Skip Navigation
Menu
Original Research |1 December 1996

Pulmonary Hypertension Developing after Alglucerase Therapy in Two Patients with Type 1 Gaucher Disease Complicated by the Hepatopulmonary Syndrome

Arthur Dawson, MD; Darlene J. Elias, MD; David Rubenson, MD; Stephen H. Bartz, MD, PhD; Paul R. Garver, MD; Andrea C. Kay, MD; Colin M. Bloor, MD; Ernest Beutler, MD

Article, Author, and Disclosure Information
Author, Article, and Disclosure Information
Copyright ©2004 by the American College of Physicians
  • From Scripps Clinic and Research Foundation and the Scripps Research Institute, La Jolla, California; and the University of California at San Diego Medical Center, San Diego, California. Grant Support: By the General Clinical Research Center Grant M01-RR00833 and Department of Academic Affairs, Scripps Clinic and Research Foundation. Requests for Reprints: Arthur Dawson, MD, Division of Chest and Critical Care Medicine, Scripps Clinic and Research Foundation, 10666 North Torrey Pines Road, La Jolla, CA 92037. Current Author Addresses: Drs. Dawson and Elias: Division of Chest and Critical Care Medicine, Scripps Clinic and Research Foundation, 10666 North Torrey Pines Road, La Jolla, CA 92037. Drs. Rubenson and Bartz: Division of Cardiovascular Diseases, Scripps Clinic and Research Foundation, 10666 North Torrey Pines Road, La Jolla, CA 92037.
×
FULL TEXT
Gaucher disease is an autosomal-recessive disorder that is caused by a deficiency of the enzyme glucocerebrosidase and results in accumulation of an insoluble glucocerebroside in macrophages. Type 1 (chronic non-neuropathic or “adult”) Gaucher disease is usually characterized by hepatosplenomegaly, thrombocytopenia, and skeletal complications (including bone pain, osteonecrosis, and pathologic fractures) [1, 2]. With the exception of splenectomy, few options were available for treating this disorder until enzyme replacement therapy with alglucerase became available [3]. Pulmonary involvement has long been recognized as a rare complication of Gaucher disease. Pulmonary hypertension, severe hypoxemia, and infiltration of the lungs with Gaucher cells have been described in case reports and small case series.
FULL TEXT
PDF
Not Available
Citations
Citation

Dawson A, Elias DJ, Rubenson D, Bartz SH, Garver PR, Kay AC, et al. Pulmonary Hypertension Developing after Alglucerase Therapy in Two Patients with Type 1 Gaucher Disease Complicated by the Hepatopulmonary Syndrome. Ann Intern Med. ;125:901–904. doi: 10.7326/0003-4819-125-11-199612010-00005

Download citation file:

© 2019

×
Permissions

Published: Ann Intern Med. 1996;125(11):901-904.

DOI: 10.7326/0003-4819-125-11-199612010-00005

34 Citations

Related Articles
SSRI use in late pregnancy was associated with increased persistent pulmonary hypertension in newborns
Annals of Internal Medicine; 156 (10): JC5-11
A Combination Treatment for Pulmonary Hypertension
Annals of Internal Medicine; 149 (8): I-38
Narrative Review: The Enigma of Pulmonary Arterial Hypertension: New Insights from Genetic Studies
Annals of Internal Medicine; 148 (4): 278-283
Evaluation and Management of the Patient with Pulmonary Arterial Hypertension
Annals of Internal Medicine; 143 (4): 282-292
View MoreView Less

Journal Club
SSRI use in late pregnancy was associated with increased persistent pulmonary hypertension in newborns
Annals of Internal Medicine; 156 (10): JC5-11
View MoreView Less

Related Point of Care
Pulmonary Hypertension
Annals of Internal Medicine; 158 (9): ITC5-1
View MoreView Less

Related Topics
Encephalopathy
Gastroenterology/Hepatology
Liver Disease
Neurology
Pulmonary Hypertension

Encephalopathy, Gastroenterology/Hepatology, Liver Disease, Neurology, Pulmonary Hypertension.

PubMed Articles
Birth Size and Gestational Age Specific Outcomes of Inhaled Nitric Oxide Therapy in Preterm Neonates with Clinically Diagnosed Pulmonary Hypertension.
Am J Perinatol 2019.
Therapeutic effects of the selective farnesoid X receptor agonist obeticholic acid in a monocrotaline-induced pulmonary hypertension rat model.
J Endocrinol Invest 2019.
View More

Results provided by: PubMed

CONTINUE WITH LIMITED ACCESS
Annals of Internal Medicine
To receive access to the full text of freely available articles, alerts, and more. You will be directed to acponline.org to complete your registration.
×
acp link acp
silverchair link silverchair

Copyright © 2019 American College of Physicians. All Rights Reserved.

Print ISSN: 0003-4819 | Online ISSN: 1539-3704

Privacy Policy

|

Conditions of Use

This site uses cookies. By continuing to use our website, you are agreeing to our privacy policy. | Accept
×

You need a subscription to this content to use this feature.

×
PDF Downloads Require Access to the Full Article.
Annals of Internal Medicine
To receive access to the full text of freely available articles, alerts, and more. You will be directed to acponline.org to complete your registration.
×
Access to this Free Content Requires Users to be Registered and Logged In. Please Choose One of the Following Options
Annals of Internal Medicine
To receive access to the full text of freely available articles, alerts, and more. You will be directed to acponline.org to complete your registration.
×