The risk of developing lymphoma in patients with Sjögren’s syndrome (SS) is 44 times higher than in the normal population with the most common lymphomas derived from marginal zone B (MZB) cells. Current understanding of the role of MZB cells in SS is primarily based on salivary gland pathology, while their contextual association with lacrimal glands and ocular manifestations largely remains unknown. We examined this possibility using a SS mouse model (thrombospondin-1 deficient (TSP1^−/−)) with well-characterized ocular disease. We determined the frequency, localization, and cytokine profiles of MZB cells and their association with an antibody response in TSP1^−/− mice treated with a TSP-derived peptide. A significantly increased frequency of MZB cells was detected in the spleens and lacrimal glands of TSP1^−/− mice in comparison to wild-type tissues as detected by immunostaining. An altered cytokine profile of TSP1^−/− MZB cells was supportive of T helper 17 (Th17)-related pathogenesis. A significantly reduced antibody response and the splenic MZB compartment against an eye-derived antigen were noted in TSP-derived peptide-treated mice. These changes correspond with the previously reported ability of the peptide to ameliorate SS-related ocular manifestations. Collectively, our results demonstrate dysregulation of MZB cells in TSP1^−/− mice and highlight their role in the context of SS-related chronic ocular surface disease.

To address the conflicting role of thrombospondin (TSP)-1 reported in acute and chronic pathologies, this study investigated the role of TSP-1 in regulating leukocyte recruitment and regulation of VCAM-1 expression using mouse models of uveitis. The spontaneously increased VCAM-1 expression and leukocyte adhesion in retinas of TSP-1-deficient mice suggested a TSP-1-mediated regulation of VCAM-1 expression. In a chronic uveitis model, induced by immunizing wild-type mice with specific interphotoreceptor retinoid-binding protein (IRBP) peptide, topically applied TSP-1-derived CD47-binding peptide significantly reduced the clinical disease course and retinal leukocyte adhesion as compared to the control peptide-treated group. In contrast, in LPS-mediated acute uveitis, TSP-1 deficiency significantly reduced the retinal leukocyte adhesion. The results of our in vitro study, using vascular endothelial cell (EC) cultures, demonstrate that unlike TNF-α, VCAM-1 expression induced by IL-17 is associated with a reduced expression of endogenous TSP-1. Such reduced endogenous TSP-1 expression in IL-17-stimulated ECs helps limit the CD36-mediated increased VCAM-1 expression, while favoring CD47-mediated inhibition of VCAM-1 expression and leukocyte adhesion. Thus, our study identifies TSP-1:CD47 interaction as a molecular pathway that modulates IL-17-mediated VCAM-1 expression, contributing to its anti-inflammatory effect in chronic inflammatory conditions.

Compared to the adult literature, there are few enhanced recovery after surgery (ERAS) protocols standardized in the pediatric population. The objective of the current study is to determine if the implementation of an ERAS protocol would improve patient outcomes in the ambulatory pediatric urologic population. A retrospective analysis was performed on pediatric patients who underwent urologic procedures (circumcision, orchiopexy, hypospadias correction, and urethroplasty) in the ambulatory surgical setting affiliated with a tertiary pediatric hospital. Outcomes measured include opioid use, home pain control, time in recovery, need for rescue pain medications, and adverse events between pediatric patients receiving standard of care (n = 30) and pediatric patients receiving the ERAS protocol (n = 29). The application of the ERAS pathway led to significantly increased opioid‐free care (7% vs. 43%, p < 0.01). There was a reduction in the cost of care, a trend toward reduced opioid use, a trend toward reduced PACU stays for ERAS patients, and families of ERAS patients reported a 100% rate of well‐controlled pain at home. These changes occurred without any increased need for rescue pain medications (16% vs. 13%, p = 1) or any change in adverse events (0% vs. 0%, p = 1.0). Postoperative pain measures are improved in pediatric patients receiving the ERAS protocol in an ambulatory surgery setting when compared to patients receiving the standard of care, without an increased risk of adverse events or the need for rescue analgesia. Therefore, this work serves as a proof of concept that ERAS protocols can improve postoperative outcomes in the pediatric ambulatory surgical population.

Background: Idiopathic granulomatous mastitis (IGM) is a rare inflammatory breast disease with variable clinical manifestations. The purpose of this study is to compare racial patterns and management of IGM at a tertiary academic center in New York. Methods: A cross-sectional study was performed on all patients with a diagnosis of IGM from 2018 to 2024 at Stony Brook University Hospital. Results: Twenty cases of IGM were identified by chart review. Hispanic women were significantly overrepresented in women with IGM (p<0.0001). Hispanic women with IGM were significantly younger (35.1 ± 6.9 years) than white women (53.4 ± 12.2 years) at diagnosis (p=0.007) and more likely to present with clinical findings rather than radiologic findings (p=0.045). Most (71.4%) patients with no recurrence had been treated with prednisone (n=5). Conclusion: This study suggests that a racial predilection to IGM exists. Further studies are needed to develop the most effective treatment strategy for IGM.

Background and Clinical Significance: Acquired myenteric hypoganglionosis is a rare dysmotility disorder that can present in childhood and adulthood, characterized by a reduced number of ganglion cells within Auerbach’s plexus. Due to the rarity of the pathology, few case reports of acquired myenteric hypoganglionosis in adolescents have been described. This case report explores the presentation, risk factors, and surgical complications associated with the ultimate diagnosis of myenteric hypoganglionosis. Case Presentation: We present a case of a 12-year-old male with a history of constipation and achalasia, presenting with constipation and abdominal distention, who underwent a colonoscopy, which was converted to an exploratory laparotomy with loop ileostomy creation due to persistent significant abdominal distention. This was complicated by colonic perforation, most likely secondary to stercoral colitis, requiring takeback to the operating room on postoperative day 11 for an exploratory laparotomy with bowel resection and mucous fistula creation. The patient was then referred to Boston Children’s Hospital for motility studies, which revealed poor colonic motility and plans to reassess motility in 1 year. Conclusions: Although rare, it is important to have high clinical suspicion for acquired myenteric hypoganglionosis in children, especially males, with severe constipation.