Nephropathic Cystinosis: A Distinct Form of CKD-Mineral and Bone Disorder that Provides Novel Insights into the Regulation of FGF23.

BACKGROUND: The rare lysosomal storage disease nephropathic cystinosis presents with renal Fanconi syndrome that evolves in time to CKD. Although biochemical abnormalities in common causes of CKD-mineral and bone disorder have been defined, it is unknown if persistent phosphate wasting in nephropathic cystinosis is associated with a biochemical mineral pattern distinct from that typically observed in CKD-mineral and bone disorder. METHODS: We assessed and compared determinants of mineral homeostasis in patients with nephropathic cystinosis across the predialysis CKD spectrum to these determinants in age- and CKD stage-matched patients, with causes of CKD other than nephropathic cystinosis. RESULTS: The study included 50 patients with nephropathic cystinosis-related CDK and 97 with CKD from other causes. All major aspects of mineral homeostasis were differentially effected in patients with CKD stemming from nephropathic cystinosis versus other causes. Patients with nephropathic cystinosis had significantly lower percent tubular reabsorption of phosphate and fibroblast growth factor-23 (FGF23) at all CKD stages, and lower blood phosphate in CKD stages 3-5. Linear regression analyses demonstrated lower FGF23 levels in nephropathic cystinosis participants at all CKD stages when corrected for eGFR and age, but not when adjusted for serum phosphate. CONCLUSIONS: Nephropathic cystinosis CKD patients have mineral abnormalities that are distinct from those in CKD stemming from other causes. Persistently increased urinary phosphate excretion maintains serum phosphate levels within the normal range, thus protecting patients with nephropathic cystinosis from elevations of FGF23 during early CKD stages. These findings support the notion that phosphate is a significant driver of increased FGF23 levels in CKD and that mineral abnormalities associated with CKD are likely to vary depending on the underlying renal disease.

Factors associated with intraoperative floppy iris syndrome.

PURPOSE: To identify factors associated with intraoperative floppy iris syndrome (IFIS) in patients undergoing routine phacoemulsification. DESIGN: Comparative case series. PARTICIPANTS: Analysis of 899 eyes of 660 patients undergoing routine cataract surgery. METHODS: All routine cases of cataract extraction with posterior chamber intraocular lens implantation between September 1, 2005, and August 31, 2006, were documented. Pertinent patient information, including age, gender, race, medical history, and current medication use (including tamsulosin [Flomax, Boehringer-Ingelheim, Ingelheim, Germany], other alpha(1)-antagonists, angiotensin antagonists, anticholinergics, cholinergic agonists, muscle relaxants, nitric oxide donors, and saw palmetto), were collected at the time of surgery. A telephone survey was used to determine previous use of tamsulosin, other alpha(1)-antagonists, and saw palmetto. Cases were identified intraoperatively as IFIS or non-IFIS following the triad of criteria developed by Chang and Campbell. MAIN OUTCOME MEASURES: Presence of IFIS associated with medication use and medical history. RESULTS: Analysis showed IFIS in 27 patients (4.1%) representing 33 eyes (3.7%). Tamsulosin use (P<0.001) and history of alpha(1)-antagonist use other than tamsulosin (P = 0.01) were shown to strongly correlate with IFIS in our study. Hypertension was noted to be a significant variable via multivariable generalized estimating equations analysis (P = 0.04) with a prevalence of 75.8% in patients with IFIS versus 56.4% in patients without IFIS. Saw palmetto showed a slight, but statistically insignificant, trend that current use or history of use may be associated with IFIS. There were no cases of posterior capsular rupture or vitreous loss. CONCLUSIONS: Exposure to tamsulosin highly correlates with IFIS. Use of other alpha(1)-antagonists is also associated with IFIS. Several patients in our series were identified to have IFIS with no history of alpha(1)-antagonist use, indicating that other etiologic factors, or a combination of factors, can also elicit this response. Multivariable regression analysis shows no proven relationship between IFIS and individual use of angiotensin antagonists, anticholinergics, cholinergic agonists, muscle relaxants, nitric oxide donors, or saw palmetto, and diabetes or congestive heart failure.

LASIK world literature review: quality of life and patient satisfaction.

