Rapid, Sensitive, and Selective "ON-OFF" Detection of Fe3+ Ions Using Novel Acetalophanes and Their Applications in Real Samples.

Three novel acetalophanes 1a-c have been designed, synthesized and characterized. The receptors 1b-c, featuring bulky anthracene groups, displayed significant selectivity for Fe3+ ions, resulting in a turn-off fluorescence mode in a DMF-buffer solution. Conversely, the non-steric probe 1a could serve as a versatile sensor for the simultaneous detection of Fe3+ and Cu2+ ions in MeOH-buffer solution. The sensing mechanism for the capability of 1a was demonstrated to be different, as evidenced by the addition of cyanide ions. The probes with Fe3+ exhibited a sensing mechanism that resulted in the deprotection of acetals to the corresponding starting materials, as confirmed by 1H NMR, IR spectra and TLC analysis. The attractive features of these practical and efficient sensors are selectivity, sensitivity (limit of detection = 0.15 µM by 1a, 0.16 µM by 1b and 0.14 µM by 1c), rapid response (less than 5 s). The on-site monitoring of various real samples, including well water, apricot, and green tea, proved to be successful for the quantitative and cost-effective detection of Fe3+. The method demonstrated good precision, even in the presence of other interfering materials.

Substrate-Controlled Diastereo- and Enantiodivergent Synthesis of Bis-Spirocyclopropyloxindoles from Available Isatin as a Single Starting Material.

The first diastereo- and enantiodivergent asymmetric synthesis of new bis-spirocyclopropyloxindole scaffolds has been accomplished from the readily available isatin as a single starting material. Four rel-(1R,2R,3R), rel-(1S,2S,3R), rel-(1R,2R,3S), and rel-(1S,2S,3S) configurations of desired products were constructed in excellent enantiopurity via a simple switch in substrates using the chiral auxiliary-controlled method. The absolute configuration of cycloadducts with three contiguous quaternary/tertiary stereogenic centers was confirmed through X-ray diffraction analysis. A facile synthesis of versatile precursor 3-chlorooxindoles was also introduced.

Regioselective synthesis of enantiopure 1,2- and 1,3-dispirooxindoles along with a DFT study.

In the present study, a library of important enantiopure dispirooxindole [indolizidine, pyrrolizidine, and pyrrolidine] derivatives with three or four contiguous and two quaternary stereogenic centers using different amino acids (pipecolic acid, sarcosine, proline and hydroxyproline) were synthesized in high yields (up to 96%) through a regio- and diastereoselective (up to 99 : 1) multicomponent 1,3-dipolar cycloaddition strategy. Based on the results, the alteration of amino acids led to a change in the regioselectivity and unusual regioisomers (pyrrolizidine versus indolizidine/pyrrolidine) were obtained to construct a novel enantiopure 1,3-dispirooxindole skeleton. The stereochemical outcome of the cycloaddition was determined by single crystal X-ray diffraction analysis and the self-disproportionation of enantiomers (SDE) test confirmed the enantiomeric purity of the desired products. The mechanism and differences in the regioselectivity of the 1,3-dipolar cycloaddition reactions between the stable azomethane ylides obtained from ninhydrin, pipecolinic acid, and proline with (E)-2-oxoindolin-3-ylideneacetyl sultam were theoretically studied through DFT calculations at the M06-2X/6-31G(d,p) level in methanol.

Tear proteomics analysis of patient suffered from delayed mustard gas keratopathy.

