Interdisciplinary collaborative eye examinations to protect preterm infant neurodevelopment: a quality improvement project.

Introduction: Infants born <31 weeks gestational age with birth weight ≤ 1,500 grams receive routine eye examinations to screen for Retinopathy of Prematurity (ROP) while in the Neonatal Intensive Care Unit (NICU) to help prevent vision threatening complications; however, preterm infants' sensory systems are underdeveloped, and repeated exposure to painful stimuli is associated with worse developmental outcomes.

Methods: An interdisciplinary NICU team designed a collaborative eye exam model (CEEM) incorporating best practice recommendations for infant pain control during exams. Pain scores and vital signs were recorded before, during, and after exams.

Retinal injury identified by overhead-mounted optical coherence tomography in two young children with infantile-onset glaucoma.

Purpose: To report and characterize unexpected retinal findings identified by imaging with overhead-mounted optical coherence tomography (OCT) in 2 young children with infantile-onset glaucoma.

Methods: Children with glaucoma were imaged during clinically indicated examinations under anesthesia using overhead-mounted HRA+OCT Spectralis with Flex module (Flex-OCT, Heidelberg, Germany) from February 2017 through February 2022. Imagers prioritized scans of the peripapillary retinal nerve fiber layer (pRNFL), optic nerve head, and macula.

Intraoperative Mounted Optical Coherence Tomography Findings Following Reversal of Optic Nerve Head Cupping in Childhood Glaucoma.

Purpose: To examine the structural changes occurring in the optic nerve head (ONH) and macula in infants with childhood glaucoma and clinically observed ONH cupping reversal following intraocular pressure (IOP)-reducing glaucoma surgery, as captured by intra-operative spectral-domain optical coherence tomography (SD-OCT).

Design: Retrospective observational case series from an ongoing prospective cohort study.

Methods: Included were 18 eyes of 14 patients with childhood glaucoma.

Minimally invasive glaucoma surgery in childhood glaucoma.

Purpose Of Review: Minimally invasive glaucoma surgery (MIGS) has been shown to be safe and effective in treatment of mild to moderate glaucoma in adults, but reports in childhood glaucoma are limited. We review the available data concerning MIGS and discuss its potential role in childhood glaucoma management.

Recent Findings: Ab interno counterparts to circumferential ab externo trabeculotomy such as gonioscopy-assisted transluminal trabeculotomy (GATT) and Trab360 show promise in treatment of primary glaucomas as reported in a few retrospective case series.

Overhead Mounted Optical Coherence Tomography in Childhood Glaucoma Evaluation.

Unlabelled: PRéCIS:: Overhead mounted spectral-domain optical coherence tomography (OCT) enables high-quality imaging of the optic nerve and macula in childhood glaucoma, and is particularly useful when standard tabletop OCT has failed or is not possible.

Purpose: Tabletop OCT, integral to adult glaucoma management, can be limited in childhood glaucoma patients because of young age, poor cooperation, and/or technical challenges. To address these imaging difficulties, we determined the feasibility and quality of an overhead mounted unit in childhood glaucoma.

Structural and mechanistic insights into Hsp104 function revealed by synchrotron X-ray footprinting.

Hsp104 is a hexameric AAA ring translocase, which drives protein disaggregation in nonmetazoan eukaryotes. Cryo-EM structures of Hsp104 have suggested potential mechanisms of substrate translocation, but precisely how Hsp104 hexamers disaggregate proteins remains incompletely understood. Here, we employed synchrotron X-ray footprinting to probe the solution-state structures of Hsp104 monomers in the absence of nucleotide and Hsp104 hexamers in the presence of ADP or ATPγS (adenosine 5'--(thiotriphosphate)).

Home Tonometry Assists Glaucoma Drainage Device Management in Childhood Glaucoma.

Unlabelled: PRéCIS:: Home tonometry is useful in detecting tube-opening and alarming intraocular pressures (IOPs) after Baerveldt glaucoma drainage device (GDD) implantation in childhood glaucoma, allowing for timely physician response and individualized patient care.

Purpose: The postoperative management of the nonvalved Baerveldt GDD presents challenges in pediatric patients due to widely variable IOP often occurring perioperatively. We evaluated the use of home tonometry in the management of Baerveldt implants for refractory childhood glaucoma.

Mechanistic Insights into Hsp104 Potentiation.

Potentiated variants of Hsp104, a protein disaggregase from yeast, can dissolve protein aggregates connected to neurodegenerative diseases such as Parkinson disease and amyotrophic lateral sclerosis. However, the mechanisms underlying Hsp104 potentiation remain incompletely defined. Here, we establish that 2-3 subunits of the Hsp104 hexamer must bear an A503V potentiating mutation to elicit enhanced disaggregase activity in the absence of Hsp70.

The Hsp104 N-terminal domain enables disaggregase plasticity and potentiation.

The structural basis by which Hsp104 dissolves disordered aggregates and prions is unknown. A single subunit within the Hsp104 hexamer can solubilize disordered aggregates, whereas prion dissolution requires collaboration by multiple Hsp104 subunits. Here, we establish that the poorly understood Hsp104 N-terminal domain (NTD) enables this operational plasticity.

Conserved distal loop residues in the Hsp104 and ClpB middle domain contact nucleotide-binding domain 2 and enable Hsp70-dependent protein disaggregation.

The homologous hexameric AAA(+) proteins, Hsp104 from yeast and ClpB from bacteria, collaborate with Hsp70 to dissolve disordered protein aggregates but employ distinct mechanisms of intersubunit collaboration. How Hsp104 and ClpB coordinate polypeptide handover with Hsp70 is not understood. Here, we define conserved distal loop residues between middle domain (MD) helix 1 and 2 that are unexpectedly critical for Hsp104 and ClpB collaboration with Hsp70.