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Sample records for prevent bronchopulmonary dysplasia

  1. 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

    The Scandinavian approach is an effective combined treatment for respiratory distress syndrome (RDS) and prevention of bronchopulmonary dysplasia (BPD). It is composed of many individual parts. Of significant importance is the early treatment with nasal continuous positive airway pressure (n...

  2. Managing Children with Bronchopulmonary Dysplasia

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    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.

  3. 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....

  4. [Clinical management of bronchopulmonary dysplasia].

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    Schachinger, H; Frank, H D; Schmid, H

    1984-09-01

    Bronchopulmonary dysplasia is caused by several factors. Avoidance of bronchopulmonary dysplasia is directed at its causes and should always attempt the rapid weaning of the patient from the respirator. Some of the preventive measures include a) avoiding an oxygen injury; b) prevention of barotrauma; 3) sufficient moisturization and warming of the respiratory gases; d) regular cleaning of the respiratory tract and bacterial controls of the tracheal secretion; e) administration of vitamin E; f) reconsideration (restricting the use) of parenteral nutrition. Assistance in the weaning from the respiratory means a) avoidance of an open ductus arteriosus; b) paced reduction of FiO2, respiratory pressure and frequency up to intermittent mandatory ventilation and final transition to spontaneous respiration with increased mean respiratory pressure; c) avoidance of cardiac insufficiency; d) administration of corticosteroids; e) theophyllin, and f) physical therapy. The number of patients with bronchopulmonary dysplasia has remained the same in the last six years. However, the mortality until 1979 round about 50% has been reduced to 1/3, in the years 1980/81.

  5. [Magnesium and bronchopulmonary dysplasia].

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    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.

  6. [Early postnatal application of glucocorticoids for preventing bronchopulmonary dysplasia in preterm infants: a Meta analysis].

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    Ji, Feng-Juan; Yin, Yong; Xu, Juan; Zhao, Li-Xia; Zhou, Ya-Juan; Zhu, Lei

    2017-06-01

    To study the clinical effect and safety of early postnatal application of glucocorticoids in the prevention of bronchopulmonary dysplasia (BPD) in preterm infants. The databases including PubMed, Cochrane Library, Embase, CNKI, Wanfang Data, and VIP were comprehensively searched for articles on early postnatal application of glucocorticoids in the prevention of BPD in preterm infants published up to June 2016. Review Manager 5.3 was used for the Meta analysis of 16 randomized controlled trials (RCTs) that met the inclusion criteria. A total of 2 962 participants were enrolled in the 16 RCTs, with 1 486 patients in the trial group and 1 476 in the control group. The Meta analysis showed that early postnatal application of glucocorticoids reduced the incidence rate of BPD at a corrected gestational age of 36 weeks (OR=0.73, 95%CI: 0.61-0.87, P=0.0004), but there was an increase in the risk of hyperglycemia (OR=1.61, 95%CI: 1.24-2.09, P=0.0003), hypertension (OR=1.63, 95%CI: 1.11-2.38, P=0.01), and intestinal perforation (OR=1.51, 95%CI: 1.12-2.04, P=0.007). At present, it is not recommended to use glucocorticoids to prevent BPD in preterm infants. Its advantages and disadvantages need further studies, with special focuses on the adverse effects of hyperglycemia, hypertension, and intestinal perforation.

  7. Update on Vitamin E and Its Potential Role in Preventing or Treating Bronchopulmonary Dysplasia.

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    Stone, Cosby A; McEvoy, Cindy T; Aschner, Judy L; Kirk, Ashudee; Rosas-Salazar, Christian; Cook-Mills, Joan M; Moore, Paul E; Walsh, William F; Hartert, Tina V

    2018-03-07

    Vitamin E is obtained only through the diet and has a number of important biological activities, including functioning as an antioxidant. Evidence that free radicals may contribute to pathological processes such as bronchopulmonary dysplasia (BPD), a disease of prematurity associated with increased lung injury, inflammation and oxidative stress, led to trials of the antioxidant vitamin E (α-tocopherol) to prevent BPD with variable results. These trials were all conducted at supraphysiologic doses and 2 of these trials utilized a formulation containing a potentially harmful excipient. Since 1991, when the last of these trials was conducted, both neonatal management strategies for minimizing oxygen and ventilator-related lung injury and our understanding of vitamin E isoforms in respiratory health have advanced substantially. It is now known that there are differences between the effects of vitamin E isoforms α-tocopherol and γ-tocopherol on the development of respiratory morbidity and inflammation. What is not known is whether improvements in physiologic concentrations of individual or combinations of vitamin E isoforms during pregnancy or following preterm birth might prevent or reduce BPD development. The answers to these questions require adequately powered studies targeting pregnant women at risk of preterm birth or their premature infants immediately following birth, especially in certain subgroups that are at increased risk of vitamin E deficiency (e.g., smokers). The objective of this review is to compile, update, and interpret what is known about vitamin E isoforms and BPD since these first studies were conducted, and suggest future research directions. © 2018 S. Karger AG, Basel.

  8. Initial respiratory management in preterm infants and bronchopulmonary dysplasia

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    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.

  9. Systemic Hydrocortisone To Prevent Bronchopulmonary Dysplasia in preterm infants (the SToP-BPD study): statistical analysis plan.

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    Onland, Wes; Merkus, Maruschka P; Nuytemans, Debbie H; Jansen-van der Weide, Marijke C; Holman, Rebecca; van Kaam, Anton H

    2018-03-09

    Bronchopulmonary dysplasia (BPD) is the most common complication of preterm birth with short-term and long-term adverse consequences. Although the glucocorticoid dexamethasone has been proven to be beneficial for the prevention of BPD, there are concerns about an increased risk of adverse neurodevelopmental outcome. Hydrocortisone has been suggested as an alternative therapy. The aim of the Systemic Hydrocortisone To Prevent Bronchopulmonary Dysplasia in preterm infants (SToP-BPD) trial is to assess the efficacy and safety of postnatal hydrocortisone administration for the reduction of death or BPD in ventilator-dependent preterm infants. The SToP-BPD study is a multicentre, double-blind, placebo-controlled hydrocortisone trial in preterm infants at risk for BPD. After parental informed consent is obtained, ventilator-dependent infants are randomly allocated to hydrocortisone or placebo treatment during a 22-day period. The primary outcome measure is the composite outcome of death or BPD at 36 weeks postmenstrual age. Secondary outcomes are short-term effects on pulmonary condition and long-term neurodevelopmental sequelae assessed at 2 years corrected age. Complications of treatment, other serious adverse events and suspected unexpected serious adverse reactions are reported as safety outcomes. This pre-specified statistical analysis plan was written and submitted without knowledge of the unblinded data. Netherlands Trial Register, NTR2768 . Registered on 17 February 2011. EudraCT, 2010-023777-19. Registered on 2 November 2010.

  10. Genetic predisposition to bronchopulmonary dysplasia.

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    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.

  11. 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....

  12. 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

  13. 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

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

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    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

  15. Does nasal CPAP reduce bronchopulmonary dysplasia (BPD)?

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    Patel, Ds; Greenough, A

    2008-10-01

    Early nasal continuous positive airway pressure (nCPAP) or early surfactant therapy with early extubation onto nCPAP rather than continued mechanical ventilation has been adopted by many centres, particularly in Scandinavia, as part of the treatment of newborns with respiratory distress syndrome. It has been suggested that bronchopulmonary dysplasia is less of a problem in centres adopting such a policy. Results from randomized trials suggest prophylactic or early nCPAP may reduce bronchopulmonary dysplasia (BPD), but further studies are required to determine the relative contributions of an early lung recruitment policy, early surfactant administration and nCPAP in reducing BPD. In addition, the optimum method of generating and delivering CPAP needs to be determined. The efficacy of nCPAP in improving long-term respiratory outcomes needs to be compared with the newer ventilator techniques with the optimum and timing of delivery of surfactant administration.

  16. Systemic Hydrocortisone To Prevent Bronchopulmonary Dysplasia in preterm infants (the SToP-BPD study); a multicenter randomized placebo controlled trial

    NARCIS (Netherlands)

    Onland, Wes; Offringa, Martin; Cools, Filip; De Jaegere, Anne P.; Rademaker, Karin; Blom, Henry; Cavatorta, Eric; Dijk, Peter H.; van Heijst, Arno F.; Kramer, Boris W.; Kroon, Andre A.; Mohns, Thilo; van Straaten, Henrica L.; te Pas, Arjan B.; Theyskens, Claire; van Weissenbruch, Mirjam M.; van Kaam, Anton H.; Beer de, A.

    2011-01-01

    Background: Randomized controlled trials have shown that treatment of chronically ventilated preterm infants after the first week of life with dexamethasone reduces the incidence of the combined outcome death or bronchopulmonary dysplasia (BPD). However, there are concerns that dexamethasone may

  17. Preclinical evaluation of cell-based strategies to prevent or treat bronchopulmonary dysplasia in animal models: a systematic review.

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    Lesage, Flore; Jimenez, Julio; Toelen, Jaan; Deprest, Jan

    2018-04-01

    Bronchopulmonary dysplasia (BPD) remains the most common complication of extreme prematurity as no effective treatment is available to date. This calls for the exploration of new therapeutic options like cell therapy, which is already effective for various human (lung) disorders. We systematically searched the MEDLINE, Embase, and Web of Science databases from the earliest date till January 2017 and included original studies on the perinatal use of cell-based therapies (i.e. cells and/or cell-derivatives) to treat BDP in animal models. Fourth publications describing 47 interventions were retrieved. Newborn mice/rats raised in a hyperoxic environment were studied in most interventions. Different cell types - either intact cells or their conditioned medium - were administered, but bone marrow and umbilical cord blood derived mesenchymal stem cells were most prevalent. All studies reported positive effects on outcome parameters including alveolar and vascular morphometry, lung function, and inflammation. Cell homing to the lungs was demonstrated in some studies, but the therapeutic effects seemed to be mostly mediated via paracrine modulation of inflammation, fibrosis and angiogenesis. Multiple rat/mouse studies show promise for cell therapy for BPD. Yet careful study of action mechanisms and side effects in large animal models is imperative before clinical translation can be achieved.

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

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    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 effects on vascular and alveolar growth.

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

    Directory of Open Access Journals (Sweden)

    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

  20. Pulmonary Hemosiderosis in Children with Bronchopulmonary Dysplasia

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    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.

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

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    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.

  2. Association between anemia and bronchopulmonary dysplasia in preterm infants

    OpenAIRE

    Jun Duan; Xiangyong Kong; Qiuping Li; Shaodong Hua; Sheng Zhang; Xiaoying Zhang; Zhichun Feng

    2016-01-01

    Anemia is commonly seen in preterm infants. It may reduce the capacity of hemoglobin to transport oxygen throughout the body and may result in tissue and organ dysfunction. This study aimed to investigate the effect of anemia on the development of bronchopulmonary dysplasia (BPD) in preterm infants. 243 infants who were admitted to BaYi Children?s Hospital Affiliated to Clinical Medical College in Beijing Military General Hospital with gestational age (GA) less than 32 weeks from February, 20...

  3. The economic impact of prematurity and bronchopulmonary dysplasia.

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    Álvarez-Fuente, María; Arruza, Luis; Muro, Marta; Zozaya, Carlos; Avila, Alejandro; López-Ortego, Paloma; González-Armengod, Carmen; Torrent, Alba; Gavilán, Jose Luis; Del Cerro, María Jesús

    2017-12-01

    Bronchopulmonary dysplasia (BPD) is one of the most serious chronic lung diseases in infancy and one of the most important sequels of premature birth (prevalence of 15-50%). Our objective was to estimate the cost of BPD of one preterm baby, with no other major prematurity-related complications, during the first 2 years of life in Spain. Data from the Spanish Ministry of Health regarding costs of diagnosis-related group of preterm birth, hospital admissions and visits, palivizumab administration, and oxygen therapy in the year 2013 were analyzed. In 2013, 2628 preterm babies were born with a weight under 1500 g; 50.9% were males. The need for respiratory support was 2.5% needed only oxygen therapy, 39.5% required conventional mechanical ventilation, and 14.9% required high-frequency ventilation. The incidence of BPD was of 34.9%. The cost of the first 2 years of life of a preterm baby with BPD and no other major prematurity-related complications ranged between 45,049.81 € and 118,760.43 €, in Spain, depending on birth weight and gestational age. If the baby required home oxygen therapy or developed pulmonary hypertension, this cost could add up to 181,742.43 €. Prematurity and BPD have an elevated cost, even for public health care systems. This cost will probably increase in the coming years if the incidence and survival of preterm babies keeps rising. The development of new therapies and preventive strategies to decrease the incidence of BPD and other morbidities associated with prematurity should be a priority. What is known: • Bronchopulmonary dysplasia (BPD) is a serious chronic lung disease related with premature birth. • BPD is an increasing disease due to the up-rise in the number of premature births. What is new: • The economic cost of preterm birth and BPD has never before been estimated in Spain nor published with European data. • Preterm babies with BPD and a good clinical outcome carry also an important economic and social burden.

  4. Association between anemia and bronchopulmonary dysplasia in preterm infants.

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    Duan, Jun; Kong, Xiangyong; Li, Qiuping; Hua, Shaodong; Zhang, Sheng; Zhang, Xiaoying; Feng, Zhichun

    2016-03-03

    Anemia is commonly seen in preterm infants. It may reduce the capacity of hemoglobin to transport oxygen throughout the body and may result in tissue and organ dysfunction. This study aimed to investigate the effect of anemia on the development of bronchopulmonary dysplasia (BPD) in preterm infants. 243 infants who were admitted to BaYi Children's Hospital Affiliated to Clinical Medical College in Beijing Military General Hospital with gestational age (GA) less than 32 weeks from February, 2014 to February, 2015 were included in the study. Maternal and infant data were recorded. Multivarariate logistic regression analysis was performed to determine the association between anemia and BPD. Of 243 preterm infants, the incidence of anemia was higher in BPD patients than non-BPD patients (p anemia was associated with an increased risk of BPD. Number of transfusions is also a significant risk factor for BPD (p = 0.001). Therefore, prevention and treatment of early anemia is necessary and reducing number of transfusions may reduce the incidence of BPD in preterm infants.

  5. 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.

  6. Systemic hydrocortisone to prevent bronchopulmonary dysplasia in preterm infants (the SToP-BPD study; a multicenter randomized placebo controlled trial

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    Onland Wes

    2011-11-01

    Full Text Available Abstract Background Randomized controlled trials have shown that treatment of chronically ventilated preterm infants after the first week of life with dexamethasone reduces the incidence of the combined outcome death or bronchopulmonary dysplasia (BPD. However, there are concerns that dexamethasone may increase the risk of adverse neurodevelopmental outcome. Hydrocortisone has been suggested as an alternative therapy. So far no randomized controlled trial has investigated its efficacy when administered after the first week of life to ventilated preterm infants. Methods/Design The SToP-BPD trial is a randomized double blind placebo controlled multicenter study including 400 very low birth weight infants (gestational age Discussion This trial will determine the efficacy and safety of postnatal hydrocortisone administration at a moderately early postnatal onset compared to placebo for the reduction of the combined outcome mortality and BPD at 36 weeks postmenstrual age in ventilator dependent preterm infants. Trial registration number Netherlands Trial Register (NTR: NTR2768

  7. 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.

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    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.

  8. Systemic Hydrocortisone To Prevent Bronchopulmonary Dysplasia in preterm infants (the SToP-BPD study); a multicenter randomized placebo controlled trial.

    Science.gov (United States)

    Onland, Wes; Offringa, Martin; Cools, Filip; De Jaegere, Anne P; Rademaker, Karin; Blom, Henry; Cavatorta, Eric; Debeer, Anne; Dijk, Peter H; van Heijst, Arno F; Kramer, Boris W; Kroon, Andre A; Mohns, Thilo; van Straaten, Henrica L; te Pas, Arjan B; Theyskens, Claire; van Weissenbruch, Mirjam M; van Kaam, Anton H

    2011-11-09

    Randomized controlled trials have shown that treatment of chronically ventilated preterm infants after the first week of life with dexamethasone reduces the incidence of the combined outcome death or bronchopulmonary dysplasia (BPD). However, there are concerns that dexamethasone may increase the risk of adverse neurodevelopmental outcome. Hydrocortisone has been suggested as an alternative therapy. So far no randomized controlled trial has investigated its efficacy when administered after the first week of life to ventilated preterm infants. The SToP-BPD trial is a randomized double blind placebo controlled multicenter study including 400 very low birth weight infants (gestational age BPD at 36 weeks postmenstrual age. Secondary outcomes are short term effects on the pulmonary condition, adverse effects during hospitalization, and long-term neurodevelopmental sequelae assessed at 2 years corrected gestational age. Analysis will be on an intention to treat basis. This trial will determine the efficacy and safety of postnatal hydrocortisone administration at a moderately early postnatal onset compared to placebo for the reduction of the combined outcome mortality and BPD at 36 weeks postmenstrual age in ventilator dependent preterm infants.

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

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    Rosane Reis de Mello

    2017-07-01

    Full Text Available ABSTRACT CONTEXT AND OBJECTIVE: 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. DESIGN AND SETTING: Cross-sectional study developed in a public tertiary-care hospital. METHODS: 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. RESULTS: 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. CONCLUSION: Bronchopulmonary dysplasia was an independent risk factor for alteration of the processing speed quotient.

  10. Bronchopulmonary dysplasia: understanding of the underlying pathological mechanisms

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    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

  11. Bronchopulmonary Dysplasia Early Changes Leading To Long Term Consequences

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    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.

  12. Cystic fibrosis in three children with bronchopulmonary dysplasia.

    Science.gov (United States)

    Holmgren, N L; Faro, A; Gondor, M I; Orenstein, D M

    2001-06-01

    Cystic fibrosis (CF) and bronchopulmonary dysplasia (BPD) are two common causes of chronic lung disease in children. Patients with BPD or CF often have recurrent respiratory symptoms, failure to thrive, and/or metabolic alkalosis during infancy and childhood. Thus, recognizing the diagnosis of CF in an infant with BPD can be difficult. We present three infants with both BPD and CF. The infants shared a history of respiratory distress and prolonged oxygen requirements. All three also had difficulty gaining weight, even after pancreatic enzyme supplementation was instituted. Metabolic alkalosis was observed in two infants. Previous studies in children with CF suggest that early diagnosis may impact both lung health and nutritional status. A high index of suspicion is necessary for clinicians to identify these children early and intervene with appropriate therapy.

  13. 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

  14. 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

  15. Late (≥ 7 days) inhalation corticosteroids to reduce bronchopulmonary dysplasia in preterm infants

    NARCIS (Netherlands)

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

    2017-01-01

    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 corticosteroids

  16. 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

  17. 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 (pPediatria de São Paulo. Publicado por Elsevier Editora Ltda. All rights reserved.

  18. 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

  19. 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.

  20. Reliability of CXR for the diagnosis of bronchopulmonary dysplasia

    International Nuclear Information System (INIS)

    Moya, M.P.; Auten, R.L. Jr.; Bisset, G.S. III; Miller, C.; Hollingworth, C.; Frush, D.P.

    2001-01-01

    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.)

  1. Ventilator flow data predict bronchopulmonary dysplasia in extremely premature neonates

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    Mariann H. Bentsen

    2018-03-01

    Full Text Available Early prediction of bronchopulmonary dysplasia (BPD may facilitate tailored management for neonates at risk. We investigated whether easily accessible flow data from a mechanical ventilator can predict BPD in neonates born extremely premature (EP. In a prospective population-based study of EP-born neonates, flow data were obtained from the ventilator during the first 48 h of life. Data were logged for >10 min and then converted to flow–volume loops using custom-made software. Tidal breathing parameters were calculated and averaged from ≥200 breath cycles, and data were compared between those who later developed moderate/severe and no/mild BPD. Of 33 neonates, 18 developed moderate/severe and 15 no/mild BPD. The groups did not differ in gestational age, surfactant treatment or ventilator settings. The infants who developed moderate/severe BPD had evidence of less airflow obstruction, significantly so for tidal expiratory flow at 50% of tidal expiratory volume (TEF50 expressed as a ratio of peak tidal expiratory flow (PTEF (p=0.007. A compound model estimated by multiple logistic regression incorporating TEF50/PTEF, birthweight z-score and sex predicted moderate/severe BPD with good accuracy (area under the curve 0.893, 95% CI 0.735–0.973. This study suggests that flow data obtained from ventilators during the first hours of life may predict later BPD in premature neonates. Future and larger studies are needed to validate these findings and to determine their clinical usefulness.

  2. 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.

  3. Differences and similarities between bronchopulmonary dysplasia and asthma in schoolchildren.

    Science.gov (United States)

    Nordlund, Björn; James, Anna; Ebersjö, Christina; Hedlin, Gunilla; Broström, Eva B

    2017-09-01

    The long-term respiratory characteristics of ex-preterm children with bronchopulmonary dysplasia (BPD) are not established. The objective of this study was to describe hallmarks of BPD at school age in comparison to children with atopic asthma. This study was a cross-sectional descriptive comparative study in a hospital-based setting. Thirty schoolchildren diagnosed with BPD (10.4 years/born at 26.6 weeks' gestation) and 30 age- and sex-matched children with asthma and sensitized to airborne allergens (IgE >0.35 kU A /L) were analyzed. Measurements included fraction of exhaled nitric oxide (FENO, ppb), dynamic and static lung function, and bronchial provocation with methacholine (PD:20) and mannitol (PD:15), as well as an evaluation of respiratory symptoms using the asthma control test (C-ACT). Lung function measures (FEV1% 77 vs 84, FEV1/FVC% 85 vs 91, FEF50% 61 vs 80) and carbon monoxide diffusion capacity (DLCO%, 81 vs 88) were all reduced in children with BPD compared to asthma (P values asthma. However, less responsiveness towards mannitol (19% vs 61%, P = 0.007) and fewer self-reported symptoms (C-ACT, median 26 vs 24, P = 0.003) were found in the BPD group. Respiratory hallmarks of BPD at school-age were reduced lung function, limited responsiveness towards indirectly acting mannitol but hyper-responsiveness towards direct acting methacholine and impairment in diffusion capacity. Children with BPD displayed less evidence of airway inflammation compared with atopic asthma. © 2017 Wiley Periodicals, Inc.

  4. Impact of bronchopulmonary dysplasia on brain and retina

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    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.

  5. 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.

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

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    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.

  7. Understanding the Impact of Infection, Inflammation, and Their Persistence in the Pathogenesis of Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Balany, Jherna; Bhandari, Vineet

    2015-01-01

    The concerted interaction of genetic and environmental factors acts 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 (BPD). Adverse perinatal exposure to infection/inflammation with added insults like invasive mecha nical 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.

  8. Bronchopulmonary dysplasia in children: contemporary diagnosis, consequences and their treatment (update 2017

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    M.O. Gonchar

    2017-10-01

    Full Text Available The article deals with the modern principles of diagnosing bronchopulmonary dysplasia (BPD in children based on the guidelines of the American Thoracic and European Respiratory Societies. The article have aim to provide an optimal patient management. In modern world literature, BPD is considered as a diffuse parenchymal lung disease of preterm infants that require artificial ventilation and oxygen therapy for acute respiratory failure in the neonatal period. The form and degree of bronchopulmonary dysplasia severity are determined by the overall duration of oxygen dependence and gestational age. In infants, the disease can run with respiratory dysfunction, respiratory failure, asthma-like syndrome, increased susceptibility to respiratory infections, tobacco smoke, and pollutants. Some children with BPD have low tolerance to physical activity with hypersensitivity to hypoxia. 25–37 % of patients have pulmonary arterial hypertension. The article is focused on which should be paid attention when specifying the anamnesis of life and disease in the parents of the child. Current monitoring of the disease includes examination, evaluation of laboratory and instrumental studies. Analysis of the fibrobronchoscopy and bodipletismography data has the weak recommendation. Eliminating hypoxia, optimizing the nutritive status, determining the presence of an open arterial duct, specific drug therapy, and the use of vasodilators should be paid attention when treating a child with bronchopulmonary dysplasia. The article presents a chart for the monitoring of a child with BPD and indications for hospitalization of the patient, expediency and usefulness necessary for practicing pediatricians, family doctors, pediatric pulmonologists.

  9. 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.

  10. 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.

  11. A Shared Pattern of β-Catenin Activation in Bronchopulmonary Dysplasia and Idiopathic Pulmonary Fibrosis.

    Science.gov (United States)

    Sucre, Jennifer M S; Deutsch, Gail H; Jetter, Christopher S; Ambalavanan, Namasivayam; Benjamin, John T; Gleaves, Linda A; Millis, Bryan A; Young, Lisa R; Blackwell, Timothy S; Kropski, Jonathan A; Guttentag, Susan H

    2018-04-01

    Wnt/β-catenin signaling is necessary for normal lung development, and abnormal Wnt signaling contributes to the pathogenesis of both bronchopulmonary dysplasia (BPD) and idiopathic pulmonary fibrosis (IPF), fibrotic lung diseases that occur during infancy and aging, respectively. Using a library of human normal and diseased human lung samples, we identified a distinct signature of nuclear accumulation of β-catenin phosphorylated at tyrosine 489 and epithelial cell cytosolic localization of β-catenin phosphorylated at tyrosine 654 in early normal lung development and fibrotic lung diseases BPD and IPF. Furthermore, this signature was recapitulated in murine models of BPD and IPF. Image analysis of immunofluorescence colocalization demonstrated a consistent pattern of elevated nuclear phosphorylated β-catenin in the lung epithelium and surrounding mesenchyme in BPD and IPF, closely resembling the pattern observed in 18-week fetal lung. Nuclear β-catenin phosphorylated at tyrosine 489 associated with an increased expression of Wnt target gene AXIN2, suggesting that the observed β-catenin signature is of functional significance during normal development and injury repair. The association of specific modifications of β-catenin during normal lung development and again in response to lung injury supports the widely held concept that repair of lung injury involves the recapitulation of developmental programs. Furthermore, these observations suggest that β-catenin phosphorylation has potential as a therapeutic target for the treatment and prevention of both BPD and IPF. Copyright © 2018 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.

  12. 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)

  13. Rib enlargement in premature infants with bronchopulmonary dysplasia

    International Nuclear Information System (INIS)

    Yoon, Hye Kyung; Han, Kim Bokyung; Chang, Yun Sil; Choo, In Wook; Kim, Kyeong Ah

    2000-01-01

    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)

  14. Respiratory Syncytial Virus Lower Respiratory Tract Infections in Premature Infants and Infants with Bronchopulmonary Dysplasia

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    D. Yu. Ovsyannikov

    2015-01-01

    Full Text Available The article is devoted to the study of features of lower respiratory tract infection associated with respiratory syncytial virus. 40 cases of RSV-bronchiolitis in preterm children under year with/without bronchopulmonary dysplasia were analyzed. It was established that disease in those groups of patients had severe course because of the respiratory failure, which dominates in clinical pictures as symptoms of bronchial obstruction and apnea. Treatment of severe RSV-infection often demand admission to intensive care unit, supplemental oxygen and/or mechanical ventilation.

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

    Science.gov (United States)

    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.

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

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    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.

  17. 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.

  18. 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.

  19. Quercetin attenuates the hyperoxic lung injury in neonatal mice: Implications for Bronchopulmonary dysplasia (BPD).

    Science.gov (United States)

    Maturu, Paramahamsa; Wei-Liang, Yanhong; Androutsopoulos, Vasilis P; Jiang, Weiwu; Wang, Lihua; Tsatsakis, Aristides M; Couroucli, Xanthi I

    2018-04-01

    Quercetin (QU) is one of the most common flavonoids that are present in a wide variety of fruits, vegetables, and beverages. This compound possesses potent anti-inflammatory and anti-oxidant properties. Supplemental oxygen is routinely administered to premature infants with pulmonary insufficiency. However, hyperoxia is one of the major risk factors for the development of bronchopulmonary dysplasia (BPD), which is also termed chronic lung disease in premature infants. Currently, no preventive approaches have been reported against BPD. The treatment of BPD is notably limited to oxygen administration, ventilatory support, and steroids. Since QU has been shown to be effective in reducing inflammation and oxidative stress in various disease models, we hypothesized that the postnatal QU treatment of newborn mice will protect against hyperoxic lung injury by the upregulation of the phase I (CYP1A/B) and/or phase II, NADPH quinone reductase enzymes. Newborn C57BL/6J mice within 24 h of birth with the nursing dams were exposed to either 21% O 2 (air) and/or 85% O 2 (hyperoxia) for 7 days. The mice were treated, intraperitoneally (i.p.) once every other day with quercetin, at a concentration of 20 mg/kg, or saline alone from postnatal day (PND) 2-6. The mice were sacrificed on day 7, and lung and liver tissues were collected. The expression levels of CYP1A1, CYP1B1, NQO1 proteins and mRNA as well as the levels of MDA-protein adducts were analyzed in lung and liver tissues. The findings indicated that QU attenuated hyperoxia-mediated lung injury by reducing inflammation and improving alveolarization with decreased number of neutrophil and macrophage infiltration. The attenuation of this lung injury correlated with the upregulation of CYP1A1/CYP1B1/NQO1 mRNA, proteins and the down regulation of NF-kB levels and MDA-protein adducts in lung and liver tissues. The present study demonstrated the potential therapeutic value of quercetin in the prevention and/or treatment of

  20. Definition and outpatient management of the very low-birth-weight infant with bronchopulmonary dysplasia.

    Science.gov (United States)

    Groothuis, Jessie R; Makari, Doris

    2012-04-01

    Bronchopulmonary dysplasia (BPD), also known as chronic lung disease of prematurity, is the major cause of pulmonary disease in infants. The pathophysiology and management of BPD have evolved over the past four decades as improved neonatal intensive care unit (NICU) modalities have increased survival rates. The likelihood for developing BPD increases with the degree of prematurity and reaches 25-35% in very low-birth-weight and extremely low-birth-weight infants. BPD affects many organ systems, and infants with BPD are at increased risk for rehospitalization and numerous complications following NICU discharge. The management of BPD and medically related problems, particularly during the first 2 years of life, remains a continuing challenge for parents and healthcare providers. It is important that a multidisciplinary team consisting of the neonatologist/attending physician, primary care physician, and other specialized support staff work in concert and meet regularly to provide continuity of care and accurate patient assessments.

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

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    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.

  2. Bronchopulmonary dysplasia as a predictor factor for motor alteration at 6 months corrected age in premature infants

    OpenAIRE

    Martins,Priscila Silveira; Mello,Rosane Reis de; Silva,Kátia Silveira da

    2010-01-01

    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 mo...

  3. Use of corticosteroids and the outcome of infants with bronchopulmonary dysplasia

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    Mataloun Marta M. G. B.

    1999-01-01

    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 < 0.05 and smaller (height: Group I - 37.22 cm ± 3.3, Group II - 33.5 ± 2.4; p< 0.05 than Group I. At 40 weeks, there were no statistically significant differences between groups in relation to anthropometric measurements. CONCLUSIONS: The use of corticosteroids in bronchopulmonary dysplasic infants may influence the somatic growth during its use. However, after its suspension, a recovery seems to occur, suggesting that its influence could be transitory.

  4. 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.

  5. A three-dimensional human model of the fibroblast activation that accompanies bronchopulmonary dysplasia identifies Notch-mediated pathophysiology.

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    Sucre, Jennifer M S; Wilkinson, Dan; Vijayaraj, Preethi; Paul, Manash; Dunn, Bruce; Alva-Ornelas, Jackelyn A; Gomperts, Brigitte N

    2016-05-15

    Bronchopulmonary dysplasia (BPD) is a leading complication of premature birth and occurs primarily in infants delivered during the saccular stage of lung development. Histopathology shows decreased alveolarization and a pattern of fibroblast proliferation and differentiation to the myofibroblast phenotype. Little is known about the molecular pathways and cellular mechanisms that define BPD pathophysiology and progression. We have developed a novel three-dimensional human model of the fibroblast activation associated with BPD, and using this model we have identified the Notch pathway as a key driver of fibroblast activation and proliferation in response to changes in oxygen. Fetal lung fibroblasts were cultured on sodium alginate beads to generate lung organoids. After exposure to alternating hypoxia and hyperoxia, the organoids developed a phenotypic response characterized by increased α-smooth muscle actin (α-SMA) expression and other genes known to be upregulated in BPD and also demonstrated increased expression of downstream effectors of the Notch pathway. Inhibition of Notch with a γ-secretase inhibitor prevented the development of the pattern of cellular proliferation and α-SMA expression in our model. Analysis of human autopsy tissue from the lungs of infants who expired with BPD demonstrated evidence of Notch activation within fibrotic areas of the alveolar septae, suggesting that Notch may be a key driver of BPD pathophysiology. Copyright © 2016 the American Physiological Society.

  6. 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.  .

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

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    Villamor-Martínez, Eduardo; Pierro, Maria; Cavallaro, Giacomo; Mosca, Fabio; Kramer, Boris W; Villamor, Eduardo

    2018-02-20

    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.

  8. 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.

  9. 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.

  10. 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.

  11. Development of a proxy-reported pulmonary outcome scale for preterm infants with bronchopulmonary dysplasia

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    Laughon Matthew M

    2011-07-01

    Full Text Available Abstract Background To develop an accurate, proxy-reported bedside measurement tool for assessment of the severity of bronchopulmonary dysplasia (also called chronic lung disease in preterm infants to supplement providers' current biometric measurements of the disease. Methods We adapted Patient-Reported Outcomes Measurement Information System (PROMIS methodology to develop the Proxy-Reported Pulmonary Outcomes Scale (PRPOS. A multidisciplinary group of registered nurses, nurse practitioners, neonatologists, developmental specialists, and feeding specialists at five academic medical centers participated in the PRPOS development, which included five phases: (1 identification of domains, items, and responses; (2 item classification and selection using a modified Delphi process; (3 focus group exploration of items and response options; (4 cognitive interviews on a preliminary scale; and (5 final revision before field testing. Results Each phase of the process helped us to identify, classify, review, and revise possible domains, questions, and response options. The final items for field testing include 26 questions or observations that a nurse assesses before, during, and after routine care time and feeding. Conclusions We successfully created a prototype scale using modified PROMIS methodology. This process can serve as a model for the development of proxy-reported outcomes scales in other pediatric populations.

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

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    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.

  13. Low postnatal serum IGF-I levels are associated with bronchopulmonary dysplasia (BPD).

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    Löfqvist, Chatarina; Hellgren, Gunnel; Niklasson, Aimon; Engström, Eva; Ley, David; Hansen-Pupp, Ingrid

    2012-12-01

    To characterize postnatal changes in serum insulin-like growth factor-1 (IGF-I) in relation to development of bronchopulmonary dysplasia (BPD) in very preterm infants. Longitudinal study of 108 infants with mean (SD) gestational age (GA) 27.2 (2.2) weeks. Weekly serum samples of IGF-I were analysed from birth until postmenstrual age (PMA) 36 weeks. Multivariate models were developed to identify independent predictors of BPD. Postnatal mean IGF-I levels at postnatal day (PND) 3-21 were lower in infants with BPD compared with infants with no BPD (16 vs. 26 μg/L, p BPD compared with infants with no BPD (0.28 vs. 0.97, p = 0.002) and mean IGF-I during PMA 30-33 weeks was lower in infants with BPD as compared with infants without BPD (22 vs. 29 μg/L, p BPD (r(2) = 0.634, p BPD. © 2012 The Author(s)/Acta Paediatrica © 2012 Foundation Acta Paediatrica.

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

    International Nuclear Information System (INIS)

    Soler, C.; Figueras, J.; Roca, I.; Perez, J.M.; Jimenez, R.

    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

  15. 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.

  16. Ceramides in tracheal aspirates of preterm infants: Marker for bronchopulmonary dysplasia.

    Directory of Open Access Journals (Sweden)

    Esther van Mastrigt

    Full Text Available In an experimental mouse model we showed that ceramides play a role in the pathogenesis of bronchopulmonary dysplasia (BPD and are a potential target for therapeutic intervention. We investigated whether ceramides are detectable in tracheal aspirates (TAs of preterm infants and differ between infants with or without BPD.Infants born ≤ 32 weeks of gestational age in need of mechanical ventilation in the first week of life were included. TAs were obtained directly after intubation and at day 1, 3, 5, 7, and 14. Ceramide concentrations were measured by tandem mass spectrometry. At 36 weeks postmenstrual age BPD was defined as having had ≥ 28 days supplemental oxygen.122 infants were included, of which 14 died and 41 developed BPD. All infants showed an increase in ceramides after the first day of intubation. The ceramide profile differed significantly between preterm infants who did and did not develop BPD. However, the ceramide profile had no additional predictive value for BPD development over GA at birth, birth weight and total days of mechanical ventilation.Ceramides are measurable in TAs of preterm born infants and may be an early marker for BPD development.

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

    Science.gov (United States)

    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.

  18. 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.

  19. Urinary metabolomics of bronchopulmonary dysplasia (BPD): preliminary data at birth suggest it is a congenital disease.

    Science.gov (United States)

    Fanos, Vassilios; Pintus, Maria Cristina; Lussu, Milena; Atzori, Luigi; Noto, Antonio; Stronati, Mauro; Guimaraes, Hercilia; Marcialis, Maria Antonietta; Rocha, Gustavo; Moretti, Corrado; Papoff, Paola; Lacerenza, Serafina; Puddu, Silvia; Giuffrè, Mario; Serraino, Francesca; Mussap, Michele; Corsello, Giovanni

    2014-10-01

    Bronchopulmonary dysplasia (BPD) or chronic lung disease is one of the principal causes of mortality and morbidity in preterm infants. Early identification of infants at the greater risk of developing BPD may allow a targeted approach for reducing disease severity and complications. The trigger cause of the disease comprehends the impairment of the alveolar development and the increased angiogenesis. Nevertheless, the molecular pathways characterizing the disease are still unclear. Therefore, the use of the metabolomics technique, due to the capability of identifying instantaneous metabolic perturbation, might help to recognize metabolic patterns associated with the condition. The purpose of this study is to compare urinary metabolomics at birth in 36 newborns with a gestational age below 29 weeks and birth weight BPD. Urine samples were collected within 24-36 h of life and immediately frozen at -80 °C. The (1)H-NMR spectra were analyzed using a partial least squares discriminant analysis (PLS-DA) model coupled with orthogonal Signal Correction. Using this approach it was possible with urine at birth to discriminate newborns that will be later have a diagnosis of BPD with a high statistics power. In particular, we found five important discriminant metabolites in urine in BPD newborns: lactate, taurine, TMAO, myoinositol (which increased) and gluconate (which decreased). These preliminary results seem to be promising for the identification of predictor's biomarkers characterizing the BPD condition. These data may suggest that BPD is probably the result of an abnormal development (respiratory bud, vascular tree, hypodysplasia of pneumocytes) and could be considered a congenital disease (genetics plus intrauterine epigenetics). Early identification of infants at the greater risk of developing BPD may allow a targeted approach for reducing disease severity and complications.

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

    Directory of Open Access Journals (Sweden)

    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.

  1. Role of biofilm formation in Ureaplasma antibiotic susceptibility and development of bronchopulmonary dysplasia in preterm neonates.

    Science.gov (United States)

    Pandelidis, Katherine; McCarthy, Amanda; Chesko, Kirsty L; Viscardi, Rose M

    2013-04-01

    Ureaplasma respiratory tract colonization is a risk factor for bronchopulmonary dysplasia (BPD) in preterm infants, but whether Ureaplasma isolates from colonized infants can form biofilms is unknown. We hypothesized that Ureaplasma isolates vary in capacity to form biofilms that contribute to their antibiotic resistance and ability to evade host immune responses. Study objectives were to (1) determine the ability of Ureaplasma isolates from preterm neonates to form biofilms in vitro; (2) compare the susceptibility of the sessile and planktonic organisms to azithromycin (AZI) and erythromycin; and (3) determine the relationship of biofilm-forming capacity in Ureaplasma isolates and the risk for BPD. Forty-three clinical isolates from preterm neonates and 5 American Tissue Culture Collection strains were characterized for their capacity to form biofilms in vitro, and antibiotic susceptibility was performed on each isolate prebiofilm and postbiofilm formation. Forty-one (95%) clinical and 4 of 5 (80%) American Tissue Culture Collection isolates formed biofilms. All isolates were more susceptible to AZI (minimum inhibitory concentration, MIC50 2 µg/mL) than erythromycin (MIC50 4 µg/mL), and biofilm formation did not significantly affect antibiotic susceptibility for the 2 tested antibiotics. The MIC50 and minimum biofilm inhibitory concentrations (MBIC50) for Ureaplasma urealyticum clinical isolates for AZI were higher than for MIC50 and MBIC50 for Ureaplasma parvum isolates. There were no differences in MIC or MBICs among isolates from BPD infants and non-BPD infants. Capacity to form biofilms is common among Ureaplasma spp. isolates, but biofilm formation did not impact MICs for AZI or erythromycin.

  2. Blood Cytokine Profiles Associated with Distinct Patterns of Bronchopulmonary Dysplasia among Extremely Low Birth Weight Infants.

    Science.gov (United States)

    D'Angio, Carl T; Ambalavanan, Namasivayam; Carlo, Waldemar A; McDonald, Scott A; Skogstrand, Kristin; Hougaard, David M; Shankaran, Seetha; Goldberg, Ronald N; Ehrenkranz, Richard A; Tyson, Jon E; Stoll, Barbara J; Das, Abhik; Higgins, Rosemary D

    2016-07-01

    To explore differences in blood cytokine profiles among distinct bronchopulmonary dysplasia (BPD) patterns. We evaluated blood spots collected from 943 infants born at ≤1000 g and surviving to 28 days on postnatal days 1, 3, 7, 14, and 21 for 25 cytokines. Infants were assigned to the following lung disease patterns: (1) no lung disease (NLD); (2) respiratory distress syndrome without BPD; (3) classic BPD (persistent exposure to supplemental oxygen until 28 days of age); or (4) atypical BPD (period without supplemental oxygen before 28 days). Median cytokine levels for infants with BPD were compared with the IQR of results among infants with NLD. The distribution of enrolled infants by group was as follows: 69 (NLD), 73 (respiratory distress syndrome), 381 (classic BPD), and 160 (atypical BPD). The remaining 260 infants could not be classified because of missing data (104) or not fitting a predefined pattern (156). Median levels of 3 cytokines (elevated interleukin [IL]-8, matrix metalloproteinase-9; decreased granulocyte macrophage colony-stimulating factor) fell outside the IQR for at least 2 time points in both infants with atypical and classic BPD. Profiles of 7 cytokines (IL-6, IL-10, IL-18, macrophage inflammatory protein-1α, C-reactive protein, brain-derived neurotrophic factor, regulated on activation, normal T cell expressed and secreted) differed between infants with classic and atypical BPD. Blood cytokine profiles may differ between infants developing classic and atypical BPD. These dissimilarities suggest the possibility that differing mechanisms could explain the varied patterns of pathophysiology of lung disease in extremely premature infants. Copyright © 2016 Elsevier Inc. All rights reserved.

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

    Science.gov (United States)

    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.

  4. 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.

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

    Directory of Open Access Journals (Sweden)

    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

  6. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  8. 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

    BACKGROUND: 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. OBJECTIVES: 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. METHODS: 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...

  9. Need for Supplemental Oxygen at Discharge in Infants with Bronchopulmonary Dysplasia Is Not Associated with Worse Neurodevelopmental Outcomes at 3 Years Corrected Age

    OpenAIRE

    Lodha, Abhay; Sauvé, Reg; Bhandari, Vineet; Tang, Selphee; Christianson, Heather; Bhandari, Anita; Amin, Harish; Singhal, Nalini

    2014-01-01

    OBJECTIVES: To determine if chronic oxygen dependency (discharge home on supplemental oxygen) in children with bronchopulmonary dysplasia (BPD; defined as requirement for supplemental O2 at 36 weeks postmenstrual age) predicts neurodevelopmental disability rates and growth outcomes at 36 months corrected age (CA). STUDY DESIGN: Longitudinal cohort study. SETTING: Southern Alberta regional center located at high altitude. PARTICIPANTS: Preterm infants weighing ≤1250 grams with no BPD, BPD, and...

  10. 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.

  11. Association of Noninvasive Ventilation Strategies With Mortality and Bronchopulmonary Dysplasia Among Preterm Infants: A Systematic Review and Meta-analysis.

    Science.gov (United States)

    Isayama, Tetsuya; Iwami, Hiroko; McDonald, Sarah; Beyene, Joseph

    2016-08-09

    Various noninvasive ventilation strategies are used to prevent bronchopulmonary dysplasia (BPD) of preterm infants; however, the best mode is uncertain. To compare 7 ventilation strategies for preterm infants including nasal continuous positive airway pressure (CPAP) alone, intubation and surfactant administration followed by immediate extubation (INSURE), less invasive surfactant administration (LISA), noninvasive intermittent positive pressure ventilation, nebulized surfactant administration, surfactant administration via laryngeal mask airway, and mechanical ventilation. MEDLINE, EMBASE, CINAHL, and Cochrane CENTRAL from their inceptions to June 2016. Randomized clinical trials comparing ventilation strategies for infants younger than 33 weeks' gestational age within 24 hours of birth who had not been intubated. Data were independently extracted by 2 reviewers and synthesized with Bayesian random-effects network meta-analyses. A composite of death or BPD at 36 weeks' postmenstrual age was the primary outcome. Death, BPD, severe intraventricular hemorrhage, and air leak by discharge were the main secondary outcomes. Among 5598 infants involved in 30 trials, the incidence of the primary outcome was 33% (1665 of 4987; including 505 deaths and 1160 cases of BPD). The secondary outcomes ranged from 6% (314 of 5587) for air leak to 26% (1160 of 4455) for BPD . Compared with mechanical ventilation, LISA had a lower odds of the primary outcome (odds ratio [OR], 0.49; 95% credible interval [CrI], 0.30-0.79; absolute risk difference [RD], 164 fewer per 1000 infants; 57-253 fewer per 1000 infants; moderate quality of evidence), BPD(OR, 0.53; 95% CrI, 0.27-0.96; absolute RD, 133 fewer per 1000 infants; 95% CrI, 9-234 fewer per 1000 infants; moderate-quality), and severe intraventricular hemorrhage (OR, 0.44; 95% CrI, 0.19-0.99; absolute RD, 58 fewer per 1000 births; 95% CrI, 1-86 fewer per 1000 births; moderate-quality). Compared with nasal CPAP alone, LISA had a lower odds

  12. A single nucleotide polymorphism in the dimethylarginine dimethylaminohydrolase gene is associated with lower risk of pulmonary hypertension in bronchopulmonary dysplasia

    Science.gov (United States)

    Trittmann, JK; Gastier-Foster, JM; Zmuda, EJ; Frick, J; Rogers, LK; Vieland, VJ; Chicoine, LG; Nelin, LD

    2016-01-01

    Aim Pulmonary hypertension (PH) develops in 25–40% of bronchopulmonary dysplasia (BPD) patients, substantially increasing mortality. We have previously found that asymmetric dimethylarginine (ADMA), an endogenous inhibitor of nitric oxide (NO) production, is elevated in patients with BPD-associated PH. ADMA is metabolized by NG,NG- dimethylarginine dimethylaminohydrolase (DDAH). Presently, we test the hypothesis that there are single nucleotide polymorphisms (SNPs) in DDAH1 and/or DDAH2 associated with the development of PH in BPD patients. Methods BPD patients were enrolled (n=98) at Nationwide Children’s Hospital. Clinical characteristics and 36 SNPs in DDAH1 and DDAH2 were compared between BPD-associated PH patients (cases) and BPD-alone patients (controls). Results In BPD patients, 25 (26%) had echocardiographic evidence of PH (cases). In this cohort, DDAH1 wildtype rs480414 was 92% sensitive and 53% specific for PH in BPD, and the DDAH1 SNP rs480414 decreased the risk of PH in an additive model of inheritance (OR=0.39; 95% CI [0.18–0.88], p=0.01). Conclusion The rs480414 SNP in DDAH1 may be protective against the development of PH in patients with BPD. Furthermore, the DDAH1 rs480414 may be a useful biomarker in developing predictive models for PH in patients with BPD. PMID:26663142

  13. [Somatic and psychomotor development of preterm infants at the age of 2 years, with and without bronchopulmonary dysplasia].

