Hepatopulmonary Syndrome in Gaucher Disease With Right-to-Left Shunt: Evaluation and Measurement Using Tc-99m MAA

• Published in: Clinical nuclear medicine Vol. 24; no. 3; pp. 164 - 166
• Main Authors: KIM, JEONG-HEE, PARK, CHAN H, PAI, MOON-SUN, HAHN, MYUNG-HO, KIM, HYON-JU
• Format: Journal Article
• Language: English
• Published: Philadelphia, PA Lippincott Williams & Wilkins, Inc 01-03-1999; Hagerstown, MD Lippincott
• Subjects: Biological and medical sciences; Child; Errors of metabolism; Gaucher Disease - complications; Hepatopulmonary Syndrome - diagnostic imaging; Hepatopulmonary Syndrome - etiology; Humans; Lipids (lysosomal enzyme disorders, storage diseases); Lung - diagnostic imaging; Male; Medical sciences; Metabolic diseases; Radionuclide Imaging; Radiopharmaceuticals; Technetium Tc 99m Aggregated Albumin; Right to left shunt; Hepatic disease; Technetium; Enzymopathy; Association; Hypoxemia; Hepatomegaly; Cyanosis; Diagnosis; Lipoidosis; Child; Quantitative analysis; Human; Nervous system diseases; Oxygen; Radiolabelling; Metabolic diseases; Intrapulmonary administration; Cerebral disorder; Genetic disease; Case study; Gaucher disease; Central nervous system disease; Distribution; Digestive diseases
• ISSN: 0363-9762; 1536-0229
• DOI: 10.1097/00003072-199903000-00005

Hepatomegaly is a common manifestation in Gaucher disease. In some patients with the disease, hepatic fibrosis and portal hypertension are observed. A patient with Gaucher disease with the hepatopulmonary syndrome associated with severe cyanosis and hypoxemia was examined for intrapulmonary right-to-left shunt using Tc-99m MAA. Quantitative evaluation revealed an approximately 50% right to left shunt as indicated by Tc-99m MAA activity in the lungs and systemic organs.