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Publication
Journal: Japanese Journal of Ophthalmology
June/4/2003
Abstract
OBJECTIVE
To assess the effects of topical betaxolol and timolol on the visual field in Japanese open-angle glaucoma (OAG) patients.
METHODS
This study was a multicenter, 2-year, prospective, randomized and double-masked study. Tests using the Humphrey 30-2 perimeter program were conducted every 6 months and the data of 95 patients were analyzed using regression analysis. Estimated regression coefficients for mean deviation (MD), corrected pattern standard deviation (CPSD), and total deviation (TD) values clustered into 15 sectors were obtained for each treatment group.
RESULTS
Estimated slopes (dB/year) for MD and CPSD showed no significant difference from zero in either group. However, in the betaxolol group, estimated slopes (dB/year) for two adjacent sectors in the inferior arcuate area were significantly positive (P =.0135,.0116) while in the timolol group, no significant difference from zero was seen in any of the sectors. IOP changes from baseline in the timolol group were greater than in the betaxolol group, although no statistical significance was seen at any of the examination times.
CONCLUSIONS
MD and CPSD showed no significant change in either group. In the betaxolol group, however, a significant trend in improvement of visual field performance was seen in the inferior arcuate subfield. Timolol reduced IOP more effectively than betaxolol in OAG patients.
Publication
Journal: Investigative Ophthalmology and Visual Science
October/11/2000
Abstract
OBJECTIVE
To determine, in stable glaucoma, the characteristics of the between-examination variability of the visual field recorded with the Humphrey Field Analyser (HFA; Humphrey Systems, Dublin, CA) using the homogeneous, LF(Ho), and heterogeneous, LF(He), components of the long-term fluctuation (LF), thereby providing a technique for separating progressive loss from fluctuation in sensitivity.
METHODS
The LF components were calculated using a two-factor analysis of variance (ANOVA) with replications and were determined between each pair of three successive HFA program 30-2 fields for each patient from two groups, each containing 30 patients with primary open-angle glaucoma. The interval between examinations for the first group was 6 to 9 months and for the second group was 3 weeks.
RESULTS
The group mean values for LF(Ho) ranged from 1.50 to 2.19 dB and for LF(He) from 1.70 to 2.05 dB. The average difference between examinations was within +/-0.35 dB for each component, and the 95% limits of agreement for the two groups, respectively, were +/- 2.31 and +/- 2.39 dB for the LF(Ho) and +/- 2.36 and +/- 2.09 dB for the LF(He). The estimate of the 90% confidence limit for the LF(Ho) was 3.30 dB and for the LF(He), 3.60 dB. Little relationship was present between the LF components and the modulus differences in mean deviation (MD), the corrected pattern SD (CPSD), or the mean MD, mean short-term fluctuation, and mean CPSD, of the two fields.
CONCLUSIONS
Estimation of the LF components and of the corresponding confidence limits yields an expression of the normal between-examination variability of two consecutive fields that can be used as a reliability index. A value outside the confidence limits indicates the necessity for a confirmatory follow-up field.
Publication
Journal: Investigative Ophthalmology and Visual Science
January/14/2013
Abstract
OBJECTIVE
To evaluate macular function in patients with open-angle glaucoma (OAG) by means of multifocal electroretinogram (mfERG).
METHODS
Twenty-four OAG patients (mean age 54.6 ± 9.1 years) and 14 age-similar controls were enrolled. OAG patients had intraocular pressure (IOP) less than 18 mm Hg with topical medical treatment, 24-2 visual field (Humphrey Field Analyzer [HFA]) with mean deviation (MD) between -2 and -12 dB, and corrected pattern standard deviation (CPSD) between +2 and +10 dB and no history or presence of cataract and/or macular disease. MfERGs in response to 61 M-stimuli presented to the central 20° of the visual field were assessed in OAG patients (24 eyes) and in controls (14 eyes). Ring (R) analysis was performed every five retinal eccentricities in areas between the fovea and midperiphery: 0° to 2.5° (R1), 2.5° to 5° (R2), 5° to 10° (R3), 10° to 15° (R4), and 15° to 20° (R5). MfERG response amplitude density of the N1-P1 components (N1-P1 RAD, nV/deg(2)) and P1 implicit time (P1 IT, ms) of the first-order binary kernel were measured for each ring.
RESULTS
OAG patients showed a significant (P < 0.01) decrease in N1-P1 RADs and an increase in P1 IT in both R1 and R2 with respect to controls. The reduction in N1-P1 RADs was significantly (P < 0.01) correlated with HFA MD and CPSD. No other significant differences between OAG and controls were found.
