Showing papers by "British Hospital published in 2018"

How immunological profile drives clinical phenotype of primary Sjögren's syndrome at diagnosis: analysis of 10,500 patients (Sjögren Big Data Project).

Abstract: OBJECTIVES: To evaluate the influence of the main immunological markers on the disease phenotype at diagnosis in a large international cohort of patients with primary Sjogren's syndrome (SjS).METHODS: The Big Data Sjogren Project Consortium is an international, multicentre registry created in 2014. As a first step, baseline clinical information from leading centres on clinical research in SjS of the 5 continents was collected. The centres shared a harmonised data architecture and conducted cooperative online efforts in order to refine collected data under the coordination of a big data statistical team. Inclusion criteria were the fulfillment of the 2002 classification criteria. Immunological tests were carried out using standard commercial assays.RESULTS: By January 2018, the participant centres had included 10,500 valid patients from 22 countries. The cohort included 9,806 (93%) women and 694 (7%) men, with a mean age at diagnosis of primary SjS of 53 years, mainly White (78%) and included from European countries (71%). The frequency of positive immunological markers at diagnosis was 79.3% for ANA, 73.2% for anti-Ro, 48.6% for RF, 45.1% for anti- La, 13.4% for low C3 levels, 14.5% for low C4 levels and 7.3% for cryoglobulins. Positive autoantibodies (ANA, Ro, La) correlated with a positive result in salivary gland biopsy, while hypocomplementaemia and especially cryoglo-bulinaemia correlated with systemic activity (mean ESSDAI score of 17.7 for cryoglobulins, 11.3 for low C3 and 9.2 for low C4, in comparison with 3.8 for negative markers). The immunological markers with a great number of statistically-significant associations (p<0.001) in the organ-by-organ ESS- DAI evaluation were cryoglobulins (9 domains), low C3 (8 domains), anti-La (7 domains) and low C4 (6 domains).CONCLUSIONS: We confirm the strong influence of immunological markers on the phenotype of primary SjS at diagnosis in the largest multi-ethnic international cohort ever analysed, with a greater influence for cryoglobulinaemic-related markers in comparison with Ro/La autoantibodies and ANA. Immunological patterns play a central role in the phenotypic expression of the disease already at the time of diagnosis, and may guide physicians to design a specific personalised management during the follow-up of patients with primary SjS. (Less)

The Role of Extracorporeal Shockwave Treatment in Musculoskeletal Disorders.

Abstract: Increasing evidence suggests that extracorporeal shockwave treatment (ESWT) is safe and effective for treating several musculoskeletal disorders.Two types of technical principles are usually included in ESWT: focused ESWT (F-ESWT) and radial pressure waves (RPW). These 2 technologies differ with res

Natural History and Predictors of Progression to Sjögren's Syndrome Among Participants of the Sjögren's International Collaborative Clinical Alliance Registry.

Abstract: Background/Purpose: To explore changes in the phenotypic features of Sjoogren's syndrome (SS), and in SS status among participants in the Sjoogren's International Collaborative Clinical Alliance (SICCA) registry over a 2 to 3-year interval. Methods: All participants in the SICCA registry who were found to have any objective measures of salivary hypofunction, dry eye, focal lymphocytic sialadenitis in minor salivary gland biopsy, or anti-SSA/B antibodies, were recalled over a window of 2 to 3 years after their baseline examinations to repeat all clinical examinations and specimen collections to determine whether there was any change in phenotypic features and in SS status. Results: As of September 15, 2013, 3,514 participants had enrolled in SICCA, and among 3,310 eligible, 771 presented for a follow-up visit. Among participants found to have SS using the 2012 ACR classification criteria, 93% again met the criteria after 2 to 3 years, and this proportion was 89% when using the 2016 ACR-EULAR criteria. Among those who did not meet ACR or ACR-EULAR criteria at baseline, 9% and 8%, respectively, had progressed and met them at follow-up. Those with hypergammaglobulinemia and hypocomplementemia at study entry were respectively 4 and 6 times more likely to progress to SS by ACR criteria than those without these characteristics (95%Confidence Interval: 1.5 – 10.1 and 1.8 – 20.4, respectively). Conclusion: While there was stability over a 2-3-year period of both individual phenotypic features of SS and of SS status, hypergammaglobulinemia and hypocomplementemia at study entry were predictive of progression to SS. This article is protected by copyright. All rights reserved.

Management of bleeding and emergency invasive procedures in patients on dabigatran: Updated guidelines from the French Working Group on Perioperative Haemostasis (GIHP) - September 2016.

Abstract: In 2013, the GIHP published guidelines for the management of severe haemorrhages and emergency surgery. This update applies to patients treated with dabigatran, with a bleeding complication or undergoing an urgent invasive procedure. It includes how to handle the available specific antidote (idarucizumab), when to measure dabigatran plasmatic concentration and when to use non-specific measures in these situations. It also includes guidelines on how to perform regional anaesthesia and analgesia procedures.

