Biomedical subjects

J Libert

Publications and source records attributed to J Libert.

83 records · Page 5Linked to original sources

PubMed1973

[Macular ectopia].

Explore the source record for details and available documents.

Diagnosis, Differential↗

PubMedDate not supplied

Prenatal diagnosis and confirmation of infantile sialic acid storage disease.

Amniocentesis was performed in a pregnancy at risk for infantile sialic acid storage disease. Greatly elevated levels of free sialic acid were found in cell-free amniotic fluid as well as in cultured amniotic cells from the fetus at risk. After incubation of the cultured amniocytes with fetuin labelled in its sialic acid moiety, pulse and chase experiments respectively showed accumulation and impaired release of TCA-soluble radioactive material in the amniotic cells at risk. These data thus clearly indicated that the fetus was affected. After pregnancy termination, ultrastructural studies of fetal organs and placenta showed a generalized storage picture characterized by clear membrane-bound inclusions. The diagnosis was further confirmed by the finding of greatly increased amounts of free sialic acid in fetal organs and cultured fibroblasts.

Amniocentesis↗

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Placental electron microscopy and histochemistry in a case of sialic acid storage disorder.

Morphological alterations in a placenta from a case of infantile sialic acid storage disease are described. Syncytiotrophoblast, villous capillary endothelial cells, amniotic and Hofbaüer cells were filled with membrane-bound inclusions which were either electron-lucent or contained fibrillogranular material. Hydrolysis of slides with neuraminidase demonstrated at six hours Alcian blue material which was not present in normal syncytium. The possibility that a storage disorder such as sialic acid storage disease can be accurately diagnosed by electron microscopy on chorion villous sampling at nine to ten weeks is emphasized.

Adult↗

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Evaluation of efficacy and safety of ciprofloxacin ophthalmic solution versus chloramphenicol.

The results of this clinical study demonstrate that ciprofloxacin ophthalmic solution 0.3% is as safe and as effective as 0.5% chloramphenicol ophthalmic solution in the treatment of conjunctivitis and blepharitis of bacterial aetiology. Both agents achieved microbiological improvement rates in excess of 90% after 1 week's treatment. On ciprofloxacin 93.5% of patients were judged clinically cured or improved versus 84.6% on chloramphenicol after 1 week. There were no serious adverse affects. One patient in each group suffered drug-related side-effects (chemosis, erythema) which resolved on discontinuation or changing of therapy. Cirpofloxacin is not associated with the rare, but serious, side effect of aplastic anaemia which is associated with chloramphenicol use. On the evidence of this study ciprofloxacin would appear to be an appropriate agent for general use as a topical ophthalmic formulation.

Acute Disease↗

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Ultrastructure of brain and retina in Kufs' disease (adult type-ceroid-lipofuscinosis).

A patient with Kufs' disease (adult type-neuronal ceroid-lipofuscinosis) is reported. He was the brother of the index case reported by Dom et al. [1979]. The diagnosis had been made by skin and skeletal muscle biopsies. The postmortem study of the brain confirmed the diagnosis and revealed a diffuse intraneuronal storage of membrane-bound inclusions with curvilinear, rectilinear and fingerprint profiles. Neurons from the frontal grey matter, the thalamic nuclei, the cerebellar cortex, the dentate nucleus, the substantia nigra and the anterior horns contained such inclusions, as demonstrated by electron microscopy. Because some light microscopic changes had been described in the retina of the brother, a complete ultrastructural examination of the eye was carried out in our patient. Except for signs of storage in the oculomotor muscles and in the smooth muscle cells of the ciliary body, the only ocular signs of storage were found in the retinal ganglion cells. Some of the latter contained inclusions which were quite analogous to the ones found in the neurons of the central nervous system. The pigmented epithelium, the photoreceptors and all the other retinal layers were normal. It is necessary to perform neurophysiological and electron microscopic studies at the retinal level to help clarify the confusion between the protracted and adult types of ceroid-lipofuscinosis since they may chronologically overlap.

Adult↗

PubMedDate not supplied