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1.
BMC Public Health ; 23(1): 241, 2023 02 04.
Artigo em Inglês | MEDLINE | ID: mdl-36737719

RESUMO

BACKGROUND: Despite impressive strides in health, social protection, and education, children continue to experience high rates of child maltreatment in Malaysia. This mixed-methods study assessed the feasibility of a five-session, social learning-based parenting program delivered by government staff in a community setting to reduce violence against children. METHODS: Parents of children from birth to 17 years were recruited from two communities near Kuala Lumpur to participate in the government-run program called the Naungan Kasih Positive Parenting Program ("Protecting through Love" in Bahasa Melayu). Quantitative data from female caregivers (N = 74) and children ages 10-17 (N = 26) were collected along with qualitative interviews and focus groups with parents, children, and facilitators. The primary outcome was child maltreatment with secondary outcomes including neglect, positive parenting, acceptability of corporal punishment, harsh parenting, positive discipline, and child behavior problems. Multilevel Poisson regression and multilevel linear regression were conducted to compare baseline and post-test outcomes. Qualitative interviews and focus groups examined how participants experienced the program utilizing a thematic analysis approach. RESULTS: Quantitative analyses found pre-post reductions in overall child maltreatment, physical abuse, emotional abuse, attitudes supporting corporal punishment, parent sense of inefficacy, and child behavior problems. There were no reported changes on positive and harsh parenting, parental mental health, and marital satisfaction, nor were there any other significant changes reported by children. Qualitative findings suggested that the program had tangible benefits for female caregivers involved in the program, with the benefits extending to their family members. CONCLUSIONS: This feasibility study is one of the few studies in Southeast Asia that examined the feasibility and initial program impact of a parenting program delivered by government staff to families with children across the developmental spectrum from birth to 17 years. Promising results suggest that the program may reduce child maltreatment across a range of child ages. Findings also indicate areas for program improvement prior to further delivery and testing, including additional training and content on sexual and reproductive health, parenting children with disabilities, and online child protection.


Assuntos
Maus-Tratos Infantis , Poder Familiar , Criança , Humanos , Feminino , Adolescente , Poder Familiar/psicologia , Estudos de Viabilidade , Malásia , Violência , Pais/psicologia , Maus-Tratos Infantis/prevenção & controle , Maus-Tratos Infantis/psicologia
2.
Disabil Health J ; 15(3): 101317, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-35410777

RESUMO

BACKGROUND: Achieving equitable medical care for people with disabilities is a complex challenge with emphasis often placed on the need for improved physician knowledge and cultural competence. Physical medicine and rehabilitation (PM&R) is a specialty dedicated to maximizing patient function, where a focus on working with and learning from patients with complex disabilities informs physician training and patient care. OBJECTIVE: The purpose of this study was to assess barriers to equitable care in PM&R clinics through a structural checklist and semi-structured interviews with clinic staff and physicians. METHODS: We used qualitative analysis with a grounded theory approach to develop a unified explanation of how existing clinic processes and provider attitudes affect equitable access to medical care. RESULTS: We found physicians comfortable with and respectful of patient differences who described leveraging unpaid time and creativity to navigate structural, resource, and awareness barriers. Staff and physicians described current barriers as negatively affecting quality of care, clinic efficiency, and, in some cases, patient and staff safety. CONCLUSION: Our results suggest that high levels of physician disability-related knowledge and cultural competence may be insufficient to the challenge of achieving equitable care.


Assuntos
Pessoas com Deficiência , Médicos , Competência Cultural , Humanos
3.
Plant Dis ; 104(10): 2541-2550, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32762502

RESUMO

Tar spot of corn has been a major foliar disease in several Latin American countries since 1904. In 2015, tar spot was first documented in the United States and has led to significant yield losses of approximately 4.5 million t. Tar spot is caused by an obligate pathogen, Phyllachora maydis, and thus requires a living host to grow and reproduce. Due to its obligate nature, biological and epidemiological studies are limited and impact of disease in corn production has been understudied. Here we present the current literature and gaps in knowledge of tar spot of corn in the Americas, its etiology, distribution, impact and known management strategies as a resource for understanding the pathosystem. This will in tern guide current and future research and aid in the development of effective management strategies for this disease.


