Antineoplastic busulfan encapsulated in a metal organic framework nanocarrier: first in vivo results.

Nanoparticles of a mesoporous iron(iii) trimesate MIL-100 nanocarrier encapsulating high amounts of the challenging antineoplastic busulfan were administered to rats and compared with the commercial Busilvex®. Large differences in serum concentration of both busulfan and trimesate revealed the great impact of drug encapsulation both on the drug and on nanoparticle pharmacokinetics during the first 24 h of administration.

[Predictors of quality of life in refractive surgery]. / Les prédicteurs de qualité de vie en chirurgie réfractive.

PURPOSE: To investigate predictors of quality of life in refractive surgery: psychological status, quality of vision, visual acuity, and patient satisfaction. METHODS: In a prospective study, 143 patients were evaluated preoperatively and after the third postoperative month using the same questionnaire. Quality of vision, visual acuity, well-being, self-esteem, coping, and patient satisfaction were analyzed with 47 parameters. Refractive procedures included PRK, LASIK, and phakic IOL. Four groups of low (G1), medium (G2), and high myopia (G3) and hyperopia (G4) were individualized to compare the results. RESULTS: We noted a mean postoperative best corrected visual acuity (BCVA) of 20/25 or more for the G1, G2, and G4 groups. For the G3 group, the mean BCVA was increased, with a gain of two Snellen Lines from 20/50 to 20/32. Postoperative patient satisfaction improved, with a significant difference (p<0.05) in all groups. We noted no significant difference in all myopic groups in well-being and no significant decrease in G4. Concerning self-esteem and coping, scores improved, with a significant difference in G3 group (p<0.05). Quality of vision was directly correlated with improvement of quality of life (psychological status), satisfaction scores, and BCVA preoperatively and postoperatively in all patients, but no correlation was noted between visual acuity and patient satisfaction. CONCLUSION: In spite of good refractive results, modification of patient satisfaction depends on quality of vision and quality of life (psychological status) scores. These results give us a new tool that provides useful additional information in refractive surgery.

[Corneoscleral involvement in congenital erythropoietic porphyria. Günther disease]. / Atteinte cornéo-sclérale au cours de la porphyrie érythropoïétique congénitale. Maladie de Günther.

Congenital erythropoietic porphyria (CEP) is an extremely rare autosomal recessively inherited disorder characterized by mutilating cutaneous photosensitivity and abnormal porphyrin heme synthesis in bone marrow. The present report describes a typical case of CEP with cornea involvement and scleromalacia in areas exposed to sunlight. The results obtained by conjunctival impression cytology are reported.

[Comparison of visual results and quality of vision between two multifocal intraocular lenses. Multifocal silicone and bifocal PMMA]. / Résultats comparatifs visuels et qualité de vision de deux implants multifocaux. Silicone multifocal et PMMA bifocal]

PURPOSE: To compare visual results and quality of vision of two multifocal intraocular lenses (IOL): SA 40 N silicone zonal-progressive multifocal IOL and P 359 TUV PMMA bifocal IOL. METHODS: 46 eyes were operated on for cataract. 24 IOL SA 40 N (group 1) and 22 IOL P 359 (group 2) were implanted. Evaluation of visual results was performed between the first and the third month postoperatively. The quality of vision was explored in both groups by using two tests of contrast sensitivity, an automated visual field and a questionnaire dealing with patient satisfaction and presence of halos and glare. RESULTS: Postoperatively, a myopic shift was noted with a statistical significant difference in the group 2 (p<0.05). The predictability was good in both groups (mean refractive error<1 D). Mean corrected distance visual acuity was similar in-groups 1 and 2 (0.6). Mean distance-corrected near visual acuity was statistically better in the group 2 (p<0.05). We noted in both groups a loss of contrast sensitivity statistically more important in the group 2 (p<0.001). Where the automated perimetry is concerned, no statistical differences were encountered. 35% of patients (group 1) and 29% (group 2) were very satisfied with the results of their surgery. Halos and glare were noted in 8% (group 1) and 9% (group 2) of the patients. CONCLUSION: The new concept of multifocality is increasing in practice of cataract surgery because of the reduction of spectacle dependency and the high level of patient satisfaction. However, the occurrence of halos and glare suggest a limitation of these indications of multifocal IOL, particularly for night driving patients.

