goniodysgenesis

淋病发生
  • 文章类型: Journal Article
    这项研究的目的是(i)评估患有与前节发育不全(ASD)相关的先天性青光眼的犬眼球中虹膜前单核细胞和纤维血管膜的患病率,原发性青光眼与性腺发育(GD)相关,和继发性青光眼,(ii)按品种检查单核细胞和纤维血管膜之间的关联,性别,光学显微镜检查的年龄和组织病理学眼部变化。
    回顾了由于失明和未控制的青光眼而导致眼睛摘除的狗的记录。青光眼在临床和组织学上分为三组:先天性/ASD,初级/GD,和继发性青光眼。对各组的虹膜前膜(单核细胞或纤维血管)的存在与否和类型以及其他眼内组织学发现进行了回顾和统计学比较。
    总共,包括108只犬球(101只狗)。在19例先天性/ASD中,有10例通过光学显微镜鉴定出虹膜前单核细胞膜,40个小学中有29个,49个继发性青光眼球中有23个。在19例先天性/ASD中观察到3例血管膜,小学40人中有9人,49个继发性青光眼球中有24个。膜的类型和品种之间没有关联,性别,或年龄。外周前粘连在有纤维血管膜的球体中更常见,葡萄膜萎缩在具有单细胞膜的球体中更为常见。
    前虹膜单细胞膜在所有类型的犬青光眼中都很常见。在原发性青光眼的情况下,最容易用光学显微镜识别它们,在其他形式的青光眼中,它们被虹膜前纤维血管膜掩盖。
    UNASSIGNED: The objectives of this study were to (i) evaluate the prevalence of pre-iridal monocellular and fibrovascular membranes in canine globes affected with congenital glaucoma associated with anterior segment dysgenesis (ASD), primary glaucoma associated with goniodysgenesis (GD), and secondary glaucoma, and (ii) examine the associations between monocellular and fibrovascular membranes by breed, gender, age and histopathologic ocular changes on light microscopic examination.
    UNASSIGNED: Records of dogs who had eyes enucleated due to blindness and uncontrolled glaucoma were reviewed. Glaucoma was categorized clinically and histologically into three groups: congenital/ASD, primary/GD, and secondary glaucoma. The presence or absence and type of pre-iridal membrane (monocellular or fibrovascular) and other intraocular histologic findings were reviewed and compared statistically for each group.
    UNASSIGNED: In total, 108 canine globes (101 dogs) were included. Pre-iridal monocellular membranes were identified with light microscopy in 10 out of 19 congenital/ASD, 29 out of 40 primary, and 23 out of 49 secondary glaucoma globes. Fibrovascular membranes were observed in 3 out of 19 congenital/ASD, 9 out of 40 in primary, and 24 out of 49 secondary glaucoma globes. There were no associations between the type of membrane and breed, gender, or age. Peripheral anterior synechiae were more common in globes with fibrovascular membranes, and uveal atrophy was more common in globes with monocellular membranes.
    UNASSIGNED: Pre-iridal monocellular membranes are common in all types of canine glaucoma. They are identified with light microscopy most easily in cases of primary glaucoma, and they are masked by pre-iridal fibrovascular membranes in other forms of glaucoma.