Understanding the molecular and cellular mechanisms involved in the pathogenesis of ocular injured induced by mustard gas can help better identify complications and discover appropriate therapies. This study aimed to analyze the proteomics of tears of chemical warfare victims with mustard gas ocular injuries and compare it with healthy individuals. In this case-control research, 10 mustard gas victims with long-term ocular difficulties (Chronic) were included in the patient group, while 10 healthy persons who were age and sex matched to the patients were included in the control group. Schirmer strips were used to collect the tears of the participants. Proteomics experiments were performed using the high-efficiency TMT10X method to evaluate the tear protein profile, and statistical bioinformatics methods were used to identify the differently expressed proteins. 24 proteins had different expressions between the two groups. Among these 24 proteins, 8 proteins had increased expression in veterans' tears, while the remaining 16 proteins had decreased expression. Reactome pathways were used to look at proteins with various expressions, and 13 proteins were found to be engaged in the immune system, 9 of which were effective in the innate immune system, and 5 proteins were effective in the complement cascade. Ocular mustard gas exposure may cause a compromised immune system on the eye's surface, exposing the cornea to external and endogenous infections, and eventually causing corneal opacity and reduced vision.

Comparison of the effect of removing bandage contact lens on the days 3 and 5 following photorefractive keratectomy.

AIM: To compare the outcomes of removing bandage contact lens (BCL) on days 3 and 5 after photorefractive keratectomy (PRK). METHODS: One hundred patients who underwent PRK (a total of 200 eyes) were enrolled in the present study. The subjects were assigned to two groups. Group 1 consisted of the right eyes of subjects and bandage contact lenses removal were on the 3rd day; Group 2 consisted of the fellow eyes of same subjects and removed bandage contact lenses on the 5th day after PRK. Then, data obtained from both groups were compared. To evaluate complications, the subjects underwent a slit-lamp examination in all visits. RESULTS: Filamentary keratitis was observed in one eye in both groups. The frequency of haze was higher in group 1; however, it was not significant between the two groups. Using mixed model analysis, significant differences were observed in the rate of complications as well as pain and eye discomfort scores between the groups (P < 0.05). No major complication was reported. CONCLUSION: Majority of post-PRK corneal epithelial defect is healed on day 3. However, keeping BCL for 5 days postoperatively instead of the three days produces a slightly lower rate of total complication.

Safety of grafting acellular human corneal lenticule seeded with Wharton's Jelly-Derived Mesenchymal Stem Cells in an experimental animal model.

The present study was conducted to evaluate safety of grafting acellular human corneal lenticule seeded with Wharton's Jelly-derived Mesenchymal Stem Cells (WJSC) in an experimental animal model. Human corneal lenticules were decellularized with a rate of about 97% with an acceptable lack of cytotoxicity and relatively intact ultrastructure of the lenticules. 12 rabbits underwent unilateral stromal pocketing with implantation of decellularized lenticules. Implantation was performed for 6 rabbits along with graft recellularization with WJSCs. Rabbits were euthanized after 1 month (n = 6) and 3 months (n = 6) to evaluate progression of graft bio-integration. No clinical rejection sign was detected during the study. Histopathological analysis showed that, grafts were integrated well with the least distortion of surrounding collagen bundles. After 3 months, labeled WJCS was detected representing viability of stem cells in the host. Increased expression of keratocyte-specific markers showed the potential of recruiting WJSCs as keratocyte progenitor cells to reinforce corneal ultrastructure.

The assessment of the concentration of candidate cytokines in response to conjunctival-exposure of atmospheric low-temperature plasma in an animal model.

