pyrenaica were affected by two protostrongylid species, while infestations with only one protostrongylid species were detected in 20% of the examined animals. The overall prevalence of larvae nematodes in the examined animals was 100%, and the overall abundance (number of the first stage larvae per gram) was 86.45+/-20.63. There was a high correlation between the two sets of data (adults and larvae). Results of the present investigation provided foundation for the effective control of bronchopulmonary nematode infection in Iberian ibex.

  9. Allergic bronchopulmonary aspergillosis in patient with cystic fibrosis - a case report.

    Science.gov (United States)

    Ionescu, Marcela Daniela; Balgradean, Mihaela; Marcu, Veronica

    2014-12-01

    Asthma with allergic bronchopulmonary aspergillosis (ABPA), a hypersensitivity disease of the lungs due to an immune response to Aspergillus fumigattus (Af) antigens, is rarely seen in children, other than complicating cystic fibrosis. We present the case of a 14 - year- old female teenager with cystic fibrosis (CF), admitted in our hospital with respiratory failure and persistent cyanosis. Chest X-ray showed perihilar and upper lobes pulmonary infiltrates. Her airway cultures were positive for methicillin resistant staphilococcus aureus (MRSA) and non-mucoid Pseudomonas aeruginosa. She was prescribed intravenous antibiotherapy with ceftazidime and vancomycine (to which MRSA and Pseudomonas aeruginosa were susceptible). Pulmonary function testing (PFT) revealed severe obstructive lung disease. After ten days of intravenous antibiotics and first five days of corticosteroid, the patient's FEV1 was 68% of predicted. Total serum IgE and IgE antibodies to Aspergillus fumigatus were elevated. These results raised the possibility of allergic bronchopulmonary aspergillosis (ABPA). The possibility of ABPA should be considered in all pulmonary exacerbation and in order to determine if ABPA is developing or if an exacerbation is occurring, a serial monitoring of IgE levels should be performed.

  10. CYTOMORPHOLOGICAL EVALUATION AND PROGNOSIS OF BRONCHOPULMONARY COMPLICATIONS IN ACUTE AND EARLY PERIODS OF SPINAL CORD TRAUMA

    Directory of Open Access Journals (Sweden)

    I.A. Norkin

    2009-09-01

    Full Text Available There were investigated 50 cytological preparations after fibro-optic bronchoscopy of 10 patients with cervical spinal cord injuries. The dynamics of broncho-pulmonary complications of spinal cord injuries was estimated on the basis of cytological broncho-alveolar lavage fluid investigations. In the work there were used clinico-neurologic methods, radiological (computer tomography and magnetic resonance imaging, endoscopic (fibro-optic bronchoscopy and cytomorphological investigations. Cytomorphological investigations of broncho-alveolar lavage fluid were carried out on the 3-4, 7, 14, 30th days. Cellular composition of the broncho-alveolar wash-out (endopulmonary cytogramme was estimated by calculation of more than 100 cells in 3 fields of the immersion microscope coverage. Quantitative changes of cellular elements were taken into account with respect to normal cell amount. The results were analyzed according to the average out method. Quantitative changes of inflammatory elements in endopulmonary cytogramme were determined by the degree of endobronchitic manifestations and were corresponding to clinico-radiological picture of development of broncho-pulmonary complications in different periods of spinal cord injury

  11. Development of lung function in very low birth weight infants with or without bronchopulmonary dysplasia: Longitudinal assessment during the first 15 months of corrected age

    Directory of Open Access Journals (Sweden)

    Schmalisch Gerd

    2012-03-01

    Full Text Available Abstract Background Very low birth weight (VLBW infants ( Methods Comprehensive lung function assessment was performed at about 50, 70, and 100 weeks of postmenstrual age in 55 sedated VLBW infants (29 with former BPD [O2 supplementation was given at 36 weeks of gestational age] and 26 VLBW infants without BPD [controls]. Mean gestational age (26 vs. 29 weeks, birth weight (815 g vs. 1,125 g, and the proportion of infants requiring mechanical ventilation for ≥7 d (55% vs. 8%, differed significantly between BPD infants and controls. Results Both body weight and length, determined over time, were persistently lower in former BPD infants compared to controls, but no significant between-group differences were noted in respiratory rate, respiratory or airway resistance, functional residual capacity as determined by body plethysmography (FRCpleth, maximal expiratory flow at the FRC (V'max FRC, or blood gas (pO2, pCO2 levels. Tidal volume, minute ventilation, respiratory compliance, and FRC determined by SF6 multiple breath washout (representing the lung volume in actual communication with the airways were significantly lower in former BPD infants compared to controls. However, these differences became non-significant after normalization to body weight. Conclusions Although somatic growth and the development of some lung functional parameters lag in former BPD infants, the lung function of such infants appears to develop in line with that of non-BPD infants when a body weight correction is applied. Longitudinal lung function testing of preterm infants after discharge from hospital may help to identify former BPD infants at risk of incomplete recovery of respiratory function; such infants are at risk of later respiratory problems.

  12. Lethal skeletal dysplasia owing to double heterozygosity for achondroplasia and spondyloepiphyseal dysplasia congenita.

    Science.gov (United States)

    Young, I D; Ruggins, N R; Somers, J M; Zuccollo, J M; Rutter, N

    1992-01-01

    A male infant with lethal short limbed dwarfism is described. His father had spondyloepiphyseal dysplasia congenita and his mother had achondroplasia. It is believed that the infant inherited both of these disorders and that their combined effects resulted in early death owing primarily to severe pulmonary hypoplasia. Images PMID:1453438

  13. Brochopulmonary dysplasia: New high resolution computed tomography scorting system and correlation between the high resolution computed tomography score and clinical severity

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Sumi; Kim, Woo Sun; Cheon, Jung Eun; Kim, Han Suk; Lee, Whal; Jung, Ah Young; Kim, In One; Choi, Jung Hwan [Seoul National University College of Medicine, Seoul (KR)

    2013-04-15

    To develop an high resolution computed tomography (HRCT) scoring system for the assessment of bronchopulmonary dysplasia (BPD) and determine its usefulness as compared with the chest radiographic score. Forty-two very low-birth-weight preterm infants with BPD (25 male, 17 female) were prospectively evaluated with HRCT performed at the mean age of 39.1-week postmenstrual age. Clinical severity of BPD was categorized as mild, moderate or severe. The HRCT score (0-36) of each patient was the sum of the number of bronchopulmonary segments with 1) hyperaeration and 2) parenchymal lesions (linear lesions, segmental atelectasis, consolidation and architectural distortion), respectively. We compared the HRCT scores with the chest radiographic scores (the Toce system) in terms of correlation with clinical severity. The HRCT score had good interobserver (r = 0.969, p < 0.001) and intraobserver (r = 0.986, p < 0.001) reproducibility. The HRCT score showed better correlation (r = 0.646, p < 0.001) with the clinical severity of BPD than the chest radiographic score (r = 0.410, p = 0.007). The hyperaeration score showed better correlation (r = 0.738, p < 0.001) with the clinical severity of BPD than the parenchymal score (r = 0.523, p < 0.001). We have developed a new HRCT scoring system for BPD based on the quantitative evaluation of pulmonary abnormalities of BPD consisting of the hyperaeration score and the parenchymal score. The HRCT score shows better correlation with the clinical severity of BPD than the radiographic score.