Assuntos
Doenças das Plantas , Zea mays , América , Estados Unidos
4.
Phytobiomes J ; 3(3): 235-243, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31867561

RESUMO

Tar spot is a fungal disease complex of corn that has been destructive and yield limiting in Central and South America for nearly 50 years. Phyllachora maydis, the causal agent of tar spot, is an emerging corn pathogen in the United States, first reported in 2015 from major corn producing regions of the country. The tar spot disease complex putatively includes Monographella maydis (syn. Microdochium maydis), which increases disease damage through the development of necrotic halos surrounding tar spot lesions. These necrotic halos, termed "fish-eye" symptoms, have been identified in the United States, though Monographella maydis has not yet been confirmed. A recent surge in disease severity and loss of yield attributed to tar spot in the United States has led to increased attention and expanded efforts to understand the disease complex and how to manage it. In this study, next-generation sequencing of the internal transcribed spacer-1 (ITS1) ribosomal DNA was used to identify fungal taxa that distinguish tar spot infections with or without fish-eye symptoms. Fungal communities within tar spot only lesions were significantly different from communities having fish-eye symptoms. Two low abundance OTUs were identified as Microdochium sp., however, neither were associated with fish-eye symptom development. Interestingly, a single OTU was found to be significantly more abundant in fish-eye lesions compared to tar spot lesions and had a 91% ITS1 identity to Neottiosporina paspali. In addition, the occurrence of this OTU was positively associated with Phyllachora maydis fish-eye symptom networks, but not in tar spot symptom networks. Neottiosporina paspali has been reported to cause necrotic lesions on various monocot grasses. Whether the related fungus we detected is part of the tar-spot complex of corn and responsible for fish-eye lesions remains to be tested. Alternatively, many OTUs identified as Phyllachora maydis, suggesting that different isolate genotypes may be capable of causing both tar spot and fish-eye symptoms, independent of other fungi. We conclude that Monographella maydis is not required for fish-eye symptoms in tar spot of corn.

6.
Anim Genet ; 50(1): 78-81, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30353927

RESUMO

Osteochondrosis is a common developmental orthopedic disease characterized by a failure of endochondral ossification. Standardbred horses are recognized as being predisposed to tarsal osteochondrosis. Prior heritability estimates for tarsal osteochondrosis in European Standardbreds and related trotting breeds have been based on pedigree data and range from 17-29%. Here, we report on genetic architecture and heritability based on high-density genotyping data in a cohort of North American Standardbreds (n = 479) stringently phenotyped for tarsal osteochondrosis. Whole-genome array genotyping data were imputed to ~2 million single nucleotide polymorphisms (SNPs). SNP-based heritability of osteochondrosis in this population was explained by 2326 SNPs. The majority of these SNPs (86.6%) had small effects, whereas fewer SNPs had moderate or large effects (10% and 2.9% respectively), which is consistent with a polygenic/complex disease. Heritability was estimated at 0.24 ± 0.16 using two methods of restricted maximum likelihood analysis, as implemented in gcta (with and without a weighted relatedness matrix) and ldak software. Estimates were validated using bootstrapping. Heritability estimates were within the range previously reported and suggest that osteochondrosis is moderately heritable but that a significant portion of disease risk is due to environmental factors and/or genotype × environment interactions. Future identification of the genes/variants that have the most impact on disease risk may allow early recognition of high-risk individuals.


Assuntos
Doenças dos Cavalos/genética , Cavalos/genética , Osteocondrose/veterinária , Polimorfismo de Nucleotídeo Único , Animais , Interação Gene-Ambiente , Predisposição Genética para Doença , Genótipo , Bloqueio Interatrial , Funções Verossimilhança , Modelos Genéticos , América do Norte , Osteocondrose/genética , Fenótipo
7.
J Phys Chem Lett ; 9(19): 5664-5671, 2018 Oct 04.
Artigo em Inglês | MEDLINE | ID: mdl-30205006

RESUMO

Near-infrared spectroscopy measurements are presented for protonated water clusters, H+(H2O) n, in the size range of n = 1-8. Clusters are produced in a pulsed-discharge supersonic expansion, mass selected, and studied with infrared laser photodissociation spectroscopy in the regions of 3600-4550 and 4850-7350 cm-1. Although there is some variation with cluster size, the main features of these spectra are a broad absorption near 5300 cm-1, a sharp doublet near 7200 cm-1, as well as a structured absorption near 4100 cm-1 for n ≥ 2. The vibrational patterns measured for the hydronium, Zundel, and Eigen ions are compared to those predicted by different forms of anharmonic theory. Second-order vibrational perturbation theory (VPT2) and a local mode treatment of the OH stretches both capture key aspects of the spectra but suffer understandable deficiencies in the quantitative description of band positions and intensities.