BACKGROUND: Atmospheric Low-Temperature Plasma (ALTP) can be used as an effective tool in conjunctival cyst ablation, but little is known about how conjunctival ALTP-exposure affects the concentration of inflammatory mediators and also the duration of inflammatory responses. METHODS: We used 8 female adult Lewis rats that were followed up in 4 groups. The right eye of each rat was selected for the test, whereas the left eye was considered as a control. The ALTP was generated and used to target 3 spots of the conjunctiva. The digital camera examinations were performed to follow-up the clinical outcomes after ALTP exposure. Tear and serum samples were isolated-at 2 days, 1 week, 1 month, and 6 months after treatment-from each rat and the concentration of candidate pro-inflammatory (i.e. IL-1α, IL-2, IL-6, IFN-γ, and TNF-α) and anti-inflammatory cytokines (i.e. IL-4 and IL-10) were measured using flow cytometry. RESULTS: The external and digital camera examinations showed no ocular surface complications in all ALTP-exposed rats after 1 week. The analyses revealed that the ALTP transiently increases the concentration of pro-inflammatory cytokines-IL-1α and IL-2 in tear samples in 1 week and 2 days after exposure, respectively; no differences were observed regarding other pro- and anti-inflammatory cytokines in the tear or serum samples. CONCLUSIONS: ALTP can probably be used as a minimally-invasive therapeutic method that triggers no permanent or continual inflammatory responses. The results of this study might help the patients to shorten the consumption of immunosuppressive drugs, e.g. corticosteroids, that are prescribed to mitigate the inflammation after ALTP-surgery.

Safety evaluation of the atmospheric low-temperature plasma (ALTP) on the conjunctiva: an animal study and histopathological findings; 6-month follow-up.

BACKGROUND: Plasma medicine is an innovative research field focused on the application of atmospheric-pressure low-temperature plasmas (ALTP) for therapeutic purposes. Considering the potentials of plasma in ophthalmology, in this study, we evaluated the safety of plasma on the conjunctival tissue in animal models for 6 months. METHODS: Twelve adult male New Zealand albino rabbits were divided into four groups. The right eye of each rabbit was chosen for the test and the left eye was considered as the control. Experiments were performed using the Plexr device (GMV, Rocca Priora, RM, Italy). Four plasma spots were applied on the superior part of the conjunctiva (from 10 to 2 o'clock positions) using the continuous mode and a low power level (white handpiece) of the Plexr. For evaluation of the plasma safety, the histopathological changes were assessed 1 week (A), 1 month (B), 3 months (C), and 6 months (D) after the intervention. RESULTS: According to the histopathological findings, a mild decrease in blood vessels and severe stromal edema, as well as a superficial epithelium loss, were observed in group A. No chronic inflammation, scar tissue, deposition, and hemorrhage were found in group B. Epithelialization was confirmed by the histological examinations after 1 month. There was no evidence of atypia or dysplasia after 3 and 6 months. CONCLUSION: In conclusion, there were no persistent histopathological changes on conjunctival tissue after plasma exposure. Then, plasma can be considered as a minimally invasive alternative method for treating some ocular surface disorders.

Safety and Efficacy of Oral Supplementation of Lentil (Lens culinaris Medic) in Dry Eye Patients.

OBJECTIVE: To evaluate the safety and efficacy of dietary lentil capsules in patients suffering from dry eye symptoms. METHODS: A randomized, triple-blind, interventional, placebo-controlled study was done. Sixty patients were randomized in two groups to receive either one capsule containing 500 mg of lentil powder or placebo daily for 3 months. UCVA, tear film breakup time (TBUT), Schirmer's test, tear film osmolarity, and OSDI score were recorded at baseline and 3 months after intervention. Data analysis was performed using IBM SPSS for Windows version 20 (SPSS, Chicago, IL, USA). RESULTS: In the lentil group, at baseline, the mean UCVA (LogMAR), OSDI, TBUT (S), tear film osmolarity (mOsm/L), and Schirmer (mm) scores were 0.104 (0.026), 22.66 (19.40), 10.31 (5.32), 301.07 (15.57), and 8.22 (6.87), respectively. These values were 0.101 (0.026), 20.85 (19.44), 13.04 (7.11), 299.81 (11.60), and 9.87 (10.11). In the placebo group, these values were 0.084 (0.027), 25.35 (20.08), 10.56 (4.95), 299.77 (15.09), and 9.35 (8.06) at baseline and 3 months later were 0.077 (0.027), 23.32 (22.90), 13.62 (6.30), 297.54 (12.08), and 8.64 (9.60), respectively. Three patients (one in the lentil group and two in the placebo group) experienced severe gastrointestinal symptoms. CONCLUSION: Although consumption of 500 mg of lentil is safe, this amount is not sufficient for reduction of dry eye syndrome in 3 months. For more validation, a clinical study with increased dosage of lentil is proposed.

