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Sample records for premature bronchopulmonary dysplasia

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

    NARCIS (Netherlands)

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

    2010-01-01

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

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

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

    2014-01-01

    Purpose Eosinophilia is common in premature infants, and its incidence increases with a shorter gestation period. We investigated the clinical significance of eosinophilia in premature infants born at Eosinophilia was defined as an eosinophil percentage of >3% of the total leukocytes. Perinatal parameters and clinical parameters were also analyzed. Results Of the 261 infants born at eosinophilia at birth. The eosinophil percentage peaked in the fourth postnatal week at 7.5%. The incidence of severe eosinophilia increased after birth up to the fourth postnatal week when 8.8% of all patients had severe eosinophilia. Severity of eosinophilia was positively correlated with a lower gestational age, birth weight, and Apgar score. Respiratory distress syndrome, bronchopulmonary dysplasia, nephrocalcinosis, intraventricular hemorrhage, and sepsis were associated with a higher eosinophil percentage. The eosinophil percentage was significantly higher in infants with bronchopulmonary dysplasia from the first postnatal week and the percentage was the highest in the fourth postnatal week, with the maximal difference being 4.1% (PEosinophilia is common in premature infants and reaches peak incidence and severity in the fourth postnatal week. The eosinophil percentage was significantly higher in bronchopulmonary dysplasia patients from the first postnatal week. Severe eosinophilia was significantly associated with the incidence of bronchopulmonary dysplasia even after adjusting for other variables. PMID:24868214

  3. 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)

  4. 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.

  5. 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

  6. Displasia broncopulmonar Bronchopulmonary dysplasia

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    Luciana F. Velloso Monte

    2005-04-01

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

  7. Ureaplasma in lung. 2. Association with bronchopulmonary dysplasia in premature newborns.

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    Benstein, Barbara D; Crouse, Dennis T; Shanklin, D Radford; Ourth, Donald D

    2003-10-01

    Infants with Ureaplasma urealyticum in the lower respiratory tract are at risk for chronic lung disease (CLD) or bronchopulmonary dysplasia (BPD) but causality has been difficult to prove. The goal of this study was to identify ureaplasma in human neonatal lung tissue using the in situ hybridization (ISH) procedure described in Part 1 (Exp. Mol. Pathol., in press) of this report. By correlating their presence with the histopathologic findings, it may be possible to provide further evidence of the pathogenicity of ureaplasmas and their association with BPD. Lung autopsy tissue from seven infants with positive cultures and seven infants with negative cultures for ureaplasma were included in the study. All culture-positive infants were positive for ureaplasma on ISH and all had histopathologic evidence of BPD. Two of the seven infants with negative cultures were positive for ureaplasma with ISH. Of interest, these two infants were also found to have BPD at autopsy. The other five infants with negative cultures were also negative for ureaplasma on ISH and had no evidence of BPD. This study correlates the presence of U. urealyticum by ISH with the finding of BPD on histopathologic evaluation and provides evidence that it has a role in the development of CLD.

  8. Clinical Analysis of Premature Infants with Bronchopulmonary Dysplasia%早产儿支气管肺发育不良症的临床分析

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    欧伟明; 张金凤; 张菊弟

    2015-01-01

    Objective To investigate the clinical features of preterm infants with bronchopulmonary dysplasia.Methods In our hospital were retrospectively analyzed clinical data of 48 cases of preterm infants with bronch-opulmonary dysplasia in children.Results 48 patients of 44 patients gradualy improved,hospitalized from 21 to 33 d after discharge;4 cases died,the mortality time 10~30 d,cause of death: 1 cases of pulmonary infection,2 cases of chronic respiratory failure,1 cases of pneumothorax.Conclusion Premature for bronchopulmonary dysplasia of newborn disease risk factors,should strengthen the prevention,a reasonable set of ventilator parameters,avoid the occurrence of complications such as infection.%目的 探讨早产儿支气管肺发育不良症的临床特点.方法 回顾性分析我院48例早产儿支气管肺发育不良症患儿的临床资料.结果 48例患儿中44例逐渐好转,住院21~33 d后出院;4例患儿病死,病死时间10~30 d,病死原因:肺部感染1例,慢性呼吸衰竭2例,气胸1例.结论 早产为新生儿支气管肺发育不良症的危险因素,临床应加强预防,合理设置呼吸机参数,避免感染等并发症出现.

  9. Progress of Treatment of Bronchopulmonary Dysplasia in Premature Infants%早产儿支气管肺发育不良的治疗进展

    Institute of Scientific and Technical Information of China (English)

    黄婕; 郑直

    2013-01-01

    Bronchopulmonary dysplasia( BPd) is currently one of the most common complications of premature infants,which has become one of the most difficult problems in neonatal intensive care unit.The pathogenesis of BPd is extremely complex, so that there is no effective treatment at present.This paper has introduced the latest definition,and progress of prevention and treatment of BPd in premature infants both in domestic and foreign.%早产儿支气管肺发育不良(broncho-pulmonary dysplasia,BPd )是目前早产儿最常见的并发症之一,已经成为新生儿重症监护病房最棘手的问题之一。BPd的发病机制极其复杂,目前尚无有效的治疗方法。现主要介绍国内外有关早产儿BPd的定义、预防及治疗新进展。

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

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    Danieli Dias Gonçalves

    2010-10-01

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

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

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    V. K. Pozharishchenskaya

    2017-01-01

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

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

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

    2017-06-01

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

  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 infan

  14. 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.

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

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    Lilian Beani

    2007-12-01

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

  16. 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.

  17. 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.

  18. Impact of bronchopulmonary dysplasia on brain and retina.

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    Poon, Annie Wing Hoi; Ma, Emilie Xiao Hang; Vadivel, Arul; Jung, Suna; Khoja, Zehra; Stephens, Laurel; Thébaud, Bernard; Wintermark, Pia

    2016-04-15

    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.

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

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    Paula Eylla Cristina Rodrigues Duarte

    2012-06-01

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

  20. 早产儿支气管肺发育不良危险因素前瞻性队列研究%Prospective cohort study on risk factor for bronchopulmonary dysplasia in the premature infant

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    钟美珍; 白海涛; 刘登礼; 王礼周; 庄德义

    2011-01-01

    目的 探讨早产儿支气管肺发育不良(BPD)的发生率及危险因素.方法 应用前瞻性队列研究的方法,对我院产科2009年1月至2010年6月出生的所有活产早产儿进行研究,统计BPD发生率,并分析相关危险因素.结果 (1)共有425例早产儿入组研究,其中男266例,女159例;胎龄(33.9±2.4)周(26~ 36周);出生体重(2038±660) g(770 ~3150 g),其中极低出生体重儿85例,超低出生体重儿7例.发生BPD 45例,发生率10.6%,死亡(包括自动出院撤机后死亡)7例,BPD病死率15.6%.BPD患儿胎龄≤32周36例(80.0%),出生体重<1500 g 29例(64.4%).(2)多因素Logistic回归分析显示胎龄<30周(OR =3.10)、出生体重<1500 g(OR=2.29)、感染性肺炎(OR =2.74)、动脉导管未闭(OR =2.12)、机械通气(OR =9.57)、H2受体抑制剂( OR=1.36)、应用碳青霉烯类抗生素>4周(OR=2.59)是BPD发病的独立危险因素(P均<0.05).结论 早产儿发生BPD的独立危险因素较多,需要综合防治才能有效控制BPD的发生.%Objective To investigate the incidence and the risk factors for bronchopulmonary dysplasia ( BPD) in premature infants in Xiamen. Methods This was a single-center prospective clinical cohort study. A total of 425 premature infants in the first hospital affiliated to Xiamen university, between January 1,2009 and June 01,2010, were enrolled. Measurements of the demographic and potential risk factors reflecting illness severity were performed. Results ( 1 ) Among 425 cases of premature infants, 266 (62.6%) cases are male, and 159 (37.4%) cases are female, with average gestational age ( 33. 9 ± 2. 4) wk, and birth weight 750 ~ 2800 g, with an average of (1820 ± 462 ) g, of which 97 cases were of very low birth weight infants. Among these infants, there were 45 BPD cases with the incidence of 10. 6% , 7 of them were death cases (including the deaths after automatic discharge from hospital and weaning mechanical ventilation) with the rough

  1. Expert system supporting an early prediction of the bronchopulmonary dysplasia.

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    Ochab, Marcin; Wajs, Wiesław

    2016-02-01

    This work presents a decision support system which uses machine learning to support early prediction of bronchopulmonary dysplasia (BPD) for extremely premature infants after their first week of life. For that purpose a knowledge database was created based on the historical data gathered including data on 109 patients with birth weight less than or equal to 1500g. The core of the database consists of support vector machine and logit regression classification results calculated specifically for that system, and obtained by considering 2(14) different combinations of 14 risk factors. Based on the results obtained and user demands, the system recommends the best methods and the most suitable parameter subset among those currently available to the user. The program is also able to estimate the accuracy, sensitivity and specificity together with their standard deviations. The user is also given information on which additional parameter it is worth adding to his measurement system most and what an increase in prediction efficiency it is expected to trigger. The BPD can be predicted by the system with the accuracy reaching up to 83.25% in the best-case scenario, i.e. higher than for most of the models presented in the literature. This work presents a set of examples illustrating the difficulties in obtaining one single model that can be widely used, and thus explaining why an expert system approach is much more useful in day-to-day clinical practice. In addition, the work discusses the significance of the parameters used and the impact of a chosen method on the sensitivity and specificity. Copyright © 2015 Elsevier Ltd. All rights reserved.

  2. 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.

  3. [Longitudinal study of children with bronchopulmonary dysplasia treated with disodium cromoglycate].

    Science.gov (United States)

    Kassur-Siemieńska, Barbara; Milewska-Bobula, Bogumiła; Dmeńska, Hanna; Idzik, Mirosława; Bauer, Anna; Marciński, Paweł; Dunin-Wasowicz, Dorota; Lipka, Bozena

    2003-01-01

    In order to improve the quality of life of children born prematurely, who developed chronic lung disease, clinical trials of drugs of different origin are undertaken. The aim of the work was the evaluation of the efficacy of disodium cromoglycate in the treatment of bronchopulmonary dysplasia in children. We retrospectively studied 15 infants with bronchopulmonary dysplasia (BPD) hospitalised in the Infant Care Department of Children's Health Memorial Institute from 01.01.1997 to 01.02.2000. All babies were premature (25-30 weeks of gestation) with LBW or VLBW A control group of 11 babies with BPD, matched for birth weight and gestational age, who did not have disodium cromoglycate therapy were also studied. Recurrent obturative bronchitis and bronchial hyperresponsiveness were stated in all cases in both groups. Disodium cromoglycate was administered in all babies in the study group. Inhaled corticosteroid (Budesonide mite) was given in 10 cases, for a short period of time, due to severe obturative bronchitis. Babies in the control group were treated with systemic and inhaled corticosteroids. Results of our trial compared with the log-rank and chi2 test show statistically, significant differences in the regression of obturative bronchitis (log-rank = 4.35, p < 0.0001) and normalization of capillary blood-gas examination (log-rank = 3.777, p < 0.0002) in favour of the studied group, treated with disodium cromoglycate.

  4. Detection of circulating fibrocytes in bronchopulmonary dysplasia in the premature infants and its significance%循环纤维细胞在早产儿支气管肺发育不良中的检测及意义

    Institute of Scientific and Technical Information of China (English)

    李春; 邓春

    2015-01-01

    Objective:To investigate the expression and clinical significance of CD34,COL Ⅰ and CXCR4 in bronchopulmonary dysplasia(BPD).Methods:Expressions of CD34,COL Ⅰ and CXCR4 in peripheral blood from 44 premature infants with BPD on the 1st,3rd,Sth,7th and 28th d after birth were measured by flow cytometry(FCM) and the expression differences of CD34,COL Ⅰ,CXCR4 between BPD group and the non-BPD group were compared.Results:On the 1st d after birth,the expression of CXCR4 increased greatly in BPD group compared with that of non-BPD group.With the increase of days after birth,COL Ⅰ level peaked on 28th d in BPD group,while CD34 and CXCR4 levels reduced to the lowest.Data of ANOVA for repeated measurement showed that time and the changes in CD34,COL Ⅰ and CXCR4 levels caused an interactive effect on the occurrence of BPD(F=77.66,P=-0.000).ROC curve displayed that area under the curve of COL Ⅰ was 0.867,higher than those of CD34 and CXCR4.When COL Ⅰ was 1.3%,the sensitivity was 69.7% and specificity was 86%.There were statistically significances in BPD group among different time points(P=0.000).Conclusion:Circulating fibrocytes with increased level of COL Ⅰ as well as decreased level of CD34 and CXCR4 are closely related with the occurrence of BPD.%目的:探讨CD34、Ⅰ型胶原(collagen Ⅰ,COL Ⅰ)、CXCR4在支气管肺发育不良(bronchopulmonary dysplasia,BPD)的表达及临床意义.方法:利用流式细胞仪(flow cytometry,FCM)检测61例早产儿生后1、3、5、7、28 d外周血中CD34、COLⅠ、CX-CR4的表达,并比较CD34、COLⅠ、CXCR4在BPD组及非BPD组间表达的差异.结果:BPD组患儿较非BPD组患儿CXCR4在生后第1天有明显增高[(61.4±13.37)%].随着日龄的增大,BPD患儿COLⅠ水平在第28天达到高峰[3.42(2.87,4.98)%],而CD34、CXCR4水平降至最低[(0.67±0.25)%;(31.9±13.48)%].重复测量设计方差分析显示,日龄的增大及CD34、COLⅠ、CX-CR4水平的变化对BPD

  5. Plasma lipid metabolites are associated with gestational age but not bronchopulmonary dysplasia.

    Science.gov (United States)

    Rogers, Lynette K; Young, Christine M; Pennell, Michael L; Tipple, Trent E; Leonhart, Karen L; Welty, Stephen E

    2012-08-01

    To test the hypothesis that plasma lipid metabolite levels in premature infants are associated with the development of bronchopulmonary dysplasia (BPD). The studies also tested a secondary hypothesis that plasma lipid metabolite levels were correlated with gestational age. Infants born <32 weeks' gestation were enrolled during the first 72 h of life. Plasma samples were obtained and lipid levels were measured by LC-MS/MS. Clinical data were collected to determine infant outcomes and BPD diagnosis. Following adjustment for confounders, lipid levels were not associated with BPD; however, levels of specific lipid metabolites were correlated with gestational age. Immature lipid metabolism pathways in premature infants may contribute to the pathogenesis of BPD and other diseases. © 2012 The Author(s)/Acta Paediatrica © 2012 Foundation Acta Paediatrica.

  6. 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.

  7. 小胎龄早产儿支气管肺发育不良发生率和危险因素分析%The incidence and risk factors of bronchopulmonary dysplasia in small gestational age premature infants

    Institute of Scientific and Technical Information of China (English)

    郑国方; 武荣; 刘石; 郝小清

    2012-01-01

    Objective To analyse bronchopulmonary dysplasia (BPD) incidence and high risk factor in the small gestational age premature infants. Methods Retrospective analyse the materials of inpatient infants whose gestational age (GA) were =?2 weeks and survived over 28 days in our neonatal intensive care unite ( NICU). The 28 cases as BPD group met the new diagnostic criteria of BPD. The 56 cases as the control group were randomly selected from all the premature infant with no BPD. Results Total of 197 cases of premature infant were included in this study. The incidence of BPD is about 14.2%. There were statistical significance in each GA period group (x2 =32.269,/* =0.000). The incidence increased when the GA decreased; There were statistical significance in each birth weight group (x2 =30. 244, P =0. 000), the incidence increased when birth weight decreased. From the comparison of the 23 risk factors for BPD, we find thai 12 factors have statistical significance (P < 0. 05), those are GA, body weight, oxygen time, maximum oxygen treatment concentration, hospital days, tracheal intubation mechanical ventilation, replacement therapy with pulmonary surfactant, anemia, application of Meropenem, the ratio of tenth day body weight to birth weight, the first blood gas analysis scores after birth and oxygen index < 300. On the basis of Logistic regression analysis of GA, birth weight, the highest inhaled oxygen volume concentration, tracheal intubation mechanical ventilation, anemia, the ratio of tenth day body weight to birth weight, we find that body weight, the highest inhaled oxygen volume concentration, the ratio of tenth body weight to birth weight are high risk factors for BPD. By compared the 18 factors between mild BPD and moderate or severe BPD, we find that asphyxia, application of diuretic, oxygen time and first blood gas analysis scores after birth have statistical significance ( P < 0.05 ) . Conclusions The birth weight, the highest inhaled oxygen volume

  8. 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 appea

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

    NARCIS (Netherlands)

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

    2010-01-01

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

  10. 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

  11. Characteristics of lung function in pretermin fants with varying degress of bronchopulmonary dysplasia

    Institute of Scientific and Technical Information of China (English)

    卫敏超

    2013-01-01

    Objective To explore the characteristics of lung function in preterm infants with varying degrees of bronchopulmonary dysplasia (BPD) .Methods There were407 infants (278 males and 129 females) were recruited from Shenzhen Children’Hospital between January 2011

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

    Science.gov (United States)

    D'Angio, Carl T; Ryan, Rita M

    2014-12-15

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

  13. Screening of susceptibility genes for bronchopulmonary dysplasia in Han premature infants%汉族早产儿支气管肺发育不良易感基因筛查

    Institute of Scientific and Technical Information of China (English)

    赵堃; 农绍汉; 余宇晖; 钟劲; 欧阳维富; 黄小穗; 聂志强; 孙云霞; 梁穗新

    2015-01-01

    Objective To explore the relationship between susceptibility loci polymorphism and bronchopulmonary dysplasia (BPD) in Han premature infants.Methods Twenty-eight susceptibility genes were speculated to be associated with BPD development according to the literature.And 46 cases of Han premature infants,who were hospitalized in Guangdong General Hospital from April to December 2013 and whose gestational age≤32 weeks,birth weight (BW) ≤ 1 500 g,were prospectively selected into the study after obtaining the informed consents from the parents.Among them,31 cases were BPD patients (BPD group),and 15 cases were non-BPD patients (control group).Blood samples were collected for the detection of 28 susceptibility genes within 3 days of hospitalization.After blood samples were extracted,and genomic DNA was amplified by PCR,the blood samples were then analyzed by single base extension technology and DNA mass spectrometry.Results Fourteen genes,whose frequencies were in Hardy-Weinberg equilibrium among the 28 susceptibility genes,were finally selected as the candidate genes for the study.Tumor necrosis factor α (TNF-α) gene rs1799724 and Toll-like receptor (TLR)-10 gene rs11096955 were founded to be possibly related to the development of BPD.A significant higher CC [93.5% (29/31 cases)] and lower TT[0(0/31 cases)]contents were presented in TNF-α gene detection in BPD group,those in the control group were 33.3% (5/15 cases),20.0% (3/15 cases),and there was significant difference between 2 groups (x2 =18.044,P < 0.01) ; while higher AC [22.6% (7/31 cases)] and lower CC [12.9% (4/31 cases)] contents in TLR-10 gene detection were presented in BPD group,those in the control group were 20.0% (3/15 cases),46.7% (7/15 cases),there was a significant difference between 2 groups (x2 =6.252,P =0.043).C allele distribution frequency of TNF-α gene in the BPD group[93.5% (29/31 cases)] increased,compared with that in control group[46.7% (7/15 cases)],there was a

  14. Circulating Fibrocytes Are Increased in Neonates with Bronchopulmonary Dysplasia

    Science.gov (United States)

    Li, Chun; Li, Xiaoyu; Deng, Chun; Guo, Chunbao

    2016-01-01

    Background 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. Methods 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. Results The patients with BPD had significantly increased numbers of fibrocytes compared to the controls (p fibrocytes and pulmonary hypertension or oxygen saturation (p Fibrocyte numbers were not correlated with other clinical or functional variables or radiologic severity scores. The fibrocyte attractant chemokine CXCL12 increased in plasma (p 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. PMID:27309347

  15. 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.

  16. 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.

  17. HLA and Bronchopulmonary Dysplasia Susceptibility: A Pilot Study

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    Gustavo Rocha

    2011-01-01

    Full Text Available There is little data on the association between Human Leucocyte Antigen (HLA alleles and Bronchopulmonary Dysplasia (BPD of the preterm newborn. Our aim was to assess associations between HLA alleles and BPD susceptibility. We studied 156 preterm neonates (82 M/74 F < 32 weeks gestational age, alive at 36 weeks gestational age. Detailed clinical data were collected. HLA typing was performed by PCR-SSO. HLA allele frequencies where determined by direct counting for BPD and no-BPD groups. Comparison between BPD and no BPD groups was performed using t-test, χ2 test or Fisher exact test and logistic regression as appropriate. Relative risks (RR and their 95% confidence intervals (95% CI were also calculated as association measures. We diagnosed 56 (35.9% neonates with mild BPD and 27 (17% with moderate/severe BPD. We found a significant association between HLA-DRB1*01 and mild BPD (OR=3.48[1.23–10.2]. The alleles HLA-A*24, -A*68, -B*51,-Cw*07, -Cw*14, -Cw*15 and -DRB1*01 presented a significant association with moderate/severe BPD. When adjusted to gestational age and birth weight HLA-A*68 (OR=5.41[1.46; 20.05], -B*51 (OR=3.09[1.11; 8.63] and -Cw*14 (OR=4.94[1.15; 21.25] were significantly associated with moderate/severe BPD. Conclusion – Our findings suggest an association between HLA-A*68, -B*51 and -C*14 and BPD susceptibility, and that an autoimmune mechanism may be implicated in the pathogenesis of the disease.

  18. Bronchopulmonary dysplasia: correlation of radiographic and clinical findings

    Energy Technology Data Exchange (ETDEWEB)

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

    1997-08-01

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

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

    Directory of Open Access Journals (Sweden)

    Milad Rezvani

    2013-01-01

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

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

    Science.gov (United States)

    Davidson, Lauren M.; Berkelhamer, Sara K.

    2017-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Kruger Thomas E

    2003-08-01

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

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

    Directory of Open Access Journals (Sweden)

    Lauren M. Davidson

    2017-01-01

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

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

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    Hercília Guimarães

    2014-01-01

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

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

    NARCIS (Netherlands)

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

    2010-01-01

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

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

    NARCIS (Netherlands)

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

    1994-01-01

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

  6. Stem cells and their mediators – next generation therapy for bronchopulmonary dysplasia

    Directory of Open Access Journals (Sweden)

    Marius Alexander Möbius

    2015-07-01

    Full Text Available Bronchopulmonary dysplasia (BPD remains a major complication of premature birth. Despite great achievements in perinatal medicine over the past decades, there is no treatment for BPD. Recent insights into the biology of stem/progenitor cells have ignited the hope of regenerating damaged organs. Animal experiments revealed promising lung protection/regeneration with stem/progenitor cells in experimental models of BPD and led to first clinical studies in infants. However, these therapies are still experimental and knowledge on the exact mechanisms of action of these cells is limited. Furthermore, heterogeneity of the therapeutic cell populations and missing potency assays limit currently our ability to predict a cell product’s efficacy. In here, we review the therapeutic potential of mesenchymal stromal, endothelial progenitor and amniotic epithelial cells for BPD. Current knowledge on the mechanisms behind the beneficial effects of stem cells is briefly summarized. Finally, we discuss the obstacles constraining their transition from bench to bedside and present potential approaches to overcome them.

  7. A Clinical Scoring System to Predict the Development of Bronchopulmonary Dysplasia

    OpenAIRE

    Gürsoy, Tuğba; Hayran, Mutlu; Derin, Hatice; Ovalı, Fahri

    2015-01-01

    A Clinical Scoring System to Predict the Development of Bronchopulmonary Dysplasia Tugba Gursoy, MD1 Mutlu Hayran, MD2 Hatice Derin, MD3 Fahri Ovali, MD3 1Department of Neonatology, School of Medicine, KOC University, Istanbul, Turkey 2Department of Preventive Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey 3Department of Pediatrics, Zeynep Kamil Maternity and Children’s Research and Training Hospital, Istanbul, Turkey 4Department of Neonatology,...

  8. 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 (nCPAP)......-postnatal treatment with nCPAP and surfactant decreases the severity and mortality of RDS and BPD. This is mainly due to a diminished use of MV in the first days of life....

  9. Transcriptome analysis in preterm infants developing bronchopulmonary dysplasia : data processing and statistical analysis of microarray data

    OpenAIRE

    2015-01-01

    Bronchopulmonary dysplasia is one of the most common chronic lung diseases and contributes greatly to morbidity of preterm infants. While moderate and severe forms of BPD are the most common forms under investigation little is known about the development of mild BPD. The aim of this work is to identify mechanisms and biomarkers, which make it possible to predict at birth whether a preterm infant is prone to develop no BPD, mild BPD, or a stronger form of BPD. Transcriptome and in particula...

  10. 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.

  11. Sildenafil for the Treatment of Pulmonary Arterial Hypertension in Infants with Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Trottier-Boucher, M N; Lapointe, A; Malo, J; Fournier, A; Raboisson, M J; Martin, B; Moussa, A

    2015-08-01

    Sildenafil, a phosphodiesterase-5 inhibitor, is a controversial treatment option for pulmonary arterial hypertension (PAH), a significant complication of bronchopulmonary dysplasia (BPD). The objective of this study was to evaluate the use of sildenafil in infants with PAH secondary to BPD. This was a retrospective review of medical records of all premature infants with PAH associated with BPD treated with sildenafil between January 2009 and May 2013 in a level 3 neonatal intensive care unit. The primary outcomes were clinical response (20 % decreases in respiratory support score or oxygen requirements) and echocardiographic response (20 % decrease in tricuspid regurgitation gradient or change of at least 1° of septal flattening). Twenty-three infants were included in the study. Significant echocardiographic and clinical responses were, respectively, observed in 71 and 35 % of cases. Most clinical responses were observed in the first 48 h of treatment, and the median time to an echocardiographic response was of 19 days. The median dose of sildenafil used was 4.4 mg/kg/day, with a median time to reach the maximum dose of 9 days. Transient hypotension was the primary reported side effect, and it was observed in 44 % of our study population. Sildenafil treatment in patients with PAH secondary to BPD was associated with an echocardiographic improvement in the majority of patients, whereas clinical improvement was observed in a minority of patients. Many infants presented with transient hypotension during the course of the treatment. Further prospective studies are required to better assess safety and efficacy of this treatment in this population.

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

    Science.gov (United States)

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

    2017-02-01

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

  13. MicroRNA-mRNA interactions in a murine model of hyperoxia-induced bronchopulmonary dysplasia

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    Dong Jie

    2012-05-01

    Full Text Available Abstract Background Bronchopulmonary dysplasia is a chronic lung disease of premature neonates characterized by arrested pulmonary alveolar development. There is increasing evidence that microRNAs (miRNAs regulate translation of messenger RNAs (mRNAs during lung organogenesis. The potential role of miRNAs in the pathogenesis of BPD is unclear. Results Following exposure of neonatal mice to 80% O2 or room air (RA for either 14 or 29 days, lungs of hyperoxic mice displayed histological changes consistent with BPD. Comprehensive miRNA and mRNA profiling was performed using lung tissue from both O2 and RA treated mice, identifying a number of dynamically regulated miRNAs and associated mRNA target genes. Gene ontology enrichment and pathway analysis revealed that hyperoxia modulated genes involved in a variety of lung developmental processes, including cell cycle, cell adhesion, mobility and taxis, inflammation, and angiogenesis. MiR-29 was prominently increased in the lungs of hyperoxic mice, and several predicted mRNA targets of miR-29 were validated with real-time PCR, western blotting and immunohistochemistry. Direct miR-29 targets were further validated in vitro using bronchoalveolar stem cells. Conclusion In newborn mice, prolonged hyperoxia induces an arrest of alveolar development similar to that seen in human neonates with BPD. This abnormal lung development is accompanied by significant increases in the levels of multiple miRNAs and corresponding decreases in the levels of predicted mRNA targets, many of which have known or suspected roles in pathways altered in BPD. These data support the hypothesis that dynamic regulation of miRNAs plays a prominent role in the pathophysiology of BPD.

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

    Science.gov (United States)

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

    2016-09-01

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

  15. High incidence of rickets in extremely low birth weight infants with severe parenteral nutrition-associated cholestasis and bronchopulmonary dysplasia.

    Science.gov (United States)

    Lee, Soon Min; Namgung, Ran; Park, Min Soo; Eun, Ho Sun; Park, Kook In; Lee, Chul

    2012-12-01

    Risk factors for rickets of prematurity have not been re-examined since introduction of high mineral formula, particularly in ELBW infants. We analyzed the incidence and the risk factors of rickets in extremely low birth weight (ELBW) infants. As a retrospective case-control study from 2004 to 2008, risk factors were analyzed in 24 patients with rickets versus 31 patients without. The frequency of rickets in ELBW infants was 24/55 (44%). Infants with rickets were diagnosed at 48.2 ± 16.1 days of age, and improved by 85.3 ± 25.3 days. By radiologic evaluation, 29% were grade 1 rickets, 58% grade 2 and 13% grade 3. In univariate analysis, infants with rickets had significantly higher incidence of patent ductus arteriosus, parenteral nutrition associated cholestasis (PNAC), severe PNAC and moderate/severe bronchopulmonary dysplasia (BPD). In multiple regression analysis, after adjustment for gestation and birth weight, rickets significantly correlated with severe PNAC and with moderate/severe BPD. Serum peak alkaline phosphatase levels were significantly elevated in rickets (P rickets of prematurity remains high and the incidence of severe PNAC and moderate/severe BPD was significantly increased 18 and 3 times, respectively.

  16. The Quality of General Movements after Treatment with Low-Dose Dexamethasone in Preterm Infants at Risk of Bronchopulmonary Dysplasia

    NARCIS (Netherlands)

    Hitzert, Marrit M.; Roescher, Annemiek M.; Bos, Arend F.

    2014-01-01

    Background: High-dose dexamethasone (DXM) treatment of preterms at risk of bronchopulmonary dysplasia leads to a deterioration in quality of their general movements (GMs). It is unknown whether low-dose DXM affects GM quality similarly. Objectives: To assess the effect of low-dose DXM treatment on t

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

    Science.gov (United States)

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

    2017-03-21

    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. 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 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.

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

    NARCIS (Netherlands)

    W. Onland; M. Offringa; F. Cools; A.P.M.C. De Jaegere; K. Rademaker; H. Blom; E. Cavatorta; A. Debeer; P.H. Dijk; A.F. van Heijst; B.W. Kramer; A.A. Kroon; T. Mohns; H.L. van Straaten; A.B. te Pas; C. Theyskens; M.M. van Weissenbruch; A.H. van Kaam

    2011-01-01

    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 dexamethaso

  20. Nasal continuous positive airway pressure combined with surfactant and NO for treatment of respiratory distress syndrome, prevention of bronchopulmonary dysplasia, and brain protection

    Institute of Scientific and Technical Information of China (English)

    Henrik Verder

    2010-01-01

    @@ Respiratory distress syndrome (RDS) is the single most important cause of mortality and morbidity in preterm infants and bronchopulmonary dysplasia (BPD)is a leading cause of neuro-muscular disablement and decreased lung function in the most preterm infants.

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

    Directory of Open Access Journals (Sweden)

    K. A. Kazakova

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Arjan B Te Pas

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

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

    Science.gov (United States)

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

    2017-08-01

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

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

    Directory of Open Access Journals (Sweden)

    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.

  5. [BRONCHOPULMONARY DYSPLASIA IN VERY LOW BIRTH WEIGHT INFANTS--THE OLD NEW PROBLEM].

    Science.gov (United States)

    Vakrilova, L; Hitrova, St; Dimitrova, V; Slancheva, B; Radulova, P; Neikova, K

    2015-01-01

    The advances in perinatal intensive care have increased the survival rate of extremely low birthweight (ELBW) and gestational age infants. Among them the risk of developing bronchopulmonary dysplasia (BPD) remains high. To evaluate the frequency of BPD by birthweight and gestational age, to identify the main postnatal risk factors and the associated comorbidities. 683 VLBW infants ( 30% and/or ventilator support). Infants with BPD were with significantly higher CRIB (9.9 ± 3.1) compared with those without BPD (4.0 ± 3.0), p birthweight 1200g. Logistic regression analysis showed that each gestational week decreased the odds of BPD by 60%; each CRIB point increased the odds by 62%. Each point increment in 1/5 min Apgar-scores reduced the risk by 40%/50% respectively The need for ventilator support increased from 1.4 ± 2.7 days (no-BPD group) to 52.8 ± 5.1 days (severe-BPD infants), p birthweight and CRIB. Additional risk factors are low A pgar scores, PDA and air leak syndrome. Associated comorbidities as severe brain injury and ROP further worsen the long term prognosis.

  6. 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.

  7. Effect of bronchopulmonary dysplasia on early intellectual development in preterm infants.

    Science.gov (United States)

    Lin, Fatao; Dong, Haipeng; Song, Yanyan; Zhang, Tengwei; Qi, Junye; Xiao, Xuwen; Cai, Yueju

    2017-06-01

    The aim of this study was to investigate the effect of bronchopulmonary dysplasia (BPD) on the early intellectual development of preterm infants. From 2011 to 2015, 83 preterm infants diagnosed with BPD were recruited to the BPD group, and 89 preterm infants without BPD and 98 healthy term infants were randomly recruited to the non-BPD and term group, respectively. Neural and intellectual development according to the Gesell Development Scale were evaluated and compared between groups at 0-3 months, 3-6 months, 6-9 months, and 9-12 months of adjusted age for preterm infants and real age for term infants. Multivariate logistic regression was used to determine the associations between BPD and adverse neurological outcomes at 9-12 months of adjusted age. Compared with term infants, preterm infants had significantly lower developmental quotients for adaptability, gross motor, fine motor, language and social skills. At follow up, deficits in one or more neurofunctions related to adaptability, gross motor, fine motor, language and social skills were significantly more frequent in preterm children with BPD than in those with no history of BPD. BPD was independently associated with adverse neurological outcome at 9-12 months of adjusted age in preterm infants. Early intelligence disturbances occurred significantly more frequently in BPD infants than in non-BPD infants. Monitoring of the development of the nervous system in BPD infants should be strengthened. © 2017 Japan Pediatric Society.

  8. A new look at bronchopulmonary dysplasia: postcapillary pathophysiology and cardiac dysfunction

    Science.gov (United States)

    Malikiwi, Andra; Paul, Eldho; Tan, Kenneth; Menahem, Samuel

    2016-01-01

    Abstract Pulmonary hypertension (PH) and right ventricular function are the focus of cardiovascular effects of bronchopulmonary dysplasia (BPD). We assessed cardiac indexes reflecting systemic afterload and pulmonary venous back pressure as pathophysiologic factors. Cardiac parameters were measured by conventional echocardiography in 20 preterm infants with severe BPD and compared with those of 10 preterm infants with no BPD and 20 healthy term infants. In infants with severe BPD, PH was noted in 5 (25%) by tricuspid regurgitation Doppler jet ≥2.8 m/s and in 15 (75%) by time to peak velocity/right ventricular ejection time valve stroke volume (4.7 ± 0.7 vs. 5.6 ± 0.6 vs. 5.9 ± 0.1; P = 0.002), and myocardial performance index (0.33 ± 0.05 vs. 0.28 ± 0.01 vs. 0.27 ± 0.05; P = 0.03). Left ventricular output was significantly lower in the BPD cohort (183 ± 45 vs. 189 ± 9 vs. 191 ± 32 mL/kg/min; P = 0.03). Altered systemic (left-sided) cardiac function was noted in infants with BPD, which may lead to pulmonary venous congestion contributing to a continued need for respiratory support. PMID:28090292

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

    Directory of Open Access Journals (Sweden)

    Anstead Michael I

    2007-06-01

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

  10. Fractal analysis of alveolarization in hyperoxia-induced rat models of bronchopulmonary dysplasia.

    Science.gov (United States)

    Porzionato, Andrea; Guidolin, Diego; Macchi, Veronica; Sarasin, Gloria; Grisafi, Davide; Tortorella, Cinzia; Dedja, Arben; Zaramella, Patrizia; De Caro, Raffaele

    2016-04-01

    No papers are available about potentiality of fractal analysis in quantitative assessment of alveolarization in bronchopulmonary dysplasia (BPD). Thus, we here performed a comparative analysis between fractal [fractal dimension (D) and lacunarity] and stereological [mean linear intercept (Lm), total volume of alveolar air spaces, total number of alveoli, mean alveolar volume, total volume and surface area of alveolar septa, and mean alveolar septal thickness] parameters in experimental hyperoxia-induced models of BPD. At birth, rats were distributed between the following groups: 1) rats raised in ambient air for 2 wk; 2) rats exposed to 60% oxygen for 2 wk; 3) rats raised in normoxia for 6 wk; and 4) rats exposed to 60% hyperoxia for 2 wk and to room air for further 4 wk. Normoxic 6-wk rats showed increased D and decreased lacunarity with respect to normoxic 2-wk rats, together with changes in all stereological parameters except for mean alveolar volume. Hyperoxia-exposed 2-wk rats showed significant changes only in total number of alveoli, mean alveolar volume, and lacunarity with respect to equal-in-age normoxic rats. In the comparison between 6-wk rats, the hyperoxia-exposed group showed decreased D and increased lacunarity, together with changes in all stereological parameters except for septal thickness. Analysis of receiver operating characteristic curves showed a comparable discriminatory power of D, lacunarity, and total number of alveoli; Lm and mean alveolar volume were less discriminative. D and lacunarity did not show significant changes when different segmentation thresholds were applied, suggesting that the fractal approach may be fit to automatic image analysis. Copyright © 2016 the American Physiological Society.

  11. Tamoxifen dosing for Cre-mediated recombination in experimental bronchopulmonary dysplasia.

    Science.gov (United States)

    Ruiz-Camp, Jordi; Rodríguez-Castillo, José Alberto; Herold, Susanne; Mayer, Konstantin; Vadász, István; Tallquist, Michelle D; Seeger, Werner; Ahlbrecht, Katrin; Morty, Rory E

    2017-02-01

    Bronchopulmonary dysplasia (BPD) is the most common complication of preterm birth characterized by blunted post-natal lung development. BPD can be modelled in mice by exposure of newborn mouse pups to elevated oxygen levels. Little is known about the mechanisms of perturbed lung development associated with BPD. The advent of transgenic mice, where genetic rearrangements can be induced in particular cell-types at particular time-points during organogenesis, have great potential to explore the pathogenic mechanisms at play during arrested lung development. Many inducible, conditional transgenic technologies available rely on the application of the estrogen-receptor modulator, tamoxifen. While tamoxifen is well-tolerated and has been widely employed in adult mice, or in healthy developing mice; tamoxifen is not well-tolerated in combination with hyperoxia, in the most widely-used mouse model of BPD. To address this, we set out to establish a safe and effective tamoxifen dosing regimen that can be used in newborn mouse pups subjected to injurious stimuli, such as exposure to elevated levels of environmental oxygen. Our data reveal that a single intraperitoneal dose of tamoxifen of 0.2 mg applied to newborn mouse pups in 10 μl Miglyol vehicle was adequate to successfully drive Cre recombinase-mediated genome rearrangements by the fifth day of life, in a murine model of BPD. The number of recombined cells was comparable to that observed in regular tamoxifen administration protocols. These findings will be useful to investigators where tamoxifen dosing is problematic in the background of injurious stimuli and mouse models of human and veterinary disease.

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

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    Dominik Monz

    2015-12-01

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

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

    Science.gov (United States)

    Nardiello, Claudio; Morty, Rory E

    2016-12-01

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

  14. 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 inc

  15. 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 inc

  16. 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

  17. Trends in survival among extremely-low-birth-weight infants (less than 1000 g without significant bronchopulmonary dysplasia

    Directory of Open Access Journals (Sweden)

    Botet Francesc

    2012-06-01

    Full Text Available Abstract Objective The aim of this study was to analyze the evolution from 1997 to 2009 of survival without significant (moderate and severe bronchopulmonary dysplasia (SWsBPD in extremely-low-birth-weight (ELBW infants and to determine the influence of changes in resuscitation, nutrition and mechanical ventilation on the survival rate. Study design In this study, 415 premature infants with birth weights below 1000 g (ELBW were divided into three chronological subgroups: 1997 to 2000 (n = 65, 2001 to 2005 (n = 178 and 2006 to 2009 (n = 172. Between 1997 and 2000, respiratory resuscitation in the delivery room was performed via a bag and mask (Ambu®, Ballerup, Sweden with 40-50% oxygen. If this procedure was not effective, oral endotracheal intubation was always performed. Pulse oximetry was never used. Starting on January 1, 2001, a change in the delivery room respiratory policy was established for ELBW infants. Oxygenation and heart rate were monitored using a pulse oximeter (Nellcor® attached to the newborn’s right hand. If resuscitation was required, ventilation was performed using a face mask, and intermittent positive pressure was controlled via a ventilator (Babylog2, Drägger. In 2001, a policy of aggressive nutrition was also initiated with the early provision of parenteral amino acids. We used standardized parenteral nutrition to feed ELBW infants during the first 12–24 hours of life. Lipids were given on the first day. The glucose concentration administered was increased by 1 mg/kg/minute each day until levels reached 8 mg/kg/minute. Enteral nutrition was started with trophic feeding of milk. In 2006, volume guarantee treatment was instituted and administered together with synchronized intermittent mandatory ventilation (SIMV + VG. The complications of prematurity were treated similarly throughout the study period. Patent ductus arteriosus was only treated when hemodynamically significant. Surgical closure of the

  18. Inhalation or instillation of steroids for the prevention of bronchopulmonary dysplasia.

    Science.gov (United States)

    Bassler, Dirk

    2015-01-01

    Survival of extremely preterm infants has increased over recent years, but bronchopulmonary dysplasia (BPD) remains a major cause of morbidity. In the USA, BPD is the most common chronic respiratory disorder of infancy and affects the pulmonary and overall health of 10,000 preterm infants annually. Preclinical and clinical studies suggest a crucial role for lung inflammation and host immune response in the pathogenesis of BPD. Inflammation may result from, amongst others, chorioamnionitis, postnatal infection, ventilation, and the administration of oxygen. Infants with BPD have worse long-term outcomes than those without chronic lung disease. They are more than twice as likely to be readmitted to hospital in their first year of life and, having survived their primary hospitalizations, they are more likely to die than very preterm infants without chronic lung disease. Survivors with BPD have an increased risk of neurodevelopmental impairment and their respiratory function remains compromised well into adolescence. As the first generations of extremely low birth weight (ELBW) survivors have not yet reached retirement age, there are currently no reliable data addressing the association between BPD and pulmonary diseases of the elderly such as chronic obstructive pulmonary disease. Although BPD is quite common in ELBW infants, there are infants who do not develop BPD, which supports the argument that BPD is a preventable disease, emphasizing the need for high-quality safety and efficacy prevention studies. However, according to an Institute of Medicine statement regarding pediatric drug studies, the therapeutic area that has the fewest drugs indicated for neonates is BPD. As inflammation seems to be a primary mediator of injury in the pathogenesis of BPD, anti-inflammatory agents such as steroids have long been the focus of preventive research activities. However, systemic steroids, although reducing BPD, have frequently been linked to adverse neurodevelopmental

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

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    Maresa E. C. Jiskoot-Ermers

    2015-10-01

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

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

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    Julio Jiménez

    2016-10-01

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

  1. A breath of fresh air on the mesenchyme: Impact of impaired mesenchymal development on the pathogenesis of Bronchopulmonary Dysplasia

    Directory of Open Access Journals (Sweden)

    Cho-Ming eChao

    2015-04-01

    Full Text Available The early mouse embryonic lung, with its robust and apparently reproducible branching pattern, has always fascinated developmental biologists. They have extensively used this embryonic organ to decipher the role of mammalian orthologues of Drosophila genes in controlling the process of branching morphogenesis. During the early pseudoglandular stage, the embryonic lung is formed mostly of tubes that keep on branching. As the branching takes place, progenitor cells located in niches are also amplified and progressively differentiate along the proximo-distal and dorso-ventral axes of the lung. Such elaborate processes require coordinated interactions between signaling molecules arising from and acting on four functional domains: the epithelium, the endothelium, the mesenchyme and the mesothelium. These interactions, quite well characterized in a relatively simple lung tubular structure remain elusive in the successive developmental and postnatal phases of lung development. In particular, a better understanding of the process underlying the formation of secondary septa, key structural units characteristic of the alveologenesis phase, is still missing. This structure is critical for the formation of a mature lung as it allows the subdivision of saccules in the early neonatal lung into alveoli, thereby considerably expanding the respiratory surface. Interruption of alveologenesis in preterm neonates underlies the pathogenesis of chronic neonatal lung disease known as Bronchopulmonary Dysplasia or BPD. De novo formation of secondary septae appears also to be the limiting factor for lung regeneration in human patients with emphysema. In this review, we will therefore focus on what is known in terms of interactions between the different lung compartments and discuss the current understanding of mesenchymal cell lineage formation in the lung, focusing on secondary septa formation.

  2. Study Advances in Molecular Pathogenesis of Bronchopulmonary Dysplasia%支气管肺发育不良的分子水平及发病机制的研究进展

    Institute of Scientific and Technical Information of China (English)

    张华; 刘漫君

    2013-01-01

    With the improvement of premature infants treatment successful rate,the pathological changes and the typing of bronchopulmonary dysplasia(BPD) will also be changed.BPD might be the result of premature birth,chronic oxygen exposure,inflammation and many other factors.It is not only the major cause of the chronic respiratory diseases for infants,but also the major cause of death for infants.The pathogenesis of BPD is a hot spot in neonatology.Here reviews the research progress of molecular level pathogenesis and related genes in BPD onset and development.%随着早产儿救治成活率的不断提高,支气管肺发育不良(BPD)的病理改变、分型等也随之发生了改变.BPD可能是早产、慢性氧暴露、炎症等多因素共同作用的结果,是婴幼儿时期慢性呼吸系统疾病的主要病因和死亡原因.BPD的发病机制是新生儿医学的研究热点.该文就BPD的分子水平发病机制、相关基因在BPD发生、发展中的调节作用等相关研究进展予以综述.

