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Biomedical subjects

L Morandi

Publications and source records attributed to L Morandi.

72 records · Page 4Linked to original sources
PubMed1990

Dystrophin analysis in Duchenne and Becker muscular dystrophy carriers: correlation with intracellular calcium and albumin.

Immunocytochemical localization and immunoblot analysis of dystrophin in muscle fibers of 11 obligate and probable, and 7 possible carriers of Duchenne and Becker muscular dystrophy revealed an abnormal expression of the protein in 3 of them. Localization of calcium and albumin, as endogenous markers of extracellular fluid penetration, showed the presence of both molecules inside some fibers lacking dystrophin. Our morphological studies show that the initial stages leading to fiber necrosis in Duchenne muscular dystrophy are present in carriers with mosaicism. Comparison of dystrophin studies with restriction fragment length polymorphism analysis and creatine kinase levels showed that neither immunocytochemical nor immunoblot techniques for dystrophin are sensitive enough to provide a basis for genetic counseling.

Adolescent↗
PubMed1990

A modelistic approach to evaluate the factors affecting the 137Cs transfer from mother to fetus in cattle.

The authors have analysed the relationship between 137Cs radioactivity and pregnancy in cattle with radiometric analysis of muscle, kidney and liver of 24 cattle at various stages of pregnancy, fed with fodder exposed to radioactive fall-out. Also the relative fetal muscle, kidney, liver and myocardium have been analysed. The automatic spectra analysis and the statistical one have given the following results: there is no correlation between 137Cs retention and the pregnancy stage in the cattle sampled; the absolute 137Cs concentration is always larger in the kidney than in the muscle (Km/Mm = 1.7), while it is just minor in minor in the liver (Lm/Mm = 0.8). The fetal concentration in muscle, kidney and liver is generally homogeneous, whereas the myocardium tends to retain more 137Cs in the first stages of the pregnancy as against the other tissues. In the last stage of pregnancy it presents a lower concentration. The authors have included the fetal body weight growth to get a more realistic description of the 137Cs transfer in the mother-fetus system; this approach enables to evaluate the kinetic constants so introduced.

Animals↗
PubMed1989

Neurogenic muscle hypertrophy. Report of two cases.

Muscle hypertrophy is rare in denervating diseases. A patient with calf enlargement associated with L5-S1 radiculopathy and another with thenar, hypothenar, forearm and calf muscle hypertrophy in the course of chronic relapsing inflammatory demyelinating polyneuropathy are described. Gastrocnemius muscle biopsy revealed both type I and type II fibre hypertrophy in the former case and predominant type I fibre hypertrophy in the latter. Passive stretching and abnormal spontaneous muscular activity might have played a role in the origin of hypertrophy in both patients, but a satisfactory explanation for denervation hypertrophy has yet to be provided.

Biopsy↗
PubMed1988

Cardiac transplantation in Becker muscular dystrophy.

A 23-year-old man with X-linked Becker type muscular dystrophy underwent cardiac transplantation because of dilated cardiomyopathy which was complicated by terminal heart failure. Impairment of muscle function was mild and slowly progressive, whereas the cardiac disease was severe and rapidly progressive. All four chambers of the removed heart were grossly dilated; microscopically, the myocardial fibres were hypertrophic and pale; the nuclei exhibited pleomorphism with variability in nuclear size, shape, and depth of staining.

Adult↗
PubMed1988

Adjuvant chemotherapy with vincristine, cyclophosphamide, and doxorubicin after radiotherapy in local-regional nasopharyngeal cancer: results of a 4-year multicenter randomized study.

