bluuebunny/crossref_metadata_2025_split huggingface dataset:
Retinal dystrophy with macular hyperpigmentation in long chain 3‐hydroxy‐acyl‐coa dehydrogenase (lchad) deficiency
Authors: PEIRO C , PINILLA I , PEIRO B , JIMENEZ B , RAMIRO P , CASAS P , PEREZ‐GARCIA D , IBANEZ J , AVENTIN MP , CABEZON L 
Published: December 2011

AbstractPurpose LCHAD deficiency is an autosomal recessive inheritance metabolic disorder associated with myopathy, cardiomyopathy, hypoglycaemia, neuropathy and retinal changes as retinitis pigmentosa. It is also related to sudden death. The purpose of this study is to present a case of LCHAD deficiency associated with retinal pigmentary changes.Methods A 3 year‐olds children diagnosed of LCHAD deficiency was examined. Parents were consanguineous. He had several hospitalizations related to hypotonia and lethargia.Results Fundoscopy examination showed a retinal dystrophy with macular hyperpigmentation and pigmented aggregations and hypopigmentation changes. Both parents and one brother were examined showing no changes in their retinal aspect.Conclusion Retinal dystrophy in children can be related to metabolic disorders. LCHAD deficiency should always be discarded.
Frequency of anisocoria in patients with astenopia
Authors: BOYCHUK IM , KHRAMENKO NI , BUSHUYEVA NN 
Published: December 2011

AbstractPurpose Anisocoria ‐ an unequal size of pupils. According to literary data, almost 10 % of the healthy population has a difference in diameter of pupils no more than 1 mm in photopic conditions of illumination and in 20 % of cases – in mezopic. Differential diagnostics of physiological and pathological anisocoria frequently causes certain difficulty and in this question the essential help is rendered by modern diagnostic methods of research, including computer pupillography which allow evaluating objectively pupil sizes and its changes on different stimuli.To reveal if anisocoria in patients with astenopia can be found and what kind of anisocoria it is.Methods 91 patient at the age 7 ‐ 23 y.o. were observed. They had astenopia complaints; some of them had pupil unequal size, no somatic pathology. Pupillography was done by elaborated pupillography devise, blood circulation of the brain was defined by method of computer reoencephalography .Results 13 % (12) of observed patients had anisocoria. During direct, consensual and accommodative reaction asymmetry of pupil’s square have increased on 45,5 %, 51% and 67 % accordingly. Volume of blood supplement in internal carotids had increased values in average on 65 % from age norms. Marked asymmetry was found between right and left a. carotid internal which reached 50 % at 9 patients, and at 3 to 70 % in the given pool.Conclusion Anisocoria was revealed in 13% of patients with astenopia. Patients who had anisocoria, astenopia and accommodative dysfunction had functional disorders of brain blood circulation.
Clinical and Molecular Genetic Aspects of Optic Nerve Hypoplasia
Authors: TRABOULSI E 
Published: December 2011

AbstractOptic Nerve Hypoplasia is one of the leading causes of childhood blindness in the USA, with estimated incidence of 1/10,000 live births. It is characterized by a subnormal number of optic nerve axons with preserved blood vessels and glial tissue support. It is congenital and non‐progressive. The speaker will present clinical data on More than 100 patients from a pediatric clinic and will review the associated neurological and systemic problems in these patients. A review of underlying molecular genetic, pathogenetic mechanisms and environmental factors will also be given. Optic nerve hypoplasia is an important cause of irreversible blindness. It is undoubtedly a heterogeneous group of conditions. Environmental causes play an important role in its etiology and associated conditions are numerous. Endocrinologic complications are treatable and neurological problems need to be identified and addressed. Studies are underway to identify genetic causes and to provide guidelines for prevention and management.
Abnormalities of optical coherence tomography and visual evoked potentials in patients with chiasmal compression syndrome
Authors: WASIK M , PILECKI W 
Published: December 2011

