Stepwise Incremental Dose Schedule of Sirolimus Is Successfully Tolerated by a Patient With Lymphangioleiomyomatosis Who Was Initially Allergic to mTOR Inhibitors.

Lymphangioleiomyomatosis (LAM) is a rare disease involving the proliferation of LAM cells in the lungs and the axial lymphatic system and mechanistic target of rapamycin (mTOR) inhibitors are the only effective medicines for treating it. Patients suffering from LAM, who are allergic to mTOR inhibitors can be treated by desensitizing them to the medicine. A 39-year-old woman presented with dyspnea caused by chylous pleural effusion, ascites, and retroperitoneal lymphangioleiomyomas.

An elderly woman with Birt-Hogg-Dubé syndrome having multiple pulmonary cysts mimicking lymphangioleiomyomatosis.

The characteristics of the pulmonary cysts on the high-resolution computed tomography (HRCT) chest images are an important diagnostic clue to distinguish among cystic lung diseases. The diagnostic accuracy of HRCT was reported to be as high as 90% by experienced pulmonologists and radiologists. Herein, we report the case of an elderly woman with Birt-Hogg-Dubé syndrome (BHDS) whose HRCT images displayed lymphangioleiomyomatosis-like features of the pulmonary cysts, rendering it difficult for us to diagnose BHDS.

Impact of wound management strategies after revascularization for chronic limb-threatening ischemia.

Objective: There is no established consensus or guidelines for wound management after revascularization for patients with chronic limb-threatening ischemia (CLTI) without severe infection. This study is designed to evaluate the clinical effect of the wound management strategy on toe wounds after revascularization for CLTI.

Methods: This retrospective cohort study was performed at eight institutions affiliated with Keio University School of Medicine in Japan and included 261 patients who underwent revascularization for CLTI between April 2019 and July 2021.

Clinicopathological Impacts of Expression of Neuronal Markers in Lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is a tuberous sclerosis complex (TSC)-associated tumor, characterized by the expression of neural crest lineages including neuronal markers. Neural crest cells can differentiate into multiple cell types that contribute to tissues associated with TSC-related tumors, and TSC-related tumors could be specifically associated with distinct neural crest subtypes. This study aimed to clarify the clinicopathological effects of expression of neuronal markers in LAM.

Clinical and genetic features of 334 Asian patients with Birt-Hogg-Dubé syndrome (BHDS) who presented with pulmonary cysts with or without a history of pneumothorax, with special reference to BHDS-associated pneumothorax.

Background: The clinical pulmonary manifestations and genetic features of Birt-Hogg-Dubé syndrome (BHDS) in Asian patients remained unclear. We aimed to clarify the clinical features of BHDS-associated pneumothorax (PTX) and retrospectively investigate potential contributing factors in the largest Asian cohort to date.

Methods: We reviewed the clinical and genetic data collected in 2006-2017, from the BHDS patients who were Asian and presented with pulmonary cysts with or without a history of PTX.

Primary pulmonary lymphoma diagnosed by ultrasound-guided transthoracic needle biopsy.

Ultrasound-guided transthoracic needle biopsy is a relatively safe procedure diagnosing subpleural pulmonary mass and has high sensitivity in the diagnosis of lung cancer. However, the usefulness in other rare malignancies is unknown. This case shows the effectiveness in diagnosing not only lung cancer but also rare malignancies including primary pulmonary lymphoma.

Subcutaneous and muscle layer seroma complicated with thoracentesis.

Diagnostic thoracentesis is a basic and relatively safe diagnostic method for patients with pleural effusion. However, complications of thoracentesis are rare and not well known because of the low incidence. Herein, we report a case of subcutaneous and muscle layer seroma following thoracentesis.

IFN-λ3 and CCL17 as predictors of disease progression in patients with mild to moderate COVID-19: A cohort study in a real-world setting.

Coronavirus disease 2019 (COVID-19) has overwhelmed hospitals worldwide. In Japan, serum interferon lambda 3 (IFN-λ3) and C-C motif ligand (CCL) 17 levels have been used as predictive markers for disease progression to severe COVID-19. However, the relationship between these predictive markers and the disease progression of COVID-19 has not been well evaluated.

A case of negative-pressure pulmonary oedema after first-time electroconvulsive therapy.

Electroconvulsive therapy (ECT) has been used for many years as an important treatment modality in patients with schizophrenia. Recently, many new oral medications have become available to treat schizophrenia. However, ECT remains a valuable therapy for patients who are resistant to oral medications.

Long-term Outcomes of the Endurant and Excluder Stent Grafts for Endovascular Aneurysm Repair in a Japanese Cohort.

