Muscle involvement in cholesterol ester storage disease.
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Child, Preschool↗
Biomedical subjects
C Navarro
Publications and source records attributed to C Navarro.
Morphological study of gastric lesions developing in the rat under several damaging conditions: modifications induced by pretreatment with zinc acexamate.
Lesions developing in the gastric mucosa of the rat after exposure to different gastric damaging agents (100 mg/kg aspirin, and 70% or 100% ethanol) were assessed by scanning electron microscopy. The severity of the lesions was quantified according to morphological criteria. Modifications in the severity of these lesions induced by pretreatment with zinc acexamate were also analyzed. The scanning electron microscope revealed that with the exception of absolute ethanol, which caused distinctive morphological features, lesions found under the different experimental agents shared a common pattern of progression. Ultrastructural lesions on surface epithelial cells preceded further alterations of parietal cells. After the integrity of the epithelial cells was lost, detachment of the parietal cells occurred, probably, through peptic digestion of the connections between cells and their extracellular matrices. Pretreatment of animals with zinc acexamate increased the presence of mucus on the gastric surface and significantly prevented the progression of lesions towards the severest stages. Ultrastructural damage of surface epithelial cells was not influenced by this treatment, but detachment of damaged cells was clearly diminished. These data confirm the protective effect of zinc acexamate against gastric aggressions. Moreover, our studies confirm the notion that mucus secretion and maintenance of continuity on the gastric lumen by surface epithelial cells is of critical importance in preventing the gastric damage induced in these experimental models.
Aminocaproates↗
[Cerebral amyloid angiopathy and recurrent cerebral hematoma. Clinicopathological study of a case].
Cerebral amyloid angiopathy (CAA) is an almost constant finding in Alzheimer's disease and in Alzheimer type senile dementia (EA/DSTA) but it has also been described in association with other processes such as in age-related hereditary or non hereditary cerebral hemorrhage (CH) relapse. The case of a non hypertensive 78 year old women is presented. Over a period of 27 months the patient had 3 cerebral hemorrhages located in the left parietotemporal, caudate nucleus and right frontobasal and right parietotemporal lobes, all of which had cortico-subcortical topography and eruption of blood to the subarachnoid space. The fundamental finding in the neuropathological study was the CAA with massive involvement in the leptomeninges and cortex, less in cerebellum and nucleus of the base, occasional in white matter and absent in the brain stem trunk. Abundant senile plaques and figures of neurofibrillar degeneration were found. Granulovacuolar degeneration or Hyrano bodies were not observed. At the level of the main intracraneal arterial trunks only a small plaque of atheroma was observed in the left vertebral artery. The association of CAA and CH in the literature and their relation with EA/DSTA are revised.
Aged↗
[Pleuro-pericardial effusion following the early intragraft thrombolysis of an occluded aortocoronary graft].
A case of contrast extravasation associated with pleuro-pericardial effusion occurring during recanalization of an early occluded aortocoronary bypass by intragraft fibrinolysis is reported. This is, to the best of our knowledge, the second report of angiographically demonstrated contrast extravasation from an aortocoronary bypass graft during this technique and the first associated with pleural effusion as a clinical manifestation.
Coronary Artery Bypass↗
[Continuing education. 69. The care of the neurosurgery trauma patient].
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Education, Nursing, Continuing↗
The hydC region contains a multi-cistronic operon (nik) involved in nickel transport in Escherichia coli.
We have cloned the multigenic hydC locus of Escherichia coli on a 7.1-kb BamHI-EcoRI fragment. Since its gene products are likely to be involved in the specific nickel transport [Wu et al., Mol. Microbiol. 3 (1989) 1709-1718], we propose a new gene designation, nik, to replace hydC. In vivo gene expression studies and complementation analysis support the notion that the nik locus is a multi-cistronic operon consisting of two to five genes. The first two genes, arranged in the order nikA and nikB, encode two proteins of 59 and 27.5 kDa, respectively. The downstream genes direct the biosynthesis of three polypeptides of 30, 28 and 25.5 kDa. Southern-blot analysis showed that mutant HYD720 carries a chromosomal deletion of 5.1-kb covering both nikA and nikB, whereas a 0.8-kb deletion in mutant HYD790 includes the sole nikB region. The cloned nik operon and the deletion mutants will aid greatly in the further molecular characterization of the multicomponent-specific transport of nickel in E. coli.