AbstractPurpose To report abnormalities of retinal nerve fiber layer (RNFL) thickness measured by optical coherence tomography (OCT) and visual evoked potentials (VEP) in patients with compressive chiasmal tumors confirmed on MRI examination.Methods 22 eyes of 12 patients with parasellar tumors causing chiasmal compression syndrome were included to the study and compared to 59 eyes of 30 healthy patients. Exclusion criteria were any other ophthalmic or general condition that can affect both VEP and OCT measurements. All patients underwent MRI of the head to confirm the diagnosis, retinal nerve fiber thickness measurements with OCT and visual evoked potentials examination.Results Average RNFL thickness in patients with chiasmal compression (OD=96,06 ± 14,2µm, OS=92,4 ± 10,2µm) was significantly lower (p=0.016 and p=0.005) than in normal eyes (OD=104,7 ± 10µm, OS=104,5 ± 15µm respectively). It was noticed also reduced RNFL thickness in nasal quadrants. Visual evoked potentials showed mean P100 latency prolongation in examined patients (OD=112 ± 8,7ms and OS=108,4 ± 7,4ms), which was statistically significant (p<0.001 and p=0.001 respectively) in comparison to control group (103,1± 4,5ms and 102,5 ± 4,2ms respectively).Conclusion In patients with compressive chiasmal syndrome both RNFL thickness measurements with OCT and visual evoked potentials were significantly changed. These methods provide useful information in the diagnosis of chiasmal lesions.
Study of retinal nerve fiber layer in obstructive sleep apnea syndrome patients
Authors: FERRANDEZ ARENAS B , CALVO P , GIL‐ARRIBAS L , GÜERRI N , OTIN S , ALTEMIR I , GARCIA E , FERRERAS A 
Published: December 2011

AbstractPurpose To evaluate the peripapillary retinal nerve fiber layer (RNFL) thickness obtained with spectral‐domain optical coherence tomography (OCT) in obstructive sleep apnea syndrome (OSA) patients, as a biological marker of neuronal damage.Methods Sixty‐four OSA patients and one hundred twenty‐nine healthy controls were prospectively selected. Only one eye per subject was randomly chosen. AOS patients were classified in three groups according to apnea/hipopnea index: mild, moderate and severe. All participants had intraocular pressure less than 21 mmHg and performed at least a reliable standard automated perimetry (SAP). Peripapillary RNFL thicknesses were measured with Cirrus OCT (Carl Zeiss Meditec, Dublin, Ca). After checking for a normal distribution of variables, differences between both groups were tested by Student t test.Results Age was 50.6 ± 9.3 years in control eyes and 47.8 ± 11.5 years in AOS patients (p = 0.09). Mean deviation of SAP was ‐0.50 ± 1.0 dB and ‐1.4± 2.3 dB, in control and AOS patients, respectively (p<0.001). Pattern standard deviation and Visual Field Index (VFI) of SAP were also different between both groups. The RNFL thickness at 7 o’clock position and the average RNFL thickness had lower thicknesses in AOS patients compared to healthy individualsConclusion OCT detected a mild reduction of RNFL thickness in AOS patients compared with healthy subjects. Visual field indices were also different between both groups.
Anti‐VEGF treatment in ocular oncology
Authors: ZOGRAFOS L 
Published: December 2011

AbstractPurpose To present the indications of Anti‐VEGF treatment in ocular oncology.Methods Intraocular anti‐VEGF injections were used for: • The prevention or the treatment of neovascular glaucoma following irradiation treatment of intraocular tumors (uveal melanomas, choroïdal hemangiomas, choroïdal metastasis). • The control of cystoids macula edema related to the conservative management of intraocular tumors. • The treatment of secondary retinal detachment related to vascular tumors and pseudo tumors and mainly the Coat’s disease.Results A dramatic decrease of the risk of neovascular glaucoma was obtained with intravitreal anti‐vasoproliferative treatment following irradiation treatment of intraocular tumors. The presence of retinal ischemia associated to the secondary exudative retinal detachment is observed with panoramic (150°) fluorescein angiography. The anti‐vasoproliferative treatment is delivered during the period preceding the reatachment of the retina which allows a photocoagulation treatment. Anti‐vasoproliferative treatment in case of established iris neovascularisation allows a functional success (IOP less than 20mm Hg) in 84% of the cases and anatomical success (reduction of the iris neovascularisation and of diffusion of the dye in fluorescein angiography) in 75% of the cases. Impressive reduction of the secondary retinal detachment is obtained following anti‐vasoproliferatives treatment in cases of Coat’s disease allowing the photoablation of the reattached retina.Conclusion Anti‐vasoproliferative treatment in ocular oncology is a major contribution for the reduction of side effects induced by irradiation treatment and to maintain useful function in treated eyes.
Hyposmotic stress triggers ATP release from porcine lens via connexin and pannexin hemichannels
Authors: DELAMERE NA , BEIMGRABEN C , MANDAL A , SHAHIDULLAH M 
Published: December 2011

