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Sample records for aspiration syndrome due

  1. Neonatal aspiration syndrome due to vernix caseosa

    International Nuclear Information System (INIS)

    Ohlsson, A.; Najjar, H.; Cumming, W.A.

    1985-01-01

    Fetal aspiration of meconium in amniotic fluid is a well-known cause of respiratory distress in newborn infants. It causes an irregular, coarse, nodular pattern on chest radiographs. Less known is that aspiration of vernix caseoasa causes a similar syncrome. We present a post-mature infant in whom aspiration of vernix caseosa caused respiratory distress, ventilatory difficulty, and radiographic changes essentially the same as aspiration of meconium. (orig.)

  2. Neonatal aspiration syndrome due to vernix caseosa

    Energy Technology Data Exchange (ETDEWEB)

    Ohlsson, A.; Najjar, H.; Cumming, W.A.

    1985-02-01

    Fetal aspiration of meconium in amniotic fluid is a well-known cause of respiratory distress in newborn infants. It causes an irregular, coarse, nodular pattern on chest radiographs. Less known is that aspiration of vernix caseoasa causes a similar syncrome. We present a post-mature infant in whom aspiration of vernix caseosa caused respiratory distress, ventilatory difficulty, and radiographic changes essentially the same as aspiration of meconium.

  3. Meconium aspiration syndrome

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/001596.htm Meconium aspiration syndrome To use the sharing features on this page, please enable JavaScript. Meconium aspiration syndrome (MAS) refers to breathing problems that a newborn ...

  4. Meconium aspiration syndrome

    Directory of Open Access Journals (Sweden)

    I.O. Bushtyreva

    2010-06-01

    Full Text Available Pathophysiological aspects of formation and development of meconium aspiration syndrome, as well as peculiaritues of its development based on data from native and foreign literature are studied. Special emphasis is pointed out to possible diagnostic techniques and prevention of syndrome

  5. Time-dependent changes in pulmonary surfactant function and composition in acute respiratory distress syndrome due to pneumonia or aspiration

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    Kuchenbuch Tim

    2007-07-01

    Full Text Available Abstract Background Alterations to pulmonary surfactant composition have been encountered in the Acute Respiratory Distress Syndrome (ARDS. However, only few data are available regarding the time-course and duration of surfactant changes in ARDS patients, although this information may largely influence the optimum design of clinical trials addressing surfactant replacement therapy. We therefore examined the time-course of surfactant changes in 15 patients with direct ARDS (pneumonia, aspiration over the first 8 days after onset of mechanical ventilation. Methods Three consecutive bronchoalveolar lavages (BAL were performed shortly after intubation (T0, and four days (T1 and eight days (T2 after intubation. Fifteen healthy volunteers served as controls. Phospholipid-to-protein ratio in BAL fluids, phospholipid class profiles, phosphatidylcholine (PC molecular species, surfactant proteins (SP-A, -B, -C, -D, and relative content and surface tension properties of large surfactant aggregates (LA were assessed. Results At T0, a severe and highly significant reduction in SP-A, SP-B and SP-C, the LA fraction, PC and phosphatidylglycerol (PG percentages, and dipalmitoylation of PC (DPPC was encountered. Surface activity of the LA fraction was greatly impaired. Over time, significant improvements were encountered especially in view of LA content, DPPC, PG and SP-A, but minimum surface tension of LA was not fully restored (15 mN/m at T2. A highly significant correlation was observed between PaO2/FiO2 and minimum surface tension (r = -0.83; p Conclusion We concluded that a profound impairment of pulmonary surfactant composition and function occurs in the very early stage of the disease and only gradually resolves over time. These observations may explain why former surfactant replacement studies with a short treatment duration failed to improve outcome and may help to establish optimal composition and duration of surfactant administration in future

  6. Meconium aspiration syndrome: historical aspects.

    Science.gov (United States)

    Fanaroff, A A

    2008-12-01

    The meconium aspiration syndrome (MAS) is a common problem that continues to concern perinatologists and neonatologists. MAS is defined as respiratory distress in an infant born through meconium-stained amniotic fluid (MSAF) whose symptoms cannot be otherwise explained. This disorder may be life threatening, complicated by respiratory failure, pulmonary air leaks and persistent pulmonary hypertension. Approaches to the prevention of MAS have changed over time with collaboration between obstetricians and pediatricians forming the foundations for care. This report details the management of babies delivered with associated MSAF before the accumulation of evidence for best practice through appropriately powered, prospective randomized controlled trials.

  7. Meconium Aspiration Syndrome: An Insight.

    Science.gov (United States)

    Raju, U; Sondhi, V; Patnaik, S K

    2010-04-01

    Meconium aspiration syndrome (MAS) is respiratory distress in a newborn baby caused by the presence of meconium in the tracheobronchial airways. The aspiration of meconium stained amniotic fluid by the fetus can happen during antepartum or intrapartum periods and can result in airway obstruction, interference with alveolar gas exchange, chemical pneumonitis as well as surfactant dysfunction. These pulmonary effects cause gross ventilation-perfusion mismatching. To complicate matters further, many infants with MAS have primary or secondary persistent pulmonary hypertension of the newborn as a result of chronic in utero stress and thickening of the pulmonary vessels. Although meconium is sterile, its presence in the air passages can predispose the infant to pulmonary infection. MAS is essentially a clinical diagnosis and should always be suspected in a child with respiratory distress and meconium-stained amniotic fluid at delivery. Though a known entity for a long time, its management still remains contentious. Intubation and direct tracheal suction is performed when meconium is observed in the amniotic fluid and the infant is not vigorous. Subsequent management involves ventilation, surfactant instillation and lavage, inhaled nitric oxide and high frequency ventilation. The role of steroids continues to be controversial.

  8. MECONIUM ASPIRATION SYNDROME : A CLINICAL STUDY

    OpenAIRE

    BEHERA, MK; KULKARNI, SD; GUPTA, RK

    1998-01-01

    Forty two babies with Meconium aspiration syndrome managed in neonatal intensive care unit of a service hospital were analysed. Incidence was 1.7 per cent. Twelve babies were delivered by caserean section and 30 babies by vaginal route. Seventeen deliveries were conducted by team of gynaecologists, pediatrician and nursing staff whereas 25 deliveries were conducted by nursing staff alone. Out of total babies with meconium aspiration syndrome, thin meconium detected in 9 and thick meconium det...

  9. Meconium aspiration syndrome: challenges and solutions

    OpenAIRE

    Goel,Ankita; Nangia,Sushma

    2017-01-01

    Ankita Goel, Sushma Nangia Department of Neonatology, Lady Hardinge Medical College and Kalawati Saran Children Hospital, Shaheed Bhagat Singh Marg, New Delhi, India Abstract: Meconium aspiration syndrome (MAS) is an important cause of morbidity and mortality among term newborns. A result of antepartum or postpartum aspiration of meconium stained amniotic fluid (MSAF), MAS causes respiratory distress of varying severity, often complicated by air leaks or persistent pulmonary...

  10. Lung ultrasound findings in meconium aspiration syndrome.

    Science.gov (United States)

    Piastra, Marco; Yousef, Nadya; Brat, Roselyne; Manzoni, Paolo; Mokhtari, Mostafa; De Luca, Daniele

    2014-09-01

    Meconium aspiration syndrome (MAS) is a rare and life-threatening neonatal lung injury induced by meconium in the lung and airways. Lung ultrasound (LUS) is a quick, easy and cheap imaging technique that is increasingly being used in critical care settings, also for newborns. In this paper we describe ultrasound findings in MAS. Six patients with MAS of variable severity were examined by LUS during the first hours of life. Chest X-rays were used as reference. The following dynamic LUS signs were seen in all patients: (1) B-pattern (interstitial) coalescent or sparse; (2) consolidations; (3) atelectasis; (4) bronchograms. No pattern was observed for the distribution of signs in lung areas, although the signs varied with time, probably due to the changing localisation of meconium in the lungs. LUS images corresponded well with X-ray findings. In conclusion, we provide the first formal description of LUS findings in neonates with MAS. LUS is a useful and promising tool in the diagnosis and management of MAS, providing real-time bedside imaging, with the additional potential benefit of limiting radiation exposure in sick neonates. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  11. Meconium aspiration syndrome: challenges and solutions

    Directory of Open Access Journals (Sweden)

    Goel A

    2017-08-01

    Full Text Available Ankita Goel, Sushma Nangia Department of Neonatology, Lady Hardinge Medical College and Kalawati Saran Children Hospital, Shaheed Bhagat Singh Marg, New Delhi, India Abstract: Meconium aspiration syndrome (MAS is an important cause of morbidity and mortality among term newborns. A result of antepartum or postpartum aspiration of meconium stained amniotic fluid (MSAF, MAS causes respiratory distress of varying severity, often complicated by air leaks or persistent pulmonary hypertension (PPHN. There has been a tremendous change in the concepts of pathophysiology and management of MAS over the last few decades. Routine endotracheal suctioning is no longer recommended in both vigorous and nonvigorous neonates with MSAF. Supportive management, along with newer therapies such as surfactant, inhaled nitric oxide, and high-frequency ventilation, has resulted in marked improvement in the overall outcome of MAS. The present review highlights the challenges in understanding the complex pathophysiology and optimal management approach to MAS. Potential future therapies and drugs in trial are also discussed briefly. Keywords: meconium aspiration syndrome, endotracheal suction, surfactant, inhaled nitric oxide, high-frequency ventilation, persistent pulmonary hypertension

  12. Destroyed lung due to aspiration of phenolphthalein.

    Science.gov (United States)

    De Wulf, M; Ceuppens, H; Gheysens, B; Inderadjaja, N

    2005-01-01

    A 76-year-old female, presented with a recurrent cough and haemoptysis after an aspiration of a 'grain de Valls'. Chest radiography and computed tomography demonstrated an atelectasis of the medial segment of the right middle pulmonary lobe. Bronchoscopy was negative for any malignancy but the onco-PET-scan showed a pathologic hypermetabolic lesion in the right middle lobe. A lobectomy was performed and pathology revealed a 'destroyed lung' with images of foreign body reactions and microabscesses without any sign of malignancy. Postoperatively, clinical improvement with complete resolution of the symptoms was observed. With this case report we demonstrate the irritative and clastogenic characteristics of phenolphtalein, the main active ingredient of the 'grain de Valls'. This case also illustrates an example of a false positive PET-scan.

  13. Recurrent Aspiration Pneumonia due to Anterior Cervical Osteophyte

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    Jae Jun Lee

    2017-02-01

    Full Text Available A 74-year-old man presented with recurrent vomiting and aspiration pneumonia in the left lower lobe. He entered the intensive care unit to manage the pneumonia and septic shock. Although a percutaneous endoscopic gastrostomy tube was implanted for recurrent vomiting, vomiting and aspiration recurred frequently during admission. Subsequently, he complained of neck pain when in an upright position. A videofluoroscopic swallowing study showed compression of the esophagus by cervical osteophytes and tracheal aspiration caused by an abnormality at the laryngeal inlet. Cervical spine X-rays and computed tomography showed anterior cervical osteophytes at the C3-6 levels. Surgical decompression was scheduled, but was cancelled due to his frailty. Unfortunately, further recurrent vomiting and aspiration resulted in respiratory arrest leading to hypoxic brain damage and death. Physicians should consider cervical spine disease, such as diffuse skeletal hyperostosis as an uncommon cause of recurrent aspiration pneumonia.

  14. Meconium aspiration syndrome: do we know?

    Science.gov (United States)

    Yurdakök, Murat

    2011-01-01

    Meconium is a common finding in amniotic fluid and placental specimens, particularly in the term or post-term pregnancy. The most important consequence of meconium-stained amniotic fluid (MSAF) is meconium aspiration syndrome (MAS), and at least 5% of infants born through MSAF develop MAS. MAS continues to be a threat to many newborns throughout the world, with a case fatality rate of 5% (as much as 40%), in addition to short- and long-term pulmonary and neurodevelopmental sequelae. The exact pathophysiology of meconium passage into the amniotic fluid and consequences of meconium aspiration are unknown. There are three prevailing and possibly compatible theories for mechanisms of meconium release. Firstly, meconium passage is probably related with the maturation of the gastrointestinal tract, because meconium passage in the preterm third trimester fetus has been reported to be a rare event, as typically it occurs near or post-term. Secondly, an alternate hypothesis for in utero meconium passage is that pathologic processes, such as stress via hypoxia or infection, can trigger meconium passage. However, the predictive values of MSAF for fetal distress and acidosis at birth are poor and controversial. Thirdly, an alternative route for the presence of MSAF in the presence of fetal hypoxia is reduced clearance of defecated meconium due to impaired fetal swallowing or unidentified placental dysfunction in addition to or instead of an increase in its passage. The pathophysiology of MAS is multifactorial and extremely complex. Meconium causes mechanical obstruction and pulmonary air leak, induces surfactant inactivation, causes pulmonary inflammation, and induces apoptosis. Preventing prolonged pregnancy by labor induction might reduce the risk of MSAF. Labor induction with prostaglandins appears to be associated with the occurrence of MSAF. Amnioinfusion for suspected umbilical cord compression has no clear effect on the occurrence of MSAF. Intrapartum suctioning of the

  15. Predictors of mortality in neonates with meconium aspiration syndrome.

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    Louis, Deepak; Sundaram, Venkataseshan; Mukhopadhyay, Kanya; Dutta, Sourabh; Kumar, Praveen

    2014-08-01

    To identify risk factors for mortality in neonates with meconium aspiration syndrome. All neonates (2004-2010) with meconium aspiration syndrome, irrespective of gestation were included. Risk factors were compared between those who died and survived. Out of 172 included neonates, 44 (26%) died. Mean (SD) gestation and birth weight were 37.9 (2.3) weeks and 2545 (646g), respectively. Myocardial dysfunction [aOR 28.4; 95% CI (8.0-101); PMeconium aspiration syndrome is associated with significant mortality. Myocardial dysfunction, birth weight, and initial oxygen requirement are independent predictors of mortality.

  16. Current Concepts in the Management of Meconium Aspiration Syndrome.

    Science.gov (United States)

    Chettri, Subhash; Bhat, B Vishnu; Adhisivam, B

    2016-10-01

    In developing countries, meconium aspiration syndrome (MAS) is an important cause of morbidity and mortality among neonates. The concepts of pathophysiology and management of meconium stained amniotic fluid (MSAF) and meconium aspiration syndrome have undergone tremendous change in recent years. Routine intranatal and postnatal endotracheal suctioning of meconium in vigorous infants is no longer recommended. Recent studies have challenged its role even in non-vigorous infants. Supportive therapy like oxygen supplementation, mechanical ventilation and intravenous fluids are the cornerstone in the management of meconium aspiration syndrome. Availability of surfactant, inhaled nitric oxide, high frequency ventilators and extracorporeal membrane oxygenation has made it possible to salvage more infants with meconium aspiration syndrome. In this review the authors have discussed the current concepts in the pathophysiology and management of MAS. Drugs in trials and future therapeutic targets are also discussed briefly.

  17. Placental Lesions in Meconium Aspiration Syndrome

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    Binnari Kim

    2017-09-01

    Full Text Available Background Meconium aspiration syndrome (MAS is defined by respiratory distress requiring supplemental oxygen in a meconium-stained neonate. MAS is clinically subclassified as mild, moderate, and severe according to the oxygen requirement. The aims of this study were to compare the histological findings in the placentas of MAS neonates with those of meconium-stained but non-MAS neonates and to analyze the correlation between the severity of MAS and the grade of its histological parameters. Methods We collected 160 singleton term placentas from neonates with meconium staining at birth from a tertiary medical center, Seoul, Republic of Korea. We reviewed hematoxylin and eosin sections of tissue samples (full-thickness placental disc, chorioamniotic membranes, and umbilical cord. Results Funisitis was present more frequently in MAS than in non-MAS (p < .01, of which the stage was correlated with the severity of MAS (p < .001. The histological findings consistent with maternal underperfusion and chronic deciduitis were more frequent in MAS than in non-MAS (p < .05. There was a correlation between the degree of chorionic vascular muscle necrosis and the severity of MAS (p < .05. Conclusions Our results suggest that fetal inflammatory response evidenced by funisitis occurs prenatally in MAS and that the stage of funisitis and of chorionic vascular muscle necrosis may be a predictive marker of the severity of MAS.

  18. Placental Lesions in Meconium Aspiration Syndrome.

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    Kim, Binnari; Oh, Soo-Young; Kim, Jung-Sun

    2017-09-01

    Meconium aspiration syndrome (MAS) is defined by respiratory distress requiring supplemental oxygen in a meconium-stained neonate. MAS is clinically subclassified as mild, moderate, and severe according to the oxygen requirement. The aims of this study were to compare the histological findings in the placentas of MAS neonates with those of meconium-stained but non-MAS neonates and to analyze the correlation between the severity of MAS and the grade of its histological parameters. We collected 160 singleton term placentas from neonates with meconium staining at birth from a tertiary medical center, Seoul, Republic of Korea. We reviewed hematoxylin and eosin sections of tissue samples (full-thickness placental disc, chorioamniotic membranes, and umbilical cord). Funisitis was present more frequently in MAS than in non-MAS (p < .01), of which the stage was correlated with the severity of MAS (p < .001). The histological findings consistent with maternal underperfusion and chronic deciduitis were more frequent in MAS than in non-MAS (p < .05). There was a correlation between the degree of chorionic vascular muscle necrosis and the severity of MAS (p < .05). Our results suggest that fetal inflammatory response evidenced by funisitis occurs prenatally in MAS and that the stage of funisitis and of chorionic vascular muscle necrosis may be a predictive marker of the severity of MAS.

  19. [Pulmonary function in children after neonatal meconium aspiration syndrome].

    Science.gov (United States)

    Djemal, N; Ben Ammar, H; Masmoudi, K; Rguaieg, R; Trigui, L; Ben Hmad, A; Kannou, M; Hmida, N; Gargouri, A; Zouari, N; Rekik, A

    2008-02-01

    Meconium aspiration syndrome is a disease of the newborn mature or post mature. The acute pulmonary consequences can be extremely severe. In the few studies of the long-term pulmonary sequelae, it seems that certain children surviving meconium aspiration syndrome keep an obstructive syndrome. The aim of our study was to assess long term respiratory residual damage from meconium aspiration syndrome. During a seven-year period going from 1994 to 2000, we reviewed the files of children hospitalized in neonatology department of Sfax for meconium aspiration syndrome. The children who were convoked (group M: n=27), underwent spirometry, followed by an exercise stress. An age matched control group (group C: n=23) of healthy children was investigated in the same way. The group M comprised 15 boys and 12 girls aged four to 11, an average of 7+/-1.9 years. With the study of the respiratory function, we did not find an obstructive syndrome. Spirometry revealed a total pulmonary capacity in an average of 133+/-55.65% of theoretical (group M) versus 105.5+/-27.96% of theoretical (group C) (Pmeconium aspiration syndrome tend to develop alveolar hyperinflation and airway hyperreactivity to exercise.

  20. Inflammatory indices in meconium aspiration syndrome.

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    Hofer, Nora; Jank, Katharina; Strenger, Volker; Pansy, Jasmin; Resch, Bernhard

    2016-06-01

    Meconium aspiration syndrome (MAS) is linked to inflammation, but data on the patterns of hematological indices and C-reactive protein (CRP) in MAS are lacking. The aim of the study was to evaluate CRP, white blood cell count (WBC), absolute neutrophil count (ANC), and immature-to-total neutrophil ratio (IT-ratio) in MAS and to assess their association with disease severity. Retrospective cross-sectional study including 239 consecutively admitted neonates with MAS to a level III NICU. Neonates with early onset sepsis were excluded. Results Neonates with severe MAS (invasive mechanical ventilation for <7 days) and very severe MAS (invasive mechanical ventilation for ≥7 days or high frequency ventilation or ECMO) had higher CRP and IT-ratio compared to neonates with non-severe MAS (no invasive mechanical ventilation) during the first 2 days of life (CRP: 13.0 and 40.9 vs. 9.5 mg/L, P = 0.039 and <0.001, respectively) and neonates with very severe MAS had lower WBC and ANC. All four inflammatory indices correlated significantly with duration of invasive mechanical ventilation, duration of respiratory support and with length of hospital stay, arterial hypotension, and persistent pulmonary hypertension. Neonates with all four inflammatory indices beyond the normal range had a more than 20-fold increase in risk for very severe MAS. High CRP and IT-ratio and low WBC and ANC values were closely linked to a more severe course of MAS during the early phases of the disease. These findings reflect the role of inflammation in the pathogenesis of MAS. Pediatr Pulmonol. 2016;51:601-606. 2015 Wiley Periodicals, Inc. © 2015 Wiley Periodicals, Inc.

  1. Blood lactate level and meconium aspiration syndrome.

    Science.gov (United States)

    Karabayir, Nalan; Demirel, Ali; Bayramoglu, Elvan

    2015-04-01

    Approximately 5% of infants born with a meconium-stained amniotic fluid (MSAF) develop meconium aspiration syndrome (MAS). Early recognition of infants at highest risk for the development of MAS and the prediction of disease severity are important for optimizing the clinical strategies for prevention and treatment. The aim of the present study was to identify the risk factors for MAS and to investigate the effect of blood lactate level on the development of MAS. Between January 2011 and January 2012, data were recorded with regard to gender, mode of delivery, gestational week, birth weight, 5-min Apgar score, and need for resuscitation of the meconium-stained depressed infants who underwent tracheal aspiration. Moreover, the number of cases developing MAS, blood pH value, and lactate level in capillary blood gases obtained during the first hour after delivery, duration of oxygen supplementation, the number of cases receiving mechanical ventilation and surfactant therapy, duration of hospital stay, and outcomes of the infants were recorded. The number of live births during the study period was 17,202, and of them, 1,341 (7.8%) infants were born through MSAF. Of 195 infants who were meconium-stained depressed, 90 were girls and 105 were boys. Their mean gestational week was 39.37 ± 0.89 weeks and mean birth weight was 3,426 ± 634 g. Eighty-four of them were born through cesarean section (C/S), and 111 were born via normal spontaneous labor. For 40 infants, Apgar score at fifth minute was less than 6. In total, resuscitation was performed on 43 (22.9%) infants. Of the infants, 141 did not develop MAS and 54 developed MAS. While there were no significant differences between infants with and without MAS with regard to gender, delivery route, gestational week, and birth weight, a significant difference was observed regarding the Apgar score (p = 0.0001). The blood pH value in capillary blood gas analysis was less than 7.25 in 18 (28.5%) cases with MAS and four (3

  2. Advances in the Management of Meconium Aspiration Syndrome

    OpenAIRE

    Swarnam, Kamala; Soraisham, Amuchou S.; Sivanandan, Sindhu

    2011-01-01

    Meconium aspiration syndrome (MAS) is a common cause of severe respiratory distress in term infants, with an associated highly variable morbidity and mortality. MAS results from aspiration of meconium during intrauterine gasping or during the first few breaths. The pathophysiology of MAS is multifactorial and includes acute airway obstruction, surfactant dysfunction or inactivation, chemical pneumonitis with release of vasoconstrictive and inflammatory mediators, and persistent pulmonary hype...

  3. Bone marrow aspiration

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    Iliac crest tap; Sternal tap; Leukemia - bone marrow aspiration; Aplastic anemia - bone marrow aspiration; Myelodysplastic syndrome - bone marrow aspiration; Thrombocytopenia - bone marrow aspiration; Myelofibrosis - bone marrow aspiration

  4. Surfactant therapy for maternal blood aspiration: an unusual cause of neonatal respiratory distress syndrome.

    Science.gov (United States)

    Celik, Istemi Han; Demirel, Gamze; Canpolat, Fuat Emre; Erdeve, Omer; Dilmen, Ugur

    2012-10-01

    Surfactant replacement therapy is the main treatment of neonatal respiratory distress syndrome. However, surfactant therapy has been shown to be effective in the treatment of other diseases causing neonatal respiratory diseases such as pulmonary hemorrhage, meconium aspiration syndrome, pneumonia/sepsis, pulmonary edema or acute lung injury resulting a secondary surfactant deficiency (SSD). Rarely, as like as in the present patient, exogenous blood aspiration such as breast milk or formula aspiration may lead to SSD. Blood in alveolus leads to a significant biochemical and functional disturbance of the surfactant system and inhibits surfactant production. Here, the authors report a preterm infant of 33 wk gestational age with secondary surfactant deficiency due to maternal blood aspiration because of abruptio placentae. She was received two courses of beractant, a natural bovine surfactant, therapy in 24 h. She was extubated on second day and did not require oxygen on 4(th) day. To the authors' knowledge, this is the first reported case of SSD due to maternal blood aspiration treated with surfactant. In conditions such as abruptio placentae, infant should be protected from blood aspiration and if respiratory distress occurs, surfactant inhibition and need for surfactant administration should be considered.

  5. Serum cytokine and chemokine profiles in neonates with meconium aspiration syndrome.

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    Okazaki, Kaoru; Kondo, Masatoshi; Kato, Masahiko; Kakinuma, Ryota; Nishida, Akira; Noda, Masahiro; Taniguchi, Kiyosu; Kimura, Hirokazu

    2008-04-01

    Various inflammatory cytokines and chemokines are thought to be associated with the pathophysiology of meconium aspiration syndrome. To clarify any such association, we compared various serum cytokine and chemokine profiles in patients with and without meconium aspiration syndrome. Using a highly sensitive fluorescence microsphere method, 17 types of cytokines and chemokines in sera were measured in 11 neonatal patients with meconium aspiration syndrome, 16 neonatal patients without meconium aspiration syndrome, and 9 healthy children. The concentrations of 8 types of proinflammatory cytokines and chemokines were significantly higher in the meconium aspiration syndrome group than in healthy controls: interleukin-1beta, interleukin-6, interleukin-8, granulocyte-macrophage colony-stimulating factor, granulocyte colony-stimulating factor, interferon-gamma, macrophage inflammatory protein-1beta, and tumor necrosis factor-alpha. Six types of proinflammatory cytokines and chemokines were significantly higher in the meconium aspiration syndrome group than in the nonmeconium aspiration syndrome group: interleukin-6, interleukin-8, granulocyte-macrophage colony-stimulating factor, granulocyte colony-stimulating factor, interferon-gamma, and tumor necrosis factor-alpha. Serum concentrations of interleukin-10 (anti-inflammatory cytokine) in the meconium aspiration syndrome group were higher than those in both the nonmeconium aspiration syndrome group and healthy children group (P = .007 and 0.001, respectively). Most types of proinflammatory cytokines and chemokines in sera of neonates with meconium aspiration syndrome were higher than those without meconium aspiration syndrome, giving support to the suggestion that elevated levels are associated with the pathogenesis of meconium aspiration syndrome.

  6. Advances in the Management of Meconium Aspiration Syndrome

    Directory of Open Access Journals (Sweden)

    Kamala Swarnam

    2012-01-01

    Full Text Available Meconium aspiration syndrome (MAS is a common cause of severe respiratory distress in term infants, with an associated highly variable morbidity and mortality. MAS results from aspiration of meconium during intrauterine gasping or during the first few breaths. The pathophysiology of MAS is multifactorial and includes acute airway obstruction, surfactant dysfunction or inactivation, chemical pneumonitis with release of vasoconstrictive and inflammatory mediators, and persistent pulmonary hypertension of newborn (PPHN. This disorder can be life threatening, often complicated by respiratory failure, pulmonary air leaks, and PPHN. Approaches to the prevention of MAS have changed over time with collaboration between obstetricians and pediatricians forming the foundations for care. The use of surfactant and inhaled nitric oxide (iNO has led to the decreased mortality and the need for extracorporeal membrane oxygenation (ECMO use. In this paper, we review the current understanding of the pathophysiology and management of MAS.

  7. Advances in the Management of Meconium Aspiration Syndrome

    Science.gov (United States)

    Swarnam, Kamala; Soraisham, Amuchou S.; Sivanandan, Sindhu

    2012-01-01

    Meconium aspiration syndrome (MAS) is a common cause of severe respiratory distress in term infants, with an associated highly variable morbidity and mortality. MAS results from aspiration of meconium during intrauterine gasping or during the first few breaths. The pathophysiology of MAS is multifactorial and includes acute airway obstruction, surfactant dysfunction or inactivation, chemical pneumonitis with release of vasoconstrictive and inflammatory mediators, and persistent pulmonary hypertension of newborn (PPHN). This disorder can be life threatening, often complicated by respiratory failure, pulmonary air leaks, and PPHN. Approaches to the prevention of MAS have changed over time with collaboration between obstetricians and pediatricians forming the foundations for care. The use of surfactant and inhaled nitric oxide (iNO) has led to the decreased mortality and the need for extracorporeal membrane oxygenation (ECMO) use. In this paper, we review the current understanding of the pathophysiology and management of MAS. PMID:22164183

  8. Risk Factors and Clinical Follow-Up Features of Meconium Aspiration Syndrome

    OpenAIRE

    Anuk İnce, Deniz; Takcı, Şahin; Altıntaş, Buket

    2015-01-01

    AbstractAim: Meconium aspiration syndrome is usually seen in full-term and post-term infants and may cause complications including respiratory failure, pulmonary air leaks, and persistent pulmonary hypertension. The aim of this study was to determine risk factors of meconium aspiration syndrome and assess the clinical course of the disease.Material and Methods: Fourteen of 508 infants diagnosed with meconium aspiration syndrome between January 2013 and April 2014 were retrospectively analyzed...

  9. Meconium aspiration syndrome - the experience of a tertiary center.

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    Espinheira, M C; Grilo, M; Rocha, G; Guedes, B; Guimarães, H

    2011-01-01

    Approximately 5 % of infants born with a meconium-stained amniotic fluid (MSAF) develop meconium aspiration syndrome (MAS). The aims of this study were to analyse demographic data, morbidity and mortality associated with MAS and to identify possible risk factors. Retrospective chart review of newborns with MAS delivered at a tertiary centre from January 1st, 1997 to December 31st, 2008. MAS was responsible for 1.4 % of all Neonatal Intensive Care Unit (NICU) admissions, with a trend towards a decreasing incidence during the study duration, especially in the cases of thin meconium. Seventy two newborns were analysed during the study period: 55.6 % (n = 40) were of the female gender, 62.5 % were delivered by caesarean section, 93 % had > 36 weeks of gestational age and 91.2 % had a birth weight over 2500g. Sixty-nine percent had an Apgar score meconium was associated with severe asphyxia and carried a bad prognosis with an increased risk of developing hypoxia (58.3 %), need of mechanical ventilatory support (43.1 %), respiratory and/or metabolic acidosis (30.6 %), pulmonary hypertension (11.1 %) and hypoxic ischemic encephalopathy (29.2 %). The mortality rate was 2.8 %. Thick meconium was associated with higher morbidity and mortality rates. The number of admissions for MAS has been decreasing mostly because of a lower admission rate due to thin meconium; the number of cases with thick meconium has remained constant throughout the years. An Apgar score < 7 at 1 minute and signs of foetal distress during labour were associated with MAS. The MAS related morbidity remains significant.

  10. A case of meconium aspiration syndrome in a bottlenose dolphin (Tursiops truncatus) calf.

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    Tanaka, Miyuu; Izawa, Takeshi; Kuwamura, Mitsuru; Ozaki, Miki; Nakao, Tatsuko; Ito, Shu; Yamate, Jyoji

    2014-01-01

    Stillbirth and neonatal mortality are significant problems in captive breeding of dolphins, however, the causes of these problems are not fully understood. Here, we report a case of meconium aspiration syndrome (MAS) in a male neonate of bottlenose dolphin (Tursiops truncates) who died immediately after birth. At necropsy, a true knot was found in the umbilical cord. The lungs showed diffuse intraalveolar edema, hyperemic congestion and atelectasis due to meconium aspiration with mild inflammatory cell infiltration. Although the exact cause of MAS in this case was unknown, fetal hypoxia due possibly to the umbilical knot might have been associated with MAS, which is the first report in dolphins. MAS due to perinatal asphyxia should be taken into account as a possible cause of neonatal mortality and stillbirth of dolphin calves.

  11. Meconium aspiration syndrome (MAS) - Where do we go? Research perspectives.

    Science.gov (United States)

    Kääpä, Pekka O

    2009-10-01

    The pathogenetic cascade of meconium aspiration syndrome (MAS) in newborn infants is complex and still incompletely studied. The variable clinical presentation of MAS is basically connected with variation of the amount and consistency of aspirated meconium and also its distribution within the affected lungs. The contributing role of other factors, like intrauterine fetal compromises, lung maturity at the time of insult as well as direct and indirect effects of meconium and its components on the lung alveolar and vascular integrity and development, remains to be studied in further detail. Better understanding of the lung injury processes in MAS, specifically inflammatory injury and non-inflammatory apoptosis and their interplay, may offer new possibilities to treat the severely affected infants, and needs therefore to be explored. Systemic dispersion of intrapulmonary meconium and its components may further induce inflammatory circulatory changes and injurious effects in distant organs, but the mechanisms and clinical significance of these systemic complications are still poorly known. It is thus evident that lung injury processes and potent long-term consequences in various extrapulmonary organs, specifically the brain, as well as development of new approaches to their treatment and prevention form great challenges for future research of MAS.

  12. Meconium aspiration syndrome: possible pathophysiological mechanisms and future potential therapies.

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    Lindenskov, Paal Helge Haakonsen; Castellheim, Albert; Saugstad, Ola Didrik; Mollnes, Tom Eirik

    2015-01-01

    Does meconium cause meconium aspiration syndrome (MAS) or is meconium discharge only a marker of fetal hypoxia? This dispute has lasted for centuries, but since the 1960s, detrimental effects of meconium itself on the lungs have been demonstrated in animal experiments. In clinical MAS, persistent pulmonary hypertension of the newborn is the leading cause of death in MAS. Regarding the complex chemical composition of meconium, it is difficult to identify a single agent responsible for the pathophysiology. However, considering that meconium is stored in the intestines, partly unexposed to the immune system, aspirated meconium could be recognized as ‘danger', representing damaged self. The common denominator in the pathophysiology could therefore be activation of innate immunity. Thus, a bulk of evidence implies that meconium is a potent activator of inflammatory mediators, including cytokines, complement, prostaglandins and reactive oxygen species. We hypothesize that the two main recognition systems of innate immunity, the Toll-like receptors and the complement system, recognize meconium as ‘danger', which leads not only to lung dysfunction but also to a systemic inflammatory response. This might have therapeutic implications in the future.

  13. How to overcome surfactant dysfunction in meconium aspiration syndrome?

    Science.gov (United States)

    Mokra, Daniela; Calkovska, Andrea

    2013-06-01

    Surfactant dysfunction in meconium aspiration syndrome (MAS) is caused by meconium components, by plasma proteins leaking through the injured alveolocapillary membrane and by substances originated in meconium-induced inflammation. Surfactant inactivation in MAS may be diminished by several ways. Firstly, aspirated meconium should be removed from the lungs to decrease concentrations of meconium inhibitors coming into the contact with surfactant in the alveolar compartment. Once the endogenous surfactant becomes inactivated, components of surfactant should be substituted by exogenous surfactant at a sufficient dose, and surfactant administration should be repeated, if oxygenation remains compromised. To delay the inactivation by inhibitors, exogenous surfactants may be enriched with surfactant proteins, phospholipids, or other substances such as polymers. Finally, to diminish an adverse action of products of meconium-induced inflammation on both endogenous and exogenously delivered surfactant, anti-inflammatory drugs may be administered. A combined therapeutic approach may result in better outcome in patients with MAS and in lower costs of treatment. Copyright © 2013. Published by Elsevier B.V.

  14. Adaption of suction connectors for use in meconium aspiration syndrome.

    Science.gov (United States)

    Green, David Anthony

    2010-01-01

    Severe meconium aspiration syndrome is difficult to manage and has a high mortality in developing countries. Guidelines are available for the initial management. If the infant has been born through particulate meconium and is not vigorous, an inspection of the vocal cords by laryngoscopy is recommended. If meconium is seen at the cords it should, ideally, be sucked out of the trachea using an endotracheal tube as a suction device. However, as this needs a way of applying suction directly to the endotracheal tube it can be problematic. Commercially available equipment does exist, but in a resource-scarce setting, its cost could be prohibitive. We have adapted cheap suction connectors which can be adapted for this purpose.

  15. Lung ultrasonography to diagnose meconium aspiration syndrome of the newborn.

    Science.gov (United States)

    Liu, Jing; Cao, Hai-Ying; Fu, Wei

    2016-12-01

    Objective To investigate the diagnostic value of lung ultrasonography for neonatal meconium aspiration syndrome (MAS). Methods This prospective observational study enrolled patients diagnosed with MAS based on medical history, clinical manifestations and chest X-ray and control newborns without MAS. During ultrasonography, each lung was divided into three regions (front, lateral, and back), using anterior and posterior axillary lines as the boundary. While scanning each region of the lungs, the hand piece was perpendicular or parallel to the ribs. Results This study enrolled 117 newborns with MAS and 100 controls. The main lung ultrasonographic findings in patients with MAS were: (i) pulmonary consolidation with air bronchogram was found in all patients; (ii) pleural line anomalies and the disappearance of the A-line was found in all patients; (iii) atelectasis was found in 19 (16.2%) severe cases, who demonstrated severe massive atelectasis and visible lung pulse; (iv) pleural effusion was found in 16 patients (13.7%); and (v) alveolar-interstitial syndrome or B-line in the non-consolidation area was found in all patients with MAS. Conclusion Ultrasonography can be used routinely to diagnose MAS in an accurate, reliable, convenient, and non-invasive manner.

  16. Lung lavage for meconium aspiration syndrome in newborn infants.

    Science.gov (United States)

    Hahn, Seokyung; Choi, Hyun Jin; Soll, Roger; Dargaville, Peter A

    2013-04-30

    Meconium aspiration syndrome (MAS) can occur when a newborn infant inhales a mixture of meconium and amniotic fluid into the lungs around the time of delivery. Other than supportive measures, little effective therapy is available. Lung lavage may be a potentially effective treatment for MAS by virtue of removing meconium from the airspaces and altering the natural course of the disease. To evaluate the effects of lung lavage on morbidity and mortality in newborn infants with MAS. We searched the Cochrane Central Register of Controlled Trials (CENTRAL, The Cochrane Library), MEDLINE, and EMBASE up to December 2012; previous reviews including cross-references, abstracts, and conference proceedings; and expert informants. We contacted authors directly to obtain additional data. We used the following subject headings and text words: meconium aspiration, pulmonary surfactants, fluorocarbons, bronchoalveolar lavage, lung lavage, pulmonary lavage. Randomised controlled trials that evaluated the effects of lung lavage in infants with MAS, including those intubated for the purpose of lavage. Lung lavage was defined as any intervention in which fluid is instilled into the lung that is followed by an attempt to remove it by suctioning and/or postural drainage. The review authors extracted from the reports of the clinical trial, data regarding clinical outcomes, including mortality, requirement for extracorporeal membrane oxygenation (ECMO), pneumothorax, duration of mechanical ventilation and oxygen therapy, length of hospital stay, indices of pulmonary function, and adverse effects of lavage. Data analysis was done in accordance with the standards of the Cochrane Neonatal Review Group. Only four small randomised controlled trials fulfilled the selection criteria. For one of these trials, no data are available for the control group. Two studies compared lavage using diluted surfactant with standard care. Meta-analysis of these two studies did not show a significant effect on

  17. Activation of Toll-like receptors in meconium aspiration syndrome.

    Science.gov (United States)

    Anand, V; Basu, S; Yadav, S S; Narayan, G; Bhatia, B D; Kumar, A

    2018-02-01

    Meconium aspiration syndrome (MAS) is a common cause of neonatal morbidity and mortality. Incomplete understanding of the pathogenesis of MAS has hindered the development of specific therapies. We hypothesized that activation of Toll-like receptors (TLRs) might play a role in the pathogenesis of MAS. The present study evaluated the expression of TLR 1, 4, 7, 8 and 9 in neonates with MAS. The study included 39 neonates with MAS and 17 healthy gestational age-matched neonates as controls. Neonates with maternal chorioamnionitis, perinatal asphyxia, sepsis and congenital malformations were excluded. Good-quality total RNA from umbilical cord blood was reverse transcribed to prepare cDNA using Bio-Rad reverse transcription kit. This cDNA was used to study the expression status of TLR 1, 4, 7, 8 and 9 by real-time quantitative polymerase chain reaction. Compared with controls, TLR1 and TLR4 were highly expressed, TLR9 was moderately expressed, TLR7 was weakly expressed and TLR8 expression was neutral in neonates with MAS. Within the MAS group, no difference in TLR expression was observed with respect to consistency of meconium, severity of the disease, oxygenation index and outcome. There is activation of TLRs in neonates with MAS. We speculate that these TLRs probably act as endogenous ligands for various components of meconium that initiate the inflammatory cascade of MAS and contribute to its pathogenesis.

  18. Respiratory Support in Meconium Aspiration Syndrome: A Practical Guide

    Directory of Open Access Journals (Sweden)

    Peter A. Dargaville

    2012-01-01

    Full Text Available Meconium aspiration syndrome (MAS is a complex respiratory disease of the term and near-term neonate. Inhalation of meconium causes airway obstruction, atelectasis, epithelial injury, surfactant inhibition, and pulmonary hypertension, the chief clinical manifestations of which are hypoxaemia and poor lung compliance. Supplemental oxygen is the mainstay of therapy for MAS, with around one-third of infants requiring intubation and mechanical ventilation. For those ventilated, high ventilator pressures, as well as a relatively long inspiratory time and slow ventilator rate, may be necessary to achieve adequate oxygenation. High-frequency ventilation may offer a benefit in infants with refractory hypoxaemia and/or gas trapping. Inhaled nitric oxide is effective in those with pulmonary hypertension, and other adjunctive therapies, including surfactant administration and lung lavage, should be considered in selected cases. With judicious use of available modes of ventilation and adjunctive therapies, infants with even the most severe MAS can usually be supported through the disease, with an acceptably low risk of short- and long-term morbidities.

  19. Mortality in meconium aspiration syndrome in hospitalized babies.

    Science.gov (United States)

    Anwar, Zahid; Butt, Tayyaba Khawar; Kazi, Muhammad Yaqub

    2011-11-01

    To determine the outcome of the babies in terms of mortality with the diagnosis of Meconium Aspiration Syndrome (MAS). An observational study. The Neonatal Unit of Services Institute of Medical Sciences and Services Hospital, Lahore, Pakistan, from February 2008 to January 2009. All the babies admitted to the neonatal unit during the period of study with the diagnosis of MAS were included. At admission, demographic, maternal, antenatal and natal data were recorded on a specific form. The progress of the baby, including need for ventilation, medications, complications and outcome were also followed and documented. One hundred and nine babies admitted with MAS, 32% died. Most of the babies (n=73) were admitted from our obstetrical unit and the rest through the emergency department. Majority (60 of 109) were admitted within the 1st hour of life. Most (14 of 15) of the newborns requiring intubation within 1st hour of life, died. Forty four babies were ventilated and 35 of these babies succumbed. Of ventilated babies, 11 developed pneumothoraces. Seventy two percent (13 out of 18) of expired babies stayed for less than 24 hours. Mortality rate for MAS was higher in the study group as compared to international figures. It was especially high in babies requiring mechanical ventilation in 1st hour of life or with co-existing severe hypoxic ischemic encephalopathy.

  20. Respiratory Support in Meconium Aspiration Syndrome: A Practical Guide

    Science.gov (United States)

    Dargaville, Peter A.

    2012-01-01

    Meconium aspiration syndrome (MAS) is a complex respiratory disease of the term and near-term neonate. Inhalation of meconium causes airway obstruction, atelectasis, epithelial injury, surfactant inhibition, and pulmonary hypertension, the chief clinical manifestations of which are hypoxaemia and poor lung compliance. Supplemental oxygen is the mainstay of therapy for MAS, with around one-third of infants requiring intubation and mechanical ventilation. For those ventilated, high ventilator pressures, as well as a relatively long inspiratory time and slow ventilator rate, may be necessary to achieve adequate oxygenation. High-frequency ventilation may offer a benefit in infants with refractory hypoxaemia and/or gas trapping. Inhaled nitric oxide is effective in those with pulmonary hypertension, and other adjunctive therapies, including surfactant administration and lung lavage, should be considered in selected cases. With judicious use of available modes of ventilation and adjunctive therapies, infants with even the most severe MAS can usually be supported through the disease, with an acceptably low risk of short- and long-term morbidities. PMID:22518190

  1. Procalcitonin as Predictor of Bacterial Infection in Meconium Aspiration Syndrome.

    Science.gov (United States)

    K, Mahendiran; Batra, Prerna; Faridi, M M A; Singh, N P

    2017-12-29

     There is a lack of definite consensus on indications for initiating antibiotics in neonates with meconium aspiration syndrome (MAS), instigating researchers to search for a biomarker that can help differentiate MAS from MAS with bacterial infection.  Our primary objective was to compare serum procalcitonin (PCT) levels in full-term vigorous neonates having MAS with or without bacterial infection.  Seventy term vigorous neonates with diagnosis of MAS were enrolled. Blood samples were taken for sepsis screen, C-reactive protein (CRP), PCT, and blood culture at 6 ± 2 hours of respiratory distress. Neonates were categorized into group 1 (MAS without bacterial infection) and group 2 (MAS with bacterial infection) based on blood culture. The duration of our study was 18 months.  Mean ± standard deviation PCT level was 2.52 ± 3.99 in group 1 and 2.71 ± 4.22 in group 2, which was comparable. At cutoff of 0.1 ng/mL, PCT had a sensitivity of 90% and specificity of 8% in detecting bacterial infection. Mean total leukocyte count, absolute neutrophil count, immature to total leucocyte ratio, microerythrocyte sedimentation rate, and CRP were comparable.  Though PCT is an early and reliable marker of neonatal infection, the levels were increased in neonates with MAS irrespective of the presence of bacterial infection. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  2. Meconium aspiration syndrome: risk factors and predictors of severity.

    Science.gov (United States)

    Oliveira, Cláudia Patrícia Lourenço; Flôr-de-Lima, Filipa; Rocha, Gustavo Marcondes Duarte; Machado, Ana Paula; Guimarães Pereira Areias, Maria Hercília Ferreira

    2017-12-08

    To identify risk factors and predictors of severity associated with meconium aspiration syndrome (MAS) in the patients admitted to the neonatal intensive care unit (NICU). Retrospective study including newborns admitted, between 2005 and 2015, with a diagnosis of MAS. Of the newborns admitted to the NICU, 0.66% were diagnosed with MAS. These had higher prevalence of caesarean delivery (p < .001), nonreassuring or abnormal cardiotocography (CTG) (p < .001), intrapartum maternal fever (p = .002), Apgar scores at the first minute <7 (p < .001) and need of endotracheal intubation at birth (p < .001). Newborns with severe MAS had higher median reactive C protein (86.9 versus 9.65, p = .001) and 73.3% had pulmonary hypertension (p = .027). They required significantly more days of oxygen therapy, mechanical ventilation, nitric oxide, inotropic, and surfactant therapy, as well as longer hospital stay. Nonreassuring or abnormal CTG and low Apgar score at the first minute were established as risk factors for MAS and need of surfactant therapy as a predictor of severity.

  3. Meconium aspiration syndrome - the experience of a tertiary center

    Directory of Open Access Journals (Sweden)

    M.C. Espinheira

    2011-03-01

    Full Text Available Background: Approximately 5% of infants born with a meconium-stained amniotic fluid (MSAF develop meconium aspiration syndrome (MAS. Aim: The aims of this study were to analyse demographic data, morbidity and mortality associated with MAS and to identify possible risk factors. Methods: Retrospective chart review of newborns with MAS delivered at a tertiary center from January 1st, 1997 to December 31st, 2008. Results: MAS was responsible for 1.4% of all Neonatal Intensive Care Unit (NICU admissions, with a trend towards a decreasing incidence during the duration of the study, especially in the cases of thin meconium. Seventy two newborns were analysed during the study period: 55.6% (n = 40 were of the female gender, 62.5% were delivered by caesarean section, 93% had > 36 weeks of gestational age and 91.2% had a birth weight over 2500 g. Sixty-nine percent had an Apgar score 36 semanas e 91,2% com peso ao nascimento > 2500g. 69% dos recém-nascidos apresentaram Índice de Apgar < 7 no 1.° minuto e 23,6% Índice de Apgar < 7 no 5.° minuto; bradicardia fetal foi observada em 26,4% dos recém-nascidos e taquicardia em 1,4%. A presença de mecónio no líquido amniótico condicionou o desenvolvimento de hipóxia (58,3%, necessidade de ventilação mecânica (43,1%, acidose respiratória e/ou metabólica (30,6%, hipertensão pulmonar (11,1% e encefalopatia hipóxico-isquémica (29,2%. A taxa de mortalidade foi de 2,8%. A presença de mecónio espesso esteve associada a maiores taxas de morbilidade e mortalidade. Conclusão: O número de internamentos por SAM tem vindo a diminuir principalmente devido ao decréscimo das admissões por líquido amniótico tingido de mecónio, enquanto o número de casos de mecónio espesso tem permanecido constante ao longo dos anos. O Índice de Apgar < 7 no 1.° minuto e a presença de sinais de sofrimento fetal durante o trabalho de parto apresentaram relação com a SAM. A morbilidade associada à SAM permanece

  4. High frequency oscillatory ventilation in meconium aspiration syndrome

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    José Nona

    2009-03-01

    Full Text Available Objective: To evaluate and compare the management and associated morbidity in inborn and outborn babies with meconium aspiration syndrome admitted to the Neonatal Intensive Care Unit and ventilated with high frequency oscillatory ventilation. Methods: A retrospective cohort study with a review of clinical data from newborns, admitted to the Neonatal Intensive Care Unit during a six-year period (from 1999 to 2004 and ventilated with early high frequency oscillatory ventilation, first intention in inborns and immediately after Neonatal Intensive Care Unit arrival in outborns. Rresults: In the present study, 27 newborns were included: 12 inborn and 15 outborn infants. Severity criteria were similar in both groups. The pulmonary morbidity associated was severe persistent pulmonary hypertension - 12 (seven outborns, pneumothorax - five (three outborns, interstitial emphysema – two (one outborn and pulmonary hemorrhage – one outborn. Hypoxic-ischemic encephalopathy II-III occurred in six newborns (four outborns. The therapeutic procedures were surfactant administration in 22 newborns (13 outborns, nitric oxide in 12 newborns (7 outborns and magnesium sulphate in four newborns (three outborns. The median length of ventilation was six days (inborn infants: four and half days; outborn infants: ten days and the median length of oxygenation supply was ten days (inborn infants: four and half days; outborn infants: 15 days. The median length of stay was 13 days (inborn infants: 11 days; outborn infants: 16 days. One outborn infant died. Cconclusions: With this ventilation strategy, we have found no significant statistical differences between the two newborn groups, except for the length of oxygenation supply that was longer in the Outborn Group.

  5. Paraneoplastic Cushing Syndrome Due To Wilm's Tumor.

    Science.gov (United States)

    Faizan, Mahwish; Manzoor, Jaida; Saleem, Muhammad; Anwar, Saadia; Mehmood, Qaiser; Hameed, Ambreen; Ali, Agha Shabbir

    2017-05-01

    Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, along with histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed.

  6. Why does meconium cause meconium aspiration syndrome? Current concepts of MAS pathophysiology.

    Science.gov (United States)

    van Ierland, Y; de Beaufort, A J

    2009-10-01

    One in every 7 pregnancies ends with meconium-stained amniotic fluid and approximately 5% of these infants develop the meconium aspiration syndrome (MAS). MAS is a severe disease of the (mainly) term neonate, characterized by respiratory distress, pulmonary inflammation, persistent pulmonary hypertension and chronic hypoxia. The pathophysiology of MAS is multifactorial and complex. In this article, we discuss the mechanical and chemical effects of meconium on a newborn's airway, meconium-induced inflammation, mediated by proinflammatory cytokines and chemokines, the complement system and the proinflammatory enzyme phospholipase A2. Furthermore, we focus on MAS-related apoptotic cell death, causing severe acute lung injury due to damage and detachment of lung airway and alveolar cells. Finally, risk factors for MAS development to identify those newborns that develop MAS and those who do not are discussed.

  7. Meconium aspiration syndrome: a role for fetal systemic inflammation.

    Science.gov (United States)

    Lee, JoonHo; Romero, Roberto; Lee, Kyung A; Kim, Eun Na; Korzeniewski, Steven J; Chaemsaithong, Piya; Yoon, Bo Hyun

    2016-03-01

    Meconium aspiration syndrome (MAS) is a leading cause of morbidity and mortality in term infants. Meconium-stained amniotic fluid (MSAF) occurs in approximately 1 of every 7 pregnancies, but only 5% of neonates exposed to MSAF develop MAS. Why some infants exposed to meconium develop MAS while others do not is a fundamental question. Patients with MSAF have a higher frequency of intraamniotic inflammation/infection than those with clear fluid. We propose that fetal systemic inflammation is a risk factor for the development of MAS in patients with MSAF. We sought to investigate whether intraamniotic inflammation and funisitis, the histopathologic landmark of a fetal inflammatory response, predispose to MAS. A prospective cohort study was conducted from 1995 through 2009. Amniotic fluid (AF) samples (n = 1281) were collected at the time of cesarean delivery from women who delivered singleton newborns at term (gestational age ≥38 weeks). Intraamniotic inflammation was diagnosed if the AF concentration of matrix metalloproteinase-8 was >23 ng/mL. Funisitis was diagnosed by histologic examination if inflammation was present in the umbilical cord. The prevalence of MSAF was 9.2% (118/1281), and 10.2% (12/118) of neonates exposed to MSAF developed MAS. There were no significant differences in the median gestational age or umbilical cord arterial pH at birth between neonates who developed MAS and those who did not (each P > .1). Mothers whose newborns developed MAS had a higher median of AF matrix metalloproteinase-8 (456.8 vs 157.2 ng/mL, P < .05). Newborns exposed to intraamniotic inflammation had a higher rate of MAS than those who were not exposed to intraamniotic inflammation [13.0% (10/77) vs 0% (0/32), P = .03], as did those exposed to funisitis [31.3% (5/16) vs 7.3% (6/82); relative risk, 4.3; 95% confidence interval, 1.5-12.3]. Among the 89 newborns for whom both AF and placental histology were available, MAS was more common in patients with both intraamniotic

  8. An Adult Patient with Pneumonia Due to Aspiration of Dental Prosthesis

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    Samil Gunay

    2014-03-01

    Full Text Available In patients with a new and suddenly beginning of bronchial asthma and repetitive respiratory infection, one should consider the possibility of obstruction by a foreign body. In this case we present a male patient with pneumonia due to aspiration of dental prosthesis. A 32-year-old man presented with a 4-month history of dyspnea, chest pain and new    compliants of diaphoresis and fever persistend despite of antibiotic treatment. Then the patient consultated to our chest surgery department. After the physical examination of patient we made the fiberoptic  flexible bronchoscopy according to the uncertain opasity seen in the chest radiography. Later in the operating room, with the patient under general anesthesia, we extracted the aspirated foreign body, dental prosthesis, by rigid bronchoscopy. We should think about the possibility of foreign body aspiration for the patients with suddenly beginning of the complaints such as, bronchial asthma, repetitive respiratory infection, diaphoresis, chest pain and fever. Sometimes people hides the true anamnesis because of be ashamed or amnesia.

  9. Toxic effects of different meconium fractions on lung function: new therapeutic strategies for meconium aspiration syndrome?

    Science.gov (United States)

    Saugstad, O D; Tølløfsrud, P A; Lindenskov, P; Drevon, C A

    2008-12-01

    To review and summarize experimental data examining the effects of different fractions of meconium, and to test the effect of albumin on meconium aspiration both as prophylactic and rescue treatment. Newborn piglets 2 to 5 days of age were made hypoxic and then instilled meconium or fractions of meconium intratracheally. Meconium-added albumin and albumin instilled after meconium were also tested. Lung function and inflammatory cytokines were measured. Both the lipid- and water-soluble fractions induce inflammation in the lungs with elevation of inflammatory cytokines. When meconium was mixed with albumin, the inflammatory effects of meconium were significantly ameliorated. Rescue therapy with intratracheal albumin 5 min after the meconium aspiration syndrome was induced also improved lung function. These results indicate that at least part of the symptoms seen in the meconium aspiration syndrome could be prevented by blocking the active substances of meconium such as bile acids and free fatty acids.

  10. Rothia dentocariosa Septicemia without Endocarditis in a Neonatal Infant with Meconium Aspiration Syndrome

    Science.gov (United States)

    Shin, Jeong Hwan; Shim, Jae Dong; Kim, Hye Ran; Sinn, Jong Beom; Kook, Joong-Ki; Lee, Jeong Nyeo

    2004-01-01

    Rothia dentocariosa, a gram-positive coccoid- to rod-shaped bacterium with irregular morphology, is a rare cause of bacteremia in patients without endocarditis. We report the first case of R. dentocariosa septicemia without endocarditis, which occurred in a neonatal infant with meconium aspiration syndrome. PMID:15472374

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

    Science.gov (United States)

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

    2015-12-01

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

  12. An Investigation of the Mortality Rate and Risk Factors in Newborn Infants With Meconium Aspiration Syndrome

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    Sabzehei

    2016-08-01

    Full Text Available Background One of the serious challenges facing neonatal medicine is meconium aspiration syndrome, delays in the treatment of which can lead to high mortality. Objectives This study was designed and conducted with the aim of determining the mortality rate and risk factors affecting this rate in newborn infants with meconium aspiration syndrome. Methods This study was conducted as a retrospective descriptive research on newborn infants with meconium aspiration syndrome hospitalized at the neonatal intensive care unit (NICU of Fatemieh and Be’sat hospitals in Hamadan city during a 10-year period from 2004 to 2014. Demographic information of the mother and the newborn, hospitalization course, the need for mechanical ventilation, and complications and outcomes of disease were extracted and were analyzed using the SPSS software version 22. Results Sixty-three newborn infants, diagnosed with meconium aspiration syndrome, were entered in this study, 40% of them were male, 85.7% wighed more than 2500 g, and 17.5% were post term, 25.3% had a five-minute Apgar Score (AS5min of less than seven, 39.6% were nonvigorous at birth, 31.8% needed to be placed on mechanical ventilation, and 14.3% died during the hospitalization course. There was a significant relationship between the need for mechanical ventilation, nonvigorous state at the birth, complications of disease and mortality rate. Conclusions Despite the progress made in medicine, meconium aspiration syndrome is still one of the causes of newborn infants’ mortality. The mortality and morbidity rates can be reduced by improvement in perinatal care, prevention of post term delivery, timely caesarean and effective neonatal resuscitation at birth.

  13. Ranitidine and sodium citrate as prophylaxis against acid aspiration syndrome in obstetric patients undergoing caesarean section.

    Science.gov (United States)

    Lim, S K; Elegbe, E O

    1992-12-01

    The efficacy of a single oral dose of 0.3M of sodium citrate alone as a prophylaxis against acid aspiration syndrome in obstetric patients undergoing LSCS (elective and emergency) was compared with that of intravenous ranitidine. One group of patients was given 30 ml of 0.3M sodium citrate orally just before the induction of anaesthesia while the other group was given 50mg of ranitidine intravenously together with the 30ml of 0.3M sodium citrate. This was done for both the elective and the emergency groups of patients. There was no significant difference in the mean pH of the gastric aspirate obtained from those given sodium citrate alone and those given sodium citrate and ranitidine in either the elective or the emergency group. However, in the emergency group, those who were given sodium citrate alone tend to have a larger volume of gastric aspiration when compared with those given ranitidine together with the sodium citrate. There were significantly more patients with gastric aspirates of more than 25ml in the emergency group to which sodium citrate alone was given. It would appear that supplementing an intravenous dose of ranitidine with the oral dose of sodium citrate is useful in emergency LSCS as a prophylaxis against acid aspiration syndrome.

  14. Bronchoalveolar lavage plus surfactant in a piglet model of meconium aspiration syndrome.

    Science.gov (United States)

    Colvero, Mauricio Obal; Fiori, Humberto Holmer; Fiori, Renato Machado; Luz, Jorge Hecker; de Paula, Davi; Oppermann, Christina; Pitrez, Paulo Márcio; da Silva, Vinícius Duval; Colvero, Aline Pieruccini

    2008-01-01

    Meconium aspiration produces airway obstruction and surfactant inhibition. Bronchoalveolar lavage (BAL) and surfactant replacement have been proposed as treatments for the syndrome. To evaluate the effect of BAL with normal saline followed by a supplementary dose of surfactant in a piglet model of meconium aspiration syndrome. 15 newborn piglets were used in the study. The animals were ventilated with fixed settings. After inhalation of 4 ml/kg of diluted meconium, the piglets were randomized into three groups: group I (n = 5) - tracheal aspiration without BAL; group II (n = 5) - BAL with normal saline (15 ml/kg), and group III (n = 5) - BAL with normal saline (15 ml/kg) followed by a supplementary dose of surfactant (Curosurf(R) 100 mg/kg). Arterial blood gas samples were obtained 30 min and 6 h after the inhalation of meconium. A significant increase of PaO(2 )values at 6 h after treatment was only observed in group III (from 51 +/- 13 to 189 +/- 115 mm Hg; p = 0.04). At this time, PaO(2) in group III was significantly higher compared to group II (189 +/- 115 and 37 +/- 11 mm Hg, respectively; p = 0.023) and showed a borderline significance when compared to group I (p = 0.066). BAL with normal saline followed by a supplementary dose of surfactant may improve oxygenation in an experimental piglet model of meconium aspiration syndrome. Copyright (c) 2007 S. Karger AG, Basel.

  15. Pseudotumour cerebri syndrome due to cryptococcal meningitis.

    Science.gov (United States)

    Cremer, P D; Johnston, I H; Halmagyi, G M

    1997-01-01

    Three cases are reported of the pseudotumour cerebri syndrome-that is, intracranial hypertension without mass lesion or enlarged ventricles, due to cryptococcal meningitis. In these patients the papilloedema was successfully treated with optic nerve sheath decompression, and the intracranial hypertension with lumboperitoneal CSF shunting. These cases support the concept that pseudotumour cerebri is a syndrome of intracranial hypertension that can be due to any disorder producing obstruction of the CSF pathways at the level of the arachnoid villi. This concept is important because it directs therapy to normalise the intracranial pressure and preserve vision. Images PMID:9010409

  16. Anti-inflammatory treatment of meconium aspiration syndrome: benefits and risks.

    Science.gov (United States)

    Mokra, Daniela; Mokry, Juraj; Tonhajzerova, Ingrid

    2013-06-01

    Meconium aspiration syndrome (MAS) is a major cause of respiratory insufficiency in the term and post-term newborns. There are several pathomechanisms participating in this disorder, particularly the airway obstruction, surfactant dysfunction, inflammation, lung edema, pulmonary vasoconstriction and bronchoconstriction. Inflammatory changes resulting from meconium aspiration cause severe impairment of lung parenchyma and surfactant, and influence the reactivity of both vascular and airway smooth muscle. Therefore, anti-inflammatory drugs may be of benefit in the management of MAS. This article reviews the pharmacological actions and side effects of various anti-inflammatory drugs used up to now in the experimental models of MAS and in the treatment of newborns with meconium aspiration. Copyright © 2013. Published by Elsevier B.V.

  17. Total liquid ventilation efficacy in an ovine model of severe meconium aspiration syndrome.

    Science.gov (United States)

    Avoine, Olivier; Bossé, Dominick; Beaudry, Benoit; Beaulieu, Alexandre; Albadine, Roula; Praud, Jean-Paul; Robert, Raymond; Micheau, Philippe; Walti, Hervé

    2011-05-01

    To test the hypothesis that total liquid ventilation enables a more effective and better tolerated lavage than a bronchoalveolar lavage performed with diluted surfactant in a newborn ovine model of severe acute meconium aspiration syndrome. Prospective, randomized, interventional study. Animal research laboratory at the Faculté de médecine et des sciences de la santé de l'université de Sherbrooke, Sherbrooke, Canada. Twenty-three newborn lambs, meconium aspiration syndrome was obtained by instillation of a 25% dilution of human meconium in saline (1 mL/kg × 2). Lambs were then randomized in 12 total liquid ventilation-bronchoalveolar lavage (minute ventilation of 160 mL/kg/min with perfluorodecalin) vs. 11 bronchoalveolar lavage performed with diluted surfactant (conventional ventilation + 30 mL/kg in two aliquots bronchoalveolar lavage with 5 mg/mL BLES surfactant). Surviving lambs were ventilated for a total of 4 hrs and euthanized. Arterial blood gases, systemic and pulmonary hemodynamic parameters using the thermodilution method, percentage of recovered meconium, and lung histologic scores. Total liquid ventilation bronchoalveolar lavage enabled a significantly higher PaO2 throughout the experiment. PaCO2, pH, and hemodynamic parameters were comparable for both groups except for an increase in mean pulmonary arterial pressure during total liquid ventilation. Total liquid ventilation bronchoalveolar lavage allowed for 43 ± 14% of the instilled meconium to be removed vs. 28 ± 10% for bronchoalveolar lavage performed with diluted surfactant (p = .022). Lung histologic analysis showed no difference between total scores. Total liquid ventilation bronchoalveolar lavage is well tolerated and more effective in terms of meconium washout and gas exchange than bronchoalveolar lavage performed with diluted surfactant in this experimental model of severe meconium aspiration syndrome. These positive results open the way to further experiments in our ovine model

  18. Prevention of meconium aspiration syndrome: an update and the Baylor experience

    Science.gov (United States)

    Charsha, Dianne S.; Chiruvolu, Arpitha

    2009-01-01

    The approach to preventing meconium aspiration syndrome (MAS) in the newborn has changed markedly over the last 30 years. In the late 1970s, all infants born through meconium-stained amniotic fluid (MSAf) had upper-airway suctioning before delivery of the shoulders and then had tracheal intubation and suctioning in the delivery room. Now suctioning of the upper airway is no longer recommended, and only “depressed” infants are intubated for tracheal suctioning. The incidence of MAS and the associated high mortality rate have both declined significantly over time. This is due to improved antepartum and intrapartum obstetrical management as well as the postdelivery resuscitation of the neonate born through MSAf. MAS is no longer considered to be solely a postnatal disorder that is preventable with routine delivery room suctioning of the trachea; rather, it is considered a complex and multifactorial disorder with antenatal as well as intrapartum factors. The incidence and severity of MAS have been positively affected by a combined obstetrical and neonatal approach to the infant born through MSAf. In this article, we detail our experience at Baylor University Medical Center with MAS and its prevention and review the current literature. PMID:19381312

  19. Racial disparity in meconium-stained amniotic fluid and meconium aspiration syndrome in the United States, 1989-2000.

    Science.gov (United States)

    Sriram, Sudhir; Wall, Stephen N; Khoshnood, Babak; Singh, Jaideep K; Hsieh, Hui-Lung; Lee, Kwang-Sun

    2003-12-01

    To estimate the prevalence of meconium-stained amniotic fluid and meconium aspiration syndrome, as well as the differences in case fatality from meconium aspiration syndrome, between non-Hispanic black and non-Hispanic white infants. We studied non-Hispanic black and non-Hispanic white live births with weights greater than 2.5 kg and gestational ages greater than 35 weeks, using the linked US birth and infant death cohorts for three periods: 1989-1991, 1995-1997, and 1998-2000. We used logistic regression to estimate the risks of meconium-stained amniotic fluid and meconium aspiration syndrome and to estimate the case fatality of meconium aspiration syndrome by maternal race, birth weight, period, and pregnancy complications. Risk of meconium-stained amniotic fluid was 80% higher in non-Hispanic blacks when compared with non-Hispanic whites (birth weight-adjusted odds ratio [OR], 1.81, 95% confidence interval [CI] 1.80, 1.82). The prevalence of pregnancy complications did not explain this racial disparity. Risk of meconium aspiration syndrome in non-Hispanic blacks was 67% higher when compared with non-Hispanic whites (birth weight-adjusted OR 1.67, 95% CI 1.64, 1.70). The case fatality rate of meconium aspiration syndrome was similar between non-Hispanic blacks and non-Hispanic whites in the three periods, with rates of 15.5, 15.2, and 11.2 per 1000 in non-Hispanic blacks and 13.5, 11.2, and 10.1 per 1000 in non-Hispanic whites in 1989-1991, 1995-1997, and 1998-2000, respectively. Our results suggest that when compared with non-Hispanic whites, non-Hispanic blacks are at significantly greater risk for meconium-stained amniotic fluid and meconium aspiration syndrome but not for meconium aspiration syndrome case fatality.

  20. latrogenic chronic compartment syndrome of leg due to ruptured ...

    African Journals Online (AJOL)

    A 45 year old male complaining of chronic pain and swelling of his left calf with features suggestive of chronic compartment syndrome following therapeutic aspiration of a popliteal cyst has been presented. It is a rare type of presentation which has not been reported to the knowledge of the authors. Review of literature and

  1. Continuous positive airway pressure and conventional mechanical ventilation in the treatment of meconium aspiration syndrome.

    Science.gov (United States)

    Goldsmith, J P

    2008-12-01

    Meconium aspiration syndrome (MAS) is a complex syndrome that ranges in severity from mild respiratory distress to severe respiratory failure, persistent pulmonary hypertension of the newborn and sometimes death. Understanding of the syndrome's complicated pathophysiology will help determine the appropriate treatment strategy, including the use of continuous positive airway pressure (CPAP), conventional mechanical ventilation (CMV) and other therapies. Approximately 30 to 50% of infants diagnosed with MAS will require CPAP or mechanical ventilation. The optimum modes of ventilation for MAS are not known. Very few studies have been conducted to determine 'best' ventilatory strategies. Despite the introduction, over the last two decades, of innovative ventilatory treatments for this disease (for example, surfactant, high-frequency ventilation, inhaled nitric oxide, extracorporeal membrane oxygenation), the majority of infants can be successfully managed with CPAP or mechanical ventilation alone.

  2. Controlled hypothermia may improve surfactant function in asphyxiated neonates with or without meconium aspiration syndrome.

    Science.gov (United States)

    Autilio, Chiara; Echaide, Mercedes; De Luca, Daniele; Pérez-Gil, Jesús

    2018-01-01

    Whole-body hypothermia (WBH) is used to improve neurological outcomes in perinatal asphyxia. Recent studies suggested a beneficial effect of hypothermia for some types of acute respiratory failure. However, no data are available about the biophysical function of human surfactant during WBH. We investigated whether WBH improves surfactant biophysical properties in asphyxiated neonates with or without meconium aspiration syndrome (MAS). Non-bronchoscopic bronchoalveolar lavage (BAL) has been collected from 10 asphyxiated neonates (2 with MAS, 8 with no lung disease (NLD)) at different time-points (pre-WBH, 24h, 48h, 72h of WBH and post-WBH). Surfactant was extracted and tested by captive bubble surfactometry (CBS) in triplicate, at 37°C and 33.5°C, through initial adsorption and dynamic compression-expansion cycling. Phosphatidylcholine and cholesterol were assayed using enzymatic methods. Clinical data were recorded in real-time. Minimum surface tension under dynamic testing was significantly improved as assessed at 33.5°C compared with its behavior at 37°C in NLD neonates: the difference was evident after at least 72h of WBH and remained significant at 6h after rewarming (72h: p = 0.009; rewarming: p = 0.040). Similar results were obtained in MAS patients whose surfactant activity improved already at 48h of hypothermia. Total cholesterol showed a trend to increase at the first 24-48h of hypothermia in NLD patients. Conversely, hypothermia seemed to reduce the excess of exogenous cholesterol in MAS surfactant. Surfactant biophysical properties may improve after 48-72h of WBH in asphyxiated neonates and the improvement is maintained shortly after rewarming. Due to study limitations, further studies are warranted to better clarify the effects of hypothermia on surfactant activity.