  14. Dyssegmental dysplasias: clinical, radiographic, and morphologic evidence of heterogeneity.

    Science.gov (United States)

    Aleck, K A; Grix, A; Clericuzio, C; Kaplan, P; Adomian, G E; Lachman, R; Rimoin, D L

    1987-06-01

    The dyssegmental dysplasias are lethal forms of neonatal short-limbed dwarfism in which vertebral segmentation defects and short, thick, bowed long bones are the prominent radiographic features. Clinically, unusual facies, short neck, narrow thorax, cleft palate, and reduced joint mobility are commonly seen. To date, 18 cases of dyssegmental dysplasia have been reported. Reports of three pairs of affected sibs suggest autosomal recessive inheritance. We have studied eight additional cases of dyssegmental dysplasia, including one pair of affected sibs. Clinical, radiographic, and histologic examination of these new cases and review of the literature demonstrates the presence of at least two distinct forms of dyssegmental dysplasia. The milder form, "dyssegmental dysplasia, type Rolland-Desbuquois," is characterized clinically by frequent survival beyond the newborn period and by distinct radiographic changes resembling Kniest dysplasia. The severe form, "dyssegmental dysplasia, type Silverman-Handmarker," is characterized by stillbirth or death within the first few days of life and by distinct and more severe radiographic changes. In addition, we have demonstrated chondro-osseous morphologic differences between the two disorders by light and electron microscopy. We conclude that there are at least two forms of dyssegmental dysplasia, each autosomal recessive, which can be delineated on clinical, radiographic and morphologic grounds.

  15. Marfan syndrome with multiseptate pneumothorax and mandibular fibrous dysplasia

    Directory of Open Access Journals (Sweden)

    Kate A

    2009-01-01

    Full Text Available We describe a rare case of pneumothorax due to Marfan syndrome associated with fibrous dysplasia of the mandible. Marfan syndrome and fibrous dysplasia were possibly due to a common etiological factor. The association between the two and other tumors described in literature related to Marfan syndrome is discussed.

  16. Whole saliva in X-linked hypohidrotic ectodermal dysplasia

    DEFF Research Database (Denmark)

    Lexner, Michala Oron; Bardow, Allan; Hertz, Jens Michael

    2007-01-01

    BACKGROUND: X-linked hypohidrotic ectodermal dysplasia (HED) is the most common type of ectodermal dysplasia. Identification of female carriers of X-linked HED can be difficult because of varying degrees of clinical symptoms due to the X-chromosome inactivation. This is the first study about whol...

  17. Anomalies of tooth formation in hypohidrotic ectodermal dysplasia

    DEFF Research Database (Denmark)

    Lexner, Michala O; Bardow, Allan; Hertz, Jens Michael

    2007-01-01

    OBJECTIVE: The X-linked hypohidrotic ectodermal dysplasia (HED) is the most common type of ectodermal dysplasia. The clinical identification of possible heterozygous females can be difficult because of the varying degrees of clinical signs caused by X-chromosome inactivation. This study is the fi...

  18. Prevalence of Cervical Dysplasia among Women in Kano Municipal ...

    African Journals Online (AJOL)

    Results: Seven hundred and forty (740) women were screened for cervical cancer between March and August 2008 and 536 results were retrieved, giving a retrieval rate of 72.43%. Fifty-seven women had cervical dysplasia giving a prevalence rate of 10.63%. Of the 57 women with cervical dysplasia, 21 (36.8%) had a low ...

  19. Reproducibility of grading systems in oral epithelial dysplasia

    Science.gov (United States)

    Shetty, Devi-Charan

    2012-01-01

    Objective: To assess inter and intra observer variability in grading oral epithelial dysplasia (OED) using Smith and Pindborg grading system, WHO classification system and Brothwell DJ et al. classification system. Study design: In the study 45 histological tissues of dysplasia, 15 each of mild, moderate and severe dysplasia were coded and blindly graded by three observers in three grading systems. Further on the same observers graded 15 slides again of the previous 45 for analyzing the reproducibility in the three grading systems. The individual significance of various indicators of dysplasia among various grades of dysplasia was also assessed. Result: Inter observer agreement was significantly higher in Brothwell system as compared to WHO and Smith and Pindborg system. Intra observer agreement was significantly higher in Smith and Pindborg system, but the predictability and the probability index was distributed over a larger range in this system. Each indicator of dysplasia was also found to be statistically significant (P<0.05) for grading dysplasia. Conclusion: The present study puts forth the inherent intricacies in the grading of oral premalignant lesions. Key words:Carcinoma, dysplasia, grading systems, reproducibility. PMID:22549675

  20. Human papillomavirus (HPV) in vulvar dysplasia and carcinoma in situ

    DEFF Research Database (Denmark)

    Junge, Jette; Poulsen, H; Horn, T

    1995-01-01

    Surgical specimens from 62 patients with vulvar dysplasia and carcinoma in situ were morphologically investigated. Lesions were classified according to WHO (mild, moderate, severe dysplasia and carcinoma in situ) and according to Toki et al. (1991) (warty, basaloid, combined warty/basaloid or bas...

  1. Automated measurement of diagnostic angles for hip dysplasia

    DEFF Research Database (Denmark)

    de Raedt, Sepp; Mechlenburg, I.; Stilling, M.

    2013-01-01

    A fully automatic method for measuring diagnostic angles of hip dysplasia is presented. The method consists of the automatic segmentation of CT images and detection of anatomical landmarks on the femur and acetabulum. The standard angles used in the diagnosis of hip dysplasia are subsequently...

  2. A new syndrome of multiple joint dislocations with metaphyseal dysplasia.

    Science.gov (United States)

    Phaoke, S R; Sharma, A K; Agarawal, S S

    1993-07-01

    A 15-day-old female child is presented with multiple joint dislocations who died during the neonatal period. She had additional features of metaphyseal dysplasia, deficient calcification of vault of the skull, growth retardation, a natal tooth, lymphoedema and facial dysmorphism. This may constitute a new syndrome of multiple joint dislocations with metaphyseal dysplasia.

  3. Cleidocranial dysplasia: Report of 4 cases and review

    Directory of Open Access Journals (Sweden)

    Virender Gombra

    2008-01-01

    Full Text Available Patients with cleidocranial dysplasia commonly present with significant dental problems such as retention of multiple deciduous teeth, impaction or delay in eruption of permanent teeth and often, the presence of supernumerary teeth. We report 4 cases of 2 families presenting with cleidocranial dysplasia disorder with their clinical and radiological diagnosis and illustrating its pathogenesis and various treatment modalities, review of literatures.

