About
Contact
Help
Sending publications
How to publish
Advanced Search
View Item 
  •   Home
  • Facultad de Medicina
  • Artículos de revistas
  • View Item
  •   Home
  • Facultad de Medicina
  • Artículos de revistas
  • View Item
JavaScript is disabled for your browser. Some features of this site may not work without it.

Browse byCommunities and CollectionsDateAuthorsTitlesSubjectsThis CollectionDateAuthorsTitlesSubjects

My Account

Login to my accountRegister
Biblioteca Digital - Universidad de Chile
Revistas Chilenas
Repositorios Latinoamericanos
Tesis LatinoAmericanas
Tesis chilenas
Related linksRegistry of Open Access RepositoriesOpenDOARGoogle scholarCOREBASE
My Account
Login to my accountRegister

The hepatic porphyrias: experience with 105 cases

Artículo
Thumbnail
Open/Download
Iconitem_0026822384.pdf (2.020Kb)
Access note
Acceso a solo metadatos
Publication date
1992
Metadata
Show full item record
Cómo citar
Armas-Merino, Rodolfo
Cómo citar
The hepatic porphyrias: experience with 105 cases
.
Copiar
Cerrar

Author
  • Armas-Merino, Rodolfo;
  • Wolff Fernández, Carlos;
  • Krause, P.;
  • Chaná, P.;
  • Parraguez, A.;
  • Soto, J.;
Abstract
Hepatic porphyria is a rare metabolic syndrome caused by abnormal enzyme activity in heme biosynthesis. Between 1974 and 1991; 105 patients have met criteria for diagnosis of hepatic porphyria based on typical clinical findings and/or laboratory abnormalities. According to type, 42% had porphyria cutanea tarda, 21% porphyria variegate, 15% protoporphyria, 6.7% acute intermittent porphyria, 6.7% coproporphyria and 1.9% porphyria due to porphobilinogen deficit. A proper classification was not established in 6.7% of patients. Porphyria cutanea tarda was more common in males (70%) and porphyria variegata, in females (90%). A family history of the disease was present in 33% of patients; 20% of patients were of European descent and 4% of Mapuche descent. Diagnosis was usually established in the third decade, somewhat later in porphyria cutanea tarda (45 years of age) and very early in protoporphyria. 10% of patients were asymptomatic and 29 patients developed at least one porphyric crisis. These were related to pregnancy in 6 patients, to hormone administration in 7, to antibiotics in 5. No cause was established in 21 cases. Severe crisis were successfully treated with Hematin. Venipuncture was used to treat 50% of patients with porphyria cutanea tarda with 95% success. Thus, hepatic porphyria is recognized with increasing frequency and can be treated successfully in most cases.
Indexation
Artículo de publicación SCOPUS
Identifier
URI: https://repositorio.uchile.cl/handle/2250/159687
ISSN: 00349887
Quote Item
Revista medica de Chile, Volumen 120, Issue 3, 1992, Pages 259-266
Collections
  • Artículos de revistas
xmlui.footer.title
31 participating institutions
More than 73,000 publications
More than 110,000 topics
More than 75,000 authors
Published in the repository
  • How to publish
  • Definitions
  • Copyright
  • Frequent questions
Documents
  • Dating Guide
  • Thesis authorization
  • Document authorization
  • How to prepare a thesis (PDF)
Services
  • Digital library
  • Chilean academic journals portal
  • Latin American Repository Network
  • Latin American theses
  • Chilean theses
Dirección de Servicios de Información y Bibliotecas (SISIB)
Universidad de Chile

© 2020 DSpace
  • Access my account