Phakic posterior chamber lenses for high myopia: functional and anatomical outcomes.

PURPOSE: To evaluate the functional and the anatomical outcomes after implantation of phakic posterior chamber intraocular lenses (IOLs) in highly myopic eyes. SETTING: Service d'Ophtalmologie, Hôpital Purpan, Toulouse, France. METHODS: Fifty-eight eyes of 46 patients that had implantation of phakic posterior chamber lenses for high myopia were evaluated. Predictability, efficiency, safety, and subjective and objective quality of vision were evaluated preoperatively and at least 6 months postoperatively. The effect of the procedure on the cornea, aqueous humor, pupil, anterior chamber angle, crystalline lens, and retina were studied. RESULTS: Mean preoperative myopia was -13.85 diopters (D) +/- 3.1 (SD) (range -8.00 to -19.25 D). Mean postoperative spherical equivalent was -1.22 +/- 0.83 D (range +0.75 to -3.50 D); 56.9% of eyes were within +/-1.00 D of the predicted result, and 77.6% gained 1 or more lines of best corrected visual acuity. All contact-lens-intolerant patients had improved quality of vision for day and night driving, distance vision, and vision under dim illumination. The mean postoperative level of contrast sensitivity without correction was higher than the mean preoperative level with correction. Adverse events were 2 cases of crystalline lens opacification 16 and 18 months after surgery and 2 cases of pigment deposits in the angle with increased intraocular pressure, which was controlled by beta-blockers. CONCLUSION: Implantation of posterior chamber phakic IOLs is effective and predictable; however, long-term follow-up is needed.

Phakic posterior chamber lens implantation in children with high myopia.

PURPOSE: To evaluate the anatomical and functional results after implantation of a phakic posterior chamber lens (implantable contact lens [ICL]) to correct high myopia with amblyopia in pediatric patients in whom conventional treatments had failed. SETTING: Department of Ophthalmology, Purpan Hospital, University of Toulouse, France. METHODS: An ICL was implanted in 5 eyes with amblyopic high myopia in 4 children aged 3 to 16 years. Mean preoperative spherical equivalent refraction was -12.8 diopters (D) (range -8.0 to -18.0 D) and best spectacle-corrected visual acuity (BSCVA) was counting fingers to 20/200. Mean follow-up was 11.8 months (range 4 to 21 months). RESULTS: Postoperatively, the ICLs appeared to be well tolerated, with no inflammatory reactions, stable intraocular pressure, and good positioning in all eyes. Predictability was +0.5 D (range -0.5 to +2.0 D) and BSCVA improved, with a gain of 3 or more Snellen lines with recovery of binocular vision in 2 cases and orthotropia in 3 patients. All parents reported an improvement in their children's quality of life. CONCLUSION: This preliminary study indicates that ICL implantation is a safe surgical procedure to correct amblyopia resulting from high myopia in children when conventional amblyopia treatments have failed. Longer follow-up of more patients is needed.

[Predictability of intraocular lens power calculation in the treatment of cataracts in children]. / La prédictibilité du calcul de la puissance de l'implant dans le traitement de la cataracte chez l'enfant.