  3. Controlled hypothermia may improve surfactant function in asphyxiated neonates with or without meconium aspiration syndrome.

    Directory of Open Access Journals (Sweden)

    Chiara Autilio

    Full Text Available Whole-body hypothermia (WBH is used to improve neurological outcomes in perinatal asphyxia. Recent studies suggested a beneficial effect of hypothermia for some types of acute respiratory failure. However, no data are available about the biophysical function of human surfactant during WBH. We investigated whether WBH improves surfactant biophysical properties in asphyxiated neonates with or without meconium aspiration syndrome (MAS.Non-bronchoscopic bronchoalveolar lavage (BAL has been collected from 10 asphyxiated neonates (2 with MAS, 8 with no lung disease (NLD at different time-points (pre-WBH, 24h, 48h, 72h of WBH and post-WBH. Surfactant was extracted and tested by captive bubble surfactometry (CBS in triplicate, at 37°C and 33.5°C, through initial adsorption and dynamic compression-expansion cycling. Phosphatidylcholine and cholesterol were assayed using enzymatic methods. Clinical data were recorded in real-time.Minimum surface tension under dynamic testing was significantly improved as assessed at 33.5°C compared with its behavior at 37°C in NLD neonates: the difference was evident after at least 72h of WBH and remained significant at 6h after rewarming (72h: p = 0.009; rewarming: p = 0.040. Similar results were obtained in MAS patients whose surfactant activity improved already at 48h of hypothermia. Total cholesterol showed a trend to increase at the first 24-48h of hypothermia in NLD patients. Conversely, hypothermia seemed to reduce the excess of exogenous cholesterol in MAS surfactant.Surfactant biophysical properties may improve after 48-72h of WBH in asphyxiated neonates and the improvement is maintained shortly after rewarming. Due to study limitations, further studies are warranted to better clarify the effects of hypothermia on surfactant activity.

  4. Albumin lavage does not improve the outcome of meconium aspiration syndrome.

    Science.gov (United States)

    Salvesen, Bodil; Mollnes, Tom Eirik; Saugstad, Ola Didrik

    2008-10-01

    Meconium aspiration syndrome is still a serious condition with high mortality and morbidity. No specific treatment is yet available, although surfactant is known to reduce the need for extracorporeal membrane oxygenation and surfactant lavage has shown promising results in animal studies. Our group has previously shown reduced oxygenation index in an experimental model of meconium aspiration syndrome in newborn pigs when mixing albumin with meconium before endotracheal instillation. Lung compliance increased when albumin was instilled after meconium as a rescue. The aim of this study was to combine the effect of albumin and lavage. Sixteen newborn pigs (six in the meconium-albumin group, six in the meconium group, and four control animals) were anesthetized and tracheotomized. Meconium 4 mL/kg was instilled endotracheally. After five minutes, albumin 15 mL/kg was instilled in the meconium-albumin group followed by endotracheal suctioning. The observation time was six hours. Respiratory and hemodynamic parameters were measured. The terminal complement complex and proinflammatory cytokines were analyzed in plasma. Oxygenation index, ventilatory index, and the terminal complement complex (sC5b-9) increased significantly in both groups, but significantly more in the meconium-albumin group. Compliance decreased, but significantly more in the meconium-albumin group. The terminal sC5b-9 complex increased in both groups, but significantly more in the meconium-albumin group. Tumor necrosis factor-alpha, interleukin (IL)- 1beta, and IL-6 increased significantly in both groups. Albumin-lavage did not improve the outcome of experimental meconium aspiration syndrome.

  5. Spectrophotometric analysis in umbilical cords of infants with meconium aspiration syndrome.

    Science.gov (United States)

    Tuncer, Oguz; Peker, Erdal; Demir, Nihat; Akbayram, Sinan; Kirimi, Ercan

    2013-07-01

    We compared spectrophotometric analysis of the umbilical cords of infants with meconium aspiration syndrome (MAS) or with meconium-stained amniotic fluid (MSAF) and healthy infants. In a prospective study, 15 infants with MAS and 37 infants with MSAF were enrolled. Twenty healthy infants formed a control group. The absorption peak of umbilical cords with meconium was significantly higher in the infants with MAS or MSAF than in controls. Spectrophotometric analysis of the umbilical cords with meconium may be useful to identify developed neonates with MAS or MSAF.

  6. Abdominal Compartment Syndrome due to OHSS

    Directory of Open Access Journals (Sweden)

    Firoozeh Veisi

    2012-03-01

    Full Text Available Abdominal compartment syndrome is a dangerous clinical situation, usually following abdominal injuries&operations. It is seldom observed in patients with gynecologic and obstetric problems. Abdominalcompartment syndrome may be consequence ovarian hyperstimulation syndrome. A 28-year-old womanpresented as a sever ovarian hyperstimulation.The increased IAP indicated that OHSS may beconsidered a compartment syndrome. Abdominal compartment syndrome needs laparotomy orparacentesis for reduction of pressure.

  7. Intratracheal catheter suction removes the same volume of meconium with less impact on desaturation compared with meconium aspirator in meconium aspiration syndrome.

    Science.gov (United States)

    Akazawa, Yohei; Ishida, Takefumi; Baba, Atsushi; Hiroma, Takehiko; Nakamura, Tomohiko

    2010-08-01

    To evaluate the impact of suction technique on the rate of meconium removal, oxygenation, and hemodynamics in an animal experimental model of meconium aspiration syndrome (MAS). MAS was induced in ventilated rabbits using 3.5 ml/kg of 20% human meconium. Tracheal suction with either catheter suction (CS) or meconium aspirator (MA) was performed after meconium instillation. Percentage of meconium collection rate, PaO(2) trends for 2h after tracheal suction, and acute-phase SpO(2) trends were compared between CS and the other three groups, the tube was withdrawn while meconium was aspirated with an MA, then the trachea was reintubated 5, 10 or 15s after suctioning of meconium. Percentage of meconium collection rate and PaO(2) showed no significant differences between groups. The MA group taking 15s for reintubation after meconium suctioning, showed a significantly lower acute-phase SpO(2) than the CS group (Por=90% was also longer in the MA group taking 15s for reintubation than in the CS group (Pmeconium with less impact on desaturation compared with meconium aspiration in an animal model of MAS. Intratracheal CS may be benefit to remove meconium in non-vigorous infants with meconium-stained amniotic fluid at birth. Copyright 2010 Elsevier Ltd. All rights reserved.

  8. Lipid aspiration pneumonia due to gastroesophageal reflux. An unusual radiographic appearance

    Energy Technology Data Exchange (ETDEWEB)

    Wolfson, B.J.; Allen, J.L.; Panitch, H.B.; Karmazin, N.

    1989-08-01

    The development of lipid aspiration pneumonia after chronic nasopharyngeal installation of mineral oil was first described in 1925 by Laughlin. Since that time this entity has been well recognized and numerous substances have been identified or implicated as the aspirated material. The classic radiographic appearance of severe chronic lipid aspiration pneumonia has been described as consisting of intense perihilar infiltrates. However, the radiographic findings are more often non-specific and usually consist of varying degrees of diffuse interstitial infiltrates that tend to be more prominent in the perihilar regions and the right lung. We are reporting a case of biopsy-proven lipid aspiration pneumonia in an infant with known gastro-esophageal reflux (GER) who had medium-chain triglyceride oil administered via nasogastric tube. Serial roentgenograms demonstrated a changing pulmonary pattern from diffuse bilateral interstitial infiltrates initially to a diffuse alveolar pattern at the time of the lung biopsy. Modern medicine has developed new methods for providing nourishment to sick newborns and infants to improve their nutritional status and help them to grow. One such method involves the administration of medium-chain triglycerides (MCT oil) into the GI tract via a nasogastric or nasoenteral tube. The purpose of this report is to describe a significant complication of this method of providing nutrition to an infant with gastroesophageal reflux (GER) and the diagnostic dilemma it presented. (orig.).

  9. Comparative effects of bronchoalveolar lavage with saline, surfactant, or perfluorocarbon in experimental meconium aspiration syndrome.

    Science.gov (United States)

    Rey-Santano, Carmen; Mielgo, Victoria E; Gastiasoro, Elena; Alvarez-Diaz, Francisco J; Lafuente, Hector; Valls-i-Soler, Adolfo; Murgia, Xabier

    2012-05-01

    Today, in meconium aspiration syndrome, treatment focuses on bronchoalveolar lavage, because it removes meconium and proinflammatory factors from airways. This technique might be more effective if different solutions were used such as saline solution, a protein-free surfactant, or a perfluorocarbon, because these would be less inhibited by meconium proteins. Pulmonary physiology research unit, Cruces Hospital. Prospective, randomized study. We studied 24 lambs (meconium (3-5 mL/Kg) and were randomly assigned to one of the following groups (n = 6): control: only continuous mechanical ventilation; saline bronchoalveolar lavage: bronchoalveolar lavage with 30 mL/kg of saline solution; dilute surfactant bronchoalveolar lavage: bronchoalveolar lavage with 32 mL/kg of diluted surfactant (lucinactant, 10 mg/mL); or perfluorocarbon bronchoalveolar lavage: bronchoalveolar lavage with 30 mL/kg of perfluorocarbon. Blood gases, cardiovascular parameters, and pulmonary mechanics were assessed. Meconium instillation produced severe hypoxia, hypercapnia, acidosis, and pulmonary hypertension with impairment of pulmonary mechanics (p meconium aspiration syndrome, bronchoalveolar lavage with diluted lucinactant is an effective therapy producing significant improvements in gas exchange, pulmonary hypertension, and pulmonary mechanics. In addition, bronchoalveolar lavage with perfluorocarbon appears to confer some advantages over lavage with equal volumes of saline or no lavage.

  10. Extracellular histones play an inflammatory role in acid aspiration-induced acute respiratory distress syndrome.

    Science.gov (United States)

    Zhang, Yanlin; Wen, Zongmei; Guan, Li; Jiang, Ping; Gu, Tao; Zhao, Jinyuan; Lv, Xin; Wen, Tao

    2015-01-01

    Systemic inflammation is a key feature in acid aspiration-induced acute respiratory distress syndrome (ARDS), but the factors that trigger inflammation are unclear. The authors hypothesize that extracellular histones, a newly identified inflammatory mediator, play important roles in the pathogenesis of ARDS. The authors used a hydrochloric acid aspiration-induced ARDS model to investigate whether extracellular histones are pathogenic and whether targeting histones are protective. Exogenous histones and antihistone antibody were administered to mice. Heparin can bind to histones, so the authors studied whether heparin could protect from ARDS using cell and mouse models. Furthermore, the authors analyzed whether extracellular histones are clinically involved in ARDS patients caused by gastric aspiration. Extracellular histones in bronchoalveolar lavage fluid of acid-treated mice were significantly higher (1.832 ± 0.698) at 3 h after injury than in sham-treated group (0.63 ± 0.153; P = 0.0252, n = 5 per group). Elevated histones may originate from damaged lung cells and neutrophil infiltration. Exogenous histones aggravated lung injury, whereas antihistone antibody markedly attenuated the intensity of ARDS. Notably, heparin provided a similar protective effect against ARDS. Analysis of plasma from ARDS patients (n = 21) showed elevated histones were significantly correlated with the degree of ARDS and were higher in nonsurvivors (2.723 ± 0.2933, n = 7) than in survivors (1.725 ± 0.1787, P = 0.006, n = 14). Extracellular histones may play a contributory role toward ARDS by promoting tissue damage and systemic inflammation and may become a novel marker reflecting disease activity. Targeting histones by neutralizing antibody or heparin shows potent protective effects, suggesting a potentially therapeutic strategy.

  11. CC10 reduces inflammation in meconium aspiration syndrome in newborn piglets.

    Science.gov (United States)

    Angert, Robert M; Pilon, Aprile L; Chester, Darrin; Davis, Jonathan M

    2007-12-01

    Complications from meconium aspiration syndrome (MAS) remain significant despite a variety of therapeutic interventions. Clara cell protein (CC10) is a novel anti-inflammatory agent that can also inhibit phospholipase A2 (PLA2) (an important component of meconium). The present study examined whether administration of recombinant human CC10 (rhCC10) would reduce inflammation and improve lung function in a piglet model of MAS. Following meconium instillation, piglets exhibited significant physiologic dysfunction that improved significantly after surfactant administration. Analysis of tracheal aspirates revealed significant increases in both tumor necrosis factor (TNF) alpha and interleukin (IL)-8 after meconium instillation. rhCC10-treated animals had significantly lower TNF-alpha levels at 24 h (561 +/- 321 versus 1357 +/- 675 pg/mL, p < 0.05) compared with saline controls. There were no differences between rhCC10-treated and untreated groups with respect to other measured physiologic variables or inflammatory markers, including secretory PLA2 activity. Histologic analyses revealed marked inflammatory infiltrates and thickened alveolar walls, but no significant differences among rhCC10 and control animals. Newborn piglets with MAS have significant physiologic dysfunction, marked inflammatory changes and histologic abnormalities, which was partially counteracted by a single dose of exogenous surfactant and rhCC10.

  12. Factors associated with mortality in newborn infants with meconium aspiration syndrome

    Directory of Open Access Journals (Sweden)

    Asril Aminullah

    2017-02-01

    Full Text Available Meconium aspiration syndrome (MAS is still one of the common causes of morbidity and mortality in neonatal period. A retrospective study was conducted from January 1993 to December 1999, to identify factors associated with mortality in MAS. Univariate analysis disclosed that preeclampsia/eclampsia, sex, Apgar scores, consistency of meconium, and use of mechanical ventilation were significantly associated with mortality in MAS, while gestational age, mode of delivery, hypertension, birth weight, tracheal suctioning, blood cultures, and complications were not. Logistic regression analysis showed that mode of delivery, preeclampsia/eclampsia, Apgar scores, consistency of meconium, and use of mechanical ventilation were associated with mortality in MAS, while other variables were not.

  13. What (not) to do at and after delivery? Prevention and management of meconium aspiration syndrome.

    Science.gov (United States)

    Vain, Nestor E; Szyld, Edgardo G; Prudent, Luis M; Aguilar, Adriana M

    2009-10-01

    Meconium aspiration syndrome (MAS) is a life-threatening disorder in newborn infants. Universal intrapartum suction of infants with meconium stained amniotic fluid (MSAF) and postnatal suction of vigorous infants have been used in an attempt to decrease the incidence and severity of the disease by clearing the airway. Both procedures have been proven fruitless when challenged through randomised control trials (RCTs). Endotracheal intubation and suctioning are currently recommended only for non-vigorous infants. Respiratory failure in infants with MAS is frequently treated initially with conventional or synchronized mechanical ventilation. Surfactant administration and high-frequency ventilation (HFV) are commonly used as rescue therapy for severe cases. Nitric oxide (NO) is added when severe pulmonary hypertension is demonstrated. ECMO is an option when other treatments have failed. In the pathophysiology of severe MAS, asphyxia and pulmonary hypertension are considered to be more important than the obstruction of the airways and/or damage to the lung produced by meconium.

  14. Surfactant for meconium aspiration syndrome in term and late preterm infants.

    Science.gov (United States)

    El Shahed, Amr I; Dargaville, Peter A; Ohlsson, Arne; Soll, Roger

    2014-12-14

    Surfactant replacement therapy has been proven beneficial in the prevention and treatment of neonatal respiratory distress syndrome (RDS). The deficiency of surfactant or surfactant dysfunction may contribute to respiratory failure in a broader group of disorders, including meconium aspiration syndrome (MAS). To evaluate the effect of surfactant administration in the treatment of late preterm and term infants with meconium aspiration syndrome. We searched The Cochrane Library (Issue 4, 2006), MEDLINE and EMBASE (1985 to December 2006), previous reviews including cross-references, abstracts, conference and symposia proceedings, expert informants, and journal handsearching, without language restrictions. We contacted study authors for additional data.We ran an updated search in November 2014 and searched the following sites for ongoing or recently completed trials: www.clinicaltrials.gov; www.controlled-trials.com; and www.who.int/ictrp. Randomised controlled trials which evaluated the effect of surfactant administration in late preterm and term infants with meconium aspiration syndrome are included in the analyses. We extracted data on clinical outcomes including mortality, treatment with extracorporeal membrane oxygenation (ECMO), pneumothorax, duration of assisted ventilation, duration of supplemental oxygen, intraventricular haemorrhage (any grade and severe IVH), and chronic lung disease. We conducted data analyses in accordance with the standards of the Cochrane Neonatal Review Group. Four randomised controlled trials met our inclusion criteria. The meta-analysis of four trials (326 infants) showed no statistically significant effect on mortality [typical risk ratio (RR) 0.98, 95% confidence interval (CI) 0.41 to 2.39; typical risk difference (RD) -0.00, 95% CI -0.05 to 0.05]. There was no heterogeneity for this outcome (I² = 0% for both RR and RD). The risk of requiring extracorporeal membrane oxygenation was significantly reduced in a meta-analysis of two

  15. Role of Meconium in the Reaction of Airways Smooth Musculature in the Newborn with Meconium Aspiration Syndrome (Mas.

    Directory of Open Access Journals (Sweden)

    Hilmi Islami

    2009-11-01

    Full Text Available The role of meconium in the respiratory system was studied in newborns, who died from various causes (250 up to 3000 g of weight. We monitored tracheal rings response to dopamine, serotonin and ethanol in different concentrations (dopamine: 0,05 mg/ml, 0,5 mg/ml, 5 mg/ml; serotonin (5-HT: 10-4, 10-3, 10-2, 10-1 mol/dm3; ethanol: 0,02 ml, 0,5 ml, 1,0 ml; 96%. Tracheal smooth musculature tonus (TSM was examined in 48 tracheal preparations taken after the newborn exitus due to different reasons. Based on functional researche of isolated preparations of tracheas, it may be concluded that: aspiration of me-conium has not changed the response of TSM to dopamine, serotonin and ethanol (p>0,1 in comparison with the control group, which have died due to different lung inflammatory processes (e.g. pneumonia, bronchopneumonia, atelectasis, cerebral hemorrhage. The results suggest that meconium does not potentiate the constricting action of dopamine, serotonin and ethanol in tracheobronchial system. Meconium causes mild relaxation of the TSM through a mechanism that is not intermediated by the products of cyclooxygenases (prostaglandins, prostacyclins from the tracheal epithelium or proteins. Also, as it seems, the direct activity of many tested acids in the smooth musculature has no significant impact on increase of the airways tonus in MAS syndrome.

  16. Role of meconium in the reaction of airways smooth musculature in the newborn with meconium aspiration syndrome (MAS).

    Science.gov (United States)

    Islami, Hilmi; Bexheti, Sadi; Shabani, Ragip; Nuraj, Bajram; Zeqiri, Fehmi; Sukalo, Aziz; Kurtishi, Ilir; Kutllovci, Skender; Qorraj, Hasime; Disha, Mentor

    2009-11-01

    The role of meconium in the respiratory system was studied in newborns, who died from various causes (250 up to 3000 g of weight). We monitored tracheal rings response to dopamine, serotonin and ethanol in different concentrations (dopamine: 0,05 mg/ml, 0,5 mg/ml, 5 mg/ml; serotonin (5-HT): 10-4, 10-3, 10-2, 10-1 mol/dm3; ethanol: 0,02 ml, 0,5 ml, 1,0 ml; 96%). Tracheal smooth musculature tonus (TSM) was examined in 48 tracheal preparations taken after the newborn exitus due to different reasons. Based on functional researche of isolated preparations of tracheas, it may be concluded that: aspiration of meconium has not changed the response of TSM to dopamine, serotonin and ethanol (p>0,1) in comparison with the control group, which have died due to different lung inflammatory processes (e.g. pneumonia, bronchopneumonia, atelectasis, cerebral hemorrhage). The results suggest that meconium does not potentiate the constricting action of dopamine, serotonin and ethanol in tracheobronchial system. Meconium causes mild relaxation of the TSM through a mechanism that is not intermediated by the products of cyclooxygenases (prostaglandins, prostacyclins) from the tracheal epithelium or proteins. Also, as it seems, the direct activity of many tested acids in the smooth musculature has no significant impact on increase of the airways tonus in MAS syndrome.

  17. Nine pulmonary aspiration syndrome cases of atypical clinical presentation, in which the final diagnosis was obtained by histological examinations.

    Science.gov (United States)

    Shimada, Masahiro; Teramoto, Shinji; Matsui, Hirotoshi; Tamura, Atsuhisa; Akagawa, Shinobu; Ohta, Ken; Hebisawa, Akira

    2014-01-01

    While pulmonary aspiration syndrome (PAS) is primarily clinically diagnosed, atypical PAS cases can be misdiagnosed clinically and are more accurately diagnosed histologically. To elucidate clinicopathological features of these rare cases, we examined PAS cases determined by histological examination of transbronchial lung biopsy (TBLB) specimens. Of 6105 TBLB cases investigated from 1990 to 2007, 11 were diagnosed as PAS based on histology. Of these, we examined 9 records in detail, as the medical records for 2 cases were unavailable. Histopathological findings indicated 8 patients with aspiration pneumonia and 1 with diffuse aspiration bronchiolitis. However, the pre-bronchoscopy diagnoses included lung cancer, mycobacteriosis, organizing pneumonia, repetitive pneumonia, fungal infection, and interstitial pneumonia. PAS was not considered before TBLB. Only 4 of the 9 patients developed subjective symptoms including fever and cough with sputum production. Laboratory findings demonstrated elevation of white blood cell (WBC) count in only 1 patient and elevation of C reactive protein (CRP) level in 4 patients. Radiographic examination revealed abnormal findings in the dorsal right lower lobes, which was the most vulnerable site for aspiration pneumonia, and also in the upper and ventral portions of the lung. Although the characteristic findings of PAS were scarce, all patients had conditions predisposing to aspiration; i.e., gastrectomy, excessive alcohol drinking, post-cerebral infarction, and sinobronchial syndrome. We diagnosed 9 PAS patients on the basis of histological findings that were distinct from clinical findings. Despite presenting with variable symptoms and laboratory and radiographic findings, they all exhibited predisposing factors to aspiration. Copyright © 2013 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.

  18. Paraneoplastic cushing syndrome due to wilm's tumor

    International Nuclear Information System (INIS)

    Faizan, M.; Anwar, S.; Hameed, A.; Manzoor, J.; Saleem, M.; Mehmood, Q.; Ali, A. S.

    2017-01-01

    Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, alongwith histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed. (author)

  19. Postmortem endogenous ethanol production and diffusion from the lung due to aspiration of wood chip dust in the work place.

    Science.gov (United States)

    Furumiya, Junichi; Nishimura, Hiroyuki; Nakanishi, Akinori; Hashimoto, Yoshiaki

    2011-07-01

    We report an autopsy case of postmortem ethanol diffusion into the cardiac blood after aspiration of wood chips, although antemortem ethanol consumption was not evident. A man in his twenties, who was loading a truck with small wood chips in a hot, humid storehouse, was accidentally buried in a heap of chips. At the time the body was discovered, 20 h after the accident, rectal temperature was 36°C. Autopsy showed the cause of death to be asphyxia due to obstruction of the airway by aspiration of wood chips. The ethanol and n-propanol levels were significantly higher in the lungs (left, 0.603 and 0.009 mg/g; right, 0.571 and 0.006 mg/g) than in other tissues. A significant difference in ethanol concentration was observed between the left cardiac blood (0.243 mg/g) and the right femoral blood (0.042 mg/g). Low levels of ethanol and n-propanol were detected in the stomach contents (0.105 and 0.001 mg/g, respectively). In order to determine whether aspiration of wood chips affects postmortem ethanol production in the lung, we measured the ethanol and n-propanol levels of homogenized rabbit lung tissue incubated with autoclaved or non-autoclaved wood chips. Levels of ethanol and n-propanol were significantly higher in the homogenates incubated with non-autoclaved chips for 24h. The results of this animal experiment suggested that the ethanol detected in the lung was produced by putrefactive bacteria within the wood chips. After death, the ethanol produced endogenously in the lung appears to have diffused and affected the ethanol concentration of the left cardiac blood. 2011 Elsevier Ireland Ltd. All rights reserved.

  20. Nicolau syndrome due to hyaluronic acid injections.

    Science.gov (United States)

    Andre, Pierre; Haneke, Eckart

    2016-08-01

    Six cases of vascular compromise after hyaluronic injection are reported. Clinical symptoms realized a Nicolau syndrome, which is characterized by immediate pain, livedoid pattern and a few days later by the appearance of scabs and skin necrosis. This type of complication is rare, but may be dramatic and injectors must be aware of that. A thorough knowledge of facial anatomy is mandatory to avoid the risky facial areas. The use of a flexible cannula instead of a sharp needle has much less risk of hurting vessels and must be preferred. The support of the patient is discussed and a treatment protocol is proposed.

  1. Meconium aspiration syndrome in infants of HIV-positive women: a case-control study.

    Science.gov (United States)

    Gupta, Sachin K; Haerr, Pam; David, Richard; Rastogi, Alok; Pyati, Suma

    2016-05-01

    Our aim was to determine whether maternal HIV infection in the current era is associated with an increased incidence of meconium aspiration syndrome (MAS) in their infants. Infants born to 149 HIV-positive women at our hospital over a 5-year period were compared with infants born to HIV-negative women in a retrospective case-control study. Charts of all 298 patients included in the study were reviewed for maternal and infant demographics, HIV treatment, vertical transmission and untoward events at delivery or during the hospital course. When compared with HIV-negative women, a greater proportion of HIV-positive women had meconium-stained amniotic fluid (MSAF), 33% vs. 13%, Pmeconium staining of the amniotic fluid and such drug exposure was more common among our HIV-positive sample, controlling for this and other possible covariates did not greatly reduce the association of HIV status with meconium-related complications of delivery. Infants born to HIV-positive women had significantly more MSAF and MAS than infants born to non-infected women. It is unclear whether this association results from maternal HIV infection itself or from anti-retroviral therapy. Maternal and infant care providers should be prepared for this complication when attending to the deliveries of HIV-positive women.

  2. Tidal Volume Requirement in Mechanically Ventilated Infants with Meconium Aspiration Syndrome.

    Science.gov (United States)

    Sharma, Saumya; Clark, Shane; Abubakar, Kabir; Keszler, Martin

    2015-08-01

    The aim of the study is to test the hypothesis that increased physiologic dead space and functional residual capacity seen in meconium aspiration syndrome (MAS) results in higher tidal volume (VT) requirement to achieve adequate ventilation. Retrospective review of infants with MAS admitted to our hospital from 2000 to 2010 managed with conventional ventilation. Demographics, ventilator settings, VT, respiratory rate (RR), and blood gas values were recorded. Minute ventilation (MV) was calculated as RR × VT. Only VT values with corresponding partial pressure of carbon dioxide (Paco 2) between 35 and 60 mm Hg were included. Mean VT/kg and MV/kg were calculated for each patient. Forty infants ventilated for lung disease other than MAS or pulmonary hypoplasia served as controls. Birth weights of the 28 MAS patients and 40 control infants were similar (3,330 ± 500 g and 3,300 ± 640 g). Two patients in each group required extracorporeal membrane oxygenation. Infants with MAS required 26% higher VT and 42% higher MV compared with controls to maintain equal Paco 2. Infants with MAS require larger VT and higher total MV to achieve similar alveolar ventilation, consistent with pathophysiology of MAS. Our findings provide the first reference data to guide selection of VT in infants with MAS. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  3. Associations of viscosity, stercobilin and bilirubin levels in meconium stained amniotic fluid to meconium aspiration syndrome

    Directory of Open Access Journals (Sweden)

    M. Sholeh Kosim

    2011-04-01

    Full Text Available Background Meconium-stained amniotic fluid (MSAF increases morbidity and mortality in neonates. Meconium aspiration syndrome (MAS occurs in 2-9% of neonates with MSAF. Viscosity of MSAF is associated with the amount of the meconium release. Objective To determine the associations between viscosity and the presence of stercobilin and bilirubin in MSAF with MAS in neonates. Methods This observational cohort study was perfonned with term babies who were born v.ith MSAF in Kariadi Hospital from August 2009 to May 2010. Amniotic fluid specimens were taken at birth and neonates were observed for respiratory symptoms until the 5th day of life. Analysis was done by chi-square test, Fisher's exact test and relative risk. Results The majority of the 48 subjects were male, Mth mean gestational age of 39.9 (SD 1.73 weeks. Classification of MSAF as thick or thin was done by macroscopic examination Mth Kappa test 0.741. The MSAF tested ositively for stercobilin and bilirubin in 12/48 and 17/48 subjects, respectively. Thick MSAF correlated significantly to MAS (P=0.03 Mth a relative risk of 10.1 (95% CI 1.2 to 87 .6, while stercobilin and bilirubin presence did not. Conclusion Thick MSAF was associated Mth lvtAS and was a risk factor for MAS. Stercobilin and bilirubin presence in MSAF were not associated with MAS.

  4. Surfactant with and without bronchoalveolar lavage in an experimental model of meconium aspiration syndrome.

    Science.gov (United States)

    Henn, Roseli; Fiori, Renato Machado; Fiori, Humberto Holmer; Pereira, Manuel Ruttkay; Colvero, Maurício Obal; Ramos Garcia, Pedro Celiny; Padoim, Priscila; Stivanin, Jaqueline Basso

    2016-08-01

    To assess the effect of surfactant administration, preceded or not by bronchoalveolar lavage (BAL) with dilute surfactant, on pulmonary function in experimental severe meconium aspiration syndrome. Twenty-one newborn pigs received 20% meconium in saline intratracheally and were randomly allocated to one of three groups: I, control; II, surfactant only (poractant alfa, 200 mg/kg); or III, dilute surfactant BAL followed by 125 mg/kg surfactant. Arterial blood gases (ABGs), lung compliance, and resistance were assessed. Thirty minutes after treatment, mean PaO2 (mm Hg) increased to 72 in group I, to 106 in group II and to 172 in group III (P=0.01). After 3 h, mean PaO2 (mm Hg) was 70 in group I, 95 in group II and 198 in group III (P<0.01). After 6 h, it was 79 in group I, 110 in group II, and 217 in group III (P=0.01). From the 30-min point onward, there were significant differences among treatment groups in all other parameters and at every point of assessment, except for compliance and resistance 3 h after treatment. BAL with dilute surfactant, followed by an additional dose of surfactant, produced significant improvements in ABGs and pulmonary mechanics as compared with a single dose of surfactant.

  5. Erythropoietin may attenuate lung inflammation in a rat model of meconium aspiration syndrome.

    Science.gov (United States)

    Turhan, Ali Haydar; Atici, Aytuğ; Muşlu, Necati; Polat, Ayşe; Sungur, Mehmet Ali

    2016-05-01

    Inflammation is believed to play a key role in the pathophysiology of meconium aspiration syndrome (MAS). The objective was to determine whether the recombinant human Erythropoietin (rhEPO) pretreatment could attenuate meconium-induced inflammation. In this study, 24 ventilated adult male rats were studied to examine the effects of recombinant human EPO (rhEPO) on meconium-induced inflammation. Seventeen rats were instilled with human meconium (1.5 mL/kg, 65 mg/mL) intratracheally and ventilated for 3 hours. rhEPO (1000 U/kg) (n = 9) or saline (n = 8) was given to the animals. Seven rats that were ventilated and not instilled with meconium served as a sham-controlled group. Analysis of the blood gases, interleukin (IL)-1β, IL-6, IL-8, and tumor necrosis factor (TNF)-α in blood and bronchoalveolar lavage (BAL) fluid samples, and lung tissue myeloperoxidase levels were performed. Intrapulmonary instillation of meconium resulted in the increase of TNF-α (p = 0.005 and p meconium and saline + meconium groups compared with the sham-controlled group. rhEPO pretreatment prevented the increase of BAL fluid IL-1β, IL-6, and IL-8 levels (p meconium-induced derangements.

  6. N-acetylcysteine advancement of surfactant therapy in experimental meconium aspiration syndrome: possible mechanisms.

    Science.gov (United States)

    Kopincová, J; Mokrá, D; Mikolka, P; Kolomazník, M; Čalkovská, A

    2014-01-01

    Meconium aspiration syndrome (MAS) is meconium-induced respiratory failure of newborns associated with activation of inflammatory and oxidative pathways. For severe MAS, exogenous surfactant treatment is used which improves respiratory functions but does not treat the inflammation. Oxidative process can lead to later surfactant inactivation; hence, surfactant combination with antioxidative agent may enhance the therapeutic effect. Young New Zealand rabbits were instilled by meconium suspension and treated by surfactant alone, N-acetylcysteine (NAC) alone or by their combination and oxygen-ventilated for 5 h. Blood samples were taken before and 30 min after meconium application and 30 min, 1, 3 and 5 h after the treatment for evaluating of oxidative damage, total leukocyte count, leukocyte differential count and respiratory parameters. Leukocyte differential was assessed also in bronchoalveolar lavage fluid. NAC alone had only mild therapeutic effect on MAS. However, the combination of NAC and surfactant facilitated rapid onset of therapeutic effect in respiratory parameters (oxygenation index, PaO(2)/FiO(2)) compared to surfactant alone and was the only treatment which prevented neutrophil migration into the lungs, oxidative damage and lung edema. Moreover, NAC suppressed IL-8 and IL-beta formation and thus seems to be favorable agent for improving surfactant therapy in MAS.

  7. High aspartate aminotransferase level predicts poor neurodevelopmental outcome in infants with meconium aspiration syndrome.

    Science.gov (United States)

    Chen, I-Lun; Ou-Yang, Mei-Chen; Chen, Feng-Shun; Chung, Mei-Yung; Chen, Chih-Cheng; Huang, Hsin-Chun

    2014-11-01

    The aim of our study is to clarify the perinatal predictive factors of meconium aspiration syndrome (MAS) with neurodevelopmental delay (ND) in infants. In this retrospective study, data were collected from the infants born between 1990 and 2008. They all had primary diagnosis of MAS. Multivariable analyzed perinatal predictive factors of MAS with ND. The developmental status of these infants was followed at least 2 years with the Wechsler Intelligence Scale for Children. A total of 114 surviving babies met the criteria of MAS. Six babies were defined as ND group. Lower 5-minute Apgar score and diastolic blood pressure were significantly related to the ND group. Elevated asparatate aminotransferase (AST), nucleated red blood cells, and white blood cells at the time of admission were significantly high in ND group. Furthermore, AST had area under the receiver operating characteristic curve of 0.879, (95% confidence interval: 0.801, 0.934), p < 0.0001. At 96 mg/dL, it had 83.33% sensitivity, 80.81% specificity, and negative predictive value of 98.8. Multivariable logistic regression analysis revealed AST was the only significant predictive factor for MAS with ND. Early intervention should be recommended in infants having MAS with high AST level at birth for improving their neurodevelopmental outcomes. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  8. Steven-Johnson syndrome due to unknown drugs [corrected].

    Science.gov (United States)

    Shivamurthy, Raghu Prasada M; Kallappa, Ravindra; Reddy, Shashikala G H; Rangappa, Druva Kumar B

    2012-01-01

    Steven-Johnson syndrome may be considered as a cytotoxic immune reaction to drugs, infections etc. This is a case report of Steven-Johnson syndrome due to an ayurvedic preparation which was used in the treatment of mental retardation in a young girl.

  9. Steven-Johnson syndrome due to ayurvedic drugs

    OpenAIRE

    Shivamurthy, Raghu Prasada M.; Kallappa, Ravindra; Reddy, Shashikala G. H.; Rangappa, Druva Kumar B.

    2012-01-01

    Steven?Johnson syndrome may be considered as a cytotoxic immune reaction to drugs, infections etc. This is a case report of Steven?Johnson syndrome due to an ayurvedic preparation which was used in the treatment of mental retardation in a young girl.

  10. Clinical predictors of lacunar syndrome not due to lacunar infarction

    Directory of Open Access Journals (Sweden)

    Comes Emili

    2010-05-01

    Full Text Available Background Lacunar syndrome not due to lacunar infarct is poorly characterised. This single centre, retrospective study was conducted to describe the clinical characteristics of patients with lacunar syndrome not due to lacunar infarct and to identify clinical predictors of this variant of lacunar stroke. Methods A total of 146 patients with lacunar syndrome not due to lacunar infarction were included in the "Sagrat Cor Hospital of Barcelona Stroke Registry" during a period of 19 years (1986-2004. Data from stroke patients are entered in the stroke registry following a standardized protocol with 161 items regarding demographics, risk factors, clinical features, laboratory and neuroimaging data, complications and outcome. The characteristics of these 146 patients with lacunar syndrome not due to lacunar infarct were compared with those of the 733 patients with lacunar infarction. Results Lacunar syndrome not due to lacunar infarct accounted for 16.6% (146/879 of all cases of lacunar stroke. Subtypes of lacunar syndromes included pure motor stroke in 63 patients, sensorimotor stroke in 51, pure sensory stroke in 14, atypical lacunar syndrome in 9, ataxic hemiparesis in 5 and dysarthria-clumsy hand in 4. Valvular heart disease, atrial fibrillation, sudden onset, limb weakness and sensory symptoms were significantly more frequent among patients with lacunar syndrome not due to lacunar infarct than in those with lacunar infarction, whereas diabetes was less frequent. In the multivariate analysis, atrial fibrillation (OR = 4.62, sensorimotor stroke (OR = 4.05, limb weakness (OR = 2.09, sudden onset (OR = 2.06 and age (OR = 0.96 were independent predictors of lacunar syndrome not due to lacunar infarct. Conclusions Although lacunar syndromes are highly suggestive of small deep cerebral infarctions, lacunar syndromes not due to lacunar infarcts are found in 16.6% of cases. The presence of sensorimotor stroke, limb weakness and sudden onset in a patient

  11. Developing a systems approach to prevent meconium aspiration syndrome: lessons learned from multinational studies.

    Science.gov (United States)

    Bhutani, V K

    2008-12-01

    Passage of fetal bowel movement (meconium) is common (in about one out of six births), and in some the staining of the amniotic fluid is a sign of fetal distress. Inhalation of meconium (aspiration syndrome, in upto one out of five to eight such births) just before or at birth may be preventable by a coordinated approach by well-trained and informed birth attendants. Respiratory failure secondary to meconium aspiration syndrome (MAS) remains a major cause of morbidity and mortality in the neonatal population. Infants with hypoxemic respiratory failure because of MAS, persistent pulmonary hypertension of the newborn and pneumonia/sepsis have an increased survival with extracorporeal membrane oxygenation (ECMO). Other treatment options earlier limited to inotropic support, continuous airway pressure (CPAP), conventional ventilatory management, respiratory alkalosis, paralysis and intravenous vasodilators have been replaced by synchronized intermittent mandatory ventilation (SIMV), high-frequency oscillatory ventilation (HFOV), surfactant and inhaled nitric oxide (iNO). HFOV has been advocated for use to improve lung inflation while potentially decreasing lung injury through volutrauma. Other reports describe the enhanced efficacy of HFOV when combined with iNO. Subsequent to studies reporting that surfactant deficiency or inactivation may contribute to neonatal respiratory failure, exogenous surfactant therapy has been implemented with apparent success. Recent studies have shown that iNO therapy in the neonate with hypoxemic respiratory failure can result in improved oxygenation and decreased need for ECMO. However, these innovative interventions are costly, require a sophisticated infrastructure and are not universally accessible. In this paper, a context of systems-approach for prenatal, natal and postnatal management of babies delivered through meconium stained amniotic fluid (MSAF) so that adverse outcomes are minimized and the least number of babies require

  12. Deterimental complication of meconium aspiration syndrome and their impact on outcome

    International Nuclear Information System (INIS)

    Shaikh, M.; Waheed, K.A.I.; Javaid, S.; Gul, R.

    2016-01-01

    Meconium aspiration syndrome (MAS) is respiratory distress in an infant born through meconium stained amniotic fluid (MSAF) whose signs cannot be otherwise explained. MAS is associated with different complications. Many studies have been conducted in developed world to find the outcome associated with MAS but data from developing countries is lacking. Present study was conducted to determine the impact of chemical pneumonitis, pulmonary hypertension (PHN) and air leak on outcome of new-borns with MAS. Methods: This cross sectional descriptive study was conducted in a tertiary care Neonatology unit. The babies diagnosed with MAS were included in the study. All patients were monitored for development of complications. Outcome in terms of mortality was recorded. Outcome was compared by chi-square test and p-value <0.05 is considered significant. Results: Seventy two babies were included in the study. Mean gestation was 37 ± 0.56 weeks and birth weight was 2.87 ± 0.49 kg. Male to female ratio was 1.57:1 and mean age of admission was 9.59 ± 5.27 hours. Chemical pneumonitis, PHN and chemical pneumonitis with PHN were observed in 23.6%, 20.8% and 26.4% neonates respectively. Mortality rate was 19.44%. The mortality was highest in babies having chemical pneumonitis with PHN (p-value=0.013) followed by chemical pneumonitis group (p-value=0.02) and PHN group (p-value=0.032). Conclusion: About three fourth babies with MAS developed one or more complications. Mortality is significantly increases with the development of complications so as more the complications more is the mortality. (author)

  13. Meconium-stained amniotic fluid and meconium aspiration syndrome: a prospective study.

    Science.gov (United States)

    Bhat, R Y; Rao, A

    2008-09-01

    The incidence of meconium aspiration syndrome (MAS), associated perinatal factors, morbidity and deaths varies widely. This study aimed to assess the perinatal attributes and morbidity associated with MAS. Over a 2-year period, all neonates born through meconium-stained amniotic fluid (MSAF) were observed for respiratory distress (RD). Birth details, chest radiograph (CXR) and clinical course were documented. Neonates with consistent CXR findings whose RD could not otherwise be explained were defined as MAS. Of 409 neonates born through MSAF, meconium was thick in 196 (47.9%). Fifty-five (13.4%) had RD and 45 (11.3%) were consistent with MAS. Six (1.5%) neonates died. Mean (SD) birthweight and gestation of MAS infants were 2721.9 (510.2) g and 38.67 (1.09) weeks, respectively. About one-third were of low birthweight and 28 were born by caesarean section. On univariate analysis, caesarean delivery, meconium in the trachea and thick meconium were the significant perinatal factors for the development of MAS. On multiple regression analysis, thick meconium was the only independent factor for MAS (OR 7.08, 95% CI 3.08-16.27, psyndrome was observed in 12 (26.7%), persistent pulmonary hypertension in seven and severe asphyxia and late sepsis in two each. Thirteen (28.9%) required mechanical ventilation and six (13.3%) died. MAS occurred in 11.3% of neonates born through MSAF. Thick meconium was found to be an important causative factor. Nearly one-third of infants with MAS required mechanical ventilation and 13.3% died.

  14. [Efficacy of pulmonary surfactant therapy in neonates with meconium aspiration syndrome: a meta-analysis].

    Science.gov (United States)

    Luo, Fei-Fei; Yang, Di-Yuan; Chen, Pan; Hua, Zi-Yu

    2012-06-01

    The efficacy of pulmonary surfactant (PS) replacement therapy for meconium aspiration syndrome (MAS) remains controversial. This study aimed to evaluate the efficacy of PS therapy in neonates with MAS by a meta-analysis. Randomized controlled trials (RCTs) on the treatment of MAS with PS were searched electronically in medical debases including PubMed, Science Citation Index, The Cochrane Central Register of Controlled Trials, Ovid, EBSCOhost, BIOSIS previews, Chinese BioMedical Literature Database, Wanfang Database and VIP Chinese Sci-Tech Periodical Database. The Cochrane Handbook 5.0.2 was employed to evaluate methodological quality. RevMan 5.0.25 software was used for the meta-analysis. Eight RCTs including 512 MAS neonates (257 cases in the PS treatment group and 255 cases in the control group) were enrolled in this meta-analysis. The meta-analysis showed that PS treatment reduced oxygenation index (MD=-2.59; 95%CI: -4.33, -0.86; P=0.003), increased arterial oxygen/alveolar oxygen ratio (MD=0.05; 95%CI: 0.05, 0.06; P<0.00001), shortened hospitalization days (MD=-4.94; 95%CI: -7.44, -2.44; P=0.0001) and decreased mortality rate (OR=0.47; 95%CI: 0.24, 0.93; P=0.03) significantly. There were no statistical differences in the durations of mechanical ventilation and oxygen therapy, and the incidences of air leak, pulmonary hemorrhage and intracranial hemorrhage between the PS treatment and control groups. Currently published evidence from RCTs suggests that PS replacement therapy is effective for MAS, however because of the limited quantity and quality of trials enrolled in the study, further evidence from RCTs is needed to prove the efficacy.

  15. Role of distinct phospholipases A2 and their modulators in meconium aspiration syndrome in human neonates.

    Science.gov (United States)

    De Luca, Daniele; Minucci, Angelo; Tripodi, Domenico; Piastra, Marco; Pietrini, Domenico; Zuppi, Cecilia; Conti, Giorgio; Carnielli, Virgilio P; Capoluongo, Ettore

    2011-07-01

    Meconium aspiration syndrome (MAS) is a life-threatening neonatal lung injury, whose pathophysiology has been mainly studied in animal models. In such models, pancreatic secretory phospholipase A2 (sPLA2-IB) and proinflammatory cytokines present in meconium challenge the lungs, catabolising surfactant and harming the alveoli. Locally produced phospholipases might perpetuate the injury and influence clinical pictures and therapeutic approaches. Our aim is to verify whether pulmonary phospholipase A2 (sPLA2-IIA) is involved in the damage and to determine if phospholipases and their modulators are associated with MAS clinical pictures. We studied distinct phospholipases A2 and their modulators in broncho-alveolar lavage (BAL) fluids and in meconium of five MAS neonates and in five control neonates ventilated for extrapulmonary reasons. MAS patients have higher amounts of pulmonary phospholipase (sPLA2-IIA; P = 0.016) and Clara cell secretory protein (CCSP; P = 0.032). The local production of such proteins by the lung is confirmed by their very low levels in meconium. sPLA2-IIA contributes to the higher total enzyme activity in MAS patients, as compared to controls (P = 0.008). Cytosolic phospholipase was not detected in meconium or alveolar fluid. sPLA2 activity and sPLA2-IIA concentrations are correlated with the TNFα and with the release of CCSP. sPLA2 total activity, sPLA2-IIA and TNFα concentrations in BAL fluids correlate with the oxygenation impairment and haemorrhagic lung oedema. Pulmonary sPLA2 is locally produced and contributes to the total sPLA2 activity during MAS. CCSP is also produced in trying to lower the inflammation. Both sPLA2 activity and sPLA2-IIA are significantly correlated with oxygenation impairment and haemorrhagic lung oedema.

  16. Detrimental Complications Of Meconium Aspiration Syndrome And Their Impact On Outcome.

    Science.gov (United States)

    Shaikh, Mehmood; Irfan Waheed, Khawaja Ahmad; Javaid, Sulman; Gul, Rafia; Hashmi, Muhammad Almas; Fatima, Syeda Tehseen

    2016-01-01

    Meconium aspiration syndrome (MAS) is respiratory distress in an infant born through meconium stained amniotic fluid (MSAF) whose signs cannot be otherwise explained. MAS is associated with different complications. Many studies have been conducted in developed world to find the outcome associated with MAS but data from developing countries is lacking. Present study was conducted to determine the impact of chemical pneumonitis, pulmonary hypertension (PHN) and air leak on outcome of new-borns with MAS. This cross sectional descriptive study was conducted in a tertiary care Neonatology unit. The babies diagnosed with MAS were included in the study. All patients were monitored for development of complications. Outcome in terms of mortality was recorded. Outcome was compared by chisquare test and p-value <0.05 is considered significant. Seventy two babies were included in the study. Mean gestation was 37±0.56 weeks and birth weight was 2.87±0.49 kg. Male to female ratio was 1.57:1 and mean age of admission was 9.59±5.27 hours. Chemical pneumonitis, PHN and chemical pneumonitis with PHN were observed in 23.6%, 20.8% and 26.4% neonates respectively. Mortality rate was 19.44%. The mortality was highest in babies having chemical pneumonitis with PHN (p-value=0.013) followed by chemical pneumonitis group (p-value=0.02) and PHN group (p-value=0.032). About three fourth babies with MAS developed one or more complications. Mortality is significantly increases with the development of complications so as more the complications more is the mortality.

  17. Risk factors differentiating mild/moderate from severe meconium aspiration syndrome in meconium-stained neonates.

    Science.gov (United States)

    Choi, Woneui; Jeong, Heejeong; Choi, Suk-Joo; Oh, Soo-Young; Kim, Jung-Sun; Roh, Cheong-Rae; Kim, Jong-Hwa

    2015-01-01

    The aim of this study was to compare the risk factors associated with mild/moderate meconium aspiration syndrome (MAS) with those associated with severe in meconium-stained term neonates. Consecutive singleton term neonates (n=671) with meconium staining at birth from all deliveries (n=14,666) in our institution from January 2006 to December 2012 were included. Both maternal and neonatal variables were examined. Among the study population, for women who underwent the trial of labor (n=644), variables associated with labor were also examined. These variables were compared between the mild/moderate MAS group, the severe MAS group, and the MAS-absent group. MAS developed in 10.6% (71/671) of neonates with meconium staining at birth. Among the neonates with MAS, 81.7% had mild MAS, 5.6% had moderate MAS, and 12.7% had severe MAS. The presence of minimal variability was significantly increased in both the mild/moderate and the severe MAS groups. The frequencies of nulliparity, fetal tachycardia, and intrapartum fever were significantly increased in the mild/moderate MAS group, but not in the severe MAS group. While a longer duration of the second stage of labor was significantly associated with mild/moderate MAS, severe MAS was associated with a shorter duration of the second stage. Notably, low mean cord pH (7.165 [6.850-7.375]) was significantly associated with mild/moderate MAS, but not with severe MAS (7.220 [7.021-7.407]) compared with the absence of MAS (7.268 [7.265-7.271]). Our data suggest the development of severe MAS is not simply a linear extension of the same risk factors driving mild/moderate MAS.

  18. Acute Upper Limb Ischemia due to Cardiac Origin Thromboembolism: the Usefulness of Percutaneous Aspiration Thromboembolectomy via a Transbrachial Approach

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Sung Kwan; Kwak, Hyo Sung; Chung, Gyoung Ho; Han, Young Min [Chonbuk National University Hospital and Medical School, Chonju (Korea, Republic of)

    2011-10-15

    To evaluate the usefulness of percutaneous aspiration thromboembolectomy (PAT) via a transbrachial approach in patients with acute upper limb ischemia. From July 2004 to March 2008, eleven patients with acute upper limb ischemia were enrolled in this study. They were initially treated with thrombolysis (n = 1), PAT (n = 6), or both (n = 4) via a femoral artery approach. However, all of the patients had residual thrombus in the brachial artery, which was subsequently managed by PAT via the transbrachial approach for removal of residual emboli. Successful re-canalization after PAT via a transbrachial approach was achieved in all patients. Two patients experienced early complications: one experienced a massive hematoma of the upper arm due to incomplete compression and was treated by stent deployment. The other patient experienced a re-occlusion of the brachial artery the day after the procedure due to excessive manual compression of the puncture site, but did not show recurrence of ischemic symptoms in the artery of the upper arm. Clinical success with complete resolution of ischemic symptoms was achieved in all patients. PAT via a transbrachial approach is a safe and effective treatment for patients with acute upper limb ischemia.

  19. Mugwort-Mustard Allergy Syndrome due to Broccoli Consumption

    Directory of Open Access Journals (Sweden)

    Yuri Sugita

    2016-01-01

    Full Text Available Pollen-food allergy syndrome (PFAS is a relatively rare form of food allergy which develops in individuals who are sensitized to pollen. Tree pollens, especially birch pollen, frequently induce PFAS; however, the incidence of PFAS due to grass or weed pollens such as ragweed or mugwort is relatively rare. Mugwort-mustard allergy syndrome (MMAS is an example of a PFAS in which individuals sensitized to mugwort may develop an allergy to mustard and experience severe reactions. We herein describe a case of MMAS due to broccoli consumption.

  20. Heterochromia iridis and Horner's syndrome due to paravertebral neurilemmoma.

    Science.gov (United States)

    Sayed, A K; Miller, B A; Lack, E E; Sallan, S E; Levey, R H

    1983-01-01

    A case of heterochromia iridis and Horner's syndrome is reported in a 7-year old girl with paravertebral neurilemmoma. These clinical findings can be useful in the early diagnosis of mediastinal tumors in the paravertebral axis. While typically associated with neuroblastoma, these findings can be due to tumors which are inately benign--in this case neurilemmoma. The mechanism for heterochromia is briefly discussed.

  1. Hybrid management of Paget-Schroetter syndrome due to thoracic outlet syndrome.

    Science.gov (United States)

    De Caridi, Giovanni; Massara, Mafalda; Greco, Michele; Villari, Simona; Squillaci, Domenico; Spinelli, Francesco

    2016-02-01

    Paget-Schroetter syndrome due to thoracic outlet syndrome is a rare but potentially disabling condition that generally affects young patients otherwise healthy. The prompt diagnosis and treatment of Paget-Schroetter syndrome is necessary to avoid major morbidity and long-term disability. The more modern treatment paradigm reported in the current literature consists of hybrid procedures with surgical decompression of the thoracic outlet and endovascular techniques to potentially improve long-term vein patency. However, there seems to be no consensus in the literature with regard to the timing and precise nature of active management, and there is presently no agreed protocol for the optimum management of Paget-Schroetter syndrome. Controversy exists partly because no randomised controlled studies are present in literature. We present a case of Paget-Schroetter syndrome due to thoracic outlet syndrome in a young male patient submitted to a multimodal procedure.

  2. A Case with Repeated Recurrent Acute Coronary Syndrome due to Pseudoephedrine Use: Kounis Syndrome

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    Metin Çeliker

    2014-01-01

    Full Text Available Allergic reaction-associated acute coronary syndrome picture is defined as Kounis syndrome. Although drug use is the most common cause of allergic reaction, foods and environmental factors may also play a role in the etiology. Herein, a case with acute coronary syndrome that developed two times at 8-month interval due to pseudoephedrine use for upper respiratory tract infection is presented.

  3. Anton's syndrome due to cerebrovascular disease: a case report

    Directory of Open Access Journals (Sweden)

    Maddula Mohana

    2009-09-01

    Full Text Available Abstract Introduction Anton's syndrome describes the condition in which patients deny their blindness despite objective evidence of visual loss, and moreover confabulate to support their stance. It is a rare extension of cortical blindness in which, in addition to the injury to the occipital cortex, other cortical centres are also affected, with patients typically behaving as if they were sighted. Case presentation We present a case report of an 83-year-old white woman with cortical blindness as a result of bilateral occipital lobe infarcts. Despite her obvious blindness, illustrated by her walking into objects, the patient expressed denial of visual loss and demonstrated confabulation in her accounts of her surroundings, consistent with a diagnosis of Anton's syndrome. Conclusions A suspicion of cortical blindness and Anton's syndrome should be considered in patients with atypical visual loss and evidence of occipital lobe injury. Cerebrovascular disease is the most common cause of Anton's syndrome, as in our patient. However, any condition that may result in cortical blindness can potentially lead to Anton's syndrome. Recovery of visual function will depend on the underlying aetiology, with cases due to occipital lobe infarction after cerebrovascular events being less likely to result in complete recovery. Management in these circumstances should accordingly focus on secondary prevention and rehabilitation.

  4. Circulação extracorpórea por membrana (ECMO em recém-nascido com insuficiência respiratória por síndrome de aspiração meconial: efeitos da administração de surfactante exógeno Extracorporeal membrane oxygenation (ECMO in a neonate with respiratory distress due to meconium aspiration syndrome: Effect of the administration of exogenous surfactant

    Directory of Open Access Journals (Sweden)

    João Gilberto Maksoud-Filho

    2001-06-01

    Full Text Available OBJETIVO: apresentar a evolução clínica de recém-nascido portador de insuficiência respiratória grave neonatal secundária à Sindrome de Aspiração Meconial tratado por Circulação Extracorpórea por Membrana, ou, conforme o termo consagrado em língua inglesa, ECMO (Extracorporeal Membrane Oxygenation, o efeito do uso de surfactante exógeno neste caso e os custos do procedimento. MÉTODOS: Descrição de um caso de Síndrome de Aspiração Meconial, tratado na UCINE (Unidade de Cuidados Intensivos Neonatais do Instituto da Criança Prof. Pedro de Alcantara, Hospital das Clínicas da Universidade de São Paulo. RESULTADOS: O suporte extracorpóreo teve a duração de 5 dias, sem complicações clínicas ou mecânicas. Surfactante exógeno de origem porcina foi administrado no 4o dia, após o quê observamos uma melhora significativa na complacência pulmonar. O recém-nascido pôde então ser rapidamente decanulado. Os custos do tratamento foram compatíveis com a realidade nacional em relação a um recém-nascido criticamente enfermo. CONCLUSÕES: a ECMO é indicada em casos de insuficiência respiratória neonatal que não respondam a outros tratamentos existentes. Deve ser disponível em Unidades de Tratamento Intensivo (UTIs neonatais de hospitais terciários e ser empregada conforme critérios bem estabelecidos. A utilização de surfactante exógeno aparentemente antecipou a retirada da ECMO e, portanto, deve ser considerada em casos semelhantes. Os custos do tratamento justificam a organização de Equipes de ECMO nessas UTIs.OBJECTIVES: to present the clinical outcome of a newborn with severe respiratory distress secondary to meconium aspiration syndrome and treated by extracorporeal membrane oxygenation (ECMO; and to present the effect of the use of exogenous surfactant in this case and the cost of the procedure. METHODS: Case report of a newborn with meconium aspiration syndrome and treated at the neonatal ICU of the

  5. Aspiration pneumonia

    Science.gov (United States)

    Anaerobic pneumonia; Aspiration of vomitus; Necrotizing pneumonia; Aspiration pneumonitis ... The type of bacteria that caused the pneumonia depends on: Your ... facility, for example) Whether you were recently hospitalized ...

  6. A CLINICORADIOLOGICAL STUDY OF MIDDLE LOBE SYNDROME DUE TO TUBERCULOSIS

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    Saurabh Karmakar

    2016-09-01

    Full Text Available BACKGROUND Although pulmonary tuberculosis is a common disease in India, tuberculosis of right middle lobe is infrequent. Tuberculosis of the right middle lobe leading to chronic collapse is a cause of Right Middle Lobe syndrome. METHODS The patients attended Pulmonary Medicine Outdoor at Era’s Lucknow Medical College, Lucknow from April 2015 to March 2016. The purpose of this study is to describe the clinicoradiological features of patients of middle lobe syndrome due to tuberculosis. All patients presented with cough with or without expectoration, fever, chest pain, haemoptysis and constitutional symptoms like loss of appetite and weight. Chest X-ray PA view revealed ill-defined opacity abutting the right cardiac border. HRCT thorax was done in each case. The diagnosis of tuberculous aetiology was based on (1 History of chronic cough and fever, not responding to antibiotic therapy and constitutional symptoms, (2 A positive tuberculin test using 2 TU of PPD RT 23 and (3 Detection of acid fast bacilli by direct smear or Mycobacterium tuberculosis by polymerase chain reaction in bronchoalveolar lavage. RESULTS Out of 10 patients, 4 (40% were males and 6 (60% were females. The mean ages of the males were 55.8 years and females were 60.8 years and overall mean age was 59 years. Most of the patients were females and belonged to the middle age and old age group. ATT was started in all the patients. CONCLUSIONS Right middle lobe syndrome predominantly affects the older population and the female gender. Although tuberculosis is a common disease in India, Middle Lobe Syndrome is a very rare presentation of the disease. Due to non-specific symptoms and usually normal chest X-ray PA view in Right Middle Lobe Syndrome, we should keep a high index of suspicion to diagnose the condition.

  7. Bronchoalveolar lavage with pulmonary surfactant/dextran mixture improves meconium clearance and lung functions in experimental meconium aspiration syndrome.

    Science.gov (United States)

    Calkovska, Andrea; Mokra, Daniela; Drgova, Anna; Zila, Ivan; Javorka, Kamil

    2008-08-01

    Surfactant lung lavage is a promising approach in the treatment of meconium aspiration syndrome (MAS). We hypothesise that the enrichment of modified natural surfactant with dextran will enhance meconium clearance from the airspaces during lung lavage and improve lung function in experimental MAS. Human meconium (30 mg/ml; 4 ml/kg) was instilled into the tracheal cannula of anaesthetised and paralysed adult rabbits to induce respiratory failure. The animals were then lavaged with saline (Sal), surfactant without (Surf) and with dextran (Surf+dex). Lung lavage (10 ml/kg in three portions) was performed with diluted surfactant (Curosurf, 10 mg/ml, 100 mg/kg) without or with dextran (3 mg/mg of surfactant phospholipids) or saline and the animals were conventionally ventilated with 100% O(2) for an additional hour. Lung functions were measured prior to and after meconium instillation, and 10, 30 and 60 min after lavage. The recovery of meconium in bronchoalveolar lavage (BAL) fluid was quantified. More meconium solids was recovered in the surfactant-lavaged than in the saline-lavaged groups (Surf: 12.4 +/- 3.9% and Surf+dex: 17.5 +/- 3.5% vs. Sal: 4.8 +/- 1.0%; both P meconium solids was obtained by Curosurf/dextran than by Curosurf-only lavage (P meconium clearance and lung functions in surfactant-lavaged rabbits with meconium aspiration.

  8. Fractures and Fanconi syndrome due to prolonged sodium valproate use.

    Science.gov (United States)

    Dhillon, N; Högler, W

    2011-06-01

    Sodium valproate (VPA) is commonly used to treat epilepsy in children. Renal dysfunction is a rare side eff ect but can present as tubulopathy such as Fanconi syndrome. We report on an 8-year-old disabled girl with myoclonic epilepsy who was referred for investigation of recurrent low impact fractures of the distal femur which were initially thought to be caused by her severe immobility. However, she was subsequently found to have hypophosphataemia secondary to Fanconi syndrome due to prolonged VPA use. After VPA withdrawal renal function and serum phosphate levels normalised and X-rays improved dramatically. The possibility of drug-induced osteoporosis and fractures should always be considered in disabled children, even in the presence of severe immobility.

  9. The effects of pentoxifylline on lung inflammation in a rat model of meconium aspiration syndrome.

    Science.gov (United States)

    Turhan, Ali Haydar; Atıcı, Aytuğ; Muşlu, Necati; Polat, Ayşe; Helvacı, İlter

    2012-06-01

    To examine the effects of pentoxifylline (PTX) on regional pulmonary and systemic inflammation after meconium aspiration, we studied 26 anesthetized and ventilated adult rats for 3 hours. Seventeen rats were instilled with human meconium (1.5 mL/kg, 65 mg/mL) intratracheally. After instillation of meconium, PTX (20 mg/kg, i.a.; n = 9) or saline (n = 8) was given to the subjects. Nine rats that were ventilated and not instilled with meconium served as sham group. Meconium instillation resulted in increased bronchoalveolar lavage (BAL) fluid tumor necrosis factor-α (TNF-α; P = 0.004 and P = 0.002, respectively), protein (P = 0.005 and P = 0.001, respectively) levels, and arterial oxygenation index (OI) in PTX and saline groups. PTX treatment prevented the increase of BAL fluid TNF-α, protein concentrations, and OI in the meconium-instilled lungs but had no statistically significant effect. These results indicate that meconium aspiration induces severe inflammation in the lung. PTX treatment affects the TNF-α production in the lungs and it may attenuate meconium-induced derangements.

  10. Thrombus-aspiration through 5 Fr guiding catheter with transradial approach in acute coronary syndromes: feasibility of a mini-invasive strategy.

    Science.gov (United States)

    Roule, Vincent; Dahdouh, Ziad; Wain-Hobson, Julien; Bignon, Mathieu; Malcor, Guillaume; Lognoné, Thérèse; Milliez, Paul; Grollier, Gilles; Sabatier, Rémi

    2012-08-01

    The purpose of this study was to assess the feasibility and safety of thrombus-aspiration through a 5 Fr guiding catheter with transradial approach in acute coronary syndromes. The use of thrombus-aspirating devices improves myocardial reperfusion but requires at least a 6 Fr guiding catheter. Transradial coronary interventions using a 5 Fr guiding catheter are attractive to reduce bleeding complications. We retrospectively selected patients presenting acute coronary syndromes with angiographically visible thrombus who underwent thrombus-aspiration through radial access using a 4 Fr multipurpose catheter in a 5 Fr guiding catheter. We described clinical and angiographic characteristics of the cohort, and the procedure's technique, success and complications. Among the 34 included patients, 29 presented ST-segment elevation myocardial infarction. Complete resolution of the ST-segment elevation was effective in 93% of these patients. TIMI flow grade after thrombus-aspiration was significantly improved compared to baseline TIMI flow grade or after passage of the guidewire (P guiding catheter with transradial approach seems to be safe and effective in selected patients with acute coronary syndrome. This mini-invasive approach brought the advantages of the transradial access in 5 Fr but also its limitations. ©2012, Wiley Periodicals, Inc.

  11. Cushing's syndrome due to interaction between inhaled corticosteroids and itraconazole.

    Science.gov (United States)

    Bolland, Mark J; Bagg, Warwick; Thomas, Mark G; Lucas, Jennifer A; Ticehurst, Rob; Black, Peter N

    2004-01-01

    To report a case of an interaction between inhaled corticosteroids and itraconazole causing iatrogenic Cushing's syndrome and provide a review of the relevant literature. A 70-year-old white woman on long-term treatment with high-dose inhaled corticosteroids for asthma was diagnosed as having Scedosporium apiospermum infection of the skin and subcutaneous tissues. As a result, she was treated with itraconazole for 2 months. She subsequently developed Cushing's syndrome due to a probable cytochrome P450-mediated interaction between itraconazole and budesonide. She also had secondary adrenal insufficiency requiring prolonged treatment with replacement hydrocortisone. Budesonide is a potent glucocorticoid that is metabolized in the liver by the CYP3A4 isoenzyme to inactive metabolites. Itraconazole is a potent cytochrome P450 inhibitor. It can inhibit the metabolism of oral or inhaled corticosteroids, producing cortisol excess leading to Cushing's syndrome and adrenal insufficiency. An assessment of causality indicated a possible adverse interaction between itraconazole and budesonide. The combination of itraconazole and inhaled corticosteroids is increasingly being used to treat conditions such as allergic bronchopulmonary aspergillosis. Clinicians need to be aware of the potential for an interaction between such a combination.

  12. Syndrome of Inappropriate Antidiuretic Hormone Secretion in a Mini-Breed Puppy Associated With Aspiration Pneumonia.

    Science.gov (United States)

    Martínez, Rocío; Torrente, Carlos

    2017-12-01

    A 3-month-old intact male Prague ratter was presented to the emergency service for evaluation of progressive lethargy, weakness, coughing and labour breathing after an episode of resistance to oral deworming. The patient exhibited depression, increased respiratory effort and cyanosis at initial presentation. Results of first diagnostic work-up (complete blood cell count, biochemistry panel and thoracic x-rays) were all consistent with aspiration pneumonia. The puppy was initially treated with balanced isotonic crystalloids, broad spectrum antibiotics, nebulization with thoracic coupage and was transferred to an infant incubator with a sustained FiO2 of 40-50%. Twenty-four hours after ICU admission the patient's condition suffered a worsening and the dog was orthopneic, severely depressed with episodes of intermittent dysphoria and seizuring. New thoracic radiographs and several samples of blood and urine were collected to go further in the diagnostic workup revealing severe hyponatremia, severe plasma hypotonicity, high natriuresis and metabolic acidosis with a worsening of the radiological pulmonary pattern. Based on these new clinical findings a diagnosis of SIADH was established. Emergency treatment with hypertonic 3% saline solution and loop diuretics was started like a sodium supplement and to inhibit water resorption in renal tubules, thus reducing the volume overload. The goal of this treatment was to achieve a progressive and controlled increase of plasma sodium concentration and promoting the excretion of positive body water imbalance. The patient's condition improved clinically over the following days, treatment was progressively discontinued and the dog was discharged 7 days after admission. To the author's knowledge this is the first report of a puppy younger than 12 weeks with respiratory distress developing SIADH associated to aspiration pneumonia. Copyright © 2017 Elsevier Inc. All rights reserved.

  13. Effect of Nasal Continuous Positive Airway Pressure on Infants With Meconium Aspiration Syndrome: A Randomized Clinical Trial.

    Science.gov (United States)

    Pandita, Aakash; Murki, Srinivas; Oleti, Tejo Pratap; Tandur, Baswaraj; Kiran, Sai; Narkhede, Sachin; Prajapati, Amrut

    2018-02-01

    Nasal continuous positive airway pressure (NCPAP) as a primary respiratory therapy in meconium aspiration syndrome (MAS) has not been studied extensively. Nasal continuous positive airway pressure, when applied in newborns with MAS, may resolve atelectasis by sufficiently expanding partially obstructed small airways and stabilizing the collapsing terminal airways to enhance oxygen exchange. To compare NCPAP vs standard care in neonates with moderate to severe respiratory failure due to MAS in reducing the need for invasive ventilation. This multicenter open-label, parallel-group (1:1 ratio) randomized clinical trial was conducted from August 5, 2014, to May 26, 2016. Data were collected from 3 tertiary care neonatal intensive care units. All infants admitted with respiratory distress, defined as Downe score greater than 4 and peripheral capillary oxygen saturation less than 90%, were assessed for study eligibility if the chest radiograph was suggestive of MAS and they met the other inclusion criteria: gestation longer than 35 weeks, a birth weight greater than 2000 g, and born through meconium-stained amniotic fluid. Infants were randomly assigned to either NCPAP or standard care (5-10 L/min hood oxygen). The primary outcome was the need for mechanical ventilation in the first 7 days of life. After excluding 14 infants, 67 infants were randomized to bubble NCPAP and 68 infants to standard care. Baseline characteristics were similar between the 2 groups. Infants randomized to the bubble NCPAP group needed mechanical ventilation less frequently in the first 7 days of life compared with standard care (2 [3.0%] vs 17 [25.0%]); odds ratio, 0.09; 95% CI, 0.02-0.43; P = .002). The need for surfactant (3 [4.5%] vs 11 [16.2%]; odds ratio, 0.24; 95% CI, 0.05-0.87) and culture-positive sepsis (4 [6.0%] vs 13 [19.0%]; odds ratio, 0.28; 95% CI, 0.09-0.93) were higher in the standard care group. There was an increased duration of oxygen therapy (median [interquartile range

  14. 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

    : An L/S algorithm was developed based on 89 aspirates. Subsequently, gastric aspirates were sampled in 136 infants of 24-31 weeks of gestation and 61 (45%) developed RDS. The cut-off value of L/S was 2.2, sensitivity was 92%, and specificity was 73%. In 59 cases, the oropharyngeal secretions had less...... aspirates were analysed with mid-infrared spectroscopy. Subsequently, L/S was measured in gastric aspirates and oropharyngeal secretions from another group of premature infants using spectroscopy and the results were compared with RDS development. The 10-minute analysis required 10 μL of aspirate. RESULTS...... valid L/S than gastric aspirate results. CONCLUSION: Our rapid test for lung maturity, based on spectroscopy of gastric aspirate, predicted RDS with high sensitivity....

  15. Strategic Aspirations

    DEFF Research Database (Denmark)

    Christensen, Lars Thøger; Morsing, Mette; Thyssen, Ole

    2016-01-01

    aspirations, in other words, have exploratory and inspirational potential—two features that are highly essential in complex areas such as sustainability and CSR. This entry takes a communicative focus on strategic aspirations, highlighting the value of aspirational talk, understood as ideals and intentions...

