Clinical and molecular study of 4 cases of pulmonary hypertension associated with sarcoidosis

Copyright © 2014 SEPAR. Published by Elsevier Espana. All rights reserved..

Sarcoidosis is a pleomorphic disease that can present with pulmonary hypertension (PH). What little information is available about the association of these two diseases comes mainly from small series of patients scheduled for transplant. We present 4 cases of mild pulmonary involvement in whom right catheterisation was performed and PH-specific therapy was administered. After obtaining written consent, a genetic study was performed that showed mutations in PH-related genes in 3 of the patients. This is the first study of its kind to yield genetic information for this type of PH.

Medienart:

E-Artikel

Erscheinungsjahr:

2015

Erschienen:

2015

Enthalten in:

Zur Gesamtaufnahme - volume:51

Enthalten in:

Archivos de bronconeumologia - 51(2015), 4 vom: 20. Apr., Seite e19-21

Sprache:

Englisch

Beteiligte Personen:

Baloira Villar, Adolfo [VerfasserIn]
Pousada Fernández, Guillermo [VerfasserIn]
Núñez Fernández, Marta [VerfasserIn]
Valverde Pérez, Diana [VerfasserIn]

Links:

Volltext

Themen:

742SXX0ICT
Ambrisentan
BMPR2 protein, human
BW9B0ZE037
Bone Morphogenetic Protein Receptors, Type II
Bosentan
Case Reports
DCR9Z582X0
EC 2.7.11.30
Epoprostenol
HW6NV07QEC
Hipertensión pulmonar
Journal Article
KCNA5 protein, human
Kv1.5 Potassium Channel
Mutaciones
Mutations
Phenylpropionates
Pulmonary hypertension
Pyridazines
Q326023R30
RNA, Messenger
RUM6K67ESG
Research Support, Non-U.S. Gov't
Sarcoidosis
Sildenafil Citrate
Sulfonamides
Tadalafil
Treprostinil

Anmerkungen:

Date Completed 21.12.2015

Date Revised 02.12.2018

published: Print-Electronic

Citation Status MEDLINE

doi:

10.1016/j.arbres.2014.03.022

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM23938606X