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PubMed2005

Heart rate variability in adult patients with isolated left ventricular noncompaction.

BACKGROUND: Isolated left venticular noncompaction (IVNC) is a rare congenital heart disease charactrized by a pattern of an excessively prominent trabecular meshwork with deep intertrabecular recesses. Heart rate variability (HRV) has been reported to be impaired in various heart diseases, though little is known regarding HRV in adult patients with IVNC. METHODS: We measured spectral components of HRV using fast Fourier transformation of 24-h Holter recordings in 10 adult patients with IVNC, 40 patients with myocardial infarction (MI), 40 patients with hypertrophic cardiomyopathy (HCM) and 40 healthy controls. RESULTS: The low frequency component and the high frequency component of HRV were lower in IVNC patients tahn those in controls (265 +/- 213 ms(2) vs. 469 +/- 195 ms(2), p < 0.01; 80 +/- 51 ms(2) vs. 185 +/- 126 ms(2), p < 0.01). Furthermore, 3 IVNC patients with a previous history of heart failure exhibited more decreased HRV (low frequency, 75 +/- 56 ms(2); high frequency, 39 +/- 18 ms(2)). Contrary, the ratio of low frequency to high frequency component was higher in patients with IVNC than controls (3.5+/-0.5 vs. 3.2 +/- 0.3, p < 0.05). The degree of impaired HRV was severest in MI patients, intermediate in IVNC patients and mildest in HCM patients compared with controls. CONCLUSIONS: HRV is impaired in adult patients with IVCN, especially in patients with a previous history of heart failure, suggesting vagal withdrawal or sympathetic enhancement. HRV in IVNC adults is less impaired than in MI patients, and more impaired than in HCM patients of our cohort.

Adult↗

PubMed2006

Magnetic resonance imaging does not always confirm left ventricular noncompaction.

Left ventricular hypertrabeculation/noncompaction (LVHT) is diagnosed echocardiographically or by other imaging techniques, like cardiac magnetic resonance imaging (CMRI). LVHT was diagnosed echocardiographically in a 48-year-old woman with peripheric embolism and a neuromuscular disorder of unknown etiology. The spongiform trabecular meshwork was located in the ventricular lateral and posterior wall. CMRI failed to visualize LVHT and showed only a homogenously thickened structure. The reason for overlooking LVHT on cardiac magnetic resonance imaging might be poor image quality due to movement and respiration artefacts, the fine meshwork, exceeding the resolution capacity of the technique, and the atypical location of LVHT. CMRI does not confirm LVHT in every case on each occasion. This is most likely due to limitations in image resolution of the technique and movement artefacts of the myocardium.

Echocardiography↗

PubMed2026

TNFα-dependent modulation of WT1-MMP9 regulatory axis links developmental and inflammatory pathways in glaucoma.

Glaucomas are heterogeneous optic neuropathies associated with extracellular matrix dysregulation, abnormal ocular morphogenesis, and inflammatory signaling. Targeted deep sequencing of 586 primary congenital glaucoma (PCG) cases and 1,757 controls identified rare pathogenic variants in multiple genes, including WT1 and MMP9. Notably, WT1 variants clustered within the nuclear export sequence. Further, functional analyses showed that combined wt1-pax6 suppression in zebrafish disrupted ocular morphogenesis, highlighting developmental interdependence. In human trabecular meshwork cells, WT1 acted as a transcriptional repressor of MMP9, while TNF-α signaling triggered nitric oxide-dependent nuclear export of WT1, resulting in delayed MMP9 upregulation. This effect was reversible by inhibiting nuclear export or nitric oxide synthase. A patient-derived mutation in the nuclear-export region of WT1, disrupted this regulatory switch, causing abnormal MMP9 expression. These findings position WT1 as an important regulator linking developmental and inflammatory mechanisms in glaucoma pathogenesis.

anterior segment dysgenesis↗

PubMed2005

Combined clear corneal phacoemulsification and ab interno trabeculectomy: three-year case series.