Bacterial Proteins↗
Anti-tumoral activity of L and D isomers of aspartic acid beta-hydroxamate on L5178Y leukemia.
D and L isomers of aspartic acid beta-hydroxamate (respectively DAH and LAH) were compared for their in vitro and in vivo activity against the murine leukemia L5178Y and their tolerance in vivo in DBA/2 mice. DAH and LAH displayed comparable cytotoxic activity against L5178Y leukemia in vitro. Death of leukemia cells was observed at concentrations above 1.2 mM for both DAH and LAH. High concentrations of L-asparagine partially reversed the growth-inhibitory effects of DAH and LAH on L5178Y cells for concentrations of DAH and LAH lower than 0.6 mM. Intraperitoneal administration of DAH and LAH to mice showed that the LD10, LD50 and LD90 of DAH was 3- to 4-fold greater for DAH than for LAH. DAH was able to eradicate L5178Y tumors in mice without inducing toxic deaths, whereas LAH at comparable doses killed all the animals treated.
Animals↗
Bone marrow transplant in a case of mucopolysaccharidosis I Scheie phenotype: skin ultrastructure before and after transplantation.
An 11-year-old girl with mucopolysaccharidosis I Scheie phenotype (MPS I-S) received a bone marrow transplant (BMT) from her heterozygous HLA-identical LMC-non-reactive mother. Multidisciplinary studies were carried out and results evaluated 21 months after transplantation. Herein we report the ultrastructural findings pre- and post-BMT in skin. Multidisciplinary studies are commonly used to evaluate the benefits of metabolic correction following BMT in some MPS and other inherited metabolic disorders, and changes in morphology have been described in liver and few other tissues. In this case, we elected skin, since connective tissue is universally involved in MPS and is safely and easily obtainable. Comparison of skin biopsy specimens taken before and after BMT showed a considerable change in dermal fibroblast morphology, with marked reduction in cell size and the number and size of abnormal lysosomes, thus indicating the clearance of storage. Our results demonstrate that dermal cells respond to enzyme replacement therapy in MPS I-S, with the clearance of glycosaminoglycan lysosomal accumulation in connective tissue fibroblasts, which had near-normal morphology 21 months after BMT. Therefore, the practice of skin biopsy after BMT in MPS and other metabolic disorders in which dermal cells are involved should be encouraged.
Bone Marrow Transplantation↗
Enzymatic responses of cat medial gastrocnemius fibers to chronic inactivity.
The role of neuromuscular activity in maintaining the normal enzyme heterogeneity found in a predominantly fast mixed muscle was studied. Enzymatic profiles of single fibers in the adult cat medial gastrocnemius (MG) were examined after almost complete elimination of neuromuscular activity for 6 mo. Inactivity was achieved by spinal cord isolation (SI), i.e., spinal transection at T12-T13 and L7-S1 combined with bilateral dorsal rhizotomy between the two transection sites. Cross-sectional area and succinate dehydrogenase (SDH) and alpha-glycerophosphate dehydrogenase (GPD) activities were determined in a population of fibers identified in frozen serial cross sections. Each fiber was categorized as light or dark on the basis of its staining characteristics for qualitative myosin adenosinetriphosphatase (ATPase), alkaline preincubation, and its reaction to fast and slow myosin heavy chain (MHC) antibodies. SI resulted in a conversion of nearly all light (approximately 36% in the control) to dark ATPase fibers. Virtually all MG fibers in the SI cats reacted with the fast MHC antibody, whereas very few fibers reacted with slow MHC antibody. On the basis of fiber cross-sectional area, it was estimated that the MG atrophied by approximately 10% after SI. Compared with the mean of the dark and light ATPase fibers in control (weighted by the percent fiber type distribution), mean SDH activity was significantly lower (approximately 70%) and mean GPD activity was significantly higher (approximately 120%) in the SI cats. These data indicate that prolonged electrical silence of a mixed fast hindlimb extensor results in virtually all fibers expressing fast MHC as well as oxidative and glycolytic enzyme profiles normally observed in fast glycolytic fibers.(ABSTRACT TRUNCATED AT 250 WORDS)
Animals↗
Experimental reactivation of chronic gastric lesions exposed to different aggressive conditions.