PURPOSE: To analyze the patient reported outcome of satisfaction after LASIK surgery. DESIGN: Systematic review. PARTICIPANTS: Patient data from previously reported studies. METHODS: A literature search conducted for the years 1988 to 2008 that included pertinent LASIK surgery information from the review of 2915 retrieved citations. All abstracts from these citations were reviewed and 1581 were deemed to be relevant for review. Complete copies of each of these relevant (1581) articles were obtained, and after thorough analysis each was rated based on the strength of the study design and weight of evidence. A level I rating was assigned to properly conducted, well-designed, randomized clinical trials; a level II rating to well-designed cohort and case-control studies; and a level III rating to case series, case reports, and poorly designed prospective and retrospective studies. Level I and II rated, peer-reviewed articles were entered into a database, and level III articles were eliminated. A total of 309 articles were incorporated into this database, representing level I and level II well-controlled studies of primary LASIK surgery. MAIN OUTCOME MEASURES: Patients' satisfaction rates and factors associated with dissatisfaction. RESULTS: Nineteen of the 309 database articles (6.1%) reported on both patient quality of life and satisfaction and together encompassed a total of 2198 subjects. The procedures from these 19 articles took place between 1995 and 2003. The overall patient satisfaction rate after primary LASIK surgery was 95.4% (2097 of 2198 subjects; range of patient satisfaction for the 19 articles was 87.2%-100%). The patient satisfaction rate after myopic LASIK was 95.3% (1811 of 1901 patients), and after hyperopic LASIK was 96.3% (286 of 297 subjects). CONCLUSIONS: Based on this review, worldwide, an average 95.4% of patients were satisfied with their outcome after LASIK surgery. With 16.3 million procedures performed worldwide, and more than a decade of clinical studies and technological innovation, LASIK surgery should be considered among the most successful elective procedures. LASIK surgery compares more favorably with other elective surgical procedures in terms of generally higher satisfaction rates.

Comparison of the Infiniti vision and the series 20,000 Legacy systems.

PURPOSE: To compare the efficiency of the Infiniti vision system and the Series 20,000 Legacy system phacoemulsification units during routine cataract extraction. METHODS: Thirty-nine eyes of 39 patients were randomized to have their cataract removed using either the Infiniti or the Legacy system, both using the Neosonix handpiece. System settings were standardized. Ultrasound time, amount of balanced salt solution (BSS) used intraoperatively, and postoperative visual acuity at postoperative days 1, 7 and 30 were evaluated. RESULTS: Preoperatively, best corrected visual acuity was significantly worse in the Infiniti group compared to the Legacy group (0.38 +/- 0.23 and 0.21 +/- 0.16, respectively; p = 0.012). The mean phacoemulsification time was 39.6 +/- 22.9 s (range 6.0-102.0) for the Legacy group and 18.3 +/-19.1 s (range 1.0-80.0) for the Infiniti group (p = 0.001). The mean amounts of intraoperative BSS used were 117 +/- 37.7 ml (range 70-195) in the Legacy group and 85.3 +/- 38.9 ml (range 40-200) in the Infiniti group (p = 0.005). No differences in postoperative visual acuity were found. CONCLUSION: The ability to use higher flow rates and vacuum settings with the Infiniti vision system allowed for cataract removal with less phacoemulsification time than when using the Legacy system.

Bronchogenic squamous cell carcinoma presenting as cicatrizing conjunctivitis.

PURPOSE: To report a case of cicatrizing conjunctivitis as the initial manifestation of a bronchogenic squamous cell carcinoma. METHODS: Case report of a 57-year-old patient with bilateral history of ocular pain, redness, and discharge, with no apparent predisposing factors. Examination revealed chemosis, pseudomembrane, and symblepharon formation, and multiple ulcerated pustular and vesicular periocular and perioral lesions literature review. RESULTS: Skin biopsy was compatible with bullous pemphigoid. Two months later he developed respiratory symptoms, and a squamous cell carcinoma was diagnosed. CONCLUSIONS: This case demonstrates cicatrizing conjunctivitis as a possible paraneoplastic syndrome associated with squamous cell lung carcinoma.

Ocular manifestations after primary varicella infection.

PURPOSE: To report a case of ocular manifestations after a primary varicella infection. METHODS: Review of the literature and a case report of a 10-year-old male patient with history of blurry vision and an enlarged pupil 2 months after a varicella infection. Examination revealed uveitis, interstitial keratitis, and internal ophthalmoplegia. RESULTS: The uveitis resolved with topical steroids; the interstitial keratitis resolved with a faint scar, and the internal ophthalmoplegia persisted. CONCLUSION: This case report describes a patient with uncommon ocular manifestations after primary varicella.

Effect of hinge location on corneal sensation and dry eye after laser in situ keratomileusis for myopia.

