idiopathic macular telangiectasia: Topics by WorldWideScience.org

A Case Report of a Patient with Hereditary Hemorrhagic Telangiectasia Treated Successively with Thalidomide and Bevacizumab

Hereditary hemorrhagic telangiectasia (Osler-Rendu-Weber disease) is a genetic disorder with autosomal dominance, variable penetrance, and an estimated prevalence of 1/10,000 inhabitants in France. Diagnosis is based on clinical criteria including epistaxis, telangiectasia, visceral manifestations, and familial occurrence. Pulmonary arteriovenous malformations, present in 15-33% of patients, are its primary visceral complications. The disease may be revealed by infectious and ischemic neurological manifestations due to paradoxical embolism. The high frequency of neurologic complications even in asymptomatic patients justifies systematic screening for pulmonary arteriovenous malformations. Treatment of these malformations is based on percutaneous transcatheter coil embolization of the feeding artery. Pulmonary arterial hypertension is rare in this disease. It may be due to systemic arteriovenous shunting in the liver, which increases cardiac ...

2005-11-01

Macular Pigment Density Evolution in Unilateral Wet AMD Versus Non AMD Patients With or Without Lutein and Zeaxanthine Supplementation

Hereditary hemorrhagic telangiectasia is characterized by mucocutaneous and visceral telangiectasia and involves several organs with vascular malformations. It is an autosomal dominant disease and is...Full Text Available

Genetics and molecular pathology of Stargardt-like macular degeneration

Exudative Age-related Macular Degeneration

2011-08-03

Nonchylous idiopathic pleural effusion in the newborn

Stargardt-like macular degeneration (STGD3) is an early onset, autosomal dominant macular degeneration. STGD3 is characterized by a progressive pathology, the loss of central vision, atrophy...Full Text Available

2010-05-01

Idiopathic (primary) achalasia

Congenital isolated pleural effusion is a rare cause of respiratory distress in neonates. It is usually chylous. Herein, we report a rare case of nonchylous congenital idiopathic pleural effusion.

2011-01-01

Ultrasonography and color Doppler of proximal gluteal enthesitis in juvenile idiopathic arthritis: a descriptive study

Idiopathic achalasia is a primary esophageal motor disorder characterized by esophageal aperistalsis and abnormal lower esophageal sphincter (LES) relaxation in response to deglutition. It is a rare...Full Text Available

The use of etoricoxib to treat an idiopathic stabbing headache: a case report

BackgroundThe presence of enthesitis (insertional inflammation) in patients with juvenile idiopathic arthritis (JIA) is difficult to establish clinically and may influence classification...Full Text Available

Immature cell populations and an erythropoiesis gene-expression signature in systemic juvenile idiopathic arthritis: implications for pathogenesis

According to the International Headache Society, idiopathic stabbing headache (ISH), an indomethacin-responsive headache syndrome, is a paroxysmal disorder of short duration manifested as head pain...Full Text Available

Idiopathic venous thromboembolism and thrombophilia

IntroductionPrevious observations suggest that active systemic juvenile idiopathic arthritis (sJIA) is associated with a prominent erythropoiesis gene-expression signature. The aim...Full Text Available

2010-01-01

Combined photodynamic therapy and intravitreal bevacizumab for idiopathic polypoidal choroidal vasculopathy: one-year follow-up

During the past decade idiopathic venous thromboembolism has become a separate entity, a chronic illness which has required prolonged anticoagulation and other prevention strategies to avoid recurrences....Full Text Available

2011-02-15

Abnormalities of GATA-1 in Megakaryocytes from Patients with Idiopathic Myelofibrosis

ObjectiveTo report the efficacy and safety of combined photodynamic therapy (PDT) and intravitreal bevacizumab (IVB) injection in the treatment of idiopathic polypoidal choroidal...Full Text Available

2010-01-01

Use of antivascular endothelial growth factor for diabetic macular edema

The abnormal megakaryocytopoiesis associated with idiopathic myelofibrosis (IM) plays a role in its pathogenesis. Because mice with defective expression of transcription factor GATA-1 (GATA-1^low...Full Text Available

2005-09-01

Prevalence of Early and Late Age-Related Macular Degeneration in India: The INDEYE Study

BackgroundDiabetic macular edema (DME) is one of the manifestations of diabetic retinopathy leading to loss of central vision and visual acuity. It manifests itself with swelling...Full Text Available

2010-01-01

Macular abnormalities in the reattached retina.