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  • 文章类型: Journal Article
    原发性青光眼是一种痛苦,进步,和许多犬科动物品种报道的致盲疾病,在没有先兆眼内疾病的情况下,以眼内压(IOP)升高为特征。患有原发性青光眼的狗的临床观察表明,除了IOP升高外,许多受影响的眼睛还并发眼内炎症。在这项工作中,我们总结了目前有关炎症与狗原发性青光眼的知识,回顾专注于遗传学的研究,生理学,组织病理学,眼液的生物分析,治疗学,青光眼患者的临床结局。通过破坏血水和血视网膜屏障,颜料分散体,房水和泪膜的生化变化,犬原发性青光眼的发病机制似乎涉及不同程度的炎症变化,并从眼睛的前部到后部产生各种后果。其中,炎症通过减少虹膜角膜角水平的房水流出进一步影响IOP,并通过促进神经元变性加速视力丧失。因此,眼部炎症和IOP升高的恶性循环可能需要使用抗炎药作为原发性青光眼犬治疗方案的核心组成部分,治疗性(即,主动青光眼)或预防性地在未受影响的对侧眼中。
    Primary glaucoma is a painful, progressive, and blinding disease reported in many canine breeds, characterized by intraocular pressure (IOP) elevation in the absence of antecedent intraocular disease. Clinical observations of dogs with primary glaucoma suggest that many affected eyes develop concurrent intraocular inflammation in addition to elevated IOP. In this work, we summarize the current knowledge that relates inflammation to primary glaucoma in dogs, reviewing studies focused on genetics, physiology, histopathology, bioanalysis of ocular fluids, therapeutics, and clinical outcomes of glaucomatous patients. Through disruption of the blood-aqueous and blood-retinal barriers, pigment dispersion, and biochemical changes to the aqueous humor and tear film, the pathogenesis of canine primary glaucoma appears to involve inflammatory changes to various extents and with various consequences from the front to the back of the eye. Among others, inflammation further impacts IOP by reducing aqueous humor outflow at the level of the iridocorneal angle and accelerates vision loss by promoting neuronal degeneration. As such, the vicious cycle of ocular inflammation and IOP elevation might warrant the use of anti-inflammatory medications as a core component of the treatment regime for dogs with primary glaucoma, either therapeutically (i.e., actively glaucomatous eye) or prophylactically in the yet unaffected contralateral eye.
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  • 文章类型: Journal Article
    目的:评估对Samoyed幼犬(6-14周龄)进行的房角镜检查是否是成年(>11月龄)的可靠预测结果。
    方法:进行了回顾性医疗记录搜索,以确定曾接受房角镜检查的Samoyed犬为小狗(6-14周龄)。作为成年人(>11个月大)进行第二次前瞻性房角镜检查,并比较结果。根据两个特征评估房角镜检查:不受果胶韧带异常(PLA)和ICA宽度影响的虹膜角膜角(ICA)百分比,PLA结果分类为未受影响(≥$$\\ge$$75%开放),中度影响(50%-74%开放),和严重影响(<50%开放)和宽度分类为宽,中度,狭窄,封闭的团体。创建了一个多变量模型,考虑了PLA、角宽度,性别,小狗的年龄,中性状态,和考试之间的时间。
    结果:77只Samoyed犬(154只眼)的眼睛符合纳入标准。在评估PLA时,90%的狗与小狗和成年狗具有相同的分类。评估ICA宽度时,53.2%的狗具有与小狗和成年狗相同的宽度分类。有强有力的证据表明,小狗和成年结果之间的差异随着检查之间的时间而增加(p=0.03)。
    结论:幼犬不受PLA影响,具有宽ICA角,作为成年人,可能会保留这些特征。选择具有理想性腺性状的幼犬可能有助于育种者选择可能保留这些性状的狗作为成年繁殖。这可以减少该品种中青光眼的发病率。应该在双眼中进行小狗检查。在繁殖之前对成年人进行检查仍然是谨慎的。
    OBJECTIVE: To assess whether gonioscopy performed on Samoyed puppies (6-14 weeks of age) is a reliable predictor of their gonioscopy results as adults (>11 months of age).
    METHODS: A retrospective medical record search was performed to identify Samoyed dogs that had undergone gonioscopy as a puppy (6-14 weeks of age). A second prospective gonioscopy examination was performed as an adult (>11 months of age) and the results were compared. Gonioscopy was assessed on two characteristics: percent of iridocorneal angle (ICA) unaffected by pectinate ligament abnormalities (PLA) and ICA width, with PLA results categorized into unaffected ( ≥ 75% open), moderately affected (50%-74% open), and severely affected (<50% open) and the width categorized into wide, moderate, narrow, and closed groups. A multivariate model was created that considered factors such as PLA, angle width, sex, puppy age, neuter status, and time between examinations.
