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Biomedical subjects

J Libert

Publications and source records attributed to J Libert.

At least 19 recordsLinked to original sources
PubMed1991

Endogenous mycotic endophthalmitis in an infant.

We report a case of atypical bilateral endogenous mycotic endophthalmitis in an infant. A diagnostic vitrectomy was done on the left eye. Electron microscope examination of the vitreous demonstrated fungal mycelia. Intravenous treatment with amphotericin B seemed effective for treating the infection of the right eye, but a vitrectomy was necessary to prevent deprivation amblyopia and retinal traction, secondary to persistence of a dense vitreous membrane.

Amphotericin B↗
PubMed1989

[Vitrectomy in uveitis].

Although its indications are relatively unfrequent, vitrectomy may be justified in certain types of uveitis. It allows differential diagnosis between vitreal inflammation and infection, accurate diagnosis in several metastatic conditions, Whipple disease with ocular involvement, amyloidosis and specific inflammations of various origin. If vitreous opacities become prominent or if retinal complications occur, therapeutic vitrectomy may be indicated without major risks. It will offer a marked improvement of the vision, an easier treatment for new inflammatory episodes and maybe a reduction of the activity of the inflammatory process.

Amyloidosis↗
PubMed1989

[Prevention of bacterial infections using ciprofloxacin in granulocytopenic patients with cancer].

The optimal approach to reduce bacterial infections in granulocytopenic patients is still controversial. Recently, fluoroquinolones have been developed and real progress has been achieved in the prevention of Gram negative bacilli septicemia. This study reports our experience with ciprofloxacin and shows the excellent tolerance of ciprofloxacin by our patients as well as promising data for the reduction of Gram negative bacilli infection. However, practical modalities to prevent infection caused by Gram positive cocci remain to be defined.

Adolescent↗
PubMed1989

Cyclosporine in Behçet's disease resistant to conventional therapy.

Cyclosporine (cyclosporine A) at 7 to 16 mg/kg/day was administered for eight to 18 months to four patients with Behçet's disease with bilateral panuveitis resistant to the combination of prednisone, colchicine, and chlorambucil. Visual acuity was maintained or improved in all eyes. Color vision also improved significantly. A dramatic and significant decrease of the chronic vitreous haze occurred within the first two weeks of therapy and persisted until the end of the study. Retinal inflammation, including hemorrhages, exudates, edema, and fluorangiographic abnormalities, decreased progressively in all eyes. The severity of the anterior and posterior acute attacks also decreased significantly. This study suggests that cyclosporine is beneficial for the treatment of refractory and severe Behçet's disease, but its side effects require careful follow-up.

Adult↗
PubMed1985

Comparative toxicity of intravitreal aminoglycoside antibiotics.

We compared the toxicity of the aminoglycoside antibiotics (tobramycin, amikacin, netilmicin, and kanamycin) by ophthalmoscopy, light and electron microscopy, and electro-retinography after intravitreal injection in rabbits in doses ranging from 100 to 3,000 micrograms. The earliest manifestations of toxicity were confined to the outer retina with each drug, with lamellar lysosomal inclusions in the retinal pigment epithelium as the earliest finding. However, the aminoglycosides displayed marked differences in the threshold dose required to produce toxic reactions, permitting the following ordering of toxicity: (most toxic) gentamicin greater than netilmicin = tobramycin greater than amikacin = kanamycin (least toxic).

Amikacin↗
PubMed1984

[Fucosidosis. Ocular ultrastructure].

An ultrastructural study of the eyes of a six year old girl affected with fucosidosis demonstrated widespread overloading of lysosomes with a fibrillo-granular material of variable density. Conjunctival and corneal epithelial cells, keratocytes, sclérocytes, fibroblasts, corneal endothelial cells, retinal ganglion cells, inner segments of photoreceptors as well as glial cells of the optic nerve were markedly involved by the storage process. On the contrary, the pigment epithelium of the retina, the ciliary body and the iris were unaffected. Membranous cytoplasmic bodies were only disclosed within Schwann cells of conjunctival and ciliary nerves. Particularly striking lesions were observed within the endothelial cells of capillaries and veins of the eyes, as well as in all tissues examined, like the liver, spleen, kidney, lung, brain and skin. On the contrary, artery walls remained intact. These lesions explain the development of retinal vascular tortuosities, conjunctival aneurisms and cutaneous angiomas that characterize the clinical picture of our patient and that are often described in fucosidosis. The massive overloading of retinal ganglion cells contrasts with the absence of a macular cherry-red spot in fucosidosis. The nature of the stored material is probably responsible for this apparent discrepancy. Indeed, ganglion cells contain membranous cytoplasmic bodies in all diseases with cherry-red spots, whereas only a fibrillo-granular material was disclosed in the present case.