  3. Prevention and management of bronchopulmonary dysplasia: Lessons learned from the neonatal research network

    Science.gov (United States)

    Kennedy, Kathleen A.; Cotten, C. Michael; Watterberg, Kristi L.; Carlo, Waldemar A.

    2017-01-01

    Despite remarkable improvements in survival of extremely premature infants, the burden of BPD among survivors remains a frustrating problem for parents and caregivers. Advances, such as antenatal steroids and surfactant replacement, which have dramatically improved survival, have not reduced BPD among survivors. Other advances that have significantly improved the combined outcome of death or BPD, such as vitamin A and avoidance of mechanical ventilation, have had smaller magnitude effects on the outcome of BPD alone. Postnatal steroids have a clear beneficial effect on BPD, but the optimal preparation, dose, and timing for maximizing benefit and minimizing harm have yet to be determined. This persistent burden of BPD among the most immature survivors remains a challenge for the NRN and other researchers in neonatal medicine. PMID:27742002

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

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    Gicelle S. Cunha

    2003-11-01

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

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

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    Kevin K. Noguchi

    2014-01-01

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

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

    Science.gov (United States)

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

    2013-04-01

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

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

    Directory of Open Access Journals (Sweden)

    Brian J Clouse

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

  8. Developmental changes in polyunsaturated fetal plasma phospholipids and feto-maternal plasma phospholipid ratios and their association with bronchopulmonary dysplasia.

    Science.gov (United States)

    Bernhard, Wolfgang; Raith, Marco; Koch, Vera; Maas, Christoph; Abele, Harald; Poets, Christian F; Franz, Axel R

    2016-10-01

    Docosahexaenoic (C22:6) and arachidonic acid (C20:4) are long-chain polyunsaturated fatty acids (LC-PUFA), essential to fetal development, and preferentially transported by plasma phospholipids. To characterize fetal and maternal plasma phospholipid changes during gestation, and to investigate whether LC-PUFA phospholipid profiles are associated with bronchopulmonary dysplasia (BPD). Cord plasma and parturient serum from N = 108 pregnancies [24-42 week postmenstrual age (PMA)] were collected. Phosphatidylcholine (PC) and phosphatidylethanolamine (PE) were analyzed with tandem mass spectrometry. PMA-associated changes were quantified, and break point analyses served to describe nonlinear changes during gestation. PC and PE were lower in cord than in parturient samples. In parturients, PC decreased until 33 week PMA, but then re-increased, whereas in cord plasma, concentrations linearly decreased. Fetal PC and PC sub-group values correlated with maternal values. C20:4-PC was twofold higher in cord than in maternal samples throughout gestation. C22:6-PC values, however, exceeded maternal values only beyond 33 week PMA. Consequently, early preterm C20:4-PC-to-C22:6-PC ratio largely exceeded term infant values. In infants born before 28 week PMA, a low C20:4-PC-to-C22:6-PC ratio was associated with BPD severity. Fetal plasma LC-PUFA-PC composition correlates with maternal values. Fetal C20:4-PC exceeds maternal values throughout gestation, whereas C22:6-PC exceeds maternal values only beyond 33 week PMA, resulting in a low fetal C20:4-PC/C22:6-PC ratio only toward end gestation. A low C20:4-PC/C22:6-PC ratio before 28 week PMA is associated with BPD severity. These data point to a concept of PMA-adjusted ARA and DHA supplementation and, potentially, cord plasma phospholipid analysis for BPD prediction.

  9. Ultrasound in detection of developmental hip dysplasia in premature born children.

    Science.gov (United States)

    Misanovic, Verica; Jonuzi, Fedzat; Maksic-Kovacevic, Hajra; Rahmanovic, Selma

    2015-04-01

    Developmental hip dysplasia represents the most common deformation of locomotor system in children. Developmental modulation of the hip is expressed during first year of life which is important for early diagnosis and treatment. Therefore, in the practice, it is very important to set a diagnosis early with application of simple and convenient methods (ultrasound) in order to achieve fast and efficient therapeutical effect and avoid permanent disability. The aim of this paper is to point out the increase of prematurely born infants and their survival thanks to the development of Unit for Intensive Neonatal Care at the Pediatric Clinics in Sarajevo. Clinical investigation included 150 infants (77 girls and 73 boys) in whom the developmental hip dysplasia was diagnosed with clinical exam, ultrasound exam and x-ray of the hips. The exams were done in period of January 2012 to August 2014. Two groups of patients were formed. The first one consisted of premature infants, total number of 75 (34 girls and 41 boys), with developmental hip dysplasia that was diagnosed at the first exam at the Ultrasound unit of the Pediatric clinics and at the Intensive Neonatal Care Unit of the Pediatric Clinics in Sarajevo. Second (control) group consistsed of patients-on term infants who had diagnosed one of developmental hip dysplasia, total of 75 (43 girls and 32 boys) during first exam in the Ultrasound unit of the Pediatric clinics in Sarajevo. The frequency of premature birth is between 5 and 10% of all labors and demonstrates increasing trend. We suggest ultrasound examination of hips in each newborn, term or premature, at the age of 6 weeks after birth.

  10. Living with Bronchopulmonary Dysplasia

    Science.gov (United States)

    ... the neonatal intensive care unit (NICU). Fatigue (tiredness). Frustration that you can't breastfeed your infant right ... the hospital. They may continue to need oxygen therapy (oxygen given through nasal prongs, a mask, or ...

  11. Life after the NNU: the long term effects on mothers' lives, managing a child at home with broncho-pulmonary dysplasia and on home oxygen.

    Science.gov (United States)

    Manns, Sarah V

    2004-12-01

    This study investigated the long-term influences on mothers' lives having managed an infant with bronchopulmonary dysplasia (BPD), on home oxygen. 16 mothers UK wide, whose children had been at home for over a year, formed this project, and completed a semi-structured questionnaire. All mothers displayed a high standard of literacy and were English/British by their own description with no indication of cross cultural or ethnic differences. Mothers, giving up jobs and careers to remain home, were the primary carers for their child, and experienced long term emotional distress, suffered from lowered self-esteem over many years, self-blame in some cases and grieved over many aspects of pregnancy, birth, babyhood and early childhood. The fear of infection led to these mothers keeping themselves and their children isolated, compounded for half the mothers who faced negative reactions from the community when they did go out with their child.

  12. 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

    three and bronchopulmonary dysplasia (BPD) or death and further to assess the impact of patent ductus arteriosus (PDA) on this association in neonates born before 32 gestational weeks. METHODS: A cohort study of 183 neonates born before 32 gestational weeks consecutively admitted to the Neonatal......-proBNP to be associated with BPD or death in very preterm neonates. This association was not only explained by the PDA. We speculate that NT-proBNP may help the identification of neonates at risk of BPD as early as postnatal day three....... 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...

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

    Directory of Open Access Journals (Sweden)

    Anna Sellmer

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

  14. 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.

  15. Development of Left Ventricular Longitudinal Speckle Tracking Echocardiography in Very Low Birth Weight Infants with and without Bronchopulmonary Dysplasia during the Neonatal Period

    Science.gov (United States)

    Czernik, Christoph; Rhode, Stefanie; Helfer, Sven; Schmalisch, Gerd; Bührer, Christoph; Schmitz, Lothar

    2014-01-01

    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. PMID:25184634

  16. 支气管肺发育不良神经系统预后的研究进展%Research Advances in Neurological Outcomes of Bronchopulmonary Dysplasia

    Institute of Scientific and Technical Information of China (English)

    张山丹

    2012-01-01

    As more infants of very low birth weight (VLBW) survive, more survivors will suffer of bronchopulmonary dysplasia (BPD). Through childhood and into early life those would normally be expected, children with BPD have higher rates of cognitive , educational and behavioural impairments , and also reduced lung function . The important affection of children with BPD neurological and respiratory problems later into adult life needs to be determined . This article focuses on discussing of the support for the neurological outcomes of BPD , so as to perfect the understanding of it .%随着极低出生体重(infants of very low birth weight,VLBW)儿存活率的升高,支气管肺发育不良(BPD)发生率也随之增高.对BPD患儿于儿童期和成年早期的神经系统预后研究发现,其出现认知、教育和行为障碍及肺功能降低几率方面较预期高.作者就目前BPD神经系统预后的研究进展,进行综述如下.

  17. Histologic Chorioamnionitis, Amniotic Fluid Interleukin 6, Krebs von den Lungen 6, and Transforming Growth Factor β for the Development of Neonatal Bronchopulmonary Dysplasia

    Directory of Open Access Journals (Sweden)

    Hisako Matsumura

    2017-03-01

    Full Text Available Background: Chorioamnionitis (CAM is an important risk factor for the development of bronchopulmonary dysplasia (BPD in preterm infants. Objectives: To evaluate the effects of CAM on the development of BPD using interleukin 6 (IL-6, Krebs von den Lungen 6 (KL-6, and transforming growth factor β 1 (TGF-β 1 in the amniotic fluid as markers for inflammation, lung injury, and fibrosis/remodeling, respectively. Methods: Amniotic fluid concentrations of IL-6, KL-6, and TGF-β 1 were measured with enzyme-linked immunosorbent assay or electrochemiluminescence immunoassay. Results: Of the 36 preterm infants, 18 were exposed to histologically confirmed CAM. Of these, 12 were later diagnosed as having BPD. The IL-6, KL-6, and TGF-β 1 levels in the amniotic fluid significantly increased with increasing histologic severity of CAM. Moreover, these markers were higher in the BPD group with histologic CAM than those without. Conclusions: Our study suggests that CAM is likely to induce inflammatory, injury, and remodeling processes in the fetal lung.

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

    Science.gov (United States)

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

    2016-05-06

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

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

    Directory of Open Access Journals (Sweden)

    Christoph Czernik

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

  20. 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

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

    Directory of Open Access Journals (Sweden)

    Lídia Mayrink de Barros

    2007-12-01

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

  2. 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)

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

    2013-05-01

    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

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

    Directory of Open Access Journals (Sweden)

    H.S. Senatorova

    2015-09-01

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

  4. Evidence for the safety of ascorbic acid administration to the premature infant.

    Science.gov (United States)

    Bass, W T; Malati, N; Castle, M C; White, L E

    1998-02-01

    Ascorbic acid (AA), a plasma antioxidant, is maintained at high levels in premature fetal blood and declines rapidly postpartum. The sudden reduction in blood AA levels secondary to premature delivery may increase the risk of oxidant injury, that is, bronchopulmonary dysplasia and intraventricular hemorrhage. There is concern that administration of AA to premature infants, in an effort to increase antioxidant capacity, may cause hemolysis. We felt that the benefits of early AA administration and prevention of the immediate postnatal drop in blood AA levels, might outweigh the risks of erthrocyte damage. Fifty one high-risk premature infants were randomized to receive either normal saline or 100 mg/kg of AA, daily for the first week of life. Double-blind comparisons were made of hemoglobin, hematocrit, erythrocyte morphology, bilirubin, number of blood transfusions and days of phototherapy, renal function tests, the incidence of infection, bronchopulmonary dysplasia, and intraventricular hemorrhage during the first month of life. The administration of AA prevented the immediate postnatal drop in AA and was not associated with evidence of increased hemolysis. No significant differences in renal function, rate of infection, bronchopulmonary dysplasia, or intraventricular hemorrhage were seen between the two groups. This study suggests that AA administration to the premature infant is safe and supports the designing and performance of larger clinical studies of the antioxidant properties of AA.

  5. How Is Bronchopulmonary Dysplasia Treated?

    Science.gov (United States)

    ... on infants and meet their basic needs of warmth, nutrition, and protection. Once doctors diagnose BPD, some ... the NICU, he or she also may need physical therapy. Physical therapy can help strengthen your child's ...

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

    Directory of Open Access Journals (Sweden)

    Marcela Raquel de Oliveira Lima

    2011-08-01

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

  7. Future Applications of Antioxidants in Premature Infants

    Science.gov (United States)

    Lee, Jennifer W.; Davis, Jonathan M.

    2012-01-01

    Purpose of Review This review will examine the unique susceptibility of premature infants to oxidative stress, the role of reactive oxygen species (ROS) in the pathogenesis of common disorders of the preterm infant, and potential for therapeutic interventions using enzymatic and/or non-enzymatic antioxidants. Recent Findings Oxidative stress is caused by an imbalance between the production of ROS and the ability to detoxify them with the help of antioxidants. The premature infant is especially susceptible to ROS-induced damage because of inadequate antioxidant stores at birth, as well as impaired upregulation in response to oxidant stress. Thus, the premature infant is at increased risk for the development of ROS-induced diseases of the newborn, such as bronchopulmonary dysplasia, retinopathy of prematurity, necrotizing enterocolitis, and periventricular leukomalacia. Summary Potential therapies for ROS-induced disease include both enzymatic and non-enzymatic antioxidant preparations. More research is required to determine the beneficial effects of supplemental antioxidant therapy. PMID:21150443

  8. Ultrasound in Detection of Developmental Hip Dysplasia in Premature Born Children

    OpenAIRE

    Misanovic, Verica; Jonuzi, Fedzat; Maksic-Kovacevic, Hajra; Rahmanovic, Selma

    2015-01-01

    Introduction: Developmental hip dysplasia represents the most common deformation of locomotor system in children. Developmental modulation of the hip is expressed during first year of life which is important for early diagnosis and treatment. Therefore, in the practice, it is very important to set a diagnosis early with application of simple and convenient methods (ultrasound) in order to achieve fast and efficient therapeutical effect and avoid permanent disability. Aim: The aim of this pape...

  9. Analysis on high risk factors of bronchopulmonary dysplasia in very low birth weight infants%极低出生体重儿支气管肺发育不良的高危因素分析

    Institute of Scientific and Technical Information of China (English)

    杨亚娟; 刘晓红; 齐利峰; 李腾; 卫敏超

    2013-01-01

    Objective: To explore the clinical high risk factors of bronchopulmonary dysplasia (BPD) in very low birth weight infants. Methods: The clinical data of 199 very low birth weight infants who were treated in NICU of the hospital were analysed retrospectively, then they were divided into BPD group and nori - BPD group; the probable risk factors of BPD were analyzed. Results: Among 199 very low birth weight infants, 48 infants were diagnosed as BPD, the morbidity was 24. 1%. There were statistically significant differences in the time of mechanical ventilation, the time of oxygen inhalation, the time of oxygen inhalation with oxygen concentration >40% , intrauterine infection, combined with patent ductus arteriosus, and nosocomial infection between BPD group and non - BPD group (P 40% were the risk factors of BPD. Conclusion: Preventing intrauterine infection can reduce the morbidity of BPD, mechanical ventilation for a long time and oxygen inhalation with high oxygen concentration are high risk factors of BPD.%目的:探讨极低出生体重儿发生支气管肺发育不良(Bronchopulmonary Dysplasia,BPD)的临床高危因素.方法:回顾性分析NICU收治的极低出生体重儿199例患儿的临床资料,根据是否发生BPD分为BPD组和非BPD组,分析BPD发生的可能危险因素.结果:199例低出生体重儿中有48例发生BPD,发病率为24.1%.与非BPD患儿组相比,BPD组患儿在机械通气时间、总吸氧时间、吸氧浓度>40%时间、宫内感染、合并动脉导管未闭、院内感染等方面比较差异有统计学意义(P<0.05),Logistic回归分析结果显示机械通气时间、宫内感染、吸氧浓度>40%时间为疾病发生的危险因素.结论:预防宫内感染可降低BPD的发生率,长时间机械通气及高浓度吸氧为BPD发生的高危因素.

  10. 支气管肺发育不良的防治——证据及临床应用%Prevention and therapy of bronchopulmonary dysplasia - evidence and clinical practice

    Institute of Scientific and Technical Information of China (English)

    Wolfgang THOMAS; Christian P SPEER; 钱莉玲

    2007-01-01

    The knowledge on the pathogenetic mechanisms of bronchopulmonary dysplasia (BPD) has increased considerably over recent years. However, the incidence of the disease has not substantially been changed by our therapeutic approaches. This review summarizes the existing evidence for a number of respiratory and medical strategies to prevent or ameliorate the disease and gives recommendations for clinical practice. Oxygen plays an important pathogenetic and therapeutic role for BPD. Targeting infants at lower oxygen saturation levels than traditionally used seems to confer major advantages. There is no sufficient evidence for a routine use of respiratory strategies like permissive hypercapnia or inhaled nitric oxide to prevent BPD. Diuretics can ameliorate lung function transiently. High intramuscular doses of vitamin A can reduce the risk of BPD. Early or prophylactic surfactant might also be advantageous. Postnatal corticosteroids are effective but,due to their severe side effects, should be restricted to the severest cases. Alpha1-proteinase inhibitor and superoxide dismutase have no proven benefits for BPD. The role of erythromycin has not been completely elucidated yet. Innovative strategies like Clara Cell 10kD protein still have to be assessed in future trials.

  11. Pulmonary arterial hypertension associated with bronchopulmonary dysplasia and congenital heart disease in preterm infants. A case report of a preterm infant with recurrent pulmonary hypertension after corrective cardiac surgery and review of the literature.

    Science.gov (United States)

    Muneuchi, Jun; Kuraoka, Ayako; Watanabe, Mamie; Ochiai, Yoshie; Joo, Kunitaka

    2015-01-01

    In preterm infants with congenital heart disease, concomitant bronchopulmonary dysplasia (BPD) is associated with relatively poor clinical outcomes because of the increased pulmonary vascular resistance and adverse effects of inflammation on the damaged lungs, even after surgery. We present herein a 1-year-old female who developed recurrent pulmonary arterial hypertension 6 months after closure of a ventricular septal defect. She was born at 26 weeks of gestation (birth weight, 470 g), and developed BPD requiring oxygen supplementation. Her systemic-to-pulmonary blood flow ratio was 2.1 preoperatively and 1.0 postoperatively, pulmonary arterial pressure was 61/15 (mean 39) mmHg preoperatively and 41/17 (mean 24) mmHg postoperatively, and pulmonary vascular resistance was 4.2 mmHg/L·minute·m(2) preoperatively and 3.6 mmHg/L·minute·m(2) postoperatively. At 1 year of age, echocardiography showed an increase in her estimated right ventricular pressure, indicating worsening pulmonary hypertension. After 3 years of treatment with oxygen supplementation, prostacyclin, and bosentan, her pulmonary arterial pressure improved to the normal range. The pathophysiology of pulmonary arterial hypertension is heterogeneous in preterm infants with congenital heart disease and concomitant BPD. Careful management of these patients is warranted even after corrective cardiac surgery.

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

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    Marta M. G. B. Mataloun

    1999-12-01

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

  13. Foveal dysplasia evident by optical coherence tomography in patients with a history of retinopathy of prematurity.

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    Recchia, Franco M; Recchia, Cynthia C

    2007-01-01

    To describe the optical coherence tomography (OCT) findings for patients with a history of retinopathy of prematurity (ROP). Clinical records, fundus photographs, and OCT findings for consecutive patients aged 8 years and older who had a history of ROP were reviewed. The main outcome measures were best-corrected visual acuity, central foveal thickness (CFT), macular anatomy, and foveal contour by OCT. Twenty eyes of 12 patients (median age, 15 years) were studied. Median gestational age at birth was 25 weeks. Six eyes had received peripheral retinal ablation for threshold ROP. Median best-corrected visual acuity was 20/40 (range, 20/20 to counting fingers). CFT was >220 microm in 70% of eyes and >240 microm in 35% of eyes. In all eyes, foveal contour was abnormal, with foveal depression either absent (n = 7 [35%]) or shallow (n = 13 [65%]). Preservation of multiple inner retinal layers within the fovea was seen in 14 eyes (70%). Vitreomacular traction or subretinal fluid was not seen in any eye. Anomalies in foveal anatomy by OCT may be a vestige of prematurity, appear to be independent of prior retinopexy, and can still be associated with excellent visual acuity.

  14. The Angiotensin Converting Enzyme Insertion/Deletion polymorphism is not associated with an increased risk of death or bronchopulmonary dysplasia in ventilated very low birth weight infants

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    Baier R John

    2004-12-01

    Full Text Available Abstract Background The ACE gene contains a polymorphism consisting of either the presence (insertion, I or absence (deletion, D of a 287 bp alu repeat in intron 16. The D allele is associated with increased ACE activity in both tissue and plasma. The DD genotype is associated with risk of developing ARDS and mortality. The frequency of the D allele is higher in patients with pulmonary fibrosis, sarcoidosis and berylliosis. The role of this polymorphism has not been studied in the development of BPD in the premature newborn. Methods ACE I/D genotype was determined in 245 (194 African-American, 47 Caucasian and 4 Hispanic mechanically ventilated infants weighing less than 1250 grams at birth and compared to outcome (death and/or development of BPD. Results The incidence of the D allele in the study population was 0.58. Eighty-eight (35.9% infants were homozygous DD, 107 (43.7% were heterozygous ID and 50 (20.4% were homozygous II. There were no significant differences between genotype groups with respect to ethnic origin, birth weight, gestation, or gender. There was no effect of the ACE I/D polymorphism on mortality or development of BPD (O2 on 28 days or 36 weeks PCA. Secondary outcomes (intraventricular hemorrhage and periventricular leukomalacia similarly were not influenced by the ACE ID polymorphism. Conclusions The ACE I/D polymorphism does not significantly influence the development of BPD in ventilated infants less than 1250 grams.

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

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    Jose L. Tapia

    2006-02-01

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

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

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    Daiana Evangelista

    2009-03-01

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

  17. VACCINATION OF PREMATURE INFANTS AND CHILDREN WITH CONGENITAL HEART DISEASE IN IRKUTSK USING CONJUGATED PNEUMOCOCCAL VACCINES

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    S. V. Il'ina

    2013-01-01

    Full Text Available Study aim: analyzing the results of pneumococcal infection vaccination conducted to reduce infantile morbidity and mortality in 2011-2012 at the expenses of the Irkutsk municipal budget. Patients and methods. Vaccination using the 7- and 13-valent pneumococcal conjugated vaccine was conducted for more than 700 risk group children: premature infants, children with congenital heart diseases or bronchopulmonary dysplasia from 2 months to 2 years of age. 193 vaccinated children had been observed for 1.5 years. 30% of premature infants and 46% of children with congenital heart diseases were vaccinated using the PCV7/PCV13 vaccine at the age of 2-6 months, 52 and 40% - at the age of 7-11 months, accordingly. The PCV7/PCV13 vaccine was administered together with other vaccines of the national preventive vaccination calendar in 65% of cases. Results. Rate of general post-vaccinal reactions (body temperature increase from 37.6 to 38.0oC – 4%; no local reactions were registered. No other unfavorable phenomena were noted in the post-vaccinal period. No cases of pneumonia, meningitis, acute otitis media and bronchoobstructive syndrome were registered within the observation period. Conclusions: pneumococcal infection vaccination of premature infants with congenital heart diseases and bronchopulmonary dysplasia conducted in Irkutsk proved high efficacy and safety of the used vaccine – PCV7/PCV13. 

  18. Bronchopulmonary sequestration and dextrocardia.

    Science.gov (United States)

    Ivanovi-Herceg, Z; Majerić-Kogler, V; Mazuranić, I; Neralić-Meniga, I; Puljić, I

    1998-06-01

    Bronchopulmonary sequestration (BPS) is usually a rare congenital anomaly, which is most frequently extralobar or intralobar. The case of a patient with positional congenital anomaly--dextrocardia (situs thoracalis inversus) and intrapulmonary sequestration (IPS) is presented. Clinical and radiological characteristics of EPS and IPS are discussed, and new combinations of congenital anomalies with bronchopulmonary sequestration are described, dextrocardia and intrapulmonary sequestration. The importance of the algorithm of diagnostic examinations is emphasized, from detection of bronchopulmonary sequestration on the chest roentgenogram to establishing a definite diagnosis by means of angiography.

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

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    Tathiana Ghisi de Souza

    2009-03-01

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

  20. Optimal oxygen saturation in premature infants

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    Meayoung Chang

    2011-09-01

    Full Text Available There is a delicate balance between too little and too much supplemental oxygen exposure in premature infants. Since underuse and overuse of supplemental oxygen can harm premature infants, oxygen saturation levels must be monitored and kept at less than 95% to prevent reactive oxygen species-related diseases, such as retinopathy of prematurity and bronchopulmonary dysplasia. At the same time, desaturation below 80 to 85% must be avoided to prevent adverse consequences, such as cerebral palsy. It is still unclear what range of oxygen saturation is appropriate for premature infants; however, until the results of further studies are available, a reasonable target for pulse oxygen saturation (SpO2 is 90 to 93% with an intermittent review of the correlation between SpO2 and the partial pressure of arterial oxygen tension (PaO2. Because optimal oxygenation depends on individuals at the bedside making ongoing adjustments, each unit must define an optimal target range and set alarm limits according to their own equipment or conditions. All staff must be aware of these values and adjust the concentration of supplemental oxygen frequently.

  1. 阿奇霉素防治早产儿支气管肺发育不良的系统评价%Azithromycin for preventing bronchopulmonary dysplasia in preterm infants:a systematic review

    Institute of Scientific and Technical Information of China (English)

    李茂军; 陈昌辉; 吴青; 石伟; 阳倩; 唐彬秩

    2015-01-01

    Objective To assess the effectiveness and safety of Azithromycin for preventing bronchopulmonary dysplasia(BPD) in preterm infants in order to provide reference for the clinical treatment of BPD.Methods The electronic retrieval were conducted to review randomized controlled trials (RCTs) from The Cochrane Library,PubMed,EM-Base,China Biological Medicine Database (CBM),Chinese Journals full-text Database (CNKI),Chinese Technological Journals Database(VIP) and Wan Fang Digital Journal Full-text Database by free texts and medical subject headings.The retrieval time was from inception to Dec.2012.Randomized or quasi-randomized studies were conducted by comparing Azithromycin therapy and no treatment or placebo management to prevent BPD among the preterm or low birth weight infants were included.The reference lists of relevant trials and conference proceedings were searched.Risk biases of the trials were assessed.Statistical analysis was performed by using Revman 5.1 offered by Cochrane.Results A total of 2 trials including 328 preterm infants were included.No significant difference was observed between the Azithromycin group and the control group in the incidence of BPD(RR =0.81,95% CI 0.55-1.19).The risk ratio of death and post-natal steroid used in the Azithromycin group were lower,but there was no difference.The risk ratio of length of stay was not significantly different.The rate of BPD in preterm infants who had a positive respiratory culture of Ureaplasma between the preventative Azithromycin group and the placebo group showed that the incidence of BPD was significantly less in the Azithromycin group.Conclusions The available data are insufficient to make a recommendation regarding treatment with prophylactic Azithromycin to prevent BPD in preterm infants.More RCTs with large-scale and high-quality are required to provide more reliable evidence.%目的 系统评价阿奇霉素防治早产儿支气管肺发育不良(BPD)的有效性及安全性,以期为本病

  2. Kidney Dysplasia

    Science.gov (United States)

    ... Disease Ectopic Kidney Medullary Sponge Kidney Kidney Dysplasia Kidney Dysplasia What is kidney dysplasia? Kidney dysplasia is a condition in which ... Kidney dysplasia in one kidney What are the kidneys and what do they do? The kidneys are ...

  3. 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

  4. Increased release and activity of matrix metalloproteinase-9 in patients with mandibuloacral dysplasia type A, a rare premature ageing syndrome.

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    Lombardi, F; Fasciglione, G F; D'Apice, M R; Vielle, A; D'Adamo, M; Sbraccia, P; Marini, S; Borgiani, P; Coletta, M; Novelli, G

    2008-10-01

    Mandibuloacral dysplasia type A (MADA; OMIM 248370), a rare disorder caused by mutation in the LMNA gene, is characterized by post-natal growth retardation, craniofacial and skeletal anomalies (mandibular and clavicular hypoplasia, acroosteolysis, delayed closure of cranial sutures, low bone mass and joint contractures), cutaneous changes and partial lipodystrophy. Little is known about the molecular mechanisms by which LMNA mutations produce bone alterations. An altered bone extracellular matrix (ECM) remodelling could play a pivotal role in this disorder and influence part of the typical bone phenotype observed in patients. Therefore, we have focused our investigation on matrix metalloproteinases (MMPs), which are degradative enzymes involved in ECM degradation and ECM remodelling, thus likely contributing to the altered bone mineral density and bone metabolism values seen in five MADA patients. We evaluated the serum levels of several MMPs involved in bone development, remodelling and homeostasis, such as MMP-9, -2, -3, -8 and -13, and found that only the 82 kDa active enzyme forms of MMP-9 are significantly higher in MADA sera compared with healthy controls (n = 16). The serum level of MMP-3 was instead lower in all patients. No significant differences were observed between controls and MADA patients for the serum levels of MMP-2, -8 and -13 and of tissue inhibitor of metalloproteinase 2, a natural inhibitor of MMP-9. Similarly, normal serum levels of tumour necrosis factor alpha (TNF-alpha), interleukin (IL)-6 and IL-1beta were detected. These data suggest a possible involvement of MMP-9 in MADA disease, underlying the potential use in diagnosis and therapy.

  5. [Pseudotumoral allergic bronchopulmonary aspergillosis].

    Science.gov (United States)

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

    2000-06-01

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

  6. 不同时间使用地塞米松对支气管肺发育不良的防治作用%Clinical study of Dexamethasone in different time for treatment and prevention of bronchopulmonary dysplasia

    Institute of Scientific and Technical Information of China (English)

    齐骥; 杨震英

    2015-01-01

    Objective To study the effect of using Dexamethasone in different time for treatment and prevention of bronchopulmonary dysplasia (BPD), in order to provide the clinical reference as to whether dexamethasone can be used to prevent and treat BPD.Methods A total of 135 very low birth weight infants hospitalized for 1 week who still survived on mechanical ventilation were studied.The total cases were divided into early Dexamethasone group and none Dexamethasone group.Whether early using dexamethasone could prevent BPD or not were observed.The BPD infants were divided into 3 groups, early Dexamethasone group, later Dexamethasone group and none Dexamethasone group.The effect of using Dexamethasone on BPD in different time were observed.Results (1) Among these 135 infants,there were 65 infants developing BPD.The incidence of BPD was 48.15%.There were no significant differences in the prevalence of BPD, the death rate and the disease index of BPD between the early Dexamethasone group and the none Dexamethasone group (all P > 0.05).(2) There were no significant differences in mechanical ventilation time among the 3 BPD groups [(19.81 ± 5.67) d vs (22.32 ± 6.20) d, (21.46 ± 7.02) d, P =0.405].But the average time of inhaling oxygen and hospital stays of later Dexamethasone BPD group were shorter than early Dexamethasone BPD group and none Dexamethasone BPD group.The differences were significant [(37.27 ± 10.14) d vs (45.96 ± 9.91) d,(43.42 ±8.73) d,P=0.012;(64.11 ±8.14) d vs (76.13 ±7.57) d,(68.59 ±8.53) d,P =0.000].(3) The incidence of infection (90.90%, 100.00% vs 81.90%), hyperglycemia (27.27 %, 30.43 % vs 10.00 %), hypertension(9.09% ,13.04% vs 0) and neonatal necrotizing enterocolitis(22.73% ,34.78% vs 15.00%) of all the infants who received Dexamethasone were higher than the infants who did not receive Dexamethasone.But the differences showed no significance(all P > 0.05).(4) There were no significant differences among the 3 BPD

  7. Anti-inflammtory effects of erythropoietin on hyperoxia-induced bronchopulmonary dysplasia in newborn rats%促红细胞生成素对高氧肺损伤炎症反应病理过程的影响

    Institute of Scientific and Technical Information of China (English)

    王晓蕾; 薛辛东

    2009-01-01

    Objective Bronchopulmonary dysplasia (BPD) is a multifactorial disease resulting from the impact of injury (including oxygen toxicity, barotrauma, volutrauma, and infection) on the immature lung. Oxygen toxicity is thought to be a major contributing factor in the pathogenesis in BPD. Previous animal studies have shown that exposure to hyperoxia in the neonatal period causes lung structural changes that are similar to the histology seen in human infants with BPD. Erythropeietin (EPO) has pleiotropic actions including antioxidant, anti-apeptotic, anti-inflammatory and angiogenic effects. Animal experiments reveal that EPO may have protective effects on hyperoxic lung injury, but the mechanisms remain unknown. The aim of the study was to evaluate the anti-inflammatory effects and understand mechanism of action of EPO on the hyperexia-induced BPD in newborn rats. Method Several litters of Wistar pups were pooled together within 12 hours after birth and randomly divided into four groups: Ⅰ. air-exposed control group, Ⅱ. air-exposed human recombinant erythropeietin (rhEPO)-treated group, Ⅲ. hyperoxia-expesed placebo group and Ⅳ. hyperoxia-expesed rhEPO-treated group. Group Ⅲ and Ⅳ rats were exposed to 85% oxygen. Group Ⅱ and Ⅳ rats received rhEPO (1200 IU/kg) subcutaneously on postnatal days 0 and 2. Group Ⅰ and Ⅲ received 0.9% saline in the same way. Pups from each group were sacrificed on days 3, 7, and 14. Blood hemoglobin concentration, hematocrit and platelet count were determined by blood cell analyzer. Total protein content in bronchoaiveolar lavage fluid (BALF) and myelopsroxidase (MPO) were measured by biochemical assay. Changes of monocyte chemoattractant protein-1 (MCP-1) and cytokine-induced neutrophil cbemoattraclant-1 (CINC-1) mRNA expressions were measured by BT-PCR. Result In group Ⅲ, there were a few inflammatory cells infiltrations in interstitium on day 3 and inflammatory response worsened on day 7. Alveolar and capillary

  8. Inflammatory and oxidative stress airway markers in premature newborns of hypertensive mothers.

    Science.gov (United States)

    Madoglio, R J; Rugolo, L M S S; Kurokawa, C S; Sá, M P A; Lyra, J C; Antunes, L C O

    2016-08-01

    Although oxidative stress and inflammation are important mechanisms in the pathophysiology of preeclampsia and preterm diseases, their contribution to the respiratory prognosis of premature infants of hypertensive mothers is not known. Our objective was to determine the levels of oxidative stress and inflammation markers in the airways of premature infants born to hypertensive and normotensive mothers, in the first 72 h of life, and to investigate whether they are predictors of bronchopulmonary dysplasia (BPD)/death. This was a prospective study with premature infants less than 34 weeks' gestation on respiratory support who were stratified into 2 groups: 32 premature infants of hypertensive mothers and 41 of normotensive women, with a mean gestational age of 29 weeks. Exclusion criteria were as follows: diabetes mellitus, chorioamnionitis, malformation, congenital infection, and death within 24 h after birth. The outcome of interest was BPD/death. Malondialdehyde (MDA), nitric oxide (NO), and interleukin 8 (IL-8) were measured in airway aspirates from the first and third days of life and did not differ between the groups. Univariate and multivariate statistical analyses were performed. The concentrations of MDA, NO, and IL-8 were not predictors of BPD/death. Premature infants who developed BPD/death had higher levels of IL-8 in the first days of life. The gestational age, mechanical ventilation, and a small size for gestational age were risk factors for BPD/death. In conclusion, the biomarkers evaluated were not increased in premature infants of hypertensive mothers and were not predictors of BPD/death.

  9. Safety evaluation of inhaled nitric oxide in treatment of bronchopulmonary dysplasia%长期吸入低浓度一氧化氮在支气管肺发育不良治疗中的安全性研究

    Institute of Scientific and Technical Information of China (English)

    韦秋芬; 李燕; 潘新年; 蒙丹华; 刘先知; 许靖; 韦玮

    2016-01-01

    Objective To discuss the safety of inhaled nitric oxide (iNO)for bronchopulmonary dysplasia (BPD).Methods We analysed 65newborns diagnosed as BPD from January 2012to December 2013in Maternal and Child Health Hospital of the Guangxi Zhuang Autonomous Region.They were divided into iNO group(n=35)and control group(n=30)according to whether administering iNO or not.The heart rate,blood pressure,oxygen supply time,assisted ventilation time,hospital stay,concentration of NO2 and methemoglobin,and the changes of coagulation function were observed dynamically before and after iNO treatment.Results There were significant differences in oxygen supply time[(37±9)d vs.(55±7)d], assisted ventilation time[(29±7)d vs.(35±9)d],hospital stay [(45±9)d vs.(54±7)d]between iNO group and control group(P﹤0.05).There were no obvious changes in heart rate,blood pressure and co-agulation function before and after iNO treatment(P﹥0.05).The incidences of the complications had no sig-nificant differences between the two groups (P﹥0.05).Conclusion Long-time iNO can shorten oxygen therapy time and assisted ventilation time,iNO therapy in BPD doesn′t found serious adverse effect to cardio-vascular function as well as coagulation function,and doesn′t increase the risk of intracranial hemorrhage and retinopathy of prematurity.%目的:探讨吸入一氧化氮(inhaled nitric oxide,iNO)治疗支气管肺发育不良的疗效及安全性。方法收集2012年1月至2013年12月在我院新生儿科收治的确诊支气管肺发育不良患儿65例,根据是否给予iNO治疗,将患儿分为iNO治疗组35例及对照组30例。动态观察iNO治疗前后两组患儿的心率、血压、给氧时间、辅助通气时间、住院时间、NO2浓度、高铁血红蛋白浓度及凝血功能的变化。结果 iNO治疗组与对照组患儿的吸氧时间[(37±9)d vs.(55±7)d],辅助通气时间[(29±7)d vs.(35±9)d]及住院时间[(45±9)d vs.(54±7)d]比较,

  10. Metastasizing Bronchopulmonary Leiomyosarcoma

    Directory of Open Access Journals (Sweden)

    Speros Livieratos MD

    2015-04-01

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

  11. Endosonography in bronchopulmonary disease

    DEFF Research Database (Denmark)

    Vilmann, Peter; Annema, Jouke; Clementsen, Paul

    2009-01-01

    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....... 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...

  12. Study on risk factors and follow-up outcome at 2 years in preterm infants with bronchopulmonary dysplasia%早产儿支气管肺发育不良危险因素及2岁时随访结局

    Institute of Scientific and Technical Information of China (English)

    尹燕丹; 祁媛媛; 洪达; 王传凯; 张晓波; 钱莉玲

    2016-01-01

    目的:探讨早产儿发生支气管肺发育不良( BPD)的危险因素及远期随访结局。方法以2012年1月至2013年12月在复旦大学附属儿科医院新生儿科病房住院的胎龄≤32周、出生体重≤1500 g及生后7 d内入院的BPD早产儿为BPD组,同期入住我院的非BPD早产儿中选取与BPD组等同样本量的病例为对照组。采集与BPD发生的母亲和新生儿因素行单因素分析和多因素分析。同时统计BPD早产儿生后1岁内和~2岁的支气管炎、肺炎、喘息发作次数和住院次数等指标。结果 BPD组和对照组均纳入了156例早产儿。单因素分析显示,BPD组母亲年龄( P=0.046)、先兆子( P=0.025)和阴道产( P<0.001)比例显著高于对照组;BPD组出生胎龄、出生体重显著低于对照组( P均<0.001)。BPD组1和5 min Apgar评分,败血症≥72 h、动脉导管未闭( PDA)、早产儿视网膜病变、应用肺表面活性物质、呼吸机相关性肺炎、机械通气≥7 d的比例均与对照组差异有统计学意义。多因素Logistic回归分析显示胎龄( OR=0.46,95%CI:0.37~0.58)、机械通气≥7 d(OR=9.47,95%CI:3.70~24.27)、PDA(OR=2.21,95%CI:1.18~4.12)、先兆子(OR=4.91,95%CI:1.26~19.15)是发生BPD的危险因素。BPD组1岁以内支气管炎、喘息的发生率高于对照组,再入院率两组差异无统计学意义;BPD组生后~2岁较生后1岁以内肺炎的发生率显著下降,支气管炎、喘息的发生率及再入院率差异无统计学意义。结论低出生胎龄、机械通气≥7 d、PDA、先兆子是发生BPD的危险因素;BPD早产儿在生后1年以内下呼吸道感染的发生率增高。%Objective To investigate the main risk factors and long term outcome of preterm infants with bronchopulmonary dysplasia( BPD). Methods Children with BPD were collected from neonatal intensive care units( NICU)in Children

  13. PREMATURITY, NEONATAL HEALTH STATUS, AND LATER CHILD BEHAVIORAL/EMOTIONAL PROBLEMS: A SYSTEMATIC REVIEW.

    Science.gov (United States)

    Cassiano, Rafaela G M; Gaspardo, Claudia M; Linhares, Maria Beatriz M

    2016-05-01

    Preterm birth can impact on child development. As seen previously, children born preterm present more behavioral and/or emotional problems than do full-term counterparts. In addition to gestational age, neonatal clinical status should be examined to better understand the differential impact of premature birth on later developmental outcomes. The aim of the present study was to systematically review empirical studies on the relationship between prematurity, neonatal health status, and behavioral and/or emotional problems in children. A systematic search of the PubMed, PsycINFO, Web of Science, and LILACS databases for articles published from 2009 to 2014 was performed. The inclusion criteria were empirical studies that evaluated behavioral and/or emotional problems that are related to clinical neonatal variables in children born preterm. Twenty-seven studies were reviewed. Results showed that the degree of prematurity and birth weight were associated with emotional and/or behavioral problems in children at different ages. Prematurity that was associated with neonatal clinical conditions (e.g., sepsis, bronchopulmonary dysplasia, and hemorrhage) and such treatments as corticoids and steroids increased the risk for these problems. The volume and abnormalities of specific brain structures also were associated with these outcomes. In conclusion, the neonatal health problems associated with prematurity present a negative impact on later child emotional and adapted behavior. © 2016 Michigan Association for Infant Mental Health.

  14. Investigation of Early Chest Radiograohic Images of Respiratory Distress Syndrome Complicated with Bronchopulmonary Dysplasia%呼吸窘迫综合征并发支气管肺发育不良早期胸部影像的研究

    Institute of Scientific and Technical Information of China (English)

    劳国荣; 张剑; 罗世康; 叶伙华; 刘超凡

    2013-01-01

    Objective:To investigate the imaging findings and significance of neonatal respiratory distress syndrome(NRDS)complicated with bronchopulmonary dysplasia(BPD),and improve the understandings of complication of chest images especially the early chest images. Method:66 newborns with neonatal respiratory distress syndrome complicated with bronchopulmonary dysplasia(BPD)admitted in the hospital from January 2006 to November 2011 were involved in this research. These cases who had clear discharge diagnosis,full chest image data and complete clinical data and followed for 2 years were analyzed retrospectively. Result:Review of imaging after average treatment for 2-3 weeks showed that 66 cases who treated with NRDS complicated with BPD all showed lungs brightness reduction,ground glass visible changes,planes and flocculent cloud shadow patches of both lung fields and blurred boundary performance. Furthermore,absorption was relatively slower and lasting time was relatively longer compared with image of pulmonary edema during the treatment. 66 cases ultimately had varied degrees of grid-like vesicle-like shadow of change and 25 cases had no significant change after continuous film image. Conclusion:Chest radiographic image change is the most intuitive,most convenient,fastest and first method of clinical dynamic observation. Diagnosis of neonatal bronchopulmonary dysplasia is mainly according on clinical history and imaging data. Although image of chest radiograph and CT has no significantly character,it is very important for the diagnosis. The high resolution CT can provide some valuable signs which contribute to the early diagnosis of development of BPD and gain important time for the prevention and treatment of patients.%  目的:探讨新生儿呼吸窘迫综合征(NRDS)并发支气管肺发育不良(BPD)影像学表现及其意义,以提高对该并发症的胸部影像的认识,尤其是该并发症的早期胸部影像的认识。方法:选择2006

  15. Rhinovirus-induced Airway Cytokines and Respiratory Morbidity in Severely Premature Children

    Science.gov (United States)

    Perez, Geovanny F.; Pancham, Krishna; Huseni, Shehlanoor; Jain, Amisha; Rodriguez-Martinez, Carlos E.; Preciado, Diego; Rose, Mary C.; Nino, Gustavo

    2017-01-01

    Background Rhinovirus (RV) has been linked to the pathogenesis of asthma. Prematurity is a risk factor for severe RV infection in early life, but is unknown if RV elicits enhanced pro-asthmatic airway cytokine responses in premature infants. This study investigated if young children born severely premature (<32 weeks gestation) exhibit airway secretion of Th2 and Th17 cytokines during natural RV infections and if RV-induced Th2-Th17 responses are linked to more respiratory morbidity in premature children during the first two years of life. Methods We measured Th2 and Th17 nasal airway cytokines in a retrospective cohort of young children aged 0–2 years with PCR-confirmed RV infection or non-detectable virus. Protein levels of IL-4, IL-13, TSLP and IL-17 were determined with multiplex immunoassays. Demographic and clinical variables were obtained by electronic medical record (EMR) review. Results The study comprised 214 children born full term (n=108), pre-term (n=44) or severely premature (n=62). Natural RV infection in severely premature children was associated with elevated airway secretion of Th2 (IL-4 and IL-13) and Th17 (IL-17) cytokines, particularly in subjects with history of bronchopulmonary dysplasia. Severely premature children with high RV-induced airway IL-4 had recurrent respiratory hospitalizations (median 3.65 hosp/year; IQR 2.8–4.8) and were more likely to have at least one pediatric intensive care unit admission during the first two years of life (OR 8.72; 95% CI 1.3–58.7; p=0.02). Conclusions Severely premature children have increased airway secretion of Th2 and Th17 cytokines during RV infections, which is associated with more respiratory morbidity in the first two years of life PMID:25640734

  16. Risk factors of different severities bronchopulmonary dysplasia in preterm infants%不同程度支气管肺发育不良早产儿的出生情况及临床疗效分析

    Institute of Scientific and Technical Information of China (English)

    陈均; 陈健; 陆俏群

    2014-01-01

    目的:分析不同程度早产儿支气管肺发育不良(BPD)的高危因素,指导该病的临床防治。方法收集2011年2月~2014年2月在我院新生儿科住院并确诊为 BPD 的128例早产儿的临床资料,按 BPD 的严重程度分为轻度、中度、重度三组,对各组的资料进行比较分析。结果轻度、中度、重度 BPD 组分别纳入患儿57例、45例、26例,各组患儿在性别、胎次、分娩方式、母患妊高征、产前应用激素、有否胎儿窘迫、出生复苏、肺表面活性物质应用、合并肺出血等方面的差异无统计学意义(P >0.05);随着 BPD 程度的加重,胎龄和出生体重呈下降趋势,阿氏评分1 min12 h、母亲孕期合并感染、羊水胎粪污染的比例上升(P 0.05).The birth weights and gestational age decreased with the in-creasing severity of BPD,meanwhile the incidence of Apgar 1 minute score 12 hours,infection in pregnancy period,meconium contamination of the amniotic fluid were in-creased,there were statistically significant differences (P <0.05).With disease worsening,longer duration of oxygen uptake,transfusion of more concentrated red blood cells,positive rates of hemoculture and sputum cul-ture specimen,more patent ductus arteriosus complications and more invasiveness therapy (mechanical ventila-tion,tracheal intubation twice or more,and nasogastric intubation)occurred,group-paired comparison yiel-ded statistically significant differences (P <0.05). Conclusion Avoidance of premature delivery,low birth weight newborn is the basis of prevention BPD.The key interventions for reducing the severity of BPD are pre-vention and therapy of infection,shortening the time of mechanical ventilation and oxygen uptake.