PURPOSE: To evaluate the efficacy and safety of a novel surgical approach, ab interno trabeculectomy, in a combined procedure (clear corneal phacoemulsification + ab interno trabeculectomy) for the management of concurrent cataract and glaucoma with prognostic factors for filtration failure. SETTING: Department of Ophthalmology, Palmanova Hospital, Udine, Italy. METHODS: A prospective noncomparative case series of 11 eyes of 11 consecutive cataract patients with medically uncontrolled primary open-angle glaucoma or pseudoexfoliation glaucoma was performed. Each patient had phacoemulsification + intraocular lens implantation + ab interno trabeculectomy, which consisted of a gonioscopically controlled ab interno removal of a quadrant (3 clock hours) of the trabecular meshwork. The main outcome measures were intraocular pressure (IOP), the number of antiglaucomatous medications used, and complications. RESULTS: Ten patients completed a 3-year follow-up. One patient had further surgery because of poor IOP control; in this case, the last valid observation was carried forward for IOP calculation. Mean preoperative IOP and IOPs measured 1, 3, 6, 12, 24, and 36 months after surgery were 25.0, 15.8, 15.4, 15.4, 15.2, 15.0, and 15.3 (-38.56%) mm Hg respectively. The number of medications averaged 2.4 before surgery and dropped to 0.8 at the end of follow-up. No major complications occurred during the follow-up period. CONCLUSIONS: The new surgical procedure combining phacoemulsification with ab interno trabeculectomy can induce a clinically relevant decrease in IOP in eyes with cataract and glaucoma with poor prognosis for filtering surgery. However, a randomized controlled clinical trial with a more extended follow-up and a larger series of patients is needed to ascertain the actual effectiveness and safety of this procedure.

Aged↗

PubMed2005

Elevated intraocular pressure in secondary piggyback intraocular lens implantation.

We report 2 cases of postoperative intraocular pressure (IOP) elevation in secondary piggyback intraocular lens (IOL) implantation without history of glaucoma or ocular hypertension. A 74-year-old woman with myopic pseudophakia and a 68-year-old man with hyperopic pseudophakia received secondary piggyback AcrySof IOL implantation in their left eyes. In both patients, the left IOP gradually increased and sustained around 30 mm Hg for about 1 year. In the first, IOP continued elevating despite topical and systemic medications. There was an episode of pupillary block in the second. Gonioscopically, heavier trabecular meshwork pigmentation in their left eyes was observed. Because of this, the 2 IOLs implanted were removed and replaced by an adequate IOL and trabeculotomy was performed in the former. The AcrySof IOL has a truncated optic edge, which increases the risk for chafing the iris, resulting in pigment dispersion syndrome; thus, it would be a poor choice for a sulcus-placed piggyback implantation.

Acrylic Resins↗

PubMed2006

The role of oxidative stress in glaucoma.

DNA damage is related to a variety of degenerative diseases such as cancer, atherosclerosis and neurodegenerative diseases, depending on the tissue affected. Increasing evidence indicates that reactive oxygen species (ROS) play a key role in the pathogenesis of primary open angle glaucoma (POAG), the main cause of irreversible blindness worldwide. Oxidative DNA damage is significantly increased in the ocular epithelium regulating aqueous humor outflow, i.e., the trabecular meshwork (TM), of glaucomatous patients compared to controls. The pathogenic role of ROS in glaucoma is supported by various experimental findings, including (a) resistance to aqueous humor outflow is increased by hydrogen peroxide by inducing TM degeneration; (b) TM possesses remarkable antioxidant activities, mainly related to superoxide dismutase-catalase and glutathione pathways that are altered in glaucoma patients; and (c) intraocular-pressure increase and severity of visual-field defects in glaucoma patients parallel the amount of oxidative DNA damage affecting TM. Vascular alterations, which are often associated with glaucoma, could contribute to the generation of oxidative damage. Oxidative stress, occurring not only in TM but also in retinal cells, appears to be involved in the neuronal cell death affecting the optic nerve in POAG. The highlighting of the pathogenic role of ROS in POAG has implications for the prevention of this disease as indicated by the growing number of studies using genetic analyses to identify susceptible individuals and of clinical trials testing the efficacy of antioxidant drugs for POAG management.

Glaucoma↗

PubMed2005

Nonpenetrating glaucoma surgery.

Nonpenetrating glaucoma surgery continues to evolve and improve. New technical refinements and increased experience among its practitioners have led to improved efficacy and longer duration of filter survival. These refinements include the use of intraoperative and postoperative antimetabolites, adjunctive space-maintaining devices, and postoperative yttrium-aluminum-garnet laser goniopuncture. Deep sclerectomy and viscocanalostomy vary significantly in their surgical approaches and mechanisms of aqueous outflow. An understanding of the microanatomy of the aqueous outflow structures is necessary to gain surgical access to Schlemm's canal and to augment aqueous outflow surgically through the trabecular meshwork. Indications and contraindications for nonpenetrating glaucoma surgery are discussed.

Glaucoma↗

PubMed2006

Late-onset retinoblastoma in a well-functioning fellow eye.