    Science.gov (United States)

    Kwinta, Przemko; Klimek, Małgorzata; Pietrzyk, Jacek J

    2005-01-01

    The assessment of psychomotor and somatic development at the age of 2 years of preterm infants with and without bronchopulmonary dysplasia (BPD). Case -- control study. Neonatal Intensive Care Unit, University Hospital. 62 preterm infants born before 32 weeks of gestation, with mean birth weight equaled to 1112 g were included into 2 groups: group A -- with BPD (n=31), group B (control) -- matched by sex, birth weight, gestational age (n=31). Evaluation of reaching milestones, neurological examination at 3, 9, 12, 18-24 months, Psyche-Cattel tests at 12 and 24 months, hearing and visions examinations, head, chest circumference, length and weight at 3, 6, 9, 12 and 24 months. Age of unsupported sitting and walking, intelligence quotient (IQ), cerebral palsy, hearing and sight impairment. Reaching motor milestones were similar in the both groups (unsupported sitting (mean) 10.4 vs. 8.7 months, unsupported walking: 15.1 vs. 13.9 months). Cerebral palsy occurred more (non-significantly) frequently in group A than in group B (7/31 vs. 2/31, p=0.15). Significantly lower IQ was found in group A at the age of 1 year in comparison with group B (x +/- SEM: 79.9 +/- 2.1 vs. 86.1 +/- 1.5, p=0.03) and at the age of 2 years (x +/- SEM: 89.2 +/- 2.7 vs. 96.0 +/- 2.1, p=0.06). Weight, length, head and chest circumferences at the term of delivery were similar in the both groups, but at the age of 9 and 24 months the group A children weighted less than group B (respectively: 7432 vs. 8128 g, 10010 vs. 11116; pdevelopment of preterm newborns.

  14. 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.

  15. Antenatal steroids and risk of bronchopulmonary dysplasia: a lack of effect or a case of over-adjustment?

    Science.gov (United States)

    Gagliardi, Luigi; Bellù, Roberto; Rusconi, Franca; Merazzi, Daniele; Mosca, Fabio

    2007-07-01

    Although antenatal steroids reduce risk factors for bronchopulmonary dysplasia (BPD) in preterm infants, their effect on BPD is conflicting. We hypothesised that the lack of protective effect found in some studies could derive from over-adjustment during analysis, caused by controlling for factors intermediate in the causal pathway between treatment and outcome. We prospectively studied a cohort of infants 23-32 weeks gestation steroids. In univariable analysis, steroids were not significantly protective against BPD; some intermediate factors (mechanical ventilation, greater severity of illness as measured by Clinical Risk Index for Babies score, patent ductus arteriosus) were significantly positively associated with (i.e. were risk factors for) BPD (OR = 11.0, 1.55, 4.42, respectively, all P steroids (OR = 0.58, 0.92, and 0.58, respectively, all P steroid-treated infants had a lower risk of BPD (OR 0.59 [95% CI 0.36, 0.97], P = 0.036); male sex (OR = 2.08), late-onset sepsis (OR = 4.26), and birthweight (OR = 0.63 for 100 g increase) were also associated with BPD, all P effect of steroids disappeared; ventilation (OR = 3.03), increased illness severity (OR = 1.11), and patent ductus arteriosus (OR = 1.90) were significant risk factors. This study suggests that including variables that are potential mediators in the causal chain can obscure the ability to detect a protective effect of treatment. We observed such a phenomenon in our analyses of the relationship between antenatal steroids and BPD, suggesting that steroid effect is partly mediated through a reduction in the classical risk factors.

  16. [Risk factors, frequency and severity of bronchopulmonary dysplasia (BPD) diagnosed according to the new disease definition in preterm neonates].

    Science.gov (United States)

    Woynarowska, Martyna; Rutkowska, Magdalena; Szamotulska, Katarzyna

    2008-01-01

    Bronchopulmonary dysplasia (BPD) is a chronic lung disease of prematurity. The new definition of BPD was introduced in 2001. It combined two existing BPD definitions - oxygen dependency at 28 days of life and at 36 weeks of postmenstrual age. New definition divided BPD into three forms: mild, moderate and severe. Despite significant progress in neonatal intensive care and increasing survival of extremely immature infants the incidence of BPD is not decreasing. The etiopathology of new disease is still discussed. The aim of the study was to assess the frequency and severity of BPD in very low birth weight (VLBW) neonates (BPD definition. We retrospectively analyzed the case records of 244 neonates boys - 112; girls - 132) at gestational age weeks 23-28 (26.4+/-1.4) treated at the Institute of Mother and Child in Warsaw between 1999 and 2004. Of these 166 (68%) infants were evaluable at 36 weeks of postmenstrual age and had BPD evaluation according to the new definition. The following variables were analyzed according to BPD status: sex distribution, gestational age, body weight at birth severity, of respiratory distress syndrome (RDS), intrauterine or late infections, congenital or late pneumonia, patent ductus arteriosus (PDA) and intraventricular haemorrhage (IVH) grade III or IV. BPD was present in 126 (76%) neonates including: severe BPD in 23 (18%), moderate 19 (15%) and mild BPD in 84 (67%) of children. Neonates with BPD had lower gestational age (26.4+/-1.3 vs 27.4+/-0.9 week, pBPD. The rates of late onset sepsis, late pneumonia and PDA were statistically higher in children with BPD than in those without BPD (respectively 70 vs 33%, 72 vs 35% and 51 vs 20% pBPD had higher rate of late infections (pBPD in VLBV neonates is high (76%) but in the majority of cases the disease is mild (67%); 2. Severe BPD was more common in neonates with late onset sepsis and IVH grade III or IV; 3. The BPD risk factors are: low gestational age, low birth weight, as well as late

  17. 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

  18. What Is Bronchopulmonary Dysplasia?

    Science.gov (United States)

    ... blood from the heart to the lungs to pick up oxygen. Cor pulmonale. Cor pulmonale is failure ... to interact more with you and the NICU staff, start talking, and develop other skills. While your ...

  19. Prenatal administration of the cytochrome P4501A inducer, Β-naphthoflavone (BNF), attenuates hyperoxic lung injury in newborn mice: Implications for bronchopulmonary dysplasia (BPD) in premature infants

    International Nuclear Information System (INIS)

    Couroucli, Xanthi I.; Liang Yanhong Wei; Jiang Weiwu; Wang Lihua; Barrios, Roberto; Yang Peiying; Moorthy, Bhagavatula

    2011-01-01

    Supplemental oxygen contributes to the development of bronchopulmonary dysplasia (BPD) in premature infants. In this investigation, we tested the hypothesis that prenatal treatment of pregnant mice (C57BL/6J) with the cytochrome P450 (CYP)1A1 inducer, ss-napthoflavone (BNF), will lead to attenuation of lung injury in newborns (delivered from these dams) exposed to hyperoxia by mechanisms entailing transplacental induction of hepatic and pulmonary CYP1A enzymes. Pregnant mice were administered the vehicle corn oil (CO) or BNF (40 mg/kg), i.p., once daily for 3 days on gestational days (17-19), and newborns delivered from the mothers were either maintained in room air or exposed to hyperoxia (> 95% O 2 ) for 1-5 days. After 3-5 days of hyperoxia, the lungs of CO-treated mice showed neutrophil infiltration, pulmonary edema, and perivascular inflammation. On the other hand, BNF-pretreated neonatal mice showed decreased susceptibility to hyperoxic lung injury. These mice displayed marked induction of ethoxyresorufin O-deethylase (EROD) (CYP1A1) and methoxyresorufin O-demethylase (MROD) (CYP1A2) activities, and levels of the corresponding apoproteins and mRNA levels until PND 3 in liver, while CYP1A1 expression alone was augmented in the lung. Prenatal BNF did not significantly alter gene expression of pulmonary NAD(P)H quinone reductase (NQO1). Hyperoxia for 24-72 h resulted in increased pulmonary levels of the F 2 -isoprostane 8-iso-PGF 2α , whose levels were decreased in mice prenatally exposed to BNF. In conclusion, our results suggest that prenatal BNF protects newborns against hyperoxic lung injury, presumably by detoxification of lipid hydroperoxides by CYP1A enzymes, a phenomenon that has implications for prevention of BPD in infants. - Highlights: → Supplemental oxygen is routinely administered to premature infants. → Hyperoxia causes lung injury in experimental animals. → Prenatal treatment of mice with beta-naphthoflavone attenuates oxygen injury

  20. 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

    OpenAIRE

    Priscila Silveira Martins; Rosane Reis de Mello; Kátia Silveira da Silva

    2010-01-01

    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 mo...

  1. 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

    Directory of Open Access Journals (Sweden)

    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.

  2. Clara Cell Protein Expression in Mechanically Ventilated Term and Preterm Infants with Respiratory Distress Syndrome and at Risk of Bronchopulmonary Dysplasia: A Pilot Study

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    José Guzmán-Bárcenas

    2017-01-01

    Full Text Available The aim of this pilot study was to determine Clara cell protein (CC16 concentration in bronchoalveolar lavages (BAL fluid from full-term and preterm (<37 weeks’ gestational age neonates requiring respiratory support, having symptoms of neonatal respiratory distress syndrome, and at risk of bronchopulmonary dysplasia (BPD. We hypothesized that CC16 may be predictive of BPD diagnosis regardless of gestational age. BAL fluid CC16 was measured by ELISA at birth and at day 7 of life. Both groups that developed BPD showed significantly decreased BAL fluid CC16 levels compared to those infants that did not develop the disease. CC16 positively correlated with diagnosis of BPD and negatively with the severity of the disease. These results suggest that BAL fluid CC16 levels may have a diagnostic value at day 7 for BPD in both term and preterm infants. This study demonstrates the potential utility of BAL fluid CC16 levels as a biomarker for BPD in term infants.

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

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    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.

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

    Science.gov (United States)

    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.

  5. Clara Cell Protein Expression in Mechanically Ventilated Term and Preterm Infants with Respiratory Distress Syndrome and at Risk of Bronchopulmonary Dysplasia: A Pilot Study

    Science.gov (United States)

    Calderón-Moore, Antonio; Baptista-González, Héctor

    2017-01-01

    The aim of this pilot study was to determine Clara cell protein (CC16) concentration in bronchoalveolar lavages (BAL) fluid from full-term and preterm (<37 weeks' gestational age) neonates requiring respiratory support, having symptoms of neonatal respiratory distress syndrome, and at risk of bronchopulmonary dysplasia (BPD). We hypothesized that CC16 may be predictive of BPD diagnosis regardless of gestational age. BAL fluid CC16 was measured by ELISA at birth and at day 7 of life. Both groups that developed BPD showed significantly decreased BAL fluid CC16 levels compared to those infants that did not develop the disease. CC16 positively correlated with diagnosis of BPD and negatively with the severity of the disease. These results suggest that BAL fluid CC16 levels may have a diagnostic value at day 7 for BPD in both term and preterm infants. This study demonstrates the potential utility of BAL fluid CC16 levels as a biomarker for BPD in term infants. PMID:28487624

  6. 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

    Directory of Open Access Journals (Sweden)

    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.

  7. A pilot study to assess short-term physiologic outcomes of transitioning infants with severe bronchopulmonary dysplasia from ICU to two subacute ventilators.

    Science.gov (United States)

    DiBlasi, Robert M; Crotwell, Dave N; Poli, Jonathan; Hotz, Justin; Cogen, Jonathan D; Carter, Edward

    2018-01-01

    This study was designed to evaluate short-term physiologic outcomes of transitioning neonates with bronchopulmonary dysplasia (BPD) from intensive care unit (ICU) ventilators to both the Trilogy 202 (Philips Healthcare, Andover, MA) and LTV 1200 (CareFusion, Yorba Linda, CA) subacute ventilators. Six infants with BPD requiring tracheostomies for support with a neonatal-specific ICU ventilator underwent placement of esophageal balloon catheters, airway pressure transducers, flow sensors, oxygen saturation (SpO 2 ), and end tidal carbon dioxide (P ET CO 2 ) monitors. Noninvasive gas exchange, airflow, and airway and esophageal pressures (P ES ) were recorded following 20 min on the ICU ventilator. The infants were placed on the Trilogy 202 and LTV 1200 ventilators in random order at identical settings as the ICU ventilator. We measured noninvasive gas exchange, pressure-rate product (respiratory rate × ΔP ES ), ventilator response times, and the percentage of spontaneous breaths that triggered the ventilator at 20 min in each subject while being supported with each of the different subacute ventilators. The mean (SD) weight of the six infants was 4.983 (0.56) kg. There were no differences in heart rate ( p = 0.51) or SpO 2 ( p = 0.97) but lower P ET CO 2 , ΔP ES , respiratory rate, pressure rate-product, response times, and greater percentage of subject initiated breaths that triggered the ventilator ( p ventilator to the Trilogy 202 ventilator. In this small group of infants with BPD, the Trilogy 202 ventilator performed better than the LTV 1200. The improved subject efforts, per cent subject triggering, and response times observed with the Trilogy are likely related to differences in triggering algorithms, location of triggering mechanisms, and gas delivery system performance within the ventilators. These pilot data may be useful for informing future clinical study design and understanding differences in the level of support provided by different subacute

  8. 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

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    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.

  9. The ontogeny of human pulmonary angiotensin-converting enzyme and its aberrant expression may contribute to the pathobiology of bronchopulmonary dysplasia (BPD).

    Science.gov (United States)

    Castro, E C; Parks, W T; Galambos, C

    2014-10-01

    The mammalian lung possesses the highest level of angiotensin converting enzyme (ACE) amongst all the organs. ACE is known to generate angiotensin (AT)-II from AT-I and to regulate serum bradykinin level, thereby controlling blood pressure. Recent data, however, indicate a role for ACE derived AT-II in angiogenesis, pulmonary hypertension, and neonatal lung disease. The ontogeny of ACE in humans has not been investigated. We studied pulmonary ACE expression during human lung development and in human bronchopulmonary dysplasia (BPD). Human fetal autopsy lung tissue representing all three trimesters (12, 13, 16, 18, 24, 34, 39, and 40 weeks of gestational age (WGA)), as well as from 1 to 10 years of age with no significant lung pathology were used. In addition lung sections of patients with BPD (n = 5) were selected. The slides were immunostained using an anti-ACE monoclonal antibody. The temporal and spatial pattern of ACE expression was contrasted to that of the pan-endothelial marker CD31. Staining intensity was graded. Mildly diffuse and strong microvascular endothelial immunreactivity for ACE was seen in the human fetus as early as 12 WGA. ACE expression peaked at mid gestation and remained high throughout gestation and postnatally. In BPD lungs ACE endothelial staining was largely absent, and when focal staining was observed the intensity was weak. We established that ACE expression is present in the human fetal lung as early as 12 WGA, remains active pre- and postnatally, and ACE expression was downregulated in BPD lungs. We speculate that ACE may be involved in the process of lung development. © 2013 Wiley Periodicals, Inc.

  10. 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.

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

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    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.

  12. 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.

  13. Diagnosis, prevention, and management of canine hip dysplasia: a review

    Directory of Open Access Journals (Sweden)

    Schachner ER

    2015-05-01

    Full Text Available Emma R Schachner, Mandi J Lopez Department of Veterinary Clinical Sciences, School of Veterinary Medicine, Louisiana State University, Baton Rouge, LA, USA Abstract: Canine hip dysplasia (CHD is a polygenic and multifactorial developmental disorder characterized by coxofemoral (hip joint laxity, degeneration, and osteoarthritis (OA. Current diagnostic techniques are largely subjective measures of joint conformation performed at different stages of development. Recently, measures on three-dimensional images generated from computed tomography scans predicted the development of OA associated with CHD. Continued refinement of similar imaging methods may improve diagnostic imaging techniques to identify dogs predisposed to degenerative hip joint changes. By current consensus, joint changes consistent with CHD are influenced by genetic predisposition as well as environmental and biomechanical factors; however, despite decades of work, the relative contributions of each to the development and extent of CHD signs remain elusive. Similarly, despite considerable effort to decipher the genetic underpinnings of CHD for selective breeding programs, relevant genetic loci remain equivocal. As such, prevention of CHD within domestic canine populations is marginally successful. Conservative management is often employed to manage signs of CHD, with lifelong maintenance of body mass as one of the most promising methods. Surgical intervention is often employed to prevent joint changes or restore joint function, but there are no gold standards for either goal. To date, all CHD phenotypes are considered as a single entity in spite of recognized differences in expression and response to environmental conditions and treatment. Identification of distinct CHD phenotypes and targeting evidence-based conservative and invasive treatments for each may significantly advance prevention and management of a prevalent, debilitating condition in canine companions. Keywords: canine

  14. 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

    Directory of Open Access Journals (Sweden)

    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

  15. 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

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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

  17. Oxidative stress in experimental bronchopulmonary dysplasia

    NARCIS (Netherlands)

    Horst, Simone Antonia Johanna ter

    2008-01-01

    Due to a lack of patient materials and ethical reasons animal models of BPD are critical for characterization the pathophysiology of BPD and testing of potential treatment options. In chapter 2 of this thesis we characterize a rat model for experimental BPD, induced in neonatal pups by prolonged

  18. 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

    Directory of Open Access Journals (Sweden)

    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

  19. Acetabuloplasties at Open Reduction Prevent Acetabular Dysplasia in Intentionally Delayed Developmental Dysplasia of the Hip: A Case-control Study.

    Science.gov (United States)

    Carsi, M Belen; Clarke, Nicholas M P

    2016-05-01

    Avascular necrosis (AVN) and residual acetabular dysplasia are the two main complications of developmental dysplasia of the hip (DDH) treatment. Although early reduction of the hip may decrease the incidence of residual dysplasia, it may increase the incidence of AVN and vice versa. However, we do not know if changes in surgical technique may lead to a modification in these outcomes. Does an incomplete periacetabular acetabuloplasty, as an added step to delayed open reduction, (1) diminish the risk of developing acetabular dysplasia; or (2) increase the rate of AVN compared with patients treated with open reduction alone? We conducted a retrospective matched case-control study comparing 22 patients (27 hips) with early isolated DDH who underwent intentionally delayed open reduction and acetabuloplasty from 2004 to 2010 and followed up > 4 years (88% of the cohort) with early historic controls treated with delayed open reduction alone. Of 53 patients available for matching, 45 (85%) had enough followup (> 10 years) to be considered. They were matched one to one for age at presentation and bilaterality (fuzz 45, 0). This generated a control group of 25 patients (27 hips). The mean followup was different between the groups (p dysplasia considered when center-edge angle 30° and pelvic osteotomies were used as our primary outcomes. The proportion of patients with AVN was also compared. Patients treated with open reduction and an incomplete periacetabular acetabuloplasty were less likely to develop acetabular dysplasia and undergo pelvic osteotomies than were patients in the control group (0% [zero of 27] versus 37% [10 of 27]; odds ratio [OR], 11; 95% confidence interval [CI], 2-80; p = 0.02 and 0% [zero of 27] versus 26% [seven of 27]; OR, 8; 95% CI, 1-60; p = 0.025, respectively). With the available numbers, there was no difference in terms of the proportion of patients who developed AVN (11 of 27 [41%] both groups; OR, 1; 95% CI, 1-2; p = 1). The addition of an

  20. Primary prevention of skin dysplasia in renal transplant recipients with photodynamic therapy

    DEFF Research Database (Denmark)

    Togsverd-Bo, K; Omland, S H; Wulf, H C

    2015-01-01

    Organ transplant recipients (OTRs) are at high risk of developing cutaneous squamous cell carcinoma (SCC); prevention includes early treatment of premalignant actinic keratosis (AK). Photodynamic therapy (PDT) is a noninvasive field therapy that reduces new AKs in patients with existing AK...... and delays SCC development in mice. We investigated the effect of repeated PDT over 5 years for primary prophylaxis of skin dysplasia. These data represent an interim analysis of an on-going randomized controlled trial. During 2008-2011, 25 renal transplant recipients with clinically normal skin were...

  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

    Directory of Open Access Journals (Sweden)

    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. VACCINE IMMUNIZATION FOR PREVENTION OF PNEUMOCOCCAL, HAEMOPHILUS INFLUENZAE AND FLU AMONG SICKLY CHILDREN, WHO OFTEN SUFFER FROM PERSISTENT HETEROSPECIFIC INFECTIOUS PATHOLOGY OF THE BRONCHOPULMONARY SYSTEM

    Directory of Open Access Journals (Sweden)

    L.I. Ilienko

    2006-01-01

    Full Text Available Among serious diseases of the lower respiratory tract a special place is taken by pneumonias and chronic infectious respiratory diseases caused by pneumococcus and Haemophilus influenzae type b (HIB. The research purpose is to determine the effectiveness of vaccine combined application to treat sickly children, who often suffer from persistent infectious pathology of the respiratory tract, for flu, pneumococcal and HIB disease. 110 children aged between 3 and 12 have been vaccinated. The first part of research implied children vaccination by means of Actahib and Pneumo 23 vaccines (Sanofi Pasteur, France, the second one consisted in immunization of children with the same pathology by means of Pneumo 23, Actahib and Vaxigrip vaccines (Sanofi Pasteur, France. The researches established that within a year after HIB and Pneumo 23 vaccination the frequency of upper and lower respiratory tract acerbations reduced by 2,3 times on average; likewise, the number of system antimicrobial dosage reduced by 7,4 times along with the total duration of dosage; the carrier state of S. pneumoniae reduced by 3,7 times, H. influenzae — by 3,9 times. In the course of application of three vaccines, the frequency of persistent heat erospecific infectious bronchopulmonary pathology acerbations reduced by 3,3 times. The carrier state of S. pneumoniae reduced by 2,5 times, H. influenzae — by 4,1 times. Thus, vaccine immunization to treat for flu, pneumococcal and HIB disease in various combinations may be recoma mended for wider application to reduce the frequency and severity of heat erospecific infectious respiratory diseases among sickly children, who often suffer from various illnesses.Key words: children with recurrent diseases, vaccination, prevention, flu, H. Influenzae, S. pneumoniae.

  3. Endosonography in bronchopulmonary disease

    DEFF Research Database (Denmark)

    Vilmann, Peter; Annema, Jouke; Clementsen, Paul

    2009-01-01

    a major impact of EUSFNA as well as EBUS-TBNA on management of patients with lung cancer as well as in patients with unknown lesions in the mediastinum. The aim of the present review is to discuss the current role of endosonography in bronchopulmonary diseases focusing on endosonographically guided biopsy...... via the esophagus, trachea and main bronchi. The concept of complete echo-endoscopic staging of lung cancer is postulated as virtually all mediastinal nodes as well as regions relevant to pulmonal medicine (liver and adrenal glands) can be reached by these two methods in combination....

  4. Allergic Bronchopulmonary Aspergillosis

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    Michael C. Tracy

    2016-06-01

    Full Text Available Allergic bronchopulmonary aspergillosis (ABPA, a progressive fungal allergic lung disease, is a common complication of asthma or cystic fibrosis. Although ABPA has been recognized since the 1950s, recent research has underscored the importance of Th2 immune deviation and granulocyte activation in its pathogenesis. There is also strong evidence of widespread under-diagnosis due to the complexity and lack of standardization of diagnostic criteria. Treatment has long focused on downregulation of the inflammatory response with prolonged courses of oral glucocorticosteroids, but more recently concerns with steroid toxicity and availability of new treatment modalities has led to trials of oral azoles, inhaled amphotericin, pulse intravenous steroids, and subcutaneously-injected anti-IgE monoclonal antibody omalizumab, all of which show evidence of efficacy and reduced toxicity.

  5. Effectiveness of Synchronized Noninvasive Ventilation to Prevent Intubation in Preterm Infants

    OpenAIRE

    Ramos-Navarro, Cristina; Sanchez-Luna, Manuel; Sanz-L?pez, Ester; Maderuelo-Rodriguez, Elena; Zamora-Flores, Elena

    2016-01-01

    Background?Noninvasive ventilation is being increasingly used on preterm infants to reduce ventilator lung injury and bronchopulmonary dysplasia. The aim of this study was to evaluate the effectiveness of synchronized nasal intermittent positive pressure ventilation (SNIPPV) to prevent intubation in premature infants. Methods?Prospective observational study of SNIPPV use on preterm infants of less than 32 weeks' gestation. All patients were managed using a prospective protocol intended to red...

  6. Neonatal Treatment with Recombinant Ectodysplasin Prevents Respiratory Disease in Dogs with X-Linked Ectodermal Dysplasia

    OpenAIRE

    Mauldin, Elizabeth A.; Gaide, Olivier; Schneider, Pascal; Casal, Margret L.

    2009-01-01

    Patients with defective ectodysplasin A (EDA) have X-linked hypohidrotic ectodermal dysplasia (XLHED; OMIM#305100), a condition comprising hypotrichosis, inability to sweat, abnormal teeth, and frequent pulmonary infections. The XLHED dogs show the same clinical signs as humans with the disorder, including frequent respiratory infections that can be fatal. The respiratory disease in humans and dogs is thought to be due to the absence of tracheal and bronchial glands which are a vital part of ...

  7. Kidney Dysplasia

    Science.gov (United States)

    ... as the fetus grows in the womb. In kidney dysplasia, the tubules fail to branch out completely. Urine that would ... to form a network of tiny structures called tubules. Kidney dysplasia in one kidney What are the kidneys ...

  8. Imaging Bronchopulmonary Dysplasia—A Multimodality Update

    Directory of Open Access Journals (Sweden)

    Thomas Semple

    2017-06-01

    Full Text Available 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.

  9. Clinical Utility of Echocardiography for the Diagnosis and Prognosis in Children with Bronchopulmonary Dsyplasia

    OpenAIRE

    Choi, Young Earl; Cho, Hwa Jin; Song, Eun Song; Jeong, In Seok; Yoon, Namsik; Choi, Young Youn; Ma, Jae Sook; Cho, Young Kuk

    2016-01-01

    Background Bronchopulmonary dysplasia (BPD) may result in chronic pulmonary artery hypertension and right ventricular (RV) dysfunction. Various echocardiographic assessments of RV dysfunction have been used to determine whether echocardiographic measurements of premature infants with BPD could provide sensitive measures of RV function that correlates with BPD severity. Methods Twenty-eight control subjects without BPD (non BPD group), 28 patients with mild BPD, 11 patients with moderate BPD, ...

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

    African Journals Online (AJOL)

    There was no episode of apneic attack throughout admission. She responded to treatment and was discharged home on intermittent oxygen therapy and nebulisation. The weight on discharge was 1.6kg. At 6 months of age, she is still having recurrent respiratory distress and supplemental oxygen at home. She is regular to ...

  11. 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...

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

    DEFF Research Database (Denmark)

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

    2013-01-01

    . The incidence of PH was 23% among infants with BPD. Furthermore, we found a significantly larger frequency of intubation at birth, postnatal infection, longer duration of continuous positive airway pressure treatment and use of oxygen therapy among infants with PH and BPD than among the remaining infants...

  13. Neonatal treatment with recombinant ectodysplasin prevents respiratory disease in dogs with X-linked ectodermal dysplasia.

    Science.gov (United States)

    Mauldin, Elizabeth A; Gaide, Olivier; Schneider, Pascal; Casal, Margret L

    2009-09-01

    Patients with defective ectodysplasin A (EDA) have X-linked hypohidrotic ectodermal dysplasia (XLHED; OMIM#305100), a condition comprising hypotrichosis, inability to sweat, abnormal teeth, and frequent pulmonary infections. The XLHED dogs show the same clinical signs as humans with the disorder, including frequent respiratory infections that can be fatal. The respiratory disease in humans and dogs is thought to be due to the absence of tracheal and bronchial glands which are a vital part of the mucociliary clearance mechanism. In our XLHED model, the genetically missing EDA was replaced by postnatal intravenous administration of recombinant EDA resulting in long-term, durable corrective effect on adult, permanent dentition. After treatment with EDA, significant correction of the missing tracheal and bronchial glands was achieved in those dogs that received higher doses of EDA. Moreover, successful treatment resulted in the presence of esophageal glands, improved mucociliary clearance, and the absence of respiratory infection. These results demonstrate that a short-term treatment at a neonatal age with a recombinant protein can reverse a developmental disease and result in vastly improved quality of life. (c) 2009 Wiley-Liss, Inc.

  14. Campomelic Dysplasia

    African Journals Online (AJOL)

    Adele

    2004-05-03

    May 3, 2004 ... Coscia MF, Bassett GS, Bowen JR et al. Spinal abnormalities in camptomelic dysplasia. J Pediatr Ortho 1989; 9:6-14. 10. Hall BD, Spranger JW. Campomelic dysplasia: Further elucidation of a distinct entity. Am J Dis Child 1980;134: 285-9. 11. Houston CS, Opitz JM, Spranger JW et al. The campomelic syn ...

  15. Oculodentodigital dysplasia

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    Dharmil C Doshi

    2016-01-01

    Full Text Available Oculodentodigital dysplasia is a rare, autosomal dominant disorder with high penetrance and variable expressivity, caused by mutations in the connexin 43 or gap junction protein alpha-1 gene. It has been diagnosed in fewer than 300 people worldwide with an incidence of around 1 in 10 million. It affects many parts of the body, particularly eyes (oculo, teeth (dento, and fingers and/or toes (digital. The common clinical features include facial dysmorphism with thin nose, microphthalmia, syndactyly, tooth anomalies such as enamel hypoplasia, anodontia, microdontia, early tooth loss and conductive deafness. Other less common features are abnormalities of the skin and its appendages, such as brittle nails, sparse hair, and neurological abnormalities. To prevent this syndrome from being overlooked, awareness of possible symptoms is necessary. Early recognition can prevent blindness, dental problems and learning disabilities. Described here is the case of a 21-year-old male who presented to the ophthalmology outpatient department with a complaint of bilateral progressive loss of vision since childhood.

  16. Cervical Dysplasia

    Science.gov (United States)

    ... pass through. Cervical dysplasia is detected in a pap test (pap smear), and diagnosed in a biopsy. Abnormal ... American Academy of Family Physicians (AAFP) recommends routine pap tests to diagnose cervical cancer early. You can check ...

  17. 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.

  18. Evaluation of a training program for general ultrasound screening for developmental dysplasia of the hip in preventive child health care

    NARCIS (Netherlands)

    Boere-Boonekamp, Magdalena M.; Ramwadhdoebe, S.; Sakkers, R.J.B.; Uiterwaal, Cuno S.P.M.; Beek, Frederik J.A.

    2010-01-01

    Background: A research study in the Netherlands showed that general ultrasound (US) screening was cost-effective in the detection of developmental dysplasia of the hip (DDH). This study was followed by a pilot implementation study. Part of this pilot implementation study is to investigate whether

  19. 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.

  20. Frontofacionasal Dysplasia

    African Journals Online (AJOL)

    rme

    226. Frontofacionasal Dysplasia: Another Observation eye. Other ocular features include small eyes, iris colobomas and cataracts. A frontally situated lipoma, causing a swelling at the nasion, was reported in one patient and an encephalocele has been described.3. Mental retardation has been reported in one patient with.

  1. Mondini Dysplasia Presenting as Otorrhea without Meningitis

    OpenAIRE

    Chien-Yu Lin; Hung-Ching Lin; Chun-Chih Peng; Kuo-Sheng Lee; Nan-Chang Chiu

    2012-01-01

    Mondini dysplasia is a rare inner ear malformation that is usually only diagnosed after recurrent meningitis. Surgical intervention is mandatory. This report highlights the case of a patient with Mondini dysplasia who presented with hearing impairment and otorrhea and was diagnosed and treated before the occurrence of meningitis, thus preventing morbidity and neurologic sequelae. Hearing impairment may be the only manifestation of Mondini dysplasia, and the benefit of hearing screening is emp...

  2. 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.

  3. Mondini dysplasia

    International Nuclear Information System (INIS)

    Takeda, Taizo; Kakigi, Akinobu; Takeuchi, Shunji; Saito, Haruo

    1992-01-01

    Recent advances in imaging technique, including high resolution thin section computed tomography scanning and magnetic resonance imaging (MRI), permit the easy diagnosis of congenital malformations of the osseous labyrinth, which have so far been lumped together as 'Mondini dysplasia'. In the present study, the anatomic patterns from the radiogrpahic appearance and the clinical manifestation were examined in 18 patients (23 ears) with radiographic abnormalities of the inner ear. The most common abnormal configuration was a large vestibule (20 of 23 ears). This anomaly of the vestibule often involved the lateral semicircular canal, revealed as a round high signal zone on T2-weighted MRI. However, a large vestibule was not always associated with an abnormal cochlea. Abnormal cochleas were found in 8 of the 20 ears with a large vestibule, and most of these 8 ears had total or profound deafness. But 4 of the 10 ears with residual hearing had low tone deafness and 6 had fluctuating hearing loss, which was frequently associated with attacks of dizziness. These clinical manifestations of Mondini dysplasia are similar to those of patients with endolymphatic hydrops. (author)

  4. Diagnosis and treatment of bronchopulmonary neuroendocrine tumours

    DEFF Research Database (Denmark)

    Tabaksblat, Elizaveta Mitkina; Langer, Seppo W; Knigge, Ulrich

    2016-01-01

    Bronchopulmonary neuroendocrine tumours (BP-NET) are a heterogeneous population of neoplasms with different pathology, clinical behaviour and prognosis compared to the more common lung cancers. The management of BP-NET patients is largely based on studies with a low level of evidence...

  5. Chondroectodermal dysplasia: a rare syndrome.

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    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.

  6. Epithelial Dysplasia in Oral Cavity

    Directory of Open Access Journals (Sweden)

    Samaneh Shirani

    2014-09-01

    Full Text Available Among oral lesions, we encounter a series of malignant epithelial lesions that go through clinical and histopathologic processes in order to be diagnosed. Identifying these processes along with the etiology knowledge of these lesions is very important in prevention and early treatments. Dysplasia is the step preceding the formation of squamous cell carcinoma in lesions which have the potential to undergo dysplasia. Identification of etiological factors, clinical and histopathologic methods has been the topic of many articles. This article, reviews various articles presenting oral cavity dysplasia, new clinical methods of identifying lesions, and the immunohistochemical research which proposes various markers for providing more precise identification of such lesions. This article also briefly analyzes new treatment methods such as tissue engineering.

  7. 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, ...

  8. Congenital bronchopulmonary foregut malformations: concepts and controversies

    International Nuclear Information System (INIS)

    Newman, Beverley

    2006-01-01

    This article addresses the scope, etiology, important associations and imaging features of congenital bronchopulmonary foregut malformations. Etiologic concepts, including airway obstruction and vascular anomalies, are highlighted. Technical imaging advances, especially CT and MR, have greatly enhanced our diagnostic abilities in evaluating these lesions; however, thorough and careful assessment of all aspects of the malformation is still necessary. Several specific lesions are discussed in more detail, particularly regarding controversial issues in classification, understanding, imaging and management. (orig.)

  9. Congenital bronchopulmonary foregut malformations: concepts and controversies

    Energy Technology Data Exchange (ETDEWEB)

    Newman, Beverley [University of Pittsburgh School of Medicine and Children' s Hospital of Pittsburgh, Pittsburgh, PA (United States)

    2006-08-15

    This article addresses the scope, etiology, important associations and imaging features of congenital bronchopulmonary foregut malformations. Etiologic concepts, including airway obstruction and vascular anomalies, are highlighted. Technical imaging advances, especially CT and MR, have greatly enhanced our diagnostic abilities in evaluating these lesions; however, thorough and careful assessment of all aspects of the malformation is still necessary. Several specific lesions are discussed in more detail, particularly regarding controversial issues in classification, understanding, imaging and management. (orig.)

  10. Analysis of two cases with bronchopulmonary neurofibromatosis

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    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.

  11. Putative Bronchopulmonary Flagellated Protozoa in Immunosuppressed Patients

    Directory of Open Access Journals (Sweden)

    Ali Ahmet Kilimcioglu

    2014-01-01

    Full Text Available Flagellated protozoa that cause bronchopulmonary symptoms in humans are commonly neglected. These protozoal forms which were presumed to be “flagellated protozoa” have been previously identified in immunosuppressed patients in a number of studies, but have not been certainly classified so far. Since no human cases of bronchopulmonary flagellated protozoa were reported from Turkey, we aimed to investigate these putative protozoa in immunosuppressed patients who are particularly at risk of infectious diseases. Bronchoalveolar lavage fluid samples of 110 immunosuppressed adult patients who were admitted to the Department of Chest Diseases, Hafsa Sultan Hospital of Celal Bayar University, Manisa, Turkey, were examined in terms of parasites by light microscopy. Flagellated protozoal forms were detected in nine (8.2% of 110 cases. Metronidazole (500 mg b.i.d. for 30 days was given to all positive cases and a second bronchoscopy was performed at the end of the treatment, which revealed no parasites. In conclusion, immunosuppressed patients with bronchopulmonary symptoms should attentively be examined with regard to flagellated protozoa which can easily be misidentified as epithelial cells.

  12. Putative bronchopulmonary flagellated protozoa in immunosuppressed patients.

    Science.gov (United States)

    Kilimcioglu, Ali Ahmet; Havlucu, Yavuz; Girginkardesler, Nogay; Celik, Pınar; Yereli, Kor; Özbilgin, Ahmet

    2014-01-01

    Flagellated protozoa that cause bronchopulmonary symptoms in humans are commonly neglected. These protozoal forms which were presumed to be "flagellated protozoa" have been previously identified in immunosuppressed patients in a number of studies, but have not been certainly classified so far. Since no human cases of bronchopulmonary flagellated protozoa were reported from Turkey, we aimed to investigate these putative protozoa in immunosuppressed patients who are particularly at risk of infectious diseases. Bronchoalveolar lavage fluid samples of 110 immunosuppressed adult patients who were admitted to the Department of Chest Diseases, Hafsa Sultan Hospital of Celal Bayar University, Manisa, Turkey, were examined in terms of parasites by light microscopy. Flagellated protozoal forms were detected in nine (8.2%) of 110 cases. Metronidazole (500 mg b.i.d. for 30 days) was given to all positive cases and a second bronchoscopy was performed at the end of the treatment, which revealed no parasites. In conclusion, immunosuppressed patients with bronchopulmonary symptoms should attentively be examined with regard to flagellated protozoa which can easily be misidentified as epithelial cells.

  13. Role of embolisation in the treatment of bronchopulmonary sequestration

    International Nuclear Information System (INIS)

    Curros, F.; Chigot, V.; Emond, S.; Sayegh, N.; Brunelle, F.; Revillon, Y.; Scheinman, P.; Lebourgeois, M.

    2000-01-01

    Background. Sequestrations represent bronchopulmonary malformations that are increasingly diagnosed antenatally. After birth, the therapeutic approach in asymptomatic children is debated, as some may spontaneously regress. Objective. To evaluate the efficacy of embolisation of the feeding systemic artery in the treatment of bronchopulmonary sequestration. Materials and methods. Sixteen children with bronchopulmonary sequestration were treated by endovascular embolisation of the feeding systemic artery. Results. Ten patients were considered cured by embolisation alone. One patient was operated on after unsuccessful embolisation, three had partial regression of the lung mass and two are still under follow-up. Conclusions. Our experience indicates that bronchopulmonary sequestrations in children can be treated by embolisation alone. (orig.)

  14. Mondini Dysplasia Presenting as Otorrhea without Meningitis

    Directory of Open Access Journals (Sweden)

    Chien-Yu Lin

    2012-12-01

    Full Text Available Mondini dysplasia is a rare inner ear malformation that is usually only diagnosed after recurrent meningitis. Surgical intervention is mandatory. This report highlights the case of a patient with Mondini dysplasia who presented with hearing impairment and otorrhea and was diagnosed and treated before the occurrence of meningitis, thus preventing morbidity and neurologic sequelae. Hearing impairment may be the only manifestation of Mondini dysplasia, and the benefit of hearing screening is emphasized. Temporal bone computed tomography should be considered in children with unilateral sensorineural or mixed-type hearing impairment.

  15. Mondini dysplasia presenting as otorrhea without meningitis.

    Science.gov (United States)

    Lin, Chien-Yu; Lin, Hung-Ching; Peng, Chun-Chih; Lee, Kuo-Sheng; Chiu, Nan-Chang

    2012-12-01

    Mondini dysplasia is a rare inner ear malformation that is usually only diagnosed after recurrent meningitis. Surgical intervention is mandatory. This report highlights the case of a patient with Mondini dysplasia who presented with hearing impairment and otorrhea and was diagnosed and treated before the occurrence of meningitis, thus preventing morbidity and neurologic sequelae. Hearing impairment may be the only manifestation of Mondini dysplasia, and the benefit of hearing screening is emphasized. Temporal bone computed tomography should be considered in children with unilateral sensorineural or mixed-type hearing impairment. Copyright © 2012. Published by Elsevier B.V.

  16. 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

  17. Prevalence of bronchopulmonary pathology in the participants of Chernobyl Nuclear Power Plant accident response

    International Nuclear Information System (INIS)

    Antonov, N.S.; Yakushin, S.P.; Stulova, O.Yu.; Zajtseva, O.Yu.; Stroev, E.A.

    1998-01-01

    Epidemiologic examination of the participants of the Chernobyl accident response is performed. Fact of acute effect of the Chernobyl aerosol inhalation on respiratory organs is found. Prevalence of bronchopulmonary diseases in participants of accident response is almost 2 times higher than that in reference group. Further program of investigations includes the hospital stage and the preventive measures at prehospital stage under ambulatory conditions. Assessments of the efficiency of performed treatment - prophylactic measures and their economic benefit are made [ru

  18. 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

  19. Progressive pseudorheumatoid dysplasia misdiagnosed as ...

    African Journals Online (AJOL)

    Progressive pseudorheumatoid dysplasia misdiagnosed as seronegative juvenile idiopathic arthritis. Ozgur Taspinar, Fatih Kelesoglu, Yasar Keskin, Murat Uludag. Abstract. Background: Progressive pseudorheumatoid dysplasia (PPD) is a rare spondylo- epi-metaphyseal dysplasia (SEMD). It can be confused with juvenile ...

  20. Oral epithelial dysplasia classification systems

    DEFF Research Database (Denmark)

    Warnakulasuriya, S; Reibel, J; Bouquot, J

    2008-01-01

    . 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...

  1. 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

  2. Oculoauriculovertebral dysplasia (Goldenhar's syndrome).

    Science.gov (United States)

    Nkrumah, F K

    1971-03-01

    A case of Goldenhar's Syndrome or Oculoauriculovertebral dysplasia in a Ghanaian infant is described. Significant were the additional findings of congenital esophageal atresia and arthrogryposis which have so far not been reported in association with the syndrome.

  3. 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.)

  4. 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.

  5. Correlation among cytokines, bronchopulmonary dysplasia and modality of ventilation in preterm newborns: improvement with melatonin treatment.

    Science.gov (United States)

    Gitto, Eloisa; Reiter, Russel J; Sabatino, Giuseppe; Buonocore, Giuseppe; Romeo, Carmelo; Gitto, Placido; Buggé, Concetta; Trimarchi, Giuseppe; Barberi, Ignazio

    2005-10-01

    Improved survival because of advances in neonatal care has resulted in an increased number of infants at risk for chronic lung disease. Even though the etiology of lung injury is multifactorial, recent animal and clinical data indicate that pulmonary damage depends in large part on the ventilatory strategies used. Ventilator-associated lung injury was believed to result from the use of high pressure, thus, the term barotraumas. This trauma is believed to involve free-radical damage. Oxidant injury is a serious cause of lung injury. In the present study, 110 newborns with respiratory distress syndrome were studied; 55 were treated with melatonin and the other 55 with placebo. All the subjects were mechanically ventilated with or without guaranteed volume. Proinflammatory cytokines [interleukin (IL)-6, IL-8 and tumor necrosis factor (TNF)-alpha] were measured in tracheobronchial aspirate and the clinical outcome was evaluated. Melatonin treatment reduced the proinflammatory cytokines and improved the clinical outcome. The beneficial action of melatonin presumably related to its antioxidative actions.

  6. Tension pneumothorax at anaesthetic induction in an ex-premature infant with bronchopulmonary dysplasia.

    Science.gov (United States)

    Freed, Chayim; Guha, Ranjan

    2012-09-11

    ME, a 12-week-old premature infant with a history of complicated ventilatory support at birth, was referred to as a large tertiary centre for urgent retinal laser photocoagulation for retinopathy of prematurity. Following routine induction by a consultant paediatric anaesthetist, immediate ventilation difficulties were encountered, associated with a distended abdomen. Rapid assessment and escalation of the situation occurred, including assistance from several consultants. ME was diagnosed as having a pneumothorax. Rapid intervention, including needle decompression and insertion of a chest drain stabilised the patient. The procedure was cancelled, and the patient returned to intensive care. Over the next few days the patient gradually improved, and was gradually stepped down. The patient continued to improve, successfully overcoming the pneumothorax, pneumonia and insertion of a ventriculoperitoneal shunt, and eventually was discharged back to the referring hospital.

  7. Developmental dysplasia of the hip

    Science.gov (United States)

    ... dislocation of the hip joint; Developmental hip dysplasia; DDH; Congenital dysplasia of the hip; Congenital dislocation of ... during pregnancy can increase a baby's risk of DDH. Other risk factors include: Being the first child ...

  8. 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.

  9. Bronchoplasty for Primary Broncho-Pulmonary Tumors

    International Nuclear Information System (INIS)

    ABDEL RAHMAN, A.M.

    2010-01-01

    Parenchyma-sparing procedures are widely used in patients with low-grade malignancies of the airway when anatomically suited lesions exist. This study was conducted to evaluate the short-term and the long-term results of bronchoplastic procedures for patients with centrally located primary bronchopulmonary tumors. Methods: Between 2000 and 2009, 36 patients with primary lung tumors required bronchoplasty were retrospectively analyzed. Preoperative assessment included computed tomography (CT) of the chest, bronchoscopy, and spirometry. Pre operative diagnosis was achieved by bronchoscopy for all patients, mediastinoscopy was done for patients with primary lung cancer. Neo adjuvant chemotherapy was given for 6 patients with non small cell lung cancer (NSCLC). Results: We had 15 males and 21 female, the mean age was 37 years and the mean hospital stay was 7.2 days. Operative procedures performed were:Sleeve lobectomy in 30 patients (13 right, 17 left), partial sleeve right pneumonectomy in 3 and bronchial resection with re-anastomosis in 3 (2 left, 1 right). Twelve patients (33.3%) suffered post-operative problems. There was one operative related mortality. Post operative pathology revealed: 27 patients with typical carcinoid, 2 with atypical carcinoid, 4 with squamous cell carcinoma, 2 with adenocarcifioma and one with hamartoma. Pathological TNM staging revealed: 17 patients with stage 1A, 11 with IB, 5 with IIA and 2 with stage IIIA. Follow-up data were available for all patients except two. Two patients died with disseminated disease 1.5 year and 2 years after surgery. The patient with hamartoma developed local recurrence 5 years later and re-excision was done. One patient with lung cancer developed bone metastases and was alive with disease, while the remaining 30 patient's were alive and disease free. The overall 5 years survival was 83.3%. Conclusion: Bronchoplastic resections achieve local control and long-term survival comparable to the standard resections in

  10. 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.

  11. 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...

  12. 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.

  13. 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...... of various paraclinical parameters in the diagnosis of ABPA in patients with CF....

  14. 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.

  15. Developmental hip dysplasia in adolescence

    Directory of Open Access Journals (Sweden)

    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.

  16. 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

  17. 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.

  18. 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.