Purpose: To compare the long-term outcomes of the most widely used third-generation stent grafts, the Endurant and Excluder stent grafts, in Japanese patients using a multicenter registry.

Materials And Methods: A retrospective analysis of endovascular aneurysm repairs for abdominal aortic and iliac artery aneurysms using either the Endurant or the Excluder stent grafts from January 2012 to July 2019 at 10 Japanese hospitals was performed.

Results: A total of 332 and 378 repairs using the Endurant and Excluder stent grafts, respectively, were analyzed.

Idiopathic dendriform pulmonary ossification diagnosed by bronchoscopic lung cryobiopsy: A case report.

Dendriform pulmonary ossification (DPO) is a rare condition characterized by heterotopic bone production of unknown origin within the pulmonary tissue. Many cases are asymptomatic with slow progression and are often diagnosed incidentally during autopsy. Thus, only few cases are diagnosed while the patient is still alive since surgical lung biopsy is often needed for pathological diagnosis.

Folliculin haploinsufficiency causes cellular dysfunction of pleural mesothelial cells.

Birt-Hogg-Dubé syndrome (BHDS), an autosomal dominant inheritance disease caused by folliculin (FLCN) mutations, is associated with lung cysts and spontaneous pneumothorax. The possibility of FLCN haploinsufficiency in pleural mesothelial cells (PMCs) contributing to development of pneumothorax has not yet been clarified. Electron microscopy revealed exposed intercellular boundaries between PMCs on visceral pleura and decreased electron density around the adherens junctions in BHDS.

Isolation and characterisation of lymphatic endothelial cells from lung tissues affected by lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is a rare pulmonary disease characterised by the proliferation of smooth muscle-like cells (LAM cells), and an abundance of lymphatic vessels in LAM lesions. Studies reported that vascular endothelial growth factor-D (VEGF-D) secreted by LAM cells contributes to LAM-associated lymphangiogenesis, however, the precise mechanisms of lymphangiogenesis and characteristics of lymphatic endothelial cells (LECs) in LAM lesions have not yet been elucidated. In this study, human primary-cultured LECs were obtained both from LAM-affected lung tissues (LAM-LECs) and normal lung tissues (control LECs) using fluorescence-activated cell sorting (FACS).

Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is a rare destructive lung disease characterized by multiple thin-walled pulmonary cysts. The currently proposed diagnostic algorithm emphasizes the characteristic cystic appearance on high-resolution computed tomography (HRCT) so uncommon HRCT appearances present challenges to establishing the proper LAM diagnosis. The objective of this study is to accrue uncommon chest HRCT appearances, determine frequencies in both tuberous sclerosis complex (TSC)-associated LAM (TSC-LAM) and sporadic LAM (S-LAM) patients.

Aqueous olanexidine versus aqueous povidone-iodine for surgical skin antisepsis on the incidence of surgical site infections after clean-contaminated surgery: a multicentre, prospective, blinded-endpoint, randomised controlled trial.

Background: Surgical site infection (SSI) is the most common problem after surgery. Although several guidelines have indicated the efficacy of antiseptics, such as chlorhexidine-alcohol and povidone-iodine, in reducing SSI rate, the optimal recommendation is still not established. Olanexidine might have higher bactericidal activity than other antiseptic agents.

Transbronchial lung biopsy for the diagnosis of lymphangioleiomyomatosis: the severity of cystic lung destruction assessed by the modified Goddard scoring system as a predictor for establishing the diagnosis.

Background: A guide of patient selection for establishing the diagnosis of lymphangioleiomyomatosis (LAM) by transbronchial lung biopsy (TBLB) has not been established, although the pathological confirmation of LAM by lung biopsy is desirable, particularly when patients have no additional test results except typical findings of computed tomography (CT) of the chest.

Methods: We retrospectively reviewed the medical records of LAM patients who visited at our hospital from January 2010 to September 2018. We found 19 patients who underwent TBLB and collected the following data to investigate which parameters could predict the TBLB diagnostic positivity for LAM: age, degree of exertional dyspnea, pulmonary function test, cystic lung destruction visually assessed by the modified Goddard scoring system (MGS), serum level of vascular endothelial growth factor-D, and TBLB-related data.

Successful endovascular treatment of an ascending aortic pseudoaneurysm using an Amplatzer Vascular Plug II.

Pseudoaneurysms are a rare complication of cardiovascular surgery, caused by disruption of the aortic structure (adventitia, media, and intima). Some reports have observed an extremely high mortality rate associated with the open surgical repair of pseudoaneurysms. In elderly or highly frail patients, the use of less invasive procedures is preferable.