An analytical enrichment-based review of structural genetic studies on keratoconus.

Keratoconus is a progressive bilateral corneal protrusion that leads to irregular astigmatism and impairment of vision. Keratoconus is an etiologically heterogeneous corneal dystrophy and both environmental and genetic factors play a role in its etiopathogenesis. In this analytical review, we have studied all the genes that are structurally associated with keratoconus and have tried to explain the function of each gene and its association with other eye disorders in a concise way. In addition, using gene set enrichment analysis, it was attempted to find the most important impaired metabolic pathways in keratoconus. Several genetic studies have been carried out on keratoconus and several genes have been identified as risk factors involved in the etiology of the disease. In the current study, 16 studies, including nine association studies, five genome-wide association studies, one linkage study, and one meta-analysis, were reviewed and based on the 19 genes found, enrichment was performed and the most important metabolic pathways involved in the disease were identified. The enrichment results indicated that the two pathways, interleukin 1 processing and assembly of collagen fibrils, are significantly associated with the disease. Obviously, the results of this study, in addition to providing information about the genes involved in the disease, can provide an integrated insight into the gene-based etiology of keratoconus and therapeutic opportunities thereof.

Organocatalytic synthesis of enantiopure spiro acenaphthyl-pyrrolizidine/pyrrolidines: justifying the regioselectivity based on a distortion/interaction model.

An efficient organocatalytic [3 + 2] reaction with Schreiner's thiourea organocatalyst for the synthesis of a small library of novel enantiopure stable spiroacenaphthyl-pyrrolidines/pyrrolizidines with high regio- and diastereoselectivity (up to 99%) is described for the first time. These chiral compounds were synthesized by a three-component 1,3-dipolar cycloaddition of (E)-1-(2-oxoacenaphthylen-1(2H)-ylidene) pyrrolidin-1-ium-2-ide as a dipolar and (S)-cinnamoyl/crotonoyl oxazolidinone as a dipolarophile. The absolute configuration of cycloadducts was confirmed by X-ray diffraction analysis. The origin of catalyst reactivity and regio- and stereoselectivity was investigated through DFT calculations. DFT calculations showed that the regioselectivity was controlled by the distortion (deformation) of reactants and Schreiner's thiourea acts as a LUMO-lowering catalyst.

Use of low-vault posterior chamber collagen copolymer phakic intraocular lenses for the correction of myopia: a 3-year follow-up.

BACKGROUND: Phakic intraocular lens (pIOL) models have indicated an association between cataract formation and inadequate vaulting. In this study, the efficacy and safety of low-vault posterior chamber pIOLs in the correction of myopia were examined over 3 years. METHODS: From 316 patients undergoing surgery, 14 eyes with a low vault (≤ 150 µm) were examined from 14 patients. After implanting the Visian Implantable Collamer pIOL for the correction of myopia (moderate and high), the pIOL vault, endothelial cell loss, uncorrected distance visual acuity (UDVA), corrected DVA (CDVA), and detrimental events were examined over 3 years. RESULTS: Based on the findings, the mean spherical equivalent reduced from - 8.15 ± 3.29 before surgery to - 1.02 ± 0.75 diopters 3 years after the surgery. In terms of visual outcomes, the mean UDVA (Log MAR) significantly increased from 0.95 ± 037 to 0.27 ± 0.28, and the mean CDVA also increased from 0.99 ± 0.11 to 0.06 ± 0.08 (P < 0.05). The mean indices of safety and efficacy were respectively 1.075 and 0.748. The eyes lost not more than two visual acuity lines. Based on the findings, 82% achieved 0.80 or better CDVA, while the total endothelial cell loss was 7.96% during 3 years. Overall, vision-threatening conditions were not reported. CONCLUSION: The introduced pIOL can be considered a safe and efficient method for myopia at moderate to high levels.

