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1

Olivero, Francesca, Thomas Foiadelli, Sabino Luzzi, Gian Luigi Marseglia, and Salvatore Savasta. "Pai syndrome: a review." Child's Nervous System 36, no. 11 (2020): 2635–40. http://dx.doi.org/10.1007/s00381-020-04788-z.

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Abstract Background Pai syndrome is a rare idiopathic developmental condition characterized by midline craniofacial abnormalities. It was originally described as the presence of a median cleft lip, cutaneous polyps of the nasal mucosa and face, and midline lipomas of the central nervous system, mostly at the corpus callosum. However, there is great phenotypical variability and these characteristics are rarely all present at once. Objective The aim of this review was to analyze the available evidence regarding Pai syndrome in order to better delineate this rare condition and its features. Metho
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2

Mishima, Katsuaki, Yoshihide Mori, Katsuhiro Minami, Masayoshi Sakuda, and Toshio Sugahara. "A Case of Pai Syndrome." Plastic and Reconstructive Surgery 103, no. 1 (1999): 166–70. http://dx.doi.org/10.1097/00006534-199901000-00026.

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3

Pérez, Cinthia Giselle, Sandra Carrera Fernández, and Agustin Rodríguez D’Aquila. "Nasal and lip polyps: Pai syndrome." Acta Otorrinolaringologica (English Edition) 71, no. 3 (2020): 195–96. http://dx.doi.org/10.1016/j.otoeng.2019.02.004.

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4

Pérez, Cinthia Giselle, Sandra Carrera Fernández, and Agustin Rodríguez D’Aquila. "Nasal and lip polyps: Pai syndrome." Acta Otorrinolaringológica Española 71, no. 3 (2020): 195–96. http://dx.doi.org/10.1016/j.otorri.2019.02.006.

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5

Ochoa, J., R. Vildoza, A. Echegaray, and A. Colubriale. "P09.04: Prenatal diagnosis of Pai syndrome." Ultrasound in Obstetrics and Gynecology 36, S1 (2010): 201. http://dx.doi.org/10.1002/uog.8422.

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6

Alessi, Marie-Christine, and Irène Juhan-Vague. "PAI-1 and the Metabolic Syndrome." Arteriosclerosis, Thrombosis, and Vascular Biology 26, no. 10 (2006): 2200–2207. http://dx.doi.org/10.1161/01.atv.0000242905.41404.68.

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7

Blouet, Marie, Frédérique Belloy, Corinne Jeanne-Pasquier, Nathalie Leporrier, and Guillaume Benoist. "Pai syndrome: challenging prenatal diagnosis and management." Pediatric Radiology 44, no. 9 (2014): 1184–87. http://dx.doi.org/10.1007/s00247-014-2966-4.

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8

Tormey, Peter, Iva Bilic Cace, and Michael A. Boyle. "Ocular dermoid in Pai Syndrome: A review." European Journal of Medical Genetics 60, no. 4 (2017): 217–19. http://dx.doi.org/10.1016/j.ejmg.2017.01.008.

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9

Juhan-Vague, I., M. C. Alessi, D. Raccah, et al. "Daytime Fluctuations of Plasminogen Activator Inhibitor 1 (PAI-1) in Populations with High PAI-1 Levels." Thrombosis and Haemostasis 67, no. 01 (1992): 076–82. http://dx.doi.org/10.1055/s-0038-1648384.

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SummaryThe mechanism underlying diurnal variations in PAI-1 as well as the cellular origin of PAI-1 in subjects with high PAI-1 levels are unknown. We evaluated diurnal changes (8:00 am vs 4:00 pm) in PAI-1 (functional and immunological assays), t-PA Ag and t-PA/PAI-1 complex levels in controls and subjects with high PAI-1 levels. Three test groups were recruited among obese hyperinsulinmic subjects, emergency care unit patients with inflammatory syndrome or infection and pregnant women.The classical afternoon decrease of PAI-1 level was observed in controls and obese subjects but its amplitud
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10

Alongi, Alessandra, Roberta Naddei, Laura De Miglio, Valentina Natoli, and Angelo Ravelli. "Macrophage activation syndrome in pediatrics." Pediatric Allergy and Immunology 31, S24 (2020): 13–15. http://dx.doi.org/10.1111/pai.13158.