To evaluate the effect of adjuvant chemotherapy in patients with local-regional nasopharyngeal carcinoma (NPC) (squamous or undifferentiated) in complete remission at the end of curative radiotherapy (RT) 229 patients were randomized from 1979 to 1983 in a multicenter study to no further therapy (116 patients) or a combination of vincristine, cyclophosphamide, and Adriamycin (doxorubicin; Adria Laboratories, Columbus, OH) (VCA) for six monthly cycles (113 patients). The RT and RT + VCA groups were well balanced for median age (50 v 49 years), histology (undifferentiated carcinoma, 73% v 70%), tumor extent (tumor limited to nasopharynx, 57% v 57%), and nodal extent (negative nodes 26% v 24%, nodes in the lower cervical levels, 17% v 16%). RT was delivered to the nasopharynx, the base of the skull, and bilateral cervical nodes using a split course technique over 10 weeks up to the dose of 60 to 70 Gy in involved sites and 50 Gy to negative nodes. Response to RT was evaluated within 65 days post-RT treatment. Analysis at 48 months did not show significant difference between the two treatment groups in terms of relapse-free survival (RT, 55.8%, RT + VCA, 57.7%, P = .45) and overall survival (RT, 67.3%, RT + VCA, 58.5%, P = .13). The pattern of relapse was similar in the two treatment arms. Distant metastases were the cause of treatment failure in about 50% of relapsing patients. Although the results of the present study did not show any benefit from VCA administered after curative RT, combined systemic chemotherapy should be further explored due to the high incidence of local and distant failure after intensive RT.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1988

Dilated cardiomyopathy and successful cardiac transplantation in Becker's muscular distrophy. Follow-up after two years.

A 23 year-old man with x-linked Becker type muscular distrophy underwent cardiac transplantation because of dilated cardiomyopathy complicated by terminal heart failure. The muscular functional impairment was mild and slowly progressive, whereas the cardiac disease was severe and rapidly progressive. The ventricular cavities of the explanted heart were hugely dilated and the left ventricular wall thickness was moderately increased. Microscopically, a diffuse hypertrophy of the myocardial fibers and a widespread interstitial collagenous fibrosis were present. At a follow-up, two years after treatment, the patient is alive and fairly well; the degree of his muscular disability is substantially unchanged.

Adult↗
PubMed1987

Myopathy during amiodarone treatment: a case report.

The case of a 69-year-old woman who developed muscle weakness on chronic amiodarone treatment is reported. Muscle biopsy showed vacuolar alterations and a marked accumulation of amiodarone and of its metabolite desethylamiodarone was found in the muscle sample despite normal blood levels. Amiodarone-induced morphological alterations of the muscle may be related to the actual tissue concentration rather than to the blood level.

Aged↗
PubMed1987

Needle biopsy for muscle diagnosis and research: an Italian experience.

We report our experience of needle biopsy over the past two and a half, viz. 395 cases. We find that the technique meets the needs of diagnosis and research very satisfactorily. We describe its advantages over open biopsy and recommend it in preference to the latter, provided that the necessary technical skill for processing the sample is available.

Adolescent↗
PubMed1986

[Acute myopathy and hyperthyroidism].

A 40 year-old woman with a rapidly progressive proximal muscle deficit of all four limbs had an acute myopathy secondary to hyperthyroidism. Biopsy of the quadriceps femoris revealed signs of non specific muscle impairment with type II fiber atrophy. Treatment with methimazole and correction of the thyroid condition led to rapid disappearance of the disorders. Eighteen months later the clinical status was normal and a second quadriceps femoris biopsy showed that the muscle had normalized.

Acute Disease↗
PubMed1985

Thymectomy for myasthenia gravis: a fourteen-year experience.

Over the last 14 years we have thymectomized 106 myasthenic patients, 62 by transcervical and 44 by transsternal approach. At the end of the follow-up 49 patients (46.2%) had improved, 12 of these (11.2%) being in remission. Our data supply no guidance regarding the prognosis of individual patients at the time of thymectomy. In our hands neither operation seemed to modify the natural course of myasthenia gravis.

Adolescent↗
PubMed1985

Mapping of X-linked Becker muscular dystrophy through crossovers identified by DNA polymorphisms and by haplotype characterization in somatic cell hybrids.

The analysis of 10 X-linked DNA polymorphisms (five mapping on the short arm and five on the long arm) in two Becker muscular dystrophy pedigrees has been used to localize this gene in the known sequence of DNA polymorphic markers on the X chromosome. In the first pedigree, the carrier mother, whose phase for Becker and for five informative polymorphisms is known, has transmitted a double recombinant X chromosome to one of her two affected sons. The discordance between these two affected brothers for four of the five informative polymorphisms indicates that the Becker gene is located between RC8 or D2 on one side and pDP34 on the other. In the second pedigree, where the maternal grandfather is dead and two maternal first cousins are affected, the phase of DNA polymorphic alleles has been identified in somatic cell hybrids resulting from the fusion of hamster fibroblasts with lymphocytes of the mothers and aunt of the patients. The discordance between the two first cousins for two of the four informative DNA polymorphisms is best explained by the occurrence of a single recombination in the X chromosome carried by one of them. This result further restricts the localization of the Becker gene to a region of the short arm delimited by B24 and L 1.28. Regional and fine gene mapping through the approach described in this paper should become useful in the future for X-linked as well as for autosomal genes.