  17. "Assessment of retinopathy of prematurity among 150 premature neonates in Farabi eye hospital "

    Directory of Open Access Journals (Sweden)

    "Riazi Esfahani M

    2001-05-01

    Full Text Available The aim of this study was to estimate the incidence of retinopathy of permaturity (ROP and to evaluate possible neonatal risk factors for ROP. The main study was a cross-sectional study including 150 high-risk neonates born at teaching hospitals of Tehran universities referring to to Farabi Eye Hospital. The chossing critertia were birth weight less than 2500 g or gestational age younger than 37 weeks. ROP was present in 9(6% newborns, all of whom aged less than 32 weeks a birth. There was also strong association between ROP and birth weight, oxygen administration, respiratory distress syndrome and intraventricular hemorrhage. There also seems to be a higher risk for developing ROP in female neoates, those who were born by multiple gestaional pregnancies or were treated by phototherapy or transfusion and those who had suffered from bronchopulmonary dysplasia or seiss.Prematurity per se remains the strongest risk factof for ROP. Suitable criteria for screening of ROP seems to be gestational age younger than 32 weeks or birth weight less than 1500 g

  18. Inflammatory and oxidative stress airway markers in premature newborns of hypertensive mothers

    Directory of Open Access Journals (Sweden)

    R.J. Madoglio

    2016-01-01

    Full Text Available Although oxidative stress and inflammation are important mechanisms in the pathophysiology of preeclampsia and preterm diseases, their contribution to the respiratory prognosis of premature infants of hypertensive mothers is not known. Our objective was to determine the levels of oxidative stress and inflammation markers in the airways of premature infants born to hypertensive and normotensive mothers, in the first 72 h of life, and to investigate whether they are predictors of bronchopulmonary dysplasia (BPD/death. This was a prospective study with premature infants less than 34 weeks’ gestation on respiratory support who were stratified into 2 groups: 32 premature infants of hypertensive mothers and 41 of normotensive women, with a mean gestational age of 29 weeks. Exclusion criteria were as follows: diabetes mellitus, chorioamnionitis, malformation, congenital infection, and death within 24 h after birth. The outcome of interest was BPD/death. Malondialdehyde (MDA, nitric oxide (NO, and interleukin 8 (IL-8 were measured in airway aspirates from the first and third days of life and did not differ between the groups. Univariate and multivariate statistical analyses were performed. The concentrations of MDA, NO, and IL-8 were not predictors of BPD/death. Premature infants who developed BPD/death had higher levels of IL-8 in the first days of life. The gestational age, mechanical ventilation, and a small size for gestational age were risk factors for BPD/death. In conclusion, the biomarkers evaluated were not increased in premature infants of hypertensive mothers and were not predictors of BPD/death.

  19. 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...

  20. Ectodermal dysplasia

    Science.gov (United States)

    ... the womb. Alternative Names Anhidrotic ectodermal dysplasia; Christ-Siemens-Touraine syndrome Images Skin layers References Grange DK. Ectodermal dysplasias. Rimoin D, Korf B, eds. In: Emery and Rimoin's Principles and Practice of Medical Genetics . 6th ed. Philadelphia, PA: Elsevier; 2013:chap ...

  1. Management of patent ductus arteriosus in premature infants.

    Science.gov (United States)

    Bhat, Rama; Das, Utpala G

    2015-01-01

    Patency of the ductus arteriosus is required for fetal survival in utero. In infants born prematurely, ductus fails to close and shunt reverses from left to right. Incidence of patent ductus arteriosus (PDA) is inversely proportional to the gestational age. A large PDA (>1.5 mm diameter) with left to right shunt in very low birth weight infants can cause pulmonary edema, congestive heart failure, pulmonary hemorrhage and increase the risk for bronchopulmonary dysplasia. Attempts to prevent or close the duct by pharmacological or surgical methods have not changed the morbidity or the long term outcome. Pharmacological treatment with indomethacin or ibuprofen is successful in 75 to 80 % of infants but its use also exposes these infants to undesirable side effects like gastrointestinal bleeding, perforation and necrotizing enterocolitis. Prophylactic therapy with indomethacin or ibuprofen to prevent PDA has not altered the morbidity or long term outcome. Currently, there is a dilemma as to how to treat, when to treat and whom to treat. Recent literature suggests a trial of conservative management during the first week followed by selective use of anti-inflammatory drugs. Surgical ligation is reserved for infants who fail medical therapy and still remain symptomatic. Spontaneous closure of the PDA has been reported in up to 40-67 % of very low birth weight (VLBW) infants by 7 d. In this review authors discuss these controversies and propose a more rational approach.

  2. Long-term respiratory consequences of premature birth at less than 32 weeks of gestation.

    Science.gov (United States)

    Greenough, Anne

    2013-10-01

    Chronic respiratory morbidity is a common adverse outcome of very premature birth, particularly in infants who had developed bronchopulmonary dysplasia (BPD). Prematurely born infants who had BPD may require supplementary oxygen at home for many months and affected infants have increased healthcare utilisation until school age. Chest radiograph abnormalities are common; computed tomography of the chest gives predictive information in children with ongoing respiratory problems. Readmission to hospital is common, particularly for those who have BPD and suffer respiratory syncytial virus lower respiratory infections (RSV LRTIs). Recurrent respiratory symptoms requiring treatment are common and are associated with evidence of airways obstruction and gas trapping. Pulmonary function improves with increasing age, but children with BPD may have ongoing airflow limitation. Lung function abnormalities may be more severe in those who had RSV LRTIs, although this may partly be explained by worse premorbid lung function. Worryingly, lung function may deteriorate during the first year. Longitudinal studies are required to determine if there is catch up growth. © 2013 Elsevier Ireland Ltd. All rights reserved.

  3. Fibrous dysplasia

    Science.gov (United States)

    ... chap 22. Czerniak B. Fibrous dysplasia and related lesions. In: Czerniak B, ed. Bone Tumors. 2nd ... Assistant Professor of Pediatrics, University of Washington School of Medicine, Seattle, WA. Also ...

  4. Bronchopulmonary lophomoniasis: emerging disease or unsubstantiated legend?

    Science.gov (United States)

    Martínez-Girón, Rafael; van Woerden, Hugo Cornelis

    2014-06-23

    The relationship between Lophomonas, a genus of multiflagellated protozoa, and respiratory pathology has recently received attention. Here, we summarize the origin, mode of transmission, pathogenic mechanism and relevant clinical data of bronchopulmonary lophomoniasis.

  5. Premature Contractions

    Science.gov (United States)

    ... Peripheral Artery Disease Venous Thromboembolism Aortic Aneurysm More Premature Contractions - PACs and PVCs Updated:Dec 15,2016 ... You felt this more-forceful beat. Types of premature contractions Premature atrial contractions (PACs) start in the ...

  6. 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.

  7. The value of improved family management model to reduce occurrence of death and rehospitalization of children with bronchopulmonary dysplasia after discharge%改良出院后家庭管理模式在降低支气管肺发育不良患儿出院后病死率及再住院率的价值

    Institute of Scientific and Technical Information of China (English)

    杨秀芳; 柳国胜

    2015-01-01

    目的 探讨改良出院后家庭管理模式对降低支气管肺发育不良(BPD)患儿出院后病死率及再住院率的价值.方法 选取2006年1月至2013年12月在中山大学附属中山医院新生儿监护室住院的BPD患儿为研究对象,其中2010年1月至2013年12月应用改良出院后家庭管理模式31例BPD患儿为试验组,2006年1月至2009年12月应用传统出院后家庭管理模式27例BPD患儿为对照组.应用x2检验比较2组出院后3个月内病死率和再住院率;应用t检验比较2组在校正胎龄40周及校正年龄3月龄时肺功能情况.结果 试验组出院后3个月内的病死率[3.22%(1/31例)]明显低于对照组[22.22%(6/27例)],差异有统计学意义(x2 =4.907,P<0.05).试验组再住院率[25.80%(8/31例)]明显低于对照组[77.78%(21/27例)],差异有统计学意义(x2=15.591,P <0.05).校正年龄3月龄时试验组(存活30例)呼吸频率低于对照组(存活21例),2组比较差异有统计学意义(t=9.225,P<0.05),试验组达峰时间、达峰容积、25%潮气量时呼气流速、50%潮气量时呼气流速和75%潮气量时呼气流速均高于对照组,2组比较差异均有统计学意义(t=3.992、3.301、4.645、3.152、2.916,P均<0.05),校正年龄3月龄时试验组BPD患儿的肺功能明显优于对照组.结论 改良出院后家庭管理模式有利于降低BPD患儿出院后3个月内的病死率和再住院率,有利于肺功能的改善.%Objective To explore the value of improved family management model to reduce the occurrence of death and rehospitalization rate of children with bronchopulmonary dysplasia (BPD) after discharge.Methods The children with BPD who were admitted in Neonatal Intensive Care Unit of Zhongshan Hospital Affiliated to Sun Yat-Sen University from January 2006 to December 2013 were enrolled.Thirty-one cases with BPD used with improved family management mode after discharge from January 2010 to December 2013 were as experimental group

  8. Recombinant human erythropoietin attenuates pulmonary inflammatory in newborn rats with chronical hyperoxia-induced bronchopulmonary dysplasia%重组人促红细胞生成素减轻慢性高体积分数氧致支气管肺发育不良新生大鼠的炎性反应

    Institute of Scientific and Technical Information of China (English)

    耿琳琳; 吕伟; 宋靖荣

    2015-01-01

    Objective To investigate anti-inflammatory effect of recombinant human erythropoietin(rhEPO) on bronchopulmonary dysplasia in newborn rats exposed to hyperoxia.Methods Ninety-six Wistar newborn rats were randomly divided into 4 groups after birth:room air-exposed control group,room air-exposed rhEPO treated group,hyperoxia-exposed group,and the hyperoxia-exposed rhEPO treated group.The last two groups were exposed to oxygen,FiO2 =850 mL/L,room air-exposed rhEPO treated and hyperoxia-exposed rhEPO treated group received rhEPO 2 400 IU/kg subcutaneously at birth,30 minutes' before oxygen exposure and 2 d after birth.The isodose of 9 g/L saline was given in the same way in room air-exposed controls and hyperoxia-exposed pups.Rats from each group were sacrificed on day 3,7 and 10.Lung histology was observed under microscope,and mRNA expression of monocyte chemoattractant protein-1 (MCP-1) and cytokine-induced neutrophil hemoattractant-1 (CINC-1) were determined with reverse transcriotion-polymerase chain reaction(RT-PCR).Results Under microscope,in the hyperoxia-exposed group,inflammatory cell influx was detected in the lungs on the 3rd day and there was marked neutrophlic infiltrate on the 7th day.Alveolar enlargement and fibrosis were evident on the 10th day.At the same time,the histopathological changes were improved greatly in the lungs of hyperoxia-exposed rhEPO treated pups compared with the hyperoxia-exposed pups.MCP-1 and CINC-1 mRNA expression increased in hyperoxia-exposed pups,compared with room air-exposed controls especially on the 7th day [(0.94 ± 0.45) vs (0.21 ± 0.03),P < 0.001 ; (1.26 ± 0.29) vs (0.26 ± 0.06),P < 0.001].MCP-1 and CINC-1 mRNA expression were greatly depressed in the hyperoxia-exposed rhEPO treated pups compared with the hyperoxia-exposed pups especially on the 7th day.[(0.65 ± 0.07) vs (0.94 ± 0.45),P<0.05;(0.83±0.07) vs (1.26±0.29),P<0.05].Conclusions The therapy of rhEPO (2 400 IU/kg) therapy can reduce lung

  9. Preclinical screening for retinopathy of prematurity risk using IGF1 levels at 3 weeks post-partum.

    Directory of Open Access Journals (Sweden)

    Alejandro Pérez-Muñuzuri

    Full Text Available Following current recommendations for preventing retinopathy of prematurity (ROP involves screening a large number of patients. We performed a prospective study to establish a useful screening system for ROP prediction and we have determined that measuring serum levels of IGF1 at week three and the presence of sepsis have a high predictive value for the subsequent development of ROP. A total of 145 premature newborn, with birthweight <1500 g and/or <32 weeks gestational age, were enrolled. 26.9% of them showed some form of retinopathy. A significant association was found between the development of retinopathy and each of the following variables: early gestational age, low birthweight, requiring mechanical ventilation, oxygen treatment, intracranial haemorrhage, sepsis during the first three weeks, bronchopulmonary dysplasia, the need for erythrocyte transfusion, erythropoietin treatment, and low levels of serum IGF1 in the third week. A multiple logistic regression analysis was used to obtain curves for the probability of developing ROP, based on the main factors linked with ROP, namely serum levels of IGF1 and presence of sepsis. Such preclinical screening has the ability to identify patients with high-risk of developing retinopathy and should lead to better prediction for ROP, while at the same time optimising the use of clinical resources, both human and material.

  10. Role of Insulinlike Growth Factor 1 in Fetal Development and in the Early Postnatal Life of Premature Infants.

    Science.gov (United States)

    Hellström, Ann; Ley, David; Hansen-Pupp, Ingrid; Hallberg, Boubou; Ramenghi, Luca A; Löfqvist, Chatarina; Smith, Lois E H; Hård, Anna-Lena

    2016-09-01

    The neonatal period of very preterm infants is often characterized by a difficult adjustment to extrauterine life, with an inadequate nutrient supply and insufficient levels of growth factors, resulting in poor growth and a high morbidity rate. Long-term multisystem complications include cognitive, behavioral, and motor dysfunction as a result of brain damage as well as visual and hearing deficits and metabolic disorders that persist into adulthood. Insulinlike growth factor 1 (IGF-1) is a major regulator of fetal growth and development of most organs especially the central nervous system including the retina. Glucose metabolism in the developing brain is controlled by IGF-1 which also stimulates differentiation and prevents apoptosis. Serum concentrations of IGF-1 decrease to very low levels after very preterm birth and remain low for most of the perinatal development. Strong correlations have been found between low neonatal serum concentrations of IGF-1 and poor brain and retinal growth as well as poor general growth with multiorgan morbidities, such as intraventricular hemorrhage, retinopathy of prematurity, bronchopulmonary dysplasia, and necrotizing enterocolitis. Experimental and clinical studies indicate that early supplementation with IGF-1 can improve growth in catabolic states and reduce brain injury after hypoxic/ischemic events. A multicenter phase II study is currently underway to determine whether intravenous replacement of human recombinant IGF-1 up to normal intrauterine serum concentrations can improve growth and development and reduce prematurity-associated morbidities.

  11. Premature infant

    Science.gov (United States)

    There are many support groups for parents of premature babies. Ask the social worker in the neonatal intensive care unit. ... Prematurity used to be a major cause of infant deaths. Improved ... Prematurity can have long-term effects. Many premature infants ...

  12. Surfactant Protein-D-Encoding Gene Variant Polymorphisms Are Linked to Respiratory Outcome in Premature Infants

    DEFF Research Database (Denmark)

    Sorensen, Grith Lykke; Dahl, Marianne; Tan, Qihua

    2014-01-01

    OBJECTIVE: Associations between the genetic variation within or downstream of the surfactant protein-D-encoding gene (SFTPD), which encodes the collectin surfactant protein-D (SP-D) and may lead to respiratory distress syndrome or bronchopulmonary dysplasia, recently were reported. Our aim was to...

  13. Short- and Long-term Pulmonary Outcome of Palivizumab in Children Born Extremely Prematurely.

    Science.gov (United States)

    Prais, Dario; Kaplan, Eytan; Klinger, Gil; Mussaffi, Huda; Mei-Zahav, Meir; Bar-Yishay, Ephraim; Stafler, Patrick; Steuer, Guy; Sirota, Lea; Blau, Hannah

    2016-03-01

    Palivizumab reduces the severity of respiratory syncytial virus infection in premature infants, but whether there is a protective effect beyond the preschool age is unknown. This study sought to assess the short- and long-term effects of palivizumab immunization on respiratory morbidity and pulmonary function at school age in children born extremely prematurely. Infants born before 29 weeks' gestation in 2000 to 2003 were assessed at school age by parental questionnaire, hospital chart review, and lung function tests. Children born immediately before the introduction of routine palivizumab prophylaxis were compared with age-matched children who received palivizumab prophylaxis during the first respiratory syncytial virus season. Sixty-three children with a mean age 8.9 years were included: 30 had received palivizumab and 33 had not (control subjects). The groups were similar in terms of gestational age, birth weight, need for mechanical ventilation, and oxygen supplementation. Fifty-three percent of the palivizumab group, compared with 39% of the control group, had bronchopulmonary dysplasia (P = .14). Wheezing occurred in the first 2 years of life in 27% of the palivizumab group and in 70% of control subjects (P = .008); respective hospitalization rates were 33% and 70% (P = .001). At school age, rates of hyperresponsiveness (provocative concentration leading to a 20% fall in FEV1 < 1 mg/mL) were 33% and 48%, respectively (P = .38). Spirometry, lung volumes, diffusion, and exhaled nitric oxide were within normal limits, with no significant differences between groups. Palivizumab prophylaxis was associated with reduced wheezing episodes and hospitalizations during the first 2 years of life in children born extremely prematurely. However, it did not affect pulmonary outcome at school age. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

  14. Assessment of pulmonary function in a follow-up of premature infants: our experience

    Directory of Open Access Journals (Sweden)

    F. Ciuffini

    2014-06-01

    Full Text Available Respiratory diseases are a major cause of morbidity in neonates, especially preterm infants; a long term complication of prematurity such as bronchopulmonary dysplasia (BPD is particularly relevant today. The exact role of the Pulmonary Function Test (PFT in this area is not yet well defined; the PFT in newborns and infants - in contrast to what happens in uncooperative children and adults - are routinely used only in a few centers. The assessment of pulmonary function in newborns and infants, however, is nowadays possible with the same reliability that in cooperative patients with the possibility to extend the assessment of polmonary function from bench to bed. The assessment of pulmonary function must be carried out with non invasive and safe methods, at the bedside, with the possibility of continuous monitoring and providing adequate calculation and management of data. The ability to assess lung function helps to define the mechanisms of respiratory failure, improving the treatment and its effects and is therefore a useful tool in the follow-up of newborn and infant with pulmonary disease.

  15. Assessment of pulmonary function in a follow-up of premature infants: our experience

    Directory of Open Access Journals (Sweden)

    F. Ciuffini

    2013-10-01

    Full Text Available Respiratory diseases are a major cause of morbidity in neonates, especially preterm infants; a long term complication of prematurity such as bronchopulmonary dysplasia (BPD is particularly relevant today. The exact role of the Pulmonary Function Test (PFT in this area is not yet well defined; the PFT in newborns and infants - in contrast to what happens in uncooperative children and adults - are routinely used only in a few centers. The assessment of pulmonary function in newborns and infants, however, is nowadays possible with the same reliability that in cooperative patients with the possibility to extend the assessment of polmonary function from bench to bed. The assessment of pulmonary function must be carried out with non invasive and safe methods, at the bedside, with the possibility of continuous monitoring and providing adequate calculation and management of data. The ability to assess lung function helps to define the mechanisms of respiratory failure, improving the treatment and its effects and is therefore a useful tool in the follow-up of newborn and infant with pulmonary disease.

  16. Premature ejaculation

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/001524.htm Premature ejaculation To use the sharing features on this page, please enable JavaScript. Premature ejaculation is when a man has an orgasm ...

  17. [Forensic importance of premature craniosynostosis].

    Science.gov (United States)

    Fehlow, P

    1991-01-01

    In agreement with Canabis craniosynostosis as a little known organic partial factor of sociopathy is demonstrated. A psychic syndrome of the frontal lobe with increased susceptibility in environmental damages is assumed to be basic disorder. In the criminals of the material sexual offenders were preponderating. Associated craniofacial dysplasias are a risk for psychic maldevelopment. The importance of premature craniosynostocis as a biological risk factor, incidence, diagnostic, indication for an operation, also in the meaning of a neurosurgical "Konflikttherapie" (cosmetical indication) are discussed.

  18. Premature infants' health at multiple induced pregnancy.

    Directory of Open Access Journals (Sweden)

    Chernenkov Yu.V.

    2015-09-01

    Full Text Available Objective: to define the risk factors adversely influencing prenatal development at premature birth at use of methods of assisted reproductive technology (ART; to estimate premature' infants health from multiple induced pregnancy according to Perinatal Center of Saratov for last 3 years. Material and Methods. Under supervision there were 139 pregnant women with application ART. 202 children (51 twins were born and 5 triplet babies, from them 83 premature infants born from multiple induced pregnancy have been analyzed. Results. The newborns examined by method ART, were distributed as follows: 22-28 weeks — 19 children; 29-32 weeks — 23; 33-36 weeks — 41. Asphyxia at birth was marked at all premature infants. Respiratory insufficiency at birth is revealed in 87,3% of cases. The most frequent pathologies in premature infants are revealed: neurologic infringements and bronchopulmonary pathology occured at all children, developmental anomaly — 33, 8%, retinopathies in premature infants — 26,5%. The mortality causes include: extreme immaturity, cerebral leukomalacia, IVN 3 degrees. Conclusion. The risk factors, premature birth at application of methods ART are revealed: aged primiparas, pharmacological influence, absence of physiological conditions of prenatal development; multifetation. The high percent of birth of children with ELBW and ULBW is revealed. RDCN with further BPD development, retinopathies in premature infants and CNS defeat is more often occured.

  19. Analysis of two cases with bronchopulmonary neurofibromatosis

    Directory of Open Access Journals (Sweden)

    Yuan Ting

    2012-07-01

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

  20. 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 and extrapol...... and extrapolation of data obtained from more common types of neuroendocrine tumours. This review reflects our view of the current state of the art of diagnosis and treatment of patients with BP-NET....

  1. 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.

  2. Oxygen resuscitation and oxidative-stress biomarkers in premature infants

    Directory of Open Access Journals (Sweden)

    Kumar VH

    2014-05-01

    Full Text Available Vasanth HS Kumar,1 Vivien Carrion,1 Karen A Wynn,1 Lori Nielsen,1 Anne Marie Reynolds,1 Rita M Ryan2 1Department of Pediatrics, The Women and Children's Hospital of Buffalo, Buffalo, NY, 2Department of Pediatrics, Medical University of South Carolina, Charleston, SC, USA Background: Resuscitation of premature infants with 100% O2 may initiate significant oxidant stress during development, predisposing them to bronchopulmonary dysplasia. In the study reported here, we examined the effects of three different oxygen concentrations at resuscitation on oxygen saturations (SpO2 and oxidant stress in premature infants. Study design: Infants <32 weeks gestational age were randomized to 21%, 40%, or 100% O2 and resuscitated as per 2005 neonatal resuscitation guidelines. Oxygen groups and SpO2 were unmasked at 10 minutes of age and FiO2 adjusted to maintain an SpO2 of 85%–95% for the next 20 minutes. Blood was collected at 24 hours, 1 week, and 4 weeks for measurement of the oxidative-stress markers, such as a reduced glutathione (GSH to oxidized glutathione (GSSG ratio (GSH/GSSG, nitrotyrosine levels, and 8-hydroxydeoxyguanosine (8-OHdG levels. The study was stopped at 30% enrollment following publication of the 2010 neonatal resuscitation guidelines. Results: We enrolled 18 patients during the study period. SpO2 increased over time (P<0.0001; however, this increase was not different among the three oxygen groups in the first 10 minutes after birth. FiO2 was significantly higher in the 100% O2 group, despite weaning (P<0.02 to maintain target saturations at 30 minutes of age. The GSH/GSSG ratio was significantly lower in the 100% O2 group at 24 hours than in the other groups (P<0.01. Plasma nitrotyrosine was significantly higher in the 40% and 100% O2 groups over time (P<0.01. Levels of 8-OHdG were significantly higher at 4 weeks compared with at 24 hours, independent of the oxygen group (P<0.0001. Conclusion: In this study, we defined the natural

  3. Oculodentodigital dysplasia

    Directory of Open Access Journals (Sweden)

    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.

  4. Correlation of serum KL-6 and CC16 levels with neurodevelopmental outcome in premature infants at 12 months corrected age

    Science.gov (United States)

    Zhang, Zhiqun; Lu, Hui; Zhu, Yunxia; Xiang, Junhua; Huang, Xianmei

    2015-01-01

    The aim of this study was to evaluate KL-6 and CC16 levels and their correlation with neurodevelopmental outcome among very low birth weight pre-term infants at 12 months corrected age. This prospective cohort study was performed from 2011 to 2013 by enrolling pre-term neonates of gestational age ≤ 32 weeks and birth weight ≤ 1500 g. Serum KL-6 and CC16 levels were determined 7 days after birth and their correlation with neurodevelopment was evaluated using Gesell Mental Developmental Scales. Of the 86 eligible pre-term infants, 63 completed follow-up, of which 15 had bronchopulmonary dysplasia. At 12 months corrected age, 49 infants had favorable outcomes and 14 infants had poor neurodevelopmental outcome. KL-6 levels were higher and CC16 levels were lower in infants with poor neurodevelopmental outcome compared with those infants who had favourable neurodevelopmental outcome. Serum KL-6 levels less than 90.0 ng/ml and CC16 levels greater than 320.0 pg/ml at 7 days of life were found to be predictive of a favourable outcome at 12 months corrected age. These biological markers could predict neurodevelopmental outcome at 12 months corrected age in very low birth weight premature infants, and help the clinician plan early therapeutic interventions to minimize or avoid poor neurodevelopmental outcome. PMID:25631862

  5. The role of surfactant and non-invasive mechanical ventilation in early management of respiratory distress syndrome in premature infants

    Institute of Scientific and Technical Information of China (English)

    Narayan Prabhu Iyer; Maroun Jean Mhanna

    2014-01-01

    Background: Surfactant replacement therapy has been used for few decades for the treatment of respiratory distress syndrome (RDS) and has significantly improved morbidity and mortality in premature infants. Non-invasive respiratory support has recently emerged as a strategy in the early management of RDS. In this review, we discuss the different strategies of early management of RDS. Data sources: A literature search of PubMed database was conducted to review the subject. The quality of evidence of key clinical studies was graded according to a modified grading system of the international GRADE group. Results: Continuous positive airway pressure (CPAP) with selective surfactant is a safe alternative to routine intubation, surfactant and mechanical ventilation in preterm infants with spontaneous breathing, and such an approach has been associated with decreased risk of death and bronchopulmonary dysplasia. There is a risk of pneumothorax when using a high pressure of CPAP (≥8 cm of H2O), a high partial pressure of carbon dioxide (PCO2 >75 mm of Hg), and a high fraction of inspired oxygen (FiO2 >0.6) as a threshold for intubation while on CPAP. Conclusion: Not all preterm infants need surfactant treatment, and non-invasive respiratory support is a safe and effective approach.

  6. CT in childhood allergic bronchopulmonary aspergillosis

    Energy Technology Data Exchange (ETDEWEB)

    Shah, A.; Bhagat, R.; Panchal, N. (Delhi Univ. (India). Vallabhabhai Patel Chest Inst.); Pant, C.S. (Institute of Nuclear Medicine and Allied Sciences, Delhi (India). Imaging Div.)

    1992-06-01

    CT of the thorax done during acute severe asthma in two paediatric patients demonstrated central bronchiectasis, a sine qua non for the diagnosis of allergic bronchopulmonary aspergillosis. Bronchography, regarded as the gold standard, was done subsequently on recovery. A comparative segmental analysis revealed that CT was able to identify immediately 24 of 27 segments which showed central bronchiectasis on bronchography. Early diagnosis with the aid of CT enabled immediate intervention which may have helped to prevent further lung damage in the paediatric patients. (orig.).

  7. Different ventilation modes combined with ambroxol in the treatment of respiratory distress syndrome in premature infants

    Science.gov (United States)

    Zhou, Bin; Zhai, Jing-Fang; Wu, Jie-Bin; Jin, Bao; Zhang, Yan-Yan

    2017-01-01

    The aim of the present study was to compare the effectiveness of different modes of mechanical ventilation in combination with secretolytic therapy with ambroxol in premature infants with respiratory distress syndrome. Seventy-three premature infants with hyaline membrane disease (HMD) (stage III–IV), also known as respiratory distress syndrome, who were supported by mechanical ventilation in the neonatal intensive care unit (NICU) of Xuzhou Central Hospital, were involved in the present study, between January 2013 and February 2015. Forty cases were randomly selected and treated with high frequency oscillatory ventilation (HFOV), forming the HFOV group, whereas 33 cases were selected and treated with conventional mechanical ventilation (CMV), forming the CMV group. Patients in the two groups were administered ambroxol intravenously at a dosage rate of 30 mg/kg body weight at the beginning of the study. The present study involved monitoring the blood gas index as well as changes in the respiratory function index in the two groups. Additionally, the incidence of complications in the premature infants in the two groups was observed prior to and following the ventilation. Pulmonary arterial oxygen tension (PaO2), the PaO2/fraction of inspired oxygen (FiO2) ratio, the oxygenation index [OI = 100 × mean airway pressure (MAP) × FiO2/PaO2], as well as the arterial/alveolar oxygen partial pressure ratio (a/APO2) = PaO2/(713 × FiO2 partial pressure of carbon dioxide (PaCO2)/0.8) of the patients in the HFOV group after 1, 12 and 24 h of treatment were significantly improved as compared to the patients of the CMV group. However, there was no significant difference between patients in the two groups with regard to the number of mortalities, complications such as pneumothorax, bronchopulmonary dysplasia (BPD), retinopathy of prematurity (ROP), intraventricular hemorrhage (IVH), periventricular leukomalacia (PVL), and the time of ventilation. In conclusion, combining HFOV

  8. 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

  9. Your Premature Baby

    Science.gov (United States)

    ... birth defects, premature birth and infant mortality. Solving premature birth Featured articles Accomplishments and lessons learned since ... Complications & Loss > Preterm labor & premature birth > Premature babies Premature babies E-mail to a friend Please fill ...

  10. Genetics Home Reference: Greenberg dysplasia

    Science.gov (United States)

    ... Email Facebook Twitter Home Health Conditions Greenberg dysplasia Greenberg dysplasia Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Greenberg dysplasia is a severe condition characterized by specific ...

  11. Immunopathogenesis of allergic bronchopulmonary aspergillosis and airway remodeling

    NARCIS (Netherlands)

    Kauffman, HF

    Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity lung disease clinically characterized by manifestation of wheezing, pulmonary infiltrates and bronchiectasis and fibrosis which afflicts asthmatic and cystic fibrosis (CF) patients. The pathophysiologic mechanisms are mediated by a

  12. APPLYING OF GAS ANALYSIS IN DIAGNOSIS OF BRONCHOPULMONARY DISEASES

    Directory of Open Access Journals (Sweden)

    Ye. B. Bukreyeva

    2014-01-01

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

  13. Cervical dysplasia - series (image)

    Science.gov (United States)

    ... to detect cervical cancer. Limited or early cervical cancer (carcinoma in situ, or cervical intraepithelial neoplasia, or dysplasia) requires treatment with ablation therapy, usually in the form of ...

  14. Metatropic dysplasia lethal variants

    Energy Technology Data Exchange (ETDEWEB)

    Hall, Christine M. [Department of Radiology, Great Ormond Street Hospital for Children NHS Trust, WCIN 3JH, London (United Kingdom); Elcioglu, Nursel H. [Department of Pediatric Genetics, Marmara University Hospital, Istanbul (Turkey)

    2004-01-01

    Background: The metatropic dysplasia group includes fibrochondrogenesis, Schneckenbecken dysplasia and metatropic dysplasia (various forms). The overlapping features of this group with other dysplasias may cause diagnostic confusion, particularly in perinatal lethal cases. Objective: To attempt to classify the radiological findings of the presented eight sporadic cases based on a broad review of the perinatally lethal metatropic group of conditions and to discuss some overlapping features in the light of current knowledge. Results: The first four cases are of recognised conditions, namely lethal metatropic dysplasia (Type 2) or hyperchondrogenesis, lethal hyperplastic metatropic dysplasia (Type 1) and fibrochondrogenesis. The remaining four cases cannot be categorised accurately and are different from each other but with some features of the metatropic group of dysplasias. Conclusions: The dysplasias within the metatropic dysplasia group are phenotypically distinct from many forms of chondrodysplasia but the pathogenesis still remains poorly understood from the morphological and molecular perspectives. Chondro-osseous morphology might be helpful in all lethal cases especially in our last four cases. (orig.)

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

    Directory of Open Access Journals (Sweden)

    Dixit R

    1998-01-01

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

  16. Osteopenia - premature infants

    Science.gov (United States)

    Neonatal rickets; Brittle bones - premature infants; Weak bones - premature infants; Osteopenia of prematurity ... the baby. This helps the baby grow. A premature infant may not receive the proper amount of ...

  17. 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.

  18. Immunopathology and immunogenetics of allergic bronchopulmonary aspergillosis.

    Science.gov (United States)

    Knutsen, Alan P

    2011-01-01

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

  19. Immunopathology and Immunogenetics of Allergic Bronchopulmonary Aspergillosis

    Directory of Open Access Journals (Sweden)

    Alan P. Knutsen

    2011-01-01

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

  20. Premature delivery

    Directory of Open Access Journals (Sweden)

    Bernardita Donoso Bernales

    2012-09-01

    Full Text Available Preterm delivery is the single most important cause of perinatal morbidity and mortality. In Chile, preterm births have increased in the past decade, although neonatal morbidity and mortality attributable to it shows a downward trend, thanks to improvements in neonatal care of premature babies, rather than the success of obstetric preventive and therapeutic strategies. This article describes clinical entities, disease processes and conditions that constitute predisposing factors of preterm birth, as well as an outline for the prevention and clinical management of women at risk of preterm birth.

  1. Spondylo-costal dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Kozlowski, K.

    1984-02-01

    Fourteen patients with spondylo-costal dysplasia were analysed. 3 of them presented without obvious associated anomalies ''pure'' spondylo-costal dysplasias; 2 had several components consistent with Vater (Vacterl)-Association; 2 showed malformations which are often encountered in Vater (Vacterl)-Association; 4 presented with minor malformations; 3 had major associated malformations rarely seen in Vater (Vacterl)-Association. Thoracic spine and costal malsegmentation can be sporadically observed in other ''errors in septation complex'' (axial mesodermal dysplasia) including severe myelomeningocoele and diastematomyelia.

  2. Cervical deciduosis imitating dysplasia.

    Science.gov (United States)

    van Diepen, Diederik Anthony; Hellebrekers, Bart; van Haaften, Anne-Marie; Natté, Remco

    2015-09-22

    Ectopic cervical deciduosis is generally an accidental finding during pregnancy, and usually presents without any symptoms or need for therapeutic intervention. However, it can sometimes imitate dysplasia or carcinoma. We report a case of a 34-year-old G2P0, with a history of cervical dysplasia, presenting at 11 weeks of gestation, with vaginal blood loss. During examination, lesions mimicking dysplasia were found on the cervix. Histological examination reported cervical deciduosis. Deciduosis is a benign change during pregnancy and will resolve spontaneously. With the increasing use of cytology and colposcopy, the reported incidence is growing. When it is hard to differentiate between dysplasia and deciduosis, histological confirmation should be considered.

  3. Focal cortical dysplasia - review.

    Science.gov (United States)

    Kabat, Joanna; Król, Przemysław

    2012-04-01

    Focal cortical dysplasia is a malformation of cortical development, which is the most common cause of medically refractory epilepsy in the pediatric population and the second/third most common etiology of medically intractable seizures in adults.Both genetic and acquired factors are involved in the pathogenesis of cortical dysplasia. Numerous classifications of the complex structural abnormalities of focal cortical dysplasia have been proposed - from Taylor et al. in 1971 to the last modification of Palmini classification made by Blumcke in 2011. In general, three types of cortical dysplasia are recognized.Type I focal cortical dysplasia with mild symptomatic expression and late onset, is more often seen in adults, with changes present in the temporal lobe.Clinical symptoms are more severe in type II of cortical dysplasia usually seen in children. In this type, more extensive changes occur outside the temporal lobe with predilection for the frontal lobes.New type III is one of the above dysplasias with associated another principal lesion as hippocampal sclerosis, tumor, vascular malformation or acquired pathology during early life.Brain MRI imaging shows abnormalities in the majority of type II dysplasias and in only some of type I cortical dysplasias.THE MOST COMMON FINDINGS ON MRI IMAGING INCLUDE: focal cortical thickening or thinning, areas of focal brain atrophy, blurring of the gray-white junction, increased signal on T2- and FLAIR-weighted images in the gray and subcortical white matter often tapering toward the ventricle. On the basis of the MRI findings, it is possible to differentiate between type I and type II cortical dysplasia. A complete resection of the epileptogenic zone is required for seizure-free life. MRI imaging is very helpful to identify those patients who are likely to benefit from surgical treatment in a group of patients with drug-resistant epilepsy.However, in type I cortical dysplasia, MR imaging is often normal, and also in both types

  4. Growth in infants with bronchopulmonary dysplasia, endocrine and pulmonary aspects : clinical and follow-up studies

    NARCIS (Netherlands)

    W.A. Huijsman (Marianne)

    2003-01-01

    markdownabstract__Abstract__ In the last decades important advances in neonatal intensive care have been made leading to an increased survival of especially very preterm infants. Major changes in survival rate have been attributed to the use of antenatal steroids and surfactant in the prevention an

  5. Craniofacial fibrous dysplasia

    Directory of Open Access Journals (Sweden)

    Aakarsh Jhamb

    2012-01-01

    Full Text Available Fibrous dysplasia can present clinically in varied forms which may appear as collision of different pathologic processes. We report a rare case of craniofacial fibrous dysplasia with coexisting epithelial lined cyst and superimposed osteomyelitis with sequestrum formation. Its clinical features and management with possible hypotheses are described along with the post operative course. Pertinent literature has been reviewed with emphasis on pathogenesis of this unique occurrence.

  6. 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.)

  7. Mandibulo-acral dysplasia

    Energy Technology Data Exchange (ETDEWEB)

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

    2000-11-01

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

  8. 咖啡因在早产新生儿疾病中的临床应用进展%Recent advances for clinical applications of caffeine in premature infants

    Institute of Scientific and Technical Information of China (English)

    耿晓萌; 史宝海

    2016-01-01

    Caffeine has been applied in clinic more than 30years,nowadays,which is one of the safe and effective drugs for the treatment of neonatal disease.It has been proved that caffeine can significantly re-duce the major acute neonatal morbidities include apnea of prematurity,bronchopulmonary dysplasia,severe retinopathy,patent ductus arteriosus with surgical ligation.Moreover,recent research also shown that caffeine can improve babies neurological outcome and lung function.This review summarized the latest progression in the pharmacological characteristics,clinical applications and untoward effect of caffeine.%咖啡因已应用于临床30余年,目前已成为治疗新生儿疾病安全有效的药物之一。已证实,咖啡因可显著降低早产儿呼吸暂停、严重的视网膜病变、支气管肺发育不良及需要手术关闭动脉导管等疾病的发病率。此外,最新研究表明,咖啡因还可改善神经系统预后及肺功能等作用。本文简要介绍咖啡因的药理特点、临床应用及可能的不良反应等方面的新进展。

  9. A hyperoxic lung injury model in premature rabbits: the influence of different gestational ages and oxygen concentrations.

    Directory of Open Access Journals (Sweden)

    Roberta Munhoz Manzano

    Full Text Available BACKGROUND: Many animal models have been developed to study bronchopulmonary dysplasia (BPD. The preterm rabbit is a low-cost, easy-to-handle model, but it has a high mortality rate in response to the high oxygen concentrations used to induce lung injury. The aim of this study was to compare the mortality rates of two models of hyperoxia-induced lung injury in preterm rabbits. METHODS: Pregnant New Zealand white rabbits were subjected to caesarean section on gestational day 28 or 29 (full term  = 31 days. The premature rabbits in the 28-day gestation group were exposed to room air or FiO₂ ≥95%, and the rabbits in the 29-day gestation group were exposed to room air or FiO₂  = 80% for 11 days. The mean linear intercept (Lm, internal surface area (ISA, number of alveoli, septal thickness and proportion of elastic and collagen fibers were quantified. RESULTS: The survival rates in the 29-day groups were improved compared with the 28-day groups. Hyperoxia impaired the normal development of the lung, as demonstrated by an increase in the Lm, the septal thickness and the proportion of elastic fibers. Hyperoxia also decreased the ISA, the number of alveoli and the proportion of collagen fibers in the 28-day oxygen-exposed group compared with the control 28-day group. A reduced number of alveoli was found in the 29-day oxygen exposed animals compared with the control 29-day group. CONCLUSIONS: The 29-day preterm rabbits had a reduced mortality rate compared with the 28-day preterm rabbits and maintained a reduction in the alveoli number, which is comparable to BPD in humans.

  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. Premature Ventricular Contractions (PVCs)

    Science.gov (United States)

    Diseases and Conditions Premature ventricular contractions (PVCs) By Mayo Clinic Staff Premature ventricular contractions (PVCs) are extra, abnormal heartbeats that begin in one of your heart's two ...

  12. Premature ejaculation

    Directory of Open Access Journals (Sweden)

    Chris G McMahon

    2007-01-01

    Full Text Available Premature ejaculation (PE is a common male sexual disorder. Recent normative data suggests that men with an intravaginal ejaculatory latency time (IELT of less than 1 minute have "definite" PE, while men with IELTs between 1 and 1.5 minutes have "probable" PE. Although there is insufficient empirical evidence to identify the etiology of PE, there is limited correlational evidence to suggest that men with PE have high levels of sexual anxiety and inherited altered sensitivity of central 5-HT (5-hydroxytryptamine, serotonin receptors. Pharmacological modulation of the ejaculatory threshold using off-label daily or on-demand selective serotonin re-uptake inhibitors is well tolerated and offers patients a high likelihood of achieving improved ejaculatory control within a few days of initiating treatment, consequential improvements in sexual desire and other sexual domains. Investigational drugs such as the ejaculo-selective serotonin transport inhibitor, dapoxetine represent a major development in sexual medicine. These drugs offer patients the convenience of on-demand dosing, significant improvements in IELT, ejaculatory control and sexual satisfaction with minimal adverse effects.

  13. Premature ejaculation.

    Science.gov (United States)

    McMahon, Chris G

    2007-04-01

    Premature ejaculation (PE) is a common male sexual disorder. Recent normative data suggests that men with an intravaginal ejaculatory latency time (IELT) of less than 1 minute have "definite" PE, while men with IELTs between 1 and 1.5 minutes have "probable" PE. Although there is insufficient empirical evidence to identify the etiology of PE, there is limited correlational evidence to suggest that men with PE have high levels of sexual anxiety and inherited altered sensitivity of central 5-HT (5-hydroxytryptamine, serotonin) receptors. Pharmacological modulation of the ejaculatory threshold using off-label daily or on-demand selective serotonin re-uptake inhibitors is well tolerated and offers patients a high likelihood of achieving improved ejaculatory control within a few days of initiating treatment, consequential improvements in sexual desire and other sexual domains. Investigational drugs such as the ejaculo-selective serotonin transport inhibitor, dapoxetine represent a major development in sexual medicine. These drugs offer patients the convenience of on-demand dosing, significant improvements in IELT, ejaculatory control and sexual satisfaction with minimal adverse effects.

  14. 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...

  15. Algorithm of rehabilitation examination of children with bronchopulmonary diseases

    Directory of Open Access Journals (Sweden)

    Nataliya Ivasyk

    2016-08-01

    Full Text Available Purpose: to develop the algorithm of rehabilitation examination for children with bronchopulmonary diseases. Material & Methods: analysis, generalization, systematization and comparison of data of scientific and methodical literature on problems of physical rehabilitation at bronchopulmonary diseases. Results: the offered algorithm of rehabilitation examination of children with bronchopulmonary diseases provides the consecutive application of such methods as: poll, physical examination, functional examination and method of indexes, which analysis allows defining the rehabilitation diagnosis, which is the basis of technology of the rehabilitation process. Conclusions: rehabilitation examination is the compound of physical rehabilitation which is carried out for the purpose of definition of the rehabilitation diagnosis and is based on subjective, objective assessments and data of supervision. The consecutive carrying out of the complex rehabilitation examination on the offered algorithm and the detailed analysis of its results will promote the determination of rehabilitation potential, the reasons of violations from various systems of organism and individualization of the rehabilitation process of children with bronchopulmonary diseases.