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11

Novembre, Elio, Francesca Mori, Simona Barni, and Neri Pucci. "Drug-Induced Enterocolitis Syndrome (DIES)." Pediatric Allergy and Immunology 25, no. 4 (2014): 415–16. http://dx.doi.org/10.1111/pai.12225.

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12

Mori, Francesca, Giulia Liccioli, Oliver Fuchs, et al. "Drug‐induced enterocolitis syndrome: Similarities and differences compared with food protein‐induced enterocolitis syndrome." Pediatric Allergy and Immunology 32, no. 6 (2021): 1165–72. http://dx.doi.org/10.1111/pai.13491.

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13

Mamanee, Pornthep, Nathapon Chantaraseno, and Somsak Geraplangsub. "Os Trigonum Syndrome or Posterior Ankle Impingement (PAI)." Bangkok Medical Journal 07, no. 01 (2014): 94–95. http://dx.doi.org/10.31524/bkkmedj.2014.02.019.

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14

Guion-Almeida, Maria Leine, and Antonio Richieri-Costa. "Severe midline craniofacial anomalies: overlap with Pai syndrome." Clinical Dysmorphology 18, no. 3 (2009): 154–57. http://dx.doi.org/10.1097/mcd.0b013e32832443c8.

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15

Guion-Almeida, Maria Leine, Cecília Mellado, Constanza Beltrán, and A. Richieri-Costa. "Pai syndrome: Report of seven South American patients." American Journal of Medical Genetics Part A 143A, no. 24 (2007): 3273–79. http://dx.doi.org/10.1002/ajmg.a.31962.

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16

Freeman, Mark, Michael Mansfield, Jenny Barrett, and Peter Grant. "Insulin resistance: an atherothrombotic syndrome." Thrombosis and Haemostasis 89, no. 01 (2003): 161–68. http://dx.doi.org/10.1055/s-0037-1613555.

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SummaryThe insulin resistance syndrome (IRS) is a clustering of atherothrombotic traits associated with increased vascular risk. We investigated the degree to which the phenotypic correlations between these traits are due to shared genetic and environmental factors.A multivariate genetic analysis was performed in 537 adults from 89 healthy white north European families. All traits showed significant heritability. BMI had significant genetic correlations with fasting insulin, systolic blood pressure (sBP), plasminogen activator activator inhibitor-1 (PAI-1) and fibrinogen and triglyceride. Fast
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17

COSACHOV, JOHN, and MARIANNE FRIERI. "Hyper-IgM Syndrome with Congenital Rubella." Pediatric Asthma, Allergy & Immunology 9, no. 2 (1995): 79–85. http://dx.doi.org/10.1089/pai.1995.9.79.

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18

LANE, JOHN R., JAMES F. CHMIEL, and KENNETH G. ZAHKA. "Marfan Syndrome and Asthma: A Review." Pediatric Asthma, Allergy & Immunology 13, no. 4 (1999): 181–87. http://dx.doi.org/10.1089/pai.1999.13.181.

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19

Infante, Sonsoles, and Lydia Zapatero. "Drug-induced enterocolitis syndrome by amoxicillin." Pediatric Allergy and Immunology 28, no. 1 (2016): 105–6. http://dx.doi.org/10.1111/pai.12643.

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20

Sahin Eroglu, D., M. Torgutalp, S. Baysal, et al. "POS1335 CLINICAL CHARACTERISTICS OF THE PULMONARY ARTERY INVOLVEMENT IN PATIENTS WITH BEHÇET’S SYNDROME: A SINGLE-CENTRE EXPERIENCE OF 61 PATIENTS." Annals of the Rheumatic Diseases 80, Suppl 1 (2021): 949.1–950. http://dx.doi.org/10.1136/annrheumdis-2021-eular.411.