A Study of Corneal Biomechanics in Delayed-Onset Mustard Gas Keratopathy Compared to Cases With Corneal Scarring and Normal Corneas.

PURPOSE: This study compares the corneal biomechanical properties, as measured with Corvis ST, in three groups of cases with delayed-onset mustard gas keratopathy (DMGK), chronic corneal scarring (CCS), and those with normal corneas. METHODS: Forty-five eyes were enrolled in the DMGK, CCS, and normal groups (15 eyes each). All patients were examined with the slitlamp and Corvis ST. Extracted corneal biomechanical parameters included first and second applanation time (AT1, AT2), first and second applanation length (AL1, AL2), first and second applanation velocity (AV1, AV2), deformation amplitude (DA), highest concavity time, peak distance, highest concavity radius (HCR), central corneal thickness, corrected intraocular pressure (IOP), and noncorrected IOP. RESULTS: The mean age of the participants in the DMGK, CCS, and normal groups was 49.20±5.16, 60.40±22.92, and 44.60±8.32 years, respectively. Comparison between DMGK and CCS groups showed significant differences in AT1, AT2, corrected IOP, and noncorrected IOP. There were also significant differences in AT1, AT2, HCR, DA, and noncorrected IOP between the DMGK and normal groups. None of the parameters were significantly different between the CCS and normal groups. CONCLUSION: There seems to be reduced corneal rigidity and stiffness in cases exposed to mustard gas, which causes the cornea in the DMGK group to become applanated more easily and take longer to rebound compared with the other two groups. Also, IOP was lower than normal in the DMGK group, and thus, misdiagnosis of glaucoma is likely for these patients.

Efficacy of complete rings (MyoRing) in treatment of Keratoconus: a systematic review and meta-analysis.

PURPOSE: We aimed to systematically review the existing evidence and determine the efficacy of MyoRing as a novel method for treatment of keratoconus using meta-analysis. METHODS: Online electronic search of Medline, ISI Web of Science, Embase, Scopus, and Cochrane Library databases was performed with reference lists of relevant articles for pre-post trials published through August 2017. Uncorrected distance visual acuity (UDVA), corrected distance visual acuity (CDVA), sphere, cylinder, spherical equivalent (SE), maximum, minimum, and mean keratometry were considered as the visual acuity outcomes. Weighted mean difference (WMD) with 95% confidence interval was used as pooled estimation of intervention efficacy using random-effects meta-analysis. Heterogeneity was measured with the Cochran Q statistic and quantified with the I2 statistic using Stata software. RESULTS: Of the 47 potentially related studies, 21 eligible studies were included in the meta-analysis. The mean of uncorrected distance visual acuity (UDVA) based on LogMAR in patients with keratoconus had a significant change 3 months after implantation/embedding of the complete ring (WMD =  - 0.73 (CI = - 0.88 to - 0.58), I2 = 79.9%, p < 0.001). Results support a statistically significance improvement in CDVA, SE, sphere, cylinder, and maximum keratometry after surgical intervention. Range of reported safety index, stability, and efficacy index by included studies was 1.7-2.7, 74-100%, and 0.9-1.96, respectively. CONCLUSIONS: MyoRing is an appropriate treatment option for keratoconus. Findings of this meta-analysis demonstrated that main visual outcomes have been improved 3, 6, and 12 months after the implantation of the complete ring (MyoRing).

Identification of a novel RPGRIP1 mutation in an Iranian family with leber congenital amaurosis by exome sequencing.