  19. Management of allergic bronchopulmonary aspergillosis: a review and update.

    Science.gov (United States)

    Mahdavinia, Mahboobeh; Grammer, Leslie C

    2012-06-01

    Since the first description of allergic bronchopulmonary aspergillosis (ABPA) in the 1950s there have been numerous studies that have shed light on the characteristics and immunopathogenesis of this disease. The increased knowledge and awareness have resulted in earlier diagnosis and treatment of patients with this condition. This article aims to provide a summary and updates on ABPA by reviewing the results of recent studies on this disease with a focus on articles published within the last 5 years. A systematic search of PubMed/Medline with keywords of ABPA or allergic bronchopulmonary aspergillosis was performed. All selected articles were reviewed with a focus on findings of articles published from December 2006 to December 2011. The relevant findings are summarized in this paper.

  20. A Clinical Evaluation of Definitive and Clinical Allergic Bronchopulmonary Mycosis

    OpenAIRE

    Matsuse, Hiroto; Nakata, Hiroko; Fukahori, Susumu; Tsuchida, Tomoko; Kawano, Tetsuya; Tomari, Shinya; Fukushima, Chizu; Matsuo, Nobuko; Asai, Sadahiro; Kohno, Shigeru

    2006-01-01

    Objective: The present study aims to overcome problems associated with the early diagnosis of allergic bronchopulmonary mycosis (ABPM) using the current criteria. Patients and Methods: Clinical features including radiographic findings from 10 patients with definitive ABPM based on the diagnostic criteria of Rosenberg-Patterson were compared with those from 9 patients with ABPM clinically diagnosed by respiratory allergy specialists. Results: ABPM should be considered in patients with peripher...

  1. 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...

  2. Hypohidrotic ectodermal dysplasia: a clinical case with a longitudinal approach.

    Science.gov (United States)

    Fraiz, Fabian Calixto; Gugisch, Renato Cordeiro; Cavalcante-Leão, Bianca Lopes; Macedo, Liliane Moreira

    2014-11-01

    This paper describes a clinical case of a male with hypohidrotic ectodermal dysplasia submitted to rehabilitation and occlusal dental interventions with follow-up from 3 to 14 years of age. Due to the severe effects on function and esthetics, the clinical manifestations of ectodermal dysplasia exert a negative impact on quality of life. However, oral rehabilitation in childhood poses a challenge due to growth and development. A male with hypohidrotic ectodermal dysplasia began dental intervention at the age of 3 years. The clinical and radiographic exams revealed the absence of several primary and permanent teeth and abnormal shape of the primary maxillary incisors. The facial characteristics were compatible with hypohidrotic ectodermal dysplasia, such as a prominent brow, everted lips, fattened bridge of the nose and small vertical facial height. The treatment proposed involved rehabilitation through successive temporary partial dentures, functional orthopedics of the jaws, esthetic reconstruction of the anterior teeth, timely occlusal intervention and preventive actions for the control of dental caries and plaque. The present case demonstrates that early care plays a fundamental role in minimizing the biopsychosocial consequences of hypohidrotic ectodermal dysplasia and preparing the patient for future oral rehabilitation. Although, the literature offers a number of papers describing dental treatment for ectodermal dysplasia, few cases include long-term follow-up and the use of a functional orthopedic appliance in combination with removable dentures in such patients.

  3. Occurrence of allergic bronchopulmonary mycosis in patients with asthma: An Eastern India experience

    Directory of Open Access Journals (Sweden)

    Sarkar Anirban

    2010-01-01

    Full Text Available Background: Allergic bronchopulmonary mycosis (ABPM is a clinical syndrome associated with immune sensitivity to various fungi notably Aspergillus spp. that colonize the airways of asthmatics. Early diagnosis and treatment with systemic corticosteroids is the key in preventing the progression of the disease to irreversible lung fibrosis. Aims: To study the occurrence of ABPM among asthma patients with fungal sensitization attending a chest clinic of a tertiary hospital of eastern India. The clinico-radiological and aetiological profiles are also described. Materials and Methods: All consecutive patients with asthma presenting to the chest clinic over a period of one year were screened for cutaneous hypersensitivity to 12 common fungal antigens. The skin test positive cases were further evaluated for ABPM using standard criteria. Results: One hundred and twenty-six asthma patients were screened using twelve common fungal antigens; forty patients (31.74% were found to be skin test positive, and ABPM was diagnosed in ten patients (7.93%. Of the 10 cases of ABPM, nine cases were those of allergic bronchopulmonary aspergillosis (ABPA and one case was identified as caused by sensitization to Penicillium spp. A majority of the cases of ABPM had advanced disease and had significantly lower FEV1 compared to non-ABPM skin test positive asthmatics. Central bronchiectasis on high resolution CT scan was the most sensitive and specific among the diagnostic parameters. Conclusion: There is a significant prevalence of ABPM in asthma patients attending our hospital and this reinforces the need to screen asthma patients for fungal sensitisation. This will help in early diagnosis and prevention of irreversible lung damage.

  4. Skeletal dysplasias: 38 prenatal cases.

    Science.gov (United States)

    Witters, I; Moerman, Ph; Fryns, J P

    2008-01-01

    To assess the prenatal diagnosis of skeletal dysplasias in a single center over a ten-years period. All antenatal detected skeletal dysplasias during the period January 1st 1996 until December 31 2005 (10 years) were retrieved from the genetic database. This database includes all skeletal dysplasias where invasive prenatal diagnosis (chorionic villus sampling/amniocentesis) was performed. The final diagnosis was sought on the basis of fetopathological examination, radiographic studies and if possible molecular testing. A total of 46 antenatal skeletal dysplasias were diagnosed during this period. Follow-up was only available in 38 cases. The other 8 cases involved prenatally presumed lethal skeletal dysplasias that were interrupted in the referral hospital with no further information sent to us. The mean gestational age at diagnosis was 23 weeks (range 12-33 weeks). A diagnosis 30 weeks (29%) and these included all achondroplasias (n = 6), hypophosphatasia (n = 1), Jeune syndrome (n = 1), osteogenesis imperfecta type II (n = l), type I (n = 1) and type III (n = 1). In 27 cases a lethal skeletal dysplasia was present (71%) and these were all correctly predicted. Of the lethal skeletal dysplasias 5 cases were diagnosed only after 24 weeks of pregnancy (19%) and 3 were only referred after 30 weeks (11.5%). A final diagnosis was obtained in 36 cases by fetopathological examination and radiographic studies and molecular testing as deemed necessary. Specific diagnoses included: achondroplasia (n = 6), achondrogenesis (n = 2), osteogenesis imperfecta type II (n = 9), osteogenesis imperfecta type I (n = 1), osteogenesis imperfecta type III (n = 1), thanatophoric dysplasia (n = 7), hypophosphatasia (n = 1), Majewski syndrome (n = 11), Mohr-Majewski syndrome (n = 11), Jeune syndrome (n = 2), Ellis-van Creveld syndrome (n = 2), Roberts syndrome (n = 1), campomelic dysplasia (n = 2). In two cases postnatal investigation revealed no certain diagnosis and these included one

  5. Undifferentiated connective tissue dysplasia in adolescents

    Directory of Open Access Journals (Sweden)

    G. Yu. Kalayeva

    2014-01-01

    Full Text Available Objective: to study the spectrum and frequency of dysplasia-dependent and connective tissue dysplasia-associated disorders in adolescents. One hundred and ten 10-to-14-year-old pupils of one of the Leninsk-Kuznetsky schools were examined. The presence and degree of undifferentiated connective tissue dysplasia (UCTD were established according to the phenotypic characters; joint mobility was rated using the standard Beighton methods. The adolescents showed a high prevalence of the signs of UCTD whose degree corresponded to that of joint hypermobility. The latter was accompanied by joint pain in 89 (33,3% adolescents in the study group or concurrent with scoliosis in 77,8% and platypodia in 22,2%. In a control group (я=29, scoliosis and platypodia were 2 and 3,3 times rarer, respectively; complaints of arthralgia were absent. After 3 years, the number of adolescents with platypodia increased up to 33,3% in the study group and up to 10,3% in the control group; that of teenagers with scoliosis did up to 81,5and 41,4%, respectively. A larger number of adolescents with CTD were noted to have vegetovascular disorders, more commonly with parasympathotonia with insufficient autonomic performance support. A great difference was retained in the frequency of myopia and biliary dyskinesia and in the incidence of respiratory infections. Thus, the high prevalence of dysplasia-dependent disorders in adolescents suggests that there is a need for the early detection of the signs of CTD in children to timely implement a package of prevention and health-improvement measures.

  6. Right pulmonary aplasia, aberrant left pulmonary artery, and bronchopulmonary sequestration with an esophageal bronchus

    International Nuclear Information System (INIS)

    Lee, Peter; McCauley, Roy; Westra, Sjirk; Baba, Timothy

    2006-01-01

    Pulmonary aplasia and bronchopulmonary foregut malformations in which a patent communication between the foregut and the pulmonary system is present are rare congenital abnormalities. Pulmonary aplasia associated with a pulmonary sling is an even rarer abnormality. We report a unique case of right pulmonary aplasia, aberrant left pulmonary artery, and bronchopulmonary sequestration with an esophageal bronchus diagnosed by multidetector helical CT. (orig.)

  7. Histologic Evidence of Intrapulmonary Bronchopulmonary Anastomotic Pathways in Neonates with Meconium Aspiration Syndrome.

    Science.gov (United States)

    Ali, Noorjahan; Abman, Steven H; Galambos, Csaba

    2015-12-01

    We examined lung histology from 8 infants who died with meconium aspiration syndrome in order to determine the presence of intrapulmonary bronchopulmonary anastomotic pathways. Each infant required mechanical ventilation to treat hypoxemic respiratory distress. Lung histology from each infant shows evidence of prominent bronchopulmonary vascular connections. Copyright © 2015 Elsevier Inc. All rights reserved.

  8. Congenital bronchopulmonary malformations: A single-center experience and a review of literature

    Directory of Open Access Journals (Sweden)

    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.

  9. 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

  10. Features of the psychological status in children with bronchopulmonary pathology

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    Gonchar M.A.

    2016-05-01

    Full Text Available The aim: study the features of the psychological status in children with bronchopulmonary pathology. Materials and methods. We compare the level of mental stress (assessed by questionnaire Beck Youth in children with acute bronchopulmonary diseases (1 group and the bronchial asthma (2 group. Results and discussion: 84.0±5.0 % children with bronchial asthma was characterized by psychosomatic disorientation and instability to stress, in the first group 28.5±3.8% patients with violation of the psychoemotional state was registreted. The average level of anxiety was observed in 40.0±3.0% patients of 2 groups, in relation to 14.3±2.7%% — in a 1 group. High level of anxiety among children with bronchial asthma in 32.0±4.5% were observed, as compared to 9.5±2.6% in a 1 group. A Self-Concept in children was determined as low, normal or high. A low Self-Concept was registreted in 64.0±5.5% children with asthma, in a 1 group of children with a low Self-Concept was not revealed; normal — accordingly in 28.0±2.5% and 71.4±3.1%; high — in 8.0±1.5% children with asthma, and in 28.6±4.2% patients of a 1 group. At 12±1.5% children of 2 group signs of depression were observed. Conclusions. The psycho-emotional state of children with asthma, characterized by increased levels of anxiety, and low self-esteem, in most cases, compared with a group of children with acute bronchopulmonary diseases.

  11. Developmental dysplasia of the hip

    Directory of Open Access Journals (Sweden)

    Shahryar Noordin

    2010-10-01

    Full Text Available Developmental dysplasia of the hip (DDH is a spectrum of anatomical abnormalities of the hip joint in which the femoral head has an abnormal relationship with the acetabulum. Most studies report an incidence of 1 to 34 cases per 1,000 live births and differences could be due to different diagnostic methods and timing of evaluation. Risk factors include first born status, female sex, positive family history, breech presentation and oligohydramnios. Clinical presentations of DDH depend on the age of the child. Newborns present with hip instability, infants have limited hip abduction on examination, and older children and adolescents present with limping, joint pain, and/or osteoarthritis. Repeated, careful examination of all infants from birth and throughout the first year of life until the child begins walking is important to prevent late cases. Provocative testing includes the Barlow and Ortolani maneuvers. Other signs, such as shorting of the femur with hips and knees flexed (Galeazzi sign, asymmetry of the thigh or gluteal folds, and discrepancy of leg lengths are potential clues. Treatment depends on age at presentation and outcomes are much better when the child is treated early, particularly during the first six months of life.

  12. Spectrum of Misdiagnosis of Allergic Bronchopulmonary Mycosis: Case Reports

    Directory of Open Access Journals (Sweden)

    Sandeep Rana

    2018-01-01

    Full Text Available Allergic bronchopulmonary mycosis (ABPM is most commonly seen in cystic fibrosis and bronchial asthma. Most of patients are misdiagnosed at initial outpatient department visit due to low suspicion, lack of awareness of entity and similarity in clinical symptoms and radiological findings with other common pulmonary diseases. Pulmonary tuberculosis is the most common entity diagnosed, and anti-tubercular treatment is the most commonly abused chemotherapy. Careful history taking, astute initial examination and awareness may help in curbing wrong diagnosis and treatment. This article present two cases of ABPM who were initially misdiagnosed as pulmonary tuberculosis and carcinoma lung, respectively.

  13. 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.

  14. A complex communicating bronchopulmonary foregut malformation: diagnostic imaging and pathogenesis

    International Nuclear Information System (INIS)

    Sumner, T.E.; Auringer, S.T.; Cox, T.D.

    1997-01-01

    We report a newborn with an esophageal lung, a rare type of communicating bronchopulmonary foregut malformation (CBPFM). Associated findings included esophageal atresia, tracheoesophageal fistula (TEF) to the distal esophagus, duodenal stenosis with annular pancreas, imperforate anus, vertebral anomalies and ambiguous genitalia. Radiologic evaluation included chest radiographs, esophagrams, chest ultrasound and chest CT. After colostomy and surgical repair of duodenal stenosis and TEF, a right thoracotomy was performed to treat an esophageal lung. Radiologic features of this unusual variant of CBPFM are presented. Accurate preoperative imaging diagnosis is essential for planning surgical treatment of an esophageal lung. (orig.). With 1 fig

  15. Anti-IgE therapy for allergic bronchopulmonary aspergillosis.

    Science.gov (United States)

    Homma, Tetsuya; Kurokawa, Masatsugu; Matsukura, Satoshi; Yamaguchi, Munehiro; Adachi, Mitsuru

    2016-06-01

    Allergic bronchopulmonary aspergillosis (ABPA) is a severe type of asthma. Some cases are resistant to treatment, even with regular use of antiasthmatic drugs and antifungal agents. The diagnosis of ABPA was made in a 40-year-old patient with ABPA according to the Rosenberg-Patterson criteria. Symptoms were not controlled despite regular use of antiasthmatic drugs, daily systemic steroids, and antifungal agents. Omalizumab, administered in an attempt to stabilize these uncontrolled symptoms, was effective with no adverse events. Our experience suggests omalizumab is a potential candidate drug for controlling steroid-dependent ABPA. Copyright © 2013. Published by Elsevier B.V.

  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

    International Nuclear Information System (INIS)

    Vignolo Puglia, W.; Freire Colla, D.; Rivara Urrutia, D.; Lujambio Grene, M.; Arbiza Bruno, T.; Oliveira, G.; Cobas Rodriguez, J.

    1997-01-01

    The arrhythmogenic right ventricular dysplasia is a condition predominantly well defined with arrhythmic events. We analyze three cases diagnosed by the group. These cases were presented as ventricular tachycardia with a morphology of left bundle branch block, presenting one of them aborted sudden death in evolution. The baseline electrocardiogram and signal averaging were abnormal in two of the three cases, like the echocardiogram. The electrophysiological study was able to induce in the three patients with sustained monomorphic ventricular tachycardia morphology of left bundle branch block. The definitive diagnosis was made by right ventriculography in two cases and magnetic resonance imaging in the other. Treatment included antiarrhythmic drugs in the three cases and the placement of an automatic defibrillator which survived a sudden death (Author)

  18. Genetics Home Reference: cranioectodermal dysplasia

    Science.gov (United States)

    ... function of one of the IFT-A subunits. Shortage or abnormal function of a single component of ... of bone, ectodermal tissues, and other tissues and organs, leading to the features of cranioectodermal dysplasia . About ...

  19. Ectodermal dysplasia with true anodontia.

    Science.gov (United States)

    Bala, Madhu; Pathak, Anuradha

    2011-05-01

    The hereditary condition known as ectodermal dysplasia is characterized by the absence or defect of two or more ectodermally derived structures. The most commonly observed forms of ectodermal dysplasia are the hidrotic and hypohidrotic types; discrimination is based on the absence or presence of sweat glands. A case of 8-year-old male child with hypohidrotic ectodermal dysplasia with complete anodontia of primary as well as secondary dentitions is presented. The child had a short stature, low intelligent quotient (I.Q.,), and was underweight. The patient experienced episodes of high fever, was intolerant to heat, and did not sweat. He exhibited smooth and dry skin, sparse light-colored eyebrows. Dental clinicians can be the first to diagnose ectodermal dysplasia due to the absence of teeth.

  20. Genetics Home Reference: Kniest dysplasia

    Science.gov (United States)

    ... dysplasia is one of a spectrum of skeletal disorders caused by mutations in the COL2A1 gene. This gene provides instructions for making a protein that forms type II collagen. This type of collagen is found mostly in ...

  1. Cervical Dysplasia: Is It Cancer?

    Science.gov (United States)

    Cervical dysplasia: Is it cancer? I had a Pap test recently, and my doctor said the results showed ... the appearance of the abnormal cells. On the Pap test report, this will be reported as a low- ...

  2. Genetics Home Reference: Greenberg dysplasia

    Science.gov (United States)

    ... navigation Home Page Search Home Health Conditions Genes Chromosomes & mtDNA Resources Help Me Understand Genetics Share: Email ... K. Mutations causing Greenberg dysplasia but not Pelger anomaly uncouple enzymatic from structural functions of a nuclear ...

  3. Genetics Home Reference: osteoglophonic dysplasia

    Science.gov (United States)

    ... DM, Econs MJ. Mutations that cause osteoglophonic dysplasia define novel roles for FGFR1 in bone elongation. Am ... for Links Data Files & API Site Map Subscribe Customer Support USA.gov Copyright Privacy Accessibility FOIA Viewers & ...

  4. Guide to Understanding Frontonasal Dysplasia

    Science.gov (United States)

    ... although most people with FND are of normal intelligence. Heart Rare cases of frontonasal dysplasia may be ... prognosis m any people with FND have normal intelligence and can expect a normal lifespan. how can ...

  5. Genetics Home Reference: geleophysic dysplasia

    Science.gov (United States)

    ... name of this condition, which comes from the Greek words for happy ("gelios") and nature ("physis"), is ... Yeung S, Dickinson DF, Karbani G, Crow YJ. Natural history of cardiac involvement in geleophysic dysplasia. Am J ...

  6. Genetics Home Reference: thanatophoric dysplasia

    Science.gov (United States)

    ... called a cloverleaf skull . The term thanatophoric is Greek for "death bearing." Infants with thanatophoric dysplasia are ... FGFR3 gene and occur in people with no history of the disorder in their family. No affected ...

  7. Genetics Home Reference: spondyloperipheral dysplasia

    Science.gov (United States)

    ... toes (brachydactyly), with the exception of the first (big) toes. Other skeletal abnormalities associated with spondyloperipheral dysplasia include short stature, shortened long bones of the arms and legs, exaggerated curvature of the lower back ( lordosis ), and ...

  8. Fibrous dysplasia of maxillary sinus

    OpenAIRE

    Tinoco, Paulo; Pereira, José Carlos Oliveira; Lourenço Filho, Rodolfo Caldas; Silva, Fabrício Boechat do Carmo; Ruela, Karol Pereira

    2009-01-01

    Introduction: The Fibrous Dysplasia is a benign bone disease, of slow growth and unknown etiology. The involvement of the craniofacial skeleton is not uncommon and, generally, produces facial asymmetries. Case Report: In this article we report the case of a patient with fibrous dysplasia occupying the entire left maxillary sinus with orbitary extension confirmed in the anatomopathological exam. Final Comments: The surgical treatment remains as the main therapeutic approach and the postoperati...

  9. [Cochleovestibular dysplasia: a case report].

    Science.gov (United States)

    Darbi, A; Cenoui, F; Atmane, A; Amil, T; Hanine, A

    2010-04-01

    Cochleovestibular dysplasia or Mondini malformation is exceptional. Cochlear dysplasia is due to early cessation of the development of the inner ear during embryonic life. We report on the case of an infant who presented with perception deafness and repeated meningitis. CT allowed the diagnosis of Mondini malformation. We underline the importance of high-resolution CT in the diagnosis of Mondini malformation. Copyright 2010 Elsevier Masson SAS. All rights reserved.

  10. Immunohistochemical Expression of MCM-2 in Oral Epithelial Dysplasias.

    Science.gov (United States)

    Zakaria, Samar H; Farag, Heba A; Khater, Dina S

    2016-03-17

    Oral cancer is one of the most frequent cancers in the world. It arises from epithelial dysplasia. Hence, identifying these lesions in an early stage could prevent their malignant transformation. The aim of the present work was to assess the cell proliferative activity of minichromosome maintenance protein (MCM-2) in oral epithelial dysplastic lesions and to correlate the results with different grades of epithelial dysplasia in an attempt to use MCM-2 in the early detection of malignancy. MCM-2 expression was determined by the nuclear count in a total of 30 oral epithelial dysplastic specimens roughly classified into 10 cases of mild, moderate, and severe dysplasia. Five cases of early invasive squamous-cell carcinomas and 5 cases of epithelial hyperplasia were also included. The MCM-2 immunostaining was found to increase gradually from mild to moderate to severe dysplasia and reached its maximum value in early invasive squamous cell carcinoma. MCM-2 is of prognostic value in cases of oral dysplasia that have a tendency to undergo malignant transformation.

  11. Perfusion lung scintigraphy in primary broncho-pulmonary cancer

    International Nuclear Information System (INIS)

    Lapergue, Paul.

    1976-01-01

    Research on primary broncho-pulmonary cancer has called extensively on scintigraphy and it seemed interesting to weigh up the pros and cons of the technique in this particular case. Our intention is to sum up objectively from recent documents the applications of scintigraphy in the study of primary broncho-pulmonary cancers and to attempt, on the basis of the results compiled, to show what benefits have been gained from this technique and to find out whether it has any pre-surgical value and of what kind. The technique was invariable; the tracer consists of human albumin macro-aggregates labelled with technetium 99m which by its short half-life and slight penetration enables four exposures to be taken during the same examination (front, back, right and left profiles), thus reducing the risks of mistaken diagnoses due to the use of a single incidence. Similarly the detection method was always the scintillation camera which explores the whole organ at once, considerably shortening the examination time. Lung scintigraphy by perfusion of labelled human albumin macro-aggregates offers the great advantage of being simple to use and harmless. It is easy to understand the important place it has taken in the range of methods available for lung circulation exploration [fr

  12. 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.

  13. Schimke immunoosseous dysplasia: Defining skeletal features

    NARCIS (Netherlands)

    K.B. Hunter (Kshamta); T. Lücke (Thomas); J. Spranger (Jürgen); S.F. Smithson (Sarah); H. Alpay (Harika); J.-L. André (Jean-Luc); Y. Asakura (Yumi); R. Bogdanovic (Radovan); D. Bonneau (Dominique); R. Cairns (Robyn); K. Cransberg (Karlien); S. Fründ (Stefan); H. Fryssira (Helen); D. Goodman (David); K. Helmke (Knut); B. Hinkelmann (Barbara); G. Lama (Guiliana); P. Lamfers (Petra); C. Loirat (Chantal); S. Majore (Silvia); C. Mayfield (Christy); B.F. Pontz (Betram); C. Rusu (Christina); J.M. Saraiva (Jorge); B. Schmidt (Beate); L. Schoemaker (Lawrence); S. Sigaudy (Sabine); N. Stajic (Natasa); D. Taha (Doris); C.F. Boerkoel (Cornelius)

    2010-01-01

    textabstractSchimke immunoosseous dysplasia (SIOD) is an autosomal recessive multisystem disorder characterized by prominent spondyloepiphyseal dysplasia, T cell deficiency, and focal segmental glomerulosclerosis. Biallelic mutations in swi/snf-related, matrix-associated, actin-dependent regulator

  14. Hemihypertrophy, renal dysplasia and benign nephromegaly.

    Science.gov (United States)

    Memon, Mohammad Amin; Mohanty, Suravi; Das, Kanishka; Garg, Isha; D'Cruz, Ashley Lucien Joseph

    2005-06-01

    Hemihypertrophy is associated with malignant visceral abdominal neoplasms in childhood. Benign nephromegaly and nephroblastomas are both known to occur with hemihypertrophy; however, association with renal dysplasia has not previously reported. We describe an infant presenting with recurrent haematuria who had segmental hemihypertrophy, ipsilateral renal dysplasia and contralateral benign nephromegaly. Although debated, renal dysplasia may predispose to and predate malignant change. Rational management and optimal surveillance of renal dysplasia and benign nephromegaly in hemihypertrophy is discussed.

  15. Allergic bronchopulmonary aspergillosis in an adult with Kartagener syndrome.

    Science.gov (United States)

    Sehgal, Inderpaul Singh; Dhooria, Sahajal; Bal, Amanjit; Agarwal, Ritesh

    2015-08-06

    Allergic bronchopulmonary aspergillosis (ABPA) is a pulmonary disorder resulting from immune responses directed against inhaled Aspergillus fumigatus antigens. It manifests with poorly controlled asthma, fleeting pulmonary opacities and structural lung damage in the form of bronchiectasis. Initially defined in individuals suffering from bronchial asthma and cystic fibrosis, it has also been described in patients with other structural lung disorders such as chronic obstructive pulmonary disease, pulmonary tuberculosis, idiopathic bronchiectasis and others. Kartagener syndrome is a manifestation of primary ciliary dyskinesia characterised by the presence of dextrocardia, bronchiectasis and chronic sinusitis. We report a case of ABPA in an adult suffering from Kartagener syndrome. We also performed a systematic review of the literature on the association between Kartagener syndrome and ABPA. 2015 BMJ Publishing Group Ltd.

  16. Role of inhaled amphotericin in allergic bronchopulmonary aspergillosis

    Directory of Open Access Journals (Sweden)

    I S Sehgal

    2014-01-01

    Full Text Available Allergic bronchopulmonary aspergillosis (ABPA is an immunological pulmonary disorder caused by immune reactions mounted against the ubiquitous fungus Aspergillus fumigatus. The disease clinically manifests with poorly controlled asthma, hemoptysis, systemic manifestations like fever, anorexia and weight loss, fleeting pulmonary opacities and bronchiectasis. The natural course of the disease is characterized by repeated episodes of exacerbations. Almost 30-40% of the patients require prolonged therapy, which currently consists of corticosteroids and anti-fungal azoles; both these agents have significant adverse reactions. Amphotericin B administered via the inhaled route can achieve a high concentration in the small airways with minimal systemic side-effects. Nebulized amphotericin B has been used in the management of invasive pulmonary aspergillosis. The aim of this review is to study the utility of inhaled amphotericin in ABPA.

  17. The other hip in unilateral hip dysplasia

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Rømer, Lone; Søballe, Kjeld

    2006-01-01

    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...... "gold" standard]). See the Guidelines for Authors for a complete description of levels of evidence....

  18. Immediate Overlay Removable Partial Dentures for a Patient with Ectodermal Dysplasia: A Clinical Report

    Directory of Open Access Journals (Sweden)

    Ramin Negahdari

    2016-01-01

    Full Text Available Ectodermal dysplasia has several characteristic signs and symptoms, including anadontia or hypodontia, conical and hypoplastic teeth, atrophic alveolar ridges, protuberant lips, and hypotrichosis. Definitive treatment options for these patients are several including fixed, removable or implant-supported prostheses. Economic limitations or other priorities can prevent patients from choosing the most desirable treatment. This clinical report describes the prosthetic rehabilitation of a patient with ectodermal dysplasia. The treatment developed an overlay immediate removable partial denture fabricated to reconstruct acceptable occlusal vertical dimension, function and esthetics. A 2-year period of follow up showed the minor chipping of composite layer on the metal substructure.   Keywords: Ectodermal dysplasia, mouth rehabilitation

  19. Mondini dysplasia with recurrent bacterial meningitis caused by three different pathogens.

    Science.gov (United States)

    Shikano, Hiroaki; Ohnishi, Hidenori; Fukutomi, Hisashi; Ito, Kimiko; Morimoto, Masahiro; Teramoto, Takahide; Aoki, Mitsuhiro; Nishihori, Takezumi; Akeda, Yukihiro; Oishi, Kazunori; Fukao, Toshiyuki

    2015-12-01

    Mondini dysplasia is rare, but has an important association with recurrent bacterial meningitis. We herein describe the case of a 3-year-old girl with unilateral sensorineural hearing loss who presented with three independent episodes of bacterial meningitis within 8 months. Temporal bone computed tomography indicated the characteristic features of Mondini dysplasia in the right inner ear. This was treated by surgical closure of the inner ear defect via oval window and additional vaccination was administered. Appropriate vaccination might prevent the recurrent bacterial meningitis associated with Mondini dysplasia. © 2015 Japan Pediatric Society.

  20. Therapeutic bronchoalveolar lavage with conventional treatment in allergic bronchopulmonary aspergillosis.

    Science.gov (United States)

    Khalil, Kanwal Fatima

    2015-05-01

    To establish the role of therapeutic bronchoalveolar lavage in addition to conventional treatment among two groups, with allergic bronchopulmonary aspergillosis, in terms of regression in serum IgE levels and clinical recurrence at 3 and 6 months of follow-up. Aquasi-experimental study. Department of Pulmonology, Fauji Foundation Hospital, Rawalpindi, from July 2010 to December 2013. The study was carried out on 132 patients who fulfilled the Greenberger and Patterson criteria and underwent a chest X-ray, an HRCT chest and classified radiologically as with Central Bronchiectasis (CB), High Attenuation Mucus (HAM) or Other Radiological Features (ORF). Baseline serum IgE levels were noted. All patients were given treatment including prednisolone and antifungal agent itraconazole for 4 months. Patients with ORF on HRCTchest and just received the medical treatment were labeled as conventional group. Those patients who had CB or HAM radiological features also underwent bronchoscopy with therapeutic Bronchoalveolar Lavage (BAL), labeled as BALgroup. Clinical recurrence and serum IgE levels were noted at 3 and 6 months. Values were compared using chi-square and Mann-Whitney tests respectively. Around 78 (59.1%) of patients underwent bronchoscopy with therapeutic bronchoalveolar lavage to remove the mucus plugs. The mean serum IgE levels at baseline were 3312.04 ± 2526.217 and 3486.15 ± 2528.324 IU/ml in the BAL and conventional groups respectively. There was a statistically significant reduction in the mean serum IgE levels at 3 (p bronchoalveolar lavage may be a useful adjunct to treatment in patients with allergic bronchopulmonary aspergillosis, serum in terms of IgE level reduction.

  1. Allergic bronchopulmonary aspergillosis treated successfully for one year with omalizumab

    Directory of Open Access Journals (Sweden)

    Collins J

    2012-11-01

    Full Text Available Jennifer Collins,1 Gabriele deVos,2 Golda Hudes,2 David Rosenstreich21New York Eye and Ear Infirmary, New York, NY, 2Albert Einstein College of Medicine/Montefiore Medical Center, Bronx, NY, USABackground: Current therapy for allergic bronchopulmonary aspergillosis (ABPA uses oral corticosteroids, exposing patients to the adverse effects of these agents. There are reports of the steroid-sparing effect of anti-IgE therapy with omalizumab for ABPA in patients with cystic fibrosis (CF, but there is little information on its efficacy against ABPA in patients with bronchial asthma without CF.Objective: To examine the effects of omalizumab, measured by asthma control, blood eosinophilia, total serum immunoglobulin E (IgE, oral corticosteroid requirements, and forced expiratory volume spirometry in patients with ABPA and bronchial asthma.Methods: A retrospective review of charts from 2004–2006 of patients treated with omalizumab at an academic allergy and immunology practice in the Bronx, New York were examined for systemic steroid and rescue inhaler usage, serum immunoglobulin E levels, blood eosinophil counts, and asthma symptoms, as measured by the Asthma Control Test (ACT.Results: A total of 21 charts were screened for the diagnosis of ABPA and bronchial asthma. Four patients with ABPA were identified; two of these patients were male. The median monthly systemic corticosteroid use at 6 months and 12 months decreased from baseline usage. Total serum IgE decreased in all patients at 12 months of therapy. Pre-bronchodilator forced expiratory vital capacity at one second (FEV1 was variable at 1 year of treatment. There was an improvement in Asthma Control Test (ACT symptom scores for both daytime and nighttime symptoms.Conclusions: Treatment with omalizumab creates a steroid-sparing effect, reduces systemic inflammatory markers, and results in improvement in ACT scores in patients with ABPA.Keywords: allergic bronchopulmonary aspergillosis

  2. Diagnosis and treatment of communicating bronchopulmonary foregut malformation

    Science.gov (United States)

    Ren, Hongxia; Duan, Liqiong; Zhao, Baohong; Wu, Xiaoxia; Zhang, Hongyi; Liu, Caixia

    2017-01-01

    Abstract Rationale: Communicating bronchopulmonary foregut malformation (CBPFM) is a rare congenital malformation involving both the digestive and respiratory systems. To our best knowledge, most cases of CBPFM reported in the literature were in infancy or adulthood and CBPFM in infantile is even rarer with a high case-fatality rate partly due to misdiagnosis. Patient concerns: We presented 2 cases of neonatal CBPFM. A 11-hour male newborn was admitted because of moaning for 7 hours, and a 1-day male newborn was referred to us with profuse foams, choking on breast-milk feeding and facial cyanosis. Diagnoses: With the assistance of upper gastrointestinal tract imaging and contrast-enhanced chest computed tomography (CT), the diagnosis was established according to the most recent diagnostic criteria. Interventions: The case one recieved a lower left pneumonectomy and surgical repair of esophageal fistula. The case two was performed with a surgical repair of esophageal atresia and esophageal tracheal fistula firstly, and then also received a repair of communicating bronchopulmonary foregut malformation two weeks after the first operation. Outcomes: The case one was cured and discharged 2 weeks after admission. Unfortunately the case two died from respiratory failure. Lessons: Pediatric surgeons should therefore be aware that type I CBPMF is rare and preoperative diagnosis is usually difficult. Maldiagnosis is uncommon because clinicians often focus their attention on esophageal atresia and neglect pulmonary abnormalities. Other than upper gastrointestinal tract radiography and CT scan, bronchoscopy should be considered in pediatric patients with esophageal atresia complicated with pulmonary abnormalities, knowing that bronchoscopy may help confirm the diagnosis and select surgical strategies. PMID:28296740

  3. Ultrasound diagnosis of pulmonary hypertension in children with chronic bronchopulmonary diseases

    International Nuclear Information System (INIS)

    Kondrat'ev, V.O.

    2000-01-01

    Ultrasound criteria of diagnosis of pulmonary hypertension and study this complication frequency in children with chronic bronchopulmonary diseases was determined. As diagnostic criteria of pulmonary hypertension Doppler echocardiographic indices of circulation in the pulmonary arteries are suggested

  4. Fibrous dysplasia of maxillary sinus

    Directory of Open Access Journals (Sweden)

    Tinoco, Paulo

    2009-06-01

    Full Text Available Introduction: The Fibrous Dysplasia is a benign bone disease, of slow growth and unknown etiology. The involvement of the craniofacial skeleton is not uncommon and, generally, produces facial asymmetries. Case Report: In this article we report the case of a patient with fibrous dysplasia occupying the entire left maxillary sinus with orbitary extension confirmed in the anatomopathological exam. Final Comments: The surgical treatment remains as the main therapeutic approach and the postoperative follow-up is necessary due to this condition recurrent nature.

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

    Directory of Open Access Journals (Sweden)

    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.

  6. Trochleoplasty in major trochlear dysplasia: current concepts

    Directory of Open Access Journals (Sweden)

    Beaufils Philippe

    2012-02-01

    Full Text Available Abstract Trochleoplasty is the theoretical solution to persistent symptoms (pain and/or instability related to trochlear dysplasia where there is not only a trochlear flatness but also a trochlear prominence. The threshold of prominence indicating surgical intervention has as yet not been determined. A bump of 5 mm is generally accepted as the inferior limit. Given the interventional nature of this demanding procedure, it should be proposed in selected cases after considerable discussion with the patient. Trochleoplasty is indicated as a primary procedure for major trochlear dysplasia with a prominence > 5 mm. Stabilization is obtained in most of the cases with the risk of residual mild anterior knee pain. It is also indicated as a salvage procedure when a previous surgery failed. Despite the reputation of the procedure, the published results are encouraging in terms of prevention of re-dislocation, satisfaction index, and radiological outcomes. Post-operative stiffness is the main complication, which may require manipulation under anaesthesia or arthroscopic arthrolysis. There are few other complications reported and to date secondary necrosis of the trochlea has not been reported. Technically speaking, the deepening trochleoplasty is a difficult procedure without reliable landmarks. We propose a recession wedge trochleoplasty which is an easier procedure. It is never undertaken as an isolated procedure, but always in conjunction with other realignment procedures of the extensor apparatus according to the "a la carte" surgery concept.

  7. Outcome of surgical management of developmental dysplasia of hip in children between 18 and 24 months

    OpenAIRE

    Ramani, Narasimhan; Patil, Mithun S; Mahna, Madhur

    2014-01-01

    Background: Developmental dysplasia of hip (DDH) is a common condition presenting to a pediatric orthopedic surgeon. There is a consensus on the surgical treatment of children with ages ranged from 18 to 24 months where majority agree on open reduction and hip spica. Open reduction was done with an additional pelvic procedure wherever required to get better results and prevent residual acetabular dysplasia (RAD) and early osteoarthritis. Materials and Methods: 35 children with unilateral D...

  8. Early intervention leads to long-term developmental improvements in very preterm infants, especially infants with bronchopulmonary dysplasia

    NARCIS (Netherlands)

    van Hus, Jwp; Jeukens-Visser, M.; Koldewijn, K.; Holman, R.; Kok, J. H.; Nollet, F.; van Wassenaer-Leemhuis, A. G.

    2016-01-01

    Various early intervention programmes have been developed in response to the high rate of neurodevelopmental problems in very preterm infants. We investigated longitudinal effects of the Infant Behavioral Assessment and Intervention Program on cognitive and motor development of very preterm infants

  9. Common questions about developmental dysplasia of the hip.

    Science.gov (United States)

    Jackson, Jonathan C; Runge, Melissa M; Nye, Nathaniel S

    2014-12-15

    Developmental dysplasia of the hip is a common musculoskeletal condition in newborns. Infants with developmental dysplasia of the hip, whether treated or untreated, have a higher incidence of early-onset hip osteoarthritis in adulthood. Evidence to support universal screening by physical examination or ultrasonography is limited and often conflicting. The U.S. Preventive Services Task Force found insufficient evidence that screening for developmental dysplasia of the hip prevents adverse outcomes. Physical examination screening is recommended by the American Academy of Pediatrics and the Pediatric Orthopaedic Society of North America. These organizations recommend use of the Ortolani and Barlow maneuvers to screen infants up to three months of age. Several recent studies support starting assessment for limited hip abduction at eight weeks of age, which is the most sensitive test for developmental dysplasia of the hip from this age on. Infants with overtly dislocated or dislocatable hips should be referred to an orthopedist on a priority basis at the time of diagnosis. Infants with equivocal hip examination findings at birth can be reexamined in two weeks. If there is subluxation or dislocation at the follow-up examination, referral should be made at that time. If the examination findings are still equivocal, the infant can undergo ultrasonography of the hips or be reexamined every few weeks through the first six weeks of life. Although equivocal findings commonly resolve spontaneously, infants with persistent equivocal findings of developmental dysplasia of the hip longer than six weeks should be evaluated by an orthopedist. Treatment generally involves flexion-abduction splinting. The benefits of treatment are unclear, and there are risks to treatment, most notably an increased occurrence of avascular necrosis of the femoral head.

  10. Cranioectodermal Dysplasia : A Probable Ciliopathy

    NARCIS (Netherlands)

    Konstantinidou, Anastasia E.; Fryssira, Helen; Sifakis, Stavros; Karadimas, Charalampos; Kaminopetros, Petros; Agrogiannis, Georgios; Velonis, Stylianos; Nikkels, Peter G. J.; Patsouris, Efstratios

    2009-01-01

    Cranioectodermal dysplasia (CED), also known as Sensenbrenner syndrome, is a rare autosomal recessive genetic disorder characterized by typical craniofacial, skeletal and ectodermal defects, and tubulointerstitial nephritis leading to early end-stage renal failure. We report on a new familial case

  11. Therapeutic Bronchoalveolar Lavage with Conventional Treatment in Allergic Bronchopulmonary Aspergillosis

    International Nuclear Information System (INIS)

    Khalil, K. F.

    2015-01-01

    Objective: To establish the role of therapeutic bronchoalveolar lavage in addition to conventional treatment among two groups, with allergic bronchopulmonary aspergillosis, in terms of regression in serum IgE levels and clinical recurrence at 3 and 6 months of follow-up. Study Design: Aquasi-experimental study. Place and Duration of Study: Department of Pulmonology, Fauji Foundation Hospital, Rawalpindi, from July 2010 to December 2013. Methodology: The study was carried out on 132 patients who fulfilled the Greenberger and Patterson criteria and underwent a chest X-ray, an HRCT chest and classified radiologically as with Central Bronchiectasis (CB), High Attenuation Mucus (HAM) or Other Radiological Features (ORF). Baseline serum IgE levels were noted. All patients were given treatment including prednisolone and antifungal agent itraconazole for 4 months. Patients with ORF on HRCT chest and just received the medical treatment were labeled as conventional group. Those patients who had CB or HAM radiological features also underwent bronchoscopy with therapeutic Bronchoalveolar Lavage (BAL), labeled as BAL group. Clinical recurrence and serum IgE levels were noted at 3 and 6 months. Values were compared using chi-square and Mann-Whitney tests respectively. Results: Around 78 (59.1 percentage) of patients underwent bronchoscopy with therapeutic bronchoalveolar lavage to remove the mucus plugs. The mean serum IgE levels at baseline were 3312.04 ± 2526.217 and 3486.15 ± 2528.324 IU/ml in the BAL and conventional groups respectively. There was a statistically significant reduction in the mean serum IgE levels at 3 (p < 0.00) and 6 months (p < 0.001) of follow-up in BALas compared to conventional group. There was no significant difference in the clinical recurrence rate in both the groups (p=0.078 at 3 and 0.343 at 6 months respectively). Conclusion: Therapeutic bronchoalveolar lavage may be a useful adjunct to treatment in patients with allergic bronchopulmonary

  12. Prediction of exacerbation chronic bronchopulmonary diseases in children with influenza

    Directory of Open Access Journals (Sweden)

    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. 

  13. Recurrent Bacterial Meningitis in a Child with Mondini Dysplasia

    OpenAIRE

    Kepenekli-Kadayifci, Eda; Karaaslan, Ayşe; Atıcı, Serkan; Binnetoğlu, Adem; Sarı, Murat; Soysal, Ahmet; Altınkanat, Gülşen; Bakır, Mustafa

    2014-01-01

    Mondini dysplasia, also known as Mondini malformation, is a developmental abnormality of the inner and middle ears that can cause hearing loss, cerebrospinal fluid (CSF) leakage, and recurrent bacterial meningitis (RBM), which is defined as two or more episodes of meningitis separated by a period of convalescence and the complete resolution of all signs and symptoms. An accurate diagnosis of the underlying pathology is crucial to prevent further episodes from occurring. Herein, we present a...

  14. Inter-society consensus document on treatment and prevention of bronchiolitis in newborns and infants

    OpenAIRE

    Baraldi, Eugenio; Lanari, Marcello; Manzoni, Paolo; Rossi, Giovanni A; Vandini, Silvia; Rimini, Alessandro; Romagnoli, Costantino; Colonna, Pierluigi; Biondi, Andrea; Biban, Paolo; Chiamenti, Giampietro; Bernardini, Roberto; Picca, Marina; Cappa, Marco; Magazz?, Giuseppe

    2014-01-01

    Acute bronchiolitis is the leading cause of lower respiratory tract infection and hospitalization in children less than 1?year of age worldwide. It is usually a mild disease, but some children may develop severe symptoms, requiring hospital admission and ventilatory support in the ICU. Infants with pre-existing risk factors (prematurity, bronchopulmonary dysplasia, congenital heart diseases and immunodeficiency) may be predisposed to a severe form of the disease. Clinical diagnosis of bronchi...

  15. Allergic bronchopulmonary aspergillosis in patients with cystic fibrosis

    Directory of Open Access Journals (Sweden)

    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.

  16. Intralobar bronchopulmonary sequestration in the newborn - a congenital malformation

    International Nuclear Information System (INIS)

    Laurin, S.; Haegerstrand, I.

    1999-01-01

    Background. Intralobar sequestration (ILS) has been suggested to be an acquired lesion. However, we have observed several young infants who had ILS. Objectives. Since this fact seems to indicate a congenital origin, we reviewed our experience. Material and methods. A retrospective review of bronchopulmonary sequestration from the Departments of Radiology and Pathology in Lund between 1964 and 1997. Results. We identified seven infants or young children with a diagnosis of intralobar sequestration. In each patient, the ILS was present before recurrent infection developed. Five had chest X-rays as neonates, one at 3 months and one at 11 months of age. All but one showed an abnormality on their first chest X-ray, consistent with sequestration. Six of the ILS were verified at angiography; all seven were surgically removed. Two of the children with ILS also had congenital cystic adenomatoid malformation (CCAM). Three children had both ILS and scimitar syndrome. Conclusions. The fact that ILS was present in seven newborn and young infants indicates that this lesion is, at least in some patients, a congenital malformation. (orig.)

  17. Animal Models, Learning Lessons to Prevent and Treat Neonatal Chronic Lung Disease

    Science.gov (United States)

    Jobe, Alan H.

    2015-01-01

    Bronchopulmonary dysplasia (BPD) is a unique injury syndrome caused by prolonged injury and repair imposed on an immature and developing lung. The decreased septation and decreased microvascular development phenotype of BPD can be reproduced in newborn rodents with increased chronic oxygen exposure and in premature primates and sheep with oxygen and/or mechanical ventilation. The inflammation caused by oxidants, inflammatory agonists, and/or stretch injury from mechanical ventilation seems to promote the anatomic abnormalities. Multiple interventions targeted to specific inflammatory cells or pathways or targeted to decreasing ventilation-mediated injury can substantially prevent the anatomic changes associated with BPD in term rodents and in preterm sheep or primate models. Most of the anti-inflammatory therapies with benefit in animal models have not been tested clinically. None of the interventions that have been tested clinically are as effective as anticipated from the animal models. These inconsistencies in responses likely are explained by the antenatal differences in lung exposures of the developing animals relative to very preterm humans. The animals generally have normal lungs while the lungs of preterm infants are exposed variably to intrauterine inflammation, growth abnormalities, antenatal corticosteroids, and poorly understood effects from the causes of preterm delivery. The animal models have been essential for the definition of the mediators that can cause a BPD phenotype. These models will be necessary to develop and test future-targeted interventions to prevent and treat BPD. PMID:26301222

  18. Complex orthopaedic management of patients with skeletal dysplasias

    Directory of Open Access Journals (Sweden)

    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.

  19. Fibrous dysplasia in paranasal cavities.

    Science.gov (United States)

    Simovic, S; Klapan, I; Bumber, Z; Bura, M

    1996-01-01

    Presentation is made of 2 boys and a girl with fibrous dysplasia involving the frontal and ethmoidal sinuses and the maxilla. This tumor-like growth of the bone was not restricted to the sinus alone but expanded to the orbit, adjacent sinus, skull base and infratemporal or pterygoid fossa. In such cases, surgical therapy is the treatment of choice, with maximal preservation of healthy tissue and avoidance of major devastations that may result in undesired cosmetic defects. The etiology of the disease is still unknown. However, it should be emphasized that all 3 patients had suffered an injury to the adjacent bony structures at various time points preceding the disease manifestation. That is why we are inclined to believe that trauma might be a factor responsible for the onset of fibrous dysplasia.