Experimental reactivation of chronic gastric lesions induced by acetic acid injection to the rat stomach was produced after exposure of the animals to different secondary damaging conditions. On day 18 after the initial injury, animals (n = 100) were distributed in five groups. One of them was used as control and the remainder were subjected to absolute ethanol, stress, pyloric ligation or aspirin. Measurements of gastric acid secretion were performed. Pyloric ligation resulted in the maximal rate of acid secretion. Computerized morphometric analysis of the gastric injuries showed a significant association (70%, p less than 0.01) of hemorrhagic lesions with the primary site of chronic injury in animals subjected to pyloric ligation. No significant association was observed after absolute ethanol (30%), aspirin (30%) or stress (35%). The presence of hemorrhage associated with the original gastric lesions was more dependent on the disorganization of the lamina propria and proliferation of chief cells in the margins of the mucosal scar than on the severity of extent of the chronic lesions. These results indicate that local conditions at the level of gastric mucosa together with an increased presence of acid in the gastric lumen provide favorable conditions for the reactivation of primary chronic lesions in the rat.
Acetates↗
[Idiopathic inflammatory myopathy. Analysis of 31 patients].
A series of 31 patients suffering idiopathic inflammatory myopathy (IIM); we describe the extramuscular manifestations, specially pulmonary, the association to neoplasia, the histopathological characteristics, and their response to treatment. Fourty three percent of IIM patients presented a pulmonary involvement, 9% presented an associated neoplasia. The histopathological study allowed us to clearly differentiate dermatomyositis and polymyositis within IIM. 65% of patients initially responded to glucocorticoids and the most usefull therapeutic alternatives were azatioprine and cyclosporin-A.
Adult↗
[Polymyositis as the first manifestation of acquired immunodeficiency syndrome].
We present a case of polymyositis (PM) in a patient with no previous relevant medical history, who had received blood transfusion four years ago during surgery. It could be demonstrated that the patient was HIV infected inspite the fact that he did not belong to any other high risk groups. PM was the clinical presentation form of HIV infection, the patient dying five months later due to a Pneumocystis carinii. The main clinical and histologic characteristics of PM in HIV infected patients are described as well as the main myopathies that these patients present.
Acquired Immunodeficiency Syndrome↗
[Streptococcus pyogenes not producing beta hemolysis during aerobiosis].
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Adult↗
Effect of zinc acexamate on gastric lesions induced by aspirin: a morphological study.
The morphology of gastric lesions induced by aspirin in the rat and their modification by pretreatment with zinc acexamate (100 mg/kg) were studied by scanning electron microscopy. The influence of mucosal levels of prostaglandin E2 (PGE2) on the development of these lesions was also investigated. High (200 mg/kg) or low (50 mg/kg) doses of aspirin inhibited PGE2 production similarly, but the morphology of these lesions differed considerably. While gross exfoliation of extensive areas of gastric mucosa was observed after 200 mg/kg aspirin, only ultrastructural lesions of surface epithelial cells were present after 50 mg/kg aspirin. Regardless of the dose of aspirin administered, pretreatment with zinc acexamate raised PGE2 levels and increased the presence of mucus. Our results showed that after zinc acexamate, the development of deep erosions appearing with high doses of aspirin was prevented and the ultrastructural lesions induced by low doses of aspirin were not observed. The fact that zinc acexamate did not modify the anti-inflammatory action of aspirin in the carrageenin-induced oedema model suggests that the protective effect of zinc acexamate is exerted locally on the gastric mucosa.
Aminocaproates↗
In vitro and in vivo anti-tumor activity of L-glutamic acid gamma-monohydroxamate against L1210 leukemia and B16 melanoma.