Leber congenital amaurosis (LCA) is a heterogeneous, early-onset inherited retinal dystrophy, which is associated with severe visual impairment. We aimed to determine the disease-causing variants in Iranian LCA and evaluate the clinical implications. Clinically, a possible LCA disease was found through diagnostic imaging, such as fundus photography, autofluorescence and optical coherence tomography. All affected patients showed typical eye symptoms associated with LCA including narrow arterioles, blindness, pigmentary changes and nystagmus. Target exome sequencing was performed to analyse the proband DNA. A homozygous novel c. 2889delT  (p.P963 fs) mutation in the RPGRIP1 gene was identified, which was likely the deleterious and pathogenic mutation in the proband. Structurally, this mutation lost a retinitis pigmentosa GTPase regulator (RPGR)-interacting domain at the C-terminus which most likely impaired stability in the RPGRIP1 with the distribution of polarised proteins in the cilium connecting process. Sanger sequencing showed complete co-segregation  in this pedigree. This study provides compelling evidence that the c. 2889delT  (p.P963 fs) mutation in the RPGRIP1 gene works as a pathogenic mutation that contributes to the progression of LCA.

Low iris and anterior chamber volume is associated with deepening after laser peripheral iridotomy in primary angle closure suspects.

PURPOSE: To evaluate the association between baseline ocular variables and the widening of the anterior chamber angle by laser peripheral iridotomy (LPI) in primary angle closure suspects (PACS) using a new Fourier-domain swept-source anterior segment optical coherence tomography (FD-ASOCT). METHOD: Sixty-six PACS eyes of 41 individuals were enrolled in this prospective interventional case series. An FD-ASOCT (Casia SS-1000 OCT; Tomey, Nagoya, Japan) was used to measure biometric baseline variables and at 1 month after the LPI. Paired t test was used to compare the difference between pre-and post-LPI measurements. Multivariate regression analysis was used to test for an association between baseline iris thickness and volume, anterior chamber depth and volume, and lens vault with a widening of the angle after an LPI. Changes in trabecular iris space area and angle opening distance after the LPI were main outcome measures. RESULTS: The mean age of participants was 58.6 ± 8.7 years, 68.2% of whom were female. The angle opening distance, recess area, and trabecular iris surface area at 500 µm increased by 48 to 73% (all P < 0.001). Lens vault and iris volume did not change. A low anterior chamber volume and low iris volume were associated with angle greater deepening by LPI. CONCLUSION: Eyes with a shallow anterior chamber and thinner irises are more likely to experience angle opening from an LPI.

Comparison of the MyoRing implantation depth by mechanical dissection using PocketMaker microkeratome versus Melles hook via AS-OCT.

BACKGROUND: This paper seeks to evaluate the depth and outcomes of MyoRing implantation using two mechanical dissection procedures including: PocketMaker microkeratome in opposition to the Melles hook method. METHODS: This retrospective study was carried out on 39 eyes of 38 keratoconus patients (28 male and 10 female) with the mean age of [Formula: see text] years and had undergone MyoRing implantation by the two mentioned methods. The MyoRing was inserted into the corneal pocket which was made manually in 18 eyes (Melles hook group) or with PocketMaker microkeratome in 21 eyes (PocketMaker group). The mean follow up time was [Formula: see text] months with pre-operative and post-operative ophthalmic examination including uncorrected visual acuity (UCVA), best-corrected visual acuity (BCVA), keratometry readings and central corneal thickness measurement. AS-OCT (Casia, SS-1000, Tomey, Nagoya, Japan) imaging was used to measure MyoRing insertion depth, exactly. RESULTS: Pre-operative and post-operative UCVA (LogMAR) mean change for the PocketMaker and Melles hook groups were recorded at 0.75 ± 0.32 and 0.78 ± 0.33, respectively. Similarly, BCVA (LogMAR) mean change were 0.27 ± 0.22 and 0.23 ± 0.22. Mean keratometry (Kmean) change were 6.06 ± 4.18 and 6.56 ± 3.55 respectively. UCVA change (P = 0.767), BCVA change (P = 0.77) and Kmean change (P = 0.693) showed that there was no statistically significant difference between both groups for any parameter. Depth measurements achieved from AS-OCT images showed that there was no statistically significant difference in pocket depth between two methods of MyoRing implantation (P = 0.413). CONCLUSIONS: The results of Myoring implantation outcomes using mechanical dissection via PocketMaker microkeratome as against Melles hook are comparable.