  4. Trichoscopic Hair Evaluation in Patients with Ectodermal Dysplasia.

    Science.gov (United States)

    Rakowska, Adriana; Górska, Renata; Rudnicka, Lidia; Zadurska, Małgorzata

    2015-07-01

    Hair abnormalities in ectodermal dysplasia may be difficult to identify. Among 16 patients with ectodermal dysplasia trichoscopy (hair dermoscopy) revealed predominance of pilosebaceous units with 1 hair (69%), abnormalities of hair shaft pigmentation (gray hair with single dark hairs, 56%), pili torti, trichothiodystrophy, trichorrhexis nodosa, and rarely, cicatricial alopecia. Copyright © 2015 Elsevier Inc. All rights reserved.

  5. FLORID CEMENTO-OSSEOUS DYSPLASIA: A REPORT OF TWO ...

    African Journals Online (AJOL)

    masses in the oral cavity.5 This may occur as the result of progressive alveolar atrophy under a denture or after extraction ... osseous dysplasia after radiological examination by ortho- panthomogram. Diagnosis of florid cemento-osseous dysplasia is possible by clinical examination and the distinct radiological presentation, ...

  6. Cervical Dysplasia In Hiv Seropositive Women In Nigeria | Tanko ...

    African Journals Online (AJOL)

    ... times higher in HIV- infected women than HIV- negative women in Jos. Socioeconomic factors such as poverty and social insecurity are risk factors for HIV infection as well are predictors of cervical dysplasia. Keywords: HIV infection, cervix, dysplasia, Nigeria Highland Medical Research Journal Vol. 4 (2) 2006 pp. 21-26 ...

  7. Polyostotic fibrous dysplasia with secondary aneurysmal bone cyst in tibia

    Directory of Open Access Journals (Sweden)

    Vandana L Gaopande

    2015-01-01

    Full Text Available Fibrous dysplasia is a benign tumor-like lesion of bone believed to be developmental in origin. Polyostotic fibrous dysplasia (FD is a rare condition. Our case was further complicated by the presence of secondary aneurysmal bone cyst (ABC. This is the second reported case of polyostotic FD with secondary ABC.

  8. Radiographic diagnosis of a rare case of oculodentodigital dysplasia

    African Journals Online (AJOL)

    Oculodentodigital dysplasia (ODDD), also known as oculodentoosseous dysplasia, is an extremely rare autosomal dominant disorder with high penetrance, intra- and interfamilial phenotypic variability, and advanced paternal age in sporadic cases. The incidence of this disease is not precisely known, with only 243 cases ...

  9. Ceramide profile in hypohidrotic ectodermal dysplasia

    DEFF Research Database (Denmark)

    Jungersted, J. M.; Høgh, Julie Kaae; Hellgren, Lars

    2012-01-01

    Background. Hypohidrotic ectodermal dysplasia (HED) is a rare genetic disease. The clinical presentation includes lack of sweating ability, and an often widely spread dermatitis resembling atopic dermatitis (AD). In AD, the skin‐barrier defect is partly ascribed to the altered lipid profile...... with HED (n = 7) and patients with AD (n = 21), using cyanoacrylate to take biopsy samples from the stratum corneum. Lipids were extracted from the biopsies and analysed using high‐performance thin‐layer chromatography. Results. The lipid profiles of HED and AD were similar in distribution, apart from...

  10. Hereditary mucoepithelial dysplasia and severe respiratory distress

    Directory of Open Access Journals (Sweden)

    Mahmoud Halawa

    2015-01-01

    Full Text Available Hereditary mucoepithelial dysplasia (HMD is a rare autosomal dominant disorder characterized by mucoepithelial disruption of the skin, hair and mucous membranes. It results from defective gap junction formation and leads to non-scarring alopecia, mucosal erythema, perineal erythematous intertrigo, involvement of the conjunctival mucosa, and pulmonary disease. We present a case of severe respiratory distress in an initially healthy full term infant born to a mother with HMD. This infant later developed signs and symptoms of HMD. A high index of suspicion for pulmonary infection with atypical organism is essential in infants with a family history of HMD who present with respiratory distress.

  11. The effect of breeding schemes on the genetic response of canine hip dysplasia, elbow dysplasia, appearance and behaviour traits

    NARCIS (Netherlands)

    Mäki, K.; Liinamo, A.E.; Groen, A.F.; Bijma, P.; Ojala, M.

    2005-01-01

    Current dog breeding programmes must be changed if genetic improvement in health and behaviour traits is to be achieved. A computer simulation programme was used to assess the possible genetic improvement in hip dysplasia (HD), elbow dysplasia (ED) and behaviour (BE) traits in a dog population

  12. Spatial predisposition of dysplasia in Barrett's esophagus segments: a pooled analysis of the SURF and AIM dysplasia trials.

    Science.gov (United States)

    Cotton, Cary C; Duits, Lucas C; Wolf, W Asher; Peery, Anne F; Dellon, Evan S; Bergman, Jacques J; Shaheen, Nicholas J

    2015-10-01

    Surveillance endoscopy detects dysplasia within Barrett's esophagus (BE) and dictates treatment. Current biopsy regimens recommend uniformly spaced random biopsies. We assessed the distribution of dysplasia in BE to develop evidence-based biopsy regimens. We performed analysis of the distribution of dysplasia within BE using pretreatment biopsy data from two randomized controlled trials (RCTs) of radiofrequency ablation for dysplastic BE: the SURF (Surveillance vs. Radiofrequency Ablation) trial and the AIM Dysplasia (Ablation of Intestinal Metaplasia (AIM) Containing Dysplasia) trial. We used generalized linear models with generalized estimating equations (GEE) to estimate prevalence differences for dysplasia depending on the standardized location of biopsies. We performed Monte Carlo simulation of biopsy regimens to estimate their yield for any dysplasia within segments. Dysplasia preferentially resides in the proximal-most half of the BE segment that is almost twice as likely to demonstrate dysplasia as the distal-most quartile. In pooled analysis, compared with the distal-most quarter, the prevalence difference in the proximal-most quarter was 22.6%, in the second proximal-most quarter 23.1%, and in the second distal-most quarter 15.3%. The best performing biopsy regimen in simulation studies acquired 8 biopsies in the most proximal cm of BE, 8 biopsies in the second cm, and 2 biopsies in each cm thereafter (q1cm: 8, 8, 2, 2…). A slightly simpler q2cm (every 2 cm) regimen (q2cm: 12, 12, 4…) was nearly as effective. The post hoc analysis of two RCTs reveals a substantially increased prevalence of dysplasia proximally in BE segments. Our simulations suggest an altered biopsy regimen could increase sensitivity of biopsies in short-segment BE by >30%.

