Vitamin D binding protein: a new biomarker of disease severity in lymphangioleiomyomatosis

Liu, Heng-Jia; Henske, Elizabeth P.

doi:10.1183/13993003.01886-2018

Cited by 1 publication

(1 citation statement)

References 30 publications

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“…A high level of VEGF-D (over 800 pg/mL) was correlated with a more rapid rate of deterioration of FEV 1 values [78] and the presence of lymph exudates to the pleural cavity and the number of pulmonary cysts [77]. Other markers which have been associated with the severity of the course of pulmonary LAM are: the concentration of extracellular matrix metalloproteinases (MMP) in urine [58], the plasma concentration of vitamin D binding protein (VTDB) [79,80], VEGF-3 receptor and chemokine CCL21 [81] or the expression of the receptor of the human epithelial growth factor 3 (HER3) w LAM cells [61].…”

Section: Evaluation Of the Stage Of The Pulmonary Form Of Lam And Factors Affecting The Severity Of Its Progressionmentioning

confidence: 99%

Rozpoznanie i leczenie limfangioleiomiomatozy (LAM) z grupy PEComa

et al. 2021

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Lymphangioleiomyomatosis (LAM) is a rare, proliferative lung disease, leading to progressive damage of their structure and is a member of the PEComa neoplasm family (perivascular epithelioid cell tumors). In the patients, solid-cystic masses described as lymphangioleiomyoma or extrapulmonary LAM (E-LAM) can occur. E-LAM foci have been described in the mediastinum, supraclavicular lymph nodes, the liver, walls of the small and large intestine, the pancreas, mesentery. E-LAM masses can attain very large sizes -tumors 15-22 cm long have been described. On the basis of positive results of clinical trials sirolimus, a drug from the group of mTOR kinase inhibitors, was registered by the Food and Drug Administration (FDA) in May 2015 as the first and currently only drug for systemic LAM therapy. Sirolimus use is recommended in patients with LAM, accompanied by rapidly progressing deterioration of respiratory system function or FEV 1 ≤ 70% predicted value and in patients with pleural lymph exudate before applying invasive methods of treatment.

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