  20. Craniofacial features of cleidocranial dysplasia

    Directory of Open Access Journals (Sweden)

    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

  1. 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.

  2. Bronchopulmonary hygiene physical therapy in bronchiectasis and chronic obstructive pulmonary disease: a systematic review.

    Science.gov (United States)

    Jones, A; Rowe, B H

    2000-01-01

    This study had two objectives: (1) to examine the effects of bronchopulmonary hygiene physical therapy on patients with chronic obstructive pulmonary disease and bronchiectasis; (2) to determine any differences between manual and mechanical techniques for bronchopulmonary hygiene physical therapy. The study design was a systematic review of the literature that used an exhaustive search for trials and review methods prescribed by the Cochrane Collaboration. Randomized controlled trials examined patient groups, interventions, and dependent variables. Patients included those with chronic obstructive pulmonary diseases (emphysema or chronic bronchitis) or bronchiectasis. Any of the following interventions or combinations thereof were included: manual interventions, such as postural drainage, chest percussion, vibration, chest shaking, directed coughing, or forced exhalation technique. Controls of the study were as follows: no intervention; placebo; coughing; and mechanical interventions, such as mechanical vibration. The search identified 99 potential trials; inclusion or exclusion analysis left 7, which examined a total of 126 patients. Mean score on trial quality was 1.4 (5 = greatest). Three separate trials (N = 51) found statistically significant effects for bronchopulmonary hygiene physical therapy on sputum production and radioaerosol clearance. No trials (N = 126) found statistically significant effects on pulmonary function variables or differences between manual and mechanical techniques. Considering the small sizes, low quality, and mixed results from the trials, the research on bronchopulmonary hygiene physical therapy is inconclusive. There is a need for adequately sized, high-quality, randomized controlled trials with uniform patient populations to examine the effects of bronchopulmonary hygiene physical therapy.

  3. Ectodermal dysplasia in identical twins

    OpenAIRE

    Puttaraju, Gurkar Haraswarupa; Visveswariah, Paranjyothi Magadi

    2013-01-01

    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 facia...

  4. Thyroid Dysplasia in Wistar Hannover GALAS Rats

    OpenAIRE

    Weber, Klaus; Ernst, Rainer; Fankhauser, Heinz; Hardisty, Jerry F.; Heider, Wolfram; Stevens, Karla

    2009-01-01

    Thyroid dysplasia was recognized in WistarHan GALAS rats and confirmed as a heritable congenital disorder. The gene or genes involved were not identified, but homozygous animals with thyroid dysplasia also exhibited stunted growth, had reduced pituitary gland growth hormone (GH) and were hypothyroid. Heterozygous animals exhibited thyroid dysplasia with normal thyroid hormonal homeostasis and no difference in the incidence of preneoplastic or neoplastic lesions in oncogenicity studies.

  5. Pelvic radiograph in skeletal dysplasias: An approach

    Directory of Open Access Journals (Sweden)

    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.

  6. Dysplasia in view of the cell cycle

    Directory of Open Access Journals (Sweden)

    RG Steinbeck

    2009-06-01

    Full Text Available Dysplasia is linked to altered tissue architecture. The lesion belongs into the diagnostic field of human pathology and is highly relevant for the clinical physician, because it breaks the criteria of hyperplasia and regeneration. Dysplasia is a precancerous disorder leading in all probability to malignant transformation if not treated. However, different descriptions do apply for dysplasia in different human tissues, and conventional pathology cannot arrive at unequivocal stringency. In contrast to the previous situation, now, dysplasia is defined by a unifying concept, which works upon cell cycle criteria. The decisive element for the proposed definition is unbalanced segregation of chromosomes and persistent genomic asymmetry through telophase, leading to aneuploid interphase nuclei. Progress of dysplasia can be estimated from the frequency of pathologic mitoses that directly measure cellular proliferation. In routine work, progress of dysplasia shall be quantified by frequency increase of aneuploidy in the increasing fraction of proliferating interphase nuclei. Thus, dysplasia is defined not only by aberrations from healthy histological architecture and normal cytological differentiation, but also by violations of the DNA standard from mitotic nuclei. The proposed classification of dysplasia measures the frequency of pathologic mitoses and the degree of genomic alterations in interphase nuclei. Both these criteria discriminate between low-grade and highgrade dysplasia and ascertain the malignant potential of a dysplastic lesion.

  7. Ischiovertebral dysplasia: a retrospective analysis of 30 consecutive cases pointing out the specifics and risks of the spine management.

    Science.gov (United States)

    Aurégan, Jean-Charles; Odent, Thierry; Coyle, Ryan M; Miladi, Lotfi; Wicart, Philippe; Dubousset, Jean; Le Merrer, Martine; Padovani, Jean-Paul; Glorion, Christophe

    2014-04-20

    A review of clinical publications, current knowledge, and recent developments regarding the etiology of ischiovertebral dysplasia was combined with a clinical review of the condition. To acquaint orthopedic spine surgeons with identification patterns of ischiovertebral dysplasia in order to provide them with guidelines about spine management and which complications to expect. Ischiovertebral dysplasia is a rare skeletal dysplasia that may appear in a sporadic fashion or be inherited with an autosomal dominant inheritance pattern. It is defined by the association of an ischiopubic ramus hypoplasia and a vertebral dysplasia. It leads to a specific spine deformity whose management and complications should be clarified. Thirty consecutive patients from 0 to 31 years of age with ischiovertebral dysplasia were included from 5 centers specialized in congenital spinal deformities. Frontal and sagittal Cobb angles before treatment, natural history of the curves, therapeutic options, and their complications were systematically analyzed. All the patients had a vertebral dysplasia and 28 of them developed a spinal deformity. This deformity was an extremely severe thoracic kyphoscoliosis in 25 cases. The other deformities were a thoracolumbar scoliosis in 1 case and a thoracolumbar kyphosis in 2 cases. The management of the thoracic kyphoscoliosis was always challenging and complications included death by respiratory failure (3 cases) and neurological impairment (9 cases). Recognizing the occurrence of ischioverterbral dysplasia is very important to allow for dedicated treatment. The authors advocate preoperative distraction and circumferential fusion to prevent progression of the curve and to avoid the potentially fatal sequelae associated with this disorder. 4.

  8. Recurrent Bacterial Meningitis in a Child with Mondini Dysplasia

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    Eda Kepenekli-Kadayifci

    2014-01-01

    Full Text Available Mondini dysplasia, also known as Mondini malformation, is a developmental abnormality of the inner and middle ears that can cause hearing loss, cerebrospinal fluid (CSF leakage, and recurrent bacterial meningitis (RBM, which is defined as two or more episodes of meningitis separated by a period of convalescence and the complete resolution of all signs and symptoms. An accurate diagnosis of the underlying pathology is crucial to prevent further episodes from occurring. Herein, we present a three-year-old boy with RBM and unilateral sensorineural hearing loss. During the evaluation to determine the etiology of the RBM, cystic malformation in the cochlea and vestibular dilatation consistent with Mondini dysplasia were detected via computerized tomography (CT of the temporal bone.

  9. Recurrent bacterial meningitis in a child with mondini dysplasia.

    Science.gov (United States)

    Kepenekli-Kadayifci, Eda; Karaaslan, Ayşe; Atıcı, Serkan; Binnetoğlu, Adem; Sarı, Murat; Soysal, Ahmet; Altınkanat, Gülşen; Bakır, Mustafa

    2014-01-01

    Mondini dysplasia, also known as Mondini malformation, is a developmental abnormality of the inner and middle ears that can cause hearing loss, cerebrospinal fluid (CSF) leakage, and recurrent bacterial meningitis (RBM), which is defined as two or more episodes of meningitis separated by a period of convalescence and the complete resolution of all signs and symptoms. An accurate diagnosis of the underlying pathology is crucial to prevent further episodes from occurring. Herein, we present a three-year-old boy with RBM and unilateral sensorineural hearing loss. During the evaluation to determine the etiology of the RBM, cystic malformation in the cochlea and vestibular dilatation consistent with Mondini dysplasia were detected via computerized tomography (CT) of the temporal bone.

  10. Non-aspergillus allergic bronchopulmonary mycosis in a pediatric patient with cystic fibrosis.

    Science.gov (United States)

    Gondor, M; Michaels, M G; Finder, J D

    1998-12-01

    This article describes a child with cystic fibrosis (CF) and allergic bronchopulmonary mycosis caused by Tricosporon beigelii. An 11-year-old boy with CF failed to respond to conventional treatment for a pulmonary exacerbation. Bronchial washings contained copious budding yeast forms, subsequently identified as T beigelii. Total serum immunoglobulin E was elevated and precipitating antibodies to T beigelii were positive. Together these findings support the diagnosis of allergic bronchopulmonary mycosis. The patient improved with antifungal therapy and systemic glucocorticoid therapy. The pathologic potential of yeast in the airways of patients with CF is unclear. The diagnosis of non-Aspergillus allergic bronchopulmonary mycosis requires a high degree of suspicion and has potentially important implications for the management of patients with CF.

  11. Clinical characteristics of biopsy-proven allergic bronchopulmonary mycosis: variety in causative fungi and laboratory findings.

    Science.gov (United States)

    Ishiguro, Takashi; Takayanagi, Noboru; Kagiyama, Naho; Shimizu, Yoshihiko; Yanagisawa, Tsutomu; Sugita, Yutaka

    2014-01-01

    The diagnosis of allergic bronchopulmonary mycosis (ABPM) has traditionally relied widely on Rosenberg's criteria, which emphasize immunologic responses while overlooking the investigation of mucous plugs as a primary criterion. Therefore, the characteristics of biopsy-proven ABPM require further elucidation. The aim of this study was to analyze the clinical characteristics of biopsy-proven ABPM and address whether full compliance with clinical criteria, such as the presence of asthma, and certain laboratory findings is necessary to establish a diagnosis of ABPM. We retrospectively analyzed 17 patients with biopsy-proven ABPM focusing on causative fungi and laboratory findings. Causative fungi included Aspergillus sp. in seven patients, Schizophyllum commune in four patients, Penicillium sp. in two patients and unknown in five patients. Bronchial asthma was observed in 10 patients, eosinophilia was observed in 10 patients and an increased serum immunoglobulin (Ig) E level was observed in 14 of the 17 patients. IgG for Aspergillus sp. was positive in six of the seven patients with ABPM due to Aspergillus and turned positive in the remaining patient during follow-up. Technological limitations prevented the measurement of specific IgE for S. commune and IgG for S. commune and Penicillium sp. in most patients. Computed tomography revealed central bronchiectasis, pulmonary infiltration and mucous plugs in all patients. Causative fungi other than Aspergillus sp. are not uncommon, and immunological tests for other fungi should be popularized. Asthma and characteristic laboratory findings, such as peripheral blood eosinophilia, increased serum IgE and precipitating antibodies, may not always be required to diagnose ABPM. The importance of typical pathologic findings of mucous plugs for diagnosing ABPM requires reevaluation. Further studies are needed to establish more elaborate diagnostic criteria for ABPM.

  12. Arginase inhibition prevents bleomycin-induced pulmonary hypertension, vascular remodeling, and collagen deposition in neonatal rat lungs.

    Science.gov (United States)

    Grasemann, Hartmut; Dhaliwal, Rupinder; Ivanovska, Julijana; Kantores, Crystal; McNamara, Patrick J; Scott, Jeremy A; Belik, Jaques; Jankov, Robert P

    2015-03-15

    Arginase is an enzyme that limits substrate L-arginine bioavailability for the production of nitric oxide by the nitric oxide synthases and produces L-ornithine, which is a precursor for collagen formation and tissue remodeling. We studied the pulmonary vascular effects of arginase inhibition in an established model of repeated systemic bleomycin sulfate administration in neonatal rats that results in pulmonary hypertension and lung injury mimicking the characteristics typical of bronchopulmonary dysplasia. We report that arginase expression is increased in the lungs of bleomycin-exposed neonatal rats and that treatment with the arginase inhibitor amino-2-borono-6-hexanoic acid prevented the bleomycin-induced development of pulmonary hypertension and deposition of collagen. Arginase inhibition resulted in increased L-arginine and L-arginine bioavailability and increased pulmonary nitric oxide production. Arginase inhibition also normalized the expression of inducible nitric oxide synthase, and reduced bleomycin-induced nitrative stress while having no effect on bleomycin-induced inflammation. Our data suggest that arginase is a promising target for therapeutic interventions in neonates aimed at preventing lung vascular remodeling and pulmonary hypertension. Copyright © 2015 the American Physiological Society.

  13. Renal dysplasia and MRI: a clinician's perspective

    International Nuclear Information System (INIS)

    Greenbaum, Larry A.

    2008-01-01

    Renal dysplasia is a common abnormality in children. The role of MRI in evaluating children with renal dysplasia is evolving. More information is clearly necessary before MRI replaces conventional imaging modalities. In order to appropriately use MRI, the radiologist must have an understanding of the clinical questions that are important in the management of children with renal dysplasia. This review provides background information on renal dysplasia for the pediatric radiologist. The focus is on unilateral disease, especially multicystic dysplastic kidneys, and bilateral dysplasia, which is the most common cause of kidney failure in children. The emphasis is on the important clinical issues, and the potential of MRI as a methodology for providing clinically useful information not otherwise available from other imaging modalities. (orig.)

  14. Cleidocranial dysplasia: A family report

    Directory of Open Access Journals (Sweden)

    Chelvan H

    2009-01-01

    Full Text Available A 10-year-old girl presented with a chief complaint of many unerupted teeth. Complete clinical and radiological examination of this patient confirmed the diagnosis of cleidocranial dysplasia (CCD. Her father also presented similar features with a lesser clinical severity. CCD is an autosomal-dominant heritable skeletal disease caused by heterozygous mutations in the osteoblast-specific transcription factor RUNX2 gene. Failure of tooth eruption is probably mainly due to this mutated gene in CCD patients. Interdisciplinary treatment approach is obligatory for rehabilitation of these patients. In confirmed cases, genetic counseling for family planning should certainly be advised.

  15. MRI of focal cortical dysplasia

    International Nuclear Information System (INIS)

    Lee, B.C.P.; Hatfield, G.A.; Bourgeois, B.; Park, T.S.

    1998-01-01

    We studied nine cases of focal cortical dysplasia (FCD) by MRI, with surface-rendered 3D reconstructions. One case was also examined using single-voxel proton MR spectroscopy (MRS). The histological features were reviewed and correlated with the MRI findings. The gyri affected by FCD were enlarged and the signal of the cortex was slightly increased on T1-weighted images. The gray-white junction was indistinct. Signal from the subcortical white matter was decreased on T1- and increased on T2-weighted images in most cases. Contrast enhancement was seen in two cases. Proton MRS showed a spectrum identical to that of normal brain. (orig.) (orig.)

  16. Mondini dysplasia with recurrent meningitis.

    Science.gov (United States)

    Lu, M Y; Lee, P I; Lee, C Y; Hsu, C J

    1996-01-01

    Mondini dysplasia is a congenital malformation of the inner ear, commonly associated with hearing impairment, cerebrospinal fluid otorrhea/rhinorrhea and recurrent meningitis. Two such cases are described, with hearing impairment, cerebrospinal fluid rhinorrhea, and several episodes of meningitis. Diagnosis was confirmed by high-resolution computed tomography. After surgical correction of the malformation, there was no recurrent episode of meningitis at subsequent follow-up. To avoid the suffering and the sequelae of recurrent meningitis, an early diagnosis and prompt surgical intervention are crucial for such patients.

  17. Cochlear implantation in Mondini dysplasia.

    Science.gov (United States)

    Daneshi, Ahmad; Hassanzadeh, Saeid; Abasalipour, Parvaneh; Emamdjomeh, Hessamaddin; Farhadi, Mohammad

    2003-01-01

    The use of cochlear implantation to treat patients with inner ear malformations such as Mondini dysplasia has been increasingly successful. Until now, conventional hearing aids in these patients have not performed well. Consequently, the hearing problem for patients with this condition has been somewhat improved with the use of cochlear implants. Various results of cochlear implantation have been reported in these patients so far. This is a report of 5 patients with Mondini malformation who have undergone cochlear implant surgery. Copyright 2003 S. Karger AG, Basel

  18. Ectodermal dysplasia in identical twins

    Directory of Open Access Journals (Sweden)

    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.

  19. Prevention of bronchiolitis from the hospital to home: enviromental and pharmacological strategies

    Directory of Open Access Journals (Sweden)

    Andrea Dotta

    2015-10-01

    Full Text Available Bronchiolitis is one of the major cause of mortality and morbidity during infancy, with high hospitalization rate during epidemic season for high risk infants during the first year of life. There is no specific therapy with proven efficacy apart hydration and hypertonic saline. Mild forms can be treated in outpatient setting avoiding a useless hospitalization. Enviromental prevention is crucial in hospital setting, in outpatient ambulatory and at home: for this purpose hand hygiene, tobacco exposure, breastfeeding must be emphasized with families and caregivers. Pharmacological prophylaxis uses a humanized monoclonal antibody (palivizumab shown effective in reducing hospitalization rate in preterm infants < 35 weeks gestational age, infants with bronchopulmonary dysplasia and infants with congenital heart disease. During the last 2 years different recommandations and guidelines confirmed the importance of prophylaxis with palivizumab with some differences between Groups and Societies based on different healthcare systems. In any case it is important a good contact between hospital and all caregivers of these patients to reduce the viral exposure and increase the defense capability of high risk patients. Proceedings of the 11th International Workshop on Neonatology and Satellite Meetings · Cagliari (Italy · October 26th-31st, 2015 · From the womb to the adultGuest Editors: Vassilios Fanos (Cagliari, Italy, Michele Mussap (Genoa, Italy, Antonio Del Vecchio (Bari, Italy, Bo Sun (Shanghai, China, Dorret I. Boomsma (Amsterdam, the Netherlands, Gavino Faa (Cagliari, Italy, Antonio Giordano (Philadelphia, USA

  20. 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

  1. Omalizumab Treatment for Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis.

    Science.gov (United States)

    Emiralioglu, Nagehan; Dogru, Deniz; Tugcu, Gokcen Dilsa; Yalcin, Ebru; Kiper, Nural; Ozcelik, Ugur

    2016-03-01

    Allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF) is characterized by destructive changes in the airways. Long-term treatment with oral corticosteroids is often required for repeated exacerbations. Because elevated total IgE is a cardinal abnormality of ABPA, omalizumab has been used sporadically to decrease corticosteroid dose or totally replace corticosteroids. The aim of this report is to describe our experience with omalizumab treatment in patients with CF and ABPA. We conducted a review of 6 CF patients with ABPA receiving omalizumab. All patients were treated with oral prednisolone and itraconazole. Omalizumab was started if the patient was not responding to steroid treatment, which was determined according to serum IgE levels and/or clinical findings or depending on if there were side effects caused by steroid treatment. The mean age of patients at the beginning of omalizumab treatment was 16.1 years. One patient had a new diagnosis of ABPA; however, the others had the first to third exacerbation when treated with omalizumab. The mean duration of ABPA by the time that treatment with omalizumab started was 13 ± 12.4 months (range = 2-29 months). With omalizumab treatment, IgE levels were decreased in all patients, and Aspergillus-specific IgE levels were decreased in 4 patients; however, FEV1(% predicted) improved only in 2 patients who had mild disease. Corticosteroids were reduced in the first, second, and third months of omalizumab treatment in 2, 1, and 3 patients, respectively. In 2 patients, steroid treatment was stopped. None of the patients suffered from side effects of omalizumab. The mean duration of omalizumab treatment was 12.5 months (range = 6-18 months). This study showed steroid-sparing effect, decreasing IgE levels, and improvement in respiratory symptoms in 6 CF patients with omalizumab treatment. Although this is a small sample of the population, omalizumab may be an alternative therapy for ABPA in CF patients who fail

  2. Long-term results of PRRT in advanced bronchopulmonary carcinoid

    International Nuclear Information System (INIS)

    Mariniello, Annapaola; Bodei, Lisa; Baio, Silvia Melania; Gilardi, Laura; Colandrea, Marzia; Papi, Stefano; Grana, Chiara Maria; Tinelli, Carmine; Valmadre, Giuseppe; Fazio, Nicola; Galetta, Domenico; Paganelli, Giovanni

    2016-01-01

    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 ( 90 Y-DOTATOC vs. 177 Lu-DOTATATE vs. 90 Y-DOTATOC + 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 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 90 Y-DOTATOC + 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 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 the potential selection biases

  3. A case of allergic bronchopulmonary aspergillosis successfully treated with mepolizumab.

    Science.gov (United States)

    Terashima, Takeshi; Shinozaki, Taro; Iwami, Eri; Nakajima, Takahiro; Matsuzaki, Tatsu

    2018-03-27

    Allergic bronchopulmonary aspergillosis (ABPA) is an allergic pulmonary disease comprising a complex hypersensitivity reaction to Aspergillus fumigatus. Clinical features of ABPA are wheezing, mucoid impaction, and pulmonary infiltrates. Oral corticosteroids and anti-fungal agents are standard therapy for ABPA, but long-term use of systemic corticosteroids often causes serious side effects. A 64-year-old woman was diagnosed with ABPA based on a history of bronchial asthma (from 40 years of age), elevated total IgE, the presence of serum precipitating antibodies and elevated specific IgE antibody to A. fumigatus, and pulmonary infiltration. Bronchoscopy showed eosinophilic mucoid impaction. Systemic corticosteroid therapy was initiated, and her symptoms disappeared. Peripheral eosinophilia and pulmonary infiltration recurred five months after cessation of corticosteroid treatment. Systemic corticosteroids were re-initiated and itraconazole was added as an anti-fungal agent. The patient was free of corticosteroids, aside from treatment with a short course of systemic corticosteroids for asthma exacerbation, and clinically stable with itraconazole and asthma treatments for 3 years. In 2017, she experienced significant deterioration. Laboratory examination revealed marked eosinophilia (3017/μL) and a chest computed tomography (CT) scan demonstrated pulmonary infiltration in the left upper lobe and mucoid impaction in both lower lobes. The patient was treated with high-dose inhaled corticosteroid/long-acting beta-agonist, a long-acting muscarinic antagonist, a leukotriene receptor antagonist, and theophylline; spirometry revealed a forced expiratory volume in 1 s (FEV 1 ) of 1.01 L. An uncontrolled asthma state was indicated by an Asthma Control Test (ACT) score of 18. Mepolizumab, 100 mg every 4 weeks, was initiated for the treatment of severe bronchial asthma with ABPA exacerbation. Bronchial asthma symptoms dramatically improved, and ACT score increased to 24

  4. Immunohistochemical staining patterns of cytokeratins 13, 14, and 17 in oral epithelial dysplasia including orthokeratotic dysplasia.

    Science.gov (United States)

    Nobusawa, Aiko; Sano, Takaaki; Negishi, Akihide; Yokoo, Satoshi; Oyama, Tetsunari

    2014-01-01

    Diagnosis of the exact grade of oral epithelial dysplasia is difficult, and interobserver variations in grading are common. The aim of this study was to investigate the expression patterns of cytokeratins (CKs) in dysplastic oral epithelia, to identify useful double immunostaining diagnostic markers. Immunoexpression of CK13, CK14, CK17, and Ki-67 were investigated in 21 normal epithelial specimens and 146 epithelial dysplasia specimens. In epithelial dysplasia specimens, orthokeratotic dysplasia (OKD) was identified using CK10 immunostaining. Most mild dysplasia specimens were CK13+ and CK17-. In moderate dysplasia, CK13 expression tended to be lower and CK17 expression tended to be higher than in mild dysplasia. All carcinoma in situ (CIS) specimens were CK17+. In differentiated type CIS specimens, CK13 expression was weakly positive. Most epithelial dysplasia specimens were CK14+. There were no significant differences in the expression patterns of CKs between OKD and non-OKD specimens in any of the grades of dysplasia. These results indicate that CK14 expression can be used to detect early epithelial dysplasia, and that CK13 and CK17 expression are useful for detecting neoplastic changes. © 2014 The Authors. Pathology International © 2014 Japanese Society of Pathology and Wiley Publishing Asia Pty Ltd.

  5. A rare case of allergic bronchopulmonary mycosis caused by Alternaria alternata.

    NARCIS (Netherlands)

    Chowdhary, A.; Agarwal, K.; Randhawa, H.S.; Kathuria, S.; Gaur, S.N.; Najafzadeh, M.J.; Roy, P.; Arora, N.; Khanna, G.; Meis, J.F.G.M.

    2012-01-01

    A rare case of allergic bronchopulmonary mycosis (ABPM), caused by Alternaria alternata, is reported in an immunocompetent resident of Delhi. Her complaints included a generalized, urticarial skin rash and occasional pain in the right lower chest. Her differential count showed eosinophils, 22%;

  6. Congenital bronchopulmonary foregut malformation initially diagnosed as esophageal atresia type C: challenging diagnosis and treatment

    NARCIS (Netherlands)

    Boersma, Doeke; Koot, Bart G.; van der Griendt, Erik Jonas; van Rijn, Rick R.; van der Steeg, Alida F.

    2012-01-01

    Communicating bronchopulmonary foregut malformations are extremely rare congenital malformations, characterized by a communicating fistula between an isolated part of the respiratory system and the esophagus or the stomach. In this article, we present a case of esophageal atresia type C, later

  7. Multiple Epiphyseal Dysplasia (MED: A Rare Type of Skeletal Dysplasia

    Directory of Open Access Journals (Sweden)

    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 

  8. Dysplasia and Cancer in Inflammatory Bowel Disease.

    Science.gov (United States)

    Huang, Lyen C; Merchea, Amit

    2017-06-01

    Inflammatory bowel disease is associated with an increased risk of dysplasia and cancer. Improvements in medical management and endoscopic surveillance have reduced these risks. Patients can develop cancer even in the absence of dysplasia or with indefinite or low-grade dysplasia. Most guidelines recommend starting surveillance colonoscopy 6 to 10 years after initial diagnosis with interval surveillance afterward every 1 to 5 years depending on risk and/or individual characteristics. Most patients should undergo total proctocolectomy with end ileostomy or reconstruction with ileal pouch anal anastomosis because segmental and subtotal resections carry a higher risk of metachronous cancers. Copyright © 2017 Elsevier Inc. All rights reserved.

  9. Psychosocial predictors of parental participation in ultrasound screening for developmental dysplasia of the hip

    NARCIS (Netherlands)

    Witting, M.; Boere-Boonekamp, M.M.; Sakkers, R.J.B.; Fleuren, M.A.H.; IJzerman, M.J.

    2013-01-01

    Ultrasound screening for developmental dysplasia of the hip (DDH) is an innovation in preventive child health care in the Netherlands. Parental participation in the screening will be essential for the success of implementation of the screening. The aim of the current study was to investigate whether

  10. Paget's disease and fibrous dysplasia.

    Science.gov (United States)

    Hullar, Timothy E; Lustig, Lawrence R

    2003-08-01

    Paget's disease and fibrous dysplasia are benign disorders that can involve the temporal bone and skull base. They commonly lead to otolaryngologic symptoms such as impingement of cranial nerves or the orbit or blockage of the external auditory canal or paranasal sinuses, although they can often be a challenge to diagnose because of their insidious presentation. Their benign nature and common presentation within the difficult-to-access confines of the skull base should lead the clinician to exercise caution in their treatment, reserving surgical intervention for either diagnosis or the relief of symptoms. As a better understanding of the etiology of these conditions develops and new pharmacotherapeutic agents are tested, it is likely that physicians will be able to turn to medical rather than surgical techniques to treat these disorders.

  11. Posttraumatic Cranial Cystic Fibrous Dysplasia

    Directory of Open Access Journals (Sweden)

    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.

  12. Ectodermal Dysplasia Skin Fragility Syndrome

    Directory of Open Access Journals (Sweden)

    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.

  13. Camptomelic dysplasia: A case report

    Directory of Open Access Journals (Sweden)

    Koš Radmila

    2007-01-01

    Full Text Available Campomelic/camptomelic dysplasia is a very rare, severe osteochondrodysplasia characterised by severe skeletal and nonskeletal malformations and lethal outcome mainly in neonatal period. Characteristic abnormality by which the syndrome got its name is short, bowed long bones of lower extremities, most often of femur, manifested by short and bowed legs. Skin dimpling on tibial anterior side is another prominent characteristic of this syndrome. Severe cases are inherited by autosomal dominant trait, by mutation Sox9 gene on chromosome 17, with lethal outcome in the first days of life. Less severe forms of the disease are due to balanced translocation t (13;17 with life span up to the third decade of life. A majority of karyotypic males present as phenotypic females. We report a case of a female neonate, without consanguinity between parents, with characteristic signs of camptomelic dysplasia with short birth length of 46 cm, macrocephaly (head circumference 39 cm, dolichocephaly, hydrocephalus, short trunk and legs. Narrow rib cage, bowed lower extremities, short hand and foot phalanges, nail hypoplasia were noticed. Anterior fontanelle was enlarged, high forehead, face small and flat, hypertelorism, low nasal bridge, micrognathia, low set ears, cleft palate, were found. Characteristic skin dimpling on anterior side of tibia was present on both legs. Bone X-ray studies presented the following changes: anterior bowing of shortened femurs, hip dislocation, cervical vertebrae, scapulas, eleven pairs of slender ribs. Hip luxation. Karyotype was normal for a female, 46 XX. Respiratory insufficiency was present since birth, exacerbated, and led to lethal outcome in the second day of life, as described in the majority of these patients. .

  14. Canine hip dysplasia: A natural animal model for human developmental dysplasia of the hip.

    Science.gov (United States)

    Pascual-Garrido, Cecilia; Guilak, Farshid; Rai, M Farooq; Harris, Michael D; Lopez, Mandi J; Todhunter, Rory J; Clohisy, John C

    2017-12-11

    Developmental dysplasia of the hip (DDH) in humans is a common condition that is associated with hip pain, functional limitations, and secondary osteoarthritis (OA). Surgical treatment of DDH has improved in the last decade, allowing excellent outcomes at short- and mid-term follow-up. Still, the etiology, mechanobiology, and pathology underlying this disease are not well understood. A pre-clinical animal model of DDH could help advance the field with a deeper understanding of specific pathways that initiate hip joint degeneration secondary to abnormal biomechanics. An animal model would also facilitate different interventional treatments that could be tested in a rigorous and controlled environment. The dog model exhibits several important characteristics that make it valuable as a pre-clinical animal model for human DDH. Dogs are naturally prone to develop canine hip dysplasia (CHD), which is treated in a similar manner as in humans. Comparable to human DDH, CHD is considered a pre-OA disease; if left untreated it will progress to OA. However, progression to OA is significantly faster in dogs than humans, with progression to OA within 1-2 years of age, associated with their shorter life span compared to humans. Animal studies could potentially reveal the underlying biochemical pathway(s), which can inform refined treatment modalities and provide opportunities for new treatment and prevention targets. Herein, we review the similarities and differences between the two species and outline the argument supporting CHD as an appropriate pre-clinical model of human DDH. © 2017 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res. © 2017 Orthopaedic Research Society. Published by Wiley Periodicals, Inc.

  15. Anti-IgE therapy for allergic bronchopulmonary aspergillosis in people with cystic fibrosis.

    Science.gov (United States)

    Jat, Kana R; Walia, Dinesh K; Khairwa, Anju

    2018-03-18

    Cystic fibrosis is an autosomal recessive multisystem disorder with an approximate prevalence of 1 in 3500 live births. Allergic bronchopulmonary aspergillosis is a lung disease caused by aspergillus-induced hypersensitivity with a prevalence of 2% to 15% in people with cystic fibrosis. The mainstay of treatment includes corticosteroids and itraconazole. The treatment with corticosteroids for prolonged periods of time, or repeatedly for exacerbations of allergic bronchopulmonary aspergillosis, may lead to many adverse effects. The monoclonal anti-IgE antibody, omalizumab, has improved asthma control in severely allergic asthmatics. The drug is given as a subcutaneous injection every two to four weeks. Since allergic bronchopulmonary aspergillosis is also a condition resulting from hypersensitivity to specific allergens, as in asthma, it may be a candidate for therapy using anti-IgE antibodies. Therefore, anti-IgE therapy, using agents like omalizumab, may be a potential therapy for allergic bronchopulmonary aspergillosis in people with cystic fibrosis. This is an updated version of the review. To evaluate the efficacy and adverse effects of anti-IgE therapy for allergic bronchopulmonary aspergillosis in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched the reference lists of relevant articles and reviews. Last search: 29 September 2017.We searched two ongoing trial registries (Clinicaltrials.gov and the WHO trials platform). Date of latest search: 24 January 2018. Randomized and quasi-randomized controlled trials comparing anti-IgE therapy to placebo or other therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis. Two review authors independently extracted data and assessed the risk of bias in the included study. They planned to perform data analysis using Review Manager. Only one

  16. CT Imaging of Craniofacial Fibrous Dysplasia

    Directory of Open Access Journals (Sweden)

    Zerrin Unal Erzurumlu

    2015-01-01

    Full Text Available Fibrous dysplasia is a benign fibroosseous bone dysplasia that can involve single (monostotic or multiple (polyostotic bones. Monostotic form is more frequent in the jaws. It is termed as craniofacial fibrous dysplasia, when it involves, though rarely, adjacent craniofacial bones. A 16-year-old girl consulted for a painless swelling in the right posterior mandible for two years. Panoramic radiography revealed ground-glass ill-defined lesions in the three different regions of the maxilla and mandible. Axial CT scan (bone window showed multiple lesions involving skull base and facial bones. Despite lesions in the skull base, the patient had no abnormal neurological findings. The lesion was diagnosed as fibrous dysplasia based on radiological and histopathological examination. In this paper, CT findings and differential diagnosis of CFD are discussed. CT is a useful imaging technique for CFD cases.

  17. Congenital distal humeral dysplasia: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Joseph, Benjamin; Varghese, Renjit A. [Department of Orthopaedics, Kasturba Medical College, Manipal 576119, Karnataka State (India)

    2003-01-01

    Congenital dysplasia of the humerus is very rare. It is characteristically seen in omodysplasia and has also been reported as one of the associated features of Larsen's syndrome. We report a 4-year-old girl with bilateral humero-ulnar dysplasia, with dislocation of the elbows, facial dysmorphism, ball-and-socket ankles and foot deformities. Although the elbow dysplasia is similar to that seen in Larsen's syndrome, other pathognomic features of Larsen's syndrome were absent. The changes seen in the elbows in this patient are also different from those encountered in omodysplasia. We believe that this condition may be a distinct form of skeletal dysplasia hitherto undescribed. (orig.)

  18. Guidelines for the management of gastroenteropancreatic neuroendocrine tumours (including bronchopulmonary and thymic neoplasms). Part II-specific NE tumour types

    DEFF Research Database (Denmark)

    Oberg, Kjell; Astrup, Lone Bording; Eriksson, Barbro

    2004-01-01

    Part II of the guidelines contains a description of epidemiology, histopathology, clinical presentation, diagnostic procedure, treatment, and survival for each type of neuroendocrine tumour. We are not only including gastroenteropancreatic tumours but also bronchopulmonary and thymic neuroendocri...

  19. Isolated fibrous dysplasia of the sphenoid sinus.

    Science.gov (United States)

    Buyuklu, Fuat; Tarhan, Erkan; Cakmak, Ozcan; Ozgirgin, Nuri; Arikan, Unser

    2005-12-01

    Fibrous dysplasia is an uncommon benign bone disorder of unknown etiology in which normal medullary bone is replaced by fibrotic and osseous tissue. Solitary involvement of the sphenoid sinus is unusual. Here, we present the case of a 28-year-old man complaining of occipital and vertical headache. Imaging modalities demonstrated an expansile lesion filling the entire sphenoid sinus. Biopsy specimen was obtained by endoscopic sphenoidotomy. Diagnosis of fibrous dysplasia was made by imaging results and pathologic examination.

  20. Aggressive fibrous dysplasia of the maxillary sinus

    Energy Technology Data Exchange (ETDEWEB)

    Shapeero, L.G. (Dept. of Radiology, Inst. Gustave-Roussy, Villejuif (France) Dept. of Radiology, California Univ., San Francisco, CA (United States)); Vanel, D. (Dept. of Radiology, Inst. Gustave-Roussy, Villejuif (France)); Ackerman, L.V. (Dept. of Pathology, State Univ. of New York, Stony Brook, NY (United States)); Terrier-Lacombe, M.J. (Dept. of Radiology, Inst. Gustave-Roussy, Villejuif (France)); Housin, D. (Dept. of Radiology, Inst. Gustave-Roussy, Villejuif (France)); Schwaab, G. (Dept. of Ear, Nose, and Throat, Inst. Gustave-Roussy, Villejuif (France)); Sigal, R. (Dept. of Radiology, Inst. Gustave-Roussy, Villejuif (France)); Masselot, J. (Dept. of Radiology, Inst. Gustave-Roussy, Villejuif (France))

    1993-11-01

    Five of 34 patients (ages 4-21 years), who were subsequently diagnosed histologically as having fibrous dysplasia of the maxillary sinus, rapidly developed soft tissue masses of the malar region over a period of less than 4 months with accompanying pain (2 patients) and nasal obstruction and exophthalmos (2 patients). Each was clinically suspected of having a sarcoma. After resection, all lesions developed regrowth. At histopathologic examination, both initial and recurrent masses proved to be typical fibrous dysplasia. (orig./UWA)

  1. Isolated fibrous dysplasia of the ethmoid sinus

    OpenAIRE

    Yenigun, Alper; Akyuz, Servet

    2015-01-01

    Fibrous dysplasia is a benign fibro-osseous lesion progressing with one or more bone involvements in the skeleton. Even though it is a benign tumor, it may potentially transform into a malignant one. While the most frequently involved zones in the head–neck zone include the maxilla, mandible, parietal, occipital, and temporal zones, the involvement of the ethmoid bone is rather rare. This article presents the case of a female patient who was diagnosed with fibrous dysplasia in the right ethmo...

  2. Aggressive fibrous dysplasia of the maxillary sinus

    International Nuclear Information System (INIS)

    Shapeero, L.G.; Vanel, D.; Ackerman, L.V.; Terrier-Lacombe, M.J.; Housin, D.; Schwaab, G.; Sigal, R.; Masselot, J.

    1993-01-01

    Five of 34 patients (ages 4-21 years), who were subsequently diagnosed histologically as having fibrous dysplasia of the maxillary sinus, rapidly developed soft tissue masses of the malar region over a period of less than 4 months with accompanying pain (2 patients) and nasal obstruction and exophthalmos (2 patients). Each was clinically suspected of having a sarcoma. After resection, all lesions developed regrowth. At histopathologic examination, both initial and recurrent masses proved to be typical fibrous dysplasia. (orig./UWA)

  3. Sudden death in spondylo-meta-epiphyseal dysplasia, short limb-abnormal calcification type.

    Science.gov (United States)

    Dias, Cristina; Cairns, Robyn; Patel, Millan S

    2009-01-01

    The spondylo-meta-epiphyseal dysplasias are an expanding group of skeletal dysplasias with specific features differentiating each subtype. We review the precocious carpal mineralization, unique metacarpal shape, triangular distal phalanges and mushroom cloud-shaped proximal phalanges present at an early age in spondylo-meta-epiphyseal dysplasia, short limb-abnormal calcification type (SMED SL-AC) and report two patients with clinical and radiographic features consistent with SMED SL-AC, who died suddenly because of spinal cord compression. The patients presented are female siblings, providing further evidence for autosomal recessive inheritance. Cervical cord compression is found in half of reported patients and is the major cause of mortality. SMED SL-AC should be added to the list of genetic causes of sudden death. Radiological features in the hand may be used in the first few years of life to support an early diagnosis and thus allow for prevention of premature demise.

  4. Anesthetic management of a pediatric patient with hypohidrotic ectodermal dysplasia undergoing emergency surgery

    Directory of Open Access Journals (Sweden)

    Elif Oral Ahiskalioglu

    2015-12-01

    Full Text Available ABSTRACT Ectodermal dysplasias are rare conditions with a triad of hypotrichosis, anodontia and anhidrosis. In literature review there have been only a few reports of anesthetic management of patients with ectodermal dysplasias. Hyperthermia is a very serious risk which may occur due to the defect of sweat glands. The present case involves a 10-year-old child with ectodermal dysplasia who presented with an acute abdomen and was considered for an emergency surgery. Our aim was to demonstrate the successful management of this case using a combination of general and epidural anesthesia. It is important for anesthesiologist to have information about this syndrome in case of emergency operations, since it can prevent serious complications and even save lives.

  5. Fibrous dysplasia of the ethmoid sinus.

    Science.gov (United States)

    Tsai, Tung-Lung; Ho, Ching-Yin; Guo, Yuan-Ching; Chen, Winby; Lin, Ching-Zong

    2003-02-01

    Although craniofacial bone is the second common site of fibrous dysplasia involvement, it is rarely found in the paranasal sinus. Among fibrous dysplasia of the head and neck, the maxilla and mandible are the most frequent sites to be involved. Fibrous dysplasia becomes dormant in adolescence and early adult life and is more common in female. It is one of the fibrous osseous lesions and should be differentiated from osteoma and ossifying fibroma. Radiographically, fibrous dysplasia showed "groundglass" bone appearance on CT scans with bone window. Histopathologically, it presents woven-type bone embedded in a cellular fibrous stroma without osteoblastic rimming. We presented a case of 25-year-old female with fibrous dysplasia in her right side ethmoid sinus. She visited to us with the chief complaint of right side headache since adolescence. The lesion was removed by endoscopic sinus surgery and pathology proved fibrous dysplasia. The patient was free of headache after operation. The advance of endoscopic sinus surgical technique, makes it an optimal method for the pathological diagnosis and treatment to avoid the cosmetic problems caused by external approach in limited paranasal sinus fibrous osseous lesions.

  6. Functional state of the bronchopulmonary system in Mayak nuclear workers inhaled plutonium-239 aerosols

    International Nuclear Information System (INIS)

    Belyaeva, Z.; Grigoryeva, E.; Khokhryakov, V.

    2006-01-01

    Full text of publication follows: The current system of the individual and collective protection facilities for nuclear personnel permits decreasing the radiation dose from internal emitters at the most. At the same time, specific production conditions do not exclude possible inhalation of plutonium-239 aerosols. As the lung is the critical organ for this isotope, the study of respiratory function is aimed at detecting of pre-clinical lung pathology. Early detection of internal exposure effects on functional state of respiratory apparatus is difficult due to a number of confounding actors of non-radiation nature, one of which is smoking. Functional state of bronchopulmonary system was studied in 386 males, workers of the first Russian nuclear facility. 1198 examinations were carried out during medical inspection as well as hospitalization for routine preventive inspection. Most of males (39.4%) started working at the age of 21-26 years and 27.2% at the age of 20 years. The main factors of occupational exposure were exposure to plutonium-239 aerosols and the external gamma -rays. The absorbed dose to lungs from incorporated plutonium-239 was 0-435.8 c Gy. Whole-body external gamma dose varied from 0 to 382 c Gy at the examination. Individual dosimetry data were provided by the Mayak Radiation Safety Department and Internal Dosimetry Laboratory of the Southern Urals Biophysics Institute. While studying respiratory function, the most informative indices characterizing the state of lung tissue and tracheobronchial system such as vital capacity inspiration, forced expiratory volume, forced inspiratory volume, test Tiffno, diffusion capacity, characteristics of the flow vs. volume of the forced vital capacity inspiration curve, and resistance were used. Analysis was done separately for smokers and nonsmokers. Smoking index, i.e. product of number of smoked cigarettes per day and number of years of smoking was considered an integral value. The study did not reveal the

  7. [Role of PET/CT in primitive non-small cell bronchopulmonary cancer].

    Science.gov (United States)

    Soumia, Fdil; Leila, Achachi; Mohamed, Raoufi; Laila, Herrak; Mustapha, Elftouh

    2017-01-01

    Bronchopulmonary cancer is a real public health problem. Morphological imaging plays a central role in its diagnosis, staging as well as post-therapeutic assessment but it has some limitations. Metabolic imaging is a more recent technique which allows to significantly improve the overall imagery performance. We conducted a retrospective, descriptive and analytical study at the Ibn Sina Hospital and at the Military Hospital of instruction Mohammed V in Rabat over a period of 18 months, between September 2014 and February 2016, in order to evaluate the role of Fluorodeoxyglucose-PET/CT in the staging and restaging of non-small cell bronchopulmonary cancer. Initial staging showed a vast majority of locally advanced and metastatic stages: stage IV (40%), Stage IIIB (36%), Stage IIIA (16%), Stage II (8%). PET-CT allowed to detect new sites which were not initially seen on CT scan in 24 cases: 15 new ganglion sites, 8 new adrenal sites and 6 sites of bone lesions. PET/CT allowed to modify initial tumor stage in 60% of cases: upstaging in 23 patients (46%) and downstaging in 7 patients(14%). The initial stage remained unchanged in 40% of patients. Our study confirms the data from the literature concerning the superiority of PET-CT in comparison with CT scan, but only in the optimization of the non-small cell bronchopulmonary cancer management, in particular in locoregional and distant staging.

  8. Locally Aggressive Fibrous Dysplasia Mimicking Malign Calvarial Lesion.

    Science.gov (United States)

    Ogul, Hayri; Keskin, Emine

    2018-05-01

    Fibrous dysplasia is an unusual benign bone tumor. It is divided into 3 groups as monostotic, polyostotic, and craniofacial form. The authors reported an unusual patient with fibrous dysplasia with an aggressive radiologic appearance.

  9. Progressive pseudorheumatoid dysplasia in North and West Africa ...

    African Journals Online (AJOL)

    Eliane Chouery

    2017-01-05

    Jan 5, 2017 ... 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 sco-.

  10. 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 ...

  11. Inter-society consensus document on treatment and prevention of bronchiolitis in newborns and infants.

    Science.gov (United States)

    Baraldi, Eugenio; Lanari, Marcello; Manzoni, Paolo; Rossi, Giovanni A; Vandini, Silvia; Rimini, Alessandro; Romagnoli, Costantino; Colonna, Pierluigi; Biondi, Andrea; Biban, Paolo; Chiamenti, Giampietro; Bernardini, Roberto; Picca, Marina; Cappa, Marco; Magazzù, Giuseppe; Catassi, Carlo; Urbino, Antonio Francesco; Memo, Luigi; Donzelli, Gianpaolo; Minetti, Carlo; Paravati, Francesco; Di Mauro, Giuseppe; Festini, Filippo; Esposito, Susanna; Corsello, Giovanni

    2014-10-24

    Acute bronchiolitis is the leading cause of lower respiratory tract infection and hospitalization in children less than 1 year of age worldwide. It is usually a mild disease, but some children may develop severe symptoms, requiring hospital admission and ventilatory support in the ICU. Infants with pre-existing risk factors (prematurity, bronchopulmonary dysplasia, congenital heart diseases and immunodeficiency) may be predisposed to a severe form of the disease. Clinical diagnosis of bronchiolitis is manly based on medical history and physical examination (rhinorrhea, cough, crackles, wheezing and signs of respiratory distress). Etiological diagnosis, with antigen or genome detection to identify viruses involved, may have a role in reducing hospital transmission of the infection. Criteria for hospitalization include low oxygen saturation (bronchiolitis, and the mainstay of therapy is supportive care. This consists of nasal suctioning and nebulized 3% hypertonic saline, assisted feeding and hydration, humidified O2 delivery. The possible role of any pharmacological approach is still debated, and till now there is no evidence to support the use of bronchodilators, corticosteroids, chest physiotherapy, antibiotics or antivirals. Nebulized adrenaline may be sometimes useful in the emergency room. Nebulized adrenaline can be useful in the hospital setting for treatment as needed. Lacking a specific etiological treatment, prophylaxis and prevention, especially in children at high risk of severe infection, have a fundamental role. Environmental preventive measures minimize viral transmission in hospital, in the outpatient setting and at home. Pharmacological prophylaxis with palivizumab for RSV bronchiolitis is indicated in specific categories of children at risk during the epidemic period. Viral bronchiolitis, especially in the case of severe form, may correlate with an increased incidence of recurrent wheezing in pre-schooled children and with asthma at school age

  12. Bottom-of-sulcus dysplasia: imaging features.

    Science.gov (United States)

    Hofman, Paul A M; Fitt, Gregory J; Harvey, A Simon; Kuzniecky, Ruben I; Jackson, Graeme

    2011-04-01

    Dysplasia at the bottom of a sulcus is a subtle but distinct malformation of cortical development relevant to epilepsy. The purpose of this study was to review the imaging features important to the clinical diagnosis of this lesion. All cases recognized as typical bottom-of-sulcus dysplasia in our comprehensive epilepsy program over the period 2002-2007 were included in the study. In the 20 cases recognized, three major features were identified: cortical thickening at the bottom of a sulcus; a funnel-shaped extension of the lesion toward the ventricular surface, commonly with abnormal signal intensity; and an abnormal gyral pattern related to the bottom-of-sulcus dysplasia, sometimes with a puckered appearance. The pathologic features of the resected lesions were typical of focal cortical dysplasia. Bottom-of-sulcus dysplasia is a distinctive malformation of cortical development that can be diagnosed on the basis of imaging characteristics. Reliable identification of this type of malformation of cortical development is difficult but clinically important because the lesion appears to be highly epileptogenic and because the prognosis for seizure control is excellent after focal resection.

