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=== Genetics ===
=== Genetics ===
* [[Ehlers-Danlos syndrome]] (EDS) forms part of a spectrum of genetically based [[connective tissue disorders]] that includes [[osteogenesis imperfecta]]. [[Ehlers-Danlos type IV syndrome|Vascular Ehlers-Danlos syndrome (EDS IV)]] is characterized by an alteration in the [[Ehlers-Danlos syndrome|COL3A1 gene]]. This gene encodes type III [[collagen]] and The alteration of this type of collagen produces aneurysms and ruptures of vessels and organs that can lead to haemothorax.
* [[Ehlers-Danlos syndrome]] (EDS) forms part of a spectrum of genetically based [[connective tissue disorders]] that includes [[osteogenesis imperfecta]]. [[Ehlers-Danlos type IV syndrome|Vascular Ehlers-Danlos syndrome (EDS IV)]] is characterized by an alteration in the [[Ehlers-Danlos syndrome|COL3A1 gene]]. This gene encodes type III [[collagen]] and The alteration of this type of collagen produces [[aneurysm]]s and ruptures of vessels and organs that can lead to haemothorax.


* [[Hereditary hemorrhagic telangiectasia|Osler–Weber–Rendu disease]], also known as [[hereditary hemorrhagic telangiectasia]] (HHT), is an [[Genetic Disorders|autosomal dominant disease]] characterized by multiple [[Skin|cutaneous]], systemic, and/or [[Lung|pulmonary]] [[arteriovenous malformations]] (AVMs). Bleeding from pulmonary lesions usually occur as [[Hemoptysis|hemoptysis]] and rarely as Spontaneous Haemothorax. There is an association between Osler-Weber-Rendu disease and bilateral heterochronic spontaneous hemothorax.
* [[Hereditary hemorrhagic telangiectasia|Osler–Weber–Rendu disease]], also known as [[hereditary hemorrhagic telangiectasia]] (HHT), is an [[Genetic Disorders|autosomal dominant disease]] characterized by multiple [[Skin|cutaneous]], systemic, and/or [[Lung|pulmonary]] [[arteriovenous malformations]] (AVMs). [[Bleeding]] from pulmonary lesions usually occur as [[Hemoptysis|hemoptysis]] and rarely as spontaneous haemothorax. There is an association between Osler-Weber-Rendu disease and bilateral heterochronic spontaneous hemothorax.


* [[Rib|Coastal]] [[exostosis]] or [[Osteochondroma]] is an [[autosomal dominant]] [[hereditary]] abnormality and the most common benign thoracic bone tumor. It often presents singly or in multiple sites that can cause laceration of the lung and hemothorax.
* [[Rib|Coastal]] [[exostosis]] or [[Osteochondroma]] is an [[autosomal dominant]] [[hereditary]] abnormality and the most common benign [[thorax|thoracic]] bone tumor. It often presents singly or in multiple sites that can cause laceration of the lung and hemothorax.


* Hemophilia A is a [[X-linked]] hereditary disorder of blood clotting that caused by the development of an inhibitor against [[Coagulation factor|coagulation factor VIII]] (FVIII). Hemophilia A manifests with early muscle and subcutaneous bleeding and rarely with haemothorax.
* Hemophilia A is a [[X-linked]] hereditary disorder of blood clotting that caused by the development of an inhibitor against [[Coagulation factor|coagulation factor VIII]] (FVIII). Hemophilia A manifests with early muscle and subcutaneous [[bleeding]] and rarely with haemothorax.