    RESULTS: The eyes of 77 Samoyed dogs (154 eyes) met inclusion criteria. When assessing PLA, 90% of dogs had the same categorization as a puppy and as an adult. When assessing ICA width, 53.2% of dogs had the same width classification as a puppy and as an adult. There is strong evidence that variation between puppy and adult results increases with time between examination (p = .03).
    CONCLUSIONS: Puppies unaffected by PLA and with a wide ICA angle, are likely to retain these features as adults. Selection of puppies with desirable gonioscopic traits may help breeders to choose dogs likely to retain these traits as adults for breeding. This may reduce the incidence of glaucoma in the breed. Puppy examinations should be performed in both eyes. Examination of adults prior to breeding remains prudent.
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  • 文章类型: Case Reports
    目的:描述一例先天性青光眼患者(Pantheratigris)。
    方法:一只8个月大的完整雌虎因怀疑右眼青光眼而转诊。右眼是布替米克和中度巩膜上注射,周围浅表角膜新生血管形成,中度角膜水肿,和一个固定的扩大瞳孔。由于成熟的白内障,没有反射。全身麻醉下的回弹眼压测定显示右眼和左眼分别为70mmHg和21mmHg,分别。
    方法:进行经结膜摘除术,并对眼球进行组织病理学检查。
    结果:组织病理学显示巩膜薄,形成无孔和发育不良的虹膜角膜角的无定形材料,发育不良晶状体严重的前后压缩,包膜下上皮增生,和摩根球,和节段性中度视网膜萎缩。周期性酸性希夫染色突出了Descemet膜的分段扩张。Masson三色染色突出了前虹膜胶原膜。
    结论:老虎的年龄和组织病理学结果与先天性性腺发育是一致的。这是老虎先天性青光眼的第一个已知报告。
    OBJECTIVE: To describe a case of congenital glaucoma in atiger (Panthera tigris).
    METHODS: An 8-month-old intact female tiger wasreferred for suspected glaucoma of the right eye. The right eye was buphthalmicwith moderate episcleral injection, circumferential superficial cornealneovascularization, moderate corneal edema, and a fixed dilated pupil. Tapetalreflection was absent due to a mature cataract. Rebound tonometry under generalanesthesia revealed 70 mmHg and 21 mmHg in the right and left eye, respectively.
    METHODS: A trans-conjunctival enucleation was performedand the globe was submitted for histopathology.
    RESULTS: Histopathology revealed a thin sclera, amorphousmaterial contouring an imperforate and hypoplastic iridocorneal angle, ahypoplastic lens with severe anterior-posterior compression, subcapsularepithelial hyperplasia, and Morganian globules, and segmental moderate retinalatrophy. Periodic acid-Schiff stain highlighted segmental dilations of theDescemet\'s membrane. Masson trichrome stain highlighted a pre-irido collagenmembrane.
    CONCLUSIONS: The tiger\'s age and histopathologic findingsare consistent with congenital goniodysgenesis. This is the first known reportof congenital glaucoma in a tiger.
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  • 文章类型: Case Reports
    先天性外翻葡萄膜(CEU)是眼前段胚胎发育的罕见异常。我们报告了一例未确诊的CEU的5岁儿童,因急性闭角发作而接受紧急治疗。
    方法:一名5岁儿童被紧急转诊以评估伴有右眼散瞳和严重头痛的不等眼。最初在儿科急诊科进行了造影剂注射的脑成像,并排除了中枢神经系统病理。右眼的初始检查显示眼内压(IOP)为37mmHg,角膜水肿,先天性外翻葡萄膜,瞳孔阻滞散瞳,房角镜检查上的闭合角度,和一个清晰的镜头。左眼的检查并不明显,没有可见的CEU。最初的管理包括局部青光眼滴剂和miotics以及10mg/kg/d的乙酰唑胺的药物治疗。全身麻醉下的重新评估显示持续的瞳孔散大,瞳孔阻滞没有消退。在对药物治疗没有完全反应的情况下进行过滤手术,允许控制IOP而不滴落和完全消退的角膜水肿。
    结论:CEU是一种罕见的畸形,和压力并发症在小儿青光眼病例中所占比例微不足道。在这种潜在的短期背景下,锐角闭合的急性表现,然而,在非常年幼的儿童中,很难在很大的痛苦中进行检测和管理。儿科文献中仅报道了一例类似病例。
    结论:急性闭角并发CEU是特殊的,在儿科背景下难以诊断。当出现眼内压升高的临床迹象时,应告知患有这种易感疾病的儿童的父母需要紧急咨询。
    Congenital ectropion uveae (CEU) is a rare anomaly of the embryonic development of the anterior segment of the eye. We report the case of a 5-year-old child with an undiagnosed CEU who was treated urgently for an acute angle closure attack.