PURPOSE: To evaluate the effects of a superior or nasal hinge location on corneal sensation and dry eye after laser in situ keratomileusis (LASIK). SETTING: Magill Research Center for Vision Correction, Storm Eye Institute, Medical University of South Carolina, Charleston, South Carolina, USA. METHODS: This prospective randomized masked study included 47 patients having bilateral myopic LASIK surgery. The first eye was randomly assigned to have a nasal or superior hinge flap; the fellow eye had the alternate location. Visual acuity, contrast sensitivity, corneal sensation, basic secretion test, tear film breakup time, conjunctival and corneal staining, and a subjective questionnaire were evaluated preoperatively and postoperatively at 1 week and 1, 3, and 6 months. The Wilcoxon signed rank test and paired t test were used for comparison. RESULTS: Dry eye occurred with the same frequency in both groups. However, eyes with the nasal hinge had significantly better nasal sensation than those with the superior hinge (P<.05) at 1 month. CONCLUSION: Better nasal corneal sensation was found at 1 month in the nasal hinge group but there was no difference in any other parameters measured.

Relationship between cycloplegic and wavefront-derived refraction.

PURPOSE: To compare cycloplegic refractions to preoperative wavefront-derived refractions from three Hartmann-Shack-based wavefront devices. METHODS: Ninety-nine eyes of 55 subjects were evaluated preoperatively using the LADARWave, WaveScan, and Zywave aberrometers. With cycloplegic refraction as the standard, the spherical equivalent refraction, sphere, and cylinder were compared to the wavefront-derived refractions using the three wavefront devices. RESULTS: The LADARWave unit showed the highest correlation between wavefront-derived sphere and mean spherical equivalent refraction values relative to cycloplegic refraction, followed by Zywave and WaveScan. The WaveScan unit exhibited higher variability than the LADARWave or Zywave. Although Alcon Laboratories (LADARWave) and Bausch & Lomb Surgical (Zywave) recommend dilation, VISX specifically recommends against dilation in their WaveScan unit. All devices showed high cylinder error vectors when the cycloplegic cylinder was greater than 1.00 D. CONCLUSION: Although wavefront-derived refractions appeared to correlate well to cycloplegic refractions, further research is required to reduce variability and improve the calculation of cylinder terms.

Refractive surgery survey 2003.

To determine the refractive surgery preferences of ophthalmologists worldwide, questionnaires were sent to 8920 members of the American Society of Cataract and Refractive Surgery. A total of 1174 questionnaires was returned by the deadline. The practice distribution included 30.0% cataract surgeons, 47.3% comprehensive ophthalmologists, 14.4% refractive surgery (RS) specialists, 4.5% corneal/external disease specialists, 1.9% glaucoma specialists, and < 1% retinal/oculoplastics/pediatrics/neurophthalmologists/researchers/retired. Responses were compared with those in the 2001 and 2002 surveys and demonstrate that RS practice patterns continue to evolve.

Comparison of wavefront sensing devices.

Wavefront measurement is an emerging technology that can evaluate low-order and high-order aberrations. This review compiles the different wavefront sensing devices and compares the most popular Hartmann-Shack aberrometers.

A review of the use of ketorolac tromethamine 0.4% in the treatment of post-surgical inflammation following cataract and refractive surgery.

The non-steroidal anti-inflammatory drug (NSAID) ketorolac tromethamine 0.4% ophthalmic solution, a recent reformulation containing 20% less active ingredient that the original formulation, is indicated for the reduction of ocular pain and burning/stinging following corneal refractive surgery. Clinical studies have shown ketorolac tromethamine 0.4% to be as effective as ketorolac tromethamine 0.5% to control inflammation after cataract surgery including prevention of cystoid macular edema (CME). Its efficacy to inhibit miosis during cataract surgery as well as its role in the treatment of dry eye has been reported. The purpose of this paper is to review the use of ketorolac tromethamine 0.4% in the treatment of post-surgical inflammation following cataract and refractive surgery.

Duplicación utero-vaginal completa con himen imperforado y agenesia renal unilateral : a proposito de 1 caso / Complete utero-vaginal duplication with imperforated hymen and unilateral and agenesis: due to one case

Reportamos el caso de una niña de 4 meses de edad que presentó desde el nacimiento un hematometrocolpos por un himen imperforado de una duplicación útero-vaginal completa, asociada a agenesia renal ipilateral. El abombamiento del himen distendido en la vulva fue el único signo de presentación. Se realizó resección humeneal. Se destacan los elementos relevantes del diagnóstico y la simplicidad del tratamiento quirúrgico. Malformaciones uterinas; malformaciones vaginales, agenesia renal