8.
Vet J ; 237: 9-15, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-30089549

RESUMO

Foot problems are very common causes of lameness in horses. With the recent diagnostic advances to evaluate and treat foot pathology as well as to monitor response to therapy, it is now possible to more accurately evaluate the effectiveness of many of these treatments. This review details some of the recent advances of the most common conservative and surgical treatment options for foot problems in horses, including an overview of evidence on the efficacy to support the use of these treatment options and on factors that may affect prognosis.


Assuntos
Doenças do Pé/veterinária , Doenças dos Cavalos/terapia , Coxeadura Animal/terapia , Animais , , Doenças do Pé/cirurgia , Doenças do Pé/terapia , Doenças dos Cavalos/cirurgia , Cavalos , Coxeadura Animal/cirurgia , Prognóstico
9.
J Chem Phys ; 145(17): 174303, 2016 Nov 07.
Artigo em Inglês | MEDLINE | ID: mdl-27825210

RESUMO

Pulsed discharges in supersonic expansions containing the vapor of different precursors (formaldehyde, methanol) produce the m/z = 30 cations with formula [H2,C,O]+. The corresponding [H2,C,O]+ Ar complexes are produced under similar conditions with argon added to the expansion gas. These ions are mass selected in a time-of-flight spectrometer and studied with infrared laser photodissociation spectroscopy. Spectra in the 2300-3000 cm-1 region produce very different vibrational patterns for the ions made from different precursors. Computational studies with harmonic methods and various forms of anharmonic theory allow detailed assignment of these spectra to two isomeric species. Discharges containing formaldehyde produce primarily the corresponding formaldehyde radical cation, CH2O+, whereas those with methanol produce exclusively the cis- and trans-hydroxymethylene cations, HCOH+. The implications for the interstellar chemistry of these cations are discussed.

10.
Appl Clin Inform ; 6(2): 334-44, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26171079

RESUMO

BACKGROUND: Clinical knowledge bases of problem-medication pairs are necessary for many informatics solutions that improve patient safety, such as clinical summarization. However, developing these knowledge bases can be challenging. OBJECTIVE: We sought to validate a previously developed crowdsourcing approach for generating a knowledge base of problem-medication pairs in a large, non-university health care system with a widely used, commercially available electronic health record. METHODS: We first retrieved medications and problems entered in the electronic health record by clinicians during routine care during a six month study period. Following the previously published approach, we calculated the link frequency and link ratio for each pair then identified a threshold cutoff for estimated problem-medication pair appropriateness through clinician review; problem-medication pairs meeting the threshold were included in the resulting knowledge base. We selected 50 medications and their gold standard indications to compare the resulting knowledge base to the pilot knowledge base developed previously and determine its recall and precision. RESULTS: The resulting knowledge base contained 26,912 pairs, had a recall of 62.3% and a precision of 87.5%, and outperformed the pilot knowledge base containing 11,167 pairs from the previous study, which had a recall of 46.9% and a precision of 83.3%. CONCLUSIONS: We validated the crowdsourcing approach for generating a knowledge base of problem-medication pairs in a large non-university health care system with a widely used, commercially available electronic health record, indicating that the approach may be generalizable across healthcare settings and clinical systems. Further research is necessary to better evaluate the knowledge, to compare crowdsourcing with other approaches, and to evaluate if incorporating the knowledge into electronic health records improves patient outcomes.