  16. Cavernous sinus syndrome due to osteochondromatosis in a cat.

    Science.gov (United States)

    Perazzi, Anna; Bernardini, Marco; Mandara, Maria T; De Benedictis, Giulia M; De Strobel, Francesca; Zotti, Alessandro

    2013-12-01

    A 1-year-old sexually intact male Korat cat was referred for ophthalmological consultation due to anisocoria. Mydriasis with external ophthalmoplegia and absence of pupillary light responses in the right eye and nasofacial hypalgesia were seen. Cavernous sinus syndrome (CSS) was suspected. Bilateral deformities of the jaw and phalangeal bones, severe spinal pain and abnormal conformation of the lumbar spine were also present. Radiographic examination revealed several mineralised masses in the appendicular and axial skeleton, indicative of multiple cartilaginous exostoses. For further investigation of the CSS-related neurological deficits, the cat underwent computed tomography (CT) examination of the skull. CT images revealed a non-vascularised, calcified, amorphous mass originating from the right lateral skull base and superimposing on the sella turcica. Based on the severity of diffuse lesions and owing to the clinical signs of extreme pain, the cat was euthanased. A diffuse skeletal and intracranial osteochondromatosis was diagnosed histologically.

  17. Effects of Natural versus Synthetic Surfactant with SP-B and SP-C Analogs in a Porcine Model of Meconium Aspiration Syndrome.

    Science.gov (United States)

    Salvesen, Bodil; Curstedt, Tore; Mollnes, Tom Eirik; Saugstad, Ola Didrik

    2014-01-01

    Meconium displaces surfactant from the alveolar surface and inhibits its function. The development of active synthetic surfactants is complicated, especially to synthesize the hydrophobic surfactant proteins SP-B and SP-C. A synthetic surfactant, CHF5633 containing SP-B and SP-C analogs, has been designed to act similarly to the natural surfactant poractant alfa. To test the resistance to meconium inactivation of CHF5633 compared to poractant alfa. Secondary outcome measurements were respiratory and inflammatory parameters. Twenty-six newborn pigs, bodyweight 1.4-2.0 kg were randomized to receive either poractant alfa or CHF5633. After anesthesia, surgery and final stabilization, meconium was instilled endotracheally followed by surfactant. Bronchial lavage fluid was obtained before intervention and every second hour. Respiratory parameters were registered and blood samples drawn before intervention and every hour. Surfactant was inactivated in both groups 6 h after meconium instillation, but CHF5633 was more resistant than poractant alfa in terms of lipid peroxidation. Respiratory parameters were similar in both groups. Inflammatory and hemostatic parameters differed between groups, suggesting that the surfactants may play different roles in the meconium-induced inflammatory process. Due to the differential effects and complex pattern observed, the data do not indicate that one of the surfactants was superior with respect to inflammatory and hemostatic responses. This study indicates that CHF5633 is as efficient as poractant alfa in experimental meconium aspiration syndrome. © 2013 S. Karger AG, Basel.

  18. [Application of humidified high-flow nasal cannula in neonates with meconium aspiration syndrome and pulmonary hypertension after extubation].

    Science.gov (United States)

    Gao, Xiao-Yan; Feng, Lin; Qiu, Yu-Fen; Pan, Xin-Nian

    2017-04-01

    To investigate the clinical value of humidified high-flow nasal cannula (HHFNC) as a respiratory support after extubation by comparing it with nasal continuous positive airway pressure (NCPAP) in neonates with meconium aspiration syndrome (MAS) and persistent pulmonary hypertension of the newborn (PPHN). A total of 78 neonates with MAS and PPHN were randomly administered with HHFNC or NCPAP immediately after extubation. The following indices were compared between the two groups: blood gas parameters, duration of noninvasive ventilation, rate of extubation failure, and incidence of complications, such as nasal damage, abdominal distension, and intraventricular hemorrhage. There were no significant differences in the rate of extubation failure, PaO 2 , PCO 2 , and PaO 2 /FiO 2 ratio at one hour after NCPAP or HHFNC, duration of noninvasive ventilation, time to full enteral feeding, length of hospital stay, and incidence of intraventricular hemorrhage between the two groups (P>0.05). The HHFNC group had significantly lower incidence of nasal damage (5.0% vs 31.6%; P<0.05) and incidence of abdominal distension (7.5% vs 34.2%; P<0.05) than the NCPAP group. Both NCPAP and HHFNC can be used as the sequential therapy for neonates with MSA and PPHN after extubation, and they both have a definite effect. As a new strategy of respiratory support, HHFNC is better tolerated, and has fewer side effects than NCPAP.

  19. Changes in mean arterial blood pressure during sildenafil use in neonates with meconium aspiration syndrome or sepsis.

    Science.gov (United States)

    Limjoco, Jamie; Paquette, Lisa; Ramanathan, Rangasamy; Seri, Istvan; Friedlich, Philippe

    2015-01-01

    The aim of this study was to evaluate changes in mean blood pressure (MBP) in late preterm and term newborns with meconium aspiration syndrome (MAS) or sepsis who, in addition to inhaled nitric oxide (iNO), received enteral sildenafil for treatment of persistent pulmonary hypertension of the newborn. Data on sildenafil dosing, MBP, and vasopressor/inotrope use were collected for 72 hours after initiation of sildenafil. Groups were compared between "low dose" (<3 mg·kg·d) versus "high dose" (≥ 3 mg·kg·d) and "early" (<7 postnatal days) versus "late" (≥ 7 postnatal days) administration of sildenafil. Seventeen patients were identified. Ten and 7 patients received "low-dose" and "high-dose" sildenafil, respectively, and 8 and 9 patients were started on sildenafil "early" and "late," respectively. At the doses used, sildenafil treatment of infants with MAS and sepsis was not associated with changes in MBP. In addition, vasopressor/inotropic support was weaned in all groups. During the first 72 hours of enteral sildenafil administration in neonates with pulmonary hypertension of the newborn secondary to MAS or sepsis, no significant decrease in MBP or increase in vasopressor/inotrope requirement occurred.

  20. Budesonide added to modified porcine surfactant Curosurf may additionally improve the lung functions in meconium aspiration syndrome.

    Science.gov (United States)

    Mikolka, P; Mokrá, D; Kopincová, J; Tomčíková-Mikušiaková, L; Calkovská, A

    2013-01-01

    Severe meconium aspiration syndrome (MAS) in newborns is often treated by exogenous surfactant. Because its efficacy is reduced by meconium-induced inflammation, glucocorticoid budesonide was added into surfactant preparation Curosurf to enhance efficacy of the surfactant therapy in experimental model of MAS. Oxygen-ventilated rabbits were intratracheally given meconium (25 mg/ml, 4 ml/kg) to induce respiratory failure. Thirty minutes later, animals were treated by intratracheal budesonide (0.25 mg/kg) or surfactant lung lavage (10 ml/kg, 5 mg phospholipids/ml) repeated twice, followed by undiluted Curosurf (100 mg phospholipids/kg) or by the above mentioned surfactant treatment with the last surfactant dose fortified with budesonide (0.25 mg/kg) or were untreated. Animals were ventilated for additional 5 hours and respiratory parameters were measured regularly. After sacrificing animals, wet-dry lung weight ratio was evaluated and plasma levels of interleukins (IL)-1beta, -6, -8, and TNF-alpha were measured by ELISA method. Efficacy of the given therapies to enhance lung functions and to diminish lung edema formation and inflammation increased from budesonide-only and surfactant-only therapy to surfactant+budesonide therapy. Combined therapy improved gas exchange from 30 min of administration, and showed a longer-lasting effect than surfactant-only therapy. In conclusions, budesonide additionally improved the effects of exogenous surfactant in experimental MAS.

  1. A Population-Based Study of Meconium Aspiration Syndrome in Neonates Born between 37 and 43 Weeks of Gestation

    Science.gov (United States)

    Fischer, C.; Rybakowski, C.; Ferdynus, C.; Sagot, P.; Gouyon, J. B.

    2012-01-01

    The epidemiology of meconium aspiration syndrome (MAS) in term neonates is described in a population-based retrospective study of data recorded for all births from 2000 to 2007 in a French region (Burgundy). Of the 132 884 eligible term newborns, the rate of meconium-stained amniotic fluid (MSAF) was 7.93%. The prevalence of severe MAS was 0.067% in the overall population. MAS rate was 0.11% at 37-38 weeks of gestation (WG), 0.20% at 39–41 WG, and 0.49% at 42-43 WG. Factors independently associated with severe MAS were identified by a case-control study, that is, thick meconium amniotic fluid, fetal tachycardia, Apgar score ≤3 at 1 minute, and birth in a level III facility. Our results confirm the high prevalence of MSAF after 37 WG but also show the low frequency of severe MAS in a period corresponding to the new international recommendations on the management of birth with MSAF. PMID:22187569

  2. Lung inflammatory and oxidative alterations after exogenous surfactant therapy fortified with budesonide in rabbit model of meconium aspiration syndrome.

    Science.gov (United States)

    Mikolka, P; Kopincová, J; Košútová, P; Čierny, D; Čalkovská, A; Mokrá, D

    2016-12-22

    Meconium aspiration syndrome (MAS) triggers inflammatory and oxidative pathways which can inactivate both pulmonary surfactant and therapeutically given exogenous surfactant. Glucocorticoid budesonide added to exogenous surfactant can inhibit inflammation and thereby enhance treatment efficacy. Neonatal meconium (25 mg/ml, 4 ml/kg) was administered intratracheally (i.t.) to rabbits. When the MAS model was prepared, animals were treated with budesonide i.t. (Pulmicort, 0.25 mg/kg, M+B); with surfactant lung lavage (Curosurf®, 10 ml/kg, 5 mg phospholipids/ml, M+S) followed by undiluted Curosurf® i.t. (100 mg phospholipids/kg); with combination of budesonide and surfactant (M+S+B); or were untreated (M); or served as controls with saline i.t. instead of meconium (C). Animals were oxygen-ventilated for additional 5 h. Cell counts in the blood and bronchoalveolar lavage fluid (BAL), lung edema formation (wet/dry weight ratio), oxidative damage of lipids/ proteins and inflammatory expression profiles (IL-2, IL-6, IL-13, TNF-alpha) in the lung homogenate and plasma were determined. Combined surfactant+budesonide therapy was the most effective in reduction of neutrophil counts in BAL, oxidative damage, levels and mRNA expression of cytokines in the lung, and lung edema formation compared to untreated animals. Curosurf fortified with budesonide mitigated lung inflammation and oxidative modifications what indicate the perspectives of this treatment combination for MAS therapy.

  3. Reflex sympathetic dystrophy syndrome due to arteriovenous fistula.

    Science.gov (United States)

    Unek, Ilkay Tugba; Birlik, Merih; Cavdar, Caner; Ersoy, Rifki; Onen, Fatos; Celik, Ali; Camsari, Taner

    2005-10-01

    A patient with end-stage renal disease presented with reflex sympathetic dystrophy syndrome (RSDS) on her left hand 1 month after arteriovenous fistula (AVF) surgery. Magnetic resonance angiography revealed steal syndrome at the AVF level. Bone scintigraphy revealed early-stage RSDS. We considered that arterial insufficiency because of steal phenomenon following AVF surgery and underlying occlusive arterial disease triggered RSDS development.

  4. Subcutaneous Implantable Cardioverter Defibrillator Lead Failure due to Twiddler Syndrome

    NARCIS (Netherlands)

    Kooiman, Kirsten M.; Brouwer, Tom F.; van Halm, Vokko P.; Knops, Reinoud E.

    2015-01-01

    We present a case of Twiddler syndrome in a patient with a subcutaneous implantable cardioverter defibrillator (S-ICD). The patient presented herself to the outpatient clinic with pain in the left chest. Chest x-ray confirmed Twiddler syndrome and ICD read-out revealed lead failure resulting in

  5. Efficiency of high-frequency oscillatory ventilation combined with pulmonary surfactant in the treatment of neonatal meconium aspiration syndrome.

    Science.gov (United States)

    Chen, Dong-Mei; Wu, Lian-Qiang; Wang, Rui-Quan

    2015-01-01

    The aim of this study was to investigate the clinical efficiency of the use high-frequency oscillatory ventilation (HFOV) combined with pulmonary surfactant (PS) for the treatment of neonatal meconium aspiration syndrome (MAS). Clinical data of 53 MAS patients admitted to neonatal intensive care unit (NICU) was collected and the patients were divided into 3 groups according to the different treatment approach: group 1 conventional mechanical ventilation (CMV); group 2 HFOV; group 3 HFOV + PS. By monitoring the changes in oxygenation function indicators such as inhaled oxygen concentration (FiO2), oxygenation index (OI) and arterial oxygen tension/alveolar arterial oxygen tension (a/ApO2) of three groups after 2, 12, 24, 48 h of treatment, the usage of the ventilator, duration of hospitalization, changes in clinical manifestations and outcomes of three groups were analyzed. As compared to group 1, the difference in all the oxygenation function indicators after treatment in group 2 and group 3 was statistically significant at different points in time (P < 0.05). However, the timing and extent of the change in the indicators in group 3 were more significant than in group 2; as compared to group 1, the ventilation time, duration of the oxygen therapy and hospitalization time of group 2 and group 3 were significantly shorter and the difference was statistically significant (P < 0.05). Early use of HFOV combined with PS to treat MAS has significant therapeutic effect, especially for the treatment of severe MAS where it can be used as a safer and more effective rescue measure.

  6. [Clinical efficacy of porcine pulmonary surfactant combined with budesonide suspension intratracheal instillation in the treatment of neonatal meconium aspiration syndrome].

    Science.gov (United States)

    Tan, Xiu-Zhen; Wu, Shi-Guang; Zhang, Jian-Hua; Li, Xiao-Fen; Gao, Ping-Ming; Wang, Yu

    2016-12-01

    To study the clinical efficacy of porcine pulmonary surfactant (PS) combined with budesonide suspension intratracheal instillation in the treatment of neonatal meconium aspiration syndrome (MAS). Seventy neonates with MAS were enrolled for a prospective study. The neonates were randomly assigned to PS alone treatment group and PS+budesonide treatment group (n=35 each). The PS alone treatment group was given PS (100 mg/kg) by intratracheal instillation. The treatment group was given budesonide suspension (0.25 mg/kg) combined with PS (100 mg/kg). The rate of repeated use of PS in the PS+ budesonide group was significantly lower than that in the PS alone group 12 hours after treatment (p<0.05). The improvement of PaO 2 /FiO 2 , TcSaO 2 , PaO 2 , and PaCO 2 in the PS+ budesonide group was significantly greater than that in the PS alone group 6, 12, and 24 hours after treatment (p<0.05). The chest X-ray examination showed that the pulmonary inflammation absorption in the PS+ budesonide group was significantly better than that in the PS alone group 48 hours after treatment (p<0.05). The incidence of complications in the PS+budesonide group was significantly lower than that in the PS alone group (p<0.05), and the average hospitalization duration was significantly shorter than that in the PS alone group (p<0.01). PS combined with budesonide suspension intratracheal instillation for the treatment of neonatal MAS is effective and superior to PS alone treatment.

  7. Combined Inhibition of C5 and CD14 Attenuates Systemic Inflammation in a Piglet Model of Meconium Aspiration Syndrome.

    Science.gov (United States)

    Thomas, Anub Mathew; Schjalm, Camilla; Nilsson, Per H; Lindenskov, Paal H H; Rørtveit, Runa; Solberg, Rønnaug; Saugstad, Ola Didrik; Berglund, Magnus M; Strömberg, Patrik; Lau, Corinna; Espevik, Terje; Jansen, Johan Høgset; Castellheim, Albert; Mollnes, Tom Eirik; Barratt-Due, Andreas

    2018-02-27

    Meconium aspiration syndrome (MAS) is a severe lung condition affecting newborns and it can lead to a systemic inflammatory response. We previously documented complement activation and cytokine release in a piglet MAS model. Additionally, we showed ex vivo that meconium-induced inflammation was dependent on complement and Toll-like receptors. To assess the efficacy of the combined inhibition of complement (C5) and CD14 on systemic inflammation induced in a forceful piglet MAS model. Thirty piglets were randomly allocated to a treatment group receiving the C5-inhibitor SOBI002 and anti-CD14 (n = 15) and a nontreated control group (n = 15). MAS was induced by intratracheal meconium instillation, and the piglets were observed for 5 h. Complement, cytokines, and myeloperoxidase (MPO) were measured by ELISA. SOBI002 ablated C5 activity and the formation of the terminal complement complex in vivo. The combined inhibition attenuated the inflammasome cytokines IL-1β and IL-6 by 60 (p = 0.029) and 44% (p = 0.01), respectively, and also MPO activity in the bronchoalveolar fluid by 42% (p = 0.017). Ex vivo experiments in human blood revealed that the combined regimen attenuated meconium-induced MPO release by 64% (p = 0.008), but there was only a negligible effect with single inhibition, indicating a synergic cross-talk between the key molecules C5 and CD14. Combined inhibition of C5 and CD14 attenuates meconium-induced inflammation in vivo and this could become a future therapeutic regimen for MAS. © 2018 The Author(s) Published by S. Karger AG, Basel.

  8. Angelman Syndrome Due to UBE3A Gene Mutation.

    Science.gov (United States)

    Goswami, Jyotindra Narayan; Sahu, Jitendra Kumar; Singhi, Pratibha

    2017-12-18

    A 12-y-old boy presented with developmental delay, autism, epilepsy, limb tremors and behavioral problems which posed a diagnostic challenge. Though his clinical profile and electroencephalogram were suggestive of Angelman syndrome, initial genetic tests were unyielding. Exome sequencing revealed a previously unreported mutation of Ubiquitin Protein Ligase E3A (UBE3A) gene, confirming the diagnosis of Angelman syndrome. The case is aimed to sensitize pediatricians about Angelman syndrome and to highlight the role of sequential investigations in establishing the diagnosis.

  9. Iatrogenic Cushing's Syndrome Due to Topical Ocular Glucocorticoid Treatment.

    Science.gov (United States)

    Fukuhara, Daisuke; Takiura, Toshihiko; Keino, Hiroshi; Okada, Annabelle A; Yan, Kunimasa

    2017-02-01

    Iatrogenic Cushing's syndrome (CS) is a severe adverse effect of systemic glucocorticoid (GC) therapy in children, but is extremely rare in the setting of topical ocular GC therapy. In this article, we report the case of a 9-year-old girl suffering from idiopathic uveitis who developed CS due to topical ocular GC treatment. She was referred to the ophthalmology department with a complaint of painful eyes, at which time she was diagnosed with bilateral iridocyclitis and started on a treatment of betamethasone sodium phosphate eye drops. Six months after the initiation of topical ocular GC treatment, she was referred to our pediatric department with stunted growth, truncal obesity, purple skin striate, buffalo hump, and moon face. Because her serum cortisol and plasma adrenocorticotropic hormone levels were undetectable, she was diagnosed with iatrogenic CS. After the doses of topical ocular GC were reduced, the clinical symptoms of CS were improved. The fact that the amount of topical ocular GC with our patient was apparently less than that of similar previous cases tempted us to perform genetic analysis of her NR3C1 gene. We found that our patient had a single heterozygous nucleotide substitution in the 3' untranslated region of the NR3C1 gene, which may explain why she developed CS. However, additional investigations are required to determine if our findings can be extrapolated to other patients. In conclusion, clinicians should be aware that even extremely low doses of topical ocular steroid therapy can cause iatrogenic CS. Copyright © 2017 by the American Academy of Pediatrics.

  10. Serotonin Syndrome Due to Overdose Intake of SSRI

    OpenAIRE

    Yavuz Orak; Sevgi Akbulut

    2015-01-01

    Serotonin syndrome is a drug side effect resulting from serotonergic hyperactivity. The severity of its symptoms can be mild and overlooked and sometimes it may cause life-threatening serious consequences. This syndrome is caused by the administration of one or more drugs having serotonergic activity. This case is a 25-year-old female patient who attempted suicide by ingesting an overdose of her prescription medications: 60 units of 100-mg Faver (Fluvoxamine), 20 units of 50-mg Setral (Setral...

  11. RRH: envenoming syndrome due to 200 stings from Africanized honeybees

    Directory of Open Access Journals (Sweden)

    Guilherme Almeida Rosa da Silva

    2013-02-01

    Full Text Available Envenoming syndrome from Africanized bee stings is a toxic syndrome caused by the inoculation of large amounts of venom from multiple bee stings, generally more than five hundred. The incidence of severe toxicity from Africanized bee stings is rare but deadly. This report reveals that because of the small volume of distribution, having fewer stings does not exempt a patient from experiencing an unfavorable outcome, particularly in children, elderly people or underweight people.

  12. Placenta accreta: adherent placenta due to Asherman syndrome

    DEFF Research Database (Denmark)

    Engelbrechtsen, Line; Langhoff-Roos, Jens; Kjer, Jens Jørgen

    2015-01-01

    It is important to be aware of the risk of abnormally invasive placenta in patients with a history of Asherman syndrome and uterine scarring. A prenatal diagnosis by ultrasonography is useful when planning of mode of delivery.......It is important to be aware of the risk of abnormally invasive placenta in patients with a history of Asherman syndrome and uterine scarring. A prenatal diagnosis by ultrasonography is useful when planning of mode of delivery....

  13. Hypotension due to Chemotherapy in a Patient with Small Cell Lung Cancer and Lambert-Eaton Myasthenic Syndrome Undergoing Hemodialysis: A First Case Report

    Directory of Open Access Journals (Sweden)

    Taiji Kuwata

    2012-01-01

    Full Text Available We present the first case of small cell lung cancer with Lambert-Eaton myasthenic syndrome during hemodialysis (HD. A 72-year-old male patient receiving HD experienced progressive muscle weakness. He was diagnosed with small cell lung cancer with Lambert-Eaton myasthenic syndrome due to an increased serum level of anti-voltage-gated calcium channel antibody and aspiration cytology on endobronchial ultrasonography for the swelling of a subcarinal lymph node. He received chemotherapy consisting of carboplatin (300 mg/m2 and etoposide (50 mg/m2, to which he had a partial response. However, the second therapy course could not be administered because of the unexpected development of severe hematological adverse events, which also prevented him from undergoing further HD. This case indicates that caution should be taken when using chemotherapy for such patients because of hypotension due to chemotherapy, with which it is impossible to undergo HD.

  14. [Sudden death of a child due to aspiration of the gastric content during an infection with Norwalk-like virus].

    Science.gov (United States)

    Thiele, Karlheinz; Müller, Lutz; Uerlings, Harald; Schmidt, Uwe

    2005-01-01

    The death of a 1.5-year-old child due to bronchoaspiration of the gastric content in the development of a letal infection caused by Norwalk-like virus was reported. The viral gastrointestinal diseases are common but they rarely produce a severe disease. Recent investigations show that Norwall-like virus (Noro-Virus) is the most frequent gastrointestinal pathogen in Central Europe. The etiological diagnosis of the gastrointestinal disease, starting from autopsy material, was only possible by polymerase chain reaction. The introduction of this technique allowed to reduce the number of cases of gastrointestinal diseases without etiological diagnosis and, at the same time, to increase the demonstration of the occurrence of infection caused by Norwalk-like virus.

  15. Aspiration pneumonia: a review of modern trends.

    Science.gov (United States)

    DiBardino, David M; Wunderink, Richard G

    2015-02-01

    The purpose was to describe aspiration pneumonia in the context of other lung infections and aspiration syndromes and to distinguish between the main scenarios commonly implied when the terms aspiration or aspiration pneumonia are used. Finally, we aim to summarize current evidence surrounding the diagnosis, microbiology, treatment, risks, and prevention of aspiration pneumonia. Medline was searched from inception to November 2013. All descriptive or experimental studies that added to the understanding of aspiration pneumonia were reviewed. All studies that provided insight into the clinical aspiration syndromes, historical context, diagnosis, microbiology, risk factors, prevention, and treatment were summarized within the text. Despite the original teaching, aspiration pneumonia is difficult to distinguish from other pneumonia syndromes. The microbiology of pneumonia after a macroaspiration has changed over the last 60 years from an anaerobic infection to one of aerobic and nosocomial bacteria. Successful antibiotic therapy has been achieved with several antibiotics. Various risks for aspiration have been described leading to several proposed preventative measures. Aspiration pneumonia is a disease with a distinct pathophysiology. In the modern era, aspiration pneumonia is rarely solely an anaerobic infection. Antibiotic treatment is largely dependent on the clinical scenario. Several measures may help prevent aspiration pneumonia. Copyright © 2014 Elsevier Inc. All rights reserved.

  16. Horner Syndrome Due to Intrathoracic Multinodular Goiter (Case Report

    Directory of Open Access Journals (Sweden)

    Selda Korkmaz

    2013-09-01

    Full Text Available Horner syndrome is characterized by miosis, ptosis, facial anhydrosis and enophtalmus and is caused by a lesion along the oculosymphathetic pathway from the hypothalamus to the eye. The long course of the oculosympathetic pathway predisposes it to a wide variety of pathologic processes, ranging from harmless vascular headaches to life-threatening conditions such as carotid artery dissection or malignancy. Thyroid neoplasms, benign or malign, are among unusual causes of Horner syndrome and represent 1.3 - 7.8% of cases. Especially, early diagnosis of benign thyroid neoplasms such as multinodular goiter has particular importance because of its reversiblity with appropriate treatment. We report a case of Horner syndrome which has been developed as a complication of intrathoracic multinodular goiter

  17. [Endoscopic treatment of bleeding due to Mallory-Weiss syndrome].

    Science.gov (United States)

    Pantsyrev, Iu M; Fedorov, E D; Timofeev, M E; Mikhalev, A I

    2003-01-01

    Experience in diagnosis and treatment of 276 patients with Mallory--Weiss syndrome (MWS) is presented. Definition of the syndrome and brief historical review (from first description of disruption of esophageal-gastric connection to first report of successful hemostasis through endoscope) are given. Particular attention is devoted to etiology and pathogenesis of this syndrome. Clinical material and picture of disease, results of endoscopic diagnosis are analyzed, efficacy of endoscopic hemostasis and prophylaxis of hemorrhage is demonstrated. Indications to various methods of endoscopic hemostasis are regarded, results of treatment and lethal outcomes are analyzed. It is demonstrated that endoscopic procedures are the method of choice in the treatment of this disease reducing emergency surgery rate to 0.4%.

  18. Serotonin Syndrome Due to Overdose Intake of SSRI

    Directory of Open Access Journals (Sweden)

    Yavuz Orak

    2015-08-01

    Full Text Available Serotonin syndrome is a drug side effect resulting from serotonergic hyperactivity. The severity of its symptoms can be mild and overlooked and sometimes it may cause life-threatening serious consequences. This syndrome is caused by the administration of one or more drugs having serotonergic activity. This case is a 25-year-old female patient who attempted suicide by ingesting an overdose of her prescription medications: 60 units of 100-mg Faver (Fluvoxamine, 20 units of 50-mg Setral (Setraline, and 10 units of 20-mg Paxil (Paroxetine.

  19. Surfactant therapy and antibiotics in neonates with meconium aspiration syndrome: a systematic review and meta-analysis.

    Science.gov (United States)

    Natarajan, C K; Sankar, M J; Jain, K; Agarwal, R; Paul, V K

    2016-05-01

    Meconium aspiration syndrome (MAS), a common cause of respiratory failure in neonates, is associated with high mortality and morbidity. The objectives of this review were to evaluate the effects of administration of (a) surfactant-either as lung lavage (SLL) or bolus surfactant (BS) and (b) antibiotics on mortality and severe morbidities in neonates with MAS. We searched the following databases: MEDLINE via PubMed, Cochrane CENTRAL, WHOLIS and CABI using sensitive search strategies. We included eight studies on use of surfactant and three studies on use of antibiotics. Neither SLL nor BS reduced the risk of mortality in neonates with MAS (relative risk (RR) 0.38, 95% confidence interval (CI) 0.09 to 1.57; and RR 0.80, 95% CI 0.39 to 1.66, respectively). Both SLL and BS reduced the duration of hospital stay (mean difference -2.0, 95% CI -3.66 to -0.34; and RR -4.68, 95% CI -7.11 to -2.24 days, respectively) and duration of mechanical ventilation (mean difference -1.31, 95% CI -1.91 to -0.72; and mean difference 5.4, 95% CI -9.76 to -1.03 days). Neonates who received BS needed extracorporeal membrane oxygenation (ECMO) less often than the controls (RR 0.64, 95% CI 0.46 to 0.91). Use of antibiotics for MAS did not result in significant reduction in the risk of mortality, sepsis or duration of hospital stay. Surfactant administration either as SLL or BS for MAS was found to reduce the duration of mechanical ventilation and hospital stay; BS also reduced the need for ECMO. Administration of antibiotics did not show any significant clinical benefits in neonates with MAS and no evidence of sepsis. Given the limited number of studies and small number of neonates enrolled, there is an urgent need to generate more evidence on the efficacy and cost-effectiveness of these two treatment modalities before recommending them in routine clinical practice.

  20. Junctional chiasmatic syndrome due to large anterior communicating artery aneurysm

    OpenAIRE

    Maruthi Kesani; Pavan Kumar Pelluru; Suchanda Bhattacharjee; Rajesh Alugolu; A K Purohit

    2017-01-01

    Even though aneurysm involving the anterior communicating artery (A Com A) was common in clinical practice, producing compressive symptoms such as visual loss was rare. We report a case, in which patient had gradually progressive visual loss with features of the junctional chiasmatic syndrome, imaging revealed partially thrombosed large A Com A aneurysm. Intraoperatively, aneurysm was found compressing the optic chiasm and right optic nerve, following clipping and decompression of the optic n...

  1. Outbreak of exogenous Cushing's syndrome due to unlicensed medications.

    Science.gov (United States)

    Azizi, Fereidoun; Jahed, Adel; Hedayati, Mehdi; Lankarani, Mahnaz; Bejestani, Hadi Shahrad; Esfahanian, Fatemeh; Beyraghi, Nargues; Noroozi, Alireza; Kobarfard, Farzad

    2008-12-01

    Despite the widespread medical use of glucocorticoids, reports of factitious administration of these hormones have been uncommon. We herein report an outbreak of Cushing's syndrome in Tehran among the addicts using Tamgesic (a brand of Buprenorphine) to help them through the narcotic withdrawal stage, without knowledge of the glucocorticoid content of the black-market drug. Case histories of 19 patients with a final diagnosis of iatrogenic Cushing's syndrome were reviewed. Liquid chromatography/mass spectrometry (LC-Mass) method was used to evaluate glucocorticoid existence in the brand. High performance liquid chromatography was used to determine plasma dexamethasone level. No buprenorphine was present in the vials. Each Tamgesic vial contained 0.4 mg of Dexamethasone disodium phosphate; Heroin was also found in them. The duration of injection abuse and the total dexamethasone intake was 4.5 (1-18) months and 2.6 (0.8-8) mg/day, respectively. Median plasma dexamethasone concentration was 5.8 nmol/l, with a range of 5-8.7. Physical findings of the cases were not different from those of the classic endogenous Cushing's syndrome but their serum cortisol and urinary free cortisol were suppressed. Severe life-threatening complications were demonstrated in five cases. Surreptitious use of steroids resulting in Cushing's syndrome may be more common in opium addicts; a high degree of suspicion is needed to uncover this disorder. Whenever facing a cushingoid appearance in addicts, the possibility of using black market drugs with corticosteroid contents should be kept in mind.

  2. West syndrome due to vitamin B12 deficiency.

    Science.gov (United States)

    Serin, Hepsen Mine; Kara, Aslıhan Oruçoğlu; Oğuz, Baran

    2015-12-01

    Vitamin B12 is one of the essential vitamins affecting various systems of the body. Vitamin B12 deficiency in infants often produces haematological and neurological deficits including macrocyticanaemia, neurodevelopmental delay or regression, irritability, weakness, hypotonia, ataxia, apathy, tremor andseizures. In this article, we report the case of a six-month-old male patient diagnosed with West syndrome associated with vitamin B12 deficiency. Although the patient had no evidence of macrocytic anemia in complete blood count, we measured the level of vitamin B12 because the patient had hypotonicity and found it to be low. No other problem was found in the other investigations directed to the etiology of West syndrome. He was being exclusively breast-fed and vitamin B12 deficiency was related with nutritional inadequacy of his mother. Vitamin B12 deficiency should be considered in the differential diagnosis of patients presenting with different neurological findings. In addition, vitamin B12 deficiency should be considered as a rare cause in West syndrome which has a heterogeneous etiology.

  3. Effects on Lung Function of Small-Volume Conventional Ventilation and High-Frequency Oscillatory Ventilation in a Model of Meconium Aspiration Syndrome.

    Science.gov (United States)

    Mikusiakova, L Tomcikova; Pistekova, H; Kosutova, P; Mikolka, P; Calkovska, A; Mokra, D

    2015-01-01

    For treatment of severe neonatal meconium aspiration syndrome (MAS), lung-protective mechanical ventilation is essential. This study compared short-term effects of small-volume conventional mechanical ventilation and high-frequency oscillatory ventilation on lung function in experimentally-induced MAS. In conventionally-ventilated rabbits, MAS was induced by intratracheal instillation of meconium suspension (4 ml/kg, 25 mg/ml). Then, animals were ventilated conventionally with small-volume (f-50/min; VT-6 ml/kg) or with high frequency ventilation (f-10/s) for 4 h, with the evaluation of blood gases, ventilatory pressures, and pulmonary shunts. After sacrifice, left lung was saline-lavaged and cells in bronchoalveolar lavage fluid (BALF) were determined. Right lung was used for the estimation of lung edema formation (wet/dry weight ratio). Thiobarbituric acid-reactive substances (TBARS), oxidative damage markers, were detected in lung tissue and plasma. Meconium instillation worsened gas exchange, and induced inflammation and lung edema. Within 4 h of ventilation, high frequency ventilation improved arterial pH and CO2 elimination compared with conventional ventilation. However, no other significant differences in oxygenation, ventilatory pressures, shunts, BALF cell counts, TBARS concentrations, or edema formation were observed between the two kinds of ventilation. We conclude that high frequency ventilation has only a slight advantage over small-volume conventional ventilation in the model of meconium aspiration syndrome in that it improves CO2 elimination.

  4. Hypercalcemic encephalopathy due to milk alkali syndrome and injection teriparatide

    Directory of Open Access Journals (Sweden)

    Sandeep Kharb

    2012-01-01

    Full Text Available An 82-year-old male, a known case of severe osteoporosis with vertebral fracture and prostatic carcinoma, was treated with gonadotropin releasing hormone analogue, calcium carbonate, cholecalciferol sachet and injection teriparatide. His diet consisted of milk and curd. He developed altered behavior and generalized weakness, and on investigation, hypercalcemia, hypokalemia, and metabolic alkalosis with low parathyroid hormone levels were detected. Injection teriparatide was stopped and he was managed with forced saline diuresis and injection zoledronic acid. He was diagnosed as a case of milk alkali syndrome in whom teriparatide and prolonged immobilization played a permissive role in the development of hypercalcemic encephalopathy.

  5. Facial paralysis due to Ramsay Hunt syndrome - A rare condition

    Directory of Open Access Journals (Sweden)

    Aline Lariessy Campos Paiva

    Full Text Available Summary Ramsay Hunt syndrome (or herpes zoster oticus is a rare complication of herpes zoster in which reactivation of latent varicella zoster virus infection in the geniculate ganglion occurs. Usually, there are auricular vesicles and symptoms and signs such otalgia and peripheral facial paralysis. In addition, rarely, a rash around the mouth can be seen. Immunodeficient patients are more susceptible to this condition. Diagnosis is essentially based on symptoms. We report the case of a diabetic female patient who sought the emergency department with a complaint of this rare entity.

  6. Junctional chiasmatic syndrome due to large anterior communicating artery aneurysm

    Directory of Open Access Journals (Sweden)

    Maruthi Kesani

    2017-01-01

    Full Text Available Even though aneurysm involving the anterior communicating artery (A Com A was common in clinical practice, producing compressive symptoms such as visual loss was rare. We report a case, in which patient had gradually progressive visual loss with features of the junctional chiasmatic syndrome, imaging revealed partially thrombosed large A Com A aneurysm. Intraoperatively, aneurysm was found compressing the optic chiasm and right optic nerve, following clipping and decompression of the optic nerve and chiasm there was gradually improvement in the vision over 2 weeks postoperatively.

  7. A case of orbital apex syndrome due to Pseudomonas aeruginosa infection

    OpenAIRE

    Takeshi Kusunoki; Kaori Kase; Katsuhisa Ikeda

    2011-01-01

    Orbital apex syndrome is commonly been thought to have a poor prognosis. Many cases of this syndrome have been reported to be caused by paranasal sinus mycosis. We encountered a very rare case (60-year-old woman) of sinusitis with orbital apex syndrome due to Pseudomonas aeruginosa infection. She had received insulin and dialysis for diabtes and diabetic nephropathy, moreover anticoagulants after heart by-pass surgery. She underwent endoscopic sinus operation and was treated with antibiotics,...

  8. Further patient with Angelman syndrome due to paternal disomy of chromosome 15 and a milder phenotype

    Energy Technology Data Exchange (ETDEWEB)

    Gillessen-Kaesbach, G.; Passarge, E.; Horsthemke, B. [Institut fuer Humangenetik, Essen (Germany)

    1995-04-10

    This {open_quotes}Letter to the Editor{close_quotes} decribes a patient with Angelman syndrome due to paternal uniparental disomy of chromosome 15 and a milder phenotype compared to Angelman syndrome patients with a 15q deletion. 10 refs., 1 fig.

  9. Expanded motor and psychiatric phenotype in autosomal dominant Segawa syndrome due to GTP cyclohydrolase deficiency.

    NARCIS (Netherlands)

    Hove, J.L. van; Steyaert, J.; Matthijs, G.; Legius, E.; Theys, P.; Wevers, R.A.; Romstad, A.; Moller, L.B.; Hedrich, K.; Goriounov, D.; Blau, N.; Klein, C.; Casaer, P.

    2006-01-01

    BACKGROUND: Segawa syndrome due to GTP cyclohydrolase deficiency is an autosomal dominant disorder with variable expression, that is clinically characterised by l-dopa responsive, diurnally fluctuating dystonia and parkinsonian symptoms. OBJECTIVE: To delineate the neurological and psychiatric

  10. Pulmonary hypertension due to acute respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    S.A. Ñamendys-Silva

    2014-10-01

    Full Text Available Our aims were to describe the prevalence of pulmonary hypertension in patients with acute respiratory distress syndrome (ARDS, to characterize their hemodynamic cardiopulmonary profiles, and to correlate these parameters with outcome. All consecutive patients over 16 years of age who were in the intensive care unit with a diagnosis of ARDS and an in situ pulmonary artery catheter for hemodynamic monitoring were studied. Pulmonary hypertension was diagnosed when the mean pulmonary artery pressure was >25 mmHg at rest with a pulmonary artery occlusion pressure or left atrial pressure <15 mmHg. During the study period, 30 of 402 critically ill patients (7.46% who were admitted to the ICU fulfilled the criteria for ARDS. Of the 30 patients with ARDS, 14 met the criteria for pulmonary hypertension, a prevalence of 46.6% (95% CI; 28-66%. The most common cause of ARDS was pneumonia (56.3%. The overall mortality was 36.6% and was similar in patients with and without pulmonary hypertension. Differences in patients' hemodynamic profiles were influenced by the presence of pulmonary hypertension. The levels of positive end-expiratory pressure and peak pressure were higher in patients with pulmonary hypertension, and the PaCO2 was higher in those who died. The level of airway pressure seemed to influence the onset of pulmonary hypertension. Survival was determined by the severity of organ failure at admission to the intensive care unit.

  11. Hypocalcemia due to 22q11.2 deletion syndrome diagnosed in adulthood

    Directory of Open Access Journals (Sweden)

    Maria Cabrer

    2018-01-01

    Full Text Available Chromosome 22q11.2 deletion syndrome (22q11.2DS is a genetic syndrome that may present with hypocalcemia due to primary hypoparathyroidism (PH at any age. We report a new diagnosis of 22q11.2DS in a 57-year-old man who presented with symptomatic hypocalcemia. It is important to consider genetic causes of hypocalcemia due to PH regardless of age.

  12. The use of single dose of sodium citrate as a prophylaxis against acid aspiration syndrome in obstetric patients undergoing caesarean section.

    Science.gov (United States)

    Lim, S K; Elegbe, E O

    1991-12-01

    The effectiveness of sodium citrate as a prophylaxis against acid aspiration syndrome was studied in 3 groups of obstetric patients. Group I was the control group which consisted of 20 patients in established labour who were not likely to require caesarean section. No antacid had been given to these patients. Group II consisted of 20 patients who underwent elective caesarean section, while Group III consisted of another 20 patients who underwent emergency caesarean section. Group II and III were given 30ml of 0.3M sodium citrate as soon as they arrived in the operation theatre. The gastric content was aspirated after the induction of anaesthesia and at the end of surgery just before extubation. The volume was measured and a sample sent for pH analysis. Sodium citrate was found to increase the gastric pH significantly in both Group II and III patients when compared with Group I patients who underwent emergency caesarean section. We conclude that 30ml of 0.3M sodium citrate is effective in increasing gastric pH though it tends to be associated with an increase in gastric volume.

  13. Secreted phospholipase A2 is increased in meconium-stained amniotic fluid of term gestations: potential implications for the genesis of meconium aspiration syndrome.

    Science.gov (United States)

    Romero, Roberto; Yoon, Bo Hyun; Chaemsaithong, Piya; Cortez, Josef; Park, Chan-Wook; Gonzalez, Rogelio; Behnke, Ernesto; Hassan, Sonia S; Gotsch, Francesca; Yeo, Lami; Chaiworapongsa, Tinnakorn

    2014-07-01

    Meconium-stained amniotic fluid (MSAF) represents the passage of fetal colonic content into the amniotic cavity. Meconium aspiration syndrome (MAS) is a complication that occurs in a subset of infants with MSAF. Secreted phospholipase A2 (sPLA2) is detected in meconium and is implicated in the development of MAS. The purpose of this study was to determine if sPLA2 concentrations are increased in the amniotic fluid of women in spontaneous labor at term with MSAF. This was a cross-sectional study of patients in spontaneous term labor who underwent amniocentesis (n = 101). The patients were divided into two study groups: (1) MSAF (n = 61) and (2) clear fluid (n = 40). The presence of bacteria and endotoxin as well as interleukin-6 (IL-6) and sPLA2 concentrations in the amniotic fluid were determined. Statistical analyses were performed to test for normality and bivariate analysis. The Spearman correlation coefficient was used to study the relationship between sPLA2 and IL-6 concentrations in the amniotic fluid. Patients with MSAF have a higher median sPLA2 concentration (ng/mL) in amniotic fluid than those with clear fluid [1.7 (0.98-2.89) versus 0.3 (0-0.6), p meconium is aspirated before birth.

  14. A new look at the pathogenesis of the meconium aspiration syndrome: a role for fetal pancreatic proteolytic enzymes in epithelial cell detachment.

    Science.gov (United States)

    Ivanov, Vadim A; Gewolb, Ira H; Uhal, Bruce D

    2010-09-01

    We hypothesized that fetal pancreatic digestive enzymes play a role in the lung damage after meconium aspiration. We studied the effect of meconium on the A549 alveolar epithelial cell line. The exposure of the cells to 0.5 to 5% meconium resulted in significant disruption of connections between A549 cells and caused dose-dependent cell detachment, without signs of cell death. A protease inhibitor cocktail prevented the A549 cell detachment induced by meconium. After the exposure to 2.5% meconium, a protective effect was quantified by measuring light absorbance by gentian violet stain of still attached cells. The protease inhibitor cocktail and chymostatin showed significant protective effects, increasing the number of attached cells by 135 and 123%, respectively (p meconium aspiration syndrome (MAS). We speculate that disruption of intercellular connections and cell detachment from the basement membrane are key events in the pathology associated with MAS. The observed protective effects of protease inhibitors suggest that they may be useful in the treatment and/or prophylaxis of MAS.

  15. Enteropathy-associated intestinal T-cell lymphoma in cavitating mesenteric lymph node syndrome: fine-needle aspiration contributes to the diagnosis.

    Science.gov (United States)

    Schwock, Joerg; Hyjek, Elizabeth M; Torlakovic, Emina E; Geddie, William R

    2015-02-01

    Cavitating mesenteric lymph node syndrome (CMLNS) is an infrequently reported manifestation of unrecognized/longstanding celiac disease and may be associated with enteropathy-associated intestinal T-cell lymphoma and hyposplenism. Unrecognized malignancy and life-threatening infections can pose a significant risk to patients in cases of delayed diagnosis. Fine-needle aspiration of the mesenteric lesions may contribute significantly to the correct diagnosis and can expedite patient management. We report on the cytologic characteristics of enteropathy-associated intestinal T-cell lymphoma first detected in a cyst fluid specimen obtained from a patient with cavitating mesenteric lesions. Image-guided fine-needle aspiration resulted in chylous fluid that contained a lymphoid cell population with neoplastic morphology and abnormal immunophenotype. Further work-up led to the diagnosis of enteropathy-associated intestinal T-cell lymphoma with bone marrow involvement. Cytologic assessment of the cyst fluid is an important part of the diagnostic cascade in patients with CMLNS to exclude clinically occult lymphoma. © 2014 Wiley Periodicals, Inc.

  16. A randomised control study of partial liquid ventilation after airway lavage with exogenous surfactant in a meconium aspiration syndrome animal model

    Science.gov (United States)

    Nakamura, T.; Matsuzawa, S.; Sugiura, M.; Tamura, M.

    2000-01-01

    AIMS—To test the hypothesis that lavage with exogenous surfactant before partial liquid ventilation in meconium aspiration syndrome (MAS) would improve debris removal, and therefore the effectiveness of partial liquid ventilation.
METHODS—12 newborn piglets were randomised into 4 groups, partial liquid ventilation or gas ventilation, with and without surfactant lavage. Physiological and blood gas data were compared between groups by analysis of variance.
RESULTS—Arterial oxygen pressure (PaO2) was improved in the group treated with surfactant lavage when compared with the group not receiving surfactant. PaO2 in the group receiving surfactant lavage followed by partial liquid ventilation was further improved when compared with the group treated with surfactant lavage followed by gas ventilation and the group receiving partial liquid ventilation alone.
CONCLUSION—The effectiveness of partial liquid ventilation in MAS might be enhanced by pretreatment with exogenous surfactant bronchial lavage.

 PMID:10685992

  17. ACUTE CORONARY SYNDROME DUE TO CORONARY VASOSPASM – CASE REPORT

    Directory of Open Access Journals (Sweden)

    S. D. Klimovskiy

    2017-01-01

    Full Text Available Spasm of the coronary arteries is a dynamic narrowing of one or more coronary arteries, leading to significant restriction of the lumen. The leading role in coronary  artery spasm diagnostic belongs to coronary  angiography and its accompanying provocative tests. Mechanisms of coronary  artery spasm development remain incompletely clarified and it's considered as a multifactorial disease with its own specific risk factors.  Though pathophysiology is no longer a matter of dispute, further  efforts should be aimed at the practical application of the recommendations. Diagnosis of the coronary  artery spasm  has important practical significance, due to the prevailing benefit of calcium antagonists in the treatment and absence of benefit from percutaneous coronary  interventions in most cases. A clinical case of the patient with multivessel, multifocal coronary  artery spasm is presented.

  18. Gluteal compartment syndrome due to prolonged immobilization after alcohol intoxication: a case report.

    Science.gov (United States)

    Iizuka, Shinichi; Miura, Naoyuki; Fukushima, Tomokazu; Seki, Tomoko; Sugimoto, Katuhiko; Inokuchi, Sadaki

    2011-07-20

    Gluteal compartment syndrome is a relatively rare condition that mostly result from atraumatic causes such as prolonged immobilization due to drug abuse or alcoholic intoxication and incorrect positioning during surgical procedures rather than traumatic causes. Early diagnosis is difficult and sometimes delayed or overlooked because of poor physical signs resulting from altered mental status and inappropriate diagnosis by clinicians. It has been reported that more than half of the cases of gluteal compartment syndrome are associated with crush syndrome and sciatic nerve palsy. Early diagnosis and immediate fasciotomy are necessary to improve the functional prognosis. Here, we report the case of a patient with gluteal compartment syndrome caused by prolonged immobilization after acute alcoholic intoxication. After disease onset, the patient developed complications of crush syndrome and sciatic nerve palsy, but immediate fasciotomy improved his condition.

  19. Superior Vena Cava Syndrome due to Thrombosis: A Rare Paraneoplastic Presentation of Bronchogenic Carcinoma

    Directory of Open Access Journals (Sweden)

    Avradip Santra

    2016-07-01

    Full Text Available Superior vena cava (SVC syndrome is not an uncommon occurrence in patients with malignancy and it is often described as a medical emergency. In majority of the cases, SVC syndrome occurs due to mechanical obstruction of the SVC by extraluminal compression with primary intrathoracic malignancies. However, intraluminal obstruction due to thrombosis can also produce symptoms and signs of SVC syndrome. Clot-related SVC obstruction is mostly associated with indwelling central venous catheter and pacemaker leads, although such thrombosis can occur spontaneously in a background of a hypercoagulable state, e.g., malignancy. Here, an unusual case of sudden onset SVC syndrome has been reported, which on initial radiologic evaluation was found to have a lung nodule without any significant mediastinal mass or adenopathy compressing SVC. Subsequent investigation with Doppler ultrasonography of the neck showed thrombosis in the right internal jugular, right subclavian and right brachiocephalic vein, which was responsible for SVC syndrome. Histopathological evaluation of lung nodule confirmed presence of an adenocarcinoma. Therefore, venous thromboembolism as a paraneoplastic syndrome should be kept in mind while evaluating a case of SVC obstruction in a cancer patient. Management of the underlying disease is of prime importance in such cases and anticoagulation is the mainstay of therapy. Ability to identify paraneoplastic syndrome may have a significant effect on clinical outcome, ranging from early diagnosis to improved quality of life of the patient.

  20. Steven Johnson syndrome due to I.V Ceftriaxone--a case report.

    OpenAIRE

    Narayanan Veena; Mamatha G; Ashok L; Rajashekar N

    2003-01-01

    Steven-Johnson syndrome (SJS) is a rare vesiculobullous disease characterized by an acute cutaneous eruption that ivolves the skin and mucous membranes including those of the oral cavity. A rare case of Steven-Johnson syndrome, an unexpected treatment response, in a 25-year-old female patient due to administration of intravenous Cefriaxone (1 gm), a third generation cephalosporin has been reported and literature reviewed.

  1. Two cases of Cushing's syndrome due to overuse of topical steroid in the diaper area.

    Science.gov (United States)

    Semiz, Serap; Balci, Yasemin Işik; Ergin, Seniz; Candemir, Maşallah; Polat, Aziz

    2008-01-01

    Topical use of corticosteroids may cause immunosuppression and iatrogenic Cushing's syndrome via hypothalamic-pituitary-adrenal axis. We report two cases with iatrogenic Cushing's syndrome with different clinical outcomes due to abuse of same potent topical steroid clobetasol propionate. One of them died because of fatal disseminated cytomegalovirus infection. The other patient recovered completely. Physicians and parents should be informed about the adverse effects of such potent topical corticosteroids and physicians should prescribe less potent agents, especially during infancy.

  2. Concomitant Cushing's syndrome due to adrenal adenoma in a patient with systemic lupus erythematosus.

    Science.gov (United States)

    Shimizu, Masatoshi; Kawata, Masahito; Okada, Toshio; Yuu, Housai; Kurahashi, Toshifumi; Yamanaka, Kunito; Umezu, Keiichi

    2002-11-01

    A 51-year-old woman had been administered 5 mg/day of prednisolone due to systemic lupus erythematosus (SLE). She developed hypertension, hypokalemia and a pathologic pubic fracture during two years before admission. Although iatrogenic Cushing's syndrome was initially suspected, we diagnosed her as concomitant Cushing's syndrome due to a left adrenal tumor. The elevated endogeneous glucocorticoids were evaluated from urinary excretions of 17-hydroxycorticosteroids, which was 2-fold higher than normal and equivalent to 10 mg of prednisolone. After laparoscopic left adrenalectomy, SLE was favorably controlled with 15 mg of prednisolone, the dosage of which was equivalent to the estimated amount of preoperative glucocorticoids.

  3. A case of orbital apex syndrome due to Pseudomonas aeruginosa infection.

    Science.gov (United States)

    Kusunoki, Takeshi; Kase, Kaori; Ikeda, Katsuhisa

    2011-09-28

    Orbital apex syndrome is commonly been thought to have a poor prognosis. Many cases of this syndrome have been reported to be caused by paranasal sinus mycosis. We encountered a very rare case (60-year-old woman) of sinusitis with orbital apex syndrome due to Pseudomonas aeruginosa infection. She had received insulin and dialysis for diabtes and diabetic nephropathy, moreover anticoagulants after heart by-pass surgery. She underwent endoscopic sinus operation and was treated with antibiotics, but her loss of left vision did not improve. Recently, sinusitis cases due to Pseudomonas aeruginosa were reported to be a increasing. Therefore, we should consider the possibility of Pseudomonas aeruginosa as well as mycosis as infections of the sinus, especially inpatients who are immunocompromised body.

  4. A case of orbital apex syndrome due to Pseudomonas aeruginosa infection

    Directory of Open Access Journals (Sweden)

    Takeshi Kusunoki

    2011-11-01

    Full Text Available Orbital apex syndrome is commonly been thought to have a poor prognosis. Many cases of this syndrome have been reported to be caused by paranasal sinus mycosis. We encountered a very rare case (60-year-old woman of sinusitis with orbital apex syndrome due to Pseudomonas aeruginosa infection. She had received insulin and dialysis for diabtes and diabetic nephropathy, moreover anticoagulants after heart by-pass surgery. She underwent endoscopic sinus operation and was treated with antibiotics, but her loss of left vision did not improve. Recently, sinusitis cases due to Pseudomonas aeruginosa were reported to be a increasing. Therefore, we should consider the possibility of Pseudomonas aeruginosa as well as mycosis as infections of the sinus, especially inpatients who are immunocompromised body.

  5. Unusual Cushing’s Syndrome and Hypercalcitoninaemia due to a Small Cell Prostate Carcinoma

    Directory of Open Access Journals (Sweden)

    Antonio Balestrieri

    2016-01-01

    Full Text Available A 75-year-old man was hospitalized because of severe hypokalaemia due to ACTH dependent Cushing’s syndrome. Total body computed tomography (TBCT and 68 Gallium DOTATATE PET/CT localized a voluminous prostate tumour. A subsequent transurethral prostate biopsy documented a small cell carcinoma positive for ACTH and calcitonin and negative for prostatic specific antigen (PSA at immunocytochemical study; serum prostatic specific antigen (PSA was normal. Despite medical treatments, Cushing’s syndrome was not controlled and the patient’s clinical condition progressively worsened. Surgical resection was excluded; the patient underwent a cycle of chemotherapy followed by febrile neutropenia and fatal intestinal perforation. This case report describes a rare case of Cushing’s syndrome and hypercalcitoninaemia due to a small cell carcinoma of the prostate, a rare tumour with very few therapeutic options and negative prognosis.

  6. A Case of Hypocalcaemia Due to Vitamin D Deficiency in ‘Hikikomori’ Syndrome

    Directory of Open Access Journals (Sweden)

    Takahiro Miyakoshi

    2017-09-01

    Full Text Available Objective: To describe hypocalcaemia due to vitamin D deficiency in ‘hikikomori’ syndrome. Materials and methods: A 37-year-old man with ‘hikikomori’ syndrome for a year was admitted with hypocalcaemia (serum ionic calcium 1.17 mmol/l. Serum 1,25(OH2-vitamin D3 determined by liquid chromatography–tandem mass spectrometry was depressed at 12.1 pg/ml (29.0 pmol/l and plasma intact PTH elevated at 324 ng/l. Administration of 1 μg/day 1α(OH-vitamin D3 and 1 g/day calcium lactate for 1 week normalized calcium and PTH, and raised 1,25(OH2-vitamin D3 to low normal levels. Conclusion: This is the first report of hypocalcaemia due to vitamin D deficiency in a patient with ‘hikikomori’ syndrome.

  7. Cushing Syndrome in a 6-Month-Old Infant due to Adrenocortical Tumor

    Directory of Open Access Journals (Sweden)

    Volmar KeithE

    2009-09-01

    Full Text Available Cushing syndrome is rare in infancy and usually due to an adrenocortical tumor (ACT. We report an infant with Cushing syndrome due to adrenocortical carcinoma. The patient presented at six months of age with a three-month history of growth failure, rapid weight gain, acne, and irritability. Physical examination showed obesity, hypertension, and Cushingoid features. Biochemical evaluation showed very high serum cortisol, mildly elevated testosterone, and suppressed ACTH. Abdominal MRI revealed a heterogeneous right adrenal mass extending into the inferior vena cava. Evaluation for metastases was negative. The tumor was removed surgically en bloc. Pathologic examination demonstrated low mitotic rate, but capsular and vascular invasion. She received no adjuvant therapy. Her linear growth has improved and Cushingoid features resolved. Hormonal markers and quarterly PET scans have been negative for recurrence 24 months postoperatively. In conclusion, adrenocortical neoplasms in children are rare, but should be considered in the differential diagnosis of Cushing syndrome.

  8. ''Dropped-head'' syndrome due to isolated myositis of neck extensor muscles: MRI findings

    International Nuclear Information System (INIS)

    Gaeta, Michele; Mazziotti, Silvio; Blandino, Alfredo; Toscano, Antonio; Rodolico, Carmelo; Mazzeo, Anna

    2006-01-01

    MRI findings of a patient with dropped-head syndrome due to focal myositis of the neck extensor muscles are presented. MRI showed oedematous changes and marked enhancement of the neck extensor muscles. After therapy MRI demonstrated disappearance of the abnormal findings. (orig.)

  9. Recombinant 4 syndrome due to an unbalanced pericentric inversion of chromosome 4.

    Science.gov (United States)

    Battaglia, A; Brothman, A R; Carey, J C

    2002-09-15

    An informative patient with a MCA/MR syndrome consisting of developmental delay, prenatal onset growth delay, microcephaly, distinctive face, iris coloboma, and a congenital heart defect was found, on chromosome analysis, to have the following complement: 46,XY,rec(4) dup(4p) inv(4)(p14q35.1) mat. He has a partial 4p trisomy/distal 4q deletion due to an unbalanced pericentric inversion inherited from his mother. Dup (4p) trisomy was originally described by Wilson et al. [1970: Am J Hum Genet 22:679-690] in a similar case with the same chromosome 4 inversion. To date, at least 85 cases of dup (4p) syndrome have been published, mostly due to unbalanced translocations. Recent articles suggest that the phenotype is hard to recognize clinically due to the lack of specificity of findings. In contrast, 4p trisomy due to an unbalanced pericentric inversion of chromosome 4(p14q35), i.e., the recombinant 4 syndrome observed in our patient, appears to be a discrete entity with relatively consistent features. In total there are four other kindreds described in the literature with this inversion, and the phenotype seems recognizable. Thus, we suggest that recombinant 4 syndrome is a discrete entity among 4p trisomy patients. Copyright 2002 Wiley-Liss, Inc.

  10. Anthropometry in Klinefelter syndrome - multifactorial influences due to CAG length, testosterone treatment and possibly intrauterine hypogonadism

    DEFF Research Database (Denmark)

    Chang, Simon; Skakkebæk, Anne; Trolle, Christian

    2015-01-01

    Context: Klinefelter syndrome, 47, XXY (KS), is underdiagnosed partly due to few clinical signs complicating identification of affected individuals. Certain phenotypic traits are common in KS. However, not all aspects of the KS phenotype are well described. Objective: To describe anthropometry...

  11. Maladaptive Behavior Differences in Prader-Willi Syndrome Due to Paternal Deletion versus Maternal Uniparental Disomy.

    Science.gov (United States)

    Dykens, Elisabeth M.; King, Bryan H.; Cassidy, Suzanne B.

    1999-01-01

    This study compared maladaptive behavior in 23 people with Prader-Willi syndrome due to paternal deletion and in 23 age- and gender-matched subjects with maternal uniparental disomy. Controlling for IQs, the deletion cases showed significantly higher maladaptive ratings, more symptom-related distress, and more behavior problems. Findings suggest a…

  12. Intussusception due to Peutz-Jeghers syndrome - a case report and review of the literature

    International Nuclear Information System (INIS)

    Grasso Filho, Luiz Eduardo; Albertotti, Flavio; Carvalho, Claudio Sobral de; Nersessian, Ana Carolina; Docema, Marcos F. Lima; Ogasawara, Aparecida M.; Peng Yong Sheng; Costacurta, Marco Antonio; Albertotti, Cesar Jose; Cerri, Giovanni Guido

    2000-01-01

    The authors report a case of a 28-year-old woman with ileocecocolic intussusception due to Peutz-Jeghers syndrome, an autosomal dominant disorder characterized by hamartomatous polyposis of the gastrointestinal tract and mucocutaneous pigmentation. This condition frequently presents complications such as intestinal obstruction due to invagination or hemorrhage. In this patient, the diagnosis of intussusception was made preoperatively. The excised material revealed three large polyps which were considered to be the cause of the intussusception. (author)

  13. Guillain-Barré Syndrome due to CMV Reactivation after Cardiac Transplantation

    Science.gov (United States)

    Steger, Christina Maria; Antretter, Herwig; Höfer, Daniel

    2012-01-01

    A 40-year-old male patient suffered from end-stage heart failure due to ischemic cardiomyopathy and received orthotopic cardiac transplantation in June 2005. The instantaneous postoperative course was uneventful, but, seven months later, he suffered from paralysis in the lower extremities finally resulting in quadriplegia and was admitted to hospital. After laboratory testings the diagnosis of a Guillain-Barré syndrome due to cytomegalovirus reactivation was confirmed. PMID:24860678

  14. Guillain-Barré Syndrome due to CMV Reactivation after Cardiac Transplantation

    Directory of Open Access Journals (Sweden)

    Christina Maria Steger

    2012-01-01

    Full Text Available A 40-year-old male patient suffered from end-stage heart failure due to ischemic cardiomyopathy and received orthotopiccardiac transplantation in June 2005. The instantaneous postoperative course was uneventful, but, seven months later, he suffered from paralysis in the lower extremities finally resulting in quadriplegia and was admitted to hospital. After laboratory testings the diagnosis of a Guillain-Barré syndrome due to cytomegalovirus reactivation was confirmed.

  15. Acute renal failure likely due to acute nephritic syndrome associated with typhoid fever.

    Science.gov (United States)

    Hayashi, Manabu; Kouzu, Hideshi; Nishihara, Masahiro; Takahashi, Tohru; Furuhashi, Masato; Sakamoto, Ken-ichi; Satoh, Naotoshi; Nishitani, Takahiro; Shikano, Yasukuni

    2005-10-01

    A 45-year-old woman was admitted to our hospital because of high fever and malaise. She also presented abnormal urine findings including macrohematuria and proteinuria with transient renal insufficiency. Salmonella Typhi was isolated from her blood and stool culture, and then she was diagnosed as having typhoid fever. Salmonella enterica serotype typhi infection can be complicated by renal involvement, although rare. In Japan, few cases have been reported about acute nephritic syndrome in typhoid fever. Here, we report a case of endemic acquired typhoid fever associated with acute renal failure probably due to acute nephritic syndrome successfully treated with levofloxacin.

  16. Endoscopic sclerotherapy in upper gastrointestinal bleeding due to the Mallory-Weiss syndrome.

    Science.gov (United States)

    Bataller, R; Llach, J; Salmerón, J M; Elizalde, J I; Mas, A; Piqué, J M; Brullet, E; Terés, J; Bordas, J M; Rodés, J

    1994-12-01

    Therapeutic endoscopic techniques have changed the need for emergency surgery in gastrointestinal bleeding episodes. However, there is only little information about endoscopic therapies in severe gastrointestinal bleeding due to the Mallory-Weiss syndrome. The aim of this clinical study was to assess the usefulness of early endoscopic examination and sclerotherapy for severe or recurrent bleeding due to the Mallory-Weiss syndrome. We studied all 50 cases of gastrointestinal bleeding secondary to the Mallory-Weiss syndrome seen in 2175 consecutive emergency endoscopic examinations performed in a University Hospital over a 3-year period. Endoscopic sclerotherapy (1/10000 adrenaline + 1% polidocanol) was performed in all patients with active bleeding or visible vessel at endoscopic examination. The remaining patients were medically treated. Active bleeding or a visible vessel were found in 13 patients; definitive hemostasis was obtained in all cases with sclerotherapy. The remaining 37 patients were successfully treated by conservative therapy. On admission, the severity of the hemorrhagic episodes was significantly higher in patients treated with sclerotherapy than in those who did not require this procedure. An esophageal perforation, successfully managed by conservative means, was the only complication recorded in the subset of patients undergoing sclerotherapy. Severe bleeding due to Mallory-Weiss syndrome can be successfully treated by sclerotherapy. Early endoscopic examination is an accurate procedure in identifying patients who do not require sclerotherapy.

  17. [Clinical effect of high-frequency oscillatory ventilation combined with pulmonary surfactant in treatment of neonatal severe meconium aspiration syndrome complicated by pulmonary hemorrhage].

    Science.gov (United States)

    Huang, Jing; Lin, Xin-Zhu; Zheng, Zhi

    2016-11-01

    To study the clinical effect and safety of high-frequency oscillatory ventilation (HFOV) combined with pulmonary surfactant (PS) in the treatment of neonatal severe meconium aspiration syndrome (MAS) complicated by neonatal pulmonary hemorrhage (NPH). A total of 48 children with severe MAS complicated by NPH were enrolled, and a retrospective analysis was performed for the clinical effects of HFOV+PS (trial group, 25 children) and HFOV alone (control group, 23 children). The blood gas parameters, oxygenation index (OI), PaO 2 /FiO 2 (P/F) value, duration of pulmonary hemorrhage, ventilation time, length of hospital stay, incidence of complications, and outcome were compared between the two groups. At 6, 12, 24, and 48 hours after treatment, the trial group had significantly better PaO 2 , OI, and P/F value than the control group (Phemorrhage (P0.05). HFOV combined with PS can better improve oxygenation function and shorten the duration of NPH and ventilation time. Meanwhile, it does not increase the incidence of adverse events. Therefore, it is a safe and effective therapy.

  18. Urotensin-II contributes to pulmonary vasoconstriction in a perinatal model of persistent pulmonary hypertension of the newborn secondary to meconium aspiration syndrome.

    Science.gov (United States)

    Simpson, Catherine M; Smolich, Joseph J; Shekerdemian, Lara S; Penny, Daniel J

    2010-02-01

    Meconium aspiration syndrome (MAS) disrupts perinatal decreases in pulmonary vascular resistance (PVR) and is the commonest cause of neonatal pulmonary hypertension. The contribution of the potent vasoactive agent urotensin-II (U-II), in the pathophysiology of this condition, is unknown. In a new perinatal model of MAS, we combined measurement of circulating U-II levels with U-II receptor blockade studies. Nineteen anesthetized lambs were instrumented then randomly allocated to the following groups: 1) control (n = 5), 2) control plus specific U-II receptor blockade with palosuran (n = 5), 3) tracheal instillation of meconium (n = 5), 4) meconium instillation plus palosuran (n = 4). Hemodynamics, PVR, and plasma U-II were measured for 6 h after delivery. After birth in controls, U-II increased (p meconium lambs displayed a greater rise in U-II levels (p < 0.05 versus control) with an increase in PVR (p < 0.005) that was attenuated by U-II receptor blockade (p < 0.001). These findings suggest that U-II normally acts as a pulmonary vasodilator after birth, but in the presence of MAS, it assumes a vasoconstrictor role. U-II receptor blockade also improves pulmonary hemodynamics in this model.

  19. Saline lavage with substitution of bovine surfactant in term neonates with meconium aspiration syndrome (MAS) transferred for extracorporeal membrane oxygenation (ECMO): a pilot study

    Science.gov (United States)

    Möller, Jens C; Kohl, Martina; Reiss, Irwin; Diederich, Wiebke; Nitsche, Esther M; Göpel, Wolfgang; Gortner, Ludwig

    1999-01-01

    Background: Meconium aspiration syndrome (MAS) is still a condition associated with a high mortality, and many patients require extracorporeal membrane oxygenation (ECMO) as rescue therapy. Beneficial effects of surfactant and perflubron lavage have been reported. However, pure surfactant supplementation has not been proven to be beneficial in the most severe forms of MAS. This study was performed to demonstrate an improvement in oxygenation in neonates transferred for ECMO and fulfilling ECMO criteria with a saline lavage and surfactant resupplementation. Methods: Twelve newborns with MAS [gestational age 36–40 weeks, mean birth weight 3200 g, age 4–16 h, oxygenation index (OI) > 40] transferred for ECMO therapy were treated with saline lavage (5–10 cm3/kg body weight, as long as green colored retrieval was observed) and resupplementation with bovine surfactant (Alveofact, Boehringer, Ingelheim, Germany). The OI at admission and 3 h after this procedure was compared using the t-test for paired samples. ECMO was available as rescue therapy at all times. Results: The OI decreased from 49.4 (SD ± 13.3) to 27.4 (SD ± 7.3), P meconium by extensive lavage is feasible as long as 16 h after birth even in infants considered for ECMO therapy; it might reduce the necessity of ECMO. PMID:11056719

  20. Lemierre syndrome from a neck abscess due to methicillin-resistant Staphylococcus aureus.

    Science.gov (United States)

    Abhishek, Agarwal; Sandeep, Singla; Tarun, Pandey

    2013-01-01

    Lemierre syndrome is characterized by acute septic thrombophlebitis of the internal jugular vein (IJV) that develops after an oropharyngeal infection, and can be complicated by septic emboli to lungs and other organs. The most frequent causative agent is Fusobacterium necrophorum, an anaerobic bacillus found in normal oropharyngeal flora. Staphylococcus aureus has emerged as a cause of Lemierre syndrome in the last decade. We report a case of a 24-year-old man who developed septic IJV thrombosis and necrotizing pneumonia due to S. aureus from an infected hematoma in the right sternocleidomastoid muscle. Antibiotics are the mainstay of therapy with few cases needing anticoagulation. A good outcome is dependent upon an awareness of the condition, a high index of suspicion, and prompt initiation of antibiotic therapy. Recognition of S. aureus as a cause of Lemierre syndrome can guide the choice of initial antibiotics to cover this virulent pathogen. Copyright © 2013 Elsevier Editora Ltda. All rights reserved.

  1. Fatal respiratory distress syndrome due to coronavirus infection in a child with severe combined immunodeficiency

    OpenAIRE

    Szczawinska‐Poplonyk, Aleksandra; Jonczyk‐Potoczna, Katarzyna; Breborowicz, Anna; Bartkowska‐Sniatkowska, Alicja; Figlerowicz, Magdalena

    2012-01-01

    Please cite this paper as: Szczawinska‐Poplonyk et al. (2012) Fatal respiratory distress syndrome due to coronavirus infection in a child with severe combined immunodeficiency. Influenza and Other Respiratory Viruses DOI: 10.1111/irv.12059. Coronaviruses have been demonstrated to contribute substantially to respiratory tract infections among the child population. Though infected children commonly present mild upper airway symptoms, in high‐risk patients with underlying conditions, particularl...

