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C Granata

Publications and source records attributed to C Granata.

At least 37 records · Page 2Linked to original sources
PubMed1997

Decreased bcl-2 expression in segmental renal dysplasia suggests a role in its morphogenesis.

OBJECTIVE: To evaluate the potential role of bcl-2 and apoptosis in the histogenesis of segmental renal dysplasia. MATERIALS AND METHODS: Surgically resected specimens of dysplastic upper poles of duplex kidneys were studied from 19 patients (mean age 3.4 years, range 2 months-9 years). Bcl-2 expression was detected immunohistochemically and apoptosis was investigated using the in situ end-labelling technique (ISEL). RESULTS: In the histologically normal areas of the specimens, bcl-2 expression was Intense in the proximal and distal convoluted tubules, moderate in Henle's loops and weak or absent in Bowman's capsule. In contrast, in the dysplastic areas there was no or markedly reduced expression of bcl-2 in mesenchyme and tubules. In the normal areas, apoptotic cells were extremely rare (0-1 per 25 high-power fields, HPFs), whereas in the dysplastic areas, some apoptotic cells were identified both in tubules (1-2 per 25 HPF) and in mesenchyme (5-8 per 25 HPF). CONCLUSIONS: As bcl-2 expression is required for normal kidney morphogenesis, finding no or markedly decreased expression of bcl-2 in the dysplastic areas may be related to the persistence of dysplastic fetal-type structures. Finding apoptosis adds further support to the concept that dysplasia represents arrested development at an early stage of morphogenesis before the period when bcl-2 assumes major importance.

Apoptosis↗
PubMed1996

Long-term results of spine surgery in Duchenne muscular dystrophy.

We report the long-term orthopaedic and functional results of segmental spinal instrumentation and fusion in 30 Duchenne patients. Twenty-nine had a mean 59% correction of scoliosis with post-operative immobilization in a brace of only three months on average and with a very limited loss of correction over time. One died after cardiac arrest. The mean vital capacity preoperatively was 57 +/- 17% with a decrease to 34 +/- 13% at 3.9 +/- 2 yr after surgery. The sitting position, aesthetic improvement and the quality of life after spinal fusion have been positively evaluated by the large majority of the patients and their parents. Head control was lost in the 14 patients who developed a more severe extension contracture of the neck measured as a significantly longer chin-sternum distance. More than 90% would have the operation or would give their consent again for their son having the operation.

Adolescent↗
PubMed1995

Reliability of dynamic strength knee muscle testing in children.

The isokinetic knee test is reliable in young individuals in a broad age range between 6 years of age to puberty, but not specifically in the youngest subgroup, in which cooperation may be less dependable. Hence, the purpose of this study was to investigate the reliability of isokinetic knee flexor/extensor muscle torque in a narrow age range: 6-8 years old. The sample consisted of 12 boys tested in three sessions with 3 days of rest between sessions. During each session, two tests were performed with each knee. Intrasession correlation coefficients ranged between .94 and .99 for quadriceps and between .78 and .96 for hamstrings. Interclass correlation coefficients, which express the reliability of a single test, were high (ranging from .85 to .95). Analysis of variance for repeated measurements showed that sixfold repetition of the test had no significant effect on peak torque measurements. These data suggest that the isokinetic knee flexion/extension torque is reliable in boys 6-8 years old.

Analysis of Variance↗
PubMed1993

Spine surgery in spinal muscular atrophy: long-term results.

Fifteen patients with spinal muscular atrophy operated on for scoliosis were reviewed and interviewed. Age at time of surgery averaged 16 yr. Follow-up averaged more than 5 yr. Eleven patients underwent posterior spinal fusion with Luque instrumentation. Four had Harrington instrumentation, with segmental wiring in three. Three out of the 15 also had anterior fusion with Dwyer instrumentation. Patients were immobilized in cast or brace for 6 months, on average, after surgery. The average curve correction was 50%. There was a loss of vital capacity after the operation, which in most cases was regained with time. In functional terms, there were improvements and deteriorations both in the short and long term. Esthetic appearance improved in all patients, as did the perceived well-being. Retrospectively all but one would have the operation again.

Adolescent↗
PubMed1993

Morphological and structural characteristics of the myopathic bone.

The aim of the present paper was to study the myopathic bone both from the morphological and structural viewpoints. To this end, samples from 8 patients affected by different neuromuscular diseases (DMD, SMA and CMD) have been examined. Thus, by SEM analysis a quantitative reduction of the trabeculae and an increase in porosity of the myopathic bone were found and also, by diffractometric analysis, the presence of pathologic crystalline phases (beta-TCP and beta-TCP-Mg).

Adolescent↗
PubMed1992

Epidemiology of neuromuscular disorders in the under-20 population in Bologna Province, Italy.