  16. 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.

  17. CONSERVATIVE TREATMENT OF HIP DYSPLASIA

    OpenAIRE

    Михаил Михайлович Камоско; Махмуд Станиславович Познович

    2014-01-01

    Hip dysplasia in children is the subject of careful study by specialists both in our country and abroad. There are three basic variants of hip dysplasia: congenital acetabular dysplasia, congenital subluxation of the hip and congenital dislocation of the hip. However, treatment of congenital dislocation of the hip, which is the most severe form of hip dysplasia in children, currently remains one of the topical problems of children’s orthopedics, despite the significant achievements of modern ...

  18. The clinical case of sildenafil administration in a very premature infant with pulmonary hypertension

    Directory of Open Access Journals (Sweden)

    Galina A. Alyamovskaya

    2015-02-01

    Full Text Available We report the use of oral sildenafil in a 7-month-old preterm newborn with severe bronchopulmonary dysplasia and pulmonary arterial hypertension refractory to captopril and inhaled budesonide, and need of consistent oxygenation. Sildenafil was prepared as a powder for oral administration. Oral sildenafil treatment was continued for 11 months. Oxygen supplement was suspended after 4 months and captopril administration was finished after 7 months of sildenafil treatment. There were no adverse effects during the treatment period. The respiratory failure decreased significantly and pulmonary arterial pressure became normal after 7 months of sildenafil treatment.

  19. Spectrum of bronchopulmonary anomalies associated with tracheoesophageal malformations

    Energy Technology Data Exchange (ETDEWEB)

    Benson, J.E.; Fletcher, B.D.; Olsen, M.

    1985-09-01

    The combination of tracheoesophageal and pulmonary malformations is unusual and reportedly carries a high mortality. We have observed six patients with esophageal atresia and tracheoesophageal fistula and one with a bronchoesophageal fistula who had associated bronchopulmonary anomalies ranging from lobar hypoplasia and agenesis to unilateral pulmonary hypoplasia or agenesis. All of the pulmonary malformations were right-sided. Vertebral or rib anomalies were present in five patients, congenital heart disease in two, imperforate anus in one and one patient had radial aplasia and Pierre Robin syndrome. The bronchopulmonary anomalies complicated the surgical care of tracheoesophageal malformations and required radiologic differentiation from aspiration pneumonia and atelectasis. Six of the seven patients survived. Mortality and morbidity were related to complications and associated cardiac anomalies as well as severity of the lung anomaly.

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

    OpenAIRE

    Collins J; de Vos G; Hudes G; Rosenstreich D

    2012-01-01

    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 the...

  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. 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...

  3. Prematurity and prescription asthma medication from childhood to young adulthood

    DEFF Research Database (Denmark)

    Damgaard, Anne Louise; Hansen, Bo Moelholm; Mathiasen, Rene

    2015-01-01

    INTRODUCTION: Preterm birth is associated with increased risk of asthma-like symptoms and purchase of prescription asthma medication in childhood. We investigated whether this association persists into adulthood and whether it is affected by accounting for neonatal respiratory morbidity (acute...... respiratory disease and bronchopulmonary dysplasia). METHODS: A national cohort of all infants born in Denmark in the period 1980-2009 was included in this register study. Data on purchase of asthma medication (combination of inhaled β-2 agonists and other drugs for obstructive airway disease) in 2010......-2011 were obtained from the Danish National Prescription Registry. Associations between gestational age (GA), neonatal respiratory morbidity and a cross-sectional evaluation of asthma medication purchase were explored by multivariate logistic regressions. RESULTS: A full dataset was obtained on 1...

  4. Bronchopulmonale dysplasie bij ex-prematuren die volwassen worden

    NARCIS (Netherlands)

    Vrijlandt, E.J.L.E.; Gerritsen, J.; Duiverman, E.J.

    2007-01-01

    Many patients born prematurely who developed bronchopulmonary dysplasia (BPD) as neonates are now approaching adulthood. Adults with BPD are at increased risk for respiratory difficulties including respiratory and lung function disorders. Respiratory symptoms include wheezing, cough and dyspnoea. In

  5. Original Research Effects of maternal hypertension on the neonatal ...

    African Journals Online (AJOL)

    Hypertension in pregnancy (HIP) constitutes a major health burden in the obstetric ... restriction, prematurity, and bronchopulmonary dysplasia. There is a dearth of .... and babies born with congenital malformations and birth asphyxia.

  6. Therapeutic potential of soluble guanylate cyclase modulators in neonatal chronic lung disease

    NARCIS (Netherlands)

    Wagenaar, Gerry T M; Hiemstra, Pieter S; Gosens, Reinoud

    2015-01-01

    Supplemental oxygen after premature birth results in aberrant airway, alveolar and pulmonary vascular development with an increased risk for bronchopulmonary dysplasia (BPD), and development of wheeze and asthma, pulmonary hypertension and COPD in survivors. Although stimulation of the nitric oxide

  7. Therapeutic potential of soluble guanylate cyclase modulators in neonatal chronic lung disease

    NARCIS (Netherlands)

    Wagenaar, Gerry T M; Hiemstra, Pieter S; Gosens, Reinoud

    2015-01-01

    Supplemental oxygen after premature birth results in aberrant airway, alveolar and pulmonary vascular development with an increased risk for bronchopulmonary dysplasia (BPD), and development of wheeze and asthma, pulmonary hypertension and COPD in survivors. Although stimulation of the nitric oxide

  8. Segmental odontomaxillary dysplasia: clinical, radiological and histological aspects of four cases

    DEFF Research Database (Denmark)

    Becktor, K.B.; Reibel, J.; Vedel, B.;

    2002-01-01

    Segmental odontomaxillary dysplasia, hemimaxillofacial dysplasia, histological and radiological findings......Segmental odontomaxillary dysplasia, hemimaxillofacial dysplasia, histological and radiological findings...

  9. Retinopathy of Prematurity (ROP)

    Science.gov (United States)

    ... of Prematurity (ROP) Facts About Retinopathy of Prematurity (ROP) This information was developed by the National Eye ... blind from ROP. Are there different stages of ROP? Yes. ROP is classified in five stages, ranging ...

  10. Lumbar-sacral dysplasias

    Energy Technology Data Exchange (ETDEWEB)

    Schumacher, M.; Thron, A.

    1984-09-01

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

  11. [Craniofacial fibrous dysplasia].

    Science.gov (United States)

    Couturier, A; Aumaître, O; Mom, T; Gilain, L; André, M

    2016-12-01

    Fibrous dysplasia of bone is a benign, uncommon, sporadic, congenital skeletal disorder resulting in deformity. This disease arises from activating somatic mutation in GNAS which encodes the α subunit of the G stimulatory protein associated with proliferation of undifferentiated osteogenic cells resulting in marrow fibrosis, abnormal matrix production, and stimulation of osteoclastic resorption upon overproduction of IL-6 observed in dysplastic cells. Fibrous dysplasia may be monostotic or polyostotic. This mutation affecting many tissues, café au lait skin macules and endocrinopathies (precocious puberty, hyperthyroidism, growth hormone excess, Cushing syndrome) may be associated in McCune-Albright syndrome, but also myxoma in Mazabraud syndrome or phosphate diabetes. Diagnosis of craniofacial fibrous dysplasia should be considered in the presence of headache, neuralgia, sensory disorders (vision, hearing, balance, smelling), functional disorders (nasal obstruction, nasolacrimal duct obstruction, non-matching occlusion), infectious complications (sinusitis, otitis, mastoiditis). Such symptoms should lead to perform craniofacial CT scan completed with MRI. Bone biopsy is not systematic. Surgical treatment is discussed in cases of nervous complication, facial deformity or active lesions. In case of pain resistant to conventional analgesics, intravenous bisphosphonates can be proposed. In non-responder patients, several case reports suggest the efficacy of a monoclonal antibody directed against the IL-6 receptor which requires to be confirmed by randomized studies.

  12. 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

  13. Pharmacotherapy for premature ejaculation

    NARCIS (Netherlands)

    Waldinger, Marcel D

    PURPOSE OF REVIEW: As there are various drugs and different treatment strategies to delay ejaculation, a review of the current drug treatments for premature ejaculation is relevant for daily clinical practice. RECENT FINDINGS: There are four premature ejaculation subtypes: lifelong premature

  14. Pharmacotherapy for premature ejaculation

    NARCIS (Netherlands)

    Waldinger, Marcel D

    2014-01-01

    PURPOSE OF REVIEW: As there are various drugs and different treatment strategies to delay ejaculation, a review of the current drug treatments for premature ejaculation is relevant for daily clinical practice. RECENT FINDINGS: There are four premature ejaculation subtypes: lifelong premature ejacula

  15. A new syndrome of 'spondylo-epi-metaphyseal dysplasia: mixed type''

    Energy Technology Data Exchange (ETDEWEB)

    Sharma, B.G. [Department of Radiology, Al Afia Hospital, Houn, Al-Jufra (Libya)

    2003-02-01

    A new type of rare bone dysplasia is described, which shares some common features with spondylo-meta-epiphyseal dysplasia: short limb-abnormal calcification type and lethal metatropic dysplasia. Besides these features, the present case has some additional unusual features. Facial malformation was very obvious and of a different type. The nose and nares were completely flattened. Hypertrophied acetabular bones, round densities on the ilia, premature ossification of many epiphyses and carpal bones, curvilinear calcifications in some joints, fusion of the ischiopubic rami, calcification of many costal cartilages and thick sclerotic base of the skull were a few of the significant findings. On the basis of the clinical and radiological features, the condition has been named ''spondylo-epi-metaphyseal dysplasia: mixed type''. (orig.)

  16. Health Issues of Premature Babies

    Science.gov (United States)

    ... Text Size Email Print Share Health Issues of Premature Babies Page Content Because premature babies are born before they are physically ready ... associated with prematurity. Because of these health concerns, premature babies are given extra medical attention and assistance ...

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

    Energy Technology Data Exchange (ETDEWEB)

    Sumner, T.E.; Auringer, S.T.; Cox, T.D. [Department of Radiology, Bowman Gray School of Medicine, Wake Forest University, Medical Center Boulevard, Winston-Salem, NC 27157-1088 (United States)

    1997-10-01

    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.

  18. 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.

  19. Allergic bronchopulmonary aspergillosis: a unique presentation in a pediatric patient

    Energy Technology Data Exchange (ETDEWEB)

    Huppmann, Michael V.; Monson, Matthew [Walter Reed Army Medical Center, Department of Radiology, Washington, DC (United States)

    2008-08-15

    Infection by the Aspergillus species of fungus can result in a variety of clinically and radiographically unique pulmonary diseases. The specific disease manifested is most dependent upon the immunocompetency of the infected individual. Allergic bronchopulmonary aspergillosis (ABPA) is most commonly seen in patients with asthma and cystic fibrosis. Since its original description in 1952, much has been published describing the radiographic manifestations of this disease. In this article, we present the unusual case of a 13-year-old whose initial radiographic presentation was that of a dense lobar consolidation. Additionally, we highlight the contributory role of the radiologist in guiding the appropriate clinical work-up and treatment of this disease. (orig.)

  20. Patterson-Lowry rhizomelic dysplasia: a potentially lethal bone dysplasia?

    Energy Technology Data Exchange (ETDEWEB)

    Kamoda, T.; Nakajima, R.; Matsui, A. [Dept. of Paediatrics, Univ. of Tsukuba, Ibaraki (Japan); Nishimura, G. [Dept. of Radiology, Nasu Chuou Hospital, Tochigi (Japan)

    2001-02-01

    We report a Japanese boy, who is considered as having Patterson-Lowry rhizomelic dysplasia, a rare, as yet undefined, skeletal dysplasia. The diagnosis was warranted by the constellation of skeletal abnormalities - mild platyspondyly, hypoplastic ilia, broad proximal femora with prominent lesser trochanters, mild brachymetacarpalia and, most importantly, rhizomelic shortening of the upper limbs with lateral bowing, medial cortical thickening, and medial metaphyseal notching of the humeri. Our patient, unlike previously reported patients, had respiratory distress and died suddenly of unknown cause in late infancy. Our experience may imply the heterogeneity or phenotypic variability of Patterson-Lowry rhizomelic dysplasia. (orig.)

  1. Bronchopulmonary allergic aspergillosis Aspergilosis broncopulmonar alérgica

    Directory of Open Access Journals (Sweden)

    Rodrigo Ramírez

    1990-03-01

    Full Text Available

    A series of well defined entitles associated with colonization or invasion by fungi of the genus Aspergillus are grouped under the term Aspergillosis; there are various modalities of pulmonary involvement, namely, extrinsec asthma, extrinsec allergic alveolitis, allergic bronchopulmonary aspergillosis, aspergilloma and an invasive form in debilitated terminal patients; besldes there exists a form of food poisoning. We report on the case of a 45 year-old asthmatic woman suffering from allerglc bronchopulmonary aspergillosis and sum. maryze the clinical and laboratory features of the different pulmonary forms of aspergillosis.

    El término Aspergllosis reúne una serle de entidades bien definidas, causadas por hongos del género Aspergillus. En este artículo se presenta el caso de una mujer de 45 años con aspergllosis broncopulmonar alérgica, atendida en el Hospital Universitario San Vicente de Paúl, de Medellín y se hace una somera revisión clínico-patológica de las diferentes formas de aspergllosis pulmonar.

  2. 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.

  3. Association of Neurodevelopmental Outcomes and Neonatal Morbidities of Extremely Premature Infants With Differential Exposure to Antenatal Steroids

    Science.gov (United States)

    Chawla, Sanjay; Natarajan, Girija; Shankaran, Seetha; Pappas, Athina; Stoll, Barbara J.; Carlo, Waldemar A.; Saha, Shampa; Das, Abhik; Laptook, Abbot R.; Higgins, Rosemary D.

    2017-01-01

    IMPORTANCE Many premature infants are born without exposure to antenatal steroids (ANS) or with incomplete courses. This study evaluates the dose-dependent effect of ANS on rates of neonatal morbidities and early childhood neurodevelopmental outcomes of extremely premature infants. OBJECTIVE To compare rates of neonatal morbidities and 18- to 22-month neurodevelopmental outcomes of extremely premature infants exposed to no ANS or partial or complete courses of ANS. DESIGN, SETTING, AND PARTICIPANTS In this observational cohort study, participants were extremely premature infants (birth weight range, 401–1000 g; gestational age, 22–27 weeks) who were born at participating centers of the National Institute of Child Health and Human Development Neonatal Research Network between January 2006 and December 2011. Data were analyzed between October 2013 and May 2016. MAIN OUTCOMES AND MEASURES Rates of death or neurodevelopmental impairment at 18 to 22 months’ corrected age. Neurodevelopmental impairment was defined as the presence of any of the following: moderate to severe cerebral palsy, a cognitive score less than 85 on the Bayley Scales of Infant and Toddler Development III, blindness, or deafness. RESULTS There were 848 infants in the no ANS group, 1581 in the partial ANS group, and 3692 in the complete ANS group; the mean (SD) birth weights were 725 (169), 760 (173), and 753 (170) g, respectively, and the mean (SD) gestational ages were 24.5 (1.4), 24.9 (2), and 25.1 (1.1) weeks. Of 6121 eligible infants, 4284 (70.0%) survived to 18- to 22-month follow-up, and data were available for 3892 of 4284 infants (90.8%). Among the no, partial, and complete ANS groups, there were significant differences in the rates of mortality (43.1%, 29.6%, and 25.2%, respectively), severe intracranial hemorrhage among survivors (23.3%, 19.1%, and 11.7%), death or necrotizing enterocolitis (48.1%, 37.1%, and 32.5%), and death or bronchopulmonary dysplasia (74.9%, 68.9%, and 65

  4. 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.

  5. Concomitant achondroplasia and developmental dysplasia of the hip

    Directory of Open Access Journals (Sweden)

    Tennison L. Malcolm, MD

    2015-12-01

    Full Text Available Achondroplasia (ACH is the most common form of hereditary dwarfism and presents with multiple musculoskeletal anomalies but is not normally associated with premature hip arthritis. Developmental dysplasia of the hip (DDH is a spectrum of disease resulting in shallow acetabular depth and a propensity for chronic femoral subluxation or dislocation; it is among the most common causes of premature arthritis. This case report describes the diagnosis of symptomatic DDH in a patient with ACH and highlights difficulties of primary total hip arthroplasty (THA as a treatment option. Intraoperative radiographic imaging is advised to ensure proper prosthesis placement. Femoral osteotomy may aid visualization, reduction, and avoidance of soft tissue injury. Concomitant ACH and DDH is a challenging problem that can be successfully treated with modified THA.

  6. Case report: allergic bronchopulmonary aspergillosis and allergic fungal sinusitis successfully treated with voriconazole.

    Science.gov (United States)

    Erwin, Gary E; Fitzgerald, John E

    2007-12-01

    Allergic bronchopulmonary aspergillosis and allergic fungal sinusitis are closely related disorders that rarely present in the same individual. The mainstay of treatment for allergic bronchopulmonary aspergillosis is systemic corticosteroids. Itraconazole is used as adjunctive therapy in refractory cases. Allergic fungal sinusitis requires initial sinus surgery followed by systemic steroids. Antifungal therapy has not proven to be beneficial in allergic fungal sinusitis. We report a case of concomitant allergic bronchopulmonary aspergillosis and allergic fungal sinusitis that was refractory to standard therapy but had dramatic clinical response following treatment with voriconazole.

  7. Ellis-Van Creveld Dysplasia

    Science.gov (United States)

    ... configuration along chromosome 4 (4p16), are responsible for EVC (3). Physical Characteristics Ellis-Van Creveld Dysplasia is ... 000 live births. More than 200 cases of EVC have now been reported (3). Face and Skull ...

  8. Genetics Home Reference: otospondylomegaepiphyseal dysplasia

    Science.gov (United States)

    ... the body that this condition affects: the ears (oto-), the bones of the spine (spondylo-), and the ... dwarfism Nance-Insley syndrome Nance-Sweeney chondrodysplasia OSMED oto-spondylo-megaepiphyseal dysplasia Related Information How are genetic ...

  9. Defining dysplasia in Barrett esophagus.

    Science.gov (United States)

    Robert, Marie E

    2003-01-01

    Histologic grading of esophageal biopsies remains the cornerstone of management in patients with Barrett esophagus. This remains true despite ongoing research into the genetic abnormalities that occur in the setting of intestinal metaplasia, dysplasia, and adenocarcinoma of the esophagus. As of this writing, there are no objective tests that can replace the pathologist's eyes in predicting prognosis in this setting. However, pathologists and clinicians are well aware that the histologic interpretation of biopsies is an inherently subjective practice. The validity of this exercise depends entirely on the experience of the pathologist. Having said that, two interobserver studies on the grading of dysplasia in Barrett esophagus suggest that, if published criteria are adhered to, the histologic grading of dysplasia is a useful tool in patient management. In this review, the updated pathologic criteria for each category of dysplasia are presented, and the results of two large interobserver variability studies are reviewed.

  10. Genetics Home Reference: osteoglophonic dysplasia

    Science.gov (United States)

    ... leads to severe head and face (craniofacial) abnormalities, dwarfism, and other features. The term osteoglophonic refers to ... and Management Resources (1 link) Seattle Children's Hospital: Dwarfism and Bone Dysplasias General Information from MedlinePlus (5 ...

  11. Genetics Home Reference: metatropic dysplasia

    Science.gov (United States)

    ... is a skeletal disorder characterized by short stature (dwarfism) with other skeletal abnormalities. The term "metatropic" is ... my area? Other Names for This Condition metatropic dwarfism metatropic dysplasia type 1 Related Information How are ...

  12. Genetics Home Reference: Kniest dysplasia

    Science.gov (United States)

    ... disorder of bone growth characterized by short stature (dwarfism) with other skeletal abnormalities and problems with vision ... for This Condition Kniest chondrodystrophy Kniest syndrome Metatropic dwarfism, type II Metatropic dysplasia type II Swiss cheese ...

  13. Genetics Home Reference: anauxetic dysplasia

    Science.gov (United States)

    ... is a disorder characterized by extremely short stature (dwarfism) and other skeletal abnormalities, an unusually large range ... in this disorder. People with anauxetic dysplasia have dwarfism with unusually short limbs for their height (disproportionate ...

  14. Genetics Home Reference: campomelic dysplasia

    Science.gov (United States)

    ... my area? Other Names for This Condition campomelic dwarfism campomelic syndrome camptomelic dysplasia Related Information How are ... Robin Syndrome Health Topic: Bone Diseases Health Topic: Dwarfism Genetic and Rare Diseases Information Center (1 link) ...

  15. Ectodermal dysplasia with true anodontia

    Directory of Open Access Journals (Sweden)

    Madhu Bala

    2011-01-01

    Full Text Available 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.

  16. Focal cortical dysplasia – review

    Science.gov (United States)

    Kabat, Joanna; Król, Przemysław

    2012-01-01

    Summary Focal cortical dysplasia is a malformation of cortical development, which is the most common cause of medically refractory epilepsy in the pediatric population and the second/third most common etiology of medically intractable seizures in adults. Both genetic and acquired factors are involved in the pathogenesis of cortical dysplasia. Numerous classifications of the complex structural abnormalities of focal cortical dysplasia have been proposed – from Taylor et al. in 1971 to the last modification of Palmini classification made by Blumcke in 2011. In general, three types of cortical dysplasia are recognized. Type I focal cortical dysplasia with mild symptomatic expression and late onset, is more often seen in adults, with changes present in the temporal lobe. Clinical symptoms are more severe in type II of cortical dysplasia usually seen in children. In this type, more extensive changes occur outside the temporal lobe with predilection for the frontal lobes. New type III is one of the above dysplasias with associated another principal lesion as hippocampal sclerosis, tumor, vascular malformation or acquired pathology during early life. Brain MRI imaging shows abnormalities in the majority of type II dysplasias and in only some of type I cortical dysplasias. The most common findings on MRI imaging include: focal cortical thickening or thinning, areas of focal brain atrophy, blurring of the gray-white junction, increased signal on T2- and FLAIR-weighted images in the gray and subcortical white matter often tapering toward the ventricle. On the basis of the MRI findings, it is possible to differentiate between type I and type II cortical dysplasia. A complete resection of the epileptogenic zone is required for seizure-free life. MRI imaging is very helpful to identify those patients who are likely to benefit from surgical treatment in a group of patients with drug-resistant epilepsy. However, in type I cortical dysplasia, MR imaging is often normal, and also

  17. Predictive factors for neuromotor abnormalities at the corrected age of 12 months in very low birth weight premature infants Fatores preditivos para anormalidades neuromotoras aos 12 meses de idade corrigida em prematuros de muito baixo peso

    Directory of Open Access Journals (Sweden)

    Rosane Reis de Mello

    2009-06-01

    Full Text Available BACKGROUND: The increase in survival of premature newborns has sparked growing interest in the prediction of their long-term neurodevelopment. OBJECTIVE: To estimate the incidence of neuromotor abnormalities at the corrected age of 12 months and to identify the predictive factors associated with altered neuromotor development in very low birth weight premature infants. METHOD: Cohort study. The sample included 100 premature infants. The outcome was neuromotor development at 12 months classified by Bayley Scale (PDI and neurological assessment (tonus, reflexes, posture. A multivariate logistic regression model was constructed. Neonatal variables and neuromotor abnormalities up to 6 months of corrected age were selected by bivariate analysis. RESULTS: Mean birth weight was 1126g (SD: 240. Abnormal neuromotor development was presented in 60 children at 12 months corrected age. CONCLUSION: According to the model, patients with a diagnosis including bronchopulmonary dysplasia, hypertonia of lower extremities, truncal hypotonia showed a 94.0% probability of neuromotor involvement at 12 months.INTRODUÇÃO: O aumento na sobrevida de recém-nascidos prematuros tem suscitado interesse crescente na predição do seu neurodesenvolvimento a longo prazo. OBJETIVO: Estimar a incidência de anormalidades neuromotoras aos 12 meses de idade corrigida e identificar os fatores associados ao desenvolvimento neuromotor alterado em prematuros de muito baixo peso. MÉTODO: Estudo de coorte. A amostra incluiu 100 crianças prematuras.O desfecho foi o desenvolvimento neuromotor aos 12 meses. Modelo de regressão logística multivariado foi construído. Variáveis neonatais e anormalidades neuromotoras até os 6 meses de idade corrigida foram selecionadas por análise bivariada. RESULTADOS: O peso de nascimento médio foi 1126g (DP:240. Aos 12 meses 60% das crianças apresentaram desenvolvimento neuromotor alterado. CONCLUSÃO: De acordo com o modelo, pacientes com diagn

  18. Pediatric aspects of skeletal dysplasia.

    Science.gov (United States)

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

    2012-10-01

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

  19. Role of inhaled amphotericin in allergic bronchopulmonary aspergillosis

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    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.

  20. Macroscopic morphology and bronchopulmonary distribution in lungs of Procyon cancrivorus

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    Celina Almeida Furlanetto Mançanares

    2013-11-01

    Full Text Available The raccoon (Procyon cancrivorus is a wild animal of great ecological importance because it is a seed disperser. Bronchopulmonary segmentation was studied in 4 raccoons, using 10% formaldehyde-fixed lung tissue. Lung lobation in P. cancrivorus follows the same pattern as in domestic carnivorous animals and coatis (four right and two left lobes. The lungs of the raccoon are divided into lobes: cranial, middle, caudal and accessory in right lung and cranial and caudal in left lung. The bronchial tree begins with the right and left primary bronchi, which divide into lobar bronchi, which in turn divide into segmental bronchi. Distribution patterns of lobar bronchi and segmental bronchi vary in quantity and arrangement, between the species studied here and other carnivorous species described in the literature. Therefore, we suggest further studies with a larger number of specimens, allowing a statistical analysis of the data to define the bronchial pattern in raccoons.

  1. Exophiala pisciphila: a novel cause of allergic bronchopulmonary mycosis

    Science.gov (United States)

    Mador, M. Jeffery

    2016-01-01

    Allergic bronchopulmonary mycosis (ABPM) is a hypersensitivity reaction to fungal antigens, which may particularly plague uncontrolled asthmatics. Non-aspergillus fungal organisms may be implicated and may elicit a more severe immunologic response. Exophiala pisciphila, a marine organism, has not been reported as a culprit yet. However, this report indicates it may be implicated in unrelenting symptoms in a severe asthmatic patient who had become dependent on corticosteroids. Proper identification and adequate therapy of this organism led to complete resolution of respiratory symptoms, with adequate subsequent control of the asthma. ABPM may complicate asthma and lead to a lack of its control. Proper awareness, testing and treatment of non-aspergillus pulmonary mycosis is essential to proper asthma care and beneficial for its control. PMID:27499992

  2. 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.

  3. Pseudomonas bronchopulmonary infections in a palliative care setting

    Directory of Open Access Journals (Sweden)

    Naveen Salins

    2015-04-01

    Full Text Available Blood stream infections and pneumonia caused by Pseudomonas aeruginosa is associated with high mortality, especially in an immunocompromised host. A large section of the palliative care patient population has varied forms of compromised immunity due to advanced cancer or cancer treatment, organ failures, chronic autoimmune disorders, degenerative conditions, and acquired immunodeficiency syndrome. The lung is one of the most frequently involved organs in a variety of complications in an immunocompromised host and infection is the most common complication. P. aeruginosa is one of the most common pathogens associated with bronchopulmonary infections in an immunocompromised host. Routine radiological tests like chest X-ray may often be unyielding and an early and a prompt initiation of treatment reduces mortality and morbidity risk.

  4. Clinical comparison of early prophylactic application of nasal continuous positive airway pressure and pulmonary surfactant in the premature very low birth weight infants%早产极低出生体重儿早期预防性应用鼻塞式持续气道正压与肺表面活性物质的临床比较

    Institute of Scientific and Technical Information of China (English)

    高薇薇; 谭三智; 杨杰; 张永; 叶秀桢; 聂川; 王越; 王俊平

    2012-01-01

    目的:探讨在早产极低出生体重儿中生后早期使用鼻塞式持续气道正压(nCPAP)或应用肺表面活性剂(PS)以预防新生儿呼吸窘迫综合征(NRDS)的临床疗效比较.方法:采用前瞻性对照研究,对出生后早期使用nCPAP辅助通气或早期使用PS替代治疗的早产极低出生体重儿进行对照研究,观察其死亡率、支气管肺发育不良发生率、辅助通气时间及临床并发症.结果:共106例患儿人选,死亡率和支气管肺发育不良(BPD)发生率组间比较差异无统计学意义.机械辅助通气、总用氧时间组间比较差异无统计学意义;nCPAP组PS使用数少于对照组(Odds Ratio:0.37,90% CI 0.209~0.655);气漏发生率低(Odds Ratio:0.476,90% CI0.233~0.971),IVH发生率低(Odds Ratio:o.778,90%CI 0.319~1.898).其他临床并发症组间比较差异无统计学意义.结论:早期应用nCPAP是预防早产极低出生体重儿呼吸窘迫综合征另一良好选择.%Objective; To explore the clinical effects of early prophylactic application of nasal continuous positive airway pressure (nCPAP) and pulmonary surfactant (PS) in prevention of neonatal respiratory distress syndrome ( NROS) among the premature very low birth weight infants. Methods: The clinical effects of early prophylactic application of nCPAP and PS in the premature very low birth weight infants were compared, the death rates, the incidences of bronchopulmonary dysplasia, the duration times of assisted ventilation, and clinical complications in the two groups were observed. Results; A total of 106 infants were enrolled in the study. There was no significant difference in the death rate and the incidence of bronchopulmonary dysplasia between the two groups. There was no significant difference in the duration time of mechanical ventilation and total using oxygen time between the two groups; the number of infants using PS in nCPAP group was fewer than that in control group (Odds ratio; 0. 37, 90

  5. 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

  6. 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.

  7. Spondylometaphyseal dysplasia with hypercalcemia. [Radiological studies

    Energy Technology Data Exchange (ETDEWEB)

    Bagga, A.; Srivastava, R.N.; Gupta, S.; Gupta, A.

    1989-08-01

    Kozlowski's spondylometaphyseal dysplasia is characterised by short-trunk dwarfism, platyspondyly, metaphyseal dysplasia and retarded bone age. We report an association of this syndrome with asymptomatic, hypocalciuric hypercalcemia, a previously undocumented finding. (orig.).

  8. Genetics Home Reference: hypohidrotic ectodermal dysplasia

    Science.gov (United States)

    ... Names for This Condition Anhidrotic Ectodermal Dysplasia Christ-Siemens-Touraine Syndrome CST syndrome HED Related Information How ... NORD) Resource list from the University of Kansas Medical Center GeneReviews (1 link) Hypohidrotic Ectodermal Dysplasia ClinicalTrials. ...

  9. 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 o

  10. Schimke immuno-osseous dysplasia: two cases

    Energy Technology Data Exchange (ETDEWEB)

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

    2003-03-01

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

  11. 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.

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

    NARCIS (Netherlands)

    Jiskoot-Ermers, M.E.C.; Antonius, T.A.J.; Looijen, M.G.; Wijnen, M.H.W.A.; Loza, B.F.; Heijst, A.F.J. van

    2015-01-01

    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 demonstrate

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

    Science.gov (United States)

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

    2017-10-01

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

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

    NARCIS (Netherlands)

    Jiskoot-Ermers, M.E.C.; Antonius, T.A.J.; Looijen, M.G.; Wijnen, M.H.W.A.; Loza, B.F.; Heijst, A.F.J. van

    2015-01-01

    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

  15. Chondroectodermal dysplasia: a rare syndrome.

    Directory of Open Access Journals (Sweden)

    Dana Tahririan

    2014-06-01

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

  16. The nature of fibrous dysplasia

    Directory of Open Access Journals (Sweden)

    Lemmer Johan

    2009-11-01

    Full Text Available Abstract Fibrous dysplasia has been regarded as a developmental skeletal disorder characterized by replacement of normal bone with benign cellular fibrous connective tissue. It has now become evident that fibrous dysplasia is a genetic disease caused by somatic activating mutation of the Gsα subunit of G protein-coupled receptor resulting in upregulation of cAMP. This leads to defects in differentiation of osteoblasts with subsequent production of abnormal bone in an abundant fibrous stroma. In addition there is an increased production of IL-6 by mutated stromal fibrous dysplastic cells that induce osteoclastic bone resorption.

  17. 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. 

  18. Micafungin in Premature and Non-premature Infants

    Science.gov (United States)

    Wu, Chunzhang; Tweddle, Lorraine; Roilides, Emmanuel

    2014-01-01

    Background: Invasive fungal infections cause excessive morbidity and mortality in premature neonates and severely ill infants. Methods: Safety and efficacy outcomes of micafungin were compared between prematurely and non-prematurely born infants premature [birth weight (BW) premature, received ≥1 dose of micafungin. Among premature patients, 14.5% were low BW (1500–2499 g), 36.4% very low BW (1000–1499 g) and 49.1% extremely low BW (premature)] died. Significantly more non-premature than premature patients discontinued treatment (P = 0.003). Treatment-related adverse events were recorded in 23% of patients with no difference between groups. More extremely low BW (n = 4, 15%) and very low BW (n = 8, 40%) infants experienced treatment-related adverse events than low BW (n = 0) and there was no relation to micafungin dose or duration. For a subgroup of 30 patients with invasive candidiasis, treatment success was achieved in 73% in both premature and non-premature groups. Prophylaxis was successful in 4/5 non-premature hematopoietic stem cell transplant patients. Conclusion: Micafungin has a safe profile in premature and non-premature infants with substantial efficacy. PMID:24892849

  19. Fetal lung dysplasia: clinical outcome based on a new classification system.

    Science.gov (United States)

    Achiron, R; Zalel, Y; Lipitz, S; Hegesh, J; Mazkereth, R; Kuint, J; Jacobson, J; Yagel, S

    2004-08-01

    immediate surgical repair. Type V miscellaneous dysplasia: One fetus demonstrated echogenic lung with split notochord syndrome and survived. Congenital bronchopulmonary and related vascular anomalies can be categorized using the new classification system. This new approach enabled prenatal evaluation of each lung component and facilitated cogent management of the fetus with congenital lung dysplasia. Copyright 2004 ISUOG

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

    Science.gov (United States)

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

    2016-07-01

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

  1. 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.

  2. Perfusion pulmonoscintigraphy in diagnostic of broncho-pulmonary paecilomycosis

    Directory of Open Access Journals (Sweden)

    Abdusalom Ashurov

    2010-12-01

    Full Text Available 74 patients with broncho-pulmonary paecilomycosis were observed for the indexes of pulmonoscintigraphy. According to the clinical manifestations of the disease all investigated patients were divided into two groups: 1st group - 25 people with predominance of the clinical signs of bronchial asthma (5 people with chronic obstructive bronchitis, 20 people with bronchial asthma and 2nd group - 54 people with predominance of the clinical signs of inflammation process of the lungs' parenchyma and interstitial tissue (38 patients with pneumonia, 16 patients with exogenous-allergic alveolitis. The results of radionuclide investigation showed that scintigraphic pictures of the investigated people of the 1st and 2nd groups were significantly differed from the lungs' picture of the patients from the control group. During visual evaluation of the scintigrams the areas of disorders of pulmonary circulation were presented by defects of different degree. Also, they had uneven decreasing of the distribution of the radiopharmaceutical drug in the injured areas, most often in the middle and lower areas of the lungs, sometimes along the whole lung areas. Density of radiopharmaceutical distribution in these areas was decreased in comparison with the adjoining lung tissue. The functional area of parenchyma and the form of the organ were not changed.

  3. Allergic bronchopulmonary aspergillosis in asthma and cystic fibrosis.

    Science.gov (United States)

    Knutsen, Alan P; Slavin, Raymond G

    2011-01-01

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

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

    Science.gov (United States)

    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 view confirmed a MLS, which was further corroborated by high resolution computed tomography. Central bronchiectasis was also observed, which prompted a work-up for ABPA. The child met 7/8 major diagnostic criteria for ABPA. She was then initiated on oral prednisolone that resulted in a marked clinical improvement within a fortnight. Radiological clearance occurred at 3 months with inflation of the middle lobe. ABPA presenting with MLS in a child is yet to be reported. A high index of suspicion is required to establish the diagnosis of ABPA in a child presenting with MLS. This would obviate the invasive investigations usually done to ascertain the cause of MLS.

  5. Oral epithelial dysplasia classification systems

    DEFF Research Database (Denmark)

    Warnakulasuriya, S; Reibel, J; Bouquot, J

    2008-01-01

    ) and was of the view that reducing the number of choices from 3 to 2 may increase the likelihood of agreement between pathologists. The utility of this need to be tested in future studies. The variables that are likely to affect oral epithelial dysplasia scoring were discussed and are outlined here; these need...

  6. 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.

  7. Skeletal dysplasias: A radiographic approach and review of common non-lethal skeletal dysplasias

    Institute of Scientific and Technical Information of China (English)

    Ananya; Panda; Shivanand; Gamanagatti; Manisha; Jana; Arun; Kumar; Gupta

    2014-01-01

    Skeletal dysplasias are not uncommon entities and a radiologist is likely to encounter a suspected case of dysplasia in his practice. The correct and early diagnosis of dysplasia is important for management of complications and for future genetic counselling. While there is an exhaustive classification system on dysplasias, it is important to be familiar with the radiological features of common dysplasias. In this article, we enumerate a radiographic approach to skeletal dysplasias, describe the essential as well as differentiating features of common non-lethal skeletal dysplasias and conclude by presenting working algorithms to either definitively diagnose a particular dysplasia or suggest the most likely differential diagnoses to the referring clinician and thus direct further workup of the patient.

  8. 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.

  9. Cleidocranial dysplasia: a case report

    Energy Technology Data Exchange (ETDEWEB)

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

    2005-12-15

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

  10. Skeletal dysplasia in ancient Egypt.

    Science.gov (United States)

    Kozma, Chahira

    2008-12-01

    The ancient Egyptian civilization lasted for over 3000 years and ended in 30 BCE. Many aspects of ancient Egyptian culture, including the existence of skeletal dysplasias, and in particular achondroplasia, are well known through the monuments and records that survived until modern times. The hot and dry climate in Egypt allowed for the preservation of bodies and skeletal anomalies. The oldest dwarf skeleton, the Badarian skeleton (4500 BCE), possibly represents an epiphyseal disorder. Among the remains of dwarfs with achondroplasia from ancient Egypt (2686-2190 BCE), exists a skeleton of a pregnant female, believed to have died during delivery with a baby's remains in situ. British museums have partial skeletons of dwarfs with achondroplasia, humeri probably affected with mucopolysaccharidoses, and a skeleton of a child with osteogenesis imperfecta. Skeletal dysplasia is also found among royal remains. The mummy of the pharaoh Siptah (1342-1197 BCE) shows a deformity of the left leg and foot. A mummified fetus, believed to be the daughter of king Tutankhamun, has scoliosis, spina bifida, and Sprengel deformity. In 2006 I reviewed the previously existing knowledge of dwarfism in ancient Egypt. The purpose of this second historical review is to add to that knowledge with an expanded contribution. The artistic documentation of people with skeletal dysplasia from ancient Egypt is plentiful including hundreds of amulets, statues, and drawing on tomb and temple walls. Examination of artistic reliefs provides a glance of the role of people with skeletal dysplasia and the societal attitudes toward them. Both artistic evidence and moral teachings in ancient Egypt reveal wide integration of individuals with disabilities into the society.

  11. MR imaging findings of acetabular dysplasia in adults

    Energy Technology Data Exchange (ETDEWEB)

    James, Steven; Connell, David [The Royal National Orthopaedic Hospital, Radiology Department, London, Middlesex (United Kingdom); Miocevic, Miranda; Malara, Frank; Pike, Jonathan [Victoria House Hospital, Radiology Department, Melbourne (Australia); Young, David [Melbourne Orthopaedic Group, Orthopaedic Surgery, Melbourne (Australia)

    2006-06-15

    To evaluate the diagnostic accuracy of MR imaging in the identification of labral and articular cartilage lesions in patients with acetabular dysplasia. Pre-operative MR imaging was performed on 27 hips in 25 consecutive patients (16 males, 9 females, age range 19-52 years, mean age 31.2 years) with radiographic evidence of acetabular dysplasia (centre-edge angle of Wiberg <20 degrees). The average duration of symptoms was 16.2 months. Two musculoskeletal radiologists assessed MR images in consensus for the presence of abnormality involving the acetabular labrum and adjacent acetabular articular cartilage. A high resolution, non-arthrographic technique was used to assess the labrum and labral chondral transitional zone. Surgical correlation was obtained in all cases by a single surgeon experienced in hip arthroscopy and ten patients with normal hip MRI were included to provide a control group. The acetabular labra in the dysplastic hips demonstrated abnormal signal intensity, and had an elongated appearance when compared with the control group (mean length 10.9 mm vs 6.4 mm). Morphological appearances in the labra included surface irregularity, fissures and cleft formation. MR imaging correctly identified the severity of chondral abnormality in 24 of 27 hips (89%) when compared with arthroscopic findings. MR imaging demonstrates an elongated labrum, focal intra-substance signal change and irregularity and fissuring of the margins in patients with acetabular dysplasia. Abnormality is also identified at the labral chondral transitional zone, where fissuring, focal clefts, chondral deficiency and subchondral cyst formation may be apparent. A high-resolution, non-arthrographic technique can provide an accurate preoperative assessment and evaluate the presence of premature osteoarthritis. (orig.)

  12. Optic Nerve Decompression for Orbitofrontal Fibrous Dysplasia

    OpenAIRE

    Abe, Takumi; Sato, Kaneshige; Otsuka, Takaharu; Kawamura, Noriyoshi; Shimazu, Motohiko; Izumiyama, Hitoshi; Matsumoto, Kiyoshi

    2002-01-01

    Orbitofrontal fibrous dysplasia often involves the bony orbit and the optic canal. Although fibrous dysplasia reportedly produces compression of the optic nerve leading to visual distrubances, optic nerve decompression in patients without clinical signs of optic neuropathy is still controversial. We describe two patients with orbitofrontal fibrous dysplasia without signs of visual disturbance and one patient with McCune-Albright syndrome and progressive visual impairment. Optic nerve decompre...

  13. Dysplasia in view of the cell cycle

    OpenAIRE

    RG Steinbeck

    2009-01-01

    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 cont...

  14. Lifestyle influences on prematurity.

    Science.gov (United States)

    Creasy, R K

    1991-01-01

    It is apparent from this review that the lifestyle of an individual gravida can potentially lead to a premature delivery. Some of these adverse behavioral characteristics may be dealt with by education and motivation, and some with actual medical treatment. However, there also appears to be significant need for public policy reorientation if we are to make a significant impact on the problem of preterm delivery.

  15. Spondyloepiphyseal Dysplasia Tarda in Twins

    Directory of Open Access Journals (Sweden)

    Ferhat Çekmez

    2011-08-01

    Full Text Available Introduction: Spondyloepiphyseal dysplasia (SED is a genetically heterogeneous disorder often associated with the early onset of osteoarthrosis. The X-linked recessive form (SEDL affects boys and is characterized by reduced height, arm span exceeding total height, and barrel chest deformity. The radiographic phenotype comprises a hump-shaped deformity of vertebral bodies and mild epiphyseal dysplasia of the femoral head associated with early signs of hip arthrosis.The disorder is caused by mutations in the SEDL (or sedlin gene on Xp22.12-p22.31. SED should be kept in mind as a differential diagnosis in men with early “primary” bilateral osteoarthrosis.Case Report: Here, 11 years old twins diagnosed as SED due to walking disability, limitation of joint motion, pain at the hips and disability in standing for a long time were presented. Discussion: This rare skelatal dysplasia must be kept in mind in patients with painful spine and hip joints, and walking disability. (Jo­ur­nal of Cur­rent Pe­di­at­rics 2011; 9: 97-9

  16. 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.

  17. Presentation of hypohidrotic ectodermal dysplasia in two siblings

    Directory of Open Access Journals (Sweden)

    Uday Ginjupally

    2015-01-01

    Full Text Available Ectodermal dysplasias are a large hereditary group of disorders which are usually manifested as X-linked recessive disorders and have a full expression in males, whereas females show little to no signs of the disorder. The two most common types of ectodermal dysplasias are hypohidrotic ectodermal dysplasia (Christ-Siemens-Touraine syndrome and hidrotic ectodermal dysplasia (Clouston syndrome. Hypohidrotic ectodermal dysplasia is characterized by hypodontia, hypotrichosis, and hypohidrosis. Here, we present two female sibling cases of hypohidrotic ectodermal dysplasia.

  18. 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

  19. 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%; abso

  20. Allergic bronchopulmonary mycosis due to co-infection with Aspergillus fumigatus and Schizophyllum commune

    Directory of Open Access Journals (Sweden)

    Masafumi Seki

    2014-01-01

    Full Text Available A 61-year-old female presented with eosinophilic pneumonia accompanied by bronchial asthma. She was finally diagnosed with allergic bronchopulmonary mycosis (ABPM due to co-infection with Aspergillus fumigatus and Schizophyllum commune detected by genetic analysis of the plug and from cultures.

  1. Improvement in health status following bronchopulmonary hygiene physical therapy in patients with bronchiectasis

    National Research Council Canada - National Science Library

    Mutalithas, Kugathasan; Watkin, Gillian; Willig, Briony; Wardlaw, Andrew; Pavord, Ian D; Birring, Surinder S

    2008-01-01

    ... therapeutic agents for patients with bronchiectasis. Bronchopulmonary hygiene physical therapy (BHPT) is the mainstay therapy for most patients and is widely prescribed as a prophylactic and therapeutic intervention. BHPT comprises patient education and a range of physical techniques that aid clearance of airway secretions. 5 Despite its routine...