Purpose.To estimate the prevalence of early and late age-related macular degeneration (AMD) in India.Methods.Of 7518 people aged 60 years and older...Full Text Available

2010-02-01

A comparative study between intravitreal triamcinolone and bevacizumab for macular edema due to central retinal vein occlusion with poor vision

Sixty-six patients in whom the macula was detached before surgery were observed for at least 1 year after retinal reattachment. Macular abnormalities were recognised clinically in most patients with...Full Text Available

1978-09-01

Consistency of ocular coherence tomography fast macular thickness mapping in diabetic diffuse macular edema

Aim:To compare the effect of intravitreal bevacizumab and triamcinolone in patients with macular edema after central retinal vein occlusion (CRVO), presenting with poor...Full Text Available

2011-03-01

Foveo-macular retinitis, solar retinopathy, and trauma.

Objectives: To investigate optical coherence tomography consistency on foveal thickness, foveal volume, and macular volume measurements in patients with and without diffuse diabetic macular edema. Introduction: Optical coherence tomography represents an objective technique that provides cross-sectional tomographs of retinal structure in vivo. However, it is expected that poor fixation ability, as seen in diabetic macular edema, could alter its results. Several authors have discussed the reproducibility of optical coherence tomography, but only a few have addressed the topic with respect to diabetic maculopathy. Methods: The study recruited diabetic patients without clinically evident retinopathy (control group) and with diffuse macular edema (case group). Only one eye of each patient was evaluated. Five consecutive fast macular scans were taken using Ocular Coherence Tomography 3; ...

2007-07-01

Aphakic macular oedema following prosthetic lens implantation.

Three patients are described with foveal lesions resembling minute holes following trauma. The similarity of the lesions to foveomacular retinitis and solar retinopathy suggests that all these conditions...Full Text Available

1978-08-01

Pulmonary vein ablation for idiopathic atrial fibrillation: six month outcome of first procedure in 100 consecutive patients

Fluorescein angiography of the iris was performed on patients with plastic lens implants with cystoid oedema of the macula, and the nature of the vascular changes was compared with controls provided...Full Text Available

1977-05-01

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LOC387715/HTRA1 gene polymorphisms and susceptibility to age-related macular degeneration: A HuGE review and meta-analysis

Objectives: To report six month outcome in patients undergoing their first pulmonary vein ablation procedure for idiopathic atrial fibrillation (AF) at a “non-pioneering”...Full Text Available

2005-01-01

The development of in vitro mutagenicity testing systems using T-lymphocytes

PurposeTo examine the association of age-related macular degeneration (AMD) with HtrA serine peptidase 1 (HTRA1) gene rs11200638 G→A polymorphism and LOC387715/...Full Text Available

Comparison of the argon tunable dye laser with the flashlamp pulsed dye laser in treatment of facial telangiectasia

This annual report describes progress in studies on hprt mutations induced by radon or Indium 111 along with the corresponding mutation frequency, cloning and molecular spectra in human T-lymphocytes. Parallel studies on the mutation susceptibility between individuals is being investigated by hprt mutation studies on ataxia telangiectasia and xeroderma pigmentosum.

1993-05-01

Positional Stretching of the Coracohumeral Ligament on a Patient with Adhesive Capsulitis: A Case Report

A prospective, side-by-side comparison study of two different lasers for the treatment of solar- induced telangiectasia was carried out in 14 patients at the Beckman Laser Institute and Medical Clinic. The argon tunable dye laser (Coherent, Palo Alto, Calif.) was used in the method modified from Orenstein and Nelson to completely treat discrete telangiectasias on one cheek. Specifically, the argon tunable dye laser (ATDL) was set at 0.7 - 0.8 watts, 585 nm wavelength, shutter-pulsed at 0.1 second duration with a spot size of 0.1 mm, and individual vessels were 'traced out' with 4X loupe magnification. Each patient's opposite cheek was then treated in the standard fashion with the flashlamp pulsed dye laser (Candela, Natick, Mass.) using a technique similar to Polla's et al. Specifically, the flashlamp pulsed dye laser (FPDL) was set at 585 nm wavelength, pulsed mode of 450 microseconds pulse duration, spot size of 5 mm, overlapping 10 - 20%, ...

1992-06-01

Paradoxical function for the receptor for advanced glycation end products in mouse models of pulmonary fibrosis

Idiopathic frozen shoulder is a common medical diagnosis for patients seeking physical therapy. Radiographic and surgical evidence exists that describes the coracohumeral ligament (CHL) as a major contributor...Full Text Available

2009-01-01

Increased wind-up to heat pain in women with a childhood history of functional abdominal pain

Idiopathic pulmonary fibrosis (IPF) is a progressive disease with poor survival. The identification of therapeutic targets is essential to improving outcomes. Previous studies found that expression...Full Text Available

2011-03-31

Disability and Psychologic Distress in Patients with Nonspecific and Specific Arm Pain

Idiopathic or functional abdominal pain (FAP) is common in school-age children and typically reflects a functional gastrointestinal disorder (FGID). FGIDs in adults have been distinguished by...Full Text Available

2011-04-01

Visible light and risk of age-related macular degeneration.

