Relevant bibliographies by topics / Soulier

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  1. Journal articles
  2. Dissertations / Theses
  3. Books
  4. Book chapters
  5. Conference papers

Academic literature on the topic 'Soulier'

Author: Grafiati
Published: 13 April 2024

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Journal articles on the topic "Soulier"

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Shitikova, A. S., Z. D. Fedorova, O. E. Belyazo, G. P. Shlyapochnikova, L. P. Papayan, L. A. Denisova, V. A. Egorova, and T. I. Popova. "Bernard-Soulier disease." Kazan medical journal 68, no. 2 (April 15, 1987): 124–30. http://dx.doi.org/10.17816/kazmj96033.

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Bernard-Soulier disease, a congenital macrocytic thrombocytopathy, was first described in 1948. Currently, this pathology has again attracted the attention of many researchers, as the study of its pathogenesis has deepened the understanding of the mechanisms of the hemostatic process concerning the interaction of platelets with the damaged vessel wall. This rare disease (70 cases were described by 1983) is inherited as an incomplete autosomal recessive trait and is observed with equal frequency in males and females. Bleeding from mucous membranes of the nose, mouth and other organs, petechiae and ecchymoses on the skin usually appear in early childhood. Hemorrhagic syndrome is usually of moderate severity, but often (in 17% of patients) is the direct cause of death due to bleeding from the gastrointestinal tract or hemorrhage in the brain and its membranes.
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Berndt, M. C., and R. K. Andrews. "Bernard-Soulier syndrome." Haematologica 96, no. 3 (February 28, 2011): 355–59. http://dx.doi.org/10.3324/haematol.2010.039883.

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López, José A., Robert K. Andrews, Vahid Afshar-Kharghan, and Michael C. Berndt. "Bernard-Soulier Syndrome." Blood 91, no. 12 (June 15, 1998): 4397–418. http://dx.doi.org/10.1182/blood.v91.12.4397.

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López, José A., Robert K. Andrews, Vahid Afshar-Kharghan, and Michael C. Berndt. "Bernard-Soulier Syndrome." Blood 91, no. 12 (June 15, 1998): 4397–418. http://dx.doi.org/10.1182/blood.v91.12.4397.412k42_4397_4418.

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MENACHE, D. "Jean-Pierre Soulier." Revue Francaise de Transfusion et Immuno-hématologie 28, no. 6 (December 1985): 565–70. http://dx.doi.org/10.1016/s0338-4535(85)80001-5.

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SOULIER-PERKINS, ADELINE, and GERNOT KUNZ. "Revision of the malagassy endemic genus Amberana Distant (Hemiptera, Cercopidae) with description of one new genus." Zootaxa 3156, no. 1 (January 10, 2012): 1. http://dx.doi.org/10.11646/zootaxa.3156.1.1.

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The genus Amberana Distant is revised. Three new species, A. attei Soulier-Perkins sp. n., A. ouvrardi Soulier-Perkinssp. n. and A. pascali Soulier-Perkins sp. n. are described. Lectotypes are designated for A. dimidiata (Signoret, 1960), A.fissurata Jacobi, 1917, A. noualhieri (Lallemand, 1920), A. sexguttata (Melichar, 1915) and A. uncinata Jacobi, 1917. Am-berana tripunctata var. completa Lallemand, 1949 and A. tripunctata Lallemand, 1920 are synonymised with A. bergevini(Lallemand, 1920). A new genus, Bourgoinrana Soulier-Perkins gen. n., is erected for B. perinetana (Synave, 1957),comb. n. (type species), B. rubescens (Synave, 1957), comb. n. and B. sandrangatensis (Synave, 1957), comb. n. Keysto species of Amberana and Bourgoinrana Soulier-Perkins gen. n. are provided. Drawings of the male genitalia for all species with exception of A. lemuria (Distant, 1908) are included.
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Louguet, Claire. "Philippe Soulier, Simplicius et." Philosophie antique, no. 15 (November 24, 2015): 291–94. http://dx.doi.org/10.4000/philosant.482.

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Desplanque, Gilles. "Ambleny (Aisne). Le Soulier." Archéologie médiévale, no. 42 (December 1, 2012): 176. http://dx.doi.org/10.4000/archeomed.10739.

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Galmiche, Thierry. "Ambleny (Aisne). Le Soulier." Archéologie médiévale, no. 41 (December 1, 2011): 183. http://dx.doi.org/10.4000/archeomed.11515.

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Rouger, P. "Professor Jean Pierre Soulier." Vox Sanguinis 84, no. 3 (April 2003): 163. http://dx.doi.org/10.1046/j.1423-0410.2003.00309.x.

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Dissertations / Theses on the topic "Soulier"

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Sandrock, Kirstin, Ralf Knöfler, Andreas Greinacher, Birgitt Fürll, Sebastian Gerisch, Ulrich Schuler, Siegmund Gehrisch, Anja Busse, and Barbara Zieger. "Novel Mutation in Bernard-Soulier Syndrome." Saechsische Landesbibliothek- Staats- und Universitaetsbibliothek Dresden, 2014. http://nbn-resolving.de/urn:nbn:de:bsz:14-qucosa-136606.