PURPOSE: To evaluate the predictability of intraocular lens (IOL) power calculation taking in account the changing refraction in children operated on for congenital cataract with primary posterior chamber IOL implantation. METHODS: We compared the predicted (PR) and objective (OR) refraction in 89 eyes of 70 children (1 month to 16 years) (1989 to 1995) at 1 month and 3 years after cataract surgery. The eyes were divided in 4 groups. Group A: < 1 year; Group B: 1 year to 3 years; Group C: 3 years to 5 years; Group D: > 5 years. For patients operated on before 3 years of age, we used a modified SRK II formula with the aim of achieving an undercorrection (85% of SRK II value in group A, 95% of SRK II value in group B). RESULTS: The average objective refraction (OR) and the average error (AE = (OR)-(PR)) were respectively at 1 month and 3 years after surgery for the Group A: (OR: +3.5 D/-1.8 D) (AE: -1 D/-6.4 D); Group B: (OR: +1.3 D/-1.3 D) (AE: -0.9 D/-3.6 D); Group C: (OR: -0.2 D/-1.4 D) (AE: +0.1 D/-1.1 D); Group D (OR: +0.1 D/-1 D) (AE: +0.8 D/-0.4 D). CONCLUSION: At 1 month after surgery, the predictability of IOL power calculation appears accurate with a refractive error between -1 D and +0.8 D in the four groups. However we noted an increase in refractive error at 3 years with a progressive myopic shift, specially in the eyes operated on before 1 year of life which had the greatest change in axial length.

Language dominance determined by whole brain functional MRI in patients with brain lesions.

BACKGROUND: Functional MRI (fMRI) is of potential value in determining hemisphere dominance for language in epileptic patients. OBJECTIVE: To develop and validate an fMRI-based method of determining language dominance for patients with a wide range of potentially operable brain lesions in addition to epilepsy. METHODS: Initially, a within-subjects design was used with 19 healthy volunteers (11 strongly right-handed, 8 left-handed) to determine the relative lateralizing usefulness of three different language tasks in fMRI. An automated, hemispheric analysis of laterality was used to analyze whole brain fMRI data sets. To evaluate the clinical usefulness of this method, we compared fMRI-determined laterality with laterality determined by Wada testing or electrocortical stimulation mapping, or both, in 23 consecutive patients undergoing presurgical evaluation of language dominance. RESULTS: Only the verb generation task was reliably lateralizing. fMRI, using the verb generation task and an automated hemispheric analysis method, was concordant with invasive measures in 22 of 23 patients (12 Wada, 11 cortical stimulation). For the single patient who was discordant, in whom a tumor involved one-third of the left hemisphere, fMRI became concordant when the tumor and its reflection in the right hemisphere were excluded from laterality analysis. No significant negative correlation was obtained between lesion size and strength of laterality for the patients with lesions involving the dominant hemisphere. CONCLUSION: This fMRI method shows potential for evaluating language dominance in patients with a variety of brain lesions.

[Predictability of amblyopia in ametropic children. Apropos of 96 cases]. / La prédictibilité de l'amblyopie chez l'enfant amétrope. A propos de 96 cas.

PURPOSE: The aim of this cross-sectional retrospective study was to analyze the predictability of amblyopia in children with myopic and hyperopic unilateral and bilateral ametropia. MATERIALS AND METHODS: One hundred and sixty two eyes of 96 children, (mean age: 8.6 years), were included in this work. Mean visual acuities and significant refractive errors were estimated for all the cases. The frequency of amblyopia and strabismus was studied in cases without amblyopia and in cases with medium and high grade amblyopia. RESULTS: Mean visual acuities were significantly higher in cases of bilateral myopia (p < 0.001) and hyperopia (p < 0.05) compared with unilateral ametropia. The frequency of myopic eyes (p < 0.01), eyes with high grade of myopia (p < 0.002) and anisomyopic eyes (p < 0.001) was significantly higher in cases of high amblyopia compared with cases without amblyopia. Strabismus (p < 0.05) were also significantly more frequent in cases of high amblyopia as well as in cases of myopic eyes (p < 0.01). Moreover, in the group of high amblyopia, 6 cases (6/7) had developed an intolerance for contact lenses. CONCLUSIONS: The risk of developing high grade amblyopia appeared significantly associated with unilateral medium and high level myopia. Strabismus and difficulties with good correction of anisometropia by contact lenses or spectacle appeared to be predisposing factors. This observation would suggest the indication of refractive surgery might be useful in these particular cases. To conclude, this study emphasizes the importance of early treatment of ametropia to reduce the incidence of amblyopia in children.