Assuntos
Crowdsourcing/métodos , Quimioterapia Assistida por Computador , Registros Eletrônicos de Saúde , Bases de Conhecimento , Centros Médicos Acadêmicos , Humanos
11.
Vet Pathol ; 52(5): 803-18, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26063173

RESUMO

Osteoarthritis (OA) is unquestionably one of the most important chronic health issues in humans, affecting millions of individuals and costing billions of dollars annually. Despite widespread awareness of this disease and its devastating impact, the pathogenesis of early OA is not completely understood, hampering the development of effective tools for early diagnosis and disease-modifying therapeutics. Most human tissue available for study is obtained at the time of joint replacement, when OA lesions are end stage and little can be concluded about the factors that played a role in disease development. To overcome this limitation, over the past 50 years, numerous induced and spontaneous animal models have been utilized to study disease onset and progression, as well as to test novel therapeutic interventions. Reflecting the heterogeneity of OA itself, no single "gold standard" animal model for OA exists; thus, a challenge for researchers lies in selecting the most appropriate model to answer a particular scientific question of interest. This review provides general considerations for model selection, as well as important features of species such as mouse, rat, guinea pig, sheep, goat, and horse, which researchers should be mindful of when choosing the "best" animal model for their intended purpose. Special consideration is given to key variations in pathology among species as well as recommended guidelines for reporting the histologic features of each model.


Assuntos
Modelos Animais de Doenças , Osteoartrite/veterinária , Animais , Cães , Cabras , Cobaias , Cavalos , Humanos , Camundongos , Osteoartrite/patologia , Coelhos , Ratos , Ovinos
12.
J Chem Phys ; 142(11): 114301, 2015 Mar 21.
Artigo em Inglês | MEDLINE | ID: mdl-25796243

RESUMO

The carbenium ion with nominal formula [C,H4,O](+) is produced from methanol or ethylene glycol in a pulsed-discharge supersonic expansion source. The ion is mass selected, and its infrared spectrum is measured from 2000 to 4000 cm(-1) using laser photodissociation spectroscopy and the method of rare gas atom tagging. Computational chemistry predicts two isomers, the methanol and methylene-oxonium cations. Predicted vibrational spectra based on scaled harmonic and reduced dimensional treatments are compared to the experimental spectra. The methanol cation is the only isomer produced when methanol is used as a precursor. When ethylene glycol is used as the precursor, methylene-oxonium is produced in addition to the methanol cation. Theoretical results at the CCSD(T)/cc-pVTZ level show that methylene-oxonium is lower in energy than methanol cation by 6.4 kcal/mol, and is in fact the global minimum isomer on the [C,H4,O](+) potential surface. Methanol cation is trapped behind an isomerization barrier in our source, providing a convenient method to produce and characterize this transient species. Analysis of the spectrum of the methanol cation provides evidence for strong CH stretch vibration/torsion coupling in this molecular ion.

13.
Equine Vet J ; 47(4): 438-44, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24819047

RESUMO

REASONS FOR PERFORMING STUDY: Osteochondrosis (OC) is commonly diagnosed in young Standardbred racehorses but its effect on performance when surgically treated at a young age is still incompletely understood. This is especially true for Standardbred pacers, which are underrepresented in the existing literature. OBJECTIVE: To characterise the short- (2-year-old) and long-term (through 5-year-old) racing performance in Standardbred pacers and trotters after early surgical intervention (<17 months of age) for tarsal OC. STUDY DESIGN: Retrospective clinical study. METHODS: The study population consisted of related, age-matched Standardbred racehorses (n = 278; 151 pacers, 127 trotters) with (n = 133) or without (n = 145) one or more tarsal OC lesions. All OC-affected horses were treated surgically prior to being sold as yearlings. Data obtained from publicly available race records for each horse included starts, wins, finishes in the top 3 (win, place or show), earnings and fastest time. Comparisons between OC-affected and unaffected horses were made for the entire population and within gaits. A smaller related population (n = 94) had these performance measures evaluated for their 2-5-year-old racing seasons. RESULTS: Osteochondrosis status was associated with few performance measures. Trotters were at higher risk for lesions of the medial malleolus but lower risk for lesions of the distal intermediate ridge of the tibia than were pacers. Horses with bilateral OC lesions and lateral trochlear ridge (LTR) lesions started fewer races at 2 years of age than those with unilateral lesions or without LTR lesions. CONCLUSIONS: Osteochondrosis seemed to have minimal effect on racing performance in this cohort, although horses with bilateral and LTR lesions started fewer races at 2 years. There was evidence for different distribution of OC lesions among pacers and trotters, which should be explored further. Standardbreds undergoing early removal of tarsal OC lesions can be expected to perform equivalently to their unaffected counterparts.