CONCLUSIONS
OAG patients show macular dysfunction detectable by the mfERG technique. Since the mfERG N1-P1 component is thought to be generated by preganglionic elements (photoreceptors and OFF bipolar cells), our data support the functional impairment of the neural generators of the macular region in patients with glaucoma.
Publication
Journal: Eye
December/16/1998
Abstract
OBJECTIVE
To assess the relationship between Humphrey visual field data and optic disc topographical data collected by the Heidelberg Retina Tomograph (HRT) scanning laser ophthalmoscope in chronic glaucoma patients.
METHODS
The mean deviation (MD) and corrected pattern standard deviation (CPSD) from Humphrey visual fields of 106 eyes of 106 patients with glaucoma were analysed for correlation with the multiple topographical measures calculated by the HRT.
RESULTS
Significant correlations were found between MD of the visual field and several optic disc measurements. These included neuroretinal rim volume, mean nerve fibre layer thickness and cross-sectional area, and the cup shape measure. CPSD correlated significantly only with mean nerve fibre layer cross-sectional area. This pattern was common to the whole circumference of the disc with the exception of the directly temporal segment.
CONCLUSIONS
Optic disc topography performed by HRT reflects the optic disc pathology in correlation with perimetry.
Publication
Journal: Journal of Structural Biology
January/25/2012
Abstract
Unique metal-dependent protein tyrosine phosphatases that belong to the polymerase and histindinol phosphatase (PHP) family are present in Gram-positive bacteria. They are distinct from the Cys-based, low-molecular-weight phosphotyrosine protein phosphatases (LMPTPs). Two representative members of the PHP family tyrosine phosphatases are YwqE from Bacillus subtilis and CpsB from Streptococcus pneumoniae. YwqE is involved in polysaccharide biosynthesis, bacterial DNA metabolism, and DNA damage response in B. subtilis. CpsB regulates capsular polysaccharide biosynthesis via tyrosine dephosphorylation of CpsD, its cognate tyrosine kinase, in S. pneumoniae. To gain insights into the active site and possible conformational changes of the metal-dependent tyrosine phosphatases from Gram-positive bacteria, we have determined the crystal structures of B. subtilis YwqE (in both the apo form and the phosphate-bound form) and S. pneumoniae CpsB (in the sulfate-bound form). Comparisons of the three structures reveal conformational plasticity of two active site loops. Furthermore, in both structures of the phosphate-bound YwqE and the sulfate-bound CpsB, the phosphate (or sulfate) ion is bound to a cluster of three metal ions in the active site, thus providing insight into the pre-catalytic state.
Publication
Journal: Eye
May/14/2003
Abstract
OBJECTIVE
The aim of the present study was to compare retinal light sensitivity between normal healthy subjects and chronic renal failure patients treated with maintenance haemodialysis (HD), as well as to determine whether there is a correlation between visual field loss and the age of HD patients, duration of HD treatment and hypertensive retinopathy in HD patients.
METHODS
A total of 50 eyes of 25 HD patients (16 male, nine female) and 30 eyes of 15 controls underwent visual field testing on the C 30-2 program of the Humphrey field analyser.
RESULTS
Significant reduction in retinal light sensitivity with mean deviation (MD) P values less than 5% was found in 36% (18 eyes), pattern standard deviation (PSD) P values less than 5% in 16 eyes (32%) and corrected pattern standard deviation (CPSD) P values less than 5% in 16 eyes (32%) of HD patients. In control group, all MD, PSD, and CPSD P values were within normal limits. No correlation between reduction of retinal light sensitivity and age or duration of dialysis treatment was observed in HD patients. The reduction of retinal light sensitivity was significantly greater in HD patients with hypertensive retinopathy.
CONCLUSIONS
In 36% of eyes from our HD patients without ophthalmoscopically evident arteriolar occlusion on fundus examination, a significant reduction in retinal light sensitivity was observed. The reduction was significantly greater in HD patients with hypertensive retinopathy.
Authors
Publication
Journal: Ophthalmology
September/18/2002
Abstract
OBJECTIVE
To evaluate the long-term effectiveness of glaucoma management in patients undergoing primary glaucoma triple procedure (PGTP) with and without adjunctive subconjunctival mitomycin-C (MMC).
METHODS
Case-controlled study.
METHODS
Of the 203 eyes of 203 primary open-angle glaucoma (POAG) patients who had undergone PGTP and in whom reliable Humphrey visual fields had been obtained both before and after surgery at 13.5 +/- 8.9 and 27.9 +/- 8.9 months, 124 of the 144 eyes that received MMC during surgery were matched to the other 59 eyes that did not with respect to cup-to-disc ratio and risk factors for filtration failure in addition to other variables.