  2. Allergic bronchopulmonary mycosis due to co-infection with Aspergillus fumigatus and Schizophyllum commune.

    Science.gov (United States)

    Seki, Masafumi; Ohno, Hideaki; Gotoh, Kazuyoshi; Motooka, Daisuke; Nakamura, Shota; Iida, Tetsuya; Miyazaki, Yoshitsugu; Tomono, Kazunori

    2014-01-01

    A 61-year-old female presented with eosinophilic pneumonia accompanied by bronchial asthma. She was finally diagnosed with allergic bronchopulmonary mycosis (ABPM) due to co-infection with Aspergillus fumigatus and Schizophyllum commune detected by genetic analysis of the plug and from cultures.

  3. Pattern recognition pathways leading to a Th2 cytokine bias in allergic bronchopulmonary aspergillosis patients

    NARCIS (Netherlands)

    Becker, K.L.; Gresnigt, M.S.; Smeekens, S.P.; Jacobs, C.W.M.; Magis-Escurra Ibanez, C.; Jaeger, M.; Wang, X.; Lubbers, R.; Oosting, M.; Joosten, L.A.B.; Netea, M.G.; Reijers, M.H.E.; Veerdonk, F.L. van de

    2015-01-01

    BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is characterised by an exaggerated Th2 response to Aspergillus fumigatus, but the immunological pathways responsible for this effect are unknown. OBJECTIVE: The aim of this study was to decipher the pattern recognition receptors (PRRs) and

  4. Aspirin induced asthma accompanied with allergic bronchopulmonary aspergillosis: a case report

    Institute of Scientific and Technical Information of China (English)

    TANG Rui; ZHANG Hong-yu

    2010-01-01

    @@ In this paper, we describe a patient with a rather severe form of aspirin-induced asthma (AIA) and allergic bronchopulmonary aspergillosis (ABPA). The patient is a man born in 1948, who first presented with rhinorrhea,nasal congestion and chronic urticaria, and had an episode of asthma after ingestion of non-steroidal anti-inflammatory drugs (NSAIDs) for the further eight years.

  5. Overexpression of Shox2 Leads to Congenital Dysplasia of the Temporomandibular Joint in Mice

    Directory of Open Access Journals (Sweden)

    Xihai Li

    2014-07-01

    Full Text Available Our previous study reported that inactivation of Shox2 led to dysplasia and ankylosis of the temporomandibular joint (TMJ, and that replacing Shox2 with human Shox partially rescued the phenotype with a prematurely worn out articular disc. However, the mechanisms of Shox2 activity in TMJ development remain to be elucidated. In this study, we investigated the molecular and cellular basis for the congenital dysplasia of TMJ in Wnt1-Cre; pMes-stop Shox2 mice. We found that condyle and glenoid fossa dysplasia occurs primarily in the second week after the birth. The dysplastic TMJ of Wnt1-Cre; pMes-stop Shox2 mice exhibits a loss of Collagen type I, Collagen type II, Ihh and Gli2. In situ zymography and immunohistochemistry further demonstrate an up-regulation of matrix metalloproteinases (MMPs, MMP9 and MMP13, accompanied by a significantly increased cell apoptosis. In addition, the cell proliferation and expressions of Sox9, Runx2 and Ihh are no different in the embryonic TMJ between the wild type and mutant mice. Our results show that overexpression of Shox2 leads to the loss of extracellular matrix and the increase of cell apoptosis in TMJ dysplasia by up-regulating MMPs and down-regulating the Ihh signaling pathway.

  6. Overexpression of Shox2 leads to congenital dysplasia of the temporomandibular joint in mice.

    Science.gov (United States)

    Li, Xihai; Liang, Wenna; Ye, Hongzhi; Weng, Xiaping; Liu, Fayuan; Liu, Xianxiang

    2014-07-24

    Our previous study reported that inactivation of Shox2 led to dysplasia and ankylosis of the temporomandibular joint (TMJ), and that replacing Shox2 with human Shox partially rescued the phenotype with a prematurely worn out articular disc. However, the mechanisms of Shox2 activity in TMJ development remain to be elucidated. In this study, we investigated the molecular and cellular basis for the congenital dysplasia of TMJ in Wnt1-Cre; pMes-stop Shox2 mice. We found that condyle and glenoid fossa dysplasia occurs primarily in the second week after the birth. The dysplastic TMJ of Wnt1-Cre; pMes-stop Shox2 mice exhibits a loss of Collagen type I, Collagen type II, Ihh and Gli2. In situ zymography and immunohistochemistry further demonstrate an up-regulation of matrix metalloproteinases (MMPs), MMP9 and MMP13, accompanied by a significantly increased cell apoptosis. In addition, the cell proliferation and expressions of Sox9, Runx2 and Ihh are no different in the embryonic TMJ between the wild type and mutant mice. Our results show that overexpression of Shox2 leads to the loss of extracellular matrix and the increase of cell apoptosis in TMJ dysplasia by up-regulating MMPs and down-regulating the Ihh signaling pathway.

  7. Premature aging syndrome.

    Science.gov (United States)

    Coppedè, Fabio

    2012-01-01

    Hutchinson-Gilford progeria syndrome and Werner syndrome are two of the best characterized human progeroid diseases with clinical features mimicking physiological aging at an early age. Both disorders have been the focus of intense research in recent years since they might provide insights into the pathology of normal human aging. The chapter contains a detailed description of the clinical features of both disorders and then it focuses on the genetics, the resulting biochemical alterations at the protein level and the most recent findings and hypotheses concerning the molecular basis of the premature aging phenotypes. A description of available diagnostic and therapeutic approaches is included.

  8. Developmental Dislocation (Dysplasia) of the Hip (DDH)

    Science.gov (United States)

    .org Developmental Dislocation (Dysplasia) of the Hip (DDH) Page ( 1 ) The hip is a “ball-and-socket” joint. In a normal hip, the ball at the ... American Academy of Orthopaedic Surgeons. .org Developmental Dislocation (Dysplasia) of the Hip cont. • Family history of DDH (parents or siblings) • ...

  9. Schneckenbecken dysplasia, radiology, and histology

    Energy Technology Data Exchange (ETDEWEB)

    Nikkels, P.G. [Rijksuniversiteit Utrecht (Netherlands). Dept. of Pathology; Stigter, R.H. [Div. of Neonatology and Obstetrics, University Medical Centre Utrecht (Netherlands); Knol, I.E. [Div. of Medical Genetics, University Medical Centre Utrecht (Netherlands); Harten, H.J. van der [Dept. of Pathology, Free University Amsterdam (Netherlands)

    2001-01-01

    To our knowledge this is the first report of Schneckenbecken dysplasia with the development of hydrops early in the second trimester. The radiological findings showed the typical hypoplastic iliac bones with medial extension and very flattened, on lateral view, oval-shaped vertebral bodies and short long bones. The histology showed hypercellular and hypervascular cartilage with chondrocytes with centrally located nucleus. The absence of the lacunar space as described before was also observed in some chondrocytes in our case. This male fetus was the product of consanguineous parents of Mediterranean origin compatible with autosomal recessive inheritance. (orig.)

  10. Canine Hip Dysplasia: Diagnostic Imaging.

    Science.gov (United States)

    Butler, J Ryan; Gambino, Jennifer

    2017-07-01

    Diagnostic imaging is the principal method used to screen for and diagnose hip dysplasia in the canine patient. Multiple techniques are available, each having advantages, disadvantages, and limitations. Hip-extended radiography is the most used method and is best used as a screening tool and for assessment for osteoarthritis. Distraction radiographic methods such as the PennHip method allow for improved detection of laxity and improved ability to predict future osteoarthritis development. More advanced techniques such as MRI, although expensive and not widely available, may improve patient screening and allow for improved assessment of cartilage health. Copyright © 2017 Elsevier Inc. All rights reserved.

  11. Васкground development questionnaire quality of life for school age children with broncho-pulmonary disease

    Directory of Open Access Journals (Sweden)

    Nataliya Ivasyk

    2015-08-01

    Full Text Available Purpose: to prove feasibility of developing a questionnaire to assess quality of life for school-age children with acute broncho-pulmonary diseases. Material and Methods: analysis of scientific and methodological literature on the study of quality of life. Results: to assess quality of life using both general and specific questionnaires. The most of special are questionnaires designed for adults, and all pulmonary questionnaires designed for chronic diseases. There are of survey questionnaire for children with acute broncho-pulmonary diseases. Conclusions: the proposed of us a questionnaire for children with broncho-pulmonary diseases include questions to determine the effect impact of symptoms of diseasea on motor activity and quality of life. In future we plans to cheak effectiveness of the application of this questionnaire for determine quality of life of children with acute broncho-pulmonary diseases with goal to determine the effectiveness of the rehabilitation process

  12. Academic Performance, Motor Function, and Behavior 11 Years After Neonatal Caffeine Citrate Therapy for Apnea of Prematurity: An 11-Year Follow-up of the CAP Randomized Clinical Trial.

    Science.gov (United States)

    Schmidt, Barbara; Roberts, Robin S; Anderson, Peter J; Asztalos, Elizabeth V; Costantini, Lorrie; Davis, Peter G; Dewey, Deborah; D'Ilario, Judy; Doyle, Lex W; Grunau, Ruth E; Moddemann, Diane; Nelson, Harvey; Ohlsson, Arne; Solimano, Alfonso; Tin, Win

    2017-06-01

    Caffeine citrate therapy for apnea of prematurity reduces the rates of bronchopulmonary dysplasia, severe retinopathy, and neurodevelopmental disability at 18 months and may improve motor function at 5 years. To evaluate whether neonatal caffeine therapy is associated with improved functional outcomes 11 years later. A follow-up study was conducted at 14 academic hospitals in Canada, Australia, and the United Kingdom from May 7, 2011, to May 27, 2016, of English- or French-speaking children who had been enrolled in the randomized, placebo-controlled Caffeine for Apnea of Prematurity trial between October 11, 1999, and October 22, 2004. A total of 1202 children with birth weights of 500 to 1250 g were eligible for this study; 920 (76.5%) had adequate data for the main outcome. Caffeine citrate or placebo until drug therapy for apnea of prematurity was no longer needed. Functional impairment was a composite of poor academic performance (defined as at least 1 standard score greater than 2 SD below the mean on the Wide Range Achievement Test-4), motor impairment (defined as a percentile rank of ≤5 on the Movement Assessment Battery for Children-Second Edition), and behavior problems (defined as a Total Problem T score ≥2 SD above the mean on the Child Behavior Checklist). Among the 920 children (444 females and 476 males; median age, 11.4 years [interquartile range, 11.1-11.8 years]), the combined rates of functional impairment were not significantly different between the 457 children assigned to receive caffeine compared with the 463 children assigned to receive placebo (145 [31.7%] vs 174 [37.6%]; adjusted odds ratio, 0.78; 95% CI, 0.59-1.02; P = .07). With all available data, including those from up to 24 Swedish trial participants, the rates of poor academic performance on 1 or more of 4 subtests (66 of 458 [14.4%] vs 61 of 462 [13.2%]; adjusted odds ratio, 1.11; 95% CI, 0.77-1.61; P = .58) and behavior problems (52 of 476 [10.9%] vs 40 of 481 [8

  13. Premature ovarian failure.

    Science.gov (United States)

    Shelling, Andrew N

    2010-11-01

    Premature ovarian failure (POF) is a common cause of infertility in women, and is characterised by amenorrhoea, hypo-oestrogenism and elevated gonadotrophin levels in women under the age of 40. Known causes include iatrogenic agents that cause permanent damage to the ovaries, such as chemotherapy, radiation therapy and surgery, autoimmune conditions, X-chromosome abnormalities and autosomal genetic conditions. However, few genes have been identified that can explain a substantial proportion of cases of POF. Most women with POF are deeply upset by the diagnosis, partly due to the unexpected menopausal symptoms, but also due to infertility. Therefore, early detection would provide better opportunity for early intervention, and furthermore, the identification of specific gene defects will help to direct potential targets for future treatment.

  14. Apnea of prematurity

    Directory of Open Access Journals (Sweden)

    Piermichele Paolillo

    2013-06-01

    Full Text Available Apnea of prematurity (AOP is one of the most frequent pathologies in the Neonatal Intensive Care Unit, with an incidence inversely related to gestational age. Its etiology is often multi factorial and diagnosis of idiopathic forms requires exclusion of other underlying diseases. Despite being a self-limiting condition which regresses with the maturation of the newborn, possible long-term effects of recurring apneas and the degree of desaturation and bradycardia who may lead to abnormal neurological outcome are not yet clarified. Therefore AOP needs careful evaluation of its etiology and adequate therapy that can be both pharmacological and non-pharmacological. Proceedings of the 9th International Workshop on Neonatology · Cagliari (Italy · October 23rd-26th, 2013 · Learned lessons, changing practice and cutting-edge research

  15. Premature ejaculation: A review

    Directory of Open Access Journals (Sweden)

    Sukumar Reddy Gajjala

    2014-01-01

    Full Text Available Premature ejaculation (PE is a common male sexual disorder. It is defined by the Diagnostic and statistical manual of mental disorders as "ejaculation occurring, without control, on or shortly after penetration and before the person wishes it, causing marked distress or interpersonal difficulty. [1] Although the timing of intravaginal ejaculatory latency time (IELT (i.e., time from penetration to ejaculation is not included in this definition, an IELT of <2 min, or ejaculation occurring before penetration, has been considered consistent with PE. [2] Management involves both the patient and his partner. Therapeutic options should suit both partners and be appropriate to their habit in planning and frequency of intercourse. Follow-up at appropriate intervals to judge efficacy, titrate dosage of pharmacological treatments and ascertain side effects is mandatory.

  16. Intrauterine infection and prematurity.

    Science.gov (United States)

    Gonçalves, Luís F; Chaiworapongsa, Tinnakorn; Romero, Roberto

    2002-01-01

    Intrauterine infection is a major cause of premature labor with and without intact membranes. Intrauterine infection is present in approximately 25% of all preterm births and the earlier the gestational age at delivery, the higher the frequency of intra-amniotic infection. Microorganisms may also gain access to the fetus before delivery. A fetal inflammatory response syndrome elicited in response to microbial products is associated with the impending onset of preterm labor and also with multi-systemic organ involvement in the human fetus and a higher rate of perinatal morbidity. The most common microorganisms involved in intrauterine infections are Ureaplasma urealyticum, Fusobacterium species and Mycoplasma hominis. The role of Chlamydia trachomatis and viruses in preterm labor remain to be determined. Use of molecular microbiology techniques to diagnose intrauterine infection may uncover the role of fastidious microorganisms that have not yet been discovered. Antibiotic administration to patients with asymptomatic bacteriuria is associated with a significant reduction in the rate of preterm birth. However, such benefit has not been demonstrated for patients with bacterial vaginosis, or women who carry Streptococcus agalactia, Ureaplasma urealyticum or Trichomonas vaginalis. Antibiotic administration to patients with preterm premature rupture of membranes is associated with prolongation of pregnancy and a reduction in the rate of clinical chorioamnionitis and neonatal sepsis. The benefit has not been demonstrated in patients with preterm labor and intact membranes. Major efforts are required to determine why some women develop an ascending intrauterine infection and others do not and also what interventions may reduce the deleterious effect of systemic fetal inflammation. Copyright 2002 Wiley-Liss, Inc.

  17. Apnea of Prematurity (For Parents)

    Science.gov (United States)

    ... other babies. The apnea of prematurity does not cause brain damage. A healthy baby who is apnea free for a week will probably never have AOP again. Although sudden infant death syndrome (SIDS) does happen more often in premature infants, no relationship between AOP and SIDS has ...

  18. Adult hip dysplasia and osteoarthritis. Studies in radiology and clinical epidemiology

    DEFF Research Database (Denmark)

    Jacobsen, Steffen

    2006-01-01

    Osteoarthritis (OA) presupposes the interaction of systemic and/or local factors. In hip joint OA, congenital or developmental malformation is believed to constitute an individual risk factor for premature degeneration. Hip dysplasia (HD) is such a malformation. The radiological and epidemiological...... subjects. In a computerized tomographic study of severely dysplastic hips we found a close relationship between insufficient anterior, acetabular containment and proximal femoral anteversion. The primary area of degeneration in dysplastic hips was in the antero-lateral quadrant of the joint....

  19. The "pirate sign" in fibrous dysplasia.

    Science.gov (United States)

    Singnurkar, Amit; Rush, Chris

    2006-11-01

    Fibrous dysplasia commonly involves the skull in both its monostotic and polyostotic variants. We present two cases of fibrous dysplasia involving the sphenoid wing, which were strikingly similar in their bone scan appearance. Both patients demonstrated intense increased uptake of Tc-99m MDP in a pattern reminding us of a "pirate wearing an eyepatch." We propose that this characteristic appearance of fibrous dysplasia of the sphenoid wing be called the "pirate sign." A review of the literature revealed several other pathologic conditions that have been reported to involve the sphenoid bone and should be considered in the differential diagnosis of abnormal bone tracer uptake in this region.

  20. Technology of physical rehabilitation of children with bronchopulmonary diseases in the conditions of hospitalization

    Directory of Open Access Journals (Sweden)

    Nataliya Ivasyk

    2016-10-01

    Full Text Available Purpose: the development of technology of physical rehabilitation of children with bronchopulmonary diseases. Material & Methods: general scientific – analysis, conception and generalization, synthesis, comparison, abstraction. Results: technology of physical rehabilitation for children with bronchopulmonary diseases (BD, which is directed to the restoration and the development of physiological functions of a child, the prevention of synchronization of sharp processes, the elimination of negative changes in health of a child, the assistance in creation of conditions for adaptation to changes as a result of disease in life situations, is offered. Conclusions: the program and its activity including basic and variable components is the compound of the offered technology of physical rehabilitation for children with BD in the conditions of hospitalization.

  1. 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.

  2. Renal infarction complicating fibromuscular dysplasia.

    Science.gov (United States)

    Gavalas, M; Meisner, R; Labropoulos, N; Gasparis, A; Tassiopoulos, A

    2014-01-01

    Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease that most commonly affects the renal and extracranial carotid arteries. We present 3 cases of renal infarction complicating renal artery FMD in 42-, 43-, and 46-year-old females and provide a comprehensive review of the literature on this topic. In our patients, oral anticoagulation therapy was used to treat all cases of infarction, and percutaneous angioplasty was used nonemergently in one case to treat refractory hypertension. All patients remained stable at 1-year follow-up. This is consistent with outcomes in previously published reports where conservative medical management was comparable to surgical and interventional therapies. Demographic differences may also exist in patients with renal infarction and FMD. A higher prevalence of males and a younger age at presentation have been found in these patients when compared to the general population with FMD.

  3. Severity grading in radial dysplasia.

    Science.gov (United States)

    Vilkki, S K

    2014-11-01

    A functional scoring method to grade the usefulness and quality of the upper limbs in congenital radial dysplasia is presented. It is based on the author's examinations of 44 arms with congenital deficiency of the radius. The hand (H), wrist (W) and proximal parts (P) of the extremity are each scored from 0 to 10 points for severity. The scoring is expressed similarly to the TNM (tumour, nodes, metastasis) tumour classification, for example as H5W4P2. The maximum severity index is 30 points. A severity grade of mild is between 1 and 8 points, moderate between 9 and 16 points and severe 17 points and over. In the author's series, the grades were mild in eight, moderate in 21 and severe in 15 cases. The functional severity grading should allow better comparison of radially deficient limbs and the results of treatment between groups of patients. © The Author(s) 2014.

  4. 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.

  5. Thanatophoric dysplasia: Antenatal to postmortem

    Directory of Open Access Journals (Sweden)

    Chanabasappa V Chavadi

    2015-01-01

    Full Text Available Thanatophoric dwarfism (TD, literally meaning death seeking dwarf, is the most common form of lethal bone dysplasia characterized by severe micromelia, extra folds of skin and narrow chest. Other signs include small ribs, underdeveloped lungs, cloverleaf skull, hypertelorism and protuberant abdomen. A short neck, depressed nasal bridge and hypoplastic mandible may also be present. Hydrocephalus is uncommon but is another poor prognostic sign. The diagnosis is usually made with the ultrasonography in the second trimester. Based on the morphological characters, this condition is sub-divided into type 1 and type 2. Fetuses with this condition are either still-born or die shortly after birth. Antenatal sonographic, postpartum radiological and autopsy findings of a case of type 1 TD with a relatively uncommon association of hydrocephalus is discussed.

  6. 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.

  7. Anatomical arrangement of the lobar bronchi, broncho-pulmonary segments and their variations

    Directory of Open Access Journals (Sweden)

    Sathidevi V. K.

    2016-11-01

    Conclusions: In man there is an increase in the number of bronchial generations and alveoli after birth. An increase in their size was noted as well. Thus the study of variations in the pattern of bronchopulmonary segments aids the clinician very much. It is necessary for Thoracic Surgeons, Anaesthetists, Chest physicians, Radiologists, Ultrasonologists, Pathologists and Anatomists for investigative procedures, diagnosis and various treatment modalities including surgeries like segmentectomy. [Int J Res Med Sci 2016; 4(11.000: 4928-4932

  8. 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. .

  9. Prenatal Stress, Prematurity, and Asthma.

    Science.gov (United States)

    Medsker, Brock; Forno, Erick; Simhan, Hyagriv; Celedón, Juan C

    2015-12-01

    Asthma is the most common chronic disease of childhood, affecting millions of children in the United States and worldwide. Prematurity is a risk factor for asthma, and certain ethnic or racial minorities such as Puerto Ricans and non-Hispanic blacks are disproportionately affected by both prematurity and asthma. In this review, we examine current evidence to support maternal psychosocial stress as a putative link between prematurity and asthma, while also focusing on disruption of the hypothalamic-pituitary-adrenal (HPA) axis and immune responses as potential underlying mechanisms for stress-induced "premature asthma." Prenatal stress may cause not only abnormalities in the HPA axis but also epigenetic changes in the fetal glucocorticoid receptor gene (NR3C1), leading to impaired glucocorticoid metabolism. Moreover, maternal stress can alter fetal cytokine balance, favoring TH2 (allergic) immune responses characteristic of atopic asthma: interleukin 6 (IL-6), which has been associated with premature labor, can promote TH2 responses by stimulating production of IL-4 and IL-13. Given a link among stress, prematurity, and asthma, future research should include birth cohorts aimed at confirming and better characterizing "premature asthma." If confirmed, clinical trials of prenatal maternal stress reduction would be warranted to reduce the burden of these common comorbidities. While awaiting the results of such studies, sound policies to prevent domestic and community violence (eg, from firearms) are justified, not only by public safety but also by growing evidence of detrimental effects of violence-induced stress on psychiatric and somatic health.

  10. 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.)

  11. Hip dysplasia in the skeletally mature patient

    National Research Council Canada - National Science Library

    Goldstein, Rachel Y; Kaye, Ian David; Slover, James; Feldman, David

    2014-01-01

    .... These characteristic changes result in intraarticular pathology leading to hip arthritis. A variety of treatment options exist based on the degree of dysplasia and the amount of concomitant hip arthritis...

  12. Mandibuloacral Dysplasia in An Iranian Girl

    Directory of Open Access Journals (Sweden)

    F. Abbasi

    2006-07-01

    Full Text Available Mandibuloacral dysplasia (MAD is a rare autosomal recessive syndrome. Less than 25 families have been reported, most of which are Italian. Here, we describe a new patient of Iranian origin, born to consanguineous parents.

  13. Advances in the treatment of premature child syndrome CPAP respiratory distress%CPAP 对早产儿呼吸窘迫综合征的治疗进展

    Institute of Scientific and Technical Information of China (English)

    蒋道菊; 池永学

    2014-01-01

    in the era of gentle ventilation ,continuous positive airway pressure (cPaP) has been proven over the years to be an effective mode of noninvasive ventilatory support and as such has gained widespread use around the world in the management of a variety of neonatal respiratory diseases, particulary respiratory distress syndrome (rds).it is relatively cheap and easy to apply and certainly feasible for routine use in underdeveloped world.there is good to fair quality supportive evidence from several studies that the use of primary cPaP can reduce the need for intubation and mechanical ventilation in premature infants and could avoid ventilator induced lung injury and decrease bronchopulmonary dysplasia(BPd).cPaP has also been shown to be effective in the treatment of respiratory distress syndrome (rds) even in very low birth weight infants. in this review, we will attempt to describe different ways in which cPaP can be applied,other advantages of clinical indications for cPaP will also be explored. although it is unclear that primary use of cPaP can reduce overall neonatal mortality and morbidity ,it is becoming increasingly clear that early cPaP use is less invasive baby friendly and decreases the need and frequency of the use of surfactants.%在提倡减少肺损伤的时代,持续气道正压通气(continuous positive airway pressure ,cPaP)作为无创通气技术近年来已经被证实有效,因此,其对新生儿呼吸系统疾病,尤其是新生儿呼吸窘迫综合征(respiratory distress syndrome ,rds)的治疗在全世界范围得到推广。此外,cPaP 便宜、操作简便、易于在发展中国家实施。有研究证实 cPaP可以减少早产儿的气管插管及机械通气,故可避免呼吸机相关性肺损伤及降低支气管肺发育不良(bronchopulmonary dysplasia,BPd),且证实其甚至对极低体重出生儿 rds 有效。本文就 cPaP 对早产儿 rds 的不同应用方式做一简单综述, cPaP 的其它优点

  14. CONGENITAL RADIAL DYSPLASIA: A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Venkatram Reddy

    2015-08-01

    Full Text Available Congenital radial dysplasia, also referred to as radial club hand , means deficiency along the preaxial or radial side of the extremity. It ranges from hypoplasia of the thumb to variou s degrees of radial hypoplasia. We present one such rare case of type 4 congenital unilateral isolated radial dysplasia with carpel anomaly , reported to our department in SVS medical C ollege, Mahabubanagar, Telangana state

  15. Bilateral anophthalmia with septo-optic dysplasia

    Directory of Open Access Journals (Sweden)

    Manisha Jana

    2010-01-01

    Full Text Available Bilateral anophthalmia is a rare entity and association with septo-optic dysplasia is an even rare condition. The condition is characterized by absent eyeballs in the presence of eyelids, conjunctiva or lacrimal apparatus. Though anophthalmia can be diagnosed clinically, imaging plays a crucial role in delineating the associated anomalies. In addition, often clinical anophthalmia may prove to be severe microphthalmia on imaging. We describe the imaging findings in an infant with bilateral anophthalmia and septo-optic dysplasia.

  16. 103例超未成熟儿及超低出生体重儿转归及影响因素分析%Outcome and influencing factors of 103 cases of extremely premature infant and extremely low birth weight infants

    Institute of Scientific and Technical Information of China (English)

    王晓磊; 梅花; 刘春枝; 张亚昱; 刘春丽; 宋丹; 张钰恒

    2016-01-01

    目的 总结超未成熟儿及超低出生体重儿发生的原因、相关影响因素及转归情况.方法 分析内蒙古医科大学附属医院2009年1月至2015年12月NICU收治的符合条件的103例超低出生体重儿及超未成熟儿的临床资料,包括母孕期病史、新生儿出生时情况、诊治经过及预后.结果 103例患儿存活67例,死亡36例,存活率65.0% (67/103).妊娠高血压综合征、感染、胎膜早破等为发生超未成熟和超低出生体重儿的主要因素,影响两者的转归因素包括胎龄、性别、出生体重、肺出血、支气管肺发育不良、坏死性小肠结肠炎(P<0.05).长期住院的存活患儿后期易并发贫血.死亡直接原因前4位包括肺出血、呼吸窘迫综合征、新生儿肺炎及坏死性小肠结肠炎.结论 加强高危妊娠监测管理,预防早产,加强NICU超未成熟儿及超低出生体重儿的监护管理,及早发现、及早处理各种并发症,防止医院感染发生,是提高两者存活率,改善生活质量的根本措施.%Objective To summarize the causes,related factors and outcome of extremely premature infants and extremely low birth weight infants.Methods One hundred and three cases of extremely premature infants and extremely low birth weight infants were admired to First Affiliated Hospital of Inner Mongolia Medical University between January 2009 and December 2015.The study was performed to analyze the clinical data of the 103 cases,included history of pregnancy,birth situation,treatment and prognosis.Results In these 103 cases,67 infants survived,36 infants died.The survival rate was 65.0% (67/103).The extremely premature infants and extremely low birth weight infants were mainly associated with pregnancy-induced hypertension,infection,premature rupture of membranes.Factors that could affect the outcome of these cases included gestational age,sex,birth weight,pulmonary hemorrhage,bronchopulmonary dysplasia and necrotizing

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

    Science.gov (United States)

    Groza, Tudor; Hunter, Jane; Zankl, Andreas

    2012-03-26

    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. 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. 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.

  18. Premature ovarian failure

    Directory of Open Access Journals (Sweden)

    Vujović Svetlana

    2012-01-01

    Full Text Available Premature ovarian failure (POF is the occurrence of hypergonadotropic hypoestrogenic amenorrhea in women under the age of forty years. It is idiopathic in 74-90% patients. Known cases can be divided into primary and secondary POF. In primary POF genetic aberrations can involve the X chromosome (monosomy, trisomy, translocations, deletions or autosomes. Genetic mechanisms include reduced gene dosage and non-specific chromosome effects impairing meiosis, decreasing the pool of primordial follicles and increasing atresia due to apoptosis or failure of follicle maturation. Autoimmune ovarian damage is caused by alteration of T-cell subsets and T-cell mediated injury, increase of autoantibody producing B-cells, a low number of effector/cytotoxic lymphocyte, which decreases the number and activity of natural killer cells. Bilateral oophorectomy, chemotherapy, radiotherapy and infections cause the secondary POF. Symptoms of POF include irritability, nervousness, loss of libido, depression, lack of concentration, hot flushes, weight gaining, dry skin, vaginal dryness, frequent infections etc. The diagnosis is confirmed by the level of FSH of over 40 IU/L and estradiol below 50 pmol/L in women aged below 40 years. Biochemical and other hormonal analysis (free thyroxin, TSH, prolactin, testosterone, karyotype (<30 years of age, ultrasound of the breasts and pelvis are advisable. Optimal therapy is combined estrogen progestagen therapy given in a sequential rhythm, after excluding absolute contraindications. Testosterone can be added to adnexectomized women and those with a low libido. Sequential estrogen progestagen replacement therapy is the first line therapy for ovulation induction in those looking for pregnancy and after that oocyte donation will be advised. Appropriate estro-progestagen therapy improves the quality of life and prevents complications such as cardiovascular diseases, osteoporosis, stroke etc.

  19. Hip dysplasia in the young adult caused by residual childhood and adolescent-onset dysplasia.

    Science.gov (United States)

    Pun, Stephanie

    2016-12-01

    Hip dysplasia is a treatable developmental disorder that presents early in life but if neglected can lead to chronic disability due to pain, decreased function, and early osteoarthritis. The main causes of hip dysplasia in the young adult are residual childhood developmental dysplasia of the hip (DDH) and adolescent-onset acetabular dysplasia. These two distinct disease processes affect the growing hip during different times of development but result in a similar deformity and pathomechanism of hip degeneration. Routine screening for DDH and counseling regarding risks for acetabular dysplasia in families with a history of early hip osteoarthritis may allow early identification and intervention in these hips with anatomical risk factors for joint degeneration.

  20. Preterm labor and premature birth: Are you at risk?

    Science.gov (United States)

    ... labor and premature birth: Are you at risk? Preterm labor and premature birth: Are you at risk? ... for preterm labor and premature birth. What are preterm labor and premature birth? Preterm and premature mean ...

  1. Expansive focal cemento-osseous dysplasia.

    Science.gov (United States)

    Bulut, Emel Uzun; Acikgoz, Aydan; Ozan, Bora; Zengin, Ayse Zeynep; Gunhan, Omer

    2012-01-01

    To present a case of expansive focal cemento-osseous dysplasia and emphasize the importance of differential diagnosis. Cemento-osseous dysplasia is categorized into three subtypes on the basis of the clinical and radiographic features: Periapical, focal and florid. The focal type exhibits a single site of involvement in any tooth-bearing or edentulous area of the jaws. These lesions are usually asymptomatic; therefore, they are frequently diagnosed incidentally during routine radiographic examinations. Lesions are usually benign, show limited growth, and do not require further surgical intervention, but periodic follow-up is recommended because occasionally, this type of dysplasia progresses into florid osseous dysplasia and simple bone cysts are formed. A 24-year-old female patient was referred to our clinic for swelling in the left edentulous mandibular premolarmolar region and felt discomfort when she wore her prosthetics. She had no pain, tenderness or paresthesia. Clinical examination showed that the swelling in the posterior mandible that was firm, nonfluctuant and covered by normal mucosa. On panoramic radiography and computed tomography, a well defined lesion of approximately 1.5 cm in diameter of mixed density was observed. The swelling increased slightly in size over 2 years making it difficult to use prosthetics and, therefore, the lesion was totally excised under local anesthesia, and surgical specimens were submitted for histopathological examination. The histopathological diagnosis was focal cemento-osseous dysplasia. In the present case, because of the increasing size of the swelling making it difficult to use prosthetics, young age of the patient and localization of the lesion, in the initial examination, cemento-ossifying fibroma was suspected, and the lesion was excised surgically; the histopathological diagnosis confirmed it as focal cemento-osseous dysplasia. We present a case of expansive focal cemento-osseous dysplasia. Differential diagnosis

  2. 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.

  3. Cervical spinal monostotic fibrous dysplasia: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ok Hwa [Dept. of Radiology, Haeundae Paik Hospital, Inje University College of Medicine, Busan (Korea, Republic of)

    2013-09-15

    Monostotic fibrous dysplasia of the cervical vertebra is quite unusual. The author reports a case of monostotic fibrous dysplasia affecting the second cervical vertebra with descriptions from the CT, MR and bone scanning findings.

  4. Fibrous dysplasia of bone: a clinicopathologic review

    Directory of Open Access Journals (Sweden)

    Mohan H

    2011-11-01

    Full Text Available Harsh Mohan1, Preeti Mittal1, Irneet Mundi1, Sudhir Kumar21Department of Pathology, 2Department of Orthopedics, Government Medical College, Sector 32, Chandigarh, IndiaAbstract: Fibrous dysplasia of the bones is an uncommon congenital skeletal disorder that is found equally in both genders and is not inherited. Its etiology has been linked to an activating mutation of Gsα and the downstream effects of the resultant increase in cAMP. Fibrous dysplasia is categorized as either monostotic or polyostotic, and may occur as a component of McCune-Albright syndrome or the rare Mazabraud syndrome. Long bones, skull bones, and ribs are the most commonly affected bones. The radiological picture is somewhat variable, including a ground-glass appearance, expansion of the bone, and sclerosis surrounding the lesion. Histologically, fibrous dysplasia shows irregularly-shaped trabeculae of immature, woven bone in a background of variably cellular, loosely arranged fibrous stroma. It may be complicated by pathologic fracture, and rarely by malignant transformation. This review examines interesting issues surrounding the etiology of fibrous dysplasia, its clinical and laboratory manifestations, radiological picture, utility of bone biopsy, gross and microscopic pathology, complications, and its differential diagnostic considerations.Keywords: fibrous dysplasia, McCune-Albright syndrome, monostotic form, polyostotic form

  5. Etiology and pathogenesis of ectodermal dysplasias.

    Science.gov (United States)

    Itin, Peter H

    2014-10-01

    Ectodermal dysplasias are a large group of heterogeneous heritable conditions characterized by congenital defects of one or more ectodermal structures and their appendages. The skin and its appendages are mainly composed by ectodermal components but development initiation of appendages is orchestrated by signals of the mesoderm with the help of placodes. A complex network of signaling pathways coordinates the formation and function of ectodermal structures. In recent years much has been discovered regarding the molecular mechanisms of ectodermal embryogenesis and this facilitates a rational basis for classification of ectodermal dysplasia. Interestingly, not only complex ectodermal syndromes but also mono- or oligosymptomatic ectodermal malformations may result from a mutation in a gene that is critical for ectodermal development. Mesodermal, and occasionally endodermal malformations may coexist. Embryogenesis occurs in distinct tissue organizational fields and specific interactions among the germ layers exist that may lead to a wide range of ectodermal dysplasias. Of the approximately 200 different ectodermal dysplasias, about 80 have been characterized at the molecular level with identification of the genes that are mutated in these disorders. Modern molecular genetics will increasingly elucidate the basic defects of these distinct syndromes and shed more light into the regulatory mechanisms of embryology. The upcoming classification of ectodermal dysplasias will combine detailed clinical and molecular knowledge.

  6. Hip arthroscopy in the setting of hip dysplasia

    OpenAIRE

    Yeung, M; Kowalczuk, M.; Simunovic, N.; Ayeni, O. R.

    2016-01-01

    Objective Hip arthroscopy in the setting of hip dysplasia is controversial in the orthopaedic community, as the outcome literature has been variable and inconclusive. We hypothesise that outcomes of hip arthroscopy may be diminished in the setting of hip dysplasia, but outcomes may be acceptable in milder or borderline cases of hip dysplasia. Methods A systematic search was performed in duplicate for studies investigating the outcome of hip arthroscopy in the setting of hip dysplasia up to Ju...

  7. BRONCHOPULMONARY LESIONS AND QUALITY OF LIFE IN PATIENTS WITH RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    M. V. Sheyanov

    2014-07-01

    Full Text Available Aim – assessment of quality of life (QoL in patients with rheumatoid arthritis (RA with the presence and severity of bronchopulmonary lesions.Materials and methods. The study included 104 patients with RA and 100 patients not suffering from RA and verified chronic respiratory diseases. The analysis of the QOL of patients using questionnaires EQ-5D (EuroQoL Group, 1990 and SGRQ (St. George's Hospital questionnaire to assess respiratory function, performed spirometry, bodyplethysmography , pulse oximetry, the definition of lung diffusioncapacity, multispiral computed tomography of the lungs.Results. Performance of all scales and the resulting indices of questionnaires EQ-5D and SGRQ showed a significant decrease in QoL ofRA patients compared with those in control group and the general population. A correlation index of EQ-5D with vital capacity (r = 0.47;p < 0.001 and diffusion capacity (r = 0.67; p < 0.01 of the lungs is revealed. The main reason for the reduction of indices of the questionnaire SGRQ in patients with RA was the presence of shortness of breath. The multi-factorial origin of dyspnea in patients with RA with theessential role of bronchopulmonary lesions was established.Conclusion. Bronchopulmonary lesions in the underlying disease have an adverse impact on the QOL of patients with RA. Promising directions for improving the QOL of RA patients with bronchial lesions can be considered for activities for the conservation of respiratory lung function, exercise control RA activity, elimination of anemia correction of psycho-emotional disturbances of anxiety-depressive character.

  8. BRONCHOPULMONARY LESIONS AND QUALITY OF LIFE IN PATIENTS WITH RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    M. V. Sheyanov

    2011-01-01

    Full Text Available Aim – assessment of quality of life (QoL in patients with rheumatoid arthritis (RA with the presence and severity of bronchopulmonary lesions.Materials and methods. The study included 104 patients with RA and 100 patients not suffering from RA and verified chronic respiratory diseases. The analysis of the QOL of patients using questionnaires EQ-5D (EuroQoL Group, 1990 and SGRQ (St. George's Hospital questionnaire to assess respiratory function, performed spirometry, bodyplethysmography , pulse oximetry, the definition of lung diffusioncapacity, multispiral computed tomography of the lungs.Results. Performance of all scales and the resulting indices of questionnaires EQ-5D and SGRQ showed a significant decrease in QoL ofRA patients compared with those in control group and the general population. A correlation index of EQ-5D with vital capacity (r = 0.47;p < 0.001 and diffusion capacity (r = 0.67; p < 0.01 of the lungs is revealed. The main reason for the reduction of indices of the questionnaire SGRQ in patients with RA was the presence of shortness of breath. The multi-factorial origin of dyspnea in patients with RA with theessential role of bronchopulmonary lesions was established.Conclusion. Bronchopulmonary lesions in the underlying disease have an adverse impact on the QOL of patients with RA. Promising directions for improving the QOL of RA patients with bronchial lesions can be considered for activities for the conservation of respiratory lung function, exercise control RA activity, elimination of anemia correction of psycho-emotional disturbances of anxiety-depressive character.

  9. 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.

  10. 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. N...

  11. Fibrous Dysplasia Characterization Using Lacunarity Analysis.

    Science.gov (United States)

    Cordeiro, Mirna S; Backes, André R; Júnior, Antônio F Durighetto; Gonçalves, Elmar H G; de Oliveira, Jefferson X

    2016-02-01

    Fibrous dysplasia (FD) is a developmental anomaly in which the normal medullary space of the affected bone is replaced by fibro-osseous tissue. This condition is typically encountered in adolescents and young adults. It affects the maxillofacial region and it can often cause severe deformity and asymmetry. Therefore, accurate diagnosis is critical to determine the appropriate treatment of each case. In this sense, computed tomography (CT) is a relevant resource among the imaging techniques for correct diagnosis of this condition. Thus, in this paper, we propose to analyze fibrous dysplasia through its texture pattern. To accomplish this task, we propose to use lacunarity analysis, a multiscale method for describing patterns of spatial dispersion. Results indicated lower lacunarity values for fibrous dysplasia in comparison to normal bone samples, an indication that their texture images are more homogeneous, and a high separability between the classes when using principal component analysis (PCA) and decision trees for statistical analysis.

  12. Surgical Treatment of Congenital Mitral Valve Dysplasia.

    Science.gov (United States)

    Vida, Vladimiro L; Carrozzini, Massimiliano; Padalino, Massimo; Milanesi, Ornella; Stellin, Giovanni

    2016-05-01

    Congenital mitral valve (MV) dysplasia is a relatively rare and highly complex cardiac disease. We present our results and illustrate the techniques used to repair these valves. Between 1972 and 2014, 100 consecutive patients underwent surgical repair of congenital MV dysplasia at our institution. Predominant MV regurgitation was present in 53 patients (53%) whereas mitral stenosis was prevalent in 47 (47%). There were five early (5%) and eight late deaths (9%). Actuarial survival was 95%, 94%, and 93% at 5, 10, and 20 years, respectively. Sixteen patients (18%) required reintervention due to subsequent MV dysfunction. Actuarial freedom from reintervention for MV dysfunction was 95%, 92%, and 89% at 5, 10, and 20 years, respectively. The mechanism underlying the valve dysfunction in congenital mitral valve dysplasia is multifactorial and requires the application of a variety of surgical techniques for repair. doi: 10.1111/jocs.12743 (J Card Surg 2016;31:352-356). © 2016 Wiley Periodicals, Inc.

  13. Dysplasia epiphysealis hemimelica: A case report

    Directory of Open Access Journals (Sweden)

    Jovanović Dalibor V.

    2014-01-01

    Full Text Available Introduction. Dysplasia epiphysealis hemimelica, also known as Trevor’s disease, is an extremely rare skeletal developmental disorder of unknown etiology, characterized by an osteocartilaginous outgrowth of one or more epiphyses or of a tarsal bone during childhood. Case report. We presented a sporadic case of dysplasia epiphysealis hemimelica developed in the talus. A 6- year-old boy complained of swelling of his right ankle joint, with painful and reduced movements. Radiographies suggested excessive overgrowth of the dorsomedial aspect of the talus. The tumor was surgically excised and the gross and histological findings were consistent with those of osteochondroma. Conclusion. Dysplasia epiphysealis hemimelica is diagnosed by clinical, radiographic and histopathological examination. Early diagnosis is important for the condition to be treated before the deformity becomes disabling.

  14. 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.

  15. Rasmussen's encephalitis presenting as focal cortical dysplasia

    Directory of Open Access Journals (Sweden)

    D.J. O'Rourke

    2014-01-01

    Full Text Available Rasmussen's encephalitis is a rare syndrome characterized by intractable seizures, often associated with epilepsia partialis continua and symptoms of progressive hemispheric dysfunction. Seizures are usually the hallmark of presentation, but antiepileptic drug treatment fails in most patients and is ineffective against epilepsia partialis continua, which often requires surgical intervention. Co-occurrence of focal cortical dysplasia has only rarely been described and may have implications regarding pathophysiology and management. We describe a rare case of dual pathology of Rasmussen's encephalitis presenting as a focal cortical dysplasia (FCD and discuss the literature on this topic.

  16. Cleido cranial dysplasia: report of a family.

    Science.gov (United States)

    González López, Blanca Silvia; Ortiz Solalinde, Clara; Kubodera Ito, Toshio; Lara Carrillo, Edith; Ortiz Solalinde, Estela

    2004-12-01

    A family case of Cleidocranial Dysplasia is presented. A mother and two adolescent girls were examined. In all three cases, a radiological series was performed over the entire body. Generalized dysplasia in bones, prolonged retention of primary teeth, and delayed eruption of permanent, as well as supernumerary teeth was diagnosed. The citogenetic study with GTG band showed normal 46, XX. Bilateral audiometry in the mother demonstrated a mild to moderate hypoacustic condition. Radiological findings are presented and the importance of early diagnosis is discussed.

  17. Sponastrime dysplasia. A radiologic-pathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Lachman, R.S.; Stoss, H.; Spranger, J.

    1989-07-01

    The 2nd family with Sponastrime Dysplasia is described. The clinical, radiologic and chondro-osseous morphology of boy and girl siblings are presented. The facial appearance is an 'oriental look' with midface hypoplasia and a saddle nose. The radiological findings include the spinal changes of lordosis, osteoporosis and pear-shaped vertebrae, as well as striated metaphyses (osteopathia striata). The morphological findings suggest a disturbance in the formation of cartilage, with a defect in collagen and proteoglycans synthesis in this rare autosomal recessive skeletal dysplasia. (orig.).