Psychological illness influences the experience and expression of pain and disability. We tested three null hypotheses: (1) patients with nonspecific pain (medically unexplained and idiopathic) and...Full Text Available

2008-11-01

Mitochondrial function and redox control in the aging eye: Role of MsrA and other repair systems in cataract and macular degenerations

Sunlight exposure has been suggested as a cause of AMD. To examine this, we collected detailed histories of ocular sun exposure in 838 watermen who work on the Chesapeake Bay. The presence and severity...Full Text Available

1990-01-01

Macular and retinal nerve fiber layer thickness in Japanese measured by Stratus optical coherence tomography

Oxidative stress occurs when the level of prooxidants exceeds the level of antioxidants in cells resulting in oxidation of cellular components and consequent loss of cellular function. Oxidative...Full Text Available

2009-02-01

Inhibition of the visual cycle by A2E through direct interaction with RPE65 and implications in Stargardt disease

The purpose of this study was to determine the thickness of the macula and the retinal nerve fiber layer (RNFL) in Japanese subjects by Stratus optical coherence tomography (OCT), and to compare the...Full Text Available

2007-06-01

Experimental Approaches to the Study of A2E, a Bisretinoid Lipofuscin Chromophore of Retinal Pigment Epithelium

Stargardt disease (STGD) is the major form of inherited juvenile macular degeneration. Pyridinium bis-retinoid A2E is a major component of lipofuscin which accumulates in retinal pigment epithelium...Full Text Available

2010-10-12

Effects of Age on Optical Coherence Tomography Measurements of Healthy Retinal Nerve Fiber Layer, Macula and Optic Nerve Head

Bisretinoid lipofuscin compounds that accumulate in retinal pigment epithelial (RPE) cells are implicated in the pathogenesis of some forms of macular degeneration. In the development of approaches...Full Text Available

2010-01-01

Clinical and tomographic aspects of macular microholes; Aspectos clinicos e tomograficos dos microburacos maculares

PurposeTo determine the effects of age on global and sectoral peripapillary retinal nerve fiber layer (RNFL), macular thicknesses and optic nerve head (ONH) parameters...Full Text Available

2009-06-01

CLINICAL VALUE, NORMATIVE RETINAL SENSITIVITY VALUES, AND INTRASESSION REPEATABILITY USING A COMBINED SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY/SCANNING LASER OPHTHALMOSCOPE MICROPERIMETER

Purpose: To describe the clinical aspects and evaluate optical coherence tomography of macular microholes. Methods: Seven patients were assessed (8 eyes) with microholes of the macula. All patients underwent complete eye examination, fundus photography, fluorescent angiography and OCT-3 imaging. Results: Ages ranged from 26 to 69 years. Six patients were female (85.7%) and five of them had microhole in the right eye. The presenting symptom was decrease in visual acuity (71.3%) and central scotoma in (14.3%). Five eyes (71.4%) had no defects shown by fluorescent angiography. A defect in the outer retina was demonstrated in all eyes on optical coherence tomography. The lesions were nonprogressive. Conclusion: Macular microholes are small lamellar defects in the outer retina. The condition is nonprogressive, generally unilateral and compatible with good visual acuity. Fundus biomicroscopy associated with an optical coherence tomography are the ...

2009-07-01

Reticulate eruptions. Part 1: Vascular networks and physiology

PurposeTo establish normative values for macular light sensitivity and to determine the intrasession fluctuation of perimetric responses using the OPKO/OTI microperimeter.Full Text Available

2011-02-01

British Library Electronic Table of Contents (United Kingdom)

Abstract Reticulate pattern is one of the most important dermatological signs of a pathological process involving the superficial vascular networks. Vascular malformations, such as cutis marmorata congenita telangiectasia and benign forms of livedo reticularis, and sinister conditions, such as meningococcal meningitis or Sneddon's syndrome, can all present with a reticulate pattern. The clinical presentation and morphology is determined by the nature and extent of the underlying pathology and the involvement of a particular vascular network. This review has been divided into four instalments. In the present paper, we discuss the anatomy and physiology of the complex network of vascular structures that support the function of the skin and subcutis.

2011-01-01

Pseudophakic and aphakic retinal detachment mimicking cystoid macular edema.

Pirfenidone in idiopathic pulmonary fibrosis: the CAPACITY program

Seventeen eyes of patients who were being followed by their ophthalmologists for aphakic (8 eyes) or pseudophakic (9 eyes) cystoid macular edema (CME) were examined on referral. All eyes showed retinal detachment (RD), and eight eyes had CME with RD. Most RDs were inferior and shallow. Proliferative vitreoretinopathy (PVR) was noted in 15 eyes (88.2%). There was history of vitreous loss during the last intraocular surgery in 88.2% eyes. Visual acuity ranged from 20/40 to hand movements. Fundus view was hazy in most eyes, and indirect ophthalmoscopy was most useful in detecting RD. Retinal reattachment with scleral buckling and/or vitrectomy was achieved in 16 eyes (94.1%). Postoperative visual recovery was jeopardized due to longstanding macular detachment of greater than 1 month in 11 eyes (64.7%). Eight eyes (47%) achieved 20/50 vision whereas 25% did not improve to better than 20/400. Factors leading to failure in making the correct ...