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Background: Bernard-Soulier syndrome (BSS) is a severe congenital bleeding disorder characterized by thrombocytopenia, thrombocytopathy and decreased platelet adhesion. BSS results from genetic alterations of the glycoprotein (GP) Ib/IX/V complex. Methods: We report on a patient demonstrating typical BSS phenotype (thrombocytopenia with giant platelets, bleeding symptoms). However, BSS was not diagnosed until he reached the age of 39 years. Results: Flow cytometry of the patient’s platelets revealed absence of GPIb/IX/V receptor surface expression. In addition, immunofluorescence analysis of patient’s platelets demonstrated very faint staining of GPIX. A novel homozygous deletion comprising 11 nucleotides starting at position 1644 of the GPIX gene was identified using molecular genetic analysis. Conclusions: The novel 11-nucleotide deletion (g.1644_1654del11) was identified as causing the bleeding disorder in the BSS patient. This homozygous deletion includes the last 4 nucleotides of the Kozak sequence as well as the start codon and the following 4 nucleotides of the coding sequence. The Kozak sequence is a region indispensable for the initiation of the protein translation process, thus preventing synthesis of functional GPIX protein in the case of deletion
Hintergrund: Das Bernard-Soulier-Syndrom (BSS) ist eine angeborene Blutungsstörung, die mit Thrombozytopenie, Thrombozytopathie und verminderter Thrombozytenadhäsion assoziiert ist. BSS wird durch genetische Veränderungen des Glykoprotein(GP)-Ib/IX/V-Komplexes verursacht. Methoden: Wir berichten über einen Patienten mit typischem BSS-Phänotyp (Thrombozytopenie mit Riesenthrombozyten, Blutungssymptome). Dennoch wurde die Diagnose BSS erst im Alter von 39 Jahren gestellt. Ergebnisse: Die Durchflusszytometrie der Thrombozyten des Patienten ergab eine fehlende Oberflächenexpression des GPIb/IX/V-Rezeptors. Zusätzlich zeigten Immunfluoreszenz-Analysen der Thrombozyten eine nur sehr schwache Anfärbung von GPIX. In der molekulargenetischen Analyse wurde eine noch nicht bekannte homozygote Deletion von 11 Nukleotiden (beginnend an Position 1644 im GPIX-Gen) identifiziert. Schlussfolgerungen: Diese neue Deletion von 11 Nukleotiden (g.1644_1654del11) wurde als Ursache für die vermehrte Blutungsneigung bei dem BSS-Patienten identifiziert. Von der homozygoten Deletion betroffen sind die letzten 4 Nukleotide der Kozak-Sequenz sowie das Startkodon und weitere 4 Nukleotide des kodierenden Bereichs. Die Kozak-Sequenz ist unerlässlich für die Initiation der Translation in der Proteinbiosynthese, so dass die bei dem Patienten nachgewiesene Deletion die Synthese des funktionellen GPIX-Proteins verhindert
Dieser Beitrag ist mit Zustimmung des Rechteinhabers aufgrund einer (DFG-geförderten) Allianz- bzw. Nationallizenz frei zugänglich
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2

Sandrock, Kirstin, Ralf Knöfler, Andreas Greinacher, Birgitt Fürll, Sebastian Gerisch, Ulrich Schuler, Siegmund Gehrisch, Anja Busse, and Barbara Zieger. "Novel Mutation in Bernard-Soulier Syndrome." Karger, 2010. https://tud.qucosa.de/id/qucosa%3A27717.

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Background: Bernard-Soulier syndrome (BSS) is a severe congenital bleeding disorder characterized by thrombocytopenia, thrombocytopathy and decreased platelet adhesion. BSS results from genetic alterations of the glycoprotein (GP) Ib/IX/V complex. Methods: We report on a patient demonstrating typical BSS phenotype (thrombocytopenia with giant platelets, bleeding symptoms). However, BSS was not diagnosed until he reached the age of 39 years. Results: Flow cytometry of the patient’s platelets revealed absence of GPIb/IX/V receptor surface expression. In addition, immunofluorescence analysis of patient’s platelets demonstrated very faint staining of GPIX. A novel homozygous deletion comprising 11 nucleotides starting at position 1644 of the GPIX gene was identified using molecular genetic analysis. Conclusions: The novel 11-nucleotide deletion (g.1644_1654del11) was identified as causing the bleeding disorder in the BSS patient. This homozygous deletion includes the last 4 nucleotides of the Kozak sequence as well as the start codon and the following 4 nucleotides of the coding sequence. The Kozak sequence is a region indispensable for the initiation of the protein translation process, thus preventing synthesis of functional GPIX protein in the case of deletion.
Hintergrund: Das Bernard-Soulier-Syndrom (BSS) ist eine angeborene Blutungsstörung, die mit Thrombozytopenie, Thrombozytopathie und verminderter Thrombozytenadhäsion assoziiert ist. BSS wird durch genetische Veränderungen des Glykoprotein(GP)-Ib/IX/V-Komplexes verursacht. Methoden: Wir berichten über einen Patienten mit typischem BSS-Phänotyp (Thrombozytopenie mit Riesenthrombozyten, Blutungssymptome). Dennoch wurde die Diagnose BSS erst im Alter von 39 Jahren gestellt. Ergebnisse: Die Durchflusszytometrie der Thrombozyten des Patienten ergab eine fehlende Oberflächenexpression des GPIb/IX/V-Rezeptors. Zusätzlich zeigten Immunfluoreszenz-Analysen der Thrombozyten eine nur sehr schwache Anfärbung von GPIX. In der molekulargenetischen Analyse wurde eine noch nicht bekannte homozygote Deletion von 11 Nukleotiden (beginnend an Position 1644 im GPIX-Gen) identifiziert. Schlussfolgerungen: Diese neue Deletion von 11 Nukleotiden (g.1644_1654del11) wurde als Ursache für die vermehrte Blutungsneigung bei dem BSS-Patienten identifiziert. Von der homozygoten Deletion betroffen sind die letzten 4 Nukleotide der Kozak-Sequenz sowie das Startkodon und weitere 4 Nukleotide des kodierenden Bereichs. Die Kozak-Sequenz ist unerlässlich für die Initiation der Translation in der Proteinbiosynthese, so dass die bei dem Patienten nachgewiesene Deletion die Synthese des funktionellen GPIX-Proteins verhindert.
Dieser Beitrag ist mit Zustimmung des Rechteinhabers aufgrund einer (DFG-geförderten) Allianz- bzw. Nationallizenz frei zugänglich.
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3