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Background:Behçet’s Syndrome (BS) is a vasculitis of unknown origin. Vascular involvement, so-called vascular Behçet’s syndrome (VBS), may involve blood vessels of all sizes belonging to both venous and arterial system, with pulmonary artery involvement (PAI) being the most frequent form of arterial involvement. PAI in BS occurs in the form of aneurysms or thrombosis, and results in significant mortality.Objectives:To report the clinical characteristics of PAI in patients with BS and to define the predictors of relapses.Methods:We performed a retrospective analysis of BS patients with PAI who
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21

HIZAL, Evren, Ozan EROL, Ceren BAŞ, and Fuat BÜYÜKLÜ. "Pai Syndrome: A Case Report and a Literature Review." Kulak Burun Boğaz ve Baş Boyun Cerrahisi Dergisi 27, no. 3 (2019): 154–58. http://dx.doi.org/10.24179/kbbbbc.2019-64929.

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22

ALTAMIRANO, HUGO G., STEPHEN J. McGEADY, and HERBERT C. MANSMANN. "Right Middle Lobe Syndrome in Asthmatic Children." Pediatric Asthma, Allergy & Immunology 5, no. 1 (1991): 33–37. http://dx.doi.org/10.1089/pai.1991.5.33.

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23

Lougaris, Vassilios, Manuela Baronio, Massimiliano Vitali, et al. "Profound T-cell defects in Dubowitz syndrome." Pediatric Allergy and Immunology 25, no. 5 (2014): 511–13. http://dx.doi.org/10.1111/pai.12238.

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24

YAMAMOTO, Koji. "A pathological role of PAI-1 in metabolic syndrome." Japanese Journal of Thrombosis and Hemostasis 17, no. 2 (2006): 120–27. http://dx.doi.org/10.2491/jjsth.17.120.

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25

Pernod, G., B. Villemur, J. L. Bosson, H. Truche, A. Franco, and B. Polack. "Leg ulcers and Klinefelter's syndrome: role of PAI-1." British Journal of Dermatology 134, no. 3 (1996): 605–6. http://dx.doi.org/10.1111/j.1365-2133.1996.tb16262.x.

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26

Chousta, A., D. Ville, I. James, et al. "Pericallosal lipoma associated with Pai syndrome: prenatal imaging findings." Ultrasound in Obstetrics and Gynecology 32, no. 5 (2008): 708–10. http://dx.doi.org/10.1002/uog.6150.

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27

Pernod, G., B. Villemur, J. L. Bosson, H. Truche, A. Franco, and B. Polack. "Leg ulcers and Klinefelter's syndrome: role of PAI-1." British Journal of Dermatology 134, no. 3 (1996): 605–6. http://dx.doi.org/10.1046/j.1365-2133.1996.t01-8-53778.x.

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28

HENLEY, W. L., M. HORLICK, and S. A. S. KONG. "Behcet's Syndrome with Antibody to a Corneal Antigen." Pediatric Asthma, Allergy & Immunology 4, no. 4 (1990): 291–95. http://dx.doi.org/10.1089/pai.1990.4.291.

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29

MOTOYOSHI, FUMIAKI, NAOMI KONDO, SHOKO IWATA, TOMOMI KATO, TSUYOSHI TAKAMI, and TADAO ORII. "A Case of Bloom's Syndrome with Malignant Lymphoma." Pediatric Asthma, Allergy & Immunology 8, no. 4 (1994): 227–31. http://dx.doi.org/10.1089/pai.1994.8.227.

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30

EVANS, JOHN ROBERT, and STEPHEN J. McGEADY. "Sjögren Syndrome in an Adolescent with IgA Deficiency." Pediatric Asthma, Allergy & Immunology 9, no. 1 (1995): 49–51. http://dx.doi.org/10.1089/pai.1995.9.49.

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31

Terlemez, Semiha, Faruk Demir, Yasin Bulut, et al. "DRESS syndrome developed related to acetylsalicylic acid use." Pediatric Allergy and Immunology 27, no. 2 (2015): 227–30. http://dx.doi.org/10.1111/pai.12484.

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32

Ronchi, Andrea, Laura Marra, Ferdinando Frigeri, Gerardo Botti, Renato Franco, and Annarosaria De Chiara. "Richter Syndrome With Plasmablastic Lymphoma at Primary Diagnosis." Applied Immunohistochemistry & Molecular Morphology 25, no. 6 (2017): e40-e45. http://dx.doi.org/10.1097/pai.0000000000000441.