1987-07-01

British Library Electronic Table of Contents (United Kingdom)

Idiopathic pulmonary fibrosis is the most lethal form of diffuse lung fibrosis, killing approximately half of those affected within 2-3 years of diagnosis. Until recently, no therapies had been shown to have an impact on disease progression. The Clinical Studies Assessing Pirfenidone (Esbriet) in IPF: Research of Efficacy and Safety Outcomes (CAPACITY) program comprised two almost identical double-blind placebo-controlled studies assessing the effects of pirfenidone on change in forced vital capacity, the primary end point, over a 72-week period. One of these studies was positive, matching in magnitude the benefit seen in two previous positive Japanese studies. The other study did not meet its primary end point but positive trends were consistent in this and a number of secondary end point...

2011-01-01

Comparison of 25- and 23-gauge sutureless microincision vitrectomy surgery in the treatment of various vitreoretinal diseases

British Library Electronic Table of Contents (United Kingdom)

PurposeTo compare the effectiveness and safety of 25- and 23-gauge sutureless microincision vitrectomy surgery (MIVS) in the management of various vitreoretinal diseases.MethodsEighty-five consecutive patients undergoing sutureless vitrectomy during January to April 2008 were randomized to either 25- or 23-gauge MIVS. Data collected prospectively included best-corrected visual acuity (BCVA), operation time, postoperative visual recovery, postoperative anterior segment change, and complications.ResultsThe most common indications for MIVS were macular hole, macular pucker, vitreous haemorrhage, and diabetic traction retinal detachment. Mean operation times of the 25-gauge (n=38) and 23-gauge groups (n=47) were 33.68 and 34.47?min, respectively (P=0.942). Mean BCVA improved significantl...

2010-01-01

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Preoperative factors predictive of postoperative decimal visual acuity ? 1.0 following surgical treatment for idiopathic epiretinal membrane

An evaluation of the utility of routine laboratory monitoring of juvenile idiopathic arthritis (JIA) patients using non-steroidal anti-inflammatory drugs (NSAIDs): a retrospective review

Purpose:To report the preoperative best-corrected visual acuity (BCVA) and foveal thickness (FT) values that lead to a postoperative decimal BCVA of ≥1.0 after surgical removal...Full Text Available

2011-01-01

Use of sodium chromate Cr/sub 51/ in diagnosing childhood idiopathic pulmonary hemosiderosis

BackgroundNo consensus evidence-based guidelines for the routine laboratory monitoring of children with JIA receiving non-steroidal anti-inflammatory drugs (NSAIDs) exist. The purpose...Full Text Available

Visual recovery after vitrectomy for macular hole using 25-gauge instruments

The diagnosis of idiopathic pulmonary hemosiderosis (IPH) may be elusive. A 6-year-old boy had microcytic hypochromic anemia and a hemolytic component. Hemosiderin-laden macrophages were not found in the gastric aspirate. He had no pulmonary signs or symptoms. Extensive hematologic and roentgenologic investigations failed to reveal the cause of the anemia. Quantitative serial scintigraphic scanning showed significant (35%) pulmonary sequestration of autologous erythrocytes labeled with sodium chromate Cr51. The half-life of the RBCs was moderately decreased (half-life, 15 days; normal, 25 to 35 days). An open-lung biopsy spece Cr51. The half-life of the RBCs was moderately decreased (half-life, 15 days; normal, 25 to 35 days). An open-lung biopsy specimen confirmed the diagnosis of IPH. A diagnosis of IPH should be considered when children have iron deficiency anemia and pulmonary signs or symptoms. Organ sequestration studies may be helpful in equivocal cases.

1984-08-01

British Library Electronic Table of Contents (United Kingdom)

Abstract. Purpose: To determine whether vitrectomy with 25-gauge instruments contributes to better postoperative visual recovery after macular hole (MH) surgery. Methods: The medical records for 46 consecutive eyes operated for MH by a single surgeon were retrospectively examined. Vitrectomy had been performed with a 25-gauge instrument in 23 eyes (25-G group) and with a 20-gauge instrument in 23 eyes (20-G group). Postoperative visual acuity (VA) in logMAR (logarithm of the minimum angle of resolution) units after 1 week and 1, 3, 6, 9 and 12 months, operating time, and volume of intraocular irrigating fluid were compared between the two groups. Results: Mean preoperative logMAR VA was 0.72 in the 25-G group and 0.68 in the 20-G group (p = 0.282, unpaired t-test). One week after surgery, ...

2008-01-01

Idiopathic renal hematuria in a military working dog.