  13. Prevention

    Science.gov (United States)

    ... Error processing SSI file About Heart Disease & Stroke Prevention Heart disease and stroke are an epidemic in ... secondhand smoke. Barriers to Effective Heart Disease & Stroke Prevention Many people with key risk factors for heart ...

  14. [Fibrous dysplasia of the frontal sinus: a case report].

    Science.gov (United States)

    Keleş, Erol; Kaplama, Mehmet Erkan; Ozkara, Sule; Cobanoğlu, Bengü

    2011-01-01

    Fibrous dysplasia is a rare and indolent benign tumor of bone. Although it is a benign tumor, it has malignant transformation potential. Fibrous dysplasia can involve a single or multiple bones. The maxilla and mandible are the most common sites in the head and neck region. Paranasal sinus involvement is rare. Surgical excision is the preferred treatment of patients with symptomatic fibrous dysplasia. In this article, a 54-year-old male patient diagnosed with fibrous dysplasia of the frontal bone is presented.

  15. Thanatophoric Dysplasia; a Rare Case Report on a Congenital Anomaly

    OpenAIRE

    Maria Francis Yuvaraj; Ponuswamy Kasirajan Sankaran; Gunapriya Raghunath; Zareena Begum Kumaresan; Kumaresan kumaresan M

    2017-01-01

    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...

  16. Joint space width in dysplasia of the hip

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Sonne-Holm, Stig; Søballe, K

    2005-01-01

    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....... The association of subluxation and/or associated acetabular labral tears with dysplasia of the hip may be a conditional factor for the development of premature osteoarthritis in mildly to moderately dysplastic hips....

  17. Cervical Dysplasia In Hiv Seropositive Women In Nigeria | Tanko ...

    African Journals Online (AJOL)

    Conclusion: We conclude that the prevalence of cervical dysplasia is two 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, ...

  18. Surgical approach of hypertelorbitism in craniofrontonasal dysplasia.

    Science.gov (United States)

    Denadai, Rafael; Roberto, Wellington Matheus; Buzzo, Celso Luiz; Ghizoni, Enrico; Raposo-Amaral, César Augusto; Raposo-Amaral, Cassio Eduardo

    2017-01-01

    to present our experience in the hypertelorbitism surgical treatment in craniofrontonasal dysplasia. retrospective analysis of craniofrontonasal dysplasia patients operated through orbital box osteotomy or facial bipartition between 1997 and 2015. Surgical data was obtained from medical records, complementary tests, photographs, and clinical interviews. Surgical results were classified based on the need for additional surgery and orbital relapse was calculated. seven female patients were included, of whom three (42.86%) underwent orbital box osteotomy and four (57.14%) underwent facial bipartition. There was orbital relapse in average of 3.71±3,73mm. Surgical result according to the need for further surgery was 2.43±0.53. surgical approach to hypertelorbitism in craniofrontonasal dysplasia should be individualized, respecting the age at surgery and preferences of patients, parents, and surgeons.

  19. Skeletal dysplasia: Respiratory management during infancy.

    Science.gov (United States)

    Alapati, Deepthi; Shaffer, Thomas H

    2017-10-01

    Skeletal dysplasia encompasses a variety of developmental disorders of the bone and cartilage that manifest as disproportionate shortening of limbs and trunk in the neonate. Many types of skeletal dysplasia are complicated by respiratory failure at or soon after birth and require intensive care and prolonged hospitalization. Respiratory complications in these infants are complex and are characterized by airway anomalies, restrictive lung disease due to a narrow and abnormally compliant chest wall, pulmonary hypoplasia, and central apnea. Appropriate management of these unique patients requires a clear understanding of the pathophysiology and use of pulmonary function tests for early recognition and management of complications. This review provides an overview of the underlying respiratory pathology and a practical guide to the newborn care provider for the diagnosis and management of respiratory complications in infants with skeletal dysplasia. Copyright © 2017 Elsevier Ltd. All rights reserved.

  20. 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

  1. Craniofacial Fibrous Dysplasia of Zygomaticomaxillary Complex.

    Science.gov (United States)

    Nilesh, Kumar; Punde, Prashant; Parkar, M I

    2017-09-01

    Fibrous dysplasia is a benign bone disease first described by Lichtenstein in 1938. It is characterized by progressive replacement of normal bone with fibro-osseous connective tissue. When the disease involves craniofacial skeleton, it results in significant disfigurement and other functional problems. This paper reports a case of large craniofacial fibrous dysplasia involving zygomaticomaxillary complex in a 24-year old male patient. Clinical presentation and imaging characteristics of the pathology is discussed in detail. The disease caused significant facial asymmetry which was satisfactorily managed by surgical recontouring.

  2. Isolated fibrous dysplasia of the ethmoid sinus.

    Science.gov (United States)

    Yenigun, Alper; Akyuz, Servet

    2015-01-01

    Fibrous dysplasia is a benign fibro-osseous lesion progressing with one or more bone involvements in the skeleton. Even though it is a benign tumor, it may potentially transform into a malignant one. While the most frequently involved zones in the head-neck zone include the maxilla, mandible, parietal, occipital, and temporal zones, the involvement of the ethmoid bone is rather rare. This article presents the case of a female patient who was diagnosed with fibrous dysplasia in the right ethmoid sinus based on the paranasal computerized tomography scan that was taken due to the symptoms of pain around the right eye, pressure, and a feeling of being pushed.

  3. Altered Chondrocyte Apoptosis Status in Developmental Hip Dysplasia in Rabbits

    Directory of Open Access Journals (Sweden)

    Yi-Shan Wei

    2016-12-01

    Full Text Available Background: Developmental dysplasia of the hip (DDH is an important factor leading to early adult osteoarthritis. Chondrocyte apoptosis has been proven to be an important factor causing osteoarthritis. Aims: The current study aims to explore whether a rabbit model of developmental dysplasia of the hip through cast immobilization in the legs results in chondrocyte apoptosis. Study Design: Animal experimentation. Methods: Thirty-two New Zealand white rabbits were divided in three groups with cast plaster-induced dislocation at 2, 4 and 6 weeks. The contralateral hip joint was utilized as a control group. Ten rabbits in each group were sacrificed, and hip specimens were obtained. Bcl-2/Bax, cleaved caspase-3 and cleaved caspase-8 expression were examined by western blot analysis. Chondrocyte apoptosis was analyzed through transmission electron microscopy (TEM and TUNEL analysis. All experiments were repeated at least three times. Results: In the experimental group, Bcl-2/Bax, cleaved caspase-3 and cleaved caspase-8 expression were significantly altered. The Bcl-2/Bax ratio decreased with time (all p<0.01, whereas levels of cleaved caspase-3 (p<0.01 and p<0.05 and cleaved caspase-8 (all p<0.05 gradually increased. Chondrocyte apoptosis was observed through transmission electron microscopy (TEM and TUNEL analysis (p<0.05 at 4 weeks and p<0.01 at 6 weeks. Conclusion: Prolonged immobilization of rabbit hip caused chondrocyte apoptosis. Reduction of the hip joint may protect chondrocytes from apoptosis, thus preventing secondary osteoarthritis.

  4. Altered Chondrocyte Apoptosis Status in Developmental Hip Dysplasia in Rabbits.

    Science.gov (United States)

    Wei, Yi-Shan; Li, Dai-He; Liu, Wan-Lin; Jiang, Dian-Ming

    2016-11-01

    Developmental dysplasia of the hip (DDH) is an important factor leading to early adult osteoarthritis. Chondrocyte apoptosis has been proven to be an important factor causing osteoarthritis. The current study aims to explore whether a rabbit model of developmental dysplasia of the hip through cast immobilization in the legs results in chondrocyte apoptosis. Animal experimentation. Thirty-two New Zealand white rabbits were divided in three groups with cast plaster-induced dislocation at 2, 4 and 6 weeks. The contralateral hip joint was utilized as a control group. Ten rabbits in each group were sacrificed, and hip specimens were obtained. Bcl-2/Bax, cleaved caspase-3 and cleaved caspase-8 expression were examined by western blot analysis. Chondrocyte apoptosis was analyzed through transmission electron microscopy (TEM) and TUNEL analysis. All experiments were repeated at least three times. In the experimental group, Bcl-2/Bax, cleaved caspase-3 and cleaved caspase-8 expression were significantly altered. The Bcl-2/Bax ratio decreased with time (all phip caused chondrocyte apoptosis. Reduction of the hip joint may protect chondrocytes from apoptosis, thus preventing secondary osteoarthritis.

  5. Deafness and Mondini dysplasia in Kabuki (Niikawa-Kuroki) syndrome. Report of a case and review of the literature.

    Science.gov (United States)

    Toutain, A; Plée, Y; Ployet, M J; Benoit, S; Perrot, A; Sembely, C; Barthez, M A; Moraine, C

    1997-01-01

    Report of a case and review of the literature: We report the case of a seven-year-old female kabuki patient suffering from severe bilateral deafness related to Mondini dysplasia and ossicular anomalies. A review of the literature in English confirms that hearing loss is a major component of Kabuki Syndrome (KS) with a frequency at around 32%. However the possible mechanisms have not been fully described and hearing loss is often attributed to otitis media, but one reported case had severe ossicular malformations, two had sensorineural deafness and three others had mixed deafness. Our observation is the first reported case of Mondini dysplasia in KS. Awareness by physicians of this problem has a major practical consequence as diagnosis of Mondini dysplasia implies searching for and surgical prevention and treatment of perilymphatic fistula in order to prevent meningitis.

  6. The Bone Dysplasia Ontology: integrating genotype and phenotype information in the skeletal dysplasia domain

    Directory of Open Access Journals (Sweden)

    Groza Tudor

    2012-03-01

    Full Text Available Abstract Background Skeletal dysplasias are a rare and heterogeneous group of genetic disorders affecting skeletal development. Patients with skeletal dysplasias suffer from many complex medical issues including degenerative joint disease and neurological complications. Because the data and expertise associated with this field is both sparse and disparate, significant benefits will potentially accrue from the availability of an ontology that provides a shared conceptualisation of the domain knowledge and enables data integration, cross-referencing and advanced reasoning across the relevant but distributed data sources. Results We introduce the design considerations and implementation details of the Bone Dysplasia Ontology. We also describe the different components of the ontology, including a comprehensive and formal representation of the skeletal dysplasia domain as well as the related genotypes and phenotypes. We then briefly describe SKELETOME, a community-driven knowledge curation platform that is underpinned by the Bone Dysplasia Ontology. SKELETOME enables domain experts to use, refine and extend and apply the ontology without any prior ontology engineering experience--to advance the body of knowledge in the skeletal dysplasia field. Conclusions The Bone Dysplasia Ontology represents the most comprehensive structured knowledge source for the skeletal dysplasias domain. It provides the means for integrating and annotating clinical and research data, not only at the generic domain knowledge level, but also at the level of individual patient case studies. It enables links between individual cases and publicly available genotype and phenotype resources based on a community-driven curation process that ensures a shared conceptualisation of the domain knowledge and its continuous incremental evolution.

  7. 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.

  8. 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.

  9. Pseudotumoral fibrous dysplasia of the maxilla

    International Nuclear Information System (INIS)

    Vanel, D.; Couanet, D.; Piekarski, J.D.; Masselot, J.; Micheau, C.; Schwaab, G.

    1980-01-01

    Clinical, radiological, and histological problems arise relating to fibrous dysplasia of the maxilla. Clinically, 11 of our 39 cases developed so rapidly that the lesions were suspected of being malignant. The histological diagnosis may be very difficult, since any tumoural, reactive, or healing process may simulate fibrous dysplasia. As a general rule, the diagnosis is established most easily by the radiological examination. The radiologist's responsibility is important, as mistakes may have serious consequences. We report four pseudotumoural forms of maxillary sinus fibrous dysplasia. All were characterised clinically by rapid evolution, radiologically by opacity of the sinus with apparent destruction of its wall, and histologically by difficulty in establishing the diagnosis. In two cases indeed, the initial histological interpretation was an osteogenic sarcoma. Due to its excellent densitometric resolution, computed tomography provides an invaluable contribution by displaying the fibrous wall of an intact or even thickened maxillary sinus when conventional radiology has suggested a destructive process. In difficult cases of maxillary fibrous dysplasia, computed tomography should be used as a supplementary investigation to establish the correct diagnosis. (orig.) [de

  10. 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

  11. Williams-Beuren Syndrome with Brain Dysplasia

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2013-10-01

    Full Text Available Investigators from Jichi and Yokohama City Universities, Japan, report a patient with the common Williams-Beuren syndrome (WBS deletion in 7q11.23 who presented with severe cerebral and cerebellar dysplasia and progressive hypertrophic cardiomyopathy.

  12. MR findings of the osteofibrous dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Jung, Joon Yong; Jee, Won Hee [College of Medicine, The Catholic University of Korea, Seoul (Korea, Republic of); Hong, Sung Hwan [Dept. of Radiology, Seoul National University College of Medicine, Seoul (Korea, Republic of); and others

    2014-02-15

    The aim of this study was to describe MR findings of osteofibrous dysplasia. MR images of 24 pathologically proven osteofibrous dysplasia cases were retrospectively analyzed for a signal intensity of the lesion, presence of intralesional fat signal, internal hypointense band, multilocular appearance, cortical expansion, intramedullary extension, cystic area, cortical breakage and extraosseous extension, abnormal signal from the adjacent bone marrow and soft tissue and patterns of contrast enhancement. All cases of osteofibrous dysplasia exhibited intermediate signal intensity on T1-weighted images. On T2-weighted images, 20 and 4 cases exhibited heterogeneously intermediate and high signal intensity, respectively. Intralesional fat was identified in 12% of the cases. Internal low-signal bands and multilocular appearance were observed in 91%. Cortical expansion was present in 58%. Intramedullary extension was present in all cases, and an entire intramedullary replacement was observed in 33%. Cortical breakage (n 3) and extraosseous mass formation (n = 1) were observed in cases with pathologic fractures only. A cystic area was observed in one case. Among 21 cases without a pathologic fracture, abnormal signal intensity in the surrounding bone marrow and adjacent soft tissue was observed in 43% and 48%, respectively. All cases exhibited diffuse contrast enhancement. Osteofibrous dysplasia exhibited diverse imaging features ranging from lesions confined to the cortex to more aggressive lesions with complete intramedullary involvement or perilesional marrow edema.

  13. 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 ...

  14. A new lethal sclerosing bone dysplasia

    International Nuclear Information System (INIS)

    Kingston, H.M.; Freeman, J.S.; Hall, C.M.

    1991-01-01

    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.)

  15. Florid periosteal reaction and focal fibrocartilaginous dysplasia

    International Nuclear Information System (INIS)

    Nakase, T.; Yasui, Natsuo; Araki, Nobuhito; Tanaka, Makoto; Yoshikawa, Hideki; Ochi, Takahiro; Kuratsu, Shigeyuki

    1998-01-01

    Focal fibrocartilaginous dysplasia (FFCD) is a rare condition causing tibia vara in childhood. It is characterized by progressive tibia vara in young children with a characteristic radiographic lesion. This paper is thought to be the first to describe FFCD exhibiting florid periosteal reaction at the time of presentation with a subtle faint osteolytic lesion in the diametaphysis of the proximal tibia. (orig.)

  16. Cochlear implantation in a bilateral Mondini dysplasia.

    Science.gov (United States)

    Turrini, M; Orzan, E; Gabana, M; Genovese, E; Arslan, E; Fisch, U

    1997-01-01

    We report the speech perception progress and programming procedures of a case of congenital profound deafness and bilateral Mondini dysplasia implanted with a Nucleus 20 + 2 cochlear implant at the age of six. Unclear relations between electrodes array and cochlear partition made implant programming difficult and non-standard procedures were set. Cochlear implantation may give excellent rehabilitative results also in cochleae with malformation.

  17. 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.

  18. 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.

  19. Excellent outcome of Aspergillous endophthalmitis in a case of allergic bronchopulmonary aspergillosis

    Directory of Open Access Journals (Sweden)

    Balbir Khan

    2014-01-01

    Full Text Available While invasive aspergillosis occurs typically in severely immunocompromised patients, cases of surgical site infections have been reported in immunocompetent individuals. The purpose is to report an eye with post-operative Aspergillus endophthalmitis, which achieved a good visual outcome following early and aggressive treatment. A young patient, known case of allergic bronchopulmonary aspergillosis presented to us with post-cataract surgery endophthalmitis. He was treated with pars plana vitrectomy and intravitreal voriconazole and systemic itraconazole. The patient regained a vision of 20/30 with follow up of 2 years.

  20. Prevention

    Science.gov (United States)

    ... Contact Aging & Health A to Z Find a Geriatrics Healthcare Professional Medications & Older Adults Making Your Wishes ... Prevention Hearing Loss Heart Attack High Blood Pressure Nutrition Osteoporosis Shingles Skin Cancer Related News Quitting Smoking, ...

  1. Prevention

    DEFF Research Database (Denmark)

    Halken, S; Høst, A

    2001-01-01

    , breastfeeding should be encouraged for 4-6 months. In high-risk infants a documented extensively hydrolysed formula is recommended if exclusive breastfeeding is not possible for the first 4 months of life. There is no evidence for preventive dietary intervention neither during pregnancy nor lactation...... populations. These theories remain to be documented in proper, controlled and prospective studies. Breastfeeding and the late introduction of solid foods (>4 months) is associated with a reduced risk of food allergy, atopic dermatitis, and recurrent wheezing and asthma in early childhood. In all infants....... Preventive dietary restrictions after the age of 4-6 months are not scientifically documented....

  2. 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

  3. The removal of inhaled 239Pu from beagle dogs by bronchopulmonary lavage and chelation therapy

    International Nuclear Information System (INIS)

    Muggenburg, B.A.; Mewhinney, J.A.; Slauson, D.O.; Miglio, J.J.; Ruoff, L.; Mersch, S.; McClellan, R.O.

    1976-01-01

    The efficacy of bronchopulmonary lavage and chelatan therapy for removing 239 Pu from beagle dogs after inhalation of 239 Pu aerosols having different solubilities has been investigated. The four aerosols used were nebulized from a solution of 239 PuCl 4 and heat treated at temperatures of 325, 600, 900 and 1150 0 C. Groups of six beagle dogs were exposed to each of the aerosols. Subsequently, three dogs in each group were treated by lavage and intravenous injections of DTPA. The remaining three dogs in each group served as untreated controls. It was found that bronchopulmonary lavage treatment was effective in removing nearly half of the 239 Pu activity from the lung regardless of the aerosol production temperature. This early removal of 239 Pu activity resulted in a significant reduction in daily dose rate and therefore cumulative α dose to lung. The effectiveness of DTPA treatment depended on aerosol production temperature, and was effective in reducing accumulation of 239 Pu in liver and skeleton of the dogs that inhaled aerosols produced at 325 0 and 600 0 C by enhancing urinary excretion of 239 Pu. (U.K.)

  4. Radiological diagnosis of immunologically mediated disorders of the bronchopulmonary system in children and adolescents

    International Nuclear Information System (INIS)

    Ball, F.

    1990-01-01

    After coverage of pathophysiological mechanisms, radiological symptoms and differential diagnosis of bacterial and opportunistic infections of the bronchopulmonary system are discussed as they occur in humoral, cellular and combined congenital and acquired immune deficiencies. The discussion is based on case reports. Humoral deficiences cause recurrent and chronic bacterial infections of the bronchopulmonary system, frequently with bronchiectasis. In the case of cellular and combined immune deficiencies, not only bacterial infections but also the very serious opportunistic infections occur. Opportunistic infections of the lung are predominantly caused by Pneumocystis carinii, by the cytomegaly virus, and by fungi such as Candida, Aspergillus and Mucor. Pneumocystis is also the most frequent cause of opportunistic infections of the lungs in children with AIDS. In contrast to the situation in adults, in children a relatively low-grade lymphocytic interstitial pneumonitis occasionally precedes the typical opportunistic infections. Lymphocytic interstitial pneumonitis and Pneumocystis pneumonia can be differentiated from each other easily in children because of their relatively characteristic appearances. Fungal infections, on the other hand, sometimes pose severe diagnostic problems. Radiological chest findings in autoimmune diseases are discussed. (orig.)

  5. Hip Ultrasonography in the Diagnosis of Developmental Dysplasia of the Hip: Bakırköy Experience

    Directory of Open Access Journals (Sweden)

    Altuğ Duramaz

    2014-12-01

    Full Text Available Aim: The purpose of the study was to determine the prevalence, incidence, and etiology as well as the risk factors for developmental dysplasia of the hip in newborns in whom we performed ultrasonography for screening using Graff’s method in our clinic. Methods: We retrospectively evaluated 2632 hip ultrasonography records of 1316 babies performed between 2008 and 2013. We analyzed the questionnaires of the Turkish Pediatric Orthopaedic Society which were filled by the physician during examination. The babies were divided into two groups according to ultrasonographic hip angles as pathological and normal. Results: The study is made on 1316 babies [680 girls (51.6%, 636 boys (48.4%]. The risk for developmental dysplasia of the hip was higher in girls, babies with a family history, babies with metatarsus adductus and those have been swaddled before. The mean gestational age and gestational weight was statistically significantly lower in the pathological group (p=0.0011. Conclusion: In our cross-sectional study, the incidence of developmental dysplasia of the hip was 0.5%. Female gender, positive family history of developmental dysplasia of the hip,metatarsus adductus and swaddling are still risk factors. Researching risk factors carefully, patient education and adding hip ultrasonograpy to newborn routine screening program are important measures in preventing developmental dysplasia of the hip.

  6. 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.

  7. Isolated fibrous dysplasia of the ethmoid sinus

    Directory of Open Access Journals (Sweden)

    Alper Yenigun

    2015-01-01

    Full Text Available Fibrous dysplasia is a benign fibro-osseous lesion progressing with one or more bone involvements in the skeleton. Even though it is a benign tumor, it may potentially transform into a malignant one. While the most frequently involved zones in the head–neck zone include the maxilla, mandible, parietal, occipital, and temporal zones, the involvement of the ethmoid bone is rather rare. This article presents the case of a female patient who was diagnosed with fibrous dysplasia in the right ethmoid sinus based on the paranasal computerized tomography scan that was taken due to the symptoms of pain around the right eye, pressure, and a feeling of being pushed.

  8. 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.

  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. MRA of fibromuscular dysplasia in cervical vessels

    International Nuclear Information System (INIS)

    Link, J.; Steffens, J.C.; Mueller-Huelsbeck, S.; Brossmann, J.; Heller, M.

    1996-01-01

    In 386 selective angiograms of cervical vessels fibromuscular dysplasia was revealed in 4 female patients in the age of 30-54 years. FMD was located in the carotid artery (n=5) and in the vertebral artery (n=2) with a total of 8 lesions. 6/8 of the lesions of the seven cervical vessels were located typically in the mid cervical portion of the vessels and 2/6 lesions were located in the atlas loop of the vertebral artery. 4 lesions showed moderate stenosis and 4 vessels showed only mild stenosis. These patterns which demonstrated the typical morphology of fibromuscular dysplasia with alternating irregular zones of widening and narrowing were evaluated well with MR angiography, the others were missed. (orig./MG) [de

  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. 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.

  13. Monostotic fibrous dysplasia with Raynaud's phenomenon.

    Science.gov (United States)

    Kumar, K V S Hari; Aravinda, K; Narayanan, K

    2015-01-01

    Fibrous dysplasia (FD) is a benign bone disorder characterized by alteration in bone morphology. Monostotic FD is the commonest variant and affects the craniofacial bones. Raynaud's phenomenon is recurrent vasospasm of the fingers and toes due to cold exposure. The disease is usually idiopathic or secondary to connective tissue disorders. Raynaud's phenomenon is not described previously with FD. We recently encountered two interesting patients of craniofacial monostotic FD with Raynaud's phenomenon and report the same in this report.

  14. Fibrous dysplasia of the paranasal sinuses.

    Science.gov (United States)

    Feldman, M D; Rao, V M; Lowry, L D; Kelly, M

    1986-09-01

    Fibrous dysplasia (FD) of the paranasal sinuses is rare and often presents a diagnostic challenge. It is usually secondary to extension of disease from adjacent bones and is rarely limited to the sinuses. We have described three cases of FD involving the paranasal sinuses, including the first reported case of FD isolated to the sphenoid sinus. A brief update of the clinical aspects, radiographic appearance, diagnosis, and management of craniofacial FD is provided.

  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. 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.)

  17. Fibrous dysplasia of the maxillary sinus: case report

    OpenAIRE

    Vijayalakshmi Subramaniam; Adarsha Tuluvinakoppa Vasudeva Herle

    2010-01-01

    Introduction and objective: Fibrous dysplasia is a non-neoplastic lesion of unknown origin with one-fourth involving head and neck. The aim of this paper is to report a case of fibrous dysplasia of the maxillary sinus, describing its clinical presentation, radiological features, histopathological appearance and surgical management. Case report: 3-year-old female patient who presented with a history of fullness of the right cheek and intraoral swelling was diagnosed to have fibrous dysplasia o...

  18. Craniofacial fibrous dysplasia surgery: a functional approach.

    Science.gov (United States)

    Béquignon, E; Cardinne, C; Lachiver, X; Wagner, I; Chabolle, F; Baujat, B

    2013-09-01

    Craniofacial fibrous dysplasia has not only esthetic but functional impact. Surgery is controversial, ranging from conservative to radical. It involves elevated hemorrhage risk, and should be progressive, based on an individual risk/benefit analysis with the aim of improving quality of life. Three patients (one male, two female; mean age, 35 years) with evolutive orbital-temporal maxillary dysplasia were treated between 2008 and 2009 in our department. All showed exophthalmia and nasal obstruction. In one patient, symptomatology was aggravated by a frontal sinus cyst within the dysplasia. Another had associated auditory canal obstruction inducing recurrent external otitis. Optic nerve decompression was achieved on a combined coronal and endonasal approach, assisted by neuronavigation. Complementary remodelling resection, dacryocystorhinostomy and internal optic nerve decompression were performed. Functional results showed 70 % improvement on a subjective scale for eye tension and nasal obstruction. Surgery was feasible in all patients, with no complications. Current surgical management allies esthetic and functional concerns. Remodeling resection is the reference technique. The coronal approach is a good primary option for optic nerve decompression. Endonasal surgery with neuronavigation improves nasal ventilation and lacrimal canal permeability. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  19. 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.

  20. Caffeine prevents bilirubin-induced cytotoxicity in cultured newborn rat astrocytes.

    Science.gov (United States)

    Deliktaş, Mehmet; Ergin, Hacer; Demiray, Aydın; Akça, Hakan; Özdemir, Özmert M A; Özdemir, Mehmet Bülent

    2018-01-02

    Unconjugated bilirubin (UCB) may cause neurotoxicity in preterm neonates due to immaturity of UGT1A1 leading to bilirubin accumulation in the brain. Caffeine used in the treatment of apnea of prematurity was reported to decrease mechanical ventilation requirement, the frequencies of bronchopulmonary dysplasia, patent ductus arteriosus, cerebral palsy and neurodevelopmental disorders in very low birth weight infants. However, the effect of caffeine on hyperbilirubinemia was not yet clarified. We used astrocyte cell cultures obtained from 2-day-old Wistar albino rats via modified Cole and de Vellis method. UCB concentration toxic to 50% of astrocytes, and caffeine concentration increasing cell viability 100% were used in experiments. While no medication was applied to the control group, UCB (50 μM) and caffeine (100 μM) were applied to the bilirubin and caffeine groups for 24 h. Prophylactic and therapeutic caffeine groups were treated with caffeine 4 h before and after UCB exposure. The effects of caffeine were investigated in rat astrocytes exposed to UCB in terms of cell viability, apoptosis, antioxidant defense, proinflammatory cytokines, and Toll-like receptor (TLR)s. Compared to the control group, UCB increased apoptosis, malondialdehyde (MDA), tumor necrosis factor-α (TNF-α), interleukin (IL)-1β, IL-6, total nitrate/nitrite, and TLR4 levels, and decreased cell viability, catalase (CAT), glutathione peroxidase (GPx), superoxide dismutase (SOD) activities, glutathione, and TLR9 levels (for all p < .001). Conversely, prophylactic and therapeutic caffeine improved the detrimental effects of UCB. Caffeine seems encouraging for the prevention and treatment of bilirubin neurotoxicity in rats by means of its antiapoptotic, antioxidant, anti-inflammatory, anti-nitrosative, and anti-TLR-4 properties.

  1. 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.

  2. Comparison of three methods to diagnose hip dysplasia in dogs

    International Nuclear Information System (INIS)

    Sharma, Vikas; Mohindroo, J.

    2009-01-01

    The present study was designed to compare the usefulness of goniometry, radiography and distraction index in diagnosis of hip dysplasia in dogs. During the study 25 clinical cases (50 joints) suspected for hip dysplasia were evaluated. Norberg angle was found to have a significant positive correlation with extension, flexion, abduction, and adduction angles and a significant negative correlation with distraction index (DI) measurements. It could be inferred that all the six parameters (NA, DI, extension, flexion, abduction, and adduction) were reliable indicators for early diagnosis of hip dysplasia.Goniometry could be used as a safe and easy method for preliminary suspicion of hip dysplasia

  3. [Mondini dysplasia: traumatic cerebrospinal fluid otorrhea with meningitis].

    Science.gov (United States)

    Kaftan, H; Adamaszek, M; Hosemann, W

    2006-08-01

    Mondini dysplasia is a rare malformation of the inner ear commonly associated with loss of hearing and vestibular function. Children with Mondini dysplasia are predisposed to developing a spontaneous cerebrospinal fluid (CSF) leak and recurrent meningitis. If there is no CSF leak but a unilateral hearing loss, the condition may go undiagnosed for years. We describe a 65-year-old man with unrecognized unilateral Mondini dysplasia who presented with CSF leak and meningoencephalitis after minor head trauma. Two operative interventions were undertaken to close the defect properly. Patients with Mondini dysplasia or their parents should be cautioned about the potential hazards of any head trauma.

  4. Dentinal Dysplasia Type I: A Case Report with a 6-Year Followup

    Directory of Open Access Journals (Sweden)

    Sezin Ozer

    2013-01-01

    Full Text Available Introduction. Dentin dysplasia is a rare disturbance of dentin formation characterized by normal enamel but atypical dentin formation with abnormal pulpal morphology that is inherited as an autosomal pulpal morphology. Case Presentation. A 7-year-old female who had problems in chewing function was referred to Oral and Maxillofacial Surgery Department at the Faculty of Dentistry in Ondokuz Mayıs University. In the radiographic examination, it was determined that some of the unerupted permanent teeth of the patient had short, blunted, and malformed roots with obliterated pulp chambers, although the bone below the teeth showed well-defined margins. This unusual case of generalized short roots presents a case demonstrating both classic and atypical features of dentinal dysplasia type I (DDI in the mixed and permanent dentitions. Conclusion. There are still many issues in the diagnosis and management of patients with dentin dysplasia. Early diagnosis, clinical and radiographic findings, as well as treatment of this condition and the initiation of effective preventive strategies may help prevent or delay loss of dentition.

  5. Stem-cell-specific endocytic degradation defects lead to intestinal dysplasia in Drosophila

    Directory of Open Access Journals (Sweden)

    Péter Nagy

    2016-05-01

    Full Text Available UV radiation resistance-associated gene (UVRAG is a tumor suppressor involved in autophagy, endocytosis and DNA damage repair, but how its loss contributes to colorectal cancer is poorly understood. Here, we show that UVRAG deficiency in Drosophila intestinal stem cells leads to uncontrolled proliferation and impaired differentiation without preventing autophagy. As a result, affected animals suffer from gut dysfunction and short lifespan. Dysplasia upon loss of UVRAG is characterized by the accumulation of endocytosed ligands and sustained activation of STAT and JNK signaling, and attenuation of these pathways suppresses stem cell hyperproliferation. Importantly, the inhibition of early (dynamin-dependent or late (Rab7-dependent steps of endocytosis in intestinal stem cells also induces hyperproliferation and dysplasia. Our data raise the possibility that endocytic, but not autophagic, defects contribute to UVRAG-deficient colorectal cancer development in humans.

  6. Inflammatory myofibroblastic tumor appendix with concomitant mucosal dysplasia, simulating pseudomyxoma on preoperative aspiration cytology

    Directory of Open Access Journals (Sweden)

    Kaushik Majumdar

    2012-01-01

    Full Text Available Inflammatory myofibroblastic tumor (IMT has been described as a pseudosarcomatous proliferation of spindled myofibroblasts admixed with lymphoplasmacytic cells. The various terminologies like inflammatory pseudotumor, plasma cell granuloma, and inflammatory myofibrohistiocytic proliferation, used to describe this entity, highlight the controversial etiopathogenesis of this relatively indolent neoplasm. IMT has now been described in different anatomic locations. However, cases occurring in the gastrointestinal tract are rare with very few cases described in the appendix. We present a case of inflammatory myofibroblastic tumor appendix with mucosal dysplasia in a 41-year-old male, presenting with abdominal pain and lump in the right iliac fossa. Aspiration cytology yielded few atypical epithelial cells and spindle cells in a mucinous background, suggesting the possibility of pseudomyxoma peritonei. Awareness of IMT appendix with rare presence of mucosal dysplasia may help in preventing overzealous resection, especially in situations that on preoperative evaluation may suggest malignancy.

  7. Typical and atypical bronchopulmonary carcinoid tumors: a clinicopathologic and KI-67-labeling study.

    Science.gov (United States)

    Costes, V; Marty-Ané, C; Picot, M C; Serre, I; Pujol, J L; Mary, H; Baldet, P

    1995-07-01

    Expression of a proliferating antigen by KI-67 immunohistochemistry was analyzed with a SAMBA 2005 computer-assisted image processor (Traitement de l'Information for des Techniques Nouvelles, Grenoble, France) in 47 surgically resected bronchopulmonary carcinoids embedded in paraffin. The clinicopathologic characteristics and KI-67 labeling, expressed in percentage of stained nuclear surface relative to the total nuclear surface, of 31 typical carcinoids and 16 atypical carcinoids were compared and assessed with respect to patient survival. The proliferation status was significantly higher in histologically atypical than in typical carcinoids. Moreover, using a 4% cutoff, we observed a significant difference for the 4-year overall survival rate. Semiquantitative analysis of the proliferation index by KI-67 immunostaining seemed to be an effective means of identifying high risk subsets among patients with histologically atypical carcinoids and for whom adjuvant chemotherapy could be proposed.

  8. 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.

  9. Computed tomography protocols used in staging bronchopulmonary carcinoma: results of a national survey.

    Science.gov (United States)

    García Garrigós, E; Arenas Jiménez, J J; Sánchez Payá, J; Sirera Matilla, M; Gayete Cara, À

    To know the protocols used for staging bronchopulmonary carcinoma by computed tomography in Spain. Radiologists in 129 hospitals were sent email questionnaires about the organization of their department, scanner type and manufacturer, study extension, techniques employed, and protocol for administering contrast material. A total of 109 hospitals responded with data from 91 teams. Most hospitals were affiliated with a university, and most departments were organized by organ-systems. Scanners were from four manufacturers, and 68% had either 16 or 64 detectors. In 61% of the hospitals, the dose of contrast agent is modified only in patients with extreme body weights, and in 22% the dose is not individualized. Most hospitals do contrast-enhanced studies of the chest and upper abdomen, 42.4% through a single thoracoabdominal acquisition and 55.9% through independent chest and abdominal acquisitions; there was a significant association between these approaches and the scanner manufacturer's protocols and whether the hospital was affiliated with a university. The most commonly used technical parameters were 120kV with dose modulation and variable milliamperage. There is very little variability among hospitals in the type of scanner used, the study extension, and the technical parameters used to stage bronchopulmonary carcinoma. Most centers individualize the dose of contrast agent only in extreme weights. There is a broad division between using one or two acquisitions to image the thorax and abdomen, and the number of acquisitions is related to the scanner manufacturer and whether the hospital is affiliated with a university. Copyright © 2016 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.

  10. Bronchopulmonary Disease Caused by Flagellated Protozoa Infection in 15 Chinese Children.

    Science.gov (United States)

    Liu, Jinrong; Li, Shaogang; Li, Huimin; Fan, Yimu; Yang, Haiming; Xu, Hui; Shen, Yuelin; Zhao, Shunying

    2017-04-01

    Bronchopulmonary disease caused by flagellated protozoa infection (BPFP) is thought to be rare in children but may be an emerging or underestimated disease, especially in developing countries. In this study, we retrospectively reviewed records of 15 patients who were presented with a cough, wheezing or bronchopulmonary disease of unknown causes during admission, and patients who were finally diagnosed with BPFP from January 2014 to January 2015 were enrolled. Protozoa were observed in bronchoalveolar lavage fluid by light microscopy. A total of 15 pediatric cases (11 male and 4 female individuals, from 1 year 8 months to 12 years 1 month of age) with flagellated protozoa infection diagnosed by light microscopy were recruited. The course of the disease at the time of diagnosis was from 10 days to 11 months. Patients presented with a fever (N = 9), cough (N = 11), wheezing (N = 5) and chest pain (N = 5). Laboratory data showed elevated peripheral blood leucocytes (N = 6), eosinophilic granulocytes (N = 3), C-reactive protein (N = 5) and immunoglobulin E (N = 3). Bronchoscopy revealed a mucus plug (N = 3) and bronchiectasis (N = 1). Lung computed tomography results indicated ground-glass opacification (N = 2), atelectasis (N = 3), bronchiectasis (N = 1), bronchial wall thickening (N = 3) or nodular opacity (N = 6, including 1 case of pulmonary embolism). All children responded to metronidazole for a 2- to 5-week treatment period. Patients with BPFP often have a chronic or recurrent course and present with recurrent fever, cough, wheezing and chest pain. Chest imaging may reveal ground-glass opacification, atelectasis, bronchiectasis or nodular opacity (including pulmonary embolism). BPFP responds favorably to metronidazole treatment.

  11. 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

  12. An unusual case of fibrous dysplasia of the maxillary sinus.

    Science.gov (United States)

    Crawford, Larry B

    2003-12-01

    Fibrous dysplasia of the maxillary sinus might initially become apparent because of facial asymmetry. It can be differentiated from other osseo-odontogenic dysplasias radiographically by its occupancy of the affected sinus and by its diffuse, ground-glass, radiopaque appearance; it can be further confirmed with histologic findings. It is normally self-limiting in growth and does not usually become malignant.

  13. Prevalence of Cervical Dysplasia among Women in Kano Municipal ...

    African Journals Online (AJOL)

    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 grade squamous intraepithelial lesions (LGSIL), while 36(63.2%) had a high grade squamous intraepithelial lesion (HGSIL). The disease was higher in grand multiparous clients ...

  14. Further delineation of spondylometaphyseal dysplasia with cone-rod dystrophy

    NARCIS (Netherlands)

    Sousa, Sérgio B.; Russell-Eggitt, Isabelle; Hall, Christine; Hall, Bryan D.; Hennekam, Raoul C. M.

    2008-01-01

    There are several entities that combine a skeletal dysplasia with a retinal dystrophy. Recently, another possibly autosomal recessive entity was added to this group characterized by a specific spondylometaphyseal dysplasia and a cone-rod dystrophy, without other significant impairments. The entity

  15. The role of the acetabular labrum in hip dysplasia

    DEFF Research Database (Denmark)

    Hartig-Andreasen, Charlotte; Søballe, Kjeld; Troelsen, Anders

    2013-01-01

    A periacetabular osteotomy (PAO) is the preferred joint preserving treatment for young adults with symptomatic hip dysplasia and no osteoarthritis. In symptomatic dysplasia of the hip, there is labral pathology in up to 90% of cases. However, no consensus exists as to whether a labral tear should...

  16. 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.

  17. 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...

  18. The Possible Relationship Between Mammary Dysplasia and Breast ...

    African Journals Online (AJOL)

    Aim: There is need to resolve the continuing difficult question regarding the possible relationship between mammary dysplasia and breast cancer. Method: This is a 30-year study of the incidences of both mammary dysplasia and breast cancer occurring among the Igbos, a major ethnic group in Nigeria, West Africa. Results: ...

  19. 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...

  20. 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.

  1. 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.

  2. FLORID CEMENTO-OSSEOUS DYSPLASIA: A REPORT OF TWO ...

    African Journals Online (AJOL)

    known to originate from periodontal ligament tissues and involve essentially the same pathological process.1. They are usually classified depending on their topography and radiographic appearances, into three main groups: periapical, focal and florid cemental dysplasias.1. Florid cemento-osseous dysplasia was first ...

  3. 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.

  4. Renal dysplasia and MRI: a clinician's perspective

    Energy Technology Data Exchange (ETDEWEB)

    Greenbaum, Larry A. [Emory University, Division of Pediatric Nephrology, Children' s Healthcare of Atlanta, Atlanta, GA (United States)

    2008-01-15

    Renal dysplasia is a common abnormality in children. The role of MRI in evaluating children with renal dysplasia is evolving. More information is clearly necessary before MRI replaces conventional imaging modalities. In order to appropriately use MRI, the radiologist must have an understanding of the clinical questions that are important in the management of children with renal dysplasia. This review provides background information on renal dysplasia for the pediatric radiologist. The focus is on unilateral disease, especially multicystic dysplastic kidneys, and bilateral dysplasia, which is the most common cause of kidney failure in children. The emphasis is on the important clinical issues, and the potential of MRI as a methodology for providing clinically useful information not otherwise available from other imaging modalities. (orig.)

  5. Cardiac sarcoidosis mimicking arrhythmogenic right ventricular dysplasia

    Science.gov (United States)

    Steger, Christina Maria; Hager, Thomas; Antretter, Herwig; Hoyer, Hans Xaver; Altenberger, Johann; Pölzl, Gerhard; Müller, Ludwig; Höfer, Daniel

    2009-01-01

    Isolated manifestation of sarcoidosis in the heart is very rare. The present work describes the case of a 41-year-old woman with ventricular tachycardia and severe symptoms of heart failure in June 2006. Clinical, MRI and echocardiographic findings revealed the diagnosis of an arrhythmogenic right ventricular dysplasia. Due to the severe progression of the disease, cardiac transplantation was performed in August 2007. Histopathological examination of the explanted heart, however, revealed numerous non-necrotising granulomas with giant cells, lymphocytic infiltration and interstitial fibrosis, finally confirming the diagnosis of a myocardial sarcoidosis. PMID:22096465

  6. Dyssegmental dysplasia in siblings: Prenatal ultrasonic diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Andersen, P.E. Jr.; Hauge, M.; Bang, J.

    1988-01-01

    Two cases of dyssegmental dysplasia (type Silverman-Handmaker) in siblings are presented. The first-born died at the age of 3 months and the second fetus was followed during pregnancy with ultrasound examinations. In the 20th week of gestation marked shortening of the extremities was found; a female infant showing the same radiologic bony malformations as the firstborn was born by cesarean section. These cases support the autosomal recessive inheritance and demonstrate the possibility of prenatal diagnosis in this type of micromelic dwarfism. (orig.)

  7. Grading oral epithelial dysplasia: analysis of individual features.

    Science.gov (United States)

    Tilakaratne, W M; Sherriff, M; Morgan, P R; Odell, E W

    2011-08-01

    Assessing epithelial dysplasia to predict malignant transformation remains problematic in many tissues because grading systems are poorly structured and individual features poorly defined. Dysplasia grading is criticised for lack of reproducibility and poor predictive value. Grading systems for upper aerodigestive tract dysplasia have evolved over several decades and are not supported by good outcome experimental data.  This study analysed the individual features of dysplasia in 86 oral dysplastic lesions and determined the reproducibility of scoring for each, and correlated them with other features and clinical factors using complex clustering analyses. A uniform pattern of dysplasia was found in 37 lesions, focal dysplasia in 36 and in 13 lesions dysplasia formed complex discontinuous patterns. There was wide variation in reproducibility of scoring of individual features and many, including thickness, some types of rete morphology, basaloid cell anisonucleosis, basal dyscohesion, and dyskeratosis as deep single cells correlated with sub-sites. Rete morphology, type of keratinisation, hyperchromatism of the basaloid compartment, prickle cell anisonucleosis and extension down salivary ducts correlated with smoking. Conventional grading and oral intraepithelial neoplasia (OIN) grading by 'thirds affected' showed strong correlation overall but scores obtained with the OIN system tended to a higher grade at all sites except soft palate/fauces. There was poor correlation between the systems for moderate dysplasia and also severe dysplasia at some sites. Individual features could not be shown to cluster to form distinct patterns of dysplasia. These variations may account in part for the lack of reproducibility and poor predictive value of the grading systems in current use and could inform the design of future grading systems. © 2011 John Wiley & Sons A/S.

  8. 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

  9. DNA Fingerprinting Abnormalities Can Distinguish Ulcerative Colitis Patients with Dysplasia and Cancer from Those Who Are Dysplasia/Cancer-Free

    Science.gov (United States)

    Chen, Ru; Rabinovitch, Peter S.; Crispin, David A.; Emond, Mary J.; Koprowicz, Kent M.; Bronner, Mary P.; Brentnall, Teresa A.

    2003-01-01

    Patients with extensive ulcerative colitis (UC) of longer than 8 years duration are at high risk for the development of colorectal cancer. The cancers in these patients appear to develop in a stepwise manner with progressive histological changes from negative for dysplasia → indefinite for dysplasiadysplasia → cancer. The aim of this study was to determine the timing and extent of genomic instability in the progression of UC dysplasia and cancer. Using two polymerase chain reaction (PCR)-based DNA fingerprinting methods, arbitrarily primed PCR and intersimple sequence repeat PCR, we assessed DNA sequence variation in biopsies across the spectrum of cancerous, dysplastic, and nondysplastic mucosa. UC patients with dysplasia/cancer had substantial genomic instability in both their dysplastic and nondysplastic colonic mucosa, whereas instability was not present in the majority of UC patients without dysplasia/cancer. The degree of instability in nondysplastic tissue was similar to that of dysplastic/cancerous mucosa from the same patient, suggesting that this instability was widespread and reached the maximum level early in neoplastic progression. These results suggest that UC patients who develop dysplasia or cancer have an underlying process of genomic instability in their colonic mucosa whereas UC patients who are dysplasia-free do not. PMID:12547724

  10. Outcome of surgical management of developmental dysplasia of hip in children between 18 and 24 months.

    Science.gov (United States)

    Ramani, Narasimhan; Patil, Mithun S; Mahna, Madhur

    2014-09-01

    Developmental dysplasia of hip (DDH) is a common condition presenting to a pediatric orthopedic surgeon. There is a consensus on the surgical treatment of children with ages ranged from 18 to 24 months where majority agree on open reduction and hip spica. Open reduction was done with an additional pelvic procedure wherever required to get better results and prevent residual acetabular dysplasia (RAD) and early osteoarthritis. 35 children with unilateral DDH were operated between 2002 and 2007 at our institute. Open reduction was performed in all using the standard anterior approach and peroperative test for hip stability was done. Nine children got an additional pelvic procedure in the form of Dega acetabuloplasty. All were followed up for a minimal period of 2 years (range 2-7 years). No hip got redislocated. At the end of 18 months, there were seven cases of RAD with acetabular index (AI) of 35° and above. These were all from the group where open reduction alone was done. We feel that a preoperative AI of >40° and a per-operative safe-zone pelvic osteotomy in age group of 18 to 24 months because in such cases, the remodeling capacity of the acetabulum is unable to overcome the dysplasia and to form a relatively normal acetabulum.

