The Robison D. Harley, MD Childhood Glaucoma Research Network International Pediatric Glaucoma Registry: The First 872 Cases.

PURPOSE: To report on epidemiologic data from an international, centralized pediatric glaucoma database of 872 patients, focusing on genetic and clinically significant factors. DESIGN: Database study utilizing retrospective analysis. SUBJECTS: 872 children, both female and male, were included in the database. After accounting for database coding errors, data from 865 patients with pediatric glaucoma were analyzed. Number of eyes analyzed fluctuated for each variable. METHODS: The registry is an open access, no charge, REDCap database. Participating clinical centers input data with local Institutional Review Board approval and subsequently have access for research purposes. We retrospectively reviewed 872 patients, comparing demographics, family history, country, disease presentation, and CGRN diagnoses. Analyses for each variable were conducted in SPSS Software v.28.0. Chi-square analyses were performed for nominal data, and ordinal and continuous data were analyzed using Mann-Whitney test, analysis of variance or Kruskal-Wallis tests with multiple comparisons. MAIN OUTCOME MEASURES: CGRN glaucoma type and markers of clinical severity by country (laterality, cup-to-disc ratio (CTD), corneal diameter, opacification, edema; visual acuity (VA), intraocular pressure (IOP), Haab striae, axial length). RESULTS: 20 clinical sites from 10 countries entered data. Centers in the USA, India, and Iran input the most data. In the USA, open angle glaucoma following cataract surgery was most common, while in India and Iran it was primary congenital glaucoma neonatal onset. Bilateral disease was more frequent in India and Iran compared to the USA (X2 = 50.6, p<0.001). Clinical measures of severity were typically worse in India compared to the USA. This included increased CTD (X2 = 24.0, p = 0.002), increased corneal diameter (X2 = 8.9, p = 0.01), presence of corneal opacification (X2 = 10.7, p = 0.001), presence of corneal edema (X2 = 11.7, p<0.001), and worse VA (U = 873.5, p<0.001). IOP and presence of Haab striae were not associated with country (p>0.05), while axial length was increased in the USA by an average of 1.04mm (U = 5787, p = 0.002). CONCLUSIONS: This registry has potential to advance our understanding of pediatric glaucoma. Differences in family history, disease presentation, and glaucoma type suggest unique country phenotypes. Registry expansion may allow for insight into best practices for pediatric glaucoma.

Clinical outcomes in paediatric tubulointerstitial nephritis and uveitis syndrome (TINU).

PURPOSE: To evaluate the clinical presentation, course, and outcomes of uveitis in paediatric patients with tubulointerstitial nephritis and uveitis syndrome (TINU). METHODS: Multicentric Retrospective Cohort Study 110 patients ≤21 years of age diagnosed with TINU from 10 sites across the United States and Canada. Clinical diagnosis of TINU required uveitis diagnosed by an ophthalmologist, elevated serum creatinine (SCr) and elevated urine ß2-microglobulin (ß2M) or abnormal urinalysis. Renal biopsy and systemic illness were not mandatory. Univariate and multivariate analysis was performed to analyse risk factors and treatment modalities. RESULTS: Median age was 13 years (Range (5.9-18.4); 52% male); median follow-up, 1.6 years (IQR 0.98-4.02). Uveitis was symptomatic in 90%, with bilateral anterior uveitis in 94%. Ninety-two (84%) patients required immunomodulatory treatment (IMT). Methotrexate (n = 44) and mycophenolate mofetil (n = 39) were the first agents after oral corticosteroids. 45% required addition of biologic agents (Adalimumab [n = 33], Infliximab [n = 8]). Younger age (p = 0.018), male sex (p = 0.011), and higher uveitis grade at presentation (p = 0.031) were associated with greater IMT ( ≥ 2) requirement. 53% had uveitis recurrence compared to 16% with nephritis recurrence. At the most recent visit, nephritis was controlled in 90%, while uveitis in 74%. Four (4%) patients required glaucoma surgery. Nine (8%) patients had renal complications. CONCLUSIONS: Most patients with TINU require steroid-sparing IMT for control of uveitis, with nearly half requiring addition of biologic agents. Urinalysis, urine ß2M and SCr testing should be considered in children presenting with uveitis, especially when the disease is bilateral and anterior.

Retinal hemorrhage variation in inertial versus contact head injuries.

BACKGROUND: Abusive head trauma (AHT) is frequently accompanied by dense/extensive retinal hemorrhages to the periphery with or without retinoschisis (complex retinal hemorrhages, cRH). cRH are uncommon without AHT or major trauma. OBJECTIVE: The study objectives were to determine whether cRH are associated with inertial vs. contact mechanisms and are primary vs. secondary injuries. PARTICIPANTS AND SETTING: This retrospective study utilized a de-identified PediBIRN database of 701 children <3-years-old presenting to intensive care for head trauma. Children with motor vehicle related trauma and preexisting brain abnormalities were excluded. All had imaging showing head injury and a dedicated ophthalmology examination. METHODS: Contact injuries included craniofacial soft tissue injuries, skull fractures and epidural hematoma. Inertial injuries included acute impairment or loss of consciousness and/or bilateral and/or interhemispheric subdural hemorrhage. Abuse was defined in two ways, by 1) predetermined criteria and 2) caretaking physicians/multidisciplinary team's diagnostic consensus. RESULTS: PediBIRN subjects with cRH frequently experienced inertial injury (99.4 % (308/310, OR = 53.74 (16.91-170.77)) but infrequently isolated contact trauma (0.6 % (2/310), OR = 0.02 (0.0004-0.06)). Inertial injuries predominated over contact trauma among children with cRH sorted AHT by predetermined criteria (99.1 % (237/239), OR = 20.20 (6.09-67.01) vs 0.5 % (2/339), OR = 0.04 (0.01-0.17)). Fifty-nine percent of patients with cRH, <24 h altered consciousness, and inertial injuries lacked imaging evidence of brain hypoxia, ischemia, or swelling. CONCLUSIONS: cRH are significantly associated with inertial angular acceleration forces. They can occur without brain hypoxia, ischemia or swelling suggesting they are not secondary injuries.

THINGS-data, a multimodal collection of large-scale datasets for investigating object representations in human brain and behavior.

Understanding object representations requires a broad, comprehensive sampling of the objects in our visual world with dense measurements of brain activity and behavior. Here, we present THINGS-data, a multimodal collection of large-scale neuroimaging and behavioral datasets in humans, comprising densely sampled functional MRI and magnetoencephalographic recordings, as well as 4.70 million similarity judgments in response to thousands of photographic images for up to 1,854 object concepts. THINGS-data is unique in its breadth of richly annotated objects, allowing for testing countless hypotheses at scale while assessing the reproducibility of previous findings. Beyond the unique insights promised by each individual dataset, the multimodality of THINGS-data allows combining datasets for a much broader view into object processing than previously possible. Our analyses demonstrate the high quality of the datasets and provide five examples of hypothesis-driven and data-driven applications. THINGS-data constitutes the core public release of the THINGS initiative (https://things-initiative.org) for bridging the gap between disciplines and the advancement of cognitive neuroscience.