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Borovansky JA et al. - Lymphangioleiomyomatosis (LAM) is a rare disease of unknown cause that traditionally affects young women of reproductive age. It is characterized by a proliferation of atypical smooth muscle cells, preferentially along the bronchovascular structures, that causes progressive respiratory failure. LAM is almost universally fatal without a lung transplant, although new clinical trials are ongoing. Because of its rareness and nonspecific presenting symptoms, patients often receive a missed or delayed diagnosis.

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