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12 gru 2022 · Hereditary hemorrhagic telangiectasias (HHT) is an autosomal dominant bleeding disorder caused by malformed vessels. The condition manifests as telangiectasias affecting the skin and mucosa and arteriovenous malformations involving organs like the lung, brain, and liver.
10 gru 2021 · Given this and the relatively high frequency of HHT as a hereditary bleeding disorder, an understanding of modern HHT management is crucial for the practicing hematologist. Conflict-of-interest disclosure
Disease definition. An inherited disorder of angiogenesis characterized by mucocutaneous telangiectases and visceral arteriovenous malformations. ORPHA:774. Classification level: Disorder. Synonym (s): HHT. Rendu-Osler disease. Rendu-Osler-Weber disease. Prevalence: 1-5 / 10 000. Inheritance: Autosomal dominant. Age of onset: All ages.
ICD-10. I78.0. Choroba Rendu-Oslera-Webera (wrodzona naczyniakowatość krwotoczna, ang. hereditary hemorrhagic telangiectasia, Osler-Weber-Rendu syndrome, ORW disease) – genetycznie uwarunkowana choroba z grupy fakomatoz. Objawia się malformacjami naczyniowymi.
10 gru 2021 · PMCID: PMC8791148. DOI: 10.1182/hematology.2021000281. Abstract. Hereditary hemorrhagic telangiectasia (HHT), the second most common inherited bleeding disorder, is associated with the development of malformed blood vessels.
I78.0 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. The 2025 edition of ICD-10-CM I78.0 became effective on October 1, 2024. This is the American ICD-10-CM version of I78.0 - other international versions of ICD-10 I78.0 may differ.
15 gru 2020 · Description: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an estimated prevalence of 1 in 5000 that is characterized by the presence of vascular malformations (VMs). These result in chronic bleeding, acute hemorrhage, and complications from shunting through VMs.