Visual outcome after paediatric cataract surgery: is age a major factor?

AIMS: To determine functional results after unilateral and bilateral cataract surgery in children with different aphakic optical correction. METHODS: In this retrospective study, we evaluated visual acuity and binocular vision in 107 children who underwent cataract surgery during the 10 year period from 1985 to 1995. Aphakia was corrected by an intracapsular intraocular lens (IOL), spectacles or contact lenses. RESULTS: Mean visual acuity was > 20/40 (< 0.3 log MAR) with normal binocular vision in 58 children over 7 months of age operated on for bilateral cataracts. Pseudophakic eyes regained visual acuity > 20/63 (< 0.5 log MAR) more often (90%) than aphakic eyes (46%) (p < 0.001). Binocular vision was also achieved more often after IOL implantation (p < 0.001). Visual outcome of early bilateral cataracts was less satisfactory in children with abnormal foveolar function. For 49 children who had surgery for unilateral cataracts, prognosis was poor when surgery was performed before the age of 7 months. For cataract surgery in older children (> or = 7 months) mean visual acuities were better with IOL implantation (p < 0.05). CONCLUSION: Cataract surgery with unilateral and bilateral IOL implantation can provide a beneficial effect on final visual outcome in children who are operated on before abnormal foveolar function develops.

[Acute orbital myositis and idiopathic inflammatory pseudotumor in children: three cases]. / Myosite orbitaire aiguë et pseudotumeur inflammatoire idiopathique de l'enfant: trois cas.

UNLABELLED: Orbital pseudo-tumors account for approximately 8% of the orbital tumors. They are scarcely described in children and raise diagnostic dilemas, especially when they present as an isolated extra-ocular muscle swelling called idiopathic orbital myositis (IOM). CASE REPORTS: A diagnosis of inflammatory orbital pseudotumors (IOPT) was made in three children aged 7, 13 and 14 years from clinical and CT scan and/or MRI findings associated with histological data in two of them. Two of these IOPT presented as IOM. Analysis was made in a effort to eliminate intra-orbital tumors, especially rhabdomyosarcoma. CONCLUSION: The abrupt onset of the orbital signs, often related in the literature, was not a specific diagnostic criterion regarding to embryonic rhabdomyosarcoma. The inconstant presence of inflammatory signs and the absence of local osseous defect could be more discriminating. Although diagnostic procedure, including histological documentation, was difficult, even dangerous, surgical biopsy should be recommended. Eventually, considering the mandatory multidisciplinary care, steroid therapy response should be evaluated as a diagnostic test.

Comparison of fluorine-surface-modified and unmodified lenses for implantation in pediatric aphakia.

PURPOSE: To compare the postoperative reaction in pediatric aphakic eyes to intercapsular intraocular lens (IOL) implantation with fluorine-modified poly(methyl methacrylate) (PMMA) IOLs and with unmodified IOLs. SETTING: Pediatric Ophthalmology Unit, Purpan University Hospital, Toulouse, France. METHODS: In this retrospective study, 107 consecutive eyes in 82 children were operated on for cataracts and intercapsular implantation. From January 1985 to December 1993, 87 eyes (Group 1) received an all-poly(methyl methacrylate) (PMMA), single-piece IOL; during 1994, 20 eyes (Group 2) received an IOL of the same design that had been fluorine surface modified. Mean age of the patients was 64.5 months (range 15 days to 16 years). Postoperatively, each group was monitored for immediate inflammatory response from the first to the eighth day and for secondary cicatricial response from 6 months to 1 year. RESULTS: There was no significant difference between the two groups in the early inflammatory response. Secondary cicatricial reaction was found more often in Group 1 in all age subgroups (P < .01), but especially 1 to 4 years (P < .02). CONCLUSION: Fluorine modification of PMMA IOLs is an effective and safe method to reduce the cicatricial response when IOL implantation is used to correct pediatric aphakia.

