Cytokines and chemokines: At the crossroads of cell signalling and inflammatory disease.

Diagnosis and Treatment of Non-Muscle Invasive Bladder Cancer: AUA/SUO Guideline

Lymphangioleiomyomatosis (LAM) is a rare lung disease, which occurs sporadically or in association with the genetic disease tuberous sclerosis complex (TSC) 1, 2. Sporadic LAM affects ∼1 in 400,000 adult females; in TSC, LAM occurs in 30–40% of adult females 3, 4 and exceptionally in males and children 5, 6.

Patients with LAM usually develop progressive dyspnoea and recurrent pneumothorax, chylous collections and occasional haemoptysis 1. Extra pulmonary lymphadenopathy and cystic masses of the axial lymphatics termed lymphangioleiomyomas can result in abdominal and pelvic lymphatic obstruction 7. LAM is often associated with angiomyolipoma in the kidneys 8, and an increased frequency of meningioma 9. LAM varies in clinical features and rate of progression: this together with an absence of clear prognostic factors results in patients being given conflicting information about prognosis.

Diagnosis is made by tissue biopsy (generally from the lung but occasionally from lymph nodes or lymphangioleiomyomas) and/or a combination of history and high-resolution computed tomography scanning (HRCT). Pathological diagnosis relies on characteristic LAM cell morphology and positive immunoreactivity to smooth muscle actin and HMB-45 antibodies. Increasingly HRCT is used to diagnose LAM without resorting to lung biopsy; however a number of conditions with multiple pulmonary cysts can mimic LAM.

As LAM is rare, there have been no controlled trials of its management. Supportive treatment includes management of airflow obstruction and hypoxaemia with bronchodilators and oxygen respectively, specific treatment for surgical or pleural complications including pneumo- and chylothorax, and interventional treatment of renal lesions 10, 11. As LAM is a disease of females and is thought to be accelerated by oestrogen, oophorectomy, tamoxifen, progesterone and gonadotropin-releasing hormone (GnRH) analogues have been used without evidence that they are effective. The recent finding of abnormalities in the TSC1/2 genes resulting …

https://erj.ersjournals.com/content/erj/35/1/14.full.pdf

European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis

Background—Periprocedural bridging with unfractionated heparin or low-molecular-weight heparin aims to reduce the risk of thromboembolic events in patients receiving long-term vitamin K antagonists. Optimal periprocedural anticoagulation has not been established. Methods and Results—MEDLINE, EMBASE, and Cochrane databases (2001–2010) were searched for English-language studies including patients receiving heparin bridging during interruption of vitamin K antagonists for elective procedures. Data were independently collected by 2 investigators (κ=0.90). The final review included 34 studies with 1 randomized trial. Thromboembolic events occurred in 73 of 7118 bridged patients (pooled incidence, 0.9%; 95% confidence interval [CI], 0.0.0–3.4) and 32 of 5160 nonbridged patients (pooled incidence, 0.6%; 95% CI, 0.0–1.2). There was no difference in the risk of thromboembolic events in 8 studies comparing bridged and nonbridged groups (odds ratio, 0.80; 95% CI, 0.42–1.54). Bridging was associated with an increased...

Periprocedural Heparin Bridging in Patients Receiving Vitamin K Antagonists Systematic Review and Meta-Analysis of Bleeding and Thromboembolic Rates

Behçet's disease – a contemporary review

Is frozen section analysis in nephron sparing surgery necessary? a clinicopathological study of 301 cases

Expansion of γδ T-cells in Behçet's disease: Role of disease activity and microbial flora in oral ulcers

Large renal angiomyolipomas: digital subtraction angiographic grading and presentation with bleeding.

OBJECTIVE. The purpose of this study was to assess the immediate and midterm effects of embolization of the angiogenic component of renal angiomyolipoma in which a mixture of ethanol and polyvinyl alcohol is used as a permanent obliterator.MATERIALS AND METHODS. Seventeen patients with 18 renal angiomyolipomas (size range, 5.5-20 cm; mean size, 10 cm) were treated with transcatheter embolization over an 8-year period. Embolization was performed with a mixture of 96% ethanol and polyvinyl alcohol particles. Follow-up with CT (mean follow-up period, 22.4 months) and one (mean, 14 months) or two (mean, 27 months) angiographic examinations were conducted to evaluate changes in the size of the tumor and to look for recurrence of the angiogenic component.RESULTS. All initial angiograms showed the characteristic tortuous, hypervascular, and aneurysm-forming angiogenic component. Immediate complete obliteration was achieved in 17 tumors (94.4% technical success rate). There was one partial technical failure. Mean...

Ethanol and Polyvinyl Alcohol Mixture for Transcatheter Embolization of Renal Angiomyolipoma

The Efficacy and Safety of Perioperative Low Molecular Weight Heparin Substitution in Patients on Chronic Oral Anticoagulant Therapy Undergoing Transurethral Prostatectomy for Bladder Outlet Obstruction

Mordechai Duvdevani

Papers

Is frozen section analysis in nephron sparing surgery necessary? a clinicopathological study of 301 cases

Expansion of γδ T-cells in Behçet's disease: Role of disease activity and microbial flora in oral ulcers

Large renal angiomyolipomas: digital subtraction angiographic grading and presentation with bleeding.

Ethanol and Polyvinyl Alcohol Mixture for Transcatheter Embolization of Renal Angiomyolipoma

The Efficacy and Safety of Perioperative Low Molecular Weight Heparin Substitution in Patients on Chronic Oral Anticoagulant Therapy Undergoing Transurethral Prostatectomy for Bladder Outlet Obstruction