  2. The impact of infection on mortality in octogenarians who were admitted due to acute coronary syndrome.

    Science.gov (United States)

    Keskin, Kudret; Çetinkal, Gökhan; Sığırcı, Serhat; Yıldız, Süleyman Sezai; Çetin, Şükrü; Gürdal, Ahmet; Kocaş, Betül Balaban; Kılıçkesmez, Kadriye Orta

    The prevalence of coronary artery disease is on the rise as the life expectancy of the population increases. However, treatment of acute coronary syndrome in the elderly patients has its own problems that have not been thoroughly addressed in the clinical trials. Since these patients are generally fragile and have multiple co-morbidities, the course of acute coronary syndrome can frequently be complicated. Infection, which co-exists either at the initial presentation or is acquired during the hospital stay, is a condition about which there is little published data. Therefore, in our study, we wanted to assess the impact of infection on mortality in octogenarians who have acute coronary syndrome METHODS: We retrospectively analyzed the data of 174 octogenarians who had been admitted to the coronary care unit with acute coronary syndrome. All-cause mortality was defined as the primary endpoint of the study. Overall 53 octogenarian patients (30.5%) had an infection along with acute coronary syndrome. The mean duration of follow-up was 10 months (1-25 months). Both in-hospital and long-term mortality were higher in these patients (18.9% vs 6.6%, p = 0.01; 52.8% vs 27.5%, p < 0.01; respectively). Kaplan-Meier analysis also showed lower cumulative survival. (p [log-rank] = 0.002). In multivariate Cox regression analysis; undergoing coronary angiography, infection (HR 1.96, 95% CI 1.15-3.34, p = 0.01), left ventricular ejection fraction and maximum C reactive protein levels were found as independent predictors of long-term survival. Infection in octogenarians who were admitted due to acute coronary syndrome was frequent and increased their mortality substantially. Copyright © 2018 Elsevier B.V. All rights reserved.

  3. [Upper digestive haemorrhage due to Mallory-Weiss syndrome. Role of endoscopic sclerotherapy].

    Science.gov (United States)

    Elizalde, I; Zozaya, J M; Rodríguez, C; Carral, D; Jiménez, F J; Borda, F

    2001-09-01

    Although Mallory-Weiss syndrome is responsible for between 0.5 and 17% of the cases of upper digestive haemorrhage, the information existing on the endoscopic treatment of this syndrome is scarce. We made a retrospective study of 71 patients with haemorrhage due to Mallory-Weiss syndrome, dividing them into two groups according to the treatment they had received (medical or medical-endoscopic). Hence, 60 patients (30 with clean laceration, 9 with signs of prior haemostasia and 21 with fresh clotting) had been treated with procinetics and/or antisecretories alone, while the 11 remaining (8 with active haemorrhage, 2 with visible vessel and 1 with fresh clotting) had also received endoscopic treatment with sclerotherapy. We compared the clinical and analytical characteristics and the evolution of both groups of patients, analysing the data by means of the Mann-Whitney U and the chi 2 test. The endoscopic sclerosis group showed maelenas more frequently and more severe analytical data of haemorrhage (pMallory-Weiss syndrome with active bleeding or visible vessel presented a haemorrhage with a greater clinical and analytical repercussion. In this group of patients, endoscopic sclerotherapy controlled the haemorrhage and/or prevented rebleeding, in the absence of complications.

  4. To study prevalence of persistent pulmonary hypertension in newborn with meconium aspiration syndrome in western Rajasthan, India: a prospective observational study.

    Science.gov (United States)

    Choudhary, Mukesh; Meena, Mahesh Kumar; Chhangani, Narendra; Sharma, Deepak; Choudhary, Jagveer Singh; Choudhary, Sushil Kumar

    2016-01-01

    To study the prevalence of persistent pulmonary hypertension (PPHN) in newborn with meconium aspiration syndrome (MAS) in western Rajasthan, India. Hundred full-term newborns who had features of MAS at birth were included in this survey and were evaluated for PPHN using laboratory investigations, including pulse oximetry, ABG, chest X-ray, ECG and 2D color echocardiography. Nineteen neonates showed PPHN, of them 16 had a shunt reversal at PFO level and the rest at PDA level. Most of these newborns were delivered by emergency cesarean section and were unplanned. A majority of neonates of PPHN (84.21%) were diagnosed within 48 h of life and 73.69% had Downey's score more than 6. Neonates of PPHN had mean PH 7.21 ± 0.07, mean PCO2 53.73 ± 6.8, mean PaO2 61.10 ± 10.61 and mean PaO2/FiO2 144.03 ± 46.31. PPHN is a genuine problem in MAS-born neonates and is commonly seen in neonates born by unplanned and unmonitored delivery, and the prevalence of PPHN can be reduced by providing good antenatal care, regular follow up of high-risk pregnancy. 2D echocardiography is an important point of care in the diagnosis of PPHN in nursery and should be promoted in nurseries of developing countries as being engaged in developed countries for more reliable treatment.

  5. Meconium aspiration syndrome--a 21-years' experience from a tertiary care center and analysis of risk factors for predicting disease severity.

    Science.gov (United States)

    Hofer, N; Jank, K; Resch, E; Urlesberger, B; Reiterer, F; Resch, B

    2013-12-01

    Aim of this study was to describe the course of perinatal factors in neonates with meconium aspiration syndrome (MAS) from 1990 to 2010 and to determine risk factors for a severe course of the disease.All neonates with MAS hospitalized in our level III neonatal intensive care unit from 1990 to 2010.Retrospective analysis of trends of perinatal factors in neonates with MAS over time and of the association of these factors with severe MAS (need for invasive mechanical ventilation for ≥7 days, or need for high frequency oscillation or need for extracorporeal membrane oxygenation).We included 205 neonates with MAS, 55 had severe MAS (27%). MAS incidence and absolute number of MAS cases per year decreased during the observation period (p=0.003 and 0.005, respectively) as well as rates of outborn deliveries (p=0.004), duration of invasive mechanical ventilation (p=0.004), and hospital stay (p=0.036). Incidence and absolute number of severe MAS cases per year decreased (p=0.008 and 0.006, respectively), though the percentage of severe MAS among all neonates with MAS did not change. Risk factors for severe MAS were acute tocolysis (odds ratio 18.2 (95% confidence interval 2.1-155.3), p<0.001) fetal distress (3.4 (1.8-6.4), p<0.001), and severe and moderate birth asphyxia (4.4 (2.0-9.7), p=0.001 and 2.9 (1.5-5.6), p=0.009).The incidence and absolute numbers of MAS and severe MAS cases changed during the study period as well as neonatal management. Acute tocolysis, fetal distress, and asphyxia were associated with severe MAS. © Georg Thieme Verlag KG Stuttgart · New York.

  6. Cushing syndrome in a young woman due to primary pigmented nodular adrenal disease.

    Science.gov (United States)

    Hackman, Kathryn L; Davis, Anna L; Curnow, Paul A; Serpell, Jonathan W; McLean, Catriona A; Topliss, Duncan J

    2010-01-01

    To report a case of Cushing syndrome due to apparently sporadic primary pigmented nodular adrenal disease in a young woman. We describe the clinical, biochemical, radiologic, and histologic findings of Cushing syndrome due to the rare condition of primary pigmented nodular adrenal disease. A 30-year-old woman presented with a 2-year history of worsening itch without rash over her shoulders and arms and weight gain, particularly around the abdomen and face. Careful questioning did not elicit any history of exogenous glucocorticoid use (systemic or topical), including hydrocortisone. On examination, the patient had a slightly rounded and plethoric face, a small buffalo hump, central adiposity, and thin skin with a few small striae on her inner thighs. No features of the Carney complex were observed. Investigations showed hypercortisolism with suppressed corticotropin and normal adrenal imaging despite documentation of enlarged adrenal glands at removal. High-dose dexamethasone administration was followed by a decrease in urinary free cortisol excretion rather than a paradoxical rise as previously reported in primary pigmented nodular adrenal disease. No mutations were detected in the PRKAR1A gene. Primary pigmented nodular adrenal disease should be suspected in patients with corticotropin-independent Cushing syndrome who have normal adrenal imaging. The role of genetic testing in apparently sporadic cases is not established, but cumulative experience may be helpful in defining the frequency of PRKAR1A mutations.

  7. Dermatomyositis presenting as a paraneoplastic syndrome due to underlying breast cancer.

    Science.gov (United States)

    Sandhu, Nicole P; Zakaria, Shaheen; Degnim, Amy C; Boughey, Judy C

    2011-02-02

    Breast cancer most often presents as a palpable mass or with an abnormal mammogram. Much less commonly, breast cancer may present as a paraneoplastic syndrome. Dermatomyositis (DM) is a rare disease most often considered a complement-mediated idiopathic inflammatory myopathy manifested by classic skin findings and proximal muscle weakness. However, DM may also be due to a paraneoplastic syndrome associated with an underlying malignancy. The authors present a case report of a woman with presumed contact dermatitis who was diagnosed with breast cancer in the setting of progressive fatigue and muscle weakness. DM was subsequently diagnosed. Treatment of DM simultaneous with treatment of the breast cancer led to regression of DM. The diagnosis of DM in an adult should raise suspicion of an underlying malignancy. Breast cancer is a common disease that may rarely present with uncommon features that may divert attention from the underlying malignancy.

  8. Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias

    Directory of Open Access Journals (Sweden)

    Hyeoh Won Yu

    2016-03-01

    Full Text Available Adrenocorticotropin-independent adrenal hyperplasias are rare diseases, which are classified into macronodular (>1 cm and micronodular (≤1 cm hyperplasia. Micronodular adrenal hyperplasia is subdivided into primary pigmented adrenocortical disease and a limited or nonpigmented form 'micronodular adrenocortical disease (MAD', although considerable morphological and genetic overlap is observed between the 2 groups. We present an unusual case of a 44-month-old girl who was diagnosed with Cushing syndrome due to MAD. She had presented with spotty pigmentation on her oral mucosa, lips and conjunctivae and was diagnosed with multiple bone tumors in her femur, pelvis and skull base at the age of 8 years. Her bone tumor biopsies were compatible with osteoblastoma. This case highlights the importance of verifying the clinicopathologic correlation in Cushing syndrome and careful follow-up and screening for associated diseases.

  9. Joint Aspiration (Arthrocentesis)

    Science.gov (United States)

    ... aspiration is diagnostic but it also can be therapeutic, helping to relieve pain and swelling caused by ... syringe. Sometimes, imaging with an X-ray or ultrasound is required to guide the aspiration needle to ...

  10. Microangiopathic antiphospholipid antibody syndrome due to anti-phosphatidylserine/prothrombin complex IgM antibody.

    Science.gov (United States)

    Senda, Yumi; Ohta, Kazuhide; Yokoyama, Tadafumi; Shimizu, Masaki; Furuichi, Kengo; Wada, Takashi; Yachie, Akihiro

    2017-03-01

    Herein we describe a case of microangiopathic antiphospholipid syndrome (MAPS) due to anti-phosphatidylserine/prothrombin complex (aPS/PT) IgM antibody successfully treated with rituximab. A significant correlation was observed between the clinical course and the aPS/PT IgM antibody titer, which can rise earlier before the appearance of clinical symptoms. Rituximab can be safely and effectively used for MAPS. Although detection of only aPS/PT IgM antibody is rare, aPS/PT IgM antibody might be associated with the pathogenesis of MAPS and might be a useful marker of disease activity. © 2017 Japan Pediatric Society.

  11. Complex Regional Pain Syndrom Due to Cat Bite: A Case Report

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    Tuncay Cakir

    2014-04-01

    Full Text Available Complex regional pain syndrome (CPRS, is a painful and disabling disorder that can effect one or more extremities, characterized by a combination of vasomotor, sudomoto rand dystrophic alterations and may cause restriction in movements. The pain of this condition out of proportion to the severity of the initial injury. There are many aetiologic factors such as soft tissue injury, surgery, immobilization or myocardial ischemia. The pathophysiology of CPRS is not clear but peripheral and central sensitization resulting in neurogenic inflammation has been held responsible. This case report discribes the clinical condition of CPRS due to cat bite, and is presented to emphasize rare etiologic factors that may cause CPRS.

  12. [Meningitis due to Bacillus cereus in an infant with Reye syndrome].

    Science.gov (United States)

    Ferroni, A; Odièvre, M H; Abachin, E; Révy, P; Casanova, J L; Saudubray, J M; Berche, P; Nassif, X

    1998-10-01

    We report the case of a 2.5-month-old infant with Bacillus cereus meningitis who was initially admitted for Reye syndrome. Gram positive bacteria was isolated in CSF and shown to be located inside the polymorphonuclears. This pathogen was further identified by sequencing of the 16S RNA. Early administration of imipenem in association with amikacin resulted in a rapid recovery. No obvious immune defect or invasive procedure could be assessed. Although Bacillus cereus is mainly associated with contamination, repeated isolations of this bacteria may be due to true infection.

  13. Novel SCN5A mutation associated with idiopathic ventricular fibrillation due to subclinical Brugada syndrome

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    Juan Jiménez-Jáimez

    2011-12-01

    Full Text Available Idiopathic ventricular fibrillation can be caused by subclinical channelopathies such as Brugada syndrome. Our objective is to study the clinical behaviour of a new SCN5A mutation found in a woman with idiopathic ventricular fibrillation. A 53-year-old woman presented with multiple episodes of ventricular fibrillation, a structurally normal heart and normal baseline electrocardiogram. Genetic testing included KCNQ1, KCNH2, SCN5A, KCNE1, KCNE2 and KCNJ2 and identified a mutation in SCN5A (D1816fs/g98747-98748insT. We studied 15 immediate family members by means of electrocardiogram, echocardiogram, flecainide challenge test and genetic study. Eight subjects had the mutation. The flecainide challenge test was positive for Brugada syndrome in two subjects in the case group and none in the control group. The PR and QRS intervals on the baseline electrocardiogram were longer in the case group. The left atrial volume indexed to body surface was higher in the case group, likely due to the fact that two patients with the mutation had atrial fibrillation and none had it in the control group. The D1816fs/g98747-98748insT mutation in SCN5A may be associated with idiopathic ventricular fibrillation and Brugada syndrome with a broad phenotypic spectrum and incomplete penetrance. Genetic testing may be useful to identify the etiology of idiopathic ventricular fibrillation in patients with a negative thorough clinical evaluation.

  14. Clear lens phacoemulsification in the anterior lenticonus due to Alport Syndrome: two case reports.

    Science.gov (United States)

    Aslanzadeh, Ghassem Amir; Gharabaghi, Davoud; Naderi, Niloofar

    2008-05-27

    Alport Syndrome has a prevalence of 1 case per 5,000 people and 85% of patients have the X-linked form, where affected males develop renal failure and usually have high-tone sensorineural deafness by age 20. The main abnormality is deficient synthesis of type IV collagen, the main component of basement membranes. Common ocular abnormalities of this syndrome consist of dot-and-fleck retinopathy, posterior polymorphous corneal dystrophy, and anterior lenticonus, but other ocular defects such as cataracts, posterior lenticonus, and retinal detachments have also been reported. We report two cases of anterior lenticonus due to Alport Syndrome and describe clear lens phacoemulsification and foldable intraocular lens implantation as an effective and safe refractive procedure in the four eyes of these two patients. All four eyes of the two patients were in good condition after surgery and achieved satisfactory optical and visual results and had no remarkable complications at six-months follow-up. Clear lens phacoemulsification with foldable intraocular lens implantation can be used as an efficient and safe procedure for vision disorders in these patients.

  15. Clear lens phacoemulsification in the anterior lenticonus due to Alport Syndrome: two case reports

    Directory of Open Access Journals (Sweden)

    Aslanzadeh Ghassem

    2008-05-01

    Full Text Available Abstract Introduction Alport Syndrome has a prevalence of 1 case per 5,000 people and 85% of patients have the X-linked form, where affected males develop renal failure and usually have high-tone sensorineural deafness by age 20. The main abnormality is deficient synthesis of type IV collagen, the main component of basement membranes. Common ocular abnormalities of this syndrome consist of dot-and-fleck retinopathy, posterior polymorphous corneal dystrophy, and anterior lenticonus, but other ocular defects such as cataracts, posterior lenticonus, and retinal detachments have also been reported. Case presentation We report two cases of anterior lenticonus due to Alport Syndrome and describe clear lens phacoemulsification and foldable intraocular lens implantation as an effective and safe refractive procedure in the four eyes of these two patients. Conclusion All four eyes of the two patients were in good condition after surgery and achieved satisfactory optical and visual results and had no remarkable complications at six-months follow-up. Clear lens phacoemulsification with foldable intraocular lens implantation can be used as an efficient and safe procedure for vision disorders in these patients.

  16. Hepatic Scintigraphic Findings of Budd-Chiari Syndrome due to Inferior Vena Caval Obstruction

    International Nuclear Information System (INIS)

    Kim, Sung Hoon; Chung, Soo Kyo; Byun, Jae Young; Lee, Sung Yong; Shinn, Kyung Sub; Kim, Choon Yul; Bahk, Yong Whee

    1988-01-01

    Budd-Chiari syndrome (BCS) is a rare clinical entity characterized by post-sinusoidal portal hypertension caused by the obstruction to the hepatic vein outflow. The diagnosis is suggested by hepatic scintigraphy and is usually confirmed by hepatic venography, inferior vena cavography and biopsy. The scintigraphic finding of BCS caused by the obstruction of main hepatic vein has been reported to consist typically of hypertrophy of the caudate lobe with increased radionuclide accumulation. Such a typical finding has been accounted for by the fact that the venous outflow from the caudate lobe is preserved when the main hepatic vein is obstructed. But usually, the hepatic venous outflow from the caudate lobe is also obstructed in BCS due to inferior vena caval obstruction. So hepatic scintigraphic findings of BCS due to inferior vena caval obstruction show different findings as compared with the BCS due to hepatic vein obstruction. We evaluate the hepatic scintigrams of the 13 cases of BCS due to inferior vena caval obstruction and review the literatures. The results are as follows: 1) We cannot observe the caudate lobe hypertrophy with increased uptake, which is known as a classic finding in BCS due to hepatic vein obstruction. 2) The most prominent hepatic scintigraphic findings of BCS are nonhomogenous uptake in the liver with extrahepatic uptake in the all cases. 3) We can see cold areas at the superior aspect of right hepatic lobe in 7 cases (54%). This is a useful finding suggesting BCS due to inferior vena caval obstruction.

  17. Poverty and aspirations failure

    NARCIS (Netherlands)

    Dalton, P.S.; Ghosal, S.; Mani, A.

    We develop a theoretical framework to study the psychology of poverty and ‘aspirations failure’, defined as the failure to aspire to one’s own potential. In our framework, rich and the poor persons share the same preferences and same behavioral bias in setting aspirations. We show that poverty can

  18. Endoscopic repair of posterior ankle impingement syndrome due to os trigonum in soccer players.

    Science.gov (United States)

    López Valerio, Víctor; Seijas, Roberto; Alvarez, Pedro; Ares, Oscar; Steinbacher, Gilbert; Sallent, Andrea; Cugat, Ramón

    2015-01-01

    An os trigonum may cause posterior ankle impingement syndrome (PAIS), which may lead to poor sports performance, especially in soccer players. The aim of the present study was to analyze the outcomes of endoscopic repaired posterior ankle impingement (PAI) secondary to os trigonum syndrome within a group of soccer players as well as their return to play time. A retrospective review of 20 soccer players with Tegner activity level 9 was performed. All players were diagnosed of PAIS due to os trigonum. Chief complaint was pain produced with forced plantarflexion when kicking the ball. Conservative treatment was first performed during a 6-week rehabilitation program. When conservative treatment failed, arthroscopic surgical resection of the os trigonum was proposed. Visual analogue scale (VAS) was used to measure pain before and after surgery as well as time until their return to previous sports level. VAS showed a mean preoperative pain score of 7.5 (SD = 0.9), whereas postoperative VAS at 1 month after surgery decreased to 0.8 (SD = 1.36). Mean symptomatic period was 8.5 months (SD = 4.3), from the beginning of symptoms up to the surgery day. Once patients had undergone surgery, mean time until their return to previous level of sports was 46.9 days (SD = 25.96), reaching the same pre-lesion Tegner level. Endoscopic treatment of posterior ankle impingement syndrome due to os trigonum showed excellent results. Hindfoot endoscopy with a posterior approach was an effective treatment and allowed for a prompt return to play in soccer players with a high activity level. Level IV, therapeutic study. © The Author(s) 2014.

  19. Respiratory distress including meconium aspiration syndrome in vigorous neonates born through meconium stained amniotic fluid: incidence, onset, severity and predictors at birth.

    Science.gov (United States)

    Singh, S N; Srivastava, Roli; Singh, Anita; Tahazzul, M; Kumar, Mala; Kanta, Chandra; Chandra, S

    2013-07-01

    This study aimed to find out incidence, predictors, onset and severity of respiratory distress including meconium aspiration syndrome (MAS) among vigorous neonates born through meconium stained amniotic fluid (MSAF), which may or may not be evident at birth. Two hundred ninety vigorous neonates were studied. Data were collected on perinatal risk factors, clinical course and development of respiratory distress. Predictors of respiratory distress were identified by logistic regression and a score based on adjusted OR was assigned for each. Diagnostic performance of the score (0-24) was assessed on another 247 vigorous neonates using receiver operator characteristic analysis (ROC). Respiratory distress developed in 97(33.4 %) infants, MAS in 75(25.9 %). The distress appeared within 12 h in 97.9 %, was severe in only 21.7 %. Of 10 risk factors significantly associated with respiratory distress, seven entered in regression analysis. Fetal distress(adj OR = 11.8; 95%CI = 6.2-22.5), prolonged labor(adj OR = 5.2; 95%CI = 2.5-10.7), and absent/poor cry(adj OR = 5.6; 95%CI = 2.4-13.3) were identified as independent predictors; each assigned a score of 12, 6 and 6, respectively. To predict respiratory distress, a cut-off score of 9 points had sensitivity-74.1 % (95%CI = 63.3 %-82.7 %), specificity-84.6 % (95 % CI = 77.9 %-89.6 %), positive predictive value- 71.6 % (95%CI = 60.8 %-80.4 %), negative predictive value- 86.2 % (95 % CI = 79.6 %-90.9 %), likelihood ratio (LR) + ve 4.8(95%CI = 3.3-7.0) and LR-ve 0.3(95%CI = 0.2-0.4). Respiratory distress occurred in one third neonates, mostly had onset within 12 h of birth, and it was mild to moderate in majority. Fetal distress, prolonged labor, and absent/poor cry predicted respiratory distress and were validated. However, larger studies in different settings are required to confirm its utility.

  20. Severe jaundice due to coexistence of Dubin-Johnson syndrome and hereditary spherocytosis: a case report.

    Science.gov (United States)

    Korkmaz, Uğur; Duman, Ali Erkan; Oğütmen Koç, Deniz; Gürbüz, Yeşim; Dındar, Gökhan; Ensaroğlu, Fatih; Sener, Selçuk Yusuf; Sentürk, Omer; Hülagü, Sadettin

    2011-08-01

    Dubin-Johnson syndrome is a chronic, benign, intermittent jaundice, mostly of conjugated hyperbilirubinemia. The level of bilirubin is not expected to be more than 20 mg/dl in this syndrome. In this article, we report a patient who was evaluated for hyperbilirubinemia and liver function test abnormalities and diagnosed with Dubin-Johnson syndrome coexisting with hereditary spherocytosis. We suggest that other diseases should be investigated if patients with Dubin-Johnson syndrome present with severe hyperbilirubinemia. Dubin-Johnson syndrome accompanied by hemolytic diseases might also have high coproporphyrin levels (as in Rotor's syndrome) than expected in pure Dubin-Johnson syndrome.

  1. Dysphagia, dystussia, and aspiration pneumonia in elderly people

    OpenAIRE

    Ebihara, Satoru; Sekiya, Hideki; Miyagi, Midori; Ebihara, Takae; Okazaki, Tatsuma

    2016-01-01

    Despite the development and wide distribution of guidelines for pneumonia, death from pneumonia is increasing due to population aging. Conventionally, aspiration pneumonia was mainly thought to be one of the infectious diseases. However, we have proven that chronic repeated aspiration of a small amount of sterile material can cause the usual type of aspiration pneumonia in mouse lung. Moreover, chronic repeated aspiration of small amounts induced chronic inflammation in both frail elderly peo...

  2. Termination of pregnancy due to Thalassemia major, Hemophilia, and Down's Syndrome: the views of Iranian physicians

    Directory of Open Access Journals (Sweden)

    Zareifar Soheila

    2008-12-01

    Full Text Available Abstract Background Genetic disorders due to kindred marriages are common medical conditions in Iran; however, the legal aspects of abortion remain controversial. This study was undertaken to determine physicians' opinions regarding the termination of pregnancy for three genetic diseases: thalassemia major, hemophilia, and Down's syndrome. Methods A questionnaire was administered to selected physicians by stratified random sampling to determine the following: age, gender, knowledge about prenatal diagnosis of diseases in high risk pregnancies, agreement with abortion, recommended gestational age for abortion, and, if opposed to abortion, the reason. Results Of 323 physicians, who participated in the study, 91.3(295, 40.6(131, and 78.6%(254 were in agreement and 8.7(28, 59.4(192, and 21.4%(69 were opposed to abortion for thalassemia major, hemophilia, and Down's syndrome, respectively. Among 289 physicians opposed to abortion in respect of each of all three conditions, the following reasons were cited: religion, 18; emotional, 10; quality of care, 23; hope to find a new treatment option in the future, 103; miscellaneous reasons, 6; and a combination of these reasons, 129. Among 680 physicians in agreement with abortion in relation to all of the diseases, 4.6%(31 were agreed with abortion in less than 12 weeks gestation, 79.2%(538 in less than 16 weeks gestation, 5.6%(38 in less than 20 weeks gestation, 2.2%(15 in less than 24 weeks gestation, and 8.4%(58 were agreed with beyond the 24 weeks of gestational age. Conclusion The majority of physicians were in agreement with abortion for thalassemia major and Down's syndrome because of the overall prognosis, but opposed to abortion for hemophilia.

  3. Angelman syndrome due to paternal uniparental disomy of chromosome 15: A milder phenotype?

    Energy Technology Data Exchange (ETDEWEB)

    Bottani, A.; Robinson, W.P.; DeLoizer-Blanchet, C.D.; Engel, E.; Morris, M.A.; Schmitt, Thun-Hohenstein, L.; Schinzel, A. [Univ. of Zuerich (Switzerland)

    1994-05-15

    The Angelman syndrome (AS) is a neurological disorder characterized by severe mental retardation, absent speech, seizures, gait disturbances, and a typical age-dependent facial phenotype. Most cases are due to an interstitial deletion on the maternally inherited chromosome 15, in the critical region q11-q13. Rare cases also result from paternal uniparental disomy of chromosome 15. In a group of 14 patients with sporadic AS diagnosed in Switzerland, we found 2 unrelated females with paternal isodisomy for the entire chromosome 15. Their phenotypes were milder than usually seen in this syndrome: one girl did not show the typical AS facial changes; both patients had late-onset mild seizures; as they grow older, they had largely undisturbed gross motor functions, in particular no severe ataxia. Both girls were born to older fathers (45 and 43 years old, respectively). The apparent association of a relatively milder phenotype in AS with paternal uniparental disomy will have to be confirmed by detailed clinical descriptions of further patients. 25 refs., 2 figs., 1 tab.

  4. Iatrogenic Cushing's syndrome and osteoporosis due to an interaction between fluticasone and ritonavir.

    Science.gov (United States)

    Azevedo, Luísa; Pêgo, Hugo; Souto Moura, Teresa; Germano, Isabel

    2015-10-29

    The advent of highly active antiretroviral therapy for HIV infection dramatically changed the landscape of the disease. Ritonavir, a protease inhibitor (PI) frequently used in low doses to 'boost' the concentrations of other PIs, inhibits the cytochrome P450 3A4 isoenzyme, a common metabolic pathway to multiple drugs, so the potential for drug interactions is not negligible. A 39-year-old man with HIV-1 infection, treated with a ritonavir-boosted PI, was started on fluticasone/salmeterol inhaler and intranasal fluticasone, in 2009, in the setting of asthma and allergic rhinitis. In 2013, he presented with 1-year evolution of symptoms suggesting Cushing's syndrome, and was experiencing recurrent falls. A spine CT showed a vertical L3 fracture and thoracolumbar erosions; a bone density scan revealed severe osteoporosis. Hormonal assays were compatible with hypothalamic-pituitary-adrenal axis suppression, and iatrogenic Cushing's syndrome due to ritonavir-fluticasone interaction was considered. Fluticasone was suspended and oral corticosteroid replacement initiated, with a favourable outcome. 2015 BMJ Publishing Group Ltd.

  5. Refeeding syndrome in a patient with advanced Kidney failure due to Nephronophthisis

    Directory of Open Access Journals (Sweden)

    Kamel El-Reshaid

    2013-01-01

    Full Text Available Refeeding syndrome (RS is a serious and potentially fatal disorder. It is caused by a shift of fluids, sodium, potassium, magnesium and phosphorus as well changes in the metabolism of glucose, protein, fat and vitamins following the refeeding of malnourished patients, whether enterally or parenterally. RS has rarely been reported in patients with advanced kidney disease probably due to the pre-existing hyperphosphatemia, hypermagnesemia and hyperkalemia in these patients. In the following report, we present a patient with nephronophthisis type 1 deletion syndrome in whom her main previous nutrition was limited to simply rehydration to avoid renal replacement therapy. On presentation, she was cachectic and dehydrated with advanced kidney failure. She was treated with medical nephrectomy using non-steroidal anti-inflammatory drugs and then placed on maintenance hemodialysis. Percutaneous endoscopic gastrostomy was used for her initial feeding. Care was exercised during her early refeeding with regard to correction of fluids and essential electrolytes, viz. potassium, phosphorus and magnesium, as well as multivitamins to avoid the cardiovascular and neurological complications of RS. However, the changes in the gut, pancreas and liver as well as her hyperlipidemia were a clear obstacle. Fortunately, the ileus and pancreatitis she developed on refeeding improved dramatically with a decrease of the feeding dose to half; however, the liver abnormalities and hyperlipidemia were severe and slow to recover. These improved after addition of ursodeoxycholic acid and permitted successful increase of the dose of feeding subsequently.

  6. Septic Shock due to Cytomegalovirus Infection in Acute Respiratory Distress Syndrome after Falciparum Malaria.

    Science.gov (United States)

    Harbarth; Meyer; Grau; Loutan; Ricou

    1997-09-01

    Incidence of falciparum malaria in developed countries has increased in recent years due to tourism to tropical countries and immigration from Asia and Africa. In Switzerland, about 250 cases of malaria were reported in 1994 to the Federal Office of Health, including three cases with fatal outcome.1 The most commonly described complications of plasmodia infection are cerebral malaria, acute renal failure, and severe anemia with disseminated intravascular coagulation. However, pulmonary involvement occurs in 3 to 10% of cases and represents the most serious complication of this infection, with a lethality of 70%.2,3 Furthermore, a pronounced general immunosuppression has been reported in malaria patients, which may predispose them to opportunistic infections.4 We report a case of Plasmodium falciparum infection complicated by severe acute respiratory distress syndrome (ARDS) with development of systemic cytomegalovirus (CMV) infection leading to death. This evolution implies a severe immune deficiency associated with malaria, as previously suggested in the literature.

  7. Corticosteroid-induced asthma: a manifestation of limited hyperinfection syndrome due to Strongyloides stercoralis.

    Science.gov (United States)

    Sen, P; Gil, C; Estrellas, B; Middleton, J R

    1995-09-01

    Inadequate therapeutic response to parenteral corticosteroids in patients with acute bronchial asthma is infrequent. We report four patients whose bronchial asthma symptoms worsened after treatment with parenteral corticosteroids. All had larvae of Strongyloides stercoralis in the stool. The new attack or the exacerbation of asthma appeared to be precipitated by systemic corticosteroid administration. The paradoxic therapeutic response of asthma to glucocorticoides was the major pulmonary manifestation of Strongyloides superinfection; there was no evidence of other organ involvement. Individuals with new onset of bronchial asthma or worsening of asthmatic episodes concurrent with the use of systemic corticosteroids should have thorough investigation for possible superinfection due to Strongyloides stercoralis. This is particularly important for patients who have resided in areas where intestinal helminthic infections are endemic. Discontinuance of steroid therapy or reduction in dosage of parenteral steroids appears necessary. Treatment with thiabendazole appears to be effective in patients with limited hyperinfection syndrome.

  8. A Pregnant Woman Who Underwent Laparoscopic Adrenalectomy due to Cushing’s Syndrome

    Directory of Open Access Journals (Sweden)

    Halit Diri

    2014-01-01

    Full Text Available Cushing’s syndrome (CS may lead to severe maternal and fetal morbidities and even mortalities in pregnancy. However, pregnancy complicates the diagnosis and treatment of CS. This study describes a 26-year-old pregnant woman admitted with hypertension-induced headache. Hormonal analyses performed due to her cushingoid phenotype revealed a diagnosis of adrenocorticotropic hormone- (ACTH- independent CS. MRI showed a 3.5 cm adenoma in her right adrenal gland. After preoperative metyrapone therapy, she underwent a successful unilateral laparoscopic adrenalectomy at 14-week gestation. Although she had a temporary postoperative adrenal insufficiency, hormonal analyses showed that she has been in remission since delivery. Findings in this patient, as well as those in previous patients, indicate that pregnancy is not an absolute contraindication for laparoscopic adrenalectomy. Rather, such surgery should be considered a safe and efficient treatment method for pregnant women with cortisol-secreting adrenal adenomas.

  9. Mild Angelman syndrome phenotype due to a mosaic methylation imprinting defect.

    Science.gov (United States)

    Fairbrother, Laura C; Cytrynbaum, Cheryl; Boutis, Paula; Buiting, Karin; Weksberg, Rosanna; Williams, Charles

    2015-07-01

    Angelman syndrome (AS) is a neurogenetic disorder causing severe to profound intellectual disability, absent or very limited speech and a high risk for seizures. AS is caused by a loss of function of the maternally-derived UBE3A allele due to one of several mechanisms including imprinting defects (ImpDs). We present a girl with AS due to a mosaic ImpD who has relatively high developmental function (VABS-II composite score of 76) and communication skills (as demonstrated in supplemental video links). Given the patient's relatively mild developmental impairment, without clinical evidence of seizures, gait disturbance or inappropriate laughter, the diagnosis of AS was not initially suspected. Initial laboratory testing for AS was inconclusive but additional studies suggested mosaic ImpD and characteristic EEG findings provided further support for the clinical diagnosis. Our patient, along with other case reports of children with AS and relatively mild phenotypes, raises the question as to whether there exists an undiagnosed group of individuals with mild intellectual disability and expressive speech delays due to mosaic methylation defects of the chromosome 15q11.2-13 region. Population studies may be needed to determine if such an undiagnosed group exists. © 2015 Wiley Periodicals, Inc.

  10. Ovarian hyperstimulation syndrome due to follicle-stimulating hormone-secreting pituitary adenomas.

    Science.gov (United States)

    Caretto, Amelia; Lanzi, Roberto; Piani, Cecilia; Molgora, Michela; Mortini, Pietro; Losa, Marco

    2017-10-01

    Gonadotroph adenomas are pituitary adenomas with inefficient and variable secretory characteristics, that is why they are usually considered as a subgroup of nonfunctioning pituitary adenomas (NFPA) and are recognized only at immunohistochemistry. When gonadotroph adenomas secrete active hormones, they may cause spontaneous ovarian hyperstimulation syndrome (OHSS) in premenopausal women. Aim of our study is to describe three women with OHSS diagnosed before the removal of the adenoma and to calculate the prevalence of OHSS in premenopausal women with a clinical diagnosis of NFPA. We reviewed clinical records of premenopausal women that underwent neurosurgery for NFPA at our centre between 1993 and 2014. OHSS was diagnosed in patients with high levels of FSH, suppressed LH, hyperestrogenism, abdominal symptoms, polymenorrhea, enlarged ovaries with cysts or previous surgery for ovarian cysts. 171 women were included into the study; 62 (36.6%) had a gonadotroph adenoma diagnosed at immunohistochemistry. Two patients were retrospectively diagnosed as having OHSS due to gonadotroph adenoma and three had OHSS diagnosed before neurosurgery. The prevalence of OHSS was 2.9% in the overall group of patients with NFPA and 8.1% among patients with a gonadotroph adenoma detected at immunohistochemistry. Frequency of OHSS due to a gonadotroph adenoma is not negligible. Increased awareness of the characteristic clinical and hormonal picture should permit an early detection of this condition in premenopausal women with a pituitary adenoma.

  11. Gender differences in patients starting long-term home mechanical ventilation due to obesity hypoventilation syndrome.

    Science.gov (United States)

    Palm, Andreas; Midgren, Bengt; Janson, Christer; Lindberg, Eva

    2016-01-01

    Obesity hypoventilation syndrome (OHS) is often diagnosed late. The aim of this study was to analyse gender differences at initiation of long-term mechanical ventilation (LTMV) in patients with (OHS), to analyse gender differences in treatment effect and to study how the prescription of LTMV due to OHS has changed over time. Data on patients on LTMV due to OHS between 1996 and 2014 were obtained from Swedevox, a nationwide health quality registry of patients on LTMV in Sweden. When starting LTMV, women were generally older (age 64.4 ± 11.2 vs. 60.1 ± 12.1 years, p obese (BMI 43.0 ± 8.2 vs. 41.5 ± 7.9 kg/m2, p differ. During the study period, the age of patients at the initiation of LTMV rose by 3.4 years/decade (P = 0.001) in women and with 1.9 years/decade (P = 0.048) in men but there were no significant changes in BMI (P = 0.425). Diagnosis of OHS is more delayed in women and as a consequence the disease is more advanced when diagnosed. In spite of this, there is no gender difference in survival rate in patients with OHS treated with LTMV. More and older patients with OHS nowadays gain access to LTMV. Copyright © 2015 Elsevier Ltd. All rights reserved.

  12. The Compartment Syndrome Associated with Deep Vein Thrombosis due to Rattlesnake Bite: A Case Report

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    Radu Ciprian Tincu

    2017-08-01

    Full Text Available Background: Snakebite is a health issue specific to some parts of the world, especially in the tropical area, where it produces many victims. The main clinical damage caused by snake bite involves hemotoxic, neurotoxic and myotoxic reactions. It is also established that the importance of systemic impairment varies according to individual factors and are related to organ dysfunction, shock or hypotension. We report the case of a young woman suffering from snakebite who developed deep vein thrombosis and compartment syndrome. Case Report: We present the case of a 32-year-old Romanian woman who was injured by her own Crotalinae snake (also known as pit viper or rattlesnake on her left forearm. When admitted to our Emergency Department, she was conscious with a Glasgow coma scale of 12/15, somnolent, febrile, suffering of headache, tachypnea; the marks of the snakebite were located in the distal part of the anterior left forearm; she had pain and bleeding at the bite site and swelling of the left upper limb with lymphangitis up to the axilla. She experienced fasciotomy-requiring compartment syndrome of the upper limb and required unfractionated heparin and closed monitored using activated partial thromboplastin time evolution due micro-thrombosis in the brachial vein. Local improvement was achieved in the next 4 days with progressive diminish of local tenderness and swelling. Conclusion: Limb deep vein thrombosis might be induced by snakebite, despite pro-hemorrhagic general condition induced by the envenomation. High index of clinical suspicion is needed for early diagnosis and timely management which can improve survival of these patients

  13. Aspiration and leadership

    NARCIS (Netherlands)

    Jagersma, P.K.

    2007-01-01

    Purpose - Management needs an aspiration as to how the company will work in the future. It needs a guide for corporate priorities. Any company - local or global - must be driven by an aspiration that energizes and motivates the company from top to bottom. Yet very few managers and executives know

  14. Aspiration and leadership

    NARCIS (Netherlands)

    Jagersma, P.K.

    2007-01-01

    Management needs an aspiration as to how the company will work in the future. It needs a guide for corporate priorities. Any company - local or global - must be driven by an aspiration that energizes and motivates the company from top to bottom. Yet very few managers and executives know how a

  15. Poverty and Aspirations Failure

    NARCIS (Netherlands)

    Dalton, P.S.; Ghosal, S.; Mani, A.

    2011-01-01

    We develop a theoretical framework to study the psychology of poverty and 'aspirations failure'. In our framework, the rich and the poor share the same preferences - and also a behavioral bias in setting aspirations. Greater downside risks imposed by poverty exacerbates the effects of this

  16. Sensory Loss Mimicking Cauda Equina Syndrome due to Cervical Spinal Lesion in a Patient with Clinically Isolated Syndrome

    OpenAIRE

    Giulia Vinceti; Andrea Zini; Paolo Nichelli; Jessica Mandrioli

    2012-01-01

    We describe the case of a 39-year-old woman with signs and symptoms suggesting cauda equina syndrome. Lumbosacral magnetic resonance imaging (MRI) demonstrated no lesion at this level, while cervical MRI showed a T2-hyperintense lesion in the middle-right anterolateral region of the cervical spinal cord, which may explain the symptoms by involving the anterior spinothalamic tract. We suggest that in cases with cauda equina syndrome presentation and normal lumbosacral MRI, a cervicodorsal lesi...

  17. An Atypical Rett Syndrome Phenotype Due to a Novel Missense Mutation in CACNA1A.

    Science.gov (United States)

    Epperson, Madison V; Haws, Michael E; Standridge, Shannon M; Gilbert, Donald L

    2018-03-01

    Some typical and atypical Rett syndrome patients lack known genetic mutations. Mutations in the P/Q type calcium channel CACNA1A have been implicated in epileptic encephalopathy, familial hemiplegic migraine, episodic ataxia 2, and spinocerebellar ataxia 6, but not Rett syndrome. Patient Description: The authors describe a female patient with developmental regression and a de novo, likely pathogenic mutation in CACNA1A who meets 3 of 4 main criteria (stereotypic hand movements, loss of purposeful hand movements, gait disturbance), and 6 of 11 supportive criteria (impaired sleep, abnormal tone, vasomotor disturbance, scoliosis, growth retardation, and screaming spells) for atypical Rett syndrome. Furthermore, she resembles the early seizure variant of Rett syndrome. Previously, 3 children with similar CACNA1A mutations have been reported, but a Rett syndrome phenotype has not been described. CACNA1A mutations should be considered in children presenting with an atypical Rett syndrome phenotype, specifically, the early seizure variant.

  18. [Good's syndrome and congenital toxoplasmosis due to maternal reactivation during pregnancy].

    Science.gov (United States)

    Tahiri, J; Fouyssac, F; Morel, O; Maatouk, A

    2017-05-01

    Good syndrome is a rare condition in which thymoma is associated with hypogammaglobulinemia. It is characterized by an increased susceptibility to infections. We report a woman with Good's syndrome diagnosed after severe congenital toxoplasmosis in her daughter, even though she was immunized against this infection during pregnancy. This presentation is very unusual by its early diagnosis and to our knowledge is the first report of parasitic infection in this syndrome. Copyright © 2016. Published by Elsevier SAS.

  19. Assessment of the Personal Losses Suffered by Correctional Officers due to Burnout Syndrome.

    Science.gov (United States)

    Stoyanova, R G; Harizanova, S N

    2016-01-01

    Professional burnout is defined as a state of depletion and loss of motivation accompanied by different mental and physical symptoms. To assess personal losses suffered by correctional officers due to burnout. This cross-sectional study conducted between June and December 2012 included 201 correctional officers in two Bulgarian prisons. The mean age of the whole group was 41.2 (SD 8.0) years. The respondents was mostly male (56.7%), married (72.6%), had a secondary educational level (61.7%), and 76.1% of them had been in current prison work over 5 years. The demographic characteristics had no influence on the occurrence of burnout but there was a correlation between level of burnout and the number of sick-leaves, the need for medical help, and the expenses spent on medications. Officers affected by burnout took more sick-leaves and this affected adversely their remuneration as they lost 3.1% of their annual wages. Their expenses spent on user fees for medical services were 3 times higher. Their monthly expenses spent on medications were 3.14 times higher than those of people without the burnout syndrome. The high level of burnout has a negative personal economic effect on the prison employees.

  20. Atypical hemolytic-uremic syndrome due to complement factor I mutation.

    Science.gov (United States)

    Almalki, Abdullah H; Sadagah, Laila F; Qureshi, Mohammed; Maghrabi, Hatim; Algain, Abdulrahman; Alsaeed, Ahmed

    2017-11-06

    Atypical hemolytic-uremic syndrome (aHUS) is a rare disease of complement dysregulation leading to thrombotic microangiopathy (TMA). Renal involvement and progression to end-stage renal disease are common in untreated patients. We report a 52-year-old female patient who presented with severe acute kidney injury, microangiopathic hemolytic anemia, and thrombocytopenia. She was managed with steroid, plasma exchange, and dialysis. Kidney biopsy shows TMA and renal cortical necrosis. Genetic analysis reveals heterozygous complement factor I (CFI) mutation. Eculizumab was initiated after 3 mo of presentation, continued for 9 mo, and stopped because of sustained hematologic remission, steady renal function, and cost issues. Despite this, the patient continued to be in hematologic remission and showed signs of renal recovery, and peritoneal dialysis was stopped 32 mo after initiation. We report a case of aHUS due to CFI mutation, which, to the best of our knowledge, has not been reported before in Saudi Arabia. Our case illustrates the challenges related to the diagnosis and management of this condition, in which a high index of suspicion and prompt treatment are usually necessary.

  1. Assessment of the Personal Losses Suffered by Correctional Officers due to Burnout Syndrome

    Directory of Open Access Journals (Sweden)

    RG Stoyanova

    2016-01-01

    Full Text Available Background: Professional burnout is defined as a state of depletion and loss of motivation accompanied by different mental and physical symptoms. Objective: To assess personal losses suffered by correctional officers due to burnout. Methods: This cross-sectional study conducted between June and December 2012 included 201 correctional officers in two Bulgarian prisons. The mean age of the whole group was 41.2 (SD 8.0 years. The respondents was mostly male (56.7%, married (72.6%, had a secondary educational level (61.7%, and 76.1% of them had been in current prison work over 5 years. Results: The demographic characteristics had no influence on the occurrence of burnout but there was a correlation between level of burnout and the number of sick-leaves, the need for medical help, and the expenses spent on medications. Officers affected by burnout took more sick-leaves and this affected adversely their remuneration as they lost 3.1% of their annual wages. Their expenses spent on user fees for medical services were 3 times higher. Their monthly expenses spent on medications were 3.14 times higher than those of people without the burnout syndrome. Conclusion: The high level of burnout has a negative personal economic effect on the prison employees.

  2. Endoscopic hemoclipping for upper GI bleeding due to Mallory-Weiss syndrome.

    Science.gov (United States)

    Yamaguchi, Y; Yamato, T; Katsumi, N; Morozumi, K; Abe, T; Ishida, H; Takahashi, S

    2001-04-01

    Endoscopic hemoclipping is known to be highly effective as hemostatic treatment for upper gastrointestinal bleeding. However, the efficacy and safety of hemoclipping for Mallory-Weiss syndrome (MWS) have not been reported. Thus, the aim of the present study was to assess prospectively the usefulness of endoscopic hemoclipping for MWS bleeding. This study was conducted from January 1994 to August 1999. Hemoclipping was performed when active bleeding (spurting, streaming or oozing), visible vessels or fresh adhesive clots were found on endoscopic examination. Patients who did not have any of these findings were conservatively treated. Follow-up endoscopy was performed within 24 hours, after 5 days and between 1 and 2 months after the procedure. MWS was diagnosed in a total of 58 patients during the study. Hemoclipping was performed in 26 patients and was technically successful in all cases. The average number of hemoclips used was 2.8 +/- 1.6 (range 1 to 8). The number of hemoclips required for hemostasis depended on the nature of the bleeding. No complications, recurrent bleeding, or deaths resulted. Follow-up endoscopy showed no evidence of hemoclip-induced tissue injury and no impairment of Mallory-Weiss tears. Endoscopic hemoclipping provided an effective and safe modality for obtaining hemostasis when bleeding is due to MWS.

  3. Trauma due to Self-aggression in Patient with Waardenburg Syndrome associated with Congenital Anomalies.

    Science.gov (United States)

    Marta, Sara Nader; Kawakami, Roberto Yoshio; Sgavioli, Claudia Almeida Prado Piccino; Correa, Ana Eliza; D'Árk de Oliveira El Kadre, Guaniara; Carvalho, Ricardo Sandri

    2016-08-01

    Waardenburg syndrome (WS) is an inherited autosomal dominant genetic disorder presenting variable penetrance and expressivity, with an estimated prevalence of 1:42,000. Clinical characteristics of WS include lateral displacement of the internal eye canthus, hyperplasia of the medial portion of the eyebrows, prominent and broad nasal base, congenital deafness, pigmentation of the iris and skin, and white forelock. A 24-year-old male patient, previously diagnosed with WS, was referred to the Special Needs Dental Clinic of Sacred Heart University, Bauru, Brazil. Parents reported that the patient was experiencing self-mutilation, particularly in the oral region. He presented multiple congenital anomalies, including anophthalmia, mental retardation, low-set ears, and leg deformities. Clinical oral examination revealed hypodontia, abnormalities in dental morphology, extensive dental caries, periodontal disease, and fistulae. Extensive scars on the tongue, lips, and hands caused by self-mutilation were also observed. In accordance with his family and neurologist, full-mouth extraction under general anesthesia was performed, especially considering his severe self-aggressive behavior and the necessity to be fed with soft-food diet due to his inability to chew. After the surgical procedure, a significant reduction in the patient's irritability and gain of weight were reported in the follow-ups of 30, 60, and 180 days.

  4. Clinical variability in neurohepatic syndrome due to combined mitochondrial DNA depletion and Gaucher disease

    Directory of Open Access Journals (Sweden)

    Julie Harvengt

    2014-01-01

    Full Text Available A 1-year-old girl born to consanguineous parents presented with unexplained liver failure, leading to transplantation at 19 months. Subsequent partial splenectomy for persistent cytopenia showed the presence of foamy cells, and Gaucher disease was confirmed by homozygosity for the p.Leu483Pro mutation in the GBA gene. She was treated by enzyme replacement therapy (ERT. Clinical follow-up showed mild developmental delay, strabismus, nystagmus and oculomotor apraxia. Biochemical studies revealed multiple respiratory chain deficiencies and a mosaic pattern of deficient complex IV immunostaining in liver and fibroblast. Molecular analysis identified a mtDNA depletion syndrome due to the homozygous p.Pro98Leu mutation in MPV17. A younger sister unaffected by mtDNA depletion, presented with pancytopenia and hepatosplenomegaly. ERT for Gaucher disease resulted in visceral normalization without any neurological symptom. A third sister, affected by both conditions, had marked developmental delay, strabismus and ophthalmoplegia but no liver cirrhosis. In conclusion, intrafamilal variability occurs in MPV17-related disease. The combined pathological effect of Gaucher and mitochondrial diseases can negatively impact neurological and liver functions and influence the outcome in consanguineous families. The immunocytochemical staining of OXPHOS protein in tissues and cultured cells is a powerful tool revealing mosaic pattern of deficiency pointing to mtDNA-related mitochondrial disorders.

  5. Iatrogenic Cushing's Syndrome Due to Intranasal Usage of Ophthalmic Dexamethasone: A Case Report.

    Science.gov (United States)

    Orton, Sarah; Censani, Marisa

    2016-05-01

    Iatrogenic Cushing's syndrome (ICS) is caused by exogenous corticosteroid administration with suppression of the hypothalamic-pituitary-adrenal axis. It has been commonly described with oral and topical steroid use, but scarce reports have documented intranasal steroid usage as the etiology in infancy. In this article, we describe a case of a 4-month-old infant who developed ICS after 6 weeks of intranasal dexamethasone ophthalmic solution administration for nasal obstruction. To our knowledge, this is the youngest patient reported with ICS due to intranasal use of a prescribed dose of an ophthalmic steroid. His hypothalamic-pituitary-adrenal axis recovered fully 4.5 months after steroid discontinuation. Because of the small body surface area and supine position during administration, infants are particularly susceptible to ICS. Given that intranasal steroids are commonly prescribed to infants and children for a variety of diagnoses, this case highlights the risks inherent in the use of intranasal steroid drops, particularly in young infants, for both adrenal suppression and linear growth deceleration, even with short-term use. Close monitoring of these patients' height and weight should occur while on steroid treatment, with every effort made to decrease or discontinue steroid use when possible. Copyright © 2016 by the American Academy of Pediatrics.

  6. [A case of occupational contact urticaria and oral allergy syndrome due to seafood].

    Science.gov (United States)

    Yamaguchi, Junko; Inomata, Naoko; Hirokado, Michiko; Shimakura, Kuniyoshi; Shiomi, Kazuo; Ikezawa, Zenro

    2007-01-01

    A 20-year-old woman was referred for evaluation after about 2 years of recurrent episodes of localized urticaria during handling of several kinds of raw fish in a sushi shop, where she had worked part-time for 2 years. She had also experienced allergic symptoms such as itching and swelling of her lips, generalized urticaria, laryngeal tightness, stridor and dyspnea immediately after ingestion of raw and cooked seafood, including sole, horse mackerel, sea eel and shellfish, over the previous 1 year before referral. Skin prick tests and blood test for specific IgE antibodies were positive for many kinds of seafood, including sole, horse mackerel, sea eel, eel, crab, and abalone, which belonged to different taxonomic phyla, including Chordata, Arthropoda, and Mollusca. A challenge with a piece of broiled sole induced swelling of the lips, obstruction of the larynx, difficulty with deglutition, and abdominal pain. In addition, serum-specific IgE antibodies to two major fish allergens, parvalbumin and collagen, were detected by ELISA, suggesting that allergic symptoms could be induced by many kinds of seafood in the present patient. She was therefore diagnosed with occupational contact urticaria and oral allergy syndrome due to seafood. At the time of this report, the present patient had been followed for one year and no reactions have occurred since she started to avoid the causative types of seafood.

  7. Recurrent Syncope Due to Carotid Sinus Hypersensitivity and Sick Sinus Syndrome

    Directory of Open Access Journals (Sweden)

    Feng-Yu Kuo

    2008-10-01

    Full Text Available Syncope is a sudden and brief loss of consciousness with postural tone. Its recovery is usually spontaneous. There are various causes of syncope including cardiac, vascular, neurologic, metabolic and miscellaneous origins. The tracing is usually time-consuming and costly. The diagnosis of carotid sinus syncope may sometimes be difficult since the symptoms are nonspecific, especially in older persons. Here, we report the case of a 72-year-old woman who sought medical attention at our hospital due to repeated syncope episodes over the previous 5 years. Neurologic examinations showed negative results (including brain computed tomography. Twenty-four-hour ambulatory electrocardiogram monitoring showed atrial and ventricular premature contractions only. Electrophysiologic study disclosed prolonged corrected sinus node recovery time (1,737 ms with poor atrioventricular conduction. Drop of blood pressure together with sinus bradycardia developed after left side carotid sinus massage. Both carotid sinus hypersensitivity with sick sinus syndrome contributed to this patient's syncope, and after pacemaker placement together with selective serotonin reuptake inhibitor treatment, she was free from syncope thereafter.

  8. Cor pulmonale due to congenital central hypoventilation syndrome presenting in adolescence.

    Science.gov (United States)

    Fine-Goulden, Miriam R; Manna, Soumendu; Durward, Andrew

    2009-07-01

    To report the first case of congenital central hypoventilation syndrome (CCHS) presenting with severe cor pulmonale in an adolescent. Case report and literature review. Our Institutional Review Board waived the need for consent. Pediatric intensive care unit in a tertiary care children's hospital. A 12-year-old girl who developed profound hypoxia following routine dental extraction under intravenous opiate sedation and became progressively obtunded due to marked hypoventilation without hypoxic arousal, requiring mechanical ventilation. She had evidence of severe right heart failure, but no cardiac, pulmonary, neurologic, or neuromuscular cause was identified. The diagnosis of CCHS was suspected and subsequently confirmed by blood polymerase chain reaction analysis that revealed a heterozygous polyalanine expansion mutation of the PHOX2B gene (five polyalanine repeats). This report describes the unusual presentation of severe cor pulmonale in an adolescent with so-called "late-onset" CCHS. CCHS was previously thought to be a disease affecting only neonates, but the late-onset phenotype has now been well described in adults. It should be considered in any child presenting with unexplained right heart failure without an identifiable cause, particularly if central sleep apnea is present, because early initiation of ventilatory support can prevent cardiac and neurologic sequelae and improve outcome.

  9. Congenital hypothyroidism due to ectopic sublingual thyroid gland in Prader-Willi Syndrome: a case report.

    Science.gov (United States)

    Bocchini, Sarah; Fintini, Danilo; Grugni, Graziano; Boiani, Arianna; Convertino, Alessio; Crinò, Antonino

    2017-09-22

    Thyroid gland disorders are variably associated with Prader-Willi syndrome (PWS). Many of the clinical features in newborns with PWS are similar to those found in congenital hypothyroidism (CH). We report a case of a girl with CH and PWS. At the age of 9 months CH caused by an ectopic sublingual thyroid was diagnosed, and hormone replacement therapy was started. In spite of this treatment a decrease in growth velocity, weight excess and delayed development were observed. At the age of 9 years PWS was suspected on the basis of phenotype and genetic tests confirmed a maternal uniparental disomy of chromosome 15. This is the second reported case of hypothyroidism due to an ectopic sublingual thyroid gland in PWS suggesting that, although rare, an association between CH and PWS may exist. In our case diagnosis of PWS was delayed because mental retardation, hypotonia, obesity and short stature were initially attributed to hypothyroidism. In this context PWS should be considered in obese children with CH who do not improve adequately with l-thyroxine therapy. Also, thyroid function in all PWS children should be assessed regularly in order to avoid delayed diagnosis of hypothyroidism.

  10. Mental health status of people isolated due to Middle East Respiratory Syndrome

    Directory of Open Access Journals (Sweden)

    Hyunsuk Jeong

    2016-11-01

    Full Text Available OBJECTIVES Isolation due to the management of infectious diseases is thought to affect mental health, but the effects are still unknown. We examined the prevalence of anxiety symptoms and anger in persons isolated during the Middle East Respiratory Syndrome (MERS epidemic both at isolation period and at four to six months after release from isolation. We also determined risk factors associated with these symptoms at four to six months. METHODS Of 14,992 individuals isolated for 2-week due to having contact with MERS patients in 2015, when MERS was introduced to Korea, 1,692 individuals were included in this study. Anxiety symptoms were evaluated with the Generalized Anxiety Disorder 7-item scale and anger was assessed with the State-Trait Anger Expression Inventory at four to six months after release from isolation for MERS. RESULTS Of 1,692 who came in contact with MERS patients, 1,656 were not diagnosed with MERS. Among 1,656, anxiety symptoms showed 7.6% (95% confidence interval [CI], 6.3 to 8.9% and feelings of anger were present in 16.6% (95% CI, 14.8 to 18.4% during the isolation period. At four to six months after release from isolation, anxiety symptoms were observed in 3.0% (95%CI, 2.2 to 3.9%. Feelings of anger were present in 6.4% (95% CI, 5.2 to 7.6%. Risk factors for experiencing anxiety symptoms and anger at four to six months after release included symptoms related to MERS during isolation, inadequate supplies (food, clothes, accommodation, social networking activities (email, text, Internet, history of psychiatric illnesses, and financial loss. CONCLUSIONS Mental health problems at four to six month after release from isolation might be prevented by providing mental health support to individuals with vulnerable mental health, and providing accurate information as well as appropriate supplies, including food, clothes, and accommodation.

  11. Creatine deficiency syndrome. A treatable myopathy due to arginine-glycine amidinotransferase (AGAT) deficiency

    NARCIS (Netherlands)

    Nouioua, S.; Cheillan, D.; Zaouidi, S.; Salomons, G.S.; Amedjout, N.; Kessaci, F.; Boulandour, N.; Hamadouche, T.; Tazir, M.

    2013-01-01

    We report two sisters, aged 11 and 6. years, with AGAT deficiency syndrome (OMIM 612718) which is the least common creatine deficiency syndrome. They were born full-term to consanguineous parents and had moderate developmental delay. Examination showed an important language delay, a progressive

  12. Sudden Cardiac Arrest due to Brugada Syndrome: a Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    R Soleimanirad

    2013-04-01

    Full Text Available Brugada Syndrome is a rare cause of sudden cardiac arrest and has a unique ECG pattern. In fact, with ST-segment elevation down sloping in the right precordial leads (v1-v3, RBBB pattern in lateral leads and J-point elevation is revealed. We must notice and avoid trigger factors of this syndrome during general anesthesia. Patient is a 39 old man who attended to emergency department with sudden cardiac arrest and resuscitate. He was transferred to ICU for management of hypoxic ischemic encephalopathy. Complementary studies concluded the diagnosis of Brugada syndrome. We must consider Brugada syndrome within patients with family history of sudden cardiac arrest. Moreover, we must avoid trigger factors of this syndrome such as fever, bradicardia and electrolyte abnormality (specialy Na, Ca abnormalities during general anesthesia and if they appear, we should treat them.

  13. Risk of spontaneous pneumothorax due to air travel and diving in patients with Birt-Hogg-Dube syndrome

    NARCIS (Netherlands)

    Johannesma, P.C.; Beek, I. van de; Wel, J.W. van der; Paul, M.A.; Houweling, A.C.; Jonker, M.A.; Waesberghe, J.H. van; Reinhard, R.; Starink, T.M.; Moorselaar, R.J. van; Menko, F.H.; Postmus, P.E.

    2016-01-01

    BACKGROUND AND OBJECTIVES: Birt-Hogg-Dube syndrome is an autosomal dominant disorder characterized by skin fibrofolliculomas, lung cysts, spontaneous pneumothorax and renal cell cancer due to germline folliculin (FLCN) mutations (Menko et al. in Lancet Oncol 10(12):1199-1206, 2009). The aim of this

  14. Aspiration of a cockroach

    Directory of Open Access Journals (Sweden)

    Albert Bousso

    2008-12-01

    Full Text Available It is described a five-month-old infant admitted to our pediatricintensive care unit who aspirated a cockroach. The cockroach wasremoved through bronchoscopy. The child arrived at the emergencyroom in cardiopulmonary arrest and despite full respiratory andcardiovascular support was discharged with evidence of severeneurological sequelae. It is known that this is the first case of acockroach aspiration in a five-month-old infant and reinforces thataspiration of an insect must be considered a possibility in patientswith an unidentified organic foreign body aspiration.

  15. Acute Coronary Syndrome Due to Spontaneous Coronary Artery Dissection in a Middle-Aged Man

    Directory of Open Access Journals (Sweden)

    Davran Cicek

    2014-08-01

    Full Text Available True spontaneous coronary artery dissection (SCAD is an extremely rare but important cause of acute coronary syndrome, with only about 200 cases reported in the literature. Diagnosis is often made at autopsy. Risk factors include oral contraceptive use, atherosclerotic disease and the peripartum period. SCAD should be considered when a healthy young patient presents with the onset of acute myocardial ischemic syndrome. A timely diagnosis and intervention are mandatory as SCAD can cause sudden death. We present a case of SCAD with an uncommon clinical presentation of acute coronary syndrome and without identifiable risk factors, and successfully treated with non-invasive (medical therapy.

  16. Rapid Death Due to Alcohol Withdrawal Syndrome: Case Report and Review of Literature

    Directory of Open Access Journals (Sweden)

    Rahul Mohanrao Band

    2015-03-01

    Full Text Available Background: Alcohol withdrawal syndrome (AWS is one of the most serious complications associated with chronic alcoholism. Sudden deaths are not uncommon in AWS. In severe stages of AWS, delirium tremens (DT occurs, which is characterized with agitation, global confusion, disorientation, visual and auditory hallucinations in addition to autonomic hyperactivity. Case report: A 30-year old man, chronic and heavy alcohol drinker for 10 years, abstained from alcohol for 3 days. Consequently, he started having palpitations, sweating and tremors. A day later, he was found having hallucinations and delirium. The patient was immediately transferred to the hospital. On admission, he was stuporous and had difficulty in breathing. He developed generalized seizures later on. He was successfully intubated, but there was bleeding through it. The patient’s condition deteriorated very rapidly and he died within two hours. After death, his body was transferred to forensic department. In autopsy, gastrointestinal tract was found to be intact. Massive pulmonary hemorrhage was present on cut section. Liver was found to be with yellowish discoloration and early cirrhotic changes. In heart, left ventricular hypertrophy with narrowed lumen was present and coronary arteries were patent. Discussion: Alcoholism is associated with liver dysfunction and especially in final phases with cirrhosis. Hence and due to resultant coagulopathy, patients are vulnerable to internal bleedings. Hypertrophic cardiomyopathy also occurs in chronic alcoholics. Therefore, we can speculate that our patient developed pulmonary hemorrhage as a result of combined effect of coagulopathy secondary to cirrhosis, alveolar damage (seizure and artificial ventilation and congestive heart failure. Conclusion: For a patient with delirium, convulsions, respiratory distress and coagulopathy, diagnosis of DT should be kept in mind.

  17. Dysphagia, dystussia, and aspiration pneumonia in elderly people.

    Science.gov (United States)

    Ebihara, Satoru; Sekiya, Hideki; Miyagi, Midori; Ebihara, Takae; Okazaki, Tatsuma

    2016-03-01

    Despite the development and wide distribution of guidelines for pneumonia, death from pneumonia is increasing due to population aging. Conventionally, aspiration pneumonia was mainly thought to be one of the infectious diseases. However, we have proven that chronic repeated aspiration of a small amount of sterile material can cause the usual type of aspiration pneumonia in mouse lung. Moreover, chronic repeated aspiration of small amounts induced chronic inflammation in both frail elderly people and mouse lung. These observations suggest the need for a paradigm shift of the treatment for pneumonia in the elderly. Since aspiration pneumonia is fundamentally based on dysphagia, we should shift the therapy for aspiration pneumonia from pathogen-oriented therapy to function-oriented therapy. Function-oriented therapy in aspiration pneumonia means therapy focusing on slowing or reversing the functional decline that occurs as part of the aging process, such as "dementia → dysphagia → dystussia → atussia → silent aspiration". Atussia is ultimate dysfunction of cough physiology, and aspiration with atussia is called silent aspiration, which leads to the development of life-threatening aspiration pneumonia. Research pursuing effective strategies to restore function in the elderly is warranted in order to decrease pneumonia deaths in elderly people.

  18. AngioVac Aspiration for Paradoxical Emboli Protection through a Fenestrated Fontan During Central Venous Thrombus Manipulation

    International Nuclear Information System (INIS)

    Al-Hakim, Ramsey; Patel, Komal; Moriarty, John M.

    2015-01-01

    This case reports describes a 39-year-old female with a history of surgically repaired hypoplastic left heart syndrome who presented with a left peripherally inserted central catheter (PICC) with associated large volume subclavian and brachiocephalic vein thrombus. Due to the presence of a right-to-left shunt via a fenestrated Fontan, there was clinical concern for a paradoxical embolism during removal of the PICC. The AngioVac aspiration system was successfully utilized to aspirate thromboemboli from the level of the proximal Glenn shunt during manipulation and removal of the PICC. This is the first reported case to demonstrate the safe and effective use of the AngioVac aspiration system for protection of paradoxical emboli through a cardiac right-to-left shunt during a procedure at high risk for thromboembolism

  19. AngioVac Aspiration for Paradoxical Emboli Protection through a Fenestrated Fontan During Central Venous Thrombus Manipulation

    Energy Technology Data Exchange (ETDEWEB)

    Al-Hakim, Ramsey, E-mail: ralhakim@mednet.ucla.edu [University of California, Department of Radiology, Division of Interventional Radiology (United States); Patel, Komal, E-mail: kdpatel@mednet.ucla.edu [University of California, Department of Anesthesiology, Division of Cardiothoracic Anesthesiology (United States); Moriarty, John M., E-mail: jmoriarty@mednet.ucla.edu [University of California, Department of Radiology, Division of Interventional Radiology (United States)

    2015-06-15

    This case reports describes a 39-year-old female with a history of surgically repaired hypoplastic left heart syndrome who presented with a left peripherally inserted central catheter (PICC) with associated large volume subclavian and brachiocephalic vein thrombus. Due to the presence of a right-to-left shunt via a fenestrated Fontan, there was clinical concern for a paradoxical embolism during removal of the PICC. The AngioVac aspiration system was successfully utilized to aspirate thromboemboli from the level of the proximal Glenn shunt during manipulation and removal of the PICC. This is the first reported case to demonstrate the safe and effective use of the AngioVac aspiration system for protection of paradoxical emboli through a cardiac right-to-left shunt during a procedure at high risk for thromboembolism.

  20. [Children less than 3 months hospitalised due to acute febrile syndrome. 5 years clinical experience].

    Science.gov (United States)

    Méndez Espinola, Benigno Miguel; Herrera Labarca, Patricio

    2015-01-01

    Acute fever of unknown origin (AFUO) is established when the anamnesis and physical examination cannot identify the cause. In infants less than 3 months-old this is situation for concern, due to the risk of a serious bacterial infection. To describe the clinical and laboratory variable of patients with AFUO, in order to look for clues in order to base studies on the decisions arising drom this problem. A report is presented on a retrospective study conducted on a cohort of children less than three months-old admitted to the Hospital Roberto del Río (2007-2011) due to an AFUO. Clinical histories were reviewed and the patients were grouped, according to the severity of the admission diagnosis, into severe and non-severe. They were compared in strata determined by the variables of clinical interest. A total of 550 children were admitted with AFUO during the study period. There was low agreement between the severity on admission and at discharge (kappa=0.079; P=.26). There were 23.8% of children in the severe group and 76.2% in the non-severe group. Urinary tract infection predominated in the severe group (68.7%) and 40.7% with acute febrile syndrome in the non-severe group. The cut-off levels for C-reactive protein, white cells, and neutrophils per mm(3), to calculate the fixed and variable indices, only showed negative predictive values of some use for ruling out serious bacterial infection. The ROC curves with white cell and neutrophil counts and C-reactive protein, did not provide andy fixed indices of clinical use. More than one-third (34.6%) of lumbar punctures were traumatic or failures. According to the results of this study, there is an obvious excess of hospital admissions, little usefulness in the examinations to identify serious bacterial infection, a high percentage lumbar punctures traumatic and lumbar punctures failures, and an excess of antibiotic treatments. A review of clinical criteria and procedures is needed. Copyright © 2015 Sociedad Chilena de

  1. A Case Report About Cluster-Tic Syndrome Due to Venous Compression of the Trigeminal Nerve.

    Science.gov (United States)

    de Coo, Ilse; van Dijk, J Marc C; Metzemaekers, Jan D M; Haan, Joost

    2017-04-01

    The term "cluster-tic syndrome" is used for the rare ipsilateral co-occurrence of attacks of cluster headache and trigeminal neuralgia. Medical treatment should combine treatment for cluster headache and trigeminal neuralgia, but is very often unsatisfactory. Here, we describe a 41-year-old woman diagnosed with cluster-tic syndrome who underwent microvascular decompression of the trigeminal nerve, primarily aimed at the "trigeminal neuralgia" part of her pain syndrome. After venous decompression of the trigeminal nerve both a decrease in trigeminal neuralgia and cluster headache attacks was seen. However, the headache did not disappear completely. Furthermore, she reported a decrease in pain intensity of the remaining cluster headache attacks. This case description suggests that venous vascular decompression in cluster-tic syndrome can be remarkably effective, both for trigeminal neuralgia and cluster headache. © 2016 American Headache Society.

