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Familial giant hemangiomas of the liver. Study of a family and review of the literature


Moser, C; Hany, A; Spiegel, Roland (1998). Familial giant hemangiomas of the liver. Study of a family and review of the literature. Praxis, 87(14):461-468.

Abstract

In this report we present for the first time strong evidence for autosomal-dominant inheritance of grant liver hemangiomas. Furthermore, we review the current literature about incidence, pathogenesis, clinics, as well as diagnostic and therapeutic aspects of LH. A large family of Italian origin is described where three female patients in three successive generations suffered from large symptomatic LH. In addition, two other female relatives exhibited asymptomatic LH on sonographic scans. The restriction of the disease to the female gender could be explained by a sex-dependent difference in penetrance or expressivity of a presumable "liver-hemangioma" gene, or by known proliferative factors such as female sex hormones. We also observed an increased incidence of adenomas of the thyroidea among members with or without LH of the presented family. This uncommon familial association has also not yet been described and its genetic aspects are discussed.

Abstract

In this report we present for the first time strong evidence for autosomal-dominant inheritance of grant liver hemangiomas. Furthermore, we review the current literature about incidence, pathogenesis, clinics, as well as diagnostic and therapeutic aspects of LH. A large family of Italian origin is described where three female patients in three successive generations suffered from large symptomatic LH. In addition, two other female relatives exhibited asymptomatic LH on sonographic scans. The restriction of the disease to the female gender could be explained by a sex-dependent difference in penetrance or expressivity of a presumable "liver-hemangioma" gene, or by known proliferative factors such as female sex hormones. We also observed an increased incidence of adenomas of the thyroidea among members with or without LH of the presented family. This uncommon familial association has also not yet been described and its genetic aspects are discussed.

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Additional indexing

Other titles:Familiäre Riesenhämangiome der Leber
Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > Institute of Medical Genetics
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Scopus Subject Areas:Health Sciences > General Medicine
Uncontrolled Keywords:Medicine
Language:German
Date:1 April 1998
Deposited On:22 Jun 2023 13:23
Last Modified:29 Mar 2024 04:43
Publisher:Hogrefe Verlag
ISSN:1661-8157
OA Status:Closed
PubMed ID:9587222
Other Identification Number:Corpus ID: 13169995
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