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Hereditary Telangiectasia and Pulmonary Fistula: Case of Large Right-to-Left Shunt Surgically Corrected

Hereditary Telangiectasia and Pulmonary Fistula: Case of Large Right-to-Left Shunt Surgically... Abstract Hereditary LEREDITARY hemorrhagic telangiectasia manifests itself by the formation of localized arteriovenous capillary connections that are tiny arteriovenous fistulas. The first comprehensive description was given by Rendu1 in 1896. Osler2 in 1901 and Weber3 in 1907 added to knowledge of the entity, which brought about the designation "Rendu-Osler-Weber syndrome." In 1909, Hanes4 used the term "hereditary hemorrhagic telangiectasia," which is the preferred designation. Hodgson and colleagues5 studied 231 members of a large family of which 91 descendants had the disease entity. Of the descendants studied, 40% had telangiectasia and 6% had pulmonary arteriovenous fistulas. In 50% of the cases of pulmonary arteriovenous fistula, telangiectasia elsewhere in the body is encountered.6 Approximately 5% to 6% of patients with Rendu-Osler-Weber syndrome have pulmonary arteriovenous fistula.7 Pulmonary arteriovenous fistulas may occur in persons with or without hereditary hemorrhagic telangiectasia, but the close relationship makes it imperative that patients with this disorder be investigated References 1. Rendu: Epistaxis répétées chez un sujet porteur de petits angiomes cutanés et muqueux , Bull Mém Soc Med Hôp 13:731-733 ( (Oct 23) ) 1896. 2. Osler, W.: On a Family Form of Recurring Epistaxis, Associated With Multiple Telangiectases of the Skin and Mucous Membranes , Bull Hopkins Hosp 12:333-337 ( (Nov) ) 1901. 3. Weber, F.P.: Multiple Hereditary Developmental Angiomata (Telangiectases) of the Skin and Mucous Membranes Associated With Recurring Haemorrhages , Lancet 2:160-162 ( (July 20) ) 1907. 4. Hanes, F.M.: Multiple Hereditary Telangiectases Causing Hemorrhage (Hereditary Hemorrhagic Telangiectasia) , Bull Hopkins Hosp 20:63-73 ( (March) ) 1909. 5. Hodgson, C.H., et al: Hereditary Hemorrhagic Telangiectasia and Pulmonary Arteriovenous Fistula: Survey of a Large Family , New Eng J Med 261: 625-636 ( (Sept 24) ) 1959.Crossref 6. Goldman, A.: Arteriovenous Fistula of the Lung: Its Hereditary and Clinical Aspects , Amer Rev Tuberc 57:266-280 ( (March) ) 1948. 7. Bergqvist, N.; Hessén, I.; and Hey, M.: Arteriovenous Pulmonary Aneurysms in Osler's Disease, (Telangiectasia Hereditaria Haemorrhagica): Report of Four Cases in the Same Family , Acta Med Scand 171:301-309, 1962.Crossref 8. McKusick, V.A.: A Genetical View of Cardiovascular Disease , Circulation 30:326-357 ( (Sept) ) 1964.Crossref 9. Hodgson, C.H., and Kaye, R.L.: Pulmonary Arteriovenous Fistula and Hereditary Hemorrhagic Telangiectasia: A Review and Report of 35 Cases of Fistula , Dis Chest 43:449-455 ( (May) ) 1963.Crossref 10. Callahan, J.A.; Helmholz, H.F., Jr.; and Kirklin, J.W.: Pulmonary Arteriovenous Fistula Located by Indicator Dilution Studies , Amer Heart J 52:916-921 ( (Dec) ) 1956.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

Hereditary Telangiectasia and Pulmonary Fistula: Case of Large Right-to-Left Shunt Surgically Corrected

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References (11)

Publisher
American Medical Association
Copyright
Copyright © 1967 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1967.00290200119011
Publisher site
See Article on Publisher Site

Abstract

Abstract Hereditary LEREDITARY hemorrhagic telangiectasia manifests itself by the formation of localized arteriovenous capillary connections that are tiny arteriovenous fistulas. The first comprehensive description was given by Rendu1 in 1896. Osler2 in 1901 and Weber3 in 1907 added to knowledge of the entity, which brought about the designation "Rendu-Osler-Weber syndrome." In 1909, Hanes4 used the term "hereditary hemorrhagic telangiectasia," which is the preferred designation. Hodgson and colleagues5 studied 231 members of a large family of which 91 descendants had the disease entity. Of the descendants studied, 40% had telangiectasia and 6% had pulmonary arteriovenous fistulas. In 50% of the cases of pulmonary arteriovenous fistula, telangiectasia elsewhere in the body is encountered.6 Approximately 5% to 6% of patients with Rendu-Osler-Weber syndrome have pulmonary arteriovenous fistula.7 Pulmonary arteriovenous fistulas may occur in persons with or without hereditary hemorrhagic telangiectasia, but the close relationship makes it imperative that patients with this disorder be investigated References 1. Rendu: Epistaxis répétées chez un sujet porteur de petits angiomes cutanés et muqueux , Bull Mém Soc Med Hôp 13:731-733 ( (Oct 23) ) 1896. 2. Osler, W.: On a Family Form of Recurring Epistaxis, Associated With Multiple Telangiectases of the Skin and Mucous Membranes , Bull Hopkins Hosp 12:333-337 ( (Nov) ) 1901. 3. Weber, F.P.: Multiple Hereditary Developmental Angiomata (Telangiectases) of the Skin and Mucous Membranes Associated With Recurring Haemorrhages , Lancet 2:160-162 ( (July 20) ) 1907. 4. Hanes, F.M.: Multiple Hereditary Telangiectases Causing Hemorrhage (Hereditary Hemorrhagic Telangiectasia) , Bull Hopkins Hosp 20:63-73 ( (March) ) 1909. 5. Hodgson, C.H., et al: Hereditary Hemorrhagic Telangiectasia and Pulmonary Arteriovenous Fistula: Survey of a Large Family , New Eng J Med 261: 625-636 ( (Sept 24) ) 1959.Crossref 6. Goldman, A.: Arteriovenous Fistula of the Lung: Its Hereditary and Clinical Aspects , Amer Rev Tuberc 57:266-280 ( (March) ) 1948. 7. Bergqvist, N.; Hessén, I.; and Hey, M.: Arteriovenous Pulmonary Aneurysms in Osler's Disease, (Telangiectasia Hereditaria Haemorrhagica): Report of Four Cases in the Same Family , Acta Med Scand 171:301-309, 1962.Crossref 8. McKusick, V.A.: A Genetical View of Cardiovascular Disease , Circulation 30:326-357 ( (Sept) ) 1964.Crossref 9. Hodgson, C.H., and Kaye, R.L.: Pulmonary Arteriovenous Fistula and Hereditary Hemorrhagic Telangiectasia: A Review and Report of 35 Cases of Fistula , Dis Chest 43:449-455 ( (May) ) 1963.Crossref 10. Callahan, J.A.; Helmholz, H.F., Jr.; and Kirklin, J.W.: Pulmonary Arteriovenous Fistula Located by Indicator Dilution Studies , Amer Heart J 52:916-921 ( (Dec) ) 1956.Crossref

Journal

Archives of Internal MedicineAmerican Medical Association

Published: Feb 1, 1967

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