Late-onset Stargardt-like macular dystrophy maps to chromosome 1p13

A 1.5-year-old male Belgian Malinosis Military Working Dog presented with a 1-month history of intermittent hematuria. Diagnostic ultrasound and contrast radiography demonstrated large blood clots in the urinary bladder and a filling defect in the right renal pelvis. At surgery, clotted blood was present in the right ureter and bladder. Following right nephrectomy, the dog returned to training. One month later, elevations in urea nitrogen and creatinine were noted. Hematuria recurred at 3 months and the dog was found dead in its kennel. Necropsy showed a blood-filled left renal pelvis and ureter. PMID:1454185

1992-10-01

Treatment of asymptomatic internal resorption of a maxillary premolar tooth in a military working dog.

Stargardt`s disease (MIM 248200), originally described in 1909, is an autosomal recessive condition of childhood, characterized by a sudden and bilateral loss of central vision. Typically, it has an early onset (7 to 12 years), a rapidly progressive course and a poor final outcome. The central area of the retina (macula) displays pigmentary changes in a ring form with depigmentation and atrophy of the retinal pigmentary epithelium (RPE). Perimacular yellowish spots, termed fundus flavimaculatus, are observed in a high percentage of patients. We have recently reported the genetic mapping of Stargardt`s disease to chromosome 1p13. On the other hand, considering that fundus flavimaculatus (MIM 230100) is another form of fleck fundus disease, with a Stargardt-like retinal aspect but with a late-onset and a more progressive course, we decided to test the hypothesis of allelism between typical Stargardt`s disease and late-onset autosomal recessive fundus flavimaculatus. Significant pairwise ...

1994-09-01

The use of peritoneal venous shunting for intractable neonatal ascites: a short case series

An asymptomatic pink discoloration of a maxillary right fourth premolar tooth was discovered during a routine oral examination on a 9 year-old Belgian Malinois dog. A radiolucent lesion was seen in the pulpal chamber on radiographic examination. The lesion had perforated the mesiobuccal root of the tooth. The primary differential diagnosis was idiopathic internal resorption. The tooth was treated by partial resection (removal of the mesiobuccal root and associated crown). A vital pulpotomy and amalgam restoration was performed on the remaining tooth structure. A follow-up 1 year later demonstrated a successful treatment outcome. The animal was asymptomatic and able to perform military duties. Clinical and radiographic signs of healing were evident and the tooth was functional. PMID:10518874

1998-12-01

British Library Electronic Table of Contents (United Kingdom)

Introduction: Intractable ascites in neonates has a varied etiology; and often, the cause is idiopathic. The management usually consists of observation, diuretics, paracentesis, albumin replacements, and self correction. However, in some cases, the above treatment remains unsuccessful. Results: We present 2 cases of intractable ascites causing metabolic abnormalities, severe protein and immunoglobulin loss, and respiratory compromise. Although the use of peritoneovenous shunts for intractable ascites has been reported previously, our cases differ in both technique and patient size. Our first patient is an ex-28-week premature, 1.4-kg infant with intractable ascites for which a peritoneal drain was initially placed. After 3 weeks and putting out nearly 300 mL of ascitic fluid daily, we plac...

2011-01-01

Mucosal Inflammation in Spondylarthritides: Past, Present, and Future

British Library Electronic Table of Contents (United Kingdom)

Spondylarthritides (SpA) and inflammatory bowel disease (IBD) are idiopathic, chronic inflammatory disorders. Although they are very distinct and well-defined entities, there is clinical and genetic evidence supporting some degree of overlap between the pathogenesis of the two. Subclinical gut inflammation is present in up to two thirds of all SpA patients and can evolve into IBD. This subclinical gut inflammation has been shown to be strongly associated with joint inflammation, providing a clue for a common pathophysiologic background. Despite extensive research progress in the field over the past few years, many questions remain unanswered. In this paper, we focus on the clinical, genetic, and pathophysiologic overlap of SpA and IBD. Furthermore, we discuss some of the targets that may i...

2011-01-01

Manganese Induces IGF-1 and Cyclooxygenase-2 Gene Expressions in the Basal Hypothalamus during Prepubertal Female Development

British Library Electronic Table of Contents (United Kingdom)

Precocious puberty is a significant child health problem, especially in girls, because 95% of cases are idiopathic. Our earlier studies demonstrated that low-dose levels of manganese (Mn) caused precocious puberty via stimulating the secretion of luteinizing hormone-releasing hormone (LHRH). Because glial-neuronal communications are important for the activation of LHRH secretion at puberty, we investigated the effects of prepubertal Mn exposure on specific glial-derived puberty-related genes known to affect neuronal LHRH release. Animals were supplemented with MnCl2 (10 mg/kg) or saline by gastric gavage from day 12 until day 22 or day 29, then decapitated, and brains removed. The site of LHRH release is the medial basal hypothalamus (MBH), and tissues from this area were analyzed by real-...

