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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

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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.
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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...
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Dawson A, Elias DJ, Rubenson D, et al. Pulmonary Hypertension Developing after Alglucerase Therapy in Two Patients with Type 1 Gaucher Disease Complicated by the Hepatopulmonary Syndrome. Ann Intern Med. 1996;125:901–904. doi: 10.7326/0003-4819-125-11-199612010-00005

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

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

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