  2. Anton’s Syndrome due to Bilateral Ischemic Occipital Lobe Strokes

    Directory of Open Access Journals (Sweden)

    Sanela Zukić

    2014-01-01

    Full Text Available We present a case of a patient with Anton’s syndrome (i.e., visual anosognosia with confabulations, who developed bilateral occipital lobe infarct. Bilateral occipital brain damage results in blindness, and patients start to confabulate to fill in the missing sensory input. In addition, the patient occasionally becomes agitated and talks to himself, which indicates that, besides Anton’s syndrome, he might have had Charles Bonnet syndrome, characterized by both visual loss and hallucinations. Anton syndrome, is not so frequent condition and is most commonly caused by ischemic stroke. In this particular case, the patient had successive bilateral occipital ischemia as a result of massive stenoses of head and neck arteries.

  3. Clear lens phacoemulsification in the anterior lenticonus due to Alport Syndrome: two case reports

    OpenAIRE

    Aslanzadeh, Ghassem Amir; Gharabaghi, Davoud; Naderi, Niloofar

    2008-01-01

    Abstract Introduction Alport Syndrome has a prevalence of 1 case per 5,000 people and 85% of patients have the X-linked form, where affected males develop renal failure and usually have high-tone sensorineural deafness by age 20. The main abnormality is deficient synthesis of type IV collagen, the main component of basement membranes. Common ocular abnormalities of this syndrome consist of dot-and-fleck retinopathy, posterior polymorphous corneal dystrophy, and anterior lenticonus, but other ...

  4. Lethal Progressive Thoracic Insufficiency in a Neonate Due to Jarcho Levin Syndrome

    Science.gov (United States)

    Bhutia, Euden; Maria, Arti; Verma, Arushi; Sethi, Sidharth Kumar

    2014-01-01

    A rare case of Jarcho Levin syndrome (JLS) presenting as a lethal progressive respiratory insufficiency in early neonatal period is reported. The neonate had classical features of this syndrome including vertebral segmentation defects, typical costo-vertebral fusion defects and scoliosis resulting in small thoracic volume and limited chest expansion; all consistent with a clinical diagnosis of JLS with thoracic insufficiency. In addition, our case had a rare association of dextrocardia and acyanotic congenital heart disease. PMID:24741543

  5. Posterior reversible encephalopathy syndrome (PRES, an acute neurological syndrome due to reversible multifactorial brain edema: a case report

    Directory of Open Access Journals (Sweden)

    Camilla Cicognani

    2013-04-01

    Full Text Available Background: The essential features of Posterior Reversible Encephalopathy Syndrome (PRES are headache, mental changes, seizures, visual symptoms and often arterial hypertension. Brain RMN typically shows cortico-sottocortical parieto-occipital edema, with a bilateral and symmetric distribution. PRES develops in clinical conditions as hypertensive encephalopathy, preeclampsia/ eclampsia, autoimmune diseases, after transplantation, infections and as an adverse effect of immunosuppressive drugs or chemotherapy. It usually completely reverses with treatment, although permanent sequelae are possible in case of delayed or missed diagnosis. Case report: We describe the case of a transsexual (M!F and tetraplegic patient, admitted for neck and low back pain. She suddenly developed headache, confusion, seizures and severe hypertension with normal blood tests. RMN showed multiple cortico-sottocortical areas of vasogenic and citotoxic edema in temporo-occipital, parietal, frontal, and cerebellar regions. Soon after the beginning of the antihypertensive therapy, clinical recovery was observed, as well as the disappearance of edema at RMN. Discussion and conclusions: Although PRES is usually associated with definite pathological conditions, it is not always the case, as was for the patient here described, who had no predisposing factors in her past clinical history, and presented hypertension only in the acute phase of the syndrome. Since, moreover, PRES usually presents with acute non specific features and it can be misdiagnosed with other serious diseases, the clinician will be helped by the knowledge of this syndrome to promptly start diagnostic workup and treatments, and avoid permanent neurological deficits.

  6. Cortical correlates of affective syndrome in dementia due to Alzheimer’s disease

    Directory of Open Access Journals (Sweden)

    Thaís T. Hayata

    2015-07-01

    Full Text Available Neuropsychiatric symptoms in Alzheimer’s disease (AD are prevalent, however their relationship with patterns of cortical atrophy is not fully known. Objectives To compare cortical atrophy’s patterns between AD patients and healthy controls; to verify correlations between neuropsychiatric syndromes and cortical atrophy. Method 33 AD patients were examined by Neuropsychiatric Inventory (NPI. Patients and 29 controls underwent a 3T MRI scanning. We considered four NPI syndromes: affective, apathy, hyperactivity and psychosis. Correlations between structural imaging and neuropsychiatric scores were performed by Freesurfer. Results were significant with a p-value < 0.05, corrected for multiple comparisons. Results Patients exhibited atrophy in entorhinal cortices, left inferior and middle temporal gyri, and precuneus bilaterally. There was correlation between affective syndrome and cortical thickness in right frontal structures, insula and temporal pole. Conclusion Cortical thickness measures revealed atrophy in mild AD. Depression and anxiety symptoms were associated with atrophy of right frontal, temporal and insular cortices.

  7. Further case of Rubinstein-Taybi syndrome due to a deletion in EP300.

    LENUS (Irish Health Repository)

    Foley, Patricia

    2012-02-01

    Rubinstein-Taybi syndrome (RSTS) is a heterogeneous disorder with approximately 45-55% of patients showing mutations in the CREB binding protein and a further 3% of patients having mutations in EP300. We report a male child with a deletion of exons 3-8 of the EP300 gene who has RSTS. He has a milder skeletal phenotype, a finding that has been described in other cases with EP300 mutations. The mother suffered from pre-eclampsia and HELLP syndrome in the pregnancy. She subsequently developed a mullerian tumor of her cervix 6 years after the birth of her son.

  8. Biosynthesis of Various Steroids in vitro by Isolated Adrenal Cells in Primary Aldosteronism, Cushing's Syndrome, and Adrenogenital Syndrome due to Adrenocortical Adenoma

    OpenAIRE

    MIZUNO, SHIGERU; FUNAHASHI, HIROOMI

    1981-01-01

    To a further understanding of the role of steroid hormones in adrenal disorders, we have prepared free cell system of adrenal cells, using adrenal tissues that had been removed by operation from (i) cases of Cushing's syndrome due to adrenocortical adenoma or adrenocortical hyperplasia, (ii) a case of primary aldosteronism, and (iii) a patient with virilizing adrenal tumor. Twelve important steroid hormones were measured, such as pregnenolone, cortisol and aldosterone, which were produced by ...

  9. Antibiotic Therapy in Comatose Mechanically Ventilated Patients Following Aspiration: Differentiating Pneumonia From Pneumonitis.

    Science.gov (United States)

    Lascarrou, Jean Baptiste; Lissonde, Floriane; Le Thuaut, Aurélie; Bachoumas, Konstantinos; Colin, Gwenhael; Henry Lagarrigue, Matthieu; Vinatier, Isabelle; Fiancette, Maud; Lacherade, Jean Claude; Yehia, Aihem; Joret, Aurélie; Lebert, Christine; Bourdon, Sandra; Martin Lefèvre, Laurent; Reignier, Jean

    2017-08-01

    To determine the proportion of patients with documented bacterial aspiration pneumonia among comatose ICU patients with symptoms suggesting either bacterial aspiration pneumonia or non-bacterial aspiration pneumonitis. Prospective observational study. University-affiliated 30-bed ICU. Prospective cohort of 250 patients admitted to the ICU with coma (Glasgow Coma Scale score ≤ 8) and treated with invasive mechanical ventilation. None. The primary outcome was the proportion of patients with microbiologically documented bacterial aspiration pneumonia. Patients meeting predefined criteria for aspiration syndrome routinely underwent telescopic plugged catheter sampling during bronchoscopy before starting probabilistic antibiotic treatment. When cultures were negative, the antibiotic treatment was stopped. Of 250 included patients, 98 (39.2%) had aspiration syndrome, including 92 before mechanical ventilation discontinuation. Telescopic plugged catheter in these 92 patients showed bacterial aspiration pneumonia in 43 patients (46.7%). Among the remaining 49 patients, 16 continued to receive antibiotics, usually for infections other than pneumonia; of the 33 patients whose antibiotics were discontinued, only two subsequently showed signs of lung infection. In the six patients with aspiration syndrome after mechanical ventilation, and therefore without telescopic plugged catheter, antibiotic treatment was continued for 7 days. Mechanical ventilation duration, ICU length of stay, and mortality did not differ between the 43 patients with bacterial aspiration pneumonia and the 49 patients with non-bacterial aspiration pneumonitis. The 152 patients without aspiration syndrome did not receive antibiotics. Among comatose patients receiving mechanical ventilation, those without clinical, laboratory, or radiologic evidence of bacterial aspiration pneumonia did not require antibiotics. In those with suspected bacterial aspiration pneumonia, stopping empirical antibiotic therapy

  10. Spontaneous Thrombosis of a Bicuspid Aortic valve due to Primary Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Sarah Farrell

    2010-08-01

    Full Text Available We present the case of a 51-year-old man who was admitted as an emergency with spontaneous thrombosis of the aortic valve and ascending aorta. At operation he was found to have a congenitally bicuspid aortic valve and subsequent investigation revealed primary antiphospholipid syndrome. He underwent successful removal of the thrombus combined with mechanical replacement of the aortic valve.

  11. Rapunzel syndrome due to ingested hair extensions: Surgical and psychiatric considerations

    Directory of Open Access Journals (Sweden)

    Devin C. Flaherty

    2015-01-01

    Conclusion: This case presents a unique and modern manifestation of Rapunzel syndrome. Surgical treatment most often is required when a patient presents with a massive gastric trichobezoar. Regular post-operative psychiatric follow-up is necessary to prevent recurrent episodes.

  12. A Case Report About Cluster-Tic Syndrome Due to Venous Compression of the Trigeminal Nerve

    NARCIS (Netherlands)

    de Coo, Ilse; van Dijk, J. Marc C.; Metzemaekers, Jan D. M.; Haan, Joost

    Background.-The term "cluster-tic syndrome" is used for the rare ipsilateral co-occurrence of attacks of cluster headache and trigeminal neuralgia. Medical treatment should combine treatment for cluster headache and trigeminal neuralgia, but is very often unsatisfactory. Case.-Here, we describe a

  13. ADULT RESPIRATORY-DISTRESS SYNDROME (ARDS) DUE TO BACTEREMIC PNEUMOCOCCAL PNEUMONIA

    NARCIS (Netherlands)

    MANNES, GPM; BOERSMA, WG; BAUR, CHJM; POSTMUS, PE

    We describe a patient, who had no pre-existing disease, with bacteraemic pneumococcal pneumonia and adult respiratory distress syndrome (ARDS), a rare complication. In spite of the use of antibiotics and intensive treatment the mortality rate of this kind of infection remains high. Streptococcus

  14. Myocardial ischemia due to compression of an unruptured thoracic aortic aneurysm in a patient with Marfan syndrome.

    Science.gov (United States)

    Minami, Hiroya; Asada, Tatsuro; Gan, Kunio; Abe, Koichiro; Izumi, Satoshi

    2007-06-01

    We report a 33-year-old woman who had a 60-mm thoracic aneurysm of the ascending aorta with Marfan syndrome and effort angina due to compression of the right coronary artery (RCA) by the aneurysm. Surgery was performed using the Bentall procedure and a coronary artery bypass graft to the RCA. Postoperatively, coronary angiography showed that the coronary flow of the RCA was restored by removing the aneurysmal compression. The patient was discharged without angina on postoperative day 21.

  15. Recurrent gastrointestinal perforation in a patient with Ehlers-Danlos syndrome due to tenascin-X deficiency.

    Science.gov (United States)

    Sakiyama, Tomo; Kubo, Akiharu; Sasaki, Takashi; Yamada, Taketo; Yabe, Nobushige; Matsumoto, Ken-ichi; Futei, Yuko

    2015-05-01

    Ehlers-Danlos syndrome (EDS) is a clinically and genetically heterogeneous disorder. Using a customized targeted exome-sequencing system we identified nonsense mutations in TNXB in a patient who had recurrent gastrointestinal perforation due to tissue fragility. This case highlights the utility of targeted exome sequencing for the diagnosis of congenital diseases showing genetic heterogeneity, and the importance of attention to gastrointestinal perforation in patients with tenascin-X deficient type EDS. © 2015 Japanese Dermatological Association.

  16. Bone marrow aspiration

    Science.gov (United States)

    Bain, B

    2001-01-01

    Bone marrow aspiration biopsies are carried out principally to permit cytological assessment but also for immunophenotypic, cytogenetic, molecular genetic, and other specialised investigations. Often, a trephine biopsy is carried out as part of the same procedure. Bone marrow aspirations should be carried out by trained individuals who are aware of the indications, contraindications, and hazards of the procedure. They should follow a standard operating procedure. The operator should have made an adequate assessment of clinical and haematological features to ensure both that appropriate indications exist and that all relevant tests are performed. For the patient's comfort and safety, the posterior iliac crest is generally the preferred site of aspiration. Films of aspirated marrow and, when appropriate, films of crushed particles should be made and labelled. Once thoroughly dry, films should be fixed and stained. As a minimum, a Romanowsky stain and a Perls' stain are required. A cover slip should be applied. The bone marrow films should be assessed and reported in a systematic manner so that nothing of importance is overlooked, using a low power, then intermediate, then high power objective. A differential count should be performed. An interpretation of the findings, in the light of the clinical and haematological features, should be given. The report should be signed or computer authorised, using a secure password, and issued in a timely manner. Key Words: bone marrow aspirate • haematological diagnosis PMID:11533068

  17. Hereditary mixed polyposis syndrome due to a BMPR1A mutation.

    LENUS (Irish Health Repository)

    O'Riordan, J M

    2010-06-01

    The conditions Juvenile Polyposis Syndrome (JPS) and Hereditary Mixed Polyposis Syndrome (HMPS) are associated with an increased risk of colorectal carcinoma. The genetic mechanisms which explain these conditions have until recently been poorly understood. Recent interest has focused on the transforming growth factor (TGF)-beta signalling pathway and, in particular, on mutations in the SMAD4 gene. However, not all cases of JPS and HMPS have mutations in SMAD4 and focus has now shifted to other components of the TGF-beta pathway to clarify the genetic mechanisms involved in these conditions. In this report, we describe the significance of a bone morphogenetic protein receptor type 1A gene mutation in an Irish family.

  18. A Turkish family with Nance-Horan Syndrome due to a novel mutation.

    Science.gov (United States)

    Tug, Esra; Dilek, Nihal F; Javadiyan, Shahrbanou; Burdon, Kathryn P; Percin, Ferda E

    2013-08-01

    Nance-Horan Syndrome (NHS) is a rare X-linked syndrome characterized by congenital cataract which leads to profound vision loss, characteristic dysmorphic features and specific dental anomalies. Microcornea, microphthalmia and mild or moderate mental retardation may accompany these features. Heterozygous females often manifest similarly but with less severe features than affected males. We describe two brothers who have the NHS phenotype and their carrier mother who had microcornea but not cataract. We identified a previously unreported frameshift mutation (c.558insA) in exon 1 of the NHS gene in these patients and their mother which is predicted to result in the incorporation of 11 aberrant amino acids prior to a stop codon (p.E186Efs11X). We also discussed genotype-phenotype correlation according to relevant literature. Copyright © 2013 Elsevier B.V. All rights reserved.

  19. Cauda Equina Syndrome Due to Lumbar Disc Herniation: a Review of Literature

    Directory of Open Access Journals (Sweden)

    Kapetanakis Stylianos

    2017-12-01

    Full Text Available Cauda equina syndrome (CES is a rare neurologic condition that is caused by compression of the cauda equina. Cauda equina consists of spinal nerves L2-L5, S1-S5 and the coccygeal nerve. The compression of these nerve roots can be caused mainly by lumbar disc herniation (45% of all causes. The diagnosis consists of two critical points: a detailed history and physical examination and b MRI or CT. The gold standard of the treatment of this syndrome is the surgical approach in combination with the timing of onset of symptoms. The surgery as an emergency situation is recommended in the fi rst 48 hours of onset of symptoms. Any delay in diagnosis and treatment leads to a poor prognosis of CES.

  20. Cauda Equina Syndrome Due to Lumbar Disc Herniation: a Review of Literature.

    Science.gov (United States)

    Kapetanakis, Stylianos; Chaniotakis, Constantinos; Kazakos, Constantinos; Papathanasiou, Jannis V

    2017-12-20

    Cauda equina syndrome (CES) is a rare neurologic condition that is caused by compression of the cauda equina. Cauda equina consists of spinal nerves L2-L5, S1-S5 and the coccygeal nerve. The compression of these nerve roots can be caused mainly by lumbar disc herniation (45% of all causes). The diagnosis consists of two critical points: a) detailed history and physical examination and b) MRI or CT. The gold standard of the treatment of this syndrome is the surgical approach in combination with the timing of onset of symptoms. The surgery as an emergency situation is recommended in the fi rst 48 hours of onset of symptoms. Any delay in diagnosis and treatment leads to a poor prognosis of CES.

  1. The role of nasal CPAP in obstructive sleep apnoea syndrome due to mandibular hypoplasia.

    LENUS (Irish Health Repository)

    Miller, Stanley D W

    2012-02-01

    Melnick Needles syndrome (MNS), Treacher Collins syndrome (TCS) and Pierre Robin syndrome (PRS) are congenital abnormalities with characteristic facial appearances that include micrognathia. A 20-year-old girl with MNS, a 16-year-old boy with TCS and a 12-year-old girl with PRS attended the sleep apnoea clinic at our institution at different times. Diagnostic sleep studies were initially performed on all three patients to confirm the diagnosis of obstructive sleep apnoea syndrome (OSAS). They subsequently commenced nasal CPAP (nCPAP) treatment and their progress was followed. A limited sleep study on the patient with MNS demonstrated moderate\\/severe OSAS with an AHI of 33 events\\/h. Commencement of nCPAP resulted in symptomatic improvement. Overnight oximetry in the patient with TCS showed repeated desaturation to SpO2<90%. Subsequent treatment by nCPAP almost completely abolished the desaturation events. Overnight polysomnography in the patient with PRS demonstrated severe OSAS with an AHI of 49 events\\/h. After 3 years of nCPAP therapy, this patient requested discontinuation of treatment. Subsequent polysomnography without nCPAP revealed an AHI of <5 events\\/h. The use of nCPAP in the patients with MNS and TCS resulted in effective control of their sleep abnormalities. Mandibular growth and enlargement of the posterior airway space led to resolution of OSAS in the patient with PRS. There is a definite role for nCPAP therapy in patients with congenital micrognathia and OSAS. The use of nCPAP may obviate the need for more invasive corrective surgery for OSAS and is not necessarily a life-long requirement.

  2. Immune reconstitution syndrome in a human immunodeficiency virus infected child due to giardiasis leading to shock

    Directory of Open Access Journals (Sweden)

    Sneha Nandy

    2015-01-01

    Full Text Available Human immunodeficiency virus (HIV-associated immune reconstitution inflammatory syndrome has been reported in association with tuberculosis, herpes zoster (shingles, Cryptococcus neoformans, Kaposi′s sarcoma, Pneumocystis pneumonia, hepatitis B virus, hepatitis C virus, herpes simplex virus, Histoplasma capsulatum, human papillomavirus, and Cytomegalovirus. However, it has never been documented with giardiasis. We present a 7-year-old HIV infected girl who developed diarrhea and shock following the initiation of antiretroviral therapy, and her stool showed the presence of giardiasis.

  3. Cushing's syndrome due to pharmacological interaction in a cystic fibrosis patient

    DEFF Research Database (Denmark)

    Main, K M; Skov, M; Sillesen, Ida Blok

    2002-01-01

    and 1,600 microg budesonide. The patient experienced symptoms of striae, moon-face, increased facial hair growth, mood swings, headaches, weight gain, irregular menstruation despite oral contraceptives and increasing insulin requirement for diabetes mellitus. Endocrine investigations revealed total...... and budesonide inhalation should be monitored regularly for adrenal insufficiency. This may be the first indicator of increased systemic exogenous steroid concentration, before clinical signs of Cushing's syndrome emerge....

  4. Episodic tremors representing cortical myoclonus are characteristic in Angelman syndrome due to UBE3A mutations.

    Science.gov (United States)

    Goto, Masahide; Saito, Yoshiaki; Honda, Ryoko; Saito, Takashi; Sugai, Kenji; Matsuda, Yuko; Miyatake, Chiharu; Takeshita, Eri; Ishiyama, Akihiko; Komaki, Hirofumi; Nakagawa, Eiji; Sasaki, Masayuki; Uto, Chieko; Kikuchi, Kenjiro; Motoki, Takahiro; Saitoh, Shinji

    2015-02-01

    Neurological manifestations including psychomotor developmental delay and epilepsy in patients with Angelman syndrome caused by ubiquitin protein ligase E3A (UBE3A) mutations has been considered similar but is relatively milder than that in patients with deletion-type Angelman syndrome. This makes the diagnosis of the former subgroup often difficult. We here characterized epilepsy, specifically the types of tremulous movement, in 4 patients (age, 3-38years) with Angelman syndrome caused by UBE3A mutations. Ictal electroencephalography was used to record episodic tremors in all study patients. Jerk-locked averaging was performed using digital electroencephalography and surface electromyogram data from patients who were monitored for 24h. All patients had tremors in the limbs, head, and trunk, which resulted in 2 patients falling backward. These tremors lasted several seconds, and could emerge in clusters for hours in older patients. In addition, the tremors coincided with 7-8Hz rhythmic activity with a frontocentral predominance, diffuse spike-wave bursts, or no apparent change on electroencephalography. In 2 patients, these tremors were confirmed as cortical myoclonus using jerk-locked averaging. The other seizure types were isolated generalized myoclonus and tonic seizures. None of the patients experienced atypical absence seizures. Levetiracetam therapy was effective in controlling the myoclonic events in 2 of the 3 patients. Semirhythmic myoclonus is common in patients with Angelman syndrome caused by UBE3A mutations, and such myoclonic events are often life disabling. The preserved expression of gamma-aminobutyric acid type A receptor subunit genes located proximal to UBE3A might explain the low prevalence of absence seizures in this population. Copyright © 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  5. A Case of Brown-Vialetto-Van Laere Syndrome Due To a Novel Mutation in Gene

    Directory of Open Access Journals (Sweden)

    Venkatraman Thulasi BA

    2017-08-01

    Full Text Available Brown-Vialetto-Van Laere syndrome is a rare disorder characterized by motor, sensory, and cranial neuronopathies, associated with mutations in SLC52A2 and SLC52A3 genes that code for human riboflavin transporters RFVT2 and RFVT3, respectively. The authors describe the clinical course of a 6-year-old girl with Brown-Vialetto-Van Laere syndrome and a novel homozygous mutation c.1156T>C in the SLC52A3 gene, who presented at the age of 2.5 years with progressive brain stem dysfunction including ptosis, facial weakness, hearing loss, dysphagia, anarthria with bilateral vocal cord paralysis, and ataxic gait. She subsequently developed respiratory failure requiring tracheostomy and worsening dysphagia necessitating a gastrostomy. Following riboflavin supplementation, resolution of facial diplegia and ataxia, improvements in ptosis, and bulbar function including vocalization and respiration were noted. However, her sensorineural hearing loss remained unchanged. Similar to other cases of Brown-Vialetto-Van Laere syndrome, our patient responded favorably to early riboflavin supplementation with significant but not complete neurologic recovery.

  6. Disorder in the serotonergic system due to tryptophan hydroxylation impairment: a cause of hypothalamic syndrome?

    Science.gov (United States)

    Schott, D A; Nicolai, J; de Vries, J E; Keularts, I M L W; Rubio-Gozalbo, M E; Gerver, W J M

    2010-01-01

    The hypothalamus regulates basic homeostasis such as appetite, circadian rhythm, autonomic and pituitary functions. Dysregulation in these functions results in the hypothalamic syndrome, a rare disorder of various origins. Since serotonin (5-HT) modulates most of the above-mentioned homeostasis, a defect in the serotonergic system can possibly participate in this syndrome. We describe a girl suffering from hypothalamic syndrome with a decreased concentration of 5-hydroxytryptophan (5-HTP) and a normal level of tryptophan in the cerebrospinal fluid (CSF) suggesting a functional defect in tryptophan hydroxylase (TPH). TPH is a rate-limiting enzyme in the synthesis of the neurotransmitter 5-HT. Therapeutic intervention with 5-HTP, carbidopa and a specific serotonin reuptake inhibitor significantly improved her clinical symptoms and caused biochemical normalisation of neurotransmitters. The girl described had the typical symptoms of a hypothalamic disorder and a defective serotonergic metabolism, a relationship which has not been reported before. Therapeutic interventions to restore 5-HT metabolism resulted in clinical improvement. We suggest that investigation of 5-HT metabolism in CSF of patients with this rare disorder is included in the aetiological work-up.

  7. DRESS syndrome due to antibiotic therapy of osteoarticular infections in children: two case reports.

    Science.gov (United States)

    Ramírez, A; Abril, J C; Cano, J

    2015-01-01

    Osteoarticular infection in children frequently occurs before 10 years of age. Surgical drainage is sometimes required, whereas acute osteomyelitis can be treated with antibiotic therapy alone. The duration of antibiotic therapy varies, 2 weeks is sufficient for septic arthritis, whereas 6 weeks is often required for complicated cases. Some of these antibiotic drugs present direct complications with low clinical impact in certain individuals. Hypersensitivity to these drugs causes different reactions in children. DRESS syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) is a severe and potentially life-threatening drug reaction. It is characterised by high fever, malaise, lymphadenopathy and skin rash. From a clinical perspective, these symptoms can lead to an exacerbation of the initial infectious process for which treatment was commenced. The liver is the organ most often affected in DRESS syndrome associated with haematological changes, potentially similar to sepsis. We present two cases of children with osteoarticular infections who developed DRESS syndrome after antibiotic therapy. Both patients made a complete recovery after cessation of the antibiotic drugs used. Copyright © 2014 SECOT. Published by Elsevier Espana. All rights reserved.

  8. Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome.

    Science.gov (United States)

    Moreira, D C; Ng, C J; Quinones, R; Liang, X; Chung, D W; Di Paola, J

    2016-12-01

    Essentials Idiopathic systemic capillary leak syndrome (SCLS) is characterized by episodes of vascular leakage. We present the case of a patient with SCLS who developed microangiopathic hemolytic anemia (MAHA). We propose that this anemia is the result of ADAMTS-13 loss in the third-space fluid. This suggests that MAHA can occur in patients with significant extravasation of proteins. Idiopathic systemic capillary leak syndrome (SCLS) is a rare process characterized by acute and recurrent episodes of vascular leakage with severe hypotension, hypoalbuminemia, hemoconcentration and edema. Anemia and thrombocytopenia are not part of this syndrome, but here we present the case of a pediatric patient with a clinical presentation consistent with SCLS who subsequently developed microangiopathic hemolytic anemia at a time when she had significant fluid loss and anasarca. Based on serial ADAMTS-13 levels, we propose that the anemia in this patient developed as a result of ADAMTS-13 loss in the third-space fluid, a novel mechanism for acquired microangiopathic hemolytic anemia. © 2016 International Society on Thrombosis and Haemostasis.

  9. Exogenous Cushing's syndrome due to topical corticosteroid application: case report and review literature.

    Science.gov (United States)

    Tempark, Therdpong; Phatarakijnirund, Voraluk; Chatproedprai, Susheera; Watcharasindhu, Suttipong; Supornsilchai, Vichit; Wananukul, Siriwan

    2010-12-01

    Prolonged use of topical corticosteroids causes systemic adverse effects including Cushing's syndrome and hypothalamic-pituitary-adrenal (HPA) axis suppression, which is less common than that of the oral or parenteral route. At least 43 cases with iatrogenic Cushing syndrome from very potent topical steroid usage (Clobetasol) in children and adult have been published over the last 35 years particularly in developing countries. In children group (n = 22), most are infants with diaper dermatitis and two cases who had started topical application at a very early age and died from severe disseminated CMV infection. For the adult group (n = 21), the most common purpose of steroid use was for treatment of Psoriasis. The recovery period of HPA axis suppression was 3.49 ± 2.92 and 3.84 ± 2.51 months in children and adult, respectively. We report on an 8-month-old female infant who developed Cushing's syndrome and adrenal insufficiency after diaper dermatitis treatment through the misuse of Clobetasol without doctor's prescription. Physiologic dose of hydrocortisone was prescribed to prevent an adrenal crisis for 3 months and discontinued when HPA axis recovery was confirmed by normal morning cortisol and ACTH levels.

  10. Cushing's syndrome due to pharmacological interaction in a cystic fibrosis patient.

    Science.gov (United States)

    Main, K M; Skov, M; Sillesen, I B; Dige-Petersen, H; Müller, J; Koch, C; Lanng, S

    2002-01-01

    Treatment of allergic bronchopulmonary aspergillosis with itraconazole is becoming more widespread in chronic lung diseases. A considerable number of patients is concomitantly treated with topical or systemic glucocorticoids for anti-inflammatory effect. As azole compounds inhibit cytochrome P450 enzymes such as CYP3A isoforms, they may compromise the metabolic clearance of glucocorticoids, thereby causing serious adverse effects. A patient with cystic fibrosis is reported who developed iatrogenic Cushing's syndrome after long-term treatment with daily doses of 800 mg itraconazole and 1,600 microg budesonide. The patient experienced symptoms of striae, moon-face, increased facial hair growth, mood swings, headaches, weight gain, irregular menstruation despite oral contraceptives and increasing insulin requirement for diabetes mellitus. Endocrine investigations revealed total suppression of spontaneous and stimulated plasma cortisol and adrenocorticotropin. Discontinuation of both drugs led to an improvement in clinical symptoms and recovery of the pituitary-adrenal axis after 3 mo. This observation suggests that the metabolic clearance of buDesonide was compromised by itraconazole's inhibition of cytochrome P450 enzymes, especially the CYP3A isoforms, causing an elevation in systemic budesonide concentration. This provoked a complete suppression of the endogenous adrenal function, as well as iatrogenic Cushing's syndrome. Patients on combination therapy of itraconazole and budesonide inhalation should be monitored regularly for adrenal insufficiency. This may be the first indicator of increased systemic exogenous steroid concentration, before clinical signs of Cushing's syndrome emerge.

  11. Angelman Syndrome due to familial translocation: unexpected additional results characterized by Microarray-based Comparative Genomic Hybridization.

    Science.gov (United States)

    Yokoyama-Rebollar, Emiy; Ruiz-Herrera, Adriana; Lieberman-Hernández, Esther; Del Castillo-Ruiz, Victoria; Sánchez-Sandoval, Silvia; Ávila-Flores, Silvia M; Castrillo, José Luis

    2015-01-01

    The 15q11q13 region is subject to imprinting and is involved in various structural rearrangements. Less than 1% of Angelman Syndrome patients are due to translocations involving 15q11q13. These translocations can arise de novo or result from the segregation of chromosomes involved in a familial balanced translocation. A 5-year-old Mexican girl presented with developmental delay, minor dysmorphic features and history of exotropia. G-banding chromosome analysis established the diagnosis of Angelman Syndrome resulting from a familial translocation t(10;15) involving the 15q11.2 region. The available family members were studied using banding and molecular cytogenetic techniques, including Microarray-based Comparative Genomic Hybridization, which revealed additional unexpected results: a coincidental and smaller 15q deletion, asymptomatic duplications in 15q11.2 and Xp22.31 regions. This report demonstrates the usefulness of array CGH for a detailed characterization of familial translocations, including the detection of submicroscopic copy number variations, which would otherwise be missed by karyotype analysis alone. Our report also expands two molecularly characterized rare patient cohorts: Angelman Syndrome patients due to familial translocations and patients with 15q11.2 duplications of paternal origin.

  12. Small intestine aspirate and culture

    Science.gov (United States)

    ... ency/article/003731.htm Small intestine aspirate and culture To use the sharing features on this page, please enable JavaScript. Small intestine aspirate and culture is a lab test to check for infection ...

  13. Rising Aspirations Dampen Satisfaction

    Science.gov (United States)

    Clark, Andrew E.; Kamesaka, Akiko; Tamura, Teruyuki

    2015-01-01

    It is commonly believed that education is a good thing for individuals. Yet, its correlation with subjective well-being is most often only weakly positive, or even negative, despite the many associated better individual-level outcomes. We here square the circle using novel Japanese data on happiness aspirations. If reported happiness comes from a…

  14. Haemolytic-uremic syndrome due to infection with adenovirus: A case report and literature review.

    Science.gov (United States)

    Birlutiu, Victoria; Birlutiu, Rares Mircea

    2018-02-01

    Haemolytic-uremic syndrome is a rare but serious complication of bacterial and viral infections, which is characterized by the triad of: acute renal failure, microangiopathic haemolytic anemia and thrombocytopenia, sometimes severe, requiring peritoneal dialysis. In Europe, hemolytic-uremic syndrome (HUS) in paediatric pathology is primarily caused by Shiga toxin-producing Escherichia coli (STEC) O157, followed by O26. Beside these etiologies, there are other bacterial and viral infections, and also noninfectious ones that have been associated to lead to HUS as well: in the progression of neoplasia, medication-related, post-transplantation, during pregnancy or associated with the antiphospholipid syndrome, systemic lupus erythematosus or family causes with autosomal dominant or recessive inheritance. In terms of pathogenesis, HUS is the result of endothelial injury, most commonly being a result of the action of Shiga toxin. The unfavorable prognosis factors being represented by the age of more than 5 years old, different etiologies from STEC, persistent oligoanuria, central nervous system and glomerular impairment, the association of fever with leukocytosis. HUS is responsible for 7% of cases of hypertension in infants, and an important cause of significant kidney damage in adults. We present one case of HUS caused by adenovirus in a boy of 1 year and 7 months old with severe evolution, which required peritoneal dialysis. Stool sample repeated examination for adenovirus antigen was positive in 2 samples. During hospitalization, the patient required 8 peritoneal dialysis sessions. The renal function was corrected on discharge, the patient required cardiovascular monitoring 1 month after discharge. Although the most common cause that leads to HUS remains STEC, other etiologies like viral ones that may be responsible for severe enteric infection with progression into HUS should not be neglected.

  15. Thoracic Outlet Syndrome in a Volleyball Player Due to Nonunion of the First Rib Fracture.

    Science.gov (United States)

    Puttmann, Kathleen T; Satiani, Bhagwan; Vaccaro, Patrick

    2016-11-01

    Fracture of the first rib with ensuing callus formation is a rare cause of thoracic outlet syndrome. We report a case of a 17-year-old female volleyball player who presented with months of chronic arm pain. Radiographic imaging demonstrated nonunion fracture of the first rib. Physical therapy had been unsuccessful in relieving the pain, and surgical management was performed with resection of the first rib through a transaxillary approach with complete resolution of symptoms. Inflammation surrounding such fractures may destroy tissue planes, making dissection more technically difficult.

  16. Adult-onset opsoclonus-myoclonus syndrome due to West Nile Virus treated with intravenous immunoglobulin.

    Science.gov (United States)

    Hébert, Julien; Armstrong, David; Daneman, Nick; Jain, Jennifer Deborah; Perry, James

    2017-02-01

    A 63-year-old female with no significant past medical history was presented with a 5-day history of progressive opsoclonus-myoclonus, headaches, and fevers. Her workup was significant only for positive West-Nile Virus serum serologies. She received a 2-day course of intravenous immunoglobulin (IvIG). At an 8-week follow up, she had a complete neurological remission. Adult-onset opsoclonus-myoclonus syndrome is a rare condition for which paraneoplastic and infectious causes have been attributed. To our knowledge, this is the first case reported of opsoclonus-myoclonus secondary to West-Nile Virus treated with intravenous immunoglobulin monotherapy.

  17. Novas opções terapêuticas na síndrome de aspiração de mecônio New therapeutic options in meconium aspiration syndrome

    Directory of Open Access Journals (Sweden)

    Mauricio Obal Colvero

    2006-01-01

    Full Text Available OBJETIVOS: revisar a literatura sobre a síndrome de aspiração de mecônio (SAM, enfocando aspectos clínicos, fisiopatológicos e abordagem terapêutica, com destaque ao uso do surfactante e lavado broncoalveolar. MÉTODOS: revisão baseada em artigos publicados na MEDLINE, SCIELO e resumos de congressos internacionais de 1988 a 2004, incluindo ensaios randomizados ou quasi-randomizados, estudos caso-controle e metanálises. RESULTADOS: devido à comprovação da inibição do surfactante na SAM, houve modificações em sua abordagem terapêutica. O manejo atual consiste na aspiração das vias aéreas na sala de parto, seguida de suporte ventilatório necessário para manter a oxigenação arterial adequada, e tratamento das complicações. Tendo em vista a obstrução mecânica do mecônio e seu efeito inibitório sobre o surfactante, a reposição e lavado broncoalveolar com surfactante estão sendo estudados atualmente. CONCLUSÕES: estudos em animais e em recém-nascidos apresentam resultados controversos quanto aos benefícios do uso de surfactante e lavado broncoalveolar na SAM. Torna-se importante a realização de mais estudos para avaliar novas estratégias ventilatórias e se existem vantagens no uso do surfactante e lavado broncoalveolar com surfactante na SAM.OBJECTIVES: to review the literature on meconium aspiration syndrome (MAS focusing on clinical aspects, pathophysiology, and treatment with emphasis on surfactant and bronchoalveolar lavage. METHODS: review including articles from MEDLINE, SCIELO and abstracts published in the national and international literature, from 1988 to 2004 using the keywords meconium aspiration syndrome, surfactant and bronchoalveolar lavage. Randomized and quasi-randomized trials, case control studies, meta-analyses and recently published reviews were selected. Other articles were included for their valuable contribution to the subject. RESULTS: the discovery of new pathophysiological mechanisms

  18. Religious orientation and life aspirations.

    Science.gov (United States)

    Steffen, Patrick R; Clayton, Spencer; Swinyard, William

    2015-04-01

    The effects of religiosity on well-being appear to depend on religious orientation, with intrinsic orientation being related to positive outcomes and extrinsic orientation being related to neutral or negative outcomes. It is not clear, however, why intrinsic and extrinsic religiosity has the relationships they do. Self-determination theory may provide a useful framework of intrinsic and extrinsic aspirations that may help to answer this question. The purpose of the present study was to examine whether intrinsic and extrinsic religiosity would be related to intrinsic and extrinsic life aspirations. We hypothesized that intrinsic religiosity would be positively related to intrinsic life aspirations and negatively related with extrinsic life aspirations, and that extrinsic religiosity would be positively related to extrinsic life aspirations and negatively related to intrinsic aspirations, and that life aspirations would partially mediate the relationships between religious orientation and outcome. To study these hypotheses, a random national sample (total number of 425, average age of 52, 59 % female) completed the measures of religious orientation, life aspirations, affect, and life satisfaction. It was found that intrinsic religiosity was positively related to positive affect, life satisfaction, and intrinsic life aspirations and was negatively related to negative affect and extrinsic life aspirations. Extrinsic religiosity was positively related to extrinsic life aspirations and was not related to the intrinsic life aspirations. When both religious orientation and life aspiration variables were included together in the model predicting outcome, both remained significant indicating that religious orientation and life aspirations are independent predictors of outcome. In conclusion, although religious orientation and life aspirations are significantly related to each other and to outcome, life aspirations did not mediate the effects of religious orientation

  19. Educational Aspirations: Individual Freedom or Social Injustice?

    Science.gov (United States)

    Deosaran, Ramesh A.

    1977-01-01

    The author examines the concepts of aspiration level (what a student would like to do) and expectation level (more "realistic" choices of action or goal) and the gap that exists between them, a gap primarily due to social class and gender. (MJB)

  20. Burning Mouth Syndrome due to Television Moans, an Enigma for Oral Physician: Treatment with Counseling

    Directory of Open Access Journals (Sweden)

    Deepak Gupta

    2014-06-01

    Full Text Available Burning mouth syndrome (BMS is a relatively common disease that can severely affect the quality of life of the patient. It causes chronic orofacial pain or oral burning sensation even in the absence of any detectable organic cause. The etiology of BMS is complex and multifactorial. It has been associated with menopause, trigger events and even genetic polymorphisms. Although its etiology remains unclear, there is still much evidence that psychological elements like stress, anxiety or depression do play a significant role. There are several studies in the literature which only report the association of BMS with psychological factors. But to the best of our knowledge, there is no such case reported in the literature which has actually highlighted the management of such a case with psychogenic elements involved. In this case report, apart from discussing the role of psychological factors, the treatment of BMS with emphasis on counseling is also emphasized. Further, it is of interest to know that such patients with psychologically induced burning mouth syndrome have to be evaluated to their deepest details. Even their commonly overlooked gestures and habits like watching a particular television soap opera may be involved in their disease process. It can be concluded that psychological counseling in general dental practice can provide an effective cure for chronic oral burning sensation with psychological factors involved.

  1. Hypertensive crisis in pregnancy due to a metamorphosing pheochromocytoma with postdelivery Cushing's syndrome.

    Science.gov (United States)

    Langton, Katharina; Gruber, Matthias; Masjkur, Jimmy; Steenblock, Charlotte; Peitzsch, Mirko; Meinel, Jörn; Lenders, Jacques; Bornstein, Stefan; Eisenhofer, Graeme

    2018-01-01

    Pheochromocytomas in pregnancy are rare but potentially lethal. Even rarer is the combination of pheochromocytoma in pregnancy with subsequent development of ectopic Cushing's syndrome. We report a 36-year-old woman, previously diagnosed with essential hypertension, who developed severe hypertension in pregnancy complicated by insulin-dependent gestational diabetes. A cesarean section was performed at 32 weeks following a hypertensive crisis after routine administration of betamethasone. Postnatal persistence of signs and symptoms of catecholamine excess led to the diagnosis of a left adrenal pheochromocytoma. Between diagnosis and planned tumor removal, the patient developed signs and symptoms of Cushing's syndrome (facial edema and hirsutism, myopathy and fatigue). Biochemical testing confirmed hypercortisolism with extremely elevated levels of plasma adrenocorticotropin, urinary cortisol and multiple steroids of a plasma panel that were all normal at previous testing. The previously noradrenergic tumor also started producing epinephrine. Histopathological examination confirmed the pheochromocytoma, which was also immunohistochemically positive for adrenocorticotropin. Full post-surgical recovery was sustained with normal blood pressure and biochemical findings after one year. This report not only underlines the chameleon behavior of pheochromocytoma but also illustrates its potential for a metamorphosing presentation. Corticosteroid administration in pregnancy requires a cautious approach in patients with hypertension.

  2. Fatal Pulmonary Embolism Due to Inherited Thrombophilia Factors in a Child With Wolfram Syndrome.

    Science.gov (United States)

    Küçüktaşçi, Kazim; Semiz, Serap; Balci, Yasemin Işik; Özsari, Tamer; Gürses, Dolunay; Önem, Gökhan; Saçar, Mustafa; Düzcan, Füsun; Yüksel, Doğangün; Semiz, Ender

    2016-10-01

    Wolfram syndrome-1 is a rare and severe autosomal recessive neurodegenerative disease characterized by diabetes mellitus (DM), optic atrophy, diabetes insipidus, and deafness. Poorly controlled type 1 DM increases the risk for thrombosis. However, coexistence of DM and hereditary thrombosis factors is rarely observed. Here we present the case of a 13.5-year-old, nonfollowed girl newly diagnosed with poorly controlled Wolfram syndrome on the basis of the results of clinical and laboratory examinations. On the eighth day after diabetic ketoacidosis treatment, pulmonary embolism developed in the subject. Thrombus identified in the right atrium using echocardiography was treated by emergency thrombectomy. Homozygous mutation in the methylenetetrahydrofolate reductase gene C677T, heterozygous factor-V Leiden mutation, and active protein C resistance were identified in the patient. The patient was lost because of a recurring episode of pulmonary embolism on the 86th day of hospitalization. We present this case to highlight the need for investigating hereditary thrombosis risk factors in diabetic patients in whom thromboembolism develops.

  3. Atypical Angelman syndrome due to a mosaic imprinting defect: Case reports and review of the literature.

    Science.gov (United States)

    Le Fevre, Anna; Beygo, Jasmin; Silveira, Cheryl; Kamien, Benjamin; Clayton-Smith, Jill; Colley, Alison; Buiting, Karin; Dudding-Byth, Tracy

    2017-03-01

    Angelman syndrome (AS) is characterized by severe intellectual disability, limited, or absent speech and a generally happy demeanor. The four known etiological mechanisms; deletions, uniparental disomy, imprinting defects, and UBE3A mutation all affect expression of the UBE3A gene at 15q11-q13. An atypical phenotype is seen in individuals who are mosaic for a chromosome 15q11-q13 imprinting defect on the maternal allele. These patients present with a milder phenotype, often with hyperphagia and obesity or non-specific intellectual disability. Unlike typical AS syndrome, they can have a vocabulary up to 100 words and speak in sentences. Ataxia and seizures may not be present, and the majority of individuals do not have microcephaly. Here we review the current literature and present three individuals with atypical AS caused by a mosaic imprinting defect to demonstrate why DNA methylation analysis at the SNRPN locus needs to be considered in a broader clinical context. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

  4. Thyrotoxicosis followed by Hypothyroidism due to Suppurative Thyroiditis Caused by Nocardia brasiliensis in a Patient with Advanced Acquired Immunodeficiency Syndrome.

    Science.gov (United States)

    Teckie, G2; Bhana, S A; Tsitsi, J M L; Shires, R

    2014-03-01

    Acute thyroiditis is an extremely rare complication of nocardiosis. We report a patient with hyperthyroidism due to suppurative thyroiditis caused by Nocardia brasiliensis. A 38-year-old Black male presented with features of thyrotoxicosis, sepsis and airway obstruction. He had no evidence of underlying thyroid disease, but was severely immunocompromised as a result of acquired immunodeficiency syndrome. He had previously been diagnosed with pulmonary nocardiosis and also had nocardial abscesses on his anterior chest wall. Investigations revealed thyrotoxicosis, with a FT4 of 43.2 pmol/l and a suppressed TSH Nocardia.

  5. Cornelia de Lange syndrome due to mosaic NIPBL mutation: antenatal presentation with sacrococcygeal teratoma.

    Science.gov (United States)

    Banait, Nishant; Fenton, Alan; Splitt, Miranda

    2015-08-14

    A male infant at 36 weeks gestation was born by section. At 20 weeks of gestation, congenital diaphragmatic hernia and sacrococcygeal teratoma had been seen on ultrasound. At birth, the infant had features suggestive of Cornelia de Lange syndrome (CdLS). He remained hypoxic despite aggressive ventilatory manoeuvres and was palliated. At postmortem, the lungs were hypoplastic. In CdLS, mutations in NIPBL are found in around 50% of cases. Mutation analysis, including multiplex ligation dependent probe amplification of the NIPBL gene from the DNA extracted from peripheral blood lymphocytes was negative, but microarray comparative genomic hybridisation on DNA from skin fibroblast showed a 0.13Mb deletion on chromosome 5p13. The deleted region includes exons 42-47 of the NIPBL gene. It is important to perform NIBPL mutation analysis on DNA from more than one tissue when testing for CdLS. 2015 BMJ Publishing Group Ltd.

  6. Angelman syndrome due to a termination codon mutation of the UBE3A gene.

    Science.gov (United States)

    Al-Maawali, Almundher; Machado, Jerry; Fang, Ping; Dupuis, Lucie; Faghfoury, Hannaneh; Mendoza-Londono, Roberto

    2013-03-01

    Angelman syndrome is a neurodevelopmental disorder characterized by global developmental delay, mental retardation, seizures, microcephaly, and severe speech delay. It may be caused by deletion of chromosome region 15q11.2 of the maternally inherited chromosome, mutations in the UBE3A gene, uniparental disomy, or imprinting defects. Most patients with this diagnosis have a severe phenotype, and a few have a mild form of the disease. We report a patient with a novel mutation in the UBE3A gene that consists of a deletion of the termination codon (c.2556-*+6del GTAAAACAAA) and results in an elongated protein E3 ubiquitin-protein ligase. Our patient has a mild phenotype compared with other patients in general and specifically to patients with UBE3A mutations. He has mild developmental delay, moderate speech delay, and no seizures. Recognition of this genotype-phenotype correlation will allow better genetic counseling to other patients with similar stop codon mutations.

  7. Fatal Abdominal Compartment Syndrome Due to Severe Triglyceride-Induced Pancreatitis in Early Pregnancy.

    Science.gov (United States)

    Chibber, Tamanna; Gibson, Paul S

    2017-10-03

    Serum levels of maternal lipids rise physiologically in normal pregnancy, and women with underlying hypertriglyceridemia may experience dramatic elevations which place them at risk for pancreatitis. We describe the case of a woman with severe familial hypertriglyceridemia and prior pancreatitis who discontinued her lipid-lowering therapy early in pregnancy. She promptly developed severe abdominal pain and was hospitalized with acute pancreatitis during the late first trimester. Despite aggressive medical treatment and critical care monitoring, she developed abdominal compartment syndrome (ACS) with associated acute renal failure, which progressed to cardiorespiratory failure and was ultimately fatal. ACS is an alarming complication of acute pancreatitis that has been poorly studied in pregnancy. Copyright © 2017 Society of Obstetricians and Gynaecologists of Canada. Published by Elsevier Inc. All rights reserved.

  8. RENOVASCULAR HYPERTENSION DUE TO RENAL ARTERY STENOSIS IN KLIPPEL-FEIL SYNDROME

    Directory of Open Access Journals (Sweden)

    Foyaca-Sibat H. MD.

    2003-01-01

    Full Text Available ABSTRACT We report one patient with Klippel-Feil (KFS syndrome, other associated anomalies, uncontrolled arterial hypertension, and renal artery stenosis. Because this patient underwent for surgical revascularization with unsuccessful result, all proposed way of treatments are revised, and we have hypothesized that probably for patients with KFS and unilateral renal artery stenosis, medical treatment with ACE inhibitors can provide more benefits than surgical revascularization or percutaneous transluminal angioplasty. We considered that those patients should be manage by a team of medical doctors being aware of their common associated anomalies, identifying all of them when it is possible then, making an integral evaluation of the each individual situation for establishing their medical priorities in order, and then address its treatments accordingly. If at this stage any surgical treatment is required, is important to bring those problems to the anesthesiologist’s attention for a very careful manipulation of the neck and head during induction of anesthesia. The final results will be strongly related with the capacity of management of the underlying cardio-respiratory, renal, skeletal, urogenital, and nervous system problems. . We also propose the term of Klippel-Feil syndrome "Plus" for those patients with cervical vertebral fusion and many other associated deformities rather than to add new eponyms to the long list that already exist. _____________ RESUMEN: HIPERTENSION RENOVASCULAR DEBIDO A ESTENOSIS DE LA ARTERIA RENAL EN EL SÍNDROME DE KLIPPEL-FEIL Reportamos un paciente afectado por un syndrome de Klippel-Feil, otras anormalias congenitas, hipertension arterial incontrolada y una estenosis unilateral de la arteria renal. Este paciente fue sometido a un tratamiento quirÚrgico de la estenosis de la arteria renal, cuyos resultados fueron no satisfactorios por lo que revisamos todas las alternativas de tratamiento para la estenosis

  9. Waardenburg syndrome: a rare cause of inherited neuropathy due to SOX10 mutation.

    Science.gov (United States)

    Bogdanova-Mihaylova, Petya; Alexander, Michael D; Murphy, Raymond P J; Murphy, Sinéad M

    2017-09-01

    Waardenburg syndrome (WS) is a rare disorder comprising sensorineural deafness and pigmentation abnormalities. Four distinct subtypes are defined based on the presence or absence of additional symptoms. Mutations in six genes have been described in WS. SOX10 mutations are usually associated with a more severe phenotype of WS with peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, and Hirschsprung disease. Here we report a 32-year-old man with a novel heterozygous missense variant in SOX10 gene, who presented with congenital deafness, Hirschsprung disease, iris heterochromia, foot deformity, and intermediate conduction velocity length-dependent sensorimotor neuropathy. This case highlights that the presence of other non-neuropathic features in a patient with presumed hereditary neuropathy should alert the clinician to possible atypical rare causes. © 2017 Peripheral Nerve Society.

  10. Intracranial hemorrhage due to intracranial hypertension caused by the superior vena cava syndrome

    DEFF Research Database (Denmark)

    Bartek, Jiri; Abedi-Valugerdi, Golbarg; Liska, Jan

    2013-01-01

    We report a patient with intracranial hemorrhage secondary to venous hypertension as a result of a giant aortic pseudoaneurysm that compressed the superior vena cava and caused obstruction of the venous return from the brain. To our knowledge, this is the first patient reported to have an intracr......We report a patient with intracranial hemorrhage secondary to venous hypertension as a result of a giant aortic pseudoaneurysm that compressed the superior vena cava and caused obstruction of the venous return from the brain. To our knowledge, this is the first patient reported to have...... an intracranial hemorrhage secondary to a superior vena cava syndrome. The condition appears to be caused by a reversible transient rise in intracranial pressure, as a result of compression of the venous return from the brain. Treatment consisted of surgery for the aortic pseudoaneurysm, which led...... to normalization of the intracranial pressure and resorption of the intracranial hemorrhage....

  11. Preventing Cushing: Iatrogenic Cushing Syndrome due to Ritonavir-Fluticasone Interaction.

    Science.gov (United States)

    Tiruneh, Fasil; Awan, Ahmad; Didana, Abiot; Doshi, Saumil

    2017-07-17

    Ritonavir is commonly used in low doses to boost plasma levels of protease inhibitors in patients with human immunodeficiency virus (HIV) infections. It is also a potent inhibitor of cytochrome P450. We present a 50-year-old African American male with past medical history of HIV on highly active antiretroviral therapy (HAART), which also included ritonavir and long standing asthma that has been treated with inhaled fluticasone, who presented with back pain. He had central obesity, prominent abdominal striae and wasted extremities on physical examination. Laboratory tests showed low morning serum cortisol and suboptimal cosyntropin test consistent with adrenal insufficiency. Computed tomography (CT) of the spine showed a fracture of inferior endplate of the lumbar (L3) vertebra. The cause of osteoporosis is believed to be iatrogenic Cushing syndrome caused by enhanced levels of inhaled fluticasone effects secondary to inhibition of cytochrome P450. The patient was managed surgically and fluticasone was discontinued.

  12. Subarachnoid hemorrhage due to ruptured intracranial aneurysm following posterior reversible encephalopathy syndrome

    Directory of Open Access Journals (Sweden)

    Takamasa Nanba

    2016-01-01

    Full Text Available Although posterior reversible encephalopathy syndrome (PRES is rarely associated with subarachnoid hemorrhage, to our knowledge, rupture of a concomitant cerebral aneurysm following PRES has not been reported. We describe a patient with atypical PRES involving the brainstem, thalamus, and periventricular white matter without cortical or subcortical edema of the parietooccipital lobe on magnetic resonance imaging, with rupture of a concomitant cerebral aneurysm. Preexisting extremely high blood pressure may trigger atypical PRES, and failure to lower blood pressure may lead to a concomitant aneurysm rupture. In the future treatment of hypertensive urgency with a recurrence of symptoms and mean arterial blood pressure >150 mmHg, it is advisable to immediately hospitalize the patient for aggressive blood pressure management, especially if PRES is suspected based on clinical and radiological features.

  13. CSR as Aspirational Talk

    DEFF Research Database (Denmark)

    Christensen, Lars Thøger; Morsing, Mette; Thyssen, Ole

    2013-01-01

    Most writings on corporate social responsibility (CSR) treat lack of consistency between organizational CSR talk and action as a serious problem that needs to be eliminated. In this article, we argue that differences between words and action are not necessarily a bad thing and that such discrepan......Most writings on corporate social responsibility (CSR) treat lack of consistency between organizational CSR talk and action as a serious problem that needs to be eliminated. In this article, we argue that differences between words and action are not necessarily a bad thing...... and that such discrepancies have the potential to stimulate CSR improvements. We draw on a research tradition that regards communication as performative to challenge the conventional assumption that CSR communication is essentially superficial, as opposed to CSR action. In addition, we extend notions of organizational...... hypocrisy to argue that aspirational CSR talk may be an important resource for social change, even when organizations do not fully live up to their aspirations....

  14. Aspiration of Barium Contrast

    Directory of Open Access Journals (Sweden)

    Cristina Fuentes Santos

    2014-01-01

    Full Text Available The aspiration of barium contrast is a rare complication that may occur during studies of the digestive tract. Barium is an inert material that can cause anywhere from an asymptomatic mechanical obstruction to serious symptoms of respiratory distress that can result in patient death. We present the case of a 79-year-old male patient in whom we observed the presence of contrast medium residue in the lung parenchyma as an incidental finding during hospitalization. When the patient’s medical file was reviewed, images were found of a barium swallow study that the patient had undergone months earlier, and we were able to observe the exact moment of the aspiration of the contrast material. The patient had been asymptomatic since the test.

  15. Aspiration of Barium Contrast

    OpenAIRE

    Fuentes Santos, Cristina; Steen, Bárbara

    2014-01-01

    The aspiration of barium contrast is a rare complication that may occur during studies of the digestive tract. Barium is an inert material that can cause anywhere from an asymptomatic mechanical obstruction to serious symptoms of respiratory distress that can result in patient death. We present the case of a 79-year-old male patient in whom we observed the presence of contrast medium residue in the lung parenchyma as an incidental finding during hospitalization. When the patient’s medical fil...

  16. An infant with hyperalertness, hyperkinesis, and failure to thrive: a rare diencephalic syndrome due to hypothalamic anaplastic astrocytoma.

    Science.gov (United States)

    Stival, Alessia; Lucchesi, Maurizio; Farina, Silvia; Buccoliero, Anna Maria; Castiglione, Francesca; Genitori, Lorenzo; de Martino, Maurizio; Sardi, Iacopo

    2015-09-04

    Diencephalic Syndrome is a rare clinical condition of failure to thrive despite a normal caloric intake, hyperalertness, hyperkinesis, and euphoria usually associated with low-grade hypothalamic astrocytomas. We reported an unusual case of diencephalic cachexia due to hypothalamic anaplastic astrocytoma (WHO-grade III). Baseline endocrine function evaluation was performed in this patient before surgery. After histological diagnosis, he enrolled to a chemotherapy program with sequential high-dose chemotherapy followed by hematopoietic stem cell rescue. The last MRI evaluation showed a good response. The patient is still alive with good visual function 21 months after starting chemotherapy. Diencephalic cachexia can rarely be due to high-grade hypothalamic astrocytoma. We suggest that a nutritional support with chemotherapy given to high doses without radiotherapy could be an effective strategy for treatment of a poor-prognosis disease.

  17. Corticobasal syndrome due to sporadic Creutzfeldt-Jakob disease: a review and neuropsychological case report.

    Science.gov (United States)

    González, David Andrés; Soble, Jason R

    2017-04-01

    Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, and fatal neurodegenerative disease with neuropsychological sequelae. This study highlighted a rare presentation of CJD (e.g. corticobasal syndrome [CBS]), reviewed updated diagnostic criteria and procedures for CJD (e.g. diffusion weighted imaging [DWI], real-time quaking-induced conversion [RT-QuIC]), and discussed differential diagnoses. Case report methodology focused on a 68-year-old, Hispanic, right-handed man with 11 years of education. He presented with a 1-2-month history of gait and motor difficulties (e.g. rigidity, myoclonus). After evaluation, a 'cortical ribboning' pattern on DWI and positive RT-QuIC was integrated with performance on neurobehavioral exam (i.e. alien limb phenomenon, unilateral ideomotor apraxia) and neuropsychological testing (i.e. frontal-parietal dysfunction pattern) to reach a diagnosis of sCJD-CBS. The patient expired 3 months after onset of symptoms. This literature review and case report highlighted the importance of staying abreast of developments in neurological literature and the added value of neuropsychology, when integrated with newer procedures, for confirming and excluding diagnostic considerations.

  18. Occult Breast Cancer due to Multiple Calcified Hamartomas in a Patient with Cowden Syndrome

    Directory of Open Access Journals (Sweden)

    E. B. Gómez García

    2012-01-01

    Full Text Available Cowden syndrome (CS is an autosomal dominant disorder characterized by presence of multiple hamartomas, and other benign and malignant abnormalities of the breasts, skin, thyroid, endometrium, gastrointestinal tract, and central nervous system. Hamartomas are benign, developmentally disorganized tumors that can develop in any of the above mentioned organs. The presence of massive calcifications in the breasts in very young women is an indication to perform a breast MRI to exclude a neoplasm since, like in the current case report, presence of breast calcifications may obscure a neoplasm. Although fibrocystic disease and cooccurrence of fibrocystic disease and breast cancer are much more common than CS, the presence of massive calcifications in the breasts of very young women should elicit the possibility of an underlying genetic disease. Furthermore, breast cancer and macrocephaly are considered major criteria for the diagnosis of CS and the combination of both is enough to establish the clinical diagnosis of this entity. Fibrocystic disease of the breasts and multinodular goiter are minor criteria. Family history is also important for the diagnosis of (any hereditary disease.

  19. First reported case of reactive airway dysfunction syndrome in a laborer due to porcelain tile dust.

    Science.gov (United States)

    Arif, Tasleem; Malik, Javid Ahmad; Shoib, Sheikh

    2013-06-01

    Reactive airway dysfunction syndrome (RADS) is a type of non-immunologically mediated asthma-like disease. It usually occurs after a massive exposure to an irritating substance in the atmosphere in the form of smoke, fumes, gases, and vapor. Unlike bronchial asthma, there is no latency to the symptoms seen in RADS. A number of agents are known to cause RADS, but tile dust, as an etiological agent, has not been previously reported. We report a 45-year-old male laborer, who presented with an acute onset of cough, chest tightness, breathlessness, and audible wheeze after his first time exposure to porcelain tile dust within 5 hours of exposure. Lab tests, including, chest X-ray, electrocardiogram, air blood gas analysis, and serum IgE, were unremarkable. Spirometry showed a mild obstruction [forced expiratory volume in 1 second (FEV1)=72% of predicted], while the bronchodilator reversibility test was significant(14% increase in FEV1 above the baseline).Bronchial biopsy revealed a chronic inflammatory reaction with lymphocytic and plasma cell infiltration and more importantly a striking absence of eosinophils. To the best of our knowledge, this is the first reported case of RADS as a result of exposure to tile dust (porcelain ceramics).

  20. Chromoblastomycosis due to Fonsecaea monophora in a man with nephritic syndrome.

    Science.gov (United States)

    Tan, Huan; Xu, Yan; Lan, Xue-Mei; Wu, Ya-Guang; Zhou, Cun-Jian; Yang, Xi-Chuan

    2015-06-01

    Chromoblastomycosis is a chronic subcutaneous mycosis caused by dematiaceous fungi. Fonsecaea monophora, a new species segregated from F. pedrosoi, may be the most prevalent pathogen of chromoblastomycosis in southern China. Herein, we report a rare case of chromoblastomycosis in a man with nephritic syndrome. He presented with an asymptomatic red plaque on the back of his left wrist that had appeared and enlarged over a period of 1.5 years, without any prior trauma. He was initially diagnosed with sporotrichosis. However, he did not respond to a 6-month course of potassium iodide treatment. The lesion slowly enlarged and became verrucous instead. Concurrently, a similar maculopapule appeared on his left forearm. Histopathological examination of a biopsy specimen indicated the presence of sclerotic bodies in the dermis. The fungus was identified as Fonsecaea spp. based on the results of a slide culture; in addition, the agent was confirmed to be F. monophora by using molecular methods. The patient demonstrated marked improvement after receiving appropriate antifungal therapy for 3 months. To our knowledge, this is the first case of chromoblastomycosis caused by F. monophora in an immunosuppressed patient. The identification of the agent by molecular techniques is important for epidemiological purposes. Thus, we believe that combination therapy with itraconazole and terbinafine would be a suitable option for infections caused by F. monophora.

  1. Transfusion-dependent thalassaemic patients with renal Fanconi syndrome due to deferasirox use.

    Science.gov (United States)

    Chuang, Gwo-Tsann; Tsai, I-Jung; Tsau, Yong-Kwei; Lu, Meng-Yao

    2015-12-01

    Deferasirox is a new oral iron chelating agent with several cases reporting renal adverse events in recent years. Our aim was to identify the incidence of deferasirox-related Fanconi syndrome (FS) and its risk factors. All transfusion-dependent thalassaemic patients who received deferasirox at the outpatient department of the National Taiwan University Hospital (NTUH) from January 2006 to February 2014 were evaluated. This cohort study included 57 patients, and mean age of deferasirox initiation was 18.2 ± 7.7 years. After 6.9 ± 1.8 years of follow-up, 5 in 57 (8.8%) thalassaemic patients had FS. Age of starting deferasirox negatively correlated with incidence of FS (correlation coefficient -0.892, P = 0.008). Other factors were not significantly associated with FS. Serum creatinine level at the start of deferasirox compared to at the end of study or onset of FS did not show significant change (P = 0.277). All the deferasirox-related FS manifested with proximal renal tubular acidosis and hypophosphataemia, which needed specific treatment or withdrawal of deferasirox use. We recommend that children, especially of young age, who regularly use deferasirox should undergo routine urinalysis and blood testing for early detection of FS. © 2015 Asian Pacific Society of Nephrology.

  2. Acute renal failure due to abdominal compartment syndrome: report on four cases and literature review

    Directory of Open Access Journals (Sweden)

    Cleva Roberto de

    2001-01-01

    Full Text Available We report on 4 cases of abdominal compartment syndrome complicated by acute renal failure that were promptly reversed by different abdominal decompression methods. Case 1: A 57-year-old obese woman in the post-operative period after giant incisional hernia correction with an intra-abdominal pressure of 24 mm Hg. She was sedated and curarized, and the intra-abdominal pressure fell to 15 mm Hg. Case 2: A 73-year-old woman with acute inflammatory abdomen was undergoing exploratory laparotomy when a hypertensive pneumoperitoneum was noticed. During the surgery, enhancement of urinary output was observed. Case 3: An 18-year-old man who underwent hepatectomy and developed coagulopathy and hepatic bleeding that required abdominal packing, developed oliguria with a transvesical intra-abdominal pressure of 22 mm Hg. During reoperation, the compresses were removed with a prompt improvement in urinary flow. Case 4: A 46-year-old man with hepatic cirrhosis was admitted after incisional hernia repair with intra-abdominal pressure of 16 mm Hg. After paracentesis, the intra-abdominal pressure fell to 11 mm Hg.

  3. Assessing the Clinical Utility of SNP Microarray for Prader-Willi Syndrome due to Uniparental Disomy.

    Science.gov (United States)

    Santoro, Stephanie L; Hashimoto, Sayaka; McKinney, Aimee; Mihalic Mosher, Theresa; Pyatt, Robert; Reshmi, Shalini C; Astbury, Caroline; Hickey, Scott E

    2017-01-01

    Maternal uniparental disomy (UPD) 15 is one of the molecular causes of Prader-Willi syndrome (PWS), a multisystem disorder which presents with neonatal hypotonia and feeding difficulty. Current diagnostic algorithms differ regarding the use of SNP microarray to detect PWS. We retrospectively examined the frequency with which SNP microarray could identify regions of homozygosity (ROH) in patients with PWS. We determined that 7/12 (58%) patients with previously confirmed PWS by methylation analysis and microsatellite-positive UPD studies had ROH (>10 Mb) by SNP microarray. Additional assessment of 5,000 clinical microarrays, performed from 2013 to present, determined that only a single case of ROH for chromosome 15 was not caused by an imprinting disorder or identity by descent. We observed that ROH for chromosome 15 is rarely incidental and strongly associated with hypotonic infants having features of PWS. Although UPD microsatellite studies remain essential to definitively establish the presence of UPD, SNP microarray has important utility in the timely diagnostic algorithm for PWS. © 2017 S. Karger AG, Basel.

  4. Posterior Reversible Encephalopathy Syndrome due to High Dose Corticosteroids for an MS Relapse

    Directory of Open Access Journals (Sweden)

    Sarah A. Morrow

    2015-01-01

    Full Text Available Increased blood pressure is a known adverse effect associated with corticosteroids but little is published regarding the risk with the high doses used in multiple sclerosis (MS. A 53-year-old female with known relapsing remitting MS presented with a new brainstem relapse. Standard of care treatment for an acute MS relapse, 1250 mg of oral prednisone for 5 days, was initiated. She developed an occipital headache and dizziness and felt generally unwell. These symptoms persisted after treatment was complete. On presentation to medical attention, her blood pressure was 199/110 mmHg, although she had no history of hypertension. MRI changes were consistent with posterior reversible encephalopathy syndrome (PRES, demonstrating abnormal T2 signal in both thalami, the posterior occipital and posterior parietal white matter with mild sulcal effacement. As her pressure normalized with medication, her symptoms resolved and the MRI changes improved. No secondary cause of hypertension was found. This is the first reported case of PRES secondary to high dose corticosteroid use for an MS relapse without a history of hypertension and with no other secondary cause of hypertension identified. This rare complication should be considered in MS patients presenting with a headache or other neurological symptoms during treatment for a relapse.

  5. Adolescent polycystic ovary syndrome due to functional ovarian hyperandrogenism persists into adulthood.

    Science.gov (United States)

    Rosenfield, Robert L; Ehrmann, David A; Littlejohn, Elizabeth E

    2015-04-01

    Menstrual irregularity and above-average testosterone levels in adolescence may presage polycystic ovary syndrome (PCOS) in adulthood but persist in only a minority. Prolonged anovulatory cycles in normal adolescents are associated with increased testosterone levels. Thus, questions have been raised about the accuracy of PCOS diagnosed in adolescents. The purpose of this study was to follow-up hyperandrogenic adolescents with features of PCOS to test the hypothesis that adolescent functional ovarian hyperandrogenism (FOH) persists into adulthood. A series of adults previously reported to have adolescent PCOS, with most documented to have FOH by GnRH agonist or dexamethasone androgen-suppression test criteria, were recalled. Recall occurred >3 years after the initial diagnosis and at the age of >18.0 years. Respondents underwent examination, baseline androgen evaluation, and an oral glucose tolerance test after discontinuing oral contraceptive therapy. Of the adolescent hyperandrogenic patients, 68% (15 of 22) were traceable, and 60% of those traced returned for follow-up, including half (n = 8) of the original FOH group. The baseline characteristics of respondents and nonrespondents were not significantly different. Patients with FOH were reevaluated when their mean age was 23.0 years (range, 18.4-29.4 years), gynecologic age was 10.7 years (range, 5.5-18.4 years), and body mass index was 42.3 kg/m(2) (range, 28.3-52.1 kg/m(2); P = .02 vs adolescence). Serum free testosterone was 24 pg/mL (range, 10-38 pg/mL, normal, 3-9 pg/mL; not significant vs adolescence); all were oligomenorrheic. Whereas 3 of 8 had impaired glucose tolerance as adolescents, at follow-up 6 of 8 had developed abnormal glucose tolerance (2 with type 2 diabetes mellitus). Adolescents with FOH, which underlies most PCOS, uniformly have persistent hyperandrogenism, and glucose tolerance tends to deteriorate. Testing ovarian androgenic function in hyperandrogenic adolescents may be of prognostic

  6. Nephrotic syndrome due to minimal change disease secondary to spider bite: clinico-pathological case of a non-described complication of latrodectism.