METHODS
Both preoperative and postoperative intraocular pressure (IOP), Humphrey visual fields and their global indices, number of glaucoma medications, and best-corrected visual acuity (BCVA).
RESULTS
There were no significant differences in demographics between the two groups (P>> 0.05 for each). Whereas both the control and the MMC groups attained significant decreases of mean IOP (18.5 +/- 5.7 mmHg-;15.6 +/- 4.6 mmHg, P = 0.0014; 19.3 +/- 7.0 mmHg-13.7 +/- 4.9 mmHg, P = 0.0001) and mean number of medications (2.1 +/- 1.3-1.3 +/- 1.3, P = 0.0001; 2.3 +/- 1.2-1.0 +/- 1.3, P = 0.0001) at 36 months after surgery, the MMC group had significantly lower mean IOP than the control group at all postoperative visits (P < 0.05 for each). The MMC group also tended to have less medical dependency after surgery than the control group. There was no significant difference in postoperative BCVA between the two groups. Patients in both groups had mean visual acuity of 20/30 or better. There was a significant worsening of corrected pattern standard deviation (CPSD) in the control group (3.97 +/- 3.18-5.17 +/- 3.36, P = 0.001) compared with no significant change in the MMC group (5.07 +/- 4.11-5.23 +/- 3.36, P = 0.93). The mean deviation did not change significantly in either group.
CONCLUSIONS
The long-term glaucoma management in POAG patients with cataract undergoing PGTP indicates a successful outcome in final IOP, medical dependency, and BCVA. Furthermore, the MMC group had better IOP control and stable visual fields (CPSD), whereas the control group had a significant worsening of CPSD.
Publication
Journal: Diseases of Aquatic Organisms
May/10/2010
Abstract
Streptococcus iniae poses a serious threat to finfish aquaculture operations worldwide. Stringent regulatory standards limit the use of antibiotics to treat S. iniae infections; improved vaccination strategies are thus of great interest. We investigated the potential for efficient, non-injectable batch vaccination via the use of live attenuated vaccines. Three attenuated S. iniae strains with genetic mutations eliminating the production of virulence factors--capsular polysaccharide (delta cpsD), M-like protein (delta simA), and phosphoglucomutase (delta pgmA)--were evaluated in parallel with an adjuvanted, formalin-killed, whole-cell S. iniae bacterin. Juvenile hybrid striped bass (HSB; Morone chrysops x M. saxatilis) were vaccinated through intraperitoneal (i.p.) injection or bath immersion and held for 800 degree-days prior to challenge with a lethal dose of the virulent wild-type (WT) S. iniae parent strain. The delta cpsD, delta pgmA, and bacterin vaccines provided the highest level of vaccination safety (0% mortality), whereas the delta simA mutant, although it caused 12 to 16% vaccination-related mortality, was the only vaccine candidate to provide 100% protection in both i.p. and immersion delivery models. Our studies demonstrate the efficacy of live attenuated vaccines for prevention of S. iniae infection, and identify immersion delivery of live vaccines as an attractive option for use in commercial aquaculture settings.
Publication
Journal: British Journal of Ophthalmology
February/26/2003
Abstract
OBJECTIVE
Polymorphisms in OPA1, the gene responsible for autosomal dominant optic atrophy, were recently found to be strongly associated with normal tension glaucoma (NTG). The aim of this study was to determine whether OPA1 polymorphisms affect the phenotype of NTG patients.
METHODS
A retrospective analysis was performed of 108 well characterised NTG patients who had been genotyped for OPA1 variations, and who had previously undergone automated perimetry and Heidelberg retina tomography (HRT). 25 NTG patients had the at-risk OPA1 genotype (IVS 8 +4 C/T; +32 T/C) and 83 NTG patients did not. Differences between groups were sought in a wide range of structural, psychophysical, and demographic factors. These included sex, age at diagnosis, family history of glaucoma, history of ischaemic risk factors and vasospasm, laterality of glaucoma, presenting and highest diurnal intraocular pressure (IOP), initial cup-disc (CD) ratio, baseline visual field global indices, and optic disc parameters as measured by HRT. For a subgroup of patients with at least 5 years of follow up and 10 visual field tests, pointwise linear regression analysis (PROGRESSOR for Windows software) was applied to the visual field series.
