PURPOSE: The aim of this study was to document the sonographic appearance and dimensions of the liver and spleen in patients affected by type I glycogen storage disease and to correlate those findings with laboratory data to evaluate the potential role of sonography in diagnosing that disease. METHODS: Fourteen patients (age range, 3-26 years; 10 patients younger than 18 years) with type I glycogen storage disease proved by liver biopsy were studied prospectively with gray-scale sonography, color Doppler sonography, and spectral analysis. The liver, kidneys, spleen, portal system, hepatic veins, and hepatic arteries were evaluated. Laboratory data were correlated with sonographic findings. RESULTS: In 13 (93%), of 14 patients, the liver was enlarged, and in 11 patients (79%), hepatic echogenicity was increased. In 9 patients (64%), both kidneys were enlarged, and in 6 cases (43%), the spleen was enlarged. In all patients, flow in the portal, splenic, and superior mesenteric veins was hepatopetal, and flow in the hepatic veins was triphasic. In 5 patients (36%), both triglyceride and total cholesterol levels were higher than normal. No focal hepatic lesions were identified. Analysis found no significant association between sonographic findings and laboratory data. CONCLUSIONS: The most frequent sonographic findings in patients with type I glycogen storage disease were hepatomegaly, increased hepatic echogenicity, and enlarged kidneys. Sonography may help in the diagnosis of type I glycogen storage disease, but a liver biopsy is required for a definitive diagnosis.
Sonographic findings in type I glycogen storage disease / C. Pozzato, A. Botta, C. Melgara, L. Fiori, M.L. Giannì, E. Riva. - In: JOURNAL OF CLINICAL ULTRASOUND. - ISSN 0091-2751. - 29:8(2001 Oct), pp. 456-461.
|Titolo:||Sonographic findings in type I glycogen storage disease|
POZZATO, CARLO PAOLO (Primo)
GIANNI', MARIA LORELLA (Penultimo)
RIVA, ENRICA LICIA (Ultimo)
|Settore Scientifico Disciplinare:||Settore MED/36 - Diagnostica per Immagini e Radioterapia|
Settore MED/38 - Pediatria Generale e Specialistica
|Data di pubblicazione:||ott-2001|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1002/jcu.10008|
|Appare nelle tipologie:||01 - Articolo su periodico|