  11. 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%.

  12. Morphological and immunohistochemical pattern of tubo-ovarian dysplasia and serous tubal intraepithelial carcinoma.

    Science.gov (United States)

    Chene, Gautier; Cayre, Anne; Raoelfils, Ines; Lagarde, Nicole; Dauplat, Jacques; Penault-Llorca, Frederique

    2014-12-01

    Histopathological examination of material from prophylactic salpingo-oophorectomies performed in patients at genetic risk of ovarian cancer can reveal abnormalities interpreted as possible pre-cancerous "ovarian dysplasia" and tubal precursors lesions. We sought to study the morphological features and immunohistochemical expression patterns of neoplasia-associated markers in prophylactically removed ovaries and fallopian tubes (pBSO) in comparison with a group of serous tubal intraepithelial carcinoma (STIC) and non-cancerous controls. Morphological features and immunohistochemical expression patterns of Ki-67 (for proliferation biomarker), p53 (key pathway of mullerian serous tumorogenesis), Bcl2 (anti-apoptotic), γH2AX (a double-strand breaks marker) and ALDH1 (a stem cell marker significantly associated with early-stage ovarian cancer) were blindly evaluated by two pathologists in 111 pBSO, 12 STICs and 116 non-cancerous salpingo-oophorectomies (control group) (nBSO). Morphological ovarian and tubal dysplasia scores were significantly higher in the pBSO than in controls (respectively, 8.8 vs 3.12, pSTICs compared with the controls whereas expression patterns of Ki67, p53 and bcl2 were low to moderate in the pBSO group. STICs overexpressed Ki67 and p53 while bcl2 expression was low; Interestingly, ALDH1 expression was low in non dysplastic epithelium, high in dysplasia and constantly low in STICs. The morphological and immunohistochemical profile of tubo-ovarian dysplasia and STICs might be consistent with progression toward neoplastic transformation in the Serous Carcinogenesis Sequence. These changes may be pre-malignant and could represent an important phase in early neoplasia. ALDH1 activation in pBSO samples and its extinction in STICs should be considered as a target for prevention. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  13. 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.

  14. Arrhythmogenic right ventricular dysplasia: MRI findings

    International Nuclear Information System (INIS)

    Wall, E.E. van der; Bootsma, M.M.; Schalij, M.J.; Kayser, H.W.M.; Roos, A. de

    2000-01-01

    Arrhythmogenic right ventricular dysplasia (ARVD) is a heart muscle disorder of unknown cause that is characterized pathologically by fibrofatty replacement of the right ventricular myocardium. Clinical manifestations include structural and functional malformations of the right ventricle, electrocardiographic abnormalities, and presentation with ventricular tachycardias with left bundle branch pattern or sudden death. The disease is often familial with an autosomal inheritance. In addition to right ventricular dilatation, right ventricular aneurysms are typical deformities of ARVD and they are distributed in the so-called ''triangle of dysplasia'', i. e., right ventricular outflow tract, apex, and infundibulum. Ventricular aneurysms at these sites can be considered pathognomonic of ARVD. Another typical hallmark of ARVD is fibrofatty infiltration of the right ventricular free wall. These functional and morphologic characteristics are relevant to clinical imaging investigations such as contrast angiography, echocardiography, radionuclide angiography, ultrafast computed tomography, and magnetic resonance imaging (MRI). Among these techniques, MRI allows the clearest visualization of the heart, in particular because the right ventricle is involved, which is usually more difficult to explore with the other imaging modalities. Furthermore, MRI offers the specific advantage of visualizing adipose infiltration as a bright signal of the right ventricular myocardium. MRI provides the most important anatomic, functional, and morphologic criteria for diagnosis of ARVD within one single study. As a result, MRI appears to be the optimal imaging technique for detecting and following patients with clinical suspicion of ARVD. (orig.) [de

  15. Mondini dysplasia and congenital cytomegalovirus infection.

    Science.gov (United States)

    Bauman, N M; Kirby-Keyser, L J; Dolan, K D; Wexler, D; Gantz, B J; McCabe, B F; Bale, J F

    1994-01-01

    We report a case of bilateral temporal bone anomalies in a child with symptomatic congenital cytomegalovirus infection and severe, bilateral sensorineural hearing loss identified at 3 months of age. High-resolution temporal bone computed tomography (HRCT) revealed bilateral findings of a short, malformed cochlea lacking an interscalar septum, a short and wide internal auditory canal, and an enlarged vestibular aqueduct, features diagnostic of bilateral Mondini dysplasia. To determine the importance of this observation, we completed HRCT in five additional children between 7 months and 9 years of age who had evidence of symptomatic congenital cytomegalovirus infection. One child with profound sensorineural hearing loss had severe bilateral temporal bone dysplasia with a small cochlea lacking an interscalar septum, an abnormal vestibule, and a large cochlear aqueduct. Of the remaining four children, hearing thresholds ranged from normal to profoundly decreased, but their HRCT scans were normal to visual inspection. When inner ear dimensions of these temporal bones were compared with norms established by Pappas and coworkers, however, seven of the eight ears had short cochleas and narrow lateral semicircular canals, and three ears had short or narrow vestibules. These results indicate that congenital cytomegalovirus infection may cause anomalies or growth disturbances of the temporal bone.

  16. General ultrasound screening reduces the rate of first operative procedures for developmental dysplasia of the hip: a case-control study.

    Science.gov (United States)

    von Kries, Rüdiger; Ihme, Nicola; Altenhofen, Lutz; Niethard, Fritz Uwe; Krauspe, Rüdiger; Rückinger, Simon

    2012-02-01

    To assess the effectiveness of general ultrasound screening to prevent first operative procedures of the hip. We conducted a case-control study in a population in which general ultrasound screening supplementing clinical screening is recommended and offered free of charge for all children. Participation in ultrasound screening before week 7 as recommended in Germany was the exposure of interest. Case ascertainment was based on active surveillance in orthopedic hospitals. The case definition was: first operative procedure for developmental dysplasia of the hip (closed reduction, open reduction, or osteotomy) in children >9 weeks old and developmental dysplasia of the hip (n = 446) were compared with 1173 control subjects for ultrasound screening. Effectiveness of ultrasound screening to prevent first operative procedures for developmental dysplasia of the hip was estimated as 52% (95% CI, 32-67). Effectiveness did not vary substantially for closed and open reductions and osteotomy. General ultrasound screening reduces the rate of operative procedures for developmental dysplasia of the hip; the impact on developmental dysplasia of the hip. Treatment rates and avascular necrosis need further assessment to balance the benefit against potential overtreatment and adverse effects. Copyright © 2012 Mosby, Inc. All rights reserved.

  17. Clinical significance and molecular characterization of nonsporulating molds isolated from the respiratory tracts of bronchopulmonary mycosis patients with special reference to basidiomycetes.

    Science.gov (United States)

    Singh, Pradeep Kumar; Kathuria, Shallu; Agarwal, Kshitij; Gaur, Shailendra Nath; Meis, Jacques F; Chowdhary, Anuradha

    2013-10-01

    Nonsporulating molds (NSMs), especially basidiomycetes, have predominantly been reported as human pathogens responsible for allergic and invasive disease. Their conventional identification is problematic, as many isolates remain sterile in culture. Thus, inconclusive culture reports might adversely affect treatment decisions. The clinical significance of NSMs in pulmonary mycoses is poorly understood. We sequenced the internal transcribed spacer (ITS) region and D1/D2 domain of the larger subunit (LSU) of 52 NSMs isolated from respiratory specimens. The basidiomycetes were the predominant NSMs, of which Schizophyllum commune was the most common agent in allergic bronchopulmonary mycosis (ABPM), followed by Ceriporia lacerata in invasive fungal disease. Porostereum spadiceum, Phanaerochaete stereoides, Neosartorya fischeri, and Marasmiellus palmivorus were the other molds observed. Application of ITS and LSU region sequencing identified 92% of the isolates. The antifungal susceptibility data revealed that all basidiomycetes tested were susceptible to amphotericin B and resistant to caspofungin, fluconazole, and flucytosine. Except for 3 isolates of S. commune and a solitary isolate of M. palmivorus, all basidiomycetes had low MICs for itraconazole, posaconazole, and voriconazole. Basidiomycetes were isolated from patients with ABPM, invasive pulmonary mycosis/pneumonia, or fungal balls. In addition, the majority of the basidiomycetes were isolated from patients with chronic respiratory disorders who were sensitized to one of the basidiomycetous fungi and demonstrated precipitating antibodies against the incriminating fungi, indicating an indolent tissue reaction. Thus, isolation of basidiomycetes from the lower respiratory tract could be significant, and it is important to monitor these patients in order to prevent subsequent lung damage.

  18. Relationship between flexible flat foot and developmental hip dysplasia.

    Science.gov (United States)

    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.

  19. Focal cemento-osseous dysplasia masquerading as a residual cyst

    Directory of Open Access Journals (Sweden)

    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.

  20. Bilateral Cerebellar Cortical Dysplasia without Other Malformations: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    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

  1. [Protein-energy malnutrition in patients with connective tissue dysplasia].

    Science.gov (United States)

    Lialiukova, E A

    2013-01-01

    In the conditions of the specialized Center of a dysplasia of a connecting tissue the assessment of an protein--energy malnutrition at 121 patients with signs of a dysplasia of a connecting tissue is carried out. High frequency of an oligotrophy at patients with a dysplasia of a connecting tissue is registered. The I degree of a gipotorofiya is taped at 26.21% of the patients, II degree--at 18.44%, the III degree--at 3.88% of patients.

  2. 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

  3. Emerging insights into the genetic basis of canine hip dysplasia

    Directory of Open Access Journals (Sweden)

    Ginja M

    2015-05-01

    Full Text Available Mário Ginja,1 Ana Rita Gaspar,1 Catarina Ginja,2,3 1Department of Veterinary Sciences-CITAB, University of Trás-os-Montes and Alto Douro, Vila Real, Portugal; 2Ce3C – Centro de Ecologia, Evolução e Alterações Ambientais, Faculdade de Ciências, Universidade de Lisboa, Lisboa, Portugal; 3CIBIO-InBIO – Centro de Investigação em Biodiversidade e Recursos Genéticos, Universidade do Porto, Vairão, Portugal Abstract: Canine hip dysplasia (CHD is the most common inherited polygenic orthopedic trait in dogs with the phenotype influenced also by environmental factors. This trait was described in the dog in 1935 and leads to a debilitating secondary hip osteoarthritis. The diagnosis is confirmed radiographically by evaluating signs of degenerative joint disease, incongruence, and/or passive hip joint laxity. There is no ideal medical or surgical treatment so prevention based on controlled breeding is the optimal approach. The definitive CHD diagnosis based on radiographic examination involves the exposure to ionizing radiation under general anesthesia or heavy sedation but the image does not reveal the underlying genetic quality of the dog. Phenotypic expression of CHD is modified by environmental factors and dogs with a normal phenotype can be carriers of some mutations and transmit these genes to their offspring. Programs based on selection of dogs with better individual phenotypes for breeding are effective when strictly applied but remain inferior to the selection of dogs based on estimation of breeding values. Molecular studies for dissecting the genetic basis of CHD are ongoing, but progress has been slow. In the future, the recommended method to improve hip quality in controlled breeding schemes, which will allow higher selection pressure, would be based on the estimation of the genomic breeding value. Since 2012, a commercial DNA test has been available for Labrador Retrievers using a blood sample and provides a probability for

  4. Nonfamilial cleidocranial dysplasia (dysostosis): a case report

    International Nuclear Information System (INIS)

    Salem, G

    1990-01-01

    Cleidocranial dysplasia, previously known as cleidocranial dysostosis, is a rare hereditary disease of unknown etiology characterized by abnormalities in the skull, jaws, shoulder girdle, as well as abnormalities of the dentition. The disease usually follows an autosomal dominant mode of transmission. This is a report on a case in a 25-year-old Saudi female from Gizan, Saudi Arabia, which seemes to be the first case reported in the Kingdom. An outstanding feature of this case is that it did not follow a familial pattern of inheritance since the patient is the only member of the family suffering from such disorder. The abnor malities present in the dentition are described together with the associated skeletal malformations. The clin ical and radiographic findings, as well as the hereditary pattern of the disease as described in the literature, are discussed. The dental management of these cases is reviewed. (author)

  5. Guidelines for genetic skeletal dysplasias for pediatricians

    Directory of Open Access Journals (Sweden)

    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.

  6. Four cases of right ventricular dysplasia

    International Nuclear Information System (INIS)

    Takamura, Ichiro; Ando, Joji; Miyamoto, Atsushi; Kobayashi, Takeshi; Sakamoto, Sanya; Yasuda, Hisakazu

    1985-01-01

    Finding of 81 Kr right ventriculography and 201 Tl myocardial perfusion imaging in 4 patients with right ventricular dysplasia (RVD) were compared with those in 28 patients with dilated cardiomyopathy. Remarkably dilated right ventricle was detected on 201 Tl myocardial perfusion imaging in the RVD group. In a patient with RVD who died suddenly, perfusion defect of the left ventricular myocardium, a decreased right ventricular ejection fraction, and an increased right ventricular end diastolic volume were seen. Perfusion defect of the left ventricular myocardium was seen in 10 of the 28 patients with dilated cardiomyopathy, 4 of whom died suddenly. In these 4 patients, a decreased left ventricular ejection fraction and an increased right ventricular end diastolic volume were seen. These findings obtained by the radionuclide techniques suggested that there are differences in cardiac dysfunction of the both ventricles between the groups with RVD and dilated cardiomyopathy. (Namekawa, K.)

  7. Cystic thymoma coexisting with vascular dysplasia.

    Science.gov (United States)

    Papadas, T; Dimopoulos, P A; Sampsonas, F; Mastronikolis, N; Spiropoulos, K; Goumas, P D

    2008-01-01

    We report the case of a 23-year-old female patient, who was admitted to our hospital because of aggravating pain in the right shoulder, right chest and ear accompanied by cough and dyspnea. Imaging revealed a soft tissue mass in the mediastinum in close relation with an additional large mass extending in the right upper thorax, right axilla and neck. The tissue obtained during surgery showed the former mass to be a cystic thymoma, being in contact with an extensive vascular dysplasia. The characteristic clinical presentation, the contribution of imaging, the results of operation, and the follow-up are rather unique. The review of the literature did not reveal similar cases.

  8. Hypohidrotic ectodermal dysplasia: a multidisciplinary approach.

    Science.gov (United States)

    Bildik, Tezan; Ozbaran, Burcu; Kose, Sezen; Koturoglu, Guldane; Gokce, Bulent; Gunaydin, Asli; Altintas, Inci

    2012-01-01

    Hypohidrotic ectodermal dysplasia (HED; Christ-Siemens-Touraine syndrome) is a genetic disorder characterized by sparse hair, oligodontia with peg-shaped teeth, reduced sweating, and defects in a number of other ectodermal organs. A partial or complete absence of eccrine glands can lead to recurrent severe overheating that may cause seizures and neurological deficits. This clinical report presents a 14-year-old male patient with hypohidrotic ectodermal dysplasia, including the clinical and radiographic findings, and multidisciplinary treatment. The Schedule for Affective Disorders and Schizophrenia for School Aged Children-Present and Lifetime Version (K-SADS-PL) was administered to patient along with mother to assess for any psychiatric disorders. The screening and rating scales completed by mother and two teachers to evaluate the severity of attention deficit hyperactivity disorder (ADHD) symptoms, and other behavioral problems. Patient's academic performance, adaptive functioning, and problem behavior was evaluated using. The Teacher Report Form. Mental capacity was assessed with the Wechsler Intelligence Scale for Children (WISC-R). Illness Perception Questionnaire Revised was used to systematically assess illness representation attributes and emotional representations of illness. On the psychiatric diagnosis assessment using K-SADS-PL sub-threshold attention deficits and anxiety symptoms were determined. In this case we established a multidisciplinary approach in his treatment with pediatric, dermatological, and dental examinations, beside his psychiatric evaluation. The prosthetic rehabilitation included restoring upper teeth with copings and fabrication of upper and lower complete dentures. Metal framework was not incorporated in the partial denture design allowing modifications as the oral and maxillofacial development continued. Removable complete or partial dentures without metal framework is a treatment of choice until the completion of facial growth at

  9. The Epidemiology and Demographics of Hip Dysplasia

    Science.gov (United States)

    Loder, Randall T.; Skopelja, Elaine N.

    2011-01-01

    The etiology of developmental dysplasia of the hip (DDH) is unknown. There are many insights, however, from epidemiologic/demographic information. A systematic medical literature review regarding DDH was performed. There is a predominance of left-sided (64.0%) and unilateral disease (63.4%). The incidence per 1000 live births ranges from 0.06 in Africans in Africa to 76.1 in Native Americans. There is significant variability in incidence within each racial group by geographic location. The incidence of clinical neonatal hip instability at birth ranges from 0.4 in Africans to 61.7 in Polish Caucasians. Predictors of DDH are breech presentation, positive family history, and gender (female). Children born premature, with low birth weights, or to multifetal pregnancies are somewhat protected from DDH. Certain HLA A, B, and D types demonstrate an increase in DDH. Chromosome 17q21 is strongly associated with DDH. Ligamentous laxity and abnormalities in collagen metabolism, estrogen metabolism, and pregnancy-associated pelvic instability are well-described associations with DDH. Many studies demonstrate an increase of DDH in the winter, both in the northern and southern hemispheres. Swaddling is strongly associated with DDH. Amniocentesis, premature labor, and massive radiation exposure may increase the risk of DDH. Associated conditions are congenital muscular torticollis and congenital foot deformities. The opposite hip is frequently abnormal when using rigorous radiographic assessments. The role of acetabular dysplasia and adult hip osteoarthritis is complex. Archeological studies demonstrate that the epidemiology of DDH may be changing. PMID:24977057

  10. Prevention of respiratory syncytial virus infections Prevenção das infecções pelo vírus sincicial respiratório

    Directory of Open Access Journals (Sweden)

    Lucia Ferro Bricks

    2001-06-01

    Full Text Available Respiratory syncytial virus is the most important cause of viral lower respiratory illness in infants and children worldwide. By the age of 2 years, nearly every child has become infected with respiratory syncytial virus and re-infections are common throughout life. Most infections are mild and can be managed at home, but this virus causes serious diseases in preterm children, especially those with bronchopulmonary dysplasia. Respiratory syncytial virus has also been recognized as an important pathogen in people with immunossupressive and other underlying medical problems and institutionalizated elderly, causing thousands of hospitalizations and deaths every year. The burden of these infections makes the development of vaccines for respiratory syncytial virus highly desirable, but the insuccess of a respiratory syncytial virus formalin-inactivated vaccine hampered the progress in this field. To date, there is no vaccine available for preventing respiratory syncytial virus infections, however, in the last years, there has been much progress in the understanding of immunology and immunopathologic mechanisms of respiratory syncytial virus diseases, which has allowed the development of new strategies for passive and active prophylaxis. In this article, the author presents a review about novel approaches to the prevention of respiratory syncytial virus infections, such as: passive immunization with human polyclonal intravenous immune globulin and humanized monoclonal antibodies (both already licensed for use in premature infants and children with bronchopulmonary dysplasia, and many different vaccines that are potential candidates for active immunization against respiratory syncytial virus.Em todo o mundo, o vírus sincicial respiratório é o principal agente de infecções agudas das vias aéreas baixas em lactentes jovens e crianças. Aos dois anos de idade, praticamente todas as crianças já foram infectadas, e as reinfeções são comuns

  11. Management of Alveolar Proteinosis by Bronchopulmonary lavage under Extra Corporeal Membrane Oxygenation (ECMO

    Directory of Open Access Journals (Sweden)

    Tahereh Parsa

    2015-12-01

    Full Text Available Normal 0 false false false EN-US X-NONE AR-SA The gold standard of treating Pulmonary Alveolar Proteinosis (PAP is bronchopulmonary lavage (BPL. We describe a rare case of BPD for PAP, who underwent ECMO due to hypoventilation in the setting of one lung ventilation. First, the clinical course of the patient is presented; furthermore, the biomolcular basis of PAP and new treatment approaches is discussed. /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:""; mso-padding-alt:0cm 5.4pt 0cm 5.4pt; mso-para-margin-top:0cm; mso-para-margin-right:0cm; mso-para-margin-bottom:10.0pt; mso-para-margin-left:0cm; line-height:115%; mso-pagination:widow-orphan; font-size:10.0pt; font-family:"Times New Roman","serif"; mso-fareast-font-family:Calibri; mso-bidi-font-family:"B Nazanin";} Normal 0 false false false EN-US X-NONE AR-SA  

  12. Beneficial effects of Omalizumab therapy in allergic bronchopulmonary aspergillosis: A synthesis review of published literature.

    Science.gov (United States)

    Li, Jian-Xiong; Fan, Li-Chao; Li, Man-Hui; Cao, Wei-Jun; Xu, Jin-Fu

    2017-01-01

    Omalizumab, a humanized mAb that binds to IgE, has been an effective therapy for patients with severe allergic asthma; however, there are few clinical trials examining the efficacy of Omalizumab in patients with allergic bronchopulmonary aspergillosis (ABPA) except some case reports. To assess the clinical and immunological effects of Omalizumab in ABPA patients, we made a synthesis review of 102 cases from 30 published literature, analyzed the effects of Omalizumab therapy in ABPA and conducted subgroup analyses to determine factors that influenced the therapy endpoints. We found that Omalizumab treatment not only provided a clinically important reduction in serum IgE, exacerbation rates and steroid requirement, but also showed attenuated asthma symptoms and improved pulmonary function parameters in patients with ABPA. Moreover, further discussion was made when interpretating the results. Double-blind, randomized, placebo-controlled trials are necessary to establish the efficacy and safety of this novel therapeutic intervention for ABPA patients. Copyright © 2016 Elsevier Ltd. All rights reserved.

  13. Two year follow-up of a garbage collector with allergic bronchopulmonary aspergillosis (ABPA).

    Science.gov (United States)

    Allmers, H; Huber, H; Baur, X

    2000-04-01

    Separate collection of biodegradable garbage and recyclable waste is expected to become mandatory in some western countries. A growing number of persons engaged in garbage collection and separation might become endangered by high loads of bacteria and fungi. Case history and examination A 29 year old garbage collector involved in emptying so-called biological garbage complained of dyspnea, fever, and flu-like symptoms during work beginning in the summer of 1992. Chest x-ray showed streaky shadows near both hili reaching into the upper regions. IgE- and IgG-antibodies (CAP, Pharmacia, Sweden) were strongly positive for Aspergillus fumigatus with 90.5 kU/L and 186%, respectively. Total-IgE was also strongly elevated with 5430 kU/L. Bronchial challenge testing with commercially available Aspergillus fumigatus extract resulted in an immediate-type asthmatic reaction. Two years later he was still symptomatic and antibodies persisted at lower levels. Our diagnosis was allergic bronchopulmonary aspergillosis (ABPA) including asthmatic responses as well as hypersensitivity pneumonitis (extrinsic allergic alveolitis) due to exposure to moldy household waste. A growing number of persons engaged in garbage collection and handling are exposed and at risk to develop sensitization to fungi due to exposure to dust of biodegradable waste. Further studies are necessary to show if separate collection of biodegradable waste increases the health risks due to exposure to bacteria and fungi in comparison to waste collection without separation. Copyright 2000 Wiley-Liss, Inc.

  14. Effectiveness of ultrasound screening for developmental dysplasia of the hip

    NARCIS (Netherlands)

    Roovers, E.A.; Boere-Boonekamp, M.M.; Castelein, R.M.; Zielhuis, G.A.; Kerkhoff, T.H.

    2005-01-01

    OBJECTIVE: To determine the effectiveness of ultrasound screening for developmental dysplasia of the hip (DDH) after the neonatal period. DESIGN: Prospective cohort study. SETTING: Child health care centres. PARTICIPANTS: Infants attending the child health care centres. INTERVENTIONS: The

  15. Effectiveness of ultrasound screening for developmental dysplasia of the hip

    NARCIS (Netherlands)

    Roovers, EA; Boere-Boonekamp, MM; Zielhuis, GA; Kerkhoff, TH

    Objective: To determine the effectiveness of ultrasound screening for developmental dysplasia of the hip (DDH) after the neonatal period. Design: Prospective cohort study. Setting: Child health care centres. Participants: Infants attending the child health care centres. Interventions: The

  16. Effectiveness of ultrasound screening for developmental dysplasia of the hip

    NARCIS (Netherlands)

    Roovers, E.A.; Boere-Boonekamp, Magdalena M.; Castelein, R.M.; Zielhuis, G.A.; Kerkhoff, Antoon

    2005-01-01

    Objective: To determine the effectiveness of ultrasound screening for developmental dysplasia of the hip (DDH) after the neonatal period. Design: Prospective cohort study. Setting: Child health care centres. Participants: Infants attending the child health care centres. Interventions: The

  17. Urethral dysontogenic metaplasia in cat with bilateral renal dysplasia

    Directory of Open Access Journals (Sweden)

    Carolina da Fonseca Sapin

    2017-10-01

    Full Text Available This paper to describe a case of dysontogenic urethral metaplasia in a one month old mongrel feline who also had bilateral renal dysplasia. Dysontogenic metaplasia in cats are scarce and this change may be associated with renal dysplasia and/or lower urinary tract. The animal had history of abdominal enlargement since birth and dysuria, eliminating urine only dropwise. Due to the poor prognosis we opted for euthanasia. At necropsy was observed enlarged and distended bladder, reduced kidneys and dilated and tortuous ureters. The urethra was thickened, hard to cut, and histologically, was replacing the connective tissue, cartilage and endochondral ossification areas, which features dysontogenic metaplasia. Both kidneys presented primitive appearance featuring dysplasia. Dysontogenic metaplasia in urinary tract feline with renal dysplasia, has not been described.

  18. Ehlers-Danlos syndrome with monostotic fibrous dysplasia

    Directory of Open Access Journals (Sweden)

    Rao A

    1979-01-01

    Full Text Available An unusual case of Ehlers-Danlos syndrome with monostotic fibrous dysplasia of the humorus is presented. The other orthopae-dic manifestations, its complications and associated features are re-viewed and summarised.

  19. Incidence of Canine Hip Dysplasia : A Survey of 272 Cases

    Directory of Open Access Journals (Sweden)

    G. D. Rao

    2010-10-01

    Full Text Available A total of 272 cases of hip dysplasia were reviewed. A review of clinical cases presented with the clinical signs of hip dysplasia were referred to Radiology Unit of Madras Veterinary College, from May 2007-April 2009 was taken for this study.The incidence was highest in young animals of age group over three months to one year (52.94 percent. The breed-wise incidence was more common in Labrador Retriever (36.76 percent. Male dogs were found to be more affected (59.55 percent than female dogs. Bilateral hip dysplasia was found to be more (88.60 percent than unilateral. Among the unilateral hip dysplasia, left side was found to be more (54.83 percent than right. [Vet. World 2010; 3(5.000: 219-220

  20. Genetics Home Reference: Schimke immuno-osseous dysplasia

    Science.gov (United States)

    ... Immune System and Disorders Health Topic: Kidney Failure Genetic and Rare Diseases Information Center (1 link) Schimke immunoosseous dysplasia Additional NIH Resources (2 links) National Institute of Allergy and Infectious Diseases: Disorders of the Immune System National Institute ...

  1. Genetics Home Reference: platyspondylic lethal skeletal dysplasia, Torrance type

    Science.gov (United States)

    ... cause platyspondylic lethal skeletal dysplasia, torrance type, and define a novel subfamily within the type 2 collagenopathies. ... for Links Data Files & API Site Map Subscribe Customer Support USA.gov Copyright Privacy Accessibility FOIA Viewers & ...

  2. Glenoid Dysplasia in the Recurrent Shoulder Dislocation: A Case Report

    Directory of Open Access Journals (Sweden)

    Hamza Sucuoglu

    2016-02-01

    Full Text Available Glenoid dysplasia, which is often ignored, is a rare developmental anomaly. In most cases the patients remain asymptomatic. Although glenoid dysplasia has been diagnosed by incidentally on chest radiograph, also it has been described because of the developing instability of shoulder joint. Our young male patient who has recurrent dislocation of the shoulder-joint 4-5 times a year for last 5 years, dislocation has been reduced in the emergency department and he has been discharged from hospital with shoulder strap and rest. Finally when he admitted to our outpatient clinic with recurrent shoulder dislocation, the diagnosis of glenoid dysplasia is revealed by X-ray examination. We present the diagnose of glenoid dysplasia with clinical and radiological findings which should be kept in mind in patients with recurrent dislocations of shoulder as in our case.

  3. Computed tomographic features of fibrous dysplasia of maxillofacial region

    OpenAIRE

    Sontakke, Subodh Arun; Karjodkar, Freny R; Umarji, Hemant R

    2011-01-01

    Purpose This study was to find the computed tomographic features of fibrous dysplasia of the maxillofacial region. Materials and Methods All eight cases included in the study reported either to Government Dental College and Hospital or Nair Hospital Dental College, Mumbai between 2003 and 2009. The patients were prescribed computed tomogram in addition to conventional radiographs of maxillofacial region which were studied for characteristic features of fibrous dysplasia. The diagnosis of fibr...

  4. Developmental Dysplasia of the Hip and Occult Neurologic Disorders

    OpenAIRE

    Luther, A. Z.; Clarke, N. M. P.

    2008-01-01

    Developmental dysplasia of the hip (DDH) is a neonatal condition with various causes. Neuromuscular dysplasia of the hip (NDH) is a sequel of neuromuscular disease, and generally presents later in childhood than DDH. Some evidence, however, supports a concept of a neuromuscular etiology of DDH: (1) a high prevalence of spinal dysraphism in DDH; and (2) abnormal sensory evoked potentials in 31% of DDH patients. To explore this suggestion we ascertained the presence of neuromuscular disease wit...

  5. Chondrosarcoma occurring in a patient with polyostotic fibrous dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    De Smet, A.A.; Travers, H.; Neff, J.R.

    1981-12-01

    A 36-year-old white man with polyostotic fibrous dysplasia was found to have a high-grade chondrosarcoma arising from the left ilium. Although a left hemipelvectomy was performed, the patient subsequently developed sacral and pulmonary metastases and succumbed to his disease. This patient represents the first documented example of an unequivocally high-grade chondrosarcoma arising in an area of fibrous dysplasia without prior irradiation.

  6. Ectrodactyly, ectodermal dysplasia, cleft lip, and palate (EEC syndrome

    Directory of Open Access Journals (Sweden)

    Mohita Marwaha

    2012-01-01

    Full Text Available The ectodermal dysplasias (EDs are a large and complex group of diseases. More than 170 different clinical conditions have been recognized and defined as ectodermal dysplasias. Commonly involved ectodermal-derived structures are hair, teeth, nails, and sweat glands. In some conditions, it may be associated with mental retardation. We report a case of 10-year-old male child with ectrodactyly, syndactyly, ED, cleft lip/palate, hearing loss, and mental retardation.

  7. The association between gender and familial prevalence of hip dysplasia in Danish patients

    DEFF Research Database (Denmark)

    El Jashi, Rima; Gustafson, Maria B; Jakobsen, Mette B

    2017-01-01

    , but this difference in risk was not statistically significant (p = 0.10). CONCLUSIONS: The study shows that females have 32% increased familial prevalence of hip dysplasia compared to males, but the increased prevalence was not statistically significant probably due to the low power of the study.......BACKGROUND: The development of hip dysplasia is associated with several risk factors. 1 of these risk factors is gender, since 80% of patients with symptomatic hip dysplasia are females. Another risk factor for hip dysplasia is familial predisposition of hip dysplasia. Several studies indicate...... that the risk of hip dysplasia is increased with familial prevalence of hip dysplasia. However, little is known about the association between the familial prevalence and gender and the development of hip dysplasia. PURPOSE: The aim of the study was to estimate the prevalence of hip dysplasia among relatives...

  8. Use of Chinese Medicine Reduces the Development of Cervical Cancer from Pap Smear-Diagnosed Cervical Dysplasia: A Case-Control Study.

    Science.gov (United States)

    Shen, Li-Ling; Muo, Chih-Hsin; Su, Shan-Yu; Morisky, Donald E

    2017-01-01

    The Pap test diagnosed cervical dysplasia, which could recover to normal or progress to cervical cancer (CC), is an early stage of cell abnormality before CC. This case-control study analyzed the differences in the risk to develop CC between Chinese medicine (CM) users and nonusers among women who had ever been diagnosed as having cervical dysplasia. A total of 750 CC patients with a cervical dysplasia history were collected between 1998 and 2011 from National Health Insurance Research Database, and controls were women with cervical dysplasia history but did not develop CC. Adjusted odds ratio (aOR) for developing CC was assessed using multivariable logistic regression after adjusting for age, urbanization of residence, and occupation. The proportion of using CM among CC patients was lower than that among CC nonpatients, with an aOR of 0.8. By analyzing the relationship between CC development and the frequency of CM usage, the trend test revealed a significant decreasing trend for developing CC among high-frequency CM users. Moreover, the most frequently used single herb high-frequency was Rheum palmatum (Da-Huang). The usage of CM might be an effective complementary method to prevent uterine cervix from progressing to CC after cervical dysplasia has occurred.

  9. Is There a Relationship between Ovarian Epithelial Dysplasia and Infertility?

    Directory of Open Access Journals (Sweden)

    Gautier Chene

    2012-01-01

    Full Text Available Aim. Ovarian epithelial dysplasia was initially described in material from prophylactic oophorectomies performed in patients at genetic risk of ovarian cancer. Similar histopathological abnormalities have been revealed after ovulation stimulation. Since infertility is also a risk factor for ovarian neoplasia, the aim of this study was to study the relationship between infertility and ovarian dysplasia. Methods. We blindly reviewed 127 histopathological slides of adnexectomies or ovarian cystectomies according to three groups—an exposed group to ovulation induction (n = 30, an infertile group without stimulation (n = 35, and a spontaneously fertile control group (n = 62—in order to design an eleven histopathological criteria scoring system. Results. The ovarian dysplasia score was significantly higher in exposed group whereas dysplasia score was low in infertile and control groups (resp., 8.21 in exposed group, 3.69 for infertile patients, and 3.62 for the controls. In the subgroup with refractory infertility there was a trend towards a more severe dysplasia score (8.53 in ovulation induction group and 5.1 in infertile group. Conclusion. These results raise questions as to the responsibility of drugs used to induce ovulation and/or infertility itself in the genesis of ovarian epithelial dysplasia.

  10. Assessment of hip dysplasia and osteoarthritis: Variability of different methods

    International Nuclear Information System (INIS)

    Troelsen, Anders; Elmengaard, Brian; Soeballe, Kjeld; Roemer, Lone; Kring, Soeren

    2010-01-01

    Background: Reliable assessment of hip dysplasia and osteoarthritis is crucial in young adults who may benefit from joint-preserving surgery. Purpose: To investigate the variability of different methods for diagnostic assessment of hip dysplasia and osteoarthritis. Material and Methods: By each of four observers, two assessments were done by vision and two by angle construction. For both methods, the intra- and interobserver variability of center-edge and acetabular index angle assessment were analyzed. The observers' ability to diagnose hip dysplasia and osteoarthritis were assessed. All measures were compared to those made on computed tomography scan. Results: Intra- and interobserver variability of angle assessment was less when angles were drawn compared with assessment by vision, and the observers' ability to diagnose hip dysplasia improved when angles were drawn. Assessment of osteoarthritis in general showed poor agreement with findings on computed tomography scan. Conclusion: We recommend that angles always should be drawn for assessment of hip dysplasia on pelvic radiographs. Given the inherent variability of diagnostic assessment of hip dysplasia, a computed tomography scan could be considered in patients with relevant hip symptoms and a center-edge angle between 20 deg and 30 deg. Osteoarthritis should be assessed by measuring the joint space width or by classifying the Toennis grade as either 0-1 or 2-3

  11. The Occurrence of Occult Acetabular Dysplasia in Relatives of Individuals With Developmental Dysplasia of the Hip.

    Science.gov (United States)

    Carroll, Kristen L; Schiffern, Alison N; Murray, Kathleen A; Stevenson, David A; Viskochil, David H; Toydemir, Reha; MacWilliams, Bruce A; Roach, James W

    2016-01-01

    This study sought to determine the hip pathology of family members of patients with developmental dysplasia of the hip (DDH). The authors evaluated 120 people from 19 families known to have at least 1 member with surgically treated DDH. Each individual's functional outcome scores and pelvic radiographs were assessed for hip symptoms or pathology. Using a genetic population database and a pediatric hospital patient population, 19 families with high rates of DDH were identified. All family members (n=120) underwent physical examination, radiographic assessment, and completion of outcome instruments [American Academy of Orthopedics (AAOS) Hip and Knee; Harris Hip Score (HHS); and Western Ontario and McMaster Universities Arthritis Index (WOMAC)]. The 120 subjects ranged from 1 to 84 years, 34 had orthopaedically treated DDH. Of the remaining 86 supposedly normal subjects, 23 (27%) had occult acetabular dysplasia (OAD) as defined by center edge angle (CEA) <20 and/or a Severin score of III or greater. Sixty percent of the 86 individuals were less than 30 years old, 74% of the OAD group were less than 30. Outcome scores of the treated DDH patients (AAOS, HHS, and WOMAC) were worse on the involved side regardless of age. Over age 30 individuals with OAD had statistically significant decreases in their AAOS Hip and Knee and WOMAC scores on the dysplastic side, but their HHS scores were not significantly different. Twenty-seven percent of first-degree and second-degree relatives of patients with DDH had unsuspected radiographic acetabular dysplasia in our study. Most of the subjects with OAD were younger than 30. After age 30, many of these patients developed symptoms. In families with a significant history of DDH, radiographic screening of siblings of patients with DDH to define OAD may be prudent. Level I—diagnostic study.

  12. Invasive treatment for carotid fibromuscular dysplasia

    Science.gov (United States)

    Maciejewski, Damian R.; Dzierwa, Karolina; Kabłak-Ziembicka, Anna; Michalski, Michał; Wójcik-Pędziwiatr, Magdalena; Brzychczy, Andrzej; Moczulski, Zbigniew; Żmudka, Krzysztof; Pieniążek, Piotr

    2015-01-01

    Introduction Fibromuscular dysplasia (FMD) is an infrequent non-inflamatory disease of unknown etiology that affects mainly medium-size arteries. The prevalence of FMD among patients scheduled for endovascular treatment of carotid artery stenosis is unknown. Aim To evaluate the prevalence and treatment options of carotid FMD in patients scheduled for carotid artery stenting (CAS). Material and methods Between Jan 2001 and Dec 2013, 2012 CAS procedures were performed in 1809 patients (66.1% men; age 65.3 ±8.4 years, 49.2% symptomatic). In case of FMD suspicion in Doppler-duplex ultrasound (DUS), computed tomography angiography was performed for aortic arch and extracranial and intracranial artery imaging. For invasive treatment of FMD carotid stenosis, balloon angioplasty was considered first. If the result of balloon angioplasty was not satisfactory (> 30% residual stenosis, dissection), stent placement was scheduled. All patients underwent follow-up DUS and neurological examination 3, 6 and 12 months after angioplasty, then annually. Results There were 7 (0.4%) (4 symptomatic) cases of FMD. The FMD group was younger (47.9 ±7.5 years vs. 67.2 ±8.9 years, p = 0.0001), with higher prevalence of women (71.4% vs. 32.7%, p = 0.0422), a higher rate of dissected lesions (57.1% vs. 4.6%, p = 0.0002) and less severe stenosis (73.4% vs. 83.9%, p = 0.0070) as compared to the non-FMD group. In the non-FMD group the prevalence of coronary artery disease was higher (65.1% vs. 14.3% in FMD group, p = 0.009). All FMD patients underwent successful carotid artery angioplasty with the use of neuroprotection devices. In 4 cases angioplasty was supported by stent implantation. Conclusions Fibromuscular dysplasia is rare among patients referred for CAS. In case of significant FMD carotid stenosis, it may be treated with balloon angioplasty (stent supported if necessary) with optimal immediate and long-term results. PMID:26161104

  13. Bronchopulmonary hygiene physical therapy for chronic obstructive pulmonary disease and bronchiectasis.

    Science.gov (United States)

    Jones, A P; Rowe, B H

    2000-01-01

    Bronchopulmonary hygiene physical therapy is a form of chest physical therapy including chest percussion and postural drainage to remove lung secretions. These are applied commonly to patients with both acute and chronic airway diseases. Despite controversies in the literature regarding its efficacy, it remains in use in a variety of clinical settings. The various forms of this therapy are labour intensive and need to be evaluated. The objective of this review was to assess the effects of bronchial hygiene physical therapy in people with chronic obstructive pulmonary disease and bronchiectasis. We searched the Cochrane Airways Group trials register and reference lists of articles up to July 1997. We also wrote to study authors. Randomised trials in which postural drainage, chest percussion, vibration, chest shaking, directed coughing or forced exhalation technique was compared to other drainage or breathing techniques, placebo or no treatment. Two reviewers applied the inclusion and exclusion criteria on masked publications independently. They assessed the trial quality independently. Only data from the first arm of crossover trials were included. The seven included trials involved six comparisons and a total of 126 people. The trials were small and not generally of high quality. The results could not be combined as trials addressed different patient groups and outcomes. In most comparisons, bronchial hygiene physical therapy produced no significant effects on pulmonary function, apart from clearing sputum in chronic obstructive pulmonary disease and in bronchiectasis. There is not enough evidence to support or refute the use of bronchial hygiene physical therapy in people with chronic obstructive pulmonary disease and bronchiectasis.

  14. WITHDRAWN: Bronchopulmonary hygiene physical therapy for chronic obstructive pulmonary disease and bronchiectasis.

    Science.gov (United States)

    Jones, Arthur P; Rowe, Brian H

    2011-07-06

    Bronchopulmonary hygiene physical therapy is a form of chest physical therapy including chest percussion and postural drainage to remove lung secretions. These are applied commonly to patients with both acute and chronic airway diseases. Despite controversies in the literature regarding its efficacy, it remains in use in a variety of clinical settings. The various forms of this therapy are labour intensive and need to be evaluated. The objective of this review was to assess the effects of bronchial hygiene physical therapy in people with chronic obstructive pulmonary disease and bronchiectasis. We searched the Cochrane Airways Group trials register and reference lists of articles up to January 2007. We also wrote to study authors. Randomised trials in which postural drainage, chest percussion, vibration, chest shaking, directed coughing or forced exhalation technique was compared to other drainage or breathing techniques, placebo or no treatment. Two reviewers applied the inclusion and exclusion criteria on masked publications independently. They assessed the trial quality independently. Only data from the first arm of crossover trials were included. The seven included trials involved six comparisons and a total of 126 people. The trials were small and not generally of high quality. The results could not be combined as trials addressed different patient groups and outcomes. In most comparisons, bronchial hygiene physical therapy produced no significant effects on pulmonary function, apart from clearing sputum in chronic obstructive pulmonary disease and in bronchiectasis. An update search carried out in January 2007 did not identify any new studies for inclusion. There is not enough evidence to support or refute the use of bronchial hygiene physical therapy in people with chronic obstructive pulmonary disease and bronchiectasis.

  15. Omalizumab in the treatment of allergic bronchopulmonary aspergillosis: One center's experience with 14 cases.

    Science.gov (United States)

    Aydın, Ömür; Sözener, Zeynep Çelebi; Soyyiğit, Şadan; Kendirlinan, Reşat; Gençtürk, Zeynep; Mısırlıgil, Zeynep; Mungan, Dilşad; Sin, Betül Ayşe; Demirel, Yavuz Selim; Çelik, Gülfem Elif; Bavbek, Sevim

    2015-01-01

    Omalizumab has been a valuable option for patients with severe allergic asthma, but there are only case reports regarding effectiveness of omalizumab in patients with allergic bronchopulmonary aspergillosis (ABPA). To evaluate the clinical and functional effectiveness of omalizumab in patients with asthma and ABPA in long-term follow-up. The study was conducted as a retrospective chart review of patients with ABPA who were treated with omalizumab injections between December 2008 and June 2014. Once treatment with omalizumab was started, data were collected at three time points: at baseline, after 1 year, and, in June 2014, at the last follow-up. Fourteen patients with ABPA (seven women and seven men; mean [± standard deviation (SD)] age, 44.21 ± 13.01 years) were included. The treatment period was 31.5 ± 3.99 months (mean ± SD). The difference between the baseline and the last evaluation of the mean percentage of forced expiratory volume in 1 second (FEV1) was significant (p = 0.02). The mean asthma control test score was increased at all-time points compared with the basal score (p = 0.001). After omalizumab treatment was initiated, the patients' mean oral corticosteroid dosage significantly decreased (p = 0.001). The baseline exacerbation rate was 2.7 ± 1.5/y (mean ± SD), and the hospitalization rate was 1.4/y, and both were zero at the last assessment (p = 0.001). Eleven of the patients (78.6%) responded perfectly, and three (21.4%) partially responded to treatment. The patients who had a total immunoglobulin E level of 1000 IU/mL (p = 0.05). Omalizumab provided a clinically important reduction in exacerbations and steroid requirement, and improved asthma symptoms and pulmonary function parameters in patients with asthma and ABPA who had previously shown an unsatisfactory response to Global Initiative for Asthma step 4 treatment.

  16. Clinical efficacy and immunologic effects of omalizumab in allergic bronchopulmonary aspergillosis.

    Science.gov (United States)

    Voskamp, Astrid L; Gillman, Andrew; Symons, Karen; Sandrini, Alessandra; Rolland, Jennifer M; O'Hehir, Robyn E; Douglass, Jo A

    2015-01-01

    Allergic bronchopulmonary aspergillosis (ABPA) often presents with persistently uncontrolled asthma despite the use of corticosteroids and antifungal therapy. Omalizumab is a humanized anti-IgE monoclonal antibody currently used to treat severe asthma. The aim was to assess the clinical and immunologic effects of omalizumab in ABPA in a randomized, placebo-controlled trial. Patients with chronic ABPA were randomized to 4-month treatment with omalizumab (750 mg monthly) or placebo followed by a 3-month washout period in a cross-over design. The main endpoint was number of exacerbations. Other clinical endpoints included lung function, exhaled nitric oxide (FeNO), quality of life and symptoms. In vitro basophil activation to Aspergillus fumigatus extract and basophil FcεR1 and surface-bound IgE levels were assessed by flow cytometry. Thirteen patients were recruited with mean total IgE 2314 ± 2125 IU/mL. Exacerbations occurred less frequently during the active treatment phase compared with the placebo period (2 vs 12 events, P = .048). Mean FeNO decreased from 30.5 to 17.1 ppb during omalizumab treatment (P = .03). Basophil sensitivity to A. fumigatus and surface-bound IgE and FcεR1 levels decreased significantly after omalizumab but not after placebo. Omalizumab can be used safely to treat ABPA, despite high serum IgE levels. Clinical improvement was accompanied by decreased basophil reactivity to A. fumigatus and FcεR1 and surface-bound IgE levels. Copyright © 2015 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  17. Case series of omalizumab for allergic bronchopulmonary aspergillosis in cystic fibrosis patients.