AbstractPurpose Purinergic receptors in the lens suggest function can be altered by agonists present in aqueous humor. Some agonists may originate from the lens itself. Here, we examine the ability of osmotic shock to trigger ATP release from the intact lens.Methods Porcine lenses were exposed to hyposmotic (200 mOsm) or hyperosmotic (500mOsm) solution and ATP in the bathing medium was measured by luciferase assay. Because hemichannels are permeable to large solutes, the ability of propidium iodide (PI) (MW 668) to enter the epithelium was examined.Results ATP release into the bathing medium was stimulated when lenses were exposed to hyposmotic solution. Hyperosmotic solution did not detectably increase ATP release. Hyposmotic solution‐induced release of ATP was partially suppressed by the connexin hemichannel inhibitor, 18α glycyrrhetinic acid (AGA) or by probenecid, a pannexin hemichannel blocker, and was abolished by AGA and probenecid added together. Consistent with opening of connexin and/or pannexin hemichannels, lenses exposed to hyposmotic solution displayed a ~4 fold increase in the ability of PI to enter the epithelium. In parallel studies, hyposmotic solution was shown to activate a Src family tyrosine kinase (SFK) and cause an SFK‐dependent increase of Na,K‐ATPase activity in the epithelium.Conclusion Hemichannels contribute to ATP release when the intact lens is subjected to hyposmotic shock. The amount of ATP release appeared sufficient to activate purinergic receptors that cause tyrosine kinase‐dependent stimulation of active Na‐K transport. The responses could perhaps signify an autoregulatory loop initiated by mechanical stress or osmotic swelling.
Stimulation of adrenergic β‐receptors enhances mydriasis in a porcine eye model
Authors: JANBAZ CC , LUNDBERG B , BEHNDIG A 
Published: December 2011

AbstractPurpose In order to achieve an improved mydriasis in human eyes during cataract surgery, the effect of intracameral phenylephrine was compared to the combination of isoprenaline plus phenylephrine, to epinephrine alone and to a negative control, in a porcine eye model.Methods A total of eighty‐nine eyes from newly slaughtered pigs were acquired and randomly split into four groups. In order to gain miosis all eyes received 2.0 mg of acetylcholine intracamerally, and after 60 seconds the eyes were injected with 0.15 ml 0.3% isoprenaline and 0.15 ml 3.0% phenylephrine sequentially with a 90‐second interval (n=21), the same substances in the reverse order (n=22), 0.15 ml of 0.025% epinephrine (n=20), or 0.15 ml of saline solution (n=26). The diameter of the pupils were measured every 15 seconds, and compared using Student’s t‐test.Results Phenylephrine injected after isoprenaline had a significantly (p<0.01) larger mydriatic effect than epinephrine. The mydriatic effect of phenylephrine, however, was significantly (p<0.05) smaller than that or epinephrine without the isoprenaline pretreatment. Isoprenaline also exhibited a small mydriatic effect of its own.Conclusion This study shows that the β‐receptor has a role in the dilation of the pupil, here demonstrated with the β‐receptor stimulator isoprenaline which augments the mydriatic effect of intracamerally injected phenylephrine. The superior mydriatic effects of a nonspecific adrenergic stimulator such as epinephrine in compare to the specific α1‐receptor stimulator phenylephrine may be explained by this β‐receptor mediated mydriasis.
Cationic oil‐in‐water emulsions protect and restore function of the injured ocular surface
Authors: DAULL P , LIANG H , BAUDOUIN C , GARRIGUE JS , BUGGAGE R , BRIGNOLE‐BAUDOUIN F 
Published: December 2011

AbstractPurpose Ocular surface damage is a consequence of tear instability arising from numerous inciting events: preserved eye drops, contact lens wear, systemic medications, environment and age. While unpreserved eye drop reduce iatrogenic toxicity they do not restore the deficient tear film which leads to ocular surface injury. Cationic oil‐in‐water emulsions have been shown to restore and reduce evaporation of the tear film. We studied the effect of cationic emulsions (Cationorm® and Catioprost®) in established animal models of ocular surface injury.Methods Acute toxicity and local tolerance were evaluated in rabbits. Healing properties were assessed in a rat model of corneal scraping. Abrasions were treated for 5 days, and corneas were evaluated clinically and histologically. Conjunctival function was assessed by goblet cell (GC) count and MUC5 immunostaining. The kinetics of ocular surface healing was assessed in an in vitro scraping assay.Results Neither Cationorm® nor Catioprost® induced toxicity as evidenced by clinical and confocal microscopy scoring. Catioprost® was well tolerated, with a reduced (‐42%) occurrence of hyperemia when compared to Xalatan®. In rats, Catioprost® improved healing, protected GC and maintained normal MUC5 secretion. In vitro, the cationic emulsions improved cell migration and maintained MUC4 expression.Conclusion Cationic emulsions were well tolerated. In contrast to preserved and unpreserved ophthalmic drops, cationic emulsions promoted healing, restored function of injured ocular surface, and protected against ocular surface injury. The findings suggest that cationic emulsions by augmenting the tear film may benefit patients with ocular surface disease.
Commercial interest
Structure of solitary sebaceous glands in the Sea Lion eye lid – a Meibomian gland equivalent ?
Authors: KNOP E , KNOP N , KELLEHER DAVIS R 
Published: December 2011