RESULTS
There was no significant difference in the two groups with respect to sex, age at diagnosis, family history of glaucoma, history of ischaemic risk factors and vasospasm, or laterality of glaucoma. The comparison of IOP, CD ratio and visual field global indices, MD and CPSD in the two groups showed no significant difference. There were no differences in the mean values for any of the HRT parameters analysed. For the subgroup of patients with at least 5 years of follow up, there was also no significant difference in the number of patients with progressing locations, the mean number of progressing locations per subject, the mean slope of the progressing locations or the mean slope for whole visual field.
CONCLUSIONS
The absence of phenotypic differences in normal tension glaucoma patients with and without the OPA1 polymorphisms IVS 8 +4 C/T; +32 T/C suggest that these OPA1 polymorphisms do not underlie any major phenotypic diversity in these patients.
Publication
Journal: American Journal of Ophthalmology
June/6/2005
Abstract
OBJECTIVE
To evaluate the impact of different definitions of primary open angle glaucoma (POAG) on assessment of the diagnostic validity of Heidelberg Retinal Tomography (HRT).
METHODS
Retrospective, cross-sectional study.
METHODS
A search of medline (1992-2003) led to the retrieval of 181 papers containing definitions of POAG, including the eight visual field (VF)-based definitions used for this study. A sample of 193 normal subjects, 222 patients with suspected POAG, and 103 with POAG underwent HRT and the 24 II Humphrey VF examinations to assess the diagnostic validity of HRT. POAG was defined on the basis of Glaucoma Hemifield Test (GHT) "out of normal limits" associated with corrected pattern standard deviation (CPSD)>> 2 dB and intraocular pressure (IOP)>> or =22 mm Hg. The VFs were re-analyzed and categorized according to the other eight definitions of POAG: IOP formed part of all the definitions, whereas the appearance of the optic disk did not. The sensitivity and specificity of each scenario were calculated by standard procedures.
RESULTS
The definitions of POAG found in the literature included 17 IOP criteria, more than 15 optic disk criteria, and more than 30 VF criteria. The sensitivity of HRT ranged from 0.51 to 0.80, and its specificity from 0.94 and 0.95 when the patients with suspected POAG were excluded; diagnostic validity was much lower and still variable when the POAG suspects were included with the normal or the POAG groups.
CONCLUSIONS
The most commonly used VF-based definitions of POAG led to substantial differences in the sensitivity and specificity of HRT when using the same large sample of normal subjects and POAG patients. A standard definition of POAG is needed to make diagnostic investigations more accurate and comparable.
Publication
Journal: International Ophthalmology
December/22/1992
Abstract
82 eyes of 82 patients with different types of glaucoma were examined with various psychophysical tests assessing central and paracentral function, including foveal temporal contrast sensitivity function, FM 100-Hue test, and foveal and parafoveal blue-on-yellow-sensitivity. For all eyes visual field tests were performed with the Humphrey-Field-Analyzer, program 30-2. Global visual field indices were calculated as follows: Mean Sensitivity MS, Mean Deviation MD, and Corrected Pattern Standard Deviation CPSD. Linear regression analysis and multiple regression analysis correcting for a possible influence of age between the central and paracentral criteria and the global indices was performed. For the entire study population highly significant correlations are present between foveal and parafoveal blue-on-yellow-sensitivity and MS, MD and CPSD. Whereas in the Normal Tension Glaucoma subgroup (19/82 eyes) no significant correlations are found, the subgroup of 35/82 eyes with markedly elevated intraocular pressure >> or = 30 mmHg) shows highly statistically significant correlations between the low- and high-frequency end of the foveal temporal contrast sensitivity function and foveal and parafoveal blue-on-yellow-sensitivity and the global field indices. The results of the present study support the idea that there are two different mechanisms of glaucomatous damage, one which is pressure-dependent and one which may be pressure-independent. The pressure-dependent mechanism is responsible for deficits of central or paracentral function which are correlated to overall visual field damage.
Publication
Journal: Australian and New Zealand journal of ophthalmology
September/20/1998
Abstract
OBJECTIVE
To assess the effect of cataract on the glaucomatous visual field.
METHODS
We prospective y assessed the visual field indices of mean deviation (MD), pattern standard deviation (PSD), short-term fluctuation (SF) and corrected pattern standard deviation (CPSD) before and after cataract extraction with posterior chamber intra-ocular lens in a consecutive series of 40 patients (51 eyes) age 75.0 +/- 6.0 years with varying degrees of field oss or suspect field loss.
RESULTS
After cataract operation, statistical analysis showed a very significant fall in the absolute magnitude of MD and a significant rise n PSD. There was considerable individual variation in results.