  13. Tapetal dysplasia in a Swedish Vallhund dog.

    Science.gov (United States)

    Scott, Erin M; Teixeira, Leandro B C; Dubielzig, Richard R; Komáromy, András M

    2013-07-01

    To describe the gross, histopathological, and ultrastructural findings in a dog with bilateral tapetal dysplasia. The globes of a 15-year-old neutered male Swedish Vallhund dog with a ventrally displaced tapetum in both eyes were fixed in 10% formalin and submitted to the Comparative Ocular Pathology Laboratory of Wisconsin for histological evaluation. Sections were stained with hematoxylin and eosin, Masson's trichrome, and Melan-A immunohistochemistry (IHC), and tissues were subsequently processed for transmission electron microscopy. Bilateral fundic and gross examination revealed a tapetal fundus inferior to the optic nerve head (ONH) and a nontapetal fundus with mild scattering of tapetal tissue superior to the ONH. Histologically, there was decreased pigmentation of the retinal pigment epithelium with only a few melanin granules in the peripheral retina. The affected tapetum was relatively acellular and fibrous with occasional tapetal cells scattered throughout the inner choroid or displaced into the vascular outer choroid. Special stains revealed that the tapetum was mostly composed of collagen (Masson's trichrome) and failed to express Melan-A (IHC) unlike a normal canine control tapetum. Ultrastructurally, the tapetum was markedly dysplastic both superior and inferior to the ONH with no uniformly arranged tapetal cells. The few cells identified within the tapetum contained irregularly arranged and disorganized electron-dense structures within their cytoplasm, which were interpreted as dysplastic tapetal rodlets. Based on microscopic and ultrastructural findings, this is the first report of tapetal dysplasia in a dog. © 2013 American College of Veterinary Ophthalmologists.

  14. Placental Mesenchymal Dysplasia: A Case Report

    Directory of Open Access Journals (Sweden)

    Rachna Agarwal

    2012-01-01

    Full Text Available Introduction. A rare case of histologically proven placental mesenchymal dysplasia (PMD with fetal omphalocele in a 22-year-old patient is reported. Material and Methods. Antenatal ultrasound of this patient showed hydropic placenta with a live fetus of 17 weeks period of gestation associated with omphalocele. Cordocentesis detected the diploid karyotype of the fetus. Patient, when prognosticated, choose to terminate the pregnancy in view of high incidence of fetal and placental anomalies. Subsequent histopathological examination of placenta established the diagnosis to be placental mesenchymal dysplasia. Conclusion. On clinical and ultrasonic grounds, suspicion of P.M.D. arises when hydropic placenta with a live fetus presents in second trimester of pregnancy. Cordocentesis can detect the diploid karyotype of the fetus in such cases. As this condition is prognostically better than triploid partial mole, continuation of pregnancy can sometimes be considered after through antenatal screening and patient counseling. However, a definite diagnosis of P.M.D. is made only on placental histology by absence of trophoblast hyperplasia and trophoblastic inclusions.

  15. A Case of Extensive polyostotic fibrous dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Byung Do [Dept. of Oral and Maxillofacial Radiology, School of Dentistry, Wonkwang University, Iksan (Korea, Republic of); Hwang, Eui Hwan; Lee, Sang Rae [Dept. of Oral and Maxillofacial Radiology, School of Dentistry, Kyunghee University, Seoul (Korea, Republic of)

    2000-06-15

    Fibrous dysplasia is a benign disorder of bone consisting of intramedullary proliferation of fibrous tissue and irregularly distributed, poorly developed bone. The disease manifests itself in the monostotic form in which only one bone is involved and the polyostotic form in which multiple bones at different sites are affected. We reported a extensive case of polyostotic fibrous dysplasia with involvement of craniofacial bones, mandible, ribs, extremities. A 18-year-old man showed remarkable right facial swelling who had been treated on right femur 3 years ago with a bone graft for pathologic fracture and he recognized facial swelling 5 years ago. Extraoral radiograms and computed tomogram showed diffuse sclerosis with a ground glass appearance of the most calvarial bones, facial bones. The right mandibular lesion showed very expansible lesion with mottled appearance. Bone scans showed multifocal increased uptakes in craniofacial bones, right mandible, bilaterally in ribs, humerus, femur, tibia and characteristic various deformity of right femur (shepherd's crook deformity). This case showed exceptionally bilateral, extensive nature of bone lesion and didn't show any features of skin pigmentation and endocrine disturbances.

  16. A Case of Placental Mesenchymal Dysplasia

    Directory of Open Access Journals (Sweden)

    Shigeki Taga

    2013-01-01

    Full Text Available Placental mesenchymal dysplasia (PMD rarely complicates with pregnancy. A 30-year-old woman, gravida 3, para 3, presenting with placentomegaly, was referred to our department at 18 weeks of gestation. An ultrasonography revealed a normal fetus with a large multicystic placenta, measuring 125 × 42 × 80 mm. The border between the lesion and normal region was not clear. Color doppler revealed little blood flow in the lesion. Magnetic resonance imaging revealed normal fetus and a large multicystic placenta. Serum human chorionic gonadotropin level was 20124.97 U/L, which was normal at 20 weeks of gestation. Thus, placental mesenchymal dysplasia rather than hydatidiform mole with coexistent fetus was suspected. Then, routine checkup was continued. Because she had the history of Cesarean section, an elective Cesarean section was performed at 37 weeks of gestation, and 2520 g female infant with apgar score 8/9 was delivered. The baby was normal with no evidence of Beckwith-Wiedemann syndrome. Placenta of 20 × 16 × 2 cm, weighing 720 g, was bulky with grape like vesicles involving whole placenta. Microscopic examination revealed dilated villi and vessels with thick wall which was lacking trophoblast proliferation. Large hydropic stem villi with myxomatous struma and cistern formation were seen. PMD was histopathologically confirmed.

  17. Acute bronchopulmonary infection due to Streptococcus milleri in a child with cystic fibrosis

    Science.gov (United States)

    Cade, A; Denton, M; Brownlee, K; Todd, N; Conway, S

    1999-01-01

    An 8 year old girl with cystic fibrosis had severe respiratory disease associated with chronic Pseudomonas aeruginosa bronchopulmonary infection. Despite regular courses of intravenous antipseudomonal antibiotics, she continued to deteriorate over 18 months with persistent productive cough, worsening respiratory function, and increasing oxygen dependence. During her 11th admission Streptococcus milleri was isolated from sputum cultures in addition to P aeruginosa. She failed to respond to antipseudomonal antibiotics but improved dramatically with the addition of intravenous benzylpenicillin. Although S milleri is considered a normal mouth commensal and its isolation from sputum of cystic fibrosis patients is of uncertain significance, it was associated with clinically significant infection in this child. S milleri was eradicated with antibiotic treatment and clinical improvement has been maintained.