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33

Imai, Yoshimichi, Shigeo Kure, Chieko Nara, Naoyuki Takagi, and Masahiro Tachi. "Mental Development and Surgical Prognosis of Pai Syndrome: A Case Report and Review of the Literature." Cleft Palate-Craniofacial Journal 56, no. 2 (2018): 273–79. http://dx.doi.org/10.1177/1055665618771419.

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Pai syndrome is a rare congenital disorder, and there are few reports about the long-term prognosis of mental development and surgical results. Here, we report a patient with Pai syndrome who was followed up from birth up to the age of 8 years. Additionally, we review 32 articles and discuss the long-term prognosis of Pai syndrome. In our case, an intracranial lipoma grew a little, but neither epilepsy nor intellectual disabilities occurred. However, she showed attention-deficit/hyperactivity disorder. Furthermore, her nasal airway was gradually obstructed by a residual intranasal polyp.
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34

MANSMANN, PARIS T. "Features of Churg—Strauss Syndrome Shared with Polyarteritis, Wegener's Granulomatosis, and Hypereosinophilic Syndrome: Is There a Common Role for the Eosinophil?" Pediatric Asthma, Allergy & Immunology 4, no. 1 (1990): 9–15. http://dx.doi.org/10.1089/pai.1990.4.9.

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35

de Larrañaga, Gabriela, Silvia Perés Wingeyer, Mabel Graffigna, et al. "Plasma Plasminogen Activator Inhibitor-1 Levels and Nonalcoholic Fatty Liver in Individuals With Features of Metabolic Syndrome." Clinical and Applied Thrombosis/Hemostasis 14, no. 3 (2008): 319–24. http://dx.doi.org/10.1177/1076029607304094.

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Fatty liver represents the liver component of metabolic syndrome and may be involved in plasminogen activator inhibitor-1 (PAI-1) synthesis. We studied plasma PAI-1 levels and relationships with risk factors for metabolic syndrome, including fatty liver, in 170 patients. Liver ultrasound scan was performed on all patients, and a liver biopsy was performed on those patients with chronically elevated transaminase levels. Plasma PAI-1 levels correlated significantly ( P < .05) with body mass index, degree of steatosis, insulin resistance, insulin level, waist circumference, triglycerides, and
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36

Chen, Jin-Shuen, Chung-Ze Wu, Nain-Feng Chu, Li-Chien Chang, Dee Pei, and Yuh-Feng Lin. "Association among Fibrinolytic Proteins, Metabolic Syndrome Components, Insulin Secretion, and Resistance in Schoolchildren." International Journal of Endocrinology 2015 (2015): 1–7. http://dx.doi.org/10.1155/2015/170987.

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We investigated the role of urokinase plasminogen activator (uPA) and its soluble receptors (suPAR) and plasminogen activator inhibitor-1 (PAI-1) in metabolic syndrome (MetS) components, insulin secretion, and resistance in schoolchildren. We enrolled 387 children, aged 10.3 ± 1.5 years, in Taipei. Anthropometry, fibrinolytic proteins, MetS components, insulin secretion, and resistance were measured. Subjects were divided into normal, overweight, and obese groups. Finally, the relationship between fibrinolytic proteins and metabolic syndrome in boys and girls was analyzed. In boys, PAI-1 was p
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37

RODDY, SARAH M., and ROBERT H. SCHWARTZ. "Tourette Syndrome: A Neurologic Disease That Can Mimic Allergy." Pediatric Asthma, Allergy & Immunology 2, no. 3 (1988): 161–68. http://dx.doi.org/10.1089/pai.1988.2.161.

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38

Rekhtman, David, and Eli M. Eisenstein. "Glucocorticoids for Treatment of Severe Pediatric Drug Hypersensitivity Syndrome." Pediatric Asthma, Allergy & Immunology 18, no. 3 (2005): 156–60. http://dx.doi.org/10.1089/pai.2005.18.156.

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39

Kapoor, Shailendra. "Dubowitz syndrome and the increased risk of developing malignancies." Pediatric Allergy and Immunology 26, no. 8 (2015): 820–21. http://dx.doi.org/10.1111/pai.12411.