CONCLUSIONS
Taking these changes into account, the indices gave a good prediction of the glaucomatous field status following cataract operation, which itself results in some increase in the underlying field defect.
Publication
Journal: Human Mutation
April/11/2004
Abstract
Carbamyl Phosphate Synthetase I deficiency (CPSID) is a rare autosomal recessive urea cycle disorder usually characterized by potentially lethal neonatal hyperammonemia. The large (5215 bp) CPS1-cDNA, expressed only in liver and epithelial cells of intestinal mucosa, has been cloned. Until now the CPS1 genomic organization was unknown. Taking advantage of the phylogenetic lineage between the CPS1 gene of Homo sapiens and Rattus norvegicus, we determined the intron-exon organization of the human CPS1 gene. Starting from the ATG codon, the CPS I gene is organized in 38 exons spanning from 50bp to 200 bp. We also report the molecular studies on an Italian patient affected by neonatal CPSD. Two novel genetic lesions (c.1370T>G and c.2429A>G) that lead to the novel amino acid substitutions V457G and Q810R, and the known N1406T polymorphism, were detected in the patient's CPS1 RNA and in genomic DNA isolated from peripheral blood lymphocytes. The characterization of the CPS1 genomic organization will allow the identification of the genetic lesions of CPSD patients, the detection of carriers, better genetic counseling and a more certain, less invasive method of prenatal diagnosis.
Publication
Journal: Klinische Monatsblatter fur Augenheilkunde
July/11/2001
Abstract
BACKGROUND
Analysis of clinical importance of the size of filling defects in fluorescein angiograms in primary open-angle glaucoma (POAG), normal-tension glaucoma (NTG), ocular hypertension and subjects with physiological excavations in comparison to visual field loss, optic nerve head morphology and hemodynamics.
METHODS
75 patients (POAG, NTG, ocular hypertension) and 10 healthy subjects with physiological excavations were included in this study. In digitized video fluorescein angiograms (Scanning Laser Ophthalmoscope) the size of absolute filling defects of the optic disc was quantified in the early venous phase and expressed by percentage of the optic disc. Visual fields were obtained by conventional static perimetry (Humphrey 24-2) and graded in stages of glaucoma visual field defects (Aulhorn I-V). Optic disc excavations were evaluated as cup-to-disc-area-ratios.
RESULTS
The filling defects correlated with the visual-field loss stages of Aulhorn and the visual field indices MD (mean deviation), PSD (pattern standard deviation) and CPSD (corrected pattern standard deviation). There was no correlation with the index SF (short-term fluctuation) and with systemic hemodynamics (blood pressure, perfusion pressure) or the IOP. Absolute filling defects correlated with the cup-to-disc-area-ratio in NTG. The absolute filling defects were larger in patients with glaucoma (POAG, NTG) in comparison to patients without glaucomatous visual field loss (ocular hypertension, glaucoma-like discs). No difference of filling defects was found in the glaucoma group (POAG, NTG). Patients with NTG had larger excavations and lower systolic blood pressures than patients with POAG.
CONCLUSIONS
The size of fluorescein filling defects may be useful as a parameter for the evaluation of an ischemic lesion of the optic nerve head. Absolute filling defects may differentiate POAG from ocular hypertension and NTG from glaucoma-like discs without field defects. The results support the hypothesis that in POAG and NTG disturbances of the circulation result in similar filling defects of the optic disc and visual field loss.
Publication
Journal: Metabolic Brain Disease
November/13/2018
Abstract
Urea cycle disorders (UCDs) are inherited metabolic diseases that lead to hyperammonemia. Neurodevelopmental outcomes of patients with UCDs depend on the maximum ammonia concentration (MAC) in the blood during onset. MAC ≥360 μM is a marker of poor neurodevelopmental outcomes. We investigated the neurodevelopmental outcomes and MAC at onset for 177 patients with UCDs in Japan (median age, 8 years and 2 months; range, 10 days-72 years), including 57 patients with male ornithine transcarbamylase (OTCD), 59 patients with female OTCD, 23 patients with carbamoyl-phosphate synthetase 1 deficiency (CPSD), 28 patients with arginosuccinate synthetase deficiency, 9 patients with arginosuccinate lyase deficiency (ALD), and 1 patient with arginase 1 deficiency. Neurodevelopmental outcomes of patients with CPSD and ALD were poor because most had neonatal onset with blood MAC ≥300 μM at onset. Although OTCD, particularly female late-onset OTCD, has good neurodevelopmental outcomes among those with UCDs, it is not necessarily a mild disease with good long-term outcomes. Patients with severe UCDs and MAC ≥300 μM at onset should undergo liver transplantation (LT). Moreover, this study suggested that if the onset of UCD began during the neonatal period, then even UCD patients with MAC <300 μM at onset should undergo LT to protect the brain.
