Secondary Intracranial Hypertension in Pediatric Patients With Cryopyrin-Associated Periodic Syndrome.

Background: Cryopyrin-associated periodic syndrome is characterized by periodic fever, rash, and joint pain. Papilledema rarely occurs. We present our series of patients with cryopyrin-associated periodic syndrome who clinically met the diagnostic criteria for Muckle-Wells syndrome and our experience with secondary intracranial hypertension.

Surgical intervention in pediatric intracranial hypertension: incidence, risk factors, and visual outcomes.

Purpose: To evaluate the incidence of surgical intervention in pediatric intracranial hypertension (IH), evaluate the visual outcomes of surgically managed patients, and identify potential predictors for surgical intervention.

Methods: The medical records of patients with primary and secondary IH at Nationwide Children's Hospital from 2010 to 2017 were reviewed retrospectively. Presenting characteristics of medically and surgically managed patients were compared, and the clinical courses of surgically managed patients were reviewed.

Diagnostic criteria in pediatric intracranial hypertension.

Background: The modified Dandy criteria and the newer diagnostic criteria for pseudotumor cerebri syndrome (PTCS) are both used to diagnose intracranial hypertension (IH). In comparison to the modified Dandy criteria, the PTCS criteria stratify the IH diagnosis into definite, probable, and suggested categories, exclude clinical symptoms, and use radiologic evidence for diagnosis. There is a lack of consensus on which criteria should be used in the pediatric population.

Incidence and Demographics of Pediatric Intracranial Hypertension.

Objective: We analyzed the incidence and demographic features of pediatric intracranial hypertension.

Methods: Inpatient and outpatient encounters of children aged 18 years or younger who were diagnosed with primary (idiopathic) or secondary intracranial hypertension between January 2010 and December 2013 were identified. Data were collected from a subspecialty clinic devoted to intracranial hypertension and the sole children's hospital in a large Midwestern city of the United States.

Pediatric Intracranial Hypertension.

Primary (idiopathic) intracranial hypertension has been considered to be a rare entity, but with no precise estimates of the pediatric incidence in the United States. There have been attempts to revise the criteria over the years and adapt the adult criteria for use in pediatrics. The clinical presentation varies with age, and symptoms tending to be less obvious in younger individuals.

Sixth Nerve Palsy in Paediatric Intracranial Hypertension.

The purpose of this study was to report the incidence and describe the characteristics of sixth cranial nerve (CN VI) palsy in paediatric patients with intracranial hypertension (IH). A retrospective chart review of central Ohio children diagnosed with IH over the 3-year period from 2010 to 2013 was conducted. IH without identifiable cause was defined as idiopathic intracranial hypertension (IIH), whereas IH with identifiable pathologic aetiology was deemed secondary intracranial hypertension (SIH).

Long-term visual acuity outcomes in patients with uveal melanoma treated with 125I episcleral OSU-Nag plaque brachytherapy.

Purpose: To report our experience in long-term follow-up of ocular melanoma patients treated with custom OSU-Nag eye plaques using (125)I sources.

Methods: A retrospective chart review was conducted for 113 consecutive ocular melanoma patients with follow-up visual acuity data who were treated with OSU-Nag plaque episcleral brachytherapy at The Ohio State University Medical Center from 1994 to 2009. Visual acuity, complication data, and recurrence rates were recorded up to 120 months after brachytherapy.

Drosophila muller f elements maintain a distinct set of genomic properties over 40 million years of evolution.

The Muller F element (4.2 Mb, ~80 protein-coding genes) is an unusual autosome of Drosophila melanogaster; it is mostly heterochromatic with a low recombination rate. To investigate how these properties impact the evolution of repeats and genes, we manually improved the sequence and annotated the genes on the D.

Intracranial hypertension without headache in children.

We aimed to determine the frequency of intracranial hypertension without headache in children. We retrospectively analyzed patients evaluated in a pediatric intracranial hypertension referral center. Patients were divided into 2 groups depending on whether they complained of headache at the time of presentation.

Periocular hemangiomas and lymphangiomas.

Infantile hemangiomas are the most common benign tumor of childhood. Lymphangiomas are benign hamartomatous vascular tumors. Both lesions can be problematic when located in the periocular region.

Screening and characterization of Staphylococcus aureus from ophthalmology clinic surfaces: a proposed surveillance tool.

Purpose: To screen environmental surfaces of an outpatient ophthalmic clinic for methicillin-susceptible and methicillin-resistant Staphylococcus aureus (MSSA and MRSA); to identify the most commonly contaminated surfaces and to phenotype and genotype all collected isolates.

Design: A single institution, 1-year prospective environmental study.

Methods: Commonly touched surfaces in examination rooms and common areas were targeted and sampled on a quarterly basis for 1 year.

Abnormal vascularization in mouse retina with dysregulated retinal cholesterol homeostasis.

Several lines of evidence suggest a link between age-related macular degeneration and retinal cholesterol maintenance. Cytochrome P450 27A1 (CYP27A1) is a ubiquitously expressed mitochondrial sterol 27-hydroxylase that plays an important role in the metabolism of cholesterol and cholesterol-related compounds. We conducted a comprehensive ophthalmic evaluation of mice lacking CYP27A1.

Spatial distribution of the pathways of cholesterol homeostasis in human retina.

Background: The retina is a light-sensitive tissue lining the inner surface of the eye and one of the few human organs whose cholesterol maintenance is still poorly understood. Challenges in studies of the retina include its complex multicellular and multilayered structure; unique cell types and functions; and specific physico-chemical environment.

Methodology/principal Findings: We isolated specimens of the neural retina (NR) and underlying retinal pigment epithelium (RPE)/choroid from six deceased human donors and evaluated them for expression of genes and proteins representing the major pathways of cholesterol input, output and regulation.

A unique case of autoimmune retinopathy associated with anti-alpha-enolase antibodies.

Background. We report a case of autoimmune retinopathy associated with anti-alpha-enolase antibodies with unique manifestations. Methods.

Quantification of cholesterol-metabolizing P450s CYP27A1 and CYP46A1 in neural tissues reveals a lack of enzyme-product correlations in human retina but not human brain.

Cytochrome P450 enzymes (CYP or P450) 46A1 and 27A1 play important roles in cholesterol elimination from the brain and retina, respectively, yet they have not been quantified in human organs because of their low abundance and association with membrane. On the basis of our previous development of a multiple reaction monitoring (MRM) workflow for measurements of low-abundance membrane proteins, we quantified CYP46A1 and CYP27A1 in human brain and retina samples from four donors. These enzymes were quantified in the total membrane pellet, a fraction of the whole tissue homogenate, using ¹⁵N-labled recombinant P450s as internal standards.

Cholestenoic Acid is an important elimination product of cholesterol in the retina: comparison of retinal cholesterol metabolism with that in the brain.

Purpose: Accumulating evidence indicates a link between cholesterol and age-related macular degeneration. Yet, little is known about cholesterol elimination from the retina and retinal pigment epithelium (RPE), the two layers that are damaged in this blinding disease. Several different pathways of enzymatic cholesterol removal exist in extraocular tissues.

Contrast sensitivity of cats and humans in scotopic and mesopic conditions.

Human contrast sensitivity in low scotopic conditions is regulated according to the deVries-Rose law. Previous cat behavioral data, as well as cat and mice electrophysiological data, have not confirmed this relationship. To resolve this discrepancy at the behavioral level, we compared sensitivity in dim light for cats and humans in parallel experiments using the same visual stimuli and similar behavioral paradigms.