    METHODS: A 5-year-old child was referred urgently for evaluation of anisocoria with mydriasis of the right eye and severe headache. Brain imaging with contrast injection was initially performed in the pediatric emergency department and ruled out central nervous system pathology. The initial examination of the right eye revealed an intraocular pressure (IOP) of 37mmHg, corneal edema, congenital ectropion uveae, mydriasis with pupillary block, a closed angle on gonioscopy, and a clear lens. The examination of the left eye was unremarkable, with no visible CEU. The initial management consisted of medical treatment with topical glaucoma drops and miotics and acetazolamide at 10mg/kg/d. Re-evaluation under general anesthesia showed persistent mydriasis and no resolution of the pupillary block. Filtering surgery was performed in the absence of a complete response to medical treatment, allowing control of IOP without drops and complete regression of the corneal edema.
    CONCLUSIONS: CEU is a rare malformation, and pressure complications represent an insignificant proportion of pediatric glaucoma cases. The acute presentation of acute angle closure in this potentially blinding short-term setting, however, makes detection and management difficult in very young children in a great deal of pain. Only one similar case has been reported in the pediatric literature.
    CONCLUSIONS: Acute angle closure complicating CEU is exceptional and difficult to diagnose in a pediatric context. Parents of children with this predisposing condition should be informed of the need to consult urgently when clinical signs of elevated intraocular pressure appear.
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  • 文章类型: Case Reports
    目的:描述通过parsplan入路,通过内囊光凝(ECP)手术成功控制难治性青光眼和视力。
    方法:一个8岁的孩子,女性斯派,白内障超声乳化吸除人工晶状体放置(IOL)后,患有青光眼的混合品种的犬,难以最大限度的药物治疗和双眼的Ahmed房角植入(OU)。
    方法:对于首次青光眼介入手术,白内障手术后进行双侧房角瓣植入术.白内障超声乳化术后2天,在左眼(OS)植入房角瓣膜,和右眼超声乳化术后14天(OD)。实现了短期眼内压(IOP)控制。然而,青光眼在超声乳化术后41天复发,并用23号氩激光内窥镜通过平坦部途径进行了腔内光凝术,以治疗睫状体300°。
    结果:在15个月的随访期间,ECP平坦部方法成功地获得了IOP控制和视力保存。术中无并发症发生。术后短期并发症包括眼内炎症伴纤维蛋白和术后高眼压。在最后一次随访检查时保持眼压控制和视力保持,术后15个月平坦部ECP。
    结论:通过平坦部方法进行的子宫内膜光凝术实现了长期(>1年)IOP控制,保存的视力,并减少了先前Ahmed房角植入术治疗未控制的青光眼失败后的抗青光眼药物数量。这是Ahmed房角植入后,通过平坦部平坦部方法进行内囊光凝术治疗不受控制的青光眼的第一例报道。有了适当的技能,这种新颖的手术方法可以被认为是犬科患者青光眼的手术干预。
    OBJECTIVE: To describe successful intraocular pressure control of refractory glaucoma and vision preservation with an endocyclophotocoagulation (ECP) procedure by pars plana approach.
    METHODS: An 8-year-old, female-spayed, mixed-breed goniodysgenic dog with glaucoma following phacoemulsification with intraocular lens placement (IOL) refractory to maximized medical therapy and Ahmed gonioimplantation in both eyes (OU).