Publication
Journal: JIMD Reports
August/8/2019
Abstract
Domino liver transplantation (DLT) involves transplanting liver from a patient with metabolic disease into a patient with end-stage liver disease with the expectation that the recipient will not develop the metabolic syndrome or the recurrent syndrome will have minimal affect. The domino donor gets a deceased donor or a segment of live-donor liver through the deceased donor organ allocation system. Waitlist mortality for the domino recipient exceeds morbidity associated with getting the donor disease. Between 2015 and 2017, four patients with three metabolic disorders at UPMC Children's Hospital of Pittsburgh underwent DLT with domino allografts from maple syrup urine disease (MSUD) patients. These included patients with propionic acidemia (PA) (n = 1), Crigler-Najjar (CN) syndrome type-1 (n = 2), and carbamoyl phosphate synthetase deficiency (CPSD) (n = 1). Mean follow-up was 1.6 years (range 1.1-2.1 years). Total bilirubin levels normalized postoperatively in both CN patients and they maintain normal allograft function. The PA patient had normal to minimal elevations of isoleucine and leucine, and no other abnormalities on low protein diet supplemented with a low methionine and valine free formula. No metabolic crises have occurred. The patient with CPSD takes normal baby food. No elevation in ammonia levels have been observed in any of the patients. DLT for a select group of metabolic diseases alleviated the recipients of their metabolic defect with minimal evidence of transferrable-branched chain amino acid elevations or clinical MSUD despite increased protein intake. DLT using allografts with MSUD expands the live donor liver pool and should be considered for select metabolic diseases that may have a different enzymatic deficiency.
Publication
Journal: Acta Ophthalmologica
February/5/2013
Abstract
OBJECTIVE
To evaluate the effects of chronic obstructive pulmonary disease (COPD) on retina and optic nerve.
METHODS
Thirty-eight patients with COPD and 29 healthy controls, totally 67 subjects, were included in the study. Visual evoked potentials (VEP) and visual field assessment (both standard achromatic perimetry (SAP) and short-wavelength automated perimetry (SWAP)) were performed on each subject after ophthalmological, neurological and pulmonary examinations.
RESULTS
Mean deviation (MD), pattern standard deviation (PSD) and corrected pattern standard deviation (CPSD) were significantly different between patient and control groups as for both SAP and SWAP measurements (p = 0.001, 0.019, 0.009 and p = 0.004,0.019, 0.031, respectively). Short-term fluctuation (SF) was not statistically different between the study and the control groups (p = 0.874 and 0.694, respectively). VEP P100 latencies were significantly different between patients with COPD and the controls (p = 0.019).
CONCLUSIONS
Chronic obstructive pulmonary disease is a systemic disease, and hypoxia in COPD seems to affect the retina and the optic nerve.
Publication
Journal: Nihon Sanka Fujinka Gakkai zasshi
January/15/1992
Abstract
Carbamyl phosphate synthetase deficiency (CPSD) is one of the enzyme defects of the urea cycle and inherited as an autosomal recessive. A definitive enzymatic diagnosis of CPSD can be made by biochemical assay of liver biopsy material, but not of cultured fibroblasts. In pregnancy at risk for CPSD, prenatal diagnosis was attempted by fetal liver biopsy, performed at 22 weeks of gestation. CPS activity was present and a healthy baby was delivered at term. The technique employed for fetal liver biopsy is described together with an evaluation of its possible role in prenatal diagnosis.
Publication
Journal: Acta ophthalmologica Scandinavica
October/13/2004
Abstract
OBJECTIVE
To investigate whether moderate cigarette smoking has any effects on the central visual field.
METHODS
This study included 30 healthy, moderate cigarette smokers (10-20 cigarettes per day for at least the past 5 years) and 22 healthy non-smokers. After two training test sessions, all individuals underwent computerized visual field examinations (Humphrey 30-2 Full Threshold Test) with both white-on-white (W-W) perimetry and blue-on-yellow (B-Y) perimetry. One eye of each subject with reliable visual field test results was evaluated. The foveal threshold, mean deviation (MD), pattern standard deviation (PSD), short-term fluctuation (SF), corrected pattern standard deviation (CPSD), glaucoma hemifield test (GHT) and number of significantly depressed points deviating at p < 5%, p < 2%, p < 1% and p < 0.5% on the pattern deviation probability map of the smokers were compared with those of the non-smokers.