Capillaries↗
PubMed1981

Multiple sulphatase deficiency with early onset.

This male infant was first brought to attention in the neonatal period because he presented clinical and radiological evidence of multiple bone deformities. He was readmitted at 21/2 months for hydrocephaly, hepatosplenomegaly and poor somatic and psychomotor development. In addition, coarse facies, corneal opacities and stiff joints were noticed. Bone X-ray anomalies and vacuolized lymphocytes supported the clinical presumption of lysosomal storage disorder. The diagnosis of multiple sulphatase deficiency rests on the presence of MPS and sulphatides in the urine, the finding of a mixed storage process in conjunctival biopsy and the demonstration of deficiencies in arylsulphatases A, B, C, iduronate sulphatase and heparan sulphatase in serum, leukocytes and cultured fibroblasts.

Abnormalities, Multiple↗
PubMed1979

Skin and conjunctival biopsies in infantile neuroaxonal dystrophy.

The diagnosis of infantile neuro-axonal dystrophy (INAD) in a 5-year-old patient was confirmed by the ultrastructural study of neuromuscular, skin and conjunctival biopsy specimens. Abnormal networks of smooth membranous, lamellar and tubular profiles were found in presynaptic terminals and in conjunctival and dermal axons. INAD is the first neurological disease outside the group of storage disorders in which skin and conjunctival biopsies contribute significiantly to the diagnosis.

Axons↗
PubMed1979

Mucolipidosis IV. Histopathology of conjunctiva, cornea, and skin.

The condition of a 4-year-old white girl of Ashkenazi Jewish parents was diagnosed as mucolipidosis IV on the basis of marked corneal clouding and severe psychomotor retardation, in the absence of facial-skeletal dysplasia or abnormal mucopolysacchariduria. The results of histochemical and ultrastructural studies of conjunctiva, skin, and corneal epithelium confirmed the combined storage of acid mucopolysaccharide and complex lipid substances. An unusual histopathologic feature of mucolipidosis IV is the predisposition of extreme storage involvement of corneal epithelial cells with relative sparing of the keratocytes, which is a finding of potential therapeutic implication. In addition, application of electron microscopic study of cultured amniotic cells and conjunctival biopsy specimens to assess for the parents the mother's subsequent pregnancy additional emphasizes the value of ultrastructural studies in the diagnosis of lysosomal storage disease.

Amniotic Fluid↗
PubMed1979

Ocular findings in metachromatic leukodystrophy. An electron microscopic and enzyme study in different clinical and genetic variants.

Histopathological studies of the eyes from three patients affected with the infantile form of metachromatic leukodystrophy (MLD) showed the storage of metachromatic complex lipids in the retinal ganglion cells, in the optic nerve and the ciliary nerves, as well as the storage of a mucopolysaccharide-like material in the nonpigmented epithelium of the ciliary body. The lesions were limited to the optic, ciliary, and sensory nerves in a fourth patient with the juvenile form of the disorder. These morphological aspects, which are probably related to differences in sulfatase A activities, may explain the variability of the ocular manifestations in metachromatic leukodystrophy. Seven children affected with infantile MLD or with mucosulfatidosis were examined by conjunctival biopsy. Typical lesions of the sensory nerves were obvious and allowed the diagnosis of the disease. However, it seemed impossible to separate the different forms by histopathological studies only. The tear enzymes were assayed in most of the cases and demonstrated a profound deficiency of arylsulfatase A, or of arylsulfatase A and B, in the classical MLD and in mucosulfatidosis, respectively.

Cerebroside-Sulfatase↗