Elevated kynurenine levels in diffuse cutaneous and anti-RNA polymerase III positive systemic sclerosis

Publication date: Available online 14 February 2019

Source: Clinical Immunology

Author(s): Corrado Campochiaro, Simon Lytton, Svetlana Nihtyanova, Dietmar Fuchs, Voon H. Ong, Christopher P. Denton

Abstract

Systemic sclerosis (SSc) is a systemic disease characterized by vasculopathy, progressive fibrosis and autoimmune activation. Tryptophan (Trp) metabolism has been linked to altered immune cell function and to malignancy. We have investigated the role of Trp metabolic pathway in SSc measuring serum Trp, Kynurenine (Kyn) and Trp/Kyn ratio in a cohort of 97 SSc patients and 10 healthy controls. Association with disease characteristics was evaluated. We found that Trp levels in SSc patients were significantly lower compared to HCs. We also found that patients with diffuse cutaneous (dcSSc) had lower levels of Trp compared to limited cutaneous (lcSSc). These results were paralleled by higher levels of Kyn found in SSc patients compared to HCs and significantly lower levels in dcSSc compared to lcSSc. The autoantibody profile was also found to be significantly associated with Kyn and Trp levels as anti-RNA-polymerase III (ARA) positive patients were shown to have lower Trp levels and higher Kyn levels compared with anti-centromere and anti-topoisomerase I positive patients. Moreover, the highest Trp/Kyn was found in ARA+ patients with dcSSc, suggesting that an activation of the Kyn pathway, is more specifically associated with this subset of SSc patients. Stability over time makes these markers of Trp metabolism feasible for SSc stratification.

http://bit.ly/2E94yzw

Multiple cutaneous metastasis of synchronous urothelial carcinoma of the bladder and the renal pelvis: a case report

Cutaneous metastatic disease arising from urinary tract carcinoma is rare and associated with a poor prognosis. We report a case of metastatic disease occurring in a patient treated for synchronous urothelial ...

http://bit.ly/2BCOh4e

Pharyngeal flap using carotid artery mobilization in 22q11.2 deletion syndrome with velopharyngeal insufficiency

Publication date: Available online 13 February 2019

Source: International Journal of Pediatric Otorhinolaryngology

Author(s): Max Greenberg, Christen Caloway, Cheryl Hersh, Daniel Ryan, Paula Goldenberg, Christopher Hartnick

Abstract

Introduction

22q11.2 deletion syndrome is the most common microdeletion syndrome in children. Many patients with this disease develop craniofacial defects including cleft palate, bifid uvula, and velopharyngeal insufficiency. Our study adds to the current body of literature by describing a novel technique of carotid mobilization performed in conjunction with pharyngeal flap surgery in patients with extensive medialization of the carotid artery.

Methods

Carotid artery mobilization followed by insertion of a superiorly based pharyngeal flap was performed on two patients, a 10-year-old girl and a 5-year-old boy, with 22q11.2 deletion syndrome concurrent with velopharyngeal insufficiency.

Results

Neither patient experienced significant post-operative issues. Following the procedure, parents of both patients noted significant speech and voice improvement. Both patients had improvements in VPI Effects On Life Outcome (VELO) scores, nasometry, and production of paragraph passages following surgery.

Conclusions

Our study describes a novel surgical treatment for children with 22q11.2 deletion syndrome with significant velopharyngeal insufficiency (VPI). The procedure wherein is characterized by an extensive mobilization of the carotid artery followed by implantation of a pharyngeal flap. This technique resulted in no significant intra-operative bleeding, and was measured to be successful as noted by nasometry scores and changes in pre- and post-op VELO scores.

http://bit.ly/2Sz9kzb

Developing and testing intervention theory by incorporating a views synthesis into a qualitative comparative analysis of intervention effectiveness

Melendez-Torres, GJ; Sutcliffe, K; Burchett, HED; Rees, R; Thomas, J; (2019) Developing and testing intervention theory by incorporating a views synthesis into a qualitative comparative analysis of intervention effectiveness. Research Synthesis Methods 10.1002/jrsm.1341 . (In press).

http://bit.ly/2X59Aof

Current insights into LMNA cardiomyopathies: Existing models and missing LINCs

Brayson, D; Shanahan, CM; (2017) Current insights into LMNA cardiomyopathies: Existing models and missing LINCs. Nucleus , 8 (1) pp. 17-33. 10.1080/19491034.2016.1260798 . Green open access

http://bit.ly/2RZcF5f

Stylistics goes to school

Cushing, IR; (2018) Stylistics goes to school. Language and Literature , 27 (4) pp. 271-285. 10.1177/0963947018794093 . Green open access

http://bit.ly/2XbuYbo

Surface and Interfacial Nanoscience of Titanium Dioxide

Nadeem, Immad Muhammed; (2018) Surface and Interfacial Nanoscience of Titanium Dioxide. Doctoral thesis (Eng.D), UCL (University College London). Green open access

http://bit.ly/2S277H9