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Photosensitivity is the clinical hallmark of both erythropoietic protoporphyria (EPP) and X-linked dominant protoporphyria (XLDPP). Both disorders result from a hereditary dysfunction in heme biosynthesis. Disease onset is usually in early childhood. However, rare patients with late-onset EPP in association with a myeloproliferative disorder or myelodysplastic syndrome have been reported. In this issue, Livideanu et al. describe the first patient with late-onset XLDPP.

In patients with erythropoietic protoporphyria, sensitivity to the sun leads to pain and compromised quality of life. In two clinical trials, one in Europe and one in the United States, a peptide analogue of an α-melanocyte–stimulating hormone alleviated symptoms. Erythropoietic protoporphyria is a rare, autosomal recessive inborn error of metabolism that typically manifests in early childhood as severe painful photosensitivity. The photosensitivity results from accumulated protoporphyrin in erythroid cells and tissues because of the decreased activity of ferrochelatase, the heme biosynthetic enzyme that inserts iron into protoporphyrin to form heme. 1 – 4 An X-linked form of erythropoietic protoporphyria 5 , 6 that accounts for 2 to 10% of cases results from a gain of function of erythroid-specific aminolevulinic acid synthase 2. Pathophysiologically, protoporphyrin is released from erythroid cells into the circulation, gains access to the vascular endothelium and liver, and is excreted . . .

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Although multiple interactions of seminiferous tubules and the interstitial testicular tissue are known, correlation of cytokeratin 18 expressing Sertoli cells with interstitial changes has still not yet been reported. Considering this fact, we focused our investigation on changes of the adjacent interstitial tissue. A total sample of 51 testicular biopsies (from infertile patients) showing mixed atrophy was examined immunohistochemically with antibodies against cytokeratin 18, vimentin, L26/CD20, CD4 and CD8. Twenty-one of the 51 cases showed single seminiferous tubules with Sertoli cells expressing cytokeratin 18. These 21 tubules consistently exhibit either spermatogenic arrest at the level of spermatogonia or only immature Sertoli cells. In the adjacent interstitial tissue of 8 of the 21 cytokeratin 18 positive tubules (39%) striking inflammatory infiltrates--predominantly expressing L26/CD20 typical for B lymphocytes and CD8 typical for T suppressor lymphocytes--were detected. These findings underline that tubules with cytokeratin 18 expressing Sertoli cells exhibit early spermatogenic arrest or only few remaining Sertoli cells. Additionally, we observed a remarkable co-localization of these tubules with lymphocytic infiltrates of the adjacent interstitial tissue.

Administration of 8-methoxypsoralen (8-MOP) in a dilute bath water solution is an effective therapeutic alternative for systemic application of 8-MOP, avoiding systemic side effects such as nausea and cataractogenesis. The aim of our study was to determine the epicutaneous penetration of 8-MOP in a dilute bath water solution with and without additional UVA irradiation in human skin under in vitro conditions. To simulate the PUVA bath procedure, 8 skin samples were exposed to radioactively labeled 8-MOP in a water solution. After 20 min, the test solution was removed and the skin surface was dried. Immediately after the bath procedure, 4 of the skin samples were irradiated with 0.5 J/cm2 UVA. During a test period of 15 h, the 8-MOP penetration was observed. In both test groups (with and without UVA irradiation) 8-MOP permeated through all skin layers between 30 min and 1 h after application. Compared to the unirradiated skin samples, the UVA-irradiated skin samples showed a significantly slower increase and a lower maximum of 8-MOP permeation. Following our results, UVA irradiation of 8-MOP-exposed skin samples led to a significantly decreased permeation rate. This might be due to UVA-induced links between 8-MOP molecules and human DNA. In addition, we investigated the levels of radioactivity emitted by tritium-labeled 8-MOP in stratum corneum, epidermis and dermis up to 30 min after 8-MOP bath in two further test groups with and without additional UVA irradiation. The statistical analysis revealed no significant differences between these two test groups. Thus, the levels of radioactivity remained constant in the epidermis and dermis during the test period of 30 min. Since the levels of radioactivity were constant up to 30 min after UVA irradiation, a previously supposed marked loss of 8-MOP concentration might not be responsible for the rapid extinction of observed in vivo photosensitivity within 1 h after PUVA bath observed in vivo in human skin.

Although multiple interactions of seminiferous tubules and the interstitial testicular tissue are known, correlation of cytokeratin 18 expressing Sertoli cells with interstitial changes has still not yet been reported. Considering this fact, we focused our investigation on changes of the adjacent interstitial tissue. A total sample of 51 testicular biopsies (from infertile patients) showing mixed atrophy was examined immunohistochemically with antibodies against cytokeratin 18, vimentin, L26/CD20, CD4 and CD8. Twenty-one of the 51 cases showed single seminiferous tubules with Sertoli cells expressing cytokeratin 18. These 21 tubules consistently exhibit either spermatogenic arrest at the level of spermatogonia or only immature Sertoli cells. In the adjacent interstitial tissue of 8 of the 21 cytokeratin 18 positive tubules (39%) striking inflammatory infiltrates–predominantly expressing L26/CD20 typical for B lymphocytes and CD8 typical for T suppressor lymphocytes–were detected. These findings underline that tubules with cytokeratin 18 expressing Sertoli cells exhibit early spermatogenic arrest or only few remaining Sertoli cells. Additionally, we observed a remarkable co-localization of these tubules with lymphocytic infiltrates of the adjacent interstitial tissue.

The Deep Brain Stimulation (DBS) Think Tank XIII was held September 2-4th, 2025, in Gainesville, Florida, at the Norman Fixel Institute for Neurological Diseases at the University of Florida. The theme was “The Evolving Landscape of DBS: New Indications, New Goals.” This theme was a continuation of the DBS Think Tank XI and XII, which were focused on emerging technology and pushing the horizon of indications. Since its founding in 2012, the DBS Think Tank has provided a global forum for leading clinicians, engineers, and researchers in both in industry and academia to present, discuss, and debate the current state of DBS technologies as well as to consider important logistics and ethical challenges. Over the course of three days, members of each panel presented and facilitated discussions on the cutting edge of DBS research. The keynote speaker was Dr. Kamil Uğurbil of the University of Minnesota, who led the first group of researchers to demonstrate the feasibility of imaging the human brain using fMRI technology and who was a pioneer in the development of high-field human MRI scanning. Nobel laureate Dr. Stanley Prusiner, from the University of California, San Francisco, used the story of the discovery of prions to demonstrate the power of pursuing a finding even when the idea conflicted with the prevailing state of the field. The think tank was divided into sections, including: Next Generation Neuromodulation for Gait, Brain Networks and Neuromodulation, Neuroscience & Society, Interventional Psychiatry & Behavior, Devices for Closing the Loop, Physiology & Closing the Loop, and A Roadmap for Genetics & Neuromodulation.