RESULTS
When the results of W-W perimetry were analysed, the smokers were found to have significantly lower foveal thresholds (p = 0.001) and mean retinal sensitivity (p = 0.02), and higher PSD (p = 0.002) and CPSD (p = 0.01) than the non-smokers. Short-term fluctuation was similar in both groups (p = 0.55). The number of significantly depressed points deviating at p < 5%, p < 2% and p < 1% on the pattern deviation probability map was similar for both groups (p>> 0.05). The number of depressed points deviating at p < 0.5% on the pattern deviation probability map was higher for the smokers than for the non-smokers (p = 0.03). The results of B-Y perimetry showed the smokers to have a significantly lower foveal threshold than the non-smokers (p = 0.03). However, there were no significant differences in the global indices of the two groups (p>> 0.05). The number of significantly depressed points deviating at p < 5%, p < 2%, p < 1% and p < 0.5% on the pattern deviation probability map was similar in both groups (p>> 0.05). No significant difference in GHT was determined with either perimetry for the smokers compared with the non-smokers (p>> 0.05).
CONCLUSIONS
This study suggests that moderate cigarette smoking is associated with both diffuse and localized reductions in retinal sensitivity with W-W perimetry. Only reduction in the foveal threshold was observed with B-Y perimetry, with no hints of diffuse and localized reductions.
Publication
Journal: Ophthalmic Epidemiology
May/12/2002
Abstract
OBJECTIVE
To examine the effects of the gold standard on sensitivity, specificity, and area under the ROC curve when determining the validity of a screening test.
METHODS
Fifty-eight people were recruited from a Veterans' Administration eye clinic. Screening included testing with the 26-point oculokinetic perimeter (OKP). We used different gold standards to categorize patients as positive or negative for glaucoma. The glaucoma hemifield test (GHT) and corrected pattern standard deviation (CPSD) from the Humphrey visual field analyzer (HVF) were used as objective gold standards. Ophthalmologist review of the HVF, using two different methods for classifying visual field defects, was also used as a gold standard.
RESULTS
Using the review of the HVF that included mild, moderate, or severe defects, the area under the ROC curve was not significantly different from 50%. However, for all the other gold standards, the area under the ROC curve was significantly different from 50%.
CONCLUSIONS
In determining the ability of the OKP to differentiate between glaucoma and normal subjects, the choice of a gold standard led to different conclusions about its usefulness as a screening test for glaucoma. Furthermore, sensitivity and specificity varied widely depending on which gold standard was used, and what cutoff point was used for the OKP. Although clinician review of the HVF may be used when specific criteria are given to define visual field defects, using objective measures from the HVF is easier and will allow for better comparisons between studies.
Publication
Journal: Journal of Ocular Pharmacology and Therapeutics
February/10/2013
Abstract
OBJECTIVE
To investigate the longitudinal changes of visual field (VF) in Asian patients with primary angle-closure glaucoma (PACG) occurring with and without an acute attack.
METHODS
In this retrospective case series, 87 consecutive patients diagnosed with bilateral PACG during the period from 2000 to 2010 were included. The eyes of the enrolled patients were categorized into 2 groups: PACG with 1 documented episode of an acute episode in the affected eye (APACG); PACG without a previous documented acute episode in the fellow eye (CPACG). The historical change in the VF in both groups was assessed every 3 months for >2 years.
RESULTS
Of the 174 eyes included in the study, 87 eyes belonged to the APACG group, and 87 eyes belonged to the CPACG group. The mean deviation (MD) in the APACG group decreased significantly from -6.22 to -6.73 dB between the 6- and 9-month follow-up periods (P=0.03). There were significant differences between the 2 groups in the MD index from the 9- to 24-month follow-up. The corrected pattern standard deviation (CPSD) in the APACG group increased significantly from 3.61 to 3.71 dB between the 6- and 9-month follow-up periods (P=0.04). The CPSD index was higher in the APACG group than in the CPACG group from the 9- to 24-month follow-up, which was a statistically significant difference.
CONCLUSIONS
Glaucomatous VF damage in Asian patients with APACG was detected after a 6-month follow-up period, despite the presence of laser peripheral iridotomy in this population. In the patients with CPACG, there was no significant difference in the MD and CPSD during the follow-up period. Patients with APACG would benefit from regular monitoring of the decline in the VF.
Publication
Journal: European Journal of Ophthalmology
January/15/2007
Abstract
OBJECTIVE
To evaluate the results of short wavelength perimetry (SWAP) of ocular hypertensive (OHT) patients and correlate these findings with central corneal thickness (CCT) measurements.
