Sleep features in Lymphangioleiomyomatosis and their relationship with disease severity: a pilot study.

Lymphangioleiomyomatosis (LAM) is a rare pulmonary disease characterized by progressive airflow limitation. We conducted a pilot trial to investigate the incidence of sleep disorders, sleep quality and their relationship with disease severity. We performed pulmonary function tests, blood gas analysis, overnight 12-channels polysomnography and clinical assessments in 15 consecutive LAM patients.

Respiratory treatment in a patient with nemaline myopathy.

Respiratory involvement of nemaline myopathy is evident on the diaphragm and intercostal muscles function. The present case study aimed to describe the characteristics of the respiratory physiotherapeutic treatment in a patient with nemaline myopathy. Here, we describe a case of a 43-yearold woman with nemaline myopathy undergoing pulmonary rehabilitation during short hospital admission for an acute exacerbation.

Efficacy of hormonal suppression in a patient with chyluria due to lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is a rare lung disease affecting almost exclusively young women, characterised by abnormal proliferation of atypical smooth muscle cells. We describe a young woman presenting with chyluria secondary to the presence of a large retroperitoneal lymphangioleiomyoma. Immunohistochemical analysis revealed HMB45-negative LAM cells (HMB45 staining is absent only in rare cases) expressing low levels of estrogen receptors.