AbstractPurpose Pinnipeds stay not only in the sea but also on land and may then require a stable tear film. It was of interest to determine whether sea lions have meibomian glands for a superficial lipid layer that prevents tear evaporation.Methods Eyelids of a normal sea lion were embedded in parafﬁn and sections of 10‐20 µm thickness stained with H&E.Results A distinct tarsus was not detectable, the lid consisted mainly of muscular tissue with the lid margin forming a tip. Underneath the epidermis was a whitish granular layer that consisted of hair follicles and sebaceous glands. Bundles of holocrine acini were arranged between hair follicles. Towards the lid margin, the number of hair follicles decreased whereas the relative volume of sebaceous glands increased. At the lid margin, the sebaceous glands formed solitary glands without association to hairs or with only rudimentary hair shafts. The acini drained via short ductules into a straight duct with a four‐layered stratiﬁed squamous epithelium. The straight ducts opened via a terminal duct onto the outer lid skin.Conclusion The results show that pinnipeds have solitary sebaceous glands at the eyelid margin that resemble human meibomian glands in structure but are different in orientation and size. It remains to be determined whether the sea lion eyelid sebaceous glands represent an equivalent to human meibomian glands.
Characteristics of goblet cells in the conjunctival epithelium of the lid wiper explain the hydrodynamic type of ocular surface lubrication during the blink
Authors: KNOP N , KORB DR , BLACKIE CA , KNOP E 
Published: December 2011

AbstractPurpose The lid wiper at the inner aspect of the posterior eyelid border forms an epithelial lip apposed to the globe for distribution the pre‐ocular tear film. The present assumption of a squamous may not explain sufficient lubrication in order to avoid friction during the blink movement.Methods Conjunctival whole‐mounts including the lid margins from twelve normal human body donors were investigated by routine histology and semithin plastic sections, using histological stains, histochemistry and immunohistochemistry for MUC5AC.Results In routine histology the lid wiper showed a conjunctival epithelium with goblet cells, single and in clusters, at the luminal surface and also deep within the epithelium. Semithin sections revealed that the goblet cells at a greater depth were connected to cryptal epithelial infoldings that opened to the surface. Most goblet cells produced mucins of the neutral (PAS) and acidic (Alcian blue) type and also stained positive for the mucin MUC5AC. Surprisingly, MUC5AC negative goblet cells were also observed in the lid wiper.Conclusion In contrast to conventional assumptions, the lid wiper is a part of the conjunctiva and contains goblet cells & goblet cell crypts that are suitable to provide by a hydrodynamic type of lubrication for sufficient reduction of sheer stress between the lid margin and globe during the blink. This is another strong indication that the lid wiper is the area of the lid margin for the distribution of the thin preocular tear film.
Differential effects of various VEGF isoforms on endothelial cells and Tenon fibroblasts
Authors: VAN BERGEN T , VANDEWALLE E , VAN DE VEIRE S , MOONS L , STALMANS I 
Published: December 2011

AbstractPurpose We have previously shown that VEGF plays an important role in scar formation after glaucoma surgery. To clarify the differential effects elicited by VEGF isoforms, we compared the biological responses and signaling pathways activated by the various isoforms on endothelial cells and Tenon fibroblasts in vitro.Methods VEGF‐R2 and neuropilin‐1 (NRP‐1) expression was analyzed on endothelial cells (HUVEC) and Tenon fibroblasts (TF) by RT‐PCR. The effect of different VEGF isoforms (VEGF189, VEGF165 and VEGF121) on HUVEC and TF proliferation was determined by WST‐1 assay. The extracellular signal‐regulated kinase (ERK) pathway was evaluated by TransAM c‐Myc assay.Results HUVEC showed a higher expression of VEGF‐R2 and NRP‐1 mRNA as compared to TF. VEGF189 only significantly increased the growth of TF, whereas VEGF165 only increased HUVEC proliferation. VEGF165 strongly binds VEGF‐R2 and NRP‐1. As such, the combined reduced expression of VEGF‐R2 and NRP‐1 on TF explained why VEGF165 was more potent in inducing proliferation of HUVEC as compared to TF. VEGF121 exerted significant proliferative effects on both cell types by binding VEGF‐R2. However, similar concentrations of VEGF121 stimulated HUVEC more than TF, due to the lower expression of VEGF‐R2 on TF. All these stimulating effects on proliferation were associated with an activation of the ERK pathway.Conclusion Our data indicate that VEGF165 and VEGF121 predominantly affect blood vessel growth, whereas VEGF189 may be more important in fibrosis. Selective inhibition of VEGF165 (pegaptanib) may therefore be less effective to reduce ocular scar formation than non‐selective VEGF‐inhibition (bevacizumab), presumably due to retained action of VEGF121 and VEGF189.
Cis‐urocanic acid inhibits SAPK/JNK signaling pathway in UV‐B exposed human corneal epithelial cells in vitro
Authors: PAIMELA T , JAUHONEN HM , KAUPPINEN A , LAIHIA JK , LEINO L , SALMINEN A , KAARNIRANTA K 
Published: December 2011