Functional MRI localization of language in a 9-year-old child.

BACKGROUND: Localizing critical brain functions such as language in children is difficult and generally requires invasive techniques. Recently sensory, motor and language functions in adults have been mapped to specific brain locations using functional imaging techniques. Of these techniques, functional MRI (fMRI) is the least invasive and has the highest spatial and temporal resolution. Its use in adults is well documented but application to children has not been as well described. In the present study lateralization and localization of language was evaluated with fMRI prior to epilepsy surgery in a nine-year-old male with complex partial seizures, attentional difficulty and decreased verbal proficiency. METHODS: Two language paradigms well studied in adults (read, verb generation) and two additional language paradigms (antonym generation, latter fluency) were studied using whole brain fMRI after stimulus items and timing were adjusted to achieve the desired performance level during imaging. The patient was also conditioned to the magnet environment prior to imaging. RESULTS: Word reading and letter fluency tasks produced lateralized and localized activation similar to that seen in adults. The patient had no language deficits following an anterior 2/3 dominant temporal lobe resection. CONCLUSIONS: With modifications of protocols such as those detailed in this report, this non-invasive method for localizing language function is feasible for the presurgical evaluation of children as well being applicable for a variety of developmental language issues.

[Visual and sensory results of surgical treatment of cataract in children. Apropos of 135 cases]. / Résultats visuels et sensoriels du traitement chirurgical des cataractes de l'enfant. A propos de 135 cas.

PURPOSE: Visual outcome after cataract surgery in children. METHODS: The authors studied the functional results (visual acuity and binocular vision) of 135 children operated for unilateral or bilateral cataracts during the 9-year period 1985-1994. Aphakia was corrected by an implant within the capsular bag, spectacles or contact lenses. RESULTS: For bilateral cataracts, (68 patients), mean visual acuity > 20/40 and normal binocular vision are found together in cases of late appearance operated after 7 months of age. Pseudophakic eyes regained visual acuity greater than 20/40 more often than non-implanted eye, with a statistically significant difference (P < 0.001). Binocular vision was also obtained more often in children after implantation (P < 0.05). The functional results of operations on early cataracts treated before the age of 8 months were not as good because of abnormal development of the foveolar function. For unilateral cataracts (67 patients), the prognosis was poor when operated before the age of 8 months. Visual acuity rarely exceeded 20/200 however the aphakia was corrected. Prognosis was better for partial cataracts and late-appearing cataracts. Implantation appeared to be of more benefit, with a statistically significant difference (P < 0.05) for visual acuity greater than 20/40 in children receiving an implant. CONCLUSION: Regained binocular vision and absence of amblyopia depend on the quality of previous visual experience and absence of post-operative strabismus. Implantation appears beneficial for final visual results.

[Intercapsular implantation in the management of cataract in children. Study of 87 cases and comparison to 88 cases without implantation]. / Implantation intercapsulaire dans les cataractes de l'enfant. Etude de 87 cas et comparaison à 88 cas sans implantation.