 PMID:10325713

  18. Multiple bronchoceles in a non-asthmatic patient with allergic bronchopulmonary aspergillosis.

    Science.gov (United States)

    Amin, Muhammad Umar; Mahmood, Rabia

    2008-09-01

    Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity reaction due to a fungus, Aspergillus fumigatus. It is typically seen in patients with long-standing asthma. Our patient was a non-asthmatic 18 years old male who presented with chronic cough for 2 years. Peripheral blood eosinophilia and elevated scrum IgE were observed. His x-ray chest revealed v-shaped opacity in the left upper lobe close to the hilum. High resolution computed tomographic scan of the chest revealed multiple dilated bronchi filled with mucous (bronchoceles) and central bronchiectasis (CB) involving main segmental bronchi. Central bronchiectasis (CB) was typical of ABPA but bronchocele formation was a rare manifestation of the disease. The patient was managed with oral prednisolone and was relieved of his symptoms. Occurrence of ABPA in non-asthmatics is very rare and deserves reporting.

  19. [Allergic bronchopulmonary aspergillosis. A report of a case and literature review].

    Science.gov (United States)

    Meza Brítez, Ricardo L; del Río Navarro, Blanca E; Ochoa López, Georgina; Pietropaolo Cienfuegos, Dino; del Río Chivardi, Jaime M; Rosas Vargas, Miguel A

    2008-01-01

    Allergic bronchopulmonary aspergillosis is a world rare disease with a prevalence between 1 and 2%. It presents in moderate-severe asthma and cistic fibrosis patients. The diagnosis is made in the basis of Rossenberg and Greenberg criteria that can be essential or non essential. We present the case of a 3-year-old boy with allergic bronchopulmonary aspergillosis without bronchiectasies and with a good response to corticosteroids. His mother complained of two years of nasal obstruction, purulent rinorrea, nasal pruritus, sneezing, chronic cough and recurrent wheezing, twice to thrice a month. He also occasionally had vomits and diarrhea in relation with strawberries, banana, cow's milk and chocolate. We made the diagnosis of asthma, allergic rhinitis, sinusitis, and probably food allergy. We treated him with step approach of ICS according to GINA 2006, albuterol PRN, and elimination diet, with bad response. Laboratory exams: Blood white cells with eosinophilia (6%), total serum IgE: 1684 ng/L, aspergillus skin prick test: 4mm, serum IgG-Aspergillus fumigatus: 2.3 mcg/mL, serum IgE-Aspergillus fumigatus: negative, chest roentgenographic parahiliar and apical infiltrates, and chest computed tomography without bronchiectasies. We added prednisone to the treatment for four months, and we observed a very good response; he is now in treatment as mild persistent asthma with ICS low doses. ABPA must be suspected in patients with moderate-severe persistent asthma and a skin prick test positive to Aspergillus fumigatus regardless the age. The treatment with oral corticosteroids is the mainstream of management, and most of the patients have a good response, as we observed with this patient.

  20. Congenital bronchopulmonary malformations: A single-center experience and a review of literature

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    Kumar Basant

    2008-01-01

    Full Text Available Purpose: To present a single-center experience with 25 cases of bronchopulmonary malformations and the review the literature. Materials and Methods: We conducted a retrospective analysis of the medical records of patients with congenital bronchopulmonary malformations who were operated between July 1997 and July 2007 in our institute; we examined the modes of presentations, management, and outcome. Outcome of all patients was assessed over a short follow-up period (average 1.8 months. Results: Out of 25 patients, 18 (72% were male and 7 (28% were female. Age of patients ranged from 1 day to 11 years. The histopathological diagnosis was congenital cystic adenomatoid malformations [CCAM; n = 14 (56%], congenital lobar emphysema [CLE; n = 5 (20%], pulmonary sequestrations [PS; n = 3 (12%], and bronchogenic cysts [BC; n = 3 (12%]. Antenatal diagnosis was available in only 2 (8% patients. The common presenting symptoms were respiratory distress and chest infections. Lobectomy was the procedure of choice . Mortality was 16% (n = 4; M: F = 3: 1. Two patients died because of overwhelming sepsis, one from compromised cardiac function, and one from aspiration which might possibly have been prevented. Conclusion: Patients with progressive respiratory distress due to these anomalies may require urgent surgical intervention regardless of age. The surgical outcome is favorable, with manageable complications. Plain x-ray chest and CT of thorax are usually sufficient for diagnosis and planning of treatment. Pathological diagnosis may differ from the imaging diagnosis. Mortality is found to be more in neonates. Apart from initial stabilization, resection of lesion and careful postoperative care is necessary to reduce mortality and morbidity.

  1. Some variations in lymphatic drainage of selected bronchopulmonary segments in human lungs.

    Science.gov (United States)

    Topol, Mirosław; Masłoń, Adrian

    2009-12-01

    Interest in the role of the pulmonary lymphatic system in the pathophysiology of pulmonary and systemic diseases induced us to carry out anatomical research on the lung lymphatic system in the Polish population. The aim of the study was to evaluate whether lymphatic vessels respect bronchopulmonary segment borders and to determine how often lymphatic vessels run to nodes of another lymphatic region. A block of organs comprising the lungs with the trachea, larynx and tongue, the heart and esophagus was removed from the cadavers at autopsy. The research involved 96 lungs (48 left and 48 right), which were taken from 31 male and 17 female cadavers. The lymphatic vessels were visualized at the mediastinal and interlobar surface of the lung by visual inspection. These vessels were then cannulated and injected with drawing ink. Next, the course of a lymphatic vessel was checked to see whether it was compatible with the bronchopulmonary segments or lobar borders. The first lymph node to become ink-colored via injection was dissected and histologically examined. A total of 135 images of lymphatic vessels (63 in the left lungs and 72 in the right lung) running on the mediastinal or interlobar surface of the lung were evaluated. In all, 12 out of 135 vessels (8.9%) were observed to cross the border of the segment (6/12 vessels) or the border of the lobe (6/12 vessels). We found 10/135 vessels (7.4%) running to the lymph nodes of another lymphatic region. 2009 Elsevier GmbH.

  2. Allergic Bronchopulmonary Mycosis due to Exposure to Eurotium herbariorum after the Great East Japan Earthquake.

    Science.gov (United States)

    Oshikata, Chiyako; Watanabe, Maiko; Saito, Akemi; Ishida, Masatsugu; Kobayashi, Seiichi; Konuma, Rumi; Kamata, Yoichi; Terajima, Jun; Cho, Junichi; Yanai, Masaru; Tsurikisawa, Naomi