Animals↗
PubMed1984

Myoadenylate deaminase deficiency in a 5-year-old boy with intermittent muscle pain.

A 5-year-old boy with occasional pain of brief duration at the right or the left leg during exercise since the age of 4 years is reported. There was no weakness nor any other abnormality at the neurological examination. The serum creatine phosphokinase activity was elevated three times out of four. The electromyogram showed myopathic abnormalities in the biceps, it was normal in quadriceps and anterior tibial muscles. A quadriceps muscle biopsy was performed. There were no histological nor histochemical abnormalities at the routine techniques. However, a completely negative reaction with myoadenylate deaminase ( MADA ) stain was found. The MADA enzymatic activity in the muscle was very low. It is likely that the clinical syndrome is related to MADA deficiency.

AMP Deaminase↗
PubMed1982

Functional evaluation of Duchenne muscular dystrophy: proposal for a protocol.

A protocol for the evaluation of functional activities in subjects with Duchenne muscular dystrophy (DMD) was designed. The aim of our study was to define objective clinical criteria for the evaluation both of the clinical status of the patient and of the natural history of the illness itself. A protocol with such criteria is particularly necessary when testing the efficacy of treatment. 43 still-ambulant children with DMD between the ages of 3.10 yr and 10.4 yr were examined. Of this number 19 children were evaluated every 4 months over a period of 12 months; of these 14 formed part of a randomized double blind trial with L-carnitine (1.2-1.8 g/day) versus placebo.

Carnitine↗
PubMed1979

[Vascular myelopathies: anterior spinal artery syndrome. Considerations on 4 cases].

Four cases of acute onset myelopathy in which clinical and instrumental findings led to the diagnosis of anterior spinal artery syndrome are reported. The physiopathology, clinical medicine and treatment of vascular myelopathies are discussed on the basis of the series presented and reported data. It is also pointed out that vascular aetiology underlies a certain number of acute myelopathies, otherwise classified indiscriminately as myelitis.

Adult↗
PubMedDate not supplied

Content of methylhistidines in normal and pathological human skeletal muscles.

The content of 3-methylhistidine (3-MH) and 1-methylhistidine (1-MH) was measured in muscle biopsy specimens from 13 normal controls, 19 patients with Duchenne muscular dystrophy, 8 limb-girdle disease patients, and 23 disease controls with different forms of muscular pathology. 3-MH and 1-MH concentrations in normal human muscle did not appear to be influenced by sex, body weight, and age, at least for subjects in the 10--60 year age group examined. Skeletal muscle 1-MH levels did not significantly differ from mean control values in any of the pathologies investigated. In the patient population examined, the mean 3-MH level per unit of noncollagen protein (NCP) was significantly lower than normal in Duchenne dystrophy only, the reduction being related to disease severity. The significantly lower concentrations of 3-MH in muscle of Duchenne patients indicate the importance of measuring 3-MH in diseased muscle to obtain reliable estimates of the myofibrillar protein catabolic rate.

Adolescent↗
PubMedDate not supplied

Bethlem myopathy: early-onset benign autosomal dominant myopathy with contractures. Description of two new families.

Bethlem myopathy is an apparently rare early-onset benign autosomal dominant limb-girdle myopathy with contractures of the fingers. To determine whether this disorder is unrecognized rather than rare we used muscle computerized tomography (CT) and isokinetic muscle testing and assiduously sought contractures of the fingers in relatives of two patients with the disease. CT showed that muscle impairment was mild but more diffuse than clinically apparent and showed an unexpected progressive degeneration of lumbar paravertebral muscles. The isokinetic test showed that the quadriceps was more involved that the hamstrings. In addition we found that contractures of the last four fingers progressed centripetally with age from the distal interphalangeal joints to the wrist. As a result we proved that 15 of the 21 examined subjects had the disease, 7 of them being unaware that anything was amiss. Careful examination may reveal that Bethlem myopathy is more common than is now thought.

Adolescent↗