  18. 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

  19. Occipital projections in the skeletal dysplasias

    Energy Technology Data Exchange (ETDEWEB)

    Takamine, Yuji; Field, Fiona M. [International Skeletal Dysplasia Registry, Department of Pediatrics and the Medical Genetics-Birth Defects Center, Cedars-Sinai Medical Center, Los Angeles, CA (United States); Lachman, Ralph S. [International Skeletal Dysplasia Registry, Department of Pediatrics and the Medical Genetics-Birth Defects Center, Cedars-Sinai Medical Center, Los Angeles, CA (United States); Department of Radiological Sciences and Pediatrics, David Geffen School of Medicine, Los Angeles, CA (United States); Rimoin, David L. [International Skeletal Dysplasia Registry, Department of Pediatrics and the Medical Genetics-Birth Defects Center, Cedars-Sinai Medical Center, Los Angeles, CA (United States); Departments of Pediatrics, Medicine and Human Genetics, David Geffen School of Medicine at UCLA, Los Angeles, CA (United States)

    2004-07-01

    Occipital projections of the cranium have been reported in a number of skeletal dysplasias and syndromes. We observed two cases of atelosteogenesis type I with a bony occipital projection. This finding has neither been noted nor reported in any form of atelosteogenesis. This led us to search the International Skeletal Dysplasia Registry for occipital projections, and we found them in four other syndromes in which they had not been reported. Thus occipital spurs are a non-diagnostic feature that can be found in at least ten distinct disorders as well as a normal variant. (orig.)

  20. [Congenital hip dysplasia, screening and therapy].

    Science.gov (United States)

    Kolb, A; Windhager, R; Chiari, C

    2015-11-01

    Congenital hip dysplasia and hip dislocation are relatively common pathological conditions of the musculoskeletal system in infants. An early and certain diagnosis can now be achieved by sonographic hip screening within the framework of screening examination programs. This early diagnostic procedure in infants is essential particularly for a conservative treatment strategy. Therefore, apart from possessing in-depth knowledge, training of the examiner in specialist courses is of central importance. This article presents an overview of the entity of congenital hip dysplasia and hip dislocation, the diagnostics and treatment with special emphasis on recent developments.

  1. Turner's syndrome, fibromuscular dysplasia, and stroke.

    Science.gov (United States)

    Lancman, M; Mesropian, H; Serra, P; Granillo, R

    1991-02-01

    We report a 43-year-old woman who presented with a right frontoparietotemporal ischemic stroke. She had been diagnosed with Turner's syndrome during childhood and had a history of chronic estrogen therapy. Cerebral angiography showed lesions characteristics of fibromuscular dysplasia involving the right internal carotid and right vertebral arteries. We are not aware of any previous reports describing an association between fibromuscular dysplasia and Turner's syndrome. Although chronic estrogen therapy cannot be ruled out as a cause of this patient's stroke, we suggest a possible etiologic relation between these two entities.

  2. Clinical and radiographic delineation of Bent Bone Dysplasia-FGFR2 type or Bent Bone Dysplasia with Distinctive Clavicles and Angel-shaped Phalanges.

    Science.gov (United States)

    Krakow, Deborah; Cohn, Daniel H; Wilcox, William R; Noh, Grace J; Raffel, Leslie J; Sarukhanov, Anna; Ivanova, Margarita H; Danielpour, Moise; Grange, Dorothy K; Elliott, Alison M; Bernstein, Jonathan A; Rimoin, David L; Merrill, Amy E; Lachman, Ralph S

    2016-10-01

    Bent Bone Dysplasia-FGFR2 type is a relatively recently described bent bone phenotype with diagnostic clinical, radiographic, and molecular characteristics. Here we report on 11 individuals, including the original four patients plus seven new individuals with three longer-term survivors. The prenatal phenotype included stillbirth, bending of the femora, and a high incidence of polyhydramnios, prematurity, and perinatal death in three of 11 patients in the series. The survivors presented with characteristic radiographic findings that were observed among those with lethality, including bent bones, distinctive (moustache-shaped) small clavicles, angel-shaped metacarpals and phalanges, poor mineralization of the calvarium, and craniosynostosis. Craniofacial abnormalities, hirsutism, hepatic abnormalities, and genitourinary abnormalities were noted as well. Longer-term survivors all needed ventilator support. Heterozygosity for mutations in the gene that encodes Fibroblast Growth Factor Receptor 2 (FGFR2) was identified in the nine individuals with available DNA. Description of these patients expands the prenatal and postnatal findings of Bent Bone Dysplasia-FGFR2 type and adds to the phenotypic spectrum among all FGFR2 disorders. © 2016 Wiley Periodicals, Inc.

  3. 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

  4. 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.

  5. Your Premature Baby: Low Birthweight

    Science.gov (United States)

    ... many low-birthweight babies are born prematurely, many risk factors for having a low-birthweight baby are the same for preterm labor and ... risk for having a low-birthweight baby. Medical risk factors for having a low-birthweight baby Preterm labor . This is labor that starts ...

  6. Music Therapy with Premature Infants

    Science.gov (United States)

    Standley, Jayne

    2003-01-01

    Over 20 years of research and clinical practice in music therapy with premature infants has been compiled into this text designed for Board Certified Music Therapists specializing in Neonatal Intensive Care clinical services, for NICU medical staff incorporating research-based music therapy into developmental care plans, and for parents of…

  7. Music Therapy with Premature Infants

    Science.gov (United States)

    Standley, Jayne

    2003-01-01

    Over 20 years of research and clinical practice in music therapy with premature infants has been compiled into this text designed for Board Certified Music Therapists specializing in Neonatal Intensive Care clinical services, for NICU medical staff incorporating research-based music therapy into developmental care plans, and for parents of…

  8. [Relationship between congenital heart disease and bronchial dysplasia].

    Science.gov (United States)

    Zeng, Shuang-Lin; Li, Ya-Jun; Huang, Ting; Tan, Li-Hua; Mei, Xi-Long; Sun, Jian-Ning

    2011-11-01

    To study the relationship of the incidence of bronchial dysplasia (bronchial anomalous origin and bronchial stenosis) with congenital heart disease. A total of 185 children with congenital heart disease or bronchial dysplasia were enrolled. Bronchial dysplasia was identified by the 64-MSCT conventional scanning or thin slice scanning with three-dimensional reconstruction. Forty-five children (25.3%) had coexisting bronchial dysplasia and congenital heart disease. The incidence rate of bronchial dysplasia in children with congenital heart disease associated with ventricular septal defect was higher than in those without ventricular septal defect (33.7% vs 15.0%; Pdysplasia between the children with congenital heart disease who had a large vascular malformation and who did not. Bronchial dysplasia often occurs in children with congenital heart disease. It is necessary to perform a tracheobronchial CT scanning with three-dimensional reconstruction to identify tracheobronchial dysplasia in children with congenital heart disease, especially associated with ventricular septal defect.

  9. Less Stress : Oxidative stress and glutathione kinetics in preterm infants

    NARCIS (Netherlands)

    D. Rook (Denise)

    2013-01-01

    textabstractDue to immature antioxidant defenses, preterm infants are at susceptible to oxidative stress, which is associated with bronchopulmonary dysplasia, retinopathy of prematurity and periventricular leukomalacia. The general aim of this thesis was to study oxidative stress in preterm infants

  10. Less Stress : Oxidative stress and glutathione kinetics in preterm infants

    NARCIS (Netherlands)

    D. Rook (Denise)

    2013-01-01

    textabstractDue to immature antioxidant defenses, preterm infants are at susceptible to oxidative stress, which is associated with bronchopulmonary dysplasia, retinopathy of prematurity and periventricular leukomalacia. The general aim of this thesis was to study oxidative stress in preterm infants

  11. Rapid test for lung maturity, based on spectroscopy of gastric aspirate, predicted respiratory distress syndrome with high sensitivity

    DEFF Research Database (Denmark)

    Verder, Henrik; Heiring, Christian; Clark, Howard

    2017-01-01

    AIM: Respiratory distress syndrome (RDS) is a major cause of mortality and morbidity in premature infants. By the time symptoms appear, it may already be too late to prevent a severe course, with bronchopulmonary dysplasia or mortality. We aimed to develop a rapid test of lung maturity for target...

  12. Optic nerve decompression for orbitofrontal fibrous dysplasia.

    Science.gov (United States)

    Abe, Takumi; Sato, Kaneshige; Otsuka, Takaharu; Kawamura, Noriyoshi; Shimazu, Motohiko; Izumiyama, Hitoshi; Matsumoto, Kiyoshi

    2002-08-01

    Orbitofrontal fibrous dysplasia often involves the bony orbit and the optic canal. Although fibrous dysplasia reportedly produces compression of the optic nerve leading to visual distrubances, optic nerve decompression in patients without clinical signs of optic neuropathy is still controversial. We describe two patients with orbitofrontal fibrous dysplasia without signs of visual disturbance and one patient with McCune-Albright syndrome and progressive visual impairment. Optic nerve decompression was performed prophylactically for two patients and therapeutically for one patient through the transcranial extradural route. Dystopias and craniofacial deformities induced by fibrous dysplasia also were corrected. The micropressure suction-irrigation system was especially effective for decreasing heat transfer and thereby preventing thermal injury of the optic nerve. The orbitofrontal area was reconstructed from cranial bone, iliac bone, and ribs. Postoperative follow-up revealed no disturbances in visual function and no evidence of cerebrospinal fluid leakage. These findings suggest that optic nerve decompression may be effective in preventing visual disturbances with minimal risk of other neurological sequelae. Subsequent orbital reconstruction yielded satisfactory cosmetic results.

  13. Prophylactic intramedullary nailing in monostotic fibrous dysplasia.

    Science.gov (United States)

    Demiralp, Bahtiyar; Ozturk, Cagatay; Ozturan, Kutay; Sanisoglu, Yavuz S; Cicek, Ilker E; Erler, Kaan

    2008-06-01

    Fibrous dysplasia of bone is an enigma with no known cure. Treatment currently consists of curettage and bone-grafting in an attempt to eradicate the lesion and to prevent progressive deformity. This study presents the results of prophylactic intramedullary nailing in 10 patients with monostotic fibrous dysplasia, pain increasing with movement, and scintigraphically established activity. Ten patients with monostotic fibrous dysplasia in their upper or lower extremities treated between 2001 and 2003 were included in the study. Seven patients were male and 3 were female; their mean age was 26.9 years. The mean duration of follow-up was 33.5 months. Closed intramedullary nail without reaming was used in all cases. Bone grafting was not performed. Patients were allowed full weight bearing on the affected extremities on the second postoperative day. Mean VAS for functional pain was 5.33 +/- 0.65 preoperatively and 2.26 +/- 0.57 at final follow-up (p < 0.05). Radiographs showed no changes in lesion size, and the intramedullary fixation appeared to be stable. Prophylactic intramedullary nailing appeared to be beneficial in monostotic fibrous dysplasia with scintigraphically proven activity and functional pain. It also avoids problems that may occur following pathological fracture.

  14. The other hip in unilateral hip dysplasia

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Rømer, Lone; Søballe, Kjeld

    2006-01-01

    We reviewed transverse pelvic computed tomography scans of 197 consecutively referred adult patients with hip pain thought to be secondary to developmental dysplasia. A center-edge angle of 20 degrees or less was considered the upper normal value. Four groups were identified: 69 patients with app...

  15. 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.

  16. 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

  17. Craniofacial fibrous dysplasia: A 10-case series.

    Science.gov (United States)

    Couturier, A; Aumaître, O; Gilain, L; Jean, B; Mom, T; André, M

    2017-09-01

    Fibrous dysplasia of bone is a rare sporadic benign congenital condition in which normal cancellous bone is replaced by fibro-osseous tissue with immature osteogenesis. Sarcomatous transformation is exceptional. Lesions may involve one bone (monostotic) or several (polyostotic). Fibrous dysplasia may be associated with café-au-lait skin macules and endocrinopathy in McCune-Albright syndrome, or with myxoma in Mazabraud's syndrome. We report ten cases of patients followed up for craniofacial fibrous dysplasia in our center between 2010 and 2015. Mean age was 43 years (range, 10-72 years). Clinical symptoms comprised headache (n=3) and sensorineural disorder: recurrent anterior uveitis (n=1), visual acuity loss, epiphora and vestibular syndrome (n=1), and hearing loss (n=1). All cases were monostotic. The sphenoid bone was most commonly involved (n=5), followed by the ethmoid (n=1), frontal (n=1), fronto-ethmoid (n=1), temporal (n=1) and fronto-ethmoido-sphenoid (n=1) bones. Five patients were treated with intravenous pamidronate, a bisphosphonate: evolution was favorable for 3 of them at 1-6 months after treatment initiation, with resolution of headache or vestibular syndrome; the other 2 patients were stable. Two patients were operated on. Diagnosis of craniofacial fibrous dysplasia should be considered in case of headache, neuralgia, sensory disorder, functional disorder or infectious ENT complications. A medico-surgical approach is useful for these patients. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  18. MODERN PRODUCTS FOR FEEDING PREMATURE BABIES

    National Research Council Canada - National Science Library

    A. V. Surzhik

    2012-01-01

    .... Adequate feeding is one of the fundamental factors of premature babies nursing. To ensure a premature baby with all necessary components for power saving in breast milk intake, breast milk fortifiers...

  19. Comparative analyses of lung transcriptomes in patients with alveolar capillary dysplasia with misalignment of pulmonary veins and in foxf1 heterozygous knockout mice.

    Directory of Open Access Journals (Sweden)

    Partha Sen

    Full Text Available Alveolar Capillary Dysplasia with Misalignment of Pulmonary Veins (ACDMPV is a developmental disorder of the lungs, primarily affecting their vasculature. FOXF1 haploinsufficiency due to heterozygous genomic deletions and point mutations have been reported in most patients with ACDMPV. The majority of mice with heterozygous loss-of-function of Foxf1 exhibit neonatal lethality with evidence of pulmonary hemorrhage in some of them. By comparing transcriptomes of human ACDMPV lungs with control lungs using expression arrays, we found that several genes and pathways involved in lung development, angiogenesis, and in pulmonary hypertension development, were deregulated. Similar transcriptional changes were found in lungs of the postnatal day 0.5 Foxf1+/- mice when compared to their wildtype littermate controls; 14 genes, COL15A1, COL18A1, COL6A2, ESM1, FSCN1, GRINA, IGFBP3, IL1B, MALL, NOS3, RASL11B, MATN2, PRKCDBP, and SIRPA, were found common to both ACDMPV and Foxf1 heterozygous lungs. Our results advance knowledge toward understanding of the molecular mechanism of ACDMPV, lung development, and its vasculature pathology. These data may also be useful for understanding etiologies of other lung disorders, e.g. pulmonary hypertension, bronchopulmonary dysplasia, or cancer.

  20. The impact of vitamin D on fetal and neonatal lung maturation

    DEFF Research Database (Denmark)

    Lykkedegn, Sine; Sorensen, Grith Lykke; Beck-Nielsen, Signe Sparre

    2015-01-01

    Med, Embase and Cochrane databases with the terms vitamin D AND (surfactant OR lung maturation OR lung development OR respiratory distress syndrome OR fetal lung OR prematurity OR bronchopulmonary dysplasia). Three human studies, ten animal studies, two laboratory studies and one combined animal......Respiratory distress syndrome (RDS) and bronchopulmonary dysplasia (BPD) are major complications to preterm birth. Hypovitaminosis D is prevalent in pregnancy. We systematically reviewed the evidence of the impact of vitamin D on lung development, surfactant synthesis, RDS and BPD searching Pub...

  1. Outcomes for Extremely Premature Infants

    Science.gov (United States)

    Glass, Hannah C.; Costarino, Andrew T.; Stayer, Stephen A.; Brett, Claire; Cladis, Franklyn; Davis, Peter J.

    2015-01-01

    Premature birth is a significant cause of infant and child morbidity and mortality. In the United States, the premature birth rate, which had steadily increased during the 1990s and early 2000s, has decreased annually for four years and is now approximately 11.5%. Human viability, defined as gestational age at which the chance of survival is 50%, is currently approximately 23–24 weeks in developed countries. Infant girls, on average, have better outcomes than infant boys. A relatively uncomplicated course in the intensive care nursery for an extremely premature infant results in a discharge date close to the prenatal EDC. Despite technological advances and efforts of child health experts during the last generation, the extremely premature infant (less than 28 weeks gestation) and extremely low birth weight infant (ELBW) (premature labor improved neonatal mortality and morbidity in the late 1990s. The recognition that chronic postnatal administration of steroids to infants should be avoided may have improved outcomes in the early 2000s. Evidence from recent trials attempting to define the appropriate target for oxygen saturation in preterm infants suggests arterial oxygen saturation between 91–95% (compared to 85–89%) avoids excess mortality. However, final analyses of data from these trials have not been published, so definitive recommendations are still pending The development of neonatal neurocognitive care visits may improve neurocognitive outcomes in this high-risk group. Long-term follow up to detect and address developmental, learning, behavioral, and social problems is critical for children born at these early gestational ages. The striking similarities in response to extreme prematurity in the lung and brain imply that agents and techniques that benefit one organ are likely to also benefit the other. Finally, since therapy and supportive care continue to change, the outcomes of ELBW infants are ever evolving. Efforts to minimize injury, preserve

  2. Outcomes for extremely premature infants.

    Science.gov (United States)

    Glass, Hannah C; Costarino, Andrew T; Stayer, Stephen A; Brett, Claire M; Cladis, Franklyn; Davis, Peter J

    2015-06-01

    Premature birth is a significant cause of infant and child morbidity and mortality. In the United States, the premature birth rate, which had steadily increased during the 1990s and early 2000s, has decreased annually for 7 years and is now approximately 11.39%. Human viability, defined as gestational age at which the chance of survival is 50%, is currently approximately 23 to 24 weeks in developed countries. Infant girls, on average, have better outcomes than infant boys. A relatively uncomplicated course in the intensive care nursery for an extremely premature infant results in a discharge date close to the prenatal estimated date of confinement. Despite technological advances and efforts of child health experts during the last generation, the extremely premature infant (less than 28 weeks gestation) and extremely low birth weight infant (death and disability with 30% to 50% mortality and, in survivors, at least 20% to 50% risk of morbidity. The introduction of continuous positive airway pressure, mechanical ventilation, and exogenous surfactant increased survival and spurred the development of neonatal intensive care in the 1970s through the early 1990s. Routine administration of antenatal steroids during premature labor improved neonatal mortality and morbidity in the late 1990s. The recognition that chronic postnatal administration of steroids to infants should be avoided may have improved outcomes in the early 2000s. Evidence from recent trials attempting to define the appropriate target for oxygen saturation in preterm infants suggests arterial oxygen saturation between 91% and 95% (compared with 85%-89%) avoids excess mortality; however, final analyses of data from these trials have not been published, so definitive recommendations are still pending. The development of neonatal neurocritical intensive care units may improve neurocognitive outcomes in this high-risk group. Long-term follow-up to detect and address developmental, learning, behavioral, and

  3. Dysplasia in Inflammatory Bowel Diseases: Definition and Clinical Impact

    Directory of Open Access Journals (Sweden)

    Karel Geboes

    1999-01-01

    Full Text Available Dysplasia is a morphological term that ethymologically means ‘malformation’. For the definition of inflammatory bowel disease-related dysplasia, the nature and origin of the malformation are stressed and the lesion is defined as an epithelial malformation that is unequivocally neoplastic but noninvasive. The use of a precise definition is necessary because of the clinical consequences related to the finding of dysplasia in IBD. The microscopic diagnosis of dysplasia, however, remains difficult. Clinically, it is important to make a proper differential diagnosis between polypoid IBD-related dysplasia and sporadic adenoma occurring in IBD, and between therapy-related ‘pseudodysplasia’ and genuine dysplasia. When dysplasia is diagnosed, a second opinion may be indicated because of the clinical consequences. Additional techniques to search for genetic defects associated with carcinogenesis can help to support the diagnosis. They can identify changes in DNA content and molecular changes resulting from defects of genes controlling cell proliferation and death or tissue structure. These changes can, however, be absent, appear early or late in the transition from normality toward dysplasia and cancer, or appear during repair. Positive findings indicate an increased cancer risk, but the magnitude of the risk remains to be defined. A positive diagnosis of genuine dysplasia necessitates clinical action – either follow-up of the patient or treatment. In practice, treatment means surgery because dysplasia can be a precursor and/or a marker of malignancy, except for sporadic adenomas, which can be removed locally.

  4. 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.

  5. MODERN PRODUCTS FOR FEEDING PREMATURE BABIES

    Directory of Open Access Journals (Sweden)

    A. V. Surzhik

    2012-01-01

    Full Text Available In recent decades there has been substantial progress in the technology of premature infants nursing, especially with extremely low birth weight. Adequate feeding is one of the fundamental factors of premature babies nursing. To ensure a premature baby with all necessary components for power saving in breast milk intake, breast milk fortifiers — specifically developed additives that adjust the composition of food for premature babies, are used for more than 20 years (for premature babies receiving breast milk. On the one hand, to preserve all benefits of breastfeeding, on the other — to prevent the deficit development of necessary elements for adequate growth and development of nutrients.

  6. Activity Level and Severity of Dysplasia Predict Age at Bernese Periacetabular Osteotomy for Symptomatic Hip Dysplasia.

    Science.gov (United States)

    Matheney, Travis; Zaltz, Ira; Kim, Young-Jo; Schoenecker, Perry; Millis, Michael; Podeszwa, David; Zurakowski, David; Beaulé, Paul; Clohisy, John

    2016-04-20

    The age when patients present for treatment of symptomatic developmental dysplasia of the hip with periacetabular osteotomy (PAO) varies widely. Modifiable factors influencing age at surgery include preexisting activity level and body mass index (BMI). The severity of the hip dysplasia has also been implicated as a factor influencing the age at arthritis onset. The purpose of this study was to determine whether activity level, BMI, and severity of dysplasia are independent predictors of age of presentation for PAO. A retrospective, institutional review board-approved review of prospectively collected data from a multicenter study group identified 708 PAOs performed for developmental dysplasia of the hip. Demographic factors that were considered in the analysis included age at surgery, BMI, history of hip disorder or treatment, and duration of symptoms. The severity of the developmental dysplasia of the hip was assessed by radiographic measurement of the lateral and anterior center-edge angles and acetabular inclination. Activity level was assessed with the University of California, Los Angeles (UCLA) activity score. Spearman correlations and t tests were used for univariable analysis. Multivariable regression analysis using generalized estimating equations was applied to determine independent predictors of age at PAO. Univariable analysis indicated that age at presentation for treatment of PAO correlated with the lateral and anterior center-edge angles (p < 0.001), UCLA score (p < 0.001), and BMI (p = 0.04). Since the lateral and anterior center-edge angles were similarly correlated (Spearman rho = 0.61, p < 0.001), the lateral center-edge angle alone was used to classify the severity of the developmental dysplasia of the hip. Multivariable linear regression confirmed that a high UCLA score and severe hip dysplasia were independent predictors of age at PAO (p < 0.001). A high activity level and severe dysplasia lead to the development of symptoms and presentation

  7. 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.

  8. Sinobronchial allergic aspergillosis with allergic bronchopulmonary aspergillosis: a less common co-existence

    Science.gov (United States)

    Upadhyay, Rashmi; Kant, Surya; Prakash, Ved; Saheer, S

    2014-01-01

    Allergic bronchopulmonary aspergillosis (ABPA) is an immunological pulmonary disorder that is characterised by a hyper-responsiveness of the airways to Aspergillus fumigatus. Although several other fungi may also present with similar clinical conditions, Aspergillus remains the most common fungal pathogen causing airway infections. Co-existence of ABPA with allergic Aspergillus sinusitis (AAS) is an uncommon presentation. The concept of one airway/one disease justifies the co-existence of ABPA with AAS, but it does not always hold true. We report a case of a 35-year-old woman who presented with symptoms suggestive of bronchial asthma. On further investigation, the radiological pattern showed fleeting shadows and CT scan showed central cystic bronchiectatic changes characteristic of ABPA. The nasal secretions were investigated for the presence of Aspergillus and were found to be positive. Hence a diagnosis of ABPA with AAS was established. The patient was treated with oral steroids and antifungal drugs. PMID:25371437

  9. 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.

  10. Sinobronchial allergic aspergillosis with allergic bronchopulmonary aspergillosis: a less common co-existence.

    Science.gov (United States)

    Upadhyay, Rashmi; Kant, Surya; Prakash, Ved; Saheer, S

    2014-11-04

    Allergic bronchopulmonary aspergillosis (ABPA) is an immunological pulmonary disorder that is characterised by a hyper-responsiveness of the airways to Aspergillus fumigatus. Although several other fungi may also present with similar clinical conditions, Aspergillus remains the most common fungal pathogen causing airway infections. Co-existence of ABPA with allergic Aspergillus sinusitis (AAS) is an uncommon presentation. The concept of one airway/one disease justifies the co-existence of ABPA with AAS, but it does not always hold true. We report a case of a 35-year-old woman who presented with symptoms suggestive of bronchial asthma. On further investigation, the radiological pattern showed fleeting shadows and CT scan showed central cystic bronchiectatic changes characteristic of ABPA. The nasal secretions were investigated for the presence of Aspergillus and were found to be positive. Hence a diagnosis of ABPA with AAS was established. The patient was treated with oral steroids and antifungal drugs.

  11. Allergic bronchopulmonary aspergillosis in garden waste (compost) collectors--occupational implications.

    Science.gov (United States)

    Poole, C J M; Wong, M

    2013-10-01

    The separation of rotting garden material from general domestic waste and its collection for processing in industrial composting sites is a relatively new industry in the UK. Two cases of allergic bronchopulmonary aspergillosis and the results of health surveillance are described in a team of 28 garden waste (compost) collectors. A few cases of extrinsic allergic alveolitis due to Aspergillus fumigatus have previously been reported in compost workers. In the absence of any guidance from research and to prevent similar cases of a potentially serious illness, we advise that new starters to the job of collecting or processing compost are screened for asthma and aspergillus sensitivity, cystic fibrosis, bronchiectasis and immunodeficiency if their exposure to high levels of Aspergillus sp cannot be controlled. Annual health surveillance for these workers is also recommended.

  12. GATA3 mutation in a family with hypoparathyroidism, deafness and renal dysplasia syndrome

    Institute of Scientific and Technical Information of China (English)

    Zi-Yang Zhu; Qiao-Li Zhou; Shi-Ning Ni; Wei Gu

    2014-01-01

    Background: The hypoparathyroidism, deafness and renal dysplasia (HDR) syndrome is an autosomal dominant disorder primarily caused by GATA3 gene mutation. We report here a case that both of a Chinese boy and his father had HDR syndrome which caused by a novel mutation of GATA3. Methods: Polymerase chain reaction and DNA sequencing was performed to detect the exons of the GATA3 gene for mutation analysis. Results: Sequence analysis of GATA3 revealed a heterozygous nonsense mutation in this family: a mutation of GATA3 at exon 2 (c.515C >A) that resulted in a premature stop at codon 172 (p.S172X) with a loss of two zinc fi nger domains. Conclusion: We identifi ed a novel nonsense mutation which will expand the spectrum of HDR-associated GATA3 mutations.

  13. Atypical parakeratosis: a marker of dysplasia?

    Science.gov (United States)

    Voytek, T M; Kannan, V; Kline, T S

    1996-11-01

    The Bethesda System categorizes atypical parakeratosis (APK) as "ASCUS or SIL depending on the degree of cellular abnormalities." APK, however, is not well-defined. We retrospectively reviewed 68 cervicovaginal specimens with follow-up material to identify specific criteria and clinical significance of APK. APK cells were small cells, 2-3 times the diameter of neutrophil, with dense, orangeophilic cytoplasm, high nuclear cytoplasmic ratio, dense, often uneven chromatin, and irregular nuclear contour. Of 62 cases with APK, 37 had accompanying dysplastic cells. Of 25 cases with APK alone, follow-up revealed 12 with squamous intraepithelial lesion (5 HSIL and 7 LSIL) and 13 with benign changes. A major diagnostic pitfall of APK was inflammation with degeneration. Abundant APK cells, minimal inflammation and degeneration, and previous history of dysplasia frequently were associated with follow-up SIL. The findings of this study identify APK as an important marker for dysplasia that warrants careful evaluation and follow-up.

  14. 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.

  15. 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.

  16. Congenital osteofibrous dysplasia Campanacci: spontaneous postbioptic regression.

    Science.gov (United States)

    Jobke, Björn; Bohndorf, Klaus; Vieth, Volker; Werner, Mathias

    2014-04-01

    Osteofibrous dysplasia Campanacci is a rare benign bone tumor most frequently observed in young childhood. The exclusive localization in the tibia is very characteristic. The incidence of congenital primary bone tumors is an absolute rarity. We report a case of a newborn with a histologically proven osteofibrous dysplasia Campanacci at the tibia presenting a regular radiographic follow-up. After a small open biopsy and spontaneous minor fracture, the lesion rapidly remodeled within 1½ months and almost completely regressed with restutio ad integrum. Surgical intervention in this tumor entity at childhood age has been shown to have a high recurrence rate but due to lack of experience with newborns, guidelines do not exist. We analyze the radiologic and histologic differential diagnosis of juvenile adamantinoma and emphasize that congenital peripheral bone tumors should be treated conservatively when malignancy is excluded.

  17. Cardiac sarcoidosis mimicking right ventricular dysplasia.

    Science.gov (United States)

    Shiraishi, Jun; Tatsumi, Tetsuya; Shimoo, Kazutoshi; Katsume, Asako; Mani, Hiroki; Kobara, Miyuki; Shirayama, Takeshi; Azuma, Akihiro; Nakagawa, Masao

    2003-02-01

    A 59-year-old woman with skin sarcoidosis was admitted to hospital for assessment of complete atrioventricular block. Cross-sectional echocardiography showed that the apical free wall of the right ventricle was thin and dyskinetic with dilation of the right ventricle. Thallium-201 myocardial imaging revealed a normal distribution. Both gallium-67 and technetium-99m pyrophosphate scintigraphy revealed no abnormal uptake in the myocardium. Right ventriculography showed chamber dilation and dyskinesis of the apical free wall, whereas left ventriculography showed normokinesis, mimicking right ventricular dysplasia. Cardiac sarcoidosis was diagnosed on examination of an endomyocardial biopsy specimen from the right ventricle. A permanent pacemaker was implanted to manage the complete atrioventricular block. After steroid treatment, electrocardiography showed first-degree atrioventricular block and echocardiography revealed an improvement in the right ventricular chamber dilation. Reports of cardiac sarcoidosis mimicking right ventricular dysplasia are extremely rare and as this case shows, right ventricular involvement may be one of its manifestations.

  18. 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....... Neither subjects with dysplasia nor controls had radiological signs of ongoing degenerative disease at admission. The primary radiological discriminator of degeneration of the hip was a change in the minimum joint space width over time. There were no significant differences between these with dysplasia...... and controls in regard to age, body mass index or occupational exposure to daily repeated lifting at admission.We found no significant differences in the reduction of the joint space width at follow-up between subjects with dysplasia and the control subjects nor in self-reported pain in the hip...

  19. 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.

  20. 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.

  1. Bronchopulmonary infection with lophomonas blattarum: two cases report and literature review

    Institute of Scientific and Technical Information of China (English)

    Yao Guozhong

    2008-01-01

    Objective: To improve the recognition and diagnosis on the bronchopulmonary infection with Lophomonas blattarum (L. blattarum). Methods: The clinical characteristics of 2 patients diagnosed and treated in our hospital were reported, and 42 cases that had been reported from years 1993 to 2007 are analyzed. Results: In our report, the firs tpatient attacked serious asthma time after time, the second patient suffered from bronchiectasis with a protracte dinfection course. Forty-four cases all have pathogen examination and parasitic expertise. The most common symptoms are fever, cough and expectoration. 1/3 of the patients have increased acidophilic granulocyte in peripheral blood. Chest X film and CT scanning suggest changes were similar to pneumonia. Chronic cases are manifested with bronchial asthma, bronchiectasis and pulmonary abscess. L. blattarum found in phlegm or specimen collected by bronchoscopy provides the most reliable evidence for the diagnosis of this disease. Conclusion: Bronchopulmonary L. blattarum infection is a new kind of diseases. The clinical manifestations are similar to pneumonia, asthma, bronchiectasis infection or pulmonary abscess. L. blattarum found in sputum smear, bronchoscopic brush smear, bronchoscopic biopsy smear, or bronchoalceolar lavage under microscope is the foundation of the diagnosis. The pathogen species has not been finally confirmed. It is still unclear how the pathogen exists in the natural environment, how to transmit to persons and what kind of people would suffer from the disease more easily. Treatment only with antibiotics is not effective to this disease. Metronidazole with dosage of 0.5 g per time and twice per day was effective to most patients, the period of treatment need to last 14-38 d, but multidrug resistance case had been reported.

  2. 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.

  3. 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.

  4. Fibrous dysplasia of bone causing unilateral proptosis

    Directory of Open Access Journals (Sweden)

    Reshma Ramakrishnan

    2015-01-01

    Full Text Available Fibrous dysplasia (FD is a slow growing benign noninherited disorder in which normal bones are replaced by fibrous tissue and immature woven bones. Here we report a case of 30-year-old female who presented with unilateral proptosis and lagophthalmos without any visual loss; was diagnosed with polyostotic FD. Patient was taken up for surgery. Osseous reconturing was done by shaving of involved facial bones. Postoperatively, there was a decrease in proptosis and lagophthalmos.

  5. 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.)

  6. Fetal MR imaging of Kniest dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Yazici, Zeynep [Uludag University, Faculty of Medicine, Department of Radiology, Gorukle (Turkey); Kline-Fath, Beth M.; Laor, Tal [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Tinkle, Bradley T. [Cincinnati Children' s Hospital Medical Center, Division of Human Genetics, Cincinnati, OH (United States)

    2010-03-15

    We present a case of Kniest dysplasia, a rare form of the type II collagenopathies, with prenatal MRI. Sonography revealed only short limbs in the fetus. Fetal MRI findings included enlarged hyaline cartilaginous structures with abnormally high T2 signal intensity, delayed ossification of the pubic and ischial bones, and platyspondyly. By delineating the cartilaginous abnormalities, fetal MRI can contribute to the prenatal diagnosis of chondrodysplasias. (orig.)

  7. Congenital Ocular Dystopia from Orbitofrontal Bone Dysplasia.

    Science.gov (United States)

    Swanson, Jordan W; Bartlett, Scott P

    2016-01-01

    Several patients with ocular dystopia and craniofacial differences have been found to have orbital dysplasia and a frontal bone defect. This deformity is characteristically different from differential diagnoses of encephalocele, sphenoid dysplasia, craniofacial dysostoses, or atypical clefting. The authors retrospectively reviewed the craniofacial registries of two pediatric centers for patients presenting with ocular dystopia or orbitofrontal anomalies between 2000 and 2014. The features and treatment of these patients were analyzed. Four patients with congenital orbitofrontal bone dysplasia were identified, three with unilateral and one with bilateral frontal bone defects. Clinical signs of hypoglobus and vertical ocular dystopia of an average of 5.0 mm on the affected side were noted shortly after birth. The transversely oriented bony defect had an average surface area of 3.9 cm in unilateral cases and 10.7 cm in bilateral cases. Patients showed a characteristic orbital vertical elongation with an average orbital height-to-width ratio of 1.30-in excess of the average normal 1.14 by 14 percent-and inferior rim displacement. Cranial contour demonstrated frontal bossing and borderline dolichocephaly. Fronto-orbital reconstruction was performed in three patients, using cranial bone grafting to obliterate the orbital roof defect and elevate the orbital floor, which was successful in reducing ocular dystopia and preserving vision in each patient. One patient followed for 11 years postoperatively has a durable result with no surgical revision. Orbitofrontal bone dysplasia has not been previously reported, and includes a frontal bone defect and ocular dystopia. Single-stage fronto-orbital reconstruction appears to adequately correct it. Therapeutic, IV.

  8. Lethality in Desbuquois dysplasia: three new cases

    Energy Technology Data Exchange (ETDEWEB)

    Hall, B.D. [Department of Pediatrics, University of Kentucky College of Medicine, Lexington (United States)

    2001-01-01

    Three new cases of Desbuquois syndrome in two brothers and a sporadic male, all of whom died in early infancy, are presented to emphasize the high rate (33 %) of lethality in this variable, but serious skeletal dysplasia. Including the three presented patients and 10 of the 36 cases in the literature who died, most did so between birth and 7 months and from respiratory-related problems. Neonatal and infancy survivors should be monitored closely, particularly relative to their pulmonary status. (orig.)

  9. 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.

  10. An index for diagnosing infant hip dysplasia using 3-D ultrasound: the acetabular contact angle.

    Science.gov (United States)

    Mabee, Myles G; Hareendranathan, Abhilash Rakkunedeth; Thompson, Richard B; Dulai, Sukhdeep; Jaremko, Jacob L

    2016-06-01

    Developmental dysplasia of the hip (DDH) is a common condition that is highly treatable in infancy but can lead to the lifelong morbidity of premature osteoarthritis if left untreated. Current diagnostic methods lack reliability, which may be improved by using 3-D ultrasound. Conventional 2-D US assessment of DDH has limitations, including high inter-scan variability. We quantified DDH on 3-D US using the acetabular contact angle (ACA), a property of the 3-D acetabular shape. We assessed ACA reliability and diagnostic utility. We prospectively collected data from January 2013 to December 2014, including 114 hips in 85 children divided into three clinical diagnostic groups: (1) normal, (2) initially borderline but ultimately normal without treatment and (3) dysplastic requiring treatment. Using custom software, two observers each traced acetabula twice on two 3-D US scans of each hip, enabling automated generation of 3-D surface models and ACA calculation. We computed inter-observer and inter-scan variability of repeatability coefficients and generated receiver operating characteristic (ROC) curves. The 3-D US acetabular contact angle was reproduced 95% of the time within 6° in the same scan and within 9° in different scans of the same hip, vs. 9° and 14° for the 2-D US alpha angle (P dysplasia of the hip were 0.954 for ACA and 0.927 for alpha angle. The 3-D US ACA was significantly more reliable than 2-D US alpha angle, and the 3-D US measurement predicted the presence of DDH with slightly higher accuracy. The ACA therefore shows promising initial diagnostic utility. Our findings call for further study of 3-D US in the diagnosis and longer-term follow-up of infant hip dysplasia.

  11. Campomelic dysplasia: a rare cause of congenital spinal deformity.

    Science.gov (United States)

    Dahdaleh, Nader S; Albert, Gregory W; Hasan, David M

    2010-05-01

    Campomelic dysplasia is a rare autosomal dominant syndrome that often results in congenital spinal deformity. As a result of improvements in respiratory care, some patients survive into childhood, requiring treatment of their spinal deformities. We present a neonate who was diagnosed with campomelic dysplasia, resulting in severe cervical and thoracic kyphoscoliosis and respiratory compromise. A review of the literature and reported treatment options are discussed. Campomelic dysplasia is a rare cause of congenital spinal deformity; however, intervention may be appropriate in certain patients.

  12. Hip arthroscopy in the setting of hip dysplasia

    Science.gov (United States)

    Yeung, M.; Kowalczuk, M.; Simunovic, N.

    2016-01-01

    Objective Hip arthroscopy in the setting of hip dysplasia is controversial in the orthopaedic community, as the outcome literature has been variable and inconclusive. We hypothesise that outcomes of hip arthroscopy may be diminished in the setting of hip dysplasia, but outcomes may be acceptable in milder or borderline cases of hip dysplasia. Methods A systematic search was performed in duplicate for studies investigating the outcome of hip arthroscopy in the setting of hip dysplasia up to July 2015. Study parameters including sample size, definition of dysplasia, outcomes measures, and re-operation rates were obtained. Furthermore, the levels of evidence of studies were collected and quality assessment was performed. Results The systematic review identified 18 studies investigating hip arthroscopy in the setting of hip dysplasia, with 889 included patients. Criteria used by the studies to diagnose hip dysplasia and borderline hip dysplasia included centre edge angle in 72% of studies but the range of angles were quite variable. Although 89% of studies reported improved post-operative outcome scores in the setting of hip dysplasia, revision rates were considerable (14.1%), with 9.6% requiring conversion to total hip arthroplasty. Conclusion The available orthopaedic literature suggests that although improved outcomes are seen in hip arthroscopy in the setting of hip dysplasia, there is a high rate of re-operation and conversion to total hip arthroplasty. Furthermore, the criteria used to define hip dysplasia vary considerably among published studies. Cite this article: M. Yeung, M. Kowalczuk, N. Simunovic, O. R. Ayeni. Hip arthroscopy in the setting of hip dysplasia: A systematic review. Bone Joint Res 2016;5:225–231. DOI: 10.1302/2046-3758.56.2000533. PMID:27313136

  13. Malignant transformation of fibrous dysplasia into chondroblastic osteosarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Kaushik, Shaifali [Department of Radiology, Medical College of Virginia, Virginia Commonwealth University, Richmond, VA (United States); Department of Radiology, Medical College of Virginia, Virginia Commonwealth University, Richmond, VA (United States); Smoker, Wendy R.K. [Department of Radiology, Medical College of Virginia, Virginia Commonwealth University, Richmond, VA (United States); Frable, William J. [Department of Pathology, Medical College of Virginia, Virginia Commonwealth University, Richmond, VA (United States)

    2002-02-01

    A case of malignant transformation of polyostotic fibrous dysplasia into maxillary chondroblastic osteosarcoma is presented. The clinical, radiographic, CT, MR imaging features and pathological findings of polyostotic fibrous dysplasia and its malignant transformation are described. Malignant transformation of fibrous dysplasia is rare and has not previously been described in the English literature in this location in McCune-Albright syndrome and in the absence of radiation treatment. (orig.)

  14. A Case Report of Camptomelic Dysplasia

    Directory of Open Access Journals (Sweden)

    Zia Islami

    2011-09-01

    Full Text Available Camptomelic Dysplasia (CMD is a rare autosomal dominant congenital dwarfism characterized by shortness and bowing of long bones (camptomelia and other severe skeletal and extra skeletal malformations. CMD is generally considered to be lethal and the majority of cases die in the neonatal period due to respiratory insufficiency.We hereunder report a term male neonate with characteristic clinical and radiological findings of CMD, hydrocephaly, no sex reversal, and a negative family history of skeletal problems who was born to non-consanguineous  healthy parents and was admitted to Shahid Sadoughi Hospital, Yazd, Iran,immediately after birth due to respiratory distress.The patient required continuous mechanical ventilation support and all attempts to reduce respiratory support failed and the patient died on the 21th day of his life. Camptomelic Dysplasia is a terrible experience for parents; thus, prenatal diagnosis of CMD by ultrasound is essential and mandatory for a better therapeutic intervention.Key words: Camptomelic dysplasia; dwarfism/congenital; bowing of longbones; sex-reversal

  15. Clinical and molecular characterization of a Brazilian cohort of campomelic dysplasia patients, and identification of seven new SOX9 mutations

    Directory of Open Access Journals (Sweden)

    Eduardo P. Mattos

    2015-03-01

    Full Text Available Campomelic dysplasia (CD is an autosomal, dominantly inherited, skeletal abnormality belonging to the subgroup of bent bone dysplasias. In addition to bowed lower limbs, CD typically includes the following: disproportionate short stature, flat face, micrognathia, cleft palate, bell-shaped thorax, and club feet. Up to three quarters of 46, XY individuals may be sex-reversed. Radiological signs include scapular and pubic hypoplasia, narrow iliac wings, spaced ischia, and bowed femora and tibiae. Lethal CD is usually due to heterozygous mutations in SOX9, a major regulator of chondrocytic development. We present a detailed clinical and molecular characterization of nine Brazilian CD patients. Infants were either stillborn (n = 2 or died shortly after birth and presented similar phenotypes. Sex-reversal was observed in one of three chromosomally male patients. Sequencing of SOX9 revealed new heterozygous mutations in seven individuals. Six patients had mutations that resulted in premature transcriptional termination, while one infant had a single-nucleotide substitution at the conserved splice-site acceptor of intron 1. No clear genotype-phenotype correlations were observed. This study highlights the diversity of SOX9 mutations leading to lethal CD, and expands the group of known genetic alterations associated with this skeletal dysplasia.

  16. 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.

  17. Focal cemento-osseous dysplasia: review and a case report.

    Science.gov (United States)

    Salem, Y M Y; Osman, Y I; Norval, E J G

    2010-10-01

    Focal cemento-osseous dysplasia is a benign fibro-osseous condition that can be seen in dentate and edentulous patients. It is an asymptomatic lesion and needs no treatment; however follow-up is essential due to the possibility that focal cemento-osseous dysplasia can progress to a condition called florid osseous dysplasia that involves multiple sites. A case report is presented here, along with a review of the differential diagnoses considered in order to reach a final diagnosis of focal cemento-osseous dysplasia.

  18. Lymphoscintigraphy patterns in newborns and children with congenital lymphatic dysplasia.

    Science.gov (United States)

    Bellini, C; Villa, G; Sambuceti, G; Traggiai, C; Campisi, C; Bellini, T; Morcaldi, G; Massocco, D; Bonioli, E; Boccardo, F

    2014-03-01

    We performed lymphoscintigraphy on 31 patients (newborns and children) affected by congenital lymphatic dysplasia according to our previously published protocol. Congenital lymphatic dysplasia may present with various degrees of clinical severity, ranging from nonimmune hydrops fetalis with visceral effusions to lymphedema alone. We recommend that lymphoscintigraphy should be strongly considered in all patients with signs of lymphatic dysplasia, including those with minimal and initial signs of lymphatic impairment, in order to obtain a very early diagnosis and to start treatment. Lymphoscintigraphy is safe and useful in the diagnosis of lymphatic dysplasia in the newborn and children. Moreover, it is well tolerated by patients and well accepted by their parents.

  19. The Ectodermal Dysplasias : Severe Palmoplantar Hyperkeratosis And Chronic Angular Cheilitis

    Directory of Open Access Journals (Sweden)

    Mahajan Vikram K

    2003-01-01

    Full Text Available The ectodermal dysplasias are congenital, non-progressive and diffuse disorders affecting primarily the tissues derived from ectoderm. Over a period, their classification has become confusing due to indiscriminate use of them “ectodermal dysplasia” for numerous syndromes with a defect in one or more epidermal defect in each element of skin; their precise classification appears difficult as yet. Only X-linked recessive ectodermal dysplasia (Christ-Siemens-Touraine syndrome remains best defined. This paper describes three cases of ectodermal dysplasias highlighting their overlapping features.

