Glanzmann's thrombasthenia: Difference between revisions

	Glanzmann's thrombasthenia
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Latest revision as of 06:33, 28 July 2020

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Omer Kamal, M.D.[2], Niyousha Danesh M.D., M.P.H.

Synonyms and keywords: Glanzmann thrombasthenia; Glanzmann's disease

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Glanzmann's thrombasthenia from Other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Treatment

Case Studies

Case #1

@@ Line 1: / Line 1: @@
 __NOTOC__
+{{Glanzmann's thrombasthenia}}
-{{SI}}
+{{CMG}} {{AE}} {{OK}}, [https://www.wikidoc.org/index.php/User:Niush.D?venotify=created Niyousha Danesh M.D., M.P.H].'''
-{{CMG}}; {{AE}} {{JH}}.
-==Overview==
+{{SK}} Glanzmann thrombasthenia; Glanzmann's disease
-'''Glanzmann's thrombasthenia''' is an extremely rare [[Blood diseases|disorder]] of the [[blood]], in which the [[platelet]]s lack [[glycoprotein IIb/IIIa]]. Hence, no [[fibrinogen]] bridging can occur, and bleeding time is significantly prolonged.
-==Pathophysiology==
+==[[Glanzmann's thrombasthenia patient information|Patient Information]]==
-The [[GpIIb/IIIa]] is an adhesion receptor and is expressed in [[thrombocytes]]. This receptor is activated when the thrombocyte is stimulated by [[Adenosine diphosphate|ADP]], [[epinephrine]], [[collagen]] and [[thrombin]]. The [[GpIIb/IIIa]] [[integrin]] is essential to the [[blood coagulation]] since it has the ability to bind [[fibrinogen]], the [[von Willebrand factor]], [[fibronectin]] and [[vitronectin]]. This enables the platelet to be activated by contact with the collagen-von Willebrand-complex that is exposed when the endothelial blood vessel lining is damaged and then aggregate with other [[thrombocytes]] via [[fibrinogen]].
-Patients suffering from Glanzmann's thrombasthenia thus have platelets less able to adhere to each other and to the underlying tissue of damaged blood vessels.
+==[[Glanzmann's thrombasthenia overview|Overview]]==
-The understanding of its pathophysiology led to the development of [[GpIIb/IIIa inhibitors]], a class of powerful [[antiplatelet agent]]s.<ref name=seligsohn>Seligsohn U. Glanzmann thrombasthenia: a model disease which paved the way to powerful therapeutic agents. Pathophysiol Haemost Thromb. 2002 Sep-Dec;32(5-6):216-7. PMID 13679645. [http://content.karger.com/ProdukteDB/produkte.asp?Aktion=ShowPDF&ArtikelNr=73569&ProduktNr=224034&Ausgabe=229381&filename=73569.pdf Free Full Text].</ref>
+==[[Glanzmann's thrombasthenia historical perspective|Historical Perspective]]==
-==Etiology==
+==[[Glanzmann's thrombasthenia classification|Classification]]==
-Glanzmann's thrombasthenia can be inherited in an [[autosomal]] [[recessive]] manner<ref name=seligsohn/> or acquired as an [[autoimmune disorder]].<ref>Tholouli E, Hay CR, O'Gorman P, Makris M. Acquired Glanzmann's thrombasthenia without thrombocytopenia: a severe acquired autoimmune bleeding disorder. Br J Haematol. 2004 Oct;127(2):209-13. PMID 15461628.</ref>
-==Clinical Features==
+==[[Glanzmann's thrombasthenia pathophysiology|Pathophysiology]]==
-Characteristically, there is increased mucosal bleeding:
-*[[epistaxis]]
-*[[menorrhagia]]
-*increased bleeding post-operatively.
-The bleeding tendency is variable but may be severe.
-Patients present with moderate [[bleeding]] and normal [[platelet]] morphology. [[Aggregation]] of [[platelet]]s occurs in response to [[ristocetin]], but not to other [[agonist]]s such as [[Adenosine diphosphate|ADP]], [[thrombin]], [[collagen]] or [[adrenalin]].
+==[[Glanzmann's thrombasthenia causes|Causes]]==
-==Eponym==
+==[[Glanzmann's thrombasthenia differential diagnosis|Differentiating Glanzmann's thrombasthenia from Other Diseases]]==
-It is named for Eduard Glanzmann.<ref>{{WhoNamedIt|synd|1289}}</ref><ref>W. E. Glanzmann. Hereditäre hämorrhägische Thrombasthenie. Ein Beitrag zur Pathologie der Blutplättchen. Jahrbuch für Kinderheilkunde, 1918; 88: 1-42, 113-141.</ref>
-==References==
+==[[Glanzmann's thrombasthenia epidemiology and demographics|Epidemiology and Demographics]]==
-{{reflist|2}}
+==[[Glanzmann's thrombasthenia risk factors|Risk Factors]]==
+==[[Glanzmann's thrombasthenia screening|Screening]]==
+==[[Glanzmann's thrombasthenia natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
+==Diagnosis==
+[[Glanzmann's thrombasthenia diagnostic study of choice|Diagnostic Study of Choice]]
+| [[Glanzmann's thrombasthenia history and symptoms|History and Symptoms]]
+| [[Glanzmann's thrombasthenia physical examination|Physical Examination]]
+| [[Glanzmann's thrombasthenia laboratory findings|Laboratory Findings]]
+| [[Glanzmann's thrombasthenia electrocardiogram|Electrocardiogram]]
+| [[Glanzmann's thrombasthenia chest x ray|Chest X Ray]]
+| [[Glanzmann's thrombasthenia echocardiography and ultrasound|Echocardiography and Ultrasound]]
+| [[Glanzmann's thrombasthenia CT|CT]]
+| [[Glanzmann's thrombasthenia MRI|MRI]]
+| [[Glanzmann's thrombasthenia other imaging findings|Other Imaging Findings]]
+| [[Glanzmann's thrombasthenia other diagnostic studies|Other Diagnostic Studies]]
+==Treatment==
+[[Glanzmann's thrombasthenia medical therapy|Medical Therapy]]
+| [[Glanzmann's thrombasthenia surgery|Surgery]]
+| [[Glanzmann's thrombasthenia primary prevention|Primary Prevention]]
+| [[Glanzmann's thrombasthenia secondary prevention|Secondary Prevention]]
+| [[Glanzmann's thrombasthenia cost-effectiveness of therapy|Cost-Effectiveness of Therapy]]
+| [[Glanzmann's thrombasthenia future or investigational therapies|Future or Investigational Therapies]]
+==Case Studies==
+[[Glanzmann's thrombasthenia case study one|Case #1]]
+[[Category:Disease]]
+[[Category:Hematology]]

Glanzmann's thrombasthenia: Difference between revisions

Latest revision as of 06:33, 28 July 2020

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