Academic Journal
Natural history of incidental sporadic and tuberous sclerosis complex associated lymphangioleiomyomatosis
| العنوان: | Natural history of incidental sporadic and tuberous sclerosis complex associated lymphangioleiomyomatosis |
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| المؤلفون: | F. Di Marco, S. Terraneo, O. Meira Dias, G. Imeri, S. Centanni, R. F. Rinaldo, L. Giuliani, E. Lesma, G. Palumbo, M. Wanderley, C. R. Ribeiro Carvalho, B. Guedes Baldi |
| المساهمون: | F. Di Marco, S. Terraneo, O. Meira Dia, G. Imeri, S. Centanni, R.F. Rinaldo, L. Giuliani, E. Lesma, G. Palumbo, M. Wanderley, C.R. Ribeiro Carvalho, B. Guedes Baldi |
| بيانات النشر: | Elsevier |
| سنة النشر: | 2020 |
| المجموعة: | The University of Milan: Archivio Istituzionale della Ricerca (AIR) |
| مصطلحات موضوعية: | Lymphangioleiomyomatosi, Natural history, Tuberous sclerosis complex, Settore MED/10 - Malattie dell'Apparato Respiratorio, Settore BIO/14 - Farmacologia |
| الوصف: | Lymphangioleiomyiomatosis (LAM) is a rare disease affecting women in childbearing age. A sporadic form (SLAM) affecting previously healthy women, and a form associated with Tuberous Sclerosis Complex (TSC-LAM) are described. Some data suggested that TSC-LAM could be a milder disease compared to S-LAM. To investigate whether the different disease behavior is real or due to overdiagnosis of screened TSC women, we compared the natural history of S-LAM and TSC-LAM in patients with incidental diagnosis. Clinical, and functional data from 52 patients (23 with S-LAM and 29 with TSC-LAM) were analysed. At diagnosis functional impairment was mild without differences between groups [FEV1 % pred was 97% (88-105) and 94% (82-106) in TSC-LAM and S-LAM, respectively, p 1⁄4 0.125]. Patients with S-LAM had less renal angiomyolipoma, and lower VEGF-D serum levels than TSC-LAM. There was no difference in the baseline extent of pulmonary cysts on CT scan and no difference in yearly rate of functional decline between TSC-LAM, and S-LAM patients [e.g. yearly rate of decline of FEV1 % pred was 0.51 ( 1.59 2.24) and 0.90 ( 1.92 -0.42) in TSC-LAM and S-LAM, respectively, p 1⁄4 0.265]. In conclusion, the natural history of TSC-LAM and S-LAM, when a potential selection bias due to screening in the latter group is balanced, is similar. Our study suggests that the prevalence of S-LAM can be significantly underestimated due to a tendency to diagnosis more frequently patients with more severe impairment, without identifying several ones with asymptomatic di |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| Relation: | info:eu-repo/semantics/altIdentifier/wos/WOS:000536125100007; volume:168; firstpage:1; lastpage:4; numberofpages:4; journal:RESPIRATORY MEDICINE; http://hdl.handle.net/2434/783804; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85084213015 |
| DOI: | 10.1016/j.rmed.2020.105993 |
| الاتاحة: | http://hdl.handle.net/2434/783804 https://doi.org/10.1016/j.rmed.2020.105993 |
| Rights: | info:eu-repo/semantics/closedAccess |
| رقم الانضمام: | edsbas.230CE44A |
| قاعدة البيانات: | BASE |
| DOI: | 10.1016/j.rmed.2020.105993 |
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