  20. Premature birth and diseases in premature infants: common genetic background?

    Science.gov (United States)

    Hallman, Mikko

    2012-04-01

    It has been proposed that during human evolution, development of obligate bipedalism, narrow birth canal cross-sectional area and the large brain have forced an adjustment in duration of pregnancy (scaling of gestational age; Plunkett 2011). Children compared to other mammals are born with proportionally small brains (compared to adult brains), suggesting shortening of pregnancy duration during recent evolution. Prevalence of both obstructed delivery and premature birth is still exceptionally high. In near term infants, functional maturity and viability is high, and gene variants predisposing to respiratory distress syndrome (RDS) are rare. Advanced antenatal and neonatal treatment practices during the new era of medicine allowed survival of also very preterm infants (gestation premature birth. Specific genes associating with diseases in preterm infants may also contribute to the susceptibility to preterm birth. Understanding and applying the knowledge of genetic interactions in normal and abnormal perinatal-neonatal development requires large, well-structured population cohorts, studies involving the whole genome and international interdisciplinary collaboration.

  1. Osseous dysplasia (cemento-osseous dysplasia) of the jaw bones in western Pennsylvania patients: analysis of 35 cases.

    Science.gov (United States)

    Owosho, Adepitan A; Potluri, Anitha; Bilodeau, Elizabeth A

    2013-01-01

    The purpose of this study is to analyze the demographic, clinical, and radiographic presentations of osseous dysplasia of the jaws in western Pennsylvania patients and its associated complications. The clinical records and radiographs of patients diagnosed with osseous (cement-osseous) dysplasia were retrieved from the electronic health record of the University of Pittsburgh, School of Dental Medicine from 2007 to 2012. All cases were reviewed; the WHO criteria and classification for osseous dysplasia was used. Clinical and demographic data, radiographic findings, and final diagnoses were collected and analyzed. 35 cases of osseous dysplasia were retrieved over the six-year period.The majority (33) were females [94.3%], with ages ranging from 26 to 89 years, with a mean age of 53.9 years +/- standard deviation of 15.6 years, 32 [91.4%] were African Americans and 3 [8.6%] were Caucasians. 17 [48.6%] were florid osseous dysplasia, 13 [37.1%] periapical osseous dysplasia and 5 [14.3%] focal osseous dysplasia. Of the 35 patients only 8 [22.9%] patients were symptomatic. All florid osseous dysplasia patients were African American females, with 7 of the patients being symptomatic and the commonest symptom being pain. Also, all periapical osseous dysplasia patients were African Americans (12 females and 1 male), with 1 of the patients presenting with widening of the diastema. Of the focal osseous dysplasia patients, 3 were Caucasians and 2 African American (4 females and 1 male). The cases occurred mostly in African American females with a peak incidence in the fifth and sixth decades of life; most cases occurred in the mandible. The commonest form of osseous dysplasias was the florid osseous dysplasia which is most likely to present with symptoms.

  2. [Premature ejaculation: pills or sexology?].

    Science.gov (United States)

    Wisard, M; Audette, N

    2008-03-26

    Premature ejaculation (PE) is a frequent male sexual complaint that affects 20 to 30% of men. The exact aetiology is unknown: psychological/behavioristic and biogenic etiologies have been proposed. The introduction of selective serotonin reuptake inhibitors (SSRI) was revolutionary in the medical treatment of PE. However precautions should be taken because of potential adverse side effects. There is no clear consensus as to whether SSRI may represent an eventual cure of PE or will be required for life. The sexocorporal approach is an other treatment of PE, but convincing scientific treatment data are also lacking.

  3. Positional moulding in premature hydrocephalics.

    Directory of Open Access Journals (Sweden)

    Kumar R

    2002-04-01

    Full Text Available Seven premature hydrocephalics presenting with lambdoid positional moulding (LPM were reviewed. All were treated for hydrocephalus secondary to aqueductal stenosis, Dandy Walker Syndrome and infection. Parenchymal hemorrhage, intraventricular bleed, cortical atrophy, septal agenesis, cortical anomalies and subdural hygroma were the other common associations. These children did not show expected improvement in their higher mental functions at 6 months to 5.4 years of follow-up, following the management of hydrocephalus. It was not the LPM but associated intracranial anomalies, which were most probably responsible for their poor outcome. The differentiation from posterior plagiocephaly is also highlighted.

  4. Metabolic bone disease of prematurity

    Directory of Open Access Journals (Sweden)

    Stacy E. Rustico, MD

    2014-09-01

    Full Text Available Metabolic bone disease (MBD of prematurity remains a significant problem for preterm, chronically ill neonates. The definition and recommendations for screening and treatment of MBD vary in the literature. A recent American Academy of Pediatrics Consensus Statement may help close the gap in institutional variation, but evidence based practice guidelines remain obscure due to lack of normative data and clinical trials for preterm infants. This review highlights mineral homeostasis physiology, current recommendations in screening and monitoring, prevention and treatment strategies, and an added perspective of a bone health team serving a high volume referral neonatal intensive care center.

  5. Current therapies for premature ejaculation.

    Science.gov (United States)

    Gur, Serap; Kadowitz, Philip J; Sikka, Suresh C

    2016-07-01

    Premature ejaculation (PE) subjectively affects 20-30% of men globally. Until recently, understanding of PE was hampered by the absence of a widely accepted definition, paucity of evidence-based clinical studies, and the absence of an appropriate animal model. Here, we elaborate on the current definition of PE, its pathogenesis, currently available therapies, and future treatment prospects. Most treatments for PE are 'off-label' and include selective serotonin reuptake inhibitors (SSRIs), topical anesthetics, tramadol, and phosphodiesterase type 5 (PDE5) inhibitors. Such knowledge of the benefit and limitations of each treatment will help to direct future drug design and formulations.

  6. Premature ventricular contractions associated with isotretinoin use.

    Science.gov (United States)

    Alan, Sevil; Ünal, Betül; Yildirim, Aytül

    2016-01-01

    Isotretinoin has been considered a unique drug for acne treatment. However, it is associated with numerous adverse effects. Isotretinoin can trigger premature ventricular contractions. This report describes a 33-year-old-woman who presented with palpitations for 1 week while undergoing 1-month isotretinoin treatment for mild-moderate facial acne. An electrocardiogram and Holter monitoring showed premature ventricular contractions during isotretinoin (Roaccutane, Roche) treatment. Isotretinoin-related premature ventricular contractions were strongly suggested in this case due to the existence of documented premature ventricular contractions on electrocardiograms and the disappearance of these premature ventricular contractions two weeks after termination of the treatment To the authors' knowledge, there has been 1 reported case of premature ventricular contractions linked to isotretinoin use; this report describes a second such case.

  7. Premature ventricular contractions associated with isotretinoin use*

    Science.gov (United States)

    Alan, Sevil; Ünal, Betül; Yildirim, Aytül

    2016-01-01

    Isotretinoin has been considered a unique drug for acne treatment. However, it is associated with numerous adverse effects. Isotretinoin can trigger premature ventricular contractions. This report describes a 33-year-old-woman who presented with palpitations for 1 week while undergoing 1-month isotretinoin treatment for mild-moderate facial acne. An electrocardiogram and Holter monitoring showed premature ventricular contractions during isotretinoin (Roaccutane, Roche) treatment. Isotretinoin-related premature ventricular contractions were strongly suggested in this case due to the existence of documented premature ventricular contractions on electrocardiograms and the disappearance of these premature ventricular contractions two weeks after termination of the treatment To the authors' knowledge, there has been 1 reported case of premature ventricular contractions linked to isotretinoin use; this report describes a second such case. PMID:28099609

  8. 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.

  9. Premature temporal theta (PT theta).

    Science.gov (United States)

    Hughes, J R; Fino, J J; Hart, L A

    1987-07-01

    A distinctive pattern called premature temporal theta (PT theta) was studied in 436 infants, ranging in age from 24 to 46 weeks. The pattern is seen in early prematurity, maximizes at 29-31 weeks and then diminishes and disappears near term. Usually the pattern is found independently on both temporal areas, but with a right-sided preference. Patients without PT theta or with a significantly low amount had either neurological or non-neurological (medical) conditions. With age there is a tendency for an increase in frequency and a decrease in amplitude. Five different peaks in the amount of this pattern are seen at approximately every month. Unilateral PT theta tends to be seen in older babies, more often on the right side and with an abnormal EEG. An abnormal EEG is usually associated with a delay in both the appearance and disappearance of this wave form. PT theta is also associated mainly with REM or active sleep. A polynomial rather than an exponential or power function best describes these data with changes of age. PT theta may arise from the inferior temporal gyrus and/or especially the transverse gyrus.

  10. 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 neuroendocrine...... tumours. These guidelines essentially cover basic knowledge in the diagnosis and management of the different forms of neuroendocrine tumour. We have, however, tried to give more updated information about the epidemiology and histopathology, which is essential for the clinical management of these tumours....

  11. A Contemporary Definition of Hip Dysplasia and Structural Instability: Toward a Comprehensive Classification for Acetabular Dysplasia.

    Science.gov (United States)

    Wilkin, Geoffrey P; Ibrahim, Mazen M; Smit, Kevin M; Beaulé, Paul E

    2017-09-01

    Hip dysplasia has long been known to be a risk factor for pain and degenerative changes in the hip joint. The diagnosis of dysplasia has historically been based on assessments of acetabular anatomy on the anteroposterior pelvic radiograph, most commonly the lateral center-edge angle. Recent advances in imaging of the dysplastic hip with computerized tomography scans have demonstrated that hip dysplasia is in fact a 3-dimensional (D) deformity of the acetabulum and that multiple patterns of hip instability exist that may not be completely assessed on 2D imaging. A more thorough understanding of acetabular anatomy permits an evolution away from vague terms such as "borderline dysplasia." A 3D assessment of the acetabulum and the resultant patterns of instability may be more appropriate since this would allow more accurate treatment to correct the structural instability with acetabular reorientation. With this information, we propose a diagnostic framework that groups symptomatic dysplastic hips into one of 3 categories based on the primary direction of instability: (1) anterior, (2) posterior, and (3) global. This framework may aid the clinician in developing a differential diagnosis for the assessment of hip pain and suspected instability, and for planning an appropriate surgical management. Copyright © 2017 Elsevier Inc. All rights reserved.

  12. Patient's Guide to Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

    Science.gov (United States)

    ... Page Patient’s Guide to Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Past to Present Crystal Tichnell , Cynthia A. James , ... Info & Metrics eLetters Introduction Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited progressive disease of ...

  13. 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...

  14. 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.

  15. 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.

  16. A meta-analysis of nasal intermittent positive pressure ventilation in treatment of respiratory distress syndrome in premature infants%早产儿呼吸窘迫综合征经鼻间歇正压通气治疗的Meta分析

    Institute of Scientific and Technical Information of China (English)

    崔蕴璞; 童笑梅; 叶鸿瑁; 王波; 汤亚南

    2009-01-01

    Objective To agsess the efficacy of nasal intermittent positive pressure ventilation (NIPPV)in treatment of respiratory distress syndrome(RDS) in premature infants.Methods According to the requirements of Cochrane systematic review,a thorough literature search was performed among PubMed(1977-2008),Embase(1989-2008),OVID,Cochrane(2008),Chinese Digtal Hospital Library (www.chkd.cnki.net) and Chinese Biomedical Literature Disk Database(CBMdisc).Quality assessments of clinical trials were carried out Randomized controHed trials(RCTs)with NIPPV and RDS were enrolledand ReVnlan 4.2 software was used for meta-analysis.The trials were analyzed using relatire risk(RR) for dichotomous data,weighted mean difference(WMD) were used for continuous data,both kind of data were expressed by 95% confidence intervals(95%CI).For homogenous data(P≥0.10),fixed effects model was calculated,for heterogeneity data(P<0.10),random effects model was calculated.Results Five RCTs involving 284 premature infants diagnosed as respiratory distress syndrome(RDS) were included.Three studies comparing NIPPV with nasal continuous positive airway pressure(NCPAP) in the postextubation period,tHe extabation failure rate was 8.34%vs 40.79% in NIPPV group and NCPAP group,the NIPPV group had significantly lower extubation failure rates[RR 0.21(95%CI:0.10-0.45;P<0.001)].Two of the above.mentioned three studies analyzed bronchopulmonary dysplasia(BPD)rates,the incidence of BPD was 39.34%vs 54.39%in NIPPV group and NCPAP group,the NIPPV group had a trend towards lower BPD rates,but this did not reach statistical significance[RR 0.73(95%CI:0.49-1.07;P=0.11)].NIPPV was used as primary mode in two studies,one compared with conventional ventilation(CV),which detected that the NIPPV group had significantly lower BPD rates(10% vs.33.33%,P:0.04);the other compared with NCPAP,which also showed that NIPPV group had significantly lower BPD rates(2.33% vs.17.07%,P=0.03).Conclusion The primary mode NIPPV was found to be

  17. The time of onset of abnormal calcification in spondylometaepiphyseal dysplasia, short limb-abnormal calcification type

    Energy Technology Data Exchange (ETDEWEB)

    Tueysuez, Beyhan [Istanbul University, Department of Pediatric Genetics, Cerrahpasa Medical School, Istanbul (Turkey); Gazioglu, Nurperi [Istanbul University, Department of Neurosurgery, Cerrahpasa Medical School, Istanbul (Turkey); Uenguer, Savas [Istanbul University, Department of Pediatric Radiology, Cerrahpasa Medical School, Istanbul (Turkey); Aji, Dolly Yafet [Istanbul University, Department of Pediatrics, Cerrahpasa Medical School, Istanbul (Turkey); Tuerkmen, Seval [Istanbul University, Department of Pediatric Genetics, Cerrahpasa Medical School, Istanbul (Turkey); Universitatsklinikum Berlin, Charite Virchow-Klinik, Berlin (Germany)

    2009-01-15

    A 1-month-old boy with shortness of extremities on prenatal US was referred to our department with a provisional diagnosis of achondroplasia. His height was normal but he had short extremities and platyspondyly, premature carpal epiphyses on both hands, and short tubular bones with irregular metaphyses on radiographs. Re-evaluation of the patient at the age of 1 year revealed very short height and premature calcification of the costal cartilages and epiphyses. Spondylometaepiphyseal dysplasia (SMED), short limb-abnormal calcification type was diagnosed. This condition is a very rare autosomal recessively inherited disorder, and most of the patients die in early childhood due to neurological involvement. At the age of 2 years and 5 months, a CT scan showed narrowing of the cervical spinal canal. One month later he died suddenly because of spinal cord injury. In conclusion early diagnosis is very important because the recurrence risk is high and patients may die due to early neurological complications. The time of onset of abnormal calcifications, a diagnostic finding of the disease, is at the age of around 1 year in most patients. When abnormal calcifications are not yet present, but radiological changes associated with SMED are present, this rare disease must be considered. (orig.)

  18. The time of onset of abnormal calcification in spondylometaepiphyseal dysplasia, short limb-abnormal calcification type.

    Science.gov (United States)

    Tüysüz, Beyhan; Gazioğlu, Nurperi; Ungür, Savaş; Aji, Dolly Yafet; Türkmen, Seval

    2009-01-01

    A 1-month-old boy with shortness of extremities on prenatal US was referred to our department with a provisional diagnosis of achondroplasia. His height was normal but he had short extremities and platyspondyly, premature carpal epiphyses on both hands, and short tubular bones with irregular metaphyses on radiographs. Re-evaluation of the patient at the age of 1 year revealed very short height and premature calcification of the costal cartilages and epiphyses. Spondylometaepiphyseal dysplasia (SMED), short limb-abnormal calcification type was diagnosed. This condition is a very rare autosomal recessively inherited disorder, and most of the patients die in early childhood due to neurological involvement. At the age of 2 years and 5 months, a CT scan showed narrowing of the cervical spinal canal. One month later he died suddenly because of spinal cord injury. In conclusion early diagnosis is very important because the recurrence risk is high and patients may die due to early neurological complications. The time of onset of abnormal calcifications, a diagnostic finding of the disease, is at the age of around 1 year in most patients. When abnormal calcifications are not yet present, but radiological changes associated with SMED are present, this rare disease must be considered.

  19. Fibrous dysplasia. Clinical review and therapeutic management.

    Science.gov (United States)

    Florez, Helena; Peris, Pilar; Guañabens, Núria

    2016-12-16

    Fibrous dysplasia is a skeletal disorder that is associated with a wide spectrum of clinical manifestations, including localized asymptomatic forms and extensive severe forms with severe bone deformities and endocrinological alterations, depending on age, location, extent and associated processes. Although the treatment of choice is based on bisphosphonates, the therapeutic efficacy of these agents in the control of disease activity remains uncertain. This article reviews the current data available on the treatment of this disease as well as the preliminary data on new therapeutic approaches. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

  20. 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.)

  1. Hereditary mucoepithelial dysplasia and severe respiratory distress

    Directory of Open Access Journals (Sweden)

    Mahmoud Halawa

    2015-01-01

    Full Text Available Hereditary mucoepithelial dysplasia (HMD is a rare autosomal dominant disorder characterized by mucoepithelial disruption of the skin, hair and mucous membranes. It results from defective gap junction formation and leads to non-scarring alopecia, mucosal erythema, perineal erythematous intertrigo, involvement of the conjunctival mucosa, and pulmonary disease. We present a case of severe respiratory distress in an initially healthy full term infant born to a mother with HMD. This infant later developed signs and symptoms of HMD. A high index of suspicion for pulmonary infection with atypical organism is essential in infants with a family history of HMD who present with respiratory distress.

  2. Tibia Vara due to Focal Fibrocartilaginous Dysplasia

    Directory of Open Access Journals (Sweden)

    A Tavakoli

    2004-06-01

    Full Text Available We present a case of unilateral tibia vara associated with an area of focal fibrocartilaginous dysplasia in the medial aspect of the right proximal tibia. Such a case has not been described previously. The affected child was 8 months old. Deformity resolved without aggressive treatment. The pathogenesis of the focal lesion remains controversial. The most likely explanation is that the mesenchymal anlage of the tibial metaphysis has for unknown reasons, developed abnormality at the insertion of the pes anserinus. Keywords: Tibia Vara, Pes Anserinus

  3. [Importance of hand films in skeletal dysplasia (author's transl)].

    Science.gov (United States)

    Fendel, H

    1976-07-01

    The hand, as part of the skeleton, is generally involved in systemic skeletal dysplasia. However, the degree of abnormalities differs considerably in the various types of dysplasia. In some, abnormal appearance of the hand predominates, in other dysplasia films of the hand provide only little or no useful diagnostic information. At their first examination for growth disorders children often reveal bone dysplasia on roentgenograms of the hand. Therefore, evaluation of hand films should be done as thoroughly as possible. Morphologic and/or metric deviations of the hand may bring the first suspicion although they are not fully diagnostic. Systemic skeletal dysplasia should be diagnosed or excluded by additional adequate radiologic and other clinical examinations.

  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. The Effect of Corticosteroid on Accelerating Fetal Lung Maturity in Premature Infants%应用糖皮质激素促进胎儿肺成熟对早产儿的影响

    Institute of Scientific and Technical Information of China (English)

    刘志军; 黄暖潮; 黄彩红

    2012-01-01

    differences be- tween two groups in neither head circumference nor body length (p 〉0. 05 ). The corticosteroid group had higher level of IgA (A1 vs. A2, p 〈 0. 05) and IgM ( M1 vs. M2, p 〈 0. 05 ), while the differ- ence of IgG level was not significant (p 〉 0. 05 ). There was lower risk of bronchopulmonary dysplasia (BPD) and retinopathy of prematurity (ROP) in corticosteroid group than in control group (B1 vs. B2, p 〈 0. 05 and R1 vs. R2 p 〈 0. 05 respectively), but there were no significant differences of intraventricular hemorrhage and neonatal necrotizing enterocolitis (NEC) between two groups (p 〉 0. 03 ). Conclusion Corticosteroid could reduce the morbidity and mortality of hyaline membrane disease ( HMD), and could help to prevent BPD and ROP. Howerver, corticosteroid might have negative effects on physical development and humoral immunity.

  6. 7 CFR 29.2290 - Premature primings.

    Science.gov (United States)

    2010-01-01

    ... 7 Agriculture 2 2010-01-01 2010-01-01 false Premature primings. 29.2290 Section 29.2290 Agriculture Regulations of the Department of Agriculture AGRICULTURAL MARKETING SERVICE (Standards... 21) § 29.2290 Premature primings. Ground leaves harvested before reaching complete growth and...

  7. 28 CFR 51.22 - Premature submissions.

    Science.gov (United States)

    2010-07-01

    ... 28 Judicial Administration 2 2010-07-01 2010-07-01 false Premature submissions. 51.22 Section 51.22 Judicial Administration DEPARTMENT OF JUSTICE (CONTINUED) PROCEDURES FOR THE ADMINISTRATION OF... § 51.22 Premature submissions. The Attorney General will not consider on the merits: (a) Any proposal...

  8. [Premature rupture of membranes and chorioamnionitis].

    Science.gov (United States)

    Lopez Garcia, R

    1988-01-01

    Despite advances in perinatal medicine in the past decade, the diagnosis and treatment of premature rupture of membranes remain controversial. Premature rupture occurs in 2.7-7.0% of pregnancies and most cases occur spontaneously without apparent cause. The disparity in reported rates of premature rupture is due to differences in the definition and diagnostic criteria for premature rupture and lack of comparability in the populations studied. Mexico's National Institute of Perinatology has adopted the definition of the American COllege of Gynecology and Obstetrics which views premature rupture as that occurring before regular uterine contractions that produce cervical dilation. 8.8% of its patients have premature rupture according to this definition. 20% of cases occur before the 36th week of pregnancy. Treatment of rupture occurring before 37 weeks must balance the threat of amniotic infection with the dangers of premature birth. Infections appear more common in low income patient populations. Chorioamnionitis is a serious complication of pregnancy and is the main argument against conservative treatment of premature rupture. The rate of maternal infection is directly related to the time elapsing between rupture of the membranes and birth. The rate increases after the 1st 24 hours and is at least 10 times higher after 72 hours. But recent studies suggest that there is no considerable increase in infection if vaginal explorations are avoided and careful techniques are used in treating the patient. Those who advise conservative treatment believe that prenatal outcomes are better because respiratory disease syndrome due to prematurity is avoided. Conservative management requires a white cell count at least every 24 hours and measurement of pulse, maternal temperature, and fetal heart rate ideally every 4 hours. Perinatal mortality rates due to premature rupture of membranes range from 2.5-50%. The principal causes are respiratory disease syndrome, infection, asphyxia

  9. Human milk for the premature infant

    Science.gov (United States)

    Underwood, Mark A.

    2012-01-01

    Synopsis Premature infants are a heterogeneous group with widely differing needs for nutrition and immune protection with risk of growth failure, developmental delays, necrotizing enterocolitis, and late-onset sepsis increasing with decreasing gestational age and birth weight. Human milk from women delivering prematurely has more protein and higher levels of many bioactive molecules compared to milk from women delivering at term. Human milk must be fortified for small premature infants to achieve adequate growth. Mother’s own milk improves growth and neurodevelopment and decreases the risk of necrotizing enterocolitis and late-onset sepsis and should therefore be the primary enteral diet of premature infants. Donor milk is a valuable resource for premature infants whose mothers are unable to provide an adequate supply of milk, but presents significant challenges including the need for pasteurization, nutritional and biochemical deficiencies and a limited supply. PMID:23178065

  10. A Case of Extensive polyostotic fibrous dysplasia

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    Lee, Byung Do [Dept. of Oral and Maxillofacial Radiology, School of Dentistry, Wonkwang University, Iksan (Korea, Republic of); Hwang, Eui Hwan; Lee, Sang Rae [Dept. of Oral and Maxillofacial Radiology, School of Dentistry, Kyunghee University, Seoul (Korea, Republic of)

    2000-06-15

    Fibrous dysplasia is a benign disorder of bone consisting of intramedullary proliferation of fibrous tissue and irregularly distributed, poorly developed bone. The disease manifests itself in the monostotic form in which only one bone is involved and the polyostotic form in which multiple bones at different sites are affected. We reported a extensive case of polyostotic fibrous dysplasia with involvement of craniofacial bones, mandible, ribs, extremities. A 18-year-old man showed remarkable right facial swelling who had been treated on right femur 3 years ago with a bone graft for pathologic fracture and he recognized facial swelling 5 years ago. Extraoral radiograms and computed tomogram showed diffuse sclerosis with a ground glass appearance of the most calvarial bones, facial bones. The right mandibular lesion showed very expansible lesion with mottled appearance. Bone scans showed multifocal increased uptakes in craniofacial bones, right mandible, bilaterally in ribs, humerus, femur, tibia and characteristic various deformity of right femur (shepherd's crook deformity). This case showed exceptionally bilateral, extensive nature of bone lesion and didn't show any features of skin pigmentation and endocrine disturbances.

  11. An extremely rare case: osteosclerotic metaphyseal dysplasia.

    Science.gov (United States)

    Kasapkara, C S; Küçükçongar, A; Boyunağa, O; Bedir, T; Oncü, F; Hasanoğlu, A; Tümer, L

    2013-01-01

    OMD (osteosclerotic metaphyseal dysplasia) is a very rare sclerosing bone disorder, first described by G. Nishimura in two Japanese siblings in 1993 (6). We report the case of a 12-month-old male with hypotonia, developmental delay and sclerosis of the metaphyses and epiphyses of specific bones. This 36-week gestation boy was born to a 26 year old gravida 5 para 1 Turkish mother and a 27 year old nonconsanguineous father. Radiographic findings obtained during the hospital stay included bilateral symmetrical osteosclerosis of the metaphyseal portions of the long bones in the upper and lower extremities with osteopenic shafts. Narrow bands of metaphyseal osteosclerosis were detected in the short tubular bones of the hands and feet. Growing parts of bilateral scapula, iliac, pubic and ischial bones show sclerotic bands. In addition superior and inferior plates of vertebras, transverse processes of sacral vertebras, all visible epiphyses, carpal and tarsal bones also show sclerotic changes. The scalp was unaffected. Based on the clinical, radiographic, and laboratory findings, a diagnosis of OMD was made. We do not know any of the osteosclerotic bone disorder with changes including hypotonia, mental and motor developmental delay and metaphyseal sclerosis of the bones with a unique distribution except OMD. The syndrome is characterized by developmental delay of a progressive nature, hypotonia, elevated alkaline phosphatase, and late-onset spastic paraplegia 18 years ago. Our patient is the 4th case of OMD described in the literature share some clinical and radiological similarities with other three reported cases of osteosclerotic metaphyseal dysplasias.

  12. Hemispheric dysplasia and hemimegalencephaly: imaging definitions.

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    Santos, Antonio Carlos; Escorsi-Rosset, Sara; Simao, Gustavo N; Terra, Vera C; Velasco, Tonicarlo; Neder, Luciano; Sakamoto, Americo C; Machado, Helio R

    2014-11-01

    Hemispheric dysplasia (HD) and hemimegalencephaly (HME) are both brain malformations with early clinical manifestation including developmental delay and intractable epilepsy. Sometimes the differentiation of these conditions is not simple. HME is an anomaly of cortical development caused by a combination of neural proliferation and cell migration dysfunction, showing lobar or hemispheric enlargement. On the other hand, HD shows no brain hypertrophy, and even brain atrophy, eventually. To compare both conditions, we reviewed clinical, MRI, and histopathology of 23 patients with developmental delay and refractory epilepsy treated with hemispheric surgery. Histologically, both groups presented polymicrogyria, focal cortical dysplasia, gray matter (GM) heterotopia, pachygyria, and agyria. The white matter (WM) showed different degrees of gliosis and myelin impairment. Even though with no specificity in histopathology, the degree of lesion was more impressive on HME. The combination of WM dysmyelination and hypertrophy leads to the so called hamartomatous appearing. Although not all HME showed brain enlargement and some HD might show no size changes or atrophy, the size of affected hemisphere and the hamartomatous appearance of the WM were the more relevant signs to differentiate both conditions. Brain MRI was the best diagnostic tolls because it allowed together high contrast resolution, whole brain coverage and spatial distribution analysis. HD and HMD showed brain asymmetry tendency, but in opposite directions. The size of affected hemisphere and the hamartomatous appearance of the WM were the more relevant signs to differentiate both conditions.

  13. Placental Mesenchymal Dysplasia: A Case Report

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    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. A Case of Placental Mesenchymal Dysplasia

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    Shigeki Taga

    2013-01-01

    Full Text Available Placental mesenchymal dysplasia (PMD rarely complicates with pregnancy. A 30-year-old woman, gravida 3, para 3, presenting with placentomegaly, was referred to our department at 18 weeks of gestation. An ultrasonography revealed a normal fetus with a large multicystic placenta, measuring 125 × 42 × 80 mm. The border between the lesion and normal region was not clear. Color doppler revealed little blood flow in the lesion. Magnetic resonance imaging revealed normal fetus and a large multicystic placenta. Serum human chorionic gonadotropin level was 20124.97 U/L, which was normal at 20 weeks of gestation. Thus, placental mesenchymal dysplasia rather than hydatidiform mole with coexistent fetus was suspected. Then, routine checkup was continued. Because she had the history of Cesarean section, an elective Cesarean section was performed at 37 weeks of gestation, and 2520 g female infant with apgar score 8/9 was delivered. The baby was normal with no evidence of Beckwith-Wiedemann syndrome. Placenta of 20 × 16 × 2 cm, weighing 720 g, was bulky with grape like vesicles involving whole placenta. Microscopic examination revealed dilated villi and vessels with thick wall which was lacking trophoblast proliferation. Large hydropic stem villi with myxomatous struma and cistern formation were seen. PMD was histopathologically confirmed.

  15. T1/ST2 promotes T helper 2 cell activation and polyfunctionality in bronchopulmonary mycosis.

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    Piehler, D; Grahnert, A; Eschke, M; Richter, T; Köhler, G; Stenzel, W; Alber, G

    2013-03-01

    Interleukin (IL)-33 enhances T helper (Th)2 immunity via its receptor T1/ST2. Infection with the yeast-like pathogen Cryptococcus neoformans is usually controlled by a Th1-mediated immune response. The mechanisms responsible for nonprotective Th2 immunity leading to allergic inflammation in pulmonary cryptococcosis are still not fully understood. Using a murine pulmonary model of C. neoformans infection, we report that T1/ST2 expression correlates with the intensity of Th2 activation, as demonstrated by the expression of CD25 and CD44 and downregulation of CD62L. Antigen-specific T1/ST2(+) Th cells are the primary source of the Th2 cytokines IL-5 and IL-13 as compared with wild-type T1/ST2(-) Th cells or Th cells from T1/ST2(-/-) mice. In addition, T1/ST2(+) Th cells almost exclusively contain bi- and trifunctional Th2 cytokine-producing Th cells compared with T1/ST2(-) Th cells or Th cells from T1/ST2(-/-) mice. Finally, T1/ST2-driven Th2 development resulted in defective pulmonary fungal control. These data demonstrate that T1/ST2 directs Th2 cell activation and polyfunctionality in allergic bronchopulmonary mycosis.

  16. A questionnaire-based study on the role of environmental factors in allergic bronchopulmonary aspergillosis

    Directory of Open Access Journals (Sweden)

    Ritesh Agarwal

    2014-01-01

    Full Text Available Background and Aims: Allergic bronchopulmonary aspergillosis (ABPA is an immunological disorder caused by hypersensitivity against Aspergillus fumigatus. The pathogenesis of ABPA remains unknown. Few studies have investigated the role of environmental factors in pathogenesis of ABPA. Herein, we investigate the role of environmental factors in ABPA. Materials and Methods: In this prospective case-control study, consecutive patients with asthma (Aspergillus sensitized and unsensitized and ABPA were investigated using a standardized questionnaire to enquire into their demographic characteristics, clinical details, exposure to organic matter and living conditions (home environment, presence of moisture in the walls, and others. Asthma severity and control was assessed using the 2002 The Global Initiative for Asthma (GINA recommendations and asthma control test, respectively. Results: During the study period, 202 subjects of asthma (103 and 99 Aspergillus unsensitized and sensitized asthma, respectively and 101 ABPA with a mean (SD age of 35.3 (14.7 years were included. The baseline characteristics were similar in the two groups except for a higher prevalence of severe persistent asthma in the ABPA group (79% vs. 44%, P = 0.0001. No significant differences in environmental factors were noted in the ABPA population compared to asthmatic patients except for a higher rural residence in ABPA (47% vs. 66%, P = 0.007. Conclusions: The study found no significant environmental differences in ABPA compared to asthmatic patients. It is likely that environmental factors are not the primary pathogenetic factors in causation of ABPA.

  17. IL-4 alpha chain receptor (IL-4Rα polymorphisms in allergic bronchopulmonary sspergillosis

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    Consolino Judy D

    2006-02-01

    Full Text Available Abstract Background Allergic bronchopulmonary aspergillosis occurs in 7–10% of cystic fibrosis (CF and 1–2% of asthmatic patients. HLA-DR restriction and increased sensitivity to IL-4 stimulation have been proposed as risk factors in these populations. Objective We examined for the presence of IL-4 receptor alpha chain (IL-4Rα single nucleotide polymorphisms (SNPs in ABPA and whether these accounted for increased sensitivity to IL-4 stimulation. Methods One extracellular (ile75val and four cytoplasmic IL-4Rα SNPs were analyzed in 40 CF and 22 asthmatic patients and in 56 non-ABPA CF and asthmatic patients. Sensitivity to IL-4 stimulation was measured by induction of CD23 expression on B cells. Results IL-4Rα SNPs were observed in 95% of ABPA patients. The predominant IL-4Rα SNP was the extracellular IL-4Rα SNP, ile75val, observed in 80% of ABPA patients. Conclusion The presence of IL-4Rα SNPs, principally ile75val, appears to be a genetic risk for the development of ABPA.

  18. Non-adenomatous forms of gastro-oesophageal epithelial dysplasia: an under-recognised entity?

    Science.gov (United States)

    Serra, Stefano; Chetty, Runjan

    2014-10-01

    Foveolar dysplasia is an uncommon form of dysplasia that is encountered in the stomach and oesophagus in the context of Barrett’s oesophagus. Glands displaying foveolar dysplasia also show architectural abnormalities that are similar to those encountered in adenomatous dysplasia. However, from a cytological point of view, foveolar dysplasia glands are lined by low-cuboidal to columnar epithelium, the cytoplasm is often clear with round-to-oval nuclei. Nuclear stratification as seen in adenomatous dysplasia is not common, although there is loss of nuclear polarity, pleomorphism and mitotic activity. It is important to distinguish low-grade foveolar dysplasia from regenerative change.

  19. Premature Ventricular Complexes and Premature Ventricular Complex Induced Cardiomyopathy.

    Science.gov (United States)

    Latchamsetty, Rakesh; Bogun, Frank

    2015-09-01

    Presentation, prognosis, and management of premature ventricular complexes (PVCs) vary significantly among patients and depend on PVC characteristics as well as patient comorbidities. Presentation can range from incidental discovery in an asymptomatic patient to debilitating heart failure. Prognosis depends on, among other factors, the presence or absence of structural heart disease, PVC burden and other factors detailed in this review. Our understanding of the clinical significance of frequent PVCs, particularly as it relates to development of cardiomyopathy, has advanced greatly in the past decade. In this article, we explore the mechanisms governing PVC initiation and discuss prevalence and frequency of PVCs in the general population. We also explore prognostic implications based on PVC frequency as well as the presence or absence of underlying heart disease. We then take a focused look at PVC-induced cardiomyopathy and identify predictors for developing cardiomyopathy. Finally, we discuss clinical evaluation and management of patients presenting with frequent PVCs. Management can include clinical observation, addressing reversible causes, lifestyle modification, pharmacotherapy, or catheter ablation.

  20. Demographics of hip dysplasia in the Maine Coon cat.

    Science.gov (United States)

    Loder, Randall T; Todhunter, Rory J

    2017-04-01

    Objectives The aim of this study was to study the demographics of feline hip dysplasia (FHD) in the Maine Coon cat. Methods The complete hip dysplasia registry (public and private) collected by the Orthopedic Foundation for Animals through April 2015 was accessed. There were 2732 unique cats; 2708 (99.1%) were Maine Coons, and only these were studied. Variables analyzed were sex, month/season of birth and hip dysplasia score. Two groups were created: those with and without FHD. P dysplasia were older. The percentage of bilateral FHD was 56%, and bilateral cases had more severe dysplasia than unilateral cases but with no age difference. Month/season of birth or geographic region of origin did not influence the prevalence of FHD. Conclusions and relevance This is the largest demographic study of FHD in the Maine Coon cat. The overall prevalence in the Orthopedic Foundation for Animals registry was 24.9%, and slightly higher in males (27.3%) than females (23.3%). Dysplasia was more severe in bilateral than unilateral cases and with increasing age. Caution should be used when extrapolating these findings to other feline breeds or other groups of Maine Coon cats. Further studies need to be performed among other breeds and geographic locations to better understand the demographics of feline hip dysplasia.

  1. 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.

  2. Psychosocial interventions for premature ejaculation

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    Tamara Melnik

    Full Text Available BACKGROUND: Premature ejaculation (PE is a very common sexual dysfunction among patients, and with varying prevalence estimates ranging from 3% to 20%. Although psychological issues are present in most patients with premature PE, as a cause or as a consequence, research on the effects of psychological approaches for PE has in general not been controlled or randomised and is lacking in long-term follow up. OBJECTIVE: To assess the efficacy of psychosocial interventions for PE. CRITERIA FOR CONSIDERING STUDIES FOR THIS REVIEW: Trials were searched in computerized general and specialized databases, such as: MEDLINE by PubMed (1966 to 2010; PsycINFO (1974 to 2010; EMBASE (1980 to 2010; LILACS (1982 to 2010; the Cochrane Central Register of Controlled Trials (Cochrane Library, 2010; and by checking bibliographies, and contacting manufacturers and researchers. SELECTION CRITERIA: Randomised or quasi-randomised controlled trials evaluating psychosocial interventions compared with different psychosocial interventions, pharmacological interventions, waiting list, or no treatment for PE. DATA COLLECTION AND ANALYSIS: Information on patients, interventions, and outcomes was extracted by at least two independent reviewers using a standard form. The primary outcome measure for comparing the effects of psychosocial interventions to waiting list and standard medications was improvement in IELT (i.e., time from vaginal penetration to ejaculation. The secondary outcome was change in validated PE questionnaires. MAIN RESULTS: In one study behavioral therapy (BT was significantly better than waiting list for duration of intercourse (MD (mean difference 407.90 seconds, 95% CI 302.42 to 513.38, and couples' sexual satisfaction (MD -26.10, CI -50.48 to -1.72. BT was also significantly better for a new functional-sexological treatment (FS (MD 412.00 seconds, 95% CI 305.88 to 518.12, change over time in subjective perception of duration of intercourse (Women: MD 2

  3. Rapidly fatal "congenital lung dysplasia": a case report and review of the literature.

    Science.gov (United States)

    Don, Massimiliano; Orsaria, Maria; Da Dalt, Eva; Tringali, Carmela; Sacher, Bruno

    2014-04-01

    Acinar dysplasia congenital alveolar dysplasia and alveolar capillary dysplasia with misalignment of pulmonary veins belong to the diffuse developmental disorders (congenital lung dysplasia), very rare fatal disorders of infancy that occur early in lung development. A case of quickly fatal congenital lung dysplasia in a full-term infant is presented and underlines the necessity to suspect this disease in a newborn suffering from severe and refractory respiratory distress.

  4. Radiological features of bilateral hereditary micro-epiphyseal dysplasia - a distinct entity in the skeletal dysplasias

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    Mostert, A.K. [Isala Clinics, Location Weezenlanden, Dept. of Orthopaedic Surgery, Zwolle (Netherlands); Dijkstra, P.F. [Jan van Breemen Inst., Dept. of Radiology, Amsterdam (Netherlands); Horn, J.R. van [Univ. Hospital Groningen, Dept. of Orthopaedic Surgery, Groningen (Netherlands); Jansen, B.R.H. [Reinier de Graaf Hospital, Dept. of Orthopaedic Surgery, Delft (Netherlands); Heutink, P. [Erasmus MCRotterdam, Dept. of Clinical Genetics, Rotterdam (Netherlands); Lindhout, D. [Univ. Medical Centre Utrecht, Dept. of Medical Genetics, Utrecht (Netherlands)

    2002-07-01

    Aim: To prove that bilateral hereditary micro-epiphyseal dysplasia (BHMED), first described by Elsbach in 1959, is a distinct disorder radiologically as well as clinically, compared with multiple epiphyseal dysplasia (MED). Material and Methods: We used the data of the revised pedigree with 84 family members, performed a medical history, physical examination and made a radiological evaluation for defining a clinical and radiological phenotype of BHMED family members. We used blood samples for genetic analysis. Results: Although there is a clear clinical picture of the dysplasia, the radiological signs are more reliable for making the diagnosis. Especially the typical deformity of the hip and knee joint are diagnostic for BHMED. By linkage analysis we excluded linkage with the three known MED-loci (EDM1, EDM2 and EDM3). Conclusion: BHMED is indeed an entity that is distinct from common multiple epiphyseal dysplasia (MED), clinically, as well as radiologically and genetically. (orig.) [German] Ziel: Es sollte dargelegt werden, dass sich eine vererbliche, laterale Mikro-Epiphysendysplasie (BHMED), Erstbeschreibung durch Elsbach 1959, klinisch, radiologisch und genetisch von einer mutiplen Epiphysendysplasie (MED) unterscheidet. Material und Methode: Anhand der Daten eines ueberarbeiteten Stammbaumes mit 84 Familienmitgliedern wurde der medizinische Werdegang rekonstruiert. Es erfolgte eine physische Untersuchung der Familienmitglieder. Schliesslich wurde eine radiologische Auswertung durchgefuehrt, um einen klinischen und radiologischen Phaenotyp der von BHMED betroffenen Familienmitglieder zu definieren. Fuer eine genetische Analyse wurden Blutproben entnommen. Ergebnisse: Obwohl es ein deutliches klinisches Bild einer Dysplasie gibt, sind die radiologischen Kennzeichen fuer die Diagnose zuverlaessiger. Insbesondere die typische Deformation der Huefte und des Kniegelenks ist diagnostisch fuer BHMED. Durch Linkage-Analyse konnte eine Verbindung zu den drei bekannten

  5. Fibrous Dysplasia versus Juvenile Ossifying Fibroma: A Dilemma.

    Science.gov (United States)

    Nair, Sreelakshmi N; Kini, Raghavendra; Rao, Prasanna Kumar; Bhandarkar, Gowri P; Kashyp, Roopashri Rajesh; Rai, Manjunath; Naik, Neel; Santhosh, Athul

    2016-01-01

    Fibrous dysplasia (FD) is a condition characterized by excessive proliferation of bone forming mesenchymal cells which can affect one bone (monostotic type) or multiple bones (polyostotic type). It is predominantly noticed in adolescents and young adults. Fibrous dysplasia affecting the jaws is an uncommon condition. The most commonly affected facial bone is the maxilla, with facial asymmetry being the chief complaint. The lesion in many instances is confused with ossifying fibroma (OF). Diagnosis of these two lesions has to be done based on clinical, radiographic, and microscopic findings. Here, we present a case of fibrous dysplasia of maxilla in a nine-year-old boy mimicking juvenile ossifying fibroma.

  6. [Florid cemento-osseous dysplasia of the jaws].

    Science.gov (United States)

    Benazzou, S; Boulaadas, M; El Ayoubi, A; Nazih, N; Essakalli, L; Kzadri, M

    2011-06-01

    Florid cemento-osseous dysplasia is a benign and rare tumor of the jaws. It is more commonly seen in middle-aged black women. Most cases are asymptomatic and are found during routine radiographic examination. We report two complicated cases of florid cemento-osseous dysplasia, one with facial deformity and the other with chronic osteitis. The diagnosis of florid cemento-osseous dysplasia is based on clinical and radiological features. The lesions are commonly bilateral and symmetrical. Copyright © 2011. Published by Elsevier Masson SAS.

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

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    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.

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

    Science.gov (United States)

    Bhandari, Rajat; Sandhu, Simarpreet V; Bansal, Himanta; Behl, Rashi; Bhullar, Ramanpreet Kaur

    2012-04-01

    Focal cemento-osseous dysplasia (FCOD) is a benign fibroosseous condition that can be seen in dentulous and edentulous patients. It is an asymptomatic lesion and needs no treatment; however, follow-up is essential due to the possibility that it can progress to a condition called florid cemento-osseous dysplasia. We report a case of FCOD of mandible in a 25-year-old female. Clinically, the lesion resembled periapical pathosis of odontogenic origin. An attempt has been made to discuss the clinical and histopathologic features along with differential diagnosis of cemento-osseous dysplasia.

