Database : MEDLINE
Search on : Lymphangioleiomyomatosis [Words]
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[PMID]: 29519926
[Au] Autor:Taveira-DaSilva AM; Julien-Williams P; Jones AM; Stylianou M; Moss J
[Ad] Address:Pulmonary Branch, NHLBI, NIH, Building 10, Room 6D05, MSC 1590, Bethesda, Maryland 20892-1590, USA.
[Ti] Title:Rates of Change in FEV and DL as Potential Indicators for mTOR Inhibitor Therapy in Pre-menopausal Lymphangioleiomyomatosis Patients.
[So] Source:Eur Respir J;, 2018 Mar 08.
[Is] ISSN:1399-3003
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:The value of rates of change in FEV and DL to predict disease progression, and initiation of mTOR inhibitor therapy has not been evaluated.In 84 pre-menopausal LAM patients, individual rates of change in FEV and DL and 95% confidence intervals (CI), were used to derive subsequent lowest values of FEV and DL that would prompt initiation of sirolimus therapy. These treatment criteria were compared with one based on FEV or DL ≤70% predicted. In 12 patients undergoing sirolimus therapy both methods for determining the optimal point for initiation of therapy were evaluated.Twenty-seven and 35 patients, respectively, who experienced rates of change in FEV and DL greater than expected, would have been excluded from therapy based on an FEV or DL >70% predicted. Twenty-five of the 84 patients were eventually treated but only when FEV or DL were ≤70%. Applying such treatment criteria to 12 patients undergoing sirolimus therapy, would have delayed treatment for many years.Pre-menopausal women in whom FEV or DL are declining at rates above the expected based on their individual rates of decline, should be considered for sirolimus therapy before the FEV or DL falls to ≤70%.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:Publisher

  2 / 1560 MEDLINE  
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[PMID]: 29228355
[Au] Autor:Kanemura H; Jinta T; Tamura T
[Ad] Address:From the Division of Pulmonary Medicine, Thoracic Center, St. Luke's International Hospital, Tokyo, Japan.
[Ti] Title:Pulmonary lymphangioleiomyomatosis and fertility treatment.
[So] Source:QJM;111(2):123-124, 2018 Feb 01.
[Is] ISSN:1460-2393
[Cp] Country of publication:England
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:In-Process
[do] DOI:10.1093/qjmed/hcx238

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[PMID]: 29506640
[Au] Autor:Sedigh Ebrahim-Saraie H; Heidari H; Khashei R; Nabavizadeh SH
[Ad] Address:Department of Bacteriology and Virology, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.
[Ti] Title:A rare case of complicated pericardial effusion with Elizabethkingia meningoseptica from Iran.
[So] Source:Cell Mol Biol (Noisy-le-grand);64(3):53-55, 2018 Feb 28.
[Is] ISSN:1165-158X
[Cp] Country of publication:France
[La] Language:eng
[Ab] Abstract:Infections due to Elizabethkingia meningoseptica, a Gram-negative oxidative bacterium are frequently founded in neonatal and immunocompromised individuals. The notable characteristic of this organism is its multi-drug resistance to common antibiotics used for infections caused by Gram-negative bacteria. We report a rare case of complicated pericardial effusion due to E. meningoseptica in a 2-year-old boy, who was admitted with chief complaints of fever and tachypnea (mentioned by his parents) and suffered from a rare lung malignancy (lymphangioleiomyomatosis). He was successfully treated with vancomycin.  E. meningoseptica infection is a rare situation in immunocompetent hosts, and we concluded that this infection was probably originated from device medicine or even hands of healthcare workers.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180306
[Lr] Last revision date:180306
[St] Status:In-Process
[do] DOI:10.14715/cmb/2018.64.3.9