Impact of Stent Graft Design on External Iliac Artery Limb Occlusion Rates After Endovascular Aneurysm Repair: Post-hoc Analysis of a Japanese Multicentre Database.

Objective/background: It was hypothesised that a helical stent with expanded polytetrafluoroethylene (ePTFE) grafts could provide a preventive effect for external iliac artery (EIA) limb occlusion following endovascular aortic aneurysm repair (EVAR). Therefore, a post-hoc analysis of a Japanese multicentre database was conducted to assess the impact of the stent graft design on EIA limb occlusion rates.

Methods: Patients who underwent EVAR with EIA limb deployment between 2008 and 2016 were evaluated.

Comparison of olanexidine versus povidone-iodine for preventing surgical site infection in gastrointestinal surgery: study protocol for a multicentre, single-blind, randomised controlled clinical trial.

Introduction: The prevalence of surgical site infection (SSI) remains higher in gastrointestinal surgery than in other surgeries. Although several guidelines have indicated the efficacy of chlorhexidine and povidone-iodine in reducing the SSI rate, the optimal recommendation has still not been established. Therefore, it is necessary to determine the more effective antiseptic for surgical site preparation.

Concurrent presentation of an intraductal tubulopapillary neoplasm and intraductal papillary mucinous neoplasm in the branch duct of the pancreas, with a superior mesenteric artery aneurysm: a case report.

Background: Since the concept of intraductal tubulopapillary neoplasm (ITPN) was introduced in the current World Health Organization classification of tumors, the number of reports of ITPN occurrence has increased gradually. However, ITPN is usually located in the main pancreatic duct, with few reports of a branch duct ITPN. As a result, imaging protocols for the diagnosis of a branch duct ITPN have not been established.

Pulmonary Intravascular Large B-cell Lymphoma (IVLBCL) Disguised as an Asthma Exacerbation in a Patient with Asthma.

A 62-year-old man with asthma presented with a 1-month history of wheezing and exertional dyspnea. Although the wheezing symptoms disappeared after systemic corticosteroid therapy, the exertional dyspnea and hypoxemia did not improve. A diagnosis of intravascular large B-cell lymphoma (IVLBCL) with pulmonary involvement was suspected because of the increased serum lactic dehydrogenase (LDH) and soluble interleukin-2 receptor (sIL-2R) level, increased alveolar-arterial oxygen difference (AaDO), decreased pulmonary diffusing capacity for carbon monoxide (D) and scintigraphic, computed tomography (CT) and F-fluorodeoxyglucose (FDG) positron emission tomography (PET)-CT findings.

Surfactant protein-D predicts prognosis of interstitial lung disease induced by anticancer agents in advanced lung cancer: a case control study.

Background: Interstitial lung diseases induced by anticancer agents (ILD-AA) are rare adverse effects of anticancer therapy. However, prognostic biomarkers for ILD-AA have not been identified in patients with advanced lung cancer. Our aim was to analyze the association between serum biomarkers sialylated carbohydrate antigen Krebs von den Lungen-6 (KL-6) and surfactant protein D (SP-D), and clinical characteristics in patients diagnosed with ILD-AA.

Comparison of early vascular morphological changes between bioresorbable poly-L-lactic acid scaffolds and metallic stents in porcine iliac arteries.

Bioresorbable scaffolds have the potential to overcome several problems associated with metallic stents. Bioresorbable poly-L-lactic acid (PLLA) scaffold implantation for the treatment of peripheral artery disease has already been reported in animal models and clinical trials; however, no studies comparing PLLA scaffolds and bare metal stents (BMSs) with regard to early vascular morphological changes, identified using intravascular ultrasound (IVUS) analysis, have been reported. In this study, PLLA scaffolds and BMSs were implanted bilaterally in iliac arteries of five miniature pigs.

Comparison of Early Sac Shrinkage with Third-Generation Stent Grafts for Endovascular Aneurysm Repair.

Purpose: To compare the effects of the currently most-used third-generation stent grafts on early aneurysm sac shrinkage, a proposed marker of successful endovascular aneurysm repair (EVAR).

Materials And Methods: EVARs performed from 2009 to 2013 at 2 institutions were retrospectively analyzed. Patients treated with a Zenith, EXCLUDER, or Endurant device who completed imaging studies before EVAR and at 1, 6, 12, and 24 months afterward were included.

Loxoprofen-induced interstitial pneumonia: a case report.

Background: Loxoprofen is a nonsteroidal anti-inflammatory drug used in the treatment of many diseases. However, there are no case reports about loxoprofen-induced pneumonia. We have encountered a rare case of loxoprofen-induced pneumonia.