A retrospective epidemiological survey of the principal neuromuscular disorders was undertaken in the population under the age of 20 yr in Bologna city and province. The incidence of Duchenne muscular dystrophy (DMD) proved to be 25.8 x 10(-5), that is, comparable with the outcome of neonatal screening studies. The incidence of spinal muscular atrophy (SMA) was very high (11.2 x 10(-5), probably the result of our greater awareness and recognition of this disorder. The prevalence of neuromuscular disorders in the 0-19 yr population is 1.5 times that in the general population; that of DMD is 28 x 10(-5) vs 6.3 x 10(-5) and that of the SMA 6.5 x 10(-5) vs 1.2 x 10(-5). Our results indicate that neuromuscular disorders, particularly DMD and SMA I and II, have a much higher prevalence in the first two decades of life than is generally thought. Awareness of the higher prevalence of neuromuscular disorders among the under-20s is essential to the adequate planning of medical services.

Adolescent↗
PubMed1992

Isokinetic muscle testing (IMT) in neuromuscular diseases. Preliminary report.

Isokinetic muscle testing (IMT) allows precise and reliable measurement of the force produced by the skeletal muscle during exercise at constant velocity and accommodating resistance. This study reports on some clinical situations that illustrate the difference between manual muscle testing (MMT) and IMT in neuromuscular patients. IMT was performed by a special method (continuous passive motion plus gravity compensation) which allowed the measurement of very weak forces, such as in the four patients described in this study. It is important to note that for the same MMT grading the corresponding isokinetic force values were very different. Therefore there is an obvious correspondence between the isokinetic measurement of muscle strength and the morphological change in the muscle on the CT scan of the thigh, which did not show up on MMT. IMT could be extremely important for research into neuromuscular disorders, where the detection of even the tiniest variations in strength is relevant.

Adolescent↗
PubMed1990

Analysis, design and development of a carbon fibre reinforced plastic knee-ankle-foot orthosis prototype for myopathic patients.

A traditional knee-ankle-foot orthosis (KAFO) for myopathic patients has been studied for the assessment of loads and fatigue resistance. Starting from this basis a thermoplastic matrix carbon fibre reinforced plastic composite (CFRP) KAFO has been developed in order to reduce the weight. A finite-element simulation programme for deformation analysis was used to compare the behaviour of conventional and CFRP orthosis. There were no breakages either of the prototype or of its parts. The CFRP orthosis allows a weight reduction of more than 40 per cent.

Ankle↗
PubMed1989

Spinal muscular atrophy: natural history and orthopaedic treatment of scoliosis.

To study the natural history of scoliosis in spinal muscular atrophy (SMA), 63 spinal radiographs of affected patients who attended the Muscle Clinic of Rizzoli Orthopaedic Institute between 1974 and 1988 were reviewed. All but one of the intermediate SMA patients, and all of the mild SMA patients who stopped walking had a scoliosis that ranged from 10 degrees to 165 degrees. Out of the 19 mild SMA patients still able to walk, 12 had a scoliosis ranging from 10 degrees to 45 degrees. Mean age at the onset of scoliosis was 4 years 4 months in intermediate SMA, and 9 years 10 months in mild SMA. Data on characteristics of the scoliotic curve are reported. The effectiveness of orthopaedic treatment in the prevention of scoliosis is discussed.

Adolescent↗
PubMed1989

Scoliosis in spinal muscular atrophy: natural history and management.

This is a report on the natural history of 109 patients with infantile spinal muscular atrophy (SMA). All 18 children with the severe form died, as did five of the 52 with the intermediate form. Functional ability was more or less stable in the 47 living patients with the intermediate form, but 16 of the 39 with mild SMA lost the ability to walk. Ventilatory function was severely impaired in those with intermediate SMA: orthopaedic treatment for scoliosis in this group did not prevent the curves from worsening with age. Scoliosis was also severe in patients with mild SMA who had stopped walking: surgical treatment in six cases resulted in stable correction of the spine, with functional and cosmetic improvement and without impairment of respiratory function.

Adolescent↗
PubMed1989

Rigid spine syndrome and rigid spine sign in myopathies.

We studied eight patients with rigid spine syndrome aged 8 to 20 years at the time of first examination. Muscle weakness, rigid spine, and flexion contracture of elbows and ankles were noted in the first 6 years of age. Radiological study of the cervical spine revealed considerable reduction not only of flexion, but also of extension, of the neck. The "alligator sign" was demonstrated. Progression of scoliosis and of contractures reduced the functional capacity in six patients. A restrictive ventilatory syndrome was observed in all, while central apneas with oxygen desaturation were shown in two. Cardiac arrhythmias were observed in four. We suggest that a distinction should be made between myopathic rigid spine and rigid spine syndrome. Myopathic rigid spine is a clinical sign which is rarely sought but is present in several types of well-defined myopathies, meaning the myopathic involvement of the extensor muscles of the spine. Rigid spine syndrome is an axial myopathy with peculiar clinical and radiographic signs and a characteristic natural history.

Adolescent↗
PubMed1988

Growth hormone evaluation in Duchenne muscular dystrophy.

Growth hormone (GH) release with pharmacological tests and sleep test, somatomedin C and auxological features were studied in 10 patients affected by Duchenne Muscular Dystrophy. GH release in these patients seems to be lower than normal; moreover some of them are of short stature without an evident relationship with GH deficit. The possible significance of the data obtained is discussed, particularly in relation to the clinical course of the disease, and to current therapeutic trials with a GH release inhibitor (mazindol).

Arginine↗