Barbier, Christèle. "Le Soulier de satin et l’art moderne." Thesis, Paris 4, 2014. http://www.theses.fr/2014PA040191.

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Le Soulier de satin, tour à tour perçu comme oeuvre Dada, drame baroque ou pièce àclef, est une oeuvre dont l’étude de la création et la réception permet de mieuxappréhender les liens de Paul Claudel avec son temps. Dans cette pièce, Claudeleffectue la somme de l’héritage artistique de remise en cause de la société positiviste desa jeunesse et livre le récit de sa vocation poétique, enracinée dans la penséebaudelairienne et mallarméenne sur la modernité. La prégnance du paradigme picturalpour livrer son manifeste esthétique et l’utilisation de l’image sous toutes ses formespour entrer en dialogue avec le sens du texte vérifient la concordance d’intérêts entreClaudel et les artistes de sa génération. L’originalité du dramaturge réside dans sonrecours à l’image pour faire de son oeuvre un ex-voto qui retrace l’itinéraire de saconversion, l’inspiration thomiste le conduisant à privilégier l’image dans l’économiedu salut à l’oeuvre dans la pièce. L’oeuvre révèle enfin son affinité avec l’art modernepar son utilisation de procédés et de formes propres à l’art moderne, comme le collageet le montage, ou redécouverts par la modernité, comme l’empreinte ou la planéité,procédés et formes que Claudel emploie de manière polysémique et dialectique. Eninscrivant la question de sa vocation poétique, de sa conversion et de la réception de sonoeuvre par ses contemporains au coeur de la pièce, avec Le Soulier de satin, Claudeldonne une oeuvre qui s’inscrit pleinement dans le programme de la modernité tout enrévélant son originalité propre
The Satin Slipper has been received alternately as a Dada work, a Baroque drama or a‘pièce à clé’, and studying both its first production and its reception allows one to graspbetter Claudel’s relationships with his days. In this work, Claudel adds up together theartistic legacy of his questioning the positivist society of his youth and gives thenarrative of his poetic vocation, which is rooted in the Baudelairean and Mallarméanthoughts on modernity. Claudel delivers his aesthetic manifesto through the primacy ofthe pictural paradigm and makes the reader establish a dialogue with the meaning of thetext, and all this makes him meet the concerns of the artists of his generation. Theplaywright’s originality lies in his resorting to the image to make his work into an exvoto that traces the itinerary of his conversion, as the Thomistic inspiration drives himto favour the image, in God’s plan at work in the play. Ultimately, the work discloses itscloseness to modern art through the use of devices and forms that are proper to modernart, such as collages and montages, or that were rediscovered by modernity, like theimprint or the planeness, that Claudel uses in a polysemous and dialectical way. As heincludes the questions of his poetic vocation, his conversion and the reception of hiswork by his contemporaries in the heart of the play, Claudel thrusts The Satin Slipperdeeply into the programme of modernity, original though it may be revealed
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Michaelides, Katerina. "Bernard-Soulier syndrome : genetic studies from man to mouse." Thesis, Imperial College London, 2006. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.428065.

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De, Rocco Daniela, and Rocco Daniela De. "STUDIO CLINICO E MOLECOLARE DELLA SINDROME DI BERNARD-SOULIER." Doctoral thesis, Università degli studi di Trieste, 2015. http://hdl.handle.net/10077/10848.

