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
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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.
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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 ]

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