    Science.gov (United States)

    Nové-Josserand, Raphaële; Grard, Soazic; Auzou, Lila; Reix, Philippe; Murris-Espin, Marlène; Brémont, François; Mammar, Benyebka; Mely, Laurent; Hubert, Dominique; Durieu, Isabelle; Burgel, Pierre-Régis

    2017-02-01

    Allergic bronchopulmonary aspergillosis (ABPA) affects up to 15% of patients with cystic fibrosis (CF). Corticosteroids are used as first-line therapy, but relapse and adverse effects commonly occur. Case reports have suggested the efficacy of the anti-IgE recombinant humanized monoclonal antibody omalizumab. A retrospective multicenter observational French study retrieved 32 CF patients (11 children and 21 adults) who have received omalizumab for more than 3 months in the context of ABPA. Clinical characteristics, concomitant medications (inhaled and oral corticosteroids, antifungal drugs), lung function, body mass index (BMI), and serum IgE were compared at the start and during the first year of omalizumab therapy. Omalizumab-related adverse effects and costs were also evaluated. No significant difference with omalizumab could be demonstrated with regard to lung function, BMI, or the number of patients receiving oral corticosteroids. At the time of initiation of omalizumab, 56% of patients were receiving oral corticosteroids. Five patients were able to discontinue corticosteroids during follow-up and nine patients were able to reduce their daily dose. A total of 78% of the patients had received antifungal therapy at the time of the initiation of omalizumab. Treatment tolerance was good (12.5% of patients experienced side effects). The median cost of omalizumab treatment was €3,620 per patient per month. Omalizumab may represent a steroid-sparing therapy in CF patients with ABPA. A randomized-controlled trial is urgently required to provide higher level of evidence regarding the efficacy and cost-effectiveness of omalizumab in CF patients with ABPA. Pediatr Pulmonol. 2017;52:190-197. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  18. Increasing Total Serum IgE, Allergic Bronchopulmonary Aspergillosis, and Lung Function in Cystic Fibrosis.

    Science.gov (United States)

    Gothe, Florian; Kappler, Matthias; Griese, Matthias

    Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity disorder contributing to lung disease in cystic fibrosis (CF) and challenging to diagnose. This study analyzed the predictive value of increasing total IgE (t-IgE) levels in a CF cohort alongside with clinical and serologic data. A total of 387 children and young adults were followed from 2000 to 2006 and retrospectively classified into 6 groups. Patients with t-IgE levels IgE were classified as "Naïve," those with Af-specific IgE (Af-sIgE) as "Sensitized." Patients with elevated t-IgE at entrance and Af-sIgE were labeled "Former ABPA," and those without, as "High t-IgE." Patients whose t-IgE values started normal and exceeded the 95th percentile during the study were labeled either "ABPA at risk" if Af-sIgE-positive or "Rising t-IgE" if not. Courses of t-IgE over time were divided into episodes with increasing IgE (ΔIgE) and related to pulmonary outcome. A total of 125 patients were classified Naïve (32%), 64 Sensitized (17%), 49 ABPA at risk (13%), 32 Rising t-IgE (8%), 102 Former ABPA (26%), and 15 High t-IgE (4%). A total of 874 ΔIgE episodes were accompanied by forced expiratory volume in 1 second (FEV 1 ) declines (r = -0.21, P IgE levels at least doubled within 3 months and exceeded the 95th age-specific percentile (P IgE levels may be helpful in diagnosing treatment requiring ABPA and predicts the effect of systemic steroid treatment on pulmonary outcome. Copyright © 2017 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

  19. Allergic bronchopulmonary aspergillosis is associated with pet ownership in cystic fibrosis.

    Science.gov (United States)

    Thronicke, Anja; Heger, Nikola; Antweiler, Elisabeth; Krannich, Alexander; Roehmel, Jobst; Brandt, Claudia; Staab, Doris; Tintelnot, Kathrin; Schwarz, Carsten

    2016-09-01

    Late diagnosis of allergic bronchopulmonary aspergillosis (ABPA) is associated with significant lung function decline and morbidity in cystic fibrosis (CF). The association of ABPA and domestic pet ownership in patients with CF has not been elucidated yet. Our objective was to determine the association of ABPA with pet ownership in patients with CF. Clinical and microbiological data from certified local patient registry were analyzed for 109 patients with CF aged 1-64 years: 55 pet owner and 54 non-pet owners. The primary outcome of the retrospective observational study was the occurrence of ABPA in pet owners and non-pet owners with CF. The free statistical software R was utilized to investigate logistic regression models for association factors. Of the 109 patients included in the study, 61 (56%) were female. The mean age of the total group was 25.4 ± 13.2 years. Adjusted analysis revealed that ABPA (OR 5.0227, 95% CI: 1.182-21.340, p = 0.029) was associated with pet ownership in patients with CF. Furthermore, ABPA in pet owners with CF was associated with an increased number of exacerbations (OR 6.446, 95% CI: 1.057-39.328, p = 0.043). Other outcomes did not significantly differ. Owning a pet was associated with ABPA in patients with CF. Future prospective multicenter longitudinal studies are needed to investigate chronological causality between pet ownership, ABPA development, and pulmonary exacerbations and to determine whether these estimates are generalizable for ABPA susceptible patients beyond CF (asthma, bronchiectasis). © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  20. Bronchopulmonary complications associated to enteral nutrition devices in patients admitted to internal medicine departments.

    Science.gov (United States)

    Marco, J; Barba, R; Lázaro, M; Matía, P; Plaza, S; Canora, J; Zapatero, A

    2013-01-01

    Enteral nutrition using feeding devices such as nasogastric (NG) tube or percutaneous endoscopic gastrostomy (PEG) is an effective feeding method subject that may give rise to complications. We have studied the relationship between enteral nutrition feeding devices in patients admitted to the Internal Medicine Departments and the development of pulmonary complications (bronchial aspiration and aspiration pneumonia). All of the patients discharge between 2005 and 2009 from the Internal Medicine (IM) Departments of the public hospitals of the National Health System in Spain were analyzed. The data of patients with bronchial aspiration or aspiration pneumonia who also were carriers of NG tubes or PEG, were obtained from the Minimum Basic Data Set (MBDS). From a total of 2,767,259 discharges, 26,066 (0.92%) patients with nasogastric tube (NG tube) or percutaneous gastrostomy (PEG) were identified. A total of 21.5% of patients with NG tube and 25.9% of patients with PEG had coding for a bronchopulmonary aspiration on their discharge report versus 1.2% of patients without an enteral feeding tube. In the multivariate analysis, the likelihood of suffering bronchoaspiration was 9 times greater in patients with SNG (OR: 9.1; 95% CI: 8.7-9.4) and 15 greater in subjects with PEG (OR: 15.2; 95% CI: 14.5-15.9) than in subjects without SNG or PEG. Mean stay (9.2 and 12.7 more days), diagnostic complexity and costs were much higher in patients with SNG or PEG compared to patients in hospital who did not require these devices. An association was found between SNG and PEG for enteral feeding and pulmonary complications. Mean stay, diagnostic complexity and cost per admission of these patients was higher in patients who did not require enteral nutrition. Copyright © 2012 Elsevier España, S.L. All rights reserved.

  1. Prevalence and progression of pectinate ligament dysplasia in the Welsh springer spaniel.

    Science.gov (United States)

    Oliver, J A C; Ekiri, A; Mellersh, C S

    2016-08-01

    To determine the prevalence of pectinate ligament dysplasia in a large group of Welsh springer spaniels; to investigate associations between pectinate ligament dysplasia and age, sex and intraocular pressure and between intraocular pressure and age and sex; and to investigate progression of pectinate ligament dysplasia in individual dogs. In a prospective study, gonioscopy was performed in both eyes of 227 Welsh springer spaniels and intraocular pressure measured by rebound tonometry. Eyes were classified as "unaffected" if 0% of the iridocorneal angle was affected with pectinate ligament dysplasia (grade 0), "mildly affected" if 90% was affected (grade 3). In a retrospective study, progression of pectinate ligament dysplasia over time was investigated for 65 dogs. One hundred and thirty-nine of 227 dogs (61·2%) were affected by pectinate ligament dysplasia (grades 1 to 3) and 82/227 (36·2%) were moderately or severely affected. There was a significant association between pectinate ligament dysplasia and age. There were no associations between pectinate ligament dysplasia and intraocular pressure or pectinate ligament dysplasia and sex. Thirty-five of 65 dogs (53·8%) demonstrated progression of pectinate ligament dysplasia. Prevalence of pectinate ligament dysplasia was high despite widespread screening and selection against the condition. Our data indicate that gonioscopic features of pectinate ligament dysplasia can progress in the Welsh springer spaniel. Dogs deemed unaffected at an early age may subsequently be diagnosed with pectinate ligament dysplasia. © 2016 British Small Animal Veterinary Association.

  2. Maxillonasal dysplasia (Binder′s syndrome and its treatment with costal cartilage graft: A follow-up study

    Directory of Open Access Journals (Sweden)

    Bhatt Yogesh

    2008-01-01

    Full Text Available Maxillonasal dysplasia or Binder′s syndrome is an uncommon congenital condition characterized by a retruded mid-face with an extremely flat nose. We report here six patients with maxillonasal dysplasia whose noses were corrected with onlay costal cartilage grafts using a combined oral vestibular and external rhinoplasty approach for nasal dorsal augmentation, columellar lengthening, and premaxillary augmentation. The cartilage graft was dipped in a solution of 100 ml 0.9% NaCl and one vial (80mg gentamicin for 30 min to prevent warping. L struts made for nasal augmentation, columellar lengthening, and premaxillary augmentation were fixed to one another by slots made in the graft. This technique has been used in children, adults, and for secondary cases with promising results. All patients were of class I dental occlusion. The nasal and premaxillary augmentation which was monitored by serial photography was found to be stable over a follow-up period of three years

  3. Preliminary study on the rate of broncho-pulmonary cancer in a Romanian department (Bihor) for the estimation of radon risk exposure

    International Nuclear Information System (INIS)

    Vaida, T.; Maghiar, F.; Cosma, C.; Ristoiu, D.; Ramboiu, S.; Pacurar, V.; Poffijn, A.

    1996-01-01

    The purpose of this paper is a retrospective estimation on the rate of lung cancer at the population in the Bihor district (Romania) in two years (1993 -1994) and the potential bronchopulmonary cancer risk from indoor radon. In this time were found 473 cases with primary bronchopulmonary cancer: 400 men (84.5%) and 73 women (15.4%). Most cases ( 64.4%) were from rural environment while the other 35.5% were from urban environment. Preliminary study on the potential lung cancer risk from indoor radon encompassed 40 cases and 66 non-cancer controls in the period March-July 1995. (author)

  4. Molecular markers in oral epithelial dysplasia: review.

    Science.gov (United States)

    Pitiyage, Gayani; Tilakaratne, W M; Tavassoli, Mahvash; Warnakulasuriya, Saman

    2009-11-01

    The clinical and histologic features alone cannot accurately predict whether potentially malignant disorders of the oral mucosa remain stable, regress or progress to malignancy. Some of them, with or without epithelial dysplasia, may transform to invasive oral squamous cell carcinomas (OSCC). Identification of molecular markers which can predict disease progression is necessary to improve the management of these disorders. Many genes and signaling pathways have been shown to be involved in the development of OSCC. This review summarizes some molecular markers researched in the detection of pre-cancer. We highlight selected markers that are reported to be significantly associated with progression of potentially malignant disorders to OSCC. These include alterations in genes/pathways which control cellular signaling, cell cycle, apoptosis, genomic stability, cytoskeleton, angiogenesis, etc. However, these genetic tumor markers have so far not gained any use in routine diagnosis and their utility in the prediction of risk of malignant transformation remains unknown. It is, however, clear from the large number of studies, some described in this review, that multiple genes/pathways are involved in the progression from normal to metaplastic/dysplastic, and subsequently to cancer. It is therefore necessary to study those significant alterations in multiple genes simultaneously in biopsy samples from large cohorts of subjects.

  5. Scattering attenuation microscopy of oral epithelial dysplasia

    Science.gov (United States)

    Tomlins, Pete H.; Adegun, Oluyori; Hagi-Pavli, Eleni; Piper, Kim; Bader, Dan; Fortune, Farida

    2010-11-01

    We present a new method for quantitative visualization of premalignant oral epithelium called scattering attenuation microscopy (SAM). Using low-coherence interferometry, SAM projects measurements of epithelial optical attenuation onto an image of the tissue surface as a color map. The measured attenuation is dominated by optical scattering that provides a metric of the severity of oral epithelial dysplasia (OED). Scattering is sensitive to the changes in size and distribution of nuclear material that are characteristic of OED, a condition recognized by the occurrence of basal-cell-like features throughout the epithelial depth. SAM measures the axial intensity change of light backscattered from epithelial tissue. Scattering measurements are obtained from sequential axial scans of a 3-D tissue volume and displayed as a 2-D SAM image. A novel segmentation method is used to confine scattering measurement to epithelial tissue. This is applied to oral biopsy samples obtained from 19 patients. Our results show that imaging of tissue scattering can be used to discriminate between different dysplastic severities and furthermore presents a powerful tool for identifying the most representative tissue site for biopsy.

  6. Pathogenetic mechanisms of focal cortical dysplasia.

    Science.gov (United States)

    Marin-Valencia, Isaac; Guerrini, Renzo; Gleeson, Joseph G

    2014-07-01

    Focal cortical dysplasias (FCDs) constitute a prevalent cause of intractable epilepsy in children, and is one of the leading conditions requiring epilepsy surgery. Despite recent advances in the cellular and molecular biology of these conditions, the pathogenetic mechanisms of FCDs remain largely unknown. The purpose if this work is to review the molecular underpinnings of FCDs and to highlight potential therapeutic targets. A systematic review of the literature regarding the histologic, molecular, and electrophysiologic aspects of FCDs was conducted. Disruption of the mammalian target of rapamycin (mTOR) signaling comprises a common pathway underlying the structural and electrical disturbances of some FCDs. Other mechanisms such as viral infections, prematurity, head trauma, and brain tumors are also posited. mTOR inhibitors (i.e., rapamycin) have shown positive results on seizure management in animal models and in a small cohort of patients with FCD. Encouraging progress has been achieved on the molecular and electrophysiologic basis of constitutive cells in the dysplastic tissue. Despite the promising results of mTOR inhibitors, large-scale randomized trials are in need to evaluate their efficacy and side effects, along with additional mechanistic studies for the development of novel, molecular-based diagnostic and therapeutic approaches. Wiley Periodicals, Inc. © 2014 International League Against Epilepsy.

  7. [Mondini dysplasia: recurrent bacterial meningitis in adolescence].

    Science.gov (United States)

    Vargas-Dĭaz, J; Garófalo-Gómez, N; Rodríguez, U; Parra, M; Barroso-García, E; Novoa-López, L; Rojas-Massipe, E; Sardiñas-Hernández, N L

    Episodes of recurrent bacterial meningitis can occur in patients due to either congenital or acquired disorders. Congenital deformity of the bony labyrinth can be linked to a fistulous tract communicating it with the intracranial subarachnoid space. Mondini deformity is a frequent malformation in congenitally deaf patients. We report the case of an adolescent with a history of being unable to hear in one ear who, from the age of 10 years, began to suffer repeated bacterial meningoencephalitis with microbiological recovery of Streptococcus pneumoniae on three occasions. The type of germ recovered in the cerebrospinal fluid (CSF) and the history of congenital deafness that was detected when the patient was 3 years old were the diagnostic clues to the possible anomaly of the inner ear with a CSF fistula. The clinically proven CSF rhinorrhea contributed to the diagnosis of an ear anomaly with a fistula. Computerised axial tomography and magnetic resonance studies of the petrous portion of the temporal bone revealed the malformation that was later found and closed during the surgical intervention on the affected ear. The clinical absence of rhinorrhea, a year's progression without new infections after operating on the patient and post-surgery imaging studies were all proof that the fistula had closed. Mondini dysplasia with CSF fistula must be included as a possible diagnosis when faced with a patient with recurrent bacterial meningoencephalitis. Imaging studies, especially magnetic resonance, enable the clinician to check the diagnosis and the CSF fistula can be closed with ear surgery.

  8. Hip dysplasia is more severe in Charcot-Marie-Tooth disease than in developmental dysplasia of the hip.

    Science.gov (United States)

    Novais, Eduardo N; Bixby, Sara D; Rennick, John; Carry, Patrick M; Kim, Young-Jo; Millis, Michael B

    2014-02-01

    Patients with Charcot-Marie-Tooth disease may develop hip dysplasia. Hip geometry in these patients has not been well described in the literature. We compared the hip morphometry in Charcot-Marie-Tooth hip dysplasia (CMTHD) and developmental dysplasia of the hip (DDH) in terms of extent of (1) acetabular dysplasia and subluxation, (2) acetabular anteversion and osseous support, (3) coxa valga and femoral version, and (4) osteoarthritis. Fourteen patients with CMTHD (19 hips; mean age, 23 years) presenting for periacetabular osteotomy were matched to 45 patients with DDH (45 hips; mean age, 21 years) based on age, sex, and BMI. We assessed acetabular dysplasia and subluxation using lateral center-edge angle (LCEA), anterior center-edge angle (ACEA), and acetabular roof angle of Tönnis (TA) on plain pelvic radiographs and acetabular volume, area of femoral head covered by acetabulum, and percentage of femoral head covered by acetabulum on three-dimensional CT reconstruction models. Acetabular version and bony support, femoral version, and neck-shaft angle were measured on two-dimensional axial CT scans. Hip osteoarthritis was graded radiographically according to Tönnis criteria. Acetabular dysplasia was more severe in CMTHD, as measured by smaller LCEA (p Hip subluxation was more pronounced in CMTHD, as demonstrated by lower area of femoral head covered by acetabulum (p = 0.034) and percentage of femoral head covered by acetabulum (p = 0.007). CMTHD was associated with higher acetabular anteversion (p hips were found in CMTHD. The extent of acetabular dysplasia, hip subluxation, acetabular anteversion, coxa valga, and hip osteoarthritis was more severe in CMTHD. These findings are important in choosing the appropriate surgical strategy for patients affected by CMTHD.

  9. Computed tomographic features of fibrous dysplasia of maxillofacial region.

    Science.gov (United States)

    Sontakke, Subodh Arun; Karjodkar, Freny R; Umarji, Hemant R

    2011-03-01

    This study was to find the computed tomographic features of fibrous dysplasia of the maxillofacial region. All eight cases included in the study reported either to Government Dental College and Hospital or Nair Hospital Dental College, Mumbai between 2003 and 2009. The patients were prescribed computed tomogram in addition to conventional radiographs of maxillofacial region which were studied for characteristic features of fibrous dysplasia. The diagnosis of fibrous dysplasia was confirmed by histopathological report. All cases showed the ill-defined margins of lesions except in the region where the lesions were extending to cortex of the involved bone. Internal structure of all cases showed ground glass appearance. Four cases of maxillary lesion showed the displacement of maxillary sinus maintaining the shape of maxillary sinus. Two cases showed complete obliteration of maxillary sinus. Displacement of inferior alveolar canal did not follow any typical pattern in any of the cases but was displaced in different directions. The craniofacial type of fibrous dysplasia is as common as fibrous dysplasia of jaw. The margins, extent, internal structure and effect on surrounding structure are well detected on computed tomographic images.

  10. [Fronto-ethmoidal fibrous dysplasia: a case-report].

    Science.gov (United States)

    Hoareau-Gruchet, F; Nils, M; Spinato, L; Rabeyrin, M; Righini, C A

    2012-01-01

    Fibrous dysplasia is a congenital bone disease, where normal bone is replaced by a fibrous-like tissue with immature osteogenesis. The cephalic extremity is affected in one out of three cases. The aim of this study was to describe a case of fibrous dysplasia and review workup and management of this pathology. We reviewed relevant publications using the Medline database and presented a case of isolated paranasal sinus fibrous dysplasia to illustrate this disease. The clinical onset was headache and left palpebral oedema in a twelve-year old. Imaging showed a left fronto-ethmoidal bone lesion with epidural empyema. No other radiological anomaly or phosphor-calcic disorder was present. The clinical course was positive using intravenous antibiotics, with a two-year follow-up. Fibrous dysplasia is diagnosed using CT-scan, without the need for histologic confirmation in most cases. Initial workup includes axial skeleton X-rays and hormonal dosages. The efficiency of biphosphonates drugs has significally reduced the number of surgical procedures. Fibrous dysplasia is often a multifocal disease and should be treated medically first. Management requires a multi-disciplinary team.

  11. Computed tomographic features of fibrous dysplasia of maxillofacial region

    International Nuclear Information System (INIS)

    Sontakke, Subodh Arun; Karjodka, Freny R; Umarji, Hemant R

    2011-01-01

    This study was to find the computed tomographic features of fibrous dysplasia of the maxillofacial region. All eight cases included in the study reported either to Government Dental College and Hospital or Nair Hospital Dental College, Mumbai between 2003 and 2009. The patients were prescribed computed tomogram in addition to conventional radiographs of maxillofacial region which were studied for characteristic features of fibrous dysplasia. The diagnosis of fibrous dysplasia was confirmed by histopathological report. All cases showed the ill-defined margins of lesions except in the region where the lesions were extending to cortex of the involved bone. Internal structure of all cases showed ground glass appearance. Four cases of maxillary lesion showed the displacement of maxillary sinus maintaining the shape of maxillary sinus. Two cases showed complete obliteration of maxillary sinus. Displacement of inferior alveolar canal did not follow any typical pattern in any of the cases but was displaced in different directions. The craniofacial type of fibrous dysplasia is as common as fibrous dysplasia of jaw. The margins, extent, internal structure and effect on surrounding structure are well detected on computed tomographic images.

  12. Computed tomographic features of fibrous dysplasia of maxillofacial region

    Energy Technology Data Exchange (ETDEWEB)

    Sontakke, Subodh Arun; Karjodka, Freny R [Nair Hospital Dental College, Mumba (India); Umarji, Hemant R [Government Dental College and Hospital, Mumbai (India)

    2011-03-15

    This study was to find the computed tomographic features of fibrous dysplasia of the maxillofacial region. All eight cases included in the study reported either to Government Dental College and Hospital or Nair Hospital Dental College, Mumbai between 2003 and 2009. The patients were prescribed computed tomogram in addition to conventional radiographs of maxillofacial region which were studied for characteristic features of fibrous dysplasia. The diagnosis of fibrous dysplasia was confirmed by histopathological report. All cases showed the ill-defined margins of lesions except in the region where the lesions were extending to cortex of the involved bone. Internal structure of all cases showed ground glass appearance. Four cases of maxillary lesion showed the displacement of maxillary sinus maintaining the shape of maxillary sinus. Two cases showed complete obliteration of maxillary sinus. Displacement of inferior alveolar canal did not follow any typical pattern in any of the cases but was displaced in different directions. The craniofacial type of fibrous dysplasia is as common as fibrous dysplasia of jaw. The margins, extent, internal structure and effect on surrounding structure are well detected on computed tomographic images.

  13. Frequency of Developmental Hip Dysplasia in a Training Hospital

    Directory of Open Access Journals (Sweden)

    Emrah Can

    2010-09-01

    Full Text Available Aim: We aimed to determine the frequency of cases with developmental hip dysplasia during the neonatal period and the associated risk factors. Methods: Hip ultrasound images of 258 full-term newborns aged one month were evaluated prospectively in conjunction with physical examination for developmental hip dysplasia in GATA well baby outpatient clinic. Results: The incidence of developmental hip dysplasia was 0.3% in one-month-old, healthy, full-term babies included in the study. 44% of them were male and 56% were female. The mean birth weight was 3311.8±511.4 g. 48% of all births were spontaneous vaginal deliveries and 52% cesarean deliveries. A history of oligohydramniosis was present in 8% of cases, 1% had breech presentation, 7% had a family history of developmental dysplasia and 7% had been swaddled. Additional congenital anomaly and torticollis were not detected at the physical examination of the babies. The risk factors were determined to be twin birth and female gender in the only baby who had developmental hip dysplasia (Type 2a. Conclusion: Developmental hip displasia is a significant public health concern in developing countries. Physical examination performed in conjunction with hip ultrasonography routinely in the neonatal period may contribute to the early diagnosis and treatment. (The Medical Bulletin of Haseki 2010; 48: 99-102

  14. Craniofacial fibrous dysplasia - A review of current management techniques

    Directory of Open Access Journals (Sweden)

    Yadavalli Guruprasad

    2012-01-01

    Full Text Available Fibrous dysplasia is a pathologic condition of bone of unknown etiology with no apparent familial, hereditary or congenital basis. Lichtenstein first coined the term in 1938 and in 1942 he and Jaffe separated it from other fibro-osseous lesions. It is a bone tumor that, although benign, has the potential to cause significant cosmetic and functional disturbance, particularly in the craniofacial skeleton. Its management poses significant challenges to the surgeon. Craniofacial fibrous dysplasia is 1 of 3 types of fibrous dysplasia that can affect the bones of the craniofacial complex, including the mandible and maxilla. Fibrous dysplasia is a skeletal developmental disorder of the bone-forming mesenchyme that manifests as a defect in osteoblastic differentiation and maturation. It is a lesion of unknown etiology, uncertain pathogenesis, and diverse histopathology. Fibrous dysplasia represents about 2, 5% of all bone tumors and over 7% of all benign tumours. Over the years, we have gained a better understanding of its etiology, clinical behavior, and both surgical and non-surgical treatments.

  15. Concurrent follicular dysplasia and interface dermatitis in Boxer dogs.

    Science.gov (United States)

    Rachid, Milene A; Demaula, Christopher D; Scott, Danny W; Miller, William H; Senter, David A; Myers, Sherry

    2003-06-01

    Recurrent or persistent follicular dysplasia and interface dermatitis are described in nine Boxers. Data on age, sex, seasonality of alopecia and histopathological features of the follicular dysplasia in these nine Boxers are comparable with those described in previous reports. The interface dermatitis was characterized by multifocal annular crusted lesions confined to the areas of follicular dysplasia. The inflammatory lesions were neither pruritic nor painful and affected dogs were otherwise healthy. Histopathologically the clinically inflammatory lesions were characterized as an interface dermatitis. Immunohistochemical studies failed to demonstrate immunoglobulins or complement at the basement membrane zone or within blood vessel walls. In dogs with recurrent or persistent disease, the follicular dysplasia and interface dermatitis ran identical, concurrent courses of spontaneous remission and recurrence, or persistence, respectively. One dog with persistent disease was treated successfully with tetracycline and niacinamide for the interface dermatitis, and melatonin for the follicular dysplasia. Although the aetiopathogenesis of this newly described condition and the relationship between the two histological reaction patterns are not known, photoperiod and genetic predisposition appear to play a role.

  16. Calcium-sensing receptor in rat vagal bronchopulmonary sensory neurons regulates the function of the capsaicin receptor TRPV1.

    Science.gov (United States)

    Gu, Qihai; Vysotskaya, Zhanna V; Moss, Charles R; Kagira, Martin K; Gilbert, Carolyn A

    2013-11-01

    Extracellular calcium-sensing receptor (CaSR) has been known to play a critical role in the maintainance of systemic Ca(2+) homeostasis. Recent studies have shown that CaSR is also expressed in many tissues that are not directly related to plasma Ca(2+) regulation, such as the central and peripheral nervous system, where the function of this receptor remains to be defined. In this study, we aimed to investigate the expression of CaSR and its potential interaction with transient receptor potential vanilloid receptor type 1 (TRPV1) in rat vagal bronchopulmonary sensory neurons. Our immunohistochemical experiments demonstrated the expression of CaSR in these sensory neurons as well as in trachea and lung parenchyma. Results from our whole-cell patch-clamp recordings in isolated neurons showed that strong activation of CaSR with high concentrations of its agonists, including spermine, NPS R-568 and Ca(2+), inhibited the capsaicin-evoked whole-cell inward current. Blockade of CaSR with its antagonists NPS 2390 and NPS 2143 significantly enhanced the capsaicin-evoked TRPV1 current. These data suggest that CaSR is likely to be involved in the integration of primary bronchopulmonary sensory inputs in physiological and/or pathophysiological conditions.

  17. Arrhythmogenic right ventricular dysplasia: MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Wall, E.E. van der; Bootsma, M.M.; Schalij, M.J. [Dept. of Cardiology, Leiden Univ. Medical Center, Leiden (Netherlands); Kayser, H.W.M.; Roos, A. de [Dept. of Radiology, Leiden Univ. Medical Center, Leiden (Netherlands)

    2000-06-01

    Arrhythmogenic right ventricular dysplasia (ARVD) is a heart muscle disorder of unknown cause that is characterized pathologically by fibrofatty replacement of the right ventricular myocardium. Clinical manifestations include structural and functional malformations of the right ventricle, electrocardiographic abnormalities, and presentation with ventricular tachycardias with left bundle branch pattern or sudden death. The disease is often familial with an autosomal inheritance. In addition to right ventricular dilatation, right ventricular aneurysms are typical deformities of ARVD and they are distributed in the so-called ''triangle of dysplasia'', i. e., right ventricular outflow tract, apex, and infundibulum. Ventricular aneurysms at these sites can be considered pathognomonic of ARVD. Another typical hallmark of ARVD is fibrofatty infiltration of the right ventricular free wall. These functional and morphologic characteristics are relevant to clinical imaging investigations such as contrast angiography, echocardiography, radionuclide angiography, ultrafast computed tomography, and magnetic resonance imaging (MRI). Among these techniques, MRI allows the clearest visualization of the heart, in particular because the right ventricle is involved, which is usually more difficult to explore with the other imaging modalities. Furthermore, MRI offers the specific advantage of visualizing adipose infiltration as a bright signal of the right ventricular myocardium. MRI provides the most important anatomic, functional, and morphologic criteria for diagnosis of ARVD within one single study. As a result, MRI appears to be the optimal imaging technique for detecting and following patients with clinical suspicion of ARVD. (orig.) [German] Die arrhythmogene rechtsventrikulaere Dysplasie (ARVD), eine Herzmuskelerkrankung unklarer Aetiologie, ist pathologisch durch fettige Degeneration des rechtsventrikulaeren Myokards gekennzeichnet. Die klinischen Symptome

  18. A case report of arrhythmogenic right ventricular dysplasia

    Directory of Open Access Journals (Sweden)

    Henry Anselmo Mayala

    2013-07-01

    Full Text Available Background Arrhythmogenic right ventricular dysplasia is an autosomal dominant disorder affecting parts of myocardium known as desmosomes, areas on the surface of heart muscle cells which link the cells together. The hallmark feature is fibro-fatty replacement of the right ventricle myocardium characterized by hypokinetic areas with associated arrhythmias originating in the right ventricle. Case Presentation In this report a 42 year old man was admitted at Wuhan union Hospital with the presenting complaints of visual hallucination and difficulty in breathing on exertion, with a family history of sudden death. Clinical and imaging findings are suggestive of Arrhythmogenic right ventricular dysplasia. Conclusion Despite being among the rare cardiac disease, Arrhythmogenic right ventricular dysplasia is an important cause of ventricular arrhythmias in children and young adults, it is also responsible for sudden cardiac death in the young population, making it necessary for this case report.

  19. A case report of arrhythmogenic right ventricular dysplasia

    Directory of Open Access Journals (Sweden)

    Henry Anselmo Mayala

    2013-01-01

    Full Text Available Background Arrhythmogenic right ventricular dysplasia is an autosomal dominant disorder affecting parts of myocardium known as desmosomes, areas on the surface of heart muscle cells which link the cells together. The hallmark feature is fibro-fatty replacement of the right ventricle myocardium characterized by hypokinetic areas with associated arrhythmias originating in the right ventricle. CasePresentation Inthisreporta42yearoldmanwasadmittedatWuhanunion Hospital with the presenting complaints of visual hallucination and difficulty in breathing on exertion, with a family history of sudden death. Clinical and imaging findings are suggestive of Arrhythmogenic right ventricular dysplasia. Conclusion Despitebeingamongtherarecardiacdisease,Arrhythmogenicright ventricular dysplasia is an important cause of ventricular arrhythmias in children and young adults, it is also responsible for sudden cardiac death in the young population, making it necessary for this case report.

  20. Decortication of fibrous dysplasia of the maxillary sinus.

    Science.gov (United States)

    Lee, Yoon Ho; Hwang, Kun

    2011-01-01

    We present 3 cases of fibrous dysplasia of the maxillary sinus that were treated by decortication through the anterior wall of the maxilla. The thickened anterior wall of the maxilla and the zygoma were contoured into normal shape with a bur, and a 2 × 2-cm window was made below the infraorbital foramen using a reciprocating saw. The removed piece of bone was 3 to 5 mm thick. All abnormal fibrous tissues in the maxillary sinus were extirpated by drilling through the window until the sinus cavity was clear. The preserved bone plate was restored in place to close the window of the maxillary anterior wall and fixed with miniplates. Three patients were treated by means of such internal decompression method. No overgrowth or disfigurement of the facial bone was appreciated 14 to 51 months postoperatively. We contend that internal decortication of fibrous dysplasia through a window in the anterior wall is effective to minimize external expansion of fibrous dysplasia.

  1. Fibrous dysplasia and ossifying fibroma of the paranasal sinuses.

    Science.gov (United States)

    Commins, D J; Tolley, N S; Milford, C A

    1998-10-01

    Fibro-osseous lesions involving the paranasal sinuses, the mid-face and anterior skull base are uncommon. In addition, there appears to be no clear pathological or clinical classification that embraces the variety of lesions that exhibit such diverse pathological and clinical behaviour, yet may still be referred to as a fibro-osseous lesion. The diagnosis of fibrous dysplasia and ossifying fibroma is made on a combination of clinical, radiological and pathological criteria. This paper emphasizes the clinical and pathological differences between fibrous dysplasia and ossifying fibroma. The more aggressive clinical behaviour of the latter is highlighted and a more radical surgical approach is recommended. In contradistinction, fibrous dysplasia can exhibit a more benign behaviour and radical surgery is not always justified. A clinicopathological distinction between these two conditions is important from a management perspective despite the fact that they both may be encompassed under the 'umbrella' term fibro-osseous lesion.

  2. Osseous Dysplasia Accompanied by a Complex Odontoma in the Mandible

    Directory of Open Access Journals (Sweden)

    N Jafari

    2017-05-01

    Full Text Available Introduction: Cement osseous dysplasia is the most common lesion of the family fibrous dysplasia which normal bone is replaced with fibrous tissue and several mineralized material. These lesions may be seen in association with various lesions. Companionship of this lesion with odontoma has been reported rarely. Case report: A 44-year-old female in one year ago has admitted to Shahid Sadoughi Faculty of Dentistry Yazd for treatment of mandibular left third molar. In panoramic radiography in the mandibular left first molar region, a mixed lesion of radiolucent-radiopaque with a corticated borderwas seen. Small swelling was observed in this area. Microscopically, the diagnosis of osseous dysplasia with odontoma for the lesion was confirmed. Conclusion: The reason of companionship of cement osseous lesions whit other lesions is not completely clear and it requires further evaluation.

  3. Radiological analysis of polyostotic fibrous dysplasia in skeletal system

    International Nuclear Information System (INIS)

    Shin, Ma Rie; Kim, Jin Sik; Kim, Han Suk; Park, Soo Soung

    1984-01-01

    Over a period of recent 3 years, the 5 cases of polyostotic fibrous dysplasia were proven histologically at National Medical Center, and they were evaluated and analyzed radiologically and clinically. The results were as follows: 1. The age of 5 patients ranged from 12 to 21. 2. In general, clinical symptoms of these patients were pain of affected sites and swelling , fracture, walking disturbance of lower extremities. 3. The order of frequent site of polyostotic fibrous dysplasia was skull (4 cases), femur (3 cases), maxilla (2 case), humerus, tibia, rib, radius, metacarpal bone and phalanx. 4. The characteristic radiological findings of polyostotic fibrous dysplasia were multicystic lesions with ground glass appearance, osteosclerosis, cortical thinning and pathologic fracture and deformity of long bones. Particularly, in the extremities, multicystic radiolucencies, groud glass appearance, shepherd's crook and coxa vara deformities were noticed, and in the skull and maxilla, sclerotic changes were principally demonstrated.

  4. Evaluation of reflux nephropathy, pyelonephritis and renal dysplasia

    International Nuclear Information System (INIS)

    Grattan-Smith, J.D.; Little, Stephen B.; Jones, Richard A.

    2008-01-01

    MR urography has the potential to significantly improve our understanding of the relationship between reflux nephropathy, pyelonephritis, vesicoureteric reflux and renal dysplasia. MR urography utilizes multiple parameters to assess both renal anatomy and function and provides a more complete characterization of acquired and congenital disease. Pyelonephritis and renal scarring can be distinguished by assessing the parenchymal contours and signal intensity. Characteristic imaging features of renal dysplasia include small size, subcortical cysts, disorganized architecture, decreased and patchy contrast enhancement as well as a dysmorphic pelvicalyceal system. Because of its ability to subdivide and categorize this heterogeneous group of disorders, it seems inevitable that MR urography will replace DMSA renal scintigraphy as the gold standard for assessment of pyelonephritis and renal scarring. MR urography will contribute to our understanding of renal dysplasia and its relationship to reflux nephropathy. (orig.)

  5. Evaluation of reflux nephropathy, pyelonephritis and renal dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Grattan-Smith, J.D. [Emory University School of Medicine, Children' s Healthcare of Atlanta, Department of Radiology, Atlanta, GA (United States); Children' s Healthcare of Atlanta, Department of Radiology, Atlanta, GA (United States); Little, Stephen B. [Children' s Healthcare of Atlanta, Department of Radiology, Atlanta, GA (United States); Jones, Richard A. [Emory University School of Medicine, Children' s Healthcare of Atlanta, Department of Radiology, Atlanta, GA (United States)

    2008-01-15

    MR urography has the potential to significantly improve our understanding of the relationship between reflux nephropathy, pyelonephritis, vesicoureteric reflux and renal dysplasia. MR urography utilizes multiple parameters to assess both renal anatomy and function and provides a more complete characterization of acquired and congenital disease. Pyelonephritis and renal scarring can be distinguished by assessing the parenchymal contours and signal intensity. Characteristic imaging features of renal dysplasia include small size, subcortical cysts, disorganized architecture, decreased and patchy contrast enhancement as well as a dysmorphic pelvicalyceal system. Because of its ability to subdivide and categorize this heterogeneous group of disorders, it seems inevitable that MR urography will replace DMSA renal scintigraphy as the gold standard for assessment of pyelonephritis and renal scarring. MR urography will contribute to our understanding of renal dysplasia and its relationship to reflux nephropathy. (orig.)

  6. Polyostotic Fibrous Dysplasia of Cranio-Maxillofacial Area

    Energy Technology Data Exchange (ETDEWEB)

    Han, Jin Woo; Kwon, Hyuk Rok; Lee, Jin Ho; Park, In Woo [Dept. of Oral and Maxillofacial Radiology, College of Dentistry, Kangnung National University, Kangnung (Korea, Republic of)

    2000-06-15

    Fibrous dysplasia is believed to be a hamartomatous developmental lesion of unknown origin. This disease is divided into monostotic and polyostotic fibrous dysplasia. Polyostotic type can be divided into craniofacial type, Lichtenstein-Jaffe type, and McCune-Albright syndrome. In this case, a 31-year-old female presented spontaneous loss of right mandibular teeth before 5 years and has shown continuous expansion of right mandibular alveolus. Through the radiographic view, the coarse pattern of the mixed radiopaque-lucent lesion was seen on the right mandibular body, and there was diffuse pattern of the mixed radiopaque-lucent lesion with ill-defined margin in the left mandibular body. In the right calvarium, the lesion had cotton-wool appearance. Partial excision for contouring, multiple extraction, and alveoloplasty were accomplished under general anesthesia for supportive treatment. Finally we could conclude this case was polyostotic fibrous dysplasia of cranio-maxillofacial area based on the clinical, radiologic finding, and histopathologic examination.

  7. Arrhythmogenic right ventricular dysplasia: Atypical clinical presentation.

    Science.gov (United States)

    Marçalo, José; Menezes Falcão, Luiz

    2017-03-01

    A 67-year-old man was admitted to our hospital after episodes of syncope preceded by malaise and diffuse neck and chest discomfort. No family history of cardiac disease was reported. Laboratory workup was within normal limits, including D-dimers, serum troponin I and arterial blood gases. The electrocardiogram showed sinus rhythm with T-wave inversion in leads V1 to V3. Computed tomography angiography to investigate pulmonary embolism showed no abnormal findings. Transthoracic echocardiography (TTE) displayed massive enlargement of the right ventricle with intact interatrial septum and no pulmonary hypertension. Cardiac magnetic resonance imaging (MRI) confirmed right ventricular (RV) dilatation and revealed marked hypokinesia/akinesia of the lateral wall. Exercise stress testing was negative for ischemia. According to the 2010 Task Force criteria for arrhythmogenic right ventricular dysplasia (ARVD), this patient presented two major criteria (global or regional dysfunction and structural alterations: by MRI, regional RV akinesia or dyskinesia or dyssynchronous RV contraction and RV ejection fraction ≤40%, and repolarization abnormalities: inverted T waves in right precordial leads [V1, V2, and V3]); and one minor criterion (>500 ventricular extrasystoles per 24 hours by Holter), and so a diagnosis of ARVD was made. After electrophysiologic study (EPS) the patient received an implantable cardioverter-defibrillator (ICD). This late clinical presentation of ARVD highlights the importance of TTE screening, possibly complemented by MRI. The associated risk of sudden death was assessed by EPS leading to the implantation of an ICD. Genetic association studies should be offered to the offspring of all ARVD patients. Copyright © 2016 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.

  8. Hereditary ectodermal dysplasia: A retrospective study.

    Science.gov (United States)

    More, Chandramani B; Bhavsar, Khusbhu; Joshi, Jigar; Varma, Saurabh N; Tailor, Mansi

    2013-07-01

    Ectodermal dysplasia (ED) is a group of rare, inherited disorders characterized by sparse hair, missing teeth and inability to sweat. To review and analyze cases of ED with an emphasis on clinical manifestations and parent's marriage history. The present retrospective study was conducted by assessing the clinical records of nineteen cases of ED, available in the archives of the department; for age, gender, family history of consanguineous marriage and clinical manifestations. It was observed that ED was more prevalent in males, with a ratio of 1.7:1. The hypohydrotic type was more common (78.95%) than hydrotic type (21.05%). The marriage history of parents revealed that 66.67% had consanguineous marriage and had 68.42% offspring's affected with ED; whereas 33.33% had history of non-consanguineous marriage and had 31.58% offspring's affected with ED. The clinical manifestations observed were- dry skin(94.74%); scaly skin(42.11%); sparse hair on scalp, eyebrows and eyelashes(100%); frontal bossing(63.18%); saddle nose (57.89%); hypertelorism (47.37%); nail abnormality(52.63%); normal sweat glands(21.05%); abnormal sweat glands(78.95%); hypoplastic maxilla(52.63%); protuberant lips (57.89%); palmo-plantar keratosis(21.05%); wrinkled & hyper pigmented facial skin(84.21%); partial anodontia(94.74%); conical shaped teeth(84.21%); high arched palate(68.42%); thin alveolar bone(100.00%); taurodontism(21.05%) and cleft lip & cleft palate(05.26%). The number of teeth present in all the cases ranged from 0 to 19. ED patients suffer from social problems and poor psychological and physiological development as a result of unacceptable esthetics and abnormal function of orofacial structures. Oral rehabilitation thus becomes mandatory, although it is often difficult; particularly in pediatric patients.

  9. A case of monostotic fibrous dysplasia of the maxillary sinus.

    Science.gov (United States)

    Erdem, L Oktay; Erdem, C Zuhal; Kargi, Sebnem

    2003-05-01

    Fibrous dysplasia is an uncommon benign disease of the bone, with slow progression. Monostotic involvement of the paranasal sinuses is rare. We report a 54-year-old woman who had complaints of facial asymmetry, chronic sinusitis, recurrent headaches, and nasal obstruction for two years. Conventional radiography showed opacification and expansion of the maxillary sinus. Axial and coronal computed tomography scans showed a heterogeneous mass that expanded the right maxillary sinus, leading to nasal obstruction and cortical thickening of the maxilla. No signs of destruction or erosion in the cortical bone were identified. An endonasal endoscopic biopsy was performed and the diagnosis of fibrous dysplasia was confirmed histologically.

  10. Treatment of craniofacial fibrous dysplasia: a case report.

    Science.gov (United States)

    Carini, F; Saggese, V; Porcaro, G; Baldoni, M

    2014-01-01

    The concept of "fibro-osseous lesions" of bone has evolved over the last several years and now includes two mayor entities: the fibrous dysplasia (FD) and the cement-ossifying fibroma (COF). Fibrous dysplasia is considered to be a developmental, tumor-like (hamartomatous), fibro-osseous disease of unknown etiology. There is a maxillary predominance when craniofacial FD occurs in the jaws and the maxillary sinus is commonly involved. Differentiation of OF from FD is important because of differences in treatment and behaviour. This article report a case of 60-year-old female with a history of painless unilateral palatal swelling.

  11. Focal Cemento-osseous Dysplasia: Report of an Unusual Case

    Directory of Open Access Journals (Sweden)

    Amita Aditya

    2013-01-01

    Full Text Available Fibro-osseous lesions of the jaws pose diagnostic and therapeutic difficulties due to their clinical, radiological and histological variability. Cemento-ossifying fibroma is a rare, benign fibro-osseous lesion that arises from the periodontal ligament. Cemento- osseous dysplasia is another variant of fibro-osseous lesion which commonly involves mandible of females. We report a case of an unusual presentation of cemento-osseous dysplasia in a 13-year-old female which posed a diagnostic dilemma clinically and histopathologically, due to its close resemblance to cemento-ossifying fibroma.

  12. Angiographic diagnosis of fibromuscular dysplasia of the renal arteries

    Energy Technology Data Exchange (ETDEWEB)

    Petkov, B.; Grigorov, G.; Nedelkov, G. (Meditsinska Akademiya, Sofia (Bulgaria). Nauchen Inst. po Rentgenologiya i Radiobiologiya)

    1982-01-01

    In the nosological structure of renovascular hypertensions fibromuscular dysplasia of the renal arteries by incidence ranks second after atherosclerosis. The illness affects mainly women, more frequently young and having borne a child. Hypertension in such patients is usually characterized by high values of the diastolic pressure, and leads to early neurologic complaints such as headache, sight impairment, vertigo and Meniere-like syndromes. Morphological and functional changes are likewise described. Some of the literature statements concerning the etiopathogenesis and classification of fibromuscular dysplasia are critically assayed. The success of the surgical management depends on the timely established exact diagnosis, and angiography appears to be the only method of primary importance in this respect.

  13. Cystic fibrous dysplasia mimicking giant cell tumor: MRI appearance

    Energy Technology Data Exchange (ETDEWEB)

    Okada, Kyoji; Yoshida, Sumiko [Department of Orthopaedics, Akita University School of Medicine, Akita (Japan); Okane, Kumiko [Department of Radiology, Akita University School of Medicine, Akita (Japan); Sageshima, Masato [Division of Clinical Pathology, Akita University Hospital, Akita (Japan)

    2000-01-01

    We report the case of a 43-year-old man who presented with an osteolytic and expansive lesion in the left distal femur mimicking a giant cell tumor. Magnetic resonance imaging (MRI) showed that most of the lesion was cystic, and histological examination revealed fibrous dysplasia with marked cystic degeneration. Radiographic findings of cystic fibrous dysplasia in the end of a long bone may be similar to those of a giant cell tumor, and a biopsy is essential for the final diagnosis. (orig.)