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2013/2014
2013/2014
La sindrome di Bernard-Soulier (BSS) è una rara piastrinopenia ereditaria causata da alterazioni a livello del complesso glicoproteico GPIb-IX-V, presente sulla membrana piastrinica e responsabile della adesione delle piastrine in seguito a danno vascolare. La BSS si trasmette come malattia autosomica recessiva (BBSA1) e i pazienti affetti presentano piastrine giganti e severi episodi di sanguinamento. Tuttavia in tempi recenti sono state descritte delle famiglie con una forma dominante nota come BSSA2. In questi pazienti la piastrinopenia è moderata e le piastrine presentano un volume leggermente aumentato. Finora sono state individuate solo 5 varianti in eterozigosi nel BSSA2:, 4 nel gene GP1BA e 1 in GP1BB. Fatta eccezione per p.Ala172Val del gene GP1BA che è relativamente frequente nella la popolazione Italiana, le altre 4 sono state descritte in singole famiglie. I pochi casi di cui disponiamo, soprattutto per la forma recessiva non ci permettono di avere informazioni sui meccanismi patogenetici e sulla sua evoluzione nel tempo. Per questo motivo è stato istituito un Consorzio Internazionale per lo studio della BSS grazie al quale è stato possibile raccogliere i dati clinici e molecolari di 132 famiglie. Tutte le informazioni sono state inserite in un database (BSS Consortium database) attualmente gestito dal nostro laboratorio e consultabile dai gruppi di studio che hanno aderito al Consorzio. Inoltre per aumentare le informazioni sulle varianti identificate nel BSSA1 abbiamo incrementato i dati molecolari delle famiglie del Consorzio con i dati di altre 79 famiglie descritte in letteratura, raggiungendo un totale di 211 famiglie. Tutte le mutazioni identificate in queste famiglie sono state poi inserite in un database pubblico disponibile in rete (LOVD: Leiden Open Variation Database). La raccolta e l’elaborazione dei dati ci ha permesso di chiarire alcuni aspetti clinici e molecolari della malattia. Tuttavia data l’eterogeneità genetica e l’elevata espressione fenotipica gli studi genotipo-fenotipo si sono rivelati difficili da eseguire. Nonostante le molte informazioni acquisite, il database risulta ancora incompleto e limitato; per questo motivo è necessario raccogliere nuovi casi e inserire assieme alle varianti anche i relativi studi funzionali che si rivelano indispensabili per poter definire l’effetto delle varianti sul complesso GPIb-IX-V. Nell’ambito invece dello studio e caratterizzazione della forma meno grave di BSS (BSSA2) sono stati selezionati 120 pazienti piastrinopenici senza diagnosi caratterizzati da piastrine grandi. In questi pazienti sono stati analizzati i geni GP1BA, GP1BB e GP9 e sono state identificate 11 diverse varianti: 1 nonsense, 2 mutazioni di framshift, 1 mutazione nel codone di inizio e 5 varianti missense. Gli studi funzionali eseguiti sulle varianti missense per stabilire il loro ruolo patogenetico sono ancora in corso. Tuttavia se gli studi dovessero confermare la loro patogenicità 11 pazienti su 120 risulterebbero BSSA2 e questa forma dovrebbe essere considerata una tra le piastrinopenie ereditarie più frequenti in Italia. In conclusione grazie a questo studio è stato possibile raccogliere la più ampia casistica di pazienti affetti da BSSA1 fin’ora descritta e ottenere numerose informazioni sia sulla clinica che sulle mutazioni coinvolte. Il BSS Consortium database permetterà ai clinici che hanno partecipato allo studio di osservare nel tempo l’andamento della malattia nei pazienti e di ottenere informazioni utili per stabilire un corretto protocollo per la presa in carico dei pazienti. Infine la caratterizzazione di nuove forme di BSSA2 rappresenta il punto di partenza per descrivere al meglio la malattia BSSA2 sia dal punto di vista clinico che molecolare. In futuro sarà quindi indispensabile estendere il BSS Consortium database anche alla forma BSSA2.
XXVII Ciclo
XXVII Ciclo
1979
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Gerisch, Sebastian [Verfasser], and Barbara [Akademischer Betreuer] Zieger. "Molekulargenetische Untersuchung von Patienten mit Bernard-Soulier-Syndrom und Morbus Glanzmann." Freiburg : Universität, 2013. http://d-nb.info/1123474486/34.

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BOUDIER, ERIC. "Contribution a l'etude du syndrome des anticorps antiphospholipidiques : a propos de 46 grossesses et 14 patientes." Besançon, 1992. http://www.theses.fr/1992BESA3011.

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Soulier, Marcel Verfasser], and Daniel [Akademischer Betreuer] [Goldmann. "Entwicklung systemdynamischer Stoffstrommodelle zur Simulation von regionalen Kupferkreisläufen / Marcel Soulier ; Betreuer: Daniel Goldmann." Clausthal-Zellerfeld : Technische Universität Clausthal, 2018. http://d-nb.info/1231364157/34.

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Kilpatrick, Robert M. "Le soulier de theramenez theory and practice of the adage in Erasmus and Montaigne /." [Bloomington, Ind.] : Indiana University, 2009. http://gateway.proquest.com/openurl?url_ver=Z39.88-2004&rft_val_fmt=info:ofi/fmt:kev:mtx:dissertation&res_dat=xri:pqdiss&rft_dat=xri:pqdiss:3378360.

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Thesis (Ph.D.)--Indiana University, Dept. of French & Italian Studies, 2009.
Title from PDF t.p. (viewed on Jul 7, 2010). Source: Dissertation Abstracts International, Volume: 70-10, Section: A, page: 3876. Adviser: Eric MacPhail.
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Lapointe, Marie-Michelle. "Du soulier à la lucarne : où, quand et comment les buts sont marqués au soccer." Thèse, Université du Québec à Trois-Rivières, 2014. http://depot-e.uqtr.ca/7398/1/030768799.pdf.

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Books on the topic "Soulier"

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Coleno, Nadine. Roger Vivier: D'un soulier l'autre. Paris: Regard, 2005.

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Le soulier de satin: Essais sur le texte et l'écriture du Soulier de satin. Paris: Belles-Lettres, 1986.

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Autrand, Michel. Le soulier de satin: Étude dramaturgique. Paris: Champion-Slatkine, 1987.

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Le soulier de satin: Étude dramaturgique. Paris: H. Champion, 1987.

