Purpose: To analyse the prevalence, incidence and aetiology of paediatric uveitis.Methods: A retrospective, population‐based cohort study of Finnish children with uveitis in Northern Ostrobothnia Hospital District in 2008–2017. The data included parameters for age, gender, age at diagnosis, laterality, chronicity, anatomical distribution of the disease, aetiology and systemic association.Results: One hundred fifty patients aged <16 years (mean age 6.9 ± 3.9 years) with uveitis were included, out of whom 53% were girls. The first uveitis episode occurred at 1–6 years in 59%, and 62% of them were girls. In the age group of 7–15 years, boys were diagnosed with uveitis more frequently than girls (61% versus 39%, respectively). Seventy percent of the girls were diagnosed with their first uveitis episode at the age of 1–6 years, whereas only 48% of the boys were diagnosed at that age. The prevalence of uveitis increased from 64/100 000 children <16 years in 2008 (95% CI, 47.7–84.2) to 106/100 000 in 2017 (95% CI, 84.6–130.2). The incidence of childhood uveitis in 2008–2017 was 14/100 000 person‐years in children <16 years (95% CI, 11.3–16.5). Eighty‐seven percent of the cases were non‐infectious, 9% were infectious, and 3% had masquerade syndromes. Sixty‐one percent of patients had juvenile idiopathic arthritis (JIA).Conclusion: The prevalence of paediatric uveitis has increased during the last decade in both genders. Uveitis is more frequent in girls, and they were diagnosed at a younger age than boys. Idiopathic cases and JIA accounted for a majority of aetiological features.

Aims To appraise the ocular manifestations of Puumala hantavirus evoked haemorrhagic fever with renal syndrome nephropathia epidemica (NE) and to clarify the mechanisms of ocular changes in the largest series of patients examined to date.Methods 92 eyes of 46 patients with serologically proven NE were examined during the acute phase and after clinical recovery. Ocular symptoms were recorded, and visual acuity, refraction, intraocular pressure and ocular dimensions were evaluated.Results 88% of the patients experienced decreased intraocular pressure (IOP) (pConclusion Ocular symptoms and disturbances are common in acute NE, and the symmetry of the ocular changes reflects the systemic nature of the disease. A decrease in IOP and myopic shift mainly due to thickening of the lens are evident in acute disease. The myopic shift only partially explains the visual disturbances supporting the possible multifactorial origin of the ocular findings in patients with NE.

Diabetic retinopathy (DR) is a common complication of diabetes and may lead to irreversible visual loss. Efficient screening and improved treatment of both diabetes and DR have amended visual prognosis for DR. The number of patients with diabetes is increasing and telemedicine, mobile handheld devices and automated solutions may alleviate the burden for healthcare. We compared the performance of 21 artificial intelligence (AI) algorithms for referable DR screening in datasets taken by handheld Optomed Aurora fundus camera in a real-world setting. Prospective study of 156 patients (312 eyes) attending DR screening and follow-up. Both papilla- and macula-centred 50° fundus images were taken from each eye. DR was graded by experienced ophthalmologists and 21 AI algorithms. Most eyes, 183 out of 312 (58.7%), had no DR and mild NPDR was noted in 21 (6.7%) of the eyes. Moderate NPDR was detected in 66 (21.2%) of the eyes, severe NPDR in 1 (0.3%), and PDR in 41 (13.1%) composing a group of 34.6% of eyes with referable DR. The AI algorithms achieved a mean agreement of 79.4% for referable DR, but the results varied from 49.4% to 92.3%. The mean sensitivity for referable DR was 77.5% (95% CI 69.1–85.8) and specificity 80.6% (95% CI 72.1–89.2). The rate for images ungradable by AI varied from 0% to 28.2% (mean 1.9%). Nineteen out of 21 (90.5%) AI algorithms resulted in grading for DR at least in 98% of the images. Fundus images captured with Optomed Aurora were suitable for DR screening. The performance of the AI algorithms varied considerably emphasizing the need for external validation of screening algorithms in real-world settings before their clinical application.

Our aim was to characterize clinical properties and laboratory parameters in patients with or without cerebrospinal fluid (CSF) findings suggestive of central nervous system (CNS) involvement, and especially those who developed serious CNS complications during acute nephropathia epidemica (NE) caused by Puumala hantavirus (PUUV) infection. A prospective cohort of 40 patients with acute NE and no signs of major CNS complications was analyzed. In addition, 8 patients with major CNS complications associated with NE were characterized. We collected data of CNS symptoms, CSF analysis, brain magnetic resonance imaging (MRI) results, electroencephalography (EEG) recordings, kidney function, and a number of laboratory parameters. Selected patients were evaluated by an ophthalmologist. Patients with a positive CSF PUUV IgM finding or major CNS complications were more often males (p < 0.05) and they had higher plasma creatinine values (p < 0.001) compared to those with negative CSF PUUV IgM. The degree of tissue edema did not explain the CSF findings. Patients with major CNS complications were younger than those with negative CSF PUUV IgM finding (52.9 vs. 38.5 years, p < 0.05). Some patients developed permanent neurological and ophthalmological impairments. CNS and ocular involvement during and after acute NE can cause permanent damage and these symptoms seem to be attributable to true infection of the CNS rather than increased tissue permeability. The possibility of this condition should be borne in mind especially in young male patients.

Background: Tumor necrosis factor α–induced protein 3 gene (TNFAIP3, also called A20) haploinsufficiency (HA20) leads to autoinflammation and autoimmunity. We have recently shown that a p.(Lys91*) mutation in A20 disrupts nuclear factor κB signaling, impairs protein-protein interactions of A20, and leads to inflammasome activation. Methods: We now describe the clinical presentations and drug responses in a family with HA20 p.(Lys91*) mutation, consistent with our previously reported diverse immunological and functional findings. Results: We report for the first time that inflammasome-mediated autoinflammatory lung reaction caused by HA20 can be treated with interleukin 1 antagonist anakinra. We also describe severe anemia related to HA20 successfully treated with mycophenolate. In addition, HA20 p.(Lys91*) was found to associate with autoimmune thyroid disease, juvenile idiopathic arthritis, psoriasis, liver disease, and immunodeficiency presenting with specific antibody deficiency and genital papillomatosis. Conclusions: We conclude that HA20 may lead to combination of inflammation, immunodeficiency, and autoimmunity. The condition may present with variable and unpredictable symptoms with atypical treatment responses.