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40

Pecora, Valentina, Giusi Prencipe, Rocco Valluzzi, et al. "Inflammatory events during food protein-induced enterocolitis syndrome reactions." Pediatric Allergy and Immunology 28, no. 5 (2017): 464–70. http://dx.doi.org/10.1111/pai.12723.

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41

Atanasković-Marković, Marina, Biljana Medjo, Marija Gavrović-Jankulović, Tanja Ćirković Veličković, Dimitrije Nikolić, and Branimir Nestorović. "Stevens-Johnson syndrome and toxic epidermal necrolysis in children." Pediatric Allergy and Immunology 24, no. 7 (2013): 645–49. http://dx.doi.org/10.1111/pai.12121.

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42

Guibas, George V., Sophia Tsabouri, Michael Makris, and Kostas N. Priftis. "Food protein-induced enterocolitis syndrome: Pitfalls in the diagnosis." Pediatric Allergy and Immunology 25, no. 7 (2014): 622–29. http://dx.doi.org/10.1111/pai.12237.

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43

Vecchiola, Andrea, Killen Garcia, Luis-Martin González-Gómez, et al. "Relationship Between Metabolic Syndrome Components and Proinflammatory Molecules." Journal of the Endocrine Society 5, Supplement_1 (2021): A25—A26. http://dx.doi.org/10.1210/jendso/bvab048.049.

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Abstract We aimed to study the associations of 5 adipocytokines, two endothelial damage markers, and hs-CRP with the MetS components to distinguish the most significant cytokines likely related to distinct metabolic profiles. Methods: Cross-sectional study with 202 Chilean subjects (18–65 years old), categorized by MetS, and No-MetS according to Harmonizing ATP III. Adipocytokines profiling included adiponectin, leptin, hs-CRP, CTRP-1, PAI-1, FABP4, and metalloproteinase (MMP)-9 and MMP-2 activity. Results: Subjects with MetS showed higher levels of the most proinflammatories molecules but sig
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44

GOLDENHERSH, M. J., and K. D. DUNN. "The Coexistence of Acquired Immune Deficiency Syndrome and Chronic Disease." Pediatric Asthma, Allergy & Immunology 1, no. 4 (1987): 247–50. http://dx.doi.org/10.1089/pai.1987.1.247.

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45

PACINI, DAVID L., ROGER A. FRIEDMAN, MICHAEL T. BRADY, BARRY T. SHANNON, STEPHEN J. QUALMAN, and DONALD C. ANDERSON. "Pneumocystis cariniiPneumonia in an Infant with the Hyperimmunoglobulinemia E Syndrome*." Pediatric Asthma, Allergy & Immunology 3, no. 1 (1989): 21–26. http://dx.doi.org/10.1089/pai.1989.3.21.

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46

KATSUNUMA, TOSHIO, AKIRA AKASAWA, HIDEYUKI KANEMOTO, et al. "Swyer-James Syndrome After Severe Pulmonary Infection Caused byMycoplasma pneumoniae." Pediatric Asthma, Allergy & Immunology 4, no. 2 (1990): 131–39. http://dx.doi.org/10.1089/pai.1990.4.131.

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47

KATO, Z., T. KATO, N. KONDO, and T. ORII. "Renal Failure and Long-Term Survival in Wiskott-Aldrich Syndrome." Pediatric Asthma, Allergy & Immunology 8, no. 1 (1994): 47–49. http://dx.doi.org/10.1089/pai.1994.8.47.

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48

Di Cesare, Silvia, Pamela Puliafito, Paola Ariganello, et al. "Autoimmunity and regulatory T cells in 22q11.2 deletion syndrome patients." Pediatric Allergy and Immunology 26, no. 6 (2015): 591–94. http://dx.doi.org/10.1111/pai.12420.

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49

Cuppari, C., S. Manti, A. Salpietro, et al. "HMGB1 levels in children with atopic eczema/dermatitis syndrome (AEDS)." Pediatric Allergy and Immunology 27, no. 1 (2015): 99–102. http://dx.doi.org/10.1111/pai.12481.

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50

Karefylaki, Styliani, and Dan Gustafsson. "Recovery from food protein-induced enterocolitis syndrome caused by fish." Pediatric Allergy and Immunology 27, no. 1 (2015): 105–6. http://dx.doi.org/10.1111/pai.12488.

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