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Autrand, Michel. Le soulier de satin de Paul Claudel. [Paris]: Gallimard, 1997.

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Weber-Caflisch, Antoinette. Le soulier de satin de Paul Claudel. Paris: Les Belles Lettres, 1987.

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Rêve et réalité dans Le soulier de satin. Rennes: Presses universitaires de Rennes, 2005.

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Millet-Gérard, Dominique. Formes baroques dans "Le Soulier de satin": Étude d'esthétique spirituelle. Paris: H. Champion, 1997.

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Recoing, Eloi. Journal de bord: Le soulier de satin, Paul Claudel, Antoine Vitez. [Paris]: Le Monde, 1991.

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Autrand, Michel. Le dramaturge et ses personnages dans Le soulier de satin de Paul Claudel. Paris: Lettres modernes, 1987.

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Book chapters on the topic "Soulier"

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Ghasemi, Bahare, and Akbar Dorgalaleh. "Bernard-Soulier Syndrome." In Congenital Bleeding Disorders, 357–77. Cham: Springer International Publishing, 2018. http://dx.doi.org/10.1007/978-3-319-76723-9_15.

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Beighton, Peter, and Greta Beighton. "Soulier, J.-P." In The Person Behind the Syndrome, 221. London: Springer London, 1997. http://dx.doi.org/10.1007/978-1-4471-0925-9_165.

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Clemetson, K. J., and R. E. Scharf. "Bernard-Soulier-Syndrom." In Hämostaseologie, 53–56. Berlin, Heidelberg: Springer Berlin Heidelberg, 1999. http://dx.doi.org/10.1007/978-3-662-07673-6_6.

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Orbach-Zinger, Sharon, Atara Davis, and Alexander Ioscovich. "Bernard–Soulier Syndrome." In Consults in Obstetric Anesthesiology, 89–91. Cham: Springer International Publishing, 2018. http://dx.doi.org/10.1007/978-3-319-59680-8_23.

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Leung, Alexander K. C., Marcus Schmitt, Christie P. Thomas, Cord Sunderkötter, Meinhard Schiller, Thomas Schwarz, Mark Berneburg, et al. "Bernard-Soulier Syndrome." In Encyclopedia of Molecular Mechanisms of Disease, 214–15. Berlin, Heidelberg: Springer Berlin Heidelberg, 2009. http://dx.doi.org/10.1007/978-3-540-29676-8_7294.

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Panzer, S., and H. Niessner. "Thrombozytenmembrandefekte — Das Bernard-Soulier-Syndrom." In 17. Hämophilie-Symposion, 284–90. Berlin, Heidelberg: Springer Berlin Heidelberg, 1988. http://dx.doi.org/10.1007/978-3-642-72830-3_60.

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Oette, Mark, Marvin J. Stone, Hendrik P. N. Scholl, Peter Charbel Issa, Monika Fleckenstein, Steffen Schmitz-Valckenberg, Frank G. Holz, et al. "Macrothrombocytopenia, Familial, Bernard-Soulier Type." In Encyclopedia of Molecular Mechanisms of Disease, 1244. Berlin, Heidelberg: Springer Berlin Heidelberg, 2009. http://dx.doi.org/10.1007/978-3-540-29676-8_7299.

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Schäfer, Christian, and KLL. "Claudel, Paul: Le soulier de satin." In Kindlers Literatur Lexikon (KLL), 1–2. Stuttgart: J.B. Metzler, 2020. http://dx.doi.org/10.1007/978-3-476-05728-0_3178-1.

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Ghasemi, Bahare, and Akbar Dorgalaleh. "Bernard-Soulier Syndrome: Diagnosis and Management." In Congenital Bleeding Disorders, 423–44. Cham: Springer International Publishing, 2023. http://dx.doi.org/10.1007/978-3-031-43156-2_16.

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Rose, Melissa J., and Amanda Jacobson-Kelly. "Care of a Toddler with Epistaxis and Bernard-Soulier Syndrome." In Pediatric Bleeding Disorders, 171–82. Cham: Springer International Publishing, 2020. http://dx.doi.org/10.1007/978-3-030-31661-7_16.

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Conference papers on the topic "Soulier"

1

Nichols, W. L., S. E. Kaese, D. A. Gastineau, L. A. Otteman, and E. J. W. Bowie. "BERNARD-SOULIER SYNDROME: WHOLE BLOOD DIAGNOSTIC ASSAYS OF PLATELETS." In XIth International Congress on Thrombosis and Haemostasis. Schattauer GmbH, 1987. http://dx.doi.org/10.1055/s-0038-1644561.