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[PMID]: 29325986
[Au] Autor:Stoller JK
[Ad] Address:Cleveland Clinic, Cleveland, OH. Electronic address: stollej@ccf.org.
[Ti] Title:The Challenge of Rare Diseases.
[So] Source:Chest;, 2018 Jan 08.
[Is] ISSN:1931-3543
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Rare diseases pose particular challenges to patients who are affected, to the clinicians who care for them, and to the investigators who study their conditions. Although individually uncommon, rare diseases are common in the aggregate, with approximately 7,000 described rare diseases affecting 25 to 30 million US adults. Challenges posed to affected individuals and their families largely regard being diagnosed, receiving optimal care, and affording disease-specific medications. Challenges facing clinicians who care for affected individuals include gaining knowledge and experience in caring for such patients, and the availability of local experts and of expert guidelines. Finally, challenges to investigators regard the difficulty and expense of assembling large cohorts of affected individuals for study, and garnering funding for research. Fortunately, in the face of these challenges, the steadfast resolve of patient and clinical/scientific communities to enhance care and generate new knowledge has fostered a large inventory of countermeasures to offset these challenges. Although further progress is surely needed, successes to date include the formation of powerful patient advocacy groups which have brokered collaborations between the patient, scientific communities, the government, and pharma/device communities in service of detection, optimal care, and research; procurement of funds to support research; formation of consortia of clinicians and scientists to collaborate; and general activation of the respective patient communities to perpetuate these successes. Persisting needs include enhanced detection strategies, dissemination of knowledge regarding optimal care, and research to prevent, treat, and cure disease.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180305
[Lr] Last revision date:180305
[St] Status:Publisher

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[PMID]: 29476190
[Au] Autor:Suspitsin EN; Yanus GA; Dorofeeva MY; Ledashcheva TA; Nikitina NV; Buyanova GV; Saifullina EV; Sokolenko AP; Imyanitov EN
[Ad] Address:St. Petersburg Pediatric Medical University, St. Petersburg, Russia. evgeny.suspitsin@gmail.com.
[Ti] Title:Pattern of TSC1 and TSC2 germline mutations in Russian patients with tuberous sclerosis.
[So] Source:J Hum Genet;, 2018 Feb 23.
[Is] ISSN:1435-232X
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Tuberous sclerosis (TS) is a rare autosomal-dominant genetic disease. TS is manifested by the development of multiple hamartomas, which affect brain, kidneys, retina, skin and other organs. This study aimed to reveal specific features of molecular epidemiology of TS in Russia. Blood DNA samples from 61 patients with definite (n = 53) or probable (n = 8) clinical diagnosis of TS were tested for mutations in TSC1 and TSC2 genes using Sanger sequencing and MLPA analysis. Five TSC1/2 mutation-negative patients were further analyzed by exome sequencing. TSC1/2 mutations were detected in 53/61 patients (87%): 39 (74%) carried mutations in the TSC2 and 14 (26%) in the TSC1. Large rearrangements (exon deletions/duplications) affected exclusively TSC2, accounting for 15% of lesions of this gene. 6/8 (75%) patients with incomplete clinical manifestation of TS carried TSC1/2 gene lesion. Overall, 96% of detected germline TSC1/2 mutations occurred de novo. Patients with no mutation identified (NMI) differed from TSC1/2 mutation carriers, being lacking cortical tubers and subependymal nodules but having higher frequencies of renal angiomyolipomas, rhabdomyomas, and lymphangioleiomyomatosis. Exome sequencing failed to identify overt disease-causing mutation candidates among NMI patients. Russian patients with TS have increased frequency of TSC2 large gene rearrangements and TSC1/2 mutations occurring de novo as compared to other studies. Patients with suspected TS diagnosis but NMI status may represent a distinct disease entity.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180224
[Lr] Last revision date:180224
[St] Status:Publisher
[do] DOI:10.1038/s10038-018-0416-0