    Science.gov (United States)

    Méndez, Gonzalo P; Enos, Daniel; Moreira, José Luis; Alvaredo, Fátima; Oddó, David

    2017-04-01

    The patient was an 18-year-old man who developed nephrotic syndrome after a 'wheat spider' bite ( Latrodectus mactans ). Due to this atypical manifestation of latrodectism, a renal biopsy was performed showing minimal change disease. The nephrotic syndrome subsided after 1 week without specific treatment. This self-limited evolution suggests that the mechanism of podocyte damage was temporary and potentially mediated by a secondary mechanism of hypersensitivity or direct effect of the α-latrotoxin. The patient did not show signs of relapse in subsequent checkup. This is the first reported case of nephrotic syndrome due to a minimal change lesion secondary to latrodectism.

  7. Zonisamide eradicated paroxysmal headache with EEG abnormalities triggered by hypertensive encephalopathy due to purpura nephritic syndrome.

    Science.gov (United States)

    Anzai, Yuki; Hayashi, Masaharu; Ohya, Tatsuo

    2006-10-01

    Generally, prognosis of hypertensive encephalopathy in childhood is favorable. We reported a 5-year-old girl who presented with a headache attack and EEG abnormalities after hypertensive encephalopathy due to purpura nephritis. The patient had suffered from hypertensive encephalopathy due to purpura nephritis, which soon ameliorated. Five months later, she developed attacks of headache, vomiting and disturbed consciousness with left side-predominant EEG abnormalities. Although carbamazepine and sodium valproate failed to improve her condition, zonisamide eradicated both the symptoms and EEG abnormalities, and an attack has not reoccurred for 5 years since completion of her treatment. It is noteworthy that delayed-onset complications can occur in child hypertensive encephalopathy, cases of which should be followed up prudently. Zonisamide should be considered for treatment of attacks of headaches with an epileptic character.

  8. Care in post-traumatic syndrome due to gender violence: a case report.

    Science.gov (United States)

    Sánchez-Herrero, Héctor; Duarte-Clíments, Gonzalo; González-Pérez, Teodoro; Sánchez-Gómez, María Begoña; Gomariz-Bolarín, David

    This article describes a clinical case of a patient attended at a continuous care point for a generalized anxiety disorder, principally due to abuse suffered from her ex partner. The patient was followed up at family nursing clinic, and the appropriate nursing interventions were developed to cover a series of needs prioritized by nurses using the AREA method and taking into account the prioritization of the user herself. Copyright © 2017 Elsevier España, S.L.U. All rights reserved.

  9. Assessment of the Personal Losses Suffered by Correctional Officers due to Burnout Syndrome

    OpenAIRE

    RG Stoyanova; SN Harizanova

    2016-01-01

    Background: Professional burnout is defined as a state of depletion and loss of motivation accompanied by different mental and physical symptoms. Objective: To assess personal losses suffered by correctional officers due to burnout. Methods: This cross-sectional study conducted between June and December 2012 included 201 correctional officers in two Bulgarian prisons. The mean age of the whole group was 41.2 (SD 8.0) years. The respondents was mostly male (56.7%), married (72.6%), had...

  10. Síndrome de aspiração 7 do mecônio: análise de resultados obstétricos e perinatais Meconium aspiration syndrome: obstetric and perinatal outcome analysis

    Directory of Open Access Journals (Sweden)

    José Mauro Madi

    2003-03-01

    Full Text Available OBJETIVOS: analisar os resultados obstétricos e perinatais de 26 casos de síndrome de aspiração de mecônio (SAM MÉTODOS: realizou-se revisão dos prontuários de 26 recém-nascidos (RN com diagnóstico de SAM. Os casos foram estudados em função da média de permanência do RN na UTIN e das principais complicações maternas e neonatais, correlacionando-as entre si. RESULTADOS: dezoito conceptos nasceram no HG-FUCS e 8 fora; no período citado, ocorreram 3.976 nascimentos no HG-FUCS, incidência de SAM de 0,45%. Dos 18 casos estudados, 9 nasceram pela via vaginal; o peso ao nascimento foi >2.500 g em dezesseis casos. Mecônio leve ocorreu em 50%, semelhante ao espesso. O Apgar no 1º minuto foi >7 em 3 casos (16,7%, entre 4 e 6, em 7 casos (38,9%, e entre 0 e 3, em 8 casos (44,4%. No 5º minuto, sete RN permaneceram deprimidos. A principal complicação neonatal foi anoxia (36% dos casos. A taxa de óbito neonatal foi de 7,7%. A internação média na UTIN foi de 19,9 dias. CONCLUSÃO: a SAM constitui grave entidade clínica neonatal, relacionando-se com altas taxas de mortalidade neonatal, mecônio espesso em pelo menos a metade dos casos e nascimento de fetos deprimidos na sua maioria.PURPOSE: to analyze obstetrical and perinatal data in 26 cases of meconium aspiration syndrome (MAS. METHODS: a retrospective review was performed in 26 newborn records diagnosed with meconium aspiration syndrome. Patients were studied emphasizing average days in neonatal intensive care unit and main maternal findings and neonatal complications, correlating them with each other. RESULTS: Eighteen babies were delivered at GH-CSUF and eight out of this hospital. At this time 3,976 deliveries occurred at GH-CSUF, with an incidence of MAS of 0.45%. Nine of 18 babies were born by vaginal delivery; weight was >2,500 g in 16 cases. One-minute Apgar score was >7 in three cases (16.7%, between 4 and 6 in seven cases (38.9%, and between 0 and 3, in eight cases (44

  11. Conus medullaris syndrome due to an intradural disc herniation: A case report

    Directory of Open Access Journals (Sweden)

    Chaudhary Kshitij

    2008-01-01

    Full Text Available A 70-year-old male patient developed acute paraplegia due to conus medullaris compression secondary to extrusion of D12-L1 disc. After negative epidural examination intraoperatively, a durotomy was performed and an intradural disc fragment was excised. Patient did not regain ambulatory status at two-year follow-up. Intraoperative finding of negative extradural compression, tense swollen dura and CSF leak from ventral dura should alert the surgeon for the possibility of intradural disc herniation. A routine preoperative MRI is misleading and a high index of suspicion helps to avoid a missed diagnosis.

  12. Acute respiratory distress syndrome in a neonate due to possible transfusion-related acute lung injury

    Directory of Open Access Journals (Sweden)

    Arti Maria

    2017-01-01

    Full Text Available Transfusion-related acute lung injury (TRALI is a potentially life-threatening complication of blood component transfusion. It is relatively underdiagnosed entity in neonates with scant literature. We report a case of TRALI in a preterm neonate developing acute respiratory distress within 6 h of blood product transfusion in the absence of preexisting lung disease. Prompt ventilator and supportive management were instituted. The baby showed clinical and radiological improvement within 12 h; however, he succumbed to death due to acute massive pulmonary hemorrhage 36 h later. Possibility of TRALI should be kept if there is sudden deterioration of lung function after blood transfusion.

  13. Evidence Report: Risk of Acute Radiation Syndromes Due to Solar Particle Events

    Science.gov (United States)

    Carnell, Lisa; Blattnig, Steve; Hu, Shaowen; Huff, Janice; Kim, Myung-Hee; Norman, Ryan; Patel, Zarana; Simonsen, Lisa; Wu, Honglu

    2016-01-01

    Crew health and performance may be impacted by a major solar particle event (SPE), multiple SPEs, or the cumulative effect of galactic cosmic rays (GCR) and SPEs. Beyond low-Earth orbit, the protection of the Earth's magnetosphere is no longer available, such that increased shielding and protective mechanisms are necessary in order to prevent acute radiation sickness and impacts to mission success or crew survival. While operational monitoring and shielding are expected to minimize radiation exposures, there are EVA scenarios outside of low-Earth orbit where the risk of prodromal effects, including nausea, vomiting, anorexia, and fatigue, as well as skin injury and depletion of the blood-forming organs (BFO), may occur. There is a reasonable concern that a compromised immune system due to high skin doses from a SPE or due to synergistic space flight factors (e.g., microgravity) may lead to increased risk to the BFO. The primary data available at present are derived from analyses of medical patients and persons accidentally exposed to acute, high doses of low-linear energy transfer (LET) (or terrestrial) radiation. Data more specific to the space flight environment must be compiled to quantify the magnitude of increase of this risk and to develop appropriate protection strategies. In particular, information addressing the distinct differences between solar proton exposures and terrestrial exposure scenarios, including radiation quality, dose-rate effects, and non-uniform dose distributions, is required for accurate risk estimation.

  14. Fournier's gangrene - a rare complication of hydrocele aspiration

    International Nuclear Information System (INIS)

    Ali, M.Z.

    2004-01-01

    A case of Fournier's gangrene of scrotum with systemic inflammatory response syndrome resulting from hydrocele aspiration in young male patient is presented. He was treated with antibiotics, wide local debridement of scrotal skin with evacuation of pus and gas; followed by serial debridements and dressings. Secondary suturing of scrotal defect was done after 02 weeks. (author)

  15. The Role of Inspiratory Muscle Training in Sickle Cell Anemia Related Pulmonary Damage due to Recurrent Acute Chest Syndrome Attacks

    Directory of Open Access Journals (Sweden)

    Burcu Camcıoğlu

    2015-01-01

    Full Text Available Background. The sickling of red blood cells causes a constellation of musculoskeletal, cardiovascular, and pulmonary manifestations. A 32-year-old gentleman with sickle cell anemia (SCA had been suffering from recurrent acute chest syndrome (ACS. Aim. To examine the effects of inspiratory muscle training (IMT on pulmonary functions, respiratory and peripheral muscle strength, functional exercise capacity, and quality of life in this patient with SCA. Methods. Functional exercise capacity was evaluated using six-minute walk test, respiratory muscle strength using mouth pressure device, hand grip strength using hand-held dynamometer, pain using Visual Analogue Scale, fatigue using Fatigue Severity Scale, dyspnea using Modified Medical Research Council Scale, and health related quality of life using European Organization for Research and Treatment of Cancer QOL measurement. Results. A significant improvement has been demonstrated in respiratory muscle strength, functional exercise capacity, pain, fatigue, dyspnea, and quality of life. There was no admission to emergency department due to acute chest syndrome in the following 12 months after commencing regular erythrocytapheresis. Conclusion. This is the first report demonstrating the beneficial effects of inspiratory muscle training on functional exercise capacity, respiratory muscle strength, pain, fatigue, dyspnea, and quality of life in a patient with recurrent ACS.

  16. The Role of Inspiratory Muscle Training in Sickle Cell Anemia Related Pulmonary Damage due to Recurrent Acute Chest Syndrome Attacks.

    Science.gov (United States)

    Camcıoğlu, Burcu; Boşnak-Güçlü, Meral; Karadallı, Müşerrefe Nur; Akı, Şahika Zeynep; Türköz-Sucak, Gülsan

    2015-01-01

    Background. The sickling of red blood cells causes a constellation of musculoskeletal, cardiovascular, and pulmonary manifestations. A 32-year-old gentleman with sickle cell anemia (SCA) had been suffering from recurrent acute chest syndrome (ACS). Aim. To examine the effects of inspiratory muscle training (IMT) on pulmonary functions, respiratory and peripheral muscle strength, functional exercise capacity, and quality of life in this patient with SCA. Methods. Functional exercise capacity was evaluated using six-minute walk test, respiratory muscle strength using mouth pressure device, hand grip strength using hand-held dynamometer, pain using Visual Analogue Scale, fatigue using Fatigue Severity Scale, dyspnea using Modified Medical Research Council Scale, and health related quality of life using European Organization for Research and Treatment of Cancer QOL measurement. Results. A significant improvement has been demonstrated in respiratory muscle strength, functional exercise capacity, pain, fatigue, dyspnea, and quality of life. There was no admission to emergency department due to acute chest syndrome in the following 12 months after commencing regular erythrocytapheresis. Conclusion. This is the first report demonstrating the beneficial effects of inspiratory muscle training on functional exercise capacity, respiratory muscle strength, pain, fatigue, dyspnea, and quality of life in a patient with recurrent ACS.

  17. Rapid respiratory deterioration and sudden death due to disseminated cryptococcosis in a patient with the acquired immunodeficiency syndrome.

    Science.gov (United States)

    Penmetsa, S; Rose, T A; Crook, E D

    1999-09-01

    We report the case of a patient with the acquired immunodeficiency syndrome (AIDS) whose death occurred within 30 hours of hospitalization due to disseminated cryptococcosis, manifested by dizziness, cough, and shortness of breath. The clinical picture was consistent with pneumocystis pneumonia, and antibiotic therapy with corticosteroids was initiated. Despite initial improvement, the patient's condition quickly worsened, resulting in cardiorespiratory arrest and death. Autopsy revealed cryptococci in several organs. Sudden, rapid deterioration and death are rare consequences of disseminated cryptococcosis, and steroids may worsen the course of the disease. On the basis of this case and review of similar cases in the literature, we recommend early consideration of disseminated cryptococcosis in AIDS patients with pneumonia. Early diagnosis and appropriate therapy are essential to reduce morbidity and mortality.

  18. Anthropometry in Klinefelter syndrome--multifactorial influences due to CAG length, testosterone treatment and possibly intrauterine hypogonadism.

    Science.gov (United States)

    Chang, Simon; Skakkebæk, Anne; Trolle, Christian; Bojesen, Anders; Hertz, Jens Michael; Cohen, Arieh; Hougaard, David Michael; Wallentin, Mikkel; Pedersen, Anders Degn; Østergaard, John Rosendahl; Gravholt, Claus Højbjerg

    2015-03-01

    Klinefelter syndrome, 47, XXY (KS), is underdiagnosed partly due to few clinical signs complicating identification of affected individuals. Certain phenotypic traits are common in KS. However, not all aspects of the KS phenotype are well described. To describe anthropometry and body composition in KS and relate findings to biochemistry and X-chromosome related genetic markers. Seventy three KS males referred to our clinic and 73 age-matched controls underwent comprehensive measurements of anthropometry and body composition in a cross-sectional, case-controlled study. Furthermore, genetic analysis for parental origin of the supernumerary X-chromosome, skewed X-chromosome inactivation and androgen receptor (AR) CAG repeat length was done. Anthropometry and body composition in KS and the effect of genotype hereon. KS males were taller (absolute difference: 5.1 cm, P Anthropometry and body composition in KS is specific and dysmorphic and affected by AR CAG repeat length and decreased exposure to testosterone already during fetal life.

  19. Hemolytic uremic syndrome with mild renal involvement due to Shiga toxin-producing Escherichia coli (STEC O145 strain

    Directory of Open Access Journals (Sweden)

    Lucía Pérez

    Full Text Available Hemolytic uremic syndrome (HUS is a disorder characterized by the presence of the classic triad: microangiopathic hemolytic anemia, thrombocytopenia and acute renal injury. HUS without acute renal failure can be confused with other hematologic diseases. An infantile HUS caused by a Shiga-toxin-producing Escherichia coli (STEC O145 strain carrying genotype stx2, ehxA, eae subtype ?1 is herein reported. The infant did not require dialysis during the acute stage of HUS, evolved favorably, maintained normal blood pressure and normal renal function and had no recurrence until the last control. This could be due to several factors, such as the characteristics of infecting STEC strain and a reduction in host susceptibility to renal injury. This report highlights the regional participation of non-O157 STEC in childhood diseases and the importance of performing active surveillance for all forms of HUS.

  20. A synonymous mutation in TCOF1 causes Treacher Collins syndrome due to mis-splicing of a constitutive exon.

    Science.gov (United States)

    Macaya, D; Katsanis, S H; Hefferon, T W; Audlin, S; Mendelsohn, N J; Roggenbuck, J; Cutting, G R

    2009-08-01

    Interpretation of the pathogenicity of sequence alterations in disease-associated genes is challenging. This is especially true for novel alterations that lack obvious functional consequences. We report here on a patient with Treacher Collins syndrome (TCS) found to carry a previously reported mutation, c.122C > T, which predicts p.A41V, and a novel synonymous mutation, c.3612A > C. Pedigree analysis showed that the c.122C > T mutation segregated with normal phenotypes in multiple family members while the c.3612A > C was de novo in the patient. Analysis of TCOF1 RNA in lymphocytes showed a transcript missing exon 22. These results show that TCS in the patient is due to haploinsufficiency of TCOF1 caused by the synonymous de novo c.3612A > C mutation. This study highlights the importance of clinical and pedigree evaluation in the interpretation of known and novel sequence alterations. 2009 Wiley-Liss, Inc.

  1. [An autopsy case of progressive generalized muscle atrophy over 14 years due to post-polio syndrome].

    Science.gov (United States)

    Oki, Ryosuke; Uchino, Akiko; Izumi, Yuishin; Ogawa, Hirohisa; Murayama, Shigeo; Kaji, Ryuji

    2016-01-01

    We report the case of a 72-year-old man who had contracted acute paralytic poliomyelitis in his childhood. Thereafter, he had suffered from paresis involving the left lower limb, with no relapse or progression of the disease. He began noticing slowly progressive muscle weakness and atrophy in the upper and lower extremities in his 60s. At the age of 72, muscle weakness developed rapidly, and he demonstrated dyspnea on exertion and dysphagia. He died after about 14 years from the onset of muscle weakness symptoms. Autopsy findings demonstrated motoneuron loss and glial scars not only in the plaque-like lesions in the anterior horns, which were sequelae of old poliomyelitis, but also throughout the spine. No Bunina bodies, TDP-43, and ubiquitin inclusions were found. Post-polio syndrome is rarely fatal due to rapid progressive dyspnea and dysphagia. Thus, the pathological findings in the patient are considered to be related to the development of muscle weakness.

  2. Clinical features, diagnosis, treatment and molecular studies in paediatric Cushing's syndrome due to primary nodular adrenocortical hyperplasia

    DEFF Research Database (Denmark)

    Storr, Helen L; Mitchell, J H; Swords, F M

    2004-01-01

    BACKGROUND: Primary nodular adrenocortical hyperplasia (PNAH) is a well recognized, but infrequently studied cause of paediatric Cushing's syndrome (CS). OBJECTIVE: To assess presentation, diagnosis, radiological imaging, treatment and molecular analysis of patients with childhood-onset CS due...... to PNAH. PATIENTS: Four males and two females (median age 12.9 years, range 10.9-16.9 years) were studied. RESULTS: All had growth failure (mean height SDS -1.2; range -2.5-0.0), weight gain [mean body mass index (BMI) SDS 3.5; range 2.5-4.6] and clinical virilization, while five had hypertension [mean...... systolic blood pressure (SBP) 130 mmHg, diastolic blood pressure (DBP) 83 mmHg]. One patient had generalized lentigines, one had a tibial chondromyxomatous cyst and two had facial freckling. One patient had a family history of primary nodular adrenocortical disease. The diagnosis of CS was based...

  3. ARCN1 Mutations Cause a Recognizable Craniofacial Syndrome Due to COPI-Mediated Transport Defects.

    Science.gov (United States)

    Izumi, Kosuke; Brett, Maggie; Nishi, Eriko; Drunat, Séverine; Tan, Ee-Shien; Fujiki, Katsunori; Lebon, Sophie; Cham, Breana; Masuda, Koji; Arakawa, Michiko; Jacquinet, Adeline; Yamazumi, Yusuke; Chen, Shu-Ting; Verloes, Alain; Okada, Yuki; Katou, Yuki; Nakamura, Tomohiko; Akiyama, Tetsu; Gressens, Pierre; Foo, Roger; Passemard, Sandrine; Tan, Ene-Choo; El Ghouzzi, Vincent; Shirahige, Katsuhiko

    2016-08-04

    Cellular homeostasis is maintained by the highly organized cooperation of intracellular trafficking systems, including COPI, COPII, and clathrin complexes. COPI is a coatomer protein complex responsible for intracellular protein transport between the endoplasmic reticulum and the Golgi apparatus. The importance of such intracellular transport mechanisms is underscored by the various disorders, including skeletal disorders such as cranio-lenticulo-sutural dysplasia and osteogenesis imperfect, caused by mutations in the COPII coatomer complex. In this article, we report a clinically recognizable craniofacial disorder characterized by facial dysmorphisms, severe micrognathia, rhizomelic shortening, microcephalic dwarfism, and mild developmental delay due to loss-of-function heterozygous mutations in ARCN1, which encodes the coatomer subunit delta of COPI. ARCN1 mutant cell lines were revealed to have endoplasmic reticulum stress, suggesting the involvement of ER stress response in the pathogenesis of this disorder. Given that ARCN1 deficiency causes defective type I collagen transport, reduction of collagen secretion represents the likely mechanism underlying the skeletal phenotype that characterizes this condition. Our findings demonstrate the importance of COPI-mediated transport in human development, including skeletogenesis and brain growth. Copyright © 2016 American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  4. Hepatoencephalopathy syndrome due to Cassia occidentalis (Leguminosae, Caesalpinioideae) seed ingestion in horses.

    Science.gov (United States)

    Oliveira-Filho, J P; Cagnini, D Q; Badial, P R; Pessoa, M A; Del Piero, F; Borges, A S

    2013-03-01

    Cassia occidentalis is a bush from the Leguminosae family, subfamily Caesalpinoideae, and is a toxic plant of veterinary interest due to the occasional contamination of animal rations. This report describes the clinical and histopathological findings of an outbreak of C. occidentalis poisoning in horses. Twenty mares were poisoned after consuming ground corn contaminated with 8% of C. occidentalis seeds. Of the 20 animals affected, 12 died: 8 mares were found dead, 2 died 6 h after the onset of clinical signs compatible with hepatic encephalopathy and the 2 other animals were subjected to euthanasia 12 h after the onset of the clinical signs. The remaining 8 mares presented with mild depression and decreased appetite, but improved with treatment and no clinical sequelae were observed. In 6 animals that underwent a necropsy, an enhanced hepatic lobular pattern was noted and within the large intestine, a large number of seeds were consistently observed. Hepatocellular pericentrolobular necrosis and cerebral oedema were the main histological findings. In one mare, there was mild multifocal semimembranosus rhabdomyocytic necrosis and haemorrhage. Seeds collected from intestinal contents and sifted from the culpable feedstuff were planted. Examination of the leaves, flowers, fruits and seeds of the resultant plants identified C. occidentalis. Horses poisoned by C. occidentalis seeds demonstrate clinical signs associated with hepatoencephalopathy and frequently die suddenly. Lesions primarily involve the liver and secondarily, the central nervous system. Cassia occidentalis poisoning should be considered a differential diagnosis in horses with hepatoencephalopathy and special caution should be taken with horse rations to avoid contamination with seeds of this toxic plant. © 2012 EVJ Ltd.

  5. Aspiration Risk and Respiratory Complications in Patients with Esophageal Atresia.

    Science.gov (United States)

    Kovesi, Thomas

    2017-01-01

    Chronic, long-term respiratory morbidity (CRM) is common in patients with a history of repaired congenital esophageal atresia, typically associated with tracheoesophageal fistula (EA/TEF). EA/TEF patients are at high risk of having aspiration, and retrospective studies have associated CRM with both recurrent aspiration and atopy. However, studies evaluating the association between CRM in this population and either aspiration or atopy have reported conflicting results. Furthermore, CRM in this population may be due to other related conditions as well, such as tracheomalacia and/or recurrent infections. Aspiration is difficult to confirm, short of lung biopsy. Moreover, even within the largest evidence base assessing the association between CRM and aspiration, which has evaluated the potential relationship between gastroesophageal reflux and asthma, findings are contradictory. Studies attempting to relate CRM to prior aspiration events may inadequately estimate the frequency and severity of previous aspiration episodes. There is convincing evidence documenting that chronic, massive aspiration in patients with repaired EA/TEF is associated with the development of bronchiectasis. While chronic aspiration is likely associated with other CRM in patients with repaired EA/TEF, this does not appear to have been confirmed by the data currently available. Prospective studies that systematically evaluate aspiration risk and allergic disease in patients with repaired EA/TEF and document subsequent CRM will be needed to clarify the causes of CRM in this population. Given the prevalence of CRM, patients with repaired EA/TEF should ideally receive regular follow-up by multidisciplinary teams with expertise in this condition, throughout both childhood and adulthood.

  6. Autosomal dominant {open_quotes}Opitz{close_quotes} GBBB syndrome due to a 22q11.2 deletion

    Energy Technology Data Exchange (ETDEWEB)

    McDonald-McGinn, D.M.; Emanuel, B.S.; Zackai, E.H. [Univ. of Pennsylvania School of Medicine, Philadelphia, PA (United States)

    1996-08-23

    The classification of Opitz GBBB syndrome has been associated with the deletion of the DiGeorge chromosome region on human chromosome 22q11.2. The broad phenotype involved in this deletion syndrome has been referred to as the DiGeorge/velocardiofacial syndrome. The clinical description of the patient will influence the diagnosis of the syndrome. More cooperation between the clinicians and the molecular researchers is necessary in order to locate the gene(s) for these disorders. 11 refs.

  7. ASPIRE: Added-value Sensing

    DEFF Research Database (Denmark)

    Anggorojati, Bayu; Cetin, Kamil; Mihovska, Albena D.

    2010-01-01

    FP7 ICT project ASPIRE is one of the coordinated European efforts to further the advancement of this technology, in the areas of enabling technology development for RFID. The focus of ASPIRE is on the design, development and adoption of an innovative, programmable, royalty-free, lightweight...

  8. Budd-Chiari syndrome due to prothrombotic disorder: mid-term patency and efficacy of endovascular stents

    Energy Technology Data Exchange (ETDEWEB)

    Pelage, Jean-Pierre; Denys, Alban; Sibert, Annie; Menu, Yves [Department of Radiology, Hopital Beaujon, AP-HP, 100 Boulevard du General Leclerc, 92110 Clichy (France); Valla, Dominique [Department of Hepatology, Hopital Beaujon, AP-HP, 100 Boulevard du General Leclerc, 92110 Clichy (France); Sauvanet, Alain; Belghiti, Jacques [Department of Surgery, Hopital Beaujon, AP-HP, 100 Boulevard du General Leclerc, 92110 Clichy (France)

    2003-02-01

    Our objective was to evaluate efficacy and patency of metallic stent placement for symptomatic Budd-Chiari syndrome (BCS) due to prothrombotic disorders. Eleven patients with proved BCS due to prothrombotic disorders were referred for endovascular treatment because of refractory ascites (n=9), abdominal pain (n=8), jaundice (n=6), and/or gastrointestinal bleeding (n=4). Stents were inserted for stenosed hepatic vein (n=7), inferior vena cava (n=2), or mesenterico-caval shunt (n=2). Clinical efficacy and stent patency was evaluated by clinical and Doppler follow-up. After a mean follow-up of 21 months, 6 patients had fully patent stents without reintervention (primary stent patency: 55%). Two patients with hepatic vein stenosis had stent thrombosis and died 4 months after procedure. Restenosis occurred in 3 cases (2 hepatic vein and 1 mesenterico-caval shunt stenosis) and were successfully treated by balloon angioplasty (n=2) and addition of new stents (n=1) leading to a 82% secondary stent patency. Of 9 patients with patent stent, 7 were asymptomatic (77%) at the end of the study. Stent placement is a safe and effective procedure to control of symptomatic BCS. Prothrombotic disorder does not seem to jeopardize patency in anticoagulated patients. (orig.)

  9. Angelman Syndrome due to a Maternally Inherited Intragenic Deletion Encompassing Exons 7 and 8 of the UBE3A Gene.

    Science.gov (United States)

    Ververi, Athina; Islam, Lily; Bewes, Beverley; Busby, Louise; Sullivan, Caroline; Canham, Natalie

    2017-01-01

    Angelman syndrome (AS) is characterised by developmental delay, lack of speech, seizures, a characteristic behavioural profile with a happy demeanour, microcephaly, and ataxia. More than two-thirds of cases are due to an approximately 5-Mb interstitial deletion of the imprinted region 15q11.2q13, which is usually de novo. The rest are associated with point mutations in the UBE3A gene, imprinting defects, and paternal uniparental disomy. Small intragenic UBE3A deletions have rarely been described. They are usually maternally inherited, increasing the recurrence risk to 50%, and may be missed by conventional testing (methylation studies and UBE3A gene sequencing). We describe a boy with AS due to an 11.7-kb intragenic deletion. The deletion was identified by array-CGH and was subsequently detected in his affected first cousin and unaffected maternal grandfather, mother, and aunt, confirming the silencing of the paternal allele. The patient had developmental delay, speech impairment, a happy demeanour, microcephaly, and an abnormal EEG, but no seizures by the age of 4 years. Delineation of the underlying genetic mechanism is of utmost importance for reasons of genetic counselling, as well as appropriate management and prognosis. Alternative techniques, such as array-CGH and MLPA, are necessary when conventional testing for AS has failed to identify the underlying genetic mechanism. © 2017 S. Karger AG, Basel.

  10. Iatrogenic Cushing's syndrome due to coadministration of ritonavir and inhaled budesonide in an asthmatic human immunodeficiency virus infected patient.

    Science.gov (United States)

    Kedem, Eynat; Shahar, Eduardo; Hassoun, Gamal; Pollack, Shimon

    2010-09-01

    Iatrogenic Cushing's syndrome (CS) is caused by exposure to glucocorticoids and may be promoted by interaction with additional drugs. It is well known in asthmatic human immunodeficiency virus (HIV)-infected patients treated with inhaled fluticasone with ritonavir-containing antiretroviral regimen (cART). The authors present an asthmatic HIV-infected Ethiopian woman, treated with fluticasone/salmeterol, commencing cART with tenofovir, emtricitabine, and lopinavir/ritonavir. During 7 months she gained 9 kg and hyperpigmentation, mild edema, marked abdominal striae, and increase in blood pressure were noted. Plasma am and urine free cortisol levels confirmed CS diagnosis and fluticasone was discontinued. Complete resolution of CS occurred within 2 months. However, frequent asthma symptoms required resumption of inhaled corticosteroid (ICS) treatment, and budesonide/formeterol was prescribed. Soon reemergence of symptomatic CS was noted. Ritonavir dose was halved, but CS symptoms continued to develop. Budesonide was stopped and montelukast initiated. Resolution of cushingoid symptoms was observed within weeks. Corticosteroids are metabolized by cytochrome P450 3A4 (CYP3A4). Fluticasone has the longest glucocorticoid receptor-binding half-life and is 300 times more lipophilic than budesonide. Inhaled fluticasone possesses a high suppression rate of hypothalamic-pituitary-adrenal axis. Ritonavir, a potent CYP3A4 inhibitor, may inhibit corticosteroid degradation and increase its accumulation. Inhaled budesonide is less likely to cause adrenal suppression. Diagnosing Cushing's syndrome presents a clinical challenge due to similarities with clinical manifestations and side effects related to cART. In patients treated with inhaled or intranasal corticosteroids together with cART there may be a higher incidence of iatrogenic CS. CS should be looked for, and management considered carefully.

  11. Workload, Aspiration, and Fun

    DEFF Research Database (Denmark)

    Muhr, Sara Louise; Pedersen, Michael; Alvesson, Mats

    2012-01-01

    Contemporary working life highlights the challenge between exploitation and exploration both on a general and a more individual level. Here, we focus on the latter, and connect the critical debate regarding selfmanagement to March’s exploitation/exploration trade-off, as this forms a useful...... theoretical frame to understand how employees make sense of their self-management efforts. The employee is subjected to an individual responsibility to understand and manage an exploration of the self while handling the norms of self-exploitation that a self-management culture creates. Through an empirical...... study of a large group of management consultants, we explore how they perform and make sense of selfexploitation and self-exploration through three specific discourses: the discourse of workload, the discourse of aspiration, and the discourse of fun. Through these, the consultants try to identify...

  12. Fanconi syndrome

    Science.gov (United States)

    De Toni-Fanconi syndrome ... Fanconi syndrome can be caused by faulty genes, or it may result later in life due to kidney damage. Sometimes the cause of Fanconi syndrome is unknown. Common causes of Fanconi syndrome in ...

  13. Severe Cushing’s syndrome due to small cell prostate carcinoma: a case and review of literature

    Directory of Open Access Journals (Sweden)

    M S Elston

    2017-07-01

    Full Text Available Cushing’s syndrome (CS due to ectopic adrenocorticotrophic hormone (ACTH is associated with a variety of tumours most of which arise in the thorax or abdomen. Prostate carcinoma is a rare but important cause of rapidly progressive CS. To report a case of severe CS due to ACTH production from prostate neuroendocrine carcinoma and summarise previous published cases. A 71-year-old male presented with profound hypokalaemia, oedema and new onset hypertension. The patient reported two weeks of weight gain, muscle weakness, labile mood and insomnia. CS due to ectopic ACTH production was confirmed with failure to suppress cortisol levels following low- and high-dose dexamethasone suppression tests in the presence of a markedly elevated ACTH and a normal pituitary MRI. Computed tomography demonstrated an enlarged prostate with features of malignancy, confirmed by MRI. Subsequent prostatic biopsy confirmed neuroendocrine carcinoma of small cell type and conventional adenocarcinoma of the prostate. Adrenal steroidogenesis blockade was commenced using ketoconazole and metyrapone. Complete biochemical control of CS and evidence of disease regression on imaging occurred after four cycles of chemotherapy with carboplatin and etoposide. By the sixth cycle, the patient demonstrated radiological progression followed by recurrence of CS and died nine months after initial presentation. Prostate neuroendocrine carcinoma is a rare cause of CS that can be rapidly fatal, and early aggressive treatment of the CS is important. In CS where the cause of EAS is unable to be identified, a pelvic source should be considered and imaging of the pelvis carefully reviewed.

  14. Guyon's canal syndrome due to tortuous ulnar artery with DeQuervain stenosing tenosynovitis, ligamentous injuries and dorsal intercalated segmental instability syndrome, a rare presentation: a case report

    OpenAIRE

    Zeeshan, Muhammad; Ahmed, Farhan; Kanwal, Darakhshan; Khalid, Qazi Saad Bin; Ahmed, Muhammad Nadeem

    2009-01-01

    The Guyon's canal syndrome is a well known clinical entity and may have significant impact on patient's quality of life. We report a case of 43-year-old male who presented with complaints of pain and numbness in right hand and difficulty in writing for past one month. On imaging diagnosis of Guyon's canal syndrome because of tortuous ulnar artery was made with additional findings of DeQuervain's stenosing tenosynovitis and dorsal intercalated segmental instability syndrome with ligamentous in...

  15. Cushing's syndrome with adrenal suppression induced by inhaled budesonide due to a ritonavir drug interaction in a woman with HIV infection.

    Science.gov (United States)

    Yoganathan, K; David, L; Williams, C; Jones, K

    2012-07-01

    A 48-year-old woman with HIV infection developed Cushingoid features while she was taking ritonavir-boosted darunavir. Cushing's syndrome was confirmed due to the drug interaction between ritonavir and budesonide. Diagnosis of iatrogenic Cushing's syndrome in HIV-positive patients who are on ritonavir-boosted protease inhibitors (PIs) presents a clinical challenge due to similar clinical features of lipohypertrophy related to ritonavir-boosted PIs. Although this complication has been widely described with the use of inhaled fluticasone, the interaction with inhaled budesonide at therapeutic dose is not widely recognized.

  16. Guyon's canal syndrome due to tortuous ulnar artery with DeQuervain stenosing tenosynovitis, ligamentous injuries and dorsal intercalated segmental instability syndrome, a rare presentation: a case report.

    Science.gov (United States)

    Zeeshan, Muhammad; Ahmed, Farhan; Kanwal, Darakhshan; Khalid, Qazi Saad Bin; Ahmed, Muhammad Nadeem

    2009-12-23

    The Guyon's canal syndrome is a well known clinical entity and may have significant impact on patient's quality of life. We report a case of 43-year-old male who presented with complaints of pain and numbness in right hand and difficulty in writing for past one month. On imaging diagnosis of Guyon's canal syndrome because of tortuous ulnar artery was made with additional findings of DeQuervain's stenosing tenosynovitis and dorsal intercalated segmental instability syndrome with ligamentous injury and subsequently these were confirmed on surgery.Although it is a rare syndrome, early diagnosis and treatment prevents permanent neurological deficits and improve patient's quality of life.

  17. Traumatic brain injury is unlikely precipitating Leigh syndrome due to the GJB2 mutation c.35delG

    Directory of Open Access Journals (Sweden)

    Josef Finsterer

    2017-06-01

    Full Text Available With interest we read the article by Ashrafi et al. about a 14-year-old female who is regarded to have developed Leigh syndrome (LS after traumatic brain injury (TBI. We have the following comments and concerns:We do not agree with the notion that traumatic brain injury was the precipitating factor for LS. The patient had a history of hypoacusis, which is a typical clinical manifestation of a mitochondrial disorder (MID. Hypoacusis obviously had developed long before the TBI. Additionally, the patient was diagnosed with neuropathy of the peripheral nerves two months after TBI. It is rather unlikely that neuropathy was triggered by TBI and more likely it was already present before the trauma. Thus, the initial manifestations of LS in the presented patient were most likely hypoacusis followed by neuropathy and TBI only might have triggered the seizure but not the MID. Why was the patient put on phenytoin, which is well-known to be mitochondrion-toxic? Phenytoin may worsen epilepsy and MID in general and it is conceivable that in fact phenytoin was responsible for worsening of the phenotype and not the TBI. In a 16-year-old female with MELAS syndrome due to the mutation m.3243A>G, phenytoin caused intestinal pseudo-obstruction one month after intravenous phenytoin for status epilepticus. In a patient with Kearns-Sayre syndrome phenytoin decreased cerebrospinal fluid (CSF folate levels. In rat hepatocytes, phenytoin increased reactive oxygen species (ROS formation, decreased intracellular reduced glutathione, increased intracellular oxidised glutathione, and enhanced lipid peroxidation and mitochondrial damage. In a hepatic microsomal system, phenytoin decreased state-3 respiration, ATP synthesis, and the mitochondrial membrane potential. In this model, phenytoin increased state-4 respiration, impaired Ca++-uptake and release, and inhibited Ca++-induced swelling. It would be interesting to know how the GJB2 mutation was detected. Was whole exome or

  18. New therapeutic options in meconium aspiration syndrome

    OpenAIRE

    Colvero, Mauricio Obal; Colvero, Aline Pieruccini; Fiori, Renato Machado; Garcia, Pedro Celiny Ramos

    2006-01-01

    OBJETIVOS: revisar a literatura sobre a síndrome de aspiração de mecônio (SAM), enfocando aspectos clínicos, fisiopatológicos e abordagem terapêutica, com destaque ao uso do surfactante e lavado broncoalveolar. MÉTODOS: revisão baseada em artigos publicados na MEDLINE, SCIELO e resumos de congressos internacionais de 1988 a 2004, incluindo ensaios randomizados ou quasi-randomizados, estudos caso-controle e metanálises. RESULTADOS: devido à comprovação da inibição do surfactante na SAM, houve ...

  19. Hemolytic uremic syndrome with mild renal involvement due to Shiga toxin-producing Escherichia coli (STEC) O145 strain.

    Science.gov (United States)

    Pérez, Lucía; Apezteguía, Lucía; Piñeyrúa, Cecilia; Dabezies, Agustín; Bianco, María N; Schelotto, Felipe; Varela, Gustavo

    2014-01-01

    Hemolytic uremic syndrome (HUS) is a disorder characterized by the presence of the classic triad: microangiopathic hemolytic anemia, thrombocytopenia and acute renal injury. HUS without acute renal failure can be confused with other hematologic diseases. An infantile HUS caused by a Shiga-toxin-producing Escherichia coli (STEC) O145 strain carrying genotype stx2, ehxA, eae subtype β1 is herein reported. The infant did not require dialysis during the acute stage of HUS, evolved favorably, maintained normal blood pressure and normal renal function and had no recurrence until the last control. This could be due to several factors, such as the characteristics of infecting STEC strain and a reduction in host susceptibility to renal injury. This report highlights the regional participation of non-O157 STEC in childhood diseases and the importance of performing active surveillance for all forms of HUS. Copyright © 2014 Asociación Colombiana de Psiquiatría. Publicado por Elsevier España. All rights reserved.

  20. A Case of Renal Tubular Acidosis with Sjogren's Syndrome Showing Paradoxical Block of PTH Due to Severe Hypomagnesemia.

    Science.gov (United States)

    Kumar, B Vinodh; Sivalingam, M; Kumaran, G Shiva; Balakrishnan, Balambal

    2017-10-01

    Distal renal tubular acidosis (RTA) manifests either as Complete/Classical form or Incomplete/Latent Form. Distal RTA causes normal anion gap metabolic acidosis and hypokalemia. Interstitial Nephritis is the most frequent renal manifestation of Sjogren's, which presents as Distal RTA in 25-40% of patients with Sjogren's syndrome. Magnesium deficiency is frequently associated with hypokalemia. Although serum calcium is the main physiological control for the secretion of parathyroid hormone (PTH) by the parathyroid, serum magnesium can also exert similar effects. While low levels of magnesium stimulate the secretion of PTH, very low serum concentrations tend to induce a paradoxical block of PTH release by activation of the alpha-subunits of heterotrimeric G-proteins. This mimicks the activation of calcium sensing receptor leading to inhibition of PTH secretion. Here we describe the case history of a 35-year-old lady who presented to our hospital with severe hypokalemia due to distal RTA and perhaps had a paradoxical block of PTH secretion in the setting of severe hypomagnesemia.

  1. Evolution of Choroidal Neovascularization due to Presumed Ocular Histoplasmosis Syndrome on Multimodal Imaging including Optical Coherence Tomography Angiography

    Directory of Open Access Journals (Sweden)

    T. Y. Alvin Liu

    2018-01-01

    Full Text Available A 37-year-old Caucasian woman presented with acute decrease in central vision in her right eye and was found to have subfoveal choroidal neovascularization (CNV due to presumed ocular histoplasmosis syndrome (POHS. Her visual acuity improved from 20/70 to 20/20 at her 6-month follow-up, after 3 consecutive monthly intravitreal bevacizumab injections were initiated at her first visit. Although no CNV activity was seen on fluorescein angiography (FA or spectral-domain optical coherence tomography (SD-OCT at her 2-month, 4-month, and 6-month follow-up visits, persistent flow in the CNV lesion was detected on optical coherence tomography angiography (OCTA. OCTA shows persistent vascular flow as well as changes in vascular flow in CNV lesions associated with POHS, indicating the continued presence of patent vessels and changes in these CNV lesions, even when traditional imaging of the lesion with OCT and FA indicates stability of the lesion with no disease activity. Additional cases with longitudinal follow-up are needed to assess how OCTA should be incorporated into clinical practice.

  2. A family with osteoporosis pseudoglioma syndrome due to compound heterozygosity of two novel mutations in the LRP5 gene.

    Science.gov (United States)

    Cheung, W M W; Jin, L Y; Smith, D K; Cheung, P T; Kwan, E Y W; Low, L; Kung, A W C

    2006-09-01

    Osteoporosis pseudoglioma syndrome (OPPG) is an autosomal recessive disorder due to mutations in the low-density lipoprotein receptor-related protein 5 (LRP5) gene. Here, we report two novel missense mutations found in a southern Chinese family of a non-consanguineous marriage. Three out of four children had blindness, low bone mineral density (BMD) and multiple fractures in their childhood. Genotyping by DNA sequencing demonstrated 2 new mutations in exon 7 of the LRP5 gene. Tryptophans at amino acid residue positions 478 and 504 were replaced by arginine (W478R) and cysteine (W504C), respectively. While the parents that possessed either heterozygous W478R or W504C were apparently normal, all affected subjects were compound heterozygotes for the W478R and W504C mutations in the LRP5 gene. W478R is located immediately C-terminal to the third YWTD repeat of the second YWTD/EGF domain in LRP5, while W504C is located between the third and the fourth YWTD repeats of the second YWTD/EGF domain in LRP5. Using LRP5-related proteins, such as the low-density lipoprotein receptor (LDLR) and nidogen as reference models, a homology model of LRP5 suggested that the observed mutations may affect the molecular interactions of LRP5 and so lead to the observed OPPG phenotypes.

  3. A Gut Gone to Pot: A Case of Cannabinoid Hyperemesis Syndrome due to K2, a Synthetic Cannabinoid

    Directory of Open Access Journals (Sweden)

    Anene Ukaigwe

    2014-01-01

    Full Text Available Cannabinoid Hyperemesis Syndrome (CHS was first described in 2004. Due to its novelty, CHS is often unrecognized by clinicians leading to expensive workup of these patients with cyclical symptoms. It may take up to 9 years to diagnose CHS. CHS is characterized by cyclical nausea and vomiting, abdominal pain, and an unusual compulsion to take hot showers in the presence of chronic use of cannabinoids. Cannabicyclohexanol is a synthetic cannabinoid, popularly known as K2 spice. It is a popular marijuana alternative among teenagers and young adults since it is readily available as herbal incense. Unlike marijuana, many users know that K2 is not detected in conventional urine drug screens, allowing those users to conceal their intake from typical detection methods. Serum or urine gas chromatography mass spectrophotometry is diagnostic, though not widely available. Thus, it is imperative for clinicians to recognize CHS, even with negative UDS, to provide cost-effective care. We present a 38-year-old man with a 10-year history of cannabis, and 1-year history of K2 abuse admitted with 1-week history of episodes of nausea, vomiting of clear fluids, and epigastric discomfort. Symptoms are relieved only by hot showers. Extensive laboratory, radiologic, and endoscopic evaluation was unrevealing. CHS was diagnosed, based on proposed criteria by Simonetti et al.

  4. Cutaneous reactions simulating erythema multiforme and Stevens Johnson syndrome due to occupational exposure to a plant-growth regulator

    Directory of Open Access Journals (Sweden)

    Inamadar Arun

    2007-01-01

    Full Text Available Background: In India, hydrogen cyanamide (Dormex ® is a plant growth regulator used mainly for the bud-breaking of grapevines. The use of this chemical may result in severe cutaneous reactions simulating erythema multiforme (EM, Stevens-Johnson syndrome (SJS and toxic epidermal necrolysis (TEN. Methods: Studies were conducted on four seasonal grapevine workers who developed severe cutaneous reactions following the unprotected use of Dormex ® (hydrogen cyanamide. Results: Two of the patients had EM-like skin lesions and the other two developed SJS-TEN-like skin lesions. A latent period of 5-7 days existed between the contact with the chemical and the development of the skin lesions. The histopathological picture was suggestive of EM. All the patients responded to systemic steroids and antihistamines. Conclusions: Hydrogen cyanamide may act as a hapten, initiating cytotoxic immunological attack on keratinocytes, resulting in EM- and SJS-TEN-like clinical picture. Awareness regarding such severe cutaneous reactions due to the inappropriate handling of Dormex ® is required. The use of personal protection equipments while handling agricultural chemicals is essential.

  5. A Novel Porcine Model of Septic Shock Induced by Acute Respiratory Distress Syndrome due to Methicillin-resistantStaphylococcus aureus.

    Science.gov (United States)

    Wang, Shuo; Wang, Jun-Yu; Wang, Tao; Hang, Chen-Chen; Shao, Rui; Li, Chun-Sheng

    2017-05-20

    Sepsis is one of the main causes of mortality in critically ill patients following progression to septic shock. To investigate the pathophysiologic changes of sepsis, we developed a novel porcine model of septic shock induced by acute respiratory distress syndrome (ARDS) due to methicillin-resistant Staphylococcus aureus(MRSA) pneumonia. Twenty-six male Landraces (Lvyuanweiye, Beijing, China) weighing 30 ± 2 kg were divided into four groups: sham group (SH; n = 5); cotton smoke inhalation group (SM; n = 6); MRSA pneumonia group (MR; n = 6); and septic shock group with cotton smoke inhalation + MRSA pneumonia (SS; n = 9). Extensive hemodynamics, oxygen dynamics, and lung function were monitored for 24 h following the injury or until death. Tissues were collected, and histopathology evaluations were carried out. Blood cultures from 6 of 9 animals in the SS group were positive for MRSA. Two hours following the injury, decreased mean arterial blood pressure (60-70 mmHg) and cardiac index (septic shock were only observed in the SS group but not significant in the other groups. The PO2/FiO2in the SM and SS groups decreased to 300 and 100, respectively. In the SS group, extravascular lung water index increased to 20 ml/kg, whereas thoracopulmonary compliance decreased to 10 ml/H2O after injury. Deterioration of pulmonary function in the SS group was more serious than the SM and MR groups. Severe lung injury in the SS group was confirmed by the histopathology evaluations. The lung injury confirmed by high-resolution thin-section computed tomography and histopathology in the SS group was more serious than those of other groups. In the present study, we developed a novel porcine model of septic shock induced by ARDS due to severe MRSA pneumonia with characteristic hyperdynamic and hypodynamic phases in 24 h, which mimicked the hemodynamic changing of septic shock in human.

  6. Respiratory chain complex I deficiency due to NDUFA12 mutations as a new cause of Leigh syndrome

    DEFF Research Database (Denmark)

    Ostergaard, Elsebet; Rodenburg, Richard J; van den Brand, Mariël

    2011-01-01

    This study investigated a girl with Leigh syndrome born to first-cousin parents of Pakistani descent with an isolated respiratory chain complex I deficiency in muscle and fibroblasts. Her early development was delayed, and from age 2 years she started losing motor abilities. Cerebral MRI showed...... basal ganglia lesions typical of Leigh syndrome....

  7. Comparison of the CDC Backpack aspirator and the Prokopack aspirator for sampling indoor- and outdoor-resting mosquitoes in southern Tanzania

    Directory of Open Access Journals (Sweden)

    Mgando Joseph

    2011-06-01

    Full Text Available Abstract Background Resting mosquitoes can easily be collected using an aspirating device. The most commonly used mechanical aspirator is the CDC Backpack aspirator. Recently, a simple, and low-cost aspirator called the Prokopack has been devised and proved to have comparable performance. The following study evaluates the Prokopack aspirator compared to the CDC backpack aspirator when sampling resting mosquitoes in rural Tanzania. Methods Mosquitoes were sampled in- and outdoors of 48 typical rural African households using both aspirators. The aspirators were rotated between collectors and households in a randomized, Latin Square design. Outdoor collections were performed using artificial resting places (large barrel and car tyre, underneath the outdoor kitchen (kibanda roof and from a drop-net. Data were analysed with generalized linear models. Results The number of mosquitoes collected using the CDC Backpack and the Prokopack aspirator were not significantly different both in- and outdoors (indoors p = 0.735; large barrel p = 0.867; car tyre p = 0.418; kibanda p = 0.519. The Prokopack was superior for sampling of drop-nets due to its smaller size. The number mosquitoes collected per technician was more consistent when using the Prokopack aspirator. The Prokopack was more user-friendly: technicians preferred using the it over the CDC backpack aspirator as it weighs considerably less, retains its charge for longer and is easier to manoeuvre. Conclusions The Prokopack proved in the field to be more advantageous than the CDC Backpack aspirator. It can be self assembled using simple, low-cost and easily attainable materials. This device is a useful tool for researchers or vector-control surveillance programs operating in rural Africa, as it is far simpler and quicker than traditional means of sampling resting mosquitoes. Further longitudinal evaluations of the Prokopack aspirator versus the gold standard pyrethrum spray catch for indoor resting

  8. The social contagion of aspirations

    DEFF Research Database (Denmark)

    Folmann, Birgitte

    2017-01-01

    succeed, making some progress along this path seems important and fuels their ongoing aspiration for the good life. Having a ‘life style’ means being able to choose and consume, and getting a ‘life style’ reflects an aspiration for social mobility. Taking the emic approach helps to explain how social......Discussions of health-related behaviors and lifestyle often become theoretical and morally laden owing to their individualistic view on risk factors and life choices. This article uses the analytical concepts of contagion and configuration to explore the spread of aspirations for the good life...... among young men in Northern Uganda. The potential social contagion of aspirations is unfolded to provide a deeper understanding of social processes not only as dynamics between people but also as processes between people and their surroundings in a society which is subject to rapid change...

  9. Studies on the prevention of respiratory distress syndrome of infants due to hyaline membrane disease with plasminogen.

    Science.gov (United States)

    Ambrus, C M; Choi, T S; Weintraub, D H; Eisenberg, B; Staub, H P; Courey, N G; Foote, R J; Goplerud, D; Moesch, R V; Ray, M; Bross, I D; Jung, O S; Mink, I B; Ambrus, J L

    1975-07-01

    Hyaline membrane disease (HMD) is leading single cause of death of newborn, premature infants. The "hyaline membranes" consist chiefly of fibrin. The clinical manifestation of HMD is the respiratory distress syndrome (RDS). Infants with RDS were treated with urokinase-activated human plasmin in a previous clinical trial. Survival rate was increased in the plasmin treated group as compared to the placebo recipients. However, cost and difficulty in the preparation of the enzyme made this treatment impractical. We, as well as others, have shown the premature infants lack serum plasminogen; thus they are unable to develop effective fibrinolysis and are defenseless against pulmonary fibrin deposition. Therefore, plamsinogen was tested as a possible preventive agent in RDS due to HMD. In a double blind, randomized study, infants between 1 and 2.5 kg birth weight received plasminogen or placebo shortly after birth, and were then followed for development of RDS. After 100 infants were entered into the study, the code was broken and results were evaluated to assure safety of the procedure. Among the 100 infants, 51 received placebo, 49 received plasminogen. Among the infants who received placebo, seven developed mild, and ten developed severe respiratory distress; of these ten, five died with histopathologically documented HMD. Two infants died from causes other than HMD. Among the 49 infants treated with plasminogen, 13 developed mild and three developed severe respiratory distress. There was no death due to HMD. Two deaths were due to other causes. Factors placing the infant at risk from HMD (degree of prematurity, sex, cesarean section, bleeding episodes during pregnancy, maternal diabetes) were found to be evenly distributed between control and treated groups. Since completing the first phase of the study, data of an additional 277 infants has become available. Although the code was not broken in this series, a preliminary look at mortality data in comparison with

  10. Unmasking Cryptococcal Meningitis Immune Reconstitution Inflammatory Syndrome due to Granulocyte Colony-Stimulating Factor Use in a Patient with a Poorly Differentiated Germ Cell Neoplasm

    Directory of Open Access Journals (Sweden)

    Nathan C. Bahr

    2014-01-01

    Full Text Available Cryptococcal meningitis immune reconstitution inflammatory syndrome (IRIS is frequently seen in patients with HIV and less frequently in patients on immune suppressive medications for other conditions. Here, we describe the first reported case of unmasking cryptococcal IRIS due to granulocyte colony-stimulating factor used in an HIV-negative patient with chemotherapy-induced neutropenia.

  11. Thalidomide for treatment of gastrointestinal bleedings due to angiodysplasia : a case report in acquired von Willebrand syndrome and review of the literature

    NARCIS (Netherlands)

    Engelen, E T; van Galen, K P M; Schutgens, R E G

    INTRODUCTION: Acquired von Willebrand syndrome is a rare bleeding disorder and treatment of the associated gastrointestinal (GI) bleeding due to angiodysplasia is challenging. AIM: The aim of this study was to present a new case on the successful use of thalidomide in a patient with acquired von

  12. Habits, aspirations and endogenous fertility

    OpenAIRE

    Luciano Fanti

    2012-01-01

    Motivated by the increasing literature on endogenous preferences as well as on endogenous fertility, this paper investigates the implications of the interaction of the endogenous determination of the number of children with habit and aspiration formation in an OLG model. In contrast with the previous literature, we show that greater aspirations may lead to higher savings, and more interestingly, always increase the neoclassical economic growth.

  13. Goodpasture's Syndrome due to IgA in a patient with clinical diagnosis of Henoch Schonlein's purpura

    International Nuclear Information System (INIS)

    Restrepo Cesar A

    2005-01-01

    This is a case of a 23 year old woman with an initial clinical syndrome compatible with glomerulonephritis of uncertain origin, who later showed lesions of purpuric rash characteristics of Henoch- Schonlein Purpura and then complicated with a pulmonary hemorrhage and a rapidly progressive glomerulonephritis, with a mixed lung-kidney syndrome. The renal biopsy showed presence of linear deposits of immunoglobulin A and extra capillary proliferative changes. The case was concluded corresponding to Goodpasture's syndrome for antibodies antiglomerular basement membrane of the type of IgA in the context of a Henoch-Schonlein Purpura.

  14. Single-Session CT-Guided Percutaneous Microwave Ablation of Bilateral Adrenal Gland Hyperplasia Due to Ectopic ACTH Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Sarma, Asha, E-mail: ashasarma@gmail.com; Shyn, Paul B., E-mail: pshyn@partners.org [Brigham and Women’s Hospital, Department of Radiology (United States); Vivian, Mark A. [University of Manitoba, Department of Radiology (Canada); Ng, Ju-Mei [Brigham and Women’s Hospital, Department of Anesthesiology (United States); Tuncali, Kemal [Brigham and Women’s Hospital, Department of Radiology (United States); Lorch, Jorchen H. [Dana Farber Cancer Institute, Department of Medicine (United States); Zaheer, Sarah N.; Gordon, Michael S. [Brigham and Women’s Hospital, Department of Endocrinology (United States); Silverman, Stuart G. [Brigham and Women’s Hospital, Department of Radiology (United States)

    2015-10-15

    Bilateral adrenalectomy is currently the only available treatment for adrenocorticotropic hormone (ACTH)-dependent Cushing’s syndrome (ectopic ACTH syndrome) that is refractory to pharmacologic therapy. We describe two patients with refractory ectopic ACTH syndrome who were treated with CT-guided percutaneous microwave ablation of both hyperplastic adrenal glands in a single session: One was not a surgical candidate, and the other had undergone unsuccessful surgery. Following the procedure, both patients achieved substantial decreases in serum cortisol, symptomatic improvement, and decreased anti-hypertensive medication requirements.

  15. Supersymmetry: aspirations and prospects

    International Nuclear Information System (INIS)

    Tata, Xerxes

    2015-01-01

    The realization in the early 1980s that weak scale supersymmetry stabilizes the Higgs sector of the spectacularly successful Standard Model (SM) led several authors to explore whether low energy supersymmetry could play a role in particle physics. Among these were Richard Arnowitt, Ali Chamseddine and Pran Nath who constructed a viable locally supersymmetric grand unified theory (GUT), laying down the foundation for supergravity GUT models of particle physics. Supergravity models continue to be explored as one of the most promising extensions of the SM. After a quick overview of some of the issues and aspirations of early researchers working to bring supersymmetry into the mainstream of particle physics, we re-examine early arguments that seemed to imply that superpartners would be revealed in experiments at LEP2 or at the Tevatron. Our purpose is to assess whether the absence of any superpartners in searches at LHC8 presents a crisis for supersymmetry. Toward this end, we re-evaluate fine-tuning arguments that lead to upper bounds on (some) superpartner masses. We conclude that phenomenologically viable superpartner spectra that could arise within a high scale model tuned no worse than a few percent are perfectly possible. While no viable underlying model of particle physics that leads to such spectra has yet emerged, we show that the (supergravity-based) radiatively driven natural supersymmetry framework serves as a surrogate for a phenomenological analysis of an underlying theory with modest fine-tuning. We outline the phenomenological implications of this framework, with emphasis on those LHC and electron–positron collider signatures that might point to the underlying natural origin of gauge and Higgs boson masses. We conclude that the supergravity GUT paradigm laid down in 1982 by Arnowitt, Chamseddine and Nath, and others, remains a vibrant possibility. (invited comment)

  16. Delayed diagnosis in a house of correction: Smith-Magenis syndrome due to a de novo nonsense RAI1 variant.

    Science.gov (United States)

    Yeetong, Patra; Vilboux, Thierry; Ciccone, Carla; Boulier, Kristin; Schnur, Rhonda E; Gahl, William A; Huizing, Marjan; Laje, Gonzalo; Smith, Ann C M

    2016-09-01

    We report a 25-year-old female confirmed to have Smith-Magenis syndrome (SMS) due to a de novo RAI1 variant. Her past history is significant for developmental and intellectual delay, early and escalating maladaptive behaviors, and features consistent with significant sleep disturbance, the etiology of which was not confirmed for over two decades. The diagnosis of SMS was initially suspected in 1998 (at age 12 years), but that was 5 years before the initial report of RAI1 variants as causative of the SMS phenotype; cytogenetic fluorescence in situ hybridization studies failed to confirm an interstitial deletion of 17p11.2. Re-evaluation for suspected SMS was pursued with RAI1 sequencing analysis in response to urgent parental concerns of escalating behaviors and aggression with subsequent incarceration of the subject for assault of a health professional. Genetic analysis revealed a de novo RAI1 (NM_030665.3) nonsense variant, c.5536C>T; p.Q1846X. This case illustrates the importance of confirming the SMS diagnosis, which is associated with cognitive and functional impairment, as well as significant psychiatric co-morbidities and behavioral problems. The diagnosis was particularly relevant to the legal discussion and determination of her competence to stand trial. As other similar cases may exist, this report will help to increase awareness of the possibility of a very late diagnosis of SMS, with the need for re-evaluation of individuals suspected to have SMS who were initially evaluated prior to the identification of the RAI1 gene. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  17. Hyponatremia in aneurysmal subarachnoid hemorrhage is due to the syndrome of inappropriate antidiuresis and acute glucocorticoid deficiency

    LENUS (Irish Health Repository)

    Hannon, M J

    2011-06-01

    Hyponatraemia is the most common electrolyte abnormality following subarachnoid haemorrhage (SAH) and contributes to increased morbidity and mortality. Retrospective data suggests that the syndrome of inappropriate diuresis (SIAD) is the most common cause of hyponatraemia in SAH, though cerebral salt wasting has been postulated by some workers to be the predominant abnormality. Data which has shown acute glucocorticoid deficiency following SAH has suggested that some cases of euvolaemic hyponatraemia may also be caused by this mechanism.We prospectively studied the hormonal and haemodynamic influences involved in the development of hyponatraemia in 100 patients (61% female, median age 53 (range 16-82)) with non-traumatic aneurysmal SAH. Each patient had plasma sodium (pNa), urea, osmolality, glucose and 0900h cortisol (PC), and urinary sodium and osmolality measured on days 1, 2, 3, 4, 6, 8, 10 and 12 following SAH. Fluid balance and haemodynamic parameters were recorded daily. Results were compared with 15 patients admitted to ITU following vascular surgery. A PC<300nmol\\/L in a patient in ITU was regarded clinically as inappropriately low.49% of patients developed hyponatraemia (pNa<135 mmol\\/L), including 14% who developed clinically significantly hyponatraemia (pNa<130 mmol\\/L). 36\\/49 (73.4%) developed hyponatraemia between days 1 and 3 post SAH. The median duration of hyponatraemia was 3 days (range 1–10 days).In 35\\/49 (71.4%), hyponatraemia was due to SIAD as defined by standard diagnostic criteria. 14% of SAH patients had at least one PC<300nmol\\/L; 5 of these (35.7%) developed hyponatraemia. In 4 patients hyponatraemia was preceded by acute cortisol deficiency and responded to hydrocortisone treatment. In contrast, all controls had PC>500 nmol\\/L on day 1, and >300 nmol on days 3–12. There were no cases of cerebral salt wasting. There was no relationship between the incidence of hyponatraemia and the defined anatomical territory or severity of

  18. Pure dysarthria and dysarthria-facial paresis syndrome due to internal capsule and/or corona radiata infarction.

    Science.gov (United States)

    Tanaka, Koji; Yamada, Takeshi; Torii, Takako; Yoshimura, Takeo; Takase, Kei-ichiro; Togao, Osamu; Wakata, Yoshifumi; Hiwatashi, Akio; Nakashima, Naoki; Kira, Jun-ichi; Murai, Hiroyuki

    2015-10-07

    Pure dysarthria (PD) and dysarthria-facial paresis syndrome (DFP) mainly result from lenticulostriate artery territory infarction. PD and DFP are rare clinical entities, often grouped without distinction. The purpose of this study was to examine clinical and radiographic differences between PD and DFP due to unilateral internal capsule and/or corona radiata infarction. Using a database that included consecutive patients with ischemic stroke admitted to the neurological stroke units of three hospitals within 7 days from onset between September 2011 and April 2014, we retrospectively extracted first-ever stroke patient data, who presented with PD or DFP with a single ischemic lesion localized in the internal capsule and/or corona radiata. Patients with weakness, ataxia, sensory deficit, or cortical symptoms were excluded. Ischemic lesion volume was calculated by the ABC/2 method on diffusion-weighted imaging (DWI). DWI images were normalized and superimposed to the template for PD and DFP. We compared patients' characteristics between PD and DFP. A total of 2126 patients, including 65 patients (3.1%) with PD or DFP, were registered. Of these, 13 PD patients and 18 patients with DFP due to unilateral internal capsule and/or corona radiata infarction were included for analysis. Compared with DFP patients, PD patients had longer onset-to-door time (median 37.5 vs. 10.8 h, p = 0.031), shorter vertical length (C component) of ischemic lesions (median 12.0 vs. 18.8 mm, p = 0.007), and smaller ischemic lesion volume (median 285 vs. 828 mm(3), p = 0.023). Ischemic lesions causing PD were located more frequently in the left hemisphere than DFP (92% vs. 56%, p = 0.045). The superimposed lesion pattern indicated that DFP had lesions more medial and involving posterior portions of the putamen and the caudate body, as well as more of the genu and posterior limb of the internal capsule, than PD. Ninety days after onset, symptoms disappeared in 21 (72%) out of 29 patients. In

  19. β-cell dysfunction due to increased ER stress in a stem cell model of Wolfram syndrome.

    Science.gov (United States)

    Shang, Linshan; Hua, Haiqing; Foo, Kylie; Martinez, Hector; Watanabe, Kazuhisa; Zimmer, Matthew; Kahler, David J; Freeby, Matthew; Chung, Wendy; LeDuc, Charles; Goland, Robin; Leibel, Rudolph L; Egli, Dieter

    2014-03-01

    Wolfram syndrome is an autosomal recessive disorder caused by mutations in WFS1 and is characterized by insulin-dependent diabetes mellitus, optic atrophy, and deafness. To investigate the cause of β-cell failure, we used induced pluripotent stem cells to create insulin-producing cells from individuals with Wolfram syndrome. WFS1-deficient β-cells showed increased levels of endoplasmic reticulum (ER) stress molecules and decreased insulin content. Upon exposure to experimental ER stress, Wolfram β-cells showed impaired insulin processing and failed to increase insulin secretion in response to glucose and other secretagogues. Importantly, 4-phenyl butyric acid, a chemical protein folding and trafficking chaperone, restored normal insulin synthesis and the ability to upregulate insulin secretion. These studies show that ER stress plays a central role in β-cell failure in Wolfram syndrome and indicate that chemical chaperones might have therapeutic relevance under conditions of ER stress in Wolfram syndrome and other forms of diabetes.

  20. Aortic valve-sparing operation after correction of heart displacement due to pectus excavatum using Nuss procedure in a Marfan syndrome patient.

    Science.gov (United States)

    Fukunaga, Naoto; Yuzaki, Mitsuru; Hamakawa, Hiroshi; Nasu, Michihiro; Takahashi, Yutaka; Okada, Yukikatsu

    2012-01-01

    Cardiovascular surgery in the setting of chest wall deformities is a clinical challenge. Pectus excavatum, for example, can cause heart displacement to the left thoracic cavity, following the poor operative field. This report highlights a case in which a successful aortic valve-sparing operation via conventional median sternotomy after correction of the heart displacement due to pectus excavatum using Nuss procedure in Marfan syndrome. This technique can be one surgical option in Marfan syndrome patients with pectus excavatum and thoracic aortic aneurysm under close follow up.

  1. Papular-purpuric "gloves and socks" syndrome due to parvovirus B19: report of a case with unusual features

    Directory of Open Access Journals (Sweden)

    PASSONI Luiz Fernando C.

    2001-01-01

    Full Text Available We present a case of papular-purpuric "gloves and socks" syndrome (PPGSS in an adult male with acute parvovirus B19 infection. The patient displayed the classical features of fever, oral lesions, and purpura on hands and feet, but the purpuric lesions on the feet evolved to superficial skin necrosis, a feature not previously described in this syndrome. We believe this is the first reported case of PPGSS occurring in Brazil.

  2. Diagnostic Dilemma: Lymphocytopenia in a Patient with Thymoma - Side Effect due to Irradiation Treatment or Development of Good's Syndrome?

    DEFF Research Database (Denmark)

    Raaschou-Jensen, Klas; Katzenstein, Terese L; Marquart, Hanne

    2010-01-01

    A case of persistent B-cell lymphocytopenia in a 40-year-old woman with lymphoid-epithelial thymoma treated with chemotherapy, surgery and irradiation is described. The possible diagnosis of Good's syndrome (hypogammaglobulinaemia and thymoma) is discussed.......A case of persistent B-cell lymphocytopenia in a 40-year-old woman with lymphoid-epithelial thymoma treated with chemotherapy, surgery and irradiation is described. The possible diagnosis of Good's syndrome (hypogammaglobulinaemia and thymoma) is discussed....

  3. Biased Aspirations and Social Inequality at School: Evidence from French Teenagers

    OpenAIRE

    Guyon, Nina; Huillery, Elise

    2016-01-01

    This paper provides empirical evidence on how aspirations are formed and affect individual behavior, decisions, and paths in the context of education. Using unique data on aspirations, academic performance and actual track assignment to high school of French ninth graders, we show that low-SES students have lower aspirations than their equally-achieving high-SES classmates, and that track assignments to high school the next year are even more unequal due to dysfunctional dynamics: first, both...

  4. Quality of canine spermatozoa retrieved by percutaneous epididymal sperm aspiration.

    Science.gov (United States)

    Varesi, S; Vernocchi, V; Faustini, M; Luvoni, G C

    2013-02-01

    To investigate the feasibility of percutaneous epididymal sperm aspiration in dogs and whether it might provide a population of epididymal spermatozoa similar to the population that can be obtained by processing isolated epididymis caudae. Concentration and total sperm number, motility, morphology and acrosomal integrity of spermatozoa retrieved by percutaneous epididymal sperm aspiration, in vitro aspiration and mincing of the cauda of the epididymis were compared. Percutaneous epididymal sperm aspiration is a feasible procedure to retrieve a population of spermatozoa in dogs. Quality is similar to that of spermatozoa collected in vitro, although a wide variation amongst animals was observed. In case of ejaculation failure due to pathological conditions in dogs, the collection of spermatozoa from the cauda of the epididymis could be an option for providing gametes for assisted reproductive technologies. Percutaneous epididymal sperm aspiration can be used in dogs with compromised reproductive performance, in which orchiectomy cannot be performed for medical or owner reasons. Further studies aimed to investigate whether the percutaneous epididymal sperm aspiration technique might be feasible for repeated semen collection and to accurately evaluate side effects are required. © 2013 British Small Animal Veterinary Association.

  5. Budd-Chiari and inferior caval vein syndromes due to membranous obstruction of the liver veins. Successful treatment with angioplasty and transcaval TIPS

    DEFF Research Database (Denmark)

    Holland-Fischer, Peter

    2004-01-01

    The case is presented of a 25-year-old Caucasian patient with Budd-Chiari syndrome due to membranous obstruction of the liver veins and inferior caval vein syndrome as a result of secondary hyperplasia of the caudate lobe of the liver, obstructing the caval vein. Diagnosis was established...... by intravascular pressure measurements, ultrasound examinations and caval and liver vein angiograms. Treatment consisting of stent placement in the outlet of a hepatic vein and subsequent transjugular intrahepatic porto-systemic shunt (TIPS) insertion via the caval vein was successful. After 34 months of follow......-up the stents remain open and the patient is symptom free. This successful combination of stent placement and TIPS has not been described before. The case report is followed by a review of the literature on the use of angioplasty in short hepatic vein stenosis and TIPS in Budd-Chiari syndrome. It is concluded...