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Diagnosis of Bernard-Soulier syndrome (BSS) is complicated by the difficulty of separating the giant platelets from other blood cells to pursue analyses of platelet function and structure. We report on the utility of three whole blood assay techniques for diagnosis of a patient with BSS. To our knowledge, these three techniques have not been simultaneously applied or compared for efficacy in laboratory diagnosis of BSS. (1) Whole blood platelet aggregation responses, studied with an electrical impedence aggregometer, were equivalent to those more laboriously obtained using platelet-rich plasma prepared by unit gravity sedimentation, studied with an optical light transmittance aggregometer. Platelet aggregation responses were normal with ADP or collagen stimulation, and absent with Ristocetin or bovine plasma stimulation. (2) Whole blood radioimmunoassay of platelet glycoprotein (GP) expression was performed using iodinated murine monoclonal antibodies HP1-1D (anti-GP IIb/IIIa) and 6D1 (anti-GPlb, kindly supplied by Dr. Barry Coller, Stony Brook, NY). After incubation with citrated whole blood, centrifugation was used to separate cell-bound antibody which was quantitated with a gamma counter. The patient’s whole blood had a normal level of cell-bound GP Ilb/IIIa, but a markedly reduced level of cell-bound GP lb (5% of normal mean; n = 20). (3) Whole blood smear immunocytochemical staining with the monoclonals (indirect immuno-alkaline phosphatase technique), and qualitative analysis by light microscopy, revealed a marked reduction of GP lb expression by the patient’s giant platelets, whereas GP Ilb/IIIa expression was normal. This latter technique might be especially valuable as a screening technique when the patient is not directly available for laboratory study. Together with the patient’s life-long history of thrombocytopenia and moderate bleeding diathesis, and other laboratory observations including markedly prolonged bleeding times and reduced whole blood prothrombin consumption, these data established diagnosis of BSS. We conclude that these three relatively simple assays of platelets in whole blood should be of particular value in the laboratory differential diagnosis of patients with congenital thrombocytopenias and giant platelet syndromes.
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2

Cunha, Vitor, and ÂNGELA CRISTINA SOARES SILVA. "DIÁTESES HEMORRÁGICAS COM POSSÍVEL ASSOCIAÇÃO AO SÍNDROME DE BERNARD-SOULIER (SBS)." In III Congresso Brasileiro de Hematologia Clínico-laboratorial On-line. Revista Multidisciplinar em Saúde, 2023. http://dx.doi.org/10.51161/hematoclil2023/15933.

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3

Fox, J. E. B., C. C. Reynolds, J. K. Boyles, R. A. Abel, and M. M. Johnson. "IDENTIFICATION OF GLYCOPROTEIN Ib8 AS THE Mr = 24,000 PLATELET POLYPEPTIDE PHOSPHORYLATED BY AGENTS THAT ELEVATE CYCLIC AMP." In XIth International Congress on Thrombosis and Haemostasis. Schattauer GmbH, 1987. http://dx.doi.org/10.1055/s-0038-1642926.

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Platelet function is inhibited by agents that elevate intracellular cyclic AMP concentrations, presumably as a result of the cyclic AMP-stimulated phosphorylation of intracellular proteins. Polypeptides that become phosphorylated are of Mr = 250,000, Mr = 51.000 (P51), Mr = 36,000 (P36), Mr = 24,000 (P24), and Mr = 22.000 (P22). The Mr = 250,000 polypeptide is actin-binding protein, but the identity of the other polypeptides 1s unknown. In the present study, we identified the P24 polypeptide. Platelets were radiolabeled with [32P]P1 and then Incubated for 2-5 min in the presence or absence of 5 μM prostaglandin E1 (PGE1). The PGE1-induced phosphorylation of P24 was detected on autoradiograms of SDS-gels. Since P24 has been shown to be membrane-associated, its molecular weight was compared with those of known membrane proteins. P24 comigrated with the β-chain of purified GP Ib on reduced gels (Mr = 24,000) and also on nonreduced gels (when GP Ibβ is disulfide-linked to GP Ibα and migrates with Mr = 170,000). Like GP Ibβ, P24 was associated with actin filaments in Triton X-100 lysates. Both GP Ibβ and P24 were selectively associated with filaments of the membrane skeleton and were released from filaments when the Ca2+-dependent protease was active. Antibodies against GP Ib immunoprecipitated P24 from platelet lysates. Finally, exposure of Bernard-Soulier platelets (that lacked GP Ib) to PGE1 resulted in phosphorylation of actin-binding protein, P51, P36, and P22, but not P24. We conclude that P24 is GP Ibβ. To determine whether phosphorylation of GP Ibβ is responsible for the inhibitory effects of PGE1 on platelets, we compared the action of PGE1 on control platelets with that on Bernard-Soulier platelets. One of the ways in which PGE1 inhibits platelet activation is by inhibiting the polymerization of actin. While PGE1 inhibited actin polymerization in control platelets, it did not in Bernard-Soulier platelets. We conclude that GP Ibβ is phosphorylated by agents that elevate cyclic AMP and that phosphorylation of this glycoprotein results in inhibition of platelet function.
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4

Holzapfel, J., N. Stadler, M. Frühwald, S. Halimeh, and M. Olivieri. "Joint Bleed as an uncommon bleeding event in a 7-years old Patient with Bernard-Soulier-Syndrom." In GTH Congress 2024 – 68th Annual Meeting of the Society of Thrombosis and Haemostasis Research – Building Bridges in Coagulation. Georg Thieme Verlag, 2024. http://dx.doi.org/10.1055/s-0044-1779163.

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5

Nagayama, R., A. Hattori, I. Fuse, T. Takeshige, S. Takizawa, and A. Shibata. "PLATELET IONIZED CALCIUM MOBILIZATION (AEQUORIN METHOD) IN PATIENTS WITH PRIMARY PLATELET DYSFUNCTION." In XIth International Congress on Thrombosis and Haemostasis. Schattauer GmbH, 1987. http://dx.doi.org/10.1055/s-0038-1644571.