2011-01-01

Vitrectomy Timing for Retained Lens Fragments After Surgery for Age-Related Cataracts: A Systematic Review and Meta-Analysis

British Library Electronic Table of Contents (United Kingdom)

PurposeTo evaluate the effect of vitrectomy timing on outcomes for patients with crystalline retained lens fragments receiving vitrectomy 3+ days after cataract surgery. DesignSystematic review and meta-analysis of retrospective interventional cases series. MethodsSearches of MEDLINE (English, 1/1/85 through 7/30/2010) and article reference lists. Articles were screened for patients with crystalline retained lens fragments after surgery for age-related cataracts, discussion of vitrectomy timing, and, for the meta-analysis, patient totals for at least 1 outcome and multiple time periods, 10+ patients, and mean follow-up ?3 months. Outcomes included visual acuity, retinal detachment, increased intraocular pressure, intraocular infection/inflammation, cystoid macular edema, and corneal...

2011-01-01

Ultraviolet-B Phototoxicity and Hypothetical Photomelanomagenesis: Intraocular and Crystalline Lens Photoprotection

British Library Electronic Table of Contents (United Kingdom)

Purpose Ultraviolet-B (UV-B) radiation can cause phototoxic macular injuries in young people who have been sunbathing but not sungazing and in welders. Welders have a reportedly increased risk of uveal melanoma. We analyze phakic and pseudophakic risks for solar and welding arc UV-B exposure. Design Optical radiation measurement, analysis, and perspective. Methods Spectral transmittances were measured for UV-transmitting, UV-blocking, and blue-blocking intraocular lenses (IOLs). The photoprotective performances of crystalline and intraocular lenses were analyzed using relevant epidemiologic and laboratory data and action spectra for acute retinal phototoxicity and melanoma photocarcinogenesis. Results Crystalline lens UV-B retinal protection is deficient in children and young adults, incre...

2010-01-01

Post harvest improvement of zeaxanthin content of vegetables

British Library Electronic Table of Contents (United Kingdom)

Zeaxanthin is a carotenoid produced by plants and has been associated with protection of the photosynthetic machinery under light stress and, together with lutein, in protection of the central retina of the eye. Zeaxanthin levels in blood plasma have been negatively correlated to the development of AMD (age-related macular degeneration) (Gale et al., 2003). Under normal conditions, plants have a low content of zeaxanthin. The aim of this study was to increase the zeaxanthin content in green vegetables by post harvest treatments. Efficient conditions for activation of the endogenous enzyme system generating zeaxanthin was established and included incubation at low pH (2.5-5.5), with the membrane permeable acetic acid/acetate buffer at room temperature or above for 30min or more. Typically m...

2010-01-01

KMeyeDB: a graphical database of mutations in genes that cause eye diseases

British Library Electronic Table of Contents (United Kingdom)

KMeyeDB () is a database of human gene mutations that cause eye diseases. We have substantially enriched the amount of data in the database, which now contains information about the mutations of 167 human genes causing eye-related diseases including retinitis pigmentosa, cone-rod dystrophy, night blindness, Oguchi disease, Stargardt disease, macular degeneration, Leber congenital amaurosis, corneal dystrophy, cataract, glaucoma, retinoblastoma, Bardet-Biedl syndrome, and Usher syndrome. KMeyeDB is operated using the database software MutationView, which deals with various characters of mutations, gene structure, protein functional domains, and polymerase chain reaction (PCR) primers, as well as clinical data for each case. Users can access the database using an ordinary Internet browser wi...

2010-01-01

Assessment of chromosomal abnormalities in sperm of infertile men using sperm karyotyping and multicolour fluorescence in situ hybridization (FISH)

Multiple courses of high-dose total skin electron beam therapy in the management of mycosis fungoides

Individuals with male factor infertility resulting from idiopathic oligo-, astheno- or teratozoospermia are frequently offered IVF in an attempt to increase their chances of having a child. A concern remains whether these infertile males have an elevated risk of transmitting chromosomal abnormalities to their offspring. Sperm chromosomal complements from these men were assayed using the human sperm/hamster oocyte fusion system and fluorescence in situ hybridization (FISH) on sperm nuclei. For each of 5 infertile patients, 100 sperm karyotypes were analyzed and multicolour FISH analysis was performed on a minimum of 10,000 sperm nuclei for each chromosome-specific DNA probe for chromosomes 1 (pUC1.77), 12 (D12Z3), X (XC) and Y (DYZ3). As a group, the infertile patients showed increased frequencies of both numerical ({chi}{sup 2}=17.26, {proportional_to} <0.001) and total abnormalities ({chi}{sup 2}=7.78, {proportional_to} <0.01) relative to control donors when ...