    2017-12-01

    Indoor mold levels typically increase after natural disasters, flooding, and water damage. Eurotium herbariorum is the sexual stage of Aspergillus glaucus. Case Presentation A 66-year-old, Japanese male, ex-smoker had been diagnosed with bronchial asthma when he was five years old; he achieved remission at the age of 13 years. He was displaced from his home during the Great East Japan Earthquake on March 11, 2011 and moved to temporary housing in Miyagi Prefecture in June 2011. He experienced the first episode of chest tightness, coughing, and wheezing in February 2012, when he again was diagnosed as having bronchial asthma. Mycofloral surveillance detected high counts of Eurotium in the air of his bedroom, kitchen, and living room, with a maximal fungal count of 163,200 colony-forming units per cubic meter (CFU/m3). Although Cladosporium and Penicillium typically predominate in the indoor air of residential dwellings, only low levels of these organisms were present in the patient's home. Morphologic identification confirmed the isolates as E. herbariorum. The patient had positive reactions to E. herbariorum in skin prick testing and the presence of antigen-specific precipitating antibodies to E. herbariorum. Computed tomography of the chest in August 2013 revealed central bronchiectasis and bronchial wall thickening. The patient experienced late reactions after provocation testing with E. herbariorum. This report presents the rare case of a patient who developed allergic bronchopulmonary mycosis (ABPM) due to exposure to E. herbariorum during temporary housing after the Great East Japan Earthquake. Oshikata C , Watanabe M , Saito A , Ishida M , Kobayashi S , Konuma R , Kamata Y , Terajima J , Cho J , Yanai M , Tsurikisawa N . Allergic bronchopulmonary mycosis due to exposure to eurotium herbariorum after the Great East Japan Earthquake. Prehosp Disaster Med. 2017;32(6):688-690.

  3. Relationship between flexible flat foot and developmental hip dysplasia.

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    Ponce de León Samper, M C; Herrera Ortiz, G; Castellanos Mendoza, C

    2015-01-01

    To evaluate the possible relationship between flexible flat foot and developmental hip dysplasia in children between six and 15 years of age. Cross-sectional study including 65 patients that had undergone surgery due to residual hip dysplasia or hip dislocation and compared against 75 healthy patients. Flexible flat foot prevalence was measured in each group, with the results showing that 61% of the group with residual hip dysplasia or hip dislocation had this condition, vs. 12% in the healthy group. The statistical analysis shows that the chances of suffering from flexible flat foot, are five times greater in the hip dysplasia or hip dislocation group, than in the healthy group. There is no evidence in the literature showing a relationship between these two conditions, even though they have a common etiology. This study shows a potential measurable relation between this two conditions. Patients with hip dysplasia or dislocation may have a higher chance of presenting flexible flat foot during late childhood, adolescence and adulthood, a fact that suggests a relationship between these two pathologies. Also, patients who seek assistance for the first time because of a flexible flat foot condition without having been evaluated during the first year of life for hip dysplasia, would be better off if evaluated for residual hip dysplasia. Copyright © 2014 SECOT. Published by Elsevier Espana. All rights reserved.

  4. [Analysis of development of hip joint dysplasia in dogs].

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    Ledecký, V; Sevcík, A; Capík, I; Trbolová, A

    1997-01-01

    Hip joint dysplasia in dogs occurs mainly in large and heavy breeds. It brings about changes on the acetabulum (socket) and the head of thigh bone, thus causing pain, tiredness, refusal to jump and refusal of increased activity. Even though presently the genetic basis of development of this disease, numerous literary sources indicate existence of pre-disposing factors that facilitate development in later stages of life. Diet and unbalanced development of skeleton and support tissues-ligaments, joint capsule and musculature also have the effect on development of dysplasia. We have analyzed acquired results of X-ray examination of dogs-German shepherds. The size of the group was 4 206 and the examination was aimed at incidence of hip-joint dysplasia during the period of 1977-1995 in the Slovak Republic. We have found out that in 1977 there were 70.7% positive cases out of the total number of examined individuals. Gradual exclusion of dogs with heavier grades of dysplasia (D, E) decreased occurrence of dysplasia to current rate of 40.8%. We considered it to be a high incidence rate. Internal structure of the positive group has changed. The number of dogs with the lightest grade of dysplasia (B) has increased, while the number of heavier grade dysplasia (C, D, E) decreased. In other breeds of dogs, of which more than 20 have been examined at the clinic, the following results have been acquired: Slovak chuvash-32%, Bavarian and Hannover bloodhound-30.6%, Rotweiler-28.6%, Newfoundland dog-26.3%, Bern sheep-dog-13.6%. At the same time we analyze the incidence of dysplasia in dogs whose parents were negative. Group of descendants of 11 negative males and 28 females consisted of 73 dogs. Through x-ray examination, 42.5% of dogs were found to have dysplasia B, C and D at the age of 1 year.

  5. Geleophysic dysplasia associated with bilateral angle closure glaucoma

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    Murat Sinan Saricaoglu

    2013-01-01

    Full Text Available In this case report, we present occurrence of bilateral angle closure glaucoma in a 9-year-old girl with geleophysic dysplasia. Bilateral YAG laser iridotomy was applied, but intraocular pressure (IOP remained at high levels, necessitating bilateral trabeculectomy with mitomycin C. On her follow-up examinations for 3 years, IOP remained in the mid-20s with no need for further intervention or antiglaucoma medication. There are few reports describing the ocular findings of geleophysic dysplasia in literature. To our knowledge, this is the first case report describing an application of glaucoma surgery and its results at geleophysic dysplasia.

  6. [Florid cemento-osseous dysplasia of the jaws].

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    Benazzou, S; Boulaadas, M; El Ayoubi, A; Nazih, N; Essakalli, L; Kzadri, M

    2011-06-01

    Florid cemento-osseous dysplasia is a benign and rare tumor of the jaws. It is more commonly seen in middle-aged black women. Most cases are asymptomatic and are found during routine radiographic examination. We report two complicated cases of florid cemento-osseous dysplasia, one with facial deformity and the other with chronic osteitis. The diagnosis of florid cemento-osseous dysplasia is based on clinical and radiological features. The lesions are commonly bilateral and symmetrical. Copyright © 2011. Published by Elsevier Masson SAS.

  7. Focal cemento-osseous dysplasia masquerading as a residual cyst.

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    Bhandari, Rajat; Sandhu, Simarpreet V; Bansal, Himanta; Behl, Rashi; Bhullar, Ramanpreet Kaur

    2012-04-01

    Focal cemento-osseous dysplasia (FCOD) is a benign fibroosseous condition that can be seen in dentulous and edentulous patients. It is an asymptomatic lesion and needs no treatment; however, follow-up is essential due to the possibility that it can progress to a condition called florid cemento-osseous dysplasia. We report a case of FCOD of mandible in a 25-year-old female. Clinically, the lesion resembled periapical pathosis of odontogenic origin. An attempt has been made to discuss the clinical and histopathologic features along with differential diagnosis of cemento-osseous dysplasia.

  8. Focal cemento-osseous dysplasia masquerading as a residual cyst

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    Rajat Bhandari

    2012-01-01

    Full Text Available Focal cemento-osseous dysplasia (FCOD is a benign fibroosseous condition that can be seen in dentulous and edentulous patients. It is an asymptomatic lesion and needs no treatment; however, follow-up is essential due to the possibility that it can progress to a condition called florid cemento-osseous dysplasia. We report a case of FCOD of mandible in a 25-year-old female. Clinically, the lesion resembled periapical pathosis of odontogenic origin. An attempt has been made to discuss the clinical and histopathologic features along with differential diagnosis of cemento-osseous dysplasia.