AbstractPurpose Urocanic acid (UCA) is a major endogenous UV‐absorbing chromophore in the epidermis and it is also an efficacious immunosuppressant. The effects of cis‐UCA on UV‐B‐induced inflammatory and apoptotic responses in HCE‐2 cells, focusing on the nuclear factor kappa B (NF‐kappaB) and AP‐1 signalling pathways were studied.Methods After exposing HCE‐2 cells to UV‐B and cis‐UCA, the DNA binding of c‐Fos, c‐Jun and NF‐kappaB were measured with ELISA. In addition, the endogenous levels of phosphorylated stress‐activated protein kinase/c‐Jun N‐terminal kinase (phospho‐SAPK/JNK and phospho‐c‐Jun were determined. The proliferative capacity of HCE‐2 cells was also quantified, and the cytotoxicity of the cis‐UCA and UV‐B treatments was monitored by measuring the release of lactate dehydrogenase enzyme in the culture medium.Results UV‐B irradiation induced the binding of transcription factors c‐Jun, c‐Fos, and NF‐kappaB to DNA. Cis‐UCA inhibited the binding of c‐Jun and c‐Fos but not that of NF‐κB. Moreover, UV‐B increased the levels of phospho‐c‐Jun and phospho‐JNK, and the expression of both was attenuated by cis‐UCA. Cis‐UCA also alleviated the UV‐B‐induced apoptosis and proliferative decline in human corneal cells.Conclusion The results from this study suggest that cis‐UCA suppresses JNK signaling pathway, which provides potential for treating UV‐B‐induced inflammatory defects in human corneal cells.
Parkinson´s disease and retinal optical coherence tomography
Authors: JIMENEZ DEL RIO B , ASCASO FJ , LOPEZ DEL VAL J , CABEZON L , CASAS P , CRUZ N , PEIRO C , CRISTOBAL JA 
Published: December 2011

AbstractPurpose The aim of the study was to assess the retinal measurements and optic nerve head (ONH) morphology in patients with Parkinson's disease (PD) and to determine whether there is any correlation among these parameters and the disease´s duration.Methods Thirty patients affected by PD (mean age 67,3 ± 8,4; range: 49 ‐ 83) were compared with 28 age‐matched controls. In all subjects, peripapillary retinal nerve fiber layer (RNFL) thickness, ONH measurements, and macular thickness and volume were measured by optical coherence tomography (OCT).Results PD patients showed a statistically significant reduction of the overall peripapillary RNFL thickness [95±9µm, range: 73‐117] compared with those values observed in control eyes [103±7µm, range:87‐117] (p<0.0001, Mann‐Whitney U test). Furthermore, a statistically significant reduction of RNFL thickness was observed in nasal [74±20µm, range:18‐114](p=0.004), inferior [118±14µm, range:88‐147] (p<0.0001) and superior quadrants [120±13µm, range:88‐145] in PD patients when compared with controls [84±14µm, range:54‐106], [132±14µm, range:101‐165] and [125±13µm, range:101‐151] respectively (p=0.023; Mann‐Whitney U test). The temporal peripapillary quadrant, ONH measurements, macular thickness and volume did not reveal any statistically significant differences between both groups.Conclusion PD patients show a decreased peripapillary RNFL thickness evaluated by OCT. We also observe that the further evolution of PD, the lower average peripapillary RNFL thickness. Our results suggest that axonal degeneration could be present in the retina of PD patients and that RNFL thickness measured by OCT could be used as a biomarker for early diagnosis and for monitoring PD progression.