  9. Fibrous Dysplasia versus Juvenile Ossifying Fibroma: A Dilemma

    Science.gov (United States)

    Rao, Prasanna Kumar; Bhandarkar, Gowri P.; Rai, Manjunath; Naik, Neel; Santhosh, Athul

    2016-01-01

    Fibrous dysplasia (FD) is a condition characterized by excessive proliferation of bone forming mesenchymal cells which can affect one bone (monostotic type) or multiple bones (polyostotic type). It is predominantly noticed in adolescents and young adults. Fibrous dysplasia affecting the jaws is an uncommon condition. The most commonly affected facial bone is the maxilla, with facial asymmetry being the chief complaint. The lesion in many instances is confused with ossifying fibroma (OF). Diagnosis of these two lesions has to be done based on clinical, radiographic, and microscopic findings. Here, we present a case of fibrous dysplasia of maxilla in a nine-year-old boy mimicking juvenile ossifying fibroma. PMID:28101383

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

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    Oh, Jung Seok; Ahn Kook Jin; Kim, Jee Young; Lee, Sun Jin; Park, Jeong Mi [Catholic University Yeouido St. Mary' s Hospital, College of Medicine, Seoul (Korea, Republic of)

    2010-06-15

    Recent advances in MRI have revealed congenital brain malformations and subtle developmental abnormalities of the cerebral and cerebellar cortical architecture. Typical cerebellar cortical dysplasia as a newly categorized cerebellar malformation, has been seen in patients with Fukuyama congenital muscular dystrophy. Cerebellar cortical dysplasia occurs at the embryonic stage and is often observed in healthy newborns. It is also incidentally and initially detected in adults without symptoms. To the best of our knowledge, cerebellar dysplasia without any related disorders is very rare. We describe the MRI findings in one patient with disorganized foliation of both cerebellar hemispheres without a related disorder or syndrome

  11. Ectodermal dysplasias associated with clefting: significance of scalp dermatitis.

    Science.gov (United States)

    Fosko, S W; Stenn, K S; Bolognia, J L

    1992-08-01

    Several clinical syndromes are characterized by ectodermal dysplasia (ED) in association with clefting of the lip and/or palate. The three most commonly recognized entities are (1) the EEC syndrome (ectodermal dysplasia, ectrodactyly, cleft lip/palate); (2) the Rapp-Hodgkin syndrome with ectodermal dysplasia, cleft lip/palate, and mid facial hypoplasia; and (3) the Hay-Wells or AEC syndrome (ankyloblepharon, ectodermal defects, cleft lip/palate). The clinical characteristics of these entities as well as several less common syndromes are reviewed and summarized. The presence of scalp dermatitis in patients with the AEC syndrome and less often the Rapp-Hodgkin syndrome is emphasized.

  12. Ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome

    Directory of Open Access Journals (Sweden)

    Reema Sharma Dhar

    2014-01-01

    Full Text Available Ectrodactyly-ectodermal dysplasia-cleft (EEC syndrome is an autosomal dominant disorder characterized by the triad of ectrodactyly-ectodermal dysplasia, and facial clefting along with some associated features. Presence of all the three major features in a single individual is extremely rare. We report a case of 4 year 11 months old child with EEC syndrome having ectodermal dysplasia-cleft lip and cleft palate and ectrodactyly with some associated features. Clinical features, diagnosis and role of a dentist in the multidisciplinary treatment approach have been elaborated in this case report.

  13. Effects of caffeine use in apnea of prematurity in the early stage versus late stage:A meta-analysis%早期或晚期咖啡因治疗早产儿呼吸暂停临床效果的 Meta 分析

    Institute of Scientific and Technical Information of China (English)

    李贤伟; 周红瀛; 李静

    2016-01-01

    Objective To assess the effects of caffeine use in apnea of prematurity (AOP) in the early stage (caffeine was used within 3 days after birth) versus late stage (caffeine was used 4 to 10 days after birth ) . Methods Medline, Science Direct, Elsevier, Embase, CBMdisc and Wanfang databases were retrieved to incorporate studies that met the inclusion criteria. The retrieval time limit was from the establishment of the databases to November, 2015. References that were in line with the inclusion criteria were selected and relevant conference data were collected by manual retrieval. Two researchers conducted meta-analysis using software RevMan 5. 2 after independent data selection, information extraction and quality evaluation according to the inclusion and exclusion criteria. Results One randomized controlled trial and four retrospective cohort studies were included. A total of 59 288 patients were involved. Meta-analysis suggested that, compared with caffeine use in the late stage, the early stage group showed significant lower incidence ( P < 0. 05 ) of mortality, bronchopulmonary dysplasia, intraventricular hemorrhage, periventricular leukomalacia, retinopathy of prematurity and surgical intervention of patent ductus arteriosus. No significant difference (OR = 0. 98, 95% CI 0. 72 - 1. 33, P = 0. 881) was observed between the 2 groups in terms of the incidence of necrotic enterocolitis. Conclusions Patients with AOP should start caffeine treatment as early as possible, which significantly decreases mortality rate and the occurrence of other complications with little adverse reactions and good clinical tolerance.%目的:对早期或晚期咖啡因治疗早产儿呼吸暂停的临床疗效进行 Meta 分析。方法检索 Medline、Science Direct、Elsevier、Embase、中国生物医学文献数据库、万方数据库等,收集符合纳入标准的研究,检索时限为从建库至2015年11月,提取符合纳入标准研究的参考文献和手工检索相关会

  14. Reducing premature KCC2 expression rescues seizure susceptibility and spine morphology in atypical febrile seizures.

    Science.gov (United States)

    Awad, Patricia N; Sanon, Nathalie T; Chattopadhyaya, Bidisha; Carriço, Josianne Nunes; Ouardouz, Mohamed; Gagné, Jonathan; Duss, Sandra; Wolf, Daniele; Desgent, Sébastien; Cancedda, Laura; Carmant, Lionel; Di Cristo, Graziella

    2016-07-01

    Atypical febrile seizures are considered a risk factor for epilepsy onset and cognitive impairments later in life. Patients with temporal lobe epilepsy and a history of atypical febrile seizures often carry a cortical malformation. This association has led to the hypothesis that the presence of a cortical dysplasia exacerbates febrile seizures in infancy, in turn increasing the risk for neurological sequelae. The mechanisms linking these events are currently poorly understood. Potassium-chloride cotransporter KCC2 affects several aspects of neuronal circuit development and function, by modulating GABAergic transmission and excitatory synapse formation. Recent data suggest that KCC2 downregulation contributes to seizure generation in the epileptic adult brain, but its role in the developing brain is still controversial. In a rodent model of atypical febrile seizures, combining a cortical dysplasia and hyperthermia-induced seizures (LHS rats), we found a premature and sustained increase in KCC2 protein levels, accompanied by a negative shift of the reversal potential of GABA. In parallel, we observed a significant reduction in dendritic spine size and mEPSC amplitude in CA1 pyramidal neurons, accompanied by spatial memory deficits. To investigate whether KCC2 premature overexpression plays a role in seizure susceptibility and synaptic alterations, we reduced KCC2 expression selectively in hippocampal pyramidal neurons by in utero electroporation of shRNA. Remarkably, KCC2 shRNA-electroporated LHS rats show reduced hyperthermia-induced seizure susceptibility, while dendritic spine size deficits were rescued. Our findings demonstrate that KCC2 overexpression in a compromised developing brain increases febrile seizure susceptibility and contribute to dendritic spine alterations. Copyright © 2016 Elsevier Inc. All rights reserved.

  15. Sexuality in pregnancy and premature labour.

    Science.gov (United States)

    Georgakopoulos, P A; Dodos, D; Mechleris, D

    1984-09-01

    The relation of sexual behaviour during pregnancy to the initiation of labour was investigated in 358 patients of whom 58 were delivered after premature labour and 300 were delivered spontaneously at term. In all patients the mean weekly coital frequency and the frequency of orgasm were investigated by means of a retrospective questionnaire. There was no significant difference in coital or orgasmic frequency between the women who had a premature labour and those who had a spontaneous delivery at term. This was also true when those having premature labour were divided into those starting labour with ruptured membranes and those starting with contractions.

  16. Acute appendicitis in a premature baby

    Energy Technology Data Exchange (ETDEWEB)

    Beluffi, Giampiero; Alberici, Elisa [Department of Radiodiagnosis, Section of Paediatric Radiology, IRCCS Policlinico S. Matteo, Piazzale Golgi 2, 27100 Pavia PV (Italy)

    2002-07-01

    A case of acute appendicitis in a premature baby in whom diagnosis was suggested on plain films of the abdomen is presented. In this baby air in a hollow viscus suspected of being an enlarged appendix was the clue to diagnosis. The diagnostic dilemma of this rare and life-threatening condition in premature babies and newborns is underlined. The relevance of different imaging modalities and of different findings in this age group is discussed. Awareness of this rare condition and possible differential diagnosis in newborns and premature babies is stressed. (orig.)

  17. [Premature newborn: a case presentation].

    Science.gov (United States)

    Pastor Rodríguez, Jesús David; Pastor Bravo, María Del Mar; López García, Visitación; Cotes Teruel, María Isabel; Mellado, Jesús Eulogio; Cárceles, José Jara

    2010-01-01

    A case is presented of a premature newborn of 27 weeks gestation and weighing 420 grams who was delivered as a result of a maternal pre-eclampsia and retarded intra-uterine growth. During the 125 days of hospitalisation, an individual care plan based on the Virginia Henderson model was devised and applied to both the child and her parents using NANDA diagnostics, interventions according to the NIC classification, and the expected results according to the NOC classification. The Marjory Gordon functional patterns were used for the initial assessment. By applying the pre-term newborn (PTNB) plan, all their needs were provided and were modified throughout the hospital stay, with new needs that were added to the established ones. These required a continuous assessment with the subsequent adapting of the care plan. Likewise, the care required by the parents varied from the initial grief due to the possible loss of their child to learning the alarm signs and the home care that their child would need. The child was finally discharged weighing 2900 grams and with normal neurological and psychomotor development, although with a lower weight appropriate to her age. Currently, at 2 years old, the child has a normal neurological and psychomotor development, but with weight and size lower than the P(3) percentile. She requires speech therapy treatment due to paralysis of the right vocal cord.

  18. Psychosexual therapy for premature ejaculation.

    Science.gov (United States)

    Althof, Stanley E

    2016-08-01

    Premature ejaculation (PE) is a male sexual dysfunction that creates considerable anguish for the man, his partner and their relationship. PE is not one disorder but includes the four subtypes (lifelong, acquired, natural and subjective) each with unique psychological concerns and issues. Psychological treatment for men and couples with PE addresses sexual skills/techniques but also focuses on issues of self-esteem, performance anxiety and interpersonal conflict. The outcome studies for psychotherapy alone are difficult to interpret and compare because of poor methodological design (lack of control groups, small sample size, poor outcome measures and lack of follow-up). However, the few studies that surmount these methodological hurdles suggest that psychological intervention offers men and couples a promising treatment option. Combination pharmaco- and psychotherapy is the most promising intervention for lifelong and acquired PE and offers superior efficacy to drug alone. This is because men and couples learn sexual skills, address the intrapsychic, interpersonal and cognitive issues that precipitate and maintain the dysfunction.

  19. The epidemiology of premature ejaculation.

    Science.gov (United States)

    Saitz, Theodore Robert; Serefoglu, Ege Can

    2016-08-01

    Vast advances have occurred over the past decade with regards to understanding the epidemiology, pathophysiology and management of premature ejaculation (PE); however, we still have much to learn about this common sexual problem. As a standardized evidence-based definition of PE has only recently been established, the reported prevalence rates of PE prior to this definition have been difficult to interpret. As a result, a large range of conflicting prevalence rates have been reported. In addition to the lack of a standardized definition and operational criteria, the method of recruitment for study participation and method of data collection have obviously contributed to the broad range of reported prevalence rates. The new criteria and classification of PE will allow for continued research into the diverse phenomenology, etiology and pathogenesis of the disease to be conducted. While the absolute pathophysiology and true prevalence of PE remains unclear, developing a better understanding of the true prevalence of the disease will allow for the completion of more accurate analysis and treatment of the disease.

  20. [Sexological intervention on premature ejaculation].

    Science.gov (United States)

    San Martín Blanco, C

    2014-07-01

    Strategies, recommendations and techniques proposed by sex therapy for intervention on premature ejaculation, have represented for nearly four decades the most effective model of intervention in this sexual dysfunction, which currently is complemented by the efficacy of dapoxetine drug treatment. Clinical experience and recent studies support that combined intervention offers the best therapeutic results. In addition in sex therapy, etiologic diagnosis is obtained from the analysis of the interrelationship of the couple. Diagnostic and therapeutic intervention has to be always centered in the relationship, so the techniques and resources must be applied with the expectation of being implemented in the sexual interaction. It will therefore be the relationship that receive treatment, even if medication is used for one of the members of the couple. On the other hand, this model of intervention can be implemented by a professional with training, although not necessarily a specialist. Copyright © 2014 Elsevier España, S.L.U. y Sociedad Española de Medicina Rural y Generalista (SEMERGEN). All rights reserved.

  1. Medical therapy for premature ejaculation

    Science.gov (United States)

    Mohee, Amar; Eardley, Ian

    2011-01-01

    Premature ejaculation (PE) is a common male sexual dysfunction. Advances in PE research have been hampered owing to a nonstandardized definition of PE, until the definition by the International Society of Sexual Medicine (ISSM) in 2009. Once the diagnosis of PE is established through a thorough history, a variety of medical therapies is available, including tricyclic antidepressants, selective serotonin reuptake inhibitors (SSRIs), centrally acting opiates, phosphodiesterase 5 inhibitors and topical desensitizing creams. Most of these treatments increase the intravaginal ejaculation latency time (IELT) and patient satisfaction scores, with the most convincing evidence for SSRIs and topical creams. Daily SSRIs such as paroxetine, although efficacious, do have a substantial and prolonged side effect profile. Dapoxetine, which is a on-demand SSRI, is the only licensed drug for the treatment of PE, increasing IELT by a factor of 2.5 to 3 with limited and tolerable side effects. In the near future, the topical aerosol PSD502 is due to be licensed for the treatment of PE, increasing IELT by up to a factor of 6 but having minimal local and negligible systemic side effects. PMID:22046199

  2. Ceramide profile in hypohidrotic ectodermal dysplasia

    DEFF Research Database (Denmark)

    Jungersted, J. M.; Høgh, Julie Kaae; Hellgren, Lars

    2012-01-01

    Background. Hypohidrotic ectodermal dysplasia (HED) is a rare genetic disease. The clinical presentation includes lack of sweating ability, and an often widely spread dermatitis resembling atopic dermatitis (AD). In AD, the skin‐barrier defect is partly ascribed to the altered lipid profile...... in the stratum corneum and partly to mutations of the filaggrin genes. To our knowledge, no data are available about the epidermal lipid profile of HED. Aim. To compare the ceramide profile for patients with HED and AD. Methods. The ceramide profile and ceramide/cholesterol ratio were compared between patients...... with HED (n = 7) and patients with AD (n = 21), using cyanoacrylate to take biopsy samples from the stratum corneum. Lipids were extracted from the biopsies and analysed using high‐performance thin‐layer chromatography. Results. The lipid profiles of HED and AD were similar in distribution, apart from...

  3. Focal cemento-osseous dysplasia of mandible.

    Science.gov (United States)

    Cankaya, Abdülkadir Burak; Erdem, Mehmet Ali; Olgac, Vakur; Firat, Deniz Refia

    2012-09-03

    Fibro-osseous lesions are disturbances in bone metabolism in which normal bone is replaced by a connective tissue matrix that then gradually develops into cemento-osseous tissue. Typically, the lesion is asymptomatic and is detected on routine radiographic examination. Radiologically, this lesion has three stages of maturation: pure radiolucent, radiopaque/mixed radiolucent, and radiopaque appearance. During these stages the lesion can be misdiagnosed. In this case report a 69-year- old patient with a a complaint of painless swelling of the left mandibular molar and premolar area is presented along with a review of the differential diagnoses considered in order to reach a final diagnosis of focal cemento-osseous dysplasia.

  4. Photoreceptor cell dysplasia in two Tippler pigeons.

    Science.gov (United States)

    Moore, P A; Munnell, J F; Martin, C L; Prasse, K W; Carmichael, K P

    2004-01-01

    Two 12-week-old Tippler pigeons were evaluated for ocular abnormalities associated with congenital blindness. The pigeons were emaciated and blind. Biomicroscopy and direct and indirect ophthalmoscopy findings of the Tippler pigeons were normal with the exception of partially dilated pupils at rest. Scotopic (blue stimuli) and photopic monocular electroretinograms were extinguished in the blind Tippler pigeons. Histological and electron microscopy studies revealed reduced numbers of rods and cones, and an absence of the double cone complex. The photoreceptor cells' outer segments were absent, and the inner segments were short and broad. The number of cell nuclei in the outer and inner nuclear layers was decreased, and the internal and external plexiform layers were reduced in width. Photoreceptor cell endfeet with developing synaptic ribbons were present in the external plexiform layer. Inflammatory cell and subretinal debris was not seen. The electroretinographic, histopathological, and ultrastructural findings of the blind Tippler pigeons support the diagnosis of a photoreceptor cell dysplasia.

  5. MRI assessment of right ventricular dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Di Cesare, Ernesto [Department of Radiology, University of L' Aquila, via Vetoio 1, 67100, L' Aquila (Italy)

    2003-06-01

    Right ventricular dysplasia is a new entity of unknown origin in the classification of cardiomyopathies. Also known as arrhythmogenic right ventricular cardiomyopathy (ARVC) or arrhythmogenic right ventricular dysplasia, it is a disease of the heart muscle characterised by fibroadipose atrophy mainly involving the right ventricle and responsible for severe ventricular arrhythmias and sudden death also in young people. Magnetic resonance imaging provides evidence of ventricular dilatation at the outflow tract, thinning and thickening of the wall, diastolic bulging areas (especially located at the level of the right ventricle outflow tract) and fatty substitution of the myocardium mainly at the level of the right ventricle. Many radiologists erroneously consider the previously described fatty substitution as the main sign of ARVC, even though an evaluation of fat substitution alone may be a source of error for two reasons: firstly, because isolated areas of fatty replacement are not synonymous with ARVC since small non-transmural focal fatty areas of fat are also present in the normal patients; and secondly, because the MRI detection of fat may be overestimated due to partial-volume artefacts with normal subepicardial fat. Cardiac MRI can also be employed for the diagnosis of idiopathic right ventricular outflow tract tachycardia. Considering the evolutive nature of the disease, the non-invasiveness of MRI allows the follow-up of these patients and may be considered an excellent screening modality for the diagnosis of ARVC in family members. Finally, MRI can be employed in electrophysiological studies to locate the arrhythmogenic focus and reduce sampling errors. (orig.)

  6. The correlation between HIV seropositivity, cervical dysplasia, and HPV subtypes 6/11, 16/18, 31/33/35

    DEFF Research Database (Denmark)

    Tweddel, G; Heller, P; Cunnane, M

    1994-01-01

    342 per mm3 for the dysplasia group and 281 per mm3 for the patients without dysplasia. Patients with dysplasia did not differ significantly from patients without dysplasia in regard to risk factors for cervical dysplasia, including history of STD, tobacco use, multiple sexual partners, age at first...

  7. 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.

  8. 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.

  9. Prominent Intrapulmonary Bronchopulmonary Anastomoses and Abnormal Lung Development in Infants and Children with Down Syndrome.

    Science.gov (United States)

    Bush, Douglas; Abman, Steven H; Galambos, Csaba

    2017-01-01

    To determine the frequency of histologic features of impaired lung vascular and alveolar development and to identify the presence of intrapulmonary bronchopulmonary anastomoses (IBA) in infants and children who died with Down syndrome. A retrospective review of autopsy reports and lung histology from 13 children with Down syndrome (ages: 0-8 years) was performed. Histologic features of abnormal lung development were identified and semiquantified, including the presence of IBA. Three-dimensional reconstructions of IBA were also performed. Comparisons were made with 4 age-matched patients without Down syndrome with congenital heart defects who underwent autopsies during this time period. Of the 13 subjects with Down syndrome, 69% died from cardiac events, 77% had a congenital heart defect, and 46% had a clinical diagnosis of pulmonary hypertension. Lung histology from all subjects with Down syndrome demonstrated alveolar simplification, and 92% had signs of persistence of a double capillary network in the distal lung. The lungs from the subjects with Down syndrome frequently had features of pulmonary arterial hypertensive remodeling (85%), and prominent bronchial vessels and IBA were observed in all subjects with Down syndrome. These features were more frequent in subjects with Down syndrome compared with control subjects. Children with Down syndrome who died of cardiopulmonary diseases often have histologic evidence of impaired lung alveolar and vascular development, including the presence of prominent IBA and pulmonary hypertension. We speculate that children with Down syndrome are at risk for reduced lung surface area and recruitment of IBA, which may worsen gas exchange in subjects with Down syndrome. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Diagnostic performance of various tests and criteria employed in allergic bronchopulmonary aspergillosis: a latent class analysis.

    Directory of Open Access Journals (Sweden)

    Ritesh Agarwal

    Full Text Available AIM: The efficiency of various investigations and diagnostic criteria used in diagnosis of allergic bronchopulmonary aspergillosis (ABPA remain unknown, primarily because of the lack of a gold standard. Latent class analysis (LCA can provide estimates of sensitivity and specificity in absence of gold standard. Herein, we report the performance of various investigations and criteria employed in diagnosis of ABPA. METHODS: Consecutive subjects with asthma underwent all the following investigations Aspergillus skin test, IgE levels (total and A.fumigatus specific, Aspergillus precipitins, eosinophil count, chest radiograph, and high-resolution computed tomography (HRCT of the chest. We used LCA to estimate the performance of various diagnostic tests and criteria in identification of ABPA. RESULTS: There were 372 asthmatics with a mean age of 35.9 years. The prevalence of Aspergillus sensitization was 53.2%. The sensitivity and specificity of various tests were Aspergillus skin test positivity (94.7%, 79.7%; IgE levels>1000 IU/mL (97.1%, 37.7%; A.fumigatus specific IgE levels>0.35 kUA/L (100%, 69.3%; Aspergillus precipitins (42.7%, 97.1%; eosinophil count>1000 cells/µL (29.5%, 93.1%; chest radiographic opacities (36.1%, 92.5%; bronchiectasis (91.9%, 80.9%; and, high-attenuation mucus (39.7%, 100%. The most accurate criteria was the Patterson criteria using six components followed by the Agarwal criteria. However, there was substantial decline in accuracy of the Patterson criteria if components of the criteria were either increased or decreased from six. CONCLUSIONS: A.fumigatus specific IgE levels and high-attenuation mucus were found to be the most sensitive and specific test respectively in diagnosis of ABPA. The Patterson criteria remain the best diagnostic criteria however they have good veridicality only if six criteria are used.

  11. Bronchopulmonary Carcinoids causing Cushing Syndrome: Results from a Multicentric Study Suggesting a More Aggressive Behavior.

    Science.gov (United States)

    Lococo, Filippo; Margaritora, Stefano; Cardillo, Giuseppe; Filosso, Perluigi; Novellis, Pierluigi; Rapicetta, Cristian; Carleo, Francesco; Bora, Giulia; Cesario, Alfredo; Stefani, Alessandro; Rossi, Giulio; Paci, Massimiliano

    2016-03-01

    Cushing syndrome (CS) caused by bronchopulmonary carcinoids (BCs) is a very rare entity. The aim of this study was to revisit the features of a multicenter clinical series to identify significant prognostic factors. From January 2002 to December 2013, the clinical and pathological data of 23 patients (treated in five different institutions) were retrospectively reviewed. Survival analysis was performed to explore the relative weight of potential prognostic factors. Median age and male/female ratio were 48 years and 14/9, respectively. Most (> 80%) of the patients presented with CS-related symptoms at diagnosis. Tumor location was peripheral in 13 patients (57%) and central in 10 (43%). All patients but two (treated with chemotherapy) underwent surgical resection with curative intent. Definitive cyto/histology was indicative of typical carcinoid (TC) in 16 cases (70%) and atypical carcinoid (AC) in 7 cases (30%). A complete remission of CS was obtained in 16 cases (70%). Lymph nodal involvement was detected in 11 cases (48%), with N2 disease occurring in 7 (∼ 30% of all cases). Four patients (22%) experienced a relapse of the disease after radical surgery. Overall 5-year survival (long-term survival, LTS) was 60%, better in TCs when compared with AC (LTS: 66 v s. 48%, p = 0.28). Log-rank analysis identified ECOG performance status, cTNM and cN staging, pTNM and pN staging, persistence of CS and relapses (local p = 0.006; distant p = 0.001) as significant prognostic factors in this cohort of patients. BCs causing CS are characterized by a high rate of lymph-nodal involvement, a suboptimal prognosis (5-year survival = 60%, 66% in TCs) and a remarkable risk of relapse even after radical resection. Advanced stage, lymph-nodal involvement and the persisting of the CS after treatment correlate with a poor prognosis. Georg Thieme Verlag KG Stuttgart · New York.

  12. Global burden of allergic bronchopulmonary aspergillosis with asthma and its complication chronic pulmonary aspergillosis in adults.

    Science.gov (United States)

    Denning, David W; Pleuvry, Alex; Cole, Donald C

    2013-05-01

    Allergic bronchopulmonary aspergillosis (ABPA) complicates asthma and may lead to chronic pulmonary aspergillosis (CPA) yet global burdens of each have never been estimated. Antifungal therapy has a place in the management of ABPA and is the cornerstone of treatment in CPA, reducing morbidity and probably mortality. We used the country-specific prevalence of asthma from the Global Initiative for Asthma (GINA) report applied to population estimates to calculate adult asthma cases. From five referral cohorts (China, Ireland, New Zealand, Saudi Arabia and South Africa), we estimated the prevalence of ABPA in adults with asthma at 2.5% (range 0.72-3.5%) (scoping review). From ABPA case series, pulmonary cavitation occurred in 10% (range 7-20%), allowing an estimate of CPA prevalence worldwide using a deterministic scenario-based model. Of 193 million adults with active asthma worldwide, we estimate that 4,837,000 patients (range 1,354,000-6,772,000) develop ABPA. By WHO region, the ABPA burden estimates are: Europe, 1,062,000; Americas, 1,461,000; Eastern Mediterranean, 351,000; Africa, 389,900; Western Pacific, 823,200; South East Asia, 720,400. We calculate a global case burden of CPA complicating ABPA of 411,100 (range 206,300-589,400) at a 10% rate with a 15% annual attrition. The global burden of ABPA potentially exceeds 4.8 million people and of CPA complicating ABPA ˜ 400,000, which is more common than previously appreciated. Both conditions respond to antifungal therapy justifying improved case detection. Prospective population and clinical cohort studies are warranted to more precisely ascertain the frequency of ABPA and CPA in different locations and ethnic groups and validate the model inputs.

  13. Medical therapy for advanced gastro-entero-pancreatic and bronchopulmonary neuroendocrine tumors

    Institute of Scientific and Technical Information of China (English)

    Mariangela Torniai; Silvia Rinaldi; Francesca Morgese; Giulia Ricci; Azzurra Onofri; Christian Groh; Rossana Berardi

    2016-01-01

    Neuroendocrine tumors (NETs) represent a spectrum of rare neoplasms arising in different organism sites. Depending on the site of onset, they also can be distinguished using lab exams (secretingvs. nonsecreting), clinical symptoms (functioningvs. nonfunctioning), behavioral, morphological characteristics (tumor cells’ architectural growth patterns, mitotic and Ki-67 index, presence of necrosis), and grade of cellular differentiation. The aim of this review is to focus on the main signaling pathways targeted by medical treatments of advanced sporadic gastro-entero-pancreatic (GEP) and bronchopulmonary (BP) neuroendocrine neoplasms. The scientiifc literature regarding treatment of advanced GEP and BP-NETs has been extensively reviewed using MEDLINE and PubMed databases, selecting principal and more recent research articles, clinical trials, and updated guidelines. Somatostatin analogues represent a valid approach to control symptoms in functioning tumors and to inhibit tumor progression in certain categories on the basis of the typical somatostatin receptor expression observed in NETs. The pathogenesis of NETs has been the subject of increased interest in recent years. Many driver mutations pathway genes have been identiifed as important factors in the carcinogenesis process and, therefore, as potential targets for new anticancer therapies. Activating mutations have been shown in epidermal growth factor receptor, stem cell factor receptor, platelet-derived growth factor receptor, vascular endothelial growth factor, basic-ifbroblastic growth factor, transforming growth factor, insulin-like growth factor-1, and their receptors. Effective M-Tor inhibition pathway modulation has led to the approval of drugs in this ifeld such as everolimus. New drugs and several combination regimens with targeted and newer biological agents are being developed and tested in recently conducted and ongoing trials.

  14. Allergic bronchopulmonary aspergillosis: A review of 42 patients from a tertiary care center in India

    Directory of Open Access Journals (Sweden)

    Prasad R

    2009-01-01

    Full Text Available Objective: To study the clinical, radiological, and laboratory profile in patients of allergic bronchopulmonary aspergillosis (ABPA. Materials and Methods: Retrospective analysis of 42 cases of ABPA, diagnosed over a period of 10 years from 1995 to 2005, for their clinical, radiological, and laboratory profiles. Results: Of 42 ABPA patients, 27 were men and 17 were women. Their mean age at the time of diagnosis was 31.2 years and mean duration of illness was 12.2 years. Breathlessness was the chief symptom. Other allergic disorders existed in 17 (40.5% patients, and family history suggestive of allergic disease was present in 22 (52.4% patients. Most common chest radiographic finding was fleeting pulmonary shadows in 28 (66.7% patients. High resolution CT thorax revealed central bronchiectasis as predominant finding. Peripheral blood eosinophilia more than 1000 cells/µl, Type I and type III cutaneous reactivity to Aspergillus antigen, elevated serum titers of total IgE antibody, A. fumigatus specific IgE and IgG antibodies, and serum precipitin against A. fumigatus were positive in majority of patients, who underwent these tests. Thirty eight (90.5% patients had had history of antitubercular treatment during the course of their illness. All 42 patients met at least four criteria for the diagnosis of ABPA. Conclusion: Any patient of bronchial asthma, presenting with recurrent shadows in chest radiograph and high peripheral blood eosinophilia, should be investigated for ABPA. Efforts need to be intensified to improve the awareness level among general physicians for early diagnosis and prompt treatment of this disease to avoid misuse of antitubercular drugs.

  15. Maternal assessment of pain in premature infants

    Directory of Open Access Journals (Sweden)

    Maria Carolina Correia dos Santos

    2015-12-01

    Full Text Available Objective: to identify mothers' perceptions about the pain in their premature babies in the Neonatal Intensive Care Unit. Methods: evaluative, quantitative study with investigative nature conducted with 19 mothers of hospitalized premature newborns. Data were obtained from closed questions, answered by mothers. Results: from the participants, two (10.5% reported that newborns are unable to feel pain. From the 17 mothers who said that premature babies can feel pain, the majority (94.1% identified crying as a characteristic of pain sensation. Eleven (64.7% stated that uneasiness is a sign of pain in newborns. Conclusion: for the proper management of neonatal pain it is essential that mothers know the signs of pain in premature newborns, and that health professionals instruct this recognition, through the enhancement of the maternal presence and practice of effective communication between professionals and newborns’ families.

  16. Apnea of prematurity: challenges and solutions

    Directory of Open Access Journals (Sweden)

    Picone S

    2014-06-01

    Full Text Available Simonetta Picone, Roberto Aufieri, Piermichele PaolilloDivision of Neonatology and Neonatal Intensive Care, Department of Maternal and Child Health, Casilino General Hospital, Rome, ItalyAbstract: Apnea of prematurity is a developmental disorder that frequently affects preterm infants, especially those with lower gestational age. Even if apnea of prematurity is by definition a self-limiting condition, it can cause serious problems during the hospital stay and can potentially have long-term neurological and cognitive consequences depending on the severity and intensity of the episodes. The diagnosis of apnea of prematurity can be made only after excluding a number of diseases of the preterm infant in which apnea may be an epiphenomenon. Etiological diagnosis is essential for selection of appropriate treatment, which may be nonpharmacological or involve use of drugs.Keywords: apnea of prematurity, idiopathic and secondary apnea, caffeine

  17. Molecular Characterization and In Vitro Antifungal Susceptibility Profile of Schizophyllum commune, an Emerging Basidiomycete in Bronchopulmonary Mycoses

    OpenAIRE

    Chowdhary, Anuradha; Kathuria, Shallu; Singh, Pradeep Kumar; Agarwal, Kshitij; Gaur, Shailendra N.; Roy, Pradip; Randhawa, Harbans S.; Meis, Jacques F.

    2013-01-01

    Schizophyllum commune (n = 30) showed lowest geometric mean MICs of isavuconazole (0.19 μg/ml), itraconazole (0.2 μg/ml), voriconazole (0.24 μg/ml), and amphotericin B (0.29 μg/ml) and high geometric mean MICs of fluconazole (19.39 μg/ml) and flucytosine (17.28 μg/ml). Five cases (of 8) of allergic bronchopulmonary mycosis that were treated with itraconazole had no recrudescence after 6 to 24 months of follow-up. One case each of invasive pulmonary mycosis and fungal ball were treated success...

  18. Molecular characterization and in vitro antifungal susceptibility profile of Schizophyllum commune, an emerging basidiomycete in bronchopulmonary mycoses.

    Science.gov (United States)

    Chowdhary, Anuradha; Kathuria, Shallu; Singh, Pradeep Kumar; Agarwal, Kshitij; Gaur, Shailendra N; Roy, Pradip; Randhawa, Harbans S; Meis, Jacques F

    2013-06-01

    Schizophyllum commune (n = 30) showed lowest geometric mean MICs of isavuconazole (0.19 μg/ml), itraconazole (0.2 μg/ml), voriconazole (0.24 μg/ml), and amphotericin B (0.29 μg/ml) and high geometric mean MICs of fluconazole (19.39 μg/ml) and flucytosine (17.28 μg/ml). Five cases (of 8) of allergic bronchopulmonary mycosis that were treated with itraconazole had no recrudescence after 6 to 24 months of follow-up. One case each of invasive pulmonary mycosis and fungal ball were treated successfully with voriconazole and itraconazole.

  19. Molecular Characterization and In Vitro Antifungal Susceptibility Profile of Schizophyllum commune, an Emerging Basidiomycete in Bronchopulmonary Mycoses

    Science.gov (United States)

    Kathuria, Shallu; Singh, Pradeep Kumar; Agarwal, Kshitij; Gaur, Shailendra N.; Roy, Pradip; Randhawa, Harbans S.

    2013-01-01

    Schizophyllum commune (n = 30) showed lowest geometric mean MICs of isavuconazole (0.19 μg/ml), itraconazole (0.2 μg/ml), voriconazole (0.24 μg/ml), and amphotericin B (0.29 μg/ml) and high geometric mean MICs of fluconazole (19.39 μg/ml) and flucytosine (17.28 μg/ml). Five cases (of 8) of allergic bronchopulmonary mycosis that were treated with itraconazole had no recrudescence after 6 to 24 months of follow-up. One case each of invasive pulmonary mycosis and fungal ball were treated successfully with voriconazole and itraconazole. PMID:23507274

  20. Cemento-osseous dysplasia in Jamaica: review of six cases.

    Science.gov (United States)

    Ogunsalu, C; Miles, D

    2005-09-01

    Six cases of cemento-osseous dysplasia (COD) of the jaw bone in Jamaicans are reviewed. Five were documented over a 15-year period (1980-1995). These include a case of florid cemento-osseous dysplasia (previously called gigantiform cementoma). Three of the initial cases were histologically diagnosed as gigantiform cementoma. There was no indication in the patient's case file whether these were familial or non-familial. The other two cases were diagnosed histologically as periapical cemento-osseous dysplasia and cementoblastoma respectively. Based on the current understanding of the nature of florid-cemento-osseous dysplasia (FLCOD), a new case was diagnosed as such solely on radiological findings. This single case of FLCOD is reported and discussed against the background of other cemento-osseous lesions. Special emphasis is placed on the radiology of COD in this paper. The confirmative role of radiology without the need for histophathology and treatment for asymptomatic FLCOD is emphasized.

  1. Axial mesodermal dysplasia complex: a new case with parental consanguinity.

    Science.gov (United States)

    Mota, C R; Azevedo, M; Rocha, G; Manuela, F; Coelho, R; Lima, M R

    2000-01-01

    A female is described with axial mesodermal dysplasia complex (AMDC) born to a consanguineous couple. This is thought to be the first description of a patient with AMDC born to consanguineous parents.

  2. PATHOLOGICAL AND MORPHOLOGICAL RESEARCH OF EXPERIMENTAL ACETABULAR DYSPLASIA

    Institute of Scientific and Technical Information of China (English)

    张自明; 马瑞雪; 吉士俊; 牛之彬

    2004-01-01

    Objective To investigate the pathological mechanism of hip dysplasia. Methods The left knee joints of eighteen rabbits were fixed in extending position with plaster cylinder for four weeks, but their hip joints were flexed. The right side served as control. Roentgenogram was made in all animals. The changes of the xray films and the pathological findings between left and right hips were compared. Results Appearance of hip dysplasia was obvious at four weeks after plaster fixation. There were pathological changes, including shallow acetabulum and flat femoral head, increased acetabular index and decreased acetabular head index on the x-ray films.Conclusion The hip dysplasia is the result of prolonged extending position of the knee joint. Abnormal knee posture seems to be one of the important factors of hip dysplasia. This kind of deformation may be worsened with time.

  3. Pelvic orientation and assessment of hip dysplasia in adults

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Sonne-Holm, Stig; Lund, Bjarne

    2004-01-01

    on the measurements of radiographic indices of hip dysplasia. MATERIAL AND METHODS: We investigated the effect of varying pelvic orientation on radiographic measurements of acetabular dysplasia using a cadaver model. Results from the cadaver study were used to validate the radiographic assessments of acetabular...... dysplasia in the longitudinal survey cohort of the Copenhagen City Heart Study (CCHS; Osteoarthrosis Sub-study). 1) Cadaver pelvises and proximal femurs from a male and a female donor were mounted anatomically in holding devices allowing independent inclination/reclination and rotation. An AP pelvic...... radiograph was recorded at each 3 degrees increment. The most widely used radiographic parameters of hip dysplasia were assessed. 2) Critical limits of acceptable rotation and inclination/reclination of pelvises were determined on 4151 standing, standardised pelvic radiographs of the CCHS cohort. RESULTS...

  4. 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

  5. Focal Cortical Dysplasia Type IIB and Human Papillomavirus

    Directory of Open Access Journals (Sweden)

    J. Gordon Millichap

    2013-03-01

    Full Text Available Researchers at Temple University School of Medicine, Philadelphia, PA tested the hypothesis that human papillomavirus type 16 oncoprotein E6 (HPV16 E6 is present in human focal cortical dysplasia type IIB (FCDIIB specimens.

  6. Genetics Home Reference: Schimke immuno-osseous dysplasia

    Science.gov (United States)

    ... 4 links) Health Topic: Bone Diseases Health Topic: Dwarfism Health Topic: Immune System and Disorders Health Topic: ... Immune Deficiency Conditions University of Kansas Medical Center: Dwarfism/Short Stature GeneReviews (1 link) Schimke Immunoosseous Dysplasia ...

  7. New ECG Criteria in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

    NARCIS (Netherlands)

    M.G.P.J. Cox; J.J. van der Smagt; A.A.M. Wilde; A.C.P. Wiesfeld; D.E. Atsma; M.R. Nelen; L.M. Rodriguez; P. Loh; M.J. Cramer; P.A. Doevendans; J.P. van Tintelen; J.M.T. de Bakker; R.N.W. Hauer

    2009-01-01

    Background-Desmosomal changes, electric uncoupling, and surviving myocardial bundles in fibrofatty tissue characterize arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Resultant activation delay is pivotal for reentry and thereby ventricular tachycardia (VT). Current task force cr

  8. 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.

  9. Premature dental eruption: report of case.

    LENUS (Irish Health Repository)

    McNamara, C M

    2011-08-05

    This case report reviews the variability of dental eruption and the possible sequelae. Dental eruption of the permanent teeth in cleft palate children may be variable, with delayed eruption the most common phenomenon. A case of premature dental eruption of a maxillary left first premolar is demonstrated, however, in a five-year-old male. This localized premature dental eruption anomaly was attributed to early extraction of the primary dentition, due to caries.

  10. Neuro-ophthalmic manifestations of prematurity

    OpenAIRE

    Preeti Patil Chhablani; Ramesh Kekunnaya

    2014-01-01

    Increasing rates of preterm births coupled with better survival of these infants have resulted in higher prevalence of systemic and ocular complications associated with prematurity. In addition to retinopathy of prematurity, infants who are born preterm may suffer from severe visual impairment as a result of hypoxic ischemic encephalopathy, hypoglycemia, and other metabolic imbalances. The effect of these processes on the anterior visual pathway may result in optic atrophy, optic nerve hypopl...

  11. PECULIARITIES OF BREAST FEEDING OF PREMATURE CHILDREN

    Directory of Open Access Journals (Sweden)

    V.К. Kotlukov

    2011-01-01

    Full Text Available The article presents main strategies of breast feeding of prematurely born infants support, such as use of Philips AVENT breast pumpfor lactation formation and feeding of the infant with native breast milk.Key words: premature infants, nursing mother, breast feeding support, modern accessories for breast feeding support. (Voprosy sovremennoi pediatrii — Current Pediatrics. 2011; 10 (6: 170–175

  12. The pathophysiology of acquired premature ejaculation

    OpenAIRE

    McMahon, Chris G; Jannini, Emmanuele A.; Serefoglu, Ege C.; Hellstrom, Wayne J.G.

    2016-01-01

    The second Ad Hoc International Society for Sexual Medicine (ISSM) Committee for the Definition of Premature Ejaculation defined acquired premature ejaculation (PE) as a male sexual dysfunction characterized by a the development of a clinically significant and bothersome reduction in ejaculation latency time in men with previous normal ejaculatory experiences, often to about 3 minutes or less, the inability to delay ejaculation on all or nearly all vaginal penetrations, and the presence of ne...

  13. Streeter Dysplasia, from Pelvic to Digits: A Case Report

    OpenAIRE

    Komang Agung Irianto; Luh Gede Djatu Anggita Dewi; Gana Adyaksa

    2016-01-01

    Background: Streeter dysplasia is a term to describe fetal congenital syndrome which mainly characterized by constriction band on appendages, prenatal amputations of extremities, and acrosyndactyly. This syndrome has wide range of clinical manifestation between patients, as reflected by many other terms to describe this syndrome. Case: The author reported five cases of Streeter dysplasia with constriction band on different locations of the body, with a patient having a constriction band aroun...

  14. Osteofibrous dysplasia of clavicle clinically mimicking chronic osteomyelitis

    Directory of Open Access Journals (Sweden)

    Nirmal Raj Gopinathan

    2016-01-01

    Full Text Available Osteofibrous dysplasia or ossifying fibroma is an uncommon benign fibro-osseous lesion of childhood, commonly described in the maxilla and the mandible. Among long bones, it usually presents in the tibia as a painless swelling or anterior bowing. Ossifying fibroma of clavicle has never been reported in English literature, to the best of our knowledge. Here, we would like to present an unusual case of osteofibrous dysplasia of clavicle clinically mimicking chronic osteomyelitis.

  15. 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.

  16. Frequency of neonatal complications after premature delivery

    Directory of Open Access Journals (Sweden)

    Gordana Grgić

    2013-04-01

    Full Text Available Introduction: Preterm delivery is the delivery before 37 weeks of gestation are completed. The incidence of preterm birth ranges from 5 to 15%. Aims of the study were to determine the average body weight, Apgar score after one and five minutes, and the frequency of the most common complications in preterminfants.Methods: The study involved a total of 631 newborns, of whom 331 were born prematurely Aims of this study were to (24th-37th gestational weeks-experimental group, while 300 infants were born in time (37-42 weeks of gestation-control group.Results: Average body weight of prematurely born infants was 2382 grams, while the average Apgar score in this group after the fi rst minute was 7.32 and 7.79 after the fifth minute. The incidence of respiratory distress syndrome was 50%, intracranial hemorrhage, 28.1% and 4.8% of sepsis. Respiratory distresssyndrome was more common in infants born before 32 weeks of gestation. Mortality of premature infants is present in 9.1% and is higher than that of infants born at term.Conclusions: Birth body weight and Apgar scores was lower in preterm infants. Respiratory distress syndrome is the most common fetal complication of prematurity. Intracranial hemorrhage is the second most common complication of prematurity. Mortality of premature infants is higher than the mortality of infants born at term birth.

  17. 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

    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...... to Danish patients with hip dysplasia operated with periacetabular osteotomy (PAO), and the degree of relationship of affected family members. Furthermore, to assess the association between gender and family predisposition in the same group of patients. METHOD: The study is a cross-sectional study...

  18. 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.

  19. The usefulness of nucleomedical procedures in diagnosis of fibrous dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Otsuka, Nobuaki; Fukunaga, Masao; Ono, Shimato (Kawasaki Medical School, Kurashiki, Okayama (Japan)) (and others)

    1989-09-01

    Bone scintigraphy with {sup 99m}Tc-phosphorous compounds and {sup 67}Ga scintigraphy were performed in 8 patients (monostotic 3 cases, polyostotic 5 cases) with fibrous dysplasia. The tendency toward abnormal accumulation of radioactivity on bone scintigraphy was high in the tibia, maxilla, mandibule and ribs. The characteristics of the scintigraphic image at the sites of bone lesion in fibrous dysplasia were judged to be marked (++), moderate (+) or poor or minimal (-), according to the degree of accumulation of radioactivity. Eleven sites of fibrous dysplasia showed marked accumulation and 5 sites showed moderate accumulation. Poor or minimal accumulation was not observed in any fibrous dysplasia lesions. Sclerotic changes on bone roentgenograms appeared as marked accumulation of radionuclides on bone scintigraphy in all cases. Cystic changes on roentgenograms showed a tendency toward moderate accumulation on scintigrams. {sup 67}Ga scans were also all positive for 2 experimental cases (3 sites) of bone lesions of fibrous dysplasia. Thus, bone and {sup 67}Ga scintigraphies appear to be useful and essential in evaluating the pathophysiology of fibrous dysplasia. (author).

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

    Energy Technology Data Exchange (ETDEWEB)

    Troelsen, Anders; Elmengaard, Brian; Soeballe, Kjeld (Orthopedic Research Unit, Univ. Hospital of Aarhus, Aarhus (Denmark)), e-mail: a_troelsen@hotmail.com; Roemer, Lone (Dept. of Radiology, Univ. Hospital of Aarhus, Aarhus (Denmark)); Kring, Soeren (Dept. of Orthopedic Surgery, Aabenraa Hospital, Aabenraa (Denmark))

    2010-03-15

    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