  9. Polypoidal Intestinal Metaplasia and Dysplasia of the External Urethral Meatus

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    Mary Mathew

    2012-01-01

    Full Text Available Urethral mucosa with intestinal metaplasia and dysplasia is a rare occurrence. To date only a single case has been reported in a male with long-standing urethral stricture. We present a 33-year-old female with polypoid intestinal metaplasia and dysplasia of the external urethral meatus in the absence of an inciting factor. Intestinal metaplasia of the urethral mucosa may undergo dysplasia, emphasizing the necessity of a high degree of clinical suspicion and vigilant pathological examination of these lesions.

  10. Bilateral Cerebellar Cortical Dysplasia without Other Malformations: A Case Report

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    Oh, Jung Seok; Ahn Kook Jin; Kim, Jee Young; Lee, Sun Jin; Park, Jeong Mi [Catholic University Yeouido St. Mary' s Hospital, College of Medicine, Seoul (Korea, Republic of)

    2010-06-15

    Recent advances in MRI have revealed congenital brain malformations and subtle developmental abnormalities of the cerebral and cerebellar cortical architecture. Typical cerebellar cortical dysplasia as a newly categorized cerebellar malformation, has been seen in patients with Fukuyama congenital muscular dystrophy. Cerebellar cortical dysplasia occurs at the embryonic stage and is often observed in healthy newborns. It is also incidentally and initially detected in adults without symptoms. To the best of our knowledge, cerebellar dysplasia without any related disorders is very rare. We describe the MRI findings in one patient with disorganized foliation of both cerebellar hemispheres without a related disorder or syndrome

  11. Radiographic features of craniometadiaphyseal dysplasia, wormian bone type.

    Science.gov (United States)

    Langer, L O; Brill, P W; Afshani, E; Williams, C A; Thomas, I T; Frias, J L

    1991-01-01

    We describe the radiographic findings in two siblings with a previously unrecognized craniotubular bone dysplasia. We call this condition craniometadiaphyseal dysplasia, wormian bone type. Because the parents of the siblings are consanguineous, this is probably a genetically determined condition with an autosomal recessive type of transmission. The findings in the siblings are compared with those of a woman with the same condition, previously reported as an example of craniometaphyseal dysplasia. The combination of findings in these patients seems diagnostic: characteristic skull changes including multiple wormian bones; wide long tubular bones without normal metaphyseal flaring; wide short tubular bones without normal diaphyseal constriction and sometimes actual diaphyseal expansion; and wide ribs and clavicles.

  12. [Diagnostic problems in gastric dysplasias: value of cytology (author's transl)].

    Science.gov (United States)

    Simatos, A; Zagury, D; Zeitoun, P; Galet, M J; Pluot, M

    Gastric smears have been performed on 835 patients, among which examinations 771 (92 percent) were available. Histology assumed in parallel showed a dysplasia lesion in 56 cases: 14 of the 601 negative smears (2.3 percent), in 33 of the 40 suspect smears (82 percent), and in 9 of the 130 cases of positive smears (7 percent). Gastric dysplasia evidence both by cytology and pathology show mostly differenciated cells, alterations of which reflect the lesion level. By contrast, cytology and histology diagnosis are not in agreement when dysplasia concern dedifferenciated cells with a severe degree of alterations which can be misinterpreted as malignant cells.

  13. Neuronal intestinal dysplasia in an infant: case report

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    Lim, Se Kyong; Kim, Jong Chul; Yoon, Chung Dae; Sul, Ji Young; Song, Kyu Sang [Chungnam National University Hospital, Daejeon (Korea, Republic of)

    2006-09-15

    Neuronal intestinal dysplasia in pediatric patients has similar clinical symptoms and often similar radiologic findings to those of Hirschsprung's disease. Yet neuronal intestinal dysplasia shows hyperplasia of the myenteric plexus for the pathology, and it requires different treatment compared with Hirschsprung disease. This disease had been reported many times in Europe but, to date, only one case has been reported in the radiologic literatures in Korea. We report here on a case of neuronal intestinal dysplasia that involved the entire colon in a two-month-old boy, and we include the radiographic findings.

  14. Ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome

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    Reema Sharma Dhar

    2014-01-01

    Full Text Available Ectrodactyly-ectodermal dysplasia-cleft (EEC syndrome is an autosomal dominant disorder characterized by the triad of ectrodactyly-ectodermal dysplasia, and facial clefting along with some associated features. Presence of all the three major features in a single individual is extremely rare. We report a case of 4 year 11 months old child with EEC syndrome having ectodermal dysplasia-cleft lip and cleft palate and ectrodactyly with some associated features. Clinical features, diagnosis and role of a dentist in the multidisciplinary treatment approach have been elaborated in this case report.

  15. Ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome.

    Science.gov (United States)

    Dhar, Reema Sharma; Bora, Amitava

    2014-01-01

    Ectrodactyly-ectodermal dysplasia-cleft (EEC) syndrome is an autosomal dominant disorder characterized by the triad of ectrodactyly-ectodermal dysplasia, and facial clefting along with some associated features. Presence of all the three major features in a single individual is extremely rare. We report a case of 4 year 11 months old child with EEC syndrome having ectodermal dysplasia-cleft lip and cleft palate and ectrodactyly with some associated features. Clinical features, diagnosis and role of a dentist in the multidisciplinary treatment approach have been elaborated in this case report.

  16. Radiological features of bilateral hereditary micro-epiphyseal dysplasia - a distinct entity in the skeletal dysplasias

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    Mostert, A.K. [Isala Clinics, Location Weezenlanden, Dept. of Orthopaedic Surgery, Zwolle (Netherlands); Dijkstra, P.F. [Jan van Breemen Inst., Dept. of Radiology, Amsterdam (Netherlands); Horn, J.R. van [Univ. Hospital Groningen, Dept. of Orthopaedic Surgery, Groningen (Netherlands); Jansen, B.R.H. [Reinier de Graaf Hospital, Dept. of Orthopaedic Surgery, Delft (Netherlands); Heutink, P. [Erasmus MCRotterdam, Dept. of Clinical Genetics, Rotterdam (Netherlands); Lindhout, D. [Univ. Medical Centre Utrecht, Dept. of Medical Genetics, Utrecht (Netherlands)