METHODS
Thirty-seven OHT patients with a mean age of 50.2+/-8.2 (SD) years and 30 control subjects with a mean age of 50.3+/-8.5 (SD) years were included in this study. A questionnaire was applied to patients to evaluate the demographic risk factors that may predict glaucoma development. After a detailed ophthalmologic examination, achromatic and short wavelength perimetries and ultrasonic pachymetry were performed and the results were compared between the two groups with Student t test and Mann-Whitney U test. A p value<0.05 is considered as statistically significant.
RESULTS
Mean CCT was higher in the OHT group (right eye; 558.13+/-28.39 microm and left eye; 558.94+/-27.30 microm) when compared with the control subjects (524.66+/-30.53 microm and 525.86+/-30.46 microm, respectively) (p<0.01). A significant positive correlation was found between CCT measurements and intraocular pressure (r=0.5, p<0.001). Four right eyes (10.8%) and five left eyes (13.5%) of OHT patients had defects in SWAP. OHT patients with SWAP abnormalities had significantly lower CCT measurements in right (527.25+/-17.34 microm) and left eye (528.80+/-13.60 microm) when compared with OHT patients without SWAP defects (561.87+/-27.29 microm and 563.65+/-25.92 microm, respectively) (p<0.05). Significant correlations were found between CCT and SWAP MD, PSD, and CPSD (p<0.05).
CONCLUSIONS
OHT patients with SWAP abnormalities had significantly lower CCT measurements than those without. CCT is considered as a risk factor for the development of glaucomatous damage in OHT patients.
Publication
Journal: Canadian Journal of Microbiology
February/29/2012
Abstract
Using the yeast two-hybrid system, intraspecific protein interactions were detected in Streptococcus iniae and Lactococcus lactis subsp. cremoris between the transmembrane activation protein (CpsC and EpsA, respectively) and the protein tyrosine kinase (CpsD and EpsB, respectively), between two protein tyrosine kinases, and between the protein tyrosine kinase and the phosphotyrosine phosphatase (CpsB and EpsC, respectively). For each of these intraspecific interactions, interspecific interactions were also detected when one protein was from S. iniae and the other was from Streptococcus thermophilus . Interactions were also observed between two protein tyrosine kinases when one protein was from either of the Streptococcus species and the other from L. lactis subsp. cremoris. The results and sequence comparisons performed in this study support the conclusion that interactions among the components of the tyrosine kinase - phosphatase regulatory system are conserved in the order Lactobacillales and that interspecific genetic exchanges of the genes that encode these proteins have the potential to form functional recombinants. A better understanding of intraspecific and interspecific protein interactions involved in regulating exopolysaccharide biosynthesis may facilitate construction of improved strains for industrial uses as well as identification of factors needed to form functional regulatory complexes in naturally occurring recombinants.
Publication
Journal: Infection and Immunity
April/12/2015
Abstract
Group B streptococci (GBS; Streptococcus agalactiae) are beta-hemolytic, Gram-positive bacteria that are common asymptomatic colonizers of healthy adults. However, these opportunistic bacteria also cause invasive infections in human newborns and in certain adult populations. To adapt to the various environments encountered during its disease cycle, GBS encodes a number of two-component signaling systems. Previous studies have indicated that the TCS comprising the sensor histidine kinase RgfC and the response regulator RgfA mediate GBS binding to extracellular matrix components, such as fibrinogen. However, in certain GBS clinical isolates, a point mutation in rgfA results in premature truncation of the response regulator. The truncated RgfA protein lacks the C-terminal DNA binding domain necessary for promoter binding and gene regulation. Here, we show that deletion of rgfC in GBS strains lacking a functional RgfA increased systemic infection. Furthermore, infection with the rgfC mutant increased induction of proinflammatory signaling pathways in vivo. Phosphoproteomic analysis revealed that 19 phosphopeptides corresponding to 12 proteins were differentially phosphorylated at aspartate, cysteine, serine, threonine, or tyrosine residues in the rgfC mutant. This included aspartate phosphorylation of a tyrosine kinase, CpsD, and a transcriptional regulator. Consistent with this observation, microarray analysis of the rgfC mutant indicated that >200 genes showed altered expression compared to the isogenic wild-type strain and included transcriptional regulators, transporters, and genes previously associated with GBS pathogenesis. Our observations suggest that in the absence of RgfA, nonspecific RgfC signaling affects the expression of virulence factors and GBS pathogenesis.
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