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Intracellular calcium level of platelets of the patients with primary platelet dysfunction (thrombasthenia, platelet cyclo-oxygenase deficiency, familial defect of A23187 induced platelet aggregation, Hermansky-Pudlak syndrome. Bernard-Soulier syndrome, each 1 case, and other 3 cases of platelet release mechanism defect of unknown etiology) were measured with the photoprotein Aequorin according to the method by Johnson et al. The peak level and the lag time to the peak were evaluated. Activation was done by 4 or more different concentrations of either thrombin (0.125-1.0μ/ml), A23187 (0.25-2.0μM), ATA2 (0.05-0.4μM) or occasionally arachidonate (0.25-100μM).In case of stimulation by thrombin, the maximum [Ca2+] level in thrombasthenia was much lower than those in normal. The lag time was prolonged in Bernard-Soulier syndrome. In case of stimulation by STA2 the maximum [Ca2+] level was very much lower in thrombasthenia and was lower in a familial defect of A23187-induced platelet aggregation and a case of platelet release mechanism defect than normals. In case of stimulation by A23187, the maximum [Ca2+] level was much lower in thrombasthenia and PCO deficiency and platelet release mechanism defect (2 cases) in which the lag time of A23187 induced platelet aggregation was also prolonged. In PCO deficiency, arachodonate less than μM produced a dose-dependent rise in intracellular calcium level and that (1-25μM) caused a rise of a consistent level although it didn't induce aggregation. Arachidonate (25-100M) caused both higher rise and aggregation. Mobilization was normal in response to STA2 and thrombin but decreased to A23187 in this deficiency.These findings suggest that [Ca2+] mobilization was deteriorated by many mechanisms such as defect of PGG2H2-and Tx-forma-tion, membranous abnormality (lack of glycoproteins) and storage pool deficiency, and further that atachidonate even at low concentration may cause mobilization without conversion to PGG2H2 or Tx.
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6

Hourdillé, P., F. Belloc, E. Heilmann, M. Pico, and A. T. Nurden. "MEGAKARYOCYTES FROM THE MARROW OF A PATIENT WITH BERNARD-SOULIER SYNDROME LACKED GP Ib AND WERE DEFICIENT IN GP IX." In XIth International Congress on Thrombosis and Haemostasis. Schattauer GmbH, 1987. http://dx.doi.org/10.1055/s-0038-1644562.

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Although it is recognized that glycoprotein (GP) Ib/IX complexes are deficient in the platelets of patients with the Bernard-Soulier syndrome (BSS), the nature of the genetic defect remains unknown. We have looked for these GPs in permeabilized megakaryocytes (MK) of a BSS patient employing immunofluorescence (IF) or immunocytochemical procedures combined with electron microscopy. The study involved the use of monoclonal antibodies AP-1 (anti-GP Ib), AP-2 (anti-GP IIb-IIIa) and FMC 25 (anti-GP IX), gifts from Drs. T. Kunicki and M. Berndt respectively. Bound IgG were revealed by biotinylated anti-mouse IgG followed by Texas Red-streptavidin and MK identified in IF by a double-staining procedure using a polyclonal antibody to fibrinogen (Fg). Platelet morphology was typical of BSS with a high percentage of "giant" platelets. Flow cytometry confirmed that platelets of all sizes were negative for AP-1 and FMC 25 but normally bound AP-2. MK from a marrow aspirate obtained by sternal puncture were concentrated on a Percoll gradient. Electron microscopy showed the MK to be of normal size with a normal granule distribution. However, a striking feature was an irregular distribution of the demarcation membranes which often had a vacuolar appearance. Whereas all permeabilized MK from normal individuals were strongly fluorescent with AP-2, AP-1 and FMC 25, those from the BSS patient were negative for AP-1, weakly positive for FMC 25 and normal for AP-2. Incubation of intact cells with AP-1, with bound antibody located by anti-IgG bound to gold particles, confirmed the absence of GP lb from the surface membranes of BSS MK. Our results show that the platelet membrane GP defect in BSS results from an abnormal synthesis and/or stability of both GP lb and GP IX in the MK.
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7

Estry, D. W., J. C. Mattson, and J. Chediak. "CONTACT ACTIVATED PLATELETS BIND VON WILLEBRAND FACTOR TO GPIIb-IIIa." In XIth International Congress on Thrombosis and Haemostasis. Schattauer GmbH, 1987. http://dx.doi.org/10.1055/s-0038-1643517.

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Using a rabbit polyclonal anti-von Willebrand factor (vWF) antibody, normal human adherent platelets extensively bind vWF in a diffuse pattern as detected by immunogold electron microscopy. This pattern differed significantly from the zonal pattern observed for direct fibrinogen-gold labelling in contact activated platelets. In order to determine if contact activated platelets bind vWF to GPIIb-IIIa or GPlb, the extent and pattern of bound vWF in platelets from patients with Glanzmann’s thrombasthenia (GT) and Bernard Soulier Syndrome (BSS) was determined. Virtually no bound VWF was detected by immunogold labeling in GT platelets previously characterized as being deficient in GPIIb-IIIa. On the other hand, BSS platelets, lacking GPlb, demonstrated extensive labeling of vWF in a pattern identical to that seen in normal platelets. This data is consistant with vWF binding to GPIIb-IIIa in contact induced adhesion and spreading.
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8

Yasunaga, K. "HEREDITARY PLATELET FUNCTION DISORDERS IN JAPAN." In XIth International Congress on Thrombosis and Haemostasis. Schattauer GmbH, 1987. http://dx.doi.org/10.1055/s-0038-1644876.

