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Biomedical subjects

A Cabello

Publications and source records attributed to A Cabello.

At least 91 records · Page 5Linked to original sources
PubMed1993

Muscle carnitine deficiency and lipid storage myopathy in patients with mitochondrial myopathy.

Abnormal carnitine distribution in muscle was found in 22 of 77 patients (29%), with mitochondrial myopathy. Furthermore, total (TC) and free (FC) carnitine levels in muscle were lower in patients than in controls (P < 0.01). Muscle long-chain acylcarnitines (LCAC) were significantly increased in these patients (P < 0.01). Muscle carnitine deficiency was found in 31.5% of patients with lipid storage myopathy (LSM) and in 25.6% of patients with ragged-red fibers (RRF). Therefore, carnitine deficiency can be found in patients with mitochondrial myopathy even in the absence of LSM. Muscle levels of TC and FC were lower in patients with respiratory chain defects than in those with normal respiratory chain (P < 0.01). In contrast, LCAC levels were significantly increased (P < 0.05). Carnitine levels did not differ significantly, among patients with different respiratory-chain defects. Consequently, these patients, owing to their biochemical block, reduce progressively the muscle carnitine pool and subsequent LCAC rise, due to long-chain fatty acid (LCFA) accumulation.

Adolescent↗
PubMed1993

[Clonal myelodysplasia: prognostic stratification and therapeutic possibilities].

Myelodysplastic syndromes represent an entire group of clonal panmyelopathies with very distinct evolutionary pathways. Their common denominator, however, is a self-maintained functional failure of the myeloid hemopoiesis which tends to evolve into severe non-lymphoid leukemia (SNLL) in 20-30% of the cases. First, the prognostic value of each the following is reviewed: the morphological classification F.A.B., the stratification system for "Bournemouth group", the abnormal placement of immature myeloid precursors (ALIP) in bone marrow, and cytogenetic changes. Second, the therapeutic potential for each of the following is assessed: vitamin and support treatments; suprarenal steroids; conventional androgens and danazol; agents of cellular differentiation (cytosine arabinoside in low doses, retinoid acids, vitamin D3, etc.). Finally, the role of aggressive chemotherapies (in succession or unrelated to marrow transplant) in the eradication of myelodysplastic clone or post-myelodysplasia SNLL is examined.

Humans↗
PubMed1992

[Diagnosis of Alzheimer's disease. Evaluation of senile plaques of the diffuse type].

The different types of plaques throughout the thickness of the pole of the temporal lobule of brains from autopsies of a patient with Alzheimer's disease and two elderly patients with normal cognitive function were quantified. Thioflavin-S techniques and cholinesterase marking indicating amyloid aggregates were employed. If the Khachaturian criteria had been followed one of the controls would have also been diagnosed with involvement of Alzheimer's disease. The aforementioned control and patients with Alzheimer's disease had numerous senile plaques, most of which were diffuse, however, only the patient with senile dementia also had neuritic plaques. This fact indicates the convenience of reviewing the pathologic diagnosis of Alzheimer's disease in two ways: firstly, specific techniques for neurofibrillary degeneration should be included and secondly, the plaques with neurofibrillary degeneration should be quantified since diffuse plaques without degeneration appear to have a questionable significance with respect to this diagnosis.

Aged↗
PubMed1992

A seroepidemiological survey of antibodies to HTLV-I/HTLV-II in selected population groups in Paraguay.

Between March 1987 and November 1989 a cross-sectional serological survey was conducted on 884 residents of Paraguay to obtain data on the prevalence of antibodies to human T-cell leukemia virus type I/II (HTLV-I/II). Sera from 8/884 individuals (0.9%) were positive, confirmed by Western blotting and radioimmunoprecipitation (RIPA). This study shows that HTLV-I/II is very rare (or absent) among the general (healthy) population (0/338) and ethnic Japanese (0/227) in Paraguay. However, it can be detected at a rate of 2-3% in prostitutes (4/178) and homosexuals (4/117), suggesting sexual transmission as an important route for spread of HTLV-I/II in Paraguay.

Cross-Sectional Studies↗
PubMed1992

[Sneddon's syndrome: its clinical characteristics and etiopathogenic factors].

Sneddon syndrome is know as the association of idiopathic livedo reticularis and cerebrovascular lesions. The most characteristic trait of this syndromes is a non-inflammatory arteriopathy in medium caliber vessels. The pathogenic role of antiphospholipid antibodies in this disease is not clear. Clinical characteristics and etiopathogenic features of eight patients with Sneddon's syndrome are reviewed, specially regarding its relationship with primary antiphospholipid syndrome. A female predominance was found (3:1) as well as a relationship with hypertension (five patients suffered hypertension), but no relation was found with contraceptive use. Three patients showed evidence of antiphospholipid antibodies, present as anticardiolipin antibodies with significative titers in three cases and lupus anticoagulant in one. Digital artery biopsy performed in four patients showed in all of them the pathologic features characteristic of this disease. Seven patients were treated with platelet activity inhibitors and one with oral anticoagulants. Six of them have had a year and half follow-up without showing any new ischemic stroke. The main etiopathogenic factor on Sneddon's syndrome is the presence of a non-inflammatory arteriopathy in medium caliber vessels. Blood hypertension and antiphospholipid antibodies could play a role in the development of cerebrovascular lesions in some cases. No relationship has been found with oral contraceptives in this series of patients. Medium term prognosis with platelet activity inhibitors therapy seems benign.