1994-09-01

International Nuclear Information System (INIS)

Purpose: A retrospective analysis was undertaken to determine the indications for, the efficacy of, and the long-term complications of two courses of total skin electron beam therapy for mycosis fungoides. Methods and Materials: A retrospective analysis of 15 patients with the pathologic diagnosis of mycosis fungoides treated in the Department of Radiation Oncology at Stanford University Medical Center between 1968 and 1990 was performed. All patients received two courses of high-dose electron beam therapy to the skin. The mean dose for the total skin treatment for the first course was 32.6 Gy and 23.4 Gy for the second course of treatment. Results: Following the first course of total skin electron beam therapy, 11 of 15 had a complete response, with a mean duration of 11.6 months. All patients received adjuvant therapies between the first and second courses of high-dose total skin electron beam therapy. The mean interval between the first and the second courses of therapy was 41.3 ...

1995-07-30

One-year follow-up of the effects of sildenafil on pulmonary arterial hypertension and veno-occlusive disease

Scientific Electronic Library Online (English)

Abstract in english We hypothesized that chronic oral administration of the phosphodiesterase-5 inhibitor sildenafil could improve the exercise capacity and pulmonary hemodynamics in patients with pulmonary arterial hypertension (PAH) on the basis of previous short-term studies. We tested this hypothesis in 14 subjects with PAH, including seven patients with the idiopathic form and seven patients with atrial septal defects, but no other congenital heart abnormalities. Patients were subjected (more) to a 6-min walk test and dyspnea was graded according to the Borg scale. Pulmonary flow and pressures were measured by Doppler echocardiography. Patients were given sildenafil, 75 mg orally three times a day, and followed up for 1 year. Sildenafil therapy resulted in the following changes: increase in the 6-min walk distance from a median value of 387 m (range 0 to 484 m) to 462 m (range 408 to 588 m; P

2005-02-01

Citrat og nyresten

DEFF Research Database (Denmark)

Citrate is an important naturally occurring inhibitor of calcium stone formation in urine. Urinary citrate excretion was examined in 43 consecutive patients with recurrent idiopathic calcium nephrolithiasis and in 50 normal controls by a specific enzymatic technique. Hypocitraturia (<1.6 mmol/24h) was found in 14 (33%) stone formers compared to 6 (12%) normal controls (p = 0.03). Citrate excretion expressed as citrate-creatinine ratio in 24 hour urine samples was significantly lower in the stone formers than in the healthy controls (p = 0.03), and significantly lower in healthy men, compared to healthy females (p = 0.006). There was a great variability in urinary citrate levels in both groups, and a considerable overlap in the urinary citrate excretion between normal subjects and stone formers. Factors other than urinary citrate excretion must therefore be of importance in the pathophysiology of calcium stone formation. Citrate-calcium ratio in urine proved to ...

1993-01-01

Stability of lutein in wholegrain bakery products naturally high in lutein or fortified with free lutein.

Celastrol regulates innate immunity response via NF-@kB and Hsp70 in human retinal pigment epithelial cells

Lutein is a yellow pigment found in common foods that promotes the health of eyes and skin and is associated with reduced risk of age-related macular degeneration and cataracts. In the present study, selected high-lutein wheat and corn were milled into wholegrain flours by two mills to improve flour uniformity. The high-lutein and lutein-fortified wholegrain flours were processed into breads, cookies, and muffins to study lutein stability during baking and subsequent storage. Lutein and its isomers were separated, identified, and quantified by LC-UV/vis and LC-MS following extraction with water-saturated 1-butanol. Baking resulted in a significant reduction in all-trans-lutein and the formation of cis-lutein and cis-zeaxanthin isomers. Subsequent storage at ambient temperature had a slight impact on the content of all-trans-lutein. Effects of processing were more pronounced in lutein-fortified products, and the degradation rate of lutein was influenced by ...

2010-09-22

British Library Electronic Table of Contents (United Kingdom)

Elevated nuclear factor kappa B (NF-@kB) activity and interleukin-6 (IL-6) secretion participates in the pathology of several age and inflammatory-related diseases, including age-related macular degeneration (AMD), in which retinal pigment epithelial cells are the key target. Recent findings reveal that heat shock protein 70 (Hsp70) may affect regulation of NF-@kB. In the current study, effects of Hsp70 expression on NF-@kB RelA/p65 activity were evaluated in human retinal pigment epithelial cells (ARPE-19) by using celastrol, a novel anti-inflammatory compound. Anti-inflammatory properties of celastrol were determined by measuring expression levels of IL-6 and endogenous NF-@kB levels during lipopolysaccharide (LPS) exposure by using enzyme-linked immunosorbent assays (ELISA). Cell viabil...