Clinical parameters, LysoGb3, podocyturia, and kidney biopsy in children with Fabry disease: is a correlation possible?

Abstract: Fabry disease is an X-linked lysosomal storage disorder caused by α-galactosidase enzyme deficiency. We present clinical, biochemical, and histologic findings in children with classical phenotypic presentation of Fabry disease. A retrospective analysis was performed using charts from 14 children with confirmed diagnosis. Clinical parameters were evaluated. Globotriaosylsphingosine -lysoGb3- detection in plasma, podocyturia, and kidney biopsy were carried out in all cases. All patients except one demonstrated at least one symptom of Fabry disease. LysoGb3 levels were above the normal range in all patients. Podocyturia was documented in all patients. Kidney biopsy revealed glomerular, interstitial, vascular, and tubular changes on light microscopy in nearly all patients. Electron microscopy showed podocyte inclusions in all patients. No difference in symptomatology was discernible between boys and girls. Podocyturia was detectable in children serving as a possible early marker of kidney injury. LysoGb3 was elevated in all cases, emphasizing the importance for diagnosis especially in female patients with normal αGal A activity. A possible association between lysoGb3 and symptom severity and histological involvement in kidney biopsy should be assessed in prospective studies with enough statistical power to determine if lysoGb3 can be used to predict nephropathy in children with Fabry disease.

Incidence and Functional Outcomes of Scapholunate Diastases Associated Distal Radius Fractures: A 2-year Follow-Up Scapholunate Dissociation.

Abstract: Background The Scapholunate Interosseous Ligament (SLIL) is the first intrinsic carpal ligament to be injured in wrist trauma, present in up to 64% of the distal radius fractures. However, it remains unclear what patients develop symptoms, making primary treatment of these injuries accompanying distal radius fractures remains questionable. Objective The aim of this study was to evaluate the functional outcomes of patients with scapholunate diastasis associated with distal radius fractures. Methods We evaluated 391 patients with a distal radius fracture. Using Computer Tomography (CT) scans the scapholunate interval was measured. We identified 14 patients with an SLD (>3mm) of the injured wrist, which underwent a CT-scan of the contralateral wrist. To evaluate the functional outcomes at a mean follow up of 136±90 weeks, we used the Quick Disabilities of the Arm, Shoulder and Hand (qDASH) Score. Results There were 8 patients with bilateral SLD and 6 patients with unilateral SLD. Five patients had a qDASH score of 0 and one patient showed a qDASH score of 18.2. The patient with a poor score had bilateral preexisting osteoarthritis of the wrist. No patient had additional surgery of the SLIL. Conclusion In patients with distal radius fractures, more than half of the 14 patients with an SL gap on CT had widening on the contralateral side. It is therefore worthwhile to image the contralateral wrist before diagnosing a SLD. The patients with unilateral SLD should not be surgically treated at initial presentation because they may have good functional outcomes after a follow up of 2 years.

FOLFIRI3-aflibercept in previously treated patients with metastatic colorectal cancer.

Abstract: Aim To evaluate the efficacy and safety of the modified FOLFIRI3-aflibercept as second-line therapy in patients with metastatic colorectal cancer. Methods This is a retrospective multicenter cohort, evaluating the efficacy and safety of the association of aflibercept with FOLFIRI3 (day 1: aflibercept 4 mg/kg, folinic acid 400 mg/m2, irinotecan 90 mg/m2, 5-fluorouracil infusion 2400 mg/m2 per 46 h; day 3: irinotecan 90 mg/m2) in patients with previously treated metastatic colorectal cancer. The primary endpoint was overall response rate (ORR). Secondary endpoints were disease control rate (DCR), progression-free survival (PFS), overall survival (OS), and safety. Results Among 74 patients treated in four French centers, nine were excluded due to prior use of aflibercept (n = 3), more than one prior treatment line in irinotecan-naive patients (n = 3), and inadequate liver function (n = 3). In the "irinotecan-naive" patients (n = 30), ORR was 43.3% and DCR was 76.7%. Median PFS and OS were 11.3 mo (95%CI: 6.1-29.0) and 17.0 mo (95%CI: 13.0-17.3), respectively. The most common (> 5%) grade 3-4 adverse events were diarrhea (37.9%), neutropenia (14.3%), stomatitis and anemia (10.4%), and hypertension (6.7%). In the "pre-exposed irinotecan" patients (n = 35), 20 (57.1%) received ≥ 2 prior lines of treatment. ORR was 34.3% and DCR was 60.0%. Median PFS and OS were 5.7 mo (95%CI: 3.9-10.4) and 14.3 mo (95%CI: 12.8-19.5), respectively. Conclusion Minimally modified FOLFIRI has improvement dramatically the FOLFIRI3-aflibercept efficacy, whatever prior use of irinotecan. A prospective randomized trial is warranted to compare FOLFIRI-aflibercept to FOLFIRI3-aflibercept.