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[PMID]: 29208258
[Au] Autor:Martirossian A; Shah S; Carrete L; Valle J; Valentine V
[Ad] Address:Department of Internal Medicine (AM, LC), University of Texas Medical Branch, Galveston, Texas; Department of Internal Medicine, Division of Pulmonary Critical Care & Sleep Medicine (SS, JV), University of Texas Medical Branch, Galveston, Texas; Department of Internal Medicine, Division of Pulmo
[Ti] Title:Durability of Sirolimus for Lymphangioleiomyomatosis.
[So] Source:Am J Med Sci;354(6):603-607, 2017 12.
[Is] ISSN:1538-2990
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Lymphangioleiomyomatosis (LAM), a rare, multisystem disorder primarily affecting women of reproductive age, is characterized by cystic-appearing lung lesions, progressive loss of lung function, chylous effusions and renal angiomyolipomas. Sirolimus, an mammalian target of rapamycin inhibitor, has been shown to stabilize lung function, reduce symptoms and resolve chylous effusions in the short term for patients with LAM. Herein, we report a premenopausal female with LAM who experienced complete and durable resolution of her chylothoraces with significant and sustained improvement in lung function on sirolimus.
[Mh] MeSH terms primary: Antibiotics, Antineoplastic/therapeutic use
Lung Neoplasms/drug therapy
Lymphangioleiomyomatosis/drug therapy
Sirolimus/therapeutic use
[Mh] MeSH terms secundary: Adult
Female
Humans
Lung Neoplasms/complications
Lung Neoplasms/diagnostic imaging
Lymphangioleiomyomatosis/complications
Lymphangioleiomyomatosis/diagnostic imaging
Magnetic Resonance Imaging
Pleural Effusion/etiology
Tomography, X-Ray Computed
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:0 (Antibiotics, Antineoplastic); W36ZG6FT64 (Sirolimus)
[Em] Entry month:1712
[Cu] Class update date: 180223
[Lr] Last revision date:180223
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:171207
[St] Status:MEDLINE

  7 / 1560 MEDLINE  
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[PMID]: 29469631
[Au] Autor:Avila NA; Dwyer AJ; Moss J
[Ad] Address:1 Veterans Affairs Medical Center, Radiology Service, Washington, DC.
[Ti] Title:Reply to "Renal Lesions in Lymphangioleiomyomatosis and Tuberous Sclerosis Complex Are Rarely Biologically Aggressive".
[So] Source:AJR Am J Roentgenol;210(3):W132, 2018 Mar.
[Is] ISSN:1546-3141
[Cp] Country of publication:United States
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180222
[Lr] Last revision date:180222
[St] Status:In-Data-Review
[do] DOI:10.2214/AJR.17.19092

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[PMID]: 29469627
[Au] Autor:Trout AT; Towbin AJ; Franz DN
[Ad] Address:1 Cincinnati Children's Hospital Medical Center, Cincinnati, OH andrew.trout@cchmc.org.
[Ti] Title:Renal Lesions in Lymphangioleiomyomatosis and Tuberous Sclerosis Complex Are Rarely Biologically Aggressive.
[So] Source:AJR Am J Roentgenol;210(3):W131, 2018 Mar.
[Is] ISSN:1546-3141
[Cp] Country of publication:United States
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180222
[Lr] Last revision date:180222
[St] Status:In-Data-Review
[do] DOI:10.2214/AJR.17.18996