Efficacy of systemic diclofenac sodium on intravitreal concentration.

BACKGROUND: Nonsteroidal anti-inflammatory drugs (NSAIDs), as an alternative, are replacing corticosteroids in ocular inflammatory diseases. Diclofenac has been used mainly topically, and recent focus has been on intravitreal delivery. Both of these methods have been shown to have complications in long-term application. PURPOSE: To assess the efficacy of slow release oral diclofenac sodium on intravitreal concentration in experimental model of chemically injured eyes. METHODS: In an experimental double-masked clinical trial, right eyes of 24 albino rabbits were chemically injured by 1 N NaOH. One hour after chemical injury, 10 cc suspension gavage containing 100 mg slow release diclofenac sodium was administered in all cases. 2, 4, 6, 12, 24, 48 h after gavage, vitreous samples were obtained in all cases. Intravitreal concentration of diclofenac sodium was evaluated in all samples using high-performance liquid chromatography (HPLC) method. RESULTS: Intravitreal diclofenac levels by oral intake were enhanced by the inflammation in all the measurements. In inflamed eyes, diclofenac concentration was ten times more than control eye (2.658 ± 0.344 vs. 0.242 ± 0.0279 and 1.617 ± 0.527 vs. 0.148 ± 0.095; in 2 and 4 h, respectively). After 6 h, diclofenac concentration was statistically different, although it reduced below 1 µg/ml. CONCLUSION: Diclofenac is delivered to the inflamed eye more than healthy eye. It seems that by oral diclofenac consumption, it is possible to make a significant intravitreal concentration.

Simultaneous Presence of Macular Corneal Dystrophy and Retinitis Pigmentosa in Three Members of a Family.

Macular corneal dystrophy (MCD) is an autosomal recessive hereditary disease. In most cases, various mutations in carbohydrate sulfotransferase 6 (CHST6) gene are the main cause of MCD. These mutations lead to a defect in keratan sulfate synthesis. Retinitis pigmentosa (RP) is another eye disorder with nyctalopia as its common symptom. It has been shown that more than 65 genes have been implicated in different forms of RP. Herein, we report on a 9-member family with 2 girls and 5 boys. Both parents, one of the girls and one of the boys had normal eye vision and another boy had keratoconus. Other children (1 girl and 2 boys) suffered from both MCD and RP. Corneal transplantation and medical supplements were used for MCD and RP during the follow-up period, respectively. Based on the family tree, it seems that the inheritance of both diseases is autosomal recessive. Based on our search of databases, there is no report on the simultaneous presence of MCD and RP. To the best of our knowledge, the present article is the first case report on this topic. Molecular genetic investigation is needed to clarify the mechanism of concurrent MCD and RP.

N-Functionalized L-proline anchored MCM-41: a novel organic-inorganic hybrid material for solvent-free aminolysis of styrene oxide under microwave irradiation.

A novel organic-inorganic hybrid material, namely L-prol-N-pMCM-41, was synthesized via two steps by covalently anchoring N-functionalized proline derivative (L-prol-N-pTMS) into the pore channels of MCM-41 silica. The prepared material was characterized by X-ray diffraction (XRD), thermogravimetric analysis (TGA), nitrogen adsorption-desorption isotherm, Brunauer-Emmett-Teller (BET) surface area analysis, and Fourier Transform Infrared spectroscopy (FTIR). The immobilized catalyst demonstrated moderate to high catalytic activity and excellent regioselectivity for the ring opening of styrene oxide with aniline derivatives under microwave irradiation and solvent-free conditions. The catalyst could be rapidly separated from the reaction mixture and reused up to five runs with good conversion and high regioselectivity.