  6. Risk of spontaneous pneumothorax due to air travel and diving in patients with Birt-Hogg-Dubé syndrome.

    Science.gov (United States)

    Johannesma, P C; van de Beek, I; van der Wel, J W T; Paul, M A; Houweling, A C; Jonker, M A; van Waesberghe, J H T M; Reinhard, R; Starink, Th M; van Moorselaar, R J A; Menko, F H; Postmus, P E

    2016-01-01

    Birt-Hogg-Dubé syndrome is an autosomal dominant disorder characterized by skin fibrofolliculomas, lung cysts, spontaneous pneumothorax and renal cell cancer due to germline folliculin (FLCN) mutations (Menko et al. in Lancet Oncol 10(12):1199-1206, 2009). The aim of this study was to evaluate the incidence of spontaneous pneumothorax in patients with BHD during or shortly after air travel and diving. A questionnaire was sent to a cohort of 190 BHD patients and the medical files of these patients were evaluated. The diagnosis of BHD was confirmed by FLCN mutations analysis in all patients. We assessed how many spontaneous pneumothoraces (SP) occurred within 1 month after air travel or diving. In total 158 (83.2 %) patients returned the completed questionnaire. A total of 145 patients had a history of air travel. Sixty-one of them had a history of SP (42.1 %), with a mean of 2.48 episodes (range 1-10). Twenty-four (35.8 %) patients had a history of pneumothorax on both sides. Thirteen patients developed SP < 1 month after air travel (9.0 %) and two patients developed a SP < 1 month after diving (3.7 %). We found in this population of BHD patients a pneumothorax risk of 0.63 % per flight and a risk of 0.33 % per episode of diving. Symptoms possible related to SP were perceived in 30 patients (20.7 %) after air travel, respectively in ten patients (18.5 %) after diving. Based on the results presented in this retrospective study, exposure of BHD patients to considerable changes in atmospheric pressure associated with flying and diving may be related to an increased risk for developing a symptomatic pneumothorax. Symptoms reported during or shortly after flying and diving might be related to the early phase of pneumothorax. An individualized advice should be given, taking also into account patients' preferences and needs.

  7. Coexisting diseases modifying each other’s presentation - lack of growth failure in Turner syndrome due to the associated pituitary gigantism

    Directory of Open Access Journals (Sweden)

    Dragović Tamara

    2016-01-01

    Full Text Available Introduction. Turner syndrome presents with one of the most frequent chromosomal aberrations in female, typically presented with growth retardation, ovarian insufficiency, facial dysmorphism, and numerous other somatic stigmata. Gigantism is an extremely rare condition resulting from an excessive growth hormone (GH secretion that occurs during childhood before the fusion of epiphyseal growth plates. The major clinical feature of gigantism is growth acceleration, although these patients also suffer from hypogonadism and soft tissue hypertrophy. Case report. We presented a girl with mosaic Turner syndrome, delayed puberty and normal linear growth for the sex and age, due to the simultaneous GH hypersecretion by pituitary tumor. In the presented case all the typical phenotypic stigmata related to Turner syndrome were missing. Due to excessive pituitary GH secretion during the period while the epiphyseal growth plates of the long bones are still open, characteristic stagnation in longitudinal growth has not been demonstrated. The patient presented with delayed puberty and primary amenorrhea along with a sudden appearance of clinical signs of hypersomatotropinism, which were the reasons for seeking medical help at the age of 16. Conclusion. Physical examination of children presenting with delayed puberty but without growth arrest must include an overall hormonal and genetic testing even in the cases when typical clinical presentations of genetic disorder are absent. To the best of our knowledge, this is the first reported case of simultaneous presence of Turner syndrome and gigantism in the literature.

  8. Coexisting diseases modifying each other’s presentation - lack of growth failure in Turner syndrome due to the associated pituitary gigantism.

    Science.gov (United States)

    Dragović, Tamara; Đuran, Zorana; Jelić, Svetlana; Marinković, Dejan; Kiković, Saša; Kuzmić-Janković, Snežana; Hajduković, Zoran

    2016-10-01

    Turner syndrome presents with one of the most frequent chromosomal aberrations in female, typically presented with growth retardation, ovarian insufficiency, facial dysmorphism, and numerous other somatic stigmata. Gigantism is an extremely rare condition resulting from an excessive growth hormone (GH) secretion that occurs during childhood before the fusion of epiphyseal growth plates. The major clinical feature of gigantism is growth acceleration, although these patients also suffer from hypogonadism and soft tissue hypertrophy. We presented a girl with mosaic Turner syndrome, delayed puberty and normal linear growth for the sex and age, due to the simultaneous GH hypersecretion by pituitary tumor. In the presented case all the typical phenotypic stigmata related to Turner syndrome were missing. Due to excessive pituitary GH secretion during the period while the epiphyseal growth plates of the long bones are still open, characteristic stagnation in longitudinal growth has not been demonstrated. The patient presented with delayed puberty and primary amenorrhea along with a sudden appearance of clinical signs of hypersomatotropinism, which were the reasons for seeking medical help at the age of 16. Physical examination of children presenting with delayed puberty but without growth arrest must include an overall hormonal and genetic testing even in the cases when typical clinical presentations of genetic disorder are absent. To the best of our knowledge, this is the first reported case of simultaneous presence of Turner syndrome and gigantism in the literature.

  9. Brown-Sequard syndrome caused by hyperextension in a patient with atlantoaxial subluxation due to an os odontoideum.

    Science.gov (United States)

    Lee, Dong-Yeong; Jeong, Soon-Taek; Lee, Tae-Ho; Kim, Dong-Hee

    2017-11-23

    Brown-Sequard syndrome is an uncommon complication of atlantoaxial arthrodesis. A 50-year-old female visited our emergency department after falling from a ladder. Radiologic evaluations revealed chronic C1-2 instability with acute spinal cord injury. The day after atlantoaxial fusion was performed, she developed left-sided motor weakness and the loss of right-sided pain and temperature sensation. Based on physical examination and radiologic findings, we diagnosed her as having Brown-Sequard syndrome. Spine surgeons performing this procedure should therefore consider Brown-Sequard syndrome if a patient displays signs of postoperative hemiplegia. Copyright © 2017 Turkish Association of Orthopaedics and Traumatology. Production and hosting by Elsevier B.V. All rights reserved.

  10. Endotracheal tubes and fluid aspiration: an in vitro evaluation of new cuff technologies.

    Science.gov (United States)

    Mariyaselvam, Maryanne Z; Marsh, Lucy L; Bamford, Sarah; Smith, Ann; Wise, Matt P; Williams, David W

    2017-03-04

    Aspiration of subglottic secretions past the endotracheal tube (ETT) cuff is a prerequisite for developing ventilator-associated pneumonia (VAP). Subglottic secretion drainage (SSD) ETTs reduce aspiration of subglottic secretions and have demonstrated lower VAP rates. We compared the performance of seven SSD ETTs against a non-SSD ETT in preventing aspiration below inflated cuffs. ETTs were positioned vertically in 2 cm diameter cylinders. Four ml of a standard microbial suspension was added above inflated cuffs. After 1 h, aspiration was measured and ETTs demonstrating no leakage were subjected to rotational movement and evaluation over 24 h. Collected aspirated fluid was used to inoculate agar media and incubated aerobically at 37 °C for 24 h. The aspiration rate, volume and number of microorganisms that leaked past the cuff was measured. Experiments were repeated (×10) for each type of ETT, with new ETTs used for each repeat. Best performing ETTs were then tested in five different cylinder diameters (1.6, 1.8, 2.0, 2.2 and 2.4 cm). Experiments were repeated as above using sterile water. Volume and time taken for aspiration past the cuff was measured. Experiments were repeated (×10) for each type of ETT. Results were analysed using non-parametric tests for repeated measures. The PneuX ETT prevented aspiration past the cuff in all experiments. All other ETTs allowed aspiration, with considerable variability in performance. The PneuX ETT was statistically superior in reducing aspiration compared to the SealGuard (p aspiration across the range of diameters compared to the SealGuard (p aspiration, relating to cuff material and design. Variability in performance was likely due to the random manner in which involutional folds form in the inflated ETT cuff. The PneuX ETT was the only ETT able to consistently prevent aspiration past the cuff in all experiments.

  11. Respiratory Failure due to Severe Obesity and Kyphoscoliosis in a 24-Year-Old Male with Molecularly Confirmed Prader-Willi Syndrome in Tertiary Hospital in Northern Tanzania

    Directory of Open Access Journals (Sweden)

    Elichilia R. Shao

    2017-01-01

    Full Text Available Obesity, mild intellectual disability, hypotonia, poor sucking, cryptorchidism in males, hypogonadism, and kyphoscoliosis are common features of Prader-Willi syndrome (PWS. We report a case who had severe respiratory complications due to extreme obesity and kyphoscoliosis, which are important causes of morbidity and mortality, and discuss management. Furthermore, this is the first molecularly confirmed PWS case in Sub-Saharan Africa outside South Africa.

  12. [Value of aspiration biopsy of subcutaneous fat in amyloidosis].

    Science.gov (United States)

    Ponce, P; Carvalho, F; Coelho, A

    1986-01-01

    Fine-needle aspiration of subcutaneous fat (FNAF) was performed in 24 patients, 12 with previously diagnosed amyloidosis presenting with proteinuria or nephrotic syndrome, and 12 presenting a nephrotic syndrome without amyloidosis on renal biopsy. FNAF was positive in 10 of 12 patients with amyloidosis (sensitivity: 83%) and negative in 12 of 12 patients with non-amyloid nephrotic syndrome (specificity: 100%). Considering a 2.5 to 10% prevalence of amyloidosis in adult patients with proteinuria or nephrotic syndrome, a positive FNAF is diagnostic of amyloidosis, and a negative FNAF rules out the diagnosis with a probability of 98 to 99%. FNAF is a simple and safe method which can be useful in patients who cannot undergo a renal biopsy.

  13. Delayed diagnosis of Birt-Hogg-Dubé syndrome due to marked intrafamilial clinical variability: a case report.

    Science.gov (United States)

    Sattler, E C; Steinlein, O K

    2018-03-16

    Birt-Hogg-Dubé syndrome is a genetic syndrome caused by mutations in the FLCN gene. The main symptoms are lung bullae and pneumothorax, benign and malignant kidney tumors, and facial fibrofolliculoma. The risk of pneumothorax is considerable between ages 20-40 years, but decreases markedly after this age range and first-time pneumothorax after age 50 years is rare. Fibrofolliculomas usually occur between ages 35 and 45 years, while the risk for kidney cancer increases steadily with age, starting in young adulthood. However, we demonstrate here that within the same family patients might develop symptoms significantly before or after the usual age range, obscuring the typical clinical pattern and delaying diagnosis. The 43 year old index patient had a history of lung bullae and recurrent pneumothoraces starting 14 years earlier. His father (age 83 years) and one of the paternal uncles experienced their first pneumothorax unusually late after the age of 60 years. The uncle subsequently had four more pneumothoraces, and was diagnosed with kidney in his early 70s. Considerable differences in age of onset were also observed with regard to facial fibrofolliculomas that both paternal uncles developed very early around age 20 years, but which the father only started to show in his eighth decade. Birt-Hogg-Dubé syndrome was finally diagnosed when the index patient started to develop fibrofolliculomas within the typical age range. The family described here illustrates that Birt-Hogg-Dubé syndrome can be difficult to recognize, if presenting with considerable intrafamilial clinical variability. With a life-time kidney cancer risk of about 14-35% the consequences of delayed diagnosis might be grave for the affected family members. The possibility of Birt-Hogg-Dubé syndrome should therefore be taken into consideration in apparently sporadic patients presenting with lung bullae and pneumothorax.

  14. Lifelong Learning: Capabilities and Aspirations

    Science.gov (United States)

    Ilieva-Trichkova, Petya

    2016-01-01

    The present paper discusses the potential of the capability approach in conceptualizing and understanding lifelong learning as an agency process, and explores its capacity to guide empirical studies on lifelong learning. It uses data for 20 countries from the Adult Education Survey (2007; 2011) and focuses on aspirations for lifelong learning. The…

  15. Meconium aspiration in South Africa

    African Journals Online (AJOL)

    This retrospective study of 569 cases of meconium aspiration from 11 institutions in South Africa reveals a high incidence varying from 4 to 11/1 000 and a mortality rate of. 12%. Mortality was significantly related to the degree of asphyxia at birth. Twenty-five per cent of the babies. (136/569) required intensive care and 36% ...

  16. Massive neonatal adrenal enlargement due to cytomegaly, persistence of the transient cortex, and hyperplasia of the permanent cortex: findings in Cushing syndrome associated with hemihypertrophy.

    Science.gov (United States)

    Carney, J Aidan; Ho, Josephine; Kitsuda, Kazuteru; Young, William F; Stratakis, Constantine A

    2012-10-01

    Described in this article is the massive enlargement of both adrenal glands in 3 newborns-2 girls and 1 boy. Two had hemihypertrophy and other congenital abnormalities but no identified genetic mutation; the third had genetically proven Beckwith-Wiedemann syndrome. Two had severe Cushing syndrome, the third had hypercortisolemia but no clinical Cushing syndrome. Bilateral adrenalectomy cured Cushing syndrome in the 2 with severe symptoms; total adrenal weight in these patients was 44 and 53 g, respectively. Unilateral adrenalectomy was performed in the third patient: the gland weighed 52 g; postoperatively, the patient's hypercortisolemia normalized, and, concomitantly, the enlarged contralateral adrenal gland had a 5-fold decrease in size with slight enlargement 6 years postoperatively. Microscopically, the 3 patients had similar pathology: massive adrenal enlargement due to a combination of cytomegaly, persistence of the transient cortex, and hyperplasia of the permanent cortex. The pathologic findings were most likely the result of the genetic mutation identified in 1 patient and of an unknown mutation in the remaining 2 patients.

  17. Aberrant muscle syndrome: hypertrophy of the hand and arm due to aberrant muscles with or without hypertrophy of the muscles.

    Science.gov (United States)

    Ogino, Toshihiko; Satake, Hiroshi; Takahara, Masatoshi; Kikuchi, Noriaki; Watanabe, Tadayosi; Iba, Kousuke; Ishii, Seiichi

    2010-06-01

    Five patients were reported in our congenital anomaly registry who had six hands in total with muscular hyperplasia, aberrant muscles, ulnar drift of the fingers in the metacarpophalangeal (MP) joints, flexion contractures of the MP joints, and enlargement of the metacarpal spaces. Thirty patients with unilateral involvement of this condition have been reported previously. We reviewed these cases and found that the condition varied in severity and that it was reported using different names. However, this condition seems different from true macrodactyly and multiple camptodactyly, including windblown hand, and seems to be an isolated entity of congenital upper limb anomaly. The authors recommend 'aberrant muscle syndrome' or 'accessory muscle syndrome' as a diagnostic name, because this seems to be the most common pathological finding in this condition.

  18. A novel fibrillin-1 mutation in an egyptian marfan family: A proband showing nephrotic syndrome due to focal segmental glomerulosclerosis

    Directory of Open Access Journals (Sweden)

    Mohammad Al-Haggar

    2017-01-01

    Full Text Available Marfan syndrome (MFS, the founding member of connective tissue disorder, is an autosomal dominant disease; it is caused by a deficiency of the microfibrillar protein fibrillin-1 (FBN1 and characterized by involvement of three main systems; skeletal, ocular, and cardiovascular. More than one thousand mutations in FBN1 gene on chromosome 15 were found to cause MFS. Nephrotic syndrome (NS had been described in very few patients with MFS being attributed to membranoproliferative glomerulonephritis secondary to infective endocarditis. Focal segmental glomerulosclerosis (FSGS had been reported in NS in conjunction with MFS without confirming the diagnosis by mutational analysis of FBN1. We hereby present an Egyptian family with MFS documented at the molecular level; it showed a male proband with NS secondary to FSGS, unfortunately, we failed to make any causal link between FBN dysfunction and FSGS. In this context, we review the spectrum of renal involvements occurring in MFS patients.

  19. Telomeres in ICF syndrome cells are vulnerable to DNA damage due to elevated DNA:RNA hybrids

    Science.gov (United States)

    Sagie, Shira; Toubiana, Shir; Hartono, Stella R.; Katzir, Hagar; Tzur-Gilat, Aya; Havazelet, Shany; Francastel, Claire; Velasco, Guillaume; Chédin, Frédéric; Selig, Sara

    2017-01-01

    DNA:RNA hybrids, nucleic acid structures with diverse physiological functions, can disrupt genome integrity when dysregulated. Human telomeres were shown to form hybrids with the lncRNA TERRA, yet the formation and distribution of these hybrids among telomeres, their regulation and their cellular effects remain elusive. Here we predict and confirm in several human cell types that DNA:RNA hybrids form at many subtelomeric and telomeric regions. We demonstrate that ICF syndrome cells, which exhibit short telomeres and elevated TERRA levels, are enriched for hybrids at telomeric regions throughout the cell cycle. Telomeric hybrids are associated with high levels of DNA damage at chromosome ends in ICF cells, which are significantly reduced with overexpression of RNase H1. Our findings suggest that abnormally high TERRA levels in ICF syndrome lead to accumulation of telomeric hybrids that, in turn, can result in telomeric dysfunction. PMID:28117327

  20. Evaluation of the natural history of patients who aspirate.

    Science.gov (United States)

    Bock, Jonathan M; Varadarajan, Varun; Brawley, Mary C; Blumin, Joel H

    2017-12-01

    The natural clinical progression of aspiration to eventual pulmonary compromise is not well understood. We hypothesized that dietary modification recommendations, Penetration-Aspiration Scale (PAS) score, and dysphagia etiology would be associated with changes in time to first pulmonary event and overall survival for patients with documented aspiration on radiologic testing. This study identified a cohort of patients with detectable unsensed penetration or aspiration on videofluoroscopic swallowing study (VFSS), and followed this cohort over time for development of pulmonary events and death. We then evaluated the association of aspiration severity and dietary modification recommendations on incidence of these endpoints. Retrospective chart review. A total of 2,616 VFSS exam reports were reviewed from our institution performed between January 1, 2009 and December 31, 2010. Aspiration or unsensed penetration (PAS of 5 or greater) was detected in 564 (21.5%) of these patients, who were then included in the study cohort. Medical records were reviewed retrospectively for development of pulmonary events (pneumonia, pneumonitis, or other life-threatening pulmonary illness) and all-cause mortality for up to 54 months after initial VFSS. Univariate Kaplan-Meier analysis and multivariate Cox regression were performed for time to first pulmonary event and survival predicted by recommended diet, PAS score, and dysphagia etiology. Dysphagia etiology was highly associated with increased development of pulmonary events for some patients, especially those with generalized nonspecific dysphagia due to deconditioning or frailty (hazard ratio [HZ] vs. stroke 2.95, 95% confidence interval [CI]: 1.53-5.69, P = .001) and esophageal dysphagia (HZ: 2.66, 95% CI: 1.17-6.02, P = .019). Dysphagia etiology was also associated with increased mortality for patients with generalized nonspecific dysphagia due to deconditioning or frailty (HZ: 3.32, 95% CI: 2.0-5.52, P penetration or aspiration

  1. Rare form of autosomal dominant familial Cornelia de Lange syndrome due to a novel duplication in SMC3.

    Science.gov (United States)

    Infante, Elena; Alkorta-Aranburu, Gorka; El-Gharbawy, Areeg

    2017-08-01

    Clinical features are variable in patients with Cornelia de Lange syndrome (CdLS). Milder forms exist with structural maintenance of chromosomes 3 (SMC3) mutations. Inherited milder forms of CdLS are uncommon and may be missed if genetic testing is limited to Nipped-B-like protein (NIPBL) and SMC1A. Parental studies should be pursued if there is a history of learning disabilities and/or dysmorphic features.

  2. Variable penetrance of hypogonadism in a sibship with Kallmann syndrome due to a deletion of the KAL gene

    Energy Technology Data Exchange (ETDEWEB)

    Parenti, G.; Rizzolo, M.G.; Ghezzi, M. [Federico II University, Naples (Italy)] [and others

    1995-07-03

    We report on the clinical and molecular characterization of 3 sibs with X-linked ichthyosis and variable expression of Kallmann syndrome. One of the affected brothers had mild hyposmia and showed normal pubertal progression. However, we demonstrated the same partial deletion of the X-linked Kallmann gene, sparing the first exon in the mildly affected patient as well as in one of his severely affected brothers. 13 refs., 1 fig., 1 tab.

  3. The Role of Inspiratory Muscle Training in Sickle Cell Anemia Related Pulmonary Damage due to Recurrent Acute Chest Syndrome Attacks

    OpenAIRE

    Camc?o?lu, Burcu; Bo?nak-G??l?, Meral; Karadall?, M??errefe Nur; Ak?, ?ahika Zeynep; T?rk?z-Sucak, G?lsan

    2015-01-01

    Background. The sickling of red blood cells causes a constellation of musculoskeletal, cardiovascular, and pulmonary manifestations. A 32-year-old gentleman with sickle cell anemia (SCA) had been suffering from recurrent acute chest syndrome (ACS). Aim. To examine the effects of inspiratory muscle training (IMT) on pulmonary functions, respiratory and peripheral muscle strength, functional exercise capacity, and quality of life in this patient with SCA. Methods. Functional exercise capacity w...

  4. Mortality Due to Porcine Reproductive and Respiratory Syndrome Virus in Immunocompromised G?ttingen Minipigs (Sus scrofa domestica)

    OpenAIRE

    Pils, Marina C; Dreckmann, Karla; Jansson, Katharina; Glage, Silke; Held, Nadine; Sommer, Wiebke; L?nger, Florian; Avsar, Murat; Warnecke, Gregor; Bleich, Andr?

    2016-01-01

    Porcine reproductive and respiratory syndrome virus (PRRSV) infection was diagnosed in 6 G?ttingen minipigs (Sus scrofa domestica) with severe interstitial pneumonia. The virus was defined as a North American (NA) subtype virus, which is common in the commercial pig population and might be derived from a widely used attenuated live-virus vaccine in Europe. The ORF5 sequence of the isolated PRRSV was 98% identical to the vaccine virus. The affected pigs were part of a lung transplantation mode...

  5. Hypercalcemia due to Milk –Alkali Syndrome and Fracture-induced Immobilization in an Adolescent Boy with Hypoparathyroidism

    Science.gov (United States)

    Henry, Rohan K.; Gafni, Rachel I.

    2016-01-01

    Background Hypercalcemia of immobilization, while rare, may occur in adolescent boys after fracture. Although not fully understood, the mechanism appears to be related to bone turnover uncoupling, in part mediated by upregulation of RANKL. Animal studies suggest that parathyroidectomy suppresses RANKL-stimulated osteoclastogenesis in immobilized bone. Thus, immobilization-induced hypercalcemia should be uncommon in patients with hypoparathyroidism. Methods/Results We present a 15-year-old boy with well-controlled hypoparathyroidism who developed hypercalcemia and milk-alkali syndrome 5 weeks after sustaining a severe tibia/fibula fracture requiring bedrest. Milk-alkali syndrome (hypercalcemia, alkalosis, and renal insufficiency) results from chronic excessive ingestion of calcium and absorbable alkali. Prior to fracture, our patient had not experienced hypercalcemia despite high doses of supplements, necessary during puberty. Supplements were discontinued and his biochemistries normalized with saline diuresis and a dose of pamidronate. Alkaline phosphatase, which was low at presentation, returned to normal 5 weeks later with remobilization. Conclusions Fracture and immobilization caused acute suppression of bone formation with persistent bone resorption in this rapidly growing adolescent; continuation of carbonate-containing calcium supplements resulted in the milk-alkali syndrome. Therefore, close monitoring of serum calcium with adjustments in supplementation are indicated in immobilized patients with hypoparathyroidism. PMID:27184240

  6. Hypercalcemia due to Milk-Alkali Syndrome and Fracture-Induced Immobilization in an Adolescent Boy with Hypoparathyroidism.

    Science.gov (United States)

    Henry, Rohan K; Gafni, Rachel I

    2016-01-01

    Hypercalcemia of immobilization, while rare, may occur in adolescent boys after fracture. Although not fully understood, the mechanism appears to be related to bone turnover uncoupling, in part mediated by upregulation of RANKL. Animal studies suggest that parathyroidectomy suppresses RANKL-stimulated osteoclastogenesis in immobilized bone. Thus, immobilization-induced hypercalcemia should be uncommon in patients with hypoparathyroidism. We present a 15-year-old boy with well-controlled hypoparathyroidism who developed hypercalcemia and milk-alkali syndrome 5 weeks after sustaining a severe tibia/fibula fracture requiring bedrest. Milk-alkali syndrome (hypercalcemia, alkalosis, and renal insufficiency) results from chronic excessive ingestion of calcium and absorbable alkali. Prior to fracture, our patient had not experienced hypercalcemia despite high doses of supplements, necessary during puberty. Supplements were discontinued and his biochemistries normalized with saline diuresis and a dose of pamidronate. Alkaline phosphatase, which was low at presentation, returned to normal 5 weeks later with remobilization. Fracture and immobilization caused acute suppression of bone formation with persistent bone resorption in this rapidly growing adolescent; continuation of carbonate-containing calcium supplements resulted in the milk-alkali syndrome. Therefore, close monitoring of serum calcium with adjustments in supplementation are indicated in immobilized patients with hypoparathyroidism. © 2016 S. Karger AG, Basel.

  7. Comparing effects of Beractant and Poractant alfa in decreasing mortality rate due to respiratory distress syndrome in premature infants

    Directory of Open Access Journals (Sweden)

    Saeidi R

    2011-02-01

    Full Text Available "nBackground: Exogenous natural and synthetic surfactants is a rescue treatment for respiratory distress syndrome (RDS. The goals of the study were to compare the clinical response and side-effects of two frequently used surfactants, poractant alfa (Curosurf and beractant (Survanta, for the treatment of respiratory distress syndrome in preterm infants."n "nMethods: This clinical trial study was performed during a two-year period in the Neonatal Intensive Care Unit of Ghaem Hospital in Mashhad, Iran. Sample size calculated by a 95% confidence and power of 80, included 104 premature neonates, 74 in survanta and 30 in curosurf groups. The level of statistical significance was considered to be < 0.05."n "nResults: There were no statistically significant differences between the infants treated by survanta or cursurf groups regarding their mean gestational age (30.58 Vs. 29.00 weeks and birth weight (1388 Vs. 1330 g, (p=0.3 There were also no significant differences between the two groups regarding incidences of broncho- pulmonary dysplasia (BPD (40.5% Vs. 40%, intraventricular hemorrhage (IVH grades III/IV (13.5% Vs. 13.3%, pneumothorax (both 20%, patent ductus arteriosus (PDA (28/3% Vs. 20% or death (28% Vs. 26.6% on the 28th day postpartum."n "nConclusion: This study showed that survanta and curosurf had similar therapeutic effects in the treatment of neonatal respiratory distress syndrome.

  8. The Hierarchical Personality Structure of Aspiring Creative Writers

    Science.gov (United States)

    Maslej, Marta M.; Rain, Marina; Fong, Katrina; Oatley, Keith; Mar, Raymond A.

    2014-01-01

    Empirical studies of personality traits in creative writers have demonstrated mixed findings, perhaps due to issues of sampling, measurement, and the reporting of statistical information. The goal of this study is to quantify the personality structure of aspiring creative writers according to a modern hierarchal model of trait personality. A…

  9. Fine Needle Aspiration: A simple and convenient alternative for ...

    African Journals Online (AJOL)

    Nine patients followed up to hospital for other medical problems unrelated to ganglion cysts and only one patient had to undergo surgical excision due to the large size of the cyst. No patient returned with a recurrence. Conclusion: Fine needle aspiration is a simple, non traumatic, inexpensive and low risk alternative method ...

  10. Desired Mobility or Satisfied Immobility? Migratory Aspirations among Knowledge Workers

    Science.gov (United States)

    Ferro, Anna

    2006-01-01

    Among the aspects discussed within the globalisation process, the international mobility of professional workers assumes considerable relevance. This paper focuses on migratory aspirations among knowledge workers within the context of economic globalisation and market restructuring in Romania. Due to a lack of literature dealing with these issues,…

  11. Aspirations of Latina Adolescent Suicide Attempters

    Science.gov (United States)

    Hausmann-Stabile, Carolina; Gulbas, Lauren; Zayas, Luis H.

    2013-01-01

    Parents' aspirations and expectations are communicated to their offspring. Children internalize their parents' aspirations and accept some of the expectations while rejecting others, all part of the developmental process and identity-consolidation. When the aspirations and expectations of youth and parents are incongruent, the outcomes…

  12. Empowering change: The effects of energy provision on individual aspirations in slum communities

    International Nuclear Information System (INIS)

    Parikh, Priti; Chaturvedi, Sankalp; George, Gerard

    2012-01-01

    This paper discusses the role of energy provision in influencing the social aspirations of people living in slums. We examine factors that influence the shift in aspirations in five slum settlements using data from 500 interviews conducted in serviced and non-serviced slums from the state of Gujarat in India. The non-serviced slums did not have access to basic services namely water, sanitation, energy, roads, solid waste and rainwater management. We find empirical evidence which suggests that when basic infrastructure provisions are met, slum dwellers shift their focus from lower order aspirations to the higher order aspirations like health, education, housing and land ownership. We argue that energy provision enhances productivity and enables slum dwellers to shift their aspirations upwards. Furthermore, we test the effect of work days lost due to illness on the relationship between higher order aspirations and aspirations for energy provision. When provision of energy is low, higher work day loss dampens higher order aspirations. For policy makers, this study highlights the critical link between the infrastructure services preferred by slum dwellers and their social aspirations for growth. - Highlights: ► We examine the role of energy provision in influencing social aspirations of people living in slums. ► 500 household interviews conducted in five Indian slum settlements used for the analysis. ► Shift in slum dwellers aspirations from non-serviced to serviced conditions examined using Herzberg’s theory. ► Link between energy provision and higher order aspirations established through a regression model. ► Policy recommendation is to encourage investment in infrastructure services as a driver for development.

  13. Nance-Horan syndrome in females due to a balanced X;1 translocation that disrupts the NHS gene: Familial case report and review of the literature.

    Science.gov (United States)

    Gómez-Laguna, Laura; Martínez-Herrera, Alejandro; Reyes-de la Rosa, Alejandra Del Pilar; García-Delgado, Constanza; Nieto-Martínez, Karem; Fernández-Ramírez, Fernando; Valderrama-Atayupanqui, Tania Yanet; Morales-Jiménez, Ariadna Berenice; Villa-Morales, Judith; Kofman, Susana; Cervantes, Alicia; Morán-Barroso, Verónica Fabiola

    2018-01-01

    The Nance-Horan syndrome is an X-linked disorder characterized by congenital cataract, facial features, microcornea, microphthalmia, and dental anomalies; most of the cases are due to NHS gene mutations on Xp22.13. Heterozygous carrier females generally present less severe features, and up to 30% of the affected males have intellectual disability. We describe two patients, mother and daughter, manifesting Nance-Horan syndrome. The cytogenetic and molecular analyses demonstrated a 46,X,t(X;1)(p22.13;q22) karyotype in each of them. No copy-number genomic imbalances were detected by high-density microarray analysis. The mother had a preferential inactivation of the normal X chromosome; expression analysis did not detect any mRNA isoform of NHS. This is the first report of Nance-Horan syndrome due to a skewed X chromosome inactivation resulting from a balanced translocation t(X;1) that disrupts the NHS gene expression, with important implications for clinical presentation and genetic counseling.

  14. Recurrent Cholangitis by Biliary Stasis Due to Non-Obstructive Afferent Loop Syndrome After Pylorus-Preserving Pancreatoduodenectomy: Report of a Case

    Science.gov (United States)

    Sanada, Yukihiro; Yamada, Naoya; Taguchi, Masanobu; Morishima, Kazue; Kasahara, Naoya; Kaneda, Yuji; Miki, Atsushi; Ishiguro, Yasunao; Kurogochi, Akira; Endo, Kazuhiro; Koizumi, Masaru; Sasanuma, Hideki; Fujiwara, Takehito; Sakuma, Yasunaru; Shimizu, Atsushi; Hyodo, Masanobu; Sata, Naohiro; Yasuda, Yoshikazu

    2014-01-01

    We report a 71-year-old man who had undergone pylorus-preserving pancreatoduodenectomy (PPPD) using PPPD-IV reconstruction for cholangiocarcinoma. For 6 years thereafter, he had suffered recurrent cholangitis, and also a right liver abscess (S5/8), which required percutaneous drainage at 9 years after PPPD. At 16 years after PPPD, he had been admitted to the other hospital because of acute purulent cholangitis. Although medical treatment resolved the cholangitis, the patient was referred to our hospital because of dilatation of the intrahepatic biliary duct (B2). Peroral double-balloon enteroscopy revealed that the diameter of the hepaticojejunostomy anastomosis was 12 mm, and cholangiography detected intrahepatic stones. Lithotripsy was performed using a basket catheter. At 1 year after lithotripsy procedure, the patient is doing well. Hepatobiliary scintigraphy at 60 minutes after intravenous injection demonstrated that deposit of the tracer still remained in the upper afferent loop jejunum. Therefore, we considered that the recurrent cholangitis, liver abscess, and intrahepatic lithiasis have been caused by biliary stasis due to nonobstructive afferent loop syndrome. Biliary retention due to nonobstructive afferent loop syndrome may cause recurrent cholangitis or liver abscess after hepaticojejunostomy, and double-balloon enteroscopy and hepatobiliary scintigraphy are useful for the diagnosis of nonobstructive afferent loop syndrome. PMID:25058778

  15. Adjuvant spinal cord stimulation improves wound healing of peripheral tissue loss due to steal syndrome of the hand: clinical challenge treating a difficult case.

    Science.gov (United States)

    De Caridi, Giovanni; Massara, Mafalda; Benedetto, Filippo; Tripodi, Paolo; Spinelli, Francesco; David, Antonio; Grande, Raffaele; Butrico, Lucia; Serra, Raffaele; de Franciscis, Stefano

    2016-02-01

    Hand ischaemia due to arterial steal syndrome is an infrequent, but potentially serious complication of arteriovenous fistula (AVF) for haemodialysis. We present a case of hand ischaemia caused by steal syndrome in a 69-year-old haemodialysis patient, 10 months after a brachiobasilic fistula creation. The patient underwent multiple operations without resolution of hand pain and tissue loss. The implantation of an adjuvant cervical spinal cord stimulator allowed the patient to obtain complete hand pain relief and wound healing. Probably, the diffuse microangiopathy typical of haemodialysis patients could be responsible for the persistence of ischaemic signs and symptoms after a surgical revascularisation. The effect of sympathetic blockade and the subsequent improvement of the arterial blood flow and tissue oxygenation because of spinal cord stimulation (SCS) can be useful to achieve complete ischaemic pain relief in order to enhance wound healing and to limit the tissue loss. In conclusion, the association of cervical spinal cord stimulation and surgical revascularisation could represent a valid option to treat a critical upper limb ischaemia following steal syndrome due to AVF. © 2014 The Authors. International Wound Journal © 2014 Medicalhelplines.com Inc and John Wiley & Sons Ltd.

  16. Impaired riboflavin transport due to missense mutations in SLC52A2 causes Brown-Vialetto-Van Laere syndrome

    OpenAIRE

    Haack, Tobias B.; Makowski, Christine; Yao, Yoshiaki; Graf, Elisabeth; Hempel, Maja; Wieland, Thomas; Tauer, Ulrike; Ahting, Uwe; Mayr, Johannes A.; Freisinger, Peter; Yoshimatsu, Hiroki; Inui, Ken; Strom, Tim M.; Meitinger, Thomas; Yonezawa, Atsushi

    2012-01-01

    Brown-Vialetto-Van Laere syndrome (BVVLS [MIM 211530]) is a rare neurological disorder characterized by infancy onset sensorineural deafness and ponto-bulbar palsy. Mutations in SLC52A3 (formerly C20orf54), coding for riboflavin transporter 2 (hRFT2), have been identified as the molecular genetic correlate in several individuals with BVVLS. Exome sequencing of just one single case revealed that compound heterozygosity for two pathogenic mutations in the SLC52A2 gene coding for riboflavin tran...

  17. Recurrent burner syndrome due to presumed cervical spine osteoblastoma in a collision sport athlete – a case report

    Directory of Open Access Journals (Sweden)

    Goins Maurice L

    2007-06-01

    Full Text Available Abstract We present a case of a 35-year-old active rugby player presenting with a history of recurrent burner syndrome thought secondary to an osteoblastoma involving the posterior arch of the atlas. Radiographically, the lesion had features typical for a large osteoid osteoma or osteoblastoma, including osseous expansion, peripheral sclerosis and bony hypertrophy, internal lucency, and even suggestion of a central nidus. The patient subsequently underwent an en bloc resection of the posterior atlas via a standard posterior approach. The surgery revealed very good clinical results. In this report, we will discuss in detail, the presentation, treatment, and return to play recommendations involving this patient.

  18. Controversies in the differential diagnosis of Brown-Sequard syndrome due to cervical spinal disease from stroke: A case series

    Directory of Open Access Journals (Sweden)

    Vaner Koksal, M.D.

    2017-09-01

    Full Text Available Stroke is generally considered to be the first preliminary diagnosis in patients presenting with acute hemiparesia in the emergency department. But rarely in unexpected spontaneous neurological pathologies that may lead to hemiparesis. The data from 8 non-traumatic patients who underwent surgical treatment for brown-sequard syndrome (BSS were reviewed retrospectively. All patients were initially misdiagnosed with strokes. Two of the patients had spinal canal stenosis, two had spinal epidural hematomas, one had an ossified herniated disc and three had soft herniated discs. None of the patients complained of significant pain at the initial presentation. All of the patients had a mild sensory deficit that was initially unrecognized. The pain of the patients began to become evident after hospitalization and, patients transferred to neurosurgery department. Cervical spinal pathologies compressing the corticospinal tract in one-half of the cervical spinal canal may present with only hemiparesis, without neck and radicular pain. If it's too late, permanent neurological damage may become inevitable while it is a correctable pathology. Keywords: Brown-Sequard syndrome, Cervical cord, Herniated disc, Spinal epidural hematoma, Stroke

  19. Streptococcal toxic-shock syndrome due to Streptococcus dysgalactiae subspecies equisimilis in breast cancer-related lymphedema: a case report.

    Science.gov (United States)

    Sumazaki, Makoto; Saito, Fumi; Ogata, Hideaki; Yoshida, Miho; Kubota, Yorichika; Magoshi, Syunsuke; Kaneko, Hironori

    2017-07-14

    Breast cancer-related lymphedema often causes cellulitis and is one of the most common complications after breast cancer surgery. Streptococci are the major pathogens underlying such cellulitis. Among the streptococci, the importance of the Lancefield groups C and G is underappreciated; most cases involve Streptococcus dysgalactiae subspecies equisimilis. Despite having a relatively weak toxicity compared with group A streptococci, Streptococcus dysgalactiae subspecies equisimilis is associated with a mortality rate that is as high as that of group A streptococci in cases of invasive infection because Streptococcus dysgalactiae subspecies equisimilis mainly affects elderly individuals who already have various comorbidities. An 83-year-old Japanese woman with breast cancer-related lymphedema in her left upper limb was referred to our hospital with high fever and acute pain with erythema in her left arm. She showed septic shock with disseminated intravascular coagulation. Blood culture showed positive results for Streptococcus dysgalactiae subspecies equisimilis, confirming a diagnosis of streptococcal toxic-shock syndrome. She survived after successful intensive care. To the best of our knowledge, this case represents the first report of Streptococcus dysgalactiae subspecies equisimilis-induced streptococcal toxic-shock syndrome in a patient with breast cancer-related lymphedema. Breast cancer-related lymphedema is a common problem, and we must pay attention to invasive streptococcal soft tissue infections, particularly in elderly patients with chronic disease.

  20. Diagnosis of Familial Wolf-Hirschhorn Syndrome due to a Paternal Cryptic Chromosomal Rearrangement by Conventional and Molecular Cytogenetic Techniques

    Directory of Open Access Journals (Sweden)

    Carlos A. Venegas-Vega

    2013-01-01

    Full Text Available The use of conventional cytogenetic techniques in combination with fluorescent in situ hybridization (FISH and single-nucleotide polymorphism (SNP microarrays is necessary for the identification of cryptic rearrangements in the diagnosis of chromosomal syndromes. We report two siblings, a boy of 9 years and 9 months of age and his 7-years- and 5-month-old sister, with the classic Wolf-Hirschhorn syndrome (WHS phenotype. Using high-resolution GTG- and NOR-banding karyotypes, as well as FISH analysis, we characterized a pure 4p deletion in both sibs and a balanced rearrangement in their father, consisting in an insertion of 4p material within a nucleolar organizing region of chromosome 15. Copy number variant (CNV analysis using SNP arrays showed that both siblings have a similar size of 4p deletion (~6.5 Mb. Our results strongly support the need for conventional cytogenetic and FISH analysis, as well as high-density microarray mapping for the optimal characterization of the genetic imbalance in patients with WHS; parents must always be studied for recognizing cryptic balanced chromosomal rearrangements for an adequate genetic counseling.

  1. Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome (APECED) due to AIRET16M mutation in a consanguineous Greek girl.

    Science.gov (United States)

    Kollios, Konstantinos; Tsolaki, Anastasia; Antachopoulos, Charalampos; Moix, I; Morris, Michael A; Papadopoulou, Maria; Roilides, Emmanuel

    2011-01-01

    Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome (APECED) or autoimmune polyendocrine syndrome type 1 (APS-1) is a rare autosomal recessive disease caused by mutations of the AutoImmune REgulator (AIRE) gene, an important mediator of tolerance to self-antigens. It is characterized by two out of three major components: chronic mucocutaneous candidiasis, hypoparathyroidism and Addison's disease. We present an 11-year-old girl suffering from recurrent episodes of mucocutaneous candidiasis and onychomycosis from 1 to 6 years of age, and transient alopecia at the age of 4 years. Hypoparathyroidism and dental enamel hypoplasia were diagnosed at 8 years. Autoantibodies to thyroid and adrenal glands were not detected and all other endocrine functions have remained normal. Genetic analysis revealed that the patient was homozygous for the mutation T16M in exon 1 of the AIRE gene (p.T16M, c.47C>T). This is the first APECED case reported for carrying this mutation in homozygous form. Parents were third cousins and heterozygous carriers of this mutation.

  2. [Foreign body aspiration in children].

    Science.gov (United States)

    Ibarz, J A Esteban; Samitier, A Sáinz; Alvira, R Delgado; Prades, P Burgués; Martínez-Pardo, N González; Pollina, J Elías

    2007-01-01

    The aim to this study is evaluate the history, symptoms, radiographic and endoscopic findings in 420 children who were admitted for suspected foreign body aspiration in our hospital between 1972 and 2005. In 13 children we didn't find foreign body and in 16 children the foreign bodies were lodged in larynx. The mean age was 33 months. The medical history, phisical exploration, auscultation and radiological findings were positive in 91,4%, 78,3%, 91,6% and 81%. The frecuency or foreign body aspiration is undervaluate and sometimes is excluded as diagnosis. Only 218 (51,9%) patients went to the hospital in the first 24 hours, although 87,8% of patients presented symptoms and 75,4% presented severe symptoms. Moreover the removed foreign bodies and suspected foreign bodies are the same in 82,95%. We think that bronchoscopy should by performed in all children who have had a choking episode.

  3. [Streptococcal Toxic Shock-like Syndrome due to Streptococcus suis Serotype 2 in a Japanese Pig Farmer].

    Science.gov (United States)

    Yamanaka, Atsushi; Shirahama, Tomohiro; Nishimura, Naoya; Ueda, Naoyasu; Himeji, Daisuke; Kawaguchi, Takeshi; Ueda, Akira

    2015-11-01

    Streptococcus suis is a major swine pathogen. It has recently been recognized as an emerging zoonosis that causes mainly meningitis and sepsis in human. In particular, toxic shock-like syndrome (TSLS) caused by this pathogen has a high mortality rate. However, misidentification of S. suis by conventional biochemical and commercial identification tests is not rare. The patient was a 71-year-old man who worked as a pig farmer who was admitted for fever, oliguria and subcutaneous hemorrhage. He was diagnosed as having septic shock and blood culture was positive for Gram-positive cocci, mainly diplococcus. This pathogen was identified with S. suis with using MALDI-TOF MS analysis, though a commercial Gram-Positive bacteria identification kit revealed viridans streptococci. His clinical features met the diagnostic criteria of TSLS, and ceftriaxone and clindamycin were administered. On admission day 28, he was discharged in good condition.

  4. Pleural empyema and streptococcal toxic shock syndrome due to Streptococcus pyogenes in a healthy Spanish traveler in Japan

    Directory of Open Access Journals (Sweden)

    Tetsuya Sakai

    2017-01-01

    Full Text Available Group A Streptococcus (GAS, Streptococcus pyogenes causes invasive infections including streptococcal toxic shock syndrome (STSS and local infections. To our knowledge, this is the first report of a case of an invasive GAS infection with pneumonia and pleural empyema (PE followed by STSS (disseminated intravascular coagulation [DIC] and acute renal insufficiency in a healthy male adult. He received combined supportive therapies of PE drainage, anti-DIC agent, hemodialysis, and antimicrobials and eventually made a clinical recovery. GAS isolated from PE was found to have emm1/speA genes, suggestive of a pathogenic strain. Clinicians should be aware of the possibility of this disease entity (pneumonia, PE, and STSS in healthy male adults as well as children and adult women.

  5. Delayed diagnosis of Herlyn-Werner-Wunderlich syndrome due to microperforation and pyocolpos in obstructed vaginal canal.

    Science.gov (United States)

    Wozniakowska, Ewa; Torres, Anna; Milart, Pawel; Wozniak, Slawomir; Czuczwar, Piotr; Szkodziak, Piotr; Paszkowski, Tomasz

    2014-08-01

    To present a rare anomaly consisting of uterus didelphys, longitudinal vaginal septum, obstructed hemivagina with pyocolpos, fistula to the open vaginal canal, and ipsilateral renal agenesis, referred as Herlyn-Werner-Wunderlich syndrome (HWWS). A 14-year-old girl with recurring purulent vaginal discharge lasting for a few months. Preoperative examination revealed one vaginal canal with one cervical opening on the right side. There was a fistula leading from the obstructed vaginal canal to the left vagina. Intravaginal ultrasound examination demonstrated a longitudinal vaginal septum and a closed pyocolpos on the right side. The longitudinal vaginal septum was excised by way of electrocauterization under direct vision. HWWS should be considered in the differential diagnosis in patients with uterus didelphys and unusual symptoms such as pyocolpos and vaginal discharge. Copyright © 2014 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  6. Iatrogenic Cushing's syndrome due to drug interaction between glucocorticoids and the ritonavir or cobicistat containing HIV therapies.

    Science.gov (United States)

    Elliot, Emilie R; Theodoraki, Aikaterini; Jain, Lakshmi R; Marshall, Neal J; Boffito, Marta; Baldeweg, Stephanie E; Waters, Laura J

    2016-10-01

    Ritonavir and cobicistat, used as pharmacokinetic enhancers in combination with some antiretrovirals (ARVs) for the treatment of HIV, are potent inhibitors of the CYP3A4 isoenzyme. Most glucocorticoids are metabolised via the CYP3A4 pathway and iatrogenic Cushing's syndrome (ICS), with possible secondary adrenal insufficiency (SAI), is a recognised complication following co-administration with ritonavir or cobicistat. A structured approach for identifying and managing potentially affected individuals has not been established.We systematically identified patients with ICS/SAI and found substantial heterogeneity in clinical practice across three large London HIV centres. While this significant drug interaction and its complications are now well-recognised, it is apparent that there is no standardised approach to management or guidance for the general physician. Here we describe the management of ICS/SAI in our current practice, review the available evidence and suggest practice recommendations. © Royal College of Physicians 2016. All rights reserved.

  7. Aspiration Pneumonia in a Pediatric Patient under General Anesthesia despite Adequate Preoperative Fasting

    Directory of Open Access Journals (Sweden)

    Sang-Il Yoon

    2015-11-01

    Full Text Available Aspiration pneumonia rarely occurs during general anesthesia; however, it can result in fatal pulmonary complications. To reduce aspiration pneumonia, a preoperative fasting time of 8 hours is recommended. A 4-year-old boy with ankyloglossia was scheduled for frenotomy. He completed preoperative fasting time and had no digestive symptoms. Pulmonary aspiration due to unexpected massive vomiting occurred during anesthesia induction. The patient’s airway was immediately secured by endotracheal tube. The vomitus in the airway tract was removed by fiberoptic bronchoscopy. Abdomen radiograph taken after this event showed paralytic ileus which can cause aspiration of gastric contents. We describe a case of pneumonia caused by aspiration of gastric contents in a pediatric patient who followed fasting instructions and who was scheduled for outpatient surgery.

  8. 22q11.2 Deletion Syndrome due to a Translocation t(6;22) in a Patient Conceived via in vitro Fertilization.

    Science.gov (United States)

    Gollo Dantas, Anelisa; Bortolai, Adriana; Moysés-Oliveira, Mariana; Takeno Herrero, Sylvia; Azoubel Antunes, Adriana; Tavares Costa-Carvalho, Beatriz; Ayres Meloni, Vera; Melaragno, Maria Isabel

    2016-02-01

    We report on a patient conceived via in vitro fertilization (IVF) with a 22q11.2 deletion due to an unusual unbalanced translocation involving chromosomes 6 and 22 in a karyotype with 45 chromosomes. Cytogenomic studies showed that the patient has a 3.3-Mb deletion of chromosome 22q and a 0.4-Mb deletion of chromosome 6p, which resulted in haploinsufficiency of the genes responsible for the 22q11.2 deletion syndrome and also of the IRF4 gene, a member of the interferon regulatory factor family of transcription factors, which is expressed in the immune system cells. The rearrangement could be due to the manipulation of the embryo or as a sporadic event unrelated to IVF. Translocation involving chromosome 22 in a karyotype with 45 chromosomes is a rare event, with no previous reports involving chromosomes 6p and 22q.

  9. Severe Uncompensated Metabolic Alkalosis due to Plasma Exchange in a Patient with Pulmonary-Renal Syndrome: A Clinician's Challenge.

    Science.gov (United States)

    Ijaz, Mohsin; Abbas, Naeem; Lvovsky, Dmitry

    2015-01-01

    Metabolic alkalosis secondary to citrate toxicity from plasma exchange is very uncommon in patients with normal renal function. In patients with advanced renal disease this can be a fatal event. We describe a case of middle-aged woman with Goodpasture's syndrome treated with plasma exchange who developed severe metabolic alkalosis. High citrate load in plasma exchange fluid is the underlying etiology. Citrate metabolism generates bicarbonate and once its level exceeds the excretory capacity of kidneys, the severe metabolic alkalosis ensues. Our patient presented with generalized weakness, fever, and oliguria and developed rapidly progressive renal failure. Patient had positive serology for antineutrophilic cytoplasmic antibodies myeloperoxidase (ANCA-MPO) and anti-glomerular basement membrane antibodies (anti-GBM). Renal biopsy showed diffuse necrotizing and crescentic glomerulonephritis with linear glomerular basement membrane staining. Patient did not respond to intravenous steroids. Plasma exchange was started with fresh frozen plasma but patient developed severe metabolic alkalosis. This metabolic alkalosis normalized with cessation of plasma exchange and initiation of low bicarbonate hemodialysis. ANCA-MPO and anti-GBM antibodies levels normalized within 2 weeks and remained undetectable at 3 months. Patient still required maintenance hemodialysis.

  10. Impaired riboflavin transport due to missense mutations in SLC52A2 causes Brown-Vialetto-Van Laere syndrome.

    Science.gov (United States)

    Haack, Tobias B; Makowski, Christine; Yao, Yoshiaki; Graf, Elisabeth; Hempel, Maja; Wieland, Thomas; Tauer, Ulrike; Ahting, Uwe; Mayr, Johannes A; Freisinger, Peter; Yoshimatsu, Hiroki; Inui, Ken; Strom, Tim M; Meitinger, Thomas; Yonezawa, Atsushi; Prokisch, Holger

    2012-11-01

    Brown-Vialetto-Van Laere syndrome (BVVLS [MIM 211530]) is a rare neurological disorder characterized by infancy onset sensorineural deafness and ponto-bulbar palsy. Mutations in SLC52A3 (formerly C20orf54), coding for riboflavin transporter 2 (hRFT2), have been identified as the molecular genetic correlate in several individuals with BVVLS. Exome sequencing of just one single case revealed that compound heterozygosity for two pathogenic mutations in the SLC52A2 gene coding for riboflavin transporter 3 (hRFT3), another member of the riboflavin transporter family, is also associated with BVVLS. Overexpression studies confirmed that the gene products of both mutant alleles have reduced riboflavin transport activities. While mutations in SLC52A3 cause decreased plasma riboflavin levels, concordant with a role of SLC52A3 in riboflavin uptake from food, the SLC52A2-mutant individual had normal plasma riboflavin concentrations, a finding in line with a postulated function of SLC52A2 in riboflavin uptake from blood into target cells. Our results contribute to the understanding of human riboflavin metabolism and underscore its role in the pathogenesis of BVVLS, thereby providing a rational basis for a high-dose riboflavin treatment.

  11. Trisomy 15 with loss of the paternal 15 as a cause of Prader-Willi syndrome due to maternal disomy

    Energy Technology Data Exchange (ETDEWEB)

    Cassidy, S.B.; Lai, Li-Wen; Erickson, R.P. (Univ. of Arizona College of Medicine, Tucson, AZ (United States)); Magnuson, L.; Thomas, E.; Herrmann, J. (Great Lakes Genetics, Milwaukee, AZ (United States)); Gendron, R. (Great Lakes Genetics, Kingsport, TN (United States))

    1992-10-01

    Uniparental disomy has recently been recognized to cause human disorders, including Prader-Willi syndrome (PWS). The authors describe a particularly instructive case which raises important issues concerning the mechanisms producing uniparental disomy and whose evaluation provides evidence that trisomy may precede uniparental disomy in a fetus. Chorionic villus sampling performed for advanced maternal age revealed trisomy 15 in all direct and cultured cells, though the fetus appeared normal. Chromosome analysis of amniocytes obtained at 15 wk was normal in over 100 cells studied. The child was hypotonic at birth, and high-resolution banding failed to reveal the deletion of 15q11-13, a deletion which is found in 50%-70% of patients with PWS. Over time, typical features of PWS developed. Molecular genetic analysis using probes for chromosome 15 revealed maternal disomy. Maternal nondisjunction with fertilization of a disomic egg by a normal sperm, followed by loss of the paternal 15, is a likely cause of confined placental mosaicism and uniparental disomy in this case of PWS, and advanced maternal age may be a predisposing factor. 38 refs., 3 figs., 2 tabs.

  12. Compartment syndrome like picture in metaphyseal comminuted fracture of tibia treated by locking plate due to tight closure

    Directory of Open Access Journals (Sweden)

    Prafulla Herode

    2013-01-01

    Full Text Available A 22-year-old male came to casualty on 5 th May 2012 after a fall from motorcycle. He complained of excruciating pain and swelling over right knee. There was an open wound of 7 × 2 cm over supra-patellar region and diffuse swelling over knee joint with severe tenderness over proximal aspect of right tibia. X-ray showed intra-articular fracture of proximal tibia extending to diaphysis classified as type 6 by Schatzker classification for proximal tibia, with fibula shaft transverse fracture. The skin over the fracture was contused. Debridement with primary wound closure was done in emergency. Skeletal traction was applied through a lower tibial Steinman pin. Patient was operated after 15 days when wound healed and swelling subsided. Locking plate was applied on medial aspect using Minimally invasive percutaneous plate osteosysthesis (MIPPO technique. Post-operatively over 4 hours patient developed severe pain and swelling in operated leg which mimicked compartment syndrome. Suture removal was done immediately in the ward from the distal aspect, which relieved the symptoms but lead to exposure of the plate. A rotational flap was done to cover the plate in coordination with a plastic surgeon on the next day.

  13. First evidence of Smith-Magenis syndrome in mother and daughter due to a novel RAI mutation.

    Science.gov (United States)

    Acquaviva, Fabio; Sana, Maria Elena; Della Monica, Matteo; Pinelli, Michele; Postorivo, Diana; Fontana, Paolo; Falco, Maria Teresa; Nardone, Anna Maria; Lonardo, Fortunato; Iascone, Maria; Scarano, Gioacchino

    2017-01-01

    Smith-Magenis syndrome (SMS) is a complex genetic disorder caused by interstitial 17p11.2 deletions encompassing multiple genes, including the retinoic acid induced 1 gene-RAI1-or mutations in RAI1 itself. The clinical spectrum includes developmental delay, cognitive impairment, and behavioral abnormalities, with distinctive physical features that become more evident with age. No patients have been reported to have had offspring. We here describe a girl with developmental delay, mainly compromising the speech area, and her mother with mild intellectual disabilities and minor dysmorphic features. Both had sleep disturbance and attention deficit disorder, but no other atypical behaviors have been reported. In both, CGH-array analysis detected a 15q13.3 interstitial duplication, encompassing CHRNA7. However, the same duplication has been observed in several, apparently healthy, maternal relatives. We, thus, performed a whole exome sequencing analysis, which detected a frameshift mutation in RAI1, de novo in the mother, and transmitted to her daughter. No other family members carried this mutation. This is the first report of an SMS patient having offspring. Our experience confirms the importance of searching for alternative causative genetic mechanisms in case of confounding/inconclusive findings such as a CGH-array result of uncertain significance. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  14. A Novel Porcine Model of Septic Shock Induced by Acute Respiratory Distress Syndrome due to Methicillin-resistant Staphylococcus aureus

    Directory of Open Access Journals (Sweden)

    Shuo Wang

    2017-01-01

    Conclusions: In the present study, we developed a novel porcine model of septic shock induced by ARDS due to severe MRSA pneumonia with characteristic hyperdynamic and hypodynamic phases in 24 h, which mimicked the hemodynamic changing of septic shock in human.

  15. Tracheobronchial Foreign Body Aspiration: Dental Prosthesis

    Directory of Open Access Journals (Sweden)

    Ataman Köse

    2014-01-01

    Full Text Available It is important to extract foreign bodies for avoiding life-threatening complications. They can lead to death if they are not treated. Different signs and symptoms could occur according to the complete or partial airway obstruction. Foreign body aspiration is a rare incident in adults. The organic foreign materials such as foods are found to be aspirated more commonly and are usually settled in the right bronchial system. However, dental prosthesis and teeth aspirations are rare in literature. In our study, a 52-year-old male patient who had aspirated the front part of his lower dental prosthesis accidentally is presented and the foreign body is extracted by using rigid bronchoscopy. There are many causes of aspiration but dental prosthetic aspirations should be kept in mind during sleep. For this reason, dental apparatus must be taken out while asleep.

  16. Early surfactant guided by lamellar body counts on gastric aspirate in very preterm infants

    DEFF Research Database (Denmark)

    Verder, Henrik Axel; Ebbesen, Finn Oluf; Fenger-Grøn, Jesper

    2013-01-01

    We have developed a rapid method, based on lamellar body counts (LBC) on gastric aspirate, for identifying newborns who will develop respiratory distress syndrome with a need for surfactant supplementation. Objective: We set out to test whether it was possible to improve the outcome when used in ...

  17. Metabolic syndrome as a risk factor for total hip or knee replacement due to primary osteoarthritis: a prospective cohort study (the HUNT study and the Norwegian Arthroplasty Register

    Directory of Open Access Journals (Sweden)

    Hellevik AI

    2018-01-01

    Full Text Available Alf Inge Hellevik,1,2 Marianne Bakke Johnsen,3,4 Arnulf Langhammer,1 Valborg Baste,5 Ove Furnes,6,7 Kjersti Storheim,3,4 John Anker Zwart,3,4 Gunnar Birkeland Flugsrud,2 Lars Nordsletten2,4 1The HUNT Research Centre, Department of Public Health and Nursing, Faculty of Medicine and Health Sciences, NTNU, Norwegian University of Science and Technology, Levanger, 2Division of Orthopaedic Surgery, Oslo University Hospital, Oslo, 3Research and Communication Unit for Musculoskeletal Health, Division of Clinical Neuroscience, Oslo University Hospital, Oslo, 4Faculty of Medicine, University of Oslo, Oslo, 5Uni Research Health, Bergen, 6The Norwegian Arthroplasty Register, Department of Orthopedic Surgery, Haukeland University Hospital, Bergen, 7Department of Clinical Medicine, Institute of Medicine and Dentistry, University of Bergen, Bergen, Norway Objective: Biochemical changes associated with obesity may accelerate osteoarthritis beyond the effect of mechanical factors. This study investigated whether metabolic syndrome and its components (visceral obesity, hypertension, dyslipidemia and insulin resistance were risk factors for subsequent total hip replacement (THR or total knee replacement (TKR due to primary osteoarthritis.Design: In this prospective cohort study, data from the second survey of the Nord-Trøndelag Health Study 2 (HUNT2 were linked to the Norwegian Arthroplasty Register for identification of the outcome of THR or TKR. The analyses were stratified by age (<50, 50–69.9 and ≥70 years and adjusted for gender, body mass index, smoking, physical activity and education.Results: Of the 62,661 participants, 12,593 (20.1% were identified as having metabolic syndrome, and we recorded 1,840 (2.9% THRs and 1,111 (1.8% TKRs during a mean follow-up time of 15.4 years. Cox regression analyses did not show any association between full metabolic syndrome and THR or TKR, except in persons <50 years with metabolic syndrome who had a decreased risk

  18. Reversible acute axonal polyneuropathy associated with Wernicke-Korsakoff syndrome: impaired physiological nerve conduction due to thiamine deficiency?

    Science.gov (United States)

    Ishibashi, S; Yokota, T; Shiojiri, T; Matunaga, T; Tanaka, H; Nishina, K; Hirota, H; Inaba, A; Yamada, M; Kanda, T; Mizusawa, H

    2003-05-01

    Acute axonal polyneuropathy and Wernicke-Korsakoff encephalopathy developed simultaneously in three patients. Nerve conduction studies (NCS) detected markedly decreased compound muscle action potentials (CMAPs) and sensory nerve action potentials (SNAPs) with minimal conduction slowing; sympathetic skin responses (SSRs) were also notably decreased. Sural nerve biopsies showed only mild axonal degeneration with scattered myelin ovoid formation. The symptoms of neuropathy lessened within two weeks after an intravenous thiamine infusion. CMAPs, SNAPs, and SSRs also increased considerably. We suggest that this is a new type of peripheral nerve impairment: physiological conduction failure with minimal conduction delay due to thiamine deficiency.

  19. ROLE OF BONE MARROW ASPIRATION IN DIAGNOSIS OF HAEMATOLOGICAL DISORDER

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    Poonam Nanwani

    2017-03-01

    Full Text Available BACKGROUND The bone marrow examination is an essential investigation for the diagnosis of disorders of the blood and bone marrow. This simple and relatively safe procedure is important, particularly in resource poor centres since access to adjuvant diagnostic techniques are often lacking or absent. MATERIALS AND METHODS 189 patients of all age groups were studied for haematological and non-haematological disorders by bone marrow aspiration in the Department of Pathology, MGM Medical College during the period of 2014 to 2016. RESULTS Majority of the patients who had bone marrow aspiration were aged 0-15 years. The male-to-female ratio was 1:1.03. Most (97% of the marrow aspirate examined had definitive pathologic features, while 14 (7% were normal marrow elements. Out of 189 cases of bone marrow aspiration, acute leukaemia was the most common haematological disease diagnosed using this procedure. Acute lymphoblastic leukaemia was more common than acute myeloid leukaemia. Aplastic anaemia was seen in 16% cases. Megaloblastic anaemia occurred more commonly than other anaemias. Megaloblastic anaemia was seen in 13 cases (7% and microcytic anaemia was seen in 5 cases (3%. There were 10 cases (5% of Idiopathic Thrombocypenic Purpura. Myelodysplastic syndrome and multiple myeloma was seen in 7% and 2% cases respectively. Storage disorder was seen in 3 cases (2%, out of this 02 cases were Gaucher’s disease and one case was Niemann-Pick’s disease. CONCLUSION Bone marrow examination is an important step to arrive at the confirmatory diagnosis of many haematological disorders. This procedure remains a veritable tool in the diagnosis and management of a wide range of haematological diseases, especially in a resource poor centre.

  20. Dhakeri-Bange syndrome in goats due to Penicillium and Aspergillus spp, in Banke district of Nepal: a Clinical-Laboratory Investigation

    Directory of Open Access Journals (Sweden)

    Kedar Karki

    Full Text Available An outbreak of syndrome of unknown etiology associated with the feeding of moldy dry forage and green fodder to goats in Dhakeri village of Banke District. Goats suddenly became ill with symptoms of knuckling of the fetlocks of the pelvic limbs, with no apparent ataxia or flexor weakness. Weight bearing was possible while the digits were extended, but with knuckling, weight was supported on the dorsal surface of the foot .The more severely affected goats were paraplegic and recumbent. Anorexia, apathy, diarrhea and ruminal stasis, flaccid posterior paralysis,hindlegs stretched forward both side of abdomin,paralysis of all four legs, head bented to sides, aimlessly head and rear shaking .On clinical examination based on history these goats were provisionally diagnosed as Dhakeri-Bange as being called locally and Endemic Mycotic polyneuropathy syndrome as seen first time in Nepal due to moldy forage/fodder poisoning in natural pasture were treated with Antidegnala liquor(sr. On mycological and microbiological examination of tissue samples from post-mortem of dead goat and forage/fodder samples from pasture and goats feeding stalls on respective medium revealed the growth of fungal pathogens like Aspergillus and Penicillium spp with E.coli.These results provide circumstantial evidence that feeding of moldy forages and green fodder leaves infected by Penicillium and Aspergillus spp may cause outbreaks of a systemic Mycosis in these goats. [Vet. World 2008; 1(12.000: 357-359

  1. Histological features of the pancreas in a patient with congenital hyperinsulinism due to Beckwith-Wiedemann syndrome

    DEFF Research Database (Denmark)

    Christensen, Lene; Christesen, Henrik Boye Thybo; Brusgaard, Klaus

    month of life. However, 5% suffer from severe and prolonged hypoglycemia necessitating intensive medical treatment, and a small number of these patients need a near-total pancreatectomy. Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in newborns, most frequently...... and glucagon, necessitating 95% pancreatectomy at 20 days of age. The patient also had an abdominal wall defect, hemihypertrophy and visceromegaly. Today, at the age of four years, she only needs a small dosage of diazoxide.Results: A preoperative F-18-DOPA PET/CT scan showed diffuse uptake of the radiotracer......, a focus of pancreatoblastoma measuring 3x1 mm was noted. Discussion and conclusion: In this report of premature neonate with severe CHI due to BWS requiring near-total pancreatectomy, we found diffuse adenomatous hyperplasia of endocrine cells. These microscopic features differ from the focal, diffuse...

  2. Ehlers Danlos syndrome, kyphoscoliotic type due to Lysyl Hydroxylase 1 deficiency in two children without congenital or early onset kyphoscoliosis.

    Science.gov (United States)

    van Dijk, Fleur S; Mancini, Grazia M S; Maugeri, Alessandra; Cobben, Jan M

    2017-10-01

    We report two children with Ehlers Danlos, kyphoscoliotic type confirmed by Lysyl Hydroxylase 1 deficiency due to bi-allelic PLOD1 mutations (kEDS-PLOD1) who were initially thought to have either a diagnosis of classical EDS (cEDS) or a neuromuscular disorder due to absence of (congenital) scoliosis. As the two patients reported here illustrate, patients with kEDS-PLOD1 do not always have a kyphoscoliosis present at birth or in the first year of life, neither do they necessarily develop kyphoscoliosis later in infancy. Using the past criteria for kEDS there was considerable overlap with the clinical diagnostic criteria for EDS classical type. In the patients reported here without (kypho) scoliosis this has delayed the diagnosis, which is unfortunate as the diagnosis of kEDS-PLOD1 results in a different recurrence risk and has management consequences. Interestingly, the new criteria for kEDS would not have prevented this diagnostic delay as congenital or early onset kyphoscoliosis (progressive or non-progressive) is deemed obligatory for the diagnosis of kEDS. Being aware of the limitations of clinical diagnostic criteria, we recommend that (i) in patients without a positive family history nor identified COL5A1/2 mutations, lysyl hydroxylase deficiency or biallelic PLOD1 mutations should be excluded before the diagnosis classical EDS can be made and (ii) PLOD1 and COL5A1/2 should be included in the same Next Generation Sequencing (NGS) gene panel. Copyright © 2017. Published by Elsevier Masson SAS.

  3. Successful Percutaneous Coronary Intervention through a Severely Bent Artificial Ascending Aorta Using the DIO Thrombus Aspiration Catheter

    Directory of Open Access Journals (Sweden)

    Akinori Fujikake

    2016-01-01

    Full Text Available A 66-year-old man was admitted to our institute because of chest pain. He had undergone replacement of the ascending aorta due to aortic dissection 9 years previously. We made a diagnosis of acute coronary syndrome, and coronary artery angiography was performed. Although the right coronary artery was successfully cannulated, a severe bend of the artificial aorta made it very difficult to advance the catheter into the left coronary artery. Ultimately, a DIO thrombus aspiration catheter was used to enter the left coronary artery, and a stent was implanted successfully. The DIO catheter is very useful when the selection of a guiding catheter is complicated, such as in the case of severe vessel tortuosity or a bend of the ascending aorta.

  4. Predisposition to the fragile X syndrome in Jews of Tunisian descent is due to the absence of AGG interruptions on a rare Mediterranean haplotype.

    Science.gov (United States)

    Falik-Zaccai, T C; Shachak, E; Yalon, M; Lis, Z; Borochowitz, Z; Macpherson, J N; Nelson, D L; Eichler, E E

    1997-01-01

    We have studied the ethnic distribution of the fragile X syndrome in Israel and have found that 36/136 (26.5%) of apparently unrelated pedigrees were of Tunisian Jewish descent. The Tunisian Jews, however, constitute only 2%-3% of the general Israeli population, identifying the first ethnic group significantly (P 35 repeats) (8/150, or 5.3%) was significantly greater (P Israel (1/136). Haplotype analysis has indicated that these large uninterrupted CGG repeat alleles are present on a previously unreported (DXS548-FRAXAC1-FRAXAC2) haplotype that accounts for all observed cases of disease among Tunisian Jewish X chromosomes. The high prevalence of disease among Tunisian Jews, we suggest, is due to a founder effect of this rare haplotype, which is completely devoid of AGG interruptions in the Jewish population of Tunisia.

  5. Choosing VET: Aspirations, Intentions and Choice

    Science.gov (United States)

    Hargreaves, Jo; Osborne, Kristen

    2017-01-01

    This summary brings together the findings from two research projects: "Choosing VET: Investigating the VET Aspirations of School Students" and "In Their Words: Student Choice in Training Markets--Victorian Examples." The research investigated school students' post-school aspirations for vocational education and training (VET),…

  6. Endometrial aspiration cytology in gynecological disorders

    Directory of Open Access Journals (Sweden)

    Meenal V Jadhav

    2016-01-01

    Full Text Available Context: Endometrial aspiration is not a popular modality for the study of the endometrium despite its simplicity and potential utility. Aim: The present study was aimed at evaluating the utility of endometrial aspiration in various gynecological disorders. Materials and Methods: In this diagnostic accuracy study, 55 prospectively registered women with various gynecological disorders were evaluated clinically and subjected to endometrial aspiration cytology and study of endometrial histology. Endometrial aspiration was performed by infant feeding tube in 10 cases and intra cath cannula in 45 cases. The slides were stained with rapid Papanicolaou (PAP stain and Leishman stain. Results: Endometrial aspiration cytology showed 90% and 94.6% sampling adequacy with infant feeding tube and intra cath cannula, respectively. Intra cath cannula was very convenient to handle and superior to infant feeding tube in aspirating the endometrium. Of the two stains used, rapid PAP stain was less time-consuming and superior to Leishman stain in studying the nuclear details. Leishman stain was helpful in detecting cytoplasmic vacuoles of secretory endometrium. Overall diagnostic accuracy of endometrial cytology was 90.4% while that for morphological hormonal evaluation was 97.6%. It enjoyed a sensitivity of 91.66%, a specificity of 88.23%, positive predictive value of 94.28%, and negative predictive value of 83.33%. Conclusion: Intra cath cannula emerged as an inexpensive, effective, and convenient device for endometrial aspiration. Endometrial aspiration proved to be a fairly effective, simple, and informative diagnostic modality.