* [[Glanzmann's thrombasthenia|Glanzmann thromboastenia]] is an [[Autosomal recessive|autosomal-recessive]] bleeding disorder characterized by a lifelong bleeding tendency due to abnormalities of the platelet [[Integrin|integrin αΠbβ3]] [[[glycoprotein]] (GP) IIb; CD41/IIIa; CD61]. Glanzmann thromboastenia usually presents with [[Mucocutaneous zone|mucocutaneous]] bleeding such as [[easy bruising]], [[purpura]], [[gingival bleeding]], [[epistaxis]], [[menorrhagia]], [[Hemarthrosis|haemarthrosis]], [[Hematuria|haematuria]], [[Intracranial hemorrhage|intracranial and visceral hemorrhage]] are rare but even rarer is Spontaneous Haemothorax.
* [[Glanzmann's thrombasthenia|Glanzmann thromboastenia]] is an [[Autosomal recessive|autosomal-recessive]] bleeding disorder characterized by a lifelong bleeding tendency due to abnormalities of the platelet [[Integrin|integrin αΠbβ3]] [[[glycoprotein]] (GP) IIb; CD41/IIIa; CD61]. Glanzmann thromboastenia usually presents with [[Mucocutaneous zone|mucocutaneous]] bleeding such as [[easy bruising]], [[purpura]], [[gingival bleeding]], [[epistaxis]], [[menorrhagia]], [[Hemarthrosis|haemarthrosis]], [[Hematuria|haematuria]], [[Intracranial hemorrhage|intracranial and visceral hemorrhage]] are rare but even rarer is Spontaneous Haemothorax.

Revision as of 21:26, 31 July 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Irfan Dotani

Overview

Haemothorax is a pathologic collection of blood within the pleural cavity, between the lung surface and inner chest wall. Three mechanisms of bleeding in haemothorax include torn adhesion between the parietal and visceral pleurae, rupture of neovascularized bullae as a complication of subpleural emphysematous blebs, and torn congenital aberrant vessels branching from the cupola and distributed in and around the bulla in the apex of the lung. There is some genetic disorder that is predisposed to haemothorax.[1][2][3][4][5]

Pathophysiology

The pathogenesis of hemothorax include[1][2][3][4][5]

Pathogenesis

Three mechanisms of bleeding in haemothorax:

  • Torn adhesion between the parietal and visceral pleurae.
  • Rupture of neovascularized bullae as a complication of subpleural emphysematous blebs.
  • Torn congenital aberrant vessels branching from the cupola and distributed in and around the bulla in the apex of the lung.

Genetics

  • Hemophilia A is a X-linked hereditary disorder of blood clotting that caused by the development of an inhibitor against coagulation factor VIII (FVIII). Hemophilia A manifests with early muscle and subcutaneous bleeding and rarely with haemothorax.

References

  1. 1.0 1.1 Álvarez K, Jordi L, Jose Angel H (October 2017). "Hemothorax in vascular Ehlers-Danlos syndrome". Reumatol Clin. doi:10.1016/j.reuma.2017.08.009. PMID 29050841.
  2. 2.0 2.1 Janik M, Straka L, Krajcovic J, Hejna P, Hamzik J, Novomesky F (March 2014). "Non-traumatic and spontaneous hemothorax in the setting of forensic medical examination: a systematic literature survey". Forensic Sci. Int. 236: 22–9. doi:10.1016/j.forsciint.2013.12.013. PMID 24529771.
  3. 3.0 3.1 Boersma WG, Stigt JA, Smit HJ (November 2010). "Treatment of haemothorax". Respir Med. 104 (11): 1583–7. doi:10.1016/j.rmed.2010.08.006. PMID 20817498.
  4. 4.0 4.1 Quero Valenzuela F, Giraldo Ospina CF, Piedra Fernández I (September 2014). "Traumatic hemothorax caused by solitary costal exostosis". Arch. Bronconeumol. 50 (9): 410. doi:10.1016/j.arbres.2013.09.013. PMID 24439464.
  5. 5.0 5.1 Kuo SM, Chen KC, Diau GY, Hua YM (May 2010). "Dangerous costal exostosis: hemothorax mimicking empyema in a child". J. Pediatr. 156 (5): 853, 853.e1. doi:10.1016/j.jpeds.2009.09.053. PMID 20060125.

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