المؤلفون: Ebens, Christen L.

المصدر: EMBO Molecular Medicine; 10/7/2021, Vol. 13 Issue 10, p1-3, 3p

مستخلص: Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited blistering skin disease, resulting from biallelic mutations in COL7A1, the gene encoding type VII collagen (C7). At mucocutaneous barriers, tissue integrity relies upon linked extracellular matrix (ECM) proteins forming a physiologic suture, connecting basal epidermal keratinocytes to the underlying dermis. C7 secreted from epidermal keratinocytes and dermal fibroblasts homotrimerizes in the upper dermis to form anchoring fibrils, a critical component of this suture. Clinical manifestations of RDEB are apparent at birth and include exquisite skin fragility, pain and itch, high metabolic demand, and complications downstream of systemic inflammation. Dermal fibrosis is a critical complication of RDEB. Repeated cycles of mechanical injury and healing trigger characteristic fibrotic changes. In addition to functional limitations from joint strictures and pseudosyndactyly formation, dermal fibrosis in RDEB is a nidus for and potential driver of aggressive squamous cell carcinoma (SCC), the leading cause of death in RDEB. A greater understanding of fibrosis in RDEB promises to inform impactful, lifeprolonging clinical trials in this patient population with no proven systemic therapy or cure. [ABSTRACT FROM AUTHOR]

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المؤلفون: Ferdjallah, Asmaa, Boull, Christina L., Stefanski, Heather, Ebens, Christen L.

المصدر: Clinical Case Reports; Dec2020, Vol. 8 Issue 12, p3625-3627, 3p

مصطلحات موضوعية: HEMATOPOIETIC stem cells, STEM cell transplantation, ACUTE myeloid leukemia, SWEET'S syndrome, LEUKEMIA

مستخلص: Sweet's syndrome associated with relapse of leukemia suggests abnormal neutrophil response to transformation of dysfunctional leukemia blast cells, and hence, relapse should be excluded in similar clinical situation. [ABSTRACT FROM AUTHOR]

: Copyright of Clinical Case Reports is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

المؤلفون: Ebens, Christen L.¹ ebens012@umn.edu, Smith, Angela R.¹, Verghese, Priya S.²

المصدر: Pediatric Transplantation. Aug2017, Vol. 21 Issue 5, pn/a-N.PAG. 10p.

مصطلحات موضوعية: *KIDNEY transplantation, *HEMATOPOIETIC stem cell transplantation, *JUVENILE diseases, *IMMUNOSUPPRESSIVE agents, *PEDIATRIC therapy, *DISEASE risk factors

مستخلص: Pediatric patients requiring kidney transplant after hematopoietic cell transplant receive multiple courses of immunosuppression placing them at risk for infection. To elucidate potential risk factors for infection, we compared the immunosuppressive regimens and infectious complications of pediatric kidney transplant recipients at a single institution who had previously undergone hematopoietic cell transplant from different donors to similar patients reported in the literature. Among the initial four post-hematopoietic cell transplant kidney transplant patients reviewed, viremia episodes were universal, including BK virus, Epstein-Barr virus, and human herpesvirus-6, with one death from presumed BK virus encephalitis. No viremia was reported in five similar cases in the literature. Risk factors for increased infection include use of lymphodepleting serotherapy in HCT conditioning, multiple HCTs, limited immune reconstitution time between transplants, increased pre- KTx viral burden, and use of T-cell-depleting versus -suppressive induction immunosuppression for KTx. These findings suggest that pediatric post- HCT KTx recipients are at increased risk for viral infections, likely benefitting from thorough pre- KTx evaluation of immune reconstitution and preferential use of non-T-cell-depleting induction therapy for KTx. We applied these recommendations to one subsequent post- HCT patient requiring KTx at our institution with excellent outcomes one year post- KTx. [ABSTRACT FROM AUTHOR]