Assuntos
Doenças dos Cavalos/cirurgia , Osteocondrose/veterinária , Tarso Animal/cirurgia , Envelhecimento , Animais , Feminino , Cavalos , Masculino , Osteocondrose/cirurgia , Estudos Retrospectivos , Esportes
14.
Acta Crystallogr F Struct Biol Commun ; 70(Pt 6): 823-6, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24915102

RESUMO

The enzyme 2,4'-dihydroxyacetophenone dioxygenase (or DAD) catalyses the conversion of 2,4'-dihydroxyacetophenone to 4-hydroxybenzoic acid and formic acid with the incorporation of molecular oxygen. Whilst the vast majority of dioxygenases cleave within the aromatic ring of the substrate, DAD is very unusual in that it is involved in C-C bond cleavage in a substituent of the aromatic ring. There is evidence that the enzyme is a homotetramer of 20.3 kDa subunits each containing nonhaem iron and its sequence suggests that it belongs to the cupin family of dioxygenases. By the use of limited chymotrypsinolysis, the DAD enzyme from Alcaligenes sp. 4HAP has been crystallized in a form that diffracts synchrotron radiation to a resolution of 2.2 Å.


Assuntos
Alcaligenes/enzimologia , Cristalografia por Raios X/métodos , Dioxigenases/química , Sequência de Bases , Cristalização , Primers do DNA , Hidrólise , Reação em Cadeia da Polimerase
16.
J Polym Sci A Polym Chem ; 52(23): 3324-3336, 2014 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-31223200

RESUMO

Linear, dibranched and miktoarm amphiphiles containing both hydrophobic and fluorophilic moieties were synthesized and characterized in an attempt to elucidate the relationship between semi-fluorinated amphiphile structure and aggregate behaviour in aqueous solution. For the linear and dibranched amphiphiles, there was an exponential decrease in critical aggregation concentration (CMC) and a logarithmic increase in core microviscosity with increasing length of the fluorocarbon segments; while the miktoarm architecture produced no notable trend in microviscosity or CMC. Furthermore, the linear and dibranched surfactants showed enhanced kinetic stability, dissociating more slowly in the presence of human serum than did either the dibranched or miktoarm amphiphiles. Finally, encapsulation studies with the hydrophobic drug paclitaxel (PTX) showed that the ability to solubilize and retain PTX increased with the presence and with the increasing size of the fluorocarbon moiety for both the linear and dibranched amphiphiles, while no such trend was observed for the miktoarm amphiphiles.

17.
Yearb Med Inform ; 8: 13-9, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23974543

RESUMO

OBJECTIVE: The field of clinical informatics has expanded substantially in the six decades since its inception. Early research focused on simple demonstrations that health information technology (HIT) such as electronic health records (EHRs), computerized provider order entry (CPOE), and clinical decision support (CDS) systems were feasible and potentially beneficial in clinical practice. METHODS: In this review, we present recent evidence on clinical informatics in the United States covering three themes: 1) clinical informatics systems and interventions for providers, including EHRs, CPOE, CDS, and health information exchange; 2) consumer health informatics systems, including personal health records and web-based and mobile HIT; and 3) methods and governance for clinical informatics, including EHR usability; data mining, text mining, natural language processing, privacy, and security. RESULTS: Substantial progress has been made in demonstrating that various clinical informatics methodologies and applications improve the structure, process, and outcomes of various facets of the healthcare system. CONCLUSION: Over the coming years, much more will be expected from the field. As we move past the "early adopters" in Rogers' diffusion of innovations' curve through the "early majority" and into the "late majority," there will be a crucial need for new research methodologies and clinical applications that have been rigorously demonstrated to work (i.e., to improve health outcomes) in multiple settings with different types of patients and clinicians.


