Sonographic findings in type I glycogen storage disease.

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.

[Unusual application of computerized tomography: the study of musical instruments]. / Un'insolita applicazione della Tomografia Computerizzata: lo studio degli strumenti musicali.

INTRODUCTION: We report on the use of CT in the study of bowed stringed instruments to assess structural defects and/or damage before proceeding to any repair. MATERIAL AND METHODS: Two antique masterpieces from the Castello Sforzesco Museum of Antique Musical Instruments were analyzed with CT. They were an exquisite wood and ivory guitar from Naples (Italy) and a very rare Giuseppe Guarneri "del Gesù" violin from Cremona (Italy), both crafted in the early years of the 18th century. We evaluated the wood thickness, the neck and its heel. In the wood structure we studied the course and thickness of hypo- and hyperdense lines. RESULTS: The examination showed three types of signs: normal wood structure: hypodense, thin, parallel lines; wormholes: hypodense lines with irregular course and variable thickness; previous repair signs: thin or thick more or less parallel hyperdense lines. CONCLUSIONS: The study confirmed that CT is a valuable tool to investigate normal structure, defects and damage, providing accurate information for the evaluation and repair of antique stringed instruments.

[Digital rotational angiography in the study of vascular diseases: technical note and initial clinical applications]. / L'angiografia digitale con rotazione del tubo radiogeno nello studio delle malattie vascolari: note di tecnica e prime applicazioni cliniche.

INTRODUCTION: Digital rotational angiography is a technique characterized by a C-arm acquiring images as it rapidly rotates around the patient. We studied the clinical potentials of this technique in the assessment of vascular diseases of the abdominal aorta and of the carotid, lower limbs and renal arteries. MATERIAL AND METHODS: We examined 108 patients (66 men and 42 women; mean age: 54.3 years, range: 34-69): 42 had vascular diseases in the carotid arteries, 47 in the abdominal aorta and lower limbs and 19 in the renal arteries. All the patients underwent digital rotational and non-rotational angiography and we analyzed the diagnostic yield, amount of contrast agent and the utility of additional views for each technique. All the examinations were reviewed with(out) subtraction, in cine-loop mode and frame by frame, as well as with(out) magnification. Finally, we considered background noise in both rotational and non-rotational images. RESULTS: The diagnostic quality of the digital rotational technique was always the same as or superior to that of the non-rotational technique. The former allows better 3D rendering, especially when viewed in the cine-loop mode; the examination is shorter and less contrast agent is needed. In contrast, image noise was increased, especially in lateral and oblique views. Digital rotational angiography was fairly well tolerated but the long breath-hold required was a problem especially to elderly patients. CONCLUSIONS: Digital rotational angiography is a useful tool to study vascular diseases in the carotid arteries and lower limbs using a lower radiation dose and less contrast agent than non-rotational examinations. As for the abdominal aorta and renal arteries, the rotational technique can be a valid adjunct to the conventional one.