Doppler ultrasound study of portal hemodynamics in patients with Gaucher disease
Gaucher disease is a lysosomal storage disorder caused by a deficiency of the enzyme glucocerebrosidase and characterized by the presence of pathological macrophages laden with glucosylceramide. Hepatosplenomegaly is a common manifestation of Gaucher disease, but symptomatic portal hyperten...
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| Format: | Article |
| Language: | English |
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University of Belgrade, University of Novi Sad
2015-01-01
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| Series: | Archives of Biological Sciences |
| Subjects: | |
| Online Access: | http://www.doiserbia.nb.rs/img/doi/0354-4664/2015/0354-46641500001S.pdf |
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| author | Šarenac-Kovač Radmila Perišić Mirjana Tomić Dragan Dimitrijević Slađana Jurišić Vladimir |
| author_facet | Šarenac-Kovač Radmila Perišić Mirjana Tomić Dragan Dimitrijević Slađana Jurišić Vladimir |
| author_sort | Šarenac-Kovač Radmila |
| collection | DOAJ |
| description | Gaucher disease is a lysosomal storage disorder caused by a deficiency of the
enzyme glucocerebrosidase and characterized by the presence of pathological
macrophages laden with glucosylceramide. Hepatosplenomegaly is a common
manifestation of Gaucher disease, but symptomatic portal hypertension is
rarely seen. The study included 20 untreated adult patients with Gaucher
disease (non-neuronopathic type 1) diagnosed with the presence of Gaucher
cells in the bone marrow, and 20 healthy subjects as controls. The
examination of patients included color Doppler ultrasonography (pulsed
Doppler mode), resistive index (RI) and Doppler perfusion index (DPI) using a
Toshiba Xario ultrasound machine and a convex array probe PVT-375AX (1.9-6
MHz) with the objective of analyzing portal hemodynamics. Results showed that
all patients had enlarged liver and spleen, and their average sizes were
significantly larger than those in the healthy controls (liver: 17.04
vs.14.02 cm; spleen: 22.2 vs. 10.74 cm). DPI values were significantly
different between patients and controls (0.15 vs. 0.21). Considering DPI
<0.15 indicates arterial liver hypoperfusion and hypoxia, it can be concluded
that a number of patients had a problem with liver oxygenation, which may be
linked to the high angiotensin-converting enzyme (ACE) levels obtained in the
patients (339.42 U/L), 10 times greater than in control subjects. Since ACE
is a potent vasoconstrictor produced by spleen macrophages in Gaucher
disease, we can suppose that elevated ACE is associated with effects on the
blood vessels of the liver and spleen. [Projekat Ministarstva nauke Republike
Srbije, br. 175056] |
| first_indexed | 2024-12-19T07:27:53Z |
| format | Article |
| id | doaj.art-58d3d9ec3e754a49adee0de1dd27863a |
| institution | Directory Open Access Journal |
| issn | 0354-4664 1821-4339 |
| language | English |
| last_indexed | 2024-12-19T07:27:53Z |
| publishDate | 2015-01-01 |
| publisher | University of Belgrade, University of Novi Sad |
| record_format | Article |
| series | Archives of Biological Sciences |
| spelling | doaj.art-58d3d9ec3e754a49adee0de1dd27863a2022-12-21T20:30:47ZengUniversity of Belgrade, University of Novi SadArchives of Biological Sciences0354-46641821-43392015-01-01671111810.2298/ABS140505001S0354-46641500001SDoppler ultrasound study of portal hemodynamics in patients with Gaucher diseaseŠarenac-Kovač Radmila0Perišić Mirjana1Tomić Dragan2Dimitrijević Slađana3Jurišić Vladimir4Clinical Center of Serbia, Clinic of Gastroenterology and Hepatology, BelgradeClinical Center of Serbia, Clinic of Gastroenterology and Hepatology, BelgradeFaculty of Medicine, BelgradeFaculty of Science, KragujevacFaculty of Medical Science, KragujevacGaucher disease is a lysosomal storage disorder caused by a deficiency of the enzyme glucocerebrosidase and characterized by the presence of pathological macrophages laden with glucosylceramide. Hepatosplenomegaly is a common manifestation of Gaucher disease, but symptomatic portal hypertension is rarely seen. The study included 20 untreated adult patients with Gaucher disease (non-neuronopathic type 1) diagnosed with the presence of Gaucher cells in the bone marrow, and 20 healthy subjects as controls. The examination of patients included color Doppler ultrasonography (pulsed Doppler mode), resistive index (RI) and Doppler perfusion index (DPI) using a Toshiba Xario ultrasound machine and a convex array probe PVT-375AX (1.9-6 MHz) with the objective of analyzing portal hemodynamics. Results showed that all patients had enlarged liver and spleen, and their average sizes were significantly larger than those in the healthy controls (liver: 17.04 vs.14.02 cm; spleen: 22.2 vs. 10.74 cm). DPI values were significantly different between patients and controls (0.15 vs. 0.21). Considering DPI <0.15 indicates arterial liver hypoperfusion and hypoxia, it can be concluded that a number of patients had a problem with liver oxygenation, which may be linked to the high angiotensin-converting enzyme (ACE) levels obtained in the patients (339.42 U/L), 10 times greater than in control subjects. Since ACE is a potent vasoconstrictor produced by spleen macrophages in Gaucher disease, we can suppose that elevated ACE is associated with effects on the blood vessels of the liver and spleen. [Projekat Ministarstva nauke Republike Srbije, br. 175056]http://www.doiserbia.nb.rs/img/doi/0354-4664/2015/0354-46641500001S.pdfGaucher diseaseDoppler ultrasonographyportal flow velocity |
| spellingShingle | Šarenac-Kovač Radmila Perišić Mirjana Tomić Dragan Dimitrijević Slađana Jurišić Vladimir Doppler ultrasound study of portal hemodynamics in patients with Gaucher disease Archives of Biological Sciences Gaucher disease Doppler ultrasonography portal flow velocity |
| title | Doppler ultrasound study of portal hemodynamics in patients with Gaucher disease |
| title_full | Doppler ultrasound study of portal hemodynamics in patients with Gaucher disease |
| title_fullStr | Doppler ultrasound study of portal hemodynamics in patients with Gaucher disease |
| title_full_unstemmed | Doppler ultrasound study of portal hemodynamics in patients with Gaucher disease |
| title_short | Doppler ultrasound study of portal hemodynamics in patients with Gaucher disease |
| title_sort | doppler ultrasound study of portal hemodynamics in patients with gaucher disease |
| topic | Gaucher disease Doppler ultrasonography portal flow velocity |
| url | http://www.doiserbia.nb.rs/img/doi/0354-4664/2015/0354-46641500001S.pdf |
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