A global perspective on acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF): results from an international survey

Abstract: Background: AE-IPF is a deadly complication of IPF, for which no international guidelines exist, resulting in global variability in prevention, diagnosis and treatment strategies. Methods: Pulmonologists with ILD expertise were invited to participate in a survey designed by an international expert panel. Results: 469 pulmonologists responded (66 countries, 64% experts). Significant geographical variability in approach to managing AE-IPF was found (figure). Common preventive measures include antifibrotics and antacids. Diagnostic differences are most pronounced regarding use of KL-6 and viral testing, while HRCT, BNP and d-dimer are broadly used. High dose steroids are widely administered (92%), but use of immunosuppressant and other strategies is highly variable (fig). Very few (4%) responders never use immunosuppression. Antifibrotics are initiated during AE-IPF by 66%. Invasive ventilation or ECMO are mainly used as a bridge to transplantation. Most physicians educate patients comprehensively on the severity of AE-IPF (82%) and consider palliative care (63%). Conclusion: International approaches to the prevention, diagnosis and treatment of AE-IPF are diverse. Global international guidelines and trials to evaluate these approaches are needed.

Colorectal Adenoma Risk Is Increased among Recently Diagnosed Adult Celiac Disease Patients.

Abstract: Background. The association between celiac disease and colorectal neoplasia has been previously studied, but the question whether recently diagnosed celiac patients show an increased colorectal adenoma prevalence remains unanswered. Aims. To compare the prevalence of colorectal adenomas between adult patients with a recent diagnosis of celiac disease versus healthy controls. Materials and Methods. A retrospective case-control study was undertaken. Patients with a diagnosis of celiac disease at an age of 45 years or more who undertook colonoscopy six months before or six months after the initiation of a gluten-free diet were enrolled as cases. Asymptomatic subjects undertaking screening colonoscopy were recruited as controls in a 2 : 1 fashion. The prevalence of colorectal adenomas and the prevalence of advanced adenomas were compared between groups. Results. 57 celiac disease patients and 118 controls were enrolled. There was a greater prevalence of female patients among the celiac group, with no significant differences in terms of age. There were more obese patients among controls and a higher proportion of tabaquism among celiac patients. Adenoma prevalence was significantly higher among celiac patients (47.37% versus 27.97%, ). Advanced adenoma detection was not different between groups. Conclusion. Adult patients with a recent diagnosis of celiac disease have an increased prevalence of colorectal adenomas.

Genital mycoplasmas and ureaplasmas in cervicovaginal self-collected samples of reproductive-age women: prevalence and risk factors.

Abstract: The purpose of this study was to characterise the prevalence and risk factors associated with genital mycoplasmas (Mycoplasma hominis [MH], M. genitalium [MG]) and ureaplasmas (Ureaplasma urealytic...

Association Between Body Weight Variation and Survival and Other Adverse Events in Critically Ill Patients With Shock: A Multicenter Cohort Study of the OUTCOMEREA Network.

Abstract: Objectives:This study in critically ill patients with shock assessed the prognostic value of body weight variations occurring each day from day 3 to day 7 on the 30-day outcome in terms of mortality, occurrence of ventilator-associated pneumonia and of bedsore, and occurrence of length of stay.Desig

Rib desmoplastic fibroblastoma: Video-assisted resection.

Abstract: A 46-year-old man was referred to our department due to chronic chest pain. A computed tomography showed an exophytic image arising from 5th rib that was projected on the middle lobule. The patient underwent an exploratory videothoracoscopic because we couldn't discard lung compromise. A corneal-like lesion emerging from the inner side of the 5th rib was revealed. Complete video-assited resection was done. Histopathology examination revealed a desmoplastic fibroblastoma. To our knowledge this is the first case of desmoplastic fibroblastoma arising from a rib and the second case published in all literature of chest wall involvement.

Entirely thoracoscopic resection of a giant emphysematous bulla.

Abstract: A 38-year-old man with longilinear shape, smoker (38 packs/year) and no other relevant medical history was referred to our department due to the finding of left pulmonary hyperlucency on a chest x-ray. A computed tomography (CT) was performed and a giant emphysematous bulla with thin-walled partitions inside was shown that replaced almost the entire left upper lobe, The patient underwent an exploratory thoracoscopy. Intraoperatively a giant bulla was seen that initially impressed to replace the entire upper lobe. Despite the large size we decided to attempt thoracoscopic resection preserving the remaining healthy parenchyma. Bullectomy was done using linear endoscopic stapling devices. To our knowledge this is the only case with such a large bulla resected entirely by VATS. Keywords: giant bulla, bullectomy, VATS

Laparoscopic retromuscular incisional hernia repair.

Abstract: A 67-year-old male with a history of a conventional right colectomy and hypertension was referred to our department for an incisional hernia and abdominal discomfort. Physical examination also showed a supraumbilical defect that was confirmed with a computed tomography scan. Laparoscopic Rives technique repair was done to repair the defect avoiding direct contact of the mesh with the intra-abdominal viscera.