The purpose of this study was to investigate the occurrence rate and predictors of photodynamic therapy (PDT) induced acute exudative maculopathy (PAEM). This retrospective study included 39 eyes of 39 patients (32 males and 7 females), who were treated with initial PDT. PAEM was defined as an increase in central retinal thickness (CRT) of 15% or more measured by OCT on day 3 after PDT compared with baseline. Sixteen of 39 eyes (41%) were classified in the PAEM+ group. CRT and central choroidal thickness (CCT) were significantly increased at 3 days in the PAEM+ group and significantly decreased at 1 month after PDT in the PAEM- group. In a multiple comparison, neovascular age-related macular degeneration (nAMD) had a significantly higher incidence of PAEM compared to polypoidal choroidal vasculopathy (PCV) and central serous chorioretinopathy (CSC). The incidence of PAEM was lower in PCV and CSC, and higher in nAMD. BCVA at 1 month was significantly worse in the PAEM group, which may be related to visual prognosis after PDT. Since both CRT and CCT decrease at 1 month, the detection of PAEM needs to be assessed a few days after PDT.

Introduction: To evaluate 1-year outcomes of intravitreal aflibercept (IVA) using a treat-andextend (TAE) regimen for polypoidal choroidal vasculopathy (PCV) and identify the factors for patients whose treatment intervals could be extended. Methods: Fifty-one eyes of treatment-naïve PCV patients treated with IVA using a TAE regimen for at least 1 year were examined retrospectively. All patients received at least three IVA injections every 5 weeks, and the intervals were then extended by 2-week adjustments up to 13 weeks. When retinal exudation recurred, the patient was treated with the same regimen, but with a shortened interval of 5 weeks.The main outcome measures were changes in bestcorrected visual acuity (BCVA) and central retinal thickness (CRT) as well as the treatment interval at 1 year. Results: The mean logarithm of the minimum angle of resolution BCVA improved from 0.24 ± 0.32 at baseline to 0.18 ± 0.31 at 12 months (p = 0.048). The mean CRT decreased from 350.3 ± 147.7 lm at baseline to 215.3 ± 75.0 lm at 4 months (p \ 0.001), after which it was maintained at this level. At 12 months, the administration interval was 5 weeks in eight eyes (15.7%), 7 weeks in six eyes (11.8%), 9 weeks in two eyes (3.9%), 11 weeks in four eyes (7.8%), and 13 weeks in 31 eyes (60.8%). Female sex, a thinner CRT at 6 months, and absence of polypoidal lesions at 12 months were significant factors related to patients whose treatment intervals could be extended without recurrence to 13 weeks. Conclusion: IVA using a TAE regimen improved visual and anatomical outcomes in eyes with PCV at 1 year using a protocol to adjust the injection intervals specifically for each patient so as to obtain no retinal exudation.

A patient with a new variant of multiple sulphatase deficiency (MSDv) is reported. Unlike the usual type, onset was late and progress was slow. The phenotypic changes were those usually seen in multiple sulphatase deficiency but much milder. Cytoplasmic accumulations were found in skin fibroblasts, and urinary mucopolysaccharides and sulphatides were high. Arylsulphatases A, B and C (ASA, B and C), heparan N-sulphatase sulphoiduronate sulphatase, and N-acetylgalactosamine 6-sulphatase all had low activity in lymphocytes and cultured skin fibroblasts. Complementation for ASA activity was found in hybrids between MSDv and metachromatic leukodystrophy (MLD) as well as between multiple sulphatase deficiency (MSD) and MLD. Complementation for ASC activity was also seen in hybrids between MSDv and X-linked ichthyosis (XLI), and between MSD and XLI. However, neither ASA nor ASC activity increased in hybrid cells of MSDv and MSD. These results suggested that the mutations of MSDv and of MSD were allelic, although of different phenotypes.

Objective Although malignant lymphoma (ML) can occur in every organ, diagnosing cardiac involvement without cardiac manifestations is difficult. We therefore investigated the incidence of cardiac involvement in ML in our hospital and clarified the transthoracic echocardiography (TTE) findings of cardiac involvement. Methods Patients with ML referred to our hospital between January 2013 and December 2019 were retrospectively reviewed. Patients During the study period, 453 patients were identified. The mean age was 64.9 years old, and 54% of the patients were men. Results Diffuse large B-cell lymphoma (DLBCL) was the most common lymphoma, followed by follicular lymphoma. Of the 453 patients, 394 (87.0%) underwent TTE at the initial diagnosis or during the clinical course. The performance rates of TTE in DLBCL, Hodgkin lymphoma, and mantle cell lymphoma were above 90%. Cardiac involvement was detected in 6 (five with DLBCL and one with B-cell lymphoma) (1.5%) of the 394 patients who underwent TTE. The involved lesions of the heart varied, and five patients had pericardial effusion. Five patients had a preserved left ventricular ejection fraction. All patients were treated with chemotherapy, and some were treated with radiation and surgery. Conclusion Cardiac involvement was observed in six (1.5%) of the patients with ML who underwent TTE. B-cell lymphoma, especially DLBCL, is a common ML with cardiac involvement. Although five patients had pericardial effusion, the involved lesions of the heart were not uniform. TTE is a useful imaging modality to noninvasively and repeatedly evaluate the tumor characteristics, response to ML treatment, and cardiac function.

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