  14. Hip Dysplasia: Clinical Signs and Physical Examination Findings.

    Science.gov (United States)

    Syrcle, Jason

    2017-07-01

    Hip dysplasia is a common developmental disorder of the dog, consisting of varying degrees of hip laxity, progressive remodeling of the structures of the hip, and subsequent development of osteoarthritis. It is a juvenile-onset condition, with clinical signs often first evident at 4 to 12 months of age. A tentative diagnosis of hip dysplasia can be made based on signalment, history, and physical examination findings. The Ortolani test is a valuable tool for identifying juvenile dogs affected with this condition. Further diagnostics can then be prioritized, contributing to prompt diagnosis and appropriate treatment. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. A case of Mondini dysplasia with recurrent Streptococcus pneumoniae meningitis.

    Science.gov (United States)

    Yilmaz Ciftdoğan, Dilek; Bayram, Nuri; Ozdemir, Yasemin; Bayraktaroğlu, Selen; Vardar, Fadil

    2009-12-01

    Mondini's dysplasia is a developmental anomaly of the middle ear characterized by cochlear malformation with dilation of the vestibular aquaduct, vestibule, and ampullar ends of the semicircular canals. These deformities may result in a connection between subarachnoid space and the middle ear resulting in recurrent episodes of meningitis. Additionally, it is commonly associated with hearing impairment. We describe here a boy with recurrent meningitis and unilateral sensorineural hearing loss. Mondini dysplasia was demonstrated with computed tomographic scans of the temporal bones in the search for pathogenesis of recurrent meningitis.

  16. CT features of fibrous dysplasia of the temporal bone

    International Nuclear Information System (INIS)

    Charrada-Ben Farhat, L.; Bourkhis, S.; Ben Yaacoub, I.; Dali, N.; Askri, A.; Hendaoui, L.

    2006-01-01

    Fibrous dysplasia is characterized by a progressive replacement of normal bone elements by fibrous tissue. The temporal bone is rarely involved. In this location, complications such as facial deformity, conductive hearing loss and facial peripheral neural involvement can occur. Positive diagnosis can be established with computerized tomography which also enables assessment of extension and detection of complications. We report a case of a 27-year-old man with extensive fibrous dysplasia of the right temporal bone presenting with conductive hearing loss secondary to progressive stenosis of the external auditory canal. Computerized tomography of the temporal region was performed. (authors)

  17. Generation and characterization of function-blocking anti-ectodysplasin A (EDA) monoclonal antibodies that induce ectodermal dysplasia.

    Science.gov (United States)

    Kowalczyk-Quintas, Christine; Willen, Laure; Dang, Anh Thu; Sarrasin, Heidi; Tardivel, Aubry; Hermes, Katharina; Schneider, Holm; Gaide, Olivier; Donzé, Olivier; Kirby, Neil; Headon, Denis J; Schneider, Pascal

    2014-02-14

    Development of ectodermal appendages, such as hair, teeth, sweat glands, sebaceous glands, and mammary glands, requires the action of the TNF family ligand ectodysplasin A (EDA). Mutations of the X-linked EDA gene cause reduction or absence of many ectodermal appendages and have been identified as a cause of ectodermal dysplasia in humans, mice, dogs, and cattle. We have generated blocking antibodies, raised in Eda-deficient mice, against the conserved, receptor-binding domain of EDA. These antibodies recognize epitopes overlapping the receptor-binding site and prevent EDA from binding and activating EDAR at close to stoichiometric ratios in in vitro binding and activity assays. The antibodies block EDA1 and EDA2 of both mammalian and avian origin and, in vivo, suppress the ability of recombinant Fc-EDA1 to rescue ectodermal dysplasia in Eda-deficient Tabby mice. Moreover, administration of EDA blocking antibodies to pregnant wild type mice induced in developing wild type fetuses a marked and permanent ectodermal dysplasia. These function-blocking anti-EDA antibodies with wide cross-species reactivity will enable study of the developmental and postdevelopmental roles of EDA in a variety of organisms and open the route to therapeutic intervention in conditions in which EDA may be implicated.

  18. Therapeutic hypercapnia prevents bleomycin-induced pulmonary hypertension in neonatal rats by limiting macrophage-derived tumor necrosis factor-α.

    Science.gov (United States)

    Sewing, A Charlotte P; Kantores, Crystal; Ivanovska, Julijana; Lee, Alvin H; Masood, Azhar; Jain, Amish; McNamara, Patrick J; Tanswell, A Keith; Jankov, Robert P

    2012-07-01

    Bleomycin-induced lung injury is characterized in the neonatal rat by inflammation, arrested lung growth, and pulmonary hypertension (PHT), as observed in human infants with severe bronchopulmonary dysplasia. Inhalation of CO(2) (therapeutic hypercapnia) has been described to limit cytokine production and to have anti-inflammatory effects on the injured lung; we therefore hypothesized that therapeutic hypercapnia would prevent bleomycin-induced lung injury. Spontaneously breathing rat pups were treated with bleomycin (1 mg/kg/d ip) or saline vehicle from postnatal days 1-14 while being continuously exposed to 5% CO(2) (Pa(CO(2)) elevated by 15-20 mmHg), 7% CO(2) (Pa(CO(2)) elevated by 35 mmHg), or normocapnia. Bleomycin-treated animals exposed to 7%, but not 5%, CO(2), had significantly attenuated lung tissue macrophage influx and PHT, as evidenced by normalized pulmonary vascular resistance and right ventricular systolic function, decreased right ventricular hypertrophy, and attenuated remodeling of pulmonary resistance arteries. The level of CO(2) neither prevented increased tissue neutrophil influx nor led to improvements in decreased lung weight, septal thinning, impaired alveolarization, or decreased numbers of peripheral arteries. Bleomycin led to increased expression and content of lung tumor necrosis factor (TNF)-α, which was found to colocalize with tissue macrophages and to be attenuated by exposure to 7% CO(2). Inhibition of TNF-α signaling with the soluble TNF-2 receptor etanercept (0.4 mg/kg ip from days 1-14 on alternate days) prevented bleomycin-induced PHT without decreasing tissue macrophages and, similar to CO(2), had no effect on arrested alveolar development. Our findings are consistent with a preventive effect of therapeutic hypercapnia with 7% CO(2) on bleomycin-induced PHT via attenuation of macrophage-derived TNF-α. Neither tissue macrophages nor TNF-α appeared to contribute to arrested lung development induced by bleomycin. That 7% CO(2

  19. Increased nuclear ?-catenin expression in oral potentially malignant lesions: A marker of epithelial dysplasia

    Science.gov (United States)

    Rojas-Alcayaga, Gonzalo; Maturana, Andrea; Aitken, Juan-Pablo; Rojas, Carolina; Ortega, Ana-Verónica

    2015-01-01

    Background Deregulation of ?-catenin is associated with malignant transformation; however, its relationship with potentially malignant and malignant oral processes is not fully understood. The aim of this study was to determine and compare the nuclear ?-catenin expression in oral dysplasia and oral squamous cell carcinoma (OSCC). Material and Methods Cross sectional study. Immunodetection of ?-catenin was performed on 72 samples, with the following distribution: 21 mild dysplasia, 12 moderate dysplasia, severe dysplasia 3, 36 OSCC including 19 well differentiated, 15 moderately differentiated and 2 poorly differentiated. Through microscopic observation the number of positive cells per 1000 epithelial cells was counted. For the statistical analysis, the Kruskal Wallis test was used. Results Nuclear expression of ?-catenin was observed in all samples with severe and moderate dysplasia, with a median of 267.5, in comparison to mild dysplasia whose median was 103.75. Only 10 samples (27.7%) with OSCC showed nuclear expression, with statistically significant differences between groups (p < 0.05). Conclusions Our results are consistent with most of the reports which show increased presence of ?-catenin in severe and moderate dysplasia compared to mild dysplasia; however the expression of nuclear ?-catenin decreased after starting the invasive neoplastic process. This suggests a role for this protein in the progression of dysplasia and early malignant transformation to OSCC. Immunodetection of ?-catenin could be a possible immune marker in the detection of oral dysplasia. Key words:Oral squamous cell carcinoma (OSCC), ?-catenin, oral dysplasia. PMID:26241451

  20. Histogenesis of retinal dysplasia in trisomy 13

    Directory of Open Access Journals (Sweden)

    Gonzalez-Fernandez Federico

    2007-12-01

    Full Text Available Abstract Background Although often associated with holoprosencephaly, little detail of the histopathology of cyclopia is available. 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. Methods A full pediatric autopsy was performed of a near term infant who died shortly after birth from multiple congenital anomalies including fused facial-midline structures. A detailed histopathological study of the ocular structures was performed. The expression of interphotoreceptor retinoid-binding protein (IRBP, cellular retinal-binding protein (CRALBP, rod opsin, and Sonic Hedgehog (Shh were studied by immunohistochemistry. Results Holoprosencephaly, and a spectrum of anatomical findings characteristic of Patau's syndrome, were found. Cytogenetic studies demonstrated trisomy 13 [47, XY, +13]. The eyes were fused but contained two developed separate lenses. In contrast, the cornea, and angle structures were hypoplastic, and the anterior chamber had failed to form. The retina showed areas of normally laminated neural retina, whereas in other areas it was replaced by numerous neuronal rosettes. Histological and immunohistochemical studies revealed that the rosettes were composed of differentiated retinal neurons and Müller cell glia. In normally laminated retina, Shh expression was restricted to retinal-ganglion cells, and to a population of neurons in the inner zone of the outer nuclear layer. In contrast, Shh could not be detected in the dysplastic rosettes. Conclusion The histopathology of cyclopia appears to be more complex than what may have been previously appreciated. In fact, the terms "cyclopia" and "synophthalmia" are misnomers as the underlying mechanism is a failure of the eyes to form separately during development. The rosettes found in the dysplastic retina are fundamentally different than those of

  1. Histogenesis of retinal dysplasia in trisomy 13

    Science.gov (United States)

    Chan, Ada; Lakshminrusimha, Satyan; Heffner, Reid; Gonzalez-Fernandez, Federico

    2007-01-01

    Background Although often associated with holoprosencephaly, little detail of the histopathology of cyclopia is available. 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. Methods A full pediatric autopsy was performed of a near term infant who died shortly after birth from multiple congenital anomalies including fused facial-midline structures. A detailed histopathological study of the ocular structures was performed. The expression of interphotoreceptor retinoid-binding protein (IRBP), cellular retinal-binding protein (CRALBP), rod opsin, and Sonic Hedgehog (Shh) were studied by immunohistochemistry. Results Holoprosencephaly, and a spectrum of anatomical findings characteristic of Patau's syndrome, were found. Cytogenetic studies demonstrated trisomy 13 [47, XY, +13]. The eyes were fused but contained two developed separate lenses. In contrast, the cornea, and angle structures were hypoplastic, and the anterior chamber had failed to form. The retina showed areas of normally laminated neural retina, whereas in other areas it was replaced by numerous neuronal rosettes. Histological and immunohistochemical studies revealed that the rosettes were composed of differentiated retinal neurons and Müller cell glia. In normally laminated retina, Shh expression was restricted to retinal-ganglion cells, and to a population of neurons in the inner zone of the outer nuclear layer. In contrast, Shh could not be detected in the dysplastic rosettes. Conclusion The histopathology of cyclopia appears to be more complex than what may have been previously appreciated. In fact, the terms "cyclopia" and "synophthalmia" are misnomers as the underlying mechanism is a failure of the eyes to form separately during development. The rosettes found in the dysplastic retina are fundamentally different than those of retinoblastoma, being

  2. Use of Zoledronic Acid in Paediatric Craniofacial Fibrous Dysplasia

    Directory of Open Access Journals (Sweden)

    Chiara Di Pede

    2016-01-01

    Full Text Available We describe a case of a paediatric patient affected by mandibular fibrous dysplasia (FD with severe and chronic pain who was successfully treated with zoledronic acid (ZOL: a third-generation bisphosphonate. Further research is needed to assess its safety and efficacy as a treatment option for FD in the paediatric population.

  3. The extracellular matrix and diffusion barriers in focal cortical dysplasias

    Czech Academy of Sciences Publication Activity Database

    Zámečník, J.; Homola, Aleš; Cicanič, Michal; Kuncová, K.; Marusič, P.; Kršek, P.; Syková, Eva; Vargová, Lýdia

    2012-01-01

    Roč. 36, č. 1 (2012), s. 2017-2024 ISSN 0953-816X R&D Projects: GA MŠk 1M0538 Grant - others:GA MZd(CZ) NS9915 Institutional research plan: CEZ:AV0Z50390512 Keywords : cortical dysplasia * diffusion * extrecellular space Subject RIV: FH - Neurology Impact factor: 3.753, year: 2012

  4. Tooth Abnormalities and Occlusal Disorders in Individuals With Frontonasal Dysplasia.

    Science.gov (United States)

    Dainezi, Vanessa Benetello; Neves, Lucimara Teixeira das; da Silva Dalben, Gisele; Gomide, Marcia Ribeiro

    2017-05-01

      Frontonasal dysplasia is a rare developmental defect of the midface, and little is known about the dental involvement in individuals with this condition. This study investigated tooth abnormalities and occlusal disorders in individuals with frontonasal dysplasia.   Cross-sectional.   Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo, Bauru, Brazil.   Clinical oral examination, analysis of patient records, and panoramic radiographs.   A total of 20 individuals with frontonasal dysplasia aged 7 to 17 years.   Prevalence of the several tooth abnormalities and occlusal disorders analyzed.   A total of 19 individuals presented at least one tooth abnormality, with highly variable findings. In radiographs, 20% of individuals (all presenting oral clefts) presented agenesis of lateral incisors and second premolars. No supernumerary teeth were observed; 65% of individuals exhibited occlusal alterations, especially anterior open bite in the two individuals with median cleft lip.   Variable clinical and radiographic alterations were observed, probably due to the large variety of phenotypic characteristics. No specific dental alteration could be related with frontonasal dysplasia.

  5. Diencephalic-Mesencephalic Junction Dysplasia: A Novel Recessive Brain Malformation

    Science.gov (United States)

    Zaki, Maha S.; Saleem, Sahar N.; Dobyns, William B.; Barkovich, A. James; Bartsch, Hauke; Dale, Anders M.; Ashtari, Manzar; Akizu, Naiara; Gleeson, Joseph G.; Grijalvo-Perez, Ana Maria

    2012-01-01

    We describe six cases from three unrelated consanguineous Egyptian families with a novel characteristic brain malformation at the level of the diencephalic-mesencephalic junction. Brain magnetic resonance imaging demonstrated a dysplasia of the diencephalic-mesencephalic junction with a characteristic "butterfly"-like contour of the…

  6. Focal fibrocartilaginous dysplasia and tibia vara: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Cockshott, W.P. (Dept. of Radiology, McMaster Univ. Medical Centre, Hamilton, ON (Canada)); Martin, R. (Dept. of Orthopedics, McMaster Univ. Medical Centre, Hamilton, ON (Canada)); Friedman, L. (Dept. of Radiology, McMaster Univ. Medical Centre, Hamilton, ON (Canada)); Yuen, M. (Dept. of Radiology, McMaster Univ. Medical Centre, Hamilton, ON (Canada))

    1994-07-01

    A 2-year-old black boy with focal fibrocartilaginous dysplasia is described with illustrations of the typical radiographic appearances supplemented by computed tomographic and magnetic resonance images. Since this rare condition is self-correcting, diagnosis is important so that surgical intervention and biopsy can be avoided and conservative management instituted. (orig.)

  7. Endoscopic appearance of dysplasia and cancer in inflammatory bowel disease

    NARCIS (Netherlands)

    Tytgat, G. N.; Dhir, V.; Gopinath, N.

    1995-01-01

    Dysplastic alteration of mucosa may occur in flat or raised mucosal lesions. Over 95% of dysplastic foci occur in flat mucosa. Flat dysplasia is occasionally visible macroscopically as areas of discolouration, velvety-villous appearance, or peculiar fine nodular thickening. The prevalence of

  8. Autosomal dominant frontometaphyseal dysplasia: Delineation of the clinical phenotype

    NARCIS (Netherlands)

    Wade, Emma M.; Jenkins, Zandra A.; Daniel, Philip B.; Morgan, Tim; Addor, Marie C.; Adés, Lesley C.; Bertola, Debora; Bohring, Axel; Carter, Erin; Cho, Tae-Joon; de Geus, Christa M.; Duba, Hans-Christoph; Fletcher, Elaine; Hadzsiev, Kinga; Hennekam, Raoul C. M.; Kim, Chong A.; Krakow, Deborah; Morava, Eva; Neuhann, Teresa; Sillence, David; Superti-Furga, Andrea; Veenstra-Knol, Hermine E.; Wieczorek, Dagmar; Wilson, Louise C.; Markie, David M.; Robertson, Stephen P.

    2017-01-01

    Frontometaphyseal dysplasia (FMD) is caused by gain-of-function mutations in the X-linked gene FLNA in approximately 50% of patients. Recently we characterized an autosomal dominant form of FMD (AD-FMD) caused by mutations in MAP3K7, which accounts for the condition in the majority of patients who

  9. Autosomal dominant frontometaphyseal dysplasia : Delineation of the clinical phenotype

    NARCIS (Netherlands)

    Wade, Emma M.; Jenkins, Zandra A.; Daniel, Philip B.; Morgan, Tim; Addor, Marie C.; Ades, Lesley C.; Bertola, Debora; Bohring, Axel; Carter, Erin; Cho, Tae-Joon; de Geus, Christa M.; Duba, Hans-Christoph; Fletcher, Elaine; Hadzsiev, Kinga; Hennekam, Raoul C. M.; Kim, Chong A.; Krakow, Deborah; Morava, Eva; Neuhann, Teresa; Sillence, David; Superti-Furga, Andrea; Veenstra-Knol, Hermine E.; Wieczorek, Dagmar; Wilson, Louise C.; Markie, David M.; Robertson, Stephen P.

    Frontometaphyseal dysplasia (FMD) is caused by gain-of-function mutations in the X-linked gene FLNA in approximately 50% of patients. Recently we characterized an autosomal dominant form of FMD (AD-FMD) caused by mutations in MAP3K7, which accounts for the condition in the majority of patients who

  10. Avascular necrosis of the hip in multiple epiphyseal dysplasia

    International Nuclear Information System (INIS)

    Mackenzie, W.G.; Bassett, G.S.; Mandell, G.A.; Scott, C.I. Jr.

    1989-01-01

    We observed radiographic changes of avascular necrosis (AVN) of the capital femoral epiphysis in 9 hips of 11 patients with multiple epiphyseal dysplasia (MED). Plain roentgenography, bone scintigraphy, and magnetic resonance imaging (MRI) studies all revealed characteristic asymmetric changes in the presence of AVN superimposed on dysplastic femoral heads

  11. Craniofacial and temporal bone CT findings in cleidocranial dysplasia

    International Nuclear Information System (INIS)

    Gonzalez, Guido E.; Caruso, Paul A.; Curtin, Hugh D.; Small, Juan E.; Jyung, Robert W.; Troulis, Maria J.

    2008-01-01

    Cleidocranial dysplasia (CCD) is a multistructural polyostotic genetic disorder that results from mutation of the CBFA1 gene. Hearing loss is a frequent finding in CCD. We describe the CT craniofacial findings in CCD and provide a comprehensive discussion of the CT temporal bone findings in these patients. (orig.)

  12. Craniofacial and temporal bone CT findings in cleidocranial dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Gonzalez, Guido E. [Massachusetts General Hospital and Harvard Medical School, Department of Radiology, Boston, MA (United States); Clinica Alemana de Santiago, Departamento de Imagenes, Santiago (Chile); Caruso, Paul A.; Curtin, Hugh D. [Massachusetts Eye and Ear Infirmary and Harvard Medical School, Department of Radiology, Boston, MA (United States); Small, Juan E. [Massachusetts General Hospital and Harvard Medical School, Department of Radiology, Boston, MA (United States); Massachusetts Eye and Ear Infirmary and Harvard Medical School, Department of Radiology, Boston, MA (United States); Jyung, Robert W. [Massachusetts Eye and Ear Infirmary and Harvard Medical School, Department of Otology, Boston, MA (United States); Troulis, Maria J. [Massachusetts General Hospital and Harvard Medical School, Department of Oral and Maxillofacial Surgery, Boston, MA (United States)

    2008-08-15

    Cleidocranial dysplasia (CCD) is a multistructural polyostotic genetic disorder that results from mutation of the CBFA1 gene. Hearing loss is a frequent finding in CCD. We describe the CT craniofacial findings in CCD and provide a comprehensive discussion of the CT temporal bone findings in these patients. (orig.)

  13. Pathophysiology, diagnosis, and treatment of canine hip dysplasia

    International Nuclear Information System (INIS)

    Cook, J.L.; Tomlinson, J.L.; Constantinescu, G.M.

    1996-01-01

    Dogs with hip dysplasia are commonly presented to veterinarians for evaluation. Although many causes of the condition have been proposed, a definitive cause has not been established. The multifactorial nature of canine hip dysplasia can confuse client education and management ofthe disease. The basic concept involved is the biomechanical imbalance between the forces on the coxofemoral joint and the associated muscle mass; the result is joint laxity in young, growing dogs. This laxity leads to incongruity; the eventual result is degenerative joint disease. Canine hip dysplasia can affect any breed but is most often reported in large and giant breeds. Understanding the pathophysiology and biomechanics involved with this developmental disease is important in providing clients with diagnostic, therapeutic, and prognostic information. The selection of treatment is influenced by the following factors:the age, health, and intended use of the patient; clinical signs; diagnostic findings; the availability of treatment; and the financial constraints of the owner. This article discusses the current concepts concerning the pathophysiology and biomechanics of canine hip dysplasia and outlines diagnostic and therapeutic options. The objective of the article is to provide practitioners with a reference for decision making and client education

  14. Prevalence of Cervical Dysplasia and Associated Risk Factors ...

    African Journals Online (AJOL)

    Background: Cervical cancer is a global burden, with increasing incidence and mortality especially in developing countries where there are no clearly define national protocols or guidelines for effective screening and prompt treatment of cervical dysplasia (precancerous condition of the cervix). Most screening facilities are ...

  15. Tricuspid valve dysplasia: A retrospective study of clinical features ...

    African Journals Online (AJOL)

    Dogs were most frequently referred for investigation of heart murmur. The most common arrhythmia was atrial fibrillation (n=3). Median survival time from diagnosis of tricuspid valve dysplasia was 2775 days (range 1-3696 days; 95% CI 1542.41-4007.59) and from onset of right-sided congestive heart failure was 181 days ...

  16. Focal fibrocartilaginous dysplasia and tibia vara: a case report

    International Nuclear Information System (INIS)

    Cockshott, W.P.; Martin, R.; Friedman, L.; Yuen, M.

    1994-01-01

    A 2-year-old black boy with focal fibrocartilaginous dysplasia is described with illustrations of the typical radiographic appearances supplemented by computed tomographic and magnetic resonance images. Since this rare condition is self-correcting, diagnosis is important so that surgical intervention and biopsy can be avoided and conservative management instituted. (orig.)

  17. Caudal regression with sirenomelia and dysplasia renofacialis (Potter's syndrome)

    International Nuclear Information System (INIS)

    Noeldge, G.; Billmann, P.; Boehm, N.; Freiburg Univ.

    1982-01-01

    A case of caudal regression in combination with sirenomelia and dysplasia renofacialis (Potter's syndrome) is reported. The formal pathogenesis of these malformations and clinical facts are shown and discussed. Findings of plain films, postmortal angiography and pathologic-anatomical changes are demonstrated. (orig.) [de

  18. Genetic heterogeneity in spondylo-epimetaphyseal dysplasias: a ...

    African Journals Online (AJOL)

    Introduction: Spondylo-epi-metaphyseal dysplasias (SEMD) are a heterogeneous group of skeletal disorders characterized by defective growth and modeling of the spine and long bones. Different types are described in the literature. Accurate classification of SEMDs is essential for proper genetic counseling. Patients and ...

  19. Corrections of lower limb deformities in patients with diastrophic dysplasia.

    Science.gov (United States)

    Al Kaissi, Ali; Kenis, Vladimir; Melchenko, Eugeniy; Chehida, Farid Ben; Ganger, Rudolf; Klaushofer, Klaus; Grill, Franz

    2014-11-01

    Accurate understanding of the cause of the underlying pathology in children with diastrophic dysplasia would help in designing targeted management of their locomotion. Diastrophic dysplasia was diagnosed in twelve patients (nine girls and three boys; age range 1-14 years), all of whom presented with small stature and apparent short extremities. Club foot (mostly talipes equinovarus) was the most frequent and consistent abnormality. Concomitant abnormalities such as hip flexion contracture, flexion contractures of the knees with excessive valgus deformity and lateral patellar subluxation, were also encountered. Muscle ultrasound and muscle magnetic resonance imaging imaging showed no myopathic changes and muscle biopsies and the respiratory chain were normal. Serum choline kinase and plasma lactate concentrations were normal. Surgical correction of the foot and ankle in patients with diastrophic dysplasia is extremely difficult because of the markedly distorted anatomy. In all of these children, plantigrade foot was achieved along with the improved function of the locomotor system. Mutations of the diastrophic dysplasia sulfate transporter (also known as solute carrier family 26 member 2) were encountered. Arthrogryposis multiplex is the usual terminology used to describe the abnormality in infants with multiple contractures. Diligent orthopaedic care should be provided based on an accurate understanding of the associated syndromes in such children. © 2014 Chinese Orthopaedic Association and Wiley Publishing Asia Pty Ltd.

  20. Unilateral Closed Lip Schizencephaly with Septo‑Optic Dysplasia ...

    African Journals Online (AJOL)

    ... be associated with septo‑optic dysplasia (SOD), optic nerve hypoplasia and absence of septum pellucidum, pachygyria, polymicrogyria, heterotopia and arachnoid cysts. We report a case of unilateral closed lip schizencephaly with SOD. Keywords: Absence of septum pellucidum, Optic nerve hypoplasia, Schizencephaly, ...

  1. Prevalence of Cervical Dysplasia and Associated Risk Factors ...

    African Journals Online (AJOL)

    Background: Cervical cancer is a global burden, with increasing incidence and mortality especially in developing countries where there are no clearly define national protocols or guidelines for effective screening and prompt treatment of cervical dysplasia. (precancerous condition of the cervix). Most screening facilities are ...

  2. Hip dysplasia in a 6-year-old Salz ram

    OpenAIRE

    Loste, Araceli; Ramos, Juan José; Sáez, Teófilo; Sever, Ramón; Marca, Carmen

    2003-01-01

    A 6-year old Salz ram was presented with a history of poor body condition, progressive gait abnormalities, pelvic limb lameness, and difficulty with copulation. Based on the history, clinical signs, hip palpation, and radiography, a diagnosis of hip dysplasia, previously unreported in sheep, was made.

  3. Canine hip dysplasia: significance of early bony spurring

    International Nuclear Information System (INIS)

    Morgan, J.P.

    1987-01-01

    It is the purpose of this study to call attention to new bone production that often occurs early in the sequence of pathological changes associated with canine hip dysplasia. New bone production extending to bony remodeling, as well as femoral head subluxation, both occur in the sequence of pathologic changes associated with canine hip dysplasia. Subluxation is considered primary, while osteoarthrosis is a secondary feature, and both are used in the diagnosis of canine hip dysplasia. This report concerns the significance of the presence of a solitary bony osteophyte, or spur, that is frequently evident on the caudal aspect of the femoral neck as viewed on the conventional ventrodorsal projection. This report utilizes findings from pelvic radiographs of 605 dogs (five breeds). There was a greater frequency (54%)of this bony change in cases diagnosed radiographically as dysplastic than in cases diagnosed as normal(15%).Thus, it is suggested that this minimal radiographic change can be used as an indicator of early canine hip dysplasia, especially in the absence of subluxation of the femoral head

  4. Prenatal 3D Ultrasound Diagnostics in Cleidocranial Dysplasia

    DEFF Research Database (Denmark)

    Hermann, NV; Hove, HD; Jørgensen, C

    2009-01-01

    A 34-year-old Caucasian woman with cleidocranial dysplasia (CCD) and a known family history of CCD was referred for an ultrasound examination in the first trimester of her second pregnancy. Molecular genetic analysis of the RUNX2 gene was non-informative. A routine 2D ultrasound examination carried...

  5. Reducing tobacco smoking and smoke exposure to prevent preterm birth and its complications.

    Science.gov (United States)

    Wagijo, Mary-Ann; Sheikh, Aziz; Duijts, Liesbeth; Been, Jasper V

    2017-03-01

    Tobacco smoking and smoke exposure during pregnancy are associated with a range of adverse health outcomes, including preterm birth. Also, children born preterm have a higher risk of complications including bronchopulmonary dysplasia and asthma when their mothers smoked during pregnancy. Smoking cessation in early pregnancy can help reduce the adverse impact on offspring health. Counselling interventions are effective in promoting smoking cessation and reducing the incidence of preterm birth. Peer support and incentive-based approaches are likely to be of additional benefit, whereas the effectiveness of pharmacological interventions, including nicotine replacement therapy, has not definitely been established. Smoke-free legislation can help reduce smoke exposure as well as maternal smoking rates at a population level, and is associated with a reduction in preterm birth. Helping future mothers to stop smoking and protect their children from second hand smoke exposure must be a key priority for health care workers and policy makers alike. Copyright © 2015 Elsevier Ltd. All rights reserved.

  6. Abduction bracing for residual acetabular dysplasia in infantile DDH.

    Science.gov (United States)

    Gans, Itai; Flynn, John M; Sankar, Wudbhav N

    2013-01-01

    Abduction bracing is often used to treat residual acetabular dysplasia in infants whose acetabular indices (AI) exceed 30 degrees after 6 months of age. However, little data exist to support this practice. The purpose of this study was to determine the efficacy of part-time abduction bracing in treating residual acetabular dysplasia by comparing a cohort of braced infants with a cohort of unbraced infants. We performed a retrospective review of a consecutive series of patients with developmental dysplasia of the hip (DDH) treated at our institution over 4 years. Children with stable, treated DDH but residual acetabular dysplasia at 6 months of age were identified; those with available anteroposterior pelvic radiographs at 6 months and 1 year of age were included. Patients who required open surgical reduction and those with syndromic or neuromuscular diagnoses were excluded. On the basis of practice variations at our institution, some orthopaedists start bracing when the 6-month radiograph demonstrates an AI≥30 degrees, whereas others do not; we compared these 2 cohorts. Braced patients were instructed to wear an abduction orthosis during nights and naps until follow-up at 1 year of age. The AI at 6 months and 1 year of age for both cohorts were then measured by a single observer and the differences compared. Seventy-six hips in 52 patients were identified with residual dysplasia on the 6-month radiograph. Thirty-nine hips (27 patients) were unbraced, 31 hips (21 patients) were braced, and 6 hips (4 patients) were excluded for cross-over. Over a 6-month period, the braced cohort had significantly better improvement in the AI of 5.3 degrees (95% confidence interval, 4.3 to 6.3 degrees) compared to the unbraced cohort which had an improvement in the AI of only 1.1 degrees (95% confidence interval 0.6 to 1.6 degrees) (PDDH. Level III-retrospective comparative study.

  7. Photodynamic Therapy for Head and Neck Dysplasia and Cancer

    Science.gov (United States)

    Rigual, Nestor R.; Thankappan, Krishnakumar; Cooper, Michele; Sullivan, Maureen A.; Dougherty, Thomas; Popat, Saurin R.; Loree, Thom R.; Biel, Merrill A.; Henderson, Barbara

    2009-01-01

    Objective To determine the response of dysplasia, carcinoma in situ (CIS), and T1 carcinoma of the oral cavity and larynx to photodynamic therapy with porfimer sodium. Design Prospective trial. Setting A National Cancer Institute–designated cancer institute. Patients Patients with primary or recurrent moderate to severe oral or laryngeal dysplasia, CIS, or T1N0 carcinoma. Intervention Porfimer sodium, 2 mg/kg of body weight, was injected intravenously 48 hours before treatment. Light at 630 nm for photosensitizer activation was delivered from an argon laser or diode laser using lens or cylindrical diffuser fibers. The light dose was 50 J/cm2 for dysplasia and CIS and 75 J/cm2 for carcinoma. Main Outcome Measures Response was evaluated at 1 week and at 1 month and then at 3-month intervals thereafter. Response options were complete (CR), partial (PR), and no (NR) response. Posttreatment biopsies were performed in all patients with persistent and recurrent visible lesions. Results Thirty patients were enrolled, and 26 were evaluable. Mean follow-up was 15 months (range, 7–52 months). Twenty-four patients had a CR, 1 had a PR, and 1 had NR. Three patients with oral dysplasia with an initial CR experienced recurrence in the treatment field. All the patients with NR, a PR, or recurrence after an initial CR underwent salvage treatment. Temporary morbidities included edema, pain, hoarseness, and skin phototoxicity. Conclusion Photodynamic therapy with porfimer sodium is an effective treatment alternative, with no permanent sequelae, for oral and laryngeal dysplasia and early carcinoma. PMID:19687399

  8. Combined Allergic Bronchopulmonary Aspergillosis and Eosinophilic Granulomatosis with Polyangiitis: Three Cases and a Review of the Literature.

    Science.gov (United States)

    Ishiguro, Takashi; Takayanagi, Noboru; Takaku, Yotaro; Kagiyama, Naho; Kurashima, Kazuyoshi; Sugita, Yutaka

    2016-01-01

    Previous reports of combined allergic bronchopulmonary mycosis (ABPM) and eosinophilic granulomatosis with polyangiitis (EGPA) are limited; however, among 41 cases of ABPM and 18 cases of EGPA treated at our hospital, we experienced three cases of combined ABPM and EGPA. In two of these cases, the diagnosis of EGPA preceded that of ABPM, however, one of the two cases had already shown findings suggestive of ABPM, such as mucous plugs and central bronchiectasis, at the time of the diagnosis of EGPA. In six previously reported cases of combined ABPM and EGPA, ABPM preceded EGPA in four cases. In the other two cases in which EGPA was diagnosed before or simultaneously with ABPM, findings suggestive of ABPM had been detected when EGPA was diagnosed, which suggests that sensitization to fungi resulting in ABPM may play an important role in the development of EGPA. Careful attention should therefore be paid to the possibility that these diseases may coexist during the course of either disease.

  9. Prevalence and co-occurrence of hip dysplasia and elbow dysplasia in Dutch pure-bred dogs

    NARCIS (Netherlands)

    Lavrijsen, I.C.M.; Heuven, H.C.M.; Meij, B.P.; Theyse, L.F.H.; Nap, R.C.; Leegwater, P.A.J.; Hazewinkel, H.A.W.

    2014-01-01

    Hip as well as elbow dysplasia (HD, ED) are developmental disorders leading to malformation of their respective joints. For a long time both disorders have been scored and targeted for improvement using selective breeding in several Dutch dog populations. In this paper all scores for both HD and ED,

  10. Prevalence and co-occurrence of hip dysplasia and elbow dysplasia in Dutch pure-bred dogs

    NARCIS (Netherlands)

    Lavrijsen, Ineke; Heuven, Henri; Meij, Bjorn; Theyse, Lars; Nap, R.C.; Leegwater, Peter; Hazewinkel, Herman

    2014-01-01

    tHip as well as elbow dysplasia (HD, ED) are developmental disorders leading to malforma-tion of their respective joints. For a long time both disorders have been scored and targetedfor improvement using selective breeding in several Dutch dog populations. In this paperall scores for both HD and ED,

  11. Bronchopulmonary lavage and DTPA treatment for the removal of inhaled 239Pu of varied solubility in beagle dogs. II

    International Nuclear Information System (INIS)

    Muggenburg, B.A.; Mewhinney, J.A.; Miglio, J.J.; Slauson, D.O.; McClellan, R.O.

    1974-01-01

    The efficacy of bronchopulmonary lavage and chelation therapy was determined for removing 239 Pu from Beagle dogs after inhalation of 239 Pu aerosols of differing in vivo solubility. The four aerosols used were nebulized from a solution of 239 PuCl 4 and heat treated at temperatures of 325, 600, 900, and 1150 0 C, respectively. Six dogs were exposed to each of the four aerosols and three dogs in each group were treated subsequently by lavage and intravenous diethylenetriaminepentaacetic acid (DTPA); three dogs served as untreated controls. Tissue accumulation of 239 Pu in the untreated control dogs at sacrifice 56 days post-exposure, expressed as a percentage of the initial lung burden (ILB), was 6 percent in liver and 9 percent in skeleton for the 325 0 C aerosol group, 1 percent in liver and 2 percent in the skeleton for the 600 0 C group, and less than 0.6 percent in these tissues for the 900 0 and 1150 0 C aerosol groups. Tissue accumulation was 1.0 percent or less of the ILB for all organs in the treated groups of dogs. The urinary excretion of 239 Pu was increased in the treated dogs compared to the control dogs that inhaled the 325 0 C and 600 0 C aerosols and was low in all dogs exposed to the 900 0 and 1150 0 C treated aerosol particles. Ten bronchopulmonary lavage procedures removed a mean of 44 percent of the ILB of 239 Pu from the lungs. The aerosol temperature and resulting differences in solubility of the particles did not influence the efficacy of the lavage procedure. An in vitro solubility test predicted the relative in vivo solubility of the four aerosols. These results are discussed in relation to the choice of therapy and its timing. (U.S.)

  12. Evaluation and Referral for Developmental Dysplasia of the Hip in Infants.

    Science.gov (United States)

    Shaw, Brian A; Segal, Lee S

    2016-12-01

    Developmental dysplasia of the hip (DDH) encompasses a wide spectrum of clinical severity, from mild developmental abnormalities to frank dislocation. Clinical hip instability occurs in 1% to 2% of full-term infants, and up to 15% have hip instability or hip immaturity detectable by imaging studies. Hip dysplasia is the most common cause of hip arthritis in women younger than 40 years and accounts for 5% to 10% of all total hip replacements in the United States. Newborn and periodic screening have been practiced for decades, because DDH is clinically silent during the first year of life, can be treated more effectively if detected early, and can have severe consequences if left untreated. However, screening programs and techniques are not uniform, and there is little evidence-based literature to support current practice, leading to controversy. Recent literature shows that many mild forms of DDH resolve without treatment, and there is a lack of agreement on ultrasonographic diagnostic criteria for DDH as a disease versus developmental variations. The American Academy of Pediatrics has not published any policy statements on DDH since its 2000 clinical practice guideline and accompanying technical report. Developments since then include a controversial US Preventive Services Task Force "inconclusive" determination regarding usefulness of DDH screening, several prospective studies supporting observation over treatment of minor ultrasonographic hip variations, and a recent evidence-based clinical practice guideline from the American Academy of Orthopaedic Surgeons on the detection and management of DDH in infants 0 to 6 months of age. The purpose of this clinical report was to provide literature-based updated direction for the clinician in screening and referral for DDH, with the primary goal of preventing and/or detecting a dislocated hip by 6 to 12 months of age in an otherwise healthy child, understanding that no screening program has eliminated late development or

  13. Increased nuclear β-catenin expression in oral potentially malignant lesions: A marker of epithelial dysplasia.

    Science.gov (United States)

    Reyes, Montserrat; Rojas-Alcayaga, Gonzalo; Maturana, Andrea; Aitken, Juan-Pablo; Rojas, Carolina; Ortega, Ana-Verónica

    2015-09-01

    Deregulation of β-catenin is associated with malignant transformation; however, its relationship with potentially malignant and malignant oral processes is not fully understood. The aim of this study was to determine and compare the nuclear β-catenin expression in oral dysplasia and oral squamous cell carcinoma (OSCC). Cross sectional study. Immunodetection of β-catenin was performed on 72 samples, with the following distribution: 21 mild dysplasia, 12 moderate dysplasia, severe dysplasia 3, 36 OSCC including 19 well differentiated, 15 moderately differentiated and 2 poorly differentiated. Through microscopic observation the number of positive cells per 1000 epithelial cells was counted. For the statistical analysis, the Kruskal Wallis test was used. Nuclear expression of β-catenin was observed in all samples with severe and moderate dysplasia, with a median of 267.5, in comparison to mild dysplasia whose median was 103.75. Only 10 samples (27.7%) with OSCC showed nuclear expression, with statistically significant differences between groups (p < 0.05). Our results are consistent with most of the reports which show increased presence of β-catenin in severe and moderate dysplasia compared to mild dysplasia; however the expression of nuclear β-catenin decreased after starting the invasive neoplastic process. This suggests a role for this protein in the progression of dysplasia and early malignant transformation to OSCC. Immunodetection of β-catenin could be a possible immune marker in the detection of oral dysplasia.

  14. Angiotensin II type 2 receptor ligand PD123319 attenuates hyperoxia-induced lung and heart injury at a low dose in newborn rats

    NARCIS (Netherlands)

    Wagenaar, Gerry T M; Sengers, Rozemarijn M A; Laghmani, El Houari; Chen, Xueyu; Lindeboom, Melissa P H A; Roks, Anton J M; Folkerts, Gert; Walther, Frans J

    2014-01-01

    Intervening in angiotensin (Ang)-II type 2 receptor (AT2) signaling may have therapeutic potential for bronchopulmonary dysplasia (BPD) by attenuating lung inflammation and preventing arterial hypertension (PAH)-induced right ventricular hypertrophy (RVH). We first investigated the role of AT2

  15. Evidence-based neonatal unit practices and determinants of Postnatal corticosteroid-use in preterm births below 30 weeks ga in Europe : A population-based cohort study

    NARCIS (Netherlands)

    Nuytten, Alexandra; Behal, Hélène; Duhamel, Alain; Jarreau, Pierre Henri; Mazela, Jan; Milligan, David; Gortner, Ludwig; Piedvache, Aurélie; Zeitlin, Jennifer; Truffert, Patrick; Martens, E.; Martens, G.; Van Reempts, P.; Boerch, K.; Hasselager, A.; Huusom, L.; Pryds, O.; Weber, T.; Toome, L.; Varendi, H.; Ancel, P. Y.; Blondel, B.; Burguet, A.; Jarreau, P. H.; Truffert, P.; Maier, R. F.; Misselwitz, B.; Schmidt, S.; Gortner, L.; Baronciani, D.; Gargano, G.; Agostino, R.; DiLallo, D.; Franco, F.; Carnielli, V.; Koopman-Esseboom, C.|info:eu-repo/dai/nl/14117739X; Van Heijst, A.; Nijman, J.; Gadzinowski, J.; Mazela, J.; Graça, L. M.; Machado, M. C.; Rodrigues, T.; Barros, H.; Bonamy, A. K.; Norman, M.; Wilson, E.; Boyle, E.; Draper, E. S.; Manktelow, B. N.; Fenton, A. C.; Milligan, D. W A; Zeitlin, J.; Bonet, M.; Piedvache, A.

    2017-01-01

    Background: Postnatal corticosteroids (PNC) were widely used to treat and prevent bronchopulmonary dysplasia in preterm infants until studies showed increased risk of cerebral palsy and neurodevelopmental impairment. We aimed to describe PNC use in Europe and evaluate the determinants of their use,

  16. Deficiency or inhibition of lysophosphatidic acid receptor 1 protects against hyperoxia-induced lung injury in neonatal rats

    NARCIS (Netherlands)

    Chen, X.; Walther, F. J.; van Boxtel, R.|info:eu-repo/dai/nl/344456110; Laghmani, E. H.; Sengers, R. M. A.; Folkerts, G.; DeRuiter, M. C.; Cuppen, E.|info:eu-repo/dai/nl/183050487; Wagenaar, G. T. M.

    Aim Blocking of lysophosphatidic acid (LPA) receptor (LPAR) 1 may be a novel therapeutic option for bronchopulmonary dysplasia (BPD) by preventing the LPAR1-mediated adverse effects of its ligand (LPA), consisting of lung inflammation, pulmonary arterial hypertension (PAH) and fibrosis. Methods In

  17. A giant cranial aneurysmal bone cyst associated with fibrous dysplasia.

    Science.gov (United States)

    Składzieriń, J; Olés, K; Zagólski, O; Moskała, M; Sztuka, M; Strek, P; Wierzchowski, W; Tomik, J

    2008-01-01

    An aneurysmal bone cyst (ABC) is a rare, benign fibro-osseous lesion, considered a vascular phenomenon secondary to fibrous dysplasia or a giant-cell tumour, and occurs mainly in long bones and vertebrae. In this case report a 16-year-old male presented with massive epistaxis. He was admitted with a 3-year history of chronic rhinitis, headaches, right ocular pain and recurrent epistaxis. CT scans showed a predominantly cystic, expansive mass obstructing both nasal cavities, extending to all paranasal sinuses and both orbits, with evidence of anterior cranial fossa skull base destruction. The patient underwent a craniofacial resection of the tumour performed with an external approach and an immediate reconstruction of the dural defect. Histology confirmed the lesion was an ABC associated with fibrous dysplasia. The patient's recovery was complete. A large facial aneurysmal bone cyst can damage the facial skeleton and skull base, and requires excision by a combined external approach.

  18. Spontaneous involution of diffuse fibrous dysplasia of paranasal sinuses.

    Science.gov (United States)

    De Carlo, D; Zotto, L Dal; Carollo, C; Porzionato, A; D'Avella, D; Pomerri, F; Battistella, P A

    2015-05-01

    We report the case of a 25-year-old patient, diagnosed at age 10, with diffuse fibrous dysplasia of the paranasal sinuses, an extremely rare idiopathic condition. This diagnosis is possible only by cerebral computed tomography (CT), cerebral and anterior skull base magnetic resonance imaging (MRI), and histopathology. Surgical treatment is common. This boy had mild symptoms: moderate headache in the morning that did not affect his daily activity, and rhinitis, partially responsive to medication. The neurologic examination was abnormal. Radiographs, CT, and MRI showed a diffuse mass in the paranasal sinuses which had a histopathological diagnosis of fibrous dysplasias. The family refused to refer the patient to surgery. The boy has been monitored annually for 15 years. He has remained asymptomatic without headache since age 11, with normal, general and neurologic examinations. Serial MRIs showed a spontaneous partial involution of the mass. © The Author(s) 2014.

  19. Squamous cell dysplasia and carcinoma of the conjunctiva

    DEFF Research Database (Denmark)

    Ramberg, Ingvild; Heegaard, Steffen; Prause, Jan Ulrik

    2015-01-01

    %) had epithelial dysplasia, 19 (13%) had carcinoma in situ, and 29 (20%) had squamous cell carcinoma. A significantly higher proportion of men were found. The median age at diagnosis was 65 years. The risk of recurrence was 10.0% [95% confidence interval (CI): 5.0–15.0] after 1 year and 17.2% (95% CI......Purpose To investigate the epidemiology of squamous cell dysplasia and carcinoma of the conjunctiva in Denmark. Methods Review of the histopathological case reports at the Eye Pathology Institute (EPI), University of Copenhagen, and the National Danish Pathology Bank from 1980 to 2011. Information...... regarding distribution of age and sex, localization, earlier pathology, comorbidity and recurrence of the condition was registered. The Cause of Death Registry at Statens Serum Institut was used to obtain information regarding cause of death. Results A total of 143 cases were identified. Ninety-five (61...

  20. Angiographic diagnosis of fibromuscular dysplasia of the renal arteries

    International Nuclear Information System (INIS)

    Petkov, B.; Grigorov, G.; Nedelkov, G.

    1982-01-01

    In the nosological structure of renovascular hypertensions fibromuscular dysplasia of the renal arteries by incidence ranks second after atherosclerosis. The illness affects mainly women, more frequently young and having borne a child. Hypertension in such patients is usually characterized by high values of the diastolic pressure, and leads to early neurologic complaints such as headache, sight impairment, vertigo and Meniere-like syndromes. Morphological and functional changes are likewise described. Some of the literature statements concerning the etiopathogenesis and classification of fibromuscular dysplasia are critically assayed. The success of the surgical management depends on the timely established exact diagnosis, and angiography appears to be the only method of primary importance in this respect. (author)