    METHODS: For the first glaucoma interventional surgery, bilateral goniovalve implantation was performed following cataract surgery. A goniovalve was implanted in the left eye (OS) 2-days post-phacoemulsification, and 14-days post-phacoemulsification in the right eye (OD). Short-term intraocular pressure (IOP) control was achieved. However, glaucoma recurred 41-days post-phacoemulsification OU, and an endocyclophotocoagulation by pars plana approach was performed OU to treat 300° of the ciliary body with a 23-gauge argon laser endoscope.
    RESULTS: Th ECP pars plana approach was successful in obtaining IOP-control and vision preservation during a 15-month follow-up period. No intraoperative complications were encountered. Short-term postoperative complications included intraocular inflammation with fibrin and postoperative ocular hypertension. Intraocular pressure-control and vision preservation were maintained at the last follow-up examination, 15 months postoperative pars plana ECP.
    CONCLUSIONS: Endocyclophotocoagulation by pars plana approach achieved long-term (>1 year) IOP-control, preserved vision, and reduced the number of anti-glaucoma medications following failure of previous Ahmed gonioimplantation for uncontrolled glaucoma. This is the first case report of an endocyclophotocoagulation procedure by a pars plana approach for uncontrolled glaucoma following Ahmed gonioplantation. With the appropriate skill set, this novel surgical approach can be considered as a surgical intervention for glaucoma in canine patients.
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  • 文章类型: Case Reports
    目的:常染色体显性遗传性中央凹发育不全(FVH1)是一种与PAX6基因突变相关的罕见疾病。作为一个孤立的疾病实体,FVH1不包括眼部疾病,如无虹膜,小眼症,白化病,和色盲.然而,它仅包括孤立的中央凹发育不全和中央凹发育不全伴老年性白内障。本报告的目的是介绍我们在来自两个FVH1家庭的四名患者中的发现,这些患者没有可见的眼科黄斑异常。
    方法:回顾两个FVH1家族的医疗记录和PAX6基因突变的遗传确认。
    方法:眼底照片,光学相干层析成像(OCT)和OCT血管造影(OCTA)图像,并确定了裂隙灯眼前节的发现。确定PAX6基因的突变类型。
    结果:一名3岁女孩(患者1)有视力发育受损的体征和症状,没有其他视网膜异常OU。OCT图像显示一个浅的中央凹窝,OCTA显示中央凹无血管区的缺失。第二名患者(患者2)是一名6岁的女孩,患有单侧轻度白内障和浅中央凹OU。在她无症状的母亲(患者3)和外祖父(患者4)中发现了类似的浅中央凹凹陷。尽管虹膜和后眼底正常,所有FVH1患者均有性腺发育.PAX6基因的遗传检测显示,患者1具有新的杂合突变(p。Asn365Lys)作为从头突变,患者2、3和4具有新的杂合突变(p。Pro20Ser)。
    结论:PAX6基因杂合突变可导致FVH1出现接近正常的黄斑。FVH1很难诊断,但是对中央凹结构和脉管系统的详细观察,检测是否存在淋病,有助于识别FVH1患者。
    OBJECTIVE: Autosomal dominant foveal hypoplasia (FVH1) is a rare disorder associated with mutations in the PAX6 gene. As an isolated disease entity, FVH1 does not include ocular disorders such as aniridia, microphthalmia, albinism, and achromatopsia. However, it only includes isolated foveal hypoplasia and foveal hypoplasia with presenile cataract. The purpose of this report is to present our findings in four patients from two families with FVH1 without visible ophthalmic macular abnormalities.
    METHODS: A review of the medical records of two families with FVH1 and genetic confirmation of mutations in the PAX6 gene.
    METHODS: Fundus photographs, optical coherence tomographic (OCT) and OCT angiographic (OCTA) images, and slit-lamp anterior segment findings were determined. The type of mutation of the PAX6 gene was determined.