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[PMID]: 29458386
[Au] Autor:Zhan Y; Shen L; Xu W; Wu X; Zhang W; Wang J; Li X; Yang Y; Tian X; Xu KF
[Ad] Address:Department of Respiratory Medicine, Peking Union Medical College Hospital, Beijing, China.
[Ti] Title:Functional improvements in patients with lymphangioleiomyomatosis after sirolimus: an observational study.
[So] Source:Orphanet J Rare Dis;13(1):34, 2018 Feb 20.
[Is] ISSN:1750-1172
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:BACKGROUND: Sirolimus has been shown to be effective in patients with lymphangioleiomyomatosis (LAM). We wish to summarize our experience using sirolimus and its effectiveness in LAM patients. METHODS: We analyzed data from 98 patients who were diagnosed with definite or probable sporadic LAM based on the European Respiratory Society diagnosis criteria for LAM in 2010 at Peking Union Medical College Hospital and who had received sirolimus during January 2007 to June 2015. The data before and after the initiation of sirolimus therapy included pulmonary function tests, arterial blood gas analysis, 6-min walking distance (6MWD), size of chylous effusion and renal angiomyolipomas (AML), St. George's Respiratory Questionnaires (SGRQ) and vascular endothelial growth factor-D (VEGF-D) levels. Serum levels of sirolimus and adverse events were collected. RESULTS: Median follow-up was 2.5 years. Most patients had forced expiratory volume in 1 s (FEV ) values less than 70% predicted or symptomatic chylothorax. The mean changes before and after the initiation of sirolimus were - 31.12 ± 30.78 mL/month and 16.11 ± 36.00 mL/month (n = 18,p = 0.002) for FEV change, and - 0.55 ± 0.60 mmHg/month and 0.30 ± 1.19 mmHg/month (n = 17, p = 0.018) for P O change. 6MWD improved from 358.8 ± 114.4 m to 415.6 ± 118.6 m (n = 46, p = 0.004) and SGRQ total score from 57.2 ± 21.0 to 47.5 ± 22.8 (n = 50, p < 0.001). The median VEGF-D concentration decreased to 1609.4 pg/mL from 3075.6 pg/mL after sirolimus therapy (n = 41, p < 0.001). Patients with sirolimus trough levels of 5-9.9 ng/mL had an increase in FEV (p < 0.05). Sixty-five percent of patients (13/20) had almost complete resolution of chylous effusions. The most frequent adverse events were mouth ulcers, menstrual disorder, hyperlipidemia and acneiform rash, all were mild. CONCLUSION: Long-term use of sirolimus is safe in patients with LAM. LAM patients with FEV less than 70% predicted and symptomatic chylothorax are suitable for receiving sirolimus therapy. The maintaining serum trough levels of sirolimus are recommended between 5 to 9.99 ng/mL.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180222
[Lr] Last revision date:180222
[St] Status:In-Data-Review
[do] DOI:10.1186/s13023-018-0775-9

  10 / 1560 MEDLINE  
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[PMID]: 29402641
[Au] Autor:Benzaquen J; Pradelli J; Padovani B; Marquette CH; Leroy S
[Ad] Address:Service de pneumologie, hôpital Pasteur, universite Côte-d'Azur, FHU OncoAge, centre hospitalier universitaire de Nice, 06000 Nice, France. Electronic address: benzaquen.j@chu-nice.fr.
[Ti] Title:Emphysème, vous avez dit emphysème ? [Emphysema, did you say emphysema?]
[So] Source:Rev Mal Respir;35(1):83-87, 2018 Jan.
[Is] ISSN:1776-2588
[Cp] Country of publication:France
[La] Language:fre
[Ab] Abstract:INTRODUCTION: Chronic obstructive pulmonary disease (COPD) is a common condition that may initially look simple but may conceal other diseases capable of accelerating its natural history or even simulating it. We describe four cases presenting as COPD with emphysema that were reclassified on the basis of certain clinical characteristics and the radiological pattern. CASE REPORTS: A 52 year old never smoking woman presenting with emphysema was eventually diagnosed as having lymphangioleiomyomatosis on the basis of an abdominal CT scan showing kidney angiomyolipomas. A 44 years old smoker presenting with rapidly evolving emphysema was eventually diagnosed as having Langerhans cell histiocytosis on the basis of a previous chest CT (four years earlier) showing cavitating nodules. An airport refueler, 73 years old, with severe emphysema despite never having smoked, was eventually diagnosed as suffering from alpha-1 antitrypsin deficiency. The last patient was a 54 year old man, a never smoker, who presented with severe airflow limitation and multilobar hyperlucency, with bronchiectasis in the same areas. He was eventually diagnosed as having a severe form of the Swyer-James-MacLeod syndrome. CONCLUSION: These four case reports underline the importance of questioning the diagnosis of COPD when certain particular phenotypic characteristics are identified.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180219
[Lr] Last revision date:180219
[St] Status:In-Process


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