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S Bruni

Publications and source records attributed to S Bruni.

24 records · Page 2Linked to original sources

Hepatocytes immobilised by microencapsulation in artificial cells: effects on hyperbilirubinemia in Gunn rats.

The possibility of using hepatocytes encapsulated in a calcium-alginate-polylysine matrix to lower bilirubin levels in hyperbilirubinemia was investigated. The animal model was the Gunn rat. The microencapsulated hepatocytes were injected intraperitoneally. 15 X 10(6) microencapsulated hepatocytes from Wistar rats, lowered the bilirubin from 14 mg/100 ml to 6 mg/100 ml after 20 days. The bilirubin is still depressed after 90 days. After encapsulation, Sprague-Dawley hepatocytes were as effective as free hepatocytes in lowering bilirubin levels in Gunn rats. After 68 days, the free Sprague-Dawley hepatocytes were not rejected.

Animals↗

The polymerase chain reaction (PCR) in the routine genetic characterization of retinoblastoma: a tool for the clinical laboratory.

The Polymerase Chain Reaction (PCR) is a highly innovative technique which allows for the generation of large amounts of DNA starting from minute quantities obtained from the blood or tissue of a patient. With the increasing knowledge concerning the structure of the human genome and the potential to amplify specific segments of DNA by the PCR technique, the molecular genetic characterization of many ocular disorders has been greatly facilitated. This is particularly true of retinoblastoma (RB) where the causative gene, RB1, gene has been identified and characterized. Using PCR technique, specific sequences of the RB1 gene can be amplified and analyzed to precisely define the genetic mutation in an affected individual. In addition, this technique can also be applied in order to characterize the genetic defect within the tumor itself. In this report we illustrate the use of the PCR technique in the genetic characterization of the RB1 gene and its application to the study of RB. These techniques are applicable even in a small clinical laboratory and can be extended to a number of ophthalmic disorders.

Chromosome Mapping↗

Three-dimensional rotational angiography of the carotid arteries with high-flow injection from the aortic arch. Preliminary experience.

PURPOSE: Three-Dimensional Rotational Angiography (3DRA) is a new technique based on a rotational angiographic acquisition able to display arterial vessels in a 3D rendering mode. The system was mainly developed for neuroradiological evaluations but preliminary extracranial experiences have also been reported. The aim of our work was to compare the results of three-dimensional angiography of the carotid arteries done with high-flow injection of contrast medium from the aortic arch with the results of selective angiography. MATERIALS AND METHODS: Twenty patients underwent digital angiography of the supra-aortic vessels in order to quantify a stenosis of the carotid bifurcations previously detected at Doppler Ultrasound. Examinations were performed with the Philips Integris Allura system provided with the rotational angiography (RA) tool connected to a workstation for three-dimensional reconstruction able to display vessels in a 3D fashion (Volume Rendering, Gradient Rendering, Shaded Surface Display), automatically remove bone structures (cervical spine, calcified plaque, etc.) and perform an automatic analysis of the vessel diameter and surface area at the point of major stenosis and in the disease-free vessel segments above and below. The carotid evaluation was done either with selective catheterization and the two standard AP and LL projections and with RA after contrast medium injection from the aortic arch followed by 3D reconstruction. RESULTS: The comparison of the selective angiography and three-dimensional images was possible in 37 out of 40 carotid bifurcations (3 internal carotid arteries were occluded) and a good diagnostic quality was obtained in 35 out of 37 cases with an high correlation in the degree of stenosis. In 2/37 cases with calcified plaques the degree of stenosis was effectively demonstrated only after electronic subtraction of the calcified component of the plaque. CONCLUSIONS: The technique we propose proved to be feasible in all cases with a good correlation in the quantification of the degree of stenosis. The practical advantage of our technique is the almost complete exclusion of risks of neurological complications due to selective catheterization, and reduced examination time.

Aged↗

Attenuated type I mucopolysaccharidosis in the differential diagnosis of juvenile idiopathic arthritis: a series of 13 patients with Scheie syndrome.

OBJECTIVE: Mucopolysaccharidosis type I (MPS I) is a genetic lysosomal storage disorder caused by deficient activity of the enzyme alpha-L-iduronidase. Incomplete breakdown of glycosaminoglycans leads to progressive accumulation of these substances in many tissues throughout the body. Patients with the less severe form of MPS I (Scheie syndrome) usually present in the first decade of life with frequent articular involvement, and may survive into adulthood. Especially in these attenuated phenotypes, a definitive diagnosis may be delayed for years because clusters of early symptoms are difficult to recognize for physicians not familiar with the disease, and since the disease progresses slowly over decades. We would like to increase the awareness of this type of MPS I disease among rheumatologists and unravel diagnostic pitfalls. METHODS: We have reviewed medical histories of 13 patients (6 males and 7 females) with Scheie syndrome seen in 5 European centers. RESULTS: All patients had prominent musculoskeletal involvement at the onset of their disease in childhood. Diagnosis was delayed in almost all cases (range 4-54 years). CONCLUSION: We suggest that patients who present with progressive non-inflammatory joint involvement in the first decade of life, particularly with stiffness of the fingers and difficulty using the hands, should be screened for metabolic diseases, including MPS I. MPS I should be considered if patients with arthropathy lack the typical characteristics of inflammatory arthropathy.

Arthritis, Juvenile↗

Procollagen type I carboxy-terminal propeptide as a marker of osteoblastic bone metastases.

Collagen type I is the sole collagen type found in bones and tendons. Carboxyterminal propeptide, deriving and cleaved from procollagen type I (PICP) during collagen synthesis, is delivered into the blood, where it can be measured. According to current knowledge, PICP correlates with bone collagen synthesis and bone formation rate. Elevated serum levels of PICP in patients with Paget's disease, compared with normal subjects and correlated with serum alkaline phosphatase (Alk.Ph.), have been previously described. Thus, PICP may be a valuable marker of bone formation. PICP, serum Alk.Ph., serum bone Gla protein and 24-h urinary hydroxyproline:creatinine ratio have been measured in 47 cancer patients: 27 with predominantly osteolytic lesions (5 myeloma, 15 breast, 3 lung, 2 kidney, 1 bladder, 1 thyroid) and 20 with predominantly osteoblastic lesions (18 prostate and 2 breast). The higher levels of PICP were noted in patients with osteoblastic or mixed metastases. In the entire group of patients, a statistically significant correlation between PICP and bone Gla protein (r = 0.57; P < 0.001), PICP and Alk.Ph. (r = 0.80; P < 0.001), and bone Gla protein and Alk.Ph. (r = 0.44; P < 0.01) was noted. In those patients with osteoblastic metastases we observed a significant correlation only between PICP and Alk.Ph. (r = 0.62; P < 0.003). During chemotherapy, 13 of 20 patients with osteoblastic metastases who achieved objective response or stable disease showed a more rapid and significant decrease in PICP with respect to the other bone markers. Serum PICP level could be considered a good marker of osteoblastic activity.

Adult↗