  7. Aspiration Curettage and its Outpatient Usage

    African Journals Online (AJOL)

    Aspiration Curettage and its Outpatient Usage. D. A. G. BARFORD, M, NOTELOVITZ. SUMMARY ... its use on a number of outpatients without anaesthesia is discussed. S. Afr. Med. l., 48, 22 (1974). In order to ... plastic aspiration chamber and suction is applied centrally at the base of the chamber, a cylindrical filter within the.

  8. Entrepreneurial aspirations, motivations, and their drivers

    NARCIS (Netherlands)

    S.J.A. Hessels (Jolanda); A.M. van Gelderen (Marco); A.R. Thurik (Roy)

    2008-01-01

    textabstractSeveral drivers of entrepreneurial aspirations and entrepreneurial motivations are investigated using country-level data from the Global Entrepreneurship Monitor (GEM) for the years 2005 and 2006. We estimate a two-equation model explaining aspirations using motivations and socioeconomic

  9. Entrepreneurial aspirations, motivations, and their drivers

    NARCIS (Netherlands)

    Hessels, J.; van Gelderen, M.W.; Thurik, A.R.

    2008-01-01

    Several drivers of entrepreneurial aspirations and entrepreneurial motivations are investigated using country-level data from the Global Entrepreneurship Monitor (GEM) for the years 2005 and 2006. We estimate a two-equation model explaining aspirations using motivations and socioeconomic variables,

  10. Shareholders’ expectations, aspiration levels, and mergers

    NARCIS (Netherlands)

    Diecidue, E.; van de Ven, J.; Weitzel, U.

    This paper offers a new explanation of value-reducing mergers and stock market driven takeovers by introducing recent research on aspiration levels and individual decision making under risk. If market valuation constitutes an aspiration level for managers, we show that managers may be tempted to

  11. An Aspirational Community Theory of the Firm

    DEFF Research Database (Denmark)

    Li, Xin

    contractual labor and aspirational labor, the former being defined as services provided by a laborer in fulfillment of a contract while the latter as services provided by a laborer in pursuit of an aspiration; and argue that firms exist because while the market may be effective and efficient for allocating...

  12. Epidemiology of Anaemia Necesitating Bone Marrow Aspiration ...

    African Journals Online (AJOL)

    Objective: The study aims at investigating, identifying and classifying the various causes of anaemia necessitating bone marrow aspiration cytology in our environment. Methodology: A retrospective review of all bone marrow aspiration cytology reports of patients referred to Haematology and Blood Transfusion department ...

  13. Ultrasound-Guided Fine Needle Aspiration Biopsy of the Thyroid

    Science.gov (United States)

    ... Physician Resources Professions Site Index A-Z Ultrasound-Guided Fine Needle Aspiration Biopsy of the Thyroid An ... Aspiration Biopsy of the Thyroid? What is Ultrasound-Guided Fine Needle Aspiration Biopsy of the Thyroid? During ...

  14. Aspiration-related organizing pneumonia complicating laparoscopic adjustable gastric banding: A lung cancer mimicker

    Directory of Open Access Journals (Sweden)

    Ahmed A Aljohaney

    2016-01-01

    Full Text Available There are several described pulmonary complications due to laparoscopic adjustable gastric banding. We report a rare case of a 32-year-old male who presented with pulmonary symptoms and a solitary lung mass 12 years after laparoscopic adjustable gastric banding. A bronchoscopic lung biopsy showed organizing pneumonia that was induced by aspiration pneumonia. The atypical radiological appearance of the aspiration pneumonia may pose a diagnostic challenge, and clinicians' awareness regarding such an entity is needed to avoid unnecessary intervention.

  15. Fatal case of hemolytic-uremic syndrome in an adult due to a rare serogroup O91 Entero hemorrhagic Escherichia coli associated with a Clostridium difficile infection. More than meets the eye

    Directory of Open Access Journals (Sweden)

    Thomas Guillard

    2015-08-01

    Full Text Available Hemolytic-uremic syndrome due to enterohemorrhagic Escherichia coli, belonging to serogroup O91 has rarely been described. We report here a case of post-diarrheal HUS due to EHEC O91 in an elderly patient for whom diagnosis was delayed given a previously diagnosed C. difficile infection. This case highlights the usefulness of Shiga-toxin detection.

  16. [Study of the predictive value of detection tests for silent aspirations].

    Science.gov (United States)

    Woisard, V; Réhault, E; Brouard, C; Fichaux-Bourin, P; Puech, M; Grand, S

    2009-01-01

    Screening for aspiration in patients with swallowing disorders is important in preventing complications. The tests used in this regard are insufficient due to silent aspiration relating to abnormal protective reflexes in many patients with swallowing problems. The aim of this study is to determine the predictive values of simple tests in screening for silent aspiration. The reference test used was videofluoroscopic examination on swallowing. In the presence of aspiration (FR+) the presence (ME+) or not (ME-) of a cough of throat clearing was noted. The tests being studied were a nasal test with isotonic saline and swallowing according to a set time. For screening for aspiration the presence of a "wet voice" was considered to be a sign of reduced protective reflexes. 1) During the nasal test, the results are 100% for the positive predictive value (VPp) and 83.3% for the negative predictive value (VPn); 2) These results are respectively 84.6% and 35.9% during the swallowing test. Regarding screening for silent aspiration, 1) during the nasal test, the results are 62.5% for the positive predictive value (VPp) and 36.3% for the negative predictive value (VPn); 2) These results are respectively 54.5% and 26.6% during the swallowing test. This preliminary study points out the lack of predictive value of the nasal test and the swallow test for the silent aspirations. However the results could be useful for other researchers developing other tests in this area.

  17. Aspirated Compressors for High Altitude Engines, Phase I

    Data.gov (United States)

    National Aeronautics and Space Administration — Aurora Flight Sciences proposes to incorporate aspirated compressor technology into a high altitude, long endurance (HALE) concept engine. Aspiration has been proven...

  18. Aspiration pneumonia in patients with cleft palate

    International Nuclear Information System (INIS)

    Lee, Seung Hun; Choi, Yo Won; Jeon, Seok Chol; Park, Choong Ki; Uhm, Ki Il

    2003-01-01

    To assess the incidence of aspiration pneumonia in infants with cleft palate and to compare the incidence between complete and incomplete types of cleft palate. A review of medical records revealed 100 infants who had undergone initial surgery to repair cleft palate in our hospital during a recent three-year period. Aspiration pneumonia was defined as the coexistence of pneumonia at chest radiography with a history of frequent choking during feeding. The anatomic distribution of aspiration pneumonia was analyzed, and the incidences of aspiration pneumonia in infants with complete and incomplete cleft palate were compared. Among 100 children, aspiration pneumonia was found in 35 (35%). Those with complete and incomplete cleft palate showed similar incidences of the condition (27 of 70 [39%] vs 8 of 30 [27%], p=0.36). Pneumonia was most commonly seen in the left lower lobe (11 of 35), followed by the right upper and lower lobes. Aspiration pneumonia is frequently associated with infants with cleft palate. There is no statistical difference in the incidence of aspiration pneumonia between the complete and the incomplete cleft palate group

  19. Aspiration pneumonia in patients with cleft palate

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Seung Hun; Choi, Yo Won; Jeon, Seok Chol; Park, Choong Ki; Uhm, Ki Il [Hanyang University College of Medicine, Seoul (Korea, Republic of)

    2003-03-01

    To assess the incidence of aspiration pneumonia in infants with cleft palate and to compare the incidence between complete and incomplete types of cleft palate. A review of medical records revealed 100 infants who had undergone initial surgery to repair cleft palate in our hospital during a recent three-year period. Aspiration pneumonia was defined as the coexistence of pneumonia at chest radiography with a history of frequent choking during feeding. The anatomic distribution of aspiration pneumonia was analyzed, and the incidences of aspiration pneumonia in infants with complete and incomplete cleft palate were compared. Among 100 children, aspiration pneumonia was found in 35 (35%). Those with complete and incomplete cleft palate showed similar incidences of the condition (27 of 70 [39%] vs 8 of 30 [27%], p=0.36). Pneumonia was most commonly seen in the left lower lobe (11 of 35), followed by the right upper and lower lobes. Aspiration pneumonia is frequently associated with infants with cleft palate. There is no statistical difference in the incidence of aspiration pneumonia between the complete and the incomplete cleft palate group.

  20. Personalized Stem Cell Therapy to Correct Corneal Defects Due to a Unique Homozygous-Heterozygous Mosaicism of Ectrodactyly-Ectodermal Dysplasia-Clefting Syndrome.

    Science.gov (United States)

    Barbaro, Vanessa; Nasti, Annamaria Assunta; Raffa, Paolo; Migliorati, Angelo; Nespeca, Patrizia; Ferrari, Stefano; Palumbo, Elisa; Bertolin, Marina; Breda, Claudia; Miceli, Francesco; Russo, Antonella; Caenazzo, Luciana; Ponzin, Diego; Palù, Giorgio; Parolin, Cristina; Di Iorio, Enzo

    2016-08-01

    : Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome is a rare autosomal dominant disease caused by mutations in the p63 gene. To date, approximately 40 different p63 mutations have been identified, all heterozygous. No definitive treatments are available to counteract and resolve the progressive corneal degeneration due to a premature aging of limbal epithelial stem cells. Here, we describe a unique case of a young female patient, aged 18 years, with EEC and corneal dysfunction, who was, surprisingly, homozygous for a novel and de novo R311K missense mutation in the p63 gene. A detailed analysis of the degree of somatic mosaicism in leukocytes from peripheral blood and oral mucosal epithelial stem cells (OMESCs) from biopsies of buccal mucosa showed that approximately 80% were homozygous mutant cells and 20% were heterozygous. Cytogenetic and molecular analyses excluded genomic alterations, thus suggesting a de novo mutation followed by an allelic gene conversion of the wild-type allele by de novo mutant allele as a possible mechanism to explain the homozygous condition. R311K-p63 OMESCs were expanded in vitro and heterozygous holoclones selected following clonal analysis. These R311K-p63 OMESCs were able to generate well-organized and stratified epithelia in vitro, resembling the features of healthy tissues. This study supports the rationale for the development of cultured autologous oral mucosal epithelial stem cell sheets obtained by selected heterozygous R311K-p63 stem cells, as an effective and personalized therapy for reconstructing the ocular surface of this unique case of EEC syndrome, thus bypassing gene therapy approaches. This case demonstrates that in a somatic mosaicism context, a novel homozygous mutation in the p63 gene can arise as a consequence of an allelic gene conversion event, subsequent to a de novo mutation. The heterozygous mutant R311K-p63 stem cells can be isolated by means of clonal analysis and given their good regenerative

  1. Oocyte cryopreservation for fertility preservation in postpubertal female children at risk for premature ovarian failure due to accelerated follicle loss in Turner syndrome or cancer treatments.

    Science.gov (United States)

    Oktay, K; Bedoschi, G

    2014-12-01

    To preliminarily study the feasibility of oocyte cryopreservation in postpubertal girls aged between 13 and 15 years who were at risk for premature ovarian failure due to the accelerated follicle loss associated with Turner syndrome or cancer treatments. Retrospective cohort and review of literature. Academic fertility preservation unit. Three girls diagnosed with Turner syndrome, 1 girl diagnosed with germ-cell tumor. and 1 girl diagnosed with lymphoblastic leukemia. Assessment of ovarian reserve, ovarian stimulation, oocyte retrieval, in vitro maturation, and mature oocyte cryopreservation. Response to ovarian stimulation, number of mature oocytes cryopreserved and complications, if any. Mean anti-müllerian hormone, baseline follical stimulating hormone, estradiol, and antral follicle counts were 1.30 ± 0.39, 6.08 ± 2.63, 41.39 ± 24.68, 8.0 ± 3.2; respectively. In Turner girls the ovarian reserve assessment indicated already diminished ovarian reserve. Ovarian stimulation and oocyte cryopreservation was successfully performed in all female children referred for fertility preservation. A range of 4-11 mature oocytes (mean 8.1 ± 3.4) was cryopreserved without any complications. All girls tolerated the procedure well. Oocyte cryopreservation is a feasible technique in selected female children at risk for premature ovarian failure. Further studies would be beneficial to test the success of oocyte cryopreservation in young girls. Copyright © 2014 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  2. Transvaginal Aspiration of Ovarian Cysts: Long-Term Follow-up

    International Nuclear Information System (INIS)

    Duke, D.; Colville, J.; Keeling, A.; Broe, D.; Fotheringham, T.; Lee, M.J.

    2006-01-01

    Background and purpose. Transvaginal aspiration of ovarian cysts has been advocated as a viable alternative to surgery in patients who are high-risk surgical candidates. We describe a retrospective study evaluating the results of transvaginal aspirations of benign ovarian cysts in patients at increased surgical risk, focusing on long-term follow-up for recurrence of the cyst and/or development of malignancy. Methods. Twenty-four women with ovarian cysts underwent 34 transvaginal drainages between October 1998 and December 2004. All patients were referred following diagnosis of a persistent ovarian cyst with a benign appearance on ultrasound. All patients were unsuitable candidates for surgery (history of previous pelvic surgery, n = 21; high risk for anesthesia, n = 1; and unsuitable for laparoscopy due to obesity, n = 2). Patients with a history of pregnancy, acute abdominal symptoms, or previous gynecologic malignancy were excluded. A 20G x 20 cm Chiba needle was used for transvaginal aspiration using an endocavity probe (Acuson XP, Mountain View, CA, USA; Siemens Sololine, Erlangen, Germany) and intravenous sedoanalgesia. Cysts were aspirated to dryness. Results. Long-term follow-up of patients was performed and revealed a recurrence rate of 75%. Eighty-three percent of cysts on the left and 42% of those on the right recurred. Nine of 15 (60%) patients with recurrence required further intervention. Two of 9 underwent surgical intervention only, 4 of 9 had repeat transvaginal aspiration(s) performed, and 3 of 9 had a combination of both transvaginal aspiration and surgery. No patient developed ovarian malignancy. Conclusion. Transvaginal cyst aspiration has many advantages including short hospital stay, rapid recovery, excellent patient tolerance, and a low rate of procedure-related complications. Our study demonstrates that ovarian cyst recurrence following transvaginal drainage is a more significant problem than previously documented, especially if the cyst is

  3. Aspirant candidate behaviour and progressive political ambition

    Directory of Open Access Journals (Sweden)

    Peter Allen

    2017-02-01

    Full Text Available In this paper we take account of the role of aspirant candidate behaviour in progressive political ambition, specifically how some individuals signal their political ambition to political actors by approaching them to discuss running for office. We examine how the effect of this behaviour compares to the more prominently studied effect of elite recruitment. We conclude that signalling behaviour by an aspirant candidate has a substantial effect, particularly with regard to actually acting on initial considerations of whether to stand, and that elite recruitment makes a difference but only in conjunction with the aspirant candidate themselves signalling their ambition to political actors.

  4. Clinical profile of children with kerosene aspiration.

    Science.gov (United States)

    Venkatesh, C; Sriram, P; Adhisivam, B; Mahadevan, S

    2011-07-01

    Accidental aspiration of kerosene is a common problem among children in developing countries. This descriptive study involved the retrospective analysis of children admitted with kerosene aspiration in a tertiary care hospital in South India for a year. Kerosene aspiration contributed to 60.4% of poisoning cases. Boys aged less than three years and from a rural background formed a significant proportion. Tachypnoea, cough and crepitations were the predominant clinical findings. Male gender and malnutrition (weight for age <80%) were significantly associated with prolonged hospital stay.

  5. Combined assessment (aspiration cytology and mammography) of ...

    African Journals Online (AJOL)

    Combined assessment (aspiration cytology and mammography) of clinically suspicious breast masses. W.F. van Wyk, D Dent, E Anne Hacking, Genevieve Learmonth, R.E. Kottler, C Anne Gudgeon, A Tiltman ...

  6. Ultrasound guided percutaneous fine needle aspiration biopsy ...

    African Journals Online (AJOL)

    2011-06-15

    )-guided percutaneous fine needle aspiration biopsy (PFNAB)/US-guided percutaneous needle core biopsy (PNCB) of abdominal lesions is efficacious in diagnosis, is helpful in treatment choice, to evaluate whether various ...

  7. Ultrasound guided percutaneous fine needle aspiration biopsy ...

    African Journals Online (AJOL)

    )-guided percutaneous fine needle aspiration biopsy (PFNAB)/US-guided percutaneous needle core biopsy (PNCB) of abdominal lesions is efficacious in diagnosis, is helpful in treatment choice, to evaluate whether various other investigations ...

  8. Rapidly progressive renal disease as part of Wolfram syndrome in a large inbred Turkish family due to a novel WFS1 mutation (p.Leu511Pro)

    DEFF Research Database (Denmark)

    Yuca, Sevil Ari; Rendtorff, Nanna Dahl; Boulahbel, Houda

    2012-01-01

    Wolfram syndrome, also named "DIDMOAD" (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness), is an inherited association of juvenile-onset diabetes mellitus and optic atrophy as key diagnostic criteria. Renal tract abnormalities and neurodegenerative disorder may occur in the third...... and fourth decade. The wolframin gene, WFS1, associated with this syndrome, is located on chromosome 4p16.1. Many mutations have been described since the identification of WFS1 as the cause of Wolfram syndrome. We identified a new homozygous WFS1 mutation (c.1532T>C; p.Leu511Pro) causing Wolfram syndrome...

  9. Bacteria Isolated From Respiratory Tract Specimens of Renal Recipients With Acute Respiratory Distress Syndrome Due to Pneumonia: Epidemiology and Susceptibility of the Strains.

    Science.gov (United States)

    Mao, P; Wan, Q Q; Ye, Q F

    2015-12-01

    We estimated species distribution and frequency of antimicrobial resistance among bacterial pathogens isolated from respiratory tract specimens of renal recipients with acute respiratory distress syndrome (ARDS) due to pneumonia. We retrospectively collected patient demographics and clinical characteristics and microbiologic culture data with the use of standard microbiologic procedures and commercially available tests. From January 2001 to August 2014, 320 respiratory tract specimens were obtained from 94 renal recipients with ARDS. Bacterial cultures were positive in 134 specimens from 68 recipients (72.3%), yielding 139 bacterial strains. The most commonly isolated species were gram-negative bacteria (111 isolates) with dominance of Acinetobacter baumanii (29.7%) and Pseudomonas aeruginosa (18.0%). The gram-negative bacteria were relatively resistant to 1st- and 2nd-generation cephalosporin and monocyclic beta-lactam and relatively sensitive to levofloxacin and meropenem, with rates of resistance of 80.2%, 76.6%, 73.9%, 36.0%, and 44.1%, respectively. The gram-positive bacteria, excluding Streptococcus uberis, were sensitive to glycopeptides and oxazolidone. Gram-negative bacteria predominated as 79.9% of isolates from respiratory tract specimens of renal recipients with ARDS. The gram-negative bacteria were relatively sensitive to levofloxacin and meropenem and the gram-positive bacteria were sensitive to glycopeptides and oxazolidone. Copyright © 2015 Elsevier Inc. All rights reserved.

  10. Huge bilateral ovarian cysts in adulthood as the presenting feature of Van Wyk Grumbach syndrome due to chronic uncontrolled juvenile hypothyroidism

    Directory of Open Access Journals (Sweden)

    K S Shivaprasad

    2013-01-01

    Full Text Available Juvenile primary hypothyroidism causing cystic ovaries and pseudoprecocious puberty (Van-Wyk Grumbach syndrome (VWGS is well documented in literature. There are only a few reports of primary hypothyroidism presenting as ovarian cysts in adults. Here we present a case of huge bilateral ovarian cysts in adulthood as the presenting feature of VWGS due to chronic uncontrolled juvenile hypothyroidism. Large uniloculor right ovarian cyst (119 × 81 × 90 mm and a multicystic left ovary (55 × 45 × 49 mm were detected in a 24 year lady with secondary amenorrhea, galactorrhea, and palpable abdominal mass with history of neonatal jaundice, delayed milestones, short stature, and precocious menarche at age of 7.5 years age. She had elevated levels of cancer antigen (CA-125 which normalized post levothyroxine supplementation. Elevated CA-125 may lead to misdiagnosis of ovarian carcinoma and inadvertent treatment. Bilateral ovarian cysts in adults are a rare presentation of juvenile hypothyroidism. It is necessary to screen for primary hypothyroidism in patients presenting with bilateral ovarian cysts to prevent unnecessary evaluation and treatment.

  11. Imaging mass spectrometry visualizes ceramides and the pathogenesis of dorfman-chanarin syndrome due to ceramide metabolic abnormality in the skin.

    Directory of Open Access Journals (Sweden)

    Naoko Goto-Inoue

    Full Text Available Imaging mass spectrometry (IMS is a useful cutting edge technology used to investigate the distribution of biomolecules such as drugs and metabolites, as well as to identify molecular species in tissues and cells without labeling. To protect against excess water loss that is essential for survival in a terrestrial environment, mammalian skin possesses a competent permeability barrier in the stratum corneum (SC, the outermost layer of the epidermis. The key lipids constituting this barrier in the SC are the ceramides (Cers comprising of a heterogeneous molecular species. Alterations in Cer composition have been reported in several skin diseases that display abnormalities in the epidermal permeability barrier function. Not only the amounts of different Cers, but also their localizations are critical for the barrier function. We have employed our new imaging system, capable of high-lateral-resolution IMS with an atmospheric-pressure ionization source, to directly visualize the distribution of Cers. Moreover, we show an ichthyotic disease pathogenesis due to abnormal Cer metabolism in Dorfman-Chanarin syndrome, a neutral lipid storage disorder with ichthyosis in human skin, demonstrating that IMS is a novel diagnostic approach for assessing lipid abnormalities in clinical setting, as well as for investigating physiological roles of lipids in cells/tissues.

  12. Unique Preservation of Neural Cells in Hutchinson- Gilford Progeria Syndrome Is Due to the Expression of the Neural-Specific miR-9 MicroRNA

    Directory of Open Access Journals (Sweden)

    Xavier Nissan

    2012-07-01

    Full Text Available One puzzling observation in patients affected with Hutchinson-Gilford progeria syndrome (HGPS, who overall exhibit systemic and dramatic premature aging, is the absence of any conspicuous cognitive impairment. Recent studies based on induced pluripotent stem cells derived from HGPS patient cells have revealed a lack of expression in neural derivatives of lamin A, a major isoform of LMNA that is initially produced as a precursor called prelamin A. In HGPS, defective maturation of a mutated prelamin A induces the accumulation of toxic progerin in patient cells. Here, we show that a microRNA, miR-9, negatively controls lamin A and progerin expression in neural cells. This may bear major functional correlates, as alleviation of nuclear blebbing is observed in nonneural cells after miR-9 overexpression. Our results support the hypothesis, recently proposed from analyses in mice, that protection of neural cells from progerin accumulation in HGPS is due to the physiologically restricted expression of miR-9 to that cell lineage.

  13. A hypothesis for the cause of complex regional pain syndrome-type I (reflex sympathetic dystrophy): pain due to deep-tissue microvascular pathology.

    Science.gov (United States)

    Coderre, Terence J; Bennett, Gary J

    2010-08-01

    Complex regional pain syndrome-type I (CRPS-I; reflex sympathetic dystrophy) is a chronic pain condition that usually follows a deep-tissue injury such as fracture or sprain. The cause of the pain is unknown. We have developed an animal model (chronic post-ischemia pain) that creates CRPS-I-like symptomatology. The model is produced by occluding the blood flow to one hind paw for 3 hours under general anesthesia. Following reperfusion, the treated hind paw exhibits an initial phase of hyperemia and edema. This is followed by mechano-hyperalgesia, mechano-allodynia, and cold-allodynia that lasted for at least 1 month. Light microscopic analyses and electron microscopic analyses of the nerves at the site of the tourniquet show that the majority of these animals have no sign of injury to myelinated or unmyelinated axons. However, electron microscopy shows that the ischemia-reperfusion injury produces a microvascular injury, slow-flow/no-reflow, in the capillaries of the hind paw muscle and digital nerves. We propose that the slow-flow/no-reflow phenomenon initiates and maintains deep-tissue ischemia and inflammation, leading to the activation of muscle nociceptors, and the ectopic activation of sensory afferent axons due to endoneurial ischemia and inflammation. These data, and a large body of clinical evidence, suggest that in at least a subset of CRPS-I patients, the fundamental cause of the abnormal pain sensations is ischemia and inflammation due to microvascular pathology in deep tissues, leading to a combination of inflammatory and neuropathic pain processes. Moreover, we suggest a unifying idea that relates the pathogenesis of CRPS-I to that of CRPS-II. Lastly, our hypothesis suggests that the role of the sympathetic nervous system in CRPS-I is a factor that is not fundamentally causative, but may have an important contributory role in early-stage disease.

  14. Overlapping of Serotonin Syndrome with Neuroleptic Malignant Syndrome due to Linezolid-Fluoxetine and Olanzapine-Metoclopramide Interactions: A Case Report of Two Serious Adverse Drug Effects Caused by Medication Reconciliation Failure on Hospital Admission

    Directory of Open Access Journals (Sweden)

    Faizan Mazhar

    2016-01-01

    Full Text Available Antipsychotic and antidepressant are often used in combination for the treatment of neuropsychiatric disorders. The concomitant use of antipsychotic and/or antidepressant with drugs that may interact can lead to rare, life-threatening conditions such as serotonin syndrome and neuroleptic malignant syndrome. We describe a patient who has a history of taking two offending drugs that interact with drugs given during the course of hospital treatment which leads to the development of serotonin syndrome overlapped with neuroleptic malignant syndrome. The physician should be aware that both NMS and SS can appear as overlapping syndrome especially when patients use a combination of both antidepressants and antipsychotics.

  15. The trisomy 18 syndrome

    Directory of Open Access Journals (Sweden)

    Cereda Anna

    2012-10-01

    Full Text Available Abstract The trisomy 18 syndrome, also known as Edwards syndrome, is a common chromosomal disorder due to the presence of an extra chromosome 18, either full, mosaic trisomy, or partial trisomy 18q. The condition is the second most common autosomal trisomy syndrome after trisomy 21. The live born prevalence is estimated as 1/6,000-1/8,000, but the overall prevalence is higher (1/2500-1/2600 due to the high frequency of fetal loss and pregnancy termination after prenatal diagnosis. The prevalence of trisomy 18 rises with the increasing maternal age. The recurrence risk for a family with a child with full trisomy 18 is about 1%. Currently most cases of trisomy 18 are prenatally diagnosed, based on screening by maternal age, maternal serum marker screening, or detection of sonographic abnormalities (e.g., increased nuchal translucency thickness, growth retardation, choroid plexus cyst, overlapping of fingers, and congenital heart defects . The recognizable syndrome pattern consists of major and minor anomalies, prenatal and postnatal growth deficiency, an increased risk of neonatal and infant mortality, and marked psychomotor and cognitive disability. Typical minor anomalies include characteristic craniofacial features, clenched fist with overriding fingers, small fingernails, underdeveloped thumbs, and short sternum. The presence of major malformations is common, and the most frequent are heart and kidney anomalies. Feeding problems occur consistently and may require enteral nutrition. Despite the well known infant mortality, approximately 50% of babies with trisomy 18 live longer than 1 week and about 5-10% of children beyond the first year. The major causes of death include central apnea, cardiac failure due to cardiac malformations, respiratory insufficiency due to hypoventilation, aspiration, or upper airway obstruction and, likely, the combination of these and other factors (including decisions regarding aggressive care. Upper airway

  16. Hyperinsulinaemic hypoglycaemia in Beckwith-Wiedemann syndrome (BWS) due to defects in the function of pancreatic ß-cell ATP-sensitive K+ channels

    DEFF Research Database (Denmark)

    Hussain, K; Cosgrove, K E; Shepherd, R M

    2005-01-01

    Beckwith-Wiedemann syndrome (BWS) is a congenital overgrowth syndrome that is clinically and genetically heterogeneous. Hyperinsulinemic hypoglycemia occurs in about 50% of children with BWS and, in the majority of infants, it resolves spontaneously. However, in a small group of patients...... the hypoglycemia can be persistent and may require pancreatectomy. The mechanism of persistent hyperinsulinemic hypoglycemia in this group of patients is unclear....

  17. Autosomal dominant Carvajal plus syndrome due to the novel desmoplakin mutation c.1678A > T (p.Ile560Phe

    Directory of Open Access Journals (Sweden)

    Josef Finsterer

    2016-09-01

    Conclusion: Phenotypic manifestations of Carvajal syndrome are even broader than so far anticipated, the number of mutations in the desmoplakin gene responsible for Carvajal syndrome is still increasing, and these patients require implantation of an ICD as soon as their diagnosis is established.

  18. Methotrexate-induced side effects are not due to differences in pharmacokinetics in children with Down syndrome and acute lymphoblastic leukemia

    NARCIS (Netherlands)

    Buitenkamp, Trudy D.; Mathôt, Ron A. A.; de Haas, Valerie; Pieters, Rob; Zwaan, C. Michel

    2010-01-01

    Children with Down syndrome have an increased risk of developing acute lymphoblastic leukemia and a poor tolerance of methotrexate. This latter problem is assumed to be caused by a higher cellular sensitivity of tissues in children with Down syndrome. However, whether differences in pharmacokinetics

  19. The analysis of the cases of aspired fuel oil and gasoline through siphonage method.

    Science.gov (United States)

    Gonullu, Hayriye; Karadas, Sevdegü; Oncü, Mehmet Resit; Dulger, A Cumhur; Keskin, Siddik

    2013-03-01

    Accidental aspiration of petroleum products in children can also be witnessed in adults working with petroleum products by siphonage or swallowing fire for demonstration purposes. Ten cases admitted to Yuzuncu Yil University Emergency Service due to fuel oil and gasoline aspiration in a three-year period were retrospectively analysed. All cases were males and their average age was determined as 32,4 +/- 7.83 years. Three of the patients aspirated gasoline and 7 fuel oil. Blood gas values in all patients were at normal levels and their average white blood cell values were 16,590. The most frequent symptom for referral to our service was shortness of breath. Infiltration was confirmed in the chest X-ray of 2 patients with aspirated fuel oil and all cases of gasoline aspiration. All patients received methylprednisolone and IV proton-pump inhibitors for treatment. Eight patients were given antibiotics. All victims were discharged from the hospital after recovery. Aspiration of petroleum products which is normally rarely seen is witnessed more frequently in under developed countries. Since the findings determined by screening methods are often nonspecific, history is important for making a diagnosis.

  20. Intermittent At-Home Suctioning of Esophageal Content for Prevention of Recurrent Aspiration Pneumonia in 4 Dogs with Megaesophagus.

    Science.gov (United States)

    Manning, K; Birkenheuer, A J; Briley, J; Montgomery, S A; Harris, J; Vanone, S L; Gookin, J L

    2016-09-01

    Megaesophagus carries a poor to guarded prognosis due to death from aspiration pneumonia. Options for medical management of regurgitation are limited to strategic oral or gastrostomy tube feeding. To describe the use and efficacy of intermittent esophageal suctioning to prevent regurgitation and associated episodes of aspiration pneumonia in dogs with megaesophagus. Four dogs with acquired idiopathic megaesophagus and recurrent aspiration pneumonia. Retrospective review of medical records of dogs with megaesophagus in which intermittent suctioning of esophageal content was employed for management of recurrent aspiration pneumonia. Intermittent suctioning of the esophagus was initiated in 4 dogs after failure of strict gastrostomy tube feeding failed to prevent regurgitation and repeated episodes of aspiration pneumonia. Suctioning was accomplished by esophagostomy tube in 3 dogs and per os in 1 dog. After initiation of esophageal suctioning, dogs survived for a median of 13.5 additional months (range, 10-30 months) during which time 2 dogs had no additional episodes of aspiration pneumonia and 2 dogs had infrequent episodes of pneumonia, but aspiration was suspected to be a contributing factor in their death. Complications included clogging of the esophagostomy tube, esophagostomy site infections, and esophagitis. Use of intermittent esophageal suctioning in dogs with megaesophagus that continue to regurgitate despite gastrostomy tube feedings can reduce or abolish clinical episodes of aspiration pneumonia. Copyright © 2016 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.

  1. Intussusception due to Peutz-Jeghers syndrome - a case report and review of the literature; Sindrome de Peutz-Jeghers e intussuscepcao - relato de um caso e revisao da literatura

    Energy Technology Data Exchange (ETDEWEB)

    Grasso Filho, Luiz Eduardo; Albertotti, Flavio; Carvalho, Claudio Sobral de; Nersessian, Ana Carolina; Docema, Marcos F. Lima; Ogasawara, Aparecida M.; Peng Yong Sheng [Hospital Sirio Libanes, Sao Paulo, SP (Brazil). Centro de Diagnostico por Imagem; Costacurta, Marco Antonio [Hospital Sirio Libanes, Sao Paulo, SP (Brazil). Servico de Radiologia Geral; Albertotti, Cesar Jose [Hospital Sirio Libanes, Sao Paulo, SP (Brazil). Servico de Tomografia Computadorizada; Cerri, Giovanni Guido [Hospital Sirio Libanes, Sao Paulo, SP (Brazil). Servico de Ultra-Som

    2000-02-01

    The authors report a case of a 28-year-old woman with ileocecocolic intussusception due to Peutz-Jeghers syndrome, an autosomal dominant disorder characterized by hamartomatous polyposis of the gastrointestinal tract and mucocutaneous pigmentation. This condition frequently presents complications such as intestinal obstruction due to invagination or hemorrhage. In this patient, the diagnosis of intussusception was made preoperatively. The excised material revealed three large polyps which were considered to be the cause of the intussusception. (author)

  2. Aspiring and Aspiration Shaming: Primary Schooling, English, and Enduring Inequalities in Liberalizing Kerala (India)

    Science.gov (United States)

    Mathew, Leya

    2018-01-01

    This paper analyzes narratives about the radical socio-economic changes accompanying liberalization in India to consider how English-medium schooling is becoming an aspirational resource for non-elite parents. I suggest that aspiring is a practice of ethics that marginalized mothers mobilize to negotiate memories of deprivation and yearnings for…

  3. A three-generation family with terminal microdeletion involving 5p15.33-32 due to a whole-arm 5;15 chromosomal translocation with a steady phenotype of atypical cri du chat syndrome.

    Science.gov (United States)

    Elmakky, Amira; Carli, Diana; Lugli, Licia; Torelli, Paola; Guidi, Battista; Falcinelli, Cristina; Fini, Sergio; Ferrari, Fabrizio; Percesepe, Antonio

    2014-03-01

    Cri du chat syndrome is characterized by cat-like cry, facial dysmorphisms, microcephaly, speech delay, intellectual disability and slow growth rate, which are present with variable frequency. The typical cri du chat syndrome, due to 5p15.2 deletion, includes severe intellectual disability, facial dysmorphisms, neonatal hypotonia and pre- and post-natal growth retardation, whereas more distal deletions in 5p15.3 lead to cat-like cry and speech delay and produce the clinical picture of the atypical cri du chat syndrome, with minimal or absent intellectual impairment. In this article we report a three-generation family with an unbalanced whole arm translocation between chromosome 5 and 15 and a microdeletion of 5.5 Mb involving 5p15.33-32. By reporting the smallest terminal deletion of 5p15.3 described so far and by reviewing the literature we discuss the genotype/phenotype correlations of the distal region of the cri du chat syndrome. The previously described critical region for the speech delay may be narrowed down and microcephaly, growth retardation and dysmorphic facial features can be included in the phenotypic expression of the atypical cri du chat syndrome due to 5p15.3 deletions. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  4. Open lung ventilation improves gas exchange and attenuates secondary lung injury in a piglet model of meconium aspiration

    NARCIS (Netherlands)

    van Kaam, Anton H.; Haitsma, Jack J.; de Jaegere, Anne; van Aalderen, Wim M.; Kok, Joke H.; Lachmann, Burkhard

    2004-01-01

    Objective: Previous studies failed to show clear benefits of high-frequency ventilation compared with conventional positive pressure ventilation (PPVCON) in experimental meconium aspiration syndrome. However, none of these studies applied an open lung ventilation strategy (OLC), which aims to reduce

  5. Risk assessment of medically assisted reproduction and advanced maternal ages in the development of Prader-Willi syndrome due to UPD(15)mat.

    Science.gov (United States)

    Matsubara, K; Murakami, N; Fukami, M; Kagami, M; Nagai, T; Ogata, T

    2016-05-01

    Recent studies have suggested that disomic oocyte-mediated uniparental disomy 15 (UPD(15)mat) is increased in patients with Prader-Willi syndrome (PWS) born after medically assisted reproduction (MAR). However, it remains unknown whether the increase is primarily due to MAR procedure itself or advanced maternal childbearing ages as a predisposing factor for the disomic oocyte production. To examine this matter, we studied 122 naturally conceived PWS patients (PWS-NC group) and 13 MAR-conceived patients (PWS-MAR group). The relative frequency of disomic oocyte-mediated UPD(15)mat was significantly higher in PWS-MAR group than in PWS-NC group (7/13 vs 20/122, p = 0.0045), and the maternal childbearing ages were significantly higher in PWS-MAR group than in PWS-NC group [median (range), 38 (26-45) vs 30 (19-42), p = 0.0015]. However, the logistic regression analysis revealed no significant association between the occurrence of disomic oocyte-mediated UPD(15)mat and MAR, after adjusting for childbearing age (p = 0.25). Consistent with this, while the frequency of assisted reproductive technology (ART)-conceived livebirths was higher in the PWS patients than in the Japanese general population (6.4% vs 1.1%, p = 0.00018), the distribution of childbearing ages was significantly skewed to the increased ages in the PWS patients (p < 2.2 × 10(-16) ). These results argue against a positive association of MAR procedure itself with the development of UPD(15)mat. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  6. Real-world analysis of the Celgene Global Drug Safety database: early discontinuation of lenalidomide in patients with myelodysplastic syndromes due to non-serious rash

    Directory of Open Access Journals (Sweden)

    Weiss L

    2015-09-01

    Full Text Available Lilia Weiss,1 Dianna Gary,1 Arlene S Swern,2 John Freeman,1 Mary M Sugrue3 1Global Drug Safety, Celgene Corporation, Summit, 2Biometrics, Celgene Corporation, Berkeley Heights, 3Medical Affairs, Celgene Corporation, Summit, NJ, USA Background: Lenalidomide is approved for treating transfusion-dependent anemia due to lower-risk del(5q myelodysplastic syndromes (MDS. In clinical trials, rash was common, although severe rash was infrequent. To examine rash in patients with MDS treated with lenalidomide in the real world, the Celgene Global Drug Safety database was analyzed and compared with clinical trials.Materials and methods: Adverse event reports in the post-marketing setting and in the MDS-003/004 clinical trials were analyzed by action taken with lenalidomide, seriousness/grade, time to onset, and treatment duration.Results: Globally, 16,942 reports representing 36,793 adverse events from the post-marketing setting were submitted to the Global Drug Safety database between December 27, 2005 and June 13, 2013. Most rash adverse events were non-serious (Global Drug Safety database, 91% or grade 1/2 (MDS-003/004 trials, 87%–93%. Unexpectedly, rash, occurring at a median of 9 days after treatment initiation, was the leading cause of permanent discontinuation of lenalidomide. Seventy-two percent of non-serious rash adverse events led to early permanent discontinuation within two cycles, while in the MDS-003/004 pivotal clinical trials, only 2%–3% of rash adverse events led to permanent discontinuation.Conclusion: Non-serious rash was the most common reason for permanent discontinuation of lenalidomide in real-world settings. Managing lenalidomide-related rash using published recommendations might improve treatment duration and optimize patient outcomes. Keywords: adverse events, safety, post-marketing setting 

  7. A case of Alagille syndrome complicated by intraocular lens subluxation and rhegmatogenous retinal detachment

    Science.gov (United States)

    Fukumoto, Masanori; Ikeda, Tsunehiko; Sugiyama, Tetsuya; Ueki, Mari; Sato, Takaki; Ishizaki, Eisuke

    2013-01-01

    This case report describes a case of Alagille syndrome with developing intraocular lens subluxation and rhegmatogenous retinal detachment 4 years after cataract surgery. A 15-year-old female patient with Alagille syndrome-associated cataracts in both eyes underwent phacoemulsification aspiration and intraocular lens implantation. Four years postoperative, intraocular lens subluxation developed in her left eye. For treatment, extraction of the dislocated intraocular lens, anterior vitrectomy, and intraocular lens fixation was performed. Three weeks later, the patient developed rhegmatogenous retinal detachment, which was well-treated by pars plana vitrectomy. Cataract surgery needs to be performed carefully in patients with Alagille syndrome due to the weakness of the zonule of Zinn. Careful postoperative observation is necessary for patients with Alagille syndrome who have undergone intraocular surgery in order to facilitate early detection of a possible rhegmatogenous retinal detachment. PMID:23898221

  8. Severe acute respiratory distress syndrome caused by unintentional sewing machine lubricant ingestion: A case report.

    Science.gov (United States)

    Kishore, Sunil; Chandelia, Sudha; Patharia, Neha; Swarnim

    2016-11-01

    Sewing machine oil ingestion is rare but is possible due to its availability at home. Chemically, it belongs to hydrocarbon family which is toxic if aspirated, owing to their physical properties such as high volatility and low viscosity. On the contrary, sewing machine lubricant has high viscosity and low volatility which makes it aspiration less likely. The main danger of hydrocarbon ingestion is chemical pneumonitis which may be as severe as acute respiratory distress syndrome (ARDS). We report a case of a 5-year-old girl with accidental ingestion of sewing machine lubricant oil, who subsequently developed ARDS refractory to mechanical ventilation. There was much improvement with airway pressure release ventilation mode of ventilation, but the child succumbed to death due to pulmonary hemorrhage.

  9. Aspiration Deaths Among Adults in Istanbul

    Directory of Open Access Journals (Sweden)

    Haşim Asil

    2014-06-01

    Full Text Available Massive aspiration of food is rare, but most common in people under the influence of alcohol or a drug and comatose patients who have impaired functioning of the central nervous system. The finding of small amounts of food material in the airway at autopsy does not indicate the true vital aspiration because of agonal or even early post-mortem overspills. Occlusion of the small airways, mainly the membranous and respiratory bronchioles, partial or total filling of bronchiolar lumen and the alveolar spaces with food or gastric content were typical morphologic findings. In our 10 year retrospective study we presented the data of 21 male and 4 female cases with a final diagnosis of fatal aspiration in age from 23 to 78 years (45.43±14.61 from the records of Morgue Specialization Department of the Council of Forensic Medicine. 13 cases found death at the scene without an eyewitness. All deaths were accidental in manner except one homicide. Toxicological analysis revealed blood alcohol concentration levels between 161 and 339 mg/dL in 7 cases. Morphine metabolites, benzodiazepine and barbituric acid derivatives, toluene and acetone were detected in 5 subjects. Aspirated materials were food in 14 cases, chewing gum in 3 cases, gastric content in 7 cases and a fabric gag in one case. The history, other evidence of external vomit on the clothing or immediate surroundings and toxicological analysis are by no means as significant as autopsy findings especially in cases of aspiration. Key words : adult; aspiration; death; autopsy

  10. The sweet lung: Chewing gummi bear aspiration.

    Science.gov (United States)

    Tavladaki, Theonimfi; Fitrolaki, Michaela-Diana; Spanaki, Anna-Maria; Ilia, Staurula; Geromarkaki, Elissabet; Briassoulis, George

    2012-07-01

    Inhalation of foreign bodies, a leading cause of accidental death, is most common in preschool children. In this article we report our experience with a 5-year-old Greek girl who presented with a 24-hour history of sore throat, chest pain, and shortness of breath. Emergency bronchoscopy was performed and multiple small chewing gummi bear (HARIBO) particles impacted in the orifices of the right main bronchus and right lobar and segmentalinic bronchi were successfully removed and aspirated. Aspiration of gummi bears, which is for the first time reported, may cause a silent choking episode leading to life-threatening bronchi obstruction at multiple sites, even in children older than 4 years.

  11. Endogenous Cushing’s Syndrome with Precocious Puberty in an 8-Year-Old Boy due to a Large Unilateral Adrenal Adenoma

    Directory of Open Access Journals (Sweden)

    Muhammad Rajib Hossain

    2013-01-01

    Full Text Available Adrenocortical tumors (ACTs causing Cushing’s syndrome are extremely rare in children and adolescents. Bilateral macronodular adrenocortical disease which is a component of the McCune-Albright syndrome is the most common cause of endogenous Cushing’s syndrome. We report the case of a boy with Cushing’s syndrome who presented with obesity and growth retardation. The child was hypertensive. The biochemical evaluation revealed that his serum cortisol levels were 25.80 g/dL, with a concomitant plasma ACTH level of 10.0 pg/mL and nonsuppressed serum cortisol on high-dose dexamethasone suppression test (HDDST to be 20.38 g/dL. Computed tomography of the abdomen demonstrated a 8 × 6 × 5 cm left adrenal mass with internal calcifications. Following preoperative stabilization, laparotomy was carried out which revealed a lobulated left adrenal mass with intact capsule weighing 120 grams. Histopathological examination revealed a benign cortical neoplastic lesion, suggestive of adrenal adenoma; composed of large polygonal cells with centrally placed nuclei and prominent nucleoli without capsular and vascular invasion. On the seventh postoperative day, cortisol levels were within normal range indicating biochemical remission of Cushing’s syndrome. On followup after three months, the patient showed significant clinical improvement and had lost moderate amount of weight and adrenal imaging was found to be normal.

  12. Microaspiration Syndrome in Pediatric Practice: Modern Features and Role in Bronchial Obstruction Syndrome Formation

    Directory of Open Access Journals (Sweden)

    S.I. Ilchenko

    2016-10-01

    Full Text Available The paper presents the data on research of aspiration syndrome prevalence among young children treated in the City children’s pulmonary department due to protracted course of obstructive bronchitis. The structure of microaspiration reasons in young children was studied (2010–2015. The most significant reasons of microaspiration syndrome development were revealed depending on children age. Modified questionnaire for the parents was used to collect anamnesis effectively. The children with perinatal impairment of nervous system, preterm children, and the children with morphological and physio­logical nasopharyngeal defects, with muscular dystonia are firstly in risk group for microaspiration syndrome. Microaspiration may manifest with frequent regurgitation, vomiting during cough, meal leaking from the nose, correlation of coughing fit with feeding, exacerbation or development of coughing in prone position, fit of night coughing and asphyxia, long-term hacking after cough attack. Pediatricians are recommended to observe attentively their patients during each feeding as dysphagy could be inconstant. Microaspiration syndrome is a challenging for diagnosis and requires complex approach, as mostly diagnostic procedures are invasive. Timely diagnosis and treatment of microaaspiration syndrome allow decrease respiratory diseases rate in young children and reduce obstructive bronchitis duration.

  13. Aspiration in injections: should we continue or abandon the practice? [version 3; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Yasir Sepah

    2017-03-01

    Full Text Available Aspiration during any kind of injection is meant to ensure that the needle tip is at the desired location during this blind procedure. While aspiration appears to be a simple procedure, it has generated a lot of controversy concerning the perceived benefits and indications. Advocates and opponents of aspiration both make logically sound claims. However, due to scarcity of available data, there is no evidence that this procedure is truly beneficial or unwarranted. Keeping in view the huge number of injections given worldwide, it is important that we draw attention to key questions regarding aspiration that, up till now, remain unanswered. In this review, we have attempted to gather and present literature on aspiration both from published and non-published sources in order to provide not only an exhaustive review of the subject, but also a starting point for further studies on more specific areas requiring clarification. A literature review was conducted using the US National Institute of Health’s PubMed service (including Medline, Google Scholar and Scopus. Guidelines provided by the World Health Organization, Safe Injection Global Network, International Council of Nursing, Center for Disease Control, US Federal Drug Agency, UK National Health Services, British Medical Association, Europe Nursing and Midwifery Council, Public Health Agency Canada, Pakistan Medical Association and International Organization of Standardization recommendations 7886 parts 1-4 for sterile hypodermics were reviewed for relevant information. In addition, curricula of several medical/nursing schools from India, Nigeria and Pakistan, the US pharmacopeia Data from the WHO Program for International Drug Monitoring network in regard to adverse events as a result of not aspirating prior to injection delivery were reviewed. Curricula of selected major medical/nursing schools in India, Nigeria and Pakistan, national therapeutic formularies, product inserts of most commonly

  14. Aspirations and occupational achievements of Dutch fathers and mothers

    NARCIS (Netherlands)

    van der Horst, Mariska; van der Lippe, Tanja; Kluwer, Esther

    2014-01-01

    Purpose - To investigate how work and family aspirations relate to occupational achievements and gender differences herein. Design/methodology/approach - Using data from 2009 we examined the relationship between career and childrearing aspirations and occupational achievements of Dutch parents.

  15. Combined assessment (aspiration cytology and mammography) of ...

    African Journals Online (AJOL)

    We examined the safety and utility of the combined assessment of aspiration cytology and mammography in. 705 women who had clinically suspicious or malignant palpable breast masses. Histological assessment confirmed 176 benign and 529 malignant lesions. There were no incorrect (false positive) diagnoses made ...

  16. Identity talk of aspirational ethical leaders

    NARCIS (Netherlands)

    Koning, J.B.M.; Waistell, J.

    2012-01-01

    This study investigates how business leaders dynamically narrate their aspirational ethical leadership identities. In doing so, it furthers understanding of ethical leadership as a process situated in time and place. The analysis focuses on the discursive strategies used to narrate identity and

  17. Gender Differences, Career Aspirations and Career Development ...

    African Journals Online (AJOL)

    The purpose of this study therefore is to explore gender differences in adolescents' career aspirations and career development barriers among secondary school students in Kisumu municipality, Kenya. The study was conducted on 348 form four secondary school students. The major findings of this study show that there ...

  18. Educational Aspirations, What Matters? A Literature Review.

    Science.gov (United States)

    Deosaran, Ramesh

    The literature review attempted to identify: 1) what we know and don't know about post-secondary aspirations, expectations, and access; and 2) where and how it is possible for us to embark on solutions or further investigation. The research in this area has been heavily sociological. Socio-economic background, family size, community origins,…

  19. EDUCATIONAL ASPIRATIONS OF FARM BOYS AND GIRLS.

    Science.gov (United States)

    SLOCUM, WALTER L.

    THE EDUCATIONAL AND OCCUPATIONAL ASPIRATIONS OF RURAL HIGH SCHOOL STUDENTS WERE STUDIED IN RELATION TO SOCIAL, CULTURAL, AND ECONOMIC FACTORS. QUESTIONNAIRES WERE ADMINISTERED TO AND COMPLETED BY 3,535 RURAL HIGH SCHOOL STUDENTS. SUPPLEMENTAL INFORMATION WAS GATHERED BY PERSONAL INTERVIEWS WITH 992 STUDENTS. COUNSELORS, ADMINISTRATORS, AND…

  20. Income Aspirations and Cooperation : Experimental Evidence

    NARCIS (Netherlands)

    Dalton, P.S.

    2010-01-01

    This article is the first attempt to study the empirical link between income aspirations and cooperation in a one shot public good game. By combining experimental with survey data, we find evidence that the more frustrated people are with their income, the lower is their propensity to cooperate with

  1. Extrapulmonary tuberculosis: Fine needle aspiration cytology ...

    African Journals Online (AJOL)

    Background: The increasing prevalence of extrapulmonary manifestation of tuberculosis with the HIV scourge is a cause for concern. Objective: To determine the role of fine needle aspiration cytology (FNAC) in the diagnosis of extrapulmonary tuberculosis. Patients and Methods: This is a consecutive 9-year analysis of ...

  2. FOREIGN BODY ASPIRATION: A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Nafia Ozlem Kazanci

    2013-03-01

    Full Text Available Foreign body aspiration (FBA is common under 3 years of age. FBA can cause sudden respiratory failure. Furthermore, it leads to many respiratory system complications. Therefore, FBA is an important cause of mortality and morbidity for this age group. FBA cases are usually followed by diagnoses like pneumonia, bronchitis or bronchial asthma in the late period that history, clinical and laboratory findings suggesting foreign body aspiration can not be detected. We reported a 2-year-old male patient with pneumonia, who were administered various treatments. In thorax CT of this case, an image compatible with foreign body was seen in the right middle lobe-upper lobe separation area. Granulation tissue was detected in the entrance of the right main bronchus by rigid bronchoscopy and marked improvement occured in lung aeration after bronchoscopy. This case was reported to emphasize the importance of early diagnosis of foreign body aspiration because of high mortality and morbidity and the necessity of considering the foreign body aspiration in children with recurrent pulmonary infections. [J Contemp Med 2013; 3(1.000: 58-61

  3. MCT8 mutation analysis and identification of the first female with Allan-Herndon-Dudley syndrome due to loss of MCT8 expression.

    NARCIS (Netherlands)

    Frints, S.G.; Lenzner, S.; Bauters, M.; Jensen, L.R.; Esch, H. van; Portes, V. des; Moog, U.; Macville, M.V.; Roozendaal, K. van; Schrander-Stumpel, C.T.; Tzschach, A.; Marynen, P.; Fryns, J.P.; Hamel, B.C.J.; Bokhoven, H. van; Chelly, J.; Beldjord, C.; Turner, G.; Gecz, J.; Moraine, C.; Raynaud, M.; Ropers, H.H.; Froyen, G.; Kuss, A.W.

    2008-01-01

    Mutations in the thyroid monocarboxylate transporter 8 gene (MCT8/SLC16A2) have been reported to result in X-linked mental retardation (XLMR) in patients with clinical features of the Allan-Herndon-Dudley syndrome (AHDS). We performed MCT8 mutation analysis including 13 XLMR families with LOD scores

  4. Rapidly progressive renal disease as part of Wolfram syndrome in a large inbred Turkish family due to a novel WFS1 mutation (p.Leu511Pro).

    Science.gov (United States)

    Yuca, Sevil Ari; Rendtorff, Nanna Dahl; Boulahbel, Houda; Lodahl, Marianne; Tranebjærg, Lisbeth; Cesur, Yasar; Dogan, Murat; Yilmaz, Cahide; Akgun, Cihangir; Acikgoz, Mehmet

    2012-01-01

    Wolfram syndrome, also named "DIDMOAD" (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness), is an inherited association of juvenile-onset diabetes mellitus and optic atrophy as key diagnostic criteria. Renal tract abnormalities and neurodegenerative disorder may occur in the third and fourth decade. The wolframin gene, WFS1, associated with this syndrome, is located on chromosome 4p16.1. Many mutations have been described since the identification of WFS1 as the cause of Wolfram syndrome. We identified a new homozygous WFS1 mutation (c.1532T>C; p.Leu511Pro) causing Wolfram syndrome in a large inbred Turkish family. The patients showed early onset of IDDM, diabetes insipidus, optic atrophy, sensorineural hearing impairment and very rapid progression to renal failure before age 12 in three females. Ectopic expression of the wolframin mutant in HEK cells results in greatly reduced levels of protein expression compared to wild-type wolframin, strongly supporting that this mutation is disease-causing. The mutation showed perfect segregation with disease in the family, characterized by early and severe clinical manifestations. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

  5. An Unusual Case of Foreign Body Aspiration in an Infant | Dias ...

    African Journals Online (AJOL)

    Inhalation of foreign body is a serious condition in childhood and may result in acute respiratory distress, lung injury or death. A nine month old baby with history of severe respiratory distress, cyanosis and gasping breathing diagnosed as foreign body aspiration due to a balloon which was recovered post mortem during the ...

  6. Meconium aspiration syndrome: identifying obstetric and neonatal risk situations

    Directory of Open Access Journals (Sweden)

    Sheila Duarte Mendonça

    2015-07-01

    Full Text Available Objetivo: identificar as situações de risco obstétricas e neonatais que favoreceram a síndrome da aspiração meconial, bem como as complicações na evolução clínica apresentadas por esses neonatos. Métodos: Estudo quantitativo, retrospectivo e de análise documental, realizado entre janeiro de 2009 a dezembro de 2010. Os dados dos 40 prontuários consultados foram registrados em banco de dados e analisados por meio de software para análise estatística descritiva. Resultados: Quanto às genitoras, 67,5% tiveram menos que seis consultas de pré-natal e 42,5% intercorrências na gestação. Predominou o parto cesáreo em 75%, indicados por sofrimento fetal. Dos neonatos, 90% apresentaram idade gestacional de 37 semanas ou mais; 82,5% tiveram Apgar abaixo de 7, necessitando de reanimação ao nascer e suporte ventilatório. Conclusão: O acompanhamento adequado da gestante em todo ciclo grávido e no trabalho de parto bem como o atendimento preciso do neonato na sala de parto podem reduzir a incidência da síndrome.

  7. Severe meconium aspiration syndrome: case report | Koigi-Kamau ...

    African Journals Online (AJOL)

    The baby was admitted to the intensive care unit (ICU) for assisted ventilation and critical care. The condition of the baby continued to deteriorate and demise occurred 18 hours after birth. The pathophysiologic processes of intrauterine meconium release, mechanisms of foetal effects and dilemmas in management are ...

  8. Esomeprazole as a prophylactic agent for acid aspiration syndrome ...

    African Journals Online (AJOL)

    -orifice gastric tube passed through an endotracheal tube placed blindly in the oesophagus after tracheal intubation and analysed for pH, volume and the presence of bile salts. Results: Thirty nine samples (33%) out of 117 were contaminated ...

  9. Gender and Leadership Aspiration : The Impact of the Organizational Environment

    NARCIS (Netherlands)

    C. Fritz (Claudia)

    2016-01-01

    markdownabstractSummary The aim of this dissertation is to examine gender differences in leadership aspiration. Although some important work regarding gender-specific aspiration has been done already, conditions fostering leadership aspiration – particularly among women – are not completely

  10. Risk of aspiration in care home residents and associated factors

    NARCIS (Netherlands)

    Maarel-Wierink, C.D. van der; Putten, G.J. van der; Visschere, L.M. De; Bronkhorst, E.M.; Baat, C. de; Schols, J.M.

    2015-01-01

    Pneumonia is a prevalent cause of death in care home residents. Dysphagia is a significant risk factor of aspiration pneumonia. The purpose of the current study was to screen for risk of aspiration in care home residents in the Netherlands and assess potential risk factors of aspiration. Five

  11. Autoaspiration versus manual aspiration in transbronchial needle aspiration in diagnosis of intrathoracic lymphadenopathy.

    Science.gov (United States)

    Boonsarngsuk, Viboon; Pongtippan, Atcharaporn; Juthakarn, Sabaitip; Boonsarngsuk, Wison; Kurimoto, Noriaki

    2009-10-01

    Traditionally, aspiration with high negative pressure is recommended to obtain a specimen in transbronchial needle aspiration (TBNA). Undeniably, however, the assistant experiences difficulty in the generation of the negative pressure and precise control of the syringe while performing the procedure. To evaluate the effectiveness of the autoaspiration method created by our plunger lock in comparison with the conventional manual aspiration in the diagnosis of intrathoracic lymphadenopathy by TBNA. A prospective study was conducted on all patients referred for diagnostic TBNA of enlarged intrathoracic lymph nodes. Both automatic and manual aspiration techniques were performed after the needle had been completely inserted into the nodes. The diagnostic yield and the numbers of diagnostic cells or benign lymphoid cells obtained by each technique were compared in the same node. A total of 31 intrathoracic lymph nodes in 24 patients were prospectively studied. Twenty-four nodes (77.4%) were malignancies whereas 7 nodes (22.6%) were benign disease. Adequate lymph node samples were obtained in 30 targets (96.8%), and TBNA revealed definite diagnosis for 25 nodes (80.6%). Both aspiration techniques showed exactly the same diagnostic yield. However, the autoaspiration technique provided significantly more adequate samples than manual aspiration techniques did (P=0.003). The autoaspiration method using our plunger lock was superior to the manual method in obtaining the numbers of adequate samples in TBNA procedures.

  12. Sequential voluntary cough and aspiration or aspiration risk in Parkinson's disease.

    Science.gov (United States)

    Hegland, Karen Wheeler; Okun, Michael S; Troche, Michelle S

    2014-08-01

    Disordered swallowing, or dysphagia, is almost always present to some degree in people with Parkinson's disease (PD), either causing aspiration or greatly increasing the risk for aspiration during swallowing. This likely contributes to aspiration pneumonia, a leading cause of death in this patient population. Effective airway protection is dependent upon multiple behaviors, including cough and swallowing. Single voluntary cough function is disordered in people with PD and dysphagia. However, the appropriate response to aspirate material is more than one cough, or sequential cough. The goal of this study was to examine voluntary sequential coughing in people with PD, with and without dysphagia. Forty adults diagnosed with idiopathic PD produced two trials of sequential voluntary cough. The cough airflows were obtained using pneumotachograph and facemask and subsequently digitized and recorded. All participants received a modified barium swallow study as part of their clinical care, and the worst penetration-aspiration score observed was used to determine whether the patient had dysphagia. There were significant differences in the compression phase duration, peak expiratory flow rates, and amount of air expired of the sequential cough produced by participants with and without dysphagia. The presence of dysphagia in people with PD is associated with disordered cough function. Sequential cough, which is important in removing aspirate material from large- and smaller-diameter airways, is also impaired in people with PD and dysphagia compared with those without dysphagia. There may be common neuroanatomical substrates for cough and swallowing impairment in PD leading to the co-occurrence of these dysfunctions.

  13. Improved microscopical detection of acid-fast bacilli by the modified bleach method in lymphnode aspirates

    Directory of Open Access Journals (Sweden)

    Annam Vamseedhar

    2009-07-01

    Full Text Available Objectives: To improve the smear microscopy for detection of acid-fast bacilli (AFB in fine needle aspiration cytology (FNAC of lymph node using the bleach method and also to compare this with cytological diagnosis and the conventional Ziehl-Neelsen (ZN method. Study Design: In 99 consecutive patients with clinical suspicion of tuberculosis (TB presenting with lymphadenopathy, FNACs were performed. Smears from the aspirates were processed for routine cytology and the conventional ZN method. The remaining material in the needle hub and/or the syringe was used for the bleach method. The significance of the bleach method over the conventional ZN method and cytology was analyzed using the χ2 test. Results: Of 99 aspirates, 93 were studied and the remaining six were excluded from the study due to diagnosis of malignancy in 4.04% (4/6 and inadequate aspiration in 2.02% (2/6. Among the 93 aspirates, 33.33% (31/93 were positive for AFB on conventional ZN method, 41.94% (39/93 were indicative of TB on cytology and the smear positivity increased to 63.44% (59/93 on bleach method. Conclusion: The bleach method is simple, inexpensive and potent disinfectant, also limiting the risk of laboratory-acquired infections. The implementation of the bleach method clearly improves microscopic detection and can be a useful contribution to routine cytology.

  14. Secondary Voice Restoration After Laryngotracheal Separation (LTS) for Dysphagia with Intractable Aspiration.

    Science.gov (United States)

    Bonte, Katrien; Huvenne, Wouter; De Loof, Marie; Deron, Philippe; Viaene, Annick; Duprez, Fréderic; Vermeersch, Hubert

    2015-12-01

    Intractable aspiration is a serious, often life-threatening condition due to its potential impact on pulmonary function. Aspiration requires therapeutic measures, starting with conservative management but often necessitating surgical treatment. The basic surgical principle is to separate the alimentary and respiratory tracts through a variety of procedures which, unfortunately, nearly all result in the loss of phonation, with the exception of total laryngectomy (TL) which includes the placement of an indwelling voice prosthesis. In this study, we present a modified laryngotracheal separation (LTS) technique that, we believe, offers multiple advantages compared to standard TL. After reviewing the medical records of 35 patients with intractable aspiration who have undergone LTS, we describe the surgical technique and present the postoperative result. In a second surgical procedure about two months following LTS, we aimed to achieve voice restoration by placement of an indwelling voice prosthesis. Intractable aspiration was successfully treated in all patients. Placement of an indwelling voice prosthesis during a second operation was successful in 15 patients, representing the largest reported cohort thus far. LTS is a reliable surgical technique to treat intractable aspiration, with restoration of oral intake, thereby improving the general condition and quality of life of these unfortunate patients. Furthermore, voice restoration can be achieved in selected patients, by placement of a voice prosthesis.

  15. Efficacy And Non Invasive Treatment Of Sialorrhea In The Goldenhar Syndrome.

    Science.gov (United States)

    Marvulli, R; Gallo, G A; Mastromauro, L; Fiore, P; Ianieri, G; Megna, M

    2018-03-28

    Goldenhar syndrome (ocular-auricular-vertebral syndrome), a rare congenital condition arising from defects in the first and second brachial arches, consists in clinical variety of features ranging from facial abnormalities, ear-eye abnormalities, vertebral defects and congenital heart problems and severe obstructive sleep apnea. Due to craniofacial abnormalities, patients presents mechanical obstructive phenomena and sialorrhea that cause prone position, language's fastening, use of nasopharyngeal cannulas and tracheal intubation. In this article, we report a case of a 16 years old child affected by Goldenhar syndrome and sialorrhea to demonstrate improvement of the daily patient management, through inoculations of botulinum toxin type A. Due to severe sialorrhea which caused tracheobronchial daily aspirations, caregivers used an external aspirators. In the first infiltration (August 2016) the parotid and submandibular glands bilaterally were inoculated with incobotulinum toxin type A (Xeomin®, Merz Pharma) with dosages of 5 UI for each of them, for a total of 20 UI without clinical efficacy (no quantitative and qualitative saliva reducing during 3 months). In the second (November 2016) and third (February 2017) infiltrations each parotid and each submandibular glands were injected with a (dosage of 7 UI and 5 UI respectively (total of 24 UI of incobotulinumtoxin A) with important clinical results (saliva production and tracheo-bronchial aspirations reduced). So, botulinum toxin type A could be a good and non invasive treatment of sialorrhea in Goldenhar syndrome to improve oral hygiene and daily patient management. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  16. Acute respiratory failure after aspiration of activated charcoal with recurrent deposition and release from an intrapulmonary cavern.

    Science.gov (United States)

    Francis, Roland C E; Schefold, Joerg C; Bercker, Sven; Temmesfeld-Wollbrück, Bettina; Weichert, Wilko; Spies, Claudia D; Weber-Carstens, Steffen

    2009-02-01

    To report on the recurrent release of charcoal from an intrapulmonary cavern in a case of acute respiratory failure after charcoal aspiration. Case report. Anaesthesiological ICU, university hospital. An 18-year-old ethanol intoxicated comatose patient regurgitated and aspirated activated charcoal during orotracheal intubation. After 2 days of mechanical ventilation, the patient was transferred to a tertiary care university hospital. On admission, acute respiratory distress syndrome with bilateral pulmonary infiltrations was diagnosed. The patient's recovery was hampered by recurrent release of charcoal from an intrapulmonary cavern. Sophisticated ventilatory support, prone positioning, secretolytics, repetitive bronchoscopy, and antibiotic therapy may have facilitated bronchoalveolar clearance and weaning after 18 days. Aspiration may be a dramatic complication if charcoal is administered in comatose patients without airway protection. In this case report, advanced intensive care measures were necessary to tackle the special feature of charcoal release from an intrapulmonary cavern.

  17. Partial trisomy due to a de novo duplication 22q11.1-22q13.1: a cat-eye syndrome variant with brain anomalies.

    Science.gov (United States)

    Karcaaltincaba, D; Ceylaner, S; Ceylaner, G; Dalkilic, S; Karli-Oguz, K; Kandemir, O

    2010-01-01

    We report a case of partial trisomy 22q with de novo duplication of chromosomal region 22q11.1-22q13.1, also confirmed by microarray comparative genomic hybridization (Array-CGH) analysis. The fetus had interhemispheric cyst and corpus callosum agenesis diagnosed by MRI which has not been reported in the literature. This novel phenotype differs from the reported cat eye syndromes by the absence of heart defects and the presence of brain anomalies.

  18. Altered Pathogenesis of Porcine Respiratory Coronavirus in Pigs due to Immunosuppressive Effects of Dexamethasone: Implications for Corticosteroid Use in Treatment of Severe Acute Respiratory Syndrome Coronavirus▿

    OpenAIRE

    Jung, Kwonil; Alekseev, Konstantin P.; Zhang, Xinsheng; Cheon, Doo-Sung; Vlasova, Anastasia N.; Saif, Linda J.

    2007-01-01

    The pathogenesis and optimal treatments for severe acute respiratory syndrome (SARS) are unclear, although corticosteroids were used to reduce lung and systemic inflammation. Because the pulmonary pathology of porcine respiratory coronavirus (PRCV) in pigs resembles SARS, we used PRCV as a model to clarify the effects of the corticosteroid dexamethasone (DEX) on coronavirus (CoV)-induced pneumonia. Conventional weaned pigs (n = 130) in one of four groups (PRCV/phosphate-buffered saline [PBS] ...

  19. Upper gastrointestinal bleed in a post menopausal woman due to combination of high first dose aspirin and clopidogrel prescribed for acute coronary syndrome

    Directory of Open Access Journals (Sweden)

    Vishal R Tandon

    2015-01-01

    Full Text Available Combination of aspirin, clopidogrel and enoxaparin remains the standard treatment for acute coronary syndrome (ACS but is known to increase the incidence of upper gastrointestinal bleed (UGIB. We hereby report an unusual case of gastrointestinal bleed (GIB as it resulted inspite of proton pump inhibitor (PPI prophylaxis within the second day of treatment in a post-menopausal woman (PMW with high first dose of aspirin clopidogrel dual combination in a patient of ACS.

  20. Acadian variant of Fanconi syndrome is caused by mitochondrial respiratory chain complex I deficiency due to a non-coding mutation in complex I assembly factor NDUFAF6

    Czech Academy of Sciences Publication Activity Database

    Hartmannová, H.; Piherová, L.; Tauchmannová, Kateřina; Kidd, K.; Acott, P. D.; Crocker, J. F. S.; Oussedik, Y.; Mallet, M.; Hodaňová, K.; Stránecký, V.; Přistoupilová, A.; Barešová, V.; Jedličková, I.; Živná, M.; Sovová, J.; Hůlková, H.; Robins, V.; Vrbacký, Marek; Pecina, Petr; Kaplanová, Vilma; Houštěk, Josef; Mráček, Tomáš; Thibeault, Y.; Bleyer, A. J.; Kmoch, S.

    2016-01-01

    Roč. 25, č. 18 (2016), s. 4062-4079 ISSN 0964-6906 R&D Projects: GA ČR(CZ) GB14-36804G; GA MŠk(CZ) LL1204 Institutional support: RVO:67985823 Keywords : Acadian variant of Fanconi syndrome * mitochondrial complex I deficiency * NDUFAF6 * C8ORF38 * non-coding mutation * alternative splicing variant * protein isoforms Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 5.340, year: 2016