    RESULTS: A 3-year-old girl (Patient 1) had signs and symptoms of an impairment in the development of vision without other retinal abnormalities OU. OCT images showed a shallow foveal pit, and OCTA showed the absence of the foveal avascular zone. The second patient (Patient 2) was a 6-year-old girl with unilateral mild cataract and shallow foveal pits OU. Similar shallow foveal pits were found in her asymptomatic mother (Patient 3) and maternal grandfather (Patient 4). Although the iris and posterior fundus were normal, all patients with FVH1 had goniodysgenesis. Genetic testing of the PAX6 gene revealed that Patient 1 had a novel heterozygous mutation (p.Asn365Lys) as a de novo mutation, and Patients 2, 3 and 4 had a novel heterozygous mutation (p.Pro20Ser).
    CONCLUSIONS: Heterozygous mutations in the PAX6 gene can cause FVH1 with nearly normal appearing macula. FVH1 is difficult to diagnose, but detailed observations of the foveal structure and vasculature, and detecting the presence of goniodysgenesis can be helpful in identifying patients with FVH1.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    OBJECTIVE: Canine primary closed-angle glaucoma (PCAG) is a complex disease caused by multiple genetic factors. A c.590G>A variant in OLFML3 was recently reported to be a candidate for pectinate ligament abnormality (PLA) and PCAG in the Border Collie. We investigated the association of this variant with PLA and PCAG in Border Collies from the United Kingdom.
    METHODS: The OLFML3 variant was genotyped in 106 Border Collies comprising 90 with normal eyes (controls) and 16 with PLA (n = 11) and/or PCAG (n = 5) (cases). Genotyping was performed in an additional 103 Border Collies to estimate variant frequency within the population. To investigate the association of the variant with disease in other breeds, genotyping was performed in 337 non-Border Collies with PLA and/or PCAG.
    RESULTS: Of the 90 controls, 71 were homozygous for the wild-type allele, two were homozygous for the variant, and 17 were heterozygous. Of the 16 cases, three were homozygous for the wild-type allele, 11 were homozygous for the variant, and two were heterozygous. The association of the variant allele with disease was significant (P = 1.1 x 10-9 ). We estimated the frequency of this variant to be 4.4% within the United Kingdom Border Collie population, and it was not identified in clinically affected dogs of any other breed.
    CONCLUSIONS: This study confirms the association of the OLFML3 variant with PLA and PCAG in Border Collies from the United Kingdom. DNA testing for the variant and selective breeding can reasonably be expected to result in a reduction of PLA and PCAG prevalence in the breed.
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  • 文章类型: Journal Article
    The purpose of this study is to describe the short-term incidence, clinical features, and management of glaucoma in children after successful surgery for stage 4 retinopathy of prematurity (ROP).
    The retrospective study included all eyes undergoing successful surgery for stage 4 ROP with good outcomes at a tertiary eye care center between June 2014 and June 2016. Cases developing postoperative glaucoma underwent examination under anesthesia for measurement of intraocular pressures (IOP), corneal diameters, Retcam-assisted fundus imaging, and gonioscopy. Outcomes of glaucoma management were evaluated.
    Hundred eyes of 70 babies underwent successful surgery for stage 4 ROP (with postoperative attached retina, and minimal sequelae) with minimum follow-up of 15 months. Six eyes (6%) developed postoperative glaucoma. Of these, four eyes had undergone lens-sparing vitrectomy and two were managed with lensectomy and vitrectomy (LV). Median time duration for development of glaucoma after primary vitreous surgery was 17.5 weeks. Two cases could be managed with topical IOP-lowering agents alone, whereas four required filtering surgeries (trabeculotomy with trabeculectomy and 0.04% mitomycin C [MMC] application). Average IOP decreased from 25 ± 2.36 to 12.2 ± 2.05 mmHg at 12 months from glaucoma diagnosis.
    Glaucoma is a potential adverse event following successful vitreous surgery for stage 4 ROP. A combined trabeculotomy-trabeculectomy along with MMC gives favorable outcome.
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