PURPOSE: To describe the documented growth, clinical course, and histopathology of retinoblastomas in an untreated and otherwise normal right eye of a 27-year-old white male with a g.153211T>A (p.Tyr606X) mutation in the retinoblastoma 1 gene, whose left eye was enucleated at age 2 years for 2 retinoblastomas. DESIGN AND PARTICIPANTS: Retrospective interventional case report. INTERVENTIONS: Over the years, the right eye was irradiated twice and underwent trans-pars plana vitrectomy, transscleral cryocoagulation, argon laser photocoagulation of tumors and their feeder vessels, extracapsular cataract extraction with posterior chamber lens implantation, and neodymium:yttrium-aluminum-garnet laser treatment of after-cataract in the form of Elschnig's pearls. Finally, the patient received combination chemotherapy with etoposide, methotrexate, actinomycin D, cisplatin, and vincristine. RESULTS: The eye finally had to be removed 12 years later due to tumor recurrences and seeding, pseudohypopyon, and elevated intraocular pressure. Histopathology showed microcellular retinoblastoma cells in the anterior chamber angle and trabecular meshwork without subconjunctival extension and in the nasal ciliary body, pars plana, internal limiting membrane, and optic nerve head anterior to the cribriform plate. The patient is without local or systemic recurrences at age 50, 11 years after the last eye was enucleated. CONCLUSIONS: This report shows that retinoblastoma patients may have tumor growth in their fellow eye 25 years after the first eye and also that Elschnig's after-cataract pearls still can arise after irradiation of a lens with 45 Gy.

Adult↗

PubMed2006

Detection of primary angle closure using anterior segment optical coherence tomography in Asian eyes.

OBJECTIVE: To evaluate noncontact anterior segment optical coherence technology (AS-OCT) as a qualitative method of imaging the anterior chamber angle and to determine its ability to detect primary angle closure when compared with gonioscopy in Asian subjects. DESIGN: Prospective observational case series. PARTICIPANTS: Two hundred three subjects were recruited from glaucoma clinics in Singapore with diagnoses of primary angle closure, primary open-angle glaucoma, ocular hypertension, or cataract. Both eyes (if eligible) of each patient were included in the study. Exclusion criteria were pseudophakia or previous glaucoma surgery. METHODS: Images of the nasal, temporal, and inferior angles were obtained with AS-OCT in dark and then light conditions. Gonioscopic angle width was graded using the Spaeth classification for each quadrant in low lighting conditions. MAIN OUTCOME MEASURES: Angle closure was defined by AS-OCT as contact between the peripheral iris and angle wall anterior to the scleral spur and by gonioscopy as a Spaeth grade of 0 degree (posterior trabecular meshwork not visible). Comparison of the 2 methods in detecting angle closure was done by eye and by individual. Sensitivities and specificities of AS-OCT were calculated using gonioscopy as the reference standard. RESULTS: Complete data were available for 342 eyes of 200 patients. Of the patients, 70.9% had a clinical diagnosis of treated or untreated primary angle closure. Angle closure in > or =1 quadrants was detected by AS-OCT in 142 (71%) patients (228 [66.7%] eyes) and by gonioscopy in 99 (49.5%) patients (152 [44.4%] eyes). The inferior angle was closed more frequently than the nasal or temporal quadrants using both AS-OCT and gonioscopy. When performed under dark conditions, AS-OCT identified 98% of those subjects found to have angle closure on gonioscopy (95% confidence interval [CI], 92.2-99.6) and led to the characterization of 44.6% of those found to have open angles on gonioscopy to have angle closure as well. With gonioscopy as the reference standard, specificity of AS-OCT in the dark was 55.4% (95% CI, 45.2-65.2) for detecting individuals with angle closure. CONCLUSION: Anterior segment OCT is a rapid noncontact method of imaging angle structures. It is highly sensitive in detecting angle closure when compared with gonioscopy. More persons are found to have closed angles with AS-OCT than with gonioscopy.

Adult↗

PubMed2006

Laser peripheral iridotomy in primary angle-closure suspects: biometric and gonioscopic outcomes: the Liwan Eye Study.