Adult↗
PubMed1991

Continuous muscle fiber activity, peripheral neuropathy, and thymoma.

Two patients, one of them with myasthenia gravis, presented symptoms of continuous muscle fiber activity syndrome before discovery of a thymoma. Peripheral neuropathy was present in both patients, with axonal and demyelinating lesions in sural nerve biopsy. The syndrome remained unchanged or worse after thymectomy. Both patients died of associated complications.

Aged↗
PubMed1991

Astroblastoma: electron microscopy and immunohistochemical findings: case report.

The clinical, histological, immunohistochemical, and electron microscopic features of a cerebral astroblastoma are reported. The patient is a young woman with a superficial parietal tumor. Macroscopic findings include a well-delineated superficial nodule with a hard central core. Histological study disclosed a predominantly papillary tumor with hyalinized vessels. Tumor cells were scarcely positive with immunohistochemical stain for glial fibrillary acidic protein, extensive and diffusely positive with vimentin and neuron-specific enolase, and intensely positive with S-100 and epithelial membrane antigen in the papillary areas. Ultrastructural study showed abundant intermediate filaments forming bundles in tumoral cytoplasms, membrane junctions, and external laminae when cells were in contact with collagen fibers. Based on immunohistochemical and ultrastructural characteristics, we believe that the filaments seen in tumor cells are mainly vimentin filaments. These peculiar immunohistochemical patterns in a glioma may aid in the histological diagnosis of this rare tumor type.

Adult↗
PubMed1991

[Congenital fiber-type disproportion: analysis of a series of 11 cases].

We present 11 patients with congenital fiber type disproportion suggesting the existence of two different clinical groups. The first group not associated with other diseases, presents a uniform clinical picture and a generally good prognosis, although the patients with severe respiratory involvement can die. The second group includes the cases in which CFTD is associated with other congenital diseases such as Lowe's Syndrome, Möbius' Syndrome, hypothyroidism and hydrocephalus. In this group the clinical presentation and prognosis is that of the associated disease added to that of CFTD. The frequent association of CFTD with other congenital diseases suggests that CFTD may not be a specific myopathy but a histological abnormality due to different pathogenic insults which interfere with the normal growth and maturation of the muscle fibres.

Abnormalities, Multiple↗
PubMed1990

[Hypothyroid myopathy. Clinico-pathologic study of 20 cases].

20 patients afflicted with primary hypothyroidism were studied in order to evaluate the association of clinical or sub-clinical myopathy, detected by neurophysiological (electromyography) (EMG) or neuropathological methods (muscular biopsy with enzymatic study). 70% of the patients had muscular weakness (moderate in 30% and severe in 40%) of the scapular and pelvic muscles. 60% of the patients had muscular cramps. There was no myodema nor muscular atrophy or hypertrophy. Seric CPK was high in 70% of the cases. EMG was myopathic in 65%. All cases with weakness registered EMG alterations. The histological findings were import findings were important. The enzymatic techniques showed alterations of the fiber subtypes in 90% of the cases. The type I fibers had sarcolemmal and mitochondrial accumules in 85% and 70% had areas without oxidative activity, similar to "core". In this study, we did not find any correlation between the evolution time of hypothyroidism, hormonal levels, CPK increase, and muscular weakness. The EMG was myopathic in cases with severe weakness, however, in patients with moderate weakness it could also prove abnormal. There was no correlation between the electric myopathic pattern, CPK levels and thyroid hormones.

Adult↗
PubMed1990

[Thyroid myopathy. Effect of treatment with thyroid hormones].

We studied 20 patients afflicted with primary hypothyroidism and treated with thyroid hormone replacements, with the aim of evaluating the clinical, biochemical, EMG and anatomo-pathological effects. Cramps, as well as subjective and objective proximal muscular weakness, disappeared. Seric CPK was normalized in all cases after 1-8 weeks of treatment. EMG myopathic patterns faded away in all cases. Anatomopathological changes took longer to observe; 5 cases with new biopsy showed an isolate atrophy of type II fibers, one of them after 18 months in treatment; type I fibers had mitochondrial accumula without change compared to the initial biopsy. There was a clear tendency for the destroyed fibers to become normalized and for the structures similar to the "core" to vanish.

Adult↗
PubMed1990

Chronic inflammatory demyelinating polyneuropathy as first manifestation of human immunodeficiency virus infection.

We studied three patients who were admitted to the hospital because of progressive weakness without other systemic signs or symptoms. All three cases were young males who had been intravenous drug user for many years. Electrophysiologic study showed prolonged distal latencies and marked slowing of motor and sensory conduction velocities, consistent with primary demyelination. Nerve biopsy also showed signs of demyelination. Antibodies against HIV in CSF and blood were detected during the diagnostic evaluation. Clinical and electrophysiological studies improved in two cases after prednisone administration. Patients with predominant motor demyelinating neuropathies and risk factors should be screen for HIV infection.

Acquired Immunodeficiency Syndrome↗