MARK WANDERLEY
Projetos de Pesquisa
Unidades Organizacionais
LIM/09 - Laboratório de Pneumologia, Hospital das Clínicas, Faculdade de Medicina
2 resultados
Resultados de Busca
Agora exibindo 1 - 2 de 2
- Natural history of incidental sporadic and tuberous sclerosis complex associated lymphangioleiomyomatosis(2020) MARCO, Fabiano Di; TERRANEO, Silvia; DIAS, Olivia Meira; IMERI, Gianluca; CENTANNI, Stefano; RINALDO, Rocco Francesco; GIULIANI, Lisa; LESMA, Elena; PALUMBO, Giuseppina; WANDERLEY, Mark; CARVALHO, Carlos Roberto Ribeiro; BALDI, Bruno GuedesLymphangioleiomyiomatosis (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 = 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 = 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 disease.
- Natural history of incidental sporadic or tuberous sclerosis complex associated lymphangioleiomyomatosis(2019) MARCO, Fabiano Di; TERRANEO, Silvia; DIAS, Olivia Olivia Meira; IMERI, Gianluca; CENTANNI, Stefano; GIULIANI, Lisa; LESMA, Elena; PALUMBO, Giuseppina; WANDERLEY, Mark; CARVALHO, Carlos Roberto Ribeiro; BALDI, Bruno Guedes