PURPOSE: To assess the immediate effect of laser peripheral iridotomy (LPI) and mechanisms of angle closure in a population-based study of primary angle closure (PAC) suspects. DESIGN: Prospective interventional study. PARTICIPANTS: People identified as PAC suspects aged 50 to 79 years from a population-based survey in Guangzhou, China. INTERVENTION: Laser peripheral iridotomy was performed in 1 randomly selected eye. Examinations were carried out before and 2 weeks after the intervention. MAIN OUTCOME MEASURES: Intraocular pressure (IOP), ultrasound biometry, optical pachymetry, and gonioscopy. RESULTS: A total of 72 people with bilateral suspected PAC participated in the study. Mean IOP decreased by 3 mmHg (P<0.001), but axial anterior chamber depth did not change significantly (P = 0.784) after LPI. Median limbal anterior chamber depth increased from 15% to 25% of peripheral corneal thickness (P<0.001, Wilcoxon signed-rank test). Median iridotrabecular angle width increased from 0 degrees to 10 degrees in the superior quadrant and from 10 degrees to 30 degrees in the inferior quadrant (P<0.001). Nevertheless, 14 eyes (19.4%) still had 3 or more quadrants in which the posterior (usually pigmented) trabecular meshwork could not be seen after laser iridotomy. CONCLUSIONS: This study confirms that LPI results in a significant increase in the angle width in Chinese people with narrow angles. However, one fifth of eyes had residual angle closure after LPI. Although this report confirms that iridotomy widens the anterior chamber angle in most PAC suspects, long-term prospective studies with a larger sample size are required to determine if the risks of PAC glaucoma and other related pathologic sequelae are reduced after prophylactic LPI and to investigate the risk-to-benefit ratio before recommending widespread use of prophylactic LPI in this population.

Aged↗

PubMed1991

Glaucoma. Classifications, treatment options, patient care.

Advances in pharmacology, cell biology, and laser and fiberoptic technology are providing many opportunities in glaucoma research. As researchers increasingly understand the physiology of the eye, they develop new therapies. A wide variety of new drugs including dopaminergics, calcium channel blockers, and prostaglandins are under investigation for glaucoma treatment. The THC:YAG laser is being developed to perform thermal sclerotomy and may eliminate much of the risk associated with standard filtering surgery. Biologic, anatomic, and biochemical studies of the trabecular meshwork are leading to greater understanding of aqueous humor formation, aqueous humor outflow in the normal eye, and aqueous humor flow resistance in the glaucomatous eye--knowledge that eventually could provide preventive measures or eliminate glaucoma entirely.

Drug Therapy↗

PubMed2001

Pigmentary dispersion syndrome induced by a posterior chamber phakic refractive lens.

PURPOSE: To report a case of bilateral pigmentary dispersion syndrome (PDS) induced by the implantation of posterior chamber phakic refractive lenses (PRLs). METHOD: Case report. RESULTS: Following bilateral implantation of posterior chamber phakic refractive lenses in 38-year-old woman, unilateral elevated intraocular pressure (IOP) developed within months that was attributable to pigment dispersion within the anterior chamber. Findings consistent with PDS included bilateral transillumination defects of the iris in areas contacting the anterior surface of the PRLs, pigment deposits on the anterior surface of the PRLs, Krukenberg spindles, and bilateral dense pigmentation of the trabecular meshwork. The patient's IOP is presently under control and she has not developed glaucomatous damage. CONCLUSIONS: The development of PDS in this case demonstrates that posterior chamber phakic refractive lenses can make contact with the posterior iris and induce pigment dispersion syndrome in susceptible patients.

Adult↗

PubMed2003

What is the risk of developing pigmentary glaucoma from pigment dispersion syndrome?

PURPOSE: To determine the probability of converting from pigment dispersion syndrome to pigmentary glaucoma. DESIGN: Retrospective community-based study of all newly diagnosed cases of pigment dispersion syndrome or pigmentary glaucoma. METHOD: Subjects were patients newly diagnosed with pigment dispersion syndrome or pigmentary glaucoma from 1976 to 1999 in Olmsted County, Minnesota. Criteria for pigment dispersion syndrome were two of three signs: midperipheral, radial iris transillumination defects; Krukenberg spindle; heavy trabecular meshwork pigmentation. Criteria for pigmentary glaucoma were pigment dispersion syndrome and two of three findings: intraocular pressure (IOP) greater than 21 mm, optic nerve damage, or visual field loss. Kaplan-Meier survival curves were used to determine the probability of conversion to pigmentary glaucoma. RESULTS: A total of 113 patients were newly diagnosed with pigment dispersion syndrome over the 24-year period. Of these, 9 persons developed pigmentary glaucoma or elevated IOP requiring therapy. The probability of converting to pigmentary glaucoma was 10% at 5 years and 15% at 15 years. An additional 23 patients were found to have pigmentary glaucoma at their initial examination. The mean age at diagnosis of pigmentary glaucoma was 42 +/- 12 years; 78% of patients were male, whereas 58% of patients with pigmentary dispersion syndrome glaucoma were male. The most significant risk factor for conversion to pigmentary glaucoma was an IOP greater than 21 mm Hg at initial examination, whereas age, refractive error, and family history of glaucoma were not correlated with conversion. CONCLUSIONS: The risk of developing pigmentary glaucoma from pigment dispersion syndrome was 10% at 5 years and 15% at 15 years. Young, myopic men were most likely to have pigmentary glaucoma. An IOP greater than 21 mm Hg at initial examination was associated with an increased risk of conversion.