    2002-07-01

    Aim: To prove that bilateral hereditary micro-epiphyseal dysplasia (BHMED), first described by Elsbach in 1959, is a distinct disorder radiologically as well as clinically, compared with multiple epiphyseal dysplasia (MED). Material and Methods: We used the data of the revised pedigree with 84 family members, performed a medical history, physical examination and made a radiological evaluation for defining a clinical and radiological phenotype of BHMED family members. We used blood samples for genetic analysis. Results: Although there is a clear clinical picture of the dysplasia, the radiological signs are more reliable for making the diagnosis. Especially the typical deformity of the hip and knee joint are diagnostic for BHMED. By linkage analysis we excluded linkage with the three known MED-loci (EDM1, EDM2 and EDM3). Conclusion: BHMED is indeed an entity that is distinct from common multiple epiphyseal dysplasia (MED), clinically, as well as radiologically and genetically. (orig.) [German] Ziel: Es sollte dargelegt werden, dass sich eine vererbliche, laterale Mikro-Epiphysendysplasie (BHMED), Erstbeschreibung durch Elsbach 1959, klinisch, radiologisch und genetisch von einer mutiplen Epiphysendysplasie (MED) unterscheidet. Material und Methode: Anhand der Daten eines ueberarbeiteten Stammbaumes mit 84 Familienmitgliedern wurde der medizinische Werdegang rekonstruiert. Es erfolgte eine physische Untersuchung der Familienmitglieder. Schliesslich wurde eine radiologische Auswertung durchgefuehrt, um einen klinischen und radiologischen Phaenotyp der von BHMED betroffenen Familienmitglieder zu definieren. Fuer eine genetische Analyse wurden Blutproben entnommen. Ergebnisse: Obwohl es ein deutliches klinisches Bild einer Dysplasie gibt, sind die radiologischen Kennzeichen fuer die Diagnose zuverlaessiger. Insbesondere die typische Deformation der Huefte und des Kniegelenks ist diagnostisch fuer BHMED. Durch Linkage-Analyse konnte eine Verbindung zu den drei bekannten

  17. Mutation-positive arrhythmogenic right ventricular dysplasia/cardiomyopathy: the triangle of dysplasia displaced.

    Science.gov (United States)

    Te Riele, Anneline S J M; James, Cynthia A; Philips, Binu; Rastegar, Neda; Bhonsale, Aditya; Groeneweg, Judith A; Murray, Brittney; Tichnell, Crystal; Judge, Daniel P; Van Der Heijden, Jeroen F; Cramer, Maarten J M; Velthuis, Birgitta K; Bluemke, David A; Zimmerman, Stefan L; Kamel, Ihab R; Hauer, Richard N W; Calkins, Hugh; Tandri, Harikrishna

    2013-12-01

    The traditional description of the Triangle of Dysplasia in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) predates genetic testing and excludes biventricular phenotypes. We analyzed Cardiac Magnetic Resonance (CMR) studies of 74 mutation-positive ARVD/C patients for regional abnormalities on a 5-segment RV and 17-segment LV model. The location of electroanatomic endo- and epicardial scar and site of successful VT ablation was recorded in 11 ARVD/C subjects. Among 54/74 (73%) subjects with abnormal CMR, the RV was abnormal in almost all (96%), and 52% had biventricular involvement. Isolated LV abnormalities were uncommon (4%). Dyskinetic basal inferior wall (94%) was the most prevalent RV abnormality, followed by basal anterior wall (87%) dyskinesis. Subepicardial fat infiltration in the posterolateral LV (80%) was the most frequent LV abnormality. Similar to CMR data, voltage maps revealed scar (disease involving the basal inferior and anterior RV, and the posterolateral LV. The RV apex is only involved in advanced ARVD/C, typically as a part of global RV involvement. These results displace the RV apex from the Triangle of Dysplasia, and provide insights into the pathophysiology of ARVD/C. © 2013 Wiley Periodicals, Inc.

  18. Spatial Predisposition of Dysplasia in Barrett's Esophagus Segments: A Pooled Analysis of The SURF and AIM – Dysplasia Trials

    Science.gov (United States)

    Cotton, Cary C; Duits, Lucas C; Wolf, W Asher; Peery, Anne F; Dellon, Evan S.; Bergman, Jacques J.; Shaheen, Nicholas J

    2016-01-01

    Introduction Surveillance endoscopy detects dysplasia within Barrett's esophagus (BE) and dictates treatment. Current biopsy regimens recommend uniformly-spaced random biopsies. We assessed the distribution of dysplasia in BE to develop evidence-based biopsy regimens. Methods We performed analysis of the distribution of dysplasia within BE, using pre-treatment biopsy data from two randomized controlled trials (RCT) of radiofrequency ablation (RFA) for dysplastic BE: the SURF Trial and the AIM Dysplasia Trial. We used generalized linear models with generalized estimating equations to estimate prevalence differences for dysplasia depending on the standardized location of biopsies. We performed Monte Carlo simulation of biopsy regimens to estimate their yield for any dysplasia within segments. Results Dysplasia preferentially resides in the proximal-most half of the BE segment, which is almost twice as likely to demonstrate dysplasia as the distal-most quartile. In pooled analysis, compared to the distal-most quarter, the prevalence difference in the proximal-most quarter was 22.6%, in the second proximal-most quarter 23.1%, and in the second distal-most quarter 15.3%. The best performing biopsy regimen in simulation studies acquired 8 biopsies in the most proximal centimeter of BE, 8 biopsies in the second cm, and 2 biopsies in each cm thereafter q1cm - (8, 8, 2, 2…). A slightly simpler q2cm regimen q2cm - (12, 12, 4…) was nearly as effective. Conclusion Post-hoc analysis of two RCTs reveals a substantially increased prevalence of dysplasia proximally in BE segments. Our simulations suggest an altered biopsy regimen could increase sensitivity of biopsies in short-segment BE by >30%. PMID:26346864

  19. Guidelines for genetic skeletal dysplasias for pediatricians

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    Sung Yoon Cho

    2015-12-01

    Full Text Available Skeletal dysplasia (SD is a kind of heterogeneous genetic disorder characterized by abnormal growth, development, differentiation, and maintenance of the bone and cartilage. The patients with SD most likely to be seen by a pediatrician or orthopedic surgeon are those who present with short stature in childhood. Because each category has so many diseases, classification is important to understand SD better. In order to diagnose a SD accurately, clinical and radiographic findings should be evaluated in detail. In addition, genetic diagnosis of SD is important because there are so various SDs with complex phenotypes. To reach an exact diagnosis of SDs, cooperative approach by a clinician, a radiologist and a geneticist is important. This review aims to provide an outline of the diagnostic approach for children with disproportional short stature.

  20. Focal cemento-osseous dysplasia of mandible.

    Science.gov (United States)

    Cankaya, Abdülkadir Burak; Erdem, Mehmet Ali; Olgac, Vakur; Firat, Deniz Refia

    2012-09-03

    Fibro-osseous lesions are disturbances in bone metabolism in which normal bone is replaced by a connective tissue matrix that then gradually develops into cemento-osseous tissue. Typically, the lesion is asymptomatic and is detected on routine radiographic examination. Radiologically, this lesion has three stages of maturation: pure radiolucent, radiopaque/mixed radiolucent, and radiopaque appearance. During these stages the lesion can be misdiagnosed. In this case report a 69-year- old patient with a a complaint of painless swelling of the left mandibular molar and premolar area is presented along with a review of the differential diagnoses considered in order to reach a final diagnosis of focal cemento-osseous dysplasia.