Biomedical subjects
Publications and source records attributed to J L Salomon.
Sarcoidosis is rare in children, who usually have only asymptomatic pulmonary involvement. We report granulomatous cheilitis in two children with sarcoidosis. Both children had systemic sarcoidosis with lymph node and pulmonary involvement.
The authors describe a valve for optic nerve sheath fenestration. The valve is composed of a silicone tube with one end placed between the two layers of the arachnoïd at the top of the optic nerve head and the other end blocked but laterally fenestrated with two slits, fixed to the sclera with two small silicone wings. The pressure inside the tube can open the two slit openings when greater than ten millimetres of mercury. This valve has been designed in order to obtain a better function for optic nerve sheath fenestration operations.
Optic nerve sheath decompression (ONSD) has become a more frequently performed surgical procedure in recent years. We describe the surgical techniques and complications and we detail the indications of ONSD in pseudotumor cerebri, ischemic optic neuropathy due to arteriosclerosis and other etiologies under investigation. With a 2% risk of postoperative blindness, this procedure must be indicated only in case of severe visual loss and performed by surgeon experienced to orbital surgery.
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Hereditary protein S deficiency was detected in three subjects belonging to three generations of one single family. The deficiency was heterozygous and was associated with recurrent venous thromboembolism in two of the subjects affected. Plasma analysis by two-dimensional immunoelectrophoresis showed that the protein S fraction bound to C4b-binding protein was quantitatively normal, whereas the free protein S fraction was much reduced. Since it has been well established that only free protein S intervenes in the regulation of blood coagulation, any quantitative deficiency, even moderate, in protein S resulting in redistribution of its free and bound fractions will have major functional repercussions.
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The present study relates to 39 children, 24 boys and 15 girls, aged 1 to 17 (mean age, 6 1/2 years) suffering from chronic airflow obstruction without muco-viscidosis. The search for a ciliary anomaly was motivated either by the coexistence of situs inversus (group I: 12 cases) or by the negative or scanty aetiological history (group II: 27 cases). The investigation of the cilia consists of a study of ciliary motility and an ultrastructural study of biopsy specimens using the electron microscope. Groups I and II were similar as regards severity of disease, assessed by the incidence of bronchiectasis and chronic hypoxia at rest; a higher incidence of neonatal respiratory distress was noted in group I (7 cases against 4 in group II). Ciliary immotility was particularly noted in group I (7 cases) and one case of weak motility in group II. The major ultrastructural anomalies of the axonemal complex were seen as well in both groups I and II; it was noted that all the structures may be totally or partially missing with the exception of the peripheral microtubules. The discussion centred on three points: 1. In the first analysis, the discordance between the existance of major ultrastructural anomalies of the axonemal complex, a priori incompatible with the conservation of ciliary motility on light microscopy; the link seems to lie in the percentage of cilia affected, the existence of ciliary dyskinesia and finally the disorientation of the basal corpuscles. 2. The immunologic abnormalities sometimes associated with a syndrome of ciliary immotility: that is the anomalies of leucokyte migration under the control of microtubular structures in the peri-centriolar region. 3. The practical consequences. Current treatment rests on daily respiratory physiotherapy and antibiotics adapted for cases of superinfection; also there are drugs stimulating ciliary activity perhaps by the effect on their residual motility; some substances carrying ATP and ATP-ase may re-establish ciliary motility anulled by the absence of dynein arms; also the observations of major ultrastructural abnormalities ought to lead to better genetic counselling than at present, where the mode or modes of transmission are uncertain.
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The purpose of this study was to determine imipenem concentrations in vitreous humor of non-infected human eyes. Ten patients undergoing vitrectomy were infused with a single dose of either 0.5g (5 patients) or 1g (5 patients) of imipenem. Vitreous humor was withdrawn 2 or 4 hours after the end of the infusion. Results differed in relation to the dose. After 0.5g, vitreous levels were stable (approximately 0.20 mg/l), but after 1g they were significantly higher (approximately 2 mg/l). These levels were above the minimum inhibitory concentration of imipenem for 90% (MIC 90) of the main species responsible for endophthalmitis.