2011-01-01

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A YAC contig encompassing the recessive Stargardt disease gene (STGD) on chromosome 1p

Sequence analysis of the ATM gene in 20 patients with RTOG grade 3 or 4 acute and/or late tissue radiation side effects

Stargardt disease (STGD) and fundus flavimaculatus are infrequent autosomal recessive conditions characterized by a juvenile macular dystrophy and variable degrees of peripheral retinal changes. Linkage analysis performed in 47 STGD/fundus flavimaculatus families demonstrated significant linkage to 13 polymorphic DNA markers on chromosome 1p. The maximum combined two-point lod score was 32.7 (maximum recombination fraction [{theta}{sub max}] = .006) with the polymorphic marker D1S188. Our data demonstrate that STGD and fundus flavimaculatus are the same disorder clinically and genetically and provide further evidence for genetic homogeneity of this phenotype. Analysis of recombination on disease chromosomes placed the STGD gene within a 4-cM interval between markers D1S435 and D1S236. A physical map was constructed of a YAC contig flanking STGD, from markers D1S500 to D1S495, and includes the critical interval delineated by historical recombinants. This contig ...

1995-12-01

International Nuclear Information System (INIS)

Purpose: Patients with ataxia-telangiectasia (A-T) show greatly increased radiation sensitivity and cancer predisposition. Family studies imply that the otherwise clinically silent heterozygotes of this autosomal recessive disease run a 3.5 to 3.8 higher risk of developing cancer. In vitro studies suggest moderately increased cellular radiation sensitivity of A-T carriers. They may also show elevated clinical radiosensitivity. We retrospectively examined patients who presented with severe adverse reactions during or after standard radiation treatment for mutations in the gene responsible for A-T, ATM, considering a potential means of future identification of radiosensitive individuals prospectively to adjust dosage schedules. Material and Methods: We selected 20 cancer patients (breast, 11; rectum, 2; ENT, 2; bladder, 1; prostate, 1; anus, 1; astrocytoma, 1; Hodgkins lymphoma, 1) with Grade 3 to 4 (RTOG) acute and/or late tissue radiation side effects by reaction ...

1999-07-15

Pituitary disease in childhood: utility of magnetic resonance; Patologia hipofisaria en la edad pediatrica: unidad de la resonance magnetica

Disorders of brain development and phakomatosis

To assess the utility of magnetic resonance (MR) imaging in the study of pediatric patients with clinical suspicion of pituitary disease. We studied 18 patients aged 7 to 18 years.Fifteen had hormonal disturbances, two presented amenorrhea and 1 complained of headache, fever and symptoms of polyuria and polydipsia. All the patients were examined using a Siemens SP 42 1-Tesla MRI scanner. Sagittal and coronal T1-weighted spin-echo images were obtained; in addition T2-weighted spin-echo or fast spin-echo imaging was performed in ten cases and intravenous gadolinium was administered in nine. We found 9 patients with hypothalamic-pituitary dysgenesis, 2 with germinoma, 2 cases of pituitary hemosiderosis in patients with thalassemia, 2 cases of microadenoma, one abscess, one case of idiopathic central diabetes insipidus and one of Langerhans cell histiocytosis. MR enabled us to assess pituitary structural alterations in children with hypothalamic-pituitary hormone ...

2000-07-01

International Nuclear Information System (INIS)

Full text: Disorders of brain development and phakomatosis are resulting from disturbed embryonic-foetal development One third of all major embryological anomalies involve CNS, and over 2000 different anomalies have been described. Anomalies of the brain often cause foetal and neonatal death, and mental and physical retardation in pediatric group. The majority of disorders of brain development and phakomatosis are idiopathic, and most of them are not hereditary or familial. Ultrasonography plays the important role in screening foetal and neonatal brain, but after closure of fontanels it is difficult to find the acoustic window. CT has limited contrast resolution, and disadvantage exposing infant to ionizing radiation. It is helpful to demonstrate the presence of calcifications. MR imaging has proved to be a diagnostic tool of major importance in children with disorders of brain development and phakomatosis. The excellent grey/white matter differentiation and ...

Assessment of bone formation and bone resorption in osteoporosis: a comparison between tetracycline-based iliac histomorphometry and whole body /sup 85/Sr kinetics

Genetic and physical location of the gene for Stargardt`s disease and further evidence for genetic homogeneity

Bone formation and resorption have been measured in patients with idiopathic osteoporosis by histomorphometry of 7.5-mm trephine biopsies and in the whole body by 85Sr radiotracer methodology and calcium balances. The studies were synchronized and most were preceded by double in vivo tetracycline labeling. Correlations between histological and kinetic bone formation indices were better when better when based on the extent of double tetracycline labels than on measurements of osteoid by visible light microscopy. Correction of the kinetic data for long-term exchange, using 5 months' serial whole body counting of retained 85Sr, improved the fit of the kinetic to the histological data. A statistical analysis of the measurement uncertainties showed that the residual scatter in the best correlations (between exchange-corrected bone formation rates and double-labeled osteoid surface indices) could be attributed to measurement imprecision alone. The ...

1987-12-01