Adolescent↗

PubMed2003

Long anterior zonules and pigment dispersion.

PURPOSE: To describe pigment dispersion associated with long anterior zonules. DESIGN: Multicenter observational case series. METHODS: Fifteen patients, seven of whom were treated for glaucoma or ocular hypertension, were identified with long anterior zonules and pigment dispersion. Transmission electron microscopy was performed on one anterior capsule specimen. RESULTS: All patients had anterior zonules that inserted centrally on the lens capsule. Signs of pigment dispersion included corneal endothelial pigmentation, loss of the pupillary ruff, and variable trabecular meshwork pigmentation. Ultrasound biomicroscopy verified the lack of posterior iris insertion and concavity. There was no exfoliation material. Transmission electron microscopy showed zonular lamellae with adherent pigment granules, and no exfoliation material. CONCLUSIONS: Long anterior zonules inserted onto the central lens capsule may cause mechanical disruption of the pigment epithelium at the pupillary ruff and central iris leading to pigment dispersion.

Adult↗

PubMed2003

Nonpenetrating filtering surgery and goniopuncture (staged trabeculectomy) for episcleral venous pressure glaucoma.

PURPOSE: To report long-term (>5 years) success of non-penetrating filtering surgery in glaucoma due to elevated episcleral venous pressure. DESIGN: Observational case report. METHODS: A 25-year-old woman developed severe glaucoma due to elevated episcleral venous pressure. Nonpenetrating filtering surgery (opening of the Schlemm canal and resection of corneal stroma overlying the trabecular meshwork and Descemet membrane) was performed with mitomycin. Three months later, the meshwork was perforated ab interno by neodymium:yttrium-aluminum-garnet laser via goniolens (goniopuncture). RESULTS: There were no complications, and no glaucoma medications were used postoperatively. Intraocular pressure ranged from 10 mm Hg to 18 mm Hg during the first 4 months and thereafter between 6 mm Hg and 12 mm Hg. CONCLUSIONS: Glaucoma due to elevated episcleral venous pressure, an entity associated with significant trabeculectomy risks, can be safely and successfully treated with non-penetrating filtering surgery followed by goniopuncture (staged trabeculectomy).

Adult↗

PubMed1995

Aggressive nevus of the iris with secondary glaucoma in a child.

PURPOSE/METHODS: We studied a case of an aggressive iris nevus that caused secondary glaucoma in a child. RESULTS/CONCLUSION: Unlike most iris tumors, this tumor grew from the superior aspect of the iris. The nevus was also unusual because it invaded the trabecular meshwork and caused secondary glaucoma.

Adolescent↗

PubMed1994

Endogenous Fusarium endophthalmitis in a patient with acute lymphocytic leukemia.

Endogenous fungal endophalmitis is an uncommon complication of systemic mycosis. Only a few cases involving Fusarium have been reported, most with unfavorable visual outcomes. We examined a 31-year-old woman with acute lymphocytic leukemia who developed sudden visual loss in her right eye. A dense, white placoid infiltrate was present in the right macula extending into the vitreous. An iris nodule and hypopyon were present in the left eye. A vitreous aspirate of the right eye was positive for Fusarium species. The patient progressively lost vision despite amphotericin B and 5-fluorocytosine therapy. She died from bronchopneumonia, fungemia, and multisystem failure. Histopathologic study disclosed a panophthalmitis with Fusarium organisms invading all the ocular coats in the right eye. Leukemic infiltrates were present in the left iris, anterior chamber, and trabecular meshwork. The ocular destructiveness of Fusarium may be caused by marked mycotic vascular invasion and occlusion with consequent infarction and necrosis of ocular tissues.

Adult↗

PubMed1989

Low intraocular pressure and postural changes in intraocular pressure in patients with Hansen's disease.

We found intraocular pressures of less than 7 mm Hg in 11 of 72 patients (15%) with early Hansen's disease. Furthermore, significant postural changes in intraocular pressure (greater than 30%) occurred in 43 of 72 patients (60%). This loss of intraocular pressure homeostasis, which results from reduced aqueous humor production or increased uveoscleral outflow with reduced local adrenergic control in the ciliary body-iris-trabecular meshwork region, suggests that early autonomic neuropathy of the eye may occur in Hansen's disease. The only predictive value for significant postural change in intraocular pressure was the presence of immunologically unstable disease.

Homeostasis↗