PURPOSE: We compared the surgical results and complications between two groups of children operated for cataract with or without lens implantation. METHOD: One hundred seventy-five eyes in 126 children, aged 15 days to 16 years were operated for cataract with a surgical procedure of limbus phacoaspiration. Eighty-seven received a primary intercapsular one-piece PMMA Intra Ocular Lens (IOL). Conditions for lens implantation were restrictive: strictly intercapsular, no other ocular pathology, at any age in unilateral and usually after 3 years of age in bilateral cataracts. Follow up ranged from 6 months to 10 years. RESULTS: Primary fibrinous anterior chamber reaction was seen in all patients in the IOL group. It always regressed under medical treatment. Glaucoma, retinal detachment and reoperations rates were similar in the two groups. There is a high correlation (p < 0.001) between initial anterior vitrectomy and keeping a clear visual axis, even in the eldest group, but no difference with or without IOL. Local tolerance of IOL was evaluated. No effect on visual results was evidenced. Evolution of refraction in pseudophakic eyes doesn't show evident myopic shift. Secondary myopias found in older groups might be associated to "genetic" myopia more than a consequence of implantation. CONCLUSION: Intercapsular lens implantation in children does not statistically increase the rate of complication of cataract surgery. It can be chosen if a real functional benefit is expected. The choice of IOL's power depends on biometric results which are a function of age. Under correction is usually recommended but immediate emetropia must be the aim in unilateral cases to ease amblyopia's treatment.

[Electric burns of the cornea. Anatomo-clinical study apropos of a case]. / Les brûlures électriques cornéennes. Etude anatomo-clinique à propos d'un cas.

The authors report a 32-year-old Kurd patient who sustained bilateral corneal electrical burn by torture in 1982. A penetrating keratoplasty surgery was performed in 1992. The clinical and histopathological corneal diseases were described.

Structural and ultrastructural changes in the developmental process of premature infants' and children's corneas.

Seven corneas ranging in age from 20 weeks of gestation to 6 months after birth were studied and changes observed in five layers. The epithelium thickens through the addition of new cellular layers and the enlargement of cells. The way junctional complexes develop with age is described. The way the collagen fibrils in Bowman's layer is arranged changes over time. The layer arrangement and orientation of the stroma's collagen fibrils are described at different stages of development. The keratocytes progressively flatten out and gather together. The thickness of Descemet's membrane increases from 0.6 m at 20 weeks of gestation to 4 m at 6 months. During the prenatal period, the banded layer appears with cross-linking bridges. The nonbanded layer appears after birth. Several changes were found in the endothelium: flattening out of the initially cube-shaped cells and changes in the intercellular junctions that can be seen from 20 weeks of fetal life. At 20 and 25 weeks of gestation, two layers of cells were observed in the corneal midperiphery. The adult structure of the cornea was reached at about 6 months after birth.

[Erdheim-Chester disease. A rare etiology of retrobulbar tumor]. / La maladie d'Erdheim-Chester. Une étiologie rare de tumeur rétrobulbaire.

The authors report the case of a 47 year old man who presented with bilateral retro-ocular tumor and an inflammatory syndrome as the first sign of his disease. Later on, the illness became polyvisceral and biopsies of retro-ocular and retro-peritoneal tissues revealed the diagnosis of Erdheim-Chester disease. The patient died a few months later.

Roles of extracellular and cytoplasmic domains of the prolactin receptor in signal transduction to milk protein genes.

We have previously shown that the long form of the PRL receptor is able to activate milk protein gene transcription. In the present study, we have determined the respective contribution of the extracellular and the intracellular domains of this receptor to transcriptional activation of a milk protein gene by PRL. The membrane-anchored intracellular domain (pTMI) expressed alone was devoid of PRL binding activity, as expected, and did not constitutively stimulate expression of the target gene. The extracellular domain (pE), expressed alone as a soluble receptor form, binds PRL with 10-fold higher affinity than the full-length membrane receptor. This form was also unable to stimulate the expression of the reporter gene. However, expression of both mutants (pE + pTMI) in the same cell partially restored the ability of PRL to activate the beta-lactoglobulin promoter. Replacement of cysteine 184 by a serine in the extracellular domain of the receptor impairs this restoration of the biological response. However, introduction of the same mutation in the full-length receptor did not affect its functional activity. These results indicate that the membrane-anchored cytoplasmic domain of the PRL receptor has no constitutive activity, and that coexpression of individual extracellular and intracellular domains leads to restoration of receptor function. We propose that restoration may be the result of reconstitution of the holoreceptor through disulfide bonding, or it may be the result of interaction of the extracellular region with an external transducing molecule.