A glutamine analogue, L-glutamic acid gamma-monohydroxamate (GAH) demonstrated complete cytotoxicity against L1210 cells in culture and marked anti-tumoral activity in vivo against L1210 leukemia and B16 melanoma. In vitro, GAH caused concentration-dependent inhibition of L1210 cell growth, with complete cell death being reached at 72 hr and at a 500 microM concentration. A minimal incubation time of 38 hr with 500 microM GAH was necessary to obtain complete cell death at 72 hr. During incubation, GAH is metabolized to hydroxylamine. Hydroxylamine acts as the active form of GAH, since the concentration-dependent inhibition of cell growth caused by hydroxylamine is the same as that observed with GAH. The cytotoxic effects of GAH and hydroxylamine on L1210 cells were not reversed or prevented by L-glutamine or L-glutamic acid and purine nucleosides but were prevented or reversed by pyruvate, 2-oxaloacetate and 2-oxoglutarate. In vivo, GAH considerably increased survival of mice bearing L1210 leukemia or a solid tumor, the B16 melanoma. Antitumor activity of GAH against L1210 leukemia and B16 melanoma was schedule-dependent. The administration of GAH 3 times daily was more effective than a twice daily treatment and the maximum ILS was observed using split-dose schedules on days 1 through 3 and 7 through 9 without noticeable toxicity. Under these conditions hydroxylamine is highly toxic, suggesting that in vivo GAH might act as an hydroxylamine releaser in the tumor cells and is not significantly metabolized in the body.
Animals↗
[Cerebral infarct in a young adult, as the presenting form of myeloencephalopathic syndrome with lactic acidosis and cerebral ischemia].
We report a 26-year-old male who developed aphasia due to an ischemic cerebral infarction caused by MELAS (myoencephalophatic syndrome with lactic acidosis and cerebral ischemia). The most common causes of cerebral infarction in young patients were ruled out by laboratory investigations. The diagnosis of MELAS was suspected on the basis of past history of epilepsy, migraine and progressive sensory deafness, and increased resting blood lactic acid. Cerebral computed tomography showed bilateral caudate-putamen-pallidal calcification and nuclear magnetic resonance scan disclosed a left ischemic parietal-temporal-occipital infarction. The diagnosis was confirmed by muscular biopsy, which was characteristic of mitochondrial myopathy showing "red disarrayed" fibers in the histologic modified trichromic Gomori stain. Our patient showed that MELAS should be considered in young adults with cerebral infarction. The diagnosis should initially be suspected on a clinical basis, and confirmed by the presence of "red disarrayed" fibers with modified trichromic Gomori stain histologic muscle study.
Acidosis, Lactic↗
Skin biopsy findings in glycogenosis III: clinical, biochemical, and electrophysiological correlations.
Electron microscopy of skin specimens was performed in 4 patients (age range, 7 months-40 years) with glycogenosis III and revealed consistent abnormalities. Massive glycogen storage was observed in epithelial secretory cells of eccrine sweat glands and, less markedly, in smooth muscle fibers from the erector pili. Other cells, including Schwann cells of myelinated and unmyelinated fibers, were not affected. The extent of glycogen storage was similar in all patients and unrelated to age or duration of disease. The extralysosomal nature and selectivity of glycogen deposits, sparing fibroblasts and other cells, differ clearly from the findings in skin from patients with glycogenosis II. The purpose of this study was to show that glycogen deposits in glycogenosis III are not restricted to skeletal muscle and liver, and to assess the usefulness of skin biopsy in this disorder.
Adolescent↗
Muscle biopsy findings in systemic capillary leak syndrome.
Two patients with systemic capillary leak syndrome (SCLS) were followed up clinically for 5 and 2 years, respectively. Muscle biopsy was performed 1 week after the end of an acute crisis in one patient, and after 3 years in the other. In both cases, muscle capillary basement membranes were extremely thick--more than 15 times thicker than normal. Capillary basement membrane enlargement appears to be a permanent lesion, probably limited to muscle vessels. This finding has not been previously reported in SCLS, and would appear to indicate a relationship with the pathogenesis and severity of the crisis.
Adult↗