Background: Recommendations regarding key aspects related to the diagnosis and pharmacological treatment of lymphangioleiomyomatosis (LAM) were recently published. We now provide additional recommendations regarding four specific questions related to the diagnosis of LAM and management of pneumothoraces in patients with LAM.Methods: Systematic reviews were performed and then discussed by a multidisciplinary panel. For each intervention, the panel considered its confidence in the estimated effects, the balance of desirable (i.e., benefits) and undesirable (i.e., harms and burdens) consequences, patient values and preferences, cost, and feasibility. Evidence-based recommendations were then formulated, written, and graded using the GRADE (Grading of Recommendations, Assessment, Development, and Evaluation) approach.Results: For women who have cystic changes on high-resolution computed tomography of the chest characteristic of LAM, but who have no additional confirmatory features of LAM (i.e., clinical, radio...

/pdf/lymphangioleiomyomatosis-diagnosis-and-management-high-qvitswf1ym.pdf

Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline.

Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management.

Birt-Hogg-Dube syndrome (BHD) is a rare, autosomal dominant disorder characterized by the development of hair follicle tumors, renal tumors and pulmonary cysts. BHD is caused by heterozygous, predominantly truncating mutations in the folliculin (FLCN) gene located on chromosome 17, which encodes a highly conserved tumor suppressor protein. Although management of renal tumors of low malignant potential is the primary focus of longitudinal care, pulmonary manifestations including cyst formation and spontaneous pneumothorax are among the most common manifestations in BHD. Due to the lack of awareness, there is commonly a delay in the pulmonary diagnosis of BHD and patients are frequently mislabeled as having chronic obstructive lung disease, emphysema or common bullae/blebs. A family history of pneumothorax is present in 35 % of patients with BHD. Certain imaging characteristics of the cysts, including size, basilar and peripheral predominance, perivascular and periseptal localization, and elliptical or lentiform shape can suggest the diagnosis of BHD based on inspection of the chest CT scan alone. Recurrent pneumothoraces are common and early pleurodesis is recommended. A better understanding of role of FLCN in pulmonary cyst formation and long term studies to define the natural history of the pulmonary manifestations of BHD are needed.

/pdf/pulmonary-manifestations-of-birt-hogg-dube-syndrome-1d2b094bcx.pdf

Pulmonary manifestations of Birt-Hogg-Dubé syndrome

Importance The rate of postoperative pancreatic fistula (POPF) after distal pancreatectomy ranges from 13% to 64% To prevent POPF, polyglycolic acid (PGA) mesh was introduced, but its effect has been evaluated only in small numbers of patients and retrospective studies Objective To evaluate the efficacy of PGA mesh in preventing POPF after distal pancreatectomy Design, Setting, and Participants Prospective randomized clinical, single-blind (participant), parallel-group trial at 5 centers between November 2011 and April 2014 The pancreatic parenchyma was divided using a stapling device; no patient was given prophylactic octreotide Perioperative and clinical outcomes were compared including POPF, which was graded according to the criteria of the International Study Group For Pancreatic Fistulas A total of 97 patients aged 20 to 85 years with curable benign, premalignant, or malignant disease of the pancreatic body or tail were enrolled (44 in the PGA group and 53 in the control group) Interventions Patients in the PGA group underwent transection of the pancreas and application of fibrin glue followed by wrapping the PGA mesh around the remnant pancreatic stump Main Outcomes and Measures The primary end point of this study was the development of a clinically relevant POPF (grade B or C by the International Study Group grading system) The secondary end point was the evaluation of risk factors for POPF Results The study therefore evaluated a total of 97 patients, 44 in the PGA group and 53 in the control group Thirty-nine patients were women and 58 patients were men There were no differences in mean (SD) age (599 [120] years vs 545 [141] years, P  = 05), male to female ratio (10:13 vs 10:17, P  = 59), malignancy (409% vs 321%, P  = 37), mean (SD) pancreatic duct diameter (192 [075] mm vs 194 [095] mm, P  = 47), soft pancreatic texture (909% vs 830%, P  = 17), and mean (SD) thickness of the transection margin (169 [54] mm vs 164 [49] mm, P  = 63) between the PGA and control groups The rate of clinically relevant POPF (grade B or C) was significantly lower in the PGA group than in the control group (114% vs 283%, P  = 04) Conclusions and Relevance Wrapping of the cut surface of the pancreas with PGA mesh is associated with a significantly reduced rate of clinically relevant POPF Trial Registration clinicaltrialsgov Identifier:NCT01550406

/pdf/effect-of-polyglycolic-acid-mesh-for-prevention-of-2mljcczd4b.pdf

Effect of Polyglycolic Acid Mesh for Prevention of Pancreatic Fistula Following Distal Pancreatectomy: A Randomized Clinical Trial.

Since 1976, fibrin glues have been attracting medical interest, spreading from their initial use as a hemostatic agent in cardiovascular surgery to other fields of surgery. Studies have compared the efficacy of fibrin glues vs sutures in surgery. However, few comparisons have been made of the efficacy and safety of the different fibrin glues commercially available. Recently, fibrin glues have been tested as a scaffold delivery system for various substances inside the body (drugs, growth factors, stem cells). The infectious risk (viruses, new germs) of this blood-derived product was also studied in assays on viral inactivation methods. The development of autologous fibrin glues offers a solution to the problem of infectious risk. This review examines the current state of knowledge on the efficacy, safety and future potential of fibrin glues.

This article is open to POST-PUBLICATION REVIEW. Registered readers (see “For Readers”) may comment by clicking on ABSTRACT on the issue’s contents page.

/pdf/organic-glues-or-fibrin-glues-from-pooled-plasma-efficacy-548jhc8qxq.pdf

Organic Glues or Fibrin Glues from Pooled Plasma: Efficacy, Safety and Potential as Scaffold Delivery Systems

Objective
Currently, the treatment of pneumothorax varies among hospitals. Three factors account for the differences. First, pneumothorax expresses the state of disease instead of the disease itself. Some pneumothoraces heal on their own but patients with pneumothorax due to chronic diseases may need further intervention. The decision for the choice of treatment is up to the physician. Second, it is insufficient to treat pneumothorax with current treatment guidelines. Third, prognosis and follow-up after pneumothorax treatment is not well documented. Therefore, verification/assessment of treatment and its effect is insufficient.

Latest treatments for spontaneous pneumothorax

Background
Spontaneous pneumothorax is a major and frequently recurrent complication of lymphangioleiomyomatosis (LAM). Despite the customary use of pleurodesis to manage pnenumothorax, the recurrence rate remains high, and accompanying pleural adhesions cause serious bleeding during subsequent lung transplantation. Therefore, we have developed a technique of total pleural covering (TPC) for LAM to wrap the entire visceral pleura with sheets of oxidized regenerated cellulose (ORC) mesh, thereby reinforcing the affected visceral pleura and preventing recurrence.

/pdf/a-total-pleural-covering-for-lymphangioleiomyomatosis-ujy7uvys0w.pdf

A Total Pleural Covering for Lymphangioleiomyomatosis Prevents Pneumothorax Recurrence

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Latest treatments for spontaneous pneumothorax

A Total Pleural Covering for Lymphangioleiomyomatosis Prevents Pneumothorax Recurrence