Document Detail

Echocardiographic assessment of pulmonary hypertension in Gaucher's disease.
MedLine Citation:
PMID:  10326537     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Enzyme therapy has been shown to decrease the signs and symptoms of Gaucher's disease. A few patients, however, develop pulmonary hypertension on such treatment. We investigated the frequency of pulmonary hypertension in Gaucher's disease. METHODS: We studied 134 adults with type 1 Gaucher's disease, including 73 patients on enzyme replacement, with echocardiography. We measured tricuspid incompetence (TI) with continuous-wave doppler. Pulmonary hypertension was indicated by a TI gradient of more than 30 mm Hg. FINDINGS: Nine (7%) patients had pulmonary hypertension: all were treated and six had undergone splenectomy. Chest radiographs confirmed the presence of pulmonary hypertension in these patients as well as in most patients with TI gradients of 25-29 mm Hg. INTERPRETATION: The confounding effects of disease severity and splenectomy in many treated patients precluded definitive conclusion of cause and effect. Nonetheless, we found an unexpectedly high rate of pulmonary hypertension and recommended routine echocardiographic monitoring of all treated and untreated patients with type 1 Gaucher's disease. We also suggest consideration of treatment withdrawal if the TI gradient progresses to more than 30 mm Hg.
D Elstein; M W Klutstein; A Lahad; A Abrahamov; I Hadas-Halpern; A Zimran
Related Documents :
18423497 - Use of microcirculatory parameters to evaluate clinical treatments of chronic venous di...
10731457 - Prevalence, treatment, control, and awareness of high blood pressure and the risk of st...
18223477 - Pharmacokinetics and pharmacodynamics of sildenafil in a patient treated with human imm...
18631327 - Macular microcirculation in hypertensive patients with and without branch retinal vein ...
8497627 - Utility of axillary views in postmastectomy patients.
19223087 - Association between atherosclerosis and newly classified chronic kidney disease stage f...
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Lancet     Volume:  351     ISSN:  0140-6736     ISO Abbreviation:  Lancet     Publication Date:  1998 May 
Date Detail:
Created Date:  1999-05-27     Completed Date:  1999-05-27     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  2985213R     Medline TA:  Lancet     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  1544-6     Citation Subset:  AIM; IM    
Gaucher Clinic, Jerusalem, Israel.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Confounding Factors (Epidemiology)
Echocardiography, Doppler*
Follow-Up Studies
Gaucher Disease / complications,  drug therapy,  surgery,  ultrasonography*
Glucosylceramidase / adverse effects,  therapeutic use
Hypertension, Pulmonary / etiology,  radiography,  ultrasonography*
Recombinant Proteins
Treatment Outcome
Tricuspid Valve Insufficiency / ultrasonography
Reg. No./Substance:
0/Recombinant Proteins; EC 3.2.1.-/alglucerase; EC 3.2.1.-/imiglucerase; EC
Comment In:
Lancet. 1998 Aug 15;352(9127):580   [PMID:  9716094 ]
Lancet. 1998 Jul 18;352(9123):240   [PMID:  9683246 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Previous Document:  Randomised vaccine trial of single dose of killed Leishmania major plus BCG against anthroponotic cu...
Next Document:  Cerebrospinal-fluid HIV-1 RNA and drug concentrations after treatment with lamivudine plus zidovudin...