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Nationwide surveys of hereditary platelet function disorders in Japan were carried out in 1976, 1981, and 1986. Information on 271 cases received in the 1986 survey was analyzed with that for 103 other cases reported in earlier surveys but not in 1986, making a total of 374 cases. The mortality rate was 6.8% of 162 cases in 1976, 6.6% of 213 cases in 1981, and 5.9% of 374 cases in 1986. Bleeding symptoms appeared at age 12 years in 56.8% of patients and the most common were epistaxis and purpura. Of the 295 cases 49.5% were isolated cases, 20.3% had siblings with confirmed bleeding tendencies, and 30.2% had other kin with bleeding tendencies, suggestings autosomal tnansmission. Consanguineous marriage was reported by 11.9% of patients.Of the 374 cases in 1986, 59.4% were thrombasthenia (TA), 11.5% Bernard-Soulier symdrome (BSS), 22.5% release abnormalities (PRA), 1.3% other, and 5.3% unclassified. Of the 84 cases of PRA, 60 were storage pool deficiency, 18 release mechanism abnormalities, and 6 undecided between the two types. The resultsof laboratory tests were as follows .
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9

Okita, J. R., M. M. Frojmovic, S. Kristopeit, T. Wong, and T. J. Kunicki. "MONTREAL PLATELET SYNDROME: DECREASED ACTIVITY OF PLATELET CALPAINS ASSOCIATED WITH AGGREGATION ABNORMALITIES." In XIth International Congress on Thrombosis and Haemostasis. Schattauer GmbH, 1987. http://dx.doi.org/10.1055/s-0038-1642822.

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The Montreal platelet syndrome (MPS) is an inherited disorder of platelet function characterized by a) severe thrombocytopenia, b) formation of "giant" platelets upon physical or biochemical stimulation, c) spontaneous aggregation (stir-induced microaggregate formation) and d) a lack of aggregation in response to thrombin. The Bernard-Soulier syndrome (BSS) is similar to MPS in that both syndromes are characterized by "giant" platelets and an abnormal aggregation response to thrombin. BSS patients have a deficiency of specific platelet glycoproteins (GPs). From our investigations we conclude MPS patients have apparently normal amounts of major platelet GPs. However, a defect in calpains (calcium-activated neutral proteinases) was detected in MPS platelets. The specific activity of calpains was decreased by 70% (p<0.001) in the cytosolic fraction of platelets from MPS patients as compared to that of platelets from normal control donors. The calpain activity of platelets from BSS patients was within the normal range.During the course of the biochemical studies, platelets from the MPS patients were shown to exhibit concurrent functional defects, i.e., stir-induced spontaneous aggregation and reduced to absent aggregation response to thrombin. It is concluded that MPS can be distinguished from BSS at the molecular level as follows: 1) MPS platelets contain normal amounts of GPs Ib, V and IX which are decreased or absent in BSS platelets; 2) The specific activity of calpains is reduced in MPS platelets but normal in BSS platelets. Supported by NIH (HL-33925, HL-32279), Amer. Heart Assoc. (85—GA—67, 83-186), Canadian Med. Res. Council (248-59) and Quebec Heart Fndn.
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10

Carroll, R. C. "OPPOSITIONAL REGULATION OF PLATELET CALCIUM FLUX BY cAMP-MEDIATED PHOSPHORYLATION OF GLYCOPROTEIN lb." In XIth International Congress on Thrombosis and Haemostasis. Schattauer GmbH, 1987. http://dx.doi.org/10.1055/s-0038-1643630.

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Glycoprotein (GP) lb, a surface receptor for von Willebrand's factor and thrombin, is composed of two disulfide-linked subunits (alpha - 130 kDa; and beta - 25 kDa). No significance has yet been attributed to the finding that the GPIb beta subunit is the only platelet glycopeptide which is phosphorylated in situ. GPIb can be quantitatively isolated from Triton X-100 wfibTeTlatelet lysates by monoclonal antibody (AN51) to GPIb and Protein A-Sepharose. Using this technique, I find that phosphorylation of the GPIb-beta subunit increased up to three-fold upon exposure of 32P-labeled platelets to agents which increase .platelet cAMP levels. In fact, this glycopeptide is identical to the phospho-peptide identified as 24P in a previous study (Cox et al., J. Cell Biol. 98:8-15, 1984). The phosphorylation of 24P was shown to correlate with cAMP-mediated reversal of platelet activation. This phosphopeptide has also been linked to enhanced calcium uptake by microsomes from platelets pretreated with cAMP elevating agents relative to untreated controls ( Fox et al_., Biochem. J. 184:651-666, 1979). I have further fractionatecTthese membrane fractions by centrifugation on 15% to 55% sucrose gradients and correlated a better than 5-fold enhanced calcium uptake with membrane fractions enriched in phosphorylated GPIb-beta. Confirming evidence was obtained using Bernard-Soulier platelets missing the GPIb complex. These platelets not only lack the 24P phosphopeptide, their activation is not readily reversible by cAMP elevating agents. These studies suggest a major role for GPIb in the inhibition/reversal of platelet activation by cAMP. My hypothesis is that GPIb phosphorylation either stimulates calcium efflux or blocks influx across the platelet plasma membrane.
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