Assuntos
Informática Médica , Sistemas de Registro de Ordens Médicas , Sistemas de Apoio a Decisões Clínicas , Atenção à Saúde , Registros Eletrônicos de Saúde , Humanos , Estados Unidos
18.
Osteoarthritis Cartilage ; 21(11): 1638-47, 2013 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-23954774

RESUMO

BACKGROUND: Osteochondrosis (OC) is a common developmental orthopedic disease affecting both humans and animals. Despite increasing recognition of this disease among children and adolescents, its pathogenesis is incompletely understood because clinical signs are often not apparent until lesions have progressed to end-stage, and examination of cadaveric early lesions is not feasible. In contrast, both naturally-occurring and surgically-induced animal models of disease have been extensively studied, most notably in horses and swine, species in which OC is recognized to have profound health and economic implications. The potential for a translational model of human OC has not been recognized in the existing human literature. OBJECTIVE: The purpose of this review is to highlight the similarities in signalment, predilection sites and clinical presentation of naturally-occurring OC in humans and animals and to propose a common pathogenesis for this condition across species. STUDY DESIGN: Review. METHODS: The published human and veterinary literature for the various manifestations of OC was reviewed. Peer-reviewed original scientific articles and species-specific review articles accessible in PubMed (US National Library of Medicine) were eligible for inclusion. RESULTS: A broad range of similarities exists between OC affecting humans and animals, including predilection sites, clinical presentation, radiographic/MRI changes, and histological appearance of the end-stage lesion, suggesting a shared pathogenesis across species. CONCLUSION: This proposed shared pathogenesis for OC between species implies that naturally-occurring and surgically-induced models of OC in animals may be useful in determining risk factors and for testing new diagnostic and therapeutic interventions that can be used in humans.


Assuntos
Osteocondrose/etiologia , Osteocondrose/veterinária , Animais , Humanos , Ossificação Heterotópica/complicações , Osteocondrose/diagnóstico , Osteocondrose/epidemiologia , Prevalência , Fatores de Risco , Especificidade da Espécie , Terminologia como Assunto
19.
Appl Clin Inform ; 4(1): 100-9, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23650491

RESUMO

BACKGROUND: In a prior study, we developed methods for automatically identifying associations between medications and problems using association rule mining on a large clinical data warehouse and validated these methods at a single site which used a self-developed electronic health record. OBJECTIVE: To demonstrate the generalizability of these methods by validating them at an external site. METHODS: We received data on medications and problems for 263,597 patients from the University of Texas Health Science Center at Houston Faculty Practice, an ambulatory practice that uses the Allscripts Enterprise commercial electronic health record product. We then conducted association rule mining to identify associated pairs of medications and problems and characterized these associations with five measures of interestingness: support, confidence, chi-square, interest and conviction and compared the top-ranked pairs to a gold standard. RESULTS: 25,088 medication-problem pairs were identified that exceeded our confidence and support thresholds. An analysis of the top 500 pairs according to each measure of interestingness showed a high degree of accuracy for highly-ranked pairs. CONCLUSION: The same technique was successfully employed at the University of Texas and accuracy was comparable to our previous results. Top associations included many medications that are highly specific for a particular problem as well as a large number of common, accurate medication-problem pairs that reflect practice patterns.


Assuntos
Mineração de Dados/métodos , Preparações Farmacêuticas , Bases de Dados de Produtos Farmacêuticos , Registros Eletrônicos de Saúde , Estudos de Viabilidade , Humanos , Reprodutibilidade dos Testes , Texas , Universidades
20.
Neuroophthalmology ; 37(5): 209-213, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-28167990

RESUMO

A 32-year-old woman with a history of biopsy-proven giant axonal neuropathy (GAN) and no past ocular history presented after failing a vision-screening test conducted by her primary doctor. Bilateral optic atrophy was observed on fundus examination with cup-to-disc ratio of 0.7. Kinetic visual fields showed moderate constriction in both eyes. Optical coherence tomography (OCT) of the retinal nerve fibre layer (RNFL) exhibited diffuse thinning, a pattern atypical of glaucomatous or nutritional optic atrophy and most likely secondary to the optic atrophy associated with GAN. Serial OCT to monitor RNFL thickness may offer a non-invasive means of monitoring progression in GAN for future therapeutic studies.

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